Introduction: We evaluated pediatric patients with Crigler Najjar disease who received LT at our center. 
Materials and Methods: We reviewed the clinical and laboratory data of patients with Crigler Najjar disease who received LT at Başkent University from 2001-2017. Histopathologic examinations of explanted livers were also recorded.
Results: There was total of 7 patients (5 female 71.4%). The mean age at the time of LT was 5±7.1 years (range: 0.2-17.5 y). The liver function tests before LT were: total bilirubin: 31.28±7.3 mg/dL (range: 20.7-43.3 mg/dL), direct bilirubin: 1.39±1.19 mg/dL (range: 0.46-3.93 mg/dL), AST: 174±330 mg/dL (range: 24-922 IU/L), ALT: 79±88 mg/dL (range: 19-276 IU/L), GGT: 67±53 mg/dL (range: 27-184 U/L) , ALP: 569±327 mg/dL (range: 115-1055 IU/L). Patients were given 11±3 hours (range: 8-16 hours) phototherapy (6/7 patients), phenobarbital (5/7 patients), cholestyramine (3/7 patients) before LT. Plasmapheresis was used in 1 patient to decrease bilirubin levels before LT. The mean body weight of the patients at the time of transplant was 20.9±19.6 kg (range: 6.7-58 kg). The donor was father in 5 and mother in 2 LT. Left lateral lobe was used in 5, left lobe was used in 1 and right lobe was used in 1 patient. Patients were followed up for 5.83±4.3 years (range: 1-11.5 y). Postoperative complications included hepatic artery thrombosis in 2 patients, hepatic vein thrombosis in 1, bile leak in 2, acute cellular rejection in 2, pneumothorax in 1, primary graft dysfunction and cholestasis in 1. One patient had neurologic sequela and died after vomiting and aspiration during his sleep at 1 year posttransplant. One patient has mild mental retardation. Other patients are alive with normal developmental milestones. One patient had cirrhosis and homozygous mutation in UGT1A1 gene consistent with Crigler Najjar type 1. She was only given ursodeoxycholic acid and cholestyramine as treatment before liver transplant. She is doing fine without any neurologic deficit after LT. Explanted livers showed normal structure in all patients except one. 5 patients had mild hepatocanalicular cholestasis, cirrhotic patient additionally had dilated bile ducts filled with cholestatic plugs and prominent ductular and vascular proliferation. 1 patient without cirrhosis had mild portoportal fibrosis, as well as mild bile ductular proliferation. 5 patients had lymphomononuclear inflammation in the portal areas. 3 patients showed ballooning degeneration in the hepatocytes.3 patients had neutrophilic infiltration in the paranchyme and subcapsular areas. 1 patient had diffuse microvesicular steatosis.
Conclusions: Severe form of Crigler Najjar disease can be successfully cured by LT. Mild increase in liver function tests other than bilirubin may be recorded before surgery. Histopathological explants may show cholestatic changes and portal/paranchymal inflammation. This finding may be secondary to co-existing liver diseases or Crigler Najjar disease itself.