NEONATAL CHOLESTASIS IN 2 SIBLINGS - A VARIANT OF DUBIN-JOHNSON-SYNDROME

Objective: Two Japanese brothers with neonatal cholestasis associated with pigment granules in the hepatocytes and hepatosteatosis were eval uated for the possible role of hepatosteatosis in the Dubin-Johnson sy ndrome. Methodology and results: The morphology of pigment accumulatio n and the laboratory data in these cases were examined. The elevation of urinary coproporphrin isomer I to more than 90% and the presence ch olestasis resembled that in the Dubin-Johnson syndrome, but the hypert riglyceridaemia (>1.13 mmol/L as triolein) and the hepatosteatosis dif fered. Both infants were thought to have familial hypertriglyceridaemi a. However, this diagnosis was difficult to confirm in the absence of data on the normal values of apolipoprotein and lipoprotein isomer for infants. Conclusions: A neonatal variant of the Dubin-Johnson syndrom e may account for the unusual findings in these infants.