endocrine and metabolic disorders
IPEX Syndrome
Introduction
IPEX (immune dysregulation, polyendocrinopathy, enteropathy, X-linked) is an X-linked recessive syndrome involving aggressive autoimmunity. , which causes regulatory T-cell dysfunction and a subsequent Autoimmune Disorders ( IPEX syndrome manifests as severe enlargement of the secondary lymphoid organs, type 1 Diabetes Mellitus (DM) , Diagnosis of IPEX syndrome is suggested by clinical features and confirmed by genetic analysis. Genetic counseling and targeted genetic testing for siblings and offspring should be considered ( General references ). 