A document by Luca Bonadies. Click on the document to view its contents.

Objective: The primary immunodeficiency syndromes of cytotoxic T lymphocyte-associated protein 4 (CTLA-4) haploinsufficiency and lipopolysaccharide-responsive and beige-like anchor protein (LRBA) deficiency present with multisystem immune dysregulation. The aim of this study was to characterize and compare the pulmonary manifestations of these two diseases. Methods: We retrospectively analyzed the pulmonary clinical, radiologic, and histopathologic characteristics of 6 patients with CTLA-4 haploinsufficiency and 4 patients with LRBA deficiency with pulmonary involvement followed at a large tertiary care center. Results: Chronic respiratory symptoms were more frequent in patients with LRBA deficiency versus CTLA-4 haploinsufficiency (4/4 versus 1/6). Cough was the most common respiratory symptom. Abnormalities in pulmonary exam and pulmonary function testing were more frequent in LRBA deficiency (4/4, 2/4) compared to CTLA-4 haploinsufficiency (1/6, 2/6). Chest CT findings included mediastinal lymphadenopathy (4/4 in LRBA deficiency versus 1/4 in CTLA-4 haploinsufficiency), pulmonary nodules (4/4, 3/4), ground-glass opacification (4/4, 3/4), and bronchiectasis (3/4, 1/4). Lymphocytic inflammation, concentrated bronchovasculocentrically and paraseptally, was observed in all patients who had lung biopsies (N=3 with LRBA deficiency; N=3 with CTLA-4 haploinsufficiency). Granulomas were seen in all patients with CTLA-4 haploinsufficiency and in no patients with LRBA deficiency. Conclusion: Despite phenotypic overlap amongst these diseases, LRBA deficiency demonstrated greater severity of pulmonary disease, indicated by respiratory symptoms, pulmonary exam, and intrathoracic radiologic findings. Lymphocytic inflammation is a key histologic feature of both of these disorders. Pediatric pulmonologists should suspect these disorders in the appropriate clinical, radiological, and pathological context to better diagnose and treat these patients.

Background Little attention has been given to the long-term respiratory outcomes of children with gastroschisis. The purpose of this study was to determine if gastroschisis survivors have more respiratory illnesses in their first 10 years-of-life compared to age-matched controls. Methods We performed a retrospective cohort study of all gastroschisis children born in Manitoba between 1991-2017. Gastroschisis cases were identified from a clinical database, and a date-of-birth matched control cohort was constructed from a population-based data repository. International Classification of Disease codes were used to compare the risk and frequency of respiratory diagnoses for children with gastroschisis to date-of-birth matched controls from 0-5 years-of-age and 5-10 years-of-age. Results The 0-5 years-of-age analysis included 117 gastroschisis cases and 1205 date-of-birth matched controls; children with gastroschisis had a higher risk of asthma (RR=1.46, 95%CI:1.03,2.55, p=0.029), acute bronchitis/bronchiolitis (RR=1.61, 95%CI:1.27,2.03, p<0.001), pneumonia (RR=1.99, 95%CI:1.45,2.72, p<0.001), viral pneumonia (RR=5.15, 95%CI:1.79,14.81, p=0.007), and pneumonia due to unspecified organism (RR=2.06, 95%CI:1.45,2.92, p<0.001). Gastroschisis children 0-5 years-of-age were also diagnosed more frequently with bronchitis/bronchiolitis (RR=2.14, 95%CI:1.79,2.57, p<0.001) and viral pneumonia (RR=8.10, 95%CI:3.79,17.31, p<0.001). The 5-10 years-of-age analysis included 73 cases and 738 controls; no difference in the risk of respiratory illness was found for gastroschisis cases and controls in this age group. However, gastroschisis cases were more frequently diagnosed with bacterial pneumonia (RR=3.03, 95%CI:1.67,5.51, p<0.001) and influenza (RR=3.03, 95%CI:1.67,5.51, p<0.001). Conclusion Our study shows that children with gastroschisis have an increased risk of asthma and respiratory infections compared to children without gastroschisis, especially in the first 5 years-of-life.

ABSTRACT Background: The goal of this study was to identify clinical features associated with abnormal infant pulmonary function tests (iPFTs), specifically functional residual capacity (FRC), in infants with cystic fibrosis (CF) diagnosed via newborn screen (NBS). We hypothesized that poor nutritional status in the first 6-12 months would be associated with increased FRC at 12-24 months. Methods: This study utilized a combination of retrospectively and prospectively collected data from ongoing research studies and iPFTs performed for clinical indications. Demographic and clinical features were obtained from the electronic medical record. Forced expiratory flows and volumes were obtained using the raised volume rapid thoracoabdominal technique (RVRTC) and FRC was measured via plethysmography. Results: A total of 45 CF NBS infants had iPFTs performed between 12-24 months. Mean forced vital capacity, forced expiratory volume in 0.5 second, and forced expiratory flows were all within normal limits. In contrast, the mean FRC z-score was 2.18 (95%CI=1.48, 2.88) and the mean respiratory rate (RR) z-score was 1.42 (95%CI=0.95, 1.89). There was no significant association between poor nutritional status and abnormal lung function. However, there was a significant association between higher RR and increased FRC, and a RR cutoff of 36 breaths/min resulted in 92% sensitivity to detect hyperinflation with 32% specificity. Conclusions: These results suggest that FRC is a more sensitive measure of early CF lung disease than RVRTC measurements and that RR may be a simple, non-invasive clinical marker to identify CF NBS infants with hyperinflation.

Malnutrition and swallowing disorders are common in Duchenne muscular dystrophy (DMD) patients. We assessed, in adult DMD with HMV (home mechanical ventilation) and cough assist device, its prevalence and the relationships with respiratory muscle strength and long-term respiratory prognosis. We reviewed the patients (n=117, age 18 to 39 years (median 24)), followed in a reference center, from 2006 to 2015, to obtain clinical baseline, nutritional status, vital capacity (VC), maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP). Median body mass index was low (15.6). Prevalence of malnutrition, swallowing disorders and gastrostomy were respectively 62, 34 and 11%. BMI and serum albumin level were significantly associated with MIP, MEP and VC. The 1/5-years cumulative incidences of respiratory events (pulmonary sepsis and acute respiratory distress) were respectively 20.7/44.5%. Using univariate analysis, predictive factors for respiratory events were swallowing disorders (p=0.001), transthyretinemia (p=0.034), MIP (p=0.039) and MEP (p=0.03) but not BMI or albuminemia. Using multivariate analysis, only swallowing disorders remained significantly associated with respiratory events (OR=4.2, IC95% 1.31-12.2, p=0.01). If prevalence of malnutrition and swallowing disorders are high in adult DMD on HMV, and respiratory muscles strength associated with serum albumin level and BMI, respiratory prognosis is mainly related to swallowing disorders.

Bronchoalveolar Lavage in Children: Still the Gold StandardShivanthan Shanthikumar1,2,3 and Sarath C Ranganathan1,2,3Respiratory and Sleep Medicine, Royal Children’s Hospital, Melbourne, AustraliaRespiratory Diseases, Murdoch Children’s Research Institute, Melbourne, AustraliaDepartment of Paediatrics, University of Melbourne, Melbourne, AustraliaCorresponding Author; Dr Shivanthan Shanthikumar; Respiratory Medicine, Royal Children’s Hospital, 50 Flemington Road, Parkville, VIC, 3052, Australia; [email protected]; The authors have no conflicts of interest to declareDear Editor,We read with great interest the recent article by Craven et al , entitled “High levels of inherent variability in microbiological assessment of bronchoalveolar lavage samples from children with persistent bacterial bronchitis and healthy controls .”1 In a small study of 18 children, funded by GlaxoSmithKline, the authors demonstrate variability in the results of bronchoalveolar lavage (BAL) collected from controls and children with protracted bacterial bronchitis (PBB). Specifically, they show that when the BAL was divided and sent to two laboratories the results were discordant in terms of both the organisms isolated and their relative abundance. From these data the authors draw conclusions which include questioning “assumptions about this procedure being the gold standard .” Whilst these data are of interest, there are significant limitations to their value especially when considering existing literature.One of the key findings of the study is the discordant results between laboratories. A lack of detail regarding the methods used at each site is a major limitation. It is recognised that laboratory processes can affect the yield of samples collected from patients with chronic airway infection, and the need for a consistent approach has led to disease specific consensus guidelines on this topic.2 The discordant results seen in the study could result from different laboratory handling of specimens, and hence the findings of this study could purely be explained by a difference in practice between two centres, not least of which was the transport of samples to the second laboratory in STGG. Molecular studies have identified that even media considered sterile can contain numerous organisms albeit in low densities.3 We note that it was laboratory 2 where additional bacteria were cultured from the BAL.Hare et al analysed BAL samples from 655 children collected and analysed at two different sites compared with 18 samples in the study of Craven et al .4 They compared bacterial pathogen load (control, negative, 102 colony forming units per ml (CFU/ml), 103 CFU/ml, 104 CFU/ml, 105 CFU/ml) and inflammatory markers to determine an appropriate definition for infection. They found that a bacterial pathogen load of ≥104 CFU/ml was associated with increased markers of inflammation and hence an appropriate threshold for defining infection. This was in keeping with previous studies.4 Whilst the authors contend the current paper does not support the use of ≥104 CFU/ml, given it only includes 13 children with PBB an explanation of the findings of Hareet al in their considerably larger study and other studies needs explanation.Another key finding of the study was the limited correlation between semiquantitative and quantitative methods of measuring bacterial pathogen load. Whilst there has not been direct comparison of different methods of determining bacterial pathogen load in PBB and other paediatric suppurative disorders, a large amount of data speaks to the validity of using a semiquantitative or qualitative approach. For instance, the previously discussed Hare et al study utilised a semiquantitative approach, and was able to clearly identify a threshold for lower airway infection that was associated with inflammation. In addition, the qualitative approach used by AREST CF (the long running study of CF patients cited in the article) to define infection is supported by the fact that this definition is associated with important clinical outcomes. For example, in a recent AREST-CF study analysing 1161 BAL from 265 children with CF, the presence of early life infection using the AREST-CF definition, was associated with future risk of structural lung disease severity.5Further, we have used molecular studies to assess the microbiome in CF and shown considerable agreement between pathogen-dominated microbiota and routine laboratory bacterial culture even though these samples were assessed by two different techniques, in two laboratories in different continents and analysed two decades apart in time.6Despite the data that contradicts the findings of their study, and while not discussed by the authors themselves, we do contend that use of both quantitative and semi-quantitative microbiologic cultures are likely problematic given that bacterial density is influenced by the dilution from the 0.9% saline used to lavage the target lobe. Dilution further depends on the volume of return retrieved on suctioning. The consensus has been that standardising for this dilution is not required but data supporting this are few.In summary, there are significant issues that limit the value of the key findings of the study by Craven et al . A large amount of published data in PBB and cystic fibrosis support the use of BAL as a biological specimen associated with important clinical outcomes. These studies have been conducted in multiple centres, over many years, and included many children. While the findings of Craven et alhighlight there can be inconsistencies in results, this potentially speaks to the methods used by the laboratories involved in handing the small number of samples. When these findings are compared to the large amount of evidence already generated, they should prompt evaluation of local practices and not just a reconsideration of whether BAL is the gold standard method of sampling the lower airway of children with suppurative lung disease. While we believe that BAL remains the gold standard for the detection of lower respiratory infection we do not believe it is a perfect test and its use and many limitations need to be considered and minimised.Therefore, we agree with the authors that interpretation of microbial culture results utilizing BAL samples can be challenging. However, we disagree that assumptions about this procedure being the “gold standard” fail to take into account its many limitations as despite these BAL remains the best test to detect endobronchial infection that is associated with lower respiratory inflammation especially in CF.

Background：Pulmonary arterial hypertension (PAH) is a rare disease in children, with significant mortality. Because of the limited research on pediatric PAH, firstly systematic review of related drugs is conducted，and then economic evaluation of PAH drug treatment programs is conducted, which to provide a reference for the choice of more cost-effective treatment options. Methods: The search includes electronic databases such as Pubmed, ScienceDirect, Embase. Through inclusion and exclusion criteria, screen high-quality randomized controlled trials. We used TreeAge Pro 2011 software to construct the markov model, that to simulate the total medical cost and quality-adjusted life years (QALYs), and to calculate the incremental cost-effectiveness ratio (ICER). Sensitivity analysis of transfer probability, utility and cost was carried out. Results: Incorporate two studies that meet the criteria, one compared the therapeutic effects of bosentan and placebo on pediatric PAH, the other compared therapeutic effects of sildenafil and placebo on pediatric PAH, both articles were of good quality. Compared with the sildenafil group (3.38QALYs and $161120.14),the QALY of the bosentan treatment group (3.33QALYs and $257411.29) was reduced by 0.05,and the cost increased by $96291.15.The estimated improvement to quality of life and reduced costs result in an estimate of economic dominance for sildenafil over bosentan. This dominant result persisted probabilistic analyses. Conclusions: Based on this model,a more cost-effective treatment drug for PAH in children is sildenafil.

Introduction: The 2017-2018 National Survey of Children’s Health estimates that 30 million (42%) US children have experienced at least one adverse childhood experience (ACE), including abuse, neglect, and household dysfunction. ACEs negatively impact long-term health, and there has been no study of ACEs in cystic fibrosis (CF). We assessed willingness to disclose ACEs experienced by children with CF by surveying their parents and adults with CF. Methods: We anonymously surveyed parents of children with CF and adults with CF at the Northwestern University/ Lurie Children’s CF Center to determine their willingness to disclose ACEs. Results: The survey was completed by 46/157 (29%) parents and 36/105 (34%) adults with CF. Few parents (22%) and adults (17%) were willing to discuss most or all specific ACEs, more were willing to disclose the number of ACEs experienced in a category (57% parents, 47% adults), and the majority were willing to participate in anonymous research about ACEs (76% parents, 67% adults). Most parents (63%) and adults (50%) would prefer to have ACEs screened separately from their CF appointment, and most parents (63%) and adults (56%) wanted to learn more about ACEs from a member of their care team. Conclusions: Participants preferred to disclose the number of categorical ACEs rather than specific ACEs and most were open to participating in anonymous ACEs research. More research is needed before widespread adoption of ACE screening in CF.

Rationale: Bronchopulmonary dysplasia (BPD) is a major complication of premature birth and the most common cause of chronic lung disease in infancy. Previous studies have shown that children with a history of BPD have impaired lung function in childhood compared to their term counterparts. However, little is known about potential modifiable factors that alter lung function trajectories and subsequent respiratory morbidity in this population. Objectives: To identify potential modifiable risk factors for the development of impaired lung function in patients with a history of prematurity and BPD. Methods: Growth parameters (birth, 2yo, 6yo) and pulmonary function testing (6yo) were retrospectively reviewed for subjects (n=598) recruited from an outpatient BPD clinic who were born ≤36 weeks gestation and were ≥5 years of age. Results: Of the 598 recruited subjects, 88 (14.7%) performed adequate pulmonary function testing at approximately six years of age. The mean FEV1% predicted was 84.5% with lower values associated with lower median household income, Nissen fundoplication, and higher weight percentiles at 2yo. The mean FVC % predicted was 94.2% with lower values associated with higher amounts of oxygen required at time of initial hospital discharge, Nissen fundoplication, and higher weight percentiles at 2yo. Conclusions: Our study found that children with BPD have different long-term pulmonary trajectories than full-term controls. Supplemental oxygen, lower income, and Nissen fundoplication at discharge were associated with lower lung function at 6 years of age. Prospective studies should focus on modifiable risk factors that could minimize the impact of BPD on later lung function.

Background: Early hypocapnia in preterm infants is associated with intraventricular haemorrhage (IVH) and bronchopulmonary dysplasia (BPD). Volume targeted ventilation (VTV) has been shown to reduce hypocapnia in moderately preterm infants. Less is known of VTV in infants born at <26 weeks gestational age (wGA). Objectives: Our aim was to investigate the short- and long-term benefits of early VTV as compared to assist-control ventilation (ACV) in extremely preterm infants on incidence of hypocapnia, days on ventilatory support, IVH and BPD. Study Design: A retrospective observational study of 104 infants born at 22-25 wGA (24+0±1+1wGA; birth weight 619±146g), ventilated with either VTV (n=44) or ACV (n=60) on their first day of life. Ventilatory data and blood gases were collected at admission and every fourth hour during the first day of life, together with perinatal characteristics and outcomes. Results: Positive inspiratory pressure (PIP) was lower in the VTV-group than in the ACV-group during the first 20 hours of life (p<0.05), without any difference in end-expiratory pressure, respiratory rate or FiO2. Incidence of hypocapnia (PaCO2<4.5kPa) was lower with VTV than ACV during the first day of life (32% vs 62%; p<0.01). Infants in the VTV-group were more frequently extubated at 24 hours (30% vs 13%; p<0.05). IVH grade ≥3, BPD and time on mechanical ventilation did not differ between the groups. Conclusions: VTV is safe to apply in infants born at <26 wGA and was observed to have lower incidence of hypocapnia compared to infants ventilated by ACV, without any differences in outcomes.

Spirometry, a gold standard technique for measuring lung functions, has been restricted to a select cohort of patients in current COVID-19 pandemic due to the enhanced risk of disease dissemination. To monitor pulmonary functions in various obstructive (e.g., asthma) and restrictive diseases (e.g., COVID-19 pneumonia) on in- and out-patients serially, there is an urgent requirement of an alternate reliable test. Impulse Oscillometry (IOS) measures lung functions by working at tidal volumes and thus reduces the risk of potential aerosol generation. Feasibility of IOS in smaller children and its ability to detect parenchymal and peripheral airway involvement are other advantages over conventional spirometry. IOS could be a potential solution to periodically monitor lung functions in current pandemic situation to keep a check on diseases affecting lung functionality.

Background: The Coronavirus Disease 2019 (COVID-19) is causing of the new global pandemic and is responsible for millions of infections and thousands of deaths in the world. The lung ultrasound is a non-invasive and easily repeatable tool and can be carried out by the pediatrician at the bedside of children with a consequent reduction in the risk of transmission of the virus. Objective: To determine whether the lung ultrasound is a useful tool in identifying the signs of lung involvement in children with COVID-19 and whether can monitor the course of the disease. Methods: The study was made in the emergency department in a tertiary level pediatric hospital. All patients with swab-confirmed COVID-19 infection were subjected a lung ultrasound within 6 hours from admission and after 96 hours. Results: Among a total of 30 children, 18 (60%) were males, 4 reported exertional dyspnea and only 1 chest pain. The mean oxygen saturation was 98.8 ± 1.0 % in ambient air in emergency department and no patient needed oxygen therapy during hospitalization. After 96 hours we had observed ultrasound abnormality al the lung ultrasound only in 20% of the children. We found a statistically significant reduction in pleural irregularities (30% vs 16.7; p: .001) and in B lines (50% vs 20%; p: .008). Conclusions: lung ultrasound is safe and useful tool in detecting lung involvement in children with COVID-19 and in monitoring these patients during the course of disease.

Background: Non-invasive ventilation (NIV) is a first-line therapy for sleep-related breathing disorders and chronic respiratory insufficiency. Evidence about predictors that may impact long-term NIV outcomes, however, is scarce. The aim of this study is to determine demographic, clinical, and technology-related predictors of long-term NIV outcomes. Methods: A ten-year multi-centred retrospective review of children started on long-term continuous or bilevel positive airway pressure (CPAP, BPAP) in Alberta. Demographic, technology-related, and longitudinal clinical data was collected. Long-term outcomes examined included ongoing NIV use, discontinuation due to improvement in underlying condition, switch to invasive mechanical ventilation (IMV) or death, patient/family therapy declination, transfer of services, and hospital admissions. Results: 622 children were included. Both younger age and CPAP use predicted higher likelihood for NIV discontinuation due to improvement in underlying conditions. Children with upper airway disorders or bronchopulmonary dysplasia were less likely to require NIV continuation while presence of central nervous system (CNS) disorders resulted in higher likelihood of hospitalizations and switch to IMV or death. The presence of obesity/metabolic syndrome and early NIV-associated complications predicted higher risk for NIV declination. Children with more co-morbidities or use of additional therapies required more hospitalizations and the latter also predicted higher risk to be switched to IMV or death. Conclusions: Demographic, clinical data, and NIV type impact long-term NIV outcomes and need to be considered during the initial discussions about therapy expectations with families. Knowledge of factors that may impact long-term NIV outcomes might help to better monitor at-risk patients and minimize adverse outcomes.

Objective Long-term respiratory consequences of bronchopulmonary dysplasia (BPD) in preterm infants born in the post-surfactant era (“new” BPD) remain partially unknown. The present study aimed to evaluate respiratory outcomes of “new” BPD in adolescents who were born preterm. Methods This multicenter, cross-sectional study included 286 adolescents born between 2003 and 2005 (mean age: 14.2 years); among them, 184 and 102 were born extremely preterm (EP) (< 28 weeks’ gestation) and moderate-late preterm (32 to < 37 weeks’ gestation), respectively. Among EP adolescents, 92 had BPD, and 92 did not. All participants underwent lung function tests, skin prick testing, and questionnaires on asthma symptoms and quality of life. Results EP adolescents with BPD had significantly lower forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC), FEV1/FVC ratio, and forced expiratory flow between 25%–75% of FVC than other included adolescents. FEV1/FVC ratios were below the lower limit of normal (z-score < −1.645) in 30.4% of EP adolescents with BPD, 13.0% of EP adolescents without BPD, and 11.8% of adolescents who were born moderate-late preterm. Bronchodilator response and air-trapping were significantly higher in BPD adolescents than in other adolescents. Diffusion capacity was significantly lower in EP adolescents than in moderate-late preterm adolescents. Asthma symptoms and quality of life scores were similar among groups. Conclusion EP adolescents with “new” BPD had poorer pulmonary function than EP adolescents without BPD or moderate-late preterm adolescents. Further studies are needed to determine whether “new” BPD is associated with early-onset chronic obstructive pulmonary disease in adulthood.

Objective: Children with Down syndrome (DS) have an increased prevalence of obstructive sleep apnoea (OSA). Non-invasive ventilation (NIV) is a common modality of OSA treatment in this cohort. This study aimed to measure adherence and efficiency of NIV delivery in children with DS. Study design: This was a retrospective cohort study involving 106 children with confirmed OSA and home NIV with downloadable data capacity. Children were divided into DS (n=44) and non-DS cohorts (n=62). Adherence, clinical outcomes apnoea-hypopnoea index (AHI), positive airway pressure delivery and leakage were recorded and compared between DS and non-DS cohorts and within the DS cohort based on past surgical history. Results: Significantly greater NIV usage was observed in the DS cohort, they showed more consistent use with an increased percentage of days used relative to their non-DS counterparts (78.95 ± 2.26 versus 72.11 ± 2.14, p=0.031). However, despite greater usage, poorer clinical outcomes in the form of increased AHI (p=0.0493) was observed in the DS cohort, where significantly greater leakage was also shown 41.00 ± 1.61L/min versus 36.52 ± 1.18L/min (p=0.022). Twenty children with DS had prior cardiac surgery; compliance across all parameters was significantly reduced relative to those without. Conclusions: These data confirm that satisfactory NIV adherence is achievable in children with DS. However, we have identified excessive system leak at the machine-patient interface as a factor, which could undermine NIV efficacy in children with DS.

Editorial: Long-term respiratory complications of OA-TOF: The need for better data and a more specialised multidisciplinary approach

Title: Congenital Insensitivity to Pain, with Impaired Pulmonary Functions, and Diminished Cough Reflex.

Background: Depression and anxiety symptoms in patients with cystic fibrosis (CF) and their caregivers are 2-3 times higher than in the normal population. This study aims to evaluate the frequency and severity of depression and anxiety symptoms and to determine possible risk factors in CF patients and their mother and/or fathers at Marmara University CF center. Methods: The study included 132 CF patients who were followed up at our CF center. Patient Health Questionnaire (PHQ-9) and the Generalized Anxiety Disorder Questionnaire (GAD-7) were used to screen depression and anxiety. The questionnaires were completed by 50 CF patients (aged 12 - 17 years) and 132 parents of patients (aged 0-17 years). Results: While moderate to severe depression were seen in 25.5% of patients, 33.7% of mothers and 14.6% of fathers; moderate to severe anxiety were present in 17.6%, 21.8% and 8.5%, respectively. None of the demographic characteristics was identified as a predictor of depression or anxiety. GAD-7 scores have shown a higher incidence of anxiety in mothers of patients with chronic methicillin-resistant Staphylococcus Aureus (p = 0.034). Additionally, hospitalization in the last 12 months was statistically significantly higher in PHQ-9 scores of fathers (p = 0.043). Analysis of patients’ adherence to medical treatment and airway clearance showed higher depression and anxiety in mothers of the non-adherent group (p = 0.002). Conclusion: Depression and anxiety were common in CF patients and their parents. These results illustrate the importance of depression/anxiety screening and psychosocial support for the CF patient and their parents.