Depression, anxiety, and resilience during COVID-19 in Dutch patients with cystic fibrosis or primary ciliary dyskinesia and their caregivers.

BACKGROUND: The COVID-19 pandemic has spread across the world, leading to government measures associated with a negative impact on mental health. The aim of this study was to evaluate the impact of the pandemic on depression, anxiety and resilience in Dutch people with cystic fibrosis (PwCF) or primary ciliary dyskinesia (PwPCD) and their caregivers. METHODS: Adolescents (12-17 years) and caregivers of children (0-17 years) with CF, and adolescents, adults and caregivers of children with PCD completed questionnaires on depression, anxiety and resilience between September 2020 and February 2021. The psychosocial impact of COVID-19 was measured by the Exposure and Family Impact Survey (CEFIS) Part 2. Mixed model analyses compared depression and anxiety results to participants' prepandemic scores. RESULTS: One hundred ten participants (10 PwCF, 31 PwPCD, 52 CF caregivers, 17 PCD caregivers) completed questionnaires during the pandemic. Prepandemic outcomes were available for 87 participants. The prevalence of symptoms of depression and anxiety (PHQ-9 or GAD-7 scores ≥5) in PwCF and PwPCD and their caregivers before and during the pandemic was high, with an increase in depression in PwCF (2.75; 95% confidence interval: 0.82-4.68) and increase in anxiety in CF caregivers (1.03; 0.09-1.96) during the pandemic. Resilience was within the normal range for all groups, CEFIS scores corresponded to a low to normal impact. CONCLUSION: PwCF and PwPCD and their caregivers were at risk of elevated depression and anxiety symptoms both before and during the pandemic, which emphasizes the importance of mental health screening and psychological care in CF and PCD.

Low incidence and severity of SARS-CoV-2 infections in people with PCD

Background: There is little data on SARS-CoV-2 infections in people with rare diseases. We studied incidence and severity of SARS-CoV-2 in people with primary ciliary dyskinesia (PCD). Method(s): We used data from COVID-PCD, an international participatory study including people with PCD, which started recruitment on 30.05.2020. Participants completed weekly online questionnaires on SARS-CoV-2 infections and symptoms. We studied severity of infections reported at baseline or during follow-up while we calculated incidence rates including only infections reported during follow-up. We used Cox proportional hazard regression to study predictors of getting infected. Result(s): By January 23, 2022, 726 people participated (40% male, median age 27 years;range 0-85). 90% of persons above 14 years have been vaccinated against COVID-19 and most (93%) wore facemasks in public. Only 62 (8.5%) had a confirmed SARS-CoV-2 infection. Severity of disease was mainly mild;11 (18%) were asymptomatic, 47 (76%) had symptoms among whom 4 (6%) were hospitalized (none in the ICU, nobody died). Severity of disease was not associated with age, sex, co-morbidity or vaccination. We had follow-up data from 651 (90%). During 633 person-years of follow-up (median 59 weeks per person), 43 incident SARS-CoV-2 infections were reported (incidence rate 6.9 per 100 person years;95% CI 5.2-9.2). Children (0-14 years) had a higher risk of infection (hazard ratio 3.0;95% CI 1.3-6.8) compared to 15-49 year-olds. Conclusion(s): SARS-CoV-2 incidence rates remained low and severity mainly mild in people with PCD, probably reflecting high vaccination rate and personal protective behaviour.

Reactivation of Tuberculosis and COVID-19 in Pediatric Patients

Objective Mycobacterium tuberculosis is an immobile aerobic bacillus that causes tuberculosis (TB) disease. We aimed to evaluate the association between coronavirus disease 2019 (COVID-19), COVID-19-related drugs, TB reactivation, and TB incidence during the pandemic. Methods Eight patients who were diagnosed as having TB in Meram Medical Faculty, Necmettin Erbakan University between March 1, 2020, and December 31, 2021, at the beginning of the pandemic, were enrolled in this study. The presence of COVID-19 infection was confirmed using COVID-19 antibody tests and the patients' COVID-19 history. We evaluated the demographic data, laboratory findings, imaging tests, and pathology results of all patients. Results We checked all our patients with TB using COVID-19 antibodies (immunoglobulin [Ig]G + IgM) or polymerase chain reaction. Seven of the eight patients were female (87.5%). The median age was 16 years. Family screening of all patients was negative, and they had bacillus Calmette-Guerin vaccine scars. Two patients had chronic diseases. One was diagnosed as having primary ciliary dyskinesia in our department (patient no. 8) and the second was under follow-up by the rheumatology department with a diagnosis of juvenile idiopathic rheumatoid arthritis. Conclusion There has been an increase in the incidence of TB in children, especially in adolescents, during the pandemic period. This may be due to the pathogenic structure of the COVID-19 virus with an unknown mechanism. In addition, lifestyle changes and changes in health care policies during the pandemic may have caused this. Further research should be performed on this topic.Copyright © 2023 Authors. All rights reserved.

Sleep disturbances in children with cystic fibrosis and primary ciliary dyskinesia at the beginning and in the first year of COVID-19 pandemic

Introduction: During the COVID-19 pandemic, the lifestyle and sleep habits of children with chronic lung disease have changed. Aims and objectives: To evaluate the differences in sleep habits in the first year of the pandemic in children with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD). Method(s): Primary caregivers of children with CF and PCD aged 3-16 years who were evaluated for sleep habits at the beginning of the pandemic were re-evaluated at the end of the first year of the pandemic. The Sleep Disturbance Scale for Children (SDSC) was used and questions were asked about sleep habits and weight changes during the first year of the pandemic. Result(s): Primary caregivers of 31 children with CF and 14 children with PCD were included in the study. The median age of the children were 10.0 (7.5-12.0) years, 42.2% of the children were female. The mean BMI of children with CF was 15.8+/-1.8, and 20.0+/-3.5 in children with PCD (p:0.001). The mean daily screen time was 2h (1-3) at the beginning and 5h (4-6) in the first year of the pandemic among children with CF, 2h (1-2.2) at the beginning and 5h (4.7-6) in the first year of the pandemic among children with PCD (p<0.001, p:0.001, respectively). There were no differences in terms of disorders of initiating and maintaining sleep, sleep breathing disorders, disorders of arousal, sleep-wake transition disorders, disorders of excessive somnolence, sleep hyperhidrosis scores between two groups during first year of pandemic. Conclusion(s): While daily screen time increased in children with CF and PCD, sleep disturbances and changes in daily habits continued in the first year of the pandemic.

Diagnostic testing in people with primary ciliary dyskinesia around the world: where do we stand?

Background: In the past, few patients with primary ciliary dyskinesia (PCD) were diagnosed with the test combination recommended by guidelines (nasal nitric oxide (nNO), genetic testing, and biopsy for electron or video microscopy) [Halbeisen, ERJ, 2019]. In a large international participatory study of people with PCD, we assessed the current situation. Method(s): We used data from COVID-PCD, an international study of people with PCD, who participated between 2020 and 2022. Participants described their diagnostic tests in an online questionnaire, and we used logistic regression to identify explanatory factors. Result(s): 728 participated (median age 27 years, IQR: 12-43;60% female). Among them, 92% reported that any diagnostic testing had been done: 49% nNO, 59% genetics, and 75% biopsy for electron or video microscopy. Most did not know whether the sample had been analysed with TEM or video microscopy. Biopsy was most frequent in all countries except in North America where genetic testing predominated. Only 36% of participants reported all three tests. This proportion was highest in Germany (61%) and lowest in Australia (19%). Recently diagnosed patients reported more tests (nNO OR 1.7, 95%CI 1.1-2.6, genetics OR 4.5, 95%CI 2.9-6.9), and those with situs inversus less (nNO OR 0.5, 95%CI 0.3-0.7, biopsy OR 0.4, 95%CI 0.3-0.7, genetics OR 0.7, 95%CI 0.5-0.97). Conclusion(s): Diagnostic testing in people with PCD differed by country and few reported having undergone all recommended tests. Patients diagnosed long ago should be recalled for supplementary testing to improve diagnostic characterisation as a prerequisite for personalised medicine.

Diagnostic testing in people with primary ciliary dyskinesia around the world: where do we stand?

Introduction In the past, only few patients with primary ciliary dyskinesia (PCD) were diagnosed with the test combination recommended by guidelines (nasal nitric oxide (nNO), genetic testing, and biopsy for electron or video microscopy) [Halbeisen, ERJ, 2019]. In a large international participatory study of people with PCD, we assessed the current situation. Methods We used data from COVID-PCD, an international study of people with PCD, who participated between 2020 and 2022. Participants described their diagnostic tests in an online questionnaire, and we used logistic regression to identify explanatory factors. Results 728 participated (median age 27 years, IQR: 12-43;60% female). Among them, 92% reported that any diagnostic testing had been done: 49% nNO, 59% genetics, and 75% biopsy for electron or video microscopy. Most did not know whether the biopsy sample had been analysed with transmission electron microscopy or video microscopy. Biopsy was most frequent in all countries except in North America where genetic testing predominated. Only 36% of participants reported all three tests. This proportion was highest in Germany (61%) and lowest in Australia (19%). Recently diagnosed patients reported more tests (nNO OR 1.7, 95%CI 1.1-2.6, genetics OR 4.5, 95%CI 2.9-6.9), and those with situs inversus less (nNO OR 0.5, 95%CI 0.3-0.7, biopsy OR 0.4, 95%CI 0.3-0.7, genetics OR 0.7, 95%CI 0.5-0.97). Conclusion Diagnostic testing in people with PCD differed by country and few reported having undergone all recommended tests. Patients diagnosed long ago should be recalled for supplementary testing to improve diagnostic characterisation as a prerequisite for personalised medicine.

Incidence and severity of SARS-CoV-2 infections in people with primary ciliary dyskinesia

Introduction After two years of COVID-19 pandemic, there is still little data on incidence and severity of SARS-CoV-2 infections in people with primary ciliary dyskinesia (PCD). We aimed to study incidence of SARS-CoV-2, severity of disease vaccination status, and social contact behaviour in people with PCD and study factors associated with risk of infection and risk of severe disease. Method COVID-PCD is an international participatory cohort study which started recruitment on 30.05.2020. Participants completed weekly online questionnaires on SARS-CoV-2 infections and symptoms. We studied severity of infections reported at baseline or during follow-up while we calculated incidence rates including only infections reported during followup. We studied factors associated with risk of infection using Poisson regression. Results By 10.05.2022, 728 people with PCD participated (40% male, median age 27 years;range 0-85). 90% of persons above 14 years were vaccinated against COVID-19 and most (93%) wore facemasks in public. In total, 87 (11.7%) reported a SARS-CoV-2 infection. We had follow-up data from 664 persons (90%) and during 716 person-years (median 61 weeks per person), 62 incident SARS-CoV-2 infections were reported (incidence rate 8.7 per 100 person years;95%CI 6.8- 11). Risk of infection was lower in adults compared to children (IRR: 0.39, 95%CI 0.20-0.77), higher in the United Kingdom compared to other countries (IRR: 1.85, 95%CI 1.08-3.19), and higher between Sep 2021 - May 2022 compared to MarNov 2020 (IRR: 1.20, 95%CI 1.05-1.38). Severity of disease was mainly mild;12 (14%) were asymptomatic, 75 (86%) had symptoms among whom 4 were hospitalized (none in the ICU, nobody died). Severity was not associated with age sex, co-morbidity, or vaccination. Conclusion SARS-CoV-2 incidence rates remained low and severity mainly mild in people with PCD, probably reflecting high vaccination rate and personal protective behaviour.

43 Jahrestagung der Gesellschaft fur Padiatrische Pneumologie

The proceedings contain 85 papers. The topics discussed include: T2-high asthma across all ages - comparative analysis in children and adults from the ALLIANCE cohort;targeting IL-6 to prevent vascular and bronchial remodeling in an experimental model of bronchopulmonary dysplasia;diagnostic testing in people with primary ciliary dyskinesia around the world: where do we stand?;effects of elexacaftor/tezacaftor/ivacaftor in children with cystic fibrosis: a comprehensive assessment using spirometry, lung-clearance-index, functional and structural lung MRI;cough phenotypes in children: findings from the Swiss Pediatric Airway Cohort;pleuropneumonia caused byNocardia cyriacigeorgica in a 14-year-old girl with PCD;in severe juvenile-onset recurrent respiratory papillomatosis of a 10-year-old, systemic bevacizumab is highly effective and well tolerated;and impact of COVID-19 related alterations in circulating respiratory viruses on children and adult patients with recurrent wheeze and asthma.

The Impact on Parents of Diagnosing PCD in Young Children.

Primary ciliary dyskinesia (PCD) is an incurable, rare, inherited, chronic condition. Treatment includes the regular clearing of airway mucus, aggressive treatment of infections and management of hearing loss. Caregiver burden has not been explored, hence we interviewed 18 mothers and 6 fathers of children under 6 years to understand the impact of diagnostic testing and implications of a positive diagnosis. Interviews were transcribed and thematically analysed and five key themes were identified. These included the parents' experiences following child's diagnosis, impact of child's treatment regimen on parent, impact of child's health status on parent, parent's coping strategies, and parental concerns for the future. Parents described their diagnostic journey, with the findings revealing how a lack of awareness among clinicians of the PCD symptom pattern can lead to a delayed diagnosis. Parents discussed the emotional and practical impact of a PCD diagnosis and the coping strategies employed to deal with challenges arising following a diagnosis. Parents use a variety of different lifestyle changes to accommodate their child's treatment regimen and to cope with disruptive life events such as the COVID-19 pandemic. This study provides valuable insights into parental adjustment and adaptation to a PCD diagnosis and management regimen. Going forward, this research highlights the need for integrated social care for PCD patients and their families.

RESPIRATORY DISTRESS IN A NEONATE - WHEN BOARDS MEET REAL LIFE

Case Report A male infant is born at 37w to a 34-year-old G3P2 mother by vaginal delivery after an uncomplicated pregnancy. Prenatal screens are negative. The patient had a birth weight of 2,620 g, with Apgar scores of 9 and 9. On day 2 after birth, had increased work of breathing which prompted transfer to a level II NICU for further management. On arrival to the unit, the infant is tachypneic with mild chest wall retractions and thick nasal secretions. A CBC and blood culture were collected and empiric antibiotic therapy was started. Respiratory viral panel and COVID test are negative. A chest radiograph shows a middle lobe opacity concerning for pneumonia (figure 1). His clinical status failed to improve and on day 4 after birth, supplemental oxygen was provided. The primary team consulted ENT and Pulmonology services. Flexible laryngoscopy showed a normal anatomy. Pulmonology recommended transferring to our NICU for a chest CT with bronchoscopy. Our differential diagnosis for this neonate with respiratory distress that fails to improve over time or with antibiotics was broad, but further testing revealed this infant's condition. A CBC, CRP and a blood gas were collected on admission and were normal. ID service was consulted. A Chest CT showed bilateral atelectasis. Bronchoscopy showed a normal anatomy. Bronchoalveolar lavage was sent. Umbilicus swab was positive for MRSA, nasal wash/sputum culture/bronchoalveolar fluid also grew moderate S. aureus. Nasal ciliary biopsy sent for electron microscopy. Positive umbilicus and nasal swab, and subsequently BAL for MRSA led to a diagnosis of MRSA neonatal rhinitis. Therapy with IV vancomycin was initiated and later changed to oral clindamycin to complete a total of 14 days of therapy. The neonate was weaned off oxygen support on day 11. His clinical symptoms improved. He was discharged on oral clindamycin with follow up appointments with pulmonology and ID clinics. His ciliary biopsy showed absence of outer and inner dynein arms, compatible with the diagnosis of primary ciliary dyskinesia (PCD) (figure 2). Genetic testing for PCD showed mutations in the DNAAF1 and CCDC40 genes. This neonate was diagnosed with primary ciliary dyskinesia (PCD) but his presentation at birth was nonspecific and the differential diagnosis was broad. There is no gold standard diagnostic test for PCD and high clinical suspicion is important. Since it is most likely an AR inheritance, screening of family members is essential. Initial management of neonates may include measures that manage the respiratory distress, airway clearance to prevent respiratory infections and treat bacterial infections. Chest physiotherapy may help if recurrent atelectasis. Flexible bronchoscopy and bronchoalveolar lavage may help both to diagnose and treat the underlying infection. Antibiotic therapy based on organism growth for exacerbations may prevent development of bronchiectasis. (Figure Presented).

Sleep quality in children with primary ciliary dyskinesia (PCD)

Introduction: PCD is a rare, progressive disease resulting in upper and lower respiratory tract manifestations that increase the risk of sleep disordered breathing. This study is the first to characterize sleep quality in Australian children with PCD and examine it is relationship to mood and health-related quality of life (HrQOL). Methods: Clinically stable children with PCD (1-18 years of age) were recruited. Subjective sleep quality was assessed with the SDSC, PDSS and OSA-18 questionnaires. HrQOL and depressive symptoms were assessed via age-appropriate QOL-PCD and CDI questionnaires. Demographic data including passive smoke exposure was recorded. Pulmonary function testing and ENT assessments were performed. Children underwent overnight polysomnography including transcutaneous CO2 and video monitoring (50% studies complete due to Covid restrictions). Results: Twenty-two participants (10 female) aged 8.1 ± 5.1 (mean ± SD) years were recruited. Mean(±SD) FEV1 was 78.2 ± 21.2%. Ninety-two percent of children assessed were diagnosed with chronic rhinosinusitis and 35% exposed to regular passive cigarette smoke. Polysomnography identified sleep fragmentation in 90% of studies and one case of mild obstructive sleep apnoea. Subjective sleep questionnaires revealed 76% of parents and 50% of children reported clinically significant scores indicating sleep disturbance and excessive daytime somnolence respectively. Twenty-seven percent of parents reported a moderate-severe impact of sleep disturbance on QOL. Forty-six percent of children had elevated depression scores. HrQOL and mood scores were correlated with poor subjective sleep quality. Children exposed to passive cigarette smoke had poorer subjective sleep quality and lower HrQOL and mood. Conclusion: Even in periods of clinical stability, children with PCD exhibit poor sleep quality and excessive daytime sleepiness and this is associated with lower mood and HrQOL. Based on these findings, we recommend routine screening for mental health and symptoms of sleep disturbance in children and adolescents with PCD. The continued education of children and families regarding the effects of cigarette smoke is advised.

Chronic lung disease following covid-19 in an infant

Case Report Chronic respiratory sequelae are well documented in adults after COVID-19 infection, however, in young children and infants, evidence is still evolving. Here we report an infant with significant chronic respiratory complications after COVID-19. Case Report A 10 month old female with no significant past medical history was admitted to the PICU secondary to hypoxemia, respiratory distress, and respiratory failure following COVID-19 infection in January 2021. She was also positive for Rhinovirus and Enterovirus. CXR displayed worsening bilateral alveolar infiltrates, and she developed subsequent pneumothorax requiring a chest tube. Apart from mechanical ventilation, she received supportive treatment and broad spectrum antibiotics. Cardiac echocardiogram revealed pulmonary hypertension, PFO, and PDA. Due to worsening respiratory status and hypoxemia, she received bronchodilators, inhaled nitric oxide, sildenafil, steroids, and magnesium. After 3 weeks, her respiratory status improved and she was discharged. The patient required another hospitalization in March and an ER visit in April for persistent cough and shortness of breath. After evaluation by pulmonology, she began inhaled steroids and airway clearance treatments including chest physical therapy, hypertonic saline, and bronchodilators. Further workup ruled out cystic fibrosis, primary ciliary dyskinesia, and immunodeficiency. Chest CT showed diffuse bilateral patchy airspace opacities representing atelectasis and scarring. Despite a short period of improvement, the patient was hospitalized for respiratory distress in June, where she was hypoxemic and diagnosed with pneumonia. She required repeated outpatient visits to the PCP for persistent respiratory symptoms. PDA closure was performed in September. The patient continues to have persistent respiratory symptoms addressed with outpatient respiratory treatment regimen. Conclusion As we have ruled out other underlying causes, the patient's chronic lung disease and persistent respiratory symptoms occurred most probable secondary to COVID-19. This case report highlights the importance of monitoring respiratory symptoms in pediatric patients with severe COVID-19 infection for early identification of chronic respiratory sequelae.

Variations in infection control practices suggest a need for guidelines in primary ciliary dyskinesia patient care.

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 impacts 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, and how practices have been influenced by the COVID-19 pandemic. The response rate was 87.0%. Before the COVID-19 pandemic, glove, gown, and mask use were 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 the 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.

Facemask Usage Among People With Primary Ciliary Dyskinesia During the COVID-19 Pandemic: A Participatory Project.

Objectives: Primary ciliary dyskinesia (PCD) is a rare genetic disease that causes recurrent respiratory infections. People with PCD may be at high risk of severe COVID-19 and protection against SARS-CoV-2 is therefore important. We studied facemask usage and problems reported in relation with their use among people with PCD. Methods: We used data from COVID-PCD, an international observational cohort study. A questionnaire was e-mailed to participants in October 2020 that asked about facemask usage. Results: In total, 282 participants from 27 countries were included (Median age 32 years; 63% female). In total, 252 (89%) wore facemasks everywhere in public, 13 (5%) wore facemasks in most places, and 17 (6%) did not wear facemasks in public. Half of the participants reported that it was uncomfortable to wear facemasks because of runny nose, cough, or difficulty breathing. Participants less often wore facemasks when there was no national requirement. Conclusion: Most people with PCD wore facemasks despite frequent respiratory problems related to their use. Facemask usage was most frequent in countries with a national requirement emphasizing the importance of nationwide policies mandating facemasks.

COVID-19 Vaccinations: Perceptions and Behaviours in People with Primary Ciliary Dyskinesia.

Primary ciliary dyskinesia (PCD) is a rare genetic disease that causes recurrent respiratory infections. People with PCD may be at higher risk of severe coronavirus disease 2019 (COVID-19), and therefore vaccination against severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is important. We studied vaccination willingness, speed of vaccination uptake, side effects, and changes in social contact behaviour after vaccination in people with PCD. We used data from COVID-PCD, an international participatory cohort study. A COVID-19 vaccination questionnaire was emailed to participants in May 2021 and 423 participants from 31 countries replied (median age: 30 years, range 1-85 years; 261 (62%) female). Vaccination uptake and willingness were high, with 273 of 287 adults (96%) being vaccinated or willing to be in June 2021; only 4% were hesitant. The most common reason for hesitancy was fear of side effects, reported by 88%. Mild side effects were common, but no participant reported severe side effects. Half of the participants changed their social behaviour after vaccination by seeing friends and family more often. The high vaccination willingness in the study population might reflect the extraordinary effort taken by PCD support groups to inform people about COVID-19 vaccination. Clear and specific information and involvement of representatives is important for high vaccine uptake.

Sleep disturbances in children with cystic fibrosis, primary ciliary dyskinesia and typically developing children during COVID-19 pandemic.

AIM: We aimed to investigate sleep disturbances in children with cystic fibrosis (CF) and primary ciliary dyskinesia (PCD) and typically developing (TD) children during the COVID-19 pandemic. METHODS: Primary care givers of children with CF and PCD aged 3-16 years were asked to enrol in the study. Primary care givers of TD children were included as control group. The Sleep Disturbance Scale for Children (SDSC) was used, and questions related to sleep habits during the pandemic were asked. Results of the three groups were compared. RESULTS: Primary care givers of 33 children with CF, 16 children with PCD and 66 TD children were included in the study. There were no differences in terms of age and gender between the three groups. Changes in sleep patterns during the pandemic were more common among TD children and their families, with 75% of the children and 80% of their families sleeping later than before. The sleep initiation and maintenance disorder scores were higher in TD children (P = 0.001), whereas the sleep breathing disorder scores were higher in children with PCD (P = 0.001), and the sleep hyperhidrosis scores were higher in children with CF and PCD (P = 0.011). No relationships were found between sleep parameters and clinical findings of children with lung disease. CONCLUSIONS: Children's sleep habits have changed during the pandemic. Children with chronic lung diseases and even TD children may experience sleep disturbances during this period.

Is Quarantine for COVID-19 Pandemic Associated with Psychological Burden in Primary Ciliary Dyskinesia?

BACKGROUND: Information on psychological impact of COVID-19 quarantine in primary ciliary dyskinesia (PCD), a chronic disorder with recurrent pulmonary exacerbations, is lacking. Psychological well-being was prospectively assessed during COVID-19 lockdown in Italy in a PCD population. METHODS: we recruited 27 PCD patients and 27 healthy controls. To assess psychological well-being, psychological general well-being index and parenting stress index-short questionnaires were administered to participants ≥15 years-old and to mothers of participants <15 years-old, respectively. The PCD exacerbations since outbreak onset and frequency of quarantine weekly chest physiotherapy were compared to the same period of 2019. OUTCOMES: 70% of PCD mothers and 90% of PCD patients did not show parental stress levels or distress levels, respectively, and these groups showed no significant difference in stress compared to controls. The PCD pulmonary exacerbations occurred less frequently and weekly chest physiotherapy sessions significantly increased compared to the same period during 2019 (p < 0.05). INTERPRETATION: During COVID-19 quarantine, a PCD population showed psychological well-being. Low exacerbation rate, explained by lower infectious exposure or improved compliance to chest physiotherapy, likely contributed to psychological well-being. Evaluating psychological burden and parental stress is a valuable tool for measuring the emotional impact of PCD and improving PCD medical care.

First contact: the role of respiratory cilia in host-pathogen interactions in the airways.

Respiratory cilia are the driving force of the mucociliary escalator, working in conjunction with secreted airway mucus to clear inhaled debris and pathogens from the conducting airways. Respiratory cilia are also one of the first contact points between host and inhaled pathogens. Impaired ciliary function is a common pathological feature in patients with chronic airway diseases, increasing susceptibility to respiratory infections. Common respiratory pathogens, including viruses, bacteria, and fungi, have been shown to target cilia and/or ciliated airway epithelial cells, resulting in a disruption of mucociliary clearance that may facilitate host infection. Despite being an integral component of airway innate immunity, the role of respiratory cilia and their clinical significance during airway infections are still poorly understood. This review examines the expression, structure, and function of respiratory cilia during pathogenic infection of the airways. This review also discusses specific known points of interaction of bacteria, fungi, and viruses with respiratory cilia function. The emerging biological functions of motile cilia relating to intracellular signaling and their potential immunoregulatory roles during infection will also be discussed.

Psychiatric and general health effects of COVID-19 pandemic on children with chronic lung disease and parents' coping styles.

BACKGROUND: We aim to assess the anxiety and depressive symptoms related to the COVID-19 pandemic in children with chronic lung disease and their parents and also to evaluate parents' coping strategies. METHODS: Parents of children aged 4-18 years, with chronic lung disease (study group n = 113) and healthy control (n = 108) were enrolled in the study. General Health Questionnaire-12, specific COVID-19 related anxiety questions, The Coping Orientation to Problems Experienced inventory, coronavirus-related psychiatric symptom scale in children-parental form were used to analyze the psychiatric effects of COVID-19. Parents were also asked about how online education affected their family life and children. All data were compared between children/parents in the study and control groups. Risk factors related with anxiety scores of children were also analyzed. RESULTS: Talking about the pandemic, concern about coronavirus transmission, taking precaution to prevent coronavirus transmission, making pressure to protect from COVID-19 were significantly higher in parents within the study group (p < .05). Parents in the study group used more problem-focused coping than parents in the control group (p = .003). Anxiety symptoms score was higher in children of the study group (p = .007). Parents in the study group found online education more useful than parents in the control group. CONCLUSION: Children with chronic lung diseases and their parents have more anxiety due to COVID-19 pandemic and these parents use more mature coping strategies to manage the stress of the pandemic. Longitudinal and larger studies should be done in all aspects of online education in children with chronic lung diseases.