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腰痛-血尿综合征

Authors
Lee A Hebert, MD
Costantino Benedetti, MD
Samir V Parikh, MD
Tibor Nadasdy, MD
Section Editors
Richard J Glassock, MD, MACP
Brad H Rovin, MD
Deputy Editor
John P Forman, MD, MSc
Translators
解倩, 副主任医师

引言

腰痛-血尿综合征(loin pain-hematuria syndrome, LPHS)于1967年在一份报告中第一次被描述，该报告包括3例具有反复发作的单侧或双侧严重腰痛并伴有肉眼血尿或镜下血尿的年轻女性患者(20-28岁)[1]。这3例患者并不没有引起腰痛和血尿的主要原因(如，肾结石和血块)(参见下文‘诊断’)。肾动脉造影提示肾脏皮质灌注局灶性受损，而肾活检显示间质纤维化和动脉硬化。

自从第一份报告发表以来，医学文献已经报告了几百例LPHS[2]。

流行病学

LPHS的流行病学尚不完全清楚。我们的200多例患者中大概有一半住在俄亥俄州中部，该地区有180万人。如果我们的患者占该区域LPHS患者的一半，则LPHS的患病率约为0.012%。根据2002年罕见疾病法案(众议院4013号决议)，该患病率决定了LPHS是一种罕见疾病(患病率低于0.07%)。

临床特征

LPHS是一种定义不明的疾病，其特征为腰痛和血尿反复或持续发作，而血尿似乎反映了肾小球性出血。大多数患者存在这两种表现，但有些患者仅有腰痛或仅有血尿[3]。极少数情况下，疼痛发作伴有低热和排尿困难，但无泌尿道感染[3]。

受累患者通常为年轻女性，几乎全部都是白人；一项回顾性研究中，患者的平均年龄为31岁，部分病例为13-18岁的儿童；大多数病例系列研究中女性患者占了70%-80%[2-4]。根据排石史或影像学检查显示结石典型特征肾脏钙化，多达50%的LPHS患者有肾结石[2]。

                                         

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Literature review current through: 2017-06 . | This topic last updated: 2016-12-22.
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