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霉酚酸酯与类固醇联合治疗抵抗性I型膜增生性肾小球肾炎的缓解情况。

Remission of resistant MPGN type I with mycophenolate mofetil and steroids.

作者信息

De Sudarsana, Al-Nabhani Dana, Thorner Paul, Cattran Daniel, Piscione Tino D, Licht Christoph

机构信息

Division of Nephrology, The Hospital for Sick Children, Toronto, ON, M5G 1X8, Canada.

出版信息

Pediatr Nephrol. 2009 Mar;24(3):597-600. doi: 10.1007/s00467-008-1023-7. Epub 2008 Oct 30.

Abstract

Very few studies have been published on how to treat children with membranoproliferative glomerulonephritis type I (MPGN I), and as yet there is only one report on the use of mycophenolate mofetil (MMF) in children with MPGN I. We report a 12-year-old boy who presented with edema, hypertension, nephrotic range proteinuria, and microscopic hematuria following an upper respiratory tract infection. Laboratory tests revealed a serum creatinine of 90 micromol/l, albumin of 20 g/l, and a C3 of 0.11 g/l (normal range: 0.7-1.4). Renal biopsy showed the presence of MPGN I. Upon failure to induce remission with prednisone, we started the patient on MMF at 500 mg/day (300 mg/m(2)), increasing up to a final dose of 2 g/day (1200 mg/m(2)), with a MMF metabolite mycophenolic acid (MPA) target range of 2-5 mg/l. Prednisone was subsequently reduced to alternate day therapy and gradually weaned to 7.5 mg on alternate days over 9 months. Within 4 months of starting MMF therapy, there was significant improvement in serum creatinine (decrease from 156 to 64 micromol/l), serum albumin (increase from 23 to 40 g/l), and proteinuria (decrease from 13 g/day to 40 mg/day). Twelve months following the introduction of MMF into his therapeutic regimen, he remains in remission with no further relapses. In summary, this case suggests that there may be potential benefit for use of MMF in children with refractory MPGN I, which supports the rationale for prospectively evaluating MMF treatment in a treatment trial of refractory MPGN I.

摘要

关于如何治疗Ⅰ型膜增生性肾小球肾炎(MPGNⅠ)患儿的研究报道极少,目前仅有一篇关于霉酚酸酯(MMF)用于MPGNⅠ型患儿的报告。我们报告一例12岁男孩,在上呼吸道感染后出现水肿、高血压、肾病范围蛋白尿和镜下血尿。实验室检查显示血清肌酐90微摩尔/升,白蛋白20克/升,C3为0.11克/升(正常范围:0.7 - 1.4)。肾活检显示为MPGNⅠ型。在使用泼尼松诱导缓解失败后,我们开始让患者服用MMF,初始剂量为500毫克/天(300毫克/平方米),最终剂量增至2克/天(1200毫克/平方米),MMF代谢产物霉酚酸(MPA)的目标范围为2 - 5毫克/升。随后泼尼松减为隔日疗法,并在9个月内逐渐减至隔日7.5毫克。在开始MMF治疗的4个月内,血清肌酐(从156降至64微摩尔/升)、血清白蛋白(从23增至40克/升)和蛋白尿(从13克/天降至40毫克/天)有显著改善。在将MMF引入其治疗方案12个月后,他仍处于缓解状态,未再复发。总之,该病例表明MMF用于难治性MPGNⅠ型患儿可能有潜在益处,这支持了在难治性MPGNⅠ型治疗试验中对MMF治疗进行前瞻性评估的理论依据。

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