Baehner

Hemophilia in the First Year of Life

Citation:

Baehner R, Strauss H: Hemophilia in the first year of life. New England Journal of Medicine 275(10):524-528, Sept 1966.

Notes:

In hemophilia, bleeding difficults are not usually encountered in the neonatal period. Major bleeding episodes are unusual until the infant becomes a toddler.
More than half of patients with severe hemophilia have a negative family history of bleeding. In 58 of 113 patients with severe disease in this study, no bleeding tendency suggestive of hemophilia was known in the family at the time of birth or even after the diagnosis was established.
The majority of infants (76/107) had only mild or no bleeding after circumcision. Even when the disease was severe, 35 of 61 patients did not hemorrhage significantly.
Easy bruising usually begins to appear between three and nine months.
By one year, more than 40% and by 18 months more than 70% had had a major bleeding event.
Overt bleeding manifestations do not occur in infants without severe disease until a significant trauma takes place.
All infants with a positive family history should undergo testing early in life regardless of the presence or absence of severe bleeding.