Christ Siemens Touraine syndrome: A rare case report.
Identifieur interne : 000189 ( PubMed/Corpus ); précédent : 000188; suivant : 000190Christ Siemens Touraine syndrome: A rare case report.
Auteurs : N. Retnakumari ; Manuja Varghese ; V P KannanSource :
- Journal of the Indian Society of Pedodontics and Preventive Dentistry [ 1998-3905 ]
English descriptors
- KwdEn :
- MESH :
- diagnosis : Ectodermal Dysplasia 1, Anhidrotic.
- etiology : Mouth, Edentulous.
- rehabilitation : Mouth, Edentulous.
- Child, Preschool, Denture, Complete, Diagnosis, Differential, Humans, Male.
Abstract
Christ-Siemens-Touraine (CST) is a rare hereditary disorder of X-linked recessive trait, characterized by abnormal development of two or more structures or tissues of ectodermal origin. The common clinical findings include hypodontia, hypohydrosis, hypotrichosis, and onychodysplasia. Although hypodontia is common, anodontia is a rare feature. Most of the patients are suffering from social rejection and consequent psychological trauma because of the facial dysmorphism and absence of multiple teeth. Oral rehabilitation is of prime importance for such patients. This article presents a case in a 5½-year-old boy presenting with altered manifestations affecting almost all the ectodermal structures like skin, hair, nails, teeth, sebaceous glands, sweat glands, salivary glands, mammary glands, and tear glands. He also had complete anodontia and dry mouth. A multidisciplinary treatment was given to the patient with the collaboration of various health professionals. The child gained confidence and was relieved from the psychological impact following the prosthetic rehabilitation.
DOI: 10.4103/0970-4388.180451
PubMed: 27080972
Links to Exploration step
pubmed:27080972Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en">Christ Siemens Touraine syndrome: A rare case report.</title><author><name sortKey="Retnakumari, N" sort="Retnakumari, N" uniqKey="Retnakumari N" first="N" last="Retnakumari">N. Retnakumari</name><affiliation><nlm:affiliation>Professor and HOD, Department of Pedodontics, Government Dental College, Kozhikode, Kerala, India.</nlm:affiliation></affiliation></author><author><name sortKey="Varghese, Manuja" sort="Varghese, Manuja" uniqKey="Varghese M" first="Manuja" last="Varghese">Manuja Varghese</name></author><author><name sortKey="Kannan, V P" sort="Kannan, V P" uniqKey="Kannan V" first="V P" last="Kannan">V P Kannan</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="????"><PubDate><MedlineDate>2016 Apr-Jun</MedlineDate></PubDate></date><idno type="RBID">pubmed:27080972</idno><idno type="pmid">27080972</idno><idno type="doi">10.4103/0970-4388.180451</idno><idno type="wicri:Area/PubMed/Corpus">000189</idno><idno type="wicri:explorRef" wicri:stream="PubMed" wicri:step="Corpus" wicri:corpus="PubMed">000189</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Christ Siemens Touraine syndrome: A rare case report.</title><author><name sortKey="Retnakumari, N" sort="Retnakumari, N" uniqKey="Retnakumari N" first="N" last="Retnakumari">N. Retnakumari</name><affiliation><nlm:affiliation>Professor and HOD, Department of Pedodontics, Government Dental College, Kozhikode, Kerala, India.</nlm:affiliation></affiliation></author><author><name sortKey="Varghese, Manuja" sort="Varghese, Manuja" uniqKey="Varghese M" first="Manuja" last="Varghese">Manuja Varghese</name></author><author><name sortKey="Kannan, V P" sort="Kannan, V P" uniqKey="Kannan V" first="V P" last="Kannan">V P Kannan</name></author></analytic><series><title level="j">Journal of the Indian Society of Pedodontics and Preventive Dentistry</title><idno type="eISSN">1998-3905</idno></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Child, Preschool</term><term>Denture, Complete</term><term>Diagnosis, Differential</term><term>Ectodermal Dysplasia 1, Anhidrotic (diagnosis)</term><term>Humans</term><term>Male</term><term>Mouth, Edentulous (etiology)</term><term>Mouth, Edentulous (rehabilitation)</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Ectodermal Dysplasia 1, Anhidrotic</term></keywords><keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Mouth, Edentulous</term></keywords><keywords scheme="MESH" qualifier="rehabilitation" xml:lang="en"><term>Mouth, Edentulous</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Child, Preschool</term><term>Denture, Complete</term><term>Diagnosis, Differential</term><term>Humans</term><term>Male</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Christ-Siemens-Touraine (CST) is a rare hereditary disorder of X-linked recessive trait, characterized by abnormal development of two or more structures or tissues of ectodermal origin. The common clinical findings include hypodontia, hypohydrosis, hypotrichosis, and onychodysplasia. Although hypodontia is common, anodontia is a rare feature. Most of the patients are suffering from social rejection and consequent psychological trauma because of the facial dysmorphism and absence of multiple teeth. Oral rehabilitation is of prime importance for such patients. This article presents a case in a 5½-year-old boy presenting with altered manifestations affecting almost all the ectodermal structures like skin, hair, nails, teeth, sebaceous glands, sweat glands, salivary glands, mammary glands, and tear glands. He also had complete anodontia and dry mouth. A multidisciplinary treatment was given to the patient with the collaboration of various health professionals. The child gained confidence and was relieved from the psychological impact following the prosthetic rehabilitation.</div></front></TEI><pubmed><MedlineCitation Status="MEDLINE" Owner="NLM"><PMID Version="1">27080972</PMID><DateCompleted><Year>2017</Year><Month>07</Month><Day>24</Day></DateCompleted><DateRevised><Year>2017</Year><Month>07</Month><Day>24</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Electronic">1998-3905</ISSN><JournalIssue CitedMedium="Internet"><Volume>34</Volume><Issue>2</Issue><PubDate><MedlineDate>2016 Apr-Jun</MedlineDate></PubDate></JournalIssue><Title>Journal of the Indian Society of Pedodontics and Preventive Dentistry</Title><ISOAbbreviation>J Indian Soc Pedod Prev Dent</ISOAbbreviation></Journal><ArticleTitle>Christ Siemens Touraine syndrome: A rare case report.</ArticleTitle><Pagination><MedlinePgn>185-8</MedlinePgn></Pagination><ELocationID EIdType="doi" ValidYN="Y">10.4103/0970-4388.180451</ELocationID><Abstract><AbstractText>Christ-Siemens-Touraine (CST) is a rare hereditary disorder of X-linked recessive trait, characterized by abnormal development of two or more structures or tissues of ectodermal origin. The common clinical findings include hypodontia, hypohydrosis, hypotrichosis, and onychodysplasia. Although hypodontia is common, anodontia is a rare feature. Most of the patients are suffering from social rejection and consequent psychological trauma because of the facial dysmorphism and absence of multiple teeth. Oral rehabilitation is of prime importance for such patients. This article presents a case in a 5½-year-old boy presenting with altered manifestations affecting almost all the ectodermal structures like skin, hair, nails, teeth, sebaceous glands, sweat glands, salivary glands, mammary glands, and tear glands. He also had complete anodontia and dry mouth. A multidisciplinary treatment was given to the patient with the collaboration of various health professionals. The child gained confidence and was relieved from the psychological impact following the prosthetic rehabilitation.</AbstractText></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Retnakumari</LastName><ForeName>N</ForeName><Initials>N</Initials><AffiliationInfo><Affiliation>Professor and HOD, Department of Pedodontics, Government Dental College, Kozhikode, Kerala, India.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Varghese</LastName><ForeName>Manuja</ForeName><Initials>M</Initials></Author><Author ValidYN="Y"><LastName>Kannan</LastName><ForeName>V P</ForeName><Initials>VP</Initials></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D002363">Case Reports</PublicationType><PublicationType UI="D016428">Journal Article</PublicationType></PublicationTypeList></Article><MedlineJournalInfo><Country>India</Country><MedlineTA>J Indian Soc Pedod Prev Dent</MedlineTA><NlmUniqueID>8710631</NlmUniqueID><ISSNLinking>0970-4388</ISSNLinking></MedlineJournalInfo><CitationSubset>D</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName UI="D002675" MajorTopicYN="N">Child, Preschool</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D003824" MajorTopicYN="N">Denture, Complete</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D003937" MajorTopicYN="N">Diagnosis, Differential</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D053358" MajorTopicYN="N">Ectodermal Dysplasia 1, Anhidrotic</DescriptorName><QualifierName UI="Q000175" MajorTopicYN="Y">diagnosis</QualifierName></MeshHeading><MeshHeading><DescriptorName UI="D006801" MajorTopicYN="N">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D008297" MajorTopicYN="N">Male</DescriptorName></MeshHeading><MeshHeading><DescriptorName UI="D009066" MajorTopicYN="N">Mouth, Edentulous</DescriptorName><QualifierName UI="Q000209" MajorTopicYN="Y">etiology</QualifierName><QualifierName UI="Q000534" MajorTopicYN="Y">rehabilitation</QualifierName></MeshHeading></MeshHeadingList></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="entrez"><Year>2016</Year><Month>4</Month><Day>16</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="pubmed"><Year>2016</Year><Month>4</Month><Day>16</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2017</Year><Month>7</Month><Day>25</Day><Hour>6</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">27080972</ArticleId><ArticleId IdType="pii">JIndianSocPedodPrevDent_2016_34_2_185_180451</ArticleId><ArticleId IdType="doi">10.4103/0970-4388.180451</ArticleId></ArticleIdList></PubmedData></pubmed></record>Pour manipuler ce document sous Unix (Dilib)
EXPLOR_STEP=$WICRI_ROOT/Wicri/Santé/explor/EdenteV2/Data/PubMed/Corpus
HfdSelect -h $EXPLOR_STEP/biblio.hfd -nk 000189 | SxmlIndent | more
Ou
HfdSelect -h $EXPLOR_AREA/Data/PubMed/Corpus/biblio.hfd -nk 000189 | SxmlIndent | more
Pour mettre un lien sur cette page dans le réseau Wicri
{{Explor lien
|wiki= Wicri/Santé
|area= EdenteV2
|flux= PubMed
|étape= Corpus
|type= RBID
|clé= pubmed:27080972
|texte= Christ Siemens Touraine syndrome: A rare case report.
}}
Pour générer des pages wiki
HfdIndexSelect -h $EXPLOR_AREA/Data/PubMed/Corpus/RBID.i -Sk "pubmed:27080972" \
| HfdSelect -Kh $EXPLOR_AREA/Data/PubMed/Corpus/biblio.hfd \
| NlmPubMed2Wicri -a EdenteV2
| This area was generated with Dilib version V0.6.32. |  |