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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...

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Autores principales: Hasan, Shamimul, Govind, Murali, Sawai, Madhuri Alankar, Ansari, Mohammad Dilshad
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2019
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.
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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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