We report a case of malignant histiocytosis which began with intermittent fever and scaly skin lesions. A 3-year-old girl presented with erythematous scaly papules on the face and the trunk, and high fever for 3 months. The cutaneous lesions consisted of widespread coin-sized erythematous scaly papules with marginal brownish pigmentation. She was anemic and thrombocytopenic and had impairment of the liver function. Histopathologic study of the skin lesions showed non-specfic findings except for hyperkeratosis. However, bone marrow examination revealed an increased number of histiocytes, mostly immature with active phagocytosis of erythroid cells, myeloid cells, and platelets. She was diagnosed as having malignant histiocytosis and treated with cyclophosphamide and vincristine. She died the next day after the treatment had begun.
Bone Marrow Examination ; Child, Preschool ; Cyclophosphamide ; Erythroid Cells ; Female ; Fever* ; Histiocytes ; Histiocytic Sarcoma* ; Humans ; Liver ; Myeloid Cells ; Phagocytosis ; Pigmentation ; Skin* ; Vincristine

No abstract available.
Erythema* ; Leukemia, Myelomonocytic, Juvenile*

Percutaneous balloon valvuloplasty (PBV) was done in 7 adults of pulmonary valvular stenosis, which was diagnosed with catheterization and cine-angiography of rigtht heart, in Seoul National University Hospital from Jan. 1986 to Dec. 1986. The results were as follows : 1) Among the seven cases six were male and the range of age was from 19 to 40 years. Atrial septal defects associated in 2 cases were not significant clinically. 2) The peak systolic gradient from right ventricle to pulmonary artery before the procedure was below 50 mmhg in 1 cases, 50 to 100 mmHg in 1 case, and above 100 mmHg in 5 cases. 3) Before PBV the mean systolic pressure of right ventricle was 121+/-42 mmHg and the peak systolic gradient was 106+/-41 mmHg. After PBV those were 58+/-30 mmHg and 40+/-31 mmHg, revealing significant improvement. 4) Balloon catheter (Medi-tech, 9F) was used in all cases. single balloon of 20 mm outer diameter was applied in 2 cases and double balloons of various sizes were applied in 5 cases in which single balloon showed inadequate dilatation. It is suggested that percutaneous balloon valvuloplasty is safe and effective alternative to surgical valvotomy of pulmonary valvular stenosis in adults.
Adult* ; Balloon Valvuloplasty* ; Blood Pressure ; Catheterization ; Catheters ; Constriction, Pathologic* ; Dilatation ; Heart ; Heart Septal Defects, Atrial ; Heart Ventricles ; Humans ; Male ; Pulmonary Artery ; Seoul

This study evaluated the consistency between two quantitative fit test devices with different methods of ambient aerosol counting. Three types of respirators (N95, half mask, and full facepiece) were worn by 50 participants (male, n = 25; female, n = 25), PortaCount (Pro+ 8038) and MT (05U) were connected to one probe to one mask, and fit factors (FFs) were measured simultaneously with the original and modified protocols. As a result of comparing MT FFs with PortaCount FFs as references and by applying for the pass/fail criteria (FF = 100), the consistency between the two devices for half masks and full facepieces was very high. N95 was somewhat weaker than the two type of respirators in the consistency; however, the correlation between the two devices was very strong (p < 0.0001). The results showed that an FF of 100 as measured by PortaCount was likely to be measured as 75 by the MT. Therefore, when performing the fit test for N95 using the MT and pass level of FF 100, a certain level of adjustment is necessary, whether end-user or putting a scaling factor by manufacturer.

BACKGROUND: Leukocyte reduction filters are widely used to prevent transfusion reactions caused by leukocytes in blood components. Commercial filters are not sufficient for removal of leukocytes for prevention of transfusion associated graft-versus-host disease; therefore, irradiation of blood components was performed using expensive equipment. Techniques using an aptamer substituted for antibody have been developed and are available in clinical areas. The purpose of this study is to develop the aptamer filter system and to evaluate its efficiency and the possibility of its clinical application. METHODS: Aptamers targeted to CD45 were selected by the Postech Aptamer Initiative. The aptamer filter in which aptamers attached to beads were bound to leukocytes and removed by magnetic field was developed. Filtration of 14 units of leukoreduction-red blood provided by Korean Red Cross Blood Services was performed using aptamer filters. Leukocyte removal rate and red cell recovery rate were evaluated and bacterial culture was performed. RESULTS: After filtration using the aptamer filters, 45.6% of leukocytes were additionally removed and the red cell recovery rate was 92.8%. No growth in the bacterial culture was observed. CONCLUSION: In order to apply the cell depletion technique utilizing an aptamer to blood filter system, we developed and evaluated the aptamer filter system. Through improvement of the binding efficiency of the aptamer and the filtering process, and application of the various aptamers for other different cells, we suggest that this technique can be applied in the clinical area, such as a substitution for the irradiation process for TAGVHD prevention.
Blood Group Incompatibility ; Filtration ; Leukocytes ; Magnetic Fields ; Red Cross

Solid pseudopapillary tumors are rare primary neoplasms of the pancreas that have a relatively favorable prognosis, with a low malignant potential. Most patients present with non-specific clinical features, such as vague abdominal discomfort and a palpable abdominal mass. A 50-year-old woman who presented with hematemesis had an incidental pancreatic cystic mass on abdominal computed tomography (CT). We performed several tests, including upper endoscopy and angiography, but we could not find the bleeding focus. Therefore, we performed a pancreaticoduodenectomy. At laparotomy, there was a 10-cm encapsulated pancreatic mass with duodenal invasion, which showed a bleeding ulcer. A solid pseudopapillary tumor was diagnosed pathologically. We report a solid pseudopapillary tumor that presented with gastrointestinal bleeding.
Angiography ; Endoscopy ; Female ; Hematemesis ; Hemorrhage ; Humans ; Laparotomy ; Middle Aged ; Pancreas ; Pancreatic Cyst ; Pancreaticoduodenectomy ; Prognosis ; Ulcer

Ventricular tachycardias(VTs) usually complicate iscemic heart disease. Cardiomyopathy or other organic heart disease and are aften associated with sudden cardiac death. Ventricular tachycardias with no apparent cardiac abnormalities, however, have been known to have different clinical features comprising a unique clinical entity among other tachycardias. Eleven patients(7men, 4 women) were investigated on their clinical features, electrophysiologic studies and late potentials. 1) The mean age at diagnosis was 23.4+/-6.5 years and the predominating clincal features were palpitation, syncope or congestive heart failure. No death occured over a mean follow up period of 29.2+/-26.5 months. 2) The resting ECGs were within normal limits in 8 patients and showed nonspecific ST-T changes in 3 patients. 3) Ventricular pacing induced sustained ventricular tachycardias in 7 patients and nonsustained VT in 1 aptient. Atrial pacing could provoke sustained VTs in 3 patients. 4) His bundle electrograms(HBE) were obtained in 7 patients during tachycardial. The HV intervals of the induced VTs distributed between 0 to -25msec in 5 patients and there was no definite His potentials in two patients. 5) The coupling interval of premature stimuli and the echo interval(the interval from the stimulus to the initiation of resulting VT) showed inverse relationship in 5 cases. No direct relationship was observed. 6) The termination mode, observed in 7 patients, showed delayed ternmination in 2 patients. 7) Late potentials(LPs) were pisitive in three patients but there was no significant statistical difference between LPs in normal control and those with idiopathic sustained left VTs. 8) Verapamil terminated VTs in 8 patients and slowed the rate of tachycardial in 1 patient. In conclusion, idiopathic sustained left ventricular tachycardias occur in younger age group and have more favorable prognosis. The findings of EPS and LPs suggest microreentry or triggered activity as their basic mechanism and verapamil can be used effectively for their termination.
Bundle of His ; Cardiomyopathies ; Death, Sudden, Cardiac ; Diagnosis ; Electrocardiography ; Follow-Up Studies ; Heart Diseases ; Heart Failure ; Humans ; Prognosis ; Syncope ; Tachycardia ; Tachycardia, Ventricular* ; Verapamil

PURPOSE: Angiogenesis is involved in the pathogenesis of chronic rhinosinusitis with nasal polyps. We aimed to investigate the effects of prostaglandin E2 (PGE2) on vascular endothelial growth factor (VEGF) production, the role of E-prostanoid (EP) 4 receptors, and the signal transduction pathway mediating VEGF production in nasal polyp-derived fibroblasts (NPDFs). METHODS: Eight primary NPDF cultures were established from nasal polyps, which were incubated with or without PGE2. Reverse transcription-polymerase chain reaction amplification of EP receptors (EP1, EP2, EP3, and EP4) and immunofluorescence staining for VEGF production were performed. VEGF production via the cyclic adenosine monophosphate (cAMP)-dependent protein kinase A (PKA) and phosphatidylinositol 3-kinase (PI3K) pathways was evaluated by enzyme-linked immunosorbent assay. RESULTS: All EP receptors were expressed in NPDFs. PGE2 significantly increased VEGF production concentration- and time dependently, and VEGF production was regulated by an EP4 receptor. Activation of intracellular cAMP regulated VEGF production. VEGF production was decreased by PKA and PI3K inhibitors via intracellular cAMP. CONCLUSIONS: PGE2 stimulates VEGF production via the EP4 receptor in NPDFs. These results indicate that PGE2-induced VEGF production is mediated, at least partially, through cAMP-dependent signaling pathways. Therapies targeting the EP4 receptor may be effective in inhibiting the development of nasal polyps.
Adenosine Monophosphate ; Cyclic AMP-Dependent Protein Kinases ; Dinoprostone ; Fibroblasts ; Fluorescent Antibody Technique ; Nasal Polyps ; Negotiating ; Phosphatidylinositol 3-Kinase ; Signal Transduction ; Vascular Endothelial Growth Factor A

Pneumonia, along with graft-versus-host disease, is a major cause of morbidity and mortality in patients receiving bone marrow transplantation. The community respiratory virus infections have been found to be large causes of pneumonia. Upper respiratory infection with Parainfluenza virus can progress to severe lower respiratory diseases in bone marrow transplant recipients, of which clinical findings are similar to those of pneumonia by exotic opportunistic pathogens. We report a patient with chronic myelogenous leukemia who had suffered a community-acquired pneumonia by Parainfluenza virus type 3 after bone marrow transplantation.
Bone Marrow Transplantation* ; Bone Marrow* ; Graft vs Host Disease ; Humans ; Leukemia ; Leukemia, Myelogenous, Chronic, BCR-ABL Positive ; Mortality ; Parainfluenza Virus 3, Human* ; Paramyxoviridae Infections* ; Pneumonia* ; Transplantation

Duodenal varices are a rare site of hemorrhage in patients with intrahepatic and extrahepatic portal hypertension, but their bleeding is life-threatening complication. Diagnosis of duodenal varices may be difficult, requiring careful inspection of the duodenal bulb during endoscopy. Diagnosis of duodenal varices may also be revealed using angiography and transhepatic portography. Treatment of duodenal varices include endoscopic injection sclerotherapy (EIS), endoscopic variceal ligation (EVL), surgical procedures, and interventional radiology. A 50-year-old woman with liver cirrhosis was admitted with melena and hematochezia. An endoscopy on admission showed esophageal and fundal varices without any bleeding stigma. Colonoscopy showed moderate amount of fresh blood in the terminal ileum. 99mTc-labelled RBC scan showed a suspicion of a distal jejunal bleeding. She had explo-laparotomy of small bowel segmental resection based on 99mTc-labelled RBC but rebleeding occured. Selective mesenteric angiography failed to reveal the source of bleeding. Repeat endoscopy revealed undetected duodenal varix which was covered with fresh blood clots. Endoscopic injection sclerotherapy with n-butyl-2-cyano acrylate (Histoacryl) was performed and achieved succesful hemostasis.
Angiography ; Colonoscopy ; Diagnosis ; Enbucrilate* ; Endoscopy ; Female ; Gastrointestinal Hemorrhage ; Hemorrhage* ; Hemostasis ; Humans ; Hypertension, Portal ; Ileum ; Ligation ; Liver Cirrhosis ; Melena ; Middle Aged ; Portography ; Radiology, Interventional ; Sclerotherapy* ; Varicose Veins*