Dermatofibrosarcoma protuberans (DFSP) is a rare, distinctive cutaneous tumor, which consists of spindle shaped ceils arranged in densely packed interlacing bundles with the storiform or cartwheel pattern. Histologically, it resembles deep growing dermatofibroma, nodular fasciitis, neurofibroma and neural sheath tumors. DFSP is one of t.he connective tissue tumors which is difficult. to diagnose histologically as well as clinically. Recently, the immunochemical staining with a monoclonal antibody to CD34 is reported to give assistance in the clear differential diagnosis of DFSP from other fibrous or neural tumors. Herein, three cases of DFSP were stained by immunohistochemical staining with S-100 protein, vimentin, factor VIII and anti-CD34 antibody in order to assess the use of anti-CD34 in the differential diagnosis of DFSP.
Connective Tissue ; Dermatofibrosarcoma* ; Diagnosis, Differential ; Factor VIII ; Fasciitis ; Histiocytoma, Benign Fibrous ; Neurofibroma ; S100 Proteins ; Vimentin

In order to identify the correlations between clinical results and quantitative data of gait analysis, we analyzed the results of 20 cases of total knee joint replacement arthroplasty in 15 patients with degenerative arthritis. We also evaluated the gait analysis of ten age-matched healthy candidates as a control group. Mean follow-up periods were 30 months. Clinical results included post-operative HSS (Hospital for Special Surgery) knee rating scores and the changes of the tibiofemoral angles. The three dimensional gait analysis included clinical assessment, video-taping, three dimensional kinematics and kinetics. The three dimensional kinematics were obtained using a 5 camera VICON system, and the three dimensional kinetic data was collected using two AMTI force plates. There was no statistical difference in linear parameters between the patient and control group. In patients group, however, double support time decreased as the HSS score increased, and range of knee motion and maximum knee flexion increased in accordance with the increase of pain score. Kinematic data of the patients group revealed that some parameters, such as knee flexion during loading response, knee flexion in swing phase, and knee varus during swing phase, were decreased. On the other hand, internal rotation of the knee from initial contact to initial swing was increased when compared with that of control group. There was no significant correlation between the degrees of tibiofemoral angle and coronal plane moment in the patients group. In three cases which showed mild varus instability post-operatively, knee flexion during loading response decreased and valgus moment in midstance increased as compared with the cases without instability. We believe that three dimensional gait analysis will be a good modality for evaluation of the results after total knee arthroplasty. With further accumulation of long term. follow-up data of gait analysis, we might be able to predict the long term results of total knee arthroplasty including possibility of loosening.
Arthroplasty* ; Arthroplasty, Replacement ; Biomechanical Phenomena ; Follow-Up Studies ; Gait* ; Hand ; Humans ; Kinetics ; Knee Joint ; Knee* ; Osteoarthritis

BACKGROUND: Hematopoietic stem cells of the human fetal liver prior to 15 weeks gestation have remakable advantages for successful engraftment due to embryological immune immaturity, especially in-utero transplantation. This study was undertaken to obtain objective assessment data about the possibility of fetal liver hematopoietic stem cell transplantation in the future. METHODS: Six cases of the fetal liver tissue were obtained from therapeutic abortions at 12~20 weeks gestation. The fetal liver was collected in RPMI media containing 10% fetal calf serum and the cell suspensions were obtained by centrifugation following physical disruption. The number of nucleated cells in each case was counted and the colony numbers in methyl cellulose media were scored according to incubation period with or without growth factors. Some of the cells were cryopreserved in the liquid nitrogen tank, thereafter cell viability and colony numbers were evaluated according to cryopreservation period. RESULTS: The nucleated cell numbers obtained from each fetal liver increased with gestational age. The colony numbers after incubation increased with gestational age and the erythroid lineage was predominant in 3 cases which are under 15 weeks gestation. The colonogenic activity after incubation with combination of hematopoietic growth factors increased in only one case. The cell viability and the colony numbers after cryopreservation was decreased compare to the value before cryopreservation. CONCLUSION: The number of nucleated cells and hematopoietic stem cell colony formation were increased with gestational age and viability of the cells after cryopreservation was decreased. Further systematic studies using more cases would be needed to obtain objective assessment data for fetal liver transplantation program in the future.
Abortion, Therapeutic ; Cell Count ; Cell Survival ; Centrifugation ; Cryopreservation ; Female ; Gestational Age ; Hematopoietic Stem Cell Transplantation ; Hematopoietic Stem Cells* ; Humans ; Intercellular Signaling Peptides and Proteins ; Liver Transplantation ; Liver* ; Methylcellulose ; Nitrogen ; Pregnancy ; Suspensions

Hemangioma is one of the most common congenital tumors in the region of the face and neck. Although histologically benign, these facial masses are clinically malignant for their deforming and inexorable growth, especially in so-called cavernous hemangioma. Today, hemagiomas have been treated by various methods; steroids, electrocoagulation, injection of sclerosing agent, cryotherapy, radiation therapy, laser therapy, and surgical treatment, etc. The response of treatment varies with the types, surgical skills, regions of hemangioma, the age of patient, but the appropriate method of the treatment has not been yet established. In the case of massive hemangioma, which involves most part of the face and neck, we prefer surgical treatment and consistently obtain better result than other treatment modalities. Wide excision and proper reconstruction of the face helps the patient for their better life aesthetically and functionally.
Cryotherapy ; Electrocoagulation ; Hemangioma* ; Hemangioma, Cavernous ; Humans ; Laser Therapy ; Neck* ; Steroids

In ischemic strokes, apoptosis is caused by excitotoxicity, ionic imbalance, oxidative/nitrosative stress, and apoptotic-like pathways. Nitric oxide (NO), a free radical, is elevated after ischemic insult. NO, which is generated primarily by neuronal nitric oxide synthase (nNOS) and inducible nitric oxide synthase (iNOS), promotes neuronal damage following ischemia. Evidence obtained in recent years has demonstrated that endoplasmic reticulum (ER)-mediated cell death plays an important role in cerebral ischemia. Agmatine is an endogenous substance synthesized from L-arginine by arginine decarboxylase (ADC) and is present in mammalian brain. We had previously reported that agmatine contributes to neuroprotection against ischemic injury. In continuation of our earlier work, we intended to investigate whether agmatine protects brain from transient global ischemia, and also tried to determine the neuroprotective mechanism of agmatine. Twenty minutes of transient global ischemia was induced by 4 vessel occlusion (4-VO). Agmatine (100 mg/kg, IP) was administered simultaneously with reperfusion. Samplings of brain were done at 6, 24, 48, and 72 h after reperfusion to determine the effect of agmatine on ischemic injured hippocampus. ER-damage was also investigated using electron microscope. Results showed that agmatine treatment prevented delayed neuronal cell death in hippocampal CA1 neurons after global cerebral ischemia. It also blocked NOS expression in the rat brain. Agmatine induced the increased expression of glucose-regulated protein 78 (Grp78). These results suggest that agmatine inhibits the production of NO by decreasing the expression of nNOS and iNOS on global forebrain ischemia and the neuroprotective effect of agmatine were concerned with the ER stress-mediated condition.
Agmatine ; Animals ; Apoptosis ; Arginine ; Brain ; Brain Ischemia ; Carboxy-Lyases ; Cell Death ; Electrons ; Endoplasmic Reticulum ; Glycosaminoglycans ; Hippocampus ; Ischemia ; Neurons ; Neuroprotective Agents ; Nitric Oxide ; Nitric Oxide Synthase ; Nitric Oxide Synthase Type I ; Nitric Oxide Synthase Type II ; Prosencephalon ; Rats ; Reperfusion ; Stroke

BACKGROUND: This study was designed to develop a Korean version of the heparin-coated vascular bypass shunt by using a physical dispersing technique. The safety and effectiveness of the thrombo-resistant shunt were tested in experimental animals. MATERIAL AND METHOD: A bypass shunt model was constructed on the descending thoracic aorta of 21 adult mongrel dogs(17.5-25 kg). The animals were divided into groups of no-treatment(CONTROL group; n=3), no-treatment with systemic heparinization(HEPARIN group; n=6), Gott heparin shunt (GOTT group; n=6), or Korean heparin shunt(KIST group; n=6). Parameters observed were complete blood cell counts, coagulation profiles, kidney and liver function(BUN/Cr and AST/ ALT), and surface scanning electron microscope(SSEM) findings. Blood was sampled from the aortic blood distal to the shunt and was compared before the bypass and at 2 hours after the bypass. RESULT: There were no differences between the groups before the bypass. At bypass 2 hours, platelet level increased in the HEPARIN and GOTT groups(p<0.05), but there were no differences between the groups. Changes in other blood cell counts were insignificant between the groups. Activated clotting time, activated partial thromboplastin time, and thrombin time were prolonged in the HEPARIN group(p<0.05) and differences between the groups were significant(p<0.005). Prothrombin time increased in the GOTT group(p<0.05) without having any differences between the groups. Changes in fibrinogen level were insignificant between the groups. Antithrombin III levels were increased in the HEPARIN and KIST groups(p<0.05), and the inter-group differences were also significant(p<0.05). Protein C level decreased in the HEPARIN group(p<0.05) without having any differences between the groups. BUN levels increased in all groups, especially in the HEPARIN and KIST groups(p<0.05), but there were no differences between the groups. Changes of Cr, AST, and ALT levels were insignificant between the groups. SSEM findings revealed severe aggregation of platelets and other cellular elements in the CONTROL group, and the HEPARIN group showed more adherence of the cellular elements than the GOTT or KIST group. CONCLUSION: Above results show that the heparin-coated bypass shunts(either GOTT or KIST) can suppress thrombus formation on the surface without inducing bleeding tendencies, while systemic heparinization(HEPARIN) may not be able to block activation of the coagulation system on the surface in contact with foreign materials but increases the bleeding tendencies. We also conclude that the thrombo-resistant effects of the Korean version of heparin shunt(KIST) are similar to those of the commercialized heparin shunt(GOTT).
Adult ; Animals ; Antithrombin III ; Aorta ; Aorta, Thoracic ; Blood Cell Count ; Blood Platelets ; Fibrinogen ; Hemorrhage ; Heparin ; Humans ; Kidney ; Liver ; Partial Thromboplastin Time ; Protein C ; Prothrombin Time ; Thrombin Time ; Thrombosis

Celiac disease is an intestinal autoimmune disorder, triggered by ingestion of a gluten-containing diet in genetically susceptible individuals. The genetic predisposition is related to human leukocyte antigen (HLA) class II genes, especially HLA-DQ2-positive patients. The prevalence of celiac disease has been estimated to be ~1% in Europe and the USA, but it is rarer and/or underdiagnosed in Asia. We report a case of celiac disease in a predisposed patient, with a HLA-DQ2 heterodimer, and Graves' disease that was treated successfully with a gluten-free diet. A 47-year-old woman complained of persistent chronic diarrhea and weight loss over a 9 month period. Results of all serological tests and stool exams were negative. However, the patient was found to carry the HLA DQ2 heterodimer. Symptoms improved after a gluten-free diet was initiated. The patient has been followed and has suffered no recurrence of symptoms while on the gluten-free diet. An overall diagnosis of celiac disease was made in a genetically predisposed patient (HLA-DQ2 heterodimer) with Graves' disease.
Asia ; Celiac Disease* ; Diagnosis ; Diarrhea ; Diet ; Diet, Gluten-Free ; Eating ; Europe ; Female ; Genes, MHC Class II ; Genetic Predisposition to Disease ; Graves Disease* ; Humans ; Leukocytes ; Middle Aged ; Prevalence ; Recurrence ; Serologic Tests ; Weight Loss

BACKGROUND: Recently, a hemoglobin A1c (HbA1c) level of 6.5% has been determined to be a criterion for diabetes mellitus (DM), and it is a widely used marker for the diagnosis of DM. However, HbA1c may be influenced by a number of factors. Anemia is one of the most prevalent diseases with an influence on HbA1c; however, its effect on HbA1c varies based on the variable pathophysiology of anemia. The aim of this study was to determine the effect of anemia on HbA1c levels. METHODS: Anemic subjects (n=112) and age- and sex-matched controls (n=217) who were drug naive and suspected of having DM were enrolled. The subjects underwent an oral glucose tolerance test and HbA1c simultaneously. We compared mean HbA1c and its sensitivity and specificity for diagnosing DM between each subgroup. RESULTS: Clinical characteristics were found to be similar between each subgroup. Also, when glucose levels were within the normal range, the difference in mean HbA1c was not significant (P=0.580). However, when plasma glucose levels were above the diagnostic cutoff for prediabetes and DM, the mean HbA1c of the anemic subgroup was modestly higher than in the nonanemic group. The specificity of HbA1c for diagnosis of DM was significantly lower in the anemic subgroup (P<0.05). CONCLUSION: These results suggest that the diagnostic significance of HbA1c might be limited in anemic patients.
Anemia ; Blood Glucose ; Diabetes Mellitus* ; Glucose Tolerance Test ; Hemoglobin A, Glycosylated* ; Humans ; Prediabetic State ; Reference Values ; Sensitivity and Specificity

BACKGROUND: Recently, a hemoglobin A1c (HbA1c) level of 6.5% has been determined to be a criterion for diabetes mellitus (DM), and it is a widely used marker for the diagnosis of DM. However, HbA1c may be influenced by a number of factors. Anemia is one of the most prevalent diseases with an influence on HbA1c; however, its effect on HbA1c varies based on the variable pathophysiology of anemia. The aim of this study was to determine the effect of anemia on HbA1c levels. METHODS: Anemic subjects (n=112) and age- and sex-matched controls (n=217) who were drug naive and suspected of having DM were enrolled. The subjects underwent an oral glucose tolerance test and HbA1c simultaneously. We compared mean HbA1c and its sensitivity and specificity for diagnosing DM between each subgroup. RESULTS: Clinical characteristics were found to be similar between each subgroup. Also, when glucose levels were within the normal range, the difference in mean HbA1c was not significant (P=0.580). However, when plasma glucose levels were above the diagnostic cutoff for prediabetes and DM, the mean HbA1c of the anemic subgroup was modestly higher than in the nonanemic group. The specificity of HbA1c for diagnosis of DM was significantly lower in the anemic subgroup (P<0.05). CONCLUSION: These results suggest that the diagnostic significance of HbA1c might be limited in anemic patients.
Anemia ; Blood Glucose ; Diabetes Mellitus* ; Glucose Tolerance Test ; Hemoglobin A, Glycosylated* ; Humans ; Prediabetic State ; Reference Values ; Sensitivity and Specificity

The Duhamel procedure has been the authors' treatment of choice for children with Hirschsprung's disease over the last 15 years. Owing to advancements in instrumentation and technique, laparoscopic correction of some congenital anomalies, including congenital megacolon, have become possible. In March and May of 1997, laparoscopic Duhamel procedures were performed on a girl and boy aged 7 months and 2 years and 9 months, respectively. The primary diagnosis in both patients was Hirschsprung's disease, which was confirmed by barium-enema and mucosal suction biopsy. The boy did not need construction of a loop colostomy, while the girl had a colostomy made neonatally. Using 4 trocars, the sigmoid colon, proximal rectum, and posterior rectal wall were mobilized laparoscopically. Immdeiately after severing the proximal resection line, the proximal end was pulled down posterior to the dentate line for side-to-side anastomosis with GIA and Endo-GIA staplers perineally. The colonic remnant was then resected with staplers and harvested through the right lower quadrent port site. Operative time was 210 minutes for the boy and 200 minutes for the girl. No perioperative complications were noted, and conversion to laparotomy was never required. Regular diet was resumed 4 days (boy) and 2 days (girl) after the operations. Postoperative hospital stay was 7 days in the two cases. We successfully performed laparoscopic Duhamel procedures for the first time in Korea, and think it feasible, safe, andminimally invasive owing to size reduction of the incision and avoidence of intraperitoneal opening of the bowel.
Biopsy ; Child ; Colon ; Colon, Sigmoid ; Colostomy ; Diagnosis ; Diet ; Female ; Hirschsprung Disease* ; Humans ; Korea* ; Laparoscopy ; Laparotomy ; Length of Stay ; Male ; Operative Time ; Rectum ; Suction ; Surgical Instruments