Congenital diaphragmatic hernia treated via fetal endoscopic tracheal occlusion improves outcome in a middle-income country.

OBJECTIVES: A recent European randomized trial - Tracheal Occlusion To Accelerate Lung Growth - demonstrated that fetoscopic endoluminal tracheal occlusion (FETO) is associated with increased postnatal survival among infants with severe congenital diaphragmatic hernia (CDH). However, this differs in middle-income countries such as Brazil, where abortion is illegal and neonatal intensive care is inadequate. This study evaluated the effects of FETO on improving the survival of infants with moderate-to-severe CDH in isolated and non-isolated cases. METHODS: This retrospective cohort study selected 49 fetuses with CDH, a normal karyotype, and a lung-to-head ratio (LHR) of <1 from a single national referral center for fetal surgery in São Paulo, Brazil, between January 2016 and November 2019. FETO was performed between 26 and 29 weeks of gestation. The primary outcomes were infant survival until discharge from the neonatal intensive care unit and survival until six months of age. RESULTS: Forty-six women with singleton fetuses having severe CDH underwent prenatal intervention with FETO. Infant survival rates until discharge and at six months of age were both 38 %. The observed-to-expected LHR increased by 25 % after FETO in neonates who survived until discharge. Spontaneous intrauterine death occurred in four growth-restricted fetuses after FETO. Preterm birth in <37 weeks and preterm rupture of membranes in <34 weeks occurred in 56.5 % (26) and 26 % (12) cases, respectively. CONCLUSIONS: FETO may increase neonatal survival in fetuses with severe CDH, particularly in countries with limited neonatal intensive care.

Effects of serum sodium and chloride levels in the outcome of critically ill pediatric patients in the post-operative period of liver transplantation.

BACKGROUND: Sodium and chloride disturbances have attracted increasing attention in recent years. Many pathophysiological effects are associated with hyperchloremia, including reduction in mean arterial pressure and acute renal disease. Pediatric patients undergoing liver transplantation are at risk of developing various electrolyte and biochemical abnormalities, with an impact on their postoperative outcomes. OBJECTIVE: To analyze the impacts of serum sodium and chloride levels on prognosis of Pediatric Liver Transplant receptors. METHODS: This was a retrospective analytical observational study performed in a single transplant reference center in Sao Paulo, Brazil. Included patients were pediatric patients who underwent liver transplantation between January 2015 and July 2019. Statistical regression analysis and General Estimating Equations analysis were performed to evaluate the impacts of sodium and chloride disturbances on the development of acute renal failure and mortality. RESULTS: A total of 143 patients were included in this study. The main diagnosis was Biliary Atresia (62.9%). Twenty-seven patients died (18.9%), and graft dysfunction was the main cause of death (29.6%). The only variable individually associated with 28-days mortality was PIM-3 score (HR 1.59, CI 95% 1.165-2.177, p = 0.004). Forty-one patients (28.6%) developed moderate or severe AKI. PIM-3 score (OR 3.052, 95% CI 1.56-5.97, p = 0.001), hypernatremia (OR 3.49, 95% CI 1.32-9.23, p = 0.012), and hyponatremia (OR 4.24, 95% CI 1.52-11.85, p = 0.006) were independently associated with the development of moderate/severe AKI. CONCLUSIONS: In pediatric patients after liver transplantation, PIM-3 score, and abnormal serum sodium levels were correlated with AKI development.

Limits of therapeutic intervention in a tertiary neonatal intensive care unit in patients with major congenital anomalies in Brazil.

AIM: Major congenital anomalies are an important cause of death in the neonatal intensive care unit (NICU). Therapeutic interventions and the suspension of those already in place often raise ethical dilemmas in neonatal care. METHODS: We analysed treatments-such as ventilatory support, vasoactive drugs, antibiotics, sedation/or analgesia, central venous access and other invasive procedures-offered up to 48 h before death to all newborns with major congenital anomalies over a 3-year period in a NICU in Brazil. We also gathered information contained in medical records concerning conversations with the families and decisions to limit therapeutic interventions. RESULTS: We enrolled 74 newborns who were hospitalised from 1 January 2015 to 31 December 2017. A total of 81.1% had central venous access, 74.3% were on ventilatory support, 56.8% received antibiotics and 43.2% used some sedative/analgesic drugs in their final moments. Conversations were registered in medical records in 76% of cases, and 46% of the families chose therapeutic intervention limits. Those who chose to limit therapeutic interventions asked for less exposure to vasoactive drugs (P = 0.003) and antibiotics (P = 0.003), as well as fewer invasive procedures (P = 0.046). There was no change in ventilatory support (P = 0.66), and palliative extubation was not performed for any patient. CONCLUSIONS: The therapeutic intervention was mainly characterised by maintenance of the current treatment when a terminal situation was identified with no introduction of new treatments that could increase suffering. The families' approach proved to be essential for making difficult decisions in the NICU.

Admission hypothermia, neonatal morbidity, and mortality: evaluation of a multicenter cohort of very low birth weight preterm infants according to relative performance of the center.

This prospective cohort study aimed to assess the association of admission hypothermia (AH) with death and/or major neonatal morbidities among very low birth weight (VLBW) preterm infants based on the relative performance of 20 centers of the Brazilian Network of Neonatal Research. This is a retrospective analysis of prospectively collected data using the database registry of the Brazilian Network on Neonatal Research. Center performance was defined by the relative mortality rate using conditional inference trees. A total of 4356 inborn singleton VLBW preterm infants born between January 2013 and December 2016 without malformations were included in this study. The centers were divided into two groups: G1 (with lower mortality rate) and G2 (with higher mortality rate). Crude and adjusted relative risks (RR) and 95% confidence intervals (95%CI) were estimated by simple and multiple log-binomial regression models. An AH rate of 53.7% (19.8-93.3%) was significantly associated with early neonatal death in G1 (adjusted RR 1.41, 95% CI 1.09-1.84) and G2 (adjusted RR 1.29, 95%CI 1.01-1.65) and with in-hospital death in G1 (adjusted RR 1.29, 95%CI 1.07-1.58). AH was significantly associated with a lower frequency of necrotizing enterocolitis (adjusted RR 0.58, 95%CI 38-0.88) in G2.Conclusion: AH significantly associated with early neonatal death regardless of the hospital performance. In G2, an unexpected protective association between AH and necrotizing enterocolitis was found, whereas the other morbidities assessed were not significantly associated with AH. What is Known: • Admission hypothermia is associated with early neonatal death. • The association of admission hypothermia with major neonatal morbidities has not been fully established. What is New: • Admission hypothermia was significantly associated with early neonatal and in-hospital death in centers with the lowest relative mortality rates. • Admission hypothermia was not associated with major neonatal morbidities and with in-hospital death but was found to be a protective factor against necrotizing colitis in centers with the highest relative mortality rates.

Epidemiology and outcomes of acute respiratory distress syndrome in children according to the Berlin definition: a multicenter prospective study.

OBJECTIVES: In 2012, a new acute respiratory distress syndrome definition was proposed for adult patients. It was later validated for infants and toddlers. Our objective was to evaluate the prevalence, outcomes, and risk factors associated with acute respiratory distress syndrome in children up to 15 years according to the Berlin definition. DESIGN: A prospective, multicenter observational study from March to September 2013. SETTING: Seventy-seven PICU beds in eight centers: two private hospitals and six public academic hospitals in Brazil. PATIENTS: All children aged 1 month to 15 years admitted to the participating PICUs in the study period. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: All children admitted to the PICUs were daily evaluated for the presence of acute respiratory distress syndrome according to the American-European Consensus Conference and Berlin definitions. Of the 562 patients included, acute respiratory distress syndrome developed in 57 patients (10%) and 58 patients (10.3%) according to the Berlin definition and the American-European Consensus Conference definition, respectively. Among patients with acute respiratory distress syndrome according to the Berlin definition, nine patients (16%) were mild, 21 (37%) were moderate, and 27 (47%) were severe. Compared with patients without acute respiratory distress syndrome, patients with acute respiratory distress syndrome had significantly higher severity scores, longer PICU and hospital length of stay, longer duration of mechanical ventilation, and higher mortality (p < 0.001). The presence of two or more comorbidities and admission for medical reasons were associated with development of acute respiratory distress syndrome. Comparisons across the three the Berlin categories showed significant differences in the number of ventilator-free days (21, 20, and 5 d, p = 0.001) and mortality for severe acute respiratory distress syndrome (41%) in comparison with mild (0) and moderate (15%) acute respiratory distress syndrome(p = 0.02). No differences in PICU or hospital stay were observed across the groups. CONCLUSIONS: The Berlin definition can identify a subgroup of patients with distinctly worse outcomes, as shown by the increased mortality and reduced number of ventilator-free days in pediatric patients with severe acute respiratory distress syndrome.

Focused cardiac ultrasound: a training course for pediatric intensivists and emergency physicians.

BACKGROUND: Focused echocardiographic examinations performed by intensivists and emergency room physicians can be a valuable tool for diagnosing and managing the hemodynamic status of critically ill children. The aim of this study was to evaluate the learning curve achieved using a theoretical and practical training program designed to enable pediatric intensivists and emergency physicians to conduct targeted echocardiograms. METHODS: Theoretical and practical training sessions were conducted with 16 pediatric intensivist/emergency room physicians. The program included qualitative analyses of the left ventricular (LV) and right ventricular (RV) functions, evaluation of pericardial effusion/cardiac tamponade and valvular regurgitation and measurements of the distensibility index of the inferior vena cava (dIVC), ejection fraction (EF) and cardiac index (CI). The practical training sessions were conducted in the intensive care unit; each student performed 24 echocardiograms. The students in training were evaluated in a practical manner, and the results were compared with the corresponding examinations performed by experienced echocardiographers. The evaluations occurred after 8, 16 and 24 practical examinations. RESULTS: The concordance rates between the students and echocardiographers in the subjective analysis of the LV function were 81.3% at the first evaluation, 96.9% at the second evaluation and 100% at the third evaluation (p < 0.001). For the dIVC, we observed a concordance of 46.7% at the first evaluation, 90.3% at the second evaluation and 87.5% at the third evaluation (p = 0.004). The means of the differences between the students' and echocardiographers' measurements of the EF and CI were 7% and 0.56 L/min/m2, respectively, after the third stage of training. CONCLUSIONS: The proposed training was demonstrated to be sufficient for enabling pediatric physicians to analyze subjective LV function and to measure dIVC, EF and CI. This training course should facilitate the design of other echocardiography training courses that could be implemented in medical residency programs to improve these physicians' technical skills and the care of critically ill patients.

Major discrepancy between clinical diagnosis of death and anatomopathological findings in adolescents with chronic diseases during 18-years.

OBJECTIVES: To evaluate the inconsistency between clinical diagnosis of death and autopsy findings in adolescents with chronic diseases. METHODS: A cross-sectional study including a sample of adolescents' autopsies who died in a pediatric and adolescent tertiary hospital over 18 consecutive years. During this period, there were n = 2912 deaths, and n = 581/2912(20%) occurred in adolescents. Of these, n = 85/581(15%) underwent autopsies and were analyzed. Further results were divided into two groups: Goldman classes I or II (high disagreement between main clinical diagnosis of death and anatomopathological findings, n = 26) and Goldman classes III, IV or V (low or no disagreement between these two parameters, n = 59). RESULTS: Median age at death (13.5 [10‒19] vs. 13 [10‒19] years, p = 0.495) and disease duration (22 [0‒164] vs. 20 [0‒200] months, p = 0.931), and frequencies for males (58% vs. 44%, p = 0.247) were similar between class I/II vs. class III/IV/V. The frequency of pneumonia (73% vs. 48%, p = 0.029), pulmonary abscess (12% vs. 0%, p = 0.026), as well as isolation of yeast (27% vs. 5%, p = 0.008), and virus (15% vs. 2%, p = 0.029) identified in the autopsy, were significantly higher in adolescents with Goldman class I/II compared to those with Goldman class III/IV/V. In contrast, cerebral edema was significantly lower in adolescents of the first group (4% vs. 25%, p = 0.018). CONCLUSION: This study showed that 30% of the adolescents with chronic diseases had major discrepancies between clinical diagnosis of death and autopsy findings. Pneumonia, pulmonary abscess, as well as isolation of yeast and virus were more frequently identified at autopsy findings in the groups with major discrepancies.

Use of right ventricular remodeling surgery with a porcine pulmonary prosthesis for congenital heart disease.

BACKGROUND: The reconstruction of the right ventricular outflow tract (RVOT) in congenital heart disease has attracted the interest of cardiac surgeons determined to alleviate the anatomic obstruction and restore RV function. METHODS: From June 1991 to September 2008, 203 consecutive patients (mean, 3.0 years; range, 2 months to 35 years) underwent operations. These patients were classified into 5 groups: group 1, tetralogy of Fallot with pulmonary hypoplasia (144 cases, 70.9%); group 2, pulmonary atresia (PA) with ventricular septal defect (VSD) (32 cases, 15.7%); group 3, truncus arteriosus (12 cases, 5.9%); group 4, transposition of the great arteries with left ventricular outflow tract obstructions (8 cases, 3.9%); and group 5, PA with intact ventricular septum (7 cases, 3.4%). Remodeling surgery of the RV consisted of patch closure of the VSD (n = 176), tricuspid valvoplasty repair (n = 25), infundibulum muscle resection, and reconstruction of the RVOT (all patients). The Lecompte procedure was performed in 8 patients in group 4, and the one and a half ventricle technique was performed in 7 patients in group 5. RESULTS: There were 21 hospital deaths (10.3%); 180 patients (88.6%) survived. Patients were followed up from 4 to 206 months (mean, 98.0 months). Sixteen patients (8.8%) underwent reoperation for prosthesis dysfunction, with 2 in-hospital deaths (12.5%). The rest of the patients (164, 80.7%) remain free of reoperation. CONCLUSION: Earlier reconstruction of the pulmonary valve and the RVOT may preserve ventricular performance for a long period. Nevertheless, the porcine pulmonary prosthesis has shown satisfactory results when it has been used for the reconstruction of different types of RV obstructions.

Association between ventilatory settings and pneumothorax in extremely preterm neonates.

OBJECTIVES: Pneumothorax is a catastrophic event associated with high morbidity and mortality, and it is relatively common in neonates. This study aimed to investigate the association between ventilatory parameters and the risk of developing pneumothorax in extremely low birth weight neonates. METHODS: This single-center retrospective cohort study analyzed 257 extremely low birth weight neonates admitted to a neonatal intensive care unit between January 2012 and December 2017. A comparison was carried out to evaluate the highest value of positive end-expiratory pressure (PEEP), peak inspiratory pressure (PIP), and driving pressure (DP) in the first 7 days of life between neonates who developed pneumothorax and those who did not. The primary outcome was pneumothorax with chest drainage necessity in the first 7 days of life. A matched control group was created in order to adjust for cofounders associated with pneumothorax (CRIB II score, birth weight, and gestational age). RESULTS: There was no statistically significant difference in PEEP, PIP, and DP values in the first 7 days of life between extremely low birth weight neonates who had pneumothorax with chest drainage necessity and those who did not have pneumothorax, even after adjusting for potential cofounders. CONCLUSIONS: Pressure-related ventilatory settings in mechanically ventilated extremely low birth weight neonates are not associated with a higher risk of pneumothorax in the first 7 days of life.

Impact of sustained neuromuscular blockade on outcome of mechanically ventilated children.

BACKGROUND: Neuromuscular blocking agents (NMBA) are commonly administered to critically ill children in pediatric intensive care units (PICU) in the USA and Europe. Although NMBA are frequently used in PICU patients, their role in the PICU setting has not yet been clearly defined. The aim of this study was to describe the sustained administration of NMBA and its impact on outcome of PICU patients. METHODS: A 3-year retrospective cohort study was conducted to compare mechanically-ventilated patients who received NMBA for at least 12 h with patients who did not (control group). RESULTS: A total of 317 consecutive patients were ventilated over 3473 days. Patients were similar in age, weight and severity scores. Thirty-four children (10.7%) received NMBA. Compared with controls, the neuromuscular blockade (NMB) group had a longer duration of mechanical ventilation (13.7 vs 5.5 days, P= 0.000), longer PICU stay (20 vs 11 days, P= 0.000) and increased occurrence of ventilator-associated pneumonia (6.6 vs 4.1/1000 ventilator days, P= 0.010). The NMB use was not associated with higher mortality (8.8% vs 17.6%, P= 0.287) or longer hospital stay (30.5 vs 23 days, P= 0.117). CONCLUSION: Although the use of NMBA was not associated with greater mortality, we found that sustained use of NMBA is associated with prolonged mechanical ventilation, longer PICU stay and higher incidence of ventilator-associated pneumonia when compared with controls. Larger studies are necessary to confirm these findings.

Intracardiac cavopulmonary connection in patients with univentricular heart using intra-atrial lateral tunnel and intra-atrial conduit techniques.

BACKGROUND: In this study, we analyzed the time course of hemodynamic efficiency and follow-up in Fontan candidates who underwent the bidirectional Glenn procedure for staged intracardiac cavopulmonary connection (ICPC). METHODS: Between 1991 and 2008, 52 patients with univentricular heart (mean age, 3.3 years; range, 2-8 years; 27 female patients [51.9%]) underwent ICPC. The cardiac malformations were as follows: tricuspid atresia, 25 cases (48.0%); common ventricle, 16 cases (30.7%); and pulmonary atresia with intact ventricular septum, 11 cases (21.1%). The intracardiac cavopulmonary procedure was indicated for all 52 cases. In 42 patients (80.7%), an intra-atrial lateral tunnel was constructed with a bovine pericardium patch. In the last 10 consecutive cases (19.3%), we performed a modified surgical technique in which we implanted an intra-atrial corrugated bovine pericardium tube sutured around the superior and inferior vena cava ostium. In all cases, a 4-mm fenestration was made to reduce the intratunnel pressure. All 52 patients had previously undergone a Glenn operation. RESULTS: There were 2 hospital deaths (3.8%) and no recorded late deaths. During the follow-up, all patients were medicated with antiplatelet drugs. To evaluate the hemodynamic performance, we used Doppler echocardiography, computed tomography, and magnetic nuclear resonance studies. There were no prosthesis thromboses during this followup period. To evaluate cardiac arrhythmias, we conducted a Holter study. The last 10 patients with an intra-atrial conduit (IAC) presented with sinus rhythm and no arrhythmias during the last 4 years. The 50 surviving patients (96.1%) have been followed up for 6 to 204 months; all these patients are free of reoperation. CONCLUSION: The Glenn operation, which is performed at an early age, prepares the pulmonary bed to receive the ICPC. The midterm results of the intracardiac Fontan procedure seem to be good. The modified surgical procedure (IAC) can be a good alternative technique to the Fontan procedure in suitable patients.

Association between fluid creep and infection in burned children: A cohort study.

INTRODUCTION: Aggressive fluid resuscitation has been extensively discussed after the establishment of fluid creep phenomenon as a morbidity and mortality factor in burn children. Sepsis is currently the leading cause of death in survivors of burn shock. OBJECTIVES: To evaluate the association between fluid creep and infection in burn children exposed to two different fluid resuscitation strategies with the use of albumin. METHODS: A cohort of 46 burn children with 15-45% of body surface area (BSA) admitted up to 12 h after the incident were evaluated. Patients from early albumin group (n = 23) received 5% albumin between 8 and 12 h from injury and patients from delayed albumin group (n = 23) received 5% albumin after 24 h. Outcomes analysed were development of fluid creep, length of stay in the hospital, number of surgery procedures and infection until hospital discharge. RESULTS: Compared to the delayed group, patients that received early albumin had a shorter length of stay in the hospital (p = 0.007), less fluid creep (4.3% × 56.5%) (p < 0.001), less skin graft procedure (47.8% × 78.3%) (p = 0.032) and less debridement (73.9% × 100%) (p = 0.022). Both length of stay in the hospital and fluid creep arising were associated with infection (p < 0.05). CONCLUSION: Fluid creep, surgery procedures and length of stay in hospital parameters showed better results in burn children treated with early albumin. Fluid creep and length of stay in the hospital were associated with infection, providing a negative prognosis.

Differences among Severe Cases of Sars-CoV-2, Influenza, and Other Respiratory Viral Infections in Pediatric Patients: Symptoms, Outcomes and Preexisting Comorbidities.

OBJECTIVES: Previous studies focusing on pediatric patients hospitalized with severe coronavirus disease 2019 (COVID-19) have been limited to small case series. We aimed to evaluate the characteristics of a large population of pediatric patients with severe COVID-19 and compare them with patients with severe cases of influenza and other respiratory viruses (ORV). METHODS: We performed a cross-sectional study of Brazilian data from the National Epidemiological Surveillance Information System, gathered from January 1st to July 14th, 2020. The sample included 4,784 patients (2,570 with confirmed COVID-19, 659 with influenza, 1,555 with ORV). Outcome measures included clinical features, preexisting comorbidities, pediatric intensive care unit admissions, need for ventilatory support, and death. RESULTS: Compared with the influenza and ORV groups, the COVID-19 group had a higher proportion of newborns and adolescents, as well as lower frequencies of fever, cough, dyspnea, respiratory distress, and desaturation. Although use of invasive ventilatory support was similar among groups, death rate was highest for COVID-19 (15.2% vs. 4.5% vs. 3.2%, p<0.001), with death risk more than three times the other groups (adjusted OR=3.7 [95% CI 2.5-5.6]). The presence of two or more comorbidities further increased this risk (OR=4.8 [95% CI 3.5-6.6]). Preexisting comorbidities were reported in 986 patients with severe COVID-19 (38%). Mortality rate among COVID-19 patients was significantly higher for almost all comorbidities reported. CONCLUSION: Severe COVID-19 had a higher mortality rate than other viral respiratory illnesses, despite the lower frequency of fever, cough, dyspnea, respiratory distress, and desaturation. Death risk was strongly associated with preexisting comorbidities.

Point-of-care lung ultrasound imaging in pediatric COVID-19.

BACKGROUND: There has been limited data regarding the usefulness of lung ultrasound (US) in children with COVID-19. OBJECTIVE: To describe lung US imaging findings and aeration score of 34 children with COVID-19. METHODS: This study included 0-16-year-old patients with confirmed COVID-19, who were admitted between April 19 and June 18, 2020 in two hospitals in the city of Sao Paulo, Brazil. Lung US was performed as part of the routine evaluation by a skilled Pediatric Emergency physician. Clinical and laboratory data were collected and severity classifications were done according to an available clinical definition. The lung US findings were described for each lung field and a validated ultrasound lung aeration score was calculated. Data obtained was correlated with clinical information and other imaging modalities available for each case. RESULTS: Thirty-four confirmed COVID-19 patients had a lung US performed during this period. Eighteen (18/34) had abnormalities on the lung US, but eight of them (8/18) had a normal chest radiograph. Ultrasound lung aeration score medians for severe/critical, moderate, and mild disease were 17.5 (2-30), 4 (range 0-14), 0 (range 0-15), respectively (p = 0.001). Twelve patients (12/34) also had a chest computed tomography (CT) performed; both the findings and topography of lung compromise on the CT were consistent with the information obtained by lung US. CONCLUSION: Point-of-care lung US may have a key role in assessing lung injury in children with COVID-19.

Abdominal compartment syndrome: A review.

OBJECTIVES: The aims of this review were to summarize a) the consensus definitions of normal and pathologic intra-abdominal pressure (IAP); b) the techniques to measure IAP; c) the risk factors for intra-abdominal hypertension (IAH) and abdominal compartment syndrome (ACS); d) the pathophysiology of ACS; and e) the current recommendations for management and prevention of ACS. DATA SOURCES: PubMed was searched using the following terms: ACS, IAH, IAP, and abdominal decompression. DATA SYNTHESIS: ACS represents the natural progression of end-organ dysfunction caused by increased IAP and develops if IAH is not recognized and treated appropriately. Although the reported incidence of ACS is relatively low in critically ill children (0.6%-4.7%) it may be under-recognized and under-reported. The diagnosis of IAH/ACS depends on a high index of suspicion and the accurate and frequent measurement of IAP in patients at risk. Mortality from ACS remains high (50%-60%) even when decompression of the abdomen is performed early, which highlights the importance of detection and treatment of elevated IAP before end-organ damage occurs. CONCLUSIONS: A widespread awareness of the recognition and current approach to management and prevention of IAH and ACS is needed among pediatric intensivists, so outcome of these life-threatening disease processes might be improved.