Crigler

Crigler is a surname. In medical literature, it is notably associated with Crigler-Najjar syndrome, which is named after the physicians who first described the disorder in 1952.

Crigler-Najjar syndrome is a rare genetic condition characterized by severe unconjugated hyperbilirubinemia resulting from deficient hepatic activity of the enzyme UDP-glucuronosyltransferase (UGT1A1). It is inherited in an autosomal recessive manner. There are two main subtypes. Type I features a complete lack of UGT1A1 activity and presents with life-threatening jaundice in infancy and a high risk of kernicterus. Type II (also called variant Crigler-Najjar) involves partial enzyme activity and generally has a milder course, with some patients responding to phenobarbital, which can increase UGT1A1 expression.

Management of Crigler-Najjar syndrome aims to prevent bilirubin neurotoxicity. For Type I, intensive phototherapy and exchange transfusions may be required, and liver transplantation can be curative in severe cases. For Type II, treatment may involve medications that induce enzyme activity and ongoing monitoring. The condition remains a focus of genetic and hepatology research due to its impact on bilirubin metabolism and neural safety in the developing nervous system. The surname Crigler, while less common, appears in various cultural and professional contexts beyond this medical eponym.