Κυριακή 12 Σεπτεμβρίου 2010

Porphyria

I was reading about the skeletal muscle relaxants and I came across with the term porphyria.
According to BNF54 acute porphyria is a hereditary disorder of haem biosynthesis. Subdivisions include: Acute intermittent porphyria (AIP), Variegate porphyria (VP), Hereditary coproporphyria (HCP), or ALA dehydratase deficiency porphyria.

There are drugs can cause acute porphyrias and their use must be avoided. If that is not possible urinary porphobilinogen excretion should be measured regularly.

A list of drugs safe to use in acute porphyrias can be found here:
http://www.wmic.wales.nhs.uk/pdfs/porphyria/Porphyria_Safe_List_2010_with_letter_August%202010.pdf


In acute porphyria crises, haema arginate (normosang) is given as an IV infusion.

More information can be found here:
http://www.wmic.wales.nhs.uk/porphyria_info.php

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