低血糖の鑑別

Differential Diagnosis of Hypoglycemia*
HYPERINSULINEMIC HYPOGLYCEMIA (INCLUDING POSTPRANDIAL)
  1. Transient: infant of diabetic mother, perinatal asphyxia, Rhesus disease, intrauterine growth retardation, Beckwith-Wiedemann syndrome

  2. Congenital: ABCC8KCNJ11GCKGDHHADHHNF4ASLC16A1

  3. Dumping syndrome

  4. Insulin receptor mutations and antibodies

  5. Insulinoma

  6. Noninsulinoma pancreatogenous hypoglycemia (adults)

  7. Gastric bypass surgery for morbid obesity

  8. Non–islet cell tumor hypoglycemia or IGF-2-oma

  9. Insulin autoimmune syndrome

  10. Insulin factitious hypoglycemia

HORMONAL DEFICIENCY/RESISTANCE
  1. Adrenocorticotropic hormone

  2. Cortisol

  3. Growth hormone

  4. Glucagon

  5. Adrenaline

DEFECTS IN HEPATIC GLYCOGEN RELEASE/STORAGE
Glycogen storage diseases: glucose-6-phosphataseamylo-1,6-glucosidase deficiency, liver phosphorylase deficiency, glycogen storage disease type 0
DEFECTS IN GLUCONEOGENESIS
Fructose-1,6-bisphosphatase deficiency, phosphoenolpyruvate carboxykinase deficiency, pyruvate carboxylase deficiency
CARNITINE METABOLISM
  1. Carnitine deficiency (primary and secondary)

  2. Carnitine palmitoyltransferase deficiency (CPT 1 and 2)

  3. Carnitine transporter defects

FATTY ACID OXIDATION
  1. Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency

  2. Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency

  3. Short-chain acyl-CoA dehydrogenase (SCAD) deficiency

  4. Long/short-chain L-3-hydroxyacyl-CoA (L/SCHAD) deficiency

DEFECTS IN KETONE BODY SYNTHESIS/UTILIZATION
  1. HMG-CoA synthase deficiency, HMG-CoA lyase deficiency

  2. Succinyl-CoA: 3-oxoacid-CoA transferase (SCOT) deficiency

METABOLIC CONDITIONS (COMMON ONES)
  1. Organic acidemias (propionic, methylmalonic)

  2. Maple syrup urine disease, galactosemia, fructosemia, tyrosinemia

  3. Hereditary fructose intolerance

  4. Mitochondrial respiratory chain complex deficiencies

  5. Congenital disorders of glycosylation (CGD)

DRUG INDUCED
  1. Oral hypoglycemic agents

  2. Insulin

  3. β-Blockers

  4. Salicylates

  5. Alcohol

  6. Quinine

  7. Haloperidol

  8. Pentamidine

  9. Levofloxacin

  10. Methadone

  11. Disopyramide

  12. Indomethacin

  13. Cibenzoline

  14. Gatifloxacin

TOXIN-INDUCED
  1. Lychee fruit
MISCELLANEOUS CAUSES (MECHANISM[S] NOT CLEAR)
  1. Idiopathic ketotic hypoglycemia (diagnosis of exclusion)

  2. Infections (sepsis, malaria), congenital heart disease