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常染色体显性多囊肾病的肾外表现

[Extra-renal manifestations of the autosomal dominant polycystic kidney disease].

作者信息

Butscher Aurélie, Phan Olivier, Bonny Olivier

机构信息

Service de néphrologie, Département de médecine, CHUV, 1011 Lausanne.

Département de pharmacologie et de toxicologie, Université de Lausanne, 1011 Lausanne.

出版信息

Rev Med Suisse. 2017 Feb 22;13(551):450-456.

Abstract

Extra-renal complications of autosomal dominant polycystic kidney disease (ADPKD) are often unrecognized. Liver cysts are usually benign, but may cause chronic pain, may interfere with nutrition and may necessitate partial liver resection or even liver transplant. Somatostatin analogs may be an alternative therapy. Cerebral aneurisms are rare, but devastating. They must be investigated in case of positive familial history. Cardiac valvulopathies will be searched for by echocardiography. Abdominal wall herniae are often identified during the physical exam in ADPKD patients. ADPKD patients may present with higher incidence of diverticulitis. Cysts of the seminal vesicle are frequent but are usually asymptomatic. Recognizing extra-renal complications helps improving the overall survival of ADPKD patients.

摘要

常染色体显性多囊肾病(ADPKD)的肾外并发症往往未被识别。肝囊肿通常是良性的,但可能引起慢性疼痛,可能干扰营养状况,可能需要进行部分肝切除甚至肝移植。生长抑素类似物可能是一种替代治疗方法。脑动脉瘤很少见,但具有破坏性。如果家族史呈阳性,必须对其进行检查。心脏瓣膜病将通过超声心动图进行排查。在ADPKD患者的体格检查中常可发现腹壁疝。ADPKD患者憩室炎的发病率可能较高。精囊囊肿很常见,但通常无症状。认识肾外并发症有助于提高ADPKD患者的总体生存率。

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