Evaluation of the relationship between mast cell activation and postural orthostatic tachycardia syndrome in children and adolescents.

Objective: Postural orthostatic tachycardia syndrome (POTS) is one of the orthostatic intolerance syndromes that are common in young adolescents and impair quality of life. POTS is a multi-systemic disease. Many mechanisms have been defined in POTS etiology, such as autonomic denervation, hypovolemia, hyperadrenergic stimulation, low condition, and hypervigilance.

Cardiac evaluation of patients with juvenile dermatomyositis.

Background: The present study aims to evaluate possible cardiac involvement in juvenile dermatomyositis (JDM) patients by conventional methods and cardiac magnetic resonance imaging (MRI) along with a systematic review of the literature on cardiac features in JDM.

Methods: The study group consisted of JDM patients who underwent cardiac MRI. We conducted a systematic review of the published literature involving JDM patients with cardiac involvement.

Behavioural interventions to increase adherence to palivizumab prophylaxis in children with CHD.

Objectives: Adherence to palivizumab prophylaxis programmes is crucial to protect infants with CHD against respiratory syncytial virus infections. We analysed the effectiveness of two nudge interventions in increasing adherence.

Methods: Our study included 229 infants, and their caregivers, from five centers in Turkey in the 2020-2021 respiratory syncytial virus season.

Mediastinal Radiotherapy-induced Early-onset Valvulopathy in a 6-Year-old Boy With Hodgkin Lymphoma.

Mediastinal radiotherapy for childhood cancers, particularly Hodgkin disease, has numerous potential adverse effects, including coronary artery disease, pericarditis, cardiomyopathy, valvular disease, and conduction abnormalities. The prevalence of valvular stenosis is relatively low, and regurgitation is more common. Mediastinal radiotherapy-induced valvular disease develops more than 10 years after radiotherapy.

Evaluation of endothelial dysfunction in hypertensive children and adolescents.

Background: Atherosclerotic changes can be attributed to early endothelial damage in individuals with hypertension. We aimed to explore the relationship between endothelial dysfunction and hypertension in newly diagnosed children without end-organ damage, considering carotid intima-media thickness (CIMT), flow-mediated dilatation (FMD), and functional capillaroscopy parameters. We also analyzed the differences between dipper and non-dipper patients.

Effect of different dose regimens of everolimus in a series of neonates with giant cardiac rhabdomyomas.

Everolimus is a mTOR inhibitor that has been increasingly used in high-risk cardiac rhabdomyomas in recent years. There are questions regarding the optimal dose and duration of therapy with everolimus for cardiac rhabdomyomas. The purpose of this study was to examine retrospectively the dosage-efficacy relationship in seven babies diagnosed with rhabdomyoma treated with different everolimus dose regimens retrospectively.

Evaluation of clinical features and outcome of eight fetuses with ectopia cordis; A study from a fetal cardiology center.

We aim to evaluate the clinical course and outcome of cases with a prenatal diagnosis of ectopia cordis in our center. In this retrospective study, we analyzed clinical variables including gestational age at diagnosis, maternal age, associated cardiac, extracardiac, genetic anomalies and, outcome in prenatally diagnosed ectopia cordis cases in our tertiary referral center. Eight ectopia cordis cases from seven pregnancies were included in the study.

Rare association of absent pulmonary valve syndrome, complete atrioventricular canal defect, double outlet right ventricle, right aortic arch, and aberrant right subclavian artery in a fetal case.

Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac anomaly. It is often associated with TOF, but may also be associated with an intact ventricular septum or, more rarely, with tricuspid atresia. It's combination with a complete atrioventricular septal defects and double outlet right ventricle has been reported extremely rare, even in the postnatal period.

Quantitative analysis of preclinical ocular microvascular changes in Multisystemic Inflammatory Syndrome in Children (MIS-C) detected by optical coherence tomography angiography.

Objectives: To evaluate the macular and optic nerve head (ONH) vascular density, foveal avascular zone area, and outer retina and choriocapillaris flow in Multisystemic Inflammatory Syndrome in Children (MIS-C) using optical coherence tomography angiography (OCTA).

Methods: Thirty-four eyes of 34 patients with MIS-C and 36 age and sex-matched healthy controls were investigated in this prospective, cross-sectional study. The superficial capillary plexus (SCP) and deep capillary plexus (DCP), ONH, FAZ parameters, the flow area of the outer retina, and choriocapillaris were evaluated using OCTA.

Prenatal diagnosis of hepatic interruption of the inferior vena cava with azygos/hemiazygos continuation without structural heart defects: A case series.

Objectives: To describe fetal spectrum and echocardiographic characteristics of interrupted inferior vena cava (IIVC) with azygos/hemiazygous continuation without other structural heart defects and to evaluate its association with visceral heterotaxy and isomerism, extracardiac and genetic anomalies, and to review neonatal outcome.

Methods: This was a retrospective study of 14 fetuses with a confirmed diagnosis of IIVC with normal intracardiac anatomy. The following variables were collected; indication for referral, gestational age at diagnosis; associated isomerism and visceral heterotaxy, heart rhythm, genetic and extracardiac abnormalities, and fetal/neonatal outcome.

The Multifaceted Presentation of the Multisystem Inflammatory Syndrome in Children: Data from a Cluster Analysis.

Background: The aim of this study was to evaluate the outcomes of patients with the multisystem inflammatory syndrome in children (MIS-C) according to phenotypes of disease and define the prognostic factors for the severe course.

Methods: This cross-sectional study included 293 patients with MIS-C from seven pediatric rheumatology centers. A two-step cluster analysis was performed to define the spectrum of disease and their outcomes were compared between each group.

Is strain echocardiography a more sensitive indicator of myocardial involvement in patients with multisystem inflammatory syndrome in children (MIS-C) associated with SARS-CoV-2?

Objective: The aim of the study was to evaluate the early myocardial dysfunction detected by strain echocardiography in children with multisystem inflammatory syndrome related to SARS-CoV-2 infection.

Methods: This cross-sectional study was conducted with 47 patients diagnosed with MIS-C and 32 healthy age- and gender-matched children. All patients underwent two-dimensional, colour, pulsed, and tissue Doppler, and 2D speckle tracking echocardiography examination at admission, 2 weeks, and 2 months after discharge.

CXCL10/IP10 as a Biomarker Linking Multisystem Inflammatory Syndrome and Left Ventricular Dysfunction in Children with SARS-CoV-2.

Background: To investigate the diagnostic accuracy of CXCL10/IP10 for left ventricular (LV) dysfunction in multisystemic inflammatory syndrome (MIS-C).

Methods: This cross-sectional, longitudinal study included 36 patients with MIS-C. Patients were classified as follows: (1) patients presenting with Kawasaki-like features (group I = 11); (2) patients presenting with LV systolic dysfunction (group II = 9); and (3) other presentations (group III = 3).

Myopericarditis in children and adolescent: is the elevated troponin and chest pain as alarming as we thought?

Aim: When encountering adolescents with chest pain and a high troponin level but with no underlying coronary artery illness, it is advisable to consider myopericarditis. Though myopericarditis is a self-limiting, benign condition, it nevertheless causes anxiety in the patient and the family.

Methods: Thirty-nine patients diagnosed with myopericarditis were included.

Multisystemic inflammatory syndrome in children associated with COVID-19: a single center experience in Turkey.

Objective: Multisystem inflammatory syndrome in children (MIS-C) associated with the coronavirus disease 2019 (COVID-19) is a new concern emerging as a severe presentation of COVID-19 in children. We aimed to describe the characteristics and short-term outcomes of children diagnosed with MIS-C.

Material And Methods: A retrospective study was conducted on 24 patients who were diagnosed with MIS-C between June 1, 2020 and December 1, 2020.

A prenatal diagnosis of unusual aortopulmonary communication: tubular aortopulmonary window.

We described a very rare case of aorto-pulmonary communication with right aortic arch and crossed pulmonary artery that cannot be placed in the typical anatomic classification of aortopulmonary window. At 23 weeks gestation, fetal echocardiography revealed a large tunnel-like communication connecting the great vessels proximal to the main pulmonary artery bifurcation, rather than a classic aortopulmonary window between the ascending aorta and the main pulmonary artery.

Myeloid sarcoma of the heart: Extramedullary relapse of acute myeloblastic leukemia, presenting with complete heart block and atrial flutter after second allogeneic stem cell transplantation.

We report isolated extramedullary relapse in a 14-year-old boy, sequentially presenting with intestinal and cardiac myeloid sarcoma (MS). Acute myeloblastic leukemia M5 was diagnosed 41 months ago. On the 14th month of the second HSCT, he presented with ileus and underwent surgical treatment.

Is there myocardial involvement in children with long-term follow-up for Kawasaki disease? A study based on two-dimensional speckle tracking echocardiography.

Objective: This study aimed to determine the possibility of subclinical myocardial dysfunction detected by strain echocardiography in the late period of children with Kawasaki disease.

Material And Methods: The study enrolled 30 patients with Kawasaki disease with a follow-up period of at least 12 months and 30 healthy age- and gender-matched children. During the follow-up period, standard echocardiography, pulsed and tissue Doppler, and strain echocardiography were recorded for both groups.

Subclinical anthracycline-induced cardiotoxicity in long-term follow-up of asymptomatic childhood cancer survivors: Assessment by speckle tracking echocardiography.

Objective: Survivors of childhood cancer treated with anthracyclines carry the risk for developing late-onset cardiotoxicity. The purpose of this study was to evaluate left ventricular (LV) function in this patient group and compare it with healthy controls by means of conventional and speckle tracking echocardiography (STE) after exposure to chemotherapy.

Material And Methods: Conventional and STE were performed in 45 childhood cancer survivors (mean age 11 ± 4.

Initial Experience with the Nit-Occlud ASD-R: Short-Term Results.

We aim to assess the safety, feasibility and efficacy of the new Nit-Occlud ASD-R (NOASD-R) device. From 2014 to 2015, transcatheter closure of atrial septal defect (ASD) using the NOASD-R was performed in 30 consecutive patients. The standard deployment technique as the left upper pulmonary vein approach was used in 25 patients.

Are cardiac magnetic resonance imaging and radionuclide ventriculography good options against echocardiography for evaluation of anthracycline induced chronic cardiotoxicity in childhood cancer survivors?

Anthracyclines are widely used for the treatment of solid tumors in pediatric oncology. However, their uses may be limited by progressive chronic cardiotoxicity related to the cumulative dosage. The aims of this study are to compare diagnostic techniques and prepare an algorithm for diagnosis of anthracycline induced chronic cardiotoxicity.

Recombinant human erythropoietin beta therapy: an effective strategy to reduce transfusion requirement in children receiving anticancer treatment.

In recent years erythropoietic agents have become important tools in the management of anemia in cancer patients, improving hemoglobin (Hb) concentrations, reducing the need for transfusion, and enhancing quality of life. In this prospective and historically controlled study, the effects of epoetin beta on Hb concentrations and red blood cell transfusion needs in children with cancer receiving chemotherapy or radiotherapy have been investigated. Epoetin beta (150 U/kg/day, 3 days a week) was given subcutaneously to 22 children with cancer when Hb concentration < or = 10 g/dL.

Granulomatous reaction in mediastinal B-cell non-Hodgkin lymphoma and intracardiac thrombosis.

Epithelioid cell granulomas may be associated with several neoplasms. Lymphomas may mimic or are associated with epithelioid granulomas. In this article the authors report a child with granulomatous reaction in B cell non-Hodgkin lymphoma and intracardiac thrombosis.