The paroxysmal kinesigenic choreoathetosis is characterized by choreathetoic, dystonic and/or tonic movements induced by sudden voluntary movements, without loss of consciousness, urinary incontinence and clonic movement. These attacks begin in childhood, and are occasionally familial. The response to anticonvulsants is usually excellent. We present 4 cases of paroxysmal kinesigenic choreoathetosis with a brief review of literature, which is believed to be the first report in Korea.
Anticonvulsants ; Korea ; Unconsciousness ; Urinary Incontinence

PURPOSE: Microdeletion of chromosome 22q11.2 are associated with DiGeorge syndrome(DGS), velocardiofacial syndrome(VCFS) and conotruncal anornaly face syndrome(CTAFS). DGS was originally described as an irnmunodeficiency disorder secondary to impaired T cell production due to thymic aplasia or hypoplasia. But the frequency E: severity of immunodeficiency of other clinical syndromes associated with the chromosome 22qll deletion has not been investigated. This study was undertaken to investigate the frequency and severity of immunodeficiency, the relation- ship of the immunodeficiency to clinical phenotypes, and the change of immunologic status with age in CATCH 22 syndromes patients. METHODS: Sixteen patients with CATCH 22 syndrome with characteristic clinical phenotype and chromosome 22qll deletion were studied. Hurnoral and cellular irnmunities were examined by measuring serurn IgG, IgA, IgM level and by T cell subset through flow cytometry and lymphocyte proliferation test by common T cell mitogens respectively. RESULTS: 69Zo of patients with CATCH 22 syndrome were found to have evidence of immunocompromise. The severity of the immunodeficiency did not correlate with any particular phenotypic features nor was it restricted to patients who were categorized as having DiGeorge syndrome. The severity of immunodeficiency tended to be normalized with age. CONCLUSION: The presence of the immunocompromise is common and its severity cannot be predicted based on the clinical phenotype of CATCH 22 syndrome. Therefore, each child with CATCH 22 syndromes regardless of clinical phenotype should be extensively assessed for earlier detection of subclinical immunodeficiency.
Child ; DiGeorge Syndrome ; Flow Cytometry ; Humans ; Immunoglobulin A ; Immunoglobulin G ; Immunoglobulin M ; Lymphocytes ; Mitogens ; Phenotype ; Ships ; T-Lymphocytes

PURPOSE: To evaluate the MR findings of extra-abdominal fibromatosis and the role of MRI in primary diagnosis MATERIALS AND METHODS: Fifteen cases in of histologically proven extra-abdominal fibromatosis in 13 patients were retrospectively reviewed. T1-weighted and T2-weighted images were obtained in axial, coronal and sagittal planes. Gd-enhancement was performed in 14 cases, and dynamic enhancement studies in two. All lesions were evaluated for mass shape and margin definition. RESULTS: Among the 15 cases, tumors of the buttock accounted for five, and tumor of the thigh for two. In eight cases tumors were intermuscular and in six cases were intramuscular. In ten cases (67 %) the mass extended along the long axis of the body and in 14 of 15 cases (93%) focal infiltration of adjacent structures was visible. The signal intensity of the lesion was in all cases inhomogeneous on both T1 and T2 weighted images. As seen on Gd-DTPA enhanced scans, the masses were inhomogeneously enhanced. In all cases MRI revealed star-shaped linear strands or a band-like low signal area in the mass. These features were not enhanced and were arranged along the long axis of the mass. CONCLUSION: MR findings of extra-abdominal fibromatosis were relatively characteristic and helpful for primary diagnosis of the condition.
Axis, Cervical Vertebra ; Buttocks ; Diagnosis ; Fibroma* ; Gadolinium DTPA ; Humans ; Magnetic Resonance Imaging ; Retrospective Studies ; Thigh

BACKGROUND: The pilots with cardiovascular events have a possibility for a risk of suffering from sudden incapacitation which is closely related to flying safety. The coagulation factors such as fibrinogen, factor VII, and factor VIII are possibly related to cardiovascular events. Several studies for general population have shown that an increase of those coagulation factors with age, a correlation of Factor VII and fibrinogen with BMI, and of fibrinogen with smoking. However, this study is to find out whether pilots' age, body weight, body mass index (BMI) and smoking are related to the baseline measurement of coagulation factor VII, factor VIII and fibrinogen. METHOD: Samples were taken from 21 pilots from Asiana Airlines: 11 smokers and 10 non-smokers. In order to measure the relationship between age, body weight, BMI, and the coagulation factors, Pearson correlation was used in this analysis. Independent two sampled t-test was used to analyze the correlation between smoking and the coagulation factors. RESULTS: Mean age, mean height, mean body weight and mean BMI of pilots were examined: 38 years, 171.81 cm, 70.67 Kg and 23.94 Kg/m(2). Mean fibrinogen, mean factor VII, and mean factor VIII were also obtained: 236.0 mg/dl, 92.93%, and 60.16%. The coagulation factor VII, factor VIII and fibrinogen were not significant related to age, body weight, BMI, smoking of pilots. CONCLUSION: This study has no correlation between age, body weight, BMI, smoking and the coagulation factors because the age of this study does not have pilots with over 60 years old and healthy behaviors (e.g., exercise, smoking, drinking, etc.) of most pilots are relatively well.
Blood Coagulation Factors* ; Body Height* ; Body Mass Index ; Body Weight* ; Cardiovascular Diseases ; Diptera ; Drinking ; Factor VII* ; Factor VIII ; Fibrinogen* ; Humans ; Middle Aged ; Smoke* ; Smoking*

The therapeutic results of pulmonary resection for metastatic bone and soft tissue sarcomas were analyzed. From 1986 to 1996, 14 patients(11 male and 3 female) underwent 15 pulmonary resections for metastatic sarcomas. One(7.1%) patient had 2 thoracotomies for recurrences. The number of metastatic tumors were from one to five. The primary malignant tumors were from bone in 4 and from soft tissues in 10. Mean survival time after thoracotomy was 29.2 months, and Kaplan-Meier's 5-year survival rate from the first metastasectomy was 33.2%. Three patients who had the tumor free interval period over 3 years were alive(mean survival period 52.6 months), whereas eleven patients of the less than 3 years were dead with disease(mean survival period 17.3 months). These results suggested that pulmonary metastasectomy in bone and soft tissue sarcoma may prolong the survival rate.
Bone Neoplasms ; Humans ; Lung Neoplasms ; Male ; Metastasectomy ; Neoplasm Metastasis ; Recurrence ; Sarcoma* ; Survival Rate ; Thoracotomy

Vascular catheters are associated with complications such as infection, thrombosis and stenosis. The embolization of a venous catheter fragment is a rare complication. A 39-year-old woman underwent placement of a totally implantable venous access device for chemotherapy to treat a recurrent liposarcoma of the left thigh. The "pinch-off sign" was noted on a routine chest X-ray but that was ignored. Three-months after implantation of the intravenous access device, the indwelling central catheter was fractured and embolized to the pulmonary trunk. The catheter in the pulmonary trunk was successfully removed through a percutaneous femoral vein approach using a pigtail catheter and goose neck snare.
Thigh/pathology ; Pulmonary Artery/*injuries/surgery ; Liposarcoma/drug therapy ; Humans ; Foreign-Body Migration/*complications/surgery ; Female ; *Equipment Failure ; Embolism/*etiology/surgery ; Catheters, Indwelling/*adverse effects ; Catheterization, Central Venous/*adverse effects/instrumentation ; Adult

PURPOSE: Gastrointestinal stromal tumors (GISTs) are the most common form of mesenchymal tumor of the gastrointestinal tract. Recently, tyrosine kinase inhibitors have improved the treatment of GISTs, and their diagnosis facilitated by immunohistochemical markers. The aim of this paper was to study the clinicopathological features of GISTs of the stomach and determine the accuracy of a new grading system and the prognostic factors. METHODS: Patients with mesenchymal tumors of the stomach, operated on between 1982 and 2004, were identified using medical and pathological files. Immunohistochemical staining for KIT (CD117), CD34, smooth muscle actin (SMA), desmin and s-100 protein were performed, and the diagnoses reviewed. Cases were classified into either the very low, low, intermediate or high risk groups according to National Institutes of Health (NIH) consensus symposium. RESULTS: 78 mesenchymal tumors were reanalyzed, and with the supportive use of immunohistochemical markers, 71 (91%) of the gastrointestinal mesenchymal tumors were shown to be GISTs. The tumors often coexpressed KIT and CD34 (90%) and were variably positive for SMA (18%), s-100 protein (11%) and desmin (23%). With a median follow-up of 73.9 months (range 1~228 months), a recurrence occurred in 10 (14%) patients. Analyses demonstrated that the mitotic index (P<0.001) and tumor size (P<0.001) were significant prognostic factors for survival. The new grading system showed a significant difference between the risk groups and the survival rates (P<0.001). CONCLUSION: Immunohistochemical staining is needed to distinguish GISTs from other mesenchymal tumors. The tumor size and mitotic count are significant prognostic factors for GISTs. The new grading system (2001 NIH) for classifying the 4 risk groups of GISTs, according to the tumor size and the mitotic count, is useful in the evaluation of the tumor behavior.
Actins ; Consensus ; Desmin ; Diagnosis* ; Follow-Up Studies ; Gastrointestinal Stromal Tumors* ; Gastrointestinal Tract ; Humans ; Mitotic Index ; Muscle, Smooth ; National Institutes of Health (U.S.) ; Prognosis* ; Protein-Tyrosine Kinases ; Recurrence ; S100 Proteins ; Stomach* ; Survival Rate

BACKGROUND: Systemic lupus erythematosus(SLE) frequently has cardiovascular complications. Pericardial inflammation and effusion. ventricular dysfunction, valvular disease and coronary atherosclerosis may result in morbidity. While the pathologic findings in SLE have been well described, the antemortem documentation of the cardiac complications has been less frequent than the postmortem findings would support, we therefore sought to study how echocariography may improve the clinical assessment of cardiac involvement in 40 consecutive patients with SLE. SUBJECTS AND METHODS: This study began in 1990 with a prospective design. An appointment was scheduled with all patients whose SLE was diagnosed at the outpatient clinic and during the admission of the internal medicine service at the Kang Nam St Mary's hospital These were 40 female patients, with a mean age of 32+/-11 years(+/-SD). The average duration of disease was 37+/-32months, the average dosage of prednisolone per day was 20+/-17mg. M-mode and 2-D echocardiogram were carried out to the 40 SLE patients. RESULT: Seventeen(43%) of the SLE patients had pericardial effusion. Reduced left ventricular contractility was evident in 8 patients(20%). Abnormality of diastolic dysfunction to be checked by A/E ratio was in 3 patients(8%). No patients had echocardiographic evidence of pulmonary arterial hypertension. Clinical findings of myocardial dysfunction in these patients included dyspnea(30%), jugular venous distension(20%), rales(10%) and S3 gallop(8%). Valvular abnormality of SLE patients included mitral regurgitation in five patients(13%) and aortic regurgitation in two patients(5%). Electrocardiogram showed myocardial ischemia, atrial fibrillation and sick sinus syndrome in 3, 1 and 1 patients respectively. CONCLUSION: In this study, pericardial effusion were the most common echocardiopraphic abnormalities in Patients with SLE. The incidence of valvular and myocardial disease were relatively common.
Ambulatory Care Facilities ; Aortic Valve Insufficiency ; Atrial Fibrillation ; Cardiomyopathies ; Coronary Artery Disease ; Echocardiography* ; Electrocardiography ; Female ; Humans ; Hypertension ; Incidence ; Inflammation ; Internal Medicine ; Lupus Erythematosus, Systemic* ; Mitral Valve Insufficiency ; Myocardial Ischemia ; Pericardial Effusion ; Prednisolone ; Prospective Studies ; Sick Sinus Syndrome ; Ventricular Dysfunction

We present a case of a 19-year-old male with portal vein gas embolism resulting from accidental ingestion of 35% hydrogen peroxide. Hydrogen peroxide is a commonly used germicidal cleansing agent. When it is applied to tissues, catalase causes its rapid molecular decomposition with the release of oxygen bubbles. The patient recovered without sequelae. Hyperbaric oxygenation may be of benefit in patients with respiratory compromise or central nervous system symptoms from gas embolism in hydrogen peroxide ingestion.
Catalase ; Central Nervous System ; Detergents ; Eating* ; Embolism, Air* ; Humans ; Hydrogen Peroxide* ; Hydrogen* ; Hyperbaric Oxygenation ; Male ; Oxygen ; Portal Vein* ; Young Adult

BACKGROUND: Percutaneous transluminal coronary angioplasty(PTCA) is a widely acceptable treatment for ischemic heart disease. Restenosis after successful PTCA, which develops in 20~30% of all patients, remains a serious late complication. This study was to evaluate the efficacy of cilostazol for the prevention of stent restenosis compared with ticlopidine. MATERIALS AND METHOD: Fifty three patients underwent coronary stent implantation were divided in to as group A(n=25) receiving 100mg aspirin and 200mg cilostazol and group B(n=28) receiving 100mg asprine and 500mg ticlopidine from Sep 1998 and Feb 1999 at Pusan Paik Hospital, Inje University. Clinical and laboratory evaluations were preformed at regular interval. RESULT: There were no differences in baseline characteristics between the two groups. Coronary artery restenosis was observed in 5(20.8%) in group A and 8(26%) in group B respectively, which were not statistically significant (p=NS). Minimal luminal diameter was 2.10+/-0.89mm in group A and 1.93+/-0.65mm in group B (p=NS). Two patients in group A had headache, while 6(21.4%) patients of group B developed side effects including thrombocytopenia in 2 patients, skin rash in 2 patents . There was no cardiac death during the follow-up period. CONCLUSION: Aspirin plus cilostazol may be safer and equally antithrombotic regimen compared results to aspirin plus ticolpidine after elective coronary stent implantation.
Aspirin ; Busan ; Coronary Vessels ; Death ; Exanthema ; Follow-Up Studies ; Headache ; Humans ; Myocardial Ischemia ; Phenobarbital ; Stents* ; Thrombocytopenia ; Ticlopidine*