Congenital immunodeficiencies are relatively rare disorders that result in
a marked predisposition to infection and to a variety of gastrointestinal p
roblems. Increasingly recognized in both children and adults, accumulating
worldwide experience with these disorders reveals that the long-term progno
sis is poor in many cases, despite available treatments. This review highli
ghts the past year's advances concerning the molecular basis of several of
these disorders, new diagnostic considerations, and novel therapeutic appro
aches concerning their gastrointestinal complications. The availability of
molecular diagnostic tools increases the options for earlier definitive tre
atments such as bone marrow transplantation and somatic gene therapy. Curr
Opin Gastroenterol 2001, 17:551-554 (C) 2001 Lippincott Williams & Wilkins,
Inc.