Respiratory Outcomes for Ventilator-Dependent Children With Bronchopulmonary Dysplasia.

OBJECTIVES: To describe outpatient respiratory outcomes and center-level variability among children with severe bronchopulmonary dysplasia (BPD) who require tracheostomy and long-term mechanical ventilation. METHODS: Retrospective cohort of subjects with severe BPD, born between 2016 and 2021, who received tracheostomy and were discharged on home ventilator support from 12 tertiary care centers participating in the BPD Collaborative Outpatient Registry. Timing of key respiratory events including time to tracheostomy placement, initial hospital discharge, first outpatient clinic visit, liberation from the ventilator, and decannulation were assessed using Kaplan-Meier analysis. Differences between centers for the timing of events were assessed via log-rank tests. RESULTS: There were 155 patients who met inclusion criteria. Median age at the time of the study was 32 months. The median age of tracheostomy placement was 5 months (48 weeks' postmenstrual age). The median ages of hospital discharge and first respiratory clinic visit were 10 months and 11 months of age, respectively. During the study period, 64% of the subjects were liberated from the ventilator at a median age of 27 months and 32% were decannulated at a median age of 49 months. The median ages for all key events differed significantly by center (P ≤ .001 for all events). CONCLUSIONS: There is wide variability in the outpatient respiratory outcomes of ventilator-dependent infants and children with severe BPD. Further studies are needed to identify the factors that contribute to variability in practice among the different BPD outpatient centers, which may include inpatient practices.

Daycare Attendance is Linked to Increased Risk of Respiratory Morbidities in Children Born Preterm with Bronchopulmonary Dysplasia.

OBJECTIVES: To test the hypothesis that daycare attendance among children with bronchopulmonary dysplasia (BPD) is associated with increased chronic respiratory symptoms and/or greater health care use for respiratory illnesses during the first 3 years of life. STUDY DESIGN: Daycare attendance and clinical outcomes were obtained via standardized instruments for 341 subjects recruited from 9 BPD specialty clinics in the US. All subjects were former infants born preterm (<34 weeks) with BPD (71% severe) requiring outpatient follow-up between 0 and 3 years of age. Mixed logistic regression models were used to test for associations. RESULTS: Children with BPD attending daycare were more likely to have emergency department visits and systemic steroid usage. Children in daycare up to 3 years of age also were more likely to report trouble breathing, having activity limitations, and using rescue medications when compared with children not in daycare. More severe manifestations were found in children attending daycare between 6 and 12 months of chronological age. CONCLUSIONS: In this study, children born preterm with BPD who attend daycare were more likely to visit the emergency department, use systemic steroids, and have chronic respiratory symptoms compared with children not in daycare, indicating that daycare may be a potential modifiable risk factor to minimize respiratory morbidities in children with BPD during the preschool years.

Factors associated with discontinuation of pulmonary vasodilator therapy in children with bronchopulmonary dysplasia-associated pulmonary hypertension.

OBJECTIVE: To evaluate factors associated with discontinuation of pulmonary vasodilator therapy in bronchopulmonary dysplasia-related pulmonary hypertension (BPD-PH). STUDY DESIGN: Retrospective study of neonatal, echocardiographic, and cardiac catheterization data in 121 infants with BPD-PH discharged on pulmonary vasodilator therapy from 2009-2020 and followed into childhood. RESULT: After median 4.4 years, medications were discontinued in 58%. Those in whom medications were discontinued had fewer days of invasive support, less severe BPD, lower incidence of PDA closure or cardiac catheterization, and higher incidence of fundoplication or tracheostomy decannulation (p < 0.05). On multivariable analysis, likelihood of medication discontinuation was lower with longer period of invasive respiratory support [HR 0.95 (CI:0.91-0.99), p = 0.01] and worse RV dilation on pre-discharge echocardiogram [HR 0.13 (CI:0.03-0.70), p = 0.017]. In those with tracheostomy, likelihood of medication discontinuation was higher with decannulation [HR 10.78 (CI:1.98-58.59), p < 0.001]. CONCLUSION: In BPD-PH, childhood discontinuation of pulmonary vasodilator therapy is associated with markers of disease severity.

Insurance coverage and respiratory morbidities in bronchopulmonary dysplasia.

INTRODUCTION: Preterm infants and young children with bronchopulmonary dysplasia (BPD) are at increased risk for acute care utilization and chronic respiratory symptoms during early life. Identifying risk factors for respiratory morbidities in the outpatient setting could decrease the burden of care. We hypothesized that public insurance coverage was associated with higher acute care usage and respiratory symptoms in preterm infants and children with BPD after initial neonatal intensive care unit (NICU) discharge. METHODS: Subjects were recruited from BPD clinics at 10 tertiary care centers in the United States between 2018 and 2021. Demographics and clinical characteristics were obtained through chart review. Surveys for clinical outcomes were administered to caregivers. RESULTS: Of the 470 subjects included in this study, 249 (53.0%) received employer-based insurance coverage and 221 (47.0%) received Medicaid as sole coverage at least once between 0 and 3 years of age. The Medicaid group was twice as likely to have sick visits (adjusted odd ratio [OR]: 2.06; p = 0.009) and emergency department visits (aOR: 2.09; p = 0.028), and three times more likely to be admitted for respiratory reasons (aOR: 3.04; p = 0.001) than those in the employer-based group. Additionally, those in the Medicaid group were more likely to have nighttime respiratory symptoms (aOR: 2.62; p = 0.004). CONCLUSIONS: Children with BPD who received Medicaid coverage were more likely to utilize acute care and have nighttime respiratory symptoms during the first 3 years of life. More comprehensive studies are needed to determine whether the use of Medicaid represents a barrier to accessing care, lower socioeconomic status, and/or a proxy for detrimental environmental exposures.

Characteristics of infants or children presenting to outpatient bronchopulmonary dysplasia clinics in the United States.

INTRODUCTION: Bronchopulmonary dysplasia (BPD) is a common respiratory sequelae of preterm birth, for which longitudinal outpatient data are limited. Our objective was to describe a geographically diverse outpatient cohort of former preterm infants followed in BPD-disease specific clinics. METHODS: Seven BPD specialty clinics contributed data using standardized instruments to this retrospective cohort study. Inclusion criteria included preterm birth (<37 weeks) and respiratory symptoms or needs requiring outpatient follow-up. RESULTS: A total of 413 preterm infants and children were recruited (mean age: 2.4 ± 2.7 years) with a mean gestational age of 27.0 ± 2.8 weeks and a mean birthweight of 951 ± 429 grams of whom 63.7% had severe BPD. Total, 51.1% of subjects were nonwhite. Severe BPD was not associated with greater utilization of acute care/therapies compared to non-severe counterparts. Of children with severe BPD, differences in percentage of those on any home respiratory support (p = .001), home positive pressure ventilation (p = .003), diuretics (p < .001), inhaled corticosteroids (p < .001), and pulmonary vasodilators (p < .001) were found between centers, however no differences in acute care use were observed. DISCUSSION: This examination of a multicenter collaborative registry of children born prematurely with respiratory disease demonstrates a diversity of management strategies among geographically distinct tertiary care BPD centers in the United States. This study reveals that the majority of children followed in these clinics were nonwhite and that neither variation in management nor severity of BPD at 36 weeks influenced outpatient acute care utilization. These findings suggest that post-neonatal intensive care unit factors and follow-up may modify respiratory outcomes in BPD, possibly independently of severity.

Pulmonary Manifestations of Congenital Heart Disease in Children.

This review addresses how anomalous cardiovascular anatomy imparts consequences to the airway, respiratory system mechanics, pulmonary vascular system, and lymphatic system. Abnormal formation or enlargement of great vessels can compress airways and cause large and small airway obstructions. Alterations in pulmonary blood flow associated with congenital heart disease (CHD) can cause abnormalities in pulmonary mechanics and limitation of exercise. CHD can lead to pulmonary arterial hypertension. Lymphatic abnormalities associated with CHD can cause pulmonary edema, chylothorax, or plastic bronchitis. Understanding how the cardiovascular system has an impact on pulmonary growth and function can help determine options and timing of intervention.

Transitioning from an ICU ventilator to a portable home ventilator.

There is a variety of portable ventilators on the market, each with its' own features. A clinician needs to understand the unique characteristics of the ventilators available in his or her region, as well as the nuances of primary and secondary settings for these portable home ventilators in order to create a comfortable breath that allows for adequate gas exchange for the patient. Understanding the interplay of the portable home ventilator and the ventilator circuit is also a key component of transitioning a patient to a portable home ventilator. This review details characteristics of some of the more commonly used machines in the United States, as well as the settings to be considered in supporting a child with chronic respiratory failure outside of the hospital. As more patients are being discharged from the hospital with mechanical home ventilation, new ventilators are being developed that expand upon features of current machines.

Prematurity as an Independent Risk Factor for the Development of Pulmonary Disease.

OBJECTIVES: To determine if premature infants without bronchopulmonary dysplasia (BPD) are at similar risk for developing pulmonary morbidity as compared with those with BPD and if there are differences in management of care. STUDY DESIGN: We retrospectively abstracted information from our electronic medical record from January 1, 2006, to December 31, 2015, for primary care patients born at <30 weeks of gestation (n = 811). Multivariate models determined the impact of BPD on a diagnosis of respiratory disease, respiratory medications, subspecialty visits, and emergency department use or hospitalizations after adjusting for gestational age, sex, insurance type, and race. RESULTS: Infants with BPD were more likely to be diagnosed with asthma than those without BPD (75% vs 60%; OR, 1.8; 95% CI, 1.27-2.54), but not all respiratory conditions (OR, 1.56; 95% CI, 0.7-3.51), and were more likely to be referred to a pulmonologist (relative risk, 5.98; 95% CI, 4.1-8.74). Infants with BPD were more likely to be hospitalized for respiratory conditions than those without BPD (50% vs 30%; relative risk, 2.44; 95% CI, 1.73-3.45). CONCLUSIONS: Although infants with BPD were more likely to have a diagnosis of asthma and be readmitted for respiratory conditions, 60% of infants without BPD were also diagnosed with asthma and 30% were readmitted. There were significant differences in the management of patients, including time to pulmonary referral and prescription rates for inhaled corticosteroids. Practitioners should consider all patients born prematurely at high risk for respiratory morbidity.

Barriers to the Use of PPE to Prevent Pertussis Exposures in a Pediatric Primary Care Network.

BACKGROUND: The resurgence of pertussis is contributing to mounting healthcare worker (HCW) exposures, especially within pediatric outpatient settings. Infection prevention and control (IPC) guidelines for ambulatory sites exist, but are not uniformly implemented. Our aim was to identify facilitators and barriers to the use of IPC practices to prevent pertussis transmission in ambulatory pediatric settings. METHODS: HCWs at 18 of 25 (72%) clinics in a pediatric ambulatory network completed a theory-based questionnaire about pertussis and personal protective equipment (PPE). The questionnaire contained randomly distributed versions of two vignettes to create an experimental design assessing barriers to PPE use. RESULTS: Three hundred forty-three of 467 (73%) HCWs completed the survey: 234 (68%) clinical and 109 (32%) nonclinical. Seventy-one percent of clinical HCWs reported wearing PPE when caring for a patient with suspected pertussis; only 27% used PPE for patients with any respiratory symptoms. Among clinical HCWs, reported PPE use when caring for patients with suspected pertussis was independently associated with attitudes toward PPE (odds ratio [OR] 5.4, 95% confidence interval [CI] 1.8, 16.6), knowledge and skills (OR 4.6, 95% CI 1.4, 15.2), and lack of perceived barriers (OR 3.2, 95% CI 1.3, 7.9). HCWs who did not perceive PPE use as a norm were less likely to report using PPE (OR 0.2, 95% CI 0.1, 0.6) or distributing masks to patients with cough (OR 0.02, 95% CI 0.00, 0.2). Reported PPE use was not influenced by perceived infection risk or mask accessibility. CONCLUSION: Our results identified barriers to PPE use and targets for education to prevent pertussis transmission in ambulatory pediatric settings.

Variability in the diagnosis and treatment of group a streptococcal pharyngitis by primary care pediatricians.

OBJECTIVE: To compare practice patterns regarding the diagnosis and management of streptococcal pharyngitis across pediatric primary care practices. DESIGN: Retrospective cohort study. SETTING: All encounters to 25 pediatric primary care practices sharing an electronic health record. METHODS: Streptococcal pharyngitis was defined by an International Classification of Diseases, Ninth Revision code for acute pharyngitis, positive laboratory test, antibiotic prescription, and absence of an alternative bacterial infection. Logistic regression models standardizing for patient-level characteristics were used to compare diagnosis, testing, and broad-spectrum antibiotic treatment for children with pharyngitis across practices. Fixed-effects models and likelihood ratio tests were conducted to analyze within-practice variation. RESULTS: Of 399,793 acute encounters in 1 calendar year, there were 52,658 diagnoses of acute pharyngitis, including 12,445 diagnoses of streptococcal pharyngitis. After excluding encounters by patients with chronic conditions and standardizing for age, sex, insurance type, and race, there was significant variability across and within practices in the diagnosis and testing for streptococcal pharyngitis. Excluding patients with antibiotic allergies or prior antibiotic use, off-guideline antibiotic prescribing for confirmed group A streptococcal pharyngitis ranged from 1% to 33% across practices (P < .001). At the clinician level, 13 of 25 sites demonstrated significant within-practice variability in off-guideline antibiotic prescribing (P ≤ .05). Only 18 of the 222 clinicians in the network accounted for half of all off-guideline antibiotic prescribing. CONCLUSIONS: Significant variability in the diagnosis and treatment of pharyngitis exists across and within pediatric practices, which cannot be explained by relevant clinical or demographic factors. Our data support clinician-targeted interventions to improve adherence to prescribing guidelines for this common condition.

Ocular manifestations of candidemia in children.

Among 254 patients with candidemia who had a dilated retinal examination, 8 patients (3.2%) were diagnosed with ocular disease resulting in retinal detachment in 2 patients and globe rupture in 1 patient. This study found that ocular candidiasis is an uncommon but serious sight-threatening complication in pediatric patients with candidemia.

Methionine metabolism in human pregnancy.

BACKGROUND: Hyperhomocysteinemia during pregnancy, which is a consequence of perturbations in methionine and/or folate metabolism, has been implicated in adverse outcomes such as neural tube defects, preeclampsia, spontaneous abortion, and premature delivery. The adaptive changes in methionine metabolism during pregnancy in humans have not been determined. OBJECTIVE: Our objective was to examine the kinetics of methionine and its rate of transsulfuration and transmethylation in healthy women with advancing gestation. DESIGN: The whole-body rate of appearance (Ra) of methionine and phenylalanine was measured in healthy pregnant women during the first (n = 10), second (n = 5), and third (n = 10) trimesters of pregnancy. These data were compared with those for nonpregnant women (n = 8). Tracers [1-(13)C]methionine, [C(2)H(3)]methionine, and [(2)H(5)]phenylalanine were administered as prime-constant rate infusions. The effect of enteral high-protein, mixed-nutrient load on tracer-determined variables was also examined. RESULTS: In pregnant women, the Ra of phenylalanine was significantly (P < 0.05) lower in the first trimester than in the second and third trimesters and was significantly lower than that in nonpregnant women. A linear positive correlation was evident between gestational age and phenylalanine Ra. The fractional rate and total rate of transsulfuration of methionine was significantly (P < 0.05) higher during the first trimester, whereas the rate of transmethylation was higher during the third trimester. Plasma concentrations of total cysteine and homocysteine were lower during pregnancy. CONCLUSIONS: Uncomplicated pregnancy in humans is associated with a higher rate of transsulfuration early in gestation and a higher rate of transmethylation of methionine in late gestation. These data may have implications for understanding the role of methionine and homocysteine in complications of pregnancy and for the nutritional care of pregnant women.