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Slovenska pediatrija 2022; 29: 72-77

https://doi.org/10.38031/slovpediatr-2022-2-03en

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Case report

PERSISTENT HYPOGLYCAEMIA IN A NEWBORN – CASE REPORT OF A GIRL WITH BECKWITH-WIEDEMANN SYNDROME

Mojca Kavčič
Klinični oddelek za neonatologijo, Pediatrična klinika, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija

Jana Lozar Krivec
Klinični oddelek za neonatologijo, Pediatrična klinika, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija

Magdalena Avbelj Stefanija
Klinični oddelek za endokrinologijo, diabetes in presnovne bolezni, Pediatrična klinika, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija

Abstract

Hypoglycaemia is a common metabolic disorder in new-borns. It is defined as a blood sugar level below 2.2 mmol/l in the first 24 hours of life and below 2.6 mmol/l from 24 to 48 hours after birth. It is mostly transient, but prolonged or recurrent episodes are associated with long-term neurological sequelae. We do not routinely check blood sugar levels in newborns who are healthy, delivered at term, after a normal pregnancy and birth and without signs of hypoglycaemia. In newborns with an increased risk of hypoglycaemia, we check their blood sugar after the first feeding, which should be within the first hour after birth. Additional investigations are performed if hypoglycaemia is persistent (lasting more than 48 hours), if the newborn with hypoglycaemia has no known risk factors for hypoglycaemia, in a newborn with epileptic seizures resulting from hypoglycaemia, and if the newborn needs an infusion of more than 12-15 mg glucose/kg/min to maintain normal blood sugar levels. The most common cause of persistent hypoglycaemia in new-borns is hyperinsulinism. Insulin inhibits lipolysis and ketone body formation. The brain is left without an alternative source of energy, which can lead to neurological damage. We describe a newborn girl with Beckwith-Wiedemann syn-drome who had persistent hypoglycaemia due to hyperinsulinism and was treated with octreotide.

Key words: hypoglycaemia, hyperinsulinaemia, neonate, Beckwith-Wiedemann syndrome