Cortical thinning in benign epilepsy with centrotemporal spikes (BECTS) with or without attention-deficit/hyperactivity (ADHD).

The aim of this study is to evaluate the abnormal cortical structures associated with newly diagnosed benign epilepsy with centrotemporal spikes (BECTS) patients and assessed the effects of comorbid attention-deficit/hyperactivity (ADHD) on these abnormalities. Newly diagnosed BECTS patients (n = 33, 23 males) and age-matched healthy controls (n = 48) were evaluated by surface and volumetric MRI. CAT12 toolbox (HYPERLINK "http://www.

Homozygous c.130-131 ins A (pW44X) mutation in the HAX1 gene as the most common cause of congenital neutropenia in Turkey: Report from the Turkish Severe Congenital Neutropenia Registry.

Background: Severe congenital neutropenia is a rare disease, and autosomal dominantly inherited ELANE mutation is the most frequently observed genetic defect in the registries from North America and Western Europe. However, in eastern countries where consanguineous marriages are common, autosomal recessive forms might be more frequent.

Method: Two hundred and sixteen patients with severe congenital neutropenia from 28 different pediatric centers in Turkey were registered.

Seizures in children with epilepsy and attention-deficit/hyperactivity disorder.

Background: Epilepsy is an important disorder that is sometimes accompanied by inattention problems. This study explored the features of seizures in children with epilepsy, with or without attention-deficit/hyperactivity disorder (ADHD).

Methods: Between January 2017 and January 2018, data records of children with epilepsy aged 5-18 years admitted to hospital were retrospectively collected.

Psychiatric morbidity and different treatment modalities in children with chronic kidney disease.

Introduction: Chronic kidney disease (CKD) is a potentially life-threatening condition leading to various psychosocial problems associated with different treatment modalities in addition to their medical advantages and disadvantages. The aim of this study was to evaluate the psychiatric morbidity in children with CKD in terms of different treatment modalities in comparison to healthy peers. In addition, parental attitudes and psychiatric symptoms in this group of mothers were examined.

Mothers of 0-3-year-old children with Down syndrome: Effects on quality of life.

Background: The aim of this study was to assess the quality of life (QoL) of mothers of children with Down syndrome (DS) and the factors related to it.

Methods: Seventy-five DS child-mother diads and 54 healthy child-mother diads were included in this study. The brief World Health Organization Quality of Life scale - Turkish version (WHOQOL-BREF-TR), Symptom Check List-90-Revised (SCL-90-TR), Beck Depression Inventory, Beck Anxiety Inventory and Maslach Burnout Inventory (MBI) were completed by mothers to assess maternal QoL, burnout, anxiety, depression and general psychiatric symptoms.

Novel mutations of SAR1B gene in four children with chylomicron retention disease.

Background: Intestinal lipid malabsorption, resulting from an impaired formation or secretion of chylomicrons and associated with severe hypobetalipoproteinemia (HBL), may be due to biallelic mutations in APOB (homozygous FHBL type-1), MTTP (abetalipoproteinemia), or SAR1B (chylomicron retention disease).

Objective: We investigated four children, each born from consanguineous parents, presenting with steatorrhea, malnutrition, accumulation of lipids in enterocytes, and severe hypocholesterolemia with an apparent recessive transmission.

Methods: We sequenced a panel of genes whose variants may be associated with HBL.

Abatacept as a Long-Term Targeted Therapy for LRBA Deficiency.

Background: LPS-responsive beige-like anchor (LRBA) deficiency presents with susceptibility to infections, autoimmunity, and lymphoproliferation. The long-term efficacy of cytotoxic T-lymphocyte-associated antigen 4-immunoglobulin (abatacept) as targeted therapy for its immune dysregulatory features remains to be established.

Objective: To determine the clinical and immunologic features of LRBA deficiency and long-term efficacy of abatacept treatment in controlling the different disease manifestations.

Bone Marrow Necrosis in a Patient Following Blinatumomab Therapy.

Bone marrow necrosis (BMN) is an extremely rare condition characterized by necrosis of the myeloid tissue and medullary stroma leaving an amorphous eosinophilic background and ill-defined necrotic cells in the hematopoietic bone marrow. Several conditions are associated with BMN, including sickle cell disease, metastatic carcinoma, and hematologic malignancies. It is also associated with the use of antineoplastic drugs, such as fludarabine, interferon alpha, and imatinib.

Subclinical Myocardial Dysfunction Demonstrated by Speckle Tracking Echocardiography in Children with Euthyroid Hashimoto’s Thyroiditis.

Objective: Thyroid hormones have an important role in the regulation of the cardiovascular system. The aim of this study was to investigate the presence of subclinical myocardial dysfunction in children with euthyroid Hashimoto’s thyroiditis (eHT) without evident heart disease using tissue doppler imaging (TDI) and speckle tracking echocardiography (STE) methods.

Methods: TDI and STE were peformed in 50 children with eHT and in 35 healthy children.

Management of acquired aplastic anemia in children : A single center experience.

Acquired aplastic anemia (AAA) is a rare and potentially life threatening disorder. We retrospectively compared the outcomes of 29 children with AAA who received immunosuppressive therapy (IST) or underwent hematopoietic stem cell transplantation (HSCT). Median age at diagnosis was 9.

Characteristics of Turkish children with Type 2 diabetes at onset: a multicentre, cross-sectional study.

Aims: To describe the baseline clinical and laboratory findings and treatment modalities of 367 children and adolescents diagnosed with Type 2 diabetes in various paediatric endocrinology centres in Turkey.

Methods: A standard questionnaire regarding clinical and laboratory characteristics at onset was uploaded to an online national database system. Data for 367 children (aged 6-18 years) newly diagnosed with Type 2 diabetes at 37 different paediatric endocrinology centres were analysed.

Recent developments in drug hypersensitivity in children.

: Drug allergy is an important public health problem that causes 0.4-10.3% of hospital admissions in children.

The Burden of Burnout Syndrome in Pediatric Intensive Care Unit and Pediatric Emergency Department: A Multicenter Evaluation.

Objective: The objective of this study was to detect variables associated with burnout syndrome (BS) in pediatric intensive care units (PICUs) and pediatric emergency medicine departments (PEDs) in high-volume centers from different parts of Turkey.

Methods: An observational, cross-sectional multicenter study was performed. The Maslach Burnout Inventory scale was administered to all of health care providers working in PICUs and PEDs.

Distinguishing prodromal stage of bipolar disorder and early onset schizophrenia spectrum disorders during adolescence.

Prodromal symptoms of bipolar disorder (BD) and early onset schizophrenia spectrum disorder (EOSSD) overlap. To date, there has been no study directly comparing the prodromal stage of both disorders. Thus, the current study is aimed at determining which prodromal symptom clusters differentiate BD and EOSSD.

The effects of parental and peer factors on psychiatric symptoms in adolescents with obesity.

Purpose: The aim of this study was to examine the relationships among psychiatric symptoms, effect of beliefs and attitudes of parents about obese people and victimization or bullying in obese adolescents.

Methods: The study group included 110 obese or overweight adolescents and 55 adolescents of normal weight as the control group. All adolescents completed the Brief Symptom Inventory (BSI) and Traditional Bullying Scale.

Impact of Prophylaxis on Bone Mineral Metabolism in Children With Hemophilia.

In this study, we aimed to investigate changes in calcium (Ca) metabolism in hemophilia patients (PWH). We also aimed to investigate the importance of diagnosis and treatment of factors impairing calcium metabolism and the significance of early diagnosis and prophylaxis with respect to these subjects. For all patients, serum calcium, phosphorus, alkaline phosphatase, 25 hydroxy vitamin D (25-OHD), parathormone (PTH), and calcitonin levels were evaluated.

Hepatosplenic Fungal Infections in Children With Leukemia-Risk Factors and Outcome: A Multicentric Study.

Background: Invasive fungal infections, including hepatosplenic fungal infections (HSFI), cause significant morbidity and mortality in children with leukemia. There are not enough data to support for the best approach to diagnosis of HSFI in children, nor for the best treatment.

Procedure: In this multicentric study, we assessed the demographic data, clinical and radiologic features, treatment, and outcome of 40 children with leukemia and HSFI from 12 centers.

Refugee children with beta-thalassemia in Turkey: Overview of demographic, socioeconomic, and medical characteristics.

Aim: Since the beginning of the Syrian civil war, more than 3.5 million Syrians have been under temporary protection status in Turkey. Because beta-thalassemia (BT) is a prevalent disorder in the Mediterranean countries, we decided to estimate the prevalence of and make an overview of the demographic, socioeconomic, medical characteristics, and healthcare problems of refugee children with BT.

Weaning practices in phenylketonuria vary between health professionals in Europe.

Background: In phenylketonuria (PKU), weaning is considered more challenging when compared to feeding healthy infants. The primary aim of weaning is to gradually replace natural protein from breast milk or standard infant formula with solids containing equivalent phenylalanine (Phe). In addition, a Phe-free second stage L-amino acid supplement is usually recommended from around 6 months to replace Phe-free infant formula.

Pictures portrayed by children with migraine with aura: a Turkish case series.

Background: Migraine is one of the most debilitating disorders with its chronic nature seen in childhood characterized by episodic bilateral or unilateral throbbing pain on the head that strikes at any time. It is divided into two categories as with or without aura and is diagnosed according to the 2013 International Headache Society criteria.

Aim And Method: This study aims to present the pictures depicted by adolescents aged 14-18 who were diagnosed with migraine with visual aura by Ankara Training and Research Hospital Child Neurology Department.

Behavioral problems of preschool children with new-onset epilepsy and one-year follow-up - A prospective study.

Objectives: Childhood emotional/behavioral problems in children with epilepsy have been reported to be higher compared with those with typical development or with other nonneurologic health conditions. Increasing interest towards understanding these behavioral comorbidities is reflected in literature. However, longitudinal investigations regarding the course of behavioral problems in children with newly diagnosed epilepsy and normal development are rare, and majority of them involve school-aged children.

Use of computed tomography and diffusion weighted imaging in children with ventricular shunt.

Purpose: To evaluate the indications, number, and imaging results of brain computed tomography (CT) and diffusion weighted imaging (DWI) in children with ventriculoperitoneal shunt, to estimate the radiation dose, and to evaluate the effectiveness of DWI.

Methods: This retrospectively study included 54 consecutive patients (boys/girls = 30/24, mean age, 3 ± 4.1 years) with shunt that were placed due to congenital abnormalities-hypoxic ischemic encephalopathy between January 2015 and March 2018.