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1st Repro > Abnormal Male Puberty > Flashcards

Abnormal Male Puberty Flashcards

1
Q

Definition – onset of pubertal development at an age earlier than expected based upon established normal standards

A
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2
Q

Beginning of enlargement of scrotum and testes; change in texture and reddening of scrotal skin

A

Tanner 2

age 11

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3
Q

Beginning growth of the penis, mainly in length; further growth of testes and scrotum

A

Tanner 3

age around 13

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4
Q

Further growth of penis in lenght and breadth; further darkening of the scrotal skin

A

Tanner 4

late 13

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5
Q

Adult sized genitals

A
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6
Q

Premature Male Puberty

  • In the United States this would generally be considered less than _______
  • Level of concern for further evaluation should increase with decreasing age at presentation
  • Initial evaluation at a minimum should include a comprehensive history and physical examination
A

9 years of age

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7
Q

Premature Male Puberty

•Three questions to ask

A

Is the child to young to have reached the pubertal milestone in

question?

What is causing the early sexual development?

Is therapy indicated and if so which therapy?

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8
Q

Premature Male Puberty

•Classifications

A
  • Gonadotropin-dependent premature puberty (GDPP)
  • Gonadotropin independent premature puberty (GIPP)
  • Incomplete premature puberty
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9
Q
  • Due to early maturation of the hypothalamic pituitary gonadal axis
  • More than 80% of cases are idiopathic
  • Almost all idiopathic cases are in girls
A

Gonadotropin-dependent premature puberty (GDPP)

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10
Q

•GDPP; most cases are idiopathic, if not the causes are CNS lesions and often Hamartomas which are:

A

Benign

• Most frequent CNS tumor in very young children

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11
Q

Most frequent CNS tumor in very young children that causes premature male puberty

A

Hamartomas; often benign and in the CNS

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12
Q

Random causes of premature male puberty

A

•Other CNS tumors

  • Astrocytoma
  • Ependymoma
  • Pinealoma
  • Optic and hypothalamic glioma

**•CNS irradiation **

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13
Q

•Other CNS lesions

  • Hydrocephalus
  • Cysts
  • Trauma
  • CNS inflammatory disease

• Congenital midline defects

A

Premature male puberty

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14
Q
  • Genetics causes of premature male puberty
  • Gain of function mutations in ______
  • Loss of function mutations in ______
A

Kisspeptin 1 gene and KISS-1R

MKRN3

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15
Q

•Previous excess steroid exposure

  • Primary hypothyroidism
  • Regress with thyroxine therapy
A

More fucking causes of preamture male puberty

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16
Q
  • Due to excess secretion of sex hormones derived either from gonads or adrenal glands, exogenous sources of steroids, or ectopic production of gonadotropin from a germ cell tumor
  • May be gender appropriate or inappropriate
A

GIPP: Gonadotropin independent premature puberty

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17
Q

GIPP

•Due to excess secretion of sex hormones derived either from

A

gonads or adrenal glands

exogenous sources of steroids

ectopic production of gonadotropin from a germ cell tumor

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18
Q

Premature Male Puberty: GIPP: Isosexual

A
  • Leydig cell tumors
  • Human chorionic gonadotropin (hCG) secreting germ cell tumors
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19
Q

•Premature male puberty from GIPP :

Human chorionic gonadotropin (hCG) secreting germ cell tumors often located in:

A

•Gonads, brain, liver, retroperitoneum, posterior mediastinum

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20
Q
  • Familial male limited premature puberty, rare
  • Result of an activating mutation in _________
A

LH receptor gene

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21
Q

Premature Male Puberty

•Adrenal pathology, Androgen secreting tumors, Enzymatic defects and adrenal steroid biosynthesis

A
  • 11 beta Beta hydroxylase deficiency
  • 3 beta hydroxysteroid dehydrogenase type II deficiency
  • Hexose 6 phosphate dehydrogenase deficiency
  • PAPSS2 deficiency
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22
Q

Premature male puberty with adrenal pathology

A
  • Estrogen secreting tumors
  • Androgen and estrogen secreting tumors
  • Androgen secreting tumors
  • Enzymatic defects and adrenal steroid biosynthesis
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23
Q
  • Rare
  • Triad of peripheral premature puberty, café au lait skin pigmentation, and fibrous dysplasia of bone
A

•McCune Albright syndrome

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24
Q
  • Exogenous estrogen
  • Pituitary gonadotropin secreting tumors

• Rare

A

Premature Male Puberty

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25
Q

Cause of incomplete premature male puberty

A

Due to increased adrenal androgen production

•Isolated male hormone mediated sexual characteristics (pubic and/or axillary hair, acne, and apocrine odor)

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26
Q

Evaluation for premature male puberty
• History and Onset
• Family history – siblings and parents

A
  • Linear growth acceleration
  • Symptoms related to etiology: headaches, seizures, abdominal pain
  • History of CNS disease or trauma
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27
Q

Examination of premature male puberty; what are we looking for?

A
  • Height, weight, height velocity • Fundoscopic
  • Visual fields
  • Dermatologic
  • Tanner staging
  • Bone age
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28
Q
  • Laboratory and Imaging
  • Measure basal LH levels, if elevated ______
  • If not clearly elevated stimulate with GnRH agonist (several protocols available)
  • Elevated LH and FSH means GDPP
  • Lack of significant increase means GIPP
A

GDPP

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29
Q
  • Laboratory and Imaging for premature male puberty
  • Measure basal LH levels, if elevated GDPP
  • If not clearly elevated stimulate with GnRH agonist (several protocols available)
    * Elevated LH and FSH means\_\_\_\_\_
* Lack of significant increase means\_\_\_\_\_
A

GDPP

GIPP

30
Q

Patient has signs of premature male puberty. You measure basal LH levels and they were elevated.

What do you suspect and what do you do next?

A

GDPP
• Pituitary MRI

  • Estradiol, testosterone, TSH, free T4
  • GH if prior cranial irradiation
31
Q

You suspect your pt has premature male puberty. You try to measure LH levles but nothing happens so you stimulate GnRH

results still show lack of significant LH rise. What is the dx and what do you do next?

A

• If GIPP

Testosterone, estradiol, LH, FSH, cortisol (drawn in the evening), and DHEA, DHEAS, 17 hydroxyprogesterone, hCG

If hCG elevated and tumor in mediastinum a karyotype for Klinefelter syndrome recommended for mediastinal germinoma

• Testicular ultrasound

32
Q

Pt has GIPP, If hCG elevated and tumor in mediastinum a karyotype for

A

Klinefelter syndrome recommended for mediastinal germinoma

33
Q

Treatment for GDPP

A

Direct therapy toward underlying pathology for identifiable CNS lesions

Exception includes :benign hypothalamic hamartoma

Majority of patients will require t**reatment with GnRH agonist **

34
Q

Direct therapy toward underlying pathology for identifiable CNS lesions

Exception includes benign hypothalamic hamartoma

Majority of patients will require treatment with GnRH agonist

A

GDPP

35
Q
  • Decision to treat in Premature Male Puberty
  • Primary goal to :
  • Do not treat primarily to address perceived psychosocial consequences
A

achieve normal adult height

36
Q

important considerations when treating for premature male puberty

A
  • Age
  • Younger age
  • Rapid progression
  • Rate of maturation • Height prediction
  • Less than 160 cm
37
Q

GnRH agonist treatment for GDPP
• Initial stimulation of pituitary gonadotropin secretion followed by a complete but _________

  • Treatment continued until it appears that it is safe and appropriate for puberty to proceed
  • Leuprolide depot (one month) given intramuscularly once monthly starting dose of 7.5 mg, 11.25 mg, or 15 mg depending on child’s weight (approximately 0.3 mg/kilogram /dose)
A

reversible suppression

38
Q

GnRH agonist treatment for GDPP
• Initial stimulation of pituitary gonadotropin secretion followed by a complete but reversible suppression

• Treatment continued until it appears that it is safe and appropriate for puberty to proceed

What is it done with?

A

• Leuprolide depot (one month) given intramuscularly once monthly starting dose of 7.5 mg, 11.25 mg, or 15 mg depending on child’s weight (approximately 0.3 mg/kilogram /dose)

39
Q

Premature male puberty; how is leuprolide less efficient as a treatement

A
  • Alternatively a 3 month preparation of leuprolide depot if desiring less frequent administration
  • Somewhat less clinical evidence for efficacy
  • Most patients start at 11.25 mg and titrate upwards, however; males with/without hypothalamic hamartoma generally require 30 mg
  • Monitor response after 3 months of therapy and adjust upward if pubertal progression is not suppressed
40
Q

Premature Male Puberty:

• Measure_____and _______just prior to second or third monthly dose or after a change in dose

  • Measure ______every 6-12 months
  • Success can be measured by performing a diagnostic _______
A

LH and sex steroid levels

bone age

GnRH agonist stimulation test

41
Q

How do we treat congenital adrenal hyperplasia causing premature male puberty

A

glucocorticoid therapy

42
Q

Boys rarely affected by McCune Albright syndrome but can be treated with _______and_______ similar to that for familial male limited premature puberty

A

anti-androgens in combination with an aromatase inhibitor

43
Q

Familial male limited premature puberty can be treated with

A

spironolactone an anti-androgen and testolactone an aromatase inhibitor

44
Q

•_________ an inhibitor of androgen sythesis is also effective but can cause hepatotoxicity and adrenal insufficiency

A

Ketoconazole

45
Q

• Secondary GDPP may develop and can be treated with

A

GnRH agonist therapy

46
Q

• Incomplete premature puberty treatment

A

• Does not require therapy but should be examined regularly

47
Q

To block GDPP we need to act at the level of the _____

A

At the level of the hypothalamus: prevent LH and FSH release from the pituitiary by blocking GnRH

48
Q

For GIPP we need to act at the level of the ______

A

the testicles and block aromatase

49
Q

Absence or incomplete development of secondary sexual characteristics based on an age where 95% of children that sex and culture have initiated sexual maturation (> 14 years of age in the United States).

A

Delayed Male Puberty

50
Q

The disease is usually the result of inadequate gonadal (testicular) steroid secretion, most often caused by _______ from the anterior pituitary, due to defective production of GnRH from the hypothalamus.

A

defective gonadotropin secretion (LH and FSH)

(can be the result of a variety of hypothalamic, pituitary, and gonadal disorders)

51
Q

Delayed male puberty: etiology is often:

A
  • Etiology can typically be identified through a careful history, physical exam, and series of laboratory and imaging
  • Treatment involves watchful waiting versus androgen therapy
52
Q

You see delayed puberty in a boy, what is the first test you should order

A

Get an LH

53
Q

You get an LH on a boy with delayed puberty, if its low what do you do next?

A

Get detailed family history; may need to look into adrenal insufficiency

54
Q

Delayed puberty in a boy with high LH and FSH, what do we need to do next?

A

karyotype

55
Q

most common cause of etiology delayed male puberty

A

constitutional delay

next is functional hypogonatotropic hypogonadism

56
Q

Patient has hypogonadal, has femal habitis, poorly virilized with gynecomastia and eunuchoidism with a small phallus

A

Klinefelters syndroem

57
Q
  • Long bone abnormality of leg independent of that related to testosterone deficiency
  • Psychosocial abnormality involving social interactions – poor insight/judgement
  • Impairment of higher level linguistic competence, but sparing of vocabulary and understanding of language
  • Attention deficit with resultant impulsiveness
A

Klinefelter’s Syndrome

58
Q

Klinefelters syndrome causes what type of pulmonary diseases and cancers?

A

• Pulmonary diseases
• Bronchitis, bronchiectasis, and emphysema

  • Cancer
  • Germ cell, breast, and non-Hodgkin lymphoma
59
Q
  • Varicose veins : Leg ulcers
  • Systemic lupus erythematosus
  • Diabetes mellitus
A

Associated with Klinefelters syndrome

60
Q

Male Hypogonadism

• Primary –

A

low testosterone and/or sperm with high LH and FSH, consider karyotype for Klinefelter’s Syndrome

61
Q

Male Hypogonadism

Secondary causes

A

low testosterone and/or sperm with low or inappropriately normal LH and FSH, needs pituitary work up

62
Q

Treatment of Male Hypogonadism

A
  • Treat or address underlying cause and associated conditions
  • Treatment options numerous based on patient goals and preferences
  • Screen for contraindications to therapy and regularly monitor for adverse events
63
Q

Primary Hypogonadism, we see huge increase in what hormones?

A

LH and FSH get a huge surge that act on the testes

64
Q

Causes of primary male hypogonadism

A

FSH and LH increase but still low testosterone

causes testicular agenesis, Klinefelters syndrome, 5alpha reductase and untreated cryptorchidism

65
Q

Seconary causes of male hypogonadism: see low testosterone and low or normal LH

A

often pituitary disorders, tumors, panhypopituitarism, hypothalmic or Kallmans

66
Q

High levels of testosterone lead to increase estradiol causing:

A

bone formaiton and breast tissue

67
Q

Testosterone leads to elevated DHT levels; what are the effects?

A

facial and body hair, acne, scalp hair loss, prostate growth

68
Q

Testosterone has what direct effects on the body

A

muscle mass, skeletal growth, spermatogenesis, sexual function

69
Q

You suspect your pt has hypogonadism, what do you look for?

A

Get morning total Testosterone

70
Q

Your patient has low testosterone, what do you do next?

A

exculde reversible illness/drugs/nutitional deficiency

Repeat T levels

LH and FSH levels should be checked

71
Q

Pt has low T, low or normal LH and FSH, what do you do next

(pt suspicious for hypogonadism)

A

Get prolactin, iron, and check for pituitary hormones

get MRI if indicated

72
Q

Pt has low T, high LH and FSH, what do we suspect?

A

Primary hypogonadism

Karyotype for klinefileter

1st Repro (16 decks)

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