Objective Proton pump inhibitors (PPIs) are widely used as the mainstay of treatment for erosive reflux esophagitis, based on the excellent results obtained in clinical trials. However, the endoscopic remission rates in patients treated with PPIs in actual clinical settings have not been fully assessed. We conducted a nationwide survey to evaluate the effectiveness of PPIs based on endoscopic findings in real-world clinical settings.
Methods This was a multicenter retrospective study conducted among 41 Japanese institutions. Endoscopic findings before and after PPI therapy were collected from the medical records of patients diagnosed with Los Angeles grade A-D reflux esophagitis who had been treated with a PPI for at least eight weeks before undergoing a second endoscopy. The remission rates of erosive esophagitis, defined as the percentage of patients with grade N or M findings following PPI therapy, were calculated.
Results Data for 541 patients were analyzed. At first endoscopy, 45.5%, 30.3%, 15.9% and 8.3% of patients were diagnosed with grade A, B, C and D esophagitis, respectively. The mean duration of PPI therapy was 410 days, and the mean remission rate was 61.6%. The remission rate was significantly lower in the patients with more severe erosive esophagitis based on the LA grade before PPI therapy.
Conclusion This study revealed that approximately 40% of the patients did not achieve remission of erosive esophagitis following PPI therapy for a mean of 1.1 years. This suggests the necessity of providing careful follow-up using periodic endoscopy and appropriately selecting PPI therapy.

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Objective It is important to detect post-liver biopsy hemorrhage early and confirm hemostasis in the clinical setting. Contrast-enhanced ultrasound (CEUS) is a sensitive and highly specific tool for detecting active bleeding. The aim of this study was to investigate the rate of detection of track microbubble flow signals on CEUS with perflubutane following liver biopsy and to assess the disappearance of these signals.
Methods Microbubble flow signals along the needle track on CEUS were examined in 100 patients who underwent percutaneous US-guided liver biopsies. The microbubble flow signals were examined repeatedly until their disappearance. The patients were followed up with clinical and laboratory data to detect clinically significant hemorrhaging.
Results Microbubble flow signals on CEUS following percutaneous liver biopsy were seen in 33% of the patients. There were no significant differences in the platelet count, prothrombin time or length of the biopsy specimen between the patients with and those without microbubble flow signals on CEUS. The microbubble flow signals disappeared over time in all patients. There were no cases of clinically significant hemorrhaging in the present study.
Conclusion Track microbubble flow signals on CEUS are frequently observed after biopsies. The disappearance of a microbubble flow signal is a useful index for confirming hemostasis of postbiopsy hemorrhaging.

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Objective We aimed to define the clinical features of liver dysfunction in patients with systemic lupus erythematosus (SLE).
Methods The frequency and causes of liver dysfunction were examined in 206 patients with SLE.
Results Liver dysfunction was evident in 123 (59.7%) of the 206 patients. Liver dysfunction in patients with SLE can be drug-induced (30.9%) or caused by SLE itself (28.5%), fatty liver (17.9%), autoimmune hepatitis (AIH) (4.9%), primary biliary cirrhosis (2.4%), cholangitis (1.6%), alcohol (1.6%) or viral hepatitis (0.8%), and it tends to be mild except when caused by AIH. Values for aminotransferase were significantly increased when AIH was the cause, whereas alkaline phosphatase (ALP) and γ-glutamyl transpeptidase (γ-GTP) were significantly increased when AIH or drugs were the cause. The liver was already dysfunctional at the time of SLE onset in 56 (45.5%) of 123 patients with liver dysfunction. Neurological involvement was more common among patients with than without liver dysfunction, whereas SLE activity and prognosis did not significantly differ between the two groups.
Conclusion Liver dysfunction in the presence of SLE can be caused by many factors, but when extant at the time of SLE onset, either SLE itself or drugs can be the cause. Autoimmune hepatitis should be considered when liver dysfunction is relatively severe.

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Objective Uric acid (UA) has shown to be a causal risk factor for the development and progression of renal disease. The aim of this study was to investigate the relationship between changes in the level of UA and trends in the renal function among hypertensive patients during a 10-year observation period.
Methods The subjects included 104 hypertensive outpatients (60 women and 44 men, mean age 60±9 (SD) years at the first visit) who had undergone at least five instances of successful 24-hour home urine collection, with the first examination completed between 1998 and 2000 and the last examination completed between 2008 and 2010.
Results The estimated glomerular filtration rate (eGFR) significantly decreased over the 10.4-year observation period, with an average change in eGFR of -0.66/mL/min/year. The uric acid clearance at the last examination was significantly lower than that observed at the first visit, while there were no significant differences in the serum UA levels during this period. The change in serum UA exhibited a significant negative correlation with the change in eGFR (r=-0.34, p<0.01). The patients whose UA level decreased more than 0.5 mg/dL during the observation period demonstrated significantly smaller declines in eGFR compared to those whose UA level increased more than 0.5 mg/dL. In the multivariate analysis, the change in serum UA and the average urinary salt excretion during the observation period were found to be significantly associated with the change in eGFR, independent of age, sex, BP changes or an increased number of antihypertensive drugs.
Conclusion Based on the findings observed over a 10-year observation period, increased UA is suggested to promote decline of the renal function in hypertensive patients. Controlling the level of UA as well as intensively restricting salt intake is required in order to preserve the renal function.

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Objective Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) is a minimally invasive technique with a high diagnostic yield used in the investigation of mediastinal diseases including sarcoidosis. Although previous reports have discussed the echoic features of metastatic mediastinal lymph nodes in lung cancer, few have addressed those features of mediastinal lymph nodes with sarcoidosis. We therefore investigated whether the echoic features of lymph nodes with sarcoidosis are distinct when compared to those of metastatic lymph nodes in lung cancer.
Methods This retrospective analysis was held in one university hospital between April 2007 and June 2011. EBUS-guided biopsies were performed on 219 patients, and thus resulting in sarcoidosis diagnoses in 53 patients. We quantitatively analyzed the echoic morphologic features of 42 lymph nodes from 34 sarcoidosis patients and 59 lymph nodes from 44 patients with lung cancer using digital image analyzing software.
Results In patients with sarcoidosis, 64.3% of the lymph nodes had a round shape, 71.4% had a distinct margin, and 88.1% exhibited homogeneous echogenicity. A germinal center structure was observed in 71.4% of the cases. In the context of shape and margin, no significant difference could be observed between sarcoidosis and lung cancer metastasis. However, homogeneous low echogenicity and the presence of a germinal center structure were observed in sarcoidosis more frequently than in lung cancer.
Conclusion Homogeneous low echogenicity and the presence of a germinal central structure may be distinctive echoic features of lymph nodes with sarcoidosis. Analyzing the echogenicity of the mediastinal lymph nodes may help to distinguish sarcoidosis from lung cancer.

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Objective Although Oshima, in the Kii Peninsula of Japan, is located within a high incidence area of amyotrophic lateral sclerosis (ALS) (Koza/Kozagawa/Kushimoto area, K area), no patients with ALS were detected between 1960 and 1999. However, the incidence recently increased between 2000 and 2009. On Oshima, the source of drinking water was changed from a regional river/wells to the Kozagawa River in the K area in 1975. We speculate that this change in water source may have played a role in the recent increase in the incidence of ALS. The aim of this study is to find contributing factors that may have triggered the locally high incidence of ALS.
Methods We investigated a possible association between the mineral content of drinking water and serum and oxidative stress markers among patients with ALS in the K area (K-ALS), residents of Oshima and controls.
Results We found that the levels of Ca and Zn in the recent drinking water in Oshima are low and that the serum levels of Ca and Zn in the Oshima residents and patients with K-ALS were significantly lower, while the oxidative stress markers were significantly higher, than those of the controls. The serum Zn and urinary 8-OHdG/creatinine levels explained 60% and 58% of the variations among the three groups, respectively. The serum Zn levels were negatively correlated with the serum Cu levels in the patients with K-ALS, and the serum Cu levels exhibited a tendency to be positively correlated with the 8-OHdG/creatinine levels in both the patients with K-ALS (r: 0.64) and the residents free from K-ALS (r: 0.32, p<0.01).
Conclusion Taken together, we suggest that the low levels of Ca and Zn in the drinking water are possibly associated with an imbalance of metal metabolism in Oshima residents and an increase in oxidative stress markers in patients with K-ALS, although the causative relationship is not clear. This is a cross-sectional study, and a prospective study is needed in the future.

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Objective Thrombocytopenia is frequently observed during bloodstream infection (BSI); however, little is known about the trends in platelet size during BSI. The aim of this study was to investigate trends in platelet indices during BSI and to determine the relationship between the mean platelet volume (MPV) levels and the prognosis of BSI patients.
Methods We conducted a four-year retrospective study to assess the trends in the platelet indices and the clinical features of BSI. We enrolled 350 patients with positive blood cultures and measured the platelet indices during five periods: 30 to seven days before onset (1st period); within one day of onset (2nd period); three to five days after onset (3rd period); seven to 10 days after onset (4th period); and 14 to 19 days after onset (5th period). The end point was defined as 30-day mortality.
Results Among the BSI patients, the average platelet count decreased during BSI (29.4 ^*10⁹/L to 24.0 ^*10⁹/L, p<0.001), while the average MPV level increased (7.33 fL to 7.89 fL, p<0.001). The degree of MPV elevation in the nonsurvivors (n=25) was lower than that observed in the survivors (n=325) between the 1st and 2nd periods (0.00 fL vs. 0.35 fL, p=0.006), whereas between the 2nd and 3rd periods, the degree of MPV elevation in the nonsurvivors was higher than that observed in the survivors (0.74 fL vs. 0.19 fL, p=0.03). MPV elevation after BSI was identified to be a negative prognostic factor for BSI (odds ratio: 1.82; 95% confidence interval: 1.00-3.32; p=0.027).
Conclusion Changes in the MPV levels after BSI may therefore be a useful prognostic marker for BSI.

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Clostridium difficile can cause pseudomembranous colitis (PMC). Antimicrobial agent exposure is a risk factor for Clostridium difficile-associated disease, whereas the use of antituberculous (anti-TB) agents is not. We herein report a case of PMC-associated with antituberculous therapy. A 63-year-old woman with tuberculous pericarditis treated with anti-TB agents was admitted for abdominal pain and diarrhea. On colonoscopy, mucoid exudate and yellowish plaque lesions were observed. The anti-TB agents were discontinued, and the patient was treated with metronidazole and clostridium butyricum. Her symptoms were relieved and did not recur when the anti-TB agents were restarted. In this report, we review the literature and discuss the pathogenesis, clinical manifestations, diagnosis and treatment of this case.

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Although the link between pulmonary arterial hypertension (PAH) and exposure to certain drugs has already been identified, we herein present the first case of herbal medicine-associated PAH in which the patient demonstrated spontaneous remission. A 38-year-old woman took the herbal medicine "bofutsushosan" for two weeks then stopped taking it due to exertional dyspnea. However, her dyspnea continued, and right heart catheterization revealed a mean pulmonary arterial pressure of 41 mmHg with a normal wedge pressure. Several months after treatment with oxygen therapy, the patient's dyspnea disappeared, and her pulmonary arterial pressure normalized. Further studies focusing on susceptibility factors to drug-induced pulmonary arterial hypertension are needed.

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We herein describe an unusual case of multicentric Castleman's disease accompanied by thrombocytopenia, ascites, renal failure and myelofibrosis in a Japanese woman. The patient was initially diagnosed as having myelodysplastic syndrome with myelofibrosis. The general condition of the patient deteriorated rapidly; however, treatment with tocilizumab, an anti-interleukin-6 receptor antibody, together with corticosteroids dramatically improved her symptoms. The clinical features of this case were similar to those of three cases previously reported by Takai et al. (Rinsho Ketsueki, 2010, 51:320-5), which were determined to be thrombocytopenia, anasarca, fever, reticulin myelofibrosis and organomegaly (TAFRO) syndrome, a possibly distinct clinical entity.

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Case 1. The laboratory findings of a hematological analysis of a 53-year-old woman with palpitations and dyspnea revealed the following: red blood cell (RBC) count: 9.4×10⁵ /μL with 60.0‰ reticulocytes; Hb: 3.7 g/dL; mean corpuscular volume (MCV): 124.5 fL; white blood cell (WBC) count: 2,800 /μL with 10.0% myeloblasts. Case 2. Similarly, a 42-year-old man with dizziness had a RBC count of 1.63×10⁶ /μL with 24.0% reticulocytes, an Hb level of 6.0 g/dL, an MCV of 120.2 fL and a WBC count of 3,100 /μL with 4.0% myeloblasts. Bone marrow aspirates in both patients confirmed a diagnosis of acute erythroid leukemia (AEL), which can present as marked macrocytic anemia with an MCV in excess of 120 fL and hemolysis.

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We herein report a case of polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) syndrome presenting with acute Guillain-Barré syndrome (GBS)-like features. The patient was healthy, except for mild dilated cardiomyopathy. She was unable to walk within ten days of the onset of weakness. A nerve conduction study (NCS) showed length-dependent, symmetric and non-focal demyelinating features with increased terminal latency indices (TLIs) in the lower limbs. Following the administration of intravenous immunoglobulin infusion, the proximal weakness of the lower extremities improved; however, the pleural effusion was aggravated and ascites newly developed. On further work-ups, splenomegaly, M-protein and sclerotic bone changes were observed. This case suggests that, although rare, POEMS syndrome can present with acute demyelinating polyneuropathy resembling GBS and that characteristic NCS features such as increased TLI and uniform demyelination are helpful for the early diagnosis of POEMS syndrome.

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A 34-year-old woman developed simultaneous bilateral severe optic neuritis and subsequent myelitis. Two months after the first attack, she developed a headache and dysesthesia in the left arm. Brain magnetic resonance imaging revealed multiple hyperintense lesions in the white matter of the right hemisphere, some of which were Baló-like concentric lesions. Our diagnosis was neuromyelitis optica spectrum disorder with Baló's concentric sclerosis (BCS), although the patient was negative for anti-aquaporin-4 (anti-APQ4) antibodies. Our case suggests that Baló's concentric sclerosis overlaps with neuromyelitis optica spectrum disorder and that this overlapping is caused by a mechanism that does not involve anti-AQP4 antibodies.

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We encountered a patient with the overlapping disorders of migraine with aura, migraine-triggered seizures and recurrent transient hemiparesis caused by atypical hemiplegic migraines with motor weakness during headache attacks, but not during the aura period, or paradoxical cerebral embolism. The patient displayed a giant Eustachian valve and patent foramen ovale, through which a spontaneous right-to-left shunt was revealed on transesophageal echocardiography. We considered that the overlapping disorders in the present case were closely related to the spontaneous right-to-left shunt caused by the giant Eustachian valve.

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A 59-year-old man presented with refractory anemia, choreoathetosis in the left upper extremity, an unsteady gait and cognitive dysfunction. The laboratory findings showed a marked decrease in ceruloplasmin. Magnetic resonance images revealed iron deposition in the brain and visceral organs. Iron accumulation was also observed in hepatocytes. Genetic analyses of the ceruloplasmin gene revealed a novel homozygous mutation of c.2185 delC in exon 12. The oral chelator deferasirox was effective in treating the left-side choreoathetosis and unsteady gait. Providing early treatment using deferasirox may be useful for preventing the progression of symptomatic neurological dysfunction.

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A chest X-ray of a young healthy African-American man with acute respiratory failure revealed bilateral multiple nodular shadows in the lungs, while community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) was detected in blood and sputum cultures. Magnetic resonance imaging showed osteomyelitis of the left thigh, and computed tomography revealed bilateral cavitary lesions in the chest, indicating necrotizing pneumonia with pulmonary embolism caused by osteomyelitis as a result of infection with CA-MRSA. CA-MRSA should be suspected as a causative agent of severe community-acquired pneumonia, even in Japan, among patients who belong to communities at high risk of CA-MRSA infection.

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A 25-year-old woman with a past history of congenital adrenal cortex hypertrophy visited our hospital complaining of a continuous cough. On chest CT, a localized bronchiectatic lesion was recognized with mucoid impaction in the right lower lobe. Because we obtained a positive smear test for acid-fast bacilli and polymerase chain reaction (PCR) of the bronchoscopic specimens was positive for M. avium, we administered combined chemotherapy; however, the clinical effect was poor and video-assisted thoracoscopic surgery (VATS) was performed. As the histological findings revealed a granuloma with caseating necrosis and mucous plugs containing M. avium in the respiratory bronchioles, we diagnosed the patient with mucoid impaction of the bronchi (MIB) due to M. avium.

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