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What is A newborn baby presented with profuse bleeding from umbilical stump after bih. Probable diagnosis is: and explain in detail?
Congenital factor XIII deficiency is a rare autosomal recessive disease usually associated with a severe bleeding diathesis. Bleeding typically is delayed, because clots form normally but are susceptible to fibrinolysis. Umbilical stump bleeding is characteristic, and there is a high risk of intracranial bleeding. Replacement can be accomplished with FFP, cryoprecipitate, or a factor XIII concentrate. Ref: Gonzalez E.A., Jastrow K.M., Holcomb J.B., Kozar R.A. (2010). Chapter 4. Hemostasis, Surgical Bleeding, and Transfusion. In F.C. Brunicardi, D.K. Andersen, T.R. Billiar, D.L. Dunn, J.G. Hunter, J.B. Matthews, R.E. Pollock (Eds), Schwaz's Principles of Surgery, 9e.