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  • Kidney transplant  (2)
  • Peroxisomes  (2)
  • Acrylamide  (1)
  • 1
    ISSN: 1432-2307
    Keywords: Peroxisomes ; Peroxisomal disorders ; Neonatal adrenoleukodystrophy ; Infantile Refsum's disease ; Pseudo-Zellweger disease ; Mitochondria
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The morphology of hepatic peroxisomes in five patients with metabolic disorders believed to be due to inherited defects of peroxisomal function or biogenesis is described. Electron microscopy and cytochemical staining for catalase were used to identify peroxisomes in two boys with infantile Refsum's disease (IRD), a girl with autopsy confirmed neonatal adrenoleukodystrophy (NALD), and two boys with pseudo-Zellweger syndrome (PZS). In the patients with IRD and NALD hepatic peroxisomes were significantly reduced in size and number and contained electron dense centres. In the liver of the patients with PZS the peroxisomes were enlarged. Morphologically abnormal peroxisomes were also detected in autopsy tissue from one boy with PZS using electron microscopy. Lamellar-lipid inclusions and mitochondria with crystalline inclusions and/or abnormal cristae are also described in two patients, one with IRD, the other with NALD.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    BBA - Protein Structure 671 (1981), S. 117-122 
    ISSN: 0005-2795
    Keywords: Acrylamide ; Azurin B ; Fluorescence quenching
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Biology
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-1076
    Keywords: Growth ; Kidney transplant ; Children ; Adolescents
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Factors affecting the growth rates of 59 children and adolescents for the first 2 years following kidney transplantation were evaluated. The factors assessed were age at transplantation, renal function, prednisone dosage, donor source, and prior history of transplantation. The observed growth velocity was expressed as the percentage, of the growth velocity predicted by bone age. Normal growth (≥80%) was exhibited by 37% of the patients and 22% had accelerated growth (≥100%). The chronologic age at transplantation did not correlate significantly with growth when bone age was used as the reference for expected velocity. Males grew better than did females. There was a unique sex/race interaction with black males growing most rapidly. Better renal function, the ability to lower prednisone dosage, alternate day prednisone administration, and a decreasing diastolic blood pressure were positively correlated with better growth rates after transplantation. The donor source and prior history of transplantation did not significantly influence grwoth rate.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-1076
    Keywords: Growth ; Kidney transplant ; Children ; Adolescents
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Factors affecting the growth rates of 59 children and adolescents for the first 2 years following kidney transplantation were evaluated. The factors assessed were age at transplantation, renal function, prednisone dosage, donor source, and prior history of transplantation. The observed growth velocity was expressed as the percentage of the growth velocity predicted by bone age. Normal growth (≥80%) was exhibited by 37% of the patients and 22% had accelerated growth (≥100%). The chronological age at transplantation did not correlate significantly with growth when bone age was used as the reference for expected velocity. Males grew better than did females. There was a unique sex/race interaction with black males growing most rapidly. Better renal function, the ability to lower prednisone dosage, alternate day prednisone, and a decreasing diastolic blood pressure were positively correlated with better growth rates after transplantation. Donor source and prior history of transplantation did not significantly influence growth rate.
    Type of Medium: Electronic Resource
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  • 5
    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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