endocrine. common pediatric endocrine disorders type i diabetes congenital hypothyroidism acquired...

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Endocrine

Common Pediatric Endocrine Disorders

• Type I Diabetes• Congenital Hypothyroidism• Acquired Hypothyroidism (Hashimoto’s

Disease)• Hyperthyroidism (Graves disease)• Growth Hormone Deficiency

The Endocrine System

• Glands• Hormones• Endocrine Disorders are either

• Hypofunction• Primary• Secondary

• Hyperfunction• Primary• Secondary

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

• Infants might manifest imbalances in concentration of fluids, electrolytes, amino acids, glucose, and trace substances

Type 1 Diabetes

Type 1 Diabetes

• Most common endocrine disorder in children

• Pancreas becomes unable to produce and secrete insulin

• Peak age: 5-7, or at puberty• Abrupt onset• Genetic link

Type 1 Diabetes

• Beta cells- type of cell found in the Islets of Langerhans within the pancreas that make and release insulin

• Insulin is a hormone required to move the glucose into cells throughout the body• If no insulin can be produced, the glucose

stays in the blood instead, where it can cause serious damage to all the organ systems of the body

Etiology

• Autoimmune process causes destruction on insulin-secreting cells in the pancreas

• At dx 90% of beta cells are destroyed

Type 1 Diabetes

No cure, but JDRF is funding studies that perfect pancreas transplantation and regeneration the body’s own beta cells without islet transplantation

Serum Glucose Levels

• Normal fasting glucose 70-110mg/dl

• Glycosated Hemoglobin (Hgb A1C) < 6.0%

Initial Clinical Manifestations

PolyuriaPolydipsia PolyphagiaFatigue Blurred visionHeadacheShortened attention spanMood changes

Diabetic Ketoacidosis(DKA)

• Medical Emergency• As glucose levels rise, child will progress

into DKA if not treated• Blood glucose levels > 300

• Cellular starvation leads to ketone production• Nausea, vomiting, abdominal pain• Acetone (fruity) breath odor• Dehydration• Kussmaul respirations

• Coma if untreated

When to Monitor for DKA

• Abdominal pain• Nausea and vomiting that persists for over

6 hours• More than five diarrheal stools in 1 day• A 1- or 2-day history of polyuria and

polydipsia• Has illness (e.g., viral or other) and is

unable to eat

Type 1 Diabetes Treatment

• Multidiscipline• Promote normal growth and development• Optimal glucose control• Minimal complications• Adjustment to disease• Treatment consists of

• Insulin replacement• Diet• Blood glucose monitoring• Exercise

Diet Therapy

• Consistent intake & timing of food to correspond to insulin prescribed

• Well-balanced, enough caloric intake to support growth and development

• Three meals, snacks spaced throughout the day

• No diet, sugar-free foods• Don’t omit meals

Exercise• Encouraged, never restricted• Lowers blood glucose levels, by

aiding the body’s use of food• Decreases insulin requirements• Proper snack before

• Add an extra 15- to 30-g carbohydrate snack for each 45-60 minutes of exercise

Blood Glucose Monitoring

Finger stick glucose monitoring several times a day

Urine testing for glucose and ketones Teach good record keeping Self-management at age appropriate level

2-6 choose food, clean finger for stick 4-6 dip own urine 6-8 Blood glucose management 8-10 insulin injections, keep diary 10-14 nutritional decisions 12-18 full management

InsulinPrecise dose cannot be predicted

Amount is based upon average capillary or serum blood glucose levels

Will change based of growth

Can be administered twice daily

Types of InsulinSynthetic Human Insulin

• Rapid acting• Lispro (Humalog)• Aspart (Novolog)

• Fast acting• Regular ®

• Intermediate• NPH (N)

• Mixed (70/30)• Long Acting

• Glargine (Lantus)• Ultra Lente

Typical Management

• The peak of the insulin should occur Post-Prandial (after meal) to avoid hypoglycemia

Insulin

• Alternate sites • Don’t inject

extremity to be used in sports

• Give at room temperature

• Always draw regular up first if mixing

Pumps

• Delivers fixed amounts of short-acting insulin continuously

• Worn on a belt, the tubing & catheter are changed Q48 hours and taped in place

• Should not be removed for > 1-2 hours

• Subject to minimal malfunction

• Must be self-motivated

Pumps• Advantages

• Less scar tissue• No daily injections• Less to carry• Private• Sense of control

• Disadvantages• Must wear continuously• Need to carry extra battery• Good BGM• If insurance dose not cover

$$$$$• Still need emergency

needles, insulin, and remember how to inject

Illness alters diabetic management

• Dosage requirements may increase, decrease, or remain unchanged depending on the severity of the illness & the child’s appetite

• Rapid-acting insulin only is used to manage hyperglycemia associated with illness

• Monitor fluids, may require extra oral fluids while ill

“Sick Day” Guidelines

• Seek medical attention for fever or other signs of infection.

• Monitor the blood glucose levels more often than routine (1 to 4 hours).

• Test urine ketones when the blood glucose level is greater than 200 mg/dL.

• Do not skip doses of insulin.

• Large fluid intake (drinks with carbohydrates) is essential if the child cannot eat as usual.

• If the child cannot consume adequate amounts of fluids, seek medical attention.

Hyperglycemia BG > 110Gradual onsetLethargic, fatigueConfusedWeakness

PolyuriaPolydypsiaPolyphagia

Glucose 250 mg/dl Large ketones in blood & urine Blurred vision Ketoacidosis Coma

Hypoglycemia BG < 70Commonly occurs before meals

when the insulin effect is peaking

Burst of physical activity without additional food

Delayed, omitted, or incompletely consumed meals or snacks

Too much insulin-wrong dose

Hypoglycemia BG < 70Rapid onset•Irritable, nervousness•Difficulty concentrating•Shaky feeling, tremors, hunger•Diplopia•Pallor•Weakness•Headache, dizziness•Sweating•Unconsciousness and convulsions

Treatment of Hypoglycemia

Give simple concentrated sugarGlucose gel or SL tablets Hard candySugar cubesLow-fat milk or OJ

Followed by a complex CHO & ProteinSlice of bread or cracker with peanut butterGlucagon SQ for severe hypoglycemia (may cause

vomiting, prevent aspiration)

Nursing Considerations Begins with survival education

Educate child & family regarding Nature of disease, hypo/hyperglycemia Meal planning (3 spaced meals, 3 snacks) Wearing ID bracelet Effective duration, onset & peak action of insulin Injection procedure, rotate sites Glucose monitoring, urine testing, record keeping Exercise regime

Nursing Considerations• Provide emotional support

• Encourage growth and development

• Identify home care needs

Disorders of the Thyroid

• Congenital Hypothyroidism

• Acquired Hypothyroidism

• Hyperthyroidism

Congenital Hypothyroidism

CONGENITAL HYPOTHYROIDISM

• Disorder at birth

• Body is producing insufficient thyroid hormone to meet metabolic needs caused by absent or underdeveloped thyroid gland

• If not treated can lead to severe cognitive impairment

• Detected in Newborn Screen

CONGENITAL HYPOTHYROIDISM

• Caused by defect in the embryonic period in thyroid glad production

• Also caused by inborn error of thyroid hormone synthesis (an inherited autosomal recessive trait)

• Can be secondary to pituitary dysfunction

• Thyroid gland is unable to produce T3 and T4

Clinical Manifestations • Mottled skin• Large fontanel• Large tongue• Hypotonia/slow

reflexes• Distended abdomen• Low T4 < 6• High TSH > 40

CONGENITAL HYPOTHYROIDISM

•An infant with a low T4 <6 and a TSH value exceeding 40 is considered to have primary hypothyroidism until proven otherwise

Treatment• Treated with lifelong thyroid replacement

therapy• Synthroid 10-15 mcg/kg/day

• Taken 30-60 minutes before meals for optimal absorption

• Crush pill and mix with formula or breast milk

Parental Education

• Monitor Growth and Development and Thermoregulation

• Labs every 2 weeks then every 3 months look for upper range of normal

• Stress medication compliance

• Teach parents to monitor for medication induced hyperthyroidism

Signs of Medication induced Hyperthyroidism

•Nervousness/anxiety•Diarrhea•Heat intolerance•Weight loss• Increased HR

Outcome

• Prevention of cognitive impairment– newborn screening on all babies

• Early treatment has had significant impact on morbidity

• Most children progress to within normal ranges on developmental assessment

• Poor prognosis in more severe cases

Acquired Hypothyroidism (Hashimoto’s disease)

Acquired Hypothyroidism

• Autoimune disorder• Antibodies and developed against

thyroid gland• Gland becomes inflamed, infiltrated by

antibodies and destroyed• Thyroid produces inadequate levels of

thyroid hormone > age 2• T4 decreases, TSH rises

Types of Acquired Hypothyroidism

• Primary (Hashimoto’s thyroiditis)• Most common• Autoimmune• Childhood, adolescents, females>males

• Secondary• Associated with other conditions that affect the

thyroid• Pituitary and hypothalmic dysfunction

• Tertiary • Radiation, surgery, trauma

Clinical Manifestations

• Goiter• Dry, thick skin• Coarse hair• Fatigue• Cold intolerance• Delayed puberty and

menses

• Decelerated growth

• Edema around eyes, face and hands

• Constipation• Sleepiness • Mental decline-not

permanent cognitive impairment

Acquired Hypothyroidism Treatment

• Thyroid hormone replacement-Synthroid• Starting dose 10 -15 mcg/kg/day

• Taken 30-60 minutes before meals for optimal absorption

• Teach child to swallow pill or crush • Repeat thyroid function test one month should see

normalization of TSH• Requires lifetime follow up• Dose and adjustments based on clinical evaluation & TSH• Prognosis is good if kept euthyroid (normal)

Acquired Hyperthyroidism (Grave’s Disease)

Acquired Hyperthyroidism• A hyperfunction of the thyroid gland• Produces excessive circulating thyroid

hormone (T3 and T4)• Four times more common in girls• Occurs between the ages of

12 – 14 yrs. (puberty)• Manifestations develop gradually with an

interval between onset & diagnosis of 6 to 12 months

• Genetics involved• Follows a viral illness or period of stress

Clinical Manifestations

• Emotional liability• Physical restlessness at rest• Decreased school performance• Excessive appetite without weight gain• Fatigue• Hair fine, unable to curl• Diarrhea• Poor attention span• Insomnia• Increased perspiration/heat intolerance

Clinical Manifestations

• Increased HR • Palpitations• Widened pulse pressure• Exothalmos• Wide-eyed expression with lid lag• Fine tremors• Systolic murmurs

Thyroid Storm

• Acute Onset• Severe irritability & restlessness• Vomiting and diarrhea• Hyperthermia• Hypertension• Severe tachycardia• Prostration• May progress to death

Treatment

• To suppress thyroxine• PTU - propythioracil • MTZ – methimazole

• Subtotal thyroidectomy

• Ablation with radioiodine

Nursing Care• Needs quiet un-stimulating environment

conducive to rest• Maintain a regular routine to minimizing

stress of coping with unexpected demands

• Physical activity is restricted• Tire easily, experience muscle

weakness and are unable to relax to recoup their strength

Nursing Care

• Increased need for calories to meet their metabolic rate

• Offer 5-6 moderate meals throughout the day, and vitamin supplements

• Stress good hygiene because of excessive sweating

• If taking PTU or MTZ observe for side effects of medications• Neutropenia and Hepatotoxicity

Nursing Care

• If surgery is planned administer iodine a few weeks before the procedure• Mixed in a strong-tasting fruit juice given

through a straw• Fear of having throat cut is real• Post-op position neck slightly flexed and

observe for bleeding• Supplemental thyroid hormone then for life

Hypothyroidism Hyperthyroidism

Tiredness/fatigue Nervousness/anxiety

Constipation Diarrhea

Cold intolerance Heat intolerance

Dry, thick skin Smooth, velvety skin

Edema of face, eyes, hands

Prominent eyes

Decreased growth Accelerated linear growth

Decreased activity/energy Emotional liability

Muscle hypertrophy Muscle weakness

Decreased heart rate Increased heart rate

Growth Hormone Deficiency

Growth Hormone Deficiency

• Failure of the pituitary to produce growth hormone

• Affected boys=girls• Boys tend to be evaluated more• 75% cause is idiopathic• Can be a result of injury and destruction of

anterior pituitary gland from• Brain tumor• Infection• radiation

Symptoms

• Normal size and weight at birth• Within first few years child will fall below

the 3rd percentile on growth chart• Late onset of puberty• Delayed dentition• High-pitched voice• Child-like face with large forehead

Criteria for Suspecting Growth Hormone (GH) Deficiency

• Consistently poor growth (<5 cm/yr)• Growth rate more than two standard

deviations below the mean for age• Downward deviation from the previous

growth curve

Assessment and Diagnosis• Evaluate family history• Prenatal/birth history R/O pituitary tumor• Growth charts

• Diagnosis• X ray, MRI to study bone age• Pituitary function tests

Management

• IM recombinant human growth hormone 2-3 times per week

• Given at bedtime when GH usually peaks• GH is a powder that needs to be mixed

with diluent• Parents/child need teaching• Rapid growth is often painful, pain

management is needed

Nursing Considerations

• Speak to child in age appropriate manner (be careful not to address as a younger child)

• Be discrete when providing step stools, etc• Provide with anticipatory guidance for

adolescence• Dress in clothing that reflects age not size• Choose sports that height is not a requirement

Practice Questions!

A 10-year old with type 1 diabetes tells the school nurse that he has some early signs of hypoglycemia. The nurse recommends that the child:

1. Take an extra injection of regular insulin2. Drink a glass of orange juice3. Skip the next dose of insulin4. Start exercising

An adolescent with Type I diabetes has had several episodes demonstrating lack of diabetic control. The nurse teaches the client by stating: “The best way to maintain control of your disease is to:

1. Check your urine glucose three times a week2. Check the HgA1C every 3 months and every 6 months

when stable3. Check your BG four times a day and HgA1C every 3

months4. Check glucose daily as long as you feel well

A 10-year-old girl with type 1 diabetes comes to the office of the school nurse after recess. She was just out of school for an extended illness and reports that she returned to her usual insulin dosing schedule today. The nurse notices she is nervous with hand tremors, pale, sweaty, and complaining of feeling drowsy. The nurse suspects:

1. Exercise-induced hypoglycemia2. Hyperglycemia caused by increased intake at lunch3. Ketoacidosis caused by infection4. The child is avoiding returning to class

After being diagnosed with Hyperthyroidism, a teenager begins taking PTU for treatment of the disease. What symptom would indicate to the nurse that the dose may be too high?

1. Weight loss2. Polyphagia3. Lethargy4. Difficulty with school work

A child with type 1 diabetes 7:00 am blood glucose is 189. The previous evening at 5:30 pm the child injected rapid insulin and NPH, ate dinner and had a 10 pm snack. The nurse concludes (select all that apply)

 1. Rapid insulin dose may be to low2. NPH insulin dose may be to low3. NPH insulin dose may be to high4. The child ate too little dinner5. The child ate too much snack

• The nurse is teaching a parent of a child with type 1 diabetes about the different types of insulin. The nurse assumes the parent understands rapid insulin peak times if the parent states that when injecting at 6:45 am, be sure the child does not miss:1. 7:00 AM Breakfast2. 12:30 PM Lunch3. 6:30 PM Dinner4. 10:00 AM Snack