No abstract available.
Leukemia, Myelogenous, Chronic, BCR-ABL Positive*

A case of neonatal diabetes mellitus is described. The child presented with low birth weight but was normal in appearance. She was acidotic and ketonuria was observed. The HLA typing was DR1 and 3, and insulin autoantibodies were negative. Genetic analysis with polymorphic DNA markers for chromosome 6 indicated biparental inheritance. She required insulin therapy for the control of hyperglycemia, and insulin dependence continues after 8 months of age.
Autoantibodies ; Child ; Chromosomes, Human, Pair 6 ; Diabetes Mellitus* ; Genetic Markers ; Histocompatibility Testing ; Humans ; Hyperglycemia ; Infant, Low Birth Weight ; Infant, Newborn ; Insulin ; Ketosis ; Wills

Objective: Vasospasm remains the leading cause of cerebral damage after aneurysmal subarachnoid hemorrhage. Although magnesium regulates the calcium influx in vascular smooth muscle and endothelial cells, it has not been reported whether L-type calcium channels are involved in magnesiuminduced vascular relaxation in rat basilar artery. So, the effect of magnesium sulfate on L-type calcium currents in freshly isolated smooth muscle cells from rat basilar artery was investigated. Methods: The smooth muscle cells were isolated from rabbit basilar artery by enzyme treatment. L-type Ca2+ currents were identified using cesium chloride, a potassium channel blocker and Bay K8644, an activator of L-type Ca2+ channel. Currents were recorded under step pulse whole cell patch clamp technique. Results: In the presence of cesium chloride (in pipette solution), inward currents were observed by depolarizing step pulses. The inward currents were significantly reduced by nimodipine (n=4, p<0.05), an L-type Ca2+ channel blocker and increased by Bay K8644 (n=5, p<0.05), an L-type Ca2+ channel activator. The L-type calcium currents (156±17.0 pA, n=12) were significantly reduced by the application of 5 mM magnesium sulfate (53.8±7.0 pA, n=12, p<0.01). Conclusion: These results suggest that magnesium may relax cerebral vessel of rat basilar artery through decreasing intracellular Ca2+ ion by inhibition of L-type Ca2+ channels.

This study was performed to evaluate the changes of serum IgG4 level in children with allergic diseases. Serum Ige, IgG and IgG4 level were measured using enyzme immunoassay (IgE) and radial immunodiffusion method (IgG and IgG4) in 21 children with bronchial asthma, allergic rhinitis or urticaria Eosinophil counts in peripheral blood and the values of serum IgE were significantly increased in allergic patients. The values of IgG4 in allergic patients were also increased compare to those in controls (24.23mg/dl versus 20.33mg/dl). But the values of IgG4 were not significantly correlated to either IgG or IgE levels. Further studies will be needed for measuring allergen specifie IgE and IgG4 levels considering the onset of diseases, methods and duration of treatment.
Asthma ; Child ; Eosinophils ; Humans ; Hypersensitivity ; Immunoassay ; Immunodiffusion ; Immunoglobulin E ; Immunoglobulin G* ; Rhinitis ; Urticaria

Kawasaki disease is an acute vasculitis of infancy and early childhood characterized by high fever, rash, mucositis, lymphadenopathy and coronary artery damage. The failure to indentify a causative organism using convetional culture and serological techniques, and the lack of response to antibiotics indicate that the disorder is probably not due to any known bacterial or viral pathogens. During the acute phase of the disease, the alterations of T and B cell functions, changes of cytokine and immunoglobulin levels have been reported. This study was performed to investigate the changes of immunoglobulins levels in patients with Kawasaki disease. IgG, IgA, IgM and IgG subclasses were measured using immunoprecipitation and EIA in the sera of patients with Kawasaki disease. The results were as follows: 1) Acute phase reactants such as CRP and ESR were significantly increased in Kawasaki patients compared to those in control patients(p<0.01). 2) Serum IgG levels in Kawasaki disease were markedly increased than those in control patients, while serum IgA and IgM levels showed no significant changes (P: No Significance). 3) IgG1 and IgG4 were predominantly increased increased in the sera of Kawasaki patients, while IgG2 and IgG3 were not significantly increased (P: No Significance). With these results, unidentified infectious organism with abnormal immune response could be suggested as an etiologic factor of Kawasaki disease.
Acute-Phase Proteins ; Anti-Bacterial Agents ; Child* ; Coronary Vessels ; Exanthema ; Fever ; Humans ; Immunoglobulin A ; Immunoglobulin G* ; Immunoglobulin M ; Immunoglobulins ; Immunoprecipitation ; Lymphatic Diseases ; Mucocutaneous Lymph Node Syndrome* ; Mucositis ; Vasculitis

Wiskott-Aldrich Syndrome is an X-linked disorder characterized by recurrent infection, thrombocypenia and eczema. Various defects in cell-mediated immunity and deficient antibody reponse to carbohydrate antigens have been described. We experienced a case of Wiskott-Aldrich Syndrome of 28 months old male patient. He has been suffered from multiple petechiae with bleeding, recurrent pyogenic infections and generalized eczema since 3 months of age. Immunological abnormalities are as follows: 1) Serum IgM was gradually decreased, while IgA and IgE were increased. 2) Antibody response against polysaccharide antigen (PRP) was not observed after 3 times of PRPT immunization. 3) CD4/CD8 ratio was reversed (0.6). 4) Proliferative response of mononuclear cells was significantly reduced, and CMI skin test also showed negative results. A brief review of literature was made.
Antibody Formation ; Child, Preschool ; Eczema ; Hemorrhage ; Humans ; Immunity, Cellular ; Immunization ; Immunoglobulin A ; Immunoglobulin E ; Immunoglobulin M ; Male ; Purpura ; Skin Tests ; Wiskott-Aldrich Syndrome*

No abstract available.
Cardiac Catheterization* ; Cardiac Catheters* ; Cardiovascular Diseases* ; Child* ; Cineangiography* ; Heart Defects, Congenital ; Humans

The purine antimetabolite 6-Mercaptopurine (6-MP) has been in clinical use for over 30 years and is still a widely used agent in the treatment of childhood acute lymphoblastic leukemia. The bioavailibility, clinical efficacy and toxicity of 6-MP administered orally for maintenance therapy of children with acute lymphoblastic leukemia are highly variable in many studies, as well as at differnt times in same patient. there are many factors affecting the bioavailibility of 6-MP. The most notably factor being that concomitantly administered drugs and foods might contribute to a decrease in the bioavailibity of this drug. In our sociocultural environment milk is a major constituent of child's foods. Cow's milk contains a high concentration of xanthine oxidase, which could potentially transform 6-TM into 6-thioxanthine (6-TX) and 6-thiouric acid (6-TUA) which have no more therapeutic effects. In this study, we evaluated the effect of various milk products on the bioavailability of 6-MP. Incubation at 37degrees C for 30 min raw or pasteurized milk resulted in transformation of a large quantity of clinically relevant concentration of 6-MP into 6-TUA. The concomitant adminstration of folic acid and allopurinol has markedly inhibitory effect on the 6-MP destroying activity of milk at clinically relevant concentrations. These observations may help to optimize modalities of administration of 6-MP for the treartment of patients with childhood leukemia.
6-Mercaptopurine* ; Allopurinol ; Biological Availability* ; Child ; Complement Factor B ; Folic Acid ; Humans ; Leukemia ; Milk* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Xanthine Oxidase

This study was performed to characterize the humoral immune responses with isotype profiles in Mycobacterium tuberculosis infection. PPD-Specific IgG and IgG subclasses were measured using ELISA in 212 patients with pulmonary tuberculosis. The values of PPD-specific IgG were significantly higher in pulmonary tuberculosis patients than those in the control group, and were correlated to the severity of illness (P < 0.01). The specificity and sensitivity of ELISA for IgG antibodies were 1.0 and 0.81, respectively as determined in 212 sera from tuberculosis patients and 44 from healthy controls. The positive predictive value was 1.0 (171/171), while negative predictive value was 0.52 (44/85). The values of PPD-specific IgG were significantly decreased after 2-4 months of treatment. Among the moderately and far advanced pulmonary tuberculosis patients, the values of PPD-specific IgG were significantly decreased in responders after 6 months of treatment. However, PPD-specific IgG in nonresponders was increased (P < 0.01). PPD-specific IgG subclass responses were evident to all four IgG subclasses. No changes of isotype response according to the severity of the disease were observed.
Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Enzyme-Linked Immunosorbent Assay ; Female ; Humans ; Immunoglobulin G/*blood/classification ; Infant ; Infant, Newborn ; Male ; Middle Aged ; Tuberculin/*immunology ; Tuberculosis, Pulmonary/*immunology

No abstract available.
Antibody Formation* ; Chickenpox*