PEDIATRIC ENDOCRINE DISORDERS

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

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

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

Clinical Manifestations Musty or Mousey body and urine odor Irritability, hyperactivity Vomiting Hypertonia, hyperreflexivity

Complication Seizure disorder Untreatable mental retardation

Phenylketonuria (PKU)Phenylketonuria (PKU)

Goal of TherapyGoal of Therapy

Keep serum phenylalanine level at Keep serum phenylalanine level at 2-6mg/dl2-6mg/dl

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

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

Treatment for PKU

GalactosemiaMaple syrup urine Tay-Sachs disease

GalactosemiaGalactosemia

Carbohydrate metabolic dysfunctionRelated to a liver enzyme deficiency (GALT)Leads to accumulation of galactose metabolites in the eyes, liver, kidney and brain

GalactosemiaGalactosemia

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 DiseaseMaple 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 DiseaseTay-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 DiseaseTay-Sachs Disease

Signs & Symptoms Deafness Blindness Decrease muscle tone Loss of motor skills Delayed mental & social skills Dementia Paralysis Seizures

Nursing Measures for Metabolic Nursing Measures for Metabolic DisordersDisorders

Genetic counseling

Dietary teaching and compliance

Mixing special preparations

Mainly supportive

Diabetes – Type 1Diabetes – Type 1

Inability of the body to produce or Inability of the body to produce or excrete insulinexcrete insulin

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

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 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

HYPERGLYCEMIA

Three P’s

Clinical Manifestations

How would you tell polyuriapolyuria in a

toddler?

Answer: Enuresis in a toilet-trained child

Other manifestations of hyperglycemia

Fatigue – unexplained

Weight Loss (gradual, over several weeks)

Blurred vision Headache Hunger

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

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

Goals of Diabetic Management

These Goals are Met by:

Insulin Administration

Regulations of nutrition and exercise

Stress Management

Blood glucose and urine ketone monitoring

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

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

Types of Insulin

Rapid (Lispro/Humalog)

Short acting (regular)

Intermediate acting (NPH, Lente)

Long acting (Lantus/Ultralente)

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

Insulin Injections – usually 3 or more per day

Continuous Subcutaneous Insulin Pump Infusion

Route of Administration

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

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

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

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 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

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

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

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

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

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

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

HyperglycemiaBlood Sugar > 160mg/dl

Symptoms: Polyuria Polydipsia Fatigue Weight Loss Blurred vision Emotional

lability Headache

Treatment: Insulin Increase oral

fluids

Home Teaching

Incorporate into the family lifestyle “Honeymoon phase” Community resources Recognizing the cognitive levels at time of teaching

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 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