Mechanisms of Disease: advances in diagnosis and treatment of hyperinsulinism in neonates.

Mechanisms of Disease: advances in diagnosis and treatment of hyperinsulinism in neonates. Research Abstract Details 

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Mechanisms of Disease: advances in diagnosis and treatment of hyperinsulinism in neonates. Abstract Text:

Diva D ,Charles A Stanley,

Hyperinsulinism is the single most common mechanism of hypoglycemia in neonates. Dysregulated insulin secretion is responsible for the transient and prolonged forms of neonatal hypoglycemia, and congenital genetic disorders of insulin regulation represent the most common of the permanent disorders of hypoglycemia. Mutations in at least five genes have been associated with congenital hyperinsulinism: they encode glucokinase, glutamate dehydrogenase, the mitochondrial enzyme short-chain 3-hydroxyacyl-CoA dehydrogenase, and the two components (sulfonylurea receptor 1 and potassium inward rectifying channel, subfamily J, member 11) of the ATP-sensitive potassium channels (K(ATP) channels). K(ATP) hyperinsulinism is the most common and severe form of congenital hyperinsulinism. Infants suffering from K(ATP) hyperinsulinism present shortly after birth with severe and persistent hypoglycemia, and the majority are unresponsive to medical therapy, thus requiring pancreatectomy. In up to 40-60% of the children with K(ATP) hyperinsulinism, the defect is limited to a focal lesion in the pancreas. In these children, local resection results in cure with avoidance of the complications inherent to a near-total pancreatectomy. Hyperinsulinism can also be part of other disorders such as Beckwith-Wiedemann syndrome and congenital disorders of glycosylation. The diagnosis and management of children with congenital hyperinsulinism requires a multidisciplinary approach to achieve the goal of therapy: prevention of permanent brain damage due to recurrent hypoglycemia.

Mechanisms of Disease: advances in diagnosis and treatment of hyperinsulinism in neonates. Publishing Authors By Initials

DD ,CA Stanley,

For similar diagnosis: prognosis: treatment outcome research abstracts see: diagnosis: prognosis: treatment outcome research

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Mechanisms of Disease: advances in diagnosis and treatment of hyperinsulinism in neonates. Journal Published:

PUBLICATION TYPE: Review

Journal: Nature clinical practice. Endocrinology & metaboli

VOLUME: 3

Page Numbers: 57-68

Journal Abbreviation:

ISSN: 1745-8366

DAY: 3

MONTH: Jan

YEAR: 2007

Mechanisms of Disease: advances in diagnosis and treatment of hyperinsulinism in neonates. Information

Number of References: 66

LANGUAGE: eng

NlmUniqueID: 101261798

Mechanisms of Disease: advances in diagnosis and treatment of hyperinsulinism in neonates. Keywords Mesh Terms:

KEYWORDS: Treatment Outcome

MESH TERMS: genetics

Chemical & Substance for Abstract: Mechanisms of Disease: advances in diagnosis and treatment of hyperinsulinism in neonates. Information

Substance Name: Glucokinase

Registry Number: EC 2.7.1.2

Grant and Affiliation Information for Mechanisms of Disease: advances in diagnosis and treatment of hyperinsulinism in neonates.

AFFILIATION: University of Pennsylvania, Philadelphia, PA, USA.

Country: England

AGENCY: United States NIDDK

GRANT: K12-DK-063682-02

ACRONYM: DK

MEDLINETA: Nat Clin Pract Endocrinol Meta

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