What is hereditary Coproporphyria?
Hereditary coproporphyria (HCP) is a rare metabolic disorder characterized by deficiency of the enzyme coproporphyrinogen oxidase. This enzyme deficiency results in the accumulation of porphyrin precursors in the body. This enzyme deficiency is caused by a mutation in the CPOX gene.
What is congenital HCP?
Hereditary coproporphyria (HCP) is a rare inherited form of liver (hepatic) porphyria, characterized by neurological symptoms in the form of episodes (acute attacks) of stomach pain, nausea, vomiting, weakness, numbness, and pain in the hands and feet (neuropathy).
Can porphyria affect pregnancy?
Studies have compared the risk of pregnancy in women with porphyria to those without the disease. The results of these studies indicate that women with porphyria have a higher risk of preeclampsia (dangerously high blood pressure during pregnancy) and a higher incidence of delivery by cesarean section.
What is AIP in pregnancy?
Acute intermittent porphyria (AIP) is a rare inherited disorder of heme metabolism. It has the ability to trigger posterior reversible encephalopathy syndrome (PRES), a rare acute neurologic condition that is characterized by acute neurological symptoms. Pregnancy may induce AIP attacks.
What does the medical term HCP mean?
Hereditary coproporphyria (HCP) is a disorder of heme biosynthesis, classified as an acute hepatic porphyria. HCP is caused by a deficiency of the enzyme coproporphyrinogen oxidase, coded for by the CPOX gene, and is inherited in an autosomal dominant fashion, although homozygous individuals have been identified.
What causes high coproporphyrin?
What does it mean if your Coproporphyrin I result is too high? Porphyrins appear elevated in urine when the cellular pathway for heme synthesis is blocked by natural or man-made toxicants or when genetic disorders that affect the enzymes of the porphyrin pathway are present.
What is HCP in medicine?
What is AIP disease?
Summary. Acute intermittent porphyria (AIP) is a rare metabolic disorder that is characterized by partial deficiency of the enzyme hydroxymethylbilane synthase (also known as porphobilinogen deaminase). This enzyme deficiency can result in the accumulation of porphyrin precursors in the body.
What are the symptoms of AIP?
Acute intermittent porphyria (AIP) can cause many different symptoms during an attack:
- Psychological symptoms such as feeling anxious, confused, or depressed.
- Muscle weakness.
- Fast heart rate.
- Pain in the arms, legs, chest, neck, or head.
- High blood pressure.
- Nausea or vomiting.
- Pain in the abdomen.
- Constipation.
What medications should be avoided with porphyria?
Which medications should be avoided by patients with porphyria?
- Barbiturates.
- Anticonvulsants.
- Progestins.
- Rifampin.
What is HCP short for?
HCP
| Acronym | Definition |
|---|---|
| HCP | Health Care Professional |
| HCP | Human Connectome Project (brain research) |
| HCP | Hearing Conservation Program |
| HCP | Hitachi Content Platform (object storage system) |
Are there any medications that are safe to take during pregnancy?
COMMON MEDICATIONS SAFE IN PREGNANCY. Clindamycin. ACNE: Benzoyl Peroxide Topical Erythromycin. Salicylic Acid. AVOID. : Accutane Metamucil. Retin-A. Tetracycline. Minocycline.
How to diagnose coproporphyria ( HCP ) in children?
Diagnosis. A diagnosis of HCP is based upon identification of characteristic symptoms, a detailed patient and family history, a thorough clinical evaluation and a variety of specialized tests. The observation of reddish brown urine that is free of blood is indicative, but not conclusive, of an acute porphyria.
Is it safe to take diphenhydramine during pregnancy?
Diphenhydramine is widely used in pregnancy as a sedative, an antihistamine, and an anti-nausea drug, although few data confirm its safety during pregnancy.
Which is the best test for hereditary coproporphyria?
The initial test for people with symptoms is quantitative urinary porphobilinogen (PBG), aminolevulinic acid (ALA), and porphyrins. Elevation of PBG, ALA and coproporphyrin (predominantly isomer III) is highly suggestive of Hereditary Coproporphyria.