[Clinical aspects of cystinuria and its treatment by thiola (author's transl)].
Cystinuria is an inherited disorder of amino acid transport affecting the epithelial cells of the renal tubules and the gastro-intestinal tract. Treatment consists of the prophylaxis of recurrent urolithiasis, which is the clinical manifestation of the disease. Long-term treatment with alpha-mercapto-propionyl-glycine (MPG; Thiola) promises to be successful. 3 cytinuric patients with recurrent urolithiasis underwent treatment over a period of 6 months. Therapy was controlled by regular follow-up investigations of the urinary excretion and serum levels of cystine and di-basic amino acids. The results did not indicate any permanent decrease in cystine excretion. No recurrence of renal calculi was observed. The possibility is discussed of a direkt mechanism of action of the drug on the metabolism of the involved amino acids.
D000596:Amino Acids / Q000378:metabolism; D000603:Amino Acids, Sulfur / Q000627:therapeutic use*; D001692:Biological Transport; D003555:Cystinuria / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D007669:Kidney Calculi / Q000209:etiology; D008625:Tiopronin / Q000627:therapeutic use*
D000596:Amino Acids; D000603:Amino Acids, Sulfur; D008625:Tiopronin
Klinische Aspekte der Cystinurie und ihre Behandlung mit Thiola