Toshio Takase

Congenital Deficiency of α2-Plasmin Inhibitor in Three Sisters

3 young Japanese sisters with congenital α2-plasmin inhibitor (α2-PI) deficiency are reported. They have mild umbilical bleeding and/or repeated prolonged bleeding after minor tr

Studies of von Willebrand factor: effects of different kinds of carbohydrate oxidases, SH-inhibitors and some other chemical reagents.

The effects of several kinds of carbohydrate oxidase, SH‐inhibitors and some other chemical reagents on the activities of von Willebrand factor, factor VIII procoagulant and factor VIII‐related antigen were studied.

Factor VIII Procoagulant Activity, Factor VIII Related Antigen and von Willebrand Factor in Newborn Cord Blood

Summary. The mean level of factor VIII procoagulant activity (VIII:C) and factor VIII related antigen (VIIIR:AG) was normal in 100 newborn cord plasmas, whereas that of von Willebrand factor (VIIIR:WF) activity was slightly lower than normal. On crossed immunoelectrophoresis, 20 of 50 newborn infants had an increased anodal mobility of VIIIR:AG. When the cord plasma showing an abnormal electrophoretic pattern was mixed with normal plasma, two precipitation peaks with a broad base were found. Similar mixing experiments with the abnormal cord plasma and plasma from a patient with atypical von Willebrands disease did not normalize the electrophoretic mobility of VIIIR:AG. Gel filtration of the cord plasma with an abnormal electrophoretic pattern of VIIIR:AG, showed that the three activities were all detected at the position corresponding to a molecular weight of about 800 000. The results suggest the presence of qualitative abnormalities of the factor VIII molecule in half of full‐term newborn cord plasma.

Patterns of Factor-VIII Related Antigen on Crossed Immunoelectrophoresis and Large Pore Polyacrylamide Gel-Crossed Immunoelectrophoresis in von Wilkbrand's Disease

Plasma samples from patients with various types of von Willebrands disease were subdivided into six patterns according to the electrophoretic mobility and shape of VIIIR: Ag on crossed immunoelectrophoresis (CIE): pattern 1 no precipitation arc, pattern 2 normal mobility with low arc, pattern 3 intermediate mobility with low arc, pattern 4 faster anodal mobility with low arc, pattern 5 normal mobility with normal arc height, pattern 6 faster anodal mobility with normal arc height. Of 62 patients, 14 had pattern 1, 6 pattern 2, 16 pattern 3, 12 pattern 4, 9 pattern 5, and 5 pattern 6.

Type IIB von Willebrand's Disease: Report on the First Case in Japan

Three members of a family are reported with type IIB von Willebrands disease (vWD), characterized by prolonged bleeding time, increased ristocetin‐induced platelet aggregation, fast anodal migration of factor VIII‐related antigen on crossed immunoelectrophoresis and an absence in plasma and presence in platelets of the larger multimers of factor VIII/von Willebrand factor (FVIII/vWF). Binding ability of the patients FVIII/vWF to washed normal platelets is significantly increased in the presence of low concentrations of ristocetin, whereas binding ability of the patients platelets to normal FVIII/vWF is not increased. These later findings are contrary to those in patients with pseudo‐vWD or platelet type vWD. The administration of DDAVP gives a shortening of bleeding time and an increase in plasma levels of factor VIII‐related activities. In addition, there is a transitory appearance of the larger multimers and a moderate diminution of the platelet count.

Transient immunoglobulin-complexed aspartate aminotransferase in infancy caused by maternal immunoglobulin G

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Congenital hypoprothrombinemia : The first reported case in Japan

A Japanese boy with congenital hypoprothrombinemia, who was discovered in the newborn period when umbilical bleeding occurred, is described. On the 6th day after birth, the patient was found to have a markedly low thrombotest and prolonged prothrombin time and partial thromboplastin time. The administration of vitamin K failed to correct the coagulation defects, and the specific coagulation and immnological assay disclosed an extreme decrease of prothrombin activity and antigen. A slight increase of prothrombin activity and antigen in the patient was observed during the first year of life. The half‐lives of prothrombin activity and antigen were both10–11 hrs in the first phase, and32–33 hrs in the second phase. The inheritance mode of the disease seemed to be an autosomal recessive from the test results of the family members.