Management of acute mitochondriopathy and encephalopathy syndrome in pediatric intensive care unite: a new clinical enti
- PDF / 719,295 Bytes
- 7 Pages / 595.276 x 790.866 pts Page_size
- 34 Downloads / 190 Views
ORIGINAL ARTICLE
Management of acute mitochondriopathy and encephalopathy syndrome in pediatric intensive care unite: a new clinical entity Şükrü Arslan1 · Alaaddin Yorulmaz2 · Ahmet Sert3 · Fatih Akin4 Received: 6 January 2019 / Accepted: 12 March 2019 © Belgian Neurological Society 2019
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. Keywords Children · Encephalopathy · Mitochondrial dysfunction · Treatment protocol
Introduction Reye’s syndrome (RS) is an acute, rare, severe pediatric illness characterized by encephalopathy, cerebral edema, and fatty degeneration and infiltration of liver [1]. Although this syndrome was clinically characterized in 1963 by Reye et al., patients with these clinical signs had been sporadically described since 1929 [1, 2]. From an etiological point of view, it is suggested that there is a relationship between the use of aspirin during viral infections and Reye’s syndrome, but this point is * Alaaddin Yorulmaz [email protected] 1
Department of Pediatric Rheumatology, Selçuk University Medical School, Konya, Turkey
2
Department of Pediatrics, Selçuk University Medical School, Konya, Turkey
3
Department of Pediatric Cardiology, Selçuk University Medical School, Konya, Turkey
4
Department of Pediatrics, Meram Medical Faculty, Necmettin Erbakan University, Konya, Turkey
controversial [3]. The frequency of the syndrome was significantly reduced gradually and this decrease was attributed to the recommendation to restrict aspirin use. RS, however, had gradually disappeared from countries such as Australia, where aspirin was not used since the 1950s [4]; and France and Belgium, w
Data Loading...
