Association between sickle cell disease and the oral health condition of children and adolescents.

BACKGROUND: Sickle cell disease (SCD) is the most prevalent monogenic hereditary pathology associated with the presence of hemoglobin SS in the world. It can affect individuals, leading to changes in the face and body, causing a deficiency in dental and bone tissue formation that can ultimately result in a higher level of predisposition to developing dental caries. This study aimed to evaluate the oral condition of children and adolescents with SCD in comparison with the condition of healthy controls. METHODS: This was a cross-sectional study of children and adolescents aged 5 to 18 of both sexes from a hematology center in Bahia, Brazil, and subjects without hemoglobinopathies from a public school of the same state (comparison group). There were 124 individuals, 63 in the comparison group and 61 in the disease group. Interviews, dental and periodontal exams using the DMFT and Periodontal Community Index, respectively, were performed, and the salivary buffer capacity and salivary flow rates of the entire sample population were evaluated. The categorical variables were compared using a chi-square test or Fisher's exact test. For comparison of means, the Student's-t test was used for independent samples that presented symmetrical distribution. RESULTS: The study showed that the DMFT was 2.08 (2.71) for the SCD group and 1.05 (1.67) for the comparison group (p = 0.013). For dmft, the values were 2.3 (2.6) and 0.88 (1.2), respectively, (p = 0.018). Exams of the periodontium showed the presence of gingival bleeding and dental calculus, with no statistical significance between groups (p = 0.984). When evaluating salivary flow and buffer capacity, no significant differences were observed for the flow rates (p = 0.485), but the SCD group presented a lower buffer capacity compared with the comparison group (p = 0.006). Individuals who used hydroxyurea had a dmft (2.50) higher than that of the comparison group (2.00), and salivary flow was lower than the normal rate in 70% of the children who did not use this medication. CONCLUSION: Children and teenagers with SCD had deficient oral health when compared with the comparison group, presenting a higher level of dental caries and lower buffer capacity.

Simultaneous administration of G-CSF and GM-CSF for re-mobilization in patients with inadequate initial progenitor cell collections for autologous transplantation.

BACKGROUND: A proportion of candidates for high-dose chemotherapy with autologous PBPC support (HDC-PBPCS) will not provide an adequate PBPC yield from their first mobilization. The value of re-mobilization and the best regimen for re-mobilization in these patients is unclear. METHODS: In 23 patients who failed to provide > or = 3 x 10(6) CD34+ cells/kg after their first mobilization, PBPC were re-mobilized using a regimen of simultaneous administration of G-CSF and GM-CSF (10 microg/kg/day each) with leukaphereses (LP) starting Day 4 or 5 of CSF administration. Yields of WBC/kg, MNC/kg and CD34+ cells/kg/L of processed blood were compared between the first and second mobilization in each patient. The ability of the combined yield from the two mobilizations to achieve the desired threshold PBPC yield and the tolerability of the re-mobilization were determined. RESULTS: The re-mobilization regimen was well-tolerated and no patient discontinued the regimen because of toxicity. Median collected WBC/kg/L (1.37 x 10(7) versus 2.62 x 10(7), p = 0.0065), MNC/kg/L (0.77 x 10(7) versus 1.97 x 10(7), p = 0.0003), CD34+ cells/kg/L (1.64 x 10(7) versus 4.18 x 10(7), p = 0.001) were significantly higher after the second mobilization (G-CSF/GM-CSF combination). Percentage of CD34+ cells in the leukapheresis was also significantly higher after the second mobilization (median 0.104% versus 0.195%, p = 0.036). Twelve of 22 patients achieved the target PBPC dose (> 3 x 10(6)/CD34+ cells/kg) after two mobilizations (six patients achieved the target from the second mobilization alone). A further eight underwent HDC-PBPCS without achieving the target PBPC dose. These patients experienced a significant delay in neutrophil and platelet engraftment when compared with those patients achieving the target dose. DISCUSSION: This study demonstrates that the combination of G-CSF and GM-CSF is an effective and tolerable method for re-mobilization of PBPC in patients who fail to provide an adequate yield from their first mobilization.