PubMed İndeksli Yayınlar Koleksiyonu
Permanent URI for this collectionhttps://hdl.handle.net/11727/4810
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Item Could Plasma Based Therapies Still Be Considered in Selected Cases with Atypical Hemolytic Uremic Syndrome?(2021) Ozlu, Sare Gulfem; Gulhan, Bora; Aydog, Ozlem; Atayar, Emine; Delibas, Ali; Parmaksiz, Gonul; Ozdogan, Elif Bahat; Comak, Elif; Tasdemir, Mehmet; Acar, Banu; Ozcakar, Zeynep Birsin; Topaloglu, Rezan; Soylemezoglu, Oguz; Ozaltin, Fatih; 35023648Background. Atypical hemolytic uremic syndrome (aHUS) occurs due to defective regulation of the alternative complement pathway (ACP) on vascular endothelial cells. Plasma based therapy (PT) was the mainstay of the treatment for aHUS for many years until the introduction of therapies targeting blockage of the complement system. The aim of this study was to evaluate patients with aHUS who had been treated with plasma based therapies alone. Methods. The outcomes of seven genetically confirmed aHUS patients (2 girls, 5 males) were evaluated by means of clinical presentation, response to plasma therapy, course of the disease during the follow-up period and last status. Results. The median age of the patients at admission was 6.7 years (IQR 0.7-7.8). Three patients received plasma exchange therapy and the other four patients were treated with plasma infusions. One patient was lost to follow-up after one year; the median duration of follow-up for other patients was 3.7 years (IQR 2.7-6.5). During the follow up, two patients from our historical records when complement blocking therapies had not been in clinical use yet in Turkey, underwent kidney transplantation. One transplant patient experienced an acute rejection episode without graft loss. The remaining five patients had a glomerular filtration rate of more than 90 ml/min./1.73 m(2) at the last visit. Conclusion. Although we had a relatively small patient population, our findings indicate that PT might still be considered in selected patients particularly in countries where complement blocking therapies are difficult to reach due to their unavailability or costs that are not covered by the health care systems.Item The relationship between body mass index and renal length in obese children(2020) Parmaksiz, Gonul; Kekec, Senay Demir; Cengiz, Nurcan Dinler; Noyan, Aytul; 0000-0003-2373-1837; 31997076; AAD-5713-2021; AAM-2935-2021Background Obesity in the pediatric population is a severe public health problem and is associated with various comorbidities. Renal length is an important clinical parameter for the diagnosis and follow-up of renal diseases. The aim of this study was to determine the relationship between renal length (measured ultrasonographically) and body mass index (BMI) in obese children, and to develop nomograms for renal length according to BMI. Methods Renal ultrasound was performed in 368 children without renal disease. Each child's age, gender, weight, height, and BMI (kg/m(2)) were recorded. The children were divided into three groups according to BMI percentiles: obese group: BMI >= 95th percentile; overweight group: BMI 85th-94th percentile; normal weight group: BMI 5th-84th percentile. Results Weight, height, BMI, and right and left renal length differed significantly between the three groups (p = 0.001). There were significant correlations between renal length with age, weight, height, and BMI. Measurement of renal length was independently associated with BMI, age, and height. BMI was used to create renal length nomograms for obese children, based on multiple regression analysis (R-2 = 0.32 and p = 0.0001). Mean renal length was highest in the obese group (96.9 +/- 13.4 mm) and lowest in the normal weight group (88.3 +/- 12.9 mm). Conclusions Ultrasonographic measurement of the renal length according to BMI in children can be a useful method in evaluating these children. Smaller-than-normal kidneys can easily remain undiagnosed in obese and overweight children and this nomogram offers an additional method to evaluate the renal size in obese children.