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Porphyrias

A diverse group of metabolic diseases characterized by errors in the biosynthetic pathway of HEME in the LIVER, the BONE MARROW, or both. They are classified by the deficiency of specific enzymes, the tissue site of enzyme defect, or the clinical features that include neurological (acute) or cutaneous (skin lesions). Porphyrias can be hereditary or acquired as a result of toxicity to the hepatic or erythropoietic marrow tissues.
Also Known As:
Porphyria
Networked: 1291 relevant articles (26 outcomes, 81 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Hepatic Porphyrias (Hepatic Porphyria)
2. Porphyria Cutanea Tarda
3. Erythropoietic Protoporphyria
4. Pemphigus (Pemphigus Vulgaris)
5. Skin Diseases

Experts

1. Bonkovsky, Herbert L: 8 articles (09/2015 - 11/2004)
2. Frank, Jorge: 8 articles (01/2015 - 06/2004)
3. San Martín de Viale, Leonor C: 8 articles (02/2013 - 07/2003)
4. Deybach, Jean-Charles: 7 articles (09/2015 - 01/2002)
5. Herrero, C: 7 articles (01/2013 - 07/2000)
6. Frank, J: 7 articles (01/2010 - 11/2001)
7. Sassa, Shigeru: 7 articles (04/2008 - 02/2002)
8. Sassa, S: 7 articles (01/2004 - 01/2000)
9. Puy, Hervé: 6 articles (09/2015 - 01/2002)
10. Phillips, John D: 6 articles (12/2014 - 01/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Porphyrias:
1. Hexachlorobenzene (HCB)IBA
2. Glucose (Dextrose)FDA LinkGeneric
3. PorphyrinsIBA
4. Chloroquine (Aralen)FDA LinkGeneric
5. Heme (Haem)IBA
6. Cytochrome P-450 Enzyme System (Cytochrome P450)IBA
7. Hemin (Panhematin)FDA Link
8. MuscimolIBA
9. Glycerol (Glycerine)FDA LinkGeneric
10. OxidoreductasesIBA

Therapies and Procedures

1. Photochemotherapy (Photodynamic Therapy)
2. Splenectomy
3. Aftercare (After-Treatment)
4. Intravenous Injections
5. Laparotomy