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Urea Cycle Disorders: more detail

Urea cycle disorders: An entry from Thomson Gale's <i>Gale Encyclopedia of Genetic Disorders, 2nd ed.</i> by Julianne Remington, 2005
Congenital disorders of the urea cycle and ammonia detoxication (Monographs in paediatrics) by Jean Pierre Colombo, 1971
The Pediatrician's Guide to Ornithine Transcarbamylase Deficiency . . . and other Urea Cycle Disorders (The National Organization for Rare Disorders, NORD Guides for Physicians #2) by The National Organization for Rare Disorders, 1999
Inherited disorders of the urea cycle by George K Summer, 1973
CONGENIAL DISORDERS OF THE UREA CYCLE AND AMMONIA DETOXICATION( Monographs in Pediatrics) by J.P. Colombo, 1971
Congenital Disorders of the Urea Cycle and Ammonia Detoxication Volume I
Congenital Disorders of the Urea Cycle and Ammonia Detoxication Volume I by Jean-Pierre Colombo, 1971-01-01
Urea Cycle Diseases (Advances in Experimental Medicine & Biology)

lists with details

Health Library -
urea cycle disorders. This information is provided as a resource and does not constitute an endorsement for any group. National urea cycle disorders Foundation.
http://myhealth.barnesjewish.org/library/healthguide/selfhelp/topic.asp?hwid=shc
UREA CYCLE DISORDERS
urea cycle disorders. urea cycle disorders (UCD) are a group of inherited conditions, characterised by enzyme deficiencies of the urea cycle.
http://www.shsweb.co.uk/metabolic/3132/products/UREACYCLE.htm

Extractions: UREA CYCLE DISORDERS Urea Cycle Disorders (UCD) are a group of inherited conditions, characterised by enzyme deficiencies of the urea cycle. The result is waste nitrogen accumulating as ammonia and glutamine, both of which are neurotoxic and may cause severe encephalopathy. The aim of dietary management is to restrict dietary protein by means of a low protein diet. The protein requirements are met by supplementing the diet with an essential amino acid mixture if necessary. Dialamine A palatable orange flavoured powder containing 30% essential amino acids and carbohydrate which when mixed with water provides a pleasant tasting drink. Dialamine may be used for oral and tube feeding where essential amino acid supplementation is required. Dialamine may be used for conditions requiring a controlled nitrogen intake.
British National Formulary: Urea Cycle Disorders
urea cycle disorders. LArginine supplement.
http://www.bnf.org/bnf/bnf/current/doc/61850.htm
National Urea Cycle Disorders Foundation
geneticalliance.org. Return to Search Page National urea cycle disorders Foundation. 4841 Hill Street La Canada, CA 910112332 Phone 818.790
http://www.geneticalliance.org/diseaseinfo/displayorganization.html?orgname=Nati
Definitions Of Genetic Disorders-U
Urea Cycle Disorder Arginase Type arch.htm Urea Cycle Disorder Arginino Succinase Type asl.htm urea cycle disorders Carbamyl Phosphate Synthetase Type cpsd.htm
http://www.icomm.ca/geneinfo/def-u.htm
N-Acetyl Glutamate Synthetase Deficiency,Inborn Errors Of Urea Synthesis Glutama
NAcetyl Glutamate Synthetase Deficiency,Inborn Errors of Urea Synthesis Glutamate Synthetase Type,NAGS Deficiency,urea cycle disorders N-Acrtyl Glutamate
http://www.icomm.ca/geneinfo/nags.htm

Extractions: N-Acetyl Glutamate Synthetase Deficiency,Inborn Errors of Urea Synthesis Glutamate Synthetase Type,NAGS Deficiency,Urea Cycle Disorders N-Acrtyl Glutamate Synthetase Type,N-Acetyl Glutamate Synthetase Deficiency,Inborn Errors of Urea Synthesis Glutamate Synthetase Type,NAGS Deficiency,Urea Cycle Disorders N-Acrtyl Glutamate Synthetase Type National Organization for Rare Disorders N-acetylglutamate synthetase (NAGS) deficiency is a rare genetic disorder characterized by complete or partial lack of the enzyme N-acetylglutamate synthetase (NAGS). NAGS is one of six enzymes that play a role in the break down and removal of nitrogen from the body, a process known as the urea cycle.
Pitch Letter
urea cycle disorders are caused by high ammonia levels in the blood stream and, if left untreated, will lead to death. National urea cycle disorders Foundation.
http://www.personal.psu.edu/users/m/c/mch193/Writing Samples.htm
Health Information Resource Database: National Urea Cycle Disorders Foundation
National urea cycle disorders Foundation. Abstract. This nonprofit organization is dedicated to the identification, treatment and cure of urea cycle disorders.
http://www.health.gov/NHIC/NHICScripts/Entry.cfm?HRCode=HR3331
Genetic Disorders, Urea Cycle
online shopping can be fun! National urea cycle disorders Foundation Information about the organization as well as the disease.
http://www.iseekhealth.com/urea_cycle-1627.php

Extractions: National Urea Cycle Disorders Foundation - Information about the organization as well as the disease. Family support, membership, newborn screening project and medical information. Urea Cycle Disorders - Information, medical links and a message board. The true story of a one family's struggle with this illness.
Resource Library Find Information On Hereditary Urea Cycle
Support Groups NATIONAL urea cycle disorders FOUNDATION 4841 Hill Street; La Canada, CA 91011 Phone 818790-2460 Toll-Free 1-800-38-NUCDF www.nucdf.org.
http://www.mercksource.com/pp/us/cns/cns_hl_adam.jspzQzpgzEzzSzppdocszSzuszSzcns
Health Library -
urea cycle disorders. This information is provided as a resource and does not constitute an endorsement for any group. National urea cycle disorders Foundation.
http://health_info.nmh.org/Library/HealthGuide/SelfHelp/topic.asp?hwid=shc29ure
:: Ez2Find :: Urea Cycle
Web Sites, National urea cycle disorders Foundation Site Info - Translate - Open New Window Information about the organization as well as the disease.
http://ez2find.com/cgi-bin/directory/meta/search.pl/Health/Conditions_and_Diseas

Extractions: Any Language English Afrikaans Arabic Bahasa Melayu Belarusian Bulgarian Catala Chinese Simplified Chinese Traditional Cymraeg Czech Dansk Deutsch Eesti Espanol Euskara Faroese Francais Frysk Galego Greek Hebrew Hrvatski Indonesia Islenska Italiano Japanese Korean Latvian Lietuviu Lingua Latina Magyar Netherlands Norsk Polska Portugues Romana Russian Shqip Slovensko Slovensky Srpski Suomi Svenska Thai Turkce Ukrainian Vietnamese Mode Guides Urea Cycle Web Sites National Urea Cycle Disorders Foundation [Site Info] [Translate] [Open New Window] Urea Cycle Disorders [Site Info] [Translate] [Open New Window] Information, medical links and a message board. The true story of a one family's struggle with this illness. URL: http://www.2endure.com
WebMD With AOL Health - Index
UR Urea Breath Test MedicalTest Urea Cycle Disorder Discussion Board urea cycle disorders Shc Urea Cycle Disorder, Arginase Type Nord Urea Cycle
http://aolsvc.health.webmd.aol.com/hw/index/index-all-U.asp
239: Phenotypic Correlation And Diagnosis Of Urea Cycle Disorders With Stable Is
Program Nr 239 Phenotypic correlation and diagnosis of urea cycle disorders with stable isotope infusions. B. Lee 1 , H. Yu 2 , F
http://www.faseb.org/genetics/ashg99/f239.htm

Extractions: Program Nr: 239 Phenotypic correlation and diagnosis of urea cycle disorders with stable isotope infusions. B. Lee , H. Yu , F. Jahoor , W. O'Brien , A.L. Beaudet , P. Reeds N-urea, O-urea, and N-glutamine enrichments measured in blood during intravenous co-infusions of 5- N-glutamine and O-urea. The ratio of N-urea/ N-urea/ N-glutamine ratio is a sensitive index of in vivo urea cycle activity, correlates with clinical everity and is a sensitive tool for evaluating efficacy of therapeutic modalities as well as for the diagnosis and management of urea cycle patients.
Genetic Disorders: Urea Cycle
National urea cycle disorders Foundation. Information about the organization as well as the disease. Family urea cycle disorders. Information
http://www.puredirectory.com/Health/Conditions-and-Diseases/Genetic-Disorders/Ur

Extractions: Home Health Conditions and Diseases Genetic Disorders : Urea Cycle google_ad_client = 'pub-3272565765518472';google_ad_width = 336;google_ad_height = 280;google_ad_format = '336x280_as';google_color_border = 'FFFFFF';google_color_bg = 'FFFFFF';google_ad_channel ='7485447737';google_alternate_color = 'FFFFFF';google_color_link = '0000FF';google_color_url = '008000';google_color_text = '000000';
Urea Cycle
urea cycle disorders Information, medical links and a message board. The true story of a one family s struggle with this illness.
http://www.thenewhealthfind.com/Health/ConditionsandDiseases/GeneticDisorders/Ur
ARS | Publication Request: In Vivo Urea Cycle Flux Distinguishes And Correlates
Technical Abstract urea cycle disorders are a group of inborn errors of hepatic metabolism that result in often lifethreatening hyperammonemia and
http://www.ars.usda.gov/research/publications/Publications.htm?seq_no_115=147060
Entrez PubMed
Click here to read urea cycle disorders. Kleppe S, Mian A, Lee B. Department of Molecular and Human Genetics and Howard Hughes Medical
http://www.biomedcentral.com/pubmed/12791198

Extractions: Summary Brief Abstract Citation ASN.1 MEDLINE XML UI List LinkOut Related Articles Cited in Books CancerChrom Links Domain Links 3D Domain Links GEO DataSet Links Gene Links Genome Links GEO Links HomoloGene Links Nucleotide Links OMIM Links PMC Links Cited in PMC PopSet Links Protein Links SNP Links Structure Links UniSTS Links Show: Sort Author Journal Pub Date Text File Clipboard E-mail Order Urea cycle disorders comprise a group of inborn errors of metabolism that represent unique gene-nutrient interactions whose significant morbidity arises from acute and chronic neurotoxicity associated with often massive hyperammonemia. Current paradigms of treatment are focused on controlling the flux of nitrogen transfer through the hepatic urea cycle by a combination of dietary and pharmacologic approaches. Evolving paradigms include the development of cell and gene therapies. Current research is focused on understanding the pathophysiology of ammonia-mediated toxicity and prevention of neural injury.
BioMed Central | Abstract | Urea Cycle Disorders
Opinion urea cycle disorders Soledad Kleppe MD, Asad Mian MD and Brendan Lee MD PhD Department of Molecular and Human Genetics and Howard Hughes Medical
http://www.biomedcentral.com/1092-8480/5/309/abstract

Extractions: Urea cycle disorders comprise a group of inborn errors of metabolism that represent unique gene-nutrient interactions whose significant morbidity arises from acute and chronic neurotoxicity associated with often massive hyperammonemia. Current paradigms of treatment are focused on controlling the flux of nitrogen transfer through the hepatic urea cycle by a combination of dietary and pharmacologic approaches. Evolving paradigms include the development of cell and gene therapies. Current research is focused on understanding the pathophysiology of ammonia-mediated toxicity and prevention of neural injury.
Argininosuccinic Aciduria (ASA)
Argininosuccinic Aciduria (ASA) A Urea Cycle Disorder. Patients with urea cycle disorders cannot convert nitrogen in the form of ammonia into urea.
http://www.savebabies.org/diseasedescriptions/asa.php

Extractions: Argininosuccinic Aciduria (ASA) is a disorder of the urea cycle. Patients with urea cycle disorders cannot convert nitrogen in the form of ammonia into urea. Build up of ammonia is toxic and can cause brain damage. Nitrogen in ammonia comes from protein in the food we eat or the breakdown of protein from the muscle when we are sick. Without the urea cycle, ammonia will build up to dangerous levels when we take in too much protein or when we are sick. ASA is one of the urea cycle disorders and is caused by deficiency of an enzyme called argininosuccinic acid lyase in the body. This prevents the conversion of argininosuccinic acid (ASA) into arginine. The build up in ASA if too high ultimately causes a build up in ammonia. At the same time, arginine may be deficient. Inheritance and Frequency The gene defect for ASA is an autosomal recessive genetic trait and is unknowingly passed down from generation to generation. This faulty gene only emerges when two carriers have children together and pass it to their offspring. For each pregnancy of two such carriers, there is a 25% chance that the child will be born with the disease and a 50% chance the child will be a carrier for the gene defect.

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