密西西比州终末期肾病的家族聚集。

Donald E Butkus
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引用次数: 0

摘要

背景:在 20-40% 的非裔美国人 (AA) ESRD 患者家族中发现了 ESRD 家族聚集现象,但在高加索人 (Cau) 中却很少观察到。由于我们的印象表明高加索人的发病率高于最近几项研究的报告,因此我们对转诊的肾移植患者进行了为期两年的前瞻性评估,以评估高加索人和非洲裔美国人ESRD家族聚集的频率:对 350 名转诊接受肾移植的 ESRD 患者进行了详细的家族病史调查,其中 335 名患者可以提供有关其一等和二等家庭成员(252AA 和 83 Cau)的适当信息,这些家庭成员至少在门诊透析维持了一个月或接受过肾移植:结果:患者的种族和性别与目前密西西比州 ESRD 患者的种族和性别相同,但移植候选者的平均年龄要小 17 岁(39.9 岁对 57.2 岁)。如果将多囊肾病(PKD)纳入分析,31.7% 的 AA 和 30.1% 的 Cau 至少有一名其他家庭成员患有 ESRD。如果剔除 PKD,则 30.9% 的 AA 和 25% 的 Cau 至少有一名其他家庭成员患有 ESRD。在这两个种族中,慢性肾小球肾炎指数病例中出现阳性家族史的频率最高,在进行肾活检的病例(n = 59)中,局灶节段性肾小球硬化症(FSGS)和系统性红斑狼疮(SLE)患者的ESRD家族史最丰富(分别为40%和36%)。患有FSGS的Cau人和AA人的ESRD家族聚集程度相当(41%对38%),但系统性红斑狼疮仅限于AA人:结论:在较年轻的 ESRD 患者中,白种人和非裔美国人发生终末期肾病家族聚集的频率几乎相同。对这两个种族的指数病例的家庭成员进行筛查,可能会确定一个高危患者群体,可针对该群体采取预防措施,以阻止肾衰竭的发展[表:见正文]。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Familial clustering of end-stage renal disease in Mississippi.

Background: Familial clustering of ESRD has been noted in the families of 20-40% of African-Americans (AA) with ESRD but has been observed much less often in Caucasians (Cau). Because our impression indicated a higher frequency in Caucasians than reported in several recent studies we conducted a 2-year prospective evaluation of patients referred for renal transplantation to assess the frequency of familial clustering of ESRD in both Caucasians and African-Americans.

Methods: Detailed family histories were obtained in 350 ESRD patients referred for renal transplantation, 335 of whom could provide appropriate information regarding first- and second-degree family members (252AA and 83 Cau) who were maintained on out-patient dialysis at least one month or had received a renal transplant.

Results: Patient race and sex paralleled that of the current Mississippi ESRD population but mean age was 17 years younger in the transplant candidates (39.9 vs. 57.2 yrs). If polycystic kidney disease (PKD) is included in the analysis, 31.7% of AA and 30.1% of Cau had at least one other family member with ESRD. If PKD is eliminated then 30.9% of AA and 25% of Cau had at least one other family member with ESRD. In both races the frequency of a positive family history was greatest in index cases with chronic glomerulonephritis, and of those with renal biopsies (n = 59), patients with focal segmental glomerulosclerosis (FSGS) and systemic lupus erythematosus (SLE) had the strongest family histories of ESRD (40% and 36%, respectively). Cau and AA with FSGS had comparable familial clustering of ESRD (41% vs, 38%) but SLE was confined to AA.

Conclusions: In younger individuals with ESRD, familial clustering of end stage renal disease occurs with almost equal frequency in Caucasians and in African Americans. Screening of family members of index cases in both races might define a high-risk group of patients in whom prophylactic measures might be directed to thwart the progression [table: see text] of renal failure.

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