Edwin M Spithoven, Niek F Casteleijn, Paul Berger, Roel Goldschmeding
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引用次数: 2
摘要
常染色体显性多囊肾病(ADPKD)是最常见的遗传性肾脏疾病。其特点是双肾进行性囊肿形成，常导致终末期肾病。手术切除ADPKD肾的适应症包括顽固性疼痛、血尿、感染或异常肿大和腹腔小妨碍供体肾脏植入。我们报告一个异常大的ADPKD肾脏，重8.7 kg (19.3 lb)，最大长度48 cm(19英寸)，囊肿充满透明和血性液体。
Nephrectomy in autosomal dominant polycystic kidney disease: a patient with exceptionally large, still functioning kidneys.
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary kidney disease. It is characterized by progressive cyst formation in both kidneys, often leading to end-stage kidney disease. Indications for surgical removal of an ADPKD kidney include intractable pain, hematuria, infection, or exceptional enlargement and small abdominal cavity hampering implantation of a donor kidney. We report the case of an extraordinarily large ADPKD kidney weighing 8.7 kg (19.3 lb) with a maximal length of 48 cm (19 inch), and with cysts filled with both clear and bloody fluid.
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