Abstract
PORPHYRIAS: TYPES AND TREATMENT

*Devu Karun and Mrs. Meppil Baby

ABSTRACT

Porphyrins are metabolic disorders of the heme biosynthesis. Porphyrins are essential for the function of hemoglobin, a protein present in the red blood cells that is linked to porphyrin, and binds with iron thus carrying oxygen to the organs and tissues. Clinically they can be differentiated into acute and non-acute porphyrias. But the lack of awareness and adequate knowledge about the clinical features of porphyria results in delayed diagnostic and therapeutic outcomes in the patient. Porphyrias still remain undiagnosed, although the examinations of plasma and urine are the first line tests for the detection of excess amounts of porphyrin in the body. This review discusses about the porphyria and its metabolism, different types of porphyria with its clinical manifestations and therapeutic management of porphyria.

Keywords: Porphyria, Porphyrin, Hepatic porphyria, Erythropoietic porphyria, Aminolaevulinic acid (ALA), Porphobilinogen, Photosensitivity, Phlebotomy.


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