AUTOSOMAL RECESSIVE POLYCYSTIC KIDNEY DISEASE WITH CONGENITAL HEPATIC FIBROSIS- A CASE REPORT

PRESENTATION OF CASEA 11 years old female child presented with pain abdomen and abdominal distension on and off ever since she was 4 years; not associated with vomiting or altered bowel habits.There was no history of fever, dysuria or increased frequency of micturition.She also had decreased growth rate along with impairment of cognition and memory.The child had a history of one episode of hematemesis when she was 9 months old.There was no history of yellowish discoloration of the skin or urine.She was born spontaneously by vaginal delivery and the neo-natal period was uneventful.There was no medically significant family history of any illness running in her family in the previous two generations.The child had pallor on clinical examination.Her blood pressure was within normal limits.She was short statured.Per-abdominally, liver was palpable, and the spleen was 4 cm below the left sub-costal margin.Her jerks were exaggerated.The laboratory parameters showed normal liver and renal function tests.Her serum TSH level was >100 IU.