Congenital smooth musele hamartomas appear at birth as hypertrichotic patches or plaques with or without hyperpigmentation. Histologic characteristic is hyperplasia of dermal smooth muscle bundles. We report a case of congenital smooth muscle harnartoma in 5-year-old female, who showed localized excessive-hairy, skin-colored patch on the lateral surface of left. elbow, which were found at birth. Biopsy specimen showed hyperpigmentation of the basal layers of epidermis, melanophages in upper dermis, and hyperplasia of smooth muscle bundles in lower dermis.
Biopsy ; Child, Preschool ; Dermis ; Elbow ; Epidermis ; Female ; Hamartoma* ; Humans ; Hyperpigmentation ; Hyperplasia ; Hypertrichosis ; Muscle, Smooth* ; Parturition

BACKGROUND: CTLA4 (CD152), which is expressed on the surface of T cells following activation, has a much higher affinity for B7 molecules comparing to CD28, and is a negative regulator of T cell activation. In contrast to stimulating and agonistic capabilities of monoclonal antibodies specific to CTLA-4, CTLA4Ig fusion protein appears to act as CD28 antagonist and inhibits in vitro and in vivo T cell priming in variety of immunological conditions. We've set out to confirm whether inhibition of the CD28-B7 costimulatory response using a soluble form of human CTLA4Ig fusion protein would lead to persistent inhibition of alloreactive T cell activation. METHODS: We have used CHO-dhfr cell-line to produce CTLA4Ig fusion protein. After serum free culture of transfected cell line we purified this recombinant molecule by using protein A column. To confirm characterization of fusion protein, we carried out a series of Western blot, SDS-PAGE and silver staining analyses. We have also investigated the efficacy of CTLA4Ig in vitro such as mixed lymphocyte reaction (MLR) & cytotoxic T lymphocyte (CTL) response and in vivo such as experimental autoimmune encephalomyelitis (EAE), graft versus host disease (GVHD) and skin-graft whether this fusion protein could inhibit alloreactive T cell activation and lead to immunosuppression of activated T cell. RESULTS: In vitro assay, CTLA4Ig fusion protein inhibited immune response in T cell-specific manner: 1) Human CTLA4Ig inhibited allogeneic stimulation in murine MLR; 2) CTLA4Ig prevented the specific killing activity of CTL. In vivo assay, human CTLA4Ig revealed the capacities to induce alloantigen-specific hyporesponsiveness in mouse model: 1) GVHD was efficiently blocked by dose-dependent manner; 2) Clinical score of EAE was significantly decreased compared to nomal control; 3) The time of skin-graft rejection was not different between CTLA4Ig treated and control group. CONCLUSION: Human CTLA4Ig suppress the T cell-mediated immune response and efficiently inhibit the EAE, GVHD in mouse model. The mechanism of T cell suppression by human CTLA4Ig fusion protein may be originated from the suppression of activity of cytotoxic T cell. Human CTLA4Ig could not suppress the rejection in mouse skin-graft, this finding suggests that other mechanism except the suppression of cytotoxic T cell may exist on the suppression of graft rejection.
Animals ; Antibodies, Monoclonal ; B7 Antigens ; Blotting, Western ; Cell Line ; Electrophoresis, Polyacrylamide Gel ; Encephalomyelitis, Autoimmune, Experimental ; Graft Rejection ; Graft vs Host Disease ; Homicide ; Humans ; Immunosuppression ; Lymphocyte Culture Test, Mixed ; Lymphocytes ; Mice ; Silver Staining ; Staphylococcal Protein A ; T-Lymphocytes

Catecholamine-induced cardiomyopathy associated with neuroblastoma is rarely reported. We report a case of catecholamine-induced cardiomyopathy associated with neuroblastoma in a 33-month-old female that was treated with extracorporeal membrane oxygenation (ECMO). She was tentatively diagnosed with acute myocarditis and presented with hypertension. Because of rapid patient deterioration despite pharmacological treatments, ECMO was applied. ECMO can be helpful in cases of catecholamine-induced cardiomyopathy associated with neuroblastoma.
Cardiomyopathies* ; Catecholamines ; Child, Preschool ; Extracorporeal Membrane Oxygenation* ; Female ; Humans ; Hypertension ; Myocarditis ; Neuroblastoma*

No abstract available.

No abstract available.

We observed a case of sebaceous epithelioma associated with a nevus sebaceus in a 25-year-old male. The tumor was bean-sized, dome-shaped nodule on the slightly yellowish plaque of nevus sebaceus. Histopathological finding shows undifferentiated cells which are arranged in a palisade fashion at the periphery of a cell mass and a fairly large number of transitional cells and groups of mature sebaeeous cells.
Adult ; Carcinoma* ; Humans ; Male ; Nevus* ; Nevus, Sebaceous of Jadassohn*

We analyzed ERCP findings of 15 patients with clonorchiasis, that were confirmed by stool examination and operation from May, 1976 to September, 1980. The results were as follows. 1) Filling defects due to adult worm of clonorchis sinensis were significant direct findings of clonorchiasis. 2) Irregular stenosis, fuzziness, raggedness, peripheral dilation and disturbance of peripheral filling of intrahepatic bile ducts were significant indirect findings of clonorchiasis. 3) ERCP was excellent method to evaluate biliary tree in patients with clonorchiasis, who showed obstructive jaundice.
Adult ; Bile Ducts, Intrahepatic ; Biliary Tract ; Cholangiopancreatography, Endoscopic Retrograde* ; Clonorchiasis* ; Clonorchis sinensis ; Constriction, Pathologic ; Humans ; Jaundice, Obstructive

PURPOSE: To suggest the optimal radiation fields after a surgical resection based on a nationwide survey on the principles of radiotherapy for rectal cancer in the Korean Patterns of Care Study. MATERIALS AND METHODS: A consensus committee, composed of radiation oncologists from 18 hospitals in Seoul Metropolitan area, developed a survey format to analyze radiation oncologist's treatment principles for rectal cancer after a surgical resection. The survey format included 19 questions on the principles of defining field margins, and was sent to the radiation oncologists in charge of gastrointestinal malignancies in all Korean hospitals (48 hospitals). Thirty three (69%) oncologists replied. On the basis of the replies and literature review, the committee developed guidelines for the optimal radiation fields for rectal cancer. RESULTS: The following guidelines were developed: superior border between the lower tip of the L5 vertebral body and upper sacroiliac joint; inferior border 2~3 cm distal to the anastomosis in patient whose sphincter was saved, and 2~3 cm distal to the perineal scar in patients whose anal sphincter was sacrificed; anterior margin at the posterior tip of the symphysis pubis or 2~3 cm anterior to the vertebral body, to include the internal iliac lymph node and posterior margin 1.5~2 cm posterior to the anterior surface of the sacrum, to include the presacral space with enough margin. Comparison with the guidelines, the replies on the superior margin coincided in 23 cases (70%), the inferior margin after sphincter saving surgery in 13 (39%), the inferior margin after abdominoperineal resection in 32 (97%), the lateral margin in 32 (97%), the posterior margins in 32 (97%) and the anterior margin in 16 (45%). CONCLUSION: These recommendations should be tailored to each patient according to the clinical characteristics such as tumor location, pathological and operative findings, for the optimal treatment. The adequacy of these guidelines should be proved by following the Korean Patterns of Care Study.
Anal Canal ; Cicatrix ; Consensus ; Humans ; Lymph Nodes ; Radiotherapy ; Rectal Neoplasms* ; Sacroiliac Joint ; Sacrum ; Seoul

A totally implanted venous access system was inserted in 19 children with cancer. The devices were utilized for the administration of antineoplastic drugs, parenteral fluids, antibiotics, and blood products. Total duration of implantation was 4,046 days for 23 implanted system (range 7-445 days). Complications included cather infection (0.247/100 catheter days), occlusion (0.692/100 catheter days), and dislodgement of needle (0.643/100 catheter days). There were major complications that necessitated removal of catheters, including systemic infections (0.09/100 catheter days) and complete occlusions (0.09/100 catheter days). The system was thought to be safe and convenient in chemotherapy, and permitted full physical activity.
Anti-Bacterial Agents ; Antineoplastic Agents ; Catheters ; Child* ; Drug Therapy ; Humans ; Motor Activity ; Needles ; Prospective Studies*

In order to observe the development of ventricular arrhythmia during regional myocardial ischemia and reperfusion, especially under the presence or absence of ST-T electrical alternans on epicardial EKG. The proximal left descending coronary artery(LAD) was ligated for 20 minutes and then reperfused suddenly in twenty-three cats. Standard lead EKG(Lead??, chest lead EKG and epicardial lead EKG were recorded simultaneously during the occlusion and reperfusion respectively. During the ligation of LAD, STEA was observed in thirteen cats(56.5%). In occlusion period, the incidence of ventricular tachycardia in STEA positive group was significantly higher than in the negative group(p<0.01) and arrhythmic score was significantly higher(p<0.005) also In the reperfusion period the incidence of vefntricular fibrillation in STEA positive group was significantly higher than in the negative group(p<0.025). But there was the tendency to be higher in arrhythmic score of STEA positive group. There were no differences in heart rate, systolic left ventricular pressure, ST elevation and ST width in both groups. Most forms of ST-T of sinus rhythm before and after development of ventricular premature beat was low form(L). It was concluded that at the presence of STEA on EKG, the incidence of ventricular arrhythmia was more prevalent. So, STEA can be available as a marker of ventricular arrhythmia and prognostic factor.
Animals ; Arrhythmias, Cardiac* ; Cardiac Complexes, Premature ; Cats ; Electrocardiography* ; Heart Rate ; Incidence ; Ligation ; Myocardial Ischemia ; Reperfusion ; Tachycardia, Ventricular ; Thorax ; Ventricular Pressure