Sare Gülfem Özlü

Background: Nephropathic cystinosis is a rare multisystem disorder in which transition from pediatric to adult care is particularly a vulnerable period because of lifelong treatment demands and the need for sustained multidisciplinary follow-up. We aimed to assess awareness, perceptions, and self-reported transition practices among pediatric nephrologists in Türkiye caring for patients with nephropathic cystinosis. Methods: We conducted a nationwide descriptive cross-sectional web-based survey of pediatric nephrologists in Türkiye who had longitudinally followed at least one patient with nephropathic cystinosis. A 59-item questionnaire covering awareness, perceptions, and self-reported practices was administered via Google Forms, and responses were analysed descriptively at the item level. Results: In this nationwide descriptive cross-sectional web-based survey, 48 pediatric nephrologists participated. Respondents worked mainly in university hospitals (47.9%) and training and research hospitals (39.6%) and had a median of 14 years of pediatric nephrology experience (IQR 6–17). Although 66.7% distinguished transition from transfer, familiarity with cystinosis-specific transition models was limited; only 16.7% had ever applied a structured model and 8.3% reported a written transition plan in their center. The most frequently perceived challenges were treatment non-adherence (89.6%) and disruption of multidisciplinary follow-up (87.5%). Most respondents reported marked differences between pediatric and adult services, and the median recommended age for initiating transition planning was 17.5 years (IQR 17–18). Overall, 91.7% supported the development of a national transition model (58.3% considered it definitely necessary and 33.3% necessary but difficult to implement without external support). Conclusions: Among pediatric nephrologists in Türkiye, awareness of transition-related risks in nephropathic cystinosis was high, but structured transition practices were uncommon and initiation of transition planning was generally recommended at a later age than suggested by current guidance. These findings indicate an implementation gap and support the development of a feasible national transition framework that can be adapted to local resources.

Purpose: Multicystic dysplastic kidney (MCDK) is the most common cystic renal disease in children and is often detected prenatally. This study evaluated the clinical features, associated anomalies, and renal outcomes of children with unilateral MCDK, focusing on the impact of recurrent urinary tract infections (UTIs) on renal function.Methods: A retrospective review of 77 patients (aged 1–18 years) diagnosed with unilateral MCDK by ultrasonography between 2019 and 2024 was conducted. Demographic, clinical, and laboratory data were analyzed, including estimated glomerular filtration rate (eGFR), UTIs, hypertension, and contralateral compensatory hypertrophy.Results: Of 77 patients, 64.9% were male and 55.8% had left-sided disease. Antenatal diagnosis was made in 77.9%, and 85.7% were asymptomatic. Urinary tract and extrarenal anomalies occurred in 27.3% and 40.3%, respectively. UTIs were observed in 31.2% and recurrent UTIs in 11.7%. Vesicoureteral reflux (VUR) was detected in 9.1% overall and in 33.3% of patients with recurrent UTIs (p=0.031). Mean follow-up was 7.2±4.4 years with a mean eGFR of 99.9±17.8 mL/min/1.73 m². Recurrent UTIs were more frequent in patients with eGFR

Background: Hypertension is an increasingly important health concern among children and adolescents. Beyond traditional risk factors such as obesity, sedentary behaviors, including prolonged internet use and video gaming, may contribute to elevated blood pressure. This study aimed to investigate the association between problematic internet use, video gaming, and ambulatory blood pressure parameters in adolescents. Methods: This cross-sectional study included adolescents aged 12–18 years who were referred to a pediatric nephrology outpatient clinic for evaluation of hypertension. Demographic, clinical, and laboratory data were obtained from medical records. Ambulatory blood pressure monitoring (ABPM) was performed to classify ambulatory hypertension and white coat hypertension. Internet use and gaming behaviors were assessed using the Young Internet Addiction Scale (YIA-SF) and the Internet Gaming Disorder Scale (IGDS9-SF). Scale scores were compared with ABPM parameters. Results: A total of 107 adolescents (40 girls, 67 boys) with a mean age of 14.9 ± 1.8 years were included. Based on ABPM findings, 55 participants (51.4%) had ambulatory hypertension, while 52 (48.6%) had white coat hypertension. Higher YIA-SF scores were weakly but significantly associated with higher mean daytime systolic blood pressure. IGDS9-SF scores were not significantly associated with ambulatory blood pressure parameters. Conclusion : Problematic internet use may be associated with higher mean daytime systolic blood pressure in adolescents, including among non-obese individuals. Although these behaviors were not independent predictors of ambulatory hypertension after adjustment for demographic and anthropometric factors, awareness of screen-based behaviors may complement lifestyle counseling in adolescents evaluated for elevated blood pressure. What is Known: • The majority of pediatric studies examining the relationship between screen-based behaviors and blood pressure have been based on office blood pressure measurements, which may not fully capture circadian blood pressure patterns compared with ambulatory blood pressure monitoring . What is New: • This study contributes to the limited literature examining the association between problematic internet use, video gaming, and ambulatory blood pressure parameters in adolescents .• Higher internet addiction scores were associated with higher mean daytime blood pressure, including among non-obese adolescents .

Objective: This study aimed to assess the frequency, causative microorganisms, antimicrobial resistance patterns, and unusual clinical and laboratory findings of culture-positive urinary episodes throughout the entire chemotherapy course in pediatric patients with Acute Lymphoblastic Leukemia (ALL). Material and Methods: We reviewed the medical records of 93 pediatric ALL patients who completed BFM-based chemotherapy protocols between 2019 and 2025. A total of 1265 urine cultures were analyzed. Culture positivity was defined as ≥105 colony-forming units per milliliter (CFU/mL) of a single or two different pathogens. For retrospective analysis, contamination was defined by a negative control culture within 48–72 hours or clinical resolution without antibiotic treatment. Data on urinalysis, neutropenia status, and antibiotic resistance were collected. Results: Forty-eight patients (51.6%) experienced a median of two culture-positive episodes. Incidence was highest during intensive treatment phases (induction, reinduction). Escherichia coli was the most common agent (39.8%), followed closely by Klebsiella pneumoniae (36.7%). Overall ESBL positivity was 26.6%. A concerning 30.6% of K. pneumoniae isolates showed carbapenem resistance. Standard urinalysis was found to be unreliable, with 71.4% of positive cultures lacking typical findings; leukocyturia was only present when the absolute neutrophil count was relatively preserved. Three cases of urosepsis (3%) were identified, all involving highly resistant organisms. Conclusion: Pediatric ALL patients, particularly during intense chemotherapy, face a high burden of culture-positive urinary episodes and a notable shift towards multidrug-resistant non-E. coli pathogens like K. pneumoniae. The unreliability of standard urinalysis in this immunocompromised population necessitates the routine collection of urine cultures during febrile neutropenia workups, irrespective of symptoms, to ensure timely, targeted therapy guided by local resistance data. The observed extreme resistance profile appears to be a transient reflection of the intensive chemotherapy environment.

Objective: Vesicoureteral reflux (VUR) is a prevalent congenital anomaly in children, with a higher incidence in those with febrile urinary tract infections. Early detection and management of VUR are crucial to prevent reflux nephropathy and its associated complications. This retrospective observational study aimed to evaluate the accuracy and advantages of ultrasonography (USG) in diagnosing VUR grade compared to voiding cystourethrography (VCUG), the gold standard. Material and Methods: The study included 147 children aged 0-5 years who underwent VCUG for urinary tract infections, as well as antenatal and postnatal hydronephrosis. Patients were categorized into two age groups: 0-24 months (Group 1) and 25-60 months (Group 2). USG findings, including parenchymal thickness, renal pelvis anteroposterior diameter, renal parenchymal echogenicity, and distal ureteral dilatation, were compared between low-grade and high-grade VUR in both groups. Results: Binary logistic regression analysis revealed that renal pelvis anteroposterior diameter and distal ureteral dilatation were independently associated with high-grade VUR in Group 1. The optimal cut-off value of renal pelvis anteroposterior diameter for predicting high-grade VUR was 5 mm, with 78.0% sensitivity and 76.0% specificity. However, no USG findings were independently associated with high-grade VUR in Group 2. Conclusion: USG examinations performed by experienced radiologists can effectively decrease radiation exposure in children, especially those with high-grade VUR under two years of age, and may prevent unnecessary radiation exposure in some pediatric patients

Hematopoietic stem cell transplantation (HSCT) is an effective treatment for various childhood diseases with long-term complications, including kidney side effects. We conducted a single-center retrospective study of 213 patients who received allogeneic HSCT between February 2011 and December 2023. Patients were followed for at least 3 months post-HSCT, with a median follow-up of 2.9 years. We evaluated pre- and post-HSCT estimated glomerular filtration rate (eGFR) and kidney complications. After HSCT, patients showed significantly higher rates of acute kidney injury (21.6% vs. 4.1% pre-HSCT), hypertension (26.8% vs. 5.2% pre-HSCT), and tubulopathy (20.2% vs. 5.2% pre-HSCT). The median final eGFR was 144 mL/min/1.73 m², which was significantly lower than the pre-HSCT eGFR (155 mL/min/1.73 m², p < 0.0001) and negatively correlated with time after HSCT (Rs = - 0.177, p = 0.009). Eight patients (3.8%) progressed to chronic kidney disease (CKD). Factors linked to CKD included inherited bone marrow failure syndromes, anti-thymocyte globulin (ATG) conditioning, urinary tract infections (UTIs), and high urinary BK virus loads. Although the rates of glomerular hyperfiltration (GHF) did not change significantly between pre-HSCT (63.8%) and post-HSCT (62.4%, p = 0.824), GHF was notable in patients with acute lymphoblastic leukemia pre-HSCT and thalassemia post-HSCT. Seventeen patients (8.4%) needed ongoing antihypertensive treatment. HSCT can cause various kidney complications. GHF is common both before and after transplant, and eGFR often declines over time. UTIs, ATG conditioning, and inherited bone marrow failure syndromes are important risk factors for CKD. Individual factors and infection surveillance should be considered in these patients to approach kidney health.

AMAÇ: Bu çalışmada, Kronik Böbrek Hastalığı (KBH) tanısıyla takipli hastaların KBH etiyolojisi, evreleri, medikal ve renal replasman tedavilerinin değerlendirilmesi amaçlanmıştır. GEREÇ VE YÖNTEM: Dr. Sami Ulus Kadın Doğum, Çocuk Sağlığı ve Hastalıkları Eğitim ve Araştırma Hastanesi Çocuk Nefroloji Kliniğinde Ocak 2008-Ocak 2013 tarihleri arasında KBH tanısı alan en az 6 ay süreyle takip ve tedavi edilen 0-19 yaş grubu 193 hastanın dosyası retrospektif olarak incelendi. BULGULAR: En sık görülen başvuru şikâyetleri; vücutta şişlik, bulantı-kusma ve büyüme geriliğiydi. Evre I’de 9 (%4,7) hasta, evre II’de 35 (%18,1) hasta, evre III’te 48 (%24,9) hasta, evre IV ‘te 40 (%20,7) hasta ve evre V’te 61 (%31,6) hasta bulundu. Yaş grubu ile KBH evreleri karşılaştırıldığında; 0-4 yaş grubunda evre V hasta sayısı (20 hasta - %55,6) daha yüksek saptandı (p:0,035). Etiyolojiye göre yapılan sınıflamada en sık görülen neden ürolojik/tübülointerstisyel hastalıklardı (%47,2). Takip süresince hastaların 78’i (%40,4) sadece konservatif tedavi ile 115 hasta (%59,6) ise renal replasman tedavisi (RRT) ile izlendi. RRT alan hastaların 55’ine periton diyalizi (PD), 46’sına hemodiyaliz (HD) tedavisi uygulanırken 14 hastaya renal transplantasyon yapıldı. Ölüm nedeni 5 hastada (%38,4) kardiyovasküler nedenler, 5 hastada (%38,4) enfeksiyon ve 2 hastada(%15,3) serebrovasküler nedenlerdi. SONUÇ: Çalışmamız erken evre KBH hastalarının tespitinin büyük önem arz ettiğini göstermiştir. KBH olan hastalarda görülen komplikasyonlar yaşam kalitesini önemli ölçüde etkilemektedir. Çalışmamız KBH’nın erken tanı ve tedavisi, gelişebilecek komplikasyonların tanınmasına olan ihtiyacı vurgulamaktadır.