Nutroterapia na porfiria aguda intermitente: Da UTI ao ambulatório
Autor(a) principal: | |
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Data de Publicação: | 2022 |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | International Journal of Nutrology (Online) |
Texto Completo: | https://ijn.zotarellifilhoscientificworks.com/index.php/ijn/article/view/177 |
Resumo: | Acute intermittent porphyria is a rare genetic disease, autosomal dominant, with variable penetrance, which causes error in the heme metabolism pathway of the liver, with accumulation of intermediate metabolites that are toxic to the body. It is manifested by crisis with multisystem symptoms, and diagnosis is often difficult. The main findings are severe abdominal pain, polyneuropathy, autonomic dysfunction, and neuropsychiatric disorders. It ranges from mild to very severe with malignant arrhythmia and bulbar paralysis with respiratory failure requiring mechanical ventilation and admission to the intensive care unit. The diagnosis is confirmed biochemistry and genetics. Treatment of the crisis is done away from precipitating factors and specifically with a diet rich in carbohydrates and hematin, with positive effect on the heme biosynthetic pathway. Within the multidisciplinary team, it must be highlighted the role of the nutrition specialist doctor, who evaluates and guides the nutrition therapy, both in crises and in the clinical monitoring of these patients. |
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International Journal of Nutrology (Online) |
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Nutroterapia na porfiria aguda intermitente: Da UTI ao ambulatórioacute intermittent porphyrianutritionAcute intermittent porphyria is a rare genetic disease, autosomal dominant, with variable penetrance, which causes error in the heme metabolism pathway of the liver, with accumulation of intermediate metabolites that are toxic to the body. It is manifested by crisis with multisystem symptoms, and diagnosis is often difficult. The main findings are severe abdominal pain, polyneuropathy, autonomic dysfunction, and neuropsychiatric disorders. It ranges from mild to very severe with malignant arrhythmia and bulbar paralysis with respiratory failure requiring mechanical ventilation and admission to the intensive care unit. The diagnosis is confirmed biochemistry and genetics. Treatment of the crisis is done away from precipitating factors and specifically with a diet rich in carbohydrates and hematin, with positive effect on the heme biosynthetic pathway. Within the multidisciplinary team, it must be highlighted the role of the nutrition specialist doctor, who evaluates and guides the nutrition therapy, both in crises and in the clinical monitoring of these patients.MetaScience Press2022-03-06info:eu-repo/semantics/articleinfo:eu-repo/semantics/publishedVersionapplication/pdfhttps://ijn.zotarellifilhoscientificworks.com/index.php/ijn/article/view/17710.1055/s-0039-1693736International Journal of Nutrology; Vol. 12 No. 1 (2019): International Journal of Nutrology (IJN) - September 2019; 18-222595-28541984-301110.1055/s-009-44420reponame:International Journal of Nutrology (Online)instname:Associação Brasileira de Nutrologia (ABRAN)instacron:ABRANenghttps://ijn.zotarellifilhoscientificworks.com/index.php/ijn/article/view/177/172Copyright (c) 2022 International Journal of Nutrologyinfo:eu-repo/semantics/openAccessSilva Filho, Paulo Venancio da2022-03-06T14:20:35Zoai:ojs2.ijn.zotarellifilhoscientificworks.com:article/177Revistahttps://ijn.zotarellifilhoscientificworks.com/index.php/ijnONGhttps://ijn.zotarellifilhoscientificworks.com/index.php/ijn/oaiijn@zotarellifilhoscientificworks.com || editorchief@zotarellifilhoscientificworks.com10.544482595-28541984-3011opendoar:2022-03-06T14:20:35International Journal of Nutrology (Online) - Associação Brasileira de Nutrologia (ABRAN)false |
dc.title.none.fl_str_mv |
Nutroterapia na porfiria aguda intermitente: Da UTI ao ambulatório |
title |
Nutroterapia na porfiria aguda intermitente: Da UTI ao ambulatório |
spellingShingle |
Nutroterapia na porfiria aguda intermitente: Da UTI ao ambulatório Silva Filho, Paulo Venancio da acute intermittent porphyria nutrition |
title_short |
Nutroterapia na porfiria aguda intermitente: Da UTI ao ambulatório |
title_full |
Nutroterapia na porfiria aguda intermitente: Da UTI ao ambulatório |
title_fullStr |
Nutroterapia na porfiria aguda intermitente: Da UTI ao ambulatório |
title_full_unstemmed |
Nutroterapia na porfiria aguda intermitente: Da UTI ao ambulatório |
title_sort |
Nutroterapia na porfiria aguda intermitente: Da UTI ao ambulatório |
author |
Silva Filho, Paulo Venancio da |
author_facet |
Silva Filho, Paulo Venancio da |
author_role |
author |
dc.contributor.author.fl_str_mv |
Silva Filho, Paulo Venancio da |
dc.subject.por.fl_str_mv |
acute intermittent porphyria nutrition |
topic |
acute intermittent porphyria nutrition |
description |
Acute intermittent porphyria is a rare genetic disease, autosomal dominant, with variable penetrance, which causes error in the heme metabolism pathway of the liver, with accumulation of intermediate metabolites that are toxic to the body. It is manifested by crisis with multisystem symptoms, and diagnosis is often difficult. The main findings are severe abdominal pain, polyneuropathy, autonomic dysfunction, and neuropsychiatric disorders. It ranges from mild to very severe with malignant arrhythmia and bulbar paralysis with respiratory failure requiring mechanical ventilation and admission to the intensive care unit. The diagnosis is confirmed biochemistry and genetics. Treatment of the crisis is done away from precipitating factors and specifically with a diet rich in carbohydrates and hematin, with positive effect on the heme biosynthetic pathway. Within the multidisciplinary team, it must be highlighted the role of the nutrition specialist doctor, who evaluates and guides the nutrition therapy, both in crises and in the clinical monitoring of these patients. |
publishDate |
2022 |
dc.date.none.fl_str_mv |
2022-03-06 |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://ijn.zotarellifilhoscientificworks.com/index.php/ijn/article/view/177 10.1055/s-0039-1693736 |
url |
https://ijn.zotarellifilhoscientificworks.com/index.php/ijn/article/view/177 |
identifier_str_mv |
10.1055/s-0039-1693736 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
https://ijn.zotarellifilhoscientificworks.com/index.php/ijn/article/view/177/172 |
dc.rights.driver.fl_str_mv |
Copyright (c) 2022 International Journal of Nutrology info:eu-repo/semantics/openAccess |
rights_invalid_str_mv |
Copyright (c) 2022 International Journal of Nutrology |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
MetaScience Press |
publisher.none.fl_str_mv |
MetaScience Press |
dc.source.none.fl_str_mv |
International Journal of Nutrology; Vol. 12 No. 1 (2019): International Journal of Nutrology (IJN) - September 2019; 18-22 2595-2854 1984-3011 10.1055/s-009-44420 reponame:International Journal of Nutrology (Online) instname:Associação Brasileira de Nutrologia (ABRAN) instacron:ABRAN |
instname_str |
Associação Brasileira de Nutrologia (ABRAN) |
instacron_str |
ABRAN |
institution |
ABRAN |
reponame_str |
International Journal of Nutrology (Online) |
collection |
International Journal of Nutrology (Online) |
repository.name.fl_str_mv |
International Journal of Nutrology (Online) - Associação Brasileira de Nutrologia (ABRAN) |
repository.mail.fl_str_mv |
ijn@zotarellifilhoscientificworks.com || editorchief@zotarellifilhoscientificworks.com |
_version_ |
1792204588294602752 |