PMON306 A Multisite Study Evaluating Frequency and Characteristics of Non-Ossifying Fibromas in Children with Congenital Forms of Rickets.
Notice bibliographique
Résumé
Abstract Purpose Non-ossifying fibromas (NOF) are benign, fibrous lesions of the skeleton estimated to occur in about 30% of children and adolescents. In the general pediatric population, they are twice as common in males, usually asymptomatic and self-limiting. Etiology is largely unknown, though multiple NOF are described with genetic syndromes such as neurofibromatosis 1. We have observed NOF in children with various forms of rickets. However, there is limited information on the frequency and characteristics of NOF in rickets. The purpose of this study was to evaluate the frequency of NOF in various congenital forms of rickets under care in tertiary care centers and describe their characteristics. Methods This was a retrospective chart review of patients with congenital forms of rickets and at least one bilateral lower extremity radiograph taken between 4 and 18 years of age at two academic children's hospitals in the United States and Canada between 2004 to 2020. Rickets diagnosis was confirmed by clinical, biochemical, radiographic, and genetic testing where available. Data are described as proportions and percentages for categorical variables and means with standard deviations for continuous variables. Results Sixty-four patients, mean age 13.3 ± 5.1 years old (66%F, 46% Hispanic) were included with the following diagnoses: X-linked Hypophosphatemia (XLH, n=55, 86%), renal hypophosphatemia [n=4, 6% (3 Fanconi syndrome, 1 Dent disease)], hereditary vitamin D resistant rickets (HVDRR, n=3, 5%), and hereditary vitamin D dependent rickets type 1 (VDDR1, n=2, 3%). Fifteen patients (23%, 67%F) had at least one NOF, of whom 13 (87%) had XLH, one (7%) had HVDRR, and one (7%) had VDDR1. All NOF were localized to the lower extremities (60% femur, 27% tibia, and 13% fibula); there were no NOF in the upper extremities or at other sites among those with relevant skeletal imaging (n=32 with upper extremity and/or full skeletal survey data). The mean NOF height was 24.1 ± 13.8 mm and width 10.1 ± 5.3 mm. Nine of 15 patients with NOF (60%) compared with 21/49 without NOF (43%) required angular deformity surgery. Three patients (20%) experienced fractures within the lesions, two (13%) had two lesions each, and one (7%) had a progressive lesion requiring surgery with grafting. Conclusion We describe the frequency of NOF in children with congenital forms of rickets followed in academic centers. NOF were identified in almost a quarter of patients, and patients with NOF required angular surgery more often. Fractures were present within the lesions in one fifth of those with NOF, suggesting clinically important osteomalacia at that site. Interestingly, in contrast to the general pediatric population, we found no male preponderance. Long-term follow up and future studies are important to further characterize NOF in various forms of rickets, their pathogenesis, progression, and association with the disease severity. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.
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Comment cette classification a été obtenuedéplier
Prédiction distillée sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Apprise à partir de 10 348 étiquettes directes de Codex et de 10 348 étiquettes directes de Gemma. Le mode candidate est l'union des têtes enseignantes seuillées; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont ni des étiquettes humaines ni des étiquettes directes de modèles de pointe.
Scores Codex et Gemma par catégorie
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,001 | 0,000 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,001 | 0,000 |
| Bibliométrie | 0,000 | 0,000 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,000 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,000 | 0,000 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule tête enseignante, pas un consensus.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».