81. Porphyrias (cme),Handa F: IJDVL IJDVL is an peerreviewed biomedical periodical of Indian Association ofDermatologists, Venereologists and Leprologists. http://www.ijdvl.com/article.asp?issn=0378-6323;year=1984;volume=50;issue=2;spag

82. BioMed Central | Abstract | The Porphyrias The porphyrias are a diverse group of metabolic diseases. Major manifestationsare episodic neurovisceral attacks of pain or other neurologic features, http://www.biomedcentral.com/1092-8472/3/487/abstract

home journals A-Z subject areas advanced search ... Issue 6 Viewing options Abstract Full text PDF Associated material: PubMed record Related literature: Articles citing this article on Google Scholar Other articles by authors Bonkovsky HL ... Similar articles (PubMed) Tools: E-mail to a friend Download references Opinion The Porphyrias Herbert L Bonkovsky MD and Graham F Barnard MD PhD Departments of Medicine and Biochemistry and Molecular Biology Division of Digestive Disease and Nutrition and The Center for Study of Disorders of Iron and Porphyria Metabolism, 55 Lake Avenue North Room S6-737, University of Massachusetts Medical School, Worcester, 01655-0001, MA Departments of Medicine and Biochemistry and Molecular Biology Division of Digestive Disease and Nutrition and The Center for Study of Disorders of Iron and Porphyria Metabolism, 55 Lake Avenue North Room S6-737, University of Massachusetts Medical School, Worcester, 01655-0001, MA Current Treatment Options in Gastroenterology Published Abstract The porphyrias are a diverse group of metabolic diseases. Major manifestations are episodic neurovisceral attacks of pain or other neurologic features, and/or dermatologic abnormalities. It is essential that a clear diagnosis be established prior to planning management. In our experience, most patients referred with a presumptive diagnosis of 'porphyria' do not have true porphyria at all, but rather have syndromes of other etiologies associated with mild, nonspecific increases in urinary porphyrin excretion (secondary porphyrinurias).

83. BioMed Central | Full Text | The Porphyrias To obtain access to Current Treatment Options in Gastroenterology through yourinstitution use the options below. If you would like information about a http://www.biomedcentral.com/1092-8472/3/487

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84. Recommendations For The Diagnosis And Treatment Of The Acute Porphyrias -- Ander The acute porphyrias, 4 inherited disorders of heme biosynthesis, causelifethreatening attacks of neurovisceral symptoms that mimic many other acute http://www.annals.org/cgi/content/abstract/142/6/439

Search Annals: Advanced search Home Current Issue Past Issues ... ACP Online Article Correction Table of Contents Full Text of this article PDF of this article (PDFs free after 6 months) Figures/Tables List Services Send comment/rapid response letter Published comments/rapid response letters Notify a friend about this article Alert me when this article is cited ... ACP Search PubMed Articles in PubMed by Author: Anderson, K. E. Desnick, R. J. Related Articles in PubMed PubMed Citation ... PubMed REVIEW Recommendations for the Diagnosis and Treatment of the Acute Porphyrias Karl E. Anderson, MD Joseph R. Bloomer, MD Herbert L. Bonkovsky, MD James P. Kushner, MD ... Robert J. Desnick, PhD, MD The acute porphyrias, 4 inherited disorders of heme biosynthesis, cause life-threatening attacks of neurovisceral symptoms that mimic many other acute medical and psychiatric conditions. Lack of clinical recognition often delays effective treatment, and inappropriate diagnostic tests may lead to misdiagnosis and inappropriate treatment. We review the clinical manifestations, pathophysiology, and genetics of the acute porphyrias and provide

85. Entrez PubMed The porphyrias. Sassa S. Laboratory of Biochemical Hematology, The RockefellerUniversity, Diagnosis, Differential; Humans; porphyrias/classification* http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=1

86. Entrez PubMed The human hereditary hepatic porphyrias are diseases due to marked deficienciesof enzymes porphyrias can be classified as either hepatic or erythroid, http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=1

87. Diagnosing Acute Porphyrias -- Sandberg And Elder 50 (5): 803 -- Clinical Chemis Four porphyrias can present with lifethreatening acute neurovisceral attacks In all of the acute porphyrias except VP and in porphyria cutanea tarda a http://www.clinchem.org/cgi/content/full/50/5/803?etoc

88. Diagnosing Acute Porphyrias -- Sandberg And Elder 50 (5): 803 -- Clinical Chemis porphyrias are rare diseases, and most clinicians will see only a few cases during Four porphyrias can present with lifethreatening acute neurovisceral http://www.clinchem.org/cgi/content/full/50/5/803

HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: Clinical Chemistry 50: 803-805, 2004; 10.1373/clinchem.2003.027623 This Article Extract Full Text (PDF) Submit a response ... Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in ISI Web of Science Similar articles in PubMed ... Download to citation manager PubMed PubMed Citation Articles by Sandberg, S. Articles by Elder, G. H. Related Collections General Clinical Chemistry Pediatric Clinical Chemistry Evidence Based Laboratory Medicine and Test Utilization Hematology ... Endocrinology and Metabolism Clinical Chemistry. Editorials Diagnosing Acute Porphyrias Sverre Sandberg ,a and George H. Elder Norwegian Porphyria Centre (NAPOS), Laboratory of Clinical Biochemistry, Haukeland University Hospital, Bergen, Norway Department of Medical Biochemistry and Immunology, University of Wales College of Medicine, Heath Park, Cardiff, United Kingdom a Address correspondence to this author at: Norwegian Porphyria Centre, Laboratory of Clinical Biochemistry, Haukeland University Hospital, N-5021 Bergen, Norway. E-mail sverre.

89. Porphyrias URL http//www.lifesteps.com/gm/Atoz/ency/porphyrias.jsp Gordon, Neal. The Acute porphyrias. Brain Development 21 (September 1999) 37377. http://www.lifesteps.com/gm/Atoz/ency/porphyrias_pr.jsp

90. Porphyrins And Porphyrias, Prague 2003 Porphyrins and porphyrias Prague, September 2124, 2003. Author Index. Full versionin PDF file. Aarsand AK 1,1, 26 Abitbol M 15, 19 Afonso SG 16 Akagi R 7 http://www.biomed.cas.cz/physiolres/2003/52_1s.htm

Physiol. Res. 52: Porphyrins and Porphyrias Prague, September 21-24, 2003 Author Index: Full version in PDF file Aarsand AK 1,1, 26 Abitbol M 15, 19 Afonso SG 16 Akagi R 7 Anderson KE 1, 14 Andersson C 10 Andersson DEH 10, 22, 23 Andrieu V 15 Andrioletti B 2, 14 Baars-Heesakkers E 7 Badminton M 6 Badminton MN 1, 17, 17 Balogh K 3. 21 Barbieri B 15 Barbieri L 2, 4. 9 Barile S 4, 9, 15 Baron JM 21 Bartz C 2 Batlle A 3, 3, 4, 16, 16, 17, 18, 18, 19, 22 Beaumont C 15 Berkes E 3, 21 Bernátková M 2, 14 Biolcati G 2, 2, 4, 4, 9, 15 Bjernevik K 26 Blázovics A 26.

91. NeLH - Skin Conditions You searched for the category Conditions Metabolic nutritional conditions porphyrias TOTAL RECORDS MATCHED 7 http://libraries.nelh.nhs.uk/skin/searchResponse.asp?categoryID=8376

92. Safety Of Anticonvulsants In Hepatic Porphyrias -- Reynolds And Miska 31 (4): 48 Safety of anticonvulsants in hepatic porphyrias. NC Reynolds Jr and RM Miska.Because acute attacks of porphyria may be precipitated by anticonvulsants, http://www.neurology.org/cgi/content/abstract/31/4/480

HOME HELP FEEDBACK SUBSCRIPTIONS ... TABLE OF CONTENTS QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: This Article Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted ... Alert me if a correction is posted Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Cited by other online articles PubMed PubMed Citation Articles by Reynolds, N. C., Jr Articles by Miska, R. M. ARTICLES Safety of anticonvulsants in hepatic porphyrias NC Reynolds Jr and RM Miska Because acute attacks of porphyria may be precipitated by anticonvulsants, a therapeutic dilemma arises when seizures complicate hepatic porphyria. The list of unsafe agents includes barbiturates, primidone, phenytoin, mephenytoin, ethotoin, ethosuximide, methsuximide, phensuximide, and trimethadione. Agents are considered unsafe if they induce experimental porphyria in animals, and short trials in patients are unreliable for

93. Porphyria Cuatanea Tarda porphyrias are a group of diseases which are all deficiencies of any of the eightenzymes The most common porphyrias are porphyria cutanea tarda (PCT), http://www.zevils.com/~matthewg/porphyria.html

Porphyria Cutanea Tarda - A Report by Matthew Sachs Porphyria Overview Porphyrias are a group of diseases which are all deficiencies of any of the eight enzymes involved in heme synthesis. One of the major causes of symptoms in patients with porphyria is the accumulation of heme precursors. To make heme, the body needs some raw materials in addition to the eight enzymes. Heme procursors collect mainly in the marrow and liver. The main heme precursors that cause problems in porphyria are toxic metabolites: delta-aminolevulinic acid and porphobilinogen, which are neurotoxins, and porphyrins. These precursors are transferred from the tissues where they accumulate into the blood plasma and from there they are excreted in urine and stools. Accumulation of these precursors can cause photosensitivity and nerve damage, even paralysis. The most common porphyrias are porphyria cutanea tarda (PCT), acute intermittent porphyria , and eryhtropoietic protoporphyria . These are veru different diseases, with distinct symptoms, tests needed for proper diagnosis, and treatments. Additionally, they all share some symptoms with rarer porphyrias. Thus, porphyria is often misdiagnosed. Test results from patients with porphyria will be markedly abnormal, but one needs to select and interpret tests with porphyria in mind. All porphyrias except for one are hereditary. PCT is not inherited directly. However, suspectibility to PCT is often inherited, in which case it is called familial (type II) PCT.

94. Examples Of Porphyria Examples of Porphyria. Two of the several types of porphyria will serve toillustrate some of the biochemical issues involved. http://medlib.med.utah.edu/NetBiochem/hi6c.htm

Examples of Porphyria Two of the several types of porphyria will serve to illustrate some of the biochemical issues involved. Acute intermittent porphyria is a defect of hepatic uroporphyrinogen I synthase activity. porphobilinogen (the substrate) accumulates, and is excreted in the urine. Note: analysis of the urine for porphobilinogen is relatively simple. Heme synthesis is reduced. As a result, there is no feedback inhibition of ALA synthase, nor is there repression of synthesis of the enzyme protein. ALA synthase activity therefore increases. Insufficient 5-alpha steroid reductase activity shunts steroids into the 5-beta reductase pathway, whose products further stimulate ALA synthase. There are neurological symptoms, including agitation, which cannot be explained. Administration of barbiturates to sedate an agitated patient would be counterproductive, as it would further activate ALA synthase, and result in more rapid clinical deterioration. Hereditary coproporphyria (pronounce) is a defect of hepatic coproporphyrinogen oxidase. Large amounts of coproporphyrin III are excreted in the feces.

95. OHSU Division Of Hematology & Medical Oncology Details about many blood products and the risks of transfusion. http://www.ohsu.edu/hemonc/

Contact Information: 3181 SW Sam Jackson Park Rd Portland, OR 97239-3098 Phone: (503) 494-6594 Email: hemonc@ohsu.edu Find a Doctor FAQ Sheet Construction Updates About our Division Clinical Trials Our Faculty About our Clinic ... Prostate Cancer Program Healthcare Professionals Physician Career Opportunities Fellowship Program Faculty Publications and Research Interests Dr. Thomas Deloughery's Famous Handouts ... Dr. Skach's Lab General Information Hem Onc in the News Helpful Links Lance Armstrong Foundation OHSU Hem Onc Wears Yellow News Upcoming Events ASH 2004 Faculty Spotlight Craig Nichols, M.D. Division Chief "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." H.G. Wells, 1901

96. öÉ×ÏÊ öÕÒÎÁÌ Cable-City & NETRONIK The summary for this Russian page contains characters that cannot be correctly displayed in this language/character set. http://lj.inetik.ru/user/1587

WinDiGo IMPerator KOBA AfinaPallada ... RID ÷ÓÅ ÚÁÐÉÓÉ ÐÏÌØÚÏ×ÁÔÅÌÑ Porphyrias: Aug 2005 Jul 2005 AfinaPallada WinDiGo Magnificent tituu ... WIILY

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