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Hereditary deficiency of vitamin-K-dependent coagulation factors in Rambouillet sheep.

Abstract

A flock of Rambouillet sheep experienced unexpected lamb mortality associated with excessive bleeding at the time of parturition. Most lambs died of blood loss through the umbilicus or into subcutaneous tissues. Subsequently, nine ewes which had previously delivered lambs that bled to death were bred to the suspected sire of the previous bleeding lambs. Fifteen lambs were born alive the following Spring, and three males and one female bled clinically. These lambs had markedly decreased factor IX (< 16%) and factor X (< 4%) activities, with variably decreased factor II (11-36%) and factor VII (20-37%) activities. Protein C chromogenic activity was also markedly decreased (< 1%) in these lambs. The results from crossed immunoelectrophoresis and 'protein-induced-in-vitamin-K-absence' determination of the plasma of affected lambs, with antiserum directed against coagulation factor X, protein C or proteins S, suggested that these proteins were not carboxylated normally. Examination of liver from one lamb in the first batch and the four subsequent lambs did not reveal a known vitamin K antagonist. The breeding data suggested that the coagulopathy in these sheep was inherited as an autosomal recessive trait. The genetic or molecular defect that exists in these lambs is unknown, but possibilities include abnormal gamma-glutamyl carboxylase activity or abnormal metabolism of vitamin K.

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  • Authors+Show Affiliations

    ,

    Department of Pathology, College of Veterinary Medicine, Colorado State University, Fort Collins 80523, USA. dcbaker@lamar.colostate.edu

    , , , ,

    Source

    MeSH

    Animals
    Blood Coagulation Factors
    Coagulation Protein Disorders
    Counterimmunoelectrophoresis
    Factor VII
    Factor X Deficiency
    Female
    Genes, Recessive
    Hemophilia B
    Liver
    Male
    Partial Thromboplastin Time
    Protein C
    Protein S
    Prothrombin
    Sheep
    Sheep Diseases
    Thrombin Time
    Vitamin K

    Pub Type(s)

    Journal Article
    Research Support, Non-U.S. Gov't

    Language

    eng

    PubMed ID

    10192655

    Citation

    Baker, D C., et al. "Hereditary Deficiency of vitamin-K-dependent Coagulation Factors in Rambouillet Sheep." Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis, vol. 10, no. 2, 1999, pp. 75-80.
    Baker DC, Robbe SL, Jacobson L, et al. Hereditary deficiency of vitamin-K-dependent coagulation factors in Rambouillet sheep. Blood Coagul Fibrinolysis. 1999;10(2):75-80.
    Baker, D. C., Robbe, S. L., Jacobson, L., Manco-Johnson, M. J., Holler, L., & Lefkowitz, J. (1999). Hereditary deficiency of vitamin-K-dependent coagulation factors in Rambouillet sheep. Blood Coagulation & Fibrinolysis : an International Journal in Haemostasis and Thrombosis, 10(2), pp. 75-80.
    Baker DC, et al. Hereditary Deficiency of vitamin-K-dependent Coagulation Factors in Rambouillet Sheep. Blood Coagul Fibrinolysis. 1999;10(2):75-80. PubMed PMID: 10192655.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Hereditary deficiency of vitamin-K-dependent coagulation factors in Rambouillet sheep. AU - Baker,D C, AU - Robbe,S L, AU - Jacobson,L, AU - Manco-Johnson,M J, AU - Holler,L, AU - Lefkowitz,J, PY - 1999/4/7/pubmed PY - 1999/4/7/medline PY - 1999/4/7/entrez SP - 75 EP - 80 JF - Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis JO - Blood Coagul. Fibrinolysis VL - 10 IS - 2 N2 - A flock of Rambouillet sheep experienced unexpected lamb mortality associated with excessive bleeding at the time of parturition. Most lambs died of blood loss through the umbilicus or into subcutaneous tissues. Subsequently, nine ewes which had previously delivered lambs that bled to death were bred to the suspected sire of the previous bleeding lambs. Fifteen lambs were born alive the following Spring, and three males and one female bled clinically. These lambs had markedly decreased factor IX (< 16%) and factor X (< 4%) activities, with variably decreased factor II (11-36%) and factor VII (20-37%) activities. Protein C chromogenic activity was also markedly decreased (< 1%) in these lambs. The results from crossed immunoelectrophoresis and 'protein-induced-in-vitamin-K-absence' determination of the plasma of affected lambs, with antiserum directed against coagulation factor X, protein C or proteins S, suggested that these proteins were not carboxylated normally. Examination of liver from one lamb in the first batch and the four subsequent lambs did not reveal a known vitamin K antagonist. The breeding data suggested that the coagulopathy in these sheep was inherited as an autosomal recessive trait. The genetic or molecular defect that exists in these lambs is unknown, but possibilities include abnormal gamma-glutamyl carboxylase activity or abnormal metabolism of vitamin K. SN - 0957-5235 UR - https://www.unboundmedicine.com/medline/citation/10192655/Hereditary_deficiency_of_vitamin_K_dependent_coagulation_factors_in_Rambouillet_sheep_ L2 - http://ovidsp.ovid.com/ovidweb.cgi?T=JS&amp;PAGE=linkout&amp;SEARCH=10192655.ui DB - PRIME DP - Unbound Medicine ER -