Bart's syndrome was initially described as a genodermatosis characterized by congenital localized absence of the skin with blistering and nail deformities 1-3. However, it is considered as any type of epidermolysis bullosa(EB) with localized congenital absence of the skin on the extremities. A 33-day-old fbmale baby was presented with congenital absence of the skin over the left shin and dorsa of both feet which were covered with the thin, translucent, and brown-red glistening membranes. Blistering of the right calf and left great toe nail deformity were also noted. She was diagnosed as a recessive dystrophic EB by the histopathological, ultra- structural and immunomapping studies.
Blister ; Congenital Abnormalities ; Extremities ; Foot ; Membranes ; Skin ; Toes

No abstract available.
Meconium* ; Retrospective Studies*

In 1989, the Bethesda System (TBS) was introduced as an attempt to standardize cervical/vaginal reporting systems. TBS nomenclature was created for reporting cytologic diagnoses to replace the currently used Cervical Intraepithelial Neoplasia (CIN) and Papanicolaou Class System, which are deemed less reproducible. The name for preinvasive squamous lesions was changed to squamous intraepithelial lesion (SIL), subdivided into low-grade and high-grade types. TBS recommends a specific format for cytologic report, starting with explicit statement on the adequacy of the specimen, followed by general categorization and descriptive diagnosis. Pathologic and epidemiologic studies performed over last 10 years have provided evidence that human papillomavirus (HPV) plays a significant role in the development of cervical neoplasia. TBS corresponds not only to currently held views of the behavior of preinvasive lesions and their HPV distribution, but also to the current guidelines for clinical management.
Azathioprine* ; Body Fluids ; Cadherins ; Cervical Intraepithelial Neoplasia ; Diagnosis ; Epidemiologic Studies ; Humans ; Immunohistochemistry ; Nephritis* ; Nephrotic Syndrome* ; Purpura, Schoenlein-Henoch*

BACKGROUND: Surgical endarterectomy had been known to be the standard treatment modality in management of carotid stenosis. However, endarterectomy had several limitations in high-risk patients, particulary with coronary artery disease. Carotid angioplasty and stenting has been suggested to be a safer and more cost-effective alternative to carotid endarterectomy in the management of symptomatic carotid artery disease. The purpose of this study is to evaluate the feasibility and safety of elective carotid artery stent implantation in patients with carotid artery stenosis. METHOD: We treated 19 patients with symptomatic and asymptomatic stenosis of >60% in 19 carotid arteries with balloon angioplasty followed by elective stent implantation. Of all carotid stenting procedures, 18 stents were implanted in obstructing atherosclerotic plaques and in one for Takayasu's arteritis. Of all patients, 10 patients were symptomatic with a history of stroke or transient ischemic attacks which were ipsilateral to the treated carotid artery. 12 patients represented a high-risk subset with myocardial infarction, previous coronary artery bypass graft and coronary artery stenosis. 6 months follow up angiogram was done in 7 patients, a patient(Takayasu's arteritis) showed restenosis. Result: Angiographic and procedural success rate were 100%, and there were no acute or subacute stent thrombosis. Immediately after initial carotid stenting, the mean(+/-SD) stenosis was reduced from 74.9+/-13.6% to 10.2+/-8.7% and the minimal luminal diameter was increased from 1.4+/-0.8mm to 5.3+/-1.0mm corresponding to an acute gain of 3.9mm. There were no major or minor stokes during follow-up. CONCLUSION: Percutaneous carotid angioplasty with stenting is a safe and feasible procedure. It is associated with high immediate success rates and relatively low complications in the management of carotid artery stenosis. Carotid stenting seems to be a reasonable alternatives to medical management for the treatment of carotid stenosis in patients deemed to be poor candidates for standard carotid endarterectomy.
Angioplasty ; Angioplasty, Balloon ; Carotid Arteries* ; Carotid Artery Diseases ; Carotid Stenosis* ; Constriction, Pathologic ; Coronary Artery Bypass ; Coronary Artery Disease ; Coronary Stenosis ; Endarterectomy ; Endarterectomy, Carotid ; Follow-Up Studies ; Humans ; Ischemic Attack, Transient ; Myocardial Infarction ; Phenobarbital ; Plaque, Atherosclerotic ; Stents* ; Stroke ; Takayasu Arteritis ; Thrombosis ; Transplants

Adult T-cell leukemia/lymphoma (ATLL) is an often aggressive and fatal malignancy of mature CD4+ T lymphocytes, characterized by leukemia, lymphadenopathy, hypercalcemia and lytic bone lesions. Diagnosis of ATLL is based cm abnormal T lymphocytes in the peripheral blood and histologically and cytologically proven lymphoid malignancy with T cell surface antigens. Anti-HTLV-I serum antibodies and demonstration of clonality to HTLV-I proviral DNA are included. We report a case of ATLL in a 64-year-old man who had a painful nodule on the right cheek and multiple scattered papules and nodules on the trunk for 2 months. He had multiple cervical and axillary lymph node enlargements and hepatosplenomegaly. On laboratory finding peripheral leukocytes count was 33,400/mm3 with 63% atypical lymphocytes. Clover-shaped lyrnphocytes with multilobulated nuclei were found in peripheral blood and bone marrow. Histologic findings showed dense bandlike infiltrations of atypical lymphocytes in the dermis with angiocentricity. The surface phenotype of these abnormal cells were CD2+, CD3+, CD4+, CD30+ and HLA-DR+ and HTLV-I specific antibodies were found.
Adult* ; Antibodies ; Antigens, Surface ; Bone Marrow ; Cheek ; Dermis ; Diagnosis ; DNA ; Human T-lymphotropic virus 1 ; Humans ; Hypercalcemia ; Leukemia ; Leukemia-Lymphoma, Adult T-Cell* ; Leukocytes ; Lymph Nodes ; Lymphatic Diseases ; Lymphocytes ; Lymphoma* ; Middle Aged ; Phenotype ; T-Lymphocytes

BACKGROUND: Herpes zoster is a relatively common disease and there have been several reports on clinical study of herpes zoster. However no clinicopathological study has been reported yet in Korea. OBJECTIVE: The purpose of this study is to investigate the clinical and pathological findings of herpes zoster. METHODS: We performed a retrospective study of eighty patients with herpes zoster in whom skin biopsies were done from 1995 through 1999. We obtained the clinical features from record reviews and reevaluated biopsy specimen of each patient. RESULTS: 1. Herpes zoster was most frequently observed in 6th decade(40.0%). There was no sexual difference. 2. The dermatome of thoracic nerve was most frequently involved(55.0%), and followed by the dermatomes of cervical nerve(28.7%), lumbar nerve(16.2%) and trigerminal nerve(8.7%). 3. Postherpetic neuralgia was the most frequent complication of herpes zoster and was noted in 22 of the total cases(27.5%). 4. Histologically, in the epidermis, ballooning degeneration was observed in 97.5%, and reticular degeneration in 81.2%. Multinuclear giant cells and inclusion bodies were observed in 42.5% and 32.5%, respectively. In the dermis, herpetic folliculitis was observed in 28.7% and syringitis in 3.7%. Leukocytoclastic vasculitis was observed in 22.5%. CONCLUSION: The results of clinical aspects were similar to those of previous studies. Histopathologically, most of the cases showed typical viral changes in epidermis and in some cases, herpetic folliculitis and syringitis were noted.
Biopsy ; Dermis ; Epidermis ; Folliculitis ; Giant Cells ; Herpes Zoster* ; Humans ; Inclusion Bodies ; Korea ; Neuralgia, Postherpetic ; Retrospective Studies ; Skin ; Thoracic Nerves ; Vasculitis

The incidence of inadvertent posterior capsular rupture at each stage of planned ECCE and posterior chamber intraocular lens implantation was studied. From June 1985 to Dec. 1986, 133 cases of planned ECCE for posterior chamber intraocular lens implantation were performed. Posterior capsular rupture with or without vitreous loss was developed in 11 cases(8.2%) out of 133. The rupture occurred during nuclear expression in 2 cases(1.5%), during cortical clean-up in 6 cases(4.5%), during remval removal of anterior capsular flaps in 2 cases(1.5%), during posterior chamber intraocular lens implantation in 1 case(0.7%), but during posterior capsule polishing in nocase(0%). Vitreous loss was combined with posterior capsular rupture in 6 cases(4.5%), but the other 5 cases were not associated with vitreous loss(3.7%). After management of vitreous loss by automated anterior vitrectomy in 10 cases, anterior chamber intraocular lens was implanted. But in the other 1 cases with mid posterior capsular rupture without vitreous loss, anterior chamber intraocular lens was implanted without anterior vitrectomy. A final visual acuity of 0.5 or better by 2 months postoperatively was observed in 10 cases out of 11(91%).
Anterior Chamber ; Incidence ; Lens Implantation, Intraocular* ; Lenses, Intraocular* ; Rupture* ; Visual Acuity ; Vitrectomy

No abstract available.
Osteoporosis* ; Radionuclide Imaging*

The mesenchymal chondrosarcoma of the orbit is so rare that only a few cases have been reported in Korea and world-wide. We experienced that a patient whose complaints were proptosis and diplopia caused by orbital tumor. After tumor removal, it was histologically diagnosed as mesenchymal chondrosarcoma. So we report a case of mesenchymal chonrosarcoma of the orbit with a review of the literature.
Chondrosarcoma, Mesenchymal* ; Diplopia ; Exophthalmos ; Humans ; Korea ; Orbit*

PURPOSE: The purpose of this study is to investigate the trends and traits of moral sensitivity and reasoning over six years of medical school. METHODS: The participants in this study consisted of 217 first to sixth-year medical students of Sungkyunkwan University School of Medicine in Korea. Essay type questionnaire was used for evaluating moral sensitivity and the Korean-Defining Issues Test (KDIT) was used for evaluating moral reasoning. RESULTS: It was found that the medical students' sensitivity of 'public welfare', 'prospective result' and 'role-taking as a doctor' was significantly higher than that of the pre-medical students'. Moral sensitivity increased in the 3rd and the 4th years but decreased in the 5th and the 6th years. Moral reasoning decreased in the 3rd and the 4th years but increased in the 5th and the 6th years. No correlation was observed between moral sensitivity and reasoning(r=0.033). Developmental trends of moral sensitivity and reasoning showed a significant difference in terms of year of medical school. CONCLUSION: The results suggest that medical school curriculum tends to impct students' moral sensitivity. This school has implemented the problem-based learning curriculum into the 3rd and the 4th years. It is necessary to consider the developmental state of the student's morality when developing an effective medical ethics program.
Curriculum ; Ethics, Medical ; Humans ; Korea ; Morals ; Problem-Based Learning ; Schools, Medical ; Students, Medical* ; Surveys and Questionnaires