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    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 Demircioglu
    Background: 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.
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    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
    [Abstract Not Available]
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    CLINICAL COURSE OF ADOLESCENT ONSET ATYPICAL HEMOLYTIC UREMIC SYNDROME: A STUDY OF TURKISH AHUS REGISTRY
    (Springer, 2022) Celegen, Kubra; Gulhan, Bora; Fidan, Kibriya; Yuksel, Selcuk; Yilmaz, Neslihan; Yilmaz, Aysun Caltik; Kilic, Beltinge Demircioglu
    [Abstract Not Available]
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    Kidney disease profile and encountered problems during follow-up in Syrian refugee children: a multicenter retrospective study
    (Springer, 2022) Balat, Ayse; Kilic, Beltinge Demircioglu; Aksu, Bagdagul; Kara, Mehtap Akbalik; Buyukcelik, Mithat; Agbas, Ayse; Eroglu, Fehime Kara
    Background Children are one of the most vulnerable groups in conflict zones, especially those with chronic diseases. This study aimed to investigate kidney disease profiles and problems during follow-up in a population of Syrian refugee children residing in Turkey. Methods Syrian refugee children aged between 0 and 18 years were included in the study. Demographic data, diagnosis, particular interventions due to nephrological problems, and problems encountered during follow-up were obtained from all participating pediatric nephrology centers. Results Data from 633 children from 22 pediatric nephrology centers were included. Mean age of the children was 94.8 +/- 61.7 months and 375 were male (59%). 57.7% had parental consanguinity and 23.3% had a close relative(s) with kidney disease. The most common kidney diseases were congenital anomalies of the kidney and urinary tract (CAKUT) (31.0%), glomerular disease (19.9%), chronic kidney disease (CKD) (14.8%), and urolithiasis (10.7%). Frequent reasons for CAKUT were nonobstructive hydronephrosis (23.0%), vesico-ureteral reflux (18.4%), and neurogenic bladder (15.8%). The most common etiology of glomerular diseases was nephrotic syndrome (69%). Ninety-four children had CKD, and 58 children were on chronic dialysis. Six children had kidney transplantation. Surgical intervention was performed on 111 patients. The language barrier, lack of medical records, and frequent disruptions in periodic follow-ups were the main problems noted. Conclusions CAKUT, glomerular disease, and CKD were highly prevalent in Syrian refugee children. Knowing the frequency of chronic diseases and the problems encountered in refugees would facilitate better treatment options and preventive measures.