Académique Documents
Professionnel Documents
Culture Documents
grates and modulates substrate mobilization, interconversion and utilization. Enzymes of glycogenolysis, gluconeogenesis and other metabolic fuels must be functional,
and there must be an adequate supply of endogenous fat,
glycogen and gluconeogenic substrates (amino acids,
glycerol, lactate) (4). Preterm, small for gestational age
and intrauterine growth-retarded infants, and infants with
hyperinsulinism (infants of diabetic mothers, BeckwithWiedemann syndrome), asphyxia, sepsis or other medical conditions, such as cardiopulmonary disease, are at
risk of developing hypoglycemia (2,5,6). Management of
neonates with hypoglycemia related to these conditions is
not the focus of this paper; there are several articles available that discuss these topics (2,5,6). However, when a
full term, apparently healthy neonate without the predisposing signs and symptoms of the above conditions develops hypoglycemia, the etiology may not be immediately
obvious. The more uncommon causes of hypoglycemia,
such as inborn errors of metabolism, should be consid-
Correspondence: Dr Sandra Marles, FE229 Community Services Building, 685 William Avenue, Winnipeg, Manitoba R3E 0S2. Telephone 204-787-2474,
fax 204-787-1419
16
docrine disorders may be confirmed and appropriate management can be instituted. Appropriate
consultation with an endocrinologist is indicated.
2. Consider further investigations, not necessarily
while hypoglycemic, such as carnitine, asparate
aminotransferase (AST), alanine aminotransferase (ALT), uric acid, capillary gas, lactic acid,
plasma amino acids, creatine kinase (CK), ammonia, acylcarnitine profile and DNA for MCAD
mutation. The last two investigations can be
done from the neonatal screening bloodspots.
Rationale: Elevated AST, ALT, lactic acid and uric
acid with ketotic hypoglycemia and possibly acidosis suggests glycogen storage diseases or fructose
1,6-bisphosphatase deficiency. The acidosis is usually more severe in the latter. Galactosemia may be
associated with abnormal AST, ALT, metabolic acidosis and amino aciduria. Fatty acid oxidation defects usually show hypoketosis, urine organic acid
abnormalities, and carnitine and acylcarnitine abnormalities. Lactic acidosis is present, and ammonia, AST and ALT may also be elevated (3,7,11,13).
Treatment of the hypoglycemic infant may begin while
investigations continue. If the neonate is asymptomatic
and the hypoglycemia is mild (serum glucose 1.7 to 2.2
mmol/L), oral feeding of glucose, 0.5 to 1.0 g/kg, may be
appropriate. If the neonate is symptomatic or the serum
glucose is less than 1.7 mmol/L, an intravenous bolus of
10% dextrose in water (2 mL/kg) given over 5 mins may
be indicated. This should be followed by an infusion of 10%
dextrose in water at 5 to 10 mg/kg/min, or 90 mL/kg/24 h to
maintain serum glucose over 3.3 mmol/L (3).
The highest glucose concentration that can be administered through a peripheral vein is 12.5% dextrose in water. Central venous access is required for more concentrated solutions. When venous access is difficult or when
glucagon is indicated, 0.3 mg/kg/dose to a total dose of
1.0 mg/kg/day may be used (16). Steroid therapy is somewhat controversial in the treatment of persistent neonatal
hypoglycemia of unknown etiology. Steriods are not effective in hyperinsulinism or other disorders not due to adrenal insufficiency. Diazoxide may be used in cases of
hyperinsulinism (3).
REFERENCES
19