GENITOURINARY DISTURBANCES IN FAMILIAL AND SPORADIC CASES OF PRIMARY AMYLOIDOSIS WITH POLYNEUROPATHY

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Abstract
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.