No abstract available.
Molecular Biology

Dysplasia epiphysialis punctata is a rare congenital disorder of infancy affecting in particular cartilage, muscle, jointtcapsules and the eyes. A case of dysplasia epiphysialis punctata with involvement of all epiphyses of extremities, spine and pelvis in 2 days old male is to be reported with review of literature.
Cartilage ; Chondrodysplasia Punctata ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Epiphyses ; Extremities ; Humans ; Male ; Pelvis ; Spine

No abstract available.
Humans ; Inpatients*

No abstract available.
Anemia, Aplastic* ; Bone Marrow Transplantation* ; Bone Marrow* ; Immunomodulation*

Mitral valvular heart diseases are known to be one of the easily detectable and the hemodynamic chane to the severity and duration of diseases can directly be described by echocardiography. The objective of this study was to determine the echocardiographic characteristics in 3 groups of mitral valvular diseases, that is mitral stenosis(112 cases), mitral stenoinsufficiency (66 cases). and mitral insufficiency(20 cases). 1) In mitral valve study, closing velocity of anterior leaflet reduced in mitral stenosis than other two groups and DE amplitude was greater in mitral insufficiency but there were no significant differences in 3 groups of mitral valvular diseases. DE and AC slope were slightly more rapid in mitral stenosis group and left ventricular outflow tract was much increased in mitral insufficiency group. 2) In aorta and left atrium study, aortic cusps separation was much increased in mitral insufficiency and left atrium was slightly more dilated in mitral stenoinsufficiency than other two groups. But left atrial dimension in mitral insufficiency was more diminished than that in mitral, stenosis, which is probably due to the short durationn of diseases and small range of materials in mitral insufficiency group. 3) In left ventricle study, thickness of interventricular septem, LSa, Ena, left ventricular dimension, LVPW, LV volume. and stroke volume were more increased in mitral insufficiency than mitral stenosis, because of the left ventricular volume overloading. Vcf and PEP/LVET were higher in mitral insufficiency than other two groups. Ejection fraction and fractional shortening were more diminished in mitral stenoinsufficiency but no significant difference was found. 4) In right ventricle study, there were no remarkable changes in right ventricular dimension and right ventricular internal dimension index in all 3 groups of mitral valvular diseases. 5) Calcification of mitral leaflets was observed in 85.7% of mitral stenosis, 90.9% of mitral stenoinsufficiency and 10% of mitral insufficiency, but heavy calcification was more remarkable in mitral stenosis group(25%). 6) Atrial fibrillation was observed in 47% of total mitral valvular diseases, mitral stenosis being 43.8%, mitral stenoinsufficiency 60.6% and mitral insufficiency 20%. In cases of atrial fibrillation, left atrial dimension was significantly enlarged compared with the group without atrial fibrillation.
Aorta ; Atrial Fibrillation ; Constriction, Pathologic ; Echocardiography* ; Heart Atria ; Heart Valve Diseases* ; Heart Ventricles ; Hemodynamics ; Mitral Valve ; Mitral Valve Insufficiency ; Mitral Valve Stenosis ; Stroke Volume

PURPOSE: We reviewed 100 cases of HLA-matched sibling allogeneic bone marrow transplantation(allo-BMT) in children and wish to share these results. MEHTODS: One hundred children had undergone allo-BMT from HLA-identical siblings between Nov. 1983 and May 1998. There were 50 males and 50 females with a median age of 10 years and a median follow-up of 38 months. Out of 100 cases, 43 children were transplanted for severe aplastic anemia (SAA), 29 for acute myelogenous leukemia (AML), 18 for acute lymphocytic leukemia (ALL), 8 for chronic myelogenous leukemia (CML) and 2 for hemophagocytic lympho-histiocytosis (HLH). RESULTS: SAA : The 5-year event free survival (EFS) of SAA was 91%. The types of events that occurred were 3 thrombotic thrombocytopenic purpura (TTP), 2 venoocclusive disease (VOD) and 1 rejection. AML : In 25 of 29 cases, the 4-year EFS after allogeneic BMT in first remission was 71%. That of the TBI-based and Busulfan-based group was 44% and 77%, respectively. The most favorable results were observed in the Busulfan-based group in first remission with an EFS of 81% (n=18). The types of events that occurred were 4 TTP, 3 VOD, 2 rejections and 1 relapse. ALL : Five-year EFS of children with complete remission (CR; n=14, 7 CR1, 7 CR2) was 81%. CML : For the 6 children who received transplants while in the first chronic phase, the event free survival was 67%. HLH : Both of the two children with HLH survived 9 months and 24 months after BMT, respectively. Acute GVHD (> or =Grade ll) was observed in 13 children. Chronic GVHD developed in 10 children; 8 cases were localized and 2 were extensive type. CONCLUSION: Allo-BMT can cure children with refractory stem cell disorders. The most important factor that influences survival after transplantation is interval between diagnosis and transplantation for patients with severe aplastic anemia and remission state at transplantation for patients with leu-
Anemia, Aplastic ; Bone Marrow Transplantation* ; Bone Marrow* ; Child* ; Diagnosis ; Disease-Free Survival ; Female ; Follow-Up Studies ; Humans ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Myeloid, Acute ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Purpura, Thrombotic Thrombocytopenic ; Recurrence ; Siblings* ; Stem Cells

PURPOSE: We reviewed 100 cases of HLA-matched sibling allogeneic bone marrow transplantation(allo-BMT) in children and wish to share these results. MEHTODS: One hundred children had undergone allo-BMT from HLA-identical siblings between Nov. 1983 and May 1998. There were 50 males and 50 females with a median age of 10 years and a median follow-up of 38 months. Out of 100 cases, 43 children were transplanted for severe aplastic anemia (SAA), 29 for acute myelogenous leukemia (AML), 18 for acute lymphocytic leukemia (ALL), 8 for chronic myelogenous leukemia (CML) and 2 for hemophagocytic lympho-histiocytosis (HLH). RESULTS: SAA : The 5-year event free survival (EFS) of SAA was 91%. The types of events that occurred were 3 thrombotic thrombocytopenic purpura (TTP), 2 venoocclusive disease (VOD) and 1 rejection. AML : In 25 of 29 cases, the 4-year EFS after allogeneic BMT in first remission was 71%. That of the TBI-based and Busulfan-based group was 44% and 77%, respectively. The most favorable results were observed in the Busulfan-based group in first remission with an EFS of 81% (n=18). The types of events that occurred were 4 TTP, 3 VOD, 2 rejections and 1 relapse. ALL : Five-year EFS of children with complete remission (CR; n=14, 7 CR1, 7 CR2) was 81%. CML : For the 6 children who received transplants while in the first chronic phase, the event free survival was 67%. HLH : Both of the two children with HLH survived 9 months and 24 months after BMT, respectively. Acute GVHD (> or =Grade ll) was observed in 13 children. Chronic GVHD developed in 10 children; 8 cases were localized and 2 were extensive type. CONCLUSION: Allo-BMT can cure children with refractory stem cell disorders. The most important factor that influences survival after transplantation is interval between diagnosis and transplantation for patients with severe aplastic anemia and remission state at transplantation for patients with leu-
Anemia, Aplastic ; Bone Marrow Transplantation* ; Bone Marrow* ; Child* ; Diagnosis ; Disease-Free Survival ; Female ; Follow-Up Studies ; Humans ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Myeloid, Acute ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Purpura, Thrombotic Thrombocytopenic ; Recurrence ; Siblings* ; Stem Cells

BACKGROUND: Standardized management and surveillance at a national level is essential to maintain blood product safety. Officials of the Korean Division of Human Blood Safety Surveillance, Korea Centers for Disease Control and Prevention, and Korean laboratory transfusion medicine specialists, currently participate as inspectors in the Korean National Blood Inspection Program for Blood Establishments. However, lack of definitive guidelines and absence of standardized inspector training programs compromise the goal of objective and consistent safety management results. In this study, we propose establishment of written inspection guidelines and a clearly documented accreditation training program. METHODS: Inspector training programs in the US and EU were reviewed online and the results of the Korean National Blood Inspection in our country performed during last 4 years were analyzed. RESULTS: We suggested inspection guidelines for every question of inspection checklists. Also, for the questions similar to those of Laboratory Accreditation Program of the Korean Society for Laboratory Medicine (KSLM), guidelines were proposed as 'Results of Laboratory Accreditation Program of the KSLM could be concerned if inspected laboratory obtained 2 year accreditation lately'. We suggest an 18hr-basic training program composed of lectures, e-learning and a visit to a blood center, as well as 12hr-continuing courses, should be established. CONCLUSION: To establish the Blood Inspection Program in a more systematic manner, thorough management and training of inspectors are essential. We expect the guidelines and training program for inspectors, suggested in our study, will be the cornerstone for creating a more professional quality management system and further ensure the safety of the national blood management system.
Accreditation ; Blood Safety ; Centers for Disease Control and Prevention (U.S.) ; Checklist ; Dietary Sucrose ; Humans ; Korea ; Lectures ; Safety Management ; Specialization

PURPOSE: To analyze the results of radiologic and clinical outcomes after pedicle subtraction osteotomy in iatrogenic flat back syndrome. MATERIALS AND METHODS: We reviewed 12 surgical cases of iatrogenic flat back syndrome from January 1998 to July 2001 with a follow-up of more than 12 months. All cases were females and an average age was 59.8 years (48-66 years). An average follow-up period was20.4 months (12-37 months). Initial diagnosis were as follows: lumbar degenerative kyphosis, in 5 cases; spinal stenosis, in 5 cases; spondylolisthesis, in 2 cases. For clinical evaluation, 10-point pain scale and 5-point scale for activity level, cosmesis and subjective satisfaction were used. Lumbar lordotic angles and sagittal vertical axis (SVA) were measured. RESULTS: In radiologic results, an average lumbar lordotic angle at preoperative, postoperative and the last follow-up were 5.13+/-21.69 degree, -31.33+/-16.32 degree, -27.50+/-16.73 degree respectively. And average SVA at preoperative, postoperative and the last follow-up were 8.46+/-6.04 cm, 0.62+/-2.24 cm, 2.82+/-2.81 cm respectively. In clinical results, preoperative pain (6), activity (2) and cosmesis (1.8) were improved to 3.2, 3 and 3.2respectively at the last follow-up. Subjective satisfaction was 3.9. CONCLUSION: Pedicle subtraction osteotomy seemed to be very useful surgical option for improvement of clinical results through recovering of lumbar lordotic angle and sagittal balance in cases of iatrogenic flat back syndrome.
Axis, Cervical Vertebra ; Diagnosis ; Female ; Follow-Up Studies ; Humans ; Kyphosis ; Osteotomy* ; Spinal Stenosis ; Spondylolisthesis

OBJECTIVE: In this study, the exposure levels of polycyclic aromatic hydrocarbons (PAHs) and urinary 1-hydroxypyrene(1-OHP) were surveyed among the workers using coal tar paint. METHOD: The study subjects for the exposed group were 107 male coal tar workers in 10 factories, and for the comparison group were 201 male clerk workers who had never been exposed to coal tar paint. Ambient PAHs, and pre-shift and end-shift urinary 1-OHP were sample and 16 PAHs were analysed. Smoking history was recorded during the survey day. RESULTS: The geometric mean of ambient concentration of total PAHs was 120.17 microgram/m3. Naphthalene had the highest level among the 16 PAHs. The pre-shift 1-OHP in the exposed group (8.89 micro mol/mol creatinine) was significantly higher than that in the control group (0.29 micro mol/mol creatinine). The end-shift 1-OHP in the exposed group (19.02 micro mol/mol creatinine) was significantly higher than that in the pre-shift (8.89 micro mol/mol creatinine) (Ed- confirm). 1-OHP of smokers was significantly higher than that of non-smokers in both groups. The difference between pre-shift and end-shift 1-OHP in smokers (12.40 micro mol/mol creatinine) was twice as high as that in non-smokers (6.06 micro mol/mol creatinine). The difference of 1-OHP between smokers and nonsmokers was 7.59 micro mol/mol creatinine in pre-shift and 13.96 micro mol/mol creatinine in end-shift. Thus, the effect of smoking and exposure to PAHs on 1-OHP may not be additive. In regression analysis for 1-OHP, the significant independent variables were pre-shift 1-OHP and PAHs. The direction of these variables was positive. When the analysis was performed in workers exposed to higher PAHs, smoking was significant independent variable. CONCLUSION: The above results suggest that not only ambient PAHs but also smoking, one of the most important non-occupational PAHs source, influenced the level of 1-OHP. Moreover, the effect of smoking to 1-OHP changed according to the exposure level of PAHs.
Coal Tar* ; Coal* ; Creatinine ; Humans ; Male ; Paint* ; Polycyclic Hydrocarbons, Aromatic* ; Smoke ; Smoking