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Hepatic Porphyrias (Hepatic Porphyria)

A group of metabolic diseases due to deficiency of one of a number of LIVER enzymes in the biosynthetic pathway of HEME. They are characterized by the accumulation and increased excretion of PORPHYRINS or its precursors. Clinical features include neurological symptoms (PORPHYRIA, ACUTE INTERMITTENT), cutaneous lesions due to photosensitivity (PORPHYRIA CUTANEA TARDA), or both (HEREDITARY COPROPORPHYRIA). Hepatic porphyrias can be hereditary or acquired as a result of toxicity to the hepatic tissues.
Also Known As:
Hepatic Porphyria; Porphyria, Hepatic; Porphyrias, Hepatic
Networked: 333 relevant articles (6 outcomes, 24 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Porphyrias (Porphyria)
2. Hepatic Porphyrias (Hepatic Porphyria)
3. Erythropoietic Protoporphyria
4. Renal Insufficiency (Renal Failure)
5. Variegate Porphyria (Protoporphyrinogen Oxidase Deficiency)

Experts

1. Bonkovsky, Herbert L: 10 articles (01/2022 - 01/2005)
2. Desnick, Robert J: 10 articles (11/2019 - 01/2010)
3. Yasuda, Makiko: 6 articles (11/2019 - 01/2010)
4. Ricci, Andrea: 4 articles (05/2022 - 12/2021)
5. Ventura, Paolo: 4 articles (05/2022 - 12/2021)
6. Rudnick, Sean: 4 articles (01/2022 - 02/2019)
7. Wang, Bruce: 4 articles (01/2021 - 10/2017)
8. Chen, Brenden: 4 articles (11/2019 - 05/2014)
9. Phillips, John D: 4 articles (11/2019 - 05/2014)
10. Puy, Hervé: 4 articles (11/2019 - 01/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hepatic Porphyrias:
1. PorphyrinsIBA
2. Heme (Haem)IBA
3. Chloroquine (Aralen)FDA LinkGeneric
4. zinc mesoporphyrinIBA
5. Hemin (Panhematin)FDA Link
6. Heme Oxygenase (Decyclizing) (Heme Oxygenase)IBA
7. kryptopyrroleIBA
8. tin protoporphyrin IXIBA
9. Hexachlorobenzene (HCB)IBA
10. Griseofulvin (Grifulvin V)FDA LinkGeneric

Therapies and Procedures

1. Therapeutics
2. Splenectomy
3. Erythrocyte Transfusion
4. Liver Transplantation
5. Anesthesia