Growth Hormone Deficiency

Report
Endocrine Disorders
Jan Bazner-Chandler
CPNP, MSN, CNS, RN
BMI

In recent years, BMI has received increased
attention for pediatric use. In 1994, an expert
committee charged with developing guidelines for
overweight in adolescent preventive services (ages
11-21 years) recommended that BMI be used
routinely to screen for overweight adolescents. In
addition, in 1997 an expert committee on the
assessment and treatment of childhood obesity
concluded that BMI should be used to screen for
overweight children, ages 2 years and older, using
the BMI curves from the revised growth charts.
BMI Calculation

Can be calculated on-line at various sites
including www.cdc.gov
Growth Charts

The growth charts consist of a series of percentile
curves that illustrate the distribution of selected body
measurements in U.S. children. Pediatric growth
charts have been used by pediatricians, nurses, and
parents to track the growth of infants, children, and
adolescents in the United States since 1977. The
1977 growth charts were developed by the National
Center for Health Statistics (NCHS) as a clinical tool
for health professionals to determine if the growth of
a child is adequate. The 1977 charts were also
adopted by the World Health Organization for
international use.
Tests and Procedures








BUN, Creatinine, electrolytes, glucose
Hormone levels
Stimulation studies
Urinalysis
Fluid deprivation studies
Radiographs: bone age studies
Thyroid scan
Ultrasound to thyroid
Endocrine Disorders





Growth hormone deficiencies
Hypo and hyper thyroid
Diabetes type I and type II
Diabetes Insipidus
PKU
Disorders of the Pituitary Gland

Disorders of the pituitary gland depend on the
location of the lesion or physiologic
abnormality.
Anterior Pituitary

The anterior pituitary is made up of endocrine
glandular tissue and secretes growth
hormone (GH), adrenocorticotropic hormone
(ACTH, TSH, FSH, LH, and prolactin).
Growth Hormone Deficiency






Failure of the pituitary to produce sufficient
growth hormone to sustain normal growth in
children
80% are idiopathic
Familial patterns
Males are referred more often
Review growth charts
Short at birth or preemie
Assessment




Cherub facial features, frontal bossing, large eyes,
and button nose
Males have small testes / micro-penis
Look much younger than chronological age
Delay of onset of puberty as a teenager
Emotional Difficulties





Emotional difficulties related to small stature
are common
Short child is often treated as if younger
Teased by peers
Child may dress as a younger child
Body image is altered
Hypopituitarism
Diagnostic Tests



Renal and Liver function test
Thyroid function
Sedimentation rate / ESR
Done to rule out other causes of delayed
growth
Definitive Diagnosis



Deficiency in the Growth Hormone
Bone age by x-ray: delayed bone age
Slow growth rate: as documented on
standard CDC growth chart
Goals of Therapy


The goal of therapy is to augment growth so
that at the time of epiphyseal close, a normal
or normally expected adult height is attained.
Child will attain a final adult height consistent
with their genetic potential
Growth Hormone Replacement

GH products are currently labeled for use in
“children who have growth failure due to an
inadequate secretion of normal endogenous
growth hormone”
Hormone Replacement Therapy


Parents and child need to be educated on
proper way to reconstitute and administer the
GH.
Subcutaneous injection 3 to 7 days per week
Interdisciplinary Interventions



Children should be managed by a pediatric
endocrinologist
Height and weight is obtained every 3 months
and plotted on the growth chart
Bone age study yearly
Ethical Issues

Social Justice Considerations



Children must meet specific criteria to be eligible
for treatment
Parents must have access to health insurance
coverage
Children who receive GH therapy will obtain the
economic and social benefits of growing taller
Outcomes of Treatment

The child will verbalize positive feelings about
his or her body image.

The child will demonstrate an increase in
age-appropriate activities with peers.

Child will be able to participate in age related
activities of daily living
Long Term Effects

Long term follow up needed:




Long term risks unknown
Physiologic trauma of daily injection
Metabolic effects of the therapy: children on GH
therapy are usually lean and muscular
Therapy associated with increase risk of
malignancies: leukemia, lymphoma, and tumors
Precocious Puberty

Development of sexual characteristics before
the usual age of onset of puberty.

Girls




Breast development before 7.5 years
Pubic hair before 8.5 years
Menses before 9.5 years
Boys

Secondary sexual characteristics before age 9
Assessment


Chart growth on growth chart.
Chronological timing of pubertal events.


Tanner Scale: true precocious puberty is
characterized by 2 signs of puberty
Family history
Management / Prognosis



Treatment to halt or reverse sexual
development.
Treatment needs to be started prior to
closure of epiphysis.
Good outcomes if treatment stared early
Delayed Puberty

Failure to develop sexually at an appropriate
age.

Girls


No breast development by age 13 or lack of menses
within 5 years.
Boys

Secondary sexual characteristics not started by 14 years
of age.
Rule out any Endocrine Abnormalities

12% will have a pathologic reason for
delayed puberty



Congenital adrenal hyperplasia
Hypothyroidism
Growth hormone deficiency
Pharmacologic Interventions

Low dose testosterone for the male.

Oral ethinyl estradiol for the girl.
Hypothyroidism


Most common endocrine disorder of
childhood
Hypothyroidism can be congenital, acquired,
or secondary
Congenital Hypothyroidism


Results from absence or abnormal
development of the thyroid gland or abnormal
synthesis of thyroid hormone.
Most common cause is incomplete
development of the thyroid gland
Importance of Thyroid Hormones


Thyroid hormones promote normal
myelination during brain development in the
first two to three years of life and normal
skeletal growth
Regulates metabolism
Assessment

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
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Dull appearance
Feeding difficulties
Inactivity
Constipation
Characteristic faces

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Flat nasal bridge
Puffy eyelids
Thick protruding tongue
Low hairline
Large posterior fontanel
Diagnosis

Diagnosis


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Positive health history
Physical findings
Low levels of T3 and T4
High levels of TSH
Neonatal screening is mandatory
Management




Replacement of sodium-l-thyroxine
Monitor TSH, T3 and T4
Monitor growth and development
Frequent visits with emphasis on importance
of therapy
CaREminder

Early diagnosis and prompt treatment of
congenital hypothyroidism is essential for
normal growth and development. The greater
the delay treating congenital hypothyroidism,
the greater the degree of cognitive challenge.
With early diagnosis and treatment, children
with congenital hypothyroidism can develop
normally.
Acquired Hypothyroidism




15% of Down Syndrome children are
hypothyroid
Auto-immune type of thyroiditis is most often
the cause
High TSH levels as young as 2 years of age
Difficult to diagnose due to overlap of
symptoms
Hyperthyroidism





Excessive secretion of thyroid hormone
More common in females 7:1
Genetic and immunologic components
HLA-B8
Autoimmune disease of unknown cause
Assessment


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Cry easily
Emotionally labile
Nervous
Short attention span
Can’t sit still / Hyperactive
Fatigue but unable to sleep at night
Accelerated growth / tall for age
Physical Exam



Enlarged thyroid gland
Asymmetric or lobular
Patient may present with neck swelling
Exophthalmos
Diagnosis
History and Physical
Levels of T3 and T4 are increased
Levels of TSH are decreased
Pharmacologic Interventions

Antithyroid drugs to block T 4 synthesis


Prophylthiouracil
Methimazole (Tapaxole)
Permanent Treatment

Radioactive Iodine is given to kill off some of
the thyroid cells


Most common negative outcome is giving too
much iodine that all thyroid producing cells are
killed.
Surgical removal of gland or nodule – not
always possible since often it is the entire
gland resulting in overproduction of the
hormone.
Disorders of the Pancrease


Diabetes Mellitus – type 1 diabetes
Type 2 Diabetes
Diabetes Mellitus / Type 1




Lack of insulin production in the pancreas.
Autoimmunity involved in destruction of beta
cells.
15 new cases per 100,000 children under 20
years of age.
Peak incidence between 10 and 14 years.
Diabetes Type I





Result of a genetic-environmental interaction
Seasonal variation – midwinter to spring
Family history
Illness or infection preceding the onset
Virus triggers the autoimmune response
Genetic Marker

Genetic Markers:


HLA –DR4 and HLA – DR3
20 to 40 % more susceptible
Natural History


Exposure of genetically predisposed
individuals to environmental triggers
Leads to inflammation of beta cells of the
pancreatic islets (islitis) and subsequent betacell injury.
Beta Cell Function

Hyperglycemia
 80 to 90% if beta cell function must be lost
before hyperglycemia develops
Pathophysiology



Insulin deficiency causes physiologic and
metabolic changes in the body.
Glucose from dietary sources cannot be
utilized by the cells.
Renal tubules have difficulty reabsorbing the
glucose.
Pathophysiology



If the blood glucose level exceeds the renal
threshold for glucose osmotic diuresis
ensues.
Renal threshold: when serum glucose levels
approach 180 mg/dl the renal tubules have
difficulty re-absorbing the glucose
Hyperglycemia impairs leukocyte function –
yeast infection
Assessment




Weight loss – as much as 30%
Elevated blood glucose leads to osmotic
diuresis. (polyuria and thirst)
Protein and fat breakdown lead to weight
loss.
Accumulation of ketones causes a drop in
pH. (metabolic acidosis) and spilling of
ketones in the urine
Presenting Symptoms
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Hyperglycemia / glucose in blood stream
Glucosuria / sugar in urine
Polyuria / increased urine output
Electrolyte imbalance from dehydration
Polydipsia / attempt to relieve dehydration
Polyphagia / attempt to compensate for lost
calories
Diagnostic Tests



Blood glucose levels greater than 200 mg/dL
Urine sample reveals glucosuria and possible
ketonuria.
Glucose tolerance test would reveal low
insulin levels in the face of elevated glucose
levels.
Goals of Management

Short term goals:




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Prevent the development of ketosis.
Prevent electrolyte abnormalities and volume depletion
secondary to osmotic diuresis.
Prevent impairment of leukocyte function
Prevent impairment of wound healing
Long term goal: prevention of microcirculatory and
neuropathic changes
Interdisciplinary Interventions


Administration of insulin
Blood glucose levels


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Initially before every meal
Every am when diabetes under control
Dietary management / refer to nutritionist
Glycosylated hemoglobin / reflects average
glucose concentration for preceding 2 to 3
months. (A1C)
Blood Glucose Levels

Target levels



Toddler and preschool: 100 to 180 mg/dL
School-age: 90 to 180 mg/dL
Adolescents (13 to 19 years): 90 to 130 mg/dL
Urine

Test urine for ketones only if blood sugar
greater than 250 or during illness
Insulin

Insulin


Short acting – often used to cover extra
carbohydrate consumption
Combination of regular and intermediate-acting
insulin


Morning and evening dosing
Children on mixed insulin dosage schedules tend
to experience hypoglycemic episodes at 11:30
and 2:30 as peaking of insulin occurs.
Hypoglycemia
Symptoms:
 Rapid onset
 Shaky feeling, hunger
 Headache
 Dizziness
 Vital signs



Shallow respirations
tachycardia
Tremors
Lab Values:

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Glucose = low, below 60
Ketones = negative
Urine output



Normal
sugar negative
negative ketones
Treatment of Hypoglycemia

Day time hypoglycemia:
 Simple concentrated sugars such as honey by mouth,
hard candy, sugar cubes, or glucose tablets will
elevate the blood sugar immediately. Orange juice or
sugar containing soda or fruit drink. (Blood Glucose
less than 70 mg/dL)
 Eat a snack if next meal is more than an hour away
 Identify reason for hypoglycemia. In children it is often
increase in activity without increase in food intake.
Hypoglycemia Prevention

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
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Using rapid-acting or Lispro insulin
Infusion pump (8 to 10 years of age)
Night time snack
Check blood glucose before bedtime
Do not skip snacks
Eat an extra snack on days of strenuous
exercise
Night time hypoglycemia


Eat 1 ½ snacks if blood glucose is less than
100 to 120 mg/dL before going to bed
Make sure the blood glucose is 100 – 120
mg/dL before going to bed
Hyperglycemia or DKA
Symptoms:
Onset = gradual
Lethargic, confused, weak
Thirsty
Abdominal pain often with nausea and vomiting
Signs of dehydration
Vital signs: deep, rapid respirations, fruity acetone
breath, and weak pulses
DKA – Diabetic Ketoacidosis

Presenting symptoms may include:



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Altered level of consciousness
Dehydration
Electrolyte disturbances
Dysrhythmias
Shock
Complete vascular collapse
Diabetic Ketoacidosis



Mild
Moderate
Severe
Mild DKA




Hyperglycemia and ketonuria with an ability
to take in and retain oral fluids.
Management: increased fluid intake
Diet drinks when blood glucose > / = 240 and
supplemental insulin administration
Check urine ketone levels
Moderate DKA



Hyperglycemia, ketonuria, and acidosis (ph
between 7.25 and 7.4) associated with an
impaired ability to retain oral fluids.
Need emergency care: IV fluids (normal
saline), supplementary insulin ( regular
insulin IV)
Management of underlying medical condition:
infections, trauma
Severe DKA

Characterized by severe acidosis (ph < 7.25),
dehydration, hyperglycemia, ketosis and a
variety of other symptoms including
Kussmaul respirations, alteration in mental
status, and unconsciousness. Severe
dehydration may lead to shock.
Management of severe DKA

3 phases of management



Resuscitation
Correction of acid-base, glucose and electrolyte
abnormalities
Transition to daily routine
Resuscitation

ABC’s: securing an airway, ensuring
adequate ventilation, and correcting shock
with IV volume expanders such as normal
saline.
Phase 2 & 3

Correct acid-base:




Intravenous fluids and insulin (regular insulin IV
drip)
Administration of bicarbonate if acidosis is severe
Slowly bring down plasma glucose levels to avoid
cerebral edema
Restart child on regular routine with
emphasis on teaching and review of routine
Life Management



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Management by endocrinologist
Insulin
Blood sugar monitoring
Diet
Exercise
Screen for retinopathy: ophthalmologic exam
annually
Nutritional Management

Goals of nutritional therapy





Maintaining near-normal blood glucose by balancing food
intake with insulin and activity.
Achieving optimal serum lipid levels.
Providing appropriate calories for normal growth and
development.
Preventing and treating acute and long-term complications.
Improving overall health through optimum nutrition
Exercise




Vital component to management of child with
diabetes.
May decrease the amount of insulin required.
Enhances insulin absorption.
Important for normal growth and
development.
Management During Exercise





Eat a snack before exercising.
Exercise lasting less than 1 hour usually requires a
small snack / complex carbohydrate or protein.
Longer exercising may require more frequent
snacks / complex carbohydrates or a protein.
Insulin adjustment may be needed if hypoglycemia
occurs during the activity.
Check blood glucose after activity and before
bedtime to prevent night time hypoglycemia
Diabetes Type 2

Between 8 and 45 percent of newly diagnoses
cases of childhood diabetes are type 2

Type 2 diabetes is caused by resistance to insulin
as well as the inability of the pancreas to keep up
with the increase demand of insulin.

Insulin resistance + chronic hyperglycemia
Type 2 diabetes





85% of children are obese
Age of onset is middle to late puberty around
13 years
Minority populations have an especially high
rate of type 2 diabetes
Native American, Alaska Native, African
American and Mexican American
Strong family history
Pathophysiology


Most often associated with obesity,
hypertension, elevated cholesterol.
Characterized by peripheral insulin resistance
with a defect of insulin secretion
(hyperinsulinemia).
Assessment






Obesity: BMI greater than 30 (normal range is 15 to
17 in the pediatric population)
Waist to hip ratio: apple shape
Acanthosis nigricans: hyper-pigmentation and
thickening of the skin into velvety irregular folds in
the neck and flexural areas – reflects
hyperinsulinemia
Hypertension
+ family history of type 2 diabetes
Ethnicity
Assessment



Chronic hyperglycemia
Often diagnosed during routine physical
Girls often present with vaginal monilial
infection
Diagnostic tests


Plasma insulin and C peptide are high
reflecting insulin resistance
Autoantibodies to the islet cell are negative in
type 2
Interdisciplinary Interventions




Comprehensive education on importance of
regular exercise and how to self-monitor for
blood glucose levels.
Dietary management
Glucose-lowering agent: drugs that improve
insulin sensitivity such as Glucophage
(Metformin)
A few may need Insulin to initiate control
Diabetes Insipidus




Disorder of the posterior pituitary
It results in deficiency in the secretion of ADH
ADH concentrates urine
Deficiency result in massive renal loss of fluid
Pathophysiology



Antidiuretic hormone works directly on the
renal collection ducts and distal tubules to
increase membrane permeability for water
and urea.
A deficiency in ADH will cause failure of
kidneys to reabsorb water.
This leads to massive water loss
Causes



Hypothalamic lesion
¼ occur after craniotomy
Idiopathic or familial
CaREminder

The first symptom of central DI seen in
children, especially in infants, are irritability
and incessant crying that can only be
alleviated with feeding water. Formula or
breast milk does not quench the child’s thirst.
Assessment




Polyuria (excessive urination)
Polydipsia (excessive thirst)
Onset on symptoms abrupt
In the older child nocturia and enuresis are
common
Urine




Very low specific gravity: 1.005
Dilute
Colorless
NO glucose or ketones
Interdisciplinary Interventions


Careful monitoring of child to prevent fluid
and electrolyte inbalance
Administering Desmopressin (DDAVP):
synthetic analogue of ADH
Parent education




Administration of the medication
Signs and symptoms of fluid imbalance:
dehydration and over-hydration
Sign of hypernatremia (irritability or change in
behavior)
Wear medi-alert tag
Nursing Diagnosis



Fluid volume deficit
Desmopressin: medication used to treat
DI…over use may result in fluid volume
excess
Activity intolerance: due to dehydration,
excessive thirst and frequent urination

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