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Encyclopedia > Acute intermittent porphyria
Acute Intermittent Porphyria
ICD-10 E80.2
ICD-9 277.1

Acute intermittent porphyria (AIP) is a rare metabolic disorder that is characterized by a deficiency of the enzyme, porphobilinogen deaminase (PBG-D), also known as uroporphyrinogen I-synthase. This enzyme deficiency results in the accumulation of porphyrins or porphyrin. Additional factors must also be present such as hormones, drugs and dietary changes that trigger the appearance of symptoms. Symptoms of AIP may include abdominal pain, constipation, and muscle weakness. The following codes are used with International Statistical Classification of Diseases and Related Health Problems. ... The following is a list of codes for International Statistical Classification of Diseases and Related Health Problems. ... Santorio Santorio (1561-1636) in his steelyard balance, from Ars de statica medecina, first published 1614 Metabolism (from μεταβολισμος (metabolismos)) is the biochemical modification of chemical compounds in living organisms anggjgjhnd cell (b). ... Ribbon diagram of the enzyme TIM. TIM is catalytically perfect, meaning its conversion rate is limited, or nearly limited to its substrate diffusion rate. ... Porphobilinogen deaminase (or hydroxymethylbilane synthase) is an enzyme involved in the third step of the metabolism of porphyrin, converting porphobilinogen into hydroxymethyl bilane. ... A porphyrin is a heterocyclic macrocycle made from 3 pyrrole subunits and one pyrroline subunit, and linked on opposite sides through 4 methine bridges. ... A hormone (from Greek horman - to set in motion) is a chemical messenger from one cell (or group of cells) to another. ... Oral medication A medication is a licenced drug taken to cure or reduce symptoms of an illness or medical condition. ... In nutrition, the diet is the sum of the food consumed by an organism. ... Abdominal pain can be one of the symptoms associated with transient disorders or serious disease. ... Constipation or irregularity, is a condition of the digestive system where a person (or animal) experiences hard feces that are difficult to eliminate; it may be extremely painful, and in severe cases (fecal impaction) lead to symptoms of bowel obstruction. ... A top-down view of skeletal muscle Muscle is contractile tissue of the body and is derived from the mesodermal layer of embryonic germ cells. ...


A high-carbohydrate diet is typically recommended; in severe attacks, a glucose 10% infusion is recommenced, which may aid in recovery. If drugs have caused the attack, discontinuing the offending substances is essential. Infection is one of the top causes of attacks and requires vigorous treatment. Pain is extremely severe and almost always requires the use of opiates to reduce it to tolerable levels. Pain should be treated early as medically possible due to its severity. Nausea can be severe; it may respond to phenothiazine drugs but is sometimes intractable. Hot water baths/showers may lessen nausea temporarily, though caution should be used to avoid burns or falls. Carbohydrates are chemical compounds that contain oxygen, hydrogen, and carbon atoms. ... Glucose (Glc), a monosaccharide (or simple sugar), is one of the most important carbohydrates. ... An infection is the detrimental colonization of a host organism by a foreign species. ... Look up Pain in Wiktionary, the free dictionary. ... Phenothiazines are the largest of the 5 main classes of antipsychotic drugs. ...


Hematin and haem arginate are the drugs of choice in acute porphyria, in the United States and the United Kingdom, respectively. These drugs need to be given very early in an attack to be effective. Effectiveness varies amongst individuals. They are not curative drugs but can shorten attacks and reduce the intensity of an attack. Side effects are rare but can be serious. These heme-like substances theoretically inhibit ALA synthase and hence the accumulation of toxic precursors. In the United Kingdom, supplies of this drug are maintained at two national centers. In the United States, one company manufactures Panhematin for infusion. The American Porphyria Foundation has information regarding the quick procurement of the drug.


Patients with a history of acute porphyria are recommended to wear an alert bracelet or other identification at all times in case they develop severe symptoms: a result of which may be they cannot explain to healthcare professionals about their condition and the fact that some drugs are absolutely contraindicated. Medical ID jewelry provides valuable information about personal medical conditions in time of emergency. ...


Patients who experience frequent attacks can develop chronic neuropathic pain in extremities as well as chronic pain in the gut. This is thought to be due to axonal nerve deterioration in affected areas of the nervous system. In these cases treatment with long-acting opioids may be indicated. Some cases of chronic pain can be difficult to manage and may require treatment using multiple modalities. Depression often accompanies the disease and is best dealt with by treating the offending symptoms and if needed the judicious use of anti-depressants.


Seizures often accompany this disease. Most seizure medications exacerbate this condition. Treatment can be problematic: barbiturates must be avoided. Some benzodiazepines are safe, and, when used in conjuction with newer anti-seizure medications such as gabapentin offer a possible regime for seizure control. This article is about the medical condition. ... Barbiturates are drugs that acts as central nervous system (CNS) depressants, and by virtue of this they produce a wide spectrum of effects, from mild sedation to anesthesia. ... Benzodiazepine tablets The benzodiazepines are a class of drugs with hypnotic, anxiolytic, anticonvulsant, amnestic and muscle relaxant properties. ... Gabapentin (brand name: Neurontin®) was initially synthesized to mimic the structure of GABA for the treatment of epilepsy. ...


Urine from a person experiencing an acute attack will be red or "port wine" in color because of the presence of porphyrins. This condition is known as porphyrinuria. To meet Wikipedias quality standards, this article or section may require cleanup. ...


On the television show House, Dr. House diagnoses his ex-girlfriend's new husband with AIP. House, also titled House, M.D., is an American television series. ... Dr. Gregory House Gregory House, MD is a fictional character portrayed by Hugh Laurie, is the protagonist of the television drama House. ...


See also

Porphyria This article is about the disease. ...


  Results from FactBites:
 
Porphyria - Encyclopedia, History, Geography and Biography (3481 words)
Acute attacks of porphyrias in patients with autoimmune diseases such as lupus have been initially labeled as "lupus complications" delaying diagnosis of the underlying porphyria and treatment resulting in deaths.
In nearly all cases of acute porphyria syndromes, urinary PBG is markedly elevated except for the very rare ALA dehydratase deficiency or in patients with symptoms due to lead poisoning or hereditary tyrosinemia type I.
Porphyria has been suggested as an explanation for the origin of vampire and werewolf legends, based upon a number of similarities between the condition and the folklore that was first speculated upon by biochemist David Dolphin in 1985.
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