Practical Recommendations in the Treatment of Acute and Chronic Life-Threatening Infectious Diseases in Patients with Acute Hepatic Porphyria
Background: Acute hepatic porphyrias (AHPs) represent inherited metabolic disorders of the heme biosynthesis pathway, leading to neurological and systemic impairment. Despite the presence of well-recognized chronic symptoms and signs, acute neurological, both neuromuscular and central neurological c...
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2025-02-01
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| author | Bruno de Mattos Lombardi Badia Paulo de Lima Serrano João Paulo Barile Daniel Delgado Seneor Patrícia Marques Mendes Renan Brandão Rambaldi Cavalheiro Kaliny Oliveira Peixoto Igor Braga Farias Roberta Ismael Lacerda Machado Wladimir Bocca Vieira de Rezende Pinto Acary Souza Bulle Oliveira Paulo Sgobbi |
| author_facet | Bruno de Mattos Lombardi Badia Paulo de Lima Serrano João Paulo Barile Daniel Delgado Seneor Patrícia Marques Mendes Renan Brandão Rambaldi Cavalheiro Kaliny Oliveira Peixoto Igor Braga Farias Roberta Ismael Lacerda Machado Wladimir Bocca Vieira de Rezende Pinto Acary Souza Bulle Oliveira Paulo Sgobbi |
| author_sort | Bruno de Mattos Lombardi Badia |
| collection | DOAJ |
| description | Background: Acute hepatic porphyrias (AHPs) represent inherited metabolic disorders of the heme biosynthesis pathway, leading to neurological and systemic impairment. Despite the presence of well-recognized chronic symptoms and signs, acute neurological, both neuromuscular and central neurological complications pose a significant challenge in clinical practice, with a potential risk of greater severity and mortality during acute decompensation episodes of AHPs. Care related to the prescription of medications, considering the risk of porphyrinogenicity, is a major and recurring concern in the acute and chronic management of AHP patients. Infectious clinical complications are significant issues in both outpatient and hospital settings for patients with AHPs. It is crucial to identify therapeutic regimens with the best safety and efficacy profiles for treating such infectious complications in AHP patients. The scarcity of structured knowledge available in guidelines and recommendations often leads to the use of therapeutic options with higher potential risks in treating patients with AHPs. Objectives: This review article aims to provide practical recommendations for managing the most significant infectious complications in clinical practice, with a focus on their impact on the clinical care of patients with AHPs. |
| format | Article |
| id | doaj-art-ac31dd762adc44caab715d156ee680bc |
| institution | DOAJ |
| issn | 2218-1989 |
| language | English |
| publishDate | 2025-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Metabolites |
| spelling | doaj-art-ac31dd762adc44caab715d156ee680bc2025-08-20T03:12:05ZengMDPI AGMetabolites2218-19892025-02-011529910.3390/metabo15020099Practical Recommendations in the Treatment of Acute and Chronic Life-Threatening Infectious Diseases in Patients with Acute Hepatic PorphyriaBruno de Mattos Lombardi Badia0Paulo de Lima Serrano1João Paulo Barile2Daniel Delgado Seneor3Patrícia Marques Mendes4Renan Brandão Rambaldi Cavalheiro5Kaliny Oliveira Peixoto6Igor Braga Farias7Roberta Ismael Lacerda Machado8Wladimir Bocca Vieira de Rezende Pinto9Acary Souza Bulle Oliveira10Paulo Sgobbi11Division of Neuromuscular Diseases, Porphyria Unit, Federal University of São Paulo (UNIFESP), São Paulo 04039-060, SP, BrazilDivision of Neuromuscular Diseases, Porphyria Unit, Federal University of São Paulo (UNIFESP), São Paulo 04039-060, SP, BrazilDivision of Neuromuscular Diseases, Porphyria Unit, Federal University of São Paulo (UNIFESP), São Paulo 04039-060, SP, BrazilDivision of Neuromuscular Diseases, Porphyria Unit, Federal University of São Paulo (UNIFESP), São Paulo 04039-060, SP, BrazilDivision of Neuromuscular Diseases, Porphyria Unit, Federal University of São Paulo (UNIFESP), São Paulo 04039-060, SP, BrazilDivision of Neuromuscular Diseases, Porphyria Unit, Federal University of São Paulo (UNIFESP), São Paulo 04039-060, SP, BrazilDivision of Neuromuscular Diseases, Porphyria Unit, Federal University of São Paulo (UNIFESP), São Paulo 04039-060, SP, BrazilDivision of Neuromuscular Diseases, Porphyria Unit, Federal University of São Paulo (UNIFESP), São Paulo 04039-060, SP, BrazilDivision of Neuromuscular Diseases, Porphyria Unit, Federal University of São Paulo (UNIFESP), São Paulo 04039-060, SP, BrazilDivision of Neuromuscular Diseases, Porphyria Unit, Federal University of São Paulo (UNIFESP), São Paulo 04039-060, SP, BrazilDivision of Neuromuscular Diseases, Porphyria Unit, Federal University of São Paulo (UNIFESP), São Paulo 04039-060, SP, BrazilDivision of Neuromuscular Diseases, Porphyria Unit, Federal University of São Paulo (UNIFESP), São Paulo 04039-060, SP, BrazilBackground: Acute hepatic porphyrias (AHPs) represent inherited metabolic disorders of the heme biosynthesis pathway, leading to neurological and systemic impairment. Despite the presence of well-recognized chronic symptoms and signs, acute neurological, both neuromuscular and central neurological complications pose a significant challenge in clinical practice, with a potential risk of greater severity and mortality during acute decompensation episodes of AHPs. Care related to the prescription of medications, considering the risk of porphyrinogenicity, is a major and recurring concern in the acute and chronic management of AHP patients. Infectious clinical complications are significant issues in both outpatient and hospital settings for patients with AHPs. It is crucial to identify therapeutic regimens with the best safety and efficacy profiles for treating such infectious complications in AHP patients. The scarcity of structured knowledge available in guidelines and recommendations often leads to the use of therapeutic options with higher potential risks in treating patients with AHPs. Objectives: This review article aims to provide practical recommendations for managing the most significant infectious complications in clinical practice, with a focus on their impact on the clinical care of patients with AHPs.https://www.mdpi.com/2218-1989/15/2/99acute hepatic porphyriainfectious disease medicineanti-infective agentsporphyrinsacute intermittent porphyria |
| spellingShingle | Bruno de Mattos Lombardi Badia Paulo de Lima Serrano João Paulo Barile Daniel Delgado Seneor Patrícia Marques Mendes Renan Brandão Rambaldi Cavalheiro Kaliny Oliveira Peixoto Igor Braga Farias Roberta Ismael Lacerda Machado Wladimir Bocca Vieira de Rezende Pinto Acary Souza Bulle Oliveira Paulo Sgobbi Practical Recommendations in the Treatment of Acute and Chronic Life-Threatening Infectious Diseases in Patients with Acute Hepatic Porphyria Metabolites acute hepatic porphyria infectious disease medicine anti-infective agents porphyrins acute intermittent porphyria |
| title | Practical Recommendations in the Treatment of Acute and Chronic Life-Threatening Infectious Diseases in Patients with Acute Hepatic Porphyria |
| title_full | Practical Recommendations in the Treatment of Acute and Chronic Life-Threatening Infectious Diseases in Patients with Acute Hepatic Porphyria |
| title_fullStr | Practical Recommendations in the Treatment of Acute and Chronic Life-Threatening Infectious Diseases in Patients with Acute Hepatic Porphyria |
| title_full_unstemmed | Practical Recommendations in the Treatment of Acute and Chronic Life-Threatening Infectious Diseases in Patients with Acute Hepatic Porphyria |
| title_short | Practical Recommendations in the Treatment of Acute and Chronic Life-Threatening Infectious Diseases in Patients with Acute Hepatic Porphyria |
| title_sort | practical recommendations in the treatment of acute and chronic life threatening infectious diseases in patients with acute hepatic porphyria |
| topic | acute hepatic porphyria infectious disease medicine anti-infective agents porphyrins acute intermittent porphyria |
| url | https://www.mdpi.com/2218-1989/15/2/99 |
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