81. WebMD - As is characteristic of the erythropoietic porphyrias, symptoms usually beginduring infancy. The porphyrias are a group of at least seven disorders. http://my.webmd.com/NR/internal.asp?GUID={B9946B88-B36D-4F93-BD2D-B0157DAF88DB}

82. Silver Hill Hospital Digital Library Disorders. porphyrias Clinical Resources. Porphyria (Keyword search)List of documents. Miscellaneous porphyrias Clinical Resources YAHOO http://silverhillhospital-dl.slis.ua.edu/clinical/metabolism/inborn/porphyria.ht

Clinical Resources by Topic: Metabolic Disorders Porphyrias Clinical Resources Emergency Pediatrics Atlases Radiology ... Miscellaneous Resources See also: Genetic Testing Clinical Resources General Genetic Disorders Clinical Resources General Metabolic Disorders Clinical Resources Porphyrias Patient/Family Resources The Merck Manual 17th Ed.-1999: Table of contents Chapter 14: The Porphyrias: Table of contents General: Access document Most Common Porphyrias: Access document Less Common Porphyrias: Access document Medicine, Ob/Gyn, Psychiatry, and Surgery (eMedicine): Table of contents Porphyria, Acute Intermittent: Access document Porphyria, Chester: Access document ALA Dehydratase Deficiency Porphyria: Access document Dermatology (eMedicine): Table of contents Porphyria Cutanea Tarda: Access document Variegate Porphyria: Access document Erythropoietic Porphyria: Access document Dermatologic Manifestations of Neurologic Disease: Access document Neuromuscular Disease Center (WUSTL): Homepage Porphyria: Access document CliniWeb: Homepage (includes links to targeted PubMed MEDLINE searches) Porphyria: List of documents Emergency Medicine Resources See also General Emergency Medicine Resources Emergency Medicine (eMedicine): Table of contents Erythropoietic Porphyria: Access document Porphyria Cutanea Tarda: Access document Variegate Porphyria: Access document Pediatrics Resources See also General Pediatrics Resources Pediatrics (eMedicine): Table of contents Porphyria, Acute:

83. EMedicine - Diseases Of Tetrapyrrole Metabolism: Refsum Disease And The Hepatic Diseases of Tetrapyrrole Metabolism Refsum Disease and the Hepatic porphyrias Refsum disease (RD) and the hepatic porphyrias are rare inherited http://www.emedicine.com/neuro/byname/diseases-of-tetrapyrrole-metabolism--refsu

(advertisement) Excerpt from Diseases of Tetrapyrrole Metabolism: Refsum Disease and the Hepatic Porphyrias Synonyms, Key Words, and Related Terms: phytanic acid storage, porphyria Please click here to view the full topic text: Diseases of Tetrapyrrole Metabolism: Refsum Disease and the Hepatic Porphyrias Background: Refsum disease (RD) and the hepatic porphyrias are rare inherited neurodegenerative conditions with exacerbations and remissions due to abnormal metabolism of large tetrapyrrole molecules. Two common examples of large tetrapyrrole molecules are chlorophyll a, the photosynthetic pigment of green plants, and heme, the prosthetic group of hemoglobin (see Picture 1 ). Side groups on both species involve relatively small organic groups (methyl, vinyl, and free propionyl); 1 major exception is phytol, a large hydrocarbon alcoholic substituent on chlorophyll. Patients in both disease categories must avoid foods and drugs that lead to high levels of the relevant biological toxin, which can trigger or perpetuate an exacerbation. Pathophysiology: The neurotoxin in RD is phytanic acid, which in affected individuals is stored in neural and visceral parenchyma because of a deficiency in phytanic acid alpha-hydroxylase. The source of phytanic acid is either direct absorption or conversion of absorbed phytol from ruminant fat in meat or milk (only ruminants can release phytol from chlorophyll during digestion). Homozygosity is required for significant phytanic acid build-up.

84. Listings Of The World Health Conditions And Diseases Nutrition Listings World Health Conditions and Diseases Nutrition and MetabolismDisorders porphyrias. Listings World, http://listingsworld.com/Health/Conditions_and_Diseases/Nutrition_and_Metabolism

85. Health Library - Porphyria Cutanea Tarda None. General Discussion. Porphyria Cutanea Tarda (PCT) is the most commonof the porphyrias. The porphyrias are a group of at least seven disorders. http://health_info.nmh.org/Library/HealthGuide/IllnessConditions/topic.asp?hwid=

86. Dr C W K Lam porphyrias. 1. CLASSIFICATION 2. These investigations must be performed ina specialist laboratory. 6. Laboratory Investigation of the porphyrias. http://www.cpy.cuhk.edu.hk/lecture/1999-2000/week25/Porphyrias.htm

Dr C W Lam 6 December 1999 DEPARTMENT OF CHEMICAL PATHOLOGY THE CHINESE UNIVERSITY OF HONG KONG PORPHYRIAS CLASSIFICATION Haem Biosynthesis Symptoms and Signs Symptoms and Signs Enzymic Defects in the Haem-synthetic Pathway Clinical indications for performing porphyrin investigations Laboratory Investigation of the Porphyrias Porphyrinuria CLASSIFICATION The Porphyrias are a heterogeneous group of either inherited or acquired disorders of haem biosynthesis. In these diseases, specific abnormalities of enzymes in the biosynthetic pathway cause generalised clinical abnormalities. They are classified, as shown below, into acute and non-acute porphyria. ACUTE PORPHYRIA ACUTE INTERMITTENT PORPHYRIA (AIP) VARIEGATE PORPHYRIA (VP) HEREDITARY COPROPORPHYRIA (HC) PLUMBOPORPHYRIA NON-ACUTE PORPHYRIA PORPHYRIA CUTANEA TARDA (PCT) ERYTHROPOIETIC PROTOPORPHYRIA (EP) CONGENITAL ERYTHROPOIETIC PORPHYRIA (CEP) Haem Biosynthesis Symptoms and Signs Different stages in haem synthesis are affected in each disease and many different symptoms may be present. However, two main groups of manifestations can be recognized, depending on the nature of the enzyme deficiency:

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89. Browsing Health Conditions And Diseases Nutrition And Metabolism Browse Health Conditions and Diseases Nutrition and Metabolism Disorders porphyrias Apersonal site about this disorder, one of the family of porphyrias. http://www.uksprite.com/search/search/Health/Conditions_and_Diseases/Nutrition_a

90. PORPHYRINS AND PORPHYRIAS PORPHYRINS AND porphyrias. Heme is a prosthetic group that is responsiblefor the color of our blood and is a fundamental element http://www.isat.jmu.edu/users/klevicca/350_Web/Sarahs_Group/Part1.htm

PORPHYRINS AND PORPHYRIAS H eme is a prosthetic group that is responsible for the color of our blood and is a fundamental element to the human respiratory system. Once heme is combined with globin to form hemoglobin, this prosthetic group carries oxygen and carbon dioxide throughout the circulatory system. Heme is also associated with cytochromes in the electron transport system as well as with the active sites of several important detoxifying enzymes. As you can see, heme plays a vital role in human physiology and metabolism, and as a result, our bodies' ability to produce adequate amounts of heme is essential for our health. Heme is biosynthesized along a biochemical pathway involving seven enzyme-mediated steps. The pathway is started by the conversion of 5-aminolevulinic acid (ALA) into a pyrrole building block that is consequently polymerized and cyclized to result in a unique macrocyclic tetrapyrrole. This intermediate is then altered by the removal of some of the peripheral carboxylic acid side chains and the introduction of three more double bonds into the macrocyclic infrastructure to form a porphyrin called protoporphyrin. The final step in the biochemical pathway is the insertion of ferrous iron. The porphyrias are a group of disorders involved with the heme biosynthetic pathway where there is a reduction in the activity of any one of the seven enzymes of porphyrin biosynthesis. Porphyria may be either acquired, for example, by the action of a toxic chemical agent on one of the enzymes, or inherited by means of a genetic defect in one of the genes encoding the heme biosynthetic enzymes. The consequential reduction in enzyme activity produces a blockage in the pathway leading to a build-up of pathway intermediates.

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92. Porphyria Links Porphyria Web Links This document maintained by Robert J. Huskey Last updated on December 14, 2000. http://www.people.virginia.edu/~rjh9u/aip.html

Porphyria Web Links Diagnosis of Acute Intermittant Porphyria "The Girl Who Mewed" Discover , Aug. 1995 Diagnosis of Congenital Erythropoietic Porphria "A Life in the Shadows" Discover , Feb. 1997 (search Archives using date) Pedigree of Variegate Porphyria Biochemistry of Heme Synthesis Biochemistry of Heme Synthesis at Hahnemann Univ. Heme and Iron from NetBiochem American Porphyria Foundation Porphynet Canadian Porphyria Foundation Porphyria: The Unknown Disease ... adam.com (porphyria) The Porphyria Forum Porphyria Links (fr Greece) Family Village Porphyria Page Texas Medical Center Descriptions (Houston) Ctr. for Study of Disorders of Iron and Porphyrin Metabolism (U. Mass.) Washington Univ. Descriptions and Links Porphyria Descriptions (Dr. Moyra Smith) Porphyria On-Line Mendelian Inheritance in Man (search for porphyria) Porphyria Treatment (New Scientist, 1999) King George III of England Queen Charlotte, Consort of King George Lead Poison Phenocopy of Porphyria Discover , Jan. 1996 (search Archives using date) Scarlett's Porphyria Place Brent Johnson's Porphyria Page (and mailing list) Porphyria's Lover (Robert Browning) Porphyria Web Forum (Harvard Med.)

93. A Canary's-Eye View — P45Os And The Poryphyrias A Canary's Eye View — Medical Links. http://www.loudzen.com/canary/links/p450-links.html

A Canary's-Eye View Introduction Articles Challenges Conditions ... The Liver Lo-carb Rotation Food Reactions Candida EI/MCS/CFIDS/ME/FM Toxic Chemicals Fluoride Garden Pesticides Mercury Living with EI Shopping Building GYN Other Canaries' Sites Other AltMed What's New ... CHC Home Cytochrome P450 Enzymes Essential P450s - Heme thiolate proteins and their place in the biosphere. A brief but comprehensive introductory survey offered as a tutorial for a Pharmacology course. The Detoxification Enzyme Systems by DeAnn J. Liska, PhD. Brief survey of Phases I, II and III, with extensive references. Cytochrome P450s in humans MENOPAUS: enzyme P450 Cytochrome P450 Cytochrome P450 Homepage - the website of the Committee for Standardized Cytochrome P450 Nomenclature, maintained by Dr. David Nelson. Updated frequently. Listings of drugs by what P450 enzyme they use P450 Metabolism; Managing Drug Interactions Clinically Used Drugs Metabolized by Cytochrome P450 P450s and Progesterone Aromatase Cytochrome P450 and in Situ Estrogen Production - conversion of androgens to estrogens Xenobiotic-Metabolizing Enzymes - by Mendelsohn et al, chapter "Xenobiotic-Metabolizing Enzymes in Biomarker Research,"

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