(1) Background: To improve the quality of emergency care for children, the Hessian Ministry for Social Affairs and Integration offered paediatric simulation-based training (SBT) for all children’s hospitals in Hesse. We investigated the quality of paediatric life support (PLS) in simulated paediatric resuscitations before and after SBT. (2) Methods: In 2017, a standardised, high-fidelity, two-day in-house SBT was conducted in 11 children’s hospitals. Before and after SBT, interprofessional teams participated in two study scenarios (PRE and POST) that followed the same clinical course of apnoea and cardiac arrest with a shockable rhythm. The quality of PLS was assessed using a performance evaluation checklist. (3) Results: 179 nurses and physicians participated, forming 47 PRE and 46 POST interprofessional teams. Ventilation was always initiated. Before SBT, chest compressions (CC) were initiated by 87%, and defibrillation by 60% of teams. After SBT, all teams initiated CC (p = 0.012), and 80% defibrillated the patient (p = 0.028). The time to initiate CC decreased significantly (PRE 123 ± 11 s, POST 76 ± 85 s, p = 0.030). (4) Conclusions: The quality of PLS in simulated paediatric cardiac arrests with shockable rhythm was poor in Hessian children’s hospitals and improved significantly after SBT. To improve children’s outcomes, SBT should be mandatory for paediatric staff and concentrate on the management of shockable rhythms.

We report a case of an otherwise healthy 14-year-old girl with febrile infection-related epilepsy syndrome (FIRES), multiple organ failure (MOF), and ultimately a lethal outcome. This is a rare case of FIRES with MOF and consecutive death. Only a few cases have been described in the literature. The adolescent girl was initially admitted to our pediatric emergency department with a first episode of generalized tonic–clonic seizures after a short history of fever a week before admission. Seizures progressed rapidly into refractory status epilepticus without any evidence of the underlying cause, and treatment subsequently had to be escalated to thiopental anesthesia. Since the initial diagnostics showed no promising leads, the rare syndrome of FIRES was suspected, representing a catastrophic epileptic encephalopathy linked to a prior benign febrile infection. Methylprednisolone, intravenous immunoglobulins, and a ketogenic diet were initiated. Respiratory, circulatory, kidney, and liver failure developed during treatment, requiring increasing intensive care. Multiple attempts to deescalate antiepileptic treatment resulted in recurrent status epilepticus. A cranial MRI on the 10th day of treatment revealed diffuse brain edema and no cerebral perfusion. The patient was declared dead on the 11th day of treatment. FIRES should be taken into account in previously healthy children with a new onset of difficult-to-treat seizures after a short febrile infection when no other cause is apparent. First-line treatment, besides seizure control, is the early initiation of immunomodulatory therapy and the start of a ketogenic diet. As treatment is difficult and MOF may develop, patients should be transferred to a specialized children’s hospital providing full intensive care.

Hintergrund Fremdkörperaspirationen sind lebensbedrohende Ereignisse, die ein schnelles Handeln der beobachtenden Personen erfordern. Das Heimlich-Manöver, erstmalig 1975 beschrieben [1] ist auch im Kindesalter ein gut etabliertes Notfallmanöver und wird bei über 1jährigen mit Fremdkörperaspiration empfohlen [2]. Komplikationen wie abdominelle und thorakale Verletzungen sind selten und wurden v.a. bei geriatrischen Patienten beobachtet. Einzelne Fallberichte bei pädiatrischen Patienten berichten von Rippenfrakturen, Pneumomediastinum oder Pankreaspseudozysten [3].

Hintergrund Die Peritonealdialyse (PD) ist ein invasives Verfahren zur Behandlung eines akuten Nierenversagens (AKI) bei Früh- und Neugeborenen. Vereinzelt wurden erfolgreiche und komplikationslose Anwendungen von PD bei extremely low birth weight (ELBW) Frühgeborenen (FG) beschrieben [1] [2]. Indikationen für eine neonatale Dialyse sind angeborene Fehlbildungen, sich früh manifestierende chronische oder schwere akute Erkrankungen, wie z.B. eine Sepsis.

Hintergrund Die Organisation des deutschen Neugeborenen-Notarzt-Systems (sog. Baby-NAW) ist sehr heterogen und hängt stark von lokalen Gegebenheiten ab. Die Anforderungen an das Versorgungsteam und an die Transportsysteme sind in entsprechenden Leitlinien festgelegt [1]. Zur Erlangung der Qualifikation „Neugeborenen-Notarzt“ muss neben der Tätigkeit auf einer neonatologischen Intensivstation (NICU) die Teilnahme an einem entsprechenden Seminar und der Nachweis verschiedener invasiver Prozeduren am Neugeborenen (NG) erfolgen [2]. Auffrischungen dieser medizinischen Fertigkeiten sind nicht vorgesehen.

We derive two fundamental laws of chiral band crossings: (i) a local constraint relating the Chern number to phase jumps of rotation eigenvalues and (ii) a global constraint determining the number of chiral crossings on rotation axes. Together with the fermion doubling theorem, these laws describe all conditions that a network of chiral band crossing must satisfy. We apply the fundamental laws to prove the existence of enforced double Weyl points, nodal planes, and generic Weyl points, among others. In addition, we show that chiral space group symmetries can not stabilize nodal lines with finite Chern numbers. Combining the local constraint with explicit low-energy models, we determine the generic topological phase diagrams of all multifold crossings. Remarkably, we find a fourfold crossing with Chern number 5, which exceeds the previously conceived maximum Chern number of 4. We identify materials crystallizing in space group 198, such as B20 materials and BaAsPt, as suitable compounds with this Chern number 5 crossing.

Introduction:Simulation-based training is increasingly used in pediatrics to teach technical skills, teamwork, and team communication, and to improve potential deficiencies in pediatric emergency care. Team performance must be observed, analyzed, and evaluated by trained raters. The structured training of medical students for the assessment of simulated pediatric emergencies has not yet been investigated. Methods:We developed a rater training program for medical students to assess guideline adherence, teamwork, and team communication in simulated pediatric emergencies. Interrater reliability was measured at each training stage using Kendall tau coefficients. Results:In 10 out of 15 pairs of raters interrater reliability was moderate to high (tau>0.4), whereas it was low in the remaining 5 pairs of raters. Discussion:The interrater reliability showed good agreement between medical students and expert raters at the end of the rater training program. Medical students can be successfully involved in the assessment of guideline adherence as well as teamwork and team communication in simulated pediatric emergencies.

Hintergrund Ein akutes Nierenversagen (AKI) betrifft etwa 27% der Patient*innen auf pädiatrischen Intensivstationen (PICU), ca. 6% werden dialysepflichtig [1]. Die Ursachen für das kindliche AKI sind vielfältig. Das AKI im Rahmen anderer Organversagen ist mit einem schlechteren Outcome, u.a. mit einer erhöhten Mortalität assoziiert [2].

Hintergrund Interprofessionelle Simulationstrainings (ST) wurden in den vergangenen Jahren zunehmend etabliert, um professionelles Personal in einer sicheren Umgebung systematisch im Notfallmanagement zu schulen. Diese ST sind mit einem hohen personellen und zeitlichen Aufwand verbunden, insb. ärztliche Instruktor*innen sind schwer aus dem Klinikalltag zu lösen. Das KinderSimulationsteam Marburg (KiSiM e.V.) besteht aus 13 mittels EPALS- und Train-the-Trainer-Kursen ausgebildeten Ärzt*innen und Pflegenden, die in interprofessionellen Teams inner- und außerklinisch pädiatrische ST durchführen. 2022 wurden erstmalig pädiatrische ST in ausschließlich aus Pflegenden bestehenden Instruktorenteams durchgeführt. Zielgruppe waren Mitarbeitende der außerklinischen Kinderkrankenpflege.

Hintergrund LUTO ist eine angeborene Fehlbildung der Urethra (Klappen oder Atresie), die mit ca. 2-3/10.000 Fällen insb. männliche Neugeborene (NG) betrifft und in 50% d. F. bereits pränatal identifiziert wird. Die resultierende Obstruktion in Kombination mit einer primären Anlagestörung der Nieren schädigen die fetalen Nieren teils bis zum Oligo-/Anhydramnion und kann zur schweren Lungenhypoplasie führen. Die Anlage vesico-amnialer Shunts (VAS), Fruchtwasserauffüllungen und fetale Zystoskopie stellen pränatale Behandlungsoptionen dar, die ein Überleben der NG bei unbehandelt sehr schlechter Prognose sichern sollen.

We identify all symmetry-enforced band crossings in nonmagnetic orthorhombic crystals with and without spin-orbit coupling and discuss their topological properties. We find that orthorhombic crystals can host a large number of different band degeneracies, including movable Weyl and Dirac points with hourglass dispersions, fourfold double Weyl points, Weyl and Dirac nodal lines, almost movable nodal lines, nodal chains, and topological nodal planes. Interestingly, spin-orbit coupled materials in the space groups 18, 36, 44, 45, and 46 can have band pairs with only two Weyl points in the entire Brillouin zone. This results in a simpler connectivity of the Fermi arcs and more pronounced topological responses than in materials with four or more Weyl points. In addition, we show that the symmetries of the space groups 56, 61, and 62 enforce nontrivial weak Z2 topology in materials with strong spin-orbit coupling, leading to helical surface states. With these classification results in hand, we perform extensive database searches for orthorhombic materials crystallizing in the relevant space groups. We find that Sr2Bi3 and Ir2Si have bands crossing the Fermi energy with a symmetry-enforced nontrivial Z2 invariant, CuIrB possesses nodal chains near the Fermi energy, Pd7Se4 and Ag2Se exhibit fourfold double Weyl points, the latter one even in the absence of spinorbit coupling, whereas the fourfold degeneracies in AuTlSb are made up from intersecting nodal lines. For each of these examples we compute the ab-initio band structures, discuss their topologies, and for some cases also calculate the surface states.

We study the occurrence of symmetry-enforced topological band crossings in tetragonal crystals with strong spin-orbit coupling. By computing the momentum dependence of the symmetry eigenvalues and the global band topology in the entire Brillouin zone, we determine all symmetryenforced band crossings in tetragonal space groups. In particular, we classify all Dirac and Weyl degeneracies on points, lines, and planes, and find a rich variety of topological degeneracies. This includes, among others, double Weyl points, fourfold-double Weyl points, fourfold-quadruple Weyl points, Weyl and Dirac nodal lines, as well as topological nodal planes. For the space groups with symmetry-enforced Weyl points, we determine the minimal number of Weyl points for a given band pair and, remarkably, find that materials in space groups 119 and 120 can have band pairs with only two Weyl points in the entire Brillouin zone. This simplifies the topological responses, which would be useful for device applications. Using the classification of symmetry-enforced band crossings, we perform an extensive database search for candidate materials with tetragonal space groups. Notably, we find that Ba5In4Bi5 and NaSn5 exhibit twofold and fourfold Weyl nodal lines, respectively, which cross the Fermi energy. Hf3Sb and Cs2Tl3 have band pairs with few number of Weyl points near the Fermi energy. Furthermore, we show that Ba3Sn2 has Weyl points with an accordion dispersion and topological nodal planes, while AuBr and Tl4PbSe3 possess Dirac points with hourglass dispersions. For each of these candidate materials we present the ab-initio band structures and discuss possible experimental signatures of the nontrivial band topology.

We consider a two-orbital tight-binding model defined on a layered three-dimensional hexagonal lattice to investigate the properties of topological nodal lines and their associated drumhead surface states. We examine these surface states in centrosymmetric systems, where the bulk nodal lines are of Dirac type (i.e., fourfold degenerate), as well as in noncentrosymmetric systems with strong Rashba and/or Dresselhaus spin-orbit coupling, where the bulk nodal lines are of Weyl type (i.e., twofold degenerate). We find that in noncentrosymmetric systems the nodal lines and their corresponding drumhead surface states are fully spin polarized due to spin-orbit coupling. We show that unique signatures of the topologically nontrivial drumhead surface states can be measured by means of quasiparticle scattering interference, which we compute for both Dirac and Weyl nodal line semimetals.

Introduction. -Pantothenate kinase-associated neurodegenerative disease (PKAN) is a secondary generalized dystonia associated with an accumulation of iron in the basal ganglia and increased motor cortex excitability. A pilot study in three patients with secondary generalized dystonia had reported a reduced frequency of painful axial spasms following inhibitory 1-Hz repetitive transcranial magnetic stimulation (rTMS) applied over the premotor cortex.Patient and methods. -We compared the effects of real versus sham rTMS on the frequency of the complex movement pattern and the need for additional benzodiazepine medication in a 6-year-old male patient with PKAN. A 20-minute session of left premotor 1-Hz rTMS was performed daily on 5 consecutive days.Results. -The occurrence of the complex movement pattern was gradually reduced from three to two attacks daily to one attack daily by real rTMS while sham rTMS had no effect. This reduction was obtained concomitantly with a similar reduction of additional benzodiazepines for both real and sham rTMS sessions.Conclusion. -Inhibitory rTMS of the premotor cortex may be used to temporarily control motor symptoms in PKAN. (C) 2008 Elsevier Masson SAS. All rights reserved.

This study reports on the developmental outcome of a sample of pre-term children with a birth weight ≤1500 g born in 1994 and 1995 studied at the age of 5 to 6 years. The sample included 60 out of 81 surviving pre-term children (74.1%) of a German neonatal intensive care unit which was matched to a control group of 60 kindergarten children of the same region. The results show significant differences between term and pre-term children with regard to their intellectual development, language comprehension, attentiveness, and hyperactivity. Moreover, the pre-term children required more early intervention to compensate for developmental problems. The developmental deficits were intensified by the presence of biological risk factors like increasingly low birth weight, bronchopulmonary dysplasia and intraventricular haemorrhage. Conclusion: differences between term and pre-term children still exist, although the early 1990s witnessed fundamental changes in the treatment of pre-term children. The persistence of these differences is explained mainly by the increase in the survival rate of children with high biological risk factors.

OBJECTIVE:To characterize a rare inherited hypokalemic salt-losing tubulopathy with linkage to chromosome 1p31.METHODS:We conducted a retrospective analysis of the clinical data for 7 patients in whom cosegregation of the disease with chromosome 1p31 had been demonstrated. In addition, in 1 kindred, prenatal diagnosis in the second child was established, allowing a prospective clinical evaluation.RESULTS:Clinical presentation of the patients was homogeneous and included premature birth attributable to polyhydramnios, severe renal salt loss, normotensive hyperreninemia, hypokalemic alkalosis, and excessive hyperprostaglandin E-uria, which suggested the diagnosis of hyperprostaglandin E syndrome/antenatal Bartter syndrome. However, the response to indomethacin was only poor, accounting for a more severe variant of the disease. The patients invariably developed chronic renal failure. The majority had extreme growth retardation, and motor development was markedly delayed. In addition, all patients turned out to be deaf.CONCLUSION:The hypokalemic salt-losing tubulopathy with chronic renal failure and sensorineural deafness represents not only genetically but also clinically a disease entity distinct from hyperprostaglandin E syndrome/antenatal Bartter syndrome. A pleiotropic effect of a single gene defect is most likely causative for syndromic hearing loss.

The objective of this study was to evaluate the effect of conventional and long-chain polyunsaturated fatty acids (LCP)-enriched preterm formula on prostanoid formation in preterm infants during their first weeks of life. In a prospective, randomized, double-blind study, healthy infants received either formula enriched with LCP (n = 10), standard preterm formula(n = 10), or (expressed) breast milk (n = 10). Urine was sampled, and anthropometric measurements were taken at study entry and after the study period of 3 wk. In vivo formation of prostaglandin E2, thromboxane A2, and prostacyclin was evaluated by measuring the urinary excretion of the respective index metabolites by gas chromatography-mass spectrometry. There were no significant differences in urinary prostanoid excretion and anthropometric data between the groups at the end of the study period. We conclude that neither conventional formula nor supplementation of a preterm formula with LCP for a period of 3 wk substantially influence prostanoid formation in healthy preterm infants.