Primary sclerosing cholangitis is a chronic disease of unknown cause, characterized by inflammation and fibrosis of the biliary tree with diffuse multifocal stricture formation. With increasing knowledge of primary sclerosing cholangitis, it is now recognized that in the setting of inflammatory bowel disease, cholangiocarcinoma is a complication of primary sclerosing cholangitis. We recently experienced a case of 41 year old female patient who had Crohns disease associated with primary sclerosing cholangitis and cholangioearcinoma. We report a case of primary sclerosing cholangitis with cholangiocarcinoma with literature review.
Adult ; Biliary Tract* ; Cholangiocarcinoma* ; Cholangitis, Sclerosing* ; Chronic Disease ; Constriction, Pathologic ; Crohn Disease ; Female ; Fibrosis ; Humans ; Inflammation ; Inflammatory Bowel Diseases ; Pancreas*

PURPOSE: Emotional and behavioral disturbances have been reported in severe short stature children. The purpose of this study was to examine psychosocial problems in children with Growth hormone deficiency(GHD), comparing with their normal height siblings. METHODS: Twenty three children with GHD(17 boys and 6 girls, mean age:13.1+/-2.8 yrs) were included. Their normal height siblings(8 boys and 2 girls, mean age:12.3+/-2.9 yrs) were included as control group. Parents of all participating children completed the Child Behavior Checklist. Patients 10 years and older completed the Youth Self Report. RESULTS: 1)Internalizing problem scores(somatic complaints, anxiety, depression, social incompetence) were higher in GHD compared to control group(49.0+/-15.0 vs 43.2+/-6.2, P<0.05). Externalizing problem scores and social competences were not different between GHD and control group. 2) Total problem scores in GHD were not different by sex. 3)Total problem scores were not different between idiopathic GHD and organic GHD. 4)Attention problems(r=0.45, P<0.05), delinquent behavior(r=0.49, P=0.01) and aggressive behavior(r=0.51, P<0.01) increased by age. 5)Height SDS negatively correlated with social problem(r=-0.47, P<0.05) and delinquent behavior(r=-0.61, P<0.01). Height SDS also negatively correlated with somatic complaints(r=-0.75, P<0.01), anxiety and depression(r=-0.66, P=0.01). CONCLUSION: Internalized problems such as somatic complaints, anxiety and depression were higher in GHD than control group. Behavioral problem scores were increased by age and behavioral problem scores were correlated with height deficit. When we treat GHD children, appropriate medical and psychological counseling should be included.
Adolescent ; Anxiety ; Checklist ; Child Behavior ; Child* ; Counseling ; Depression ; Female ; Growth Hormone* ; Humans ; Parents ; Self Report ; Siblings

To generate drug delivery vector to locales in the body, genetic engineering and expression techniques have been used to produce antibody avidin fusion proteins. Chicken avidin has been fused to mouse-human chimeric IgG3 immediately after the hinge with a flexible linker (H-Flex-Av) and at the end of CH2 (CH2-Av). Fusion heavy chains were expressed with the expected molecular weight, assembled as H2L2 forms with a co-expressed light chain, and were secreted. The expression level of H- Flex-Av was 1~10 ug/ml/10(8)/24 hrs, but that of C2-Av was a very little (0.08~0.9 ug/ ml/10(8)/24 hrs). The resulting H-Flex-Av and CH2-Av fusion proteins continued to bind antigen dansyl and also bound biotinylated bovine serum albumin; both H-Flex-Av and CH2-Av had shown to retain 3-4 times higher relative affinity than that of CH3-Av in ELISA. Importantly the fact that both avidin fusion proteins had a higher relative affinity suggests that these avidin fusion proteins can be effectively used to deliver biotinylated ligands such as drugs and peptides to a certain locale, such as the brain.
Avidin ; Biotin* ; Brain ; Chickens ; Enzyme-Linked Immunosorbent Assay ; Genetic Engineering ; Immunoglobulin G ; Ligands ; Molecular Weight ; Peptides ; Serum Albumin, Bovine

Development of antibody-based cancer therapies will be greatly facilitated if antibodies are better standardized in two fundamental issues that are specificity analysis of antibody reactivity and the detailed biodistribution and pharmacokinetic profile of antibodies. In the current endeavor we attempted to use an antibody binding specificity to target the tumor in a syngeneic carcinoembryonic antigen (CEA) tumor model. CEA, a 180 kDa glycoprotein, expressed at high levels on the surface of nearly all tumors of the gastrointestinal tract was used a potential target for antibody immunotherapy of gastrointestinal carcinomas. Using the CEA model antibody-based cancer therapy directed against CEA has been evaluated in a syngeneic animal model of disseminated disease. We constructed mouse/human chimeric anti-CEA IgG3, which has been evaluated for the specificity for CEA and the detailed biodistribution and pharmacokinetic profiles. Anti-CEA IgG3 heavy chain was expressed with the expected 180kDa molecular weight, assembled as H2L2 forms with a co-expressed mouse/human chimeric anti-CEA light chain, and were secreted. On FACS the purified anti-CEA IgG3 specifically recognized the mouse colon adenocarcinoma cell line MC-38 transduced with CEA (MCA32a), but not MC 38 without expressing CEA. After subcutaneous injection in C57BL/6 mice the half- lives of anti-CEA IgG3 and an irrelevant anti-dansyl IgG3 showed the bi-phasic kinetic patterns, and their pharmacokinetics of the distribution and the elimination were similar in mice. However, the biodistribution patterns of anti-CEA IgG3 were very different from those of anti-dansyl IgG3. Anti-dansyl IgG3 was mainly distributed into kidney until 72 hours, but anti-CEA IgG3 was slowly rernoved from blood and distributed into liver, kidney, spleen, and tumor. It is note worthy that anti- CEA IgG3 increased in targeting MCA32a tumor expressing human CEA by time, but the targeting to MC38 tumor was negligible. Thus, the increased targeting of anti- CEA IgG3 made MCA32a tumor grow slowly
Adenocarcinoma ; Animals ; Antibodies ; Carcinoembryonic Antigen ; Cell Line ; Colon ; Gastrointestinal Tract ; Glycoproteins ; Humans ; Immunoglobulin G* ; Immunotherapy ; Injections, Subcutaneous ; Kidney ; Liver ; Mice ; Models, Animal ; Molecular Weight ; Pharmacokinetics ; Sensitivity and Specificity ; Spleen

Chusid et al proposed diagnostic criteria of hypereosinophilic syndrome (HES) that remain valid today. These were, (1) a sustained peripheral blood eosinophil count of more than 1500/L present for longer than 6 months ; (2) no evidence of other apparent causes for eosinophilia, and (3) presumptive signs of parenchymal organ involvement. Any organ system may be affected in HES, but the most severe clinicopathological involvements are of the heart and nervous system. Although multiple organ systems may be involved, the most common cause of morbidity and mortality is cardiac involvement with extensive fibrous thickening of the endomyocardium and overlying thrombus. We report a case of acute peri-myocarditis with eosinophilia, which was confirmed as eosinophilic myocarditis by endomyocardial biopsy, with literature review.
Biopsy ; Edema* ; Eosinophilia ; Eosinophils* ; Heart ; Hypereosinophilic Syndrome ; Mortality ; Myocarditis* ; Nervous System ; Thrombosis

Chusid et al proposed diagnostic criteria of hypereosinophilic syndrome (HES) that remain valid today. These were, (1) a sustained peripheral blood eosinophil count of more than 1500/L present for longer than 6 months ; (2) no evidence of other apparent causes for eosinophilia, and (3) presumptive signs of parenchymal organ involvement. Any organ system may be affected in HES, but the most severe clinicopathological involvements are of the heart and nervous system. Although multiple organ systems may be involved, the most common cause of morbidity and mortality is cardiac involvement with extensive fibrous thickening of the endomyocardium and overlying thrombus. We report a case of acute peri-myocarditis with eosinophilia, which was confirmed as eosinophilic myocarditis by endomyocardial biopsy, with literature review.
Biopsy ; Edema* ; Eosinophilia ; Eosinophils* ; Heart ; Hypereosinophilic Syndrome ; Mortality ; Myocarditis* ; Nervous System ; Thrombosis

It has been proven that the newborn screening programs for congenital hypothyroidism are very important for its early detection and the early treatment of congenital It has been proven that the newborn screening programs for congenital hypothyroidism are very important for its early detection and the early treatment of congenital hypothyroidism can ameliorate and possibly reverse its consequences. Treatment with L-thyroxine is started in case of suspicious hypothyroidism at the first visit. Serum concentration of total and free thyroxine become normal within 1week after start of therapy but TSH values become normal from 2 months to 6 months after L-thyroxine therapy. The possible explanations for prolonged TSH elevation in congenital hypothyroidism are poor compliance for therapy, an inadequate dose of L-thyroxine, elevated threshold for thyrotropin suppression and two novel mutations in the thyrotropin (TSH) receptor gene in a child with resistance to TSH. Authors have experienced two cases of prolonged TSH elevation with normal T3 and T4 levels till 18months and 27 months of age after optimal L-thyroxine therapy and literature were reviewed.
Child ; Compliance ; Congenital Hypothyroidism* ; Humans ; Hypothyroidism ; Infant, Newborn ; Mass Screening ; Thyrotropin ; Thyroxine

No abstract available.
Body Weight* ; Cholesterol*

No abstract available.
Brain* ; Cerebral Palsy* ; Electroencephalography* ; Evoked Potentials*

We investigated the occurrence of color vision loss in 70 organic solvent mixtures exposed workers and in 47 controls. Color Vision was assessed with a color arrangement test designed to identify the defective color sense, the Han Double 15-Hue Test. The results of the test were no significant difference between exposed workers and controls in the proportion of subjects who committed one or two errors. Quantitative analysis, using color confusion index(CCI), showed no signifiant difference between exposed workers and controls. A significant linear correlation was present between age and CCI in both exposed workers(CCi=0.0056age + 0.94; r=0.23; p<0.05) and controls(CCI=0.0066age + 0.86; r=0.33; p<0.05). Qualitative analysis of the patterns on the hue circle showed that the prevalence of acquired dyschromatopsia was 21% in both and no significant difference. Multiple regression analyses showed that age was significantly related to color vision loss. These results did not provide evidence of a relationship between organic solvents exposure and incidence of color vision loss. In field studies for monitor the people at risk of the acquired color vision loss involving low-dose organic solvents exposed workers, both quantitative and qualitative information should be considered.
Color Vision ; Incidence ; Prevalence ; Solvents