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題 名 | Long-Term Clinical and Morphological Evaluation of Primary Membranoproliferative Glomerulonephritis=原發性膜厚性增殖性腎炎: 長期臨床表現評估 |
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作 者 | 吳明儒; 徐國雄; 陳禮平; 呂源三; 鄭志雄; 許松山; 徐約翰; 連榮達; | 書刊名 | 中華醫學雜誌 |
卷 期 | 57:1 1996.01[民85.01] |
頁 次 | 頁34-41 |
分類號 | 415.812 |
關鍵詞 | 膜厚性增殖性腎絲球腎炎; 腎病症候群; 腎臟穿刺; Membranoproliferative glomerulonephritis; Nephrotic syndrome; Renal biopsy; |
語 文 | 英文(English) |
中文摘要 | 背景:膜厚性增殖性腎絲球腎炎是較少見的原發性腎絲球腎炎,過去二十年的病例也逐漸減少中。在台灣,這類病例的臨床表現甚少被報告。為提升對此少見腎絲球腎炎之了解,我們回溯性探討十二年來確定診斷之22病例。 方法:自1982年11月至1994年12月,在814例原發性腎絲球腎炎中,有22例確定診斷為膜厚性增殖性腎絲球腎炎。我們追溯檢查臨床數據,病歷記錄及病理切片,並評估其臨床表現,病理特徵及預後。 結果:病例中有15位男性及7 位女性,年齡介於11至67歲,而追蹤期限1至140個月,平均46.3個月。其中11位經電子顯微鏡做病理分類,9位屬於第I型及2位屬於第III型。診斷時的臨床表現包括:86.4%有腎病症候群,63.6%有腎功能不良,50%有顯微血尿,31.8%肉眼可見血尿,50%有高血壓。低血中補體出現在40.9%的病例(第I型44.4%,第III型50%)。雖然藥物治療結果不理想,22位病人中,17位惡化,有4位維持正常腎功能但仍有蛋白尿,只有1位完全緩解。15位進入洗腎者,其中有6位是第I型而第III型則無。兩位第III型的病例均能維持正常腎功能對治療也有反應。五年及十年的腎臟存活率分別是33.3%及16.7%。平均腎臟存活時間為51.2個月。 結論:大多數在診斷時有腎功能不良(86.7%)及高血壓(85%)者,後來都惡化為尿毒,有腎病症候群者50%惡化為尿毒。延遲診斷及醫囑服從性差可能是腎臟存活率較國外差的原因。而兩位第III型的病例癒後都不錯。雖然藥物治療不能絕對有效,但是至少有5位(22.7%)治療後能維持正常腎臟功能;所以,若無禁忌症,免疫抑制劑的治療效果是可以適用的。 |
英文摘要 | Background: Membranoproliferative glomerulonephritis (MPGN) is a relatively rare primary glomerulonephritis (GN). Its incidence has decreased progressively in the past two decades. To improve knowledge of this rare GN, a retrospective review of 22 patients during a 12-year period was undertaken. Methods: From November 1982 to December 1994, from a total 814 cases of primary GN, 22 patients with primary MPGN were diagnosed. Clinical data, medical records, renal pathology and outcome were reviewed. Results: Patients included 15 males and 7 females, aged from 11 to 67 years. The average follow-up period was 46.3 months, with a range of 1 to 140 months. Tissue was available for electromicroscopic study in 11 cases; of which 9 cases fulfilled morphologic criteria of Type I MPGN; the other 2 cases were Type III MPGN. The clinical presentations at diagnosis included nephrotic syndrome 86.4%), impaired renal function (63.6%), microhematuria (50%), gross hematuria (31.8%) and hypertension (50%). Low serum C3 was found in 40.9% cases, 44.4% in Type I and 50% in Type III MPGN. The positive rate of hepatitis B virus infection was 22.7%, with 33.3% in Type I and none in Type III MPGN. All 22 patients received various combined antihypertensive agents, immunosuppressant, anticoagulant and antiplatelet agents at diagnosis, but 17 had progressive disease, 4 maintained normal renal function with proteinuria and only 1 had complete remission. Fifteen patients, including six Type I and no Type III MPGN, progressed to end-stage renal failure. Both patients with Type III MPGN maintained normal renal function and responded to treatment. The 5 and 10 year actuarial renal survival rates were 33.3% and 16.7% respectively. The median kidney survival time was 51.2 months. Conclusions: A majority of cases with MPGN presenting with impaired renal function (86.7%) and hypertension (85%) at diagnosis progressed to end-stage renal failure, while 50% of these patients with nephrotic syndrome at diagnosis progressed to end-stage renal failure. Delayed diagnosis and poor compliance were possible reasons for compared with for worse prognosis previous reports. But two patients with Type III MPGN had favorable prognosis previously described. Treatments generally failed to halt disease progression. Since at least five cases (22.7%) maintained normal renal function after treatments, a course of immunosuppressant is probably indicated if there is no contraindication. Further study with a larger population is warranted to clarify this issue. |
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