A case of Sjögren syndrome and anti-neutrophil cytoplasmic antibody-associated vasculitis.

Sjögren's syndrome (SS) is a rare disease with the highest reported prevalence of 0.01-0.09%. Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is another rare auto-immune disease (prevalence of 0.0009-0.01%). The co-occurrence of these two separate clinical entities in one patient might rarely be encountered as an overlap syndrome. Here, we present the case of a 60-year-old female patient who had complaints of headache, nausea, weakness, gritty sensation in her eyes, and dry mouth [unstimulated saliva production of 0.033 mL/minute (normal; >0.1 mL/minute)] with a blood pressure of 190/110 mmHg, hypertensive retinopathy, proteinuric kidney disease, positivity of myeloperoxidase anti-neutrophil cytoplasmic antibodies, anti-Ro-52, anti-Ro, and anti-La antibodies. Pauci-immune crescentic proliferative glomerulonephritis was found in a kidney biopsy and successfully treated with cyclophosphamide and methylprednisolone. The co-occurrence of these diseases was first reported in 1992 by Böttinger et al. Since then, nearly 37 cases of SS and AAV have been reported. By reporting this case of primary SS and AAV, we emphasize the importance of auto-antibody tests in searching for the etiology of patients with proteinuria.

Thyroid nodules classified as atypia or follicular lesions of undetermined significance deserve further research: Analysis of 305 surgically confirmed nodules.

OBJECTIVE: The objective of the present study was to determine the malignancy risk for nodules categorised as atypia or follicular lesions of undetermined significance atypia of undetermined significance/follicular lesion of undetermined significance (AUS/FLUS) and to investigate the predictors of malignancy. METHODS: All nodules diagnosed as AUS/FLUS on fine needle aspiration (FNAs) performed between January 2011 and December 2015 were retrospectively reviewed. Clinical data, ultrasonographic features, follow-up data and the final pathological results were recorded. After further exclusion, only nodules that underwent surgical excision were included in the final analysis. The malignancy rate and the range of malignancy rates were calculated. Clinical and ultrasound features were examined to determine the predictors of malignancy. RESULTS: During the study period, FNA was performed on 9938 nodules, and 1019 (10.2%) nodules were diagnosed as AUS/FLUS. After further exclusion, 976 nodules were evaluated. After the initial diagnosis of AUS/FLUS, 139 (14.2%) patients underwent surgery, 518 (53.1%) had repeated FNAs. A total of 305 (31%) had undergone surgical excision at different time points. For surgically confirmed nodules, the malignancy rate after the initial FNA was 34.5% (the lower and upper thresholds for the malignancy rate were 19.3% and 66.3%, respectively), and 37.9% after the repeated FNA. No ultrasound feature was determined as a predictor, whereas age (>55 years) was a predictor for malignancy. CONCLUSIONS: The overall malignancy rate for nodules diagnosed as AUS/FLUS and the malignancy rate for nodules that underwent repeated FNA after AUS/FLUS were higher than the expected malignancy rates of the National Cancer Institute. It is, therefore, suggested that the current recommendations should be reconsidered.

Perspective on rhabdomyolysis-induced acute kidney injury and new treatment options.

AIM: The purpose of this study was to assess the role of caspase-dependent apoptosis, caspase 1, calpain 1, inducible nitric oxide synthase (iNOS) and endothelial nitric oxide synthase (eNOS) and the protective effect of grape seed proanthocyanidin extract (GSPE) in the development of rhabdomyolysis-induced acute kidney injury (AKI). MATERIALS AND METHODS: Twenty-one rats were divided into 3 groups - control, rhabdomyolysis and rhabdomyolysis + GSPE. Rhabdomyolysis was induced in the rhabdomyolysis and rhabdomyolysis + GSPE groups with the injection into both hind limbs of 10 ml/kg hypertonic (50%) glycerol following 24-hour dehydration on the 6th day. The rhabdomyolysis + GSPE group was given GSPE at 100 mg/kg by gavage for 7 days. The experiment was concluded 48 h after glycerol injection. Blood specimens were collected, and kidney tissues were extracted for histopathological examination. RESULTS: We identified an increase in blood urea nitrogen, creatinine, histopathological score, iNOS, caspase 3, caspase 1 and calpain 1 expression in the rhabdomyolysis group compared to the controls and a decrease in eNOS expression. In the rhabdomyolysis + GSPE group, however, there was a decrease in these mediators, together with an increase in eNOS expression. CONCLUSION: This study shows for the first time in the literature that calpain 1 is involved in the pathogenesis of rhabdomyolysis-induced AKI, and that GSPE may have a renoprotective effect.

Association between Expanded Disability Status Scale score and dietary antioxidant capacity in patients with multiple sclerosis.

Multiple sclerosis (MS), a neuroinflammation that results in neurodegeneration, is the most prevalent central nervous system inflammatory disease in young people. A diet rich in antioxidants is known to decrease the production/activity of pro-inflammatory cytokines and have a positive impact on the prognosis of MS. The purpose of this study was to assess if dietary antioxidant capacity is related to Expanded Disability Status Scale (EDSS) scores in patients with MS. Patients with MS (n=220; 137 women and 83 men) were asked to complete a questionnaire on diet. According to the EDSS score, patients were split into two groups (group 1: EDSS ≤5 and group 2: EDSS >5). Analyzed risk variables were antioxidant levels and demographic data. A nutritional database tool (BeBiS 4 software, Germany) created for the evaluation of Turkish foods was used to examine the questionnaire findings. Age, vitamin A, retinol, vitamin D, vitamin E, and vitamin C were significantly different between groups (P<0.05). The levels of vitamins A, D, E, C, and retinol were significantly correlated, according to Pearson's correlation analysis. Receiver operator characteristic curve analysis revealed that vitamin A, vitamin D, and vitamin C levels were discriminating variables in group 2 patients (EDSS >5). The current study has shown that antioxidant levels obtained by EDSS may be useful in determining illness severity and treatment success of patients with MS. Further clinical trials have been initiated in MS patients with more effective antioxidants.

To screen or not to screen renal cell cancer in a kidney transplant patient.

Background: The prevalence of malignancies is higher among kidney transplant recipients (KTR) than general population. Although the incidence of renal cell cancer (RCC) after KTR was reported as 0.6 % with a high mortality rate of 13.9 %, none of the guidelines except the European Best Practice Guideline (EBPG) recommends RCC screening based on cost-effectiveness and lack of solid evidence. The EBPG recommends RCC screening in native kidneys (not the allograft) by ultrasonography (USG) every 1-3 years. Case description: A 55-year-old male patient who had a kidney transplant from a living donor 14 years before, presented with sudden onset uncontrolled hypertension (180/110 mmHg) and rapid deterioration in graft functions (increase in serum creatinine to two-times baseline). Evaluations revealed RCC in the allograft. Abdominal pain, hematuria, fever, weight loss, flu-like syndrome, recurrent urinary tract infections, weakness, hypertension, and allograft dysfunction are reported as the main complaints and signs of RCC in KTR patients. Our patient's findings were hypertension and allograft dysfunction. Conclusion: It is essential to follow EBPG for KTR and not to forget the annual USG for screening of RCC both in native kidneys and allograft. HIPPOKRATIA 2023, 27 (2):69-71.

A case report of antineutrophil cytoplasmic antibody-associated vasculitis and glomerular immune depositions.

BACKGROUND: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic disease leading to renal complications of pauci-immune focal and segmental necrotizing crescentic glomerulonephritis (PI-NCGN). CASE DESCRIPTION: We present a 57-year-old female patient with rapidly progressive glomerulonephritis, multiple systemic infections [candidiasis and severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2)], severe weight loss, arthralgia, positive myeloperoxidase ANCA, acute deterioration of cardiac function and NCGN with heavy deposition of immunoglobulin (Ig) A and complement 3 (C3) in kidney biopsy. After two months of follow-up and appropriate treatments [methylprednisolone (60 mg/day), cyclophosphamide (15 mg/kg)], our patient recovered from multiple life-threatening infections, including candidiasis treated by fluconazole and SARS-CoV-2 treated by methylprednisolone and acute cardiac failure. In addition, she was saved from dialysis despite all poor prognostic factors. CONCLUSION: AAV might lead to immune complex deposition in kidneys due to different pathogenetic mechanisms like complement activation and immune complex formation, apart from losing tolerance to neutrophil proteins. HIPPOKRATIA 2022, 26 (2):86-88.

Is C1q nephropathy associated with a WDR19 gene mutation? A case report.

BACKGROUND: Even though complement 1q nephropathy (C1qN) was first introduced in 1985, this entity is still unknown and recognized by clinicians due to its rare prevalence (0.2 - 2.5 %) and insufficient emphasis. DESCRIPTION OF THE CASE: A 50-year-old woman was incidentally found to have non-nephrotic proteinuria with a normal glomerular filtration rate. Renal biopsy revealed C1qN with severe fibrosis. The presence of consanguinity and kidney diseases in family members of the patient led to genetic research, and homogenous mutation of c.991G>T (p.G331C) in the WD-repeat domain 19 (WDR19) gene was found. The same homozygous and heterozygous mutations in the WDR gene were found in the relatives of our patient with kidney diseases. One year of follow-up with methylprednisolone and mycophenolate mofetil treatment resulted in partial remission of the kidney disease. CONCLUSION: Renal biopsy for patients with non-nephrotic proteinuria without delay is suggested as it might be a surrogate marker of severe injury. Genetic mutations in the WDR19 gene should be searched for C1qN pathogenesis. This is the first adult case report on C1qN from Turkey.HIPPOKRATIA 2021, 25 (2):87-90.

Association between Expanded Disability Status Scale score and dietary antioxidant capacity in patients with multiple sclerosis

Multiple sclerosis (MS), a neuroinflammation that results in neurodegeneration, is the most prevalent central nervous system inflammatory disease in young people. A diet rich in antioxidants is known to decrease the production/activity of pro-inflammatory cytokines and have a positive impact on the prognosis of MS. The purpose of this study was to assess if dietary antioxidant capacity is related to Expanded Disability Status Scale (EDSS) scores in patients with MS. Patients with MS (n=220; 137 women and 83 men) were asked to complete a questionnaire on diet. According to the EDSS score, patients were split into two groups (group 1: EDSS ≤5 and group 2: EDSS >5). Analyzed risk variables were antioxidant levels and demographic data. A nutritional database tool (BeBiS 4 software, Germany) created for the evaluation of Turkish foods was used to examine the questionnaire findings. Age, vitamin A, retinol, vitamin D, vitamin E, and vitamin C were significantly different between groups (P<0.05). The levels of vitamins A, D, E, C, and retinol were significantly correlated, according to Pearson's correlation analysis. Receiver operator characteristic curve analysis revealed that vitamin A, vitamin D, and vitamin C levels were discriminating variables in group 2 patients (EDSS >5). The current study has shown that antioxidant levels obtained by EDSS may be useful in determining illness severity and treatment success of patients with MS. Further clinical trials have been initiated in MS patients with more effective antioxidants.

Pathological effects of prenatal exposure to a 900 MHz electromagnetic field on the 21-day-old male rat kidney.

We investigated the effects on kidney tissue of 900 megahertz (MHz) EMF applied during the prenatal period. Pregnant rats were exposed to 900 MHz EMF, 1 h/day, on days 13-21 of pregnancy; no procedure was performed on control group pregnant rats or on mothers or newborns after birth. On postnatal day 21, kidney tissues of male rat pups from both groups were examined by light and electron microscopy. Malondialdehyde (MDA), superoxide dismutase (SOD), catalase (CAT) and glutathione levels also were investigated. Light microscopy revealed some degenerative changes in the tubule epithelium, small cystic formations in the primitive tubules and large cysts in the cortico-medullary or medullary regions in the experimental group. Electron microscopy revealed a loss of peritubular capillaries and atypical parietal layer epithelial cells in the experimental group. Biochemical analysis showed significantly increased MDA levels in the experimental group and decreased SOD and CAT levels. EMF applied during the prenatal period can caused pathological changes in kidney tissue in 21-day-old male rats owing to oxidative stress and decreased antioxidant enzyme levels.