No abstract available.
Pregnancy*

Ullrich's disease is a congenital muscular dystrophy clinically characterized by generalized muscle weakness, multiple contractures of the proximal joints, and hyperextensibility of the distal joints. All the patients develop rigidity of spine, often assoicated with scoliosis, failure to thrive, and early and severe respiratory involvement, irrespective of their levels of motor function. Intellectual development is normal. The biopsied muscles show dystrophies including remarkable variation in the fiber size, notably proliferated endomysial connective tissues, and a lot of degenerated and regenerated fibers. The expression of merosin and dytrophin is normal. Recent studies have demonstrated that collagen VI is deficient in the muscles of the patients with Ullrich's disease, and some result from recessive mutations of the collagen VIalpha 2 gene(COL6A2). And a marked reduction of fibronectin receptors in the extracellular matrix of skin and cultured fibroblasts of these patients is also reported. These results suggest that collagen VI deficiency may lead to the reduction of fibronectin receptors and that any abnormalities of cell adhesion may be involved in the pathogenesis of the disease. A case of Ullrich's disease has not been reported yet in Korea. So, we describe a male patient with Ullrich's disease with a brief review of the literature.
Cell Adhesion ; Collagen ; Connective Tissue ; Contracture ; Extracellular Matrix ; Failure to Thrive ; Fibroblasts ; Humans ; Integrin alpha5beta1 ; Joints ; Korea ; Laminin ; Male ; Muscle Weakness ; Muscles ; Muscular Dystrophies ; Receptors, Fibronectin ; Scoliosis ; Skin ; Spine

OBJECTIVE: Activated endothelial cells mediate the cascade of reactions in response to hypoxia for adaptation to the stress. It has been suggested that hypoxia, by itself, without reperfusion, can activate the endothelial cells and initiate complex responses. In this study, we investigated whether hypoxia-induced endothelial products alter the endothelial permeability and have a direct cytotoxic effect on nerve cells. METHODS: Hypoxic condition of primary human umbilical vein endothelial cells(HUVEC) was induced by CoCl2 treatment in culture medium. Cell growth was evaluated by 3,4,5-dimethyl thiazole-3,5-diphenyl tetrazolium bromide (MTT) assay. Hypoxia-induced products (IL-1beta, TGF-beta1, IFN-gamma, TNF-alpha, IL-10, IL-6, IL-8, MCP-1 and VEGF) were assessed by enzyme-linked immunosorbent assay. Endothelial permeability was evaluated by Western blotting. RESULTS: Prolonged hypoxia caused endothelial cells to secrete IL-6, IL-8, MCP-1 and VEGF. However, the levels of IL-1, IL-10, TNF-alpha, TGF-beta, IFN-gamma and nitric oxide remained unchanged over 48 h hypoxia. Hypoxic exposure to endothelial cells induced the time-dependent down regulation of the expression of cadherin and catenin protein. The conditioned medium taken from hypoxic HUVECs had the cytotoxic effect selectively on neuroblastoma cells, but not on astroglioma cells. CONCLUSION: These results suggest the possibility that endothelial cell derived cytokines or other secreted products with the increased endothelial permeability might directly contribute to nerve cell injury followed by hypoxia.
Anoxia ; Astrocytoma ; Blotting, Western ; Culture Media, Conditioned ; Cytokines ; Down-Regulation ; Endothelial Cells* ; Enzyme-Linked Immunosorbent Assay ; Humans ; Interleukin-1 ; Interleukin-10 ; Interleukin-6 ; Interleukin-8 ; Neuroblastoma ; Neurons* ; Nitric Oxide ; Permeability ; Reperfusion ; Stroke* ; Transforming Growth Factor beta ; Transforming Growth Factor beta1 ; Tumor Necrosis Factor-alpha ; Umbilical Veins ; Vascular Endothelial Growth Factor A

OBJECTIVE: To elucidate the role of aquaporin-4(AQP4) in cerebral edema formation, we studied the expression and subcellular localization of AQP4 in astrocytes after focal cerebral ischemia. METHODS: Cerebral ischemia were induced by permanent middle cerebral artery(MCA) occlusion in rats and estimated by the discoloration after triphenyltetrazolium chloride(TTC) immersion. Change of AQP4 expression were evaluated using western blot. Localization of AQP4 was assessed by confocal microscopy and its interaction with alpha-syntrophin was analyzed by immunoprecipitation. RESULTS: After right MCA occlusion, the size of infarct and number of apoptotic cells increased with time. The ratio of GluR1/GluR2 expression also increased during ischemia. The polarized localization of AQP4 in the endfeet of astrocytes contacting with ventricles, vessels and pia mater was changed into the diffuse distribution in cytoplasm. The interactions of AQP4 and Kir with alpha-syntrophin, an adaptor of dystrophin complex, were disrupted by cerebral ischemia. CONCLUSION: The deranged spatial buffering function of astrocytes due to mislocalized AQP4/Kir4.1 channel as well as increased assembly of Ca2+ permeable AMPA receptors might contribute to the development of edema formation and the excitotoxic neuronal cell death during ischemia.
Animals ; Apoptosis ; Aquaporin 4 ; Astrocytes ; Blotting, Western ; Brain Edema* ; Brain Ischemia* ; Cell Death ; Cerebral Infarction ; Cytoplasm ; Dystrophin ; Edema ; Immersion ; Immunoprecipitation ; Ischemia ; Microscopy, Confocal ; Neurons ; Pia Mater ; Rats* ; Receptors, AMPA ; Receptors, KIR

BACKGROUND: In 2000~2001 there was a nationwide outbreak of measles in the Republic of Korea. Since supplementary vaccination program was implemented in 2001, the control strategy of this measles outbreak has been stepped up from the control phase to the elimination phase. We here present the result of survey for the epidemiologic characteristics of the nationwide outbreak of measles in 2000~2001. METHODS: Using the data based on the sheet of epidemiologic investigation, incidences rates per 100,000 population and distributions of cases with vaccination history were investigated by the age. The seasonal or regional distribution were also studied with the onset days of cases. RESULTS: In 2000~2001, a measles outbreak occurred with 52,897 reported cases. The incidence rate per 100,000 population of measles was 111.9 cases and that of geopolitical unit (province) was high nationwide, 35.9~386.3 cases. The incidence rates were very high in less than 12 months and 10 years of age, 954.4 and 714.9, respectively. The 1st vaccination rate of measles/mumps/rubella (MMR) in 1~3 years old was 26.3~77.1%, and especially, that of 1 year old showed very low coverage, 26.3%. However, there was no special propensity in the 1st vaccine coverage in the age of 7~15 years, which had 87.7~91.4%. On the other hand, the 2nd MMR vaccination rate of the cases was very low, 17.7% at the age of 7~10 years. From the 21st May, 2001 a mass catch-up campaign with measles/rubella (MR) vaccine was launched, resulting in prominent reduction in the incidence of measles case. CONCLUSION: This survey showed that the measles outbreak in 2000~2001 had a high incidence nationwide from October 2000 to April 2001 and in less than 12 month and 10 years of age. Our results also showed that no routine immunization made the age group under 1 year vulnerable to measles infection. In cases of school-age group after 7 years of age, some cases with vaccination history were also infected. The characteristics of recent outbreaks indicates the need for the introduction of a specific vaccination programme at that age group. This survey was helpful to establish the effective vaccination strategy and it would be used as reference data on survey continuously for the epidemiologic characteristics of measles case for elimination of indigenous measles transmission in Republic of Korea.
Disease Outbreaks ; Epidemiology ; Hand ; Humans ; Immunization ; Incidence ; Korea* ; Measles* ; Republic of Korea ; Seasons ; Vaccination

BACKGROUND: This study reports the incidences of measles in Koea and the epidemiologic characteristics for the incidence reported for the period 1980~1999. It provides the epidemeologic adta for measles before implementing the catch-up campaign in 2001. METHODS: We obtained information for the survey from the data for the incidence reported from health centers. Incidences per 100,000 population were calculated for the 1963~1999. And the following variables were studied: seasonal or regional distribution for the 1980~1999. RESULTS: The incidence rate of measles declined after the systemic immunization in 1990, the outbreak of measles occurred periodically and incidences per 100,000 population increased from 8.4 cases in 1990 to 17.7 in 1994. In 1990 and 1994, measles cases centered around the 5~6th month. On the other hand, in non-epidemic year measles cases were scattered around the year. The incidence rates of measles by the province were greatly defferent whenever the outbreak of measles occurred. CONCLUSION: In 1990s, the outbreak of measles occurred periodically at the interval of 4 years until 1994. The seasonal distribution may be caused by Iimitation of transmission due to the increased measles immunity in outbreak region. Our results showed that the measles outbreak did not occur in the specific province, but it occurred nationwide whenever measles outbreak occurred, even though the incidence rates of the provinces were different.
Epidemiology ; Hand ; Immunization ; Incidence* ; Korea* ; Measles* ; Seasons

OBJECTIVES: The facial bones are the most noticeable area in the human body, and facial injuries can cause significant functional, aesthetic, and psychological complications. Continuous study of the patterns of facial bone fractures and changes in trends is helpful in the prevention and treatment of maxillofacial fractures. The purpose of the current clinico-statistical study is to investigate the pattern of facial fractures over a 4-year period. MATERIALS AND METHODS: A retrospective analysis of 1,824 fracture sites was carried out in 1,284 patients admitted to SMG-SNU Boramae Medical Center for facial bone fracture from January 2010 to December 2013. We evaluated the distributions of age/gender/season, fracture site, cause of injury, duration from injury to treatment, hospitalization period, and postoperative complications. RESULTS: The ratio of men to women was 3.2:1. Most fractures occurred in individuals aged between teens to 40s and were most prevalent at the middle and end of the month. Fractures occurred in the nasal bone (65.0%), orbital wall (29.2%), maxillary wall (15.3%), zygomatic arch (13.2%), zygomaticomaxillary complex (9.8%), mandibular symphysis (6.5%), mandibular angle (5.9%), mandibular condyle (4.9%), and mandibular body (1.9%). The most common etiologies were fall (32.5%) and assault (26.0%). The average duration of injury to treatment was 6 days, and the average hospitalization period was 5 days. Eighteen postoperative complications were observed in 17 patients, mainly infection and malocclusion in the mandible. CONCLUSION: This study reflects the tendency for trauma in the Seoul metropolitan region because it analyzes all facial fracture patients who visited our hospital regardless of the specific department. Distinctively, in this study, midfacial fractures had a much higher incidence than mandible fractures.
Adolescent ; Facial Bones ; Facial Injuries ; Female ; Hospitalization ; Human Body ; Humans ; Incidence ; Male ; Malocclusion ; Mandible ; Mandibular Condyle ; Maxillofacial Injuries ; Nasal Bone ; Orbit ; Postoperative Complications ; Retrospective Studies* ; Seoul ; Zygoma

Hemangiomas result from proliferation of immature capillary vessels. This disorder in the genitourinary tract is a rare lesion. We experienced one case of a 31 years old man suffered from cavernous hemangioma of the penis shaft with painful erection. He was treated successfully with surgical excision. Herein, we report our experience and a critical review of the literature.
Capillaries ; Caves ; Hemangioma ; Hemangioma, Cavernous ; Male ; Penis

We experienced the case of infected mesh with subsequent infective cellulitis of obturator foramen after a TOT procedure. We reported our case of a 47-year-old woman who presented with persistent vaginal discharge, pain and motion limitation of left leg after having a TOT placed for stress incontinence. Inflammation around obturator foramen was demonstrated on computed tomography (CT) scan. We performed total excision of the mesh material. She ultimately had an uneventful postoperative course and no recurrence of incontinence after follow-up 1 month.
Cellulitis* ; Female ; Follow-Up Studies ; Humans ; Inflammation ; Leg ; Middle Aged ; Recurrence ; Suburethral Slings* ; Vaginal Discharge

Clinical features, laboratory and operative findings were reviewed in 17 cases adult Ebstein's anomaly admitted to Seoul National University Hospital from Feburary,1979 to October, 1987. 1) Mean age of first diagnosis was 28.6 tears old(range ; 14-57 years old). 2) Exertional duspnea(88.2%) and palpitation(64.7%) were the predominant symptoms, and PSVT was documented in 4 cases among 11 patoents with palpitation. 3) Echocardiogram showed displacement or delayed closure of the tricuspid valve in 13 cases. 4) On similtaneous recording of the intracardiac ECG and pressure, right ventricular electrogram with atrial pressure in atrialized right ventricle was observed in all cases. 5) Right ventriculogram revealed displacement of the tricuspid valve in 9 cases and tricuspid regurgitation in 10 cases. 6) Interatrial communication via ASD(8) and PFO(3) was present in 11 cases(64.7%). 7) Operative finding of 9 cases showed abnormalities of septal leaflet in 9(100%), posterior leaflet in 8(88.8%), and anterior leaflet in 2 cases(22.2%).
Adult* ; Atrial Pressure ; Diagnosis ; Ebstein Anomaly* ; Electrocardiography ; Heart Ventricles ; Humans ; Seoul ; Tricuspid Valve ; Tricuspid Valve Insufficiency