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PEDIATRIC ENDOCRINE DISORDERS

PEDIATRIC ENDOCRINE DISORDERS. Pediatric Differences in the Endocrine System. The endocrine system is less developed at birth than any other body system Hormonal control of many body functions is lacking until 12-18 months of age

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PEDIATRIC ENDOCRINE DISORDERS

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  1. PEDIATRIC ENDOCRINE DISORDERS

  2. Pediatric Differences in theEndocrine System • The endocrine system is less developed at birth than any other body system • Hormonal control of many body functions is lacking until 12-18 months of age • Infants might manifest imbalances in concentration of fluids, electrolytes, amino acids, glucose, and trace substances

  3. Understanding the Endocrine System in Children • Puberty brings many changes • ↑GH released • ↑ production of LH and FSH in girls • Development of sexual characteristics • Feedback mechanism in place

  4. Collecting data during an Endocrine Assessment • Percentiles on weight and height • Distinguishing facial features, abdominal fat • Onset of puberty • Routine NB screening • Blood glucose levels • Detection of chromosomal disorders

  5. Phenylketonuria (PKU) • Genetic metabolic disorder characterized by absence of enzyme phenylalanine hydroxylase to breakdown phenylalanine to the amino acid Tyrosine. • As a result, excessive phenylalanine builds up in the blood stream causing permanent damage to brain

  6. Phenylketonuria (PKU) • Clinical Manifestations • Musty or Mousey body and urine odor • Irritability, hyperactivity • Vomiting • Hypertonia, hyperreflexivity • Complication • Seizure disorder • Untreatable mental retardation

  7. Goal of Therapy Keep serum phenylalanine level at 2-6mg/dl

  8. Newborn Screening • Required by state law • Should not be done until feeding on breast milk or formula • Should be done ~48 hrs. after birth • If test shows elevated levels of plasma phenylalanine, repeat test done and if that is elevated, treatment started.

  9. Treatment and Nursing Care for PKU • Special low-phenylalanine formula - Lofenalac, Minafen, and Albumaid XP • Diet low in phenylalanine – for life • Avoid high-protein foods such as meats, fish, eggs, cheese, milk, and legumes. • Avoid aspartame • Take a phenylalanine-free protein supplement to maintain growth • Family support

  10. Treatment for PKU

  11. Galactosemia Maple syrup urine Tay-Sachs disease Inborn Errors Of Metabolism

  12. Galactosemia Carbohydrate metabolic dysfunction Related to a liver enzyme deficiency (GALT) Leads to accumulation of galactose metabolites in the eyes, liver, kidney and brain

  13. Galactosemia • Signs and Symptoms • Poor sucking • Failure to gain weight / IUGR • Vomiting and diarrhea • Hypotonia • Cataracts • Infections • Treatment • Lactose-free formula and diet

  14. Maple Syrup Urine Disease • Disorder of amino acid metabolism • Diagnosis made by UA • Signs and Symptoms: • Poor appetite • Lethargy • Vomiting • High-pitched cry • Seizures • Sweet odor of maple syrup in body fluids • Treatment/management • Removal of the amino acids and metabolites • Diet low in proteins and amino acids

  15. Tay-Sachs Disease • Deadly inherited disease • No treatment • Death by age 4 • Eastern European Ashkenazi Jews • Absence of hexosaminidase A, a protein in nerve tissue which breaks down gangliosides (fatty substances that build up in nerve cells of the brain)

  16. Tay-Sachs Disease • Signs & Symptoms • Deafness • Blindness • Decrease muscle tone • Loss of motor skills • Delayed mental & social skills • Dementia • Paralysis • Seizures

  17. Nursing Measures for Metabolic Disorders Genetic counseling Dietary teaching and compliance Mixing special preparations Mainly supportive

  18. Diabetes – Type 1 Inability of the body to produce or excrete insulin

  19. When are Children most likely to be diagnosed with Diabetes? • Peak incidence is: • 5-7 years of age • Puberty It can occur at any age.

  20. Emerging Trends of Diabetes • Incidence of Type 1 diabetes increasing, the etiology is unknown. This trend is most apparent in very young children • Obesity is causing increased incidence of Type 2 diabetes in children and teens • As children with chronic illness survive longer(i.e. cystic fibrosis)with more extreme measures and (i.e. transplants), diabetes becomes another side effect of their illness

  21. Etiology • Autoimmune process • Inflammatory process in the insulin secreting islet cells of the pancreas • Destruction of the islet cells • Failure to produce or excrete insulin

  22. Pathophysiology Failure to produce insulin leads to elevated blood glucose HYPERGLYCEMIA

  23. Clinical Manifestations Three P’s

  24. How would you tell polyuria in a toddler? Answer: Enuresis in a toilet-trained child

  25. Other manifestations of hyperglycemia • Fatigue – unexplained • Weight Loss (gradual, over several weeks) • Blurred vision • Headache • Hunger

  26. Diagnosis Symptoms of diabetes plus Plasma Glucose Levels of: • Fasting plasma glucose ≥ 126 mg/dl or • Two-hour plasma glucose ≥200 mg/dl or • Random serum glucose concentration ≥200 mg/dl **Ketonuria is a frequent finding**

  27. Therapeutic Management Managed and educated by a multidisciplinary team of experts in pediatric diabetes

  28. Goals of Diabetic Management

  29. These Goals are Met by: • Insulin Administration • Regulations of nutrition and exercise • Stress Management • Blood glucose and urine ketone monitoring

  30. Insulin Therapy • Goal of Insulin Therapy is to replace the insulin the child • is no longer able to make thereby: • Lower blood glucose levels • Stabilize glucose levels • Eliminate ketones

  31. Goals of Insulin Therapy Maintain serum glucose levels from: • Toddlers and preschoolers • 100 – 180 before meals • 110-200 at bedtime • School-age • 90- 180 before meals • 100 – 180 at bedtime • Adolescents • 90 – 130 before meals • 90 – 150 at bedtime

  32. Types of Insulin • Rapid (Lispro/Humalog) • Short acting (regular) • Intermediate acting (NPH, Lente) • Long acting (Lantus/Ultralente)

  33. Basal-bolus Therapy • ADA recommendations for children Administration • Basal insulin administered once a day Glargine (Lantus) or twice daily (Humulin or Ultralente) • Bolus of rapid-acting insulin (Lispro or Aspart) given with each meal and snack or consumes carbohydrates

  34. Route of Administration • Insulin Injections – usually 3 or more per day • Continuous Subcutaneous Insulin Pump Infusion

  35. External Insulin Infusion Pumpin Children Disadvantages • Requires motivation • Requires willingness to be connected to device • Change sites every 2-4 days • More time/energy to monitor BS • Syringe, cath changes every 2-3 days Advantages • Delivers continuous infusion • Maintain better control •  # of injection sites • hypo/hyper episodes • More flexible lifestyle • Eat with more flexibility • Improves growth in child

  36. Factors which may affect insulin dosage in children • Stress • Infection • Illness • Growth spurts (such as puberty) • Meal coverage for finicky toddlers • Adolescents concerned about weight gain not wanting to eat AM snack

  37. Evaluation of Insulin Therapy • Monitored every 3 months • Draw glycosylated hemoglobin value (A1c) • Want the glycosylated hemoglobin value (A1c) to be no higher than 7.5%-8%.

  38. Nutrition and Insulin Needs Children use carbohydrate counting: • 1 CHO choice =15 gm CHO • Young children consume 2-4 choices /meal • Older children and adolescents consume 6-8 choices /meal • **1 unit of insulin covers 8 Gm of CHO. So insulin dosing is based upon meal consumption and number of CHO choices • If >CHO choices are consumed= adjust insulin dose

  39. About Insulin • Store insulin in a cool, dry place; do not freeze or expose to heat or agitation • Check the expiration date on the vial before using • Once opened, date the vial and discard as recommended • When mixing two different types of insulin, inject the appropriate amount of air into both vials, then withdraw the short-acting (clear) insulin first

  40. Treatment and Nursing Care

  41. Newly Diagnosed • Many times the newly diagnosed child is admitted to the hospital in ketoacidosis (DKA) Signs of DKA • Signs of hyperglycemia plus • Abdominal pain / “Stomachache” • Nausea and vomiting • Acetone (fruity)breath odor • Dehydration • Increasing lethargy • Kussmaul respirations • Coma

  42. Treatment for DKA • IV Fluids (boluses) • IV insulin - Wean off IV insulin when clinically stable • Electrolyte replacement • Oral feedings introduced when alert • Prevention of future episodes

  43. Nursing Management at the time of diagnosis • Child is admitted to hospital • Nursing assessments directed toward: • Vital Signs • LOC • Hydration • Hourly monitoring of BS ____________________________________ • Dietary and caloric intake • Ability of family to manage

  44. Focus of Child and Parent Education • Signs and symptoms of hypoglycemia and hyperglycemia and related treatment • Blood-glucose monitoring / urine ketone monitoring • Administration of insulin • “Sick day” guidelines • Nutrition

  45. Sick Day Guidelines • Monitor blood glucose levels more often • Test urine ketones when blood glucose is high • Do NOT skip doses of insulin • Usual doses of insulin may be increased • Encourage large fluid intake,

  46. Hypoglycemia:Blood Sugar < 70mg/dl Symptoms: • Trembling • Sweating, clammy skin • Tachycardia • Pallor • Personality change/ irritable • Slurred speech Treatment: • 15g carbohydrate • Glucagon sub-q • IV glucose • OJ, sweet beverage, raisins, cheese and crackers, candy

  47. HyperglycemiaBlood Sugar > 160mg/dl Symptoms: • Polyuria • Polydipsia • Fatigue • Weight Loss • Blurred vision • Emotional lability • Headache Treatment: • Insulin • Increase oral fluids

  48. Home Teaching • Incorporate into the family lifestyle • “Honeymoon phase” • Community resources • Recognizing the cognitive levels at time of teaching

  49. Nutrition for the Child withType I Diabetes Mellitis • Meals and snacks are balanced with insulin action • Both the timing of the meal or snack and the amount of food are important in avoiding hyperglycemia or hypoglycemia • Adherence to a daily schedule that maintains a consistent food intake combined with consistent insulin injections aids in achieving metabolic control

  50. Exercise for the Child withType I Diabetes Mellitis • Exercise • Avoid exercising during insulin peak • Add an extra 15 to 30g carbohydrate snack for each 45-60 minutes of exercise

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