儿童毛细血管内皮细胞增生性紫癜性肾炎的临床病理及预后分析

目的 探讨以弥漫性毛细血管内皮细胞增生为主要病理表现的紫癜性肾炎（DEP-HSPN）的临床、病理及预后。 方法 回顾性分析本院近10年来经肾活检确诊的8例DEP-HSPN患儿临床、病理和预后资料，并分别与同病理级别或具有同等蛋白尿水平（肾病水平蛋白尿）的非DEP-HSPN患儿进行比较。 结果 （1）DEP-HSPN起病急，临床表现重，8例患儿中，4例临床表现为肾炎性肾病，3例表现为肾病水平蛋白尿伴血尿，1例呈急性肾炎综合征，4例患儿合并有肉眼血尿。病理分级均为Ⅲ-b级，光镜主要表现为弥漫性毛细血管内皮细胞和系膜细胞增生，常合并毛细血管袢坏死及肾小球内炎性细胞浸润，4例患儿合并细胞性新月体。（2）与病理为Ⅲ-b级的非DEP-HSPN患儿比较，DEP-HSPN患儿病程较短，临床多见肉眼血尿，24 h尿蛋白量高，更多呈肾炎性肾病表现。病理上，DEP-HSPN肾小球毛细血管袢坏死更常见。与具有肾病水平蛋白尿的非DEP-HSPN患儿相比，DEP-HSPN合并新月体的比例较低。（3）8例患儿均采用口服泼尼松联合静脉滴注环磷酰胺（CTX）冲击，病程早期给予2个疗程甲泼尼龙冲击治疗方案。平均随防（7.00±2.20）月，1例临床痊愈，5例持续镜下血尿，2例微量蛋白尿及持续镜下血尿。两组患儿预后差异无统计学意义。 结论 DEP-HSPN起病较急，临床以大量蛋白尿或肾炎性肾病为主要表现，并且常合并肉眼血尿。病程早期给予积极的免疫抑制剂治疗常能取得较满意的近期疗效。

Analysis of clinicopathology and prognosis of childhood Henoch-Schonlein purpura nephritis with diffused endothelial cell proliferation

Objective To investigate the clinicopathological characteristics and prognosis of Henoch-Schonlein purpura nephritis with diffused endothelial cell proliferation (DEP-HSPN) in children. Methods Data of 8 DEP-HSPN cases in Nanjing Children's Hospital within recent ten years were retrospectively reviewed. The clinicopathological features, efficacy and prognosis were compared between DEP-HSPN cases and 48 cases of non-DEP-HSPN. Non-DEP-HSPN cases were divided into two groups according to the clinical classification or the pathological classification.Results (1) In DEP-HSPN, HSP developed nephritis within 4 to 15 days after the initial onset of purpuric rashes. Hematuria was present in all the 8 patients. The main clinical manifestation of DEP-HSPN was nephritic-nephrotic syndrome (4 cases), nephrotic level proteinuria (3 cases) and acute nephritic syndrome (1 case). Four cases had macrohematuria. Six cases had abdominal symptoms and two cases had arthritis. Pathology of all the cases showed grade Ⅲ-b lesion with diffused endocapillary proliferation and segmental necrotizing lesion of the capillary wall, always accompanied with intraglomerular inflammatory cell infiltration. Crescent was found in 4 cases. (2)Compared to non-DEP-HSPN grades Ⅲ, DEP-HSPN showed a shorter course of disease.Macrohematuria, heavy proteinuria, nephritic-nephrotic syndrome, and segmental necrotizing lesion of capillary wall were more common in DEP-HSPN. Compared to non-DEP-HSPN with nephrotic level proteinuria, DEP-HSPN had a lower rate of crescent. (3) Methylprednisolone pulse therapy in early stage, then prednisone combined with cyclophosphamide were used in the treatment of DEP-HSPN.After an average follow-up period of seven months, one patient showed complete remission, five showed persistent microhematuria, and two showed persistent microhematuria accompanied with minor proteinuria. No significant difference of prognosis was found between DEP-HSPN and nonDEP-HSPN. Conclusions DEP-HSPN has an acute onset. The main clinical manifestation of DEP-HSPN is nephritic-nephrotic syndrome and nephrotic level proteinuria, always accompanied with macrohematuria. Immunosuppressant treatment in the early stage of disease is effective for a short-term outcome.