Internal medicine
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Type 1 autoimmune pancreatitis (AIP) is characterized by a high serum IgG4 concentration and is closely associated with the HLA-DRB1(*)04:05-DQB1(*)04:01 haplotype, for which family studies may disclose its immunogenetic significance. In the present study, we encountered two male siblings with type 1 AIP who exhibited diffuse pancreatic swelling with a capsule-like rim and diffuse pancreatic duct stricture. The younger brother also displayed characteristic IgG4-related sialadenitis and retroperitoneal fibrosis. Contrary to our expectations, the siblings showed only normal or slightly elevated values of serum IgG4 and no HLA DRB1(*)04:05-DQB1(*)04:01 haplotype, suggesting that type 1 autoimmune pancreatitis is associated with multiple immunogenetic factors.
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Case Reports
Pulmonary tumor thrombotic microangiopathy with circulatory failure treated with imatinib.
Pulmonary tumor thrombotic microangiopathy is a lethal, yet difficult to diagnose, complication of gastrointestinal carcinoma. Even if properly diagnosed, there is no treatment, especially after a circulatory collapse. ⋯ Pulmonary hypertension was dramatically ameliorated and the patient was able to be weaned from percutaneous cardiopulmonary support within 20 days of treatment. Imatinib may be effective for ameliorating pulmonary hypertension that is caused by pulmonary tumor thrombotic microangiopathy.
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To assess longitudinal changes in the clinical features of patients with amyotrophic lateral sclerosis (ALS), we performed a retrospective hospital-based study covering 35 years. ⋯ Our findings demonstrate that, among patients with sporadic ALS, the age at disease onset, the proportion of patients with disease onset at 70 years of age or higher, the proportion of patients with dementia and the proportion of patients with bulbar onset ALS have increased significantly over the past 35 years. The longitudinal changes observed in the clinical features of ALS may reflect the increasing age at disease onset.
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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. ⋯ Changes in the MPV levels after BSI may therefore be a useful prognostic marker for BSI.
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Case Reports
Papillary squamous cell carcinoma of the trachea associated with human papillomavirus-18 infection.
A 75-year-old man presented with the chief complaints of coughing and dyspnea. Chest computed tomography revealed narrowing of the trachea. ⋯ Multiplex polymerase chain reaction confirmed human papillomavirus type 18 DNA in the tumor. At the 12-month follow-up visit following the administration of radiotherapy, the patient exhibited no local recurrence or distant metastasis.