Bcl-2 oncoprotein is being localized to mitochondria and interfering with programmed cell death (apoptosis) independent of promoting cell division in the lymphoid and nonlymphoid cells. The bcl-2 oncoprotein expression has been reported in follicular lymphomas as well as in diffuse non-Hodgkin's lymphoma, leukemia and a variable propotion of Hodgkin's lymphoma cases. Recent evidence suggests that some lymphomas protected from apoptosis is conferred through expression of Epstein-Barr virus(EBV) latent membrane protein which turn to cause upregulation of bcl-2. To define the role of the bcl-2 oncoprotein and EBV in lymphoid malignancy, we tried immunohistochemical studies with anti-bcl-2 antibody and In situ hybridization (ISH) with EBV-encoded small nuclear RNAs(EBER) in the paraffin embedded sections of 46 non-Hodgkin's lymphoma (NHL) cases and 20 Hodgkin's lymphoma (HL) cases. Bcl-2 oncoprotein expression was found in 37 of 46 cases (80%) of NHL with relatively strong cytoplasmic staining, and in 14 of 20 cases (70%) of HL with weak cytoplasmic staining in limited small numbers of RS, Hodgkin and lacunar cells. The widespread presence of bcl-2 oncogene in many different types of both NHL and HL supports that the extended cell survival through overexpression of bcl-2 gene protein may be a growth advantage of neoplastic lymphoid cells. In the ISH analysis for EBV, the presence of EBV was detected in 17 of 20 cases (85%) of HL, compared to 6 of 44 cases(13.6%) of NHL. It appears to be no direct correlation between overexpression of bcl-2 oncoprotein by neoplastic lymphoid cells and the presence of EBV in NHL but it seems to be a definite association between EBV and HL.

Clear cell sarcoma of kidney(CCSK) is a rare pediatric neoplasm characterized by a predominating component of clear cells, a predilection for metastases to bone, and a poor prognosis. The incidence of CCSK peaks during the 2nd year of life and adult cases are very rare. We report a case of CCSK encountered in the right kidney of a 39-year-old man. Grossly, it was a lobulated mass showing infiltrative margin, measured 7x5.5x5cm and had a homogeneous gray-tan color with a soft, fish-flesh consistency. Microscopically, about half of the tumor revealed the classic pattern of CCSK, having tumor cell cords or nests separated by the characteristic alveolar capillary networks. The tumor cells had clear pale cytoplasm, bland looking round nuclei and inconspicuous nucleoli. The other half showed the epithelioid-trabecular pattern forming pseudorosette or cord-like structures. Immunohistochemically, there was only a focal positive reaction to vimentin. Ultrastructurally, the tumor cells showed the primitive nephrogenic mesenchymal differentiation such as electron lucent cytoplasm, a small amount of organelles, scanty heterochromatin, inconspicuous nucleoli, and a lack of flocculant basal lamina material around the cytoplasmic membrane. We consider that this is a case of CCSK occuring in the oldest patient ever reported, confirmed by both immunohistochemistry and electron microscopy.
Adult ; Male ; Female ; Humans ; Incidence ; Neoplasm Metastasis

The purpose of this study is to compare the educational needs and perception of mothers of normal neonates and high risk infants. This research was designed as a descriptive study. Data were collected for two months from April 2002 to March 2002. Subjects were 41 mothers of high risk infants and 60 mothers of normal neonates in one general hospital in Seoul participated in the study. Measurement tools used in this study were the educational needs scale developed by Cho Kyoul Ja et al and the neonatal perceptive inventories scale developed by Broussard. They ask mothers to rate each item on a four point Likert type scale. The collected data were analyzed using SPSS 8.0 program. The variables were listed as frequency, mean, standard deviation, X2 test, t-test, ANOVA. The results were as follows: The educational needs of the mothers of primi pregnancy was higher than the mothers of multi pregnancy. The educational needs on management of diseases such as prevention of infection, symptoms of disease, mental development, attachment promotion, congenital metabolism test, management of convulsion, care of vomiting and fever were higher than general care of infants such as immunization, measurement of temperature, hiccough care, follow up care. The informations must be included in nursing intervention program to reduce the mothers' stress level. In conclusion, in order to promote positive mother infant relationship, nurse need to give information and educate the mothers of high risk infant and normal neonate.
Equipment and Supplies ; Fever ; Hiccup ; Hospitals, General ; Humans ; Immunization ; Infant* ; Infant, Newborn* ; Metabolism ; Mothers* ; Nursing ; Pregnancy ; Seizures ; Seoul ; Vomiting ; Child Health

BACKGROUND AND OBJECTIVES: Mutations of the SLC26A4 gene cause congenital hearing loss and enlarged vestibular aqueduct (EVA). A considerable proportion of patients with SLC26A4 mutations have significant residual hearing at birth that eventually worsen and become the cause for cochlear implantation (CI) later in their adolescence or adulthood. We analyzed the auditory outcome and prognostic factors of CI in patients with EVA and biallelic SLC26A4 mutations showing progressive early-onset hearing loss, who eventually had implantation in their adolescent or adult periods. SUBJECTS AND METHOD: Sixteen patients with EVA carrying biallelic SLC26A4 mutations who received CI after 12 years of age were included for analysis. The outcome and prognostic factors of CI were analyzed. The postoperative follow-up period ranged from 3 to 48 months. RESULTS: The age at CI ranged from 12 to 44 years. The categories of auditory performance score was significantly improved after CI from 3.1 to 4.9 (p < 0.05). The mean sentence scores improved significantly in the auditory-visual and auditory-only conditions (p < 0.05). The significant prognostic factors were measurable bone conduction thresholds, preoperative residual hearing, recent history of sudden aggravation of hearing loss, and preoperative speech intelligibility rating scores. There was a tendency of lower postoperative sentence scores in the group with homozygous H723R mutation, but statistical significance was not reached. CONCLUSION: Despite the early-onset of hearing loss, significant improvement in auditory performance can be expected after CI in adolescent and adult patients with EVA and biallelic SLC26A4 mutations. Significant prognostic factors should be considered in selecting candidates and preoperative counseling for CI.
Adolescent* ; Adult* ; Bone Conduction ; Cochlear Implantation* ; Cochlear Implants* ; Counseling ; Extravehicular Activity ; Follow-Up Studies ; Hearing ; Hearing Loss ; Humans ; Methods ; Parturition ; Speech Intelligibility ; Vestibular Aqueduct*

PURPOSE: Leigh syndrome is a kind of mitochondrial disease with a pathological production of lactate and variable clinical manifestations. Recently biochemical assay of the mitochondrial enzyme activity and MRS are becoming a powerful tool for the definitive antemorterm diagnosis of this disease. The aim of this study was designed to determine the usefulness of biochemical enzyme assay and MRS in Leigh syndrome. METHODS: A clinical study of 8 cases of pediatric patients were diagnosed as Leigh syndrome on the basis of clinical manifestations and MRI features at the department of pediatrics, Ulsan university hospital, from July, 1992 to June, 1997. Biochemical enzyme assay of the cultured skin fibroblasts was consulted to hospital for sick children, Toronto, Canada. Cerebral lactates were detected on MRS were analysed in relation to the MRI findings and lactate levels in the CSF. RESULTS: 1) Age at diagnosis ranged from 7 months to 8.9 years; seven were boys and one was girl. 2) Main clinical symptoms were as follows in the order of frequency; seizure(75%), ophthalmoplegia(50%), hemiplegia(50%), hypotonia(50%), respiratory difficulty(50%), developmental delay(38%), consciousness change(38%), mental retardation( 25%). 3) Anatomical locations of the focal lesions detected on the initial MRI were as follows in the order of frequency; putamen(75%), caudate nuclei(63%), medulla oblongata (18,50%), substantia nigra(38%), thalamus(38%). 4) Biochemical enzyme assay revealed deficiency of NADH cytochrome c reductase (complex I) and deficiency of cytochrome c oxidase(complex N) respectively in 2 patients who had markedly elevated serum and CSF lactate levels and lactate/pyruvate ratio. 5) Cerebral lactates were detected by MRS from 6 lesions of 1 week-4 months old in 6 patients who had normal or mildly elevated serum and CSF lactate levels and lactate/pyruvate ratio. CONCLUSION: MRS detected the brain parenchymal lactate non-invasively from the acute or subacute lesions. Therefore, combined evaluation by MRS and biochemical enzyme assay will provide an important information for the diagnosis and estimation of disease activity in pediatric patients who are suspected to have Leigh syndrome.
Brain ; Canada ; Child ; Consciousness ; Cytochromes c ; Diagnosis ; Enzyme Assays* ; Female ; Fibroblasts ; Humans ; Lactates ; Lactic Acid ; Leigh Disease* ; Magnetic Resonance Imaging ; Medulla Oblongata ; Mitochondrial Diseases ; NADH Dehydrogenase ; Pediatrics ; Skin ; Ulsan

OBJECTIVES: The purpose of this study was to observe the reaction kinetics and the degree of polymerization of composite resins when cured by different light sources and to evaluate the effectiveness of the blue Light Emitting Diode Light Curing Units (LED LCUs) compared with conventional halogen LCUs. MATERIALS AND METHODS: First, thermal analysis was performed by a differential scanning calorimeter (DSC). The LED LCU (Elipar Freelight, 320 mW/cm2) and the conventional halogen LCU (XL3000, 400 mW/cm2) were used in this study for curing three composite resins (SureFil, Z-250 and AEliteFLO). Second, the degree of conversion was obtained in the composite resins cured according to the above curing mode with a FTIR. Third, the measurements of depth of cure were carried out in accordance with ISO 4049 standards. Statistical analysis was performed by two-way ANOVA test at 95% levels of confidence and Duncan's procedure for multiple comparisons. RESULTS: The heat of cure was not statistically different among the LCUs (p > 0.05). The composites cured by the LED (Exp) LCUs were statistically more slowly polymerized than by the halogen LCU and the LED (Std) LCU (p < 0.05). The composite resin groups cured by the LED (Exp) LCUs had significantly greater degree of conversion value than by the halogen LCU and the LED (Std) LCU (p = 0.0002). The composite resin groups cured by the LED (Std) LCUs showed significantly greater depth of cure value than by the halogen LCU and the LED (Exp) LCU (p < 0.05).
Composite Resins* ; Hot Temperature ; Kinetics ; Polymerization* ; Polymers* ; Spectroscopy, Fourier Transform Infrared

PURPOSE: Carcinosarcomas are rare neoplasms in which both malignant epithelial and mesenchymal elements are identified. We have found only twenty one cases of primary cutaneous carcinosarcoma in the English language literature. Therefore it has been difficult to diagnosis because its unclear etiology and low frequency. METHODS: A 31-year-old young man with a protruding mass on his ankle dorsum was examined. The tumor was 7 x 6 x 3 cm sized and arose from a burn scar. The mass was wide excised and applied split thickness skin graft. Characteristic pathologic finding was a mixture of squamous cell carcinoma and malignant fibrous histiocytoma. RESULTS: Three months after the operation, the patient died of multiple metastasis to the liver, lung and finally of sepsis despite adjuvant chemotherapy and adjuvant radiotherapy. CONCLUSION: If a metastatic cutaneous carcinosarcoma is diagnosed, wide excision is required. There should be more aggressive management to minimize the risk of recurrence. Further research into the etiology and pathophysiology of the disease and a more careful differential diagnosis may allow improvement in treatment.
Adult ; Animals ; Ankle ; Burns ; Carcinoma, Squamous Cell ; Carcinosarcoma ; Chemotherapy, Adjuvant ; Cicatrix ; Diagnosis, Differential ; Histiocytoma, Malignant Fibrous ; Humans ; Liver ; Lung ; Neoplasm Metastasis ; Recurrence ; Sepsis ; Skin ; Transplants ; Ulcer

BACKGROUND AND OBJECTIVES:In order to analyze and understand the oculomotor system completely, it is essential to measure horizontal, vertical, and torsional eye movements. We developed a new system of 3-dimensional videooculography that can accurately estimate the pupil center and measure torsional eye movement in real time while minimizing the errors caused by upper eyelid droop, eyelashes, corneal reflection, and eye blinking. MATERIALS AND METHOD:For accurate estimation of the center and diameter of the pupil, the edges of the pupil were searched by using a sector-form window and circle fitting was performed using the least square regression. Torsional eye movement was measured using polar cross-correlation technique with modification to lessen the errors produced by the change in pupil size. Calibrations were performed and to verify the proposed system, the linearity between the measured and actual eye movements was measured. Also, the results of the proposed system were compared with those of another commercialized system. RESULTS: The linearity between the measured and actual eye movements showed errors of less than 1%. The spatial resolutions of the system for horizontal, vertical, and torsional eye movements were 0.3, 0.4, and 0.2, respectively whereas the temporal resolution was 30 frames/sec. In comparison to the commercialized system, our system showed less artifact by eye blinking when measuring vertical eye movement. CONCLUSION:We proposed an algorithm and a system for measuring horizontal, vertical, and torsional eye movements which minimized the influence of partial eyelid closure, eyelashes, corneal reflections, and change in pupil size.
Artifacts ; Blinking ; Calibration ; Eye Movements* ; Eyelashes ; Eyelids ; Pupil

Dysgerminoma developed in a 21-year-old phenotypic female patient with 46,XY pure gonadal dysgenesis, Swyer syndrome. This patient presented with pelvic mass associated with abdominal pain and primay amenorrhea. Clinical characteristics showed a typical stigmata of gonadal dysgenesis: primary amenorrhea, sexual infantilism, a small uterus and left streak gonad. A 46,XY karyotype was made by lymphocyte culture. The patient was counseled to undergo operation, chemotherapy and hormon therapy. She underwent bilateral gonadectomy with total hysterectomy, partial omentectomy and multiple pelvic wall random biopsy. Histological examination revealed dysgenetic gonads with dysgerminoma. After surgery, the patient received chemotherapy and also was started on hormone replacement therapy. She is currently alive with no evidence of disease after 19 months from surgery.
Abdominal Pain ; Amenorrhea ; Biopsy ; Christianity ; Drug Therapy ; Dysgerminoma* ; Female ; Gonadal Dysgenesis ; Gonadal Dysgenesis, 46,XY* ; Gonads ; Hormone Replacement Therapy ; Humans ; Hysterectomy ; Karyotype ; Lymphocytes ; Sexual Infantilism ; Uterus ; Young Adult

PURPOSE: This study was undertaken to provide prerequisites for accreditation of medical genetics training program and certification process for medical genetics professionals as clinical specialist and set up guidelines on curriculum of medical genetics training program in Korea. METHODS: Six ad hoc committees for clinical geneticist, clinical cytogeneticist, clinical molecular geneticist, clinical biochemical geneticist, medical genetics technologists and genetic counselors were organized for reviewing current status in Korea as well as foreign countries. Each committee is composed of 6-8 members. They summarized their opinions according to the structured questionnaire inquiring the ways of accrediting training program, qualification of program director, trainee requirements, contents of curriculum, duration of training program, certification process, estimation of numbers of each specialist needed in next 5 years in Korea. RESULTS: Both prerequisites for the accreditation of medical geneticist training institutions and qualification of program director are suggested. Candidacy of trainees requires MD with board of medical specialty, or PhD degree with professional experiences in related field except clinical genetics program which only accepts MD with board of medical specialty, and Non-MD genetic counselor and medical technologists with degrees of BS or MS. General duration of fellowship will be 2-3 years depending on the categories they are enrolled into. Contents of curriculum for each speciality training are described. For the certification of each category, the candidacy should submit a log book detailing the cases they experienced during the fellowship, prove that they successfully completed course work and clinical experiences in the accredited program, and pass the written examination. CONCLUSION: As medical genetics becomes more important in daily routine clinical practice, the accreditation of medical genetics training program and certification of personnel are urgently needed. In this regard, the study will be providing guidelines and prerequisites for accreditation of medical genetics training program and certification process for medical genetics professionals as clinical specialist.
Accreditation ; Certification* ; Counseling ; Curriculum ; Education* ; Fellowships and Scholarships ; Genetics ; Genetics, Medical* ; Humans ; Korea* ; Medical Laboratory Personnel ; Specialization* ; Surveys and Questionnaires