Rapid improvement in the chemical pathology of congenital erythropoietic porphyria with treatment with superactivated charcoal.
Non-absorbable sorbents that bind porphyrins in the gastrointestinal tract may be useful in the treatment of porphyrias whose manifestations result from porphyrin excess. To test this, we assessed the effect of oral charcoal on porphyrin economy in a patient with a probable congenital erythropoietic porphyria. Treatment with a superactivated charcoal (Super Char), 25 g three times daily, was associated with a precipitous drop in erythrocyte porphyrin (from 21.4 +/- 2.9 [SD] to 7.4 +/- 0.4 nmole/ml; p less than 0.025) and plasma porphyrin (from 1.56 +/- 0.24 to 0.70 +/- 0.08 nmole/ml; p less than 0.01). Urinary porphyrin excretion appeared to rise, from 103 +/- 45 to 160 +/- 30 mumole/d, but the change was not statistically significant. Constipation appeared to limit compliance with the charcoal regimen by the end of the study period. Nonetheless, superactivated charcoal may be a useful therapy in this disfiguring porphyria.
Tishler
P V
PV
Brockton/West Roxbury Veterans Administration Medical Center, MA.
Winston
S H
SH
D000293:Adolescent; D002606:Charcoal / Q000009:adverse effects / Q000627:therapeutic use*; D003248:Constipation / Q000139:chemically induced; D006801:Humans; D008297:Male; D011164:Porphyrias / Q000151:congenital / Q000188:drug therapy*; D011166:Porphyrins / Q000097:blood* / Q000652:urine; D012871:Skin Diseases / Q000151:congenital / Q000188:drug therapy*
D011166:Porphyrins; D002606:Charcoal