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Acute, severe cardiomyopathy as main symptom of late-onset very long-chain acyl-coenzyme A dehydrogenase deficiency

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Abstract

A 5-year-old boy with late-onset very long-chain acyl-CoA-dehydrogenase (VLCAD) deficiency presented with acute cardiomyopathy, myopathy, gross myoglobinuria and normoglycaemia. The clinical course after diagnosis was favourable.

Conclusion late-onset VLCAD deficiency may present as acute cardiomyopathy.

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Authors and Affiliations

  1. Laboratorio di Biochimica e Genetica, Istituto Neurologico “C. Besta”, Milano, Italy, , , , , , IT

    B. Garavaglia, E. Lamantea & M. Rimoldi

  2. Sezione di Cardiologia Pediatrica, Istituti Clinici di Perfezionamento, Milano, Italy, , , , , , IT

    V. Fesslova

  3. Laboratorio di Biochimica-Sezione Malattie Metaboliche, Istituti Clinici di Perfezionamento, Milano, Italy, , , , , , IT

    D. Melotti

  4. Unità di Terapia Intensiva e Rianimazione, Istituto “G. Gaslini”, Genova Quarto, Italy, , , , , , IT

    M. L. Massone

  5. Servizio di Igiene Mentale dell' Età Evolutiva (SIMEE), Zona 1, Milano, Italy, , , , , , IT

    P. Vizziello

  6. Divisione di Medicina III, Istituto “G. Gaslini”, Genova Quarto, Italy, , , , , , IT

    R. Gatti

  7. Clinica Pediatrica II, Istituti Clinici di Perfezionamento, Via Commenda 9, I-20122 Milano, Italy, e-mail: Rossella.Parini@unimi.it, , , , , , IT

    R. Parini & F. Menni

Authors
  1. R. Parini
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  2. F. Menni
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  3. B. Garavaglia
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  4. V. Fesslova
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  5. D. Melotti
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  6. M. L. Massone
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  7. E. Lamantea
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  8. M. Rimoldi
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  9. P. Vizziello
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  10. R. Gatti
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Additional information

Received: 23 September 1997 / Accepted in revised form: 27 March 1998

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Parini, R., Menni, F., Garavaglia, B. et al. Acute, severe cardiomyopathy as main symptom of late-onset very long-chain acyl-coenzyme A dehydrogenase deficiency. Eur J Pediatr 157, 992–995 (1998). https://doi.org/10.1007/s004310050984

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  • DOI: https://doi.org/10.1007/s004310050984

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