Background SCID is a syndrome characterized by profound T cell deficiency. BCG vaccine is contraindicated in SCID patients. Because most countries encourage BCG vaccination at birth, a high percent of SCID patients are vaccinated before their immune defect is detected. Objectives To describe the complications and risks associated with BCG vaccination in SCID patients. Methods An extensive standardized questionnaire evaluating complications, therapeutics, and outcome regarding BCG in patients diagnosed with SCID was widely distributed. Summary statistics and association analysis was performed. Results Data on 349 BCG vaccinated SCID patients from 28 centers in 17 countries was analyzed. Fifty-one percent of the patients developed BCG complications, 34% disseminated and 17% localized (a 33,000 and 400 fold increase, respectively, over the general population). Patients receiving early vaccination (≤ 1 month) showed an increased prevalence of complications (p=0.006) and death due to BCG complications (p<0.0001). The odds of experiencing complications among patients with T cells ≤ 250/uL at diagnosis was 2.1 times higher (95% CI, 1.4-3.4; p = 0.001) than among those with T cells > 250/uL. BCG complications were reported in 2/78 patients who received anti-mycobacterial therapy while asymptomatic and no deaths due to BCG complications occurred in this group. In contrast 46 BCG-associated deaths were reported among 160 patients treated with anti-mycobacterial therapy for a symptomatic BCG infection (p<0.0001). Conclusions BCG vaccine has a very high rate of complications in SCID patients, which increase morbidity and mortality rates. Until safer and more efficient anti-tuberculosis vaccines become available, delay in BCG vaccination should be considered to protect highly vulnerable populations from preventable complications.

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Objectives. To translate into Argentine Spanish and cross-culturnlly adapt the Childhood Health Assessment Questionnaire [CHAQ) and validate the adapted in strum en t in Argentine p a tien ts with ju venile rheumatoid arthritis [JRA). Methods. Five bilingual Argentine pediatric rheuniatologists translated into Argentine Spanish and cross-culturally adapted the United States English CHAQ. Pretesting was done in a sample of 23 INTRODUCTIONJuvenile rheumatoid arthritis (JRA) is a pediatric illness characterized by chronic inflammation of the joints and, in some situations, extra-articular manifestations such as iridocyclitis, pericarditis, and lymphadenopathy. Current treatment is ameliorative rather than curative. The disease may produce substantive changes in the child's ability to carry on the usual activities of daily living. Functional limitations may be minor, such as inability to fully extend an extremity. More severe limitations may interfere with activities in several domains, such as dressing and grooming, arising, eating, ambulation, and grip (I).The importance of assessing the impact of arthritis on functional ability in routine practice as well as therapeutic trials is now well recognized. For exam- Several instruments have been developed to measure functional ability in patients with adult rheumatoid arthritis. One of the most popular and widely used is the Stanford Health Assessment Questionnaire (HAQ. This self-administered questionnaire has undergone extensive validation in over 30 studies and has been administered well over 100,000 times in national and international studies (4). The HAQ attempts to quantitatively describe disability in terms that represent the patient's perceptions of his or her condition. Disability is assessed in 8 areas: dressing and grooming, arising, eating, walking, hygiene, reach, grip, and activities.The disability section of the HAQ was adapted for use in children by adding several new questions so that for each of the 8 functional areas there is at least 1 question that is relevant to children of all ages (5). The adapted instrument is referred to as the childhood HAQ, or CHAQ. It was designed as a selfadministered questionnaire for the parents of patients with JRA. It has also been given successfully as a self-administered questionnaire to children as young as 8 years of age (5-7). To eliminate discrepancies introduced by growth and development, parents are asked to note only those difficulties that are caused by arthritis. Each question is scored from 0 to 3: 0 = no difficulty, 1 = some difficulty, 2 = much difficulty, 3 = unable to do. The question with the highest score determines the score for that functional area. If aids or devices are used or help is needed to complete tasks in a certain area, a minimum score of 2 is recorded for the corresponding functional area. The scores for each of the 8 functional areas are averaged to calculate the disability index (DI). Thus, possible scores of the DI range from 0 to 3, with lower scores representing better functional ...

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This study evaluated the success of a national program for the prevention of mother-to-child transmission (MTCT) of HIV-1 in 874 mother-infant pairs from Buenos Aires and surroundings. This population was referred to the National Reference Center for AIDS for diagnosis of neonatal infection during 1993-2000. The data revealed an increase in the use of antiretroviral therapy during pregnancy from 3.2% in 1993-1994 to 73.1% in 1999-2000 and in the use of cesarean delivery (reaching 54.8% in 1999-2000). However, the proportion of HIV-infected women who continued to breast-feed their children remained steady (around 12%). General improvement of the conditions for decreasing MTCT resulted in a significant decrease in the proportion of infected infants from 37.3% before 1995 to 10.7% in 1999-2000 and even 6.5% during 2001. Data on the time of diagnosis indicated that only 42.7% of the women knew about their HIV status before pregnancy, 44.8 knew during pregnancy, and 12.3% knew after the birth of their child. The main risk factor for HIV infection in the mothers was heterosexual contact (73%), and in the fathers, it was injection drug use (67%). These results point out the urgent need to develop additional strategies for prevention of MTCT of HIV-1 to generalize education, counseling, and testing of young women.