Kim G. Nielsen
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View article: Limitations of PICADAR as a diagnostic predictive tool for primary ciliary dyskinesia
Limitations of PICADAR as a diagnostic predictive tool for primary ciliary dyskinesia Open
Background The Primary Ciliary Dyskinesia Rule (PICADAR) is a diagnostic predictive tool currently recommended by the European Respiratory Society (ERS) to assess the likelihood of a primary ciliary dyskinesia (PCD) diagnosis. Despite its …
View article: Physical Functioning Report and Pulmonary Function Deficit in Childhood and Adolescent Acute Lymphoblastic Leukemia Survivors: A National Cross‐Sectional ALL‐STAR Lungs Study
Physical Functioning Report and Pulmonary Function Deficit in Childhood and Adolescent Acute Lymphoblastic Leukemia Survivors: A National Cross‐Sectional ALL‐STAR Lungs Study Open
Background Childhood acute lymphoblastic leukemia (ALL) survivors face an increased risk of pulmonary function deficit (PFD), which may affect physical functioning. We aimed to evaluate parent proxy‐ and self‐reported physical functioning …
View article: Mental Health in a Diverse Group of Children, Adolescents, and Adults Previously Treated for Childhood Interstitial Lung Disease
Mental Health in a Diverse Group of Children, Adolescents, and Adults Previously Treated for Childhood Interstitial Lung Disease Open
Background While most studies on Childhood Interstitial Lung Disease (chILD) focus on somatic outcomes, little is known about the long‐term outcome on mental health in this population. Therefore, we assessed mental health in former chILD p…
View article: Limitations of PICADAR as a diagnostic predictive tool for primary ciliary dyskinesia
Limitations of PICADAR as a diagnostic predictive tool for primary ciliary dyskinesia Open
Background The Primary Ciliary Dyskinesia Rule (PICADAR) is a diagnostic predictive tool currently recommended by the European Respiratory Society (ERS) to assess the likelihood of a primary ciliary dyskinesia (PCD) diagnosis. Despite its …
View article: Real‐World Improvements of Lung Clearance Index and Ventilation Distribution Efficiency in Children With Cystic Fibrosis After Elexacaftor/Tezacaftor/Ivacaftor Initiation
Real‐World Improvements of Lung Clearance Index and Ventilation Distribution Efficiency in Children With Cystic Fibrosis After Elexacaftor/Tezacaftor/Ivacaftor Initiation Open
Introduction Elexacaftor/tezacaftor/ivacaftor (ETI) is a breakthrough therapy for cystic fibrosis (CF). We aimed to assess ETI's real‐world impact on peripheral airway disease assessed as ventilation distribution inhomogeneity using nitrog…
View article: Natural variability of lung function in primary ciliary dyskinesia: longitudinal analysis from the PROVALF-PCD cohort
Natural variability of lung function in primary ciliary dyskinesia: longitudinal analysis from the PROVALF-PCD cohort Open
Background The extent to which changes in lung function are due to natural variability in patients with primary ciliary dyskinesia (PCD) is unknown. We aimed to assess intra-individual variability in forced expiratory volume in 1 s (FEV 1 …
View article: Diagnostic Evaluation and Clinical Findings in Children With Persistent Tachypnea of Infancy/Neuroendocrine Cell Hyperplasia of Infancy
Diagnostic Evaluation and Clinical Findings in Children With Persistent Tachypnea of Infancy/Neuroendocrine Cell Hyperplasia of Infancy Open
Diagnosis of PTI/NEHI relies on clinical symptoms and HRCT imaging results, with lung biopsies less commonly performed. Differences exist among countries regarding the number and type of investigations. A need exists for guidelines that wi…
View article: International consensus statement on routine blood testing in primary ciliary dyskinesia
International consensus statement on routine blood testing in primary ciliary dyskinesia Open
Background Primary ciliary dyskinesia (PCD) is a rare genetic disorder characterised by dysfunction of motile cilia. Symptoms include recurrent and chronic airway infections which can lead to deteriorating lung function and inflammatory de…
View article: Tracheomalacia and surgical options: A case series perspective
Tracheomalacia and surgical options: A case series perspective Open
Tracheomalacia causes considerable morbidity in children, and the best treatment options remain debated. This paper presents a case series of seven Danish children who underwent surgical interventions, such as tracheopexy and aortopexy, de…
View article: Associations between respiratory pathogens and lung function in primary ciliary dyskinesia: cross-sectional analysis from the PROVALF-PCD cohort
Associations between respiratory pathogens and lung function in primary ciliary dyskinesia: cross-sectional analysis from the PROVALF-PCD cohort Open
Introduction Respiratory pathogens are frequently isolated from airway samples in primary ciliary dyskinesia (PCD) patients. Few studies have investigated associations between these pathogens and lung function, with current management base…
View article: A range of 30–62% of functioning multiciliated airway cells is sufficient to maintain ciliary airway clearance
A range of 30–62% of functioning multiciliated airway cells is sufficient to maintain ciliary airway clearance Open
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 d…
View article: A systematic literature review of the clinical and socioeconomic burden of bronchiectasis
A systematic literature review of the clinical and socioeconomic burden of bronchiectasis Open
Background The overall burden of bronchiectasis on patients and healthcare systems has not been comprehensively described. Here, we present the findings of a systematic literature review that assessed the clinical and socioeconomic burden …
View article: Lung function improvement on triple modulators: high-resolution, nationwide data from the Danish Cystic Fibrosis Cohort
Lung function improvement on triple modulators: high-resolution, nationwide data from the Danish Cystic Fibrosis Cohort Open
Background People living with cystic fibrosis in Denmark had early, universal access to triple modulator treatment with elexacaftor/tezacaftor/ivacaftor. Close monitoring allowed us to assess the impact of treatment on lung function and pr…
View article: Analyses of 1236 genotyped primary ciliary dyskinesia individuals identify regional clusters of distinct DNA variants and significant genotype–phenotype correlations
Analyses of 1236 genotyped primary ciliary dyskinesia individuals identify regional clusters of distinct DNA variants and significant genotype–phenotype correlations Open
Background Primary ciliary dyskinesia (PCD) represents a group of rare hereditary disorders characterised by deficient ciliary airway clearance that can be associated with laterality defects. We aimed to describe the underlying gene defect…
View article: Lung Function in Fontan Patients Over a Ten-Year Period: Is the Fontan Circulation Impairing Lung Development?
Lung Function in Fontan Patients Over a Ten-Year Period: Is the Fontan Circulation Impairing Lung Development? Open
Few studies have investigated how the Fontan circulation affects lung function, and no studies have investigated the development of lung function over longer time in these patients. We aimed to describe the development of lung function in …
View article: Close monitoring and early intervention: management principles for cystic fibrosis in Denmark
Close monitoring and early intervention: management principles for cystic fibrosis in Denmark Open
Cystic fibrosis (CF) care in Denmark has been characterized by close monitoring and pre‐emptive treatment of lung disease and other CF‐related complications. Continuous evaluation through data collection and commitment to clinical research…
View article: Long‐term effects of high‐dose systemic corticosteroids on growth and bone mineral density in patients treated for childhood interstitial lung disease (chILD)
Long‐term effects of high‐dose systemic corticosteroids on growth and bone mineral density in patients treated for childhood interstitial lung disease (chILD) Open
Background Children's interstitial lung disease (chILD) is a rare and potentially life‐threatening condition. For many chILD conditions, systemic corticosteroids (sCCS) are considered the primary treatment despite a broad spectrum of poten…
View article: Surfactant protein D is associated with pulmonary manifestations of chronic graft‐versus‐host disease following hematopoietic stem cell transplantation
Surfactant protein D is associated with pulmonary manifestations of chronic graft‐versus‐host disease following hematopoietic stem cell transplantation Open
Impairment of pulmonary function is reported in around 60% of children after allogeneic hematopoietic stem cell transplantation (HSCT), used as a treatment of high-risk acute leukemias and severe hematological disorders.1 The decline in pu…
View article: A BEAT-PCD consensus statement: a core outcome set for pulmonary disease interventions in primary ciliary dyskinesia
A BEAT-PCD consensus statement: a core outcome set for pulmonary disease interventions in primary ciliary dyskinesia Open
Background Consistent use of reliable and clinically appropriate outcome measures is a priority for clinical trials, with clear definitions to allow comparability. We aimed to develop a core outcome set (COS) for pulmonary disease interven…
View article: Pulmonary radioaerosol mucociliary clearance assessment: searching for genotype-specific differences and potential as an outcome measure in primary ciliary dyskinesia
Pulmonary radioaerosol mucociliary clearance assessment: searching for genotype-specific differences and potential as an outcome measure in primary ciliary dyskinesia Open
Background Pulmonary radioaerosol mucociliary clearance (PRMC) is a reliable method for assessing in vivo whole lung mucociliary clearance and has been used at the Danish PCD Centre as a supplementary diagnostic test for primary ciliary dy…
View article: Automatic analysis of bronchus-artery dimensions to diagnose and monitor airways disease in cystic fibrosis
Automatic analysis of bronchus-artery dimensions to diagnose and monitor airways disease in cystic fibrosis Open
Background Cystic fibrosis (CF) lung disease is characterised by progressive airway wall thickening and widening. We aimed to validate an artificial intelligence-based algorithm to assess dimensions of all visible bronchus-artery (BA) pair…
View article: Nationwide lung function monitoring from infancy in newborn-screened children with cystic fibrosis
Nationwide lung function monitoring from infancy in newborn-screened children with cystic fibrosis Open
Background Cystic fibrosis (CF) lung disease starts in infancy and can be assessed for structural lung abnormalities using computed tomography or magnetic resonance scans, or for lung function impairment using multiple breath washout (MBW)…
View article: Incidence and aetiology of Danish children with community-acquired pneumonia treated with chest tube drainage in 2022–2023 versus the previous three decades
Incidence and aetiology of Danish children with community-acquired pneumonia treated with chest tube drainage in 2022–2023 versus the previous three decades Open
In conclusion, we found a fivefold increase in children with complicated community-acquired pneumonia treated with CTD in 2022-2023 versus the previous three decades, primarily due to GAS.For the individual child, GAS pneumonia treated wit…
View article: Multiple breath washout and oscillometry after allogenic HSCT: a scoping review
Multiple breath washout and oscillometry after allogenic HSCT: a scoping review Open
Pulmonary chronic graft- versus -host disease (cGVHD) is a substantial cause of pulmonary morbidity and mortality post-haematopoietic stem cell transplantation (HSCT). Current spirometry-based monitoring strategies have significant limitat…
View article: Quantitative<sup>99m</sup>Tc-albumin colloid nasal mucociliary clearance as an outcome in primary ciliary dyskinesia
Quantitative<sup>99m</sup>Tc-albumin colloid nasal mucociliary clearance as an outcome in primary ciliary dyskinesia Open
Background Primary ciliary dyskinesia (PCD) is an inherited disorder in which dyskinetic cilia cause impaired mucociliary clearance of upper and lower airways. Airway ciliary movement can be indirectly tested in vivo after administration o…
View article: Pulmonary Radioaerosol Mucociliary Clearance Parameters as Potential Outcomes in Primary Ciliary Dyskinesia Trials
Pulmonary Radioaerosol Mucociliary Clearance Parameters as Potential Outcomes in Primary Ciliary Dyskinesia Trials Open
Background Pulmonary Radioaerosol Mucociliary Clearance (PRMC) is an in vivo whole lung ciliary function test reliable for assessing mucociliary clearance for diagnostic purposes in individuals suspected of primary ciliary dyskinesia (PCD)…