Green A, Allos M, Donohoe J, Carmody M, Walshe J
Department of Nephrology and Transplantation, Beaumont Hospital, Dublin.
Ir Med J. 1990 Mar;83(1):11-3.
Little information is available concerning hereditary renal disease in the Irish population. We studied the prevalence and types of hereditary renal disease that lead to end stage renal failure by looking at the underlying hereditary disease in our dialysis populations and in all patients transplanted at the national unit. Twenty eight (15.7%) of 178 dialysis patients had hereditary renal disease with a mean age of 31 years (range nine to 65), and a male to female ratio of 1.54:1. Eighty-nine of 842 patients (10.3%) with renal transplants had hereditary renal disease with a male to female ratio of 1.63:1. The commonest entity was autosomal dominant polycystic kidney disease (68% in dialysis patients, 64% in transplants), and the next most frequent was Alport's syndrome (21.4%, 16.9%). Nephronophthisis was the third most common problem (10.7%, 10.1%) followed by hereditary non-Alport's nephritis, Fabry's disease, cystinosis and familial interstitial nephritis. The high prevalence of hereditary renal disease among the dialysis and renal transplant population emphasises the need for proper registration of the nature of renal disease in this group, and for a comprehensive genetic counselling service in this country.
关于爱尔兰人群中的遗传性肾病，目前可获得的信息较少。我们通过研究透析人群以及在国家肾脏移植中心接受移植的所有患者的潜在遗传性疾病，来探讨导致终末期肾衰竭的遗传性肾病的患病率和类型。178名透析患者中有28名（15.7%）患有遗传性肾病，平均年龄为31岁（9至65岁），男女比例为1.54:1。842名肾移植患者中有89名（10.3%）患有遗传性肾病，男女比例为1.63:1。最常见的类型是常染色体显性多囊肾病（透析患者中占68%，移植患者中占64%），其次是阿尔波特综合征（21.4%，16.9%）。肾单位肾痨是第三常见的问题（10.7%，10.1%），其次是遗传性非阿尔波特肾炎、法布里病、胱氨酸病和家族性间质性肾炎。透析和肾移植人群中遗传性肾病的高患病率强调了对该组肾脏疾病性质进行适当登记以及在该国提供全面遗传咨询服务的必要性。
