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NGD > Puberty > Flashcards

Puberty Flashcards

1
Q

Normal pubertal onset

A

reactivation of HPG axis: increased nocturnal pulsatile secretion of LH and early morning secretion of gonadal steroid hormones
Appearance of an LH-predominant response during GnRH stimulation testing
usually preceded 2-3 years by a rise in adrenal androgen levels (Adrenarche) which occurs independently of GnRH

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2
Q

FSH/LH testing in childhood

A

Can’t measure since axis is shut down
over time, develop decreased sensitivity to negative feedback –> can ramp up FSH/LH to drive through puberty during adolescence

Can test during mini puberty (less than 6 mo)

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3
Q

Signs of onset of normal puberty in girls

A

85% Thelarche (breast development) first sign
15% pubarche, first outward sign
want to see thelarche first, since pubarche before thelarche is likely pathological

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4
Q

Signs of onset of normal puberty in boys

A

nearly all: gonadarche, testicular enlargement to volume >=4 cc (length >=2.5 cm), first outward sign of puberty
must palpate testicles, pubarche in isolation doesn’t count

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5
Q

Pubertal onset timing for girls

A

White NA girls:

  • thelarche mean 10
  • pubarche 10.5
  • menarche 12.9

Black NA girls

  • thelarche 8.9
  • pubarche 8.8
  • menarche 12.2

Asian girls also thought to mature earlier

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6
Q

Normal pubertal progression in girls

A

peak growth velocity at Tanner 3 (earlier than boys)
mean growth after menarche ~3 cm
breasts, pubes, flow, grow
- may get growth spurt before breasts, since estrogen is potent at the bones

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7
Q

Pubic hair Tanner staging

A 
I: none
II: sparse, pigmented, long, striahgt
III:darker, coarser, curlier
IV: adult, but decreased distribution
V: adult in quantity and type with spread to medial thighs
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8
Q

Breast development Tanner staging

A 
I: preadolescent
II: breast budding
III: continued enlargement
IV: areola and papilla form secondary mound
V: mature female breast
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9
Q

Normal pubertal onset timing for boys

A

96% between 9-14 NA boys
no significant difference between black and white males
first sign: testicular enlargement

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10
Q

Testicular volume

A

prepubertal 2.0 cm, 1-3 cc
early puberty should be around 4 cc
adult 4-6 cm, 15-25 cc

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11
Q

Normal pubertal progression for boys

A

voice breaks around age 13
axillary, facial hair appear at about age 14
gynecomastia frequent at Tanner 4 due to peak in testosterone and peripheral aromatization
peak growth velocity at Tanner 4
continued virilization into late teens

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12
Q

Growth spurt

A

Gonadal steroid hormone induced increase in GH secretion
Peak growth v at 11.5 in girls, 13.5 in boys
~15% adult height achieved in puberty
99% reached at bone age 15 in girls, 17 in boys

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13
Q

Precocious puberty Dx in girls

A

breast budding/pubic hair before 8

menarche befoer 10

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14
Q

Precocious puberty Dx in boys

A

testicular enlargement or pubic hair before 9

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15
Q

Physical examination of precocious puberty

A

follow growth curve
Tanner staging
Isosexual pubertal development (evidence of sex hormone production consistent with sex)
Contrasexual pubertal development - worrisome (clitoromegaly, hirsutism, gynecomastia - for real breast tissue, not fat)
Otehr disease processes?
- CNS disease or abnormalities
- Cafe au lait spots or other neurocutaneous lesions
- endocrine disease

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16
Q

DDx of precocious puberty

A

GnRH-dependent (always isosexual, true, central)

GnRH-independent ( can be iso or contrasexual, peripheral)

17
Q

GnRH dependent precocious puberty

A

central

idiopathic (75% girls, less than 10% boys)

18
Q

CNS lesions associated with central precocious puberty

A

Hypothalamic hamartoma (17-30% girls, 50% boys)
optic glioma (neurofibromatosis)
Craniopharyngioma, dysgerminoma, other tumors
arachnoid/suprasellar cyst
hydrocephalus
congenital defects: myelodysplasia, septo-optic dysplasia
infectious: encephalitis, abscess, meningitis, granuloma
abuse, hemorrhage, trauma, irradiation
tuberous sclerosis

19
Q

Growth in central precocious puberty

A

decreased final height due to premature fusion of epiphyses
deficit not as great as previously thought in many children
adult height lowest in those with earliest onset of puberty
- early diagnosis is important, if therapy to be effective in preserving height

20
Q

Other side effects of central precocious puberty

A

co-existing pathology

Psychological issues

21
Q

GnRH-independent precocious puberty in girls etiology

A

exogenous gonadal hormones (BCPs, estrogen creams)
- massive soy consumption?
Severe primary hypothyroidism (TSH spillover on FSH receptor)
Ovarian tumours/cysts
Adrenal tumours
McCune-Albright syndrome

22
Q

McCune-Albright syndrome

A

precocious puberty, menarche
bony lesions - polyostotic fibrous dysplasia
irregular cafe-au-lait spots
may have other autonomous endocrine tumours - toxic thyroid nodules, pituitary adenomas
may have non-endocrine invovement - hepatobiliary, cardiac

23
Q

GnRH-independent precocious puberty in boys - etiology

A

Untreated CAH
exogenous gonadal hormones (anabolid steroids)
hCG-secreting dysgerminoma/hepatoma (LH receptor)
testicular tumors
adrenal tumors
testotoxicosis (autonomous LH receptor)

24
Q

Premature thelarche

A

may appear at any age, usually younger than 5
common condition of isolated breast development that usually resolves within 6 mo - 1 year
often asymmetrical, may regress
etiology unknown - ?transient ovarian follicle
maybe the first sign of true precocious puberty
may be associated with “smoldering early puberty”
generally benign, if bone age is not advancing

25
Premature adrenarche
usually warrants some investigation increasingly common especially in obese girls may also have BO, axillary hair, acne, hirsutism, acanthosis nigricans (insulin resistance) R/O non-classical or late-onset CAH generally benign if bone age not advancing associated with later development of PCOS in many girls
26
Gynecomastia
65% of normal boys at Tanner 4 resolves in 75% within a year more noticeable in obese boys consider possibility of Klinefelter: palpate small testicles Also consider medications, marijuana - usually affect liver function so estrogen levels increase
27
Precocious puberty: investigations before referral
measure height and growth velocity carefully bone age TSH LH, FSH, DHEAS, estradiol or testosterone - not always helpful All hormone levels should be obtained as early in day as possible (pulsatile secretion)
28
Precocious puberty: investigations at endocrinologist
GnRH stim test, look for rise of LH to >=6-8U/L (normal unactivated axis should not respond) If central precocious puberty: MRI head Other tests as indicated: - Hormones: testosterone, DHEAS, estradiol - tumors, CAH: 17-OHP, DHEAS, androstenedione, renin, aldosterone - Boys: betahCG - US: abdominal, testicular, pelvic _ Cushing: 24h urine free cortisol - Contrasexual development: karyotype
29
Treatment of CPP consideration
``` Patient's age and Tanner stage rate of pubertal development bone age advancement and predicted adult height coexisting pathology compelling psychosocial considerations ```
30
DevoProvera
CPP | Injections can be given to control menstrual periods and some sexual behaviours without affecting adult height
31
GnRH agonists
GnRH stimulates gonadotropin secretion if given in PULSES CONTINUOUS: inhibition of gonadotropin secretion analogues are true agonists of natural GnRH with substitutions at 6th aa inhibit gonadotropin secretion because of prolonged half-life can halt pubertal development and menses absolute indication still not clear may cause some regression of secondary sex characteristics Appears to increase final height most in those who start puberty earliest and those wiht most advanced bone age not currently recommended for use in girls who begin puberty at age 6-8 with slowly progressive puberty and/or acceptable predicted adult height
32
Leuprolide acetate
The only GnRH agonist licensed for treatment of CPP in Canada IM q3-4 weeks requries frequnet physical monitoring of growth/bone age, pubertal progression/regression, predicted adult height
33
Delayed puberty dx in girls
no breast development by age 13 or no menarche by age 16 | absence of menarche within 5 years of pubertal onset
34
Dx delayed puberty in boys
no increase in testicular length (>2.5 cm, volume 4 cc) by age 14
35
DDx of delayed puberty
Hypergonadotropic: - ovarian/testicular dysgenesis: Turner, Klinefelters - Gonadal toxins: chemo/radiotherapy - Enzyme: 17alpha hydroxylase, 177 ketosteroid reductase - androgen insensitivity Hypogonadotropic: - multiple trophic hormone deficiencies/isolated GH deficiency - Kallman: isolated GnRH deficiency - systemic conditions, nutrition and psychogenic, increased energy expenditure - other endocrine causes: hypothyroidism, GC excess, hyperPRL - Constitutional (CDGA)
36
Investigations of delayed puberty before referral
measure height and growth velocity bone age TSH/possibly fT4, prolactin LH, FSH helpful if elevated (hypergonadotropic hypogonadism) Karyotype as indicated for Turner, Klinefelter
37
Treatment of CDGA considerations
Considerations: age, Tanner stage coexisting pathology psychosocial
38
Tx of CDGA boys
best - watchful waiting coupled with explanation of wide variation of pubertal development 6 mo course of low dose depot testosterone will increase growth rate by ~75%, and advance pubic hair by one stage, without deleterious effect on height potential usually not initiated before age 13 (minimum bone age 11) otherwise puberty will not be sustained
39
Tx of CDGA girls
minimum bone age 10 to initiate therapy most start at ethinyl estradiol at 2.5 microg/d with max dose of 5 usually not initiated befoer age 13 (minimum bone age 11) - otherwise puberty will not be sustained

NGD (16 decks)

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