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Öğe Adolescence-onset atypical hemolytic uremic syndrome: is it different from infant-onset?(Springer, 2024) Celegen, Kubra; Gulhan, Bora; Fidan, Kibriya; Yuksel, Selcuk; Yilmaz, Neslihan; Yilmaz, Aysun Caltik; Kilic, Beltinge DemirciogluBackground: 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. Methods: A total of 28 patients (21 girls, 7 boys) who were diagnosed as aHUS between the ages of >= 10 years and <18 years were included in this study. All available data in the Turkish Pediatric aHUS registry were collected and analyzed. Results: The mean age at diagnosis was 12.8 +/- 2.3 years. Extra-renal involvement was noted in 13 patients (46.4%); neurological involvement was the most common (32%). A total of 21 patients (75%) required kidney replacement therapy. Five patients (17.8%) received only plasma therapy and 23 (82%) of the patients received eculizumab. Hematologic remission and renal remission were achieved in 25 (89.3%) and 17 (60.7%) of the patients, respectively. Compared with the infantile-onset aHUS patients, adolescent patients had a lower complete remission rate during the first episode (p = 0.002). Genetic analyses were performed in all and a genetic variant was detected in 39.3% of the patients. The mean follow-up duration was 4.9 +/- 2.6 years. At the last visit, adolescent patients had lower eGFR levels (p = 0.03) and higher rates of chronic kidney disease stage 5 when compared to infantile-onset aHUS patients (p = 0.04). Conclusions: Adolescence-onset aHUS is a rare disease but tends to cause more permanent renal dysfunction than infantile-onset aHUS. These results may modify the management approaches in these patients.Öğe Adolescence-onset atypical hemolytic uremic syndrome: is it different from infant-onset? (MAY, 10.1007/s10157-024-02505-7, 2024)(Springer, 2024) Celegen, Kubra; Gulhan, Bora; Fidan, Kibriya; Yuksel, Selcuk; Yilmaz, Neslihan; Yilmaz, Aysun Caltik; Kilic, Beltinge Demircioglu[Anstract Not Available]Öğe Cognitive performance, psychiatric comorbidities, and quality of life in pediatric patients with juvenile idiopathic arthritis: a comparative analysis with healthy controls(Routledge Journals, Taylor & Francis Ltd, 2024) Tezer, Damla; Basay, Burge Kabukcu; Basay, Omer; Yener, Gulcin Otar; Yuksel, SelcukThis study aimed to assess the extent of cognitive impairment in children and adolescents with Juvenile Idiopathic Arthritis (JIA). While cognitive deficits are recognized in other systemic rheumatic diseases, exploration within the pediatric JIA population remains limited. The investigation utilized a comprehensive approach to examine neuropsychological test performance. A cohort of 160 participants (79 JIA, 81 healthy controls aged 8-17) underwent evaluations using the Pediatric Quality of Life Inventory (PedsQL), Schedule for Affective Disorders and Schizophrenia for School Age Children-Present and Lifetime Version (K-SADS-PL), and the computerized neurocognitive test battery Central Nervous System Vital Signs (CNSVS). Children with JIA exhibited statistically significant cognitive deficits across various parameters (p < .05). This was associated with an increased prevalence of lifelong psychiatric illnesses and diminished overall quality of life compared to healthy counterparts (p < .05). Analysis highlighted that specific JIA subtypes, excluding Oligoarthritis, significantly elevated the risk of neurocognitive impairments, emphasizing the impact on various cognitive outcomes (OR range: 3.1-5.1, 95% CI: 1.163-19.980). Additionally, the active disease stage was identified as a specific risk factor, amplifying the likelihood of low executive functions by 4.3 times (OR: 4.363, 95% CI: 1.095-17.378). This study underscores the critical importance of recognizing and addressing neurocognitive impairments in children with JIA. Specific attention to disease subtypes and activity levels is crucial, with the potential for targeted interventions to enhance overall cognitive well-being and quality of life in this vulnerable population.Öğe Determining Split Renal Function in Children With Ureteropelvic Junction Stenosis: Technetium-99m Mercaptoacetyltriglycine (Tc99m MAG-3) or Technetium-99m Dimercaptosuccinic Acid (Tc-99m DMSA)?(Springernature, 2024) Ozdemir, Hale; Girisgen, Ilknur; Yaylali, Olga; Becerir, Tuelay; Herek, Oezkan; Senol, Hande; Yuksel, SelcukBackground Ureteropelvic junction stenosis (UPJS) is the most common cause of clinically significant antenatal hydronephrosis. We compared separate renal function results obtained using technetium-99mmercaptoacetyltriglycine (Tc-99m MAG-3) and technetium-99m-dimercaptosuccinic acid (Tc-99m DMSA) in pediatric patients with UPJS to evaluate the adequacy of Tc-99m MAG-3 scintigraphy and the necessity of additional Tc-99m DMSA scintigraphy during follow-up. Methodology Patients diagnosed with hydronephrosis in the Pediatric Nephrology Department of Pamukkale University Faculty of Medicine over a period of 10 years (2012-2022) were evaluated retrospectively. Patients who had been diagnosed with UPJS and underwent both Tc-99m MAG-3 and Tc-99m DMSA scintigraphy during follow-up were included in the study. Technetium-99m-labeled MAG-3 and DMSA scans were re-evaluated for all patients by the Department of Nuclear Medicine. Results The study included 52 children with unilateral UPJS (12 girls and 40 boys) with a mean age of 6.34 +/- 4.81 years (range: 2.97-9.79 years). Thirty-six patients (69.2%) were diagnosed antenatally. Differential renal function in Tc-99m DMSA was 46.94 +/- 10.64 and in Tc-99m MAG-3 was 43.08 +/- 11.18; the functions were lower in Tc-99m MAG-3, but the values were within normal limits for both groups (p=0.0001, z=-3.893). When differential renal functions were compared between Tc-99m DMSA and Tc-99m MAG-3 results, a statistically significant positive and strong correlation was found in the kidney with ureteropelvic junction obstruction (UPJO) (p=0.0001, r=0.752). When classifying the Tc-99m MAG-3 and Tc-99m DMSA results in the kidney with UPJO (supranormal, normal, low function) for the determination of differential renal functions, there was a consistency of 76%, and it was correlated (p=0.0001, k=0.456). While two patients had supranormal function and 13 patients had low function in Tc-99m MAG-3, five patients had supranormal function, and eight patients had low function in Tc-99m DMSA. Conclusions Some studies in the literature have reported that Tc-99m MAG-3 causes supranormal function measurements in patients with UPJS; our results showed that Tc-99m DMSA resulted in a higher rate of supranormal values for affected kidneys. We believe that Tc-99m DMSA should not be performed in addition to Tc-99m MAG-3 scintigraphy in the follow-up of every patient with UPJS but can be utilized in select cases, such as patients with surgical indications and those suspected before surgery.Öğe How do gene mutation diversity and disease severity scoring affect physical capacity and quality of life in children/adolescents with Familial Mediterranean Fever?(Elsevier Espana Slu, 2024) Kabul, Elif Gur; Bali, Merve; Calik, Bilge Basakci; Tekin, Zahide Ekici; Yener, Gulcin Otar; Yuksel, SelcukObjectives: The aim of this study is to examine how gene mutation diversity and disease severity affect physical capacity and quality of life in children/adolescents with Familial Mediterranean Fever (FMF). Methods: Eighty children/adolescents (42 female, 38 male) diagnosed with FMF according to TellHashomer diagnostic criteria were included in this study. Disease severity score (PRAS), running speed and agility and strength subtests of Bruininks-Oseretsky Test of Motor Proficiency Second Edition Short Form (BOT-2 SF), Physical Activity Questionnaire, Pediatric Quality of Life Inventory 3.0 Arthritis Module (PedsQL) was used for evaluation. Participants were divided into 2 groups as M694V and other mutations according to MEFV gene mutation and were divided into 3 groups as mild, moderate and severe according to PRAS. Results: When the data were compared between groups; in terms of gene mutation, a significant difference was observed in treatment subtest of PedsQL-parent form in favor of the M694V gene mutation group (p < 0.05). In terms of PRAS, significant difference was seen in the pain, treatment subtests and total score of the PedsQL-child form, and in the pain, treatment, worry subtests and total score of the PedsQL-parent form in favor of the mild group (p < 0.05). Conclusions: MEFV gene mutations in children and adolescents with FMF did not differ on physical capacity and quality of life. PRAS was not effective on physical parameters, but quality of life decreased as the severity score increased. Encouraging children/adolescents with FMF to participate in physical activity and to support them psychosocially can be important to improve their quality of life. (c) 2024 Elsevier Espana, S.L.U. and Sociedad Espanola de Reumatolog & imath;a y Colegio Mexicano de Reumatolog & imath;a. All rights are reserved, including those for text and data mining, AI training, and similar technologies.Öğe Investigation of motor skill in patients with juvenile idiopathic arthritis: A cross sectional study(Asociacion Colombiana de Reumatologia, 2024) Yenil, Sinem; Gur Kabul, Elif; Basakci Calik, Bilge; Kilbas, Gulsah; Yuksel, SelcukIntroduction: The inflammatory process of Juvenile Idiopathic Arthritis (JIA) is associated comorbidities. The JIA patients can fall behind their healthy peers, and motor and functional skills can reduce. Objectives: The primary aim is to compare the motor skills of JIA patients with healthy controls. The secondary aim is to determine whether disease activity affects patients with JIA. Materials and methods: Fifteen patients with JIA and 15 healthy controls were included in the study. Motor skills were evaluated with Bruininks-Oseretsky Test of Motor Proficiency Second Edition Short Form (BOT-2 SF) in patients with JIA and healthy controls. BOT-2 SF measures four motor area composites with eight subtests. Disease activity was evaluated with Juvenile Arthritis Disease Activity Score-27 (JADAS-27), disability level with Childhood Health Assessment Questionnaire Disability Index (CHAQ-DI), and disease-related quality of life with Pediatric Quality of Life Inventory (PedsQL) 3.0 Arthritis Module for JIA. According to disease activity, patients with JIA were divided into two groups as remission and active. Results: The patients with JIA had significantly lower scores in the total and four motor area of BOT-2 SF compared to healthy controls (p < .05). When the remission and active groups were compared, there was no difference in the total and four motor area of BOT-2 SF, CHAQ-DI, or PedsQL (p > .05). Conclusion: The motor skills of patients with JIA are lower than their healthy peers, and their motor skills, quality of life, and disability did not make a difference between the remission and active period. © 2023 Asociación Colombiana de ReumatologíaÖğe Investigation of Physical Fitness in Children and Adolescents with Juvenile Idiopathic Arthritis: A Case-Control Study(Aves, 2024) Bozcuk, Sinem; Calik, Bilge Basakci; Kabul, Elif Gur; Tekin, Zahide Ekici; Yuksel, SelcukObjective: Swelling, effusion, tenderness, and pain seen in the joints of juvenile idiopathic arthritis (JIA). This disease may cause limitation in joint movements, muscle weakness, atrophy, balance, and gait disorders. Physical fitness is accepted as an important determinant of health in both childhood and adolescence. The aim was to evaluate the physical fitness of children/ adolescents with JIA and compare it with healthy peers. Materials and Methods: Seventy children/adolescents were included (35 JIA and 35 healthy). The Childhood Health Assessment Questionnaire (CHAQ) and the Brockport physical fitness test battery were used for evaluation. The Brockport physical fitness test battery consists of dominant handgrip strength, curl-up, push-up, trunk lift, shoulder stretch, sit and reach tests, skinfold thickness (calf /triceps/subscapular) measurements, and PACER 20 m test. Results: A significant difference was found in all sub-parameters of CHAQ (P < .05) and dominant hand grip strength (P = .037), curl-up test (P < .001), trunk lift test (P = .018), shoulder stretch (P < .001) and PACER 20 m test (P < .001) tests in favor of the healthy group. Conclusion: Children/adolescents with JIA demonstrated lower performance compared to their healthy peers in muscular and cardiovascular capacity tests (curl-up test, PACER 20 m test, trunk lift test, dominant hand grip strength test, and shoulder stretch test). Their functional abilities are more impaired, and they experience higher levels of pain and lower levels of general well-being compared to healthy peers.Öğe Panniculitis in Anti-MDA-5 Positive Juvenile Dermatomyositis: A Case Report(Nepal Medical Association, 2025) Ayduran, Semra; Kilbas, Gulsah; Tigrak, Saadet Nilay; Yuksel, Selcuk; Comut, Erdem; Turkucar, SerkanPanniculitis is a rare clinical finding in dermatomyositis. There are few reported cases in the medical literature. In this report, we describe a 17-year-old male patient with anti-MDA5 positive hypomyopathic dermatomyositis who, eight months after diagnosis, presented with indurated nodules on the right forearm and right thigh despite methotrexate and monthly intravenous immunoglobulin treatment. A skin biopsy revealed lobular panniculitis with lymphocytic infiltrate. His lesions were successfully controlled with hydroxychloroquine and azathioprine. This article presents a case regarding the rarity of panniculitis in juvenile dermatomyositis and its treatment strategy. © 2025, Nepal Medical Association. All rights reserved.Öğe Variable phenotype and genotype of pediatric patients with HNF1B nephropathy(Dustri-Verlag Dr Karl Feistle, 2024) Gulhan, Bora; Ekici, Ozan; Dursun, Ismail; Goknar, Nilufer; Yuksel, Selcuk; Alaygut, Demet; Ozcakar, Zeynep BirsinAims: Hepatocyte nuclear factor 1 beta ( HNF1B ) mutations are the most common monogenic cause of congenital anomalies of the kidney and urinary tract (CAKUT). We aimed to investigate clinical and genetic characteristics of patients with HNF1B nephropathy to expand its phenotypic and genetic spectrum. Materials and methods: This retrospective cohort study included 16 unrelated pediatric patients (6 females, 10 males) from 13 families with genetically confirmed HNF1B -related nephropathy. Results: Abnormal prenatal kidney abnormalities were present in 13 patients (81.3%). The most common antenatal kidney abnormality was kidney cysts, which were observed in 8 patients (61.5%). Urinary system abnormalities (vesicoureteral reflux (VUR) and ure teropelvic junction obstruction (UPJO)) were present in 4 patients (25%). HNF1B analysis uncovered missense variants in 4 families (30.8%) as the most common genetic abnormality. In addition, 4 novel pathological variations have been defined. During followup, hypomagnesemia and hyperuricemia were observed in 7 (43.8%) and 5 patients (31.3%), respectively. None of the patients with a missense variant had hypomagnesemia. However, 7 out of 12 patients (58.3%) with a non-missense variant had hypomagnesemia (p = 0.09). None of the patients had an HNF1B score below 8, and the mean score was 15.3 +/- 4.4. The mean follow-up period was 7.4 +/- 5.0 years. While 100% of patients (n = 4) with missense variants were in various stages of CKD (CKD2: 2 patients,CKD3: 2 patients), 25% of those with nonmissense variants had CKD (CKD2, 3, and 5; 1 patient, respectively) (p = 0.026). Conclusion: Patients with HNF1B-associated disease have concomitant urinary system abnormalities such as VUR or UPJO. Missense variants seem to be the most common pathological variations in HNF1B gene and have higher risk of CKD.Öğe Variable phenotype and genotype of pediatric patients with HNF1B nephropathy [2](Springer, 2024) Gulhan, Bora; Ekici, Ozan; Dursun, Ismail; Goknar, Nilufer; Yuksel, Selcuk; Alaygut, Demet; Ozcakar, Zeynep Birsin[No abstract available]
