Children with Beckwith-Wiedemann syndrome and idiopathic hemihypertrophy (BWS/HH) are at increased risk for developing Wilms tumor and screening with abdominal sonography is frequently recommended.
However, there is a paucity of published data supporting this strategy.
The purpose of this study was to determine whether sonographic screening at intervals of 4 months or less reduced the proportion of late-stage Wilms Tumor (WT) in children with BWS/HH.
A case series analysis was employed to compare the proportion of late-stage (stage III or IV) Wilms tumor in patients with BWS/HH who were screened with sonography (n=15) to the proportion of late-stage Wilms tumor in unscreened patients with BWS/HH (n=59).
Patients were identified from the BWS Registry and from previously published studies.
Screened patients had sonograms at intervals of 4 months or less.
None of the 12 screened children with Wilms tumor had late-stage disease, whereas 25 of 59 (42%) of unscreened children had late-stage Wilms tumor, a difference that was statistically significant (P<0.003).
Three children had false positive screening studies.
They were operated on for suspected Wilms tumor but the lesions proved to be complicated renal cysts (n=2) or nephroblastomatosis (n=1).
This study suggests that children with BWS/HH may benefit from screening sonograms at intervals of 4 months or less. (...)
Mots-clés Pascal : Beckwith Wiedemann syndrome, Complication, Tumeur Wilms, Echographie, Dépistage, Etats Unis, Amérique du Nord, Amérique, Enfant, Homme, Endocrinopathie, Hypophyse pathologie, Abdomen pathologie, Stomatologie, Syndrome complexe, Malformation, Appareil urinaire pathologie, Rein pathologie, Tumeur maligne, Exploration ultrason
Mots-clés Pascal anglais : Beckwith Wiedemann syndrome, Complication, Wilms tumor, Echography, Medical screening, United States, North America, America, Child, Human, Endocrinopathy, Pituitary diseases, Abdominal disease, Stomatology, Complex syndrome, Malformation, Urinary system disease, Kidney disease, Malignant tumor, Sonography
Notice produite par :
Inist-CNRS - Institut de l'Information Scientifique et Technique
Cote : 99-0164458
Code Inist : 002B04C. Création : 16/11/1999.