Inflammatory bowel disease (IBD), including Crohn's disease (CD) and ulcerative colitis (UC), is a chronic inflammatory disease of the gastrointestinal tract, with increasing prevalence worldwide. IBD Ahead is an international educational program that aims to explore questions commonly raised by clinicians about various areas of IBD care and to consolidate available published evidence and expert opinion into a consensus for the optimization of IBD management. Given differences in the epidemiology, clinical and genetic characteristics, management, and prognosis of IBD between patients in Japan and the rest of the world, this statement was formulated as the result of literature reviews and discussions among Japanese experts as part of the IBD Ahead program to consolidate statements of factors for disease prognosis in IBD. Evidence levels were assigned to summary statements in the following categories: disease progression in CD and UC; surgery, hospitalization, intestinal failure, and permanent stoma in CD; acute severe UC; colectomy in UC; and colorectal carcinoma and dysplasia in IBD. The goal is that this statement can aid in the optimization of the treatment strategy for Japanese patients with IBD and help identify high-risk patients that require early intervention, to provide a better long-term prognosis in these patients.
Asian Continental Ancestry Group ; Colectomy ; Colitis, Ulcerative ; Colorectal Neoplasms ; Consensus ; Crohn Disease ; Disease Management* ; Disease Progression ; Early Intervention (Education) ; Epidemiology ; Expert Testimony ; Gastrointestinal Tract ; Hospitalization ; Humans ; Inflammatory Bowel Diseases* ; Japan* ; Prevalence ; Prognosis

A 49-year-old female with ruptured left common iliac mycotic arterial aneurysm (Lt. CIAA) was brought to our hospital as an emergency case. In Japan, endovascular treatment is unsuitable for the treatment of mycotic aneurysms, but findings from a Swedish national database showed that there was no difference in the long-term prognosis lasting over 10 years. Therefore, we performed endovascular aortic repair (EVAR) and saved the patient's life. The diameter of the proxymal sealing zone was larger than that of the distal zone. We used the Gore Excluder leg, which was inverted and implanted to match the caliber. Percutaneous abscess drainage was also performed on postoperative day 7 when hemostasis was confirmed for early infection control. The patient was discharged at 8 weeks postoperatively. After discharge from the hospital, oral antibiotics were continued until 6 months after the surgery. Six months postoperatively, contrast-enhanced computed tomography showed that the abscesses have disappeared. Blood samples that were taken at one month after the completion of antibiotics showed no evidence of the recurrence of infection and a curative course was achieved.

When mitral valve dysfunction occurs in infants and mitral valve repair is difficult, mitral valve replacement (MVR) is required. However, commercially available prosthetic heart valves can be too large to implant in infants with a small annulus. In these children, the technique of supra-annular MVR is useful. Here we report two cases of supra-annular MVR, which were performed using an expanded polytetrafluoroethylene (ePTFE) graft as a skirt for a prosthetic valve. This method has been previously reported by Sung et al. The first case was a 16-month-old, 6.7-kg male infant who suffered from Shone' syndrome, mitral stenosis (MS) with a parachute mitral valve, coarctation of the aorta (CoA), and ventricular septal defect (VSD). MS progressed after CoA repair and VSD closure and a supra-annular MVR was performed. The second case was a 5-month-old, 4.9-kg female infant who suffered from polysplenia, intermediate atrioventricular septal defect (AVSD), and severe left atrioventricular valve regurgitation. AVSD repair was performed at the age of 3 months. However, valve stenosis and regurgitation gradually progressed postoperatively and consequently, a supra-annular MVR was performed. Postoperative prosthetic valve function was good in both cases. We believe that this method of performing supra-annular MVR is useful for infants with a small annulus.

Few reports have described assessment methods or exercise interventions in detail for patients isolated with COVID-19. Here, we report our experience of providing physical therapy to these patients based on motor assessment with consideration of infection control. This study involved 4 patients with COVID-19 who needed physical therapy due to a fall or frailty during isolation. The assessment method used was intended to minimize physical contact, to be easily performed, and to reflect muscle strength and balance. Based on the assessment, we created exercise programs and provided exercise intervention to the patients in cooperation with nurses. One patient was discharged early; the other 3 patients showed improved motor function and activities of daily living as a result of the intervention in the acute ward. However, they needed ongoing rehabilitation in the rehabilitation ward. No physical therapists showed signs of COVID-19 infection. Performing motor assessments and providing exercise intervention to these patients isolated with COVID-19 contributed to motor improvement and enabled us to promptly determine whether they needed ongoing rehabilitation.

Neonatal Marfan syndrome is the most severe form of Marfan syndrome usually showing critical cardio-respiratory symptoms from the neonatal period or early infancy. We report a boy with this syndrome who presented with heart failure at 3 months of age and was referred to our department at 6 months old after intense medical treatment. He had enophthalmos, funnel chest, arachnodactyly, and Steinberg's thumb sign, but had no family history of Marfan syndrome or other cardiac diseases. Left ventricular dilatation, severe mitral regurgitation and moderate tricuspid regurgitation were noted on echocardiography. Mitral valvuloplasty and tricuspid annuloplasty were performed, and the regurgitation improved to trivial and mild level, respectively. However, rapid exacerbation of mitral regurgitation occurred, and the patient fell into circulatory collapse which needed circulatory support with extracorporeal membrane oxygenator (ECMO) on 18th postoperative day. In the emergency operation, the previous surgical procedures on the mitral valve were intact and we thought that rapid progression of the mitral annular dilatation and valve expansion to be the cause of exacerbation. Mitral valve replacement (Regent® 21 mm aortic) was performed, and the cardiac function improved, but ECMO was still needed because of the depressed respiratory function. Furthermore, tricuspid regurgitation due to annular dilatation and valve expansion was aggravated rapidly which needed tricuspid valve replacement (ATS® 20 mm mitral) 9 days after the mitral valve replacement. ECMO was ceased on the 37th day and the patient was extubated on 71st day. He was discharged from the hospital 5 months after the first operation. One year has passed after discharge, and he is doing well with anticoagulation. In the treatment of neonatal Marfan syndrome, surgical procedure for valve repair is still controversial and it should be remembered that rapid exacerbation of the atrioventricular valve can occur even after satisfactory valve repair and there should be no hesitation regarding surgical intervention when needed.

A 47-year-old female was admitted to our hospital for management of dyspnea. She had undergone surgery for an atrial septal defect (ASD) at the age of 17. Computed tomography revealed left isomerism, inferior vena cava interruption with azygos continuation and a residual ASD. Intra-operative findings showed that the residual ASD was positioned across the orifice of the hepatic vein. The previous suture line could be identified in the partially-closed atrial septum above the residual defect. Re-closure was performed without difficulties, and the patient's condition was good at discharge. Closure of ASD is a simple and basic procedure in cardiac surgery but care must be taken not to leave a shunt at the lower part of the defect, especially in cases with ASD defects in the lower margin.