Multidisciplinary treatment for a patient with Pentalogy of Cantrell in the COVID-19 pandemic.

In this article, we present a multidisciplinary approach for the treatment of a patient with Pentalogy of Cantrell, dextrocardia, perimembranous ventricular septal defect, secundum atrial septal defect, pulmonary valvular stenosis, and left ventricle diverticulum during the novel coronavirus disease 2019 pandemic.

Aorto-left ventricular tunnel: the necessity of early surgical intervention.

Aorto-left ventricular tunnel, also known as aorto-left ventricular fistula, is a rare CHD characterised by an abnormal connection between the aorta and the left ventricle. This article presents a comprehensive case report on aorto-left ventricular tunnel, encompassing clinical presentation, diagnostic approaches, treatment options, and outcomes, with a specific focus on the necessity of early surgical intervention.

Turkish validity and reliability study of Pediatric Quality of Life Inventory (PedsQL) 3.0 cardiac module for 8-18 years old children and parents.

Background: This study aimed to evaluate the Turkish validity and reliability of the 8-12- and 13-18-years child and parent forms of the Pediatric Quality of Life Inventory (PedSQL) 3.0 Cardiac Module.

Methods: This methodological study was conducted in children (8-18 years old) with cardiac disease and their parents.

The importance of aeroallergen sensitivity in children with cystic fibrosis.

Background: Cystic fibrosis (CF) is an inherited autosomal recessive disorder that causes chronic airway disease. In addition to genetic factors, environmental factors may affect the clinical phenotype of CF. In this study, the presence of aeroallergen sensitivity in our patients with CF and its effects on clinical findings are evaluated.

Interobserver reliability of a recently proposed semiological classification in psychogenic nonepileptic seizures in children.

Background: The aim of this study is to examine the semiological features of Psychogenic Nonepileptic Seizures (PNES) in children and to evaluate interobserver reliability (IR) of two different classifications. to identify the sources of any variance in agreement and to estimate the IR of the classification systems METHODS: Semiological features of 137 pediatric patients with PNES with and without epilepsy were analyzed. Two different, blinded observers evaluated these semiological features according to A.

Validation of the Turkish Version of the Catheterization Risk Score for Pediatrics.

Background: The aim of this study was to perform a validity analysis of the Turkish version of the Catheterization Risk Score for Pediatrics.

Methods: The study sample consisted of 419 pediatric patients who underwent cardiac catheterization. Patient risk factors and outcomes were collected using the revised (r) Catheterization Risk Score for Pediatric score (21 points) and Catheterization Risk Score for Pediatric score-20 point (Nykanen score).

Thoraco-abdominal duplication cyst of the jejunum presenting as respiratory distress in a young infant-a case report.

Thoraco-abdominal duplication cysts are rare congenital anomalies that can accompany vertebral and spinal cord pathologies and occur most frequently in the small intestines. Symptoms such as respiratory distress, dyspnea, tachypnea, cough, hemoptysis, cyanosis, vomiting and dysphagia may develop depending on the location. The cyst has several clinical and radiological dilemmas.

Breastfeeding and Infant Nutrition Knowledge, Attitude, and Practices of Parents.

Objective: This study aimed to evaluate the knowledge, attitude, and practices of parents about breastfeeding, complementary food, and infant nutrition who have healthy infants born at term and under 2 years of age.

Materials And Methods: This is a cross-sectional study conducted among the parents of infants who came for well-child visits to pediatric clinics of 4 hospitals. Healthy infants under 2 years of age and who had been born at term were interviewed.

Outcomes of Aortic Valve Replacement Via Right Anterior Minithoracotomy and Central Cannulation Versus Conventional Aortic Valve Replacement in Obese Patients.

Introduction: The minimally invasive implantation of aortic valve prosthesis via thoracotomy has numerous advantages and is comfortable, especially during the early postoperative period. Disadvantages of this method include peripheral vessel complications and groin infections. Central cannulation (direct aortic cannulation with superior vena cava cannulation) eliminates these drawbacks.

Central Diabetes Insipidus in Children and Adolescents: Twenty-Six Year Experience from a Single Centre.

Introduction: Paediatric cohorts of central diabetes insipidus (CDI) have shown varying prevalence for different causes of CDI. The objective of this study was to determine the causes of CDI and long-term outcome in children and adolescents from a Tertiary Paediatric Endocrinology unit.

Methods: The clinic database was searched to identify patients with CDI managed between 1993 and 2019.

Cardioplegia Application with A Hand-Squeezed Cardioplegia Bag. Is It Safe?

Objectives: Cardioplegia solutions have a role not only in arresting the heart but also in protecting the myocardium from ischemia. While antegrade cardioplegia is given by the heart-lung machine in many centers, it is given by a hand-squeezed bag in very few centers. The pressure of cardioplegia given antegrade from the heart-lung machine is certain (60-90 mmHg).

Inborn errors of metabolism in palliative care.

Background: In parallel with the increase in the number of children with life-limiting or life-threatening diseases, the need for and right to pediatric palliative care is also increasing. This study aimed to examine the metabolic diseases, evaluate the symptoms, and review the needs of children who were admitted to the pediatric palliative care service.

Methods: Following the approval of the local ethics committee (18/7/2019-107), the computer records of patients hospitalized in the pediatric palliative care service between December 1, 2018 and January 6, 2019 were reviewed retrospectively.

Short-term efficacy of ORS formulation and propranolol regimen in children with POTS.

Background: To evaluate the short-term effectiveness of reduced-osmolarity oral rehydration salt formulation (ORS) and propranolol in children diagnosed with postural orthostatic tachycardia syndrome (POTS) in head-up tilt testing (HUTT).

Methods: Children were admitted with symptoms of orthostatic intolerance (OI) occurring in a standing position and disappearing in the supine position. Patients with heart rate increments of ≥40bpm and symptoms of OI constituted the pediatric POTS group in HUTT.

Infective endocarditis developing early after percutaneous closure of a patent ductus arteriosus in a child using the Amplatzer Duct Occluder II.

A 10-month-old girl underwent patent ductus arteriosus closure with an Amplatzer Duct Occluder II. After 1 week, she was admitted to our emergency room with tachypnoea, fatigue, and fever. Consecutive blood cultures yielded vancomycin-sensitive Staphylococcus aureus.

The impact of 21-hydroxylase deficiency on cardiac repolarization changes in children with 21-hydroxylasedeficient congenital adrenal hyperplasia.

Korkmaz HA, Özdemir R, Küçük M, Karadeniz C, Meşe T, Özkan B. The impact of 21-hydroxylase deficiency on cardiac repolarization changes in children with 21-hydroxylase-deficient congenital adrenal hyperplasia. Turk J Pediatr 2019; 61: 228-235.

A rare vascular lesion of newborn: cutis marmorata telangiectatica congenital.

Cutis marmorata telangiectatica congenita is a rare, benign, sporadic and cutaneous vascular disease. A newborn female baby whose mother was aged 29 years and used propylthiouracil during pregnancy was hospitalized because of varicose lesions on the skin of the lower extremity and on the back, which were present at birth. It was observed that the lesions did not disappear, although appropriate room temperature was provided.

Optimal clinical management of children receiving dietary therapies for epilepsy: Updated recommendations of the International Ketogenic Diet Study Group.

Ketogenic dietary therapies (KDTs) are established, effective nonpharmacologic treatments for intractable childhood epilepsy. For many years KDTs were implemented differently throughout the world due to lack of consistent protocols. In 2009, an expert consensus guideline for the management of children on KDT was published, focusing on topics of patient selection, pre-KDT counseling and evaluation, diet choice and attributes, implementation, supplementation, follow-up, side events, and KDT discontinuation.

Effect of unconjugated hyperbilirubinemia on neonatal autonomic functions: evaluation by heart rate variability.

Background: Serum bilirubin levels beyond the physiological limits, may lead to alterations in autonomic regulation in a newborn infant. Heart rate variability (HRV), is a noninvasive and quantitative marker of the activity of the autonomic nervous system (ANS). To date, few studies have demonstrated the undesirable effects of severe unconjugated hyperbilirubinemia (UHB) on autonomic functions, and only one study has used HRV as a marker of the autonomic activity.

Severe Cutaneous Adverse Drug Reactions in Pediatric Patients: A Multicenter Study.

Background: The severe cutaneous adverse drug reactions (SCARs) are rare but could be life-threatening. These include drug reaction with eosinophilia and systemic symptoms (DRESS), Stevens-Johnson syndrome, toxic epidermal necrolysis (TEN), and acute generalized exanthematous pustulosis.

Objective: The purpose of this study was the evaluation of the clinical characteristics of patients with the diagnosis of SCARs.

Low Iron Stores in Otherwise Healthy Children Affect Electrocardiographic Markers of Important Cardiac Events.

Both an excess of iron and iron deficiency (ID) may lead to significant cardiac problems. Parameters that represent ventricular repolarization heterogeneity, like QT dispersion (QTd), corrected QT dispersion (QTcd), the interval between the peak and the end of the T wave (Tp-e), and Tp-e dispersion, have not been evaluated in otherwise healthy children with low iron levels before. Here we assessed the effects of low iron storage on P wave dispersion (PWd), QTd, Tp-e intervals, and Tp-e dispersion in otherwise healthy children.

Assessment of early atherosclerosis and left ventricular dysfunction in children with 21-hydroxylase deficiency.

Aim: We analysed 25 children with 21-hydroxylase deficiency who received glucocorticoid and/or mineralocorticoid treatment for at least 12 months to determine the effects of the disease and its treatment on vascular structures and ventricular function.

Methods: Twenty-five patients with 21-hydroxylase-deficient congenital adrenal hyperplasia (CAH) and 25 control subjects were enrolled into this observational, cross-sectional study. The patients were investigated in terms of fasting blood glucose and insulin; fasting serum lipid profile; serum 17-hydroxyprogesterone; dehydroepiandrosterone sulphate; androstenedione; and adrenocorticotropic hormone.

Global Deformation Parameters Response to Exercise in Adolescents with Repaired Tetralogy of Fallot.

Exercise stress echocardiography is rarely used in the follow-up of children with congenital heart disease following surgical repair. There are no data on global longitudinal strain (GLS) and global circumferential strain (GCS) during exercise in adolescents with repaired tetralogy of Fallot (TOF). The purpose of this prospective study was to investigate GLS and GCS using speckle tracking two-dimensional echocardiography (2D-STE) at rest and during exercise in adolescents with repaired TOF.

Parameters indicative of persistence of valvular pathology at initial diagnosis in acute rheumatic carditis: the role of albumin and CD19 expression.

Objective: The aim of this study is to define the predictors of chronic carditis in patients with acute rheumatic carditis (ARC).

Methods: Patients diagnosed with ARC between May 2010 and May 2011 were included in the study. Echocardiography, electrocardiography, lymphocyte subset analysis, acute phase reactants, plasma albumin levels, and antistreptolysin-O (ASO) tests were performed at initial presentation.

Red blood cell distribution width: can it be a predictive marker for long-term valvular involvement in children with acute rheumatic carditis?

Introduction: In this study, we aimed to evaluate Red blood cell distribution width (RDW) in patients with acute rheumatic carditis during the acute phase and after anti-inflammatory therapy.

Methods: Pediatric patients diagnosed with acute rheumatic carditis (ARC) between 2006 and 2014 and age- and sex-matched controls were retrospectively analyzed. At the time of diagnosis and after 2 months of medical therapy, we reviewed the obtained demographic features; echocardiographic data; complete blood count reports, including RDW; acute phase reactants, including C-reactive protein; and erythrocyte sedimentation rate values.