The patient was a 68-year-old woman who had undergone initial mitral repair at 24 years of age, and had undergone mitral replacement using the Björk-Shiley convexo-concave valve at 30 years of age. She developed exertional dyspnea 38 years after mitral replacement with hemolytic anemia. Precise examination revealed mitral stenosis and perivalvular leak. At the reoperation, severe calcified pannus was found at the ventricular side just beneath the mitral artificial valve, and made stenosis with the inadequate leaflet opening. The mitral valve remnant ring was severely calcified and the sawing ring was detached partially. Repeated valve replacement was successfully done by the reinforcement of the mitral valve ring with xenopericardium.

Objective: Maternal embryonic leucine zipper kinase (MELK) is receiving an attention as a therapeutic target in various types of cancers. In this study, we aimed to evaluate the prognostic significance of MELK expression in ovarian cancer using clinical samples, and assessed the efficacy of a small molecule MELK inhibitor, OTS167, using patient-derived ovarian cancer cells as well as cell lines. Methods: Expression levels of MELK in 11 ovarian cancer cell lines were confirmed by western blotting. Inhibitory concentration of OTS167 was determined by colorimetric assay.MELK messenger RNA (mRNA) expression was evaluated in 228 ovarian cancer patients by quantitative polymerase chain reaction. Growth inhibition of OTS167 was also evaluated using freshly-isolated primary ovarian cancer cells including spheroid formation condition. Results: MELK mRNA expression was significantly higher in ovarian cancer than in normal ovaries (p<0.001), and high MELK mRNA expression was observed in patients with advanced stage, positive ascites cytology and residual tumor size. Patients with high MELK mRNA expression showed shorter progression-free survival (p=0.001). Expression of MELK was also confirmed in 10 of 11 ovarian cancer cell lines tested, and the half maximal inhibitory concentration of MELK inhibitor, OTS167, ranged from 9.3 to 60 nM. Additionally, OTS167 showed significant growth inhibitory effect against patient-derived ovarian cancer cells, regardless of their tumor locations, histologic subtypes and stages. Conclusions We demonstrated MELK as both a prognostic marker and a therapeutic target for ovarian cancer using clinical ovarian cancer samples. MELK inhibition by OTS167 may be an effective approach to treat ovarian cancer patients.

Ankylosing spondylitis is chronic, progressive, inflammatory disease involving the spine, peripheral joints, and periarticular structures. Cardiac abnormalities associated with ankylosing spondylitis are well recognized, but a case with DDD pacemaker implantation for complete atrioventricular block and aortic valve replacement for aortic regurgitation has not been previously reported. We report a case of a 66-year-old man with ankylosing spondylitis who was successfully treated by DDD pacemaker implantation for complete atrioventricular block and aortic valve replacement for severe aortic regurgitation.

Background : Perioperative care in congenital heart surgery has evolved in recent years, and it was considered a contributive factor to improve surgical outcome and prognosis. Objective : To extract perioperative clinical protocols that have been applied in our hospital, then assess their usefulness for better clinical outcome. Methods : We retrospectively reviewed our patients' records to analyze representative perioperative protocols that might have contributed to surgical outcome, such as intraoperative transesophageal echocardiography (ITEE), extubation in the operating room on patients of atrial septal defect (ASD), ventricular septal defect (VSD), tetralogy of Fallot (TOF), Glenn procedure and Fontan procedure. We also assessed clinical pathway of ASD and VSD, and each protocol was individually explored to calculate achievement ratio in order to show its adequacy. Results : This study included 482 of on-pump surgery patients and 146 of off-pump surgery patients from June 2007 to June 2014. ITEE was performed in 474 of on-pump surgery patients and 102 of off-pump surgery patients. No case had a residual lesion immediately after operation. Extubation in the operating room was performed in cases without severe pulmonary hypertension (PH). The extubation ratio was 94.7% (ASD repair), 60.0% (VSD repair), 50.0% (TOF repair), 42.5% (Glenn procedure), and 45.2% (Fontan procedure), respectively. Clinical pathways of ASD and VSD included patients without severe PH. Achievement ratio of the clinical pathway was 98.2% in ASD and 94.2% in VSD patients, respectively. Four patients were excluded because of high c-reactive protein (CRP), and one patient because of familial circumstance. Conclusion : ITEE was useful in evaluation of cardiac function, residual issue and residual air at weaning of cardiopulmonary bypass. Reintubation did not occur in any clinical course of extubation in the operating room, but the extubation rate was not high because of safety concerns. Achievement ratio of the clinical pathways of both ASD and VSD was more than 90%, therefore, application of the clinical pathway was considered appropriate.

We report a patient with Leriche syndrome who had ileal conduit and a right lower quadrant stoma. A 47-year-old man with a history of bladder cancer had undergone radical total cystectomy with formation of an ileal conduit and right lower quadrant stoma 2 years previously. CT scanning revealed total occlusion of the distal aorta. He experienced right lower leg pain after 30 m of walking. Through a repeat midline laparotomy incision, the abdominal aorta was dissected with a transperitoneal approach. To avoid dissection around the ileal conduit, the retroperitoneum was incised (open) at the right of the ascending colon and at the left of the sigmoid colon. A prosthetic graft (Interguard 14×7 mm) was pulled bilaterally through these incisions, to the external iliac arteries. Abdominal aorto-bilateral external iliac artery bypass grafting was performed and the patient was discharged without complications on the 15th postoperative day.

Bicuspid aortic valve (BAV) is one of the more common congenital anomalies. It is well known that the ascending aorta and aortic root sometimes dilate in patients with BAV, even when the valve function is normal. We examined the morphological type and histological features of the dilated ascending aorta in patients with BAV. Of 276 patients who underwent aortic valve replacement (including coronary artery bypass grafting) from 2004 onwards, 60 (21.5%) with BAV were included in this study. The type of BAV was defined according to the Sievers classification. Type 1 BAV was the most common, and enlargement of the ascending aorta (≥45 mm) was the most common in the L/R type of BAV (48%). The morphology of the dilated ascending aortic wall was evaluated using three-dimensional CT angiography. The majority of dilations were asymmetric, but dilation was symmetric in the patient with dilation of the aortic root. Histological examination graded cystic medial necrosis of the ascending aortic walls using the aortic wall score. All patients with BAV had degeneration of the aortic wall, even when there was no dilation. The aortic walls of patients with dilated aortic roots showed advanced degeneration compared with the aortic walls of other patients. Therefore, aggressive root replacement may be appropriate, when the root is mildly dilated in patient with BAV.

BACKGROUND/AIMS: Autoimmune pancreatitis (AIP) is frequently associated with diabetes mellitus (DM). This study evaluated the effect of steroid therapy on the course of DM in AIP. METHODS: Glucose tolerance was examined in 69 patients with AIP. DM onset was classified as either a simultaneous onset with AIP or an exacerbation of pre-existing DM. Based on the changes in the HbA1c levels and insulin dose, the responses of DM to steroids were classified as improved, no change, or worsened. RESULTS: Thirty (46%) patients were diagnosed as having DM (simultaneous onset, n=17; pre-existing, n=13). Three months after starting the steroid treatment, the DM improved in 13 (54%) of 24 DM patients. The DM improved in 55%, had no change in 36%, and worsened in 9% of the 11 simultaneous onset DM patients, and it improved in 54%, had no change in 31%, and worsened in 15% of the 13 pre-existing DM patients. At approximately 3 years after starting the steroid treatment, the DM improved in 10 (63%) of 16 patients. The pancreatic exocrine function improved in parallel with the changes in the DM in seven patients. CONCLUSIONS: Because approximately 60% of DM associated with AIP is responsive to steroids in the short- and long-terms, marked DM associated with AIP appears to be an indication for steroid therapy.
Diabetes Mellitus ; Glucose ; Humans ; Insulin ; Pancreatitis ; Steroids