Prolidase deficiency: dento-facial aspects in a paediatric patient.
Eur J Paediatr Dent
; 15(2 Suppl): 224-8, 2014 Jul.
Article
em En
| MEDLINE
| ID: mdl-25101509
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. CASE REPORT 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.
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Base de dados:
MEDLINE
Assunto principal:
Anormalidades Dentárias
/
Face
/
Deficiência de Prolidase
Tipo de estudo:
Prognostic_studies
Limite:
Child
/
Humans
/
Male
Idioma:
En
Ano de publicação:
2014
Tipo de documento:
Article