Biomaterial Database

Cockayne's syndrome: literature review and case report. 1991

R A Boraz

University of Kansas Medical Center, Kansas City.

Cockayne's syndrome is a rare, autosomal recessive disorder characterized clinically by cachectic dwarfism, cutaneous photosensitivity, loss of adipose tissue, mental retardation, skeletal and neurological abnormalities, and pigmentary degeneration of the retina. Dental caries is a common finding. The case of a 4-year-old male with Cockayne's syndrome is presented. A dental rehabilitation involving outpatient surgery is described including the difficulties encountered because of a small oral cavity and restricted mandibular range of motion.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D003057	Cockayne Syndrome	A syndrome characterized by multiple system abnormalities including DWARFISM; PHOTOSENSITIVITY DISORDERS; PREMATURE AGING; and HEARING LOSS. It is caused by mutations of a number of autosomal recessive genes encoding proteins that involve transcriptional-coupled DNA REPAIR processes. Cockayne syndrome is classified by the severity and age of onset. Type I (classical; CSA) is early childhood onset in the second year of life; type II (congenital; CSB) is early onset at birth with severe symptoms; type III (xeroderma pigmentosum; XP) is late childhood onset with mild symptoms.	Progeria-Like Syndrome,Cockayne Syndrome Type 3,Cockayne Syndrome Type C,Cockayne Syndrome, Group A,Cockayne Syndrome, Group B,Cockayne Syndrome, Group C,Cockayne Syndrome, Type A,Cockayne Syndrome, Type B,Cockayne Syndrome, Type C,Cockayne Syndrome, Type I,Cockayne Syndrome, Type II,Cockayne Syndrome, Type III,Dwarfism-Retinal Atrophy-Deafness Syndrome,Group A Cockayne Syndrome,Group B Cockayne Syndrome,Group C Cockayne Syndrome,Progeroid Nanism,Type A Cockayne Syndrome,Type B Cockayne Syndrome,Type C Cockayne Syndrome,Type I Cockayne Syndrome,Type II Cockayne Syndrome,Type III Cockayne Syndrome,Progeria Like Syndrome,Progeria-Like Syndromes,Syndrome, Cockayne,Syndrome, Progeria-Like
D003730	Dental Care for Disabled	Dental care for the emotionally, mentally, or physically disabled patient. It does not include dental care for the chronically ill (	Dental Care for Handicapped,Dentistry for Disabled,Dentistry for Handicapped,Disabled, Dentistry for,Handicapped, Dentistry for
D003731	Dental Caries	Localized destruction of the tooth surface initiated by decalcification of the enamel followed by enzymatic lysis of organic structures and leading to cavity formation. If left unchecked, the cavity may penetrate the enamel and dentin and reach the pulp.	Caries, Dental,Carious Lesions,Dental Cavities,Dental Cavity,Dental Decay,Dental White Spots,Carious Dentin,Decay, Dental,Dental White Spot,White Spot, Dental,White Spots, Dental,Carious Dentins,Carious Lesion,Cavities, Dental,Cavity, Dental,Dentin, Carious,Dentins, Carious,Lesion, Carious,Lesions, Carious,Spot, Dental White,Spots, Dental White
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man