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Crescentic glomerulonephritis in children.
Pediatric Nephrology ( IF 3 ) Pub Date : 2020-02-12 , DOI: 10.1007/s00467-019-04436-y
Ulrike Mayer 1 , Jessica Schmitz 2 , Jan Hinrich Bräsen 2 , Lars Pape 1
Affiliation  

BACKGROUND To date, there is insufficient knowledge about crescentic glomerulonephritis (cGN), the most frequent immunologic cause of acute kidney injury in children. METHODS Over a period of 16 years, we retrospectively analyzed kidney biopsy results, the clinical course, and laboratory data in 60 pediatric patients diagnosed with cGN. RESULTS The underlying diseases were immune complex GN (n = 45/60, 75%), including IgA nephropathy (n = 19/45, 42%), lupus nephritis (n = 10/45, 22%), Henoch-Schoenlein purpura nephritis (n = 7/45, 16%) and post-infectious GN (n = 7/45, 16%), ANCA-associated pauci-immune GN (n = 10/60, 17%), and anti-glomerular basement-membrane GN (n = 1/60, 2%). Patient CKD stages at time of diagnosis and at a median of 362 days (range 237-425) were CKD I: n = 13/n = 29, CKD II: n = 15/n = 9, CKD III: n = 16/n = 7, CKD IV: n = 3/n = 3, CKD V: n = 13/n = 5. Course of cGN was different according to class of cGN, duration of disease from first clinical signs to diagnosis of cGN by biopsy, percentage of crescentic glomeruli, amount of tubular atrophy/interstitial fibrosis and necrosis on renal biopsy, gender, age, nephrotic syndrome, arterial hypertension, dialysis at presentation, and relapse. Forty-eight/60 children were treated with ≥ 5 (methyl-) prednisolone pulses and 53 patients received oral prednis(ol)one in combination with mycophenolate mofetil (n = 20), cyclosporine A (n = 20), and/or cyclophosphamide (n = 6), rituximab (n = 5), azathioprine (n = 2), tacrolimus (n = 1), and plasmapheresis/immunoadsorption (n = 5). CONCLUSIONS The treatment success of cGN is dependent on early diagnosis and aggressive therapy, as well as on the percentage of crescentic glomeruli on renal biopsy and on the underlying type of cGN. CsA and MMF seem to be effective alternatives to cyclophosphamide.

中文翻译:

小儿新月型肾小球肾炎。

背景技术迄今为止,关于新月型肾小球肾炎(cGN)(儿童急性肾损伤最常见的免疫学原因)的知识尚不充分。方法在16年的时间里,我们回顾性分析了60名经诊断为cGN的小儿患者的肾脏活检结果,临床过程和实验室数据。结果潜在疾病为免疫复合物GN(n = 45/60,75%),包括IgA肾病(n = 19/45,42%),狼疮性肾炎(n = 10/45,22%),过敏性紫癜肾炎(n = 7/45,16%)和感染后GN(n = 7/45,16%),ANCA相关的伪免疫GN(n = 10/60,17%)和抗肾小球基底-膜GN(n = 1 / 60,2%)。患者在诊断时和中位362天(范围237-425)的CKD分期为CKD I:n = 13 / n = 29,CKD II:n = 15 / n = 9,CKD III:n = 16 / n = 7,CKD IV:n = 3 / n = 3,CKD V:n = 13 / n =5。cGN的病程因cGN的类别,从首次临床体征到通过活检诊断cGN的疾病持续时间,新月肾小球百分比,数量而异肾萎缩/间质纤维化和坏死对肾活检,性别,年龄,肾病综合征,动脉高压,就诊透析和复发的影响。48/60名儿童接受了≥5(甲基)泼尼松龙脉冲治疗,53例患者接受了口服泼尼松(ol)联合霉酚酸酯(n = 20),环孢霉素A(n = 20)和/或环磷酰胺(n = 6),利妥昔单抗(n = 5),硫唑嘌呤(n = 2),他克莫司(n = 1)和血浆置换/免疫吸附(n = 5)。结论cGN的治疗成功取决于早期诊断和积极治疗,以及肾活检和cGN潜在类型的新月肾小球百分比。CsA和MMF似乎是环磷酰胺的有效替代品。
更新日期:2020-02-12
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