Objective The purpose of this study is to determine gender-related and city- and countryside-related differences of ulcerative colitis in a Chinese population, and to determine information on the role of lifestyle in the onset of ulcerative colitis.
Methods Patients with a diagnosis of ulcerative colitis established between Jan 2000 and Dec 2007 were investigated according to the age, gender and inductive factors of onset. Assessment of living condition of the patients was performed according to the place of living: city or countryside. Statistical analysis was performed using the chi-square test.
Results A total of 293 ulcerative colitis patients were involved in the study. The onset age of the rural patients was earlier than that of the urban patients (p=0.00295), while there was no difference in onset age between male and female patients (p=0.067995). Some inductive factors, such as psychological stress, fatigue, cold catching, and spicy or unwashed food, may contribute to the progress of ulcerative colitis.
Conclusion Both gender-related and city- and countryside-related differences were found in the expression of ulcerative colitis in the Chinese population. Lifestyle factors such as psychological stress and fatigue may contribute to the expression of ulcerative colitis.

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Objective To determine the clinical usefulness of long-term intensive insulin therapy in Japanese patients with type 2 diabetes.
Patients and Methods Various clinical indicators and potential of withdrawal from insulin therapy were investigated in 20 type 2 diabetic patients receiving treatment in the outpatient clinic over a period of 8 years after starting intensive insulin therapy between April 1997 and March 1999.
Results The mean glycated hemoglobin (HbA1c) was approximately 6.5%, and there was no significant increase in insulin dose over a period of 8 years after commencement of intensive insulin therapy. Withdrawal from insulin therapy could be sustained in previously untreated patients.
Conclusion Our results indicated the clinical usefulness of intensive insulin therapy over the long term in Japanese patients with type 2 diabetes and that such therapy could be especially useful for previously untreated patients.

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Objective Smoking is an important risk factor in the development of community-acquired pneumonia (CAP). This study was designed to investigate the relationship between nicotine dependence level and the development of CAP.
Materials and Methods The Fagerstrom test for nicotine dependence (FTND) was performed to measure nicotine dependence level (NDL). Subjects with a Fagerstrom score (FS) of 5 or lower were defined as low dependence level and a FS score of 6 or higher was defined as high dependence level.
Results The risk of pneumonia development was higher in smokers than in nonsmokers (OR=2.19, 95% CI 1.13-4.23). The pneumonia development risk was 1.91 times higher in the low dependence level group compared to nonsmokers (OR=1.91, 95% CI 0.95-3.83). In the high nicotine dependence level group pneumonia risk was 2.93 times higher than in nonsmokers (OR=2.93, 95% CI 1.34-6.36). We also studied the relationship between CAP and the time to the first cigarette of the day. Risk was the lowest in the smoker group of after 60 minutes and risk ratios increased with decreased time.
Conclusion In this study, a high nicotine dependence level was found to be a risk factor associated with smoking for CAP development. The time period of the first cigarette after waking up is also important in pneumonia development as well as in the nicotine dependence level.

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Background The granulomatous inflammatory response is a manifestation of many lung diseases.
Objective To evaluate the diagnostic value of epithelioid cell granulomas in bronchoscopic biopsies in daily clinical practice.
Methods The data of 105 patients with epithelioid cell granulomas in biopsy tissue who had undergone the bronchoscopic lung biopsy or bronchial biopsy at the Centre of Pulmonology and Allergology of Vilnius University Hospital Santariski klinikos (Vilnius, Lithuania) were examined. All cases were divided into non-necrotizing epithelioid cell granulomas and epithelioid cell granulomas with necrosis.
Results Of all the cases 66% had non-necrotizing epithelioid cell granulomas and 34% had epithelioid cell granulomas with necrosis. Without respect to the presence of necrosis in granulomas, the majority of the patients (79%) had sarcoidosis or tuberculosis; 94% of the patients with sarcoidosis had non-necrotizing epithelioid cell granulomas and the remaining 6% had granulomas with necrosis. The sensitivity of non-necrotizing epithelioid cell granuloma for the diagnosis of sarcoidosis was 94% and specificity 60%. The positive and negative predictive values were 68% and 92%, respectively.
Of the patients with tuberculosis 76% had epithelioid cell granulomas with necrosis and 24% had non-necrotizing epithelioid cell granulomas. The sensitivity of epithelioid cell granuloma with necrosis for the diagnosis of tuberculosis was 76% and specificity 85%. The positive and negative predictive values were 69%, and 88%, respectively.
Conclusion A significant overlap in types of granulomatous inflammation between tuberculosis and sarcoidosis was found. The type of epithelioid cell granuloma alone was not sufficient for the final clinical diagnosis.

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Objective We investigated the psychiatric disorders in subacute myelo-optico-neuropathy (SMON) patients by structured interview. The prevalence of major depressive disorder in SMON patients was estimated by structured interview and using Beck's depression inventory (BDI) questionnaires.
Materials and Methods Psychiatric conditions were evaluated in 26 SMON patients (9 males, 17 females, mean age 70.7 years) living in Kyoto prefecture through a structured interview given by psychiatrists. BDI questionnaires and clinical symptoms of SMON were investigated in 106 patients, ranging from 51 to 91 years in age (mean, 73.5) with SMON patients living in Kinki area. BDI questionnaires were obtained from 92 age-matched aged healthy people, ranging from 57 to 91 years in age (mean, 75.8), living in Kyoto city.
Results Among the psychiatric disorders in SMON patients, the prevalence of major depressive disorder and suicidal ideation significantly increased during the period of clioquinol intake and four patients (15.4%) out of 26 SMON patients still suffer from major depressive disorder. The prevalence of major depressive disorder in SMON patients was estimated at 15.1% (16/106) and this percentage was about seven times as frequent as in the age-matched aged healthy people (2.2%; 2/92). In female SMON patients, the degree of the depressive states was significantly correlated with the severe degree of dysesthesia of the lower extremities, and it was inversely correlated with the duration of SMON disease and the total scores of the Barthel index.
Conclusion This is the first report that shows the prevalence of major depressive disorder in SMON patients at present, which was seven times more frequent than age-matched aged healthy persons.

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Objective The progression of cardiac amyloidosis is a prognostic factor after liver transplantation (LT) in familial amyloid polyneuropathy (FAP). The aim of this study was to assess myocardial changes in FAP amyloidgenic transthyretin (ATTR) Val30Met after LT.
Patients and Methods Twelve Japanese FAP ATTR Val30Met patients who underwent LT and were followed for more than 2 years, were examined with serial echocardiography after LT. Serum BNP levels were measured in 9 patients.
Results A significant increase in mean left atrial diameter and interventricular septal thickness was observed after LT. The increase in left atrial diameter was correlated with the presence of granular sparkling echo (GSE) at preoperative examination. Serum brain natriuretic peptide (BNP) levels in patients with left atrial diameter dilation (152.0±157.6 pg/mL) were higher than in those without left atrial diameter dilation (32.0±30.0 pg/mL).
Conclusion LAD and IVS were significantly increased after LT compared with preoperative examinations in Japanese FAP ATTR Val30Met patients. BNP is an important biochemical indicator of myocardiac dysfunction in FAP patients. GSE is a useful echocardiographic marker to predict cardiac amyloidosis after LT.

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Staphylococcus epidermidis is a common cause of infections associated with prosthetic devices and immunocompromised patients. Spontaneous pyomyositis due to the above pathogen is very uncommon. Kikuchi-Fujimoto disease (KFD) is a subacute necrotizing lymphadenitis, first described in Japan. A T cell-mediated hyperimmune response to various pathogens in a genetically susceptible individual has been primarily been considered in its pathogenesis. We report a patient who developed spontaneous pyomyositis caused by S. epidermidis concurrently with KFD, and discuss the possibility of S. epidermidis infection being the stimulant of KFD.

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This report describes the case of 74-year-old man who had a gastric yolk sac tumor complicated with beta human chorionic gonadotropin (βhCG) producing metastases. He was a heavy drinker. He was admitted to the hospital for abdominal pain and fullness. Based on computed tomography and gastric endoscopy he was diagnosed to have gastric cancer that had metastasized to the liver, lung and lymph nodes. The level of serum alpha fetoprotein (AFP) was high at 523 ng/mL. He died of multiorgan failure six days after admission. The autopsy revealed the stomach tumor to be an AFP-positive yolk sac tumor. Lymph node metastases showed the same characteristics as the stomach tumor. However, the lesions on the liver and lung were negative for AFP and positive for hCG. The yolk sac tumor seemed to have retrodifferentiated to form an hCG-producing tumor in the metastatic lesions. A gastric yolk sac tumor is extremely rare and only eight cases of gastric yolk sac tumors have been previously reported in the literature. To avoid a misdiagnosis, careful attention should be paid to the above-mentioned morphological features and immunohistochemical findings, in addition to the clinical findings.

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Pheochromocytoma is a rare, catecholamine-secreting tumor. The classic symptoms are headache, diaphoresis, and tachycardia with paroxysmal hypertension. Other less common cardio-vascular manifestations, such as arrhythmias, angina pectoris, acute myocardial infarction, dilated cardiomyopathy, acute heart failure, and cardiogenic shock, have occasionally been reported. Here, we report two middle-aged men with acute myocarditis and cardiogenic shock, who needed an intra-aortic balloon pump and extra-corporeal membrane oxygenation for life support. They were diagnosed with pheochromocytoma and underwent laparoscopic adrenectomy that restored cardiac function. These cases illustrate diagnostic and management considerations in pheochromocytoma complicated by acute myocarditis and cardiogenic shock.

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Two elderly patients with mineralocorticoid excess state due to 11 β-hydroxysteroid dehydrogenase type 2 (11β-HSD2) impairment are described. Moreover, the role of the precursor-product ratios of the steroids reflecting 11β-HSD2 activity was estimated in 5 patients, including 3 patients reported previously by us. Significant elevations of urinary cortisol/cortisone ratios were observed, whereas urinary tetrahydrocortisol (THF)+allo-THF/tetrahydrocortisone (THE) ratios were not elevated significantly. Furthermore, an even more distinct elevation of serum cortisol/cortisone ratio was evident in all instances of 5 patients, suggesting a significant clinical role of the serum cortisol/cortisone ratio in the diagnosis of 11β-HSD2 impairment.

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Distant metastases of a papillary thyroid carcinoma (PTC) is rare, and usually involves the lung or the bones. Adrenal metastasis of a PTC has been described only in three patients. We describe a 74-year-old woman with adrenal metastasis of a PTC, detected with a total body iodine scan and a PET-CT scan.

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Choledocholithiasis can be caused by either primary or secondary bile duct stones. Although patients with sickle cell disease (SCD) are at high risk of development of pigmented gallstones due to chronic hemolysis, primary choledocholithiasis in SCD is very uncommon. The delay in the diagnosis of biliary tract pathology can occur in patients who had a prior cholecystectomy. There is a possible relationship between prior cholecystectomy and the subsequent occurrence of primary common bile duct stones, due to the higher probability of infection of the biliary system. Here, we report an unusual and severe case of multiple primary choledocholithiasis, associated with pancreatitis, in a patient with SCD, fourteen years after cholecystectomy.

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Protein-losing enteropathy (PLE) is characterized by gastrointestinal loss of serum protein. It is usually caused by hypersecretion from a tumor, ulcer, or long standing lymphangiectasia. However, we report a 47-year-old man of peritoneal nodal follicular lymphoma who developed PLE with none of them. Oozing of whitish fluid from duodenal bulbar mucosa was endoscopically seen, resulting in continuous loss of protein. Chemotherapy was effective and PLE was rapidly diminished. Nodal lymphoma lesion was considered to disturb lymphatic flow and to regurgitate it to duodenal mucosa. To our knowledge, this is the second report of a lymphoma patient presenting PLE without a gastrointestinal mucosal lesion.

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The present patient was diagnosed as having human herpes virus-8 (HHV-8)/Kaposi sarcoma herpes virus (KSHV)-negative and CD20-positive primary effusion lymphoma (PEL) of the right-sided pleural effusion. After pleural drainage, malignant cells disappeared spontaneously in a small amount of the remaining pleural effusion without chemotherapy. The patient was treated with six cycles of chemotherapy consisting of rituximab, cyclophosphamide, doxorubicin, vincristine and prednisone. He has been in complete remission for more than 22 months. It is suggested that effusion drainage followed by chemotherapy containing rituximab is a potential treatment strategy for patients with HHV-8/KSHV-negative and CD20-positive PEL.

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A Japanese woman developed gait disturbances at 25 years of age, and subsequently underwent gradual changes in her personality. By the age of 42, she showed clear signs of dementia and cerebellar ataxia, and displayed behavioral abnormalities, choreic movements and hyperreflexia. The findings of MRI not only showed cerebellar and cerebral atrophy, but also revealed putaminal rim hyperintensity on T2-weighted images. We identified a heterozygously expanded CAG/CAA repeat (45/36) within the TATA-binding protein gene, leading to a diagnosis of SCA17. These results show that a 45 CAG/CAA repeat is pathological, giving rise to early-onset SCA17.

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Osteomyelitis and septic arthritis are serious complications of sickle cell disease which occur at rates of 18% and 7%, respectively. In two small studies of osteomyelitis in sickle cell patients, the most common causative organisms were Salmonella spp. and Staphylococcus spp. Anaerobic infection is uncommon. Here, we report an 18-year-old girl with sickle cell disease and suspected spontaneous vertebral osteomyelitis due to Bacteroides fragilis. The diagnosis was based on multiple positive blood cultures. Despite extensive investigation, the port of entry could not be determined. The patient responded well to a four-week therapy with metronidazole, without the need for surgical debridement.

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Mycobacterium malmoense is a very rare pathogen of pulmonary infectious disease in Japan. We encountered a case of M. malmoense infectious lung disease which could be cured by surgical operation without chemotherapy. M. malmoense strains were isolated in both the bronchial washing lavage and the removed lung specimen, and it were identified using 16S rRNA gene and rpoB gene sequencing. This case might indicate that pulmonary infectious disease caused by a rare non-tuberculous mycobacteria pathogen should be positively considered to be treated surgically as an initial therapy when the patient's condition is admissive, and also indicated the importance of identification of the causative pathogen from surgical specimens. In addition, this was the second report of M. malmoense infectious disease, and the first case of surgical treatment case of M. malmoense lung disease in Japan, as far as we could determine.

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