Multiple External Root Resorption in a Pediatric Patient with Familial Expansile Osteolysis.

External root resorption (ERR) of permanent teeth is a pathological process that can lead to their loss. There are several systemic and/or genetic abnormalities that have been associated with ERR. Among them, familial expansile osteolysis (FEO) is an autosomal dominant disease characterized by skeletal defects, middle ear deafness, and abnormal root resorption. The purpose of this paper was to describe the case of a 10-year-old female with familial expansile osteolysis born with missing ossicles and, therefore, deafness. The patient was not diagnosed with FEO until the age of 10 years, when she presented to the dental clinic with advanced ERR in several permanent teeth. The series of tests she underwent for diagnosis and the treatments rendered are presented and discussed. It is recommended that when ERR cannot be explained by local etiologic factors, systemic abnormalities and genetic testing should be considered.

Inhibition of bone morphogenetic protein 6 receptors ameliorates Sjögren's syndrome in mice.

Primary Sjögren's syndrome (pSS) is a chronic autoimmune disease, with only palliative treatments available. Recent work has suggested that increased bone morphogenetic protein 6 (BMP6) expression could alter cell signaling in the salivary gland (SG) and result in the associated salivary hypofunction. We examined the prevalence of elevated BMP6 expression in a large cohort of pSS patients and tested the therapeutic efficacy of BMP signaling inhibitors in two pSS animal models. Increased BMP6 expression was found in the SGs of 54% of pSS patients, and this increased expression was correlated with low unstimulated whole saliva flow rate. In mouse models of SS, inhibition of BMP6 signaling reduced phosphorylation of SMAD1/5/8 in the mouse submandibular glands, and led to a recovery of SG function and a decrease in inflammatory markers in the mice. The recovery of SG function after inhibition of BMP6 signaling suggests cellular plasticity within the salivary gland and a possibility for therapeutic intervention that can reverse the loss of function in pSS.