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Titel:

Treatment of Crigler-Najjar type 1 disease: relevance of early liver transplantation.

Dokumenttyp:
Journal Article; Article
Autor(en):
Schauer, R; Stangl, M; Lang, T; Zimmermann, A; Chouker, A; Gerbes, AL; Schildberg, FW; Rau, HG
Abstract:
BACKGROUND: Crigler-Najjar syndrome type 1 (CNS1) is characterized by severe unconjugated hyperbilirubinemia from birth, caused by total failure of UDP-glucuronyltransferase activity. Only orthotopic liver transplantation (OLT) offers the prospect of cure. However, because the onset of neurologic deficits is unpredictable, timing of OLT remains difficult. METHODS: In our transplant center, 3 patients underwent early OLT for CNS1. Two of them (7 yr, 12 yr) showed mild to moderate neurologic defic...     »
Zeitschriftentitel:
J Pediatr Surg
Jahr:
2003
Band / Volume:
38
Heft / Issue:
8
Seitenangaben Beitrag:
1227-31
Sprache:
eng
PubMed:
http://view.ncbi.nlm.nih.gov/pubmed/12891498
Print-ISSN:
0022-3468
TUM Einrichtung:
Fachgebiet Perinatalmedizin und Perinatalphysiologie (Prof. Schneider)
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