China
Africa
Explore chapters and articles related to this topic
Rheumatic and musculoskeletal disorders
Published in Rachel U Sidwell, Mike A Thomson, Concise Paediatrics, 2020
Rachel U Sidwell, Mike A Thomson
Autosomal recessive syndrome featuring: Skeletal dysplasia including mild thoracic involvementFour limb postaxial polydactylyConical teeth, oligodontia, dysplastic nailsCHD (40%)
Oral and craniofacial disorders
Published in Angus Clarke, Alex Murray, Julian Sampson, Harper's Practical Genetic Counselling, 2019
Hypodontia, or lack of one or a few permanent teeth, is extremely common (5%–10% in most surveys) and is often inherited as a variable autosomal dominant trait. One gene implicated in both syndromic and non-syndromic tooth agenesis is MSX1. Hypodontia may be the only significant finding in female heterozygotes for X-linked hypohidrotic ectodermal dysplasia (see Chapter 18), where incisors may also be peg shaped. Oligodontia and complete anodontia are commonly associated with this disorder in males, but can also occur in other ectodermal dysplasia syndromes, in orofaciodigital (OFD) syndrome type 1 (X-linked dominant) and with iris dysplasia in Rieger syndrome (autosomal dominant). A single central incisor tooth may be associated with midline abnormalities such as holoprosencephaly (e.g. in association with SHH).
Immunology (primary Immunodeficiency Syndromes
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Stephan Strobel, Alison M. Jones
Affected children have absent sweat glands, hypo/oligodontia with abnormal peg-shaped teeth, sparse hair, eczema and typical facial features with frontal bossing and a depressed nasal bridge. There is increased susceptibility to pyogenic infections, mycobacterial infection and herpes viruses. Diarrhoea and failure to thrive are common. Autoimmunity also occurs frequently. There may be a family history of incontinentia pigmenti.
Experiences of daily life and oral rehabilitation in oligodontia – a qualitative study
Published in Acta Odontologica Scandinavica, 2019
Solfrid Sørgjerd Saltnes, Amy Østertun Geirdal, Rønnaug Saeves, Janicke Liaaen Jensen, Hilde Nordgarden
Oligodontia is defined as the congenital absence of six or more permanent teeth, excluding third molars. The condition is associated with a reduction in the size and shape of the teeth, delayed tooth formation, anomalies of the enamel, and disturbances of alveolar growth [1]. Oligodontia can be an isolated trait [1] or be part of a general medical condition, with ectodermal dysplasia (ED) being the most common. In addition to teeth, ED affects other ectodermal tissues like hair, skin, nails, and sweat glands [2,3]. Our studies indicate that there is an emotional cost associated with living with oligodontia, which is consistent with studies on patients with other chronic disorders [4–6]. Other researchers have also reported that congenital missing teeth poses significant clinical, functional, aesthetic, and therapeutic challenges [7,8].