Creutzfeldt-Jakob disease(CJD) is characterized clinically by rapidly progressive dementia with pyramidal, extrapyramidal, and cerebellar symptoms and signs, and histologically by spongiform change, neuronal loss and reactive gliosis. We have experienced 2 cases of CJD. Case 1 was a 36-year-old male who had suffered from myoclonus and cerebellar symptoms including sluggish speech, gait and balance disturbance. Case 2 was a 70-year-old female who had showed cognitive dysfunction, ataxic gait and disturbance of extraocular movement. Both patients, underwent brain biopsy. Case 1 revealed marked cortical atrophy, 2mm in thickness, with neuronal loss and astrocytic proliferation extending into white matter. The spongiform change, made up of many small, usually rounded or oval, vacuoles was noted mainly in the neuropil. Case 2 revealed remarkable spongiform change throughout the cortex and cytoplasmic vacuoles compressing the nuclei of neuronal cells were numerous. Neuronal loss and gliosis were also found without considerable change in the white matter. On double immunostaining against GFAP and PrP(Prion Protein), there was a weak positive reaction for PrP in the perinuclear cytoplasm in case 1, and a strongly positive reaction in case 2. The electron microscopic examination showed numerous membrane-bound vacuoles in neuropil and perikarya of neurons. The majority of the vacuoles were multiseptated by thin membranous structures. They demonstrated curled, or disrupted membrane, that had foldings and protrusions into the vacuolar clear spaces. There were neither identifiable virus-like particles nor amyloid deposition.
Female ; Male ; Humans

Malignant germ cell tumore occur in children and young women in reproductive age, of all the germ cell maligaanci orily pure dysgerminiomas had a high cure rate prior to 1970. This was due to the exquisite wdioseneitivity of these tumors. Multiple agent chemotherapy has dramatieally improved the pr nosis af patients with malignant ovarian germ cell tumors. The purpose of this study is to report the experience at Aaan Medical Center, department of Obstetrics and Gynecology, in 16 patients withmalignant ovarian germ cell tumors treated between July, l989 and June, l994. We analyzed the effect of age, histolagic subtype, FIGO stage, surgical pmcedurse and regimens of chemotherapy, on the prognosis of thwe tumors. The results obtained were as follows: 1. In histologic subtypes, dysgenninoma(38.0%), endodermal sinus tumor(25.0%), squamous cell carcinoma arising in mature cystic teratoma(19.0%), mixed cell tumor(6.0%), immature teratoma(6.0%), malignant ectodermal tumor in mature cystic teratoma(6.0%), were counted in order. 2. No site predilection was identifed. 3. Main initial symptoms were abdominal distension(31.0%), abdominal pain(31.0%), abdominal mass palpation(25.0%), amenorrhea(6.0%) in order. 4. Multiple tumor markers were considered to be useful in diagnosis and follow up of malignant germ cell tumors of ovary. 5. The mean age of malignant ovarian germ cell tumors was 29.5 years, and 11 cases(69.3%) of tumors under the age of 30.0 years. 6. The survival rate seemed to be decreased with advancing FIGO stage.
Carcinoma, Squamous Cell ; Child ; Diagnosis ; Drug Therapy ; Ectoderm ; Endoderm ; Female ; Follow-Up Studies ; Germ Cells* ; Gynecology ; Humans ; Neoplasms, Germ Cell and Embryonal* ; Obstetrics ; Ovary* ; Prognosis ; Survival Rate ; Biomarkers, Tumor

"The Supporting Program for Obstetric Care Underserved Areas (SPOU)" provides financial aids to rural community (or district) hospitals to reopen prenatal care and delivery services for regions without obstetrics and gynecology clinics or hospitals. The purpose of this study was to evaluate the early stage effect of the SPOU program. The proportion of the number of birth through SPOU was calculated by each region. Also survey was conducted to investigate the extent of overall satisfaction, elements of dissatisfaction, and suggestions for improvement of the program; 209 subjects participated from 7 to 12 December, 2012. Overall, 20% of pregnant women in Youngdong (71 cases) and Gangjin (106 cases) used their community (or district) hospitals through the SPOU whereas Yecheon (23 cases) was 8%; their satisfaction rates were high. Short distance and easy accessibility was the main reason among women choosing community (or district) hospital whereas the reasons of not selecting the community (or district) hospital were favor of the outside hospital's facility, system, and trust in the medical staffs. The SPOU seems to be currently effective at an early stage. However, to successfully implement this program, the government should make continuous efforts to recruit highly qualified medical staffs and improve medical facility and equipment.
Adult ; Delivery, Obstetric/economics/*utilization ; Female ; Hospitals ; Humans ; Pregnancy ; Prenatal Care/economics ; *Program Evaluation ; Republic of Korea ; Rural Population

No abstract available.
Paramyxoviridae Infections* ; Polymerase Chain Reaction* ; Reverse Transcription*

OBJECTIVES: This study was conducted to describe the demographic characteristics, and clinical signs and symptoms of patients who visited a general hospital because of the release of chemically hazardous hydrogen fluoride that occurred on September 27, 2012 in Gumi City, Korea. METHODS: The medical records at 1 general hospital 9 km from the accident site were reviewed using a standardized survey format. There were 1,890 non-hospitalized and 12 hospitalized patients exposed to hydrogen fluoride between September 27 and October 13 2012. RESULTS: Among the 12 hospitalized patients, 11 were discharged within 1 week and the other was hospitalized for 10 days. The chief complaints were respiratory symptoms such as hemoptysis and shortness of breath, gastrointestinal symptoms, neurologic symptoms, sore throat, and lip burn. The number of non-hospitalized patients exhibited a bimodal distribution, peaking on the first and twelfth days after the accident. Their chief complaints were sore throat (24.1%), headache (19.1%), cough (13.1%), and eye irritation (9.2%); some patients were asymptomatic (6.2%). Patients who visited the hospital within 3 days (early patients) of the spill more often had shortness of breath (27.0%) and nausea (6.3%) as the chief complaints than patients who visited after 3 days (late patients) (3.5% and 2.6%, respectively). However, cough and rhinorrhea were more common in the late patients (14.0% and 3.3%, respectively) than in the early patients (5.0% and 0.0%, respectively). Patients who were closer to the accident site more often had shortness of breath and sputum as the chief complaints than patients who were farther away. The mean serum calcium concentration was 9.37 mg/dL (range: 8.4-11.0 mg/dL); none of the patients had a decreased serum calcium level. Among 48 pulmonary function test results, 4 showed decreased lung function. None of the patients had abnormal urine fluoride levels on the eighth day after exposure. CONCLUSIONS: Patients hospitalized due to chemical hazard release of hydrogen fluoride had acute respiratory, gastrointestinal, and neurologic health problems. Non-hospitalized patients have acute symptoms mainly related to upper respiratory irritation.
Burns ; Calcium ; Chemical Hazard Release ; Cough ; Dyspnea ; Fluorides ; Gyeongsangbuk-do ; Headache ; Hemoptysis ; Hospitals, General ; Humans ; Hydrofluoric Acid* ; Hydrogen* ; Korea ; Lip ; Lung ; Medical Records ; Nausea ; Neurologic Manifestations ; Pharyngitis ; Respiratory Function Tests ; Sputum

BACKGROUND: Isocitrate dehydrogenase (IDH) catalyzes the oxidative decarboxylation of isocitrate to yield alpha-ketoglutarate (alpha-KG) with production of reduced nicotinamide adenine dinucleotide (NADH). Dysfunctional IDH leads to reduced production of alpha-KG and NADH and increased production of 2-hydroxyglutarate, an oncometabolite. This results in increased oxidative damage and stabilization of hypoxia-inducible factor alpha, causing cells to be prone to tumorigenesis. METHODS: This study investigated IDH mutations in 61 Ewing sarcoma family tumors (ESFTs), using a pentose nucleic acid clamping method and direct sequencing. RESULTS: We identified four cases of ESFTs harboring IDH mutations. The number of IDH1 and IDH2 mutations was equal and the subtype of IDH mutations was variable. Clinicopathologic analysis according to IDH mutation status did not reveal significant results. CONCLUSIONS: This study is the first to report IDH mutations in ESFTs. The results indicate that ESFTs can harbor IDH mutations in previously known hot-spot regions, although their incidence is rare. Further validation with a larger case-based study would establish more reliable and significant data on prevalence rate and the biological significance of IDH mutations in ESFTs.
Carcinogenesis ; Constriction ; Decarboxylation ; Humans ; Incidence ; Isocitrate Dehydrogenase ; NAD ; Prevalence ; Sarcoma, Ewing*

OBJECTIVES: This study aimed to analyze the relationship between clinical status and work characteristics of firefighters and other public officers who engaged on collection duties in the site of the hydrogen fluoride spill that occurred on September 27, 2012, in Gumi City, South Korea. METHODS: We investigated the clinical status, personal history, and work characteristics of the study subjects and performed physical examination and several clinical examinations, including chest radiography, echocardiography, pulmonary function test, and blood testing in 348 firefighters, police officers, volunteer firefighters, and special warfare reserved force who worked at the hydrogen fluoride spill area. RESULTS: The subjects who worked near the accident site more frequently experienced eye symptoms (p = 0.026), cough (p = 0.017), and headache (p = 0.003) than the subjects who worked farther from the accident site. The longer the working hours at the accident area, the more frequently the subjects experienced pulmonary (p = 0.027), sputum (p = 0.043), and vomiting symptoms (p = 0.003). The subjects who did not wear respiratory protective devices more frequently experienced dyspnea than those who wore respiratory protective devices (p = 0.013). In the pulmonary function test, the subjects who worked near the accident site had a higher decease in forced vital capacity than the subjects who worked farther from the site (p = 0.019); however, no statistical association was found between serum calcium/phosphate level, echocardiography result, chest radiographic result, and probation work characteristics. CONCLUSIONS: The subjects who worked near the site of the hydrogen fluoride spill, worked for an extended period, or worked without wearing respiratory protective devices more frequently experienced upper/lower respiratory, gastrointestinal, and neurological symptoms. Further follow-up examination is needed for the workers who were exposed to hydrogen fluoride during their collection duties in the chemical plant in Gumi City.
Chemical Hazard Release ; Cough ; Dyspnea ; Echocardiography ; Firefighters* ; Gyeongsangbuk-do ; Headache ; Hematologic Tests ; Humans ; Hydrofluoric Acid* ; Hydrogen* ; Physical Examination ; Plants ; Police ; Radiography ; Radiography, Thoracic ; Republic of Korea ; Respiratory Function Tests ; Respiratory Protective Devices ; Sputum ; Thorax ; Vital Capacity ; Vomiting

Aggressive angiomyxomas (AAMs) are rare, benign, but locally aggressive, hypocellular soft tissue neoplasms found in the vulvovaginal region. AAMs are most commonly found in women in their reproductive years. Only about 150 cases of this rare, soft tissue tumor have been reported thus far, most of which are reported from the gynecologic, obstetric, urologic, and pathologic fields. AAMs are considered to be a slow-growing mesenchymal tumor with a locally infiltrative growth pattern. There are only two reports of metastasic disease. We have managed a case of AAM which occurred during adolescence and was pathologically-confirmed.
Adolescent ; Female ; Humans ; Myxoma ; Soft Tissue Neoplasms ; Vulva

Aggressive angiomyxomas (AAMs) are rare, benign, but locally aggressive, hypocellular soft tissue neoplasms found in the vulvovaginal region. AAMs are most commonly found in women in their reproductive years. Only about 150 cases of this rare, soft tissue tumor have been reported thus far, most of which are reported from the gynecologic, obstetric, urologic, and pathologic fields. AAMs are considered to be a slow-growing mesenchymal tumor with a locally infiltrative growth pattern. There are only two reports of metastasic disease. We have managed a case of AAM which occurred during adolescence and was pathologically-confirmed.
Adolescent ; Female ; Humans ; Myxoma ; Soft Tissue Neoplasms ; Vulva

BACKGROUND: Bacterial infections in diabetic patients are an important cause of increased morbidity and mortality. It has been reported that bacterial infections in diabetics showed more impaired PMN functions such as reduced PMN respiratory burst and decreased microbicidal activity in inflammed tissues. Also, apoptosis(programmed cell death) is postulated to be a key mechanism for neutrophil elimination. It is very important that PMN apoptosis keeps the balance from an area of inflammation. Actuallly, as little was known about PMN apoptosis and respiratory burst in diabetes, we investigated PMN apoptosis and hydrogen peroxide production after endotoxin exposure. METHODS: Peripheral venous blood samples were collected by routine venipuncture from healthy volunteers and diabetics to harvest neutrophils. We respectively measured the PMN apoptosis, the production of hydrogen peroxide, and the cell viability. RESULTS: Normal neutrophils showed a tendency to decreased apoptosis after endotoxin treatment. In patients with diabetes, PMN apoptosis was significantly decreased compared with healthy controls. In addition, the LPS-induced neutrophils in diabetics demonstrated more decreased apoptosis. However, the production of hydrogen peroxide was not different between groups. CONCLUSION: These observations suggest that the decreased PMN apoptosis in diabetics with endotoxin exposure may also affect the increased susceptibility and severity of infections.
Apoptosis* ; Bacterial Infections ; Cell Survival ; Healthy Volunteers ; Humans ; Hydrogen Peroxide ; Inflammation ; Mortality ; Neutrophils* ; Phlebotomy ; Respiratory Burst