Julia Carlens
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View article: Fibrosing interstitial lung disease in childhood: prevalence and disease trajectories
Fibrosing interstitial lung disease in childhood: prevalence and disease trajectories Open
Background Pulmonary fibrosis is of critical importance in childhood interstitial lung disease (chILD), yet fibrosis prevalence, impact on the clinical progression, and survival have not been systematically evaluated. Methods Data were ext…
View article: Rigid and Flexible Bronchoscopy for Foreign Body Removal in Children: Complications, Risk Factors and Anesthetic Management
Rigid and Flexible Bronchoscopy for Foreign Body Removal in Children: Complications, Risk Factors and Anesthetic Management Open
Background Foreign body (FB) aspiration is a typical emergency experienced by young children and associated with significant morbidity and mortality unless diagnosed early and treated adequately. Data on anesthetic management, applicabilit…
View article: Post-operative management of children after lung transplantation
Post-operative management of children after lung transplantation Open
Post-operative care for children and adolescents who undergo lung transplantation is a challenge because of the potential for numerous complications during this period, which can considerably impact the short- and long-term outcomes. The i…
View article: Infant lung transplantation: First German experience including two cases of ABO blood group incompatible transplantations
Infant lung transplantation: First German experience including two cases of ABO blood group incompatible transplantations Open
View article: An update on diagnosis and treatments of childhood interstitial lung diseases
An update on diagnosis and treatments of childhood interstitial lung diseases Open
Childhood interstitial lung diseases (chILDs) are rare and heterogeneous disorders associated with significant morbidity and mortality. The clinical presentation of chILD typically includes chronic or recurrent respiratory signs and sympto…
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: Impact of donor organ quality on recipient outcomes in lung transplantation: 14-Year single-center experience using the Eurotransplant lung donor score
Impact of donor organ quality on recipient outcomes in lung transplantation: 14-Year single-center experience using the Eurotransplant lung donor score Open
ECD lungs represented an important resource in lung transplantation. However, their use may be associated with a worse long-term graft and CLAD-free survival.
View article: JAK inhibitors to treat STAT3 gain-of-function: a single-center report and literature review
JAK inhibitors to treat STAT3 gain-of-function: a single-center report and literature review Open
Objective The signal transducer and activator of transcription 3 (STAT3) gain-of-function (GOF) syndrome (STAT3-GOF) is an inborn error of immunity (IEI) characterized by diverse manifestations of immune dysregulation that necessitate syst…
View article: Health‐related quality scores in childhood interstitial lung disease: Good agreement between patient and caregiver reports
Health‐related quality scores in childhood interstitial lung disease: Good agreement between patient and caregiver reports Open
Introduction Childhood interstitial lung disease (chILD) is a heterogeneous group of mostly chronic respiratory disorders. Assessment of health‐related quality of life (HrQoL) in chILD has become increasingly important in clinical care and…
View article: Paediatric Lung Transplantation for Childhood Interstitial Lung Disease Shows Favorable Outcome Compared with LuTx for Cystic Fibrosis or Pulmonary Hypertension
Paediatric Lung Transplantation for Childhood Interstitial Lung Disease Shows Favorable Outcome Compared with LuTx for Cystic Fibrosis or Pulmonary Hypertension Open
View article: Ten-year experience of whole lung lavage in pediatric Pulmonary Alveolar Proteinosis
Ten-year experience of whole lung lavage in pediatric Pulmonary Alveolar Proteinosis Open
Background Pulmonary Alveolar Proteinosis (PAP) is extremely rare and can be caused by hereditary dysfunction of the granulocyte macrophage colony-stimulating factor receptor (GM-CSF) receptor, autoantibodies against GM-CSF, or other disea…
View article: Bilateral lung transplantation for pediatric pulmonary arterial hypertension: perioperative management and one-year follow-up
Bilateral lung transplantation for pediatric pulmonary arterial hypertension: perioperative management and one-year follow-up Open
Background Bilateral lung transplantation (LuTx) remains the only established treatment for children with end-stage pulmonary arterial hypertension (PAH). Although PAH is the second most common indication for LuTx, little is known about op…
View article: Diffuse alveolar haemorrhage in children: an international multicentre study
Diffuse alveolar haemorrhage in children: an international multicentre study Open
Background Paediatric diffuse alveolar haemorrhage (DAH) is a rare heterogeneous condition with limited knowledge on clinical presentation, treatment and outcome. Methods A retrospective, descriptive multicentre follow-up study initiated f…
View article: Diagnostic workup of childhood interstitial lung disease
Diagnostic workup of childhood interstitial lung disease Open
Childhood interstitial lung diseases (chILDs) are rare and heterogeneous diseases with significant morbidity and mortality. An accurate and quick aetiological diagnosis may contribute to better management and personalised treatment. On beh…
View article: ABCA3-related interstitial lung disease beyond infancy
ABCA3-related interstitial lung disease beyond infancy Open
Background The majority of patients with childhood interstitial lung disease (chILD) caused by pathogenic variants in ATP binding cassette subfamily A member 3 (ABCA3) develop severe respiratory insufficiency within their first year of lif…
View article: Diffuse alveolar hemorrhage in children with interstitial lung disease: Determine etiologies!
Diffuse alveolar hemorrhage in children with interstitial lung disease: Determine etiologies! Open
Objective Diffuse alveolar hemorrhage (DAH) in children is a rare condition resulting from different underlying diseases. This study aimed at describing characteristics and diagnostic measures in children with ILD (children's interstitial …
View article: Minimal important difference in childhood interstitial lung diseases
Minimal important difference in childhood interstitial lung diseases Open
Background Monitoring disease progression in childhood interstitial lung diseases (chILD) is essential. No information for the minimal important difference (MID), which is defined as the smallest change in a parameter that is perceived as …
View article: COVID-19 in pediatric lung transplant recipients: Clinical course and outcome
COVID-19 in pediatric lung transplant recipients: Clinical course and outcome Open
View article: Acquired von Willebrand syndrome (AVWS) type 2, characterized by decreased high molecular weight multimers, is common in children with severe pulmonary hypertension (PH)
Acquired von Willebrand syndrome (AVWS) type 2, characterized by decreased high molecular weight multimers, is common in children with severe pulmonary hypertension (PH) Open
Background and objectives Emerging evidence suggests that increased degradation of von Willebrand factor and decrease in high molecular weight multimers occurs in patients with pulmonary hypertension (PH). However, the link between acquire…
View article: Randomized controlled phase 2 trial of hydroxychloroquine in childhood interstitial lung disease
Randomized controlled phase 2 trial of hydroxychloroquine in childhood interstitial lung disease Open
Background No results of controlled trials are available for any of the few treatments offered to children with interstitial lung diseases (chILD). We evaluated hydroxychloroquine (HCQ) in a phase 2, prospective, multicentre, 1:1-randomize…
View article: Full Recovery of Right Ventricular Systolic Function in Children Undergoing Bilateral Lung Transplantation for Severe PAH
Full Recovery of Right Ventricular Systolic Function in Children Undergoing Bilateral Lung Transplantation for Severe PAH Open
View article: Acute exacerbations in children’s interstitial lung disease
Acute exacerbations in children’s interstitial lung disease Open
Introduction Acute exacerbations (AEs) increase morbidity and mortality of patients with chronic pulmonary diseases. Little is known about the characteristics and impact of AEs on children's interstitial lung disease (chILD). Methods The K…
View article: Healthcare resource utilisation and medical costs for children with interstitial lung diseases (chILD) in Europe
Healthcare resource utilisation and medical costs for children with interstitial lung diseases (chILD) in Europe Open
Background No data on healthcare utilisation and associated costs for the many rare entities of children’s interstitial lung diseases (chILD) exist. This paper portrays healthcare utilisation structures among individuals with chILD, provid…
View article: Interventional Bronchus Occlusion Using Amplatzer Devices – A Promising Treatment Option for Children with Persistent Air Leak
Interventional Bronchus Occlusion Using Amplatzer Devices – A Promising Treatment Option for Children with Persistent Air Leak Open
Background: Persistent air leak (PAL) is a severe complication of secondary spontaneous pneumothorax (SSP). Surgical interventions are usually successful when medical treatment fails, but can be associated with significant complications an…
View article: Full recovery of right ventricular systolic function in children undergoing bilateral lung transplantation for severe PAH
Full recovery of right ventricular systolic function in children undergoing bilateral lung transplantation for severe PAH Open
We demonstrate full recovery of RV systolic function in children within two months after LuTx for severe PAH, independently of the patients' age, weight, and hemodynamic compromise preceding the LuTx. Even in end-stage pediatric PAH with p…
View article: Unusual phenotypes in patients with a pathogenic germline variant in DICER1
Unusual phenotypes in patients with a pathogenic germline variant in DICER1 Open
View article: Current Practices on Diagnosis, Prevention and Treatment of Post-Transplant Lymphoproliferative Disorder in Pediatric Patients after Solid Organ Transplantation: Results of ERN TransplantChild Healthcare Working Group Survey
Current Practices on Diagnosis, Prevention and Treatment of Post-Transplant Lymphoproliferative Disorder in Pediatric Patients after Solid Organ Transplantation: Results of ERN TransplantChild Healthcare Working Group Survey Open
(1) Background: Post-transplant lymphoproliferative disease (PTLD) is a significant complication of solid organ transplantation (SOT). However, there is lack of consensus in PTLD management. Our aim was to establish a present benchmark for…
View article: Influenza A viruses limit NLRP3‐NEK7‐complex formation and pyroptosis in human macrophages
Influenza A viruses limit NLRP3‐NEK7‐complex formation and pyroptosis in human macrophages Open
View article: Persistent tachypnea of infancy: Follow up at school age
Persistent tachypnea of infancy: Follow up at school age Open
Background Persistent tachypnea of infancy (PTI) is a rare pediatric lung disease of unknown origin. The diagnosis can be made by clinical presentation and chest high resolution computed tomography after exclusion of other causes. Clinical…
View article: Pulmonary function testing in children's interstitial lung disease
Pulmonary function testing in children's interstitial lung disease Open
The use of pulmonary function tests (PFTs) has been widely described in airway diseases like asthma and cystic fibrosis, but for children's interstitial lung disease (chILD), which encompasses a broad spectrum of pathologies, the usefulnes…