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Abstract: Aim: Human immunodeficiency virus (HIV)/acquired immune deficiency syndrome (AIDS) is a worldwide pandemic. Mother-to-child transmission programs should theoretically minimize vertical transfer of the virus, but with variable effectiveness of implementation a significant number of children become infected and may present for emergency, diagnostic, and elective surgery. The aim of this study was to prospectively document the clinical presentation, the spectrum of pathology, and surgical outcomes of patients presenting to our hospital. This formed part of a pilot study of a collaborative international working group studying HIV infection in children, which included the Buzzi Children''s Hospital Milan, Italy; the University of San Diego, California, USA; and the Red Cross War Memorial Children''s Hospital and University of Cape Town, School of Adolescent and Child Health, Cape Town, South Africa. Method: Clinical data from all children admitted to the surgical service of the Red Cross War Memorial Children''s Hospital between July 2004 and December 2006 with either a history of HIV exposure (born to an HIV-infected mother) or confirmation of HIV infection by ELISA or polymerase chain reaction was collected. The clinical course was documented prospectively for the duration of admission and subsequent follow-up as recorded in case records review. The spectrum of pathology, surgical intervention, outcome, complications, World Health Organization stage of AIDS, and type of antiretroviral therapy were all noted. Comparative outcomes and subgroup analysis were not done in this part of the study. Results: One hundred and thirteen patients were included in the study over the 30-month period. The average age was 24 months (1 day to 11 years). Seventy-nine (70%) of the 113 patients were infected and 34 (30%) were exposed, 9 of whom subsequently tested negative. Of the infected group, 53 (67%) patients were on antiretroviral therapy. The extent of disease in the infected group of patients according to the 2006 World Health Organization criteria was as follows: stage 1, 4 (5%); stage 2, 12 (15%); stage 3, 51 (65%); and stage 4, 12 (15%). All patients had nutritional assessments and were plotted on growth curves. Sixty-two (54%) were found to be malnourished and 41 (36%) of the children were found to have comorbid disease processes. Eighteen patients (16%) were treated with antibiotics or conservative therapy alone. The remaining 95 patients (84%) underwent an average of 1.6 procedures (range, 1-35), 59 (52%) in an elective manner and 36 (31%) as an emergency. When assessing the relationship of HIV to the presenting disease state, 58 (73.4%) had HIV-related diseases and 52 (46%) presented with sepsis. A total of 29 (25%) patients had surgical complications of which 6 (20%) were not considered to be HIV related. Nine (31%) had, in retrospect, incorrect management of the presenting disease, leaving 14 (48%) who potentially had HIV-related complications of poor wound healing and sepsis. A total of 100 (88%) were discharged alive, 6 (5.3%) died, and 7 (6 %) were lost to follow-up. Long-term follow-up of 50 patients for an average of 8 months revealed one further mortality. Conclusion: Human immunodeficiency virus–positive and –exposed patients present a unique challenge in management which is complicated by concomitant disease and poor nutrition. These patients require an expanded differential diagnosis. We believe that, although on the surface there may be a higher complication rate, this needs to be confirmed in an expanded comparative cohort study, which is underway and that patients should still receive the benefit of full surgical intervention. [Copyright &y& Elsevier]