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題名 | Intravenous Cyclophosphamide Pulse Therapy on Children with Severe Active Lupus Nephritis=脈衝性靜脈注射Cyclophosphamide療法用於嚴重活性期狼瘡腎炎之孩童 |
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作者姓名(中文) | 顏大欽; 周正成; 蔡銘哲; 江伯倫; 曹永魁; 謝貴雄; | 書刊名 | 中華民國小兒科醫學會雜誌 |
卷期 | 36:3 民84.05-06 |
頁次 | 頁203-209 |
分類號 | 417.5743 |
關鍵詞 | 狼瘡性腎炎; 脈衝性靜脈注射Cyclophosphamide療法; Lupus nephritis; Intravenous cyclophosphamide pulse therapy; |
語文 | 英文(English) |
中文摘要 | 爲了評估使用隔月注射的脈衝性靜脈注射cyclophosphamide(IVCY)療法,用來治療患有活性期狼瘡腎炎孩童之臨床療效和安全性;我們在1993年6月到1994年5月共收集了13個已同時接受類固醇和脈衝性靜脈注射cyclophosphamide療法的病人(平均年齡:14±0.9歲,女比男的比例是11比2),將其臨床資料加以分析比較。符合接受IVCY治療的適應症是:不但病人臨床上必須處於活性期的腎炎,而且必須至少要符合下列三點中之任一種情形:1)腎臓切片顯示是廣泛性增生性腎絲球腎炎;2)臨床上有腎病症侯群且對高劑量類固類的治療沒有反應;3)類固醇治療期間發生了嚴重的副作用。 IVCY之療法爲開始每隔一個月接受一次IVCY的治療共6個月,之後依臨床反應,改爲2至3月接受一次治療直到2年結束,到目前爲止,平均每個病人接受過治療的次數爲6.5±0.5次。Cyclophosphamide的開始劑量是500mg/mm^2,然後隔月增加250mg/mm^2,至最高量100mg/mm^2。在治療六個月後,血清第三補體、第四補體、和抗DNA抗體價位有很明顯的改善,在治療前和6個月後的變化分別是:C3:41.6±5.1比96.7±11.3mg/dl,C4:11.7±1.1比9.1±2.8IU/ml,均p<0.01。合併使用的類固醇也能順利減量(治療前和6個月後:48.5±0.8比15.6±3.2mg/day,p<0.01)。肌氨酸酐的平均廓清率(clearance of creatinine)也是有意義的改善,在治療前和6個月後分別是44.7±5.7比81.9±2.7ml/min/1.73mm^2,p<0.01;平均24小時的尿蛋白也明顯降低(治療前和6個月後:6.7±0.9比3.0±0.6gm/day,p<0.02)。常見的合併症多是輕微可恢複的。沒有人髮生出血性膀胱炎。發生感染的機會也很低(共2.6%,全部78次的注射中,只有2次發生,分別是肺炎及蜂窩組織炎)。我們的結論是脈衝性靜脈注射cyclophosphamide對於有活性期狼瘡腎炎的孩童是一種有效的療法。在我們的研究中,到目前爲止,此療法具有短期的療效和安全性。在治療6個月期間內,能夠壓抑狼瘡活性,改善腎功能和降低蛋白尿,而且副作用不大。但是長期療效和副作用,仍需要長期追蹤和觀察,才能進一步了解。 |
英文摘要 | We conducted a preliminary study of monthly intravenous cyclophosphamide (IVCY) therapy on children with active lupus nephritis to evaluate the clinical efficacy and safety of JVCY pulse therapy. From June 1993 to May 1994, 13 patients (mean age: 14±0.9 years old; female: male = 11:2) with active lupus nephritis were treated with intermittent 1VCY in addition to their regular corticosteroid therapy. Criteria for patients who received intermittent IVCY therapy were associated with any one of the following conditions: 1) with renal biopsy-proved diffuse proliferative glomerulonephritis; 2) with nephrotic syndrome and are inert to high dose of prednisolone therapy; and 3) with severe side effect of steroid therapy. Cyclophosphamide (CY) was administrated monthly for the first 6 doses, and at 2 to 3-month interval afterward for 2 years. The mean IVCY pulses per patient was 6.5±0.5 times. The initial dose of CY was 0.5 gm/mm^2, then gradually increased to 1 gm/mm^2 with a monthly increment of 0.25 gm/mm^2. After 6 months, cyclophosphamide treatment was associated with significant improvement in mean levels of serum C3 (41.6±5.1 vs. 96.7±11.3 mg/dl), C4 (11.7±1.1 vs. 35.3±5.0 mg/dl) and anti-DNA titer (65.4±17.1 vs. 9.1±2.8 IU/ml) (all p<0.0l), despite a significant reduction in mean prednisolone dosage (48.5±4.8 vs.15.6±3.2 mg/day; p<0.0l). The mnea,i creatinine clearance also improved significantly from 44.7±5.7 to 81.9±2.7 ml/min/1.73 mm^2 (p<0.01) after 6 months of therapy. There was a decrease in 24-hour urine protein excretion from 6.7±0.9 to 3.0±0.6 gm/day (p<0.02) after 6 months of therapy. The complications were mild and reversible. None had hemorrhagic cystitis. The infection rate was low, only 2 infections (pneumonia and cellulitis) occurred out of 78 episodes of treatments (2.6%). We concluded that IVCY pulse therapy is an efficient therapy for children with active lupus nephritis. Short- term efficacy and safety of IVCY pulse therapy were demonstrated in our study. This treatment could suppress the lupus activities, improve the renal function and reduce proteinuria with acceptable side effects over 6-month period. However, further investigation and regular follow-up are necessary to elucidate the long-term effects and complications of IVCY pulse therapy on the patients. |
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