Get the latest news, exclusives, sport, celebrities, showbiz, politics, business and lifestyle from The VeryTime,Stay informed and read the latest news today from The VeryTime, the definitive source.

Pediatric Von Willebrand Disease Differential Diagnoses

9
Pediatric Von Willebrand Disease Differential Diagnoses

Diagnostic Considerations



Other platelet function abnormalities, such as Glanzmann thrombasthenia, storage pool defects, and acquired abnormal platelet function due to medication (eg, aspirin, long-term nonsteroidal anti-inflammatory drug [NSAID] use); hyperextensibility syndromes

Differential Diagnoses

  • Bernard-Soulier Syndrome
  • Hemophilia A and B
  • Hemophilia C
  • Platelet Disorders


Workup

Suchitra S Acharya, MBBS, MD Program Head, Bleeding Disorders and Thrombosis Program, Cohen Children's Medical Center of New York; Associate Professor of Pediatrics and Pediatrics in Medicine, Hofstra North Shore-LIJ School of Medicine at Hofstra University

Suchitra S Acharya, MBBS, MD is a member of the following medical societies: American Society of Hematology, International Society on Thrombosis and Haemostasis, Hemophilia and Thrombosis Research Society, World Federation of Hemophilia

Specialty Editor Board

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

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; Associate Clinical Professor, Department of Pediatrics, Creighton University School of Medicine; 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 Society of Pediatric Hematology/Oncology, American Federation for Clinical Research, Council on Medical Student Education in Pediatrics, Hemophilia and Thrombosis Research Society, American Academy of Pediatrics, American Association for Cancer Research, American Society of Hematology

Chief Editor

Max J Coppes, MD, PhD, MBA Executive Vice President, Chief Medical and Academic Officer, Renown Heath

Max J Coppes, MD, PhD, MBA is a member of the following medical societies: American College of Healthcare Executives, American Society of Pediatric Hematology/Oncology, Society for Pediatric Research

J Martin Johnston, MD Associate Professor of Pediatrics, Mercer University School of Medicine; Director of Hematology/Oncology, The Children's Hospital at Memorial University Medical Center; Consulting Oncologist/Hematologist, St Damien's Pediatric Hospital

J Martin Johnston, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, International Society of Paediatric Oncology

Acknowledgements

John D Geil, MD Associate Professor of Pediatrics, Division of Hematology/Oncology, University of Kentucky College of Medicine; Consulting Staff, Department of Pediatric Hematology/Oncology, University of Kentucky Children's Hospital

John D Geil, MD is a member of the following medical societies: American Academy of Pediatrics, American Society of Pediatric Hematology/Oncology, and Society for Neuro-Oncology

Disclosure: Nothing to disclose.

References

  1. Schneppenheim R. The pathophysiology of von Willebrand disease: therapeutic implications. Thromb Res. 2011. 128 Suppl 1:S3-7. [Medline].
  2. Grabowski EF, Curran MA, Van Cott EM. Assessment of a cohort of primarily pediatric patients with a presumptive diagnosis of type 1 von Willebrand disease with a novel high shear rate, non-citrated blood flow device. Thromb Res. 2012 Apr. 129(4):e18-24. [Medline].
  3. Akin M. Laboratory diagnostic approach of the parents-children relationship in differentiating low-level von Willebrand factor from mild type 1 von Willebrand disease. Blood Coagul Fibrinolysis. 2012 Jun. 23(4):351-3. [Medline].
  4. Lindsay H, Bergstrom K, Srivaths L. Co-inheritance of mild hemophilia A and heterozygosity for type 2N von Willebrand disease: a diagnostic and therapeutic challenge. Pediatr Blood Cancer. 2014 Oct. 61(10):1888-90. [Medline].
  5. Bowman M, Hopman WM, Rapson D, Lillicrap D, Silva M, James P. A prospective evaluation of the prevalence of symptomatic von Willebrand disease (VWD) in a pediatric primary care population. Pediatr Blood Cancer. 2010 Mar 8. [Medline].
  6. Tosetto A, Castaman G, Rodeghiero F. Evidence-based diagnosis of type 1 von Willebrand disease: a Bayes theorem approach. Blood. 2008 Apr 15. 111(8):3998-4003. [Medline].
  7. Sidonio RF Jr, Gunawardena S, Shaw PH, Ragni M. Predictors of von Willebrand disease in children. Pediatr Blood Cancer. 2012 May. 58(5):736-40. [Medline].
  8. Rodriguez KD, Sun GH, Pike F, et al. Post-tonsillectomy bleeding in children with von Willebrand disease: a single-institution experience. Otolaryngol Head Neck Surg. 2010 May. 142(5):715-21. [Medline].
  9. Loeffelbein F, Funk D, Nakamura L, Zieger B, Grohmann J, Siepe M, et al. Shear-stress induced acquired von Willebrand syndrome in children with congenital heart disease. Interact Cardiovasc Thorac Surg. 2014 Sep 16. [Medline].
  10. [Guideline] Nichols WL, Hultin MB, James AH, et al. von Willebrand disease (VWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA). Haemophilia. 2008 Mar. 14(2):171-232. [Medline].
  11. [Guideline] Nichols WL, Rick ME, Ortel TL, et al. Clinical and laboratory diagnosis of von Willebrand disease: A synopsis of the 2008 NHLBI/NIH guidelines. Am J Hematol. 2009 Mar 16. [Medline].
  12. Batlle J, Torea J, Rendal E, Fernandez MF. The problem of diagnosing von Willebrand's disease. J Intern Med Suppl. 1997. 740:121-8. [Medline].
  13. Carcao MD, Blanchette VS, Dean JA, et al. The Platelet Function Analyzer (PFA-100): a novel in-vitro system for evaluation of primary haemostasis in children. Br J Haematol. 1998 Apr. 101(1):70-3. [Medline].
  14. Federici AB, Mannucci PM. Management of inherited von Willebrand disease in 2007. Ann Med. 2007. 39(5):346-58. [Medline].
  15. [Guideline] Lee CA, Brettler DB. Guidelines for the diagnosis and management of von Willebrand disease. Haemophilia. 1997. 3:1-25.
  16. Nichols WC, Ginsburg D. von Willebrand disease. Medicine (Baltimore). 1997 Jan. 76(1):1-20. [Medline].
  17. Sadler JE, Budde U, Eikenboom JC, et al. Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor. J Thromb Haemost. 2006 Oct. 4(10):2103-14. [Medline].
  18. Werner EJ. von Willebrand disease in children and adolescents. Pediatr Clin North Am. 1996 Jun. 43(3):683-707. [Medline].
  19. Zhang Z, Blomback M, Anvret M. Understanding von Willebrand's disease from gene defects to the patients. J Intern Med Suppl. 1997. 740:115-9. [Medline].


Structure and domains of von Willebrand factor.
Source...
Subscribe to our newsletter
Sign up here to get the latest news, updates and special offers delivered directly to your inbox.
You can unsubscribe at any time

Leave A Reply

Your email address will not be published.