[Treatment of the porphyrias].

Abstract

There are seven porphyrias which are caused by defective functions of the enzymes in the haem biosynthesis. The clinical classification of porphyrias are divided into three types which are neuroporphyria, neurocutaneous porphyria and cutaneous porphyria. For acute porphyric attack of neuroporphyria and neurocutaneous porphyria, the treatments of choice are administration of glucose and/or hematin, haem arginate and tinprotoporphyrin. Porphyria cutanea tarda in cutaneous porphyria is controlled by removal of iron by phrebotomies or low-dose chloriquine. A novel approach of intravenous administration of interferon may be useful to control the associated case of chronic hepatitis type C. Skin symptoms in erythropoietic protoporphyria can be alleviated with beta carotene. Hepatic failure due to protoporphyria may need a liver transplantation. Cimetidine, a H2-receptor antagonist, may be useful in the treatment of acute porphyric attack and in remission stage in neuroporphyrias, neurocutaneous porphyrias and cutaneous porphyrias such as porphyria cutanea tarda and protoporphyria.

• Authors: Y Horie, J Okano, S Kitaoka, F Tajima, H Kawasaki
• Journal: Nihon rinsho. Japanese journal of clinical medicine (Nihon Rinsho) Vol. 53 Issue 6 Pg. 1456-62 (Jun 1995) ISSN: 0047-1852 [Print] Japan
• PMID: 7542353 (Publication Type: English Abstract, Journal Article, Review)

Chemical References

• beta Carotene
• Carotenoids
• Hemin
• Cimetidine
• Interferons
• Glucose

Topics

• Bloodletting
• Carotenoids (therapeutic use)
• Cimetidine (therapeutic use)
• Female
• Glucose (administration & dosage)
• Hemin (administration & dosage)
• Humans
• Interferons (therapeutic use)
• Liver Transplantation
• Male
• Porphyrias (classification, therapy)
• beta Carotene