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Background The Borna disease virus (BoDV-1) is an emerging zoonotic virus causing severe and mostly fatal encephalitis in humans. Methods and Results A local cluster of fatal BoDV-1 encephalitis cases was detected in the same village three years apart affecting two children. While the first case was diagnosed late in the course of disease, a very early diagnosis and treatment attempt facilitated by heightened awareness was achieved in the second case. Therapy started as early as day 12 of disease. Antiviral therapy encompassed favipiravir and ribavirin, and, after bioinformatic modelling, also remdesivir. As the disease is immunopathogenetically mediated, an intensified anti-inflammatory therapy was administered. Following initial impressive clinical improvement, the course was also fatal, although clearly prolonged. Viral RNA was detected by qPCR in tear fluid and saliva, constituting a possible transmission risk for health care professionals. Highest viral loads were found post mortem in the olfactory nerve and the limbic system, possibly reflecting the portal of entry for BoDV-1. Whole exome sequencing in both patients yielded no hint for underlying immunodeficiency. Full virus genomes belonging to the same cluster were obtained in both cases by next-generation sequencing. Sequences were not identical, indicating viral diversity in natural reservoirs. Specific transmission events or a common source of infection were not found by structured interviews. Patients lived 750m apart from each other and on the fringe of the settlement, a recently shown relevant risk factor. Conclusion Our report highlights the urgent necessity of effective treatment strategies, heightened awareness and early diagnosis. Gaps of knowledge regarding risk factors, transmission events, and tailored prevention methods become apparent. Whether this case cluster reflects endemicity or a geographical hot spot needs further investigation.

Persistent air leak (PAL) is a severe complication of secondary spontaneous pneumothorax (SSP). Surgical interventions are usually successful when medical treatment fails, but can be associated with significant complications and loss of potentially recoverable lung parenchyma.Retrospective analysis of efficacy and safety of interventional bronchus occlusions (IBO) using Amplatzer devices (ADs) in children with PAL secondary to SSP.Six patients (four males, 4-15 years of age) underwent IBO using ADs as treatment for PAL. Necrotizing pneumonia (NP) was the most common cause (n=4) of PAL. Three patients were previously healthy and three suffered from chronic lung disease. All patients required at least two chest tubes prior to the intervention for a duration of 15-43 days and all required oxygen or higher level of ventilatory support. In three cases, previous surgical interventions had been performed without success. All children improved after endobronchial intervention and we observed no associated complications. All chest tubes were removed within 5-25 days post IBO. In patients with PAL related to NP (n=4), occluders were removed bronchoscopically without re-occurrence of pneumothorax after a mean of 70 days (IQR: 46.5-94).IBO using ADs is a safe and valuable treatment option in children with PAL independent of disease severity and underlying cause. A major advantage of this procedure is its less invasiveness compared to surgery and the parenchyma- preserving approach.Hintergrund: Die persistierende Luftleckage stellt eine schwere Komplikation eines sekundären Spontanpneumothorax (SSP) dar. Chirurgische Interventionen sind bei erfolgloser konservativer Therapie oft erfolgreich aber können mit schweren Komplikationen und dem Verlust von potentiell erholungsfähigem Lungengewebe behaftet sein. Ziel: Retrospektive Analyse über die Effektivität und Sicherheit von interventionellen Bronchus-Okklusionen (IBO) mit Amplatzer Devices (ADs) bei Kindern mit persistierender Luftleckage. Ergebnise: Bei 6 Patienten (4 männlich, Alter 4–15 Jahre) wurde eine IBO zur Therapie einer persistierenden Luftleckage durchgeführt. Die nekrotisierende Pneumonie (NP) war die häufigste zugrundeliegende Ursache einer persistierenden Leckage (n=4). 3 Kinder waren zuvor gesund, bei 3 Kindern lag eine chronische Lungenerkrankung vor. Alle Patienten benötigten vor Intervention mindestens 2 Thoraxdrainagen für 15–43 Tage und alle Kinder benötigten Sauerstoff oder eine zusätzliche Atemunterstützung. Bei 3 Patienten erfolgten zuvor erfolglose chirurgische Interventionen. Es traten keine Komplikationen im Zusammenhang mit der IBO auf. Alle Patienten profitierten von der Prozedur, die Thoraxdrainagen konnten nach 5–25 Tagen gezogen werden. Bei allen Kindern mit persistierender Luftleckage aufgrund einer NP (n=4) wurden die ADs nach durchschnittlich 70 Tagen (IQR:46.5–94) entfernt ohne dass ein Pneumothorax-Rezidiv auftrat. Schlussfolgerung: Die IBO mithilfe von ADs stellt unabhängig von der Ursache und Krankheitsschwere ein vielversprechendes und sicheres Therapieverfahren zur minimal-invasiven Behandlung von Kindern mit persistierender Luftleckage dar. Vorteile dieser Prozedur im Vergleich zu operativen Verfahren sind die geringe Invasivität und der parenchymerhaltende Ansatz.

Introduction Pulmonary alveolar proteinosis (PAP) is defined by increased accumulation of surfactant in the alveolar space. PAP has been reported to be associated with a large number of clinical conditions and diseases. Whole lung lavages can be helpful to stabilize the clinical course of PAP until the underlying condition is identified, which may enable more specific treatment. Recently, heterozygous OAS1 gain-of-function variants were described as cause in a patient with infantile-onset PAP combined with hypogammaglobulinemia. Case presentation At age 4 months, a female infant born to term was diagnosed with hypogammaglobulinemia and treated with monthly immunoglobulin injections. At age 15 months, the girl needed supplemental oxygen at night, and at age 18 months, also during the day. At age 2 years, pulmonary alveolar proteinosis of unknown etiology was diagnosed by computed tomography scan and open lung biopsy. Subsequently, monthly whole lung lavages (WLLs) were started, which stabilized the clinical course for over 2 years until a disease-causing OAS1 variant was diagnosed and the patient was successfully treated by hematopoietic stem cell transplantation. Conclusion Here, we describe the successful management of a female patient with severe PAP caused by a heterozygous OAS1 gain-of-function variant until a definitive diagnosis was made and cured by hematopoietic stem cell transplantation. This article is protected by copyright. All rights reserved.

Background Long segment laryngotracheoesophageal clefts (LTECs) are very rare large‐airway malformations. Over the last 40 years mortality rates declined substantially due to improved intensive care and surgical procedures. Nevertheless, long‐term morbidity, comorbidity, and clinical outcomes have rarely been assessed systematically. Methods In this retrospective case series, the clinical presentation, comorbidities, treatment, and clinical outcomes of all children with long‐segment LTEC that were seen at our department in the last 15 years were collected and analyzed systematically. Results Nine children were diagnosed with long segment LTEC (four children with LTEC type III and five patients with LTEC type IV). All children had additional tracheobronchial, gastrointestinal, or cardiac malformations. Tracheostomy for long‐time ventilation and jejunostomy for adequate nutrition was necessary in all cases. During follow‐up one child died from multiorgan failure due to sepsis at the age of 43 days. The clinical course of the other eight children (median follow‐up time 5.2 years) was stable. Relapses of the cleft, recurrent aspirations, and respiratory tract infections led to repeated hospital admissions. Conclusions Long‐segment LTECs are consistently associated with additional malformations, which substantially influence long‐term morbidity. For optimal management, a multidisciplinary approach is essential.

Background: Pulmonary alveolar proteinosis (PAP) is a heterogeneous condition with more than 100 different underlying disorders that need to be differentiated to target therapeutic options, which are generally limited. Methods: The clinical course of two brothers with pathogenic variants in the methionyl-tRNA synthetase (MARS)1 gene was compared to previously published patients. Functional studies in patient-derived fibroblasts were performed and therapeutic options evaluated. Results: The younger brother was diagnosed with PAP at the age of 1 year. Exome sequencing revealed the homozygous MARS1 variant p.(Arg598Cys), leading to interstitial lung and liver disease (ILLD). At 2 years of age, following surgery hypoglycemia was detected, the pulmonary condition deteriorated, and the patient developed multiorgan failure. Six therapeutic whole lung lavages (WLL) were necessary to improve respiratory insufficiency. Methionine supplementation was started and a high protein diet ensured, leading to complete respiratory recovery. The older brother, homozygous for the same MARS1 variant, had a long-known distinct eating preference of methionine-rich food and showed a less severe clinical phenotype. Decreased aminoacylation activity confirmed the pathogenicity of p.(Arg598Cys) in vitro. In agreement with our review of currently published ILLD patients, the presence of hepatopathy, developmental delay, muscular hypotonia, and anemia support the multisystemic character of the disease. Conclusions: Catabolic events can provoke a severe deterioration of the pulmonary situation in ILLD with a need for repetitive WLL. Although the precise role of oral methionine supplementation and high protein intake are unknown, we observed an apparent treatment benefit, which needs to be evaluated systematically in controlled trials.

Childhood arterial ischaemic stroke (AIS) is a rare, but potentially life-threatening event which requires early diagnosis and adequate treatment. The reported significant time delay to childhood AIS diagnosis may be associated with low awareness, the more nonspecific clinical presentation as well as difficult clinical differentiation to more common "stroke mimics" and a less established "acute care structure" with delayed access to proper neuroimaging. Compared with adult stroke care, experiences with acute reperfusion therapies like thrombolysis and mechanical thrombectomy are promising but limited and not based on clinical trials. The etiological work-up is absolutely essential, as the child's individual risk profile determines acute management, secondary prevention, risk of recurrence and outcome. Follow-up care should be organized in a multidisciplinary setting covering all bio-psycho-social aspects to achieve the best integration of the child into its educational, later professional and social environments.Der Schlaganfall im Kindes- und Jugendalter ist ein seltenes, aber potentiell lebensbedrohliches Ereignis und erfordert rasche Diagnosestellung und Therapie. Aufgrund der geringen Awareness, der häufigen unspezifischen klinsichen Präsentation mit einer Fülle zu bedenkender, wahrscheinlicherer Differenzialdiagnosen sowie der noch wenig etablierten Versorgungsstrukturen wird der kindliche Schlaganfall oft erst mit einer deutlichen Zeitverzögerung diagnostiziert. In der Erwachsenenneurologie etablierte Revaskularisationstherapien können in der Pädiatrie aktuell nur als off-label Therapien mit begrenzten Erfahrungen eingesetzt werden. Die ätiologische Abklärung ist unabdingbar, bestimmt doch das individuelle Risikoprofil neben Akutmanagement und Rezidivprophylaxe auch das Rezidivrisiko und Outcome. Die Langzeitbetreuung der betroffenen Kinder und Jugendlichen sollte im multidisziplinären Setting unter Berücksichtigung aller bio-psycho-sozialer Aspekte erfolgen. Denn neben dem klinisch neurologischen Outcome sind insbesondere auch die Auswirkungen des Schlaganfalls auf Kognition und Verhalten für eine erfolgreiche Integration des Kindes in sein schulisches und soziales Umfeld mitentscheidend.

Continuous EEG monitoring (cEEG) is frequently used in neurocritical care. The detection of seizures is one of the main objectives. The placement of the EEG electrodes is time consuming, therefore a reduced montage might lead to an increased availability in the ICU setting. It is unknown whether such a reduction of electrodes reduces the number of seizure patterns that are detected. A total of 95 seizure and 95 control EEG sequences from a pediatric epilepsy monitoring unit (EMU) were anonymized and reduced to an eight-lead montage. Two experts evaluated the recordings and the seizure detection rates using the reduced and the full montage were compared. Sensitivity and specificity for the seizure detection were calculated using the original EMU findings as gold standard. The sensitivity to detect seizures was 0.65 for the reduced montage compared to 0.76 for the full montage (p = 0.031). The specificities (0.97 and 0.96) were comparable (p = 1). A total of 4/9 (44%) of the generalized, 12/44 (27%) of the frontal, 6/14 (43%) of the central, 0/1 (0%) of the occipital, 6/20 (30%) of the temporal, and 5/7 (71%) of the parietal seizure patterns were not detected using the reduced montage. The median time difference between the onset of the seizure pattern in the full and reduced montage was 0.026s (IQR 5.651s). In this study the reduction of the EEG montage from 21 to eight electrodes reduced the sensitivity to detect seizure patterns from 0.76 to 0.65. The specificity remained virtually unchanged.

In childhood, a multitude of causes lead to pulmonary alveolar proteinosis (PAP), an excessive surfactant accumulation in the alveolar space, limiting gas exchange. Autoantibodies against granulocyte-macrophage colony-stimulating factor (GM-CSF) causing autoimmune PAP, the principal aetiology in adults, are rare. In this first case series on autoimmune PAP, we detail the presentation and management issues of four children. Whereas three children presented insidiously with progressive dyspnoea, one was acutely sick with suspected pneumonia. During management, one patient was hospitalised with coronavirus disease 2019, noninvasively ventilated, and recovered. All treatment modalities known from adults including whole-lung lavage, augmentation of GM-CSF by inhaled GM-CSF, removal of neutralising antibody by plasmapheresis and interruption of antibody production using rituximab were considered; however, not all options were available at all sites. Inhaled GM-CSF appeared to be a noninvasive and comfortable therapeutic approach. The management with best benefit-to-harm ratio in autoimmune PAP is unknown and specialised physicians must select the least invasive and most effective treatment. To collect this cohort in a rare condition became feasible as patients were submitted to an appropriate registry. To accelerate the authorisation of novel treatments for autoimmune PAP, competent authorities should grant an inclusion of adolescents into trials in adults.

Objective Childhood acute ischemic stroke (CAIS) is a rare event. Diverse etiologies, risk factors, symptoms and stroke mimics hamper obtaining a fast diagnosis and implementing immediate recanalization strategies. Methods Over a period of 3 years (2015–2017), the data of 164 pediatric patients (> 28 days of life-18 years) with a first episode of AIS were submitted to a hospital-based nationwide surveillance system for rare disorders (ESPED). We report a subgroup analysis of patients who have undergone recanalization therapy and compare these data with those of the whole group. Results Twenty-eight patients (17%) with a median age of 12.2 years (range: 3.3–16.9) received recanalization therapy. Hemiparesis, facial weakness and speech disturbance were the main presenting symptoms. The time from onset of symptoms to confirmation of diagnosis was significantly shorter in the intervention group (4.1h vs. 20.4 h, p =

Analysis of autoinflammatory and immunodeficiency disorders elucidates human immunity and fosters the development of targeted therapies. Oligoadenylate synthetase 1 is a type I interferon-induced, intracellular double-stranded RNA (dsRNA) sensor that generates 2'-5'-oligoadenylate to activate ribonuclease L (RNase L) as a means of antiviral defense. We identified four de novo heterozygous OAS1 gain-of-function variants in six patients with a polymorphic autoinflammatory immunodeficiency characterized by recurrent fever, dermatitis, inflammatory bowel disease, pulmonary alveolar proteinosis, and hypogammaglobulinemia. To establish causality, we applied genetic, molecular dynamics simulation, biochemical, and cellular functional analyses in heterologous, autologous, and inducible pluripotent stem cell-derived macrophages and/or monocytes and B cells. We found that upon interferon-induced expression, OAS1 variant proteins displayed dsRNA-independent activity, which resulted in RNase L-mediated RNA cleavage, transcriptomic alteration, translational arrest, and dysfunction and apoptosis of monocytes, macrophages, and B cells. RNase L inhibition with curcumin modulated and allogeneic hematopoietic cell transplantation cured the disorder. Together, these data suggest that human OAS1 is a regulator of interferon-induced hyperinflammatory monocyte, macrophage, and B cell pathophysiology.

Dieses Kapitel gibt einen Uberblick uber die chirurgische und traumabedingte Intensivtherapie. Zunachst werden die peri- und postoperative Intensivtherapie diskutiert, bevor anschliesend Polytrauma und einzelne Organtraumata – Schadel-Hirn-Trauma, Abdominal-, Wirbelsaulentrauma, stammnahe und Extremitatenverletzungen – beschrieben werden. Abschliesend wird auf die Besonderheiten von Elektrounfallen hingewiesen.

Analysis of autoinflammatory and immunodeficiency disorders elucidates human immunity and fosters the development of targeted therapies. Oligoadenylate synthetase 1 is a type I interferon-induced, intracellular dsRNA sensor that generates 2’−5’-oligoadenylate to activate RNase L as a means of antiviral defense. We identified four de novo heterozygous OAS1 gain-of-function variants in six patients with a polymorphic autoinflammatory immunodeficiency characterized by recurrent fever, dermatitis, inflammatory bowel disease, pulmonary alveolar proteinosis, and hypogammaglobulinemia. To establish causality, we applied genetic, molecular dynamics simulation, biochemical, and cellular functional analyses in heterologous, autologous and inducible pluripotent stem cell derived macrophages. We found that upon interferon-induced expression, OAS1 variant proteins displayed dsRNA-independent activity, which resulted in RNase L-mediated RNA-cleavage, transcriptomic alteration, translational arrest, and dysfunction and apoptosis of monocytes, macrophages, and B-cells upon interferon stimulation. RNase L-inhibition with curcumin modulated and allogeneic hematopoietic cell transplantation cured the disorder. Together, these data suggest that human OAS1 is a regulator of interferon-induced hyperinflammatory monocyte and B-cell pathophysiology.

Background Intravenous artesunate (ivA) is the standard treatment for severe malaria. Data systematically evaluating the use of ivA in pediatric patients outside malaria-endemic regions are limited. The aim of this case series was to summarize efficacy and safety of ivA for imported severe malaria in children in Germany. Methods Our retrospective case series included pediatric patients with imported severe malaria treated with at least 1 dose of ivA (Artesun, Guilin Pharmaceutical; Shanghai, China) at 4 German tertiary care centers. Severe malaria was defined according to World Health Organization criteria. Results Between 2010 and 2018, 14 children with a median [interquartile range (IQR)] age of 6 (1;9.5) years were included. All children were of African descent. All but 2 patients had Plasmodium falciparum malaria; 1 child had P. vivax malaria and 1 child had P. falciparum and P. vivax co-infection. Median (IQR) parasitemia at admission in patients with P. falciparum was 9.5% (3;16.5). Patients were treated with 1-10 [median (IQR) 3 (3;4)] doses ivA. All but one patient received a full course of oral antimalarial treatment. Parasite clearance was achieved within 2-4 days, with the exception of 1 patient with prolonged clearance of peripheral parasitemia. Three patients experienced posttreatment hemolysis but none needed blood transfusion. Otherwise ivA was safe and well tolerated. Conclusions ivA was highly efficacious in this pediatric cohort. We observed episodes of mild to moderate posttreatment hemolysis in approximately one-third of patients. The legal status and usage of potentially lifesaving ivA should be evaluated in Europe.

Complete signal transducer and activator of transcription 1 (STAT1) deficiency causes a rare primary immunodeficiency that is characterized by defective IFN-dependent gene expression leading to life-threatening viral and mycobacterial infections early in life.To characterize a novel STAT1 loss-of-function variant leading to pathological infection susceptibility and hyperinflammation.Clinical, immunologic, and genetic characterization of a patient with severe infections and hemophagocytic lymphohistiocytosis-like hyperinflammation was investigated.We reported a child of consanguineous parents who presented with multiple severe viral infections that ultimately triggered hemophagocytic lymphohistiocytosis and liver failure. Despite intensified therapy with antivirals and cytomegalovirus-specific donor cells, the child died after hematopoietic stem cell transplantation because of cytomegalovirus reactivation with acute respiratory distress syndrome. Exome sequencing revealed a homozygous STAT1 variant (p.Val339ProfsTer18), leading to loss of STAT1 protein expression. Upon type I and type II IFN stimulation, immune and nonimmune cells showed defective upregulation of IFN-stimulated genes and increased susceptibility to viral infection in vitro. Increased viral infection rates were paralleled by hyperinflammatory ex vivo cytokine responses with increased production of TNF, IL-6, and IL-18.Complete STAT1 deficiency is a devastating disorder characterized by severe viral infections and ensuing hyperinflammatory responses. Early diagnosis can be made by exome sequencing and variant validation by functional testing of STAT1-dependent programmed cell death 1 ligand 1 surface expression on monocytes. Furthermore, high awareness for hyperinflammatory complications and potential targeted treatment strategies such as IL-18 binding protein could be considered. Hematopoietic stem cell transplantation is the only definitive treatment strategy but remains challenging.

Background: Genetic pulmonary alveolar proteinosis (PAP) is a term describing inborn diseases with abundant surfactant accumulation in the lungs. The determination of the molecular cause is critical to make the correct therapy decisions Recently three families with PAP due to mutations in OAS1 gene have been described. Here we present another case of this novel disease. Case Presentation: At 4 months of age a term-born female presented with recurrent severe respiratory and gastrointestinal tract infections. Hypogammaglobulinaemia was noted, but no immune-deficiency could be diagnosed. She was treated with monthly immunoglobulins. Over time the clinical course deteriorated; at age of 12 months the girl needed supplemental oxygen during night times and at 18 months also during daytime. The diagnosis of a pulmonary alveolar proteinosis (PAP) of unknown cause was made by CT scan and open lung biopsy. Whole lung lavages (WLL) were started at 2 years of age every 4 weeks. Whole exome sequencing identified a new de novo heterozygous mutation in OAS1. With this the diagnosis the patient was prepared for allogeneic haematopoietic stem cell transplantation (HSCT) with a matched unrelated donor, which was performed at the age of 4 years. During the first few days following HSCT oxygen demand increased, making another WLL necessary. Then the pulmonary clinical course rapidly improved day by day until normalizing of gas exchange at day 15. Conclusion: WLL in patients with pulmonary alveolar proteinosis can stabilize patients until a definitive diagnosis can be made. Bone marrow transplantation may cure PAP due to a mutation in the OAS1 gene.

This chapter shows that nival and alpine plant distributions can be separated, not only according to community patterns, but can also be correlated to basic habitat features, e.g. temperature and snowcover duration. Temperature is the outstanding driving factor which triggers the altitudinal turnover of species and vegetation types in mountain systems. The various aspects of the temperature regime, however, influence mountain plant distribution patterns differently. Depending on the respective situation, they are more or less threatened by climate warming. This is important for any global account of mountain biodiversity and its possible changes. Climate change scenarios suggest that, as a consequence of earlier snowmelt and/or warming, alpine species may fill the nival niche, putting strong pressure on the less competitive nival species. In the international literature the term alpine is commonly used to describe the uppermost vegetation zone of high mountain systems, from the treeline upwards to the limits of plant life.

Der Schlaganfall im Kindesalter ist ein Notfall mit hoher Mortalitat und Morbiditat. Er hat – in Abhangigkeit von der zugrunde liegenden Atiologie – ein klinisch bedeutsames Rezidivrisiko. Die Atiologie ist vielfaltig. Vaskulopathien, Infektionen, kardiale Probleme und Koagulopathien spielen pathophysiologisch eine wesentliche Rolle. Die atiologische Abklarung muss umfassend sein – der kindliche Schlaganfall ist eine „Multiple-Risk-Erkrankung“. Klassische Leitsymptome sind die akute Hemiparese, die faziale Parese und die Sprachstorung: Sie bestehen bei ca. 75 % der betroffenen Kinder. Aufgrund der mannigfaltigen, teils unspezifischen Symptome, der breiten Differentialdiagnosen und des fehlenden Bewusstseins fur den kindlichen Schlaganfall wird die Diagnose mittels Bildgebung oft erst nach einer erheblichen Zeitverzogerung gestellt (im Mittel nach 24 h). Die in speziellen Fallen moglichen Therapien wie die Thrombolyse oder die mechanische Thrombektomie konnen dabei grundsatzlich nur innerhalb weniger Stunden nach dem Ereignis eingesetzt werden – sie sind spezialisierten Zentren vorbehalten. Als neurologisches Residualsymptom steht die Hemiparese im Vordergrund, je nach betroffenem Infarktareal treten cerebrale Krampfanfalle, Sprachstorungen etc. auf. Die Auswirkungen auf Kognition und Verhalten werden in der Versorgungspraxis oft ubersehen und unterschatzt.

The pediatric Glasgow coma scale (pGCS) is a consciousness score that, although widely applied, requires skill to apply. The AVPU scale uses four simple categories (Alert; Verbal response; response to Pain; Unresponsive), but has not been studied in a large pediatric population. We compared the performance of the AVPU and pGCS scales in a large pediatric cohort in an acute, prehospital setting.In a six-month-long prospective cohort study, AVPU and pGCS scores were determined by emergency physicians in children less than 10 years of age at their first prehospital encounter.We included 302 children (median age 2.3 years) with a broad spectrum of diagnoses. Data were complete for 287 children. AVPU and pGCS scores showed good a correlation in the extreme categories A and U (positive predictive values of 98% and 100%, respectively). Corresponding pGCS scores for each AVPU category were as follows: 11-15 for A; 5-15 for V; 4-12 for P; and 3-5 for U. The positive predictive value to detect patients with pGCS ≥ 8 for AVPU category V was 100%.We demonstrated good correlation of simple and fast consciousness AVPU scoring to the standard pGCS in a large cohort of pediatric patients in a prehospital setting. The AVPU category "V" identifies patients with a pGCS of or exceeding 8 and, therefore, identifies children at low risk requiring more invasive procedures or intensive care treatment.Glasgow coma scale (GCS); Alert-verbal-pain-unresponsive-score; AVPU-score; consciousness assessment; children, pediatric emergency.