本文關(guān)鍵詞： 抗腎小球基底膜抗體 腎臟病理 抗中性粒細(xì)胞胞漿抗體 貧血 血漿置換　出處：《吉林大學(xué)》2014年碩士論文　論文類型：學(xué)位論文

【摘要】：目的 探討抗腎小球基底膜(GBM)病的特點(diǎn)，以加深對(duì)該組疾病的認(rèn)識(shí)，為臨床診療提供幫助，提高早期診斷率，早期治療，降低死亡率，改善預(yù)后。方法 對(duì)我科近3年來檢測(cè)出的19例抗GBM抗體相關(guān)疾病患者的臨床病理資料進(jìn)行回顧性分析。 結(jié)果 1、19例患者臨床表現(xiàn)輕重不一，15例表現(xiàn)為急性腎炎綜合征伴急進(jìn)性腎衰竭，進(jìn)行性貧血，無腎功能正常患者，3例伴有肺出血，1例住院期間死亡。 2、13/19例(68.4%)患者為單純抗GBM抗體陽(yáng)性，3/19例(15.8%)抗GBM抗體伴抗中性粒細(xì)胞胞漿抗體(ANCA)陽(yáng)性，3/19例(15.8%)抗GBM抗體伴某種抗核抗體(ANA)陽(yáng)性，其中1例同時(shí)伴抗SSa（++）、抗組蛋白抗體（++），1例抗52ka抗體（++）、抗組蛋白抗體（++），，1例抗組蛋白抗體（++）。 伴ANCA陽(yáng)性組ANCA抗體濃度較低，與其他兩組相比，發(fā)病年齡較大，吸煙率高，其中一例為19例患者中病情最重（重度貧血、抗GBM抗體轉(zhuǎn)陰時(shí)間最長(zhǎng)50天、血漿置換次數(shù)最多24次，環(huán)磷酰胺沖擊6次）。 3、進(jìn)行激素聯(lián)合環(huán)磷酰胺、血漿置換的18例患者中，4例初次就診時(shí)肌酐＜500umol/L，14例初次就診時(shí)肌酐＞500umol/L。 4、13例患者行腎活檢，11/13例為新月體腎炎，1例新月體腎炎伴急性腎小管損傷，1例為輕度系膜增生性腎小球腎炎。 9例患者免疫熒光為典型的IgG、C3沿腎小球毛細(xì)血管壁線樣沉積。3例免疫熒光沿系膜區(qū)及毛細(xì)血管壁顆粒樣分布，1例僅存在系膜區(qū)免疫復(fù)合物沉積。 5、11例患者入院時(shí)存在肺部感染，并使用抗生素治療；出院后，維持透析患者均為輕度貧血。 結(jié)論 1.抗GBM抗體相關(guān)疾病的臨床表現(xiàn)輕重不一。 2.腎臟免疫病理表現(xiàn)一部分為典型的IgG、C3沿腎小球毛細(xì)血管攀呈線樣沉積，一部分表現(xiàn)為顆粒樣沉積，另存在一定特殊的其他類型病理表現(xiàn)。 3.血漿置換，糖皮質(zhì)激素、環(huán)磷酰胺聯(lián)合免疫治療對(duì)該組疾病效果較好，早期診斷，及時(shí)強(qiáng)化免疫抑制治療對(duì)改善患者預(yù)后至關(guān)重要。 4.抗GBM病導(dǎo)致終末期腎病靠透析治療的患者貧血程度較輕。 5.少數(shù)患者合并某種ANA抗體陽(yáng)性，結(jié)合患者入我院前曾多處就診，服用藥物有關(guān)。
[Abstract]:Purpose. To explore the characteristics of anti-GBM disease in order to deepen the understanding of the disease, to provide help for clinical diagnosis and treatment, to improve the rate of early diagnosis, early treatment, reduce mortality and improve prognosis. The clinicopathological data of 19 patients with anti GBM antibody associated diseases in recent 3 years were analyzed retrospectively. Results. 1the clinical manifestations of 19 patients with acute glomerulonephritis with progressive renal failure and progressive anemia were 15 cases with acute nephritis syndrome, 3 cases with normal renal function and 1 case with pulmonary hemorrhage. 213 / 19 / 19 cases (68.4%) patients were only positive for anti GBM antibody and 3 / 19 cases for anti GBM antibody plus anti neutrophil cytoplasmic antibody (ANCA) positive for 3 / 19 cases.) Anti GBM antibody with some antinuclear antibody (Ana) was positive in 19 / 3% of the patients with anti GBM antibody and anti neutrophil cytoplasmic antibody (ANCA) positive in 3 / 19 cases. Among them, one case was accompanied by anti-SSaA, anti-histone antibody (AHA) and anti-histone antibody (anti-histone antibody) in 1 case, and anti-histone antibody (AHA) in 1 case. The concentration of ANCA antibody was lower in the positive group with ANCA. Compared with the other two groups, the age of onset was older and the smoking rate was higher. One of the 19 patients had the most serious condition (severe anemia, the longest negative period of anti-#en2# antibody was 50 days). The number of plasma exchange was up to 24 times and cyclophosphamide shock 6 times. (3) in 18 patients with plasma exchange, the creatinine was less than 500 umoll / L in 14 of the 18 patients who were treated with hormone and cyclophosphamide for the first time, and the creatinine was > 500 umol/ L in 14 patients at the first visit. 4 Renal biopsy was performed in 13 patients with crescene glomerulonephritis (n = 13), crescentin nephritis (n = 1), acute tubular injury (n = 1) and mild Mesangial proliferative glomerulonephritis (n = 1). Immunofluorescence of 9 patients was typical IgG C 3 deposition along the glomerular capillary wall. 3 cases had immunofluorescence distribution along the Mesangial area and capillary wall granulocyte. Only 1 case had the Mesangial area immune complex deposition. Five hundred and eleven patients had pulmonary infection on admission and were treated with antibiotics, and maintenance dialysis patients were mild anemia after discharge. Conclusion. 1. The clinical manifestations of anti-GBM antibody-related diseases are different. 2. Some of the renal immunopathologic manifestations were linear deposition along the glomerular capillary climbing, some were granular deposition, and some other special pathological manifestations. 3.The effect of plasma exchange, glucocorticoid and cyclophosphamide combined immunotherapy is good. Early diagnosis and timely intensive immunosuppressive therapy are very important to improve the prognosis of the patients. 4. Anemia in patients with end-stage nephropathy treated by dialysis was mild as a result of anti-GBM disease. 5. A few patients with some ANA antibody positive, combined with the patients before entering our hospital have many visits, drug-related.
【學(xué)位授予單位】：吉林大學(xué)
【學(xué)位級(jí)別】：碩士
【學(xué)位授予年份】：2014
【分類號(hào)】：R692.6

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