title: Case report: Dental management of Berardinelli-Seip congenital lipodystrophy.
abstract: BACKGROUND
  Berardinelli-Seip congenital lipodystrophy (BSCL) is a very rare autosomal recessive condition caused by mutations of the AGPAT2 gene or the BSCL2 gene. BSCL is associated with a number of dental manifestations. There are no published case reports of dental treatment under general anaesthesia (GA) in patients with this condition.
  
  
  METHODS
  A 6-year-old girl with BSCL characterised by classical features of the condition including a growth disorder, precocious puberty, endocrine disturbances, hypertrophic cardiomyopathy and fatty infiltration of the liver was referred for treatment under GA. Dental manifestations included aberrant tooth morphology, macrodontia and generalised severe crowding.
  
  
  METHODS
  Dental treatment was carried out under GA following consideration of various options. In conjunction with other medical specialists, a number of peri-operative precautions were taken. Comprehensive dental care was provided which included restorations, extractions and fissure sealants.
  
  
  RESULTS
  The patient was reviewed post-operatively and at regular intervals between 3 and 6 months for review and prevention.
  
  
  CONCLUSIONS
  The patient had a number of dental manifestations associated with BSCL. Peri-operative planning is essential for some patients who may have a number of medical conditions.
authors.affiliation: Guy's and St Thomas' NHS Trust, 6 The Heights, London, SE7 8JH, UK. nabinabhujel@hotmail.com.
authors.forename: N
authors.initials: N
authors.lastname: Bhujel
authors.affiliation: Salaried Dental Service, Bradford District Care Trust, Bradford, UK.
authors.forename: H
authors.initials: H
authors.lastname: Clark
mesh_terms: D002648:Child; D003729:Dental Care; D005260:Female; D006801:Humans; D052497:Lipodystrophy, Congenital Generalized; D008310:Malocclusion; D009154:Mutation
keywords: Berardinelli-Seip congenital lipodystrophy; Dental management