In this scoring system, major, minor, and supportive features are represented by macroglossia, omphalocele, placental mesenchymal dysplasia, family history of inheritable BWS, and BWS-related typical tumors (major features); macrosomia, visceromegaly, polyhydramnios, and placentomegaly (minor features); and nephroureteral anomalies, elevated beta-human chorionic gonadotropin (β-hCG)/alpha-fetoprotein (α-FP)/pregnancy-associated plasma protein A (PAPP-A), monozygotic twinning, and pregnancy from ARTs (supportive features) [96]. The gene discussed is PAPPA; the disease is macroglossia.