Albright’s hereditary osteodystrophy – case report

Pseudohypoparathyroidism (PHP) is a group of genetically conditioned metabolic diseases characterised by resistance of target tissues to parathormone. Type 1a, namely Albright’s hereditary osteodystrophy (AHO) or Albright’s syndrome, is associated with a mutation of the maternal allele of the GNAS gene encoding the α subunit of the Gs signalling protein. It is characterised by short stature, obesity, round face, brachydactyly, shortened 4th and 5th metatarsal and metacarpal bones and dental abnormalities such as enamel hypoplasia, root morphology disorders, eruption disorders, crowdings, hypodontia.

Aim
The aim of the study was to characterise a patient with Albright’s syndrome, to compare the case with other cases described in the literature and to present the treatment applied.

Material
The material consisted of: documentation provided by the patient and orthodontic documentation containing orthopantomographic images, lateral cephalograms, intra- and extraoral photographs, and diagnostic models.

Case report
The paper shows a case of a 10-year-old male patient with Albright’s syndrome with advanced reinclusion of deciduous molars and canines, and lack of 10 permanent tooth buds. Extractions of reincluded teeth and temporary prosthetic restorations were ordered. In the future, the patient will require orthodontic treatment to prepare for the final prosthetic restoration.

Conclusions
Due to a variety of disorders in the oral cavity, patients with Albright’s syndrome require treatment conducted by specialists in paediatric dentistry, orthodontics, prosthetics, and dental surgery. When there is reinclusion, tooth positions should be monitored regularly. Considering the increased risk of obstructive sleep apnoea, symptoms should be monitored and appropriate treatment recommended. Dental abnormalities in Albright’s syndrome have been extensively described in the literature, but there is no data on the occurrence of malocclusions, and it requires further research.