Internal medicine
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Multicenter Study Clinical Trial
Polymyxin B-immobilized fiber column (PMX) treatment for idiopathic pulmonary fibrosis with acute exacerbation: a multicenter retrospective analysis.
The prognosis of idiopathic pulmonary fibrosis (IPF) patients with acute exacerbation (AE) is reported to be extremely poor. Several clinical studies suggest that direct hemoperfusion with polymyxin B-immobilized fiber (PMX) may have beneficial effects on AE in patients with interstitial pneumonia (IP). The aim of this multicenter retrospective analysis was to investigate whether PMX treatment could provide improvement of oxygenation and survival benefits in IPF patients with AE. ⋯ PMX treatment may improve oxygenation and survival in IPF patients with AE. Prospective, controlled trials of PMX treatment for IPF with AE are warranted to verify this potential benefit.
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Review Case Reports
Hypertrophic pachymeningitis and tracheobronchial stenosis in IgG4-related disease: case presentation and literature review.
Immunoglobulin G4 (IgG4)-related disease is a distinctive mass-forming disorder with frequent systemic involvement, most commonly in the pancreas, salivary glands and lacrimal glands. A few cases of dural involvement and one case of central airway stenosis have also been described. ⋯ We currently lack international standards for the diagnosis of extrapancreatic IgG4-related disease. Based on the findings of the present case and those reported previously, we discuss the distinctive features of IgG4-related pachymeningitis.
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Review Case Reports
Down-Klinefelter syndrome (48,XXY,+21) in a child with congenital heart disease: case report and literature review.
Congenital heart disease (CHD) is extremely rarely reported in 48, XYY, +21 karyotype. Herein, we reported one case of 48,XYY,+21 karyotype with CHD and reviewed the available literature. The phenotypic characteristics of the 4-month-old child showed the presence of features typical of mongoloid slant. ⋯ Doppler echocardiogram detection showed atrial septal and ventricular septal defects with patent ductus arteriosus, pulmonary hypertension and mild tricuspid regurgitation. Including this case, 63 cases of 48, XYY, +21 chromosome pattern have been reported. However, only 9 cases have CHD.
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A 55-year-old non-smoking woman was admitted to our hospital for re-evaluation of unimproved peripheral ground-glass opacities despite prednisolone and cyclosporine treatment. She was diagnosed with autoimmune pulmonary alveolar proteinosis (PAP) based on transbronchial lung biopsy and granulocyte/macrophage colony-stimulating factor (GM-CSF) antibody testing. ⋯ To avoid inappropriate immunosuppressant treatment, PAP should be considered in the differential diagnosis of such peripheral opacities. GM-CSF antibody might be useful for diagnosis.
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Multicenter Study
Validity and reliability assessment of a Japanese version of the Snaith-Hamilton pleasure scale.
Anhedonia is one of the main non-motor symptoms in Parkinson's disease (PD); it is assessed using the Snaith-Hamilton pleasure scale (SHAPS). To assess anhedonia in the Japanese population, we prepared a Japanese language version of SHAPS (SHAPS-J), and evaluated its validity and reliability in 8 neurological centers. Seventy subjects (48 patients with PD and 22 healthy subjects) were enrolled in this study. ⋯ These results indicate that SHAPS-J has good validity, test-retest reliability, and internal consistency, thus establishing an available measure of anhedonia in Japanese.