Avcıoğlu Sümeyra Nergiz, Altınkaya Sündüz Özlem, Küçük Mert, Zafer Emre, Demircan Sezer Selda, Odabaşı Ali Rıza
Adnan Menderes University Faculty of Medicine, Department of Gynecology and Obstetrics, Aydın, Turkey.
Muğla Sıtkı Koçman University Faculty of Medicine, Department of Gynecology and Obstetrics, Muğla, Turkey.
Turk J Obstet Gynecol. 2015 Mar;12(1):56-59. doi: 10.4274/tjod.24434. Epub 2015 Mar 15.
Wilson's disease (WD) is an autosomal recessive disorder. It is characterized by toxic accumulation of copper mainly in the liver and brain but also in cornea and kidney due to a defect in biliary excretion of copper. The hepatic manifestation of WD is diverse and may include asymptomatic elevation of aminotransferase, chronic hepatitis, cirrhosis, or acute/fulminant hepatic failure. Characteristic of acute hepatic failure in WD is concomitance of acute intravascular hemolytic anemia. Acute intravascular hemolytic anemia and thrombocytopenia in WD may be interpreted as a feature of Hemolysis, Elevated Liver Enzymes, Low Platelet Count (HELLP) syndrome besides acute liver failure. The differential diagnosis may be very difficult. Here, WD in pregnancy presenting with clinical symptoms of HELLP syndrome and developing acute liver failure in postpartum period is discussed.
威尔逊病（WD）是一种常染色体隐性疾病。其特征是由于铜的胆汁排泄缺陷，导致铜主要在肝脏和大脑中有毒性蓄积，也可在角膜和肾脏中蓄积。WD的肝脏表现多样，可能包括转氨酶无症状升高、慢性肝炎、肝硬化或急性/暴发性肝衰竭。WD急性肝衰竭的特征是伴有急性血管内溶血。WD中的急性血管内溶血和血小板减少除急性肝衰竭外，可解释为溶血、肝酶升高、血小板计数降低（HELLP）综合征的特征。鉴别诊断可能非常困难。本文讨论了妊娠期WD出现HELLP综合征临床症状并在产后发展为急性肝衰竭的情况。
