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The porphyrias 

The porphyrias

Chapter:
The porphyrias
Author(s):

T.M. Cox

DOI:
10.1093/med/9780199204854.003.1205_update_001

Update:

Expanded discussion of (1) role of sex-steroid metabolism in acute neurovisceral attacks; (2) incidence of inherited porphyrias; (3) long term sequelae of acute porphyrias; (4) treatment of congenital erythropoietic porphyria.

Updated on 28 Nov 2013. The previous version of this content can be found here.
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date: 18 August 2017

The porphyrias are metabolic disorders characterized by overproduction of haem precursors, principally in the liver and bone marrow. Most porphyrias are inborn errors that affect enzymatic steps in a tightly regulated biosynthetic pathway for haem; acquired forms also occur.

Hepatic synthesis of haem undergoes rapid and wide oscillations, but haem formation for erythropoiesis is generally constant; it increases many fold as the erythron expands to meet the demands of blood loss or destruction, or as a consequence of ineffective erythropoiesis....

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