JAMA · 1983 · 410 citations · 32 references
MedicineImmunologyPediatricsClinical ImmunologyImmunopathologyPediatric ImmunologyImmunocompromised Host InfectionsImmunodeficiency DisordersNeonatal ImmunologyPediatric Clinical ImmunologyPregnancy ImmunologySexually Transmitted InfectionsAcquired Immunodeficiency SyndromeEpstein-barr Virus PersistenceMaternal Human Immunodeficiency Virus Infection
A new syndrome of acquired immunodeficiency has been identified in seven children who were small for gestational age at birth and subsequently have exhibited failure to thrive, lymphadenopathy, parotitis, hepatosplenomegaly, interstitial pneumonia, and recurrent infections. All have a profound cell-mediated immunodeficiency with reversed T4/T8 ratios. Six are hypergammaglobulinemic and one has low IgG levels. The mothers of five of the seven children are sexually promiscuous and/or drug addicts. Three mothers have an immunodeficiency similar to that found in their infants. One of them died at age 33 years with a diagnosis of acquired immunodeficiency syndrome. In five of the children and in three of their mothers, there is evidence of a persistent Epstein-Barr virus (EBV) infection. We speculate that a perinatal or in utero transmission of EBV can induce an "infectious immunodeficiency." The clinical, histopathologic, and immunologic features resemble those described in adult homosexuals and drug addicts.
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