ArticleJournal of internal medicine2022
Risk of primary liver cancer in acute hepatic porphyria patients: A matched cohort study of 1244 individuals.
Article in Journal of internal medicine, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers, 1 of them a synthesis that pooled it.
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Who cites it
28 citing papers in PubMed, 1 synthesis or guideline pooled it, 57 citations in OpenAlex.
- AGA Clinical Practice Update on Diagnosis and Management of Acute Hepatic Porphyrias: Expert Review.Gastroenterology · 2023Guideline
- Porphyrias: Pathophysiology and clinical management recommendations for hepatologists.Hepatology communications · 2025Review
- Epidemiological characteristics and natural history of porphyria - a twenty-year population-based analysis in Taiwan.Orphanet journal of rare diseases · 2025Article
- A Prospective, Blinded Study of Symptom Prevalence and Specificity of Porphyrin Precursors in Carriers of Acute Hepatic Porphyria.Liver international : official journal of the International Association for the Study of the Liver · 2025Article
- Orbital fibroblastic reticular cell tumor: A case report and literature review for a rare disease.Medicine · 2025Review
- Hepatocellular Carcinoma in Acute Porphyria: Incidence and Risk Factors.Digestive diseases and sciences · 2025Article
- Hepatocellular Carcinoma in Acute Porphyria: Incidence and Risk Factors.Digestive diseases and sciences · 2025Article
- Liver transplantation and primary liver cancer in porphyria.Liver international : official journal of the International Association for the Study of the Liver · 2025Review
- Acute hepatic porphyria in Denmark; a retrospective study.Orphanet journal of rare diseases · 2025Article
- Successful Pregnancy After Combined Liver and Renal Transplantation in a Patient With Acute Intermittent Porphyria.Case reports in transplantation · 2025Article
- Reducing diagnostic delays in acute hepatic porphyria using health records data and machine learning.Journal of the American Medical Informatics Association : JAMIA · 2025Article
- Givosiran: a targeted treatment for acute intermittent porphyria.Hematology. American Society of Hematology. Education Program · 2024Review
- Review
- Heme (dys)homeostasis and liver disease.Frontiers in physiology · 2024Review
- Causal effect of porphyria biomarkers on alcohol-related hepatocellular carcinoma through Mendelian Randomization.PloS one · 2024Article
- Potential Biomarkers for the Earlier Diagnosis of Kidney and Liver Damage in 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
- Heterogeneous molecular behavior in liver tumors (HCC and CCA) of two patients with acute intermittent porphyria.Journal of cancer research and clinical oncology · 2023Review
- BALB.NCT-Molecular genetics and metabolism reports · 2023Article
- Intrahepatic Cholangiocarcinoma and Acute Intermittent Porphyria: A Case Report.Journal of clinical medicine · 2023Article
Corrections and comments
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Authors and funding
9 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundThe acute hepatic porphyrias (AHP) are associated with a risk of primary liver cancer (PLC), but risk estimates are unclear, and what AHP characteristics that predict PLC risk are unknown. In this register-based, matched cohort study, we assessed the PLC risk in relation to biochemical and clinical porphyria severity, genotype, age, and sex.
methodsAll patients in the Swedish porphyria register with acute intermittent porphyria (AIP), variegate porphyria (VP), or hereditary coproporphyria (HCP) during 1987-2015 were included. This AHP cohort was compared with age-, sex-, and county-matched reference individuals from the general population. National register-based hospital admissions for AHP were used to indicate the clinical severity. For AIP, the most common AHP type, patients were stratified by genotype and urinary porphobilinogen (U-PBG). Incident PLC data were collected from national health registers.
resultsWe identified 1244 individuals with AHP (1063 [85%] AIP). During a median follow-up of 19.5 years, we identified 108 incident PLC cases, including 83 AHP patients (6.7%) and 25 of 12,333 reference individuals (0.2%). The adjusted hazard ratio for AHP-PLC was 38.0 (95% confidence interval: 24.3-59.3). Previously elevated U-PBG and hospitalizations for porphyria, but not AIP genotype or sex, were associated with increased PLC risk. Patients aged >50 years with previously elevated U-PBG (n = 157) had an annual PLC incidence of 1.8%.
conclusionThis study confirmed a high PLC risk and identified a strong association with clinical and biochemical AIP activity. Regular PLC surveillance is motivated in patients older than 50 years with a history of active AIP.
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