1/4. lesch-nyhan syndrome: a preventive approach to self-mutilation.lesch-nyhan syndrome (LNS), a rare inborn error of metabolism, is characterized by self-injurious behaviour, which results in partial or total destruction of oral and perioral tissues and/or fingers and hands. Prevention of self-mutilation raises significant difficulties. Treatment modalities include drug therapy, extraction of teeth, oral appliances and orthognathic surgery, all with variable success. A case of a 10-year-old boy with aggressive behaviour and severe lower lip injuries is presented. A palatal plate fabricated to raise the anterior bite proved to have satisfactory results. Although two adjustments were required during the recall visits, the effectiveness of the appliance in assisting healing of soft tissue damage was immediate and still apparent during the 3 years and 8 month follow-up period.- - - - - - - - - - ranking = 1keywords = extraction (Clic here for more details about this article) |
2/4. Case report: the lesch-nyhan syndrome.BACKGROUND: The lesch-nyhan syndrome (LNS) is a rare x-linked excessive disorder of purine metabolism, caused by the congenital absence of hypoxanthine guanine phosphoribosyl transferase (HGPRT). CASE REPORT: In January 2000 a 2 year old boy was referred to a paediatric dental office in Landshut, germany, because of severe and repeated lip chewing and aggressive tongue biting. A medical history revealed a normal pregnancy with no complications but a diagnosis of muscular hypotonia was made at four months of age. At 18 months a diagnosis of LNS was established through biochemical analysis and molecular examinations. The child displayed self-destructive behaviour, typical in children with LNS. Shortly thereafter the patient was supplied with arm cuffs for self-protection which were not tolerated and the self-mutilation continued. Eventually the extraction of all primary teeth was deemed necessary to prevent additional medical problems for this child. FOLLOW-UP: One year after the dental extractions the patient presented with no bite injuries but was now using his fingers to injure himself.- - - - - - - - - - ranking = 2keywords = extraction (Clic here for more details about this article) |
3/4. Self-mutilation behaviour in lesch-nyhan syndrome.lesch-nyhan syndrome (LNS), first described in 1964 by Lesch and Nyhan, is a rare X-linked genetic disorder involving (near) absence of the enzyme hypoxanthine-guanine phosphoribosyl transferase (HPRT). It occurs in 1:100,000 to 380,000 live births (1, 2). The deficiency of HPRT activity leads to an excessive uric acid production resulting in neurological, renal and musculoskeletal manifestations. death usually occurs in the second or third decade from infection or renal failure. Clinical presentation is characterized by mental retardation, choreoathetosis, spasticity, hyperuricemia and cerebral palsy. A characteristic feature of LNS is the appearance of intractable self-injurious behaviour (SIB), usually in the form of severe lip and finger biting, gouging of eyes, face scratching and head banging requiring extreme management techniques such as the application of restraints and or extraction of teeth at an early age. In this case report a unique approach of SIB in LNS is presented.- - - - - - - - - - ranking = 1keywords = extraction (Clic here for more details about this article) |
4/4. A preventive approach to oral self-mutilation in lesch-nyhan syndrome: a case report.lesch-nyhan syndrome is a rare X-linked recessive disorder of purine metabolism, caused by complete absence of the enzyme hypoxanthine-guanine phosphoribosyl transferase. persons affected with this incurable disease are developmentally and physically delayed, and suffer from self-injurious behavior. The most typical feature results in partial or total destruction of perioral tissues. The purpose of this paper was to discuss a case of lesch-nyhan syndrome with self-mutilative behavior and lip injuries, including some suggestions of a preventive approach avoiding the extraction of teeth. A soft mouthguard fabricated to prevent the destruction of perioral soft tissues and combined psychiatric pharmacologic therapy proved to have satisfactory results.- - - - - - - - - - ranking = 1keywords = extraction (Clic here for more details about this article) |