WILSONS DISEASE IN ADULTS A SERIES OF 13 CASES IN THE GASTROENTEROLOGY DEPARTMENT OF THE UNIVERSITY HOSPITAL OF FEZ

Wilsons disease (WD) is a rare genetic disorder with autosomal recessive transmission. It is a copper toxicosis characterized by an accumulation of free copper mainly in the liver, brain and pericorneal. The objective of this work is to study the clinical, biological and morphological parameters of Wilsons disease as well as the evaluation of liver damage and its complications. We conducted a retrospective study in the gastroenterology department at the HASSAN II University Hospital in Fez on 13 patients over a period of 16 years. There were five men and eight women. The average age of discovery of the disease was 15 years and 8 months, with extremes of 5 years and 36 years. Consanguinity was found in seven patients. Ascites was the main reason for consultation encountered in five patients, followed by jaundice in four patients. Neurological involvement such as tremor and dysarthria was found in four patients. Only one patient presented with acute hepatitis with signs of hepatic encephalopathy. The KAYSER FLEISHER ring was found in four patients. Seven patients had liver damage without neurological damage. The ceruloplasmin measured in twelve patients was low in eight. The copper urine measured in all patients was found to be increased in ten of them. Abdominal ultrasound showed chronic liver disease in eleven patients. Eleven patients were treated with D-penicillamine and two patients were treated with zinc acetate. In terms of evolution, ten patients showed good clinical improvement. One patient presented with hemorrhagic decompensation with death. One patient developed hepatocellular carcinoma and one patient was lost to follow-up after a one-year follow-up period. Early diagnosis of Wilsons disease allows for better management, with an improvement in the vital and functional prognosis of patients.