成人肾小球微小病变的临床病理研究

发布时间：2018-07-08 14:15

本文选题：肾小球微小病变 + 肾病综合征　；参考：《上海交通大学》2014年硕士论文

【摘要】：肾小球微小病变（minimal change disease，MCD）是肾病综合征（NS）常见的一种病理类型，约70%~90%小于10岁的儿童NS、约50%10岁以上的儿童NS为MCD，是儿童NS最常见病理类型。成人MCD的发病率较儿童低，报道约10%-37.4%的成人NS为MCD。与儿童MCD相比，有关成人尤其中国成人MCD的临床病理特点的报道远远要少，对于成人MCD，还有很多方面未被认识，也不明确中国成人MCD的临床病理表现与其他种族的报道是否相似。本文收集了上海交通大学医学院附属瑞金医院肾脏科2009年1月至2013年6月间临床符合NS肾活检病理诊断为MCD、年龄18岁的162例成人患者的病例资料和随访数据，结果显示162例患者，男性98例，女性64例，男女比例1.53:1，平均年龄33.8±14.8岁（18~76岁），平均发病年龄33.114.7岁，男性明显早于女性患者，18-30岁年龄段的患者占总数的54.3%，男性患者主要集中在18-30岁，高达66.3%，女性患者主要分布在18-30岁及31-40岁，所占比例分别为35.9%和31.3%。162例患者均表现为不同程度的水肿、大量蛋白尿、低白蛋白血症伴高脂血症，47例（29.0%）患者发病时存在不同诱因，以感染最为常见（61.7%），仅17.9%的患者尿蛋白定量4g/24h，45.7%的患者尿蛋白定量介于4-8g/24h，36.4%的患者尿蛋白定量8g/24h。28.4%的患者伴有镜下血尿，17.3%的患者血清肌酐115umol/L，以MDRD公式估算肾小球滤过率（eGFR），31.5%的患者eGFR在60-90ml/min/1.73m2，13.6%和8.0%的患者eGFR为30-60ml/min/1.73m2和30ml/min/1.73m2，而以EPI公式计算，15.4%的患者eGFR介于60-90ml/min/1.73m2，4.9%和8.0%的患者EPI-eGFR介于30-60ml/min/1.73m2和30ml/min/1.73m2，60岁以上eGFR均90ml/min/1.73m2，随着年龄的增高肾功能受累的比例越高。10例（6.2%）患者合并高血压病，2例合并2型糖尿病，其中1例患者为糖尿病合并高血压。18-30岁与31-60岁年龄组、初发和复发组、激素敏感和激素依赖组相比，，两组在24h尿蛋白定量、血浆白蛋白、肾功能水平方面均未发现统计学差异。对162例成人MCD肾组织病理改变进行统计发现，肾小球球性硬化、肾间质纤维化、肾间质炎细胞浸润、肾小管萎缩、肾血管病变的比率分别为32.7%、42.6%、55.6%、45.7%、37.0%，除肾间质炎细胞浸润，余病理改变的比率均随着年龄的增长而增加。经年龄纠正后，肾小球球性硬化的比率与年龄的关联消失。161例标本肾间质纤维化和肾小管萎缩程度均为轻度，96.7%伴有肾间质炎细胞浸润的标本其炎细胞浸润程度为轻度，其余为中度，部分标本炎细胞浸润程度重于肾小管萎缩及肾间质纤维化。11例（6.8%）患者的肾组织标本合并急性肾小管坏死，该患者均有肾功能受累，血肌酐418.5±265.8umol/L（143~956umol/L）。11例患者中2例年龄30岁（2.3%），7例（11.5%）介于31-60岁之间，2例（14.3%）为60岁或60岁以上。90%（9/10例）伴有高血压的MCD患者可见肾血管病变。初发和复发组、激素敏感和激素依赖组相比，两组肾脏病理改变上无统计学差异。88.3%的标本免疫荧光均为阴性，仅少部分标本可见系膜区IgM（8.0%）、IgA（7.4%）或C3（3%）弱阳性或阳性，局灶节段或弥漫节段沉积。所有电镜标本均可见足细胞足突弥漫融合，2例合并薄基底膜病。综上所述，本研究通过入选大组的成人MCD患者，总结了其临床及病理特征，发现男性多于女性，发病年龄男性早于女性；感染为MCD最常见的诱发因素，伴发镜下血尿少；合并高血压，糖尿病亦可并发MCD，肾小球球性硬化、肾间质纤维化、肾小管萎缩、肾血管病变的发生率和严重程度随年龄增长而增长，肾血管病变与年龄和高血压均有关，部分患者可合并急性肾功能损伤，甚至引起急性肾小管坏死，尤其老年60岁患者，更易并发，极少数患者可合并薄基底膜病。初发与复发、激素敏感与激素依赖两组的临床病理改变无明显差异，提示依据临床病理改变不能判断对单纯激素治疗的反应。
[Abstract]:Minimal change disease (MCD) is a common pathological type of nephrotic syndrome (NS), about 70%~90% less than 10 years old in children, and NS in children above the age of 10, and NS is MCD in children aged over 50%10 years. The clinicopathological features of Chinese adult MCD, especially in Chinese adults, are far from being reported. For adult MCD, many aspects are not known, nor are the clinicopathological manifestations of Chinese adult MCD similar to those of other ethnic groups.
The case data and follow-up data of 162 adult patients, aged 18 years old, were collected from the Department of Nephrology, Ruijin Hospital Affiliated to Shanghai Jiaotong University School of Medicine, from January 2009 to June 2013. The results showed that 162 patients, 98 males and 64 females, male and female ratio 1.53:1, and the average age of 33.8 + 14.8 years (18~). At the age of 76), the average age of onset was 33.114.7 years old, the male was earlier than the female, and the 18-30 year old patients accounted for 54.3% of the total. The male patients were mainly concentrated at 18-30 years old and 66.3%. The female patients were mainly distributed at 18-30 and 31-40 years old. The proportion of patients in the proportion of 35.9% and 31.3%.162, respectively, showed to varying degrees of edema and a large amount of eggs. White urine, hypoalbuminemia with hyperlipidemia, 47 cases (29%) have different causes, the most common (61.7%) infection (61.7%), only 17.9% of the urine protein quantitative 4g/24h, 45.7% of patients with urine protein quantitative 4-8g/24h, 36.4% of the urine protein quantitative 8g/ 24h.28.4% patients with microscopic hematuria, 17.3% of the patients with serum creatinine 115um Ol/L, the glomerular filtration rate (eGFR) was estimated with the MDRD formula. In 31.5% of patients with eGFR in 60-90ml/min/1.73m2,13.6% and 8%, eGFR was 30-60ml/min/1.73m2 and 30ml/min/1.73m2, and in EPI formula, 15.4% of patients eGFR were between 60-90ml/min/1.73m2,4.9% and 8%. The higher eGFR was 90ml/min/1.73m2, the higher the proportion of renal function increased with age.10 (6.2%) patients with hypertension, 2 cases with type 2 diabetes, of which 1 patients with diabetes combined with hypertension and 31-60 years of age, primary and recurrent groups, Ji Sumin and hormone dependence group, the two group in the 24h urine protein quantitative, blood No statistical difference was found in plasma albumin and renal function.
The pathological changes of renal tissue in 162 adult MCD cases showed that glomerular sclerosis, renal interstitial fibrosis, renal interstitial inflammation, renal tubule atrophy, renal tubule atrophy and renal vascular lesions were 32.7%, 42.6%, 55.6%, 45.7%, 37% respectively, except the infiltration of renal interstitial inflammatory cells, and the ratio of the remaining pathological changes increased with age. After correction, the incidence of glomerular sclerosis was associated with age. The degree of renal interstitial fibrosis and renal tubule atrophy were mild in.161 specimens. 96.7% of the specimens accompanied by infiltration of renal interstitial cells were mild, the other was moderate, and the degree of infiltration of some specimens was heavier than that of renal tubule atrophy and renal interstitial fiber. Renal tissue specimens of.11 cases (6.8%) were combined with acute renal tubular necrosis. The patients had renal function involvement, 2 cases of blood creatinine 418.5 + 265.8umol/L (143~956umol/L).11 patients were 30 years old (2.3%), 7 cases (11.5%) were between 31-60 years, 2 (14.3%) was 60 years old or 60 years older than.90% (9/10 cases) with hypertension and MCD patients showed renal vessels. In the initial and recurrent groups, compared with the hormone sensitivity and hormone dependence group, there was no statistical difference in the pathological changes of the two groups. The immunofluorescence of the specimens of.88.3% was negative, only a few specimens were found in the mesangial region IgM (8%), IgA (7.4%) or C3 (3%) were weakly positive or positive, and the focal segmental or diffuse segments were deposited. All the electron microscopic specimens showed podocyte. The foot process was filled with diffuse fusion, and 2 cases with thin basilar membrane disease.
To sum up, this study summed up the clinical and pathological features of adult MCD patients in a large group of adults, and found that males were more than women and were older than women; infection was the most common inducing factor of MCD, with less hematuria, combined hypertension and MCD, glomerular sclerosis, renal interstitial fibrosis, and kidney. The incidence and severity of renal vascular lesions increase with age. Renal vascular lesions are associated with age and hypertension. Some patients can merge acute renal damage and even cause acute renal tubular necrosis. In particular, the elderly 60 year old patients are more likely to be complicated. A few patients can combine thin basilar membrane disease. There was no significant difference in clinicopathological changes between the two groups of hormone sensitivity and hormone dependence, suggesting that the response to simple hormone therapy can not be judged according to clinical pathological changes.
【学位授予单位】：上海交通大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R692.6

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