Background The evaluation of the disease of vitamin K deficiency bleed
ing (VKDB). Method 108 reported cases between 1980 and 1990 from Germa
ny. Results VKDB occurs preferentially (90%) in fully breastfed infant
s, males are affected nearly twice as often as females. The peak age i
s four weeks; the majority (79%) of the infants are between three and
seven weeks old. 58% of the patients suffer from intracranial bleeding
, which results in a total mortality rate of 19% and in neurological d
amage in 21%. Generally the VKDB occurred suddenly as no warning signs
were noticed or they were so insignificant as not to be heeded. In,at
least 37% of the patients cholestasis was detected. The Quick value w
as pathologically low in every case. Vitamin K dependent factors were
low and PIVKA was detectable, whereas vitamin K independent hemostatic
parameters were normal or even elevated. The combination of low Quick
value and normal fibrinogen as well as platelet level is a goad diagn
ostic indicator which can be confirmed by administration of vitamin K,
after which the Quick value will rise within 30 minutes. Vitamin K pr
ophylaxis reduces the incidence of VKDB from 5.13 per 100 000 births t
o a tenth of that; single dose oral prophylaxis reduces the risk by a
factor of 3.3 and a single parenteral dose by 14.3. Parenteral prophyl
axis is more effective in patients with hepatobiliary disorders. Patie
nts who suffered VKDB despite having received vitamin K prophylaxis ar
e older at onset (without prophylaxis 32 days, with oral prophylaxis 3
7 days, and with parenteral prophylaxis 63 days) and have less intracr
anial bleeding (35%) than patients who received none (62%). Conclusion
Late form of VKDB is a rare but serious disease which can be prevente
d by VK-prophylaxis.