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Background/Aim: We recently reported that renal tubular acidosis (RTA) in Sjögren’s syndrome (SjS) is associated with high titers of an autoantibody against carbonic anhydrase (CA) II, an important enzyme in renal acid-base regulation. The purpose of this study was to determine whether a CA-II antibody could cause RTA in a mouse model of SjS. Methods: PL/J mice were immunized with human CA II to induce CA II antibody formation, whereas controls were injected with phosphate-buffered saline and adjuvant. After 6 weeks, anti-CA-II antibody titers were measured, then ammonium chloride was administered orally for 1 week to detect any acidification defect. Results: CA-II-immunized mice showed higher anti-CA-II antibody titers than control mice. Pathologically, lymphocytic and plasma cell infiltration was seen in the salivary glands and kidneys of CA-II-immunized mice, but not in controls. On acid loading, blood pH and urine pH decreased in both groups of mice, but the slope of urine pH versus blood pH was less steep in the CA-II-immunized mice, suggesting that these mice had an impaired ability to reduce their urine pH in the face of metabolic acidosis. Conclusion: CA-II-immunized mice had a urinary acidification defect, which may be similar to that seen in patients with SjS.

Background: Infected cysts are a frequent and serious complication of autosomal dominant polycystic kidney disease. Such infections are classified into those affecting hepatic cysts and those affecting renal cysts. The purpose of this study was to compare the clinical course of infected hepatic cysts with that of infected renal cysts in patients with autosomal dominant polycystic kidney disease. Methods: We analyzed 43 patients referred to us for additional treatment of severely infected cysts between January 2004 and December 2006. All patients who required further treatment in addition to antibiotic therapy were included. Results: Aspiration was performed in all 28 patients with infected hepatic cysts. As a result, 17 patients were cured, 4 remain under treatment, and 6 died. One patient was cured by partial hepatectomy. Among the 15 patients with renal cysts, aspiration was performed in 4 with identifiable infected cysts, while renal transcatheter arterial embolization after appropriate antibiotic therapy was performed in 11 without identifiable infected cysts. No patient developed recurrence. Conclusion: In patients with infected renal cysts, aspiration or renal transcatheter arterial embolization after appropriate antibiotic therapy was effective. Although aspiration was often effective in patients with infected hepatic cysts, a good outcome was less likely than in those with renal cysts.

Nephrotic syndrome (NS) may be complicated by thromboembolism, which occasionally manifests as stroke. Although the optimal, standardized approach to the prophylaxis and management of thromboembolic complications associated with NS has not been established, anticoagulation with heparin and subsequent warfarin is the de facto standard of treatment. Dabigatran, a novel direct thrombin inhibitor, has become a substitute for warfarin and heparin for many indications, including the prophylaxis of stroke associated with nonvalvular atrial fibrillation and postoperative thromboprophylaxis in orthopedic patients. We report a 35-year-old male with NS due to membranous nephropathy (MN) that presented with carotid thromboembolism. Because the patient developed drug-induced hepatitis due to warfarin, we attempted treatment with dabigatran and were successful in continuing the medication without any complications. We also reviewed the literature on stroke associated with NS. Twenty-one prior cases have been reported, and the review of these cases revealed some interesting points. The age of onset ranged from 19 to 59 years. Most of the reported cases sustained a stroke at earlier ages than patients with atherosclerosis and atrial fibrillation, which suggests that NS may independently predispose individuals to arterial and venous thromboses. MN was the most common underlying pathology. Given that a standardized approach to the prophylaxis and management of thrombotic complications associated with NS has not been established, our experience suggests that dabigatran is a valid new treatment option for thrombotic complications of NS.

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