Leaker B R, Gordge M P, Patel A, Neild G H
Department of Nephrology, University College and Middlesex School of Medicine, London.
Q J Med. 1990 Sep;76(281):969-79.
Twenty-five patients (seven male, 18 female) were diagnosed as having the loin pain and haematuria syndrome. Presenting symptoms were either loin pain alone or pain associated with macroscopic or microscopic haematuria, and were longstanding, having been present for mean of 9.3 years in males, and 10 years in females. Ten patients described symptoms of passing gravel or renal stones but these were only demonstrated radiologically in two patients. Investigation of all patients showed anatomically normal renal tracts, normal renal function, and no significant proteinuria. Phase-contrast microscopy during episodes of haematuria revealed dysmorphic red cells in all 10 patients studied. Renal biopsies were performed in 20 patients and showed no glomerular pathology, but arteriolar and arterial hyalinosis was seen in 13 of 20 (65 per cent), fibro-elastosis in larger vessels in eight of 20 (40 per cent) and red blood cells in tubules in 13 of 20 (65 per cent) patients. The histological appearance in vessels was similar to that seen in cyclosporin A nephrotoxicity and would be consistent with the hypothesis that regional vasospasm occurs in the cortical circulation. Haematological studies in 22 patients, when compared with age and sex matched controls, showed the presence of circulating platelet aggregates, elevation of plasma beta-thromboglobulin (p less than 0.001), and increased platelet aggregation in response to serotonin and ADP (p less than 0.05 and p less than 0.03, respectively). Plasma concentrations of D dimer (p less than 0.02) and C-reactive protein (p less than 0.03) were also significantly elevated in the patient group. There was no deterioration of renal function during a mean observation period of 3.7 years and no patients developed proteinuria. Treatment was largely supportive; seven patients with intractable loin pain underwent surgical denervation with the relief of pain in four.
25例患者（7例男性，18例女性）被诊断为患有腰痛血尿综合征。主要症状为单纯腰痛或伴有肉眼血尿或镜下血尿的疼痛，且症状持续时间长，男性平均存在9.3年，女性平均存在10年。10例患者描述有排出沙砾样物质或肾结石的症状，但仅2例经放射学检查证实。对所有患者的检查显示尿路解剖结构正常、肾功能正常且无明显蛋白尿。血尿发作期间的相差显微镜检查显示，所研究的10例患者均有异形红细胞。20例患者进行了肾活检，结果显示无肾小球病变，但20例中有13例（65%）可见小动脉和动脉玻璃样变性，20例中有8例（40%）较大血管出现纤维弹性组织增生，20例中有13例（65%）患者肾小管内可见红细胞。血管的组织学表现与环孢素A肾毒性所见相似，这与皮质循环中发生局部血管痉挛的假说相符。与年龄和性别匹配的对照组相比，22例患者的血液学研究显示存在循环血小板聚集、血浆β-血小板球蛋白升高（p<0.001），以及对5-羟色胺和二磷酸腺苷的血小板聚集增加（分别为p<0.05和p<0.03）。患者组中D-二聚体（p<0.02）和C反应蛋白（p<0.03）的血浆浓度也显著升高。在平均3.7年的观察期内肾功能无恶化，且无患者出现蛋白尿。治疗主要是支持性的；7例顽固性腰痛患者接受了手术去神经支配，4例疼痛缓解。
