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

The effects of vanadate on cellular Ca2+ movements across the sarcolemma of cardiac muscle cells were investigated by measuring the intracellular and extracellular Ca2+ activities of guinea pig papillary muscle with Ca2+-selective electrodes. During the rest period following a steady-state of 2 contractions per second the extracellular Ca2+ concentration was increased over the basal level within a minute. During the rest period Ca2+ was transported across the sarcolemma into the extracellular space. Vanadate decreased the change in extracellular Ca2+ concentration during the rest period implying that the Ca2+ efflux across the sarcolemma was decreased by vanadate. Vanadate increased intracellular Ca2+ activities significantly (from 1.9 X 10(-7) M to 10(-6)M) resulting in an increase in resting tension. These results suggest that vanadate decreases Ca2+ efflux from the cells into the extracellular space by blocking Ca2+ transport across the sarcolemma, possibly blocking the Na+-Ca2+ exchange transport.
Animal ; Calcium/metabolism* ; Female ; Guinea Pigs ; Ion Channels/drug effects* ; Male ; Membrane Potentials/drug effects ; Papillary Muscles/drug effects* ; Vanadates ; Vanadium/pharmacology*

Gastrointestinal stromal tumors are notorious for their unpredictable clinical behavior. To assess the cellular proliferating activities, four different methods were used: mitotic count, nucleolar organizer region(AgNOR) staining, immunostaining of proliferating cell nuclear antigen (PCNA) and DNA ploidy were used on 39 cases of gastrointestinal stromal tumors. Additionally, we analysed cellularity, cellular atypism and necrosis. Among 39 cases of gastrointestinal stromal tumors, 11 cases were diagnosed as benign lesions according to clinicopathologic findings. Malignant lesions were arbitrarily classified into low grade(n=ll) and high grade(n=17) on the basis of absence or presence of recurrence, metastasis or tumor-related death during the follow-up period. Numbers of mitosis, AgNORs, PCNA index and DNA ploidy were correlated with grades of tumor and prognosis. Among them, AgNORs counting appeared to be the most useful in predicting prognosis. Numbers of mitosis, PCNA index and DNA ploidy showed varying degrees of overlap among the 3 groups. Among the histological parameters, cellular atypia showed some relationship with the prognosis that others did not reveal.
Neoplasm Metastasis

No abstract available.
Hepacivirus* ; Hepatitis C* ; Hepatitis* ; Korea* ; Prevalence*

No abstract available.
Hepatitis E virus* ; Hepatitis E* ; Hepatitis* ; Korea* ; Prevalence*

PURPOSE: To report two cases of nasal type Natural Killer (NK)/T-cell lymphoma of the orbit. METHODS: (Case 1) A 42-year-old woman presented with a 3-month history of painful proptosis and eyelid swelling in the right eye. Under the diagnosis of inflammatory pseudotumor, she was treated with intravenous and oral steroids. She visited our clinic with a 3-day history of proptosis with decreased visual acuity. Computed tomography (CT) revealed a large infiltrative mass in the right orbit. Incisional biopsy was performed in the right lower lid. (Case 2) A 42-year-old man was referred for consultation with a 1-month history of fever of unknown origin. Two months previously uveitis had developed in the right eye and had been treated with topical steroid. CT revealed an infiltrative soft-tissue like mass in the left orbit. Incisional biopsy was performed in the orbital area. RESULTS: Histopathologically, a diffuse infiltration of atypical lymphocytes was observed with angiocentric pattern in both patients. The infiltrating cells were positive on immunohistochemical stains for CD3 and CD56. The tumor cells were negative for CD20 and CD30. In situ hybridization demonstrated Epstein-Barr virus-encoded nuclear RNA. A diagnosis was made with NK/T-cell lymphoma of nasal type associated with Epstein-Barr virus infection. CONCLUSIONS: NK/T-cell lymphoma of the orbit is rare and has poor prognosis. It is important to distinguish a NK/T-cell lymphoma from the pseudotumor or uveitis unresponsive to steroid therapy.
Adult ; Biopsy ; Coloring Agents ; Diagnosis ; Exophthalmos ; Eyelids ; Female ; Fever of Unknown Origin ; Granuloma, Plasma Cell ; Herpesvirus 4, Human ; Humans ; In Situ Hybridization ; Lymphocytes ; Lymphoma* ; Orbit* ; Prognosis ; RNA, Nuclear ; Steroids ; Uveitis ; Visual Acuity

The Accreditation Board for Medical Education in Korea, ABMEK, is nongovernmental appraisal organization that was established at July 2, 1998. The organization is contributing to the improvement of medical education by progressing the first cycle accreditation successfully. But, the organization has various problems and subjects related to the accreditation system. The authors examined the related literature focusing on the current status and problems of accreditation system. The result of this research was as follows. First, the ABMEK needs to propel legal personality of organization and should install independent executive office. Second, the ABMEK should establish the alteration procedure of accreditation standards and develop the accreditation standards of the second cycle that take into account international flowing of medical education. Third, the ABMEK must decide forms and scope to investigate medical college present situation. Finally, to propel development tasks effectively, it needs to get the recognition of Ministry of Education and Human Development.
Accreditation* ; Education ; Education, Medical ; Human Development ; Korea* ; Schools, Medical*

OBJECTIVES: To assess height, weight and body mass index from childhood to adolescence according to the age at menarche and hence to study the influence of childhood growth on the menarche age. METHODS: "The Kangwha Study" was a community-based prospective cohort study which included the entire population of 219 female first graders in Kangwha county in 1986. Among the 219 girls, 119 girls who had received complete follow up checks during the study period(1986~1997), were included in this study, except one for whom menarche age information was unavailable. The remaining 118 girls were divided into three groups according to the timing of menarche : early(<25 percentile), intermediate and late(> or =75 percentile) maturers. RESULTS: The average age at menarche was 12.7 years : early 11.3 years, intermediate 12.6 years and late 13.7 years. The early maturers were taller and heavier between 6~8 years. But, the mean weight and body mass index at the menarche age did not differ statistically among the three groups. The weight and body mass index of the early maturers were consistently higher than those of the late maturers over the entire period of the study. CONCLUSIONS: Critical body weight and body mass index must be attained for menstruation to be attained and the age at menarche is largely determined by the childhood growth. In addition, it seems that childhood growth and age at menarche are associated with adolescent weight and body mass index.
Adolescent* ; Body Mass Index ; Body Weight ; Cohort Studies ; Female ; Follow-Up Studies ; Humans ; Menarche* ; Menstruation ; Prospective Studies

We have experienced three cases of mucinous ductal ectasia of the pancreas. They showed the characteristic duodenoscopic findings and underlying pathology was hyperplasia in two cases and adenocarcinoma in one case. When endoscopic retrograde pancreatography was performed, bulging ampulla of Vater, patulous ampullary orifice and mucus leakage from papillary orifice were noted. Also cyst-like dilatation of main duct or side branch of the uncinate process were observed.
Adenocarcinoma ; Ampulla of Vater ; Dilatation ; Dilatation, Pathologic* ; Hyperplasia ; Mucins* ; Mucus ; Pancreas* ; Pathology

BACKGROUNDS: A mucin-producing bile duct tumor is a rare disease causing diffuse bile duct dilatation and intermittent obstructive jaundice due to mucin accumulation in the bile duct. A massive amount of mucin in the dilated bile duct is confirmed during surgery, endoscopic drainage, or percutaneous drainage. These tumors are usually slow-growing, well-differentiated papillary adenocarcinomas that shows intraductal spreading or an intraductal papillary growth pattern. PURPOSE: The purpose of this study was to evaluate the clinical, radiological, and histopathological characteristics of mucin-producing bile duct tumors. METHODS: We treated 10 patients with mucin-producing bile duct tumors during the recent 5 years. Clinical features were reviewed, including symptoms and signs, radiologic characteristics, operative findings, and pathological characteristics. RESULTS: The main clinical symptoms were recurrent abdominal pain, fever, chill, and intermittent jaundice. The characteristic radiologic findings were marked dilatation of the bile ducts distal to the tumors on computed tomography (CT) and ultrasonography and multiple, large, amorphous filling defects on cholangiography. In three cases, percutaneous transhepatic cholangioscopy (PTCS) was useful not only in making a diagnosis but also in delineating the extents of the tumors. In seven of ten cases involved (70%), the tumors were located mainly in the left intrahepatic duct. Pathologically, six (60%) cases were differentiated papillary adenocarcinoma. CONCLUSIONS: A mucin-producing bile duct tumor should be suspected if the patient has diffuse bile duct dilatation without definite evidence of stones, and it can be confirmed by a large amount of mucin secretion. If this type of tumor is suspected, careful preoperative evaluation, including PTCS, should be considered for making an accurate preoperative diagnosis and for determining the extent of the tumor.
Abdominal Pain ; Adenocarcinoma, Papillary ; Bile Duct Neoplasms ; Bile Ducts* ; Bile* ; Cholangiography ; Diagnosis ; Dilatation ; Drainage ; Fever ; Humans ; Jaundice ; Jaundice, Obstructive ; Mucins ; Rare Diseases ; Ultrasonography