Acar S, Gencdal G, Tokac M, Eren E, Alkara U, Tellioglu G, Dinckan A, Akyildiz M
Department of Internal Medicine and Organ Transplantation, Yeniyuzyil University Gaziosmanpasa Hospital, Istanbul, Turkey.
Department of Gastroenterology and Organ Transplantation, Yeniyuzyil University Gaziosmanpasa Hospital, Istanbul, Turkey.
Transplant Proc. 2017 Apr;49(3):603-605. doi: 10.1016/j.transproceed.2017.01.033.
Polycystic liver disease is characterized by multiple cystic lesions on the liver. It is an uncommon autosomal dominant disease. The cysts' diameters range from 20 to 30 cm to small microscopic nodules. Generally, more than half of the liver parenchyma is covered. The mass effect of the liver created by the large cysts can cause life-threatening symptoms such as weight loss, reduction of oral intake, and malnutrition. Liver transplantation is the best treatment option in symptomatic patients. We present a patient who had polycystic liver and kidney disease, and we performed liver transplantation because of his life-threatening symptoms.
多囊肝病的特征是肝脏出现多个囊性病变。它是一种罕见的常染色体显性疾病。囊肿直径从20至30厘米到微小的显微镜下可见的结节不等。一般来说,超过一半的肝实质被覆盖。大囊肿对肝脏造成的占位效应可导致危及生命的症状,如体重减轻、口服摄入量减少和营养不良。肝移植是有症状患者的最佳治疗选择。我们报告一名患有多囊肝和多囊肾病的患者,因其出现危及生命的症状而接受了肝移植。