Introduction 26 23 13 22 16 1 1 1 1 29 2 7 10 11 Therefore, a one-year surveillance was started again to study the current incidence and the aetiology of late VKDB in the Netherlands. Discussion will rise about the efficacy of the current guideline in order to completely prevent late VKDB. Methods 8 17 The paediatricians were asked to report all infants in whom bleeding may have resulted from VKDB. The reported cases were validated with a questionnaire asking for information about the infant, type of feeding, clinical presentation, dose of vitamin K prophylaxis, route of vitamin K prophylaxis, associated diseases and laboratory data. For all reported cases, we asked for an anonymous hospital discharge letter to verify the diagnosis of late-onset VKDB. 23 Platelet count normal or raised and normal fibrinogen Prothrombin assay returned to normal after vitamin K administration Concentration of proteins induced by vitamin K absence (PIVKA) exceeded normal controls 20 Idiopathic cases were defined as cases in whom no factor predisposing to vitamin K deficiency was identified. Secondary cases were those in whom an underlying condition such as cholestasis or other causes of malabsorption of vitamin K could be identified. 1 1 The Konakion mixed micellar preparate (Phytomenadion 10 mg/ml, Hoffmann-La Roche, Basel, Switzerland), which can be used for both oral, intramuscular and intravenous administration, an oral preparate called Davitamon K (marketed by Chefaro, Rotterdam, the Netherlands, 25 μg/5gtt) and an oral preparate, Phytomenadion, which is made by the Dutch pharmacist Mixtura (Phytomenadion 10 mg/ml FNA) were the only vitamin K preparations licensed in the Netherlands. P Results The response rate to the surveillance amounted to 93.4% and the response to our questionnaires was 100%. In total, seven cases of late VKDB were reported: four reports from academic paediatric centres and three from general district hospitals. A hospital discharge letter was obtained in six cases. In all cases, the diagnosis could be verified by the completed questionnaire or the additional discharge letter. One case did not fulfil the criteria of the case definition because there were no clinical signs of bleeding, only biochemical clotting disorders due to a vitamin K deficiency. 1 Table 1 Confirmed cases of late vitamin K deficiency bleeding (VKDB) in the Netherlands from 1 January to 31 December 2005 Patient; sex; age (weeks) Presentation Feeding Vitamin K prophylaxis Prothrombin assay (after vitamin K administration)  Additional morbidity Outcome of bleeding A; female; 5 + 1/7 Intracranial bleeding Breastfeeding Birth: 1 mg p.o.→25 μg/day PT>120 s (13.3); APTT>120 s (37) No Died B; female; 6 + 6/7 Intracranial bleeding Breastfeeding Birth: 1 mg p.o.→25 μg/day INR>8 (INR<2); APTT>240 s (49) Biliary atresia Hemipareses right C; male; 4 + 4/7 Intracranial bleeding Breastfeeding Birth: 1 mg p.o.→25 μg/day PT>90 s (30); APTT>120 s (38); Vitamin K: 0.1 nmol/l Biliary atresia Hemipareses right D; male; 5 + 2/7 Nasal bleeding Breastfeeding Birth: 1 mg p.o.→25 μg/day PT>75 s (normal); APTT: 118 s (normal) Biliary atresia No sequelae E; male; 6 + 2/7 Nasal bleeding Breastfeeding Birth: 1 mg p.o.→25 μg/day PT>165 s (normal); APTT>240 s (normal) Biliary atresia No sequelae F; male; 3 + 6/7 Nasal bleeding Breastfeeding Birth: 1 mg p.o.→25 μg/day PT>71 s (12.7); APTT: 128 s (39.1) Cholestasis No sequelae Concerning the cause of VKDB, there was one case of idiopathic VKDB, who presented with signs of bleeding at the age of 5 weeks. She was breastfed and died due to the consequence of an intracranial bleeding. She received exactly the recommended prophylaxis. Unfortunately, no post-mortem obduction was performed but, biochemically, there were no signs of cholestasis. Five out of six cases could be validated as secondary late VKDB. The age of presentation in this group was between 3 and 7 weeks of age. All of these bleedings were due to an underlying cholestasis which was diagnosed after the first presentation of bleeding. In two cases, an intracranial bleeding was the first presenting sign. Four cases were diagnosed as having a bile duct atresia. All infants had been exclusively breastfed and all had received exactly the recommended prophylaxis. 21 Discussion 1 3 12 14 25 32 33 5 1 4 18 Since the report of Golding et al., there has been an increasing trend towards oral vitamin K administration and many oral prophylaxis regimes have been developed. 1 1 1 3 2 P 1 1 1 15 31 15 1 1 1 9 1 32 27 22 22 24 31 3 13 14 19 22 30 31 33 Secondly, the surveillance lasted only 1 year. In all probability, the confidence interval would be smaller when the length of the surveillance was prolonged. Third, the proportion of infants that is breastfed is unknown, and, additionally, we lack data about compliance to the prophylaxis. It is likely, however, that less than 100% of the eligible cases received the recommended prophylaxis. Prophylaxis failure, therefore, is likely to be underestimated due to a greater denominator than the true numbers exposed. 1 1 6 1 28 1 1 1 Furthermore, an earlier recognition of symptoms associated with cholestatic liver disease and immediate investigation and treatment of “warning bleeds” helps to prevent the severe consequences of secondary late VKDB. 1