Masked Hypertension in Pediatric Heart Transplant Recipients.

BACKGROUND: Masked hypertension (HTN), especially, isolated nocturnal HTN (INH) has been shown to be a risk factor for cardiovascular disease (CVD) but is not studied well in pediatric heart transplant (PHT) patients. Ambulatory blood pressure monitoring (ABPM) is known to identify patients with HTN but is not used routinely in PHT. METHODS: A single-center, prospective, cross-sectional study of PHT recipients was performed to observe the incidence of masked HTN using 24-h ABPM. The relationship between ABPM parameters and clinical variables was assessed using Spearman correlation coefficient. p value < 0.05 was considered significant. RESULTS: ABPM was performed in 34 patients, mean age 14 ± 5 years, median 5.5 years post-PHT. All patients had normal cardiac function, left ventricular mass index and blood pressure measurements in the clinic. Four patients had known prior HTN and on medications, one of them was uncontrolled. Of the remaining 30 patients, 18 new patients were diagnosed with masked HTN, of which 14 had INH. Diurnal variation was abnormal in 82% (28/34) patients. 24-h diastolic blood pressure (DBP) index correlated with glomerular filtration rate (GFR) (r = - 0.44, p = 0.01). There was no correlation between other ABPM parameters with tacrolimus trough levels. CONCLUSIONS: ABPM identified masked HTN in 60% of patients, with majority being INH. Abnormal circadian BP patterns were present in 82% and an association was found between GFR and DBP parameters. HTN, especially INH, is under-recognized in PHT recipients and ABPM has a role in their long-term care.

Use of bivalirudin for anticoagulation in pediatric extracorporeal membrane oxygenation (ECMO).

This study describes the use of bivalirudin in children on extracorporeal membrane oxygenation (ECMO). Pediatric patients receiving bivalirudin were compared to patients receiving heparin as the anticoagulant on ECMO. Data was collected for children under 18 years of age supported by ECMO from January 2016 to December 2019. Data collected included demographics, diagnosis, ECMO indication, type, and duration, indication for bivalirudin use, dose range, activated partial thromboplastin time (aPTT) levels, minor and major bleeding, hemolysis, and mortality. Forty pediatric patients received ECMO; eight received bivalirudin primarily for anticoagulation. The median age was 4 months (IQR 0.5, 92) in the heparin cohort, 0.6 months (IQR 0.0, 80.0) in the primary bivalirudin cohort. The indication for ECMO was respiratory in 5 patients (18%) in the heparin group versus 6 (75%) in the primary bivalirudin group, cardiac in 18 (67%) in heparin versus 1 (12.5%) in primary bivalirudin, and extracorporeal-cardiopulmonary resuscitation (E-CPR) in 4 (15%) in heparin versus 1 (12.5%) in primary bivalirudin. Bivalirudin was the initial anticoagulant for eight patients (66.6%) while three (25%) were switched due to concern for heparin-induced thrombocytopenia (HIT) and one (8%) for heparin resistance. The median time to achieve therapeutic aPTT was 14.5 hours compared to 12 hours in the heparin group. Sixty-five percent of aPTT values in the bivalirudin and 44% of values in the heparin group were in the therapeutic range in the first 7 days. Patients with primary bivalirudin use had significantly lower dose requirement at 12 (p = 0.003), 36 (p = 0.007), and 48 (p = 0.0002) hours compared to patients with secondary use of bivalirudin. One patient (12.5%) had major bleeding, and two patients (25%) required circuit change in the primary bivalirudin cohort. Bivalirudin may provide stable and successful anticoagulation in children. Further large, multicenter studies are needed to confirm these findings.

Characterization and Outcomes of Hospitalized Children With Coronavirus Disease 2019: A Report From a Multicenter, Viral Infection and Respiratory Illness Universal Study (Coronavirus Disease 2019) Registry.

OBJECTIVES: Multicenter data on the characteristics and outcomes of children hospitalized with coronavirus disease 2019 are limited. Our objective was to describe the characteristics, ICU admissions, and outcomes among children hospitalized with coronavirus disease 2019 using Society of Critical Care Medicine Discovery Viral Infection and Respiratory Illness Universal Study: Coronavirus Disease 2019 registry. DESIGN: Retrospective study. SETTING: Society of Critical Care Medicine Viral Infection and Respiratory Illness Universal Study (Coronavirus Disease 2019) registry. PATIENTS: Children (< 18 yr) hospitalized with coronavirus disease 2019 at participating hospitals from February 2020 to January 2021. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The primary outcome was ICU admission. Secondary outcomes included hospital and ICU duration of stay and ICU, hospital, and 28-day mortality. A total of 874 children with coronavirus disease 2019 were reported to Viral Infection and Respiratory Illness Universal Study registry from 51 participating centers, majority in the United States. Median age was 8 years (interquartile range, 1.25-14 yr) with a male:female ratio of 1:2. A majority were non-Hispanic (492/874; 62.9%). Median body mass index (n = 817) was 19.4 kg/m2 (16-25.8 kg/m2), with 110 (13.4%) overweight and 300 (36.6%) obese. A majority (67%) presented with fever, and 43.2% had comorbidities. A total of 238 of 838 (28.2%) met the Centers for Disease Control and Prevention criteria for multisystem inflammatory syndrome in children, and 404 of 874 (46.2%) were admitted to the ICU. In multivariate logistic regression, age, fever, multisystem inflammatory syndrome in children, and pre-existing seizure disorder were independently associated with a greater odds of ICU admission. Hospital mortality was 16 of 874 (1.8%). Median (interquartile range) duration of ICU (n = 379) and hospital (n = 857) stay were 3.9 days (2-7.7 d) and 4 days (1.9-7.5 d), respectively. For patients with 28-day data, survival was 679 of 787, 86.3% with 13.4% lost to follow-up, and 0.3% deceased. CONCLUSIONS: In this observational, multicenter registry of children with coronavirus disease 2019, ICU admission was common. Older age, fever, multisystem inflammatory syndrome in children, and seizure disorder were independently associated with ICU admission, and mortality was lower among children than mortality reported in adults.

Acute Kidney Injury in Pediatric Acute Respiratory Distress Syndrome.

OBJECTIVES: To describe the incidence of and risk factors for acute kidney injury (AKI) in children with acute respiratory distress syndrome (ARDS) and study the effect of AKI on patient outcomes. DESIGN: A single-center retrospective study. SETTING: A tertiary care children's hospital. PATIENTS: All patients less than 18 years of age who received invasive mechanical ventilation (MV) and developed ARDS between July 2010 and July 2013 were included. Acute kidney injury was defined using p-RIFLE (risk, injury, failure, loss, and end-stage renal disease) criteria. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: One hundred fifteen children met the criteria and were included in the study. Seventy-four children (74/115, 64%) developed AKI. The severity of AKI was risk in 34 (46%) of 74, injury in 19 (26%) of 74, and failure in 21 (28%) of 74. The presence of AKI was associated with lower Pao 2 to Fio 2 (P/F) ratio (P = .007), need for inotropes (P = .003), need for diuretics (P = .004), higher oxygenation index (P = .03), higher positive end-expiratory pressure (PEEP; P = .01), higher mean airway pressure (P = .008), and higher Fio 2 requirement (P = .03). Only PEEP and P/F ratios were significantly associated with AKI in the unadjusted logistic regression model. Patients with AKI had a significantly longer duration of hospital stay, although there was no significant difference in the intensive care unit stay, duration of MV, and mortality. Recovery of AKI occurred in 68% of the patients. A multivariable model including PEEP, P/F ratio, weight, need for inotropes, and need for diuretics had a better receiver operating characteristic (ROC) curve with an AUC of 0.75 compared to the ROC curves for PEEP only and P/F ratio only for the prediction of AKI. CONCLUSIONS: Patients with ARDS have high rates of AKI, and its presence is associated with increased morbidity and mortality.

Pediatric Acute Respiratory Distress Syndrome (PARDS) in Children With Pulmonary Contusion.

OBJECTIVE: There is paucity of data about prevalence of pediatric acute respiratory distress syndrome (PARDS) in children with pulmonary contusion (PC). We intend to evaluate PC in children with chest trauma and the association between PC and PARDS. DESIGN: Retrospective review of Institutional Trauma Registry for patients with trauma. SETTING: Level 1 trauma center. PATIENTS: Age 18 years and younger with a diagnosis of PC. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Of the 1916 children with trauma, 50 (2.6%) had PC. Patients with PC and PARDS had lower Glasgow Coma Scale (GCS) score (7 [3-15] vs 15 [15-15], P = .0003), higher Injury Severity Scale (ISS) score (29 [22-34] vs 19 [14-22], P = .004), lower oxygen saturations (96 [93-99] days vs 99 [98-100] days, P = .0009), higher FiO2 (1 [1-1] vs 0.21 [0.21-0.40], P < .0001), lower oxygen saturation/FiO2 (S/F) ratios (97 [90-99] vs 457 [280-471], P < .0001), need for invasive mechanical ventilation (IMV; 86% vs 23%, P < .0001), and mortality (28% vs 0%, P = .006) compared to those without PARDS. Forty-two percent (21/50) of patients needed IMV, of these 61% (13/21) had PARDS. Patients who needed IMV had significantly lower GCS score (8 [3-11] vs 15 [15-15], P < .0001), higher ISS score (27 [22-34] vs 18 [14-22], P = .002), longer length of stay (LOS; 7.5 [4-14] days vs 3.3 [2-5] days, P = .003), longer hospital LOS (18 [7.0-25] vs 5 [4-11], P = .008), higher PARDS rate (62% vs 7%, P < .0001), and lower S/F ratios (99 [94-190] vs 461 [353-471], P < .0001) compared to those who did not require IMV. Lower GCS score was independently associated with both PARDS and need for IMV. CONCLUSIONS: Pediatric ARDS in children with PC is independently associated with lower GCS score, and its presence significantly increased morbidity and mortality. Further larger studies are needed to explore association of lower GCS and higher injury score in children with PARDS and PC.

Left Heart Decompression on Veno-Arterial Extracorporeal Membrane Oxygenation in Children With Dilated Cardiomyopathy and Myocarditis: An Extracorporeal Life Support Organization Registry Review.

OBJECTIVES: To describe the association between left heart decompression on veno-arterial extracorporeal membrane oxygenation and survival in patients with myocarditis and dilated cardiomyopathy. The secondary outcome is to study association of left heart decompression with survival in children with myocarditis compared with those with dilated cardiomyopathy. DESIGN: Retrospective study of a multicenter registry database. SETTING: Data reported to Extracorporeal Life Support Organization from international extracorporeal membrane oxygenation centers. PATIENTS: Patients less than or equal to 18 years old with a diagnosis of myocarditis or dilated cardiomyopathy receiving extracorporeal membrane oxygenation. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 1,438 pediatric extracorporeal membrane oxygenation runs were identified. Thirty-seven percent of the patients had myocarditis (n = 532), whereas the rest had dilated cardiomyopathy. Survival to hospital discharge was 63%. Median extracorporeal membrane oxygenation duration was 148 hours with interquartile range (84-248 hr). Nineteen percent of patients (n = 274) had left heart decompression. Multivariable analysis revealed using left heart decompression (adjusted odds ratio, 1.42; 95% CI, 1.06-1.89; p = 0.02), e-cardiopulmonary resuscitation (adjusted odds ratio, 0.63; 95% CI, 0.51-0.79; p < 0.001), higher pH (adjusted odds ratio, 3.69; 95% CI, 1.80-7.53; p < 0.001), and diagnosis of myocarditis (adjusted odds ratio, 1.69; 95% CI, 1.35-2.08; p < 0.001) were associated with greater odds of survival. In the multivariable analysis for patients with dilated cardiomyopathy, left heart decompression failed to reveal a significant association with survival (20% among survivors vs 17% among nonsurvivors, 95% CI, -2.2% to 8.0%). Meanwhile in patients with myocarditis, the multivariable analysis failed to exclude the possibility that left heart decompression was associated with up to a three-fold greater odds of survival (adjusted odds ratio, 1.77; 95% CI, 0.99-.15). CONCLUSIONS: Retrospective review of the Extracorporeal Life Support Organization registry revealed an association between left heart decompression and greater odds of survival in children with myocarditis and dilated cardiomyopathy on extracorporeal membrane oxygenation. When comparing patients with dilated cardiomyopathy against those with myocarditis, we could not exclude a three-fold greater odds of survival associated with the use of left heart decompression. This finding warrants further prospective evaluation.

Clinical Characteristics and Outcomes of Hospitalized and Critically Ill Children and Adolescents with Coronavirus Disease 2019 at a Tertiary Care Medical Center in New York City.

OBJECTIVE: To describe the clinical profiles and risk factors for critical illness in hospitalized children and adolescents with coronavirus disease 2019 (COVID-19). STUDY DESIGN: Children 1 month to 21 years of age with COVID-19 from a single tertiary care children's hospital between March 15 and April 13, 2020 were included. Demographic and clinical data were collected. RESULTS: In total, 67 children tested positive for COVID-19; 21 (31.3%) were managed as outpatients. Of 46 admitted patients, 33 (72%) were admitted to the general pediatric medical unit and 13 (28%) to the pediatric intensive care unit (PICU). Obesity and asthma were highly prevalent but not significantly associated with PICU admission (P = .99). Admission to the PICU was significantly associated with higher C-reactive protein, procalcitonin, and pro-B type natriuretic peptide levels and platelet counts (P < .05 for all). Patients in the PICU were more likely to require high-flow nasal cannula (P = .0001) and were more likely to have received Remdesivir through compassionate release (P < .05). Severe sepsis and septic shock syndromes were observed in 7 (53.8%) patients in the PICU. Acute respiratory distress syndrome was observed in 10 (77%) PICU patients, 6 of whom (46.2%) required invasive mechanical ventilation for a median of 9 days. Of the 13 patients in the PICU, 8 (61.5%) were discharged home, and 4 (30.7%) patients remain hospitalized on ventilatory support at day 14. One patient died after withdrawal of life-sustaining therapy because of metastatic cancer. CONCLUSIONS: We describe a higher than previously recognized rate of severe disease requiring PICU admission in pediatric patients admitted to the hospital with COVID-19.

Multisystem Inflammatory Syndrome in Children Associated with Severe Acute Respiratory Syndrome Coronavirus 2 Infection (MIS-C): A Multi-institutional Study from New York City.

OBJECTIVE: To assess clinical characteristics and outcomes of severe acute respiratory syndrome coronavirus 2-associated multisystem inflammatory syndrome in children (MIS-C). STUDY DESIGN: Children with MIS-C admitted to pediatric intensive care units in New York City between April 23 and May 23, 2020, were included. Demographic and clinical data were collected. RESULTS: Of 33 children with MIS-C, the median age was 10 years; 61% were male; 45% were Hispanic/Latino; and 39% were black. Comorbidities were present in 45%. Fever (93%) and vomiting (69%) were the most common presenting symptoms. Depressed left ventricular ejection fraction was found in 63% of patients with median ejection fraction of 46.6% (IQR, 39.5-52.8). C-reactive protein, procalcitonin, d-dimer, and pro-B-type natriuretic peptide levels were elevated in all patients. For treatment, intravenous immunoglobulin was used in 18 (54%), corticosteroids in 17 (51%), tocilizumab in 12 (36%), remdesivir in 7 (21%), vasopressors in 17 (51%), mechanical ventilation in 5 (15%), extracorporeal membrane oxygenation in 1 (3%), and intra-aortic balloon pump in 1 (3%). The left ventricular ejection fraction normalized in 95% of those with a depressed ejection fraction. All patients were discharged home with median duration of pediatric intensive care unit stay of 4.7 days (IQR, 4-8 days) and a hospital stay of 7.8 days (IQR, 6.0-10.1 days). One patient (3%) died after withdrawal of care secondary to stroke while on extracorporeal membrane oxygenation. CONCLUSIONS: Critically ill children with coronavirus disease-2019-associated MIS-C have a spectrum of severity broader than described previously but still require careful supportive intensive care. Rapid, complete clinical and myocardial recovery was almost universal.

Clinical Manifestations and Outcomes of Critically Ill Children and Adolescents with Coronavirus Disease 2019 in New York City.

OBJECTIVES: To describe the clinical manifestations and outcomes of critically ill children with coronavirus disease-19 (COVID-19) in New York City. STUDY DESIGN: Retrospective observational study of children 1 month to 21 years admitted March 14 to May 2, 2020, to 9 New York City pediatric intensive care units (PICUs) with severe acute respiratory syndrome coronavirus 2 infection. RESULTS: Of 70 children admitted to PICUs, median age was 15 (IQR 9, 19) years; 61.4% male; 38.6% Hispanic; 32.9% black; and 74.3% with comorbidities. Fever (72.9%) and cough (71.4%) were the common presenting symptoms. Twelve patients (17%) met severe sepsis criteria; 14 (20%) required vasopressor support; 21 (30%) developed acute respiratory distress syndrome (ARDS); 9 (12.9%) met acute kidney injury criteria; 1 (1.4%) required renal-replacement therapy, and 2 (2.8%) had cardiac arrest. For treatment, 27 (38.6%) patients received hydroxychloroquine; 13 (18.6%) remdesivir; 23 (32.9%) corticosteroids; 3 (4.3%) tocilizumab; and 1 (1.4%) anakinra; no patient was given immunoglobulin or convalescent plasma. Forty-nine (70%) patients required respiratory support: 14 (20.0%) noninvasive mechanical ventilation, 20 (28.6%) invasive mechanical ventilation (IMV), 7 (10%) prone position, 2 (2.8%) inhaled nitric oxide, and 1 (1.4%) extracorporeal membrane oxygenation. Nine (45%) of the 20 patients requiring IMV were extubated by day 14 with median IMV duration of 218 (IQR 79, 310.4) hours. Presence of ARDS was significantly associated with duration of PICU and hospital stay, and lower probability of PICU and hospital discharge at hospital day 14 (P < .05 for all). CONCLUSIONS: Critically ill children with COVID-19 predominantly are adolescents, have comorbidities, and require some form of respiratory support. The presence of ARDS is significantly associated with prolonged PICU and hospital stay.

Use of bivalirudin as a primary anticoagulant in a child during Berlin Heart EXCOR ventricular assist device support.

We describe our experience of bivalirudin use, a newer direct thrombin inhibitor, in an infant who was supported with Berlin Heart EXCOR VAD (Berlin VAD) as bridge to transplant for 122 days without complications and without need for pump exchange. An 11-month-old girl with dilated cardiomyopathy with acute heart failure was awaiting cardiac transplant. Lack of improvement despite maximizing medical therapy and anticipating a prolonged waitlist time, she was supported with Berlin LVAD as a bridge to transplant. Anticoagulation with bivalirudin was started and titrated with a goal partial thromboplastin time of 60-90 seconds. Therapeutic anticoagulation was achieved with bivalirudin for 50% of the days (61/122 days) on a dose of 2.1 mg/kg/hour and in a narrow dose range of 1.9 to 2.3 mg/kg/hour for 80% of the days (98/122 days). Antiplatelet regimen was started initially with aspirin and clopidogrel added later. She was supported for 122 days on a single pump without any evidence of thrombus or need for pump change. Berlin VAD explant and orthotopic heart transplant with biatrial anastomosis were performed uneventfully. Explanted Berlin VAD had no evidence of clot/fibrin or thrombus formation. The child was discharged to home uneventfully 15 days after cardiac transplant.

Acute Kidney Injury in Pediatric Acute Decompensated Heart Failure.

OBJECTIVE: Acute kidney injury in adult patients with acute decompensated heart failure is associated with increased mortality. There is limited literature in pediatric patients with acute decompensated heart failure and acute kidney injury. We aim to study acute kidney injury in the pediatric acute decompensated heart failure population and its association with specific outcomes. DESIGN: Retrospective, case-control study. SETTING: Cardiac ICU in a children's tertiary care hospital. PATIENTS: Index admissions of patients younger than 21 years with acute decompensated heart failure between January 2008 and December 2012. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Index admissions of patients younger than 21 years with acute decompensated heart failure between January 2008 and December 2012 were reviewed, and the presence or absence of acute kidney injury at admission was determined based on the Pediatric Risk, Injury, Failure, Loss, End-Stage criteria. Descriptive statistics and multivariate analyses were performed to determine the association between acute kidney injury and a composite outcome of cardiac transplantation and/or mortality. Fifty-seven patients, with median age 12 years (interquartile range, 1.1, 16), were included for study. The median left ventricular ejection fraction was 27% (interquartile range, 18, 48). Twenty-one patients (36%) underwent cardiac transplantation and five patients (8.7%) died. Of the 57 patients, 44 (77%) had evidence of acute kidney injury (41% Risk; 39% Injury; 20% Failure). Of the 44 patients with acute kidney injury, 25 (57%) met the composite outcome, compared with 1 (7%) without acute kidney injury. Multivariate analyses demonstrated that a left ventricular ejection fraction up to 25% was significantly associated with the presence of acute kidney injury (adjusted odds ratio, 12.3; 95% CI, 1.4-109; p = 0.03), and acute kidney injury was significantly associated with the composite outcome (adjusted odds ratio, 19.1; 95% CI, 2.3-160; p < 0.001). CONCLUSIONS: Acute kidney injury is common during the initial presentation of pediatric patients with acute decompensated heart failure. A left ventricular ejection fraction up to 25% is associated with acute kidney injury. The presence of acute kidney injury in this population is significantly associated with cardiac transplantation and/or death.

Volumetric Capnography in Pediatric Extracorporeal Membrane Oxygenation: A Case Series.

The kinetics of carbon dioxide elimination (VCO 2 ) may be used as a surrogate for pulmonary blood flow. As such, we can apply a novel use of volumetric capnography to assess hemodynamic stability in patients requiring extracorporeal membrane oxygenation (ECMO). We report our experience of pediatric patients requiring ECMO support who were monitored using volumetric capnography. We describe the use of VCO 2 and its association with successful decannulation. This is a prospective observational study of pediatric patients requiring ECMO support at The Children's Hospital at Montefiore from 2017 to 2019. A Respironics NM3 monitor was applied to each patient. Demographics, hemodynamic data, blood gases, and VCO 2 (mL/min) data were collected. Data were collected immediately prior to and after decannulation. Over the course of the study period, seven patients were included. Predecannulation VCO 2 was higher among patients who were successfully decannulated than nonsurvivors (109 [35, 230] vs. 12.4 [7.6, 17.2] mL/min), though not statistically significant. Four patients (57%) survived without further mechanical support; two (29%) died, and one (14%) was decannulated to Berlin. Predecannulation VCO 2 appears to correlate with hemodynamic stability following decannulation. This case series adds to the growing literature describing the use of volumetric capnography in critical care medicine, particularly pediatric patients requiring ECMO. Prospective studies are needed to further elucidate the use of volumetric capnography and optimal timing for ECMO decannulation.

Feeding Pathway for Children on High Flow Nasal Cannula Decreases Time to Enteral Nutrition.

High Flow Nasal Cannula (HFNC) is commonly used for children with respiratory failure, yet no standardized guidelines exist on how to initiate, escalate, and maintain enteral nutrition (EN) for these patients. EN in critically ill children is associated with decreased hospital length of stay, decreased ventilator days, and fewer acquired infections. We aimed to decrease the mean time to EN initiation by 50% after the start of HFNC in 6 months. Methods: This quality improvement project used the Model for Improvement to inform interventions. A multidisciplinary team created an EN pathway for critically ill patients on HFNC. We conducted Plan-Do-Study-Act cycles related to implementing a standardized pathway for EN on HFNC. The primary outcome was time to EN initiation once on HFNC. Secondary outcomes were time to goal caloric EN, duration of HFNC, and adverse events. Outcomes were plotted on statistical process control charts and analyzed for special cause variation between baseline and intervention periods. Results: We included 112 patients in the study. Special cause variation occurred for both primary and secondary outcomes. The mean time to EN initiation decreased from 24.6 hours to 11.7 hours (47.5%). Mean time to goal feeds decreased from 25.8 hours to 15.1 hours (58.5%). Mean HFNC duration did not show any special cause variation. There were no episodes of aspiration. Conclusion: Implementation of a standardized pathway for EN on patients receiving HFNC resulted in decreased time to initiation of EN and time to goal caloric EN with no significant increase in adverse events.

Pediatric Shock: An Uncommon and Underrecognized Etiology.

Kawasaki disease, also known as mucocutaneous lymph node syndrome, is a well-known disease entity. Kawasaki shock syndrome (KSS), on the other hand, is less well recognized and has been reported in small single-center international studies and case reports. We report a case in the United States of an 11-year-old male with multiorgan failure and shock, presumed to be secondary to toxic shock but later diagnosed with KSS, an underrecognized entity in the US and review the literature. KSS should be considered in a critically ill child with unexplained shock.

Extracorporeal and advanced therapies for progressive refractory near-fatal acute severe asthma in children.

Asthma is the most common chronic illness and is one of the most common medical emergencies in children. Progressive refractory near-fatal asthma requiring intubation and mechanical ventilation can lead to death. Extracorporeal membrane oxygenation (ECMO) can provide adequate gas exchange during acute respiratory failure although data on outcomes in children requiring ECMO support for status asthmaticus is sparse with one study reporting survival rates of nearly 85% with asthma being one of the best outcome subsets for patients with refractory respiratory failure requiring ECMO support. We describe the current literature on the use of ECMO and other advanced extracorporeal therapies available for children with acute severe asthma. We also review other advanced invasive and noninvasive therapies in acute severe asthma both before and while on ECMO support.

E-cigarette or Vaping-Associated Acute Lung Injury and Hemophagocytic Lymphohistiocytosis.

In this report, we describe the case of a 17-year-old boy with progressive respiratory failure requiring extracorporeal support who met clinical criteria for a presumptive diagnosis of electronic cigarette or vaping-associated acute lung injury (EVALI), with clinical, pathologic, and laboratory evidence of hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS). The patient in our report had a history of tetrahydrocannabinol and nicotine electronic cigarette use for months leading up to his presentation of fever, headache, emesis, and weight loss with respiratory distress. Multiple potential diagnoses were explored, and the patient's respiratory status improved, and he was initially discharged from the hospital. Roughly one week later, the patient was readmitted for worsening respiratory distress. The patient then met sufficient criteria for a potential diagnosis of HLH and MAS (elevated ferritin level, inflammatory markers, and cytopenia) to warrant a bone marrow aspirate, which revealed rare hemophagocytic cells. Given the severity of his symptoms and laboratory evidence of HLH and MAS, the patient was started on a course of steroids and anakinra. Although laboratory markers improved after treatment, the patient's respiratory failure worsened, ultimately progressing to a need for mechanical ventilation and extracorporeal support and leading to worsening multiorgan system failure and, ultimately, death. To the best of our knowledge, this is the first report of a patient with a presumptive diagnosis of EVALI with evidence of HLH and MAS, raising the possibility that macrophage activation may play a role in the pathogenesis of EVALI.

A winter to remember! Extracorporeal membrane oxygenation for life-threatening asthma in children: A case series and review of literature.

Progressive refractory near-fatal asthma requiring intubation and mechanical ventilation can lead to death. Data on outcomes in children requiring extracorporeal membrane oxygenation (ECMO) support for status asthmaticus is sparse. We describe our experience of three patients in the winter of 2018 to 2019 successfully rescued with ECMO. We also report our novel use of extubation while still being on ECMO support. Awareness and use of ECMO in refractory asthma can help lower the mortality for this very common disease in children. We also review the current literature on the use of ECMO and other extracorporeal therapies in asthma.