Mid-term results and late events after the Fontan operation: A single-center experience.

Background: This study aimed to review our institutional experience with the Fontan operation, the adverse severe events we encountered during mid-term follow-up, and the associated risk factors.

Methods: In the study, the medical records of 40 patients (22 males, 18 females) who underwent Fontan operation between August 1993 and August 2020 were retrospectively reviewed. The patients were followed up for at least six months.

COVID-19 in pediatric nephrology centers in Turkey.

Background/aim: There is limited data on COVID-19 disease in children with kidney disease. We aimed to investigate the characteristics and prognosis of COVID-19 in pediatric nephrology patients in Turkey.

Materials And Methods: This was a national, multicenter, retrospective cohort study based on an online survey evaluating the data between 11 March 2020 and 11 March 2021 as an initial step of a detailed pediatric nephrology COVID-19 registry.

Pediatric mixed connective tissue disease versus other overlap syndromes: a retrospective multicenter cohort study.

Pediatric mixed connective tissue disease (MCTD) is a subgroup of overlap syndromes. We aimed to compare the characteristics and outcomes in children with MCTD and other overlap syndromes. All MCTD patients met either Kasukawa or Alarcon-Segovia and Villareal criteria.

Evaluation of Baseline Characteristics and Prognostic Factors in Multisystemic Inflammatory Syndrome in Children: Is It Possible to Foresee the Prognosis in the First Step?

Background: Two years after the first cases, critical gaps remain in identifying prognostic factors in multisystem inflammatory syndrome in children (MIS-C). Methods: This retrospective study included 99 patients with MIS-C hospitalized between August 2020 and March 2022 in a pediatric tertiary center. The patients were divided into two groups according to clinical severity (low- and high-risk).

Number of Episodes Can Be Used as a Disease Activity Measure in Familial Mediterranean Fever.

Objective: To evaluate the number of episodes in the past 12 months as an indicator of the overall disease activity status in Familial Mediterranean fever (FMF).

Methods: In this cross-sectional study, patients were recruited from tertiary pediatric hospitals. Demographic data, main clinical symptoms of the episodes, treatment modalities, and genetic mutations were recorded.

Long-Term Follow-Up of Patients with a Diagnosis of Posterior Reversible Encephalopathy Syndrome.

Objective: The essential characteristics of posterior reversible encephalopathy syndrome (PRES) are the presence of acute onset neurologic symptoms, focal vasogenic edema at neuroimaging, and reversible clinical and/or radiologic findings. This study aimed to evaluate the clinical findings, causes, radiologic findings, and prognoses of patients with PRES.

Methods: Patients with PRES confirmed with clinical and radiologic findings by a pediatric neurologist were evaluated retrospectively.

Hematological involvement in pediatric systemic lupus erythematosus: A multi-center study.

Systemic lupus erythematosus (SLE) may present with features of several systems, including hematological manifestations. In this study, we aimed to evaluate the characteristics of hematological involvement and assess possible associations and correlations in pediatric SLE patients. This is a retrospective multi-center study.

Next Generation Sequencing Based Multiplex Long-Range PCR for Routine Genotyping of Autoinflammatory Disorders.

Background: During the last decade, remarkable progress with massive sequencing has been made in the identification of disease-associated genes for AIDs using next-generation sequencing technologies (NGS). An international group of experts described the ideal genetic screening method which should give information about SNVs, InDels, Copy Number Variations (CNVs), GC rich regions. We aimed to develop and validate a molecular diagnostic method in conjunction with the NGS platform as an inexpensive, extended and uniform coverage and fast screening tool which consists of nine genes known to be associated with various AIDs.

Evaluation of the thicknesses of cartilage and enthesis in familial Mediterranean fever and enthesitis-related arthritis.

Objective: Subclinical inflammation is still a controversial issue in inflammatory diseases. There is no reliable, easy, and cheap inflammation marker in daily clinical practices currently. This study aims to predict clinical remission using cartilage and tendon thicknesses.

The Performances of the ACR 1997, SLICC 2012, and EULAR/ACR 2019 Classification Criteria in Pediatric Systemic Lupus Erythematosus.

Objective: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease. The American College of Rheumatology (ACR) 1997, Systemic Lupus International Collaborating Clinics (SLICC) 2012, and European League Against Rheumatism (EULAR)/ACR 2019 SLE classification criteria are formed based on data mainly from adult patients. We aimed to test the performances of the SLE classification criteria among pediatric patients with SLE.

Evaluation of familial Mediterranean fever patients concomitant with juvenile spondyloarthropathy.

Objectives: Familial Mediterranean fever (FMF) may present with various concomitant diseases. This study aims to evaluate the clinical characteristics of patients with FMF with Juvenile Spondyloarthropathy (jSpA).

Method: Thirty-two patients diagnosed with FMF/jSpA, sixty-four with FMF, and fifty-four with jSpA were included in this retrospective study.

Refractory and Fatal Presentation of Severe Autoimmune Hemolytic Anemia in a Child With the DNASE1L3 Mutation Complicated With an Additional DOCK8 Variant.

Various autoimmune diseases may be associated with primary immune deficiencies. We reported a case with a loss-of-function mutation in DNASE1L3, a gene described previously in families with systemic lupus erythematosus. In addition, the patient showed a novel homozygous missense variant in DOCK8, a gene known to be responsible for the hyper-IgE recurrent infection syndrome (HIES).

Patient Outcomes of Henoch-Schönlein Purpura Nephritis According to the New Semiquantitative Classification.

A new semiquantitative classification (SQC) for pediatric Henoch-Schönlein nephritis (HSN) was defined recently. The outcomes of pediatric HSN patients are reevaluated according to the new classification. Primary kidney biopsies from 80 HSN patients were scored using the new SQC.

Evaluation of factors affecting the duration of disease-modifying anti-rheumatic drugs application in patients with enthesitis-related arthritis.

Objective: Treatments for enthesitis-related arthritis (ERA) consist of a mono- or combination therapy with non-steroidal anti-inflammatory drugs, disease-modifying anti-rheumatic drugs (DMARDs), and biological agents, and they are primarily based on adult studies and studies on other forms of juvenile idiopathic arthritis, depending on whether there is axial or peripheral involvement. We use DMARDs frequently in our daily practice, even in patients with axial involvement. The main reason for this is that the health insurance system in Turkey does not allow the use of Tumor Negrosis Factor (TNF) blockers as the first line of treatment.

Increased circulating endothelial microparticles in children with FMF.

Objective: Endothelial microparticles (EMPs) are considered as markers of endothelial dysfunction. In this study, we aimed to examine whether there is endothelial dysfunction in children with familial Mediterranean fever (FMF), hypothesizing that endothelial dysfunction would be present especially with acute-phase response in the active period of the disease.

Methods: This cross-sectional study included 65 FMF patients (41 attack free, 24 attack period) and 35 healthy controls.

Voiding cystourethrogram: How much should we be selective?

Objective: In this study, we examined the patients' characteristics, who underwent voiding cystourethrography (VCUG), in order to determine any selectivity for indication of this invasive method.

Material And Methods: After exclusion of indications of neurogenic bladder or antenatal hydronephrosis and control VCUGs, 159 VCUGs performed in our clinic within one year were evaluated. Patients are divided into three groups accoding to age.

Urinary oxalate to creatinine ratios in healthy Turkish schoolchildren.

Aim: we aimed to establish reference values for urinary oxalate to creatinine ratios in healthy children aged 6-15 years and to investigate the relationship between their nutritional habits and oxalate excretion.

Materials And Methods: Random urine specimens from 953 healthy children aged 6-15 years were obtained and analyzed for oxalate and creatinine. Additionally, a 24-h dietary recall form was prepared and given to them.

Investigating the Impacts of Preoperative Steroid Treatment on Tumor Necrosis Factor-Alpha and Duration of Extubation Time underwent Ventricular Septal Defect Surgery.

Background: Cardiopulmonary bypass is known to cause inflammatory events. Inflammation occurs due to many known important biological processes. Numerous mechanisms are known to be responsible for the development of inflammatory processes.

Comparison of the efficacy of once- and twice-daily colchicine dosage in pediatric patients with familial Mediterranean fever--a randomized controlled noninferiority trial.

Background: In this study, we examined the efficacy and safety of a once-daily dosage schema of colchicine compared with a twice-daily dosage schema in pediatric patients with familial Mediterranean fever (FMF).

Methods: In this 24-week, multicenter, randomized controlled noninferiority trial, pediatric patients newly diagnosed with FMF carrying a homozygous or compound heterozygous mutation and not receiving any treatment were included. Patients were randomly assigned using a block randomization method to receive treatment with a once- or twice-daily dosage.