[轻链沉积病累及肾脏]。

Sbornik lekarsky Pub Date : 2002-01-01
M Merta, R Rysavá, J Zabka, A Stejskalová, Z Vernerová, J Haber, I Spicka, V Tesar, P Klener
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引用次数: 0

摘要

综述了与单克隆γ病变(MG)相关的不同类型肾脏病变,重点是轻链沉积病(LCDD)。LCDD的病理生理基础仍然是轻链组织沉积。在组织沉积的免疫球蛋白的稳定域)。这种机制是单克隆免疫球蛋白过量产生的典型机制。LCDD的临床表现反映了疾病的多器官特征,其中肾脏病变是最常见、最严重、记录最充分的病变。临床资料参考了一组6例患者,治疗在我肾内科提出。LCDD的诊断建立在肾活检的基础上。所有患者在诊断时肾功能均下降,而血液透析治疗在1例患者中开始。结论探讨了LCDD治疗的可能性,其中对肾功能衰竭的若干对症治疗联合糖皮质激素治疗和细胞抑制剂治疗;多数患者预后仍然严重。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
[Kidney involvement in light-chain deposition disease].

An overview concerning different types of kidney involvement associated with monoclonal gammapathy (MG) is given, focused on light-chain deposition disease (LCDD). Pathophysiologic basis of LCDD remains in the light-chain tissue deposition (resp. in tissue deposition of immunoglobulin's stable domain). This mechanism is typical for monoclonal immunoglobulin's overproduction as found in MG. Clinical picture of LCDD reflects multiorgan character of disorder, while renal lesions rank among the most frequent, serious and best documented ones. Clinical data referring to a group of six patients, treated in our nephrologic department are presented. Diagnosis of LCDD was established on basis of the renal biopsy finding. Renal functions were decreased at the time of diagnosis in all patients, whereas haemodialysis treatment was started in one patient. On conclusion therapeutic possibilities of LCDD are discussed, in which number symptomatic therapy of renal failure is combined with corticosteroids therapy and cytostatic therapy; prognosis of most patients remains serious.

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