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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. DermIS / Main Menu / PeDOIA / Table Of Contents / 2 Genodermatoses / 2.8 The 
/ table of contents / 2 Genodermatoses / 2.8 The porphyrias. images for thediagnosis The porphyrias . Click to enlarge. Click to enlarge.
http://dermis.multimedica.de/doia/diagnose.asp?zugr=p&lang=e&diagnr=234&topic=t

62. * Porphyrias - (Disease): Definition
porphyrias Online Encyclopedia. The porphyrias are disorders in which thebody produces too much porphyrin and insufficient heme (an ironcontaining
http://en.mimi.hu/disease/porphyrias.html
Home Menu(0); pozicio('Disease','Porphyrias') MimiF1("Disease",0);
Porphyrias
See also: Blood Porphyria Pain Acute ... Genital CreateTd(0) Porphyrias
Definition
The porphyrias are disorders in which the body produces too much porphyrin and insufficient heme (an iron-containing non protein portion of the hemoglobin molecule) ... callurl('http://www.healthatoz.com/healthatoz/Atoz/ency/porphyrias.jsp');
CreateTd(1) Porphyrias Under normal circumstances, when heme concentrations are at an appropriate level, precursor production decreases. However, a glitch in the biosynthesis ... callurl('http://www.ahealthyme.com/topic/topic100587325');
CreateTd(2) The porphyrias are life-long intermittent illnesses, but with good long-term management, the affected person can expect long problem-free periods.
Complications:
Calling your health care provider ... callurl('http://www.kernanhospital.com/ency/article/001208trt.htm');
CreateTd(3) Porphyrias are characterized by three major findings: photo dermatitis ( causing ), neuropsychiatric complaints, and viscera l complaints (such as or cramp ing).

63. British National Formulary 9.8.2 Acute Porphyrias
The acute porphyrias (acute intermittent porphyria, variegate porphyria, Since acute porphyrias are hereditary, relatives of affected individuals should
http://www.bnf.org/bnf/bnf/current/noframes/5181.htm

64. Testing Information For Porphyrics
inherited porphyrias, fall into three clinical categories In the acutehepatic porphyrias, an acute porphyria may present
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Testing Information for Porphyrics
Laboratory Testing and the Porphyrias
Helpful Tidbits Regarding Porphyria Testing
Delta-ALA Porphyrin Urine Test
PBG Urine Testing ...
Return to Porphyria Home Page
TESTING FOR PORPHYRIA
Today there is increasing need for testing for porphyrin disorders:
The primary porphyrin disorders, which are considered the
inherited porphyrias, fall into three clinical categories:
[1] The acute attack forms of porphyria are usually
characterized by neurological and psychiatric manifestations.
[2] Those with cutaneous photosensitivity. [3] Those with both neurological symptoms and photosensitivity. When testing is begun to determine if a patient has a definitive diagnosis of porphyria tsting is usually begun with the basic 24 urine porphyrin test. Based upon the results of that test a variety of other porphyrin tests will follow. In the acute hepatic porphyrias which include: AIP [acute intermittent porphyria; VP [variegate porphyria]; and HCP [hereditary

65. THE PORPHYRIAS
The porphyrias. © 199598 Dr. Maged Nabih Kamel Boulos. All rights reserved.http//www.bitsmart.com/dermocad. Acronyms AIP, Acute Intermittent Porphyria;
http://members.tripod.com/~DermoCAD/porphyria.html
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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.

66. Vindex - Zoekresultaten Voor
Diagnosing the porphyrias. An article by Thomas G. DeLoughery, Associate Professor A personal site about this disorder, one of the family of porphyrias.
http://www.vindex.nl/dmoz.jspx?topic_id=Top/Health/Conditions_and_Diseases/Nutri

67. Articles - Porphyria
Reference. Kauppinen R. porphyrias. Lancet 2005;36524152. PMID 15652607.Loftus LS, Arnold WN. Vincent van Gogh s illness acute intermittent porphyria?
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Overview
In humans porphyrins are the main precursors of heme , an essential constituent of hemoglobin myoglobin , and cytochrome
Deficiency in the enzymes of the porphyrin pathway leads to insufficient production of heme. This is, however, not the main problem; most enzymes - even when less functional - have enough residual activity to assist in heme biosynthesis. The largest problem in these deficiencies is the accumulation of porphyrins, the heme precursors, which are toxic to tissue in high concentrations. The chemical properties of these intermediates determine in which tissue they accumulate, whether they are photosensitive , and how the compound is excreted (in the urine or feces
Subtypes
There are eight enzymes in the heme biosynthetic pathway: the first and the last three are in the mitochondria , while the other four are in the cytosol
  • δ-aminolevulinate (ALA) synthase δ-aminolevulinate (ALA) dehydratase hydroxymethylbilane (HMB) synthase uroporphyrinogen (URO) synthase uroporphyrinogen (URO) decarboxylase coproporphyrinogen (COPRO) oxidase protoporphyrinogen (PROTO) oxidase ferrochelastase
  • Hepatic porphyria
    The hepatic porphyrias include:
    • ALA dehydratase deficiency acute intermittent porphyria (AIP): a deficiency in HMB synthase hereditary coproporphyria (HCP): a deficiency in COPRO oxidase variegate porphyria (VP): a deficiency in PROTO oxidase porphyria cutanea tarda (PCT): a deficiency in URO decarboxylase

    68. Porphyria - Wikipedia, The Free Encyclopedia
    They are broadly classified as hepatic porphyrias or erythropoietic porphyrias, The erythropoietic porphyrias primarily affect the skin,
    http://en.wikipedia.org/wiki/Porphyria
    You did it! Over US$240,000 was donated in the 21 day fund drive. Thank you for your generosity! You can still donate or purchase Wikipedia merchandise
    Porphyria
    From Wikipedia, the free encyclopedia.
    This article is about the disease. For the poem by Robert Browning see Porphyria's Lover
    The porphyrias are inherited or acquired disorders of certain enzymes in the heme biosynthetic pathway (also called porphyrin pathway). They are broadly classified as hepatic porphyrias or erythropoietic porphyrias , based on the site of the overproduction and mainly accumulation of the porphyrins (or their chemical precursors).
    Contents
    • Overview Subtypes edit
      Overview
      In humans porphyrins are the main precursors of heme , an essential constituent of hemoglobin myoglobin , and cytochrome Deficiency in the enzymes of the porphyrin pathway leads to insufficient production of heme. This is, however, not the main problem; most enzymes - even when less functional - have enough residual activity to assist in heme biosynthesis. The largest problem in these deficiencies is the accumulation of porphyrins, the heme precursors, which are toxic to tissue in high concentrations. The chemical properties of these intermediates determine in which tissue they accumulate, whether they are photosensitive , and how the compound is excreted (in the urine or feces edit
      Subtypes
      There are eight enzymes in the heme biosynthetic pathway: the first and the last three are in the mitochondria , while the other four are in the

    69. Digital Termpapers: Term Papers On The Point Of View In Porphyrias Lover
    The Point of View in porphyrias Lover porphyrias Lover is an exhilarating lovestory given from a lunatics point of vie.
    http://www.digitaltermpapers.com/a7904.htm
    Term Papers Count: Home Join Login Sign Out ... Contact for:
    The Point Of View In Porphyrias Lover
    Term Paper Title The Point Of View In Porphyrias Lover # of Words # of Pages (250 words per page double spaced)
    The Point of View in "Porphyria's Lover"
    "Porphyria's Lover" is an exhilarating love story given from a lunatic's
    point of view. It is the story of a man who is so obsessed with Porphyria that
    he decides to keep her for himself. The only way he feels he can keep her,
    though, is by killing her. Robert Browning's poem depicts the separation of
    social classes and describes the "triumph" of one man over an unjust society.
    As is often the case in fiction, the speaker of "Porphyria's Lover" does not
    give accurate information in the story.
    The speaker is a deranged man who will stop at nothing to keep his dear
    Porphyria. Although the introduction refers to the weather, it also does an effective job in describing the speaker. In this case, it is nighttime, and the thunder is roaring. The speaker starts by saying: "The rain set early in tonight,/The sullen wind was soon awake,/ It tore the elm-tops down for spite,/

    70. ÿþ HTML HEAD META Http - Equiv = Content - Type
    The enzymatic defects that cause porphyrias may be either inherited or acquired Acute forms of the porphyrias may be life threatening and may be
    http://www.acpmedicine.com/abstracts/sam/med0905.htm
    The Porphyrias Abstract December 2003 Section 9 Metabolism V The Porphyrias Shigeru  Sassa, M.D., Ph.D. Associate Professor and Head, Laboratory of Biochemical Hematology, Rockefeller University Attallah  Kappas, M.D. Professor and Head, Laboratory of Pharmacology, Rockefeller University © 2004 WebMD Inc. All Rights Reserved. WebMD Professional Publishing, P.O. Box 1819, Danbury CT 06813 (800-545-0554 or 203-790-2087)

    71. Porphyrias -- James And Hift 85 (1): 143 -- British Journal Of Anaesthesia
    The conditions highlighted in boxes are the acute porphyrias. This fact isof great relevance in the development of the porphyrias, in which an increase
    http://bja.oxfordjournals.org/cgi/content/full/85/1/143
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    Request Permissions PubMed PubMed Citation Articles by James, M. F. M. Articles by Hift, R. J. British Journal of Anaesthesia, 2000, Vol. 85, No. 1
    The Board of Management and Trustees of the British Journal of Anaesthesia
    Porphyrias
    M. F. M. James and R. J. Hift Departments of Anaesthesia and Medicine, University of Cape Town and Groote Schuur Hospital, South Africa*Corresponding author: Department of Anaesthesia, University of Cape Town Medical School, Anzio Road, Observatory 7925, Cape, South Africa Keywords : complications, porphyria; biotransformation (drug); anaesthesia, evaluation, preoperative

    72. Porphyrias -- James And Hift 85 (1): 143 -- British Journal Of Anaesthesia
    porphyrias. MFM James and RJ Hift. Departments of Anaesthesia and Medicine,University of Cape Town and Groote Schuur Hospital, South Africa*Corresponding
    http://bja.oxfordjournals.org/cgi/content/extract/85/1/143
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    ISI Web of Science (17)
    Request Permissions PubMed PubMed Citation Articles by James, M. F. M. Articles by Hift, R. J. British Journal of Anaesthesia, 2000, Vol. 85, No. 1
    Porphyrias
    M. F. M. James and R. J. Hift Departments of Anaesthesia and Medicine, University of Cape Town and Groote Schuur Hospital, South Africa*Corresponding author: Department of Anaesthesia, University of Cape Town Medical School, Anzio Road, Observatory 7925, Cape, South Africa Keywords : complications, porphyria; biotransformation (drug); anaesthesia, evaluation, preoperative
    Introduction
    Br J Anaesth The porphyrins play a critical role in biology, being involved

    73. Rural Nurse Organization Clinic Digital Library
    Chapter 14 The porphyrias Table of contents. General Access document;Most Common porphyrias Access document; Less Common porphyrias Access document
    http://ruralnurseorganization-dl.slis.ua.edu/clinical/metabolism/inborn/porphyri
    Clinical Resources by Topic: Metabolic Disorders
    Porphyrias Clinical Resources
    Pediatrics Atlases Radiology Genetics ... Miscellaneous Resources See also:

    74. Porphyrias
    Question_set 168The QAD topic this week is porphyrias. QuestionA-Day for Monday, September 20, For those porphyrias which are characterized by acute attacks,
    http://www.health-nexus.com/porphyrias.htm
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    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

    75. OB-GYN-L Messages For June, 1998: Re: GYN: Porphyrias And OC's
    Hormonal oral contraceptives, urinary porphyrin excretion and porphyrias. Gross U,Honcamp M, Daume E, Frank M, Dusterberg B, Doss MO
    http://forums.obgyn.net/ob-gyn-l/OBGYNL.9806/0382.html
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    Re: GYN: Porphyrias and OC's
    From: art fougner, md evsono@pipeline.com
    Wed Jun 17 14:26:55 1998
    well - the literature yields its usual mixed bag of results. apparently we need to know more about which of the porphyrias you are dealing with. oral contraceptives either precipitate or prevent certain porphyrias. additionally, there is one case report of a woman's developing porphyria on withdrawal of oc's. go figure. see below: J Am Acad Dermatol 1994 Aug;31(2 Pt 2):390-392 Porphyria cutanea tarda: pregnancy versus estrogen effect. Urbanek RW, Cohen DJ Department of Medicine, Staten Island University Hospital, NY 10305. We describe the worsening of porphyria cutanea tarda in a young woman while she was taking oral contraceptives. However, she did not have an exacerbation during two pregnancies. We conclude that estrogens produced during pregnancy do not exert the same effect as orally administered medications that contain estrogen. The pronounced effect of oral ethinyl estradiol on the liver may be attributed to its first-pass effect on that organ. Horm Metab Res 1995 Aug;27(8):379-383

    76. OB-GYN-L Messages For June, 1998: Re: GYN: Porphyrias And OC's
    we need to know more about which of the porphyrias you are dealing with. oral contraceptives either precipitate or prevent certain porphyrias.
    http://forums.obgyn.net/ob-gyn-l/OBGYNL.9806/0468.html
    -VISIT OUR OTHER FORUMS- OB-GYN-L Ultrasound Ultrasound History Physicians-in-Training Tech Talk OBSTET-L (portuguese) OBGIN-L (spanish) Nursing
    Re: GYN: Porphyrias and OC's
    From: Robert Brenner robbrenner@earthlink.net
    Sun Jun 21 10:44:51 1998
    At 02:28 PM 6/17/98 -0500, you wrote:
    Now how did I miss these articles? Robert Brenner MD FACOG Pikesville MD robbrenner@earthlink.net http://docs.obgyn.net/drbrenner use when must restrict search to only the ob-gyn-l forum... Enter search keywords: Returns per screen: Require all keywords: Return to Mail a New Message to the Forum: ob-gyn-l@obgyn.net
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    77. The Porphyrias.
    The porphyrias.The heterogeneous group of diseases called the porphyrias may In the acute porphyrias; acute intermittent porphyria, urophorphyrinogen 1
    http://www.pdg.cnb.uam.es/UniPub/iHOP/gp/3412366.html
    The porphyrias .The heterogeneous group of diseases called the porphyrias may all be characterised by derangement of specific stages in the haem biosynthetic pathway.
    In the acute porphyrias ; acute intermittent porphyria , urophorphyrinogen 1 synthase, hereditary coproporphyria, coproporphyrinogen oxidase and variegate porphyria , ferrochelatase or protoporphyrinogen oxidase , are the enzymes affected, whilst in the non acute porphyrias , cutaneous hepatic porphyria , uroporphyrinogen decarboxylase, congenital porphyria , uroporphyrinogen cosynthase; and erythropoietic protoporphyria; ferrochelatase are the enzymes affected.
    In each of the porphyrias , the activity of the initial and rate controlling enzyme of the pathway, delta-aminolaevulinic acid synthase is raised which constitutes the principal control point of the pathway.
    Secondary control in each of these diseases lies at the leve of uroporphyrinogen 1 synthase.
    As a consequence of this secondary control, there is excessive excretion of the porphyrin precursors delta-aminolaevulinic acid and porphobilinogen in the acute porphyrias and excessive excretion of porphyrins leading to solar photosensitivity in the non-acute porphyrias and in variegate and hereditary coproporphyria.

    78. Amiodarone Is A Pharmacologically Safe Drug For Porphyrias.
    as well as skin photosensitization, as seen in porphyrias. Clinical signs inporphyrias might be triggered by the socalled porphyrinogenic drugs.
    http://www.pdg.cnb.uam.es/UniPub/iHOP/gp/1838288.html
    Amiodarone is a pharmacologically safe drug for porphyrias Amiodarone (AD) is an effective antidysrythmic drug, however, there can be serious side effects, such as hepatic and neurological alterations, as well as skin photosensitization, as seen in porphyrias
    Clinical signs in porphyrias might be triggered by the so-called porphyrinogenic drugs.
    Without sound basis, Amiodarone has been classified as an unsafe drug for porphyric patients.
    The aim of this work has been to study the effect of AD, both in vivo and in vitro, on heme metabolism.
    In the in vivo assays, the activities of 5-aminolevulinate synthetase (ALA-S), ALA dehydratase (ALA-D), porphobilinogenase (PBGase) and PBG-deaminase ( PBG-D ) in blood, liver , and kidney; hepatic and fecal porphyrins , urinary ALA, PBG and porphyrins in male mice strain CF1 treated with AD (100 mg i.p. daily) for 1 week and 1 month, were measured.
    No significanat differences were found for any of these parameters in the AD treated animals as compared to controls.
    In the in vitro experiments human blood, and mice blood

    79. Nutritional And Metabolic Diseases
    Peroxisomal Disorders Phenylketonurias - Phenylketonurias - Phenylketonurias -porphyrias - Progeria - Pseudo-Gaucher Disease (not on MeSH) - Refsum
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    Diseases and Disorders Links pertaining to Nutritional and Metabolic Diseases Alert! Patients and laypersons looking for guidance among the target sources of this collection of links are strongly advised to review the information retrieved with their professional health care provider. Start Page Contents: Acid-Base Imbalance Acidosis Alkalosis Alkaptonuria ... Zellweger Syndrome
    Nutritional and Metabolic Diseases FDA Center for - (US) International Union of Nutritional Sciences - (AU) The USDA Nutrient Database for Standard Reference - (US) NATS - Nutritional Analysis Tool and System About some Diagnostic Tests Used in Evaluation of Malabsorption www.FoodSafety.gov Food Safety including a list of Organisms of concern - N Carolina Coop. Ext. Serv. (US) About Food Irradiation - BFE (DE) Facts about Food Irradiation - IAEA (AT) The British Nutrition Foundation Int'l Food Information Council , including a page on Food Additives , and Food Color Facts , and a

    80. Dermatology Times : Newest Approaches For Treating Pediatric Porphyrias Discusse
    Dermatology Times Newest Approaches for Treating Pediatric porphyrias Discussed @HighBeam Research.
    http://static.highbeam.com/d/dermatologytimes/april011990/newestapproachesfortre
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      Start D Dermatology Times April 01, 1990 ... Newest Approaches for Treating Pediatric Porphyrias Discussed
      Newest Approaches for Treating Pediatric Porphyrias Discussed
      Dermatology Times; April 01, 1990;
      Dermatology Times
      April 01, 1990
      MILAN, ITALY - Promising new approaches to treating a variety of pediatric porphyrias were detailed in two presentations at the Fifth International Congress of Pediatric Dermatology here.
      In one study involving two brothers (2 and 10 years old) with erythropoietic protoporphyria, treatment with the drug S-adenosyl-L-methionine produced significant improvement in clinical and biochemical parameters, said Dr. A-M Pierini, department of pediatric dermatology, Hospital de Pediatria "J. P. Garrahan" and Center for Investigation of Porphyrias and Porphyrins, Buenos Aires, Argentina.
      When the two brothers were treated with one to three courses - 12 to 15 mg/kg/day for 20 days - of S-adenosyl-L-methionine, clinical improvement was noticeable at the end of one course. Blood levels of protoporphyrin returned to normal (150 ng/ml red blood cells) from their respective pretreatment levels of 199 and 291 ng/100 ml red blood cells. The porphyrin plasma index followed a

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