Publications by authors named "Beltinge Demircioglu-Kilic"
Background/objectives: Eculizumab is a first-line treatment for atypical hemolytic uremic syndrome (aHUS), and patients undergoing eculizumab therapy may become more susceptible to infection caused by (). While meningococcal vaccination is required for patients undergoing eculizumab therapy, there is limited knowledge about meningococcal carriage in children with aHUS. We aimed to evaluate (1) the prevalence of carriage, (2) serogroup distribution, and (3) the immunization status of children undergoing eculizumab treatment for aHUS.
View Article and Find Full Text PDFBackground: Atypical hemolytic uremic syndrome (aHUS) is a rare, mostly complement-mediated thrombotic microangiopathy. The majority of patients are infants. In contrast to infantile-onset aHUS, the clinical and genetic characteristics of adolescence-onset aHUS have not been sufficiently addressed to date.
View Article and Find Full Text PDFArticle Synopsis
- HNF1B mutations are a leading genetic cause of congenital kidney and urinary tract abnormalities, prompting a study of 16 pediatric patients to better understand its clinical and genetic features.
- The research found that 81.3% of patients had prenatal kidney abnormalities, with kidney cysts being the most prevalent, and identified missense variants as the most common genetic issues linked to these conditions.
- The study suggests that patients with missense variants are at a greater risk for chronic kidney disease (CKD), while urinary system abnormalities like vesicoureteral reflux are also common in this group.
Pediatric kidney care experience after the 2023 Türkiye earthquake.
Nephrol Dial Transplant
August 2024
Article Synopsis
- Two devastating earthquakes in Türkiye on February 6, 2023, resulted in significant injuries to 903 children, with a focus on crush syndrome-related acute kidney injury (Crush-AKI) and mortality.
- The study tracked various factors, including the time under rubble, treatment protocols, and patient outcomes, revealing that Crush-AKI developed in 36% of the patients, and over half required dialysis.
- Key findings included that serum levels of creatine phosphokinase above 20,950 U/L and inadequate initial intravenous fluid volume were linked to Crush-AKI, and most deaths occurred among those with Crush-AKI within the first four days of admission.
Article Synopsis
- C3 glomerulopathy (C3G) is a kidney disease influenced by genetic factors, and this study explores the differences in clinical outcomes among pediatric patients with and without mutations in complement-related genes.
- Among 60 patients studied, 17 had genetic mutations with the CFH gene being the most common; those with mutations typically showed asymptomatic urinary issues and were diagnosed at an older age.
- Despite differences in presentation, both groups had similar long-term kidney survival and response to mycophenolate mofetil treatment, indicating that genetic mutations may not significantly impact treatment outcomes.
Article Synopsis
- Myelomeningocele (MMC) is a condition that affects many kids in developing countries like Turkey, and it can lead to kidney problems known as chronic kidney disease (CKD).
- A study in Turkey looked at children with MMC to find out how many had different stages of CKD and what factors made their kidney health worse.
- The results showed that 5.3% of the kids had CKD stage 5, and certain medical conditions were linked to an increased risk of serious kidney problems. To help these kids, doctors suggest taking early action in managing their bladder issues to prevent kidney failure.
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.
Article Synopsis
- * Common neuropsychiatric symptoms included headaches (50.3%), seizures (38.3%), and acute confusional states (33.6%), with five distinct clusters of symptoms identified based on their clinical and laboratory findings.
- * Key factors associated with increased risk of neurologic issues included positivity for antiphospholipid antibodies, history of plasmapheresis treatment, and higher SLEDAI scores, providing insights for better diagnosis and management.
Article Synopsis
- - The study examines the perceptions of patients, caregivers, nurses, and physicians regarding the automated peritoneal dialysis system (Homechoice with Claria sharesource system - CSS), focusing on its advantages and disadvantages for home treatment monitoring.
- - Questionnaires were filled out by 30 patients/caregivers, 22 pediatric nephrologists, and 15 PD nurses, revealing that most healthcare professionals believe CSS enhances patient monitoring and saves time, although many nurses felt other doctors weren't familiar with it.
- - While 90% of patients reported feeling safer due to constant monitoring, only 20% noticed reduced hospital visits, highlighting the need for more extensive research to fully understand CSS's impact on patient quality of life.
Article Synopsis
- - Hyponatremic-hypertensive syndrome (HHS) is a rare condition in children, exemplified by a case involving a 19-month-old boy who presented with severe hypertension and multiple symptoms including vomiting and seizures.
- - Laboratory tests indicated several issues, such as low sodium (hyponatremia), low potassium (hypokalemia), and high levels of hormones like renin and aldosterone, alongside renal artery stenosis.
- - The boy's symptoms improved dramatically after undergoing nephrectomy, highlighting the importance for clinicians to recognize HHS quickly for effective treatment.
Article Synopsis
- The study analyzed 25 genetically confirmed cases of PLCε1-related kidney disease to explore the clinical spectrum, genotype-phenotype correlations, and treatment impacts on kidney outcomes.
- 36% of patients presented with isolated proteinuria, while others had nephrotic syndrome or chronic kidney disease, with significant variations in histopathology results among those biopsied.
- The findings suggest that while genetic variations do not correlate with clinical presentations, the type of kidney condition and histological features significantly influence prognosis and treatment responses.
Article Synopsis
- The study investigated the kidney disease profiles of Syrian refugee children in Turkey, highlighting their vulnerability and specific health issues within conflict zones.
- Data from 633 children revealed that congenital anomalies of the kidney and urinary tract (CAKUT), glomerular disease, and chronic kidney disease (CKD) were the most common conditions, with significant demographic factors like parental consanguinity and familial history of kidney disease noted.
- Challenges faced during follow-up included language barriers, lack of medical records, and disruptions in care, which emphasize the need for improved treatment options and preventive measures for these children.
Article Synopsis
- Henoch-Schönlein purpura nephritis (HSN) affects the kidneys and involves symptoms like hematuria and proteinuria; the study aimed to analyze data from HSN patients who had renal biopsies to see how various factors impact outcomes.
- The study evaluated 72 HSN patients over a ten-year period, categorized their biopsy results, and compared clinical data, treatment approaches, and both short- and long-term outcomes.
- Key findings indicated that conditions like scrotal involvement, hypertension, and low creatinine clearance were linked to worse outcomes, suggesting the need for closer monitoring and considering specific treatments like cyclosporin-A and cyclophosphamide for patients unresponsive to steroids.
aHUS is caused by the over-activation and dysregulation of the alternative complement pathway. Data regarding outcomes of pediatric aHUS patients after kidney transplantation are still very scarce. Accordingly, the aim of this study was to describe the clinical findings and outcomes of pediatric aHUS patients after renal transplantation.
View Article and Find Full Text PDFObjective: To investigate the prevalence of obesity and associated factors during childhood in Southeastern Turkey. Another objective was to determine the cut-off points of Waist to Height Ratio (WHtR) values for defining obesity/abdominal obesity.
Methods: The community-based descriptive cross-sectional study was conducted in Gaziantep Turkey between November 2011 and December 2011 with 2718 primary school/high schools students aged 6-17 years.
Objective: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are very rare in childhood with an increased risk of morbidity and mortality. We aimed to evaluate renal prognostic factors in childhood AAV from the perspective of ANCA serotype, histopathological classification, and five-factor score (FFS).
Methods: Pediatric AAV patients from 11 referral centers in Turkey had been included to the study.
Article Synopsis
- Acute post-streptococcal glomerulonephritis (APSGN) is a common kidney issue in children; this study aimed to identify risk factors linked to reduced kidney function (GFR) in affected patients.
- A retrospective analysis of 75 children diagnosed with APSGN revealed that many had symptoms like edema, hematuria, and hypertension, with several showing low protein levels and increased inflammation markers.
- The study found that low GFR was significantly associated with factors like low C4 levels, hypoalbuminemia, and elevated inflammatory markers, indicating their role in the severity of kidney involvement in APSGN cases.
Objectives: This study aims to evaluate the frequency of skin, gastrointestinal, joint, and renal involvement in children with Henoch-Schönlein purpura and investigate the risk factors for organ/system involvement.
Patients And Methods: The data of 186 patients (97 boys, 89 girls; mean age 7.4±2.
Purpose: The association between fat mass and obesity-associated (FTO) gene and obesity is unclear in both adults and adolescents. The aim of this study was to examine the role of the FTO gene variant rs9939609 as a candidate gene for obesity and the relationship between insulin resistance (IR), metabolic syndrome (MetS), estimated glomerular filtration rate (eGFR) and neutrophil-to-lymphocyte ratio (NLR).
Methods: Obese adolescents (n=100) and healthy controls (n=100) were included.
Article Synopsis
- Uteroglobin (UG) is a protein with anti-inflammatory effects, and this study investigated the G38A genetic variation's role in childhood idiopathic nephrotic syndrome (INS) and its relation to steroid response.
- One hundred thirty-six children with INS and 70 healthy controls were analyzed, revealing that the AA genotype of the UG gene G38A polymorphism was significantly associated with increased risk of both steroid-sensitive and steroid-resistant INS.
- The findings suggest that the UG gene influences the development of INS, particularly the AA genotype, prompting the need for further research on genetic variations and their impacts in larger populations.
The Clinical and Mutational Spectrum of Turkish Patients with Cystinosis.
Clin J Am Soc Nephrol
October 2017
Background And Objectives: Infantile nephropathic cystinosis is a severe disease that occurs due to mutations in the cystinosis gene, and it is characterized by progressive dysfunction of multiple organs; >100 cystinosis gene mutations have been identified in multiple populations. Our study aimed to identify the clinical characteristics and spectrum of cystinosis gene mutations in Turkish pediatric patients with cystinosis.
Design, Setting, Participants, & Measurements: We identified the clinical characteristics and spectrum of cystinosis gene mutations in Turkish patients with cystinosis in a multicenter registry that was established for data collection.
Article Synopsis
- The study focuses on the kidney disease profile of Syrian refugee children admitted to the Pediatric Nephrology Department at the University of Gaziantep between 2012 and 2015.
- Among 130 children studied, 59.6% were girls, with an average age of about 7 years; various kidney-related conditions were documented, including congenital abnormalities and chronic kidney disease.
- The findings highlight the high prevalence of kidney conditions among these children and suggest that ongoing political instability may worsen their health and quality of life.
Background: Dextranomer/hyaluronic acid (Dx/Ha;Dexell®) and polyacrylate-polyalcohol copolymer (PPC;Vantris®) are the popular tissue-augmenting substances using for the endoscopic injections of vesicoureteral reflux (VUR). The aim of the study is to evaluate and compare Dx/Ha and PPC in terms of effectiveness, injection techniques and complications with special emphasis on vesicoureteral junction obstruction (VUJO).
Methods: A total of 95 patients who underwent endoscopic VUR treatment between 2009 and 2015 were retrospectively reviewed.