P2RX7 Purinoceptor: A Therapeutic Target for Ameliorating the Symptoms of Duchenne Muscular Dystrophy

open access articleDuchenne muscular dystrophy (DMD) is the most common inherited muscle disease, leading to severe disability and death in young men. Death is caused by the progressive degeneration of striated muscles aggravated by sterile inflammation. The pleiotropic effects of the mutant gene also include cognitive and behavioral impairments and low bone density. Current interventions in DMD are palliative only as no treatment improves the long-term outcome. Therefore, approaches with a translational potential should be investigated, and key abnormalities downstream from the absence of the DMD product, dystrophin, appear to be strong therapeutic targets. We and others have demonstrated that DMD mutations alter ATP signaling and have identified P2RX7 purinoceptor up-regulation as being responsible for the death of muscles in the mdx mouse model of DMD and human DMD lymphoblasts. Moreover, the ATP–P2RX7 axis, being a crucial activator of innate immune responses, can contribute to DMD pathology by stimulating chronic inflammation. We investigated whether ablation of P2RX7 attenuates the DMD model mouse phenotype to assess receptor suitability as a therapeutic target

Parental perceptions of dental health and need for treatment in children with epilepsy: a multicenter cross-sectional study

Ahmed Hussein Subki,1 Abdel Moniem Mukhtar,2 Omar M Saggaf,1 Ragaa A Ali,3 Khalid A Khalifa,3 Dalia M Al-Lulu,3 Mohammed Saad Alsallum,1 Diyaa H Bokhary,1 Ayman M Baabdullah,1 Sulaiman M Kassar,1 Basil M Jan,4 Muhab M Hindi,1 Mohammed M Jan1 1Department of Pediatrics, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia; 2Department of Family and Community Medicine, Faculty of Medicine, King Abdulaziz University, Jeddah, Saudi Arabia; 3Batterjee Medical College, Jeddah, Saudi Arabia; 4Faculty of Dentistry, King Abdulaziz University, Jeddah, Saudi Arabia Background: Epilepsy is a common neurological disorder in childhood. However, there have been limited studies on its impact on the oral health of affected children. Our study aimed to assess the oral health of children with epilepsy in the city of Jeddah, Saudi Arabia, as perceived by their mothers. Methods: We conducted a cross-sectional study in three hospitals. We included children 2–18 years old with physician-confirmed epilepsy diagnosis. We assessed parental perception of dental status and need for dental care using a standardized questionnaire that was completed by the mothers. To adjust for potential confounding variables, we used univariate and multivariate logistic regression. Results: We included 96 children with epilepsy in our study. Their mean age was 6.4±3.4 years. In 55.2% (n=53), dental status was rated as bad, and in 84.4% (n=81) a need for dental care was expressed. Cerebral palsy (OR 5.06, 95% CI 1.28–19.99; P=0.021), motor disability (OR 6.41, 95% CI 1.12–36.73; P=0.037), referral from a pediatric neurology clinic to a dentist (OR 10.755, 95% CI 3.290–35.151; P<0.001), and irregular brushing of teeth (OR 5.397, 95% CI 1.536–18.961; P=0.009) were significantly associated with increased risk of perceived bad dental status. Perception of the child as being overweight (OR 0.117, 95% CI 0.034–0.400; P=0.001) was significantly associated with decreased risk of perceived bad dental status. Motor disability (OR 5.73, 95% CI 1.64–20.04; P=0.006) was significantly associated with increased parental expression of need for dental care. Conclusion: In most children with epilepsy, perceived dental status was bad and there was a high expressed need for dental care. Interventions to improve the dental health of children with epilepsy should focus on those with cerebral palsy and motor disability. Keywords: oral, dental, teeth, hygiene, health, epilepsy, chil