These tools will no longer be maintained as of December 31, 2024. Archived website can be found here. PubMed4Hh GitHub repository can be found here. Contact NLM Customer Service if you have questions.


Pubmed for Handhelds

PUBMED FOR HANDHELDS

Search MEDLINE/PubMed

  • Title: Immunoreactive uroporphyrinogen decarboxylase in the liver in porphyria cutanea tarda.
    Author: Elder GH, Urquhart AJ, De Salamanca RE, Munoz JJ, Bonkovsky HL.
    Journal: Lancet; 1985 Aug 03; 2(8449):229-33. PubMed ID: 2862415.
    Abstract:
    Immunoreactive and catalytic uroporphyrinogen decarboxylase were measured in liver from 15 patients with sporadic porphyria cutanea tarda (PCT) and 4 patients with familial PCT at different stages of the disorder. In sporadic PCT, catalytic activity was lowest and immunoreactive enzyme concentration was highest when active skin lesions were present; this pattern was also seen in the one familial PCT patient who had skin lesions. During remission, the ratio of catalytic activity to immunoreactive enzyme concentration returned towards normal. Immunoreactive enzyme was increased by comparison with controls in sporadic patients with skin lesions; in familial PCT mean concentration was 59% of the overall sporadic value. In 4 sporadic patients in prolonged (4-8 years) remission (following venesection) enzyme activity and immunoreactive enzyme concentrations were normal. It is suggested that clinically overt PCT is precipitated by an iron-dependent process which inactivates the active centres of uroporphyrinogen decarboxylase molecules in the liver. Treatment by venesection eventually leads to complete reversal of this biochemical defect in at least some patients with sporadic PCT. The findings are consistent with the view that sporadic PCT is an acquired disorder.
    [Abstract] [Full Text] [Related] [New Search]