Faculty Bibliography

BACKGROUND:People with cystic fibrosis (pwCF) may be at risk of complications from COVID-19 but the impact of COVID-19 on pwCF remains unknown. METHODS:We conducted a multicenter retrospective cohort study to assess the impact of the COVID-19 pandemic first wave on pwCF in the New York metropolitan area (NY) from March 1, 2020 to August 31, 2020. Objectives were to determine (1) the prevalence of COVID-19 by PCR and IgG antibody testing, (2) the clinical characteristics of COVID-19, (3) delay in routine outpatient care, and (4) the effect on anxiety and depression in pwCF. RESULTS:There were 26 COVID-19 cases diagnosed by PCR or antibody testing among the study cohort of 810 pwCF. The prevalence of COVID-19 by PCR (1.6%) and IgG antibody (12.2%) testing was low. 58% of cases were asymptomatic and 82% were managed at home. 8% were hospitalized and 1 person died. 89% of pwCF experienced delay in care. The prevalence of anxiety increased from 43% baseline to 58% during the pandemic (P<0.01). In post-hoc analysis, the proportion of patients with diabetes (38% versus 16%, P<0.01) and pancreatic insufficiency (96% versus 66%, P<0.01) were higher while CFTR modulator use was lower (46% versus 65%, P = 0.05) in pwCF who tested positive for COVID-19. CONCLUSIONS:The prevalence of COVID-19 among pwCF in NY during the pandemic first wave was low and most cases were managed at home. CFTR modulators may be protective. PwCF experienced delay in routine care and increased anxiety.

Background: The New York State (NYS) Cystic Fibrosis Newborn Screening Consortium (NYSCFNBS) has cooperated in advocating for continued monitoring of outcomes and improvement in CF newborn screening (NBS) through quality improvement (QI) since 2002. The10 CF Foundation-accredited CF centers have a close working relationship with the NYS Department of Health (DOH) Wadsworth Screening Lab. This cooperative approach has resulted in several interventions to improve the screening program in NYS. In 2002, NYS initiated an IRT, 39 CF mutation screening algorithm. On December 1, 2017, infants with 1 CF mutation identified began to undergo full CFTR gene sequencing; those infants with 2 CF mutations are referred to CF centers. Subsequently, the state replaced the 39 mutation panel with a 338 mutation panel, followed by the sequencing step when 1 CF mutation is detected. The new screening algorithm increases infants classified as CF-related metabolic syndrome (CRMS) in the United States, or CF screen positive inconclusive diagnosis (CFSPID) in Europe. CRMS is used to describe these infants with a sweat chloride value < 30 mmol/L and 2 CFTR mutations, or an intermediate sweat chloride value (30-59 mmol/L) and 1 or no CF-causing mutations. Between 10% and 20% of CRMS/CFSPID individuals can develop clinical features suggestive of CF. This project is an extension of a 2-year CFNBS QI project, which was developed due to the COVID-19 pandemic. NYS was the epicenter of the pandemicin the spring of2020. Due to statewide lockdown, all CF centers were closed for 2 months, and sweat testing for infants with an abnormal CFNBS was not available. Parents of CRMS/CFSPID infants were lost to follow-up because of anxiety about returning to the CF center during the pandemic. This QI project aims to educate the parents and primary care physicians (PCP) to increase awareness and monitor these infants over several years and standardizing care across the 10 NYSCFcare centers.
Method(s): Since the initiation of the sequencing algorithm in December 2017, 250 CRMS/CFSPID infants had been diagnosed. Aparental questionnaire was developed to assess their willingness to be contacted by the CF team to return for a CF clinic visit and repeat swe at test. Parentalagreement to permit the CF team to contact the PCP to educate them concerning CRMS/CFSPID was requested. The questionnaire and QI project were shared with the CF Foundation Clinical Research Community Engagement specialist to facilitate parental feedback from the CF community voice team. Monthly Zoom meetings were held with all 10 NYS CF teams to implement the QI effort.
Result(s): Each CF center is in the process of contacting CRMS/CFSPID patients and their pediatricians and assessing their previous evaluation, including genetic counseling, their knowledge of CRMS/ CFSPID, and willingness to follow up at the CF center again. This data is being collected and analyzed currently.
Conclusion(s): Despite CRMS/ CFSPID guidelines published in 2009 [1], there is controversy regarding management and follow-up of these infants, as well as on the education of busy PCP on this topic. The NYS NBS program offers a unique opportunity to assess infants with CRMS/CFSPID due to the full genetic sequencing available in these infants, and the NYSCFNBS QI data on the follow-up of these infants will help in the understanding and monitoring of this condition.
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Cough is a common pediatric condition. Acute cough is often considered to be self-limiting and not requiring clinical management. However, pediatric patients and their parents often seek remedies for an acute cough. Traditional Chinese Medicine (TCM) specialists have been treating pediatric cough for millennia. Here we present a case of pediatric cough and the approaches taken to it by a Western and Chinese respiratory specialist. We conclude that TCM may provide important and useful insights into the treatment of such pediatric respiratory disease.

The causes of kidney disease in pediatric patients are evenly divided between congenital abnormalities of the kidney and urinary tract and acquired disorders. Nearly 10% to 15% of adults in the United States have chronic kidney disease (CKD); there are no comparable data in children. Regardless of patient age, CKD is a systemic problem that affects every organ system, including the lung. We review the tests used to diagnose and evaluate kidney disease and the main clinical syndromes that are likely to be encountered to aid the pulmonology consultant who is asked to evaluate patients with kidney disease.

Endobronchial sarcoid lesions have previously been described and visualized upon bronchoscopy in adult patients with pulmonary sarcoid involvement. Endobronchial ultrasound-guided transbronchial fine-needle aspiration (EBUS-TBNA) has come into favor as the preferred method of diagnosis, but it remains a novel technique in pediatric pulmonology. We describe the first two known cases of visualized endobronchial sarcoid lesions in the pediatric population with pathological confirmation of sarcoidosis with endobronchial and EBUS-TBNA biopsies.

Tracheoesophageal fistula (TEF) with esophageal atresia (EA) is a common congenital anomaly that is associated with significant respiratory morbidity throughout life. The objective of this document is to provide a framework for the diagnosis and management of the respiratory complications that are associated with the condition. As there are no randomized controlled studies on the subject, a group of experts used a modification of the Rand Appropriateness Method to describe the various aspects of the condition in terms of their relative importance, and to rate the available diagnostic methods and therapeutic interventions on the basis of their appropriateness and necessity. Specific recommendations were formulated and reported as Level A, B, and C based on whether they were based on "strong", "moderate" or "weak" agreement. The tracheomalacia that exists in the site of the fistula was considered the main abnormality that predisposes to all other respiratory complications due to airway collapse and impaired clearance of secretions. Aspiration due to impaired airway protection reflexes is the main underlying contributing mechanism. Flexible bronchoscopy is the main diagnostic modality, aided by imaging modalities, especially CT scans of the chest. Noninvasive positive airway pressure support, surgical techniques such as tracheopexy and rarely tracheostomy are required for the management of severe tracheomalacia. Regular long-term follow-up by a multidisciplinary team was considered imperative. Specific templates outlining the elements of the clinical respiratory evaluation according to the patients' age were also developed.

Pediatric pulmonologists have been involved in the care of adult COVID-19 patients in a variety of ways, particularly in areas with a high concentration of cases. This invited commentary is a series of questions to Dr Mikhail Kazachkov, a pediatric pulmonologist at New York University, about his experiences to date in a major COVID-19 "hotspot" and his thoughts about how other pediatric pulmonologists facing this situation can best support their colleagues.

The effects of childhood exposure to perfluoroalkyl substances (PFASs) on lung function remain mostly unknown. Previous research indicates that children living or going to school near the World Trade Center (WTC) disaster were exposed to high levels of PFASs, among other toxic chemicals. To explore the effects of PFAS exposure on lung function, we measured serum PFASs in a cohort of children from the WTC Health Registry and a matched control group. Perfluorooctanesulfonate had the highest median concentrations in both groups (WTCHR = 3.72 ng/mL, Comparison = 2.75 ng/mL), while the lowest median concentrations were seen for perfluoroundecanoic acid (WTCHR = 0.12 ng/mL, Comparison = 0.01 ng/mL). Lung function outcomes were measured by spirometry, plethysmography, and oscillometry. Asthma diagnosis and serum eosinophil count were also recorded. We examined the relationships of each PFAS with lung function parameters and eosinophil count using linear regressions. Odds ratios for asthma were obtained for each PFAS using logistic regression. The effect of total PFASs on these outcomes was also assessed. All regression models were adjusted for sex, race/ethnicity, age, body mass index (BMI) and tobacco smoke exposure. We found that serum PFASs were not statistically associated with the measured lung function parameters, asthma diagnosis, or eosinophil count in this cohort (p < 0.05). These findings highlight the need for more longitudinal studies to explore the long-term effects of childhood PFAS exposure on lung function past adolescence and early adulthood.

AIM/OBJECTIVE:Children with severe uncontrolled asthma (SUA) have a high burden of symptoms and increased frequency of asthma exacerbations. Reflux esophagitis and eosinophilic esophagitis are important co-morbid factors for SUA. Both are associated with the presence of eosinophils in esophageal mucosa. We hypothesized that esophageal eosinophils are frequently present and correlate with the presence of airway eosinophils in children with SUA. METHOD/METHODS:We performed a retrospective analysis of a prospective database of children who underwent "triple endoscopy" (sleep laryngoscopy, bronchoscopy with bronchoalveolar lavage [BAL] and endobronchial biopsy [EBB], and esophagogastroduodenoscopy with esophageal biopsy [EsB]) at our Aerodigestive Center for evaluation of SUA. Children with known cystic fibrosis, primary ciliary dyskinesia, and aspiration-related lung disease were excluded. RESULT/RESULTS:Twenty-four children (21 males) ages 2-16 years were studied. Elevated BAL eosinophils were found in 10 (42%) patients, endobronchial eosinophils in 16 (67%); 7 (29%) had endobronchial eosinophils without elevated BAL eosinophils. Esophageal eosinophils were found in 11 (46%) patients. There was a correlation between the amount of eosinophils in BAL and EBB (R = 0.43, P = 0.05) airway eosinophils, defined as elevated BAL and/or EBB eosinophils, correlated with esophageal eosinophils (R = 0.41, P = 0.047). CONCLUSION/CONCLUSIONS:We concluded that airway and esophageal eosinophils are frequently present in children with SUA.