Consequences of Delayed Dental Extraction in Lesch-Nyhan Disease
Identifieur interne : 000406 ( Pmc/Corpus ); précédent : 000405; suivant : 000407Consequences of Delayed Dental Extraction in Lesch-Nyhan Disease
Auteurs : Emily M. Goodman ; Rosa J. Torres ; Juan G. Puig ; H. A. JinnahSource :
- Movement disorders clinical practice (Hoboken, N.J.) [ 2330-1619 ] ; 2014.
Abstract
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.
Url:
DOI: 10.1002/mdc3.12040
PubMed: 25419535
PubMed Central: 4237204
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PMC:4237204Le document en format XML
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<author><name sortKey="Goodman, Emily M" sort="Goodman, Emily M" uniqKey="Goodman E" first="Emily M." last="Goodman">Emily M. Goodman</name>
<affiliation><nlm:aff id="A1">Department of Neurology, Emory University, Atlanta, Georgia 30322 USA</nlm:aff>
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<author><name sortKey="Torres, Rosa J" sort="Torres, Rosa J" uniqKey="Torres R" first="Rosa J." last="Torres">Rosa J. Torres</name>
<affiliation><nlm:aff id="A2">Division of Clinical Biochemistry, Hospital Universitario La Paz, IdiPaz, Madrid, Spain</nlm:aff>
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<author><name sortKey="Jinnah, H A" sort="Jinnah, H A" uniqKey="Jinnah H" first="H. A." last="Jinnah">H. A. Jinnah</name>
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<author><name sortKey="Puig, Juan G" sort="Puig, Juan G" uniqKey="Puig J" first="Juan G" last="Puig">Juan G. Puig</name>
<affiliation><nlm:aff id="A3">Division of Internal Medicine, Hospital Universitario La Paz, IdiPaz, Madrid, Spain</nlm:aff>
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<author><name sortKey="Jinnah, H A" sort="Jinnah, H A" uniqKey="Jinnah H" first="H. A." last="Jinnah">H. A. Jinnah</name>
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<front><div type="abstract" xml:lang="en"><p id="P1">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.</p>
</div>
</front>
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<pmc article-type="research-article"><pmc-comment>The publisher of this article does not allow downloading of the full text in XML form.</pmc-comment>
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<front><journal-meta><journal-id journal-id-type="nlm-journal-id">101630279</journal-id>
<journal-id journal-id-type="pubmed-jr-id">42422</journal-id>
<journal-id journal-id-type="nlm-ta">Mov Disord Clin Pract (Hoboken)</journal-id>
<journal-id journal-id-type="iso-abbrev">Mov Disord Clin Pract (Hoboken)</journal-id>
<journal-title-group><journal-title>Movement disorders clinical practice (Hoboken, N.J.)</journal-title>
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<article-categories><subj-group subj-group-type="heading"><subject>Article</subject>
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<title-group><article-title>Consequences of Delayed Dental Extraction in Lesch-Nyhan Disease</article-title>
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<contrib-group><contrib contrib-type="author"><name><surname>Goodman</surname>
<given-names>Emily M.</given-names>
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<degrees>BS</degrees>
<xref ref-type="aff" rid="A1">1</xref>
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<contrib contrib-type="author"><name><surname>Torres</surname>
<given-names>Rosa J.</given-names>
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<degrees>MD</degrees>
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<given-names>Juan G</given-names>
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<degrees>MD</degrees>
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<aff id="A1"><label>1</label>
Department of Neurology, Emory University, Atlanta, Georgia 30322 USA</aff>
<aff id="A2"><label>2</label>
Division of Clinical Biochemistry, Hospital Universitario La Paz, IdiPaz, Madrid, Spain</aff>
<aff id="A3"><label>3</label>
Division of Internal Medicine, Hospital Universitario La Paz, IdiPaz, Madrid, Spain</aff>
<aff id="A4"><label>4</label>
Departments of Neurology, Human Genetics & Pediatrics, Emory University, Atlanta, Georgia 30322 USA</aff>
<author-notes><corresp id="cor1"><bold>Correspondence:</bold>
H. A. Jinnah, M.D., Ph.D., Professor, Departments of Neurology, Human Genetics & Pediatrics, Emory University, Suite 6300 Woodruff Memorial Building, 101 Woodruff Circle, Atlanta GA, 30322, Phone: 404-727-9107, Fax: 404-712-8576, <email>hjinnah@emory.edu</email>
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<pub-date pub-type="nihms-submitted"><day>31</day>
<month>5</month>
<year>2014</year>
</pub-date>
<pub-date pub-type="ppub"><month>9</month>
<year>2014</year>
</pub-date>
<pub-date pub-type="pmc-release"><day>01</day>
<month>9</month>
<year>2015</year>
</pub-date>
<volume>1</volume>
<issue>3</issue>
<fpage>225</fpage>
<lpage>229</lpage>
<pmc-comment>elocation-id from pubmed: 10.1002/mdc3.12040</pmc-comment>
<abstract><p id="P1">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.</p>
</abstract>
<kwd-group><kwd>Lesch-Nyhan disease</kwd>
<kwd>developmental disorders</kwd>
<kwd>inherited metabolic disease</kwd>
<kwd>self-injurious behavior</kwd>
<kwd>self-biting</kwd>
<kwd>review</kwd>
</kwd-group>
</article-meta>
</front>
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