Background The incidence of metabolic syndrome (MetS) has not been fully studied.
Methods and Results The data of 35,534 subjects who underwent a health examination both in 1996 and 2001 were analyzed. Since the waist circumference was not available, modified criteria of MetS was used for those with 3 or more of the following items: 1) body mass index (BMI) ≥25 kg/m², 2) blood pressure ≥130 mm Hg in systolic and/or ≥85 mm Hg in diastolic, 3) triglycerides ≥150 mg/dL, 4) high-density lipoprotein (HDL) cholesterol ≤40 mg/dL in men, ≤50 mg/dL in women, and 5) fasting blood glucose (FBG) ≥100 mg/dL. The subjects who underwent therapy for hypertension, diabetes mellitus, and high TG were considered to have these items. The incidence of MetS over 5 years was determined and its risks were evaluated by Cox proportional-hazards models. During the follow-up of exactly 5 years, MetS developed in 2,853 (9.32%) among 30,623 subjects who had no MetS at baseline. The subjects who developed MetS were older, and had elevated BMI, blood pressure, TG and FBG (p<0.001 for all) and lower HDL (p<0.034 for men and p<0.001 for women). Each item of MetS and their combination was associated with a higher risk to develop MetS. Obesity (BMI ≥25 kg/m²) alone or in combination with another item was associated with a higher risk to develop MetS suggesting it plays a key role.
Conclusion MetS using BMI developed in 9.32% over 5 years or 1.86%/year. Each item of MetS or their combination showed a high risk to develop MetS. Obesity was associated with a higher hazard ratio to develop MetS.

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Background Thrombotic microangiopathies (TMAs) are pathological conditions characterized by generalized microvascular occlusion by platelet thrombi, thrombocytopenia, and microangiopathic hemolytic anemia. Two typical phenotypes of TMAs are hemolytic-uremic syndrome (HUS) and thrombotic thrombocytopenic purpura (TTP). Severe deficiency of plasma ADAMTS13 activity (ADAMTS13: AC) is more specific for TTP, but not for HUS. Since 1998, our laboratory has functioned as a nationwide referral center for TMAs by analyzing ADAMTS13.
Methods Of 1,564 patients tested from 426 hospitals, 919 were positive for TMA. Levels of ADAMTS13: AC and the ADAMTS13 neutralizing autoantibody (ADAMTS13: INH) were determined by chromogenic act-ELISA and/or by classic von Willebrand factor multimer assay.
Results TMA patients consisted of two groups: severe (less than 3% of normal control) and non-severe deficiency of ADAMTS13: AC. Both groups were divided into congenital (n=65) and acquired (n=854) TMA. Of the former, 41 had congenital deficiency of ADAMTS13: AC, while the remaining 24 had disease of unknown etiology. The 854 patients with acquired TMA could be largely grouped into three categories: idiopathic TTP (n=284), idiopathic HUS (n=106), and secondary TMAs (n=464). The secondary TMAs were observed in heterogeneous patient groups and were associated with drugs, connective tissue diseases, malignancies, transplantation, pregnancy, E. coli O157: H7 infection, and other factors. All of the patients with acquired severe ADAMTS13: AC deficiency were positive for ADAMTS13: INH.
Conclusion Although TMAs are highly heterogeneous pathological conditions, one-third of TMA patients have severe deficiency of ADAMTS13: AC. Platelet transfusions to such patients are contraindicated. Rapid ADAMTS13: AC assays are therefore prerequisite to appropriately treat TMA patients.

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Objective Patients treated in a protective isolation unit (PIU) often experience loneliness and increased feelings of seclusion, leading to elevated levels of distress, but the relationship between psychiatric distress and environmental factors is unclear. Therefore, we retrospectively compared the impact of environmental factors on insomnia and depression between patients in the PIU and those in a standard ward (SW).
Methods Subjects were 246 patients with hematological disorders hospitalized in Meitetsu Hospital between April 1, 2007 and July 31, 2008 (62 PIU patients and 184 SW patients). We assessed insomnia and depression, as well as concomitant corticosteroid (CS) administration, stem cell transplantation (SCT) therapy, and complications resulting from these therapies. Details of medical history and patient information were retrospectively extracted from patients' charts, medical records and the electronic laboratory database at the hospital.
Results Patients in the PIU tended to be complicated by insomnia or depression more than those in the SW, but the stay in the PIU was not significantly related to the incidence of insomnia or depression. Our findings indicated that use of CS was a risk factor for insomnia. The prevalence of depression was higher in patients with therapeutic complications. All PIU patients with depression also received SCT.
Conclusion Our findings suggest an increased potential for insomnia after administration of CS and depression in cases with complications after SCT, which is important to keep in mind for patients with hematological disorders.

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Objective The purpose of this study was to clarify the differences in physiological properties of the airways between asthma and COPD using an impulse oscillation system (IOS).
Patients and Methods Subjects comprised 95 stable COPD patients, 52 never-smoker asthma patients and 29 healthy never-smokers >60 years old, all matched for age, in whom respiratory impedance was examined by IOS.
Results In both asthma and COPD patients, a significant increase in respiratory resistance (Rrs5) and more negative value of respiratory reactance (Xrs5) at 5 Hz of oscillatory frequency with an increase in resonant frequency (fres) were observed when compared with healthy never-smokers. In asthma, a significant increase in respiratory resistance at 20 Hz (Rrs20) was also observed when compared with healthy never-smokers and COPD. The increases in Rrs5 and relative changes of Xrs5 to more negative were remarkable with increasing severity of COPD. On the other hand, among patients with asthma, these changes in Rrs5 and Xrs5 were also observed in asthmatics with normal FEV₁/FVC. Interestingly, Xrs5 showed further changes to more negative in expiration of tidal breath in severe COPD, whereas no significant changes in Xrs5 to more negative in expiration was observed in healthy never-smokers and asthmatics with and without normal FEV₁/FVC.
Conclusion IOS may be useful for detecting pathophysiological changes of respiratory system in accordance with severity of COPD and even in asthmatics with normal FEV₁/FVC. The larger within-breath changes of Xrs5 to more negative in severe COPD may represent easy collapsibility of small airways in expiration of tidal breath. These properties may help to analyze airway mechanics and to identify abnormalities of the airways that cannot be found by spirometry alone.

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Objective The aim of this study was to investigate a large food-borne outbreak associated with eggs contaminated by Salmonella Enteritidis in a military unit using pulse field gel electrophoresis (PFGE) and the Repetitive-sequence-based PCR (rep-PCR) employing the DiversiLab system.
Materials and Methods In mid-January 2008, a food-borne outbreak associated with S. Enteritidis occurred in a military unit located in the city centre of Isparta. A total of 2,469 patients were registered to six hospitals with gastrointestinal disease symptoms such as diarrhea and abdominal pain. Of those registered, 445 were hospitalized. S. Enteritidis was isolated from 276 stool samples and a blood sample of the hospitalized patients and from a food item. The PFGE patterns after XbaI digestion and rep-PCR profiles produced by the DiversiLab system^TM were determined for eight randomly selected stool isolates, one blood isolate and one food isolate.
Results The PFGE patterns of all isolates were identical. The Rep-PCR profiles produced by using the DiversiLab system showed that all isolates were indistinguishable. The PFGE and rep-PCR interpretations were concordant for the S. Enteritidis isolates. All stool isolates, one blood isolate and one food isolate were susceptible to all antibiotics tested.
Conclusion This data suggest that the DiversiLab system may be a reasonable alternative to PFGE for investigation and control of S. Enteritidis outbreaks, since it is easy to use, rapid and does not require highly skilled operators.

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Concurrent glomeruli and liver involvement in patients with Sjögren's syndrome (SS) has seldom been reported. Meanwhile, IgA nephropathy (IgAN) associated with SS is not as common as other types of glomerulonephritis, such as membranoproliferative glomerulonephritis (MPGN) and membranous nephropathy (MN). Two patients with SS and IgAN are presented. Case 1 was diagnosed as SS complicated with IgAN (Haas subclass III) and severe AIH, while case 2 was diagnosed as SS and IgAN (Haas subclass I) with serious tubulointerstitial lesions. The true prevalence of IgAN secondary to SS is certainly underestimated and the crucial pathogenetic factors may be polyclonal hypergammaglobulinemia and hypocomplementemia (especially C4). The frequent presence of multiple immunoglobulin and complement deposition in glomeruli, especially weak deposits of C4 and (or) C1q, is an immunofluorescence staining feature of IgAN secondary to SS and may be associated with disease severity and activity.

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Pulmonary alveolar microlithiasis (PAM) is a rare disease characterized by the presence of small calculi in the alveolar space. The SLC34A2 is thought to be responsible for the disease. We encountered three siblings of an inbred family who have PAM. We examined the family of the proband who was admitted with dyspnea on exertion and cough, and eventually was diagnosed with PAM. Genetic analysis revealed that both parents (a consanguineous marriage) of the proband were carriers with heterozygous mutation of SLC34A2 gene, and three of their children were diagnosed with PAM with homozygous mutation in the SLC34A2 gene. These findings suggest that impaired activity of the SLC34A2 gene may be responsible for familial PAM.

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We report a 39-year-old man with intravascular large B-cell lymphoma (IVLBCL) who had been treated as a case with pulmonary arterial hypertension (PAH) for one year. After he became worse, diffuse pulmonary ¹⁸F-fluorodeoxyglucose (FDG) uptake in positron emission tomography (PET) suggested the existence of IVLBCL in the lung showing normal CT images. The diagnosis was confirmed with random transbronchial lung biopsy, and he was then successfully treated. Since IVLBCL presenting PAH has been rare and is difficult to diagnose, early application of FDG-PET may provide early recognition of the disorder, leading to a better outcome.

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Spontaneous resolution of bulla associated with infection or tumor is occasionally observed. However, resolution of progressively enlarging giant bullous emphysema (GBE) after medical therapy has not been reported. A 51-year-old man smoker with GBE on the right lung was referred to Samsung Medical Center for the consideration of bullectomy. A review of his medical records revealed that right-side bullous emphysema had been detected 4 years previously and it had progressively enlarged with a concomitant deterioration of lung function. Although he had a history of chronic asthma, he had never been treated on a regular basis. After combination therapy, including regular tiopropium, a salmeterol/flucatisone inhaler, a salbutamol inhaler as needed, and oral theophylline therapy, right side bulla showed marked regression. This regression of bulla was associated with an improved forced expiratory volume in one second and normalization of residual volume. This case serves as a reminder to clinicians that medical treatment for underlying diseases should precede surgical consideration in patients with GBE.

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The effects of direct hemoperfusion with polymyxin B-immobilized fiber (PMX-DHP) treatment for acute exacerbation of interstitial pneumonia have been reported. Here, we report 3 severe cases of drug-induced interstitial lung disease (DI-ILD) that were treated with PMX-DHP. Two DI-ILD cases were caused by methotrexate, and the third case was caused by the herbal medicine sanmotsu-ogon-to. The arterial oxygen tension/inspiratory oxygen fraction ratio improved during PMX-DHP treatment in all 3 patients. Finally, 2 patients survived and 1 died. The study findings indicate that PMX-DHP treatment is a viable option for the management of acute respiratory failure in patients with DI-ILD.

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Diffusion-weighted imaging can depict secondary signal changes of the substantia nigra in patients with ipsilateral striatal infarction. We report four patients who demonstrated obvious signal changes of the substantia nigra in the subacute phase of stroke. Embolic stroke was diagnosed in all of the cases, and none of the patients presented clinical deterioration in their course. Embolic mechanism might be more closely related to the secondary change of the substantia nigra than thrombosis. The relationship between secondary nigral degeneration and stroke etiology or between the nigral lesions and recanalization of the middle cerebral artery remains unclear.

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We describe a 52-year-old man with a history of increasing fatigability and gait disturbances that were first attributed to hypothyroidism. On examination, he had bilateral pseudo-internuclear ophthalmoplegia with weakness of adduction and abducting nystagmus. Convergence was also impaired and he showed proximal weakness of the limb. Intravenous edrophonium almost completely abolished the nystagmus and adducting muscle weakness, and improved the strength of proximal muscles groups. The clinical response to the administration of edrophonium, the presence of AChR binding antibodes and the repetitive nerve stimulation test findings indicated that the patient had pseudo-internuclear ophthalmoplegia as a manifestation of generalized myasthenia gravis. Whereas hypothyroidism was effectively controlled with levothyroxine, his subsequent response to thymectomy, prednisolone and cholinesterase inhibitors confirmed the diagnosis of generalized myasthenia gravis.

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We encountered two patients with acute pandysautonomia who subacutely exhibited extensive autonomic dysfunction after antecedent infections. Although these patients had been suffering from autonomic disturbance for several months, they both had a good clinical course after plasma exchange and intravenous immunoglobulin therapy. Thin-layer chromatography (TLC)-immunostaining did not demonstrate any antibodies against gangliosides, but immunoblot analysis showed antibodies against a neuroblastoma cell line, SH-SY5Y, in serum samples. Furthermore, ganglionic acetylcholine receptor autoantibodies were detected in one patient. These findings suggest that neuronal antibodies against the autonomic nervous system play an important role in the pathogenesis of acute pandysautonomia.

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