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Assisting individuals and families affected by BWS |
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The Beckwith-Wiedemann Children’s Association of New Zealand |
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Contact us: |
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E-mail: info@beckwith-wiedemann-nz.org
Phone: 0274 926 165 (in New Zealand) ++64-274-926-165 (Outside NZ)
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Screening Protocol for Beckwith-Wiedemann Syndrome |
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As with all screening programs there is a risk benefit ratio that has to be determined by each parent and physician for the child with BWS. Our recommendations to screen with AFP up to age 4 years and ultrasound up to 8 years is based on the observation that most but not all hepatoblastoma and Wilms tumor will occur by these ages. The decision to screen beyond these ages is an individual one, where the benefits must be weighed against the risks. The major risk of screening is a misdiagnosis of cancer resulting in inappropriate surgery. We have had three such incidents. We conducted a cost effective model describing the costs and benefits of screening for cancer in this population; although not perfect, the model coupled with the data strongly favors screening for Wilms tumor and hepatoblastoma based on the above mentioned data. Med Pediatr Oncol. 2001 Oct;37(4):349-56.
Children with BWS can develop other cancers like neuroblastoma, rhabdomyosarcoma, or adrenocortical carcinoma; however, there is no proven benefit in screening for these cancers. Fortunately they are rare in children with BWS.
Please feel free to contact me if you have any questions.
Warm regards, Michael DeBaun, M.D., MPH Principal Investigator of the BWS Registry Associate Professor of Pediatrics and Biostatistics
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A full copy of this Screening Protocol can be downloaded for printing by clicking here
A chart of expected AFP (Alpha Feto-Protein) Levels for children with Beckwith-Wiedemann Syndrome can be downloaded here
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Caption describing picture or graphic. |
Michael R. DeBaun, MD. MPH
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