ABSTRACT
A 12-year-old boy developed cholestatic hepatitis with Steven-Johnson syndrome following the use of amoxicillin. The skin lesion and general condition were improved over 2 weeks, but jaundice was gradually aggrevated. We performed liver biopsy, on 30th hospital day, which showed cholestatic hepatitis. The patient improved gradually and liver function was normalized 5 months later.
Subject(s)
Child , Humans , Male , Amoxicillin , Biopsy , Hepatitis , Jaundice , Liver , SkinABSTRACT
Glanzmann's thrombasthenia is a rare autosomal recessive hemorrhagic disorder characterized by prolonged bleeding time, ad deficient or absent clot retraction in the presence of normal platelet count. The major underlying abnormality in this disease is grossly defective first-phase aggregation of platelet, which are unresponsive to ADP or other platelet agonists such as epinephrine, collagen, thrombin in any concentration. This disability is caused by a decrease or absence of the platelet membrans glycoprotein IIb-IIIa complex, a member of the integrin family of adhesive receptors involved in cell-cell and cell-matrix fibronectin, and vitronectin On the development of surface labeling technique, a variety of biochemical techniques such as radioimmunoassay, crossed immunoelectrophoresis and SDS-PAGE have been used to study the structure and the function of platelet membrane glycoproteins, and to detect the platelet functional defect. But all of these techniques demand a relatively large amount of homogeneous paletelet population that requires manipulation through isolation and washing procedures before analysis. In order to eliminaste such an intricate procedure, we have applied method for analyzing platelet surface components in whole blood using monoclonal antibody and flow cytometry to recognize the absence of severe reduction of platelet membrane glycoprotien llb-llla complex. Platelet analysis by flow cytometry is a successful alternative rapid diagnostic technique for Glanzmann's thrombasthenia patients as well as well as for carriers of this disease. Fow cytometry technique provides a sensitive tool for investigating platelet functional defects caused by altered expression or deficiency of platelet surface proteins.
Subject(s)
Humans , Adenosine Diphosphate , Adhesives , Bleeding Time , Blood Platelets , Clot Retraction , Collagen , Electrophoresis, Polyacrylamide Gel , Epinephrine , Fibronectins , Flow Cytometry , Glycoproteins , Hemorrhagic Disorders , Immunoelectrophoresis, Two-Dimensional , Membrane Glycoproteins , Membrane Proteins , Membranes , Platelet Count , Platelet Membrane Glycoproteins , Radioimmunoassay , Thrombasthenia , Thrombin , VitronectinABSTRACT
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.
Subject(s)
Child, Preschool , Humans , Male , Antibody Formation , Eczema , Hemorrhage , Immunity, Cellular , Immunization , Immunoglobulin A , Immunoglobulin E , Immunoglobulin M , Purpura , Skin Tests , Wiskott-Aldrich SyndromeABSTRACT
Direct intraarterial measurement of blood pressure is an important diagnostic procedure in critical patients and premature baby care. Direct measurement was more accepted and confident method in physician than the indirect methods. But the presence of a significant amplification of systolic pressure in peripheral artery may lead to important error. So we have investigated the degree of the systolic pressure amplification from the ascending aorta to the femoral artery in children. Pressure waves recorded in the ascending aorta, aortic arch, diaphragmatic level of the descending aorta, common iliac artery, and femoral artery in 37 patients with congenital heart disease, aged 9 months to 21 years old (mean 7.16 yrs old) during diagnostic cardiac catheterization. The results were as follows 1) Amplification of the systolic pressure increased progressively from the ascending aorta to the femoral artery (16.7%) 2) Diastolic pressure decreased progressively from the ascending aorta to the femoral artery (6.5%) 3) Mean pressure decreased progressively from the ascending aorta to the femoral artery (1.5%) 4) Pulse pressure increased progressively from the ascending aorta to the femoral artery (42.8%) 5) There was significant drop (7%) of the systolic pressure of the ascending aorta compared with that of left ventricle. The results showed that the peripheral amplifications were limited to the systolic and pulse pressure, but the diastolic and mean pressure in peripheral arteries were lower than that of central artery.