The effect of inspiratory muscle training in PCD and CF patients: A pilot study.

BACKGROUND: Effective work of breathing and bronchial hygiene requires synergy of inspiratory and expiratory muscles. Inspiratory muscle training (IMT) is a part of pulmonary rehabilitation in chronic obstructive pulmonary disease (COPD). There is some evidence of its efficacy in cystic fibrosis (CF) and, recently, in long COVID-19. We are not aware of studies on IMT in primary ciliary dyskinesia (PCD). Our aim was to assess the effect of IMT on respiratory muscle strength and pulmonary function in PCD and CF patients. METHODS: A single center pilot study. Spirometry, lung clearance index (LCI), maximal inspiratory pressure (MIP), and maximal expiratory pressure (MEP) were measured at baseline (visit 1), after a month of IMT with ®POWERbreathe (visit 2), and at follow-up (visit 3). RESULTS: The cohort included 27 patients (19 PCD, 8 CF); mean age 18.4 ± 9.8 years. After a month of IMT, there was a significant increase in MIP and MIP% (6.19-7.44, p = .015; and 81.85%-100.41%, p = .046, respectively), which was sustained at visit 3. Compliance ≥90% led to higher improvement in MIP. In sub-group analysis, improvement in MIP and MIP% remained significant for PCD patients (p = .026 and p = .049, respectively). No significant changes were found in spirometry, MEP or LCI. CONCLUSIONS: IMT was well-tolerated and led to improved inspiratory muscle strength in PCD patients. The clinical implication of improved MIP should be further investigated. Larger, long-term studies are needed to evaluate long-term effects of IMT on pulmonary function, respiratory muscle strength, pulmonary exacerbations, and quality of life.

Foreign body aspiration in Israeli children during the COVID-19 pandemic.

BACKGROUND: Since the outbreak of the coronavirus disease 2019 (COVID-19) pandemic, there has been a decline in pediatric emergency department visits. Our aim was to assess the pattern of pediatric foreign body aspiration (FBA) during the first year of the COVID-19 pandemic, in comparison to the prior years. METHODS: In this retrospective multicenter study, we compared the number of children who presented with FBA during the COVID-19 year (March 1, 2020 to February 28, 2021) to the annual average of the years 2016-2019. We also compared the lockdown periods to the postlockdown periods, and the percentage of missed FBA, proven FBA, and flexible bronchoscopy as the removal procedure. RESULTS: A total of 345 children with FBA from six centers were included, 276 in the pre-COVID-19 years (average 69 per year) and 69 in the COVID-19 year. There was no difference in the prevalence of FBA between the COVID-19 year and any of the prior 4 years. Examining the lockdown effect, the monthly incidence of FBA dropped from a pre-COVID-19 average of 5.75 cases to 5.1 cases during lockdown periods and increased to 6.3 cases in postlockdown periods. No difference in the percentage of missed FB or proven FB was observed. There was a significant rise in the usage of flexible bronchoscopy as the removal procedure (average of 15.4% vs. 30.4%, p = 0.001). CONCLUSION: There were fewer cases of pediatric FBA during lockdown periods, compared to post-lockdown periods, presumably related to better parental supervision, with no difference in the prevalence of FBA during the COVID-19 year.

Understanding the interplay between factors that influence bone mineral density in CF.

BACKGROUND AND OBJECTIVES: Multiple factors affect bone mineral density (BMD) in cystic fibrosis (CF). Our aim was to perform comprehensive analyses of parameters potentially contributing to BMD. METHODS: A prospective single-center study assessing BMD, and correlations with multiple parameters including pancreatic status, lung functions, 6-minute walk test (6MWT), clinical score (modified Shwachman-Kulczycki [SK] score), vitamin D, nutritional intake, hand grip strength (HGS), habitual physical activity (smart watches), and quality of life (SF-36 questionnaire). RESULTS: Forty CF patients, mean age 18.3 ± 8.1 years, forced expiratory volume in 1 second 74.7% ± 17.9% predicted. Fifteen (37.5%) and 11 (27.5%) had osteopenia and osteoporosis, respectively. BMD was similar in pancreatic sufficient (pancreatic sufficient [PS], n = 15) and insufficient (pancreatic insufficient [PI], n = 25); median hip z score -1.5 ((-2.7)-(+0.2)) vs -1.5 ((-3.5)-(+0.7)), P = .79; spine -0.8 ((-2.2)-(+2)) vs -1.2 ((-4.4)-(+1.5)), P = .39 in PS vs PI, respectively. BMD correlated with HGS (r = .72, P < .001 hip; r = .52, P = .001 spine) and fat-free mass index (r = .81, P < .001 hip; r = .63, P < .001 spine). BMD z score correlated weakly with SK score and moderately with SF-36 general health. Data from smart watches, nutrition questionnaires, and 6MWT did not correlate with BMD. In a multivariate model, age and SK score predicted spine z score BMD. CONCLUSIONS: A substantial number of CF patients have low BMD. Similar rates in PS and PI suggest that other factors, such as disease severity, may contribute to low BMD. SK and age, which can easily be obtained even with limited resources, were the best predictors of low BMD. Further larger multicenter studies are warranted to evaluate the contribution of multifactorial etiologies to low BMD in CF.

Comprehensive cardiopulmonary assessment in α mannosidosis.

INTRODUCTION: α Mannosidosis is an extremely rare, progressive, and complex lysosomal storage disease, characterized by mental retardation, hearing impairment, coarse facial features, skeletal abnormalities, and pulmonary involvement. While bone marrow transplantation has been the only therapeutic option to date, nowadays new treatment options are being explored, which may affect pulmonary and exercise capacity. AIM AND METHODS: To assess cardiopulmonary involvement in patients with α mannosidosis by pulmonary function tests, cardiopulmonary exercise testing, and low irradiation chest computed tomography (CT). RESULTS: Five patients aged 11 to 28 years were followed in our Respiratory-Metabolic Clinic. All five had pulmonary symptoms and received inhaled therapy. Three patients underwent bone marrow transplantation. Parenchymal lung disease was evident in 3/5 chest CT tests. Pulmonary function tests were abnormal in all patients and showed obstructive/restrictive impairment with air trapping. All five patients showed reduced peak oxygen uptake (median 23.1; range 20.4-32.2 mL/minute/kg, median %predicted 62; range %predicted 59-79). CONCLUSIONS: Pulmonary involvement is a known complication in this rare disease. Comprehensive cardiopulmonary evaluation is feasible among these patients and may help in assessing disease progression and response to new treatment modalities.

Eosinophil cell count in bronchoalveolar lavage fluid in early childhood wheezing: is it predictive of future asthma?

Objective: Increased eosinophil level in bronchoalveolar lavage fluid (BALF) characterizes asthma in school-age children and adults and has been suggested as a marker for disease severity and response to treatment. We aimed to investigate the occurrence and yield of BALF eosinophil cell count in preschool children with recurrent wheezing and its possible relation to future diagnosis of asthma. Methods: BALF was retrospectively studied in young wheezy children and its relation to asthma at age 6 years was evaluated. BALF from children aged 1-48 months (mean = 20.4) was analyzed in preschool wheezy children. Children with anatomical airway obstruction and other lower airway/lung diseases who underwent BALF served as controls. Assessment of asthma was accomplished at 6 years. Results: Eighty-two children were included. The mean age during bronchoscopy and BAL was 20.4 ± 14.4 months (range: 1-48 months). Twenty-six patients had recurrent preschool wheezing, 13 anatomical airway obstruction and 43 had other lower airways/lung diseases. Groups were comparable for age during bronchoscopy and gender. No difference was found between groups for any of the BALF cell types. Eosinophils were very low in all three groups [mean (interquartile range): 0 (0-0.4), 0 (0-0.8), and 0.4 (0-1), respectively, p = 0.25]. No difference in eosinophil levels during bronchoscopy was found between asthmatic children to non-asthmatic as defined at age 6 years. Conclusions: Wheezing in preschool children is not associated with increased BALF eosinophils; hence, at this age, the diagnostic yield of BALF for cell count analysis for diagnosing asthma is limited and is not routinely indicated.

Laryngeal ultrasound for the diagnosis of laryngomalacia in infants.

INTRODUCTION: Laryngomalacia is the most prevalent cause of congenital stridor. Flexible laryngobronchoscopy (FLB) is the gold standard for diagnosis. However, FLB requires venous access, deep sedation or general anesthesia, and is associated with patient's and parental discomfort and a considerable cost. Laryngeal ultrasound (LUS) has been shown to provide good evaluation of the normal anatomy and the dynamic motion of laryngeal structures. We investigated the yield of LUS in the diagnosis of laryngomalacia in infants with congenital stridor compared to FLB. METHODS: A prospective blind study of consecutive infants referred for FLB due to congenital stridor with unknown cause. The presence of arytenoids adduction on LUS during inspiration was used to diagnose laryngomalacia. LUS was followed by FLB under anesthesia. The physician performing the FLB was blinded to the LUS results. RESULTS: Twenty-four patients participated in the study (54% males). Median (IQR) age and weight were 3.5 (1-7.7) months and 5.1 (4-8.4) kg, respectively. LUS was well tolerated in all infants. Compared to FLB, LUS correctly diagnosed laryngomalacia in 11/14 (78.6%) infants. In 10 infants FLB ruled out laryngomalacia of whom LUS concurred in 9 infants and one was falsely diagnosed with laryngomalacia. LUS had a sensitivity of 78.5% (CI 52-95%), specificity of 90% (CI 49-94%), NPV of 75%, and PPV of 92%. ROC analysis demonstrated AUC of 0.84 (P < 0.01, 95%CI: 0.67-1.0). CONCLUSION: We suggest that LUS has a role in screening congenital stridor in otherwise healthy infants when laryngomalacia is highly probable.

Variability in anesthesiologists' approach to the preoperative management of asthmatic children.

OBJECTIVE: No consensus guidelines exist for the preoperative treatment of asthmatic children referred for elective surgery. We investigated the attitude of pediatric anesthesiologists to this issue. DESIGN: A questionnaire survey was conducted. SETTING: National survey. SUBJECTS: Certified Israeli pediatric anesthesiologists from all 24 general hospitals in Israel. MEASUREMENT: Twenty-one questions regarding the approach to preoperative management of asthmatic children including 6 case scenarios with a variety of clinical situations and treatments of asthmatic children. The results were compared with the attitude of pediatric pulmonologists recently published using a similar methodology. MAIN RESULTS: Forty-four pediatric anesthesiologists from all 24 general hospitals in Israel responded. Twenty-five percent of pediatric anesthesiologists answered that, in addition to pediatric anesthesiologists, the primary pediatrician should be consulted, and 70% believed that a pediatric pulmonologists should also be consulted. Overall, results showed a wide variability between responders especially for preschool children and unstable school-aged asthmatic children for both disciplines. The variability referred to the use of any treatment, bronchodilators, inhaled corticosteroids and their combination, addition of systemic corticosteroids, and the length of preoperative treatment. Compared with pediatric pulmonologists, a better within-discipline agreement was observed by the pediatric anesthesiologists for stable school-aged asthmatic children with a lower inclination to augment preoperative treatment (P< .001). No difference was observed for the preschool children with asthma and for the unstable school-aged asthmatic child. CONCLUSIONS: A wide variability exists in pediatric anesthesiologists' approach to the preoperative management of asthmatic children for most common case scenarios. This is probably explained by the heterogeneity of asthma, the type of surgery, the lack of guidelines, and the paucity of data. Similarities as well as differences exist between pediatric anesthesiologists and pulmonologists. Further studies and implementation of consensus guidelines are needed.

Adenoidectomy for Obstructive Sleep Apnea in Children.

STUDY OBJECTIVE: Adenotonsillectomy is the recommended treatment for children with obstructive sleep apnea (OSA). Since adenoidectomy alone may be associated with significantly lower morbidity, mortality, and cost, we aimed to investigate whether adenoidectomy alone is a reasonable and appropriate treatment for children with OSA. METHODS: Five-hundred fifteen consecutive children diagnosed with moderate-to-severe OSA (apnea-hypopnea index > 5) based on polysomnography and who underwent adenoidectomy or adenotonsillectomy were reevaluated after 17-73 months (mean 41) for residual or recurrent OSA using a validated questionnaire (Pediatric Sleep Questionnaire, PSQ). Failure of OSA resolution was defined as a positive mean PSQ score ≥ 0.33. Contribution of age, obesity, tonsil size, and OSA severity at baseline to adenoidectomy or adenotonsillectomy failure was examined. RESULTS: Positive PSQ score occurred in 15% of the entire sample and was not influenced by age or gender. No difference in failure rate was observed between adenoidectomy and adenotonsillectomy for children who were not obese with apnea-hypopnea index < 10 and had small tonsils (< 3). Children with apnea-hypopnea index ≥ 10 and/or tonsil size ≥ 3 showed a higher failure rate after adenoidectomy compared to adenotonsillectomy (20% versus 9.8%, p = 0.028). CONCLUSIONS: We suggest that subjective, long term outcomes of adenoidectomy are comparable to those of adenotonsillectomy in non-obese children under 7 years old with moderately OSA and small tonsils. Hence, adenoidectomy alone is a reasonable option in some children. Future prospective randomized studies are warranted to define children who may benefit from adenoidectomy alone and those children in whom adenoidectomy alone is unlikely to succeed.

Alveolar ventilation in children during flexible bronchoscopy.

BACKGROUND: Hypoxia and hypercarbia complicate flexible bronchoscopy (FB). Unlike oxygenation by pulse-oximetry, alveolar ventilation is not routinely monitored during FB. The aim of this study was to investigate ventilation in children undergoing FB by measuring carbon-dioxide (CO2 ) levels using the transcutaneous technique. METHODS: Children admitted for FB were recruited. In addition to routine monitoring, transcutaneous CO2 (TcCO2 ) levels were recorded. All were sedated using the same protocol. RESULTS: Ninety-five children were studied. There was no association between peak TcCO2 or rise in TcCO2 and age, weight percentile, bronchoscope size, or diagnosis. Median baseline TcCO2 was 36 mmHg (IQR 32,40), median peak TcCO2 was 51 mmHg (IQR 43,62) with median TcCO2 rise of 17 mmHg (IQR 6.5,23.7). A rise of 15 mmHg or higher was recorded in 55% (n = 52) patients. Children requiring total propofol dose over 3.5 mg/kg had a significantly higher TcCO2 peak of 57.6 mmHg (IQR 47.8,66.7) compared to 47.1 mmHg (IQR 40,57) (P = 0.004) and a higher rise in TcCO2 22.5 mmHg (IQR 17,33.9) compared to 13.6 mmHg (6,22) (P = 0.001). Results were not affected by intranasal midazolam and broncho-alveolar lavage. No complications were reported. Non clinically significant (i.e., not lower than 90%) brief drops in oxygen saturation were observed. CONCLUSIONS: A large proportion of children undergoing FB have significant alveolar hypoventilation indicated by a rise in TcCO2 . Monitoring ventilation with TcCO2 is feasible and should be added during FB particularly in cases that are expected to require large amounts of sedation and patients susceptible to complications from respiratory acidosis. Pediatr Pulmonol. 2016;51:1177-1182. © 2016 Wiley Periodicals, Inc.

Pulmonary functions in children with inflammatory bowel disease.

OBJECTIVE: To investigate fractional exhaled nitric-oxide (FeNO) levels in children with Crohn's disease (CD) and ulcerative colitis (UC) and their correlation to disease activity. MATERIALS AND METHODS: Children with CD and UC (aged 8-18 years) and age-matched healthy controls without respiratory symptoms were recruited. Disease activity was assessed using validated scores. All children performed spirometry and FeNO tests and the association between intestinal disease parameters and pulmonary functions was studied. RESULTS: Thirty-five children with CD, nine with UC, and 24 healthy controls were enrolled. The mean FeNO level was higher in children with CD compared with the controls. Increased FeNO levels (>23 parts per billion) were more common among CD and UC compared with healthy children (46, 33, and 0%, respectively, P<0.05). Nevertheless, FeNO levels did not correlate with disease activity. There were no significant differences between CD, UC patients, and healthy controls in any of the spirometric variables. CONCLUSION: FeNO level, a marker of airway inflammation, is elevated in children with inflammatory bowel diseases irrespective of their intestinal disease activity. Increased FeNO levels are not associated with respiratory symptoms, suggesting a latent pulmonary involvement in the systemic disease.

Pediatric pulmonologists approach to the pre-operative management of the asthmatic child.

OBJECTIVE: No consensus guidelines exist for the respiratory treatment of asthmatic children referred for elective surgery. The aim of this study was to evaluate the attitude of pediatric pulmonologists regarding the pre-operative management of these children. METHODS: A survey of pre-operative management of asthmatic children was conducted. All 48 certified pediatric pulmonologists in Israel completed a questionnaire that comprised 20 questions regarding their approach to pre-operative management including six case scenarios with a variety of clinical situations and treatments of children with asthma. RESULTS: Response rate was 100%. All believed that pre-operative treatment should be considered in all asthmatic children. Almost 50% suggested that a pediatric pulmonologist should be consulted in all pre-operative assessments. 50% recommended consultation only in individual cases. Overall, results showed a very wide variability between responders especially in pre-school and poorly controlled school children. The variability referred to the use of bronchodilators, inhaled corticosteroids and their combination during the pre-operative days, the addition of systemic CS and the length of pre-operative treatment. Almost all participants suggested either the initiation or augmentation of pre-operative treatment in high risk situations. CONCLUSIONS: This data demonstrate an important variability among pediatric pulmonologists in Israel regarding the practice of pre-operative treatment of infants and children with asthma especially for the less controlled and high risk children. This is most probably explained by the paucity of evidence-based data and the lack of established guidelines. Consensus guidelines for the pre-operative management of asthmatic children are needed.

Respiratory Involvement in Children with Inflammatory Bowel Disease.

Inflammatory bowel diseases (IBDs), including Crohn's disease and ulcerative colitis, are systemic diseases with a variety of extra-intestinal manifestations. Respiratory involvement, whether clinically symptomatic or latent, may be more common than previously thought. As opposed to adults, most of the cases in children involve Crohn's disease. The pathogeneses of the pulmonary manifestations are obscure. The inflammatory process is not restricted to the bowel as has been suggested by findings of high levels of fractional exhaled nitric oxide (FeNO) in the airways. Increased FeNO has been reported even during clinical remission, while increasing further during exacerbations. Pulmonary manifestations develop usually after the onset of the bowel disease; however, they may emerge after colectomy and cessation of therapy. Pulmonary lesions in children usually involve the lung parenchyma with granulomatous infiltrates. Other less common injuries involve the airways and the pleura. The most prevalent pathologies in adults are bronchiectasis, cryptogenic organizing pneumonia, and small airway disease. Pulmonary involvement in IBD is often latent or sub-clinical and may be detected solely by laboratory or imaging techniques. Abnormalities in pulmonary function tests (PFTs) are not consistent except for low-diffusion capacity for carbon monoxide, which was found in both children and adults. High-resolution computed tomography (CT) is a sensitive tool for detecting lung involvement and may reveal abnormalities even when PFTs are normal and the patient is asymptomatic. Medications used for IBD, especially nonsteroidal anti-inflammatory drugs, are also a risk factor for lung injury. Treatment of pulmonary involvement often includes systemic corticosteroids with subsequent prolonged treatment of inhaled steroids in appropriate selected cases. Other immune-modulators have been tried. Pediatricians, especially those treating children with IBD, pediatric gastroenterologists, and pediatric pulmonologists should have a high index of suspicion and be aware of tools to be used in the assessment and treatment of pulmonary complications in IBD.