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Etiology: Classification - PowerPoint PPT Presentation


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Glucagon. Epinephrine. Insulin. Glycogen synthesis. Glucagon receptors. Parasympathetic nervous system activation. Glycogenolysis. Sympathetic nervous system activation. glycogen. glucose 1- phosphate. glycogen. Etiology: Classification. Inadequate production

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Presentation Transcript
slide1

Glucagon

Epinephrine

Insulin

Glycogen

synthesis

Glucagon

receptors

Parasympathetic

nervous system

activation

Glycogenolysis

Sympathetic

nervous system

activation

glycogen

glucose 1- phosphate

glycogen

slide2

Etiology: Classification

  • Inadequate production
  • Increased utilization
slide3

Hormonal Response to Hypoglycemia

  • Suppression of insulin secretion
  • Glucagon release
  • Epinephrine and cortisol levels increase
  • Growth hormone levels increase
  • Results in suppression of glycogenolysis, activates gluconeogenesis, promotes lipolysis, and stimulates ketogenesis
slide4

Etiology: inadequate production of glucose

  • Prematurity, IUGR, Perinatal stress
  • Glycogen storage disease
    • GSD type 1: glucose-6-phosphatase deficiency, catalyzes final common step in glycogenolysis and gluconeogenesis
    • GSD type 3: debrancher deficiency, inability to degrade stored glycogen. Autosomal recessive and manifestations include hepatomegaly, hypoglycemia, skeletal myopathy, cardiomyopathy
slide5

Congenital Hyperinsulinism

  • Sporadic and familial, incidence from 1/50,000-1/2500
  • In the presence of hypoglycemia:
    • inadequately suppressed insulin level and evidence of excessive insulin action for the degree of hypoglycemia:
      • inappropriately low plasma ketones and free fatty acids, inappropriately large glycemic response to glucagon.
slide6

Congenital Hyperinsulinism

  • To date, mutations in 4 different genes have been identified
    • Mutations in either of the two subunits of the ß-cell ATP-sensitive potassium channel (KATP)
    • Mutations in glucokinase (glucose sensor of the ß-cell)
    • Mutations in glutamate dehydrogenase
    • At least 50% of cases have no identified genetic cause
slide8

Congenital Hyperinsulinism

  • Recessive hyperinsulinism
    • Previous referred to as nesidioblastosis
    • Islet hyperplasia throughout pancreas
    • Severe hypoglycemia
    • LGA newborns
    • Genetic defect: SUR1 or kir6.2 components of the KATP channel. Thus, therapeutic interventions that act via the SUR such as diazoxide are ineffective
  • Most infants require near-total pancreatectomy
slide9

Congenital Hyperinsulinism

  • Focal Hyperinsulinism
    • Area of islet hyperplasia is localized to a small 3-5 mm diameter region described as focal adenomatous hyperplasia
    • Clinical presentation similar to diffuse disease (severe early hypoglycemia)
    • Results as a localized clonal loss of the maternal 11p15 region and expression of a paternally inherited SUR1 or Kir6.2 mutation. The 11p15 region contains several maternally imprinted tumor suppressor genes.
    • Can respond to partial pancreatectomy
slide10

Treatment of hyperglycemia

  • Decrease glucose infusion rate!
  • Only administer insulin if osmotic diuresis is present or glucose levels remain significantly elevated despite a reduction in glucose infusion rate.
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