A5049538967- Neonatal Respiratory Health Research
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Pneumonia and Respiratory Infections
- Chronic Obstructive Pulmonary Disease (COPD) Research
- Congenital Diaphragmatic Hernia Studies
- Pleural and Pulmonary Diseases
- Asthma and respiratory diseases
- Respiratory and Cough-Related Research
- Childhood Cancer Survivors' Quality of Life
- Ultrasound in Clinical Applications
- Medical Imaging and Pathology Studies
- Chronic Lymphocytic Leukemia Research
- Respiratory viral infections research
- Tuberculosis Research and Epidemiology
- Tracheal and airway disorders
- Pneumocystis jirovecii pneumonia detection and treatment
- Inhalation and Respiratory Drug Delivery
- Gastroesophageal reflux and treatments
- Acute Lymphoblastic Leukemia research
- Myasthenia Gravis and Thymoma
- Respiratory Support and Mechanisms
- Immunodeficiency and Autoimmune Disorders
- Pediatric health and respiratory diseases
- Eosinophilic Esophagitis
- Child and Adolescent Health
Medical University of Warsaw
2016-2025
Polish Mother’s Memorial Hospital Research Institute
2020
Centrum Leczenia Oparzeń
2018
Szpital Uniwersytecki w Krakowie
2018
University of Warsaw
2015
Centralny Szpital Kliniczny
2013
ENT and Allergy
2009
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 life and succumb to if not transplanted. This register-based cohort study reviews ABCA3 who survived beyond the age 1 year. Method Over a 21-year period, diagnosed as chILD due deficiency were identified from Kids Lung Register database. 44 long-term clinical...
The majority of cases with severe pulmonary alveolar proteinosis (PAP) are caused by auto-antibodies against GM-CSF. A multitude genetic and exogenous causes responsible for few other cases. Goal this study was to determine the prevalence GATA2 deficiency in children adults PAP hematologic disorders.Of 21 patients GM-CSF-autoantibody negative PAP, 13 had no organ involvement 8 some form disorder. latter were sequenced GATA2.Age at start ranged from 0.3 64 years, 4 children. In half subjects...
The aim of this study was to evaluate the factors that could predict development local complications (parapneumonic effusion/pleural empyema, necrotizing pneumonia, and lung abscess) in children with community-acquired pneumonia (CAP).Demographic, clinical, laboratory data were prospectively collected compared noncomplicated complicated CAP.Two-hundred three patients aged from 2 months 17 years enrolled. There 141 62 CAP, respectively. Significantly longer duration fever a higher level acute...
Pathogenic variants in genes encoding aminoacyl-tRNA synthetases cause numerous disorders characterized by involvement of neurons, muscles, lungs and liver. Recently, biallelic FARSB defects have been shown to severe growth restriction with combined brain, liver lung (Rajab interstitial disease [ILD] brain calcifications). Herein, for the first time, we present a patient similar condition associated mutations FARSA (NM_004461.3: c.766T>C:p.Phe256Leu c.1230C>A:p.Asn410Lys). Both detected are...
ABSTRACT Background Childhood interstitial lung diseases (chILD) are rare, chronic characterized by symptoms such as tachypnea, dyspnea, hypoxemia, crackles, and diffuse parenchymal abnormalities on chest imaging. Objective To evaluate the etiologic spectrum, clinical presentation, management, outcomes of chILD at a Polish referral center. Methods We retrospectively reviewed data from patients (0−18 years) diagnosed with chILD, admitted to Department Pediatric Pulmonology Allergy, Medical...
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 from the European network for translational research in children's adult interstitial lung disease (Cost Action CA16125) chILD-EU CRC (the Research Collaboration Children's Interstitial Lung Disease). Inclusion criteria were DAH of any cause diagnosed before...
The goal of this study was to assess the pulmonary sequelae COVID-19 pneumonia in children.Children (0-18 years old) diagnosed with hospitalized between March 2020 and 2021 were included observational study. All children underwent follow-up visits 3 months postdischarge, if any abnormalities stated, a second visit after next scheduled. Clinical assessment medical history, physical examination, lung ultrasound (LUS) using standardized protocol, function tests (PFTs). PFTs results compared...
In about 3% of children, viral infections the airways that develop in early childhood lead to narrowing laryngeal lumen subglottic region resulting symptoms such as hoarseness, abarking cough, stridor, and dyspnea. These may eventually cause respiratory failure. The disease is often called acute laryngitis (ASL). Terms pseudocroup, croup syndrome, obstructive spasmodic are used interchangeably when referencing this disease. Although differential diagnosis should include other rare diseases...
Introduction Acute exacerbations (AEs) increase morbidity and mortality of patients with chronic pulmonary diseases. Little is known about the characteristics impact AEs on children's interstitial lung disease (chILD). Methods The Kids Lung Register collected data AEs, clinical course quality life (patient-reported outcomes - PRO) rare paediatric Characteristics were obtained. Results Data 2822 2887 register visits 719 chILD recorded. characterised by increased levels dyspnoea (74.1%),...
Primary immunodeficiencies (PIDs) are rare disorders of the immune system encompassing inborn errors immunity. antibody deficiencies constitute largest group PID with common variable immunodeficiency (CVID) being most symptomatic form. Combined (CID) accompanied by deficiency can mimic CVID and these patients need verification final diagnosis. Respiratory involvement, especially interstitial lung disease (ILD), poses a relevant cause morbidity mortality among in some cases is first...
Abstract Background Persistent tachypnea of infancy (PTI) is a rare pediatric lung disease unknown origin. The diagnosis can be made by clinical presentation and chest high resolution computed tomography after exclusion other causes. Clinical courses beyond have rarely been assessed. Methods Patients included in the Kids Lung Register diagnosed with PTI as infants now older than 5 years were identified. Initial presentation, extrapulmonary comorbidities, spirometry outcome analyzed. Results...
No data on healthcare utilisation and associated costs for the many rare entities of children's interstitial lung diseases (chILD) exist. This paper portrays structures among individuals with chILD, provides a pan-European estimate 3-month interval per-capita delineates crucial cost drivers.Based longitudinal resource pattern 445 children included in Kids Lung Register diagnosed chILD across 10 European countries, we delineated direct medical non-medical care per interval. Country-specific...