Objective This study aimed to evaluate the possible effects of Helicobacter pylori (H. pylori) infection in reflux esophagitis with scleroderma.
Patients and Methods There were a total of 138 patients with scleroderma in our hospital between October 1998 and June 2005. Among these patients, 64 consecutive patients of scleroderma, who did not receive medication for gastrointestinal diseases, underwent endoscopy after informed consent. H. pylori was examined using an H. pylori IgG ELISA. The endoscopists graded esophageal mucosal breaks according to the Los Angeles Classification of Esophagitis.
Results Among the 64 patients, 37 patients (57.8%) were positive for H. pylori infection. Reflux esophagitis was observed in 10 of 37 H. pylori-positive patients and in 19 of 27 H. pylori-negative patients. Significantly fewer H. pylori-infected patients had reflux esophagitis than H. pylori-negative patients (p<0.01). The odds ratio for H. pylori infection and reflux esophagitis was 0.16 (95%CI; 0.052-0.47).
Conclusion These findings suggest an important role for H. pylori infection in reflux esophagitis with scleroderma.

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Objective We investigated the factors contributing to whether or not hypertensive patients brought their home blood pressure records to the outpatient clinic.
Method We studied 325 hypertensive patients [169 men (66.3±11.4 years old) and 156 women (68.1±11.2 years old)] who had received medical treatment for hypertension in our outpatient clinic from June to August 2006.
Results Of the 325 patients studied, 206 (63.4%, 101 men, 105 women) brought their home blood pressure records to our outpatient clinic. Logistic analysis showed age [odds ratio (OR) =0.95; 95% confidence interval (CI): 0.93-0.98; p=0.0002], systolic blood pressure in outpatient clinic (OR=1.02; 95% CI: 1.00-1.04; p=0.0488) and the number of medicines prescribed (OR=1.94; 95% CI: 1.37-2.75; p=0.0002) were independent factors contributing to whether or not hypertensive patients bring along their home blood pressure records to the outpatient clinic.
Conclusion The contributing factors determining whether the patients bring their home blood pressure records to the outpatient clinic were: younger age, higher systolic blood pressure in the outpatient clinic, and a higher number of antihypertensive drugs. In conclusion, our results suggest that physicians should further motivate older patients, with well-controlled blood pressure in the outpatient clinic, to bring their home blood pressure records to the outpatient clinic.

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Objective Cyclosporine has been used for patients with nephrotic syndrome. Because of substantial inter- and intra-patient variability and a narrow therapeutic window, drug monitoring of cyclosporine is mandatory. To confirm the therapeutic effects of a cyclosporine microemulsion (CSAME), the absorption profile of the agent after preprandial administration was determined in steroid-resistant patients with refractory nephrotic syndrome.
Methods Fourteen patients were enrolled into the study (mean age, 31.2±12; 6 men, 8 women). The patients received 1.5 mg/kg of cyclosporine 30 minutes before breakfast for 6 months. Blood cyclosporine concentration was measured 5 times serially: before administration (C0) and at 1-hour intervals until 4 hours after administration of cyclosporine (C1-C4). In addition, area under the concentration-time curve from 0-4 hours (AUC_0-4) was calculated.
Results After 6 months, CSAME showed marked improvement in proteinuria levels (8.3±4.8 g/day vs 0.8±0.4 g/day, p<0.001). No changes in serum creatinine and urea nitrogen levels were observed. In 83% of the patients, the CSAME peak concentration appeared within 1 hour after administration (C1). A strong positive correlation was noted between AUC_0-4 and C1 (R²=0.90312) and C2 (R²=0.78431). The mean steroid (prednisolone) dose was 40 mg/day when CSAME treatment was started, but a lowering of the dose to 17.5 mg/day (p<0.001) was achieved at 6 months after CSAME therapy.
Conclusion Preprandial administration of CSAME is effective in steroid-resistant patients with refractory nephrotic syndrome. C1 or C2, but not C0, was a good clinical marker for CSAME exposure.

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Objective The maxillofacial characteristics of patients with obstructive sleep apnea syndrome (OSAS) have previously been analyzed using standard cephalometric analysis. Malocclusion influences the occurrence of sleep apnea, but the pathology of malocclusion in OSAS has not yet been fully investigated. Therefore, we investigated malocclusion in patients with OSAS using cephalometric and dental analysis.
Methods Cephalometric and dental analyses were performed to evaluate malocclusion in 97 male patients with OSAS (49.7±11.7 years). The number of apnea and hypopnea episodes per hour (apnea-hypopnea index: AHI) was determined by standard polysomnography.
Results The overall prevalence of severe overjet (the horizontal distance between the upper and lower incisors of ≥6 mm) was 43.3%. AHI was significantly correlated with body mass index (BMI) in obese OSAS patients (r=0.385, p=0.010), whereas it was significantly correlated with overjet in non-obese OSAS patients (BMI<25 kg/m²) (r=0.313, p=0.022). Multiple regression analysis revealed that BMI was the significant factor contributing to increased AHI in all patients, and overjet was in non-obese OSAS patients. There were no significant differences between non-obese and obese OSAS patients in the angle of protrusion of the superior alveolar base (SNA) or in the angle of protrusion between the superior and inferior alveolar bases (ANB). The angle of protrusion of the inferior alveolar base (SNB) was significantly smaller in non-obese than in obese OSAS patients.
Conclusion We have shown that overjet was associated with the severity of OSAS in non-obese patients. Our findings suggest that malocclusion may play an important role in the development of sleep apnea/hypopnea.

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Objective A fine vascular network is developed in the subepithelial layer of bronchial mucosa. Cigarette smoking is thought to influence angiogenesis. However, the bronchial microvasculature in smokers has not been fully investigated. The aim of this study was to determine the characteristics of the subepithelial microvessels in the large airways of smokers.
Methods Between August 2000 and July 2004, 12 current smokers and 12 nonsmokers were enrolled in this study. The nonsmokers had no abnormal findings in the large airways in the examination of conventional bronchoscopy. Smoking histories varied from 20 to 50 years. The average smoking index was 31.3±19.9 (mean ± SD) pack-years. High magnification bronchovideoscopy was used in this study, which provides information on bronchial mucosa with a maximum magnification of 110 times. To evaluate the characteristics of subepithelial microvessels, 4 parameters were compared between the two groups: 1) vessel area ratio, 2) vessel length ratio, 3) vessel area to length ratio, and 4) hemoglobin index.
Results Subepithelial microvessels of large airways were narrow and sparsely distributed in smokers. Vessel area ratio, vessel length ratio, vessel area to length ratio and hemoglobin index were significantly lower in the smokers than in the nonsmokers (p<0.0001, p<0.0001, p<0.0001, p=0.0002, respectively).
Conclusion Subepithelial microvessels of smokers were decreased and narrow, which suggested a decrease in microcirculation in the subepithelial layer of the large airway.

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Background Most split-night sleep (SNS) studies have enrolled Caucasian patients with moderate-to-severe obstructive sleep apnea-hypopnea syndrome (OSAHS), with different apnea-hypopnea index (AHI) criteria for diagnosis and successful CPAP titration from the standard full-night study (FNS). This study aimed to describe a shortened diagnostic SNS in the Taiwanese population using diagnostic and CPAP therapeutic criteria identical to the standard FNS, and to generate a predictive equation for effective CPAP (P_eff) titration.
Methods A total of 418 patients with moderate-to-severe OSAHS were enrolled in this study. The duration of the diagnostic SNS was shortened to approximately 1-2 hours. The diagnostic accuracy and the success rate of CPAP titration in SNS were evaluated and a statistical model for P_eff was built. The accuracy of the predictive equation was validated in another 127 patients.
Results Eighty-nine percent of patients with moderate-to-severe OSAHS diagnosed in the preceding FNS yielded the same grade of OSAHS in SNS. These reproducible patients were more severe, with 88% attaining successful CPAP titration in SNS. The predictive P_eff=1.98+0.184×BMI+0.01×AHI_SNS +0.016×DI_SNS (R²=0.28, p<0.0001) where BMI was the body mass index and DI the desaturation index. The | predictive P_eff- P_eff | was within 2 cm H₂O in 84% of the study group and 73% of the validation group.
Conclusion The modified split-night protocol and the predictive equation for CPAP can be useful in Taiwanese patients with moderate-to-severe OSAHS. Our findings may shorten the waiting time for polysomnography.

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Objective Polymyositis (PM) and dermatomyositis (DM) are idiopathic inflammatory myopathies; autoimmune mechanisms are thought to play an important role in their pathogenesis. We investigated the immunocytochemical characteristics and Th1/Th2 balance of peripheral blood lymphocytes in PM and DM using flow cytometry.
Patients and Methods Eight patients with PM and 13 with DM were enrolled in this study. Of these, 8 patients with DM were examined before and after clinical remission. No patients were receiving any treatment for PM or DM at enrollment. Ten healthy subjects were used as controls.
Results Patients with PM showed significant increases in CD3⁺CD4⁺HLA-DR⁺ (p<0.01) and CD19⁺CD23⁺ cells (p<0.05), and significant decreases in CD3⁺CD4⁺ (p<0.005) and CD4⁺CD45RO⁺ cells (p<0.05) compared with controls. Patients with DM showed significant increases in CD19⁺ (p<0.05) and CD19⁺CD23⁺ cells (p<0.05), and significant decreases in CD4⁺CD45RO⁺ cells (p<0.005) and the CD4⁺CD45RO⁺/CD4⁺CD45RA⁺ ratio (p<0.005) compared with controls. CD4⁺interferon (IFN)-γ⁺ cells and the intracellular IFN-γ/interleukin (IL)-4 ratio in CD4⁺ cells were significantly lower in patients with DM than in those with PM (p<0.05) or controls (p<0.0005 and p<0.001, respectively). The intracellular IFN-γ/IL-4 ratio in CD4⁺ cells was significantly increased in DM after clinical remission compared with before (p<0.05).
Conclusion Both B and helper T cells are activated in peripheral blood of active PM. Th2 cells predominate in peripheral blood of active DM, and the intracellular IFN-γ/IL-4 ratio in CD4⁺ cells may be useful as a clinical marker indicating disease activity.

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Obscure gastrointestinal bleeding is a very rare entity which accounts for less than 5% of all gastrointestinal bleeding cases. Small bowel tumors are rare but a serious source of obscure gastrointestinal bleeding. Lipomas are the second most common benign tumors in the small bowel and can produce many complications, including gastrointestinal bleeding. Herein, we describe a case of obscure gastrointestinal bleeding caused by a small bowel tumor which was detected by capsule endoscopy and double-balloon enteroscopy preoperatively. Finally, the tumor was surgically confirmed to be a lipoma.

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The perforation of a mitral valve aneurysm is a rare disease which induces acute mitral regurgitation and is usually induced by infective endocarditis; however, in this case report, acute heart failure was caused by a perforated mitral valve aneurysm that was speculated to be due to Libman-Sacks endocarditis with systemic lupus erythematosis and secondary anti-phospholipid syndrome. Mitral valve plasty was performed and thereafter heart failure improved.

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A 53-year-old woman with hereditary motor and sensory neuropathy was admitted to our hospital with congestive heart failure, followed by cardiogenic shock and acute renal failure. The patient's neuropathy was a rare entity documented first in a local area of Okinawa, Japan. Intensive supportive therapy was performed and the patient recovered within 4 weeks. Echocardiography revealed takotsubo-type left ventricular wall motion abnormality at onset. During hospitalization, restoration and recurrence of apical ballooning were observed at shorter intervals of days. The unique left ventricular wall motion abnormality of takotsubo cardiomyopathy is possibly a dynamically repetitive phenomenon. Serial echocardiographic studies are necessary so as not to miss recurrences within short intervals.

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A patient with multiple endocrine neoplasia type 1 (MEN1) developed a mediastinal seminoma. The patient was a 46-year-old man who presented with respiratory symptoms. A diagnosis of mediastinal seminoma was pathologically confirmed and a complete remission was achieved by chemotherapy. During his hospital stay, hyperparathyroidism and multiple pancreatic tumors associated with hypergastrinemia were found. A diagnosis of MEN1 was made genetically. Although patients with MEN1 manifest a variety of neoplastic disorders, no cases of concurrent seminoma and MEN1 have previously been reported. In addition, no etiological relationship between seminoma and MEN1 has yet been reported.

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We report a unique male patient presenting with portosystemic encephalopathy (PSE) due to intrahepatic portohepatic venous (PHV) shunts. He was diagnosed as having Klippel-Trénaunay-Weber syndrome (KTWS) based on the findings of a hemitruncal port-wine stain with subcutaneous arteriovenous fistulae and varicose veins in the legs. However, limb-hypertrophy, which is one of the most cardinal manifestations of KTWS, was absent, and in KTWS, PSE is quite a rare clinical manifestation. Hence, the clinical picture of this patient was unusual. Our clinical observation suggests that KTWS can be one of the underlying disorders causing PSE.

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We report the first adult case of Influenza A virus infection with acute unilateral oculomotor nerve palsy. Unlike previous reports, our patient showed isolated unilateral oculomotor nerve palsy as soon as she developed general symptoms with Influenza A infection, and demonstrated no significant increases of anti-ganglioside antibodies including anti-GQ1b IgG antibody. She recovered immediately after treatment of oseltamivir phosphate. As for the mechanism by which Influenza A infection caused ophthalmoparesis, small vessel vasculitis due to direct invasion of the virus was speculated. Although influenza encephalitis/encephalopathy including acute necrotizing encephalopathy are most frequently reported in children, it is noteworthy that influenza virus can also cause focal neurological signs such as ophthalmoparesis in adult cases.

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