Objective: We investigated the long-term outcome of autoimmune pancreatitis (AIP) including morphological changes in the pancreas, pancreatic duct, biliary tract, pancreatic function, and changes in the clinical manifestations after oral prednisolone (PSL) therapy.
Patients and Methods: We prospectively followed 12 patients for a period of over 12 months (median follow-up period: 41 months; range: from 13 to 133 months). All twelve patients were treated with PSL. The morphological findings consisted of pancreatic enlargement (n=12), an irregularly narrowed main pancreatic duct (n=12), and bile duct stricture (n=10), and salivary gland swelling was observed in six patients. The initial dose of PSL was 30-40 mg/day, and it was subsequently tapered.
Results: All 12 patients responded to PSL therapy. The enlargement of the pancreas and the irregularly narrowed main pancreatic duct improved to almost normal. Pancreatic atrophy developed in four of them (4/12, 33%), but no pancreatic calcification was observed in any of the patients. The bile duct stricture improved to various degrees in all 10 patients , but it persisted in the lower part of the bile duct in four of them (4/10, 40%). The salivary gland swelling also improved after PSL therapy. There was no recurrence of enlargement of the pancreas or irregularly narrowed main pancreatic duct after PSL therapy, but the bile duct stricture recurred in one case, and in three cases there was a relapse of salivary gland swelling that required a temporary increase in PSL dose during tapering. No deterioration of pancreatic exocrine function was detected in any of the patients. A malignant tumor was diagnosed in two patients during PSL therapy: early gastric cancer in one and rectal cancer in the other. All patients are alive.
Conclusions: AIP treated with PSL has a favorable long-term outcome based on the morphological findings and assessments of pancreatic function. However, since two of the twelve patients developed a malignancy during PSL therapy, strict follow up should be part of the management of AIP.

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Objective: The aim of this study was to evaluate the influence of conventional cardiovascular risk factors on the degree of adaptive response of the carotid arterial wall to atherosclerotic disease.
Patients and Methods: We evaluated the diameter and intima-media thickness (IMT) of common carotid artery (CCA) by ultrasonography in 351 men aged 70.3 (range, 14-97) years and 474 women aged 75.6 (range, 19-103) years in the medical department of Seiyo Municipal Nomura Hospital. We assessed cross-sectionally the relationships between CCA diameter and IMT and cardiovascular risk factors by gender.
Results: In multiple linear regression analyses, after controlling for traditional cardiovascular risk factors, a significant correlation was found between CCA diameters and age (p=0.034), body mass index (BMI) (p<0.001), smoking status (p=0.039), alcohol consumption (<0.001) and uric acid (UA) (p=0.021) in men, and between CCA diameters and age (<0.001), BMI (p<0.001), systolic blood pressure (SBP) (p=0.013) and antihypertensive drug use (p=0.005) in women. Analysis of covariance showed that the two regression lines between carotid IMT and diameter in those with or without plaque were significantly different in both men (F=16.4; p<0.001) and women (F=15.0; p<0.001). After adjustment for carotid IMT and plaque, associations with carotid diameters still persisted for age (p<0.001), BMI (p<0.001), smoking status (p=0.006), alcohol consumption (p<0.001) and SBP (p=0.001) in men, and age (p=0.005), BMI (p<0.001), SBP (p=0.047) and UA (p=0.001) in women.
Conclusion: This study shows that the CCA diameters correlated with conventional cardiovascular risk factors including alcohol consumption. These findings suggest that the CCA diameters may reflect the ability of adaptive remodeling to the atherosclerosis before plaque formation and can be an important factor during the development of atherosclerosis.

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Objectives: To examine the factors related to actual patient recruiters among the physicians who initially agreed to collaborate in a randomized control trial.
Methods: We conducted a questionnaire survey of 679 physicians (512 actual recruiter and 167 non-recruiters) who had initially agreed to recruit patients for a clinical trial to determine factors to predict who would actually do so.
Results: Response rates among recruiters and non-recruiters were 87.5% and 73.1%, respectively. Multivariate logistic regression model showed that the proportions of regular users of computer [odds ratio (OR) =2.1, 95% confidence intervals (CI)=1.3-3.3] (p=0.002) and current participants in other clinical trials (OR=2.2, CI=1.5-3.4) (p=0.001) were significantly higher among recruiters than non-recruiters. Patients' reasons for non-participation as perceived by the physicians did not differ between recruiters and non-recruiters.
Conclusion: Results of this study might be useful in predicting actual recruiters at the outset of clinical trials.

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The patient was a 63-year-old woman with attacks of fever and abdominal pain, starting from the age of 53 years and recurring every month. Despite various examinations at another hospital, the etiology remained unclear. She was under symptomatic treatment, and was referred to our department for further evaluation. Although she had onset in middle age, the clinical symptoms and examination findings suggested familial Mediterranean fever, and administration of colchitine inhibited the attacks completely. Therefore, the patient was diagnosed as having the disease. We were not able to analyze the entire MEFV gene, but detected only a heterozygous M694I mutation. Amyloidosis did not develop as a complication. The disease is rare in Japan, and its onset in the fifties is extremely rare in the world.

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Insulinoma is the most common cause of fasting hypoglycemia resulting from autonomous insulin hypersecretion. We describe herein a unique case with paradoxical hypoglycemic episodes induced by hyperglycemia. A 55-year-old female had repeated hypoglycemic episodes after meal or during increased physical activity. Although fasting (10 hr) failed to provoke hypoglycemia, oral glucose tolerance test (GTT) caused an exaggerated insulin response (885 μU/ml) at 30 min, followed by hypoglycemia (36 mg/dl) after 90 min. Moreover, intravenous GTT also induced an exaggerated insulin response (>2900 μU/ml) at 10 min, followed by hypoglycemia (34 mg/dl) after 40 min. Although MRI and CT scan of the abdomen failed to detect any mass lesions in the pancreas, Octreoscan revealed increased radioactive uptake around the pancreatic head region. Treatment with a daily injection of octreotide (100 μg) alleviated her hypoglycemic episodes. At surgery, two islet cell adenomas were identified in the pancreas and resected. Postoperatively, she was free from hypoglycemic episodes after meal. Postoperative oral and intravenous GTT did not induce hypoglycemia. Thus, this is a very rare case of glucose-responsive and octreotide-sensitive insulinoma in whom GTT and octreotide proved to be a useful provocation and treatment for hypoglycemic episodes.

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A 54-year-old man with a past history of multi-operation for solitary fibrous tumor was hospitalized for hypoglycemia. Computed tomography demonstrated multiple tumors in the liver but not in the pancreas. His serum insulin-like growth factor (IGF)-II level was normal, but immunoblot analysis and immunohistochemistry revealed a high molecular weight form of IGF-II in the serum and tumor. Surgical resection was impossible, because of tumor dissemination. Hypoglycemia was repeated despite infusion of glucose and glucagon. Glucocorticoid dramatically and continuously abolished hypoglycemia. In cases of inoperable IGF-II-producing tumor, glucocorticoid therapy may be promising for hypoglycemia.

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A 30-year-old woman who showed remission of membranoproliferative glomerulonephritis (MPGN) 20 years previously developed membranous lupus nephritis (MLN). She had photosensitivity, facial erythema, proteinuria of 2.59 g/24 hr, anti-nuclear antibody and anti-ds-DNA antibody. To confirm whether a misdiagnosis of MPGN was made 20 years ago, the clinical data at that time were evaluated retrospectively. She had only mild proteinuria and hematuria but no photosensitivity or facial erythema. Anti-nuclear antibody was negative. Renal biopsy showed occasional lobulation and glomerular capillary double contour. The diagnosis of MPGN was definite. This might be a rare case of one person suffering from two types of glomerulonephritis, MPGN and MLN.

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A 47-year-old Japanese woman was admitted to our hospital with a 2-week history of dry cough and shortness of breath. She had been engaged in Enoki mushroom production for 22 years. Chest X-ray and chest computed tomography (CT) scan showed bilateral fine-nodular shadows and ground glass opacity. Bronchoalveolar lavage fluid demonstrated an increase of total cell counts with predominant lymphocytosis. Pathological specimens obtained by video-assisted thoracoscopic surgery revealed alveolitis and noncaseating granuloma with giant cells. Lymphocyte stimulation test showed positive responses with Enoki mushroom, culture medium, and Penicillium citrinum. On double immunodiffusion test, a precipitation line was observed between patient's serum and Penicillium citrinum antigen. She was found to have hypersensitivity pneumonitis caused by Penicillium citrinum. This is the first report of mushroom worker's lung caused by Penicillium citrinum.

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The patient was a 57-year-old man with a chief complaint of anterior chest pain who was diagnosed with clinical stage IV (c-T2N2M1) non-small-cell lung cancer (adenocarcinoma). Tenderness in the sternoclavicular joint, acne, periodontitis, and palmoplantar pustulosis were evident, and SAPHO syndrome was diagnosed. SAPHO syndrome is a rare disorder that results in synovitis, acne, pustulosis, hyperostosis, and osteomyelitis. Bone scintigraphy showed tracer accumulation in the costal cartilage, sternoclavicular joint, and cervical vertebrae 6-7. Although the bone lesions of SAPHO syndrome were difficult to differentiate from bone metastasis of pulmonary adenocarcinoma, metastatic bone tumors were ruled out by magnetic resonance imaging, computed tomography, and fluorodeoxyglucose positron emission tomography. There have been no previously reported cases of lung cancer with comorbid SAPHO syndrome. We report such a case and discuss the relevant literature, particularly that concerned with the evaluation of bone lesions.

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A 38-year-old Japanese man with severe aplastic anemia had invasive pulmonary aspergillosis as a complication. He was treated with amphotericin B for six weeks, but the aspergillosis did not improve. Then he experienced a fatal myocardial infarction. An autopsy revealed disseminated aspergillosis involving pericarditis and Aspergillus embolization to the coronary arteries. This led to the acute myocardial infarction. Cardiac aspergillosis is rare, but should be included within the differential diagnosis when chest pain of unknown origin occurs in an immunosuppressed patient.

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Bilateral vestibular dysfunction is a rare condition, of which peripheral disorders are most common, whereas central disorders are extremely rare. A 35-year-old woman developed fever, headache, dizziness, convulsion, and disturbance of consciousness at the same time. MRI findings were normal. Based on the neurological findings and laboratory abnormalities, she was diagnosed as having aseptic meningoencephalitis. Steroid pulse therapy successfully ameliorated her encephalitis sign. However, isolated bilateral vestibular ataxia and dizziness, together with severe decrease of Caloric nystagmus, became apparent and lasted for the following 3 months, without cerebellar/brainstem involvement.

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We report a case in which convergence movement was substituted for bilateral horizontal gaze palsy due to a pontine tegmental hemorrhage. When visual fixation was suppressed with Frenzel goggles (FG), substituted convergence movements were prominent in our patient. The observation suggests that both vergence eye movements and horizontal gaze movements act synergistically and lesioning of the version system results in overactivity of the convergence system after a pontine hemorrhage. Furthermore, it may be worth using FG in the observation of substituted eye movements in patients with horizontal gaze palsy due to a pontine tegmental injury.

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A 75-year-old man was transferred to our department because of development of severe renal impairment after coronary artery bypass grafting. Hemodialysis was initiated for postsurgical oliguria and lung congestion. On transfer, he showed systemic purpura rashes and diffuse blue mottlings on his toes with marked eosinophilia and an elevated level of C-reactive protein. Cutaneous biopsy revealed cholesterol crystal embolism and leukocytoclastic vasculitis in dermal arterioles. Myeloperoxidase-anti-neutrophil cytoplasmic antibody titer was increased. Upon oral corticosteroid therapy following intravenous pulse steroid therapy, the purpura dramatically diminished, renal function improved, and hemodialysis was discontinued. Active treatment with corticosteroids may be effective for cholesterol embolization syndrome, particularly when clinical and laboratory manifestations mimic systemic vasculitis.

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A 49-year-old woman with amyotrophic sequelae of poliomyelitis experienced progressive left upper limb weakness and breathing discomfort while walking that had developed over one year prior to presentation. She had flaccid quadriplegia which was more marked in the left upper limb, with the C4- and C5-innervated muscles being most severely affected. Chest radiographs double exposed at maximal inspiration and expiration revealed poor respiratory movements in the left hemidiaphragm. Phrenic nerve conduction study demonstrated conspicuous diaphragmatic paralysis on the left side. The electrophysiological study supports radiological findings and provides useful evidence for insidious phrenic nerve involvement in postpolio syndrome.

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