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LCHAD dehydrogenase deficiency

The long chain defects, very long-chain acyl-CoA dehydrogenase deficiency (VLCAD) and long-chain 3-hydroxacyl-CoA dehydrogenase deficiency (LCHAD), generally give a low P/M ratio. The mean P/M ratio for LCHAD is 0.60 0.07 (n = 18) and... [Pg.321]

Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD)... [Pg.21]

Table 14.8A,B. Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD-a and LCHAD-j ) (>100 patients) ... Table 14.8A,B. Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD-a and LCHAD-j ) (>100 patients) ...
LCHAD deficiency 600890 Long-chain 3 -hydroxyacyl-Co A dehydrogenase >1 75,000 Cardiomyopathy, liver disease, retinopathy, peripheral neuropathy AFLP, HELLP syndrome, preeclampsia, placental floor infarction... [Pg.2234]

Associated biochemical defect Fetal deficiencies of long chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD). [Pg.180]

As noted above, there have been reports that link some cases of APLP with a defect in fatty acid metabolism in the fetus. These include fetal deficiencies of long chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD), carnitine-palmitoyl transferase 1 (CPT 1), and medium chain acyl-coenzyme A dehydrogenase (MCAD). The mechanism by which defective fetal fatty acid oxidation causes maternal illness is not known. However, since the fetus uses primarily glucose metabolism for its energy needs, it is likely that toxic products from the placenta, which does use fatty acid oxidation, cause the maternal liver failure. [Pg.185]

Gutierrez Junquera C, et al. Acute fatty liver of pregnancy and neonatal long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency. Eur J Pediatr. 2009 168(l) 103-6. [Pg.58]

Long-Chain 3-Hydroxyacyl-Coenzyme A Dehydrogenase (LCHAD) Deficiency... [Pg.241]

Fig. 23.1 Body composition and lipid deposition. Data are presented as means SD. (a) There is a trend for long-chain 3-hydroxy acyl-CoA dehydrogenase (LCHAD)-deficient patients (n = 9 closed bars) to have less fat-free mass and more fat mass compared with control subjects (n = 9 open bars) when expressed as %body mass, (b) There was no difference in fat-free or fat mass expressed as mass/surface area between groups, (c) There was a trend for LCHAD-deficient patients (n = 9 closed bars) to have more extramyocellular Upid EMCL) but no difference in intramyocellular lipid IMCL) compared with... Fig. 23.1 Body composition and lipid deposition. Data are presented as means SD. (a) There is a trend for long-chain 3-hydroxy acyl-CoA dehydrogenase (LCHAD)-deficient patients (n = 9 closed bars) to have less fat-free mass and more fat mass compared with control subjects (n = 9 open bars) when expressed as %body mass, (b) There was no difference in fat-free or fat mass expressed as mass/surface area between groups, (c) There was a trend for LCHAD-deficient patients (n = 9 closed bars) to have more extramyocellular Upid EMCL) but no difference in intramyocellular lipid IMCL) compared with...
Gillingham MB, et al. Metabolic control during exercise with and without medium-chain triglycerides (MCT) in children with long-chain 3-hydroxy acyl-CoA dehydrogenase (LCHAD) or trifunctional protein (TFP) deficiency. Mol Genet Metab. 2006 89(l-2) 58-63. [Pg.282]

See other pages where LCHAD dehydrogenase deficiency is mentioned: [Pg.701]    [Pg.174]    [Pg.189]    [Pg.2232]    [Pg.184]    [Pg.805]    [Pg.2245]    [Pg.180]    [Pg.349]    [Pg.332]    [Pg.40]    [Pg.245]    [Pg.253]    [Pg.254]    [Pg.270]    [Pg.275]    [Pg.311]    [Pg.332]   


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