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1. rarediseases.org › rare-diseases › very-long-chainVery Long Chain Acyl CoA Dehydrogenase Deficiency (LCAD)

   rarediseases.org › rare-diseases › very-long-chain

   Mar 14, 2024 · Learn about Very Long Chain Acyl CoA Dehydrogenase Deficiency (LCAD), including symptoms, causes, and treatments. If you or a loved one is affected by this.

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3. metabolicsupportuk.org › condition › very-long-chainVery-Long Chain Acyl CoA Dehydrogenase Deficiency

   metabolicsupportuk.org › condition › very-long-chain

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   Medium Chain Triglyceride oil is often used for VLCAD patients. The oil contains medium chain fatty acids which are fats that the body of patients can break down. Be sure to talk to a doctor before using any supplements. When treated early, babies with VLCAD can have healthy growth and development.

4. rarediseases.info.nih.gov › diseases › 5508Very long chain acyl-CoA dehydrogenase deficiency

   rarediseases.info.nih.gov › diseases › 5508

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   Very long chain acyl-CoA dehydrogenase deficiency (VLCAD deficiency) is a condition in which the body is unable to properly breakdown certain fats (called very long-chain fatty acids) into energy, particularly during periods without food (fasting). Signs and symptoms may include low blood sugar (hypoglycemia), lack of energy, and muscle weakness.

5. www.ncbi.nlm.nih.gov › sites › booksVery Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency

   www.ncbi.nlm.nih.gov › sites › books

   May 28, 2009 · Depending on the severity of very long-chain acyl-coenzyme A dehydrogenase (VLCAD) deficiency, individuals can present with hypoketotic hypoglycemia, hepatomegaly, cardiomyopathy, and myopathy with recurrent rhabdomyolysis, triggered by a catabolic state.

6. cptraininghub.nhs.uk › event › patient-safety-andPatient safety and LCAD: how to prevent follow-up patients ...

   cptraininghub.nhs.uk › event › patient-safety-and

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   Oct 7, 2022 · Learn more about using the Latest Clinically Appropriate Date (LCAD) and Earliest Clinically Appropriate Date (ECAD) data fields to prevent avoidable patient harm.

7. www.jbc.org › article › S0021-9258(20)40331-XLong-chain Acyl-CoA Dehydrogenase Deficiency as a Cause of ...

   www.jbc.org › article › S0021-9258(20)40331-X

   Long-chain acyl-CoA dehydrogenase (LCAD) is a mitochondrial fatty acid oxidation enzyme whose expression in humans is low or absent in organs known to utilize fatty acids for energy such as heart, muscle, and liver. This study demonstrates localization of LCAD to human alveolar type II pneumocytes, which synthesize and secrete pulmonary surfactant.

8. People also ask

   What does LCAD stand for?

   • Very Long Chain Acyl CoA Dehydrogenase Deficiency (LCAD) NORD gratefully acknowledges Jerry Vockley, MD, PhD, University of Pittsburgh, Chief of Medical Genetics, Children’s Hospital of Pittsburgh of UPMC, for assistance in the preparation of this report. NORD strives to open new assistance programs as funding allows.

   Very Long Chain Acyl CoA Dehydrogenase Deficiency (LCAD)

   rarediseases.org/rare-diseases/very-long-chain-acyl-coa-dehydrogenase-deficiency-lcad/

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   Does LCAD play a role in fatty acid metabolism?

   • Similarly, based on microarray studies in lung tumors, LCAD was included in a core set of genes whose expression levels distinguish cancerous from normal tissue (13). These reports prompted us to hypothesize that LCAD may play a role in fatty acid metabolism specific to lung. In this study, we confirmed localization of LCAD to human ATII cells.

   Long-chain Acyl-CoA Dehydrogenase Deficiency as a Cause

   www.jbc.org/article/S0021-9258(20)40331-X/fulltext

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   Does LCAD dehydrogenate branched-chain acyl-CoA substrates?

   • Additionally, LCAD has been shown to dehydrogenate branched-chain acyl-CoA substrates (5). VLCAD expression predominates in tissues that are known to rely upon fatty acids for energy such as liver, heart, and muscle. Correspondingly, VLCAD deficiency causes life-threatening metabolic decompensation, cardiomyopathy, and muscle weakness (6, 7).

   Long-chain Acyl-CoA Dehydrogenase Deficiency as a Cause

   www.jbc.org/article/S0021-9258(20)40331-X/fulltext

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   Is LCAD deficiency a lung disease?

   • We further observed surfactant deficiency and altered lung mechanics in LCAD-deficient mice. Based on these findings, it is postulated that LCAD deficiency in humans may manifest primarily as a lung disease rather than resembling other FAO disorders.

   Long-chain Acyl-CoA Dehydrogenase Deficiency as a Cause

   www.jbc.org/article/S0021-9258(20)40331-X/fulltext

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   Does LCAD play a role in human lung pathophysiology?

   • We postulate that LCAD may play a role in human lung pathophysiology. Disorders of fatty acid metabolism have previously been linked to sudden infant death syndrome (45). We used Western blotting to evaluate LCAD antigen in samples of lung from six infants who suffered unexplained deaths under the age of 1 year.

   Long-chain Acyl-CoA Dehydrogenase Deficiency as a Cause

   www.jbc.org/article/S0021-9258(20)40331-X/fulltext

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   What does VLCAD stand for?

   • Nutrition management guideline for very-long chain acyl-CoA dehydrogenase deficiency (VLCAD): an evidence- and consensus-based approach. Mol Genet Metab. 2020;131:23-37. [ PubMed] Vellekoop P, Diekman EF, van Tuijl I, de Vries MMC, van Hasselt, PM, Visser G (2011) Perioperative measures in very long chain acyl-CoA dehydrogenase deficiency.

   Very Long-Chain Acyl-Coenzyme A Dehydrogenase Deficiency

   www.ncbi.nlm.nih.gov/sites/books/NBK6816/

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9. en.wikipedia.org › wiki › Very_long-chain_acylVery long-chain acyl-coenzyme A dehydrogenase deficiency

   en.wikipedia.org › wiki › Very_long-chain_acyl

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   Treatment and management of VLCAD deficiency involve dietary restrictions as well as implementation of proper hydration to avoid further complications. Hospitalization due to VLCAD deficiency can be treated with intravenous (IV) glucose for hydration and alkalization of urine and prevention of renal malfunction or failure. [10]