In Beckwith-Wiedemann syndrome presenting with omphalocele and macroglossia, which metabolic abnormality is commonly observed?

In Beckwith-Wiedemann syndrome presenting with omphalocele and macroglossia, which metabolic abnormality is commonly observed?

• A. Hyperglycemia
• B. Hypoglycemia
• C. Hyperkalemia
• D. Hypocalcemia

Answer: (B)

Beckwith-Wiedemann syndrome often involves pancreatic islet cell hyperplasia that causes hyperinsulinism after birth. This excess insulin drives glucose into cells, leading to low blood sugar in the newborn. So, hypoglycemia is the metabolic abnormality most commonly observed in this scenario. In practice, anticipate low glucose, monitor frequently, and treat with early feeds or IV dextrose to keep blood sugar in a safe range during the neonatal period or perioperative care. Hyperglycemia, hyperkalemia, or hypocalcemia are not typical metabolic findings associated with this presentation.

Beckwith-Wiedemann syndrome often involves pancreatic islet cell hyperplasia that causes hyperinsulinism after birth. This excess insulin drives glucose into cells, leading to low blood sugar in the newborn. So, hypoglycemia is the metabolic abnormality most commonly observed in this scenario. In practice, anticipate low glucose, monitor frequently, and treat with early feeds or IV dextrose to keep blood sugar in a safe range during the neonatal period or perioperative care. Hyperglycemia, hyperkalemia, or hypocalcemia are not typical metabolic findings associated with this presentation.