长期二硫化碳暴露所致肾损害9例临床病理特征及超微结构分析

Clinicopathological features and ultrastructure analysis of renal damage caused by long-term carbon disulfide exposure in nine cases

探讨9例长期二硫化碳暴露所致肾损害的临床病理特征,分析其病因及预后。回顾性分析9例长期二硫化碳暴露所致肾损害的临床病理资料,总结其临床特征、病理类型、免疫组化及电镜特点,并复习相关文献。9例患者病理改变相似,但病变程度不同。9例均表现为结节状系膜基质增生伴系膜细胞增生,其中4例伴"类K-W结节"形成;6例伴基底膜弥漫性增厚、局灶节段硬化或球性硬化;4例伴新月体形成(其中纤维性新月体2例、细胞性新月体2例);1例肾小球弥漫球性增生硬化。9例均有不同程度肾小管萎缩/间质纤维化伴慢性炎症细胞浸润,以及肾小球动脉硬化。9例IgA、IgM、C3d、C4d、C1q及Fib等免疫组化均无特异性表达,IgG、Ⅲ型胶原、Fibronectin、Amyloid A、κ、λ、HBsAg及HBcAg均阴性。9例中7例行电镜检查,超微结构显示2例见肾小球呈结节状球性硬化,2例在节段硬化区周围见少量高电子致密物沉积,其余病例节段硬化的肾小球均未见明显电子致密物沉积,也无其他特殊改变;各病例肾小管及肾间质均无特征性改变。结合光镜及电镜检查结果,9例患者最终诊断为特发性弥漫结节状系膜增生硬化性肾病,该病以肾小球弥漫结节状系膜增生硬化性伴"类K-W结节"形成为特征,该类病变可能是长期二硫化碳暴露所致肾损害的一种特殊的病理特征,我们推测该病变可能是肾脏疾病病理改变的一种新的类型。

To investigate the clinicopathological features and prognosis of nine cases of renal damage caused by long-term carbon disulfide exposure.The clinicopathological data of nine cases of renal injury caused by long-term carbon disulfide exposure were retrospectively analyzed,and the clinical features,pathological types,immunohistochemical and electron microscopic characteristics were summarized,and related literatures were reviewed.The pathological changes of nine patients were similar,but the degree of pathological changes was different.All nine cases presented nodular mesangial matrix hyperplasia accompanied by mesangial cell proliferation,and four cases were accompanied by"K-W-like nodules".Six cases had diffuse thickening,focal segmental hardening or spherical hardening of basement membrane.Four cases were associated with crescent formation,including two cases of fibrous crescent and two cases of cellular crescent.Diffuse bulbous hyperplasia and sclerosis of glomeruli in one case.All nine patients had varying degrees of renal tubular atrophy/interstitial fibrosis with chronic inflammatory cell infiltration and glomerular arteriosclerosis.There was no specific expression of IgA,IgM,C3 d,C4 d,C1 q and Fib in nine cases.IgG,typeⅲcollagen,Fibronectin,Amyloid A,κ,λ,HBsAg and HBcAg were negative.seven of the nine cases were examined by electron microscopy,and the ultrastructure showed nodular glomerular sclerosis in two cases,a small amount of high electron dense deposits around segmental sclerosis in two cases,and no obvious electron dense deposits in segmental sclerosis glomerular segments in the rest of the cases,and no other special changes.There were no characteristic changes in renal tubules and interstitium in all cases.In combination with light microscope and electron microscope examination results,nine patients were eventually diagnosed with idiopathic diffuse nodular hyperplasia of mesangial sclerosing kidney disease,the disease to diffuse glomerular nodular hyperplasia of mesangial sclerosis"K-W-like nodules"form,characterized by such lesions may be caused by long-term exposure to carbon disulfide a special pathological features of renal damage,We hypothesized that this lesion might be a new type of renal pathology.