ICL 2.4: Normal Puberty/Disorders of Puberty/Precocious Puberty Flashcards

1
Q

a 10 year old boy has started puberty. these changes pubic hair growth, testis growth and body growth spurt. what is the usual progression?d

A

testis growth –> hair growth –> body growth spurt

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2
Q

an 8 year old girl has started to exhibit changes of puberty including pubic hair growth, menarche, breast growth and body growth spurt. what is the normal sequence?

A

breast growth –> pubic hair –? body growth spurt –> menarche

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3
Q

do you treat precocious puberty?

A

not always!

treat if it’s pathologically caused or if it’s related to adult impairment

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4
Q

does sexual abuse effect puberty?

A

there are increased reports that precocious puberty is associated with child sexual and physical abuse

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5
Q

when do boys vs. girls get referred for delayed puberty?

A

girls with delayed puberty are referred fro evaluation less frequently than boys with delayed puberty

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6
Q

4 year old girl with acne since age 3. rapid growth compared to twin brother. 1st tooth in at 4 months old.

what things would you ask?

A

premature adrenarche

  1. parent height and puberty pattern –> mom is 5’6”with menarche at 10, dad is 6’with normal puberty
  2. past medical history –> N/A
  3. family history –> N/A
  4. growth chart
  5. bone age –> look at hand bones to look at patterns of fusion and calcifications to determine how old the bones are
  6. labs –> LH, FSH, testosterone normal but DHEA-S is mildly elevated
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7
Q

when do boys usually stop growing?

A

16.5 years old

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8
Q

what is premature adrenarch?

A

benign for most kids

can present as acne, body odor, axillary hair or pubic hair without other signs of puberty

no treatment needed

just in case though you need to asses for benign adrenal hyperplasia that could be causing the elevated DHEA like 21-alpha-hydroxylase deficiency

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9
Q

what does increased weight gain in kids indicate?

A

increased weight gain is associated with increased height velocity

in true precocious puberty you have accelerated growth!

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10
Q

what is the presentation for congenital adrenal hyperplasia?

A
  1. baby boy born full term presents at 1-2 weeks old with jaundice, colic and lethargy

state newborn screen positive for elevated 17OHP

  1. 6 year old boy with pubic hair development, normal prepubertal testes without masses, height is 90%, bone age is 12.2 years and testosterone levels in the low adult range, 17-alpha-hydroxyprogesterone was markedly elevated
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11
Q

how does lavender oil effect puberty?

A

can cause elevated estrogen and result in premature thelarche aka breast development

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12
Q

what is the peak growth veolicty in males vs females?

A

13-14 in males

11-12 in females

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13
Q

what hormones controls growth?

A

growth during childhood depends o thyroid hormone and GH

the adolescent growth spurt depends on estradiol and GH levels in both genders

estradiol first promotes growth by acting on bone and increasing GH

then they get so high they cause closure of the epiphyseal growth plates which ends growth

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14
Q

what is delayed puberty?

A

absence of secondary sexual characteristics by an age at which 95% of children have initiated sexual maturation

13 years old without breast development for girls and 14 without testicular enlargement for boys

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15
Q

what is hypogonadotropic hypogonadism?

A

delayed puberty with low LS and FSH

usually self correcting, aka late bloomers

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16
Q

what can cause hypogonadotropic hypogonadism?

A
  1. damage to hpothalamus/pituitary

2. hypothyroidism, hyperprolactinemia, hypercortisolism

17
Q

what is acquired hypogonadotropic hypogonadism?

A

anything that prevents energy from being available for growth

can be caused by anorexia, malnutrition, excessive exercise with decreased body fat, celiac disease, malabsorption, chronic illness

18
Q

what is congenital hypogonadotropic hypogonadism?

A

can co-occur with other endocrinopathies like malformation of the hypothalamus/pituitary glands

19
Q

what is isolated congenital hypogonadotropic hypogonadism?

A

when only the gonadal axis is effected

twosbtypes:
1. normosmic IHH caused by GnRH signaling and secretion defects

  1. Kallmann syndrome with anosmia

can be caused by a mutation in a gene that normally protects GnRH neurons from apoptosis during development

20
Q

what are the causes of delayed puberty with high LH and FSH?

A
  1. sex steroid receptor deficiencies or aromatase deficiency
  2. damage or malformation of gonads
  3. Turner syndrome/gonadal dysgenesis (XO)
  4. Klinefelter’s syndrome (XXY)

treat with E2/T if possible to promote 2ndary sexual characteristics, normal growth and proportions

21
Q

what is delayed puberty with normal LH and FSH?

A

mullein duct genesis

leads to blind vaginal pouch, no upper vagina cervix or uterus

no menses

ovary normal so secondary sexual characteristics and gonadotropin levels are normal

22
Q

what is precocious puberty?

A

signs of puberty before 8 in girls and 9 in boys

associated with reduced adult height due to early epiphyseal closure

treat with GnRH long acting analog – constant exposure to GnRH downr egulates the receptor – improves the final heigh for some

23
Q

what is peripheral precocious puberty?

A

low Lh

aka pseudo puberty

appearance of pubertal development independent of pituitary gonadotropins

causes:
1. congenital adrenal hyperplasia

  1. tumors that secrete hCG
  2. tumors of the adrenal glands or gonads
  3. exposure to exogenous sex steroids
24
Q

what is central precocious puberty?

A

high LH because the HPG axis wakes up early

often idiopathic/genetic causes like activating mutation in genes encoding kisspeptin and its receptor

can result from CNS neoplasms, injuries, infections, seizures

exposure to exogenous sex steroids primes the system and can start a positive feedback look

25
Q

how does obesity effect puberty?

A
  1. adipose tissue has aromatase action = more estrogens

2. insulin resistance lowers SHBG levels

26
Q

what do people speculate causes earlier menarche over the decades?

A
  1. overall health
  2. increased body weight
  3. endocrine disruptors in the household