Six Cases of Alpha-1-Antitrypsin Deficiency Presenting as a Bleeding Diathesis with Intracranial Hemorrhage in the Newborn

  • Günter Auerswald
  • Anton H. Sutor
Conference paper


Alpha-1-antitrypsin (alpha-1-AT), or alpha-1-protease inhibitor is a glycoprotein with a molecular weight of about 52. 000 daltons. More than 90% of it is synthesized by the liver. The concentration in serum in healthy children is 2.0–3.3 g/1. Alpha-1-AT constitutes about 90% of the whole alpha-1-globulin fraction. The gene responsible for the production of alpha-1-AT is situated on the long arm of chromosome 14.


Intracranial Hemorrhage Disseminate Intravascular Coagulation Disseminate Intravascular Coagulation Prothrombin Complex Concentrate Bleeding Diathesis 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Psacharopoulos HT, Mowat AP, Cook PJL, Carlile PA, Portmann B, Rodeck CH (1983) Outcome of liver disease associated with alpha-1-antitrypsin deficiency (Pi Z). Arch Dis Child 58: 882–887PubMedCrossRefGoogle Scholar
  2. 2.
    Payne NR, Hasegawa DK (1984) Vitamin K deficiency in newborns: a case report in α-1-antitrypsin deficiency and a review of factors predisposing to hemorrhage. J Pediatr 73: 712–716Google Scholar
  3. 3.
    Fidalgo I, Vazquez C, Rodriguez-Soriano J (1982) Intracranial hemorrhage due to vitamin K deficiency associated with alpha-1-antitrypsin deficiency type Pi Z. Arch Dis Child 57: 722PubMedCrossRefGoogle Scholar
  4. 4.
    Jenkins HR, Leonard JV, Kay JDS (1982) Alpha-1-antitrypsin deficiency, bleeding diathesis, and intracranial hemorrhage. Arch Dis Child 57: 722–723PubMedCrossRefGoogle Scholar
  5. 5.
    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: 68–70PubMedGoogle Scholar
  6. 6.
    Hutchison DCS (1988) Natural history of alpha-1-protease inhibitor deficiency (Suppl. 6A). Am J Med 84: 3–12PubMedGoogle Scholar

Copyright information

© Springer Japan 1991

Authors and Affiliations

  • Günter Auerswald
    • 1
  • Anton H. Sutor
    • 2
  1. 1.Hess-KinderklinikBremen 1Federal Republic of Germany
  2. 2.Universitäts-KinderklinikFreiburg i.Br.Federal Republic of Germany

Personalised recommendations