Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 1 de 1
Filtrar
Más filtros











Base de datos
Intervalo de año de publicación
1.
Am J Med Genet A ; 188(1): 160-177, 2022 01.
Artículo en Inglés | MEDLINE | ID: mdl-34569146

RESUMEN

Robin sequence (RS), the triad of micrognathia, glossoptosis, and airway obstruction, is a major cause of respiratory distress and feeding difficulties in neonates. Robin sequence can be associated with other medical or developmental comorbidities in ~50% of cases ("syndromic" RS). As well, RS is variably associated with cleft palate (CP). Previous studies have not investigated differences in clinical characteristics of children with RS based on presence or absence of CP. We retrospectively reviewed 175 children with RS and compared genetic diagnoses, medical and developmental comorbidities, severity of airway obstruction, and feeding outcomes between those with and without CP. Strikingly, 45 of 45 (100%) children with RS without CP were classified as syndromic due to presence of comorbidities unrelated to RS, while 83 of 130 (64%) children with RS with CP were classified as syndromic. Among 128 children with syndromic RS, there were no differences in severity of airway obstruction, surgical intervention rate or type, or feeding outcome at 12 months based on CP status. Our findings support the conclusion that the pathogenesis of RS without CP is distinct from RS with CP and more likely to cause additional medical or developmental problems. Alternatively, children with RS without CP and without additional anomalies present may be under recognized.


Asunto(s)
Obstrucción de las Vías Aéreas , Fisura del Paladar , Micrognatismo , Síndrome de Pierre Robin , Obstrucción de las Vías Aéreas/diagnóstico , Obstrucción de las Vías Aéreas/genética , Niño , Fisura del Paladar/complicaciones , Fisura del Paladar/diagnóstico , Fisura del Paladar/genética , Humanos , Recién Nacido , Micrognatismo/complicaciones , Síndrome de Pierre Robin/diagnóstico , Síndrome de Pierre Robin/epidemiología , Síndrome de Pierre Robin/genética , Estudios Retrospectivos
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA