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    The Relationship Between Dietary Intakes and Total Kidney Volume in Patients with Autosomal Dominant Polycystic Kidney Disease Dietary Intake and Polycystic Kidney Volume
    (2022) Sevim, Yonca; Cebeci, Egemen; Ozkan, Ozlem Persil; Savas, Yildiray; Ozturk, Savas; Kiziltan, Gul
    Aim: There is a need to understand autosomal dominant polycystic kidney disease (ADPKD) patients' dietary habits since dietary interventions may have potential effects on ADPKD. In this study, we aimed to analyze the relationship between dietary nutrient intake and total kidney volume (TKV).Methods: This cross-sectional study was conducted on 54 ADPKD patients recruited from the Nephrology outpatient clinic between June and July 2014. TKV was determined by magnetic-resonance imaging and general characteristics, biochemical and urinary parameters were determined. The nutrient intakes of patients were calculated using the three-day dietary records obtained on three consecutive days.Results: The total kidney-volume median was found to be 1407 mL. Patients' total dietary energy and protein intakes were 25.8 +/- 9.4 kcal/kg, 0.9 +/- 0.3 g/kg, respectively. The percentage of carbohydrates, protein, and fat in energy was 49 +/- 7%, 14 +/- 3%, 37 +/- 7%, respectively. The mean intakes of thiamin, riboflavin, B6, calcium, magnesium, and zinc were sufficient, the mean dietary potassium intake was insufficient; and sodium intake was excessive in both sexes. In females, there was a negative but weak correlation between dietary vitamin C intake and TKV. In males, a negative but weak correlation was found between TKV and dietary intake of fiber, water, vitamin B6, vitamin K, magnesium, and iron. Conclusions: Dietary micronutrient intake may affect TKV according to sex.
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    Rapidly Progressive Renal Failure in AA Amyloidosis: A New Clinical and Histopathological Entity for an Old Disease
    (2020) Celebi, Zeynep Kendi; Kiremitci, Saba; Sadioglu, Rezzan Eren; Keven, Kenan; 0000-0003-3279-9796; ABB-9570-2020
    Objective: Secondary renal AA amyloidosis (RAAA) presents with proteinuria and/or as nephrotic syndrome and progresses to end stage renal disease (ESRD) insidiously. However, some patients with secondary amyloidosis show a more rapid renal disease progression than the usual course. In this study, we aimed to investigate the underlying cause of the rapidly progressive renal disease in the patients with secondary amyloidosis. Materials and Methods: Patients with kidney biopsy proven secondary RAAA were divided into 2 groups: the rapidly progressive group (estimated glomerular filtration rate >60 mL/min, who needed renal replacement therapy within one year of diagnosis) and the control group. Biopsy specimens were reevaluated for glomerular-vascular amyloid load, tubular atrophy, interstitial fibrosis, and interstitial inflammation. The biopsy characteristics and biochemical parameters were compared between the groups. Results: Histopathological examination showed global amyloid deposition, vascular pole involvement, peritubular capillary amyloid deposition, and severe interstitial inflammation associated with rapidly progressive disease. Estimated glomerular filtration rate was lower and proteinuria was higher in the rapidly progressive group than in the control group. Vascular pole amyloid deposition was found to be a predictor of ESRD in multivariate analysis. Conclusion: This study shows that higher amyloid deposition and severe inflammation revealed in in kidney biopsy of secondary RAAA cases can be risk factors for rapidly progressive renal failure.
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    Primary Leiomyosarcoma of the Kidney: Four Cases
    (2015) Akkaya, Bahar; Tuncer, Saniye Sevim; Akkaya, Hampar; Gurer, Elif Inanc; Baykara, Mehmet
    Renal sarcomas are rare tumors. They constitute only 1-2% of malignant renal tumors in adulthood. Leiomyosarcoma is the most common histological type of renal sarcoma (50-60%). Renal leiomyosarcomas are aggressive tumors arising from the renal capsule, renal vein, pelvic musculature or the renal parenchyma. Diagnosis is usually post operative and requires a thorough sampling of the tumor to rule out an epithelial component. We report 4 new cases of primary renal leiomyosarcomas. Typical morphologic pattern shows alternating fascicles of spindle cells with blunt-ended, non-tapering nuclei and eosinophilic cytoplasm. Nuclear pleomprphisim atypia, mitotic figures and necrosis are seen in different ratios. Immunohistochemically the tumor cells of leiomyosarcoma are positive for SMA, desmin, calponin and h-caldesmon and negative for CK, S-100, HMB-45 and CD117. These tumors are classified using the French Federation of Cancer Centers System. To make a diagnosis of a primary renal sarcoma the following criteria should be met: 1) The patient must not have a sarcoma elsewhere to rule out metastasis. 2) Gross must be compatible with origin in the kidney rather than involvement due to retroperitoneal sarcoma. 3) Sarcomatoid renal cell carcinoma must be excluded. They usually have poor prognosis. But small size (<5 cm), low histological grade, absence of lymph node metastases and radical operations are all associated with better prognosis. Despite radical nephrectomy the tumors can run an aggressive clinical course and early local and distant recurrences are common.