Pediatric pulmonary embolism: Not as rare as we think

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Phenotypic variance in pediatric obstructive sleep apnea

It is crucial that clinicians understand what underpins the considerable phenotypic variance in pediatric obstructive sleep apnea syndrome (OSAS), if they are to implement individually tailored phenotype-based approaches to diagnosis and management. This review summarizes the current literature on how disease severity, comorbidities, genetic and environmental/lifestyle factors interact to determine the overall OSAS phenotype. The first part discusses the impact of these factors on OSAS-related morbidity in the context of otherwise healthy children, whilst the second half details children with complex conditions, particularly focusing on the anatomical and functional abnormalities predisposing to upper airway obstruction unique to each condition. One can then understand the need for a multidimensional assessment strategy for pediatric OSAS; one that incorporates the history, physical examination, sleep study results, and biomarkers to enable precise stratification, so vital for effective determination of the timing and the nature of the therapeutic interventions required. © 2021 Wiley Periodicals LL

Pancreatitis in a patient with cystic fibrosis taking ivacaftor

Pancreatitis is rare in pancreatic insufficient cystic fibrosis patients. While pancreatic insufficiency has been considered irreversible until now, in the current era of new therapies with modulators of the Cystic Fibrosis Transmembrane Regulator CFTR channel, there are reports of improvement of pancreatic exocrine function. We describe the case of an adolescent with cystic fibrosis who developed pancreatitis after the partial recovery of pancreatic function while taking ivacaftor. This case adds to the limited body of evidence that CFTR modulators lead to the improvement of pancreatic exocrine function in cystic fibrosis. © 2020 by the authors. Licensee MDPI, Basel, Switzerland

Nebulizer care and inhalation technique in children with cystic fibrosis

Nebulizers are used by the great majority of cystic fibrosis patients for delivery of cornerstone treatments. Inhalation technique and adequate disinfection and maintenance are important for optimizing medication delivery. In this study, inhalation technique and nebulizer disinfection/maintenance were assessed in cystic fibrosis patients by direct observation in clinic and completion of a scoring sheet. A total of 108 patients were recruited. The maximum inhalation technique score was attained by 30.5% and adequate inhalation technique score by 74.08% of patients. The inhalation technique score was best with the vibrating mesh nebulizer (p = 0.038), while patient age and number of nebulized medications did not affect ITS significantly (p > 0.05). Nebulizer disinfection/maintenance score was excellent in only 31.48%. Most families kept the nebulizer clean and used appropriate disinfection method, but only half of them replaced the nebulizer and nebulizer cup at the recommended time intervals. Nebulizer disinfection/maintenance score was positively affected by a number of nebulized medications and negatively by years of equipment use (p = 0.009 and p = 0.001, respectively). Even though inhalation technique and disinfection/maintenance practices were found to be adequate in a large proportion of cases, there is still a need for regular review and education. The type of nebulizer was associated with improved inhalation technique, but more data are required before making specific recommendations. © 2020 by the authors. Licensee MDPI, Basel, Switzerland

Bronchiolitis, bronchitis, asthma exacerbation, bronchopneumonia, pneumonia: A didactic stepwise approach

Background: The clinical presentation of lower respiratory tract illness in children frequently resembles more than one disease entities of those described in textbooks. Not uncommonly, terms like “bronchiolitis”, “bronchitis”, “asthma exacerbation”, “bronchopneumonia” and “pneumonia” are used interchangeably. Methods: In this article, a didactic simplified algorithm for the management of the child from the community presenting with cough and fever is described after systematic review of the literature. Results: In step 1 of the algorithm, findings from the physical examination are used to localize the diseased anatomic part(s) of the lower respiratory tract i.e. trachea, bronchi, bronchioles or alveoli. In step 2, speculations are made regarding the infectious agent most likely affecting the lower respiratory tract based on the patient’s symptoms and past medical history and the known epidemiology of respiratory disorders. In step 3, the appropriate treatment plan is selected taking under consideration the affected anatomic part(s) and the speculated pathogen. Finally, in step 4, clinical response is evaluated and the diagnostic assumptions and treatment strategy are modified accordingly. Conclusion: When findings from the physical examination are matched with information from the current and past medical history and the known epidemiology of respiratory disorders, speculations regarding the most likely etiologic agents can be made and appropriate treatment interventions can be selected. © 2017 Bentham Science Publishers

Triage for malnutrition risk among pediatric and adolescent outpatients with cystic fibrosis, using a disease-specific tool

Malnutrition prevails in considerable proportions of children with Cystic Fibrosis (CF), and is often associated with adverse outcomes. For this, routine screening for malnutrition is pivotal. In the present cross-sectional study, we aimed to assess the risk for malnutrition in pediatric outpatients with CF. A total of 76 outpatients (44 girls, 11.9 ± 3.9 years old, 39.5% adolescents) were recruited and anthropometric, clinical, dietary and respiratory measures were collected. All outpatients were screened for malnutrition risk with a validated disease-specific instrument. Most children exhibited a low risk for malnutrition (78.9%), whereas none of the participants were characterized as having a high malnutrition risk. In the total sample, malnutrition risk was positively associated with age (r = 0.369, p = 0.001), and inversely related to the body mass index (r = −0.684, p < 0.001), height z-score (r = −0.264, p = 0.021), and forced expiratory volume (FEV1%, r = −0.616, p < 0.001). Those classified as having a low malnutrition risk were younger (p = 0.004), heavier (p < 0.001) and taller (p = 0.009) than their counterparts with a moderate risk. On the other hand, patients in the moderate risk group were more likely pubertal (p = 0.034), with a reduced mid-upper arm fat area (p = 0.011), and worse pulmonary function (p < 0.001). Interestingly, none of the children attaining ideal body weight were classified as having a moderate malnutrition. risk, whereas 37.5% of the patients allocated at the moderate risk group exhibited physiological lung function. In this cohort of outpatients with CF that were predominantly well-nourished and attained physiological lung function, malnutrition risk was identified only in small proportions of the sample. Our data support that patients that are older, pubertal, or have diminished fat mass are at greater risk for malnutrition. © 2020 by the authors. Licensee MDPI, Basel, Switzerland

C-reactive protein as a potential biomarker of residual obstructive sleep apnea following adenotonsillectomy in children

Study Objectives: Adenotonsillectomy (AT) is first-line treatment for pediatric obstructive sleep apnea (OSA), with most children having improvements in polysomnography (PSG). However, many children have residual OSA following AT as determined through PSG. Identification of a biomarker of residual disease would be clinically meaningful to detect children at risk. We hypothesize serum high-sensitivity C-reactive protein (hsCRP), an inflammatory biomarker, is predictive of residual OSA following AT. Methods: PSG was performed both preoperatively and postoperatively on children undergoing AT for the diagnosis of OSA. HsCRP serum concentrations were determined in all children pre-AT, and in most children post-AT. Resolution of OSA after AT was defined by a post-AT apnea-hypopnea index (AHI) 5/h TST, which is considered clinically significant. Results: AT significantly improved the AHI from 15.9 ± 16.4 to 4.1 ± 5.3/h TST in 182 children (P 5) was seen in 46 children (25%). Among children who had hsCRP levels measured pre- And post-AT (n = 155), mean hsCRP levels pre-AT were 0.98 ± 1.91 mg/L and were significantly reduced post-AT (0.63 ± 2.24 mg/dL; P = 0.011). Stratification into post-AT AHI groups corresponding to 5/h TST revealed post-AT hsCRP levels of 0.09 ± 0.12, 0.57 ± 2.28, and 1.49 ± 3.34 mg/L with statistical significance emerging comparing residual AHI > 5/h TST compared to post-AT AHI < 1.5/h TST (P = 0.006). Hierarchical multivariate modeling confirmed that pre-AT AHI and post-AT hsCRP levels were most significantly associated with residual OSA. Conclusions: Even though AT improves OSA in most children, residual OSA is frequent. Assessment of post-AT hsCRP levels emerges as a potentially useful biomarker predicting residual OSA