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Factor XI deficiency in an Ashkenazi Jewish child, causing severe postoperative hemorrhage
1Department of Pediatrics, Sinai Hospital of Baltimore, MD, USA.
Insights
Preoperative screening for factor XI deficiency is recommended for Ashkenazi Jews due to its high incidence. This proactive approach can prevent severe postoperative bleeding in patients with undiagnosed inherited coagulation disorders.
Area of Science:
- Hematology
- Genetics
- Surgical Medicine
Background:
- Inherited coagulation factor deficiencies are uncommon, particularly in individuals without a personal or family history of bleeding.
- Undiagnosed hemostatic disorders can lead to severe complications during and after surgical procedures.
Observation:
- A case is presented where an inherited coagulation factor deficiency was not diagnosed preoperatively.
- This patient experienced significant hemostatic problems following surgery.
Findings:
- Factor XI deficiency is notably prevalent within the Ashkenazi Jewish population.
- Testing for activated partial thromboplastin time (aPTT) preoperatively is advised for all Ashkenazi Jews, irrespective of their bleeding history.
Implications:
- Awareness of uncommon hemostatic disorders and their management is crucial for surgeons and anesthesiologists.
- Proactive screening can mitigate risks associated with unexplained postoperative hemorrhage in susceptible populations.
Abstract:
Although inherited coagulation factor deficiencies with negative bleeding histories are rare, cases such as the one presented herein may not be diagnosed at the time of surgery and may experience severe hemostatic problems postoperatively. It may be prudent to obtain an activated partial thromboplastin time preoperatively for all Ashkenazi Jews, including those with a negative history for a hemostatic disorder, because of the high incidence of factor XI deficiency in their population. Surgeons and anesthesiologists should be aware of the existence of this and other uncommon hemostatic disorders and their treatment when faced with serious, apparently unexplained postoperative hemorrhage.