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Severe recurrent and uncontrolled Ig overproduction is increasingly recognised as a cause of life-threatening illness in otherwise healthy children, and is associated with repeated infections, failure to thrive, cataract and corneal opacities. Over the last two decades, the clinical characteristics and the genetic causes of the syndrome have been clarified, and novel treatments have become available. However, many questions remain as to the detailed pathogenesis of this disorder, including the requirements for the stimulatory signals and the requisite degree of B-cell activation. Gene therapy, which may now be available, holds great promise as a definitive treatment in this group of patients. UNPUBLISHED
UNITED STATES COURT OF APPEALS
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