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Treatment of Erythropoietic Protoporphyria With N-Acetylcysteine-Reply

Treatment of Erythropoietic Protoporphyria With N-Acetylcysteine-Reply Abstract It is not too surprising that there is a difference in the results of the studies we reported1 and the study reported above by Norris et al and the study reported by Bijlmer-Lest et al,2 as there were differences in study design and there are differences in the mechanism of action of the two compounds investigated. As to study design, we administered cysteine for 12 weeks, Norris et al administered N-acetylcysteine for 4 weeks, and Bijlmer-Lest administered N-acetylcysteine for 3 weeks, although the dosage used by both groups was higher than ours. The fact that the Dutch group found no significant alteration between groups in red blood cell— reduced glutathione and glutathione reductase would also suggest too short a period of medication. Additionally, the parameters measured differed somewhat. The Dutch group recorded the time it took to develop delayed erythema on phototest; they stated that immediate erythema on References 1. Roberts JE, Mathews-Roth MM. Cysteine ameliorates photosensitivity in erythropoietic protoporphyria . Arch Dermatol. 1993;129:1350-1351.Crossref 2. Bijlmer-Lest J, Baart de la Faille H, van Asbeck BS, van Weelden H, Marx J, Koningsberger JC. Protoporphyria photosensitivity cannot be attenuated by oral N-acetylcysteine . Photodermatol Photoimmunol Photomed. 1993;9:245-249. 3. Mathews-Roth MM, Pathak MA, Fitzpatrick TB, Harber LC, Kass EH. β-Carotene as an oral photoprotective agent in erythropoietic protoporphyria . JAMA. 1974;228:1004-1008.Crossref 4. Mathews-Roth MM, Pathak MA, Fitzpatrick TB, Harber LC, Kass EH. Phototesting as an objective measure of improvement in erythropoietic protoporphyria . Arch Dermatol. 1977;115:1381-1382. 5. Mauzerall D, Feher G. A study of the photoinduced porphyrin free radical by electron spin resonance . Biochim Biophys Acta. 1964;79:430-432.Crossref 6. Rougee M, Bensasson RV, Land EJ, Pariente R. Deactivation of singlet molecular oxygen by thiols and related compounds, possible protectors against skin photosensitization . Photochem Photobiol. 1988;47:485-490.Crossref 7. Roberts JE, Kinley JS, Young AR, Jenkins G, Atherton SJ, Dillon J. In vivo and photophysical studies on photo-oxidative damage to lens proteins and their protection by radioprotectors . Photochem Photobiol. 1991;53:33-38.Crossref 8. Dillon J, Kennedy JC, Pottier RH, Roberts JE. In vitro and in vivo protection against side effects of photodynamic therapy by radioprotective agents WR-2721 and WR-77913 . Photochem Photobiol. 1988;48:235-238.Crossref 9. Spikes JD. Quantum yields and kinetics of the photobleaching of hematoporphyrin, photofrin II, tetra(4-sulfonatophenyl)-porphine and uroporphyrin . Photochem Photobiol. 1992;55:797-808.Crossref 10. He D, Lim HW. Irradiation of porphyric mice induces down-regulation of epidermal eicosanoid metabolism . J Invest Dermatol. 1991;97:488-494.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Archives of Dermatology American Medical Association

Treatment of Erythropoietic Protoporphyria With N-Acetylcysteine-Reply

Treatment of Erythropoietic Protoporphyria With N-Acetylcysteine-Reply

Abstract

Abstract It is not too surprising that there is a difference in the results of the studies we reported1 and the study reported above by Norris et al and the study reported by Bijlmer-Lest et al,2 as there were differences in study design and there are differences in the mechanism of action of the two compounds investigated. As to study design, we administered cysteine for 12 weeks, Norris et al administered N-acetylcysteine for 4 weeks, and Bijlmer-Lest administered N-acetylcysteine for 3...
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Publisher
American Medical Association
Copyright
Copyright © 1995 American Medical Association. All Rights Reserved.
ISSN
0003-987X
eISSN
1538-3652
DOI
10.1001/archderm.1995.01690150120027
Publisher site
See Article on Publisher Site

Abstract

Abstract It is not too surprising that there is a difference in the results of the studies we reported1 and the study reported above by Norris et al and the study reported by Bijlmer-Lest et al,2 as there were differences in study design and there are differences in the mechanism of action of the two compounds investigated. As to study design, we administered cysteine for 12 weeks, Norris et al administered N-acetylcysteine for 4 weeks, and Bijlmer-Lest administered N-acetylcysteine for 3 weeks, although the dosage used by both groups was higher than ours. The fact that the Dutch group found no significant alteration between groups in red blood cell— reduced glutathione and glutathione reductase would also suggest too short a period of medication. Additionally, the parameters measured differed somewhat. The Dutch group recorded the time it took to develop delayed erythema on phototest; they stated that immediate erythema on References 1. Roberts JE, Mathews-Roth MM. Cysteine ameliorates photosensitivity in erythropoietic protoporphyria . Arch Dermatol. 1993;129:1350-1351.Crossref 2. Bijlmer-Lest J, Baart de la Faille H, van Asbeck BS, van Weelden H, Marx J, Koningsberger JC. Protoporphyria photosensitivity cannot be attenuated by oral N-acetylcysteine . Photodermatol Photoimmunol Photomed. 1993;9:245-249. 3. Mathews-Roth MM, Pathak MA, Fitzpatrick TB, Harber LC, Kass EH. β-Carotene as an oral photoprotective agent in erythropoietic protoporphyria . JAMA. 1974;228:1004-1008.Crossref 4. Mathews-Roth MM, Pathak MA, Fitzpatrick TB, Harber LC, Kass EH. Phototesting as an objective measure of improvement in erythropoietic protoporphyria . Arch Dermatol. 1977;115:1381-1382. 5. Mauzerall D, Feher G. A study of the photoinduced porphyrin free radical by electron spin resonance . Biochim Biophys Acta. 1964;79:430-432.Crossref 6. Rougee M, Bensasson RV, Land EJ, Pariente R. Deactivation of singlet molecular oxygen by thiols and related compounds, possible protectors against skin photosensitization . Photochem Photobiol. 1988;47:485-490.Crossref 7. Roberts JE, Kinley JS, Young AR, Jenkins G, Atherton SJ, Dillon J. In vivo and photophysical studies on photo-oxidative damage to lens proteins and their protection by radioprotectors . Photochem Photobiol. 1991;53:33-38.Crossref 8. Dillon J, Kennedy JC, Pottier RH, Roberts JE. In vitro and in vivo protection against side effects of photodynamic therapy by radioprotective agents WR-2721 and WR-77913 . Photochem Photobiol. 1988;48:235-238.Crossref 9. Spikes JD. Quantum yields and kinetics of the photobleaching of hematoporphyrin, photofrin II, tetra(4-sulfonatophenyl)-porphine and uroporphyrin . Photochem Photobiol. 1992;55:797-808.Crossref 10. He D, Lim HW. Irradiation of porphyric mice induces down-regulation of epidermal eicosanoid metabolism . J Invest Dermatol. 1991;97:488-494.Crossref

Journal

Archives of DermatologyAmerican Medical Association

Published: Mar 1, 1995

References