Characteristics of pediatric multiple sclerosis: The Turkish pediatric multiple sclerosis database.

Objective: To document the clinical and paraclinical features of pediatric multiple sclerosis (MS) in Turkey.

Methods: Data of MS patients with onset before age 18 years (n = 193) were collected from 27 pediatric neurology centers throughout Turkey. Earlier-onset (<12 years) and later-onset (≥12 years) groups were compared.

Idiopathic Pulmonary Hemosiderosis Mimicking Iron Deficiency Anemia: A Delayed Diagnosis?

Idiopathic pulmonary hemosiderosis (IPH) is an uncommon chronic disorder in children. It is characterized by recurrent pulmonary hemorrhage and may result in hemoptysis and pulmonary insufficiency. The most common hematologic manifestation of IPH is iron deficiency anemia.

Clinical presentations, metabolic abnormalities and end-organ complications in patients with familial partial lipodystrophy.

Objective: Familial partial lipodystrophy (FPLD) is a rare genetic disorder characterized by partial lack of subcutaneous fat.

Methods: This multicenter prospective observational study included data from 56 subjects with FPLD (18 independent Turkish families). Thirty healthy controls were enrolled for comparison.

Does atopy affect the course of viral pneumonia?

Background: The presence of atopy is considered as a risk factor for severe respiratory symptoms in children. The objective of this study was to examine the effect of atopy on the course of disease in children hospitalised with viral pneumonia.

Methods: Children between the ages of 1 and 6 years hospitalised due to viral pneumonia between the years of 2013 and 2016 were included to this multicentre study.

Metabolic control and periodontal treatment decreases elevated oxidative stress in the early phases of type 1 diabetes onset.

Objective: Recently, increasing concern has been focused on the contribution of oxidative stress in the pathology of periodontal disease and diabetes mellitus. Firstly, the present study aimed to analyze gingival crevicular fluid (GCF), salivary, and serum oxidative status in children with type 1 diabetes mellitus (T1DM) at diagnosis and systemically healthy children with and without gingivitis. Additionally, the diabetic patients were reevaluated after diabetes and periodontal treatment.

Infantile-onset thiamine responsive megaloblastic anemia syndrome with SLC19A2 mutation: a case report.

Background: Thiamine-responsive megaloblastic anemia syndrome (TRMA), also known as Rogers syndrome, is characterized by megaloblastic anemia, sensorineural hearing loss, and diabetes mellitus. Disturbances of the thiamine transport into the cells results from homozygous or compound heterozygous mutations in the SLC19A2 gene.

Case Presentation: We report a girl which presented with sensorineural deafness treated with a hearing prosthesis, insulin requiring diabetes, macrocytic anemia, treated with thiamine (100 mg/day).

A Different SLC2A1 Gene Mutation in Glut 1 Deficiency Syndrome: c.734A>C.

Background: Glucose transporter type 1 deficiency syndrome is the result of impaired glucose transport into the brain. Patients with glucose transporter type 1 syndrome may present with infantile seizures, developmental delay, acquired microcephaly, spasticity and ataxia.

Case Report: Here, we report a rare case of glucose transporter type 1 deficiency syndrome caused by a different pathogenic variant in a 10-day-old neonate who presented with intractable seizures and respiratory arrest.

Using cardiovascular imaging modalities to determine cardiac disorders before starting sports activities.

Objective: We re-examined children who had previously been declared eligible to participate in competitive sports activities for cardiac disorders, using cardiac investigation protocol.

Methods: Total of 250 children (224 males [89.6%], and 26 females [10.

Effect of the timing of dialysis initiation on left ventricular hypertrophy and ınflammation in pediatric patients.

Background: The optimal time for dialysis initiation in adults and children with chronic kidney disease remains unclear. The aim of this study was to evaluate the impact of dialysis timing on different outcome parameters, in particular left ventricular (LV) morphology and inflammation, in pediatric patients receiving peritoneal dialysis and hemodialysis.

Methods: The medical records of pediatric dialysis patients who were followed-up in nine pediatric nephrology centers in Turkey between 2008 and 2013 were retrospectively reviewed.

Neurodevelopmental Outcome of Children with Congenital Hypothyroidism Diagnosed in a National Screening Program in Turkey.

Objective: To study the factors affecting a neurodevelopmental status of children with congenital hypothyroidism, diagnosed on national screening program.

Methods: The study was performed in the Pediatric Endocrinology Department of Dr. Behcet Uz Children's Hospital between May 2012 and May 2013.

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.

The First Use of Pralidoxime in a Child With Rivastigmine Poisoning.

Unlabelled: The aim of this report is to describe the successful use of pralidoxime in a pediatric patient who accidentally ingested 12 mg of rivastigmine and presented to the emergency department with weakness, drowsiness, hyporeactivity to environmental stimuli, and full cholinergic syndrome.

Case: The patient presented to the emergency department 2 hours after a suspected ingestion of rivastigmine. He was sleepy but oriented and cooperative, hypotonic, and hyporeflexic and has a Glasgow Coma Scale score of 13 (E3M6V4).

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.

Diagnostic values for egg white specific IgE levels with the skin prick test in Turkish children with egg white allergy.

Background: The diagnostic values for the skin prick test (SPT) diameters and egg white-specific IgE (EW-sIgE) levels that will allow us to predict the result of the oral food challenge test (OFC) in the diagnosis of egg white allergy vary by the community where the study is carried out.

Objective: This study aimed to determine the diagnostic values of SPT and EW-sIgE levels in the diagnosis of egg white allergy.

Methods: 59 patients followed with the diagnosis of egg allergy September 2013 to September 2015 were included in our retrospective cross-sectional study.

Tolerance development in food protein-induced allergic proctocolitis: Single centre experience.

Background: Food protein-induced allergic proctocolitis (FPIAP) is characterised by inflammation of the distal colon in response to one or more food proteins. It is a benign condition of bloody stools in a well-appearing infant, with usual onset between one and four weeks of age.

Objective: Our objective was to examine the clinical properties of patients with FPIAP, tolerance development time as well as the risk factors that affect tolerance development.

Treating an Adolescent with Long QT Syndrome for Bipolar Disorder: A Case Presentation.

Objectives: Long QT syndrome (LQTS) is described as the development of sudden syncope attacks or death as a result of ventricular tachycardia (VT) episodes that might be observed as elongated QT interval in electrocardiography (ECG). Implantable Cardioverter Defibrillator (ICD) is recommended as first-line treatment for the condition in guidelines. We aimed to present an adolescent recently diagnosed with Bipolar Disorder (BD) who had LQTS that was treated with ICD, discussing her follow up and treatment along with relevant literature.

Higher-Than-Conventional Subcutaneous Regular Insulin Doses Following Diabetic Ketoacidosis in Children and Adolescents.

Objective: To evaluate the effect of initial insulin dosage on glycemic control in the first 48 hours of subcutaneous regular insulin therapy after resolution of diabetic ketoacidosis (DKA).

Methods: Records of patients with DKA hospitalized in the past 3 years [n=76, median age=10.0 (6.

Effect of socioeconomic status on allergic diseases and atopy in school children.

Sipahi S, Nacaroğlu HT, Can D, Günay İ, Ünsal-Karkıner CŞ, Kamalı H, Özdemir A, Günay T. Effect of socioeconomic status on allergic diseases and atopy in school children. Turk J Pediatr 2017; 59: 670-677.

Child with RET proto-oncogene codon 634 mutation.

İnce D, Demirağ B, Ataseven E, Oymak Y, Tuhan H, Karakuş OZ, Hazan F, Abacı A, Özer E, Mutafoglu K, Olgun N. Child with RET proto-oncogene codon 634 mutation. Turk J Pediatr 2017; 59: 590-593.

Assessment of sleep in pediatric cancer patients.

İnce D, Demirağ B, Karapınar TH, Oymak Y, Ay Y, Kaygusuz A, Töret E, Vergin C. Assessment of sleep in pediatric cancer patients. Turk J Pediatr 2017; 59: 379-386.

A child with subcorneal pustular dermatosis responded to IVIG treatment (Sneddon-Wilkinson disease).

Subcorneal pustular dermatosis (SPD) is a rare, chronic, recurrent dermatosis characterised by sterile pustules. It develops mainly in middle-aged or elder women, but is also rarely seen in children. The exact aetiology of the disease is unknown.

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.

The new classification system for inner ear malformations: the INCAV system.

Conclusions: The proposed INCAV system standardizes reporting of inner ear malformations; gives adequate information about the structures of inner ear; defines the ears which could not be classified before; and helps in the selection of the ear as the cochlear implant candidate. Also it is easy-to-use for radiologists, and useful to the referring otolaryngologists.

Objective: This study was conducted to explore a more specific, definitive classification system which was based on radiological criteria for inner ear malformations.