Sir,

The concept of congenital hyperbilirubinemia, its frequency and its benign nature is not clear in our country. These patients are being investigated as acute and chronic liver disease and managed as such. This approach causes considerable anxiety and inconvenience to the patient and their relatives. A retrospective analysis was undertaken to know the behaviour and long term prognosis of these patients. Of 3629 patients attending the outpatient’s department of PMRC Research Centre, Jinnah Post­graduate Medical Centre with various liver diseases, 21 (0.58%) had congenital hyperbilirubinemia. Of these, 4had conjugated and 17 unconjugated hyperbilirubinemia. Amongst the unconjugated, 10 had Gilberts syndrome and 7 Crigler Najjar Syndrome Type H. All the cases of conjugated hyperbilirubinemia and cases with splenomegaly1 were ex­cluded from the studybecause of incomplete investigations. Mean age at first detection of jaundice was 19 years for Gilbert’s syndrome and 10 years for Crigler Najjar syndrome Type II. Male preponderance was noted in both the diseases, ratio being 9:1 and 2.5:1, respectively. In contrast no sex difference was noted by other workers1. Presenting symptoms in majority of the cases were repeated episodes of jaundice alongwith fatigue, malaise, anorexia, nausea and vomiting. Few cases had pruritus, fever with rigors and passage of dark coloured urine. All cases of Crigler Najjar and 7 of 10 cases of Gilbert’s Syndrome were jaundicated at the time of examination. Liver was just palpable in 6 cases except in one where it was palpable 5cm below the costal margin. Liver biopsy was done in6 patients; it was normal in all except mild cholestasis and round cell infiltration in one case. Of 17 cases, 10 were followed from 1-13 years. No complication was found in any case; however, icteric sciera persisted intermittently in most cases which increased with physical activity, intercurrent infections and warm weather. Response to phenobarbitone was good in 5 cases, variable in 3 and none in 2 cases. Multiplicity of symptoms and failure to elicit any common pattern of complaints indicates that symptoms may be unrelated to the disorder of bilirubin metabolism. Symptoms are likely to develop after the patients become aware of. jaundice and; therefore, are probably anxiety related. Thus if a patient has congenital hyperbilirubinemid with a normal health all that is required is reassurance.

Waquar Uddin Ahmed
PMRC Research Centre, Jinnah Post graduate Medical Centre, Karachi.