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Tad Miya
Case
• 18-month-old male with “recurrent hypoglycemia” and a 4-day
history of nausea, vomiting, diarrhea, fatigue and decreased
oral intake. His family has been attempting to hydrate him with
juice and Pedialyte. He has been given corn syrup to prevent
further “lows”. His urine output decreased. He became
lethargic, non-responsive followed by a 5-10 second
generalized tonic-clonic seizure and remained unresponsive
after. A neighbor with medical training couldn’t feel a pulse,
started CPR x 10sec and had one after.
• Blood Glucose:
• Home: 79mg/dL
• EMS: 50mg/dL
• OSH: 33mg/dL
Course to Floor:
• OSH: “Several D10 Boluses”
• Transfer: D5-containing fluids
• PCH ED:
• BG: 30-45mg/dL; D10-bolus x 3
• D10-1/2NS+20KCl @ 1.0 mIVF
Triglycerides: 41
Uric Acid: 5.6 (1.8-4.9)
Triglycerides: 41
Uric Acid: 5.6 (1.8-4.9)
Triglycerides: 41
Uric Acid: 5.6 (1.8-4.9)
Hyperinsulinemia:
• Dec’d FFA’s (Lipogenesis)
Re-test to confirm:
D10 D7.5…......D5.......D2.5.....
Re-test to confirm: Insulin: 6.2 (4-45)
FFAs: 0.26 (<1.0)
Lactate: 1
B-Hydroxy B: 0.1
Coritsol: 1 (5-10)
Growth Hormone: 0.71
BG: 47mg/dL
Re-test to confirm:
N= 90 Hyperinsulinemia w/ Hypoglycemia
~1/3 w/ Undetectable insulin levels
- No Statistical correlation
Al-Otaibi, et al 2013
Re-test to confirm:
Ferrara, et al 2016
Re-test to confirm: Insulin: 6.2 (4-45)
FFAs: 0.26 (<1.0)
Lactate: 1
B-Hydroxy B: 0.1
Coritsol: 1 (5-10)
Growth Hormone: 0.71
Hypoglycemia-associated autonomic
failure (HAAF)
• Heterogeneous Disorder
• Birth-Childhood
• Degree of hypoglycemia/hyperinsulinemia
• Presenting symptoms
• Response to therapy
Hyperinsulinemia
• Epi: 1:30,000 births
• Pathophys:
• Many genetic mutations of the insulin regulation in the B-cells.
• Autosomal Recessive>>Dominant mutations
Hyperinsulinemia
• Inhibits:
• Glycogenolysis, Lipolysis(FFO), Ketone production, Proteinolysis
Hyperinsulinemia
• Clinical Features:
• Infant:
• Poor feeding, Jitteriness, Lethargy
• LGA (1/3)
• Hypoglycemia despite oral feeds.
• Recurrent Hypoglycemia
• Neurologic Symptoms: Seizure, Coma
Hyperinsulinemia: Diagnosis
• Hypoglycemia, Non-ketotic, Non-acidotic.
• Short durations of euglycemia post feeds (< 1-2hr)
• Inappropriately elevated Insulin levels
• Normal levels support, undetectable cannot exclude
• Glucagon-Responsive (Excessive)
Hyperinsulinemia: Diagnosis
Glucagon: 30mcg/kg IM/IV
10,20,30-min post-
administration
Suggestive of insulin-driven
glycogenolysis/storage in
hepatocytes
BG: 47mg/dL
Hyperinsulinemia: Management
• Euglycemic (Neonate>50mg/dL, Child > 60-80mg/dL)
• GIR: 10-40mg/kg*min. May need >D10 (Central Line)
• Frequent BG monitoring
RESCUE:
• Glucagon Kits
Hyperinsulinemia: Management
• Alternatives:
• Octreotide
• Sirolimus
• Ca-Channel Blockers
• Surgical:
• Focal pancreatic hypertrophy
• Uniparental disomies
• Failed pharmacologic therapies
Hyperinsulinemia: Morbidity
• Neurological Sequelae
• Children/Neonates: Higher metabolic rates
• Head to body ratio
• Seizures
• Developmental
• Psychomotor Delay/Retardation (21-44%)
• Motor/Speech
• Cognitive Deficits (memory, arithmetic)
• Epilepsy (18-25%)
Summary:
• Hypoglycemia: Intake/Production/Storage/Utilization
• Clinical Examination>Laboratory Data
• Hyperinsulinemia:
• Variable Presentation/Pathology
• Non-Ketotic, Non-acidotic, Unremitting Hypoglycemia.
• Insulin Levels: “Abnormally Normal”
• Undetectable levels do NOT exclude diagnosis
• Glucagon Challenge. (+) > 30mg/dL rise.
• Treatment: Acute:
• Dextrose-containing fluids (GIR 10-40), Glucagon, OraGel
• Treatment: Maintenance:
• Diazoxide + Diuril.
• +/- Surgery
Summary:
References:
• Uptodate.com
• Ferrara, Christine, Payal Patel, Susan Becker, Charles A. Stanley, and Andrea Kelly.
“Biomarkers of Insulin for the Diagnosis of Hyperinsulinemic Hypoglycemia in Infants and
Children.” The Journal of Pediatrics 168 (January 2016): 212–19.
https://doi.org/10.1016/j.jpeds.2015.09.045.
• Lonlay, Pascale de, Jean-Christophe Fournet, Guy Touati, Marie-Sylvie Groos, Delphine
Martin, Caroline Sevin, Véronique Delagne, et al. “Heterogeneity of Persistent
Hyperinsulinaemic Hypoglycaemia. A Series of 175 Cases.” European Journal of Pediatrics
161, no. 1 (October 2002): 37–48. https://doi.org/10.1007/s004310100847.
• Thornton, Paul S., Charles A. Stanley, Diva D. De Leon, Deborah Harris, Morey W.
Haymond, Khalid Hussain, Lynne L. Levitsky, et al. “Recommendations from the Pediatric
Endocrine Society for Evaluation and Management of Persistent Hypoglycemia in Neonates,
Infants, and Children.” The Journal of Pediatrics 167, no. 2 (August 2015): 238–45.
https://doi.org/10.1016/j.jpeds.2015.03.057.