Severe protein C deficiency in newborn infants. 1988

M J Manco-Johnson, and R A Marlar, and L J Jacobson, and T Hays, and B A Warady
Department of Pediatrics, University of Colorado School of Medicine, Denver 80262.

Eleven infants initially seen in the neonatal period had levels of protein C suggestive of homozygous protein C deficiency but as an apparently acquired condition. Family studies failed to document parental carrier status, the clinical course was not typical of that reported with homozygous protein C deficiency, and protein C levels increased in all restudied infants, six of whom received heparin anticoagulation. No infant had evidence of vitamin K deficiency. Care is advised in the evaluation of infants with low levels of protein C. Parental blood studies, delayed testing, and serial assays can help to establish the correct diagnosis.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D008297 Male Males
D011486 Protein C A vitamin-K dependent zymogen present in the blood, which, upon activation by thrombin and thrombomodulin exerts anticoagulant properties by inactivating factors Va and VIIIa at the rate-limiting steps of thrombin formation.
D011516 Prothrombin A plasma protein that is the inactive precursor of thrombin. It is converted to thrombin by a prothrombin activator complex consisting of factor Xa, factor V, phospholipid, and calcium ions. Deficiency of prothrombin leads to hypoprothrombinemia. Coagulation Factor II,Factor II,Blood Coagulation Factor II,Differentiation Reversal Factor,Factor II, Coagulation,Factor, Differentiation Reversal,II, Coagulation Factor
D005260 Female Females
D006493 Heparin A highly acidic mucopolysaccharide formed of equal parts of sulfated D-glucosamine and D-glucuronic acid with sulfaminic bridges. The molecular weight ranges from six to twenty thousand. Heparin occurs in and is obtained from liver, lung, mast cells, etc., of vertebrates. Its function is unknown, but it is used to prevent blood clotting in vivo and vitro, in the form of many different salts. Heparinic Acid,alpha-Heparin,Heparin Sodium,Liquaemin,Sodium Heparin,Unfractionated Heparin,Heparin, Sodium,Heparin, Unfractionated,alpha Heparin
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D014427 Twins Two individuals derived from two FETUSES that were fertilized at or about the same time, developed in the UTERUS simultaneously, and born to the same mother. Twins are either monozygotic (TWINS, MONOZYGOTIC) or dizygotic (TWINS, DIZYGOTIC). Twin
D020151 Protein C Deficiency An absence or deficiency in PROTEIN C which leads to impaired regulation of blood coagulation. It is associated with an increased risk of severe or premature thrombosis. (Stedman's Med. Dict., 26th ed.) Deficiency, Protein C,Hereditary Thrombophilia Due To Protein C Deficiency,Deficiencies, Protein C,Protein C Deficiencies

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