No abstract available.
Child* ; Humans

No abstract available.
Diaphragmatic Eventration* ; Humans ; Infant, Newborn*

No abstract available.

No abstract available.
Hallermann's Syndrome*

Oral administration of antigen has long been used in the induction of immune tolerance in various animal models of autoimmune diseases including rheumatoid arthritis (RA). Alleveation of arthritogenic symptoms has been reported from RA patients who received oral administration of type II collagen (CII) without side effects, however its rather inconsistent therapeutic efficacy and variation among patients calls for more detailed investigation on the mechanism of oral tolerance to be settled as regular treatment for RA. In an attempt to understand the immunogenic processes underpinning tolerance induction by orally administered CII, we analyzed changes in the expression of costimulatory molecules and STAT/SOCS signaling messengers in the mouse model of collagen induced arthritis (CIA). We found thatin the spleen of CIA mice, that has been undergone repeated oral feeding of CII prior to the induction of arthritis, showed increased promortion of CTLA4 expressing lymphocytes than in the spleen of PBS fed control. On the other hand, cells expressing CD28 or ICOS were decreased in the spleen of tolerized mice. Tolerance induction by oral CII administration also enhanced the expression of STAT6 in both RNA and protein level, while not affecting the expression of STAT3. The expression of SOCS3, which hasbeen known to transmit STAT-mediated signals from Th2 type cytokines, remained unchanged in the spleen of tolerized mice. Interestingly transcript of SOCS1, which has been associated with Th1 related pathways, was only visible in the spleen of tolerized but not of control mice, suggesting that as in the case of IL-6 signaling, it may exert a feed back inhibition toward the Th1 type stimulation.
Administration, Oral* ; Animals ; Arthritis* ; Arthritis, Rheumatoid ; Autoimmune Diseases ; Collagen ; Collagen Type II* ; Cytokines ; Hand ; Humans ; Immune Tolerance ; Interleukin-6 ; Lymphocytes ; Mice* ; Models, Animal ; RNA ; Spleen

BACKGROUND: To determine the molecular structure of type II collagen-specific T-cell receptors associated with rheumatoid arthritis (RA). METHODS: We generated CII-specific T-cell lines of 8 RA patient s by prolonged in vitro culture with bovine CII (bCII) and the immunogenic peptide (256-270) of human CII. The proliferation response towards CII stimulation was measured from the uptake of 3 H-thymidine. Changes in the secretion of Th 1 and Th2 cytokines in the culture supernatent were measured by ELISA. The TCR clonotypes of these T-cells were examined by RT-PCR/ SSCP analyses of all 22 V beta chains. RESULTS: T-cells from patients' tissue exhibited strong proliferation index upon CII stimulation, which was maintained up to 6 months in the culture. The secretion of INF-gamma from these T-cells increased along with the duration of culture time, while the amount of IL-4 production did not show significant changes. The SSCP band patterns of patients' T-cells appear as discrete bands unlike the smeary streak produced from normal samples. Some SSCP bands, each representing selected expansion of a TCR containing certain subtype of V beta peptides, appeared to be identical in more than one patients. Among these, the expansion of SSCP band representing the V beta 14 CDR3 region persisted after switching the antigen to the immunogenic human peptide (256-270). CONCLUSION: CII-reactive T-cells expressing distinct CDR3 motifs are selectively expanded in the peripheral blood and synovial fluid of RA patients, and their persistent proliferation upon CII stimulation, as well as the production Th 1-type cytokines, may play pivotal roles in RA pathogenesis.
Arthritis, Rheumatoid* ; Cytokines ; Enzyme-Linked Immunosorbent Assay ; Humans ; Interleukin-4 ; Molecular Structure ; Peptides ; Polymorphism, Single-Stranded Conformational ; Receptors, Antigen, T-Cell ; Synovial Fluid ; T-Lymphocytes*

No abstract available.
Interleukin-4* ; Interleukins* ; Receptors, Interleukin-4* ; Rheumatic Diseases*

No abstract available.

Congenital diverticulum of the left ventricle is a very rare disease. Ventricular diverticulum is usually associated with other anomalies including intracardiac and midline thoracoabdominal defect. We describe a case with congenital left ventricular diverticulum presenting as an isolated lesion. A 37-year-old man presented with 4-year history of chest pain. Diverticulum was diagnosed by echocardiography and left ventriculogram.
Adult* ; Chest Pain ; Diverticulum* ; Echocardiography ; Heart Ventricles ; Humans ; Rare Diseases

BACKGROUND: Aging is associated with increased cardiovascular risk and firmly established as a risk factor for the development of atherosclerosis. However the exact mechanism of age-related damage to the arterial wall and its relation to the atherosclerotic process are not well known.The endothelium plays an important role for the regulation of vascular tone and the endothelial function is impaired in the presence of risk factors early in the process of atherosclerosis. Assessment of endothelial function appears to be a valuable tool for the diagnosing and therapeutic monitoring of coronary artery disease. Anti-oxidants are known to improve endothelial dysfunction in atherosclerosis patients. The aim of this study was, (1) to evaluate the endothelial function in elderly, (2) to investigate whether vitamin C administration has benefit on the endothelial function in elderly. METHODS: The endothelial function was estimated using venous occlusion plethysmography(VOP) in 7 elderly and 7 young healthy volunteers. The strain guage was connected to plethysmograph to record the forearm volume change. A rapid cuff inflator was used to inflate the arm cuff to 40 mmHg instantaneuosly thus occluding venous return from the forearm. The measurement of forearm volume change was repeated for 7 times each stage. The change of the forearm blood flow(FBF) was measured with the acetylcholine infusion through brachial artery and also with intra-arterial vitamin C. RESULTS: Endothelium-dependent vasodilatation was significantly impaired in the elderly group compared to the young group(321 +/-17% in elderly group vs 509 +/-81%, mean+/-SEM) Forearm blood flow response to acetylcholine was significantly enhanced with inraarterial infusion of vitamin C in elderly group(321+/-17% in elderly group vs 78% in vitamin C) Coinfusion of L-NMMA, an inhibitor of nitric oxide synthase, blunted forearm blood flow response to acetylcholine. CONCLUSIONS: Even though the mechanisms leading to drpressed endothelial function in elderly remains to be elucidated, our study shows that vitamin C result in demonstrable improvement by a mechanism that is probably related to antioxidant activity.
Acetylcholine ; Aged* ; Aging ; Arm ; Ascorbic Acid* ; Atherosclerosis ; Brachial Artery ; Coronary Artery Disease ; Endothelium ; Forearm ; Healthy Volunteers ; Humans ; Nitric Oxide Synthase ; omega-N-Methylarginine ; Risk Factors ; Vasodilation* ; Vitamins*