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Pediatrics Exam 1 > Growth Abnormalities > Flashcards

Growth Abnormalities Flashcards

1
Q

Most notable time for physical growth & ↑ in complexity of function

A

0-5 Years Old

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2
Q

Brain grows to 2/3 of the adult brain size
by age _____

A

2½–3 years

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3
Q

Identifying disturbances in development
during these early years is critical because time sensitive interventions may be instituted to address developmental issues

A

Ages 2-5

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4
Q

Concerns with 2000 growth charts

A

2000 growth charts use NHANES data (1960’s-1990s) which compares current childs weight status to a time before the current obesity epidemic

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5
Q

CDC growth charts

A
  • 16 charts (8 for boys, 8 for girls): Ability to plot head circumference, height, weight, BMI and (in some cases) compare these to one another
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6
Q

A child’s growth & height potential is determined largely by _____

A

genetic factors

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7
Q

Most children achieve an adult height within ____ of
the midparental height

A

8 cm
* Target (midparental) height of a child =
* Mean parental height + 6.5 cm for boys
* Mean parental height – 6.5 cm for girls

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8
Q

Bone Age

A
  • Radiograph of the left hand & wrist
  • Atlas of Skeletal Maturity, Greulich & Pyle
  • Delayed or advanced bone age is NOT diagnostic
    of any specific disease
  • Skeletal maturation determines remaining
    growth potential
  • Allows prediction of adult height
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9
Q

Normal vs. Pathologic Short Stature

A

Normal: Familial short stature, constitutional growth delay
Pathologic: Growth hormone deficiency,
Intrauterine growth restriction, Short stature associated w/ syndromes, Achondroplasia

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10
Q

Familial Short Stature growth

A
  • Normal birth weight & length in the first 2 years of life
  • Linear growth velocity decreases as they near their genetically determined percentile
  • Once the target percentile is reached, Resumes normal linear growth parallel to the growth curve, Usually 2 to 3 years old
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11
Q

______ are consistent with chronologic age

A

Skeletal maturation & timing of puberty

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12
Q

Constitutional Growth Delay

A

● Growth pattern similar to those with familial short stature EXCEPT
* Children follow a growth percentile that is below what is expected
based on parental heights
* Delay in skeletal maturation & the onset of puberty
● Growth continues beyond the time the average child stops growing
* Final height is appropriate for target height

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13
Q

We usually refer to these patients as being “late bloomers”

A

Constitutional Growth Delay

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14
Q

Growth Hormone Deficiency

A

Anterior Pituitary Gland → Human Growth Hormone (GH)
* Secretion stimulated by Growth Hormone Releasing Hormone (GHRH)
* Secretion inhibited by somatostatin
* GH is secreted in a pulsatile pattern (testing is problematic)

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15
Q

Growth hormone deficiency (GHD) is characterized by _____

A

decreased growth
velocity and delayed skeletal maturation in the absence of other explanations

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16
Q

____ is the MOST COMMON deficiency state

A

Idiopathic GHD

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17
Q

Infants with GHD typically have ____

A

normal birth weight w/ slightly reduced
length

18
Q

Common 1st step evaluating for GHD

A

Serum concentrations of IGF-1 reasonably correlate to GH secretion & action in an adequately nourished child

19
Q

Physical Exam Findings of GHD

A
  • Hypoglycemia: Often associated with pituitary deficiencies
  • Microphallus: Newborn males with gonadotropin & GH deficiency
  • Many GH deficient children have excess truncal adiposity
  • Isolated GHD & hypopituitarism may be unrecognized until late in infancy or childhood
20
Q

Imaging that should be done to patients with GHD

A

All patients diagnosed with GHD should have an MRI of the hypothalamus and
pituitary gland to evaluate for a tumor prior to starting therapy

21
Q

Growth Hormone Deficiency Treatment

A
  • Subcutaneous Recombinant GH
  • SQ daily injection
    Expensive
22
Q

Who is GH deficiency treatment indicated for

A
  • Chronic renal failure
  • Girls with Turner syndrome
  • Prader-Willi syndrome
  • Noonan’s syndrome
  • Children born small for gestational (SGA) who fail to demonstrate catch-up growth by age 4
  • Children with idiopathic short stature: Final height ~5–7 cm taller in this population
23
Q

SGA infants

A
  • Birth weight and/or length <3rd
    percentile
  • Includes constitutionally small
    infants & infants with IUGR
24
Q

If mild SGA/IUGR and no fetal abnormalities, may exhibit catch-up growth during the _____

A

first 3 years

25
Q

Small for Gestational Age/Intrauterine Growth Restriction causes

A
  • Poor maternal environment
  • Intrinsic fetal abnormalities
  • Congenital infections
  • Fetal malnutrition
26
Q

In contrast to children with constitutional growth delay, SGA/IUGR have _____

A

skeletal maturation that corresponds to chronologic age or is only mildly delayed.

27
Q

Short stature is associated with many syndromes, including:

A
  1. Turner Syndrome
  2. Prader-Willi Syndrome
  3. Down Syndrome
28
Q

Any girl with unexplained short stature for family → _____

A

Chromosomal evaluation (turner syndrome)

29
Q

Most common form of inherited disproportionate short stature

A

Achondroplasia (Dwarfism)
* Autosomal dominant

30
Q

Etiology of Achondroplasia (Dwarfism)

A
  • Autosomal dominant: 80% of cases result from new mutations.
  • Pathogenic variants in the fibroblast
    growth factor receptor 3 (FGFR3)
    gene
  • Mutation disrupts chondrocyte
    proliferation & differentiation,
    disrupting growth plate structure
31
Q

Clinical presentation Achondroplasia (Dwarfism)

A
  • Disproportionate short stature
  • Short limbs w/ redundant skin folds
  • Macrocephaly
  • Normal-sized trunk
  • Limited elbow extension
  • Genu varum
  • Large head with frontal bossing (rounded prominence)
  • Midface hypoplasia
  • Trident configuration of hands
  • Thoracolumbar gibbus (kyphosis) in infancy
  • Exaggerated lumbar lordosis
32
Q

Diagnosis of Achondroplasia (Dwarfism)

A
  • Prenatal ultrasound: fetus with short stature & limb foreshortening, Macrocephaly
  • Imaging: X-ray & baseline MRI or CT of brain
  • Genetic testing: Fibroblast growth factor receptor-3 (FGFR3) gene mutations confirm diagnosis
33
Q

Achondroplasia (Dwarfism) complications

A
  • Speech development problems
  • Teeth crowding
  • Sleep Apnea
  • Pneumonia
34
Q

Psychosocial Short Stature
(Psychosocial Dwarfism)

A
  • Growth impairment associated with
    emotional deprivation
  • Under-nutrition probably plays a
    role as well
35
Q

Is GH therapy used in Psychosocial Short Stature?

A
  • GH secretion in children with
    psychosocial short stature is
    diminished
  • GH therapy is usually not
    beneficial
36
Q

How can psychosocial short stature be improved?

A

A change in the psychological
environment at home improves:
* growth
* GH secretion
* personality
* eating behaviors

37
Q

Growth Hormone Excess

A

*Excessive GH secretion is rare & generally associated with a
functioning pituitary adenoma
*GH excess leads to gigantism → if the epiphyses are open (before puberty)
*GH excess leads to acromegaly → if the epiphyses are closed (After puberty)

38
Q

Precocious Puberty

A

Tall stature for age or rapid growth
*Typically associated with early signs of puberty & advanced bone age
*Patients can be tall for age, but they do not achieve a taller final
height than would be genetically & phenotypically expected

39
Q

History workup for growth

A
  • The upper limit of acceptable height in both sexes has increased over time
  • Concerns about excessive growth in girls are less frequent than in the past
  • History of chronic illness
  • Medications
  • Birth weight & height, pattern of growth since birth
  • Family history & family growth patterns, growth curves
40
Q

Physical exam for assessing growth

A
  • Height, weight, head circumference
  • Pubertal (Tanner) stage, & assessment of skeletal maturation allow estimation
    of final adult height
  • Dysmorphic features, body segment proportion, & psychological health.
  • Child with poor weight gain as the primary disturbance, a nutritional
    assessment is indicated.
41
Q
A

Pediatrics Exam 1 (6 decks)

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