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Reduced factor X activity

MedGen UID:
892449
Concept ID:
C4024702
Finding
Synonyms: Decreased factor x activity; Factor X deficiency
 
HPO: HP:0008321

Definition

Reduced activity of coagulation factor X. The extrinsic and intrinsic pathways converge at factor X (fX). The extrinsic pathway activates fX by means of d factor VII with its cofactor, tissue factor. The intrinsic pathway activates fX by means of the tenase complex (Ca2+ and factors VIIIa, IXa and X) on the surface of activated platelets. Factor Xa in turn activates prothrombin (factor II) to thrombin (factor IIa). [from HPO]

Term Hierarchy

Conditions with this feature

Hereditary factor X deficiency disease
MedGen UID:
543976
Concept ID:
C0272327
Disease or Syndrome
A rare inherited bleeding disorder with a decreased antigen and/or activity of factor X (FX) and characterized by mild to severe bleeding symptoms.
Body skin hyperlaxity due to vitamin K-dependent coagulation factor deficiency
MedGen UID:
332067
Concept ID:
C1835813
Disease or Syndrome
Body skin hyperlaxity due to vitamin K-dependent coagulation factor deficiency is a very rare genetic skin disease characterized by severe skin laxity affecting the trunk and limbs.
Vitamin K-dependent clotting factors, combined deficiency of, type 2
MedGen UID:
334505
Concept ID:
C1843832
Disease or Syndrome
Deficiency of all vitamin K-dependent clotting factors leads to a bleeding tendency that is usually reversed by oral administration of vitamin K. Familial multiple coagulation factor deficiency is rare. Clinical symptoms of the disease include episodes of intracranial hemorrhage in the first weeks of life, sometimes leading to a fatal outcome (Fregin et al., 2002). For a general phenotypic description and a discussion of genetic heterogeneity of combined deficiency of vitamin K-dependent clotting factors, see VKCFD1 (277450).
Vitamin K-dependent clotting factors, combined deficiency of, type 1
MedGen UID:
376381
Concept ID:
C1848534
Disease or Syndrome
Deficiency of all vitamin K-dependent clotting factors leads to a bleeding tendency that is usually reversed by oral administration of vitamin K. Acquired forms of the disorder can be caused by intestinal malabsorption of vitamin K. Familial multiple coagulation factor deficiency is rare. Clinical symptoms of the disease include episodes of intracranial hemorrhage in the first weeks of life, sometimes leading to a fatal outcome. The pathomechanism is based on a reduced hepatic gamma-carboxylation of glutamic acid residues of all vitamin K-dependent blood coagulation factors, as well as the anticoagulant factors protein C (612283) and protein S (176880). Posttranslational gamma-carboxylation of proteins enables the calcium-dependent attachment of the proteins to the phospholipid bilayer of membranes, an essential prerequisite for blood coagulation. Vitamin K1 acts as a cofactor for the vitamin K-dependent carboxylase in liver microsomes, GGCX. Genetic Heterogeneity of Combined Deficiency of Vitamin K-Dependent Clotting Factors Combined deficiency of vitamin K-dependent clotting factors-2 (VKFCD2; 607473) is caused by mutation in the gene encoding vitamin K epoxide reductase (VKORC1; 608547) on chromosome 16p11.

Professional guidelines

PubMed

Fallah A, Shams M, Agi E, Jazebi M, Baghaipoor MR, Naderi T, Rezvany MR
Blood Coagul Fibrinolysis 2022 Mar 1;33(2):75-82. doi: 10.1097/MBC.0000000000001101. PMID: 35140190

Recent clinical studies

Diagnosis

Fair DS, Plow EF, Edgington TS
J Clin Invest 1979 Oct;64(4):884-94. doi: 10.1172/JCI109554. PMID: 90058Free PMC Article

Therapy

Fair DS, Plow EF, Edgington TS
J Clin Invest 1979 Oct;64(4):884-94. doi: 10.1172/JCI109554. PMID: 90058Free PMC Article

Clinical prediction guides

Fair DS, Plow EF, Edgington TS
J Clin Invest 1979 Oct;64(4):884-94. doi: 10.1172/JCI109554. PMID: 90058Free PMC Article

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