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Neonatal Dubin‐Johnson Syndrome with Severe Cholestasis: Effective Phenobarbital Therapy
Author(s) -
KIMURA A.,
USHIJIMA K.,
KAGE M.,
MAHARA R.,
TOHMA M.,
INOKUCHI T.,
SHIBAO K.,
TANAKA N.,
FUJISAWA T.,
ONO E.,
YAMASHITA F.
Publication year - 1991
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1991.tb11867.x
Subject(s) - phenobarbital , cholestasis , medicine , bilirubin , neonatal cholestasis , jaundice , urine , gastroenterology , urinary system , endocrinology , bile acid , biliary atresia , transplantation , liver transplantation
. We described Dubin‐Johnson syndrome (DJS) with severe cholestasis in a 20‐day‐old Japanese boy. Although neonatal DJS has been sporadically reported, DJS with severe cholestasis has not to our knowledge been described in the English literature. The ratio of urinary coproporphyrin isomer I to urinary total coproporphyrin in our patient was high (93%). Liver histology showed cytoplasmic pigment granules in the liver cells. Administration of phenobarbital (PB) significantly decreased the levels of bilirubin and bile acids in the serum. There was a significant elevation of 1β‐hydroxylated bile acids in the urine. It is predicted that severe cholestasis in neonatal DJS may cause metabolic abnormalities in both bilirubin and bile acids transport.