Biomaterial Database

Oral self-mutilation by a 17-month-old child with Lesch-Nyhan syndrome. 1997

N Rashid, and H Yusuf

Department of Oral and Maxillofacial Surgery, North East Lincolnshire NHS Trust, Grimsby, UK.

We report a 17-month-old boy who was a known case of Lesch-Nyhan syndrome and presented with self-inflicted oral ulcerations of his lips and cheeks. He had a normal complement of caries-free deciduous teeth. Initially a conservative approach was planned and a bite plate made, but as a result of poor compliance and persistent ulceration and after consultation with his parents it was decided to extract all deciduous teeth.

UI	MeSH Term	Description	Entries
D007223	Infant	A child between 1 and 23 months of age.	Infants
D007926	Lesch-Nyhan Syndrome	An inherited disorder transmitted as a sex-linked trait and caused by a deficiency of an enzyme of purine metabolism; HYPOXANTHINE PHOSPHORIBOSYLTRANSFERASE. Affected individuals are normal in the first year of life and then develop psychomotor retardation, extrapyramidal movement disorders, progressive spasticity, and seizures. Self-destructive behaviors such as biting of fingers and lips are seen frequently. Intellectual impairment may also occur but is typically not severe. Elevation of uric acid in the serum leads to the development of renal calculi and gouty arthritis. (Menkes, Textbook of Child Neurology, 5th ed, pp127)	Choreoathetosis Self-Mutilation Hyperuricemia Syndrome,Hypoxanthine-Phosphoribosyl-Transferase Deficiency Disease,Choreoathetosis Self-Mutilation Syndrome,Complete HGPRT Deficiency Disease,Complete HPRT Deficiency,Complete Hypoxanthine-Guanine Phosphoribosyltransferase Deficiency,Deficiency Disease, Complete HGPRT,Deficiency Disease, Hypoxanthine-Phosphoribosyl-Transferase,Deficiency of Guanine Phosphoribosyltransferase,Deficiency of Hypoxanthine Phosphoribosyltransferase,HGPRT Deficiency,HGPRT Deficiency Disease, Complete,Hypoxanthine Guanine Phosphoribosyltransferase 1 Deficiency,Hypoxanthine Guanine Phosphoribosyltransferase Deficiency,Hypoxanthine Phosphoribosyltransferase Deficiency,Juvenile Gout, Choreoathetosis, Mental Retardation Syndrome,Juvenile Hyperuricemia Syndrome,Lesch-Nyhan Disease,Primary Hyperuricemia Syndrome,Total HPRT Deficiency,Total Hypoxanthine-Guanine Phosphoribosyl Transferase Deficiency,X-Linked Hyperuricemia,X-Linked Primary Hyperuricemia,Choreoathetosis Self Mutilation Hyperuricemia Syndrome,Choreoathetosis Self Mutilation Syndrome,Choreoathetosis Self-Mutilation Syndromes,Complete HPRT Deficiencies,Complete Hypoxanthine Guanine Phosphoribosyltransferase Deficiency,Deficiencies, Complete HPRT,Deficiencies, HGPRT,Deficiencies, Hypoxanthine Phosphoribosyltransferase,Deficiencies, Total HPRT,Deficiency Disease, Hypoxanthine Phosphoribosyl Transferase,Deficiency Diseases, Hypoxanthine-Phosphoribosyl-Transferase,Deficiency, Complete HPRT,Deficiency, HGPRT,Deficiency, Hypoxanthine Phosphoribosyltransferase,Deficiency, Total HPRT,Guanine Phosphoribosyltransferase Deficiencies,Guanine Phosphoribosyltransferase Deficiency,HGPRT Deficiencies,HPRT Deficiencies, Complete,HPRT Deficiencies, Total,HPRT Deficiency, Complete,HPRT Deficiency, Total,Hyperuricemia Syndrome, Juvenile,Hyperuricemia Syndrome, Primary,Hyperuricemia Syndromes, Juvenile,Hyperuricemia Syndromes, Primary,Hyperuricemia, X-Linked,Hyperuricemia, X-Linked Primary,Hyperuricemias, X-Linked,Hyperuricemias, X-Linked Primary,Hypoxanthine Phosphoribosyl Transferase Deficiency Disease,Hypoxanthine Phosphoribosyltransferase Deficiencies,Hypoxanthine-Phosphoribosyl-Transferase Deficiency Diseases,Juvenile Hyperuricemia Syndromes,Lesch Nyhan Disease,Lesch Nyhan Syndrome,Phosphoribosyltransferase Deficiencies, Guanine,Phosphoribosyltransferase Deficiencies, Hypoxanthine,Phosphoribosyltransferase Deficiency, Guanine,Phosphoribosyltransferase Deficiency, Hypoxanthine,Primary Hyperuricemia Syndromes,Primary Hyperuricemia, X-Linked,Primary Hyperuricemias, X-Linked,Self-Mutilation Syndrome, Choreoathetosis,Self-Mutilation Syndromes, Choreoathetosis,Syndrome, Choreoathetosis Self-Mutilation,Syndrome, Juvenile Hyperuricemia,Syndrome, Primary Hyperuricemia,Syndromes, Choreoathetosis Self-Mutilation,Syndromes, Juvenile Hyperuricemia,Syndromes, Primary Hyperuricemia,Total HPRT Deficiencies,Total Hypoxanthine Guanine Phosphoribosyl Transferase Deficiency,X Linked Hyperuricemia,X Linked Primary Hyperuricemia,X-Linked Hyperuricemias,X-Linked Primary Hyperuricemias
D008046	Lip	Either of the two fleshy, full-blooded margins of the mouth.	Philtrum,Lips,Philtrums
D008297	Male		Males
D002610	Cheek	The part of the face that is below the eye and to the side of the nose and mouth.	Bucca,Buccas,Cheeks
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012652	Self Mutilation	The act of injuring one's own body to the extent of cutting off or permanently destroying a limb or other essential part of a body.	Autotomy Human,Self Mutilating Behavior,Autotomy Humans,Behavior, Self Mutilating,Mutilating Behavior, Self,Mutilation, Self,Self Mutilating Behaviors
D014081	Tooth Extraction	The surgical removal of a tooth. (Dorland, 28th ed)	Extraction, Tooth,Extractions, Tooth,Tooth Extractions
D017090	Occlusal Splints	Rigid or flexible appliances that overlay the occlusal surfaces of the teeth. They are used to treat OBSTRUCTIVE SLEEP APNEA, clenching, BRUXISM, and their sequelae, and to provide temporary relief from muscle or temporomandibular joint pain.	Dental Night Guard,Mandibular Advancement Devices,Mandibular Advancement Splints,Splints, Occlusal,Advancement Device, Mandibular,Advancement Devices, Mandibular,Advancement Splint, Mandibular,Advancement Splints, Mandibular,Dental Night Guards,Device, Mandibular Advancement,Devices, Mandibular Advancement,Guard, Dental Night,Guards, Dental Night,Mandibular Advancement Device,Mandibular Advancement Splint,Night Guard, Dental,Night Guards, Dental,Occlusal Splint,Splint, Mandibular Advancement,Splint, Occlusal,Splints, Mandibular Advancement
D019226	Oral Ulcer	A loss of mucous substance of the mouth showing local excavation of the surface, resulting from the sloughing of inflammatory necrotic tissue. It is the result of a variety of causes, e.g., denture irritation, aphthous stomatitis (STOMATITIS, APHTHOUS); NOMA; necrotizing gingivitis (GINGIVITIS, NECROTIZING ULCERATIVE); TOOTHBRUSHING; and various irritants. (From Jablonski, Dictionary of Dentistry, 1992, p842)	Mouth Ulcer,Mouth Ulcers,Oral Ulcers,Ulcer, Mouth,Ulcer, Oral,Ulcers, Mouth,Ulcers, Oral

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