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Hypohidrotic ectodermal dysplasia with autosomal recessive inheritance pattern: Report of a rare and unusual case with a brief review of literature
Ectodermal dysplasia (ED) is a congenital disorder primarily affecting the ectodermal tissue, with infrequent dysfunction of mesodermally derived tissues. Clinically, there are two major forms seen, hypohidrotic/Christ–Siemens–Touraine syndrome and hidrotic/Clouston syndrome, depending on the number...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Wolters Kluwer - Medknow
2019
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6948050/ https://www.ncbi.nlm.nih.gov/pubmed/31942145 http://dx.doi.org/10.4103/jomfp.JOMFP_98_19 |
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author | Hasan, Shamimul Govind, Murali Sawai, Madhuri Alankar Ansari, Mohammad Dilshad |
author_facet | Hasan, Shamimul Govind, Murali Sawai, Madhuri Alankar Ansari, Mohammad Dilshad |
author_sort | Hasan, Shamimul |
collection | PubMed |
description | Ectodermal dysplasia (ED) is a congenital disorder primarily affecting the ectodermal tissue, with infrequent dysfunction of mesodermally derived tissues. Clinically, there are two major forms seen, hypohidrotic/Christ–Siemens–Touraine syndrome and hidrotic/Clouston syndrome, depending on the number and function of sweat glands. A multidisciplinary treatment protocol is usually followed and necessitates collective efforts by medical and dental professionals. Dental intervention should be done as early as possible to ameliorate the patient's esthetics and enhance the emotional and psychological quotient in these patients. This case report aims to highlight a rare and interesting report of hypohidrotic ED in a young female patient with a possible autosomal recessive inheritance pattern. |
format | Online Article Text |
id | pubmed-6948050 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2019 |
publisher | Wolters Kluwer - Medknow |
record_format | MEDLINE/PubMed |
spelling | pubmed-69480502020-01-15 Hypohidrotic ectodermal dysplasia with autosomal recessive inheritance pattern: Report of a rare and unusual case with a brief review of literature Hasan, Shamimul Govind, Murali Sawai, Madhuri Alankar Ansari, Mohammad Dilshad J Oral Maxillofac Pathol Case Report Ectodermal dysplasia (ED) is a congenital disorder primarily affecting the ectodermal tissue, with infrequent dysfunction of mesodermally derived tissues. Clinically, there are two major forms seen, hypohidrotic/Christ–Siemens–Touraine syndrome and hidrotic/Clouston syndrome, depending on the number and function of sweat glands. A multidisciplinary treatment protocol is usually followed and necessitates collective efforts by medical and dental professionals. Dental intervention should be done as early as possible to ameliorate the patient's esthetics and enhance the emotional and psychological quotient in these patients. This case report aims to highlight a rare and interesting report of hypohidrotic ED in a young female patient with a possible autosomal recessive inheritance pattern. Wolters Kluwer - Medknow 2019 /pmc/articles/PMC6948050/ /pubmed/31942145 http://dx.doi.org/10.4103/jomfp.JOMFP_98_19 Text en Copyright: © 2019 Journal of Oral and Maxillofacial Pathology http://creativecommons.org/licenses/by-nc-sa/4.0 This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms. |
spellingShingle | Case Report Hasan, Shamimul Govind, Murali Sawai, Madhuri Alankar Ansari, Mohammad Dilshad Hypohidrotic ectodermal dysplasia with autosomal recessive inheritance pattern: Report of a rare and unusual case with a brief review of literature |
title | Hypohidrotic ectodermal dysplasia with autosomal recessive inheritance pattern: Report of a rare and unusual case with a brief review of literature |
title_full | Hypohidrotic ectodermal dysplasia with autosomal recessive inheritance pattern: Report of a rare and unusual case with a brief review of literature |
title_fullStr | Hypohidrotic ectodermal dysplasia with autosomal recessive inheritance pattern: Report of a rare and unusual case with a brief review of literature |
title_full_unstemmed | Hypohidrotic ectodermal dysplasia with autosomal recessive inheritance pattern: Report of a rare and unusual case with a brief review of literature |
title_short | Hypohidrotic ectodermal dysplasia with autosomal recessive inheritance pattern: Report of a rare and unusual case with a brief review of literature |
title_sort | hypohidrotic ectodermal dysplasia with autosomal recessive inheritance pattern: report of a rare and unusual case with a brief review of literature |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6948050/ https://www.ncbi.nlm.nih.gov/pubmed/31942145 http://dx.doi.org/10.4103/jomfp.JOMFP_98_19 |
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