Evolution over Time of Leg Length Discrepancy in Patients with Syndromic and Isolated Lateralized Overgrowth.
Beckwith-Wiedemann syndrome
hemihyperplasia
hemihypertrophy
lateralized overgrowth
leg length discrepancy
overgrowth
Journal
The Journal of pediatrics
ISSN: 1097-6833
Titre abrégé: J Pediatr
Pays: United States
ID NLM: 0375410
Informations de publication
Date de publication:
07 2021
07 2021
Historique:
received:
02
09
2020
revised:
09
01
2021
accepted:
12
01
2021
pubmed:
20
1
2021
medline:
9
11
2021
entrez:
19
1
2021
Statut:
ppublish
Résumé
To provide information on evolution over time of leg length discrepancy in patients with syndromic and isolated lateralized overgrowth. This retrospective study investigates leg length discrepancy longitudinally in 105 patients with lateralized overgrowth either isolated (n = 37) or associated with Beckwith-Wiedemann spectrum (n = 56) or PIK3CA-related overgrowth spectrum (n = 12). Discrepancy was measured by standard methods and categorized as minor, mild, severe, and critical, based on the thresholds of 1, 2 and 5, respectively. The period of observation from diagnosis was 1.7 ± 2.6 to 9.0 ± 6.0 years. Leg length discrepancy was 11.0 ± 7.2 mm at diagnosis and 17.1 ± 14.4 mm at last visit. Both final leg length discrepancy and change over time were correlated with discrepancy at diagnosis (r Leg length discrepancy associated with isolated or syndromic lateralized overgrowth tends to worsen with growth and correlates with discrepancy at first observation. Among the genotypic groups, isolated lateralized overgrowth tends to have a milder evolution, whereas Beckwith-Wiedemann spectrum predisposes to a more severe outcome, especially if associated with paternal chromosome 11 uniparental disomy genotype.
Identifiants
pubmed: 33465347
pii: S0022-3476(21)00027-5
doi: 10.1016/j.jpeds.2021.01.020
pii:
doi:
Types de publication
Journal Article
Comment
Langues
eng
Sous-ensembles de citation
IM
Pagination
123-127Commentaires et corrections
Type : CommentIn
Type : CommentOn
Informations de copyright
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