Hebert L A, Betts J A, Sedmak D D, Cosio F G, Bay W H, Carlton S
Department of Internal Medicine, Ohio State University, Columbus, USA.
Kidney Int. 1996 Jan;49(1):168-73. doi: 10.1038/ki.1996.23.
Loin pain-hematuria (LPH) syndrome is a poorly understood disorder in which the patients, mainly young women, experience unexplained severe chronic unilateral or bilateral flank pain associated with gross and/or microscopic hematuria. By contrast, thin glomerular basement membrane (GBM) disease is generally thought to be a benign disorder, affecting males and females equally, in which the major manifestation is asymptomatic microscopic hematuria. Herein we describe seven patients (6 females, 1 male) in whom thin GBM appeared to be the cause of the LPH syndrome. The gross hematuria in these patients could be attributed to thin GBM disease because the renal biopsy demonstrated red cells in renal tubules (indicating glomerular hematuria) and the only glomerular abnormality present with thin GBM. In addition, the other causes of gross hematuria were excluded by appropriate testing. The flank pain in these patients might also have been the result of their thin GBM disease. This is suggested by renal biopsy findings of multiple renal tubules filled with red cells, apparently occluding the tubules. We suggest that occlusion of a relatively small fraction of renal tubules could cause renal pain if back-leak of glomerular filtrate occurred that was of sufficient magnitude to expand renal parenchymal volume and stretch the renal capsule. Preliminary observations suggest that treatment with the angiotensin converting enzyme (ACE) inhibitor enalapril importantly reduces the frequency and severity of the episodes of gross hematuria and flank pain in most patients. ACE inhibition might decrease glomerular hemorrhage in patients with think GBM by decreasing glomerular hydrostatic pressure. We conclude that (1) Thin GBM disease can be the cause of gross hematuria, apparently as a result of rupture of thin GBM. (2) Rupture of thin GBM resulting in hemorrhage into renal tubules may be the cause of the flank pain and gross hematuria in some patients with the LPH syndrome.
腰背痛-血尿（LPH）综合征是一种了解甚少的疾病，患者主要为年轻女性，会出现原因不明的严重慢性单侧或双侧胁腹痛，并伴有肉眼血尿和/或镜下血尿。相比之下，薄肾小球基底膜（GBM）病通常被认为是一种良性疾病，男女均可发病，主要表现为无症状镜下血尿。在此，我们描述了7例患者（6例女性，1例男性），薄GBM似乎是其LPH综合征的病因。这些患者的肉眼血尿可归因于薄GBM病，因为肾活检显示肾小管中有红细胞（提示肾小球性血尿），且是薄GBM存在的唯一肾小球异常。此外，通过适当检测排除了肉眼血尿的其他病因。这些患者的胁腹痛也可能是薄GBM病所致。肾活检发现多个肾小管充满红细胞，显然阻塞了肾小管，提示了这一点。我们认为，如果肾小球滤过液发生足够量的回漏，导致肾实质体积增大并拉伸肾包膜，那么相对少量肾小管的阻塞可能会引起肾痛。初步观察表明，用血管紧张素转换酶（ACE）抑制剂依那普利治疗可显著降低大多数患者肉眼血尿发作的频率和严重程度以及胁腹痛。ACE抑制可能通过降低肾小球静水压来减少薄GBM患者的肾小球出血。我们得出结论：（1）薄GBM病可能是肉眼血尿的病因，显然是薄GBM破裂所致。（2）薄GBM破裂导致肾小管出血可能是某些LPH综合征患者胁腹痛和肉眼血尿的病因。
