The value of 2019 EULAR/ACR classification criteria in predicting lupus nephritis in childhood-onset systemic lupus erythematosus.

BACKGROUND: We investigated the role of European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria for the prediction of LN among children with SLE. METHODS: The data of the patients with childhood-onset SLE diagnosed based on 2012 Systemic Lupus International Collaborating Clinics (SLICC) criteria were retrospectively evaluated. Based on 2019 EULAR/ACR classification criteria, the scoring was done at the time of renal biopsy. RESULTS: Fifty-two patients (12 with LN, 40 without LN) were included. The mean score was higher in patients with LN than those without (30.8±6.14, 19.8±7.76, respectively, p=0.000). The score value had indicative value for LN (area under curve [AUC]:0.863±0.055, cut-off value:22.5, p=0.000). Lymphocyte counts had a predictive value for LN (cut-off value:905/mm3, AUC:0.688±0.087, p=0.042). The score was positively associated with SLE disease activity index (SLEDAI) and activity index (r=0.879, p=0.000; r=0.811, p=0.001, respectively). There were significant negative associations between score value and GFR (r=-0.582, p=0.047). The patients with renal flare had higher the mean score than those of without renal flare (35±2/25.4±5.57, respectively, p=0.019). CONCLUSIONS: The EULAR/ACR criteria score could reflect the activity of disease and severity of nephritis in childhood-onset SLE. A point of 22.5 as score value might be an indicator for LN. During scoring, it should be taken into account that lymphopenia might guide the prediction of LN.

Amyloid A amyloidosis on medullary sponge kidney in a 28-year-old male with gout: A case report and literature review.

Amyloidosis is a large group of diseases that occur through misfolding of extracellular proteins that accumulate in tissues and organs. Gout is the most common inflammatory arthritis worldwide and starts with the crystallization of uric acid within the joints and soft tissues. Although gouty arthritis is accompanied by inflammation, AA amyloidosis is rarely seen in patients with gout. Here we present a case of AA amyloidosis on the medullary sponge kidney in a 28-year-old man with gout. Our case had been diagnosed with gout 3 years previously, and his older brother was also diagnosed with early-onset gout. As a result of the hyperuricemic nephropathy clinic and familial history, a whole gene sequence analysis was performed on the HPRT1 gene and UMOD gene, but no pathogenic changes were detected. Renal ultrasound revealed a bilateral medullary sponge kidney and amyloidosis was detected in the renal needle biopsy performed for the etiology of proteinuria. In our literature review, we found 16 cases in which gout was accompanied by AA amyloidosis. We present a 17th case and compare it with the other 16 cases.

Minimal Change Disease and Primary Sjogren Syndrome Concurrence: Case-Based review.

Primary Sjogren's syndrome is a chronic autoimmune disease with glandular and extraglandular features. Renal involvement is less frequent when compared with other systemic manifestations. Glomerulonephritis is a relatively rare manifestation of primary Sjogren's syndrome. Among all types of glomerular manifestations, minimal change disease is rarely identified, and there are only a few cases in the literature. Herein, we present a 53-year-old male patient who was diagnosed with primary Sjogren's syndrome and minimal change disease while searching for the etiopathogenesis of nephrotic syndrome. The patient had edema, dyspnea, hypertension, and 12 g/day proteinuria at admission. Serum albumin level was 1.82 g/dL, and renal function tests were within normal ranges. Renal biopsy findings were consistent with minimal change disease. At the same time, he was diagnosed with primary Sjogren's syndrome based on dry eyes demonstrated with Schirmer's test, positive antinuclear antibody, anti-SS-A, and anti-SS-B antibodies. Hydroxychloroquine with methylprednisolone 1 mg/kg (64 mg/day) was started, and methylprednisolone was slowly tapered. His proteinuria regressed to 79.2 mg/day, creatinine level was 0.83 mg/dL, and serum albumin level increased to 3.88 g/dL on the second week of the glucocorticoid treatment. In this case-based review, we present our case with 5 other reports of minimal change disease associated with primary Sjogren's syndrome. Our aim was to increase the awareness of this rare concurrence both among rheumatologists and nephrologists in light of the literature review.

Eosinophilic Vacuolated Tumor of Kidney: Clinical Perspective of a New Pathologic Emerging Entity.

The eosinophilic vacuolated tumor (EVT) of the kidney is a newly identified and pathological emerging entity. In this case report, EVT diagnosed due to a partial nephrectomy performed for a suspicious kidney mass in a 47-year-old patient is presented. A review of the literature and this case indicates that EVT, also called high-grade oncocytoma, does not show clinically aggressive behavior. However, in case of clinical suspicion, tumors with this oncocytic morphology should be treated with nephron-sparing treatment methods, considering that they may be hybrid malignancies.

Immunohistochemical evaluation of IL-1ß, IL-6, TNF-α and IL-17 cytokine expression in peripheral giant cell granuloma and peripheral ossifying fibroma of the jaws.

OBJECTIVE: To examine and compare the immunohistochemical expressions of IL-1ß, IL-6, IL-17 and TNF-α in peripheral giant cell granuloma (PGCG) and peripheral ossifying fibroma (POF). DESIGN: The study included 20 POF and 20 PGCG cases diagnosed at the Pathology Department of Eskisehir Osmangazi University Medical Faculty. Hematoxylin & Eosin-stained slides obtained from each biopsy specimen were re-evaluated, and IL-1ß, IL-6, IL-17 and TNF-α antibodies were investigated immunohistochemically. While staining in stromal cells was examined in POF cases, staining in both stromal spindle cells and multinucleated giant cells was evaluated in PGCG cases. An immunoreactivity score was established for each case by evaluating the staining percentage and intensity for each individual case. The significance level was set at 5% (p < 0.05). RESULTS: The level of IL-6 and TNF-α expressions in the multinucleated giant cells in PGCG lesions was found higher than that in stromal cells (p < 0.005 and p < 0.000, respectively). In PGCG lesions, there was no significant difference between giant cells and stromal cells in terms of IL-1ß and IL-17 expression levels. There was no significant difference between PGCG and POF lesions in terms of IL-1ß and IL-6 expression. TNF-α expression levels were significantly higher in spindle cells of PGCG lesions than that of POF lesions (p < 0.00). However, IL-17 expression levels were significantly lower in PGCG lesions than in POF lesions (p < 0.05). CONCLUSION: The study results showed that TNF-α expression was significantly higher in PGCG lesions and IL-17 expression in POF lesions. IL-1ß, IL-6, IL-17 and TNF-α are involved in the pathogenesis of both PGCG and POF lesions.

Prognostic significance of tumor budding in muscle invasive urothelial carcinomas of the urinary bladder.

OBJECTIVE: The aim of the present study was to analyze the prognostic significance of tumor budding in muscle-invasive urothelial carcinomas of the urinary bladder, and also to determine an optimal threshold value in evaluation. PATIENTS AND METHODS: The study included 108 patients diagnosed with muscleinvasive conventional urothelial carcinoma between 2010 and 2020. Tumor budding was evaluated on H&E-stained slides. The critical tumor budding number was determined with the "receiver operating characteristics (ROC)" curve. Cases with a tumor budding number of ≤6 were categorized as low, and cases with >6 as high tumor budding. RESULTS: The univariate Cox proportional hazards regression model for recurrence-free survival showed that lymphovascular invasion (P = 0.001), tumor budding (P = 0.012), pT stage (T4 vs. T2) (P = 0.005), and lymph node metastasis (P = 0.009) were significantly associated with recurrence-free survival. The multivariate Cox proportional hazards regression model utilizing backward stepwise (wald) method revealed that only LVI (P = 0.001) was independent risk factor for recurrence-free survival. The univariate Cox analysis showed that lymphovascular invasion (P = 0.001), tumor budding (P = 0.004), pT stage (T4 vs. T2) (P = 0.003), and lymph node metastasis (P = 0.001) were significantly associated with overall survival. The multivariate Cox analysis (backward stepwise (wald) method) revealed that tumor focality (P = 0.018), pT stage (T4 vs. T2) (P = 0.015), and lymphovascular invasion (P = 0.002) were independent factors for overall survival. CONCLUSIONS: Our findings suggested that the evaluation of tumor budding may be a useful parameter for predicting outcome in patients with muscle-invasive bladder cancer.

Evaluation and Prognostic Significance of Galectin-3 Expression in Oral Squamous Cell Carcinoma.

INTRODUCTION: In squamous cell carcinomas of the oral cavity, factors that can predict prognosis are being studied. However, new prognostic factors are needed to provide information on the biological properties of tumors. Galectin-3 is known to be involved in many biological events such as cell adhesion and differentiation, angiogenesis, apoptosis, tumorigenesis, and metastasis. In our study, the prognostic role of galectin-3 expression in squamous cell carcinomas of the oral cavity was investigated. MATERIALS AND METHODS: The study included 60 patients with a diagnosis of squamous cell carcinoma of the oral cavity. The demographic characteristics of the patients were compiled from the patient files. Galectin-3 expression was studied immunohistochemically. Cytoplasmic and nuclear expressions of galectin-3 were evaluated separately. In addition, the total expression score was calculated by multiplying the percentage and intensity scores for each cases. The critical expression score was determined according to the median value of the total scores. RESULTS: Among 60 cases, 28 tumors were located on the lip, 21 on the tongue, 4 on the floor of the mouth, 3 on the orobuccal mucosa, 3 on the retromolar trigone, and 1 on the gingivobuccal junction. There was no statistically significant relationship between prognosis and nuclear or cytoplasmic galectin-3 expression. Tumor grade and invasion pattern were found to be associated with the galectin-3 total expression score. CONCLUSION: Although galectin-3 total expression score was correlated with the tumor grade and invasion pattern, it is not associated with overall survival or recurrence rates. However, there is a need for a large series of cases in which methods are standardized in order to clarify the prognostic role of galectin-3 expression in oral squamous cell carcinomas.

Rare renal pathologic manifestation of antineutrophil cytoplasmic antibodies associated vasculitis as suppurative interstitial nephritis: A case report and review of the literature.

A 69-year old male patient attended our clinic with fatigue, fever, anuria, nephritic syndrome and severe renal failure. Kidney biopsy showed pauci-immune crescentic glomerulonephritis with an unusual association of suppurative interstitial nephritis. Though most patients with renal involvement linked to antineutrophil cytoplasmic antibodies associated vasculitis (AAV) have pauci-immune glomerulonephritis, only a few patients were identified to have atypical renal pathology. In most cases, mononuclear tubulointerstitial infiltrate may be a feature of AAV, suppurative interstitial nephritis is very rare. In the literature, we found only one case reported associated with suppurative interstitial nephritis without glomerulonephritis who later developed classic pauci-immune necrotizing glomerulonephritis. Here, we report a case diagnosed as AAV, presenting with pauci-immune crescentic glomerulonephritis and suppurative interstitial nephritis. It is not clear whether suppurative interstitial nephritis is a severe disease variant in AAV-associated renal disease. As described in the first case the lack of improvement in renal functions in spite of intense immunosuppressive treatment leads to the conclusion that suppurative interstitial nephritis is a marker of poor prognosis.

Renal clear cell sarcoma presenting as a spontaneous renal hematoma: A rare presentation.

Clear cell sarcoma of the kidney (CCSK) is an uncommon renal neoplasm of childhood. It represents between 2% and 9% of all pediatric renal tumors, and generally arises before the age of 5 years. It often mimics other pediatric renal tumors. Presently described is the case of a 7-year-old girl who presented with complaints of vomiting and abdominal pain. Abdominal ultrasonography revealed a right renal mass, and the patient developed a renal hematoma a few hours after admission. The patient underwent a nephroureterectomy with a provisional diagnosis of Wilms tumor; however, histopathological examination of a specimen revealed CCSK. CCSK is similar to Wilms tumor in terms of the typical age of appearance and clinical and histopathological features, but the treatment method and prognosis are different. Therefore, the differential diagnosis is very important. This case was presented to draw attention to a rare presentation of clear cell sarcoma. CCSK should be kept in mind in the differential diagnosis of a renal mass.

Efficacy of Platelet-Rich Plasma on Fat Grafts in the Repair of Tympanic Membrane Perforations: An Experimental Study.

OBJECTIVE: We investigated the use of autologous platelet-rich plasma (PRP) to improve the success rate of fat graft myringoplasty in perforated tympanic membranes of rats. MATERIALS AND METHODS: A total of 20 healthy Wistar albino female rats were divided into two groups. In Group 1, the left tympanic membranes were perforated and grafted with a fat graft that was harvested from the inguinal region. In Group 2, the left tympanic membranes were perforated, and a fat graft was also harvested from the inguinal region. Then, the fat was soaked in 0.5 mL PRP and grafted at the perforation. After the procedure, the rats were examined, and the graft situation was assessed at 3, 5, 7, 10, and 14 days. All of the rats were sacrificed 21 days after perforation, and a histopathological examination was made. RESULTS: We compared fat graft histopathological and otomicroscopic findings between the groups. While we did not observe graft rejections in Group 2, we saw 1 case of graft rejection in Group 1. In the histopathological examination, PRP prevents fat graft resorption by the terms of the adipocyte area, granulation tissue area, and vacuolization area. CONCLUSION: This study demonstrated the efficacy of fat grafts prepared with PRP on rat tympanic membranes. The fat graft with PRP did not statistically improve the success rate compared to the graft without PRP. Histopatologic findings of the study showed that PRP prevents fat graft resorption. Further studies are needed to further examine the advantages of the graft with PRP.

Nephrogenic adenoma of the urinary tract: A 6-year single center experience.

BACKGROUND: Nephrogenic adenoma is an uncommon benign lesion that occurs at several sites in urinary tract, from the renal pelvis to urethra, with the highest frequency in urinary bladder. Nephrogenic adenoma displays a broad spectrum of architectural and cytological features. Hence, recognition of its characteristic histopathological features is needed to distinguish this lesion from its mimickers. MATERIALS AND METHODS: A retrospective series of 21 cases of nephrogenic adenoma in 18 patients, which were diagnosed in our department between 2010 and 2016, were analyzed. All histological slides were reviewed by two pathologists and the diagnosis of each case was confirmed. Immunohistochemistry was performed for PAX-8 in all cases. CK7, PAX-2, PSA, p53, p63, GATA-3 and α-methylacyl-CoA racemase (AMACR) were applied in problematic cases. RESULTS: The most common location of the lesion was urinary bladder (14 patients) followed by renal pelvis (2 patients), ureter (1 patient) and urethra (1 patient). A history of urothelial carcinoma and repeated TUR procedures were observed in 12 patients. There were 2 pediatric patients aged 3 years. Both of them had undergone previous urosurgery because of megaureter in one and bladder exstrophy in the other. Other clinical antecedents included bladder diverticulum (1 patient), cystitis (1 patient) and nephrolithiasis (1 patient). Recurrence of lesion was seen in two patients (once in one case and twice in the other one). The median time to disease recurrence in these patients was 11 months (range, 2-20 months). Histologically, the lesions exhibited various morphological findings, with mixed (15 cases, 71.4%), pure tubular (3 cases, 14.3%), pure papillary (2 cases, 9.5%) and pure flat (1 case, 4.8%) growth patterns. Of the 15 cases with mixed patterns, 8 cases were tubulocystic and flat, 3 cases were tubular and flat, 2 cases were tubular, papillary and flat, 1 case was tubulocystic, papillary and flat, and 1 case was tubular and papillary. Flat pattern was observed in 15 cases (71.4%). It was seen in association with other patterns in 14 cases (mixed morphology) and purely in 1 case. Our findings suggested that the flat pattern is a frequent finding in nephrogenic adenomas. Notably one case in this series showed superficial extension into bladder muscularis propria. CONCLUSIONS: Histologically nephrogenic adenoma may simulate a variety of malignancies. Awareness of characteristic morphologic features of nephrogenic adenoma is needed to diagnose this lesion correctly.

Clinical and Hormonal Features of a Male Adolescent with Congenital Isolated Follicle-Stimulating Hormone Deficiency.

AIM: Our aim was to describe the clinical and genetic findings in an adolescent male with isolated follicle-stimulating hormone (FSH) deficiency and demonstrate the efficacy of recombinant human FSH (rhFSH) replacement in this case. METHODS: A 14.5-year-old adolescent male was referred with normal pubertal development and small testes. Serum testosterone, FSH, and luteinising hormone (LH) were measured at baseline and after gonadotropin-releasing hormone (GnRH) stimulation. Testicular biopsy was performed, and rhFSH replacement was administered for 6 months. The patient's FSHß gene was amplified and sequenced. RESULTS: Basal and GnRH-stimulated FSH levels were undetectable, in contrast with increased LH levels under both conditions. Histopathological investigation of a testicular biopsy specimen revealed a reduced number of Sertoli cells, the absence of germ cells, Leydig cell hyperplasia, and a thickened basement membrane in seminiferous tubules. The testicular size changed from 1 ml at baseline to 6 ml after 6 months of rhFSH replacement. Sequencing of the FSHß gene exon 3 revealed a new missense mutation (c.364T>C, resulting in p.Cys122Arg) in a homozygous state in the patient; both parents and a sister carried the same mutation in a heterozygous state. We also compared our case with all similar cases published previously. CONCLUSION: We herein described an adolescent male with isolated FSH deficiency due to a novel FSHß gene mutation associated with a prepubertal testes size and normal virilisation.

Ill-fitting dentures as primary presentation of mantle cell lymphoma: A case report and literature review of the primary mantle cell lymphomas of the hard palate.

Mantle cell lymphoma (MCL) is a subtype of B-cell non-Hodgkin's lymphoma seen predominantly in males. Common extra-nodal sites of involvement of MCL are Waldeyer's ring, gastrointestinal tract, bone marrow and peripheral blood. The extra-nodal palatal localization of MCL is quite uncommon. MCL is seen in predominantly older patients, therefore undiagnosed MCL patients are likely to have total prosthesis. In this study, a case of MCL, initially presenting as palatal swelling was reported with relevant literature review and the possible role of dental professionals in the diagnosis of this rare entity was discussed.

Unusual intraosseous capillary hemangioma of the mandible.

Intraosseous hemangioma is a benign vascular neoplasm, which is mostly seen in vertebrae, maxillofacial bones, and long bones. Intraosseous hemangioma is rarely seen on jaw bones compared to other skeletal bones and usually occurs in the cavernous form. Capillary intraosseous hemangioma of jaws is an uncommon form of intraosseous hemangioma and has not been thoroughly described so far. In this study, a case of capillary intraosseous hemangioma of the mandible was presented with relevant literature review.

Do Leukaemia Inhibitory Factor and Vascular Endothelial Growth Factor Have Any Roles in Intrauterine Device Mechanism of Action? An Experimental Rat Study.

OBJECTIVE: To assess leukaemia inhibitory factor (LIF) and vascular endothelial growth factor (VEGF) expression as markers of intrauterine device (IUD) efficacy in a rat model. METHODS: Twenty nulliparous female Wistar rats were divided into two groups with 10 animals per group: group I (IUD) and group II (control group, no IUD). In group I, a 2-cm 3-0 silk suture was placed into one horn of the rat bicornuate uterus. On day 20 (after IUD insertion) rats were sacrificed and their uteri removed. The number of vessels and the distribution of LIF and VEGF were compared among the uterine horns. RESULTS: There were no significant differences in LIF and VEGF expression in the groups and all horns (p > 0.05). The number of vessels was higher in the IUD+ horn than in the IUD- horn of group I and in the horn of group II (p < 0.05). There was no significant difference in the number of vessels between the IUD- horns of groups I and II (p > 0.05). CONCLUSION: LIF and VEGF expression did not correlate with IUD efficacy in a rat model. An IUD may increase the number of vessels in the uterine horn independent of VEGF expression. © 2015 S. Karger AG, Basel.

Renal hemosiderosis and rapidly progressive glomerulonephritis associated with primary hemochromatosis.

Hereditary hemochromatosis leads to the accumulation of iron in many organs including the liver, spleen and heart and results in injury and dysfunction of these organs. On the other hand, iron accumulation and functional impairment in kidney is extremely rare. We report a 61-year-old male patient with hereditary hemochromatosis, in whom the renal function was deteriorated rapidly. Renal biopsy revealed crescentic glomeruli and hemosiderin accumulation in tubular epithelial cells.

Prognostic impact of intratumoral C-reactive protein expression in patients with clear cell renal cell carcinoma.

BACKGROUND: Elevated serum C-reactive protein (CRP) level has been demonstrated to predict poorer survival of both localized and metastatic renal cell carcinoma. However, the prognostic value of intratumoral CRP expression has not been sufficiently studied. PATIENTS AND METHODS: In the present study, the expression of CRP was evaluated with immunohistochemical analysis in 127 patients who had undergone curative surgery for clear cell renal cell carcinoma. CRP staining was scored using the immunoreactivity score. An immunoreactivity score of 4 (median value) or lower was considered negative, a score higher than 4 was considered positive CRP expression. RESULTS: Univariate analysis revealed that Fuhrman grade, necrosis, vascular invasion, TNM stage and CRP expression were associated with tumor-specific survival. Multivariate analysis using the Cox proportional hazards regression method revealed only TNM stage as an independent predictor of tumor-specific survival (p = 0.001). A trend towards significance (p = 0.066) was observed with CRP expression, but it did not reach significance. Patients with a positive CRP score were 3.46 times more likely to die than patients with a negative CRP score. CONCLUSION: Our results suggest that the assessment of intratumoral CRP expression may be a useful tool for predicting the prognosis in patients with clear cell renal cell carcinoma.

Prognostic value of osteopontin in patients treated with primary radiotherapy for head and neck cancer.

BACKGROUND: The prognostic value of tumor osteopontin (OPN) in patients with squamous-cell head and neck cancer (SCHNC) was investigated. MATERIALS AND METHODS: OPN expression was assessed by immunohistochemical methods in 50 patients, who were treated with primary radiotherapy (RT) for locally advanced SCHNC. The effects of OPN on clinical parameters, local-regional control after RT and metastasis-free survival, was assessed. RESULTS: The rate of OPN expression in tumor tissue was 76%. OPN positive cases had lower Hb levels (p=0.088). Mean time to local recurrence was 53.8 months (SE 3.9) in OPN-negative cases and 39.1 months (SE 4.7) in OPN-positive cases (p=0.047). OPN increased the risk of local recurrence 5.9 times (p=0.085). It had no effect on metastasis-free (p=0.116) or overall survival (p=0.123). OPN was positive in 12 of 19 cases that developed grade 3-4 acute radiation dermatitis (p=0.096). CONCLUSIONS: OPN expression is associated with an increase in local recurrence in patients who were treated with primary RT for locally advanced SCHNC.