vitamin-k-1 and Hemorrhagic-Disorders

Topics: Aged, 80 and over; Anabolic Agents; Antifibrinolytic Agents; Blood Coagulation Disorders; Female; Hemorrhagic Disorders; Humans; International Normalized Ratio; Oxandrolone; Treatment Outcome; Vitamin K 1

Free-selling rodenticides contain highly potent and long-acting coumarins that may cause severe and prolonged coagulopathies.. A 46-year-old male was admitted after accidental ingestion of a rodenticide with spontaneous hematomas and a nonmeasurable prothrombin time (International Normalized Ratio [INR]). He had a prolonged coagulopathy and required high doses of vitamin K(1) to normalize the prothrombin time. A dose reduction of vitamin K(1) was followed by a new rise in the INR.. In cases of coagulopathy and suspicion of an intoxication with anticoagulants, an ingestion of rodenticides containing highly potent coumarins should be taken into account. Intoxications with these "superwarfarins" require high doses of vitamin K(1) given for several weeks.

Topics: Coumarins; Hemorrhagic Disorders; Humans; International Normalized Ratio; Male; Middle Aged; Prothrombin Time; Rodenticides; Vitamin K 1

In 1995, a new form of vitamin K prophylaxis with two oral doses of 2 mg mixed micellar phylloquinone (Konakion MM) on the 1st and 4th day of life was introduced in Switzerland. It was hoped that this new galenic preparation of phylloquinone would protect infants with insufficient or absent bile acid excretion from late vitamin K deficiency bleeding (VKDB). Subsequently, the occurrence of VKDB was monitored prospectively between July 1, 1995 and June 30, 2001 with the help of the Swiss Paediatric Surveillance Unit (SPSU). Over a period of 6 years (475,000 deliveries), there were no cases of early (<24 h of age), one case of classical (2-7 days of life), and 18 cases of late (1-12 weeks) VKDB fulfilling standard case definitions. In 13/18 patients with late VKDB there was pre-existing liver disease and in 4/18 patients, parents had refused prophylaxis. The incidence of late VKDB for infants with completed Konakion MM prophylaxis was 2.31/100,000 (95% CI: 1.16-4.14) and for the entire population 3.79/100,000 (95% CI: 2.24-5.98). There was only one case of late VKDB after complete prophylaxis in an infant without underlying liver disease.. two oral doses of 2 mg of a mixed micellar vitamin K preparation failed to abolish VKDB. The recommendations for vitamin K prophylaxis in Switzerland have therefore been changed to include a third dose at 4 weeks of age. Starting on January 1, 2004, the incidence of vitamin K deficiency bleeding will again be monitored prospectively by the Swiss Paediatric Surveillance Unit.

Topics: Administration, Oral; Hemorrhagic Disorders; Humans; Incidence; Infant, Newborn; Micelles; Prospective Studies; Switzerland; Vitamin K 1; Vitamin K Deficiency

Familial multiple coagulation factor deficiency (FMFD) of factors II, VII, IX, X, protein C, and protein S is a very rare bleeding disorder with autosomal recessive inheritance. The phenotypic presentation is variable with respect to the residual activities of the affected proteins, its response to oral administration of vitamin K, and to the involvement of skeletal abnormalities. The disease may result either from a defective resorption/transport of vitamin K to the liver, or from a mutation in one of the genes encoding gamma-carboxylase or other proteins of the vitamin K cycle. We have recently presented clinical details of a Lebanese family and a German family with 10 and 4 individuals, respectively, where we proposed autosomal recessive inheritance of the FMFD phenotype. Biochemical investigations of vitamin K components in patients' serum showed a significantly increased level of vitamin K epoxide, thus suggesting a defect in one of the subunits of the vitamin K 2,3-epoxide reductase (VKOR) complex. We now have performed a genome-wide linkage analysis and found significant linkage of FMFD to chromosome 16. A total maximum 2-point LOD score of 3.4 at theta = 0 was obtained in the interval between markers D16S3131 on 16p12 and D16S419 on 16q21. In both families, patients were autozygous for 26 and 28 markers, respectively, in an interval of 3 centimorgans (cM). Assuming that FMFD and warfarin resistance are allelic, conserved synteny between human and mouse linkage groups would restrict the candidate gene interval to the centromeric region of the short arm of chromosome 16.

Topics: Animals; Blood Coagulation Factors; Centromere; Child; Chromosome Mapping; Chromosomes, Human, Pair 16; DNA Mutational Analysis; Drug Resistance; Female; Genes, Recessive; Genetic Markers; Genotype; Germany; Glutathione Transferase; Hemorrhagic Disorders; Humans; Infant, Newborn; Lebanon; Lod Score; Male; Mice; Microsatellite Repeats; Mixed Function Oxygenases; Multienzyme Complexes; Pedigree; Rats; Species Specificity; Vitamin K; Vitamin K 1; Vitamin K Epoxide Reductases; Warfarin

Topics: Carbon-Carbon Ligases; Consanguinity; Global Health; Hemorrhagic Disorders; Humans; India; Infant, Newborn; Mixed Function Oxygenases; Registries; Vitamin K 1; Vitamin K Epoxide Reductases

Topics: Antifibrinolytic Agents; Coumarins; Hemorrhagic Disorders; Humans; Vitamin K 1; Vitamin K Deficiency

In 1995, a new water-soluble mixed-micellar analogue of vitamin K1 (Konakion MM paediatric) was introduced in Switzerland to replace the formerly used fat-soluble Konakion drops for the prevention of vitamin K1-deficiency-bleeding (VKDB) in infants. According to the new guidelines, an oral dose of 2 mg is given after birth and again on the 4th day of life. We examined the compliance with these guidelines and the impact on the incidence of VKDB. To assess compliance, questionnaires were sent to all hospitals with delivery services 6 months after the introduction of the new guidelines. Using the database of the Swiss Paediatric Surveillance Unit (SPSU) which records rare paediatric diseases, we assessed the incidence of VKDB in Switzerland between July 1995 and June 1998. In addition, we determined the precise circumstances under which the episodes of VKDB occurred. More than 99% of infants received vitamin K1 prophylaxis. Since July 1995, 93% of newborns have received prophylaxis according to the new guidelines; the remaining infants were given fat-soluble Konakion drops or parenteral vitamin K1. Within 3 years, one case of classical and 12 cases of late-onset VKDB (11 confirmed, 1 probable) were reported to the SPSU. Of the 11 confirmed late-onset cases, 7 received the recommended prophylaxis, whereas 3 had not and 1 had been given fat-soluble Konakion drops. All confirmed cases of late-onset VKDB occurred in fully breast-fed infants and 8 of 11 had hepatobiliary disease.. With the introduction of two oral doses of a mixed-micellar vitamin K1 preparation administered in the 1st week of life, the incidence of late vitamin K1-deficiency-bleeding has decreased from 7.2:100,000 between 1986-1987 to 2.8:100,000 between 1995 and 1998. This regimen may be suitable for prophylaxis of vitamin K1-deficiency-bleeding, however, it does not fully protect infants with cholestatic disease from late-onset bleeding. If oral prophylaxis is considered for these infants, vitamin K1 has to be administered repeatedly to all infants during the breast feeding period.

Topics: Administration, Oral; Antifibrinolytic Agents; Child Health Services; Female; Follow-Up Studies; Guidelines as Topic; Health Surveys; Hemorrhagic Disorders; Humans; Incidence; Infant, Newborn; Male; Primary Prevention; Registries; Switzerland; Treatment Outcome; Vitamin K 1; Vitamin K Deficiency

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

A 14-year-old girl presented with severe haemorrhagic diathesis. Her past history suggested a congenital bleeding disorder. Investigations disclosed severe deficiency of all four vitamin K-dependent factors and a functional defect of platelets. These were caused by simultaneous administration of vitamin K antagonists and anti-inflammatory drugs. A complete clinical and laboratory recovery took place following withdrawal of drugs. The severity of the haemorrhagic diathesis prompted us to describe the case in order to draw the attention of medical personnel to the dangerous potentiation effects of different drugs administered with vitamin K antagonists.

Topics: Adolescent; Blood Coagulation; Coumarins; Diagnosis, Differential; Female; Hemorrhagic Disorders; Hemostasis; Humans; Indomethacin; Partial Thromboplastin Time; Prothrombin Time; Thrombophlebitis; Vitamin K 1

Topics: Adrenochrome; Aminocaproates; Animals; Antifibrinolytic Agents; Carbon Tetrachloride Poisoning; Cyclohexanecarboxylic Acids; Dicumarol; Ethylamines; Factor Analysis, Statistical; Hemorrhagic Disorders; Hemostatics; Hydroquinones; Male; Phenols; Rats; Semicarbazones; Sulfonic Acids; Vitamin K; Vitamin K 1

Topics: Adult; Anticoagulants; Factor X; Hemorrhagic Disorders; Humans; Hypoprothrombinemias; Middle Aged; Vitamin K 1

Topics: Aminocaproates; Antifibrinolytic Agents; Aprotinin; Blood Coagulation Factors; Blood Proteins; Factor VII; Factor VIII; Fibrinogen; Hemorrhagic Disorders; Humans; Protamines; Vitamin K 1

Topics: Adrenocorticotropic Hormone; Aspirin; Blood Transfusion; Caffeine; Diphenhydramine; Hematoma; Hemorrhagic Disorders; Hypoprothrombinemias; Mouth; Phenacetin; Salicylates; Toxicology; Vitamin K 1

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

Topics: Biliary Tract; Female; Hemorrhage; Hemorrhagic Disorders; Humans; Liver Diseases; Postpartum Hemorrhage; Postpartum Period; Vitamin K 1

Topics: Antifibrinolytic Agents; Hemorrhagic Disorders; Humans; Infant, Newborn; Infant, Premature, Diseases; Vitamin K; Vitamin K 1; Vitamin K Deficiency Bleeding

Topics: Hemorrhagic Disorders; Humans; Hypoprothrombinemias; Infant, Newborn; Medical Records; Prothrombin; Vitamin K 1; Vitamin K Deficiency Bleeding

Topics: Antifibrinolytic Agents; Hemorrhagic Disorders; Hemostatics; Humans; Infant, Newborn; Naphthoquinones; Vitamin K; Vitamin K 1; Vitamin K Deficiency Bleeding

Topics: Administration, Intravenous; Antifibrinolytic Agents; Hemolysis; Hemorrhagic Disorders; Humans; Hypoprothrombinemias; Prothrombin; Vitamin K; Vitamin K 1

Topics: Antifibrinolytic Agents; Hemorrhagic Disorders; Hypoprothrombinemias; Prothrombin; Vitamin K; Vitamin K 1; Vitamins

Topics: Administration, Intravenous; Anticoagulants; Antidotes; Coumarins; Dicumarol; Hemorrhagic Disorders; Hypoprothrombinemias; Prothrombin; Vitamin K 1

Topics: Antifibrinolytic Agents; Hemorrhagic Disorders; Humans; Hypoprothrombinemias; Naphthoquinones; Vitamin K; Vitamin K 1

Topics: Antifibrinolytic Agents; Factor VII; Hemorrhagic Disorders; Humans; Hypoprothrombinemias; Infant, Newborn; Naphthoquinones; Retinoids; Vitamin K; Vitamin K 1

Topics: Anticoagulants; Antifibrinolytic Agents; Hemorrhagic Disorders; Heparin Antagonists; Hypoprothrombinemias; Phenindione; Vitamin K; Vitamin K 1

Topics: Antifibrinolytic Agents; Coumarins; Dicumarol; Hemorrhagic Disorders; Heparin Antagonists; Hypoprothrombinemias; Naphthoquinones; Vitamin K; Vitamin K 1; Vitamin K 3

Topics: Antifibrinolytic Agents; Hemorrhagic Disorders; Heparin Antagonists; Hypoprothrombinemias; Naphthoquinones; Vitamin K; Vitamin K 1