チスチン結石の治療 : 自験10例の臨床経過を中心として

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1. Ten cases of cystine calculi (8 male and 2 female) experienced in our clinic between 1963 and 1973 were reported. Their age were distributed from 14 to 64. 2. Their first episodes with urinary stone were seen at the age of 4 to 26, at 18 as a mean. All of them had had one or more episodes of stone discharge, or had been performed surgical operations once to 4 times until the admission to our clinic. One of them had experienced a stone discharge every year for the preceding 20 years. 3. Diagnosis of cystinuria was made by analysis of exstirpated stones in 6 cases and by examination of urine sediment in which cystine crystal was found in 4 cases. 4. Seven of the ten cases had hyperuricemia. It is suspected that it may have some relation with the formation of stone. 5. The statures of male patients were 146-169 cm, which were below those of the general populations. 6. D-Penicillamine was administered for 4 patients. It was effective for all of them in the sense that there was no recurrent stone, but rash and fever were seen in one case. 7. α-Mercatopropionylglycine (Thiora[○!R]) was administered in 9 cases, which prevented stone to recur or grow, except in one case, for 5 to 26 months. In addition, dissolution of stones was seen in two cases. Rash on limbs and trunk occurred in one case as a toxic effect. 8. The dose of Thiora required depends on the urinary excretion of cystine. We aim to keep this below 250 mg/L for prevention of recurrent stones and below 100 mg/L for dissolution of stones. 9. Studies of pedigree were done for 6 cases and one or more cystinurics were found in every case. None of them without a sister of case 2 had any episode of urinary tract calculus.
1975-12-20

チスチン結石の治療 : 自験10例の臨床経過を中心として

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