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BE SURE: Know the signs of ADA-SCID

Recurrent ear infection or sinusitis that doesn’t resolve, along with other symptoms like persistent diarrhea, pneumonia, or thrush, are classic symptoms of ADA-SCID.1-5 When puzzled by recurring severe infection and a child’s failure to grow, apply the simple acronym BE SURE. If the child displays this infection pattern, he or she should be referred for screening.

chart-Be-Sure

ADA-SCID can be detected with a simple blood test.4 The earlier ADA-SCID is detected and treated, the better the outcome.1-5

A family history of unexplained infant death, or infant death due to infection may indicate an inherited immunodeficiency, such as severe combined immunodeficiency disease (SCID).3 When there is a history of affected male relatives, X-linked SCID, the most common form of the disorder, may be suspected.6

References: 1. National Human Genome Research Institute. Learning about severe combined immunodeficiency (SCID). Available at http://www.genome.gov/13014325. Accessed February 28, 2011.
2. Buckley RH, Schiff RI, Schiff SE, et al. Human severe combined immunodeficiency: genetic, phenotypic, and functional diversity in one hundred eight infants. J Pediatr. 1997;130:378-387. 3. Schwartz RA, Sinha S. Pediatric severe combined immunodeficiency. Available at http://emedicine.medscape.com/article/888072-overview. Accessed February 28, 2011. 4. Hershfield MS, Mitchell BS. Immunodeficiency diseases caused by adenosine deaminase deficiency and purine nucleoside phosphorylase deficiency. In: Scriver CR, Beaudet AL, Sly WS, Valle D, eds. The Metabolic and Molecular Bases of Inherited Disease. 7th ed. New York, NY: McGraw-Hill; 1995:1725-1768. 5. Gennery AR, Cant AJ. Diagnosis of severe combined immunodeficiency.
J Clin Pathol. 2001;54:191–195. 6. Blaese MR, Winkelstein JA, eds. Patient & Family Handbook for Primary Immunodeficiency Diseases. 4th ed. Towson, MD: Immune Deficiency Foundation; 2007.