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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Variegate porphyria


Other Names for this Disease
  • Porphyria variegate
  • Porphyria, South African type
  • PPOX deficiency
  • Protoporphyrinogen oxidase deficiency
  • VP
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Treatment


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How is variegate porphyria treated?

Acute attacks are managed and may be prevented as in acute intermittent porphyria.[1][2] Hospitalization is often necessary for acute attacks. Medications for pain, nausea and vomiting, and close observation are generally required. A high intake of glucose or other carbohydrates can help suppress disease activity and can be given by vein or by mouth. Intravenous heme therapy is more potent in suppressing disease activity. It can be started after a trial of glucose therapy. However, the response to heme therapy is best if started early in an attack. Heme must be administered by vein. Panhematin is the only commercially available heme therapy for treatment and prevention of acute porphyric attacks in the United States. Heme arginate, which is marketed in some other countries, is another preparation of heme for intravenous administration.[3]
Last updated: 7/19/2010

References
  1. Variegate Porphyria (VP). American Porphyria Foundation. 2010; http://www.porphyriafoundation.com/about-porphyria/types-of-porphyria/VP. Accessed 7/19/2010.
  2. Porphyria, Variegate. National Organization for Rare Disorders (NORD). 2001; http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=Porphyria%2C%20Variegate. Accessed 7/19/2010.
  3. Acute Intermittent Porphyria (AIP). American Porphyria Foundation. 2010; http://www.porphyriafoundation.com/about-porphyria/types-of-porphyria/AIP. Accessed 7/19/2010.


Management Guidelines

  • The American Porphyria Foundation offers a document that includes information about porphyria, types, testing, and treatment with Panhematin®.  Click the "document" link above to view these guidelines.