Six Cases of Alpha-1-Antitrypsin Deficiency Presenting as a Bleeding Diathesis with Intracranial Hemorrhage in the Newborn
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.
KeywordsIntracranial Hemorrhage Disseminate Intravascular Coagulation Disseminate Intravascular Coagulation Prothrombin Complex Concentrate Bleeding Diathesis
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