Evaluating Persistent Hypoglycemia-Recommendations

http://www.jpeds.com/article/S0022-3476(15)00358-3/fulltext

For infants and younger children who are unable to reliably communicate symptoms, suggestted evaluation and management only of those whose Plasma Glucose concentrations are documented by laboratory quality assays to be below the normal threshold for neurogenic responses (<60 mg/dL [3.3 mmol/L]). GRADE 2+++0. Free fatty acids cannot be used by brain as fuel, whereas, Beta Hydroxy Buteric Acid (BOHB)and lactate can be used by the brain. When glucose level is <60mg/dl, measure lactate, FFA, BOHB, and HCO3 Insulin, cpeptide, GH (growth hormone)cortisol, acyl carnitine, and free carnitine may need to be measured.

HyperInhyperinsulinemic states--LOW BOHB, and FFA will be seen, and HCO3 will be normal.

In Fatty Acid Oxidation defects--- LOW BOHB, but INCREASED FFA, with normal HCO3 will be seen.

In Gluconeogenesis defects: LOW HOCO3, and INcreased Lactate is noted.

In GH, or Cortisol deficiency-Low HCO3, and Increased BOHB.

An exaggerated glycemic response (>30 mg/dL [>1.7 mmol/L]) is nearly pathognomonic of hyperinsulinism.


Because plasma insulin concentration is sometimes not above the lower limit of detection,

it is important to include the following tests when assessing the possibility of hypoglycemia due to hyperinsulinism: plasma BOHB and FFA (both inappropriately low; BOHB <1.5 mmol/L [<15 mg/dL] and FFA <1.0-1.5 mmol/L [<28-42 mg/dL]), and an increased glycemic response to glucagon. For neonates with a suspected congenital hypoglycemia disorder and older infants and children with a confirmed hypoglycemia disorder, recommend that the goal of treatment be to maintain a PG concentration >70 mg/dL.

For high-risk neonates without a suspected congenital hypoglycemia disorder, we suggest the goal of treatment be to maintain a PG concentration >50 mg/dL (>2.8 mmol/L) for those aged <48 hours and >60 mg/dL (>3.3 mmol/L) for those aged >48 hours.