Management of hyperbilirubinemia and prevention of kernicterus in 20 patients with Crigler-Najjar disease

We summarize the treatment of 20 patients with Crigler-Najjar disease (CND) managed at one center from 1989 to 2005 (200 patient-years). Diagnosis was confirmed by sequencing the UGTA1A gene. Nineteen patients had a severe (type 1) phenotype. Major treatment goals were to maintain the bilirubin to a...

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Veröffentlicht in:	European journal of pediatrics 2006-05, Vol.165 (5), p.306-319
Hauptverfasser:	STRAUSS, Kevin A, ROBINSON, Donna L, VREMAN, Hendrik J, PUFFENBERGER, Erik G, HART, Graham, MORTON, D. Holmes
Format:	Artikel
Sprache:	eng
Schlagworte:	Adolescent Adult Age Albumins - therapeutic use Biological and medical sciences Blood Child Child, Preschool Cholagogues and Choleretics - therapeutic use Cholecystectomy Crigler-Najjar Syndrome - complications Crigler-Najjar Syndrome - genetics Crigler-Najjar Syndrome - therapy Follow-Up Studies Gastroenterology. Liver. Pancreas. Abdomen General aspects Glucose - therapeutic use Hospitalization - statistics & numerical data Humans Infant Infusions, Intravenous Kernicterus - etiology Kernicterus - prevention & control Laboratories Light emitting diodes Light therapy Liver Transplantation - statistics & numerical data Liver. Biliary tract. Portal circulation. Exocrine pancreas Medical sciences Other diseases. Semiology Patients Phototherapy - methods Skin Sweetening Agents - therapeutic use Transplants & implants Traumatic brain injury Ursodeoxycholic Acid - therapeutic use
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Zusammenfassung:	We summarize the treatment of 20 patients with Crigler-Najjar disease (CND) managed at one center from 1989 to 2005 (200 patient-years). Diagnosis was confirmed by sequencing the UGTA1A gene. Nineteen patients had a severe (type 1) phenotype. Major treatment goals were to maintain the bilirubin to albumin concentration ratio at
ISSN:	0340-6199 1432-1076
DOI:	10.1007/s00431-005-0055-2