Lesch-Nyhan syndrome and the lower lip guard

Evans, Jeff; Sirikumara, Madurasinghe; Gregory, M C

doi:10.1016/0030-4220(93)90009-s

Cited by 21 publications

(27 citation statements)

References 7 publications

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“…Evans et al (1993), descrevem o comportamento auto mutilante e condutas de tratamento em um paciente de 11 anos de idade, com a síndrome de Lesch Nyhan, com hábitos de auto mordidas persistentes em lábio inferior e região jugal. Inicialmente optou-se pelas extrações de alguns dentes decíduos e do primeiro molar permanente.…”

Section: Discussionunclassified

Síndrome de Lesch Nyhan e Odontologia: relato de caso

Andrade¹,

Rangel²,

Dultra³

et al. 2014

cmbio

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ResumoIntrodução: Síndrome de Lesch Nyhan é uma doença ligada ao cromossomo X, causada pela ausência ou deficiência da enzima hipoxantina fosforibosil transferase (HPRT) e que apresenta como uma das principais características a autoagressão compulsiva, como mordedura dos lábios, língua e dedos. Objetivo: Relatar, através de um de caso clinico, a atuação do cirurgião dentista e a conduta de tratamento odontológico para melhoria na qualidade de vida de pacientes que apresentam essa síndrome. Metodologia: O paciente IAS, 06 anos com diagnóstico comprovado de sindrome de Lesch Nyran, associado a presença de retardo mental. Apresentava-se com amputações traumáticas em falanges distais das mãos por auto mordidas, bem como também lesões oculares provocadas pelo próprio paciente. As automutilações estavam progredindo e a mãe referiu que não conseguia o controle do hábitos deletérios, sendo assim, foi-se discutido com os responsáveis legais a possibilidade de remoção de todos os dentes decíduos presentes e com a concordância destes o procedimento odontológico foi realizado. Conclusão: Não há métodos padronizados para a prevenção de auto mutilação, pois existem diferentes abordagens. Há autores que optam por tratamento conservador, outros por abordagem cirúrgicas, onde o cirurgião dentista pode ser chamado a intervir, por meio de exodontias múltiplas, no intuito de melhorar a qualidade de vida e limitar os danos provocados pelas mutilações por auto mordidas. Palavras

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Section: Discussionunclassified

Síndrome de Lesch Nyhan e Odontologia: relato de caso

Andrade¹,

Rangel²,

Dultra³

et al. 2014

cmbio

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“…Over the years many inventive and effective approaches involving an assortment of hand restraints, dental mouth guards, and dental extractions have been utilized 4,5,11 and with appropriate allopurinol treatment, renal function is generally preserved. It has no reported effect on behavioral and neurological symptoms.…”

Section: Discussionmentioning

confidence: 99%

“…15,16 Currently, there is no cure for the syndrome; prevention is therefore the standard of care. Over the years many inventive and effective approaches involving an assortment of hand restraints, dental mouth guards, and dental extractions have been utilized 4,5,11 and with appropriate allopurinol treatment, renal function is generally preserved. 17 LNS is usually associated with renal calculi and renal failure because of hyperuricemia and it has been established by several studies but vesicoureteric reflux as an association with LNS has not been reported till date so far.…”

Section: Introductionmentioning

confidence: 99%

“…A consistent presentation in all cases of LNS is the abnormal compulsion toward selfmutilation usually reported after 1 year of age. 4,11 Perioral self-mutilating behavior is thought to begin with the eruption of teeth; patients then begin to bite themselves. The behavior continues and results in partial or total destruction of perioral tissue especially the lower lip.…”

Section: Introductionmentioning

confidence: 99%

“…3 The loss of enzyme function is caused by gene mutations on the long arm of the X-chromosome. 4 This enzyme catalyses the reaction of hypoxanthine or guanine with phosphoribosyl pyrophosphateto form inosinic and guanylic acids. The increased production of purine leads to an accumulation of uric acid in the blood that leading to hyperuricemia.…”

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

Bilateral Vesicoureteric Reflux may be a Rare Association in Lesch-Nyhan Syndrome: A Case Report

Rahman

Anjum

Biswas

et al. 2017

Bangladesh J Child Health

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Consequences of Delayed Dental Extraction in Lesch‐Nyhan Disease

Goodman

Torres

Puig

et al. 2014

Movement Disord Clin Pract

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Patients with Lesch-Nyhan disease (LND) often engage in self-injurious biting. This problem requires difficult management choices, sometimes including removal of the teeth. Although many health care professionals are reluctant to remove teeth in a child because of the permanent negative cosmetic consequences of the edentulous state, disfigurement of the face and tongue from self-biting can be worse. We analyzed the records of 5 LND patients who used mouth guards to spare the teeth. Success was variable, and dental extraction ultimately was required in 4 cases. We also reviewed previously published cases on the use of dental devices to spare teeth in LND. Various devices have been recommended, but failure rates are high, and tooth extraction often is still needed. Although dental extraction is not required in all cases, it should not be delayed when biting is severe.

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Lesch-Nyhan syndrome and the lower lip guard

Cited by 21 publications

References 7 publications

Síndrome de Lesch Nyhan e Odontologia: relato de caso

Síndrome de Lesch Nyhan e Odontologia: relato de caso

Bilateral Vesicoureteric Reflux may be a Rare Association in Lesch-Nyhan Syndrome: A Case Report

Consequences of Delayed Dental Extraction in Lesch‐Nyhan Disease

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