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: | , , |
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| Format: | Article |
| Language: | English |
| Published: |
Bucharest College of Physicians
2023-03-01
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| Series: | Modern Medicine |
| Subjects: | |
| Online Access: | https://medicinamoderna.ro/wp-content/uploads/2023/03/Acute-Hepatic-Porphyria-%E2%80%93-Minireview.pdf |
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| Summary: | 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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| ISSN: | 1223-0472 2360-2473 |