Huang Yan-Jie, Yang Xiao-Qing, Zhai Wen-Sheng, Ren Xian-Qing, Guo Qing-Yin, Zhang Xia, Yang Meng, Yamamoto Tatsuo, Sun Yuan, Ding Ying
, Zhengzhou, China.
Department of Pediatrics, First Affiliated Hospital of Henan University of Traditional Chinese Medicine, Beijing, China.
World J Pediatr. 2015 Nov;11(4):338-45. doi: 10.1007/s12519-014-0527-4. Epub 2014 Nov 20.
The aim of this retrospective study was to define the clinical manifestations, pathological features and prognosis of children with membranoproliferative-like Henoch-Schönlein purpura nephritis (HSPN), representing International Study of Kidney Disease in Children (ISKDC) grade VI.
Among 245 patients with HSPN treated in our hospital between 2008 and 2010, nine patients (3.7%) were diagnosed with HSPN of ISKDC grade VI (males = 5, females = 4, age: 9.5 ± 2.03 years, mean ± SD). The clinical features, laboratory and pathological findings, treatment and outcome of the 9 patients were retrospectively analyzed.
Of the 9 patients, 7 (78%) presented with hematuria and nephrotic syndrome, and were treated with steroids (oral prednisone or intravenous methylprednisolone pulse therapy) and immunosuppressants (oral tripterygium glycosides or intravenous cyclophosphamide pulse therapy). One (11%) patient had hematuria and nephrotic range proteinuria (> 50 mg/kg per 24 hours) and was treated with oral prednisone and tripterygium glycosides. Another (11%) patient presented with hematuria and moderate proteinuria (25-50 mg/kg per 24 hours) and was treated with oral tripterygium glycoside only. Histopathological examination showed diffuse glomerular mesangial and endocapillary proliferation, mesangial interposition, double-contour formation, podocyte hypertrophy, shedding, and cytoplasmic absorption droplets. The percentages of glomeruli with small cellular crescents varied from 4%-25% in 6 of 9 patients. Follow-up for 2 to 4 years showed excellent recovery in all patients.
The main clinical feature of ISKDC grade VI HSPN in children is a nephrotic syndrome with hematuria. The excellent prognosis of the disease was probably related to early diagnosis and treatment with steroids and/or immunosuppressants, and mild degree of glomerulosclerosis and tubulointerstitial damage.
本回顾性研究旨在明确具有膜增生样改变的儿童过敏性紫癜性肾炎（HSPN）（相当于儿童肾脏病国际研究（ISKDC）分级VI级）的临床表现、病理特征及预后。
2008年至2010年在我院接受治疗的245例HSPN患者中，9例（3.7%）被诊断为ISKDC分级VI级的HSPN（男性5例，女性4例，年龄：9.5±2.03岁，均值±标准差）。对这9例患者的临床特征、实验室及病理检查结果、治疗及转归进行回顾性分析。
9例患者中，7例（78%）表现为血尿和肾病综合征，接受了类固醇（口服泼尼松或静脉注射甲泼尼龙冲击治疗）和免疫抑制剂（口服雷公藤多苷或静脉注射环磷酰胺冲击治疗）治疗。1例（11%）患者有血尿和肾病范围蛋白尿（>50mg/kg每24小时），接受口服泼尼松和雷公藤多苷治疗。另1例（11%）患者表现为血尿和中度蛋白尿（25 - 50mg/kg每24小时），仅接受口服雷公藤多苷治疗。组织病理学检查显示弥漫性肾小球系膜和内皮细胞增生、系膜插入、双轨形成、足细胞肥大、脱落及胞质吸收滴。9例患者中有6例肾小球小细胞性新月体的比例为4% - 25%。随访2至4年显示所有患者恢复良好。
儿童ISKDC分级VI级HSPN的主要临床特征是伴有血尿的肾病综合征。该疾病预后良好可能与早期诊断并使用类固醇和/或免疫抑制剂治疗以及肾小球硬化和肾小管间质损伤程度较轻有关。
