title: Prolidase deficiency: dento-facial aspects in a paediatric patient.
abstract: BACKGROUND
  Prolidase Deficiency (PD) is a rare hereditary disease consisting in developmental delay, mental retardation, facial dysmorphism, splenomegaly, recurrent pulmonary infections and skin lesions.
  
  
  METHODS
  The present study reports a case of PD treated in the Paediatric Section of the Department of Dentistry and Surgery at the University of Bari. A special diagnostic and clinical approach to the patient was useful to improve his quality of life and identify some new aspects of this systemic disease. In particular, clinical features never described before are reported: low hair line, decreased osteotendinous reflexes, long upper lip, microrhinia, dentoskeletal Class III, dental age (Proffit) older than chronological age, fusion of 2nd and 3rd cervical vertebrae, incomplete atlanto-occipital fusion.
authors.affiliation: Department of Life, Health and Environmental Sciences, Dental Clinic; University of L'Aquila, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Lacarbonara
authors.affiliation: Department of Dentistry and Surgery, University of Bari, Italy.
authors.forename: A P
authors.initials: AP
authors.lastname: Cazzolla
authors.affiliation: Department of Dentistry and Surgery, University of Bari, Italy.
authors.forename: V A
authors.initials: VA
authors.lastname: Lacarbonara
authors.affiliation: Department of Dentistry and Surgery, University of Bari, Italy.
authors.forename: D
authors.initials: D
authors.lastname: Di Venere
authors.affiliation: Department of Life, Health and Environmental Sciences, Dental Clinic; University of L'Aquila, Italy.
authors.forename: M
authors.initials: M
authors.lastname: Capogreco
authors.affiliation: Department of Life, Health and Environmental Sciences, Dental Clinic; University of L'Aquila, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Marzo
mesh_terms: D002508:Cephalometry; D002648:Child; D005145:Face; D006801:Humans; D008297:Male; D056732:Prolidase Deficiency / Q000150:complications* / Q000628:therapy; D011862:Radiography, Panoramic; D014071:Tooth Abnormalities / Q000209:etiology*