ENDO_TEST 1: Martin_Growing Up Flash Cards

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Title:	ENDO_TEST 1: Martin_Growing Up
Description:	RT
Number of Cards:	37
Save Count:	0
Author:	dabunn9
Created:	2012-01-18
Tags:	dabunn9 endo wvsom
Private	No

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- Question
- Answer
- Side 3

- what is short stature?
- Short stature is a standing height more than 2 SD below the mean for gender.
- 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
- Bone age is linked to what?
  
  A) sexual maturity rating
  
  B) chronological age
- A) sexual maturity rating
- Constitutional Growth Delay
  Chronic Illness
  Malnutrition (months/years)
  Endocrinopathies
  Psychosocial Deprivation
  
  are causes for what?
- Delayed bone age
- 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
- What is constitutional growth delay?
- 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
- 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
- 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
- GH is released in what fashion? when?
- pulsatile
  
  Most likely during stage III and IV (non-REM) sleep
- Sleep
  Exercise
  Physical stress
  Puberty
  Fasting (short term)
  
  will do to what to GH?
- Stimulate its release
- Hyperglycemia
  Hypothyroidism
  Glucocorticoids
  
  do what to GH?
- inhibit GH release
- GH produces growth by generating what?
- IGF (primarily in the liver)
- in addition to producing growth, what else does IGF do (along with GH)?
- exerts negative feedback on the pituitary and hypothalamus (stimulate somatostatin inhibition)
- what will the bone age be like in pts with GH deficiency?
  
  *yellow
- delayed bone age
  
  determine by low levels of IGF 1
- there are 4 populations in which GH is approved for as tx...what are they
- GH deficiency
  
  Chrnoic renal insufficiency
  
  Turner Syndrome
  
  Familial short stature with considerable anxiety in pt
- 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
- hypersecretion of GH in puberty? post puberty?
- Prepuberty: gigantism
  
  Postpuberty: acromegaly—bony and soft tissue overgrowth
- Osteochondroplasias affect what kind of bone?
- long
- What is achondroplasia?
- Most common non-lethal chondrodysplasia
  Autosomal dominant (but may arise from spontaneous mutation)
  
  Mutation inhibits linear bone growth
  
  DWARFISM
- what is Diastrophic Dysplasia
- abnormal shape and twisting of the bones
  
  clubfoot is common, phalanges are irregular, many complicatoins are orthopedic
- most common genetic disorder associated with growth retardation?
- Down syndrome
- the majority of people with Turner's syndrome are mosaic..this means that...
- their phenotype is not entirely XO...so their attributes may not look like the textbook
  
  in fact, many true XOs are aborted
- What is the key to diagnosis for Turner syndrome?
  
  *yellow
- Short stature
- Webbed neck, peripheral edema, increased carrying angle, ovarian failure-
- Turners
- Short adolescent females with amenorrhea should all have what done? Why?
  
  *YELLOW STAR
- Karyotype
  
  looking for XO Turners
- Hypothyroidism can be a secondary cause of growth disorders, what will a pt's bone age be like?
  
  *yellow
- DELAYED
- cortisol will do what to growth?
- stunt it...
  
  exogenous--oral, topical inhalant
  
  endogenous--CUshing disease (in yellow)
  
  note: will have delayed bone age (in yellow)
- What is psychosocial dwarfism? tx?
- 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.
- 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
- 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
- What is the definition of short stature?
- standing height more than 2 SD below the mean for gender
- How is bone age determined?
- AP x ray of hand/wrist --> send to radiologist who determines epiphyseal maturation by comparing it to published standards
- Bone age is linked more closely with sexual maturity rating or chronological age?
- sexual maturity rating
- Why do we measure IGF1 to diagnose GH deficiency? Why can't we just measure GH?
- 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.
- What genetic mutation is associated with Achondroplasia?
- FGRG3 (fibroblast growth factor receptor)
- Child presents with a flat, puffy face and a huge tongue. Blood work shows decreased circulating T3 and T4. What is wrong?
- Cretinism!
  
  hypothyroidism in children
- Radiologically, how could you diagnose congenital hypothyroidism?
- Absence of distal femoral epiphysis or epiphyseal dysgenesis