BACKGROUND: @entity6563 ( @entity6563 ) is a very rare @entity74 caused by mutations of the @entity24056 gene or the @entity16900 gene. @entity6563 is associated with a number of dental manifestations. There are no published case reports of dental treatment under general anaesthesia (GA) in @entity1 with this condition. CASE REPORT: A 6-year-old @entity1 with @entity6563 characterised by classical features of the condition including a @entity851 , precocious puberty, endocrine disturbances, @entity1121 and @entity1560 of the liver was referred for treatment under GA. Dental manifestations included aberrant tooth morphology, @entity51280 and generalised severe crowding. TREATMENT: 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. FOLLOW-UP: The @entity1 was reviewed post-operatively and at regular intervals between 3 and 6  months for review and prevention. CONCLUSION: The @entity1 had a number of dental manifestations associated with @entity6563 . Peri-operative planning is essential for some @entity1 who may have a number of medical conditions.

Case report: Dental management of XXXX .

@entity1 :: ('9606', 'Species') :: ['girl', 'patients', 'patient'] @entity851 :: ('MESH:D006130', 'Disease') :: ['growth disorder'] @entity1560 :: ('MESH:D005234', 'Disease') :: ['fatty infiltration'] @entity51280 :: ('MESH:C537015', 'Disease') :: ['macrodontia'] @entity6563 :: ('MESH:D052497', 'Disease') :: ['Berardinelli-Seip congenital lipodystrophy', 'BSCL'] @entity16900 :: ('26580', 'Gene') :: ['BSCL2'] @entity24056 :: ('10555', 'Gene') :: ['AGPAT2'] @entity74 :: ('MESH:D030342', 'Disease') :: ['autosomal recessive condition'] @entity1121 :: ('MESH:D002312', 'Disease') :: ['hypertrophic cardiomyopathy']
@entity6563 :: (MESH:D052497,Disease) :: ['Berardinelli-Seip congenital lipodystrophy']
