Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2009 Feb;5(2):101-12.
doi: 10.1038/ncpendmet1046.

Advances in the diagnosis and management of hyperinsulinemic hypoglycemia

Ritika R Kapoor  1 , Chela James, Khalid Hussain
Affiliations
Review

Advances in the diagnosis and management of hyperinsulinemic hypoglycemia

Ritika R Kapoor et al. Nat Clin Pract Endocrinol Metab. 2009 Feb.

Erratum in

  • Nat Clin Pract Endocrinol Metab. 2009 Mar;5(3):180

Abstract

Hyperinsulinemic hypoglycemia (HH) is a consequence of unregulated insulin secretion by pancreatic beta-cells and is a major cause of hypoglycemic brain injury and mental retardation. Congenital HH is caused by mutations in genes involved in regulation of insulin secretion, seven of which have been identified (ABCC8, KCNJ11, GLUD1, CGK, HADH, SLC16A1 and HNF4A). Severe forms of congenital HH are caused by mutations in ABCC8 and KCNJ11, which encode the two components of the pancreatic beta-cell ATP-sensitive potassium channel. Mutations in HNF4A, GLUD1, CGK, and HADH lead to transient or persistent HH, whereas mutations in SLC16A1 cause exercise-induced HH. Rapid genetic analysis combined with an understanding of the histological features (focal or diffuse disease) of congenital HH and the introduction of (18)F-L-3,4-dihydroxyphenylalanine PET-CT to guide laparoscopic surgery have totally transformed the clinical approach to this complex disease. Adult-onset HH is mostly caused by an insulinoma; however, it has also been reported to present as postprandial HH in patients with noninsulinoma pancreatogenous hypoglycemia syndrome, in those who have undergone gastric-bypass surgery for morbid obesity, and in those with mutations in the insulin-receptor gene.

PubMed Disclaimer

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Horm Res. 2008;70(2):65-72 - PubMed
    1. PLoS Med. 2007 Apr;4(4):e118 - PubMed
    1. Pediatr Surg Int. 2001 Jul;17(5-6):351-5 - PubMed
    1. Surgery. 2000 Dec;128(6):937-44;discussion 944-5 - PubMed
    1. Virchows Arch A Pathol Anat Histol. 1975;366(3):223-36 - PubMed

MeSH terms

LinkOut - more resources