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Acute Intermittent Porphyria (Porphyria, Acute) Summary

Description: An autosomal dominant porphyria that is due to a deficiency of HYDROXYMETHYLBILANE SYNTHASE in the LIVER, the third enzyme in the 8-enzyme biosynthetic pathway of HEME. Clinical features are recurrent and life-threatening neurologic disturbances, ABDOMINAL PAIN, and elevated level of AMINOLEVULINIC ACID and PORPHOBILINOGEN in the urine.

Also Known As: Porphyria, Acute; Intermittent Porphyria, Acute; Acute Porphyria; Porphyria, Acute Intermittent; Acute Intermittent Porphyrias Show All >>

Networked: 840 relevant articles (30 outcomes, 56 trials/studies) for this Disease

Key Drugs and Agents for Acute Intermittent Porphyria

Efficacy Chart >>
Drugs and Important Biological Agents (IBA) related to treatments:
  1. Hemin (Panhematin) : 6 outcomes 2 studies in 32 results : FDA 1
  2. Heme (Haem) : 3 outcomes 5 studies in 205 results : IBA
  3. Porphobilinogen : 3 outcomes 4 studies in 80 results : IBA
  4. Glucose (Dextrose) : 2 outcomes 1 study in 15 results : FDA 175 Generic
  5. heme arginate : 2 outcomes in 15 results : IBA
  6. Cimetidine (Biomet) : 2 outcomes in 8 results : FDA 57 Generic
  7. Hydroxymethylbilane Synthase (Porphobilinogen Deaminase) : 1 outcome 14 studies in 211 results : IBA
  8. Erythropoietin : 1 outcome 2 studies in 3 results : FDA 1
  9. Enalapril : 1 outcome 2 studies in 2 results : FDA 28 Generic
  10. Hormones : 1 outcome 1 study in 33 results : IBA
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Diseases Related to Acute Intermittent Porphyria

  1. Hereditary Coproporphyria (Coproporphyrinogen Oxidase Deficiency)
  2. Erythropoietic Protoporphyria
  3. Hypertension (High Blood Pressure)
  4. Hepatic Porphyrias (Hepatic Porphyria)
  5. Renal Insufficiency (Renal Failure)
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Key Therapies for Acute Intermittent Porphyria

Efficacy Chart >>
  1. Liver Transplantation : 1 outcome 1 study in 9 results
  2. Bone Marrow Transplantation (Transplantation, Bone Marrow) : 1 outcome 1 study in 1 result
  3. Transplantation (Transplant Recipients) : 1 outcome in 3 results
  4. Enzyme Replacement Therapy : 1 outcome in 1 result
  5. Renal Dialysis (Hemodialysis) : 1 study in 7 results
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