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Active vitamin D is cardioprotective in experimental uraemia but not in children with CKD Stages 3-5
(NLM (Medline), 2021) Schön A.; Leifheit-Nestler M.; Deppe J.; Fischer D.-C.; Bayazit A.K.; Obrycki L.; Mir S.
BACKGROUND: Uraemic cardiac remodelling is associated with vitamin D and Klotho deficiency, elevated fibroblast growth factor 23 (FGF23) and activation of the renin-angiotensin system (RAS). The cardioprotective properties of active vitamin D analogues in this setting are unclear. METHODS: In rats with 5/6 nephrectomy (5/6Nx) treated with calcitriol, the cardiac phenotype and local RAS activation were investigated compared with controls. A nested case-control study was performed within the Cardiovascular Comorbidity in Children with Chronic Kidney Disease (4C) study, including children with chronic kidney disease (CKD) Stages 3-5 [estimated glomerular filtration rate (eGFR) 25?mL/min/1.73?m2] treated with and without active vitamin D. Echocardiograms, plasma FGF23 and soluble Klotho (sKlotho) were assessed at baseline and after 9?months. RESULTS: In rats with 5/6Nx, left ventricular (LV) hypertrophy, LV fibrosis and upregulated cardiac RAS were dose-dependently attenuated by calcitriol. Calcitriol further stimulated FGF23 synthesis in bone but not in the heart, and normalized suppressed renal Klotho expression. In the 4C study cohort, treatment over a mean period of 9?months with active vitamin D was associated with increased FGF23 and phosphate and decreased sKlotho and eGFR compared with vitamin D naïve controls, whereas LV mass index did not differ between groups. CONCLUSIONS: Active vitamin D ameliorates cardiac remodelling and normalizes renal Klotho expression in 5/6Nx rats but does not improve the cardiac phenotype in children with CKD Stages 3-5. This discrepancy may be due to further enhancement of circulating FGF23 and faster progression of CKD associated with reduced sKlotho and higher serum phosphate in vitamin D-treated patients. © The Author(s) 2020. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.
Advillin acts upstream of phospholipase C ?1 in steroid-resistant nephrotic syndrome
(American Society for Clinical Investigation, 2017) Rao J.; Ashraf S.; Tan W.; Van Der Ven A.T.; Gee H.Y.; Braun D.A.; Fehér K.; George S.P.; Esmaeilniakooshkghazi A.; Choi W.-I.; Jobst-Schwan T.; Schneider R.; Schmidt J.M.; Widmeier E.; Warejko J.K.; Hermle T.; Schapiro D.; Lovric S.; Shril S.; Daga A.; Nayir A.; Shenoy M.; Tse Y.; Bald M.; Helmchen U.; Mir S.; Berdeli A.; Kari J.A.; El Desoky S.; Soliman N.A.; Bagga A.; Mane S.; Jairajpuri M.A.; Lifton R.P.; Khurana S.; Martins J.C.; Hildebrandt F.
Steroid-resistant nephrotic syndrome (SRNS) is a frequent cause of chronic kidney disease. Here, we identified recessive mutations in the gene encoding the actin-binding protein advillin (AVIL) in 3 unrelated families with SRNS. While all AVIL mutations resulted in a marked loss of its actin-bundling ability, truncation of AVIL also disrupted colocalization with F-actin, thereby leading to impaired actin binding and severing. Additionally, AVIL colocalized and interacted with the phospholipase enzyme PLCE1 and with the ARP2/3 actin-modulating complex. Knockdown of AVIL in human podocytes reduced actin stress fibers at the cell periphery, prevented recruitment of PLCE1 to the ARP3-rich lamellipodia, blocked EGF-induced generation of diacylglycerol (DAG) by PLCE1, and attenuated the podocyte migration rate (PMR). These effects were reversed by overexpression of WT AVIL but not by overexpression of any of the 3 patient-derived AVIL mutants. The PMR was increased by overexpression of WT Avil or PLCE1, or by EGF stimulation; however, this increased PMR was ameliorated by inhibition of the ARP2/3 complex, indicating that ARP-dependent lamellipodia formation occurs downstream of AVIL and PLCE1 function. Together, these results delineate a comprehensive pathogenic axis of SRNS that integrates loss of AVIL function with alterations in the action of PLCE1, an established SRNS protein.
Are urodynamic studies really necessary in voiding dysfunction in children? [Çocuklarda işeme bozukluklarında ürodinamik çalışmalar gerçekten gerekli mi?]
(Galenos, 2018) Conkar S.; Mir S.
Aim: Urodynamic examination is considered the most reliable, but also a quite invasive tool for the diagnosis of voiding dysfunctions in children. In this study, we evaluated the role of invasive urodynamics in the diagnosis of lower urinary tract dysfunction in children. Methods: One thousand one hundred twenty seven patients underwent urodynamic studies in the pediatric nephrology clinic in Ege University Faculty of Medicine between March 2011 and March 2016. A retrospective analysis of data including symptoms of voiding dysfunction (urinary frequency, urgency, nocturia and/or urge incontinence) and findings of physical examination, urodynamics and ultrasonography was performed. Results: Two hundred and seventy-seven (30.8%) boys and 620 (69.1%) girls with a mean age of 7.52 (±2.6) years underwent urodynamic studies. The most common abnormality was overactive bladder detected in 630 patients (70.2%). 19.9% (n=179) of the participants had dysfunctional voiding, while 9.8% (n=88) had normal results. Conclusion: A small and frequent voiding pattern, enuresis nocturna with daytime symptoms, and postvoid urinary residual volume were the common findings seen in patients with overactive bladder. In addition, dyssynergic voiding and a bladder with large capacity but residual volume after voiding were also commonly found. To that end, we may use ultrasound, clinical examination, symptoms and voiding frequency as first-line diagnostic tools. © 2018 by The Medical Bulletin of University of Health Sciences Haseki Training and Research Hospital The Medical Bulletin of Haseki published by Galenos Yayınevi.
Association between RAS gene polymorphisms (ACE I/D, AGT M235T) and Henoch-Schönlein purpura in a Turkish population
(Hindawi Limited, 2013) Nalbantoglu S.; Tabel Y.; Mir S.; Serdaroglu E.; Berdeli A.
Henoch-Schönlein purpura (HSP) is a small-vessel vasculitis of autoimmune hypersensitivity, and renin-angiotensin system (RAS) regulates vascular homeostasis and inflammation with activation of cytokine release. Thus, we aimed to investigate the association between HSP and ACE I/D and AGT M235T polymorphisms. Genotyping was determined by allele specific PCR and PCR-RFLP. We obtained a significant difference in genotype distribution (p=0.003) and allele frequencies (p<0.001) of ACE I/D polymorphism between patients and controls, while no significant association was detected in genotype distribution (p> 0.05) and allele frequencies (p> 0.05) of the AGT M235T polymorphism. Risk assessment showed significant risk for HSP in the subjects both with the ID + DD genotype (p=0.019, OR: 2.288, 95% CI: 1.136-4.609) and D allele (OR: D vs. I: 2.0528, 95% CI: 1.3632-3.0912, p=0.001) while no significant risk was obtained for HSP in the subjects both with the MT + TT genotype (p=0.312, OR: 1.3905, 95% T vs. M: 1.065, 95% CI: 0.7326-2.6391) and T allele (OR: patients were stratified by the presence of certain systemic complications of HSP, no significant association was detected with ACE I/D, and AGT M235T polymorphisms. Our findings suggest that ACE I/D polymorphism is significantly associated with HSP susceptibility. © 2013 IOS Press and the authors. All rights reserved.
Association of clearance of middle- and large-molecular-weight substance with arterial stiffness and left ventricular mass in children receiving renal replacement therapy
(Edizioni Minerva Medica, 2017) Ozdemir K.; Yilmaz E.; Dincel N.; Bozabali S.; Apaydin S.; Gun Z.H.; Sozeri B.; Mir S.
BAC KGROUND: The prominent cause of mortality in children receiving dialysis treatment is cardiovascular diseases. Risk factors related to chronic renal disease, are effective in the development of cardiovascular diseases. The aim of study was to investigate cardiovascular system (CVS) involvement for functional and structural alterations in children receiving dialysis, and display any association between cardiovascular morbidity and uremic toxins. MET HODS: 20 dialysis patients and 20 healthy controls were included to the study. Clearance of small, middle and large molecular-weight uremic toxins was evaluated in blood samples collected 30 minutes before (D0) and 2 hour after dialysis (D2), and change value was calculated as D0-D2/D0. Cardiovascular involvement was determined by comparing arterial stiffness, carotid intima-media thickness (CI MT) and Left Ventricular Mass Index (LVMI) with the control group. RESULTS: Four patients receiving hemodialysis and two patients in continuous ambulatory peritoneal dialysis (CAPD) group who have significant differences in all functional and structural parameters were detected. Four dialysis patients with detected cardiovascular disease have distinctively lower beta-2 microglobulin and homocysteine clearances compared to the patients with no CVS involvement. C ONCLUSIONS: The clearance of middle and large molecular-weight substances should be closely monitored in children receiving dialysis.
Association of fas -670a/g and fasl -843c/t gene polymorphisms on allograft nephropathy in pediatric renal transplant patients
(Kowsar Medical Publishing Company, 2010) Ertan P.; Mir S.; Ozkayin N.; Berdeli A.
Objective: FAS and FASL polymorphisms are suggested to play an important role in tubulitis that is a major component of acute rejection. The aim of this study was to investigate the role of FAS-670A/G and FASL-843C/T gene polymorphisms on allograft nephropathy in pediatric renal transplant patients Methods: Fifty three patients (22 males 31 females) aged 2 to 20 years (mean 12.3±0.6) who had renal transplantation and fifty healthy control subjects (25 males 25 females) were enrolled in the study. Pearson's Chi Square test was used for the statistical analysis. Survival rates were estimated with the Kaplan Meier method. Age, sex, chronic renal failure etiology, treatment modality and duration and donor type were recorded. FAS-670A/G and FASL- 843C/T gene polymorphisms were compared between renal transplant patients and normal healthy population as well as between renal transplant patients with and without acute rejection. Findings: FAS-670A/G genotypes or alleles were not significantly different between control and transplant patients and among transplant patients with and without acute rejection (P>0.05 for all). FASL-843C/T genotypes and alleles were not different between transplantation and control groups (P>0.05 for all). However, FASL-843C/T alleles were significantly different between patients with and without AR (P=0.02). The percentages of Callele were higher in children with acute rejection (68.8% vs 44.6%). Conclusion: FASL gene polymorphisms may play a major role in acute rejection while FAS polymorphisms have not been found to be different between patients with and without acute renal graft rejection. © 2010 by Pediatrics Center of Excellence.
Association of macrophage migration inhibitory factor -173C allele polymorphism with steroid resistance in children with nephrotic syndrome
(2005) Berdeli A.; Mir S.; Ozkayin N.; Serdaroglu E.; Tabel Y.; Cura A.
The potential effects of macrophage migration inhibitory factor (MIF) on the natural immune response are due to the inhibition of immune cell activation, which is regulated by glucocorticoids. In this study, we investigated MIF -173G/C genotype and C allele frequency in 214 patients with idiopathic nephrotic syndrome (INS) and 103 healthy volunteers. We found significant increases in GC genotype (OR=3, p=0.0009) and C allele frequency (OR=2.5, p=0.0007) in INS. Upon classifying patients as steroid responsive (n =137) or resistant (n =77), a 20-fold over-expression of the CC-genotype was found in the steroid-resistant group (OR=20, p=0.0002). Moreover, a significant increase in C allele frequency in patients with focal segmental glomerulosclerosis (FSGS) has also been noted when compared with other histopathological groups (OR=3.2, p=0.0017). Furthermore, significant increases in the CC genotype (15.6% vs 3.3%) and C allele (75% vs 32%) frequencies have been found in patients with permanent renal function failure (p =0.013 and p =0.0002, respectively). Patients with the CC genotype were found to be at considerably increased risk of permanent renal failure (OR=5.43, p=0.013) and end-stage renal disease (OR=5.53, p=0.020). Additionally, there was a correlation between age of detection of proteinuria and CC genotype. We found an earlier age of onset of proteinuria in patients with the CC genotype (1.9±1.7 years) than in patients who were GC-heterozygous (3.7±3.1 years) and GG-homozygous (3.6±2.9 years, p=0.88). In summary, our results indicate that the MIF-173 C allele confers an increased risk of susceptibility to INS and plays a crucial role in glucocorticoid responsiveness. © IPNA 2005.
Association of vitamin D deficiency with increased pulse wave velocity and augmentation index in children with chronic kidney disease
(Iranian Society of Nephrology, 2018) Conkar S.; Mir S.; Doğan E.; Tutar Z.Ü.
Introduction. It is known that in children with chronic kidney disease (CKD), cardiovascular damage starts in the form of arterial stiffness. There are risk factors other than the traditional ones such as arterial stiffness hypertension, obesity, hypercholesterolemia, and insulin resistance. Vitamin D deficiency is rather common in CKD, and it was introduced as a risk factor for atherosclerosis; however, its relationship with arterial stiffness is not known completely. The purpose of this study was to research the relationship between 25-hydroxyvitamin D levels and arterial stiffness. Materials and Methods. Arterial stiffness was evaluated by measuring augmentation index (AI) and pulse wave velocity (PWV) from the radial and carotid arteries with a Vicorder. The 25-hydroxyvitamin D levels were measured by an immunoassay method. Results. In the 81 CKD patients (mean age, 13.21 ± 6.02 years; mean body mass index, 19.42 ± 5.12 kg/m 2 ; and 56.8% male), the mean vitamin D level was 60.71 ± 39.52 ng/mL, the mean AI was 7.93 ± 7.77%, and the mean PWV was 9.79 ± 4.36 m/s. Serum levels of 25-hydroxyvitamin D was correlated with AI (r =-0.482, P = 0.001) and PWV (r =-0.57, P =.001). Conclusions. In this study, it was proven that vitamin D deficiency in children was related to nondiabetic and nondialysis CKD. © 2018, Iranian Society of Nephrology. All rights reserved.
Bilateral renal dysplasia and situs inversus totalis in an infant girl [2]
(2003) Mir S.; Akil I.
[No abstract available]
Cardiovascular phenotypes in children with CKD: The 4C study
(American Society of Nephrology, 2017) Schaefer F.; Doyon A.; Azukaitis K.; Bayazit A.; Canpolat N.; Duzova A.; Niemirska A.; Sözeri B.; Thurn D.; Anarat A.; Ranchin B.; Litwin M.; Caliskan S.; Candan C.; Baskin E.; Yilmaz E.; Mir S.; Kirchner M.; Sander A.; Haffner D.; Melk A.; Wühl E.; Shroff R.; Querfeld U.; 4C Study Consortium
Background and objectives Cardiovascular disease is the most important comorbidity affecting long-term survival in children with CKD. Design, setting, participants, & measurements The Cardiovascular Comorbidity in Children with CKD Study is a multicenter, prospective, observational study in children ages 6–17 years old with initial GFR of 10–60 ml/min per 1.73 m2. The cardiovascular status is monitored annually, and subclinical cardiovascular disease is assessed by noninvasive measurements of surrogate markers, including the left ventricular mass index, carotid intima-media thickness, and central pulse wave velocity. We here report baseline data at study entry and an explorative analysis of variables associated with surrogate markers. Results A total of 737 patients were screened from October of 2009 to August of 2011 in 55 centers in 12 European countries, and baseline data were analyzed in 688 patients. Sixty-four percent had congenital anomalies of the kidney and urinary tract; 26.1% of children had uncontrolled hypertension (24-hour ambulatory BP monitoring; n=545), and the prevalence increased from 24.4% in CKD stage 3 to 47.4% in CKD stage 5. The prevalence of left ventricular hypertrophy was higher with each CKD stage, from 10.6% in CKD stage 3a to 48% in CKD stage 5. Carotid intima-media thickness was elevated in 41.6%, with only 10.8% of patients displaying measurements below the 50th percentile. Pulse wave velocity was increased in 20.1%. The office systolic BP SD score was the single independent factor significantly associated with all surrogate markers of cardiovascular disease. The intermediate end point score (derived from the number of surrogate marker measurements >95th percentile) was independently associated with a diagnosis of congenital anomalies of the kidney and urinary tract, time since diagnosis of CKD, body mass index, office systolic BP, serum phosphorus, and the hemoglobin level. Conclusions The baseline data of this large pediatric cohort show that surrogate markers for cardiovascular disease are closely associated with systolic hypertension and stage of CKD. © 2016 by the American Society of Nephrology.
Childhood vasculitides in Turkey: A nationwide survey
(2007) Ozen S.; Bakkaloglu A.; Dusunsel R.; Soylemezoglu O.; Ozaltin F.; Poyrazoglu H.; Kasapcopur O.; Ozkaya O.; Yalcinkaya F.; Balat A.; Kural N.; Donmez O.; Alpay H.; Anarat A.; Mir S.; Gur-Guven A.; Sonmez F.; Gok F.
Aim: The aims of this study were to evaluate the characteristics of childhood vasculitides and to establish the first registry in Turkey, an eastern Mediterranean country with a white population. Patients and methods: A questionnaire was distributed to the main referral centers asking for the registration of the Henoch-Schönlein purpura (HSP) patients in the last calendar year only and 5 years for other vasculitides. Demographic, clinical, and laboratory data were assessed. Results: Vasculitic diseases were registered from 15 pediatric centers. These centers had a fair representation throughout the country. In the last calendar year, incidences were as follows: HSP 81.6%, Kawasaki disease (KD) 9.0%, childhood polyarteritis nodosa (C-PAN) 5.6%, Takayasu arteritis (TA) 1.5%, Wegener's granulomatosis 0.4%, and Behçet disease 1.9%. There was no clear gender dominance. The mean age was 11.05±4.89 years. Acute phase reactants were elevated in almost all, highest figures being in C-PAN. Renal involvement was present in 28.6% of HSP and 53% of the C-PAN patients. Abdominal aorta was involved in all TA patients. Among the C-PAN patients, 25% had microscopic PAN with necrotizing glomerulonephritis; antineutrophil cytoplasmic antibody was positive in those who were studied. Among the patients, 12.5% and 15% had classic PAN and cutaneous PAN, respectively. The remaining majority were classified as systemic C-PAN diagnosed with biopsies and/or angiograms demonstrating small to midsize artery involvement. The overall prognosis was better than reported in adult series. Conclusion: This is the largest multicenter study defining the demographic data for childhood vasculitides. The distribution of childhood vasculitides was different in our population where KD is much less frequent, whereas HSP constitutes an overwhelming majority. C-PAN was more frequent as well. © Clinical Rheumatology 2006.
Clinical outcome in children with Henoch-Schönlein nephritis
(2007) Mir S.; Yavascan O.; Mutlubas F.; Yeniay B.; Sonmez F.
Henoch-Schönlein purpura (HSP) is the most common vasculitis in childhood. The long-term prognosis is variable and depends on renal involvement. The aim of this study was to evaluate both clinical features of the children with HSP and the prognoses of short- and long-term outcome of patients diagnosed as HSP nephritis (HSN). This is a retrospective data study of all children with HSP hospitalized from January 1991 to December 2005. The patients with HSN were classified according to their initial presentation, histologic findings, type of treatment and clinical outcome. All patients have been evaluated once every 2 months. Fifty-three of the patients had kidney biopsies. The patient population consisted of 141 children included 78 boys (55.3%) and 63 girls (44.7%) ranging in age at disease onset from 2 to 17 (8.9±3.29) years. Renal involvement was determined in 58.1%. Nephrotic and/or nephritic syndrome were found to be an unfavorable predictor both for short and long-term outcome (P<0.05). However, 35% of these patients and 62% of them showed complete remission after 6 months and long-term course. Overall prognosis of HSN is relatively good and long-term morbidity is predominantly associated with initial presentation and renal involvement. © IPNA 2006.
Does NPHS1 polymorphism modulate P118l mutation in NPHS2?
(2013) Dincel N.; Mir S.; Berdeli A.; Bulut I.K.; Sozeri B.
Nephrotic syndrome (NS) in the first year of life is uncommon and makes up a heterogeneous group of disorders. Subsequent studies have further defined the phenotype associated with mutations in the NPHS2 gene, revealing that patients usually develop NS from birth to 6 years of age. We report a child aged 4 months with steroid-resistant NS who had polymorphism of NPHS1 (E117K) and mutation of NPHS2 (P118L). Our patient was carrying a polymorphic NPHS1 mutation, while phenotypically she had a poor prognostic NPHS2 mutation. However, it must be questioned whether this polymorphic change (E117K) alters the signaling pathways of the podocytes and leads to P118L mutation, thus making it behave differently. Perhaps, this would be called a genetic modifier in future.
Early proteinuria lowering by angiotensin-converting enzyme inhibition predicts renal survival in children with CKD
(American Society of Nephrology, 2018) Van Den Belt S.M.; Heerspink H.J.L.; Gracchi V.; De Zeeuw D.; Wühl E.; Schaefer F.; Anarat A.; Bakkaloglu A.; Ozaltin F.; Peco-Antic A.; Querfeld U.; Gellermann J.; Sallay P.; Drozdz D.; Bonzel K.-E.; Wingen A.-M.; Zurowska A.; Balasz I.; Trivelli A.; Perfumo F.; Müller-Wiefel D.E.; Möller K.; Offner G.; Enke B.; Wühl E.; Gimpel C.; Mehls O.; Schaefer F.; Emre S.; Caliskan S.; Mir S.; Wygoda S.; Hohbach-Hohenfellner K.; Jeck N.; Klaus G.; Ardissino G.; Testa S.; Montini G.; Charbit M.; Niaudet P.; Caldas-Afonso A.; Fernandes-Teixeira A.; Dušek J.; Matteucci M.C.; Picca S.; Mastrostefano A.; Wigger M.; Berg U.B.; Celsi G.; Fischbach M.; Terzic J.; Fydryk J.; Urasinski T.; Coppo R.; Peruzzi L.; Arbeiter K.; Jankauskiené A.; Grenda R.; Litwin M.; Janas R.; Laube G.; Neuhaus T.J.
Background Although pharmacotherapeutic proteinuria lowering was found to be nephroprotective in adults, the predictive value of early drug-induced proteinuria reduction for long-term renal survival in pediatric CKD is unknown. We analyzed data from the ESCAPE Trial for a potential association between initial antiproteinuric effect of standardized angiotensin-converting enzyme (ACE) inhibition and renal disease progression in children with CKD. Methods In total, 280 eligible children with CKD stages 2-4 (mean age 11.7 years old, median eGFR 46 ml/min per 1.73 m2, 71% congenital renal malformations) received a fixed dose of ramipril (6 mg/m2 per day) and were subsequently randomized to conventional or intensified BP control. We assessed initial proteinuria reduction from baseline to first measurement on ramipril (at 2.561.3 months). We used multivariable Cox modeling to estimate the association between initial proteinuria reduction and the risk of reaching a renal end point (50% eGFR decline or ESRD), which occurred in 80 patients during 5 years of observation. Results Ramipril therapy lowered proteinuria by a mean of 43.5% (95% confidence interval, 36.3% to 49.9%). Relative to proteinuria reduction,30%, 30%-60% and .60% reduction resulted in hazard ratios (95% confidence intervals) of 0.70 (0.40 to 1.22) and 0.42 (0.22 to 0.79), respectively. This association was independent of age, sex, CKD diagnosis, baseline eGFR, baseline proteinuria, initial BP, and concomitant BP reduction. Conclusions The early antiproteinuric effect of ACE inhibition is associated with long-term preservation of renal function in children with CKD. Proteinuria lowering should be considered an important target in the management of pediatric CKD. Copyright © 2018 by the American Society of Nephrology.
Effect of colchicine-resistant familial mediterranean fever on growth parameters [Kolşisine dirençli ailesel akdeniz ateşinin büyüme parametlerine etkisi]
(2011) Sözeri B.; Yilmaz E.; Mir S.; Berdeli A.
Objectives: This study aims to evaluate the growth and development in patients with Familial Mediterranean fever (FMF) resistant to colchicine treatment. Patients and methods: Eighty-seven patients (45 females, 42 males; mean age 9.6±3.8 years; range 2 to 17 years) diagnosed with FMF according to Tel Hashomer criteria in the Pediatric Nephrology Department of Ege University Faculty of Medicine were included in the study. All patients had leukocytosis and elevated C-reactive protein, fibrinojen levels, erythrocyte sedimentation rate and serum amyloid A levels during attacks. The patients were divided into t wo group s accor ding to colchicines treatment response. Twenty-seven patients (13 girls, 14 boys; mean age; 9.9±3.2 years) having frequent attacks (>1 attact/3 month) despite receiving 2 mg/day colchicine treatment were defined as colchicine-resistant. Sixty patients were defined as colchicine treatment responders. Anthropometric evaluations of the patients were performed at diagnosis and at the end of the follow-up. The height and weight Z scores of patients were used as growth parameters. Results: No statistically significant difference in mutation frequency was found between the groups. There were no significant differences between both groups in the weight and height Z scores calculated from the anthropometric parameters detected at the diagnosis. In the evaluation performed at the end of the follow-up, we found that the height and weight score for age and the body mass index Z score were significantly decreased in the colchicine resistance group (p=0.008, p=0.013, p=0.027). Conclusion: Effective suppression of inflammatory response in patients with FMF provides a positive impact on growth. While colchicine is known as the most effective drug in the treatment of FMF, it is known that there are also patients who fail to respond adequately. The patients with colchicine-resistant FMF should be identified in the early period of the disease and treatment should be arranged accordingly. The detection of growth retardation was found to be important in detecting resistance to colchicine in patients with FMF. © 2011 Turkish League Against Rheumatism. All rights reserved.
The effect of primary hypertension on cardiovasculary system and diagnostic methods in childhood: Review [Çocukluk döneminde primer hipertansiyonun kardiyovasküler sistem üzerine etkisi ve tani yöntemleri]
(2012) Sözeri B.; Mir S.
Primary hypertension in children is often lately because of increasing application in relation to obesity is increasing. Hypertension is a known risk factor for cardiovasculary disease in adults, and the presence of childhood hypertension may contribute to the early development of cardiovasculary disease. Recent studies have shown that changes of cardiovasculary system are increased in hypertensive as compared with normotensive children. The pathological basis for cardiovasculary disease is arterial damage in the form of arteriosclerosis. Arteriosclerosis is a broader term that usually describes diffuse thickening and stiffening of mainly large- and medium-sized arteries and is observed in different conditions. Among them, arterial hypertension is the most important factor. Sustained elevation of blood pressure impacts not only the cardiovascular system, but also other structures, such as the kidney and central nervous system, producing so-called hypertension-induced target organ damage. The damage in the developing cardiovascular system, increases the mortality of the disease. The relationship between early markers such as, left ventricule hypertrophy, carotid wall thickness and aortic pulse wave velocity, and blood pressure values in children and adolescents has been the focus of several studies. The prompt recognition of early organ damage can help in making decisions about the convenience of starting pharmacological treatment aiming to reduce hypertension-induced consequences later in life. Moreover, monitoring changes in organ damage during antihypertensive treatment can offer valuable information about the success of the treatment itself. In this review the threshold of childhood primary hypertension and cardiovascular changes that significantly affect the prognosis of the disease and their evaluation methods will be discussed. Copyright © 2012 by Türkiye Klinikleri.
The effect of recurrent urinary tract infections on somatic growth in children [Çocuklarda tekrarlayan idrar yolu enfeksiyonlarinin büyüme üzerine etkisi (ön çalişma)]
(2008) Keskinoglu A.; Mir S.
Aim: Urinary tract infection is one of the common bacterial infections in children and may lead to substantial morbidity. In this study, the effect of recurrent urinary tract infections on the growth of children aged between 0-12 years was investigated. Material and Method: In this prospective study, 72 children who had the definite diagnosis of recurrent urinary tract infections and followed up for at least 6 months, in a period of one year in the outpatient clinic of nephrology were included. The infection was evaluated with urine culture and growth charts. Data were analyzed with Pearson and Mc Nemar chi-square tests, t-test, Mann Whitney U test and corelation analysis. Results: The mean age of the children was 42.9 months, 66.7% of those were females. Height for age and weight for age were found to be lower in 16.7% and 22.2%, respectively. Escherischia coli was the most common pathogen found in urine cultures. Renal scarring was determined in 20.8% of the subjects. While the attack numbers of urinary trcat infections were increasing, height and weight measurements for age were significantly decreasing. Weight for age was significantly low in boys at the beginning of the study. Weight for age score improved after a 6-month follow-up period. Conclusions: The treatment and the prophylaxy of the recurrent urinary tract infections resulted in a positive effect on growth of the children.
An Elevated Hemidiaphragm 3 Months after Internal Jugular Vein Hemodialysis Catheter Placement
(2003) Mir S.; Serdaroglu E.
Phrenic nerve palsy following central venous catheterization is a rare complication and is not well recognized. We present a 33 months old girl who has renal failure secondary to nephrotic syndrome. A left internal jugular catheter was placed using the Seldinger technique after a single injection of 2 ml prilocaine hydrochloride for local anesthesia and a single internal jugular vein cannulation. Subsequent chest roentgenograms confirmed proper catheter and diaphragm position. Three months after catheter placement, decreased breath sounds on the left side of the chest were noted. Left phrenic nerve palsy was demonstrated with fluoroscopy and electromyography with external diaphragmatic elecrodes. The nerve damage was delayed after catheter placement, it seems unlikely that it was related to direct nerve trauma from the cannulation needle, local anesthetic infiltration of the nerve, or subsequent hematoma formation in this case. The phrenic nerve is in close proximity to both the catheter and the vein in which the catheter rests, an inflammatory reaction related to the catheter has been suggested as the cause for the nerve damage.
Genetic, environmental, and disease-associated correlates of vitamin D status in children with CKD
(American Society of Nephrology, 2016) Doyon A.; Schmiedchen B.; Sander A.; Bayazit A.; Duzova A.; Canpolat N.; Thurn D.; Azukaitis K.; Anarat A.; Bacchetta J.; Mir S.; Shroff R.; Yilmaz E.; Candan C.; Kemper M.; Fischbach M.; Cortina G.; Klaus G.; Wuttke M.; Köttgen A.; Melk A.; Querfeld U.; Schaefer F.; 4C Study Consortium
Background and objectives: Vitamin D deficiency is endemic in children with CKD.We sought to investigate the association of genetic disposition, environmental factors, vitamin D supplementation, and renal function on vitamin D status in children with CKD. Design, setting, participants, & measurements Serum: 25-hydroxy-vitamin D, 1,25-dihydroxy-vitamin D, and 24,25-dihydroxy-vitamin D concentrations were measured cross-sectionally in 500 children from 12 European countries with CKD stages 3-5. All patients were participants of the Cardiovascular Comorbidity in Children with Chronic Kidney Disease Study, had CKD stage 3-5, and were age 6-18 years old. Patients were genotyped for single-nucleotide polymorphisms in the genes encoding 25-hydroxylase, vitamin D binding protein, 7-dehydrocholesterol reductase, and 24-hydroxylase. Associations of genetic status, season, local solar radiation, oral vitamin D supplementation, and disease-associated factors with vitamin D status were assessed. Results: Two thirds of patientswere vitamin D deficient (25-hydroxy-vitamin D<16 ng/ml). 25-Hydroxy-vitamin D concentrations varied with season and were twofold higher in vitamin D-supplemented patients (21.6 [14.1] versus 10.4 [10.1] ng/ml; P<0.001). Glomerulopathy, albuminuria, and girls were associated with lower 25-hydroxy-vitamin D levels. 24,25-dihydroxy-vitamin D levels were closely correlated with 25-hydroxy-vitamin D and 1,25-dihydroxy-vitamin D (r=0.87 and r=0.55; both P<0.001). 24,25-dihydroxy-vitamin D concentrations were higher with higher c-terminal fibroblast growth factor 23 and inversely correlated with intact parathyroid hormone. Whereas 25-hydroxy-vitamin D levels were independent of renal function, 24,25-dihydroxy-vitamin D levels were lower with lower eGFR. Vitamin D deficiency was more prevalent in Turkey than in other European regions independent of supplementation status and disease-related factors. Single-nucleotide polymorphisms in the vitamin D binding protein genewere independently associated with lower 25-hydroxy-vitamin Dand higher 24,25-dihydroxy-vitamin D. Conclusions: Disease-related factors and vitamin D supplementation are the main correlates of vitamin D status in children with CKD. Variants in the vitamin D binding protein showedweak associations with the vitamin D status. © 2016 by the American Society of Nephrology.
Genetics and consequences of atypical hemolytic-uremic syndrome in Turkish children
(Iranian Society of Nephrology, 2019) Conkar S.; Mir S.; Berdeli A.
Introduction. Atypical hemolytic uremic syndrome (aHUS) is associated with mutations or antibodies that affect the regulation of the alternative complement pathway. Several studies were published recently, describing these mutations. We present the initial clinical findings, treatments, and long-term follow-up results of 19 patients hospitalized with the diagnosis of aHUS. Methods. Nineteen patients who were diagnosed as aHUS were enrolled from January 2010 to March 2017. Initial clinical signs and clinical follow-up of patients with aHUS were evaluated. The reasons for complement factor H (CFH) mutations were investigated. Results. CFH mutations were detected in 5 of the 19 aHUS cases. Of these, one was novel, while four were previously reported. We reported here the clinical course of aHUS patients with CFH previously defined mutations (p.Glu936Asp, Val 1197Ala) and a novel mutation (Glu927Lys), which caused previously defined aHUS. Two of the CFH mutation cases developed end-stage kidney disease that required hemodialysis, and one patient developed chronic kidney disease. Two cases were in remission; one of them under supportive therapy and the other one in remission with eculizumab treatment. Conclusions. Morbidity rates are higher in children with aHUS. However, renal prognosis and morbidity rates are higher in children with CFH mutations than other children with aHUS. Poor prognosis in aHUS-children with CFH mutation depends on the genetic background. © 2019, Iranian Society of Nephrology.

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