The clinical features of SBS in Japan are comparable to those in the Western literature. However, we have also found some idiosyncratic features in Japanese SBS cases; the mother is more frequently the perpetrator and abusive parents are older than in the United States, possibly reflecting current trends in Japan toward later marriage.
Questionnaires were sent to 1,218 hospitals with more than 200 beds, in order to know the incidence of hemorrhagic disease due to vitamin K deficiency in infancy beyond 2 weeks after birth, during 4 and a half years, i.e. from January 1981 to June 1985. Out of the 534 cases reported, 407 had no obvious reasons for vitamin K deficiency: "idiopathic vitamin K deficiency in infancy". Other 68 cases had bleedingepisodes due to vitamin K deficiency associated with hepatobiliary lesions (e.g.congenital bile duct atresia), chronic diarrhea, long term antibiotic therapy and so on: "secondary vitamin K deficiencyin infancy". The third group consisting of 59 cases was so called "near miss" type, in which hemorrhagic tendency was discovered at the time of mass screening tests for vitamin K deficiency or by chance withoutany clinical hemorrhage. In the idiopatic group, 345 cases (84.8%) developed their bleeding episodes between 21 and 59 days of age, and 368 cases (90.4%) were wholly breast-fed. Intracranial hemorrhage was seen in 338 cases (83.0%) of this group. In most cases of this series (97.3%),no vitamin K was supplemented after birth. Administration of vitamin K is an urgent routine procedure during the first one or two months of life for all newbornbabies, although the incidence of the idiopathic vitamin K deficiency in infancyhas not decreased significantly comparedto the results of the first nation-wide survey (Jan. 1978 - Dec. 1980). This study was sponsored by the Ministry of Health and Welfare of Japan.
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