ERM2 - Growth and Puberty

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Author:
jknell
ID:
184598
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ERM2 - Growth and Puberty
Updated:
2012-11-20 01:15:21
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Endocrine Reproductive Pathology
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Endocrine and Reproductive Pathology
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  1. Arm Span
    -arm span should be within 2cm of height

    • -short arm span = epiphysial dysplasia
    • -long arm span = spinal dysplasia
  2. Upper to Lower Segment Ratio
    -abnormality could indicate skeletal dysplasia

    • -measure height and upper segment
    • -birth ratio is 1.7 and decreases until puberty
  3. Mid-Parental and Target Height
    •Girls: ((Mom’s height + Dad’s height) - 5 inches)/2

    •Boys:((Mom’s height + Dad’s height) + 5 inches)/2

    Target height is +/- 4 cm of midparental height
  4. Bone Age
    • -xray of L wrist to determine stage of epiphyseal fusion
    • -can be used to estimate a predicated height
  5. Causes of Short Stature
    • 1. Normal
    •      -Constitutional Delay
    •      -Familial Short Stature

    • 2. Pathologic
    •      -Endocrine
    •      -Genetic Syndrome
    •      -IUGR and SGA
    •      -Chronic diseases and malnutrition
  6. Constitutional Delay
    • -more common in boys
    • -infants are normal birth length and weight
    • -growth deceleration occurs by 12-24 months
    • -bone age is delayed
    • -growth velocity is normal
    • eventually return to normal height
  7. Familial Short Stature
    • -deceleration in height seen around 6-12 mo
    • -wt tends to be normal for age
    • -bone age is normal
    • -height is concordant with genetic potential
    • -normal growth velocity
    • eventually reach target height, although that is short
  8. Normal Growth Physiology: Endocrine Factors
    • -GH and IGF1 (+)
    • -Thyroid hormone (+)
    • -Estrogen
    •      -Growth Spurt (+)
    •      -Epiphyseal Fusion (-)
    • -Glucocorticoid Excess (-)
  9. Normal Growth Physiology: Non-Endocrine Factors
    • -Genetic
    • -IUGR and SGA (-)
    • -malnutrition (-)
    • -chronic disease (-)
    • -psychologic stress/maternal deprivation (-)
  10. GH physiology
    • -GH release from the anterior pituitary controlled by stimulatory GHRH and inhibitory Somatostatin

    -Stimulated by: deep sleep, alpha adrenergic, fasting, exercise, ACh, sex steroids, AAs, hypoglycemia

    -Inhibited by: obesity, beta adrenergic, glucocorticoids, high FFA, hyperglycemia, hypothyroidism, IGF1
  11. Endocrine Causes of short stature
    • -Growth Hormone Deficiency
    • -Hypothyroidism
    • -Glucocorticoid Excess
  12. Growth Hormone Deficiency
    • -prevalence: 1/4000 - 1/80,000
    • -frontal bossing, depressed nasal bridges ("cherubic faces")
    • -isolated GH deficiency usually presents after toddler years

    -diagnosis: poor growth, hypoglycemia, microphallus, hx of brain irradiation or trauma

    -measure IGF-1 or IGFBP-3

  13. Hypothyroidism
    • -significant short stature with delayed bone age
    • -other sx: apathy, lethary, coarse features/hair, cretinism
    • -screen with TSH
  14. Glucocorticoid Excess
    • -Endogenous: Cushing's Disease
    • -Exogenous: steroid meds
  15. Non-Endocrine Causes of Short Stature
    • -Turner's Syndrome
    • -IUGR and SGA
    • -Chronic Disease (CV, Renal, CF, Celiac, IBD, anorexia)
    • -Psychosocial dwarfism/maternal deprivation
  16. Turner Syndrome
    • -45XO (gonadal dysgenesis)
    • -1/2000 births
    • -Signs and symptoms:
    •      -streak gonads and infertility
    •      -webbed neck
    •      -epicanthal folds
    •      -broad chest
    •      -cubitus valgus
    •      -peripheral lymphedema
    •      -aortic coarctation
    •      -reanl abnormalities

  17. Mini Puberty at birth
    • -brief spurt of central puberty axis activity occurs at birth
    • -spontaneous GnRH pulses recur during the 1st month of life
    • -males: decreases by 3-6 months
    • -females: decreases by 18 months
  18. Pre Pubertal Pause Mechanism
  19. Normal Puberty
    • -reactivation of nocturnal pulses of GnRH secretion
    • -adrenarche is independent of GnRH axis
  20. LH
    -stimulates Leydig cells in testes to produce testosterone

    -stimulates theca cells in ovaries to produce androgen and stimulates ovulation
  21. FSH
    -stimulates sertoli cells in testes to proliferate

    -stimulates granulosa cells in ovaries to produce E2
  22. Sex Steroid Synthesis in adrenal glands and gonads
  23. Testosterone
    • -produced by Leydig cells
    • -peripheral conversion of androsteindione frome testes and adrenal glands
    • -metabolized to DHT by 5 alpha reductase

    • Causes:
    • -pubic and axillary hair
    • -acne
    • -voice change
    • -spermatogenesis
    • -increased muscle mass
    • -bone maturation
  24. DHT
    • Causes
    • -facial hair
    • -penile enlargement
  25. Estradiol
    • -produced by granulosa cells in the ovary
    • -peripheral aromatization of adrenal androstendione and testosterone

    • Causes:
    • -breast development
    • -uterine growth
    • -vaginal secretions
    • -advances bone age
    • -closes epiphyseal plates
    • -increases bone density
  26. Normal Pubertal Onset
    • -Thelarche is first sign in females
    • -8-13 years

    • -Testicular enlargement is first sign in males (4ml or 2.5cm)
    • -9-14 years
  27. Normal sequence of changes in female
    • -breast buds
    • -pubic hair
    • -growth spurt
    • -menarche
  28. Normal sequence of changes in males
    • -testicular growth
    • -pubic hair
    • -penis growth
    • -spermarche
  29. Precocious Puberty
    • Female < 8
    • Male < 9
  30. Delayed Puberty
    • Female > 13
    • Male > 14
  31. Precocious Puberty Causes
    • 1. GnRH dependent or central
    • 2. GnRH independent or peripheral
    • 3. Benign premature thelarche
    • 4. Premature adrenache
  32. GnRH dependent or Central PP
    • -idiopathic
    • -usually females
    • -CNS tumor (hypothalamic hamartoma)
  33. GnRH Independent or Peripheral PP
    • -adrenal: CAH or tumor
    • -Gonads: tumors

    • -exogenous E or T
    • -phytoestrogens (lavendar, tea tree oil)
  34. Benign Premature Thelarche
    • -onset 3-12mo
    • -only breasts, no pubic hair, normal growth velocity and bone age
    • -30% will regress
  35. Premature Adrenarche
    • -no breasts
    • -no virilization or growth spurt
    • -normal bone age
    • - <8 years
    • -rule out CAH
    • -may be a precursor to PCOS
  36. Causes of Delayed Puberty
    • 1. Central Hypogonadism
    • 2. Gonadal Dysfunction
  37. Central Hypogonadism
    • -congenital: Kallman's syndrome
    • -Acquired: CNS tumor, trauma, irradiation
  38. Gonadal Dysfunction
    1. Congenital: tumor, Klinefelter, Cryptorchidism, anorchia, 17 OHase deficiency

    2. Acquired: testicular injury or infection, autoimmune

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