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PubMed1976

[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.

Amino Acids↗
PubMed1978

Experimental cystinuria: the cycloleucine model. II. Amino acid efflux from intestinal and renal tissues.

Loading and unloading experiments using intestinal sacs and renal cortex slices were undertaken to ascertain the role of amino acid efflux in cycloleucine-induced amino-aciduria. The presence of cycloleucine, lysine, or valine on the luminal or antiluminal side of the intestine caused an increased leakage of [14C] cycloleucine, [14C] lysine, and [35S] cystine from the tissue. Similar results were obtained when using kidney cortex slices, except for cystine efflux. The latter phenomenon was inhibited by cycloleucine and lysine. Data, also obtained with renal cortex slices, suggest that cystine and cysteine are recognized by different transport sites although one (the oxidized form) may be typically extracellular and the other (the reduced form), intracellular. A comparison of these data with previous works done in our laboratory shows that cycloleucine affects efflux less than influx and further suggests that in rats given cycloleucine, renal transport is impaired only at the brush border level for cystine and at both luminal and antiluminal membranes for dibasic amino acids.

Amino Acids↗
PubMed1977

Cystinuria.

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Cystinuria↗
PubMed1978

Cystinuria in a maned wolf.

A renal calculus composed principally of the amino acid, cystine, was found in an 8-year-old male maned wolf (Chrysocyon brachyurus). Cystine crystals were found in the urine sediment. The renal clearance of 10 amino acids was abnormal, whereas reabsorption of others was normal. The renal clearance of cystine, lysine, ornithine, and arginine exceeded the filtered load. The renal tubular handling of glucose, phosphate, sodium, potassium, and uric acid was identical to that for the clinically normal dog. These findings indicated an isolated renal tubular defect for cystine and other amino acids.

Animals↗
PubMed1976

Cystinuria.

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Adult↗
PubMed1977

Low Dose D-penicillamine in cystinuria.

(1) A single dose of D-penicillamine (not more than 750 mg) taken at 2200 h, together with a prescribed fluid intake of two to three litres during the waking hours, without extra drinks at night, is sufficient to keep the concentration of the urinary cystine below the saturating concentration of urine throughout the 24 h period. (2) This regime does not materially reduce the total 24 h excretion of cystine. (3) The effect of D-penicillamine is mainly seen in the urine excreted between 0200 and 0800 h. (4) This regime is provisionally recommended for the prevention of cystine stone recurrence but not for stone dissolution. Larger total amounts of D-penicillamine given in divided doses are still recommended for the latter purpose.

Adolescent↗