Literature DB >> 3122912

Acylcoenzyme A dehydrogenase deficiency in heart tissue from infants who died unexpectedly with fatty change in the liver.

F Allison1, M J Bennett, S Variend, P C Engel.   

Abstract

Heart muscle from infants who died unexpectedly and who showed fatty changes in the liver at necropsy was analysed for long chain and medium chain acylcoenzyme A dehydrogenase activities by using the natural electron acceptor. In two of the seven cases investigated a deficiency in acylcoenzyme A dehydrogenase activity was found. In one case the deficiency was in medium chain acylcoenzyme A dehydrogenase activity and in the other long chain acylcoenzyme A dehydrogenase activity. These findings emphasise the importance of investigating fatty acid oxidation in infants who have died unexpectedly.

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Year:  1988        PMID： 3122912      PMCID： PMC2544643          DOI： 10.1136/bmj.296.6614.11

Source DB:  PubMed          Journal:  Br Med J (Clin Res Ed)        ISSN： 0267-0623


  5 in total

1.  Protein measurement with the Folin phenol reagent.

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2.  Inherited disorders of straight chain fatty acid oxidation.

Authors:  R J Pollitt
Journal:  Arch Dis Child       Date:  1987-01       Impact factor: 3.791

3.  Sudden infant death and inherited disorders of fat oxidation.

Authors: 
Journal:  Lancet       Date:  1986-11-08       Impact factor: 79.321

4.  Genetic deficiency of medium-chain acyl coenzyme A dehydrogenase: studies in cultured skin fibroblasts and peripheral mononuclear leukocytes.

Authors:  P M Coates; D E Hale; C A Stanley; B E Corkey; J A Cortner
Journal:  Pediatr Res       Date:  1985-07       Impact factor: 3.756

5.  Glutaric aciduria type II: treatment with riboflavine, carnitine and insulin.

Authors:  P D Mooy; H Przyrembel; M A Giesberts; H R Scholte; W Blom; H H van Gelderen
Journal:  Eur J Pediatr       Date:  1984-12       Impact factor: 3.183
  5 in total
  10 in total

1.  Sudden unexpected death and covert homicide in infancy.

Authors:  S Levene; C J Bacon
Journal:  Arch Dis Child       Date:  2004-05       Impact factor: 3.791

2.  Acquired deficiency of long-chain acyl-CoA dehydrogenase in liver: a cautionary tale.

Authors:  F Allison; M J Bennett; R J Pollitt; S Variend
Journal:  J Inherit Metab Dis       Date:  1990       Impact factor: 4.982

Review 3.  Primary lipid cardiomyopathy.

Authors:  A Zimmermann; P Wyss; F Stocker
Journal:  Virchows Arch A Pathol Anat Histopathol       Date:  1990

4.  Abnormal expression of glucose-6-phosphatase in preterm infants.

Authors:  R Hume; A Burchell
Journal:  Arch Dis Child       Date:  1993-02       Impact factor: 3.791

5.  Analysis of abnormal urinary metabolites in the newborn period in medium-chain acyl-CoA dehydrogenase deficiency.

Authors:  M J Bennett; P M Coates; D E Hale; D S Millington; R J Pollitt; P Rinaldo; C R Roe; K Tanaka
Journal:  J Inherit Metab Dis       Date:  1990       Impact factor: 4.982

Review 6.  Disorders of mitochondrial beta-oxidation: prenatal and early postnatal diagnosis and their relevance to Reye's syndrome and sudden infant death.

Authors:  R J Pollitt
Journal:  J Inherit Metab Dis       Date:  1989       Impact factor: 4.982

Review 7.  Medium-chain acyl-CoA dehydrogenase deficiency: molecular aspects.

Authors:  Y Matsubara; K Narisawa; K Tada
Journal:  Eur J Pediatr       Date:  1992-03       Impact factor: 3.183

8.  Medium-chain acyl-CoA dehydrogenase deficiency does not correlate with apparent life-threatening events and the sudden infant death syndrome: results from phenylpropionate loading tests and DNA analysis.

Authors:  J M Penzien; G Molz; U N Wiesmann; J P Colombo; R Bühlmann; B Wermuth
Journal:  Eur J Pediatr       Date:  1994-05       Impact factor: 3.183

9.  Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients.

Authors:  T Aoyama; M Souri; S Ushikubo; T Kamijo; S Yamaguchi; R I Kelley; W J Rhead; K Uetake; K Tanaka; T Hashimoto
Journal:  J Clin Invest       Date:  1995-06       Impact factor: 14.808

Review 10.  Medium chain acyl-CoA dehydrogenase deficiency.

Authors:  E H Touma; C Charpentier
Journal:  Arch Dis Child       Date:  1992-01       Impact factor: 3.791
  10 in total

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