Pulmonary artery sling is a rare childhood vascualr ring that is frequently associated with tracheal stensois. Consequently, neonates may present with a crictal airway obstruction if tehre is long segemt tarcheal stensosi and complete rings. Rapid diagnosis of this cardiac vascular malfomation and extent airway involvement is essentail as longterm outcoem can be excellent follwoing surgicla repair. In this review we focus on airway invetsigation and management for this challenging congential condition.

Introduction: Congenital diaphragmatic hernia (CDH) is known with high mortality rates and significant pulmonary morbidities. The objective of this study was to describe the histopathological findings of necropsy and clinical manifestations in CDH patients to find the clinicopathological correlations. Methods: We reviewed the postmortem findings with associated clinical characteristics retrospectively in 8 CDH cases from 2017 to2022 July. Results: Of the eight cases, one was bilateral congenital eventration diagnosed by autopsy. Severe pulmonary hypertension with a right to left shunting of large patent ductus arteriosus (PDA) obtained from echocardiogram were most common. And the average time of survive was 46 (8-624) h. According to the autopsy reports, the major pathological lung changes were diffuse alveolar damages (congestion and hemorrhage) and hyaline membrane formation. Notably, although the lung volume was significantly reduced, pulmonary structural dysplasia was not observed at all, presented normal lung development (50%); bilateral (25%) or ipsilateral (25%). Lobulated deformities were accompanied in three (37.5%) cases. All patients exhibited large PDA and a patent foramen ovale with increased right ventricle (RV) volume, and the myocardial fibers were slightly congested and swollen. Pulmonary vessels showed mild to moderate arterial media thickening. Lung hypoplasia and diffuse lung damages reduced gas exchange, meanwhile the PDA and PH caused RV failure, contributed a clinical picture of organ dysfunction, which lead to death. Conclusions: Pulmonary structures have certain heterogeneity in CDH. The arteries’ pathological changes are not consistent with clinical diagnosis. And the adverse outcome may should be due to the cardiopulmonary vicious cycle.

The current system for disclosing financial conflicts of interest (COIs) can be traced back to the “Uniform requirements for manuscripts submitted to medical journals,” published in 1997. Meanwhile, new molecular and genetic therapies are transforming clinical medicine and these therapies have radically altered the financial landscape of drug development. The average price of new drugs has risen sharply, and in niche areas like rare diseases, the cost of new therapies can be stratospheric. Now, even rare or “orphan” diseases are funded by private investment. With high profitability, there is a new pathway for drug development involving unprecedented ties between industry and academia. The potential for COIs has greatly expanded and decisions that were once science-driven have become profit-driven. As a result, the risk that marginally effective, ineffective and even hazardous treatments will make their way to patients has greatly increased. In this editorial, we propose a system built on process-based COIs. This new system would trace the developmental journey of a drug from the lab to patients. All financial ties that institutions and individuals have to the drug would be disclosed at each developmental step. The goal would be to create a healthy skepticism and debate over the studies behind new drugs, restoring a scientific focus to the assessment of drug efficacy. When society’s limited resources are used to pay for drugs whose excessive cost primarily benefits investors and corporations, it derails scientific objectivity, harms patients, and threatens the financial stability of our health care systems.

Addressing the recognised challenges and inequalities in providing high quality health care for rare diseases such as children’s interstitial lung disease (chILD) requires collaboration across institutional, geographical, discipline, and system boundaries. The Children’s Interstitial Lung Disease Respiratory Network of Australia and New Zealand (chILDRANZ) is an example of a clinical network that brings together multidisciplinary health professionals for collaboration, peer learning, and advocacy with the goal of improving the diagnosis and management of this group of rare and ultra-rare conditions. This narrative review explores the multifaceted benefits arising from social learning spaces within rare disease clinical networks by applying the Value Creation Framework. The operation of the chILDRANZ network is used as an example across the framework to highlight how value is generated, realised, and transferred within such collaborative clinical and research networks. The community of clinical practice formed in the chILDRANZ multidisciplinary clinical peer support meetings provides a strong example of social learning that engages with the uncertainty inherent in rare disease diagnosis and management and pays attention to generate new knowledge and best practice to make a difference for children and families living with chILD. This review underscores international calls for further investment in, and support of, collaborative expert clinical networks for rare disease.

Objective: SARS-COV-2 pandemic had a profound impact on acute bronchiolitis epidemiology, especially in RSV diffusion and the burden of disease with implications on the management of prophylaxis and health resources. We aimed to compare clinical and epidemiological characteristics of bronchiolitis before and during the SARS-CoV-2 pandemic.   Methods: We conducted an observational study involving children aged 0-2 years with bronchiolitis admitted to a tertiary children’s hospital during the last 5 years. Demographic, clinical, microbiological, and outcome data were collected. Comparison between pre- and post-pandemic period, RSV positive versus non-RSV patients, and SARS-CoV-2 positive vs negative patients were carried out.    Results: A total of 647 patients admitted for bronchiolitis were included (264 female,40.8%, median age 78 days). Molecular diagnostic tests were performed in 617 patients (95.4%) with RSV detected in 62.5% of patients. SARS-CoV-2 was found in 3.9% of hospitalized bronchiolitis (3.9%). We observed a progressive increase in bronchiolitis admissions and a statistically significant increase over the years in the need for respiratory support. Conversely, this was not true for mechanical ventilation, duration of respiratory support, ICU admission, and length of stay. During the pandemic period children with prematurity increased, although only 1 child had an indication for prophylaxis. Discussion: We confirm the stronger impact of bronchiolitis in the 2021-22 season, which is likely explained by the higher prevalence of RSV and the immunity debt theory. However, our findings were conflicting in terms of worsening clinical severity. The increase of children with prematurity and the inter-seasonal spread of RSV highlight the importance of epidemiological surveillance systems that monitor RSV circulation.

Asthma deaths were rare in children but attention should be paid to those with a severe exacerbation (requiring systemic steroids, an ED visit or hospitalization) in the past year, more than three beta-agonist refills in a year and poor adherence to controller medication.

Right and left ventricular dysfunction are commonly encountered in pulmonary disorders. Pulmonary hypertension exerts pressure load on the right ventricle, subsequent RV-LV interactions through shared septum, pericardium and blood supply lead to LV dysfunction. The early detection and treatment of pulmonary hypertension is crucial to improve outcomes of pulmonary disorders. New advanced echocardiographic techniques such as pulmonary artery circumferential strain and pulmonary artery blood speckle tracking might help in replacing invasive hemodynamics, for early detection of pulmonary vasculopathy in pulmonary disorders.

Backround: Mechanical ventilation is associated with mortality/morbidities in preterm infants. Nearly a third of these infants fail extubation and this may increase morbidities. Objective: To evaluate the association of symptomatic patent ductus arteriosus with failure of extubation among preterm infants. Methods: This was a retrospective study on preterm infants (birth weight <1,250 grams and gestational age ≥23weeks) born between January 2009 and December 2016, who were mechanically ventilated and extubated within the first 60 days of age. Results: 360 infants were evaluated, of these, 26% failed and 74% succeeded the initial extubation attempt. On adjusted analysis, symptomatic ductus was associated with an increased risk of extubation failure. Conclusion(s): Presence of symptomatic patent ductus arteriosus was associated with extubation failure. Further investigations are needed to establish whether screening for presence of ductus and treatment of the same, prior to extubation among these infants, improves chances of successful extubation and cardiorespiratory outcomes.

Background: Uniformity and compliance with clinical practice guidelines (CPGs) for use of palivizumab in preventing severe RSV infection in Australian high-risk infants remain unclear. Methods: An online survey was conducted across the Australian and New Zealand Neonatal Network (ANZNN) to determine clinical practices around palivizumab. A literature search was also performed to identify and compare national and international guidelines. Results: Sixty-five of 422 ANZNN members completed the survey. Respondents included 61 senior medical staff of consultants/staff specialists (78%) and four nursing staff (6%). Infants most likely to be recommended palivizumab included preterm infants born <29 weeks gestational age (GA) (30%), children with chronic lung diseases (CLD) born <32 weeks GA (40%), and with hemodynamically significant heart disease (35%). Many respondents (53%) stated that CPGs for palivizumab were developed locally. Twenty guidelines (10 international and 10 domestic) were obtained in total; 16(80%) recommended palivizumab use in preterm infants, 16(80%) recommended use in infants with CLD, 17(85%) congenital heart disease (CHD) and six (30%) bronchopulmonary dysplasia (BPD). Eight (40%) guidelines provided specific recommendations for immunocompromised infants. Canada, Western Australia, and American Academy of Paediatrics provided recommendations for Indigenous children. Frequency and dosage of palivizumab was universal across all CPGs. None of the international guidelines obtained were from low- or middle-income countries. Conclusions: Standardisation of CPGs may improve clinical decision making around use of palivizumab in high-risk infants.

Objectives: Intralobar pulmonary sequestration (ILS) is rare and its optimal clinical management remains ambiguous. This study aimed to introduce our ten-year experience in clinical management of ILS. And the application of our novel surgical method, thoracoscopic anatomical lesion resection (TALR) on ILS was introduced. Materials and Methods: Patients with ILS who received treatment between December 2010 and December 2020 were included in this study, retrospectively. A binary logistic regression model was used to assess risk factors for preoperative symptoms. Intraoperative and postoperative outcomes were compared between the thoracoscopic lobectomy and lung-sparing surgery groups. Results: A total of 112 patients were included in this study. Age and maximum cyst diameter were risk factors for preoperative symptoms. Lung-sparing surgery proved to be safe and feasible with no residual lesions. Conclusions: The overall prognosis of early thoracoscopic surgery for ILS was good. Lung-sparing surgery, especially TALR could be used as a first-line surgery for ILS. It may resolve the long-standing controversy over whether surgery for asymptomatic patients with ILS.

Objective: Patent ductus arteriosus (PDA) is a common complication among premature infants, and it may be responsible for prematurity-related complications, such as bronchopulmonary dysplasia (BPD). It is unclear whether different interventional methods contribute to the severity of BPD. To date, surgical ligation and the transcatheter approach have shown equal success in premature infants with hemodynamically significant PDA after medical treatment failure. Immediate improvement in the respiratory condition after transcatheter closure has been reported. However, the short-term pulmonary outcome has not been clarified yet. Methods: This retrospective study investigated infants born with a body weight less than 1000 g and underwent either surgical ligation or transcatheter closure of PDA in a single tertiary institution. The infants were divided into groups according to the type of procedure (surgical ligation or transcatheter occlusion). The primary outcome was the severity of BPD at discharge or at post-menstrual age of 36 weeks. The outcome was analyzed with logistic regression. Results: Forty-four patients met the inclusion criteria, and of these, 14 underwent transcatheter occlusion and 30 underwent surgical ligation. The overall birth body weight and gestational age ranges were not different. The univariate model revealed an association between the procedure type and BPD severity. The multivariate model confirmed associations of BPD severity with procedure type and severe respiratory distress syndrome requiring surfactant after adjusting for confounders. Conclusion: Compared with the transcatheter approach, surgery for PDA in extremely preterm infants is associated with severe BPD at discharge. Further large-scale studies are needed to determine the exact mechanism.

In response to: Spurr R, Ng E, Onchiri FM, Rapha B, Nakatumba-Nabende J, Rosenfeld M, Najjingo I, Stout J, Nantanda R, Ellington LE. Performance and usability of a new mobile application for measuring respiratory rate in young children with acute lower respiratory infections. Pediatr Pulmonol. 2022 Aug 22. doi: 10.1002/ppul.26125.

Clinician perspectives may inform health service strategies to meet optimal nutrition needs for infants with cystic fibrosis (CF). We conducted a qualitative study with CF-specialized dietitians (RDs) and physicians between July to December 2020 to characterize the current state of infant nutrition care delivery and organize input into a conceptual model to inform CF care program strategies. Among 42 participants, 36 completed survey responses and 6 completed interviews; 93% were RDs. Three global themes emerged in the current care model: nutrition management, family-centered connections, and collaborative care delivery. Within nutrition management clinicians emphasized providing education, setting goals, and maintaining adequate follow-up with families. Under family-centered connections clinicians expressed the need to foster relationships with families and link families to resources for assistance to social stressors such as food insecurity. Collaborative care delivery for clinicians interviewed was defined by sharing expertise from across the interdisciplinary team. Based on the timing of this study, clinicians reported compelling examples for various modes of telehealth and home weight monitoring to facilitate and support these domains of nutrition care, including potential advantages for education, supporting family needs, and communication. We integrate these themes to propose a conceptual model for integration of in-person and telehealth activities to enhance quality infant CF nutrition care delivery. Future implementation can refine this model through testing of practical telehealth interventions to optimize nutrition outcomes for infants with CF.

Background. Cystic fibrosis (CF) is an autosomal recessive disorder associated with an increased susceptibility to respiratory infections that cause progressive decline in lung function and lead to lung damage and chronic respiratory failure. To analyze the CF-related mortality trends in the Mexican population during a 22-year period.  We conducted a mortality trend analysis using death certificates data. Trends in CF-specific and in age-specific mortality rates were evaluated using Joinpoint regression analysis. Among subjects ≤40 years, 1184 CF-related deaths were identified. In 1999–2009 median age at death was 7 years compared to 10 years in 2010–2020. Overall mortality rate increased from 0.03 per 100,000 in 1999 to 0.06 per 100,000 in 2020. A decline in mortality rate for patients ≤28 days and an increase in mortality rate in older age groups was observed. Conclusion. The increasing trend in overall mortality, associated with a downward trend in neonatal mortality and an increase in median age at death is conceivably due to enhanced diagnosis, as well as major advances in treatment modalities, leading to higher survival rates. Key words: age at death, cystic fibrosis, epidemiology, low- and middle-income countries, mortality, survival rate.

As the population of ventilator-dependent children (VDC) with tracheostomies due to underlying severe BPD grows, there is an increasing need to shift the care of these children from hospital to home. Transitioning the ventilator-dependent child from the hospital to home is a complex process that requires coordination between the medical team and the family. One crucial step in the process is transitioning from an Intensive care unit (ICU) ventilator to a portable home ventilator (PHV). The Clinical team needs to understand the nuances in transitioning to PHV, including assessing readiness to transition and choosing the optimum settings on an available home ventilator. In recent years, various ventilator modes have been available in PHV that can help achieve synchronous breathing to allow for adequate gas exchange for the infant. This review details some approaches to asses readiness to transition and the process of Transition along with commonly used modes of support available in PHV, as well as the primary and secondary settings in which we should be mindful in supporting a child with chronic respiratory failure in the home setting.

While our survey showed that PwCF felt that the COVID-19 pandemic had impacted their mental health more than ETI therapy, around 9% of survey responders felt that ETI did cause an increase in either anxiety or depression. This finding was similar to the numbers Spoletini and colleagues found in their adult clinic. They also found that a dose reduction of ETI improved or resolved mental health adverse events for most patients. [2] A recently published study by Guimbellot and colleagues studied ivacaftor levels in PwCF who were on ivacaftor monotherapy and showed many patients had levels higher than the published minimum effective concentrations.[6] Thus, there may be a correlation between ETI levels and mental health adverse events that should be further explored in a prospective manner.

NTM-infected CF patients may be at risk of being underdiagnosed or inappropriately treated when relying on culture and susceptibility results from non-specialized laboratories. Since this was a small study with convenience samples, a larger study needs to be carried out. If our findings are confirmed, the drivers should be elucidated for the discrepant results. Given the increasing prevalence of NTM in the population at large and not in the CF community alone[[13]](#ref-0013), elucidating any differences in testing to ensure the correct identification, including subspeciation and antimicrobial susceptibilities should be paramount

Introduction: Bronchopulmonary dysplasia (BPD) is characterized by lung injury with varying degrees of disrupted alveolarization, vascular remodeling, inflammatory cell proliferation, and pulmonary edema. Diuretics are often used to ameliorate the symptoms or progression of BPD. Our primary objective was to use lung ultrasound (LUS) to determine if diuretics decrease pulmonary edema in infants with BPD. The secondary objective was to assess changes in respiratory support during the first week after initiation of diuretics. Methods: Premature infants requiring non-invasive respiratory support and starting diuretic therapy for evolving BPD were compared with a similar group of infants not receiving diuretics (control). For the diuretic group, LUS exams were performed before and on days 1, 3 and 6 after initiation of treatment. For the control group, LUS was performed at equivalent time points. A composite pulmonary edema severity (PES) score of 0 to 5 was calculated based on the total number of B-lines in 6 scanned areas. Respiratory support parameters (FiO2, nasal cannula flow or CPAP) were also recorded. Results: Infants in the diuretic (n=28) and control (n=23) groups were recruited at median corrected gestational ages of 34.2 (33.3-35.9) and 34.0 (33.4-36.3) weeks, respectively ( p=0.82). PES scores, FiO2, and respiratory flow support decreased significantly from day 0 to 6 ( p<.0001, p=0.001, and p=0.01, respectively) in the diuretic group, but not in the control group. Conclusion: Diuretic use is associated with decreased pulmonary edema and improved oxygenation in infants with BPD during the first week of treatment.