A Descriptive Survey of Pediatric Human Immunodeficiency Virus–infected Long-term Survivors Karin Nielsen, MD, MPH*; George McSherry, MD; Ann Petru, MD**; Toni Frederick, PhD§; Diane Wara, MD‡‡; Yvonne Bryson, MD*; Natasha Martin, FMIBS‡; Cecelia Hutto, MD#; Arthur J. Ammann, MD‡; Samuel Grubman, MD¶; James Oleske, MD, MPH; and Gwendolyn B. Scott, MD# ABSTRACT. Objective. To identify the population of human immunodeficiency virus–infected pediatric long- term survivors (LTS) followed in major medical institu- tions in California, Florida and New Jersey. Methods. A cross-sectional survey was performed with data collection forms sent to all investigators. De- mographic, clinical, and laboratory data were obtained on all living patients 8 years infected in the perinatal period with human immunodeficiency virus. Results. A total of 143 perinatally infected and 54 children infected by neonatal transfusion were identi- fied. Fifty-four children (27%) had absolute CD4 counts 500 cells/mm 3 (group 1: mean age 9.8 years), 54 children (27%) had CD4 counts between 200 and 500 cells/mm 3 (group 2: mean age 10.1 years), and 89 children (45%) had CD4 counts <200 cells/mm 3 (group 3: mean age 10.4 years). Ninety-five (48%) patients had developed AIDS defining conditions; 14 (26%) in group 1, 26 (48%) in group 2, and 55 (62%) in group 3. Ninety-two percent of patients had received antiretrovirals. Perinatally human immunodeficiency virus-infected children tended to be younger (mean age 9.8 years) than children infected via a blood transfusion (mean age 11 years). Generalized lymphadenopathy was the most prevalent clinical find- ing. Lymphoid interstitial pneumonia and recurrent bac- terial infections were the most prevalent acquired im- mune deficiency syndrome-defining conditions. Twenty percent of LTS had CD4 counts 500 cells/mm 3 and no immune deficiency syndrome-defining conditions. Conclusions. Pediatric LTS were in variable stages of disease progression. The proportion of children within each CD4 strata did not differ by mode of acquisition of infection. Increased CD4 counts were inversely propor- tional to age. Only 20% of pediatric LTS had minimal to no disease progression. Pediatrics 1997;99(4). URL: http: //www.pediatrics.org/cgi/content/full/99/4/e4; HIV, pedi- atric long-term survivors, slow disease progression. ABBREVIATIONS. HIV-1, human immunodeficiency virus type 1; AIDS, acquired immunodeficiency syndrome; LTNS, long-term nonprogressive survivors; LTPS, long-term progressive survivors; LIP, lymphoid interstitial pneumonitis. Human immunodeficiency virus type 1 (HIV-1) disease in the pediatric population progresses more rapidly than in adults and has a bimodal distribution of disease presentation. 1,2 The group of children who develop early and severe disease, frequently with the development of acquired immunodeficiency syn- drome (AIDS)– defining illnesses and with a higher likelihood of death in the first years of life, have been identified as rapid progressors. 2 There is, however, a group of children who have survived for many years after the diagnosis of HIV infection. In this group, two populations have emerged: the long-term non- progressive survivors (LTNS), who have remained asymptomatic or only mildly symptomatic over a period of years, and those who have survived de- spite clinical and laboratory evidence of disease progression, the long-term progressive survivors (LTPS). The availability of antiretroviral therapy and early medical intervention with prophylaxis and treatment of infections has altered to some degree the natural history of HIV infection and increased survival time of infected patients. 3 Long-term survi- vors provide a unique opportunity to investigate the immunologic, virologic, and genetic characteristics of HIV-infected children to determine what factors may slow progression to disease and death. In October 1993, the Pediatric AIDS Foundation sponsored a workshop on HIV-infected pediatric long-term survivors with the participation of inves- tigators from seven research institutions. The work- shop focused on a discussion of virological, immu- nological, and genetic factors that potentially could contribute to long-term survival. As a result of this workshop, investigators representing five large uni- versity affiliated medical centers nationwide volun- teered to cooperate in a collaborative study of their patient populations. We summarize the results of an initial survey conducted at these sites of children with perinatal HIV infection or with transfusion- associated HIV-acquired infection describing their demographics, clinical findings, immunological sta- tus, and antiretroviral therapy. From the *Department of Pediatrics, UCLA School of Medicine, Los Ange- les, California; ‡Pediatric AIDS Foundation, Novato, California; §Pediatric AIDS Surveillance Study, Los Angeles Pediatric AIDS Consortium, Los Angeles County Dept of Health Services, Los Angeles, California; Depart- ment of Pediatrics, UMD–New Jersey Medical School and Children’s Hos- pital of New Jersey, Newark, New Jersey; ¶Department of Pediatrics, Saint Vincents Hospital and Medical Center of New York, New York; #Depart- ment of Pediatrics, University of Miami School of Medicine, Miami, Florida; **Children’s Hospital Oakland, Oakland, California; and ‡‡Department of Pediatrics, University of California, San Francisco School of Medicine, San Francisco, California. Received for publication Apr 24, 1996; accepted Jul 15, 1996. Reprint requests to (G.B.S.) University of Miami School of Medicine, De- partment of Pediatrics D4-4, PO Box 016960, Miami, FL 33101. PEDIATRICS (ISSN 0031 4005). Copyright © 1997 by the American Acad- emy of Pediatrics. http://www.pediatrics.org/cgi/content/full/99/4/e4 PEDIATRICS Vol. 99 No. 4 April 1997 1 of 7 by guest on July 11, 2017 Downloaded from