21. Porphyrias porphyrias. porphyrias (pronounce) may be divided into two major types. Erythropoieticporphyria is a defect of porphyrin metabolism of bloodproducing tissues. http://medlib.med.utah.edu/NetBiochem/hi6.htm

Porphyrias Porphyrias (pronounce) may be divided into two major types. Erythropoietic porphyria is a defect of porphyrin metabolism of blood-producing tissues. Hepatic porphyria is a defect in porphyrin metabolism of the liver. Either type may be hereditary (caused by a gene defect) or acquired (due,say, to poisoning). The symptoms of porphyria cannot be completely explained by our current understanding of the biochemical defect. In all cases there is an identifiable abnormality of the enzymes which synthesize heme. This leads to accumulation of intermediates of the pathway and a deficiency of heme Accumulation of porphyrinogens in the skin can lead to photosensitivity The neurological symptoms cannot be explained. They are especially puzzling since the metabolic defect is confined to non-neural tissue, and no known diffusible intermediate can cause the observed neurological symptoms. Two examples of porphyria will serve to illustrate some of the biochemical issues involved. Return to Main Menu jb Last modified 1/5/95

22. Porphyrias porphyrias may be divided into two major types erythropoietic porphyria and Hepaticporphyria. porphyrias (pronounce) may be divided into two major types. http://www.porphyrin.net/Heme_iron/porphyrins/_phyrias.html

Go to site Porphynet MediPorph Antiporphy Guests GPS Home Heme and Iron Heme metabolism Porphyrins Heme synthesis Porphyrias Heme Degradation Iron topics Porphyrias Quiz Porphyrias (pronounce) may be divided into two major types. Erythropoietic porphyria is a defect of porphyrin metabolism of blood-producing tissues. Hepatic porphyria is a defect in porphyrin metabolism of the liver. Either type may be hereditary (caused by a gene defect) or acquired (due,say, to poisoning). The symptoms of porphyria cannot be completely explained by our current understanding of the biochemical defect. In all cases there is an identifiable abnormality of the enzymes which synthesize heme. This leads to accumulation of intermediates of the pathway and a deficiency of heme Accumulation of porphyrinogens in the skin can lead to photosensitivity The neurological symptoms cannot be explained. They are especially puzzling since the metabolic defect is confined to non-neural tissue, and no known diffusible intermediate can cause the observed neurological symptoms. Two examples of porphyria will serve to illustrate some of the biochemical issues involved.

23. A Selection Of Books About Porphyrias Go to site. porphyrias the books. Other Macalpine, The porphyrias J. LyndalYork, http://www.porphyrin.net/Mediporph/porphyria_books.html

Go to site Porphynet MediPorph Antiporphy Guests GPS Home Porphyrias the books Other Porphyrin Book Topics Dolphin Bookset Porphyrin Books: Bio Topics Porphyrin Books: the "classics" Porphyrin Books: Metal Complexes, Physical Chemistry Porphyrin Books: PDT, Medical Porphyrin Library Main Page Enter keywords... Worldwide delivery in association with Amazon.com For out of print titles, Amazon.com will attempt to secure them from other sources on request. Porphyria: The Unknown Disease Diana Deats-O'Reilly Our Price: $17.95 Read more about this title... Chemical porphyria in man George III and the mad-business Ida Macalpine The porphyrias J. Lyndal York Porphyria in Australia: a review of the literature, and Australian experience Roderick McEwin Porphyria's lover Maggie Power The porphyrias: a story of inheritance and environment Geoffrey Dean Porphyria : The Woman Who Has 'the Vampire Disease' Ships in 2-3 days Tammy Evans / Hardcover / May 1997 Our Price: $22.95 Read more about this title... Diagnosis and therapy of porphyrias and lead intoxication Mechanisms of chemical-induced porphyrinopathies Mechanisms of Chemical-Induced Porphyrinopathies (Annals of the New York Academy of Sciences, Vol 514) Ellen K. Silbergold, Bruce A. Fowler (Editor) / Hardcover / February 1988

24. Porphyrias porphyrias are a group of defects in the heme synthesis leading to theaccumulation of some precursors and to characteristic symptoms. http://erasmeinfo.ulb.ac.be/globule/English/porphyrias.htm

Porphyrias Porphyrias are a group of defects in the heme synthesis leading to the accumulation of some precursors and to characteristic symptoms. Two kinds of clinical presentations are seen (together or separately): acute crises (abdominal and neurological signs) cutaneous lesions (skin fragility and photosensitivity) Last Update: Webmaster

25. Disease Directory : Nutrition And Metabolism Disorders : Porphyrias Includes registration form for an email list. American Porphyria Foundation The porphyrias - The porphyrias. Drugs unsafe for use in acute porphyrias. http://www.diseasedirectory.net/Nutrition_and_Metabolism_Disorders/Porphyrias/de

Wednesday, June 02, 2004 Nutrition and Metabolism Disorders Amyloidosis Cholesterol and Other Fats Glycogen Storage Disease Type I ... Nutrition and Metabolism Disorders : Porphyrias Acute Intermittent Porphyria Forum - Information and links about Porph. Includes registration form for an email list. American Porphyria Foundation - The Porphyrias - The Porphyrias. The information that was on this page is now out of date. If you have copies of old versions of this page then please American Porphyria Foundation - The Porphyrias - The Porphyrias. The information that was on this page is now out of date. If you have copies of old versions of this page then please British National Formulary: 9.8.2 Acute porphyrias - 9.8.2 Acute porphyrias. or, (0113) 283 7010. St Thomas' Hospital, London, (020) 7928 9292. Sub-sections. HAEM ARGINATE. Drugs unsafe for use in acute porphyrias. Canadian Porphyria Foundation - Mission statement, objectives of the organization, newsletters and safe and unsafe drugs. Also: A Guide to Porphyria - a complete booklet of information. Childhood Porphyrias - 77:825-836 825 © 2002 Mayo Foundation for Medical Education and Research Subspecialty Clinics: Dermatology I FTIKHAR A HMED , MD Childhood Porphyrias From the

26. Disease Directory : Porphyrias More porphyrias Categories » Submit Your Siteto the porphyrias category. Submit Your Site to the porphyrias category....... Title porphyrias http://www.diseasedirectory.net/detailed/22413.aspx

Wednesday, June 02, 2004 Nutrition and Metabolism Disorders Amyloidosis Cholesterol and Other Fats Glycogen Storage Disease Type I ... Porphyrias : Porphyrias Directory Listing Title: Porphyrias Description: Date Added: 2/13/2004 3:00:12 PM URL: http://www.iseekhealth.com/porphyrias-2177.php

27. Porphyrias porphyrias. 1. CLASSIFICATION. The porphyrias are a heterogeneous groupof either inherited or acquired disorders of haem biosynthesis. http://ruby.med.cuhk.edu.hk/~cpy/lecture/porphyrias.html

Dr C W Lam 2 November 1998 DEPARTMENT OF CHEMICAL PATHOLOGY THE CHINESE UNIVERSITY OF HONG KONG PORPHYRIAS 1. CLASSIFICATION 2. HAEM BIOSYNTHESIS 3. SYMPTOMS AND SIGNS 4. ENZYMIC DEFECTS IN THE HAEM-SYNTHETIC PATHWAY 5. CLINICAL INDICATIONS FOR PERFORMING PORPHYRIN INVESTIGATIONS 6. LABORATORY INVESTIGATION OF THE PORPHYRIAS 7. PORPHYRINURIA 1. 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) 2. Haem Biosynthesis 3. 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: Photosensitivity is often a feature of those porphyrias in which there are excessive amounts of uro-, copro- or protoporphyrin in tissues. In practice, photosensitivity may be present in all

28. Porphyrias porphyrias. Definition The porphyrias are a group of rare disordersthat affect heme biosynthesis. Heme is an essential http://www.chclibrary.org/micromed/00061410.html

Main Search Index Definition Description Causes ... Resources Porphyrias Definition The porphyrias are a group of rare disorders that affect heme biosynthesis. Heme is an essential component of hemoglobin as well as many enzymes throughout the body. Description Biosynthesis of heme is a multistep process that starts with simple molecules and ends with a large, complex heme molecule. Each step of the biosynthesis pathway is directed by its own task-specific protein, called an enzyme. As a heme precursor molecule moves through each step, an enzyme modifies it in some way. If the precursor is not modified, it cannot proceed to the next step. 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 porphyrin (heme precursors) are prevented from proceeding further along the pathway. Instead, precursors accumulate at the stage of the enzyme defect and cause an array of physical symptoms in the 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 an inherited condition, but it may be acquired after exposure to poisonous substances. Heme Heme is produced in several tissues in the body, but its primary biosynthesis sites are the liver and the bone marrow. Heme synthesis for immature red blood cells, namely the erythroblasts and the reticulocytes, occurs in the bone marrow.

29. Nutrition And Metabolism Disorders, Porphyrias More porphyrias Categories » Submit Your Site to the porphyrias category.Sponsored porphyrias Sites. Submit Your Site to the porphyrias category. http://www.iseekhealth.com/porphyrias-2177.php

Home About Us Contact Submit Your Site Search : Home Health Conditions and Diseases Nutrition and Metabolism Disorders ... Porphyrias More Porphyrias Categories: Submit Your Site to the Porphyrias category Sponsored Porphyrias Sites Subscribe To Mayo Clinic Health Letter Nutrition Learn your dream career from home. Request a Free Career Info Kit and get started today! Nutrition Now At Total Health Save up to 20% on Nutrition Now Vitamins and Supplements. Free shipping offers. Try Seasilver For Free! We're so sure you'll love Seasilver we want you to try it Free! No strings attached, no hassle. Just click through and it's yours. High Quality Nutritional Supplements Slim Factors weight loss, MSM, first milk colostrum, Carlson DHA/EPA fish oils, chlorella, oregano / essential oils, joint care, grape seed extract, ionic trace minerals, msm lotion, green juice pdrs, celtic sea salt, Wobenzym N, Barlean's flax oil.. Acute Intermittent Porphyria Forum - Information and links about Porph. Includes registration form for an email list. Canadian Porphyria Foundation - Mission statement, objectives of the organization, newsletters and safe and unsafe drugs. Also: A Guide to Porphyria - a complete booklet of information.

30. Nutrition And Metabolism Disorders, Porphyrias Category Home Health Conditions and Diseases Nutrition and MetabolismDisorders porphyrias. * Site Title · The name of the site. http://www.iseekhealth.com/directory/index.php?method=show_link_exchange&directo

31. The Porphyrias Michael W. KING, Ph.D / IU School of Medicine / mking@medicine.indstate.edu SergioMARCHESINI/Professor of Biochemistry/University of Brescia / marchesi@med http://www.med.unibs.it/~marchesi/porphyrias.html

The Porphyrias Congenital Erythropoietic Porphyria (CEP) Erythropoietic Protoporphyria (EPP) ALA Dehydratase Deficiency Porphyria (ADP) Acute Intermittent Porphyria (AIP) ... Hepatoerythropoietic Porphyria (HEP) Michael W. KING, Ph.D / IU School of Medicine / mking@medicine.indstate.edu Sergio MARCHESINI/Professor of Biochemistry/University of Brescia / marchesi@med.unibs.it Last Updated: 22.09.2003

32. Porphyrias porphyrias. Teixeira Basto Medstudents Homepage porphyrias Clinical Manifestations,Diagnosis and Treatment DIAGNOSING THE porphyrias http://www.health-nexus.com/porphyrias.htm

Health-Nexus.Net Health-Nexus.Org The #1 Health information site Home ... Up Search Health-Nexus for: Match ALL words Match ANY word Email this page to a friend ! Post a question or comment on our Message Board Home Page Health Specialties Health News ... Alternative Health Options Substance Abuse Animal Health Search: Books Magazines Video Keywords: Find it Here Porphyrias Porphyrias: Clinical Manifestations, Diagnosis and Treatment MEDSTUDENTS-METABOLIC DISORDERS Clinical manifestations, diagnosis, etiology and treatment of six types of porphyrias are rapidly ... Teixeira Basto Medstudents' Homepage Porphyrias: Clinical Manifestations, Diagnosis and Treatment ... DIAGNOSING THE PORPHYRIAS ... DIAGNOSING THE PORPHYRIAS. ... REFERENCES. Desnick RJ and Anderson KE. Heme biosynthesis and its abnormalities: the porphyrias and sideroblastic anemia. ... THE MERCK MANUALHome Edition, Sec. 12, Ch. 141, Porphyrias ... Porphyrias. Porphyrias are a group of disorders caused by deficiencies of enzymes involved in the synthesis of heme. ... THE MERCK MANUAL, Sec. 2, Ch. 14, The Porphyrias

33. THE PORPHYRIAS The porphyrias. © 199598 Dr. Maged Nabih Kamel Boulos. All rightsreserved. http//www.bitsmart.com/dermocad. Acronyms AIP, Acute http://members.tripod.com/~DermoCAD/porphyria.html

var cm_role = "live" var cm_host = "tripod.lycos.com" var cm_taxid = "/memberembedded" The Porphyrias http://www.bitsmart.com/dermocad Acronyms: AIP, A cute I ntermittent P orphyria; CEP, C ongenital E rythropoietic P orphyria; PCT, P orphyria C utanea T arda; HEP, H epato e rythropoietic P orphyria; HCP, H ereditary C opro p orphyria; VP, V ariegate P orphyria; EPP, E rythropoietic P roto p orphyria Mechanism of porphyrin-induced photosensitivity: The photosensitivity of the skin in porphyria is due to porphyrins absorbing ultraviolet radiation in the 400 nm range and emitting an intense red fluorescence. Porphyrins, when irradiated with light of the appropriate wavelength in the presence of oxygen, will cause photodynamic effects. Light energy absorbed by the porphyrin raises electrons into an excited state. Energy released on return of the molecule to its original state reacts with oxygen to produce free radicals and singlet oxygen (O) which damages molecules, cells and tissues. Unsaturated lipids are particular targets for activated oxygen species; cell damage results from the resulting plasma membrane and lysosomal membrane injury and also from complement activation. -Carotene, a known quencher of free radicals and singlet oxygen, has a photoprotective effect in porphyria.

34. ScienceDaily -- Browse Topics: Health/Conditions_and_Diseases/Nutrition_and_Meta News about porphyrias More news about porphyrias . Books about porphyrias More books about porphyrias . Links about porphyrias http://www.sciencedaily.com/directory/Health/Conditions_and_Diseases/Nutrition_a

Match: sort by: relevance date Free Services Subscribe by email RSS newsfeeds PDA-friendly format loc="/images/" A A A Find Jobs In: Healthcare Engineering Accounting College Contract / Freelance Customer Service Diversity Engineering Executive Healthcare Hospitality Human Resources Information Tech International Manufacturing Nonprofit Retail All Jobs by Job Type All Jobs by Industry Relocating? Visit: Moving Resources Moving Companies Mortgage Information Mortgage Calculator Real Estate Lookup Front Page Today's Digest Week in Review Email Updates ... Nutrition and Metabolism Disorders Porphyrias (17 links) See Also: Health: Conditions and Diseases: Genetic Disorders Health: Conditions and Diseases: Rare Disorders News about Porphyrias [ More news about Porphyrias Books about Porphyrias Amazon.com's Price: Prices subject to change. The Official Patient's Sourcebook on Porphyria: A Revised and Updated Directory for the Internet Age by: Icon Health Publications August, 2002 Amazon.com's Price: Prices subject to change. Skin Pharmacology and Applied Skin Physiology Physiology: Porphyria by: Hans F. Merk

35. CCHS Clinical Digital Library Disorders. porphyrias Clinical Resources. INFO ) Chapter 346 Theporphyrias Table of contents Heme Biosynthesis Access document; http://cchs-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: Genetic Testing Clinical Resources General Genetic Disorders Clinical Resources General Metabolic Disorders Clinical Resources Porphyrias Patient/Family Resources Harrison's Principles of Internal Medicine 15th Ed.-2001: Table of contents Health Sciences Library subscription INFO Chapter 346: The Porphyrias: Table of contents Heme Biosynthesis: Access document Regulation of Heme Biosynthesis: Access document The Hepatic Porphyrias: Access document Introduction: Access document ALA Dehydratase-Deficient Porphyria: Access document Treatment: Access document Acute Intermittent Porphyria: Access document Treatment: Access document Porphyria Cutanea Tarda: Access document Treatment: Access document Hereditary Coproporphyria: Access document Treatment: Access document Variegate Porphyria: Access document Treatment: Access document The Erythropoietic Porphyrias: Access document Introduction: Access document X-Linked Sideroblastic Anemia: Access document Treatment: Access document Congenital Erythropoietic Porphyria: Access document Treatment: Access document Erythropoietic Protoporphyria: Access document Treatment: Access document Hoffman: Hematology: Basic Principles and Practice 3rd Ed.-2000 (MD Consult):

36. CCHS Clinical Digital Library porphyrias Patient/Family Resources. Spanish Miscellaneous. Miscellaneousporphyrias Patient/Family Resources Healthfinder (US DHHS) Homepage http://cchs-dl.slis.ua.edu/patientinfo/metabolism/inborn/porphyria.htm

Patient/Family Resources by Topic: Metabolic Disorders Porphyrias Patient/Family Resources Spanish Miscellaneous See also: Genetic Testing Patient/Family Resources General Genetic Disorders Patient/Family Resources Porphyrias Clinical Resources National Institute of Diabetes and Digestive and Kidney Diseases: Homepage Kidney Diseases Publications Online: List of documents Porphyrias: Access document MEDLINE plus Medical Encyclopedia: Table of contents Acute Porphyria: English Spanish Hepatic Porphyria: English Spanish Porphyria: English Spanish Merck Manual - Second Home Edition Table of contents Section 12. Disorders of Nutrition and Metabolism: Table of contents Chapter 160. Porphyrias: Table of contents Introduction: Access document Porphyria Cutanea Tarda: Access document Acute Intermittent Porphyria: Access document Erythropoietic Protoporphyria: Access document Spanish MEDLINE plus Medical Encyclopedia: Table of contents Acute Porphyria: English Spanish Hepatic Porphyria: English Spanish Porphyria: English Spanish Miscellaneous Porphyrias Patient/Family Resources Healthfinder (US DHHS): Homepage Genetics: List of documents National Library of Medicine MEDLINE plus Health Topics: Index Porphyria: List of documents Genetic Testing/Counseling: List of documents Genetic Disorders: List of documents YAHOO - Health:Diseases and Conditions:Porphyria Additional Porphyria resources ( These sites have not been reviewed.

37. American Porphyria Foundation - The Porphyrias The porphyrias. The information that was on this page is now out ofdate. If you have copies of old versions of this page then please http://www.enterprise.net/apf/overview.html

The Porphyrias The information that was on this page is now out of date. If you have copies of old versions of this page then please delete those copies because the information no longer reflects the latest research. If you have links to this page then please remove those links because this page is no longer being maintained. For more general information on Porphyria, please see the main page of the American Porphyria Foundation

38. The Porphyrias The porphyrias by Shane Greene, Pharm.D. Introduction The porphyrias are a groupof inherited or acquired genetic defects in the heme biosynthetic pathway. http://www.clevelandclinicmeded.com/medical_info/pharmacy/septoct1999/pharm.htm

The Porphyrias by Shane Greene, Pharm.D. Introduction: The porphyrias are a group of inherited or acquired genetic defects in the heme biosynthetic pathway. These defects cause complete or near complete inactivation of the genes encoding for the enzymes that catalyze the transformation of porphyrin precursors. A deficiency in one of these enzymes leads to overproduction and accumulation of these precursors. The specific type of porphyria depends on the location of the accumulation and the enzyme affected. The porphyrias mainly manifest as effects on the nervous system and skin. Classification: The porphyrias are classified as either hepatic or erythropoietic. Hepatic porphyrias have normal red blood cell porphyrin concentrations and present clinically with neurologic symptoms, abdominal pain, neuropathy, and/or psychiatric symptoms. Erythropoietic porphyrias present with an increased concentration of porphyrin within the red blood cells and cutaneous photosensitivity manifestations. Epidemiology: Etiology and Pathogenesis: A different gene encodes each enzyme within the heme biosynthetic pathway. A deficiency in one of the genes is associated with a specific type of porphyria

39. The Porphyrias The porphyrias. Congenital Erythropoietic Porphyria (CEP); ErythropoieticProtoporphyria (EPP); ALA Dehydratase Deficiency Porphyria (ADP); http://www.dentistry.leeds.ac.uk/biochem/thcme/porphyrias.html

The Porphyrias Congenital Erythropoietic Porphyria (CEP) Erythropoietic Protoporphyria (EPP) ALA Dehydratase Deficiency Porphyria (ADP) Acute Intermittent Porphyria (AIP) ... back to Inborn Errors Page Return to Medical Biochemistry Page Michael W. King, Ph.D / IU School of Medicine / mking@medicine.indstate.edu Last modified:

40. Target PG.... Biochemistry Porphyrias Hemoglobin Heme Synthesis High Yield Point Target PG porphyrias. porphyrias. Enyme No. Enxyme deficient. Other Name. Prodcut.Chromosome. Inheritance. Name. Other Name. Liver. Erythrocyte. Fibroblast. Lymphocyte. http://www.targetpg.com/portal/files/subjects/bioc01porphyria.php

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