Mesiha Ekim

Ankara University, Engüri, Ankara, Turkey

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Publications (140)354.25 Total impact

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    ABSTRACT: Colchicine is the standard treatment in familial Mediterranean fever (FMF) patients. New treatment strategies are needed in FMF patients who were unresponsive to colchicine therapy or who had developed amyloidosis. The aim of this study was to present clinical-laboratory features and treatment responses of pediatric FMF patients that were treated with anti-IL-1 therapies. Files of patients who had been followed in our department with diagnosis of FMF were retrospectively evaluated. Patients that have been receiving anti-IL-1 therapies (anakinra or canakinumab) were included to the study. All patients were interpreted with respect to the demographic data, clinical and laboratory features of the disease, genetic analysis of MEFV mutations and treatment responses. Among 330 currently registered FMF patients, 13 patients were included to the study. Seven of them received anti-IL-1 therapy due to colchicine resistance and 6 due to FMF-related amyloidosis (1 of them with nephrotic syndrome, 2 with chronic kidney disease, 3 with renal transplantation). In all treated patients, attacks completely disappeared or decreased in frequency; partial remission occured in nephrotic syndrome patient; and their life quality improved. Anti-IL-1 therapies can be successfully used in colchicine-resistant FMF patients and patients with amyloidosis during childhood and adolescent period without major side effects.
    Clinical Rheumatology 09/2014; · 1.77 Impact Factor
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    ABSTRACT: Systemic AA amyloidosis is a serious complication of many chronic inflammatory disorders and chronic infections. Renal involvement is seen in the majority of the patients and can lead to end-stage renal disease. Renal transplantation can be performed in these patients; however, amyloidosis can recur in the transplanted kidneys. On the other hand, de novo AA amyloidosis in renal transplant patients has been rarely reported. We report a 17-yr-old patient with end-stage renal disease due to genitourinary anomalies who developed recurrent pyelonephritis after transplantation. Three yr after transplantation, renal biopsy was performed for proteinuria and AA amyloidosis was identified in the renal allograft. Although rare, chronic infections might cause de novo amyloidosis in renal transplant patients. Therefore, amyloidosis should be kept in mind in those types of patients who present with proteinuria.
    Pediatric Transplantation 09/2014; · 1.63 Impact Factor
  • Pediatric nephrology (Berlin, Germany). 08/2014;
  • Pediatric nephrology (Berlin, Germany). 08/2014;
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    ABSTRACT: Previous studies suggest that 6-46% of children suffer from lower urinary tract dysfunction (LUTD). This study evaluated the prevelance of LUTD in children with a urinary tract infection (UTI) and assessed the impact of standard urotherapy on patients with LUTD.
    Acta paediatrica (Oslo, Norway: 1992). Supplement 06/2014;
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    ABSTRACT: There is increasing focus on the problems involved in the transition and transfer of young adult patients from paediatric to adult renal units. This situation was addressed by the 2011 International Pediatric Nephrology Association/International Society of Nephrology (IPNA/ISN) Consensus Statement on transition. We performed a survey of transition practices of 15 paediatric nephrology units across Europe 2 years after publication of the consensus statement. Two thirds of units were aware of the guidelines, and one third had integrated them into their transition practice. Forty-seven per cent of units transfer five or fewer patients with chronic kidney disease (CKD) stage 5 per year to a median of five adult centres, with higher numbers of CKD stages 2-4 patients. Seventy-three per cent of units were required by the hospital or government to transfer patients by a certain age. Eighty per cent of units commenced transition planning after the patient turned 15 years of age and usually within 1-2 years of the compulsory transfer age. Forty-seven per cent of units used a transition or transfer clinic. Prominent barriers to effective transition were patient and parent attachment to the paediatric unit and difficulty in allowing the young person to perform self-care. Whereas awareness of the consensus statement is suboptimal, it has had some impact on practice. Adult nephrologists receive transferred patients infrequently, and the process of transition is introduced too late by paediatricians. Government- and hospital-driven age-based transfer policies distract focus from the achievement of competencies in self care. Variable use of transition clinics, written patient information and support groups is probably due to economic and human-resource limitations. The consensus statement provides a standard for evolving and evaluating transition policies jointly agreed upon by paediatric and adult units.
    Pediatric Nephrology 04/2014; · 2.88 Impact Factor
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    ABSTRACT: The pathogenesis of Henoch-Schönlein Purpura (HSP) has not been clearly defined. Inflammatory cytokines have been associated with HSP but there are only a few reports that have focused on coagulation. The endothelial protein C receptor (EPCR), which has anticoagulant and antiinflammatory activity, is the key component of the protein C pathway. Recent studies have implicated the soluble form of EPCR (sEPCR) in Wegener's granulomatosis, Behçet's disease, and systemic lupus erythematosus. The aim of this study was to evaluate the levels of sEPCR in HSP children. Twenty-two children with HSP and 17 healthy children were included. We found no significant differences (P > .05) between patient and control groups in the levels of von Willebrand factor and thrombomodulin. The median sEPCR values in the HSP group were lower than the control group (79 vs. 102 ng/mL, respectively) (P > .05). The mean sEPCR value in HSP patients with severe abdominal pain was lower than without (88.8 ± 54.9 vs. 108.2 ± 66.3 ng/mL, respectively) (P > .05). In addition, the mean IL-6 serum levels were significantly elevated in HSP patients during the acute stage of HSP (2.1 ± 1.7 vs. 1.5 ± 1.2 pg/mL, P = .038). We also observed a slight negative correlation between the levels of sEPCR and IL-6 (R = -.135, P > .05). To our knowledge, this was the first study to analyze sEPCR levels in HSP. Our results did not conclusively identify a direct role of sEPCR in HSP, but our findings warrant further investigations, especially in severe HSP cases characterized by gastrointestinal bleeding or renal involvement.
    Pediatric Hematology and Oncology 12/2013; · 0.90 Impact Factor
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    ABSTRACT: Many factors may impact upon choice of renal replacement therapy (RRT) for children and adolescents, including patient and family choice, patient size and distance from the renal centre as well as logistic issues such as facilities and staffing at the unit. We report a survey of factors influencing treatment choice in 14 European paediatric nephrology units. METHODS: A questionnaire was developed by consensus and completed by 14 members of the European Paediatric Dialysis Working Group on facilities, staffing and family assessments impacting on choice of therapy as well as choice of therapy for 97 patients commencing initial RRT in 2011. RESULTS: All units offered all modalities of RRT, but there were limitations for pre-emptive transplantation (PET) and largely adult surgical dependence for creation of arteriovenous fistulae and transplantation. The average waiting time for a deceased donor kidney was 18.5 (range 3-36) months. Full time dietetic support was available in six of the 14 units. There was no social worker, psychology, play therapy or teaching support in three, two, seven and four units, respectively. Assessment by other members of the multidisciplinary team and home visits before choice of therapy was carried out in 50 % of units, and although all patients were discussed at team meetings, the medical opinion predominated. In terms of types of RRT, 50 % of patients were commenced on chronic peritoneal dialysis (PD), 34 % on haemodialysis (HD) and 16 % underwent pre-emptive transplantation (PET). Chronic PD predominated in patients aged <5 years and HD predominated in those aged >10 years. Patient and family choice and age or size of patient were predominant factors in choice of therapy with a predictable decline in renal function favouring PET and social factors HD. CONCLUSIONS: Chronic peritoneal dialysis predominated as primary choice of RRT, especially in younger children. The PET rates remain low. The influence of surgeons predominanted, and national transplant rules may be significant. Most units had insufficient multiprofessional support, which may impact upon initial choice of therapy as well as sustaining families through RRT.
    Pediatric Nephrology 12/2013; Epub ahead of print. · 2.88 Impact Factor
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    ABSTRACT: Renal transplantation is the treatment of choice for children with end-stage renal disease. The aim of this study was to evaluate retrospectively of our 37 pediatric renal allograft recipients, including 20 boys and 17 girls from July 2007 to August 2012. The overall mean age at transplantation was 12.16 ± 4.25 years. Three patients (8.1%) were transplanted preemptively; two were ABO-incompatible transplantations. The majority of recipients received living donor grafts (81%). The mean duration of follow-up was 25.10 ± 14.95 months. Seven acute rejection episodes were observed in 6 patients (16.2%). Eleven recipients developed serious viral infections: cytomegalovirus (n = 8), parvovirus (n = 2), BK virus (polyoma hominis 1) (n = 2), or Ebstein-Barr virus (n = 1). Three patients died; one from posttransplant lymphoproliferative disease, one from primary disease recurrence with infection, and one from sepsis. In conclusion, kidney transplantation is the treatment of choice for end-stage renal disease. Infection was the major concern after this procedure.
    Transplantation Proceedings 04/2013; 45(3):917-8. · 0.95 Impact Factor
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    ABSTRACT: Background Encapsulating peritoneal sclerosis (EPS) is a rare complication of peritoneal dialysis (PD) that is associated with significant morbidity and mortality in adults. There are scarce data for children. We performed a 10-year survey to determine the prevalence, risk factors and outcome for EPS in children.Methods Chronic PD patients in 14 dialysis units participating in the European Paediatric Dialysis Working Group between January 2001 and December 2010 were included in this study.ResultsTwenty-two cases of EPS were reported (prevalence 1.5%; 8.7 per 1000 patient-years on PD). Median PD vintage was 5.9 (1.6-10.2) in EPS and 1.7 (0.7-7.7) years in the remainder of the PD population (P < 0.0001). EPS patients had a significantly higher peritonitis rate than non-EPS patients (P = 0.2). EPS was diagnosed while the child was on PD in 17 (77%), after conversion to haemodialysis (HD) in 3 and after transplantation in 2. Fifteen of 17 (88%) developed ultrafiltration (UF) failure. The median interval between UF failure and presentation with bowel obstruction was 2.8 (0.02-5.8) months. Twenty (91%) had clinical and radiological signs of bowel obstruction. Enterolysis was performed in 14 and 19 received immunosuppression or tamoxifen. Nine required parenteral nutrition. At final follow-up 4.8 (1.3-8.7) years after EPS diagnosis, 3 patients died, 11 had a functioning transplant and 8 were on HD.Conclusions The prevalence of EPS in European children on PD is comparable with that of adult PD patients, but mortality from paediatric EPS is significantly lower. A high index of suspicion is required for the diagnosis of EPS in children with longer dialysis duration, a high peritonitis rate and UF failure.
    Nephrology Dialysis Transplantation 01/2013; · 3.37 Impact Factor
  • Pediatric Nephrology 06/2012; · 2.88 Impact Factor
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    Pediatric Nephrology 06/2012; · 2.88 Impact Factor
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    ABSTRACT: Familial Mediterranean fever (FMF) is an autosomal recessive disease characterized by recurrent self-limited attacks of fever and polyserositis. Reactive amyloid A amyloidosis is the most devastating complication of FMF, and amyloidosis continues to occur in the colchicine era in untreated and noncompliant patients. Unfortunately, there is no proven effective treatment for established amyloidosis. In this report, we present four FMF-related amyloidosis patients that were treated with long term infliximab therapy with the longest duration of follow-up, together with the literature review. Infliximab was very effective in controlling gastrointestinal system findings and protracted arthritis, and it also had a favorable impact on the clinical findings of nephrotic syndrome in these patients. In conclusion, by controlling debilitating complaints of amyloidosis with infliximab, quality of life increases in these patients, and they get rid of recurrent hospitalizations and return to school or work.
    Clinical Rheumatology 06/2012; 31(8):1267-71. · 1.77 Impact Factor
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    ABSTRACT: Previous studies have suggested that inflammatory bowel disease is particulary frequent and severe in familial Mediterranean fever (FMF) families. An 8-month-old boy was admitted to our hospital with chronic bloody diarrhea, failure to thrive and high-grade fever. He was diagnosed as Crohn's disease (CD) based on clinical, laboratory and histological findings and, corticosteroid therapy was started. The patient did not respond to intensive medical therapy including intravenous corticosteroid, mesalazine, azathioprine, intravenous cyclosporine and enteral feeding. MEFV gene mutation analysis revealed homozygous M694V mutation. In addition to azathioprine and cyclosporine therapy, with the diagnosis of FMF, colchicine therapy was started and partial remission was observed within 2 weeks. To the best of our knowledge, this is the first report of association of CD and FMF in an infant. In cases of CD resistant to medical therapy, possibility of underlying FMF should be considered, especially in countries where FMF is prevalent.
    Rheumatology International 03/2012; 32(3):783-5. · 1.63 Impact Factor
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    ABSTRACT: The purpose of our study was to evaluate and analyse the prevalence and association of acute kidney injury (AKI) as defined by paediatric Risk, Injury, Failure, Loss of kidney function and End-stage kidney disease (pRIFLE) and Acute Kidney Injury Network (AKIN) classifications in a paediatric intensive care unit (PICU). A prospective analysis of all patients that were admitted to our PICU between June 2009 and December 2010 was performed. Patients were classified according to AKIN and pRIFLE criteria. One hundred and eighty-nine patients (mean age 45.9 ± 54.7 months; 110 male, 79 female) were enrolled. Sixty-three (33.3%) patients developed AKI by AKIN criteria and 68 (35.9%) patients developed AKI by pRIFLE criteria. All patients that had AKI according to AKIN criteria also had this diagnosis with pRIFLE criteria. Five patients had developed AKI only according to pRIFLE classification, four of them owing to reduction in their estimated creatinine clearance and one of them owing to changes over 1-week period. The mean length of PICU stay was longer, need for mechanical ventilation and mortality rates were higher in patients with AKI when compared to patients without AKI. Although both pRIFLE and AKIN criteria were very helpful in the detection of patients with AKI even in the early stages of it, pRIFLE seems to be more sensitive in paediatric patients.
    Acta Paediatrica 11/2011; 101(3):e126-9. · 1.97 Impact Factor
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    ABSTRACT: It is well known that the serum procalcitonin (PCT) levels increase in severe bacterial infections. However, there is little information about the levels of PCT in diverse diseases except mainly the infectious diseases. The aim of this study was to investigate the progress of serum levels of PCT together with traditional acute phase reactants in children with familial Mediterranean fever (FMF) during the attack and attack-free periods and to test whether PCT could help to diagnose the attack in FMF patients. The study group comprised 21 FMF patients (mean age 10 ± 4.6 years) and 19 healthy controls (mean age 10.6 ± 4.2 years). Serum levels of PCT and traditional acute phase reactants were measured during the attack and attack-free periods. Blood samples were obtained within the first 6-24 h of the attack period, 7 days later, and at least 2 months after the attack. Traditional acute phase reactants (hs-CRP, ESR, and fibrinogen) during the attack period were significantly higher than the attack-free levels and controls. PCT levels of the FMF patients during the attack period were also significantly higher than the attack-free and control group levels (median values, 0.044 ng/ml vs. 0.028 ng/ml and 0.031 ng/ml, P = 0.04, respectively). Although this difference was statistically significant (P = 0.04), median PCT values of the attack, attack-free period, and healthy subjects were lower than 0.05 ng/ml. As a result, these findings suggested that PCT levels were not conspicuously affected from inflammation and could not be used as a descriptive marker for attack in FMF patients.
    Rheumatology International 11/2011; · 1.63 Impact Factor
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    ABSTRACT: DICER1 is crucial for embryogenesis and early development. Forty different heterozygous germline DICER1 mutations have been reported worldwide in 42 probands that developed as children or young adults, pleuropulmonary blastoma (PPB), cystic nephroma (CN), ovarian sex cord-stromal tumors (especially Sertoli-Leydig cell tumor [SLCT]), and/or multinodular goiter (MNG). We report DICER1 mutations in seven additional families that manifested uterine cervix embryonal rhabdomyosarcoma (cERMS, four cases) and primitive neuroectodermal tumor (cPNET, one case), Wilms tumor (WT, three cases), pulmonary sequestration (PS, one case), and juvenile intestinal polyp (one case). One carrier developed (age 25 years) a pleomorphic sarcoma of the thigh; another carrier had transposition of great arteries (TGA). These observations show that cERMS, cPNET, WT, PS, and juvenile polyps fall within the spectrum of DICER1-related diseases. DICER1 appears to be the first gene implicated in the etiology of cERMS, cPNET, and PS. Young adulthood sarcomas and perhaps congenital malformations such as TGA may also be associated.
    Human Mutation 08/2011; 32(12):1381-4. · 5.05 Impact Factor
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    ABSTRACT: Aluminum (Al) is an ingredient of a variety of foodstuffs and medications as well as of domestic water supplies. The patients with chronic kidney disease (CKD) are more susceptible to bone toxicity of Al. The aim of the study was to investigate the interactions between serum Al, parathyroid hormone (PTH) and active vitamin-D in CKD. A total of 10 pediatric patients with CKD and 20 healthy controls were enrolled in study. The blood calcium, aluminum, PTH, alkaline phosphatase and phosphorus were evaluated at onset and following a regimen of oral 1,25 dihydroxycholecalciferol (1,25 DHC) for 4 weeks. Although median values of PTH, calcium, phosphorus and alkaline phosphatase did not differ (P > 0.05) after calcitriol administration, the aluminum levels (median: 27.2 ng/ml, range: 11.3-175) declined significantly (median: 3.8 ng/ml, range: 0.64-11.9) after a regimen of oral 1,25 DHC for 4 weeks in all participants (P < 0.05). The median levels of aluminum after 1,25 DHC did not show statistically significant difference with median aluminum levels of healthy controls (median: 2.5 ng/ml, range: 0.2-33.2) (P < 0.05). Calcitriol may lead to decline in serum Al levels in CKD patients.
    International Urology and Nephrology 06/2011; 43(2):467-70. · 1.29 Impact Factor
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    ABSTRACT: We report the case of a child with clinical and radiological signs of nutcracker syndrome who had a history of inferior vena cava ligation during a previous surgery. He was referred for evaluation of abdominal pain and hematuria. Entrapment of the left renal vein between the superior mesenteric artery and the aorta with aneurysmal dilatation was detected on Doppler ultrasonography. Magnetic resonance angiography revealed paravertebral and epidural collateral vessels.
    Journal of Clinical Ultrasound 03/2011; 39(7):418-21. · 0.80 Impact Factor

Publication Stats

920 Citations
354.25 Total Impact Points


  • 1992–2014
    • Ankara University
      • • Department of Pediatric Nephrology
      • • Department of Pediatric Gastroenterology
      • • Department of Radiology
      • • Department of Pediatric Genetic Disorders
      • • Faculty of Medicine
      • • Department of Pediatrics
      Engüri, Ankara, Turkey
  • 2011
    • Pamukkale University
      Denisli, Denizli, Turkey
  • 2006
    • Case Western Reserve University School of Medicine
      Cleveland, Ohio, United States