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  • 1
    ISSN: 1432-1076
    Keywords: Infantile Refsum disease ; Phytanic acid ; Dietary treatment ; Peroxisomes ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Two patients with infantile phytanic acid storage disease (infantile Refsum disease), one of whom showed the presence of morphologically normal peroxisomes in a liver biopsy, were treated with a low phytanic acid diet for more than 2 years and the effects of treatment on certain clinical, biochemical and ultrastructural parameters were examined. Both patients showed evidence of either an improvement or stabilisation in their clinical condition. Plasma phytanic acid levels decreased to near normal values in approximately 6 weeks after the introduction of the diet; plasma pipecolic acid also declined markedly but the decrease was not so rapid and its level remained abnormal. C26∶C22 fatty acid ratios decreased very slowly and even after 2 years the values remained grossly abnormal. Despite the marked reduction of phytanic acid in the liver, there was an increase in the C26∶C22 fatty acid ratios and this appeared to be paralleled by an increase in inclusion bodies. Our data suggest that some patients with the infantile form of Refsum disease may show some clinical benefit from dietary management and this is reflected biochemically by decreases in the plasma levels of phytanic acid and pipecolic acid.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0878
    Keywords: Adenohypophysis, duck ; Gonadotrophes ; Immunofluorescence ; Ultrastructure
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary An indirect immunofluorescence technique and an anti-chicken LH serum were used to localize cells in the adenohypophyses of drakes at different stages of their breeding cycle, after castration, and after castration combined with thyroxine treatment. Immunofluorescent cells were distributed throughout both lobes of the adenohypophyses from control and experimental birds and were shown to be alcian blue positive, PAS negative, basophiles. Immunofluorescent cells were as numerous in castrated birds as in castrated birds treated with thyroxine. Adjacent thin and semi-thin sections were used to study the cells binding anti-LH serum at light microscope and ultrastructural levels. The cells contained spherical granules with variable densities and diameters ranging between 40 and 280 nm in the rostral (= cephalic) lobe, and between 60 and 260 nm in the caudal lobe. The light microscope and ultrastructural observations showed that the anti-LH serum binds to cells which have been classified by other authors in the Pekin duck, quail and pigeon as TSH producing delta cells. The experimental technique used did not permit a distinction to be made between cells producing FSH and LH.
    Type of Medium: Electronic Resource
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