Shared Flashcard Set

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Abnormal Development
fd
17
Business
Undergraduate 4
03/02/2011

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Cards

Term
T/F: TH, sex steroids and GCCs all promote growth
Definition
F: Thyroid – essential for normal growth
- hypothyroidism is a common cause of severe growth delay
Sex steroids – bone maturation is dependent on estrogen
- testosterone can enhance GH secretion
Glucocorticoids – potent inhibitor of growth
Term
A mother brings her child in concerned about height saying he is too small. Your plot of his growth over the past 4 months shows a normal growth velocity. Does he have a hormonal problem?
Definition
Can't tell b/c GV should be measured over at least a 6-12 month period
more the # of height points used to calculate GV –
more reliable is the interpretation
assessment of pubertal status is critical
for interpretation of GV
Normal GV – is a strong argument AGAINST a
significant hormonal abnormality
Term
a 10yo kid has a younger "bone age" as shown by an x-ray of his left wrist. what does this mean?
Definition
he has a longer time left to grow.


Usefulness - prediction of final height
- age of onset of puberty closely linked to bone age
- corroborates diagnosis, but is never diagnostic
Caveats – imprecise / ethnic variability
Term
What are GV, bone age and chronicological age differences in familial/genetic short stature vs. late bloomers
Definition
F/G: final ht appropriate for parental ht
normal size at birth
GV may be  in 0-3 yrs of age
BA = CA

Constitutional delay:

family history
normal size at birth
normal GV
delayed puberty
BA < CA

BUT F/G IS A DIAGNOISIS OF EXCLUSION!!!!!!
Term
A child with Psychosocial Dwarfism
Emotional Deprivation Syndrome

can be expected to have what type of short stature?
Definition
proportionate pathological SS
Term
Causes of impaired GV
Definition
Malnutrition
Chronic systemic illness
IUGR
Psychosocial
Chromosomal abnormalities
Endocrine
Malabsorption
Bone dysplasias
Term
You run labs looking for abnormalities in:

CBC, ESR, BUN, IGF-1, IGFBP3

What did you exclude?
What karyotype abnormalities are you looking for?
Definition
Tissue Transglutaminase ab
FT4, TSH


KARYOTYPE
in girls to exclude TURNER
dysmorphic features
Term
You have a patient with Delayed dentition / mid-facial hypoplasia
Increase in adiposity

what other features should you look for in GH deficiency?
Definition
Decreased growth velocity

history of neonatal problems (Neonatal – normal size / hypoglycemia / jaundice / micropenis /
midline defe
Term
Which genetic defects are associated w/ GHD?
Definition
Genetic Defects –
Isolated Growth Hormone Deficiency (IGHD)
PROP1 / POU1F1 (Pit1)
Term
what can a craniopharyngioma cause?
Definition
GHD
Term
QUIZ: Diagnosis of GHD - made clinically
Definition
Clinical (NOT laboratory) diagnosis
GV < 2 SD
Low IGF-1 & IGFBP-3
Provocative GH Level < 7-10 ng/ml

Supporting evidence: Delayed BA
Related pathology
Term
how can we provoke GH release?
Definition
Insulin induced hypoglycemia is the “Gold standard” after overnight fast...but it's hard to do and risky....

pulsatility of GH makes it harder to do random measureentns
Term
What do IGF-1 / IGFBP3 levels depend on?
Definition
nutritional status, pubertal status and age
Term
Treatment
Indications for GH Therapy
Definition
Growth hormone deficiency
Turner syndrome
Renal disease, before transplant
Small for gestational age
Prader–Willi syndrome
Idiopathic short stature
Term
what are bad side effects of the GHRT and what do you need to be monitoring for besides the labs?
Definition
Secondary/tertiary hypothyroidism
Worsening of scoliosis
Slipped capital femoral epiphysis
Pseudotumor cerebri so make sure measure ICP
Term
young girl has clinodactyly, webbing of neck, and inc carrying angle. What's her lab abnormality?
Definition
45X karyotype
low GV
normal IGF-1/BP3 and normal BA
Term
2 most common features in turner's?
Definition
Growth Failure 80-100%
Gonadal Dysgenesis 80-100%
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