Time for a gut check: Pancreatic sufficiency resulting from CFTR modulator use.

Pancreatic exocrine insufficiency in cystic fibrosis is genetically determined and generally felt to be irreversible. However, recent studies in young children started on cystic fibrosis transmembrane conductance regulator (CFTR) modulators have suggested improvement of pancreatic functioning over time. Here, we present the case of a 10-year-old child with pancreatic exocrine insufficiency since birth who regained pancreatic functioning after 4 years on the CFTR corrector drug, ivacaftor.

Asthma is associated with increased probability of needing CPAP in children with severe obstructive sleep apnea.

OBJECTIVE: Initiation of continuous positive airway pressure (CPAP) in children with severe obstructive sleep apnea syndrome (OSAS) is challenging and the distinct features of the subset of children requiring CPAP are poorly defined. Asthma often coexists with OSAS in children. The goal of this study was to explore the influence of asthma in the need for CPAP therapy in children with severe OSAS. HYPOTHESIS: Asthmatic children with severe OSAS have higher probability of needing CPAP than children with severe OSAS without asthma. METHODS: Cross-sectional study of clinical presentation, individual risk factors, and initial overnight polysomnogram (PSG) parameters in children with severe OSAS. Severe OSAS was defined as an obstructive apnea hypopnea index ≥10/h. The association between asthma and CPAP initiation was studied individually and adjusted by pertinent covariates. RESULTS: Four hundred eligible children (mean age 7 years, ±SD 5.3) with severe OSAS were enrolled and 133 individuals (33%) were identified to have asthma. The proportion of children needing CPAP was significantly higher in asthmatics with severe OSAS (29%) compared to those with OSAS alone (14%) (P < 0.01). Multivariate analysis demonstrated that the association between asthma and the need of CPAP in pediatric severe OSAS was independent of demographics, OSAS severity, obesity, and history of adenotonsillectomy (P < 0.01). CONCLUSION: Asthmatic children with severe OSAS have higher probability of needing CPAP independent of relevant covariables. This study further substantiates the link between OSAS and asthma in children and suggests the diagnosis of asthma may influence the need of CPAP therapy for severe OSAS.

Clinical definition of respiratory viral infections in young children and potential bronchiolitis misclassification.

Viral respiratory infections are often grouped as a single respiratory syndrome named 'viral bronchiolitis', independently of the viral etiology or individual risk factors. Clinical trials and guidelines have used a more stringent definition of viral bronchiolitis, including only the first episode of wheezing in children less than 12 months of age without concomitant respiratory comorbidities. There is increasing evidence suggesting that this definition is not being followed by pediatric care providers, but it is unclear to what extent viral respiratory infections are currently misclassified as viral bronchiolitis using standard definitions. We conducted a retrospective analysis of hospitalized young children (≤3 years) due to viral respiratory infections. Bronchiolitis was defined as the first wheezing episode less than 12 months of age. Demographic variables and comorbidities were obtained by electronic medical record review. The study comprised a total of 513 hospitalizations (n=453). Viral bronchiolitis was diagnosed in 144 admissions (28.1%). Notably, we identified that the majority of children diagnosed with bronchiolitis (63%) were misclassified as they had prior episodes of wheezing. Many children with bronchiolitis misclassification had significant comorbidities, including prematurity (51%), neuromuscular conditions (9.8%), and congenital heart disease (9.8%). Misclassification of bronchiolitis is a common problem that may lead to inappropriate management of viral respiratory infections in young children. A comprehensive approach that takes into consideration viral etiology and individual risk factors may lead to a more accurate clinical assessment of this condition and would potentially prevent bronchiolitis misclassification.

Phenotypical characterization of human rhinovirus infections in severely premature children.

BACKGROUND: Human Rhinovirus (HRV) has been identified as the most common cause of acute respiratory infections and hospitalizations in premature children. It is unclear if premature children are more susceptible to HRV due to their decreased pulmonary reserve or because they have enhanced lower airway reactivity to HRV. METHODS: We conducted a retrospective analysis of the clinical respiratory presentation of all PCR-confirmed HRV infections in full-term and premature children aged ≤3 years in our institution. Standardized respiratory distress scores were developed to examine lower airway obstruction (i.e., wheezing, hyperinflation, and sub-costal retractions) along with markers of decreased pulmonary reserve (hypoxemia and tachypnea) in young children with HRV infections. Demographic and clinical variables were obtained from reviewing electronic medical records (EMR). RESULTS: This study included a total of 205 children; 71% of these children were born full-term (>37 weeks gestation), 10% preterm (32-37 weeks) and 19% severely premature (<32 weeks). Our results demonstrated that: 1) HRV infections in the first 3 years of life were associated with higher overall respiratory distress scores in severely premature children relative to children born preterm or full-term; 2) HRV-infected severely premature children ≤3 years old were more likely to have lower airway obstruction than HRV-infected children born preterm or full-term; and 3) other clinical signs of respiratory distress such as tachypnea and hypoxemia were not more common in severely premature than in preterm and full-term children during an HRV infection. CONCLUSIONS: Our results indicate that HRV infections in severely premature children are associated with lower airway obstruction rather than hypoxemia or tachypnea. The latter suggests that enhanced airway reactivity is the underlying mechanism for the increased susceptibility to HRV in severely premature children. Longitudinal studies are needed to understand why premature babies develop airway hyper-reactivity to HRV and the long-term effects of early HRV infection in this population.

Age-Related Effect of Viral-Induced Wheezing in Severe Prematurity.

Premature children are prone to severe viral respiratory infections in early life, but the age at which susceptibility peaks and disappears for each pathogen is unclear. Methods: A retrospective analysis was performed of the age distribution and clinical features of acute viral respiratory infections in full-term and premature children, aged zero to seven years. Results: The study comprised of a total of 630 hospitalizations (n = 580 children). Sixty-seven percent of these hospitalizations occurred in children born full-term (>37 weeks), 12% in preterm (32-37 weeks) and 21% in severely premature children (<32 weeks). The most common viruses identified were rhinovirus (RV; 60%) and respiratory syncytial virus (RSV; 17%). Age-distribution analysis of each virus identified that severely premature children had a higher relative frequency of RV and RSV in their first three years, relative to preterm or full-term children. Additionally, the probability of RV- or RSV-induced wheezing was higher overall in severely premature children less than three years old. Conclusions: Our results indicate that the vulnerability to viral infections in children born severely premature is more specific for RV and RSV and persists during the first three years of age. Further studies are needed to elucidate the age-dependent molecular mechanisms that underlie why premature infants develop RV- and RSV-induced wheezing in early life.