(Q72801663)

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29 October 2019

title

Long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency--diagnosis, plasma carnitine fractions and management in a further patient (English)

1 reference

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long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency

29 October 2019

main subject

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author name string

R Moore

1

1 reference

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29 October 2019

J F Glasgow

2

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29 October 2019

M A Bingham

3

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29 October 2019

J A Dodge

4

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29 October 2019

R J Pollitt

5

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29 October 2019

S E Olpin

6

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29 October 2019

B Middleton

7

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29 October 2019

K Carpenter

8

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29 October 2019

publication date

1 May 1993

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European Journal of Pediatrics

29 October 2019

published in

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29 October 2019

volume

152

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29 October 2019

issue

5

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29 October 2019

page(s)

433-436

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https://scigraph.springernature.com/pub.10.1007/bf01955905

29 October 2019

exact match

0 references

cites work

3-Hydroxydicarboxylic aciduria due to long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency associated with sudden neonatal death: protective effect of medium-chain triglyceride treatment

1 reference

12 December 2020

The existence of an inner-membrane-bound, long acyl-chain-specific 3-hydroxyacyl-CoA dehydrogenase in mammalian mitochondria

1 reference

12 December 2020

Sensitive assay of carnitine palmitoyl transferase activity in tissue homogenates with a modified spectrophotometric method for enzymatic carnitine determination

1 reference

12 December 2020

Systemic carnitine deficiency. Report of a fatal case with multisystemic manifestations

1 reference

12 December 2020

Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency: identification of a new inborn error of mitochondrial fatty acid beta-oxidation

1 reference

12 December 2020

The use of phenylpropionic acid as a loading test for medium-chain acyl-CoA dehydrogenase deficiency

1 reference

12 December 2020

A simple screening test for medium-chain acyl CoA dehydrogenase deficiency

1 reference

12 December 2020

Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency

1 reference

12 December 2020

Progressive neuropathy and recurrent myoglobinuria in a child with long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency

1 reference

12 December 2020

Effects of ciprofibrate and 2-[5-(4-chlorophenyl)pentyl]oxirane-2-carboxylate (POCA) on the distribution of carnitine and CoA and their acyl-esters and on enzyme activities in rats. Relation between hepatic carnitine concentration and carnitine acet

1 reference

12 December 2020

Fluorometric assay of acyl-CoA dehydrogenases in normal and mutant human fibroblasts

1 reference

12 December 2020

Long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency

1 reference

12 December 2020

Purification and properties of mitochondrial and peroxisomal 3-hydroxyacyl-CoA dehydrogenase from rat liver

1 reference

12 December 2020

A comparison of [9,10-3H]palmitic and [9,10-3H]myristic acids for the detection of defects of fatty acid oxidation in intact cultured fibroblasts

1 reference

12 December 2020

The laboratory diagnosis of inborn errors of mitochondrial fatty acid oxidation

1 reference

12 December 2020

Cis-4-decenoic acid in plasma: a characteristic metabolite in medium-chain acyl-CoA dehydrogenase deficiency

1 reference

12 December 2020

Identification of phenylpropionylcarnitine, a new metabolite of phenylpropionic acid, in a patient with medium chain acyl-CoA dehydrogenase deficiency

1 reference

12 December 2020

Deficiency of long-chain 3-hydroxyacyl-CoA dehydrogenase: a cause of lethal myopathy and cardiomyopathy in early childhood

1 reference

12 December 2020

Possible deleterious effect ofL-carnitine supplementation in a patient with mild multiple acyl-CoA dehydrogenation deficiency (ethylmalonic-adipic aciduria)

1 reference

12 December 2020

Hypoglycemia, hepatic dysfunction, muscle weakness, cardiomyopathy, free carnitine deficiency and long-chain acylcarnitine excess responsive to medium chain triglyceride diet.

1 reference

12 December 2020

Urinary C6-C12 dicarboxylic acylcarnitines in Reye's syndrome

1 reference

12 December 2020

3-hydroxydecanedioic acid and related homologues: urinary metabolites in ketoacidosis

1 reference

12 December 2020

Identifiers

DOI

10.1007/BF01955905

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:8319713%20AND%20SRC:MED&resulttype=core&format=json

29 October 2019

1 reference