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Primary PNP > PNP-PC Part 4 > Flashcards

PNP-PC Part 4 Flashcards

1
Q

Ambiguous genitalia in infant

A

Congenital adrenal hyperplasia

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2
Q

Any child over 2 years who is more than 2 SD below mean on standard growth charts or has decreased growth velocity

A

Short stature/Poor linear growth

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3
Q

Born with short arms and legs and a long torso

A

Skeletal dysplasia

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4
Q

Deficiency in vitamin D (nutrition or endocrine disorder; causes softening of the growth plates at the end of the long bones

A

Rickets

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5
Q

Body proportions at birth

A

1.7:1

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6
Q

Body proportions at 3 years

A

1.3:1

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7
Q

Body proportions at 7 years

A

1:1

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8
Q

Not a disease
Delayed bone age with normal growth rate for bone age
Slowed linear growth at 1-3 years; height at or just below 3rd percentile; delayed puberty; history of similar pattern in family

A

Constitutional Growth Delay

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9
Q

Delayed bone age with normal growth rate for bone age

child may be 13 years old, but based on left wrist x-ray, they may only be 10 years old for bone age, so their height needs to be adjusted for what a 10-year-old would be

A

Constitutional Growth Delay

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10
Q

Inhibits somatic growth OVERALL

Strongly associated with Turner Syndrome, Downs Syndrome

A

Growth Hormone Deficiency

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11
Q

Diagnosis based on radioimmunoassay of plasma GH levels

A

Growth Hormone Deficiency

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12
Q

How much does the growth rate change with growth hormone replacement?

A

From 3.5-4cm/yr (1.4 in-1.5 in/year) to 8-9cm/yr (3in-3.5in/year)

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13
Q

Common side effects of growth hormone replacement

A

**Slipped Capital Femoral Epiphysis
**Hypothyroidism
**Pseudotumor Cerebri
Hypertension
Fluid retention

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14
Q

Early-onset of puberty

Defined as sexual development before age 9 in boys or before age 8 in girls

A

Precocious puberty

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15
Q

What is common in precocious puberty in males?

A

CNS tumors

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16
Q

Leuprolide/Lupron

A

Slows pre-pubertal growth to normal rates
Long-acting GRH antagonist
will be discontinued at normal pubertal changes to resume

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17
Q

No scrotal development in males at 14 years of age

A

Delayed Puberty

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18
Q

No breast bud development in females at 13 years of age

A

Delayed Puberty

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19
Q

Bone age x-ray
Free T4 and TSH
Growth hormones
LH and FSH

A

Delayed Puberty

Precocious Puberty

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20
Q

Present at birth
Can lead to cognitive delay if untreated
Included on mandatory newborn screening

A

congenital Hypothyroidism

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21
Q 
***Prolonged jaundice
Poor appetite and suck
****Large tongue (Macroglossia)
Dyspnea with feeding
****Constipation
Sluggishness
Hypothermia
Bradycardia
A

Congenital Hypothyroidism

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22
Q

“Best baby ever…hardly ever cries”

A

Congenital Hypothyroidism

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23
Q

***Slightly increased head circumference (frontal bossing)
Dry, thick, scaly, coarse skin with jaundice
Dry, coarse, brittle hair
**Hypotonia, short extremities

A

Congenital Hypothyroidism

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24
Q

Elevated TSH
Low T4
Low T3

A

Congenital Hypothyroidism

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25
Q

Present with goiter
Result of autoimmune process
Most common cause of hypothyroidism in children and adolescent

A

Acquired Hypothyroidism

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26
Q 
***Dry skin
Apathetic affect
***Sensitivity to cold
Decreased appetite
***Lethargy
***Constipation
***Weight gain***
Difficulty with concentration
Poor school performance
Delayed puberty
Enlarged and firm thyroid ****
A

Acquired hypothyroidism

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27
Q

Increased TSH
Normal or low T4
Normal or low T3

A

Acquired hypothyroidism

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28
Q

Most common cause of hyperthyroidism
Autoimmune condition in which excess thyroid hormones are produced by the enlarged thyroid gland
Peaks: 11-15 years

A

Graves Disease

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29
Q 
Goiter
Thyroid bruit
Tachycardia
Wide pulse pressure
Underweight
Exophthalmos
Warm/moist skin
Tremor
Hyperreflexia
A

Hyperthyroidism

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30
Q

Low TSH
High T3
High T4

A

Hyperthyroidism

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31
Q

Dehydration
Weight loss
Tachycardia
Vaginal yeast, thrush, other infection

A

Type 1 Diabetes

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32
Q

Strong association with obesity, sedentary lifestyles, high-calorie, lipid-rich foods

A

Type 2 Diabetes

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33
Q 
Polydipsia, polyuria,***polyphagia 
Excessive weight gain
Fatigue
Recent illness
Repeated infections (esp with yeast)
****Vomiting and abdominal pain****
Positive family history
A

Type 2 Diabetes

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34
Q

80% of all diabetes in children younger than 9

A

Type 1 Diabetes

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35
Q

More than 50% of children with Type 1 Diabetes present with _____

A

Diabetic Ketoacidosis

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36
Q

Vomiting, lethargy, obtunded neurologic status
Fruity breath, slow, labored breathing
Vaginal yeast, thrush, other infection
***Polydipsia, polyuria, polyphagia

A

Diabetic Ketoacidosis

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37
Q

Insidious onset diabetes

A

Type 2

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38
Q

Acute onset of diabetes

A

Type 1

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39
Q

1st sign of diabetes ketoacidosis

A

vomiting and abdominal pain

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40
Q

To diagnose diabetes, NP knows a fasting glucose serum glucose must be: ____ mg/dl on __ separate occasions

A

126 mg/dl on 2 separate occasions

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41
Q

A1C goal for all pediatric age groups

A

<7.5%

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42
Q

A1C goal for children less than 5

A

<8.5%

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43
Q

A1C goal for children 6-12

A

<8%

44
Q

A1C goal for children 13-19

A

<7.5%

45
Q

What activities/instances increase glucose?

A

Sickness and Stress

46
Q

What activities/instances decrease glucose?

A

Alcohol and Exercise

47
Q

What is the mainstay for type 1 diabetes management with insulin?

A

Fact acting insulin (Novolog, Humalog, Apidura) with multiple injections to cover meals and snacks and a long-acting for basal coverage (Lantus/Levemir)

48
Q

If BG is > 300 two times in a row or on sick days, what test should type 1 diabetes take?

A

Urine testing

49
Q

Management for Type 2 Diabetes

A 
BG monitoring
Diet plan
Weight reduction
Exercise
Referral to endocrine
50
Q

Management of Overweight/Obesity

A

Balanced diet avoiding concentrated sugar
60 minutes of exercise a day
Behavior modification
Diet & Exercise only for 6 months then referral
Monitor for comorbidities

51
Q

Picture of chromosome lined up in pairs

A

Karyotype

52
Q

Occur when mutations affect a single gene

A

Monogenetic

53
Q

Changes in number/structure in chromosome (missing, extra, irregularity)

A

Chromosomal abnormalities

54
Q

Altered changes in some cells

A

Mosaicism

55
Q

Teratogen

Combination of genetic/environmental

A

Multifactorial

56
Q 
Sickle cell disease
Hemophilia and von Willebrand Disease
Marfan syndrome
Fragile X Syndrome
Duchene Muscular Dystrophy
A

Monogenetic Disorders

57
Q

Autosomal recessive disorder

Hallmarks: vaso-occlusive syndrome, hemolysis

A

Sickle cell disease

58
Q

***Hemoglobin electrophoresis
CBC with indices
Screen in pregnancy for African, Mediterranean, Middle Eastern

A

Sickle Cell Disease

59
Q

X-linked recessive disorder
Prolonged bleeding
Factor VIII deficiency/absence

A

Hemophilia A

60
Q

Males primarily affected

A

Hemophilia A and B

61
Q

Factor IX deficiency

X-linked recessive disorder

A

Hemophilia B

62
Q

Abnormality of vWF protein that affects both sexes

Most common inherited bleeding disorder

A

Von Willebrand

63
Q

Excessive bruising
Prolonged bleeding (prolonged aPTT)
Hemarthrosis
Factor VII or IX specific assay

A

Hemophilia A & B

64
Q

Hx of ecchymosis
Prolonged/excessive bleeding (menses, epistaxis, trauma)
Factor VII clotting activity
vWF antigen and vWF decreased

A

Von Willebrand

65
Q

Mutation that affects the fibrillin gene

Affects the skeleton, eyes, and cardiovascular system

A

Marfan Syndrome

66
Q 
Tall stature
Long fingers
Pigeon-breast deformity***
Hyper-extensible joints
High-arched palate
BL subluxation of lens
Floppy mitral valve
Aortic aneurysm and dissection
Defects in skin, lungs
Spontaneous pneumothorax**
A

Marfan Syndrome

67
Q

Fibrillin-1 genetic test

A

Marfan Syndrome

68
Q

Children with this condition are at risk for spontaneous pneumothorax, aortic root dilation, and mitral valve prolapse

A

Marfan Syndrome

69
Q

Most common inherited cause of intellectual disability

Mutation in 1 gene (FMR 1)

A

Fragile X Syndrome

70
Q

Mild to severe mental retardation and learning disabilities
Delayed language
Long/narrow face
Prominent/cupped ears
Enlarge testicles
Hyperextensible finger joints, pes planus
Hyperactivity or ADHD

MALES

A

Fragile X Syndrome Males

71
Q

Boys with developmental delays consider which genetic condition

A

Fragile X syndrome

72
Q

Mild cognitive deficits to mental retardation
Delayed language
Shy, social anxiety
Prominent ears
Long, narrow face or high-arched palate
Hyperextensible finger joints, pes planus
Inattention but < hyperactivity

FEMALES

A

Fragile X Syndrome

73
Q

What conditions are children with fragile x syndrome at risk for?

A

Hypertension

Mitral valve prolapse

74
Q

X-linked recessive gene
Males are majority affected
Mutation in gene Xp21

A

Duchene Muscular Dystrophy

75
Q 
Boys exhibit progressive decline in motor milestones
Growth delay
Consistent pattern of weakness
Clumsiness, frequent falls
\+GOWERS****
Calf pseudohypertrophy
A

Duchene Muscular Dystrophy

76
Q

**Elevated CK
Genetic analysis (deletions/duplications)
Muscle biopsy
EMG

A

Duchene Muscular Dystrophy

77
Q

Most common chromosomal disorder

Extra copy of genetic material on 21st chromosome

A

Down syndrome

78
Q 
short stature
brachycephaly
midface hypoplasia
epicanthal folds with palpebral fissures that slant down to midline
small mouth with protruding tongue
myopia
lax joints
single palmar crease
exaggerated space between great and 2nd toes
A

Down syndrome

79
Q

Individuals with Down syndrome have increased risk of…

A

Heart defects
GI problems (GERD, celiac disease)
Hypothyroidism
Increased risk of leukemia, duodenal atresia, cardiac anomalies

80
Q

Most common form of syndromic obesity

Absence of group of genes on chromosome 15

A

Prader Willi Syndrome

81
Q 
***Neonatal hypotonia
Failure to thrive in infancy
Short stature
***Central obesity later in childhood--due to an increased desire to eat and eat a lot
Strabismus, Myopia/hyperopia
Enamel hypoplasia
A

Prader Willi Syndrome

82
Q

Excessive weight gain, neonatal/infant hypotonia, feeding issues, hypogonadism, DD, hyperphagia

A

Prader Willi Syndrome-Major criteria

83
Q

Most common sex-chromosome abnormality in girls

A

Turner Syndrome

84
Q 
Short stature for family
Short neck with webbing
Posteriorly rotated ears
Shield chest-wide spread nipples, webbed neck
Ptosis
Short 4th and 5th metacarpals
Short legs
Horseshoe kidney
Cardiac disease
Delayed puberty
A

Turner Syndrome

85
Q

Most common neural tube defects

A

Spina Bifida or myelodysplasia

86
Q

Cleft in vertebral column with a corresponding defect in the skin so that the spinal column is exposed

A

Spina Bifida

87
Q

Hair tufts
Dimpling
Hemangiomas
Dermoid cysts

A

Neural tube defects

88
Q

Lower extremity weakness or atrophy, absence of sensation
foot deformities
bowel/bowel dysregulation

A

neural tube defects

89
Q

Fissure of upper lip along frenulum hat often include bone of the upper jaw and/or gum

A

Cleft lip

90
Q

Fissure in either the hard palate and /or soft palate

Difficulty feeding because of inability to suck

A

Cleft palate

91
Q

S/S of upper airway obstruction related to micrognathia

A

Pierre Robin Sequence

92
Q

Nasal-sounding speech

A

Submucous cleft palate

93
Q

Autosomal recessive

Deficiency in enzyme phenylalanine hydroxylase

A

Phenylketonuria

94
Q 
Insidious onset
Symptoms not usually present until early infancy
Neonatal vomiting
Mousy odor of urine and sweat
Fine, rapid, irregular tremor
A

Phenylketonuria

95
Q

leads to irreversible intellectual disability, seizures, behavioral abnormalities, microcephaly, and skin disease

A

Phenylketonuria

96
Q

Management for Phenylketonuria

A

Diet low in phenylalanine
No breastmilk
Supplement tyrosine
Child needs specific formula

97
Q

Preventable cause of intellectual disability

A

Congenital Hypothyroidism

98
Q

Asymptomatic at birth
Severe mental retardation and growth retardation
Irreversible neurologic problems after 16 days of no therapy

A

Congenital Hypothyroidism

99
Q

Deficiency of enzyme gal-1-PUT

Altered metabolism of galactose

A

Galactosemia

100
Q 
Symptoms after galactose in diet:
Jaundice
Vomiting
Hepatosplenomegaly
Failure to thrive
Poor feeding
Lethargy
Diarrhea
Sepsis
A

Galactosemia

101
Q

Treamtent for Galactosemia

A

Galactose free diet

-no breastmilk or dairy formula

102
Q

Inherited disorder due to missing enzyme affecting steroid/hormone production

A

Congenital Adrenal Hyperplasia (CAH)

103
Q

Most common Congenital Adrenal Hyperplasia (CAH)

A

21-hydroxylase deficiency (21-OCH)

104
Q

Early pubic hair and bread growth, growth spurts, body odor in males

A

Congenital Adrenal Hyperplasia (CAH)

105
Q

Excessive androgen production

A

Congenital Adrenal Hyperplasia (CAH)

106
Q

Girls with classic form present with genital atypia

A

Congenital Adrenal Hyperplasia (CAH)

107
Q

Treatment Congenital Adrenal Hyperplasia (CAH)

A

Glucocorticoid replacement

Mineralocorticoids and sodium replacement

Primary PNP (8 decks)

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