Neurodevelopmental Outcomes

Lord, Katherine; Leon-Crutchlow, Diva De

doi:10.1007/978-3-030-02961-6_14

Cited by 2 publications

(4 citation statements)

References 20 publications

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“…High GIR is an indicator of hyperinsulinism 13 and thus can be used as a predictor for potential need for medications such as glucagon or diazoxide. 12 13…”

Section: Discussionmentioning

confidence: 99%

“…23 24 25 26 Higher target levels of glucose concentration are currently used for patients with hyperinsulinemia because of lack of alternative fuels. 12 13 There is considerable heterogeneity across studies about which level of hypoglycemia is associated with long-term consequences; controversy may not be resolved until school-age outcomes are available for the randomized trial by van Kempen et al 27 Neonates in this cohort would currently be eligible to either a validated bundle of care 25 or glucose gel, 28 which have been shown to reduce the risk of hypoglycemia in at-risk neonates. 29 Variables used in this model should be tested again in the current setting using glucose gel, which may reduce the need for IV glucose and admission to the NICU and increase the percentage of exclusive breastfeeding at discharge.…”

Section: Discussionmentioning

confidence: 99%

“…Hyperinsulinemia is the most common cause of neonatal hypoglycemia. 12 Neonates with stress-induced hyperinsulinism (e.g., due to sepsis, perinatal asphyxia, IUGR) typically require a high GIR; the mean and median maximum GIR reported were 11.7 and 10.0 mg/kg / min, respectively, in a recent study. 13…”

mentioning

confidence: 97%

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Factors Associated with Need for Intravenous Glucose Infusion for the Treatment of Early Neonatal Hypoglycemia in Late Preterm and Term Neonates

et al. 2023

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Objective: To determine which late-preterm (35-36wk gestational age [GA]) and term neonates with early-onset hypoglycemia in the first 72 hours postnatal required a continuous glucose infusion in order to achieve and successfully maintain euglycemia. Study Design: Retrospective cohort study of late preterm and term neonates born in 2010-2014 and admitted to the Mother-Baby Unit at Parkland Hospital who had laboratory-proven blood glucose concentration <40 mg/dL (2.2 mmol/L) during the first 72h of life. Among the subgroup needing iv glucose infusion, we analyzed which factors predicted a maximum glucose infusion rate (GIR) ≥10 mg · kg-1 · min-1. The entire cohort was randomly divided into a derivation cohort (n=1288) and a validation cohort (n=1298). Results: In multivariate analysis, the need for intravenous glucose infusion was associated with small size for GA, low initial glucose concentration, early-onset infection and other perinatal variables in both cohorts. A GIR ≥10 mg · kg-1 · min-1 was required in 14% neonates with blood glucose value <20 mg/dL during the first 3h of observation. The likelihood of a GIR ≥10 mg · kg-1 · min-1 was associated with lower initial blood glucose value and lower umbilical arterial pH. Conclusion: Need for intravenous glucose infusion was associated with small size for GA, low initial glucose concentration, early-onset infection and variables associated with perinatal hypoxia-asphyxia. The likelihood of a maximum GIR ≥10 mg · kg-1 · min-1 was greater in neonates with lower blood glucose value during the first 3 hours of observation and lower umbilical arterial pH.

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“…High GIR is an indicator of hyperinsulinism 13 and thus can be used as a predictor for potential need for medications such as glucagon or diazoxide. 12 13…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Factors Associated with Need for Intravenous Glucose Infusion for the Treatment of Early Neonatal Hypoglycemia in Late Preterm and Term Neonates

et al. 2023

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“…The exact and sometimes overlapping molecular mechanisms leading to hypoglycemia in these syndromes have not fully been elucidated, although hyperinsulinism, augmented energy consumption, and dysregulation of growth hormone and cortisol have been reported [ 5 , 6 ]. Early intervention is essential to minimize the risk of poor neurologic outcomes and developmental delay [ 7 , 8 ]. Moreover, since peculiar dysmorphic signs might be mild at birth and in early childhood, both neonatologists and pediatricians should train to look for and recognize them [ 9 , 10 ].…”

Section: Introductionmentioning

confidence: 99%

Neonatal hyperinsulinemic hypoglycemia: case report of kabuki syndrome due to a novel KMT2D splicing-site mutation

et al. 2020

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Background Persistent neonatal hypoglycemia, owing to the possibility of severe neurodevelopmental consequences, is a leading cause of neonatal care admission. Hyperinsulinemic hypoglycemia is often resistant to dextrose infusion and needs rapid diagnosis and treatment. Several congenital conditions, from single gene defects to genetic syndromes should be considered in the diagnostic approach. Kabuki syndrome type 1 (MIM# 147920) and Kabuki syndrome type 2 (MIM# 300867), can be associated with neonatal hyperinsulinemic hypoglycemia. Patient presentation We report a female Italian (Sicilian) child, born preterm at 35 weeks gestation, with persistent hypoglycemia. Peculiar facial dysmorphisms, neonatal hypotonia, and cerebellar vermis hypoplasia raised suspicion of Kabuki syndrome. Hyperinsulinemic hypoglycemia was confirmed with glucagon test and whole-exome sequencing (WES) found a novel heterozygous splicing-site mutation (c.674-1G > A) in KMT2D gene. Hyperinsulinemic hypoglycemia was successfully treated with diazoxide. At 3 months corrected age for prematurity, a mild global neurodevelopmental delay, postnatal weight and occipitofrontal circumference growth failure were reported. Conclusions Kabuki syndrome should be considered when facing neonatal persistent hypoglycemia. Diazoxide may help to improve hyperinsulinemic hypoglycemia. A multidisciplinary and individualized follow-up should be carried out for early diagnosis and treatment of severe pathological associated conditions.

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Neurodevelopmental Outcomes

Cited by 2 publications

References 20 publications

Factors Associated with Need for Intravenous Glucose Infusion for the Treatment of Early Neonatal Hypoglycemia in Late Preterm and Term Neonates

Factors Associated with Need for Intravenous Glucose Infusion for the Treatment of Early Neonatal Hypoglycemia in Late Preterm and Term Neonates

Neonatal hyperinsulinemic hypoglycemia: case report of kabuki syndrome due to a novel KMT2D splicing-site mutation

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