You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information.
What is A newborn baby presented with profuse bleeding from umbilical stump after bih. What is the MOST probable diagnosis? and explain in detail?
This neonate is most probably suffering from factor XIII deficiency. Umbilical stump bleeding is a common feature of factor XIII deficiency. Patients usually have severe bleeding manifestations and hemorrhage can occur into any tissue. Factor XIII deficiency is caused by a defect in plasma transglutaminase that covalently cross links the fibrin alpha and gamma chains to form an impermeable fibrin clot. Screening test for clotting functions are normal in this disorder. Treatment is with cryoprecipitate. Ref: Molecular Pathology of Hematolymphoid Diseases edited by Cherie H. Dunphy page 516.