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         Porphyrias:     more books (87)
  1. Porphyria cutanea tarda (PCT).(What's Your Assessment?): An article from: Dermatology Nursing by Barbara Bielan, 1996-06-01
  2. Porphyrias: An entry from Thomson Gale's <i>Gale Encyclopedia of Children's Health: Infancy through Adolescence</i> by Mark Best, Julia Barrett, et all 2006
  3. 21st Century Complete Medical Guide to Porphyria and Related Disorders: Authoritative Government Documents, Clinical References, and Practical Information for Patients and Physicians by PM Medical Health News, 2004-06
  4. Porphyrias: An entry from Thomson Gale's <i>Gale Encyclopedia of Genetic Disorders, 2nd ed.</i> by Julia Barrett, Judy, MS Hawkins, 2005
  5. Diseases:Porphyrias - Spasm (Volume 7 of ISBN 0717276171) by Bryan Bunch, 1997
  6. Porphyria
  7. The Porphyrias: a Story of Inheritance and Environment, 2nd Edition by Geoffrey Dean, 1971
  8. Diagnosis and Therapy of Porphyrias and Lead Intoxication: International Symposium Clinical Biochemistry
  9. A neuropsychiatric and genetical investigation of acute intermittent porphyria (Scandinavian university books) by Lennart Wetterberg, 1967
  10. Porphyria in Australia: A review of the literature, and Australian experience by Roderick McEwin, 1973
  11. The "insanity" of King George III: A classic case of porphyria by Ida Macalpine, 1966
  12. Contribution to the elucidation of the mechanism of hepatic porphyria induced by hexachlorobenzene and related polyhalogenated hydrocarbons by Felix M. H Debets, 1981
  13. Porphyria, a royal malady: Articles published 1966 & 1968! in or commisioned by the 'British Medical Journal'
  14. Skin Pharmacology and Applied Skin Physiology Physiology: Porphyria (Skin Pharmacology & Applied Skin Physiology)

61. Porphyrias | BluePrint For Health
You are here Home Health A to Z porphyrias. porphyrias. Barrett,Julia. This situation is the main characteristic of the porphyrias.
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Definition

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Causes and symptoms Diagnosis ... Resources Definition The porphyrias are disorders in which the body produces too much porphyrin and insufficient heme (an iron-containing nonprotein portion of the hemoglobin molecule). Porphyrin is a foundation structure for heme and certain enzymes. Excess porphyrins are excreted as waste in the urine and stool. Overproduction and overexcretion of porphyrins causes low, unhealthy levels of heme and certain important enzymes, creating various physical symptoms. Description Biosynthesis of heme is a multistep process that begins with simple molecules and ends with a large, complex heme molecule. Each step of the chemical pathway is directed by its own task-specific protein, called an enzyme. As a heme precursor molecule moves through each step, an enzyme modifies the precursor in some way. If a precursor molecule is not modified, it cannot proceed to the next step, causing a buildup of that specific precursor. This situation is the main characteristic of the porphyrias. Owing to a defect in one of the enzymes of the heme biosynthesis pathway, protoporphyrins or porphyrins (heme precursors) are prevented from proceeding further along the pathway. These precursors accumulate at the stage of the enzyme defect causing an array of physical symptoms in an affected person. Specific symptoms depend on the point at which heme biosynthesis is blocked and which precursors accumulate. In general, the porphyrias primarily affect the skin and the nervous system. Symptoms can be debilitating or life threatening in some cases. Porphyria is most commonly an inherited condition. It can also, however, be acquired after exposure to poisonous substances.

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64. Porphyrias
There are a several inherited disorders associated with the porphyrin syntheticpathway. There are three porphyrias with high photosensitivity.
http://www.photodermatology.com/porphyriasgen.htm
PORPHYRIAS Biochemistry of porphyrin and Heme synthesis Porphyrins are synthesized by a multi-step reaction starting with succinyl-CoA and glycine. The first step of the reaction sequence is catalyzed by the enzyme gamma-aminolevulinate synthetase which is regulated through feedback inhibition by the ultimate endpoint of the sequence, heme. The chelation of iron in the final step produces heme which is utilized as a prosthetic group in hemoglobin, myoglobin, catalase, peroxidase, and cytochrome c There are a several inherited disorders associated with the porphyrin synthetic pathway. Ther e are three porphyrias with high photosensitivity Porphyria cutanea tarda Erythropoetic Protoporphyria Erythropoietic Porphyria

65. Directory :: Look.com
porphyrias (19) See Also. information. Diagnosing the porphyrias An articleby Thomas G. DeLoughery, Associate Professor of Medicine.
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66. Fayette Medical Center Digital Library
porphyrias Clinical Resources. Chapter 27 Heme Biosynthesis and itsDisorders porphyrias and Sideroblastic Anemias Access document
http://fayettemc-dl.slis.ua.edu/clinical/metabolism/inborn/porphyria.htm
Clinical Resources by Topic: Metabolic Disorders
Porphyrias Clinical Resources
Emergency Pediatrics Atlases Radiology ... Miscellaneous Resources See also:

67. Poetry Essay An Essay Comparing Porphyrias Lover, With My Last Duchess.
Poetry Essay an Essay Comparing porphyrias Lover, With My Last Duchess. PoetryEssay an Essay Comparing porphyrias Lover, With My Last Duchess.
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71. Health/Conditions_and_Diseases/Nutrition_and_Metabolism_Disorders/Porphyrias
Health / Conditions_and_Diseases / Nutrition_and_Metabolism_Disorders / porphyrias. Apersonal site about this disorder one of the family of porphyrias.
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The result of changes in the blood's nitrogen-containing substances, or porphyrins. Health Porphyrias Porphyria Page
A patient guide for all people with this disorder. Compiled by Professor Michael Moore.
URL: http://www.uq.edu.au/porphyria/
The American Porphyria Foundation

Non-profit organization advancing awareness research and treatment of Porphyria. Fact sheets and links to related resources.
URL: http://www.enterprise.net/apf/
Porphyrias

A brief look at the two major types the symptoms and an example is given.
URL: http://medic.bgu.ac.il/mirrors/netbiochem/hi6.htm
HealthCentral - Porphyria
General overview of this rare disease. URL: http://www.healthcentral.com/mhc/top/001208.cfm Porphyrias: Diagnosis and Treatment Clinical manifestations diagnosis etiology and treatment of six types of porphyrias are discussed. URL: http://www.medstudents.com.br/metdis/metdis3.htm Porphyria: The Woman Who Has The Vampire Disease Review by the FindArticles.com on this book on porphyria written by Lenore B. Weinstein. URL: http://www.findarticles.com/cf_0/m0FSP/3_21/53578846/p1/article.jhtml

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73. Porphyria Cuatanea Tarda
Porphyria Overview. porphyrias are a group of diseases which are all deficienciesof any of the eight enzymes involved in heme synthesis.
http://www.zevils.com/~matthewg/porphyria.html
Porphyria Cutanea Tarda - A Report by Matthew Sachs
Porphyria Overview
Porphyrias are a group of diseases which are all deficiencies of any of the eight enzymes involved in heme synthesis. One of the major causes of symptoms in patients with porphyria is the accumulation of heme precursors. To make heme, the body needs some raw materials in addition to the eight enzymes. Heme procursors collect mainly in the marrow and liver. The main heme precursors that cause problems in porphyria are toxic metabolites: delta-aminolevulinic acid and porphobilinogen, which are neurotoxins, and porphyrins. These precursors are transferred from the tissues where they accumulate into the blood plasma and from there they are excreted in urine and stools. Accumulation of these precursors can cause photosensitivity and nerve damage, even paralysis. The most common porphyrias are porphyria cutanea tarda (PCT), acute intermittent porphyria , and eryhtropoietic protoporphyria . These are veru different diseases, with distinct symptoms, tests needed for proper diagnosis, and treatments. Additionally, they all share some symptoms with rarer porphyrias. Thus, porphyria is often misdiagnosed. Test results from patients with porphyria will be markedly abnormal, but one needs to select and interpret tests with porphyria in mind. All porphyrias except for one are hereditary. PCT is not inherited directly. However, suspectibility to PCT is often inherited, in which case it is called familial (type II) PCT.

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Discussion. Porphyria Cutanea Tarda (PCT) is the most common of the porphyrias. Theporphyrias are a group of at least seven disorders. The
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