Fragile X syndrome in a girl with variant Turner syndrome and an isodicentric X chromosome.


Journal

BMJ case reports
ISSN: 1757-790X
Titre abrégé: BMJ Case Rep
Pays: England
ID NLM: 101526291

Informations de publication

Date de publication:
26 Jul 2022
Historique:
pmc-release: 26 07 2024
entrez: 26 7 2022
pubmed: 27 7 2022
medline: 29 7 2022
Statut: epublish

Résumé

Fragile X (FXS) and Turner (TS) syndromes are X-chromosome-associated disorders. Herein, we report the case of a girl in middle childhood with bicuspid aortic valve in infancy, growth failure, global developmental delay (GDD), visual problems, and coexisting attention-deficit/hyperactivity and anxiety disorders. A high-resolution karyotype in 20 cells revealed 46,X,Idic(X)(p11.21)[19]/45,X[1], suggestive of variant TS. Given her atypical phenotype, subsequent DNA testing was performed. Four

Identifiants

pubmed: 35882436
pii: 15/7/e247901
doi: 10.1136/bcr-2021-247901
pmc: PMC9330300
pii:
doi:

Substances chimiques

FMR1 protein, human 0
Fragile X Mental Retardation Protein 139135-51-6
Guanine 5Z93L87A1R

Types de publication

Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

Informations de copyright

© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.

Déclaration de conflit d'intérêts

Competing interests: RH is funded by the Azrieli Foundation for a controlled trial of metformin in FXS. FT received funding from the Azrieli Foundation and from Zynerba for studies in FXS. SS holds the Children’s Miracle Network endowed chair in paediatrics and receives grant support from it. The other authors declare that they have no competing interests.

Auteurs

Nattaporn Tassanakijpanich (N)

Pediatrics, Faculty of Medicine, Prince of Songkla University, Hat Yai, Songkhla, Thailand.
UC Davis MIND Institute, UC Davis Health, Sacramento, California, USA.

Rachel Wright (R)

Biology, Texas A&M University, College Station, Texas, USA.

Flora Tassone (F)

UC Davis MIND Institute, UC Davis Health, Sacramento, California, USA.
Biochemistry and Molecular Medicine, UC Davis, School of Medicine, Davis, California, USA.

Suma P Shankar (SP)

UC Davis MIND Institute, UC Davis Health, Sacramento, California, USA.
Pediatrics, UC Davis, School of Medicine, Sacramento, California, USA.

Randi Hagerman (R)

UC Davis MIND Institute, UC Davis Health, Sacramento, California, USA rjhagerman@ucdavis.edu.
Pediatrics, UC Davis, School of Medicine, Sacramento, California, USA.

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Classifications MeSH