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Porphyrias:     more books (87)

Porphyria by Tammy Evans, 1997-05-15 Porphyria: The Unknown Disease by Diana Deats-O'Reilly, 1998-03-02 The Official Patient's Sourcebook on Porphyria: A Revised and Updated Directory for the Internet Age by Icon Health Publications, 2002-08 Porphyria: A Lyon's Share of Trouble by Desiree Lyon Howe, 2004-08 Relationships in Dermatology: The Skin and Mouth, Eye, Sarcoidosis, Porphyria (New Clinical Applications: Dermatology) Porphyria's Lover by Maggie Power, 1996-11-04 Porphyria: Webster's Timeline History, 1788 - 2007 by Icon Group International, 2009-07-08 Porphyria - A Medical Dictionary, Bibliography, and Annotated Research Guide to Internet References by ICON Health Publications, 2004-04-27 The Porphyrias, (American Lecture Series, Publication No. 842, A Monograph in American Lectures in Living Chemistry) by J. Lyndal York, 1972 Porphyrins and Porphyria (Colloques INSERM) Chemical Porphyria in Man Porphyria - A Royal Malady : Articles Published in or Comissioned by the British Medical Journal by Unnamed Unnamed, 1968 Chester Porphyria: The Story of Dobson's Complaint by Giles R. Youngs, 1998-04-01 Diagnosis and therapy of porphyrias and lead intoxication

lists with details

1. Porphyrias: Clinical Manifestations, Diagnosis And Treatment MEDSTUDENTS-METABOL
   Clinical manifestations, diagnosis, etiology and treatment of six types ofporphyrias are rapidly discussed. Pathogenesis constitutes a complex and wide  
   http://www.medstudents.com.br/metdis/metdis3.htm
   
   Extractions: Medstudents' Homepage Porphyrias are inherited or adquired diseases that result from an abnormal metabolism of heme biosynthesis pathway. The main causes are enzyme deficiencies that lead to heme pathways intermediates accumulation. Heme biosynthesis occurs almost entirely in the liver and bone marrow and is composed by eight reactions, each one of these being catalyzed by an especific enzyme. Therefore, partial or complete deficiency of one of these enzymes result in diferents clinical pictures. We will focus our discussion mainly on clinical grounds. There are two major groups of porhyrias according to the site where dysfunction occurs, i.e, hepatic and erythropoietic porphyrias. Among erythropoietic types, there are: Congenital Erythropoietic Porphyria and Protoporphyria. Hepatic porphyrias are: Intermitent Acute Porphyria, Hereditary Coproporphyria, Variegate Porphyria and Porphyria Cutanea Tarda. It is important to recognize that the three most common types of porphyrias are Intermitent Acute Porphyria (IAP), Porphyria Cutanea Tarda (PCT) and Protoporphyria (PP). Intermitent Acute Porphyria: The majority of patients are assymptomatic. Signs and symptoms rarely start before puberty. The initial and commonest manifestation is abdominal pain, which can be diffuse or localized, colicky. The clinical picture may mimic an accute inflammatory abdominal disease. It is caused by an altered autonomic activity. Peripheral neuropathy is primarily motor, beginning in proximal muscles, more often in the arms. Cranial nerves manifestations my lead to optic nerve atrophy. CNS abnormalities include seizures, delirium and coma. Hepatic dysfunctions are common. Precipitating factors are drugs as barbiturates, sulfonamides, estrogens and dietary restriction of carbohydrates. Diagnosis is made on clinical grounds and increased erythrocytic and urinary porphobilinogen and aminolevolinic acid (ALA) levels (metabolic intermediates of heme).

2. OHSU Division Of Hematology & Medical Oncology
   An article by Thomas G. DeLoughery, Associate Professor of Medicine.  
   http://www.ohsu.edu/som-hemonc/handouts/deloughery/por.shtml
   
   Extractions: "The disease of cancer will be banished from life by calm, unhurrying, persistent men and women, working with every shiver of feeling controlled and suppressed in hospitals and laboratories, and the motive that will conquer cancer will not be pity or horror; it will be curiosity to know how and why."

3. Porphyria Page
   A patient guide for all people with this disorder. Compiled by Professor Michael Moore.  
   http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

4. Porphyrias: Clinical Manifestations, Diagnosis And Treatment MEDSTUDENTS-METABOL
   A short review of hemochromatosis, giving atention mainly to its etiology, clinicalmanifestations, treatment and prognosis.Importance about early diagnosis  
   http://www.medstudents.com.br/metdis/metdis4.htm
   
   Extractions: Medstudents' Homepage Hemochromatosis is a disease due to increased iron absorption that leads to accumulation of iron in diverse tissues. It can be hereditary (primary hemochromatosis) or secondary to chronic anemias as ß-thalassemias (ineffective erythropoiesis leading to increased erythrocitic destruction) or defects in hemoglobin synthesis ( as in porphyria cutanea tarda) ;multiple transfusion therapy ;or others less common conditions. Iron absorption from the gut occurs normally in a rate of 1 mg per day. In hemochromatosis this absortion reaches the rate of 4 to 5 mg/d and there is a progressive accumulation to 15 to 40 grams of body iron (normal ~ 2 to 3 g). Thus, the disease develops in men above age 20 and postmenopausal women, due to stopping in iron loss in menses. The iron accumulates in every tissue of the body, but main clinical manifestations are secondary to iron deposition in: Liver, thyroid, hypothalamus, heart, pancreas, gonads and joints. Many patients are asymptomatic. Common symptoms are fatigue, arthalgias, impotence, amenorrhea and palpitations. The most characteristical clinical manifestation occur in the liver because the portal circulation filters the iron initially absorbed fom the gut. Usually patients develop cirrhosis. Heart manifestations include arrythmias and disturbances of conduction. Skin adquires an diffuse hyperpigmentation mainly in scars, sun exposed and unexposed areas and genitalia. Arthropathy occurs in wrists, hips, knees and ankles. To distinguish this arthopathy from others, searching for initial involvement of second and third metacarpophalangeal joints is helpful. Hypogonadism hipogonadothrofic is secondary to iron deposition in hypophisary cells and leads to loss of libido, amenorrhea, impotence and body hair losing. Diabetes Mellitus results from pancreatic accomitment and the signs and symptons, as the treatment, do not differ from conventional diabetes.

5. Porphyrias Clinical Manifestations, Diagnosis And Treatment
   Clinical manifestations, diagnosis, etiology and treatment of six types of porphyrias are discussed.  
   http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

6. THE PORPHYRIAS
   Recognize the common porphyrias. Suspect porphyria when appropriate, order the right The porphyrias are a family of diseases caused by errors of heme  
   http://www.pathguy.com/lectures/porphyri.htm
   
   Extractions: Cyberfriends: The help you're looking for is probably here. Welcome to Ed's Pathology Notes, placed here originally for the convenience of medical students at my school. You need to check the accuracy of any information, from any source, against other credible sources. I cannot diagnose or treat over the web, I cannot comment on the health care you have already received, and these notes cannot substitute for your own doctor's care. I am good at helping people find resources and answers. If you need me, send me an E-mail at scalpel_blade@yahoo.com Your confidentiality is completely respected. DoctorGeorge.com is a larger, full-time service. There is also a fee site at myphysicians.com , and another at www.afraidtoask.com Translate this page automatically With one of four large boxes of "Pathguy" replies. I'm still doing my best to answer everybody. Sometimes I get backlogged, sometimes my E-mail crashes, and sometimes my literature search software crashes. If you've not heard from me in a week, post me again. I send my most challenging questions to the medical student pathology interest group, minus the name, but with your E-mail where you can receive a reply. Slice of Life videodisk. No medical student should be without access to this wonderful resource. Someday you may be able to access these pictures directly from this page.

7. The Merck Manual Endocrine And Metabolic Disorders
   Chapters on a variety of endocrine problems such as hyperlipidemia, polyglandular deficiency syndromes, the porphyrias, amyloidosis, and others.  
   http://www.merck.com/mrkshared/mmanual/section2/sec2.jsp

8. MEDIC CLINICAL HANDBOOK
   porphyrias (in development)  
   http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

9. MEDIC: CLINICAL HANDBOOK
   porphyrias are a group of inherited disorders of heme synthesis. Only one normalgene is present to produce a key functional enzyme in heme synthesis.  
   http://medic.med.uth.tmc.edu/path/00000884.htm
   
   Extractions: Porphyrias are a group of inherited disorders of heme synthesis. Only one normal gene is present to produce a key functional enzyme in heme synthesis. This results in at least a 50% of the normal enzyme activity. This reduced level of enzyme results in a build up of precursors behind the deficient enzyme which then accumulate in body fluids and tissues. The clinical manifestations, cutaneous lesions, neurologic dysfunction, and hepatic disease, characteristic to each type of porphyria are due to the toxic nature of these precursor compounds. delta - Aminolevulinic Acid (ALA) dehydrase deficiency Acute Intermittent porphyria Congenital erythropoietic porphyria Porphyria cutanea tarda Hepatoerythropoietic porphyria ... Protoporphyria Laboratory Diagnosis Summary

10. EPP Frames
    Short for Erythropoetic ProtoPorhyria, which is a rare metabolic disease. A personal site about this disorder, one of the family of porphyrias.  
    http://members.tripod.com/~Alzuko/epp.htm

11. Home - Canadian Porphyria Foundation
    Mission statement, objectives of the organization, newsletters and safe and unsafe drugs. Also A Guide to Porphyria a complete booklet of  
    http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

12. THE PORPHYRIAS
    The porphyrias. © Dr. Maged N. Kamel Boulos MediCAD Multimedia, 1995-98.All rights reserved. http//www.medicad.com. Acronyms AIP, Acute Intermittent  
    http://www.geocities.com/stantonios/mc/porphyria.html
    
    Extractions: The Porphyrias http://www.medicad.com 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. b -Carotene, a known quencher of free radicals and singlet oxygen, has a photoprotective effect in porphyria.

13. Porphyria Pediatric Oncall
    Information on porphyrias including the various types and its management.  
    http://www.pediatriconcall.com/fordoctor/diseasesandcondition/porphyrias.asp
    
    Extractions: A: Porphyrias are a rare disorder. They occur due to specific abnormality of various enzymes in the biosynthetic pathway of heme production. As a result, excess amounts of porphyrias and their precursors accumulate in the body causing generalized clinical abnormalities. Q: What are the various types of porphyrias ?

14. Porphyria
    porphyrias general mechanism Overproduction syndromes Acute porphyrias General. Syndromes Acute intermittent porphyria  
    http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

15. Cheryl Ann's Porphyria Links
    porphyrias Introduction, Types and Laboratory Diagnosis Summary Porphyrins andporphyrias 1997 Meeting Page Rare Diseases Clinical Trials Pertaining to  
    http://www.geocities.com/porphyria2000/porphs.htm

16. Acute Intermittent Porphyria Forum
    fluctuations in female sex hormones may also precipitate acute porphyrias infections starvation  
    http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

17. THE MERCK MANUAL, Sec. 2, Ch. 14, The Porphyrias
    The porphyrias A group of disorders cause by deficiencies of enzymes of the porphyrias and related disorders are associated with deficiencies of the  
    http://www.merck.com/mrkshared/mmanual/section2/chapter14/14a.jsp

18. Porphyria
    Erythropoietic porphyrias Hepatic porphyrias Erythropoietic porphyrias Hepatic porphyrias  
    http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

19. THE MERCK MANUAL--SECOND HOME EDITION, Introduction In Ch. 160, Porphyrias
    porphyrias are a group of disorders caused by deficiencies of enzymes involved porphyrias are a number of different diseases, each caused by a specific  
    http://www.merck.com/mmhe/sec12/ch160/ch160a.html
    
    Extractions: var externalLinkWarning = "The link you have selected will take you to a site outside Merck and The Merck Manuals.*n*nThe Merck Manuals do not review or control the content of any non-Merck site. The Merck Manuals do not endorse and are not responsible for the accuracy, content, practices, or standards of any non-Merck sources."; Search The Second Home Edition , Online Version Search Index A B C D ... Z Sections Accidents and Injuries Blood Disorders Bone, Joint, and Muscle Disorders Brain, Spinal Cord, and Nerve Disorders ... Women's Health Issues Resources Anatomical Drawings Multimedia Pronunciations Weights and Measures ... , Online Version Section Disorders of Nutrition and Metabolism Chapter Porphyrias Topics Introduction Acute Intermittent Porphyria Erythropoietic Protoporphyria Porphyria Cutanea Tarda Introduction Buy The Book Print This Topic Email This Topic Pronunciations acute intermittent porphyria cutaneous erythropoietic protoporphyria hemoglobin ... porphyria Porphyrias are a group of disorders caused by deficiencies of enzymes involved in the production of heme. Heme is produced in the bone marrow and liver through a complex process regulated by eight different enzymes. As this production process progresses, several different intermediate compounds (heme precursors) are created and modified. If there is a deficiency in one of the enzymes that are essential for heme production, certain heme precursors may accumulate in tissues (especially in the bone marrow or liver), appear in excess in the blood, and get excreted in the urine or stool. The specific precursors that accumulate depend on which enzyme is deficient. One group of heme precursors is called the porphyrins.

20. Centre Fran Ais Des Porphyries
    Centre de diagnostic, de traitement, de pr vention et de recherche sur les Porphyries humaines  
    http://tmsyn.wc.ask.com/r?t=an&s=hb&uid=24312681243126812&sid=343126

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