No abstract available.
Protein-Tyrosine Kinases*

Human stem cells have been isolated from embryos as well as diverse somatic tissues. Much efforts have been made to apply those stem cells for therapeutic purpose. Molecular dissection of "stemness" and differentiation process will provide us a clue to further understanding of stem cell biology. The fate of stem cell is determined in part by internal regulation mediated by transcriptional control. A set of genes related to specific functions might be expressed and suppressed upon external or internal signaling. Therefore, it is necessary to identify the complete gene expression profile that defines the stem cell. Moreover, we should verify the potential of therapeutic stem cells regarding differentiation, specific function, tumorigenecity, and toxicity rigorously before the clinical application. At present we have only limited information on "stemness" and differentiation into specific lineages. In the genome era, we can draw a molecular portrait on these functional and morphologic changes along the differentiation, trans-differentiation as well as de-differentiation using the DNA chip technology. We need to accumulate database for gene expression at various steps of stem cell differentiation, which will enhance our knowledge for research on and medical application of stem cell.
Biology ; Embryonic Structures ; Gene Expression ; Gene Expression Profiling ; Genome ; Genomics ; Humans ; Oligonucleotide Array Sequence Analysis ; Stem Cells* ; Transcriptome

Epithelioid smooth muscle tumor is relatively rare and potentially malignant, especially in retroperitoneum. Distinct criteria for malignancy still have not been clarified in this epithelioid variant arising in retroperitoneum. We report a deceptively benign-appearing epithelioid leiomyosarcoma in a 50-year-old female. She was admitted with abdominal discomfort and dysuria. Abdominal CT showed a well-demarcated, 10 10 cm sized, solid mass in retroperitoneum. Concomitant metastatic lesions were noted in right lung field. Surgical excision of retroperitoneal mass and right lung lobectomy were performed. The retroperitoneal mass showed yellowish-tan, well-delineated and lobulated appearance. Histologically, this tumor was composed of predominantly epithelioid, round to oval cells with distinct clear cytoplasm and slightly atypical nuclei. Mitosis was rare (0~1/50 HPF). Lung lesions were morphologically similar to that of retroperitoneum.
Cytoplasm ; Dysuria ; Female ; Humans ; Leiomyosarcoma* ; Lung ; Middle Aged ; Mitosis ; Smooth Muscle Tumor ; Tomography, X-Ray Computed

PURPOSE: For the early diagnosis of Henoch-Schonlein purpura(HSP) presenting with acute abdominal pain preceding skin rash. METHODS: The clinical, endoscopic and radiological records of 23 cases of HSP, presenting with gastrointestinal symptoms preceding skin rash were reviewed. RESULTS: The intervals from the onset of abdominal pain to the development of the skin rash were one day to 30 days(median five days), most of them were within two weeks. The presenting abdominal symptoms were abdominal pain(23 cases), vomiting(16 cases), hematochezia or melena(eight cases) and hematemesis(three cases). The abnormal endoscopic findings include coalescing erythematous lesions, areas of submucosal hemorrhage and superficial erosions and ulcers. The upper gastro intestinal endoscopy showed the abnormalities in 21 of 23 cases, which were observed in the duodenum(21 cases), the stomach(12 cases) and the esophagus(one case). Duodenitis with hemorrhage and/or erosions in the descending duodenum was the sole endoscopic abnormality in two cases and was the most marked finding in three cases. Sigmoidoscopy showed the abnormalities in six of eight cases. The abdominal ultrasonogram showed abnormalities in 12 of 17 cases, which included small bowel wall thickening(eight cases) and intramural hemorrhage(three cases). Recurrences after three months of symptom free intervals developed in four cases; three of them had persistent nephritis beyond one year. CONCLUSION: The erosive hemorrhagic duodenitis in the descending duodenum in the upper endoscopy and the small bowel wall thickening in the abdominal ultrasonogram can be useful findings in the diagnosis of HSP presenting with acute abdomen.
Abdomen, Acute ; Abdominal Pain* ; Diagnosis ; Duodenitis ; Duodenum ; Early Diagnosis ; Endoscopy ; Exanthema* ; Gastrointestinal Hemorrhage ; Hemorrhage ; Nephritis ; Purpura* ; Recurrence ; Sigmoidoscopy ; Skin* ; Ulcer ; Ultrasonography

No abstract available.

Based on the genetic homology between mouse chromosome 16 and human chromosome 21, experimentally induced trisomy 16 mouse has been considered to serve as a suitable model for human Down syndrome. Mice with trisomy 16 express several phenotypic characteristics of human trisomy 21 syndrome; i.e., intrauterine growth retardation, anarsarca, congenital heart disease, brain abnormality, etc. To elucidate morphogenesis of characteristic craniofacial malformation in human Down syndrome, we studied trisomy 16 mouse fetuses that were produced by crossing karyotypically normal C57BL/6 female ice with males carrying the two Robertsonian translocation chromosome Rb(16.17)/Rb(11.16). We examined a series of trisomy 16 conecptuses and their normal littermate controls from day 14 to day 18 of gestation by gross observation and serial microscopic sections. In addition to smaller size and generalized edema, we observed variable, but definite delay in brain and craniofacial development in trisomy 16 mice. The brain revealed less stratified telencephalon, underdeveloped thalamus and hypothalmus with relatively wide third ventricle, and small rhombencephalon. Craniofacial underdevelopment was characterized by persistent open eye, cochlea with fewer turns, delayed closure of the palate, more simple nasal cavity, etc. The tongue was shorter and convex upward, that were especially prominent at 14 days of gestation. The convex tongue and underdeveloped brain made the cranial base convex upward, and the angle between the cranial base an vertebral axis more obtuse. Small head with increase cephalic index and midfacial hypoplasia appeared to account for brain underdevelopment.
Female ; Male ; Humans ; Mice ; Animals

No abstract available.
Bone Marrow* ; Histiocytes* ; Niemann-Pick Diseases*

We identified differentially expressed genes in RAW264.7 cells in response to single and double ligand treatments (LPS, IFNgamma, 2MA, LPS plus IFNgamma, and LPS plus 2MA). The majority of the regulated transcripts responded additively to dual ligand treatment. However, a significant fraction responded in a non-additive fashion. Several cytokines showing non-additive transcriptional responses to dual ligand treatment also showed non-additive protein production/secretion responses in separately performed experiments. Many of the genes with non-additive responses to LPS plus 2MA showed enhanced responses and encoded pro-inflammatory proteins. LPS plus IFNgamma appeared to induce both non-additive enhancement and non-additive attenuation of gene expression. The affected genes were associated with a variety of biological functions. These experiments reveal both dependent and independent regulatory pathways and point out the specific nature of the regulatory interactions.
Animals ; Cytokines ; Gene Expression* ; Macrophages* ; Mice* ; Signal Transduction

BACKGROUND/AIMS: Combined measurement of perinuclear antineutrophil cytoplasmic autoantibodies (pANCA) and anti-Saccharomyces cereviseae mannan antibodies (ASCA) has recently been suggested as a valuable diagnostic approach to inflammatory bowel disease (IBD) in the pediatric age group. The aim of this study was to test the accuracy of the assay using pANCA and ASCA in diagnosing pediatric ulcerative colitis (UC) and Crohn's disease (CD). METHODS: Sera were collected from 25 patients with IBD (17 with CD, 8 with UC) and 32 healthy controls. The levels of pANCA and ASCA were determined by using a standard indirect immunofluorescence technique on ethanol-fixed granulocytes and an ELISA assay, respectively. RESULTS: In patients with UC, the sensitivity, specificity, and positive predictive value of the pANCA test were 38%, 88%, and 60%, respectively. Such values were not changed significantly in the case of positive pANCA and negative ASCA. The sensitivity, specificity, and positive predictive value of ASCA test in diagnosing CD were 71%, 88%, and 92%, respectively. The combination of pANCA negative and ASCA positive was not significant. CONCLUSIONS: ASCA and pANCA assays are highly disease specific for CD and UC, respectively. These serological tests can assist clinicians in diagnosing and categorizing patients with IBD and may be useful in making therapeutic decisions.
Adolescent ; Antibodies, Antineutrophil Cytoplasmic/*analysis ; Antibodies, Fungal/*analysis ; Child ; Child, Preschool ; Colitis, Ulcerative/*diagnosis ; Crohn Disease/*diagnosis ; Enzyme-Linked Immunosorbent Assay ; Female ; Fluorescent Antibody Technique ; Humans ; Male ; Mannans/immunology ; Predictive Value of Tests ; Saccharomyces cerevisiae/*immunology ; Sensitivity and Specificity