Prevalence of oral diseases in Shwachman-Diamond syndrome.

The aim of this study was to determine the prevalence and severity of oral diseases in patients with ShwachmanDiamond syndrome (SDS). Thirty-five persons with SDS were compared to 20 healthy controls. A cross-sectional survey was carried out using self-reporting questionnaires and dental radiographs collected from the subjects and their dentists. Overall, oral diseases were more prevalent among subjects with SDS when compared to controls (p < 0.001). Persons with SDS also had more caries in both primary (p < 0.03) and permanent dentitions (p < 0.01), and also had delayed dental development (p < 0.04). Oral soft tissue pathoses, such as recurrent oral ulcerations (p < 0.00) and gingival bleeding upon brushing (p < 0.00), were significantly more prevalent in subjects with SDS. Pain on eating was also more frequent amongst persons with SDS (p < 0.008) and was often associated with oral ulcerations (p < 0.002). In conclusion, based on self-completed subject and dentist questionnaires, diseases of oral hard and soft tissues were more prevalent and severe in persons with SDS when compared with healthy controls.

Oral health-related quality of life of children with neutropenia.

This study evaluated the oral health-related quality of life (OHQoL) in children with neutropenia. Twenty-seven children with neutropenia were compared to 33 healthy, age-matched control subjects. Previously validated age-specific, multidimensional and self-reporting child OHQoL questionnaires were used. Overall and subscale scores were compared between the two groups. Respondents in the group of children with neutropenia reported that their disease had a significant impact on their oral health in terms of oral symptoms (p < 0.0001), functional limitations (p < 0.0001), and social well-being (p < 0.0001). In global ratings, they rated their oral health to be markedly worse than that of the healthy subjects (p < 0.0001). However, there was no difference between the groups in the extent to which their oral condition affected their lives overall. These results, along with responses to individual measures of social and emotional well-being, suggest that children in this group with neutropenia have psychologically adapted to the oral health challenges they experience because of their condition.

Timing of neutrophil tissue repopulation predicts restoration of innate immune protection in a murine bone marrow transplantation model.

It has been suggested that neutrophil tissue repopulation following bone marrow transplantation (BMT) serves as an earlier and more relevant marker of susceptibility to infection than circulating neutrophil counts. In a previous study using an oral rinse protocol, we found that oral neutrophil recovery always preceded blood neutrophil engraftment and that the day of oral neutrophil detection served as a predictor of patient susceptibility to infection after BMT. Consequently, we have developed and validated a mouse BMT model which uses bone marrow transplants containing enhanced green fluorescent protein-expressing neutrophils to follow neutrophil tissue repopulation after BMT. Using this in vivo cell migration model, we assessed the significance of neutrophil tissue recruitment kinetics with neutrophil functionality and in vivo bacterial killing after BMT. Using the animal model, we have demonstrated that protection against bacterial infection is conferred at the time of neutrophil tissue delivery, which always occurs before neutrophils are detected in the blood. We therefore conclude that neutrophil tissue recovery is an early measure of the restoration of cellular innate immune function after BMT. This model will help us better understand the factors regulating neutrophil recruitment to the tissues.