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Freeman–Sheldon Syndrome: First Molecularly Confirmed Case from Sub-Saharan Africa
We report a case of a male baby who has characteristic signs of Freeman–Sheldon syndrome, a rare but recognizable, severe autosomal dominant form of distal arthrogryposis. Diagnosis was based on the distinctive clinical characteristics of the syndrome and confirmed by genetic analysis that showed a...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5443993/ https://www.ncbi.nlm.nih.gov/pubmed/28584669 http://dx.doi.org/10.1155/2017/9327169 |
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author | Ali, A. M. Mbwasi, R. M. Kinabo, G. Kamsteeg, E.-J. Hamel, B. C. Dekker, M. C. J. |
author_facet | Ali, A. M. Mbwasi, R. M. Kinabo, G. Kamsteeg, E.-J. Hamel, B. C. Dekker, M. C. J. |
author_sort | Ali, A. M. |
collection | PubMed |
description | We report a case of a male baby who has characteristic signs of Freeman–Sheldon syndrome, a rare but recognizable, severe autosomal dominant form of distal arthrogryposis. Diagnosis was based on the distinctive clinical characteristics of the syndrome and confirmed by genetic analysis that showed a de novo missense mutation c.2015G>A (p.Arg672His) of the MYH3 gene. We highlight the different features present in our patient and describe the etiology of the Freeman–Sheldon phenotype and how its clinical complications can be dealt with. To the best of our knowledge, this is the first molecularly confirmed case of Freeman–Sheldon syndrome in sub-Saharan Africa. |
format | Online Article Text |
id | pubmed-5443993 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2017 |
publisher | Hindawi |
record_format | MEDLINE/PubMed |
spelling | pubmed-54439932017-06-05 Freeman–Sheldon Syndrome: First Molecularly Confirmed Case from Sub-Saharan Africa Ali, A. M. Mbwasi, R. M. Kinabo, G. Kamsteeg, E.-J. Hamel, B. C. Dekker, M. C. J. Case Rep Genet Case Report We report a case of a male baby who has characteristic signs of Freeman–Sheldon syndrome, a rare but recognizable, severe autosomal dominant form of distal arthrogryposis. Diagnosis was based on the distinctive clinical characteristics of the syndrome and confirmed by genetic analysis that showed a de novo missense mutation c.2015G>A (p.Arg672His) of the MYH3 gene. We highlight the different features present in our patient and describe the etiology of the Freeman–Sheldon phenotype and how its clinical complications can be dealt with. To the best of our knowledge, this is the first molecularly confirmed case of Freeman–Sheldon syndrome in sub-Saharan Africa. Hindawi 2017 2017-05-11 /pmc/articles/PMC5443993/ /pubmed/28584669 http://dx.doi.org/10.1155/2017/9327169 Text en Copyright © 2017 A. M. Ali et al. https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Ali, A. M. Mbwasi, R. M. Kinabo, G. Kamsteeg, E.-J. Hamel, B. C. Dekker, M. C. J. Freeman–Sheldon Syndrome: First Molecularly Confirmed Case from Sub-Saharan Africa |
title | Freeman–Sheldon Syndrome: First Molecularly Confirmed Case from Sub-Saharan Africa |
title_full | Freeman–Sheldon Syndrome: First Molecularly Confirmed Case from Sub-Saharan Africa |
title_fullStr | Freeman–Sheldon Syndrome: First Molecularly Confirmed Case from Sub-Saharan Africa |
title_full_unstemmed | Freeman–Sheldon Syndrome: First Molecularly Confirmed Case from Sub-Saharan Africa |
title_short | Freeman–Sheldon Syndrome: First Molecularly Confirmed Case from Sub-Saharan Africa |
title_sort | freeman–sheldon syndrome: first molecularly confirmed case from sub-saharan africa |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5443993/ https://www.ncbi.nlm.nih.gov/pubmed/28584669 http://dx.doi.org/10.1155/2017/9327169 |
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