Objective The aim of this prospective clinical study was to evaluate the efficacy and safety of long-term proton pump inhibitor (PPI) treatment for two years in Japanese patients with reflux esophagitis (RE).
Methods The efficacy and safety of two-year (104-week) treatment with rabeprazole (RPZ) 10 mg were studied in patients confirmed to have been cured of RE by PPI and who required long-term maintenance therapy with PPI. We performed serial endoscopy, checked gastroesophageal reflux disease (GERD) symptoms, adverse events, laboratory values and serum gastrin. We also monitored gastric mucosal histology, atrophy and polyps.
Results The endoscopic non-relapse rate for RE was 87.3% for the 104-week period. GERD symptoms improved based on the fact that the mean change from baseline in GERD symptom score after treatment was a negative value. Treatment was safe; and atrophy was found to have developed in virtually no cases. A few new benign fundic gland or hyperplastic polyps developed throughout the study, but no ECL carcinoids were found to have developed. Serum gastrin levels tended to increase up to 24 weeks, but there were no subsequent changes thereafter up to 104 weeks.
Conclusion The results confirmed oral RPZ 10 mg to be effective for maintenance therapy in Japanese patients with RE. Although effects on the gastric mucosa were not ruled out, long-term use of RPZ was confirmed to be safe overall.

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Objective Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) occurs during the chronic progressive course of idiopathic pulmonary fibrosis. Mortality is estimated to be >70%, because no effective treatment has been established. We evaluated the effectiveness of combination therapy of tacrolimus and methylprednisolone for AE-IPF.
Methods Patients of AE-IPF treated with methylprednisolone pulse therapy with or without tacrolimus (targeting 20 ng/mL) during the period between January 2001 and April 2010 were retrospectively reviewed. The primary endpoints were survival rate and duration. We also observed lactate dehydrogenase levels, partial pressure of arterial oxygen/fraction of inspired oxygen ratio (P/F ratio), KL-6, occurrence of re-exacerbation, and computed tomography score.
Results Fifteen Japanese patients [tacrolimus group aged 74.2±6.0 years old (n=5), non-tacrolimus group aged 75.1±12.8 years old (n=10)] were identified. Pre-treatment clinical parameters were not significantly different between the two groups. Four of 5 tacrolimus group patients and 1 of 10 non-tacrolimus group patients survived (p<0.05). The median survival durations were >92 days (tacrolimus group) and 38 days (non-tacrolimus group) (p<0.05). Lactate dehydrogenase levels and the P/F ratio were also significantly favorable in the tacrolimus group. KL-6 and CT score were not significantly different in both groups. Four re-acute exacerbations were observed only in the non-tacrolimus group.
Conclusion Combined tacrolimus and methylprednisolone pulse therapy mitigates AE-IPF, prevents re-acute exacerbation, and contributes to a better prognosis.

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Objective This study aimed to identify factors associated with the functional outcomes of patients with cerebral embolism due to nonvalvular atrial fibrillation.
Methods We retrospectively investigated the short-term functional outcomes of 134 patients diagnosed with cardiogenic cerebral embolism due to nonvalvular atrial fibrillation during the period of May 2006 to August 2008. Functional state was evaluated using the modified Rankin Scale (mRS) on admission and at discharge.
Results A good functional outcome (mRS ≤2) at discharge was significantly associated with low mRS on admission (OR: 0.07; CI: 0.03-0.18; p<0.001), and a low C-reactive protein (CRP) level (OR: 0.19; CI: 0.04-0.89; p<0.05).
Functional improvement during admission was positively associated with the presence of dyslipidemia (OR: 2.74; CI: 1.11-6.76; p<0.05), whereas high diastolic blood pressure (OR: 0.95; CI: 0.90-0.99; p<0.05) and a high blood sugar level (OR: 0.98; CI: 0.97-0.99; p<0.05) on admission were inversely associated with functional improvement. Furthermore, no relationship existed between mRS on admission and functional improvement during hospitalization.
Conclusion The results suggest that a good functional state at discharge was associated with a good functional state on admission as well as a low serum CRP level. On the other hand, functional improvement was associated with the presence of dyslipidemia, low diastolic blood pressure, and low blood sugar level on admission.

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Objective To compare serum folate levels between atopic asthmatics, non-atopic asthmatics, and healthy controls.
Methods This case-control study included 60 asthmatics with at least one positive skin prick test (SPT) reaction (atopic asthma group), 60 asthmatics with negative SPT reactions (non-atopic asthma group), and 60 healthy controls with no history of asthma or other allergic diseases, and with negative SPT reactions. Serum folate and total IgE levels were measured in all subjects. In addition, lung functions were assessed by spirometry.
Results Serum folate levels were significantly lower among the atopic asthma group [9.1 (4.9, 12.1) ng/mL] as compared to the non-atopic asthma group [11.3 (7.5, 14.8) ng/mL] and the control group [12.0 (8.3, 15.1) ng/mL], p= 0.001. Among atopic asthmatics, serum folate levels were inversely correlated with total serum IgE levels (r=-0.483, p<0.001), and the number of positive SPT reactions (r=-0.442, p<0.001). Atopic asthmatics with a total serum IgE ≤200 IU/mL had significantly higher levels of serum folate than those with a total serum IgE >200 IU/mL. Regression analysis showed that higher folate levels independently predicted lower total serum IgE levels. Folate was not found to be an independent predictor of asthma. No association was observed between serum folate levels and values of forced expiratory volume in 1s.
Conclusion Among asthmatics, serum folate levels are significantly lower among atopics, and correlate inversely with the degree of atopy.

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Objective Carnett's test is a simple clinical test in which abdominal tenderness is evaluated while the patient tenses the abdominal muscles. It is useful for differentiating abdominal wall pain from intra-abdominal pain. However, no study has reported its association with psychogenic abdominal pain. We evaluated its diagnostic usefulness in psychogenic abdominal pain.
Methods Two physicians performed Carnett's test on each patient, but only one received the medical history. The other physician only conducted the test. Based on the final diagnosis, patients were categorized into 3 groups: psychogenic pain, abdominal wall pain, or intra-abdominal pain. Each group was analyzed in association with the results of Carnett's test conducted by the blinded physician.
Patients A total of 130 outpatients with the chief complaint of abdominal pain who had abdominal tenderness.
Results There were 22 patients with psychogenic abdominal pain, 19 with abdominal wall pain and 62 with intra-abdominal pain. In patients with psychogenic pain or abdominal wall pain, Carnett's test was usually positive, whereas the test was usually negative in patients with intra-abdominal pain (p<0.001, respectively). The positive likelihood ratio of Carnett's test for psychogenic abdominal pain was 2.91 (95% confidence interval [CI], 2.71-3.13), while the negative likelihood ratio was 0.19 (95% CI, 0.11-0.34). The corresponding values for abdominal wall pain were 2.62 (95% CI, 2.45-2.81) and 0.23 (95% CI, 0.13-0.41), respectively.
Conclusion Carnett's test may be useful for ruling in and ruling out psychogenic abdominal pain in addition to distinguishing between abdominal wall pain and intra-abdominal pain.

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Wegener's granulomatosis (WG) is a multisystemic disease of unknown etiology characterized by necrotizing vasculitis and granulomatous inflammation (1-3). The disease typically involves the upper airways, lungs and kidneys, and gastrointestinal involvement is uncommon. Described here is a 33-year-old man who presented at the hospital with abdominal pain. Colonoscopy revealed multiple ulcers, including round ulcers, throughout the large intestine. Small bowel ulcers were detected by double balloon enteroscopy (DBE). Further study confirmed that these ulcers were caused by gastrointestinal complications of WG. The patient was administered prednisolone and cyclophosphamide and remains in remission. This case indicates the importance of considering a gastrointestinal complication of WG as the potential cause of abdominal symptoms among WG patients as well as the use of DBE in detecting such a complication.

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Since its clinical definition in 1987, the syndrome called, "periodic fever, aphtous stomatitis, pharyngitis and cervical adenitis" syndrome (PFAPA) has been considered peculiar to pediatric age. In the recent literature there are a few case reports of PFAPA in adults. We describe a case of a 21-year-old female affected by PFAPA who presented a history of tonsillectomy at the age of four. To our knowledge this is the fourth case described with a diagnosis of PFAPA in an adult with a history of tonsillectomy during childhood. Although the role of tonsillectomy in the treatment of PFAPA is still controversial, due to the lack of definitive data in literature, this case suggests that fever episodes may relapse several years after surgery.

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A 52-year-old man was evaluated for incidentally discovered bilateral adrenal masses. He had drug-resistant hypertension but lacked Cushingoid features. Endocrinological tests revealed autonomous secretion of cortisol and aldosterone with suppressed plasma ACTH and renin activity. A selective adrenal venous sampling demonstrated aldosterone hypersecretion from the left adrenal vein. The clinical diagnosis of subclinical Cushing's syndrome due to ACTH-independent macronodular adrenal hyperplasia (AIMAH) associated with primary aldosteronism was made, and he underwent left adrenalectomy; the resected adrenal lesion was consistent with the pathological diagnosis of AIMAH coexistent with aldosterone-producing adenoma (APA). This is a very rare case of AIMAH with concomitant unilateral APA, whose hypertension improved after surgery.

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We report a case of membranous nephropathy with primary biliary cirrhosis (PBC). A 76-year-old Japanese man had been treated for PBC and was referred to our department because of acute severe proteinuria (10 g/day). Renal biopsy was performed and was compatible with a diagnosis of membranous nephropathy. The patient was treated with cyclosporine A (CsA; Neoral^®), which was followed by normalization of liver function and partial remission of proteinuria (0.68 g/day) within six months without any significant side effects. This case suggests that CsA monotherapy is effective for the treatment of both liver dysfunction and membranous nephropathy associated with PBC.

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Congenital coronary artery fistula (CAF) with huge coronary artery aneurysm is a very rare condition. In this paper, we describe a 26-year-old asymptomatic male patient with right coronary artery (RCA) to the left ventricle fistula with a huge coronary artery aneurysm which was diagnosed by multidetector computed tomography and coronary angiography. The patient received surgical treatment for coronary artery after diagnosis. Both RCA and a giant aneurysm were excised; surgical closure of CAF and coronary artery bypass grafting were performed on this patient. Two months after surgery, the enlarged left ventricle returned to normal as evaluated by echocardiography.

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A 60-year-old woman was found to have proteinuria and a lung nodule. The surgically resected left upper lobe contained a nodule, in which the adenocarcinoma was surrounded by a heavy deposition of amyloid. Subsequent renal and gastric biopsies demonstrated amyloid deposition with Aλ immunoreactivity. She was treated with 2 courses of VAD (vincristine, doxorubicin and dexamethasone), resulting in the disappearance of Bence Jones proteinuria. Her nephrotic syndrome has been improving during the subsequent 3 years. The rare lung nodule consisting of adenocarcinoma and amyloid deposition was a diagnostic clue in this primary systemic AL amyloidosis patient.

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Only a few pathologic reports exist describing adult onset Still's disease (AOSD) with pulmonary involvement. We report this very rare case of AOSD complicated with cryptogenic organizing pneumonia (COP). A 32-year-old woman was referred with high spiking fever, salmon-pink rash in her arms and legs, and polyarthralgia. The laboratory data showed marked increases in white blood cell count, an erythrocyte sedimentation rate, and C reactive protein, ferritin, and liver dysfunction. All cultures remained negative, as were autoantibodies and rheumatoid factor. The patient was strongly suspected of AOSD according to specific diagnostic criteria. However, chest X ray disclosed an infiltrative shadow accompanied by air bronchogram in the upper lobe of the right lung and therapy with antibiotics was initiated. As the patient did not respond to antibiotics and a remittent fever of over 38°C, a flexible bronchoscopy was performed. Organizing pneumonia was diagnosed by transbronchial lung biopsy (TBLB) histology and radiologically, and the lesions were thought to be due to pulmonary involvement of AOSD. Therefore, she was diagnosed with AOSD complicated with COP. Oral treatment with prednisolone (30 mg/day) resulted in rapid disappearance of the infiltrative shadow. Symptoms and markers of inflammation also improved. Clinicians should be aware that COP can be a complication of AOSD.

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A 66-year-old woman with seropositive rheumatoid arthritis (RA) and latent tuberculosis infection developed minimal-change nephrotic syndrome following the initiation of anti-tuberculosis chemoprophylaxis with isoniazid. This is the first reported case of an isoniazid-induced nephrotic syndrome. Isoniazid as a single-drug intervention is widely accepted as a safe and effective means of anti-tuberculosis chemoprophylaxis, particularly for RA patients with latent tuberculosis infection; the present case, however, demonstrates that isoniazid has the potential to induce minimal-change nephrotic syndrome, even when used as a single-drug intervention.

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