Ehlers-Danlos syndrome presenting with primary nocturnal enuresis.

congenital disorders connective tissue disease genetic screening/counselling musculoskeletal and joint disorders urinary and genital tract disorders

Journal

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

Informations de publication

Date de publication:
04 Feb 2020
Historique:
entrez: 7 2 2020
pubmed: 7 2 2020
medline: 8 10 2020
Statut: epublish

Résumé

Ehlers-Danlos syndrome (EDS), hypermobility type, is probably the most common EDS type, as well as the most common heritable connective tissue disorder. Bladder dysfunction is a rare clinical manifestation of EDS and manifests itself as primary nocturnal enuresis. We present a 10-year-old boy referred to the paediatrics nephrology consultation due to primary nocturnal enuresis and day time symptoms of urinary urgency. During the appointment, a tendency to joint hypermobility was noted. On evaluation the skin was hyperextensible and the Beighton score was positive. The genetic testing revealed a variant of the

Identifiants

pubmed: 32024714
pii: 13/2/e231977
doi: 10.1136/bcr-2019-231977
pmc: PMC7021119
pii:
doi:

Substances chimiques

COL5A1 protein, human 0
Collagen Type V 0

Types de publication

Case Reports Journal Article

Langues

eng

Sous-ensembles de citation

IM

Informations de copyright

© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.

Déclaration de conflit d'intérêts

Competing interests: None declared.

Références

J Urol. 2003 Nov;170(5):1971-4
pubmed: 14532836
ISRN Dermatol. 2012;2012:751768
pubmed: 23227356
Eur J Pediatr. 2014 Feb;173(2):197-201
pubmed: 23959325
Curr Opin Pediatr. 2017 Dec;29(6):640-649
pubmed: 28906340

Auteurs

Margarida Cunha (M)

Paediatrics, Centro Hospitalar Barreiro Montijo EPE, Barreiro, Portugal cunhapmargarida@gmail.com.

Mafalda Matias (M)

Paediatrics, Centro Hospitalar Barreiro Montijo EPE, Barreiro, Portugal.

Inês Marques (I)

Paediatrics, Centro Hospitalar Barreiro Montijo EPE, Barreiro, Portugal.

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Classifications MeSH