106 younger than 3 years [5, 7] or the false-positive results which can be observed with immunohistochemistry in this age group [7]. In conclusion, however, early recognition of the warning bleeds or cholestasis, correct diagnosis using pathology as a diagnostic instrument in addition to the existing methods and adjustment of the standard vitamin K prophylaxis protocols to prevent VKDB complications can be life saving in AAT deficiency patients especially in the neonatal period. Open Access This article is distributed under the terms of the Creative Commons Attribution Noncommercial License which per- mits any noncommercial use, distribution, and reproduction in any medium, provided the original author(s) and source are credited. References 1. Brantly M (2006) Efficient and accurate approaches to the laboratory diagnosis of alpha1-antitrypsin deficiency: the promise of early diagnosis and intervention. Clin Chem 52:2180–2181 Eur J Pediatr (2011) 170:103–106 2. Brantly M, Nukiwa T, Crystal RG (1988) Molecular basis of alpha-1-antitrypsin deficiency. Am J Med 84(suppl 6A):13–31 3. Fregonese L, Stolk J (2008) Hereditary alpha-1-antitrypsin deficiency and its clinical consequenties. Orphanet J Rare Dis 3:16 4. Hope PL, Hall MA, Millward-Sadler GH, Normand ICS (1982) Alpha-1-antitrypsin deficiency presenting as a bleeding diathesis in the newborn. Arch Dis Child 57:68–70 5. Hutchinson DCS (1988) Natural history of alpha-1-protease inhibitor deficiency. Am J Med 84(Suppl 6A):3–12 6. Israels SJ, Gilfix BM (1999) Alpha-1-antitrypsin deficiency with fatal intracranial hemorrhage in a newborn. J Pediatr Hematol Oncol 21(5):447–450 7. Jevon GP, Dimmick JE (1998) Histopathologic approach to metabolic liver disease: part 1. Pediatr Dev Pathol 1:179–199 8. Lomas DA, Evans DL, Finch JT, Carrell RW (1992) The mechanism of Z alpha-1-antitrypsin accumulation in the liver. Nature 357(6379):605–607 9. Luisetti M, Seersholm N (2004) α1-Antitrypsin deficiency. 1: Epidemiology of α1-antitrypsin deficiency. Thorax 59:164–169 10. Uitentuis J (1990) Administration of vitamin K to neonates and infants. Ned Tijdschr Geneeskd 134:1642-6 11. Van der Anker JN, Sinaasappel M (1993) Bleeding as presenting symptom of cholestasis. J Perinatol 13(4):322–324 12. Van Hasselt PM, Kok K, Vorselaars AD et al (2009) Vitamin K deficiency bleeding in cholestatic infants with alpha-1-antitrypsin deficiency. Arch Dis Child Fetal Neonatal Ed 94:F456–F460