Autoimmune enteropathy (AIE) is a rare disease, characterized by intractable diarrhea, villous atrophy of the small intestine, and the presence of circulating anti-enterocyte autoantibodies. Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome, and mutations in FOXP3, which is a master gene of regulatory T cells (Tregs), are major causes of AIE. Recent studies have demonstrated that mutations in other Treg-associated genes, such as CD25 and CTLA4, show an IPEX-like phenotype. We present the case of a 13-year-old girl with CTLA4 haploinsufficiency, suffering from recurrent immune thrombocytopenic purpura and intractable diarrhea. We detected an autoantibody to the AIE-related 75 kDa antigen (AIE-75), a hallmark of the IPEX syndrome, in her serum. She responded well to a medium dose of prednisolone and a controlled dose of 6-mercaptopurine (6-MP), even after the cessation of prednisolone administration. Serum levels of the soluble interleukin-2 receptor and immunoglobulin G (IgG) were useful in monitoring disease activity during 6-MP therapy. In conclusion, autoimmune-mediated mechanisms, similar to the IPEX syndrome, may be involved in the development of enteropathy in CTLA4 haploinsufficiency. Treatment with 6-MP and monitoring of disease activity using serum levels of soluble interleukin-2 receptor and IgG is suggested for such cases.

Retrograde type A aortic dissection (RTAD) following thoracic endovascular aortic repair (TEVAR) is a lethal complication. A 54-year-old woman with bovine aortic arch presented with dilatation of the descending aorta due to chronic type B aortic dissection. She underwent TEVAR in zone 2 for closure of the entry site just below the origin of the left subclavian artery. On the day after TEAVR, she showed right hemiparesis, and was diagnosed with cerebral infarction on MRI and RTAD on CT. She underwent an emergent operation. The entry was at the proximal end of the bovine trunk, where the edge of the bare stent stuck out. We performed partial arch replacement with entry resection. Her postoperative course was uneventful. She was transferred to another hospital for rehabilitation 37 days after the surgery.

A 42 year-old woman with Marfan syndrome, who had replacement of the ascending aorta for acute aortic dissection several years ago, was found to have distal aortic arch aneurysm. The aneurysm had small entries at cervical arterial branches and large re-entry at the left external iliac artery. It was necessary to perform two-staged operation Bentall procedure with total arch replacement and abdominal aortic replacement with re-entry closure. It was usually performed with a primary entry closure for chronic aortic dissection, but massive invasion was expected. We performed catheter angiography for entry and re-entry, and decided to perform preceding re-entry closure. First, we underwent replacement of the abdominal aorta, and then successfully performed the Bentall procedure with total aortic arch replacement. The catheter angiography was useful for decision-making for medical treatment.

Background：A palliative care education program based on the Cancer Control Act, namely the Palliative care Emphasis Program on symptom management and Assessment for Continuous medical Education（PEACE）, has been run in Japan since 2008. Purpose：The aim of this study was to evaluate the usefulness of PEACE for clinicalpractice. Method：Focus group interviews were conducted with 12 physicians who attended the program and were chosen by theoretical sampling. Two investigators independently performed thematic content analysis. Results：Qualitative analysis identified five main categories of usefulness of the program：（1）acquiring knowledge of palliative care；（2）acquiring skills to provide palliative care；（3）mastering an attitude of focusing on suffering；（4）acquiring knowledge about home palliative care；and（5）standardizing knowledge of basic palliative care in the community. Three main categories for program improvement were identified, namely the content and structure of the program, as well as the method of running the program. Conclusion：PEACE may be useful in providing systematic education in basic palliative care through the acquisition of basic knowledge and skills, discussion of cases with colleagues, observing good communication by other physicians, and networking in the community.

A 74-year-old man had been taking warfarin for atrial fibrillation, but warfarin was discontinued due to upper gastrointestinal bleeding. One week later, left hemiplegia occurred, and cranial magnetic resonance imaging revealed multiple cerebral infarctions. Systemic examination revealed thrombi in both atria as well as duodenal cancer. Because all of the thrombi in both atria were larger than 30 mm in diameter, the risk of embolism or sudden death was assumed to be high. Although the use of cardiopulmonary bypass for cancer patients is controversial, bilateral atrial thrombectomy was performed 4 weeks after cerebral infarction onset because reasonable survival duration was expected with surgery for duodenal cancer after thrombectomy and further treatment. The timing of and indications for surgery in this case are discussed.

BACKGROUND/AIMS: Postoperative endoscopic recurrence (PER) occurs in nearly 80% of patients 1 year after ileocecal resection in patients with Crohn's disease (CD). Biological agents were more effective in reducing the rates of PER in comparison with conventional therapy, in prospective trials. The aim of this study was to compare the PER rates of biological versus conventional therapy after ileocecal resections in patients with CD in real-world practice. METHODS: The MULTIPER (Multicenter International Postoperative Endoscopic Recurrence) database is a retrospective analysis of PER rates in CD patients after ileocecal resection, from 7 referral centers in 3 different countries. All consecutive patients who underwent ileocecal resections between 2008 and 2012 and in whom colonoscopies had been performed up to 12 months after surgery, were included. Recurrence was defined as Rutgeerts' score > or =i2. The patients were allocated to either biological or conventional therapy after surgery, and PER rates were compared between the groups. RESULTS: Initially, 231 patients were evaluated, and 63 were excluded. Of the 168 patients in the database, 96 received anti-tumor necrosis factor agents and 72 were treated with conventional therapy after resection. The groups were comparable regarding age, gender, and perianal disease. There was longer disease duration, more previous resections, and more open surgical procedures in patients on biologicals postoperatively. PER was identified in 25/96 (26%) patients on biological therapy and in 24/72 (33.3%) patients on conventional therapy (P=0.310). CONCLUSIONS: In this retrospective observational analysis from an international database, no difference was observed between biological and conventional therapy in preventing PER after ileocecal resections in CD patients.
Biological Factors ; Biological Therapy* ; Colonoscopy ; Crohn Disease* ; Humans ; Necrosis ; Observational Study* ; Recurrence* ; Referral and Consultation ; Retrospective Studies ; Tumor Necrosis Factor-alpha

  A 86-year-old woman visited us, complaining about sharp abdominal pain she had very morning when she got up. Ultrasound and computer tomography scans of the abdomen revealed notable parietal hypertrophy and swelling of the gallbladder. The old woman was diagnosed with acute cholecystitis and immediately admitted to the hospital. Her condition did not improve on conservative management. Percutaneous transhepatic gallbladder drainage (PTGBD) was performed. The bile thus aspirated was bloody, which led us to suspect necrotizing cholecystitis. Emergency cholecystectomy was done. The abdominal operation found the gallbladder wandering with a torsion of 360 degrees around the gallbladder neck as the axis and the leakage of bile in the abdominal cavity from what could be presumed to be the area where the PTGBD was placed. After the torsion was corrected, the gallbladder was surgically removed. Neither gallstones nor tumors were found in it. There were signs of hemorrhagic necrosis in the mucus membrane. The patient made good progress after the operation and was discharged on the 15th hospital day. Torsion of the gallbladder is a comparatively rare entity and its symptoms are not always specific. Therefore, it defies preoperative diagnosis. In this paper, we report our experience with a case of this disease which presented characteristic radiographic images and discuss the treatment strategies including PTGBD.

An 84-year-old man, who had been given a diagnosis of advanced aortoventricular block 2 years previously, underwent implantation of a pacemaker (PM) through the left subclavian vein. However, 7 months later a skin ulcer developed at the implantation site, but without any evidence of bacterial infection. Therefore, a PTFE-covered PM battery was reimplanted at the same site. Three months later, the skin ulcer recurred and he received a third implantation in the right side. However, another skin ulcer with infection developed in the right side. He was then transferred to our hospital for another PM reimplantation. We covered the battery and the entire lead with PTFE, then placed the PM lead directly into the cardiac muscle, and implanted the PM battery below the rectal muscle under general anesthesia. A patch test 4 months later revealed a positive reaction to nickel and silicon. Finally, we diagnosed pacemaker dermatitis. The patient has remained free of skin ulcers for over 1 year.

There is disagreement regarding the indications of surgery for cases of severe aortic stenosis (AS) with a decrease in left ventricular ejection fraction (EF) and a low aortic pressure gradient (PG), since there is a high perioperative risk associated with this condition. Hence, we investigated the surgical outcome of AS cases with impaired left ventricular function. Our department performed 144 aortic valve replacements (AVRs) for cases of AS and AS-dominant mild regurgitation (ASr) between January 2000 and September 2005. Among these cases, 9 patients had an EF under 35%, and these patients were selected as subjects and compared with a control group with an EF of more than 35%. Patients with accompanying coronary artery diseases that required treatment were excluded to avoid confounding effects on cardiac function. The mean age of the 9 subjects (4 men and 5 women) was 67.8±10.8 years old, with a range from 53 to 80 years old, and the subjects had the following mean background data: EF, 34.4±0.5%; left ventricular end-diastolic dimension (LVDd), 57.3±5.8mm; left ventricular end-systolic dimension (LVDs), 49.3±5.7mm; interventricular septum thickness (IVSth), 11.9±1.9mm; and left ventricular posterior wall thickness (LVPWth), 11.1±2.6mm. Characteristics such as left ventricular dilatation and thinning of the left ventricle myocardium were noted in these data. The cases were classified as severe AS because the mean aortic valve area (AVA) was 0.58±0.2cm², but the peak aortic pressure gradient (peak PG) (65.2±32.7mmHg) in the 9 subjects was lower than that of the control group (97.0±65.2mmHg). All 9 subjects underwent aortic valve replacements (AVRs), with simultaneous mitral annuloplasty (MAP) in 3 cases, mitral valve replacement (MVR) in 1 case and performance of a Maze procedure in 1 case. No deaths occurred while the patients were in hospital. Postoperative complications included 2 cases of transient atrial fibrillation and 1 case of postoperative bleeding requiring rethoracotomy for hemostasis. The EF in the late postoperative period showed improvement in 8 cases and was unchanged in the remaining case; the mean postoperative EF was 56.9% for the 9 subjects. All cases were rated as improved based on the NYHA classification of cardiac performance, and the significant improvement in EF in 8 of the 9 cases suggests that surgery is safe and can improve prognosis for patients with advanced AS with myocardium thinning and decreased EF.

Magnetic resonance imaging (MRI) for diagnostic evaluation of the breast was performed in 61 patients who visted the Breast Clinic of our hospital and were suspected to have malignant tumors by physical examination and mammography between January and December 1999. In 58 patients undergoing histological diagnostic tests (8 with benignancy and 50 with breast cancer), we compared the imaging characteristics and the time-signal intensity curves acquired by dynamic imaging between benign and malignant lesions, and evaluated the usefulness of analyzing enhancement patterns on contrast MRI. Contrast MRI revealed strong tumor enhancement in all patients; the mean time required for the signal intensity to reach a peak was about 7 min in patients with benign tumors and about 2 min in those with breast cancer. Peripheral ring enhancement was observed in 40 of the 50 patients with breast cancer (80.0%), while such enhancement was not noted in any of the patients with benign tumors.
Although diagnosis of breast disease by imaging has primarily relied on mammography and ultrasonography, the pattern of contrast enhancement on dynamic MRI also appears to be useful for determining the treatment method of breast tumors.