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Dr. Yatin Vyas, pediatric hematologist/oncologist at Children’s Hospital of Pittsburgh of UPMC, shares the clinical and genetic aspects of Wiskott-Aldrich Syndrome.

Educational Objectives:

Upon completion of this activity, participants should be able to:

  • Identify the immunological basis for the congenital immunodeficiency disorder, Wiskott-Aldrich Syndrome (WAS)
  • Recognize that even missense mutations of WAS gene result in higher grades of disease severity that include life-threatening infections, intracranial bleeding, eczema, autoimmunity, and cancer.
  • Recognize that WASp impacts the biology of CD4 T lymphocytes through its effects both in the cytoplasm and nucleus

Reading Resources:

  1. Contributions of Wiskott-Aldrich syndrome family cytoskeletal regulatory adapters to immune regulation. Zhang J, Dong B, Siminovitch KA.  Immunol Rev. 2009 Nov;232(1):175-94. Review.
  2. WASP: a key immunological multitasker. Thrasher AJ, Burns SO.  Nat Rev Immunol. 2010 Mar;10(3):182-92. Review.
  3. WASP (Wiskott-Aldrich syndrome protein) gene mutations and phenotype. Imai K, Nonoyama S, Ochs HD. Curr Opin Allergy Clin Immunol. 2003 Dec;3(6):427-36. Review.
  4. Wiskott-Aldrich Syndrome at the nexus of autoimmune and primary immunodeficiency diseases. Cleland SY, Siegel RM. FEBS Lett. 2011 Dec 1;585(23):3710-4. Epub 2011 Oct 25. Review.
  5. Nuclear role of WASp in the pathogenesis of dysregulated TH1 immunity in human Wiskott-Aldrich syndrome. Taylor MD, Sadhukhan S, Kottangada P, Ramgopal A, Sarkar K, D'Silva S, Selvakumar A, Candotti F, Vyas YM. Sci Transl Med. 2010 Jun 23;2(37):37ra44.

Disclosures:

Dr. Vyas has no relevant relationships with any entities producing healthcare goods or services.

Accreditation Statement:

The University of Pittsburgh School of Medicine is accredited by the Accreditation Council for Continuing Medical Education (ACCME) to provide continuing medical education for physicians.
The University of Pittsburgh School of Medicine designates this enduring material for a maximum of 1.0 AMA PRA Category 1 CreditsTM. Each physician should only claim credit commensurate with the extent of their participation in the activity. Other health care professionals are awarded (0.1) continuing education units (CEU) which are equivalent to 1.0 contact hours.


For your credit transcript, please access our website 4 weeks post-completion at http://ccehs.upmc.edu and follow the link to the Credit Transcript page. If you do not provide the last 5 digits of your SSN on the next page you will not be able to access a CME credit transcript. Providing your SSN is voluntary.

Release Date: 6/5/2012 | Last Modified On: 6/5/2012 | Expires: 6/4/2013

This course has been expired.