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ObjectiveThis 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.MethodsFifty subjects, aged 1–17 years, received flexible fiberoptic bronchoscopy under deep sedation. The BALF of 39 subjects was analyzed with reference to cytology and microbiology.ResultsThe percentage of neutrophils from the total cell count ranged from 0% to 95.3% (median 2.7). Nineteen patients (49%) had a percentage of ≥3.0%. Pearson's correlation showed a high correlation (r = 0.529, p = 0.001) between the coughing episodes per minute and the neutrophil percentage in the BALF. Analysis of variance showed a significant difference in neutrophil percentage between the indication groups (p = 0.013). The t-test (p = 0.019) showed a significant difference between the neutrophil percentage for patients with a probable airway infection under immunosuppression (median 2.9) and patients with cystic fibrosis (median 49.6). The linear regression analysis showed a significantly stronger impact of the neutrophil percentage on coughing frequency than the sedation regime (βneutrophils = 0.526 with p = 0.001 vs. βsedation = 0.165 with p = 0.251).ConclusionWhen bronchoscopy is to be performed on a pediatric patient with suspected bacterial or viral infection, and therefore neutrophilic airway inflammation, coughing is to be expected.

Background/purposeMEGDHEL 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 SERAC1 gene. Due to the unspecific symptoms and the diverse manifestations of the clinical phenotype, the diagnosis is challenging. Infantile MEGDHEL syndrome often has a severe disease course with acute liver failure. Differentiation from other metabolic disorders is difficult and requires a multidisciplinary approach.Case presentationA two-day-old small for gestational age neonate was admitted to our pediatric intensive care unit (PICU) due to severe liver failure with distinct hyperammonemia and hypoglycemia without elevation of transaminases or cholestasis. Due to high ammonia level, continuous hemodialysis was established immediately after admission. In addition, protein intake was stopped, and the patient anabolized with intravenous glucose. Temporary stabilization could be achieved after four days. In the further course, severe neurological and cardiocirculatory complications occurred, which ultimately led to the infant's death. In the metabolic diagnostics, a pronounced lactate acidosis and in urine an increased excretion of 3-methylglutaconic acid as well as other metabolites of mitochondrial energy metabolism has been the leading findings besides the hyperammonemia. Post-mortem trio whole genome analysis detected a homozygous pathogenic variant in SERAC1 with evidence of SERAC1 deficiency leading to the diagnosis of infantile MEGDHEL syndrome.ConclusionWhen pediatricians are faced with hepatopathy or even acute liver failure without elevation of transaminases or cholestasis in newborns, SERAC1 deficiency should be considered as a potential differential diagnosis. The initial treatment is based on the recommended management of suspected metabolic disorders. Even while no cure is available yet, patients should be offered proper supportive management through a multidisciplinary team. In addition, genetic confirmation of the diagnosis is important for the families, especially regarding further family planning.If a newborn presents with hyperammonemia, hypoglycemia and impaired liver synthesis function without elevation of transaminases or cholestasis, the possible presence of MEGDHEL syndrome due to a SERAC1 mutation should be considered.

BackgroundIntensified treatment protocols have improved survival of pediatric oncology patients. However, these treatment protocols are associated with increased treatment-related morbidity requiring admission to pediatric intensive care unit (PICU). We aimed to describe the organizational characteristics and processes of care for this patient group across PICUs in Europe.MethodsA web-based survey was sent to PICU directors or representative physicians between February and June 2021.ResultsResponses were obtained from 77 PICUs of 12 European countries. Organizational characteristics were similar across the different countries of Europe. The median number of PICU beds was 12 (IQR 8–16). The majority of the PICUs was staffed by pediatric intensivists and had a 24/7 intensivist coverage. Most PICUs had a nurse-to-patient ratio of 1:1 or 1:2. The median numbers of yearly planned and unplanned PICU admissions of pediatric cancer patients were 20 (IQR 10–45) and 10 (IQR 10–30, respectively. Oncology specific practices within PICU were less common in participating centres. This included implementation of oncology protocols in PICU (30%), daily rounds of PICU physicians on the wards (13%), joint mortality and morbidity meetings or complex patients’ discussions (30% and 40%, respectively) and participation of parents during clinical rounds (40%).ConclusionOur survey provides an overview on the delivery of critical care for oncology patients in PICU across European countries. Multidisciplinary care for these vulnerable and challenging patients remains complex and challenging. Future studies need to determine the effects of differences in PICU organization and processes of care on patients’ outcome.

Objective: The aim of our study was to assess the use of aEEG in our pediatric intensive care unit (PICU), indications for neuromonitoring and its findings, utility for seizure detection, and associations with outcome.Design: We retrospectively analyzed non-neonates who were treated in our PICU and received amplitude-integrated EEG (aEEG).Patients: 27 patients aged between 29 days and 10 0/12 years (median 7.3 months) were included, who received a total of 35 aEEGS.Measurements: aEEG tracings were assessed for background (BG) pattern and its evolution, seizures, and side differences using a visual classification (Hellström-Westas). Clinical data were collected from patients' histories and analyzed for correlation with aEEG findings.Main results: While rare in early years, there was an increase in use over time. Most aEEGs were conducted because of (suspected) seizures or for management of antiepileptic treatment. aEEG had low sensitivity but high specificity for recognition of pathological BG pattern with reference to conventional EEG. Worsening of BG pattern or failure to improve was associated with death. Seizure detection rates by aEEG were higher than by clinical observation, especially for identification of non-convulsive epileptic state (ES). Side differences in aEEG were rare, but if present, they were associated with unilateral brain injury.Conclusions: aEEG is useful for the detection of seizures and ES in pediatric intensive care patients. Abnormal BG pattern and poor evolution of BG are negatively associated with survival. aEEG is a potential supplement to conventional EEG, facilitating long-term surveillance of cerebral function when continuous full-channel EEG is not available. Further investigation is needed.