ABSTRACT
OBJECTIVE: To investigate the clinical characteristics, diagnosis and therapy of Keutel syndrome, and thereby to minimize the misdiagnosis. METHOD: Data of a case of Keutel syndrome diagnosed at the Provincial Hospital Affiliated to Shandong University were analyzed and related literature were reviewed. RESULT: An 8-month-26-day-old boy was presented with inspiratory and expiratory stridor and wheezing after movement on lung auscultation. His craniofacial appearance was characterized by midfacial hypoplasia with a broad depressed nasal bridge. The nose was small and flat. A grade 2-3/6 systolic murmur was heard between the second and third ribs at left edge of the sternum. The end phalanges of his fingers were thickened. Chest radiograph showed tracheobronchial cartilage calcification and tracheobronchial stenosis. Echocardiographic examination revealed the right pulmonary stenosis. With endoscopic surgery, antiobstructive and antibiotic therapy clinical symptoms were improved. Three weeks later he died of lung reinfection after he was discharged from our hospital. English literature search with "Keutel syndrome" as the key word at "PubMed" showed 22 articles covering 26 patients, and the clinical symptoms were hearing loss (91%), persistent respiratory symptoms (68%), recurrent otitis media/sinusitis (67%), growth development delay (52%) in turn, and signs were brachytelephalangism (100%), low nasal bridge (95%), midfacial hypoplasia (93%), cardiac murmur (69%), and auxiliary examinations showed abnormal cartilage calcification (100%), pulmonary arterial stenosis (72%), tracheobronchial stenosis (50%). CONCLUSION: The diagnosis of Keutel syndrome should be considered in patients with brachytelephalangism, abnormal cartilage calcification, peripheral pulmonary stenosis, and midfacial hypoplasia. Tracheal stenosis was main clinical manifestation in part of patients.
Subject(s)
Abnormalities, Multiple/diagnosis , Calcinosis/diagnosis , Cartilage Diseases/diagnosis , Hand Deformities, Congenital/diagnosis , Pulmonary Valve Stenosis/diagnosis , Tracheal Stenosis/diagnosis , Abnormalities, Multiple/diagnostic imaging , Abnormalities, Multiple/therapy , Bone and Bones/diagnostic imaging , Calcinosis/diagnostic imaging , Calcinosis/therapy , Cartilage/diagnostic imaging , Cartilage Diseases/diagnostic imaging , Cartilage Diseases/therapy , Diagnosis, Differential , Hand Deformities, Congenital/diagnostic imaging , Hand Deformities, Congenital/therapy , Humans , Infant , Male , Pulmonary Valve Stenosis/diagnostic imaging , Pulmonary Valve Stenosis/therapy , Radiography, Thoracic , Retrospective Studies , Tomography, X-Ray Computed , Tracheal Stenosis/diagnostic imagingABSTRACT
OBJECTIVE: To study the effects of budesonide on hypoxia inducible factor 1α(HIF-1α) and vascular endothelial growth factor (VEGF) expression, angiogenesis and airway remodeling in the chronic asthmatic mouse model. METHODS: Thirty female BALB/c mice were randomly divided into normal control, asthma model and treatment groups (10 in each group).The asthmatic mouse model was established via OVA challenge test. Mice in the treatment group were administered with aerosol budesonide (100 µg/kg) an hour before the OVA challenge test from the 28th day. Mice in the control group were treated with PBS instead of OVA. Hematoxylin and eosin staining was performed to observe thickness of the airway wall. Masson staining was used for examing collagen deposition of lung tissues. Angiogenesis and HIF-1α and VEGF expression were measured using immunohistochemistry and Western blot. The relationship of airway wall thickness and vessel area to HIF-1α and VEGF expression was investigated. RESULTS: Vessel area, collagen deposition of lung tissues and airway wall thickness increased in the asthma model group. Levels of HIF-1α and VEGF were also elevated. Administration of budesonide significantly reduced angiogenesis, collagen deposition of lung tissues and airway wall thickening, as well as expression of HIF-1α and VEGF. The vessel area and airway wall thickness were positively correlated with expression of HIF-1α and VEGF. A positive correlation was also found between the expression of HIF-1α and VEGF. CONCLUSIONS: Budesonide can decease angiogenesis and airway remodeling by inhibiting HIF-1α and VEGF expression in asthmatic mice.
Subject(s)
Airway Remodeling/drug effects , Asthma/drug therapy , Bronchodilator Agents/pharmacology , Budesonide/pharmacology , Hypoxia-Inducible Factor 1, alpha Subunit/analysis , Vascular Endothelial Growth Factor A/analysis , Animals , Asthma/metabolism , Asthma/pathology , Bronchi/pathology , Disease Models, Animal , Female , Mice , Mice, Inbred BALB C , Neovascularization, PhysiologicABSTRACT
OBJECTIVE: To investigate the relationship between the expression of neuronal acetylcholine receptor alpha 7 (nAChRalpha7) and the role of nAChRalpha7 in the development of asthma by detecting the expression of nAChR alpha7 on CD(4)(+) T lymphocytes in the peripheral blood and the level of cytokines IFN-gamma and IL-4 in the blood serum from children with asthma. METHODS: Thirty patients in the asthma group and 20 healthy children in the normal control group were included. Peripheral blood was collected, and the serum was harvested. The expression of nAChRalpha7 on CD(3)(+) and CD(4)(+) cells were detected by flow cytometry. The levels of IFN-gamma and IL-4 in the serum were detected by ELISA. RESULTS: The expression of nAChRalpha7 on CD(4)(+) T lymphocytes from children with asthma was higher than that from normal controls (t = 2.53, P < 0.05). Compared with the control group, the level of IL-4 in the serum of the asthma group was higher (t = 5.42, P < 0.01) but the level of IFN-gamma was lower (t = 4.11, P < 0.01). There were positive correlation between nAChRalpha7 and IL-4 (r = 0.688, P < 0.01) and negative correlation between nAChRalpha7 and IFN-gamma (r = -0.476, P < 0.01). CONCLUSION: nAChRalpha7 was shown to influence the balance of Th1/Th2 and it may play an important role in the development of asthma.
Subject(s)
Asthma/metabolism , CD4-Positive T-Lymphocytes/metabolism , Receptors, Nicotinic/metabolism , Asthma/blood , Case-Control Studies , Child , Child, Preschool , Female , Humans , Interferon-gamma/blood , Interleukin-4/blood , Male , Th1 Cells/metabolism , Th2 Cells/metabolism , alpha7 Nicotinic Acetylcholine ReceptorABSTRACT
OBJECTIVE: To understand the mechanism of effect of conditioned immune response in curing bronchial asthma. METHODS: An experimental asthma modal was produced on healthy BALB/C mice (female, 4 - 6 weeks old) by sensitization and stimulation with ovalbumin (OV A). Totally 105 mice were divided into 7 groups randomly with 15 in each and treated differently: in group CIR(1), noise was used as conditioned stimulus (CS) and budesonide and salbutamol as unconditioned stimulus (UCS) respectively, a conditioned immune response model of mice with asthma was established by the combination of CS and UCS 7 times (7 days), then the mice were given CS only, and the combination were given once a week for 20 weeks. In group CIR(2) saccharin (SAC) was taken as CS, and the other treatments were the same as the group CIR(1). In the group of conventional therapy, the mice were given inhalation of nebulized budesonide and salbutamol only for 20 weeks. In the group of lower dose conventional therapy, the mice were given nebulized inhalation of budesonide and salbutamol for the first 7 days, then once a week for 20 weeks. In the noise group the mice were given noise only everyday for 20 weeks. In SAC group the mice were treated with SAC only everyday for 20 weeks. In the blank control group the mice were treated with placebo for 20 weeks. The mice in all the groups were stimulated with OVA once a day. The mice in the healthy control group were given PBS inhalation for 20 weeks. After 20 weeks therapy, the bronchoalveolar lavage fluid (BALF) was taken for eosinophils (EOS) counting. The spleens were taken to obtain CD4(+)T lymphocytes and the expression of neuronal acetylcholine receptor alpha 7 (nAChRalpha7), IL-4, IFN-gamma and IL-17 were detected by flow cytometry. RESULTS: (1) The percent of EOS of groups CIR(1), CIR(2), conventional therapy and healthy control was much lower than that of blank control (P < 0.01), and there was no significant difference among groups CIR(1), CIR(2) and conventional therapy (P > 0.05). (2) The expression of nAChRalpha7, IL-4 and IL-17 of groups CIR(1), CIR(2), conventional therapy and healthy control was much lower than that in blank control group, IFN-gamma was much higher (P < 0.01), and no significant difference was found among groups CIR(1), CIR(2) and conventional therapy (P > 0.05). There was a positive correlation between nAChRalpha7 and IL-4 (r = 0.76, P < 0.01), nAChRalpha7 and IL-17 (r = 0.46, P < 0.01). There was a negative correlation between nAChRalpha7 and IFN-gamma (r = 0.69, P < 0.01). (3) In the groups treated with lower dose of conventional therapy, noise, SAC and blank control, the epithelial tissue of airway were much thicker, the lumens were much narrower, and inflammatory cells and collagen fibers were much more than in the healthy control group, and after therapy, the inflammation in groups CIR(1), CIR(2) and conventional therapy was significantly improved. CONCLUSION: The conditioned immune response models established by both noise and SAC as CS and budesonide and salbutamol as UCS can downregulate nAChRalpha7 on CD4(+)T lymphocytes, regulate the function of CD4(+)T lymphocytes, and achieve the same therapeutic efficacy in treatment of asthma.
Subject(s)
Asthma/immunology , CD4-Positive T-Lymphocytes/immunology , Administration, Inhalation , Animals , Asthma/drug therapy , Budesonide/therapeutic use , CD4-Positive T-Lymphocytes/metabolism , Female , Gene Expression Regulation , Mice , Mice, Inbred BALB C , Receptors, Nicotinic/metabolism , alpha7 Nicotinic Acetylcholine ReceptorABSTRACT
OBJECTIVE: To investigate the factors affecting prognosis of patients with primary hepatocellular carcinoma (PHC) after resection. METHODS: From 1976 to 1992, 213 patients with PHC treated with hepatectomy were followed up for more than 5 years. Thirty-one of the patients who had survived for more than 5 years were compared with 56 patients who had survived for less than 5 years. RESULTS: Early detection of tumor, radical resection, number of tumors, capsule formation, operation safe distance, presence of portal tumor embolus, presence of cirrhosis and tumor size were important factors affecting the prognosis of patients with PHC. The proportion of small liver cancer in the patients who had survived for more than 5 years was greatly larger than that in the control group. CONCLUSIONS: Early detection of tumor and radical resection are of value in raising the 5-year survival rate. Much remains to be investigated about the relationship between safe margin distance and prognosis of patients with PHC.