Persistent Hypoglycemia, Newborn, N/IICU — Differential Diagnosis — Clinical Pathway: ICU

Newborn Persistent Hypoglycemia Clinical Pathway — N/IICU

Differential Diagnosis of Persistent Hypoglycemia

Common
  • IUGR/SGA
  • Stress-induced Hyperinsulinism
Less Common
  • Hypo-Ketotic Hypoglycemic Disorders
  • Hyperinsulinism: genetic forms, Beckwith-Wiedemann Syndrome, congenital disorders of glycosylation
  • Panhypopituitarism
  • Fatty acid oxidation disorders
    • Medium-chain acyl-CoA dehydrogenase deficiency (MCD/ACADM)
    • Primary carnitine deficiency
    • Carnitine-palmitoyl transferase 1 deficiency
    • Long chain 3 hydroxyacyl-CoA dehydrogenase deficiency
    • 3-Hydroxy-3-methyl-glutarul-CoA synthase deficiency
  • Gluconeogenesis Disorders
    • Glucose-6-phosphatase deficiency (Glycogen storage disease 1)
    • Fanconi-Bickel syndrome
    • Fructose 1,6 bisphosphatase deficiency
    • Pyruvate carboxylase deficiency
  • Ketotic Hypoglycemic Disorders
  • Normal in prolonged fast
  • Cortisol and/or Growth hormone deficiency
  • Defects in glycogen synthesis/degradation
    • Glycogen synthase deficiency (type 0)
    • Debrancher deficiency (type 3)
    • Phosphorylase deficiency (type 6)
    • Phosphorylase-kinase deficiency (type 9)
    • Phosphoglucomutase 1 deficiency
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Other conditions
  • Tyrosinemia