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Case Report



Management of hypodysfibrinogenemia presenting multiple episodes of internal and external bleeding: a case report

Nithish Sattoju, Anvesh Maram, Rishitha Sanjana Abbagoni, Sai Ram Ganapaka, Vydhika Anneboina.




Abstract
Cited by 0 Articles

Background: Fibrinogen, a major clotting factor, can impact the bleeding issues clinically, precipitating uncontrolled bleeding, which is to be managed with plasma transfusion. If its levels or functioning is altered, it results in fibrinogen disorders, of which Hypo-, Dys-, and Hypodys-fibrinogenemia are the three major types.
Material and Methodology:
A single patient of hypodysfibrinogenemia, the rarest of its type, presented with multiple episodes of internal and external bleeding and was diagnosed with SDH secondary to hypodys-. Due to uncontrolled bleeding, the patient is operative two times and is managed with plasma transfusion.
In the postoperative period, the patient is transfused with Fresh Frozen Plasma and Cryoprecipitate to manage bleeding episodes.
Results and Conclusion:
Upon transfusing FFP once every 3 or 4 days, the bleeding is seen within 24 hrs, and recurrence of SDH is seen thrice within a period of 28 days. On transfusion of CRYO, the bleeding was not seen for 61 days, and even the CT scan showed a reversal of SDH.
CRYO can manage bleeding episodes very efficiently in hypodysfibrinogenemia than FFP. However, the evidence provided here is of a single patient and is insufficient to justify the above proof. Still, it may help in deciding the therapeutic regimen for treating hypodysfibrinogenemia.

Key words: Hypodysfibrinogenemia, Burr Hole Craniotomy with Evacuation of SDH, Fresh Frozen Plasma (FFP), Cryoprecipitate (CRYO), Factor 7 Deficiency, Clotting Factor Assay, Fibrinogen, Fibrinogen Activity and Antigen Level






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