BACKGROUND AND OBJECTIVES: Idiopathic restrictive cardiomyopathy is a very rare and poorly recognized disease in children. This study is performed to describe the clinical course and to define potential predictors of outcome. MATERIAL AND METHOD: We reviewed the medical records and diagnostic studies of 11 consecutive patients during the period from Jan.1991 to Aug. 2000. RESULTS: The age at diagnosis was 1.2-13 years (median 7 years) and the duration of follow up was 3-90 months (median 3.6 years). All except one were symptomatic (dyspnea in ten, chest pain in four). The chest pain was associated with significant ST depression on both resting and exercise ECG, suggesting myocardial ischemia. Two had complete heart block as either initial or terminal event. Cardiac catheterization was done in nine ( mean pulmonary arterial wedge pressure 23+/-6mmHg, systolic pulmonary arterial pressure 47+/-14mmHg, mean right atrial pressure 11+/-9mmHg). Echocardiographic dimensional ratio of left atrium and aorta (LA/Ao) was 2.41+/-0.58. Mitral E/A inflow ratio was 2.72+/-1.42, E wave deceleration time was 93.6+/-44.2ms. During follow up, six died. The 2 year and 5 year cumulative survival rates were 54.5% and 18.8% respectively. The predictor for nonsurvivor were pulmonary venous congestion and LA/Ao >2.5(p<0.05). Verapamil was tried in 6 cases without favorable effect in all. CONCLUSION: Considerable numbers of restrictive cardiomyopathy have myocardial ischemia associated with ST depression and chest pain. The patients with pulmonary venous congestion and severe left atrial enlargement (LA/Ao>2.5) were at risk for death, requiring prompt definitive treatment such as cardiac transplantation.
Aorta ; Arterial Pressure ; Atrial Pressure ; Cardiac Catheterization ; Cardiac Catheters ; Cardiomyopathy, Restrictive* ; Chest Pain ; Child* ; Deceleration ; Depression ; Diagnosis ; Echocardiography ; Electrocardiography ; Follow-Up Studies ; Heart Atria ; Heart Block ; Heart Transplantation ; Humans ; Hyperemia ; Medical Records ; Myocardial Ischemia ; Pulmonary Wedge Pressure ; Survival Rate ; Verapamil

Sixty one cases of gastric adenocarcinoma were studied immunohistochemically for expression of HLA-DR and secretory component(SC) in order to analyze the relationship between expression of these in gastric cancer cells and the adjacent mucosa. Immunostaining was detected within the cytoplasm and on the cell memgrane. The rate of HLA-DR and SC expressions in cancer cells were 59.0% and 49.2%, respectively, and 52.5%/52.5% and 31.2%/50.8% the mucosa in adjacent/remote from the site of to cancer. The SC expression in the adjacent mucosa was lower than that of the remote mucosa(p=0.027). The HLA-DR expression in the cancer cells in the intestinal type of gastric adenocarcinoma(73.9%) was higher than that of the diffuse type(14.3%) and it was statistically significant(p=0.02). The presence of an increased amount of lymphoid infiltration in the gastric mucosa was closely related to the expression of HLA-DR and SC. Decreased or absent expression of SC at the transitional mucosal cells was possibly a result of exposure to genotoxic agents due to the lack of protective function of SC-IgA. From these results, one can postulate that the expression of HLA-DR and SC may play an important role in atleration in microenvironment with lymphoid infiltration.
Adenocarcinoma ; Stomach Neoplasms

No abstract available.
Female ; Mucins* ; Ovary*

No abstract available.
Glomerulonephritis, IGA* ; Immunoglobulin A*

Frey's syndrome is a fairly common sequelae of surgery, trauma and inflammation of the parotid gland. Some patients with Frey's syndrome may be socially compromised by their gustatory sweating and flushing. In the past three decades, a variety of surgical and pharmacological methods have been unsuccessful in alleviating these symptoms. And since misdirection of auriculotemporal secretomotor nerve fibers has been found to play an important role in the development of the syndrome, a study was initiated to attempt mechnical interference with regenerating nerve fibers. Therefore, the recent trend in management of Frey's syndrome has been the use of prophylactic procedures performed at the time of parotidectomy to prevent its symptoms postoperatively. An additional benefit of this approach is prevention of the typical cheek contour deformity after parotidectomy We reviewed our experience with interposition of a vascularized temporoparietal fascial flap between the parotid bed and overlying skin immediately after complete superficial parotidectomies to prevent Frey's syndrome and hollow contour defects. The result of seven consecutive attempts revealed it to be an effective technique, achieving both goals in all patients with minimal morbidity.
Cheek ; Congenital Abnormalities ; Flushing ; Humans ; Inflammation ; Nerve Fibers ; Parotid Gland ; Skin ; Sweating, Gustatory

No abstract available.
Carcinoma, Renal Cell* ; Caveolin 1*

No abstract available.

Teratomas are neoplasm composed of a wide variety of tissues foreign to the organs or anatomic sites in which they arise. They generally arise in gonads but a few cases are described in extragonadal sites, such as sacro-coccygeal region, mediastinum, neck, and retroperitoneum. Most sacrococcygeal teratomas are encountered in infants, particularly in neonates, and a primary sacrococcygeal teratoma is a extremely rare neoplasm in adults. Recently, we experienced two cases of presacral teratomas arising in adult, therefore we present these cases with a brief review of the literatures
Adult* ; Gonads ; Humans ; Infant ; Infant, Newborn ; Mediastinum ; Neck ; Teratoma*

PURPOSE: To correlate histologic subtypes with MR signal intensity in meniagioma and to find etiologic factors responsible for the signal characteristics of T2WI. MATERIALS AND METHODS: We. reviewed MRIs and histopathologic studies in 35 cases of meningioma. MR signal intenisty was measured with respect to cerebral cortex(gray matter) as hypointense, isointense, or hyperintense. Pathologically, meningioma was classified into subtypes, acording to the new WHO classification of brain tumors. The degree of cellularity, collagen, and vascularity was graded from 1 to 3, and presence or absence of psammoma bodies, microcysts, micronecrosis and microhemorrhage was obeserved. Multiple linear regression analysis was done to find relationship between the pathologic findings and MR signal intensity of T2WI. RESULTS: Even in the same subtype, cellularity, collagen and vascularty of the tumor were different. T1WI was not useful in discriminating pathologic subtype because most tumors were isointense or hypointense to the cortex regardless of histologic type. Most tumors showed various signal intensity on T2WI, but T2WI were not useful, either. Exceptionally, all five cases of microcystic meningiomas were hyperintense on T2W1. In analysing the relationship between MR signal intensity and pathologic factor, increased collagen content produced decreased signal intensity(P<0.01) and the existence of microcyst resulted in high signal intensity(P<0.01). Cellularity, vascularity, microcalcification, micronecrosis and microhemorrhage had no relationship with signal intensity on T2WI. CONCLUSION: Except for the five microcystic meningiomas with hyperintenty on T2WI there was no relationship between MR signal intensity and subtype of meningiomas. Pathologic factors influencing T2 signal intensity were microcyst and collagen. Even in the same subtypes of meningiomas, the T2 signal intensity was different. This may be due to different ratio of microcyst and collagen.
Brain Neoplasms ; Classification ; Collagen ; Linear Models ; Magnetic Resonance Imaging* ; Meningioma*

The decrease of bone density after fracture is a well-known phenomenon. Several studies of the changes of bone density after fracture demonstrated bone density decreased not only at the fracture site, b ut also along the whole fractured bone. Being treated with locked intramedullary (IM) nailing, 15 patients with the long bone fractures of lower limbs were examined by Quantitative Computed Tomography (QCT) or Dual-Energy X-ray Bone Densitometer (Lunar DPX-L) a few days after nail removal. In the cases of QCT (5 patients of the tibia shaft fracture), cortical bone density, cortical thickness and geometrical shape (i.e. external antero-posterior and medio-lateral diameters) of the fracture site were compared to those of the contralateral side. On the other hand, in the case of Lunar' (5 patients of the tibia shaft fracture & 5 patients of the femur shaft fracture), only cortical bone density was compared to that of the contralateral side. The results of this study were as follows: 1. When treated by the locked intramedullary nailing of the long bone fractures, the bone density of the tibia & femur shaft fractures measured from the fracture site, proximal and distal sites to mid-fracture decreased. 2. There was a difference in the bone density of the uninjured long bone diaphysis of the tibia & femur shaft fractures. That is, in the case of the tibia shaft fracture, the bone density of the ipsilateral femur diaphysis measured by QCT increased 2 %, and increased 7 % when measured by Lunar. But, in the case of the femur shaft fracture, the bone density of the ipsilateral tibia diaphysis measured by Lunar decreased 4%. 3. In the case of the tibia shaft fracture, the cortical thickness in the fracture site measured by QCT increased 72 %, and external antero-posterior and medio-lateral diameters increased 7 % and 63 %. It seemed to be the formation of peripheral callus due to medullary reaming preceding intramedullary nail insertion. Therefore, this clinical study demonstrated that the mechanical property of the long bone fractures was restored and restricted weight bearing hardly needs to recommended when the nail was removed after the fracture union. 4. The bone densities of the tibia shaft fratures measured by QCT were not different with those measured by Lunar.
Bone Density* ; Bony Callus ; Diaphyses ; Femur ; Fracture Fixation, Intramedullary* ; Fractures, Bone* ; Hand ; Humans ; Lower Extremity ; Tibia ; Weight-Bearing