Background: Diagnosis and treatment of obstructive sleep apnea (OSA) in infants and young children is challenging because of its clinical heterogeneity and lack of age-specific guidelines. Aim: We report the management and treatment outcome of OSA in children below two years of age. Treatment decisions were based upon the pattern of upper airway (UA) obstruction, clinical presentation and OSA severity. Methods: Retrospective, non-randomized observational cohort study at a tertiary center. Children with OSA who underwent an UA evaluation (drug-induced sleep endoscopy or direct laryngoscopy) were included. Results: The study population comprised 100 patients, 57 boys and 43 girls, with a median age of 0.72 years (range 0.0-2.0) and OSA confirmed by polysomnography. Multilevel UA collapse was present in 26%, (adeno)tonsillar hypertrophy in 31% and 21% had laryngomalacia. Laryngomalacia was more common in children below six months of age and adenotonsillar hypertrophy was observed mainly in children older than 1.5 year of age. Treatment improved OSA severity in the entire study population with a significant reduction in obstructive apnea/hypopnea index from 10.8/h (range 2.1-99.1) to 1.7/h (range 0.0-73.0) (p<0.001), an improvement in mean oxygen saturation from 96.9% (range 88.9-98.4) to 97.4% (range 92.3-99.0) (p<0.001) and in minimal oxygen saturation from 85.4% (range 37.0-96.0) to 88.8% (range 51.0-95.5) (p<0.001). Conclusion: Multidisciplinary management of young children with OSA guided by the pattern of UA obstruction and OSA severity, results in favorable treatment outcomes. The pattern of UA obstruction changes in the first two years of life from a dynamic collapse to structural abnormalities.

Misconceptions on Behavioral Cough Suppression Therapy for Pediatric Nonspecific Cough: A Response to Weinberger and Buettner’s Commentary on Fujiki et al.Laurie Slovarp, PhD, School of Speech, Language, Hearing, & Occupational Sciences, University of MontanaMarie Jette, PhD, Department of Otolaryngology – Head and Neck Surgery, University of Colorado Anschutz Medical CampusJane Reynolds, PhD, School of Speech, Language, Hearing, & Occupational Sciences, University of MontanaAmanda Gillespie, PhD, Department of Otolaryngology and Head and Neck Surgery, Emory UniversityJulie Barkmeier-Kraemer, PhD, Division of Otolaryngology – Head and Neck Surgery, University of UtahMary Sandage, PhD, Speech, Language, and Hearing Sciences, Auburn UniversityJaclyn Smith, PhD, Biology, Medical and Health Sciences, The University of ManchesterJemma Haines, BSc(Hons), Medical and Health Sciences, The University of Manchester, Manchester University NHS Foundation TrustAnne Vertigan, PhD, School of Medicine and Public Health, University of NewcastleStuart Mazzone, PhD, Department of Anatomy and Physiology, University of MelbourneCorresponding author: Laurie Slovarp, PhD,School of Speech, Language, Hearing, & Occupational Sciences, University of Montana, Missoula, MT, 59812. [email protected] (p) 406-243-2107 (f) 406-243-2362 Orcid ID: 0000-0002-3547-4092Key words : pediatric chronic cough, habit cough, suggestion therapy, speech-language pathologyFunding : noneConflicts of Interest: The authors declare no conflicts of interest.Author Contributions :Laurie Slovarp: conceptualization, writing original draft, review and editingMarie Jette: conceptualization, writing original draft, review and editingJane Reynolds: conceptualization, writing original draft, review and editingAmanda Gillespie: conceptualization, writing original draft, review and editingJulie Barkmeier-Kraemer: review and editingMary Sandage: review and editingJaclyn Smith: review and editingJemma Haines: review and editingAnne Vertigan: review and editingStuart Mazzone: writing, review, and editingAbstract/Summary : This commentary responds to Weinberger and Buettner’s critique of Fujiki et al.’s study on behavioral cough suppression therapy (BCST) for pediatric chronic cough. While acknowledging their contributions, it addresses inaccuracies and clarifies key aspects of BCST. The commentary highlights discrepancies in terminology, challenges assertions regarding diagnostic evaluations, and emphasizes the need for controlled trials to assess treatment efficacy.In their engagement with the study by Fujiki et al.1, Weinberger and Buettner2 bring attention to critical aspects of behavioral cough suppression therapy (BCST) and pediatric chronic cough treatment. While their contributions are valued, it is imperative to address certain inaccuracies and clarify key aspects of the study and BCST provided by specialized speech-language pathologists (SLPs).Firstly, Weinberger & Buettner inaccurately state that Fujiki et al. identified the children as having ”behavioral cough.” The term ”behavioral cough” in the paper was used only in the context of ”behavioral cough suppression therapy,” describing the treatment rather than the children’s cough diagnosis. The authors, in fact, used the term ”nonspecific cough/tic cough” in accordance with current CHEST and ERS guidelines3-5. Weinberger & Buettner’s assertion that ”historical evidence and current practice support “habit cough” as the appropriate diagnosis” lacks substantiation and contradicts contemporary CHEST guidelines5. We are particularly puzzled by Weinberger & Buettner’s advocacy for the term habit cough when Dr. Weinberger is listed as an author on a 2015 CHEST guidelines publication that explicitly advises against the use of these terms, deeming them ”out of date and inaccurate”5.Weinberger & Buettner also criticize the use of extensive evaluations for the children in the sample, arguing that “habit cough” can be diagnosed based on clinical presentation alone—specifically in the presence of a barking or honking cough and absence of the cough during sleep. Interestingly, the same CHEST guidelines, of which Weinberger is an author, specifically cautions against using the diagnostic terms of “psychogenic” or “habit cough” solely based on a barking or honking sound or the absence of cough during sleep, stating that these three clinical presentations lack specificity5. A barking or honking cough can occur with other diseases such as tracheomalacia or bronchiectasis6; and it is well documented that sleep inhibits the cough reflex in adults even when an organic disease process is identified7; 8. Without evidence to the contrary, it is should be assumed that sleep similarly suppresses cough in children. Further, the purpose of Fujiki et al.’s paper was to examine whether BCST improves cough in children and to describe cough characteristics and comorbidities, not to advocate for extensive assessments. Although, it is noteworthy that laryngoscopy revealed structural laryngeal pathology in several cases, challenging Weinberger & Buettner’s assertion that the described assessments were unjustified.Regarding the treatment described in Fujiki et al., Weinberger & Buettner characterize BCST as an “alternative to suggestion therapy.” This overlooks that BCST instructs in strategies to suppress cough, akin to his “suggestion therapy.” Therefore, it seems that BCST is not necessarily an alternative but rather a similar therapy with a different name. This overlap emphasizes the need for clarity in terminology. Importantly, in adults with chronic cough a loss of centrally (brain) mediated cough suppression has been demonstrated using functional brain imaging9.  Although empirical data in children is not available, it seems conceivable that similar mechanisms may be in operation, providing a neurobiological basis for why cough suppression training is often beneficial and supporting the use of terminology that emphasizes this aspect of the therapy.Additionally, Weinberger & Buettner’s argument that 45 pediatric pulmonologists ”already indicate they readily diagnose habit cough and successfully treat with suggestion therapy,” implies a stance against SLP treatment. However, considering there are thousands of pediatric pulmonologists in the U.S. alone, the sample size of 45 falls short of substantiating the claim that ”many” pulmonologists universally adopt this approach or that additional clinicians, such as SLPs, are unnecessary to assist these patients effectively. The argument lacks robust evidence and fails to account for the role of additional clinicians, such as SLPs, to address pediatric chronic cough effectively. Further, a substantial proportion of children in Fujiki et al.’s sample presented with phonotraumatic lesions (e.g., vocal nodules), which SLPs are specifically trained to address through behavioral treatment.Lastly, Weinberger & Buettner suggest that conducting a controlled trial for suggestion therapy is impractical due to the reported low frequency of referrals. We disagree that the size of the population is a valid argument for not conducting a randomized controlled trial. A control group is needed to robustly assess the efficacy of any treatment, particularly in the case of cough interventions which are particularly vulnerable to placebo effect10; 11. Weinberger & Buettner suggest that it would be unethical to withhold suggestion therapy for the sake of a randomized clinical trial; however, one could easily design a study that allowed for those randomized to a control treatment to be eligible for suggestion therapy upon completion of the control arm. Given Dr. Weinberger’s data suggests suggestion therapy eliminates nonspecific/tic cough in less than one week, such children would need to wait no more than one to two weeks before becoming eligible for suggestion therapy and it should be possible to demonstrate such a substantial effect in a relatively small number of subjects in a randomized controlled trial.We agree with Dr. Weinberger that SLP skills vary. As is the case with any clinical discipline, there are many subspecialties in the field of speech-language pathology and varying levels of skill and expertise within those subspecialties. BCST training is not standard in SLP training programs, and, therefore, requires special training.In conclusion, we think these clarifications underscore the importance of understanding Fujiki et al.’s paper in its correct context, and will advance a more nuanced and informed discussion on pediatric cough disorders and BCST’s role in treatment. We acknowledge Weinberger & Buettner’s contribution to pediatric cough management. The data reported in this letter and other publications12; 13, although anecdotal, suggests that behavioral treatment focused on cough suppression is effective. Given this, we encourage Weinberger and Buettner to complete a randomized controlled efficacy trial. If the data confirms efficacy, the study would significantly elevate the legitimacy of the treatment, increasing its use by other practitioners and benefitting pediatric patients with chronic cough.REFERENCES1. Fujiki RB, Wright ML, Fujiki AE, Thibeault SL. 2023. Factors influencing behavioral cough suppression therapy in children with nonspecific chronic cough. Pediatr Pulmonol. 58(12):3466-3477.2. Weinberger M, Buettner D. 2024. The habit cough syndrome. Pediatr Pulmonol. 59(2):260-262.3. Chang AB, Oppenheimer JJ, Irwin RS, Panel CEC. 2020. Managing chronic cough as a symptom in children and management algorithms: CHEST guideline and expert panel report. Chest. 158(1):303-329.4. Morice AH, Millqvist E, Bieksiene K, Birring SS, Dicpinigaitis P, Domingo Ribas C, Hilton Boon M, Kantar A, Lai K, McGarvey L et al. 2020. ERS guidelines on the diagnosis and treatment of chronic cough in adults and children. Eur Respir J. 55(1).5. Vertigan AE, Murad MH, Pringsheim T, Feinstein A, Chang AB, Newcombe PA, Rubin BK, McGarvey LP, Weir K, Altman KW et al. 2015. Somatic cough syndrome (previously referred to as psychogenic cough) and tic cough (previously referred to as habit cough) in adults and children: CHEST guideline and expert panel report. Chest. 148(1):24-31.6. Spinou A, Lee KK, Sinha A, Elston C, Loebinger MR, Wilson R, Chung KF, Yousaf N, Pavord ID, Matos S et al. 2017. The objective assessment of cough frequency in bronchiectasis. Lung. 195(5):575-585.7. Lee KK, Birring SS. 2010. Cough and sleep. Lung. 188 Suppl 1:S91-94.8. Power JT, Stewart IC, Connaughton JJ, Brash HM, Shapiro CM, Flenley DC, Douglas NJ. 1984. Nocturnal cough in patients with chronic bronchitis and emphysema. Am Rev Respir Dis. 130(6):999-1001.9. Ando A, Smallwood D, McMahon M, Irving L, Mazzone SB, Farrell MJ. 2016. Neural correlates of cough hypersensitivity in humans: Evidence for central sensitisation and dysfunctional inhibitory control. Thorax. 71(4):323-329.10. Eccles R. 2009. Central mechanisms iv: Conscious control of cough and the placebo effect. Handb Exp Pharmacol. (187):241-262.11. Eccles R. 2010. Importance of placebo effect in cough clinical trials. Lung. 188 Suppl 1:S53-61.12. Lokshin B, Lindgren S, Weinberger M, Koviach J. 1991. Outcome of habit cough in children treated with a brief session of suggestion therapy. Ann Allergy. 67(6):579-582.13. Weinberger M, Hoegger M. 2016. The cough without a cause: Habit cough syndrome. J Allergy Clin Immunol. 137(3):930-931.

Opportunities for Trauma-Informed Medical Care in Cystic FibrosisTo the Editor:People living with Cystic Fibrosis (PwCF) face a lifetime of potentially traumatic medical experiences. These experiences can range from invasive medical treatments (e.g. venipuncture, nasogastric tube placement) to daily illness-related events (e.g. illness related bullying). These experiences place PwCF at high risk for a type of posttraumatic stress called Medical Traumatic Stress (MTS)[1]. With the growing recognition of the high prevalence and impact of MTS, we anticipate that cystic fibrosis care teams may soon be tasked with integrating trauma-informed medical care into their clinical practices. CF care teams are well poised to prevent and screen for MTS because they 1) create the environment for many illness-related experiences; 2) have established workflows for mental health screening; and, in many cases, 3) have trusting relationships with PwCF and their families. Here, we seek to highlight the opportunities for implementing trauma-informed medical care within the cystic fibrosis-specific context.MTS is defined as the psychological and physiological responses of children and their families to pain, injury, serious illness, medical procedures, and invasive or frightening treatment experiences[2]. MTS is common in PwCF – 55% of these youth report experiencing potentially traumatic medical experiences and 30% report MTS symptoms[1]. MTS symptoms have the potential to impact medical care, health outcomes and quality of life[3].While highly effective modulator therapies may decrease the frequency and severity of medical care, PwCF have identified these therapies as an additional source of potentially traumatic experiences[4]. New potentially traumatic medical experiences in modulator-eligible PwCF can include lost sense of purpose as their identity shifts from a “sick” person to a “well” person; survivor guilt from benefiting from therapies not available to others; and financial distress as they face planning for a longer than anticipated life expectancy [4]. The challenge of navigating the healthcare system, such as advocating with care teams and insurance companies, has been identified as potentially traumatic by PwCF, particularly those who are modulator-ineligible[4].A trauma-informed approach to medical care may be helpful for cystic fibrosis care teams to mitigate MTS. The Pediatric Psychosocial Preventive Health Model (PPPHM) provides a framework for tiered implementation of trauma-informed medical care[5]. It includes recommendations for universal practices gauged towards all children and families, targeted approaches for those at high risk of developing MTS, and treatment for those with severe, escalating, or persistent MTS symptoms (Figure 1).Universal MTS interventions for PwCF include prevention and screening. Preventive interventions can include MTS awareness education for all PwCF, family members of PwCF and cystic fibrosis care team members. This education can highlight approaches to modifying potentially traumatic medical experiences and identifying MTS symptoms that indicate the need for further support. Universal systems interventions may also include the implementation of workflows to minimize the traumatic potential of healthcare experiences (e.g. workflows to minimize needle distress with comfort positioning, numbing cream, and distraction as part of standard care. Care teams can also integrate shared decision-making processes to optimize patient perception of control and implement systems that facilitate communication between cystic fibrosis care team and PwCF.While annual mental health screening for depression and anxiety is the current recommendation for all PwCF, healthcare providers should consider integrating routine MTS screening into their practice. Screening workflows can include screening for patient risk factors and MTS symptoms, as well as family member symptoms of MTS. Currently, there are no screening tools designed to specifically capture the cystic fibrosis experience of MTS; however, acute stress screeners such as the Acute Stress Checklist for Children can aid in the identification of MTS, thereby guiding providers on who to refer for further mental health assessments or support. One common concern regarding screening for MTS is managing the burden of time as well as supporting those PwCF who screen at-risk. Thus, in planning for MTS screening, teams ought to consider the time required for screening, appropriate training for those performing the screening, and the development of a clear workflow for management and referrals. While these barriers require a thoughtful approach to processes and care, the benefits outweigh the consequences of not screening. MTS is present for many regardless of if screening occurs and screening allows the medical team to optimize overall healthcare.The second tier of the PPPHM recommends targeted approaches to MTS for those with risk factors or early symptoms. Possible MTS risk factors in PwCF include parent posttraumatic stress disorder, high daily medication burden, and emergency room and intensive care [1]. Interventions for PwCF who have MTS risk factors or early symptoms may include increasing support during potentially traumatic medical experiences (e.g. having a child life expert prepare a patient before a procedure); altering the medical care plan when possible (e.g. minimizing daily medication burden); educating family members on what to say to PwCF before, during, and after potentially traumatic medical experiences; and integrating patient-specific resilience factors into individualized coping and support plans. In some cases, brief therapeutic psychological interventions may help address specific symptoms (e.g., targeted CBT needle phobia interventions, behavioral sleep interventions, brief psychoeducation interventions).The third tier of the PPPHM recommends treatment for MTS symptoms that impact medical care or impair daily functions. PwCF with significant MTS symptoms should be referred to a mental health provider with expertise in the treatment of trauma symptoms. Ideally, the provider should have familiarity with chronic medical conditions, and, ideally, cystic fibrosis specifically. While we do not yet have evidence-based MTS-specific interventions for PwCF, approaches may include cognitive behavioral techniques such as restructuring of hospital-related thoughts, behavioral activation, or trauma-focused cognitive behavioral therapy. Mental health providers could also collaborate with families and cystic fibrosis care teams to develop an individualized plan for minimizing re-traumatization and reducing the impact of MTS on medical care and quality of life.In summary, given the substantial impact that MTS can have on PwCF, cystic fibrosis care teams should consider integrating trauma-informed approaches into the medical care of PwCF to optimize overall health (including mental health). Comprehensive approaches to MTS mitigation in PwCF include 1) universal awareness, prevention, and screening, 2) targeted interventions for those at high risk, and 3) individualized MTS treatment administered by mental health professionals. To optimize cystic fibrosis care with attention to MTS, future research should prioritize more definitive identification of MTS risk factors, the development of validated cystic fibrosis-specific MTS screening tools, the creation and dissemination of evidence-based MTS prevention programs, and the development of evidence-based MTS mental health interventions tailored to PwCF.Figure 1: Application of the Pediatric Psychosocial Preventative Health Model to Cystic Fibrosis. Adapted with permission from the Center for Healthcare Delivery Science at Nemours Children’s Health System 2018-2019. All rights reserved[5].

FEV 1/FVC normally decreases through childhood, increases briefly during early adolescence, and then declines throughout life. The physiology behind this temporary increase during early adolescence is not well understood. The objective of this study was to determine if this pattern also occurs in children with asthma. Design: Single-center, cross-sectional, retrospective analysis of pulmonary function tests (PFTs) obtained over a 5-year period in children 5 to 18 years of age with persistent asthma. Results: 1,793 patients satisfied all inclusion and exclusion criteria. Mean age (± SD) was 10.4 ± 3.8 years. 48% were female. Mean FEV 1 /FVC was 0.83 ± .09. FEV 1 /FVC in children with persistent asthma declined from age 5 to age 11 by 5.7 % compared to 7.3% in healthy girls, and 5.8% compared to 9.4% in healthy boys. FEV 1/FVC increased by 1.2% until age 16 in children with asthma, compared to 2.2% in healthy girls, and 2.5% compared to 2.3% in healthy boys. The ratio was lower in obese children with asthma at all ages but demonstrated the same curvilinear shape as in healthy children. In absolute terms, FEV 1 grew proportionately more than FVC during early adolescence, so the ratio of FEV 1/FVC increased during that period. The curvilinear shape of the curve remained in postbronchodilator testing, though significantly blunted. Conclusions: The “Shepherd’s Hook” pattern in the FEV 1/FVC curve is preserved in children with persistent asthma. This was also true in obese patients with asthma, although their FEV 1/FVC ratios were lower throughout all stages of childhood and adolescence.

Real-time ultrasound guided segmental bronchoscopic insufflation in a Tay-Sachs patient with atelectasisMatej Šapina, MD, PhD1,2,3*, Bojana Olujić, MD1,2, Tihana Nađ, MD1,2, Hrvoje Vinković, MD1,2, Zdravka Krivdić Dupan, MD1,2, Nikolina Hamidović, MD1, Mia Damašek, MD1,2, Krešimir Milas, MD1, Željko Zubčić, MD, PhD1,2, and Ivan Erić, MD, PhD1,21University hospital center Osijek, 31000 Osijek, Croatia2Medical faculty Osijek, 31000 Osijek, Croatia3Faculty of dental medicine and health Osijek, 31000 Osijek, Croatia*Corresponding author: [email protected]: +385 31 512 279Keywords: bronchoscopy, atelectasis, Tay-Sachs diseaseIntroductionTo the Editor,Atelectasis is the collapse of lung tissue and is a common problem in patients suffering from severe neurological diseases. Treatment depends on the underlying cause, and ranges from respiratory physiotherapy, mucus clearance methods, and in some cases bronchoscopic interventions. If left untreated recurrent infections may occur, along with impaired gas exchange and in the long run fibrosis of the affected area1.A female patient at the age of 3 years with Tay-Sachs disease and consequent severe global psychomotor delay and epilepsy was initially admitted due to a respiratory infection to the neuropediatric department, where she developed an epileptic status and desaturation, prompting a transfer to the Intensive Care Unit. The refractory epileptic status resolved only with several antiepileptics, which lead to intubation requiring invasive mechanical ventilation, after which the patient stabilized.An attempt to reduce sedation the next day resulted in repeated seizures. On the third day, inflammatory parameters increased, with a radiological finding suggestive of pneumonia and a large left atelectasis confirmed by ultrasound. Antibiotics were empirically started, and Moraxella catarrhalis and Adenovirus in the respiratory tract were obtained in the cultures.Lung ultrasound showed a large consolidation in the dorsal upper two thirds of the left hemithorax. Due to the size of the atelectasis, we decided to perform bedside bronchoscopy.A single use paediatric flexible bronchoscope (Ambu® aScope™ 5 Broncho) was introduced through the ETT. The tracheobronchial morphology did not show any obstructions. We applied our recently published modified segmental insufflation technique, however without the use of surfactant2.First, a bronchoalveolar lavage was performed for microbiological and cytological analysis, followed by ultrasound guided bronchoscopic treatment of the affected area. The patient was positioned on the right lateral decubitus position. As described previously, a cuff pressure manometer (AG Cuffill Cuff) and a 50 ml syringe were attached to a three-way stopcock connected to the bronchoscope’s working channel. Pressure controlled segmental insufflation of the lingula and upper lobe was performed directly observing an aeration of the atelectatic areas on the ultrasound with no complications.Subsequent ultrasound monitoring resulted in total expansion of the affected lung and resolution of inflammation, confirmed radiologically (Figure 1.). The patient could be safely extubated and transferred to the wards. No reoccurrence of atelectasis was observed.ConclusionThe treatment of pediatric atelectasis poses a challenge, with limited approaches available and a lack of evidence-based guidelines to guide clinical decision-making. A question that remains to be answered is not only the mode of treatment, but timing. Even with bronchoscopic treatment, different methods exist. In obstructive atelectasis, the approach is simple - to eliminate the obstruction, however, nonobstructive atelectasis remain challenging. Bronchoscopic lung insufflation technique has been explored in adults, with limited cases reported in children3. Several case reports utilized pressure-controlled insufflation with or without surfactant in long standing atelectasis as an add on treatment2; 4.We believe that patients with severe neurological diseases require special attention regarding their susceptibility for developing atelectasis5. Our approach is early bronchoscopic pressure-controlled insufflation in non-obstructive atelectasis. Depending on the duration, we additionally instill surfactant in long standing ones.Ultrasound is recently being used in lung recruitment maneuvers to monitor lung expansion, however, it was only recently reported to be utilized for segmental insufflation monitoring in a spinal muscular atrophy type I patient4. However, to our best knowledge, our case report is the first successful use on a Tay-Sachs patient using real time ultrasound monitoring during pressure-controlled segmental bronchoscopic insufflation. The benefits of ultrasound guidance is the direct visualisation of lung expansion reducing the need for fluoroscopic radiation exposure of visualisation and further monitoring. Besides it increases the safety of the procedure allowing for detection of development of pneumothorax.In conclusion, pressure controlled real-time ultrasound guided bronchoscopic segmental insufflation, with its low-cost, lightweight, and flexible setup, seems to be a safe and promising new modality for treatment of non-obstructive atelectasis, especially in children requiring complex care.FIGURESFigure 1. On the left a large left sided atelectasis can be seen causing the mediastinal shift. On the right complete regression of the atelectasis is observed before extubatingReferences1. Ogake S, Bellinger C. 2020. Role of bronchoscopy in atelectasis. Clinical Pulmonary Medicine. 27(1):30-32.2. Šapina M, Olujic B, Nađ T, Vinkovic H, Dupan ZK, Bartulovic I, Milas K, Kos M, Divkovic D, Zubčić Z. 2023. Bronchoscopic treatment of pediatric atelectasis: A modified segmental insufflation‐surfactant instillation technique. Pediatric Pulmonology.3. Abu‐Hasan MN, Chesrown SE, Jantz MA. 2013. Successful use of bronchoscopic lung insufflation to treat left lung atelectasis. Pediatric Pulmonology. 48(3):306-309.4. Esteban IA, Osona B, Ballestín AS, Rubio CC, de Mendiola JMFP, Gómez VA, López ME, Vicente JCDC. 2024. Bronchoscopic segmental alveolar recruitment in a patient with spinal muscular atrophy and massive atelectasis. Pediatric pulmonology.5. St‐Laurent A, Zysman‐Colman Z, Zielinski D. 2022. Respiratory prehabilitation in pediatric anesthesia in children with muscular and neurologic disease. Pediatric Anesthesia. 32(2):228-236.

Title: Major aortopulmonary collateral artery aneurysms- a rare cause of airway narrowing

Late Development of Pneumatoceles in Necrotizing PneumoniaSila Y. Kocer 1, Nathan C. Hull MD2, D. Dean Potter, Jr. MD 3, Theresa Madigan MD 4, Jennifer M. Boland MD5 and Nadir Demirel MD 61Ondokuz Mayis University School of Medicine, Samsun, Turkey2Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] of Pediatric Surgery, Department of Surgery, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] of Pediatric Infectious Diseases, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] of Pediatric Pulmonology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] : Sila Y. Kocer, Ondokuz Mayis University School of Medicine, Körfez, 55270 Samsun, Turkey. Email address: [email protected]. Tel.: +905514571963Conflict of interest: The authors declare no conflict of interest.Author contributions : Sila Y. Kocer: Writing – original draft. Nathan C. Hull: Writing – review and editing.D. Dean Potter Jr.: Writing – review and editing.Theresa Madigan: Writing – review and editing.Jennifer M. Boland: Writing – review and editing.Nadir Demirel: Writing – review and editing.Keywords : Lung cysts; complicated pneumonia; lobectomy; imaging; childrenTo the editor,Community-acquired pneumonia (CAP) is one of the most common serious infections in children, although it usually has a good prognosis1. Necrotizing pneumonia (NP), a rare but severe complication of CAP, consists of the destruction of the consolidated lung parenchyma, potentially leading to the formation of thin-walled cavities known as pneumatoceles 2. While they often resolve spontaneously without sequalae, progressively enlarging pneumatoceles have been reported 5. We report a pediatric case with an unusual course of pneumatocele development.A 3-year-old female with history of congenital hypothyroidism and mild asthma presented to her primary care physician for evaluation of intermittent fever for the past 6 days, with a maximum temperature of 38.8°C. She had a worsening wet cough and complained of chest pain while coughing. Lung examination was normal except for mild tachypnea. Based on the patient’s presentation, empiric oral amoxicillin 90 mg/kg/day was started for possible CAP. The patient presented to the clinic on day 6 of her antibiotic treatment with persistent low-grade fever and ongoing cough. She had normal vital signs, and there were no signs of respiratory distress. The lung examination revealed crackles at right lung fields. A chest X-ray (CXR) showed a large consolidation and an airspace with an air-fluid level in the right upper lobe (RUL) (Figure 1A). There was no pleural effusion. The patient was diagnosed with complicated pneumonia and was referred to our hospital.A chest computed tomography (CT) without intravenous (IV) contrast showed a large consolidation in the RUL with scattered internal cystic areas containing air-fluid levels (Figure 1B, C). These findings raised concern for NP, abscess, or congenital pulmonary airway malformation with superimposed pneumonia. Laboratory test results showed mild anemia (hemoglobin concentration of 10 g/dl), leukocytosis (white blood cell count of 14.1 x 109/L), thrombocytosis (platelet count of 752 x 109/L), and high C-reactive protein (CRP) (80 mg/L, normal: <5 mg/L). She was admitted and started on IV ceftriaxone and IV vancomycin. She remained afebrile during admission and was clinically well appearing. A nasal swab culture for Methicillin-resistant Staphylococcus aureus was negative, therefore, on day 2 of admission, IV vancomycin was discontinued. AStreptococcus pneumoniae urine antigen test was positive. Serologic testing for endemic fungi was negative. A QuantiFERON-TB Gold was indeterminate due to inadequate mitogen response. On day 3 of admission, in preparation for discharge, IV ceftriaxone was switched to oral cefdinir 14 mg/kg/day to complete a 4-week course. She remained afebrile and well after an additional 24-hour period of observation and was subsequently discharged.Towards the conclusion of her antibiotic course, a follow-up CXR showed near resolution of the RUL consolidative opacity with a few small residual lucencies in the RUL, presumed to be residual pneumatoceles (Figure 1D). She was asymptomatic without a cough, and her lung examination was normal. Inflammatory markers, including CRP and sedimentation rate, as well as white blood cell count and platelet count, were in the normal range. One month later, while the patient remained asymptomatic, a follow-up CXR revealed an enlarged pneumatocele (Figure 2A, B). A chest CT with IV contrast demonstrated a 7.2 x 5.3 x 7.6 cm air-filled cavity in the RUL (Figure 2C, D). No definite connection to the adjacent airways was seen on the chest CT. The patient then underwent a thoracoscopic right upper lobectomy. The procedure was challenging due to adhesions and bleeding (Figure 2E). The pathology examination of the resected lung tissue showed a simple fibrous-walled cyst devoid of epithelial lining, consistent with pneumatocele (Figure 2F). Gram stain, fungal smear, bacterial culture and fungal culture of the explanted lung tissue were negative. The patient made a full recovery, both radiologically and clinically.Patients with NP usually present with symptoms of CAP, unresponsiveness to initial outpatient treatment, such as high fever, cough, tachypnea, and general unwell appearance 1. The initial treatment of NP consists of IV antibiotics covering the most common etiologic agents of NP, which are known to be S. pneumoniae , Group AStreptococci and S. aureus . The optimal duration of antimicrobial therapy is not clearly defined; however, usually prolonged with a median duration of 4 weeks reported in the literature2, which aligns with guideline suggestions for therapy of empyema and parapneumonic effusion 3. Improvement in clinical and laboratory parameters usually allows for IV to oral antibiotic transition, which was accomplished relatively early for our patient, due to her less severe initial presentation and rapid clinical improvement. Pneumatoceles, air-filled cysts that can arise as a complication of NP, typically regress over weeks to months when NP is treated, but might require segmental or lobar resection if they become tense (exceeding more than 50% of the involved lobe), infected, or rupture 1. In our patient, many of the small pneumatoceles decreased in size after antibiotic treatment, with subsequent delayed and marked enlargement of one of them. A report on giant lung cysts emerging after NP suggested that, when patients remain clinically stable, treatment of pneumatoceles should be conservative with antibiotics alone regardless of the size of the cysts, as interventional procedures carry a risk of complications such as bronchopleural fistula 4. However, a study that proposed a treatment algorithm for pneumatoceles, recommended surgical resection for those that remained unresolved despite a conservative approach and gradually grew in size and wall thickness5. Our patient had no symptoms related to the pneumatocele. Nevertheless, it can be challenging to anticipate the progression of pneumatoceles, as they can enlarge enough to compromise respiration. The unusual expansion within a span of 1 month in our case, led to the decision of surgical resection.To summarize, we present a 3-year-old otherwise healthy girl with NP. After 4 weeks of antibiotic therapy, the right lung consolidations resolved and pneumatoceles decreased in size. However, one month later, while she remained clinically asymptomatic, a follow-up CXR revealed the progressive enlargement of a pneumatocele which eventually required surgical resection. Based on this experience, we suggest a close radiological follow-up of patients with post-infectious pneumatoceles, regardless of symptoms, until complete radiologic resolution is demonstrated.References1. de Benedictis FM, Kerem E, Chang AB, Colin AA, Zar HJ, Bush A. Complicated pneumonia in children. Lancet. 2020;396(10253):786–798. doi:https://doi.org/10.1016/s0140-6736(20)31550-62. Masters IB, Isles AF, Grimwood K. Necrotizing pneumonia: an emerging problem in children? Pneumonia (Nathan). 2017;9(1). doi:https://doi.org/10.1186/s41479-017-0035-03. Bradley JS, Byington CL, Shah SS, Alverson B, Carter ER, Harrison C, Kaplan SL, Mace SE, McCracken GH, Moore MR, et al. The Management of Community-Acquired Pneumonia in Infants and Children Older Than 3 Months of Age: Clinical Practice Guidelines by the Pediatric Infectious Diseases Society and the Infectious Diseases Society of America. Clin Infect Dis. 2011;53(7):e25–e76. doi:https://doi.org/10.1093/cid/cir5314. Gross I, Gordon O, Cohen‐Cymberknoh M, Reiter J, Tsabari R, Gileles‐Hillel A, Erlichman I, Hevroni A, Shoseyov D, Kerem E. Giant lung cysts following necrotizing pneumonia: Resolution with conservative treatment. Pediatr Pulmonol. 2019;54(6):901–906. doi:https://doi.org/10.1002/ppul.243215. Imamoğlu M, Cay A, Koşucu P, Ozdemir O, Cobanoğlu U, Orhan F, Akyol A, Sarihan H. Pneumatoceles in postpneumonic empyema: an algorithmic approach. J Pediatr Surg. 2005;40(7):1111–1117. doi:https://doi.org/10.1016/j.jpedsurg.2005.03.048Authors and affiliations : Sila Y. Kocer 1, Nathan C. Hull MD 2, D. Dean Potter, Jr. MD3, Theresa Madigan MD 4, Jennifer M. Boland MD 5 and Nadir Demirel MD 61Ondokuz Mayis University School of Medicine, Samsun, Turkey2Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] of Pediatric Surgery, Department of Surgery, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] of Pediatric Infectious Diseases, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] of Pediatric Pulmonology, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota, USA. Email address: [email protected] of interest: The authors declare no conflict of interest.

Title: Determinants of school absences due to respiratory tract infections among children during COVID-19 pandemic: a cross-sectional study of the Sentinel Schools Network.Antoni Soriano-Arandes1,●,○, Andreu Colom-Cadena2,3,●, Anna Bordas2,3, Fabiana Ganem2,3,4, Lucia Alonso3,5, Marcos Montoro2,3, Mireia Gascon6,7,8, Maria Subirana6,7,8, Ariadna Mas9, Jordi Sunyer6,7,8, Pere Soler-Palacin1, Jordi Casabona2,3,4,8 ; on behalf of Sentinel School Network of Catalonia*Paediatric Infectious Diseases and Immunodeficiencies Unit. Children’s Hospital. Vall d’Hebron Barcelona Hospital Campus, Barcelona, Catalonia, Spain.Centre d’Estudis Epidemiològics sobre les ITS i Sida de Catalunya (CEEISCAT). Badalona, Spain.Institut d’Investigació Germans Trias i Pujol (IGTP), Badalona, Spain.Departament de Pediatria, d’Obstetrícia i Ginecologia i de Medicina Preventiva i de Salut Publica, Universitat Autònoma de Barcelona, Bellaterra, Spain.Fundació Lluita contra les infeccions, Badalona, Spain.ISGlobal, Barcelona. Spain.Universitat Pompeu Fabra (UPF), Barcelona, SpainCentro de Investigación Biomédica en Red de Epidemiología y Salud Pública (CIBERESP) Instituto de Salud Carlos III, Madrid, Spain.Direcció Assistencial d’Atenció Primària i Comunitària, Institut Català de la Salut, Barcelona, Catalonia, Spain● Contributed equally to this work with: Antoni Soriano-Arandes, Andreu Colom-Cadena○ Corresponding author: Antoni Soriano-Arandes,[email protected]. ORCID: 0000-0001-9613-7228Keywords: COVID-19, respiratory infections, ventilation, school, CO2, Indoor air qualityTo the editor:Different public health measures were implemented during the COVID-19 pandemic to maintain the schools open1,2. Regarding COVID-19 vaccination, at the beginning of this study, more than 92% and 40% of population older than 12 years and children aged 5-11 years in Spain was fully vaccinated against COVID-19, respectively3.Ventilation was proposed as one of the main strategies to reduce aerosol transmission for SARS-CoV-2 during the pandemic. A study from Germany assessed the efficiency of air purifiers in reducing aerosols in high-school classrooms4. However, no clinical endpoints were investigated and, therefore, a very low certainty of evidence was graded by a Cochrane review5. In an Italian study, the authors concluded that ventilation reduced the likelihood of SARS-CoV-2 infection in classrooms by 80%6. Moreover, a survey study from the US reported that elementary schools with face masks and ventilation strategies in place had lower SARS-CoV-2 incidence rates7. In a Lancet Task Force Commission review8, the authors concluded that improving building ventilation systems may carry benefits beyond protection from COVID-19. However, there is scarce evidence that demonstrate the association of epidemiological and environmental risk factors, including CO2 concentrations, with the incidence of respiratory tract infections (RTI) in children who are attending schools.The present study is part of COVID-19 Sentinel Schools Network of Catalonia (CSSNC), a project including 23 schools and 5687 students, teachers and other school staff with the aim to monitor SARS-CoV-2 and other respiratory viruses, their determinants and preventive measures in Catalonia9. The objectives of this study are to assess the potential association of demographic and epidemiological factors, including the indoor environmental conditions (CO2levels) in the classrooms, with students’ school absence due to RTI.We conducted a cross-sectional study in two different periods, the first from April 19 to June 21, 2022, and the second from November 8 to December 21, 2022. The main outcome of the study was the absence of the child from attending the school in person due to RTI or other non-respiratory medical causes. The study population was composed of 253 students (4-11 years) belonging to 20 classrooms, 11 in spring 2022 and 9 in autumn 2022, attending 4 and 16 classrooms of preschool (4 to 5 years-old) and primary school (6 to 11 years-old) stages, respectively. A written informed consent was previously obtained from parents or guardians of children.Absences from school attendance were notified by the tutor/teacher to the study researchers. We proceed to collect epidemiological and clinical/diagnostic data within the first 48 hours through a case report form deposited on the digital platform REDCap©. All the absences (cases) were followed-up through telephone calls made by health professionals until their return to the school. RTI was registered according to the symptoms described during the telephonic interview and confirmed through the computerised health record programme of the Health Ministry (eCAP).Sensors to monitor the CO2 concentrations (DIOXCARE DX700 PDF, Smartcare Services, Spain) were installed in classrooms, recording data every 10 minutes. For the analysis we only used the measurements taken during the time in which the students were in the classroom. We obtained a median [IQR] of CO2 levels which was used to conduct our analyses. All the participating schools received an operational protocol to install the sensor correctly and download data weekly.We calculated the RTI incidence per week and for the total study period. We performed a descriptive analysis of all survey variables, stratifying them by respiratory-related absence or absence due to other causes. Additionally, we ran a univariate logistic model to study the association between the type of absence and each described variable to obtain the corresponding odds ratios, 95% confidence intervals and p-values.Furthermore, we conducted a Latent Class Analysis (LCA) to explore potential groups of students with similar symptomatology. We started exploring the optimal number of latent classes, trying two to five classes, and we selected the best model using the entropy criterion, which indicates the accuracy of the latent classes, combined with other goodness of fit criteria such as BIC, cAIC and likelihood ratio. All these measures suggested that the optimal number of classes was two. However, we also examined the rest of number of classes to see if their classification had more clinical significance. All statistical analyses were performed in R (version 4.2.2).We registered one-hundred and five school absences during the study, 43 in the first study period (spring 2022), and 62 in the last one (autumn 2022) (Figure 1S ). Among these, we could obtain data related to the diagnosis in 98 (93%) cases, the rest of them were lost during the follow-up of the absence. Seventy-one absences were respiratory-related and 27 were due to other causes, mainly with gastrointestinal symptoms (66.7%). Among the absences due to RTI, the most were upper RTI (56/71, 78.9%), and only six (8.5%) were confirmed infections, 2 caused by influenza virus and 4 due to SARS-CoV-2 infection. These results represent a RTI incidence of 15.9 and 33.1 cases per 100 population in the first and second study period, respectively. The maximum weekly RTI incidence was of 10.2 cases per 100 population in December 12-18, 2022.The clinical, epidemiological and environmental characteristics of study sample are summarized in table 1 , categorized by type of absence (respiratory versus non-respiratory). We found a statistically significant association with absences due to RTI when someone else at home had respiratory symptoms (OR=9.12, CI 95%=2.54-33.39). We found a positive association between higher median levels of CO2at class and respiratory-related absences (OR=1.2, CI 95%=0.98-1.46). Moreover, there were more respiratory absences in autumn (OR=2.4, CI 95%=0.97-5.94). However, these last two associations did not reach statistical significance at a level of 0.05; although their p-values were lower than 0.1.No other epidemiological risk factors were associated with RTI incidence, such as household floor level, number of people living at home, living with smokers, having any comorbidity or being vaccinated against COVID-19.Finally, in Figure 1 , we present the symptomatology of absences belonging to each of the two latent classes. We can see that in the first cluster, the most frequent symptoms were cough, nasal congestion and fever, whereas in the second one, fever, gastrointestinal symptoms, and fatigue predominated.Our findings confirm that the RTI incidence during the study period was very high in children attending in the CNSSC schools, and the main medical cause of school absence. Although differences on school absence due to RTI were observed between spring and autumn, they were not statistically significant and in any case they may be due to the respiratory viruses’ seasonal pattern. In fact, the most important and significantly associated risk factor for RTI was the presence of someone else at home with respiratory symptoms, suggesting that households could be the main setting for initiating of the transmission of RTI.We observed a slightly association between RTI and median of CO2 levels in classrooms (p=0.07), which is an indicator of the degree of ventilation. However, we cannot exclude other potential factors such as rainfall, ambient temperature, or air pollutants (e.g. PM2.5, NO2, etc.) influencing on this outcome, as suggested by other authors10. To our knowledge, previous studies assessed the CO2concentration as a proxy of ventilation to evaluate the risk transmission of SARS-CoV-2 in schools11,12, but they did not analyse the association between CO2 median values and RTI incidence.Finally, we studied the symptomatology associated with the school absences through a LCA. The best approach to differentiate RTI from other causes was using two latent classes, and the most frequent symptoms were cough, nasal congestion and fever.The major strength of this study is our extensive data collection on clinical, epidemiological and environmental factors related to the school and also to the households of the participants. However, there were limitations such as possible incomplete reporting of RTI or insufficient sample size to determine small effect sizes. CO2 concentration was only measured in a selection of classrooms per school, so it may not be representative for the entire study period and school.In conclusion, RTI incidence was very high during the study period being the most important and significantly associated factor with RTI to have anyone else at home with respiratory symptoms. This suggests that households and not schools could be the key epidemiological factor for initiating the transmission of RTI to the children. Improving household preventive measures could reduce childhood RTI. In the LCA, the most frequent symptoms associated with RTI were cough, nasal congestion and fever. Although we found a slightly association between RTI and reduced ventilation we cannot exclude other potential factors influencing on this outcome. The study has been crucial to assess the feasibility and potential utility of collecting both school absence and morbidity data for further developing a systematic monitoring system.

Introduction. Respiratory syncytial virus infection is the leading cause of lower respiratory infection globally. Recently, nirsevimab has been approved to prevent RSV infection. This study explores the economically justifiable price of nirsevimab for preventing RSV infection in Colombia’s children under one year of age. Materials and methods. A static model was developed using the decision tree microsimulation to estimate the quality-adjusted costs and life years of two interventions: a single intramuscular dose of nirsevimab versus not applying nirsevimab. This analysis was made during a time horizon of 1 year and from a societal perspective. Results The annual savings in Colombia associated with this cost per dose ranged from U$ 2.5 to 4.1 million. Based on thresholds of U$4828, U$ 5128, and U$19 992 per QALY evaluated in this study, we established economically justifiable drug acquisition prices of U$ 21.88, U$ 25.04, and U$ 44.02 per dose of nirsevimab. Conclusion the economically justifiable cost for nirsevimab in Colombia is between U$21 to U$44 per dose, depending on the WTP used to decide its implementation. This result should encourage more studies in the region that optimize decision-making processes when incorporating this drug into the health plans of each country.

A document by Miles Weinberger. Click on the document to view its contents.

Paediatric home invasive mechanical ventilation patients are a small but resource intensive cohort, requiring close monitoring and multidisciplinary care. Patients are often dependent on their ventilator for life support, with any significant complications such as equipment failure, tracheostomy blockage, or accidental decannulation becoming potentially life threatening, if not identified quickly. This review discusses the indications and variations in practice worldwide, in terms of models of care, including home care provision, choice of equipment and monitoring. With advances in technology, optimal monitoring strategies for home, continue to be debated: In-built ventilator alarms are often inadequately sensitive for paediatric patients, necessitating additional external monitoring devices to minimise risk. Pulse oximetry has been the preferred monitoring modality at home, though in some special circumstances such as congenital central hypoventilation syndrome, home carbon dioxide monitoring may be important to consider. Children should be under regular follow up at specialist respiratory centres where clinical evaluation, nocturnal oximetry and capnography monitoring and/or poly(somno)graphy and analysis of ventilator download data can be performed regularly to monitor progress. Recent exciting advances in technology, particularly in telemonitoring, which have potential to hugely benefit this complex group of patients are also discussed.

A Pediatric Case of Neuromyelitis Optica and Pulmonary Inflammatory Myofibroblastic TumorAlyson Win, BS ([email protected])a : Conceptualization; writing- original draft; writing- review and editing.Jesper Jiang, BS ([email protected])a:Conceptualization; writing- original draft. John Fitzwater, MD ([email protected])b : writing- review and editing; supervision. Edwin Hernandez Caro, MD ([email protected])c : writing- review and editing. Amy Cruickshank, DO ([email protected])d : writing- review and editing. Duriel Hardy, MD ([email protected])e : writing- review and editing. Ydamis Estrella Perez, MD ([email protected])f : writing- review and editing. Michele Prater, PNP ([email protected])c: writing- review and editing. Malvika Sagar, MD ([email protected])c : Conceptualization; writing- review and editing; supervision.a: Texas A&M Health Science Center School of Medicine, Temple, TXb: Department of Pediatric Surgery, Baylor Scott and White McClane Children’s Medical Center, Temple, TXc: Department of Pediatric Pulmonology, Baylor Scott and White McClane Children’s Medical Center, Temple, TXd: Department of Pediatric Hematology/Oncology, Baylor Scott and White McClane Children’s Medical Center, Temple, TXe: Department of Pediatric Neurology, Dell Children’s Medical Center, Austin, TXf: Department of Pathology, Baylor Scott and White Medical Center, Temple, TX

As our understanding of the uncommon group of disorders called plastic bronchitis has evolved, so too has the classification of these disorders.

Background: Globally, observational studies have demonstrated an association between high levels of air pollution and asthma attacks in children. It remains unclear whether and to what extent exposure may be associated with increased near fatal and fatal attacks. Objective: To systematically review the evidence for an association between ambient outdoor air pollution and fatal and/or near fatal asthma (NFA). Methods: Following Cochrane methodology, we searched MEDLINE, EMBASE, Web of Science, Scopus and Open Grey electronic databases for studies reporting the association of fatal/NFA and air pollution (Particulate Matter (PM), sulphur dioxide, nitrogen dioxide, black carbon and ozone) in children. NFA was defined as requiring intensive care (ICU) management. Results: Two reviewers independently screened 1,358 papers. 276 studies identified asthma attacks related to air pollution, 272 did not meet inclusion criteria after full text review. Four observational studies described fatal/NFA, of which 3 addressed NFA. PM2.5 and ozone (22ppb) were associated with NFA in one study (RR 1.26 CI (1.10-1.44)). PM10 was associated with ICU admission in the context of thunderstorm asthma. Elemental carbon was associated equally with NFA that did not require an ICU admission (p=0.67). Studies of fatal asthma including children did not demarcate age within analysis. Conclusions: Ozone and PM2.5 have been associated with NFA in children but synthesis is limited by the paucity of studies and methodological heterogeneity. Poor reporting of severities of asthma attack does not enable assessment of whether outdoor air pollution is associated with an increased number of NFA and fatal attacks in children.

Tracheostomies are indicated in children to facilitate long-term ventilatory support, aid in the management of secretions, or to manage upper airway obstruction. Children with tracheostomies often experience ongoing airway complications, of which respiratory tract infections are common. They subsequently receive frequent courses of broad spectrum antimicrobials for the prevention or treatment of respiratory tract infections. However, there is little consensus in practice with regard to the indication for treatment/ prophylactic antimicrobial use, choice of antimicrobial, route of administration, or duration of treatment between different centres. Routine antibiotic use is associated with adverse effects and an increased risk of antimicrobial resistance. Tracheal cultures are commonly obtained from paediatric tracheostomy patients, with the aim of helping guide antimicrobial therapy choice. However, a positive culture alone is not diagnostic of infection and the role of routine surveillance cultures remains contentious. Inhaled antimicrobial use is also widespread in the management of tracheostomy associated infections; this is largely based upon theoretical benefits of higher airway antibiotic concentrations. The role of prophylactic inhaled antimicrobial use for tracheostomy associated infections remains largely unproven. This systematic review summarises the current evidence base for antimicrobial selection, duration, and administration route in paediatric tracheostomy associated infections. It also highlights significant variation in practice between centres and the urgent need for further prospective evidence to guide the management of these vulnerable patients.

Pathogenic variants in the Surfactant Protein C gene ( SFTPC) result in fibrotic childhood interstitial lung disease (chILD). We previously reported three children with SFTPC pathogenic variants with respiratory failure who were supported by chronic invasive ventilation via tracheostomy as an alternative to lung transplantation or comfort care [(1)](#ref-0001). We present two children with SFTPC pathogenic variants treated with non-invasive ventilation (NIV) (Figure 1).

In recent decades, the patient survival is increased due to the advances in intensive care units and development of modern mechanic ventilators. Unfortunately, it is not always possible to wean these children from mechanical ventilation. Recently, after placement a tracheostomy tube, they can support at home with non-invasive or invasive mechanical ventilation. Most of the children who need ventilation support at home have neurological impairment. The nutritional issues and gastrointestinal complications are well defined in critically ill patients, but there are very limited studies on the children with tracheostomy. Considering that majority of the patients has neuromuscular disorders, the nutritional and gastrointestinal problems of the children with tracheostomy are discussed, in the light of the knowledge on critically ill patients.

We are presenting a teenage girl with a recent history of virilism who presented with tension pneumothorax. Subsequent contrast-enhanced chest CT revealed multiple bilateral irregular thin-walled cysts of varying sizes with numerous solid noncalcified pulmonary nodules and pulmonary artery pseudoaneurysms. Lung biopsy revealed trophoblastic neoplasm with metastasis to both lungs. We discussed the differential diagnosis of diffuse cystic lung diseases. While diffuse cystic lung diseases in children are rarely due to malignancy, we emphasize that clinicians be aware of the possibility of metastatic lung diseases and their radiologic features when encountering diffuse cystic lung diseases.