The University of Chicago Header Logo

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.

expand / collapse MeSH information
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.

expand / collapse publications
This graph shows the total number of publications written about "Porphyria, Hepatoerythropoietic" by people in this website by year, and whether "Porphyria, Hepatoerythropoietic" was a major or minor topic of these publications.
To see the data from this visualization as text, click here.