BACKGROUND AND OBJECTIVES: Pulmonary arterial stenosis is a relatively common complication after corrective operation of congenital heart disease. Unilateral stenosis of pulmonary arteries could result in decrease perfusion of affected lung, pulmonary regurgitation, or elevation of right ventricular pressure. Eventually there are increasing risks of right ventricular failure, arrhythmia, or sudden death. However we have limited data of pulmonary arterial stent in paediatric population as the treatment of branch pulmonary stenosis. This study aimed at validating the effectiveness and investigating complications of pulmonary arterial stent implantation in a single institution during mid-term follow up period. SUBJECTS AND METHODS: A total of 42 patients (50 stents) were implanted for treating branch pulmonary arterial stenosis. We used cardiac catheterization for comparing diameter after stent implantation directly and lung perfusion scan indirectly. We also investigated any adverse effect relating the procedure. RESULTS: Percent stenosis of stenotic lesions were decreased from 54.1+/-10.7% to 22.8+/-12.5% (p<0.001) and degree of decrement in affected lung perfusion was declined from 22.7+/-8.0% to 10.3+/-9.0% (p<0.001) immediately and lasts during mid-term follow up period. Complication rate relating the procedure was 12% (6 out of 12) and there was no mortality case. CONCLUSION: This series showed immediate and short term effectiveness of pulmonary arterial stent in congenital heart defects. We concluded that percutaneous transcatheter implantation of pulmonary arterial stent was safe and effective during short and mid-term follow up period.
Arrhythmias, Cardiac ; Cardiac Catheterization ; Cardiac Catheters ; Catheterization ; Constriction, Pathologic ; Death, Sudden ; Follow-Up Studies ; Heart ; Heart Defects, Congenital ; Heart Diseases ; Humans ; Lung ; Perfusion ; Pulmonary Artery ; Pulmonary Valve Insufficiency ; Pulmonary Valve Stenosis ; Stents ; Ventricular Pressure

BACKGROUND: We assessed the intermediate-term result of tricuspid annuloplasty (TAP) for tricuspid valve regurgitation (TR) associated with congenital heart disease in adults. Risk factors for residual TR were also analysed. MATERIAL AND METHOD: From August 1989 to June 2001, seventy three adult patients, 51 females and 22 males, underwent TAP for TR associated with various congenital heart disease. Their age ranged from 16 years to 73 years (mean:43). Associated heart anomalies were atrial septal defect (55), ventricular septal defect (6), partial anomalous pulmonary venous return (4) and others (8). Preoperative and postoperative TR velocities were 3.25 m/sec and 2.56 m/sec respectively, and the types of TAP were De Vega in 43, Kay in 18 and Ring annuloplasty in 12. Postoperative follow-up duration was 2,347 patient-month (mean: 32.6 months), and 134 two-dimensional echocardiographic examinations were done during this period. Residual TR greater than III/IV was considered as TAP failure. RESULT: TAP failure was observed in 7 patients (9.6%), and one patient among them underwent tricuspid valve replacement. Risk factors for TAP failure were diagnosis other than atrial septal defect (p=0.001), preoperative (p=0.038) and postoperative (p=0.028) high TR velocity. There was no statistical significance in terms of TAP methods. CONCLUSION:Careful evaluation of valve morphology and aggressive surgical intervention are mandatory for the repair of TR with preoperative or residual RV pressure overload.
Adult* ; Diagnosis ; Echocardiography ; Female ; Follow-Up Studies ; Heart ; Heart Defects, Congenital* ; Heart Septal Defects, Atrial ; Heart Septal Defects, Ventricular ; Heart Valve Diseases ; Humans ; Male ; Risk Factors ; Scimitar Syndrome ; Tricuspid Valve ; Tricuspid Valve Insufficiency*

Left ventricular inflow obstruction can be caused by a persistent left superior vena cava (SVC) and a dilated coronary sinus. A 31-day-old male infant with secondum atrial septal defect (ASD) and bilateral SVC underwent an operation for treating his uncontrollable congestive heart failure. The preoperative 2-dimensional echocardiography showed a normally sized mitral valve shrouded by a dilated coronary sinus. The operation consisted of pericardial patch closure of the ASD, coronary sinus unroofing and left SVC transfer to the right atrial auricle. The postoperative course was complicated by persistent chylothorax, which was controlled by thoracic duct ligation. He was discharged to home at the postoperative day 39. He has been followed up for 9 months and has displayed normal development.
Chylothorax ; Coronary Sinus* ; Echocardiography ; Heart Failure ; Heart Septal Defects, Atrial ; Humans ; Infant ; Ligation ; Male ; Mitral Valve ; Thoracic Duct ; Vena Cava, Superior*

BACKGROUND: There have been bone mass studies for the treatment of osteoporosis, nonetheless, little attention has been paid to the management of osteopenia. This study was to evaluate the effects of estrogen, alendronate and their combination on bone mineral density and bone metabolism in the postmenopausal women with osteopenia. METHODS: A total of 150 healthy regional patients with osteopenia from Busan were enrolled in prospective randomized clinical trial and randomly assigned to receive conjugated equine estrogen (group I), alendronate (group II), or combination of the two (group III). Assessments included BMD of L2-4 spines and femur neck by DEXA and markers of bone turnover including serum osteocalcin, total alkaline phosphatase and urine deoxydyridinoline (Dpd). BMD and markers of bone turnover were re-evaluated at 6 and 12 months after the treatment. RESULTS: BMD of the lumbar spines increased significantly at 12 months after treatment in the three groups (P<0.05). BMD of the femur neck increased at 12 months after treatment in the three groups, but significantly in group III (P<0.05). Serum osteocalcin decreased at 12 months after treatment in the three groups, but only significantly in group III. Urine Dpd decreased at 12 months after treatment in three groups, but significantly in group, II and III (P<0.05). Serum total alkaline phosphatase decreased at 12 months after treatment in only group III (P<0.05). There was more favorable benefit for group III in BMD of the lumbar spines and serum osteocalcin and urine Dpd at 12 months after treatment compared to group, II and III (P<0.05). CONCLUSION: These results indicated a favorable benefit of conjugated equine estrogen, alendronate, or combination of the two in BMD and important markers of bone turnover. The combined treatment with conjugated equine estrogen and alendronate was more effective in postmenopausal women with osteopenia. Long-term studies are required to confirm these results.
Alendronate ; Alkaline Phosphatase ; Bone Density* ; Bone Diseases, Metabolic* ; Busan ; Estrogens ; Female ; Femur Neck ; Humans ; Metabolism* ; Osteocalcin ; Osteoporosis ; Prospective Studies ; Spine

In adipocytes, insulin stimulates glucose transport primarily by promoting the translocation of GLUT4 to the plasma membrane. Requirements for Ca2+/ calmodulin during insulin-stimulated GLUT4 translocation have been demonstrated; however, the mechanism of action of Ca2+ in this process is unknown. Recently, myosin II, whose function in non-muscle cells is primarily regulated by phosphorylation of its regulatory light chain by the Ca2+/calmodulin-dependent myosin light chain kinase (MLCK), was implicated in insulin-stimulated GLUT4 translocation. The present studies in 3T3- F442A adipocytes demonstrate the novel finding that insulin significantly increases phosphorylation of the myosin II RLC in a Ca2+-dependent manner. In addition, ML-7, a selective inhibitor of MLCK, as well as inhibitors of myosin II, such as blebbistatin and 2,3-butanedione monoxime, block insulin- stimulated GLUT4 translocation and subsequent glucose transport. Our studies suggest that MLCK may be a regulatory target of Ca2+/calmodulin and may play an important role in insulin-stimulated glucose transport in adipocytes.
Protein Transport/drug effects ; Phosphorylation ; Naphthalenes/pharmacology ; Myosin-Light-Chain Kinase/antagonists & inhibitors/*metabolism ; Myosin Type II/*metabolism ; Mice ; Insulin/*pharmacology ; Glucose Transporter Type 4/*metabolism ; Enzyme Inhibitors/pharmacology ; Dose-Response Relationship, Drug ; Calmodulin/antagonists & inhibitors/physiology ; Azepines/pharmacology ; Animals ; Adipocytes/cytology/*drug effects/metabolism ; 3T3 Cells

In patients with colorectal cancer (CRC), the BRAF V600E mutation has been reported to be associated with several clinicopathological features and poor survival. However, the prognostic implications of BRAF V600E mutation and the associated clinicopathological characteristics in CRCs remain controversial. Therefore, we reviewed various clinicopathological features, including BRAF status, in 349 primary CRCs and analyzed the relationship between BRAF status and various clinicopathological factors, including overall survival. Similar to previous studies conducted in Eastern countries, the incidence of the BRAF V600E mutation in the current study was relatively low (5.7%). BRAF-mutated CRC exhibits distinct clinicopathological features from wild-type BRAF-expressing cancer independent of the microsatellite instability (MSI) status. This mutation was significantly associated with a proximal tumor location (P = 0.002); mucinous, signet ring cell, and serrated tumor components (P < 0.001, P = 0.003, and P = 0.008, respectively); lymphovascular invasion (P = 0.004); a peritumoral lymphoid reaction (P = 0.009); tumor budding (P = 0.046); and peritoneal seeding (P = 0.012). In conclusion, the incidence of the BRAF V600E mutation was relatively low in this study. BRAF-mutated CRCs exhibited some clinicopathological features which were also frequently observed in MSI-H CRCs, such as a proximal location; mucinous, signet ring cell, and serrated components; and marked peritumoral lymphoid reactions.
Colorectal Neoplasms* ; Humans ; Incidence ; Microsatellite Instability* ; Microsatellite Repeats* ; Mucins

Targeted therapies guided by molecular diagnostics have become a standard treatment of lung cancer. Epidermal growth factor receptor (EGFR) mutations and anaplastic lymphoma kinase (ALK) rearrangements are currently used as the best predictive biomarkers for EGFR tyrosine kinase inhibitors and ALK inhibitors, respectively. Besides EGFR and ALK, the list of druggable genetic alterations has been growing, including ROS1 rearrangements, RET rearrangements, and MET alterations. In this situation, pathologists should carefully manage clinical samples for molecular testing and should do their best to quickly and accurately identify patients who will benefit from precision therapeutics. Here, we grouped molecular biomarkers of lung cancers into three categories—mutations, gene rearrangements, and amplifications—and propose expanded guidelines on molecular testing of lung cancers.
Biomarkers ; Gene Rearrangement ; Humans ; Lung Neoplasms* ; Lung* ; Lymphoma ; Pathology, Molecular ; Phosphotransferases ; Precision Medicine ; Protein-Tyrosine Kinases ; Receptor, Epidermal Growth Factor

This study was carried out to investigate the relationship between brain magnetic resonance imaging (MRI) and neurological findings in welders exposed to manganese fume. Thirty five welders from a bus manufacturing factory who were occupationally exposed to low dose manganese fume were examined. We collected the information on the demo graphic factors and welding careers. Laboratory investigations included blood and urine manganese concentration and blood chemistry. Brain MRI was checked, and high signal intensity of the globus pallidus was graded from 0 to 3 compared to the white matter of the frontal lobe. Neurologic examination wart carried out. The results were as follows ; 1. The mean age of workers was 48.3+/-5.7 years, mean duration of welding was 21.4 +/-5.6 Years. 2. On brain MRI, high signal intensity in the globes pallidus was observed in 27 workers (77.1%) Among them, 18 cases (66.7%) showed remarkably high signal intensity (grade 2) Mean concentration of blood manganese was higher in those with high signal intensity and correlated to the grade of high signal intensity (p=0.02) 3. On neurologic examination, no workers showed the finding of typical parkinsonism. But 11 workers (31.4%) showed subtle neurological abnormalities such as gait without associated arm movement, postural instability, intention tremor, and so on. Blood manganese concentration of neurologically abnormal group was 2.4+/-0.7 microgram/dL, which was significantly higher, compared to 1.8+/-0.7 microgram/dL of neurologically normal group (p=0.03). 4. Among 27 workers who showed high signal intensity on brain MRI, 10 workers (37%) were neurologically abnormal. Higher grade of signal intensity on globus pallidus showed higher rate of neurologic abnormality (p<0.05) Especially postural instability and gait without associated arm movement were prominent findings. These bindings suggest that high signal intensity on brain MRI might be associated with a prodromal sign of manganese intoxication. Careful neurologic examination should be tool lowed up on welders showing high signal intensity in the golbus pallidus of brain MRI.
Arm ; Brain* ; Chemistry ; Frontal Lobe ; Gait ; Globus Pallidus ; Magnetic Resonance Imaging* ; Manganese* ; Neurologic Examination ; Neurologic Manifestations* ; Occupations ; Parkinsonian Disorders ; Prodromal Symptoms ; Tremor ; Warts ; Welding

Bone is a complex tissue in which resorption and formation continue throughout life. The bone tissue contains various types of cells, of which the bone forming osteoblasts and bone resorbing osteoclasts are mainly responsible for bone remodeling. Periodontal disease represents example of abnormal bone remodeling. Osteoclasts are multinucleated cells present only in bone. It is believed that osteoclast progenitors are hematopoietic origin, and they are recruited from hematopoietic tissues such as bone marrow and circulating blood to bone. Cells present in the osteoclast microenvironment include marrow stromal cells, osteoblasts, macrophages, T-lymphocytes, and marrow cells. These cells produce cytokines that can affect osteoclast formation. In vitro model systems using bone marrow cultures have demonstrated that IL-1 beta, IL-3, TNF-alpha, bFGF can stimulate the formation of osteoclasts. In contrast, IL-4 inhibits osteoclast formation. Knowledge of cytokines and bFGF that affect osteoclast formation and their capacity to modulate the bone-resorbing process should provide critical insights into normal calcium homeostasis and disorders of bone turnover such as periodontal disease, osteoporosis and Paget's disease.
Bone and Bones ; Bone Marrow* ; Bone Remodeling ; Calcium ; Cytokines* ; Fibroblast Growth Factor 2 ; Homeostasis ; Interleukin-1beta ; Interleukin-3 ; Interleukin-4 ; Macrophages ; Osteoblasts ; Osteoclasts* ; Osteoporosis ; Periodontal Diseases ; Stromal Cells ; T-Lymphocytes ; Tumor Necrosis Factor-alpha

Transition metal ions including Se2+, Cd2+, Hg2+ or Mn2+ have been thought to disturb the bone metabolism directly. However, the mechanism for the bone lesion is unknown. In this study, we demonstrated that MC3T3E1 osteoblasts, exposed to various transition metal ions; selenium, cadmium, mercury or manganese, generated massive amounts of reactive oxygen species (ROS). The released ROS were completely quenched by free radical scavengers-N-acetyl cysteine (NAC), reduced glutathione (GSH), or superoxide dismutase (SOD). First, we have observed that selenium (10 micrometer), cadmium (100 micrometer), mercury (100 micrometer) or manganese (1 mM) treatment induced apoptotic phenomena like DNA fragmentation, chromatin condensation and caspase-3-like cysteine protease activation in MC3T3E1 osteoblasts. Concomitant treatment of antioxidant; N-acetyl-L-cysteine (NAC), reduced-form glutathione (GSH), or superoxide dismutase (SOD), prevented apoptosis induced by each of the transition metal ions. Catalase or dimethylsulfoxide (DMSO) has less potent inhibitory effect on the apoptosis, compared with NAC, GSH or SOD. In line with the results, nitroblue tetrazolium (NBT) stain shows that each of the transition metals is a potent source of free radicals in MC3T3E1 osteoblast. Our data show that oxidative damage is associated with the induction of apoptosis in MC3T3E1 osteoblasts following Se2+, Cd2+, Hg2+ or Mn2+ treatment.
Acetylcysteine ; Apoptosis* ; Cadmium ; Catalase ; Chromatin ; Cysteine ; Cysteine Proteases ; Dimethyl Sulfoxide ; DNA Fragmentation ; Free Radicals ; Glutathione ; Ions ; Manganese ; Metabolism ; Metals ; Nitroblue Tetrazolium ; Osteoblasts* ; Reactive Oxygen Species ; Selenium ; Superoxide Dismutase