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Wiskott-Aldrich syndrome

Wiskott-Aldrich syndrome (WAS) is a rare X-linked recessive disease characterized by eczema, thrombocytopenia (low platelet counts), immune deficiency, and bloody diarrhea (due to the low platelet counts). It is also sometimes called the eczema-thrombocytopenia-immunodeficiency syndrome in keeping with Aldrich's original description in 1954. more...

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Signs and symptoms

WAS generally becomes symptomatic in children. Due to its mode of inheritance, the overwhelming majority are male. It is characterised by bruising caused by thrombocytopenia (low platelet counts), small platelet size on blood film, eczema, recurrent infections, and a propensity for autoimmune disorders and malignancies (mainly lymphoma and leukemia).

In Wiskott-Aldrich syndrome, the platelets are small and do not function properly. They are removed by the spleen, which leads to low platelet counts. Also, patients develop a type of itchy rash called eczema. Autoimmune disorders are also found in patients with WAS.

Diagnosis

The diagnosis is made on the basis of clinical parameters, the blood film and low immunoglobulin levels. Skin immunologic testing (allergy testing) may reveal hyposensitivity. It must be remembered that not all patients will have a family history, since they may be the first to harbor the gene mutation. Often, leukemia may initially be suspected on the basis of the low platelets and the infections, and bone marrow biopsy may be performed. Decreased levels of Wiskott-Aldrich syndrome protein and/or confirmation of a causative mutation provides the most definitive diagnosis.

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Continuing education activity sponsored by CDC applying public health strategies to primary immunodeficiency diseases: a potential approach to genetic
From Morbidity and Mortality Weekly Report, 1/16/04

EXPIRATION--January 16, 2007

You must complete and return the response form electronically or by mail by January 16, 2007, to receive continuing education credit. If you answer all of the questions, you will receive an award letter for 1.5 hours Continuing Medical Education (CME) credit; 0.15 Continuing Education Units (CEUs); or 2.0 contact hours Continuing Nursing Education (CNE) credit. If you return the form electronically, you will receive educational credit immediately. If you mail the form, you will receive educational credit in approximately 30 days. No fees are charged for participating in this continuing education activity.

INSTRUCTIONS

By Internet

1. Read this MMWR (Vol. 53, RR-1), which contains the correct answers to the questions beginning on the next page.

2. Go to the MMWR Continuing Education Internet site at <http:// www.cdc.gov/mmwr/cme/conted.html>.

3. Select which exam you want to take and select whether you want to register for CME, CEU, or CNE credit.

4. Fill out and submit the registration form.

5. Select exam questions. To receive continuing education credit, you must answer all of the questions. Questions with more than one correct answer will instruct you to "Indicate all that apply."

6. Submit your answers no later than January 16, 2007.

7. Immediately print your Certificate of Completion for your records.

By Mail or Fax

1. Read this MMWR (Vol. 53, RR-1), which contains the correct answers to the questions beginning on the next page.

2. Complete all registration information on the response form, including your name, mailing address, phone number, and e-mail address, if available.

3. Indicate whether you are registering for CME, CEU, or CNE credit.

4. Select your answers to the questions, and mark the corresponding letters on the response form. To receive continuing education credit, you must answer all of the questions. Questions with more than one correct answer will instruct you to "Indicate all that apply."

5. Sign and date the response form or a photocopy of the form and send no later than January 16, 2007, to Fax: 404-639-4198 Mail: MMWR CE Credit

Office of Scientific and Health Communications

Epidemiology Program Office, MS 008

Centers for Disease Control and Prevention

1600 Clifton Rd, N.E. Atlanta, GA 30333

6. Your Certificate of Completion will be mailed to you within 30 days.

Goal and Objectives

This MMWR provides recommendations regarding public health strategies for primary immunodeficiency (PI) diseases. These recommendations were prepared by CDC staff and other specialists in PI diseases after consultation with a multidisciplinary panel. The goal of this report is to familiarize readers with a public health framework for addressing health problems resulting from a group of primarily single-gene disorders. Upon completion of this continuing education activity, the reader should be able to describe 1) the four components of a public health framework; 2) how public health assessment can be applied to PI and other genetic diseases; 3) the framework for evaluating genetic tests, including analytic validity, clinical validity, clinical utility, and ethical, legal, and social considerations; 4) two public health interventions to increase early diagnosis and treatment for genetic diseases (i.e., newborn screening and early clinical recognition); and 5) the key components of an effective health education program for PI diseases.

To receive continuing education credit, please answer all of the following questions.

COPYRIGHT 2004 U.S. Government Printing Office
COPYRIGHT 2004 Gale Group

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