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*

BACKGROUND: Due to rapidly growing elderly population, there are increasing numbers of older persons with multiple chronic disorders and geriatric problems arising from polypharmacy. In this study we tried to find out the state of polypharmacy and inappropriate drug prescription and their related factors in community-dwelling elderly by review of drugs taken by older persons visiting a day health center. METHODS: From April 2007 to July 2007, 80 subjects of 65 year-old or over with chronic illness who visited a elderly-wellness and health care center were randomly sampled. All of them were surveyed by structured questionnaires, medical records review, pill counts about all medications they are taking and experience of adverse drug reactions. And all the prescribed medications were reviewed or their drug prescription's appropriateness for each elderly according to Beers criteria. Data results were evaluated by frequency and correlation analyses. RESULTS: The average counts of drugs taken by elderly with chronic disorders were 7.23, minimum 1 to maximum 27 drugs a day. Patients experienced more adverse effects significantly when more prescribed medications were taken (P=0.005), and patients with lack of information about their drugs had taken increased number of medications (P<0.001). Referred to Beers criteria, inappropriate cases of prescription were observed in 26 persons. Those drugs were NSAIDs including aspirin in 17 subjects (21%), amitrityline in 3 (4%), short-acting benzodiazepines in 3 (4%), long acting benzodiazepines in 2 (3%), and anticholinergic antihistamine in 1 (1%). CONCLUSION: Polypharmacy is very common in community-dwelling elderly with chronic disorders. More medications were related to more adverse drug reactions and lack of information about their drugs related to increased number of drug taking. High proportion of inappropriate drug prescriptions was observed in the elderly, which may have resulted from poor education concerning geriatric care of the medical personnels.
Aged ; Anti-Inflammatory Agents, Non-Steroidal ; Aspirin ; Beer ; Benzodiazepines ; Chronic Disease ; Delivery of Health Care ; Drug Prescriptions ; Drug Toxicity ; Humans ; Medical Records ; Polypharmacy ; Prescriptions ; Surveys and Questionnaires

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: 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

PURPOSE: Previous studies have not showed consistent results for the level of expression of sodium/iodide symporter (NIS) in thyroid diseases, especially malignant tumor. We undertook this study to evaluate the distribution of NIS expression in malignant thyroid diseases and compare with that in benign thyroid disease. MATERIALS AND METHODS: Total patients were 119 cases (Men 15, 48+/-13 yrs). Total number of samples were 205 pieces. In malignant thyroid disease, there were 153 samples: 90 in papillary carcinoma, 4 in follicular carcinoma, 2 in medullary carcinoma and 57 in metastatic lymph node. In benign thyroid disease, there were 52 samples: 36 in goiter/cyst, 11 in thyroiditis and 5 in follicular adenoma. Using immunohistochemical methods, we probed 205 samples with monoclonal anti-NIS Ab. Grading of staining was scored as 0 (negative or absent), 1 (weakly positive), 2 (moderately positive) or 3 (strongly positive). Expression rate (ER) of NIS positivity in individual disease entity was expressed as percentage of total number divided by number in 2 plus 3 grade. RESULTS: ERs of malignant thyroid diseases were 63% in papillary carcinoma, 81% in metastatic lymph node, 71% in follicular carcinoma and 100% in medullary carcinoma. ERs of benign thyroid disease were 53% in goiter/cyst, 64% in thyroiditis and 40% in follicular adenoma. ER of malignant thyroid diseases was higher than benign thyroid diseases (71% vs 54%). Grading of NIS expression in papillary carcinoma or goiter/cyst was heterogeneously distributed in considerable cases. Normal tissue also showed heterogeneous distribution of NIS expression, which was not correlated with that of primary lesion. CONCLUSION: In papillary thyroid carcinoma, distribution of NIS expression was heterogeneous and increased, and not different compared with that of benign thyroid disease.
Adenoma ; Carcinoma, Medullary ; Carcinoma, Papillary ; Humans ; Immunohistochemistry ; Ion Transport* ; Lymph Nodes ; Thyroid Diseases* ; Thyroid Gland* ; Thyroid Neoplasms ; Thyroiditis

BACKGROUND: Ebstein's anomaly is characterized by various degrees of apical displacement of the proximal attachment of the tricuspid valve. The disease has an extremely variable course in presenting itself. Therefore, we examined the morphologic and clinical features of Ebstein's anomaly as presented in the fetus to define the factors that determine its outcome. METHODS: We reviewed the history and echocardiographic studies of 10 fetuses (mean gestational age 28.0 weeks) diagnosed in utero with Ebstein's anomaly. The reason for referral was cardiomegaly on routine obstetric scanning in 5 cases, fetal arrhythmia in 3 cases, small main pulmonary artery in 3 cases, vessel view in 1, and twin pregnancy complicated by polyhydramniosis in 1. RESULTS: Of the 10 cases, pregnancy was terminated after diagnosis in 5, 2 cases were carried to term, and 1 case was at 34 weeks of gestation. We were unable to follow-up 2 cases. There were no intrauterine deaths and no misdiagnoses. Massive cardiomegaly and severe tricuspid regurgitation were detected in 7 cases; mild cases were detected in two, and one was nearly normal. Associated cardiac lesions included pulmonary artresia in 4 cases, pulmonary stenosis in 3, and pulmonary hypoplasia in 1. The cases with right ventricular outflow tract (RVOT) obstruction showed a tendency to develop more cardiomegaly and tricuspid regurgitation than with normal RVOT. There was no abnormality in other organ systems and karyotyping. CONCLUSION: Although increased cardiothoracic ratio and associated lesions of the right ventricular outflow tract contribute to the poor outcome in the prenatally detected cases, the absence of these features does not always indicate a good prognosis because progression of the disease can occur with advancing gestational age. No absolute measurement or single echocardiographic feature emerged as a consistent predictive factor for prognosis.
Arrhythmias, Cardiac ; Cardiomegaly ; Diagnosis ; Diagnostic Errors ; Ebstein Anomaly* ; Echocardiography* ; Fetus* ; Follow-Up Studies ; Gestational Age ; Karyotyping ; Pregnancy ; Pregnancy, Twin ; Prognosis ; Pulmonary Artery ; Pulmonary Valve Stenosis ; Referral and Consultation ; Tricuspid Valve ; Tricuspid Valve Insufficiency