TREATMENT OF SEVERE IGA NEPHROPATHY IN CHILDREN
TREATMENT OF SEVERE IGA NEPHROPATHY IN CHILDREN
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DOI：
10.1007/bf00858524
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发表时间：
1989-07-01
影响因子：
3
通讯作者：
BERGSTEIN, JM
中科院分区：
文献类型：
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作者：
ANDREOLI, SP;BERGSTEIN, JM
我们使用泼尼松和硫唑嘌呤治疗了 10 名患有严重 IgA 肾病 (IgAN) [蛋白尿 > 1 g/天、高血压、肾功能不全、节段性硬化、新月体形成和/或 IgA 肾小球基底膜 (GBM) 沉积] 的儿童 1 年。治疗一年后，十名儿童中的七名接受了重复的肾活检。所有活检均针对活动性（显示新月体形成的肾小球百分比、系膜增殖和间质浸润的程度；最高分 = 9）和慢性性（显示纤维新月体、节段性硬化、整体性硬化的肾小球百分比以及肾小管萎缩和间质纤维化程度；最高分 = 12）进行评分。治疗1年后，所有儿童的蛋白质排泄量均从4,052.+-显着下降（P < 0.01）。 3,190 毫克/天至 1,692 .+-。 1,634 毫克/天。活动得分从 4.35 .+- 显着下降（P < 0.01）。治疗前 0.94 至 2.28.+-。治疗后为 0.75，而慢性评分未改变（5.42 .+-. 1.7 vs 5.85 .+-. 2.0）。显示细胞新月体的肾小球百分比从 21.2.+- 下降（P < 0.05）。治疗前 21.7% 降至 0.94 .+-。治疗后2.4%。治疗后，系膜 IgA 沉积持续存在，但 GBM 沉积不太明显。在随访期间（平均 2.6 年，范围 9 个月至 7.5 年），一名儿童因活检证实活动性疾病复发而需要短暂再治疗，两名儿童因无炎症情况下进行性疤痕形成而出现肾功能不全，而其余 7 名儿童情况稳定。我们建议泼尼松和硫唑嘌呤治疗可能对患有严重 IgAN 的儿童有益，并且有必要进行对照临床试验。
We treated ten children with severe IgA nephropathy (IgAN) [proteinuria > 1 g/day, hypertension, renal insufficiency, segmental sclerosis, crescent formation and/or glomerular basement membrane (GBM) deposition of IgA] with prednisone and azathioprine for 1 year. Following the year of therapy, seven of the ten children underwent a repeat kidney biopsy. All biopsies were scored for activity (percentage of glomeruli demonstrating crescent formation, degree of mesangial proliferation and interstitial infiltrate; maximum score = 9) and chronicity (percentage of glomeruli demonstrating fibrous crescents, segmental sclerosis, global sclerosis, and degree of tubular atrophy and interstitial fibrosis; maximum score = 12). After 1 year of therapy, the protein excretion of all the children decreased significantly (P < 0.01) from 4,052 .+-. 3,190 mg/day to 1,692 .+-. 1,634 mg/day. The activity score decreased significantly (P < 0.01) from 4.35 .+-. 0.94 prior to therapy to 2.28 .+-. 0.75 after therapy while the chronicity score was unchanged (5.42 .+-. 1.7 vs 5.85 .+-. 2.0). The percentage of glomeruli demonstrating cellular crescents decreased (P < 0.05) from 21.2 .+-. 21.7% prior to therapy to 0.94 .+-. 2.4% after therapy. Mesangial deposition of IgA persisted but GBM deposition of IgA was less prominent after therapy. During the follow-up period (mean 2.6 years, range 9 months-7.5 years), one child required brief retreatment for biopsy-confirmed recurrence of active disease, two children have developed renal insufficiency due to progressive scarring in the absence of inflammation, while the remaining seven are stable. We suggest that treatment with prednisone and azathioprine may be beneficial in children with severe IgAN and that a controlled clinical trial is warranted.