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D C Cusworth

Publications and source records attributed to D C Cusworth.

13 recordsLinked to original sources

Comparison of 2-mercaptopropionylglycine and D-penicillamine in the treatment of cystinuria.

We have compared 2-mercaptopropionylglycine (MPG) with D-penicillamine in its capacity to reduce the urinary excretion of cystine by patients with cystinuria. Satisfactory ion-exchange chromatographic methods for the measurement of urinary MPG, D-penicillamine, cystine and the cysteine-disulfides of the two drugs have been developed, and we have established the time course of the excretion of these substances after oral intake of each drug. MPG was found to be approximately one and a half times as effective as D-penicillamine, both in reducing urinary excretion of free cystine and in the amounts of mixed disulfide which appeared in the urine.

Adult↗

The adult presenting idiopathic Fanconi syndrome.

The adult presenting Fanconi syndrome is a rare familial disorder. A 30-year follow-up of one of the original families in the literature is reported here. Two important points have emerged. Firstly, the inheritance in this family is dominant, not recessive as originally suggested, and there remains no good example in the literature of a recessive inheritance of this disorder. Second, in this family lactic aciduria and tubular proteinuria are probably the earliest manifestations of the disorder in childhood, with glycosuria and aminoaciduria developing in the second decade and osteomalacia from the start of the fourth decade. Glomerular function deteriorates slowly but is compatible with a normal lifespan.

Adult↗

Homocystinuria.

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Adult↗

Homocystinuria.

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Child↗