Acute Hepatic Porphyria – Minireview
| Autor: | Aida SAVU, Andrei EDU, Lucian NEGREANU |
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| Jazyk: | angličtina |
| Rok vydání: | 2023 |
| Předmět: | |
| Zdroj: | Modern Medicine, Vol 30, Iss 1, Pp 13-16 (2023) |
| Druh dokumentu: | article |
| ISSN: | 1223-0472 2360-2473 |
| DOI: | 10.31689/rmm.2023.30.1.13 |
| Popis: | 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. |
| Databáze: | Directory of Open Access Journals |
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