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DEMS Unit 4 > Evaluation of Worrisome Growth > Flashcards

Evaluation of Worrisome Growth Flashcards

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1
Q

Why do we care about growth

A
  • poor growth can be first/only sign of underlying health problem
  • consequences of delayed/missed diagnoses include potential permanent height deficits
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2
Q

AAP 2000 Recommendations

A
  • Measure height/weight at least at birth, 2-4 days, 1, 2, 4, 6, 9, 12, 15, 18, 24 months, and every year after through age 21
  • Plot on growth charts
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3
Q

which growth chart for ages 2-20 years old

A

CDC Growth Chart

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4
Q

What chart do you use for 0-3 years

A

WHO growth chart

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5
Q

difference between WHO and CDC growth chart

A
  • WHO = international
  • based on children who are primarily breastfed
  • WHO has steeper weight gain in first few years but that drops off to be leaner than CDC
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6
Q

Short Stature

A

-2 SD (3% = 1.9) based on age/gender
OR
> 2 SD below midparental target height

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7
Q

Dwarfism

A

height below -3 SD for age

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8
Q

Midget

A

dwarf with normal body proportions

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9
Q

Growth concern

A

abnormally slow linear growth velocity or dropping across 2 major centile lines on growth chart

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10
Q

difference between short stature adn growth velocity

A

abnormal growth velocity when line crosses 2 major centiles vs short stature is consistently low growth points (nl growth velocity)

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11
Q

Men are on average how much taller than women

A

5”

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12
Q

Genetic potential for boys

A

[Mom’s height+ 5in + Father’s height]/2

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13
Q

acceptable range for genetic potential

A

+/- 3”

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14
Q

Genetic potential for girls

A

[Dad’s height - 5in + Mom’s height]/2

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15
Q

Bone Age

A

there is direct correlation between degree of skeletal maturation and time of epiphyseal closure

  • greater bone age delay, longer the time before epiphyseal fusion ceases growth
  • height predictions can be made using child’s height and bone age
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16
Q

are growth predictions using bone age accurate in children with growth disorders

A

NO

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17
Q

why might growth predictions with bone age be inaccurate

A
  • can’t predict pubertal tempo

- growth disorder

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18
Q

how can we use bone age

A
  • help in differentiating causes of short stature
  • may just have delayed bone age but still potential for nl height (hormone deficiency–potential to reach nl height if adequate treatment)
  • some disorders where bone age may not be delayed but just a bit, but height projection still lower than target–usually from skeletal dysplasias (Turner’s syndrome, Newman’s syndrome) — don’t have potential to reach nl potential
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19
Q

Body Proportions

A

Upper to lower body ratio starts at 1.7 at birth and falls to 1.0 by 10

  • arm span shorter than height in boys before 10-11 years and girls before 10-14 after which arm span exceeds height

avg adult male has arm span of 5.2cm >ht and girls 1.2cm >ht

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20
Q

Nl causes of short stature/abnormal growth

A
  • Constitutional short stature

- Familial short stature

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21
Q

Constitutional short stature

A
  • growth deceleration during first 2 years of life (weight and height) followed by nl growth paralleling lower percentile curve throughout prepubertal years
  • skeletal maturation delayed
  • catch- upgrowth achieved by late puberty adn delayed fusion of growth plates
  • generally end up along lower end of nl height range
  • polygenic trait; positive family in 60-80% pts
  • Genetic defects causing CGD unclear
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22
Q

is constitutional growth delay common

A

YES

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23
Q

treatment for constitutional growth delay

A

reassurance of nl growth pattern

  • can treat bosy with testosterone if bone age greater or equal to 11.5 years to avoid compromising nl height
  • can give girls estrogen (not as common)
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24
Q

are ppl with constitutional growth delay or familial short stature born small

A

no for both

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25
Q

Familial short stature

A

children who have nl growth velocity and height that are within nl limits for parent’s heights

  • initially decrease in growth rate between 6-18 months
  • some families with short stature may have tubular bone alterations (brachydactyly syndromes, SHOX haploinsufficiencies)
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26
Q

when will kids with constitutional growth delay fall of in growth

A

in 1st 2 years

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27
Q

SHOX

A

gene on short arm of X and Y; important for height; can be mutated in short stature

28
Q

does Constitutional Growth Delay affect height, weight, or both?

A

both

29
Q

Does familial short stature affect height, weight, or both?

A

weight generally not affected

30
Q

Causes of failure to thrive

A

psychosocial and poor nutrition

- may look like constitutional growth delay

31
Q

Nutritional Growth Retardation

A

linear grwoth stunting from poor weight gain in children over 2 years

  • may be secondary to systemic illness such as celiac, IBD
  • Stimulant meds
  • sometimes hard to distinguish from constitutional growth delay and constitutional thinness
32
Q

Children Born Small for Gestational Age

A

defined as less than -2SD for birth weight or length

33
Q

causes of children born small for gestational age

A
  • maternal: infection, nutritional deficiencies, uterine abnormalities, smoking, alcohol, drugs
  • Placental: previa, abruption, structural, multiple gestation
  • Fetal: chromosomal abnormalities, metabolic, infections, malformations
34
Q

Will children born small for gestational age catch up

A

Most healthy infants born SGA achieve catch-up height by 2 years old –most in first 6 months

  • 10-15% remain short as adults
  • final height may also be compromised by early or rapid puberty
35
Q

pathophys of SGA

A

not sure but perhaps fetal response to prolonged nutritional deficiencies late in gestation may be prematurely reset to a slow growth rate with a degree of resistance to GH, IGF-1, and insulin

36
Q

Treatment in SGA

A

approved in 2001, GH approved for SGA children who have failed to catch-up by 2 years

  • average increase in final height 3 inches after years of treatment–may not have big height improvement
37
Q

do hormonal causes of worrisome growth affect weight

A

generally weight is spared– chubby or weight nl for height

38
Q

ormonal causes of worrisome growth

A

hypothyroidism
growth hormone/IGF-1 abnormalities
Cushing syndrome (usually see if on oral/inhaled steroids not endogenous)
Rickets

39
Q

Hypothyroidism in children

A

generally acquired hypothyroidism

  • many clinical features from adults lacking in children
40
Q

labs for primary vs central hypothyroidism

A
  • primary– low T4, high TSH

- central hypothyroidism- low/nl TSH, low T4

41
Q

Growth Hormone

A

from anterior pituitary; main function to promote linear growth in children
-also affects body composition-increases lean body mass and decreases fat

42
Q

Growth Hormone Deficienc

A

absent or inadequate production of GH

  • continuum – range of GH levels seen
  • may be associated with deficiencies in other pituitary hormones or isolated
43
Q

Congenital GHD

A
  • hypothalamic pituitary malformations
    • Holoprosencephaly (forebrain doesn’t divide), Schizencephaly (abn clefts in brain)
    • Isolated cleft lip or palate
    • septo-optic dysplasia- 50% have hypopituitarism (–born with abnormalities with septum pellucida with optic abnormalities)
    • Optic nerve hypoplasia (alone)
    • Empty sella syndrome (pit there but flattened against sella–often post pit up in hypopthalamus –empty sella and ectopic post pituitary–can have nl pituitary fxn or GH deficiency
44
Q

Acquired GH deficiency

A
  • Trauma
  • CNS infection
  • CNS tumors- craniopharyngioma, germinoma
  • Cranial irradiation after other brain tumors
45
Q

Diagnose GHD

A

abnormal growth velocity with exclusions of other causes

46
Q

how to evaluate babiestoddlers for short stature

A

harder since can be nl or abnormal for babies to cross percentiles

  • look at degree of falloff, parents, etc
47
Q

Other sxs of GHD

A
  • decreased muscle build
  • increased subcutaneous fat, esp around trunk
  • face immature for age
  • prominent forhead, depressed midface (facial bones won’t grow nl but head will)
  • other midline facial defects
  • in males, small phallus
  • may have history of prolonged jaundice or hypoglycemia newborn pd
48
Q

GHD eval

A

bone age
IGF-1 (IGFBP-3-specific but not sensitive so if low will help– in infants since IGF-1 low in infants; also affected by nutrition and will be low in really skinny kid)
-NOT GH since it is pulsatile)
- Stimulation testing (never draw random GH level)- clonidine, arginine, glucagon, L-dopa

49
Q

Do you ever check GH levels

A

NO! pulsatile secretion

50
Q

Stimulation test

A

use clonidine or arginine usually to see if you can stimulate GH

51
Q

Syndromic Short Stature

A

skeletal dysplasias and other genetic syndromes

  • Turner syndrome
  • Prader-Willi syndrome
  • Noonan syndrome
52
Q

Turner syndrome

A
  • Haploinsufficiency of SHOX genes
  • gene important for growth
  • most common sex chromosome abnormality in female
  • complete or partial absence of 1 of X chromosomes
53
Q

Prader-Willi syndrome

A
  • GH deficient plus lots of other things
54
Q

Noonan syndrome

A
  • similar to Turner’s but can affect males and females abnormal GH post-receptor signalling
55
Q

Turner Syndrome phenotype

A

virtually all short; final height about 20 cm shorter than they would have been if untreated

  • sk/growth abnormalities
  • growth pattern– initial drop off
  • short stature, increased carrying angle, short neck, micro or retrognathia (chin)
  • lymphatic obstruction from fetal life– webbed neck, lymphedema, low hairline/trident
  • moles
  • Cardiac abnormalities- bicuspid, coarctation
  • renal-horseshoe kidney, ovarian insufficiency, hypothyroidism/celiac, otitis media, hearing loss, nonverbal learning disability
  • *GONADAL Dysgenesis– need hormone replacement
56
Q

what is the height projection in ekeletal dysplasias

A

shorter than projected

57
Q

Treatment for Turner’s syndrome

A
  • GH can improve growth/final height even though not generally GH deficient
  • start treatment early important to offer best potential for growth
58
Q

Eval of worrisome growth

A
  • Bone age (L hand/wrist to look for growth potential and can help with diagnosis)
  • metabolic panel/phosphorus–RTA/rickets
    -CBC - anemia (chronic dz, skeletal dysplasia)
  • TSH &T4
    -IGF-1 or IGFBP-3
    -Karyotype in girls -Turner
  • TTG and IgA for celiac
    -ESR- IBD
    -
59
Q

Hx GH

A
  • GHfrom beef and made other animals grow but not humans

- used it from cadavers but needed a LOT since only 1 days worth in 1 pituitary

60
Q

Limitations to cadaver GH

A
  • 1 human pit gland to produce enough for 1 day for 1 person
  • some pts develop Ab to GH
  • found to be associated with Creutzfeld-Jacob disease–slow viral infeciton of CNS
61
Q

when was recombinant GH available

A

1985

62
Q

FDA approved uses of GH

A

GHD, Chronic renal insufficiency, adults with GHD, Turner syndrome, Prader Willi, Small for gestational age, idiopathic short stature (conroversial), SHOX deficiency, Noonan syndrome

63
Q

Ethical Issues of GH

A
  • GHD clinical diagnosis since test not perfect
  • deficient response seen without endocrine disease
  • variability in assays
  • intraindividual variability from day to day
  • definition of nl response arbitrary
  • WHo to treat (according to FDA approval, if fam wants to pay, developmentally delayed children, who makes decisions)
  • How Long (until final height reached, until genetic potential, nl height but what is that?, until height no longer disability
  • very expensive–who pays
64
Q

Cost of GH

A

mean cost of $20,000/year; $35,000 per inch of height

65
Q

failure to thrive

A

deceleration in wt gain to below 3% or fall in wt across 2 or more percentiles

DEMS Unit 4 (16 decks)

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