ReviewWorld journal of gastroenterology2010
Liver disease and erythropoietic protoporphyria: a concise review.
Review in World journal of gastroenterology, 2010. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 39 papers, 1 of them a synthesis that pooled it.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
39 citing papers in PubMed, 1 synthesis or guideline pooled it, 96 citations in OpenAlex.
- NR1I2 genetic polymorphisms and the risk of anti-tuberculosis drug-induced hepatotoxicity: A systematic review and meta-analysis.Pharmacology research & perspectives · 2020Pooled it
- A brief history of the human liver peroxisome.Histochemistry and cell biology · 2026Review
- Animal Models of Porphyria with Hepatic Involvement.Seminars in liver disease · 2025Review
- Acute Protoporphyric Hepatopathy as the Initial Presentation of Erythropoietic Protoporphyria in Adulthood.ACG case reports journal · 2025Article
- Validation of the sunlight exposure diary and the erythropoietic protoporphyria impact questionnaire (EPIQ).Orphanet journal of rare diseases · 2025Article
- Jaundice in an Adult Female Patient With Photosensitivity.ACG case reports journal · 2025Article
- Self-Reported Liver Disease and the Burden of Erythropoietic Protoporphyria.JIMD reports · 2025Article
- Scavenger endothelial cells alleviate tissue damage by engulfing toxic molecules derived from hemolysis.Proceedings of the National Academy of Sciences of the United States of America · 2025Article
- Oxidative balance scores and gallstone disease: mediating effects of oxidative stress.Nutrition journal · 2025Article
- Erythropoietic protoporphyrias: Pathogenesis, diagnosis and management.Liver international : official journal of the International Association for the Study of the Liver · 2025Review
- Target-centric analysis of hepatitis B: identifying key molecules and pathways for treatment.Scientific reports · 2024Article
- Peptide-Oligonucleotide Conjugation: Chemistry and Therapeutic Applications.Current issues in molecular biology · 2024Review
- Successful treatment of severe hepatic impairment in erythropoietic protoporphyria: A case report and review of literature.World journal of hepatology · 2024Article
- Unraveling the mechanisms underlying drug-induced cholestatic liver injury: identifying key genes using machine learning techniques on human in vitro data sets.Archives of toxicology · 2023Article
- Protoporphyrin IX-induced phototoxicity: Mechanisms and therapeutics.Pharmacology & therapeutics · 2023Review
- Afamelanotide Is Associated with Dose-Dependent Protective Effect from Liver Damage Related to Erythropoietic Protoporphyria.Life (Basel, Switzerland) · 2023Article
- Article
- An Enigmatic Case of Jaundice and Photosensitivity in an Adolescent.ACG case reports journal · 2023Article
- Diagnosis and treatment of icteric hepatitis caused by erythropoietic protoporphyria: A case report.Liver research (Beijing, China) · 2022Article
- Severe aplastic anemia in a patient with erythropoietic protoporphyria successfully treated by avatrombopag.Annals of hematology · 2022Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
4 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The porphyries are a group of metabolic disorders characterized by deficiencies in the activity of enzymes involved in the biosynthesis of heme. In erythropoietic protoporphyria (EPP), in the majority of cases an autosomal dominant disease, there is a mutation of the gene that encodes ferrochelatase (FECH). FECH deficiency is associated with increased concentrations of protoporphyrin in erythrocytes, plasma, skin and liver. The prevalence of this inherited disorder oscillates between 1:75 000 and 1:200 000. Clinical manifestations of EPP appear in early infancy upon first exposure to the sun. Nevertheless, approximately 5%-20% of patients with EPP develop liver manifestations. Retention of protoporphyrin in the liver is associated with cholestatic phenomena and oxidative stress that predisposes to hepatobiliary disease of varying degrees of severity, such as cholelithiasis, mild parenchymal liver disease, progressive hepatocellular disease with end-stage liver disease and acute liver failure. Liver damage is the major risk in EPP patients, so surveillance and frequent clinical and biochemical liver follow-up is mandatory. The diagnostic approach consists in detecting increased levels of protoporphyrin, decreased activity of FECH and genetic analysis of the FECH gene. A variety of non-surgical therapeutic approaches have been adopted for the management of EPP associated with liver disease, but none of these has been shown to be unequivocally efficacious. Nevertheless, some may have a place in preparing patients for liver transplantation. Liver transplantation does not correct the constitutional deficiency of FECH. Consequently, there is a risk of recurrence of liver disease after liver transplantation as a result of continuing overproduction of protoporphyrin. Some authors recommend that bone marrow transplantation should be considered in liver allograft recipients to prevent recurrence of hepatic disease.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.