Complications of Liver Transplant in Adult Patients With the Hepatic Form of Wilson Disease.

Abstract

Wilson disease is an autosomal, recessive, inherited disorder of copper metabolism that results in the accumulation of copper in many organs and tissues. This disease is mainly characterized by dysfunction due to copper accumulation in the liver, kidney, brain, cornea, bone, heart, and blood cells. The clinical spectrum is broad in Wilson disease. Asymptomatic Wilson disease may be present, but findings related to the involvement of an individual organ or multiple organ failure can be seen. These findings can include neurologic and neuropsychiatric complications. Our aim here was to examine the neurologic complications and our clinical experience in patients who underwent liver transplant for Wilson disease in our clinic. We retrospectively reviewed the medical records of transplant patients with Wilson disease who were seen at Baskent University Faculty of Medicine Transplantation Science between 2005 and 2017. Patient demographics, neurologic complaints, findings from neurologic examinations, and imaging findings were recorded. We also recorded the presence of the Kayser-Fleischer ring, serum ceruloplasmin, 24-hour copper urine levels, and levels of dry copper in liver in each patient. Our study included 19 patients who ranged in age range from 18 to 44 years (mean age of 26 years). Seven of 19 patients (36.8%) had neurologic symptoms, including epileptic seizures in 2 patients (10.5%), encephalopathy in 1 patient (5.2%), tremor in 3 patients (15.7%), and headache in 1 patient (5.2%). The cause of these long-term neurologic complications was the immunosuppressive drugs. Patients with epileptic seizures were provided with seizure control medication (levetiracetam). Tremor did not need treatment. In Wilson disease, neurologic complications can be severe. The most common complication seen in our patients was tremor. Early diagnosis and treatment may slow down neurologic disability.