Hypohidrotic Ectodermal Dysplasia (ED): A Case Series
Identifieur interne : 001429 ( Pmc/Checkpoint ); précédent : 001428; suivant : 001430Hypohidrotic Ectodermal Dysplasia (ED): A Case Series
Auteurs : Mallika Kishore ; Sunil R. Panat ; Ashish Aggarwal ; Nupur Agarwal ; Nitin Upadhyay ; Kratika Ajai ; Abhijeet AlokSource :
- Journal of Clinical and Diagnostic Research : JCDR [ 2249-782X ] ; 2014.
Abstract
Ectodermal Dysplasia (ED) is a rare disorder with defects in two or more of the following structures: the teeth and the skin and its appendages including hair, nails, eccrine, and sebaceous glands. Dental manifestations include hypodontia, complete anodontia or malformed teeth. The most common form of the ED syndrome is hypohidrotic ED and is usually inherited as an X-linked recessive trait. Female carriers may have a variable degree of clinical manifestations. The condition is thought to occur in approximately 1 in every 100,000 live births. Dental treatment for these patients varies on an individual basis. Children with ED are often treated dentally with conventional adult appearing prosthesis which are focused only on the oral manifestations of the syndrome. We are here reporting two classical cases of hypohidrotic ED with a review of the literature.
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DOI: 10.7860/JCDR/2014/6597.3951
PubMed: 24596794
PubMed Central: 3939504
Affiliations:
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<front><div type="abstract" xml:lang="en"><p>Ectodermal Dysplasia (ED) is a rare disorder with defects in two or more of the following structures: the teeth and the skin and its appendages including hair, nails, eccrine, and sebaceous glands. Dental manifestations include hypodontia, complete anodontia or malformed teeth. The most common form of the ED syndrome is hypohidrotic ED and is usually inherited as an X-linked recessive trait. Female carriers may have a variable degree of clinical manifestations. The condition is thought to occur in approximately 1 in every 100,000 live births. Dental treatment for these patients varies on an individual basis. Children with ED are often treated dentally with conventional adult appearing prosthesis which are focused only on the oral manifestations of the syndrome. We are here reporting two classical cases of hypohidrotic ED with a review of the literature.</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>
<front><journal-meta><journal-id journal-id-type="nlm-ta">J Clin Diagn Res</journal-id>
<journal-id journal-id-type="iso-abbrev">J Clin Diagn Res</journal-id>
<journal-id journal-id-type="hwp">Journal of Clinical and Diagnostic Research</journal-id>
<journal-id journal-id-type="publisher-id">JCDR</journal-id>
<journal-title-group><journal-title>Journal of Clinical and Diagnostic Research : JCDR</journal-title>
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<issn pub-type="ppub">2249-782X</issn>
<issn pub-type="epub">0973-709X</issn>
<publisher><publisher-name>JCDR Research and Publications (P) Limited</publisher-name>
<publisher-loc>Delhi, India</publisher-loc>
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<article-id pub-id-type="doi">10.7860/JCDR/2014/6597.3951</article-id>
<article-categories><subj-group subj-group-type="heading"><subject>Dentistry</subject>
<subj-group subj-group-type="heading"><subject>Dental Case Report</subject>
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</subj-group>
</article-categories>
<title-group><article-title>Hypohidrotic Ectodermal Dysplasia (ED): A Case Series</article-title>
</title-group>
<contrib-group><contrib contrib-type="author"><name><surname>Kishore</surname>
<given-names>Mallika</given-names>
</name>
<xref ref-type="aff" rid="aff1">1</xref>
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<contrib contrib-type="author"><name><surname>Panat</surname>
<given-names>Sunil R.</given-names>
</name>
<xref ref-type="aff" rid="aff2">2</xref>
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<contrib contrib-type="author"><name><surname>Aggarwal</surname>
<given-names>Ashish</given-names>
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<xref ref-type="aff" rid="aff3">3</xref>
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<contrib contrib-type="author"><name><surname>Agarwal</surname>
<given-names>Nupur</given-names>
</name>
<xref ref-type="aff" rid="aff4">4</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Upadhyay</surname>
<given-names>Nitin</given-names>
</name>
<xref ref-type="aff" rid="aff5">5</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Ajai</surname>
<given-names>Kratika</given-names>
</name>
<xref ref-type="aff" rid="aff6">6</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Alok</surname>
<given-names>Abhijeet</given-names>
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<aff id="aff1"><label>1</label>
Post Graduate Student, Department of Oral Medicine and Radiology,<institution>Institute of Dental Sciences</institution>
,<addr-line>Bareilly, U.P., India</addr-line>
.</aff>
<aff id="aff2"><label>2</label>
Professor & Head of Department, Department of Oral Medicine and Radiology,<institution>Institute of Dental Sciences</institution>
,<addr-line>Bareilly, U.P., India</addr-line>
.</aff>
<aff id="aff3"><label>3</label>
Senior Lecturer, Department of Oral Medicine and Radiology,<institution>Institute of Dental Sciences</institution>
,<addr-line>Bareilly, U.P., India</addr-line>
.</aff>
<aff id="aff4"><label>4</label>
Senior Lecturer, Department of Oral Medicine and Radiology,<institution>Institute of Dental Sciences</institution>
,<addr-line>Bareilly, U.P., India</addr-line>
.</aff>
<aff id="aff5"><label>5</label>
Senior Lecturer, Department of Oral Medicine and Radiology,<institution>Institute of Dental Sciences</institution>
,<addr-line>Bareilly, U.P., India</addr-line>
.</aff>
<aff id="aff6"><label>6</label>
Post Graduate Student, Department of Oral Medicine and Radiology,<institution>Institute of Dental Sciences</institution>
,<addr-line>Bareilly, U.P., India</addr-line>
.</aff>
<aff id="aff7"><label>7</label>
Post Graduate Student, Department of Oral Medicine and Radiology,<institution>Institute of Dental Sciences</institution>
,<addr-line>Bareilly, U.P., India</addr-line>
.</aff>
<author-notes><corresp id="cor1">NAME, ADDRESS, E-MAIL ID OF THE CORRESPONDING AUTHOR: Dr. Mallika Kishore, Post Graduate Student, Department of Oral Medicine and Radiology, Institute of Dental Sciences, Bareilly, U.P., India. E-mail: <email>dr.mallika.kishore01@gmail.com </email>
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<year>2014</year>
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<pub-date pub-type="epub"><day>12</day>
<month>1</month>
<year>2014</year>
</pub-date>
<volume>8</volume>
<issue>1</issue>
<fpage>273</fpage>
<lpage>275</lpage>
<history><date date-type="received"><day>31</day>
<month>5</month>
<year>2013</year>
</date>
<date date-type="rev-request"><day>25</day>
<month>10</month>
<year>2013</year>
</date>
<date date-type="accepted"><day>19</day>
<month>12</month>
<year>2013</year>
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</history>
<permissions><copyright-statement>© 2014 Journal of Clinical and Diagnostic Research</copyright-statement>
<copyright-year>2014</copyright-year>
</permissions>
<abstract><p>Ectodermal Dysplasia (ED) is a rare disorder with defects in two or more of the following structures: the teeth and the skin and its appendages including hair, nails, eccrine, and sebaceous glands. Dental manifestations include hypodontia, complete anodontia or malformed teeth. The most common form of the ED syndrome is hypohidrotic ED and is usually inherited as an X-linked recessive trait. Female carriers may have a variable degree of clinical manifestations. The condition is thought to occur in approximately 1 in every 100,000 live births. Dental treatment for these patients varies on an individual basis. Children with ED are often treated dentally with conventional adult appearing prosthesis which are focused only on the oral manifestations of the syndrome. We are here reporting two classical cases of hypohidrotic ED with a review of the literature.</p>
</abstract>
<kwd-group><kwd>Ectodermal dysplasia</kwd>
<kwd>Genetic disorder</kwd>
<kwd>Hypohidrotic</kwd>
</kwd-group>
</article-meta>
</front>
</pmc>
<affiliations><list></list>
<tree><noCountry><name sortKey="Agarwal, Nupur" sort="Agarwal, Nupur" uniqKey="Agarwal N" first="Nupur" last="Agarwal">Nupur Agarwal</name>
<name sortKey="Aggarwal, Ashish" sort="Aggarwal, Ashish" uniqKey="Aggarwal A" first="Ashish" last="Aggarwal">Ashish Aggarwal</name>
<name sortKey="Ajai, Kratika" sort="Ajai, Kratika" uniqKey="Ajai K" first="Kratika" last="Ajai">Kratika Ajai</name>
<name sortKey="Alok, Abhijeet" sort="Alok, Abhijeet" uniqKey="Alok A" first="Abhijeet" last="Alok">Abhijeet Alok</name>
<name sortKey="Kishore, Mallika" sort="Kishore, Mallika" uniqKey="Kishore M" first="Mallika" last="Kishore">Mallika Kishore</name>
<name sortKey="Panat, Sunil R" sort="Panat, Sunil R" uniqKey="Panat S" first="Sunil R." last="Panat">Sunil R. Panat</name>
<name sortKey="Upadhyay, Nitin" sort="Upadhyay, Nitin" uniqKey="Upadhyay N" first="Nitin" last="Upadhyay">Nitin Upadhyay</name>
</noCountry>
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