From hyperinsulinaemic hypoglycaemia to ketotic hypoglycaemia: the range of glucose abnormalities in patients born with intrauterine growth retardation

Kochar, Inderpal Singh; Hussain, Khalid

doi:10.1007/s00431-006-0371-1

Cited by 13 publications

(5 citation statements)

References 22 publications

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“…These patients present early in life with severe ketoacidosis that results in lethargy, vomiting, and coma, requiring aggressive intravenous hydration, bicarbonate, and glucose and insulin therapy (20,62,105,143,149,181,192,210). Some CoA transferase-deficient patients also present with hypoglycemia, and CoA transferase deficiency may contribute to a subset of idiopathic ketotic hypoglycemia cases (20,27,52,83,92,108). If maintained on carbohydrate-rich diets that are mildly reduced in protein content, CoA transferase-deficient patients seem to thrive, albeit with persistent hyperketonemia.…”

Section: Ketone Body Metabolismmentioning

confidence: 97%

Ketone body metabolism and cardiovascular disease

Cotter

Schugar

Crawford

2013

American Journal of Physiology-Heart and Circulatory Physiology

366

304

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Ketone bodies are metabolized through evolutionarily conserved pathways that support bioenergetic homeostasis, particularly in brain, heart, and skeletal muscle when carbohydrates are in short supply. The metabolism of ketone bodies interfaces with the tricarboxylic acid cycle, β-oxidation of fatty acids, de novo lipogenesis, sterol biosynthesis, glucose metabolism, the mitochondrial electron transport chain, hormonal signaling, intracellular signal transduction pathways, and the microbiome. Here we review the mechanisms through which ketone bodies are metabolized and how their signals are transmitted. We focus on the roles this metabolic pathway may play in cardiovascular disease states, the bioenergetic benefits of myocardial ketone body oxidation, and prospective interactions among ketone body metabolism, obesity, metabolic syndrome, and atherosclerosis. Ketone body metabolism is noninvasively quantifiable in humans and is responsive to nutritional interventions. Therefore, further investigation of this pathway in disease models and in humans may ultimately yield tailored diagnostic strategies and therapies for specific pathological states.

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Section: Ketone Body Metabolismmentioning

confidence: 97%

Ketone body metabolism and cardiovascular disease

Cotter

Schugar

Crawford

2013

American Journal of Physiology-Heart and Circulatory Physiology

366

304

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“…In accordance, it has been suggested that these children have no metabolic abnormalities but rather represent the end of the Gaussian curve for fasting tolerance in children due to accelerated starvation (7,8). In contrast, it has been reported that children born small for gestational age who develop transient neonatal hyperinsulinemic hypoglycemia can present later in childhood with ketotic hypoglycemia (9). This indicates a more complex metabolic or endocrine underlying cause, at least in some patients.…”

Section: Discussionmentioning

confidence: 90%

Insufficient Ketone Body Use Is the Cause of Ketotic Hypoglycemia in One of a Pair of Homozygotic Twins

Marcus

Alkén

Eriksson

et al. 2007

The Journal of Clinical Endocrinology &Amp; Metabolism

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“…Given that relatively short duration diazoxide therapy (3−4 months) was effective for most and no surgical intervention was required, those treated for hyperinsulinism likely met the criteria for an entity called 'prolonged neonatal hyperinsulism' (7,10). Moreover, many of our infants had risk factors for 'prolonged neonatal hyperinsulinism' which included early preterm birth, male sex, intrauterine growth restriction, or birth asphyxia (7,(11)(12)(13). Infants with this disorder were first described in 1984; however, it was Hoe et al in 2006, who created this diagnostic category (7).…”

Section: Discussionmentioning

confidence: 99%

Frequency and etiology of persistent neonatal hypoglycemia using the more stringent 2015 Pediatric Endocrine Society hypoglycemia guidelines

Skovrlj

Marks

Rodd

2018

Paediatrics &Amp; Child Health

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Objective: To determine if there was a significant increase in Endocrine consultations postinitiation of the more stringent 2015 guidelines for persistent neonatal hypoglycemia. Methods: A retrospective chart review was conducted using data from November 2011 to October 2016. All infants with persistent hypoglycemia past 72 hours of life were included. Data included age, critical sample values, anthropometric measures, and maternal health. Descriptive statistical analyses were performed as was an interrupted time series analysis assuming a Poisson distribution. Results: Fifty-eight infants were evaluated. Postintervention, there was a significant increase in the number of consults (P<0.03, 95% confidence interval [CI]: 1.14 to 8.93). Most infants with hypoglycemia persisting >72 hours were hypoglycemic shortly after birth. Half had intrauterine growth restriction; 75% were male. The median age for investigation was 8.3 days. Hyperinsulinism was the most common etiology (52/58 infants); diazoxide treatment was utilized in roughly half (29/52 to 56%) with a median duration of treatment for 91 days. The phenotype of the infants and duration of diazoxide pre-and post-Pediatric Endocrine Society protocol did not differ; two infants on diazoxide developed pulmonary hypertension. Mothers were typically of lower socioeconomic status. Conclusion: Not surprisingly, there was significant increase in the number of infants with persistent hypoglycemia using the 2015 guidelines. Prolonged hyperinsulinism was the major cause; medical management was typically sufficient and typically well tolerated. Care to reduce adverse effects of diazoxide is advised. We postulate that infants diagnosed using the more stringent 2015 guidelines have real disease based on the protracted medical management required.

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From hyperinsulinaemic hypoglycaemia to ketotic hypoglycaemia: the range of glucose abnormalities in patients born with intrauterine growth retardation

Abstract: Newborns with a history of IUGR and HH have an increased risk of developing ketotic hypoglycaemia in the childhood period.

Cited by 13 publications

References 22 publications

Ketone body metabolism and cardiovascular disease

Ketone body metabolism and cardiovascular disease

Insufficient Ketone Body Use Is the Cause of Ketotic Hypoglycemia in One of a Pair of Homozygotic Twins

Frequency and etiology of persistent neonatal hypoglycemia using the more stringent 2015 Pediatric Endocrine Society hypoglycemia guidelines

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