New developments in primary immunodeficiencies: Report on the 2006 CIS Primary Immunodeficiency Diseases Consortium Conference, June 1, 2006.
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Biomedical subjects
Publications and source records attributed to Kathleen Sullivan.
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X-linked agammaglobulinemia (XLA) is a primary immunodeficiency caused by mutations in the gene for Bruton tyrosine kinase (BTK) that result in the deficient development of B lymphocytes and hypogammaglobulinemia. Because the disorder is uncommon, no single institution has had sufficient numbers of patients to develop a comprehensive clinical picture of the disorder. Accordingly, a national registry of United States residents with XLA was established in 1999 to provide an updated clinical view of the disorder in a large cohort of patients. A total of 201 patients were registered by 66 physicians. The estimated birth rate for the 10-year period of 1988-1997 was 1/379,000. Infection was the most common initial clinical presentation (85%), followed by a positive family history (41%) and neutropenia (11%). Although the average age of diagnosis was younger in patients with a positive family history (mean, 2.59 yr) than in patients with a negative family history (mean, 5.37 yr) (p < 0.001), only 34.5% of patients with a positive family history at the time of their birth were diagnosed before clinical symptoms developed-that is, based on family history alone. Seventy percent of patients had at least 1 episode of otitis, 62% at least 1 episode of pneumonia, 60% at least 1 episode of sinusitis, 23% at least 1 episode of chronic/recurrent diarrhea, 21% at least 1 episode of conjunctivitis, 18% at least 1 episode of pyoderma and/or cellulitis, 11% at least 1 episode of meningitis/encephalitis, 10% at least 1 episode of sepsis, 8% at least 1 episode of septic arthritis, 6% at least 1 episode of hepatitis, and 3% at least 1 episode of osteomyelitis. Fourteen of 201 (6.9%) patients were dead at the time they were entered in the Registry. However, in a prospective 4 /4-year follow-up of living patients, only 3/80 (3.75%) patients died. Causes of death included disseminated enterovirus infection (n = 6), pulmonary insufficiency (n = 5), adenovirus infection (n = 1), sepsis (n = 1), acquired immunodeficiency disease syndrome (AIDS) (n = 1), myocarditis (n = 1), hepatitis (n = 2), and stem cell transplantation (n = 1).
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OBJECTIVES: Togliatti City is witness to a large epidemic of human immunodeficiency virus (HIV) associated with injecting drug use (IDU). GOAL: This study sought to examine whether risk behaviors and risk factors associated with HIV differed across a sample IDUs by gender and sex work. STUDY: A sample of IDU (n = 423) comprising female sex workers (SWs) (n = 66), non-sex workers (nonSWs) (n = 89) and men (n = 268) were recruited by field workers in community settings. Behavioral and HIV prevalence data were collected. RESULTS: HIV prevalence did not differ across the groups ( approximately 56%), but gender adversely affected some risk factors. A comparison of risk behaviors indicated that SWs were more likely to engage in risky injecting behaviors than either men or nonSWs. They were also more likely to report a history of sexually transmitted infections. CONCLUSIONS: IDUs involved in sex work and IDU nonSWs require specific and targeted interventions to facilitate safer injecting and sexual behaviors.
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Although many schools use scores on the Dental Admission Test (DAT) to evaluate applicants, the association of these scores with students' performance on Part I of the National Board Dental Examinations (NBDE) has not been recently evaluated. In this study, the hypothesis that the DAT scores would be a significant predictor of Part I of the NBDE scores was tested. We analyzed by multiple regression the scores on both examinations for the 114 students matriculating in the University of Mississippi School of Dentistry in 1992, 1993, 1994, and 1995. The results indicate that DAT reading comprehension was a statistically significant predictor (p value less than or equal to 0.05) of all four subtests of Part I of the NBDE. The DAT biology and organic chemistry scores were statistically significant predictors of NBDE biochemistry-physiology, and the DAT quantitative analysis score was a statistically significant predictor of NBDE dental anatomy and occlusion. DAT perceptual ability and general chemistry were not significant predictors.