Factor IX Deficiency (Hemophilia B) Questions & Answers

Updated: Mar 09, 2021
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Srikanth Nagalla, MD, MS, FACP  more...
  • Print
Questions & Answers

Overview

What is factor IX deficiency (FIX) (hemophilia B)?

When was factor IX deficiency (FIX) (hemophilia B) first differentiated from hemophilia A?

What is the structure, production, and half-life of factor IX (FIX)?

What is the role of activation in the pathophysiology of factor IX deficiency (hemophilia B)?

What are is the role of thrombin activatable fibrinolytic inhibitor in the pathophysiology of factor IX (FIX) deficiency (hemophilia B)?

What is the role of cell surface–directed hemostasis in the pathophysiology of factor IX (FIX) deficiency (hemophilia B)?

What is the prevalence of factor IX deficiency (FIX) (hemophilia B) in the US?

What is the global prevalence of factor IX deficiency (FIX) (hemophilia B)?

What is the mortality and morbidity associated with factor IX deficiency (FIX) (hemophilia B)?

What are the racial predilections of factor IX deficiency (FIX) (hemophilia B)?

What are the sexual predilections of factor IX deficiency (FIX) (hemophilia B)?

At what age is factor IX deficiency (FIX) (hemophilia B) typically diagnosed?

What are the treatment recommendations for pregnant carriers and newborns with factor IX deficiency (FIX) (hemophilia B)?

Presentation

How does the basal level of factor IX (FIX) correlate with the severity of bleeding in factor IX deficiency (FIX) (hemophilia B)?

Which clinical history findings are characteristic of factor IX deficiency (FIX) (hemophilia B)?

Which physical findings are characteristic of factor IX deficiency (FIX) (hemophilia B)?

What causes factor IX deficiency (FIX) (hemophilia B)?

What is the role of factor IX inhibitors in the etiology of factor IX deficiency (FIX) (hemophilia B)?

What is the role of combined congenital deficiencies in the etiology of factor IX deficiency (FIX) (hemophilia B)?

DDX

Which conditions are included in the differential diagnoses of factor IX deficiency (FIX) (hemophilia B)?

What are the differential diagnoses for Factor IX Deficiency (Hemophilia B)?

Workup

What is the role of lab tests in the workup of factor IX deficiency (FIX) (hemophilia B)?

What is the role of imaging studies in the workup of factor IX deficiency (FIX) (hemophilia B)?

What is the role of ECG in the workup of factor IX deficiency (FIX) (hemophilia B)?

When is FIX replacement required for patients with factor IX deficiency (FIX) (hemophilia B) undergoing routine procedures?

Treatment

How is factor IX deficiency (FIX) (hemophilia B) treated?

What is the role of fibrinolytic inhibitors in the treatment of factor IX deficiency (FIX) (hemophilia B)?

What is the role of analgesics in the treatment of factor IX deficiency (FIX) (hemophilia B)?

What is the role of gene therapy in the treatment of factor IX deficiency (FIX) (hemophilia B)?

What is included in the care of patients with factor IX deficiency (FIX) (hemophilia B) undergoing surgical procedures?

Which consultations are beneficial to patients with factor IX deficiency (FIX) (hemophilia B)?

Which activity modifications are used in the treatment of factor IX deficiency (FIX) (hemophilia B)?

Medications

What is the role of medication in the treatment of factor IX deficiency (FIX) (hemophilia B)?

Which medications in the drug class Antifibrinolytics are used in the treatment of Factor IX Deficiency (Hemophilia B)?

Which medications in the drug class Coagulation Factors are used in the treatment of Factor IX Deficiency (Hemophilia B)?

Follow-up

What is included in the long-term monitoring of factor IX deficiency (FIX) (hemophilia B)?

When is inpatient care indicated for factor IX deficiency (FIX) (hemophilia B)?

What are the benefits of home therapy for the treatment of factor IX deficiency (FIX) (hemophilia B)?

Which medications are contraindicated in patients with factor IX deficiency (FIX) (hemophilia B)?

When is patient transfer considered for the treatment of factor IX deficiency (FIX) (hemophilia B)?

What are the potential complications of factor IX deficiency (FIX) (hemophilia B)?

What is the prognosis of factor IX deficiency (FIX) (hemophilia B)?

What is included in patient education about factor IX deficiency (FIX) (hemophilia B)?