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    Am J Med Genet. 1991 Aug 1;40(2):211-3.

    Dietary management reverses grooving and abnormal polarization of hair shafts in argininosuccinase deficiency.

    Kvedar JC, Baden HP, Baden LA, Shih VE, Kolodny EH.

    Source

    Harvard Medical School, Department of Dermatology, Boston, MA.

    Abstract

    We have observed that the fragile hair of two untreated patients with argininosuccinic aciduria showed abnormal alternating zones of bright and dark banding by polarizing microscopy. Scanning electron microscopy documented discontinuous grooves with a 50 to 100 microns periodicity. Results of amino acid analysis of the hair were essentially normal. After the patients were treated with a low-protein, arginine-supplemented diet, the hair assumed a normal appearance. Five patients already treated with diet showed no hair abnormalities. The pathogenesis of the hair changes in unknown, but our findings suggest that products generated in the disease can adversely affect metabolically active tissue such as hair.

    PMID:
    1897577
    [PubMed - indexed for MEDLINE]

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