Pulmonary Alveolar Proteinosis: A Case of Profound Hypoxemia in a Previously Healthy Teenager.

ABSTRACT: The profoundly hypoxemic child presents an interesting set of diagnostic and management challenges in the pediatric emergency department. While common pathologies including pneumonia, asthma, bronchiolitis, and pneumothoraces are managed using evidence-based algorithms, more enigmatic pathologies may present the treating physician with less diagnostic and therapeutic clarity. We present the case of a profoundly hypoxemic 16-year-old girl who presented in minimal distress, with oxyhemoglobin saturation of 63% on room air.

Pediatric pulmonology year in review 2021 and 2022: Physiology.

Pulmonary physiology is a core element of pediatric pulmonology care and research. This article reviews some of the notable publications in physiology that were published in Pediatric Pulmonology in 2021 and 2022.

The impact of obstructive sleep apnea in children with sickle cell disease and asthma.

INTRODUCTION: Asthma and obstructive sleep apnea (OSA) are chronic diseases that disproportionately affect children with sickle cell disease (SCD). The literature describes the negative impact that both conditions have on children with SCD separately; however, the effect of OSA on asthmatic children with OSA is less specific. We hypothesized that the presence of OSA in children with SCD and asthma is associated with specific hematologic markers, worse clinical outcomes, and greater healthcare utilization. METHODS: We retrospectively evaluated children with both SCD and asthma who underwent polysomnography (PSG). We assessed their demographic information, PSG data, hematologic indices, and healthcare utilization based on the concurrent presence of OSA. RESULTS: Fifty-nine percent of the cohort had OSA with a lower oxygen saturation (SpO2 ) nadir (87% vs. 93%, p < 0.001) and a lower median daytime SpO2 (96.5% vs. 98.5%, p < 0.05); those with OSA were more likely to have the hemoglobin SS genotype (86% vs. 46.5%, p = 0.03). Additionally, those with OSA had a higher mean corpuscular volume (87 vs. 77.2 fL, p = 0.03) and reticulocyte count (10.1% vs. 5.5%, p < 0.01). There was no difference in asthma severity or healthcare utilization between those with OSA and those without OSA. DISCUSSION: Overall, children with SCD and asthma might be at increased risk for developing OSA, and screening for sleep-disordered breathing should be incorporated as part of their routine care.

Eosinophilic plastic bronchitis: Case series and review of the literature.

Plastic bronchitis is a term used to describe group of life-threatening disorders characterized by the presence of large obstructing casts in the airways. Eosinophilic plastic bronchitis is a subtype of plastic bronchitis that occurs mainly in children and has not been well-described in the literature. Patients may have a history of asthma or atopy, but many do not. They often present with cough and wheezing, and frequently have complete collapse of one lung seen on imaging. The severity of presentation varies depending on the location of the casts, ranging from mild symptoms to severe airway obstruction and death. Bronchoscopy is often required to both diagnose and treat this condition. A variety of medical therapies have been used, although no formal studies have evaluated their efficacy. Symptoms may resolve after initial cast removal, but in some patients, cast formation recurs. Here, we report a case series of nine patients with eosinophilic plastic bronchitis and review the existing literature of this condition.

Clinical scope and healthcare utilization in childhood interstitial lung disease at a tertiary center.

Childhood interstitial lung disease (chILD) is a heterogeneous group of diffuse lung diseases that can be challenging to diagnose. With relative rarity of individual entities, data are limited on disease prevalence, care patterns, and healthcare utilization. The objective of this study was to evaluate chILD prevalence and review diagnostic and clinical care patterns at our center. A single-center, retrospective cohort study was conducted of patients receiving care at the Children's Hospital of Philadelphia (CHOP) between 1 January 2019 and 31 December 2021. Through query of selected ICD-10 billing codes relevant for chILD and medical chart review, a total of 306 patients were identified receiving pulmonary care during this period. Respiratory symptom onset was documented to have developed before 2 years of age for 40% of cases. The most common diagnostic categories included those with oncologic disease (21.2%), bronchiolitis obliterans (10.1%), and connective tissue disease (9.5%). Genetic testing was performed in 49% of cases, while 36% underwent lung biopsy. Hospitalization at CHOP had occurred for 80.4% of patients, with 45.1% ever hospitalized in an intensive care unit. One-third of children had required chronic supplemental oxygen. Seven (2.3%) patients died during this 3-year period. Collectively, these data demonstrate the scope of chILD and extent of health care utilization at a large volume tertiary care center. This approach to cohort identification and EMR-driven data collection in chILD provides new opportunities for cohort analysis and will inform the feasibility of future studies.

The Effect of the COVID-19 Pandemic on Pediatric Physician Wellness: A Cross-Sectional Study.

The COVID-19 pandemic has provided challenges to all healthcare workers. While the brunt of treating COVID-19 patients fell upon adult providers, pediatricians also experienced significant stressors and disruptions. Academic pediatricians and trainees (fellows and residents) were redeployed to manage adult patients in hospitalist and intensive care settings and/or had major changes to their clinical schedules. In this study, we aimed to describe levels of self-reported depression, anxiety, and burnout in pediatric physicians following the initial wave of the pandemic at the largest integrated health system in New York State. A cross-sectional study was conducted among pediatric physicians who cared for patients during the COVID-19 pandemic within the Northwell Health System as part of the Northwell Wellbeing Registry, a longitudinal registry assessing the psychological impact of COVID-19 on healthcare providers. A total of 99 pediatric physician respondents were included in this study; 72% of whom were attendings, 28% of whom were trainees. Compared to attendings, trainees reported significantly higher proportions of burnout-emotional exhaustion (p = 0.0007) and burnout-depersonalization (p = 0.0011) on the Abbreviated Maslach Burnout Inventory. There was not a similar trend in probable depression or probable anxiety using the Patient Health Questionnaire. In a multivariable logistic regression model, being a trainee was significantly associated with increased odds of burnout-emotional exhaustion (OR 5.94, 95% Confidence Interval: 1.85-19.02). These findings suggest that fellows and residents were a vulnerable population during the COVID-19 pandemic. Training programs should pay special attention to their trainees during times of crisis, and future studies can help to identify protective factors to reduce the risk of burnout during these times.

Clinical utility of repeated positive airway pressure titrations in children with obstructive sleep apnea syndrome.

STUDY OBJECTIVES: Positive airway pressure (PAP) is the second line of treatment for obstructive sleep apnea syndrome in children. It is common practice following initiation of PAP to perform repeat titration polysomnography to re-evaluate the patient's therapeutic pressure; however, data supporting this practice are lacking. We hypothesized that repeat PAP titration would result in significant setting changes in children with obstructive sleep apnea syndrome. METHODS: We retrospectively analyzed demographic, polysomnographic, and PAP data of children with obstructive sleep apnea syndrome aged 0-18 years who were initiated on PAP and underwent 2 titration studies over a 2-year period. PAP mode and recommended pressure differences between the 2 titrations were compared. RESULTS: 64 children met inclusion criteria. The median (interquartile range) baseline obstructive apnea-hypopnea index and SpO2 nadir were 14.8 (8.7-32.7) events/h and 88.5% (85-92%), respectively. The mean differences in obstructive apnea-hypopnea index, SpO2 nadir, and % total sleep time with SpO2 < 90% between both titrations were negligible, including children with obesity, adenotonsillar hypertrophy, and trisomy 21. Additionally, there was no significant difference in mean PAP pressure between 2 separate titration studies for those on continuous PAP or bilevel PAP. CONCLUSIONS: Overall, repeat PAP titration in children with obstructive sleep apnea syndrome within the timeframe here described did not result in significant changes in PAP mode, continuous PAP pressure, or obstructive apnea-hypopnea index. Based on these data, repeat PAP titration within 2 years of an initial titration does not appear to be necessary. CITATION: Yendur O, Feld L, Miranda-Schaeubinger M, et al. Clinical utility of repeated positive airway pressure titrations in children with obstructive sleep apnea syndrome. J Clin Sleep Med. 2022;18(4):1021-1026.

NT-proBNP levels and cardiopulmonary function in children with sickle cell disease.

Patients with sickle cell disease (SCD) are living longer and subsequently more apt to develop cardiopulmonary dysfunction. N-terminal pro-brain natriuretic peptide (NT-proBNP) levels have been used in adults with SCD to assess for pulmonary hypertension and mortality. While the incidence of PH is low in pediatrics, it is reasonable to presume that NT-proBNP levels can be used to assess risk for the development of cardiopulmonary morbidity. We hypothesized that NT-proBNP levels would be increased in patients with SCD compared to age-adjusted healthy children; additionally, these levels would be associated with labs indicative of hemolysis and would demonstrate evidence of obstructive lung disease and cardiac dysfunction. We retrospectively evaluated patients with SCD, 8-18 years old, at a large, tertiary care children's hospital. NT-proBNP levels were assessed in correlation with hemolytic lab work, spirometry, and echocardiographic data. The age group 8-14 years old, 75% of our cohort's population, had a median NT-proBNP of 70 pg/ml, greater than their age-adjusted counterparts (52 pg/ml). NT-proBNP levels were associated with an increased degree of hemolysis when compared with hemoglobin (Hb) (r = -0.43, p < .0001), reticulocyte count (r = .25, p = .01) and lactate dehydrogenase levels (r = .47, p < .0001). An inverse trend was found between NT-proBNP and spirometric data. Finally, a positive correlation was found between NT-proBNP and diastolic left ventricular size (r = .28, p = .047]. The correlations found suggest that NT-proBNP may be used prospectively to identify patients with SCD at increased risk for the development of cardiopulmonary dysfunction.

A Case Series of the 2019 Novel Coronavirus (SARS-CoV-2) in 3 Febrile Infants in New York.

We describe 3 febrile infants <2 months of age admitted to a large tertiary care children's hospital in New York and subsequently found to be infected with severe acute respiratory syndrome coronavirus 2. All 3 patients presented with fever, feeding difficulty, lymphopenia, and thrombocytosis on laboratory evaluation. Two of the 3 patients were found to have neutropenia, and 2 had known exposures to sick contacts. In this case series, we describe 3 of the youngest patients to be reported with severe acute respiratory syndrome coronavirus 2 in the United States.

Characteristics and risk factors associated with critical illness in pediatric COVID-19.

BACKGROUND: While much has been reported regarding the clinical course of COVID-19 in children, little is known regarding factors associated with organ dysfunction in pediatric COVID-19. We describe critical illness in pediatric patients with active COVID-19 and identify factors associated with PICU admission and organ dysfunction. This is a retrospective chart review of 77 pediatric patients age 1 day to 21 years admitted to two New York City pediatric hospitals within the Northwell Health system between February 1 and April 24, 2020 with PCR + SARS-CoV-2. Descriptive statistics were used to describe the hospital course and laboratory results and bivariate comparisons were performed on variables to determine differences. RESULTS: Forty-seven patients (61%) were admitted to the general pediatric floor and thirty (39%) to the PICU. The majority (97%, n = 75) survived to discharge, 1.3% (n = 1) remain admitted, and 1.3% (n = 1) died. Common indications for PICU admission included hypoxia (50%), hemodynamic instability (20%), diabetic ketoacidosis (6.7%), mediastinal mass (6.7%), apnea (6.7%), acute chest syndrome in sickle cell disease (6.7%), and cardiac dysfunction (6.7%). Of PICU patients, 46.7% experienced any significant organ dysfunction (pSOFA > = 2) during admission. Patients aged 12 years or greater were more likely to be admitted to a PICU compared to younger patients (p = 0.015). Presence of an underlying comorbidity was not associated with need for PICU admission (p = 0.227) or organ dysfunction (p = 0.87). Initial white blood cell count (WBC), platelet count, and ferritin were not associated with need for PICU admission. Initial C-reactive protein was associated with both need for PICU admission (p = 0.005) and presence of organ dysfunction (p = 0.001). Initial WBC and presenting thrombocytopenia were associated with organ dysfunction (p = 0.034 and p = 0.003, respectively). CONCLUSIONS: Age over 12 years and initial CRP were associated with need for PICU admission in COVID-19. Organ dysfunction was associated with elevated admission CRP, elevated WBC, and thrombocytopenia. These factors may be useful in determining risk for critical illness and organ dysfunction in pediatric COVID-19.

Early Experience of COVID-19 in a US Children's Hospital.

OBJECTIVES: We aim to describe the demographics, clinical presentation, hospital course, and severity of pediatric inpatients with coronavirus disease 2019 (COVID-19), with an emphasis on healthy, immunocompromised, and chronically ill children. METHODS: We conducted a single-center retrospective cohort study of hospitalized children aged younger than 22 years with COVID-19 infection at Steven and Alexandra Cohen Children's Medical Center at Northwell Health. Cases were identified from patients with fever and/or respiratory symptoms who underwent a nucleic acid amplification-based test for severe acute respiratory syndrome coronavirus 2. RESULTS: Sixty-five patients were identified. The median age was 10.3 years (interquartile range, 1.4 months to 16.3 years), with 48% of patients older than 12 years and 29% of patients younger than 60 days of age. Fever was present in 86% of patients, lower respiratory symptoms or signs in 60%, and gastrointestinal symptoms in 62%. Thirty-five percent of patients required ICU care. The white blood cell count was elevated in severe disease (P = .0027), as was the C-reactive protein level (P = .0192), compared with mild and moderate disease. Respiratory support was required in 34% of patients. Severity was lowest in infants younger than 60 days of age and highest in chronically ill children; 79% of immunocompromised children had mild disease. One death was reported. CONCLUSIONS: Among children who are hospitalized for COVID-19, most are younger than 60 days or older than 12 years of age. Children may have severe infection requiring intensive care support. The clinical course of immunocompromised patients was not more severe than that of other children. Elevated white blood cell count and C-reactive protein level are associated with greater illness severity.