Nager acrofacial dysostosis, also known as Nager syndrome, is a genetic disorder that affects the face and the limbs. Its characteristics can include underdevelopment of the cheek and jaw area, downward-sloping eye openings, missing lower eyelashes, a shortened soft palate, possible cleft palate, poorly developed internal and external ears with hearing loss, underdevelopment or absence of the thumb, shortened forearms with limited elbow movement, hammer toes, kidney or stomach reflux and sometimes accessory tragi. Vertebral anomalies such as scoliosis occasionally appear. The pattern of inheritance is autosomal, but sources argue over whether it is dominant or recessive, and most cases are sporadic. The syndrome shares many features with other craniofacial syndromes, namely Miller, Treacher Collins and Pierre Robin. This entry records descriptions and is not medical advice.
Facts
Classification
ICD-10 ChapterXVII: Congenital Malformations, Deformations and Chromosomal Abnormalities 1 Communicable or Non-communicable Causative Agent
Causative Agent (category) Sources
1. World Health Organization: ICD-10 Version:2019 - Q65-Q79 Congenital malformations and deformations of the musculoskeletal system
World Health OrganizationCategory Q75.4Quote, Category Q75.4
Q75.4 Mandibulofacial dysostosis
View the Source 2. Nager acrofacial dysostosis (Wikipedia)
Lead sentence
Nager acrofacial dysostosis, also known as Nager syndrome, is a genetic disorder
Lead: disease category
Nager acrofacial dysostosis, also known as Nager syndrome, is a genetic disorder which displays several or all of the following characteristics: underdevelopment of the cheek and jaw area, down-sloping of the opening of the eyes, lack or absence of the lower eyelashes, kidney or stomach reflux, hammer toes, shortened soft palate, lack of development of the internal and external ear, possible cleft palate, underdevelopment or absence of the thumb, hearing loss (see hearing loss with craniofacial syndromes) and shortened forearms, as well as poor movement in the elbow, and may be characterized by accessory tragi.
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