| ID |
http://id.who.int/icd/entity/1098453659 |
| Preferred Name |
Syndrome de Crigler-Najjar type 1 |
| Definitions |
Crigler-Najjar syndrome type 1 is the most severe form ofCrigler-Najjar syndrome, a hereditary disorder of hepatic bilirubin conjugation, characterized by severe neonatal unconjugated hyperbilirubinemia due to a complete absence of hepatic bilirubin glucuronosyltransferase (BGT). |
| Type |
http://www.w3.org/2002/07/owl#Class |
| comment | le syndrome de crigler-najjar de type 1 est la forme la plus sévère du syndrome de crigler-najjar, un trouble héréditaire de conjugaison de la bilirubine hépatique, caractérisé par une hyperbilirubinémie néonatale non conjuguée grave due à l'absence complète de la bilirubine glucuronosyltransférase (bgt) hépatique. crigler-najjar σύνδρομο τύπου 1 είναι η πιο σοβαρή μορφή σύνδρομο crigler-najjar, μια κληρονομική διαταραχή της ηπατικής συριγμού χολερυθρίνης, χαρακτηρίζεται από σοβαρή neonatal unconjugated hyperbilirubinemia οφείλεται σε πλήρη απουσία ηπατική χολερυθρίνη glucuronosyltransferase (bgt). Crigler-Najjar syndrome type 1 is the most severe form ofCrigler-Najjar syndrome, a hereditary disorder of hepatic bilirubin conjugation, characterized by severe neonatal unconjugated hyperbilirubinemia due to a complete absence of hepatic bilirubin glucuronosyltransferase (BGT). Das Crigler-Najjar-Syndrom Typ 1 ist die schwerste Form des Crigler-Najjar-Syndroms, einer erblichen Störung der hepatischen Bilirubin-Konjugation, die durch eine schwere neonatale unkonjugierte Hyperbilirubinämie aufgrund des völligen Fehlens der hepatischen Bilirubin-Glucuronosyltransferase (bgt) gekennzeichnet ist. crigler-najjar typ 1 jest najcięższą formą syndromu crigler-najjar, dziedziczne zaburzenie koniugacji wątroby bilirubiny, charakteryzuje się ciężką neonatalną hiperbilirubinemia z powodu całkowitej braku wątroby bilirubiny glucuronosyltransferase (bgt). |
| broaderTransitive | |
| prefLabel | Syndrome de Crigler-Najjar type 1 |
| browserUrl | NA |
| label | Crigler-Najjar-Syndrom Typ 1 Syndrome de Crigler-Najjar type 1 Crigler-Najjar syndrome type 1 crigler-najjar σύνδρομο τύπου 1 zespół crigler-najjar typ 1 |
| subClassOf |
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