P0181 ORAL VITAMIN K PROPHYLAXIS DOES NOT PREVENT LATE HEMORRHAGIC DISEASE OF THE NEWBORN IN PIZZ ALPHA-1 ANTITRYPSIN DEFICIENCY.

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Introduction: Late hemorrhagic disease (LHD) of the newborn is defined as a bleeding episode due to vitamin (vit) K deficiency which occurs between 2–12 weeks of age. It is seen in exclusively breastfed infants who received inadequate vit K prophylaxis and in babies with intestinal malabsorption. Intramuscular (IM) administration of a single dose of 1 mg vit K has been shown to prevent LHD. The reported association between IM vit K administration and childhood cancer has caused worldwide concern. Although still unconfirmed, it prompted the change to schedules of repeated oral doses of vit K. Until now there’s no uniform policy and different regimens are used. The Dutch protocol, providing a daily oral dose of 25 microgram vit K following an initial oral dose of 1 mg immediately after birth, has been shown to approach the effectiveness of IM vit K. Methods: We report on 2, exclusively breastfed, 5 week old babies (1 boy, 1 girl), who presented with acute bleeding after correct oral vit K following the Dutch protocol. Results: The girl presented with spontaneous bruising and epistaxis. She was found to have severe coagulopathy (PT<10%, aPTT>180 sec, fibrinogen 305 mg/dl, platelets 701 109/l) and a subcutaneous hemorrhage of the thoracic wall was diagnosed on CT scan. Mild cholestasis (total bilirubin 3.5 mg/dl, direct bilirubin 2.76 mg/dl) was also noted, AST was 80 IU/l, GGT 288 IU/l. The boy was admitted with shock and respiratory distress. Blood results showed a PT<10%, aPTT>180 sec, fibrinogen 326 mg/dl, platelets 404 109/l. Total bilirubin was 6.3 mg/dl, direct bilirubin 3.6 mg/dl, AST 184 IU/l and GGT 988 IU/l. CT scan revealed a hemothorax. The vit K dependent coagulation factors were reduced in both babies. Both infants were treated with intravenous vit K and fresh frozen plasma. The coagulation normalised within hours. Further investigations led to the diagnosis of PiZZ alpha-1 antitrypsin deficiency (AAT) in both children. Conclusion: LHD of the newborn can be the first life threatening symptom of undiagnosed cholestatic liver disease, with AAT and biliary atresia as most common causes. Vit K is essential in the prevention of LHD. Despite correct oral vit K administration following the Dutch regimen, our patients developed LHD. Our limited experience indicates that oral vit K prophylaxis may be inadequate to prevent bleeding diathesis in children with liver disease. LHD is unpredictable and warning signs are often unrecognized. Therefore developing screening methods identifying patients at risk is a challenge for the future, meanwhile however it might be safer to prefer parenteral vit K prophylaxis.