Evaluation of dynamic/thiol disulphide balance and ischaemia modified albumin in children with trauma.

Aim: Trauma is the most common cause of death in childhood. Tissue damage, ischaemia-reperfusion injury and inflammatory response are mainly responsible for increasing free oxygen radicals. In this study, we aimed to investigate the use of thiol-disulphide and ischaemia-modified albumin levels as a diagnostic laboratory parameter in trauma children.

Severe Rare Bleeding Disorders: A Single-center Experience.

Although rare bleeding disorders (RBDs) are not common diseases, they are important for life-threatening bleedings and prophylaxis approaches, especially in severe forms. In this retrospective study, the authors have analyzed data from children with severe RBDs who were examined at the center over a period of 10 years to describe the distribution, clinical features, treatment patterns, and outcome of severe RBDs in patients. Data from all children (age under 18 y) with RBDs who were examined in the center between 2005 and 2015 were retrospectively reviewed.

Biopsy-proven Henoch-Schönlein purpura nephritis: a single center experience.

Background: Knowledge on normal progress and treatment of Henoch-Schönlein purpura nephritis (HSPN) is limited. This study reviews outcome, clinical, pathological, and therapeutic factors affecting the prognosis of HSPN patients.

Methods: Forty-nine children with biopsy-confirmed HSPN diagnosed between September 2008 and 2018 were included.

Cross-sectional study: long term follow-up care for pediatric cancer survivors in a developing country, Turkey: current status, challenges, and future perspectives.

Aim: The main purpose of this study is to determine the current status of long-term follow-up (LTFU) for childhood cancer survivors and the challenges of LTFU for pediatric cancer survivors at pediatric oncology institutions in Turkey.

Material And Methods: A questionnaire was e-mailed to the directors of 33 pediatric oncology centers (POCs) registered in the Turkish Pediatric Oncology Group (TPOG). Of these 33 active TPOG institutions, 21 participated in the study and returned their completed questionnaires.

Pontine Tegmentum Lesions Accompanying Myelitis During an Enterovirus Outbreak: Differential Diagnosis and Outcome.

Aim: To investigate etiology and prognostic significance of pontine tegmentum lesions accompanying a cluster of acute flaccid myelitis.

Method: We retrospectively examined patients from 6 centers in Turkey who manifested encephalitis or myelitis associated with dorsal pontine lesions on magnetic resonance imaging (MRI) between July 2018 and February 2019.

Results: Twenty-two patients were evaluated.

A Safe and Minimally Invasive Method for Thoracoscopic Lung Biopsy in Interstitial Lung Disease.

Interstitial lung disease (ILD) represents a spectrum of lung diseases that may contain different levels of fibrosis and inflammation. Sometimes, the clinical picture and imaging studies are insufficient to determine the diagnosis and lung biopsy becomes crucial, which may be performed in a thoracoscopic or open manner. The aim of this study was to present a new minimally invasive biopsy method that seems to be technically easy, safe, and inexpensive.

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.

Assessment of novel biomarkers: sTREM-1, pentraxin-3 and pro-adrenomedullin in the early diagnosis of neonatal early onset sepsis.

Background: Early onset bacterial sepsis in neonates (EOS) is recognized as an important health condition. Early diagnosis is crucial. However, blood culture results are released in 48-72 hours.

Transinguinal laparoscopic evaluation of contralateral side during unilateral inguinal hernia repair for children.

Background: Inguinal hernia repair is a common procedure in daily pediatric surgical practice.

Objectives: The present study was planned to find out whether transinguinal laparoscopic exploration (TILE) of the contralateral groin is effective in reducing the need of operation for contralateral metachronous inguinal hernia (CMIH) in children.

Study Design: Charts of 1103 children who underwent inguinal hernia repair between 2006 and 2016 were retrospectively analyzed.

Exome Sequencing of a Primary Ovarian Insufficiency Cohort Reveals Common Molecular Etiologies for a Spectrum of Disease.

Context: Primary ovarian insufficiency (POI) encompasses a spectrum of premature menopause, including both primary and secondary amenorrhea. For 75% to 90% of individuals with hypergonadotropic hypogonadism presenting as POI, the molecular etiology is unknown. Common etiologies include chromosomal abnormalities, environmental factors, and congenital disorders affecting ovarian development and function, as well as syndromic and nonsyndromic single gene disorders suggesting POI represents a complex trait.

The Frequency of Lysosomal Acid Lipase Deficiency in Children With Unexplained Liver Disease.

Objectives: Evidence suggests that lysosomal acid lipase deficiency (LAL-D) is often underdiagnosed because symptoms may be nonspecific. We aimed to investigate the prevalence of LAL-D in children with unexplained liver disease and to identify demographic and clinical features with a prospective, multicenter, cross-sectional study.

Methods: Patients (aged 3 months-18 years) who had unexplained transaminase elevation, unexplained hepatomegaly or hepatosplenomegaly, obesity-unrelated liver steatosis, biopsy-proven cryptogenic fibrosis and cirrhosis, or liver transplantation for cryptogenic cirrhosis were enrolled.

Validation of Renal Risk Score Models for Coronary Artery Bypass Surgery in Diabetic Patients.

Background: Coronary artery bypass grafting is applicable with very low mortality and morbidity rates around the world. However, exposure to even one of the risk factors increases mortality and morbidity significantly. There are three acute kidney injury definitions, and classification methods are applicable (Kidney Disease: Improving Global Outcomes (KDIGO); Risk, Injury, Failure, Loss of kidney function and End-stage kidney disease (RIFLE);" (for accuracy) and Acute Kidney Injury Network (AKIN)), for understanding and grading of renal impairment.

Investigation of an algorithm for anti HCV EIA reactivity in blood donor screening in Turkey in the absence of nucleic acid amplification screening.

Purpose: In this study we aimed to propose an algorithm for initial anti HCV EIA reactive blood donations in Turkey where nucleic acid amplification tests are not yet obligatory for donor screening.

Methods: A total of 416 anti HCV screening test reactive donor samples collected from 13 blood centers from three cities in Turkey were tested in duplicate by Ortho HCV Ab Version 3.0 and Radim HCV Ab.

A case of neuroblastoma in DICER1 syndrome: Chance finding or noncanonical causation?

DICER1 syndrome is an inherited disorder associated with at least a dozen rare, mainly pediatric-onset tumors. Its characterization remains incomplete. Some studies suggested that neuroblastoma (NB) may be involved in this syndrome.

The Impact of Iron Overload in Acute Leukemia: Chronic Inflammation, But Not the Presence of Nontransferrin Bound Iron is a Determinant of Oxidative Stress.

In the literature, studies on the oxidant effects of nontransferrin bound iron [NTBI (eLPI assay)] during chemotherapy of acute lymphoblastic leukemia and acute myeloblastic leukemia are lacking. We established NTBI and oxidative stress determinants (OSD), iron parameters, high-sensitive C-reactive protein (hs-CRP) levels, liver tests, cumulative chemotherapeutic doses, and transfused blood in 36 children with acute leukemia throughout chemotherapy. These parameters were determined at the beginning and end of chemotherapy blocks (11 time points) and in 20 healthy children using enzyme-linked immunosorbent assay, and colorimetric and fluorometric enzymatic methods.

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.

CD55 Deficiency, Early-Onset Protein-Losing Enteropathy, and Thrombosis.

Background: Studies of monogenic gastrointestinal diseases have revealed molecular pathways critical to gut homeostasis and enabled the development of targeted therapies.

Methods: We studied 11 patients with abdominal pain and diarrhea caused by early-onset protein-losing enteropathy with primary intestinal lymphangiectasia, edema due to hypoproteinemia, malabsorption, and less frequently, bowel inflammation, recurrent infections, and angiopathic thromboembolic disease; the disorder followed an autosomal recessive pattern of inheritance. Whole-exome sequencing was performed to identify gene variants.

Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children.

We investigated the value of genetic, histopathologic, and early treatment response information in prognosing long-term renal outcome in children with primary steroid-resistant nephrotic syndrome. From the PodoNet Registry, we obtained longitudinal clinical information for 1354 patients (disease onset at >3 months and <20 years of age): 612 had documented responsiveness to intensified immunosuppression (IIS), 1155 had kidney biopsy results, and 212 had an established genetic diagnosis. We assessed risk factors for ESRD using multivariate Cox regression models.

Cardiac MRI and 3D contrast-enhanced MR angiography in pediatric and young adult patients with Turner syndrome.

Background/aim: This study aimed to describe the spectrum and frequency of cardiovascular abnormalities in pediatric and young adult patients with Turner syndrome (TS) using cardiac MRI and MR angiography.

Materials And Methods: This prospective study consisted of 47 female patients of pediatric age and young adults with a karyotypically confirmed diagnosis of TS. All patients underwent cardiac MRI and contrast-enhanced MR angiography.

Clinical and epidemiological characteristics of children with germ cell tumors: A single center experience in a developing country.

İncesoy-Özdemir S, Ertem U, Şahin G, Bozkurt C, Yüksek N, Ören AC, Balkaya E, Alkan A. Clinical and epidemiological characteristics of children with germ cell tumors: A single center experience in a developing country. Turk J Pediatr 2017; 59: 410-417.