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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...

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Detalles Bibliográficos
Autores principales: Ali, A. M., Mbwasi, R. M., Kinabo, G., Kamsteeg, E.-J., Hamel, B. C., Dekker, M. C. J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi 2017
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.
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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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