Objective: Report a novel technique of flexible endoscopy (FE) with noninvasive ventilation (NIV) and sustained pharyngeal inflation (FE-NIV-SPI) in assessing aeroesophageal tracts (AET) to facilitate early detect laryngeal clefts (LC). Methods: Medical charts and FE videos of the children who were diagnosed as LC in a tertiary care hospital between 2000 and 2020 were retrospectively reviewed and analyzed. FE-NIV-SPI technique was applied for all the reviewed children. Results: Of all the FE videos reviewed, twelve infants with laryngeal clefts were collected. This equates to a prevalence of 0.28% in all the children underwent FE at our institution. Their mean age was 5.0 ± 4.9 month-old and the mean body weight was 4.7 ± 2.3 kg. Nine (75%) infants were referred in without LC diagnosis, which was missed by 11 prior bronchoscopy and 5 computer tomography exams. With FE-NIV-SPI, the pharyngolaryngeal space could be pneumatically expanded and allowed detailed assessment. All LC types and coexisted AET lesions were visualized at the first FE-NIV-SPI examination with a mean time of 4.2 ± 0.9 minutes. They were eight type I, two type II and one type III. Ten (83.3%) infants coexisted airway malacia. Conclusion: Routinely using the FE-NIV-SPI technique can help detect the LC defect and associated AET lesions at the first assessment.

Introduction A risk associated with cystic fibrosis newborn screening (CFNBS) is parental misunderstanding of genetic information generated by the over 6,600 positive screens reported annually in the US. CFNBS algorithms incorporating DNA analysis can generate genetic information that requires clinical interpretation and has significance for the newborn, parents, and other relatives. Engagement between CF care centers and trained genetic counseling providers, such as licensed and/or certified genetic counselors (GCs), is variable and limited in providing information to CFNBS positive (CFNBS+) families. Methods Using a modified Delphi process, a workgroup of CF experts developed recommendations for engagement of genetic counseling services in CF care centers where CFNBS+ diagnostic evaluations are performed. Statements were assessed over three rounds of surveys, one face-to-face meeting, and through public feedback. Results Seventeen statements achieved >80% consensus (range: 82-100%). The workgroup affirmed prior CFF policy statements recommending genetic counseling for parents of infants with CFNBS+. The remaining statements addressed infrastructure and logistics of genetic counseling services, including defining appropriate training for genetic counseling providers and counseling content, establishing a path to equal access to genetic counseling providers across CF care centers, and setting a standard for client-centered CFNBS genetic counseling that is respectful of diverse patient needs and autonomy. Conclusions Implementation of client-centered genetic counseling for CFNBS+ families in CF care centers by providers with expertise in both CF and genetic counseling will require efforts to further define core concepts, enhance education of providers, and develop opportunities for access via telemedicine.

Objective: Non-invasive respiratory support has reduced the need for mechanical ventilation and surfactant administration in very premature neonates. We sought to determine how the increased use of non-invasive ventilation and less surfactant instillation has impacted the development of bronchopulmonary dysplasia (BPD) and compared BPD outcome applying four currently used definitions. Study Design: This is a retrospective, single center cohort study of neonates born at less than 28 weeks gestation between 2010 and 2018. A respiratory practice change (less surfactant and more non-invasive ventilation) occurred in 2014 following participation in the SUPPORT trial. Therefore, patients were divided into 2 epochs to compare postnatal respiratory and clinical course and BPD outcomes across four currently relevant definitions (VON, NICHD, Canadian, NRN). Results: Clinical and demographic variables were similar between epochs. Despite significant differences in maternal and infant characteristics and clinical course, the incidence of BPD was not significantly different between the 2 epochs regardless of the BPD definition utilized. There was a wide range in the incidence of BPD depending on the definition used. Conclusions: Despite decreased use of invasive mechanical ventilation and surfactant administration between the two epochs, the incidence of BPD did not change and there was wide variation depending on the definition used. A better understanding of the risk factors associated with BPD and a consensus definition is urgently needed in order to facilitate the conduct of clinical trials and the development of novel therapeutic interventions to improve outcome.

Introduction: Temporal trends in CF survival from low-middle-income settings are poorly reported. We describe changes in CF survival after diagnosis over 40 years from a South African (SA) CF center. Methods: An observational cohort study of people diagnosed with CF from 1974 to 2019. Changes in age-specific mortality rates from the year 2000 (versus before 2000) were estimated using multivariable Poisson regression. Data were stratified by current age < or ≥ 10 years and models controlled for diagnosis age, sex, ethnicity, genotype, and P. aeruginosa (PA) infection. A second analysis explored association of mortality with weight and FEV1z-scores at age 5-8 years. Results: 288 people (52% male; 57% Caucasian; 44% p.Phe508del homozygous) were included (median diagnosis age 0.5 years: Q1,Q3: 0.2, 2.5); 58 (35%) died and 30 (10%) lost to follow-up. Among age >10 years, age-specific mortality from year 2000 was significantly lower (adjusted hazard ratio aHR: 0.14; 95% CI: 0.06,0.29; p<0.001), but not among age <10 years (aHR: 0.67; 95% CI: 0.28,1.64; p=0.383). In children <10 years, Caucasian ethnicity was associated with lower mortality (aHR 0.17; 95% CI 0.05,0.63), and time since first PA infection with higher mortality (aHR 1.31; 95% CI 1.01,1.68). Mortality was 7-fold higher if FEV1z was < -2.0 at age 5-8 years (aHR 7.64; 95% CI 2.58,22.59). Conclusion: Overall, CF survival has significantly improved in SA from year 2000 in people older than 10 years. However, increased risk of mortality persists in young non-Caucasian children, and with FEV1z<-2.0 at age 5-8 years.

Objective: The COVID-19 pandemic is an important cause of morbidity and mortality, which has had a negative impact worldwide. Our aim was to describe clinical findings and outcomes of SARS-CoV-2 viral infection and Covid-19 disease cared for at a large pediatric tertiary care hospital during the first year of the pandemic. Methods: Patients aged 1 month to 18 years who were diagnosed as having COVID-19 between March 2020 and April 2021 were included. The files of patients diagnosed with covid-19 were reviewed retrospectively. Results: 467 children were included in the study. There were 34 (7.3%) patients under one year of age, 111 (23.8%) between 1-5 years, 98 (30.4%) between 5-10 years, 142 (30.4%) between 11-15 years, and 82 (17.6%) age over 15 years. Fever (88.2%), vomiting (32.4%), and diarrhea (29.4%) in patients aged under 1 year, sore throat (36.6%) in patients aged 11-15 years, and dysgeusia (11%), anosmia (14.6%), headache (18.3%), malaise (40.8%), myalgia (28%), and dyspnea (17.1%) in those aged over 15 years of age were found significantly more common compared with the other age groups (p<0.05). Thirty-five (7.5%) patients were asymptomatic, 365 (78.1%) had mild disease, 35 (7.5%) were moderate, 27 (5.8%) were severe, and five (1.07%) were critical. Leukocyte count, erythrocyte sedimentation rate, ferritin, and C-reactive protein values were significantly higher in hospitalized patients. Four patients died during the study period (0.8%, 4/467). Conclusion: While SARS-CoV-2 infection may be asymptomatic and Covid-19 disease usually has a mild clinical course, some children have severe disease or mortality.

Primary ciliary dyskinesis (PCD) is an autosomal recessive disorder associated with impaired mucociliary clearance caused by defects in ciliary structure and function. The major clinical feature of PCD is recurring or persistent respiratory tract infection. Respiratory tract colonization with drug-resistant organisms impact the frequency of infections and lung function decline. Protective gear has been employed by caregivers in an attempt to control respiratory tract bacterial spread between patients with cystic fibrosis but use in PCD is not known. We conducted a web-based survey to investigate infection control and prevention practices of PCD centers in North America. The response rate was 87.0%. Prior to the COVID-19 pandemic, glove, gown and mask use was variable, and only 3.7% of centers used masks during encounters with PCD outpatients. After COVID-19 mandates are lifted, 48.1% of centers plan to continue to use masks during outpatient care, while the practice regarding use of gloves and gowns was not influenced by the current pandemic. There is no uniform practice for infection control in PCD care indicating the need for practice guidelines. Mitigation of respiratory virus transmission learned during the COVID-19 pandemic may impact future infection control approaches used for patients with PCD and other lung diseases.

Background: Parapneumonic effusions and empyema are the most frequent complication of paediatric pneumonia. Treatment options include chest drain and fibrinolytics (CDF) or thoracoscopic surgery. CDF is considered less invasive, more cost effective though with higher rates of reintervention. Pleural fluid characteristics on ultrasound may identify cases at increased risk of treatment failure with primary CDF. Methods: A retrospective cohort 2011-2018 of complicated pneumonia managed with primary CDF. Cases were reviewed using ultrasound criteria to describe pleural fluid. We compared ultrasound findings and treatment failure. Results: We report 137 cases with a median age 3.8 years and 43% female. Treatment failure occurred for 32/137 (23%) cases. The interobserver reliability was substantial for the number of septations (Kappa 0.72, 95% CI 0.6 to 0.8), moderate for the size of the largest locule (Kappa 0.55, 95% CI 0.4 to 0.7) and fair for the level of echogenicity (Kappa 0.22, 95%CI 0.1 to 0.3), pleural thickening (Kappa 0.28, 95% CI 0.2 to 0.4), maximum effusion depth (Kappa 0.37, 95%CI 0.2 to 0.5) and radiologist’s risk for reintervention (Kappa 0.32, 95% CI 0.2 to 0.5). Hyperechoic pleural fluid was associated with treatment failure, with cases nearly five times more likely than anechoic fluid to have a reintervention (OR 4.9 95%CI 1.7 to 14.2, p=0.04). Treatment failure was not associated with other variables. Conclusion: We did not find an association between ultrasound characteristics and treatment failure for complicated pneumonia managed with primary CDF. Inter-observer agreement of ultrasound findings was difficult despite more objective criteria.

Abstract Objective: To analyze baseline clinical and laboratory characteristics and explore the possible predictors of lung necrosis severity in children with community-acquired necrotizing pneumonia (NP). Methodology: This retrospective observational study was performed in a tertiary referral center. A total of 104 patients aged <15 years with community-acquired pneumonia and radiologically confirmed NP were included. Patients were classified into the mild, moderate, or massive necrosis groups. Results: Among them, 29, 41, and 34 patients had mild, moderate, and massive necrosis, respectively. Moreover, 34.6% of the patients were admitted to the pediatric intensive care unit. Massive necrosis was more likely to occur during winter (p<0.05) and was associated with more severe clinical outcomes, such as longer duration of fever, longer hospitalization, increased mortality, and a higher risk of subsequent surgical intervention (p<0.05). Multivariate analysis demonstrated that the following were independent risk factors for massive necrosis: C-reactive protein (CRP) ≥ 122 mg/L (adjusted odds ratio [aOR], 8.780; 95% confidence interval [CI], 3.320–21.089; p=0.003), serum albumin ≤ 30.8 g/L (aOR, 11.608; 95% CI, 5.147–27.058; p=0.001), and immunoglobulin M (IgM) ≤ 95.7 mg/dL (aOR, 7.152; 95% CI, 2.240–17.692; p=0.021). Receiver operating characteristic analysis demonstrated that these variables showed good diagnostic performance for differentiating patients with massive necrosis from all patients with NP. Conclusion: NP is a potentially severe complication of pediatric community-acquired pneumonia. Different severities of lung necrosis can lead to different clinical outcomes. CRP, serum albumin, and IgM levels are independent predictors of the degree of lung necrosis.

Introduction: Non-adherence to inhaled corticosteroids (ICS) in children with asthma leads to significant morbidity and mortality. Few interventions to improve adherence have been effective and little is known about what contributes to intervention effectiveness. This systematic review summarises the efficacy of these interventions and the characteristics of effective interventions to inform future studies aiming to improve adherence to ICS in children with asthma. Methods: PubMed, Embase, PsychINFO, Medline, Web of Science, and International Pharmaceutical Abstracts were systematically searched on the 3rd of October 2020 for randomised control trials measuring adherence to ICS in children with asthma. A narrative synthesis was conducted focusing on intervention efficacy and study reliability. Intervention content was coded based on the NICE guidelines for medicines adherence (The Perceptions and Practicalities Approach, PAPA) and Behaviour Change Techniques (BCT), to determine the effective aspects of the intervention. Results: Of 240 studies identified, 25 were eligible for inclusion. Thirteen of the twenty-five studies were categorised as being highly reliable. Nine of the thirteen studies were effective at increasing adherence and six of those met the criteria for a PAPA intervention. Conclusion: Adherence interventions in children with asthma have mixed effectiveness. Effective studies tended to be of higher quality, were tailored to individuals perceptual and practical adherence barriers, and used multiple BCTs. However, due to the small number of included studies and varying study design quality, conclusions drawn here are preliminary. Future research is needed to test a PAPA-based intervention with a rigorous study design as outlined in this review

Type of Study : Letter to EditorFull Title : High Flow Nasal Cannula (HFNC) during flexible bronchoscopy, is it cost-effective?Gaurav Gautam MD, Prawin Kumar MD, Jagdish P. Goyal MDDepartment of Pediatrics, All India Institute of Medical Sciences Jodhpur, Rajasthan, IndiaAbbreviated title: HFNC during flexible bronchoscopy

Introduction Understanding the impact of scoliosis surgery on lung function is important for counseling patients about risks and benefits of surgery. We prospectively compared the trends in lung function test (LFT) results prior to and after scoliosis surgery in children with neuromuscular diseases, or dysmorphic syndromes. We hypothesized a stabilization. Methods We prospectively included children with neuromuscular or syndromic scoliosis able to perform LFTs. We studied (Forced) Vital Capacity ((F)VC), the ratio of Forced Expiratory Volume in 1 second (FEV1) and FVC, and Peak Expiratory Flow (PEF). Preoperative LFT results were compared with results 3-4 months after surgery. The mean monthly change in LFT results up to 2 years after surgery was compared with the preoperative natural history using linear mixed effects models. Results We included 43 patients. No significant change was observed in absolute values of (F)VC, FEV1/FVC and PEF prior to and after surgery. Median standardized VC, FVC and PEF decreased significantly after surgery from 59 to 58%, 60 to 51% and 61 to 53% respectively. The monthly rate of change in FVC was -0.13 % (95% CI -0.42 to 0.17) prior to surgery and -0.20% (95% CI -0.42 to 0.03) after surgery, mean difference -0.07 (95% CI -0.46 to 0.31; p=0.36). Conclusion No stabilization of lung function 3-4 months after scoliosis surgery was observed in children with neuromuscular and syndromic scoliosis with restrictive lung function disease. The effect of surgery on the rate of lung function decline remains inconclusive.

Thrombotic complications of SARS-CoV-2 have been increasingly recognized as an important component of COVID-19 in adults; however, they have been less evident in children. We report a case of a teenager with positive SARS‐CoV‐2 RT–PCR and underlying prothrombotic risk factors, including aromatase inhibitor therapy, who developed deep vein thrombosis resulting in pulmonary embolism. Laboratory tests revealed deranged coagulation parameters (high D-dimers and Factor VIII and low antithrombin). The patient required intensive care and was managed with anticoagulants, dexamethasone and antithrombin concentrate. Clinical condition and hemostatic profile gradually improved. A review of the available literature for similar cases is presented.

A document by Rajath R. Click on the document to view its contents.

Background: The largest age group among children and adolescents referred for lung transplantation for cystic fibrosis (CF) have been those who in the pubertal or post pubertal age range. However, over 100 younger patients with CF have undergone lung transplantation over the last three decades in the USA. Methods: We performed a retrospective review of our experience with 18 children with CF who underwent lung transplantation in our center before the age of 11 years. Results: Notable findings were a high prevalence of methicillin-resistant Staphylococcus aureus, a high prevalence of diabetes mellitus and an impressive prevalence of consolidated lobar or whole lung disease. Post-transplant outcomes, however, were comparable to those older than 10 years of age in our center. Conclusions: In an era of increasingly effective medications modifying the natural history of CF, identification of risk factors for early severe lung disease in CF remains relevant and potentially more important for effective intervention in order to extend life.

Objective: To assess the quality of life (QoL) of Cystic fibrosis (CF) patients in West Bank, Palestine using the Cystic Fibrosis Questionnaire-Revised (CFQ-R) form. Method: A cross-sectional study involving application of CFQ-R questionnaire in CF patients attending pediatric pulmonology clinic at Caritas Baby Hospital (CBH). Their health status was assessed by measuring different parameters including pulmonary function test (FEV1) and body mass index (BMI). Results: The sample consisted of 77 patients from 58 families: 46.75% were males. Mean age was 10.7 years. Patients were divided into three groups by age in years: group I (< 6), II (6-13), and III (≥ 14). The highest and lowest CFQ scores were for the eat domain in group III (55.56 ± 22.49) and the body domain in group II (14.48 ± 17.67), respectively. Illness severity as measured by FEV1 mean value 69.6. BMI mean value of 15.998. The overall mean age at time of diagnosis in our sample was 4.16 years (± 6.239). The study showed that 1.7% of families had four affected siblings and 21% had death cases related to CF. Finally, all parameters for CF patients in West Bank, Palestine appear to be noticeably lower than those reported in other countries. Conclusions: quality of life for patients with CF is poor relative to international standards, the medications used including hypertonic saline and Gentamycin IV form used as nebulizer solution are not first line therapies around the world. This study illustrates the need of new therapies for CF patients in Palestine.

Background: Bronchopulmonary dysplasia (BPD) is the most common cause of chronic lung disease in children born prematurely. There is little information about the epidemiology and severity of BPD places with high altitude. This study aimed to evaluate the frequency of BPD severity levels and the associated risk factors with severity in a cohort of preterm newborns ≤34 weeks of gestational age born in Rionegro, Colombia Materials and methods: We carried out a retrospective analytical cohort of preterm newborns without major malformations from Rionegro, Colombia between 2011-2018 admitted to neonatal intensive unit at high altitude (2200m above sea level). The main outcomes were the incidence and severity of bronchopulmonary dysplasia. Results: The bronchopulmonary dysplasia incidence was 25.7% (95% CI, 21.6-29.9). Bronchopulmonary dysplasia was moderate in 62.1% of patients and severe in 26.7%. After modeling regression analysis, the final variables associated with BPD severity levels were: sepsis (OR 2.37 CI 95% 1.04-5.40) and pulmonary hypertension (OR 3.79 CI95% 1.19-12). Conclusion: The incidence of BPD was higher and similar to cities with higher altitudes. In our population, the variables associated with BPD severity levels were: duration of oxygen therapy and pulmonary hypertension. It is necessary to increase the awareness of risk factors, the effect of clinical practices, and early recognition of bronchopulmonary dysplasia to reduce morbidity in patients with this pathology.

Background Solid organ transplantation (SOT) has become commonly used in children and is associated with excellent survival rates into adulthood. Data regarding long-term respiratory outcomes following pediatric transplantation are lacking. We aimed to describe the prevalence and nature of respiratory pathology following pediatric heart, kidney, and liver transplant, and identify potential risk factors for respiratory complications. Methods Retrospective review involving all children under active follow-up at the provincial transplant service in British Columbia, Canada, following SOT. Results Of 118 children, 33% experienced respiratory complications, increasing to 54% in heart transplant recipients. Chronic or recurrent cough with persistent chest x-ray changes was the most common clinical picture, and most infections were with non-opportunistic organisms typically found in otherwise healthy children. A history of respiratory illness prior to transplant was significantly associated with risk of post-transplant respiratory complications. 8% were diagnosed with bronchiectasis, which was more common in recipients of heart and kidney transplant. Bronchiectasis was associated with recurrent hospital admissions with lower respiratory tract infections, treatment of acute rejection episodes, and treatment with sirolimus. Interpretation Respiratory morbidity is common after pediatric SOT, and bronchiectasis rates were disproportionately high in this patient group. We hypothesise that this relates to recurrent infections resulting from iatrogenic immunosuppression. Direct pulmonary toxicity from immunosuppression drugs may also be contributory. A high index of suspicion for respiratory complications is needed following childhood SOT, particularly in those with a history of respiratory disease prior to transplant, experiencing recurrent or severe respiratory tract infections, or exposed to intensified immunosuppression.

Objectives: To examine the success rate of less invasive surfactant administration (LISA), to identify early predictive factors for the outcome of LISA and to compare neonatal outcomes between the LISA failure group and the group of infants who were succesfully treated with LISA. Design: A retrospective cohort study. Patients: Infants born at less than 33 weeks of gestation (n=158) and treated with LISA for respiratory distress syndrome. Results: LISA was successful in 86 cases (54.4%), 72 preterm infants (45.6%) needed additional surfactant therapy and/or mechanical ventilation in the first 72 hours. In a multivariate logistic regression analysis, six independent predictors were identified: core temperature at the time of admission (adjusted OR 3.56), suboptimal dose of surfactant (adjusted OR 0.254), elevated CRP (>10 mg/L) at 24 hours of life (adjusted OR 0.28), highest respiratory severity score during the first hour of life or at the time of LISA (adjusted OR 0.463), maternal age (adjusted OR 0.923) and birth weight (adjusted OR 1.003). The ROC curve created by using the identified factors indicates good predictive power with an area under the curve of 0.85. LISA failure was associated with a substantially higher risk of pneumothorax, bronchopulmonary dysplasia, intraventricular hemorrhage, severe retinopathy of prematurity, longer duration of mechanical ventilation and prolonged length of hospital stay. Conclusion: Failure of LISA is relatively frequent event in very preterm infants and is associated with adverse outcomes. Prevention of hypothermia during early stabilization and appropriate dosing of surfactant increase may LISA success rates and improve patient outcome.