IllnessGilbert [Meulengracht] syndrome, unconjugated hyperbilirubinemia

X (UGT1A1 promotor analysis)

In the German-speaking medical world, terms such as Morbus Meulengracht, Meulengracht syndrome or Gilbert-Meulengracht syndrome (German ICD-10 80.4) are often used for a clinical picture that is largely uniformly referred to as "Gilbert syndrome" in other countries. Gilbert syndrome results in benign, chronic, stable or intermittent, unconjugated hyperbilirubinaemia; no structural liver disease or haemolysis is present. Gilbert's syndrome is the most common disorder of bilirubin metabolism in the liver and also often causes neonatal jaundice.

Laboratory data:

Crigler-Najjar syndrome type 1: total serum bilirubin 20-45mg/dL [complete UGT1A1 deficiency].

Crigler-Najjar syndrome type 2: total serum bilirubin 6-20mg/dL [<20% UGT1A1 activity].

DD: Gilbert syndrome/M. Meulengracht: total serum bilirubin 1-6mg/dL [UGT1A1 promoter deficiency].

• Arias syndrome - Crigler-Najjar syndrome 2
• Bilirubin uridinediphosphate glucuronosyltransferase deficiency type 1 [CNS1]/Type 2 [CNS2]
• Bilirubin-UGT deficiency type 1 [Crigler-Najjar syndrome 1]/Type 2 [Crigler-Najjar syndrome 2]
• Crigler-Najjar syndrome, type I (UGT1A1)
• Crigler-Najjar syndrome, type II (UGT1A1)
• Gilbert syndrome (UGT1A1)
• Hereditary unconjugated hyperbilirubinemia type 1 [Crigler-Najjar syndrome 1]/type 2 [CNS2]
• Hyperbilirubinaemia, familial transient neonatal (UGT1A1)
• UGT deficiency type 1 [Crigler-Najjar syndrome 1]/Type 2 [Crigler-Najjar syndrome 2]