PEDBASE.org - The Pediatric Database - Detailed information of HYPOGLYCEMIA

HYPOGLYCEMIA

DEFINITION:

A blood glucose concentration less than 2.2 mmol/L at any age.

EPIDEMIOLOGY:

incidence: 4.4/1000 live births
2-3/1000 older children
age of onset:
- any
risk factors:
- low birth weight infants (15.6/1000)
- infants of diabetic mothers

DIFFERENTIAL DIAGNOSIS:

1. Metabolic Disorders

1. Carbohydrate Metabolism

1. Glycogen Storage Disease

Ia - von Gierke Disease
III - Forbes Disease
VI - Hers Disease
IX

2. Gluconeogenesis

Galactosemia I + III
Hereditary Fructose Intolerance
Fructose-1,6-Diphosphatase Deficiency
Pyruvate Carboxylase Deficiency
Phosphoenolpyruvate Carboxykinase (PEPCK) Deficiency

2. Lipid Metabolism

MCAD, LCAD, SCAD
Carnitine Deficiency (Primary & Secondary)
Carnitine Palmitoyl Transferase Deficiency

3. Amino Acid Metabolism

Maple Syrup Urine Disease (MSUD)
Tyrosinemia-I

4. Organic Acidemias

Ethylmalonic-Adipic Aciduria
Glutaric Acidemia Type II
Methylmalonic Acidemia
Propionic Acidemia
3-OH,3-Methylglutaric Acidemia

2. Endocrine Disorders

Hypopituitarism
Adrenal Insufficiency
Growth Hormone Deficiency
Hyperthryroidism (Ketotic Hypoglycemia)
Hyperinsulinemia
- Adenomas, Insulinomas
- Infant of a Diabetic Mother (IDM)
- Nesidioblastosis

3. Miscellaneous

Beckwith-Wiedemann Syndrome
Jamaican Vomiting Sickness
Ketotic Hypoglycemia (Accelerated Starvation)
Munchausen-By-Proxy
Overdose
- alcohol
- oral hypoglycemic agents
- salicylate
Stress
- asphyxia
- heart disease
- sepsis
- severe respiratory distress

CLINICAL FEATURES:

1. Neurological Manifestations

high pitched cry
infantile hypotonia
irritability
jitteriness
lethargy -> coma
seizures
tremors

2. Others

apnea & cyanosis
feeding difficulties
pallor
tachypnea

3. Complications

mental retardation with repetitive episodes of intractable hypoglycemia

INVESTIGATIONS:

1. First Line Investigations

1. Serum

glucose, electrolytes, gas
uric acid
amino acids
bilirubin, transaminases

2. Urine

urinalysis - glucose, ketones
reducing substances (Clinitest, Clinistix)
drug screen (alcohol, oral hypoglycemic agents, salicylates)
organic acids and amino acids
2,4-dinitrophenylhydrazine (2,4 DNPH) reagent

2. Second Line Investigations

1. Serum (when hypoglycemic)

plasma insulin, growth hormone, cortisol, glucagon, catecholamines
beta-hydroxybutyrate, acetoacetate, lactate, pyruvate

2. Biopsy

liver

MANAGEMENT:

1. Supportive

1. Airway

2. Breathing

3. Circulation

4. Dextrose

start IV of D10-25W and run to give 4-6 mg glucose/kg/min and increase to keep glucose within the normal range (above 3 mmol/L)
if using >10-15 mg glucose/kg/min (consider hyperinsulinemic states) and add:

5. Prednisone

start at 2 mg/kg/day po bid
impairs responsiveness to insulin

6. Diazoxide (Proglycem)

start at 5-10 mg/kg/day po tid and may increase to 20-25 mg/ kg/day
suppresses insulin secretion

7. Glucagon Infusion

2. Surgery

indicated for medical failure to control hyperinsulinemia (hypoglycemia)
total or subtotal (80-99%) pancreatectomy

3. Correct Underlying Disorder

1. Hyperinsulinemia

Prednisone +/- Diazoxide
Pancreatectomy

2. Hypopituitarism

Growth Hormone +/- Prednisone

3. Glycogen Storage Diseases

raw uncooked cornstarch po q4-5h as a slurry to provide 6-10 mg/kg/min of carbohydrate (GSD-Ia); allows prolonged, steady absorption of carbohydrate

Pediatric Database - HYPOGLYCEMIA

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