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Nesidioblastosis

Reviewed 06/2019
 


BASICS

DESCRIPTION

  • Nesidioblastosis (NB) is a disease characterized by hyperfunctioning pancreatic β cells, usually associated with hypoglycemia in the presence of high endogenous insulin levels. It is...

DIAGNOSIS

HISTORY

  • Infants/children: irritability, lethargy, convulsions, coma, hunger, mental retardation

  • Adults: lethargy, confusion, sweating, chills, palpitations, dizziness, loss of consciousness, s...

TREATMENT

GENERAL MEASURES

Frequent consistent meals throughout the day to maintain normal glucose levels 

MEDICATION

First Line

  • Diazoxide: blocks receptors on pancreatic β cells, keeping the K channel op...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Endocrinology

  • Nutrition

  • Surgery: if patient failed conservative therapy

  • Genetics

Patient Monitoring

  • For infants and children, watch for signs of neurohypoglycemia beca...

REFERENCES

1
Bernard B, Kline GA, Service FJ. Hypoglycaemia following upper gastrointestinal surgery: case report and review of the literature. BMC Gastroenterol.  2010;10:77. [View Abstract on Ov...

ADDITIONAL READING

Palladino AA, Stanley CA. Nesidioblastosis no longer! It’s all about genetics. J Clin Endocrinol Metab.  2011;96(3):617–619. [View Abstract on OvidMedline] 

CODES

ICD10

E16.9 Disorder of pancreatic internal secretion, unspecified 

ICD9

251.9 Unspecified disorder of pancreatic internal secretion 

SNOMED

  • 42681006 Islet cell hyperplasia (disorder)

  • 66149005 Hyperpl...

CLINICAL PEARLS

  • NB is a genetically linked disorder, typically seen in infancy. However, there has been an association with gastric bypass surgery.

  • Insulinoma symptoms generally occur in the fasting st...

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