Factor X Deficiency: A Rare Cause of Puberty Menorrhagia.

09:38 EST 31st January 2015 | BioPortfolio

Summary of "Factor X Deficiency: A Rare Cause of Puberty Menorrhagia."

Factor X deficiency is an extremely rare coagulation defect inherited as an autosomal recessive disorder with variable bleeding manifestations. The authors report case of a 16 y-old girl born from a consanguineous marriage who presented with excessive bleeding at the start of menarche. Investigations revealed severe anemia, prolongation of both prothrombin time and activated partial thromboplastin time and moderate deficiency of factor X (1 %). She was given multiple transfusions including packed cells and fresh frozen plasma and was advised to remain under regular follow up.

Affiliation

Department of Pediatrics, SMGS Hospital, Shalamar, Jammu, India, virta266@yahoo.in.

Journal Details

This article was published in the following journal.

Name: Indian journal of pediatrics
ISSN: 0973-7693
Pages:

Links

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Medical and Biotech [MESH] Definitions

A deficiency of blood coagulation factor V (known as proaccelerin or accelerator globulin or labile factor) leading to a rare hemorrhagic tendency known as Owren's disease or parahemophilia. It varies greatly in severity. Factor V deficiency is an autosomal recessive trait. (Dorland, 27th ed)

Storage-stable glycoprotein blood coagulation factor that can be activated to factor Xa by both the intrinsic and extrinsic pathways. A deficiency of factor X, sometimes called Stuart-Prower factor deficiency, may lead to a systemic coagulation disorder.

The lack of development of SEXUAL MATURATION in boys and girls at a chronological age that is 2.5 standard deviations above the mean age at onset of PUBERTY in a population. Delayed puberty can be classified by defects in the hypothalamic LHRH pulse generator, the PITUITARY GLAND, or the GONADS. These patients will undergo spontaneous but delayed puberty whereas patients with SEXUAL INFANTILISM will not.

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A deficiency of blood coagulation FACTOR XIII or fibrin stabilizing factor (FSF) that prevents blood clot formation and results in a clinical hemorrhagic diathesis.

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