We herein report two patients with epidermolysis bullosa acquisita(EBA), who had showed the atypical clinical features. A 25-year-old male presented with multiple pruritic vesicles, erosion and crusts which occurred more severely on the face than any other sites of the skin and healed with atrophic scar formation. Histopathologically, there was a subepidermal neutrophilic blister with moderate perivascular lymphohistiocytic infiltrates in the dermis, and the diagnosis of EBA was confirmed by means of direct immunofluorescence and salt-split direct immunofluorescence test performed on the perilesional skin. The other patient was a 24-year-old female who had had multiple painful ulcerative lesions on the oral mucosa for 4 months. After then, vesicles and bullae developed to progressively generalize to the anterior neck, chest and lower leg. Histolopathologic examination of peribullous skin showed a subepidermal bulla with neutrophils and eosinophils. The salt-split skin direct immunofluence test showed IgG and IgM binding to the dermal side only. We diagnosed this patient as nonscarring inflammatory EBA. Both patients were treated with prednisolone, colchicine, and dapsone resulting in clinical improvement, but their skin lesions recurred several months after discontinuing medication.
Adult ; Blister ; Cicatrix ; Colchicine ; Dapsone ; Dermis ; Diagnosis ; Eosinophils ; Epidermolysis Bullosa Acquisita* ; Epidermolysis Bullosa* ; Female ; Fluorescent Antibody Technique, Direct ; Humans ; Immunoglobulin G ; Immunoglobulin M ; Leg ; Male ; Mouth Mucosa ; Neck ; Neutrophils ; Prednisolone ; Skin ; Thorax ; Ulcer ; Young Adult

PURPOSE: Grave's disease is an autoimmune disease with chronic and systemic features. It affects the orbital fat and muscle bringing about defect in extrinsic eye motility, diplopia, optic nerve defect and lid retraction. In patients with lagopthalmos and resulting facial deformity, treatment can be done by rectus muscle recession or filling with various material. Autogenous auricular cartilage graft is often used and synthetic material such as synthetic acellular dermis, polyethylene meshs are also used for filling of the depressed area. Nevertheless, autogenous auricular cartilage grafts are difficult to utilize and synthetic materials sometimes result in protrusion or infection. Therefore, hard palate mucosa was considered as an alternative. We report two cases of patients with lower eyelid retraction corrected with autogenous hard palate mucosa. METHODS: We performed this operation in two patients of Graves' ophthalmopathy. The capsulopalpebral fascia was incised and elevated through an incision on the conjunctiva. Then, the harvested hard palate mucosa was sutured to the inferior border of the tarsus and covered with the conjunctiva. RESULTS: The lower eyelid retraction was corrected successfully. No hypertrophy or deformation of the transplanted hard palate mucosa was noted 6 months after the surgery. CONCLUSIONS: From the results above, we may conclude that the hard palate mucosa serves as an ideal spacer for the curvature and the inner lining in lower lid retraction. Hard palate mucosa is as sturdy as the autogenous cartilage but is much easier to utilize. It can be also used for lid retraction after lower lid aesthetic surgeries or traumas.
Acellular Dermis ; Ankle ; Autoimmune Diseases ; Cartilage ; Congenital Abnormalities ; Conjunctiva ; Diplopia ; Ear Cartilage ; Eyelids* ; Fascia ; Humans ; Hyperthyroidism ; Hypertrophy ; Mucous Membrane* ; Optic Nerve ; Orbit ; Palate, Hard* ; Polyethylene ; Transplants

PURPOSE: Helicobacter pylori infection is thought to be correlated with iron-deficiency anemia (IDA) at puberty. The H. pylori feoB gene, a high-affinity ferrous iron transporter, plays a central role in iron acquisition. This study aims to analyze the H. pylori feoB status according to the presence of antral gastritis with or without IDA. METHODS: Fourteen H. pylori-positive patients aged from 10~18 years were categorized into subgroups based on the presence or absence of IDA. Eight patients had IDA, and the other six showed normal hematological findings. Genomic DNA was isolated from cultured H. pylori. Five sets of primers were used for PCR amplification of the feoB gene. The feoB region, 1.93 kb, was generated by linking of the PCR products and sequenced. The feoB gene sequences of H. pylori J99 and 26695 were used to compare with the clinical strains. Sequence comparisons of the feoB regions between the IDA (+) and (-) groups were performed. RESULTS: Sequence analysis of the complete coding region of the feoB revealed 16 sites of polymorphism. Among these, 3 polymorphisms-Glu/Thr254Ala, Ile263Val, and Lys511Gln - were indigenous to Korean strains. Although statistically significant differences appear in 4 sites between IDA (+) and (-), the number of specimens are too low to assess the real differences. CONCLUSION: The 4 polymorphisms in the feoB gene seem to be related with IDA, but it is unclear yet because of small number of study strains. Further studies are required to prove the correlation of IDA and H. pylori infection.
Adolescent ; Anemia, Iron-Deficiency* ; Clinical Coding ; DNA ; Gastritis ; Genes, vif ; Helicobacter pylori* ; Helicobacter* ; Humans ; Iron ; Korea* ; Polymerase Chain Reaction ; Puberty ; Sequence Analysis

BACKGROUND: Allogeneic bone marrow transplantation across ABO incompatibility barriers may result in immune mediated hemolysis. Hemolysis may be avoided by RBC depletion from the graft. In vitro graft manipulations carry the risk of hematopoietic stem cell loss, a factor that may be most important in graft failure. We report 16 major ABO blood group incompatible allogeneic bone marrow transplants using erythrocyte depletion of marrow prior to infusion. METHODS: From March 1997 to July 2001 in Yonsei University College of Medicine, 16 patients underwent ABO blood group incompatible allogeneic BMT: 5 for acute myelocytic leukemia, 5 for severe aplastic anemia, 3 for acute lymphocytic leukemia, 2 for chronic myelocytic leukemia, and 1 for myelodysplastic syndrome. RBC depletions were done with automatic cell separator, COBE Spectra (COBEBCT Inc., Lakewood, USA). RBC removal rates and mononuclear cell recovery rates were calculated. And the evidence of successful engraftment and intravascular hemolysis were also evaluated. RESULTS: The RBC removal rate was 99.1+/-0.0% and a mean of 1% of the original red cell volume was contained in the final infusate. The mononuclear cell recovery rate was 70.0+/-16.3% from the original MNCs. Fourteen patients tolerated the infusion of the marrow concentrates without any adverse effects. Two patients experienced hemoglobinuria, but disappeared within 2 days by continued observation. After transplantation, absolute neutrophil counts exceeded 500/nL by 10.8+/-1.9 days, platelet counts exceeded 50,000/nL by 30.5+/-8.5 days, and reticulocytosis sustained at >1% was by 25.8+/-13.9 days. CONCLUSION: RBC depletion from ABO major mismatched bone marrow aspirates by the automatic cell separator is a safe and effective technique.
Anemia, Aplastic ; Blood Component Removal* ; Bone Marrow Transplantation ; Bone Marrow* ; Cell Size ; Erythrocytes ; Hematopoietic Stem Cells ; Hemoglobinuria ; Hemolysis ; Humans ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Leukemia, Myeloid, Acute ; Myelodysplastic Syndromes ; Neutrophils ; Platelet Count ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Reticulocytosis ; Transplants

No abstract available.
Hemorrhage* ; Humans ; Prognosis*

OBJECTIVE: To investigate the effect of treadmill aerobic exercise for eight weeks on cardiopulmonary fitness and functional mobility in stroke patients METHOD: Twenty one stroke patients who could walk independently and had MMSE score of 24 or higher were included. We used maximal exercise test by means of modified Harbor protocol before and after eight weeks' treadmill aerobic exercise. We monitored patients' status with EKG, heart rate, blood pressure and Borg rating of perceived exertion, and measured maximal O2 consumption, maximal heart rate, blood pressure, minute ventilation, rate pressure product and respiratory exchange ratio during maximal exercise test. We also assessed functional mobility by Fugl-Meyer assessment of lower extremity, Berg balance scale, five times sit to stand test, timed up & go test, dynamic gait index scale, activities-specific balance confidence scale and Korean-modified Barthel index before and after 8 weeks' training. RESULTS: Twenty one patients completed treadmill aerobic exercise training. After eight weeks' treadmill aerobic exercise, the cardiopulmonary fitness, measured by maximal O2 consumption, maximal heart rate, minute ventilation, systolic blood pressure, functional mobility measured by Fugl-Meyer assessment of lower extremity, Berg balance scale, five times sit to stand test, timed up & go test, dynamic gait index scale, activities-specific balance confidence scale and Korean-modified Barthel index showed statistically significant improvement (p<0.05). CONCLUSION: Eight weeks' treadmill aerobic exercise improved the cardiopulmonary fitness and functional mobility in ambulatory stroke patients.
Blood Pressure ; Electrocardiography ; Exercise ; Exercise Test ; Gait ; Heart Rate ; Humans ; Lower Extremity ; Stroke ; Ventilation

DNA barcoding has been widely used in species identification and biodiversity research. A short fragment of the mitochondrial cytochrome c oxidase subunit I (COI) sequence serves as a DNA bio-barcode. We collected DNA barcodes, based on COI sequences from 156 species (529 sequences) of fish, insects, and shellfish. We present results on phylogenetic relationships to assess biodiversity the in the Korean peninsula. Average GC% contents of the 68 fish species (46.9%), the 59 shellfish species (38.0%), and the 29 insect species (33.2%) are reported. Using the Kimura 2 parameter in all possible pairwise comparisons, the average interspecific distances were compared with the average intraspecific distances in fish (3.22 vs. 0.41), insects (2.06 vs. 0.25), and shellfish (3.58 vs. 0.14). Our results confirm that distance-based DNA barcoding provides sufficient information to identify and delineate fish, insect, and shellfish species by means of all possible pairwise comparisons. These results also confirm that the development of an effective molecular barcode identification system is possible. All DNA barcode sequences collected from our study will be useful for the interpretation of species-level identification and community-level patterns in fish, insects, and shellfish in Korea, although at the species level, the rate of correct identification in a diversified environment might be low.
Biodiversity ; DNA ; DNA Barcoding, Taxonomic ; DNA, Mitochondrial ; Electron Transport Complex IV ; Insects ; Korea ; Shellfish

OBJECTIVES: To quantitate apoptosis and Fas antigen expression of T lymphocytes by activation in aplastic anemia (AA) and compare with that of normal controls and completely-recovered AA, and to investigate the apoptotic sensitivity to anti-fas antibody of activated T lymphocytes in AA. METHODS: We studied the expression of Fas antigen on fresh T lymphocytes of twenty patients with AA [13 newly diagnosed, 7 recorvered AA after immunosuppressive therapy (IST)], and investigated the activation-induced cell death (AICD) and Fas expression by activation [interleukin-2 (200 U/ml) and phytohemagglutinin (50 micrograms/ml)] in 5 newly-diagnosed AA, 5 normal controls and 5 AA in complete response (CR). Apoptotic sensitivity to anti-Fas antibody was assessed by the time-course kinetics of induction of cell death by anti-Fas antibody (500 ng/ml). RESULTS: There was no significant difference of Fas antigen expression on freshly-isolated T lymphocytes among newly-diagnosed severe AA, normal control s and patients with AA in CR after IST. In normal controls, T lymphocytes death was greatly increased at 3 days of activation, and Fas antigen expression on T lymphocytes was increased above baseline at day 1 of activation. In contrast, in newly-diagnosed AA, T lymphocytes showed delayed cell death, which correlated with a slowed increase of Fas antigen expression by activation. Also, anti-Fa s antibody sensitivity of activated T lymphocytes was decreased in newly-diagnosed AA. In completely recovered AA, these abnormal AICD and Fas antigen expressions by activation were recovered to normal range. CONCLUSIONS: Abnormal AICD plays a role in the immune pathophysiology of AA, and defective Fas system is involved in this process.
Anemia, Aplastic/pathology ; Anemia, Aplastic/immunology* ; Antigens, CD95/blood ; Apoptosis ; Case-Control Studies ; Human ; In Vitro ; Lymphocyte Transformation ; T-Lymphocytes/pathology* ; T-Lymphocytes/immunology* ; Time Factors

Laparoscopic surgery has recently extended its indications and it has also become an acceptable surgical approach for splenectomy. In the last five years, we have performed 40 laparoscopic splenectomies for immune thrombocytopenic purpura. Thirty-five patients were female and 5 patients were male. The mean age was 34, varying from 17 to 56. After learning to perform laparoscopic splenectomy with five ports, we are now usually using three or four ports in a right lateral kidney position. There was no case of conversion to exploratory laparotomy. The mean hospital stay was 7 days. There was no perioperative mortality; but in 2 cases we had postoperative subphrenic abscesses which were successfully managed by catheter drainage. Since undergoing laparoscopic splenectomy, 28 patients (70%) were weaned effectively from their steroid medications. Eight patients (20%) have been on small doses of steroid, and 4 patients (10%) have been on the same doses of steroid with no response. The patient group with rapidly increasing platelet count after splenectomy showed a statistically significant relation with the complete response group (p < 0.001). Laparoscopic splenectomy is a safe and reasonable operative procedure for patients with immune thrombocytopenic purpura.
Adolescence ; Adult ; Female ; Human ; Laparoscopy* ; Male ; Middle Age ; Purpura, Thrombocytopenic, Idiopathic/surgery* ; Splenectomy*

BACKGROUND: It is known that various subtypes of obesity exist and there has been a growing interest in the various subtypes of obesity, especially in the unique "metabolically healthy but obese" subtype. The purpose of this study was to analyze the features of obese adults without metabolic complications and to provide data which can be used in strategy formation for the effective treatment of obesity. METHODS: A total of 4,561 obese adults with a BMI greater than or equal to 25 kg/m2, were recruited from a health promotion center of a university hospital. Sociodemographic and life style information were gathered by self-questionnaire. We classified them into 2 groups-one having no features (metabolically healthy obese; MHO) and the other having 1~5 features (metabolically unhealthy obese; MUHO)-using the ATP III definition for metabolic syndrome, and used the multiple logistic regression analysis to examine the association between various factors and MHO. RESULTS: Age and BMI were related with MHO independently. Adjusted odds ratio for the MHO men was 2.17 compared with women (P<0.001). Adjusted odds ratio for the MHO regular exercise group was 1.66 compared with the no exercise group (P<0.007). Additionally, those who exercised more had a greater probability of being in the MHO group. Adjusted odds ratio for the MHO in the large income group was 1.88 (P=0.007) compared with those in the small income group. As was seen with exercise, there was a direct relationship between higher income and the probability of being in the MHO group. CONCLUSION: Men compared with women, large income compared with small income, and regular exercise compared with no exercise were features of obese adults which increased their probability of being categorized as "metabolically healthy but obese".
Adenosine Triphosphate ; Adult* ; Female ; Health Promotion ; Humans ; Life Style ; Logistic Models ; Male ; Obesity ; Odds Ratio