0 Denys-Drash syndrome and Beckwith–Wiedemann syndrome

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Denys-Drash syndrome is a syndrome characterized by the following conditions:
  • gonadal dysgenesis
  • nephropathy
  • Wilms tumor

Beckwith–Wiedemann syndrome (BWS) is an overgrowth disorder present at birth characterized by an increased risk of childhood cancer and certain features.

Five common features used to define BWS are: macroglossia, macrosomia - (birth weight and length >90th percentile), midline abdominal wall defects (omphalocele, umbilical hernia, diastasis recti), ear creases or ear pits, and neonatal hypoglycemia (low blood sugar after birth).

Children with BWS are much more likely (~600 times more) than other children to develop certain childhood cancers, particularly Wilms' tumor (nephroblastoma) and hepatoblastoma.








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