Porphyria classification: Difference between revisions
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{{Porphyria}} | {{Porphyria}} | ||
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==Overview== | ==Overview== | ||
==Classification== | ==Classification== | ||
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|bgcolor="#eeeeee"| ''' | |bgcolor="#eeeeee"| '''Enzyme''' | ||
|bgcolor="#eeeeee"| '''Location of enzyme''' | |bgcolor="#eeeeee"| '''Location of enzyme''' | ||
|bgcolor="#eeeeee"| '''Associated porphyria''' | |bgcolor="#eeeeee"| '''Associated porphyria''' | ||
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==References== | ==References== | ||
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[[Category:Dermatology]] | |||
[[Category:Gastroenterology]] | |||
[[Category:Hematology]] | |||
[[Category:Endocrinology]] | |||
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{{WS}} | {{WS}} | ||
Latest revision as of 13:30, 22 July 2016
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Porphyria Microchapters |
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Porphyria classification On the Web |
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American Roentgen Ray Society Images of Porphyria classification |
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Risk calculators and risk factors for Porphyria classification |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
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Overview
Classification
The 8 porphyrias are:
- Acute intermittent Porphyria (AIP)
- ALA dehydratase deficiency
- Porphyria cutanea tarda (PCT)
- Congenital Erythropoetic Porphyria (CEP)
- Hepatoerythropoetic Porphyria (HEP)
- Erythopoetic Protoporphyria (EPP)
- Hereditary coproporphyria (HCP)
- Variegate Porphyria (VP)
These disorders can also be divided based on the type of precursors that accumulate and the subsequent manifestations that occur. ALA dehydratase deficiency (Plumboporphyria) and AIP are characterized by the accumulation of -aminolevulinic acid (ALA) and Porphobilinogen (PBG). Their manifestations are primarily neuropathic. PCT, CEP, HEP and EEP are characterized by accumulation of porphyrin and have cutaneous manifestations only. HCP and VP have accumulation of porphyrin precursors and porphyrins. They have both neuropathic and cutaneous manifestations.
| Enzyme | Location of enzyme | Associated porphyria | Type of porphyria |
| δ-aminolevulinate (ALA) synthase | Mitochondrion | X-linked sideroblastic anemia (XLSA) | Erythropoietic |
| δ-aminolevulinate (ALA) dehydratase | Cytosol | Doss porphyria/ALA dehydratase deficiency | Hepatic |
| hydroxymethylbilane (HMB) synthase (or PBG deaminase) | Cytosol | acute intermittent porphyria (AIP) | Hepatic |
| uroporphyrinogen (URO) synthase | Cytosol | Congenital erythropoietic porphyria (CEP) | Erythropoeitic |
| uroporphyrinogen (URO) decarboxylase | Cytosol | Porphyria cutanea tarda (PCT) | Hepatic |
| coproporphyrinogen (COPRO) oxidase | Mitochondrion | Hereditary coproporphyria (HCP) | Hepatic |
| protoporphyrinogen (PROTO) oxidase | Mitochondrion | Variegate porphyria (VP) | Mixed |
| Ferrochelatase | Mitochondrion | Erythropoietic protoporphyria (EPP) | Erythropoietic |