The Child with Endocrine Dysfunction
Chapter 29
Disorders of Pituitary Function
• Pituitary gland is also called “Hypophysis”
• Pituitary gland is the “master gland”
• Responsible for regulating other glands
• Two portions; each with unique functions
• Anterior, Posterior
Hypopituitarism:
• Growth Hormone Deficiency
• Inhibits somatic growth
• Primary site of dysfunction appears to be in the
hypothalamus
Diagnostic Evaluation of GH Deficiency
• Family history
• Growth patterns and health history
• Definitive diagnosis bases of radioimmunoassay of plasma
GH levels
• Hand x-rays to evaluate growth potential vs. ossification
• Endocrine studies to detect deficiencies
Therapeutic Management of GH Deficiency
• Biosynthetic growth hormone (injections)
• Other hormone replacements as needed
– Thyroid extract
– Cortisone
– Testosterone or estrogens and progesterone
Prognosis
• GH replacement successful in 80% of affected
children
• Growth rate of 3.5-4 cm/yr before treatment and
increase to 8-9 cm/yr after treatment
• Response varies based on age, length of treatment,
frequency of doses, dosage, weight, and GH receptor
amount
Creutzfeldt-Jakob Disease (CJD)
• Rare neurodegenerative condition reported in some
patients after receiving human GH from cadavers
• Does not occur with biosynthetic GH
• Blood banks will not accept donation from former
human GH recipients for risk of infection with CJD
Nursing Considerations
• Family support needs
• Child’s body image
• Preparing child for daily injections
• Treatment very expensive ($20,000 - $30,000/year)
Pituitary Hyperfunction
• Excess GH before closure of epiphyseal shafts results
in overgrowth of long bones
• Reach heights of 8 feet or more
• Vertical growth + increased muscle
• Weight generally in proportion to height
Pituitary Hyperfunction: Acromegaly
• Excess GH after epiphyseal closure is called
acromegaly
• Typical facial features
Diagnostic Evaluations
• Radiologic studies
• Endocrine studies
Therapeutic Management
• Surgical treatment to remove tumor
• Radiation and radioactive implants
• Hormone replacement therapy after surgery in some
cases
Nursing Considerations
• Early identification of children with excessive growth
rates
• Early treatment for improved outcomes
• Emotional support
• Body image concerns
Precocious Puberty
• Defined as sexual development before age 9 in boys or
before age 8 in girls
• Occurs more frequently in girls
• Potential causes
– Disorder of gonads, adrenal glands, or
hypothalamic-pituitary gonadal axis
– No causative factor in 80%-90% of girls
and 50% boys
Types of Precocious Puberty
• True or complete precocious puberty
• Precocious pseudopuberty
• Incomplete puberty
Therapeutic Management
• Treatment of specific cause if known
• May be treated with Lupron
– Slows prepubertal growth to normal rates
– Treatment is discontinued at age for normal
pubertal changes to resume
• Psychologic support for child and family
Diabetes Insipidus (DI)
• The principle disorder of the posterior pituitary
• Results from hyposecretion of ADH
• Produces uncontrolled diuresis
The Child with Endocrine Dysfunction

• Primary causes: familial or idiopathic • Myxedematous skin changes

• Secondary causes: trauma, tumors, CNS infection, – Dry skin
aneurysm – Sparse hair
Clinical Manifestations of DI – Periorbital edema
• Cardinal signs: polyuria and polydipsia Therapeutic Management
• First sign is often enuresis • Oral thyroid hormone replacement
• Infants: Irritability relieved with feedings of water but • Prompt treatment needed for brain growth in infant
not milk; dehydration often occurs
• May administer in increasing amounts over 4-8 weeks
Therapeutic Management of DI to reach euthyroidism
• Instruct parents in difference between diabetes
insipidus and diabetes mellitus • Compliance with medication regimen is crucial
• Daily hormone replacement of vasopressin Goiter
• Hypertrophy of the thyroid gland
• Drug of choice: DDAVP
• Congenital
– Nasal spray or IV administration
– Usually results from maternal ingestion of
– Requires treatment for life antithyroid drugs during pregnancy
Syndrome of Inappropriate Antidiuretic Hormone: • Acquired
SIADH
• Produced by hypersecretion of the posterior pituitary – Result of neoplasm, inflammatory disease,
(increased ADH) dietary deficiency (but rarely in children),
or increased secretion of pituitary
• S/S: Fluid retention and hypotonicity thyrotropic hormone
• Kidneys unable to reabsorb water Nursing Considerations
• Anorexia, nausea/vomiting, irritability, personality • Thyroid enlargement at birth may compromise
changes newborn’s airway
• Symptoms disappear when ADH is decreased • May become noticeable during periods of rapid
Nursing Management of DI growth
• Accurate I&O • Large goiters may be obvious, smaller nodules evident
only on palpation
• Observe for signs of fluid overload
• TH replacement necessary for treatment of
• Seizure precautions hypothyroidism
• Administer ADH-antagonizing meds Lymphocytic Thyroiditis
Disorders of Thyroid Function • Hashimoto disease or juvenile autoimmune thyroiditis
• Thyroid hormone regulates BMR • Most common cause of thyroid disease in children and
• Thyroid secretes two types of hormones teens
– Thyroid hormone, which is made up of • Accounts for largest percent of juvenile
hypothyroidism
• Thyroxin (T )
4 Pathophysiology of
Triiodothyronine (T ) Lymphocytic Thyroiditis
• 3
• Genetic predisposition but specifics unclear
– Thyrocalcitonin
• Characterized by lymphocytic infiltration of the gland,
• May have hypothyroidism or hyperthyroidism inflammation, hyperplasia, which may be replaced
• May have disturbance in secretion of TSH with fibrous tissue
Juvenile Hypothyroidism • Child usually is euthyroid, with some symptoms of
hyperthyroidism
• Congenital
Therapeutic Management of Lymphocytic Thyroiditis
– Congenital hypoplastic thyroid gland
• Goiter may be transient, asymptomatic
• Acquired
• May resolve spontaneously within 1-2 yrs
– Partial or complete thyroidectomy for CA
or thyrotoxicosis • Oral TH often decreases the goiter significantly
– Following radiation for Hodgkin or other • Surgery contraindicated for this disorder
malignancy Hyperthyroidism (Graves' Disease)
• Rarely occurs from dietary insufficiency in US • Most common cause of hyperthyroidism in childhood
Clinical Manifestations is Graves' disease
of Juvenile Hypothyroidism • Believe caused by serum thyroid-stimulating
immunoglobulin, but no specific etiology
• Decelerated growth
• Enlarged thyroid gland and exophthalmos
• Constipation
• Peak incidence 12-14 yrs age, but may be present at
• Sleepiness birth
The Child with Endocrine Dysfunction

• Familial association Hyperparathyroidism

Graves' Disease
• Manifestations develop gradually, often over 6-12
months
• Diagnosis based on increased levels of T4 and T3 with
suppressed TSH
Graves' Disease Management
• Therapy is controversial
• Goal of therapy: to retard rate of hormone secretion
• Treatments
– Antithyroid drugs (PTU and methimazole)
– Subtotal thyroidectomy
– Ablation with radioiodine
Thyrotoxicosis
• Thyroid “crisis” or “storm”
• May occur from sudden release of hormone
• Unusual in children, but can be life threatening
• May be precipitated by infection, surgery, or
discontinuation of antithyroid therapy
• Treatment of thyroid storm
– Antithyroid drugs
– Propranolol
Nursing Considerations
• Identify children with hypothyroidism
• Alert for signs and symptoms
• Child needs quiet environment, rest periods
• Help family cope with emotional lability associated
with disorder
• Dietary requirements to meet child’s increased
metabolic rate
• Medications: side effects
Disorders of Parathyroid Function
• Parathyroid glands secrete parathormone (PTH)
• Function of PTH: to maintain serum calcium by
– Increasing release of calcium and
phosphate from bone demineralization
– Increase absorption of calcium and
excretion of phosphate by the kidneys
– Promote calcium absorption in GI tract
Hypoparathyroidism
• Autoimmune hypoparathyroidism
– Deficient production of PTH
• Pseudohypoparathyroidism
– Production of PTH is increased but end
organs are unresponsive to the hormone
– Thought to be inherited as X-linked
dominant trait with variable expressivity
Clinical Manifestations
• Dry, scaly skin with eruptions
• Brittle hair, thin nails with transverse grooves
• Tetany, paresthesias, tingling, laryngeal stridor, and/or
spasms
• Headache, seizures, emotional lability, depression,
confusion, memory loss
• Positive Chvostek and/or Trousseau signs
• Primary: adenoma of the gland
• Secondary: chronic renal disease, congenital
anomalies of urinary tract
• Common factor is hypercalcemia
• Rare in children
Hyperparathyroidism
• Diagnostic evaluation
• Therapeutic management: surgical removal or treat
underlying cause if possible
• Nursing consideration
Disorders of Adrenal Function
• Adrenal cortex secretes three groups of “steroids”
– Glucocorticoids (cortisol, corticosterone)
– Mineralocorticoids (aldosterone)
– Sex steroids (androgens, estrogens, and
progestins)
• Altered levels of these produces significant
dysfunction
Disorders of Adrenal Function
• Adrenal medulla secretes catecholamines: epinephrine
and norepinephrine
• Catecholamine-secreting tumors are the primary cause
of adrenal medullary hyperfunction
Acute Adrenocortical Insufficiency
• “Adrenal crisis”
• Etiologic factors: hemorrhage into the gland from
trauma, fulminating infections, abrupt withdrawal of
exogenous cortisone, failure to increase cortisone
during times of stress
• Diagnosis generally based on clinical symptoms
Acute Adrenocortical Insufficiency
• Clinical symptoms
• Therapeutic management
• Nursing considerations
Chronic Adrenocortical Insufficiency: Addison Disease
• Rare in children
• When it occurs, usually result of neoplams or lesion of
adrenal glands or idiopathic cause
• Symptoms appear gradually after 90% of adrenal
tissue is nonfunctional
Addison Disease
• Diagnosis
• Therapeutic management
• Nursing considerations
Cushing Syndrome
• A characteristic group of manifestations caused by
excessive circulating free cortisol
• May be caused by excessive or prolonged steroid
therapy
• Condition is reversible once steroids are discontinued
• Abrupt withdrawal of steroids may precipitate acute
adrenal insufficiency
Etiologies of Cushing Syndrome
– Pituitary: excess of ACTH
The Child with Endocrine Dysfunction

– Adrenal: hypersecretion of glucocorticoids • Function of Islets of Langerhans

– Ectopic: extrapituitary neoplasm – Alpha cells produce glucagon
– Iatrogenic: administration of excessive – Beta cells produce insulin
steroids – Delta cells produce somatostatin (believed
– Food dependent: inappropriate adrenal to regulate insulin and glucagon)
response to secretion of polypeptide Diabetes Mellitus (DM)
Cushingoid Appearance • Characterized by a total or partial deficiency of the
• Excessive hair growth hormone insulin
• Moon face, red cheeks • The most common endocrine disorder of childhood
• Weight gain • Peak incidence in early adolescence
• Pendulous abdomen with red striae Three Major Groups of DM
• Poor wound healing • Type 1
• Ecchymosis • Type 2
Diagnostic Evaluation • Maturity onset diabetes of the young (MODY)
• Confirm excess cortisol levels Type 1 Diabetes
• X-rays: evaluate for osteoporosis and skull films to • Characterized by destruction of beta cells, usually
look for enlargement of sella turcica leading to absolute insulin deficiency. Typically, onset
• Laboratory tests in childhood and adolescence, but can occur at any
– Fasting blood glucose age.
– Serum electrolytes • Most DM of childhood is Type 1.
– 24 hr urine Type 2 Diabetes
Cushing Syndrome • Arises because of insulin resistance
• Therapeutic management • Onset usually after age 40
– Surgery • Native American, Hispanic, and African-American
children are at increased risk of Type 2 DM
– Replacement of growth hormone, ADH,
TH, gonadotropins, and steroids • Affected persons may or may not require insulin
injections
• Nursing considerations
MODY
Hyperaldosteronism
• Transmitted as autosomal-dominant disorder with
• May be caused by adrenal tumor formation of structurally abnormal insulin with
• May be caused by adrenogenital syndromes decreased biologic activity
• Results in hypertension, hypokalemia, and polyuria • Onset is generally before age 25
that fails to respond to ADH administration Etiology
Hyperaldosteronism (cont’d) • Type 1 DM believed to be autoimmune disease arising
• Therapeutic management when a person with a genetic predisposition is
exposed to a precipitating event such as viral infection
– Replacement of K
• Heredity is prominent factor in etiology
– Administration of spironolactone to block
effects of aldosterone Pathophysiology of DM
• Nursing considerations • With a deficiency of insulin, glucose is unable to enter
the cell, and remains in blood, causing hyperglycemia
– Alert for signs/symptoms of hypokalemia
and hyperkalemia • When serum glucose exceeds renal threshold, glucose
spills into urine (glycosuria)
Pheochromocytoma
• Adrenal tumor that secretes catecholamines • Cells break down protein for conversion to glucose by
the liver (glucogenesis)
• May occur around adrenal medulla, along paraganglia
of aorta, or thoracolumbar sympathetic chain Ketoacidosis
• Children often have bilateral, multiple, benign tumors • When glucose is unavailable for cellular metabolism,
the body breaks down alternate sources of energy.
• Increased production of catecholamines; may mimic Ketones are released, and excess ketones are
other disorders
eliminated in urine (ketonuria) or by the lungs
Therapeutic Management (acetone breath).
of Pheochromocytoma
• Ketones in the blood are strong acids that lower serum
• Surgical removal of tumor(s) pH and produce ketoacidosis.
• May require bilateral adrenalectomy and lifelong Kussmaul Respirations
glucocorticoid and mineralcorticoid therapy
• Hyperventilation characteristic of metabolic acidosis,
• Nursing considerations resulting from respiratory system’s attempt to
Pancreatic Hormone Function eliminate excess CO by increased depth and rate
2
The Child with Endocrine Dysfunction

Diabetic Ketoacidosis: DKA • Risk for related to hypoglycemia

• Pediatric emergency
• Results from progressive deterioration with • Knowledge deficit (diabetes management) related to
dehydration, electrolyte imbalance, acidosis, coma, care of child with newly diagnosed diabetes mellitus
and may cause death
Long-Term Complications of DM
• Microvascular complications, especially nephropathy
and retinopathy
• Macrovascular disease, neuropathy
Maturity-Onset Diabetes
of the Young (MODY)
• Similar to Type 2 DM
• May be seen in obese teens
• May be controlled with oral hypoglycemic agents and
diet modifications
• More benign disease, but increasing in frequency in
pediatric population
Therapeutic Management
of Type 1 DM
• Insulin therapy
• Glucose monitoring: goal range 80-120 mg/dl
• Lab measurement of hemoglobin A ⅽ
1
• Urine testing for ketones
– Not routinely used EXCEPT:
– Helpful to test q3h during illness and
whenever glucose is ≥240 mg/dl when
illness not present
Therapeutic Management
of Type 1 DM (cont’d)
• Nutrition
• Exercise
• Teach patient and family how to manage
hypoglycemic episodes
• Illness management
• Management of DKA
Patient Education:
DM and Insulin Therapy
• Nature of the disease
• Meal planning
• Insulin therapy: types of insulin, duration, onset and
peak action, mixing and administration of types of
insulin, rotation of injection sites
• Insulin pump therapy in some cases
• Glucose monitoring
Patient Education (cont’d)
• Recognition and treatment of hypoglycemia and
hyperglycemia
• Management of “minor” illnesses
• Record keeping
• Hygiene
• Family support
• Acute care
Possible Nursing Diagnoses for Pediatric Client with
DM
• Risk for injury related to insulin deficiency