Interstitial cystitis: a rare manifestation of primary Sjögren’s syndrome, successfully treated with low dose cyclosporine

Chronic interstitial cystitis (IC), mostly affecting middle-aged women, is a very rare manifestation of primary Sjögren’s syndrome (pSS). Hereby, we report a 42-year-old woman with pSS, presenting with dysuria, urinary frequency, and suprapubic pain. She was diagnosed to have chronic IC, based upon the cystoscopic biopsy finding of chronic inflammation in the bladder wall. Systemic corticosteroid and azathioprine treatments together with local intravesical therapies were not effective. Therefore, cyclosporine (CSA) therapy was initiated. Initial low dose of CSA (1.5 mg/kg/d) improved the symptoms of the patient, with no requirement for dose increment. After 4 months of therapy, control cystoscopic biopsy showed that bladder inflammation regressed and IC improved. This case suggests that even low doses of CSA may be beneficial for treating chronic IC associated with pSS syndrome

Effect of octreotide on oxidative stress in the erythrocyte and kidney tissue in adriamycin-induced experimental nephrotic syndrome model

Abstract Introduction: Nephrotic syndrome (NS) is one of the reasons of end-stage kidney disease, and elucidating the pathogenesis and offer new treatment options is important. Oxidative stress might trigger pathogenesis systemically or isolated in the kidneys. Octreotide (OCT) has beneficial antioxidant effects. We aimed to investigate the source of oxidative stress and the effect of OCT on experimental NS model. Methods: Twenty-four non-uremic Wistar albino rats were divided into 3 groups. Control group, 2 mL saline intramuscular (im); NS group, adriamycin 5 mg/kg intravenous (iv); NS treatment group, adriamycin 5 mg/kg (iv) and OCT 200 mcg/kg (im) were administered at baseline (Day 0). At the end of 21 days, creatinine and protein levels were measured in 24-hour urine samples. Erythrocyte and renal catalase (CAT) and thiobarbituric acid reactive substance (TBARS) were measured. Renal histology was also evaluated. Results: There was no significant difference among the 3 groups in terms of CAT and TBARS in erythrocytes. Renal CAT level was lowest in NS group, and significantly lower than the control group. In treatment group, CAT level significantly increased compared with NS group. In terms of renal histology, tubular and interstitial evaluations were similar in all groups. Glomerular score was significantly higher in NS group compared with control group and it was significantly decreased in treatment group compared to NS group. Conclusions: Oxidative stress in NS might be due to the decrease in antioxidant protection mechanism in kidney. Octreotide improves antioxidant levels and histology in renal tissue and might be a treatment option

Rapidly progressive glomerulonephritis in a child with Henoch-Schönlein Vasculitis and familial Mediterranean fever

Henoch-Schonlein Vasculitis (HSV) is systemic small vessel vasculitis involving the skin, kidney, joints, and gastrointestinal tract. The proportion of patients reported to have renal involvement varies between 20% and 80%. Rapidly progressive glomerulonephritis (RPGN)is rare syndrome in children, characterized by clinical features of glomerulonephritis (GN) and rapid loss of renal function. We present a severe kidney involvement in a 14 year old boy with HSV in who is carring MEFV mutation. A 14 year old boy had developed sudden onset of palpable purpuric rash on his extensor surfaces of lower extremities. He had elevated an erythrocyte sedimentation rate (ESR) (45 mm/h), C-reactive protein (3.74 mg/dl), serum urea 66 mg/dl, serum creatinine 1.8 mg/dl. Also, he had hypocomplementemia. Antinuclear antibody, anti ds DNA, antineutrophil cytoplasmic antibody, anticardiolipine antibodies were negative. Urinalysis revealed macroscopic hematuria and proteinuria with a 24-h urinary protein excretion of 55 mg/m2/h. The renal biopsy specimen showed crescentic and necrotizing glomerulonephritis. He had also M694V/E148Q compound heterozygote mutation. Clinical symptoms and renal failure resolved with intermittant hemodialysis and medical therapy

Renal complications of lipodystrophy: A closer look at the natural history of kidney disease

Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/144612/1/cen13732_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/144612/2/cen13732.pd

Prognostic model to predict postoperative acute kidney injury in patients undergoing major gastrointestinal surgery based on a national prospective observational cohort study.

Background: Acute illness, existing co-morbidities and surgical stress response can all contribute to postoperative acute kidney injury (AKI) in patients undergoing major gastrointestinal surgery. The aim of this study was prospectively to develop a pragmatic prognostic model to stratify patients according to risk of developing AKI after major gastrointestinal surgery. Methods: This prospective multicentre cohort study included consecutive adults undergoing elective or emergency gastrointestinal resection, liver resection or stoma reversal in 2-week blocks over a continuous 3-month period. The primary outcome was the rate of AKI within 7 days of surgery. Bootstrap stability was used to select clinically plausible risk factors into the model. Internal model validation was carried out by bootstrap validation. Results: A total of 4544 patients were included across 173 centres in the UK and Ireland. The overall rate of AKI was 14·2 per cent (646 of 4544) and the 30-day mortality rate was 1·8 per cent (84 of 4544). Stage 1 AKI was significantly associated with 30-day mortality (unadjusted odds ratio 7·61, 95 per cent c.i. 4·49 to 12·90; P < 0·001), with increasing odds of death with each AKI stage. Six variables were selected for inclusion in the prognostic model: age, sex, ASA grade, preoperative estimated glomerular filtration rate, planned open surgery and preoperative use of either an angiotensin-converting enzyme inhibitor or an angiotensin receptor blocker. Internal validation demonstrated good model discrimination (c-statistic 0·65). Discussion: Following major gastrointestinal surgery, AKI occurred in one in seven patients. This preoperative prognostic model identified patients at high risk of postoperative AKI. Validation in an independent data set is required to ensure generalizability

Morphologic and Molecular Charecteristics of Renal Tumors

WOS: 000219417400007Renal cell carcinomas (RCCs) are heterogeneous group of cancers that arise from the tubuler cells and are responsible for 80-85% of all primary renal neoplasms. Less than 5% of all RCCs are thought to be due to a hereditary syndrome. Family studies and molecular genetics have provided important insights into the molecular pathways underlying the pathogenesis of RCCs as well as new insights into classification of RCC. Morphologically based classification of RCCs has evolved over the last 3 decades with the incorporation of molecular genetics into the diagnostic features RCCs. The most recent 2012 Vancouver classification recognized the newly established entities tubulocystic RCC, acquired cystic disease-associated RCC, clear cell (tubulo) papillary RCC, microphthalmia transcription factor family translocation RCC and hereditary leiomyomatosis RCC syndrome-associated RCC. In this article, the molecular and genetic properties of RCCs were summarized and the mechanisms underlying the development new entities of RCCs were discussed. The use of immunohistochemistry and molecular techniques were also evaluated in establishing an accurate diagnosis in new entities

TECHNICAL ASPECTS IN STUDYING PERITONEAL MORPHOLOGY IN ANIMAL MODELS OF PERITONEAL DIALYSIS

12th Congress of the International-Society-for-Peritoneal-Dialysis -- JUN 20-24, 2008 -- Istanbul, TURKEYWOS: 000263937100007PubMed ID: 19270229Objective: Peritoneal biopsies are considered useful for gaining a better understanding of the pathophysiology of the peritoneum during experimental peritoneal dialysis (PD). Different peritoneal tissue samples (i.e., abdominal wall, liver, diaphragm, intestine, and omentum) may be used, but there can be artifacts due to peritoneal tissue processing. Aim: To investigate differences in peritoneal membranes from different parts of the peritoneum, and also 2 different fixatives, in experimental PD and a peritonitis model in rats. Methods: Peritoneal tissues from the anterior abdominal wall, liver, omentum, and intestine were taken from each of 3 groups of animals: sham, experimental PD, and peritonitis model. Tissue samples were immediately fixed with 4% formaldehyde and routinely processed for histological examination. Two parietal peritoneal tissue samples according to longitudinal and horizontal sections of anterior wall inner abdominal muscle were also taken. All samples were immediately fixed with 4% formaldehyde and B5 fixative (B5), and then routinely processed for histological examination. Results: In all groups, histopathological findings were more commonly seen in the abdominal wall samples. There were no changes observed in peritoneal membranes other than those of anterior abdominal wall samples from both sham and PD model rats. However, there was a significant difference between anterior and posterior facets of liver in the peritonitis model. Furthermore, the antimesenteric site of intestinal peritoneum was less affected than the mesenteric site. There were no significant histopathological differences between B5 and 4% formaldehyde fixation (p > 0.05). Conclusion: Our results suggest that peritoneum obtained from the anterior abdominal wall is the most affected area and therefore the most suitable site to investigate peritoneal changes in the experimental rat PD model. There were no significant differences between fixation with 4% formaldehyde and B5 solution. Abdominal wall samples should be of the same direction of inner abdominal muscle, that is, horizontal sectioning should be used for measurements of the submesothelial area.Int Soc Peritoneal Dialysi

A Proposed Histopathologic Classification, Scoring, and Grading System for Renal Amyloidosis Reply

WOS: 00028743050000