Article
Liver transplantation for an infant with neonatal intrahepatic cholestasis caused by citrin deficiency using heterozygote living donor.
Pediatric transplantation - 1 Nov 2010
Shigeta Takanobu, Kasahara Mureo, Kimura Takuya, Fukuda Akinari, Sasaki Kazunari, Arai Katsuhiro, Nakagawa Atsuko, Nakagawa Satoshi, Kobayashi Keiko, Soneda Shun, Kitagawa Hiroaki
Abstract excerpt
NICCD is an autosomal recessive genetic disorder, characterized by cholestasis, coagulopathy, hypoglycemia, fatty liver and multiple amino acidemia. NICCD develops in the neonatal/infantile period and has been reported as a "naturally curable" disease within one yr of life. Recently, we experienced an infantile NICCD who developed progressive liver failure, and required subsequent LT using a heterozygote living...
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