Abstract

CVI is a heterogeneous complex of diseases characterized by defective B-cell maturation. This defect can result in multiple complications, including fixed obstructive lung disease, as well as intractable diarrhea, malabsorption, parasitic infestation, and an increased incidence of malignancy. Prompt recognition of the syndrome and diagnosis is essential in order to treat the protean manifestations of this disorder and, it is hoped, to prevent the irreversible sequelae.

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