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[单克隆丙种球蛋白病中的肾功能损害。临床研究]

[Renal impairment in monoclonal gammapathies. Clinical study].

作者信息

Spicka I, Merta M, Cieslar P, Klener P, Chrz M, Zabka J, Mokrejsová M, Rysavá R, Slabý J, Zounar R

机构信息

I. interní klinika FVL UK a VFN, Praha.

出版信息

Cas Lek Cesk. 1995 Aug 2;134(15):478-81.

PMID:7585865
Abstract

BACKGROUND

Renal involvement is an important and frequent complication in patient with monoclonal gammapathy (MG), especially in multiple myeloma (MM). Light chain proteinuria produces many renal manifestations, the most serious form is acute renal failure, which occurs in 5-10% of patients with MM. The frequency and form of renal involvement was determined in a group of patients with MG. The disturbances observed were correlated with the concentration and type of paraprotein in serum and urine.

METHODS AND RESULTS

We investigated 82 patients, 37 men and 45 women with an average age of 63.5 years. Apart from standard nephrologic tests the aminoaciduria/24 h and urine acidification capacity was determined. In some patients renal biopsy was performed. Proteinuria was observed in 66 cases (80.5%), in 54 of them of Bence-Jones type. Nephrotic syndrome developed in 4 patients, in all cases the renal amyloidosis was present. Renal insufficiency was diagnosed in 39 patients (47.5%), mostly in MM. In 14 cases was renal insufficiency reversible, in 14 remain stable and in 11 progressed during the course of disease. Irreversible progression developed in terminal phase of disease in most cases. Acute renal failure was observed in 6 patients, only in four of them further course of renal disease could be evaluated. In half of these 4 patients the renal failure was reversible.

CONCLUSIONS

Higher frequency of proteinuria and renal insufficiency was detected in patients with light chain paraprotein of lambda type, or biclonal kappa+lambda type. Aminoaciduria was diagnosed in 40% of patients, we did not observe complete Fanconi's syndrome. Incompleted form of renal tubular acidosis we diagnosed in 52% of cases without other signs of renal involvement.

摘要

背景

肾脏受累是单克隆丙种球蛋白病(MG)患者重要且常见的并发症,尤其是在多发性骨髓瘤(MM)患者中。轻链蛋白尿会引发多种肾脏表现,最严重的形式是急性肾衰竭,在5% - 10%的MM患者中会出现。我们确定了一组MG患者肾脏受累的频率和形式。观察到的紊乱情况与血清和尿液中副蛋白的浓度及类型相关。

方法与结果

我们研究了82例患者,其中37例男性和45例女性,平均年龄63.5岁。除了标准的肾脏检查外,还测定了24小时氨基酸尿和尿液酸化能力。部分患者进行了肾活检。66例(80.5%)患者出现蛋白尿,其中54例为本-周蛋白类型。4例患者发展为肾病综合征,所有病例均存在肾淀粉样变性。39例(47.5%)患者被诊断为肾功能不全,大多为MM患者。14例患者的肾功能不全可逆转,14例保持稳定,11例在疾病过程中进展。大多数情况下,不可逆进展发生在疾病末期。6例患者出现急性肾衰竭,其中仅4例可评估肾脏疾病的后续病程。这4例患者中有半数肾功能衰竭可逆转。

结论

在λ型轻链副蛋白或双克隆κ + λ型患者中,蛋白尿和肾功能不全的发生率更高。40%的患者被诊断出氨基酸尿,我们未观察到完全性范可尼综合征。52%的病例被诊断为不完全性肾小管酸中毒,且无其他肾脏受累迹象。

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[Renal involvement in monoclonal gammopathies].
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