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β-地中海贫血-Hb-E病中的肾小球肾炎:临床表现、组织病理学研究及预后

Glomerulonephritis in beta-thalassemia Hb-E disease: clinical manifestations, histopathologic studies and outcome.

作者信息

Ongajyooth L, Siritanaratkul N, Pootrakul P, Parichatikanond P, Malasit P, Fucharoen S, Nimmannit S, Piankijagum A, Nilwarangkur S

机构信息

Renal Unit, Siriraj Hospital, Mahidol University, Bangkok, Thailand.

出版信息

J Med Assoc Thai. 1995 Mar;78(3):119-26.

PMID:7643028
Abstract

We presented 8 patients with beta-thal/Hb E with glomerular diseases. Diverse glomerular lesions were seen, but diffuse endocapillary glomerulonephritis was the most common. The clinical manifestations of acute glomerulonephritis in beta-thal/Hb E differed from typical cases in the older age group, female preponderance, longer duration of edema, less hypertension, marked proteinuria, hypoalbuminemia and hypertriglyceridemia and also a longer period of recovery but their outcome was still favorable despite many risk factors of renal injury. Renal biopsy was necessary in doubtful cases to detect the correct diagnosis and give proper management. The association and mechanism of glomerulonephritis in these patients require further prospective study.

摘要

我们报告了8例患有β地中海贫血/Hb E且合并肾小球疾病的患者。观察到多种肾小球病变,但弥漫性毛细血管内增生性肾小球肾炎最为常见。β地中海贫血/Hb E患者急性肾小球肾炎的临床表现与老年组典型病例不同,以女性居多,水肿持续时间较长,高血压较少,蛋白尿明显,低白蛋白血症和高甘油三酯血症,恢复时间也较长,但尽管存在许多肾损伤危险因素,其预后仍然良好。对可疑病例进行肾活检对于明确诊断和进行适当治疗很有必要。这些患者肾小球肾炎的关联及机制需要进一步的前瞻性研究。

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