81. Entrez PubMed Fibroblasts of patients with other fatty acid oxidation disorders showed distinctelevations of diseasespecific acylcarnitines. http://ghr.nlm.nih.gov/condition=longchain3hydroxyacylcoenzymeadehydrogenasedefi

My NCBI [Sign In] [Register] All Databases ... Books Search PubMed Protein Nucleotide Structure Genome Books CancerChromosomes Conserved Domains 3D Domains Gene Genome Project GENSAT GEO Profiles GEO DataSets HomoloGene Journals MeSH NCBI Web Site NLM Catalog OMIA OMIM PMC PopSet Probe PubChem BioAssay PubChem Compound PubChem Substance SNP Taxonomy UniGene UniSTS for Limits Preview/Index History Clipboard Details About Entrez Text Version Entrez PubMed Overview FAQ Tutorial New/Noteworthy ... E-Utilities PubMed Services Journals Database MeSH Database Single Citation Matcher Batch Citation Matcher ... Cubby Related Resources Order Documents NLM Mobile NLM Catalog NLM Gateway ... PubMed Central Display Summary Brief Abstract Citation MEDLINE XML UI List LinkOut ASN.1 Related Articles Cited Articles Cited in Books CancerChrom Links Domain Links 3D Domain Links GEO DataSet Links Gene Links Gene (GeneRIF) Links Genome Links Project Links GENSAT Links GEO Profile Links HomoloGene Links Nucleotide Links OMIA Links OMIM Links BioAssay Links Compound Links Compound via MeSH Substance Links Substance via MeSH PMC Links Cited in PMC PopSet Links Probe Links Protein Links SNP Links Structure Links UniGene Links UniSTS Links Show Sort by Author Journal Pub Date Send to Text File Clipboard E-mail Order All: 47 Review: 7 Items 1 - 20 of 47 of 3 Next Shen JJ, Matern D, Millington DS, Hillman S, Feezor MD, Bennett MJ, Qumsiyeh M, Kahler SG, Chen YT, Van Hove JL.

82. BONNET D BACKGROUND The clinical manifestations of inherited disorders of fatty acid Arrhythmia is an unusual presenting symptom of fatty acid oxidation http://www.necker.fr/irnem/Unites 1999/Fédération de Pédiatrie.htm

BONNET D., MARTIN D., de LONLAY P., VILLAIN E., JOUVET P., RABIER D., BRIVET M. SAUDUBRAY J.M. Arrhythmias and conduction defects as presenting symptoms of fatty acid oxidation disorders in children. Circulation, (Services cités : Cardiologie Pédiatrique, Fédération de Pédiatrie, Biochimie Médicale, Métabolisme-Neurologie Génétique Pédiatrique COLOMB V. Home parenteral nutrition: the pediatric point of view. Nutrition, (Services cités : Fédération de Pédiatrie, Gastroentérologie Pédiatrique ROBERT J.J., TETE M.J., GUEST G., GAGNADOUX M.F., NIAUDET P. BROYER M. Diabetes mellitus in patients with infantile cystinosis after renal transplantation. Pediat. Nephrol., (Services cités : Fédération de Pédiatrie, Néphrologie Pédiatrique SAUDUBRAY J.M., TOUATI G., DELONLAY P., JOUVET P., NARCY C., LAURENT J., RABIER D., KAMOUN P., JAN D. REVILLON Y. Liver transplantation in urea cycle disorders. Eur. J. Pediat., (Suppl 2), S55-S59, 1999 (Services cités : Fédération de Pédiatrie, Biochimie Médicale SAUDUBRAY J.M., TOUATI G., DELONLAY P., JOUVET P., SCHLENZIG J., NARCY C., LAURENT J., RABIER D., KAMOUN P., JAN D. REVILLON Y.

83. FATTY ACID OXIDATION DISORDERS – LCHAD, VLCAD fatty ACID oxidation disorders LCHAD, VLCAD. Several defects of beta oxidationpathways are known resulting clinically in a range of symptoms including http://www.shsweb.co.uk/metabolic/3132/products/FATTYACID.htm

FATTY ACID OXIDATION DISORDERS LCHAD, VLCAD Several defects of beta oxidation pathways are known resulting clinically in a range of symptoms including liver failure, cardiomyopathy, muscle weakness and life threatening coma when ill. Dietary management of LCHAD and VLCAD and disorders of carnitine transport, aims to limit long chain fat and to maximise carbohydrate with frequent feeding and avoidance of fasting. Medium chain fat can be included provided there is no defect in medium chain fatty acid oxidation. Monogen A powdered nutritionally complete low fat, whole whey protein feed containing 90% of fat as medium chain triglycerides (MCT) and a small amount of essential fatty acids, designed for use in LCAD deficiency where MCT is tolerated. It can also be used in other lipid and lymphatic disorders. Age Range Pack Size infants and children 400g can PRECAUTIONS Only intended for use by patients with Long Chain Fat Disorders Use under medical supervision Not for parenteral use

84. NEJM -- A Fetal Fatty-Acid Oxidation Disorder As A Cause Of Liver Disease In Pre Original Article from The New England Journal of Medicine A Fetal fattyAcidOxidation Disorder as a Cause of Liver Disease in Pregnant Women. http://content.nejm.org/cgi/content/short/340/22/1723

HOME SEARCH CURRENT ISSUE PAST ISSUES ... HELP Please sign in for full text and personal services Previous Volume 340:1723-1731 June 3, 1999 Number 22 Next A Fetal Fatty-Acid Oxidation Disorder as a Cause of Liver Disease in Pregnant Women Jamal A. Ibdah, M.D., Ph.D., Michael J. Bennett, Ph.D., Piero Rinaldo, M.D., Ph.D., Yiwen Zhao, B.S., Beverly Gibson, B.S., Harold F. Sims, B.A., and Arnold W. Strauss, M.D. Full Text PDF Add to Personal Archive Add to Citation Manager ... PubMed Citation ABSTRACT Background Acute fatty liver of pregnancy and the HELLP syndrome (hemolysis, elevated liver-enzyme levels, and a low platelet count) are serious hepatic disorders that may occur during pregnancy in women whose fetuses are later found to have a deficiency of long-chain 3-hydroxyacyl-coenzyme A (CoA) dehydrogenase. This enzyme resides in the mitochondrial trifunctional protein, which also contains the active site of long-chain 2,3-enoyl-CoA hydratase and long-chain 3-ketoacyl-CoA thiolase. We undertook this study to determine the relation between mutations in the trifunctional protein in infants with defects in fatty-acid oxidation and acute liver disease during pregnancy in their mothers.

85. NEJM -- A Fetal Fatty-Acid Oxidation Disorder As A Cause Of Liver Disease In Pre Original Article from The New England Journal of Medicine A Fetal fattyAcidOxidation Disorder as a Cause of Liver Disease in Pregnant Women. http://content.nejm.org/cgi/content/abstract/340/22/1723

HOME SEARCH CURRENT ISSUE PAST ISSUES ... HELP Please sign in for full text and personal services Previous Volume 340:1723-1731 June 3, 1999 Number 22 Next A Fetal Fatty-Acid Oxidation Disorder as a Cause of Liver Disease in Pregnant Women Jamal A. Ibdah, M.D., Ph.D., Michael J. Bennett, Ph.D., Piero Rinaldo, M.D., Ph.D., Yiwen Zhao, B.S., Beverly Gibson, B.S., Harold F. Sims, B.A., and Arnold W. Strauss, M.D. Full Text PDF Add to Personal Archive Add to Citation Manager ... PubMed Citation ABSTRACT Background Acute fatty liver of pregnancy and the HELLP syndrome (hemolysis, elevated liver-enzyme levels, and a low platelet count) are serious hepatic disorders that may occur during pregnancy in women whose fetuses are later found to have a deficiency of long-chain 3-hydroxyacyl-coenzyme A (CoA) dehydrogenase. This enzyme resides in the mitochondrial trifunctional protein, which also contains the active site of long-chain 2,3-enoyl-CoA hydratase and long-chain 3-ketoacyl-CoA thiolase. We undertook this study to determine the relation between mutations in the trifunctional protein in infants with defects in fatty-acid oxidation and acute liver disease during pregnancy in their mothers.

86. Entrez PubMed BACKGROUND Acute fatty liver of pregnancy and the HELLP syndrome (hemolysis,elevated liverenzyme http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=1

87. Metabolic Genetic Conditions Glutaric Acidemia Type I (GAI) An Organic Acid Disorder, SaveBabies;Glutaric Acidemia Type-II, fatty oxidation Disorder (FOD) Support http://www.kumc.edu/gec/support/metaboli.html

Metabolic Conditions Includes: aspartylglusomarinuria, biotinidase deficiency , carbohydrate deficient glycoprotein syndrome (CDGS), Crigler-Najjar syndrome , cystinosis, diabetes insipidus, Fabry fatty acid metabolism disorders galactosemia , Gaucher, glucose-6-phosphate dehydrogenase (G6PD), glutaric aciduria , Hurler, Hurler-Scheie, Hunter, hypophosphatemia, I-cell, Krabbe , lactic acidosis, long chain 3 hydroxyacyl CoA dehydrogenase deficiency (LCHAD), lysosomal storage diseases, mannosidosis maple syrup urine , Maroteaux-Lamy, metachromatic leukodystrophy, mitochondrial , Morquio, mucopolysaccharidosis , neuro-metabolic, Niemann-Pick, organic acidemias , purine, phenylketonuria (PKU) , Pompe, porphyria , pseudo-Hurler, pyruvate dehydrogenase deficiency, Sandhoff, Sanfilippo, Scheie, Sly, Tay-Sachs, trimethylaminuria (Fish-Malodor syndrome) urea cycle conditions , vitamin D deficiency rickets Association for Neuro-Metabolic Disorders 5223 Brookfield Lane Sylvania, OH 43560-1809 Phone: (419) 885-1497 E-mail: volk4olks@aol.com

88. Fatty Oxidation Disorder, Eastern Carolina fatty oxidation Disorder University Health Systems of Eastern Carolina servestarboro, ahoskie, edento, winsor, maxhead, dear county, outebanks counties http://www.uhseast.com/117681.cfm

Health News Health Library Health Topics Healthy Living ... Nutrition Self-Help Resources Information about national and local self-help organizations and support groups. Fatty Oxidation Disorder This information is provided as a resource and does not constitute an endorsement for any group. It is the responsibility of the reader to decide whether a group is appropriate for his/her needs. For evidence-based information on diseases, conditions, symptoms, treatment and wellness issues, continue searching this site. FOD Family Support Group International network. Founded 1991. Provides mutual support and networking for parents of children with Floating Harbor syndrome. Newsletter, literature, phone support. Pictures of children with Floating Harbor syndrome. WRITE: FOD Family Support Group c/o Deb and Dan Gould 805 Montrose Dr. Greensboro, NC 27410 CALL: 336-547-8682 FAX: 336-292-0536 E-MAIL: deb@fodsupport.org WEBSITE: http://www.fodsupport.org VERIFIED: 10/8/2004 The above information was "verified" as correct on the date at the end of each entry. Since American Self-Help Group Clearinghouse's database is extensive but staffing is limited and information for these organizations can change, it is not possible to keep every entry in American Self-Help Group Clearinghouse database completely current and accurate. Please check with the organizations listed for the most current information. For additional information on self-help groups, please visit the American Self-Help Group Clearinghouse web site at http://www.mentalhelp.net/selfhelp

89. Fatty Acid Oxidation The process of fatty acid oxidation is termed boxidation since it occurs Refsum s Disease Refsum s disease is a rare inherited disorder in which http://web.indstate.edu/thcme/mwking/fatty-acid-oxidation.html

Introduction Mobilization of Fat Stores Oxidation Reactions Alternative Oxidation Pathways ... Return to Medical Biochemistry Page Introduction Utilization of dietary lipids requires that they first be absorbed through the intestine. As these molecules are oils they would be essentially insoluble in the aqueous intestinal environment. Solubilization (emulsification) of dietary lipid is accomplished via bile salts that are synthesized in the liver and secreted from the gallbladder. The emulsified fats can then be degraded by pancreatic lipases (lipase and phospholipase A ). These enzymes, secreted into the intestine from the pancreas, generate free fatty acids and a mixtures of mono- and diacylglycerols from dietary triacylglycerols. Pancreatic lipase degrades triacylglycerols at the 1 and 3 positions sequentially to generate 1,2-diacylglycerols and 2-acylglycerols. Phospholipids are degraded at the 2 position by pancreatic phospholipase A releasing a free fatty acid and the lysophospholipid. Following absorption of the products of pancreatic lipase by the intestinal mucosal cells, the resynthesis of triacylglycerols occurs. The triacylglycerols are then solubilized in lipoprotein complexes (complexes of lipid and protein) called chylomicrons . A chylomicron contains lipid droplets surrounded by the more polar lipids and finally a layer of proteins. Triacylglycerols synthesized in the liver are packaged into VLDLs and released into the blood directly. Chylomicrons from the intestine are then released into the blood via the lymph system for delivery to the various tissues for storage or production of energy through oxidation.

90. Health Library - Allergies Asthma Back Pain Cancer Caregiver Depression Diabetes Digestive DisordersHeart Disease Kidney Disease Men s Health fatty oxidation Disorder http://12.31.13.113/library/healthguide/en-us/SelfHelp/topic.asp?hwid=shc29fod

91. One More Thought On Sudden Infant Death Syndrome -- Albers And Levy 107 (4): 809 This factor, a metabolic disorder of fatty acid oxidation notably, but notexclusively, medium-chain acyl-CoA dehydrogenase deficiency (MCADD) - is http://pediatrics.aappublications.org/cgi/content/full/107/4/809

QUICK SEARCH: [advanced] Author: Keyword(s): Year: Vol: Page: This Article Extract P Rs: Submit a response Alert me when this article is cited ... Alert me if a correction is posted Services E-mail this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal ... Download to citation manager PubMed PubMed Citation Articles by Albers, S. Articles by Levy, H. L. PEDIATRICS Vol. 107 No. 4 April 2001, pp. 809 One More Thought on Sudden Infant Death Syndrome To the Editor. The American Academy of Pediatrics (AAP) Task Force on Infant Sleep Position and Sudden Infant Death Syndrome (SIDS) has identified a number of risk factors for SIDS, including prone sleeping, SIDS among siblings, infanticide, and cardiac arrhythmias. However, one important and preventable risk factor was omitted. This factor, a metabolic disorder of fatty acid oxidation notably, but not exclusively, medium-chain acyl-CoA dehydrogenase deficiency (MCADD) is increasingly recognized as a cause of infant death frequently mistaken for SIDS and as a major cause of sudden death among siblings.

92. Obstetrical & Gynecological Survey - Abstract: Volume 54(11) November 1999 P 679 A Fetal fattyAcid oxidation Disorder as a Cause of Liver Disease in Pregnant Women.Obstetrical Gynecological Survey. 54(11)679, November 1999. http://www.obgynsurvey.com/pt/re/obgynsurv/abstract.00006254-199911000-00002.htm

LWWOnline LOGIN eALERTS REGISTER ... Archive A Fetal Fatty-Acid Oxidation Disorder... ARTICLE LINKS: Fulltext Permissions A Fetal Fatty-Acid Oxidation Disorder as a Cause of Liver Disease in Pregnant Women. Ibdah, Jamal A.; Bennett, Michael J.; Rinaldo, Piero; Zhao, Yiwen; Gibson, Beverly; Sims, Harold F.; Strauss, Arnold W. Abstract: Both acute fatty liver, a devastating illness, and the more common hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome are disorders of late pregnancy whose clinical and biochemical features overlap. Some fetuses of affected women are later found to have a deficiency of an enzyme found in the mitochondrial trifunctional protein, long-chain 3-hydroxyacyl-coenzyme A (CoA) dehydrogenase. This protein also contains the active site of long-chain 2,3-enoyl-CoA hydratase and long-chain 3-ketoacyl-CoA thiolase. This study examined the relationship between mutations in the trifunctional protein in infants with defective fatty acid oxidation and acute liver disease during pregnancy. The study included 24 families in which one child had clinical features suggesting defective fatty acid oxidation as well as an isolated deficiency of 3hydroxyacyl-CoA dehydrogenase (or complete deficiency of the trifunctional protein). These findings strongly indicate that pregnant women whose fetus has an isolated deficiency of long-chain 3hydroxyacyl-CoA dehydrogenase are at risk of lifethreatening liver disease. Testing for the Glu474Gln mutation is indicated for all women who develop acute fatty liver of pregnancy or the HELLP syndrome, as well as their partners and children.

93. Long Chain Acyl CoA Dehydrogenase Deficiency,LCAD ,Acyl-CoA deficiency is a rare genetic disorder of fatty acid metabolism (ie, fatty acidoxidation disorder) that is transmitted as an autosomal recessive trait. http://www.icomm.ca/geneinfo/lcad.htm

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