PURPOSE: In colon cancer, the most frequent genetic alteration is found in p53 tumor suppressor gene residing on the short arm of chromosome 17. In order to investigate the significance of wild-type p53, we transfected wild type p53 into human colon cancer cell lines and analysed tbeir biologic effects. MATERIALS AND METHODS: For analysis of p53 status in cell lines, polymerase chain reaction-single stranded confonnation polymorphism (PCR-SSCP), PCR-direct sequencing and Western blot analysis were employed. Transient transfection with liposome-p53 complex was followed by cell biologic assay. RESULTS: We found that twelve of fifteen human colon cancer cell lines showed mutation of p53 by PCR-SSCP method. These results almost corresponded to p53 protein accumulations assessed by Westem blot using PAbl801. After transfection with lipafect- AMINE and wild type p53 complex on p53 mutant type cell line (LS1034), viability was reduced to 17.9%, and invasiveness was reduced to 37.3%. Morphologically, wild type p53 transfected cells showed lumen formation and apoptosis after induction of differentiation by Matrigel. CONCLUSION: Wild type p53 transfection into p53 mutated colon cancer ceil line resulted in restoration of tumor suppressor effect of p53, and this model would be one of the experimental systems for p53-based gene therapy.
Apoptosis ; Arm ; Biological Assay ; Blotting, Western ; Cell Line* ; Chromosomes, Human, Pair 17 ; Colon* ; Colonic Neoplasms* ; Genes, p53* ; Genes, Tumor Suppressor ; Genetic Therapy ; Humans* ; Liposomes ; Transfection*

The accumulation of abnormal protein aggregates is a major characteristic of many neurodegenerative disorders, including Parkinson's disease (PD). The intracytoplasmic deposition of alpha-synuclein aggregates and Lewy bodies, often found in PD and other alpha-synucleinopathies, is thought to be linked to inefficient cellular clearance mechanisms, such as the proteasome and autophagy/lysosome pathways. The accumulation of alpha-synuclein aggregates in neuronal cytoplasm causes numerous autonomous changes in neurons. However, it can also affect the neighboring cells through transcellular transmission of the aggregates. Indeed, a progressive spreading of Lewy pathology among brain regions has been hypothesized from autopsy studies. We tested whether inhibition of the autophagy/lysosome pathway in alpha-synuclein-expressing cells would increase the secretion of alpha-synuclein, subsequently affecting the alpha-synuclein deposition in and viability of neighboring cells. Our results demonstrated that autophagic inhibition, via both pharmacological and genetic methods, led to increased exocytosis of alpha-synuclein. In a mixed culture of alpha-synuclein-expressing donor cells with recipient cells, autophagic inhibition resulted in elevated transcellular alpha-synuclein transmission. This increase in protein transmission coincided with elevated apoptotic cell death in the recipient cells. These results suggest that the inefficient clearance of alpha-synuclein aggregates, which can be caused by reduced autophagic activity, leads to elevated alpha-synuclein exocytosis, thereby promoting alpha-synuclein deposition and cell death in neighboring neurons. This finding provides a potential link between autophagic dysfunction and the progressive spread of Lewy pathology.
Adenine/analogs & derivatives/pharmacology ; Animals ; *Autophagy/drug effects ; Cell Line ; *Exocytosis/drug effects ; Extracellular Space/*metabolism ; Humans ; Mice ; Mice, Knockout ; Microtubule-Associated Proteins/deficiency/metabolism ; Phagosomes/drug effects/metabolism ; Protein Structure, Quaternary ; Protein Transport/drug effects ; alpha-Synuclein/chemistry/*metabolism/secretion/toxicity

PURPOSE: The prevalence of childhood obesity has increased dramatically. It is important to know about life style and dietary habits of the obese children because the treatment of childhood obesity focuses on using behavioral modification techniques. We aimed to develop a questionnaire for the purpose of providing convenient and useful guidance to pediatricians who evaluate and treat obese children. METHODS: Previously developed questionnaire was given to 94 obese children and their parents who had visited clinic for obese children and adolescents. We analyzed response rates on questions and reliability between children and their parents. RESULTS: The response rates on questions were somewhat high. Agreement of paired questions of both parents and children was also moderately high (63~92%). It is acceptable to complete questions by either parents or children alone. Items for hours of playing video games or computer, maternal job, kind of consuming beverage and food outside home were added. CONCLUSION: We concluded that some questions are not needed to be given to both parents and their children. It would be better to have parents record life style of their children and to have children record their food intake with physical activity outside home.
Adolescent ; Beverages ; Child* ; Eating ; Food Habits ; Health Behavior* ; Humans ; Life Style ; Motor Activity ; Obesity ; Parents ; Pediatric Obesity ; Prevalence ; Surveys and Questionnaires* ; Video Games

Pulmonary embolism is a common and lethal, which accounts for substantial morbidity and mortality. Clinical manifestations of pulmonary embolism are nonspecific during general anesthesia. A 60 years old female received elective operation for left femur fracture under general anesthesia. At the end of operation, she suddenly became hypotensive and developed cyanosis. Immediate cardiopulmonary resuscitation(CPR) was performed without definitive diagnosis. Pulmonary embolism was suspected by clinical signs and echocardiography. So, patient was transferred to intensive care unit and with intensive care and aggressive treatment, patient's vital signs and ventilatory status were progressively improved. However, the endotracheal tube was accidentally extubated by the patient at the second postoperative day, and then cardiac arrest was developed and the patient expired. The primary goal of therapy for pulmonary embolization is to prevent reembolization. In the pulmonary thromboembolization, early diagnosis and intensive care improve outcome.
Anesthesia, General* ; Cyanosis ; Diagnosis ; Early Diagnosis ; Echocardiography ; Embolism ; Female ; Femur* ; Heart Arrest ; Humans ; Critical Care ; Intensive Care Units ; Middle Aged ; Mortality ; Pulmonary Embolism* ; Vital Signs

Right-sided varicocele is a relatively rare disease, which could be caused by retroperitoneal pathology, situs Inversus or ananomalous termination of the right internal spermatic vein into the right renal vein or the left renal vein. Herein we report a rare case of right-sided varicocele in a 31-year-old fertile man with situs inversus.
Adult ; Humans ; Pathology ; Rare Diseases ; Renal Veins ; Situs Inversus* ; Varicocele* ; Veins

BACKGROUND: Topical nasal corticosteroid therapy produces clinical improvement in patients with nasal polyposis, but there are lots of controversy about the pathogenesis of nasal polyps and the mode of action of steroid therapy. OBJECTIVES: To examine the mechanism of steroid effect, we investigated the histological change in apex, body and stalk of nasal polyps individually after topical steroid therapy. MATERIALS AND METHODS: Ten patients with nasal polyposis were treated with budesonide nasal spray, 100ng(50ng/puff) twice daily in each nostril for 4 weeks. we quantified the number of inflammatory cell types and compared the histologic types and structures in each portion of nasal polyps from the patients. RESULTS: Administration of topical corticosteroid over 2 weeks caused changes in each portion of nasal polyps histologically. The histologic changes showed decreased edema and increased fibrosis under light microscope and it was evident with longer administration of the topical corticosteroid. The number of plasma cells in body portion was decreased after treatment. Electron microscopy showed that the number of degranulated granules of mast cell was increased in 2 weeks after treatment and then decreased in 4 weeks at pedicle portion of nasal polyps. CONCLUSION: The results of this study suggest that the administration of topical corticosteroid may cause activation of healing process histologically.
Budesonide ; Edema ; Fibrosis ; Humans ; Mast Cells ; Microscopy, Electron ; Nasal Polyps* ; Plasma Cells

Giant cell tumors acount for 4% of all bone tumors and usually occur in the epiphyses of long bones and rarely involve the head and neck region, representing about 9% of them. The incidence of giant cell tumor arising in Paget's disease, which is characterized by a bizarre and greatly exaggerated skeletal turnover, is probably less than 0.2%. We experienced a case of giant cell tumor in the bilateral maxilla with Paget's disease of bone, which was treated by excision and curettage of the tumor mass via endoscopic approach.
Curettage ; Epiphyses ; Giant Cell Tumors* ; Giant Cells* ; Head ; Incidence ; Maxilla* ; Neck ; Osteitis Deformans

Parkinson's disease (PD) and dementia with Lewy bodies (DLB) are characterized by abnormal deposition of alpha-synuclein aggregates in many regions of the central and peripheral nervous systems. Accumulating evidence suggests that the alpha-synuclein pathology initiates in a few discrete regions and spreads to larger areas in the nervous system. Recent pathological studies of PD patients have raised the possibility that the enteric nervous system is one of the initial sites of alpha-synuclein aggregation and propagation. Here, we evaluated the induction and propagation of alpha-synuclein aggregates in the enteric nervous system of the A53T alpha-synuclein transgenic mice after injection of human brain tissue extracts into the gastric walls of the mice. Western analysis of the brain extracts showed that the DLB extract contained detergent-stable alpha-synuclein aggregates, but the normal brain extract did not. Injection of the DLB extract resulted in an increased deposition of alpha-synuclein in the myenteric neurons, in which alpha-synuclein formed punctate aggregates over time up to 4 months. In these mice, inflammatory responses were increased transiently at early time points. None of these changes were observed in the A53T mice injected with saline or the normal brain extract, nor were these found in the wild type mice injected with the DLB extract. These results demonstrate that pathological alpha-synuclein aggregates present in the brain of DLB patient can induce the aggregation of endogenous alpha-synuclein in the myenteric neurons in A53T mice, suggesting the transmission of synucleinopathy lesions in the enteric nervous system.
alpha-Synuclein ; Animals ; Brain ; Dementia ; Enteric Nervous System ; Humans ; Inflammation ; Lewy Bodies ; Mice ; Mice, Transgenic ; Nervous System ; Neurons ; Parkinson Disease ; Peripheral Nervous System ; Tissue Extracts

Huntington disease (HD) is an inherited neurodegenerative disorder characterized by motor and cognitive dysfunction caused by expansion of polyglutamine (polyQ) repeat in exon 1 of huntingtin (HTT). In patients, the number of glutamine residues in polyQ tracts are over 35, and it is correlated with age of onset, severity, and disease progression. Expansion of polyQ increases the propensity for HTT protein aggregation, process known to be implicated in neurodegeneration. These pathological aggregates can be transmitted from neuron to another neuron, and this process may explain the pathological spreading of polyQ aggregates. Here, we developed an in vivo model for studying transmission of polyQ aggregates in a highly quantitative manner in real time. HTT exon 1 with expanded polyQ was fused with either N-terminal or C-terminal fragments of Venus fluorescence protein and expressed in pharyngeal muscles and associated neurons, respectively, of C. elegans. Transmission of polyQ proteins was detected using bimolecular fluorescence complementation (BiFC). Mutant polyQ (Q97) was transmitted much more efficiently than wild type polyQ (Q25) and forms numerous inclusion bodies as well. The transmission of Q97 was gradually increased with aging of animal. The animals with polyQ transmission exhibited degenerative phenotypes, such as nerve degeneration, impaired pharyngeal pumping behavior, and reduced life span. The C. elegans model presented here would be a useful in vivo model system for the study of polyQ aggregate propagation and might be applied to the screening of genetic and chemical modifiers of the propagation.
Age of Onset ; Aging ; Animals ; Complement System Proteins ; Disease Progression ; Exons ; Fluorescence ; Glutamine ; Humans ; Huntington Disease ; Inclusion Bodies ; Mass Screening ; Nerve Degeneration ; Neurodegenerative Diseases ; Neurons ; Pharyngeal Muscles ; Phenotype ; Venus

PURPOSE: We investigated the clinical presentation of febrile pediatric patients with acute pyelonephritis (APN) with a mixed urine culture from an aseptic urine sample, and compared with that of those with a single culture. METHODS: We retrospectively reviewed the medical charts of 95 patients diagnosed as APN with fever between January 2008 and October 2010 at Korea University Medical Center. We classified the patients with APN into two groups with a positive single culture (S group) and a positive mixed culture (M group) from an aseptic urine sample of suprapubic bladder aspiration or urethral catheterization and compared the fever duration, laboratory markers such as serum white blood cell (WBC) counts and C-reactive protein (CRP) values in peripheral blood, and the presence of hydronephrosis, renal scar and vesicoureteral reflux (VUR) between the two groups (If presence of hydronephrosis, scar and VUR=1 and no=0). RESULTS: Total pediatric patients with febrile APN were 95 patients, a positive S group was 89 patients and a positive M group was 6 patients. Fever duration (S vs. M, 4.7+/-3.1 vs. 6+/-5.7 days), serum WBC (S vs. M, 18,630+/-6,483 vs. 20,153+/-7,660/microL) and CRP (S vs. M, 100.6+/-2.46 vs. 81.1+/-0.09 mg/L) values, and the presence of hydronephrosis, renal scar and VUR were not different between the two groups. CONCLUSION: Our data shows that there were no specific differences of clinical manifestation between a positive single urine culture and a positive mixed urine culture in pediatric APN. A mixed urine culture from an aseptic urine sample should be interpreted cautiously.
Academic Medical Centers ; Bacteriuria ; Biomarkers ; C-Reactive Protein ; Cicatrix ; Coinfection ; Fever ; Humans ; Hydronephrosis ; Korea ; Leukocytes ; Pyelonephritis ; Retrospective Studies ; Urinary Bladder ; Urinary Catheterization ; Urinary Catheters ; Vesico-Ureteral Reflux