Pediatric endocrine disorders
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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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Pediatric endocrine disorders

PEDIATRIC ENDOCRINE DISORDERS


Pediatric differences in the endocrine system

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


Understanding the endocrine system in children

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


Collecting data during an endocrine assessment

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


Phenylketonuria pku

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


Phenylketonuria pku1

Phenylketonuria (PKU)

  • Clinical Manifestations

    • Musty or Mousey body and urine odor

    • Irritability, hyperactivity

    • Vomiting

    • Hypertonia, hyperreflexivity

  • Complication

    • Seizure disorder

    • Untreatable mental retardation


Pediatric endocrine disorders

Goal of Therapy

Keep serum phenylalanine level at 2-6mg/dl


Newborn screening

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.


Treatment and nursing care for pku

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


Treatment for pku

Treatment for PKU


Galactosemia maple syrup urine tay sachs disease

Galactosemia

Maple syrup urine

Tay-Sachs disease

Inborn Errors

Of

Metabolism


Galactosemia

Galactosemia

Carbohydrate metabolic dysfunction

Related to a liver enzyme deficiency (GALT)

Leads to accumulation of galactose metabolites in the eyes, liver, kidney and brain


Galactosemia1

Galactosemia

  • Signs and Symptoms

    • Poor sucking

    • Failure to gain weight / IUGR

    • Vomiting and diarrhea

    • Hypotonia

    • Cataracts

    • Infections

  • Treatment

    • Lactose-free formula and diet


Maple syrup urine disease

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


Tay sachs disease

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)


Tay sachs disease1

Tay-Sachs Disease

  • Signs & Symptoms

    • Deafness

    • Blindness

    • Decrease muscle tone

    • Loss of motor skills

    • Delayed mental & social skills

    • Dementia

    • Paralysis

    • Seizures


Nursing measures for metabolic disorders

Nursing Measures for Metabolic Disorders

Genetic counseling

Dietary teaching and compliance

Mixing special preparations

Mainly supportive


Diabetes type 1

Diabetes – Type 1

Inability of the body to produce or excrete insulin


When are children most likely to be diagnosed with diabetes

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.


Emerging trends of diabetes

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


Etiology

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


Pathophysiology

Pathophysiology

Failure to produce insulin leads to elevated blood glucose

HYPERGLYCEMIA


Clinical manifestations

Clinical Manifestations

Three

P’s


Pediatric endocrine disorders

How would you tell polyuria in a toddler?

Answer: Enuresis in a toilet-trained child


Other manifestations of hyperglycemia

Other manifestations of hyperglycemia

  • Fatigue – unexplained

    • Weight Loss (gradual, over several weeks)

    • Blurred vision

    • Headache

    • Hunger


Diagnosis

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**


Therapeutic management

Therapeutic Management

Managed and educated by a multidisciplinary team of experts in pediatric diabetes


Goals of diabetic management

Goals of Diabetic Management


These goals are met by

These Goals are Met by:

  • Insulin Administration

  • Regulations of nutrition and exercise

  • Stress Management

  • Blood glucose and urine ketone monitoring


Insulin therapy

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


Goals of insulin therapy

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


Types of insulin

Types of Insulin

  • Rapid (Lispro/Humalog)

  • Short acting (regular)

  • Intermediate acting (NPH, Lente)

  • Long acting (Lantus/Ultralente)


Basal bolus therapy

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


Route of administration

Route of Administration

  • Insulin Injections – usually 3 or more per day

  • Continuous Subcutaneous Insulin Pump Infusion


External insulin infusion pump in children

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


Factors which may affect insulin dosage in children

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


Evaluation of insulin therapy

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%.


Nutrition and insulin needs

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


About insulin

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


Pediatric endocrine disorders

Treatment and Nursing Care


Newly diagnosed

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


Treatment for dka

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


Nursing management at the time of diagnosis

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


Focus of child and parent education

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


Sick day guidelines

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,


Hypoglycemia blood sugar 70mg dl

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


Hyperglycemia blood sugar 160mg dl

HyperglycemiaBlood Sugar > 160mg/dl

Symptoms:

  • Polyuria

  • Polydipsia

  • Fatigue

  • Weight Loss

  • Blurred vision

  • Emotional lability

  • Headache

Treatment:

  • Insulin

  • Increase oral fluids


Home teaching

Home Teaching

  • Incorporate into the family lifestyle

  • “Honeymoon phase”

  • Community resources

  • Recognizing the

    cognitive levels

    at time of teaching


Nutrition for the child with type i diabetes mellitis

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


Exercise for the child with type i diabetes mellitis

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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