型胶原纤维肾小球病的临床及病理特征分析

目的：分析Ⅲ型胶原纤维肾小球病患者的临床和病理资料,提升该病诊治水平。方法：回顾性分析2007年3月至2015年6月肾活检患者的病例资料,进一步分析其中Ⅲ型胶原纤维肾小球病患者的临床和病理资料。结果：共4例Ⅲ型胶原纤维肾小球病患者,其中2例男性,2例女性;年龄（45.5±10.7）岁。4例患者均无家族史,均无肾外累及。就诊时最常见临床表现为蛋白尿、高血压、肾功能不全。肾活检病理改变多样,可表现为膜增生样改变、结节性病变、局灶性节段性肾小球硬化;其中1例同时伴有IgA肾病。1例患者肾功能轻度减退伴少量蛋白尿,肾功能及尿蛋白定量长期稳定;2例患者糖皮质激素联合免疫制剂治疗有效,肾功能较前好转,蛋白...

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Published in中国临床医学 Vol. 24; no. 4; pp. 582 - 586
Main Author 龚劭敏 骆伟丽 金是 刘学光 丁小强 刘红
Format Journal Article
LanguageChinese
Published 复旦大学附属中山医院肾内科,上海市肾病与透析研究所,上海市肾脏疾病与血液净化重点实验室,上海 200032%复旦大学基础医学院病理学系,上海,200032 2017
Subjects
型胶原纤维肾小球病
治疗
肾组织病理
治疗
treatment
肾组织病理
型胶原纤维肾小球病
renal histopathology
collagen type Ⅲ glomerualopathy
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ISSN1008-6358
DOI10.12025/j.issn.1008-6358.2017.20170140

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Summary:目的：分析Ⅲ型胶原纤维肾小球病患者的临床和病理资料,提升该病诊治水平。方法：回顾性分析2007年3月至2015年6月肾活检患者的病例资料,进一步分析其中Ⅲ型胶原纤维肾小球病患者的临床和病理资料。结果：共4例Ⅲ型胶原纤维肾小球病患者,其中2例男性,2例女性;年龄（45.5±10.7）岁。4例患者均无家族史,均无肾外累及。就诊时最常见临床表现为蛋白尿、高血压、肾功能不全。肾活检病理改变多样,可表现为膜增生样改变、结节性病变、局灶性节段性肾小球硬化;其中1例同时伴有IgA肾病。1例患者肾功能轻度减退伴少量蛋白尿,肾功能及尿蛋白定量长期稳定;2例患者糖皮质激素联合免疫制剂治疗有效,肾功能较前好转,蛋白尿部分缓解;1例患者进展至终末期肾病,接受血液透析治疗。结论：Ⅲ型胶原纤维肾小球病呈散发性,可表现为蛋白尿、高血压、肾功能减退;肾活检光镜病理表现多样,容易漏诊;免疫组织化学和电镜检查有助于确诊。
Bibliography:31-1794/R
Objective：To provide basis for the diagnosis and treatment of collagen type Ⅲ glomerualopathy by analyzing the clinical and pathological data of patients.Methods：After searching the data of the patients receiving renal biopsy from March 2007 to June 2015 in nephrology department,the clinical and pathological features of patients who were proven as collagen type Ⅲ glomerualopathy were analyzed.Results：Two male and two female patients aged（45.5±10.7） years old were included.None of the patients were reported to have positive familial history of any kidney diseases.None of them were found to have extra renal involvement.The most common clinical manifestations were proteinuria,hypertension and impaired renal function.Membrane proliferative glomerulonephritis,nodular glomerulosclerosis,focal segmental proliferative and sclerosis glomerulonephritis were found under the light microscopy.One of the patients was accompanied by IgA nephropathy.As for the follow-up results,1 case with minor declined eGFR and pr
ISSN:1008-6358
DOI:10.12025/j.issn.1008-6358.2017.20170140