Treatment of cystinuria / Wilson G. Smillie, M.D. Boston.

  • Wilson G. Smillie
Date:
1915
Catalogue details

Licence: In copyright

Credit: Treatment of cystinuria / Wilson G. Smillie, M.D. Boston. Source: Wellcome Collection.

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    fever and chorea and was ill for eight or nine months. Rheumatic fever recurred every spring for two or three years. Two years ago he was operated on at the Boston City Hospital for appendicitis and inguinal hernia and made a satisfactory convalescence. Present Illness.—Six months after the operation the patient passed a small stone in the urine. This was soon followed by many others. Several patent medicines were tried, without relief. In August, 1913, the patient went to the Boston City Hospital with complete anuria, which he said had existed for three days. He was in the hospital three days, during which time he passed no urine. Being of a stubborn and intractable disposition, he refused cystoscopy or operation. He was discharged against advice and walked home. On the journey, “something gave way” and during the day he passed over “a gallon” of urine. The patient was seen by us first in January, 1913, when he came to the Peter Bent Brigham Hospital outdoor department. His chief symptoms then were pain in the lower lumbar region, pain over the symphysis pubis and blood and gravel in the urine. Physical Examination.*—The patient’s weight was 110 pounds; he was poorly developed, though fairly well nourished. The heart was slightly hypertrophied, with a high-pitched systolic murmur at the apex. The abdomen showed slight tenderness in the costovertebral angle on both sides. No abdominal masses or tenderness were made out. Examination otherwise was negative. The urine showed large numbers of typical cystin crystals and a small amount of blood, but no casts. Blood pressure was normal, hemoglobin, 95 per cent., and white blood cell count, 10,000. The patient was admitted to the house for metabolic study Jan. 31, 1914. The following data was recorded: intake of fluid, intake of nitrogen, output of urine, specific gravity of urine, the reaction of the urine as measured by the hydrogen ionization method, output of total nitrogen, urea, uric acid, ammonia, total sulphates and inorganic sulphates. (The results are shown in the table.) The hydrogen ionization was determined according to the colorimetric method of Henderson and Palmer.4 The factor 7.4 represents the reaction point of blood, the most alkaline figure, 8.4, is definitely pink to phenolphthalein. The diet was standardized by Locke’s5 6 tables. The urinary nitrogen, urea, uric acid and ammonia were determined by the microchemical methods of Folin0 and his associates. The total sulphates and inorganic sulphates were determined by the method of Folin. Alsberg and Folin1 have shown that cystin is best quantitated by the deter¬ mination of neutral sulphur. Ethereal sulphur was not determined since it remains constant. On 119 gm. protein intake the normal average excretion of ethereal sulphur as S03 is 0.22 gm. in twenty-four hours. The normal average of neutral sulphur as SOs is 0.17 gm. In cystinuria there is a marked increase in the neutral sulphur, which is due to the sulphur of cystin. The patient was put on a standard diet containing 10.5 gm. of nitrogen. He remained on this diet two days before the records were begun. His blood examination showed: Nonprotein nitrogen . 28.0 mg. per 100 c.c. of blood Urea nitrogen . 14.0 mg. per 100 c.c. of blood Uric acid. 1.4 mg. per 100 c.c. of blood Creatinin . 1.2 mg. per 100 c.c. of blood Creatin . 10.0 mg. per 100 c.c. of blood The creatinin and creatin determinations were made by Dr. W. Denis. On February 5, 2 gm. of cystin were given to the patient. This was excreted at once as inorganic sulphur, substantiating the observation of Alsberg and Folin, 4. Henderson and Palmer: Jour. Biol. Chem., 1912, xiii, 363. 5. Locke: Food Values, New York, D. Appleton & Co., 1911. 6. Folin, O.: Jour. Biol. Chem., 1912, xi, 493. *
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