Whyte, Michael P.; McAlister, William H.; and et al, ,"Enzyme-replacement therapy in life-threatening hypophosphatasia." The New England Journal of Medicine.366,10. 904-913. (2012).

To assess the utility of radiographs taken immediately after the application of a cast in the management of pediatric torus (or buckle) fractures and to determine the need for serial radiographs taken at follow-up visits. Design: Retrospective medical record review; survey questionnaire of a panel of experts. Setting: The pediatric emergency department (PED) and the pediatric orthopedic clinic at an urban, tertiary care hospital. Patients: All children with torus fractures referred to the pediatric orthopedic clinic for follow-up visits between February 1995 and February 1997. Main Outcome Measures: The number of patients whose postcast studies was obtained in the PED; number of follow-up visits and studies conducted at the pediatric orthopedic clinic; usual regional practices as extracted from a panel of experts by survey questionnaire. Results: Of 70 patients, 46 (66%) were evaluated by a single, precast study in the PED, and 24 (34%) were evaluated by both precast and postcast studies in the PED. The time range of the first follow-up study was the first to fifth week after the patient's injury. The range of the number of follow-up studies for each patient was 0 to 5. Our cohort's total radiology charges for 70 patients were $27 251. Regional directors of pediatric orthopedic surgery unanimously agreed that postcast studies in the PED are unnecessary. The range of the number of follow-up studies they obtained is 0 to 3 per patient. Conclusions: Postcast studies of torus fractures are unnecessary. Multiple radiographs taken during follow-up visits, especially early in the healing process, do not change fracture management. Relying on the clinical examination, perhaps combined with a single follow-up study, is a more appropriate regimen for the management of pediatric torus fractures and translates into a cost savings of over $10 000 for our 70 patients.

Early thymic involution is associated with rapidly progressive disease in HIV-infected children.

One of the principal targets of HIV infection is the human peripheral blood CD4+ T cell, resulting in progressive CD4+ lymphocyte loss. Hypothesized mechanisms for this loss include apoptosis, cytolytic reactions, V-beta gene deletion of the T-cell receptor (TCR) by superantigens, CD4+ lymphocyte syncytium formation, and autoimmune reactions. In adults with HIV infection, the critical decline in CD4+ lymphocyte number that heralds the onset of AIDS-defining conditions is well characterized, whereas in infants and children the critical level of CD4+ cells predisposing to the development of AIDS-defining conditions or mortality is not fully characterized, due to an incomplete knowledge of CD4+ lymphocyte number and changes with age in normal and HIV-infected children. In a prospective study of 317 infants born to HIV-infected women, early results show that the monthly change in absolute CD4+ lymphocyte number over a 3- to 9-month period in HIV-infected infants was -109 cells/mm3 per month, at least double the rate of decline measured in HIV-noninfected infants in the study or that calculated from normal infants' values reported in the literature. In other clinical studies in HIV-infected infants and children, it was possible to study the effect of low CD4+ cell counts on clinical status and mortality. In HIV-infected pediatric patients younger than 1 year, it was possible to correlate low CD4+ cell number with advanced disease status (CDC pediatric class P-2). It was also possible to correlate extremely low CD4+ cell counts (< 200 cells/mm3) in HIV-infected children with a significant risk of mortality within the next 3 months of life. Sequential CD4+ cell analysis of HIV-high-risk infants will delineate the rate of HIV-related decline in CD4+ cells, thus facilitating the diagnosis of HIV infection and aiding in identification of HIV-infected children at high risk of disease progression or death.

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