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Nesidioblastosis

Niyomi De Silva, MD, William D Bass, DO, MS and Afsha Rais Kaisani, FAAFP, MD Reviewed 04/2024
 


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

Due to a lack of a pathophysiological understanding of the condition, conservative treatment options are limited and mostly ineffective (1). 

GENERAL MEASURES

Frequent, consistent low-carbohy...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Endocrinology

  • Nutrition

  • Surgery (if patient failed conservative therapy)

  • Genetic counseling

Patient Monitoring

  • For infants and children, watch for neurologic signs of ...

REFERENCES

1
Dieterle MP, Husari A, Prozmann SN, et al. Diffuse, Adult-Onset Nesidioblastosis/Non-Insulinoma Pancreatogenous Hypoglycemia Syndrome (NIPHS): Review of the Literature of a Rare Cause of H...

ADDITIONAL READING

Palladino  AA, Stanley  CA. Nesidioblastosis no longer! It's all about genetics. J Clin Endocrinol Metab.  2011; 96(3): 617– 619. 

CODES

ICD10

E16.9 Disorder of pancreatic internal secretion, unspecified 

SNOMED

  • 42681006 Islet cell hyperplasia (disorder)

  • 66149005 Hyperplasia of pancreatic islet beta cell (disorder)

CLINICAL PEARLS

  • NB is a genetically linked disorder, typically seen in infancy. However, an emerging association has been discovered in adults.

  • Insulinoma symptoms generally occur in the fasting state.

  • ...

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