Long-Term Lung Function and Infection in Genotyped Primary Ciliary Dyskinesia.

Primary ciliary dyskinesia is a rare genetic disorder characterized by progressive lung disease. is a major pathogen in this disease, known to impact lung function. Previous genotype-phenotype studies have been limited by cross-sectional designs, isolated adult or pediatric populations, small numbers, or short follow-up durations.

A range of 30-62% of functioning multiciliated airway cells is sufficient to maintain ciliary airway clearance.

Background: Primary ciliary dyskinesia is a genetic disorder caused by aberrant motile cilia function that results in defective ciliary airway clearance and subsequently leads to recurrent airway infections and bronchiectasis. We aimed to determine: how many functional multiciliated airway cells are sufficient to maintain ciliary airway clearance?

Methods: To answer this question we exploited the molecular defects of the X-linked recessive primary ciliary dyskinesia variant caused by pathogenic variants in (), characterised by immotile cilia in affected males. We carefully analysed the clinical phenotype and molecular defect (using immunofluorescence and transmission electron microscopy) and performed studies (particle tracking in air-liquid interface cultures) and studies (radiolabelled tracer studies) to assess ciliary clearance of respiratory cells from female individuals with heterozygous and male individuals with hemizygous pathogenic variants.

Treat and release: an observational study of non-conveyed high-acuity dispatches in a Danish emergency medical system.

Non-conveyance refers to the practice of treating a patient on-site without transporting them to a medical facility. It may decrease unnecessary hospital transfers and improve patient satisfaction. Nonetheless, ensuring patient safety remains paramount.

Foreign body airway obstruction resulting in out-of-hospital cardiac arrest in Denmark - Incidence, survival and interventions.

Background: Foreign body airway obstruction (FBAO) stands as an important contributor to accidental fatalities, yet prompt bystander interventions have been shown to improve survival. This study aimed to evaluate the incidence, interventions, and survival outcomes of patients with out-of-hospital cardiac arrest (OHCA) related to FBAO in comparison to patients with non-FBAO OHCA.

Methods: In this population-based cohort study, we included all OHCAs in Denmark from 2016 to 2022.

Prehospital interventions and outcomes in traumatic cardiac arrest: a population-based cohort study using the Danish Helicopter Emergency Medical Services data.

Background And Importance: Traumatic cardiac arrest is associated with poor prognosis, and timely evidence-based treatment is paramount for increasing survival rates. Physician-staffed helicopter emergency medical service use in major trauma has demonstrated improved outcomes. However, the sparsity of data highlights the necessity for a comprehensive understanding of the epidemiology of traumatic cardiac arrest.

Pulmonary radioaerosol mucociliary clearance assessment: searching for genotype-specific differences and potential as an outcome measure in primary ciliary dyskinesia.

Background: Pulmonary radioaerosol mucociliary clearance (PRMC) is a reliable method for assessing whole lung mucociliary clearance and has been used at the Danish PCD Centre as a supplementary diagnostic test for primary ciliary dyskinesia (PCD) for more than two decades. This study aimed to investigate genotype-specific differences in PRMC measures and evaluate its potential as an outcome parameter.

Material And Methods: The study was based on a retrospective analysis of PRMC tests performed over a 24-year period (1999-2022) in individuals referred for PCD work-up and included patients with genetically confirmed PCD and non-PCD controls.

Danish Drowning Formula for identification of out-of-hospital cardiac arrest from drowning.

Background: Accurate, reliable, and sufficient data is required to reduce the burden of drowning by targeting preventive measures and improving treatment. Today's drowning statistics are informed by various methods sometimes based on data sources with questionable reliability. These methods are likely responsible for a systematic and significant underreporting of drowning.

Geographical Association Between Basic Life Support Courses and Bystander Cardiopulmonary Resuscitation and Survival from OHCA in Denmark.

Introduction: Annually, approximately 4% of the entire adult population of Denmark participate in certified basic life support (BLS) courses. It is still unknown whether increases in BLS course participation in a geographical area increase bystander cardiopulmonary resuscitation (CPR) or survival from out-of-hospital cardiac arrest (OHCA). The aim of the study was to examine the geographical association between BLS course participation, bystander CPR, and 30-day survival from OHCA.

Pediatric out-of-hospital cardiac arrest in Denmark.

Background: Pediatric out-of-hospital cardiac arrest (POHCA) has received limited attention. All causes of POHCA and outcomes were examined during a 4-year period in a Danish nationwide register and prehospital medical records. The aim was to describe the incidence, reversible causes, and survival rates for POHCA in Denmark.

Chronic airway disease in primary ciliary dyskinesia-spiced with geno-phenotype associations.

Primary ciliary dyskinesia (PCD) can be defined as a multiorgan ciliopathy with a dominant element of chronic airway disease affecting the nose, sinuses, middle ear, and in particular, the lower airways. Although most patients with PCD are diagnosed during preschool years, it is obvious that the chronic lung disease starts its course already from birth. The many faces of the clinical picture change, as does lung function, structural lung damage, the burden of infection, and of treatment throughout life.

Out-of-hospital cardiac arrest related to exercise in the general population: Incidence, survival and bystander response.

Background: Regular exercise is known to prevent cardiovascular disorders, but it may also trigger acute cardiac events. This study examined the incidence, prognosis, and outcomes of out-of-hospital cardiac arrest (OHCA) related to exercise in the general population of Denmark.

Methods: This retrospective cohort study examined all the OHCAs in the Danish Cardiac Arrest Registry from 2016 to 2019.

A retrospective review of Achromobacter species and antibiotic treatments in patients with primary ciliary dyskinesia.

Primary ciliary dyskinesia (PCD) is a rare congenital disease with defective mucociliary clearance causing frequent and often persistent pulmonary infections. Achromobacter species are opportunistic pathogens renowned for the difficulty of effective treatments and deteriorating effects on lung function. We aimed to describe the occurrence, treatment, and rate of successful eradication of Achromobacter species in patients with PCD.

Socio-demographic characteristics of basic life support course participants in Denmark.

Background: Bystander-initiated basic life support (BLS) plays an important role in improving survival after out-of-hospital cardiac arrest. In 2009, laws mandating BLS course participation when acquiring a driver's licence were implemented in Denmark. The aim of this study was to characterise Danish BLS course participants.

Strategies to Handle Increased Demand in the COVID-19 Crisis: A Coronavirus EMS Support Track and a Web-Based Self-Triage System.

Background: In emergencies, such as the COVID-19 pandemic, there is an increased need for contact with emergency medical services (EMS), and call volume might surpass capacity. The Copenhagen EMS operates two telephone line the 1-1-2 emergency number and the 1813 medical helpline. A separate coronavirus support track was implemented on the 1813 medical helpline and a web-based self-triage (web triage) system was created to reduce non-emergency call volume.

The impact of mannose-binding lectin polymorphisms on lung function in primary ciliary dyskinesia.

Objective: Primary ciliary dyskinesia (PCD) is a congenital lung disease that leads to recurrent and chronic lung infection. The resulting inflammation causes lung damage and declines in lung function. Mannose-binding lectin (MBL) is a first line host defense protein of importance for the innate immunity.

Proof of Concept: Very Rapid Tidal Breathing Nasal Nitric Oxide Sampling Discriminates Primary Ciliary Dyskinesia from Healthy Subjects.

Introduction: Nasal nitric oxide (nNO) is extremely low in individuals with primary ciliary dyskinesia (PCD) and is recommended as part of early workup. We investigated whether tidal breathing sampling for a few seconds was as discriminative between PCD and healthy controls (HC) as conventional tidal breathing sampling (cTB-nNO) for 20-30 s.

Methods: We performed very rapid sampling of tidal breathing (vrTB-nNO) for 2, 4 and 6 s, respectively.

Variation in treatment strategies for the eradication of Pseudomonas aeruginosa in primary ciliary dyskinesia across European centers.

Primary ciliary dyskinesia (PCD) is a rare disease causing motile cilia dysfunction, recurrent airway infection, and bronchiectasis. Airway infection management strategies are borrowed from cystic fibrosis. The aim of this study is to describe the management of airway infection with Pseudomonas aeruginosa ( PA) in children and adults with PCD across European centers.

Fitness and lung function in children with primary ciliary dyskinesia and cystic fibrosis.

Background: Peak oxygen uptake (VO) is associated with morbidity and mortality in health and disease, and provides important information of global physical health not achieved from standard pulmonary function tests. Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are genetically determined diseases involving different basic defects, but both showing impaired mucociliary clearance leading to chronic infections and pulmonary destruction early in life. PCD is generally considered a milder disease than CF and it is hypothesized that children with CF would have consistently lower VO and pulmonary function than children with PCD.

Initiating transitional care for adolescents with cystic fibrosis at the age of 12 is both feasible and promising.

Aim: Adolescence is a vulnerable period in cystic fibrosis, associated with declining lung function. This study described, implemented and evaluated a transition programme for adolescents.

Methods: We conducted a single centre, nonrandomised and noncontrolled prospective programme at the cystic fibrosis centre at Copenhagen University Hospital Rigshospitalet from 2010 to 2011, assessing patients aged 12-18 at baseline and after 12 months.