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Research Article Free access | 10.1172/JCI105609
1Medical Professorial Unit, [Dunn Laboratories], St. Bartholomew's Hospital, London, E.C.1., England
†Address requests for reprints to Dr. R. W. E. Watts, Dunn Laboratories, St. Bartholomew's Hospital, London, E.C.1., England.
*Submitted for publication October 17, 1966; accepted March 21, 1967.
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1Medical Professorial Unit, [Dunn Laboratories], St. Bartholomew's Hospital, London, E.C.1., England
†Address requests for reprints to Dr. R. W. E. Watts, Dunn Laboratories, St. Bartholomew's Hospital, London, E.C.1., England.
*Submitted for publication October 17, 1966; accepted March 21, 1967.
Find articles by Scowen, E. in: JCI | PubMed | Google Scholar
1Medical Professorial Unit, [Dunn Laboratories], St. Bartholomew's Hospital, London, E.C.1., England
†Address requests for reprints to Dr. R. W. E. Watts, Dunn Laboratories, St. Bartholomew's Hospital, London, E.C.1., England.
*Submitted for publication October 17, 1966; accepted March 21, 1967.
Find articles by Thompson, C. in: JCI | PubMed | Google Scholar
1Medical Professorial Unit, [Dunn Laboratories], St. Bartholomew's Hospital, London, E.C.1., England
†Address requests for reprints to Dr. R. W. E. Watts, Dunn Laboratories, St. Bartholomew's Hospital, London, E.C.1., England.
*Submitted for publication October 17, 1966; accepted March 21, 1967.
Find articles by Watts, R. in: JCI | PubMed | Google Scholar
Published July 1, 1967 - More info
The renal clearance of cystine, lysine, ornithine, arginine, and glycine has been compared with the simultaneously determined glomerular filtration rate in nine cystinuric patients. Five were studied before and after stabilization on penicillamine therapy, two were studied only while taking penicillamine, and two were studied only in the absence of penicillamine administration. The renal clearances of cysteine-penicillamine and of penicillamine disulfide were also determined in the patients who were taking the drug.
Amino acids were determined by quantitative ion exchange chromatography, and the reliability of the method has been evaluated in terms of its reproducibility and of the recovery of known amounts of amino acids added to plasma and to urine. The plasma levels of cystine and of the basic amino acids were less than normal in all the patients. Cysteine-penicillamine and penicillamine disulfide were cleared by the kidney at rates similar to that of cystine. Two of the patients had glycine clearances that were considerably above the normal value. The renal clearance of cystine exceeded the glomerular filtration rate in six of the nine patients. The results form a continuum from values approximately equal to the glomerular filtration rate to values about twice this amount. The renal clearances of cystine and of the basic amino acids vary independently of one another in the disease.
The significance of these results is discussed in terms of the nature of the renal tubular transport defect that underlies the disorder.