Matches in SemOpenAlex for { <https://semopenalex.org/work/W1574255071> ?p ?o ?g. }
Showing items 1 to 85 of
85
with 100 items per page.
- W1574255071 endingPage "131" @default.
- W1574255071 startingPage "123" @default.
- W1574255071 abstract "Current dietary management of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD; long-chain-(S)-3-hydroxyacyl-CoA:NAD+ oxido-reductase, EC 1.1.1.211) deficiency (LCHADD) is based on avoiding fasting, and minimizing energy production from long-chain fatty acids. We report the effects of various dietary manipulations on plasma and urinary laboratory values in a child with LCHADD. In our patient, a diet restricted to 9% of total energy from long-chain fatty acids and administration of 1.5 g medium-chain triglyceride oil per kg body weight normalized plasma acylcarnitine and lactate levels, but dicarboxylic acid excretion remained approximately ten times normal. Plasma docosahexaenoic acid (DHA, 22:6n-3) was consistently low over a 2-year period; DHA deficiency may be related to the development of pigmentary retinopathy seen in this patient population. We also conducted a survey of metabolic physicians who treat children with LCHADD to determine current dietary interventions employed and the effects of these interventions on symptoms of this disease. Survey results indicate that a diet low in long-chain fatty acids, supplemented with medium-chain triclyceride oil, decreased the incidence of hypoketotic hypoglycaemia, and improved hypotonia, hepatomegaly, cardiomyopathy, and lactic acidosis. However, dietary treatment did not appear to effect peripheral neuropathy, pigmentary retinopathy or myoglobinuria." @default.
- W1574255071 created "2016-06-24" @default.
- W1574255071 creator A5000032345 @default.
- W1574255071 creator A5014486492 @default.
- W1574255071 creator A5029737903 @default.
- W1574255071 creator A5072550388 @default.
- W1574255071 creator A5078185313 @default.
- W1574255071 date "1999-04-01" @default.
- W1574255071 modified "2023-09-22" @default.
- W1574255071 title "Dietary management of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD). A case report and survey" @default.
- W1574255071 cites W1516102547 @default.
- W1574255071 cites W1841137819 @default.
- W1574255071 cites W1926187054 @default.
- W1574255071 cites W1979660645 @default.
- W1574255071 cites W1996908614 @default.
- W1574255071 cites W2003661489 @default.
- W1574255071 cites W2011826268 @default.
- W1574255071 cites W2019741751 @default.
- W1574255071 cites W2163645162 @default.
- W1574255071 cites W2168168825 @default.
- W1574255071 cites W273463978 @default.
- W1574255071 doi "https://doi.org/10.1023/a:1005437616934" @default.
- W1574255071 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/2694044" @default.
- W1574255071 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/10234607" @default.
- W1574255071 hasPublicationYear "1999" @default.
- W1574255071 type Work @default.
- W1574255071 sameAs 1574255071 @default.
- W1574255071 citedByCount "72" @default.
- W1574255071 countsByYear W15742550712012 @default.
- W1574255071 countsByYear W15742550712013 @default.
- W1574255071 countsByYear W15742550712014 @default.
- W1574255071 countsByYear W15742550712015 @default.
- W1574255071 countsByYear W15742550712016 @default.
- W1574255071 countsByYear W15742550712018 @default.
- W1574255071 countsByYear W15742550712019 @default.
- W1574255071 countsByYear W15742550712021 @default.
- W1574255071 countsByYear W15742550712022 @default.
- W1574255071 countsByYear W15742550712023 @default.
- W1574255071 crossrefType "journal-article" @default.
- W1574255071 hasAuthorship W1574255071A5000032345 @default.
- W1574255071 hasAuthorship W1574255071A5014486492 @default.
- W1574255071 hasAuthorship W1574255071A5029737903 @default.
- W1574255071 hasAuthorship W1574255071A5072550388 @default.
- W1574255071 hasAuthorship W1574255071A5078185313 @default.
- W1574255071 hasBestOaLocation W15742550712 @default.
- W1574255071 hasConcept C126322002 @default.
- W1574255071 hasConcept C134018914 @default.
- W1574255071 hasConcept C2778163477 @default.
- W1574255071 hasConcept C2778913445 @default.
- W1574255071 hasConcept C2779014545 @default.
- W1574255071 hasConcept C2908647359 @default.
- W1574255071 hasConcept C71924100 @default.
- W1574255071 hasConcept C99454951 @default.
- W1574255071 hasConceptScore W1574255071C126322002 @default.
- W1574255071 hasConceptScore W1574255071C134018914 @default.
- W1574255071 hasConceptScore W1574255071C2778163477 @default.
- W1574255071 hasConceptScore W1574255071C2778913445 @default.
- W1574255071 hasConceptScore W1574255071C2779014545 @default.
- W1574255071 hasConceptScore W1574255071C2908647359 @default.
- W1574255071 hasConceptScore W1574255071C71924100 @default.
- W1574255071 hasConceptScore W1574255071C99454951 @default.
- W1574255071 hasIssue "2" @default.
- W1574255071 hasLocation W15742550711 @default.
- W1574255071 hasLocation W15742550712 @default.
- W1574255071 hasLocation W15742550713 @default.
- W1574255071 hasLocation W15742550714 @default.
- W1574255071 hasOpenAccess W1574255071 @default.
- W1574255071 hasPrimaryLocation W15742550711 @default.
- W1574255071 hasRelatedWork W1969808273 @default.
- W1574255071 hasRelatedWork W1993873099 @default.
- W1574255071 hasRelatedWork W2013998010 @default.
- W1574255071 hasRelatedWork W2023333646 @default.
- W1574255071 hasRelatedWork W2028937980 @default.
- W1574255071 hasRelatedWork W2035484352 @default.
- W1574255071 hasRelatedWork W2151578115 @default.
- W1574255071 hasRelatedWork W2162912823 @default.
- W1574255071 hasRelatedWork W2350174576 @default.
- W1574255071 hasRelatedWork W2418447797 @default.
- W1574255071 hasVolume "22" @default.
- W1574255071 isParatext "false" @default.
- W1574255071 isRetracted "false" @default.
- W1574255071 magId "1574255071" @default.
- W1574255071 workType "article" @default.