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PEDIATRIC ENDOCRINE DISORDERS
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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
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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
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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
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Phenylketonuria (PKU)
Genetic metabolic disorder Genetic metabolic disorder characterized by absence of characterized by absence of enzymeenzyme
Phenylalanine hydroxylase to Phenylalanine hydroxylase to breakdown phenylalanine. breakdown phenylalanine.
As a result, excessive As a result, excessive phenylalanine builds up in the phenylalanine builds up in the blood stream causing blood stream causing permanent damage to brainpermanent damage to brain
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Clinical Manifestations Musty or Mousey body and urine odor Irritability, hyperactivity Vomiting Hypertonia, hyperreflexivity
Complication Seizure disorder Untreatable mental retardation
Phenylketonuria (PKU)
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Goal of TherapyGoal of Therapy
Keep serum phenylalanine level at Keep serum phenylalanine level at 2-6mg/dl2-6mg/dl
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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.
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Treatment and Nursing Care for PKU
1. Special low-phenylalanine formula - Lofenalac, Minafen, and Albumaid XP
2. Diet low in phenylalanine – for life Avoid high-protein foods such as meats, fish,
eggs, cheese, milk, and legumes. Avoid aspartame
3. Take a phenylalanine-free protein supplement to maintain growth
4. Family support
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Treatment for PKU
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GalactosemiaMaple syrup urine Tay-Sachs disease
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Carbohydrate metabolic dysfunctionRelated to a liver enzyme deficiency (GALT)Leads to accumulation of galactose metabolites in the eyes, liver, kidney and brain
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Signs and Symptoms Poor sucking Failure to gain weight / IUGR Vomiting and diarrhea Hypotonia Cataracts Infections
Treatment Lactose-free formula and diet
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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
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Nursing Measures for Metabolic Disorders
Genetic counseling
Dietary teaching and compliance
Mixing special preparations
Mainly supportive
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DiabetesDiabetes
Inability of the body to produce or Inability of the body to produce or excrete insulinexcrete insulin
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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.
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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
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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
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Failure to produce insulin leads to elevated blood glucose
HYPERGLYCEMIA
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Three P’s
Clinical Manifestations
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How would you tell polyuriapolyuria in a
toddler?
Answer: Enuresis in a toilet-trained child
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Other manifestations of hyperglycemia
Fatigue – unexplained
Weight Loss (gradual, over several weeks)
Blurred vision Headache Hunger
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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**
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Therapeutic Management
Managed and educated by a multidisciplinary team of experts in pediatric diabetes
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Goals of Diabetic Management
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These Goals are Met by:
Insulin Administration
Regulations of nutrition and exercise
Stress Management
Blood glucose and urine ketone monitoring
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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
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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
Goals of Insulin Therapy
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Types of Insulin
Rapid (Lispro/Humalog)
Short acting (regular)
Intermediate acting (NPH, Lente)
Long acting (Lantus/Ultralente)
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Basal-bolus Therapy
ADA recommendations for childrenAdministrationAdministration
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
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Insulin Injections – usually 3 or more per day
Continuous Subcutaneous Insulin Pump Infusion
Route of Administration
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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
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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
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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%.
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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
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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
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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
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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
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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
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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
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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,
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Hypoglycemia:Blood Sugar < 70mg/dl
Symptoms: Trembling Sweating, clammy
skin Tachycardia Pallor Personality change/
irritable Slurred speech
Treatment: 15g carbohydrate Glucogon sub-q IV glucose OJ, sweet beverage, raisins, cheese and crackers, candy
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HyperglycemiaBlood Sugar > 160mg/dl
Symptoms: Polyuria Polydipsia Fatigue Weight Loss Blurred vision Emotional
lability Headache
Treatment: Insulin Increase oral
fluids
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Home Teaching
Incorporate into the family lifestyle “Honeymoon phase” Community resources Recognizing the cognitive levels at time of teaching
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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
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Exercise for the Child withType I Diabetes Mellitis
Exercise Avoid exercising during insulin peak Add an extra 15- to 30-g carbohydrate
snack for each 45-60 minutes of exercise