Chapter 51: The Child with an Endocrine or Metabolic Alteration Flashcards

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1
Q

Manifestations include irritability, tachycardia, increased appetite without weight gain, prominent eyes, hypertension

A

neonatal Graves disease

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2
Q

Thyroxine (T4) and TSH levels vary with _______

A

age

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3
Q

An infant with a _____ T4 and a TSH value exceeding _______ mU/mL is considered to have primary/congenital hypothyroidism.

A

low, 40

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4
Q

In phenylalanine hydroxylase deficiency (PAH), metabolic alterations usually start _____________ but may not be noticed until approximately _____________.

A

immediately, 3 months

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5
Q

A water deprivation test is stopped immediately if the child loses ___% or more from baseline weight, has intolerable ___________, shows signs of ____________, or has significant ________________ changes or _____________ status change.

A

5, thirst, dehydration, vital sign, neurologic

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6
Q

metabolic disorder that results in an abnormal buildup of gangliosides on neurons

A

Tay-Sachs disease

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7
Q

In diagnosing precocious puberty, __________________ can locate tumors or cysts in adrenal gland or ovaries and can show signs of puberty changes in ovaries and uterus.

A

pelvic ultrasound

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8
Q

hormone that stimulates cell growth and reproduction

A

growth hormone (GH)

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9
Q

While treating a child with congenital hypothyroidism, parents should be educated about manifestations of ________________

A

hyperthyroidism

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10
Q

In acquired hypothyroidism, the body produces autoantibodies called _________________ that bind to receptor sites in thyroid and decrease production of _______ and _________.

A

TSH receptor blockers, T3, T4

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11
Q

third-line (final) treatment of Graves disease

A

subtotal or partial thyroidectomy

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12
Q

Graves disease is caused by autoantibodies called ________________ which stimulate the thyroid to produce T3 and T4

A

thyroid stimulating immunoglobulin (TSIs)

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13
Q

oral radioactive iodine is used in children older than ___________

A

10 years

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14
Q

GnRH blockers for precocious puberty are often administered by __________ every _________ or a ______________ implantation every ________.

A

IM injection, 1-3 months, subcutaneous, 1-2 years

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15
Q

Preschoolers are at an increased risk for hypoglycemia due to increased _____________ levels

A

energy

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16
Q

Treatment of precocious puberty is aimed at _____________________ or ______________ puberty development, involving _______________

A

stopping, reversing, GnRH blockers

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17
Q

Hormonal release is controlled by the _____________

A

circadian rhythm

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18
Q

growth hormone deficiency is defined as consistently poor growth (less than __ cm per year)

A

5

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19
Q

While GH deficiency can be idiopathic, it can also be associated with _____________, malformation of the ____________, brain ___________, and cranial _____________.

A

hypopituitarism, pituitary, tumors, radiation

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20
Q

Mild/early symptoms of hyponatremia include _______________, ________________, _____________, and ________________.

A

anorexia, headache, nausea, vomiting

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21
Q

stress hormone that stimulates the release of cortisol

A

adrenocorticotropic hormone (ACTH)

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22
Q

Injury to the parathyroid gland can result in _______________.

A

hypocalcemia

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23
Q

most common cause of syndrome of inappropriate antidiuretic hormone (SIADH) is inappropriate use of ____________ in treatment of diabetes insipidus (DI)

A

vasopressin

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24
Q

Manifestations for growth hormone deficiency include height below ____ percentile for age and sex, diminished growth rate (__ standard deviations below mean), hypoglycemia, delayed puberty, diminished muscle mass with increased body mass, and micropenis.

A

5th, 2

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25
Q

first-line treatment of Graves disease

A

medication: methimazole

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26
Q

type of diabetes insipidus (DI) in which the kidneys are insensitive to vasopressin

A

nephrogenic diabetes insipidus (DI)

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27
Q

Manifestations include goiter, increased appetite, weight loss, nervousness, heat intolerance, diarrhea, muscle weakness, increased heart rate, palpitations, tremors, exophthalmos, poor attention span, and behavior problems

A

hyperthyroidism or Graves disease

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28
Q

In a water deprivation test, normally, urine output _________ and specific gravity _____________. In a patient with diabetes insipidus (DI), large amounts of ______________ are produced, and serum ____________ level increases.

A

decreases, increases, dilute urine, sodium

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29
Q

The endocrine system is (more, less) developed at birth than any other system

A

less

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30
Q

methimazole works by _____________________

A

blocking thyroid hormone production

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31
Q

Lab studies for syndrome of inappropriate antidiuretic hormone (SIADH) may show hypo_______, hypo________, and low serum ____________.

A

natremia, chloremia, osmolality

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32
Q

The treatment PLAN for congenital adrenal hyperplasia (CAH) includes frequent _____________________ and ___________ plan that usually involves a __________ or ____________ dose of ________________ replacement.

A

endocrinologist reevaluations, sick-day, doubled, triple, glucocorticoid

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33
Q

In diagnosing precocious puberty, _____ and ______ can identify any pituitary gland tumors

A

CTs, MRIs

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34
Q

hormone that stimulates thyroid to release hormones to regulate energy use

A

thyroid stimulating hormone (TSH)

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35
Q

The medication ____________ can be given to some individuals with phenylalanine hydroxylase deficiency (PAH) to lower the circulating levels of phenylalanine (PHE).

A

sapropterin (KUVAN)

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36
Q

Neonatal Graves is caused by maternal transfer of _________________ across placenta to developing fetus

A

thyroid stimulating immunoglobulin (TSIs)

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37
Q

Phenylalanine hydroxylase deficiency (PAH) is tested within first _______ after birth, but the test is not ___________.

A

24 hours, diagnostic

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38
Q

Hyperthyroidism or Graves disease is more common in (boys, girls) with peak age of diagnosis between ____________ of age.

A

girls, 10-14 years

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39
Q

phase marked by lowered or normal BG levels and decreased need for insulin

A

honeymoon phase

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40
Q

deficiency in enzyme that converts galactose to glucose during digestion

A

galactosemia

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41
Q

most common form of diabetes insipidus (DI)

A

central diabetes insipidus (DI)

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42
Q

gonadotropin hormone that stimulates ovulation in females and production of testosterone in males

A

luteinizing hormone (LH)

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43
Q

May have no signs and symptoms or may present with skin mottling, large anterior fontanel, large tongue, distended abdomen, slow reflexes, or hypotonia

A

congenital hypothyroidism

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44
Q

other causes include infections, trauma, tumors, generalized seizures

A

syndrome of inappropriate antidiuretic hormone (SIADH)

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45
Q

_____________ precautions should be used when serum sodium level is less than 125 while treating syndrome of inappropriate antidiuretic hormone (SIADH)

A

Seizure

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46
Q

second-line treatment for Graves disease

A

oral radioactive iodine

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47
Q

very rare disorder that negatively affects metabolism of certain amino acids

A

maple syrup urine disease

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48
Q

Children with phenylalanine hydroxylase deficiency (PAH) should have lifelong monitoring of ________________ levels, and women who have phenylalanine hydroxylase deficiency (PAH) should have _____________________ before becoming pregnant.

A

phenylalanine (PHE), genetic counseling

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49
Q

Doses of DDAVP for diabetes insipidus (DI) should be timed so the child has a mild increase in ___________ to prevent _______________ and water _____________.

A

urination, overtreatment, retention

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50
Q

When treating Graves disease with oral radioactive iodine, results are seen in ______________ after treatment

A

6-18 weeks

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51
Q

While manifestations of hyperthyroidism or Graves disease are self limiting, cardiac ________ and _________ can occur if poorly treated or unrecognized.

A

failure, death

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52
Q

In treating syndrome of inappropriate antidiuretic hormone (SIADH) during period of severe hyponatremia, (fast, slow) IV infusions may be needed to avoid CNS damage.

A

slow

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53
Q

A late sign and long-term consequence of phenylalanine hydroxylase deficiency (PAH) is ______________ which results in __________________.

A

CNS damage, cognitive impairment

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54
Q

Growth hormone is often given _____ as a __________ before bedtime.

A

daily, subcutaneous injection

55
Q

Diagnosis of diabetes insipidus (DI) often involves a water deprivation test, where the child is deprived of all fluid intake for __________.

A

7-8 hours

56
Q

gold standard for officially diagnosing congenital adrenal hyperplasia (CAH)

A

corticotropin stimulation test

57
Q

Elevated ____ and ____ levels with suppressed _____ levels are highly suggestive of Graves disease.

A

T3, T4, TSH

58
Q

honeymoon phase can last __________

A

7 months

59
Q

gonadotropin hormone that regulates growth and puberty maturation

A

follicle stimulating hormone (FSH)

60
Q

most common pediatric endocrine disorder

A

type 1 diabetes

61
Q

inadequate secretion of human growth hormone, resulting in poor growth and short stature

A

growth hormone deficiency

62
Q

After administering DDAVP for diabetes insipidus (DI), a decrease in urine output is seen in __________ (time period)

A

1-2 hours

63
Q

group of disorders that causes inability of the adrenal gland to create adequate cortisol

A

congenital adrenal hyperplasia (CAH)

64
Q

type of diabetes insipidus (DI) caused by genetic mutation, hypercalcemia, low protein diet, hypokalemia, release of ureteral obstruction, or certain medications

A

nephrogenic diabetes insipidus (DI)

65
Q

Treatment for diabetes insipidus (DI) is aimed at maintaining normal __________________ and administering synthetic ______________, also known as ________.

A

fluid and electrolyte balance, vasopressin, DDAVP

66
Q

Oral radioactive iodine works when it is __________ by the thyroid gland and __________ thyroid tissues

A

absorbed, destroys

67
Q

Acquired hypothyroidism is most commonly caused by the autoimmune disease ___________________

A

Hashimoto’s thyroiditis

68
Q

The gonadotropin-releasing hormone (GnRH) stimulation test signals the body to release ____ and _____, and then serial samples are drawn.

A

LH, FSH

69
Q

If galactosemia is left untreated, the child will usually _______. Even with treatment, the infant may have _______________ or ___________ deficits later in life.

A

die, developmental, learning

70
Q

Manifestations include goiter, dry skin, dull hair, fatigue, constipation, weight gain, cold intolerance, decreased growth, edema on face and hands, irregular or delayed menses

A

acquired hypothyroidism

71
Q

insulin injection sites must be rotated to prevent development of ____________ that can inhibit absorption

A

fatty lumps

72
Q

Blood sugar elevations for _____ consecutive days requires a need for increasing insulin doses

A

3-4

73
Q

The goal of treatment for Tay-Sachs disease is _____________________ and _______________.

A

symptom management, family support

74
Q

type of diabetes insipidus (DI) in which there is a deficiency in vasopressin, the antidiuretic hormone (ADH)

A

central diabetes insipidus (DI)

75
Q

Manifestations include abdominal pain, nausea, vomiting, fruity smelling breath, altered LOC, Kussmaul respirations, dehydration

A

diabetic ketoacidosis (DKA)

76
Q

With type 1 diabetes, avoid exercising during insulin ______

A

peak

77
Q

To assess growth hormone levels, a _______________ is given and serial GH levels are drawn.

A

stimulant

78
Q

Most often, precocious puberty is ______________ but can result from ____________ or ______________.

A

idiopathic, trauma, infections

79
Q

phenylalanine hydroxylase deficiency (PAH) is most common in these ethnic groups

A

white children (Turkish, Irish)

80
Q

In diagnosing precocious puberty, radiographs of the _______ can be useful to check for _______________ and ________________

A

wrist, growth plate closure, bone maturation

81
Q

gold standard diagnostic test for precocious puberty

A

gonadotropin-releasing hormone (GnRH) stimulation test

82
Q

In congenital adrenal hyperplasia (CAH), increased effort to create more ____________ results in an excess of ______________.

A

cortisol, androgens

83
Q

three required newborn screenings

A

PAH, CAH, TSH level (hypothyroidism)

84
Q

A child with Tay-Sachs disease will have normal development until ____________, when they have _____________________ delay and ______________ degeneration.

A

6 months, developmental, neurologic

85
Q

In maple syrup urine disease, the buildup of amino acids causes ____________ within __________ of birth.

A

ketoacidosis, 72 hours

86
Q

With type 1 diabetes, add an extra _______ g carbohydrate snack for each ______ minutes of exercise

A

15-30, 45-60

87
Q

Graves disease involves an increased uptake of _______________.

A

radioactive iodine

88
Q

Manifestations include polyuria, polydipsia, nocturia, and dehydration

A

diabetes insipidus (DI)

89
Q

Nonadherence to therapy for precocious puberty can (suppress, promote) puberty.

A

promote

90
Q

The diet for phenylalanine hydroxylase deficiency (PAH) must be closely monitored to balance ___________ to meet growth requirements

A

amino acids

91
Q

Careful management of growth is plotted over __________ for growth hormone deficiency, and these six tests rule out other causes

A

6-12 months
thyroid, electrolytes, renal function, CBC, bone radiographs, karyotyping

92
Q

Treatment for syndrome of inappropriate antidiuretic hormone (SIADH) is to correct ____________________. Some providers may order temporary _______________ to correct hyponatremia.

A

underlying cause, fluid restriction

93
Q

While treating syndrome of inappropriate antidiuretic hormone (SIADH), _______ and ____________ should be calculated and recorded accurately along with a daily ______

A

intake, output, weight

94
Q

Severe symptoms of hyponatremia include __________ and ________________.

A

seizures, coma

95
Q

Treatment for congenital hypothyroidism includes lifelong ___________________ replacement, most often with medication ______________, which is titrated based on _______ and ____________

A

thyroid hormone, levothyroxine, age, weight

96
Q

In (congenital, acquired) hypothyroidism, the signs and symptoms will resolve as the medication becomes therapeutic.

A

acquired

97
Q

autoimmune response that results in excess thyroid hormone and enlarged thyroid gland

A

hyperthyroidism or Graves disease

98
Q

Acquired hypothyroidism is most often seen in children with a _____________ and is more common in (boys, girls)

A

family history of thyroid disease, girls

99
Q

Diagnostics of diabetes insipidus (DI) include polyuria with sodium levels greater than _______, urine specific gravity less than __________, and serum osmolality greater than _________ with absence of hyperglycemia, hypokalemia, hypercalcemia, and chronic renal insufficiency.

A

150, 1.005, 300

100
Q

hormone that plays a role in multiple body processes, including stimulating female body to create breastmilk during pregnancy and shortly after childbirth

A

prolactin

101
Q

type of diabetes insipidus (DI) which is frequently the result of head traumas, tumors, infections, or cranial radiation

A

central diabetes insipidus (DI)

102
Q

Diagnosis of syndrome of inappropriate antidiuretic hormone (SIADH) is made when there is a __________ in urine output despite ____________ fluid intake

A

decrease, adequate

103
Q

Moderate symptoms of hyponatremia include _______________, __________________, _______________, and __________________.

A

confusion, irritability, lethargy, altered LOC

104
Q

In addition to secondary sex characteristics, a patient with precocious puberty has an accelerated _____________ and advanced _______ maturation.

A

growth rate, bone

105
Q

Precocious puberty occurs before ___ years in girls and ____ years in boys

A

8, 9

106
Q

deficient growth hormone levels are less than ____ ng/mL, and ___ positive results are required for diagnosis

A

10, 2

107
Q

The first signs of phenylalanine hydroxylase deficiency (PAH) are ____________ problems such as ____________ and __________.

A

digestive, nausea, vomiting

108
Q

________% of children who undergo a subtotal or partial thyroidectomy develop ______________ and will need to begin taking __________________.

A

60-80%, hypothyroidism, thyroid replacement hormones

109
Q

treatment for galactosemia includes a lifelong diet that restricts __________

A

lactose

110
Q

Congenital hypothyroidism is caused by a(n) ________ or ___________ thyroid gland or ____________ or _____________ disorder

A

aplastic, undeveloped, hypothalamic, hypopituitary

111
Q

Contributing factors in girls include obesity, ethnicity, genetic predisposition, stress, endocrine disruption from environmental chemicals

A

precocious puberty

112
Q

syndrome of inappropriate antidiuretic hormone (SIADH) is usually __________ and resolves with correction of _______________

A

transient, underlying cause

113
Q

Manifestations include ambiguous genitalia in female infant, postnatal virilization, and salt wasting crisis

A

congenital adrenal hyperplasia (CAH)

114
Q

Phenylalanine hydroxylase deficiency (PAH) is treated with a special diet that restricts __________ intake, including high ________ foods such as ______, ______, ___________, _______, __________, and _________.

A

phenylalanine (PHE), protein, meat, fish, eggs, milk, cheese, legumes

115
Q

Treatment of congenital adrenal hyperplasia (CAH) involves prompt correction of ___________________ imbalances and lifelong ________________ replacement.

A

fluid and electrolyte, glucocorticoid

116
Q

condition which results in the inability to concentrate urine

A

diabetes insipidus (DI)

117
Q

Untreated hypothyroidism causes _________________ impairment in children.

A

intellectual

118
Q

Manifestations include hyponatremia, decreased urine output, increased urine specific gravity, fluid retention, weight gain, increased urine osmolality

A

syndrome of inappropriate antidiuretic hormone (SIADH)

119
Q

Hormonal control of many body functions is lacking until __________ of age.

A

12-18 months

120
Q

In diagnosing syndrome of inappropriate antidiuretic hormone (SIADH), ____________, _____________, and ______________ function tests rule out other causes of hyponatremia

A

adrenal, thyroid, renal

121
Q

early onset of puberty and secondary sex characteristics

A

precocious puberty

122
Q

formerly known as phenylketonuria

A

phenylalanine hydroxylase deficiency (PAH)

123
Q

In precocious puberty, early _____________________ can result in short statures in adulthood.

A

growth plate fusion

124
Q

Characterized by low serum sodium, high potassium, hypovolemia, and eventually hypotensive crisis

A

salt wasting crisis

125
Q

Infants may manifest imbalances in concentration of these 5 things

A

fluids, electrolytes, amino acids, glucose, trace substances

126
Q

Acquired hypothyroidism is treated with the medication _____________, and the dose is titrated to maintain ____ in the upper normal range and ____ in the normal range.

A

levothyroxine, T4, TSH

127
Q

Nursing management involves blood glucose checks, vitals, and neuro checks every ______; strict ______ and _______; frequent lab draws to check _________________; IV _______ replacement; _________ replacement; and IV _______ drip

A

hour, intake, output, fluid and electrolyte balance, fluid, potassium, insulin

128
Q

four diseases associated with short stature

A

Turner syndrome
Prader-Willi syndrome
renal disease
inflammatory bowel disease

129
Q

signs and symptoms of hypoglycemia in infants and toddlers can be mistaken for ______________

A

temper tantrums

130
Q

inability of body to excrete free water

A

syndrome of inappropriate antidiuretic hormone (SIADH)

131
Q

Treatment for maple syrup urine disease involves _________ to reduce amount of circulating amino acids and a lifelong diet ____ in protein and __________ amino acids.

A

dialysis, low, restricted

132
Q

Risks of subtotal or partial thyroidectomy include _____________ paralysis and injury to the _____________.

A

vocal cord, parathyroid glands

133
Q

Signs and symptoms of excess DDAVP (3)

A

decreased urine output, headaches, water retention