41. Archives Of Pathology And Laboratory Medicine 127:e325-e328, 2004 e325–e328. propionic acidemia A Neuropathology Case Report and Review of PriorCases. Brady Feliz, MD, a David R. Witt, MD, a and Brent T. Harris, MD, PhD a http://arpa.allenpress.com/arpaonline/?request=get-abstract&doi=10.1043/1543-216

42. Propionic Acidemia propionic acidemia is a rare inherited genetic disorder of propionate metabolismcharacterized by greatly increased concentrations of propionate and related http://www.shsna.com/html/propionic.htm

Propionic Acidemia is a rare inherited genetic disorder of propionate metabolism characterized by greatly increased concentrations of propionate and related metabolites in blood and urine. The disorder is caused by a defective enzyme responsible for the metabolism of methionine, threonine, valine, and isoleucine as well as odd chain fats leading to elevated blood and tissue concentrations of related toxic organic acids. Clinical symptoms of propionic acidemia include metabolic acidosis, hyperammonemia, vomiting, failure-to-thrive, lethargy, respiratory distress, and coma. Untreated, a child may suffer from irreversible brain damage and death. Symptoms may be prevented and the disorder may be managed by diet. The treatment of propionic acidemia consists of strict dietary control of methionine, threonine, valine, and isoleucine intake. High protein foods are restricted in this diet (i.e., meat and meat products, milk and milk products, legumes, nuts, etc.). Low protein medical foods, minimal quantities of low-protein grains, fruits, and vegetables in combination with fat and sugar are permitted. Without additional calories and amino acids (excluding the above mentioned), a propionic acidemia patient would experience malnutrition. Thus, the majority of the patient's daily intake must come from a medical food free of methionine, threonine, valine, and isoleucine, but complete with vitamins, minerals, trace elements and essential and nonessential amino acids.

43. Taste And Choice Are The Key To Diet Compliance. SHS Offers A Wide Formulas are available for Phenylketonuria, Maple Syrup Urine Disease, Tyrosinemia(Types I II), Homocystinuria, propionic acidemia and Methylmalonic Acidemia http://www.shsna.com/html/metabolic.htm

Indications Product Recommended Age Group Description Phenylketonuria (PKU) XPhe Analog Infants birth to 1 year phenylalanine-free XP Maxamaid Children 1 to 8 years XP Maxamum Children 8 years to adults Periflex Children over 1 year Phlexy-10 System Children and adults Maple Syrup Urine Disease (MSUD) MSUD Analog Infants birth to 1 year valine-, leucine-, isoleucine-free MSUD Maxamaid Children 1 to 8 years MSUD Maxamum Children 8 years to adult Acerflex Children over 1 year Tyrosinemia Type I XPhe, XTyr Analog Infants birth to 1 year phenylalanine-, tyrosine-free XPHEN, TYR Maxamaid Children 1 to 8 years XPTM Analog Infants birth to 1 year phenylalanine-, tyrosine-, methionine-free Hypermethioninemia Homocystinuria -Vitamin B non-responsive due to Cystathionine B-Synthase Deficiency XMet Analog Infants birth to 1 year methionine-free XMET Maxamaid Children 1 to 8 years XMET Maxamum Children 8 years to adult Propionic Acidemia XMTVI Analog Infants birth to 1 year methionine-, threonine-, valine-free, isoleucine low XMTVI Maxamaid Children 1 to 8 years XMTVI Maxamum Children 8 years to adult Methylmalonic Acidemia Vitamin B12 non-responsive XMTVI Analog Infants birth to 1 year methionine-, threonine-, valine-free, isoleucine low

44. Health - Conditions And Diseases - Genetic Disorders - Propionic Top Health Conditions and Diseases Genetic Disorders PropionicAcidemia eMedicine An in depth look at propionic acidemia. http://www.sedirectory.net/Health/Conditions_and_Diseases/Genetic_Disorders/Prop

Web Hosting Dir Web Design Dir Search Engine Dir Hardware Info ... Resources Search: Top Health Conditions and Diseases Genetic Disorders ... Propionic Acidemia See also: Health: Conditions and Diseases: Rare Disorders Dr. Greene: Breastfeeding with a Metabolic Disorder and Propionic Acidemia - Breastfeeding a child with propionic acidemia is discussed in this question and answer format. eMedicine - An in depth look at Propionic acidemia. NORD: Acidemia, Propionic - Includes the synonyms, a general discussion and further resources. Pediatric Database - Offers a definition of propionic acidemia as well as the epidemiology, pathogenesis, clinical features, investigations and management.

45. ScienceDaily -- Browse Topics: Health/Conditions_and_Diseases/Genetic_Disorders/ Click Here. Top Health Conditions and Diseases Genetic Disorderspropionic acidemia (4 links). News about propionic acidemia http://www.sciencedaily.com/directory/Health/Conditions_and_Diseases/Genetic_Dis

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 ... Genetic Disorders Propionic Acidemia (4 links) See Also: Health: Conditions and Diseases: Rare Disorders News about Propionic Acidemia Focusing On Preservatives: How They Keep Food Fresh (November 13, 2002) full story Cattle Diets Could Control E. Coli Danger (September 11, 1998) full story [ More news about Propionic Acidemia Books about Propionic Acidemia Amazon.com's Price: Prices subject to change. Transforming Academia: Challenges and Opportunities for an Engaged Anthropology (American Ethnological Society Monograph Series, No. 8) by: Linda G. Basch

46. Propionic Acidemia - Encyclopedia Article About Propionic Acidemia. Free Access, PillSupplier.com Conditions and Diseases/Genetic Disorders Category propionic acidemia. HOME ABOUT US ORDER STATUS BMI CALCULATOR FAQ CONTACT US. Conditions and Diseases/Genetic Disorders/propionic acidemia. http://encyclopedia.thefreedictionary.com/Propionic acidemia

Dictionaries: General Computing Medical Legal Encyclopedia Propionic acidemia Word: Word Starts with Ends with Definition Propionic acidemia is an inborn error of intermediary metabolism Metabolism is the following: When concerning an organism in its entirety, metabolism (also called total metabolism ) is all of the living organism's chemical processes. The organism's metabolism can be dichotomized into the synthesis of organic molecules (anabolism) and their breakdown (catabolism). When concerning a particular substance, metabolism (also called specific metabolsim ) is the chemical activity involving this substance in a living organism. This is commonly the digestion of food, and the disposal of wastes. When concerning a particular living cell, metabolism (also called cell metabolism ) is all of the chemical processes in that cell. Click the link for more information. that may present in the early neonatal period with progressive encephalopathy and death due to a secondary hyperammonemia Hyperammonemia is a metabolic disturbance characterised by an excess of ammonia in the blood. It is a dangerous condition that may lead to encephalopathy and death. It may be primary or secondary. Ammonia is a substance that contains nitrogen. It is a product of the catabolism of protein. It is converted to the non-toxic substance urea prior to excretion in urine by the kidneys. The metabolic pathways that synthesise urea are located in mitochondria. The process is known as the urea cycle, which comprises several enzymes acting in sequence.

47. Propionic Acidemia Top Health Conditions and Diseases Genetic Disorders propionic acidemia (4) Previewsby Thumbshots eMedicine An in depth look at propionic acidemia. http://www.oobdoo.com/directory/Health/ConditionsandDiseases/GeneticDisorders/Pr

World Wide Search Engine and Portal to the Best Sites on the Internet Over 15million sites and over 550,000 categories Top Health Conditions and Diseases Genetic Disorders : Propionic Acidemia (4) See Also: Health: Conditions and Diseases: Rare Disorders Pediatric Database - Offers a definition of propionic acidemia as well as the epidemiology, pathogenesis, clinical features, investigations and management. eMedicine - An in depth look at Propionic acidemia. Dr. Greene: Breastfeeding with a Metabolic Disorder and Propionic Acidemia - Breastfeeding a child with propionic acidemia is discussed in this question and answer format. NORD: Acidemia, Propionic - Includes the synonyms, a general discussion and further resources. Back to ooBdoo Suggest a Site Feedback Link to ooBdoo ... Guarantee your site is listed This page also has modified,additions and deletions of information obtained from the Open Directory Project Help build the largest human-edited directory on the web. Submit a Site Open Directory Project Become an Editor

48. Wauu.DE: Health: Conditions And Diseases: Genetic Disorders: Propionic Acidemia Search DMOZVerzeichnis All Categories Categories Onlye. Links URLhinzufügen. eMedicine An in depth look at propionic acidemia. http://www.wauu.de/Health/Conditions_and_Diseases/Genetic_Disorders/Propionic_Ac

Home Health Conditions and Diseases Genetic Disorders : Propionic Acidemia Search DMOZ-Verzeichnis: All Categories Categories Onlye Links: eMedicine An in depth look at Propionic acidemia. http://www.emedicine.com/NEURO/topic577.htm NORD - Acidemia, Propionic Includes the synonyms, a general discussion and further resources. Pediatric Database Offers a definition of propionic acidemia as well as the epidemiology, pathogenesis, clinical features, investigations and management. http://www.icondata.com/health/pedbase/files/PROPIONI.HTM © 1998- 2002 Ein Service von Wauu.de UserNet.DE Linktip: MnogoSearch.ORG Map TopTen TopTen eng Aktuelle Linktips Mozilla 1.7 RC 1 Basteln 0190 Warner Free Rund ums Rad ... Fantasy - Shop Webmaster-Links Webspace Free Subdomain Service Merchandise Fun SUMA - Forum ... Abmahnungen œber Wauu Regeln Webmasterservice Impressum b

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50. RedNova News: Propionic Acidemia February 01, 2003. RedNova Health Link. propionic acidemia. SynonymsPCC Deficiency. Propionyl CoA Carboxylase Deficiency. Ketotic Glycinemia. http://www.rednova.com/news/stories/2/2003/02/01/story049.html

Forum Check E-mail My RedNova Join Us ... Tell a Friend - Win $500 Search February 01, 2003 RedNova Health Link Propionic Acidemia Synonyms: PCC Deficiency Propionyl CoA Carboxylase Deficiency Ketotic Glycinemia Hyperglycinemia with Ketoacidosis and Lactic Acidosis, Propionic Type More from the world's of science, space, and technology from RedNova Email this story to a friend Post your thoughts on this story

51. Startplane /Health/Conditions_and_Diseases/Genetic_Disorders/Propionic_Acidemia propionic acidemia. See also eMedicine An in depth look at propionic acidemia.This category needs an editor. Last Updated 2003-08-29 182844. http://www.startplane.com/Health/Conditions_and_Diseases/Genetic_Disorders/Propi

Start Plane About Us Free Webpages Top Health Propionic Acidemia Top Propionic Acidemia Sites Your Site Here! - Get your site listed on StartPlane! The #1 performance based directory online! See also: Top/Health/Conditions and Diseases/K Top/Health/Conditions and Diseases/P Links Dr. Greene: Breastfeeding with a Metabolic Disorder and Propionic Acidemia - Breastfeeding a child with propionic acidemia is discussed in this question and answer format. NORD: Acidemia, Propionic - Includes the synonyms, a general discussion and further resources. Pediatric Database - Offers a definition of propionic acidemia as well as the epidemiology, pathogenesis, clinical features, investigations and management. eMedicine - An in depth look at Propionic acidemia. This category needs an editor Last Updated: 2003-08-29 18:28:44 Search: search the entire directory search this category only Help build the largest human-edited directory on the web. Submit a Site Open Directory Project Become an Editor The content of this directory is based on the Open Directory and may have been modified by DWodp Powered by DWodp pro Dominion Web

52. Conditions And Diseases: Genetic Disorders: Propionic Acidemia| Treasure Coast H Information on Conditions and Diseases, Genetic Disorders, PropionicAcidemia and much more Treasure Coast Health. Treasure Coast http://treasurecoasthealth.com/treasurecoasthealth.php/Health/Conditions_and_Dis

Home Doctor Directory Health Resources Resources for Women Newsletter Sign Up ... Advertise with Us ** Buy text Ads! About Us Conditions and Diseases: Genetic Disorders: Propionic Acidemia Canadian Pharmacy See also: Health: Conditions and Diseases: Rare Disorders Dr. Greene: Breastfeeding with a Metabolic Disorder and Propionic Acidemia - Breastfeeding a child with propionic acidemia is discussed in this question and answer format. eMedicine - An in depth look at Propionic acidemia. NORD: Acidemia, Propionic - Includes the synonyms, a general discussion and further resources. Pediatric Database - Offers a definition of propionic acidemia as well as the epidemiology, pathogenesis, clinical features, investigations and management. Sponsors: Xango Mangosteen - Order Xango juice online Chilean Pharmacies - Buy Chile drugs and save Oral Chelation Therapy - Oral Chelation Therapy PCA-Rx is an alternative treatment for and mercury toxicity. The result of years of rigorous research and development.PCA-Rx English Prescriptions - 80% savings from English pharmacy online British Prescription Drugs - 80% savings from British pharmacy online On Line Pharmacy Get prescription drugs online today at our pharmacy.

53. ORPHANET - Rare Diseases - Orphan Drugs Printing version, DISEASE propionic acidemia, Synonym(s) Glycinemia, ketoticPropionylCoA carboxylase deficiency, ICD E71.1, Propionicacidemia http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=GB&Expert=35

54. »»Reviews For Propionic Acidemia«« propionic acidemia Reviews. Related Subjects Genetic_Disorders More Pagespropionic acidemia Page 1 2 3. Search site for a Book Review. http://www.booksunderreview.com/Health/Conditions_and_Diseases/Genetic_Disorders

Propionic Acidemia Reviews Related Subjects: More Pages: Propionic Acidemia Page 1 Book reviews for "Propionic Acidemia" sorted by average review score: Academic Affairs: Love and Murder in Academia Author: Phillip Gay Amazon base price: List price: that's off! Average review score: Academic Affairsa cleverly written murder mystery. Phillip Gay fuses the romantic intrigues of academia and the psyche of a killer into a cleverly constructed murder mystery. Intriguing; a great read! Very enjoyable! This book was sexy, exciting and provided a behind-the-scenes look into the academic world. There were a few editing mistakes that I caught, but besides that it was very well written! Academic Affairs: Love and Murder in Academia Enjoyable characters, locations, and situations. Couldn't put it down. Lots of actiondouble murder on page 1. Can't wait for the sequel. Hope there's a movie American Academia and the Survival of Marxist Ideas Published in Hardcover by Praeger Publishers (September, 1996) Author: Dario Fernandez-Morera Amazon base price: Used price: Average review score: Reveals much about the Marxist bent of American academics Career Strategies for Women in Academia : Arming Athena Published in Paperback by Sage Publications (September, 1998)

55. Propionic Acidemia From Linkspider UK Health Directory propionic acidemia by Linkspider UK, propionic acidemia links and PropionicAcidemia topics from our Health directory. Helping you http://linkspider.co.uk/Health/ConditionsandDiseases/GeneticDisorders/PropionicA

Match » -All words -Any word -Exact text Search » The Web Jobs / Vacancy Images / Photos FTP / Downloads United Kingdom United States of America Argentina Austria Australia Bangladesh Belgium Bolivia Brazil Bulgaria Canada Chile China Cuba Cyprus Czech Republic Czechoslovakia Denmark Dominican Republic Ecuador Egypt Estonia Finland France Germany Ghana Greece Hong Kong Hungary Iceland India Indonesia Ireland Israel Italy Japan Jordan Kenya Kuwait Latvia Lithuania Luxembourg Malaysia Malta Mexico Moldavia Monaco Morocco Mozambique Nepal Netherlands New Zealand Nicaragua Nigeria North Korea Norway Pakistan Panama Paraguay Peru Philippines Poland Portugal Qatar Romania Russian Federation Saudi Arabia Singapore South Africa South Korea Spain Sri Lanka Sweden Switzerland Taiwan Tanzania Thailand Tunisia Turkey Ukraine United Arab Emirates Uruguay Venezuela Yemen Yugoslavia Zambia Zimbabwe Ranking » On (no duplicate) Off (allow duplicate) Add my Site Toolbar Affiliates Directory Topic Propionic Acidemia assoicated to Health Directory Tree: Top Health Conditions and Diseases Genetic Disorders : Propionic Acidemia ( See Also: Health: Conditions and Diseases: Rare Disorders Pediatric Database - Offers a definition of propionic acidemia as well as the epidemiology, pathogenesis, clinical features, investigations and management.

56. Effect Of Chemically Induced Propionic Acidemia On Neurobehavioral Development O Pharmacol Biochem Behav. 1999 Nov;64(3)52934. Effect of chemicallyinduced propionic acidemia on neurobehavioral development of rats. http://hair-loss-alopecia.com/ref-hair-growth/hair-growth-research-abs3.389.html

References: Hair growth and hair loss Pharmacol Biochem Behav. 1999 Nov;64(3):529-34. Effect of chemically induced propionic acidemia on neurobehavioral development of rats. Brusque AM, Mello CF, Buchanan DN, Terracciano ST, Rocha MP, Vargas CR, Wannmacher CM, Wajner M. Departmento de Bioquimica, Instituto de Biociencias, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil. Biochem Pharmacol. 1990 Mar 15;39(6):1077-83. Mechanism of growth inhibition of melanoma cells by 4-S-cysteaminylphenol and its analogues. Inoue S, Ito S, Wakamatsu K, Jimbow K, Fujita K. Institute for Comprehensive Medical Science, School of Hygiene, Fujita Health University, Toyoake, Japan. Fundam Appl Toxicol. 1990 May;14(4):842-9. Immunotoxicity of the pyrrolizidine alkaloid monocrotaline following subchronic administration to C57Bl/6 mice. Deyo JA, Kerkvliet NI. College of Veterinary Medicine, Oregon State University, Corvallis 97331-4802. Dream Pharmaceuticals Rx Online Lipitor Dream Pharmaceuticals Rx Online Paxil Dream Pharmaceuticals Rx Online Phentermine DreamPharm: Herbal and Nutritional supplements online Hair Million herbal formula for hair loss and hair growth Hair Million, excellent herbal formula, wards off hair loss and promotes hair growth

57. The Turkish Journal Of Pediatrics Neonatal onset propionic acidemia without acidosis a case report. Ýpek Akman 1, Sebahat Ýmamoðlu 1 , Mübeccel Demirkol 2 , Harika Alpay 1 , Eren Özek 1. http://tjp.dergisi.org/summary_en.php3?id=14

58. REFERENCES Campeau E, Dupuis L, Leclerc D, Gravel RA (1999a) Detection of a normally rare transcriptin propionic acidemia patients with mRNA destabilizing mutations in http://www.uchsc.edu/cbs/pcc/references.htm

REFERENCES * An asterisk denotes an unpublished observation. PCCA Mutations Campeau E , Dupuis L, Leclerc D, Gravel RA Detection of a normally rare transcript in propionic acidemia patients with mRNA destabilizing mutations in the PCCA gene. Hum Mol Genet [MEDLINE] Campeau E , Dupuis L, Le on-Del-Rio A, Gravel R Coding sequence mutations in the alpha subunit of propionyl-CoA carboxylase in patients with propionic acidemia. Mol Genet Metab [MEDLINE] Campeau E, Desviat LR, Leclerc D, Perez B, Ugarte M, Gravel RA. Structure of the pcca gene and distribution of mutations causing propionic acidemia. Mol Genet Metab Sep-Oct;74(1-2):238-47 [MEDLINE] Desviat LR, Campeau E, Leclerc D, Perez B, Ugarte M, Scherer S, Gravel RA. Structure of the PCCA gene and identification of novel mutations in propionic acidemia. J Inherit Metab Dis (Suppl.1), p56 [MEDLINE not available] Heptinstall LE , Gillaspy EE, Loftus HM, Till J, Walter JH, Wraith JE, Besley GTN Mutation analysis in propionic acidaemia J Inherit Metab Dis (Suppl.2), p79

59. About PCC propionic acidemia. A disorder of metabolic acidosis. propionic acidemiawas first described in 1961 by Childs et al. (1961). The http://www.uchsc.edu/cbs/pcc/about_pcc.htm

ABOUT PCC KRAUSLAB MAIN PAGE CBS PCC PCCA mutations ... Mutation submission About PCC Other PCC pages DIAGNOSTICS SEARCH PROPIONIC ACIDEMIA A disorder of branch-chain amino acid metabolism characterized by the build-up of propionic acid resulting in episodes of vomiting, dehydration, and severe metabolic acidosis. Propionic acidemia was first described in 1961 by Childs et al. (Fenton and Rosenberg 1995) . Surtees et al. have also reported a high prevalence of neurologic sequelae, including dystonia, severe chorea, and pyramidal signs, particularly in patients who survive longer. Leukopenia and thrombocytopenia, perhaps due to marrow suppression by one or more of the toxic metabolites produced, is also not uncommon. Recently, magnetic resonance imaging of the brain in three PCC patients revealed delayed myelination and some cerebral atrophy. Proton magnetic resonance spectroscopy from a voxel located in basal ganglia revealed a decrease in N-acetylaspartate and myo-inositol peaks and an elevation of GLN/GLU. The presence of spectroscopic abnormalities indicates that the metabolic balance on cerebral parenchymal level is less optimal than estimated from biochemical analysis of urine, plasma, or cerebrospinal fluid (Bergman et al. 1996). Biochemically, patients with this disorder present with elevated levels of propionic acid, methylcitrate

60. Propionic Acidemia propionic acidemia Directory Guide to propionic acidemiasites on the internet. propionic acidemia. http://www.directory.net/Health/Conditions_and_Diseases/Genetic_Disorders/Propio

Propionic Acidemia Directory: Guide to Propionic Acidemia sites on the internet. Search Engines: Google Yahoo MSN FindWhat ... City Guides Propionic Acidemia Health Conditions and Diseases Genetic Disorders Propionic Acidemia Websites Pediatric Database http://www.icondata.com/health/pedbase/files/propioni.htm Offers a definition of propionic acidemia as well as the epidemiology, pathogenesis, clinical features, investigations and management. eMedicine http://www.emedicine.com/neuro/topic577.htm An in depth look at Propionic acidemia. Dr. Greene: Breastfeeding with a Metabolic Disorder and Propionic Acidemia http://www.drgreene.com/21_692.html Breastfeeding a child with propionic acidemia is discussed in this question and answer format. NORD: Acidemia, Propionic http://www.rarediseases.org/search/rdbdetail_abstract.html?disname=acidemia%2c%20propionic Includes the synonyms, a general discussion and further resources. Got eight minutes? Join in and participate in Stanford's Web Credibility Project's latest study. Help build the largest human-edited directory on the web. Submit a Site Open Directory Project Become an Editor The content of Directory.NET

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