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Congenital Hypoprothrombinemia: The First Reported Case in Japan
Author(s) -
Fujimura Yoshihiro,
Nakaue Noriko,
Sugimoto Mitsuhiko,
Matsuyama Ikuko,
Takase Toshio,
Yoshioka Akira,
Fukui Hiromu
Publication year - 1984
Publication title -
pediatrics international
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.49
H-Index - 63
eISSN - 1442-200X
pISSN - 1328-8067
DOI - 10.1111/j.1442-200x.1984.tb01862.x
Subject(s) - hypoprothrombinemia , medicine , prothrombin time , partial thromboplastin time , coagulation , thromboplastin , vitamin k deficiency , pediatrics , gastroenterology , vitamin k
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.