Factor XIII Deficiency Questions & Answers

Updated: Apr 02, 2018
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Perumal Thiagarajan, MD  more...
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Questions & Answers

Overview

What is factor XIII (FXIII) deficiency?

How does the site of bleeding affect the physical findings of factor XIII (FXIII) deficiency?

What are the signs and symptoms of factor XIII (FXIII) deficiency?

What are the signs and symptoms of joint bleeding in factor XIII (FXIII) deficiency?

Which lab tests are performed in the workup of factor XIII (FXIII) deficiency?

What is included in the qualitative screening test for severe factor XIII (FXIII) deficiency?

What is the role of quantitative testing in the workup of factor XIII (FXIII) deficiency?

What is included in inhibitor testing for factor XIII (FXIII) deficiency?

How is factor XIII (FXIII) deficiency diagnosed prenatally?

What is the role of FXIII replacement in factor XIII (FXIII) deficiency?

Which replacement factor XIII concentrates are used in the treatment of factor XIII (FXIII) deficiency?

How is factor XIII (FXIII) deficiency treated in patients with factor XIII inhibitors?

How was factor XIII (FXIII) deficiency first recognized?

What is the structure, production, and half-life of factor XIII (FXIII)?

What is the role of activation in the pathophysiology of factor XIII (FXIII) deficiency?

What is the role of factor XIII (FXIII) cross-linking and resistance to lysis in the pathophysiology of factor XIII (FXIII) deficiency?

Which factors affect the level and activity of factor XIII (FXIII) in the pathophysiology of factor XIII (FXIII) deficiency?

How does factor XIII (FXIII) deficiency affect pregnancy outcomes?

How does factor XIII (FXIII) deficiency affect wound healing?

What is the role of nitric oxide (NO) in the pathophysiology of factor XIII (FXIII) deficiency?

What is the role of venom and toxins in the pathophysiology of factor XIII (FXIII) deficiency?

What is the role of simvastatin in the pathophysiology of factor XIII (FXIII) deficiency?

What is the role of synthetic inhibitors in the pathophysiology of factor XIII (FXIII) deficiency?

What is the role of plasma and tissue transglutaminases in the pathophysiology of factor XIII (FXIII) deficiency?

What is the role of factor XIII (FXIII) deficiency in the pathophysiology of degenerative brain disorders?

What is the role of thrombin-activatable fibrinolytic inhibitor in the pathophysiology of factor XIII (FXIII) deficiency?

What is the role of cell surface–directed hemostasis, in the pathophysiology of factor XIII (FXIII) deficiency?

What is the prevalence of factor XIII (FXIII) deficiency in the US?

What is the global prevalence of factor XIII (FXIII) deficiency?

What is the mortality and morbidity associated with factor XIII (FXIII) deficiency?

Which patient groups have the highest prevalence of factor XIII (FXIII) deficiency?

Presentation

Which clinical history findings are characteristic of factor XIII (FXIII) deficiency?

Which physical findings are characteristic of factor XIII (FXIII) deficiency?

What causes factor XIII (FXIII) deficiency?

What is the role of the Val34Leu mutation in the etiology of factor XIII (FXIII) deficiency?

What is the role of genetic polymorphisms in the etiology of factor XIII (FXIII) deficiency?

What is the role of disorders of fibrin stabilization in the etiology of factor XIII (FXIII) deficiency?

DDX

Which conditions are included in the differential diagnoses of factor XIII (FXIII) deficiency?

Which disorders are included in the differential diagnoses of acquired factor XIII (FXIII) deficiency?

Which factor XIII (FXII) inhibitors are included in the differential diagnoses of factor XIII (FXIII) deficiency?

What are the differential diagnoses for Factor XIII Deficiency?

Workup

What is included in the initial workup of factor XIII (FXIII) deficiency?

Which findings on a qualitative screening test suggest factor XIII (FXIII) deficiency?

What is the stepped approach to diagnostic testing of factor XIII (FXIII) deficiency?

How is a diagnosis of acquired factor XIII (FXIII) deficiency confirmed?

Which tests are performed in the prenatal workup of factor XIII (FXIII) deficiency?

What is the role of liver function tests in the workup of factor XIII (FXIII) deficiency?

Which imaging studies are included in the workup of factor XIII (FXIII) deficiency?

When is ECG performed in the workup of factor XIII (FXIII) deficiency?

What procedures are included in the prenatal workup of factor XIII (FXIII) deficiency?

What is the role of colonoscopy and endoscopy in the workup of factor XIII (FXIII) deficiency?

Treatment

How is factor XIII (FXIII) deficiency treated?

What are the reported uses of factor XIII (FXIII) in unusual circumstances?

How is acquired factor XIII (FXIII) deficiency treated?

What is the role of prophylactic factor replacement in the treatment of factor XIII (FXIII) deficiency?

What advancements have been made in the storage and transport of factors used in factor XIII (FXIII) deficiency treatment?

What is the role of PLAS+S in the treatment of factor XIII (FXIII) deficiency?

What is the role of antifibrinolytic agents in the treatment of factor XIII (FXIII) deficiency?

What is the role of NSAIDs in the treatment of factor XIII (FXIII) deficiency?

Which routine preventive care should be provided to patients with factor XIII (FXIII) deficiency?

What is the role of gene therapy in the treatment of factor XIII (FXIII) deficiency?

What is included in perioperative management of patients with factor XIII (FXIII) deficiency undergoing routine surgical procedures?

Which specialist consultations are beneficial to patients with factor XIII (FXIII) deficiency?

Which dietary modifications are used in the treatment of factor XIII (FXIII) deficiency?

Which activity modifications are used in the treatment of factor XIII (FXIII) deficiency?

Medications

What is the role of medications in the treatment of factor XIII (FXIII) deficiency?

Which medications in the drug class Antifibrinolytic agents are used in the treatment of Factor XIII Deficiency?

Which medications in the drug class Blood Products are used in the treatment of Factor XIII Deficiency?

Which medications in the drug class Clotting factors are used in the treatment of Factor XIII Deficiency?

Follow-up

What is included in long-term monitoring of factor XIII (FXIII) deficiency?

When is inpatient care indicated in the treatment of factor XIII (FXIII) deficiency?

Which medications are contraindicated in factor XIII (FXIII) deficiency?

When is patient transfer indicated for the treatment of factor XIII (FXIII) deficiency?

How are exacerbations and complications of factor XIII (FXIII) deficiency prevented?

What are the possible complications of factor XIII (FXIII) deficiency?

What is the prognosis of factor XIII (FXIII) deficiency?

What is included in the patient education about treatment of factor XIII (FXIII) deficiency?