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 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

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

Galactosemia maple syrup urine tay sachs disease


Maple syrup urine

Tay-Sachs disease

Inborn Errors




Carbohydrate metabolic dysfunction

Related to a liver enzyme deficiency (GALT)

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


  • 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


  • Autoimmune process

  • Inflammatory process in the insulin secreting islet cells of the pancreas

  • Destruction of the islet cells

  • Failure to produce or excrete insulin


Failure to produce insulin leads to elevated blood glucose


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


Symptoms of diabetes plus Plasma Glucose Levels of:

  • Fasting plasma glucose ≥ 126 mg/dl


  • Two-hour plasma glucose ≥200 mg/dl


  • 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

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


  • 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


  • 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


  • 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

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


  • Trembling

  • Sweating, clammy skin

  • Tachycardia

  • Pallor

  • Personality change/ irritable

  • Slurred speech


  • 15g carbohydrate

  • Glucagon sub-q

  • IV glucose

  • OJ, sweet beverage, raisins,

    cheese and crackers, candy

Hyperglycemia blood sugar 160mg dl
HyperglycemiaBlood Sugar > 160mg/dl


  • Polyuria

  • Polydipsia

  • Fatigue

  • Weight Loss

  • Blurred vision

  • Emotional lability

  • Headache


  • 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