Clinical outcome of Schonlein-Henoch purpura nephritis in children

Clinical outcome of Schonlein-Henoch purpura nephritis in children
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DOI:
10.1007/s004670050707
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发表时间:
1999-11-01
影响因子:
3
通讯作者:
Schafer, F
Schafer, F
中科院分区:
医学3区
文献类型:
--
作者:
Schärer, K;Krmar, R;Schafer, F

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我们对 64 名经活检证实患有过敏性紫癜 (SHP) 肾炎的儿童进行了 1-23 年随访的长期结果研究。发病后 10 年的总体肾存活率为 73%。多变量逻辑回归分析确定初始肾功能不全(P = 0.004)、肾病综合征(P = 0.037)和组织学改变的严重程度(根据肾小球新月体的比例定义)(P = 0.051)是进行性肾衰竭的重要独立预测因素。 4 名患者随访超过 19 年,在短暂恢复后出现肾小球损伤。 8 名患有新月体肾炎且伴有快速进展病程和/或持续性肾病综合征的儿童在紫癜发作后 16 周内接受了至少 7 次血浆置换 (PE) 治疗。治疗期间,每位患者的血清肌酐水平从平均 2.3 毫克/分升下降至 1.1 毫克/分升,随后又反弹上升。 5 名患者重复进行 PE 疗程产生了类似的反应。 4 名接受 PE 的患者在发病后 1.2.-3.7 年达到终末期肾病,3 名患者最终处于终末期肾衰竭(7-13.5 年后肌酐 3.2-6.1 mg/dl),1 名患者达到正常肾小球滤过率。我们的经验表明,初始肾功能不全是 SHP 肾炎儿童进一步临床病程的最佳单一预测因子​​。早期 PE 似乎可以延缓某些患有严重、快速进展疾病的患者的进展。
We studied the long-term outcome of 64 children with biopsy-proven Schonlein-Henoch purpura (SHP) nephritis over 1-23 years of follow-up. Overall renal survival 10 years after onset was 73%. Multivariate logistic regression analysis identified initial renal insufficiency (P=0.004), nephrotic syndrome (P=0.037), and the severity of histological alterations, as defined by the proportion of glomerular crescents (P=0.051), as significant independent predictors of progressive renal failure. Four patients followed for more than 19 years showed glomerular damage after transient recovery. Eight children with crescentic nephritis associated with a rapidly progressive course and/or persistent nephrotic syndrome were treated by at least seven sessions of plasma exchange (PE) within 16 weeks of onset of purpura. During treatment serum creatinine levels dropped in each patient from a mean of 2.3 to 1.1 mg/dl, followed by a rebound increase. Repeated courses of PE in 5 patients produced comparable responses. Four patients undergoing PE reached end-stage renal disease at 1.2.-3.7 years after onset, whilst 3 finally were in preterminal renal failure (creatinine 3.2-6.1 mg/dl after 7-13.5 years), and 1 patient reached a normal glomerular filtration rate. Our experience suggests that initial renal insufficiency is the best single predictor of the further clinical course in children with SHP nephritis. Early PE appears to delay the progression in some patients with severe, rapidly progressive forms of the disease.