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Path o Physiology
Path o Physiology
Path o Physiology
The body normally defends against hypoglycemia by decreasing insulin secretion and
increasing glucagon, epinephrine, growth hormone, and cortisol secretion. These
hormonal changes combine to increase hepatic glucose output, to increase alternative fuel
availability, and to decrease glucose use (see image below). The increase in hepatic
glucose production is initially caused by the breakdown of liver glycogen stores due to
lower insulin levels and increased glucagon levels. When glycogen stores become
depleted and protein breakdown increases because of increased cortisol levels, hepatic
gluconeogenesis replaces glycogenolysis as the primary source of glucose production.
This breakdown of protein is reflected by increased plasma levels of the gluconeogenic
amino acids, alanine, and glutamine.
Physical
• Infants in the first or second day of life may be asymptomatic or have life-
threatening CNS and cardiopulmonary disturbances.
o Hypotonia
o Lethargy, apathy
o Poor feeding
o Jitteriness, seizures
o Congestive heart failure
o Cyanosis
o Apnea
o Hypothermia
• Clinical manifestations associated with activation of the autonomic nervous
system
o Anxiety, tremulousness
o Diaphoresis
o Tachycardia
o Pallor
o Hunger, nausea, and vomiting
• Clinical manifestations of hypoglycorrhachia or neuroglycopenia
o Headache
o Mental confusion, staring, behavioral changes, difficulty concentrating
o Visual disturbances (eg, decreased acuity, diplopia)
o Dysarthria
o Seizures
o Ataxia, somnolence, coma
o Stroke (hemiplegia, aphasia), paresthesias, dizziness, amnesia, decerebrate
or decorticate posturing
Causes
Differential Diagnoses
Glycogen-Storage Disease Type 0 Infant of Diabetic Mother
Glycogen-Storage Disease Type I Malnutrition
Glycogen-Storage Disease Type IVMaple Syrup Urine Disease
Glycogen-Storage Disease Type V Panhypopituitarism
Glycogen-Storage Disease Type VIPrematurity
Growth Hormone Deficiency Toxicity, Ethanol
Hyperinsulinemia Toxicity, Oral Hypoglycemic Agents
Hyperthyroidism Toxicity, Salicylate
Hypopituitarism
Addison disease
Adrenal crisis
Exogenous insulin administration
Medium chain acyl-CoA dehydrogenase deficiencies
Sepsis
Differential Diagnoses
Adrenal Insufficiency and Adrenal CrisisPlant Poisoning, Hypoglycemics
Hypopituitarism Shock, Septic
Hypothyroidism and Myxedema Coma Toxicity, Alcohols
Munchausen Syndrome Toxicity, Salicylate
Pediatrics, Reye Syndrome
Fasting
Malnutrition
Diarrhea
Enzymatic defects of glycogen synthetic pathways
Enzymatic defects of glycogenolytic pathways
Enzymatic defects of gluconeogenic pathways
Glucagon deficiency
Congenital hyperinsulinism (eg, nesidioblastosis, leucine sensitive hypoglycemia)
Defects of beta cell regulation
Large tumors
Decreased or absent fat stores
Enzymatic defects in fatty acid oxidation
Workup
Laboratory Studies
Imaging Studies
Treatment
Prehospital Care
Consultations
Medication
Case reports have shown that nifedipine may help maintain normoglycemia in children
with persistent hyperinsulinemic hypoglycemia of infancy (PHHI).
Cortisol should not be used because it has minimal acute benefit and may delay the
diagnosis of the cause of hypoglycemia. Cortisol stimulates gluconeogenesis and causes
decreased use of glucose, which leads to overall elevated blood glucose and may mask
the true cause of hypoglycemia.
Anti-hypoglycemic agents
Dextrose
Treatment of choice. Absorbed from the intestine resulting in rapid increase in blood
glucose concentration when administered PO. Give IV dextrose to infants of diabetic
mothers with transient neonatal hyperinsulinemia for several days until hyperinsulinemia
abates. Avoid hyperglycemia evoking prompt insulin release, which may produce
rebound hypoglycemia. SGA infants and those with maternal toxemia or perinatal
asphyxia require dextrose IV infusion rates >20 mg/kg/min to control levels. Treatment
may be necessary for 2-4 wk.
• Dosing
• Interactions
• Contraindications
• Precautions
Diazoxide (Hyperstat)
Increases blood glucose by inhibiting pancreatic insulin release, and possibly through an
extrapancreatic effect. Hyperglycemic effect starts within an hour and usually lasts a
maximum of 8 h with normal renal function. Reportedly effective in SGA infants and
those with maternal toxemia or perinatal asphyxia.
Octreotide (Sandostatin)
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1. Causes
1. Decreased Glucose stores
1. Prematurity
2. Intrauterine Growth Retardation (IUGR)
3. Hypoxia or asphyxia
4. Sepsis
5. Hypothermia
6. Congenital Heart Disease
7. Glycogen Storage Disease
8. Glucagon deficiency
9. Adrenal Insufficiency
10. Galactosemia
11. Fructose intollerance
2. Hyperinsulinism
1. Mother with Diabetes Mellitus
2. Erythroblastosis Fetalis
3. Exchange Transfusions
4. Beckwith-Wiedemann Syndrome
5. Nesidioblastosis
6. Islet Cell adenoma
7. Leucine sensitivity
3. Maternal Medications
1. Salicylates
2. Beta-Sympathomimetics
3. Chlorpropamide
4. Benzothiadiazide
2. Associated Conditions in children of diabetic mothers
1. Perinatal asphyxia
2. Birth Trauma (Shoulder dystochia)
3. Congenital anomalies
4. Hypocalcemia
5. Hyperbilirubinemia
6. Respiratory distress syndrome
7. Polycythemia
8. Feeding problems
9. Renal vein thrombosis
3. Symptoms
1. Jittery or Tremors
2. Lethargic
3. Hypotonia
4. Apnea
5. Hypothermia
6. Cyanosis
7. Seizures
8. Weak or high pitched cry
9. Poor feeding
4. Labs
1. Blood Glucose Monitoring
1. Hours of life: 1, 2, 3, 6, 12, 24, and 48 hours
2. Increase frequency of checks for symptoms
2. Serum Calcium
1. Check if lethargic or jittery despite normal glucose
3. Hematocrit
1. For signs of Polycythemia
4. Neonatal Bilirubin (as indicated)
5. Arterial Blood Gas
1. Indicated for signs of respiratory distress
5. Radiology
1. Chest XRay indicated for respiratory distress
6. Management: General Approach
1. Monitor Blood Sugar closely at above intervals
2. Glucose 35 to 45 mg/dl
1. Oral glucose replacement (see below)
2. Parenteral glucose replacement if symptomatic
3. Glucose 25-34 mg/dl
1. Attempt oral glucose replacement (see below)
2. Parenteral glucose replacement usually indicated
4. Glucose <25 mg/dl
1. Parenteral Glucose Replacement (initially with bolus)
2. Strongly consider NICU Admission
3. Glucagon if Intravenous Access delayed
1. Dose: 0.1 mg/kg/dose to 1 mg max IM or SQ q30 min
2. Not effective in SGA infants
7. Oral Glucose Replacement
1. Gavage or oral feedings hourly until glucose normal
2. Use 5% Dextrose in Water (D5W) or Infant Formula
8. Parenteral Glucose Replacement
1. Preparation of Glucose Solutions
1. D10W = 1:4 Dilution of D50W in sterile water
2. Do NOT use >12.5% glucose solutions in newborns
2. Intravenous Glucose Maintenance
1. Load: 2 ml/kg D10W at 2 ml/min
2. Maintenance: 80 ml/kg/day D10W
3. Emergent Glucose replacement
1. Dose: 0.5-1.0 g/kg (5-10 ml/kg D10W) IV over 20 min