vitamin-k-1 and Hemophilia-B

The indications for transfusions are anemia compromising delivery of oxygen, acute blood loss, cardiopulmonary bypass, exchange transfusion, maintenance of hemostasis, and sepsis associated with granulocytopenia. When transfusion therapy is indicated, only that component of whole blood which is needed for correction of the problem should be given. The options for use each component have been discussed.

Topics: Acute Disease; Agranulocytosis; Anemia; Anemia, Aplastic; Blood Cells; Blood Coagulation; Blood Platelets; Blood Transfusion; Blood Transfusion, Autologous; Cardiopulmonary Bypass; Colloids; Erythroblastosis, Fetal; Erythrocytes; Exchange Transfusion, Whole Blood; Factor VIII; Female; Hemophilia A; Hemophilia B; Hemorrhage; Hemostasis; Hepatic Encephalopathy; Humans; Pregnancy; Purpura, Thrombocytopenic; Thrombocytopenia; Vitamin K 1

Hereditary combined deficiency of vitamin K-dependent factors is a rare entity. We report a 7-year-old girl of Arab origin with hereditary deficiency of the procoagulants factors II, VII, IX and X and the natural anticoagulants proteins C and S. The patient is the tenth offspring of a consanguinous marriage and presented at 6 weeks with spontaneous intracerebral haemorrhage. Symptoms improved following plasma infusion. A sibling died at 5 d from uncontrollable umbilical bleeding. Blood coagulation work-up at 6 years showed: factor II:C (activity) 12 U/dl, factor II:Ag (antigen) 40 U/dl; factor VII:C 12 U/dl; factor IX:C 36 U/dl, factor IX:Ag 57 U/dl; factor X:C 17 U/dl, factor X:Ag 54 U/dl; protein C activity 43 U/dl; protein C:Ag 45 U/dl; protein S:Ag 34 U/dl; levels of factors V:C and VIII:C were normal. Assays of coagulation factors in the parents and five of the siblings were within the normal range. Following acute infection and dilantin therapy procoagulant activity levels were reduced further and were partially increased after vitamin K infusion. Crossed immunoelectrophoresis of prothrombin in the presence of calcium lactate revealed a population of des-carboxyprothrombin. Serum vitamin K epoxide levels were undetectable. The data suggest that the defect in our patient stems from abnormal carboxylation of the vitamin K-dependent proteins and that the mode of inheritance is autosomal recessive.

Topics: Blood Coagulation Disorders; Child; Factor VII Deficiency; Factor X Deficiency; Female; Glycoproteins; Hemophilia B; Hemorrhagic Disorders; Humans; Hypoprothrombinemias; Male; Pedigree; Protein C Deficiency; Protein S; Vitamin K; Vitamin K 1

Topics: Animals; Blood Transfusion; Combined Modality Therapy; Dog Diseases; Dogs; Hemophilia B; Heterozygote; Male; Vitamin K 1

Topics: Blood Coagulation Tests; Blood Transfusion; Factor VII Deficiency; Hemoglobinometry; Hemophilia B; Humans; Infant, Newborn; Prothrombin Time; Vitamin K 1; Vitamin K Deficiency Bleeding

Topics: Anemia; Exchange Transfusion, Whole Blood; Factor VII Deficiency; Factor VIII; Hemophilia B; Hemorrhagic Disorders; Humans; Hyperbilirubinemia; Hypoprothrombinemias; Infant; Infant, Newborn; Infant, Newborn, Diseases; Prednisone; Scalp; Skull Fractures; Thromboplastin; Vitamin K 1