Browsing by Author "Parmaksiz, Gonul"

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Can RDW be used as a screening test for subclinical inflammation in children with FMF? Is RDW related to MEFV gene mutations?
(2023) Parmaksiz, Gonul; Noyan, Z. Aytul; 36103024
Objective Subclinical inflammation, an insidious feature of familial Mediterranean fever (FMF), can lead to life-threatening amyloidosis. We aimed to investigate acute phase reactants and complete blood count parameters to identify a useful marker for subclinical inflammation in children with FMF. A secondary aim was to identify an association between subclinical inflammation and specific Mediterranean fever (MEFV) gene mutations. Methods This study included 420 pediatric patients with FMF. Laboratory parameters of patients during the attack-free period and MEFV gene mutation analyses were recorded. Results Of the 420 patients, 88 (21%) had subclinical inflammation. Of those with subclinical inflammation, 48 (55%) had mutations in exon 10, 36 (41%) had M694V mutation, and 10 (11%) had M694V homozygous mutation. Red cell distribution width (RDW) value was higher in exon 10, M694V, and M694V homozygous mutations compared to other mutations. RDW was positively correlated with serum amyloid A (SAA) (r = 0.390, p = 0.0001). Analysis of a receiver-operating characteristic curve of RDW revealed that its optimal cut-off value for subclinical inflammation was 12.69%, its sensitivity was 64.10%, and its specificity was 50.90%. The area under the curve was 0.616 (p = 0.004, 95% confidence interval = 0.538-0.695). Conclusion We suggest that RDW can be used as a screening test as a marker of subclinical inflammation. A high RDW value should alert the clinician about subclinical inflammation in FMF children's patients with M694V (heterozygous, homozygous, compound heterozygous) mutation.
Can We Differentiate Pyelonephritis and Cystitis without 99mTc-Dimercaptosuccinic Acid Scan in Children?
(2015) Kilicaslan, Buket; Noyan, Aytul; Cengiz, Nurcan; Sariturk, Cagla; Parmaksiz, Gonul; Baskin, Esra
Purpose: Urinary tract infection is one of the most common infections in childhood. Because of the long term sequelae, differentiation of pyelonephritis from cystitis is important. The aim of this study is to determine the value of biomarkers such as C-reactive protein and procalcitonin and whether preferred to predict pyelonephritis in children without 99mTc-Dimercaptosuccinic Acid scan. Material and Methods: Fifty children aged 3 months to 16 years with a first urinary tract infection were included in this retrospective observational study. The medians, sensitivity, specificity, and cut-off values of serum C - reactive protein and procalcitonin to predict pyelonephritis were determined. Results: Thirty-two (64%) patients were diagnosed with pyelonephritis and 18 (36%) were diagnosed with cystitis. The cut-off value for C - reactive protein was 34 mg/L to predict pyelonephritis, with 69% sensitivity and 61% specificity. The cut-off value for procalcitonin was 0.23 ng/mL to predict pyehlonephritis, with 69% sensitivity and 66% specificity. In combination, these biomarkers were 63% sensitive and 78% specific to predict pyelonephritis. Conclusion: Using a combination of procalcitonin and C-Reactive Protein is preferred to predict pyelonephritis in children, instead of the 99mTc-Dimercaptosuccinic Acid scan. Because of its disadvantages, the 99mTc-Dimercaptosuccinic Acid scan should be avoided in children.
Cardiac Function in Children After Kidney Transplantation
(2018) Arslan, Alev; Parmaksiz, Gonul; Noyan, Aytul; Caliskan, Kenan; Yildirim, Sedat; Haberal, Mehmet; 0000-0003-4444-0027; 0000-0003-2373-1837; 0000-0002-8767-5021; 0000-0002-5735-4315; 0000-0002-3462-7632; V-1112-2019; AAM-2935-2021; AAD-5713-2021; AAJ-7201-2021; AAF-4610-2019; AAJ-8097-2021
The Clinical Characteristics and Prognosis of Exon 2 Mutations in Familial Mediterranean Fever
(2023) Avci, Begum; Parmaksiz, Gonul; Sahin, Feride; Noyan, Aytul; 0000-0001-7308-9673; AAC-7232-2020
Objective: It is unclear whether exon 2 mutations are variations or mutations that causes the disease. This study aimed to evaluate the clinical features and prognosis exon 2 mutations in Familial Mediterranean Fever. Methods: The clinical features, disease severity and prognosis of all patients with at least one exon 2 mutations were evaluated retrospectively. These data were compared separately for homozygous (Group 1), heterozygous (Group 2), compound heterozygous (Group 3), and complex alleles (Group 4), and the data were compared by grouping patients into those with and without exon 10 mutations. Results: There were a total of 119 patients with exon 2 mutations, including 11.7% in Group 1, 36.1% in Group 2, 21.8% in Group 3, and 30.2% in Group 4 were similar in terms of demographic data, clinical characteristics, and disease course. When compared patients with exon 10 mutations (+) to those with exon 10 mutations (-), the exon 10 mutations (+) group had a higher presence of chest pain (100%, p = 0.02) and a significantly higher mean Pras severity score (6.66 +/- 1.87, 6.01 +/- 1.40; p=0.02). Additionally, a higher number of patients with exon 10 mutation (-) achieved remission with treatment (76 (67.9%), 36 (32.1%); p = 0.03). Conclusion: Exon 2 mutations have a milder course and higher remission rates but they should be considered as Familial Mediterranean Fever disease because of their similar clinical presentation and response to colchicine treatment with exon 10 mutations. Early treatment and close follow- up should be performed.
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; 35023648
Background. 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.
Education Status of Pediatric Dialysis Patients in Turkey
(2018) Delibas, Ali; Ekim, Mesiha; Yildirim, Zeynep Yuruk; Dusunsel, Ruhan; Kara, Ashhan; Noyan, Aytul; Bayazit, Aysun Karabay; Conkar, Secil; Yazicioglu, Burcu Ozer; Serdaroglu, Erkin; Aksoy, Gulsah Kaya; Akinci, Nurver; Yilmaz, Dilek; Bek, Kenan; Ertan, Pelin; Gurgoze, Metin Kaya; Kabasakal, Caner; Doven, Serra Surmeli; Turkkan, Ozde Nisa; Gunay, Neslihan; Parmaksiz, Gonul; Melek, Engin; Erdogan, Semra; Ezgu, Sevcan Azime Bakkaloglu; Sever, Lale; https://orcid.org/0000-0003-2373-1837; AAD-5713-2021; AAM-2935-2021
Follow-Up Results of Patients with ADCK4 Mutations and the Efficacy of Coq10 Treatment
(2017) Atmaca, Mustafa; Gulhan, Bora; Korkmaz, Emine; Inozu, Mihriban; Soylemezoglu, Oguz; Candan, Cengiz; Bayazit, Aysun Karabay; Elmaci, Ahmet Midhat; Parmaksiz, Gonul; Duzova, Ali; Besbas, Nesrin; Topaloglu, Rezan; Ozaltin, Fatih; https://orcid.org/0000-0003-2373-1837; 28337616; AAM-2935-2021
ADCK4-related glomerulopathy is an important differential diagnosis in adolescents with steroid-resistant nephrotic syndrome (SRNS) and/or chronic kidney disease (CKD) of unknown origin. We screened adolescent patients to determine the frequency of ADCK4 mutation and the efficacy of early CoQ10 administration. A total of 146 index patients aged 10-18 years, with newly diagnosed non-nephrotic proteinuria, nephrotic syndrome, or chronic renal failure and end-stage kidney disease (ESKD) of unknown etiology were screened for ADCK4 mutation. Twenty-eight individuals with bi-allelic mutation from 11 families were identified. Median age at diagnosis was 12.4 (interquartile range [IQR] 8.04-19.7) years. Upon first admission, all patients had albuminuria and 18 had CKD (6 ESKD). Eight were diagnosed either through the screening of family members following index case identification or during genetic investigation of proteinuria in an individual with a history of a transplanted sibling. Median age of these 8 patients was 21.5 (range 4.4-39) years. CoQ10 supplementation was administered following genetic diagnosis. Median estimated glomerular filtration rate (eGFR) just before CoQ10 administration was 140 (IQR 117-155) ml/min/1.73m(2), proteinuria was 1,008 (IQR 281-1,567) mg/m(2)/day. After a median follow-up of 11.5 (range 4-21) months following CoQ10 administration, proteinuria was significantly decreased (median 363 [IQR 175-561] mg/m(2)/day, P=0.025), whereas eGFR was preserved (median 137 [IQR 113-158] ml/min/1.73m(2), P=0.61). ADCK4 mutations are one of the most common causes of adolescent-onset albuminuria and/or CKD of unknown etiology in Turkey. CoQ10 supplementation appears efficacious at reducing proteinuria, and may thereby be renoprotective.
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-2935-2021
Background 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.
Rituximab Treatment In Children With Difficult-To-Treat Nephrotic Syndrome
(2022) Parmaksiz, Gonul
Purpose: Rituximab (RTX) has been offered as rescue therapy for patients with difficult-to-treat nephrotic syndrome (frequent relapsing, steroid-dependent and steroid resistant). We aimed to assess the efficacy and long-term outcomes of RTX treatment in children with difficult nephrotic syndrome and shared our experiences Materials and Methods: Medical records of children with difficult nephrotic syndrome who were treated with RTX were retrospectively evaluated. The relapse-free survival rate at 12 month and monitoring of B-cell depletion were assessed. Results: In the study included 20 children of which 8 had steroid-dependent (SDNS), 6 had frequent relapsing (FRNS), and 6 had steroid-resistant nephrotic syndrome (SRNS). The median number of relapses at 1 year before and after treatment in FRNS/SDNS patients receiving RTX treatment were compared. The median number of relapses decreased from 2 (1-4) to 0 (0-1) times/year. The mean duration of the follow-up period after RTX treatment was 23 (12-59) months, and 8 patients developed relapse. Repeated doses of RTX were administered to 5 patients who relapsed after RTX treatment. In these patients, CD19+B cells re-emerged during remission, while depletion of memory B-cells remained. Conclusion: The RTX treatment prolonged the remission time in FRNS/SDNS patients, but it was ineffective in SRNS patients. It was determined that the RTX doses can be repeated to maintain remission in these patients, and the best memory B-cell counts can help in timing the repeat doses.
Role of New Biomarkers for Predicting Renal Scarring in Vesicoureteral Reflux: NGAL, KIM-1, And L-FABP
(2016) Parmaksiz, Gonul; Noyan, Aytul; Dursun, Hasan; Ince, Emine; Anarat, Ruksan; Cengiz, Nurcan; https://orcid.org/0000-0003-2373-1837; 26324091; AAM-2935-2021; AAD-5713-2021; AAW-8783-2020
Reflux nephropathy is the most serious complication of vesicoureteral reflux (VUR). The aim of this study was to assess the role of urinary levels of neutrophil-gelatinase-associated lipocalin (NGAL),kidney injury molecule-1 (KIM-1), and liver-type fatty-acid-binding protein (L-FABP) in the early diagnosis of reflux nephropathy in patients with VUR. This study assessed 123 patients with primary VUR and 30 healthy children as a control group. The children were divided into five groups: Group A, patients with VUR and renal parenchymal scarring (RPS); Group B, patients with VUR and without RPS; Group C, patients with RPS and resolved VUR; Group D, patients with resolved VUR and without RPS; Group E, healthy reference group. Median urinary NGAL (uNGAL)/Creatinine (Cr) was significantly higher in patients with than those without RPS and the control group (p = 0.0001). Median uKIM-1/Cr was similar in all groups (p = 0.417). Median uL-FABP/Cr was significantly higher in patients with RPS than in the reference group (p < 0.05). Urinary NGAL levels may be used as a noninvasive diagnostic marker for predicting renal scarring in reflux nephropathy.
Time-Averaged Hemoglobin Values, Not Hemoglobin Cycling, Have an Impact On Outcomes in Pediatric Dialysis Patients
(2018) Bakkaloglu, Sevcan A.; Kandur, Yasar; Serdaroglu, Erkin; Noyan, Aytul; Bayazit, Aysun Karabay; Tasdemir, Mehmet; Ozlu, Sare Gulfem; Ozcelik, Gul; Dursun, Ismail; Alparslan, Caner; Akcaboy, Meltem; Atikel, Yesim Ozdemir; Parmaksiz, Gonul; Atmis, Bahriye; Sever, Lale; 30105415; AAD-5713-2021
During erythropoietin-stimulating agent (ESA) treatment, hemoglobin (Hb) levels usually fluctuate; this phenomenon is known as "Hb cycling (HC)." In this study, we aimed to evaluate the predictors of HC and its impact on left ventricular hypertrophy (LVH) as a patient-important outcome parameter in pediatric dialysis patients. Records of patients followed up in nine pediatric nephrology centers between 2008 and 2013 were reviewed. More than 1 g/dL decrease or increase in Hb level was considered as HC. Patients were divided into two groups according to 12-month Hb trajectory as rare cycling (RC) (<= 3) and frequent cycling (FC) (> 3 fluctuation) as well as three groups based on T-A-Hb levels: < 10, 10-11, and > 11 g/dL. Two hundred forty-five dialysis (160 peritoneal dialysis (PD) and 85 hemodialysis (HD)) patients aged 12.3 +/- 5.1 (range 0.5-21) years were enrolled in this study. Fifty-two percent of the patients had RC, 45% had FC, and only 3% had no cycling. There were no differences between HC groups with respect to age, dialysis modality, having anemia, hospitalization rate, residual urine volume, and mortality. Although left ventricular mass index (LVMI) tended to be higher in RC than FC group (65 +/- 37 vs 52 +/- 23 g/m(2.7), p = 0.056), prevalence of LVH was not different between the groups (p = 0.920). In regression analysis, FC was not a risk factor for LVH, but low T-A Hb level (< 10 g/dL) was a significant risk for LVH (OR = 0.414, 95% CI 0.177-0.966, p = 0.04). The target Hb levels were more often achieved in PD patients, and the number of deaths was significantly lower in non-anemic patients (Hb level > 11 g/dL). Hb cycling is common among dialysis patients. Severity of anemia rather than its cycling has more significant impact on the prevalence of LVH and on inflammatory state.
Transplantation in Fsgs: A Multicentric Study From Turkey
(2018) Koyun, Mustafa; Akman, Sema; Comak, Elif; Bakkaloglu, Sevcan; Parmaksiz, Gonul; Alparslan, Caner; Poyrazoglu, Hakan; Canpolat, Nur; Bayazit, Aysun Karabay; Delibas, Ali; Yildiz, Nurdan; Ozcelik, Gul; Ozdogan, Elif Bahat; https://orcid.org/0000-0003-2373-1837; AAM-2935-2021
Transplantation in pediatric aHUS within the era of eculizumab therapy
(2020) Ozcakar, Zeynep Birsin; Ozaltin, Fatih; Gulhan, Bora; Comak, Elif; Parmaksiz, Gonul; Baskin, Esra; Topaloglu, Rezan; Kasap Demir, Belde; Canpolat, Nur; Yuruk Yildirim, Zeynep; Demircioglu Kilic, Beltinge; Yuksel, Selcuk; Soylemezoglu, Oguz; 0000-0003-4361-8508; 33217100; B-5785-2018
aHUS is caused by the over-activation and dysregulation of the alternative complement pathway. Data regarding outcomes of pediatric aHUS patients after kidney transplantation are still very scarce. Accordingly, the aim of this study was to describe the clinical findings and outcomes of pediatric aHUS patients after renal transplantation. This is a retrospective, multicenter study including 12 patients from the national registry system. Among the 12 patients, eight had received prophylactic eculizumab and none of those patients (except one) had experienced aHUS recurrence during a median follow-up period of 58.5 (min-max, 4-94) months. Although eculizumab had been started on the day before transplantation in one of them, aHUS recurrence occurred during the transplantation procedure. Eculizumab had been stopped in only one patient who had no complement gene mutation after 35 months of therapy, and recurrence had not been observed during the 19 months of follow-up. In three patients, maintenance doses had been spaced out without any recurrence. One additional patient with anti-CFH antibody received only two doses of eculizumab for transplantation and had been followed for 46 months without aHUS recurrence. The remaining three patients had not received anti-C5 therapy and none of those patients experienced aHUS recurrence during a median follow-up period of 21 (min-max, 9-42) months. Prophylactic eculizumab is a safe and effective treatment for the prevention of aHUS recurrence. Eculizumab interval prolongation, discontinuation, and transplantation without eculizumab prophylaxis can be tried in selected patients with close follow-up.
Turkish Atypical Hemolytic Uremic Syndrome Registry: Eculizumab Treatment İn Ahus Patients
(2018) Baskin, Esra; Canpolat, Nur; Gulleroglu, Kaan; Yilmaz, Alev; Melek, Engin; Yuksel, Selcuk; Gulhan, Bora; Kalyoncu, Mukaddes; Parmaksiz, Gonul; Ozcakar, Birsin; Ozaltin, Fatih; Soylemezoglu, Oguz; https://orcid.org/0000-0003-4361-8508; https://orcid.org/0000-0003-1434-3824; https://orcid.org/0000-0003-2373-1837; B-5785-2018; F-3294-2013; AAM-2935-2021
Urinary NGAL, KIM-1 and L-FABP Concentrations in Antenatal Hydronephrosis
(2015) Noyan, Aytul; Parmaksiz, Gonul; Dursun, Hasan; Ezer, Semire Serin; Anarat, Ruksan; Cengiz, Nurcan; 0000-0002-8817-494X; 0000-0003-2373-1837; 0000-0002-9597-3264; 26096437; AAJ-9529-2021; AAB-7105-2020; AAD-5713-2021; AAW-8783-2020; GPX-7059-2022; AAM-2935-2021
The clinical tests currently in use for obstructive nephropathy (such as renal ultrasonography, differential radionuclide renal scans and urinary creatinine concentration data) are not efficient predictors of the subsequent clinical course. Novel and simple biomarkers are required which, if proven, could be clinically beneficial in determining if a patient is eligible for surgery or reno-protective therapy. More recently, the interest of clinicians has focused on the potential of urinary neutrophil gelatinase-associated lipocalin (uNGAL), urinary kidney injury molecule-1 (uKIM-1) and urinary liver-type fatty acid-binding proteins (uL-FABP) as biomarkers for renal function in children with hydronephrosis (HN). Objective The purpose of this study was to investigate possible clinical applications of uNGAL, uKIM-1 and uL-FABP as beneficial non-invasive biomarkers to determine whether or not surgical intervention is required in children with HN. Study design Renal ultrasonography and radionuclide renal scans were used as diagnostic tools to detect HN. Patients were divided into two groups based on the anteroposterior diameter of their renal pelvis and the presence of dysfunction. Group 1 included 26 children with severe HN (with dysfunction), and group 2 consisted of 36 children with mild HN (without dysfunction). Urine samples were collected from 62 children with HN and 20 healthy children. Results Hydronephrosis was more common in males than in females, with a male to female ratio of 9: 1 in the study sample. The incidence of left kidney involvement (32 patients) was slightly higher than right kidney involvement (28 patients). Compared with controls and group 2, the ratio of uNGAL to creatinine was significantly higher in group 1 (p < 0.05). The biomarker uNGAL/Cr exhibited fairly good diagnostic accuracy, with an area under the curve of 0.68 [95% confidence interval 0.6-0.7] and an optimal cut-off value of 0.16 ng/mg Cr (sensitivity 58%, specificity 75%) (p < 0.05). There was a positive correlation between the uNGAL/Cr ratio and the uKIM-1/Cr ratio (r = 0.582, p < 0.05) and uL-FABP/Cr ratio (r = 0675, p < 0.05) in group 1. Discussion The results clearly demonstrated that children with hydronephrosis and dysfunction had significantly increased uNGAL, and uNGAL/Cr concentrations. However, uKIM-1, uKIM-1/Cr, uL-FABP and uL-FABP/Cr concentrations were not significantly different when compared with controls. These results support the use of uNGAL concentrations as an early marker for renal dysfunction in HN. Conclusions The study clearly demonstrated that pediatric patients with hydronephrosis and dysfunction had significantly higher uNGAL to creatinine concentrations as compared with controls.
Urinary Ngal, Kim-1 and L-fabp Levels in Antenatal Hydronephrosis
(2014) Dursun, Hasan; Noyan, Aytul; Parmaksiz, Gonul; Ezer, Semire Serin; Anarat, Ruksan; Cengiz, Nurcan; https://orcid.org/0000-0002-8817-494X; https://orcid.org/0000-0003-2373-1837; AAW-8783-2020; AAD-5713-2021; AAM-2935-2021; AAJ-9529-2021
Urinary Ngal, Kim-1 and L-fabp Levels in Patients with Vesicoureteral Reflux
(2014) Parmaksiz, Gonul; Noyan, Aytul; Dursun, Hasan; Ince, Emine; Anarat, Ruksan; Cengiz, Nurcan; https://orcid.org/0000-0003-2373-1837; https://orcid.org/0000-0002-8817-494X; AAM-2935-2021; AAD-5713-2021; AAB-7105-2020