N Tümer

Ankara University, Engüri, Ankara, Turkey

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Publications (49)150.68 Total impact

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    Cüneyt Ensari · Arzu Ensari · Necmiye Tümer · Ergun Ertug
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    ABSTRACT: The aim of the present study was to assess the correlation of immunohistochemical subtyping with clinical diagnosis in order to achieve useful epidemiological data regarding amyloidosis in Turkish patients. We carried out immunohistochemical studies on 128 biopsies from various sites of 111 patients with biopsy-proven amyloidosis and, based on the results, classified the patients. We assessed the correlation of immunohistochemical subtype with clinical diagnosis and gathered epidemiological data. The sites most biopsied were kidney and rectum, followed by the testicle, liver, small intestine and bladder. Amyloid deposits showed positive staining with a single antibody in 120 biopsies. Pure amyloid A (AA) positivity was seen in 113 biopsies; six biopsies were positive for amyloid lambda (AL) and one for beta2-microglobulin (beta2MG). The clinical diagnoses of 81 patients (98 biopsies all AA positive) were suggestive of familial Mediterranean fever (FMF). Also AA positive were eight patients with tuberculosis, seven patients with rheumatoid arthritis, four patients with bronchiectasis and one patient with Crohn's disease. The biopsies from seven patients clinically suspected to have plasma cell dyscrasias were AL positive. One patient undergoing haemodialysis was beta2MG positive. Two patients without definite diagnoses showed double or triple positivity, which could not be interpreted and classified immunohistochemically. This study demonstrates that the predominant association of AA amyloidosis is with FMF. It also suggests that the routine immunohistochemical study of patients with amyloidosis who are of certain ethnic backgrounds suffices for classifying the subtype of amyloid fibril protein and the related disease.
    Preview · Article · Sep 2005 · Nephrology Dialysis Transplantation
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    Preview · Article · Sep 2002 · Peritoneal dialysis international: journal of the International Society for Peritoneal Dialysis
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    ABSTRACT: Familial Mediterranean fever (FMF) is an autosomal recessive disorder. Although the pathogenesis of the disease is not yet completely understood, enhanced acute-phase responsiveness is considered to be one of the most important mechanisms. The presence of high levels of antistreptolysin O (ASO) antibodies and streptococcus-associated diseases, such as acute poststreptococcal glomerulonephritis (AGN) and acute rheumatic fever (ARF), has been reported in patients with FMF. In order to better understand the effect of FMF on antistreptococcal antibody response, we measured ASO and antideoxyribonuclease B (anti-DNAse B) levels in patients with FMF and compared them with those in healthy controls. The study consisted of two parts. In the first step, antistreptococcal antibody levels were analysed in 44 patients with FMF and 165 healthy children who had no history or clinical evidence of upper respiratory tract infection (URTI) for the last 4 months. In the second step, antistreptococcal antibody levels were measured in 15 patients with FMF and 22 healthy controls in response to documented group A beta-haemolytic streptococcal pharyngitis. In the first part of the study, ASO and anti-DNAse B levels in patients with FMF were found to be significantly higher than those in healthy controls (P<0.001). In the second part, ASO and anti-DNAse B titres were found to be significantly higher in patients with FMF than in controls (P<0.001 and <0.05, respectively) 4 weeks after a positive throat culture. We concluded that patients with FMF have an exaggerated response to streptococcal antigens and might be prone to poststreptococcal non-suppurative complications, such as ARF.
    No preview · Article · Sep 2002 · Clinical Rheumatology
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    ABSTRACT: Background: Cardiovascular complications are the most frequent cause of death in patients with end-stage renal failure (ESRF). We aimed to investigate systolic and diastolic functions in children with ESRF.Methods: Thirty-nine children with ESRF (17 on continuous ambulatory peritoneal dialysis (CAPD), eight on hemodialysis and 14 on predialysis) were examined to assess systolic and diastolic functions by echocardiography and ultrasound Doppler. Left ventricular systolic and diastolic functions were measured both in patients and age-matched healthy controls (n = 20) and the indices of cardiac performance were compared.Results: Increased left ventricular mass index (LVMI) and decreased volume/mass ratio with normal systolic left ventricular function was found in patients, as compared with controls. Left ventricular diastolic dysfunction was observed in dialysis patients. In most of these patients, left ventricular isovolumic relaxation time was prolonged, except in CAPD patients. The peak of late diastolic flow (A) velocities were increased with a reduction of the early diastolic flow velovity (E) – the E/A ratio. The E velocities were unchanged in all patients as compared with controls. Our data indicated an abnormality of myocardial relaxation in patients with ESRF. We found no relationship between E/A ratio and LVMI. Among three groups of patients, the LVMI and diastolic abnormalities were highest in the hemodialysis group indicative of poor control of hypervolemia and hypertension.Conclusions: The technique of CAPD has some advantages as a renal replacement therapy for preserving cardiac functions as compared with hemodialysis. However, it must be remembered that patients with hemodialysis have features that effects cardiac status, such as higher volume load and higher afterload (hypertension).
    No preview · Article · Mar 2002 · Pediatrics International
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    ABSTRACT: Cardiovascular complications are the most frequent cause of death in patients with end-stage renal failure (ESRF). We aimed to investigate systolic and diastolic functions in children with ESRF. Thirty-nine children with ESRF (17 on continuous ambulatory peritoneal dialysis (CAPD), eight on hemodialysis and 14 on predialysis) were examined to assess systolic and diastolic functions by echocardiography and ultrasound Doppler. Left ventricular systolic and diastolic functions were measured both in patients and age-matched healthy controls (n = 20) and the indices of cardiac performance were compared. Increased left ventricular mass index (LVMI) and decreased volume/mass ratio with normal systolic left ventricular function was found in patients, as compared with controls. Left ventricular diastolic dysfunction was observed in dialysis patients. In most of these patients, left ventricular isovolumic relaxation time was prolonged, except in CAPD patients. The peak of late diastolic flow (A) velocities were increased with a reduction of the early diastolic flow velocity (E)--the E/A ratio. The E velocities were unchanged in all patients as compared with controls. Our data indicated an abnormality of myocardial relaxation in patients with ESRF. We found no relationship between E/A ratio and LVMI. Among three groups of patients, the LVMI and diastolic abnormalities were highest in the hemodialysis group indicative of poor control of hypervolemia and hypertension. The technique of CAPD has some advantages as a renal replacement therapy for preserving cardiac functions as compared with hemodialysis. However, it must be remembered that patients with hemodialysis have features that effects cardiac status, such as higher volume load and higher afterload (hypertension).
    No preview · Article · Mar 2002 · Pediatrics International

  • No preview · Article · Nov 2001 · Pediatrics International
  • Gülhis Deda · Mesiha Ekim · A Güven · Ugur Karagöl · Necmiye Tümer
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    ABSTRACT: Neurologic manifestations can accompany systemic diseases, and primary disease can be identified with a careful history, physical examination, and laboratory investigations. A 14-year-old girl with paraplegia and absence of deep tendon reflexes in the lower extremities after 2 days of vomiting and diarrhea was referred to our pediatric neurology department with a diagnosis of Guillain-Barré syndrome. Short stature, dehydration, motor and mental retardation, bilateral cataracts, glaucoma, and band keratopathy were detected on physical examination. Hypopotassemia and severe metabolic acidosis were found on biochemical examination. Her paraplegia improved after appropriate fluid and electrolyte replacement, but metabolic acidosis persisted after cessation of intravenous therapy, and isolated proximal renal tubular acidosis was detected. Because she had isolated proximal renal tubular acidosis and other abnormalities, she was diagnosed with Donckerwolcke-Winsnes syndrome.
    No preview · Article · Nov 2001 · Journal of Child Neurology
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    ABSTRACT: Familial Mediterranean fever (FMF) is an autosomal recessive disorder of childhood characterized by attacks of fever and serositis. Renal amyloidosis is the most important complication of the disease that determines the prognosis. Forty-eight Turkish FMF patients with amyloidosis who have been followed at the two hospitals in Ankara were included in this study. All patients with amyloidosis had been symptomatic for FMF at the time of the diagnosis (Phenotype I), none had received regular colchicine therapy and all presented with proteinuria. Ten of them had asymptomatic proteinuria; 38 had nephrotic syndrome and 8 of them had renal insufficiency (CRI) as well, at the time of the diagnosis. Regular colchicine therapy was commenced to all of the patients. At the end of observation period of 4.5 +/- 2.23 years (range 2-12 yrs) on treatment, nephrotic syndrome resolved in 13 patients and proteinuria was lost in 5 of them. None but 2 of the patients who were diagnosed at proteinuric stage progressed to end stage renal failure (ESRF). Seven MEFV mutations (M694V, M680I, V726A, M694I, K695R, R761H, E148Q) were systematically investigated in 32 patients. Six of the seven studied mutations were found in these patients and clinical diagnosis was confirmed by mutation analysis in 24 patients. Eight patients were found to have mutations on one of the alleles. Amyloidosis is the most serious complication of FMF. Colchicine treatment ameliorates the progression of renal disease in the patients who presented with proteinuria and even with nephrotic syndrome. No correlation between the outcome of the patients with nephrotic syndrome and the degree of proteinuria and/or serum albumin levels at the initiation of treatment were noted. Progression to ESRF seems inevitable despite colchicine therapy after the development of CRI in patients with FMF associated amyloidosis.
    Full-text · Article · Sep 2001 · Clinical and experimental rheumatology
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    ABSTRACT: Various immunological abnormalities leading to impaired immune status have been described in uraemic adults; however, few data are available for uraemic children. In this study, peripheral blood total lymphocyte count and lymphocyte subsets (CD3+, CD4+, CD8+, CD16+, CD20+) were evaluated, skin tests with PPD and Candida antigens were performed, and serum immunoglobulin (IgG, IgA, IgM) and complement (C3, C4) levels were measured in 30 children with end-stage renal failure (10 before dialysis, 10 on continuous ambulatory peritoneal dialysis, and 10 on haemodialysis) and the results compared with those of 15 healthy controls. The data showed significant lymphopenia in predialysis and haemodialysis groups. No significant change was observed in the CD4+/CD8+ ratio or in the percentages of lymphocyte subsets in either group studied, while the absolute values of some lymphocyte subsets were significantly lower in all groups as compared with controls. In skin test evaluation, only the patients in the predialysis group showed a significantly decreased response to Candida antigen. The serum immunoglobulin levels were significantly decreased in the continuous ambulatory peritoneal dialysis group as compared with the control group. Our results, together with those of other paediatric studies, reported in the literature, suggest that uraemic children are not immunocompromised, though the effects of uraemia may cause some variation in their immune status.
    No preview · Article · Sep 2001 · Nephron
  • SA Bakkaloglu · Mesiha Ekim · Necmiye Tümer · Özden Tulunay · T Ozer
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    ABSTRACT: A 14-year-old boy with classic polyarteritis nodosa (cPAN) and a clinical picture resembling rapidly progressive glomerulonephritis (RPGN) is described. He had severe hypertension, malaise, weight loss, fever, myalgia, and rapid deterioration of renal function. Renal biopsy revealed acute necrotizing vasculitis. Angiography showed small saccular aneurysmatic dilatations in the intrarenal branches of the right renal artery and the intrahepatic branches of the hepatic artery. cPAN was diagnosed and pulse methylprednisolone (MP), pulse cyclophosphamide (CYC) and subsequently oral prednisolone were given. Clinical and laboratory findings improved dramatically and remission was attained rapidly. The patient has remained in remission for the last 11 months. cPAN should be considered in patients who present with severe systemic symptoms and hypertension. Progressive renal insufficiency can occur during the acute course of cPAN due to renal vascular involvement without glomerulonephritis. Prompt and aggressive corticosteroid and cytotoxic therapy is essential to suppress disease activity and to maintain remission.
    No preview · Article · Mar 2001 · Pediatric Nephrology
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    S A Bakkaloğlu · M Ekim · G Koçak · S Atalay · N Tümer
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    ABSTRACT: Left ventricular hypertrophy is a major cause of morbidity and mortality among patients with chronic renal failure. Uremia-related risk factors play a fundamental role in its occurrence, thus better prognosis and prolonged survival can be attained by successful dialytic therapies. To investigate whether dialysis adequacy has a beneficial effect on cardiac structure and function in children receiving continuous ambulatory peritoneal dialysis (CAPD). Cross-sectional study in the Pediatric Peritoneal Dialysis Unit of a university hospital. Eighteen children, aged 13.3 +/- 2.8 years, being treated with CAPD, and 20 healthy age- and sex-matched control subjects were enrolled in this study. Echocardiographic evaluation was performed in all subjects. Dialysis adequacy indices [weekly urea (Kt/V) and creatinine clearance (TCCr)] were calculated in the dialysis group. Interventricular septal thickness, left ventricular (LV) posterior wall thickness, LV mass index (LVMI), and LV end systolic and diastolic dimensions were all found to be significantly higher in the CAPD group compared to the control subjects (p < 0.01). Ejection fraction and fractional shortening of the LV were not significantly different between the two groups. Mean Kt/V was 2.02 +/- 0.71 and mean TCCr was 58 +/- 33 L/wk/1.73 m2. There were significant negative correlations between dialysis adequacy indices and LV end systolic and diastolic dimensions (p < 0.05 and p < 0.001). Ejection fraction and fractional shortening were positively correlated with Kt/V (p < 0.01). Systolic and diastolic blood pressures were positively correlated with LVMI (r= 0.501 and r = 0.523). Significant inverse correlations between mean arterial pressure and both Kt/V and TCCr (r = -0.555 and r = -0.520) were detected. These data clearly document that cardiac structure and function are remarkably influenced by the uremic state and dialysis therapy in pediatric CAPD patients. The close relationships between echocardiographic findings and dialysis adequacy indices suggest that adequate dialysis has a beneficial effect on cardiac function via effective removal of toxic substances.
    Preview · Article · Jan 2001 · Peritoneal dialysis international: journal of the International Society for Peritoneal Dialysis
  • F Yalçinkaya · M Tekin · N Cakar · E Akar · N Akar · N Tümer
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    ABSTRACT: We compared the frequencies of seven MEFV mutations (M694V, M680I, V726A, M694I, K695R, R761H, E148Q) and the clinical findings in 20 Turkish FMF patients who had not developed amyloidosis by the age of 40 years in the absence of colchicine therapy, with those in 27 Turkish amyloidosis patients. No mutation frequency, including that of M694V, was different between the two groups. Family history of amyloidosis and parental consanguinity were noted to be higher in the amyloidosis group. The seven mutations do not appear to be sufficient to explain the development of amyloidosis in Turkish FMF patients. Other genetic factors may be important for this association.
    No preview · Article · Nov 2000 · QJM: monthly journal of the Association of Physicians
  • M Ekim · N Akar · E Tutar · S Kemahli · N Tümer

    No preview · Article · Sep 2000 · Pediatric Nephrology
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    SA Bakkaloglu · M Ekim · N Tümer · K Soylu

    Preview · Article · Sep 2000 · Peritoneal dialysis international: journal of the International Society for Peritoneal Dialysis
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    ABSTRACT: Familial Mediterranean fever (FMF) is an autosomal recessive disease. It is characterized by recurrent febrile episodes in association with peritonitis, pleuritis, and arthritis. Progressive systemic amyloidosis is the most important complication of FMF that inevitably leads to chronic renal failure. Recently, the gene for FMF, MEFV, has been cloned and four missense mutations have been described: M694V, M680I, V726A, and M694I. Initial studies have suggested that the presence of the M694V mutation carries a significant risk for the development of amyloidosis. In this study, we present seven families, in which at least two individuals have been diagnosed with FMF and at least one with amyloidosis. Among 18 individuals, in whom molecular testing was performed for the four aforementioned mutations, ten had amyloidosis. None of these ten individuals was found to be homozygous for the M694V mutation. In three families, there were two sibs with amyloidosis. None of the sib-pairs with amyloidosis was found to have the same genotype. There were two or more sibs with the same genotype in four families. Only one sib from each family developed amyloidosis in these families. These results provide evidence that FMF patients without the M694V mutation are also at risk for the development of amyloidosis. Particular mutations themselves do not appear to be sufficient to explain the occurrence of amyloidosis in all cases with FMF.
    No preview · Article · Jul 2000 · Clinical Genetics
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    ABSTRACT: Familial Mediterranean fever (FMF) is an autosomal recessive disease. It is characterized by recurrent febrile episodes in association with peritonitis, pleuritis, and arthritis. Progressive systemic amyloidosis is the most important complication of FMF that inevitably leads to chronic renal failure. Recently, the gene for FMF, MEFV, has been cloned and four missense mutations have been described: M694V, M680I, V726A, and M694I. Initial studies have suggested that the presence of the M694V mutation carries a significant risk for the development of amyloidosis. In this study, we present seven families, in which at least two individuals have been diagnosed with FMF and at least one with amyloidosis. Among 18 individuals, in whom molecular testing was performed for the four aforementioned mutations, ten had amyloidosis. None of these ten individuals was found to be homozygous for the M694V mutation. In three families, there were two sibs with amyloidosis. None of the sib-pairs with amyloidosis was found to have the same genotype. There were two or more sibs with the same genotype in four families. Only one sib from each family developed amyloidosis in these families. These results provide evidence that FMF patients without the M694V mutation are also at risk for the development of amyloidosis. Particular mutations themselves do not appear to be sufficient to explain the occurrence of amyloidosis in all cases with FMF.
    No preview · Article · May 2000 · Clinical Genetics
  • C Ensari · A Ensari · D Ayli · M Ekim · F Yalçinkaya · N Tümer

    No preview · Article · Apr 2000 · Nephron
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    S A Bakkaloğlu · M Ekim · N Tümer · G Deda · I Erden · T Erdem

    Preview · Article · Mar 2000 · Nephrology Dialysis Transplantation

  • No preview · Article · Mar 2000 · Nephron
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    ABSTRACT: Familial Mediterranean Fever (FMF) is an autosomal recessive disease characterized by recurrent self-limited attacks of fever accompanied by peritonitis, pleuritis and arthritis. Approximately 5% of individuals with FMF have been reported to have Henoch-Schönlein purpura (HSP) and about 1% have polyarteritis nodosa (PAN). Protracted febrile myalgia is another vasculitis-associated clinical entity among patients with FMF. Recently, the gene responsible for FMF, MEFV, has been cloned and four missense mutations (M680I, M694V, V726A and M694I) have been described. In this report, we present clinical and laboratory findings and mutation results of 23 children with FMF-associated vasculitis. HSP, PAN and protracted febrile attacks have been diagnosed in 11, 2 and 10 children, respectively. Mutation analysis shows that 3 children are homozygotes for the M694V mutation and 11 are compound heterozygotes for 2 of the studied mutations. M694V/V726A mutations were identified in 8, M694V/M694I in 2 and M680I/M694V in 1 of these children. In six children only one mutation was found and in three none of the studied mutations were identified. This study confirms that most children with FMF-associated vasculitis have identifiable mutations in the MEFV gene. Environmental and/or other genetic factors are possibly involved in the pathogenesis of vasculitis in FMF; elucidation of these mechanisms will help to understand pathogenesis of childhood vasculitides.
    No preview · Article · Mar 2000 · Acta Paediatrica

Publication Stats

831 Citations
150.68 Total Impact Points

Institutions

  • 1992-2002
    • Ankara University
      • • Department of Pediatric Nephrology
      • • Department of Pathology
      • • Department of Pediatrics
      Engüri, Ankara, Turkey
  • 2001
    • Gazi University
      • Department of Pediatric Nephrology
      Engüri, Ankara, Turkey
  • 2000
    • Virginia Commonwealth University
      • Department of Pediatrics
      Richmond, VA, United States