Background and objectivesThe flexible bronchoscope has become widely used by pediatric pulmonologists as a diagnostic and therapeutic tool. Nevertheless, there are several gaps in our knowledge to help refine its use and reduce its complications. In this study, we aimed to evaluate the utility and complications of pediatric bronchoscopy.Design and settingWe conducted a retrospective review of bronchoscopy cases between March 2006 and April 2015 at a tertiary care medical center (King Fahad Medical City). One-hundred forty nine patients were studied.Patients and methodsWe evaluated how bronchoscopy contributed to the patients' diagnosis, assessed the accuracy of bronchoalveolar lavage white blood cell count (BAL WBC) to differentiate between infectious and non-infectious conditions, assessed the ability of clinical factors to predict high risk of desaturation during bronchoscopy, and finally summarized the reported procedural complications.ResultsWe found pediatric bronchoscopy was a crucial diagnostic (confirming, ruling out, and discovering unexpected diagnosis) and therapeutic tool. The accuracy of BAL WBC counts is poor (AUC (95% CI) = 0.609 (0.497–0.712)); however, using two cutoff values (≤10 WBCs (sensitivity = 84.44% and specificity = 29.27%) to rule out, and ≥400 WBCs (sensitivity = 33.33% and specificity 81.49%) to rule in infection) helped in early differentiation between infectious and non-infectious conditions. From the factors that we test, none we found predictive of desaturation. The most common procedural complication was desaturation (pooled incidence (95% CI) = 13 (8–19)%) followed by cough, mild airway bleeding, and spasm.ConclusionsFlexible bronchoscopy is an important and relatively safe diagnostic and therapeutic tool in pediatric medicine, and utilization of this service should be encouraged after a careful consideration of which patient needs this procedure and a rigorous estimate of its pros and cons.

Bronchiolitis is the leading cause of admissions in children less than two years of age. It has been recognized as highly debated for many decades. Despite the abundance of literature and the well-recognized importance of palivizumab in the high risk groups, and despite the existence of numerous, high-quality, recent guidelines on bronchiolitis, the number of admissions continues to increase. Only supportive therapy and few therapeutic interventions are evidence based and proved to be effective. Since Respiratory Syncytial Virus (RSV) is the major cause of bronchiolitis, we will focus on this virus mostly in high risk groups like the premature babies and children with chronic lung disease and cardiac abnormalities. Further, the prevention of RSV with palivizumab in the high risk groups is effective and well known since 1998; we will discuss the updated criteria for allocating infants to this treatment, as this medication is expensive and should be utilized in the best condition. Usually, diagnosis of bronchiolitis is not challenging, however there has been historically no universally accepted and validated scoring system to assess the severity of the condition. Severe RSV, especially in high risk children, is unique because it can cause serious respiratory sequelae. Currently there is no effective curative treatment for bronchiolitis. The utility of different therapeutic interventions is worth a discussion.

BackgroundMutations in the gene encoding filamin A (FLNA) lead to diseases with high phenotypic diversity including periventricular nodular heterotopia, skeletal dysplasia, otopalatodigital spectrum disorders, cardiovascular abnormalities, and coagulopathy. FLNA mutations were recently found to be associated with lung disease. In this study, we report a novel FLNA gene associated with significant lung disease and unique angiogenesis.Case presentationHere, we describe a 1-year-old Saudi female child with respiratory distress at birth. The child then had recurrent lower respiratory tract infections, bilateral lung emphysema with basal atelectasis, bronchospasm, pulmonary artery hypertension, and oxygen and mechanical ventilation dependency. Molecular testing showed a new pathogenic variant of one copy of c.3153dupC in exon 21 in the FLNA gene.ConclusionsOur data support previous reports in the literature that associate FLNA gene mutation and lung disease.

No abstract

Sleep-disordered breathing (SDB) includes disorders of breathing that affect airway patency, which impair children's sleep and lead to negative consequences. Obstructive sleep apnea, hypoventilation and upper airway resistance syndrome are common causes of morbidity and mortality in childhood. These clinical practice guidelines, intended for use by pediatricians and primary care clinicians, provide a clear recommendation for the diagnosis and management of sleep-disordered breathing, focusing on the most serious disorder, obstructive sleep apnea syndrome (OSAS). These clinical guidelines formulate clear recommendations to identify patients with suspected OSAS. Further, the manuscript will highlight the potential consequences of SBD in children, and how to overcome such difficulties, what could be the therapeutic options, a 12 recommendations and what are the future direction for pediatric sleep medicine.