Myocardial damage in multisystem inflammatory syndrome associated with COVID-19 in children and adolescents.

Background: In multisystem inflammatory syndrome in children (MIS-C) temporarily associated with coronavirus disease-19 (COVID-19), myocardial damage has been reported.

Materials And Methods: A retrospective observational cohort study included children under 18 who had a myocardial injury related to COVID-19 treated in mother and child health institute from April 2020 to August 2020. Myocardial injury related to COVID-19 was manifested by elevated serum cardiac troponin and NT-proBNP with LV dysfunction, arrhythmias, and coronary arteries (CAs) dilatation or aneurysms.

Factors for severe outcomes following SARS-CoV-2 infection in people with cystic fibrosis in Europe.

Background: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in people with cystic fibrosis (pwCF) can lead to severe outcomes.

Methods: In this observational study, the European Cystic Fibrosis Society Patient Registry collected data on pwCF and SARS-CoV-2 infection to estimate incidence, describe clinical presentation and investigate factors associated with severe outcomes using multivariable analysis.

Results: Up to December 31, 2020, 26 countries reported information on 828 pwCF and SARS-CoV-2 infection.

Biologicals in childhood severe asthma: the European PERMEABLE survey on the .

Introduction: Severe asthma is a rare disease in children, for which three biologicals, anti-immunoglobulin E, anti-interleukin-5 and anti-IL4RA antibodies, are available in European countries. While global guidelines exist on who should receive biologicals, knowledge is lacking on how those guidelines are implemented in real life and which unmet needs exist in the field. In this survey, we aimed to investigate the and identify open questions in biological therapy of childhood asthma across Europe.

Recent Experience: Corticosteroids as a First-line Therapy in Children With Multisystem Inflammatory Syndrome and COVID-19-related Myocardial Damage.

Background: Cardiovascular complications with myocarditis in multisystem inflammatory syndrome in children (MIS-C) associated with severe acute respiratory syndrome coronavirus 2 infection have been reported, but the optimal therapeutic strategy remains unknown.

Methods: A retrospective cohort study included 19 patients with acute left ventricular systolic dysfunction associated with MIS-C, average years of age 13.2 ± 3.

Incidence of SARS-CoV-2 in people with cystic fibrosis in Europe between February and June 2020.

Background: Viral infections can cause significant morbidity in cystic fibrosis (CF). The current Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic could therefore have a serious impact on the health of people with CF (pwCF).

Methods: We used the 38-country European Cystic Fibrosis Society Patient Registry (ECFSPR) to collect case data about pwCF and SARS-CoV-2 infection.

Surgical treatment of ascending aorta floating thrombus in a patient with recent SARS-CoV-2 infection.

The 44-year-old female was admitted to the hospital due to the severe pain in the right arm. Doppler ultrasonography revealed occlusion of the right brachial and right common carotid artery. Subsequently, computed tomography (CT) scan confirmed the occlusion of the right brachial and common carotid artery, and revealed pedunculated floating ascending aortic mass.

Differences Between Pediatric Acute Myocarditis Related and Unrelated to SARS-CoV-2.

Background: Acute myocarditis (AM) is defined as inflammation of the myocardium. The aim of our study is a comparative analysis of the differences between AM related and unrelated to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2).

Methods: The retrospective study included children with AM treated from January 2018 to November 2020.

Surgical Treatment of Pseudoaneurysm of the Ascending Aorta in a Patient with Sepsis.

Pseudoaneurysm of the ascending aorta (PAA) is a hazardous and potentially fatal cardiovascular disease. This condition is caused by the rupture of at least one layer of the vessel and contained by the remaining vascular layers or the surrounding mediastinal structures. We presented the surgical treatment of a patient with sepsis and large PAA and brachiocephalic trunk, which was compressing the brachiocephalic trunk leading to syncope.

Kawasaki-like disease and acute myocarditis in the SARS-CoV-2 pandemic - reports of three adolescents.

The novel coronavirus disease (COVID-19) may induce multisystem inflammatory syndrome (MIS) in children, which may be associated with Kawasaki-like disease and cardiac injury. In this study, we presented three male adolescents with MIS and myocardial injury admitted to the hospital during the peak of COVID-19 pandemic. All of the three patients had a history of fever, gastrointestinal symptoms, polymorph rash, non-exudative  onjunctivitis, and signs of acute myocarditis (AM).

A Scimitar Syndrome Variant Associated with Critical Aortic Coarctation in a Newborn.

BACKGROUND Scimitar syndrome (SCS) is a rare congenital cardiopulmonary malformation, characterized by anomalous pulmonary venous drainage from the right lung associated with aortopulmonary collateral arteries and pulmonary hypoplasia. The variant described in the case presented here, with total anomalous right pulmonary venous drainage into the superior and inferior vena cava, can be expected in 2% of patients with scimitar syndrome. To the best of our knowledge, the association between the variant of SCS and coarctation of aorta described in our patient has never been reported before in the literature.

Predictive factors of recurrence after pediatric acute pericarditis.

Objective: The predisposing factors for pericarditis recurrence in the pediatric population have not yet been established. This study aimed to define the risk factors for the unfavorable prognosis of pediatric acute pericarditis.

Methods: This was a retrospective study that included all patients with acute pericarditis treated from 2011 to 2019 at a tertiary referent pediatric center.

The Clinical and Genetic Spectrum of 82 Patients With Deficiency Including a c.256_257delAA Founder Variant in Slavic Countries.

Variants in recombination-activating genes () are common genetic causes of autosomal recessive forms of combined immunodeficiencies (CID) ranging from severe combined immunodeficiency (SCID), Omenn syndrome (OS), leaky SCID, and CID with granulomas and/or autoimmunity (CID-G/AI), and even milder presentation with antibody deficiency. We aim to estimate the incidence, clinical presentation, genetic variability, and treatment outcome with geographic distribution of patients with the defects in populations inhabiting South, West, and East Slavic countries. Demographic, clinical, and laboratory data were collected from -deficient patients of Slavic origin via chart review, retrospectively.

Flexible Bronchoscopy in Evaluation of Persistent Wheezing in Children-Experiences from National Pediatric Center.

Persistent wheezing (PW) is defined as prolonged or recurrent episodes of wheezing despite regular treatment. Flexible bronchoscopy (FB) is recommended to determine the etiology of PW in children. This study aimed to determine the etiology of PW based on FB findings in a national pediatric center.

Lipid profile and left ventricular geometry pattern in obese children.

Background: Left ventricular hypertrophy (LVH) is an important risk factor for cardiovascular and all-cause mortality. Previous studies reported conflicting results concerning the relationship between serum lipid levels and left ventricular geometry pattern. We sought to explore the relationship between standard serum lipid profile measures with left ventricular geometry pattern in obese children.

Changing epidemiology of the respiratory bacteriology of patients with cystic fibrosis-data from the European cystic fibrosis society patient registry.

Background: Monitoring changes in the epidemiology of cystic fibrosis (CF) pathogens is essential for clinical research, quality improvement, and clinical management.

Methods: We analyzed data reported to the European Cystic Fibrosis Society Patient Registry (ECFSPR) from 2011 to 2016 to determine the overall and the age-specific annual prevalence and incidence of selected CF pathogens and their trends during these years. The ECFSPR collects data on three chronic infections: Pseudomonas aeruginosa (PsA), Burkholderia cepacia complex Species (BCC) and Staphylococcus aureus (SA), as well as on the occurrence of non-tuberculous mycobacteria (NTM) and Stenotrophomonas maltophilia (SM).

Decannulation in children affected by congenital central hypoventilation syndrome: A proposal of an algorithm from two European centers.

Rationale: Long-life ventilatory assistance is necessary for survival in pediatric patients with congenital central hypoventilation syndrome (CCHS). Invasive mechanical ventilation (IMV) through tracheostomy is the most used method, especially in the first years of life when the central nervous system is maturing. Nevertheless, IMV via tracheostomy is not ideal because tracheostomy in children is associated with impaired speech and language development, also frequent infections of the lower airway tract occur.

CFTR activity is enhanced by the novel corrector GLPG2222, given with and without ivacaftor in two randomized trials.

Background: Several treatment approaches in cystic fibrosis (CF) aim to correct CF transmembrane conductance regulator (CFTR) function; the efficacy of each approach is dependent on the mutation(s) present. A need remains for more effective treatments to correct functional deficits caused by the F508del mutation.

Methods: Two placebo-controlled, phase 2a studies evaluated GLPG2222, given orally once daily for 29 days, in subjects homozygous for F508del (FLAMINGO) or heterozygous for F508del and a gating mutation, receiving ivacaftor (ALBATROSS).

Congenital B-cell Acute Lymphoblastic Leukemia with Congenital Rubella Infection.

Background: Congenital B-cell Acute lymphoblastic leukemia (ALL) is a rare malignancy.

Characteristics: A newborn infant presented with purpuric spots and ecchymotic patches, blueberry muffin rash, depressed neonatal reflexes, respiratory distress and hepatosplenomegaly. Peripheral smear revealed atypical blast cells.

The future of paediatric and adolescent gynaecology in Europe.

Paediatric and Adolescent Gynaecology (PAG) is a subspecialty under the umbrella of Obstetrics and Gynaecology but linked to other branches of medicine including Paediatrics, Surgery, Endocrinology and Urology. Therefore future developments in clinical care and education requires a multidisciplinary approach combining aspects of all the above medical specialties, and also with inputs from Public Health, Genetics, Radiology and Psychology. A multidisciplinary collaboration among different specialists is as important as the establishment and adoption of standards in education, training and management.

Respiratory Muscle Strength and Exercise Performance in Cystic Fibrosis-A Cross Sectional Study.

Decreased respiratory muscle strength in patients with cystic fibrosis (CF) may cause progressive exercise intolerance during cardiopulmonary exercise testing (CPET), and may contribute to the development of chronic respiratory insufficiency. The aim of this study is to evaluate exercise tolerance during CPET of children and adults with clinically stable CF who exhibit different respiratory muscle strength. Sixty-nine clinically stable CF subjects aged 8-33 years underwent spirometry, body plethysmography, CPET, and respiratory muscle strength measurement.

A 6-month follow-up of disability, quality of life, and depressive and anxiety symptoms in pediatric migraine with magnesium prophylaxis.

Magnesium is frequently used for pediatric migraine prophylaxis. The aim of this study was to evaluate to which extent the disability levels, quality of life (QOL), and anxiety and depressive symptoms change after 6-month magnesium prophylaxis in pediatric migraine. This is a follow-up study of 34 children aged 7-17 years with migraine treated with oral magnesium.