Kawasaki Disease Immunoglobulin Resistance. Diagnosis and Therapeutical Management

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Peertechz Journal of Pediatric Therapy Carlos Alberto Sanchez Salguero1* and Alvaro Isidro Sanchez Chacon2 Head of Pediatric Allergy and Neumology Section. Professor of Pediatric. School of Medicine. University Hospital Puerto Real, Spain 2 Pediatric Department. University Hospital Puerto Real, Spain 1

Dates: Received: 07 March, 2016; Accepted: 29 March, 2016; Published: 31 March, 2016 *Corresponding author: Carlos Alberto Sánchez Salguero, Head of Pediatric Allergy and Pneumology Department, Professor of Pediatric. Medicine School of Cádiz. University Hospital Puerto Real (Cádiz). Spain. E-mail: www.peertechz.com Keywords: Kawasaki disease; Resistance; Immunoglobulin

Research Article

Kawasaki Disease Immunoglobulin Resistance. Diagnosis and Therapeutical Management Abstract Background: Kawasaki disease (KD) is an acuteself-limited vasculitis that primarily affects children under 5 years. It is the leading cause of acquired heart disease in children in developed countries. Intravenous immunoglobulin (IVIG) and aspirin are the main stay of treatment. 20% of patients are resistant to IVIG, presenting increased risk of coronary aneurysms. Several scores have been developed in Japan to identify patients with increased risk of resistance to IVIG. Objectives and Methods: To analyze the cases of KD diagnosed, during four years, in a tertiary hospital, and analyze the characteristics of patients who were resistant to IVIG. Retrospective study of cases that met clinical criteria for EK, in our hospital, during the years 2009-2013 was performed. Results: 48 patients were diagnosed. The boy / girl ratio was 26/22. The age range was 3.3months to 13 years. Thirteen patients had atypical age. The most common clinical manifestations were fever (100%), followed by polymorphous rash (89%), changes in oral cavity (83%), bilateral conjunctivitis (78%), changes in extremities (68%) and cervical lymphadenopathy (43%). 18% were incomplete forms and three patients developed shock. 25% had echocardiographic abnormalities in the acute phase. All were treated with IVIG and aspirin. There were 8 cases refractory to IVIG. None has cardiac sequelae. Conclusions: To a KD with severity data (refractory to IVIG, persistently elevated CRP, <1 Year old or shock) there is an increased risk of developing coronary aneurysms.

Introduction

Objective

Kawasaki disease is an acute, self-limited vasculitis that primarily affects children under 5 years. It is the leading cause of acquired heart disease in children in developed countries.

Describe the epidemiological, clinical, laboratory, treatment, cardiac involvement and sequelae of cases of Kawasaki disease, diagnosed for four years in a tertiary hospital and analyze the specific characteristics of the patients, who were resistant to treatment with IGIV and compare them with those responding properly.

It is characterized by fever, non-exudative bilateral conjunctivitis, erythematous lips and oral mucosa, changes in extremities, rash and cervical lymphadenopathy. Its importance is that 15-25% of the untreated children develop aneurysms in the coronary arteries can lead to myocardial infarction, sudden death or ischemic heart disease. There incomplete or atypical, more frequent extreme ages, that for delay in diagnosis and consequently in treatment have a higher risk of developing coronary aneurysms cases. Treatment with intravenous gamma globulin (IVIG) in the first 10 days of illness significantly decreases the prevalence of aneurysms, (from 20% to <5%). 20% of patients are resistant to IGI V1. Several scores have been developed in Japan to identify patients at increased risk of IVIG treatment resistance, and therefore greater risk of developing coronary aneurysms. Kobayashi et al they proposed in 2006 a model prediction refractory to treatment with IVIG [1], based on 7 variables: age <12 months start time of treatment <5 days and laboratory abnormalities (percentage of neutrophils> 80%, elevated AST> 100 IU / L, sodium <133 mmol / l, CRP> 10 mg / dl, platelets <300,000 / mm3) [2]. These scores have low sensitivity and specificity in no patients Japanese’s [3].

Materials and Methods Retrospective study of cases that met clinical criteria for EK in our hospital during 2009-2013. The stories were reviewed individual clinics discharge patients diagnosed with EK. It was defined as refractory patients in the fever persisted 24-48 hours after the first dose of IVIG, or resorted to 36 hours or more after completing the infusion of IGIV EK. We compared the variables of the score of Kobayasi of IVIG responders with no responders.

Results EK was diagnosed 48 patients during this period. Nine cases were incomplete forms, and 3 cases developed cardiogenic shock and required admission to the Unit Pediatric Intensive Care (PICU). The boy / girl ratio was 26/22. The mean age at diagnosis was 3.5 years, with a range of 3.3 months to 13 years. Thirteen of the patients had atypical age (less than six months or more than five years) to diagnosis. Regarding

Citation: Sanchez Salguero CA, Sanchez Chacon AI (2016) Kawasaki Disease Immunoglobulin Resistance. Diagnosis and Therapeutical Management. Peertechz J Pediatr Ther 2(1): 001-003.

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