21. Porphyrias porphyrias (pronounce) may be divided into two major types. Erythropoietic porphyriais 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. Iron, Heme And Porphyrin Metabolism Some disorders of heme biosynthesis are more insidious such as the various porphyrias, a list of which can be found below and in the Inborn http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

23. Porphyrias LABORATORY INVESTIGATION OF THE porphyrias 7. PORPHYRINURIA. 1. CLASSIFICATION.The porphyrias are a heterogeneous group of either inherited or acquired 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

24. MedlinePlus Medical Encyclopedia: Porphyria porphyrias are characterized by 3 major findings photodermatitis (light sensitivity The porphyrias are lifelong intermittent illnesses, but with good http://www.nlm.nih.gov/medlineplus/ency/article/001208.htm

@import url(/medlineplus/images/advanced.css); Skip navigation Medical Encyclopedia Other encyclopedia topics: A-Ag Ah-Ap Aq-Az B-Bk ... Z Porphyria Contents of this page: Illustrations Alternative names Definition Causes, incidence, and risk factors ... Prevention Illustrations Porphyria cutanea tarda on the hands Alternative names Return to top Acute intermittent porphyria; Hereditary coproporphyria; Congenital erythropoietic porphyria; Erythropoietic protoporphyria Definition Return to top Porphyria is a group of inherited disorders involving abnormalities in the production of heme pigments (the base material responsible for hemoglobin (red blood cell pigment), myoglobin (reddish muscle cell pigment), and another group of materials called cytochromes. Porphyrias are characterized by 3 major findings: photodermatitis ( light sensitivity causing rashes ), neuropsychiatric complaints, and visceral complaints (such as abdominal pain or cramping). Causes, incidence, and risk factors Return to top The porphyrias may be inherited as either autosomal dominant (caused by inheriting 1 abnormal copy of the gene) or autosomal recessive (requires inheritance of 2 abnormal genes) traits, depending on which type of porphyria is involved. Some types of porphyria begin in early childhood, some at puberty, and others begin during adulthood. A classical attack of acute porphyria generally begins with colicky abdominal pain (severe), and is then followed by vomiting and constipation. Personality changes may develop during an

25. Www.nlm.nih.gov/cgi/mesh/2005/MB_cgi?mode= Ter research services and healthcare information to physicians, http://www.nlm.nih.gov/cgi/mesh/2005/MB_cgi?mode=&term=PORPHYRIAS

26. Porphyrias porphyrias may be divided into two major types Erythropoietic Porphyria andHepatic Porphyria. 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.

27. A Selection Of Books About Porphyrias The porphyrias a story of inheritance and environment Geoffrey Dean Diagnosis and therapy of porphyrias and lead intoxication 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

28. Porphyrias The site informs the user of many important proteins which contain haem, porphyrins,haem synthesis regulation and degradation, porphyrias, http://bioresearch.ac.uk/browse/mesh/D011164.html

low graphics Porphyrias Heme and iron An introduction to haem and iron metabolism. The site informs the user of many important proteins which contain haem, porphyrins, haem synthesis regulation and degradation, porphyrias, and iron metabolism, absorption, uptake, transport and storage. A set of multiple choice exam questions are available for the user to test their understanding of the subject. This site is produced by NetBiochem which is intended to be a "complete Medical Biochemistry center which can be accessed over a computer network". NetBiochem is made available by the University of Utah. Teaching Materials Porphyrins Porphyrias Iron / metabolism ... Audiovisual Aids Last modified: 09 Sep 2005

29. Porphyrias Gordon, Neal. The Acute porphyrias. Brain Development 21 (September 1999)37377. Thadani, Helen et al. Diagnosis and Management of Porphyria. http://www.healthatoz.com/healthatoz/Atoz/ency/porphyrias.jsp

30. Porphyrias porphyrias Up/Haut Menu français English Menu Biosynthesis Porphyrins Acute Porph. Cutaneous Porph. porphyrias are a group of defects in the heme 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

31. ScienceDaily -- Browse Topics: Health/Conditions_and_Diseases/Nutrition_and_Meta Search Gale Encyclopedia of Nursing and Allied Health porphyrias Diagnosing the porphyrias An article by Thomas G. DeLoughery, Associate Professor http://www.sciencedaily.com/directory/Health/Conditions_and_Diseases/Nutrition_a

@import "/styles/navbar.css"; @import "/styles/tabStyles.css"; Set home page Bookmark site Add search Latest News ... Email to friend Text Size A A A Front Page ... Nutrition and Metabolism Disorders : Porphyrias Subtopics See Also: Health: Conditions and Diseases: Genetic Disorders Health: Conditions and Diseases: Rare Disorders Search Google: Porphyrias News Books Links [ More news 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 01, 2002 Amazon.com's Price: Prices subject to change. Gale Encyclopedia of Medicine : Porphyrias by: Judy C. Hawkins MS August 01, 2002 Amazon.com's Price: Prices subject to change. Gale Encyclopedia of Nursing and Allied Health : Porphyrias by: Barbara Wexler August 01, 2002 Amazon.com's Price: Prices subject to change. Porphyria: A Medical Dictionary, Bibliography, And Annotated Research Guide To Internet References by: Icon Health Publications April 30, 2004 Amazon.com's Price:

32. Porphyrias - Patient UK porphyrias Patient UK. A directory of UK health, disease, illness and relatedmedical websites that provide patient information. http://www.patient.co.uk/showdoc/40001141/

PatientPlus articles are written for doctors and so the language can be technical. However, some people find that they add depth to the articles found in the other sections of this website which are written for non-medical people. Porphyrias Group of inherited disorders caused by defective biosynthesis of haem. There are 7 enzymes in the haem synthesis pathway and a partial deficiency in 1 of them causes accumulation of porphyrin and porphyrin precursors producing characteristic clinical and biochemical features. Porphyrias are divided into: Neuropsychiatric – acute intermittent porphyria and plumboporphyria. Dermatological – congenital erythropoietic porphria, porphyria cutanea tarda, erythropoietic protoporphyria. Mixed – hereditary coproporphyria, variegate porphyria (cause both cutaneous and neuropsychiatric symptoms). Epidemiology Incidence Prevalence Uncommon. 1/10,000 population are heterozygous for the acute porphyria gene with 5-10% developing an acute attack. Much more common in population of South Africa (1/1,000 population). Presentation and Investigations Acute intermittent porphyria Commonest form, widely varying clinical presentation. Commonest symptom is a severe

33. Porphyrias Lover - A Poem By Robert Browning We hope that you liked this poem and the sentiments in the words of porphyriasLover by Robert Browning you will find even more poem lyrics by this famous http://www.poetry-online.org/browning_robert_porphyrias_lover.htm

Page Back Poetry Index Porphyria's Lover - a poem by Robert Browning The rain set early in tonight, The sullen wind was soon awake, It tore the elm-tops down for spite, And did its worst to vex the lake: I listened with heart fit to break. When glided in Porphyria; straight She shut the cold out and the storm, And kneeled and made the cheerless grate Blaze up, and all the cottage warm; Which done, she rose, and from her form Withdrew the dripping cloak and shawl, And laid her soiled gloves by, untied Her hat and let the damp hair fall, And, last, she sat down by my side And called me. When no voice replied, She put my arm about her waist, And made her smooth white shoulder bare, And all her yellow hair displaced, And, stooping, made my cheek lie there, And spread, o'er all, her yellow hair, Murmuring how she loved meshe Too weak, for all her heart's endeavor, To set its struggling passion free From pride, and vainer ties dissever, And give herself to me forever. But passion sometimes would prevail, Nor could tonight's gay feast restrain A sudden thought of one so pale For love of her, and all in vain:

34. Porphyrin: Definition And Much More From Answers.com The porphyrias are inherited or acquired disorders of certain enzymes in the heme They are broadly classified as hepatic porphyrias or erythropoietic http://www.answers.com/topic/porphyria

showHide_TellMeAbout2('false'); Business Entertainment Games Health ... More... On this page: Dictionary Medical WordNet Wikipedia Mentioned In Or search: - The Web - Images - News - Blogs - Shopping porphyrin Dictionary por·phy·rin p´r fə-rÄ­n n. Any of various organic compounds containing four pyrrole rings, occurring universally in protoplasm, and functioning as a metal-binding cofactor in hemoglobin, chlorophyll, and certain enzymes. [Greek porphurā , shellfish yielding purple dye, purple + –IN Medical por·phy·rin p´r fə-rÄ­n n. Any of various heterocyclic compounds, derived from pyrrole, that occur universally in protoplasm, contain a central metal atom, and provide the foundation structure for hemoglobin, chlorophyll, and certain enzymes. WordNet Note: click on a word meaning below to see its connections and related words. The noun porphyrin has one meaning: Meaning #1 any of various pigments distributed widely in living tissues Wikipedia porphyrin A porphyrin is a heterocyclic macrocycle made from 4 pyrrole subunits linked on opposite sides through 4 methine bridges. The macrocycle, therefore, is completely

35. The Porphyrias The porphyrias. Congenital Erythropoietic Porphyria (CEP) ErythropoieticProtoporphyria (EPP) ALA Dehydratase Deficiency Porphyria (ADP) http://web.indstate.edu/thcme/mwking/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 / miking at iupui.edu Last modified: Tuesday, 23-Aug-2005 15:26:45 EST

36. Hardin MD : Porphyria / Porphyrias From the University of Iowa, the *best* lists of Internet sources in Porphyria /porphyrias. http://www.lib.uiowa.edu/hardin/md/porphyria.html

Porphyria / Porphyrias A service of the University of Iowa Site Map Diseases Home ... Diseases Search Hardin MD See also: Home Skin Diseases Blood Diseases Popular Women's Health Dermatology Nursing Pharm Infect Disease AIDS +Pictures Anemia +Pictures Blood Diseases Chiuldrens Diseases Eye Diseases +Pictures Genetic Disorders +Pictures Hemochromatosis +Pictures Hepatitis C +Pictures Immune System +Pictures Liver Disease +Pictures Medical Pictures Nervous System Diseases +Pictures Oral Diseases +Pictures Skin Pictures Sun Poisoning +Pictures Porphyria Pictures / Porphyrias Pictures MEDLINE plus Porphyria National Library of Medicine Open Directory Project : (Other versions: Google Karolinska : Porphyrias Medical Library, Karolinska Institute, Sweden LookSmart : Porphyria Yahoo : Porphyria Porphyria Pictures / Porphyrias Pictures Porphyria cutanea tarda Dermatology Online Atlas (DermIS), Univ Erlangen/Univ Heidelberg, Germany Porphyria cutanea tarda (Includes pictures from some sites on this page) Aldo Campana, Univ Geneva, Switzerland Porphyria cutanea tarda DermAtlas (Dermatology Image Atlas), Johns Hopkins Univ

37. CCHS Clinical Digital Library Chapter 33 Heme Biosynthesis and its Disorders porphyrias and SideroblasticAnemias Access Chapter 270 - Hepatic porphyrias Access document http://cchs-dl.slis.ua.edu/clinical/metabolism/inborn/porphyria.htm

Clinical Resources by Topic: Metabolic Disorders Porphyrias Clinical Resources Pediatrics Atlases Radiology Pathology ... 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 16th Ed.-2005: Table of contents Health Sciences Library subscription INFO Chapter 337. The Porphyrias: Table of contents Introduction: Access document The Hepatic Porphyrias: Access document The Erythropoietic Porphyrias: Access document Further Reading: Access document Chapter 354. Disorders of the Autonomic Nervous System: Table of contents Introduction: Access document Anatomic Organization: Access document Clinical Evaluation: Access document Approach to the Patient: Access document Specific Syndromes of ANS Dysfunction: Access document Treatment: Access document Acknowledgement: Access document Further Reading: Access document Hoffman: Hematology: Basic Principles and Practice 4th Ed.-2005 (MD Consult): Table of contents Health Sciences Library subscription INFO Part IV - Red Blood Cells: Access document Chapter 33 - Heme Biosynthesis and its Disorders: Porphyrias and Sideroblastic Anemias: Access document Introduction: Access document Heme Biosynthesis: Access document Porphyrias: Access document [Introduction]: Access document Acute Intermittent Porphyria: Access document Other Acute Porphyrias: Access document Nonacute or Cutaneous Porphyrias: Access document Porphyria Cutanea Tarda or Cutaneous Hepatic Porphyria:

38. CCHS Clinical Digital Library porphyrias Patient/Family Resources. Spanish Miscellaneous porphyriasTable of contents. Introduction Access document; Porphyria Cutanea Tarda 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 Digestive Diseases: Table of contents A-Z List of Topics and Titles: List of documents Porphyria: Access document MedlinePlus 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 MedlinePlus 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 MedlinePlus (National Library of Medicine) Health Topics: Index Porphyria: List of documents Genetic Testing: List of documents Genetic Counseling: List of documents Genetic Disorders: List of documents YAHOO - Health:Diseases and Conditions:Porphyria Additional Porphyria resources ( These sites have not been reviewed.

39. Porphyrias Research Unit in the Department of Medicine at the University of Queensland,Australia. porphyrias; Patient Education Handout Publication Type; http://omni.ac.uk/browse/mesh/D011164.html

low graphics Porphyrias Pophyria : a patient's guide An online document providing information to anyone interested in or affected by the condition acute intermittent porphyria (AIP), a rare metabolic disorder which is an autosomal dominant deficiency in porphobilinogen deaminase. Written by Professor Michael Moore at the Porphria Research Unit in the Department of Medicine at the University of Queensland, Australia. Porphyrias Patient Education Handout [Publication Type] Last modified: 09 Sep 2005

40. UpToDate Understanding The Porphyrias INTRODUCTION — The porphyrias are genetic or acquired deficiencies in the activity In many of the porphyrias, the diagnosis can be confirmed by directly http://patients.uptodate.com/topic.asp?file=red_cell/19523

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