PMID: 9638723Jun 25, 1998Paper

Management of the acute porphyrias

Photodermatology, Photoimmunology & Photomedicine
R Kauppinen

Abstract

Three hepatic porphyrias--acute intermittent porphyria, hereditary coproporphyria and variegate porphyria--are characterized by episodic acute attacks that consist of various neuro-psychiatric symptoms and signs, such as abdominal pain, vomiting, constipation, hypertension and tachycardia associated with increased excretion of porphyrins and porphyrin precursors. Peripheral neuropathy is manifested as pain in the extremities, and it may progress to a severe motor neuropathy. Measurement of porphobilinogen in the urine gives a prompt diagnosis during acute attacks. Attacks are often induced by precipitating factors such as drugs, alcohol, infection, fasting or changes in sex-hormone balance, and they should be eliminated when a patient is treated during an attack. Heme, the end biosynthetic product, is the most effective therapy for restoration of porphyrin biosynthesis to normal, and it is usually infused at 3 mg/kg daily for 4 days. Adequate calories are necessary and parenteral nutrition with carbohydrates may be necessary. Attacks may also require therapy for hypertension, pain and epileptic seizures. Strict avoidance of all precipitating factors may not be necessary in the asymptomatic phase.

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Citations

Jan 26, 2006·Journal of Inherited Metabolic Disease·L M MillwardT J Peters
Nov 14, 2000·Clinical Biochemistry·A KühnelM O Doss
Dec 19, 2000·Free Radical Biology & Medicine·M E RochaE J Bechara
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Feb 19, 2009·American Journal of Clinical Dermatology·Miranda A FarageHoward I Maibach
Sep 1, 2003·Irish Journal of Psychological Medicine·Dympna GibbonsMalcom Garland

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