Internal medicine
-
Comparative Study
Analysis of serum IgG subclasses in Churg-Strauss syndrome--the meaning of elevated serum levels of IgG4.
Mikulicz's disease (MD) is characterized by symmetrical and persistent enlargement of the lacrimal and salivary glands. Recently it has been categorized as an 'Ig (immunoglobulin) G4-related disease.' It presents with elevated serum levels of IgG4 and abundant infiltration of IgG4-bearing plasmacytes in involved organs. Allergic symptoms are often observed in patients with IgG4-related disease. On the other hand, allergic diseases are often complicated with Churg-Strauss syndrome (CSS). Here we focused on CSS and analyzed the relation of IgG4 in its pathogenesis. ⋯ IgG4-bearing plasmacytes may be involved in the pathogenesis of CSS, and it is possible that an allergic reaction plays an important role in the pathogenesis of IgG4-related disease.
-
Case Reports
A distinct phenotype of leg hyperreflexia in a Japanese family with Gerstmann-Sträussler-Scheinker syndrome (P102L).
Gerstmann-Sträussler-Scheinker Syndrome (GSS) is an inherited prion disease characterized by midlife onset and slowly progression of cerebellar ataxia and dementia. We report a distinct phenotype of leg hyperreflexia in a Japanese family with GSS. A 38-year-old woman noticed unsteady gait at 33 years of age. ⋯ Phenotypic hallmark of our patient indicates leg hyperreflexia from an early disease course. This neurological sign differs from the previously reported clinical expression of Japanese and foreign patients with GSS (P102L). Thus, physicians should pay more attention to phenotypic heterogeneity in this prion disease.
-
Case Reports
Paradoxical brain embolism induced by Mycoplasma pneumoniae infection with deep venous thrombus.
A 21-year-old man had sudden-onset right hemiplegia and aphasia with respiratory infection. A chest X-ray disclosed consolidation in both lungs and magnetic resonance imaging showed an embolism in the left middle cerebral artery. ⋯ Antibodies to M. pneumoniae were highly elevated, and hypercoagulability was subsequently detected. This case suggests that the possible pathogenic mechanism for M. pneumoniae infection-related stroke might be paradoxical brain embolism with deep venous thrombus as a consequence of the hypercoagulability related to this infection.
-
Necrotizing fasciitis (NF) is a necrotizing soft tissue infection that can cause rapid local tissue destruction, necrosis and life-threatening severe sepsis. Predisposing conditions for NF include diabetes, malignancy, alcohol abuse, and chronic liver and kidney diseases. ⋯ The cardinal manifestations of NF are severe pain at onset out of proportion to local findings, hemorrhagic bullae and/or vital sign abnormality. In such cases, NF should be strongly suspected and immediate surgical intervention should be considered, along with broad-spectrum antimicrobials and general supportive measures, regardless of the findings of imaging tests.
-
Review Case Reports
Idiopathic portal hypertension in a patient with mixed connective tissue disease and protein C deficiency.
We report a 29-year-old woman with a 2.5 year history of mixed connective tissue disease (MCTD) who developed idiopathic portal hypertension (IPH) and thrombocytopenia as a result of hypersplenism. She had recurrent esophagogastric variceal rupture. ⋯ However, portal vein thrombosis developed after splenectomy. This was a rare case of severe complications of IPH accompanying MCTD and protein C deficiency.