The quantity of unheated milk tolerated as a predictor of tolerance to baked milk.

Background: Tolerance of baked milk indicates a good prognosis in IgE-mediated cow's milk allergy.

Objective: The present study aims to investigate the predictors of baked milk tolerance, particularly the amount of milk tolerated in the first oral food challenge (OFC) test, in children with IgE-mediated cow's milk allergy.

Methods: The study included 35 cases who were diagnosed with IgE-mediated cow's milk allergy upon open OFC testing in the Pediatric Allergy Clinic.

Late diagnosis of leukocyte adhesion deficiency type II and Bombay blood type in a child: a rare case report.

Leukocyte adhesion deficiency type II (LAD II) is a rare, autosomal, recessive inherited immunodeficiency disease that induces frequent and recurrent infections, persistent leukocytosis, severe mental and growth retardation, and impaired wound healing. The Bombay blood group is a rare blood group phenotype that is characterised by the deficiency of H, A, and B antigens on the surface of red cells. LAD II and the Bombay blood group are always seen together, because both of them are associated with a global defect in the common pathway of fucose metabolism.

Can distal ureteral diameter measurement predict primary vesicoureteral reflux clinical outcome and success of endoscopic injection?

Objective: The objective of the study was to evaluate the predictive value of distal ureteral diameter ratio (UDR) on outcome of primary vesicoureteral reflux (VUR) and reflux resolution after endoscopic injection.

Patients And Methods: Three hundred eighty-three patients treated for primary VUR between January 2010 and October 2015 were retrospectively reviewed. The parameters analyzed were age at diagnosis, sex, grade and lateralite of VUR, complaints at admission (febrile urinary tract infection, antenatal hydronephrosis, family history),bladder-bowel dysfunction (urgency, incontinence, constipation), dimercaptosuccinic acid (DMSA) scintigraphy findings, follow-up period, clinical course (spontaneous resolution or surgical correction), time of spontaneous resolution, surgical treatment time and age, materials used for injection and success of endoscopic injection.

Pediatric emergency department visit characteristics of the patients on the ketogenic diet.

Background: The ketogenic diet (KD) has been frequently used for the patients with drug-resistant epilepsy in recent years. The management of these patients in emergency departments (EDs) has some difficulties due to the special needs of KD. We aimed to determine the characteristics and the management of the patients on the KD in the pediatric ED setting.

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.

A New Homozygous Mutation (c.393-394del TA/c.393-394del TA) in the NT5C3 Gene Associated With Pyrimidine-5'-Nucleotidase Deficiency: A Case Report.

Pyrimidine-5-nucleotidase (P5'N-1) deficiency is a rare nonspherocytic hemolytic anemia due to pyrimidine nucleotide deposition within erythrocytes. This rare erythrocyte disorder shows autosomal recessive inheritance with mutation of the pyrimidine-5'-nucleotidase gene, which is localized on 7p15-p14. Consanguinity of parents increases the probability of disease with novel mutations.

Reliability and accuracy of smartphones for paediatric infectious disease consultations for children with rash in the paediatric emergency department.

Objective: Smartphones and associated messaging applications have become the most common means of communication among health care workers and the general population. The aim of this study was to evaluate the reliability and accuracy of smartphones for the diagnosis of rash in children admitted to emergency departments during the night shift.

Methods: The images of the children who were admitted to the paediatric emergency department with rash were included in this study, and at least two images taken with smartphones by residents or paediatric infectious disease fellows were re-directed to the chief consultant of the Paediatric-Infectious Department via smartphone.

A 3-Year Retrospective Study of the Epidemiology of Acute Respiratory Viral Infections in Pediatric Patients With Cancer Undergoing Chemotherapy.

Background: Acute viral respiratory infections are common causes of febrile episodes in children. There are still limited data about distribution of acute viral respiratory infections in children with cancer.

Objective: The first aim of this study was to evaluate the viral etiology and seasonality of acute viral respiratory infection in pediatric patients with cancer in a 3-year study.

The role of cardiac troponin T in detection of cardiac damage and long term mortality in children with chronic renal disease.

Karadaş U, Özdemir Karadaş N, Bak M, Serdaroğlu E, Yılmazer MM, Meşe T. The role of cardiac troponin T in detection of cardiac damage and long term mortality in children with chronic renal disease. Turk J Pediatr 2019; 61: 873-878.

GPR56 homozygous nonsense mutation p.R271* associated with phenotypic variability in bilateral frontoparietal polymicrogyria.

Öncü-Öner T, Ünalp A, Porsuk-Doru İ, Ağılkaya S, Güleryüz H, Saraç A, Ergüner B, Yüksel B, Hız-Kurul S, Cingöz S. GPR56 homozygous nonsense mutation p.R271* associated with phenotypic variability in bilateral frontoparietal polymicrogyria.

Genetic Evaluation of Common Neurocutaneous Syndromes.

The neurocutaneous syndromes are a group of multisystem disorders that affect the skin and central nervous system. Neurofibromatosis 1, neurofibromatosis 2, tuberous sclerosis complex, and Sturge-Weber syndrome are the four major neurocutaneous disorders that mainly present in childhood. In this review, we discuss the clinical findings and genetic diagnosis, related genes/pathways and genotype-phenotype correlations of these four neurocutaneous syndromes.

The Emerging Resistance in Nosocomial Urinary Tract Infections: From the Pediatrics Perspective.

Background: Healthcare-associated infections results in increased health care costs and mortality. There are limited studies concerning the distribution of the etiologic agents and the resistance patterns of the microorganisms causing healthcare-associated urinary tract infections (HA-UTI) in pediatric settings.

Objectives: The aim of this study was to evaluate the distribution and antibiotic susceptibility patterns of pathogens causing HA-UTI in children.

Mutations in multiple components of the nuclear pore complex cause nephrotic syndrome.

Steroid-resistant nephrotic syndrome (SRNS) almost invariably progresses to end-stage renal disease. Although more than 50 monogenic causes of SRNS have been described, a large proportion of SRNS remains unexplained. Recently, it was discovered that mutations of NUP93 and NUP205, encoding 2 proteins of the inner ring subunit of the nuclear pore complex (NPC), cause SRNS.

Evaluation of ventricular arrhythmogenesis in children with acute rheumatic carditis.

Background: Recent studies have shown that the Tp-e interval, which on an electrocardiogram (ECG) is the interval between the peak and the end of the T wave, can be used as an index of transmural dispersion of ventricular repolarisation (TDR). Both Tp-e/QT and Tp-e/QTc ratios have also been used in that capacity. However, these novel repolarisation indices have not previously been studied in children with acute rheumaticcarditis (ARC).

Non-typhoidal bacteraemia in paediatric leukaemia patients.

Non-typhoidal (NTS) is an important pathogen that causes gastroenteritis, bacteraemia, and focal infections. Herein, we present our experience with bloodstream infections caused by in paediatric leukaemia patients, which has been reported for the first time in both Europe and the US. According to our research, NTS might be a cause of serious infections in paediatric haematology-oncology patients.

Genotype-phenotype correlation, gonadal malignancy risk, gender preference, and testosterone/dihydrotestosterone ratio in steroid 5-alpha-reductase type 2 deficiency: a multicenter study from Turkey.

Background: Studies regarding genetic and clinical characteristics, gender preference, and gonadal malignancy rates for steroid 5-alpha-reductase type 2 deficiency (5α-RD2) are limited and they were conducted on small number of patients.

Objective: To present genotype-phenotype correlation, gonadal malignancy risk, gender preference, and diagnostic sensitivity of serum testosterone/dihydrotestosterone (T/DHT) ratio in patients with 5α-RD2.

Materials And Methods: Patients with variations in the SRD5A2 gene were included in the study.

Antifungal consumption, indications and selection of antifungal drugs in paediatric tertiary hospitals in Turkey: Results from the first national point prevalence survey.

Objectives: The aim of this point prevalence survey was to evaluate the consumption, indications and strategies of antifungal therapy in the paediatric population in Turkey.

Methods: A point prevalence study was performed at 25 hospitals. In addition to general data on paediatric units of the institutes, the generic name and indication of antifungal drugs, the presence of fungal isolation and susceptibility patterns, and the presence of galactomannan test and high-resolution computed tomography (HRCT) results were reviewed.

Time-averaged hemoglobin values, not hemoglobin cycling, have an impact on outcomes in pediatric dialysis patients.

Background: During erythropoietin-stimulating agent (ESA) treatment, hemoglobin (Hb) levels usually fluctuate; this phenomenon is known as "Hb cycling (HC)." In this study, we aimed to evaluate the predictors of HC and its impact on left ventricular hypertrophy (LVH) as a patient-important outcome parameter in pediatric dialysis patients.

Methods: Records of patients followed up in nine pediatric nephrology centers between 2008 and 2013 were reviewed.

Validity and Reliability of the Turkish Version of DSM-5 Level 2 Anxiety Scale (Child Form for 11-17 Years and Parent Form for 6-17 Years).

Introduction: This study aimed to assess the validity and reliability of the Turkish Version of DSM-5 Level 2 Anxiety Scale's child and parent forms.

Methods: The scale was constructed by carrying out the translation and back translation of DSM-5 Level 2 Anxiety Scale. The study group consisted of a community and clinical sample.

A New Variant of PKLR Gene Associated With Mild Hemolysis may be Responsible for the Misdiagnosis in Pyruvate Kinase Deficiency.

Pyruvate kinase deficiency (PKD) is the most common glycolytic defect leading to hemolytic anemia. PKD is caused by the mutations in the PKLR gene; however, the detection of a decreased PK activity should be first measured for rapid diagnosis. We report here the case of a 1-year-old girl with mild hemolysis and PKD.

Platelet activation markers in children with congenital heart disease associated with pulmonary arterial hypertension.

Background: Mean platelet volume (MPV), platecrit, and platelet distribution width (PDW) are markers of platelet activation. Previous studies have found that platelet activation occurs in patients with pulmonary arterial hypertension. Platelet indices including MPV, PDW, and platecrit have not been studied in children with congenital heart disease associated pulmonary arterial hypertension (APAH-CHD) who survived and those who died.

Central line bundle for prevention of central line-associated bloodstream infection for totally implantable venous access devices (ports) in pediatric cancer patients.

Objective: The clinical impact of central line bundle programs for central line-associated bloodstream infections has been well demonstrated in intensive care units. However, the experience of central line bundle programs in totally implantable venous access devices (ports) in pediatric-hematology patients was limited.

Methods: A retrospective study was designed to compare and evaluate the clinical impact of implementing a central line bundle for a 2-year 5-month period, including 10 months of prebundle period, 11 months of central line bundle (that includes needleless split-septum devices), and finally 8 months of central line bundle period in which single-use prefilled flushing devices were added to the previous central line bundle.

A multicenter cross-sectional study to evaluate the clinical characteristics and nutritional status of children with cerebral palsy.

Background & Aims: This study was designed to assess clinical characteristics and nutritional status of pediatric outpatients with cerebral palsy (CP) and to determine prevalence of malnutrition based on physicians' clinical judgment and on anthropometric data in relation to percentile reference values.

Methods: A total of 1108 pediatric neurology outpatients (mean ± SEM age: 7.2 ± 0.