Disorders of the Adrenal Gland:
The adrenal glands - inner cortex, outer medulla

Cortex - hormones called steroids >
Glucocorticoids (cortisol metabolizes glucose, proteins, fats, involved in stress reactions and inhibition of inflamation)
Mineralocorticoids (Aldosterone maintains extracellular fluid volume, and blood pressure by conserving sodium, chloride, water, excretion of potassium)

Medulla - secretes catecholamines epinephrine and norepinephrine

*These hormones are produced by the sympathetic nervous system so when the adrenal gland is not working properly it is life threatening*

Congenital adrenal hyperplasia (CAH):
Deficiency in enzyme needed for synthesis of cortisol and sometimes aldosterone
Most common 21-hydroxylase (21-OH) 95% of all cases

Pathophysiology:
Cortisol synthesis blocked > this increases the production of ACTH (adrenocorticotrophic hormone) by the anterior pituitary.
Too much ACTH causes hyperthrophy of the adrenal glands and excess androgen production

Clinical Manifestations:
The enzyme deficiency causes the fetus to be exposed to excess androgens.
In males no effect, virilizes the female infants -
Ambiguous genitalia, pseudohermaphrodism, ovaries, fallopian tubes, uterus normal.
75% have salt wasting defect since cannot synthesize aldosterone
Leads to life threatening adrenal crisis by day 10-14 > hyponatremia, hyperkalemia, hypovolemia

Atypical 21-OH later toddler or preschool > premature adrenarche (pubic hair development) with accelerated growth, acne, Hirsutism (masculine body hair distribution)
Prenatal diagnosis possible
In newborn testing in 42 states (detect before adrenal crisis and wrong sex assignment)
Confirmation through repeated blood tests, electrolyte levels and physical exam performed immediately

Treatment:
Supression of adrenal secretion of androgens
Prevention of progressive virilization
Replacement of the steroids the adrenal gland cannot produce
Glucocorticoid hydrocortisone and mineralocorticoid fludrocortisone (Florinef)
Normal replacement has no symptoms
Too much = hypertension, acne, growth impairment
Most difficult is female with ambiguous genitalia and surgery considerations

Nursing management/ Family teaching:
Newborn maintain homeostasis with fluids, cortisone
Monitor electrolytes, vital signs
Safe environment for caregivers to ask questions
Your baby, your child, not he, she, it
Genitals, sex organs not penis or clitoris
Cortisone necessary to maintain life oral or IM
Illness, stress or surgery cortisol will be increased (stressors typically increase cotisol levels)
Sex assignment


Congenital Adrenal Hyperplasia








Cushing Disease (Adenocortical Hyperfunction)
Cushing's Disease is adding a cushion of glucocorticords, cushions are fluffy and provide excess weight
Excess glucocorticoids (especially cortisol) in the bloodstream
Caused by a malignant carcinoma or hyperplasia of the adrenal glands or a benign tumor
Mnemonic trick - Cushings = Cushion, excess weight

Clinical Manifestations/Symptoms include:
Reduced linear growth
Mental or behavioral problems
Cushingoid appearance - moon face, fat pads on the shoulders and back
( most of the time the appearance is due to consumption of steroids for other reasons)
Muscle, capillary weakness, fatigue due to catabolism of protein
Demineralization of bones, osteoporosis due to decreased absorption of calcium
Excessive weight gain due to increased appetite
Increased blood pressure and blood volume due to salt retention

Nursing Measures:
Monitor vital signs
Monitor electrolytes (calcium), fluid volume, and weight
Monitor muscle strength
Cluster care, provide rest periods
Teach family to monitor blood pressure
Nutritional counseling
Medic alert bracelet
Teach parents to recognize an adrenal crisis and or insufficiency after corticosteroids are withdrawn

*If child has had the adrenal glands removed and is on hydrocortisone (Solu-Cortef, Cortisone acetate) be sure that they understand administration pattern best mimics the body to administer early in the morning. If the child is vomiting and cannot take the medication orally give injection or could lead to severe illness or Cardiovascular collapse.

While Sharmyn is not a child her story typifies the struggles with Cushing's disease.



Chanelle's Cushing's story



Chanelle was the first to notice something different with her body.
What did she notice?
What other symptoms did she notice?
What was her treatment?
What would be important nursing considerations for her.



Addison's Disease (Adrenal Insufficiency)
Remember 'Add'ison's Disease is really a subtraction problem! Minus adequate gluco and mineralocorticords!
Deficiency of glucocorticoids, mineralocorticords (aldosterone) and adrenal androgens
This affects the bodies ability to handle stress
Caused by autoimmune reponse , metabolic disease, infection or malignancy
Develops slowly
Early signs - weakness, fatigue, emotional lability, anorexia, salt craving, poor weight gain
Hyperpigmentation
Abdominal pain, nausea, vomiting, diarrhea, hypoglycemia
After a stressful event adrenal crisis may occur

Diagnostic tests:
Cortisol and urinary 17-hydroxycorticoid measured in am
ATCH stimulation test

Treatment and Nursing Management:
Replacement of hormones
Parental education
Management of an adrenal crisis






Diabetes -
Type 1 diabetes mellitus and type 2 diabetes mellitus

Type 1 Diabetes























Incidence/etiology -
Idiopathic or autoimmune destruction of pancreatic beta cells
Genetic predisposition and environmental factors may contribute
By the time symptoms appear 90% of beta cells have been destroyed
peaks at age 2, 4-6, and again at 10-14
Equal among boys and girls
More prevalent in caucasians

Pathophysiology: Type 1
autoimmune destruction results in inadequate insulin secretion
10-20% of normal produces symptoms
Insulin is not able to help transport glucose across the cell membrane = hyperglycemia
Low insulin = hepatic glucose increase, hyperlipidemia, ketones
Growth hormone, cortisone, glucagon increase, which promote insulin resistence
Glycogenesis - glycogen from non-carbohydrate sources
Proteolysis - protein breakdown
Lipolysis - fat breakdown

Blood glucose levels > 150-180mg/dl filtered out of blood and into urine
Polyuria - glucose in urine creates a shift with additional water excreted
Polydipsia - Fluid loss creates thirst
Polyphagia - less glucose used for energy production
Ketones are acid bodies buffered by bicarbonate, as bicarb is depleted metabolic acidosis increases
Kussmaul breathing slow deep breathing with acetone breath odor

Clinical Manifestations Type 1:
Polyuria
Polyphagia
Polydipsia
weight loss
Dehydration
Occasionally, abdominal pain, vomiting
Irritability/fatigue
Diabetic ketoacidosis (DKA)

Diagnosis:
Presentation characteristics often enough, may present with 3 P's and glucose >200 mg/dl
Or in diabetic ketoacidosis (DKA)
Oral glucose tolerance test (OGTT)
Fasting serum glucose
Fasting plasma glucose preferred
A1c not recommended for diagnosis

Nursing interventions/management
If in DKA
Labs for diagnosis
Monitor vital signs, neurological status
Stabilize with fluids, insulin
Strict I&O
Monitor labs
Teach glucose monitoring, insulin administration, diet, exercise, S&S of hypo and hyperglycemia
Medic alert band
















































Type 2 Diabetes
Prevalent among children now
contributing factors food consumption patterns
lack of exercise
obesity
Higher in AA, Hispanic, Asian or American Indian descent
90% have acanthosis skin condition with hyperpigmented patches in creases

Pathophysiology Type 2 diabetes
Social, environmental, behavioral contributors
Initially insulin resistence then beta cell failure

Clinical Manifestations:
Type 2:
Body mass index over 95%
Fatigue
Frequent infections

Diagnosis for type 2 = Fasting glucose >126 or random glocose >200 mg/dl

Treatment:
Education and prevention





Obesity = BMI > 85% overweight, BMI > 95% obese
2002 - 32% of children ages 6-19 were at risk for being overweight
Higher prevalence in the Hispanic, African American, and American Indian population
Birth weight is not a future indicator of obesity
Positive correlation between childhood obesity and parental obesity
Fewer than 5% shown to be due to an underlying disease process
Causes:
Appetite regulation
Familial influence
Physical inactivity - unsafe communities
Institutional factors - vending machines
Psychological factors of comfort
Can lead to significant health problems:
High blood pressure
Elevated cholesterol,
Respiratory disorders,
Orthopedic conditions,
Gall stones,
Fatty liver,
Type 2 diabetes,
Low self esteem,
Isolation
Therapeutic Management -
Nutritional counseling
Behavioral therapy
Group involvement
Family involvement
Prevention

Disorders related to sex chromosome abnormalities:**

Turners Syndrome


May be diagnosed at birth, preschool or adolescence.
Occurs in 1/2000 female births
Missing or partially absent X chromosome

Characteristics include:
Lymphedema of hands and feet
Webbed neck
Low hair line
Low set ears
Small mandibles, resulting in need for orthodontics
Short stature
Delayed breast development, lack of spontaneous menstrual cycles
Infertile need estrogen to stimulate secondary sex characteristics
Possibly socially ackward, immature
Possible heart defects (coartation of the aorta)
Strabismus
Hip dysplasia, scoliosis

Nursing Management:
Monitoring growth rates
Observing for s/s of cardiac, musculoskeletal disorders, renal, GI or thyroid dysfunction
Treat child age appropriately, not according to size
Parental Education

Klinefelter Syndrome




Extra x chromosome in males.
One of the most common genetic syndromes, 1/500 males
Infrequent diagnosis before puberty.

Characteristics:
Infertile and may have cognitive delays.
Tall stature, less facial and body hair
Gynecomastia (breast development)
Noted when secondary sexual characteristics do not occur.
They may have difficulty with learning disabilities in school
Therapy includes hormone replacement (testosterone) beginning at 11 or 12

Nursing Considerations include:
Parental education





References:

Hockenberry, M. J. (2009). Chapter 29: The child with endocrine dysfunction. In M. J. Hockenberry & D. Wilson (Eds.), Wong's essentials of pediatric nursing (2009 ed., pp. 1023-1059). St. Louis, MO: Mosby Elsevier.

Videos:

Cares Foundation Congenital Adrenal Hyperplasia
http://youtu.be/4oRKwboWezQ

The Magic Foundation Congenital Adrenal Hyperplasia
http://youtu.be/qLoc0jrlHyo

Cushing's Disease by Sharmyn 411
http://youtu.be/ib5CGOqujSY

Cushing's Disease by Sousini17
http://youtu.be/QXAHtxis1fM

Addison's Disease by drmdk
http://youtu.be/qqgIYDKd75E

Videos:

Type 1 Diabetes by Clearly Health
http://youtu.be/_OOWhuC_9Lw

Children with Diabetes
http://youtu.be/yx1t4yZ1nQU

Nick Jonas Diabetes
http://youtu.be/hOW7FTLcFkI

Pediatric diabetes by Health Matters
http://youtu.be/Vr86FaTdp5E

Turner syndrome: located at
http://youtu.be/ldjb-FR-PKo

Klinefelters Syndrome
https://youtu.be/c9ne4Cwy9T4