Publications by authors named "E Tschiedel"

Objective: This study is an addition to the already published prospective randomized double-blinded trial by Tschiedel et al. that compared two different sedation regimes in fiberoptic flexible bronchoscopy in pediatric subjects. The objective of the presented study is to analyze the correlation between the neutrophil percentage of the bronchoalveolar lavage fluid (BALF) and coughing episodes during bronchoscopy.

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Precise assessment of hypnotic depth in children during procedural sedation with preserved spontaneous breathing is challenging. The Narcotrendindex (NI) offers uninterrupted information by continuous electrocortical monitoring without the need to apply a stimulus with the risk of assessment-induced arousal. This study aimed to explore the correlation between NI and the Comfort Scale (CS) during procedural sedation with midazolam and propofol and to identify an NI target range for deep sedation.

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Background/purpose: MEGDHEL syndrome is a rare autosomal recessive metabolic disorder, which is characterized by 3-methylglutaconic aciduria with deafness-dystonia, hepatopathy, encephalopathy and Leigh-like syndrome. It is caused by biallelic pathogenic variants in the gene. Due to the unspecific symptoms and the diverse manifestations of the clinical phenotype, the diagnosis is challenging.

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Article Synopsis
  • - Pediatric acute liver failure (PALF) is a serious condition with up to 50% of cases remaining unexplained, hindering effective treatment options like liver transplantation.
  • - In a study involving 260 children from 19 countries, whole-exome sequencing (WES) identified genetic causes in 37% of indeterminate PALF cases, with a particularly high diagnostic rate in infants and those with recurrent liver failure.
  • - The research uncovered 36 distinct genes associated with PALF, highlighting mitochondrial diseases as the most common cause and underscoring the need for advanced genetic testing in diagnosing and treating this condition.
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Article Synopsis
  • Mycobacterium chimaera is a non-tuberculous mycobacterium linked to difficult infections in cystic fibrosis patients, primarily affecting the lungs and rare in other sites unless there's immune deficiency.
  • A case study of an 8-year-old cystic fibrosis patient showed recurrent pulmonary issues and a confirmed M. chimaera infection in a central venous port, diagnosed using next-generation sequencing.
  • The patient's recovery highlights the need for catheter removal and targeted therapy as key steps in treating MAC infections, underscoring the importance of biofilm formation in such cases.
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