Therapeutic Efficacy of Oral Charcoal in Congenital Erythropoietic Porphyria

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Abstract
GüNTHER'S disease, or congenital erythropoietic porphyria, is a rare autosomal recessive disorder of heme biosynthesis characteristically associated with diminished uroporphyrinogen III cosynthetase activity.1 , 2 Disfiguring photomutilation starts in childhood. Avoidance of sunlight has been the only sure way to prevent or retard photosensitivity. The treatments that have been tried have had limited success. A partial benefit after splenectomy has been reported,3 but there is little evidence of specific, long-term improvement.4 5 6 Haining and colleagues first reported suppression of bone marrow release of porphyrins by induced polycythemia,7 supporting the notion that disease activity can be modulated by diminishing porphyrin overproduction at the source . . .