Administration of oral activated charcoal in variegate porphyria results in a paradoxical clinical and biochemical deterioration

British Journal of Dermatology

Volumn 149, Issue 6, 2003, Pages 1266-1269

  Hift, R J ^a   Todd, G ^a   Meissner, P N ^a   Kirsch, R E ^a  

^a UNIVERSITY OF CAPE TOWN   (South Africa)

Author keywords

Activated charcoal; Bile porphyrins; Enterohepatic cycle; Variegate porphyria

Indexed keywords

ACTIVATED CARBON; AMINOLEVULINIC ACID; CHARCOAL; PORPHOBILINOGEN; PORPHYRIN; SORBENT;

ADOLESCENT; ADULT; ANALYSIS OF VARIANCE; ARTICLE; BLOOD; CHEMICAL ANALYSIS; CLINICAL ARTICLE; CLINICAL TRIAL; COMPARATIVE STUDY; CONTROLLED CLINICAL TRIAL; CONTROLLED STUDY; DISEASE EXACERBATION; DRUG ACTIVITY; DRUG BLOOD LEVEL; DRUG URINE LEVEL; FEMALE; HEPATIC PORPHYRIA; HUMAN; INTESTINE ABSORPTION; LIVER METABOLISM; MALE; MEASUREMENT; METABOLISM; ORAL DRUG ADMINISTRATION; PATHOLOGY; PORPHYRIA VARIEGATA; PORPHYRIN METABOLISM; PRIORITY JOURNAL; PROSPECTIVE STUDY; SKIN DEFECT; SKIN DISEASE; SOUTH AFRICA; TREATMENT FAILURE; URINALYSIS; URINE;

ADMINISTRATION, ORAL; ADOLESCENT; ADULT; AMINOLEVULINIC ACID; ANALYSIS OF VARIANCE; CHARCOAL; FEMALE; HUMANS; MALE; PORPHOBILINOGEN; PORPHYRIAS, HEPATIC; PORPHYRINS; PROSPECTIVE STUDIES; SOUTH AFRICA; TREATMENT FAILURE;

EID: 0347356315     PISSN: 00070963     EISSN: None     Source Type: Journal    
DOI: 10.1111/j.1365-2133.2003.05548.x     Document Type: Article

References (16)

1. Logan GM, Weimer MK, Ellefson M et al. Bile porphyrin analysis in the evaluation of variegate porphyria. N Engl J Med 1991; 324: 1408-11.
2. Ibrahim GW, Watson CJ. Enterohepatic circulation and conversion of protoporphyrin to bile pigment in man. Proc Soc Exp Biol Med 1968; 127: 890-5.
3. Pimstone NR, Gandhi SN, Mukerji SK. Therapeutic efficacy of oral charcoal in congenital erythropoietic porphyria. N Engl J Med 1987; 316: 390-3.
4. Slathers GM. Porphyrin-binding effect of cholestyramine. Results of in-vitro and in-vivo studies. Lancet 1966; ii: 780-3.
5. Tishler PV, Gordon BJ, O'Connor JA. The adsorption of porphyrins and porphyrin precursors by sorbents: a potential therapy for the porphyrias. Meth Find Exp Clin Pharm 1982; 4: 125-31.
6. Hift RJ, Meissner PN, Kirsch RE. Effect of oral activated charcoal on the course of congenital erythropoietic porphyria. Br J Dermatol 1993; 129: 14-17.
7. Gorchein A, Foster GR. Liver failure in protoporphyria: long-term treatment with oral charcoal. Hepatology 1999; 29: 995-6.
8. Gorchein A, Guo R, Lim CK et al. Porphyrins in urine, plasma, erythrocytes, bile and faeces in a case of congenital erythropoietic porphyria (Gunther's disease) treated with blood transfusion and iron chelation: lack of benefit from oral charcoal. Biomed Chromatogr 1998; 12: 350-6.
9. Day RS, de Salamanca RE, Eales L. Quantitation of red cell porphyrins by fluorescence scanning after thin-layer chromatography. Clin Chim Acta 1978; 89: 25-33.
10. Kerr TA, Saeki S, Schneider M et al. Loss of nuclear receptor SHP impairs but does not eliminate negative feedback regulation of bile acid synthesis. Dev Cell 2002; 2: 713-20.
11. Herrick AL, Moore MR, Thompson GG et al. Cholelithiasis in patients with variegate porphyria. J Hepatol 1991; 12: 50-3.
12. Beukeveld GJJ, Meerman L, Huizenga JR et al. Determination of porphyrins in bile using high performance liquid chromatography and some clinical applications. Eur J Chem Clin Biochem 1994; 32: 153-9.
13. Beukeveld GJJ, Wolthers BG. Cholestyramine orally administered to patients with erythropoietic protoporphyria results in urinary excretion of protoporphyrin: to be explained by the Herbst-Volkheimer effect? Clin Chim Acta 1995; 233: 119-26.
14. Berenson MM, Garcia-Marin JJ, Günther C. Effect of bile acid hydroxylation on biliary protoporphyrin excretion in rat liver. Am J Physiol 1988; 255: G382-8.
15. Beukeveld GJ, in't Veld G, Havinga R et al. Relationship between biliary lipid and protoporphyrin secretion: potential role of mdr2 P-glycoprotein in hepatobiliary organic anion transport. J Hepatol 1996; 24: 343-52.
16. Lillienau J, Crombie DL, Munoz J et al. Negative feedback regulation of the ileal bile acid transport system in rodents. Gastroenterology 1993; 104: 38-46.