vitamin-k-semiquinone-radical and Deficiency-Diseases

The purpose of this review is to summarize the research to date on vitamin K status in chronic kidney disease (CKD). This review includes a summary of the data available on vitamin K status in patients across the spectrum of CKD as well as the link between vitamin K deficiency in CKD and bone dynamics, including mineralization and demineralization, as well as ectopic mineralization. It also describes two current clinical trials that are underway evaluating vitamin K treatment in CKD patients. These data may inform future clinical practice in this population.

Topics: Bone and Bones; Bone Density; Deficiency Diseases; Humans; Kidney Failure, Chronic; Nutritional Status; Renal Insufficiency, Chronic; Vitamin K; Vitamins

We report the case of a 57-year old woman whose severe protein C deficiency was revealed soon after oral anticoagulants were introduced into her treatment. Two previous episodes of deep leg vein thrombosis followed by a third episode with suspicion of embolus migration had led to treatment with heparin later replaced by oral anticoagulants. On the 4th day of anticoagulant therapy, she developed skin necrosis of the left calf. A protein C assay showed severe deficiency (19% level as compared with the 70-120% normal levels). The main causes of acquired protein C deficiency were excluded. The first results of a family study demonstrated moderate protein C deficiency in a 30-year old, asymptomatic daughter.

Topics: Adult; Anticoagulants; Deficiency Diseases; Female; Humans; Leg; Middle Aged; Necrosis; Protein C Deficiency; Skin; Thromboembolism; Vitamin K

Topics: Ascorbic Acid; Child, Preschool; Deficiency Diseases; Female; Fever; Folic Acid; Humans; Infant; Infant Food; Infant Nutritional Physiological Phenomena; Infant, Newborn; Infections; Kinetics; Male; Milk, Human; Nutritional Physiological Phenomena; Nutritional Requirements; Pyridoxine; Thiamine; Vitamin A; Vitamin A Deficiency; Vitamin D; Vitamin E; Vitamin K; Vitamins

Topics: Animals; Cattle; Deficiency Diseases; Dogs; Humans; Nutritional Physiological Phenomena; Rats; Vitamin K

alpha-Methylacyl-CoA racemase (Amacr) deficiency in humans leads to sensory motor neuronal and liver abnormalities. The disorder is recessively inherited and caused by mutations in the AMACR gene, which encodes Amacr, an enzyme presumed to be essential for bile acid synthesis and to participate in the degradation of methyl-branched fatty acids. To generate a model to study the pathophysiology in Amacr deficiency we inactivated the mouse Amacr gene. As per human Amacr deficiency, the Amacr(-/-) mice showed accumulation (44-fold) of C27 bile acid precursors and decreased (over 50%) primary (C24) bile acids in bile, serum and liver, however the Amacr(-/-) mice were clinically symptomless. Real-time quantitative PCR analysis showed that, among other responses, the level of mRNA for peroxisomal multifunctional enzyme type 1 (pMFE-1) was increased 3-fold in Amacr(-/-) mice. This enzyme can be placed, together with CYP3A11 and CYP46A1, to make an Amacr-independent pathway for the generation of C24 bile acids. Exposure of Amacr(-/-) mice to a diet supplemented with phytol, a source for branched-chain fatty acids, triggered the development of a disease state with liver manifestations, redefining the physiological significance of Amacr. Amacr is indispensable for the detoxification of dietary methyl-branched lipids and, although it contributes normally to bile acid synthesis from cholesterol, the putative pMFE-1-mediated cholesterol degradation can provide for generation of bile acids, allowing survival without Amacr. Based upon our mouse model, we propose elimination of phytol from the diet of patients suffering from Amacr deficiency.

Topics: Animals; Aryl Hydrocarbon Hydroxylases; Bile Acids and Salts; Body Weight; Cholesterol; Cholesterol 24-Hydroxylase; Clofibrate; Cytochrome P-450 CYP3A; Deficiency Diseases; Deoxyribonucleases, Type II Site-Specific; Dietary Fats; Disease Models, Animal; Female; Gene Expression Regulation; Hypolipidemic Agents; Kidney; Lipids; Liver; Male; Membrane Proteins; Mice; Mice, Mutant Strains; Oxidoreductases, N-Demethylating; Phytol; Racemases and Epimerases; Steroid Hydroxylases; Vitamin K

Topics: Animals; Body Weight; Choline; Deficiency Diseases; Diet; Drinking Behavior; Fatty Liver; Feces; Feeding Behavior; Female; Hematocrit; Hemoglobins; Hemorrhage; Male; Nitrogen; Nutritional Physiological Phenomena; Organ Size; Rats; Time Factors; Urine; Vitamin K

Topics: Adenoidectomy; Child; Child, Preschool; Deficiency Diseases; Female; Hemorrhage; Humans; Male; Postoperative Complications; Preoperative Care; Prothrombin Time; Tonsillectomy; Vitamin K

Topics: Alcoholism; Anemia; Anemia, Macrocytic; Anorexia Nervosa; Ascorbic Acid; Avitaminosis; Celiac Disease; Deficiency Diseases; Diet; Diet Therapy; Female; Folic Acid; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Pregnancy; Pregnancy Complications; Sprue, Tropical; United Kingdom; Vitamin A; Vitamin B 12; Vitamin B Complex; Vitamin D; Vitamin K; Vitamins; Vomiting

Topics: Amino Acids; Amino Acids, Essential; Ammonia; Blood Chemical Analysis; Deficiency Diseases; Erythropoiesis; Humans; Liver Diseases; Postoperative Care; Preoperative Care; Proteins; Vitamin A; Vitamin K; Vitamins; Wound Healing

Topics: Child; Deficiency Diseases; Humans; Vitamin A; Vitamin K; Vitamins

Topics: Deficiency Diseases; Diet; Diet Therapy; Glossitis; Glossitis, Benign Migratory; Humans; Malnutrition; Minnesota; Placebos; Students; Vitamin A; Vitamin K; Vitamins

Topics: Child; Deficiency Diseases; Diet; Humans; Infant; Malnutrition; Vitamin A; Vitamin K; Vitamins

Topics: Deficiency Diseases; Eye; Humans; Ophthalmology; Vitamin A; Vitamin K; Vitamins

Topics: Carbamoyl-Phosphate Synthase I Deficiency Disease; Deficiency Diseases; Vitamin A; Vitamin K; Vitamins

Topics: Deficiency Diseases; Glossitis; Humans; Vitamin A; Vitamin K; Vitamins

Topics: Avitaminosis; Deficiency Diseases; Humans; Vitamin A; Vitamin K; Vitamins

Topics: Avitaminosis; Deficiency Diseases; Humans; Skin; Skin Diseases; Vitamin A; Vitamin K; Vitamins

Topics: Deficiency Diseases; Eye; Eye Diseases; Vitamin A; Vitamin K; Vitamins

Topics: Avitaminosis; Deficiency Diseases; Muscles; Oculomotor Muscles; Vitamin A; Vitamin K; Vitamins

Topics: Deficiency Diseases; Diet, Reducing; Humans; Nutrition Therapy; Nutritional Status; Vitamin K; Vitamins

Topics: Deficiency Diseases; Diet, Reducing; Humans; Vitamin K; Vitamins

Topics: Avitaminosis; Deficiency Diseases; Humans; Vitamin A; Vitamin A Deficiency; Vitamin K; Vitamins

Topics: Carbamoyl-Phosphate Synthase I Deficiency Disease; Deficiency Diseases; Humans; Spinal Cord; Vitamin A; Vitamin K; Vitamins

Topics: Deficiency Diseases; Dietetics; Nutrition Therapy; Nutritional Sciences; Nutritional Status; Vitamin K; Vitamins

Topics: Antifibrinolytic Agents; Deficiency Diseases; Humans; Liver; Retinoids; Vitamin K

Topics: Deficiency Diseases; Folic Acid; Humans; Stomatitis; Vitamin A; Vitamin B Complex; Vitamin K; Vitamins

Topics: Deficiency Diseases; Eye; Humans; Metabolism; Scotoma; Vitamin A; Vitamin K; Vitamins

Topics: Blood Proteins; Deficiency Diseases; Diet, Reducing; Serum; Vitamin A; Vitamin K; Vitamins

Topics: Avitaminosis; Carbamoyl-Phosphate Synthase I Deficiency Disease; Deficiency Diseases; Disease; Humans; Nutritional Sciences; Urine; Vitamin A; Vitamin K; Vitamins

Topics: Avitaminosis; Deficiency Diseases; Humans; Male; Vitamin A; Vitamin K; Vitamins

Topics: Deficiency Diseases; Dermatology; Humans; Skin Diseases; Vitamin A; Vitamin K; Vitamins

Topics: Avitaminosis; Calculi; Deficiency Diseases; Humans; Vitamin A; Vitamin K; Vitamins

Topics: Deficiency Diseases; Humans; Mouth Diseases; Vitamin A; Vitamin K; Vitamins

Topics: Deficiency Diseases; Humans; Skin Diseases; Vitamin A; Vitamin K; Vitamins