No abstract available.

BACKGROUND: Hereditary spherocytosis(HS) is a clinically and biochemically very heterogeneous disorder The purpose of this study is to detect erythrocyte membrane protein abnormalities by SDS-PAGE and to investigate the frequency of erythrocyte membrane protein defects in hereditary spherocytosis and correlation between some of the hereditary spherocytosis biochemical subsets and the selected clinical phenotype. METHODS: We evaluated the clinical and laboratory characteristics of 14 normal healthy persons and 23 hereditary spherocytosis patients and 8 their family members. The patients were divided into three groups based on clinical and hematological severity(mild, typical, severe). In addition to routine hematologic determlnatlons, osmotic fragility and autohemolysis, RBC membrane protein analysis were performed in all patients by densitometric tracing of SDS-PAGE(sodium dodecyl sulphate polyacrylamide gel electrophoresis) stained by Coomassle blue utilizing both the discontinuous buffer system of Laemmli with acrylamide linear gradient from 4% to 12% and the continuous buffer system of Fairbank with exponential gradient of acrylamide from 3.5% to 17%. RESULTS: 1) The patients could be seperated into three classes of different clinical severity as mild(3 cases), moderate(16 cases) and severe(4 cases) on the clinical feature. 2) Eighteen patients(82.6%) among 23 hereditary spherocytosis revealed abnormal erythrocyte membrane protein and we detected six patients(26.1%) with spectrin deficiency combined with ankyrin reduction, 4 patients(17.4%) with ankyrin deficiency, 4 patients(17.4%) with isolated spectrin deficiency and 3 patients(13.0%) with band 3 deficiency. Five HS patients(21.7%) showed normal RBC membrane protein. 3) Eight HS and their family members showed same RBC membrane protein deficiency. 4) The type and degree of RBC membrane protein reduction were variale with spectrin at 66~94%, with ankyrin at 48~82% of normal levels. These showed that each patient had different clinical severities according to different RBC membrane protein levels and type. CONCLUSION: RBC membrane protein abnormalities were observed in 82.6% of HS patients. The combined spectrin and ankyrin deficiency is the most common molecular defect in HS. The clinical severity and biochemical expression is heterogeneous. SDS-PAGE analysis of RBC membrane protein was provided the diagnosis of RBC membrane defects and basic molecular studies. We believed that the early identification of the biochemical defect responsible for HS is important because it is helpful starling point for the identification of the primary molecular defect, and it could help to anticipate the clinical outcome of the disease. For these reasons, we consider the SDS-PAGE of the red cell membrane to be of crucial importance for a complete evaluation of children with HS. Further studies with more cases would be to clarify the correlation between clinical and biochemical phenotypes.
Acrylamide ; Ankyrins ; Cell Membrane ; Child ; Diagnosis ; Electrophoresis, Polyacrylamide Gel* ; Erythrocyte Membrane* ; Erythrocytes* ; Erythrocytes, Abnormal ; Humans ; Membrane Proteins ; Membranes ; Osmotic Fragility ; Phenotype ; Population Characteristics* ; Spectrin ; Starlings

BACKGROUND: Midkine (MK) is a member of the heparin-binding growth factor family. Overexpression of MK is observed not only in cancerous tissue but also in precancerous lesions of the colon and the prostate. Using immunohistochemical methods, we investigated MK expression in preinvasive and invasive neoplasia of the uterine cervix. METHODS: We performed immunohistochemical analysis of archived cone biopsy and hysterectomy specimens from 161 squamous cell lesions of the uterine cervix (29 cervical intraepithelial neoplasia 1 (CIN1), 35 CIN2, 49 CIN3, 30 microinvasive squamous cell carcinomas (MIC), and 18 invasive squamous cell carcinoma). In addition, we examined if there is a correlation between MK expression and status of human papilloma virus infection determined by a commercially available DNA chip. RESULTS: None of the normal cervical mucosa showed MK immunostaining. The level of MK expression gradually increased according to the histologic grade. Moderate and strong expressions were most frequently observed in cervical tissue with CIN3 and MIC. MK immunostaining was more accentuated in the invasive border of MIC. CONCLUSION: MK may play a functional role in the disease progression of cervical squamous cell neoplasia.
Biopsy ; Carcinoma, Squamous Cell ; Cervical Intraepithelial Neoplasia ; Cervix Uteri* ; Colon ; Disease Progression ; Female ; Humans ; Hysterectomy ; Immunohistochemistry ; Mucous Membrane ; Oligonucleotide Array Sequence Analysis ; Papilloma ; Prostate

PURPOSE: This study was designed to investigate the fitness of adjustable dental impression trays on the Chinese and the Japanese. MATERIAL AND METHODS: Initial design of the adjustable dental trays was developed from the results of the dental arch size of Korean adults. This design was applied to the CAD-CAM process in order to create tray model samples. Simple silicon-base molds were then replicated based on these sample models. Polyurethane injection into the silicon-base molds completed the process of creating a large number of test products. 60 Chinese dental students (male:30, female:30) from the Shanghai Second Medical University and 60 Japanese alumni from the Kumamoto high school (male:30, female:30) were selected for taking irreversible hydrocolloid impression with these trays. The width and length of the impression body were measured on several measuring points by Vernier caliper. The results were analyzed statistically to evaluate the fitness of the trays. RESULTS: 1. Uniform impression material thickness was achieved on the Chinese and Japanese by controlling the width of the tray using stops and beveled guides. The material thickness was generally within the range of 3 mm to 6 mm. 2. In the maxillary tray of the Chinese, average thickness of the impression material of the labial vestibule of the incisal teeth was 6.2 mm, the canine was 5.9 mm and the midpalatal part 10.5 mm and the posterior palatal part 9.7 mm. These were relatively large values. 3. In the mandibular tray of the Chinese, average length of the impression material of the lingual vestibule of first, second premolar contact point was 8.9 mm, the incisal teeth was 7.8 mm and thickness of the labial part of canine was 6.8 mm and premolars 7.0 mm. These were relatively large values. 4. In the maxillary tray of the Japanese, average thickness of the impression material of the labial vestibule of the incisal teeth was 7.4 mm, the canine was 7.7 mm and the midpalatal part 9.1 mm. These were relatively large values. 5. In the mandibular tray of the Japanese, average thickness of the impression material of the labial vestibule of first, second premolar contact point was 8.4 mm, and thickness of the labial part of canine was 7.4 mm. These were relatively large values. CONCLUSION: This adjustable dental tray shows good accuracy to Korean because it was designed by the analysis of the dental arch size of Korean adult model. With this result, it can be applied to Chinese and Japanese, we can take more easy and accurate dental impressions.
Adult ; Asian Continental Ancestry Group ; Bicuspid ; Colloids ; Computer-Aided Design ; Dental Arch ; Fungi ; Humans ; Polyurethanes ; Students, Dental ; Tooth

BACKGROUND: Heavily transfused patients commonly become refractory to platelet transfusion. Patients with platelet refractoriness due to HLA alloimmunization need HLA-matched platelet transfusion. We have established an hospital-based donor registry of HLA-typed platelet donors for the first time in Korea and evaluated the possibility of HLA-matched platelet supplies. METHODS: A total of 450 donors were registered and typed for HLA class I (A, B, C) antigens. A computer program was developed and used for the donor registry and HLA-matched donor search. A simulation study was performed on the availability of HLA-matched platelets for 100 patients from a pool of 450 donors. The availability of HLA-matched and cross reactive epitope group (CREG)-matched donors were analysed for HLA-A, B antigens. The CREGs defined by Fuller, Rodey, and UCLA criteria were used. RESLUTS: Among 100 patients, 63% had HLA-identical or HLA-matched (match grade: A, B1U, B2U) donors with only a low number of donors (mean 1.4) available per patient. Including CREG-matches (match grade: B1X, B2UX, B2X), majority (98%) of the patients had HLA- or CREG-matched (match grade: A~B2X) platelet donors with a higher number of donors (mean 26.6~40.5 by different CREG criteria) available per patient. Majority (86-98%) of the patients had 20 or more A~B4X matched donors, and about two thirds (61-74%) of the patients had 20 or more A~B2X matched donors. However, the number of ABO-identical matched donors was less than 30% of the total HLA- or CREG-matched donors. CONCLUSION: We established an HLA-matched platelet donor registry and using a donor pool size of < 500 donors, attainable in an hospital-based donor registry, the possibility of HLA-matched platelet supplies was confirmed in this study. However, for more satisfactory and ABO-matched platelet supplies a larger pool size is needed.
Blood Platelets* ; Equipment and Supplies ; HLA-A Antigens ; Humans ; Korea ; Platelet Transfusion ; Tissue Donors*

BACKGROUND: The testes are one of the most common extramedullary sites of relapse in boys with acute lymphoblastic leukemia(ALL). The reported incidence of isolated testicular relapse varies from 3 to 40%. If these patients are treated exclusively with testicular irradialion, a systemic relapse occurs within a few months. Recently, the use of intensive chemotherapy and testicular irradiation improved the survival rate for boys with testicular leukemia. So, we performed this study to identify clinical manifestations, disease free survival and prognostic factors of testicular leukemia in children. METHODS: We reviewed 33 patients of testicular leukemia among total 410 boys with ALL diagnosed at the Department of Pediatrics, Seoul National University Children's Hospital from Jan. 1970 to Aug. 1996. Testicular leukemia was confirmed by testicular biopsy in all 33 patients. These patients were treated with combined local testicular irradiation(2,400~2,500 cGy/8~12fractions) and systemic chemotherapy. Two patients, in whom testicular relapse was diagnosed before 1979, unilateral orchiectomy of the involved site and testicular irradiation of the opposite site were performed. Probability estimates of disease free survival (DFS) were calculated by the method of Kaplan and Meier, and the relationship of prognostic factors to DFS was compared using the chi-square test in survival analysis. RESULTS: In 410 boys with ALL, testicular leukemia occurred in 33 patients(8%). Of 33 patients, 6 patients presented with testicular involvement at initial diagnosis, 16 patients had testicular relapse while still receiving chemotherapy and 11 patients had testicular relapse 3 to 57 months(median : 15 months) after cessation of chemotherapy. The median age of 33 patients was 7.4 yrs(9 months~18 yrs) and median WBC count 7,600/ L(2,700~270,000/L). All patients presented with painless testicular enlargement and testicular leukemia was confirmed by testicular biopsy. Among 33 patients, 2 had prior CNS relapse and 11 had concomitant bone marrow and/or CNS relapse. Twenty nine patients were treated with combined local testicular irradiation and systemic chemotherapy. Eleven had second relapse(6 bone marrow, 3 CNS, 2 opposite testis). Seventeen have been followed until now: 6 patients on chemotherapy and 11 patients(37.9%) in complete remission for 48.5+/-22.3 months(19~86 months). The 3 year DFS for 29 patients was 55.3%+/-10.1%. The following prognostic factors showed no significant association with DFS in testicular relapse : age and WBC count at initial diagnosis, age at testicular relapse, and concomitant relapse. Whether testicular relapse occurred on initial therapy or off initial therapy has prognostic value in predicting DFS. The 3 year DFS for boys with testicular relapse on and off initial therapy were 40.0%+/-12.9% and 78.8%+/-13.4%, respectively(P: 0.046). CONCLUSION: With the use of chemotherapy and testicular irradiation, prolonged second re mission can be achieved in many patients with testicular leukemia. The patients with testicular relapse off initial therapy fared significantly better than patients on therapy. So, to improve the DFS for boys with testicular leukemia, a better understanding of its biology and prognostic factors is needed.
Biology ; Biopsy ; Bone Marrow ; Child ; Diagnosis ; Disease-Free Survival ; Drug Therapy ; Humans ; Incidence ; Leukemia ; Missions and Missionaries ; Orchiectomy ; Pediatrics ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Recurrence ; Seoul ; Survival Rate ; Testis

A 13 year-old girl with osteosarcoma and pulmonary tumor recurrence developed acute renal failure following high dose methotrexate (12 g/m2) therapy, she had previously tolerated high dose methotrexate and her renal and hepatic functions were normal. Briefly, 48 hours after beginning methotrexate infusion her methotrexate concentration and creatinine level were 1338.8microM/L and 5.8 mg/dl, respectively. Grade IV oral mucositis and neutropenia with fever developed at 144 hours after MTX infusion. Hydration and alkalinization were continued and leucovorin rescue was intensified based on the plasma MTX concentrations. Plasma exchange was performed twice and hemodialysis 3 times without problems, but methotraxate and creatinine levels remained high, 91.9 microM/L, and 2.5 mg/dl, respectively. After 3 courses of hemodialysis carboxypeptidase-G2 (CPDG2) was administered at 50 U/kg, intravenously over 5 minutes. After 15 minutes of CPDG2 (Voraxaze(TM)) infusion, her plasma MTX concentration was 0.91microM/L and no rebound elevation or side effects developed. Thirteen days post-MTX infusion her renal function had normalized. We report here our experience of a dramatic methotrexate level reduction caused by CPDG2 administration.
Acute Kidney Injury ; Adolescent ; Creatinine ; Female ; Fever ; Humans ; Leucovorin ; Methotrexate ; Neutropenia ; Osteosarcoma ; Plasma ; Plasma Exchange ; Recurrence ; Renal Dialysis ; Stomatitis

PURPOSE: Cepharanthin (R) has been reported to improve the symptoms of intractable or steroid-resistant chronic idiopathic thrombocytopenic purpura (ITP). We report the clinical efficacy of oral high dose Cepharanthin (R) in refractory chronic ITP. METHODS: We analyzed eleven patients who were diagnosed as chronic ITP at the Department of Pediatrics, Seoul National University Hospital from January, 2002 to February, 2004. After informed consent patients with platelet count below 20, 000/mm3 were treated with Cepharanthin (R) at the initial dose of 5 gm/day. The doses were increased up to 15 gm/day if there was no response. For the purposes of this study, complete remission was defined as an elevation of the platelet count > or =50, 000/mm3 from the baseline after 4 weeks of treatment. Partial remission was defined as an elevation of the platelet count 20, 000/mm3 and < = or50, 000/mm3 from the baseline. RESULTS: Seven boys and four girls with a median age of 10 were enrolled. Two to four weeks after the initiation of this therapy, 4 patients showed their platelet counts over 50, 000/mm3 1 patients reached partial remission state, 6 patients had no response. Side effects of Cepharanthin (R) were not observed in all patients. CONCLUSION: We suggest that the oral administration of Cepharanthin (R) could be a beneficial and a safe treatment strategy for the refractory chronic ITP patients. Further studies should be performed to elucidate the mechanism of responses.
Administration, Oral ; Child* ; Female ; Humans ; Informed Consent ; Pediatrics ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic* ; Seoul

Ibandronate (a bisphosphonate) is commonly used as an treatment of osteoporosis in combination with vitamin D. Monthly DP-R206-a novel, fixed-dose combination tablet (150 mg ibandronate/24,000 IU vitamin D3)-was recently developed to enhance patient compliance. This open, randomized, two-period crossover study was conducted to compare the pharmacokinetics of ibandronate when administered as DP-R206 or 150 mg ibandronate to healthy adult volunteers. Each volunteer was randomly allocated to receive single-dose DP-R206 or ibandronate with a 28-day washout period between treatments. Blood samples were assessed using pharmacokinetic analysis. Plasma ibandronate concentrations were determined using liquid chromatography-tandem mass spectrometry. Safety and tolerability assessments were performed throughout the study. In total, 103 participants received the study drugs and 72 participants completed the study. The geometric mean ratios (DP-R206/ibandronate) of the maximum concentration (C(max)) and the area under the plasma concentration time curve from time zero to the last concentration (AUC(last)) values were 0.959 (90% CI: 0.820-1.120) and 0.924 (90% CI: 0.805-1.060), respectively. The frequencies of adverse events (AEs) and drug reactions were similar between treatment groups, and all AEs were recovered without sequalae. Ibandronate pharmacokinetics, tolerability, and safety are comparable when administered to healthy individuals, regardless if administered as DP-R206 or ibandronate.
Adult* ; Cross-Over Studies ; Humans ; Mass Spectrometry ; Osteoporosis ; Patient Compliance ; Pharmacokinetics ; Plasma ; Vitamin D ; Vitamins* ; Volunteers

OBJECTIVES: Routine vaccination against influenza is recommended for all people aged 65 years or more. Without active encouragement to receive the vaccine, the rate of compliance is generally low. A study was undertaken to assess and compare the effectiveness of two reminder systems in improving the influenza vaccination rate. METHODS: A total of 2,017 patients aged 65 or over in a tertiary care hospital family practice center were randomly assigned to a control, a telephone reminder, or a postcard reminder group before the influenza season in 1998. The outcome measure, receipt of vaccination, was determined by telephone interview. Demographic and clinical characteristics were checked through the medical chart review. RESULTS: Among the 1,312 patients who actually received reminders and interviewed, the vaccination rate was 46.7% in the control group, 56.3% in the postcard reminder group, and 63.3% in the telephone reminder group. Vaccination compliance was significantly higher in people reminded by telephone (Odds ratio [OR], 2.00; 95% confidence interval [CI], 1.52-2.64) and postcard (OR, 1.55; 95% CI, 1.18-2.02) compared to that in the control group. Of the characteristics investigated, number of high risk co-morbidity was positively associated with vaccination compliance while current smoking was negatively associated. CONCLUSIONS: This result suggests that telephone and postcard reminders can significantly improve compliance with influenza vaccination in this group of Korean elderly. However, additional strategies need to be developed to encourage vaccination among noncompliant.
Aged* ; Compliance ; Family Practice* ; Humans ; Influenza, Human* ; Interviews as Topic ; Outcome Assessment (Health Care) ; Reminder Systems ; Seasons ; Smoke ; Smoking ; Telephone* ; Tertiary Healthcare* ; Vaccination*