A Long-Term Survivor with the Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked Syndrome

Abstract

To the Editor: In their report of bone marrow transplanta- tion in a case of the immune dysregulation, polyendocri- nopathy, enteropathy, X-linked syndrome (IPEX), Baud et al. (June 7 issue)1 incorrectly stated that our patient (described in our 1995 report2 ) had died. On the contrary, he survived, is now 11 years old, and continues to receive treatment with betamethasone and tacrolimus.2 The diagnosis of IPEX has been confirmed by sequence analysis of the FOXP3 gene. He has various complications, including renal tubular dam- age, severe osteoporosis with multiple compression fractures of vertebrae, short stature, hypotonicity, and cataracts, which