No abstract available.
Hearing* ; Humans ; Tinnitus*

No abstract available.
Hemangioblastoma* ; Spinal Cord*

No abstract available.
Audiometry*

Chronic granulomatous disease is characterized by recurrent life-threatening infections and the formation of granulomatous lesions caused by the failure of phagocytic oxidative microcidal activity that has resulted from a defect of the oxidase system. The fungal osteomyelitis of the spine in this disease is almost always caused by Aspergillus, and its treatment is not yet established although surgical management, antifungal agents, granulocyte transfusions or combination methods have been reported. We report a patient with aspergillosis who had a long-standing pulmonary lesion which subsequently spread to the adjacent thoracic wall and spine. After decompression and drainage by costotransversectomy, the severe pain was relieved but the kyphosis increased and a paraplegia developed slowly because of resistance to the antifungal therapy and the inability to perform a bone graft. The patient died 2 months after the operation because of pulmonary complications.
Antifungal Agents ; Aspergillosis* ; Aspergillus ; Decompression ; Drainage ; Glucose Oxidase ; Granulocytes ; Granulomatous Disease, Chronic* ; Humans ; Kyphosis ; Osteomyelitis ; Oxidoreductases ; Paraplegia ; Spine* ; Thoracic Wall ; Transplants

PURPOSE: To evaluate the possibility that human muscle derived stem cells (hMDSCs) can be differentiated into neurons in vitro. MATERIAL AND METHODS: Muscle derived stem cells were isolated from the hamstring muscles during the anterior cruciate ligament reconstruction by preplate technique. For the characterization of these cells, desmin staining, CD 34, Sca-1, CD 29 using the Flow cytometry were performed. In the experimental group, neuronal induction media was added to differentiate hMDSCs to neuronal cells. These cells were evaluated by neuronal markers such as neuron-specific enolase (NSE), neurofilament (NF), TrkA using immunocytochemistry. For the control group, no induction media was added. Statstical analyses were performed by use of Kruskal-Wallis H test and Student-Newman-Keuls test (P<0.01). RESULT: Desmin staining was positive in 92.3+/-6%. Flow cytometry was negative for CD 34 and Sca-1. However it was positive for CD 29. (69.4+/-10%). The immunocytochemical result revealed NSE, NF and TrkA positive with 63.2+/-2.3%, 59.2+/-2.5%, 55+/-2.4% respectively. However, these were negative in the control group. CONCLUSION: Our observations indicate that hMDSCs have the capacity to differentiate into neurons in a specialized culture media.
Anterior Cruciate Ligament Reconstruction ; Culture Media ; Desmin ; Flow Cytometry ; Humans* ; Immunohistochemistry ; Muscles ; Neurons* ; Phosphopyruvate Hydratase ; Stem Cells*

No abstract available.
Animals ; Heart* ; Rats*

No abstract available.
Adolescent ; Ataxia Telangiectasia/*genetics ; Ataxia Telangiectasia Mutated Proteins/*genetics ; Base Sequence ; Chromosome Inversion ; Chromosomes, Human, Pair 14/genetics ; Chromosomes, Human, Pair 7/genetics ; DNA Mutational Analysis ; Female ; Humans ; Karyotyping ; Magnetic Resonance Imaging ; Mutation ; RNA Splicing ; Translocation, Genetic

BACKGROUND: Spinocerebellar ataxia (SCA) type 8 (SCA8) is an inherited neurodegenerative disorder caused by the expansion of untranslated CTA/CTG triplet repeats on 13q21. The phenomenology of SCA8 is relatively varied when compared to the other types of SCAs and its spectrum is not well established. CASE REPORT: Two newly detected cases of SCA8 with the nonataxic phenotype and unusual clinical manifestations such as dopaminergic-treatment-responsive parkinsonism and amyotrophic lateral sclerosis (ALS) are described herein. Family A expressed good dopaminergic treatment-responsive parkinsonism as an initial manifestation and developed mild cerebellar ataxia with additional movements, including dystonic gait and unusual oscillatory movement of the trunk, during the disease course. The proband of family B presented as probable ALS with cerebellar atrophy on brain MRI, with a positive family history (a brother with typical cerebellar ataxia) and genetic confirmation for SCA8. CONCLUSIONS: Our findings support that the non-ataxic phenotypes could be caused by a mutation of the SCA8 locus which might affect neurons other than the cerebellum.
Amyotrophic Lateral Sclerosis* ; Atrophy ; Brain ; Cerebellar Ataxia ; Cerebellum ; Gait ; Humans ; Neurodegenerative Diseases ; Neurons ; Parkinson Disease* ; Parkinsonian Disorders ; Phenotype* ; Siblings ; Spinocerebellar Ataxias ; Spinocerebellar Degenerations ; Trinucleotide Repeats

BACKGROUND: Asthma is a chronic inflammatory disease of the bronchial mucosa and is associated with excess production of Th2 cytokines (IL-4, IL-5) relative to Th1 cytokine (IFN-V). The NK cell and TNK cell are supposed to be involved in the pathogenesis of allergic inflammation by cytokine regulation. OBJECTIVES: The aim of this study was to investigate the effect of allergen (Der p 2) on the production of IFN-V by CD3+T cell, CD56+NK cell and CD3+CD56+TNK cells in patients with mild persistent asthma. METHOD: Peripheral blood mononuclear cells (PBMCs) from patients with mild persistent asthma (n=12) who were sensitive to dust mite, were cultured with or without Der p 2 for 3 days, and phorbol ester plus calcium ionophore and intracellular protein transport inhibitor were added 4 hours before staining. A three-color flow cytometric analysis was done to detect intracytoplasmic IFN-V, surface DC3 and CD56 antigen simultaneously. RESULTS: When PBMCs were cultured only in media, there were no significant differences in the percentage of IFN-V positive CD3+T cell, CD56+NK cell and CD3+CD56+TNK cells between asthmatic patients and normal subjects. However, there were significant decreases in the percent change of IFN-V positive CD3+T cell, CD56+NK cell and CD3+CD56+TNK cell in asthmatic patients comparde to normal subjects after stimulation of PBMCs with Der p 2. CONCLUSION: This study suggests that NK cell and TNK cell may participate in allergic reaction by IFN-V regulation.
Antigens, CD56 ; Asthma* ; Calcium ; Cytokines ; Dust ; Humans ; Hypersensitivity ; Inflammation ; Interferons* ; Killer Cells, Natural ; Mites ; Mucous Membrane ; Protein Transport

PURPOSE: To investigate the expression of Vascular Endothelial Growth Factor (VEGF) in diabetic frozen shoulders. MATERIALS AND METHODS: We preformed arthroscopic adhesiolysis on 9 diabetic frozen shoulder patients, and observed the arthroscopic findings. Also, we examined the potential role of VEGF by using samples of synovial tissues from 5 patients, and 2 normal synovial tissues. Immunohistochemical staining and Western blotting were performed using polyclonal antibodies against VEGF. RESULTS: There was hyperemic synovitis in the 9 diabetic frozen shoulder patients. In the 5 patients' tissue samples, there was strong immunostaining and expression to VEGF, but there was little staining and expression in the control group. CONCLUSION: We postulate that VEGF is synthesized and secreted in the synovium of diabetic frozen shoulders and that secreted VEGF binds specific receptors on the endothelial cells of nearby small blood vessels, and leads to the subsequent development of frozen shoulders in diabetic patients.
Antibodies ; Blood Vessels ; Blotting, Western ; Bursitis* ; Endothelial Cells ; Humans ; Shoulder ; Synovial Membrane ; Synovitis ; Vascular Endothelial Growth Factor A*