Urits Ivan, Li Nathan, Berger Amnon A, Walker Paul, Wesp Brendan, Zamarripa Alec M, An Daniel, Cornett Elyse M, Abd-Elsayed Alaa, Kaye Alan D
Beth Israel Deaconess Medical Center, Department of Anesthesia, Critical Care, and Pain Medicine, Harvard Medical School, 330 Brookline Ave, Boston, MA, 02215, USA.
Department of Anesthesiology, Louisiana State University Health Shreveport, Shreveport, LA, USA.
Curr Pain Headache Rep. 2021 Jan 25;25(1):6. doi: 10.1007/s11916-020-00925-0.
Loin pain hematuria syndrome (LPHS) is rare and seldom diagnosed, yet it has a particularly significant impact on those affected. This is a review of the latest and seminal evidence of the pathophysiology and diagnosis of LPHS and presents the typical clinical presentation and treatment options available.
LPHS is typically found in young women with characteristic symptoms, including severe recurrent flank pain and gross or microscopic hematuria. The majority of patients will experience crippling pain for many years without effective therapy, often requiring frequent use of narcotic medication. However, the lack of conclusive pathophysiology, in conjunction with the rarity of LPHS, has prohibited the development and trial of definitive treatment options. Nevertheless, in order to combat this rare but severe disease, management strategies have continued to evolve, ranging from conservative measures to invasive procedures. This review presents an overview of the current hypotheses on the pathophysiology of LPHS in addition to summarizing the management strategies that have been utilized. Only 30% of LPHS patients will experience spontaneous resolution, whereas the majority will continue to face chronic, crippling pain. Several methods of treatment, including invasive and non-invasive, may provide an improved outcome to these patients. Treatment should be individually tailored and multi-disciplinary in nature. Further research is required to further elucidate the pathophysiology and develop new, specific, treatment options.
腰腹痛血尿综合征（LPHS）较为罕见，很少得到诊断，但对患者影响尤为显著。本文回顾了LPHS病理生理学和诊断方面的最新及重要证据，并介绍了其典型临床表现和可用的治疗选择。
LPHS多见于有特征性症状的年轻女性，包括严重的复发性胁腹痛和肉眼或镜下血尿。大多数患者多年来饱受剧痛折磨却无有效治疗方法，常需频繁使用麻醉药物。然而，由于缺乏确凿的病理生理学依据，加上LPHS罕见，阻碍了确定性治疗方案的开发和试验。尽管如此，为对抗这种罕见但严重的疾病，管理策略不断发展，从保守措施到侵入性手术都有。本综述除了总结已采用的管理策略外，还概述了目前关于LPHS病理生理学的假说。只有30%的LPHS患者会自发缓解，而大多数患者将继续面临慢性剧痛。包括侵入性和非侵入性在内的几种治疗方法可能会改善这些患者的预后。治疗应个体化且具有多学科性质。需要进一步研究以进一步阐明病理生理学并开发新的、特定的治疗方案。
