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Background/Aims: The aim of this study is to investigate the utility of clinical [age, gender, mean arterial pressure (MAP)] and laboratory parameters [eGFR, hemoglobin (Hgb), serum levels of creatinine, uric acid, albumin, proteinuria, hematuria] and also histopathological lesions (Oxford classification parameters, crescents, intensity and pattern of staining for C3, C1Q, IgA, IgG, IgM) as progression markers in patients with IgA Nephropathy (IgAN). Methods: A total of 111 IgAN patients with a follow-up period >1 year or who reached kidney failure [GFR category G5 chronic kidney disease (CKD)] <1 year were investigated. Primary endpoint was the development of kidney failure or eGFR decline ≥50% from the baseline. Kaplan-Meier and Cox proportional hazards analyses were performed. Results: Mean follow-up period was 33±29 months. Thirty-seven (33.3%) patients progressed to kidney failure and 4 (3.6%) patients developed eGFR decline ≥50% from the baseline after a median of 23 and 65 months, respectively. In multivariate Cox regression analysis, baseline levels of Hgb (HR:0.782, 95% CI 0.559-0.973, p=0.037), serum uric acid (HR:1.293, 95% CI 1.023-1.621, p=0.046), eGFR (HR:0.966, 95% CI 0.947-0.984, p=0.004) and intensity of C3 staining (HR:1.550, 95% CI 1.198-1.976, p=0.049) predicted primary endpoint. Serum uric acid level was associated independently with T score (β=0.303, p=0.005) in patients with eGFR>30 ml/min/m2. Conclusions: Hyperuricemia and the deposition of C3 are independent risk factors for IgAN progression. [ABSTRACT FROM AUTHOR]

Background Data on use of interleukin (IL)-1 blockers in kidney transplant recipients (KTRs) with familial Mediterranean fever (FMF) are very limited. We aimed to evaluate the efficacy and safety of anakinra and canakinumab in the transplantation setting. Methods In this retrospective cohort study, we included KTRs who suffered from AA amyloidosis caused by FMF and treated with anakinra or canakinumab (study group, n  = 36). Using propensity score matching, we selected 36 patients without FMF or amyloidosis from our database of 696 KTRs as the control group. Primary outcomes were patient and graft survival. Biopsy-confirmed graft rejection, changes in estimated glomerular filtration rate (eGFR), high-sensitivity CRP (hsCRP), erythrocyte sedimentation rate (ESR), proteinuria and number of monthly attacks were secondary outcomes. Results All KTRs with FMF began IL-1 blocker therapy with anakinra and nine (25%) were switched to canakinumab. Overall death was more frequent in the study group (19.4% vs 0%) (P  = .005); however, overall graft loss was comparable between study (27.8%) and control groups (36.1%) (P  = .448). Five- and 10-year graft survival rates were significantly higher in the study group (94.4% and 83.3%, respectively) than in the control group (77.8% and 63.9%, respectively) (P  = .014 and P  < .001, respectively). Rejections were numerically lower in study group (8.3% vs 25%), but it did not reach to statistical significance (P  = .058). When compared with the pre-treatment period, with IL-1 blockers, the number of attacks per month (P  < .001), and eGFR (P  = .004), hsCRP (P  < .001) and ESR (P  = .026) levels were lower throughout the follow-up, whereas proteinuria levels were not. Conclusions Anakinra and canakinumab are effective in KTRs suffering from FMF; however, the mortality rate may be of concern. [ABSTRACT FROM AUTHOR]

Background/Aims: We aimed to investigate the effects of glomerular IgM and C3 deposition on outcomes of adult patients with primary focal segmental glomerulosclerosis (FSGS). Methods: In this retrospective analysis, 86 consecutive adult patients with biopsy-proven primary FSGS were stratified into 3 groups according to their histopathological features: IgM– C3–, IgM+ C3–, and IgM+ C3+. Primary outcome was defined as at least a 50% reduction in baseline estimated glomerular filtration rate (eGFR) or development of kidney failure, while complete or partial remission rates were secondary outcomes. Results: Glomerular IgM deposits were found in 44 (51.1%) patients, 22 (25.5%) of which presented with accompanying C3 deposition. Patients in IgM+ C3+ group had higher level of proteinuria (5.6 g/24 h [3.77–8.5], p = 0.073), higher percentage of segmental glomerulosclerosis (20% [12.3–27.2], p = 0.001), and lower levels of eGFR (69 ± 37.2 mL/min/1.73 m2, p = 0.029) and serum albumin (2.71 ± 0.85 g/dL, p = 0.045) at the time of diagnosis. Despite 86.3% of patients in IgM+ C3+ group (19/22) received immunosuppressive treatment, the primary outcome was more common in patients in the IgM+ C3+ group compared with patients in IgM+ C3– and IgM– C3– groups (11 [50%] vs. 2 [9%] and 11 [26.1%] respectively [p = 0.010]). Complete or partial remission rates were lower in patients in the IgM+ C3+ group (5/22, 22.7%), as well (p = 0.043). Multivariate Cox regression analysis revealed that IgM and C3 co-deposition was an independent risk factor associated with primary outcome (hazard ratio 3.355, 95% CI 1.349–8.344, p = 0.009). Conclusions: Glomerular IgM and C3 co-deposition is a predictor of unfavorable renal outcomes in adult patients with primary FSGS. [ABSTRACT FROM AUTHOR]

Background: The family of heat shock proteins (HSPs) contains several members that regulate the cell response to any hazardous factors to protect protein structure. Theaimof the studywasto determinewhetherserumlevels of HSPsincrease in children with IgA nephropathy/Immunoglobulin A - associated vasculitis nephritis (IgAN/IgAVN) and to assess their relationship with the prognostic factors. Patients and Methods: Twenty - nine patients with IgAN/IgAVN, 41 patients with idiopathic nephrotic syndrome (INS) as a patient control group, and 24 healthy controls were enrolled in the study. Renal function was normal in all participants. Serum levels of HSP27, HSP40, HSP60, HSP70, and HSP90 were measured using an enzyme - linked immunosorbent assay (ELISA). Results: Median serum levels of HSP27, HSP40, HSP60, HSP70 and HSP90 were significantly higher in the IgAN/IgAVN group than in the INS group and controls (P < 0.05). Among these HSPs, only HSP60 had no overlap between patients and controls. Serum HSP40, HSP60, HSP70 levels were significantly higher in the patients with INS than in controls (P< 0.05), whereas HSP27 and HSP90 were not different. HSP27 was significantly higher in patients with proteinuria in the IgAN/IgAVN group than in patients with no proteinuria (P = 0.045). None of these HSPs was related to the presence of crescent and severity of proteinuria in the IgAN/IgAVN group. Conclusions: Our results demonstrated that serum levels of HSP27, 40, 60, 70, 90 increased in the patients with IgAN/IgAVN considering HSPs might have a role in pathogenesis of the disease. Among these HSPs, HSP60 was a prominent marker because it had no overlap between patients and controls. Explaining the role of these HSPs in IgAN/IgAVN may pave the way for future treatment. [ABSTRACT FROM AUTHOR]

Background: C3 glomerulopathy (C3GP) is a recently identified and described disease that has a high risk of progressing into end-stage renal disease. We aimed to evaluate the effects of various immunosuppressive regimens on C3GP progression because there are conflicting data on the treatment modalities.Methods: In this retrospective study of 66 patients with C3GP, 27 patients received mycophenolate mofetil (MMF)-based treatment, 23 received non-MMF-based treatment (prednisolone or cyclophosphamide), and 16 received conservative care. The study groups were compared with each other with specific focus on primary outcomes defined as (1) kidney failure and (2) estimated glomerular filtration rate (eGFR) decline ≥50% from the baseline value.Results: Overall, 17 (25.8%) patients reached the primary outcome after a median period of 28 months. The number of patients who reached the primary outcome were similar among the study groups (MMF-based: 8/27 [29.6%], non-MMF-based: 4/23 [17.4%], and conservative care: 5/16 [31.3%], p = 0.520). In the Cox regression analysis, age (HR 0.912, p = 0.006), eGFR (HR 0.945, p = 0.001), and proteinuria levels (HR 1.418, p = 0.015) at the time of biopsy, percentage of crescentic (HR 1.035, p = 0.001) and sclerotic glomeruli (HR 1.041, p = 0.006), severity of interstitial fibrosis (HR 1.981, p = 0.048), as well as no remission of proteinuria (HR 2.418, p = 0.002) predicted the primary outcome.Conclusion: Although patients receiving immunosuppressive treatments had higher proteinuria and lower serum albumin at baseline, there were no differences between these patients and those receiving conservative care alone in proteinuria remission or in the decline of renal function. Younger age, higher proteinuria, lower eGFR, and the presence of crescentic and sclerotic glomeruli, severity of interstitial fibrosis, and no remission of proteinuria predicted the progression of kidney disease. [ABSTRACT FROM AUTHOR]

The presence of occlusion/near-occlusion of glomerular capillaries was recently added to the existing definition of glomerulitis (g). We retrospectively re-evaluated 135 renal allograft biopsies regarding g to ensure no antibody-damaged grafts were missed. Previous and revised g scores (pg and rg, respectively) were compared for clinicopathologic correlations. The g score did not change in 100 (74.1%) biopsies. Thirty-five (25.9%) biopsies were changed to a lower score. Sensitivity and specificity of pg and rg for the presence of donor-specific antibodies ( DSA) were 76% vs. 58% and 70% vs. 79%, respectively. Pg score indicated graft loss with 65% sensitivity and 63% specificity, whereas rg showed 46% sensitivity and 71% specificity. Area under the curve ( AUC) values in ROC analysis for DSA and graft loss were as follows: pg, 0.773; rg, 0.693; and pg, 0.635; rg, 0.577, respectively. A comparison of the two AUC values revealed a significant difference between pg and rg only for DSA ( P = 0.0076). Pg and post-transplant time of biopsy independently predicted graft loss, whereas rg did not. In conclusion, revised g scores showed lesser sensitivity but higher specificity for DSA and graft loss. Recent definition of g missed antibody-mediated rejection in few cases, and it was not an independent predictor for graft loss. [ABSTRACT FROM AUTHOR]

Objective: We aimed to define the histopathologic features and proliferative rate of congenital mesoblastic nephroma (CMN) as a risk factor for recurrence.Methods: Fourteen cases of CMN among 138 registered pediatric renal tumors were retrospectively reviewed. The prognostic impact for mitotic rate and Ki67 index was investigated.Results: There were four (28.6%) classic, six (42.9%) cellular, and four (28.6%) mixed type CMNs, with average Ki-67 counts of 16.75% in the classic CMN, and 53.2% in the tumors with cellular components (both mixed and cellular CMNs). Twelve patients (85.7%) were aged less than six months. Tumors with cellular component showed significantly larger tumor diameter and higher Ki-67 index (p = 0.015andp = 0.016, respectively). The patient with cellular CMN, whose tumor showed the highest mitotic rate (4.9/HPF), but not the highest Ki67 index (57.4%), died of recurrent disease with distant metastasis.Conclusion: Proliferative markers-mitotic count and Ki67 index-have limited value to predict recurrence or metastasis in CMNs with a cellular component. [ABSTRACT FROM PUBLISHER]

We present a case of a teratoma with somatic type malignancy (TSM) in the form of papillary renal cell carcinoma (pRCC) within supraclavicular and retroperitoneal lymph node metastases of a testicular pure teratoma. Resection of both masses revealed a teratoma without any other germ cell tumor component. A papillary carcinoma component was also detected intermingled with the teratomatous elements. The carcinoma cells displayed eosinophilic cytoplasm and prominent nucleoli. Groups of foamy histiocytes in the fibrovascular cores was a striking finding that brought pRCC to mind. Immunoreactivity for CK7, PAX8, AMACR, CD10, napsin, and vimentin along with morphologic findings confirmed renal cell differentiation. No radiological evidence of a primary renal cell carcinoma was found in the kidney. Consequently, pRCC arising in a teratoma was diagnosed. TSM is described as teratoma with a malignant component that is typically encountered in other organs and tissues. TSM in the form of pRCC is an extremely rare entity. Our case is the second example of a testicular germ cell tumor metastasis with a somatic malignancy in the form of pRCC. In conclusion, carcinomas of renal cell differentiation should be kept in mind as a rare form of TSM, especially in metastatic germ cell tumors. [ABSTRACT FROM AUTHOR]

Background/Aims: The aim of this study is to investigate the utility of clinical [age, gender, mean arterial pressure (MAP)] and laboratory parameters [eGFR, hemoglobin (Hgb), serum levels of creatinine, uric acid, albumin, proteinuria, hematuria] and also histopathological lesions (Oxford classification parameters, crescents, intensity and pattern of staining for C3, C1Q, IgA, IgG, IgM) as progression markers in patients with IgA Nephropathy (IgAN). Methods: A total of 111 IgAN patients with a follow-up period >1 year or who reached kidney failure [GFR category G5 chronic kidney disease (CKD)] <1 year were investigated. Primary endpoint was the development of kidney failure or eGFR decline ≥50% from the baseline. Kaplan-Meier and Cox proportional hazards analyses were performed. Results: Mean followup period was 33±29 months. Thirty-seven (33.3%) patients progressed to kidney failure and 4 (3.6%) patients developed eGFR decline ≥50% from the baseline after a median of 23 and 65 months, respectively. In multivariate Cox regression analysis, baseline levels of Hgb (HR:0.782, 95% CI 0.559-0.973, p=0.037), serum uric acid (HR:1.293, 95% CI 1.023- 1.621, p=0.046), eGFR (HR:0.966, 95% CI 0.947-0.984, p=0.004) and intensity of C3 staining (HR:1.550, 95% CI 1.198-1.976, p=0.049) predicted primary endpoint. Serum uric acid level was associated independently with T score (β=0.303, p=0.005) in patients with eGFR>30 ml/ min/m2. Conclusions: Hyperuricemia and the deposition of C3 are independent risk factors for IgAN progression. [ABSTRACT FROM AUTHOR]

We report a case that developed nephrotic syndrome during hospitalization for severe ovarian hyperstimulation syndrome without history of acute renal failure. During hospitalization, she developed persistent ascites and respiratory distress. The 24 hours urine protein analysis revealed significant proteinuria and renal biopsy showed global and segmental sclerosis in glomeruli, mesangial arteritis, proliferations in visceral epithelial cells (IgA nephropathy). To the best of our knowledge, such a complication will be presented for the first time in the literature. [ABSTRACT FROM AUTHOR]

Background. Membranous nephropathy (MN) is a common cause of nephrotic syndrome. In most cases it is idiopathic, while it may also be secondary to many diseases. In this study, prevalence of H. pylori infection and the effects of H. pylori eradication on proteinuria levels were investigated. Methods. Thirty five patients with MN (19 male), 12 patients with IgA nephropathy (4 male) and 12 patients with focal segmental glomerulosclerosis (FSGS) (8 male) were studied. The presence of H. pylori antigen was investigated in renal tissues obtained by biopsy, and the effects of H. pylori eradication on proteinuria levels were investigated. Results. Immunohistochemistry with H. pylori antigen revealed no positive staining in the glomeruli of all patients. 19 patients (54%) with MN, 10 (83%) with IgA nephropathy and 4 (33%) with FSGS were positive for H. pylori stool antigen test (P = 0.045). Patients with H. pylori infection were administered eradication therapy (lansoprazole, 30 mg twice daily, plus amoxicillin, 0.75 g twice daily, plus clarithromycin, 250 mg twice daily, for 14 days). Before the eradication therapy the mean proteinuria of patients with MN, IgA nephropathy and FSGS were 2.42 ± 3.24 g/day, 2.12 ± 1.63 g/day and 1.80 ± 1.32 g/day, respectively. Three months after eradication, baseline proteinuria levels of patients with MN significantly decreased to 1.26 ± 1.73 g/day (P = 0.031). In all three groups there were no significant differences with regard to serum creatinine, albumin and C-reactive protein levels before and after eradication therapy. Conclusions. The eradication of H. pylori infection may be effective to reduce proteinuria in patients with MN, while spontaneous remission of MN could not be excluded in this patient cohort. This trial is registered with NCT00983034. [ABSTRACT FROM AUTHOR]

Abstract: INTRODUCTION: Achieving hemostasis in laparoscopic nephron sparing surgery (LNSS), a technically demanding procedure, is challenging. Absorbable hemostatic agents, such as oxidized regenerated cellulose (ORC) are frequently used for hemostasis in laparoscopic nephron sparing surgery. Retention of this material is a very rare situation. PRESENTATION OF THE CASE: We are presenting a case of ORC granuloma after nephron sparing surgery for renal cell carcinoma (RCC) mimicking recurrent renal mass. A 50-year-old woman diagnosed with upper pole renal mass underwent laparoscopic nephron sparing surgery. Oxidized regenerated cellulose was used to achieve hemostasis for an oozing from the perirenal tissue. Resection confirmed RCC. Imaging at 6th month follow-up revealed a lesion with contrast enhancement at the location of the adrenal gland. Positron emission tomography suggested inflammation or metastasis. Histopathological evaluation of the mass revealed foreign body granuloma. DISCUSSION: Laparoscopic nephron sparing surgery is becoming a standardized treatment of select renal tumors. Hemostatic agents, such as ORC, are frequently used to minimize complications in LNSS. In case of ORC application, which is completely absorbed in 8 weeks, to or to a location with close proximity to the primary resection site, surgical granuloma formation should be considered. However its use should not be discouraged and biopsy may be considered for definitive diagnosis. CONCLUSION: Oxidized regenerated cellulose granuloma may mimic different entities such as including invasive carcinoma. Thus it is of utmost importance, if ORC was used during LNSS, the location and use of this material should be noted precisely. [Copyright &y& Elsevier]

Summary: We describe here a patient with a metastatic malignant melanoma displaying crescentic lesions in the glomeruli. A 64-year-old woman was referred to our hospital because of a sudden increase of serum creatinine. Clinical diagnosis suggested rapidly progressive glomerulonephritis. Renal biopsy revealed diffuse crescentic lesions containing metastatic melanoma cells. These cells were also seen in the glomerular capillary lumina and tubules, and a few scattered tumor cells were also present in the interstitium. Glomerular metastasis is a very rare entity. To the best of our knowledge, this patient is the first to be described in the English language literature showing intraglomerular metastasis of malignant melanoma as demonstrated by needle biopsy and is only the second patient with intraglomerular metastasis presenting with acute renal failure. [ABSTRACT FROM AUTHOR]

Primary renal mucinous cystadenocarcinoma is a very rare lesion of kidney which originates from the metaplasia of the renal pelvic uroepithelium. Only one case with primary mucinous cystadenocarcinoma has been reported in the English literature. We report second case of mucinous cystadenocarcinoma which was radiologically classified as type-IIF Bosniak cyst in peripheral localization. We aimed to present this extreme and unusual entity with its radiological, surgical, and pathologic aspects under the light of literature. [ABSTRACT FROM AUTHOR]

Background: We aimed to review our experience with childhood lupus nephritis (LN) in respect to the analysis of the clinical and histopathological presentation of LN and prognostic factors affecting the kidney and patient outcomes. Method: Forty-three children (39 girls, 4 boys) with biopsy-proven LN were included in the study. The mean age of the children was 12.0 ± 2.8 years. Based on the renal histopathology and clinical presentation, patients were treated with oral prednisone, intravenous pulses of methylprednisolone or intravenous cyclophosphamide. The final clinical status was classified as follows: (1) renal and extrarenal remission; (2) clinically active renal disease, or (3) adverse outcome, i.e., end-stage renal failure (ESRF) or death. Results: The mean duration of follow-up was 7.2 ± 2.8 years (1 month to 14.2 years). All 43 children had hematuria and 53.5% had proteinuria at admission. Fourteen children were in nephrotic status at the onset of disease. Class IV (diffuse proliferative) nephritis was observed in 29 patients as the most frequent histopathology (67.4%). The patients with class IV nephritis had a tendency to develop nephrotic syndrome, heavy proteinuria, increased Cr levels and persistent hypertension at initial evaluation. Thirty-two of 43 children (74.4%) were in renal remission at the last visit. Five-year kidney and patient survival rates from the time of diagnosis to the endpoints of ESRF or death were 83.7 and 90.7% respectively in the whole group while it was 75.9 and 86.2% respectively in the class IV group. Adverse outcome was significantly associated with the persistent hypertension, anemia, high serum Cr level, heavy proteinuria, nephrotic syndrome and class IV nephritis at presentation. Conclusion: We can conclude that the prognosis of LN in children is primarily dependent on the histopathological lesions. Severity of the clinical renal disease at admission and presence of persistent hypertension are the main poor prognostic factors rather than age, gender, low C3 and C4 levels, ANA positivity and the treatment modalities in Turkish children.Copyright © 2001 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Karyomegalic interstitial nephritis (KIN) is a rare entity associated with biallelic FAN1 (FANCD2/FANCI‐Associated Nuclease 1) gene variants. In FAN1‐related KIN, abnormal liver function tests and respiratory involvement are common, in addition to chronic kidney disease. Karyomegalic changes have also been reported in many other organs in patients with FAN1‐related KIN in various studies. We report the case of a 35‐year‐old male with chronic kidney disease of unknown aetiology, concurrent recurrent upper and lower respiratory tract infections, and elevated liver function test results with unidentified aetiology. The patient's family history was remarkable for consanguineous parent marriage and history of kidney transplantation in his aunt. A kidney biopsy was performed, which was consistent with KIN. Clinical exome sequencing revealed a homozygous nonsense variant NM_014967.5 (FAN1): c. 2260C > T (p.Arg754Ter). According to the American College of Medical Genetics (ACMG) criteria, this variant is pathogenic and, to the best of our knowledge, has not been previously reported, homozygously. Therefore, the histopathological and clinical diagnoses of KIN were confirmed by genetic studies in our patient. This case report expands the genetic spectrum of FAN1‐related KIN, and briefly reviews the current literature data. [ABSTRACT FROM AUTHOR]

Presents a case study of renal cell carcinoma found in an old male with a right renal mass and a caval thrombus after an episode of hematuria. Clinical history of the patient; Results of physical and laboratory examinations performed on the patient; Information on the disease.

The article describes the case of a 45-year-old man with microscopic polyangiitis (MP). The patient experienced symptoms of fever, weight loss and elevated serum C-reactive protein (CRP) level. The autoimmune disease was confirmed by 18F-fluorodeoxyglucose (FDG) positron emission tomography (PET) and computed tomography (CT). The case showed the function of FDG PET to localize sites of active inflammation due to vasculitis.

The article presents a case study of a 61-year-old woman with intravascular large B-cell lymphoma (IVLBCL) presented with fever and weakness.

Celiac disease (CD) may coexist with another autoimmune disorders. We presented a woman with coeliac disease coexistent with autoimmune hypothyroidism, nephrosclerosis and severe hyperparathyroidism. Forty years-old hypertensive woman, who had diarrhea without tenesmus, blood and mucus for 20 years, was admitted to hospital. Parathyroid hormone level was very high. Creatinine clearance was 44.5 ml/minute, and protein was 596mg/day. Renal biopsy revealed immunofluorescence negative chronic changes mimicking nephrosclerosis. The clinical and biochemical presentation of CD maybe complicated or differed from in renal failure patient. Especially management of serum phosphorus and PTH levels may have great importance. [ABSTRACT FROM AUTHOR]