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    Characteristics and predictors of chronic kidney disease in children with myelomeningocele: a nationwide cohort study
    (Springer, 2024) Dogan, Cagla Serpil; Taner, Sevgin; Tiryaki, Betul Durucu; Alaygut, Demet; Ozkayin, Nese; Kara, Aslihan; Gencler, Aylin
    Background Myelomeningocele (MMC) is highly prevalent in developing countries, and MMC-related neurogenic bladder is an important cause of childhood chronic kidney disease (CKD). This nationwide study aimed to evaluate demographic and clinical features of pediatric patients with MMC in Turkey and risk factors associated with CKD stage 5.Methods Data from children aged 0-19 years old, living with MMC in 2022, were retrospectively collected from 27 pediatric nephrology centers. Patients > 1 year of age without pre-existing kidney abnormalities were divided into five groups according to eGFR; CKD stages 1-5. Patients on dialysis, kidney transplant recipients, and those with eGFR < 15 ml/min/1.73 m2 but not on kidney replacement therapy at time of study constituted the CKD stage 5 group.Results A total of 911 (57.8% female) patients were enrolled, most of whom were expectantly managed. Stages 1-4 CKD were found in 34.3%, 4.2%, 4.1%, and 2.4%, respectively. CKD stage 5 was observed in 5.3% of patients at median 13 years old (range 2-18 years). Current age, age at first abnormal DMSA scan, moderate-to-severe trabeculated bladder on US and/or VCUG, and VUR history were independent risk factors for development of CKD stage 5 (OR 0.752; 95%; CI 0.658-0.859; p < 0.001; OR 1.187; 95% CI 1.031-1.367; p = 0.017; OR 10.031; 95% CI 2.210-45.544; p = 0.003; OR 2.722; 95% CI 1.215-6.102; p = 0.015, respectively). Only eight CKD stage 5 patients underwent surgery related to a hostile bladder between 1 and 15 years old.Conclusion MMC-related CKD is common in childhood in Turkey. A proactive approach to neurogenic bladder management and early protective surgery in selected cases where conservative treatment has failed should be implemented to prevent progressive kidney failure in the pediatric MMC population in our country.
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    Multicenter evaluation of ambulatory blood pressure in children with CAKUT: distinctive profiles in the cystic dysplasia subgroup
    (Springer, 2026) Yel, Sibel; Gunay, Neslihan; Yildirim, Pelin Abdal; Demir, Belde Kasap; Soyaltin, Eren; Kara, Aslihan; Gurgoze, Metin Kaya
    Background Hypertension (HT) is an important risk factor in patients with congenital anomalies of the kidney and urinary tract (CAKUT), independent of the existing anomaly in childhood. This study aimed to investigate masked hypertension and/or blood pressure profiles in different subgroups with a normal glomerular filtration rate (GFR). Methods The study included participants from seven different pediatric nephrology centers in T & uuml;rkiye. Ambulatory blood pressure monitoring (ABPM) was performed on patients aged 5-18 years, diagnosed with CAKUT, with a GFR above 100 ml/min/m(2), and normal office blood pressure measurements. Validated Mobil-O-Graph or Spacelabs devices were used in all centers. Results In total, 118 healthy control data and 322 patients with CAKUT were evaluated, consisting of 73 (22.7%) with agenesis, 79 (24.5%) with cystic dysplasia, 92 (28.6%) with vesicoureteral reflux, 18 (5.6%) with UPJ (ureteropelvic junction) obstruction, and 60 (18.6%) with other conditions. In all CAKUT patients, daytime systolic blood pressure (SBP) and both day and nighttime diastolic blood pressure (DBP) loads were significantly higher compared to the healthy control group (p < 0.05). Nocturnal hypertension was identified in 58 (18%) of 322 children, whereas none of the control group presented hypertension. Children with cystic dysplasia had the highest nighttime hypertension proportions (22.7%) when compared to other subgroups. All data revealed higher total systolic-diastolic SDS and total mean SDS in the cystic dysplasia subgroup (p < 0.05). Conclusions The findings underscore the importance of blood pressure monitoring in the follow-up of patients with CAKUT, especially those with cystic dysplasia, even in the absence of GFR decline.