eMedicine Specialties > Pediatrics: General Medicine > Endocrinology
Hyperinsulinemia: Follow-up
Updated: Jul 9, 2008
Follow-up
Further Inpatient Care
- Admit patients for stabilization of blood glucose, further testing, and medical or surgical care.
- Blood glucose level should be determined before each oral feeding and when any symptom or sign of hypoglycemia is present. The most accurate blood glucose assessment is made by free blood drawn into a NaF-containing tube (gray top), with immediate processing to avoid spuriously low measurements resulting from glycolysis. A bedside glucometer can provide faster results, which need to be confirmed in the central laboratory only when the bedside value is below 60 mg/dL.
- All portable glucometers are inaccurate by as much as 20% when the measured blood glucose level is below 70 mg/dL. To reduce the possibility of neurologic injury, the blood glucose level should be maintained above 60 mg/dL at all times.
- Before discharging the patient from the hospital, perform a short fasting study (6-8 h) to ensure that the infant can safely tolerate a missed or inadequate feeding at home. The infant must be able to maintain a blood glucose level above 60 mg/dL throughout the fast.
- Ensure the training of caretakers and adequate home healthcare support for pump infusions (octreotide or glucagon) before discharging the patient from the hospital.
Further Outpatient Care
- Monitor medication dosages and side effects carefully, with frequent glucose level determinations.
- Monitor for symptoms and signs of hypoglycemia.
- Train caretakers to monitor blood glucose levels and to administer all medications at home.
Inpatient & Outpatient Medications
- Medications include diazoxide, octreotide, nifedipine, glucagon, growth hormone, and glucocorticoids. The choice of medications varies with etiology and severity of hypoglycemia.
Transfer
- Transfer of patient to a tertiary care facility is required to provide prompt diagnosis and medical treatment or surgical intervention.
- Referral to one of the aforementioned centers (see above) is preferred.
Deterrence/Prevention
- Avoid prolonged fasting. Seek medical attention if emesis or anorexia develops.
- Have source of glucose and glucagon emergency kit readily available if hypoglycemic symptoms appear.
Complications
- Seizures
- Permanent brain damage
- Death
Prognosis
- Multiple factors affect prognosis, such as the severity of the disease at presentation, duration of hypoglycemia, etiology of hyperinsulinism, and presence of neurologic complications.
- Improving diagnostic techniques make earlier and more appropriate surgical intervention (partial pancreatectomy or near-total pancreatectomy) possible.
- Patients who have had near-total pancreatectomy are at risk for developing exocrine pancreatic insufficiency and diabetes mellitus. Diabetes mellitus, which develops in patients with diffuse disease, is caused by dysregulation of insulin secretion in the residual beta cells after pancreatectomy.
Patient Education
- Counsel the patient, family members, and school personnel how to recognize the symptoms of hypoglycemia and how to administer glucose in the event of a hypoglycemic episode.
- Families should be equipped with glucagon and instructed in its use in case hypoglycemia does occur.
Miscellaneous
Medicolegal Pitfalls
- Failure to recognize and treat hypoglycemia
- Failure to recognize the cause of hypoglycemia
- Failure to counsel family how to recognize signs and symptoms of hypoglycemia and how or when to administer glucose or glucagon
- Failure to recognize associated conditions, such as cardiomyopathy in infants of diabetic mothers and associated problems with asphyxia
Special Concerns
- Some children with a known history of hypoglycemia may not be symptomatic. A high index of suspicion is essential for early detection and therapy.
More on Hyperinsulinemia |
| Overview: Hyperinsulinemia |
| Differential Diagnoses & Workup: Hyperinsulinemia |
| Treatment & Medication: Hyperinsulinemia |
Follow-up: Hyperinsulinemia |
| Multimedia: Hyperinsulinemia |
| References |
| « Previous Page | Next Page » |
References
Abdulhadi-Atwan M, Bushmann J, et al. Novel de novo mutation in sulfonylurea receptor 1 presenting as hyperinsulinism in infancy followed by overt diabetes in early adolescence. Diabetes. Jul 2008;57(7):1935-40. [Medline].
Arbizu Lostao J, Fernandez-Marmiesse A, Garrastachu Zumarran P, et al. [18F-fluoro-L-DOPA PET-CT imaging combined with genetic analysis for optimal classification and treatment in a child with severe congenital hyperinsulinism.]. An Pediatr (Barc). May 2008;68(5):481-5. [Medline].
Glaser B, Kesavan P, Heyman M, et al. Familial hyperinsulinism caused by an activating glucokinase mutation. N Engl J Med. 1998;338:226-30. [Medline].
Grimberg A, Ferry RJ Jr, Kelly A, et al. Dysregulation of insulin secretion in children with congenital hyperinsulinism due to sulfonylurea receptor mutations. Diabetes. 2001;50:322-8. [Medline].
Shah JH, Maguire DJ, Munce TB, Cotterill A. Alanine in HI: a silent mutation cries out!. Adv Exp Med Biol. 2008;614:145-50. [Medline].
Stanley CA, Baker L. The causes of neonatal hypoglycemia. N Engl J Med. Apr 15 1999;340(15):1200-1. [Medline].
Stanley CA, Lieu YK, Hsu BY, et al. Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene. N Engl J Med. 1998;338:1352-7. [Medline].
Suchi M, MacMullen CM, Thornton PS. Molecular and immunohistochemical analyses of the focal form of congenital hyperinsulinism. Mod Pathol. 2006;19:122-9. [Medline].
Thomas PM, Cote GJ, Wohllk N, et al. Mutations in the sulfonylurea receptor gene in familial persistent hyperinsulinemic hypoglycemia of infancy. Science. 1995;268:426-9. [Medline].
Hardy OT, Hernandez-Pampaloni M, Saffer JR, et al. Accuracy of [18F]fluorodopa positron emission tomography for diagnosing and localizing focal congenital hyperinsulinism. J Clin Endocrinol Metab. 2007;92:4706-11. [Medline].
Craver RD, Hill CB. Cure of hypoglycemic hyperinsulinism by enucleation of a focal islet cell adenomatous hyperplasia. J Pediatr Surg. 1997;32:1526-7. [Medline].
Cucchiaro G, Markowitz SD, Kaye R, et al. Blood glucose control during selective arterial stimulation and venous sampling for localization of focal hyperinsulinism lesions in anesthetized children. Anesth Analg. 2004;99:1044-8, table of contents. [Medline].
De Leon DD, Stanley CA. Mechanisms of Disease: advances in diagnosis and treatment of hyperinsulinism in neonates. Nat Clin Pract Endocrinol Metab. 2007;3:57-68. [Medline].
de Lonlay-Debeney P, Poggi-Travert F, Fournet JC. Clinical features of 52 neonates with hyperinsulinism. N Engl J Med. 1999;340:1169-75. [Medline].
Ferry RJ Jr, Franklin SL, Geffner ME. Hypoglycemia. In: Kappy MS, Allen DB, Geffner ME, eds. Principles and Practice of Pediatric Endocrinology. Springfield, Ill: Charles C Thomas Publisher, Ltd; 2005:607-34.
Ferry RJ Jr, Kelly A, Grimberg A, et al. Calcium-stimulated insulin secretion in diffuse and focal forms of congenital hyperinsulinism. J Pediatr. 2000;137:239-46. [Medline].
Hoe FM, Thornton PS, Wanner LA. Clinical features and insulin regulation in infants with a syndrome of prolonged neonatal hyperinsulinism. J Pediatr. Feb 2006;148(2):207-12. [Medline].
Hussain K, Aynsley-Green A, Stanley CA. Medications used in the treatment of hypoglycemia due to congenital hyperinsulinism of infancy (HI). Pediatr Endocrinol Rev. Nov 2004;2 Suppl 1:163-7. [Medline].
Kane C, Shepherd RM, Squires PE, et al. Loss of functional KATP channels in pancreatic beta-cells causes persistent hyperinsulinemic hypoglycemia of infancy. Nat Med. 1996;2:1344-7. [Medline].
Levitt Katz LE, Satin-Smith MS, Collett-Solberg P, et al. Insulin-like growth factor binding protein-1 levels in the diagnosis of hypoglycemia caused by hyperinsulinism. J Pediatr. Aug 1997;131(2):193-9. [Medline].
Lovvorn HN III, Nance ML, Ferry RJ Jr. Congenital hyperinsulinism and the surgeon: lessons learned over 35 years. J Pediatr Surg. 1999;34:786-92; discussion 792-3. [Medline].
Palladino AA, Bennett MJ, Stanley CA. Hyperinsulinism in infancy and childhood: when an insulin level is not always enough. Clin Chem. 2008;54:256-63. [Medline].
Stanley CA. Hyperinsulinism/hyperammonemia syndrome: insights into the regulatory role of glutamate dehydrogenase in ammonia metabolism. Mol Genet Metab. Apr 2004;81 Suppl 1:S45-51. [Medline].
Steinkrauss L, Lipman TH, Hendell CD. Effects of hypoglycemia on developmental outcome in children with congenital hyperinsulinism. J Pediatr Nurs. Apr 2005;20(2):109-18. [Medline].
Suchi M, Thornton PS, Adzick NS, et al. Congenital hyperinsulinism: intraoperative biopsy interpretation can direct the extent of pancreatectomy. Am J Surg Pathol. Oct 2004;28(10):1326-35. [Medline].
Further Reading
Keywords
hyperinsulinemia, beta-cell hyperplasia, hyperinsulinemic hypoglycemia, nesidioblastosis, neonatal hyperglycemia, primary hyperinsulinemia, hypoglycemia in children, hypoglycemia in infants, hyperinsulinism, growth hormone deficiency, hypocortisolemia, maternal diabetes, birth asphyxia, macrosomia, Beckwith-Wiedemann syndrome, omphalocele, macroglossia, visceromegaly, drug-induced hyperinsulinism, maternal toxemia, erythroblastosis fetalis, Munchausen syndrome by proxy, nesidioblastosis, islet adenomatosis, beta-cell adenoma, persistent hyperinsulinemic hypoglycemia of infancy, PHHI, leucine-sensitive hypoglycemia, islet cell dysmaturation syndrome
Follow-up: Hyperinsulinemia