ABSTRACT
BACKGROUND: IPF is characterized by chronic, fibrosing inflammatory lung disease of unknown etiology. Typical symptoms of IPF are exertional dyspnea with nonproductive cough. Why patients with typical IPF have dry cough rather than productive cough, is unknown. IP-10 plays an important regulatory role in leukocyte trafficking into the lung. The present study investigated the effect of IP-10 in the pathogenesis of dry cough rather than productive cough in IPF patients. METHODS: IP-10 concentration was measured by ELISA from BALF of IPF patients. To evaluate the role of IP-10 in mucin expression, the expression of the MUC5AC mucin gene was measured in NCI-H292 cells, a human pulmonary mucoepidermoid carcinoma cell line, after stimulation by TNF-alpha with or without IP-10 pretreatment. EGFR-MAPK expression was also examined as a possible mechanism. RESULTS: IP-10 levels were significantly higher in the BALF of IPF patients compared to healthy controls. IP-10 pretreatment reduced TNF-alpha induced MUC5AC mucin expression by inhibiting the EGFR-MAPK signal transduction pathway in NCI-H292 cells. CONCLUSION: These findings suggest that little mucus production in IPF patients might be attributable to IP-10 overproduction, which inhibits the EGFR-MAPK signal transduction pathway required for MUC5AC mucin gene expression.
Subject(s)
Humans , Carcinoma, Mucoepidermoid , Cell Line , Cough , Dyspnea , Enzyme-Linked Immunosorbent Assay , Gene Expression , Idiopathic Pulmonary Fibrosis , Leukocytes , Lung , Lung Diseases , Mucins , Mucus , ErbB Receptors , Signal Transduction , Sputum , Tumor Necrosis Factor-alphaABSTRACT
McCune-Albright syndrome is characterized by polyostotic fibrous dysplasia, Caf -au-lait pigmentation and precocious puberty or other endocrinopathy. It can be caused by substitution of His, Cys or Gly for Arg 201st amino acid of the Gs protein subunit. The case of a 32-year-old woman, with atypical McCune-Albright syndrome, is reported. She had no skin lesion or precocity puberty. The polyostotic fibrous dysplasia was examined by a simple radiological image and whole body scan. She developed hyperthyroidism, with a multinodular toxic goiter. No thyroid related autoantibodies were detected. The cause of hyperthyroidism was thought to be a non- autoimmune thyroid hyperfunction. The level of growth hormone was not suppressed by oral glucose load. After a bromocriptine suppression test, the level of growth hormone decreased. There was no mass in the pituitary gland on a sellar MRI. A case of atypical McCune-Albright syndrome, including hyperthyroidism and hypersecretion of growth hormone, is reported.
Subject(s)
Adolescent , Adult , Female , Humans , Autoantibodies , Bromocriptine , Fibrous Dysplasia, Polyostotic , Glucose , Goiter , Growth Hormone , Hyperthyroidism , Magnetic Resonance Imaging , Pigmentation , Pituitary Gland , Protein Subunits , Puberty , Puberty, Precocious , Skin , Thyroid Gland , Whole Body ImagingABSTRACT
A 43 years old woman with a previous history of healed tuberculosis presented with a recurrent hemoptysis. On a broncoscopy inspection, three nodular shaped mass-like lesions (0.5cm in diameter) were found on the right lower lobar bronchus. Massive hemoptysis occurred when the specimen were taken during bronchoscopy. The bleeding could not be controlled by non-surgical treatment. Consequently, she underwent a right lower lobectomy in order to control the bleeding. The patient died of respiratory failure 3 days later. A brochoscopic biopsy revealed an abnormal blood vessel just beneath the bronchial respiratory epithelium.
Subject(s)
Female , Humans , BiopsyABSTRACT
A 43 years old woman with a previous history of healed tuberculosis presented with a recurrent hemoptysis. On a broncoscopy inspection, three nodular shaped mass-like lesions (0.5cm in diameter) were found on the right lower lobar bronchus. Massive hemoptysis occurred when the specimen were taken during bronchoscopy. The bleeding could not be controlled by non-surgical treatment. Consequently, she underwent a right lower lobectomy in order to control the bleeding. The patient died of respiratory failure 3 days later. A brochoscopic biopsy revealed an abnormal blood vessel just beneath the bronchial respiratory epithelium.
Subject(s)
Female , Humans , BiopsyABSTRACT
Primary venous thrombosis caused by deficiency or qualitative abnormality of antithrombin III, protein C and protein S is usually inherited as an autosomal dominant trait. Usually, deep vein thrombosis or pulmonary thromboembolism is developed by such abnormalities, however, mesenteric vein thrombosis is rarely reported. A 27-year-old man with previous history of deep vein thrombosis underwent segmental resection of jejunum due to mesenteric vein thrombosis complicated by necrosis of jejunum. Postoperative investigation disclosed combined deficiency of antithrombin III and protein C. His son also showed deficiency of antithrombin III. Postoperatively, he is on life-long warfarin therapy without experiencing recurrence of venous thrombosis.