【摘要】 22例肝豆状核变性患者(其中12例已经青霉胺治疗,10例未经治疗),有效肾血浆流量降低,尿氨基氮升高,肾小管酸化能力减退,尿盘状电泳见不同程度低、中分子蛋白尿.病变主要影响肾小管功能。部分肾功能韵改变与神经系统症状轻重平行。治疗后患者尿氨基氮较未治疗组明显为少,提示青霉胺可能使肾功能好转。4例作肾活检,虽肾脏病变轻微,但却已有明显的肾功能改变。肾组织中铜含量升高,提示肾脏损害与铜沉积有关。更多还原

【Abstract】 Several renal function tests were determined in 22 cases of Wilson’s disease.Theresults showed decreased effective renal plasma flow,defects of tubular acidifying abi-lity and increased amount of aminoacids in urine.Disc electrophoresis of urine showedmild proteinuria with low or middle molecular weight.It was found that the changes ofsome renal functions paralleled the severity of neurological symptoms.The amounts ofaminoacids in urine in untreated cases(10)was higher than those in treated ones(12),indicating that penicillamine was effective to improve the renal functions.Renal biopsieswere performed in 4 cases,showing mild renal damage only,however,their renaldysfunctions were marked. The amount of copper was increased in biopsy samples.Copper deposit in kidneys was considered to be related with renal damage.These defectsare thought reversible and may be partly corrected by early treatment.更多还原