局灶节段性肾小球硬化的病理生理学与组织病理学新进展

Update in pathophysiology and histopathology of focal segmental glomerulosclerosis.

作者信息

Kanjanabuch Talerngsak, Lewsuwan Songkiat, Kitiyakara Chagriya, Cheunsuchon Boonyarit, Eiam-Ong Somchai

机构信息

Division of Nephrology, Department of Medicine, Faculty of Medicine, Chulalongkorn University Hospital, Bangkok, Thailand.

出版信息

J Med Assoc Thai. 2006 Aug;89 Suppl 2:S262-79.

PMID:17044482

Abstract

Focal segmental glomerulosclerosis (FSGS) is the leading cause of nephrotic syndrome in an adult worldwide. The prevalence of FSGS is estimated as being 20-30% in adults over the age of 15 years and slightly higher (30-35%) in the elderly (age > 60 years). The diagnosis solely relies on pathologic findings, which sclerosis involves some, but not all glomeruli (focal), and sclerosis affects a portion, but not the entire, glomerular tuft (segmental). The pathogenesis remains inconclusive but podocyte injury has been postulated. Even though steroid is the mainstay treatment, only 20-40% of patients are complete respond.

摘要

局灶节段性肾小球硬化（FSGS）是全球成年人肾病综合征的主要病因。据估计，15岁以上成年人中FSGS的患病率为20%-30%，老年人（年龄>60岁）中患病率略高（30%-35%）。诊断仅依靠病理结果，即硬化累及部分而非全部肾小球（局灶性），且硬化影响部分而非整个肾小球小叶（节段性）。发病机制尚无定论，但推测与足细胞损伤有关。尽管类固醇是主要治疗方法，但只有20%-40%的患者能完全缓解。