A case of Sjögren's syndrome with worsening of pleural effusion due to steroid discontinuation

The etiology of Sjögren's syndrome (SS) remains unclear and is associated with many other autoimmune diseases. In particular, symptoms of SS are not apparent when steroids are already being administered for other autoimmune diseases. This report documents a case of autoimmune hepatitis with SS, which was diagnosed on the basis of the worsening of unilateral pleural effusion after the discontinuation of steroids as well as the manifestation of symptoms, such as dry mouth. Adrenal insufficiency due to the discontinuation of steroids was assumed to be the cause of the worsening, rather than infection stress, because no indicators of infectious diseases were observed. The diagnosis of SS was confirmed via lip biopsy examination and anti-SS antibody positivity. Re-administration of steroids rather than antibiotics drastically reduced the pleural effusion and improved the dry mouth symptom. SS with pleural effusion in a case of autoimmune disease was reported to show both unilateral and bilateral pleural effusion predominantly containing lymphocytes. SS with pleural effusion may be more common than expected and should be differentiated from traditional SS. Moreover, biopsy examination should be considered if necessary because the condition might remain latent when steroids are administered

Rapid Decline of IFN-γ Spot-Forming Cells in Pleural Lymphocytes during Treatment in a Patient with Suspected Tuberculosis Pleurisy

A differential diagnosis of tuberculosis pleurisy is often difficult. A 48-year-old Japanese man with no previous medical history visited the outpatient department for dyspnea and fever. His chest-XP and laboratory findings, especially high C-reactive protein levels, indicated pleuritis with pleural effusion. Pleural lymphocytes showed high numbers of spot forming responses in interferon gamma release assay (IGRA). Pleural effusion contained high levels of adenosine deaminase and hyaluronic acid, but no Mycobacterium tuberculosis (TB) antigen was detected by culture or polymerase chain reaction (PCR). Although the infectious agent was not detected, the clinical and laboratory findings strongly suggested that he was suffering from tuberculosis pleurisy. After treatment with anti-TB drugs, a rapid decline of spot-forming cells (SFCs) of pleural lymphocyte was observed, despite persistently high levels of other biomarkers and increased pleural lymphocytes. This case demonstrates that an IGRA of pleural lymphocytes would be useful for therapeutic diagnosis for TB pleurisy suspected for TB

重複腎盂尿管と尿管異所開口を伴った巨大水腎症の1例

33歳女.腹部膨隆を主訴とし, 腹部超音波検査, DIPと順行性腎盂造影, CT, MRI, 膀胱鏡検査等で, 両側重複腎盂尿管の右上半腎より発生した巨大水腎症と術前診断された.腰部斜切開で後腹膜腔に入ると右上半腎所属の尿管は明らかな腎盂尿管移行部狭窄を伴っており, これにより巨大水腎症が発生したと思われた.尿管異所開口の部位は断定できないが, すくなくとも外尿道括約筋群よりも遠位であると考えられた.巨大水腎とその所属尿管は一塊として摘出され, 巨大水腎の内容量は2, 640mlであった.術後1年目のDIPで良好な経過を取っているGiant hydronephrosis is an uncommon clinical entity. Even more uncommon is the association of giant hydronephrosis with a double collecting system and ectopic ureter. Here, we report a case of giant hydronephrosis of the bilateral duplex systems associated with ureteral ectopia. The patient underwent upper pole nephrectomy and upper ureterectomy. To our knowledge, only four similar cases have been reported previously

同一腎に腎細胞癌と腎血管筋脂肪腫を合併した1例

結節性硬化症の合併は認めない.腹部超音波検査にて, 右腎の中部外側に高エコーを呈する腫瘤を, 下極付近に前者よりも更に高エコーを呈する腫瘤と指摘され当科受診.CT, MRI, 血管造影にて, 右腎中部外側の腫瘤は腎細胞癌と術前診断された.1990年3月経腹膜的根治的右腎摘除術を施行, 中部外側の腫瘍は腎細胞癌, alveolar type, common type, clear cell subtype, G1と, 下極付近の腫瘍は腎血管筋脂肪腫と病理診断された.術後経過は良好で, 術後61ヵ月再発転移を認めないCoexistence of renal cell carcinoma and angiomyolipoma in the same kidney is rare. A 54-year-old woman without tuberous sclerosis was admitted for further examination of incidental renal masses on ultrasonography. Computerized tomography revealed a 17-mm high density mass in the mediolateral portion of the right kidney and a 5-mm low density mass near the right lower pole. Because the former mass showed a typical tumor pattern on selective renal angiography and the latter mass was strongly hyperechoic on ultrasonography, a clinical diagnosis of renal cell carcinoma and angiomyolipoma was made. A right radical nephrectomy confirmed the preoperative diagnosis. She has been followed for 61 months with no recurrence

Prognostic value of myocardial perfusion SPECT images in combination with the maximal heart rate at exercise testing in Japanese patients with suspected ischemic heart disease: A sub-analysis of J-ACCESS

Objectives We assessed whether a combination of summed stress scores (SSS) using exercise myocardial perfusion SPECT (Ex-SPECT) and maximal heart rate accurately predicts cardiac events through a sub-analysis of J-ACCESS (Japanese Assessment of Cardiac Events and Survival Study by Quantitative Gated SPECT) which was conducted to evaluate the prognosis of Japanese patients with suspected ischemic heart disease. Methods In J-ACCESS, 2, 373 patients with suspected coronary artery disease not receiving beta-blocker treatment underwent Ex-SPECT. These patients were categorized into the following four groups: Group A [achieved target heart rate (THR) and SSS < 4: n = 631], B (did not achieve THR and SSS < 4: n = 612), C (achieved THR and SSS ≥ 4: n = 570), and D (did not achieve THR and SSS ≥ 4: n = 560). We evaluated the incidence rate of cardiac events including cardiac death, myocardial infarction, and heart failure requiring hospital admission during a 3-year period. Results In Group A, B, C, and D, 9 of 631 (1.4%), 15 of 612 (2.4%), 23 of 570 (4.0%) and 30 of 560 (5.4%) patients experienced cardiac events, respectively. Although the hazard ratio of the SSS ≥ 4 was 2.45 (p < 0.001) and that of the attained THR was 0.69 (p = 0.10) in the multiple Cox regression analysis, Kaplan–Meier curves showed that the cardiac events rate was lower in the order of A, B, C, and D (p < 0.001). Conclusion The combination of SSS using Ex-SPECT and the maximal heart rate is a useful predictor of cardiac events in patients with suspected coronary artery disease

Expression patterns of miRNA-423-5p in the serum and pericardial fluid in patients undergoing cardiac surgery

Background: Recently, it has been reported that specific microRNA (miRNA) levels are elevated in serum and can be used as biomarkers in patients with cardiovascular diseases. However, miRNAs expression profiles and their sources in pericardial fluid (PF) are unclear. Methods and Results: The purpose of this study was to identify the levels of miRNAs in PF in relation to those in the serum in patients undergoing cardiac surgery. Serum (S) and PF from patients undergoing coronary artery bypass graft (CABG) due to stable angina pectoris (sAP) and unstable AP (uAP) and aortic valve replacement due to aortic stenosis (AS) were analyzed for the detection of miRNAs. We named these samples S-sAP, S-uAP, S-AS, PF-sAP, PF-uAP, and PF-AS, respectively. We first measured the levels of miR-423-5p, which was recognized previously as a biomarker for heart failure. miR-423-5p levels were significantly higher in PF than serum. Although there was no difference in miR-423-5p levels among the PF-AS, PF-sAP, and PF-uAP, its levels were significantly elevated in S-uAP compared with those in S-AS and S-sAP. In order to clarify the source of miR-423-5p in PF, we measured the levels of muscle-enriched miR-133a and vascular-enriched miR-126 and miR-92a in the same samples. miR-133a levels were significantly higher in serum than in PF, and it was elevated in S-uAP compared with S-AS. miR-126 level was significantly increased in serum compared with PF, and the level of miR-92a the similar tendency. miR-423-5p is located in the first intron of NSRP1. There is another miRNA, miR-3184, encoded in the opposite direction in the same region. In vitro experiments indicated that the duplex of miR-423-5p and miR-3184-3p was more resistant to RNase than the duplex of miR-423-5p and miR-133-3p, which may help to stabilize miR-423-5p in the PF. Conclusions: Our results suggested that miR-423-5p is enriched in PF, and serum miR-423-5p may be associate with uAP. Its expression pattern was different to that of muscle- and vascular-enriched miRNAs, miR-133a, miR-126, and miR-92a

A Case of Manila Type Mycobacterium Tuberculosis Infection in Japan

A 76-year-old Japanese woman contracted a Mycobacterium tuberculosis (TB, Manila type) infection in Japan, despite never having traveled. However, her son was treated for TB in the Philippines 3 years before he stayed at her house. Spoligotyping allows us to identify the TB genotype and identify the route of infection