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成人多囊肾病(APKD)是常染色体显性遗传性疾病,其表现各样,可从出生时即出现囊性肾直至>80岁大多数患者常于50-60岁发现,约有25%发展至终未期肾病(ESRD)。作者对136例APKD患者作回顾性研究。APKD的诊断,为尿路造影或超声检查证实双侧有多囊改变。并有常染色体显性遗传相关疾病的家族史。在无症状患者,每个肾至少有3个囊,方可诊断APKD。平均随访6年3个月(范围2～10年)。37例患者在失去随访前观察2～9年(平均4年9个月)。慢性肾衰(CRF)为血清肌酐(Scr)>1.5mg/dl。所有患者从出生起都有发展至ESRD的危险,2例死于CRF。高血压为收缩压或舒张压分别>150mmHg或100mmHg。结果:107例患者(58例男性,49例女性)发病率
Adult polycystic kidney disease (APKD) is an autosomal dominant genetic disease that manifests itself in various manifestations from the time of presentation of the cystic kidney at birth up to the age of 80 Most patients 50-60 years of age are commonly found and about 25% develop End-stage renal disease (ESRD). The authors retrospectively studied 136 APKD patients. The diagnosis of APKD, for urography or ultrasound confirmed bilateral changes in the capsule. And has a family history of autosomal dominant genetic related diseases. In asymptomatic patients, each kidney has at least 3 sacs to diagnose APKD. The average follow-up of 6 years and 3 months (range 2 to 10 years). Thirty-seven patients were observed 2 to 9 years (mean, 4 years and 9 months) before losing follow-up. Chronic renal failure (CRF) is serum creatinine (Scr)> 1.5 mg / dl. All patients had the risk of developing ESRD from birth and 2 died of CRF. Hypertension systolic or diastolic blood pressure were> 150mmHg or 100mmHg. Results: The incidence of 107 patients (58 males and 49 females)