MCQ-Paediatric

FRCPath Part1 Course

Paediatric Pathology MCQ 1 A neonate who failed to pass the meconium presents with fever, diarrhoea and abdominal distension. A rectal biopsy is taken to reach a diagnosis. Which of the following is true?

A. A full thickness biopsy is essential for the diagnosis B. The biopsy should be put in formalin immediately after the procedure to ensure good fixation for Acetylecholinesterase staining.

C. Presence of thick nerve fibres in the biopsy excludes the diagnosis.

D. Presence of ganglion cells in appropriate sample excludes the diagnosis under consideration.

E. Biopsy should be taken at muco-cutaneous junction.

MCQ 2 In a suspected case of HD which one immunohistochemistry stain you would ask for on a rectal biopsy?

A. B. C. D. E.

NSE NFP Calretenin MUC 1 MUC5

MCQ 3 An abdominal tumour from a 3 month old infant shows a small round blue cells with focal rosettes.

In places the background is fibrillary (neuropil). Which of the following features of this tumour are associated with poor prognosis?

A. B. C. D. E.

Age under <1 y Hyperploidy Presence of many ganglion cells N-myc gene amplification Underlying malformation of Kidney.

MCQ 4 An autopsy done on an 8 month old child revealed Pneumocystis carinii pneumonia. He was also noted to have abnormal facies, very small thymus gland and small ventricular septal defect.

The review of clinical records revealed that he was born at term and had tetany at one hour of life.

Since then he had multiple episodes of respiratory infections.

Which of the following mechanisms, best explains the clinical features.

A. B. C. D. E.

Abnormal development of the pharyngeal arches and pouches.

Failure of maturation of B cells into plasma cells.

Lack of adenosine deaminase Acquisition of maternal HIV infection at delivery Failure of differentiation of PreB cells into B cells.

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FRCPath Part1 Course MCQ 5 A baby was delivered at 31 gw by emergency caesarean section as mother had severe preeclampsia.

The Apgar scores were 5 and 6 at 1 and 5 min but within 1 hour she experienced severe respiratory distress and dies despite resuscitative measures. The lung histology at autopsy showed atelectasis and alveolar hyaline membranes.

Which of the following conditions best accounts for this finding: A. B. C. D. E.

Foetal anaemia Congenital toxoplasmosis Immaturity of lungs Oligohydramnios Maternal toxaemia

MCQ 6 Which of the following does not increase the risk of Hyaline Membrane Disease ?

A. B. C. D. E.

Prematurity Maternal diabetes Twins Caesarean sections without trial of labour Infants with prenatal stress

MCQ 7 A 9 year old boy died of septicaemia. Pseudomonas aerugenosa was cultured from the blood and sputum before the death. There is a history of diarrhoea with bulky foul smelling stool for last 5 years. The autopsy findings included necrotizing pneumonia, mucus plugging of the pancreatic ducts with exocrine pancreatic atrophy. Which of the following is most likely to explain the findings in this child?

A. Galactose -1 –Phosphate uridyltransferase deficiency B. LDL receptor gene mutation C. Phenylalanine hydroxylase deficiency D. Impaired epithelial cell transport of chloride ion.

E. Common variable immunodeficiency.

MCQ 8 Which of the following is not a feature of Cystic fibrosis?

A. Recurrent pulmonary infections B. Pancreatic insufficiency C. Steatorrhea D. Intestinal obstruction E. Male infertility F. Hepatic cirrhosis G. Mental retardation

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FRCPath Part1 Course MCQ 9 Which of the following is not true about Cystic fibrosis?

A. Defect in epithelial chloride channel protein B. Mutations of CFTR gene on chromosome 7 C. There is increased resorption of sodium and chloride in the sweat duct.

D. There is reduction in chloride secretion to the lumen of respiratory tract.

MCQ 10 A neonate died at few hours of life after being born by caesarean section done for markedly enlarged abdomen which interfered with delivery. The autopsy showed bilateral, markedly enlarged cystic kidneys. The cut-surface had sponge like appearance with elongated cysts at right angles to the cortical surface.

The liver on histology showed ductal plate malformation with mild portal fibrosis. Pulmonary hypoplasia secondary to oligohydramnios was the cause of death. What is the most likely primary diagnosis?

A. Autosomal recessive polycystic kidney disease B. Autosomal dominant polycystic kidney disease C. Multicystic dysplastic Kidney disease D. Medullary sponge kidney E. Nephronophithisis MCQ 11 Which of the following is not true about Autosomal dominant polycystic kidney disease (ADPKD) ?

A. B. C. D. E. F.

Also called adult PKD It may occur in infancy and childhood In paediatric age group may show prominent glomerular cysts.

May also show cysts in liver, pancreas and seminal vesicles.

Ductal plate malformation / hepatic fibrosis is never seen Cysts are of variable size and round rather than elongated.

MCQ 12 Which of the following is not true about Multicystic dysplastic kidney disease?

A. B. C. D.

It is usually unilateral It is the most common cause of renal mass in neonate It is usually not associated with multisystem syndromic abnormality It should be distinguished by diffuse dysplasia which is bilateral and often a component of syndromes.

E. Patent ureter should be present for definitive diagnosis.

F. Morphologically dysplasia is seen as lobar disorganisation, immature mesenchyme, cysts and chondroid islands.

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FRCPath Part1 Course MCQ 13 Which of the following is not true about Medullary Sponge Kidney?

A. B. C. D.

Congenital cystic dilatation of terminal collecting ducts in medullary pyramids.

Normal size of the kidneys or only mild enlargement Common cause of renal failure in first decade of life.

Urolitheasis and UTI are common presentation.

MCQ 14 Which of the following is not true about Nephronophthesis?

A. Cysts predominantly at cortico-medullary junction.

B. Kidney size is normal or decreased.

C. It is autosomal recessive with early onset of renal failure as opposed to Medullary cystic disease complex which is autosomal dominant with late onset renal failure.

D. It is due to a defect in collagen type IV E. Histology is characterised by tubular and glomerular cysts F. Tubulo- interstitial inflammation is prominent

Match the following:

ARPKD ADPKD Multicystic dysplasia Diffuse cystic dysplasia Nephronophthisis Medullary sponge Kidney MCQ 15 Which of the following sarcoma is negative (normal) for EWS break apart FISH ?

A. B. C. D. E.

Ewings sarcoma Clear cell sarcoma DSRCT Myxoid Chondrosarcoma Alveolar soft part sarcoma

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FRCPath Part1 Course EMQ 1 A. Wilms tumour B. Clear cell sarcoma of the Kidney C. Ewings/ PNET D. Pancreatoblastoma E. Desmoblastic small round cell tumour F. Pleomorphic rhabdomyosarcoma G. Neuroblastoma H. Synovial Sarcoma I. Alveolar rhabdomyosarcoma J. Epitheliod sarcoma K. Large cell lymphoma L. Melanoma M. Embryonal rhabdomyosarcoma N. Fibrosarcoma 1. Multiple intra- abdominal mass in a 16-year-old male. Small round cells showing positivity for EMA, CK (globoid), NSE, desmin and WT1. The tumour shows (11; 22) translocation.

2. A polypoid vaginal mass in an 11-month old female showing dense zone of undifferentiated tumour cells immediately beneath the epithelium (Cambium layer) 3. A 4- year-old male showing macrosomia, hemi hypertrophy and exomphalus presents with an abdominal tumour. Histology showed a triphasic tumour with epithelial, mesenchymal and blastemal elements.

4. A left sided abdominal tumor from an infant feature small round blue cells which show following immunophenotype.Desmin -, myogenin,- CD99-, CD45-, NB84+, NSE+ 5. A fore arm tumour from a 12 year old child shows sheets of small round cells without fibrosis. The tumour cells are Myo D1 positive and show translocation t(2:13)

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FRCPath Part1 Course EMQ 2 Definition of terms used in the study of errors in morphogenesis.

A. B. C. D. E. F. G. H. I. J. K. L. M.

Malformation Syndrome Sequence Dysplasia Disruption Agenesis Atresia Deformation Hypoplasia Hypertrophy Inflammation Aplasia Minor anomaly

1. It results from secondary destruction of an organ or body region that was previously normal.

2. These are result of primary errors of morphogenesis or intrinsically abnormal developmental process.

3. A constellation of congenital anomalies believed to be pathologically related and that cannot be explained on the basis of single, localized, initiating events.

4. Anomalies resulting as a result of complete absence of an organ and its primordium.

5. In context of malformation, this term describes abnormal organisation of cells in a tissue.

NOTES

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FRCPath Part1 Course

BONUS QUESTIONS

1.

Which of the following is true for CIC-DUX 4 Sarcoma ? a. Undifferentiated small cell sarcoma b. More atypical features than typical EWS/ PNET c. Show focal or multifocal CD99 positivity. d. Positive for WT1 e. Negative for EWS translocation. f. All of the above

2. Which of the following is not true ? a. INI 1 is normally expressed in all nucleated cells. b. Also called hSNF5, SMARCB1 and BAF47 c. Renal and extra renal Rhabdoid tumours are negative for INI-1 d. Loss of INI1 expression usually associated with deletion or mutation of the INI1 gene on 22q11.2 e. Germline INI1 mutations associated with sporadic schwannomatosis f. Epithelioid hemangioendothelioma show loss of INI 1 expression.

3 Which of the following is true about decidual vasculopathy ? a. Results from abnormal / incomplete remodeling of the spiral arteries. b. Observed in placentas from pre-eclamptic pregnancies as well as several collagenvascular and in those with evidence of growth restriction. c. Lesions vary from non-necrotic, non-transformed decidual arterioles to early fibrinoid necrosis of vessel walls and late atherosis. d. Acute atherosis is morphologically seen as fibrinoid necrosis with subendothelial lipid-laden macrophages e. All of the above 4

Which of the following is true about “villous dysmaturity / delayed villous maturation” a. Proportion of cases are associated with diabetes. b. Placenta is usually large for gestational age. c. Terminal villi may be hypervascular and edematous with reduced numbers of syncytial knots. d. It is known to be associated with late fetal hypoxia and IUD e. All of the above.

5 Which of the following is not a feature of fetal vascular malperfusion ?

Thrombosis of chorionic plate vessel.

Avascular villi Villous stromal‐vascular karyorrhexis (VSK) Perivillous fibrin deposition Intramural fibrin deposition, chorionic plate vessel

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