Introduction: We evaluated 16 years of experience of pediatric patients with fulminant Wilson's disease (WD). 
Materials and Methods: Of 98 pediatric patients with fulminant liver failure, we reviewed the clinical and laboratory data of 12 (12.2%) who had fulminant WD. PELD-MELD, Child-Pugh scores and new Wilson indexes at the time of application were calculated. The prognoses were also recorded.
Results: There were a total of 12 fulminant WD patients, 9 were female (75%). Mean age at time of arrival was 9.3±1.59 years (range: 7.3-12.5 y). 4 patients did not have findings of encephalopathy, while 2 patients had stage 1, 3 had stage 2 and 3 had stage 3 encephalopathy at the time of admission. 6 patients had ascites.
Mean hemoglobin, white blood cell count and thrombocyte levels were 7.43±2.67 g/dL, 20,330±12,430/mm3 and 131,820±93,650/mm3, respectively. The liver function tests at the time of reference were; total bilirubin: 39.26±15.66 mg/dL, direct bilirubin: 27±11.76 mg/dL, AST: 513.66±943.6 U/L, ALT: 164.6±331.8 U/L, GGT: 78.4±71.8 U/L, ALP: 234±250.6 U/L, albumin 2.8±0.73 g/dL, prothrombin time 36.44±16.29 sec, INR 4±2.25. Mean creatinine value was 0.83±0.24 mg/dL, mean glomerular filtration rate was 77.66±64.84 ml/min/1.73 m2 and 5 patients had tubulopathy during follow up. 9 patients had low seruloplasmin levels. The seruloplasmin levels varied between 5.9-26 mg/dL (mean 13.8±7.4 mg/dL). 24-hour urine copper was measured in 4 patients and the mean level was 430±600 µgr/dL. The challenge test was performed in another 4 patients and mean urine copper was 3155±2490 µgr/dL.
Mean PELD values, Child Pugh scores and new Wilson indexes were 32.1±11.5 (range: 16-48), 11.44±1.66 (range: 10-15) and 15.44±1.94 (range: 12-18), respectively. There was only 1 patient over age 11 with a MELD value of 40.
Plasmapheresis was used in all patients, while D-penicillamine were also given in 3 patients, D-penicillamine, zinc and steroids to 2; trientine, zinc and steroids to 2 patients. 4 patients died, 6 underwent liver transplantation and 2 spontaneously survived. Histopathologic examinations of explant livers or postmortem liver necropsy samples were evaluated in 9 patients. 7 patients had cirrhosis, 1 had chronic active hepatitis and 1 had diffuse microvesicular steatosis. Mean tissue copper level was 440±347 µgr/g dry liver tissue.
5 patients who underwent liver transplant had living related donors and 1 patient had deceased donor. They were followed up for 9±3.3 years (range: 4.5-12 y) after transplantation.
Conclusion: Fulminant WD is extremely fatal if liver transplant cannot be performed. Introduction of plasmapheresis and chelating therapy may be life-saving in low risk patients. Most patients have findings of cirrhosis on liver histopathological examination at time of arrival.