Tıp Fakültesi / Faculty of Medicine
Permanent URI for this collectionhttps://hdl.handle.net/11727/1403
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Item Extra-Renal Manifestations of Atypical Hemolytic Uremic Syndrome in Children(2018) Fidan, Kibriya; Goknar, Nilufer; Gulhan, Bora; Melek, Engin; Yildirim, Zeynep Y.; Baskin, Esra; Hayran, Mutlu; Gulleroglu, Kaan; Ozcakar, Zeynep B.; Ozaltin, Fatih; Soylemezoglu, Oguz; https://orcid.org/0000-0003-4361-8508; https://orcid.org/0000-0003-1434-3824; 29610995; B-5785-2018; AAJ-8833-2021Atypical hemolytic uremic syndrome (aHUS) is a chronic disease characterized by thrombotic microangiopathy and a high risk of end-stage kidney disease. Dysregulation and/or excessive activation of the complement system results in thrombotic microangiopathy. Interest in extrarenal manifestations of aHUS is increasing. This study aimed to determine the clinical characteristics of patients with extrarenal manifestations of aHUS in childhood. This study included 70 children with extrarenal manifestations of HUS from the national Turkish aHUS Registry. The demographics, clinical characteristics, genetic test results, all treatments, and renal/hematologic status of aHUS patients with extrarenal involvement were recorded. The most common extrarenal manifestation was neurological system involvement (n = 46 [27.2%]), followed by gastrointestinal (n = 20 [11.8%]), cardiovascular (n = 12 [7%]), and respiratory (n = 12 [7%]) involvement. The patients with neurological involvement had a higher mortality rate and a lower estimated glomerular filtration rate (eGFR) than the other patients at last follow-up. Eculizumab (with or without plasma exchange/plasma infusion) treatment increased the renal and hematologic recovery rates. The most common and serious extrarenal manifestation of aHUS is neurological involvement and treatment outcome findings presented herein are important to all relevant clinicians.Item Anocutaneous Reflex Revisited: How Valuable Is Its Determination in Children with Spina Bifida? A Descriptive Study in A Cohort of 217 Patients(2022) Ozel, Kerem; Canmemis, Arzu; Goknar, Nilufer; Candan, Cengiz; Alizada, Orkhan; Alatas, Ibrahim; 35147971AIM: To determine the clinical value of anocutaneous reflex (AR) in children with neurogenic bladder due to spina bifida (SB). MATERIAL and METHODS: Patients who were diagnosed with SB were prospectively evaluated; moreover, AR and bulbocavernous reflex were examined. Patients were divided into those with and without AR. Age, gender, diagnosis, ventriculoperitoneal shunt presence, symptomatic urinary tract infections, leg movements, clean intermittent catheterization and anticholinergic therapy, lesion level, urodynamic detrusor, and sphincter activity were evaluated. Chi-square test and univariate regression analysis were done. The AR value was evaluated by two by two contingency table. RESULTS: This study evaluated 217 patients (109 boys and 108 girls). AR was present and absent in 53 and 164 patients, respectively. Anticholinergic therapy was necessary in 37.7% and 23.8% of patients with and without AR (p=0.015), respectively. Patients with AR had higher lesion level (p=0.005), more detrusor overactivity, and less detrusor underactivity (p=0.007). Less detrusor sphincter dyssynergia (DSD) was noted in patients with AR (p=0.029). AR specificity was 83%, and positive predictive value in predicting detrusor overactivity and DSD was 76% and 80, respectively. CONCLUSION: AR determination is a valuable and simple tool in neurogenic bladder. This report delineates the clinical significance of this reflex and is the largest cohort describing this significance. This simple examination should not be skipped in the initial evaluation and follow-up of these patients