02-26-2007, 05:50 PM
A 3-year-old boy is seen in your clinic because of an apparent ataxia that has been progressive for the past year. Recently he has also developed "bloodshot" eyes, which is not associated with eye drainage. His other significant history shows that he suffers from rather severe and resistant sinopulmonary infections and otitis media. An exam reveals bilateral telangiectasis of the conjunctiva. You are concerned about an immunodeficiency syndrome. Which of the following is the most accurate information to give to the family?
A. This condition is transmitted as an autosomal dominant trait; therefore a parent
should have this condition, or it is a result of a genetic mutation B. Agammaglobulinemia frequently accompanies this condition C. Thymus hypoplasia is associated with this condition D. The ataxia which occurs is usually a static condition and not progressive E. T-cell function is abnormal, and therefore lymphoproliferative disorders are usually of very low incidence
A. This condition is transmitted as an autosomal dominant trait; therefore a parent
should have this condition, or it is a result of a genetic mutation B. Agammaglobulinemia frequently accompanies this condition C. Thymus hypoplasia is associated with this condition D. The ataxia which occurs is usually a static condition and not progressive E. T-cell function is abnormal, and therefore lymphoproliferative disorders are usually of very low incidence