[Case of Kartagener syndrome and clinical study of the development of bronchiectasis].
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Biomedical subjects
Publications and source records attributed to E Matsui.
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Serial changes of MRI scanning of an 11-year-old boy with hemiparesis due to a germ cell tumor in the basal ganglia are presented. Initial brain MRI T1-weighted images revealed a subtle mixed signal intensity lesion at left anterior and posterior limbs of the internal capsule. This lesion was not enhanced with Gd-DTPA, however, T2-weighted images showed a misty high signal intensity lesion in the same region. The MR images also showed hemiatrophy of the left basal ganglia. Histologic examination of biopsy specimens of the tumor revealed an embryonal carcinoma. The patient was treated with chemotherapy with subsequent improvement in the hemiparesis. Our case suggests that germ cell tumors must be considered in the differential disorders of lesions in the ipsilateral hemiatrophic basal ganglia in pediatric patients with hemiparesis.
BACKGROUND: The atopic patient has a predisposition to selective synthesis of IgE antibodies to common environmental antigens. IgE production is upregulated by interleukin-4 (IL-4) and downregulated by interferon-gamma (IFN-gamma). IL-12 is a cytokine that induces IFN-gamma production. The signal of IL-12 is transduced through the IL-12 receptor (IL-12R) and Stat4. METHODS: We examined IFN-gamma production in peripheral blood mononuclear cells (PBMCs) following stimulation with IL-12 or phytohemagglutinin (PHA) in healthy controls and atopic patients. Moreover, sequences of the IL-12R beta(2) chain gene were analyzed. RESULTS: The serum IgE levels were negatively correlated (p < 0.001) with IFN-gamma production. In 24 out of 75 atopic patients, IFN-gamma production in PBMCs following stimulation with IL-12 was under the detection limit, but PHA stimulation elicited detectable IFN-gamma production. Sequence analysis of the cDNA of IL-12R beta(2) revealed three kinds of distinct genetic mutations (2496 del 91, 1577 A to G and 2799 A to G) in 10 unrelated subjects of the 24 whose IFN-gamma production following IL-12 stimulation was under the detection limit. PBMCs cultured with IL-12 and PHA in these 10 subjects showed decreased tyrosine phosphorylation of Stat4. CONCLUSIONS: The results of our study indicate that atopic diseases are caused, in part, by impairment of the IL-12 signal cascade, which downregulates IgE production, and that the mutation of the IL-12R beta(2) chain gene is one of the causative genes for atopy.
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Pleomorphic adenoma is the most common form of major salivary gland tumors,but it is exceedingly rare in the trachea. We present a case of pleomorphic adenoma of the trachea in an 80-years-old man. Chest three-dimensional CT demonstrated a bosselated mass with a lobulated surface arising from the membranous portion of the upper trachea. Transbronchoscopic biopsy from the mass showed a biphasic histologic appearance produced by a mixture of glandular structure and hyaline-like stroma. The pathological diagnosis was pleomorphic adenoma.