Inborn errors of metabolism: the Croonian lectures delivered before the Royal College of Physicians of London, in June, 1908 / by Archibald E. Garrod.

  • Garrod, Archibald E. (Archibald Edward), Sir, 1857-1936.
Date:
1909
Catalogue details

Licence: In copyright

Credit: Inborn errors of metabolism: the Croonian lectures delivered before the Royal College of Physicians of London, in June, 1908 / by Archibald E. Garrod. Source: Wellcome Collection.

Provider: This material has been provided by Royal College of Physicians, London. The original may be consulted at Royal College of Physicians, London.

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    but when the injection was made into the branches of the portal vein it was apparently destroyed in the liver and no cystinuria resulted. However neither Blum, nor Rothera,^® who also tried the experiment, was able to bring about the destruction of cystin by adding it to crushed hepatic tissue. Obviously the whole of the cystin ingested in pro- tein foods, as distinguished from that derived from the tissues, is not burnt straightway to sulphates and excreted as such. Some, as we have seen, is probably utilized in the formation of taurin, and some must escape direct destruction and be built up into the tissue proteins, the cystin content of which must necessarily be maintained. We may now turn to the consideration of the derangement of sulphur metaboHsm in cystin- uria, in virtue of which the subjects of that anomaly excrete some part of their cystin as such in their urine, often to their serious disad- vantage. That cystinuria is less rare than alkap- tonuria I am convinced, although I cannot bring forward any statistical evidence in support of this statement, which is based upon personal experience acquired during a search for examples of both anomahes during the past ten years. That a far larger number of cases of cystinuria than of alkap- tonuria are on record counts for httle, for the former has been known for weU-nigh a century, whereas our knowledge of the latter does not extend back more than sixty years. Moreover, the surgical consequences of cystinuria are such as compel