Addressing early childhood development and developmental difficulties in Turkey: a training programfor developmental pediatrics units.

Introduction: Addressing early childhood development (ECD) and developmental difficulties (DDs) in low and middle-income countries (LMICs) requires applicable and sustainable programs. Seven years after its implementation in 2010, we evaluated the Developmental Pediatrics Unit (DPU) Training Program in pediatric referral centers in Turkey.

Population And Methods: We applied crosssectional design and quantitative/qualitative methods to assess services, training, research and advocacy of the DPUs.

Association between vitamin D level and community-acquired late-onset neonatal sepsis.

Introduction: The objective was to determine the relationship between mother and infant vitamin D levels and late onset sepsis.

Population And Methods: Infants born > 37 weeks of gestational age who were hospitalized with the diagnosis of late-onset sepsis were enrolled to this prospective case control study. VitaminD levels of the infants and their mothers in the study and a control group were compared.

A mysterious case with abdominal pain and syndrome of inappropriate anti-diuretic hormone secretion.

Background: Acute intermittent porphyria (AIP) is a rare, hereditary, metabolic disease caused by a defect in heme biosynthesis. Hormonal changes may trigger porphyria attacks.

Case: Here we present a 17 -year- old adolescent refugee mother who applied to the pediatric emergency department with the complaint of diffuse abdominal pain at puerperium.

Acute myocarditis following varicella zoster infection in an immunocompetent adolescent: An uncommon complication.

Myocarditis is a serious complication of varicella zoster virus infection. A 15 year-old boy was admitted to the Emergency Department for chest pain, tachycardia and hypotension. An electrocardiogram showed sinus tachyicardia.

Patients eligible for modulator drugs: Data from cystic fibrosis registry of Turkey.

Background: A better understanding of cystic fibrosis transmembrane conductance regulator biology has led to the development of modulator drugs such as ivacaftor, lumacaftor-ivacaftor, tezacaftor-ivacaftor, and elexacaftor-tezacaftor-ivacaftor. This cross-sectional study evaluated cystic fibrosis (CF) patients eligible for modulator drugs.

Methods: Data for age and genetic mutations from the Cystic Fibrosis Registry of Turkey collected in 2018 were used to find out the number of patients who are eligible for modulator therapy.

Children with lymphoma presenting with hemophagocytic lymphohistiocytosis.

Background: < 7b > Hemophagocytic lymphohistiocytosis (HLH) may precede malignancy, in particular lymphomas and leukemias. However, the causative factors, appropriate treatment and the prognosis of this association is not established.

Case: Herein, we present two patients, one with nodular sclerosing Hodgkin lymphoma (HL) and concomitant Epstein-Barr virus (EBV) infection, and the other with anaplastic large cell lymphoma (ALCL), presented as malignancy associated HLH.

Clinical features and accompanying findings of Pseudo-Bartter Syndrome in cystic fibrosis.

Background: Pseudo-Bartter syndrome (PBS) is a rare complication of cystic fibrosis (CF) and there are limited data in the literature about it. We aimed to compare clinical features and accompanying findings of patients with PBS in a large patient population.

Methods: The data were collected from the Cystic Fibrosis Registry of Turkey where 1170 CF patients were recorded in 2017.

Mother-child interaction and the development status of children who have been accidentally poisoned.

Positive parent-child interaction, in particular bond between mother and child, is important for the mental and behavioral development of children. The aim of this study was to evaluate both mother-child interactions as well as the developmental status of children admitted to the pediatric emergency department with accidental poisoning using Parenting Interactions with Children: Checklist of Observations Linked to Outcomes tool (PICCOLO) and Denver Developmental Screening Test-II (DDST-II). Children between ages 1 to 5 years who were admitted to the emergency department with accidental poisoning were included in the study alongside a control group selected from healthy volunteers.

Monitoring Crimean-Congo haemorrhagic fever virus RNA shedding in body secretions and serological status in hospitalised patients, Turkey, 2015.

IntroductionCrimean-Congo haemorrhagic fever (CCHF) is a tick-borne disease in Africa, Asia, the Balkan peninsula, the south-east of Europe and the Middle East, with mortality rates of 3-30%. Transmission can also occur through contact with infected animals or humans.AimThis observational, prospective case series aimed to investigate detectable viral genomic RNA in whole-body fluids and antibody dynamics in consecutive daily samples of patients diagnosed with CCHF until discharge from hospital.

The effect of deferasirox on endocrine complications in children with thalassemia.

Endocrine system dysfunctions are the significant complications of excessive iron overload in beta thalassemia patients. The aim of this study was to evaluate the long-term effect of chelation with deferasirox on endocrine complications. The study group consisted of children with beta thalassemia who had been evaluated for the growth and pubertal development, bone metabolism, thyroid/parathyroid functions, glucose metabolism dysfunctions in the department of pediatric hematology of Ankara Dışkapı Child Health and Diseases Hematology Oncology Training And Research Hospital between 2009-2011 and reevaluated after deferasirox chelation therapy in 2018.

Heart rate variability in juvenile systemic lupus erythematosus patients.

Başaran Ö, Çetin İİ, Aydın F, Uncu N, Çakar N, Ekici F, Çelikel Acar B. Heart rate variability in juvenile systemic lupus erythematosus patients. Turk J Pediatr 2019; 61: 733-740.

Evaluation of Thiol/Disulfide Homeostasis in Pediatric Patients with Diabetic Ketoacidosis.

Aims And Objective: This study aimed to investigate the value of Thiol/Disulfide homeostasis in pediatric diabetic ketoacidosis patients suffering from type 1 diabetes mellitus.

Materials And Methods: This study featured children who were diagnosed with diabetic ketoacidosis and who were consecutively admitted to pediatric intensive care within one year of their diagnosis. Thiol/disulfide homeostasis was evaluated in 45 pediatric patients suffering from DKA, as well as 45 healthy controls of parallel gender and age.

The evaluation of malignancies in Turkish primary immunodeficiency patients; a multicenter study.

Background: There are no data regarding the prevalence of malignancies in patients with primary immunodeficiency (PID) in Turkey. Along with the prevalence of malignancy, we aimed to present the types of malignancy and define the underlying immune deficiency of the patients.

Method: Between the years 1992 and 2018, from five tertiary immunology clinics, fifty-nine patients with PID who developed malignancy were included.

Role of a second transplantation for children with acute leukemia following posttransplantation relapse: a study by the Turkish Bone Marrow Transplantation Study Group.

We examined outcomes of 51 pediatric patients with relapsed acute leukemia (AL) who underwent a second allogeneic hematopoietic stem cell transplantation (alloHSCT). After a median follow-up of 941 days (range, 69-2842 days), leukemia-free survival (LFS) and overall survival (OS) at 3 years were 26.6% and 25.

8 Gene Mutation Spectrum of 270 Patients with Hemophilia A: Identification of 36 Novel Mutations.

Objective: Hemophilia A (HA) is the most severe X-linked inherited bleeding disorder caused by hemizygous mutations in the gene. The aim of this study is to determine the mutation spectrum of the gene in a large HA cohort from Turkey, and then to establish a phenotype-genotype correlation.

Materials And Methods: All HA cases (270 patients) analyzed molecularly in the Ege University Pediatric Genetics Molecular Laboratory between March 2017 and March 2018 were included in this study.

The use of intravenous immunoglobulin therapy in a tertiary pediatric hospital.

To determine demographic features of patients provided intravenous immunoglobulin (IVIG) excluding replacement therapy in the inpatient services and to evaluate indications for IVIG, and side effects related to therapy. Methods: Patients who received IVIG therapy between January 2016 and August 2018 were retrospectively identified. The demographic features, diagnosis, IVIG dose, number of days they underwent IVIG therapy, and whether or not they experienced IVIG-related side effects were recorded.

Evaluation of hypersensitivity reactions to cancer chemotherapeutic agents in pediatric patients.

Background: Hypersensitivity reactions (HSRs) to chemotherapeutic agents have been increasingly documented.

Objective: The aim of this study was to investigate HSRs due to chemotherapeutics agents in childhood.

Methods: From January 2007 to June 2019, the patients who were treated for neoplastic diseases in our hospital were evaluated.

Inherited coagulation disorders in Turkish children: A retrospective, single-center cohort study.

Objective: This study aims to investigate the distribution, clinical characteristics and outcome of inherited coagulation disorders (ICD) in Turkish children.

Subjects And Methods: Data from all children (age<18 years) with ICD examined in our center were retrospectively reviewed.

Results: There were 403 children with ICD (233 males and 170 females) with a median age of four years at diagnosis.

Alpha methyl acyl CoA racemase deficiency: Diagnosis with isolated elevated liver enzymes.

Gündüz M, Ünal Ö, Küçükçongar-Yavaş A, Kasapkara Ç. Alpha methyl acyl CoA racemase deficiency: Diagnosis with isolated elevated liver enzymes. Turk J Pediatr 2019; 61: 289-291.

Neonatal form of biotin-thiamine-responsive basal ganglia disease. Clues to diagnosis.

Değerliyurt A, Gündüz M, Ceylaner S, Ünal Ö, Ünal S. Neonatal form of biotin-thiamine-responsive basal ganglia disease. Clues to diagnosis.

Subcortical gray matter changes in pediatric patients with new-onset juvenile myoclonic epilepsy.

Objective: The objective of the study was to identify the relationship between subcortical gray matter (GM) volumes and juvenile myoclonic epilepsy (JME).

Methods: We analyzed the brain magnetic resonance imaging (MRI) scans that were performed during the time of the diagnosis of epilepsy by using voxel-based morphometry (VBM) method. The volumetric three-dimensional sequence was used for structural investigation.

The negative predictive value of 5-day drug provocation test in nonimmediate beta-lactam allergy in children.

Background: Extending the drug provocation test (DPT) period is recommended for patients with suspected nonimmediate beta-lactam antibiotic (BLA) allergy and negative DPT. No consensus has been reached regarding the duration of prolonged provocation.

Objective: We aimed to determine the negative predictive value (NPV) of the 5-day extended DPT.