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Incidence of 1 in 4000; Screening measures levels of thryoid simulating hormone
Congenital Hypothyroidism
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Incidence 1 in 13,000; deficiency in phenylalanine hyroxylase
Phenylketonuria
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S/S of congenital hypothyroidism
Poor feeding, lethargy
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S/S of PKU
vomiting, irritability, hyperactivity
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incidence of 1 in 30,000; absencse of galactose-phosphate uridyl transferase; S/S cataracts, weight loss; treat with lactase formula
Galactosemia
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PKU must be tested after ingestion of what?
Protein
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What signifies a positive Guthrie test for PKU?
>4mg/dl
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Treatment for PKU includes:
Restrict dietary protein, keep phenylalanine in a safe range (2-8 mg/dl); begin diet before 3 weeks of age; maintain low protein diet for life
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What is the master gland that is responsible fo regulating other glands?
Pituitary gland
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The anterior pituitary gland produces what hormones?
GH, sex hormones, TSH
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The posterior pituitary gland produces what hormones?
ADH, oxytocin
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Pituitary gland is controlled by hormones that are produced by the ___
hypothalamus
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Inhibits somatic growth; primary site of dysfunction is hypothalamus; most common causes is pituitary tumors/hypothalamic tumors
Growth Hormone Deficiency
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What are some clinical manifestations of GH deficiency?
Appears younger than age; bone age is retarded; permanent teeth are delayed; sexual development is delayed
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Daily treatment of ___ to ___ ug/kg of biosynthetic growth hormones is needed in GH deficiency until acceptable height is achieved
25 to 50
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Nursing considerations for hormone replacement therapy for GH deficiency includes: (3)
administer at bedtime; administer 6 of 7 days; encourage self management in school age children
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Excessive growth hormone before closure of the epiphyseal shafts results in:
overgrowth of long bones
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excess GH after epiphyseal closure is ___
acromegaly
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Typical facial features of acromegaly
Separation, malocclusion of teeth, prominent forehead, increased facial hair
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Therapeutic management of acromegaly (3)
Surgery to remove tumor, radiation, hormone replacement therapy after surgery
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Nursing considerations for acromegaly (4)
identify children with excessive growth rates early; early treatment; emotional support; body image concerns
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Sexual developments before age 9 in boys/8 in girls
Precocious Puberty
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Isolated appearance of breast development
Premature thelarche
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Early appearance of pubic hair
Premature pubarche
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What does the medication Lupron treat?
Peripheral Precocious Pseudopuberty; slows prepubertal growth to normal rates; discontinue at puberty!!
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the principal disorder of the posterior pituitary
Diabetes Insipidus (DI)
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Results from hyposecretion of ADH; produces uncontrolled diuresis
DI
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Secondary causes of DI includes (4)
trauma, tumors, CNS infection, aneurysm
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Some clinical manifestations of DI include: (7)
Polyuria, polydipsia, enuresis (usually 1st sign), irritability, dehydration, hypernatremia, sometimes fever
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DI needs a daily hormone replacement of ___
Vasopressin
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Drug of choice for DI
DDAVP
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Nursing considerations for DI: (5)
Identify disorder, I&O, observe for s/s of fluid overload, seizure precautions, administer ADH antagonizing meds
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SIADH stands for:
Syndrome of Inappropriate Antidiuretic Hormone
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produced by hypersecretion of the posterior pituitary (increased ADH)
SIADH
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S/S of SIADH (5)
Fluid retention, hypotonicity, decreased sodium, anorexia, iriitability, personality changes
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Treatment of SIADH (3)
Fluid restriction, Seizure precautions, long term ADH antagonizing medications
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Thyroid hormone regulates what?
BMR
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Thyroid hormone secretes what 2 hormones?
Thryoid hormone (T4/T3) and thyrocalcitonin (which maintains blood calcium levels)
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What hormone stimulates the thyroid?
TSH
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Most commonly results from agenesis, dysplasia, ectopy of thyroid; can also becaused by autosomal recessive defects
Juvenile Hypothyroidism
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Clinical Manifestations of Juvenile Hypothyroidism (9)
Decelerated growth, constipation, sleepiness, skin changes, elevated weight, bradycardia, large fontanelles/tongue, delayed dentition, goiter development
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Hypertrophy of the thyroid gland
Goiter
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(Congenital) usually results from maternal ingestion of antithyroid drugs during pregnancy
Goiter
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(acquired) result of neoplasm, inflammatory disease, dietary deficiency, increased secretion of pititary thyrotropic hormone
Goiter
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Therapeutic management for goiter (4)
oral thyroid hormone replacement, prompt treatment for brain growth in infant, administer in increasing amounts over 4-8 weeks, compliance
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Nursing considerations for Goiter (4)
Thyroid enlargement may compromise airway in infant; may be noticable during periods of rapid growth; large goiters may be obvious upon palpation; TH replacement is necessary!
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most common cuase of hyperthyroidism in childhood is what?
Graves disease
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Peak incidence of hyperthyroidism is between ___ and ___ years old
12 to 14
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Some clinical manifestations of Grave's disease: (8)
Exophthalmos, weight loss, increased appetite, nocturia, palpitations, irritability, sleeplessness, inability to concentrate
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PTU specifically inhibits the peripheral conversion of ___ to ___
T4 to T3
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Transmitted as autosomal dominant disorder with formation of structurally abnormal insulin with decreased biologic activity
Maturity Onset Diabetes of the Young
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The first symptom of DM is usually:
Nocturnal enuresis
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S/S of hypoglycemia (3)
Dizziness, confused, shaky
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acute metabolic changes in the body due to lack of insulin; dehydration, acidosis, hyperglycemia
Ketoacidosis
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Hyperventilation characteristic of metabolic acidosis resulting from respiratory system's attempt to eliminate CO2 (in DKA)
Kussaul Respirations
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Rapid acting insulin; what is the onset, peak, and duration?
Insulin lispro (Humalog); onset is 10 to 30 mins, peak is 1-2 hours, duration 2-4 hours; give 15 mins before/after a meal
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Short acting insulin; onset, peak, duration?
Regular insulin (Humulin R, Novolin R); onset 0.25 to 1 hr; peak is 1.5 to 4 h; duration 5 to 9 hr
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Intermediate acting insulin; onset, peak, duration?
NPH (Humulin N, Novolin N); onset 3-4 hrs; peak 8-14 hrs; duration 16-24 hrs
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The adrenal cortex secretes 3 groups of what steriods?
Glucocorticoids (cortisol, corticosterone); Mineralocorticoids (aldosterone); sex steroids (androgens, estrogens, progestins)
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Adrenal medulla secretes what? (2)
Epinephrine, norepinephrine
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What kind of tumors are the primary cause of adrenal medullary hyperfunction?
Catecholamine-secreting tumors
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"Adrenal crisis"; caused by hemorrhage from trauma, fulminating infections, abrupt withdrawal from exogenous cortisone, failure to increase cortisone in times of stress
Acute adrenocortical insufficiency
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Symptoms of Acute adrenocirtical insufficiency in infants includes: (4)
High temp, tachypnea, cyanosis, seizures
-
S/s of acute adrenocoritcal insufficiency in children: (5)
irritability, headache, abdominal pain, weakness, diarrhea
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nursing considerations for acute adrenocortical insufficiency (6)
Recognize symptoms promptly, monitor vital signs, seizure precautions, monitor fluid therapy/electrolytes, support parents
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Rare in children; usually occurs as a result of neoplasm, lesion of adrenal glands, idiopathic cuase
chronic adrenocortical insufficiency
-
Side effects of this medication are gastric irritation, irritability, sleeplessness, weight gain, depression, euphoria
Glucocorticoids (medication for chronic adrenocortical insufficiency)
-
Side effects of this medication includes generalized edema, hypertension, headache, cardiac arrhythmias, hypokalemia
Mineralcorticoids (used for chronic adrenocortical insufficiency)
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Characteristic group of manifestations caused by excessive circulating free cortisol; may be caused by excessive/prolonged steroid therapy
Cushing syndrome
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A person with this appearance has excessive hair growth, moon face, red checks, weight gain, pendulous abdomen with red striae, poor wound healing, ecchymoses
Cushingoid appearance
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Post op complications of Cushing syndrome: (3)
anorexia, muscle/joint pain, depression
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Therapeutic management of cushing syndrome (2)
Bilateral adrenalectomy, replacement of GH, ADH, TH, gonadotropins, steroids
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