紫癜性肾炎伴大量蛋白尿患儿临床病理相关性分析

Analysis of clinical manifestations and pathological findings in children with massive proteinuria with Henoch-Schonlein purpura nephritis

目的 探讨伴有大量蛋白尿的紫癜性肾炎（HSPN）患儿的临床病理特点.方法 回顾性研究了2008年1月-2013年1月于首都儿科研究所附属儿童医院肾脏内科住院的伴有大量蛋白尿的52例HSPN患儿资料,比较患儿的临床表现和病理特点,分析了解临床与病理特点之间的关系.结果 （1） 52例伴有大量蛋白尿的HSPN患儿中,男30例,女22例,发病年龄4岁8个月～14岁1个月,病程9 d～24个月.出现阳性症状体征（肉眼血尿、少尿、水肿、高血压）的16例（31％）,血清白蛋白达到肾病水平的7例（13％）.（2）病理分级Ⅲ级以上者占72％;其中有新月体形成、粘连、硬化、毛细血管内增生及小动脉管壁病变的分别占56％,52％,19％,67％,62％;伴有重度系膜增生者占42％;病理分级≥Ⅲ级的患儿存在系膜重度增生和小动脉壁改变的较病理分级＜Ⅲ级的显著增多（x2=10.971,P=0.001;x2 =4.132,P=0.042）.（3）临床与病理间的关系：病理中有新月体形成的患儿,其新月体比例与24 h尿蛋白定量呈正相关;存在临床伴随表现（肉眼血尿、高血压、肾功能异常或补体C3下降）的患儿病理中有节段硬化、毛细血管内增生表现的多于无伴随症状的患儿（x2=19.69,P=0.00;x2 =3.887,P=0.049）.结论 伴有大量蛋白尿的儿童HSPN,虽临床表现相对较重,但症状不典型.病理表现多样,其中系膜重度增生和小动脉病变在病理分级较重的患儿中较为常见,而新月体形成、节段硬化、毛细血管内增生与患儿临床表现存在一定关系.

Objective To evaluate clinical manifestations and pathology in children with massive proteinuria with Henoch-Schonlein purpura nephritis （HSPN）.Method The data of the 52 children with massive proteinuria with Henoch-Schonlein purpura nephritis who were hospitalized in the department of nephrology in our hospital from January 2008 to January 2013 were retrospectively studied.Clinical manifestation and pathologic characteristics were summarized and compared.The relationship between clinical manifestations and pathologic characteristics were evaluated.Result （1） Among the children,16 （31%） cases had positive symptoms and signs including gross hematuria,edema,oliguria and hypertension,and in only 7 （13%） cases serum levels of albumin were below 25 g/L.（2） Children with pathological grade 〉 Ⅲ accounted for 72%.Children with crescent formation,glomerular capsule adhesion,segmental glomeralosclerosis,endocapillary proliferation and lesions in the walls of arterioles accounted for 56%,52%,19%,67%,62%,respectively,and 42% of the children suffered from severe mesangial proliferation or mesangial sclerosis.The frequencies of severe mesangial proliferation and changes in the walls of arterioles in children with pathological grade ≥ Ⅲ was significantly higher than those in children with pathological grade 〈 Ⅲ （x2 =10.971,P =0.001 ; x2 =4.132,P =0.042）.（3） The relationship between clinical manifestation and pathologic characteristics：The number of crescents was positively correlated with 24-hour urinary protein quantity in children with crescent formation.The frequencies of segmental glomeralosclerosis and endocapillary proliferation of the children with clinical symptoms including gross hematuria,hypertension,renal dysfunction or decreased complement C3 were significantly higher than those of children without clinical symptoms（x2 =19.69,P =0.00 ;x2 =3.887,P =0.049）.Conclusion The clinical manifestations of children with massive proteinuria with HSPN were relatively severe but the symptoms were not typical.The pathological manifestations wer variable,of which severe mesangial proliferation and lesions in arterioles are the most common in children with higher pathological grade.Clinical manifestations in children with HSPN are associated with formation of crescent,segmental glomeralosclerosis,glomerular capsule adhesion,and endocapillary proliferation.