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Clinical Aspects

cystine, urine, brothers, calculi, free and albumin

CLINICAL ASPECTS.

But little can be said upon the clinical aspects of cystinuria. The substance has been known to have been passed intermittently in the urine for years without visible impairment of the bodily functions, but usually there are marked signs of deterioration of the general health, and the nutrition is bad. It has been noticed in tubercular, anemic and chlorotic patients. The influence of debilitating agencies in increasing the deposit was most pronounced in Barry's case. Its most marked clinical aspect is the tendency which it exhibits to be hereditary. Golding Bird mentions a series of cases of cystine calculus appearing in three successive generations.

Dr. Marcet mentions two brothers who died with renal cystine calculus. Lenoir and Civiale extracted cystine calculi from the bladders of two brothers. Teale cut a boy for cystine stone and two of his brothers had cystinuria. Dr. Joel found cystine in a mother and two daughters. Poland says that out of twenty-two collected cases of cystine calculi ten occurred in four families, while in three cases the subjects were brothers.

Drs. Picchini and Conti relate the following case, which is given as an example of the many factors which may be at work in produc ing this metabolic modification: The patient was a woman aged twenty-nine, who had good health until the age of nineteen, when she had acute rheumatism; at twenty two she had another attack, accompanied by cardiac and pleural troubles from which she never subsequently became quite free. At twenty-nine she was attacked by subacute polyarthritis rheumatica, with joint deformities, pleural effusion, dry pericarditis, and general cardiac hypertrophy with mitral stenosis. She had also a febrile temperature, and her liver became considerably enlarged. The urine was scanty, 200 to 300 c.c., and showed a trace of albumin with hyaline casts and many leucocytes, also a trace of urobilin; the sediment con tained abundant crystals of cystine. The bladder was found on sound

ing to be free from calculi. Her condition improved greatly during her stay in the hospital; the liver became reduced in size, the fever disappeared, also the urine became free from casts and albumin and increased in amount to about normal. The cystine, however, was never absent. The total amount of cystine varied with the quantity of urine passed and, contrary to what was noted in Ebstein's case, more of it was secreted in the day than at night. A nitrogenous diet made no difference, but milk increased the amount of cystine. Uric acid was increased during the day and lessened during the night, while urea was generally diminished.

Cystine calculi do not differ in their clinical characters from other forms. The following case which came under the care of my col league, Mr. Heycock, is interesting as showing a gouty diathesis and as presenting the largest cystine calculus on record.

M. N., aged 50, was admitted into St. Peter's Hospital, Lon don, with a history of calculous symptoms for the past nine months. He had been fairly well except for attacks of gout in the knees and hand, from which he had suffered twice a year during the last fifteen years. His bladder had been washed out and sounded by various medical men. The prostate was much enlarged, the urine showed a specific gravity of 1.015 ; it was acid, and contained a good deal of pus, albumin, and cystine. Median cystotomy was performed and the stone crushed with difficulty after Dolbeau's method. The fragments were soft and waxy, of a slight fawn color, and showed no concentric lines of increment. The weight proved to be two and one-quarter ounces. The patient recovered.