Genitourinary disturbances in familial and sporadic cases of primary amyloidosis with polyneuropathy.
Genitourinary disturbances in familial and sporadic cases of primary amyloidosis with polyneuropathy.
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原发性淀粉样变性伴多发性神经病的家族性和散发性病例的泌尿生殖系统紊乱。
DOI：
10.1111/j.0954-6820.1974.tb08096.x
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发表时间：
2009
影响因子：
--
通讯作者：
P. Hofer
中科院分区：
文献类型：
--
作者：
R. Andersson;P. Hofer
． 本文分析了34例家族性或散发性淀粉样变性伴多神经病变患者（男性24例，女性10例）泌尿生殖系统障碍的发生情况。13例患者死亡，其中9例进行了尸检。25例患者中9例出现尿潴留，10例同时出现尿潴留和尿失禁。膀胱功能障碍晚期的特点是膀胱充盈感丧失，排尿欲望降低，排尿困难。尿失禁通常表现为坐肌悖论性失禁（溢出性失禁）。淀粉样蛋白沉积发生在膀胱壁，位于神经、血管壁和逼尿肌的平滑肌。膀胱功能明显受损是由于神经对膀胱的影响，可能也是由于逼尿肌内淀粉样蛋白的沉积。24例男性患者中有16例发生完全性阳痿，被认为主要是由于淀粉样蛋白对神经的影响。只有两名患者出现尿毒症，其中一人有肾病综合征的迹象。肾脏中淀粉样蛋白沉积的数量变化范围很广。这种物质甚至可能不存在。9例患者中有4例肾小球沉积，均有蛋白尿。在接受检查的28名患者中，有13名患有细菌尿，其中10名患有蛋白尿。这种淀粉样变形式的蛋白尿的原因可能是肾脏中的淀粉样蛋白沉积或尿路感染。
. The occurrence of genitourinary disturbances has been analysed in 34 successive patients (24 males and 10 females) with familial or sporadic amyloidosis with polyneuropathy. Thirteen patients died, autopsy was performed on 9 of them. Out of 25 patients 9 had urinary retention and 10 both retention and incontinence. Advanced stages of urinary bladder dysfunction were characterized by loss of sensation of bladder fullness, reduced desire to void and difficulty in initiating the voiding. The incontinence usually had the character of ischuria paradoxa (overflow incontinence). Amyloid deposition occurred in the wall of the urinary bladder, located in the nerves, vessel walls, and in the smooth musculature of the detrusor muscle. The function of the urinary bladder was obviously impaired by the affection of the nerves to the bladder, and presumably also by the amyloid deposition in the detrusor musculature. Total impotence, occurring in 16 out of the 24 male patients, was considered to be due mainly to the amyloid affection of the nerves. Uraemia appeared in two patients only, signs of a nephrotic syndrome in one of them. The amounts of amyloid deposits in the kidneys varied within wide limits. The substance could even be absent. Out of 9 cases examined glomerular deposits were found in 4, all of whom had proteinuria. Thirteen out of 28 patients examined had bacteriuria, 10 of them also proteinuria. The cause of proteinuria in this form of amyloidosis may be either amyloid deposition in the kidneys or infection of the urinary tract.