Endocrine/Metabolic Alterations

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Endocrine/Metabolic Alterations

• NUR 264
• Pediatrics
• Angela Jackson, RN, MSN

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Developmental Differences

• The endocrine system is incompletely developed at
  birth
• Less mature than any other body system
• Pituitary gland is formed by the 4th month of
  gestation and measurable amounts of hormone can
  be detected
• Newborns level of TSH is 10 times higher than
  levels seen in older children. Initial thyroid
  function tests cannot be interpreted using normal
  standards of childhood or adults

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Endocrine Glands

• Anterior pituitary
• Posterior pituitary
• Thyroid
• Parathyroid
• Adrenal cortex
• Adrenal medulla
• Ovaries
• Testes
• Pancreas

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Growth Hormone Deficiency

• Characterized by poor growth and short stature
• Occurs equally in both sexes
• May result from injury, destruction of the
  anterior pituitary gland by a brain tumor,
  infection, or irradiation, but is usually
  idiopathic

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GHD Pathophysiology

• Hypothalamus secretes growth hormone-releasing
  hormone (GRH)
• Production of growth hormone (GH) by the
  pituitary is stimulated
• In GHD, the pituitary is unable to respond to the
  GRH, and GH is not produced

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GHD Clinical Manifestations

• Short stature
• Deteriorating or absent rate of growth
• Higher weight to height ration
• Delayed bone age (Determined by x-ray of the hand
  and wrist)
• Increased fat in trunk area
• Childlike face with a large, prominent forehead
• High-pitched voice
• Hypoglycemia
• Micropenis and small testes in males
• Delayed sexual maturation
• Delayed dentition

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GHD Diagnosis

• Family history
• Review of previous growth records
• Physical examination
• Determination of growth rate
• Radiographic bone studies
• Baseline blood testing
• Pituitary function testing

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GHD Treatment

• Goal of treatment is to promote normal growth
  rates by administration of growth hormone
• Growth hormone is given IM or SC
• Treatment is discontinued once the epiphyseal
  growth plates have fused
• Treatment is expensive (20,000 to 30,000 /
  year, depending on dosage)

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GHD Potential Complications of Treatment

• Slipped femoral epiphysis
• Pseudotumor cerebri
• Edema
• Sodium retention

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GHD Nursing Management

• Monitor growth
• Maintain growth chart
• Provide teaching to family concerning normal
  growth and development
• Teach family proper medication administration
  techniques and side effects
• Monitor medication dosages
• Provide emotional support

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Precocious Puberty

• Breast development before the age of 7 in
  Caucasian girls and before the age of 6 in
  African-American girls
• Development of secondary sex characteristics in
  boys less than 9 years old
• Five times more common in girls
• Idiopathic in girls, related to central nervous
  system abnormalities in boys

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Precocious Puberty Pathophysiology

• Results from premature activation of the
  hypothalamic-pituitary-gonadal axis
• Hypothalamus secretes gonadatrophin releasing
  hormone, which stimulates the pituitary to
  produce leutinizing hormone and follicle
  stimulating hormone. Estrogen or testosterone is
  also produced

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Precious Puberty Clinical Manifestations

• Accelerated growth rate
• Advanced bone age
• Secondary sex characteristics
• Acne
• Body odor
• May be emotionally labile, aggressive, and mood
  swings may occur
• Potentially fertile

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Precocious Puberty Diagnosis

• Physical exam and history
• Tanner staging
• Measurement of height and weight
• X-rays for bone age, pelvic ultrasound for
  females to identify size of uterus and ovaries,
  CT, MRI or skull film to detect CNS lesions for
  males
• Lab tests for LH, FSH, estradiol or testosterone
• GnRH stimulation testing

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Precocious Puberty Treatment

• Administration of luteinizing hormone releasing
  hormone (Lupron) SC on a monthly basis
• Surgery, radiation or chemotherapy if caused by
  CNS tumor
• Treatment results in a decrease in growth rate,
  stabilization or regression of secondary sex
  characteristics
• Puberty resumes when therapy is discontinued

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Precocious Puberty Nursing Management

• Monitor growth
• Provide psychological support
• Teach parents about normal growth and development
• Instruct parents that childs mental age is
  congruent with chronologic age
• Teach parents about medication administration and
  potential side effects

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Diabetes Insipidus (DI)

• Disorder of water regulation
• Deficiency of ADH results in excretion of large
  amounts of dilute urine
• Most often seen as a complication following head
  injury or cranial surgery to remove tumors of the
  hypothalamic-pituitary region
• Other causes include vascular anomalies,
  infection, and genetic defect

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DI Clinical Manifestations

• Polyuria
• Polydipsia
• Nocturnal enuresis
• Urine output can range from a few liters to
  eighteen liters a day
• Urine specific gravity is 1.005 or less, urine
  osmolarity is lt200mmol/l
• Serum sodium concentration and plasma osmolarity
  are elevated

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DI Diagnosis

• UA for osmolarity, specific gravity, and sodium
• Serum osmolarity, sodium and creatinine levels
• Water deprivation test. Requires several hours to
  complete with close monitoring (IO, weight,
  vital signs, hydration assessment, and urine and
  blood samples)

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DI Treatment

• Goals of treatment include antidiuresis,
  uninterrupted sleep, and increased ability to
  participate in school and other programs
• Treated with daily replacement of ADH
• Drug of choice is DDAVP, which is given
  intranasally or orally

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DI Nursing Management

• Strict IO and daily weight
• Teach parents about the condition
• Teach parents about lifelong need for medication
  and medication administration
• Teach parents to monitor IO and daily weights

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Congenital Hypothyroidism (CH)

• Present at birth
• Reduced rate of metabolism caused by a low
  concentration of circulation thyroid hormones (T3
  and T4)
• More females than males are affected
• Caused by a defect in the embryonic development
  of the thyroid gland, inborn error of thyroid
  hormone synthesis, and pituitary dysfunction
• Thyroid gland is unable to produce T3 and T4 in
  response to increasing elevated levels of TSH
  secreted by the pituitary gland

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CH Clinical Manifestations

• Asymptomatic at birth
• Large posterior fontanel
• Umbilical hernia
• Constipation
• Prolonged jaundice
• Pallor hypothermia

• Enlarged tongue
• Hypotonia, hypoactivity
• Feeding difficulties
• Delayed mental responsiveness
• Cool, dry, scaly skin
• Swollen eyelids

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CH Diagnosis

• Mandatory newborn screening
• Low T4 and a high TSH indicate CH
• Thyroid scan to evaluate for absence or ectopic
  placement of the thyroid gland

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CH Treatment

• Goal of therapy is to quickly normalize thyroid
  function
• Maintain the level of T4 in the upper half of the
  normal range and TSH in the normal range
• Thyroid replacement with synthroid is initiated
  as soon as possible, starting dose of 10-15
  mcg/kg/day
• Close monitoring of thyroid function
• Lifelong replacement is necessary to maintain
  normal metabolism

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CH Nursing Management

• Monitor growth and development
• Monitor lab values every 2-4 weeks until thyroid
  function is within target range and medication
  dose is stabilized, every 3-4 months for first
  several years of life, every 6-12 months in
  adolescence
• Teaching parents proper medication
  administration, side effects, importance of
  continuing medication for rest of child's life
  and importance of regular blood tests to monitor
  thyroid function

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Questions??