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Desensitization in patients with hypersensitivity to haem arginate: A case report

BACKGROUND: Porphyria comprises a group of metabolic disorders caused by the irregular activities of enzymes within the haem biosynthetic pathway. This disease can provoke a large variety of symptoms. Acute porphyria attacks need to be treated urgently to avoid prolonged illness and fatal complicati...

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Autores principales: Chapman, Edgardo, Leal, Drixie, Matijasevic, Eugenio, García, Elizabeth
Formato: Online Artículo Texto
Lenguaje:English
Publicado: World Allergy Organization 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439413/
https://www.ncbi.nlm.nih.gov/pubmed/30937127
http://dx.doi.org/10.1016/j.waojou.2018.11.002
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author Chapman, Edgardo
Leal, Drixie
Matijasevic, Eugenio
García, Elizabeth
author_facet Chapman, Edgardo
Leal, Drixie
Matijasevic, Eugenio
García, Elizabeth
author_sort Chapman, Edgardo
collection PubMed
description BACKGROUND: Porphyria comprises a group of metabolic disorders caused by the irregular activities of enzymes within the haem biosynthetic pathway. This disease can provoke a large variety of symptoms. Acute porphyria attacks need to be treated urgently to avoid prolonged illness and fatal complications. Haem arginate, a concentrated haem solution stabilized with arginine, is the only preparation available for treatment in Europe and South America. This report describes a safe desensitization protocol for patients who require such treatment and have haem arginate hypersensitivity. CASE PRESENTATION: A 25-year-old female patient diagnosed with acute intermittent porphyria, who had an anaphylactic reaction while receiving haem arginate. The patient was treated with a desensitization protocol for patients with hypersensitivity to haem arginate. CONCLUSION: Porphyria is a disease that can significantly compromise a patient's quality of life. The desensitization protocol for patients with hypersensitivity to haem arginate is a safe and effective treatment option for patients with a history of haem arginate allergies, to whom it is not possible to administer haematin.
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spelling pubmed-64394132019-04-01 Desensitization in patients with hypersensitivity to haem arginate: A case report Chapman, Edgardo Leal, Drixie Matijasevic, Eugenio García, Elizabeth World Allergy Organ J Article BACKGROUND: Porphyria comprises a group of metabolic disorders caused by the irregular activities of enzymes within the haem biosynthetic pathway. This disease can provoke a large variety of symptoms. Acute porphyria attacks need to be treated urgently to avoid prolonged illness and fatal complications. Haem arginate, a concentrated haem solution stabilized with arginine, is the only preparation available for treatment in Europe and South America. This report describes a safe desensitization protocol for patients who require such treatment and have haem arginate hypersensitivity. CASE PRESENTATION: A 25-year-old female patient diagnosed with acute intermittent porphyria, who had an anaphylactic reaction while receiving haem arginate. The patient was treated with a desensitization protocol for patients with hypersensitivity to haem arginate. CONCLUSION: Porphyria is a disease that can significantly compromise a patient's quality of life. The desensitization protocol for patients with hypersensitivity to haem arginate is a safe and effective treatment option for patients with a history of haem arginate allergies, to whom it is not possible to administer haematin. World Allergy Organization 2019-01-26 /pmc/articles/PMC6439413/ /pubmed/30937127 http://dx.doi.org/10.1016/j.waojou.2018.11.002 Text en © 2019 The Authors http://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Article
Chapman, Edgardo
Leal, Drixie
Matijasevic, Eugenio
García, Elizabeth
Desensitization in patients with hypersensitivity to haem arginate: A case report
title Desensitization in patients with hypersensitivity to haem arginate: A case report
title_full Desensitization in patients with hypersensitivity to haem arginate: A case report
title_fullStr Desensitization in patients with hypersensitivity to haem arginate: A case report
title_full_unstemmed Desensitization in patients with hypersensitivity to haem arginate: A case report
title_short Desensitization in patients with hypersensitivity to haem arginate: A case report
title_sort desensitization in patients with hypersensitivity to haem arginate: a case report
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6439413/
https://www.ncbi.nlm.nih.gov/pubmed/30937127
http://dx.doi.org/10.1016/j.waojou.2018.11.002
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