Extrahepatic biliary atresia and caudal regression syndrome in an infant of a diabetic mother.
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Extrahepatic biliary atresia and caudal regression syndrome in an infant of a diabetic mother. / Herrmann, Jochen; Brauer, Martin; Scheer, Ianina; Barthlen, Winfried; Bührer, Christoph.
In: J PEDIATR-US, Vol. 39, No. 1, 1, 2004, p. 20-22.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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T1 - Extrahepatic biliary atresia and caudal regression syndrome in an infant of a diabetic mother.
AU - Herrmann, Jochen
AU - Brauer, Martin
AU - Scheer, Ianina
AU - Barthlen, Winfried
AU - Bührer, Christoph
PY - 2004
Y1 - 2004
N2 - Although most cases of extrahepatic biliary atresia are thought to result from perinatal obliterating inflammation, some are associated with a faulty morphogenesis. The authors report on a baby girl of a mother with ill-managed insulin-dependent diabetes mellitus. The baby presented with sacro-coccygeal agenesis, clubfoot, and ano-urinary incontinence. In addition, there was polysplenia, no inferior vena cava, and the portal vein was grossly distorted. Progressive conjugated hyperbilirubinemia prompted liver biopsy at 4 weeks of age, showing intracanalicular cholestasis without fibrosis. The diagnosis of extrahepatic biliary atresia was confirmed during laparotomy performed for hepatoportojejunostomy. Some cases of extrahepatic biliary atresia might be part of a spectrum of malformations associated with maternal diabetes mellitus.
AB - Although most cases of extrahepatic biliary atresia are thought to result from perinatal obliterating inflammation, some are associated with a faulty morphogenesis. The authors report on a baby girl of a mother with ill-managed insulin-dependent diabetes mellitus. The baby presented with sacro-coccygeal agenesis, clubfoot, and ano-urinary incontinence. In addition, there was polysplenia, no inferior vena cava, and the portal vein was grossly distorted. Progressive conjugated hyperbilirubinemia prompted liver biopsy at 4 weeks of age, showing intracanalicular cholestasis without fibrosis. The diagnosis of extrahepatic biliary atresia was confirmed during laparotomy performed for hepatoportojejunostomy. Some cases of extrahepatic biliary atresia might be part of a spectrum of malformations associated with maternal diabetes mellitus.
M3 - SCORING: Zeitschriftenaufsatz
VL - 39
SP - 20
EP - 22
JO - J PEDIATR-US
JF - J PEDIATR-US
SN - 0022-3476
IS - 1
M1 - 1
ER -
