Biomaterial Database

Focal dermal hypoplasia (Goltz-Gorlin) syndrome with taurodontism. 1996

T McNamara, and C A Trotman, and A M Hahessy, and P Kavanagh

Western Health Board, Merlin Park Hospital, Galway, Ireland.

This paper is a case report of a 10-year-old girl with focal dermal hypoplasia (FDH) who presented for dental care. She displayed many of the well-documented oral features associated with this syndrome. Additionally, she had taurodontism of a permanent mandibular molar. Although taurodontism affected only one tooth in this patient, the presentation of taurodontism with FDH has not been reported previously.

UI	MeSH Term	Description	Entries
D008963	Molar	The most posterior teeth on either side of the jaw, totaling eight in the deciduous dentition (2 on each side, upper and lower), and usually 12 in the permanent dentition (three on each side, upper and lower). They are grinding teeth, having large crowns and broad chewing surfaces. (Jablonski, Dictionary of Dentistry, 1992, p821)	Molars
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D003786	Dental Pulp Cavity	The space in a tooth bounded by the dentin and containing the dental pulp. The portion of the cavity within the crown of the tooth is the pulp chamber; the portion within the root is the pulp canal or root canal.	Pulp Canal,Pulp Chamber,Root Canal,Cavities, Dental Pulp,Cavity, Dental Pulp,Dental Pulp Cavities,Pulp Cavities, Dental,Pulp Cavity, Dental,Canal, Pulp,Canal, Root,Canals, Pulp,Canals, Root,Chamber, Pulp,Chambers, Pulp,Pulp Canals,Pulp Chambers,Root Canals
D005260	Female		Females
D005489	Focal Dermal Hypoplasia	A genetic skin disease characterized by hypoplasia of the dermis, herniations of fat, and hand anomalies. It is found exclusively in females and transmitted as an X-linked dominant trait.	Dermal Hypoplasia, Focal,Goltz Gorlin Syndrome,Goltz Syndrome,Goltz's Syndrome,Goltz-Gorlin Syndrome,Dermal Hypoplasias, Focal,Focal Dermal Hypoplasias,Goltzs Syndrome,Gorlin Syndrome, Goltz,Hypoplasia, Focal Dermal,Hypoplasias, Focal Dermal,Syndrome, Goltz,Syndrome, Goltz Gorlin,Syndrome, Goltz's,Syndrome, Goltz-Gorlin
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013577	Syndrome	A characteristic symptom complex.	Symptom Cluster,Cluster, Symptom,Clusters, Symptom,Symptom Clusters,Syndromes
D014071	Tooth Abnormalities	Congenital absence of or defects in structures of the teeth.	Odontome,Abnormalities, Teeth,Abnormalities, Tooth,Teeth Abnormalities,Abnormality, Teeth,Abnormality, Tooth,Odontomes,Teeth Abnormality,Tooth Abnormality