Cantú syndrome: A new case and evolution of clinical conditions during first 2-year follow-up.

ABCC9 gene Cantú syndrome congenital hypertrichosis hypertrichotic osteochondrodysplasia

Journal

Clinical case reports
ISSN: 2050-0904
Titre abrégé: Clin Case Rep
Pays: England
ID NLM: 101620385

Informations de publication

Date de publication:
Mar 2023
Historique:
received: 07 09 2022
revised: 28 12 2022
accepted: 19 01 2023
entrez: 6 3 2023
pubmed: 7 3 2023
medline: 7 3 2023
Statut: epublish

Résumé

Cantú syndrome, or hypertrichotic osteochondrodysplasia, is a rare autosomal dominant disease characterized by congenital hypertrichosis, characteristic dysmorphisms, skeletal abnormalities and cardiomegaly. We report on a 7-year-old girl with congenital generalized hypertrichosis, coarse facial appearance and cardiac involvement, with a de novo heterozygous mutation (c.3461G > A) in the ABCC9 gene. During the annual cardiac follow-up at the age of nine the echocardiogram showed mild left ventricular dilatation in consideration of which she started ramipril treatment. The progression of the clinical manifestations of Cantú syndrome highlights the relevance of an early diagnosis, including genetic analysis, and a multidisciplinary approach with long-term follow-up.

Identifiants

pubmed: 36873080
doi: 10.1002/ccr3.6928
pii: CCR36928
pmc: PMC9979969
doi:

Types de publication

Case Reports

Langues

eng

Pagination

e6928

Informations de copyright

© 2023 The Authors. Clinical Case Reports published by John Wiley & Sons Ltd.

Déclaration de conflit d'intérêts

None.

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Auteurs

Alessandra Mattiucci (A)

Section of Dermatology and Venereology, Department of Medicine University of Verona Verona Italy.

Giampiero Girolomoni (G)

Section of Dermatology and Venereology, Department of Medicine University of Verona Verona Italy.

Matteo Cassina (M)

Clinical Genetics Unit, Department of Women's and Children's Health University of Padova Padova Italy.

Thomas Zoller (T)

Pediatric Clinic, Department Surgical Sciences, Dentistry, Gynecology and Pediatrics University of Verona Verona Italy.

Franco Antoniazzi (F)

Pediatric Clinic, Department Surgical Sciences, Dentistry, Gynecology and Pediatrics University of Verona Verona Italy.

Donatella Schena (D)

Section of Dermatology and Venereology, Department of Medicine University of Verona Verona Italy.

Classifications MeSH