eMedicine Specialties > Pediatrics: General Medicine > Hematology

Inherited Abnormalities of Fibrinogen: Follow-up

Author: Sara J Israels, MD, FRCPC, Professor of Pediatric Hematology/Oncology, Section Head of Pediatric Hematology/Oncology/BMT, Department of Pediatrics and Child Health, University of Manitoba
Contributor Information and Disclosures

Updated: Feb 12, 2009

Follow-up

Further Outpatient Care

  • Ideally, individuals with afibrinogenemia and symptomatic dysfibrinogenemias should be monitored by a comprehensive bleeding disorder care team experienced in diagnosing and managing inherited bleeding disorders.

Deterrence/Prevention

  • Individuals who may require plasma-derived coagulation factor concentrates should be immunized with the hepatitis A and hepatitis B vaccine.
  • Patients should avoid taking aspirin and other drugs that affect platelet function.
  • Consider prophylactic therapy for patients with recurrent bleeding episodes, CNS hemorrhage, or during pregnancy for women with recurrent miscarriage.

Patient Education

  • Patients and families should be provided with instruction and educational materials to enhance understanding of their coagulation disorder, improve their ability to recognize the symptoms and signs of bleeding and/or thrombosis, and to identify emergency situations.
  • Patients should know how to contact their treatment center for immediate treatment and where to go to receive emergency care.
  • Patients should wear a MedicAlert bracelet or carry other identification of their hemostatic disorder and recommended therapy.

Miscellaneous

Medicolegal Pitfalls

  • Failure to make a specific diagnosis of either congenital afibrinogenemia or dysfibrinogenemia
  • Failure to investigate other family members for the abnormality identified in the propositus
  • Failure to appropriately treat bleeding episodes with fibrinogen replacement in patients with afibrinogenemia
  • Failure to provide fibrinogen replacement prior to surgical procedures in patients with afibrinogenemia

Special Concerns

  • In pregnant patients, recurrent spontaneous abortion may be prevented by routine prophylaxis with fibrinogen concentrate.
  • Patients may be at risk of thrombosis, even if their primary clinical presentation is bleeding. Administration of fibrinogen replacement may increase thrombotic risk, particularly in settings that are prothrombotic such as surgery.
 


More on Inherited Abnormalities of Fibrinogen

Overview: Inherited Abnormalities of Fibrinogen
Differential Diagnoses & Workup: Inherited Abnormalities of Fibrinogen
Treatment & Medication: Inherited Abnormalities of Fibrinogen
Follow-up: Inherited Abnormalities of Fibrinogen
Multimedia: Inherited Abnormalities of Fibrinogen
References
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References

  1. Haverkate F, Samama M. Familial dysfibrinogenemia and thrombophilia. Report on a study of the SSC Subcommittee on Fibrinogen. Thromb Haemost. Jan 1995;73(1):151-61. [Medline].

  2. Asselta R, Duga S, Tenchini ML. The molecular basis of quantitative fibrinogen disorders. J Thromb Haemost. Oct 2006;4(10):2115-29. [Medline].

  3. Bolton-Maggs PH, Perry DJ, Chalmers EA, et al. The rare coagulation disorders--review with guidelines for management from the United Kingdom Haemophilia Centre Doctors' Organisation. Haemophilia. Sep 2004;10(5):593-628. [Medline].

  4. Cunningham MT, Brandt JT, Laposata M, Olson JD. Laboratory diagnosis of dysfibrinogenemia. Arch Pathol Lab Med. 2002;126:499-505. [Medline].

  5. Martinez J. Congenital dysfibrinogenemia. Curr Opin Hematol. Sep 1997;4(5):357-65. [Medline].

  6. Parameswaran R, Dickinson JP, de Lord S, et al. Spontaneous intracranial bleeding in two patients with congenital afibrinogenaemia and the role of replacement therapy. Haemophilia. Nov 2000;6(6):705-8. [Medline].

  7. Peyvandi F, Haertel S, Knaub S, Mannucci PM. Incidence of bleeding symptoms in 100 patients with inherited afibrinogenemia or hypofibrinogenemia. J Thromb Haemost. Jul 2006;4(7):1634-7. [Medline].

  8. Roberts HR, Stinchcombe TE, Gabriel DA. The dysfibrinogenaemias. Br J Haematol. Aug 2001;114(2):249-57. [Medline].

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Further Reading

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Keywords

afibrinogenemia, congenital afibrinogenemia, hypofibrinogenemia, dysfibrinogenemia, congenital dysfibrinogenemia, fibrinogen deficiency, fibrinogen abnormalities, clotting disorder, blood disorder, inherited abnormalities of fibrinogen, defective fibrinogen synthesis, venous thrombosis, factor XIII deficiency

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Contributor Information and Disclosures

Author

Sara J Israels, MD, FRCPC, Professor of Pediatric Hematology/Oncology, Section Head of Pediatric Hematology/Oncology/BMT, Department of Pediatrics and Child Health, University of Manitoba
Sara J Israels, MD, FRCPC is a member of the following medical societies: American Society of Hematology, American Society of Pediatric Hematology/Oncology, Canadian Medical Association, Children's Oncology Group, International Society on Thrombosis and Haemostasis, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Medical Editor

Gary R Jones, MD, Associate Medical Director, Clinical Development, Berlex Laboratories
Gary R Jones, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, and Western Society for Pediatric Research
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from financial planner; Avanir Pharma Stock Investment from financial planner ; WebMD Salary and stock Employment and investment from financial planner

Managing Editor

James L Harper, MD, Associate Professor, Department of Pediatrics, Division of Hematology/Oncology and Bone Marrow Transplantation, Associate Chairman for Education, Department of Pediatrics, University of Nebraska Medical Center; Assistant Clinical Professor, Department of Pediatrics, Creighton University; Director, Continuing Medical Education, Children's Memorial Hospital; Pediatric Director, Nebraska Regional Hemophilia Treatment Center
James L Harper, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Federation for Clinical Research, American Society of Hematology, American Society of Pediatric Hematology/Oncology, Council on Medical Student Education in Pediatrics, and Hemophilia and Thrombosis Research Society
Disclosure: Nothing to disclose.

CME Editor

Helen SL Chan, MBBS, FRCP(C), FAAP, Senior Scientist, Research Institute; Professor, Division of Hematology/Oncology, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, Canada
Helen SL Chan, MBBS, FRCP(C), FAAP is a member of the following medical societies: American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology, and Royal College of Physicians and Surgeons of Canada
Disclosure: Nothing to disclose.

Chief Editor

Max J Coppes, MD, PhD, MBA, Executive Director, Center for Cancer and Blood Disorders, Children's National Medical Center; Professor of Medicine, Oncology, and Pediatrics, Georgetown University
Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American Association for Cancer Research, American Society of Pediatric Hematology/Oncology, and Society for Pediatric Research
Disclosure: Nothing to disclose.

 
 
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