Chapter 51: The Child with an Endocrine or Metabolic Alteration Flashcards
1
Q
Manifestations include irritability, tachycardia, increased appetite without weight gain, prominent eyes, hypertension
A
neonatal Graves disease
2
Q
Thyroxine (T4) and TSH levels vary with _______
A
age
3
Q
An infant with a _____ T4 and a TSH value exceeding _______ mU/mL is considered to have primary/congenital hypothyroidism.
A
low, 40
4
Q
In phenylalanine hydroxylase deficiency (PAH), metabolic alterations usually start _____________ but may not be noticed until approximately _____________.
A
immediately, 3 months
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
6
Q
metabolic disorder that results in an abnormal buildup of gangliosides on neurons
A
Tay-Sachs disease
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
8
Q
hormone that stimulates cell growth and reproduction
A
growth hormone (GH)
9
Q
While treating a child with congenital hypothyroidism, parents should be educated about manifestations of ________________
A
hyperthyroidism
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
11
Q
third-line (final) treatment of Graves disease
A
subtotal or partial thyroidectomy
12
Q
Graves disease is caused by autoantibodies called ________________ which stimulate the thyroid to produce T3 and T4
A
thyroid stimulating immunoglobulin (TSIs)
13
Q
oral radioactive iodine is used in children older than ___________
A
10 years
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
15
Q
Preschoolers are at an increased risk for hypoglycemia due to increased _____________ levels
A
energy
16
Q
Treatment of precocious puberty is aimed at _____________________ or ______________ puberty development, involving _______________
A
stopping, reversing, GnRH blockers
17
Q
Hormonal release is controlled by the _____________
A
circadian rhythm
18
Q
growth hormone deficiency is defined as consistently poor growth (less than __ cm per year)
A
5
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
20
Q
Mild/early symptoms of hyponatremia include _______________, ________________, _____________, and ________________.
A
anorexia, headache, nausea, vomiting
21
Q
stress hormone that stimulates the release of cortisol
A
adrenocorticotropic hormone (ACTH)
22
Q
Injury to the parathyroid gland can result in _______________.
A
hypocalcemia
23
Q
most common cause of syndrome of inappropriate antidiuretic hormone (SIADH) is inappropriate use of ____________ in treatment of diabetes insipidus (DI)
A
vasopressin
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
25
first-line treatment of Graves disease
medication: methimazole
26
type of diabetes insipidus (DI) in which the kidneys are insensitive to vasopressin
nephrogenic diabetes insipidus (DI)
27
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
hyperthyroidism or Graves disease
28
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.
decreases, increases, dilute urine, sodium
29
The endocrine system is (more, less) developed at birth than any other system
less
30
methimazole works by _____________________
blocking thyroid hormone production
31
Lab studies for syndrome of inappropriate antidiuretic hormone (SIADH) may show hypo_______, hypo________, and low serum ____________.
natremia, chloremia, osmolality
32
The treatment PLAN for congenital adrenal hyperplasia (CAH) includes frequent _____________________ and ___________ plan that usually involves a __________ or ____________ dose of ________________ replacement.
endocrinologist reevaluations, sick-day, doubled, triple, glucocorticoid
33
In diagnosing precocious puberty, _____ and ______ can identify any pituitary gland tumors
CTs, MRIs
34
hormone that stimulates thyroid to release hormones to regulate energy use
thyroid stimulating hormone (TSH)
35
The medication ____________ can be given to some individuals with phenylalanine hydroxylase deficiency (PAH) to lower the circulating levels of phenylalanine (PHE).
sapropterin (KUVAN)
36
Neonatal Graves is caused by maternal transfer of _________________ across placenta to developing fetus
thyroid stimulating immunoglobulin (TSIs)
37
Phenylalanine hydroxylase deficiency (PAH) is tested within first _______ after birth, but the test is not ___________.
24 hours, diagnostic
38
Hyperthyroidism or Graves disease is more common in (boys, girls) with peak age of diagnosis between ____________ of age.
girls, 10-14 years
39
phase marked by lowered or normal BG levels and decreased need for insulin
honeymoon phase
40
deficiency in enzyme that converts galactose to glucose during digestion
galactosemia
41
most common form of diabetes insipidus (DI)
central diabetes insipidus (DI)
42
gonadotropin hormone that stimulates ovulation in females and production of testosterone in males
luteinizing hormone (LH)
43
May have no signs and symptoms or may present with skin mottling, large anterior fontanel, large tongue, distended abdomen, slow reflexes, or hypotonia
congenital hypothyroidism
44
other causes include infections, trauma, tumors, generalized seizures
syndrome of inappropriate antidiuretic hormone (SIADH)
45
_____________ precautions should be used when serum sodium level is less than 125 while treating syndrome of inappropriate antidiuretic hormone (SIADH)
Seizure
46
second-line treatment for Graves disease
oral radioactive iodine
47
very rare disorder that negatively affects metabolism of certain amino acids
maple syrup urine disease
48
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.
phenylalanine (PHE), genetic counseling
49
Doses of DDAVP for diabetes insipidus (DI) should be timed so the child has a mild increase in ___________ to prevent _______________ and water _____________.
urination, overtreatment, retention
50
When treating Graves disease with oral radioactive iodine, results are seen in ______________ after treatment
6-18 weeks
51
While manifestations of hyperthyroidism or Graves disease are self limiting, cardiac ________ and _________ can occur if poorly treated or unrecognized.
failure, death
52
In treating syndrome of inappropriate antidiuretic hormone (SIADH) during period of severe hyponatremia, (fast, slow) IV infusions may be needed to avoid CNS damage.
slow
53
A late sign and long-term consequence of phenylalanine hydroxylase deficiency (PAH) is ______________ which results in __________________.
CNS damage, cognitive impairment
54
Growth hormone is often given _____ as a __________ before bedtime.
daily, subcutaneous injection
55
Diagnosis of diabetes insipidus (DI) often involves a water deprivation test, where the child is deprived of all fluid intake for __________.
7-8 hours
56
gold standard for officially diagnosing congenital adrenal hyperplasia (CAH)
corticotropin stimulation test
57
Elevated ____ and ____ levels with suppressed _____ levels are highly suggestive of Graves disease.
T3, T4, TSH
58
honeymoon phase can last __________
7 months
59
gonadotropin hormone that regulates growth and puberty maturation
follicle stimulating hormone (FSH)
60
most common pediatric endocrine disorder
type 1 diabetes
61
inadequate secretion of human growth hormone, resulting in poor growth and short stature
growth hormone deficiency
62
After administering DDAVP for diabetes insipidus (DI), a decrease in urine output is seen in __________ (time period)
1-2 hours
63
group of disorders that causes inability of the adrenal gland to create adequate cortisol
congenital adrenal hyperplasia (CAH)
64
type of diabetes insipidus (DI) caused by genetic mutation, hypercalcemia, low protein diet, hypokalemia, release of ureteral obstruction, or certain medications
nephrogenic diabetes insipidus (DI)
65
Treatment for diabetes insipidus (DI) is aimed at maintaining normal __________________ and administering synthetic ______________, also known as ________.
fluid and electrolyte balance, vasopressin, DDAVP
66
Oral radioactive iodine works when it is __________ by the thyroid gland and __________ thyroid tissues
absorbed, destroys
67
Acquired hypothyroidism is most commonly caused by the autoimmune disease ___________________
Hashimoto's thyroiditis
68
The gonadotropin-releasing hormone (GnRH) stimulation test signals the body to release ____ and _____, and then serial samples are drawn.
LH, FSH
69
If galactosemia is left untreated, the child will usually _______. Even with treatment, the infant may have _______________ or ___________ deficits later in life.
die, developmental, learning
70
Manifestations include goiter, dry skin, dull hair, fatigue, constipation, weight gain, cold intolerance, decreased growth, edema on face and hands, irregular or delayed menses
acquired hypothyroidism
71
insulin injection sites must be rotated to prevent development of ____________ that can inhibit absorption
fatty lumps
72
Blood sugar elevations for _____ consecutive days requires a need for increasing insulin doses
3-4
73
The goal of treatment for Tay-Sachs disease is _____________________ and _______________.
symptom management, family support
74
type of diabetes insipidus (DI) in which there is a deficiency in vasopressin, the antidiuretic hormone (ADH)
central diabetes insipidus (DI)
75
Manifestations include abdominal pain, nausea, vomiting, fruity smelling breath, altered LOC, Kussmaul respirations, dehydration
diabetic ketoacidosis (DKA)
76
With type 1 diabetes, avoid exercising during insulin ______
peak
77
To assess growth hormone levels, a _______________ is given and serial GH levels are drawn.
stimulant
78
Most often, precocious puberty is ______________ but can result from ____________ or ______________.
idiopathic, trauma, infections
79
phenylalanine hydroxylase deficiency (PAH) is most common in these ethnic groups
white children (Turkish, Irish)
80
In diagnosing precocious puberty, radiographs of the _______ can be useful to check for _______________ and ________________
wrist, growth plate closure, bone maturation
81
gold standard diagnostic test for precocious puberty
gonadotropin-releasing hormone (GnRH) stimulation test
82
In congenital adrenal hyperplasia (CAH), increased effort to create more ____________ results in an excess of ______________.
cortisol, androgens
83
three required newborn screenings
PAH, CAH, TSH level (hypothyroidism)
84
A child with Tay-Sachs disease will have normal development until ____________, when they have _____________________ delay and ______________ degeneration.
6 months, developmental, neurologic
85
In maple syrup urine disease, the buildup of amino acids causes ____________ within __________ of birth.
ketoacidosis, 72 hours
86
With type 1 diabetes, add an extra _______ g carbohydrate snack for each ______ minutes of exercise
15-30, 45-60
87
Graves disease involves an increased uptake of _______________.
radioactive iodine
88
Manifestations include polyuria, polydipsia, nocturia, and dehydration
diabetes insipidus (DI)
89
Nonadherence to therapy for precocious puberty can (suppress, promote) puberty.
promote
90
The diet for phenylalanine hydroxylase deficiency (PAH) must be closely monitored to balance ___________ to meet growth requirements
amino acids
91
Careful management of growth is plotted over __________ for growth hormone deficiency, and these six tests rule out other causes
6-12 months
thyroid, electrolytes, renal function, CBC, bone radiographs, karyotyping
92
Treatment for syndrome of inappropriate antidiuretic hormone (SIADH) is to correct ____________________. Some providers may order temporary _______________ to correct hyponatremia.
underlying cause, fluid restriction
93
While treating syndrome of inappropriate antidiuretic hormone (SIADH), _______ and ____________ should be calculated and recorded accurately along with a daily ______
intake, output, weight
94
Severe symptoms of hyponatremia include __________ and ________________.
seizures, coma
95
Treatment for congenital hypothyroidism includes lifelong ___________________ replacement, most often with medication ______________, which is titrated based on _______ and ____________
thyroid hormone, levothyroxine, age, weight
96
In (congenital, acquired) hypothyroidism, the signs and symptoms will resolve as the medication becomes therapeutic.
acquired
97
autoimmune response that results in excess thyroid hormone and enlarged thyroid gland
hyperthyroidism or Graves disease
98
Acquired hypothyroidism is most often seen in children with a _____________ and is more common in (boys, girls)
family history of thyroid disease, girls
99
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.
150, 1.005, 300
100
hormone that plays a role in multiple body processes, including stimulating female body to create breastmilk during pregnancy and shortly after childbirth
prolactin
101
type of diabetes insipidus (DI) which is frequently the result of head traumas, tumors, infections, or cranial radiation
central diabetes insipidus (DI)
102
Diagnosis of syndrome of inappropriate antidiuretic hormone (SIADH) is made when there is a __________ in urine output despite ____________ fluid intake
decrease, adequate
103
Moderate symptoms of hyponatremia include _______________, __________________, _______________, and __________________.
confusion, irritability, lethargy, altered LOC
104
In addition to secondary sex characteristics, a patient with precocious puberty has an accelerated _____________ and advanced _______ maturation.
growth rate, bone
105
Precocious puberty occurs before ___ years in girls and ____ years in boys
8, 9
106
deficient growth hormone levels are less than ____ ng/mL, and ___ positive results are required for diagnosis
10, 2
107
The first signs of phenylalanine hydroxylase deficiency (PAH) are ____________ problems such as ____________ and __________.
digestive, nausea, vomiting
108
________% of children who undergo a subtotal or partial thyroidectomy develop ______________ and will need to begin taking __________________.
60-80%, hypothyroidism, thyroid replacement hormones
109
treatment for galactosemia includes a lifelong diet that restricts __________
lactose
110
Congenital hypothyroidism is caused by a(n) ________ or ___________ thyroid gland or ____________ or _____________ disorder
aplastic, undeveloped, hypothalamic, hypopituitary
111
Contributing factors in girls include obesity, ethnicity, genetic predisposition, stress, endocrine disruption from environmental chemicals
precocious puberty
112
syndrome of inappropriate antidiuretic hormone (SIADH) is usually __________ and resolves with correction of _______________
transient, underlying cause
113
Manifestations include ambiguous genitalia in female infant, postnatal virilization, and salt wasting crisis
congenital adrenal hyperplasia (CAH)
114
Phenylalanine hydroxylase deficiency (PAH) is treated with a special diet that restricts __________ intake, including high ________ foods such as ______, ______, ___________, _______, __________, and _________.
phenylalanine (PHE), protein, meat, fish, eggs, milk, cheese, legumes
115
Treatment of congenital adrenal hyperplasia (CAH) involves prompt correction of ___________________ imbalances and lifelong ________________ replacement.
fluid and electrolyte, glucocorticoid
116
condition which results in the inability to concentrate urine
diabetes insipidus (DI)
117
Untreated hypothyroidism causes _________________ impairment in children.
intellectual
118
Manifestations include hyponatremia, decreased urine output, increased urine specific gravity, fluid retention, weight gain, increased urine osmolality
syndrome of inappropriate antidiuretic hormone (SIADH)
119
Hormonal control of many body functions is lacking until __________ of age.
12-18 months
120
In diagnosing syndrome of inappropriate antidiuretic hormone (SIADH), ____________, _____________, and ______________ function tests rule out other causes of hyponatremia
adrenal, thyroid, renal
121
early onset of puberty and secondary sex characteristics
precocious puberty
122
formerly known as phenylketonuria
phenylalanine hydroxylase deficiency (PAH)
123
In precocious puberty, early _____________________ can result in short statures in adulthood.
growth plate fusion
124
Characterized by low serum sodium, high potassium, hypovolemia, and eventually hypotensive crisis
salt wasting crisis
125
Infants may manifest imbalances in concentration of these 5 things
fluids, electrolytes, amino acids, glucose, trace substances
126
Acquired hypothyroidism is treated with the medication _____________, and the dose is titrated to maintain ____ in the upper normal range and ____ in the normal range.
levothyroxine, T4, TSH
127
Nursing management involves blood glucose checks, vitals, and neuro checks every ______; strict ______ and _______; frequent lab draws to check _________________; IV _______ replacement; _________ replacement; and IV _______ drip
hour, intake, output, fluid and electrolyte balance, fluid, potassium, insulin
128
four diseases associated with short stature
Turner syndrome
Prader-Willi syndrome
renal disease
inflammatory bowel disease
129
signs and symptoms of hypoglycemia in infants and toddlers can be mistaken for ______________
temper tantrums
130
inability of body to excrete free water
syndrome of inappropriate antidiuretic hormone (SIADH)
131
Treatment for maple syrup urine disease involves _________ to reduce amount of circulating amino acids and a lifelong diet ____ in protein and __________ amino acids.
dialysis, low, restricted
132
Risks of subtotal or partial thyroidectomy include _____________ paralysis and injury to the _____________.
vocal cord, parathyroid glands
133
Signs and symptoms of excess DDAVP (3)
decreased urine output, headaches, water retention
