Authors report a typical case of congenital fiber type disproportion (CFTD) with unique clinicopathologic characteristics. The patient was a 13-year-old boy who presented with weakness of lower extremities, especially proximal muscle, since his infancy. He has suffered from severe scoliosis which got worse since the age of 12. He showed mild dysarthria, high arched palate, and fish face. All routine laboratory data were within normal limits. EMG findings suggested myopathy. The muscle biopsy revealed fiber type disproportion with type 1 predominance. While most of the type 1 myofibers were atrophic or normal in size, the type 2 fibers showed universal hypertrophy. The difference of mean diameter between the larger and the smaller fibers was 27.9%. The patient's clinicopathologic settings fulfilled the criteria of CFTD.
Adolescent ; Biopsy ; Dysarthria ; Humans ; Hypertrophy ; Lower Extremity ; Male ; Muscular Diseases* ; Myopathies, Structural, Congenital* ; Palate ; Scoliosis

Menetrier's disease is a rare, characterized by the presence of large rugal folds involving part or all of the stomach. Patients with hypertrophic gastropathy often have distressing abdominal symptoms, weight loss, and edema due to gastric protem loss. A 64 year old male patient was admitted with the chief complaint of epigastric discomfort. Endoscopic, radiologic and histologic examination, revealed characteristic findings of Menetrier's disease. A case of Menetrier's disease is reported with a brief review of literature.
Edema ; Gastritis, Hypertrophic* ; Humans ; Male ; Middle Aged ; Stomach ; Weight Loss

BACKGROUND: Atrial fibrillation is the commonest arrhythmia seen in clinical practice. However, there have been no epidemiologic data on its prevalence in general population of Korea. We performed this study to investigate the prevalence of atrial fibrillation in Korea. METHODS: From March 1, 1999 to July 31, 2000, Kangwon Branch, Korea Association of Health (KAH) parformed electrocardiograms to investigate the health status in the general public in Kangwon Province. We analyzed these data by of chi square(2) test. RESULTS: A total of 45,133 persons participated in the health screening performed by KAH. The number of persons above 40 years old were 13,768, with men and women 6,367 (46.2%) and 7,401 (53.8%)respectively. Prevalence among those above 40 years old was 0.72% and the prevalence among men, 0.96% (61 persons) was significantly higher than that of women, 0.51% (38 persons)(p<0.01). In the group above 60 years old with the prevalence of 1.24%, a similar phenomenon was observed with 1.57% (40 persons) in men and 0.97% (29 persons) in women (p<0.05). CONCLUSION: Prevalence of atrial fibrillation became higher as age increased, specifically in persons older than 40 years.
Adult ; Arrhythmias, Cardiac ; Atrial Fibrillation* ; Electrocardiography ; Female ; Gangwon-do* ; Humans ; Korea ; Male ; Mass Screening ; Middle Aged ; Prevalence*

Paraneoplastic limbic encephalitis (PLE) is a rare neurologic disorder that can be combined with several systemic tumor. PLE is commonly associated with small cell lung carcinoma, but others are uncommon. The cause is unknown, but it is thought to be a autoimmune disorder developing secondary to carcinomatous process. Progressive neurologic symptoms usually predate the diagnosis of cancer, most symptoms usually improve after resection of tumor. PLE can combined with several tumors, but it is very rare with immature ovarian teratoma. We report a case of 31-year-old woman with PLE combined with an immature ovarian teratoma whose neurologic symptoms improved after resection of primary tumor.
Adult ; Diagnosis ; Female ; Humans ; Limbic Encephalitis* ; Nervous System Diseases ; Neurologic Manifestations ; Prednisolone ; Small Cell Lung Carcinoma ; Teratoma*

BACKGROUND: Topoisomerase II (topo II) is a major target of anthracyclines and epipodophyllotoxins for anticancer treatment. The expression of topo II is low in drug resistant cell lines. High levels of glutathione S-transferase (GST)pi have been associated with emergence of cell lines resistant to alkylating agents or adriamycin. METHODS: By immunostaining with paraffin embedded bone marrow tissues, the expression of topo II alpha and GSTpi was investigated in 51 patients with acute myeloid leukemia (AML), and the relation of topo II alpha and GSTpi expression to treatment response in 29 patients with AML following induction chemotherapy was also evaluated. RESULTS: Topo II positive cells varied from less than 1% to 60% of leukemic cells and 20 (39.2%) were negative for topo II (positive cells<10%). Treatment response following chemotherapy was not related to topo II. 26 (51.0%) were positive for GSTpi. GSTpi expression was related to treatment resistance of the patients following chemotherapy. In the patients who showed both topo II alpha negative and GSTpi positive, the frequency of treatment resistance following chemotherapy was high. CONCLUSIONS: This study suggests that immunostaining of topo II alpha and GSTpi with the bone marrow paraffin sections of AML patients can be useful to predict the treatment response following chemotherapy and that further study including more patients with prospective study may substantiate topo II alpha and GSTpi as multidrug resistant markers.
Alkylating Agents ; Anthracyclines ; Bone Marrow ; Cell Line ; DNA Topoisomerases, Type II* ; Doxorubicin ; Drug Resistance, Multiple ; Drug Therapy ; Glutathione S-Transferase pi* ; Glutathione Transferase* ; Glutathione* ; Humans ; Immunohistochemistry ; Induction Chemotherapy ; Leukemia, Myeloid, Acute* ; Paraffin ; Podophyllotoxin

Anomalous unilateral single pulmonary vein is a rare abnormality of the pulmonary venous system characterized by a tortuous pulmonary venous confluence which never crosses the diaphragm and drains all arterialized blood from a lung to the left atrium, into which it normally flows. Cardiac and pulmonary anomalies are not associated. We report a case in which this condition was comfirmed by the findings of chest radiography, computed tomography, and magnetic resonance angiography.
Diaphragm ; Heart Atria ; Lung ; Magnetic Resonance Angiography ; Pulmonary Veins* ; Radiography ; Thorax

PURPOSE: Atopic dermatitis is often accompanied by food allergies which occur through skin barrier defects. Especially Staphylococcus aureus colonization can exacerbate skin barrier defects that cause sensitization and increase specific IgE (sIgE) to food. We investigated the association between skin colonization and food sIgE changes in children with atopic dermatitis. METHODS: Atopic dermatitis was diagnosed by a pediatric allergist in patients between 3 months and 3 years of age. Total IgE and sIgE to egg white, cow's milk, wheat, and peanuts were taken. Eosinophil count and eosinophil cationic protein were also taken. Comparisons were done between the groups with and without S. aureus colonization. RESULTS: It was found that 50.3% of the 294 enrolled patients had S. aureus colonization on lesional skin. Statistically significant sensitization to wheat and peanut were increased with S. aureus colonization. Statistically significant increases in sIgE (above cutoff level) were also found in egg white, milk, wheat and peanut. Higher S. aureus colony counts also increased sIgE of all foods. Methicillin-resistant S. aureus showed no statistical difference compared to methicillin-susceptible S. aureus in severity and sIgE levels. CONCLUSION: S. aureus colonization increases the risk of food sensitization in children with atopic dermatitis.
Arachis ; Child* ; Colon* ; Dermatitis, Atopic* ; Egg White ; Eosinophil Cationic Protein ; Eosinophils ; Food Hypersensitivity ; Humans ; Immunoglobulin E ; Methicillin Resistance ; Milk ; Skin ; Staphylococcus aureus* ; Staphylococcus* ; Triticum

We report a suspicious case of spontaneous bacterial peritonitis (SBP) caused by Providencia rettgeri and Clostridium perfringens in a patient with alcoholic cirrhosis. The patient presented with altered mentality and was taken to the emergency room. He was diagnosed with SBP after abdominal paracentesis and computed tomography and was treated with ceftriaxone and metronidazole. The pathogens were identified under suspicion of polymicrobial infection because of Gram-staining discrepancies between broth from blood culture bottles and colonies on solid media. He died of septic shock despite transfer to the intensive care unit. Although we could not conclude which organism had the leading role in this case of SBP and septicemia, we did verify the importance of Gram staining in a microbiology laboratory in terms of quality assurance.
Ceftriaxone ; Clostridium perfringens* ; Coinfection ; Emergency Service, Hospital ; Humans ; Intensive Care Units ; Liver Cirrhosis, Alcoholic ; Metronidazole ; Paracentesis ; Peritonitis* ; Providencia* ; Sepsis* ; Shock, Septic

Tumor necrosis factor (TNF)-alphacan induce ultrastructural changes in cultured human nasal epithelial cells. The aims of this study are to determine the recovery time from the changes induced by TNF-alphaand observe whether various degrees of changes will result in a complete return to normal status. The recovery of cilia was observed on the third day after cessation of adding TNF-alphaand the normalization of secretory cell area was observed on the fifth day. The morphologic changes induced by TNF-alpha were reversible except with the concentration of 100 ng/ml. The results suggest that the ultrastructural changes induced by TNF-alpha are mostly reversible and that this reversibility corresponds with the in vivo state.
Cilia ; Epithelial Cells* ; Humans* ; Tumor Necrosis Factor-alpha*

BACKGROUND: During the past few years, transesophageal echocardiography(TEE) became one of most impartant method to detect various cardiac disease. But it was performed less than 10% of transthoracic echocardiography(TTE) in most of echocardiographic laboratoties because of major and minor complicatios associated with this procedure. The purposes of this study are to evaluate the clinical applications and the usefulness of transesophageal echocardiography in various cardiac disorders. METHOD: From 1990 to 1995, 942 cases were included in this study. All subjects were taken TTE and TEE simultaneously The causes of request, related side effect, cause of failed cases, and the benefit of TEE compared to TTE were analysed. The benefit of TEE were classified into major and minor benefit. Major benefit was defined as to diagnose the disease or to change the therapeautic method by TEE. Minor benefit was defined as to have additional findings by TEE whic could not be enrolled in major benefit. RESULTS: 1) The success rate of TEE was 95.5%(900/942). In 42 failed cases(4.5%), 19 were interrupted procedure earlier, 16 were failed to insert the TEE probe, 4 cases showed arrhythmia, and 3 cases had esophageal stricture or varices. 2) The causes of request were to assess the prosthetic valve function(35.5%), cardiac source of embolism(23.7%), valvular heart disease(22.7%), congenital heart disease(10.2), aortic disease(2.7%), endocarditis(2.3%), cardiac mass(1%), and coronary artery disease(1%), in orders. 3) The benefit of TEE was 77%, congenital heart disease was revealed greatest benefit(97%), but major benefit was highest for the evaluation of cardiac source of emboli(100%). CONCLUSION: TEE was very useful method for the detection of intra-cardiac and aortic abnormalities. TEE is a relatively safe procedure but it is a semi-invasive method and has few complications. So it is wise to apply TEE as adjuvant method of TTE.
Arrhythmias, Cardiac ; Coronary Vessels ; Echocardiography ; Echocardiography, Transesophageal ; Esophageal Stenosis ; Heart ; Heart Defects, Congenital ; Heart Diseases ; Methods ; Varicose Veins