成人型多囊肾病多器官损害205例临床分析

为了解成人型多囊肾病的遗传性疾病的情况，分析了我院２０５例成人型多囊肾病患者的临床表现，男性１０７例，女性９８例，年龄１０～７１岁。２０５例患者来自１８０个家系，其中１３８例有确切的家族史。本病多器官受损表现结果为：双肾多发性囊肿为１００％、多囊肝（１１９例，占５８％）、胰腺囊肿２例、脾脏囊肿３例、心脏瓣膜病变２５例、颅内动脉瘤３例及卵巢囊肿３例等。

SYSTEMIC MANIFESTATIONS OF ADULT POLYCYSTIC KIDNEY DISEASE: AN ANALYSIS OF 205CASES

dult polycystic kidney disease (APKD)is a common genetic disease and one of the important reasons of end stage renal failure.Although renal multiple cysts are clearly an important manifestation of APKD;other systemic manifestations are both common and clinically important.The authors reviewed 205 cases from 180 APKD families(107 male 98 female).Their age ranged from 10 to 71 years. Renal cyst is one of the many renal manifestations.Hypertension,hematurla and flank pain are its major complications, Hep-atic cysts,pancreatic cysts,cardiac valvular lesions, intracranial aneurysms and splenic cysts are included in the array of svstemic manifestations.