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Management of acute mitochondriopathy and encephalopathy syndrome in pediatric intensive care unite: a new clinical entity

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Abstract

Acute mitochondriopathy and encephalopathy syndrome (AMES) is described differently by different authors in the literature. As a new clinical entity, we aimed to present the clinical signs and symptoms, diagnosis and treatment algorithm of our patients with AMES. 56 patients aged between 2 months and 18 years who were followed up in pediatric intensive care units of Konya Training and Research Hospital and Selcuk University Medical Faculty Hospital, between January 2010 and June 2017 were included. Patients’ data were obtained retrospectively from the intensive care unit patient files. 34 (60.7%) of the patients were male and 22 (39.3%) were female. The median age of our patients was 10.0 months. At the time of admission, 42 (75%) of the patients had fever, 35 (62.5%) vomiting, 27 (48.2%) abnormal behaviour and agitation and 28 (50%) convulsion. The etiological classification of patients with AMES was divided into four groups as infection, metabolic disorder, toxic, and hypoxic-ischemic. 39 (69.6%) patients were found to have infection, 10 (17.9%) patients hypoxia, 7 (12.5%) patients metabolic disorders. AMES occurs rarely, but should be kept in mind in the differential diagnosis of patients with any encephalopathy of unknown origin especially in those with a history of ingestion of drugs, previous viral infection and vomiting. Early recognition and treatment is imperative to reduce morbidity and mortality in children with AMES.

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Authors and Affiliations

  1. Department of Pediatric Rheumatology, Selçuk University Medical School, Konya, Turkey

    Şükrü Arslan

  2. Department of Pediatrics, Selçuk University Medical School, Konya, Turkey

    Alaaddin Yorulmaz

  3. Department of Pediatric Cardiology, Selçuk University Medical School, Konya, Turkey

    Ahmet Sert

  4. Department of Pediatrics, Meram Medical Faculty, Necmettin Erbakan University, Konya, Turkey

    Fatih Akin

Authors
  1. Şükrü Arslan
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  2. Alaaddin Yorulmaz
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  3. Ahmet Sert
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  4. Fatih Akin
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Contributions

All authors contributed to revising critically the final version of the manuscript. Interpretation of data and revising of manuscript: ŞA, FA. Study conception and design: AY. Preparation of manuscript: AY, FA. Literature review: ŞA, Review and supervision of manuscript: AS.

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Correspondence to Alaaddin Yorulmaz.

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The ethics committee approval for the study was received from the ethical committee of Selcuk University (2018/01).

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Informed consent was obtained from all individual participants included in the study.

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Arslan, Ş., Yorulmaz, A., Sert, A. et al. Management of acute mitochondriopathy and encephalopathy syndrome in pediatric intensive care unite: a new clinical entity. Acta Neurol Belg 120, 1115–1121 (2020). https://doi.org/10.1007/s13760-019-01125-3

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  • DOI: https://doi.org/10.1007/s13760-019-01125-3

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