Abstract
BackgroundWilson disease (WD) is a genetic disease of abnormal copper metabolism, pediatric acute liver failure (PALF) is a life-threatening illness. Wilson’s disease with acute liver failure has rapid progress, and high mortality, especially in children.Case presentationThe patient was a 14-year-old girl who developed vomiting, jaundice within 7 days, leading to the initial suspicion of acute liver failure. Results of further investigation by geen and histological examination were consistent with a diagnosis of Wilson disease. She was treated with plasma exchange and DPMAS. After six days of treatment, coagulation routine and bilirubin improved, and she was gradually conscious. On the 8th day after admission, the condition was deteriorate, she was confusion, and had dyspnea, bleeding. The coagulation routine continued to deteriorate, bilirubin did not decrease significantly. Doppler ultrasound examination showed that the portal vein blood flow direction was away from the liver. Abdominal CT showed gastric varicose veins. She underwent liver transplantation. Thirty-three days later, she recovered. Patients with PALF should be transferred to a center with a transplant unit early. Once conservative treatment fails, LT should be performed. ConclusionA review of the literature shows that Wilson disease can occur at any age, including childhood. Wilson’s disease with acute liver failure has rapid progress, and high mortality. Early diagnosis and treatment is the key to improving the prognosis.
Wilson Disease-Induced Acute Liver Failure (NWI = 13) Salvaged without Liver Transplant by Plasmapheresis
