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Amylo?dosis Complicating Behcet’s Disease

机译:淀粉样变性合并白塞氏病

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Background: Behcet’s disease is a vasculitis affecting several organs. A renal involvement is rarely described. It is most of the time about a renal amyloidosis. Patients and methods: It is a descriptive retrospective study concerning the patients followed in our department for a Behcet’s disease having presented a renal amyloidosis. Results: It is about 4 men with average age of 38.25 years old. The renal involvement was revealed after an average delay of 5.7 years by a nephrotic syndrome in all cases. A renal insufficiency was noted in 3 cases with an average serum creatinine of 587 μmol/l (127 - 1490). The type of the amyloidosis was AA in 2 cases. The treatment contained colchicines in every case. The evolution was marked by the worsening of the renal function leading to end stage renal disease in 3 cases. Death occurred in 1 case and one patient lost to follow up. Discussion: Renal amyloidosis can complicate the evolution of a Behcet’s disease. It occurs generally 1 to 10 years after the beginning of the disease. Once installed, it evolves generally towards the chronic renal insufficiency and can condition the forecast of this affection. Conclusion: Amyloidosis is a rare complication of the Behcet’s disease. Its screening is so desirable to improve the renal prognosis of these patients.
机译:背景:白塞病是一种影响多个器官的血管炎。很少描述肾脏受累。大多数时候是关于肾脏淀粉样变性的。患者和方法:这是一项描述性回顾性研究,涉及在我科因白塞氏病而出现肾脏淀粉样变性的患者。结果:大约4名男性,平均年龄为38.25岁。在所有病例中,肾病综合征平均延迟5.7年后才显示出肾脏受累。 3例患者出现肾功能不全,平均血清肌酐为587μmol/ l(127-1490)。淀粉样变性的类型为AA 2例。在每种情况下,治疗均含有秋水仙碱。 3例患者的肾功能恶化导致终末期肾脏疾病,这标志着这种进化。 1例死亡,一名患者失访。讨论:肾脏淀粉样变性可使白塞氏病的发展复杂化。它通常在疾病开始后的1至10年内发生。一旦安装,它通常会演变成慢性肾功能不全,并可能影响这种疾病的预测。结论:淀粉样变性病是贝塞特氏病的罕见并发症。因此,对其进行筛选对于改善这些患者的肾脏预后是非常理想的。

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