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Psychological Aspect and Quality of Life in Porphyrias: A Review

The World Health Organization (WHO) describes “health” as a state of physical, mental, and social well-being and not merely the absence of disease or infirmity. Therefore, a biopsychosocial approach should be considered as an integral part of patients’ management. In this review, we summarize the av...

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Autores principales: Granata, Francesca, Nicolli, Annamaria, Colaiocco, Alessia, Di Pierro, Elena, Graziadei, Giovanna
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9140101/
https://www.ncbi.nlm.nih.gov/pubmed/35626348
http://dx.doi.org/10.3390/diagnostics12051193
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author Granata, Francesca
Nicolli, Annamaria
Colaiocco, Alessia
Di Pierro, Elena
Graziadei, Giovanna
author_facet Granata, Francesca
Nicolli, Annamaria
Colaiocco, Alessia
Di Pierro, Elena
Graziadei, Giovanna
author_sort Granata, Francesca
collection PubMed
description The World Health Organization (WHO) describes “health” as a state of physical, mental, and social well-being and not merely the absence of disease or infirmity. Therefore, a biopsychosocial approach should be considered as an integral part of patients’ management. In this review, we summarize the available data starting from 1986 on the biological, psychological, and social aspects of porphyrias in order to provide a useful tool for clinicians about the missing knowledge within this field. Porphyrias are a group of rare metabolic disorders affecting the heme biosynthetic pathway and can be categorized into hepatic and erythropoietic. Here, a total of 20 articles reporting the psychological and the quality of life (QoL) data of porphyria patients affected by acute hepatic porphyrias (AHPs), Porphyria Cutanea Tarda (PCT), and Erythropoietic Protoporphyria (EPP) were analyzed. These 13 articles include reported quantitative methods using questionnaires, while the reaming articles employed qualitative descriptive approaches through direct interviews with patients by psychology professionals. We conclude that the use of questionnaires limits the complete description of all areas of a patient’s life compared to a direct interview with specialists. However, only a combined use of these methods could be the best approach for the correct disorder management.
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spelling pubmed-91401012022-05-28 Psychological Aspect and Quality of Life in Porphyrias: A Review Granata, Francesca Nicolli, Annamaria Colaiocco, Alessia Di Pierro, Elena Graziadei, Giovanna Diagnostics (Basel) Review The World Health Organization (WHO) describes “health” as a state of physical, mental, and social well-being and not merely the absence of disease or infirmity. Therefore, a biopsychosocial approach should be considered as an integral part of patients’ management. In this review, we summarize the available data starting from 1986 on the biological, psychological, and social aspects of porphyrias in order to provide a useful tool for clinicians about the missing knowledge within this field. Porphyrias are a group of rare metabolic disorders affecting the heme biosynthetic pathway and can be categorized into hepatic and erythropoietic. Here, a total of 20 articles reporting the psychological and the quality of life (QoL) data of porphyria patients affected by acute hepatic porphyrias (AHPs), Porphyria Cutanea Tarda (PCT), and Erythropoietic Protoporphyria (EPP) were analyzed. These 13 articles include reported quantitative methods using questionnaires, while the reaming articles employed qualitative descriptive approaches through direct interviews with patients by psychology professionals. We conclude that the use of questionnaires limits the complete description of all areas of a patient’s life compared to a direct interview with specialists. However, only a combined use of these methods could be the best approach for the correct disorder management. MDPI 2022-05-10 /pmc/articles/PMC9140101/ /pubmed/35626348 http://dx.doi.org/10.3390/diagnostics12051193 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Review
Granata, Francesca
Nicolli, Annamaria
Colaiocco, Alessia
Di Pierro, Elena
Graziadei, Giovanna
Psychological Aspect and Quality of Life in Porphyrias: A Review
title Psychological Aspect and Quality of Life in Porphyrias: A Review
title_full Psychological Aspect and Quality of Life in Porphyrias: A Review
title_fullStr Psychological Aspect and Quality of Life in Porphyrias: A Review
title_full_unstemmed Psychological Aspect and Quality of Life in Porphyrias: A Review
title_short Psychological Aspect and Quality of Life in Porphyrias: A Review
title_sort psychological aspect and quality of life in porphyrias: a review
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9140101/
https://www.ncbi.nlm.nih.gov/pubmed/35626348
http://dx.doi.org/10.3390/diagnostics12051193
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