title: Early progression of disease in HIV-infected infants with thymus dysfunction.
abstract: BACKGROUND
  Infants with congenital thymic deficiency (the DiGeorge syndrome) have immunodeficiency and a characteristic pattern of low CD4+ and CD8+ T-lymphocyte counts and low CD5+ B-lymphocyte counts. Because the thymus is essential for the generation of CD4+ cells, we sought evidence of thymus dysfunction in infants infected perinatally with the human immunodeficiency virus (HIV).
  
  
  METHODS
  We studied the immunophenotypes of 59 infants with maternally transmitted HIV, 5 infants with the DiGeorge syndrome, and 168 infants exposed to HIV but not infected. The criteria for a presumed thymic defect were reductions in both the CD4+ and CD8+ T-cell subgroups during the first six months of life that were confirmed in a subgroup of infants by low counts of CD4+CD45RA+ and CD4+CD45RO+ T cells and CD5+ B cells.
  
  
  RESULTS
  Of the 59 HIV-infected infants, 17 had immunophenotypes similar to those of infants with the DiGeorge syndrome. The risks of the acquired immunodeficiency syndrome (AIDS) by the ages of 12 and 24 months were 75 percent and 92 percent in these 17 infants, as compared with 14 and 34 percent in the other 42 infants (P<0.001). Nine of the HIV-infected infants with the DiGeorge-like immunophenotype (53 percent) died within six months of the progression to AIDS, as compared with only three of the other infants (7 percent, P=0.006).
  
  
  CONCLUSIONS
  In some infants infected perinatally with HIV, a pattern of lymphocyte depletion develops that resembles the pattern in congenital thymic deficiency. Since HIV disease progresses rapidly in such infants, they may be candidates for early antiviral therapy and attempts at immune reconstitution.
authors.affiliation: Department of Pediatrics, Emory University, Atlanta, GA 30303, USA.
authors.forename: A P
authors.initials: AP
authors.lastname: Kourtis
authors.forename: C
authors.initials: C
authors.lastname: Ibegbu
authors.forename: A J
authors.initials: AJ
authors.lastname: Nahmias
authors.forename: F K
authors.initials: FK
authors.lastname: Lee
authors.forename: W S
authors.initials: WS
authors.lastname: Clark
authors.forename: M K
authors.initials: MK
authors.lastname: Sawyer
authors.forename: S
authors.initials: S
authors.lastname: Nesheim
mesh_terms: D000163:Acquired Immunodeficiency Syndrome / Q000276:immunology* / Q000401:mortality; D004062:DiGeorge Syndrome / Q000276:immunology*; D018450:Disease Progression; D005260:Female; D015658:HIV Infections / Q000276:immunology* / Q000635:transmission; D006801:Humans; D016130:Immunophenotyping; D007223:Infant; D018445:Infectious Disease Transmission, Vertical; D018655:Lymphocyte Count; D008297:Male; D011446:Prospective Studies; D013950:Thymus Gland / Q000276:immunology* / Q000503:physiopathology