INDIAN JOURNALS CLINICAL PRACTIES by IJCP

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Indian Journal of

CLINICAL PRACTICE 301-360 Pages

IJCP

November 2010

Volume 21, Number 6

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Silent CP Angle Tumor

Dr KK Aggarwal

Group Editor-in-Chief

Head Office: 39 Daryacha, Hauz Khas Village, New Delhi, India. e-Mail: emedinews@gmail.com, Website: www.ijcpgroup.com

emedinews is now available online on www.emedinews.in or www.emedinews.org From the Desk of Editor in Chief Padma Shri and Dr BC Roy National Awardee

Dr KK Aggarwal

President, Heart Care Foundation of India; Sr Consultant and Dean Medical Education, Moolchand Medcity; Member, Delhi Medical Council; Past President, Delhi Medical Association; Past President, IMA New Delhi Branch; Past Hony Director. IMA AKN Sinha Institute, Chairman IMA Academy of Medical Specialities & Hony Finance Secretary National IMA; Editor in Chief IJCP Group of Publications & Hony Visiting Professor (Clinical Research) DIPSAR

9th November 2010, Tuesday Screening Older Smokers, Ex-smokers for Lung Cancer may Save Lives Largest study ever of lung cancer screening indicates that it helps reduce the likelihood of death. Effectiveness of CT scanning for lung cancer has been debated for years, because the test can pick up lung abnormalities like scars from past infections that are not cancer. Such irregularities are common in heavy smokers and can result in costly anxiety producing tests. Radiation is also a concern, because a CT scan, even low dose, delivers about 15 times more radiation than a chest x–ray. However, the authors of the latest study concluded that low–dose spiral CT scan can actually help reduce the death rate from lung cancer. Chest X–rays, an earlier form of screening tested repeatedly in the 1970s, have never been shown to save lives. The current eight–year study conducted at 33 sites, known as the National Lung Screening Trial (NLST), is the first to provide ‘clear evidence’ of a significant reduction in lung–cancer deaths with screening in a randomized controlled trial. Researchers noted 20 % fewer deaths from lung cancer among those screened with spiral CTs than among those given chest X–rays. The results were so conclusive that the study was terminated ahead of schedule. Dr KK Aggarwal Editor in Chief

Infertility Update What are the specific female causes for infertility? For a woman to conceive, certain things have to happen: intercourse must take place around the time when an egg is released from her ovary; the systems that produce eggs and sperm have to be working at optimum levels and her hormones must be balanced. Some women are infertile because their ovaries do not mature and release eggs. Problems affecting women include endometriosis or damage to the fallopian tubes. Other factors that can affect a woman’s chances of conceiving include being over– or underweight for her age. Female fertility declines sharply after the age of 35. Sometimes it can be a combination of factors, and sometimes a clear cause is never established. Some of the common causes of infertility of females include: ovulation problems, tubal blockage, age-related factors, uterine problems, previous tubal legation. —Dr Kaberi Banerjee, Infertility and IVF Specialist Max Hospital; Director Precious Baby Foundation

Medicolegal Update What is the approach to acute ethanol and isopropanol poisoning? • Do a full medical examination to exclude other causes of the patient’s condition, such as head injury • Correct fluid and electrolyte imbalance. • Hypoglycemia should be treated with oral or intravenous glucose.

Certain Type 2 Diabetes Patients at High Risk for CV Events Type 2 diabetes patients with gated myocardial perfusion single– photon computed tomography (SPECT) imaging abnormalities, who are otherwise asymptomatic, are at high risk for cardiovascular (CV) events and death according to the study published in Diabetes Care. Other significant risk factors were having a low estimated glomerular filtration rate and being a current smoker. Shopping for Shoes Here are few tips from The American Academy of Orthopaedic Surgeons on how to choose the right footwear and keep your feet happy: • • • •

Always measure both feet each time you go shoe shopping. Try on shoes late in the day, when the feet tend to be a bit larger. Women should opt for heels that are no higher than 2 1/4 inches. Make sure the shoes fit properly in the heels and the toes, with inch of space between the shoe and your longest toe. • Try on both shoes to be sure that they fit comfortably on both feet. Opt for the size that is most comfortable on your largest foot. • Walk around the store with both shoes on to make sure the fit is comfortable. Don’t count on ‘breaking them in.’ New Rules for Truck Drivers Returning after Stroke A U.S. expert panel has recommended that commercial truck and bus drivers who suffer a stroke should wait at least a year and be able to pass a driving test and a series of health assessments before getting in front of a wheel again. The panel, appointed by the Department of Transportation, also recommends annual check–ups on health and driving records as a requirement for continued driving. Levels of Coumarin in Cassia Cinnamon vary Greatly even in Bark from the Same Tree There is a vast variation in the amounts of coumarin in bark samples of cassia cinnamon from trees growing in Indonesia, scientists are reporting in a new study. That natural ingredient in the spice may carry a theoretical risk of causing liver damage in a small number of sensitive people who consume large amounts of cinnamon. The report appears in the Journal of Agricultural and Food Chemistry. —Dr Monica and Brahm Vasudev

—Dr Sudhir Gupta, Associate Professor, Forensic Medicine & Toxicology, AIIMS

Question of the Day What is a malaria paroxysm? Malaria causes an acute febrile illness which, in its most typical form, consists of febrile paroxysms occurring every 48 hours followed by afebrile intervals. These regular paroxysms separated by virtually asymptomatic intervals represent the only typical clinical feature suggestive of malaria. Typically, the following sequence may be observed: A cold stage, followed by a hot stage and a sweating stage. The total duration of the attack is 8-12 hours. The febrile paroxysm may be preceded by symptoms of fatigue, headache, dizziness, nausea and vomiting for 2-3 days before the attack. Children may have convulsions. The interval between paroxysms is determined by the length of the erythrocytic cycle of the parasite species involved (“tertian” or every 48 hours for Plasmodium falciparum, Plasmodium vivax and Plasmodium ovale; “quartan” or every 72 hours for Plasmodium malariae). Splenomegaly may be the only reliable sign at this stage. Nail or palmar pallor (anemia) and dark–colored urine, may be suggestive. In case of falciparum malaria, the paroxysm may rapidly evolve towards a severe complication of the disease (e.g., cerebral malaria or severe anemia): This is a medical emergency where diagnosis needs to be confirmed urgently and treatment started. In non–falciparum infections, fever disappears after a few paroxysms, even in the absence of treatment; relapses or recrudescences may occur a few weeks or months later. Lab Update Uric acid To detect high levels of uric acid, which could be a sign of the condition gout, or to monitor uric acid levels when undergoing chemotherapy or radiation treatment. —Dr Arpan Gandhi and Dr Navin Dang

Drug Update List of Drugs Prohibited for Manufacture and Sale through Gazette Notifications under Section 26a of Drugs & Cosmetics Act 1940 by the Ministry of Health and Family Welfare Fixed dose combination containing Pectin and/or Kaolin with any drug which is systemically absorbed from GI tract except for combinations of Pectin and/or Kaolin with drugs not systemically absorbed.

Indian Journal of

Online Submission

Clinical Practice

Volume 21, Number 6, November 2010

Contents

An IJCP Group Publication Dr Sanjiv Chopra Prof. of Medicine & Faculty Dean Harvard Medical School Group Consultant Editor Dr Deepak Chopra Chief Editorial Advisor

From the Desk of Group Editor-in-Chief

Alternative Drug Option for Cholera Treatment 305 KK Aggarwal

Dr KK Aggarwal CMD, Publisher and Group Editor-in-Chief Dr Veena Aggarwal Joint MD & Group Executive Editor Anand Gopal Bhatnagar Editorial Anchor IJCP Editorial Board

Original Article

A Randomized Placebo-controlled Comparative Study to Evaluate the Efficacy of HiOra-SG Gel in Stomatitis 307 VG Sukumaran, Amutha, P Vivekananda, D Palaniyamma

Dr Alka Kriplani Asian Journal of Obs & Gynae Practice

Review Article

Dr VP Sood Asian Journal of Ear, Nose and Throat Dr Praveen Chandra Asian Journal of Clinical Cardiology Dr Swati Y Bhave Asian Journal of Paediatric Practice Dr Vijay Viswanathan The Asian Journal of Diabetology Dr M Paul Anand, Dr SK Parashar Cardiology Dr CR Anand Moses, Dr Sidhartha Das Dr A Ramachandran, Dr Samith A Shetty Diabetology Dr Ajay Kumar Gastroenterology Dr Koushik Lahiri Dermatology Dr Georgi Abraham Nephrology Dr Sidharth Kumar Das Rheumatology

Managing the Adverse Effects of Radiation Therapy

312

Franklin J. Berkey

Managing Disruptive Air Passenger at Airport/In Sky

322

BK Singh

Clinical Study

A Study to Find Out Prevalence of Goiter in School Children of Dahod District, Gujarat

334

NG Padhiyar, JR Damor, VS Mazumdar

Dr V Nagarajan Neurology Dr Thankam Verma, Dr Kamala Selvaraj Obs and Gyne Advisory Bodies Heart Care Foundation of India Non-Resident Indians Chamber of Commerce & Industry World Fellowship of Religions

Case Report

Silent CP Angle Tumor SA Kareem, Valluvan M

339

Indian Journal of

Clinical Practice

Volume 21, Number 6, November 2010

Contents

Published, Printed and Edited by Dr KK Aggarwal, on behalf of IJCP Publications Pvt. Ltd. and Published at Daryacha, 39, Hauz Khas Village New Delhi - 110 016 E-mail: editorial@ijcp.com

Case Report

Ruptured Pulmonary Hydatid Cyst: The Camalote Sign

Printed at IG Printers Pvt. Ltd., New Delhi E-mail: igprinter@rediffmail.com printer_ig@yahoo.com

341

Manjot Kaur, Rakendra Singh

ÂŠ Copyright 2010 IJCP Publications Pvt. Ltd. All rights reserved. The copyright for all the editorial material contained in this journal, in the form of layout, content including images and design, is held by IJCP Publications Pvt. Ltd. No part of this publication may be published in any form whatsoever without the prior written permission of the publisher.

Infection Associated Hemophagocytic Lymphohistiocytosis

344

Rajani Dube, Subhranshu Sekhar Kar, Samarendra Mahapatra, Rajib Ray

Editorial Policies The purpose of IJCP Academy of CME is to serve the medical profession and provide print continuing medical education as a part of their social commitment. The information and opinions presented in IJCP group publications reflect the views of the authors, not those of the journal, unless so stated. Advertising is accepted only if judged to be in harmony with the purpose of the journal; however, IJCP group reserves the right to reject any advertising at its sole discretion. Neither acceptance nor rejection constitutes an endorsement by IJCP group of a particular policy, product or procedure. We believe that readers need to be aware of any affiliation or financial relationship (employment, consultancies, stock ownership, honoraria, etc.) between an author and any organization or entity that has a direct financial interest in the subject matter or materials the author is writing about. We inform the reader of any pertinent relationships disclosed. A disclosure statement, where appropriate, is published at the end of the relevant article. Note: Indian Journal of Clinical Practice does not guarantee, directly or indirectly, the quality or efficacy of any product or service described in the advertisements or other material which is commercial in nature in this issue.

Branchial Cyst with a Primary Carcinoma Thyroid

349

Samarth Shukla, Sourya Acharya, Sunita Vagha, Sobha Grover, Devendra Rajput

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From the Desk of Group Editor-in-Chief From the Desk of Group Editor-in-Chief

Alternative Drug Option for Cholera Treatment

Dr KK Aggarwal

Padma Shri and Dr BC Roy National Awardee Sr Physician and Cardiologist, Moolchand Medcity President, Heart Care Foundation of India Group Editor-in-Chief, IJCP Group Editor-in-chief, eMedinewS Chairman Ethical Committee, Delhi Medical Council Director, IMA AKN Sinha Institute (08-09) Hony. Finance Secretary, IMA (07-08) Chairman, IMA AMS (06-07) President, Delhi Medical Association (05-06) emedinews@gmail.com http://twitter.com/DrKKAggarwal Krishan Kumar Aggarwal (Facebook)

n a randomized double-blind trial, single dose azithromycin treatment of cholera resulted in better clinical and bacteriological results than six doses of ciprofloxacin. The study results were presented by Wasif Khan, MBBS, of the International Centre for Diarrhoeal Diseases Research in Dhaka, Bangladesh at the annual meeting of the Infectious Diseases Society of America (IDSA). He said that single-dose azithromycin is now the standard of care at the center, which treats around 30,000 cholera cases annually. The objective of the study was to find an alternative for ciprofloxacin, which in recent years had shown declining efficacy, though frank resistance was rare. A single dose of ciprofloxacin had clinical and bacteriological success rates of 93% and 97%, respectively in 1993-1994. The respective rates were 27% and 10% in 2003-2004. The center compensated by administering multiple doses of the drug, which brought the rates back upto 67% and 60%. The study included 246 men with cholera, who were randomly assigned to receive either a single dose of azithromycin 1 g or six doses of ciprofloxacin twice-daily for three days. The first two doses were 1 g each and the last four were 500 mg each. The salient observations of the study were as follows:  Clinical success (no watery stool within 48 hours of starting the drug): Azithromycin 63% vs ciprofloxacin 44%; the difference was significant at p = 0.005.  Bacteriological success (no cholera bacteria in the stool after 48 hours of starting the drug): Azithromycin 68% vs ciprofloxacin 45%; the difference was significant at p < 0.001.  Patients on azithromycin had significantly fewer stools and lower stool volume, at p = 0.004 and p < 0.001, respectively. The minimum inhibitory concentration (MIC) needed to block 90% of the bacteria was 0.404 µg/ml for ciprofloxacin, some 135 times greater than the amount needed when the drug was first tested against cholera. n

Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

305

306

Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Original article

A Randomized Placebo-controlled Comparative Study to Evaluate the Efficacy of HiOra-SG Gel in Stomatitis VG Sukumaran*, Amutha**, P Vivekananda†, D Palaniyamma‡

Abstract Stomatitis, a relatively common oral disease, is defined as the inflammation of the soft tissues of the oral cavity. Present treatment options for stomatitis are associated with adverse effects and there is a need for novel therapies that are effective and cause decreased morbidity. The present study was conducted to evaluate the clinical efficacy and safety of polyherbal formulation (HiOra-SG gel) in the management of stomatitis. Hundred individuals of either sex, from the age group of 28-44 years, with clinical diagnosis of stomatitis, were included in this randomized, double-blind, placebo-controlled clinical study. The individuals were randomized into Group A (HiOra-SG gel) or Group B (Placebo). All the individuals were advised to take HiOra-SG gel or similar-looking placebo on the tip of the index finger and apply it over their mouth ulcers 4-5 times daily for a period of three weeks. The individuals were evaluated for reduction in mouth ulcers, pain and swelling, and halitosis at weekly intervals for a period of three weeks by using a visual analog scale of 0-3. Statistical analysis was performed by repeated measures of ANOVA using Friedman’s test followed by Dunnett’s multiple comparison posthoc test. In the 50 individuals treated with HiOra-SG gel, a significant reduction (p < 0.001) was observed in mouth ulcer and swelling and pain at the end of three weeks of treatment. In subjective evaluation, majority of the individuals experienced a remarkable overall improvement. There was no relief in individuals treated with placebo. No adverse drug effects were either reported or observed during the entire study period. The beneficial clinical efficacy of polyherbal formulation (HiOra-SG gel) in the management of stomatitis could be due to the synergistic actions of its potent herbs. Therefore, from the above findings it can be concluded that HiOra-SG gel is clinically effective and safe in the management of stomatitis. Key words: HiOra-SG gel, stomatitis, polyherbal formulation

tomatitis, one of the most commonly encountered oral complaints vexing for both physicians and patients, is defined as the inflammation of soft tissues of the oral cavity occurring as a result of mechanical, chemical, thermal, bacterial, viral, electrical or radiation injury, or reactions to allergens, or as secondary manifestations of a systemic disease. Stomatitis can be the final common manifestation of a spectrum of conditions such as epithelial damage resulting from trauma; an immunological attack as in lichen planus, pemphigoid or pemphigus; damage because of an immune defect as in acquired immunodeficiency syndrome (AIDS) and leukemia; *Head of Department **Lecturer †Professor Dept. of Conservative Dentistry and Endodontics Sree Balaji Dental College and Hospital Velachery Main Road, Pallaikaranai, Chennai ‡Medical Advisor R&D Center, The Himalaya Drug Company, Bangalore Address for correspondence Dr D Palaniyamma Medical Advisor R&D Center The Himalaya Drug Company Makali, Bangalore - 562 123 E-mail: dr.palani@himalayahealthcare.com

Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

infections such as herpes viruses, tuberculosis and syphilis; cancer; nutritional defects such as vitamin deficiencies and inflammatory bowel disease.1 Recurrent aphthous stomatitis (RAS) is a specific type of stomatitis that presents with shallow, painful ulcers usually located on the lips, cheeks, gums or roof or floor of the mouth. The ulcers typically last from 7 to 14 days. The etiology of the disease is not known but appears to be multifactorial. Polymorphisms resulting in the increased production of interleukin-1p and tumor necrosis factor-α increase the risk for RAS.2 RAS can be classified according to clinical characteristics into minor RAS, major RAS and herpetiform aphthous stomatitis.3 Diagnosis is based on appearance and on exclusion, because there are no definitive histologic features or laboratory tests. Immunofluorescence is useful in the differential diagnosis between RAS and bullous skin diseases.4 Behçet syndrome may also manifest with classical RAS and a range of systemic complications affecting the eyes, joints, neurological system and skin.5 Chronic ulcerative stomatitis (CUS), another type of stomatitis, is common in women in their late middle age. 307

Original article Patients with CUS are reported to have a clinical history of painful, exacerbating and remitting oral erosions, and ulcerations. The histologic features are nonspecific, with a chronic inflammatory infiltrate, often appearing similar to oral lichen planus. Diagnosis of CUS requires surgical biopsy with immunofluorescence microscopic examination.6 Stomatitis venenata, an inflammation of the oral mucosa, is the result of contact allergy. The most common causative agents are volatile oils, iodides, dentifrices, mouthwashes, denture powders and topical anesthetics. Possible manifestations include erythema, angioneurotic edema, burning sensations, ulcerations and vesicles. Infections causing stomatitis are mainly viral, especially the herpes, Coxsackie and HIV viruses. Primary herpes simplex infection causes multiple vesicular lesions on the intraoral mucosa on both keratinized and nonkeratinized surfaces and always includes the gingiva. These lesions rapidly ulcerate. Clinical manifestations occur most often in children. Subsequent reactivations (secondary herpes simplex, cold sore) usually appear starting in puberty on the lip at the vermilion border and, rarely, on the hard palate. Bacterial causes of stomatitis are less common. Syphilis and tuberculosis are uncommon but increasing, especially in people infected with HIV. Fungal and protozoal causes of ulcers are also uncommon but increasingly seen in immunocompromised persons, and travelers from the developing world. Lichen planus, an autoimmune skin condition, may have oral and genital involvements. Oral lichen planus may also occur as an isolated entity. The ulceration is typically superficial, often described as erosion, and blends with the surrounding inflamed tissue. The ulcer may be associated with desquamative full thickness gingivitis. Drug-related stomatitis may mimic aphthous ulcers (aphthous-like ulceration) or oral lichenoid lesions. Many patients who present with an oral ulcer as the initial sign of malignancy have had symptoms for more than three weeks. Oral squamous cell carcinoma is the most common epithelial malignancy within the oral cavity. Location of oral lesions may help identify the cause. Interdental ulcers occur with primary herpes simplex or acute necrotizing ulcerative gingivitis. Lesions on 308

keratinized surfaces suggest herpes simplex, RAS or physical injury. Physical injury typically has an irregular appearance and occurs near projections of teeth, dental appliances, or where biting can injure the mucosa. If stomatitis is recurrent, viral and bacterial cultures, complete hemogram, serum iron, ferritin, vitamin B12, folate, zinc and endomysial antibody are done. Biopsy can be done for persistent lesions that do not have an obvious etiology. Treatment of stomatitis is based on the underlying cause. Conditions that predispose to oral ulceration, such as iron deficiency anemia, vitamin B12 deficiency, and folate deficiency must be treated. Potential triggers such as oral hygiene products containing sodium lauryl sulfate, trauma, food and drink with a low pH, and offending medications and possible allergens need to be removed.7,8 Treatment for recurrent stomatitis is usually palliative, because there is no known cure. Several therapies have been tried for recurrent stomatitis9 including topical and systemic corticosteroids, colchicine, thalidomide, and dapsone.10 Systemic immunomodulatory drugs (corticosteroids and thalidomide) have been the most effective at suppressing disease activity, presumably by modifying the underlying disease process. However, most of these drugs are associated with serious longterm adverse effects limiting their usage.11 In the present study, a polyherbal formulation (HiOra-SG gel) was evaluated in the management of stomatitis. The principal ingredients of this formulation include Glycyrrhiza glabra, Jasminum grandiflorum, Azadirachta indica, Ocimum basilicum, Boerhaavia diffusa, Syzygium aromaticum and Triphala. Aim of The Study

The present study was conducted to evaluate the clinical efficacy and safety of HiOra-SG gel in the management of stomatitis. Study Design

A double-blind, randomized, placebo-controlled comparative clinical study was conducted at Sree Balaji Dental College and Hospital between November 2009 and August 2010. The study protocol, case report forms, regulatory clearance documents, productrelated information and informed consent form were submitted to the â&#x20AC;&#x2DC;Institutional Ethics Committeeâ&#x20AC;&#x2122; and were approved by the same. Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Original article Material and Methods Inclusion Criteria

Individuals of either sex, aged more than 18 years, and who were clinically diagnosed with stomatitis, were included in the study provided they were able to attend the clinic on all assessment visits and were willing to give the informed consent, and to comply with the study procedures. Exclusion Criteria

Individuals below 18 years of age, with other dental and oral disorders; with active skin infection; or with known history or present condition of allergic response to cosmetic/pharmaceutical products, toiletries or their components or ingredients were excluded from the study. Pregnant and lactating women as well as individuals with genetic and endocrinal disorders and pre-existing systemic disease necessitating long-term medications were also excluded from the study. Study Procedure

Hundred individuals (29 males and 71 females) in the age group of 28-44 years who were clinically diagnosed with stomatitis were included in the study. After obtaining the informed consent, baseline history (which included personal data, description of symptoms, details of past medical history, family history and history of possible exacerbating factor/s) was obtained from each individual. The individuals were randomly divided into two groups: Group A (HiOra-SG gel) and Group B (placebo) - of 50 each. The mean ages (in years) of the individuals were 34.12 ± 4.8 in the HiOra-SG gel group and 33.84 ± 6.5 in the placebo group. There was no statistical difference between the two groups at entry (Table 1). All individuals were advised to take HiOra-SG gel or similar-looking placebo on the tip of their index finger and apply it over the mouth ulcers 4-5 times daily for a duration of three weeks. No other topical or systemic antibiotics were permitted during the trial. Individuals were evaluated for reduction in mouth ulcers, pain and swelling, and halitosis at weekly intervals for a period of three weeks. During each follow-up visit, local skin examination was done and observations were recorded in the case report form. All adverse events, either reported or observed by patients, were recorded with information about severity, date of onset, duration and action taken regarding the Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Table 1. Demographic Data of Individuals on Entry (n = 100) Parameters No. of individuals Mean age (years) (mean ± SD) Male:Female Smokers Alcohol Diet (vegetarian)

HiOra-SG gel 50 34.12 ± 4.8

Placebo 50 33.84 ± 6.5

14:36 12 11 17

15:35 12 10 19

study drug. Relation of adverse events to the study medication was predefined as ‘Unrelated’ (follows a reasonable temporal sequence from the administration of the drug), ‘Possible’ (follows a known response pattern to the suspected drug, but could have been produced by the patient’s clinical state or other modes of therapy administered to the patient) and ‘Probable’ (follows a known response pattern to the suspected drug that could not be reasonably explained by the known characteristics of the patient’s clinical state). Patients were allowed to voluntarily withdraw from the study, if they so desired without assigning reasons. For those withdrawing from the study, efforts were made to ascertain the reason for dropout. Noncompliance (defined as failure to take less than 80% of the medication) was not regarded as treatment failure, and reasons for noncompliance were noted. Primary and Secondary Endpoints

The predefined primary endpoints were rapid improvements in the symptoms of stomatitis, whereas the predefined secondary endpoints were incidence of adverse effects and patient compliance to the treatment. Follow-up The individuals were followed up at the end of first, second and third weeks of the treatment for various clinical parameters such as reduction in mouth ulcers, pain and swelling, and halitosis. The symptoms were evaluated using a visual analog score of 0-3, where 0 = nil, 1 = mild, 2 = moderate and 3 = severe. Statistical Analysis Statistical analysis was performed by repeated measures of ANOVA using Friedman’s test followed by Dunnett’s multiple comparison posthoc test. The scores for symptomatic relief from various parameters were expressed as mean ± SD. 309

Original article The minimum level of significance was fixed at p < 0.05. Statistical analysis was carried out using GraphPad Prism Software Version 4.03.

to 2.84 ± 0.12 (at the end of third week; p < 0.001). A reduction in the symptoms of stomatitis in individuals treated with placebo was observed; however, the values were not statistically significant (Table 2). No adverse drug effects were reported during the entire study period.

Results Results of the study showed that most of the individuals treated with HiOra-SG gel started responding to the therapy at the end of the first week of treatment.  The mouth ulcer score reduced from 2.64 ± 0.28 (at entry) to 2.35 ± 0.20 (at the end of first week), 1.45 ± 0.64 (at the end of second week; p < 0.05) and 0.23 ± 0.12 (at the end of third week) with a significance of p < 0.001.  Pain score reduced from 2.75 ± 0.18 (at entry) to 2.33 ± 0.50 (at the end of first week), 1.54 ± 0.48 (at the end of second week; p < 0.05) and 0.32 ± 0.15 (at the end of third week) showing significant (p < 0.001) improvement.  Swelling was reduced from 2.21 ± 0.62 (at entry) to 2.03 ± 0.41 (at the end of first week), 1.15 ± 0.22 (at the end of second week; p < 0.05) and 0.26 ± 0.04 (at the end of third week) showing significant reduction (p < 0.001).  Halitosis was reduced from 2.30 ± 0.48 (at entry) to 2.00 ± 0.22 (at the end of first week), 1.80 ± 0.16 (at the end of second week) and 1.44 ± 0.56 (at the end of third week); however, the values were not statistically significant.

Discussion Stomatitis, observed in numerous systemic diseases, affects upto 25% of the population7 and may be induced by trauma, nutritional deficiency, stress and allergens. Treatment is symptomatic, the goal being to lessen symptoms, reduce ulcer number and size, and increase disease-free periods. The best treatment is the one that will control ulcers for the longest period with minimal adverse effects. The treatment approach should be determined on the basis of disease severity (pain), the individual’s medical history, the frequency of flare-ups and the individual’s ability to tolerate the medication. The currently available treatments for stomatitis are associated with adverse effects and therefore, there is a need for novel therapies that are effective with less morbidity. In the present study, HiOra-SG gel (a polyherbal formulation) is evaluated for its efficacy and safety in the management of stomatitis. Deglycyrrhizinated licorice from G. glabra, an ingredient of HiOra-SG gel, has ulcer-healing as well as antiallergic effect on IgE, which is an important factor in triggering off the inflammatory process in the oral mucosa.12 Triphala, another ingredient of the gel, possesses antimicrobial, astringent and ulcer-healing activities.13 J. grandiflorum exhibits wound-healing activity.14 S. aromaticum has analgesic

In subjective evaluation, majority of the individuals experienced a remarkable improvement. The overall response to the treatment improved from 1.25 ± 0.50 (at the end of first week) to 2.50 ± 0.47 (at the end of second week; p < 0.05), which further improved

Table 2. Effect of Treatment on the Clinical Symptoms of Stomatitis Clinical symptom score

Initial

Mouth ulcers

2.64 ± 0.28

HiOra-SG gel (n = 50) First Second Third week week week 2.35 ± 1.45 ± 0.23 ± 0.20 0.64a 0.12b

Pain

2.75 ± 0.18

2.33 ± 0.50

1.54 ± 0.48a

0.32 ± 0.15b

2.21 ± 0.62

2.03 ± 0.41

1.15 ± 0.22a

0.26 ± 0.04b

2.30 ± 0.48 -

2.00 ± 0.22 1.25 ± 0.50

1.80 ± 0.16 2.50 ± 0.47a

1.44 ± 0.56 2.84 ± 0.12b

Swelling Halitosis Overall impression

p value ap

< 0.05

< 0.001 ap < 0.05

< 0.001 NS

ap bp

< 0.05

< 0.001

2.54 ± 0.18

Placebo (n = 50) First Second Third week week week 2.48 ± 2.22 ± 2.08 ± 0.18 0.24 0.36

2.74 ± 0.24

2.62 ± 0.18

2.58 ± 0.24

2.43 ± 0.08

2.11 ± 0.33

2.08 ± 0.28

1.96 ± 0.12

1.68 ± 0.27

2.28 ± 0.37 -

2.18 ± 0.25 0.22 ± 0.12

2.06 ± 0.04 0.46 ± 0.36

1.89 ± 0.14 0.98 ± 0.22

Initial

p value NS

Values are expressed in mean ± SD. a: Significant as compared to initial versus two weeks; b: Significant as compared to initial versus three weeks; NS: Not significant.

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Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Original article and mild anesthetic effects on sensitive nerve endings and is thus helpful in the management of pain associated with mouth ulcers.15 A. indica,16 G. glabra, and the alkaloids from leaves of J. grandiflorum17 are effective antimicrobials against certain bacteria, which are commonly found in dentureinduced stomatitis.18 O. basilicum is known to possess antimicrobial activity. B. diffusa exhibits excellent immunomodulatory activity, which helps in correcting the immune dysregulation, an important cause for the formation of recurrent aphthous ulcers. Boerhaavia alkaloids are also known to possess anti-inflammatory19 and wound-healing activities.20 The beneficial effects of HiOra-SG gel could be attributed to the synergistic actions (antimicrobial, antiallergic, anti-inflammatory, analgesic, anesthetizing, ulcer-healing, astringent and immunomodulatory activities) of its ingredients. Conclusion Currently available treatment options for the management of stomatitis have various limitations and adverse effects. In the present study, there was a significant reduction in the symptoms of stomatitis such as mouth ulcer, swelling and pain at the end of the treatment in the 50 individuals treated with HiOra-SG gel. Majority of the individuals experienced a remarkable overall improvement. Also, there was a reduction in halitosis though values were not statistically significant. This clinical efficacy of the polyherbal formulation (HiOra-SG gel) could be attributed to the synergistic actions of its potent herbs. In addition, no adverse drug effects were reported or observed during the entire study period. Therefore, it may be concluded that HiOra-SG gel is clinically effective and safe in the management of stomatitis. References 1. Scully C, Felix DH. Oral medicine - update for the dental practitioner. Aphthous and other common ulcers. Br Dent J 2005;199(5):259-64. 2. Guimaräes AL, Correia-Silva Jde F, Sá AR, Victöria JM, Diniz MG, Costa Fde O, et al. Investigation of functional gene polymorphisms IL-1 beta, IL-6, IL-10 and TNFalpha in individuals with recurrent aphthous stomatitis. Arch Oral Biol 2007;52(3):268-72. 3. Greenberg MS, Pinto A. Etiology and management of recurrent aphthous stomatitis. Curr Infect Dis Rep 2003;5(3):194-8. 4. Wilhelmsen NS, Weber R, Miziara ID. The role of immunofluorescence in the physiopathology and differential diagnosis of recurrent aphthous stomatitis. Braz J Otorhinolaryngol 2008;74(3):331-6.

5. Schwartz T, Langevitz P, Zemer D, Gazit E, Pras M, Livneh A. Behçet’s disease in Familial Mediterranean fever: characterization of the association between the two diseases. Semin Arthritis Rheum 2000;29(5):286-95. 6. Solomon LW. Chronic ulcerative stomatitis. Oral Dis 2008;14(5):383-9. 7. Scully C. Clinical practice. Aphthous ulceration. N Engl J Med 2006;355(2):165-72. 8. Bruce AJ, Rogers RS 3rd. Acute oral ulcers. Dermatol Clin 2003;21:1-15. 9. Porter SR, Scully C, Pedersen A. Recurrent aphthous stomatitis. Crit Rev Oral Biol Med 1998;9(3):306-21. 10. Muzyka BC, Glick M. Major aphthous ulcers in patients with HIV disease. Oral Surg Oral Med Oral Pathol 1994;77(2):116-20. 11. Thornhill MH, Baccaglini L, Theaker E, Pemberton MN. A randomized, double-blind, placebo-controlled trial of pentoxifylline for the treatment of recurrent aphthous stomatitis. Arch Dermatol 2007;143(4):463-70. 12. Shin YW, Bae EA, Lee B, Lee SH, Kim JA, Kim YS, et al. In vitro and in vivo antiallergic effects of Glycyrrhiza glabra and its components. Planta Med 2007;73(3): 257-61. 13. Kumar MS, Kirubanandan S, Sripriya R, Sehgal PK. Triphala promotes healing of infected full-thickness dermal wound. J Surg Res 2008;144(1):94-101. 14. Umamaheswari M, Asokkumar K, Rathidevi R, Sivashanmugam AT, Subhadradevi V, Ravi TK. Antiulcer and in vitro antioxidant activities of Jasminum grandiflorum L. J Ethnopharmacol 2007;110(3):464-70. 15. Lee MH, Yeon KY, Park CK, Li HY, Fang Z, Kim MS, et al. Eugenol inhibits calcium currents in dental afferent neurons. J Dent Res 2005;84(9):848-51. 16. Prashant GM, Chandu GN, Murulikrishna KS, Shafiulla MD. The effect of mango and neem extract on four organisms causing dental caries: Streptococcus mutans, Streptococcus salivavius, Streptococcus mitis, and Streptococcus sanguis: an in vitro study. Indian J Dent Res 2007;18(4):148-51. 17. Joy P, Raja P. Antibacterial activity studies of Jasminum grandiflorum and Jasminum sambac. Ethnobot Leaflets 2008;12:481-3. 18. Baena-Monroy T, Moreno-Maldonado V, FrancoMartìnez F, Aldape-Barrios B, Quindós G, SànchezVargas LO. Candida albicans, Staphylococcus aureus and Streptococcus mutans colonization in patients wearing dental prosthesis. Med Oral Patol Oral Cir Bucal 2005;10(Suppl 1):E27-39. 19. Bhalla TN, Gupta MB, Sheth PK, Bhargava KP. Antiinflammatory activity of Boerhaavia diffusa. Indian J Physiol Pharmacol 1968;12:37. 20. Manu KA, Kuttan G. Immunomodulatory activities of Punarnavine, an alkaloid from Boerhaavia diffusa. Immunopharmacol Immunotoxicol 2009;31(3):377-87.

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Review Article

Managing the Adverse Effects of Radiation Therapy Franklin J. Berkey

Abstract Nearly two thirds of patients with cancer will undergo radiation therapy as part of their treatment plan. Given the increased use of radiation therapy and the growing number of cancer survivors, family physicians will increasingly care for patients experiencing adverse effects of radiation. Selective serotonin reuptake inhibitors have been shown to significantly improve symptoms of depression in patients undergoing chemotherapy, although they have little effect on cancer-related fatigue. Radiation dermatitis is treated with topical steroids and emollient creams. Skin washing with a mild, unscented soap is acceptable. Cardiovascular disease is a well-established adverse effect in patients receiving radiation therapy, although there are no consensus recommendations for cardiovascular screening in this population. Radiation pneumonitis is treated with oral prednisone and pentoxifylline. Radiation esophagitis is treated with dietary modification, proton pump inhibitors, promotility agents, and viscous lidocaine. Radiation-induced emesis is ameliorated with 5-hydroxytryptamine3 receptor antagonists and steroids. Symptomatic treatments for chronic radiation cystitis include anticholinergic agents and phenazopyridine. Sexual dysfunction from radiation therapy includes erectile dysfunction and vaginal stenosis, which are treated with phosphodiesterase type 5 inhibitors and vaginal dilators, respectively. Key words: Radiation dermatitis, radiation pneumonitis, radiation esophagitis, emesis

hysicians diagnosed an estimated 1,480,000 new cancer cases in the United States in 2009 (excluding basal cell and squamous cell skin cancers, and carcinomas in situ, except urinary bladder).1 Nearly two thirds of these patients have treatment plans that may involve radiation therapy.2 Radiation therapy is used in curative, palliative, and prophylactic treatment plans, and is delivered through external beam, internal placement, or systemic administration, depending on the type of cancer and treatment goals. Radiation therapy works by damaging a cellâ&#x20AC;&#x2122;s DNA and inhibiting its ability to reproduce. Healthy, noncancerous cells usually recover after radiation therapy, whereas cancerous cells are often unable to repair the radiation-induced damage. Careful treatment planning, including the use of radiosensitizers and radioprotectants, aims to limit radiation exposure to noncancerous cells, thus limiting adverse effects. Table 1 lists adverse effects of

FRANKLIN J. BERKEY, DO, is an assistant professor of family and community medicine at the Penn State College of Medicine, Hershey, Pa. and a practicing family physician in the Penn State Hershey Medical Group in State College, Pa. He is also medical director of Home Nursing Agency Hospice in Centre County, Pa. Source: Adapted from Am Fam Physician 2010;82(4):381-8.

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radiation therapy, including risk factors and treatment recommendations.3-48 For most types of cancer, at least 75 percent of patients receiving radiation therapy are treated with intent to cure.2 Thus, with increasing numbers of cancer survivors and a relative lack of subspecialists, family physicians play an important role in cancer follow-up care.49 Although early radiation toxicities are usually part of the subspecialistâ&#x20AC;&#x2122;s care, patients with late effects often present to the family physician. Adverse effects from radiation therapy are classified as early or late. Early adverse effects occur during treatment or just after its completion, and usually resolve within four to six weeks. Late adverse effects are noted months to years after treatment completion and are often permanent. Secondary malignancies from radiation therapy, usually manifesting 10 to 15 years after treatment, are proportional to the amount of radiation received and inversely proportional to the age at which the radiation was received.50 Depression and Fatigue The prevalence of depression among patients with cancer varies from zero to 60 percent, depending on criteria, methodology, and populations studied. Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Review Article Table 1. Adverse Effects of Radiation Therapy, with Risk Factors and Treatment Options Adverse effect Psychological Depression Fatigue Skin Dermatitis Radiation recall

Associated cancers

Risk factors

Breast, lung, pancreatic, oropharyngeal3; brain4

Personal or family history of treated depression5

Head and neck, breast, prostate, perineal â&#x20AC;&#x201D;

Obesity11; concurrent chemotherapy11; high body mass index12 Chemotherapy agents (e.g., doxorubicin, fluorouracil, hydroxyurea, methotrexate, paclitaxel)

Topical steroids13,14; routine skin care with mild, unscented soap11 Same as dermatitis

Younger age, longer follow-up, increased radiation dose16

Awareness, although no definitive guidelines for cardiovascular screening17 Steroids19,21; pentoxifylline22

Selective serotonin reuptake inhibitors effective for depression but not for fatigue6,7 Brain, head and neck, breast, Low Karnofsky performance score, female Exercise9; sleep hygiene, stress lung, pelvic, lymphatic sex, active cancer8 reduction, cognitive and relaxation system4 therapies10

Cardiovascular and pulmonary Cardiovascular Hodgkin lymphoma, breast, disease lung15 Pneumonitis

Breast, lung, mediastinal

Gastrointestinal Xerostomia

Increased radiation dose, volume of lung irradiated, concurrent chemotherapy18; comorbid lung disease, poor baseline pulmonary function test, low Karnofsky performance score19; effect of smoking unclear19,20

Head and neck

Concurrent chemotherapy

Mucositis and esophagitis

Head and neck, thoracic

Enteritis (diarrhea)

Abdominal, pelvic

Proctitis

Anal, rectal, cervical, uterine, prostate, bladder, testicular

Emesis

Upper abdominal, craniospinal, pelvic

Genitourinary Cystitis

Erectile dysfunction Vaginal dryness and stenosis Infertility and teratogenicity

Treatment

Prostate, colorectal, bladder, pelvic36

Saliva stimulants and substitutes; pilocarpine23; amifostine for prevention and treatment24 Increased radiation dose, concurrent Viscous lidocaine, proton pump chemotherapy25,26 inhibitors, promotility agents25; bland diet; avoidance of alcohol, coffee, and acidic foods26 Increased radiation dose, hypertension, Low-residue diet, stool softeners, diabetes mellitus, prior abdominal surgery, loperamide25; small amounts of prior pelvic inflammatory disease25 dairy products27,28 Increased radiation dose, concurrent Oral sulfasalazine for prevention30; chemotherapy, inflammatory bowel sucralfate enema for chronic disease29 proctitis; hyperbaric oxygen31 2 Prior chemotherapy, fields above 400 cm , 5-hydroxytryptamine3 receptor radiation of upper abdomen32, total body antagonists and steroids33,34; 33 irradiation dopamine antagonists; acupressure35 Concurrent chemotherapy36

Pretreatment erectile dysfunction40,41; concurrent androgen ablation therapy, brachytherapy40 Cervical, endometrial, vaginal Age older than 50 years increases risk of stenosis46 Cervical, pelvic, testicular Age older than 40 years, greater dose to ovaries48 Prostate, colorectal

Intravenous hydration,37 uroprotective agents,38 and hyperbaric oxygen for hemorrhage39; symptomatic care for chronic cystitis36 Phosphodiesterase type 5 inhibitors (sildenafil, tadalafil)42-44; counseling, therapy45 Lubrication47; vaginal dilation46; counseling, therapy45 Egg and sperm preservation; ovarian transposition48

Information from references 3 through 48.

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Review Article Depression is highly associated with brain, breast, lung, pancreatic, and oropharyngeal malignancies, and is less associated with colon and gynecologic tumors.3,4 Risk factors include a personal or family history of treated depression.5 Because of comorbidities and combined treatment regimens with other modalities (e.g., chemotherapy), it is difficult to assess the direct effect of radiation on depression. Although selective serotonin reuptake inhibitors (based on studies of paroxetine) appear to significantly improve depressive symptoms among patients undergoing chemotherapy, they have no effect on cancer-related fatigue.6,7

Table 2. Management Strategies for Cancerrelated Fatigue

Patients with cancer often find fatigue to be the most distressing adverse effect of radiation therapy, more than the pain, nausea, and vomiting associated with the cancer and treatments.51 All patients with cancer should be assessed for fatigue at regular intervals,51 because radiation-related fatigue occurs in 80 percent of patients acutely and 30 percent chronically.4 Radiation-related fatigue is closely associated with irradiated tumors of the brain, head and neck, breast, lung, pelvis, and lymphatic system.4 Risk factors include low Karnofsky performance score, female sex, and active cancer.8 Exercise is often recommended for those undergoing treatment, and a mild to moderate improvement in fatigue has been demonstrated in some, but not all studies. A small randomized study of patients undergoing radiation therapy for prostate cancer demonstrated less fatigue and improved cardiovascular function in those who completed an eight-week exercise program.9 Proper sleep hygiene, stress reduction techniques, and cognitive and relaxation therapies may be beneficial.10 The use of psychostimulants, including methylphenidate and modafinil, remains investigational, and optimal dosing in patients with cancer has not been established.51 Yoga has been demonstrated to reduce depression, perceived stress, and morning cortisol levels in patients with breast cancer undergoing radiation therapy.52 Additional nonpharmacologic recommendations for managing cancer-related fatigue are listed in Table 2.51

Set priorities

Radiation Dermatitis Radiation dermatitis is a common adverse effectâ&#x20AC;&#x2018;of radiation therapy, often complicating treatment of breast, prostate, perineal, and head and neck 316

Activity enhancement* Maintain optimal level of activity Consider exercise program Consider physical or occupational therapy CBT for sleep Sleep restriction (limit naps, total time in bed) Sleep hygiene (avoid caffeine after noon, establish a sleepconducive environment) Stimulus control (going to bed at same time each night) Energy conservation Pace activities Delegate responsibilities Schedule activities at time of peak energy Postpone nonessential activities Limit naps to 20 to 30 minutes or less to minimize interference with nighttime sleep quality Maintain structured daily routine Attend to one activity at a time Psychosocial interventions CBT Stress management Relaxation techniques Support groups CBT = Cognitive behavior therapy. *Caution should be used in patients with metastatic bone disease, immunosuppression, thrombocytopenia, anemia, fever or active infection, or comorbid illness. Information from reference 51.

malignancies. However, there is no general agreement, and there are few controlled trials regarding prevention and treatment.53 Furthermore, clinical practice is often based on anecdotal evidence, and patient advice is variable and often contradictory.54 Early skin changes include erythema (Fig. 1), dry desquamation, and moist desquamation (Figs. 2 and 3); late adverse effects include pigmentation changes, telangiectasias (Fig. 4), hair loss, atrophy, fibrosis, and ulceration (Table 3).55 Risk factors for dermatitis include obesity, concurrent chemotherapy, and high body mass index.11,12 Radiation recall is a phenomenon of rapid onset skin irritation in a previously radiated field shortly after starting certain chemotherapy agents, most notably doxorubicin, fluorouracil, hydroxyurea, methotrexate, and paclitaxel. Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Review Article Table 3. Skin Reactions from Radiation Therapy Early changes

Late changes

Erythema

Pigmentation changes

Pink coloration

Hair loss (permanent)

Mild edema

Telangiectasia

Itching, burning, mild discomfort

Atrophy

Dry desquamation Partial loss of epidermal basal cells Dryness, scaling, peeling Hyperpigmentation Moist desquamation Complete destruction of basal cell layer

Ulceration Fibrous changes Recall phenomenon Rapid onset and progression after administration of chemotherapy drugs in previously irradiated area Symptoms of moist desquamation

Blister formation Nerve pain Serous drainage Information from reference 55.

Topical steroids and dexpanthenol-containing emollients are often prescribed for radiation dermatitis, although there is insufficient evidence to firmly support or refute this recommendation.13 A randomized, double-blind study evaluating 0.1% methylprednisolone and 0.5% dexpanthenol demonstrated a mild clinical benefit with both agents when used prophylactically and during treatment, compared with use of no topical creams, although data suggest a slightly greater benefit from the steroid cream.14 Although previous recommendations warned against the use of soap and water on skin in the radiation field, current advice permits use of routine skin washing with a mild, unscented soap.11 Deodorant use does not increase radiodermatitis,56 although antiperspirant with aluminum has not been well-studied. Topical aloe vera gel does not prevent or treat radiation dermatitis.57 Patients undergoing radiation therapy should avoid swimming, especially in chlorinated pools and hot tubs, and use care when applying adhesive bandages on skin in the radiated field.55 Soft tissue radionecrosis and osteoradionecrosis are ameliorated with traditional wound care techniques and, in some cases, hyperbaric oxygen therapy.58 Cardiovascular and Pulmonary Adverse Effects Cardiovascular disease secondary to radiation therapy is a well-recognized adverse effect, primarily seen in Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

patients who receive radiation for Hodgkin lymphoma and, to a lesser extent, breast and lung cancers.15 The estimated relative risk of fatal cardiovascular events after mediastinal radiation ranges from 2.2 to 7.2 for Hodgkin lymphoma and 1.0 to 2.2 for left-sided breast cancer.16 The risk increases with a younger age at treatment, longer follow-up, and increased radiation dose.16 There are currently no definitive guidelines for cardiovascular screening among such cancer survivors. Additionally, most of the research on secondary malignancies and cardiovascular adverse effects is based on patients who completed now outdated treatments for Hodgkin lymphoma.17 Pericarditis, an uncommon acute adverse effect, is seen primarily in patients treated for Hodgkin lymphoma.16 Radiation pneumonitis is seen in 5 to 15 percent of patients irradiated for breast, lung, and mediastinal tumors.18 The risk of developing radiation pneumonitis is directly related to the volume of irradiated lung, the amount of radiation given, and the use of concurrent chemotherapy.18 Additional risk factors include comorbid lung disease, poor baseline pulmonary function testing, and low Karnofsky performance score.19 Opinions vary regarding the effect of smoking. Some studies identify smoking as a risk for pneumonitis,19 whereas others suggest smoking may lessen the risk.20 Symptoms of radiation pneumonitis, including lowgrade fever, congestion, dry cough, pleuritic chest pain, and a sensation of chest fullness, usually develop one to three months after completion of radiation therapy. Diagnosis is difficult, often complicated by comorbid conditions and radiation injury to adjacent structures (e.g., esophagus, pericardium). Prednisone, in dosages of at least 50 to 60 mg per day for one week followed by an extended taper, has been shown to abate symptoms and improve lung function.19,21 Pentoxifylline is beneficial in preventing early and late lung toxicity.22 Supplemental oxygen may be necessary. Late pulmonary adverse effects include pulmonary fibrosis, which is often permanent and marked by progressive dyspnea. Gastrointestinal Toxicity Xerostomia, resulting from radiation injury of the salivary gland, is common with irradiation of the head and neck, especially with concurrent chemotherapy. Xerostomia is treated conservatively with saliva substitutes (e.g., water or glycerin-based) 317

Review Article and saliva stimulants (e.g., sour sweets, chewing gum). Pilocarpine is more effective than artificial saliva, although its effectiveness may not be seen until 12 weeks of therapy.23 Mucositis from radiation damage of the oral epithelium responds to topical anesthetics. Oral candidiasis is treated with topical anti-fungal washes or systemic antifungal agents. Amifostine, a free-radical scavenger radioprotectant, is beneficial in preventing and treating xerostomia, but there is insufficient evidence to support its use for radiation-induced mucositis and esophagitis.24 Radiation esophagitis is a common, dose-limiting, early adverse effect of radiation treatment of thoracic tumors. Its incidence is greater with higher radiation dose and concurrent chemotherapy.25,26 Early symptoms, usually following two to three weeks of treatment, include dysphagia and odynophagia. Topical anesthetics such as viscous lidocaine, proton pump inhibitors, and promotility agents may provide symptomatic relief.25 Dietary modifications shown to reduce incidence and severity include a bland diet and avoidance of alcohol, coffee, and acidic foods.26 Acute enteritis secondary to radiation therapy is usually a self-limiting process treated conservatively with dietary changes and antidiarrheal medications. Chronic symptoms begin three months or more after completion of radiation therapy and may last indefinitely. Risk of enteritis increases with greater radiation dose, as well as a history of abdominal surgery, pelvic inflammatory disease, hypertension, and diabetes mellitus.25 Mild intermittent symptoms of chronic enteritis are managed with a low-residue diet, stool softeners, and loperamide.25 Normal portions (one half cup or larger) of foods with moderate-to-high fiber content can exacerbate symptoms of chronic radiation enteritis.27 Lactose intolerance is common following radiation,28 although patients may tolerate small portions of dairy products.27 Radiation proctitis is seen in radiation treatment for anal, rectal, cervical, uterine, bladder, testicular, and, particularly, prostate cancers. Increased radiation dose and concurrent chemotherapy are risk factors, as is the presence of inflammatory bowel disease in patients receiving external beam radiation to the pelvis.29 Oral sulfasalazine is effective in the prevention of proctitis in all patients receiving pelvic radiation.30 Sucralfate enemas are recommended for treatment of chronic radiation-induced proctitis. Hyperbaric oxygen 318

therapy significantly improved healing responses in patients with refractory radiation proctitis in a study of 120 patients (number needed to treat = 3).31 Radiation-induced Emesis Risk factors for radiation-induced emesis include previous chemotherapy, radiation of the upper abdomen, and radiation fields greater than 400 cm2.32 Patients at greatest risk are those receiving total-body irradiation. Those at moderate risk include patients with radiation of the upper abdominal, pelvic, and craniospinal fields. Current clinical guidelines recommend the use of 5-hydroxytryptamine3 (5-HT3) receptor antagonists, which have antiemetic properties equal or superior to dopamine antagonists but with a more favorable adverse effect profile.33 The addition of dexamethasone (4 mg daily for five days) to the receptor antagonist 5-HT3 ondansetron provides additional benefit for prophylaxis against radiationinduced emesis.34 Lorazepam and diphenhydramine are also useful adjuncts, but are not recommended as single agents. Nonpharmacologic strategies, including dietary alteration, hypnosis, and acupressure,35 may benefit patients with radiation-induced emesis. Radiation Cystitis Acute radiation cystitis, including the more severe hemorrhagic cystitis, is an uncommon adverse effect of radiation therapy. Concurrent chemotherapy increases the risk.36 Intravenous hydration37 and uroprotective agents, including mesna38 and amifostine, have demonstrated effectiveness in treating hemorrhagic cystitis, although the studies are primarily in patients receiving chemotherapy. Hyperbaric oxygen therapy has also demonstrated improved outcomes in patients with radiation-induced hemorrhagic cystitis.39 Chronic radiation cystitis occurs most commonly following radiation for prostate, colorectal, bladder, and pelvic tumors. Medical therapy is aimed primarily at symptom amelioration. In the absence of infection, phenazopyridine is appropriate for dysuria, oxybutynin for urinary urgency, and flavoxate for bladder spasm.36 Sexual Dysfunction Sexual dysfunction, including impotence, is common following radiation therapy for prostate cancer and, to a lesser extent, colorectal malignancies. Assessment of the true incidence is difficult, complicated by the Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Review Article comorbidities often found in older patients, who are most likely to be diagnosed with prostate cancer. Erectile dysfunction is more common with brachytherapy than with external beam radiation.40 Combined treatment protocols with radiation and total androgen ablation, such as with leuprolide and bicalutamide, are synergistic in causing erectile dysfunction. Associated bladder and bowel dysfunction often lead to decreased intimacy and self-esteem. Initially after radiation, few men experience erectile dysfunction. However, erectile function begins to decline 12 months after treatment and levels off at two years posttreatment. One study of men who had normal erectile function before radiation therapy found that 38 percent of men had erectile dysfunction one year after treatment and 59 percent after two years.41 The same study found a higher rate of posttreatment erectile dysfunction in men who had pretreatment erectile dysfunction.41 Phosphodiesterase type 5 inhibitors, such as sildenafil and tadalafil, are effective for radiation-associated erectile dysfunction.42-44 Female sexual dysfunction is most common after radiation therapy for cervical and endometrial cancer. Adverse effects include decreased sexual interest, vaginal dryness and stenosis, dyspareunia, and general sexual dissatisfaction.59 There is some evidence to support the use of vaginal lubricating creams for vaginal irritation following radiation.47 Women older than 50 years are at higher risk of vaginal stenosis.46 The American Cancer Society recommends intercourse or use of a vaginal dilator three times per week to prevent stenosis.60 Men and women, as well as their spouses, may benefit from counseling or referral to a sex therapist or support group. Sexual counseling before treatment is also advised.45 Sperm or egg preservation should be discussed with patients considering posttreatment pregnancy. Ovarian transposition is an option for younger women requiring pelvic irradiation.48 Because of the teratogenicity of radiation, a pretreatment pregnancy test and birth control are essential. Hormonal contraception is contraindicated in patients with breast cancer and is a relative contraindication with several other malignancies. A male condom with spermicide provides a high rate of birth control, protection against sexually transmitted infections, and containment of seminal and vaginal secretions that may contain cytotoxic drugs.61 (To view full article please visit www.aafp.com)

Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

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27. Sekhon S. Chronic radiation enteritis: women’s food tolerances after radiation treatment for gynecologic cancer. J Am Diet Assoc. 2000;100(8):941-3. 28. Yeoh EK, Horowitz M, Russo A, Muecke T, Robb T, Chatterton BE. Gastrointestinal function in chronic radiation enteritis-effects of loperamide-N-oxide. Gut. 1993;34(4):476-82. 29. Willett CG, Ooi CJ, Zietman AL, et al. Acute and late toxicity of patients with inflammatory bowel disease undergoing irradiation for abdominal and pelvic neoplasms. Int J Radiat Oncol Biol Phys. 2000;46(4):995‑8. 30. Kiliç D, Egehan I, Ozenirler S, Dursun A. Doubleblinded, randomized, placebo-controlled study to evaluate the effectiveness of sulphasalazine in preventing acute gastrointestinal complications due to radiotherapy. Radiother Oncol. 2000;57(2):125-9. 31. Clarke RE, Tenorio LM, Hussey JR, et al. Hyperbaric oxygen treatment of chronic refractory radiation proctitis: a randomized and controlled double-blind crossover trial with long-term follow-up. Int J Radiat Oncol Biol Phys. 2008;72(1):134-43. 32. Radiation-induced emesis: a prospective observational multicenter Italian trial. The Italian Group for Antiemetic Research in Radiotherapy. Int J Radiat Oncol Biol Phys. 1999;44(3):619-25. 33. Kris MG, Hesketh PJ, Somerfield MR, et al.; American Society of Clinical Oncology. American Society of Clinical Oncology guideline for antiemetics in oncology: update 2006 [published correction appears in J Clin Oncol. 2006;24(33):5341–5342]. J Clin Oncol. 2006;24(18):2932-47. 34. Wong RK, Paul N, Ding K, et al.; National Cancer Institute of Canada Clinical Trials Group (SC19). 5-hydroxytryptamine-3 receptor antagonist with or without short-course dexamethasone in the prophylaxis of radiation induced emesis: a placebo-controlled randomized trial of the National Cancer Institute of Canada Clinical Trials Group (SC19). J Clin Oncol. 2006;24(21):3458-64. 35. Dibble SL, Luce J, Cooper BA, et al. Acupressure for chemotherapy-induced nausea and vomiting: a randomized clinical trial. Oncol Nurs Forum. 2007;34(4):813-20.

25. Coia LR, Myerson RJ, Tepper JE. Late effects of radiation therapy on the gastrointestinal tract. Int J Radiat Oncol Biol Phys. 1995;31(5):1213-36.

36. Marks LB, Carroll PR, Dugan TC, Anscher MS. The response of the urinary bladder, urethra, and ureter to radiation and chemotherapy. Int J Radiat Oncol Biol Phys. 1995;31(5):1257-80.

26. Sasso FS, Sasso G, Marsiglia HR, et al. Pharmacological and dietary prophylaxis and treatment of acute actinic esophagitis during mediastinal radiotherapy. Dig Dis Sci. 2001;46(4):746-9.

37. Ballen KK, Becker P, Levebvre K, et al. Safety and cost of hyperhydration for the prevention of hemorrhagic cystitis in bone marrow transplant recipients. Oncology. 1999;57(4):287-92. Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Review Article 38. Andriole GL, Sandlund JT, Miser JS, Arasi V, Linehan M, Magrath IT. The efficacy of mesna (2-mercaptoethane sodium sulfonate) as a uroprotectant in patients with hemorrhagic cystitis receiving further oxazaphosphorine chemotherapy. J Clin Oncol. 1987;5(5):799-803.

50. American Cancer Society. Second cancers caused by cancer treatment. http://ww2.cancer.org/docroot/MBC/ content/MBC_2X_Second_Cancers_Caused_By_ Cancer_Treatment.asp. Accessed January 2, 2009.

39. Chong KT, Hampson NB, Corman JM. Early hyperbaric oxygen therapy improves outcome for radiation-induced hemorrhagic cystitis. Urology. 2005;65(4):649-53.

51. National Comprehensive Cancer Network. Clinical practice guidelines in oncology. Cancer-related fatigue. http://www.nccn.org/professionals/physician_gls/f_ guidelines.asp. Accessed August 19, 2009.

40. Catalona WJ, Han M. Definitive therapy for localized prostate cancer—an overview. In: Wein AJ, Kavoussi LR, Novick AC, Partin AW, Peters CA, eds. CampbellWalsh Urology. 9th ed. Philadelphia, Pa.: Saunders Elsevier; 2006.

52. Vadiraja HS, Raghavendra RM, Nagarathna R, et al. Effects of a yoga program on cortisol rhythm and mood states in early breast cancer patients undergoing adjuvant radiotherapy: a randomized controlled trial. Integr Cancer Ther. 2009;8(1):37-46.

41. Turner SL, et al. Sexual dysfunction after radical radiation therapy for prostate cancer: a prospective evaluation. Urology. 1999;54(1):124-9. 42. Incrocci L, Hop WC, Slob AK. Efficacy of sildenafil in an open-label study as a continuation of a double-blind study in the treatment of erectile dysfunction after radiotherapy for prostate cancer. Urology. 2003;62(1):116-20. 43. Incrocci L, Slob AK, Hop WC. Tadalafil (Cialis) and erectile dysfunction after radiotherapy for prostate cancer: an open-label extension of a blinded trial. Urology. 2007;70(6):1190-3. 44. Incrocci L, Slagter C, Slob AK, Hop WC. A randomized, double-blind, placebo-controlled, crossover study to assess the efficacy of tadalafil (Cialis) in the treatment of erectile dysfunction following threedimensional conformal external-beam radiotherapy for prostatic carcinoma. Int J Radiat Oncol Biol Phys. 2006;66(2):439‑44. 45. Incrocci L, Madalinska JB, Essink-Bot ML, Van Putten WL, Koper PC, Schröder FH. Sexual functioning in patients with localized prostate cancer awaiting treatment. J Sex Marital Ther. 2001;27(4):353-63. 46. Brand AH, Bull CA, Cakir B. Vaginal stenosis in patients treated with radiotherapy for carcinoma of the cervix. Int J Gynecol Cancer. 2006;16(1):288-93. 47. Miles CL, Candy B, Jones L, Williams R, Tookman A, King M. Interventions for sexual dysfunction following treatments for cancer. Cochrane Database Syst Rev. 2007;(4):CD005540.

53. Naylor W, Mallett J. Management of acute radiotherapy induced skin reactions: a literature review. Eur J Oncol Nurs. 2001;5(4):221-33. 54. Glean E, Edwards S, Faithfull S, et al. Intervention for acute radiotherapy induced skin reactions in cancer patients: the development of a clinical guideline recommended for use by the college of radiographers. J Radiother Pract. 2000;2(2):75-84. 55. BC Cancer Agency. Care of radiation skin reactions. March 2006. http://www.bccancer.bc.ca/ HPICancerManagementGuidelines/SupportiveCare/ RadiationSkinReactions. Accessed July 4, 2009. 56. Théberge V, Harel F, Dagnault A. Use of axillary deodorant and effect on acute skin toxicity during radiotherapy for breast cancer: a prospective randomized noninferiority trial. Int J Radiat Oncol Biol Phys. 2009;75(4):1048-52. 57. Richardson J, Smith JE, McIntyre M, Thomas R, Pilkington K. Aloe vera for preventing radiationinduced skin reactions: a systematic literature review. Clin Oncol (R Coll Radiol). 2005;17(6):478-84. 58. Bui QC, Lieber M, Withers HR, Corson K, van Rijnsoever M, Elsaleh H. The efficacy of hyperbaric oxygen therapy in the treatment of radiation-induced late side effects. Int J Radiat Oncol Biol Phys. 2004;60(3):871-8. 59. Jensen PT, Groenvold M, Klee MC, Thranov I, Petersen MA, Machin D. Longitudinal study of sexual function and vaginal changes after radiotherapy for cervical cancer. Int J Radiat Oncol Biol Phys. 2003;56(4):937-49.

48. Marhhom E, Cohen I. Fertility preservation options for women with malignancies. Obstet Gynecol Surv. 2007;62(1):58-72.

60. American Cancer Society. Dealing with sexual problems. http://ww2.cancer.org/docroot/MIT/content/MIT_ 7_2X_Ways_of_Dealing_With_Specific_Sexual_ Problems.asp. Accessed April 1, 2010.

49. Miedema B, MacDonald I, Tatemichi S. Cancer followup care. Patients’ perspectives. Can Fam Physician. 2003;49:890-5.

61. Laurence V, Gbolade BA, Morgan SJ, Glaser A. Contraception for teenagers and young adults with cancer. Eur J Cancer. 2004;40(18):2705-16.

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Review Article

Managing Disruptive Air Passenger at Airport/ In Sky BK Singh

Abstract Objective: Review on management of air rage in the Sky/Airport and presenting a new concept of gradation and on multifactorial matrix of causation in era of current global hostility. Air rage is defined as an event of air passenger misconduct, as occurrence of hostility to crew, co-passengers and/or the aircraft, which threaten the safety of aircraft and its passengers at the airport or in the aircraft. Air passenger misconduct is a new phenomenon in civil aviation that is increasingly being experienced all over globe. In its extreme form, it may present as event like 9/11 of USA. Epidemiology and medicolegal aspect: The South Asian region contributes 10% of 2.5 billion air passengers who have globally travelled in 2008. There is no standard guideline for the problem of air rage in the Asia-Pacific region mainly within South Asian countries. The misfortune of air rage is dealt as per law of the nation of registration of aircraft. In the US, European countries and in the Asia-Pacific countries there is a law for handling air rage. In the Indian sky and probably in South Asian countries, the ground staff and or cabin crew experience minor incidences of misbehavior or other acts; about 1900-2000 cases at all 124 airports of India. For example, at Delhi Airport, there were 4-5 major incidences in domestic and 8-10 incidences at International Terminal in the year 2008. Majority of cases of bad behavior of air passengers are successfully handled by the ground staff/cabin crew and are not reported. Only a few cases are bought to notice of local police or to central authorities by management of air carrier. Now there is need to uniform global training, to educate public, the global Good Samaritan Law, R&D on travel health for effective handling of such situations. A new insight on causation: There is no predictor for misconduct by the air passenger. However, variables of airport tumult, factors of aircraft ambience and matrix of dimensions of health dynamics of individual interplay with each other leading to increase in stress on the ‘P²-S²’ ‘(Physical-Psychic-Social-Spiritual)’ unit more than the stress tolerance limit (STL) of the concerned person. At a particular moment, during life, events like air travel, thought process in a stressed mind hampers clear objective thinking in relation to time, place and person to his/her personal or point social need, which is compounded by some triggering event. It may result into aggressive behavior by a passenger as defence mechanism to her/his apparent threat, may present as psychosomatic illness and or may aggravate existing compromised physiology of an air traveller. This is grouped under Aero-Nomadic Stress complex (ANSC) and manifestations of symptoms of air travel related problems/diseases are part of Aero-Nomadic Stress syndrome. Handling of air rage: For the first-time in medical literature, the act of air passenger misbehavior has been classified in six grades depending on the gravity of act in relation to safety and security of passengers/crew and of aircraft. Methodology of handling rogue passenger on air needs proper training of crew as well as of the healthcare professionals traveling as co-passengers. There is need for strict law to handle air passenger misconduct and a Good Samaritan Law to protect interest of attending doctor, which is globally applicable. Key words: Air rage, stress tolerance limit, Aero-Nomadic Stress, Airport tumult, aircraft ambience, health dynamics, flight diversion, Good Samaritan Law

ir travel is the safest and fastest mode of travel of modern world. Presently 2.50 billion passengers have travelled in 2008.1 The International Air Traveller Association has projected 2.75 billion passengers by 2011.2 To which, approximately 10% contribution will be made by the Asia-Pacific’s South Asian region. With advanced techniques, Airports Authority of India Address for correspondence Dr (Major) BK Singh C/o: Adv. AP Singh, C-50E, Park, Maha Nagar Extension Near Horner’s School, Lucknow - 226 006 E-mail: majbks@hotmail.com

322

future long distance travel in the large aircrafts with approximately 800 passengers will soon be in operation. Mostly air travel passes smoothly in a pleasant and amiable atmosphere. Sometimes, suddenly, a rogue passenger disturbs the serene atmosphere of aircraft with unruly and violent conduct in the airplane making it a ‘sick aircraft’. Any incident in a flight or at the airport makes news. The incidence of September 11, 2001 in the USA is an extreme example of organized air passenger misconduct with devastating results both geopolitically as well as on the global safety and security. For the first-time in the world, civil aircrafts were used as weapon of mass destruction. All of us in Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Review Article our civilized world are conscious of its implication on the civil aviation. For the first-time in civil aviation literature, this article reviews grading of air passenger misconduct and structured handling of unruly behavior of the airline passenger/s at airport or on board in view of the current global hostility. Each airline during its operation makes a flight diversion due to life-threatening in-flight medical condition or on account of some unruly behavior by one or more air passenger/s endangering safety of other passengers, crew or the aircraft. In case of medical emergency crew seeks help from Good Samaritan healthcare professional available on board. In one study, it was found that there are 35-40% chances of getting a doctor on board.3 In 1999, in one of the International Flight, a disruptive passenger had died due to overuse of medication.4 At Delhi Airport, the writer has attended to few flight diversions due to medical reasons as well as due to unruly behavior of air passengers. Sometimes handling an unruly passengers may turn into a tragedy in form of injury or death either due to rough handling of agitated passengers or due to strapping of the accused passengers in a wrong posture or overmedication. It is time to share these experiences with the staff of air carriers/airports, the probable in-flight doctors and the policy makers of civil aviation, as well as with public to a standardized method to handle such incidences. A new term ‘Air-rage’, has been coined for misconduct by air passengers, on pattern of road-rage, where sensational example of passenger misconduct in mid-air has made the problem difficult because of lack of clarity on definition.5 The Federation Aviation Administration defines air passenger misconduct as incidents of aggression to crew, co-passengers and/or the aircraft, which threaten the safety of aircraft and its passengers.6 The International Transport workers Federation (ITF) defines the phrase air rage as: ‘One of the labels given to a wide variety of behaviors (displayed by passengers) that can range in their effects from causing discomfort to putting lives at risk’7 McDonnell suggests that there is an impression that violent behavior, in general, is increasing in society and while there are many examples of similarly unruly, aggressive, outrageous and seemingly excessively behavior in contemporary society. The unique environment of airport or an Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

aircraft in-flight makes such conduct disturbing, frightening and potentially dangerous.8 In event of such incidents, neither passengers and/or ground staffs have an opportunity to remove themselves, nor is there the option of requesting immediate outside assistance. In-flight, psychiatric emergencies cause a distinctive public health risk by putting the entire plane at risk, affecting the well-being of all co-passengers and the crew. Thus air passenger misconduct is a behavior of nonconformance to local socially acceptable activity or the act of hostility to crew, co-passengers either at airport and/or in aircraft, where safety of aircraft or of involved person/s is under threat. Epidemiology The International Transport Workers Federation (ITF) (2000a), consider that most statistics are likely to underestimate the problem. A survey by the International Air Transport Association (IATA) shows a five-fold increase in air rage incidents from 1,132 incidents in 1994, to 5,416 in 1997 (IATA 2000).9 According to the Aviation Safety Reporting System (ASRS) of the USA, unruly passenger reports have increased eight-fold 534 in 1999. In a study by Dr Douglous Watson of UK in 2000, it was found that from April-October 1999, 800 incidents were reported that were extrapolated to 1,200 for one year. Out of which 336 incidents were significant and 39 were of serious nature. In 43% of passengers related incidents, flying crew experienced some level of distraction from flying duties. In more than half of these duties, pilot deviation from flying duty was of some consequence. In 22% cases, a flight crew member left the cockpit to assist the flight attendant in dealing with an unruly passenger. On further analysis, out of total air-rage cases, 75% were males, and two-third were in age group of 20-30 years. Less than one-fourth were traveling alone.10 The two major Australian Airlines began recording of the data from 1998 when 30 episodes were noted, this increased to 650 in 1999. The main Japanese carriers report a year on year doubling of unruly behavior to 330 in 1999. Business class passengers are just as likely to cause trouble as those traveling in the economy class. In 1998, four out of 10 business class travelers witnessed verbal or physical abuse of 323

Review Article passengers or crew in-flight. In first class cabins, 6% of female business travelers reporting unwanted advances from fellow passengers.11

Table 1. Rank Order of Triggering Factors of Air Rage According to Airlines/Airport Staff (n = 110) Alcohol

22%

Smoking

11%

The FAA reported 214 cases in 2007 and 269 cases in 2008. In Australia, the office of Transport Security recorded 211 cases in 2007, and 279 cases in 2008;184 passengers were removed from flight and 15 diversions were made in USA in 2008. Ms Rachel Williams wrote in The Guardian on 7th December 2008, that the Civil Aviation Authority recorded 2,219 cases in 2007, and 1,359 in 2006. Out of which 42 passengers were physically restrained, violence in 58 cases, 19 times take-off of the aircraft was stopped, 14 diversions of flight, 235 passengers were removed from aircraft and 345 cases were reported to police while others were disposed of by concerned airlines due to minor nature of misdemeanor. According to a study conducted in the US, one in 37,000 passengers experiences in-flight medical emergency corresponding to 33 medical events per day in US sky. The incidence of psychiatric behavior is 3.5-15% of inflight medical emergencies12 presented mostly in form of aviophobia, claustrophobia, agoraphobia, social anxiety disorder, fear of flying, etc.

Baggage problem

18%

Employee attitude

09%

Lewd behavior

14%

Seat disputes

08%

Low stress tolerance capacity

13%

Food

05%

For air passenger misconduct, there is no centralized data available in India and probably in South Asian nations. According to a rough assessment, there are 1,900-2,000 cases of air passenger misconduct in a year in all 124 Indian Airports or Indian sky covering 2.8 million square nautical miles, where major cases of Grades 2-5 are 150-175 in number. However, at Delhi Airport, where the writer has long working experience of >20 years, minor incidences of misbehavior experienced by the ground staff of airport and/or cabin crew are about 250-275; while 4-5 major ones in domestic and 8-10 incidences of moderate degree occurred at International Terminal in year 2008. Sixty-four cases were not allowed to board, 19 were disembarked, 12 take-offs were delayed due to unruly behavior.13 In Table 1, analysis of triggering factors shows that more demand of alcohol, has been the triggering factor in approximately one-fourth cases as per opinion of dealing staffs. There are 90-100 diversions in a year mainly due to dense fog, weather conditions or technical reasons. Out of these, 8-10 flight diversions are due to medical 324

Table 2. Predisposing Factors of Air Rage in Order of Occurrence, According to Opinion of Airline/Airport Employees at IGI Airport, New Delhi 2008 (n = 170) 

Alcohol



Cramped conditions



Passenger personality



Excess baggage



Flight delays



High passenger expectation



Stress of air travel



Crew mismanagement



Smoking ban



Passenger denied upgrade

reasons or due to misconduct in a year. At Delhi Airport, out of 10 flight diversions from June 2007 to May 2008, there have been two incidents due to unruly behavior of a passenger while others were due to in-flight medical emergency. Table 2 illustrates an analysis of predisposing factors as opined by concerned ground staff of airport/airlines and cabin crew involved in handling of air passengers. All staff had work experience ranging from 2-10 years. As per study by Dr Watson of UK conducted in 2000, predisposing factors observed are alcohol intake obtained legally but consumed secretly in >50% cases and smoking in toilet or in aircraft in 36% of cases. The occurrence of incidence was seen 10% during climb or take off, 80% during cruising, 10% at time of descent and landing. Why Does It Happen? A New Insight Migration is a form of instinctive behavior of human beings. Human vitality coupled with sophisticated capability for innovation, has resulted in dramatic disturbance in environment, which humans now dwell in. These environments display an astounding diversity in comparison with stable characteristics of primitive humans both in genotype and in phenotype. This, in the language of information technology, may be termed as software and hardware of humans. The speed at which man-made environment has been altered in recent centuries has outstripped the pace at which natural selection can advance in the Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Review Article time-honored Darwinian manner. The evolutionary psychologists have conceptualized that human behavior is the outcome of archetypal needs and its fulfilment from environment. The archetypal needs are conceived as ‘P²-S²’ unit ‘(physical-psychic-social-spiritual)’14 unit which evolved through natural selection and which are responsible for determining the behavioral characteristics as well as cognitive experiences typical of human beings. Dr Anthony Stevens and John Price in 2000, the evolutionary psychiatrist opined that the possibility is that the gap between archetypal needs and environment fulfillment of those needs leads to stress. The wider the gap, more incapacitating the illness.

Health dynamics

Aircraft ambience

Airport tumult

Evolutionally human beings are nomadic in nature and now with rapid development of humans, as a society, ‘movement en-block’, has taken a form of organized sector, in from of travel and tourist industry. With high speed of travel and pressure of multitasking in a specific time period puts stress on the individual. In case of air travel, due to interaction of the matrix grouped in ‘airport tumult, aircraft ambience and the health status of individual’ creates stress on ‘P²-S²’unit as shown in Figure 1. Human beings are able to tolerate this stress well due to inherent coping ability. Gradually, with passage of time, during actual movement of travel with the sequential flow of point needs of a specific passenger, this stress keeps on increasing within the person and has cumulative impact. Majority of air passengers can cope-up with chain of needs/events. However, in some persons, at some particular moment, due to complexity of human nature, the stress on the ‘P²-S²’ unit of concerned

Air passenger with stress tolerance limit

Figure 1. Depiction of ‘P²-S²’ ‘(Physical-PsychicSocial-Spiritual) unit of air traveler.

Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

person, hampers in proper objective judgment making relation to time, place and person to his/her archetypal needs (social and personal). The stress, after crossing of its threshold, manifests into some symptoms depending upon the predominant factor of dynamics of health in the affected person presenting as some illness. In a few cases with personality traits as well as other factors along with precipitating variables, it manifests into misconduct or unruly behavior. This milieu of stress on ‘P²-S²’ unit is named as Aero-Nomadic Stress complex (ANSC). After crossing the threshold of tolerance limit due to stress excess, it manifests into various travelrelated illnesses grouped as the Aero-Nomadic Stress syndrome.15 All illnesses developing due to factors related to air travel like jet lag (desynchronosis), Economy Class syndrome, DVT, terminal syndrome, hypoxia, motion sickness, illness due to less humidity, illness due to altered cabin altitude, barotrauma leading to barosinusitis, otitic barotrauma, barodontalgia, problems due to air turbulence, features of Sick Aircraft syndrome (cf Sick Building syndrome), aviophobia, claustrophobia, agoraphobia, etc. Few so far, with our present knowledge we cannot screen and predict the action in the mind of a fidayeen (suicidal terrorist). Stressors of Air Travel

There are no predictors to find out air passenger misconduct beforehand. Alcohol intake, substance abuse, tobacco intake with underlying psychological stress in ambience of environmental anemia (less oxygen and re-circulated air) contribute in precipitating unruly behavior in a stressed airline passenger. The predisposing factors that cause accumulation of stressors leading to unruly behavior of air passengers following some triggering event, either at the airport or in the aircraft, can be grouped into different categories, which are:  Health dynamics of the passenger: Physical and mental health condition of the individual including his/her tolerance to others and spiritual acceptance to others faith, hunger, sleep deprivation, anxiety, immigration problems, security delays, separation from near and dear ones, impending reunions, traveling with agitated children, extreme work stress, alcohol intake, substance abuse or withdrawal, medications (time zone effects on dosing schedule, side effects of medication), 327

Review Article

Quantification of Stressors

Different variables of ‘P²-S²’ can be measured on stress scale and on impact range after conducting studies. This may open new dimension in air travel healthcare management. Figure 2 shows that an individual is able to cope-up with the stress of air travel because of inherent stress 328

Health dynamics

Aircraft ambience

Airport tumult

Air passenger with stress tolerance limit Figure 2. Graphic presentation of stress on ‘P²-S²’ unit and formation of ANSC.

Air

Health dynamics

Aircraft ambience



Coping with Stress by Air Passenger

Airport tumult



intoxication, personality traits (impulsive, anxious, paranoid, aggressive person, bi-polar disorder or frank psychosis), high-sensitivity on religious faith, skin color, nationality and on specific attire of the person, etc. Airport tumult or Airport/Air carrier factors: Troubled airport commuting from point of origin of journey, (en-route traffic congestion, civil strike or call for agitation in city by some political party or due to any other reason); difficulty in car parking, less number of gates for entry and airport seating specially during congestion at airport, underlying industrial problem of human resource management at airport leading to sudden strike by ground agents; ticketing problems, difficult check-in and flight delays; inadequately trained ground agents and flight crew, long queue for lavatories, inoperative or/inaccurate information displays, cabin baggage restrictions, no smoking places/policies, unsatisfactory food/ beverages, unsatisfactory seating arrangements, restricted movements due to security reasons, etc. Aircraft ambience or external and internal environmental factors: Inclement weather like extreme cold, dense fog, torrential rain, extreme heat, poor ventilation, prolong seating in an stationary aircraft due to operational reasons like sudden reduction of visibility at airport due to thick fog, reduced oxygen pressure, poor air conditioning of cabin due to technical reason, low humidity, physical confinement of cramped conditions frequently seen among deportees, uncomfortable seating in aircraft, excessive noise and vibrations, severe air turbulence, lack of privacy or proximity to stranger, activities of air crew/copassengers, perceived slow or unresponsive service, nonavailability of culturally viable food in aircraft, perceived feeling of threatened and victimization by crew or co-passengers, cultural and language misunderstanding, any technical snag, etc.

Passenger

Figure 3. Cracks in coping with stress of air passenger.

tolerance capacity. However, with continuous increase in stress, of stress tolerance capacity starts, as shown in the Figure 3. Due to increase in stress level, disintegration of coping mechanism starts. It results into manifestation of symptoms related to travel mainly in hemodynamically compromised person or with low stress coping ability. These symptoms can be grouped in Aero-Nomadic Stress syndrome as shown in Figure 4. Presentation of Misconduct/Air Rage

Usually passenger behaves in a manner, that does not conform to local socially acceptable behavior. Usually, he/she presses the service button frequently, makes unnecessary call for service, asking for personal phone number or other personal information from cabin crew, trying to become physical. Some times he/she starts demanding provision of some unacceptable Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Review Article

Health dynamics

Aircraft ambience

Air tumult



Air Passenger

Figure 4. Disintegration of coping mechanism graphic presentation of cracking of ‘P²-S²’ unit presenting as ANSC and Aero-Nomadic Stress syndrome.

items mostly more alcohol on board, does not comply with request to tie seat belt during take-off or during landing, or obey lawful instructions of crew/staff, etc. He/she starts behaving by speaking in high pitch and tone, turns verbally abusive or becomes aggressive in body language, may cause unprovocative attack on co-passengers or on crew/staff, coming out naked, indulging into obscene act including sexual act in public view in flying aircraft, fighting with cabin crew, smoking in aircraft or in its toilets, forcing entry into flight deck of aircraft, trying to open door or emergency exit of flying aircraft endangering safety, damaging safety equipments placed in aircraft or damaging fixed structure within plane. In extreme cases, a group of fundamentalists disguised as air passengers may plot to take control of flight operation by suddenly pouncing on flight deck and cabin crew basically to create terror and to get international attention. Classification of Air Passenger Misconduct

There is no classification of unruly behavior of air passenger available in aviation literature. A small misadventure of passing comment to cabin crew to the extreme conduct of serious violent conduct is measured with a same yardstick of aircraft misconduct rules. Mostly air passengers feel harassed when charged under such rules. Therefore, there is a need to grade misconduct of air passenger. A suggested classification of grading of misconduct is as follows:  Grade 1: Minor incidents (undesirable language or gestures effectively persuaded and resolved by crew Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010



member). Normal in-flight operation. Grade 2: Incidents of a moderate nature which are not being resolved by persuasion (repetition of Grade 1, obscene or lewd behavior), establishment of procedures for crew members to report incidents to the operator/controlling agency. Initiation of standing operative procedure (SOP). Grade 3: Incidents with serious safety concerns (threats, physical blows or assault with co-passagers or crew, hampering flight operations, tampering emergency or safety equipment) forcing use of additional help or force by cabin crew and/or copassengers to control situation. Slightly disturbed in-high operation. Grade 4: Threat to safety (attempt to enter flight deck, use of weapons, sabotage, creating a sense of terror in cruising aircraft by violent conduct forcing commander for priority landing at nearest/ destination airport. Completely disturbed in-high operation leading to stand by for activation of alternate SOP for possible nonscheduled landing. Grade 5: Serious injury to co-passengers and/or to crew members/to ground staff/or to accused forcing commander for an urgent emergency landing to nearest airport. Activation of alternate SOP for nonscheduled landing. Grade 6: More than one passengers involved in rage leading to chaos and attempt to overpower flight operation initiating activation of emergency procedure for hijacking of aircraft by commander of aircraft. Activation of aircraft emergency procedure.

Benefits of Classification

The grading of air passengers misconduct, will help in reporting of the incident, its structured management, possible legal issues, awarding punitive measures by competent authority, acknowledging and rewarding the help from Good Samaritan fellow healthcare professional and fellow passengers, crew members, who have helped in managing a rogue air passenger, awarding insurance claim, if any. Compulsory incident reporting of air passenger misconduct is mandatory in the West while in many countries of the world probably in South Asian countries, it is not obligatory, unless there is serious implication or legal issues are involved. Monthly/Quarterly submission of required data by all 329

Review Article air carriers and airport operator including ‘Nil’ report are submitted to Director General Civil Aviation/ Ministry of Civil Aviation/Controlling Authority as per proforma given below. The data required includes:  Flight information including aircraft type and flight identification  Date and time of incident  Name(s) of crew members/staff involved in the occurrence  Description of the incident  Category of grade of interference  Any suspected causal factor/s, if known  Description of injuries to any passenger on board or to crew members/ground staff, passengers including the accused from the incident  Action(s) taken by pilot-in-command and crew member(s)/Senior ground staff during the incident and the level of success of those actions  Description of involvement of law enforcement official  Whether the incident occurred, while the aircraft was on the ground or during flight and if so, what phase of flight  Any available information on passenger identity  Any additional information, which the crew member deems it pertinent. Medicolegal Aspect Conduct aboard aircraft is governed by laws of the nation in which aircraft is registered according to the Tokyo Convention signed by 170 countries in 1963 and The Suppression of Unlawful Acts Against Safety of Civil Aviation Act, 1982 (Act No. 66 of 1982). The Tokyo convention pertains to acts, which deals with safety or good or bad discipline on board. This is modification of Hague Convention of 1970 and Montreal Convention of 1971. It deals with offences of committing violence on board, offence at airport, destruction of/damage to air navigation facilities, powers and jurisdiction, etc. In most severe instances 330

of disruptive passengers, deaths have occurred due to either the aggressive responses of co-passengers to the individual or due to effects of administered medicines (mostly tranquillizers) combined with pre-existing alcohol or other substances. Doctors are reluctant to acknowledge their presence due to different specialty, litigation fears or to be summoned as a witness. For doctors in specific, when acting within scope of one’s practice, liability in the use of physical and or chemical restraint is also mitigated by Good Samaritan Laws in USA. In India, there is no such law, but till date, no doctor has been charged for professional misconduct while discharging duty on board. In 2002, in one case of death of an US citizen during an international flight originating from Delhi Airport, members of the US Court had come to Delhi to record witness of the attending doctor who had examined the case at Delhi and had opined that the patient had to be shifted to the hospital. Handling the Hostile Air Passenger By Crew/Staff

The cabin crew/staff are trained in Crew Resource Management (CRM) techniques in handling unruly passengers. Some airlines use a Yellow Card system similar to that used in soccer. Every crew has been trained to handle the agitated passenger on board. But almost every one has asked for an annual refresher course. Before seeking help from a doctor traveling as co-passenger, the crew’s thought process is enmeshed with clouds of doubt regarding presence of doctor, his/her specialty, capacity and experience in handling such internationally sensitive avio-medico-social situation along with availability of in-flight resources. In making a final decision by the concerned commander, the gravity of situation, recommendation by healthcare professional and corporate policy of air carrier also plays a role. Once a final decision to seek assistance is made, an announcement of SOS is made on public announcement system. Healthcare Professional

The usual sequence of events of unruly behavior originate with passenger behaving and acting in threatening manner to cabin crew then with Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Review Article co-passengers first verbally followed by physical and violent manners. He/she may become violent and may damage the equipments or may try to open the exit gate or may try to force himself to the cockpit. This can start at any time during the flight. Presently there are long nonstop 12-14 hours international flights with more than 350 passengers on board originating from Indian airports to intercontinental distances. Due to threat of terrorism, passengers are more aware and may be more reactionary in their responses leading to mob hysteria or mob mentality, which may be disastrous to agitated passenger. A suggested approach to passenger misconduct management is as follows:  Decide whether to offer assistance. By physician/ healthcare professional: n Legal obligation: Pre-existing patient doctor relationship is a requirement in most of the states of the USA and Canada to render assistance on board. In most of the European nations, however physicians are legally obligated to provide ‘Good Samaritan’ care. In India, and probably in South Asian countries, there is no such obligation to a physician. n Ethical obligation: Medical code of ethics provides emergency care on humanitarian duty as a social responsibility of a physician.  Identify yourself to the flight crew, indicating the assistance you can provide.  Unruly passenger management n Role of the volunteer healthcare professional is to act as a consultant to the crew, who ideally remain in charge of the incident n Determine if there are other clinician co-passengers who may be of assistance n After establishing rapport and attempting verbal reassurance, assess medical and psychiatric symptoms of agitated passenger as much as possible n Offer oral medication (diazepam/haloperidol, 5-10 mg) if appropriate and available n Develop a plan for physical moderation in the event of oral medication is ineffective and/or refused n Proceed with physical containment by hand cuffing and tying legs and ensure appropriate Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010



positioning of the individual in left lateral position on floor of aircraft or by strapping on the seat n Consider the use of adjunctive chemical restraint (injection diazepam 5/10 mg slowly intravenously) if necessary n Closely monitor and document the status of the individual for the duration of physical and chemical restraint during the flight n Counsel and give appropriate help to other affected co-passenger, crew, the family member/s to adopt antianger technique by cognitive behavior therapy. Consider whether the situation warrants aircraft diversion and advice crew accordingly. Document all actions undertaken.

Restraining of Agitated Passenger

Utilization of resources of in-flight medical kit, in-flight personnel, involuntary physical restraint and chemical restraint materials depends upon discretion of the attending physician. One should be careful in applying physical restraint. It should be administered competently and in a humanitarian manner. Ideally, it is applied with help of a team of 2-4 members who can be crew or fellow passengers. Every attempt should be used to maintain the self-respect of individual. Once physical restraint is applied, the individual should be placed in left lateral recumbent position and secured to a stretcher, board or at aircraft seating by strap or tape if available. Thereafter restrained individual should be monitored for possible complications of restraint such as:  Aspiration  Respiratory compromise  Dehydration  Peripheral neuropathies or vascular compromise due to wrong positioning of restrained person  Head or soft tissue injury secondary to struggling  Adverse reaction or allergic reaction to administered medications  Sudden death Monitoring

Recommended monitoring includes recording heart rate, respiratory rate, skin color, pulse in limbs, warmth 331

Review Article and color of limbs, level of consciousness, grade of agitation, time and dose of medication administered, response to medication, intake of any food or liquid, discussion with restrained passengers and/or their family members. To handle Grade 5 and 6 of air rage, crew should be trained and should be in preparedness.



Diversion of Flight

Once the Commander of aircraft decides to divert flight on recommendation of cabin crew or of Good Samaritan Doctor. He/she has to consider the availability of the nearest safe airport for safety of aircraft and its passengers, capacity and grading of airport to handle such flight, provision of basic facilities to handle medical emergency besides considering other flight operation requirement like fuel load, meteorological conditions, safety concern and international standing as per ICAO norms. The Air Navigation Service of respective country provides the required information on VHF radio frequency mode. Economic Burden of a Flight Diversion

Before deciding to divert a flight, the commander has to access pros and cons about emergency, life of passenger, phase of flight and position of aircraft in flight path, availability of nearest airport from security and for proper infrastructure to tackle situation of the in-flight emergency. There is no information available on economic impact of diversion of a transcontinental international flight due to air rage or on board medical emergency. However, as per internal analysis of a reputed IATA approved globally operational airline, on a flight diversion, approximately 600 US Dollars per minute is the expense for a Boeing 737 with complete passenger load.16 This does not include the medical expenses of victim or accused or compensation of delay of flight time of other passengers. Usually in a diverted flight, time consumed is 45-180 minutes depending on the situation of emergency and or aircraft operational needs including fuel load. Lessons 

332

Need of legislation: In many countries of world, the doctor, if available on board or at airport, may volunteer to provide professional help due to ethical reasons or medical reason and or both. Due



to litigation apprehensions, sometimes doctors refrain from rendering help. There is a need of global Good Samaritan Law like legislation. The experts from ICAO, IATA and ACI (Airports Council International) may be requested to ponder on the issue. Training needs: In Civil Aviation with increase in number of incidences there is need for training of ground staff as well as the crew of all air carriers to deal with passenger misconduct in an effective way. Passenger education on misconduct and its implication is also an important tool in minimizing such incidences. Policy makers and Controllers of Civil aviation of regional countries including India should make a ‘Zero-tolerance’ policy for misconduct of some unruly cloud appearing as air passenger.

Further Scope

Additional work is required to identify ‘P²-S²’ unit and quantify nomadic stress complex and its tolerance limit in cases of air travel and also in the other modes of travel like surface transport (Water and Road) among travelers in a global travel health study. After identifying contribution of different factors culminating into manifestation of symptoms, one can develop some package to de-stress mechanism to minimize this morbidity pattern of air travel.17 Conclusion Training, Education and Effective Law, R&D is need of the hour in this part of civil aviation of the world, along with global networking. Acknowledgements Dr Karan Singh, Member of Parliament, Former Minister of Civil Aviation and Health, Government of India. Ministry of Civil Aviation of India including Airports Authority of India; Dr SPS Gaur, Head, Experimental Medicine Central Drug Research Institute, Lucknow, UP, India; National Medical Library, Director General Health Services, New Delhi.

References 1. International Civil Aviation Organization (ICAO) Statistics 2009. 2. Passenger numbers to reach 2.75 billion by 2011. IATA Press release 2007. Available at: http://www.iata.org/ pressroom/pr/papers/2007-24-10-01.aspx Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Review Article 3. Skjenna OW, Evans JF, Moore MS, Thibeault C, Tucker AG. Helping patients travel by air. CMAJ 1991;144(3):287-93. 4. Associated Press (1999). ‘The Unruly Passenger dies inflight’ in ABCNEWS.Com http://1204.204.202.137 .115/sections/travel/DailyNews/hungary981207.html. 26 March 2000. 5. Wikipedia on Google search engine. http://en.wikipedia. org/wiki/Air_rage. 6. Pierson K, Power Y, Marcus A, Dahlberg A. Airline passenger misconduct: management implications for physicians. Aviat Space Environ Med 2007;78(4): 361‑7. 7. International Transport Workers’ Federation (2000a) Air rage: the prevention and management of disruptive passenger behaviour. A guide to good pratice and practical action from aviation trade unionists, the transport industry and safety regulators. ITF Publications May 2000. 8. McDonnell AA. Defusing violent situations: low arousal approaches. Travel Med Intern 1999;17(1):22-6. 9. International Air Transport Association, (2000). Key initiatives on disruptive passengers. http://www.iata.org/ pr/pr00mare.htm.

10. Peter Rolfe. ‘Air-Rage’ Disruptive Passengers. The causes and the cures, 2000. 11. Independent (2000) Bad Business. http://www. independent.co.uk/enjoyment/travel/IndyTraveller/ airrage180300popb.shtml. 12. Matsumoto K, Goebert D. In-flight psychiatric emergencies. Aviat Space Environ Med 2001;72 (10):919‑23. 13. Personal information from officials of air carriers and from domestic (Palam) and International Police Stations of Indira Gandhi International Airport, New Delhi. Mail Today 2009;p8(col):1-5. 14. World Health Organization’s definition of Health as a complete state of being of good physical, mental, social and spiritual well being and not merely absence of disease or infirmity. www.who.int -constitution. 15. Singh BK. Study of reported sick air passengers passing through Delhi Airport. IJCP 2004;(2):38-42. 16. Personal information from a senior executive of an IATA approved international airlines having fleet of >250 aircrafts. 17. Singh BK. Stress and sickness of air traveller: an Eastern approach to destress. IJCP 2005;(4):38-47.

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clinical study

A Study to Find Out Prevalence of Goiter in School Children of Dahod District, Gujarat NG Padhiyar*, JR Damor**, VS Mazumdar†

Abstract Aim: To find out prevalence of goiter in school children of Dahod district, Gujarat. Study design: Cross-sectional study done in 30 randomly selected wards/villages of Dahod district. Material and methods: The iodine deficiency disorder (IDD) survey at the Dahod district was conducted by population proportionate-to-size (PPS) cluster sampling: A sample of 90 children (45 boys and 45 girls) of age group of 6-12 years from the school and out of school/households of selected village/ward has to be selected in proportion to their school enrolment. Results: The overall goiter rate was found to be 27.6% (Grade 1 + 2). Looking into an age-wise prevalence, it was higher at age 8 (17.4%) and age 12 (17.1%). Highest prevalence was observed in Garbada (33.4%). Lowest prevalence of goiter in Fatehpura. Conclusion: The overall prevalence of goiter Grade I and II was seen more with increase in age. Maximum prevalence was in age 12 years. Grade I goiter was found in majority of goiter cases. Key words: Iodine deficiency disorders, goiter

odine is an essential micronutrient with an average daily requirement of 100-150 µg for normal human growth and mental development. Inadequate or poor intake of iodine can result in physical and mental retardation. It affects people of all ages, both sexes and of different socioeconomic backgrounds. The disorders caused due to deficiency of nutritional iodine in the food or diet are called iodine deficiency disorders (IDDs).1 IDDs are a worldwide major public health problem. Their effects are hidden and profound, affecting quality of human life.2 The goal of National Iodine Deficiency Disorders Control Programme (NIDDCP) was to reduce the prevalence of IDDs to below 10% in endemic districts of the country by the year 2000.3 India has made considerable progress in its IDDCP. Total goiter rate (TGR) of <5% was found in nine out of 15 districts in 11 states by an Indian Council of Medical Research (ICMR) study.4 Starting with Bharuch district in 1982, the state government brought the entire state under IDD *Assistant Professor **Associate Professor †Professor and Head Dept. of Preventive and Social Medicine Medical College, Vadodara, Gujarat Address for correspondence Dr JR Damor D-27, Akanksha Duplex Opposite Laxmikunj Society, Laxmipura Road Gorwa, Vadodara, Gujarat - 390 016 E-mail: jivrajdamor@yahoo.co.in

334

control program in a phased manner by the year 1994. Surveys conducted by the Depts. of Preventive and Social Medicine (PSM) of the Govt. Medical Colleges in the state showed that IDD is still a health problem in several districts of the state. The high prevalence rate was found in Dangs, Bharuch and Valsad districts.5 The present IDD study was conducted by the Dept. of Preventive and Social Medicine, Medical College, Vadodara in Dahod district with financial assistance from State Nutrition Cell, Government of Gujarat. Aims and Objectives 



To find out the prevalence of goiter in school children aged 6-12 years. To find out the prevalence of goiter in the community children aged 6-12 years.

Material and Methods

The IDD survey at the Dahod district was conducted by population proportionate-to-size (PPS) cluster sampling. Selection of villages/wards by PPS was done from list of villages/wards along with the population from the latest census. The data are available for all districts of the country on CD from Registrar General Office. A sample of 30 villages/wards was to be selected from the district. The method of sampling to be used was PPS systematic sampling. Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

clinical study A sample of 90 children (45 boys and 45 girls) of age group of 6-12 years from the school and out of school/ households of selected village/ward were selected in proportion to their school enrolment.

Limitations: As it is a cross-sectional study, children who were not present at their house at the time of study could not be examined. Results

Classification

In 72.4% children, no goiter was found. As age increased the goiter prevalence was higher except in the age group of 9 and 11 years. Nodule was found in two children, each 8 and 12 years of age (Table 1).

To assess the iodine deficiency among school children, following classification was used. Grade 0: Not palpable, not visible Grade 1: Palpable, but not visible Grade 2: Visible and palpable

Overall goiter rate was 27.6% (Grade 1 + 2). As regards age-wise prevalence, it was higher at age 8 (17.4%) and age 12 (17.1%) (Table 2).

Statistical analysis: Using Microsoft Excel 2007 Table 1. Age-wise Overall Prevalence of Goiter in Children Age (years)

Grade of goiter

CI (95%)

Total

Nodule

Present

Absent

331 (76.3%)

86 (19.8%)

17 (3.9%)

19.9-28.1

434

317 (74.4%)

100 (23.5%)

9 (2.1%)

21.6-30.1

426

312 (73.1%)

100 (23.4%)

15 (3.5%)

22.8-31.5

427

425

311 (73%)

102 (23.9%)

13 (3.1%)

22.9-31.5

426

298 (70.4%)

106 (25.1%)

19 (4.5%)

25.3-34.2

423

306 (71.5%)

107 (25%)

15 (3.5%)

24.3-33.1

428

290 (68.4%)

112 (26.4%)

22 (5.2%)

27.2-36.3

424

422

2,165 (72.4%)

713 (23.9%)

110 (3.7%)

26- 29.2

2,988

2,984

Total

Table 2. Prevalence of Goiter according to Age and Gender

Table 3. Taluka-wise Goiter Prevalence with Respect to Gender

AGE (years)

Taluka

Goiter prevalence

Observed children

Boys

Girls

Total

49 (22.5%)

54 (25%)

103 (23.7%)

434

Fatehpura

55 (25.9%)

54 (25.2%)

109 (25.6%)

426

Jhalod

60 (27.9%)

55 (25.9%)

115 (27.0%)

427

61 (28.4%)

54 (25.6%)

115 (27.0%)

426

71 (33.6%)

54 (25.5%)

125 (29.6%)

423

62 (29.1%)

60 (27.9%)

122 (29.6%)

428

63 (29.7%)

71 (33.5%)

134 (31.6%)

424

Total

421 (28.1%)

402 (26.9%)

823 (27.6%)

2,988

Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Goiter prevalence

Total

Boys

Girls

Total

54 (24.1%)

50 (22.3%)

104 (23.3%)

448

81 (30%)

75 (28%)

156 (29.0%)

538

Limkheda

61 (24.7%)

55 (22.1%)

116 (23.4%)

495

Dahod

89 (29.4%)

108 (35.5%)

197 (32.5%)

607

50 (27%)

38 (20.5%)

88 (23.8%)

370

Dhanpur

29 (28.7%)

23 (23%)

52 (25.8%)

201

Garbada

57 (34.3%)

53 (32.5%)

110 (33.4%)

329

421

402

832

2,988

Devgadhbaria

Total

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clinical study Highest prevalence was observed in Garbada (33.4%). Lowest prevalence of goiter was noted in Fatehpura taluka (Table 3). Conclusion  The prevalence of goiter was 22.7% in 1989. In 1998-99, the prevalence was 7.93% when Dahod was a part of Panchmahals district.  The overall prevalence of goiter Grade I and II was seen more with increase in age. Maximum prevalence was in age 12 years. Grade I goiter was found in majority of goiter cases.  The prevalence of goiter in boys (34.3%) was highest in Garbada taluka while, in girls (35.5%) it was in Dahod. But in general, the prevalence was maximum (33.4%) in Garbada. Recommendations 

At all PDS shops, adequate and regular supply of iodized salt should be made available and IEC material and awareness should be made through PDS shops.



Efforts to increase awareness regarding use of iodized salt through school children and mass media are recommended.

References 1. Revised Policy Guidelines on National Iodine Deficiency Disorders Control Programme, IDD and Nutrition Cell, DGHS, Ministry of Health and Family Welfare, Govt. of India, New Delhi, October, 2006. 2. Tiwari BK, Kandu AK, Bansal RD. National Iodine Deficiency Disorders control Programme in India. Indain J Public Health 1995;39:151-6. 3. Mohapatra SSS, Bulliyya G, Kerkertta AS, Acharya AS. Thyroxine and thyrotropin profile in neonates and school children in an iodine deficiency disorders endemic area of Orissa. Indian J Nutr Dietet 2001;38:98-101. 4. Toteja GS, Singh P, Dhilon BS, Saxena BN. Iodine deficiency disorder in 15 districts of India. Indian J Pediatr 2004;71:25-8. 5. State Nutrition Cell, Government of Gujarat Document on Iodine Deficiency Disorders Control Programme Gujarat, 2008.

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Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Case Report

Silent CP Angle Tumor SA Kareem*, Valluvan M**

Abstract Patients with brain tumors usually have symptoms and other related signs. We report a case who had large CPA tumor with not even a single symptom related to brain tumor. We describe in detail how this case was diagnosed by proper ophthalmic examination, along with relevant investigations. Key words: CPA tumor, vestibular schwannoma, epidermoid tumor, papilledema

atients with brain tumors usually have symptoms like headache, vomiting, fits, defective vision, and other related signs, especially when the tumor is situated in places where there are lot of structures such as pituitary and cerebellopontine angle (CPA). We report a case seen in our institution, who had large CPA tumor with not even a single symptom related to brain tumor. Most of the patients with such a tumor will have tinnitus, deafness, vertigo, headache, facial paresis, and >90% will land in head neck department. This patient did not have any of these symptoms. He came to us only for burning sensation of both eyes in the hot summer season. We describe in detail how this case was diagnosed by proper ophthalmic examination, along with relevant investigations. Case Report A male patient aged 40 years presented to eye OPD with complaint of burning sensation in both eyes for past one month. He had come to us four months earlier for a similar complaint. He is working in a bank and he is reading a lot for the competitive CA examination in the leisure time. He thought he was putting his eyes under too much of strain and so he came to us. Routine eye examination showed mild allergic conjunctivitis in both eyes. Vision in both eyes, distance and near, was normal. Anterior segments of eyes, intraocular tension and ocular movements were normal. Fundus exam showed mild papilledema with one dioptre elevation with normal cup in both eyes. *Professor **Senior Resident Dept. of Ophthalmology Sri Balaji Medical College and Hospital Chennai - 600 044

Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Background retina was perfectly normal. Four months earlier, his vision, eye examination and fundi were normal except for the allergic conjunctivitis. Then we conducted a through neuro ophthalmic examination; all his cranial nerves were normal. His blood pressure was normal. We asked many leading questions to assess whether he had any symptoms of brain tumor. He told us that he was enjoying good health and he had none of the symptoms, other than burning sensation of eyes. ENT examination and nervous system exam revealed no abnormal findings. Investigations Routine blood investigations were normal. CT brain showed a large hypotense lesion in the right CPA with mass effect on the brain stem and right cerebellum along with the widening of the right internal auditory canal. MRI with gadolinium was done for better evaluation. The MRI report showed a well-defined heterogeneous mass 41 Ă&#x2014; 37 Ă&#x2014; 35 mm in right CPA cistern with extension into right internal auditory canal causing erosion of the canal, mass effect on the right middle cerebellar peduncle, lower brain stem and effacement of fourth ventricle. Diagnosis: Silent large CPA tumor right-side with mass effect. Management

The case was handed over to the neurosurgeon for further management of the tumor. Discussion Cerebellopontine angle is a space filled with spinal 339

Case Report

Sag contrast

Showing mass effect

Tumor measurement

fluid, bounded on its medial side by the brainstem and posterior surface of the temporal bone as its lateral boundary. The cerebellum forms the roof and posterior boundary. The floor is formed by the lower cranial nerves (IX-XI) and their surrounding arachnoid investments. The VII and VIII cranial nerves cross the CPA to enter the internal auditory canal. CPA tumors, accounting for about 10% of all intracranial tumors, are the most common tumors of the posterior cranial fossa. Most of these tumors are benign and about 85% of them are vestibular schwannomas.

there are lot of important structures in and around it. Besides, it has caused mass effect with erosion and widening of the internal auditory canal. In spite of all these, there are no symptoms. Only few cases of epidermoid tumor are known to be present without symptoms. Brain tumors may have an initial silent period, but, it is very rare that such a large tumor with mass effect, that too in the CPA, presents without any symptom.

Differential Diagnosis

1. Haberkamp TJ, Monsell EM, House WF, Levine SC, Piazza L. Diagnosis and treatment of arachnoid cysts of the posterior fossa. Otolaryngol Head Neck Surg 1990;103(4):610-4.

Commonest     

Vestibular schwannoma (80%) Meningiomas (3-13%) Epidermoids (2-6%) Facial and lower cranial nerve schwannomas (1-2%) Arachnoid cysts (1%)

There are few other less common tumors also like lipomas, dermoid, trigeminal schwannoma and ependymoma. Conclusion Brain tumors produce symptoms and signs due to increased intracranial tension and by brain displacement when they grow larger. There can be an initial silent period, if the tumor happens to be in a free space or an area where there are no structures nearby. But the MRI picture showed a large tumor in the CPA, where

Suggested Reading

2. Langman AW, Jackler RK, Althaus SR. Meningioma of the internal auditory canal. Am J Otol 1990;11 (3):201‑4. 3. Lo WM. Tumors of the temporal bone and cerebellopontine angle. In: Head and Neck Imaging. Som PM, Bergeron RT, (Eds.), St. Louis, Mo: Mosby;1991. 4. McElveen JT, Saunders JE. Tumors of the cerebellopontine angle: neuro-otologic aspects of diagnosis. In: Neurosurgery. Wilkins RH, Rengachary SS, (Eds.), McGraw-Hill; New York: NY; 1996:1038-48. 5. Nadol JB. Cerebellopontine angle tumors. In: Surgery of the Ear and Temporal Bone. Nadol JB, Schuknecht, HF, (Eds.), Raven Press New York: NY; 1993:391-413. 6. Obrador S, Lopez-Zafra JJ. Clinical features of the epidermoids of the basal cisterns of the brain. J Neurol Neu Psych 1969;32(5):450-54.

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Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Case Report

Ruptured Pulmonary Hydatid Cyst: The Camalote Sign Manjot Kaur*, Rakendra Singh**

Abstract Echinococcosis or hydatid disease is caused by larvae of the tapeworm Echinococcus. It remains an important health problem; especially in regions with inadequate hygienic environment and poor veterinarian control. Liver is the most common organ of involvement in adults; lungs are commonly involved in younger people. The cysts are characteristically seen as solitary or multiple circumscribed or oval masses on imaging. The characteristic imaging appearance changes with rupture of cyst. Surgery is the recommended form of treatment. We present a case of ruptured pulmonary hydatid cyst with communicating rupture into the bronchus which is not very common. Key words: Hydatid cyst, rupture, computed tomography

he vast majority of infestations in humans are caused by Echinococcus granulosus. E. granulosus causes cystic echinococcosis, which has a worldwide distribution.1 Humans are exposed less frequently to E. multilocularis, which causes alveolar echinococcosis. The hydatid tapeworm (E. granulosus) requires two hosts to complete its lifecycle. Dogs (and other canines) are the definitive hosts and a variety of species of warm-blooded vertebrates (sheep, cattle, goats, horses, pigs, camels and humans) are the intermediate hosts. Humans are accidental hosts and do not play a role in the biological cycle. As two mammalian species are required for completion of the life cycle, direct transmission of echinococcosis from human to human does not occur. Case Report A 70-year-old male came to the medicine department of our hospital with complaints of dyspnea, productive cough and pain chest following a mild chest trauma. He mentioned that his expectoration was salty in taste. On general examination, patient was hemodynamically stable and had mild respiratory distress. He was afebrile and had no cyanosis or pallor. A previous *Assistant Professor, Dept. of Radiodiagnosis **Assistant Professor, Dept. of Internal Medicine Adesh Institute of Medical Sciences and Research, Bathinda Address for correspondence Dr Manjot Kaur 118/1, Gurjaipal Nagar Jalandhar, Punjab - 144 001 E-mail: drmanjot@hotmail.com

Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

CT scan, multislice scanner, done about a month back showed a well-defined cystic lesion measuring 10.1 Ă&#x2014; 7.2 cm in size in posterior basal segment of left lower lobe (Fig. 1) and suspected diagnosis of intrapulmonary hydatid cyst was given. On presentation at our institute a computed tomography scan was planned. CT scanning of the lungs with CT/e scanner (GE; Milwaukee, USA) using 130 mA, 120 kV, 7-s scan time, 512 Ă&#x2014; 512 matrix and 7 mm section thickness was done. An AP scout film from CT scan showed a well-defined oval cavitating lesion with air-fluid level in the lower zone of left lung abutting the left dome of diaphragm (Fig. 2). On CT axial sections, a large spherical air-filled cavity with collapsed membranes was seen in the posterior basal segment of left lung (Figs. 3 and 4). Surrounding consolidation was seen. Left pleural effusion was noted. Well-defined cysts were also seen in the liver and gastrophrenic ligament on CT (Fig. 5). Diagnosis of findings suggestive of pulmonary hydatid cyst with communicating rupture into bronchus was given. The Casoni and indirect hemagglutination tests were found to be positive. Diagnosis was confirmed at surgery. Discussion Hydatid disease is one of the most important helminthic diseases. The lung is the second most common involved organ, but in children it is the commonest site. The lungs may be affected when the liver is bypassed via the lymphatic system.2 Pulmonary hydatid cyst may rupture into pleural cavity, pericardium or the bronchial tree leading to cough, chest pain and 341

Case Report

Figure 1. Axial CT of chest (mediastinal window) showing well defined cystic lesion in the posterior basal segment of left lung lower lobe.

Figure 2. CT scout film showing a cavitating lesion in the left lower zone abutting the left dome of diaphragm.

hemoptysis. The patients define this as a salty or peppery water expectoration, indicating spring-water expectoration, and it may even be an expectoration of membrane particles.3

Figure 3. Axial CT of chest (lung window) shows spherical cystic lesion (HC) with air-fluid level and surrounding consolidation in the posterior basal segment of left lung lower lobe.

Figure 4. Axial CT of chest (mediastinal window) shows a cystic lung lesion (HC) with dependent wavy contour consistent with germinative membranes (GM). The camalote or water lily sign. Minimal left pleural effusion seen.

The most frequent complication of pulmonary hydatid disease is the rupture of the cyst into a bronchus, which is also regarded as a complicated cyst.4 The rupture of echinococcal cysts is of three types: Contained, communicating and direct. ď Ź

ď Ź

342

Contained rupture occurs when only the parasitic endocyst ruptures and the cyst contents are confined within the host-derived pericyst. When cyst contents escape bronchial or biliary radicles that are incorporated in the pericyst, the rupture is communicating.

Figure 5. Axial CT of upper abdomen showing a well-defined cystic lesion in the gastrophrenic ligament proven to be hydatid cyst at surgery.

Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Case Report 

Direct rupture occurs when both the endocyst and the pericyst tear, spilling cyst contents directly into the peritoneal or pleural cavities or occasionally into other structures.

Communicating and direct forms have more serious clinical implications than contained rupture, but even contained rupture should have prompt surgical attention to prevent it from developing into one of the other forms.5 In our case, a cavity with air-fluid level and collapsed germinative layers consistent with direct rupture of hydatid cyst into left lower bronchus was seen. Surrounding consolidation and mild pleural effusion was likely due to inflammatory response to endobronchial spread of cyst contents as no larvae/membranes could be elucidated in the pleural fluid. Conventional chest radiography has been the mainstay investigation for diagnosis of hydatid disease of the lungs, often coupled with Casoni’s skin testing and serologic testing for antibodies. A variety of descriptive terms have been given to plain film findings if rupture of cyst occurs with the air around or within the endocyst, an air-fluid level and the collapsed, crumpled membranes floating in the fluid. The ‘camalote or water lily’ sign is described when endocyst membrane float on top of remaining fluid due on collapse of endocyst and partial evacuation of fluid. This was demonstrated in our case. The other signs are the meniscus/crescent’ sign, the sign of the ‘rising sun’, the ‘serpent’ sign, the ‘whirl’ sign, the ‘onion peel’ sign and the ‘cumbo’ sign.6-8 Sometimes, after rupture of a cyst into the bronchus, an inflammatory reaction may close the draining bronchus and imprison the membrane (incarcerated membrane). In these cases the definite regular outline is lost and there may be a blurred shadow that can easily be mistaken for tuberculous focus or carcinoma (Ivanissevich sign).3 CT scan is useful to confirm the diagnosis and it is also able to demonstrate cysts not identified with plain radiographs.8 Apart from the classically described

features of pulmonary hydatid disease, a crescentshaped rim of air at the lower end of the cyst (inverse crescent sign) and a bleb of air in the wall of as-yet unruptured cysts (signet ring sign) have also been described on CT scan.9 Conclusion CT helps to elucidate the cystic nature of the lung mass, its accurate localization and evidence of any form of rupture so that surgical treatment can be planned at the first opportunity to avoid potentially fatal complications. References 1. Kurt Y, Sücüllü I, Filiz AI, Urhan M, Akin ML. Pulmonary echinococcosis mimicking multiple lung metastasis of breast cancer: the role of fluoro-deoxyglucose positron emission tomography. World J Surg Oncol 2008;6:7. 2. Rebhandl W, Turnbull J, Felberbauer FX, Tasci E, Puig S, Auer H, et al. Pulmonary echinococcosis (hydatidosis) in children: results of surgical treatment. Pediatr Pulmonol 1999;27:336-40. 3. Xanthakis D, Efthimiadis M, Papadakis G, Primikirios N, Chassapakis G, Roussaki A, et al. Hydatid disease of the chest. Thorax 1972;27:517-28. 4. Dogan R, Yuksel M, Cetin G, Suzer K, Alp M, Kaya S, et al. Surgical treatment of hydatid cysts of the lung: report on 1055 patients. Thorax 1989;44:192-9. 5. Lewall DB, McCorkell SJ. Rupture of echinococcal cysts: diagnosis, classification, and clinical implications. Am J Roentgenol 1986;146:391-4. 6. Pedrosa I, Saíz A, Arrazola J, Ferreirós J, Pedrosa CS. Hydatid disease: radiologic and pathologic features and complications. Radiographics 2000;20:795-817. 7. Haliloglu M, Saatci I, Akhan O, Ozmen MN, Besim A. Spectrum of imaging findings in pediatric hydatid disease. Am J Roentgenol 1997;169:1627-31. 8. Celik M, Senol C, Keles M, Halezeroglu S, Urek S, Haciibrahimoglu G, et al. Surgical treatment of pulmonary hydatid disease in children: report of 122 cases. J Pediatr Surg 2000;35:1710-13. 9. Koul PA, Koul AN, Wahid A, Mir FA. CT in pulmonary hydatid disease: unusual appearances. Chest 2000; 118:1645-7.

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Case Report

Infection Associated Hemophagocytic Lymphohistiocytosis Rajani Dube*, Subhranshu Sekhar Kar**, Samarendra Mahapatra**, Rajib Rayâ&#x20AC;

Abstract The term hemophagocytosis describes the pathologic finding of activated macrophages, engulfing erythrocytes, leukocytes, platelets and their precursor cells. This phenomenon is an important finding in patients with hemophagocytic syndrome, more properly referred to as hemophagocytic lymphohistiocytosis (HLH). HLH is a distinct clinical entity characterized by fever, pancytopenia, splenomegaly and hemophagocytosis in bone marrow, liver or lymph nodes. It has been associated with a variety of viral, bacterial, fungal and parasitic infections, as well as collagen vascular diseases and malignancies and is uniformly fatal if left untreated. We report Staphylococcus aureus-induced hemophagocytic lymphohistiocytosis in a 3-month-old girl presenting with respiratory distress, sepsis and multiorgan failure. This case report may at least in part guide pediatricians and other physicians to recognize this rare entity of infection triggering fatal HLH and thus proper treatment may be instituted in those affected with this disease at the earliest. Key words: Hemophagocyte, infection, histiocyte

he term hemophagocytosis describes the pathologic finding of activated macrophages, engulfing erythrocytes, leukocytes, platelets and their precursor cells.1 It is a serious and potentially lifethreatening histiocytic disorder in children and adults. A hallmark of hemophagocytic lymphohistiocytosis (HLH) is impaired or absent function of natural killer (NK) cells and cytotoxic T cells (CTL).2,3 In its most severe form, it leads to a sepsis-like picture and multiorgan failure (MOF). We report the case of a 3-month-old female child who presented with very severe pneumonia (due to Staphylococcus aureus) resulting in HLH with acute respiratory distress syndrome (ARDS) and MOF. This case report may at least in part guide pediatricians to recognize this rare entity of infection triggering fatal HLH.

*Assistant Professor, Dept. of Obstetrics and Gynecology **Associate Professor â&#x20AC; Assistant Professor, Dept. of Pediatrics Hi-Tech Medical College, Pandara, Bhubaneswar Address for correspondence Dr Subhranshu Sekhar Kar Qrs No.-8/II, Hi-Tech Medical College Campus Pandara, Bhubaneswar, Orissa - 751 010 E-mail: drsskar@yahoo.co.in, drsskar@gmail.com

344

Case Report A 3-month-old girl child was referred to our institute with the history of fever, rash, abdominal distention and refusal to feed of three weeks duration. She had been having progressive dyspnea, abdominal distension and low grade fever since she was two months of age. Investigations done in a local hospital were noncontributory and she was put on bronchodilators and antibiotics with the diagnosis of sepsis. She improved marginally with the medications. Three weeks back her symptoms worsened and she developed fever and rash. She was then referred to our hospital for further management. On examination, she was conscious, febrile (39oC) and tachypneic. She was having generalized edema, conjunctival congestion, ecchymosis and icterus. She was not cyanosed. Pulse was 162/min with NIBP of 80/50 mmHg. She had splenomegaly and free fluid in the abdomen. There were fine crepitations involving the left lung base. Heart sounds were normal without any murmur. There were no signs of meningeal irritation, no focal neurological deficits and normal fundus. Urine examination showed trace protein and a few pus cells. Blood examination revealed Hb of 8.0 g/dl; TLC of 2,500/cm3; DLC P35L60E5, ESR of Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Case Report 18 mm/1st hour. She had thrombocyto-penia, with platelet count 37,000/cm3. Peripheral smear report showed microcytic hypochromic anemia, anisopoikilocytosis and polychromatic cells, shift to left with toxic granules, decreased platelet count and no malarial parasite. ECG showed sinus tachycardia. Chest X-ray revealed alveolar opacities involving left lower zone. ABG showed hypoxemia. Liver function tests: Bilirubin 7.2 mg/dl, total protein 4.3 mg/dl, albumin 2 mg/dl, SGOT/SGPT 324/156, SAP 204. Renal functions tests: blood urea 60 mg/dl and creatinine 1 mg/dl. RBS was 116 mg/dl; there was hypocalcemia with calcium of 7 mg/dl and phosphate 3.8 mg/dl. Serial monitoring of INR showed values of 1.75, 1.9, and 2.5, respectively. Antinuclear antibody, viral markers for human immunodeficiency virus (HIV), hepatitis A, B, C and E, serology for Weilâ&#x20AC;&#x2122;s and dengue, Widal test, rapid malarial test, Mantoux test, gastric lavage for AFB were negative. Thyroid profile was normal. Echo showed no evidence of infective endocarditis and normal systolic function. Blood culture and sensitivity isolated S. aureus species. Urine culture was sterile. Sonogram of abdomen revealed splenomegaly, ascites and right pleural effusion. Serum ferritin was elevated [1,250 ng/ml; (0-150 ng/ml)]. With the diagnosis of staphylococcal pneumonia with multiorgan dysfunction she was shifted to the pediatric intensive care unit where she was started on extended spectrum penicillins and ceftazidime alongwith blood products for coagulopathy. She had already received a course of antibiotics from the local hospital from which she was referred. With the course of time there was no clinical improvement. So a bone marrow study was done, which showed increased number of histiocytes with hemophagocytosis. The clinical scenario was very much suggestive of hemophagocytic lymphohistiocytosis (macrophage activation syndrome secondary to staphylococcal pneumonia and sepsis) with fever, rash, splenomegaly, pancytopenia and hemophagocytosis. She was put on ventilatory support and given a course of intravenous immunoglobulin, in addition to the antibiotics and steroids. In spite of our efforts, she succumbed on the sixth post admission day. Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Figure 1. Showing histiocytes (engulfing nuclei of normoblasts).

Discussion Hemophagocytic lymphohistiocytosis (HLH) is a rare but potentially fatal disease of normal but overactive histiocytes and lymphocytes that commonly appears in infancy, although it has been seen in all age groups. It is of two types - primary HLH4 and secondary HLH (acquired HLH), which occurs after strong immunologic activation by systemic infection (virus, bacteria and protozoa), autoimmune disorders or underlying malignancy. There is overwhelming activation of normal T cells and macrophages which can cause clinical and hematological alterations. The pathological hallmark of this disease is the aggressive proliferation of activated macrophages and histiocytes, which phagocytose other cells, namely RBCs, WBCs and platelets, leading to the clinical symptoms. The uncontrolled growth is nonmalignant and does not appear clonal in contrast to the lineage of cells in Langerhans cells histiocytosis (histiocytosis X). The spleen, lymph nodes, bone marrow, liver, skin and membranes that surround the brain and spinal cord are preferential sites of involvement.5 A current accepted theory involves an inappropriate immune reaction caused by proliferating and activated T cells associated with macrophage activation and inadequate apoptosis of immunogenic cells.6 Although the precise mechanism remains unclear, many research teams propose convincing pictures for the role of perforin and NK cells in the HLH subtypes.7-9 345

Case Report The clinical presentation is in many aspects similar to the so-called systemic inflammation response syndrome (SIRS). Death is inevitable in the absence of treatment. The clinical entity has to be suspected when patients present with fever unresponsive to antibiotics, general fatigue, falling ESR, pancytopenia of unknown origin and liver dysfunction with elevated ferritin. The diagnostic criteria are as follows.2  Familial disease/known genetic defect  Clinical and laboratory criteria (5/8 criteria) n Fever n Splenomegaly n Cytopenia ≥2 cell lines n Hemoglobin <90 g/l (below four weeks <120 g/l) n Neutrophils <1 × 109/l n Hypertriglyceridemia and/or hypofibrinogenemia fasting triglycerides ≥3 mmol/l n Fibrinogen <1.5 g/l n Ferritin ≥500 μg/l n sCD25 ≥2,400 U/ml n Decreased or absent NK-cell activity n Hemophagocytosis in bone marrow, CSF or lymph nodes Supportive evidence are cerebral symptoms with moderate pleocytosis and/or elevated protein, elevated transaminases and bilirubin, LDH >1,000 U/l. For confirmation, tissue diagnosis is needed. Hemophagocytosis must be demonstrated in the bone marrow, spleen or lymph nodes. In our case, almost all criteria were present. The newest treatment protocol, HLH-2004, is based on the Histiocyte Society’s original HLH-94 protocol, with some minor modifications. It represents a consolidation of the various approaches to treatment, with the goals being to first achieve clinical stability and then to cure with bone marrow transplantation (BMT). Antimycotic prophylaxis is used during the initial doses of dexamethasone. Sulfamethoxazole and trimethoprim (i.e. cotrimoxazole) is continuously administered as prophylaxis for Pneumocystis carinii because of immune suppression.10 346

One group found that intravenous immunoglobulin (IVIG) was effective in suppressing symptoms when administered within hours of disease onset. Serum ferritin was used as a marker for macrophage activation, and treatment was administered accordingly.11 Patients may be classified into high-risk and low-risk groups, with only the high-risk groups receiving the etoposide (i.e. VP-16) regimens. Patients who are at low risk may be treated as effectively with only cyclosporine, corticosteroids or IVIG.12 In the absence of prospective controlled trials, corticosteroids, cyclosporin A and etoposide are administered with varied success. Recent case reports show promising results with an anti-TNF-α approach and plasmapheresis. Supportive care is needed to ensure that the patient with HLH remains stable until a bone marrow donor can be found. This includes transfusions of RBCs, platelets, and fresh frozen plasma, as well as nutritional support in addition to the treatment protocol.10 This case is presented to enlighten pediatricians and other physicians regarding the clinical entity of hemophagocytic lymphohistiocytosis to be kept in mind, when patients present with fever unresponsive to antibiotics, organomegaly, pancytopenia of unknown origin and liver dysfunction with elevated ferritin. References 1. Favara B. Hemophagocytic lymphohistiocytosis: a hemophagocytic syndrome. Semin Diagn Pathol 1992;9:63-74. 2. Janka GE, Schneider EM. Modern management of children with haemophagocytic lymphohistiocytosis. Br J Haematol 2004;124:4-14. 3. Janka G, Imashuku S, Elinder G, Schneider M, Henter JI. Infection- and malignancy-associated hemophagocytic syndromes. Secondary hemophagocytic lymphohistiocytosis. Hematol Oncol Clin North Am 1998;12:435-44. 4. Farquhar JW, Claireaux AE. Familial haemophagocytic reticulosis. Arch Dis Child 1952;27:519-25. 5. Arico M, Allen M, Brusa S, et al. Haemophagocytic lymphohistiocytosis: proposal of a diagnostic algorithm based on perforin expression. Br J Haematol 2002;119(1):180-8. 6. Imashuku S, Ueda I, Teramura T, et al. Occurrence of haemophagocytic lymphohistiocytosis at less than 1 year of age: analysis of 96 patients. Eur J Pediatr 2005;164(5):315-9.

Cont’d on page 352...

Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Hospital Infrastructure India 2010 (December 7-9, 2010, Bombay Exhibition Centre, Mumbai)

Start to End Solutions for Building or Upgrading Hospitals of the Future All Under One Roof

he first of its kind exhibition and conference on hospital infrastructure in India to be held from India and abroad from 7-9 December 2010 at Bombay Exhibition Centre, Mumbai will feature the latest technology and solutions in hospital infrastructure, medical equipment, healthcare architecture, hospital planning, and supplies from India and abroad. Some of the world’s biggest suppliers will be on hand to demonstrate their newest products and services at Hospital Infrastructure India (HII) 2010. Exhibitors include names like Larsen and Toubro (L&T), Sony, Armstrong, Draeger, Forbo Flooring, HKS, Everest Industries, GMP Technical, Promark Associates, HLL Lifecare, and so on. There will be special features like product demonstrations, workshops, conferences, and seminar forums for the visitors at HII 2010. Health missions have been organized by United Kingdom Trade & Investment (UKTI) and Australian Trade Promotion (Austrade) to coincide with the show. Private hospital professionals, hospital entrepreneurs, government hospitals, healthcare planners, engineers, architects, project management consultants, and senior officials from state and central health departments will assemble to network and make valuable new business contacts. Hospital Infrastructure India conference A world class technical conference is being organized, in association with HOSMAC, for industry professionals to benefit from the experience of the renowned industry leaders from across the world. There would be modular sessions and the major topics would include healthcare design/build, healthcare infrastructure, planning, public private partnerships (PPP), and interactive panel discussions. Some of the eminent industry experts have been invited to speak at the conference. The HII conference would be an ideal platform for sharing knowledge, exchanging ideas, and building profitable networks. Technology Preview at HII 2010 Featured below are some of the exhibitors with the technology on display at Hospital Infrastructure India 2010. Healthcare planning and designing solutions from TAHPI. An Australia-based specialist healthcare planning and design firm, will showcase their solutions for the Indian market at HII 2010. They provide the full spectrum of services from health service planning to briefing, architectural and interior design, equipment, and commissioning. TAHPI offers the popular web-

based toolkit known as the Health Facility Briefing System (HFBS) and training. Healthcare designing from HKS. HKS will take the opportunity of participating at HII to showcase their achievements in successfully integrating the internationally adopted best practices in healthcare design in the regional context of India. They see this opportunity as a great platform to share their extensive experience in designing world class healthcare facilities. Wall protection material from InPro Corporation. InPro Corporation is a premier manufacturer of architectural products in the United States. Their product range includes door and wall protection, expansion joint systems, privacy curtain tracks, and way-finding signage. Among InPro’s top products are hand rails, wall guards, corner guards, and anti-bacterial fabrics. They will be introducing G2, the world’s greenest wall protection material. Floor covering and disinfecting surface solutions from Graboplast. Graboplast’s newest development, an active disinfecting surface solution to the hospitals, will be displayed at HII 2010. Grabo Silver Knights due to the double defense line build up from nanosilver and nanoTi02 particles eliminate more than 99 percent of bacteria. Total Spectrum Air Purification System by Promark. Promark has introduced innovative technology in particulate, chemical, and biological filtration, and climate control. Their groundbreaking Total Spectrum Air Purification System, winner of a number of prestigious design awards, will be on display at HII 2010. Designing and manufacturing of floors and ceilings by Armstrong World. A range of resilient flooring and acoustical ceilings for hospital and healthcare sector will be displayed by Armstrong at HII 2010. Smart Networks International (SNI) to participate along with a consortium of seven European companies. SNI will showcase technologically advanced, scientifically tested, globally approved and accepted products, technology, and services in the field of wellness and healthcare at HII 2010 including wellness and fitness equipment/system modules for the age group 2-80 years; preventive diagnosis and structured life style monitoring; and endoskopia for spinal surgeries and back pains of typical/critical nature. For free fast track entrance to the exhibition, please register at www.hospitalinfra-india.com/register or call at 91-22-4020 3344.

Case Report

Branchial Cyst with a Primary Carcinoma Thyroid Samarth Shukla*, Sourya Acharya**, Sunita Vagha†, Sobha Grover‡, Devendra Rajput¶

Abstract The thyroid gland is derivative of the branchial pouches and so is the branchial cleft cysts. Considering the common embryological inheritance of both the anatomic structures they also inherit a common set of pathology within them. Cystic swellings in the neck, most of times, is a lymph node pathology, the considerations in terms of differentials for a lateral neck swelling involving the Wolfler’s area has to be given its due accord, which obviously includes the thyroglossal cyst, branchial cleft cyst, ectopic thyroid structure. This case is particularly interesting considering the brevity of de novo thyroid pathologies arising in a branchial cyst. Key words: Thyroid gland, branchial pouch, cyst, thyroglossal

he branchial cysts can arise from any of the branchial arches. These are congenital epithelial cysts, formed from a failure of obliteration commonly of the second branchial cleft in embryonic development.1 Branchial cysts are relatively uncommon anomalies of the soft tissues of the neck, typically found in the angle between the sternocleidomastoid muscle and the mandible. The origin of the cysts is uncertain. In spite of their putative embryological origin, they present most commonly in the second and third decades.2,3 Cystic lesions are not uncommon in the neck region and their pathology in the neck can be extremely dubious and deceptive many a times. Considering the anatomic location (laterally on the neck) of the cystic swelling, the differentials to be made up in case of a young adult with a lateral neck cyst can range from an extremely trivial diagnosis of simple cyst (inclusion) to cervical lymph nodes pathology and or branchial cyst. The cystic swellings can be particularly ominous in the sense that they present as malignancies, either metastatic deposits from a primary elsewhere or still dubious from an occult primary, the rarest being a malignant transformation in a pre-existing ectopic rest. *Associate Professor, Dept. of Pathology **Associate Professor, Dept. of Medicine †Professor and Head ‡Emeritus Professor, Dept. of Pathology JN Medical College, DMIMS University, Wardha, Maharashtra ¶Assistant Professor, Dept. of Medicine, LNMC, Bhopal

Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Case Report A 25-year-old female patient presented with a cyst in neck in the surgery OPD of our hospital. The swelling was of five-month duration, On further evaluation, the patient revealed that the swelling initially was the size of a pea and gradually increased to attain the present size. On physical examination, the swelling was 4 × 4 cm in size, painless, nontender, cystic in consistency. It was located on the left lateral side of the neck below the sternocleidomastoid muscle. The skin above was not adherent to the swelling, which was free from the underlying structures as well. No secondary changes were evident on the skin. The regional groups of lymph node (cervical) was unremarkable. Systemic examination along with the oral cavity was normal. The patient was advised FNA biopsy from the cyst and it revealed colloid-like material mixed with blood clots and nonspecific chronic inflammatory infiltrate (lymphocytes, plasma cells, macrophages, along with reactive lymphoid tissue, few atypical epithelial cells) were seen. The cytology was labeled as suggestive of inflammatory cystic lesion. However, CT was advised taking in view the atypical epithelial cells to rule out secondaries. CT revealed a hypodense cystic lesion size of 40 mm in diameter lying in the anterior cervical triangle surrounded with fibrofatty pad, intracystic septations, few hyperdense solid areas within the cyst; neither cervical, intrathyroid 349

Case Report

Figure 1.

Figure 2.

nodes nor pharyngeal or laryngeal nodes or masses were observed. The CT report was suspicious of neoplastic character of the lesion (metastatic) and an excision biopsy was carried out by means of lateral cervicotomy. The excised cyst on cut section show brownish hemorrhagic fluid, with intervening septae and necrotic solid areas; microscopically the tissue revealed pseudostratified columnar epithelial lining with partial capsule formation with fibrofatty connective tissue (Fig. 1), intracystic hemorrhagic areas along with plenty of scattered lymphoid tissue rests were present. A two places, there are areas of papillary formations lined by cuboidal cells and well-developed fibrovascular core (Fig. 2). The cuboidal cells have vesicular (orphan annie-eyed nucleus) with nuclear grooving, along with psammoma body formations, features are suggestive of papillary carcinoma of thyroid (Metastatic eposists within a branchial cyst) (Fig. 3). 350

Figure 3.

However, when extensive thyroid scans by CT and scintigraphy scans were carried out, no evidence of the primary thyroid glandular pathology was evident. Such a finding is but expected as at times CT and MRI besides scintigraphy may fail to rule out the primary pathology within the thyroid gland, total thyroidectomy was advised to zero down on the primary lesion. The thyroid with both lobes and isthmus was removed as an operative procedure and thoroughly grossed in the surgical histopathology laboratory, the thyroid was sectioned and all its areas where completely embedded. However, no grossly suspicious areas in thyroid were observed during grossing the thyroid. The patient was advised a monthly follow up for a year with complete excision of the branchial cystic swelling. Discussion Heterotropic thyroid tissue can be found not only as a component of thyroglossal duct cyst but anywhere along the thyroglossal tract, including sites like anterior tongue, submandibular region, larynx, trachea, mediastinum and heart. But the common denominator in all the sites is the fact that the ectopic thyroid tissue and there pathologies can arise anywhere in the Wolflerâ&#x20AC;&#x2122;s area, described by the anatomists as the isosceles triangle with base formed by the edge of the mandible and the apex by the concavity of the aorta.4,5 Thus it can be clearly stated that the embryology of the thyroid gland as well as the branchial cleft cyst having crossed pathways in their development, becomes particularly significant. At the fourth week of embryonic life, the development of 4 branchial (or pharyngeal) clefts results in 5 ridges Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Case Report known as the branchial (or pharyngeal) arches, which contribute to the formation of various structures of the head, the neck, and the thorax. If a portion of the cleft fails to involute completely, the entrapped remnant forms an epithelium-lined cyst with or without a sinus tract to the overlying skin.6,7 Branchial cleft cysts are the most common congenital cause of a neck mass. An estimated 2-3% of cases are bilateral. A tendency exists for cases to cluster in families. The human thyroid gland derives mainly from one median anlage, and the lateral anlages of two fourth branchial pouches which share the development the gland. From the last, two superior parathyroid glands and the lateral thyroid are derived. A number of anomalies may develop either from the gland or from parts of it during this process. These ectopic tissues may develop the same diseases as the thyroid gland.8 The embryological development becomes particularly significant, as both the thyroid gland as well as the branchial cleft cysts are derived from the branchial arches, the pathologies arising in the branchial cyst though rarely can be due to an ectopic rest of thyroid tissue present within the branchial cyst (common embryological development). Clinically a branchial cyst commonly presents as a solitary, painless mass in the neck of a child or a young adult. Branchial cysts are smooth, nontender, fluctuant masses, which occur along the lower one-third of the anteromedial border of the sternocleidomastoid muscle between the muscle and the overlying skin. The important differentials which have to be kept into consideration as the tentative diagnosis includes Lymphadenopathy (reactive, neoplastic, lymphoma, metastasis), vascular neoplasms and malformations, capillary hemangioma, carotid body tumor, lymphatic malformation (cystic hygroma), ectopic thyroid tissue, ectopic salivary tissue. But there is no question considering the overwhelming majority of the cystic lesions in the lateral neck containing recognizable foci of papillary carcinoma of thyroid represent cervical lymph node which has undergone secondary cystic changes, unless proved otherwise. But cases do exist, albeit rare, where tumors like papillary carcinoma of thyroid have arisen from the ectopic rest of thyroid tissue in the branchial cyst.9 Previous reports showed that ectopic thyroid tissue may present metastasis from thyroid carcinoma Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

and very rarely it may harbor a primary thyroid carcinoma. Of the latter, about 100 cases have been so far described in literature. Most of them have been shown to occur in the thyroglossal duct, 1% out of all thyroglossal cysts carcinomas are papillary carcinomas. Only four cases of primary thyroid carcinomas arising in neck branchial cyst have been described by Balasubramaniam et al,10 Jadusingh et al11 Matsumoto et al.12 Traditionally, all the foci of malignant thyroid tissue in the neck were metastatic foci from primary thyroid lesions. However, new theories hypothesize that ectopic thyroid tissue may be present associated with a branchial cyst. There are complex situations wherein besides the cervical nodes, it is the branchial cleft cyst which become the epicenter of such pathologies. Here in come most of the controversial debates pertaining to the common embryogenesis of the thyroid and the branchial pouches (4th and the 5th). There are often two evident case scenarios which exist, the first states that the metastasis from the malignant foci in the thyroid gland deposits in the branchial cleft cyst and undergoes cystic degeneration in that cyst, in such a scenario it should be rather evident to find or search for the primary malignant foci in the thyroid gland. But in case of occult primary (foci <1.5 cm within the gland), a thorough search should be carried out in terms of rigorous grossing of the thyroid gland. The second theory suggests that in a situation wherein no primary can be identified in the thyroid gland, it is suggested that an ectopic rest is already present in a such a branchial cyst and has undergone a malignant transformation. A less rarer scenario in the second theory is that papillary thyroid carcinoma in lateral neck cysts has been established in relation to benign metastases to cervical lymph nodes and or branchial cleft cyst from the thyroid gland that undergoes ulterior malignization. Conclusion Considering the above mentioned theories, the concerned endocrinologist, the operating surgeon as well as the histopathologist should keep in mind that the absence of primary tumour within the whole thyroid gland despite extensive histological examination (millimetre in size) may favor the latter theory, although it has been traditionally assumed that misdiagnosis in the histological study may be present. The presence of one or more foci of papillary carcinoma within 351

Case Report the thyroid gland supports the first diagnostic option, such clinical scenarios may pose a difficult challenge in management and prognostification of the clinical subject. The management in such situations should be carried pertaining to the combined net result of all special investigations as well as the clinical findings of the patient. References 1. Wagner AM, Hansen RC. Neonatal skin and skin disorders. In: Pediatric Dermatology. Vol 1. 2nd 2nd editions, Schachner LA, Hansen RC, (Eds.), Churchill Livingston; New York: NY; 1995:291-3. 2. Bhaskar SM, Bernier JL. Histogenesis of branchial cysts. A report of 468 cases. Am J Pathol 1959;35:407-23. 3. Agaton-Borilla FC, Gay-Escoda C. Diagnosis and treatment of branchial cleft cysts and fistulae. A retrospective study of 183 patients. Int J Oral Maxillofac Surg 1996;25:449-52. 4. Kantelip B, Lusson JR, DeRiberolles C, Lamaison D, Bailly P. Intracardiac ectopic thyroid. Human Pathol 1986;17:1293-6.

5. Osammor JY, Bulman CH, Blewitt RW. Intralaryngotracheal thyroid. J Laryngol Otol 1990;104:733-6. 6. Doi O, Hutson JM, Myers NA, McKelvie PA. Branchial remnants: a review of 58 cases. J Pediatr Surg 1988;23(9):789-92. 7. Little JW, Rickles NH. The histogenesis of the branchial cyst. Am J Pathol 1967;50(3):533-47. 8. De Felice M, Di Lauro R. Thyroid development and its disorders: genetics and molecular mechanisms. Endocr Rev 2004;25:722-46. 9. Matsumoto K, Watanabe Y, Asano G. Thyroid papillary carcinoma arising in ectopic thyroid tissue within a branchial cleft cyst. Pathol Int 1999;49:444-6. 10. Balasubramaniam GS, Stillwell RG, Kennedy JT. Papillary carcinoma arising in ectopic thyroid tissue within a branchial cyst. Pathology 1992;24:214-6. 11. Jadusingh W, Shah DJ, Shaw H, Lyn C. Thyroid papillary carcinoma arising in a branchial cleft cyst. West Indian Med 1996;45:122-4. 12. Matsumoto K, Watanabe Y, Asano G. Thyroid papillary carcinoma arising in ectopic thyroid tissue within a branchial cleft cyst. Pathol Intern 1999;49:444-6.

...Contâ&#x20AC;&#x2122;d from page 346 7. Risma KA, Frayer RW, Filipovich AH, Sumegi J. Aberrant maturation of mutant perforin underlies the clinical diversity of hemophagocytic lymphohistiocytosis. J Clin Invest 2006;116(1):182-92. 8. Katano H, Cohen JI. Perforin and lymphohistiocytic proliferative disorders. Br J Haematol 2005;128(6): 739-50. 9. Rieux-Laucat F, Le Deist F, De Saint Basile G. Autoimmune lymphoproliferative syndrome and perforin. N Engl J Med 2005;352(3):6.

10. Henter JI, Samuelsson-Horne A, Arico M, et al. Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation. Blood 2002;100(7):2367-73. 11. Emmenegger U, Spaeth PJ, Neftel KA. Intravenous immunoglobulin for hemophagocytic lymphohistiocytosis?. J Clin Oncol 2002;20(2): 599â&#x20AC;&#x2018;601. 12. Imashuku S, Teramura T, Morimoto A, Hibi S. Recent developments in the management of haemophagocytic lymphohistiocytosis. Expert Opin Pharmacother 2001;2(9):1437-48.

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Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Emedinews Section

From the eMedinewS

Extending Zoledronic Acid Regimen Maintains Bone Density

Legius Syndrome may be Incorrectly Identified as Neurofibromatosis Type 1

Findings from a study of more than 1,000 patients show that those who continued annual treatment with zoledronic acid for six years had significantly better bone mineral density and fewer morphometric vertebral fractures as compared to patients who received treatment for three years and then stopped taking the drug. The treatment was also safe. The study was presented by Dennis M. Black, Ph.D., professor of epidemiology and biostatistics at the University of California, San Francisco at the annual meeting of the American Society for Bone and Mineral Research.

Legius syndrome can be easily misdiagnosed as neurofibromatosis type 1 (NF1). First described only three years ago, by Dr Eric Legius and co-workers at the Catholic University of Leuven (Belgium), the hallmarks of Legius syndrome include multiple café au lait macules, axillary freckling and autosomal dominant transmission, all of which are also among the NF1 diagnostic criteria established by the National Institutes of Health. However, bone lesions, plexiform or cutaneous neurofibromas, Lisch nodules in the iris, and nervous system tumors, which are the other typical lesions of NF1 are not found in patients with Legius syndrome. Legius syndrome, unlike NF1, does not carry an increased cancer risk. (Dr. Sirkku Peltonen at Annual Congress of the European Academy of Dermatology and Venereology).

ACIP Adds Meningococcal Vaccine Booster Dose at Age 16 Years A booster dose of meningococcal conjugate vaccine should be given to adolescents at 16 years old if they received a first dose at age 11-12 years, and a booster should be given five years after the first dose - upto age 21 years - to those who first received the vaccine at age 13-15 years. That was the vote of the Advisory Committee on Immunization Practices (ACIP) of the Centers for Disease Control and Prevention on Oct. 27th, but it was not unanimous. The panel was split 6 to 5, with 3 abstentions. Following that vote, ACIP also voted to include the booster dose under the federal Vaccines for Children program. The CDC usually adopts the ACIP’s recommendations but is not obligated to do so. In 2007, quadrivalent meningococcal conjugate vaccine (MCV4), was recommended for 11-12-yearolds at the established preteen visit, and to 13-18year-olds who had not been previously vaccinated. Recent data however suggest that immunity from the vaccine wanes within five years after vaccination, thereby possibly failing to protect those at highest risk, particularly college students living in dorms, according to Dr Amanda Cohn of the CDC’s National Center for Immunization and Respiratory Diseases (NCIRD). Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Elderly More Likely to Die From H1N1 Influenza According to the results of a retrospective analysis, mortality rates from H1N1 influenza show a J-shaped curve, with those aged 70 years and older having the greatest risk for death. The study is published online November 12 in The Lancet. Spouses who Care for Partners with Dementia at 6-fold Higher Risk of Same Fate Husbands or wives who care for spouses with dementia are six times more likely to develop the memoryimpairing condition than those whose spouses don’t have it, according to results of a 12-year study led by Johns Hopkins, Utah State University, and Duke University. The increased risk that the researchers saw among caregivers was on par with the power of a gene variant known to increase susceptibility to Alzheimer’s disease. The study is reported in the Journal of the American Geriatrics Society. 355

emedinews Section No Hard Evidence Supports Supplements’, Vitamins’ Promise in Slowing Cognitive Decline A seemingly steady stream of new research purports to show supplements’ and vitamins’ promise in preventing or slowing cognitive decline, but in reality no hard evidence supports taking any of them. The article discussed claims regarding omega-3 fatty acid fish oils, gingko biloba and vitamin E for preserving cognition, pointing out that studies have found inconclusive or mixed benefits for each of these supplements. FDA Approves ‘Mini’ Dose Oral Contraceptive The FDA has approved an oral contraceptive (Lo Loestrin Fe) that reduces estrogen exposure but maintains efficacy. It contains estrogen 10 µg, which is the lowest estrogen dose approved by the FDA as an oral contraceptive. But despite the low-dose, the drug is contraindicated in patients with a high risk of arterial or venous thrombotic diseases, present or past breast or other estrogen- or progestin-sensitive cancer, liver tumors or liver disease, abnormal uterine bleeding and pregnancy, The new pill was developed by Warner Chilcott. Tool Predicts Postop Problems from Sleep Apnea A simple, 8-item questionnaire may be an effective way to identify patients at risk for postoperative complications because of hidden obstructive sleep apnea syndrome. Patients with high scores on the STOP-BANG questionnaire were significantly more likely to have postoperative complications than those with low scores (19.6% vs 1.3%, p < 0.001), according to Tajender Vasu, MD, of Thomas Jefferson University Hospital in Philadelphia, and colleagues. Certolizumab Heals Crohn’s Ulcers Findings of healing with certolizumab pegol (Cimzia) in Crohn’s disease patients that were observed by local endoscopists were confirmed when the same recordings were reviewed by central readers, researchers said here. According to Xavier Hebuterne, MD, professor of medicine at University Hospital Nice, mucosal healing in these patients was uncommon, perhaps due to the severity of the illness. The study findings were presented at the annual meeting of the American College of Gastroenterology. 356

Gastro Update What is the Clinical Presentation of Ulcerative Colitis?

Bloody, mucous diarrhea is the hallmark of UC. It can be initially intermittent, resolving spontaneously.  Mild disease: <4 stools/day with passage of blood less than daily and no systemic symptoms.  Moderate to severe colitis: >5 loose stools with blood/day often with nocturnal stools and urgency. Tenesmus is extremely common. Pain of colitis is predefecation, cramp like, suprapubic that is relieved by defecation.  Severe colitis: >12 bloody stools/day, with abdominal distention and tenderness with tachycardia and fever. It causes toxic megacolon. Dr Neelam Mohan, Director Pediatric Gastroenterology, Hepatology and Liver Transplantation, Medanta- The Medicity

Low Sodium may Boost Fracture Risk Low serum sodium may be a risk factor for fractures in the elderly. In a long-running cohort study in Holland, mild hyponatremia was associated with an increased risk of nonvertebral fractures and prevalent vertebral fractures, according to Carola Zillikens, MD, of Erasmus Medical Center in Rotterdam. Treating GERD Improves Sleep, Performance Nighttime dyspepsia and associated sleep disturbance improved significantly during treatment with a proton pump inhibitor, which in turn improved alertness on a simulated driving test according to a new study reported at the meeting of the American College of Gastroenterology. Chikungunya Update Management of Chikungunya Supportive Treatment   

Rest and mild movements of joints Cold compresses for inflamed joints Liberal fluid intake or IV fluids RP Vashist, Consultant and Head Public Health, Govt. of Delhi

Indian Journal of Clinical Practice, Vol. 21, No. 6, November 2010

Indian Journal of

Clinical Practice

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Paintal AS. Impulses in vagal afferent fibres from specific pulmonary deflation receptors. The response of those receptors to phenylguanide, potato S-hydroxytryptamine and their role in respiratory and cardiovascular reflexes. Q. J. Expt. Physiol. 1955;40:89-111.

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Stansfield AG. Lymph Node Biopsy Interpretation Churchill Livingstone, New York 1985.

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Strong MS. Recurrent respiratory papillomatosis. In: Scott Brown’s Otolaryngology. Paediatric Otolaryngology Evans JNG (Ed.), Butterworths, London 1987;6:466-470.

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PPIs may Cause Bone Fractures When Used for More Than One-year or at Higher Doses: FDA

H Dr KK Aggarwal

Group Editor-in-Chief Dr KK Aggarwal

Padma Shri and Dr BC Roy Awardee Sr Physician and Cardiologist, Moolchand Medcity President, Heart Care Foundation of India Group Editor-in-Chief, IJCP Group Chief Editor, eMedinewS Member, Delhi Medical Council Director, IMA AKN Sinha Institute (08-09) Hony. Finance Secretary, IMA (07-08) Chairman, IMA AMS (06-07) President, Delhi Medical Association (05-06) drkk@ijcp.com

igh doses or long-term use of PPIs or proton-pump inhibitors can lead to an increased risk of bone fractures. This holds especially true for those over the age of 50, and for people on the high dose. The latest warning is based on a FDA review of several studies of the treatment. These epidemiologic studies revealed an elevated fracture risk at the hip, wrist and spine. But the studies do not, definitively prove that PPIs are the cause of the fractures.

FDA has instructed the manufacturers of the drugs to change the labels for both the prescription and the over-the-counter versions of the PPIs. The FDA said they should only be taken for 14 days to help ease frequent heartburn and under no circumstances should over-the-counter PPIs be taken for more than three 14-day periods in a year.

iddle-aged women who move around more in their daily life have lower levels of intra-abdominal fat, a risk factor for heart disease. Minor modifications in daily routine such as reducing the time watching TV or increasing the walk time to work, can make a difference in the long-term health. Visceral fat is a hot topic because of metabolic syndrome, which predisposes people to disease. Intra-abdominal fat, or the fat that wraps around the organs in the abdomen and chest, tends to accumulate at midlife and can contribute to developing diabetes, hypertension and heart disease. The fat around the organs is known to be more related to heart disease and diabetes. A woman does not need to appear outwardly heavy to have a potentially troublesome extra â&#x20AC;&#x2DC;tireâ&#x20AC;&#x2122; around her organs. Exercise for long has been known to reduce the amount of intra-abdominal fat.

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