Delayed and precocious puberty

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ICS pg 71 to 78

Signs of secondary sexual charcteristics before what age is abnormal, In females? in males? **

-- Female < 8y/o
-- Male < 9.5
Note: Typically occurs between 8 and 13 years of age
Note: 2.5 SD earlier than normal is pre-cocious

Define gonadarche:

-- growth and maturation of the gonad.
-- Associated with increased sex steroid production and spermatogenesis/folliculogenesis

Define Adrenarche:

-- maturation of adrenal cortex
-- with increased production of adrenal androgens (DHEAS, Androstendione).

Do gonadarche and Adrenarche always happen together?

NO THESE EVENTS ARE INDEPENDENT
Note: Those 2 events totally random - can have isolated adrenarche and gonad development proceeds at normal rate.

What is the median age of menarche? **

12.8

When, on average does thelarche happen wrt menarche? ***

-- On avg. thelarche precedes menarche by 3 yrs.
Note: with menarche ~12.8 --> thelarche ~9.8y/o

When does the Maximum growth spurt (PEAK GROWTH) occur? ***

occurs about 2 years after thelarche and 1 yr prior to menses

What are the ages that puberty typically occurs:

Typically occurs between 8 and 13 years of age

What is precocious puberty:

-- development of secondary sexual characteristics prior to:
* age 8 in girls
* and 9.5 in boys

What is delayed puberty:

-- age 13 without secondary sexual characteristics or
-- age 15 with secondary sexual characteristics (Boys age 14 without testicular enlargement)

What is a mnemonic for the phases of puberty / sexual development in girls:

-- Boobs, pubes, grow and flow. (In medical lingo that's thelarche, adrenarche, growth spurt, and menarche.)
-- Also GRaB PAM: Breasts (thelarche), pubarche, adrenarche, menarche

How does the timing of puberty differ in african americans

African americans earlier by about a year.
Less melanin delays the onset of pubertal development.

What is the Role of Total Body Fat in Timing of Puberty (leptin)

-- Critical body weight must be reached to achieve menarche.
Note: Leptin
*proposed hormone responsible for initiation and progression of puberty
*produced by adipocytes
*serum leptin concentration correlated to body fat content

What is the growth potential after menarche?

*limited growth potential after menarche 4-6 cm
Note:
Average girl reaches growth peak 2 years after breast budding and one year prior to menarche - KNOW THIS

What mineral is important during puberty:

*half total body calcium laid down during puberty.
Note: calcium supplementation during adolescence results in increase bone density

What is tanner stage 1:

prepubertal

What is tanners stage 2:

-- small breast mound
-- sparse pubic hair

What is tanner stage 3:

-- breasts grow some
-- more pubic hair

What is tanner stage 4:

-- areola sticks out from breast
-- adult pubic hair on mons only

What is tanner stage 5:

-- areola recesses
-- adult pubic hair in quantity and distribution

What is considered precocious puberty wrt menarche: **

Onset of menarche prior to age 10

What is central / true precocious puberty?

GnRH dependent = central = true
Premature activation of the axis
Sequence of development typically normal just occurs earlier
Note: girls usu ideopathic; boys usu CNS lesion
Note:
*Central PP: HPO will have LH in response to GnRH stim
*Noncentral PP: HPO will have FSH in response to GnRH stim
Note: Treat with GnRH agonists to halt progression of puberty this is to allow for emotional development and preserve adult height.

What is peripheral = pseudo precocious puberty?

GnRH independent = peripheral = pseudo
Note: Exogenous sex steroids
Endogenous production of steroids
Ovarian Cysts: granulosa cell tumor most common.
Ovarian Neoplasms
Feminizing adrenal tumors
McCune-Albright Syndrome
Thyroid Dysfunction (primary hypothyroidism)

Note:
*Central PP: HPO will have LH in response to GnRH stim
*Noncentral PP: HPO will have FSH in response to GnRH stim

What is Isosexual precocious puberty:

Isosexual-development of sexual characteristics appropriate for gender

What is Heterosexual/Contrasexual precocious puberty:

Heterosexual/Contrasexual-development of sexual characteristics inappropriate for gender e.g. virilization in a female.

When is advanced skeletal age considered abnormal?

Advanced skeletal age = 2 years greater than chronological age is abnormal

What can you use to determine GnRH pulsatility:

Really important to look at LH (correlates to GnRH pulsatility)

What is the most serious side effect of abnormal pubertal development:

The most serious side effect is short adult stature

What congenital CNS lesions can cause precocious puberty?

-- Congenital CNS lesions
Hypothalamic Hamartoma: para- or intrahypothalamic. No growth or metastasis.
-- Optic Gliomas
Note: do MRI

What acquired CNS lesions can cause precocious puberty?

Acquired CNS disorders
Post inflammatory, Post XRT, abscess, hydrocephalus, trauma, tumor
Note: do MRI

What are you thinking:
Polyosthotic fibrous dysplasia (bone cysts /bony fibrous dysplasia, )
Irregular cutaneous pigmentation (café au lait spots,
Precocious puberty
Autonomous early production of estrogen by the ovaries, low FSH/LH
G-protein-cAMP-kinase defect
Gene mutation leads to gain of function of G protein with constitutive activation of endocrine glands
Treatment: Aromatase inhibitors (Antiestrogens) or antitestosterones in males
Bisphosphonate therapy may benefit bone dz

McCune-Albright Syndrome (5% of all cases of precocity)
GnRH Independent
Always on exams.
* Bony cysts
* Café au lait
* Precocious puberty.

What are you thinking about:
autosomal dominant, assoc with mucocutaneous pigmentation, GI polyps, and sex cord tumors (multifocal and bilateral: granulosa or Sertoli cell tumors), at risk for colon, stomach, small intestine, breast, and pancreatic cancer

-- Peutz-Jeghers
Peutz-Jeghers: - they like to ask Qs about this
* GI polyps
* Sex chord tumors
* Colon stomach, small intestine and panc cancers increase.

What is Precocious Puberty Pseudopuberty

-- Premature thelarche
-- Premature adrenarch
-- Premature menarche
No treatment is indicated
Isolated pubertal development
Diagnosis of exclusion and prolonged follow up maybe necessary to ensure this is not the initial sign of true precocity
Follow closely b/c you can get isolated development and it doesn't mean anything - if isolated just follow; don't do a biopsy and destroy normal breast in a 2 year old when nothing is actually wrong.

What is the typical age range for Isolated Premature Thelarche

Typically between 9 - 18 months of age
Unilateral or bilateral
Note: LH concentrations normal (nl GnRH stim response) normal growth velocity
Usually regresses spontaneously, Important not to biopsy permanent damage
Serial follow up

What is the Most common cause of Isolated Premature Menarche

-- Most common: functional ovarian cyst
Note: Vaginal bleeding at inappropriate time in absence of other secondary sex characteristics
Consider: tumor, trauma, foreign body, abuse, infection

What drugs cause gynecomastia:

-- marijuana,
-- isoniazid,
-- spironolactone,
-- ketoconazole,
-- cimetidine

When is premature adrenarche: for girls? for boys?

Premature Adrenarche: prior to age 8 in girls (9.5 in boys), pubarche, axillary hair, and apocrine odor (but appropriate skeletal maturation)
Note: Associated with increased body wt, insulin resistance, dyslipidemia.DDX PCOS.

When is it considered delayed puberty: for girls? For boys?

Girls: no thelarche by 13; no menarche by 15
Boys: No testicular volumetric increase by 14
Note: Measure length in the testis 2.4 cm start to see development.

What are you thinking?
anosmia with no sexual development
Undernutrition/Chronic Illness/Extreme Exercise
GnRH Dependent Hypogonadotropic Hypogonadism

Kallmann's
Note: Kallmann's: anosmia - they like to ask about this.
Undernutrition/Chronic Illness/Extreme Exercise: eating disorders etc. , stress fractures, extreme exercise; Hypogonadotropic (no LH FSH) Hypogonadism

Pituitary FSH-B mutations cause what issues?

-- absent breast development and amenorrhea
-- (FSH low LH high)

Pituitary LH-B mutations cause what issues?

-- in girls assoc with subfertility,
-- lower hz of PCOS, delayed breast Ca

What intervention for Turner syndrome helps them grow to a normal height?

Growth hormone to help achieve normal height - can usu get them around 5 foot with a combination.

What are you thinking:
characterized by mucocutaneous candidiasis, hyperparathyroidism, and adrenal insuff (APS-1 due to mutation of AIRE gene)
Assoc with autoantibodies to steroidogenic enzymes
APS-2: DM, chronic lymphocytic thyroiditis, vitligo, and pernicious anemia
Unexplained infertility (antibodies may precede FSH elevation)

Autosomal-recessive type I autoimmune polyglandular syndrome
Gonadal Causes of delayed puberty: Auto-Immune Ovarian Failure

What are you thinking:
autosomal recessive 3 different enzymes (GALK, GALT, GALE), FSH signal transduction is impaired

Galactosemia:
a gonadal cause of pubertal delay.

What is the work up for delay in puberty:

-- History/Physical (growth records)
-- Labs: LH, FSH, DHEAS, Sex Steriods, TSH, Prolactin
-- Bone Age
-- Imaging Studies of brain/abdomen

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