ArticleClinical journal of gastroenterology2017
Cimetidine/lactulose therapy ameliorates erythropoietic protoporphyria-related liver injury.
Article in Clinical journal of gastroenterology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 7 papers.
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Who cites it
7 citing papers in PubMed, 14 citations in OpenAlex.
- Porphyrias: Pathophysiology and clinical management recommendations for hepatologists.Hepatology communications · 2025Review
- Of Zebrafish, Mice, and Men: Identifying New Biology and Developing New Treatments for Erythropoietic Protoporphyria.Cellular and molecular gastroenterology and hepatology · 2025Article
- Evidence-based consensus guidelines for the diagnosis and management of erythropoietic protoporphyria and X-linked protoporphyria.Journal of the American Academy of Dermatology · 2023Article
- Article
- Role of phlebotomy in the treatment of liver damage related to erythropoietic porphyria.Scientific reports · 2022Article
- Recognized and Emerging Features of Erythropoietic and X-Linked Protoporphyria.Diagnostics (Basel, Switzerland) · 2022Review
- Erythropoietic Protoporphyria-related Hepatopathy Successfully Treated with Phlebotomy.Internal medicine (Tokyo, Japan) · 2018Article
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Authors and funding
16 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
A 21-year-old Japanese man was admitted to our hospital because of severe abdominal pain and jaundice. He had been suffering from abdominal pain attacks and liver dysfunction since 18 years of age. Liver histology showed amorphous brown deposits in the sinusoidal space and significant periportal fibrosis without apparent hepatitis. Increased protoporphyrin in serum and feces and ferrochelatase gene mutation confirmed the final diagnosis of erythropoietic protoporphyria (EPP). Since ursodeoxycholic acid (UDCA) intake and glucose infusion are insufficient to ameliorate jaundice and abdominal attacks, cimetidine and lactulose were added in order to suppress hepatic delta-aminolevulinic acid synthase and limit re-absorption of protoporphyrin, respectively. Afterwards, the jaundice, liver dysfunction and abdominal symptoms improved and UDCA, cimetidine, and lactulose administration was continued. A repeat biopsy at 1.5 years after adding cimetidine/lactulose revealed marked attenuation of periportal fibrosis and protoporphyrin deposits. As far as we know, this is the first demonstration of histological improvement of EPP-induced liver abnormalities due to persistent cimetidine/lactulose administration. These treatments may be useful for EPP-related liver injury.
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