Review article: Accelerated starvation of childhood: Have I judged ketones?

Abstract

Acute ketosis is an important physiological mechanism to prevent irreversible neurological damage from hypoglycaemia during starvation, and represents a significant metabolic stress. A cohort of children adapt to relatively short periods of reduced caloric intake by generating large quantities of ketone bodies. When excessive, the gastrointestinal symptoms of starvation ketosis such as nausea and pain may create a vicious cycle that delays spontaneous resolution. The presence of ketones can be dismissed as a normal feature of childhood metabolism, sometimes even when extreme. A broader understanding of this process under the banner of ‘accelerated starvation of childhood’ is helpful for clinicians managing acute illness in children. We advocate that children less than 7 years of age with a history suggestive of accelerated starvation of childhood should be screened by emergency clinicians for ketosis using a simple and cheap bedside capillary test, even if glucose levels are greater than 2.6 mmol/L. Identification and appropriate management of ketosis may alleviate the distressing gastrointestinal symptoms associated with many minor illnesses, and potentially prevent hypoglycaemia in some children. Appropriate advice to carers may be helpful to prevent further episodes. Illustrative case examples from our own practice are provided.

Severe neuroglycopenic symptoms due to nonketotic hypoglycemia in children with cardio‐facio‐cutaneous syndrome

“…Ketone bodies provide an alternate fuel source and potentially prevent neurological injury from brain hypoglycemia (Drachmann et al, 2021). The concentration of 3‐HB was most often defined as <0.6, 1.0–3.0, and >3.0 mmol/L for normal range, hyperketonemia, and ketoacidosis, respectively (Laffel, 1999; Millar & Harding, 2019; Drachmann et al, 2021).…”

Investigating paediatric hypoglycaemia: Dynamic studies at a tertiary paediatric hospital

“…The most common diagnosis made was accelerated starvation (17% of diagnostic studies). We use this term, rather than ‘ketotic hypoglycaemia’ as accelerated starvation may be seen in the absence of hypoglycaemia 13 . Accelerated starvation of childhood is an accelerated version of normal starvation ketosis that occurs in some children.…”

“…12 Ketosis was defined as an elevation of beta-hydroxybutyrate (BHB) >0.6 mmol/L. 13 Elevated FFA was defined as >0.6 mmol/L (local laboratory defined). Relative hypoketosis was defined as FFA:BHB ratio >2.5.…”

Review article: Ketoacidosis in the emergency department

“…The delay of ketone body production in the fasting state with increasing age may be secondary to decrease in energy demands as a function of body weight, and increase in muscle mass providing a reserve for gluconeogenesis. Accelerated starvation of childhood most frequently occurs in boys aged less than 6 years with low body mass . Accelerated starvation of childhood is associated with elevated ketones, glucagon, FFAs and cortisol and low levels of insulin.…”