What is the prognosis for DiGeorge syndrome?
The prognosis is variable; many infants with DiGeorge syndrome die from overwhelming infection, seizures, or heart failure within the first year. One study of a series of 558 patients reported 8% mortality within six months of birth, with heart defects accounting for all but one of the deaths. Infections resulting from severe immune deficiency are the second most common cause of death in patients with DGA. Advances in heart surgery indicate that the prognosis is most closely linked to the severity of the heart defects and the partial presence of the thymus gland. In most children who survive, the number of T cells, a type of white blood cell, in the blood rises spontaneously as they mature. Survivors are likely to be mentally retarded, however, and to have other developmental difficulties, including seizures or other psychiatric and neurological problems in later life. Source: The Gale Group.
