Acute intermittent porphyria
Other Names for this Disease
- HMBS deficiency
- Hydroxymethylbilane synthase deficiency
- PBGD deficiency
- Porphobilinogen deaminase deficiency
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Treatment of AIP may vary based on the trigger of the attack and the symptoms present. Treatment may include stopping medications that cause or worsen the symptoms, treating any infections which may be present, administration of pain medication, monitoring fluid balance and/or correcting electrolyte disturbances, monitoring neurologic status and administering respiratory support. Mild attacks can be manged with increased caloric intake and fluid replacement. Recurrent acute attacks should be managed by a porphyria specialist. Hospitalization is often necessary. Panhematin, an intravenous medication used to correct heme deficiency, may also be prescribed. More detailed information about the use of Panhematin for the treatment of AIP can be found by clicking here.
Last updated: 11/11/2015
- Whatley SD, Badminton MN. Acute Intermittent Porphyria. GeneReviews. February 2013; http://www.ncbi.nlm.nih.gov/books/NBK1193/. Accessed 11/11/2015.
- Acute Intermittent Porphyria (AIP). American Porphyria Foundation. 2015; http://www.porphyriafoundation.com/about-porphyria/types-of-porphyria/AIP. Accessed 11/11/2015.
- Treatment Options. American Porphyria Foundation. 2015; http://www.porphyriafoundation.com/treatment. Accessed 11/11/2015.
- Finding Treatment Information - A video developed by GARD Information Specialists that explains how you can find information about treatment for a rare disease.
- GeneReviews provides current, expert-authored, peer-reviewed, full-text articles describing the application of genetic testing to the diagnosis, management, and genetic counseling of patients with specific inherited conditions. Click on the link to view the article on this topic.
- Orphanet Emergency Guidelines is an article which is expert-authored and peer-reviewed that is intended to guide health care professionals in emergency situations involving this condition.
- 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.
- ClinicalTrials.gov lists trials that are studying or have studied Acute intermittent porphyria. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.
The FDA has approved this product to be used in this manner.
|Amelioration of recurrent attacks of acute intermittent porphyria (AIP) temporarily related to the menstrual cycle in susceptible women and similar symptoms which occur in other patients with AIP, porphyria variegata and hereditary coproporphyria.|
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