Definition (Pediatrics 1998;192:E69; Vandemecum Metabolicum 2004:6)
• Glucose <2.6 mmol/L (45 mg/dL) at all ages
History and Clinical Manifestations
• Determine time since last meal, drugs
• Check for hepatomegaly, liver failure signs (palmar erythema, spider angiomata, gynecomastia, jaundice), small genitals, hyperpigmentation, short stature
Ddx
• Disorders of protein intolerance, carbohydrate metabolism, or fatty acid oxidation
• Hepatic glycogen storage dz (except Pompe); no glycogenolysis (worse with fasting)
• In neonate: Need to rule out sepsis, SGA, maternal diabetes; maybe slow adaptation
• Persistent neonatal hypoglycemia → hyperinsulinemia or hypopituitarism
Labs While Hypoglycemic
• As above + insulin, cortisol, lactate, free fatty acids, 3-hydroxybutyrate, Ketostix (urine)
• Acylcarnitine (dried blood spots or plasma); for fatty acid ox d/o + organic acidurias, C-peptide level
• Spare tube for additional labs
• Organic acids in the urine
Treatment
• IV glucose at 7–10 mg/kg/min, for calories and to replace normal liver glucose production → D10% glucose, 110–150 cc/kg/d with electrolytes
• Keep FS >100; if glucose needs >10 mg/kg/min → likely hyperinsulinism