Osteopetrosis encompasses a group of rare metabolic bone diseases characterized by impaired osteoclast activity or development, resulting in high bone mineral density. Existing guidelines focus on treatment of the severe infantile forms with hematopoietic cell transplantation (HCT) but do not address the management of patients with less severe forms for whom HCT is not the standard of care. Therefore, our objective was to develop expert consensus guidelines for the management of these patients.
A modified Delphi method was used to build consensus among participants of the Osteopetrosis Working Group, with responses to an anonymous online survey used to identify areas of agreement and conflict and develop a follow-up survey. The strength of recommendations and quality of evidence was graded using the Grading of Recommendations Assessment, Development and Evaluation system.
Consensus was found in the areas of diagnosis, monitoring, and treatment. We recommend relying on characteristic radiographic findings to make the diagnosis and found that genetic testing adds important information by identifying mutations associated with unique disease complications. We recommend ongoing monitoring for changes in mineral metabolism and other complications, including cranial nerve impingement, anemia, leukopenia, and dental disease. We suggest that calcitriol should not be used in high doses and instead recommend symptom-based supportive therapy for disease complications because noninfantile osteopetrosis has no effective treatment.
Scarcity of published studies on osteopetrosis reduce the ability to develop evidence-based guidelines for the management of these patients. Expert opinion-based guidelines for this rare condition are nevertheless important to enable improved care.

BACKGROUND: Knowledge and interpretation of the 2007 American Heart Association (AHA) guidelines regarding infective endocarditis (IE) prophylaxis among the dental community is not well established. The authors\' aim was to determine how dentists and dental hygienists interpret the 2007 AHA guidelines.
METHODS: The authors sent a cross-sectional survey to a random sample of 450 dental hygienists and 450 dentists in Alberta, Canada. The survey ascertained whether the practitioner would recommend IE prophylaxis to a high-risk cardiac patient undergoing a variety of dental procedures and for a variety of cardiac lesions in patients requiring gingival manipulation.
RESULTS: One hundred forty-nine hygienists (33%) and 194 dentists (43%) completed the survey. Use of prophylaxis for specific dental procedures was heterogeneous; 43% of hygienists recommended prophylaxis for polishing, 46% did not, and 11% replied \"sometimes.\" Hygienists were more likely than dentists to inappropriately recommend IE prophylaxis for low-risk lesions including mitral valve prolapse (54% of hygienists versus 42% of dentists recommending prophylaxis; P = .037) and hypertrophic cardiomyopathy (23% versus 15%; P = .057). The authors also observed a failure to recommend IE prophylaxis for high-risk lesions, including mechanical valve (that is, 81% of hygienists and 91% of dentists recommending prophylaxis; P = .008).
CONCLUSIONS: There is heterogeneity within the dental community with respect to IE prophylaxis. Dental hygienists are more likely than dentists to recommend IE prophylaxis for low-risk cardiac lesions. Both dentists and hygienists did not consistently recommend prophylaxis for all high-risk cardiac lesions.
CONCLUSIONS: Greater emphasis on IE prophylaxis education is required in training programs and continuing professional development.

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