Acute Hepatic Porphyria – Minireview

Acute Hepatic Porphyria (AHP) is an uncommon and hereditary illness that belongs to a group of disorders known as porphyries. This condition results from a deficiency of the porphobilinogen deaminase enzyme, which plays a role in heme production, a crucial component of haemoglobin in the bloodstream...

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Main Authors: Aida SAVU, Andrei EDU, Lucian NEGREANU
Format: Article
Language:English
Published: Bucharest College of Physicians 2023-03-01
Series:Modern Medicine
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Online Access:https://medicinamoderna.ro/wp-content/uploads/2023/03/Acute-Hepatic-Porphyria-%E2%80%93-Minireview.pdf
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author Aida SAVU
Andrei EDU
Lucian NEGREANU
author_facet Aida SAVU
Andrei EDU
Lucian NEGREANU
author_sort Aida SAVU
collection DOAJ
description Acute Hepatic Porphyria (AHP) is an uncommon and hereditary illness that belongs to a group of disorders known as porphyries. This condition results from a deficiency of the porphobilinogen deaminase enzyme, which plays a role in heme production, a crucial component of haemoglobin in the bloodstream. This deficiency leads to the accumulation of substances called porphyrins in the body, which can trigger the appearance of severe and potentially life-threatening symptoms. In the following, we will discuss classifications - with a focus on the similarities and differences between subtypes of porphyria, the pathophysiology of acute hepatic porphyria, risk factors – and their influence on the onset of the disease, clinical manifestations, diagnosis, and management – both curative and symptomatic, all of which play a very important role in understanding this rare condition.
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spelling doaj-art-9b248da9900d4de7af0469c7a5e9666e2025-08-20T02:20:26ZengBucharest College of PhysiciansModern Medicine1223-04722360-24732023-03-013011316https://doi.org/10.31689/rmm.2023.30.1.13Acute Hepatic Porphyria – MinireviewAida SAVU0Andrei EDU1Lucian NEGREANU2https://orcid.org/0000-0003-3042-0754”Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania”Carol Davila” University of Medicine and Pharmacy, Bucharest, Romania”Carol Davila” University of Medicine and Pharmacy, Bucharest, RomaniaAcute Hepatic Porphyria (AHP) is an uncommon and hereditary illness that belongs to a group of disorders known as porphyries. This condition results from a deficiency of the porphobilinogen deaminase enzyme, which plays a role in heme production, a crucial component of haemoglobin in the bloodstream. This deficiency leads to the accumulation of substances called porphyrins in the body, which can trigger the appearance of severe and potentially life-threatening symptoms. In the following, we will discuss classifications - with a focus on the similarities and differences between subtypes of porphyria, the pathophysiology of acute hepatic porphyria, risk factors – and their influence on the onset of the disease, clinical manifestations, diagnosis, and management – both curative and symptomatic, all of which play a very important role in understanding this rare condition.https://medicinamoderna.ro/wp-content/uploads/2023/03/Acute-Hepatic-Porphyria-%E2%80%93-Minireview.pdfporphyriahaemoglobinacute hepatic porphyria (ahp)
spellingShingle Aida SAVU
Andrei EDU
Lucian NEGREANU
Acute Hepatic Porphyria – Minireview
Modern Medicine
porphyria
haemoglobin
acute hepatic porphyria (ahp)
title Acute Hepatic Porphyria – Minireview
title_full Acute Hepatic Porphyria – Minireview
title_fullStr Acute Hepatic Porphyria – Minireview
title_full_unstemmed Acute Hepatic Porphyria – Minireview
title_short Acute Hepatic Porphyria – Minireview
title_sort acute hepatic porphyria minireview
topic porphyria
haemoglobin
acute hepatic porphyria (ahp)
url https://medicinamoderna.ro/wp-content/uploads/2023/03/Acute-Hepatic-Porphyria-%E2%80%93-Minireview.pdf
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