An 11-year-old boy, who underwent a Rastelli operation using a 14mm artificial graft and left pulmonary artery (PA) plasty with an autologous pericardium patch 7 years previously, had severe recurrent left pulmonary stenosis. Reoperation was performed including right ventricular outflow tract reconstruction and left PA plasty. The PA at the most stenotic site was only 2mm in diameter; it was enlarged to 10mm by good exposure and an incision on the pulmonary intima. A bovine pericardium patch with a handmade ePTFE valve was sutured onto the autologous tissue not onto the pulmonary intima to avoid restenosis and in expectation of the growth of the pulmonary orifice. On postoperative 3-D CT, the left pulmonary artery was patent and 9mm in diameter. Pulmonary scintigraphy showed an improvement in the left pulmonary perfusion. This sutureless technique was useful in this case of severe pulmonary stenosis.

Objective: Since the conventional drip-infusion method for measuring inulin clearance (Cin) has problems related to its accuracy and performance, we explored a more accurate and concise method by rapid intravenous injection of a newly developed inulin fraction (Inulead^®), in which spot urine sampling was omitted and the administration period of inulin was shortened from 120 to 5 minutes. Patients and Methods: Twenty seven patients (M/F: 15/12, 67.8 ± 12.9 years old) admitted to the Nephrology ward were enrolled in this study. Inulead^®, 1500 mg dissolved in 150 mL of saline, was intravenously administered in 5 minutes. Then, sequential blood samplings and urine collection were performed for 24 hours. Cins were calculated by the following three formulae: (1) a pharmacokinetic analysis using a two compartments model based on the plasma inulin concentration to determine Cin, which was the administered dose divided by the area under the curve (AUC) from 0 to ∞, (2) urinary inulin excretion divided by the AUC for 24 hours and (3) the Bayesian method using a three-point set of plasma inulin concentrations to predict the change of inulin concentration to determine Cin as in 1. These Cins were compared with levels of estimated GFR (eGFR), creatinine clearance (Ccr), serum β2 microglobulin (β2MG) and serum cystatin C (Cys C). Results: Cins obtained by the above three methods were well correlated with each other (r. = 0.9088 – 0.9998) and with eGFR (r. = 0.8286 – 0.8650), Ccr (r. = 0.821 – 0.864), 1/β2MG (r. = 0.631 –0.752) and 1/CysC (r. = 0.830 – 0.857). The averaged differences of each Cin from eGFR were distributed between –4.4 and –4.5 mL/min. Conclusion: Since the Cins by rapid inulin injection showed satisfactory correlation and differences with other GFR parameters, this method will be a good alternative to the drip infusion method, and may reduce the burden of patients and medical staff.

This study investigated the effects of exercise intervention with a 12-week slightly-weighted shoe on lower-limb skeletal muscle and gait patterns in the elderly. A total of 29 healthy elderly Japanese who had irregular walking habits were randomly assigned to either slightly-weighted-shoe (WS group, n = 14; Age, 70.6 ± 5.7 years; WS, 493 g) or normal-shoe (NS group, n = 15; Age, 69.3 ± 6.9 years; NS, 293 g) intervention groups. The participants were instructed to maintain their normal daily physical activity (PA) during the intervention period. Segmental intracellular water (ICW) and muscle thickness (MT) were measured as an index of skeletal muscle mass in the lower limb, and kinematic gait data were acquired by motion analysis. Walking stability was assessed as a standard deviation of the vertical fluctuation in whole-body center of mass (COM fluctuation). The daily PA was monitored using an accelerometer and an activity record. ICW in the upper leg and MT of rectus femoris increased significantly in the WS group compared with the NS group (ICW: 13.8% vs. 2.2%, MT: 12.1% vs. 1.3%), while COM fluctuation was significantly reduced in the WS group (p<0.05) during normal walking. The present study demonstrated that interventions with a slightly-weighted-shoe may be able to increase muscle volume in the upper leg and change gait patterns in the healthy elderly.

The 76-year-old woman underwent double bioprosthetic valve replacement for aortic and mitral valve regurgitation without any postoperative complication. About 33 months later, the patient complained of sudden dyspnea and was diagnosed with mechanical hemolytic anemia, severe aortic regurgitant jet collision with stent post (SP) at mitral position and acute heart failure. The cause of mechanical hemolysis was suspected to be a collision of the regurgitant jet due to structural valve deterioration (SVD) with the SP because of the absence of any paravalvular leak (PVL). The externally-mounted bioprosthetic aortic valve was replaced and the inadequate projection of the SP in left ventricular outflow tract was recognized simultaneously. The patient fully recovered from heart failure and hemolytic anemia after surgery. Early SVD of externally-mounted bioprosthetic valves has often been reported, and the eccentric regurgitant jet due to SVD may collide with any sub-valvular structures. We report a rare case of hemolytic anemia due to SVD.

Background/Aims: Thiopurines are key drugs for inflammatory bowel disease (IBD), including ulcerative colitis (UC) and Crohn’s disease (CD). Recently, NUDT15 polymorphism (R139C, c.415C > T) has been shown to be associated with thiopurineinduced adverse events in Asian populations. In patients with the C/T genotype, low-dose thiopurine treatment is recommended, but its long-term efficacy and tolerability remain unclear. This study aimed to uncover the long-term efficacy and appropriate dosage of thiopurine for IBD patients with the C/T genotype. Methods: A total of 210 patients with IBD (103 UC and 107 CD) determined to have NUDT15 R139C variants were enrolled. Clinical data were retrospectively reviewed from medical records. Results: Of 46 patients (21.9%) with the C/T genotype, 30 patients (65.2%) were treated with thiopurines. Three of whom (10.0%) discontinued thiopurine treatment due to adverse events and 27 of whom continued. The median maintenance dosage of 6-mercaptopurine was 0.25 mg/kg/day (range, 0.19–0.36 mg/kg/day), and 6-thioguanine nucleotides level was 230 (104–298) pmol/8 × 108 red blood cells. Cumulative thiopurine continuation rates for 120 months for patients with the C/C and C/T genotypes were not significantly different (P= 0.895). Cumulative non-relapse rates in the patients with UC treated with thiopurine monotherapy and surgery-free rates in CD patients treated with combination therapy (thiopurines and anti-tumor necrosis factor-α agents) for maintenance remission were not significantly different at 60 months (C/C vs. C/T, P= 0.339 and P= 0.422, respectively). Conclusions Low-dose thiopurine treatment is an effective and acceptable treatment for patients with C/T genotype.

Trousseau's syndrome is a condition in which thromboembolic events are triggered by hypercoagulation caused by a malignancy. Here, we report two cases in which gynecologic cancer was detected after cerebral infarction. Patient 1 was a 66-year-old gravida 0, para 0 who presented with left-sided arm and leg weakness. Head magnetic resonance imaging (MRI) facilitated a diagnosis of cerebral infarction. Subsequent contrast computed tomography (CT) revealed an ovarian tumor, and she underwent surgery on day 8 after admission. The tumor was diagnosed as ovarian clear cell carcinoma on pathological examination. She received adjuvant chemotherapy and the cancer has not progressed in more than 2 years after the surgery. Patient 2 was a 41-year-old gravida 1, para 1 who presented with impairment of consciousness, right hemiplegia, and aphasia. Head MRI facilitated a diagnosis of cerebral infarction. Contrast MRI and contrast CT findings were suggestive of endometrial cancer, and she underwent surgery on day 19 after admission. The cancer was diagnosed as grade 3 endometrial cancer on pathological analysis. She received adjuvant chemotherapy and the cancer has not progressed in more than 4 years after the surgery. Although treatment of the primary disease is considered to affect prognosis in Trousseau’s syndrome, many patients have poor survival prospects because, when detected, their cancer is too advanced to be treated surgically. These cases illustrate the need for close cooperation with other departments such as neurology and cerebrovascular surgery to ensure that these cancers are diagnosed quickly and the opportunity to start multimodality treatment is not missed.

In Japan, there are no predetermined guidelines on the management of pregnant women at 41 weeks of pregnancy. In our hospital, pregnant women at 41 weeks of pregnancy are candidates for induction, but induction may result in emergency caesarean section, atonic bleeding postpartum hemorrhage, and neonatal emergencies with admission to the Neonatal Intensive Care Unit (NICU). Therefore, we conducted a retrospective investigation to determine which cases are likely to end in such events. Our hospital recorded a total of 3,492 deliveries during the period 2013-2015. Of these, 382 pregnancies were delivered at 41 weeks. We compared the labor induction group and the spontaneous delivery group. The rate of emergency caesarean section for multiparas was 1％ and was as high as 21％ for primiparas, so we focused on primiparas in this study. The 258 primiparas from among all 382 cases were divided into the induction group (n＝122) and the spontaneous group (n＝136). Compared with the spontaneous group, the induction group had older primiparas, more body weight gain during pregnancy, and a high risk of emergency caesarean section. There was no significant difference in the proportion of neonates admitted to the NICU. In the analysis focused on the induction group, the rate of emergency cesarean section increased as the number of cases requiring induction and the number of elderly primiparas increased. With the recent increasing age of primiparas, it is necessary to educate women on the risks of weight gain during pregnancy.

Background/Aims: Endoscopic self-expandable metal stent (SEMS) placement is currently the standard technique for treating unresectable malignant distal biliary obstructions (MDBO). Therefore, covered SEMS with longer stent patency and fewer migrations are required. This study aimed to assess the clinical performance of a novel, fully covered SEMS for unresectable MDBO. Methods: This was a multicenter single-arm prospective study. The primary outcome was a non-obstruction rate at 6 months. The secondary outcomes were overall survival (OS), recurrent biliary obstruction (RBO), time to RBO (TRBO), technical and clinical success, and adverse events. Results: A total of 73 patients were enrolled in this study. The non-obstruction rate at 6 months was 61%. The median OS and TRBO were 233 and 216 days, respectively. The technical and clinical success rates were 100% and 97%, respectively. Furthermore, the rate of occurrence of RBO and adverse events was 49% and 21%, respectively. The length of bile duct stenosis (<2.2 cm) was the only significant risk factor for stent migration. Conclusions The non-obstruction rate of a novel fully covered SEMS for MDBO is comparable to that reported earlier but shorter than expected. Short bile duct stenosis is a significant risk factor for stent migration.

Objective: The purposes of this trial were to demonstrate the feasibility and effectiveness of the hybrid of intracavitary and interstitial brachytherapy (HBT) for locally advanced cervical cancer patients in the phase I/II prospective clinical trial. Methods: Patients with FIGO stage IB2-IVA uterine cervical cancer pretreatment width of which was ≥5 cm measured by magnetic resonance imaging were eligible for this clinical trial. The protocol therapy included 30–30.6 Gy in 15–17 fractions of whole pelvic radiotherapy concurrent with weekly CDDP, followed by 24 Gy in 4 fractions of HBT and pelvic radiotherapy with a central shield up to 50–50.4 Gy in 25–28 fractions. The primary endpoint of phase II part was 2-year pelvic progression-free survival (PPFS) rate higher than historical control of 64%. Results: Between October 2015 and October 2019, 73 patients were enrolled in the initial registration and 52 patients proceeded to the secondary registration. With the median follow-up period of 37.3 months (range, 13.9–52.9 months), the 2- PPFS was 80.7% (90% confidence interval [CI]=69.7%–88%). Because the lower range of 90% CI of 2-year PPFS was 69.7%, which was higher than the historical control ICBT data of 64%, therefore, the primary endpoint of this study was met. Conclusion The effectiveness of HBT were demonstrated by a prospective clinical study. Because the dose goal determined in the protocol was lower than 85 Gy, there is room in improvement for local control. A higher dose might have been needed for tumors with poor responses.