Porphyria
Definition
Porphyrias are a rare group of disorders passed down through families in which an important part of hemoglobin, called heme, does not develop properly. Heme is also found in myoglobin, a protein found in certain muscles.
Alternative Names
Acute intermittent porphyria; Hereditary coproporphyria; Congenital erythropoietic porphyria; Erythropoietic protoporphyriaCauses
Normally, the body breaks down chemicals, called porphyrins, into heme. The porphyrins leave the body through urine or stools. But persons with porphyria have a genetic defect that interrupts this process. As a result, porphyrins build up in the body. This can lead to rashes, light sensitivity, abdominal pain, and other symptoms.
There are many different forms of porphyria. The most common type is porphyria cutanea tarda.
Drugs, infection, alcohol, and hormones such as estrogen may trigger attacks of certain types of porphyria.
References
Hoffman R, Benz EJ, Shattil SS, et al. Hematology: Basic Principles and Practice. 4th ed. Orlando, Fl: Churchill Livingstone; 2005.
Dombeck TA. The porphyrias. Emerg Med Clin North Am. Aug 2005; 23(3): 885-99, x.
A.D.A.M., Inc. is accredited by
URAC, also known as the American
Accreditation HealthCare Commission. URAC's accreditation program is
the first of its kind, requiring compliance with 53 standards of quality and
accountability, verified by independent audit.
The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2005 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.