Inborn Errors of Immunity in Pediatric Intensive Care: Prevalence, Characteristics, and Prognosis.

Inborn errors of immunity (IEI) are a heterogeneous group of genetic diseases characterized by impaired immune system function. This prospective study aimed to determine the frequency, characteristics, and clinical course of IEI patients admitted to the pediatric intensive care unit (PICU) and identify mortality-related factors. Using a comprehensive immunological evaluation protocol, we screened 753 PICU admissions for potential IEIs during three years. Patients with pre-existing IEI diagnoses, chronic diseases, ongoing chronic medication regimens, other known comorbidities, trauma cases, post-surgical cases, and poisonings were excluded. Thirty-three patients were newly diagnosed with IEIs during or as a result of their PICU stay, representing an incidence of 4.39%. The most common disorders were immunodeficiencies with immune dysregulation (48.5%), followed by combined immunodeficiencies (24.2%). Severe viral infections (61%) and life-threatening infections (51.7%) were the most frequent warning signs. Only 31% of patients exhibited at least two Jeffrey Modell Foundation warning signs. The mortality rate was 58%, highlighting the need for early diagnosis and treatment. Newborn screening and family segregation studies are crucial to improving outcomes for IEI patients in intensive care settings.

Comparison of radiologic findings between SARS-CoV-2 and other respiratory tract viruses in critically ill children during the COVID-19 pandemic.

Background/aim: This study was planned because the radiological distinction of COVID-19 and respiratory viral panel (RVP)-positive cases is necessary to prioritize intensive care needs and ensure non-COVID-19 cases are not overlooked. With that purpose, the objective of this study was to compare radiologic findings between SARS-CoV-2 and other respiratory airway viruses in critically ill children with suspected COVID-19 disease. Materials and methods: This study was conducted as a multicenter, retrospective, observational, and cohort study in 24 pediatric intensive care units between March 1 and May 31, 2020. SARS-CoV-2- or RVP polymerase chain reaction (PCR)-positive patients' chest X-ray and thoracic computed tomography (CT) findings were evaluated blindly by pediatric radiologists. Results: We enrolled 225 patients in the study, 81 of whom tested positive for Coronovirus disease-19 (COVID-19) caused by severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2). The median age of all patients was 24 (7-96) months, while it was 96 (17-156) months for COVID-19-positive patients and 17 (6-48) months for positive for other RVP factor (p < 0.001). Chest X-rays were more frequently evaluated as normal in patients with SARS-CoV-2 positive results (p = 0.020). Unilateral segmental or lobar consolidation was observed more frequently on chest X-rays in rhinovirus cases than in other groups (p = 0.038). CT imaging findings of bilateral peribronchial thickening and/or peribronchial opacity were more frequently observed in RVP-positive patients (p = 0.046). Conclusion: Chest X-ray and CT findings in COVID-19 patients are not specific and can be seen in other respiratory virus infections.

The skills of defibrillation practice and certified life-support training in the healthcare providers in Turkey.

AIM OF THE STUDY: Successful cardiopulmonary resuscitation and early defibrillation are critical in survival after in- or out-of-hospital cardiopulmonary arrest. The scope of this multi-centre study is to (a) assess skills of paediatric healthcare providers (HCPs) concerning two domains: (1) recognising rhythm abnormalities and (2) the use of defibrillator devices, and (b) to evaluate the impact of certified basic-life-support (BLS) and advanced-life-support (ALS) training to offer solutions for quality of improvement in several paediatric emergency cares and intensive care settings of Turkey. METHODS: This cross-sectional and multi-centre survey study included several paediatric emergency care and intensive care settings from different regions of Turkey. RESULTS: A total of 716 HCPs participated in the study (physicians: 69.4%, healthcare staff: 30.6%). The median age was 29 (27-33) years. Certified BLS-ALS training was received in 61% (n = 303/497) of the physicians and 45.2% (n = 99/219) of the non-physician healthcare staff (P < .001). The length of professional experience had favourable outcome towards an increased self-confidence in the physicians (P < .01, P < .001). Both physicians and non-physician healthcare staff improved their theoretical knowledge in the practice of synchronised cardioversion defibrillation (P < .001, P < .001). Non-certified healthcare providers were less likely to manage the initial doses of synchronised cardioversion and defibrillation: the correct responses remained at 32.5% and 9.2% for synchronised cardioversion and 44.8% and 16.7% for defibrillation in the physicians and healthcare staff, respectively. The indications for defibrillation were correctly answered in the physicians who had acquired a certificate of BLS-ALS training (P = .047, P = .003). CONCLUSIONS: The professional experience is significant in the correct use of a defibrillator and related procedures. Given the importance of early defibrillation in survival, the importance and proper use of defibrillators should be emphasised in Certified BLS-ALS programmes. Certified BLS-ALS programmes increase the level of knowledge and self-confidence towards synchronised cardioversion-defibrillation procedures.

A multicentered study on efficiency of noninvasive ventilation procedures (SAFE-NIV)

Background/aim: To characterize the clinical course of noninvasive positive pressure ventilation (NIPPV) and high flow humidified nasal cannula ventilation (HFNC) procedures; perform risk analysis for ventilation failure. Material and methods: This prospective, multi-centered, observational study was conducted in 352 PICU admissions (1 month-18 years) between 2016 and 2017. SPSS-22 was used to assess clinical data, define thresholds for ventilation parameters and perform risk analysis. Results: Patient age, onset of disease, previous intubation and hypoxia influenced the choice of therapy mode: NIPPV was preferred in older children (p = 0.002) with longer intubation (p < 0.001), ARDS (p = 0.001), lower respiratory tract infections (p < 0.001), chronic respiratory disease, (p = 0.005), malignancy (p = 0.048) and immune deficiency (p = 0.026). The failure rate was 13.4%. sepsis, ARDS, prolonged intubation, and use of nasal masks were associated with NIV failure (p = 0.001, p < 0.001, p < 0.001, p = 0.025). The call of intubation or re-intubation was given due to respiratory failure in twenty-seven (57.5%), hemodynamic instability in eight (17%), bulbar dysfunction or aspiration in 5 (10.6%), neurological deterioration in 4 (8.5%) and developing ARDS in 3 (6.4%) children. A reduction of less than 10% in the respiration within an hour increased the odds of failure by 9.841 times (OR: 9.841, 95% CI: 2.0021­48.3742). FiO2 > 55% at 6th hours and PRISM-3 >8 were other failure predictors. Of the 9.9% complication rate, the most common complication was pressure ulcerations (4.8%) and mainly observed when using full-face masks (p = 0.047). Fifteen (4.3%) patients died of miscellaneous causes. Tracheostomy cannulation was performed on 16 children due to prolonged mechanical ventilation (8% in NIPPV, 2.6% in HFNC) Conclusion: Absence of reduction in the respiration rate within an hour, FiO2 requirement >55% at 6th hours and PRISM-3 score >8 predict NIV failure.

A rare aspect of Crohn's disease: Pulmonary involvement in a child.

Crohn's disease (CD), known as the disease of gastrointestinal system, is a granulamatous systemic disorder with extraintestinal manifestations including the respiratory system. The resemblance in the embriological origins and the immunities of both organ systems' mucosae, also the circulating immune complexes and the autoantibodies are accepted as contributing factors. The shift of inflammation may become prominent when the colon is removed after colectomy and independent of the bowel disease activity; pulmonary involvement may be exarbecated. In the pediatric population, CD associated pulmonary involvement is very rare, mainly in the form of subclinical alterations and the data are limited mostly to case reports. Therefore, it is possibly overlooked since the diagnosis relies on suspicion. We represent a 5-year-old CD patient with previous bronchiolitis episodes that might have resulted from CD-associated pulmonary involvement; whom later developed severe pneumonia resulting in acute respiratory distress syndrome and bronchiectasia following a colectomy operation.

BIG score is a strong predictor of mortality and morbidity for high-energy traumas in pediatric intensive care unit.

BACKGROUND: Severe traumatic injuries not only constitute an important population of pediatric intensive care unit (PICU) but they also play a major role in mortality and morbidity. Mortality risk assessment of traumatic injuries in the PICU is a delicate issue as it influences the treatment decisions. BIG score (Base Deficit +[2.5 × INR] + [15-GCS]) and the Pediatric Trauma Score (PTS) are utilized in pediatric trauma centers for the assessment of trauma severity. In this research, we aimed to elucidate the predictivity of trauma severity scores, the PRISM-3 (pediatric risk of mortality), and admission laboratory parameters in pediatric patients with high-energy traumas. METHODS: Children who had been exposed to high-energy polytraumas between 2018 and 2020 and treated in a tertiary care PICU were included in this retrospective analysis. Newly developed mental or motor disabilities, post-traumatic acquired epilepsy, requirement for tracheostomy, and/or extremity loss at PICU discharge were defined as morbidity. The PTS, the BIG score, PRISM-3 score, and admission laboratory parameters were utilized for mortality and morbidity prediction. RESULTS: A total of 155 patients were included in the study. The median age of the participants were 66 months (25-134). The origin of trauma was fall from height in 45.2% (n=70) of the subjects and traffic accident 54.8% (n=85) of the cases. New morbidities had occurred in 8.7% (n=13) and 3.2% (n=5) of the patients deceased in the ICU. The results of logistic regression analysis indicated that BIG score (p=0.01), PTS (p=0.003), PRISM-3 (p=0.02), admission D-dimer (p=0.01), and albumin levels (p=0.001) were significantly associated with mortality. The receiver operating characteristics curve analysis denoted that BIG score (cutoff >21.5, area under the curve [AUC]: 0.984 95% CI: 0.943-0.988), PRISM-3 score (cutoff >18, AUC: 0.997 95% CI: 0.970-1), the PTS (cutoff ≤3, AUC: 0.969 95% CI: 0.928-0.990), admission albumin level (cutoff ≤3 g/dL, AUC: 0.987 95% CI: 0.953-0.998), and D-dimer level (cutoff >13,100 mcg/L, AUC: 0.776 95% CI: 0.689-0.849) all had high predictive values for mortality. CONCLUSION: Regarding the results of this research, one can conclude that BIG score is a strong predictor of mortality and morbidity in high-energy pediatric traumas. Although PRISM-3 score has a similar predictive capability, the earlier and easier calculation as-sets of BIG score positions itself as a more useful and powerful predictor for mortality and morbidity in pediatric high-energy traumas.

Tranexamic Acid Utilization in Craniosynostosis Surgery.

AIM: To analyze the impact of Tranexamic acid (TXA) on perioperative hemodynamics in craniosynostosis surgery. MATERIAL AND METHODS: Data of thirty-six children (operated between 2014-2017) were categorized into two groups depending on TXA delivery. Patient demographics, preoperative, intraoperative, postoperative data on hemostasis and metabolic outcomes were recorded. Blood loss from the drains, estimated blood loss (EBV loss), volume of blood transfusions, hemodynamic alerations and complications were extracted. Postoperative outcome involved variables at admission, 2 < sup > nd < /sup > , 6 < sup > th < /sup > , 12 < sup > th < /sup > , 24 < sup > th < /sup > hours. A multiple logistic regression analysis was also performed. RESULTS: Demographics presented mean age of 8.14 ± 3.53 months, male/female ratio:1.76/1, procedure length 3.98 ± 0.78 hours. Intraoperative analysis indicated TXA deliveries manifested fewer blood transfusion volumes (p=0.002) due to lower EBV loss (4.02 ± 1.19 ml/kg vs. 5.97 ± 1.61 ml/kg, p < 0.001) with better metabolic outcome. Postoperative outcomes presented all children manifested hematocrit decline after surgey. TXA did not influence postoperative hemodynamic alterations (p=0.090, p=0.112), despite reduced blood loss from the drains and transfusion necessity (p=0.015, p=0.0175). Intraoperative transfusion volumes and EBV loss were associated with postoperative hemodynamics (OR: 3.033, 95% CI: 1.286-7.154; p=0.011; OR: 0.280, 95% CI: 0.081-0.972; p=0.045, respectively). ROC analysis indicated 10.13 ml/kg of intraoperative blood transfusion requirement as the cut off value for hemodynamic instability with 91% sensitivity and 80% specificity. One unit increase in intraoperative transfused blood volume increased the odds of developing hemodynamic alterations by 3.033 times. CONCLUSION: Intraoperative TXA is crucial for successful surgical management; however postoperative period carries out paramount importance due to excessive bleeding after surgery. In case of severe intraoperative transfusion necessity, postoperative TXA utilization might be considered to minimize potential risks by balancing the pros and cons of the drug.

Early postoperative follow-up after craniosynostosis surgery

Background/aim: Declined morbidity rates after craniosynostosis surgery indicate bypassing the pediatric intensive care unit (PICU) course to minimize treatment costs and bed usage. The aim of this study is to examine the incident rates of PICU admission and assess its necessity. Materials and methods: A retrospective analysis of 41 patients (operated on by open surgical techniques) between July 2011 and December 2015 was carried out. Intraoperative/postoperative vital signs, hemodynamic and metabolic parameters, estimated blood loss (EBVloss), blood transfusions, length of PICU, and hospitalizations were recorded. Results: Major and minor events reached 51.2% and 82.9%, respectively. EBVloss within 24 h was calculated as 39.58 ± 8.19 (median: 38.44, 25.68-66.34) with 75.6% blood transfusion rate. Hypotension and hypothermia were associated with prolonged surgery (P = 0.001 and P = 0.007, respectively), but were not related to age (P = 0.054, P = 0.162) or procedure types (P = 0.558, P = 0.663). Prolonged surgery and younger age had an impact on the complications. One patient died of persistent hemorrhage at 96 h. Conclusion: Monitoring cardiovascular and metabolic dynamics at PICU during the first 24 h after surgery is crucial. Additional studies are needed to define the threshold values of several metabolic and hemodynamic markers in risk assessment after cranial vault surgery.