Objective Transnasal endoscopy was conducted to examine gastric mucosal damage in Japanese patients with ischemic heart disease who were receiving low-dose aspirin for preventing the onset or recurrence of cardiovascular disease.
Patients and Methods An endoscopist assessed gastric mucosal damage. Furthermore, the MOS 36-Item Short-Form Health Survey (SF-36^®) and the Gastrointestinal Symptom-Rating Scale (GSRS) were used to assess the outcomes of their quality of life (QOL) and the possible presence of gastric cancer and H. pylori infection.
Results Seventy-five patients were studied; and 24 (32.0%) and 16 (21.3%) of them concurrently received antithrombotic drugs other than aspirin and antiulcer drugs, respectively. Regarding gastric mucosal damage, 15 (20.0%) and 8 (10.7%) of the patients were endoscopically diagnosed with ulcer and hemorrhagic gastritis, respectively. Furthermore, 5 patients (6.7%) were found to have esophageal or gastric cancer. The positivity rate of Helicobacter pylori (H. pylori) was 45.3%. Patients receiving low-dose aspirin showed a decreased QOL. Consequently, no significant differences were found among the groups. Regarding endoscopic findings, no differences were found in the scores of both SF-36^® and GSRS with respect to the presence or absence of gastric ulcer, hemorrhagic gastritis, and H. pylori infection.
Conclusion Transnasal endoscopy was possible to perform during the oral intake of low-dose aspirin without causing any hemorrhagic complications. Many patients with gastric mucosal lesions showed no subjective symptom, and patients receiving aspirin were strongly recommended to undergo regular transnasal endoscopy, regardless of the presence or absence of symptoms.

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Objective To investigate the relationship between patients diagnosed with nodal generalized osteoarthritis (NGOA) and tissue antigens HLA-A and HLA-B in the Western Black Sea Region of Turkey.
Patients and Methods Forty-six patients with NGOA (64.74±8.46) and 60 controls (62.32±6.8) were included in the study. Inclusion criteria were: (i) stage 2 and higher OA of the hand and knee based on the Kellgren-Lawrence classification, and (ii) stage 2 and higher lumbar disc degeneration according to Lawrence classification. Family histories were taken from patients. HLA-A and HLA-B were typed by PCR using sequence specific primer.
Results The frequencies of HLA-A^*02 and HLA-B^*38 were 58.7% and 15.2%, respectively, in patients with NGOA, and there was a statistically significant relationship between the disease and HLA-A^*02 and HLA-B^*38. The relationship between positive family history and HLA-B^*44 allele was also statistically significant. In the control group, the frequency of HLA-A^*29 was 11.7% and it was statistically significant.
Conclusion To our knowledge this is the first study to demonstrate the epidemiologic association between HLA-A^*02 and HLA-B^*38 with NGOA in our population. We conclude that, HLA-B^*44 positivity may be associated with familial NGOA and HLA-A^*29 may be a preventive factor against NGOA.

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Objective This study aimed to evaluate the relevance of ratios of urinary potassium to urinary sodium + potassium (U_K/U_{Na + K}) to edema status in minimal-change nephrotic syndrome (MCNS).
Methods We retrospectively studied 26 adults with newly diagnosed MCNS with significant pitting edema. On the basis of mean value (0.46±0.21) of U_K/U_{Na + K} determined from spot urine samples on admission, patients were classified into 2 groups.
Results On admission, 12 of 26 patients had U_K/U_{Na + K} >0.46 (0.65±0.16, Group H), 14 patients had U_K/U_{Na + K} <0.46 (0.29±0.08, Group L). The level of serum albumin was similarly decreased in these 2 groups. Noteworthy were lower urine volume, fractional excretion of sodium (FENa), serum sodium, and higher hematocrit in the group H as compared with the group L. The group H had a shorter mean time required from onset of edema to hospitalization, and tended to have a longer mean time to complete remission than group L. High U_K/U_{Na + K} levels in group H decreased significantly after remission, eventually becoming equal to those of group L (0.24±0.05 vs. 0.25±0.05).
Conclusion U_K/U_{Na + K} determined from spot urine sample on admission relates to laboratory or clinical indices to distinguish edema status in adult patients with MCNS.

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Background The aim of this study was to evaluate the factors contributing to an accurate diagnosis of small (≤15 mm) peripheral pulmonary lesions (PPLs) by standard bronchoscopy and to determine the most suitable technology for such a diagnosis.
Objective Bronchoscopy was performed for 115 PPLs (≤15 mm diameter) on chest computed tomography (CT) between August 2003 and December 2006.
Methods Univariate and multivariate analyses were conducted retrospectively with the R software.
Results The diagnostic yield of the 115 PPLs was 65.2%; the yield was 61.9% and 69.2% for the malignant and benign lesions, respectively. In the univariate analysis, the approach to the lesion contributed the most to successful diagnosis, followed by skill and the use of hemostasis. In the multivariate analysis, the most important factor was approach, followed by lower lobe lesion and the use of hemostasis. Although it was better to use a sedative, operator skill was not a contributing factor.
Conclusion The approach to the lesion is the most important factor for a successful diagnosis of PPLs by bronchoscopy. Bronchoscopy is time consuming and painful; therefore, it is very important to establish an accurate diagnosis as soon as possible. Further, endobronchial ultrasonography with a guide sheath (EBUS-GS) and navigation systems are useful tools for the diagnosis of small PPLs.

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Background and Objective The appropriate target level for PaCO₂ after the introduction of long-term noninvasive positive pressure ventilation (NPPV) in patients with COPD remains uncertain, and therefore must be tested.
Methods Data on 54 patients with COPD receiving long-term domiciliary NPPV were examined retrospectively. PaCO₂ a few months after NPPV and potential confounders were analyzed with discontinuation of long-term NPPV as the primary outcome. The differences in annual hospitalization rates due to respiratory deterioration between those from 1 year before to 2 years after initiation of NPPV were compared according to the PaCO₂ measured at 6 months after NPPV (6-mo PaCO₂).
Results 6-mo PaCO₂ seemed to be most related to continuation of NPPV (p=0.019). Patients with 6-mo PaCO₂ of less than 60 mmHg had maintained a significantly lower PaCO₂ value 6 to 24 months after NPPV (p=0.04) and had a significantly higher continuation rate of NPPV (p=0.03) than those with a 6-mo PaCO₂ of 60 mmHg or more. Annual hospitalization rates due to respiratory deterioration were not associated with the 6-mo PaCO₂ level, but fatal hospitalization rates during the first year of NPPV were significantly correlated with relatively high 6-mo PaCO₂ (p=0.008).
Conclusion A relatively low 6-mo PaCO₂ value was predictive of long-term use of NPPV. The target values of 6-mo PaCO₂ may, therefore, be less than 60 mmHg in COPD patients with extremely severe hypercapnia, although more prospective studies are needed.

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A 26-year-old woman with an eating disorder presented to the emergency department with severe abdominal pain following binge eating. A plain film X-ray demonstrated a huge dilatation of the stomach with a high air-fluid level. Serum amylase was 2,265 IU/L, and serum lipase was 2,001 IU/L. Abdominopelvic computed tomography scan revealed a massive gastric dilatation and completely compressed duodenum. The distended right colonic loop and small bowel loops were reduced to the pelvic area and the displaced small bowel and mesenteries tightly pulled on the mesenteric vasculature. After nasogastric tube decompression and irrigation, her abdominal pain subsided. On the 15th day after admission, a follow-up abdominopelvic computed tomography scan demonstrated mild edematous changes of the pancreas compatible with pancreatitis.

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A 50-year-old man was referred to our hospital because of persistent atypical chest pain. His past medical history was remarkable for a non ST elevation myocardial infarction, treated five months previously with PCI on the right coronary artery. Two months later, for chest pain, he underwent coronary angiography that showed a right coronary artery with slight ectasia near the stent. Five months later for the persistence of atypical chest pain he came to our clinic. Chest CT showed a 31.5 mm hematoma of the right coronary artery. Coronary angiography revealed a giant aneurysm, proximal to the stent. In the light of rapid growth of aneurysm, the risk of rupture and symptoms, we decided to treat the aneurysm with covered stents. The patient underwent successful PCI with regression of symptoms.

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An 85-year-old woman with hypertension was referred to our hospital for the management of chest pain. Echocardiography showed left ventricular (LV) hypertrophy with impaired systolic function (ejection fraction, 40%) and mitral regurgitation. Pulsed Doppler echocardiography for checking mitral inflow showed triphasic mitral inflow velocity. Simultaneous recording of the LV and pulmonary artery wedge (PAW) pressures showed a high mean PAW pressure with a prominent v wave. The LV pressure showed a temporary elevation in early diastole; it was unusually decreased in mid-diastole, and finally was elevated to such an extent that it exceeded PAW pressure. In this patient, the mid-diastolic decrease in LV pressure, despite the presence of simultaneous LV filling flow, cannot be explained by conventional pressure-volume relation; rather it can be attributed to the abrupt increase in the inflow volume due to the impaired diastolic function. This case shows the characteristics of triphasic mitral inflow in patients with diastolic dysfunction.

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A 60-year-old man presented with drug-resistant hypertension with hypokalemia, a high plasma aldosterone concentration (PAC) and suppressed plasma rennin activity (PRA). Imaging examinations showed multiple macronodules in the left adrenal gland. Endocrinological findings demonstrated autonomous aldosterone secretion and ¹³¹I-adosterol scintigraphy demonstrated a left sided uptake. Laparoscopic left adrenalectomy normalized serum potassium levels and PAC, and substantially improved hypertension. Pathological and immunohistochemical analysis demonstrated that these nodules were positive for 3β-hydroxysteroid dehydrogenase (HSD3B) but not for CYP17. In addition, zona glomerulosa demonstrated "paradoxical hyperplasia", in which these cells were negative for HSD3B. All of these data indicated that the nodules in the left adrenal gland were mainly responsible for the autonomous aldosterone secretion. We conclude that the primary aldosteronism in this case was caused by multiple macronodules. This is a very rare case of primary aldosteronism caused by multiple adrenocortical macronodules.

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Renal involvement of mixed connective tissue disease (MCTD) shows systemic lupus erythematosus (SLE)-like immune complex glomerulonephritis. The prognosis of this condition is generally good. We report the case of an elderly female patient with MCTD who developed autoimmune pleurisy and rapidly progressive glomerulonephritis. Myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA) was positive with a titer of 59.0 EU. Anti-DNA antibody and complement levels were normal. Renal biopsy revealed crescentic glomerulonephritis and mild mesangial proliferation. However, immunofluorescence examination revealed immune-complex glomerulonephritis. These findings suggest that the renal involvement of MCTD developed concurrently with MPO-ANCA-related glomerulonephritis.

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Systemic capillary leak syndrome (SCLS) is a life-threatening disorder which presents with periodic episodes of hypovolemic shock, due to plasma leakage to the extra-vascular space reflected by accompanying hypoalbuminemia, hemoconcentration and edema often with associated monoclonal gammopathy. We describe a 28-year-old woman with SCLS who required aggressive fluid resuscitation and was successfully treated with corticosteroid, terbutaline, and theophylline. At exacerbation, the levels of serum granulocyte colony-stimulating factor (G-CSF) were increased. Thus, G-CSF might play an important role and can be a useful biomarker for the severity of attacks in SCLS.

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We report a case of acute respiratory distress syndrome (ARDS) after influenza A infection who was successfully treated with combined treatment including direct hemoperfusion with polymyxin B-immobilized fiber (PMX-DHP) column. A 56-year-old Japanese man was admitted to our hospital in January 2010 because of progressive dyspnea, hypoxemia, fever and bilateral diffuse infiltration on chest radiograph after pandemic influenza A infection. His chest computed tomography showed diffuse and patchy bilateral ground-glass opacities, and we diagnosed ARDS after influenza A infection. The patient was successfully treated with PMX-DHP in addition to the treatment with oseltamivir, corticosteroid, sivelestat and antibiotics with mechanical ventilation, and the patient recovered with only minor pulmonary fibrotic change. Although the efficacy of PMX-DHP treatment in patients with acute lung injury (ALI)/ARDS after influenza virus infection is not well established, this treatment could be a possible therapeutic modality in treating the patients with this disease.

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A 43-year-old-man was diagnosed as having primary mediastinal nonseminomatous germ cell tumor based on pericutaneous biopsy and an elevated serum concentration of alpha-fetoprotein (AFP). Although AFP decreased after chemotherapy, the mass size grew and developed tracheal compression by the tumor. The patient was treated with mechanical ventilation and subsequent stent implantation for keeping airway patency. After three cycles of chemotherapy and normalization of AFP, the increased mass was successfully resected and the pathological examination demonstrated a mature teratoma. This case showed a rare clinical manifestation of mediastinal growing teratoma syndrome and successful outcome by multimodal therapies.

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We report on the outcome of 4 patients with therapy-resistant systemic mast cell activation disease (MCAD) treated with the anti-IgE monoclonal antibody omalizumab in compassionate use. Two patients achieved an impressive persistent clinical response to treatment with omalizumab. In the third patient symptoms gradually improved. In the fourth patient omalizumab treatment had to be discontinued due to intolerable mast cell mediator-induced symptoms. In conclusion, omalizumab can lessen the intensity of the symptoms of systemic MCAD. Hence, omalizumab should be considered as a therapeutic option in cases of systemic MCAD that are resistant to evidence-based therapy.

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Primary hepatic lymphoma is a very rare condition, and the majority of the cases reported are of B-cell origin. We report a case of a 65-year-old man with primary hepatic peripheral T-cell lymphoma, not otherwise specified (PTCL-nos) who presented with 15% weight loss and general fatigue over the previous 9 months. Imaging studies and bone marrow examination could not confirm a diagnosis of lymphoma. Liver biopsy was performed because of an elevated soluble interleukin-2 receptor (sIL-2R) level (17,000 U/I) and hepatomegaly. After the diagnosis of primary hepatic PTCL-nos, treatment with low-dose corticosteroid was initiated, and the sIL-2R level decreased. Discontinuation of corticosteroid treatment resulted in the re-elevation of the sIL-2R level, and subsequently, treatment with low-dose corticosteroid was reinitiated. The sIL-2R level decreased rapidly, and the patient is alive with no evidence of lymphoma for 50 months after diagnosis. Thus, we found that a low-dose corticosteroid was effective in the long-term control of the disease, whereas many previous studies reported that primary hepatic PTCL-nos has a poor prognosis.

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A 46-year-old woman with Graves' disease was admitted for anemia and thrombocytopenia. She had previously been treated with methimazole but she self-discontinued the treatment 6 months prior to admission. She was diagnosed with Evans syndrome associated with Graves' disease and treated with propylthiouracil without corticosteroids, which normalized her thyroglobulin level. Surprisingly, while Evans syndrome is characterized by frequent relapses, this patient has been in remission of Evans syndrome for approximately 4 years. The remission of Evans syndrome associated with Graves' disease in the absence of immunosuppressive therapy suggests that these 2 diseases have a common pathogenetic mechanism.

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Tumor resection is recommended in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, however it is often difficult during an early stage of the disease. We report here the efficacy of early tumor removal in a patient with anti-NMDAR encephalitis. This 21-year-old woman was admitted to another hospital with rapidly progressive psychiatric symptoms, a decreased level of consciousness, and seizures. Abdominal CT showed a pelvic mass. On day 1 of admission to our center, she developed hypoventilation requiring mechanical support. She had orofacial dyskinesias with well-coordinated, pseudo-piano playing involuntary finger movements. Based on these clinical features, she was immediately scheduled for tumor resection on day 3. While awaiting surgery, she began to receive high-dose intravenous methylprednisolone. After tumor removal, she received plasma exchange, followed by intravenous immunoglobulin and additional high-dose methylprednisolone. Two weeks after tumor removal, she started following simple commands and progressive improvement, although she remained on mechanical ventilation for 10 weeks due to nocturnal central hypoventilation. Anti-NMDAR antibodies in serum/CSF were detected. Pathological examination showed immature teratoma with foci of infiltrates of B- and T-cells. Early tumor resection with immunotherapy facilitates recovery from this disease, but central hypoventilation may require long mechanical support. Non-jerky elaborate finger movements suggest antibody-mediated disinhibition of the cortico-striatal systems.

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We describe a 51-year-old man with Fisher syndrome (FS) and immune thrombocytopenic purpura (ITP) that developed after upper respiratory infection (URI). Laboratory investigations demonstrated immunoglobulin (Ig) G class of anti-GQ1b autoantibody and reduced platelet count with platelet-associated IgG, which spontaneously improved in parallel with neurologic symptoms. Thus the possible association of ITP should be considered when encountering a patient with FS. This patient suggests that there may be a certain infectious agent causing URI, leading to the co-occurrence of FS and ITP.

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Tardive dystonia is a side effect of dopamine receptor-blocking agents, which are mainly used as antipsychotic drugs. The treatment of tardive dystonia is difficult and often unsuccessful. An 82-year-old woman experienced mandibular deviation to the left due to spasm of the masticatory muscles with involuntary chewing movement and Parkinsonism. She had been treated with sulpiride for motility disorder for 5 years. Parkinsonism almost disappeared after the withdrawal of sulpiride, but tardive oromandibular dystonia showed no improvement. Aripiprazole treatment at 3 mg/day improved tardive oromandibular dystonia without worsening Parkinsonism. Low-dosage aripiprazole may be effective for tardive oromandibular dystonia in patients with no other psychiatric disorder.

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A 63-year-old woman receiving tumor necrosis factor (TNF) inhibitors for rheumatoid arthritis (RA) was found to have smoldering IgA-kappa type multiple myeloma (MM). Retrospective examination of stored serum samples revealed a steady increase of serum IgA levels after the start of TNF inhibitor therapy. The patient's articular symptoms showed marked exacerbation when TNF inhibitors were discontinued because of fear of worsening the MM. Tocilizumab improved RA symptoms dramatically and stabilized serum IgA levels for 13 months after a transient steep rise. This case suggests that tocilizumab can be used safely in patients with inflammatory disorders with coexisting MM.

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We describe an 18-year-old woman infected with H1N1 influenza followed by thrombotic microangiopathy. During the acute phase, her plasma levels of von Willebrand factor (VWF) were remarkably elevated, whereas those of ADAMTS13 were reduced without its inhibitors, generating a markedly high ratio of VWF to ADAMTS13 in circulation. A retrospective analysis established the following hypothesis: an influenza-mediated cytokine storm induced an enhanced release of unusually large VWF multimers (UL-VWFM) from vascular endothelial cells, generating platelet thrombi in microcirculatures under high shear stress. Plasma exchange removed UL-VWFM and cytokines, and rescued her life. This report sheds a light on a hitherto unrecognized influenza complication.

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A 44-year-old man was referred to our hospital because of persistent high fever. Both CT and PET-CT demonstrated lymph node lesions limited to the mediastinal region without cervical lymphadenopathy. Histology of a mediastinal lymph node obtained by video-assisted thoroscopic excision confirmed the diagnosis of histiocytic necrotizing lymphadenitis. To our knowledge, this is the first report of Kikuchi-Fujimoto disease with isolated mediastinal lymphadenopathy. Although Kikuchi-Fujimoto disease is rare, we should consider this disease in patients with a high fever and no other symptoms.

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