X-linked recessive bulbospinal muscular atrophy (Kennedy's syndrome) is a variant of the spinal muscular atrophies caused by mutation of androgen receptor gene on X-chromosome. A 69-year-old man had suffered from slowly progressive lower extremity weakness and gynecomastia. Muscle weakness was more severe in proximal muscles and showed symmetrical features. He had fascicular contraction on his face and tongue. All tendon reflexes were absent and pyramidal signs were not detected. Nerve conduction studies were normal except low amplitude of sensory nerve action potential in median nerve. Needle electromyography revealed widespread chronic denervation potentials in all sampling muscles of extremities, facial and tongue muscles. Histopathologic findings showed chronic denervation atrophy. DNA analysis showed abnormal expansion of CAG repeats in the androgen receptor gene and we confirmed this case as Kennedy's syndrome. If an adult patient has slowly progressive muscle weakness, bulbar symptoms and signs of male genital failure, DNA analysis should be taken to differentiate Kennedy syndrome from other motor neuron disease or myopathy.
Action Potentials ; Adult ; Aged ; Atrophy ; Bulbo-Spinal Atrophy, X-Linked ; Denervation ; DNA ; Electromyography ; Extremities ; Gynecomastia ; Humans ; Lower Extremity ; Male ; Median Nerve ; Motor Neuron Disease ; Muscle Weakness ; Muscles ; Muscular Atrophy* ; Muscular Diseases ; Needles ; Neural Conduction ; Receptors, Androgen ; Reflex, Stretch ; Tongue ; Trinucleotide Repeats

PURPOSE: The purpose of this study was to compare clinical manifestations of human bocavirus (hBoV), respiratory syncytial virus (RSV), and human rhinovirus (hRV) in children with acute wheezing. METHODS: We retrospectively investigated 549 virus-positive throat swabs obtained between January 2006 and December 2008 from pediatric inpatients with acute respiratory tract disease at Kwangju Christian Hospital. Among them, 109 patients, who had lower respiratory tract infections with wheezing, were enrolled in this study. The medical records of patients with positive results were reviewed for clinical data. RESULTS: The mean age of the patients with RSV was 7.15 months, 15.66 months in those with hRV, and 15.04 months in those with hBoV. The mean fever duration and frequency of patients with fever was 2.43 days and 47.9% for RSV, 2.86 days and 51.7% for hRV, and 3.75 days and 69.6% for hBoV. The frequency of patients with acute otitis media was 20.8% in the RSV, 20.7% in the hRV, and 13.0% in the hBoV groups. The frequency of lung infiltration on chest X-ray was 12.5% in the RSV, 20.7% in the hRV, and 47.8% in the hBoV groups. CONCLUSION: We compared the clinical manifestations of respiratory viral infections in infants and children with wheezing. However, further surveillance will be necessary to clarify the clinical manifestations of the viruses.
Bocavirus ; Child ; Fever ; Human bocavirus ; Humans ; Infant ; Inpatients ; Korea ; Lung ; Medical Records ; Otitis Media ; Pharynx ; Respiratory Sounds ; Respiratory Syncytial Viruses ; Respiratory Tract Diseases ; Respiratory Tract Infections ; Retrospective Studies ; Rhinovirus ; Thorax

Spontaneous bladder perforation is a very rare event. Prompt diagnosis of this injury is very important, particularly with intraperitoneal perforation, because mortality increases if surgical repair is delayed. Previous studies have reported that plain cystography is the primary modality of imaging study rather than relatively insensitive computed tomography (CT) when bladder perforation is suspected. We report here a rare case of spontaneous intraperitoneal perforation of the bladder associated with urothelial carcinoma with divergent histologic differentiation, as diagnosed with CT cystography.
Diagnosis, Differential ; Rupture, Spontaneous ; Urinary Bladder ; Urinary Bladder Neoplasms

No abstract available.
Humans* ; Pregnancy* ; Sperm Injections, Intracytoplasmic* ; Spermatozoa*

No abstract available.
Syphilis ; Syphilis, Cutaneous

No abstract available.
Humans ; Hyperlipoproteinemia Type II ; Xanthomatosis

No abstract available.
Child* ; Humans ; Mucinoses* ; Nevus, Pigmented*

BACKGROUND: To treat anastomosis site stenosis and occlusion of the artificial vessels used in vascular surgery, tissue-engineered artificial vessels using autologous cells have been constructed. We developed artificial vessels using a polymer scaffold and autologous bone marrow cells and performed an in vivo evaluation. MATERIAL AND METHOD: We manufactured a vascular scaffold using biodegradable PLCL (poly lactide-co-epsilon-caprolactone) and PGA (poly glycolic acid) fibers. Then we seeded autologous bone marrow cells onto the scaffold. After implantation of the artificial vessel into the abdominal aorta, we performed an angiography 3 weeks after surgery. After the dogs were euthanized we retrieved the artificial vessels and performed histological analysis. RESULT: Among the six dogs, 2 dogs died of massive bleeding due to a crack in the vascular scaffold 10 days after the operation. The remaining four dogs lived for 3 weeks after the operation. In these dogs, the angiography revealed no stenosis or occlusion at 3 weeks after the operation. Gross examination revealed small thrombi on the inner surface of the vessels and the histological analysis showed three layers of vessel structure similar to the native vessel. Immunohistochemical analysis demonstrated regeneration of the endothelial and smooth muscle cell layers. CONCLUSION: A tissue engineered vascular graft was manufactured using a polymer scaffold and autologous bone marrow cells that had a structure similar to that of the native artery. Further research is needed to determine how to accommodate the aortic pressure.
Angiography ; Animals ; Aorta, Abdominal ; Arterial Pressure ; Arteries ; Blood Vessel Prosthesis ; Bone Marrow ; Bone Marrow Cells ; Constriction, Pathologic ; Dogs ; Glycosaminoglycans ; Hemorrhage ; Myocytes, Smooth Muscle ; Polymers ; Prostaglandins A ; Regeneration ; Seeds ; Tissue Engineering ; Transplants

A two-year-old male Pointer had been presented with anorexia, cachexia, and weight loss of 10-day duration. Upon physical examination, fever, lethargy, superficial lymph node enlargement, and tick infestation were noted. The only abnormality in CBC and serum chemistry analyses was mild hyperglobulinemia. Spleen was enlarged by radiography, and the lymph nodes showed neutrophilic lymphadenitis by cytological examination. A polymerase chain reaction test for babesiosis and commercial ELISA tests for Ehrlichia canis, heartworm, and Lyme disease was negative except for Lyme disease, which was verified by both an IFA-IgG test and a quantitative C6 assay. Doxycycline was administered for 2 weeks and the recovery was uneventful. Post-treatment C6 titer decreased to within normal limits.
Animals ; Anti-Bacterial Agents/therapeutic use ; Dog Diseases/*diagnosis/drug therapy/epidemiology/pathology ; Dogs ; Doxycycline/therapeutic use ; Enzyme-Linked Immunosorbent Assay/veterinary ; Korea/epidemiology ; Lyme Disease/drug therapy/epidemiology/pathology/*veterinary ; Lymph Nodes/pathology ; Male ; Spleen/radiography

BACKGROUND AND OBJECTIVES: Exercise myocardial perfusion scans in patients with hypertrophic cardiomyopa-thy have shown reversible perfusion abnormalities with unknown clinical significance. We performed this study to characterize dipyridamole Tl-201 SPECT imaging and correlate with clinical findings in patients with hyper-trophic cardiomyopathy. METHODS: Tl-201 SPECT was performed in 25 patients of hypertrophic cardiomyopa-thy with asymmetric septal hypertrophy and 20 normal controls after dipyridamole infusion (0.56 mg/kg). Myocardial wall was divided into 8 segments. Tl-201 uptake and relative washout rate were calculated. RESULTS: Tl-201 SPECT showed significantly lower Tl-201 uptake in basal septal (81.3+/-3.4% vs 78.2+/-6.4%, p<0.05) and apical septal wall on stress (88.2+/-4.7% vs 83.9+/-6.5%, p<0.05) and higher apical septal (86.6+/-5.2% vs 89.2+/-3.1%, p<0.05) and apical anterior wall uptake (88.7%+/-4.0% vs 91.4+/-4.9%, p<0.05) on redistribution images in patients with hypertrophic cardiomyopathy. Basal lateral wall uptake of hypertrophic cardiomyopathy was significantly lower than normal control on both stress (84.7+/-3.5% vs 81.2+/-7.3%, p<0.05) and redistribution images (85.0+/-5.8% vs 76.8+/-7.2%, p<0.0001). The septum/lateral uptake ratio of patients on rest image was significantly higher than that of normal controls (0.98+/-0.07 vs 1.07+/-0.10, p<0.001). There was no difference in age, sex, symptom, cardiac medication and the parameters of 2D-echo including left ven-tricular outflow obstruction between subgroups of normal vs abnormal washout in patients with hypertrophic cardiomyopathy. CONCLUSION: Dipyridamole Tl-201 myocardial SPECT shows reduced coronary vasodilatory capacity of myocardium, especially septum in patients with hypertrophic cardiomyopathy. High septal/lateral uptake ratio on redistribution image may be a characteristic finding. However, no correlation between abnorm-al Tl-201 washout and clinical findings was observed.
Cardiomyopathies ; Cardiomyopathy, Hypertrophic* ; Dipyridamole* ; Humans ; Myocardium ; Perfusion* ; Tomography, Emission-Computed, Single-Photon*