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タイトル: 姉妹にみられたチスチン尿石症 ; D-penicillamine治療を中心として
その他のタイトル: Cases of cystinuria with urinary tract stones in sisters
著者: 原田, 卓  KAKEN_name
高橋, 陽一  KAKEN_name
加藤, 篤二  KAKEN_name
著者名の別形: Harada, Takashi
Takahashi, Yoichi
Kato, Tokuji
キーワード: Adolescent
Adult
Amino Acids/analysis
Cystinuria/complications/drug therapy/genetics
Female
Humans
Kidney Calculi/drug therapy
Penicillamine/therapeutic use
Urinary Bladder Calculi/drug therapy
Urinary Calculi/complications/genetics
発行日: Sep-1971
出版者: 泌尿器科紀要刊行会
誌名: 泌尿器科紀要
巻: 17
号: 9
開始ページ: 577
終了ページ: 584
抄録: Cystinuria with urolithiasis was found in 19 and 12 years old sisters. Serum and urinary amino acids constitutions were determined by column chromatography and they revealed excess excretion of four amino acids; cystine, lysine, ornitine and arginine in their urine. Daily urinary output of cystine exceeded more than 250 mg per gm. creatinine which Harris and his collaborators mentioned as possible stone forming urinary cystine concentration. Recessive homozygous trait was thought to be eligible after surveys for their family tree and biochemical analysis of their urine. After surgical procedures for each case, pyelolithotomy or cystolithotomy, the residue staghorn stones in the left kidney of elderly one has been treated by D-penicillamine and alkali for three years. No significant complication was observed during therapeutic period under substitution of pyridoxine. Consequently, the stones reduced their size to such an extent as discharged spontaneously. The authors discussed about pathogenesis of the stone formation in cystinuric patient with emphasis on the genetic aspect of this inborn error in metabolism.
URI: http://hdl.handle.net/2433/121300
PubMed ID: 5166128
出現コレクション:Vol.17 No.9

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