A successful surgical correction of an incomplete endocardial cushion defect (ECD) with an ostium primum defect in a 63-year-old man is reported. Incomplete ECD with ostium primum defect often causes severe heart failure in infancy. Reports of its surgical correction in elderly patients are few. The patient had upper abdominal discomfort and grade 1 mitral valve regurgitation. The preoperative diagnosis was complete ECD (Rastelli type A) because we misdiagnosed a leakage from a cleft between the left superior leaflet and the left inferior leaflet for a flow through a ventricular septal defect when we analyzed a preoperative left ventriculography. The importance of not misdiagnosing the leakage and echocardiography in preoperative diagnosis of ECD was therefore realized. The operative procedure involved patch closure of the ostium primum defect and mitral valve annuloplasty by Kay's procedure and the mitral valve regurgitation completely disappeared. The postoperative course was uneventful. The upper abdominal discomfort and cardiomegaly improved. If there is no severe dysfunction of other organs, surgical correction of incomplete ECD should be recommended even for elderly patients.

A 46-year-old man who developed fever and general fatigue was referred to our hospital with suspicion of infective endocarditis. A ventricular septal defect had been previously diagnosed. Transthoracic echocardiography revealed vegetation on the aortic, mitral, and pulmonary valves, and each valve had significant regurgitation. An emergency operation was performed because of congestive heart failure. The aortic and mitral valves were replaced with mechanical valves. The pulmonary valve was repaired ; the anterior leaflet was resected and replaced by glutaraldehyde-treated autologous pericardium. The patient's postoperative course was uneventful. Recurrence of infection was not observed for 3 years after the operation. Triple-valve endocarditis, especially that involving a combination of the aortic, mitral, and pulmonary valves, is rare. Involvement of multiple valves on both sides of the heart may be attributed to a congenital intracardiac shunt. Early surgical intervention may be useful to control infection and heart failure, as in the present case.

75-year-old man had the right lower lobe resected because of pulmonary adenocarcinoma (stage IIB) and received 4 courses of postoperative chemotherapy 4 years earlier. Thereafter, he continued to complain of cough, sputum, and progressive exertional breathlessness. The preoperative chest CT showed ground glass opacity (GGO) at the bottom of both lung fields, and over time the GGO changed to honeycombing with traction bronchiectasis. He was administered prednisolone, clarithromycin, and pirfenidone but with little improvement. He exhibited hypoxemia (PaO₂ 56 mmHg) and was admitted. An interview revealed that he had worked in the poultry farming business for 45 years having had contact with and breeding 3,000 game fowl at the time of hospitalization. We suspected bird-related hypersensitivity pneumonitis. Results of the reaction to pigeon dropping extracts (PDE) were high, with PDE IgG 0.697 and PDE IgA 0.445. He was diagnosed with chronic bird-related hypersensitivity pneumonitis. Although the chest CT appearance was difficult to distinguish from that of idiopathic interstitial pneumonia, the test for PDE and the interview were useful for reaching a diagnosis.

Side to side difference in tennis players' mid-radius and cross-sectional study on mid-tibia of jumpers and sedentary controls suggest that the improvement of mechanical properties of cortical bone in response to long-term exercise is related to geometric adaptation and not to volumetric bone mineral density. In the present study, geometric and mechanical properties of right tibia were estimated along 64 directions centering center of gravity of the bone on cross-sectional peripheral quantitative computed tomography (pQCT) images. The tibias of 17 jumpers (7 females, 10 males) and 15 controls (8 females, 7males), aged 18-23, were scanned at mid site using pQCT. Periosteal and endocortical radius were larger, cortical thickness was thicker, and mechanical properties (moment of inertia of area and strength strain index) were greater in jumpers compared to those of controls. The differences in cortical thickness between the two groups were dependent on direction of measurement. Defined a direction from tibia's center of gravity to fibula's as 0°, difference in the cortical thickness between jumpers and controls was the greatest at around 240°. Along this direction, differences in mechanical properties were also the most significant, suggesting that the site-specific adaptation of bone to long-term exercise is due to geographical relation of bone to muscle.

PURPOSE: The aim of this study was to investigate the utility of echo intensity and contrast enhancement in the differential diagnosis between intraductal papillary mucinous neoplasm with an associated invasive carcinoma (IPMN-IC) and pancreatic ductal adenocarcinoma (PDAC) on ultrasonography. METHODS: This study included eight and 37 patients who had pathologically confirmed IPMN-IC and PDAC, respectively, and were enrolled for a comparative analysis of the sonographic features of the tumors. In the quantitative echo intensity evaluation, the two groups were compared with respect to the difference between the tumor intensity and the pancreatic intensity (TI-PI) and between the tumor intensity and the vascular intensity (TI-VI). In the quantitative contrast enhancement evaluation, the increase in echo intensity (ΔTI) and increase in echo intensity per unit of time (slope) were compared between the groups. The echo intensity and contrast enhancement were also compared between the two groups in patients with T3-T4 disease. In addition, the correlations of the histological type, tumor size, stromal type, and T factor with echogenicity and contrast enhancement were analyzed. RESULTS: IPMN-IC had significantly greater echo intensity and contrast enhancement than PDAC (TI-PI, P=0.004; TI-VI, P=0.001; ΔTI, P=0.012; slope, P=0.002). In T3-T4 disease, IPMN-IC also showed greater echo intensity and faster enhancement than PDAC. Echo intensity and contrast enhancement were correlated with histological type (TI-PI, P=0.003; TI-VI, P<0.001; ΔTI, P=0.007; slope, P<0.001). CONCLUSION: IPMN-IC and PDAC can be differentiated by the quantitative evaluation of echo intensity and contrast enhancement.
Adenocarcinoma* ; Carcinoma, Pancreatic Ductal ; Diagnosis, Differential* ; Evaluation Studies as Topic ; Humans ; Mucins* ; Pancreatic Ducts* ; Ultrasonography*

The concept of pervasive developmental disorders (PDD) and autism spectrum disorders (ASD) closely resemble each other. Both ICD-10 and DSM-IV use the term PDD. The authors surveyed the perception of PDD/ASD and attitudes toward terminology. The subjects of this study were 205 medical/social-welfare professionals working in fields relating to developmental disorders. Questionnaires were mailed to site investigators at the collaborating institutes. With regard to what the scope of ASD and PDD encompasses, the answers were almost equally divided among three views: ASD and PDD are the same, PDD is wider in scope and ASD is wider. The terms PDD and autism were used in slightly different ways depended upon the situation. Our results demonstrate that the parameters of PDD and ASD are unclear and that the terms related to PDD/ASD are often used differently. Further studies are required to develop more clear and reliable diagnostic criteria for PDD.
Academies and Institutes ; Asperger Syndrome ; Autistic Disorder ; Child ; Autism Spectrum Disorder ; Diagnostic and Statistical Manual of Mental Disorders ; Humans ; International Classification of Diseases ; Postal Service ; Surveys and Questionnaires ; Research Personnel

A man in his fifties was diagnosed with retrograde type A acute aortic dissection. He underwent total arch replacement using the Gelweave Lupiae graft and staged thoracic endovascular aortic repair. Five days after discharge from the hospital, he was referred to our institution because of severe hemolytic anemia. Electrocardiography-gated computed tomography angiography demonstrated significant kinking of the graft adjacent to the proximal anastomotic site. Cine cardiac magnetic resonance imaging showed that graft kinking deteriorated during systole. We concluded that his hemolytic anemia was caused by graft kinking and surgical repair was required. The graft was transected at the kinking site and then reconstructed. After surgical repair, the hemolytic anemia improved immediately.

Interstitial cystitis/bladder pain syndrome (IC/BPS) is a chronic disease characterized by suprapubic pain and lower urinary tract symptoms. Perhaps because of the heterogeneous nature of this disease and its multifactorial etiology, clinical trials in allinclusive populations of IC/BPS patients without phenotyping in the last decade have mainly failed to discover new therapeutic modalities of IC/BPS. Thus, phenotyping IC/BPS, aimed at identifying bladder-centric and/or bladder-beyond pathologies, including cystoscopic observation of Hunner or non-Hunner lesions of the bladder mucosa, is particularly important for the future of IC/BPS management. Based on recent discussions at international conferences, including the International Consultation on IC, Japan, it has been proposed that Hunner-lesion IC should be separated from other non-Hunner IC/BPS because of its distinct inflammatory profiles and epithelial denudation compared with non-Hunner IC/BPS. However, there are still no standard criteria for the diagnosis of Hunner lesions other than typical lesions, while conventional cystoscopic observations may miss atypical or small Hunner lesions. Furthermore, diagnosis of the bladder-centric phenotype of IC/BPS requires confirmation that identified mucosal lesions are truly a cause of bladder pain in IC/BPS patients. This review article discusses the current status of IC/BPS pathophysiology and diagnosis, as well as future directions of the proper diagnosis of bladder-centric IC/BPS, in which pathophysiological mechanisms other than those in inflammatory pathways, such as angiogenic and immunogenic abnormalities, could also be involved in both Hunner-lesion IC and non-Hunner IC/BPS. It is hoped that this new paradigm in the pathophysiological evaluation and diagnosis of IC/BPS could lead to pathology-based phenotyping and new treatments for this heterogeneous disease.

Interstitial cystitis/bladder pain syndrome (IC/BPS) is a chronic disease characterized by suprapubic pain and lower urinary tract symptoms. Perhaps because of the heterogeneous nature of this disease and its multifactorial etiology, clinical trials in allinclusive populations of IC/BPS patients without phenotyping in the last decade have mainly failed to discover new therapeutic modalities of IC/BPS. Thus, phenotyping IC/BPS, aimed at identifying bladder-centric and/or bladder-beyond pathologies, including cystoscopic observation of Hunner or non-Hunner lesions of the bladder mucosa, is particularly important for the future of IC/BPS management. Based on recent discussions at international conferences, including the International Consultation on IC, Japan, it has been proposed that Hunner-lesion IC should be separated from other non-Hunner IC/BPS because of its distinct inflammatory profiles and epithelial denudation compared with non-Hunner IC/BPS. However, there are still no standard criteria for the diagnosis of Hunner lesions other than typical lesions, while conventional cystoscopic observations may miss atypical or small Hunner lesions. Furthermore, diagnosis of the bladder-centric phenotype of IC/BPS requires confirmation that identified mucosal lesions are truly a cause of bladder pain in IC/BPS patients. This review article discusses the current status of IC/BPS pathophysiology and diagnosis, as well as future directions of the proper diagnosis of bladder-centric IC/BPS, in which pathophysiological mechanisms other than those in inflammatory pathways, such as angiogenic and immunogenic abnormalities, could also be involved in both Hunner-lesion IC and non-Hunner IC/BPS. It is hoped that this new paradigm in the pathophysiological evaluation and diagnosis of IC/BPS could lead to pathology-based phenotyping and new treatments for this heterogeneous disease.

  In 2008, Japanese Society of Drug Informatics (JASDI) organized the Future Vision Committee (the Committee) to propose the essential focus of drug informatics.  To explore a future vision about the drug information sciences, it was necessary to collect a variety of opinions widely from researchers.  Therefore, at the 11^th annual meeting of JASDI in July 5-6, 2008, the Committee convened a workshop to extract problems in the researches of drug informatics by using KJ method and evaluated the contents.  The major problems raised were “the field of drug informatics is too broad” and “there is no definition and/or no system of the drug informatics”.  Related problems raised are the shortness of the history and lack of originality in the study.  From different viewpoints, it was also pointed out that the methodology of the research is not well established and no systematic education is provided.  Taken together, major problems in drug informatics are concluded to be the lack of definition and the lack of systematizations, and will be solved to a certain extent by defining the outcome of the researches in drug informatics.