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37 Cards in this Set
- Front
- Back
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what is short stature?
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Short stature is a standing height more than 2 SD below the mean for gender.
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in your history taking for a child who is "short" what is an important assessment to make?
*yellow |
Assess child’s feelings regarding his/her stature
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Bone age is linked to what?
A) sexual maturity rating B) chronological age |
A) sexual maturity rating
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Constitutional Growth Delay
Chronic Illness Malnutrition (months/years) Endocrinopathies Psychosocial Deprivation are causes for what? |
Delayed bone age
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What is familial short stature?
What are their pubertal growth spurts like? Does bone age=chronological age? |
short parents have short kids (70-80%)
Pubertal growth spurts are normal in timing and magnitude. Bone age=Chronological age |
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What is constitutional growth delay?
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Bone age is delayed 2-4 years.
LATE BLOOMERS bone age is consistent with height and sexual maturity rating rather than chronological age usually has 1st degree relative with constitutional delay |
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A 12 year old boy is on the 5th percentile for height and the 10th for weight. Both his parents are below average for height. Which of the following is likely true?
Puberty will be delayed His bone age will be delayed Bone age>chronological age Pubertal growth spurt will occur on time |
Pubertal growth spurt will occur on time
this is a kid with familial short stature! -he is small, with small parents Bone age=chronological age |
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A 12 year old boy is on the 5th percentile for height and the 10th for weight. Both his parents were below average for height, but are now within the normal range. Which of the following is likely true?
Puberty will be delayed His bone age will be delayed Bone age>chronological age Pubertal growth spurt will occur on time |
His bone age will be delayed
this is constitutional growth delay |
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GH is released in what fashion? when?
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pulsatile
Most likely during stage III and IV (non-REM) sleep |
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Sleep
Exercise Physical stress Puberty Fasting (short term) will do to what to GH? |
Stimulate its release
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Hyperglycemia
Hypothyroidism Glucocorticoids do what to GH? |
inhibit GH release
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GH produces growth by generating what?
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IGF (primarily in the liver)
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in addition to producing growth, what else does IGF do (along with GH)?
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exerts negative feedback on the pituitary and hypothalamus (stimulate somatostatin inhibition)
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what will the bone age be like in pts with GH deficiency?
*yellow |
delayed bone age
determine by low levels of IGF 1 |
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there are 4 populations in which GH is approved for as tx...what are they
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GH deficiency
Chrnoic renal insufficiency Turner Syndrome Familial short stature with considerable anxiety in pt |
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Major side effect of GH therapy?
*yellow |
INCREASED DEVELOPMENT OF MALIGNANCIES- so GHT is contraindicated in a patient with a history of a malignancy!!!
others: Insulin resistance Mild sodium and water retention Transient lowering of thyroxine levels Scoliosis Gynecomastia Slipped capital femoral epiphysis |
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hypersecretion of GH in puberty? post puberty?
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Prepuberty: gigantism
Postpuberty: acromegaly—bony and soft tissue overgrowth |
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Osteochondroplasias affect what kind of bone?
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long
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What is achondroplasia?
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Most common non-lethal chondrodysplasia
Autosomal dominant (but may arise from spontaneous mutation) Mutation inhibits linear bone growth DWARFISM |
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what is Diastrophic Dysplasia
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abnormal shape and twisting of the bones
clubfoot is common, phalanges are irregular, many complicatoins are orthopedic |
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most common genetic disorder associated with growth retardation?
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Down syndrome
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the majority of people with Turner's syndrome are mosaic..this means that...
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their phenotype is not entirely XO...so their attributes may not look like the textbook
in fact, many true XOs are aborted |
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What is the key to diagnosis for Turner syndrome?
*yellow |
Short stature
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Webbed neck, peripheral edema, increased carrying angle, ovarian failure-
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Turners
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Short adolescent females with amenorrhea should all have what done? Why?
*YELLOW STAR |
Karyotype
looking for XO Turners |
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Hypothyroidism can be a secondary cause of growth disorders, what will a pt's bone age be like?
*yellow |
DELAYED
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cortisol will do what to growth?
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stunt it...
exogenous--oral, topical inhalant endogenous--CUshing disease (in yellow) note: will have delayed bone age (in yellow) |
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What is psychosocial dwarfism? tx?
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Disturbed eating and sleeping patterns develop in disturbed families.
Children may develop transient pituitary hormone deficiencies. Delayed bone age Following removal from the adverse environment, hormone levels quickly normalize and catch up growth occurs. |
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A 14 y.o. is concerned because he’s shorter than his classmates. On exam he has the sexual maturity rating of a 12 year old. His dad says he had a similar problem, but grew out of it by the time he reached college. Which of the following would you expect?
Abnormal thyroid function Delayed bone age Bone age=chronologic age Abnormal karyotype |
Delayed bone age
he has constitutional growth delay |
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Which of the following is the key to diagnosis of Turner Syndrome? (besides genetics)
Increased carrying angle Webbed neck Short stature Absence of secondary sexual characteristics All of the above |
Short stature
all of the above COULD be there but short stature is ALWAYS there |
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What is the definition of short stature?
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standing height more than 2 SD below the mean for gender
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How is bone age determined?
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AP x ray of hand/wrist --> send to radiologist who determines epiphyseal maturation by comparing it to published standards
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Bone age is linked more closely with sexual maturity rating or chronological age?
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sexual maturity rating
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Why do we measure IGF1 to diagnose GH deficiency? Why can't we just measure GH?
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You can't just willy nilly measure GH because it is released in a pulsatile fashion and has a short half life. We use IGF because it is a direct reflection of GH levels and has a longer half life.
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What genetic mutation is associated with Achondroplasia?
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FGRG3 (fibroblast growth factor receptor)
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Child presents with a flat, puffy face and a huge tongue. Blood work shows decreased circulating T3 and T4. What is wrong?
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Cretinism!
hypothyroidism in children |
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Radiologically, how could you diagnose congenital hypothyroidism?
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Absence of distal femoral epiphysis or epiphyseal dysgenesis
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