Porphyria, Hepatoerythropoietic
"Porphyria, Hepatoerythropoietic" is a descriptor in the National Library of Medicine's controlled vocabulary thesaurus,
MeSH (Medical Subject Headings). Descriptors are arranged in a hierarchical structure,
which enables searching at various levels of specificity.
An autosomal recessive cutaneous porphyria that is due to a deficiency of UROPORPHYRINOGEN DECARBOXYLASE in both the LIVER and the BONE MARROW. Similar to PORPHYRIA CUTANEA TARDA, this disorder is caused by defects in the fifth enzyme in the 8-enzyme biosynthetic pathway of HEME, but is a homozygous enzyme deficiency with less than 10% of the normal enzyme activity. Cutaneous lesions are severe and mutilating.
MeSH Number(s)
C06.552.830.437
C16.320.565.708.400.437
C16.320.850.742.437
C17.800.827.742.437
C17.800.849.617.400.437
C18.452.648.708.400.437
C18.452.811.400.437
C18.452.880.617.400.437
Concept/Terms
Porphyria, Hepatoerythropoietic- Porphyria, Hepatoerythropoietic
- Hepatoerythropoietic Porphyrias
- Porphyrias, Hepatoerythropoietic
- Hepatoerythropoietic Porphyria
- Porphyria, Erythrohepatic
- Erythrohepatic Porphyria
- Erythrohepatic Porphyrias
- Porphyrias, Erythrohepatic
Below are MeSH descriptors whose meaning is more general than "Porphyria, Hepatoerythropoietic".
- Diseases [C]
- Digestive System Diseases [C06]
- Liver Diseases [C06.552]
- Porphyrias, Hepatic [C06.552.830]
- Porphyria, Hepatoerythropoietic [C06.552.830.437]
- Congenital, Hereditary, and Neonatal Diseases and Abnormalities [C16]
- Genetic Diseases, Inborn [C16.320]
- Metabolism, Inborn Errors [C16.320.565]
- Porphyrias [C16.320.565.708]
- Porphyrias, Hepatic [C16.320.565.708.400]
- Porphyria, Hepatoerythropoietic [C16.320.565.708.400.437]
- Skin Diseases, Genetic [C16.320.850]
- Porphyrias, Hepatic [C16.320.850.742]
- Porphyria, Hepatoerythropoietic [C16.320.850.742.437]
- Skin and Connective Tissue Diseases [C17]
- Skin Diseases [C17.800]
- Skin Diseases, Genetic [C17.800.827]
- Porphyrias, Hepatic [C17.800.827.742]
- Porphyria, Hepatoerythropoietic [C17.800.827.742.437]
- Skin Diseases, Metabolic [C17.800.849]
- Porphyrias [C17.800.849.617]
- Porphyrias, Hepatic [C17.800.849.617.400]
- Porphyria, Hepatoerythropoietic [C17.800.849.617.400.437]
- Nutritional and Metabolic Diseases [C18]
- Metabolic Diseases [C18.452]
- Metabolism, Inborn Errors [C18.452.648]
- Porphyrias [C18.452.648.708]
- Porphyrias, Hepatic [C18.452.648.708.400]
- Porphyria, Hepatoerythropoietic [C18.452.648.708.400.437]
- Porphyrias [C18.452.811]
- Porphyrias, Hepatic [C18.452.811.400]
- Porphyria, Hepatoerythropoietic [C18.452.811.400.437]
- Skin Diseases, Metabolic [C18.452.880]
- Porphyrias [C18.452.880.617]
- Porphyrias, Hepatic [C18.452.880.617.400]
- Porphyria, Hepatoerythropoietic [C18.452.880.617.400.437]
Below are MeSH descriptors whose meaning is more specific than "Porphyria, Hepatoerythropoietic".
This graph shows the total number of publications written about "Porphyria, Hepatoerythropoietic" by people in Harvard Catalyst Profiles by year, and whether "Porphyria, Hepatoerythropoietic" was a major or minor topic of these publication.
To see the data from this visualization as text,
click here.
Year | Major Topic | Minor Topic | Total |
---|
1994 | 1 | 0 | 1 |
1998 | 1 | 0 | 1 |
1999 | 3 | 0 | 3 |
2000 | 1 | 0 | 1 |
2002 | 2 | 0 | 2 |
Below are the most recent publications written about "Porphyria, Hepatoerythropoietic" by people in Profiles.
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Long-term treatment of erythropoietic protoporphyria with cysteine. Photodermatol Photoimmunol Photomed. 2002 Dec; 18(6):307-9.
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Treatment of recurrent allograft dysfunction with intravenous hematin after liver transplantation for erythropoietic protoporphyria. Transplantation. 2002 Mar 27; 73(6):911-5.
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Zebrafish dracula encodes ferrochelatase and its mutation provides a model for erythropoietic protoporphyria. Curr Biol. 2000 Aug 24; 10(16):1001-4.
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Erythropoietic protoporphyria: treatment with antioxidants and potential cure with gene therapy. Methods Enzymol. 2000; 319:479-84.
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Photosensitivity and perioperative polyneuropathy complicating orthotopic liver transplantation in a patient with erythropoietic protoporphyria. Anesthesiology. 1999 Oct; 91(4):1173-5.
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Long-term cure of the photosensitivity of murine erythropoietic protoporphyria by preselective gene therapy. Nat Med. 1999 Jul; 5(7):768-73.
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The value of intravenous heme-albumin and plasmapheresis in reducing postoperative complications of orthotopic liver transplantation for erythropoietic protoporphyria. Transplantation. 1999 Mar 27; 67(6):922-8.
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Neuropathy in erythropoietic protoporphyrias. Neurology. 1998 Jul; 51(1):262-5.
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The treatment of erythropoietic protoporphyria. Semin Liver Dis. 1998; 18(4):425-6.
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Burst-forming units-erythroid from erythropoietic protoporphyria patients fluoresce under 405 nm light. Blood. 1996 May 15; 87(10):4480-1.