ISSN 1662-4009 (online)

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Yearbook of Paediatric Endocrinology 2022

ey0019.2-1 | Neonatal hypoglycaemia | ESPEYB19

2.1. Dextrose gel for neonates at risk with asymptomatic hypoglycemia: a randomized clinical trial

K Gupta , P Amboiram , U Balakrishnan , A C , T Abiramalatha , U Devi

Pediatrics. 2022 1;149(6):e2021050733. doi: 10.1542/peds.2021-050733. PMID: 35582897.Brief Summary: This randomized clinical trial in ‘at-risk’ neonates with asymptomatic hypoglycemia tested whether oral dextrose gel prevented the need for intravenous fluids. Oral dextrose gel reduced the need for intravenous fluids as well as admission to the neonatal intensive care unit (NICU).</...

ey0019.2-2 | Neonatal hypoglycaemia | ESPEYB19

2.2. Oral dextrose gel for the treatment of hypoglycaemia in newborn infants

T Edwards , G Liu , M Battin , DL Harris , JE Hegarty , PJ Weston , JE Harding

Cochrane Database Syst Rev. 2022 Mar 18;3(3):CD011027. doi: 10.1002/14651858.CD011027.pub3. PMID: 35302645.Brief Summary: This systematic review assessed the evidence for oral dextrose gel to treat hypoglycemia in newborns. Oral dextrose gel compared with placebo gel probably improved the correction of hypoglycaemic events and may result in a slight reduction in the risk of ma...

ey0019.2-3 | Neonatal hypoglycaemia | ESPEYB19

2.3. Continuous glucose monitoring in the management of neonates with persistent hypoglycemia and congenital hyperinsulinism

M Win , R Beckett , L Thomson , A Thankamony , K Beardsall

J Clin Endocrinol Metab. 2022 Jan 1;107(1):e246-e253. doi: 10.1210/clinem/dgab601. PMID: 34407200.Brief Summary: This retrospective single center study in neonates with hyperinsulinaemic hypoglycaemia examined the utility of real-time continuous glucose monitoring (CGM). CGM is best placed as an adjunct to routine intermittent blood glucose monitoring, providing information on glucose trends d...

ey0019.2-4 | Neonatal hypoglycaemia | ESPEYB19

2.4. Continuous glucose monitoring for the prevention of morbidity and mortality in preterm infants

A Galderisi , D Trevisanuto , C Russo , R Hall , M Bruschettini

Cochrane Database Syst Rev. 2021 Dec 21;12(12):CD013309. doi: 10.1002/14651858.CD013309.pub3. PMID: 34931697.Brief Summary: This systematic review assessed the evidence for continuous glucose monitoring (CGM) to prevent morbidity and mortality in preterm infants. There was uncertainty about the safety of CGM and the available management algorithms, and many morbidities remain unreporte...

ey0019.2-5 | Neonatal hypoglycaemia | ESPEYB19

2.5. PNC2 (SLC25A36) deficiency associated with the hyperinsulinism/hyperammonemia syndrome

MA Shahrour , FM Lasorsa , V Porcelli , I Dweikat , MA Di Noia , M Gur , G Agostino , A Shaag , T Rinaldi , G Gasparre , F Guerra , A Castegna , S Todisco , B Abu-Libdeh , O Elpeleg , L Palmieri

J Clin Endocrinol Metab. 2021 19;107(5):1346-1356. doi: 10.1210/clinem/dgab932. PMID: 34971397.Brief Summary: This is a case report of a potentially new genetic disorder that causes hyperinsulinaemic hypoglycemia, protein sensitivity and high serum ammonia level (Hyperinsulinism/hyperammonemia syndrome (HI/HA) syndrome). Mutations in the solute the carrier family 25, member 36 (SLC25A36) may b...

ey0019.2-6 | Neonatal hypoglycaemia | ESPEYB19

2.6. The bihormonal bionic pancreas improves glycemic control in individuals with hyperinsulinism and postpancreatectomy diabetes: a pilot study

A Rayannavar , LM Mitteer , CA Balliro , FH El-Khatib , KL Lord , CP Hawkes , LS Ballester , ER Damiano , SJ Russell , DD De Leon

Diabetes Care. 2021 Nov;44(11):2582-2585. doi: 10.2337/dc21-0416. PMID: 34518377.Brief Summary: This pilot study, in patients with post-pancreatectomy diabetes due to congenital hyperinsulinism, assessed pump-delivered insulin and glucagon (Bihormonal Bionic Pancreas, BHBP) to regulate blood glucose levels. BHBP controlled post-prandial glucose levels better than a conventional insulin pump but fur...

ey0019.2-7 | Neonatal hypoglycaemia | ESPEYB19

2.7. Stem cell based models in congenital hyperinsulinism - perspective on practicalities and possibilities

V Lithovius , T Otonkoski

Front Endocrinol (Lausanne). 2022 Feb 18;13:837450. doi: 10.3389/fendo.2022.837450. PMID: 35250887.Brief Summary: This paper discusses the use of Pluripotent Stem Cell (PSC)-derived pancreatic islets (SC-islets) for studying the basic biology, molecular mechanisms and therapeutic potentials of patients with congenital hyperinsulinism (CHI). The availability of CHI patient islets opens new a...

ey0019.2-8 | Neonatal hypoglycaemia | ESPEYB19

2.8. CRISPR/Cas9 ADCY7 knockout stimulates the insulin secretion pathway leading to excessive insulin secretion

Y Alhaidan , HT Christesen , E Lundberg , MAA Balwi , K Brusgaard

Front Endocrinol (Lausanne). 2021 Jun 11;12:657873. doi: 10.3389/fendo.2021.657873. PMID: 34177802.Brief Summary: This is a single case report describing a possible new candidate gene (ADCY7) for congenital hyperinsulinism. Mutations in the ADCY7 gene may be a new cause of congenital hyperinsulinism but more cases are needed and the molecular mechanisms studied in more det...