Delayed clotting in abetalipoproteinemia is due to:
Ref: Harrison's Principles of Internal Medicine 18th edition, pg 3153Explanation:Lipoproteins-Transport hydrophobic lipids thro' plasma fluids to & from tissuesAbsorption of dietary cholesterol, fatty-acids & fat soluble vitamins (A. D, E, K)Transport of cholesterol, triglycerides & fat- soluble vitamins from liver to per tissuesTransport of cholesterol from peripheral tissues to liverAbetalipoproteinemiaIt is an autosomal recessive disorder.M utation i n the microsomal triglyceride transfer protein (MTTP) gene needed for transport of lipids and chplesterol & fat-soluble vitamins.Low VLDL, chylomicronsFat malabsorption-Vitamin K deficiency- coagulopathySevere vit E deficiency-neurological manifestations A can thocytoisisHypocholesterolemiaRetinitis pigmentosa, posterior column neuropathy & myopathyTreated with high dose of Vitamin E & other vitamins.Vitamin K dependent clotting factore are-II (prothrombin), VII, IX & XVit K is needed for post-translational modification of glutamate residues to a-carboxyglutamate, needed for activity of the above factors.Vit K is a fat-soluble vitamin, it is a co-factor for post-translational modification of glutamate residues to y-carboxyglutamate on the clotting factors II. VIE IX & X. Warfarin blocks the reductase & competitively inhibits the effects of vitamin K.
A Decreased VLDL
Reduced clotting factor synthesis
Decreased chylomicrons
Decreased fatty acid synthesis
Bleeding and Thrombotic Disorders