Neonatal Dubin-Johnson syndrome with severe cholestasis: effective phenobarbital therapy.
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 1 beta-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.
Kimura
A
A
Department of Pediatrics and Child Health, Kurume University School of Medicine, Fukuoka Prefecture, Japan.
Ushijima
K
K
Kage
M
M
Mahara
R
R
Tohma
M
M
Inokuchi
T
T
Shibao
K
K
Tanaka
N
N
Fujisawa
T
T
Ono
E
E
D001647:Bile Acids and Salts / Q000097:blood / Q000652:urine; D001663:Bilirubin / Q000097:blood; D002779:Cholestasis / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D007231:Infant, Newborn; D007566:Jaundice, Chronic Idiopathic / Q000150:complications* / Q000188:drug therapy; D008099:Liver / Q000473:pathology; D008297:Male; D010634:Phenobarbital / Q000627:therapeutic use*
D001647:Bile Acids and Salts; D001663:Bilirubin; D010634:Phenobarbital