TIN-PROTOPORPHYRIN (Sn-pP) EFFECT ON HYPER- BILIRUBINEMIA DUE TO CRIGLER-NAJJAR DISEASE (CND) TYPE 1

Crigler-Najjar syndrome type 2.

Crigler-Najjar syndrome is a rare disorder of bilirubin metabolism with two distinct forms: type 1 and type 2. We report three patients with Crigler-Najjar syndrome type 2 (CN-2). All patients had serum bilirubin values higher than 171 micromol/L and deep yellow skin color. The results of other liver function tests, glucose-6-phosphate dehydrogenase activity and hematology tests were normal, an...

Crigler-Najjar syndrome

Inheritance of type 2 Crigler-Najjar hyperbilirubinaemia.

The families of three patients with Crigler-Najjar hyperbilirubinaemia, type 2, whose plasma bilirubin levels had responded to phenobarbitone treatment, were investigated. All the parents and several relatives had mildly raised bilirubin levels. It is suggested that this condition may be an example of genetic heterogeneity and that the propositi had inherited two different abnormal genes. The s...

The Successful Anaesthetic Management of Crigler-Najjar Syndrome Type 2

We describe the peri-operative management of a patient with Crigler-Najjar Syndrome type 2, who presents with acute cholecystitis and undergoes open cholecystectomy. We review the pathophysiology of this disease as well as the enzyme defect responsible; we highlight the important management principles during the preand postoperative period; and with the knowledge of the metabolic pathways invol...

Crigler-Najjar syndrome type II in a Chinese boy

11 Background: The UGT1A1 gene encodes a responsible enzyme, 12 UDP-glucuronosyltransferase1A1 (UGT1A1), for bilirubin metabolism. Many 13 mutations have already been identified in patients with inherited disorders with 14 unconjugated hyperbilirubinemia, such as Crigler-Najjar syndromes and Gilbert’s 15 syndrome. 16 Case presentation: In this report, we presented a boy with intermittent 17 unc...