Treatment package to reduce SIB in a Lesch-Nyhan patient. 1984

S K Wurtele, and A C King, and R S Drabman

Self-destructive behaviour in LNS children results in a tremendous burden to parents, teachers, staff members, and other responsible for their care. As constant vigilance is not always feasible, developing alternate methods of managing SIB becomes imperative. This report presents our efforts to improve the care of an LNS child and to ease the burden on staff members and parents by obtaining special devices (e.g. the custom-made mouthguard and the gloves) to prevent him from injuring himself. Others (e.g. Letts & Hobson, 1975) have likewise reported success in fabricating custom-designed chairs and devices in an effort to manage SIB in LNS children. Their devices were, however, more elaborate and much more expensive than the ones used in this report. The cost of obtaining a mouthguard similar to the one used with K. is estimated at between $20-$30 (US), while the gloves cost only $14.00. The major advantages of using such devices are that they safely allow employment of the extinction procedure along with allowing the child to participate more fully in activities of daily living. It is recognized that K. was in some ways an atypical Lesch-Nyhan child in that he possessed low normal receptive intelligence and scored fairly high (relative to most LNS children) on a test of verbal intelligence. His cognitive and verbal abilities made him a unique case in that he responded favourably to therapeutic instructions regarding relaxation and self-control tactics. The therapists were able to rely on K.'s verbalizations and feelings about his biting in structuring the treatment approach.(ABSTRACT TRUNCATED AT 250 WORDS)

UI MeSH Term Description Entries
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
D008297 Male Males
D001734 Bites, Human Bites inflicted by humans. Bite, Human,Human Bite,Human Bites
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000293 Adolescent A person 13 to 18 years of age. Adolescence,Youth,Adolescents,Adolescents, Female,Adolescents, Male,Teenagers,Teens,Adolescent, Female,Adolescent, Male,Female Adolescent,Female Adolescents,Male Adolescent,Male Adolescents,Teen,Teenager,Youths
D001521 Behavior Therapy The application of modern theories of learning and conditioning in the treatment of behavior disorders. Behavior Change Techniques,Behavior Modification,Behavior Treatment,Conditioning Therapy,Therapy, Behavior,Therapy, Conditioning,Behavior Change Technique,Behavior Modifications,Behavior Therapies,Conditioning Therapies,Modification, Behavior,Technique, Behavior Change,Treatment, Behavior
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

Related Publications

S K Wurtele, and A C King, and R S Drabman
June 1992, Developmental medicine and child neurology,
S K Wurtele, and A C King, and R S Drabman
March 2012, Revista espanola de anestesiologia y reanimacion,
S K Wurtele, and A C King, and R S Drabman
January 2002, Revista de neurologia,
S K Wurtele, and A C King, and R S Drabman
September 1989, The Journal of clinical investigation,
S K Wurtele, and A C King, and R S Drabman
June 1982, Pediatric dentistry,
S K Wurtele, and A C King, and R S Drabman
January 1991, Advances in experimental medicine and biology,
S K Wurtele, and A C King, and R S Drabman
July 2014, The American journal of medicine,
S K Wurtele, and A C King, and R S Drabman
January 1994, Advances in experimental medicine and biology,
S K Wurtele, and A C King, and R S Drabman
February 1980, Developmental medicine and child neurology,
S K Wurtele, and A C King, and R S Drabman
January 1968, Federation proceedings,
Copied contents to your clipboard!