Kim P K, Kim K S, Pai K S, Kim J H, Choi I J
Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea.
Yonsei Med J. 1997 Oct;38(5):307-18. doi: 10.3349/ymj.1997.38.5.307.
Twenty-nine children with nephrotic syndrome were treated with cyclosporine (CsA), 100 mg/m2/day for 6 months and prednisone, 2 mg/kg every other day for 1 month and then subsequently 1 mg/kg every other day for 5 months. A renal biopsy had shown minimal change disease (MCD) in 18 children, focal segmental glomerulosclerosis (FSGS) in 3 children, membranous glomerulonephritis (MGN) in 4 children, membranoproliferative glomerulonephritis (MPGN) in 2 children, and IgA nephropathy in 2 children. All MCD patients went into complete remission during therapy. Five out of 11 steroid-sensitive patients (45.5%) remained in complete remission, while the remaining 6 (54.5%) had 2 to 3 relapses, 19 to 47 months after CsA discontinuation. Two out of 7 steroid-resistant patients (28.6%) were still in complete remission and 5 (71.4%) had 1 to 6 relapses 25 to 49 months after CsA withdrawal. The mean number of relapses in the steroid-sensitive group before and after CsA treatment decreased more (8.5 vs 1.4) than in the steroid-resistant group (8.1 vs 2.4) (p < 0.05). At the most recent examination, 1 of 3 FSGS patients achieved complete remission and 2 had a partial response. Three of 4 MGN patients were in complete remission and 1 was in partial remission. One of 2 MPGN patients achieved complete remission and 1 showed partial remission. Two patients with IgA nephropathy were in partial remission. We compared MCD patients in sustained remission and relapse; the mean CD4/CD8 ratio decreased from 1.5 to 0.9 in the remission group, in comparison with no change in the relapsed group (p < 0.05). The posttreatment renal biopsy showed lesions of nephrotoxicity in 3 of 18 children with MCD whose renal function did not alter after CsA treatment. We concluded: 1) A 6-month treatment of CsA, in combination with a low-dose alternate-day steroid, proved to be effective in maintaining the remission of steroid-sensitive and steroid-resistant MCD patients. 2) The CD4/CD8 ratio can be used as a index to predict remission or relapse after CsA therapy.
29例肾病综合征患儿接受环孢素（CsA）治疗，剂量为100mg/m²/天，持续6个月，同时接受泼尼松治疗，开始剂量为2mg/kg隔日一次，持续1个月，随后1mg/kg隔日一次，持续5个月。肾活检显示，18例患儿为微小病变性肾病（MCD），3例为局灶节段性肾小球硬化（FSGS），4例为膜性肾小球肾炎（MGN），2例为膜增生性肾小球肾炎（MPGN），2例为IgA肾病。所有MCD患者在治疗期间均完全缓解。11例激素敏感患者中有5例（45.5%）持续完全缓解，其余6例（54.5%）在停用CsA后19至47个月复发2至3次。7例激素抵抗患者中有2例（28.6%）仍完全缓解，5例（71.4%）在停用CsA后25至49个月复发1至6次。CsA治疗前后，激素敏感组的平均复发次数减少幅度（8.5对1.4）大于激素抵抗组（8.1对2.4）（p<0.05）。在最近一次检查时，3例FSGS患者中有1例完全缓解，2例部分缓解。4例MGN患者中有3例完全缓解，1例部分缓解。2例MPGN患者中有1例完全缓解，1例部分缓解。2例IgA肾病患者部分缓解。我们比较了持续缓解和复发的MCD患者；缓解组的平均CD4/CD8比值从1.5降至0.9，而复发组无变化（p<0.05）。18例MCD患儿中有3例在CsA治疗后肾功能未改变，但治疗后肾活检显示有肾毒性病变。我们得出结论：1）CsA联合低剂量隔日激素治疗6个月，被证明对维持激素敏感和激素抵抗的MCD患者的缓解有效。2）CD4/CD8比值可作为预测CsA治疗后缓解或复发的指标。
