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Morning Report

Morning Report. November 2, 2010. Glycemic Control. Normal Endocrine system Responds and regulates Functional enzymes Produce glucose Adequate supply of substrate. Sources of Glucose. Meals Muscle or Hepatic Glycogen Gluconeogenesis. Hypoglycemia. <40 or 50 mg/ dL

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Morning Report

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  1. Morning Report November 2, 2010

  2. Glycemic Control • Normal Endocrine system • Responds and regulates • Functional enzymes • Produce glucose • Adequate supply of substrate

  3. Sources of Glucose • Meals • Muscle or Hepatic Glycogen • Gluconeogenesis

  4. Hypoglycemia • <40 or 50 mg/dL • Symptoms in Infancy • Irritable • Jittery • Feeding problems • Lethargy • Cyanosis • Tachypnea • Hypothermia

  5. Hypoglycemia Autonomic neuro • Sweaty • Weakness • Tachycardia • Tremor • Nervous • Hunger • “warning signs” • BG 40-70 • Lethargy • Irritable • Confusion • Strange behavior • Hypothermia • Seizure • Coma • BG – 10-50

  6. Critical Sample • 5-10mL of blood • Substrates • Glucose, FFAs, β-hydroxybutyrate, lactate, total and free carnitine and acylcarnitine • Hormones • Insulin, C-peptide, cortisol, and growth hormone • Other • Lytes, LFTs, ammonia, tox, metabolic • Urine • Ketones, reducing substances

  7. Treatment • PO glucose • IM • Glucagon 0.3mg/kg (max 1mg) • IV • D10 - 2cc/kg • GIR 6-9 • Frequent Accu checks • 70-120 • Avoid fasting

  8. Diagnosis • Depends on history and symptoms • Glucagon challenge • Hyperinsulinemia • Elective fast • Measure critical sample

  9. Etiologies

  10. Impaired Carb Metabolism • Disorders of glycogenolysis • Glycogen storage diseases • Disorder of gluconeogenesis • Glucose-6-phosphatase def, fructose 1,6 diphosphatase def, pyruvatecarboxylase def, PEPCK def • Galactosemia • Hereditary Fructose Intolerance

  11. Disorders of AA Metabolism • Organic Acidemias • Maple syrup urine disease, propionicacidemia, methylmalonicacidemia, glutaricaciduria, and tyrosinemia • Fatty Acid Oxidation • Carnitine deficiency, fatty acid transportation defects, and defects of beta-oxidation enzymes Disorders of FA Metabolism

  12. Increased Utilization of Glucose • Hyperinsulinism • Insulinoma, exogenous • Oral hypoglycemics • Diabetes mellitus • Treatment

  13. Miscellaneous • Ketotic hypoglycemia • 18m-5y • Resolves by 8-9y • Unknown cause • Diagnosis of exclusion • Treatment • Increased carbs • Increased protein • Frequent meals

  14. Miscellaneous • Ketotic hypoglycemia • Diagnosis • Decreased insulin • Increased • GH, cortisol, FFA and ketones • Decreased alanine • Normal thyroxine • Normal carnitine • No reducing substances in urine

  15. Miscellaneous • Hormone deficiencies • GH • Cortisol • Ingestions • Ethanol • Salicylates • Oral hypoglycemics • β-blockers

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