Neuromonitoring in neonatal intensive care units-an important need towards individualized neuroprotective care.

Neuromonitoring has been widely accepted as an important part in neonatal care. Amplitude-integrated EEG (aEEG) and near-infrared spectroscopy (NIRS) are often mentioned in this context, though being only a part of the fully array of methods and examinations that could be considered neuromonitoring. Within the broad array of medical conditions that could be encountered in a neonatal patient, it is important to be aware of the indications for neuromonitoring and especially which neuromonitoring technique to use best for the individual condition. aEEG is now a widely accepted neuromonitor in neonatology with its value in hypoxic events and seizures only rarely questioned. Other methods like NIRS still have to prove themselves in the future. The SafeBoosC-III trial showed that it still remains difficult for some of these methods to prove their value for the improvement of outcome. Bute future developments such as multimodal neuromonitoring with data integration and artificial intelligence analysis could improve the value of these methods.

A Complex Intervention to Prevent Medication-Related Hospital Admissions.

BACKGROUND: Children are often treated off-label and are at a disadvantage in pharmacotherapy. The aim of this study was to implement and evaluate a quality assurance measure (PaedPharm) for pediatric pharmacotherapy whose purpose is to reduce medication-related hospitalizations among children and adolescents. METHODS: PaedPharm consisted of the digital pediatric drug information system PaedAMIS, pediatric pharmaceutical quality circles (PaedZirk), and an adverse drug event (ADE) reporting system (PaedReport). The intervention was implemented in a cluster-randomized trial (DRKS 00013924) in 12 regions, with a pediatric and adolescent medicine clinic in each and a total of 152 surrounding private practitioners, in 6 sequences over 8 quarters. In addition to the proportion of ADE-related hospital admissions (primary endpoint), comprehensive process evaluation included other endpoints such as coverage, user acceptance, and relevance to practice. RESULTS: 41 829 inpatient admissions were recorded, of which 5101 were patients of physicians who participated in our study. 4.1% of admissions were ADE-related under control conditions, and 3.1% under intervention conditions (95% CI: [2.3; 5.9] and [1.8; 4.5], respectively). A model-based comparison yielded an intervention effect of 0.73 (population-based odds ratio; [0.39; 1.37]; p = 0.33). PaedAMIS achieved moderate user acceptance and PaedZirk achieved high user acceptance. CONCLUSION: The introduction of PaedPharm was associated with a decrease in medication-related hospitalizations that did not reach statistical significance. The process evaluation revealed broad acceptance of the intervention in outpatient pediatrics and adolescent medicine.

Case Report: DPM1-CDG: Novel Variant with Severe Phenotype and Literature Review.

Background: Congenital disorders of glycosylation (CDG) type I include variants in the DPM1 gene leading to DPM1-CDG. The nine previously reported patients showed developmental delay, seizures, electroencephalography abnormalities and dysmorphic features with varying disease onset and severity. Methods: Clinical features of a new patient are described. Whole exome sequencing using NGS was performed, followed by molecular simulation of the structural changes in the protein. Results: Our patient with DPM1-CDG presented with more severe symptoms and an earlier onset, specifically non-febrile seizures from the age of 3 weeks, global developmental delay, and severely retarded motor skills. She died at the age of 11 weeks after fulminant sepsis. We identified compound heterozygous variants in the DPM1 gene, one previously reported point mutation c.1A > C p.? as well as the novel variant c.239_241del p.(Lys80del), resulting in the first in-frame deletion located in exon 2. Loss of Lys80 may lead to an impaired α-helical configuration next to the GDP/GTP binding site. Conclusion: The presented case extends the spectrum of DPM1-CDG to a very young and severely affected child. The deletion of Lys80 in DPM1 results in an impaired helical configuration. This has implications for further understanding the association of structure and function of DPM1.

Guiding Antiepileptic Therapy in a Pediatric Patient with Severe Meningoencephalitis and Decompressive Craniectomy with the Use of Amplitude-Integrated Electroencephalography.

Introduction Amplitude-integrated electroencephalography (aEEG) is one of the most widely used neuromonitoring tools in neonatology today. However, little is known about its clinical indications and potential benefits in pediatric intensive care patients. Based on limited experience, its impact on therapeutic decision-making in this patient population is unclear. Case Description We report the case of a 16-year-old boy who, after a pansinusitis, developed a severe meningoencephalitis and intracranial empyema with increased intracranial pressure that required drainage and decompressive craniectomy. He subsequently developed status epilepticus despite a combination of various anticonvulsants. Only after the initialization of an aEEG, we were able to adequately diagnose and continuously monitor his seizure activity and titrate the effect of the antiepileptic drugs. During his hospital stay, we were able to clearly monitor and guide our therapy by accurately identifying the termination of status epilepticus and the recurrence of seizures. Discussion With the help of aEEG, it was easy to identify the nonconvulsive status epilepticus (NCSE) and the ongoing seizure activity in this teenage patient. NCSE is a clinical problem with an effect on the outcome of the patient and is often underdiagnosed. AEEG enabled a rapid detection and management of seizure activity and thereby reduced the overall seizure burden, which was associated with better neurologic outcome.