Cleidocranial dysplasia is a well-documented rare autosomal dominant skeletal dysplasia characterized by hypoplastic/aplastic clavicles, brachycephalic skull, patent sutures and fontanelles, midface hypoplasia, and abnormalities of dentition. Patients with cleidocranial dysplasia often complain about undesirable esthetic appearance of their forehead and skull. Notwithstanding many studies of molecular, genetics and skeletal abnormalities of this congenial disorder, there have been very few written reports of cranioplasty involving cleidocranial dysplasia. Thus, we report a rare case of successful cranioplasty using a modified split calvarial graft technique in patient with cleidocranial dysplasia.
Clavicle ; Cleidocranial Dysplasia* ; Dentition ; Forehead ; Genetics ; Humans ; Skull ; Sutures ; Transplants*

OBJECTIVE: The risk of complications is high for patients with a large cranial defect and hydrocephalus, undergoing cranioplasty and ventriculoperitoneal (VP) shunt operation. The purpose of this study is to examine retrospectively such cases with complications and contrive an operative technique to reduce complications. METHODS: Nineteen patients underwent cranioplasty and VP shunt operation due to large cranial defects and hydrocephalus. These patients were divided into two groups: Group A with 10 patients who underwent staged-operations, and Group B with 9 patients who underwent one-stage operation. Their complications in each group were retrospectively reviewed. Another five patients underwent a one-stage operation with temporary occlusion of the distal shunt catheter to improve on the technique and were categorized as Group C. Complications in these groups were compared and analyzed. RESULTS: The results of the data analysis revealed that complications related to anesthesia (40%) and those related to antibiotic prophylaxis (30%) were high in Group A, while non-infectious delayed complications (45%) and perioperative complications such as intracranial hematoma (33%) were high in Group B. However, for patients in Group C, it showed less complication with the operative technique devised by these authors, as opposed to two previous procedures. CONCLUSION: In patients with hydrocephalus and a large cranial defect, complications arising from existing one-stage operation or staged-operations can be reduced by implementing the technique of "one-stage operation with temporary occlusion of the distal shunt catheter."
Anesthesia ; Antibiotic Prophylaxis ; Catheters ; Decompressive Craniectomy ; Hematoma ; Humans ; Hydrocephalus* ; Retrospective Studies ; Statistics as Topic ; Ventriculoperitoneal Shunt*

PURPOSE: To determine if stem cells transplanted directly into a bone defect of rabbit tibias have osteogenic induction potential. MATERIALS AND METHODS: Immature white New Zealand rabbits underwent tibial osteotomies, and were divided into three groups according to the implant material used: stem cells embedded in agar (group 1); agar alone (group 2); nothing (group 3). For all rabbits, radiographs were taken weekly for 8 weeks, and histological studies of the newly formed-bone were performed. CM-Dil was used to label the stem cells prior to transplantation to ascertain whether or not the newly formed bone was derived from the transplanted stem cells. RESULTS: Fibroblasts and osteoblasts (osteoid matrix-forming cells) derived from the stem cells were identified by electron microscopy. Interspersed enchondral ossification (probably induced by osteogenic cells from the remaining periosteum and marrow) and pure osteoids (created directly from the osteoblasts originating from the transplanted stem cells) were identified. Fluorescent-labeled cells were conspicuous in the new bones until 6 weeks after surgery, which indicates that the new bones were induced by the stem cells. CONCLUSION: The osteogenic induction potential of the undifferentiated stem cell has promise for therapeutic application, which may be used for the treatment of bone defects in the future.
Agar ; Fibroblasts ; Microscopy, Electron ; Osteoblasts ; Osteotomy ; Periosteum ; Rabbits ; Stem Cells* ; Tibia

Antiphospholipid syndrome is a thrombotic disorder characterized by the association of arterial and venous thrombosis with the antibodies directed toward phospholipids. The presence of these antibodies in systemic lupus erythematosus(SLE) has been shown to be related to several clinical and analytical alterations. We experienced one case of lupus nephritis with positive antiphospholipid antibodies in a 10-year-old girl whose chief complaint was persistent microscopic hematuria. We report this case with a brief review of related literatures.
Antibodies ; Antibodies, Antiphospholipid* ; Antiphospholipid Syndrome ; Child ; Female ; Hematuria ; Humans ; Lupus Erythematosus, Systemic ; Lupus Nephritis* ; Mass Screening* ; Phospholipids ; Venous Thrombosis

Background: Thioacetamide is a classic hepatotoxic reagent which leads to the reproducible hepatic fibrosis in rats. Thioacetamide-induced fibrosis is an appropriate model for cirrhosis in humans due to the long duration of course and similiar histology. Thioacetamide produces hepatotoxicity through lipid peroxidation but it is unclear whether lipid peroxidation directly correlated with hepatic fibrosis. Pentoxifylline, a derivative of the methylxanthine, showed an antifibrogenic effect in cell cultures of human fibroblasts and some animal models. But this antifibrogenic effect is controversial. Pentoxifylline revealed a hepatoprotective effect in some toxic hepatitis. This hepatoprotective effect seems to influence cell cycle regulatory protein during regeneration. This study aimed to evaluate an effect of pentoxifylline on fibrosis and cell cycle regulatory protein during liver regeneration in thioacetamide-induced rat cirrhosis. Lipid peroxidation assay was compared with collagen content so as to evaluate the correlation with fibrosis. METHOD: Liver cirrhosis was induced by 0.03% oral administration of thioacetamide. Pentoxifylline was administered simultaneously with thioacetamide. The semiquantitative fibrosis index was measured based on histologic finding. Collagen content was estimated by spectrophotometric assay. Activated hepatic stellate cells were counted using alpha-SMA immunohistochemistry. Malondialdehyde, lipid peroxidation metabolite, was estimated by thiobarbituric acid reactive substance assay. Cell cycle regulatory protein was evaluated by western blot. RESULTS: There was no difference in semiquantitative fibrosis index, collagen content and hepatic stellate cell count between thioacetamide treated rats and simultaneous pentoxifylline treated rats. Lipid peroxidation product was not correlated with collagen content. Western blot showed no difference in cell cycle regulatory protein. CONCLUSION: Pentoxifylline does not show an antifibrogenic effect in thioacetamide-induced rat cirrhosis, in which thioacetamide induced hepatocellular damage and fibrosis. Lipid peroxidation may be a secondary effect rather than primary mediating mechanism in hepatic fibrosis.
Administration, Oral ; Animals ; Blotting, Western ; Cell Culture Techniques ; Cell Cycle* ; Collagen ; Drug-Induced Liver Injury ; Fibroblasts ; Fibrosis* ; Hepatic Stellate Cells ; Humans ; Immunohistochemistry ; Lipid Peroxidation ; Liver Cirrhosis* ; Liver Regeneration ; Liver* ; Malondialdehyde ; Models, Animal ; Negotiating ; Pentoxifylline* ; Rats* ; Regeneration ; Thioacetamide

OBJECTIVE: Angiogenesis is an essential step in growth and metastasis of solid tumors. Vascular endothelial growth factor(VEGF) is one of the most important mediators of angiogenesis. VEGF selectively stimulates endothelial cell proliferation and induces angiogenesis. Also VEGF was expressed by several human solid tumors and serum VEGF levels have previously been shown to be raised in patients with breast cancer, gastrointestinal cancer, renal cancer and melanoma. To evaluate the clinical value of plasma VEGF of patients with stomach cancer we studied correlations between plasma VEGF, tumor stage, tumor resection and microvessel invasion by tumor. METHODS: VEGF level was measured by ELISA methods in plasmas from 88 patients and after surgical tumor resection from 48 patients with gastric carcinoma. Microvessel staining was done by immunohistochemical staining using anti-CD34 monoclonal antibody on paraffin embedded tissues. RESULTS: The plasma VEGF levels were significantly higher in the stomach cancer patients than in normal controls(p<0.0001). In stomach cancer patients, plasma VEGF level was significantly increased according to stage progression(p<0.05). Moreover early cancer, T1, showed a significantly elevation of plasma VEGF level than that of controls. The level of plasma VEGF fell after surgical resection(n=48) of stomach cancer(p<0.05). Also the plasma VEGF levels were significantly higher in microvessel invasion by tumor(n=25) than in those without invasion(n=19)(p<0.05). CONCLUSION: In conclusion, plasma VEGF may be useful for predicting disease status and prognosis of patients with stomach cancer.
Breast Neoplasms ; Endothelial Cells ; Enzyme-Linked Immunosorbent Assay ; Gastrointestinal Neoplasms ; Humans ; Kidney Neoplasms ; Melanoma ; Microvessels ; Neoplasm Metastasis ; Paraffin ; Plasma* ; Prognosis ; Stomach Neoplasms* ; Stomach* ; Vascular Endothelial Growth Factor A*

PURPOSE: Multiparametric flow cytometry is a powerful tool for analyzing the phenotypic, cell kinetic and ploidy heterogeneity of tumor cell populations. But there are major problems such as inaccurate results by the contribution of non-neoplastic cell contamination and the substantial spectral overlap of PI (propidium iodide) into PE (phycoery- thrin) fluorescent emissions on a standard flow cytometer. Recent studies suggested that the emission spectral overlap from PI into PE could be sufficiently compensated electrically and the cytokeratin, a marker for epithelial tumor cells, are successfully used in conjunction with DNA specific dye so as to obtain DNA profiles selectively for cytokeratin-positive tumor cells. The aim of this study was to investigate the feasibility that multiparametric analysis in heterogeneous cell populations of cell lines like solid tumors, which were stained triply with PE, fluorescein isothiocyanate FITC, and PI, can be done without any influences by the contaminated normal diploid cell populations and without spectral overlap between fluorochromes on a standard flow cytometer. MATERIALS AND METHODS: MCF-7 cell lines and heterogeneous cell populations mixed with MCF-7 cells and human peripheral blood lymphocytes were fixed with 1% paraformal- dehyde and permeabilized with 100% methanol. Cytokeratin was labeled with PE and some proliferat!on-associated markers were labeled with FITC, which were followed by DNA staining by PI. These triply stained cells were measured on a standard FACScan flow cytometer equipped with 488 nm single laser and those acquired data were analyzed with WinList 3.0 and ModFit LT software programs on personal computor. RESULTS: Coefficient of variation (CV) of GoG1> peak of MCF-7 cells alone was 4.3. GoG1, S, and G2M phase fractions were 44.9%, 45.9%, and 9.2% respectively. FITC, PE and PI fluorochromes could be detected without any interference between them. CVs of GoG1 peak of PBL and MCF-7 cells in those heterogeneous population were 2.3 and 4.2 respectively. The DNA index of MCF-7 cells was 1.7. MCF-7 cells expressed the cyto- keratin, PCNA, p53, c-erbB/2 and c-myc antigen and in contrast, PBL did not express cytokeratin. The cell cycle phase fractions and oncoprotein expressions could be detected separately in diploid PBL and aneuploid MCF-7 cells in the mixed cell population without any influences by each other. CONCLUSION: These results suggested that the cellular antigen expressions of the malignant cells can be analyzed selectively without influences of fluorescent signals from nonneo- plastic cells. The neoplastic tumor subpopulations are clearly identified on the basis of both ploidy status and antigen expressions. The positive cytokeratin expressions indicate that they were derived from the epithelium, providing objective evidence of the tissue of origin and more precise analysis of DNA contents, ploidy, and oncogene expressions selectively with possible correlation between them. Thus, this method offers new possibilities for multiparameter flow cytometric analysis in the heterogeneous solid tumor cell populations.
Aneuploidy ; Breast Neoplasms* ; Breast* ; Cell Cycle ; Cell Line* ; Diploidy ; DNA ; Epithelium ; Flow Cytometry* ; Fluorescein* ; Fluorescein-5-isothiocyanate ; Fluorescent Dyes ; Humans ; Keratins ; Lymphocytes ; MCF-7 Cells ; Methanol ; Oncogenes ; Phycoerythrin* ; Plastics ; Ploidies ; Population Characteristics ; Proliferating Cell Nuclear Antigen ; Propidium*

The incidence of malignant change of ovarian mature teratoma is 1~2%. The majority is squamous cell cancer, the others was adenocarcinoma. Neuroepithelial tissue was frequently detected in mature cystic teratoma, but their malignant change was extremely rare. Only, two cases of neuroblastoma of ovarian teratoma were reported in the world. We report one case of neuroblastoma arising in ovarian mature teratoma with a brief review. Our case is the third reported one in the world.
Adenocarcinoma ; Female ; Incidence ; Neoplasms, Squamous Cell ; Neuroblastoma* ; Ovary* ; Teratoma*

We compared the therapeutic effects of concomitant chemoradiotherapy (CRT) using cisplatin to single radiotherapy (RT) in uterine cervical cancer. 34 cases of non-operable uterine cervical cancer were reviewed retrospectively from Mar, 1993 to May, 1996 in St. Mary' s Hospital. The patients were randomly selected to compare the effects of both methods. 22 patients were included in CRT group and 12 patients in RT group. The results were as follows: 1. The decrease of tumor size was not significant (2.17 cm in CRT and 1.95 cm in RT) (p=0.61), but the number of responders of CRT group was larger than that of RT group significantly (p<0.05). 2. The tumor markers showed no significant difference between CRT and RT groups (p>0.05) 3. The overall survival rate showed no difference between two groups (p>0.05). The disease-free survivals for 38 months were 17.02% in CRT and 11.36% in RT, but it was not significant (p>0.05). In conclusion, concomitant chemoradiotherapy showed better rate of response, but size of tumor decrease and tumor markers showed no difference. CRT might improve the overall survival and disease-free survival, although it was not significant in this study. The clinical significance of CRT remains to be determined in large randomized clinical trial.
Chemoradiotherapy ; Cisplatin ; Disease-Free Survival ; Humans ; Radiotherapy* ; Retrospective Studies ; Survival Rate ; Biomarkers, Tumor ; Uterine Cervical Neoplasms*

BACKGROUNDS: Living-donor liver transplantation (LDLT) has been established as an efficacious option to resolve the shortage of cadaveric donor organs for pediatric recipients. This surgical innovation has significantly reduced the pretransplantation mortality for children, but the crisis of increasing scarcity of donor organs in our hospital has led us to extend LDLT to adult recipients. However, the extension of LDLT from pediatric recipients to adult recipients has been made only with limited success largely because of the inability of a relatively small-size left-lobe graft to meet the metabolic demands of an adult recipient. It has been postulated that a left-lobe graft smaller than 40% of the recipient's standard liver volume will not result in a successful adult-to-adult LDLT in chronic parenchymal liver disease. METHODS: From February 1997 to October 1997, 10 LDLTs, using 9 extended left-lobe grafts and 1 right-lobe graft, were performed on patients with end-stage parenchymal liver diseases (9 cases of B-hepatitis-induced cirrhosis with or without an associated hepatocellular carcinoma and 1 case of alcoholic cirrhosis) at the Department of Surgery, Asan Medical Center. The ratios of the graft to the standard liver volume of the recipients were in the range of 30% to 55%. RESULTS: All grafts showed immediate function, but delayed normalization of the serum total bilirubin was demonstrated in all recipients receiving left-lobe grafts. There were no mortalities and serious complications in donors. Two recipients died of sepsis 21 days and 40 days after transplantation, and 8 recipients (80%) are alive with good liver function at a median follow-up of 5.1 months (range 2~10 months). CONCLUSIONS: The aim of this article is to report our experience with adult-to-adult LDLT shows that a graft size greater than 30% of the recipient's standard liver volume is able to meet the metabolic demands of adult recipients with chronic parenchymal liver disease and that LDLT might open a new donor pool for adult recipients when the supply of cadaveric organs is severely restricted.
Adult ; Alcoholics ; Bilirubin ; Cadaver ; Carcinoma, Hepatocellular ; Child ; Chungcheongnam-do ; Fibrosis ; Follow-Up Studies ; Humans ; Liver Diseases ; Liver Transplantation* ; Liver* ; Living Donors* ; Mortality ; Sepsis ; Tissue Donors ; Transplants