Clinical & Nutritional Assessments of Long-term Survivors of Biliary Atresia

ABSTRACT

To assess the clinical & nutritional status of long-term survivors of biliary atresia, history taking, medical record review, physical examination (height, weight, midarm circumference, triceps skin fold thickness), blood tests (LFT, prothrombin time, platelet count, prealbumin, calcium) and liver needle biopsy were performed in 12 patients in whom Kasai procedure had been performed up to 10 years before at Department of Pediatric Surgery in Seoul National University Hospital. In the height and weight, there was no patient below 5 percentile. Triceps skin fold thickness (TSF) was above 75 percentile in all patients and showed good fat nutritional status. Midarm circumference (MAC) was above 5 percentile in all patients, therefore there was no skeletal muscle protein malnutrition. Serum prealbumin level was abnormal in 2 patients with abnormal liver function and revealed visceral protein malnutrition. Serum calcium level was decreased below normal range in 4 patients with abnormal liver function. One patient had mild ascites. Five patients had abnormal liver function and 7 patients showed clinical manifestation of portal hypertension. Liver needle biopsy was performed in 5 patients and no cirrhotic change was observed. Some patients showed visceral protein malnutrition and vitamin D malabsorption but growth, development and nutritional status were generally satisfactory. Five patients (42%) showed normal liver function and no portal hypertension. In conclusion, Kasai procedure is satisfactory as a primary treatment in biliary atresia but significant portion (7/12) of long-term survivors had abnormal liver function or portal hypertension. Continuous and careful follow-up is needed not to miss the proper time of liver transplantation.