A 46-year-old female patient with non-alcoholic steatohepatitis (NASH) was administered nateglinide, an insulin secretagogue, for 7 months, and then polyenephosphatidylcholine, an anti-oxidant medication, in accordance with the two-step hypothesis of NASH, with insulin resistance as the initial pathogenesis and oxidative stress as the second. HOMA-R, an index of insulin resistance, improved, and hepatic marker levels improved markedly by four weeks after initiation of polyenephosphatidylcholine. The beneficial effects of treatment continued over the 24 months of the study. A liver biopsy evaluated using Brunt's criteria showed improvement from stage 2 to stage 0 after 9 months. These findings suggest the therapeutic efficacy of step by step treatment of NASH in accordance with the two-stage hypothesis.
therapeutic aspects ; month ; Diagnostic Neoplasm Staging ; agreement ; Oral

The persistent fifth aortic arch is rare vascular anomaly. To our knowledge, this is the 24th reported case of the persistent fifth aortic arch. This patient was a 31 days old male infant and had the persistent fifth aortic arch associated with atresia of the fourth aortic arch, patent ductus arteriosus, a double-outlet right ventricle, and a mesocardia. He underwent a fifth aortic arch division and an extended aortic arch anastomosis with a division of ductus arteriosus. There was no blood pressure gradient between upper and lower limbs after the repair. However, no weaning from a cardiopulmonary bypass after the subsequent radical operation for double-outlet right ventricle caused his death.

Postoperative quantitative evaluation of left internal thoracic artery (LITA) grafts is usually performed by angiography, scintigraphy and Doppler flowire. However it is difficult to observe the characteristics of the intima of the LITA graft. The purpose of this study was to evaluate the characteristics and quantity of plaque of intima of LITA grafts in 6 cases after coronary artery bypass surgery using an intra-vascular ultrasound device (IVUS). There was no stenosis or calcification of LITA grafts on angiography. However we found atherosclerotic plaque in all LITA grafts by IVUS. Characteristics of plaque were eccentric in all cases, and soft, hard and mixed plaque were found. The average minimal lumen diameter of LITA grafts was 2.6±0.2mm. The average lumen area of LITA grafts was 5.4±0.7mm². The rate of plaque area was 37.1±5.9%. The eccentric arteriosclerotic plaques were seen in all cases, contradicting the established theory that LITA do not form arteriosclerosis easily. We suggest that IVUS is an effective follow-up device for evaluating the morphological findings and quantitative evaluation of LITA graft in a timely manner.

OBJECTIVETo summarize the clinical features of cytopenia with bone marrow hypoplasia in 100 children and to investigate an effective treatment regimen for myelodysplastic syndrome (MDS) in children.

METHODSA retrospective analysis was performed on the clinical data of 100 children non-randomly selected from Japan and China who were diagnosed with cytopenia with bone marrow hypoplasia between 2006 and 2011. The data of patients from China were subjected to prognostic analysis.

RESULTSThere was no significant difference in the proportion of MDS cases and acquired aplastic anemia (AA) cases between the Japanese and Chinese children. Of the 100 patients, there were 29 cases of acquired AA, 58 cases of refractory cytopenia of childhood (RCC) and 13 cases of refractory cytopenia with multilineage dysplasia (RCMD). There were significant differences in reticulocyte absolute value in peripheral blood and degree of bone marrow proliferation among the three patient groups (P<0.05). The patients from China were followed up for 16-70 months (median, 41 months). After being treated with cyclosporine (CsA) combined with stanozolol, the patients with AA had response rates of 25% and 75%, the patients with RCC had response rates of 47.1% and 82.4%, and the patients with RCMD had response rates of 60% and 60% respectively at 3 and 6 months after treatment.

CONCLUSIONSThere are significant differences in reticulocyte absolute value in peripheral blood and degree of bone marrow proliferation among patients with RCC, RCMD and acquired AA. CsA combined with stanozolol has a good therapeutic efficacy in the treatment of acquired AA and hypoplastic MDS in children, but studies of more cases and a longer follow-up duration are needed.

Adolescent ; Anemia, Aplastic ; blood ; drug therapy ; pathology ; Bone Marrow ; pathology ; Child ; Child, Preschool ; Cyclosporine ; therapeutic use ; Female ; Follow-Up Studies ; Hematopoietic Stem Cell Transplantation ; Humans ; Infant ; Male ; Myelodysplastic Syndromes ; blood ; drug therapy ; pathology ; Pancytopenia ; blood ; drug therapy ; pathology

OBJECTIVE: Interest in the "at-risk mental state" (ARMS) for psychosis has increased because early intervention is expected to delay or prevent the onset of schizophrenia. However, the optimum intervention strategy remains controversial, especially with regard to antipsychotics. Although administration of antipsychotic medications is often associated with adverse effects and raises ethical considerations, recent studies have shown that some novel antipsychotics are safer and more tolerable for young people than conventional antipsychotics. We investigated whether administration of perospirone, a combined serotonin (5-HT)/dopamine antagonist and 5-HT1A receptor agonist, could alleviate prodromal symptoms and be well tolerated by clinical high risk patients. METHODS: The participants were outpatients seeking help. The Structured Interview for Prodromal Symptoms was performed in patients identified as being at clinical high risk. The Scale of Prodromal Symptoms (SOPS) was also completed and changes of subjective experience were assessed with the Subjective Well-being under Neuroleptics, short version. The incidence of akathisia was recorded by using the Barnes Akathisia Scale. Subjects were monitored for 26 weeks after starting medication. RESULTS: SOPS scores improved significantly after 26 weeks of perospirone therapy, while BAS scores did not show deterioration. No serious adverse events occurred during the study. CONCLUSION: This trial suggests that perospirone therapy provides a clinical benefit for clinical high risk subjects without causing serious adverse events. Although further placebo-controlled studies are needed for confirmation, perospirone might be one of optimum treatments for individuals at imminent risk of psychosis.
Antipsychotic Agents ; Early Intervention (Education) ; Humans ; Incidence ; Outpatients ; Prodromal Symptoms ; Psychomotor Agitation ; Psychotic Disorders ; Receptor, Serotonin, 5-HT1A ; Schizophrenia ; Serotonin

A 75-year-old Japanese man with chronic hepatitis C was found to have a large liver tumor and multiple nodules in the bilateral lungs. We diagnosed the tumor as hepatocellular carcinoma (HCC) with multiple lung metastases based on imaging studies and high titers of HCC tumor markers. Remarkably, without any anticancer treatment or medication, including herbal preparations, the liver tumor decreased in size, and the tumor makers diminished. Moreover, after 1 year, the multiple nodules in the bilateral lungs had disappeared. Fifteen months after the first medical examination, transcatheter arterial chemoembolization (TACE) was performed for the residual HCC. Because local relapse was observed on follow-up computed tomography, a second TACE was performed 13 months after the first one. At 4 years after the second TACE (7 years after the initial medical examination), there was no recurrence of primary or metastatic lesions. Spontaneous regression of HCC is very rare, and its mechanism remains unclear. Understanding the underlying mechanism of this rare phenomenon may offer some hope of finding new therapies, even in advanced metastatic cases.
Aged ; Carcinoma, Hepatocellular/pathology/*secondary ; Chemoembolization, Therapeutic ; Humans ; Liver Neoplasms/*pathology ; Lung Neoplasms/*secondary ; Male ; Neoplasm Recurrence, Local/pathology ; Neoplasm Regression, Spontaneous/*pathology ; Tomography, X-Ray Computed

PURPOSE: The aim of this study was to evaluate the validity and reliability of the Seattle Angina Questionnaire, Japanese version (SAQ-J) as a disease-specific health outcome scale in patients with coronary artery disease. METHODS: Patients with coronary artery disease were recruited from a university hospital in Tokyo. The patients completed self-administered questionnaires, and medical information was obtained from the subjects' medical records. Face validity, concurrent validity evaluated using Short Form 36 (SF-36), known group differences, internal consistency, and test-retest reliability were statistically analyzed. RESULTS: A total of 354 patients gave informed consent, and 331 of them responded (93.5%). The concurrent validity was mostly supported by the pattern of association between SAQ-J and SF-36. The patients without chest symptoms showed significantly higher SAQ-J scores than did the patients with chest symptoms in 4 domains. Cronbach's alpha ranged from .51 to .96, meaning that internal consistency was confirmed to a certain extent. The intraclass correlation coefficient of most domains was higher than the recommended value of 0.70. The weighted kappa ranged from .24 to .57, and it was greater than .4 for 14 of the 19 items. CONCLUSIONS: The SAQ-J could be a valid and reliable disease-specific scale in some part for measuring health outcomes in patients with coronary artery disease, and requires cautious use.
Asian Continental Ancestry Group ; Coronary Artery Disease ; Coronary Vessels ; Humans ; Informed Consent ; Medical Records ; Reproducibility of Results ; Thorax ; Tokyo ; Surveys and Questionnaires

Background/Aims: The Heart Failure Association (HFA)-PEFF score is recognized as a simple method to diagnose heart failure (HF) with preserved ejection fraction (HFpEF). This study aimed to evaluate the relationship between HFA-PEFF scores and cardiovascular outcomes in HFpEF patients. Methods: A total of 502 consecutive HFpEF patients were prospectively observed for up to 1,500 days. Cardiovascular outcomes were compared between two groups of patients, defined by their HFA-PEFF scores: those who scored 2–4 (the intermediate-score group) and those who scored 5–6 group (the high-score group). Overall, 236 cardiovascular events were observed during the follow-up period (median, 1,159 days). Results: Kaplan-Meier analysis showed that there were significant differences in composite cardiovascular events and HF-related events between the intermediate-score group and the high-score group (p = 0.003 and p < 0.001, respectively). Multivariate Cox proportional hazards analysis showed that the HFA-PEFF scores significantly predicted future HF-related events (hazard ratio, 1.66; 95% confidence interval [CI], 1.11 to 2.50; p = 0.014); receiver operating characteristic analysis confirmed this relationship (area under the curve, 0.633; 95% CI, 0.574 to 0.692; p < 0.001). The cutoff HFA-PEFF score for the identification of HF-related events was 4.5. Decision curve analysis revealed that combining the HFA-PEFF score with conventional prognostic factors improved the prediction of HF-related events. Conclusions HFA-PEFF scores may be useful for predicting HF-related events in HFpEF patients.

We report a case of a 64-year-old woman with a history of radiation therapy for breast cancer 27 years ago who developed malignant pericardial mesothelioma. Since 3 years ago, the recurrent bloody pericardial effusion was getting worse, which caused general edema and nocturnal dyspnea. She had a thickened pericardium and the right ventricular pressure curve showed a dip-and-plateau pattern. We diagnosed constrictive pericarditis and performed a pericardiectomy and waffle procedure on the thickened epicardium without cardiopulmonary bypass. The post-operative histology confirmed malignant pericardial mesothelioma and she died on the 17th postoperative day. Pericardial malignant mesothelioma is a rare disorder but very aggressive. This fatal disease may be considered in a patient with recurrent bloody pericardial effusion who has a history of thoracic radiation therapy.

We report the case of a 55-year-old man who received a hard blow to his chest from a liquid nitrogen hose that caused traumatic aortic dissection (Stanford type A, DeBakey type II). He did not have any other hemorrhagic injury ; therefore, we decided to perform an emergency surgery. The postoperative course was uneventful, and he was discharged on postoperative day 19. Pathological findings were compatible with traumatic aortic dissection. Blunt thoracic aortic injury is a potentially life-threatening injury ; therefore, it is worth remembering that relatively low-energy blunt trauma can cause aortic injury in patients with severe atherosclerosis. The optimal timing of intervention should be individualized in traumatic aortic injury with consideration of associated injuries.