Unexplained Immunodeficiency in Children

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Abstract
From Oct 1, 1982, to Oct 1, 1983, the Centers for Disease Control (CDC) received reports of 35 children whose illness met the CDC definition of acquired immune deficiency syndrome (AIDS). All of the children had serious opportunistic infections without a known underlying illness to explain susceptibility to the infections. The 35 children were residents of ten different states; cases clustered in five major metropolitan areas. Three of the children had a parent with AIDS, and one child who had been previously reported had received a blood transfusion from a person in whom AIDS later developed. Most of the children had at least one parent in a population group in which adult AIDS cases have occurred. Many of the children had histories of prodromal symptoms, including pneumonitis, lymphadenopathy, hepatomegaly, and oral thrush. The mean age at onset of illness was 5 months, and the mean age at diagnosis was 12 months. To determine whether opportunistic infection in children without underlying immunodeficiency was truly a new phenomenon, a review of requests to the CDC for the drug pentamidine isethionate was undertaken. This revealed an apparent increase from 1979 to 1983 inPneumocystis cariniipneumonia in children without known underlying immunodeficiency. (JAMA1984;252:639-644)