Praga M, Martínez M A, Andrés A, Alegre R, Vara J, Morales E, Herrero J C, Novo O, Rodicio J L
Department of Nephrology, Hospital Universitario 12 de Octubre, Madrid, Spain.
Kidney Int. 1998 Sep;54(3):915-20. doi: 10.1046/j.1523-1755.1998.00065.x.
Familial persistent microhematuria with normal renal function is the most common presentation of thin basement membrane nephropathy (TBMN). Gross hematuria episodes and loin pain attacks are other manifestations of the disease. On the other hand, it has been shown that hypercalciuria (HC) and hyperuricosuria (HU) can produce both gross or microscopic non-glomerular hematuria, in addition to their role in renal stone formation.
We studied the prevalence of HC, HU and nephrolithiasis in a group of 27 biopsy-proven TBMN as well as in 19 non-biopsied first-degree relatives with persistent microhematuria and 25 first-degree relatives without microhematuria. A group of 27 patients with IgA nephropathy (IgAN) and persistent microhematuria, and another group of 20 healthy subjects without known renal diseases were selected as control groups.
Ten (37%) patients with TBMN and 8 (42%) relatives with microhematuria showed HC and/or HU at presentation; relatives without microhematuria, IgAN patients and normal controls showed a significantly lower prevalence of HC and HU. The prevalence of previous nephrolithiasis among TBMN patients (25%) was significantly higher than in IgAN patients (3%; P < 0.05). Family history of nephrolithiasis was recorded in 14 (51%) of the 27 TBMN families, in contrast with 2 of 27 (7%) with IgAN and 1 of 20 (5%) in normal controls (P < 0.05). The prevalence of nephrolithiasis, gross hematuria bouts and loin pain episodes among TBMN patients and microhematuric relatives showing HC and/or HU at presentation (44%, 44% and 27%, respectively) were significantly higher than those of TBMN patients and microhematuric relatives with normal calcium and uric acid urinary excretions (10%, 7% and 3%, respectively; P < 0.05). At the end of follow-up (8.8+/-4.1 years in TBMN patients and 9.1+/-4.2 years in relatives with microhematuria), all the cases maintained normal renal function.
We found a high prevalence of HC, HU, and nephrolithiasis among TBMN patients and relatives with microhematuria. Our study also shows a significant relationship between the presence of HC and/or HU and the prevalence of nephrolithiasis, gross hematuria bouts and loin pain episodes.
家族性持续性镜下血尿且肾功能正常是薄基底膜肾病(TBMN)最常见的表现。肉眼血尿发作和腰痛发作是该疾病的其他表现。另一方面,研究表明高钙尿症(HC)和高尿酸尿症(HU)除了在肾结石形成中起作用外,还可导致肉眼或镜下非肾小球性血尿。
我们研究了27例经活检证实的TBMN患者、19例有持续性镜下血尿的未活检一级亲属以及25例无镜下血尿的一级亲属中HC、HU和肾结石的患病率。选取27例IgA肾病(IgAN)伴持续性镜下血尿患者和另一组20例无已知肾脏疾病的健康受试者作为对照组。
10例(37%)TBMN患者和8例(42%)有镜下血尿的亲属在就诊时表现出HC和/或HU;无镜下血尿的亲属、IgAN患者和正常对照组中HC和HU的患病率显著较低。TBMN患者中既往有肾结石的患病率(25%)显著高于IgAN患者(3%;P<0.05)。27个TBMN家族中有14个(51%)有肾结石家族史,相比之下,27个IgAN家族中有2个(7%),20个正常对照组中有1个(5%)(P<0.05)。就诊时表现出HC和/或HU的TBMN患者和镜下血尿亲属中肾结石、肉眼血尿发作和腰痛发作的患病率(分别为44%、44%和27%)显著高于钙和尿酸排泄正常的TBMN患者和镜下血尿亲属(分别为10%、7%和3%;P<0.05)。随访结束时(TBMN患者为8.8±4.1年,有镜下血尿的亲属为9.1±4.2年),所有病例肾功能均正常。
我们发现TBMN患者和有镜下血尿的亲属中HC、HU和肾结石的患病率较高。我们的研究还表明HC和/或HU的存在与肾结石、肉眼血尿发作和腰痛发作的患病率之间存在显著关系。