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Hepatoerythropoietic Porphyria Summary

Description: 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.

Also Known As: Porphyria, Hepatoerythropoietic; Erythrohepatic Porphyria; Erythrohepatic Porphyrias; Hepatoerythropoietic Porphyrias; Porphyrias, Erythrohepatic Show All >>

Networked: 38 relevant articles (0 outcomes, 4 trials/studies) for this Disease

Key Drugs and Agents for Hepatoerythropoietic Porphyria

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Uroporphyrinogen Decarboxylase (Uroporphyrinogen III Decarboxylase) : 4 studies in 31 results : IBA
  2. Heme (Haem) : 8 results : IBA
  3. Porphyrins : 4 results : IBA
  4. Complementary DNA (cDNA) : 1 result : IBA
  5. Protoporphyrins : 1 result : IBA
  6. Hemoglobins (Hemoglobin) : 1 result : IBA
  7. Enzyme Precursors (Zymogens) : 1 result : IBA
  8. Porphobilinogen Synthase (delta-Aminolevulinate Dehydratase) : 1 result : IBA
  9. Adenosine Diphosphate (ADP) : 1 result : IBA
  10. Coproporphyria : 1 result : IBA
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Diseases Related to Hepatoerythropoietic Porphyria

  1. Porphyria Cutanea Tarda
  2. Porphyrias
  3. Erythropoietic Protoporphyria
  4. Variegate Porphyria (Protoporphyrinogen Oxidase Deficiency)
  5. Hereditary Coproporphyria (Coproporphyrinogen Oxidase Deficiency)
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Key Therapies for Hepatoerythropoietic Porphyria

Efficacy Chart >>

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