title: Congenital adrenal hyperplasia: a case report with premature teeth exfoliation and bone resorption.
abstract: Congenital adrenal hyperplasia (CAH) is an inherited autosomal recessive disorder characterized by insufficient production of cortisol. The aim of this case report was to present a child with CAH, premature exfoliation of primary teeth and accelerated eruption of his permanent teeth related to bone resorption. A 4.5-year-old Caucasian boy with CAH and long-term administration of glucocorticoids was referred for dental restoration. Clinical examination revealed primary molars with worn stainless steel crowns, severe attrition of the upper canines, and absence of the upper incisors. Before the completion of treatment, abnormal mobility of the first upper primary molars and the lower incisors was detected, and a few days later the teeth exfoliated prematurely. Histologic examination revealed normal tooth structure. Alkaline phosphatase and blood cells values were normal. Eruption of the permanent dentition was also accelerated. Tooth mobility was noticed in the permanent teeth as soon as they erupted, along with bone destruction. Examination revealed an elevated level of receptor activator of nuclear factor-κB ligand and lower-than-normal osteoprotegerin and vitamin D levels. The patient was treated with vitamin D supplements, and his teeth have been stable ever since. CAH is a serious chronic disorder appearing in children with accelerated dental development and possibly premature loss of primary teeth.
authors.affiliation: Division of Pediatric Dentistry, Marquette University School of Dentistry, Milwaukee, Wisconsin; matinangelop@yahoo.gr.
authors.forename: Matina V
authors.initials: MV
authors.lastname: Angelopoulou
authors.affiliation: Department of Restorative Sciences, Texas A&M Baylor College of Dentistry, Dallas, Texas; and.
authors.forename: Elias
authors.initials: E
authors.lastname: Kontogiorgos
authors.affiliation: Department of Paediatric Dentistry, Dental School, University of Athens, Greece.
authors.forename: Dimitris
authors.initials: D
authors.lastname: Emmanouil
mesh_terms: D000312:Adrenal Hyperplasia, Congenital / Q000150:complications*; D001862:Bone Resorption / Q000209:etiology*; D002675:Child, Preschool; D006801:Humans; D008297:Male; D014078:Tooth Eruption; D014080:Tooth Exfoliation / Q000209:etiology*