ReviewFrontiers in neurology2022
Pain in acute hepatic porphyrias: Updates on pathophysiology and management.
Review in Frontiers in neurology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 17 citations in OpenAlex.
- The Case of a 37-Year-Old Woman Presenting With Subacute Weakness and Paresthesias.Annals of clinical and translational neurology · 2026Article
- Noncanonical role of ALAS1 as a heme-independent inhibitor of small RNA-mediated silencing.Science (New York, N.Y.) · 2024Article
- Neuralgic amyotrophy presentation of acute intermittent porphyria: A case report.Journal of the peripheral nervous system : JPNS · 2024Article
- Hereditary Coproporphyria in Which the Patient's Course Improved after the Discontinuation of Givosiran.Internal medicine (Tokyo, Japan) · 2024Article
- Acute Intermittent Porphyria in an Adolescent Patient: Diagnostic and Treatment Challenges.Cureus · 2024Article
- Xenobiotics Triggering Acute Intermittent Porphyria and Their Effect on Mouse Brain Respiratory Complexes.Journal of xenobiotics · 2024Article
- Obstacles to Early Diagnosis of Acute Hepatic Porphyria: Current Perspectives on Improving Early Diagnosis and Clinical Management.Clinical and experimental gastroenterology · 2024Review
- Article
- Autocrine positive feedback of tumor necrosis factor from activated microglia proposed to be of widespread relevance in chronic neurological disease.Pharmacology research & perspectives · 2023Review
- Mitochondrial DNA Copy Number Drives the Penetrance of Acute Intermittent Porphyria.Life (Basel, Switzerland) · 2023Article
- First Report of a Low-Frequency Mosaic Mutation in the Hydroxymethylbilane Synthase Gene Causing Acute Intermittent Porphyria.Life (Basel, Switzerland) · 2023Article
- Article
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Authors and funding
3 authors at 3 institutions in 2 countries.
Funding
Abstract
Acute hepatic porphyrias (AHPs) typically present with recurrent acute attacks of severe abdominal pain and acute autonomic dysfunction. While chronic symptoms were historically overlooked in the literature, recent studies have reported increased prevalence of chronic, mainly neuropathic, pain between the attacks. Here we characterize acute and chronic pain as prominent manifestations of the AHPs and discuss their pathophysiology and updated management. In addition to the severe abdominal pain, patients could experience low back pain, limb pain, and headache during acute attacks. Chronic pain between the attacks is typically neuropathic and reported mainly by patients who undergo recurrent attacks. While the acute abdominal pain during attacks is likely mediated by autonomic neuropathy, chronic pain likely represents delayed recovery of the acute neuropathy with ongoing small fiber neuropathy in addition to peripheral and/or central sensitization. δ-aminolaevulinic acid (ALA) plays a major role in acute and chronic pain
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