[Plasma kallikrein-kinin system in hypercoagulable state (author's transl)].
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Biomedical subjects
Publications and source records attributed to T Kamiya.
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alpha(2)-Plasmin inhibitor (alpha(2)PI) is a recently characterized, fast-reacting plasmin inhibitor in human plasma that appears to play an important role in regulation of in vivo fibrinolysis. We report here a case of complete deficiency of alpha(2)PI in man. The patient, a 25-yr-old Japanese man, had a life-long severe bleeding tendency (hemarthrosis and excessive bleeding after trauma). The following tests were within normal limits: platelet count, bleeding time, thrombin time, prothrombin time, partial thromboplastin time, titers of known clotting factors, platelet glass bead retention, Factor VIII-related antigen, platelet aggregation by ADP, collagen and ristocetin, and clot retraction. Routine liver function tests were also normal. The only abnormal finding was that whole blood clot lysis was extemely rapid and was complete in 4-8 h. The concentration of plasma protease inhibitors, including alpha(2)-macro-globulin, antithrombin III, alpha(1)-antitrypsin, and C1INH, were all normal. The concentration of alpha(2)-PI in the patient's plasma, assayed by immunological methods, was <0.1 mg/100 ml (normal concentration, 6.1+/-0.88 mg/100 ml [mean+/-SE]) and functional assays showed a complete deficiency of alpha(2)PI. Addition of purified alpha(2)PI to the patient's whole blood completely corrected the accelerated fibrinolysis. The patient's parents, four siblings, and four other members of this family were asymptomatic, but the titers of alpha(2)PI in their plasmas were congruent with50% of normal pooled plasma. There were three consanguineous marriages in this family, and the alpha(2)PI deficiency appears to have been inherited as an autosomal recessive trait. We speculate that alpha(2)PI deficiency in this patient has led to uninhibited in vivo fibrinolysis that probably causes the severe hemorrhagic tendency. Thus, this study indicates the important role of alpha(2)PI in hemostasis.
Fourteen patients with SVAS served as subjects of echocardiographical and angiocardiographical studies and the data obtained were compared. There were significant decreases in aortic diameter at the level of ascending aorta compared to those of the aortic leaflets and significant differences of the percentage change of the aorta between the patients with SVAS and the normals. Echocardiography is, therefore, a useful noninvasive method for evaluating SVAS. If the values of percentage change of the aortic diameter obtained by echocardiogram are below -30%, the values of peak systolic pressure gradients across the supravalvular stenotic region should be higher than 20 mmHg. The value of LV M/V under 1.0 suggests that the pressure gradient is lower than 20 mmHg.
A case of atrial septal defect with idiopathic thrombocytopenia was successfully operated with the aid of heart-lung machine. The operation was performed under the administration of platelet rich plasma, fresh blood and adrenocorticosteroids before, during and after the operation. With the aid of these treatments, no severe hemorrhagic diasthesis was encountered.
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About 1,300-fold purification of poly(adenosine diphosphate ribose) polymerase has been achieved from the extract of bovine thymus with a recovery of 10 to 20%. The final preparation has a purity of 99%, and the enzyme is composed of a single peptide with a molecular weight of 130,000. The purified enzyme required NAD+, Mg2+, a thiol compound, DNA, and histones for full activity. Whereas DNA is essential for activation of the enzyme, histones are not. The observed stimulation of the reaction by histones is shown to be due to masking of the inhibitory effect of contaminating denartured DNA in native DNA preparation. The concentration of DNA required for half-maximal enzyme activity (apparent Km for DNA) is proportional to the concentration of enzyme in the reaction mixture. The minimum estimation of the number of nucleotide pairs of DNA required for half-maximal activation of one enzyme molecule is 220 to 240 for bulk of calf thymus DNA, while the value is 10 for a calf thymus DNA fraction, "active DNA," which was separated from the enzyme fraction in a stage of the purification. These results suggest that the enzyme is activated by binding to a specific site on calf thymus DNA. The apparent Km for NAD+ and the maximum velocity of the enzyme are estimated to be 60 micrometer and 0.91 mumolper min per mg, respectively.
Papillary projections along the anterolateral margin of the tongue were observed in fetal and young stages of the striped dolphin, Stenella coeruleoalba. Papillary projections appear in the prenatal period and attain maximum development in the early postnatal period. They almost disappear by weaning. Vestigial eminences remain at the corresponding region, but they completely disappear in the adult. The papillary projections observed differ markedly from the lingual papillae of the general mammalian tongue as they are temporary, localized at the anterolateral margin and large in size. The projections were also present in young individuals of some other dolphins. No taste buds could be seen on the projections in any of the stages of all specimens observed. Such projections may have important mechanical functions during suckling in these mammals.
(1) ECG surveys were performed on 12,523 middle school students. The incidence of A-V block was 0.04% (0.37% in males and 0.43% in females). The incidence of A-V dissociation was 0.11% (0.09% in males and0.13% in females). (2) There was no case of Mobitz type II or complete A-V block in this survey. (3) Sixty-four school students were picked at random from the students with A-V block who had been diagnosed by ECG surveys during the last there years and were given the Master's two-step test. The following four types of response were recognized; Type 1, Type 2, Type 3A, B, C, and Type 4. (4) Elevation of ASLO and antistreptkinase (ASK) were found in 6.4% and 13.5%, respectively. The serum cold agglutinin titer was elevated in four of the 12 students tested. (5) Most students with A-V block or A-V dissociation had no past history of severe disease or no major complaints.