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.

Behçet’s disease (BD) is a multisystem inflammatory disease of unknown origin. Rarely, BD occurs together with myelodysplastic syndrome (MDS). Interestingly, it is speculated that these are not simple coexistence but that the etiology of intestinal BD is at least partly derived from MDS itself. Furthermore, there is a relationship between MDS in patients with intestinal BD and trisomy 8. Immunosuppressive agents alone are insufficient to control MDS-associated BD, and many of these patients die of infection or hemorrhage. Surgery is considered for intestinal BD patients who are unresponsive to medical treatment or those with bowel complications such as perforation or persistent bleeding. We report a case of intestinal BD associated with MDS and trisomy 8. The patient was unresponsive to oral steroids and immunosuppressive treatment; the patient improved by surgical repair of a bowel perforation. Five years after the surgery, the patient is free of recurrence and not on medication. Our experience suggests that surgery may provide an effective therapeutic option for the treatment of MDS-related BD.

Objective: Both infected subdural hematoma (ISH) and Edwardsiella tarda infections are rare in humans. E. tarda is a motile, facultative anaerobic, gram-negative rod bacterium, which is isolated from fresh or brackish water, but not usually from humans. Extra-intestinal E. tarda infections are rare and might cause severe clinical symptoms. However, ISH caused by E. tarda has not been reported previously. We report the first case of ISH due to E. tarda.Patient: A 76-year-old man was admitted to our hospital with a headache, loss of appetite, and nausea. Computed tomography revealed bilateral subdural hematoma.Results: We performed burr hole drainage. A hematoma with pus was found on the left side and chronic hematoma was found on the right side. Consequently, we diagnosed him with ISH on the left side and chronic subdural hematoma on the right side. E. tarda was detected in a culture from the hematoma with pus on the left side. As postoperative antibiotic therapy, we administered ceftriaxone and metronidazole for 47 days. The patient was discharged with no residual neurological deficit.Conclusion: Our case implied that favorable outcomes can be obtained by drainage and appropriate antibiotic therapy for ISH caused by E. tarda.

Our hospital is responsible for acute care as part of a regional-based integral medical care center and uses Diagnosis Procedure Combination (DPC). We attempt to increase patient discharge rates within period II in the DPC / Per-Diem Payment System (DPC/PDPS). However, hospitalization of cerebrovascular disease patients tends to be prolonged by reduced activities of daily living caused by disease and patient background. Thus, we surveyed factors related to extended hospitalization of patients with cerebral infarction, who are among the largest number of patients with cerebrovascular disease. In this survey, we retrospectively analyzed 109 hospitalized patients who underwent medical treatment from May 2016 to March 2017. Patients were divided into two groups (within period II, and period III and above). We performed univariate and multivariate analysis on factors contributing to prolonged hospitalization. Multivariate logistic regression analysis revealed that place of residence (pre- and posthospitalization) affected the hospitalization period. Among them, patients who were admitted from their homes and discharged to facilities other than their homes had the longest hospital stay. Interestingly, univariate analysis showed a significant difference (P<0.001) between the two groups in the National Institute of Health Stroke Scale (NIHSS) score on admission, while there was no significant difference (P = 0.65) with multivariate analysis. It implied that NIHSS score affected the decision about recuperation location after discharge, but not the hospitalization period. Based on our study, we identified key issues that need to be addressed: 1) the time to determine appropriate medical care after the acute phase, and 2) the waiting period for proper transfer. Therefore, to promptly discharge patients from hospital, we believe it is important to provide early intervention by ward nurses at the time of hospitalization, and to review our ongoing efforts to strengthen the system and enhance collaboration within our medical center.

Post-polio syndrome (PPS) is the term used to describe the symptoms that may develop many years after acute paralytic poliomyelitis( APP). In the case of PPS, the symptoms and signs include progressive muscle wasting and weakness, limb pain, and/or fatigue, occurring one or more decades after maximal recovery from APP. An overuse of enlarged motor units is suspected to cause the deterioration of some nerve terminals or the loss of the motor units themselves. This could in turn induce PPS symptoms such as new muscle weakness and atrophy. Electromyography (EMG) is often a strong tool to diagnose and evaluate PPS. Some studies have shown that mild to moderate intensity muscular strengthening has a positive effect in patients affected by PPS. Rehabilitation for PPS patients should utilize a multiprofessional and multidisciplinary approach. PPS patients should be advised to avoid both inactivity and overuse of the affected muscles. Finally, patient evaluation is often required to access the need of orthoses and assistive devices.

The routine tuberculosis (TB) surveillance system in Cambodia has been strengthened under the National TB Programme (NTP). This paper provides an overview of the TB surveillance data for Cambodia at the national level for the period 2000 to 2013 and at the subnational level for 2013. The proportion of the total population that were screened for TB rose from 0.4% in 2001 to 1.1% in 2013, while the smear-positivity rate decreased from 28.9% to 8.1% in the same period. The total number of notified TB cases increased steadily from 2000; this has stabilized in recent years with 39 055 cases notified in 2013. The proportion of all TB cases that were smear-positive decreased from 78% in 2000 to 36% in 2013. Case notification rates (CNRs) for all forms of TB and new smear-positive TB in 2013 were 261 and 94 per 100 000 population, respectively. Higher CNRs were found in the north-west and south-east parts of the country and were higher for males especially in older age groups. The increase in TB screening, decline in the smear-positive rate and decline in notified smear-positive TB cases likely reflect a long-term positive impact of the NTP. A negative correlation between the proportion of the population screened and the smear-positivity rate at the subnational level helped identify where to find undiagnosed cases. Subnational differences in case notification of the elderly and in children provide more specific targets for case-finding and further encourage strategic resource allocation.

A 20-year-old male was referred to our hospital to undergo operative treatment due to aortic valve insufficiency which had gradually worsened. The patient's chief complaint was a loss of breath upon effort which had progressively worsened after undergoing aortic valve plasty (AVP) for aortic valve insufficiency with infective endocarditis at another institution. AVP by the cusp extension method had been performed because of the patient's youth and there had been no change in the morbid state, except for the presence of a non-coronary cusp. In addition, the aortic valve insufficiency was controlable and postoperative course was also excellent. The cusp extension method was therefore considered to be an appropriate procedure for this case since it would allow the patient to return it to a state with a more normal heart, since the valve organization after this procedure would be able to reach a maximum level.

Staged arterial switch operation without homologous blood transfusion was successfully performed in 5 patients weighing 4.1-11.0kg (double outlet right ventricle: 2 cases, transposition of great arteries: 3 cases). The postoperative hemodynamics and respiratory status were uneventful in all patients (initial central venous pressure after ICU admission: 9.0-14.5cmH₂O, mean 12.5cmH₂O, duration of intubation: 3.5-18.0h, mean 7.8h). Autologous blood donation immediately after induction of anesthesia and minimization of bypass circuit were effective methods for open heart surgery without homologous blood transfusion, particularly in staged arterial switch operation requiring prolonged cardiopulmonary bypass.