[ XXXX : clinical observations and therapeutic results].

@entity1 @entity1398
@entity1398 is a rare disease. A family with four affected newborns is described in which the course of the disease aggravated from the first to the third @entity1 . The third @entity1 died from generalized infection. In the mother's serum specific antibodies against neutrophils (anti-NA 1) were detected which reacted with cell suspensions from the father and the other @entity1 . In the fourth @entity1 no neutrophils were present even immediately after birth. In this @entity1 the diaplacental transferred antibodies were detectable in falling concentrations over 8 weeks. The number of neutrophils normalized when the antibodies disappeared. Infection in the newborn was prevented by care in an environment poor in bacteria and by antimicrobial prophylaxis. Repeated exchange transfusions with NA 1-free blood only lowered the antibody titer. Transfusions of white blood cell concentrates had only shortlived effects on the @entity1398 .
