本文關(guān)鍵詞： 輕鏈型淀粉樣變性 全切片掃描分析 臨床特點(diǎn) 預(yù)后　出處：《腎臟病與透析腎移植雜志》2017年02期 　論文類型：期刊論文

【摘要】：目的:探討輕鏈型淀粉樣變性(AL型)淀粉樣物質(zhì)在腎組織沉積部位及程度與臨床表現(xiàn)及預(yù)后的關(guān)系。方法:回顧分析經(jīng)腎活檢確診的AL型淀粉樣變性患者的臨床病理資料,利用全切片掃描技術(shù)計算淀粉樣物質(zhì)占組織的百分比,分析其與臨床及預(yù)后的關(guān)系。結(jié)果:AL型淀粉樣變性患者259例,男性147例,中位年齡57歲,腎臟均受累,腸道受累71.06%,心臟受累43.22%。中位病程7月,蛋白尿4.80±3.15g/24h,19.31%的患者腎功能不全。中位隨訪時間22.3月,患者平均生存時間24.24月,1、2、3和5年人生存率分別為81.8%、64.1%、52.4%和29.6%;腎臟生存率分別為90.8%、82.3%、76.8%和47.8%。淀粉樣物質(zhì)累及腎小球(99.61%),腎血管(92.66%)和腎間質(zhì)(50.19%)。淀粉樣物質(zhì)占腎小球、腎血管、腎間質(zhì)及整個腎組織的百分比分別為11.81%±11.38%、14.14%±14.05%、3.34%±5.36%和4.25%±5.77%,且與多項(xiàng)臨床指標(biāo)具有相關(guān)性。淀粉樣物質(zhì)占腎小球百分比、年齡、尿N-乙酰-β-D-氨基葡萄糖苷酶(NAG)及尿視黃醇結(jié)合蛋白(RBP)為患者預(yù)后的獨(dú)立危險因素。淀粉樣物質(zhì)占腎血管、腎間質(zhì)及整個腎組織百分比,血清肌酐,尿RBP為腎臟預(yù)后的獨(dú)立危險因素。結(jié)論:全切片掃描定量分析是觀察淀粉樣物質(zhì)在腎臟不同部位沉積及其程度的有效方法,并證實(shí)蛋白尿、血清肌酐等與淀粉樣物質(zhì)腎臟不同部位沉積有一定相關(guān)性。腎臟不同部位淀粉樣物質(zhì)沉積與患者預(yù)后和腎臟預(yù)后相關(guān)。
[Abstract]:Objective: to investigate the relationship between the location and degree of amyloid deposition in renal tissue and the clinical manifestation and prognosis. Methods: the clinicopathological data of patients with AL type amyloidosis diagnosed by renal biopsy were retrospectively analyzed. The percentage of amyloid in tissue was calculated by whole section scanning technique, and the relationship between amyloid content and clinical and prognosis was analyzed. Results 259 cases of amyloidosis of type A were male 147 cases, median age 57 years old, all of them were involved in kidney. The median course of disease on July was 4.80 鹵3.15g / 24 h proteinuria (4.80 鹵3.15g / 24 h, 19.31%), and the median follow-up time was 22.3 months. The average survival time of the patients was 24.24 months and 52.4% and 29.664%, respectively, while the renal survival rates were 90.8% and 82.3% and 47.8%, respectively. The amyloid involvement of the glomeruli was 99.61and 92.666.66) and the interrenal interstitial mass was 50.190.The amyloid substances occupied the glomeruli, the renal blood vessels, the renal vessels, and the renal vessels, and the renal vessels, the renal vessels, the renal vessels, the renal vessels, the renal vessels, the renal vessels, the renal vessels, the renal vessels, the renal vessels, the renal vessels, and the amyloid substances. The percentage of renal interstitial tissue and the whole renal tissue were 11.81% 鹵11.38% 鹵14.14% 鹵14.05% 鹵3.34% 鹵5.36% and 4.25% 鹵5.77%, respectively. Urinary N-acetyl- 尾 -Daminoglycosidase (NAG) and urinary retinol binding protein (RBP) were independent prognostic factors. The percentage of amyloid in renal vessels, renal mesenchyme and the whole renal tissue, serum creatinine, serum creatinine, serum creatinine, serum creatinine, serum creatinine, and serum creatinine were determined. Conclusion: full slice scanning quantitative analysis is an effective method to observe the deposition and degree of amyloid in different parts of kidney, and to confirm proteinuria. Serum creatinine was correlated with the deposition of amyloid in different parts of the kidney, which was related to the prognosis of the patients and the prognosis of the kidney.
【作者單位】： 南京大學(xué)醫(yī)學(xué)院附屬金陵醫(yī)院(南京軍區(qū)南京總醫(yī)院)腎臟科國家腎臟疾病臨床醫(yī)學(xué)研究中心全軍腎臟病研究所;無錫市人民醫(yī)院;
【基金】：國家重點(diǎn)研發(fā)計劃課題(2016YFC0904103,2016YFC0901202) 國家科技支撐計劃課題(2015BAI12B02,2015BAI12B05)
【分類號】：R597.2
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本文編號：1537676