Role of video capsule endoscopy in patients with constitutional mismatch repair deficiency (CMMRD) syndrome: report from the International CMMRD Consortium.

BACKGROUND AND STUDY AIMS: Constitutional mismatch repair deficiency (CMMRD) syndrome, also known as biallelic mismatch repair deficiency (BMMRD) syndrome is a rare autosomal-recessive genetic disorder that has a high mortality due to malignancy in childhood and early adulthood. The small bowel phenotype in CMMRD is not well described and surveillance protocols for small bowel cancer have not been well established. This study was conducted to evaluate the usefulness and clinical impact of video capsule endoscopy (VCE) for small bowel surveillance. PATIENTS AND METHODS: We retrospectively reviewed the prospectively maintained International CMMRD Consortium database. Treating physicians were contacted and VCE report data were extracted using a standardized template. RESULTS: Among 58 patients included in the database, 38 VCE reports were collected from 17 patients. Polypoid lesions were first detected on VCE at a median age of 14 years (range: 4 - 17). Of these, 39 % in 7 patients (15/38) showed large polypoid lesions (> 10 mm) or multiple polyps that prompted further investigations. Consequently, three patients were diagnosed with small bowel neoplasia including one patient with adenocarcinoma. Small bowel neoplasia and/or cancer were confirmed histologically in 35 % of the patients (6/17) who had capsule surveillance and the lesions in half of these patients were initially visualized on VCE. Multiple polyps were identified on eight VCEs that were completed on three patients. Ten VCEs (28 %) were incomplete due to slow bowel transit; none required capsule removal. CONCLUSIONS: Small bowel surveillance in patients with CMMRD should be initiated early in life. VCE has the potential to detect polyps; however, small bowel neoplasias are often proximal and can be missed, emphasizing the importance of concurrent surveillance with other modalities. MEETING PRESENTATIONS: Digestive Disease Week 2017 and World Congress of Pediatric Gastroenterology, Hepatology and Nutrition 2016.

First results of electron temperature measurements by the use of multi-pass Thomson scattering system in GAMMA 10.

A multi-pass Thomson scattering (TS) has the advantage of enhancing scattered signals. We constructed a multi-pass TS system for a polarisation-based system and an image relaying system modelled on the GAMMA 10 TS system. We undertook Raman scattering experiments both for the multi-pass setting and for checking the optical components. Moreover, we applied the system to the electron temperature measurements in the GAMMA 10 plasma for the first time. The integrated scattering signal was magnified by approximately three times by using the multi-pass TS system with four passes. The electron temperature measurement accuracy is improved by using this multi-pass system.

Changes in influenza vaccination rates among healthcare workers following a pandemic influenza year at a Japanese tertiary care centre.

BACKGROUND: Although influenza vaccination, which is a key part of a control plan to prevent nosocomial influenza transmission, is recommended for all healthcare workers (HCWs), the achievement of a high influenza vaccination rate among HCWs is a challenge. In Japan, there are limited data on influenza vaccination rates among HCWs. Moreover, the effect of pandemic influenza on influenza vaccination rates among HCWs following a pandemic year remains unclear. AIM: To determine influenza vaccination rates among HCWs at a tertiary medical centre in Japan, and to evaluate the need for further interventions to enhance the vaccination rate among HCWs. METHODS: The 2005-2010 influenza vaccination rates among HCWs at a 550-bed, tertiary care centre in Sapporo, Japan were reviewed retrospectively using the hospital's occupational health service database. FINDINGS: There was a gradual increase in the seasonal influenza vaccination rate in this population from 2005 to 2010, and a high vaccination rate among HCWs during the 2009-2010 pandemic H1N1 influenza season. However, the seasonal influenza vaccination rate in 2010-2011 was significantly lower than that for the pandemic vaccine in 2009-2010, with doctors having the lowest vaccination rate among all HCWs. CONCLUSION: Pandemic influenza may not have a sustained effect on the uptake of influenza vaccination in subsequent years. Thus, vaccination rates among HCWs after a pandemic year still need to be monitored, and the implementation of interventions such as a mandatory vaccination programme should be considered to maintain consistently adequate vaccination rates.

Variability of parvovirus B19 genotype 2 in plasma products with different compositions in the inactivation sensitivity by liquid-heating.

BACKGROUND AND OBJECTIVES: Our previous report showed that parvovirus B19 genotype 1 in different solutions derived from plasma preparations showed different heat-sensitivity patterns during liquid-heating. In this study, we similarly examined B19 genotype 2. MATERIALS AND METHODS: Two plasma samples one containing B19 genotype 1 and the other genotype 2 DNA were used. Four process samples collected immediately before the heat treatment step in the manufacture of albumin, immunoglobulin, haptoglobin and antithrombin preparations were spiked with B19 and subsequently treated at 60°C for 10 h. A low pH immunoglobulin solution was also spiked with B19 and treated at room temperature for 14 days. Infectivity was then measured. RESULTS: B19 genotype 2, similar to genotype 1, showed three patterns of inactivation: (i) a rapid inactivation in the albumin and immunoglobulin preparations, (ii) a slow inactivation in the haptoglobin preparation and (iii) only limited inactivation in the antithrombin preparation. Its sensitivity in the low pH immunoglobulin solutions also resembled that of genotype 1. CONCLUSION: Both genotypes 1 and 2 of B19 varied in sensitivity to liquid-heating and low pH among different plasma preparations.

An Asian variant of intravascular lymphoma: unique clinical and pathological manifestation in the gallbladder.

We here present a rare case of intravascular lymphoma (IVL) in a Japanese man. 4 months after cholecystectomy due to cholecystitis, a diagnosis of intravascular lymphoma (IVL) was strongly suspected. Lymphoma cells were diffusely observed in the bone marrow parenchyma, but were absent in the vascular spaces. The patient died of respiratory failure and at autopsy a small number of lymphoma cells in the extravascular parenchyma of the adrenal gland and bone marrow were seen. Serial sections of the surgically resected gallbladder retrospectively confirmed the diagnosis of IVL. In addition, congestion and edema were observed in the connective tissue layer. It is possible that edema or ischemia in the gallbladder wall or at other anatomic sites due to the circulation disturbance induced by the intravascular obstruction of lymphoma cells may have caused the initial symptoms. In conclusion, clinicians and pathologists should keep in mind that the gallbladder may be initially involved in IVL.

Bridging necrosis and reticulin bridging fibrosis induced by intrahepatic involvement of acute biphenotypic leukemia.

A 47-year-old Japanese woman was diagnosed as having acute biphenotypic leukemia with association of t(9;22)(q34;q11). Cholestatic liver dysfunction arose, and she died of cachexia and intracranial hemorrhage. Autopsy showed unusual hepatic fibrosis. In the liver, bridging infiltration, bridging necrosis and bridging fibrosis by leukemic cells were seen. It seemed that the degree of fibrosis was associated with the number of aggregates of infiltrating leukemic cells. The fibrotic foci were predominantly composed of reticulin and collagen fibers, and distortion of the lobules was observed. Immunohistochemically, dense bundles of alpha-smooth muscle actin (ASMA)-positive stromal cells, namely activated hepatic stellate cells (HSCs), were observed in the immature fibrotic foci as well as along the sinusoids densely infiltrated by leukemic cells. No cells positive for TGF-beta1 or PDGF-BB were identified. In conclusion, extensive intrahepatic involvement by neoplastic cells in adult acute biphenotypic leukemia may cause the unusual "disorganized" hepatic fibrosis.

[Mitral valve repair of valve insuffficiency with broad and complex lesions due to infective endocarditis; report of a case].

Surgical intervention is necessary for the treatment of infective endocarditis, although antibiotic therapy has been shown to be effective for treatment of this disorder. Mitral valve infective endocarditis frequently presents with broad and complex lesions, and thus a variety of valve repair is needed. A 40-year-old woman with mitral valve insufficiency due to infective endocarditis underwent mitral valve repair. During the operation, torn chordae, aneurysm with perforation of the anterior leaflet, and torn chordae of the posterior leaflet were found. The chordae of the anterior leaflet were reconstructed and the aneurysm was excised, and autopericardial patch repair was performed. Then, resection and suturing of the prolapsing lesion of posterior leaflet were performed. Mitral valve repair preserves the left ventricular apparatus and function. Therefore, mitral repair results in better prognosis than valve replacement. The repair of the mitral valve should be attempted for the treatment of valve insufficiency due to infective endocarditis.

[Infarct exclusion for postinfarction left ventricular free wall rupture with severe congestive heart failure].

A 70-year-old man was transferred to our hospital with severe congestive heart failure and ventricular arrhythmia due to acute myocardial infarction. He had experienced chest pain 3 weeks previously and was admitted to another hospital for dyspnea, where he required assist ventilation, 1 week prior to the transfer. An echocardiogram revealed a broad anteroseptal infarction and very poor left ventricular function with an ejection fraction (EF) of 22%. He remained in a severe congestive heart failure condition despite a full administration of catecholamines. Coronary angiogram findings revealed an occlusion of the proximal left anterior descending coronary artery and 1 week later severe hypotension was suddenly presented. An echocardiogram showed pericardial effusion with signs of cardiac tamponade. A pericardiocentesis was performed and hemodynamic improvement was obtained for a short time, after which the patient underwent urgent open heart surgery. During the operation, exclusion of the anteroseptal akinetic area using an oval patch was performed under a cardiopulmonary bypass and ventricular fibrillation. Severe cardiac failure remained postoperatively and the patient could not be weaned from cardiopulmonary bypass, therefore, we implanted a percutaneous cardiopulmonary support (PCPS) and started intraaortic balloon pumping (IABP). The patient was weaned from PCPS at 26 days after surgery and from IABP at 30 days. Following hospital release, he has continued to do well without heart failure for 39 months after the operation.

[Critical hypertrophic obstructive cardiomyopathy patient with improved hemodynamics following mitral valve replacement: report of a case].

A 75-year-old man with dyspnea was admitted to our hospital in critical condition. Catheterization showed normal coronary arteries and good left ventricular function. Transesophageal echocardiography showed left ventricular hypertrophy and severe mitral regurgitation. We decided to perform mitral valve replacement because the patient was in critical condition and it was necessary to complete the operation smoothly. During the operation, we could see the dilated mitral valve annulus and hypertrophic mitral valve, which was restricted. The patient's hemodynamics showed improvement after mitral valve replacement with a mechanical prosthesis, and he was discharged on postoperative day 21. In conclusion, mitral valve replacement is a beneficial method for the treatment of patients with critical hypertrophic obstructive cardiomyopathy.

[Mitral valve plasty and coronary artery bypass grafting under beating heart cardiopulmonary bypass for severe left ventricular dysfunction; report of a case].

A 54-year-old man with ischemic mitral regurgitation and severe heart failure due to broad myocardial infarction successfully underwent mitral valve plasty and coronary artery bypass grafting under beating heart. He had an old anterior myocardial infarction and was admitted to our hospital with acute inferior myocardial infarction. Two weeks later, the cathetelization revealed moderate mitral regurgitation and triple vessel coronary artery disease. We selected antegrade continuous blood perfusion for myocardial protection on operation. He recovered uneventfully and discharged on postoperative day 31. We could perform this procedure safety and satisfactorily, we could this procedure for heart valve operation with other complications.

[The efficiency of nifekalant hydrochloride for the prevention of ventricular tachycardia during cardiac surgery].

Many cases have been successfully treated by us with experimented nifekalant hydrocholoride to prevent ventricular tachycardia (VT) during cardiac surgery. The 13 patients who underwent cardiac surgery at our hospital from 1999 to 2002 were retroactively given nifekalant hydrocholoride against VT. Lidocaine hydrochloride was not effective for VT, and it was difficult for 3 patients to be weaned for cardio-pulmonary bypass, while 6 patients needed aortic balloon pumping or percutaneous cardio-pulmonary support. Nifekalant hydrochloride suppressed VT induction in 9 patients (69.2%). Blood pressure and heart rate did not change, but QTc intervals were significantly increased with nifekalant hydrochloride (p < 0.005). Proarrhythmic events (Torsades de pointes) occurred in 2 patients, but none of the cases showed drug-induced worsening of cardiac function. Nifekalant hydrochloride is a class III antiarrhythmic drug that has been found to be effective against VT and ventricular fibrillation. While class I antiarrhythmic drugs are usually ineffective and induce severe heart failure, nifekalant hydrochloride can be effective.

[Apico-aortic conduit for severe aortic stenosis in elderly].

We described a case of an 84-year-old woman with end stage aortic stenosis in whom favorable results were obtained after constructing apico-aortic conduit bypass. The patient admitted due to complete atrio ventricular(AV) block and respiratory insufficiency. Although a permanent pacemaker was implanted, heart failure did not improve. Echocardiograms showed small aortic annulus and severe calcific aortic stenosis with a measured trans valve gradient of 100 mmHg. Since cardiac failure deteriorated and she lapsed into hypotension and acute renal failure, an emergency operation was performed. The left lateral thoracotomy was performed with cardiopulmonary bypass. A bioprosthetic valved conduit was anastmosed to the left ventricle apex. Another graft was anastomosed to the discending thoracic aorta. The operation was completed by anastomosing the tailored ends of the grafts together. Although the patient required hemodialysis for 2 weeks after operation, she was discharged on 57th day after operation without any complications. Apico-aortic conduit is considered to be an useful procedure for elderly patients who were not good candidates for conventional valve replacement.

[Usefulness of silicone blake drains after cardiac surgery].

In the present study, the drainage system consisting of a silicon blake drain and a portable suction reservoir (J-vac system) was applied to the patients who received cardiac surgery (SD group). Postoperative drainage volume, postoperative pericardial effusion volume, and postoperative length of hospital stay in SD group were compared with those in the patients who received cardiac surgery and were applied a conventional chloroethlene drain (CD group). There were no significant differences in postoperative drainage volume, postoperative pericardial effusion volume, or postoperative length of hospital stay between SD and CD groups. Therefore, its was thought that a silicon back drain could be safely used as a drainage system after heart surgery. Recently the development of less-invasive cardiac surgery made the early hospital discharge possible, and J-vac system might be very useful in view of its portability under such clinical settings.

[Clinical outcome of emergency off-pump coronary artery bypass grafting].

From January 2001 to January 2003, we performed 25 emergency off-pump coronary artery bypass grafting (CABG) procedures for patients with acute myocardial infarction (AMI) or unstable angina pectoris. During the same period, we also performed 2 emergency on-pump beating CABG procedures for patients with left main coronary trunk (LMT) shock syndrome. For the present study, we evaluated the operative results of the 25 cases of emergency or urgent off-pump CABG. The patients were divided into 3 groups, those with acute AMI with cardiogenic shock (group 1; n = 8), acute myocardial infarction without shock (group 2; n = 8), and unstable angina (group 3; n = 9). There were no differences between groups 1 and 2 with regard to age, number of diseased vessels, and preoperative use of an intraaortic balloon pump, however, patients in group 1 had a higher number of completely obstructed coronary arteries. Patients in groups 1 and 2 underwent off-pump CABG within 3.5 hours after a coronary angiography or coronary intervention procedure, while those in group 3 underwent off-pump CABG within 2 days of coronary angiography. The mean number of grafts per patient was 1.8, 2.1, and 2.3 in groups 1, 2, and 3, respectively. One group 1 patient with an LMT lesion was transferred to on-pump beating CABG because of hemodynamic instability. The 30-day mortality rate was 38% (3 of 8) in group 1, whereas it was 0% in groups 2 and 3. Intubation time, ICU stay, and postoperative stay were similar among the 3 groups. An early angiographic study was undertaken in all surviving patients and the results demonstrated patency in all of the examined grafts. Although our results are limited, emergency off-pump CABG was found to be safe and feasible for AMI without cardiogenic shock or unstable myocardial ischemia. However, the outcome of this procedure for patients with preoperative cardiogenic shock was not satisfactory, therefore, a combination therapy of appropriate mechanical circulatory support, prior revascularization by catheter intervention, and emergency surgical revascularization are considered to improve survival of those patients.

[Economic evaluation of off-pump coronary artery bypass grafting; compared to conventional coronary artery bypass grafting].

This study was undertaken to compare economic outcomes of off-pump coronary artery bypass grafting (off-pump CABG: OPCAB) and conventional CABG (CCABG). We reviewed the medical records of 5 patients with ischemic coronary disease (IHD) who underwent OPCAB and 5 patients with IHD who underwent CCABG. A hospital income which was based on a current Japanese health insurance system was indicated. There were significant differences in total cost (OPCAB = 208,200 +/- 7,383 yen versus CCABG = 324,300 +/- 10,290 yen, 35.8% decreased), costs of medical materials and transfusions (OPCAB = 14,760 +/- 3,270 yen versus CCABG = 87,540 +/- 3,326, 81.3% decreased) and charges of surgery and anesthesia (OPCAB = 130,400 +/- 1,853 yen versus CCABG = 147,650 +/- 4,167 yen, 12% decreased) OPCAB markedly reduces the cost of hospitalization. But charges of surgery and anesthesia of OPCAB seems to be valued properly in a current Japanese health insurance system.

[Aortic valve replacement with a freestyle stentless valve using the modified subcoronary technique and hemiarch replacement for bicuspid aortic valve and ascending aortic aneurysma; report of a case].

An association between bicuspid aortic valve disease and ascending aortic aneurysma has long been recognized. Root replacement with a composite valve graft for such disease is a well-established technique. But it may involve serious technical difficulties, and may be a more time-consuming procedure than separate valve replacement and graft replacement. We performed an aortic valve replacement with Freestyle stentless valve using the modified subcoronary technique and hemiarch replacement for a 72-year-old man with severe aortic stenosis and ascending aortic aneurysma. Angiographic studies after surgery showed no residual aortic regurgitation (AR) and no deformity of aorta. This technique is an acceptable option for an aortic disease and ascending aneurysma in elderly patients.

[Graft holding system; a new device for coronary artery bypass grafting].

A new graft holding system for coronary artery bypass grafting was described. This system was an application of a memo-clip, which was composed of a flexible arm and 2 paper clips at both ends. A graft was wrapped by a piece of sponge and pinched indirectly by one of the clips. The other clip was fixed at an edge of the sternal retractor. Regardless of the size of the graft or the amount of the surrounding tissue, the graft can be fixed securely at one's pleased position due to a flexible arm of the device. Intimal injury can be avoided referring to the gentle holding of a piece of sponge. It enables us to place precise stitches and to minimize handling, anastomosis time, and leakage.

Recurrent palmar dislocation of the distal radioulnar joint. A case report.

Recurrent palmar dislocation of the distal radioulnar joint is not a common injury. We report one case in a 73-year-old female. This injury was incorrectly diagnosed at the first presentation because there has been no distinct deformity at the wrist and extension-flexion was normal. The need for proper physical examination and accurate radiographic positioning is stressed. Distal diaphysis resection combined with distal radioulnar arthrodesis (modified Sauve-Kapandji procedure) was the preferred method of treatment in an old patient. Two years after the injury, the patient was asymptomatic.