No abstract available.
Humans ; Hypercholesterolemia*

Leiomyosarcoma arising in the skin is rare tumor, and diagnosis usually is made microscopically. After local excision, these lesions recur in large proportion of pat ients. The authors herein report a 53-year-old male with leiomyosarcoma appeared in the skin of the right forearm and presenting as a dark reddish colored, 5*6cm in diameter, superficial ulcerated single firm nodule with intermittent pain. Histopathological examination showed poorly circumscribed tumor consisting of interlacing bundles of spindle shaped smooth muscle cells in the middle and lower parts of the dermis. The nuclei were hyperchromatic, large, vacuolated, and irregular in shape. Electron microscopic findings revealed cytoplasmic organelles such as rough endoplasmic reticulum and mitochondria of malignant smooth muscle cells in the paranuclear area, Characteristic subsarcoelmmal caveolae and dense plaque were noted and myofilaments were distributed in the peripheral cytoplasm. The tumor did not recur in 10 months' follow-up.
Caveolae ; Cytoplasm ; Dermis ; Diagnosis ; Endoplasmic Reticulum, Rough ; Follow-Up Studies ; Forearm ; Humans ; Leiomyosarcoma* ; Male ; Middle Aged ; Mitochondria ; Myocytes, Smooth Muscle ; Myofibrils ; Organelles ; Skin* ; Ulcer

BACKGROUND: Transforming growth factor (TGF)- has a large variety of biological functions, including the modulation of inflammation and the immune system, and is presumed to play important roles in repairing wounds and reducing scarring. The objective of this study is to examine the effects of TGF-1 on healing wounds and reducing scarring. We have also analysed the ability of the hemagglutinating virus of Japan (HVJ) liposome mediated antisense oligodeoxynucleotides (ODNs) to specifically inhibit wound-induced expressions of TGF-1 proteins and mRNA in the rat skin. METHODS: Skin wounds were created on the backs of 80 anesthetized rats. The first group of wounds, as the controls, was unmanipulated. The second group of wounds, as positive controls or an excessive scarring model, was injected with TGF-1 subcutaneously. The third group of wounds was injected with anti-TGF-1 antibody subcutaneously. The fourth group of wounds was injected with HVJ liposome mediated antisense ODNs for TGF-1 subcutaneously. The wounds of all groups were bisected and analysed histologically 5, 10, 15, 30, and 50 days after the wounds were made. RESULTS: All control wounds (TGF-1 or no injection) healed with scarring, whereas the wounds treated with the antibody or antisense ODNs healed with less scar formation compared to the control group. The wounds treated with the antibody or antisense ODNs had fewer macrophages, less collagen and fibronectin contents than the other wounds. Northern blotting and in situ hybridization analysis showed that wound sites treated with HVJ liposome mediated antisense ODNs for TGF-1 exhibited decreased levels of TGF-1 mRNA after injury. CONCLUSIONS: These findings suggest an important new approach to controlling scarring in normal wound healing, complementing the practice of adding exogenous growth factors to chronic wounds in the attempt to inhibit collagen deposition.
Animals ; Blotting, Northern ; Cicatrix* ; Collagen ; Complement System Proteins ; Fibronectins ; Genetic Therapy* ; Immune System ; In Situ Hybridization ; Inflammation ; Intercellular Signaling Peptides and Proteins ; Liposomes ; Macrophages ; Oligodeoxyribonucleotides ; Oligoribonucleotides ; Rats ; RNA, Messenger ; Sendai virus ; Skin ; Transforming Growth Factor beta ; Transforming Growth Factors ; Wound Healing ; Wounds and Injuries

PURPOSE: The expression of c-Fos protein has been shown to be a useful marker for elevated levels of neuronal activity generated in the brain following different stimuli, including seizures. This study was conducted to investigate distribution and numbers of neurons where dentate and cingulate gyrus become activated following pentylenetetrazol-induced seizures by means of expression patterns of c-Fos protein. METHODS: Rats were sacrificed at increasing times(1 hour, 2 hours, 8 hours, 1 day, 4 days and 7 days) after pentylenetetrazol-induced seizure. Rat brains were removed and sliced in rat brain matrix. Brain slices were coronal sectioned at interaural 5.70-6.70mm. Serial sections were immunohistochemically reacted with polyclonal c-Fos antibody. The distribution and numbers of c-Fos protein immunoreactive neurons in dentate gyrus and cingulate gyrus were examined and analyzed statistically with Mann-Whitney U test. RESULTS: The numbers of c-Fos protein immunoreactive neurons in dentate gyrus peaked at 1 hours and reached almost normal conditions at 7 days after seizure. Also, same patterns were occurred in cingulate gyrus. Concentration value that pentylenetetrazol can induce was different from each animals and c-Fos immunoreactive cells were various kinds of neurons. CONCLUSION: Higher numbers of c-Fos protein immunoreactive neurons were found in dentate and cingulate gyrus at the same times after seizure. These findings suggest that neurons of dentate and cingulate gyrus play a crucial role in seizure onset following pentylenetetrazol-induced seizure.
Animals ; Brain* ; Dentate Gyrus ; Gyrus Cinguli ; Neurons ; Pentylenetetrazole ; Rats* ; Seizures*

No abstract available.
Hysterectomy*

BACKGROUNDS: The purpose of this study is to clarify therapeutic efficacy of percutaneous vertebroplasty(PVP) using polymethylmethacrylate(PMMA) for the osteoporotic compression fractures of the spine and to define the complications following the procedures. METHODS: The authors retrospectively analyzed the clinical outcome of 51 consecutive patients with recent osteoporotic compression fractures undergone PVP between January, 2000 and December, 2001. We reviewed medical records, radiographic findings, and telephone questionaires. The clinical outcomes were categorized by visual analogue scale(VAS). RESULTS: The mean age of the patients was 70.2 years old(range, 60~84) and the male/female ratio was 9:42(17.6%:82.4%). Mean duration of symptom was 2.75 months(0.25~12). Mean follow up periods were 12.2 months(5~27). Among the 63 treated segments, most of them were thoracolumbar junction(69.8%, 44/63) and treated via bipedicular route(bipedicular/unipedicular: 47/16). Thirty-eight patients showed complete or partial relief of pain(82.6%). Age, sex, duration of symptom, number of involved segments, and approach(uni-/bipedicular) had no statistical significance to clinical outcome. Nonetheless leakage of acrylic cement was noted in 37 patients(72.5%), most of them were asymptomatic. The complications were 4 symptomatic leakages(4 transient radicular pain), 1 pulmonary embolisms, and 1 fractured acrylic cement. CONCLUSION: PVP using PMMA is a minimally invasive, safe and effective treatment modality providing primary stability and prompt pain relief for the osteoporotic vertebral compression fractures. As most of the complications are related with leakage of acrylic cement, preoperative CT scan, vertebral phlebography and fluoroscopic guidance are mandatory as well as cautious injection of adequate amount of PMMA.
Follow-Up Studies ; Fractures, Compression* ; Humans ; Medical Records ; Phlebography ; Polymethyl Methacrylate ; Pulmonary Embolism ; Retrospective Studies ; Spine ; Telephone ; Tomography, X-Ray Computed ; Treatment Outcome* ; Vertebroplasty*

BACKGROUND: While primary prophylaxis is a well-established and recommended treatment for children with severe hemophilia, there has not been Korean own data. The aim of this study is to lay the foundation for the optimal and available management of hemophilia in Korea.METHODS: We reviewed the medical records of the patients treated with regular factor VIII concentrates infusion as long term continuous treatment at Seoul Clinic of Korea Hemophilia Foundation since August, 2004, retrospectively. We analyzed the efficacy of prophylaxis with the frequency of joint bleed and clinically meaningful bleeding episodes. We also assessed the cost of primary prophylaxis, secondary prophylaxis and on-demand therapy by the factor concentrates consumption.RESULTS: The data from 65 patients were available to evaluate. Of 65 patients, 22 were treated with primary prophylaxis, and 43 with secondary prophylaxis. In the primary and secondary prophylaxis groups, there were 1.9 joint bleeds and 3.3 other bleeding episodes, 7.2 and 9.0, respectively. The mean consumption of factor concentrates per person for primary prophylaxis, secondary prophylaxis and on-demand therapy groups were 2,658.8 IU/kg/year, 2,665.1 IU/kg/year, and 1,271.9 IU/kg/year respectively.CONCLUSION: Now prophylaxis is the recommended treatment for severe hemophilia patients, we should manage our hemophilia patients with standard treatment. And the optimizing treatment regimen for each individual will be needed to establish by studies of individual pharmacokinetics and bleeding tendency.
Child ; Factor VIII ; Hemophilia A ; Hemorrhage ; Humans ; Joints ; Korea ; Medical Records ; Retrospective Studies

PURPOSE: A hypomotor seizure is a useful descriptive terminology, but its clinical and ictal characteristics are not fully understood. We investigated the clinical and electrophysiological characteristics of hypomotor seizures in children. METHODS: We reviewed 394 video-taped recordings, performed between Dec. 1994 and Feb. 2003., to select patients of hypomotor seizures. Exclusion criteria were as follows; i) hypomotor seizures accompanied with other types of seizures in a single event, ii) patients without neurocognitive dysfunctions who were older than 3 years old, iii) non-convulsive status epilepticus, iv) poor video quality. A total of 329 hypomotor seizures from 42 patients (M:F=27:15) were included. The mean age of onset was 1.9 years old, the mean age at the monitoring 4.5 years old, and the mean duration of follow-ups 3.9 years. RESULTS: Thirteen patients (31%) showed generalized onset, hypomotor seizures while 29 patients (69%) showed partial onset hypomotor seizures. 35 patients (83.3%) had neurocognitive deficits. Significant abnormalities in the on brain MRIs were revealed in 23 patients (54.8 %). The concordance rates of interictal epileptiform discharges were 29.0%. In 8 patients, (hypomotor seizures were separately accompanied by other types of seizures (19.0%)). Comparing generalized seizures (group 1) with partial seizures (group 2), there were no significant differences in the clinical parameters between the two groups. In brain MRIs, group 2 tended to have focal lesions. On the analysis of ictal rhythms in group 1, diffuse spike and wave discharges were noted in 8 patients, rhythmic beta in 2 patients, semirhythmic theta in 1 patient, diffuse attenuation of the background in 1 patient, and bilateral beta in 1 patient. Among 5 patients with ictal rhythms other than diffuse spike and wave discharges, 4 patients were younger than 3 years old. On the analysis in group 2, rhythmic ictal patterns were noted in 24 patients (82.8%), seimirhythmic in 2 patients (6.9%), and irregular in 3 patients (10.3%). The distribution of ictal frequencies was as follows:alpha in 3 (10.3%), beta in 4 (13.8%), theta in 10 (34.5%), delta in 4 (13.8%), repetitive spikes or sharp waves in 5 (17.3%), and spikes and waves in 3 (10.3%). Rhythmic beta patterns tended to be localized into the posterior quadrant. CONCLUSION: Hypomotor seizures consist of generalized-onset (31%) and partial-onset (69 %). No clinical parameters can predict the ictal patterns. Various patterns in patients with generalized onset hypomotor seizures under 3 years old may suggest different mechanisms of generalized hypomotor seizures from absence seizures. Rhythmic beta patterns from the posterior quadrant may suggest the localization-specific ictal patterns.
Age of Onset ; Brain ; Child* ; Child, Preschool ; Electroencephalography ; Epilepsy, Absence ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Seizures* ; Status Epilepticus

PURPOSE: The channelopathies has been implicated in the pathogenesis of idiopathic generalized epilepsy(IGE). Recently, nonsense and missense mutations in a voltage-gated chloride channel gene(CLCN2) have been shown to be associated with IGE. Loss of CLCN2 function results in loss of sustained GABA inhibition, increasing the risk of uncontrolled firing leading to seizure activity. This study assessed the relevance of mutations in the CLCN2 gene in the Korean patients with IGE. METHODS: Twenty seven patients with IGE were recruited in the Seoul National University Boramae Hospital. PCR and direct sequencing of genomic DNA were done to analyze the complete coding region of CLCN2. 99 controls were tested for two identified polymorphisms. Genotypes and allelic frequencies were compared to controls with epilepsy patients and subgroup of IGE: 10 generalized epilepsy with febrile seizure plus(GEFSP), 9 childhood absence epilepsy(CAE) and 8 other IGE patients. RESULTS: Twelve CLCN2 polymorphisms: 3 exonic, 2 promotor and 7 intronic, were found in 22 patients(81%) and 2 polymorphisms were noble. Two polymorphisms in the exonic region with changes of amino acid, p.L15P and p.T668S and 2 polymorphisms in the promoter regions(c.1-1990T>C, c.1-693G>A) may affect on the CLCN channel function. The odds ratio for developing other IGE in patients with RS9820367-CG type was 4.2 compared to individuals with CC type. In addition, the odds ratio for developing other GEFSP in patients with RS9820367-CC type was 4.0 compared with individuals with CG type. CONCLUSION: Our findings suggest that genomic variations of CLCN2 may be implicated in the pathogenesis of IGE.
Channelopathies ; Chloride Channels ; Clinical Coding ; DNA ; Epilepsy ; Epilepsy, Generalized ; Exons ; Fires ; gamma-Aminobutyric Acid ; Genotype ; Humans ; Immunoglobulin E ; Introns ; Mutation, Missense ; Odds Ratio ; Polymerase Chain Reaction ; Seizures ; Seizures, Febrile