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Junior - endo II > 10 - Male Hypogonadism > Flashcards

10 - Male Hypogonadism Flashcards

1
Q

What is hypogonadism in males?

A

Deficient testosterone secretion by testes

Leads to: 
Diminished libido/Erections
Fatigue
Depression
Reduced exercise endurance
Small testes
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2
Q

Primary vs secondary hypogonadism?

A

Primary - problem in testes (hypergonadotropic)

Secondary - not enough gonadotropin from pituitary (hypogonadotropic)

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3
Q

What determines the clinical effects of hypogonadism?

A

The time and degree of onset

  1. Early prenatal
  2. Later prenatal
  3. Puberty
  4. Post-pubertal
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4
Q

What happens with early prenatal testosterone deficiency?

A

Ambiguous genitalia
Pseudohermaphroditism
- sex organs underdeveloped/inappropriate

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5
Q

Difference between pseudohermaphroditism and “true” hermaphroditism?

A

Pseudo: testies or ovaries not both

True: both testies and ovaries present

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6
Q

Effects of later prenatal testosterone deficiency?

A

Micro penis

Cryptorchidism (failure to descend)

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7
Q

Effects of puberty testosterone deficiency?

A 
Poor muscle  development
Decreased strength/endurance
High pitched voice
Sparse axillary and pubic hair
No facial/body hair 
Lack of sexual differentiation
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8
Q

Low T effects on bones during puberty?

A
  • Long bones continue to grow out of proportion to axial skeleton (GH influence)
  • Eunuchoidal proportions
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9
Q

What are eunuchoidal proportions?

A

Arm span > height (5+ cm)

Crown-pubis>pubis-floor

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10
Q

Why does low T lead to eunuchoidal proportions and long bone growth?

A

Testosterone closes epiphyses

Lack of testosterone allows for greater growth

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11
Q

What effects are seen with post-pubertal testosterone deficiency?

A 
Decreased libido
Impotence
Low energy
Wrinkles (mouth and eyes)
Diminished hair (face/body)
Small or normal testes
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12
Q

What is hypogonadal habitus?

A

Feminine body distribution and absence of body and facial hair

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13
Q

What hormones are involved in hypogonadism?

A

GnRH
LH: stim T production
FSH: spermatogenesis
Total and free T

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14
Q

When are testosterone levels the highest?

A

Highest in the morning
Non-fasting

Levels fall 25-50% during day

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15
Q

Testosterone breakdown

A

Total: 60-75% bound to SHBG
Free: 1-2%

Free testosterone is the biologically active portion

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16
Q

Low testosterone + high LH/FSH?

A

Hypergonadotropic hypogonadism

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17
Q

Low testosterone + low LH/FSH

A

Hypogonadotropic hypogonadism

Evaluate other pituitary abnormalities (hyperprolactinemia)

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18
Q

3 types of hypogonadism

A 
A. Primary - defect in testes
- L T, H LH/FSH
B. Secondary - defect in hypothalamus-pituitary
- L T, L LH/FSH
C. Defect in androgen action/resistance 
- H T, H LH/FSH
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19
Q

Causes of primary hypogonadism?

A

The testes are the problem:

  • klinefelter syndrome
  • cryptorchidism
  • bilateral anorchia
  • acquired gonadal failure
  • other (lymphoma, chemo radiation etc)
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20
Q

What is the MC cause of primary hypogonadism?

A

Klinefelter syndrome

- seminiferous tubule dysgenesis

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21
Q

When does klinefelters syndrome become apparent?

A

The testes are normal during childhood but adolescence causes them to become firm, fibrotic, non-tender and small

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22
Q

S/s common with klinefelters?

A

Puberty:

  • Abnormal testes
  • Variable virilization
  • Gynecomastia (85%)
  • Tall
  • Eunuchoidal
  • Coordination problems
  • Socially awkward
  • Learning disabled
  • Mediastinal malignancies

Adults

  • infertility
  • impotence
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23
Q

Comorbidities with Klinefelter syndrome?

A 
Breast cancer
Chronic pulmonic disease
Varicosities of legs
DM
Osteoporosis
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24
Q

Labs for klinefelter syndrome?

A

LH/FSH and T
Karyotyping (47, XXY)
PCR testing for XIST in peripheral leukocytes

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25
Q

Classic presentation of klinefelter syndrome

A 
Small firm testes
Small-normal phallus
Female habitus
Poorly virilized
Gynecomastia
Eunuchoidism
Sparse hair
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26
Q

Other causes of primary hypogonadism?

A

Chriptorchidism
Bilateral anorchia
Acquired gonadal failure

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27
Q

What is cryptorchidism

A

Undescended testes (only 2% still affected by age 1)

It compromises sertoli cells -> infertility

Uncorrected -> Increased risk of testicular cancer

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28
Q

What must be done on PE for cryptorchidism?

A

Must distinguish from retracted testes

Begin at inguinal canal and move down

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29
Q

If no testicle is found on PE what then?

A

US or MRI to find it

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30
Q

Treatment for cryptorchidism?

A

HCG (1500mg IM x 3 days) - raises T ad 25% descend

Surgery: orchiopexy

31
Q

Malignancy risk for cryptorchidism?

A

Repaired: 0.06%

Un-repared: 5%

32
Q

If hCH is administered to pt with cryptorchidism and Testosterone doesnt go up what is it?

A

Anorchia: There is no testicle

33
Q

What is bilateral anorchia?

A

“Vanishing testicle syndrome”

Present during 1st 14-16 weeks of gestation and missing at birth

34
Q

How does bilateral anorchia affect growth and development?

A

It is normal until secondary sexual development fails to occur at puberty

35
Q

What are some causes of acquired gonadal failure at the seminiferous tubules (sertoli cells)

A

Mumps, gonorrhea, leprosy
Irradiation
Vascular injury
Chemo

36
Q

What about acquired gonadal failure at the leydig cell?

A 
Same as seminiferous tubules  
\+
aging 
-LH elevated (T neg feedback)
-FSH normal (spermatogenesis a-ok)
37
Q

Treatment for hypogonadism?

A

Testosterone replacement

38
Q

Testosterone replacement formulations

A

Testosterone IM q 2-3 weeks

Transdermal patches

Topical testosterone

Buccal testosterone

Oral Testosterone

Testosterone pellets

39
Q

What to monitor during testosterone therapy?

A

Check T levels 14 days after starting

Repeat q 6 months

Check (q 6 mo)

  • LFT
  • Hemoglobin
  • Hematocrit

DRE q 6 months

40
Q

Side effects of testosterone replacement?

A 
Acne
Decreased HDL
Gynecomastia
Polycythemia
Liver abnormalities
41
Q

Who cant get testosterone therapy?

A

People with
Prostate hyperplasia
Prostate cancer
Boys < 13 yrs

42
Q

What does everyone who gets testosterone therapy need first?

A

Prostate cancer screening

DRE and PSA

43
Q

What is secondary hypogonadism?

A

Indicates pituitary-hypothalamus dysfunction

44
Q

Causes of secondary hypogonadism?

A

Kallmann syndrome
Panhypopituitarism
Hyperprolactinemia
And more stuff

45
Q

What is the MC type of secondary hypogonadism?

A

Kallman syndrome

46
Q

What is kallman syndrome and how do you get it?

A

An isolated gonadotropin deficiency

It is an x linked inheritance

47
Q

What is a common anomaly associated with kallman syndrome?

A

Anosmia:

The inability to discriminate odors

48
Q

Signs and symptoms of kallmann syndrome?

A

Prepubertal testes (small, rubbery)
Eunuchoidal proportions
Gynecomastia
Renal

49
Q

Kallman kidney problems?

A

50% associated with unilateral renal agenesis

50
Q

Labs that diagnose kallmann syndrome?

A

Free ant total T < 100

LH/FSH low-normal or decreased

51
Q

Etiologies of panhypopituitarism?

A 
Congenital disorders
Tumors
Infarction from vascular insufficiency
Autoimmune diseases
Trauma
Infections
52
Q

Another cause of secondary hypogonadism is hyperprolactenima, what is the MC cause if this?

A

Pituitary adenoma

53
Q

What effects does the pituitary adenoma that cause hyperprolactinemia have?

A

Inhibits normal GnRH release
Inhibits action of testosterone
Decreases effectiveness of LH

54
Q

What are some other causes of secondary hypogonadism?

A 
ETOH abuse
BMI > 40
Marijuana
Idiopathic
Cushings syndrome
Hypothyroidism
Cancer
Aids
55
Q

How is secondary hypogonadism treated?

A

Depends on the location or cause:

May use Testosterone + another hormone based on desire for fertility

56
Q

Some effects of defect in androgen action or resistance?

A

Testicular feminization

Incomplete testicular feminization

5-alpha-reductase deficiency

57
Q

What are the features of feminized testicles?

A

Testes function normally but are located intra-abdominally

However

Abnormal receptors in the pituitary gland do not recognize testosterone so development proceeds as if there is a lack of testosterone

58
Q

PE for androgen action/resistance with testicular feminization

A

External female genitalia - pre-pubescent girl
No uterus or fallopian tubes
No male accessory organs (cryptorchid)

59
Q

What happens to the patients with testicular feminization at puberty?

A

Large amounts of testosterone is secreted which is converted by adipose tissue to estrogen

This leads to phenotypic females with well developed breasts but no menses

60
Q

What labs are present with testicular feminization?

A

Elevated FSH/LH and testosterone

61
Q

General overview of testicular feminization?

A

A genetic male with cryptorchidism produces testosterone that is not recognized so it is converted to estradiol by aromatase in the adipose tissue

62
Q

What happens if the testicular feminization is incomplete? (Incomplete androgen insensitivity)

A

The patient will have a mixed pattern of virilization and feminization

63
Q

Can the androgen receptors be restored

A

Androgen receptors cannot be restored

64
Q

How do we treat androgen action/resistance with testicular feminization?

A

Pt lives as female
Intra-abdominal testes are removed (cancer risk)
Estrogen tx is used to develop as female

65
Q

What happens if there is a 5-a-reductase deficiency?

A

Testosterone cannot be converted to DHT

DHT is required to masculinization of external genitalia in utero

66
Q

Where is 5-a-reductase deficiency more common?

A

Dominican republic
New guinea
Turkey

67
Q

When is DHT supposed to develop target organs during gestation?

A

Weeks 8-12

68
Q

What gender are 5-a-reductase children?

A

They appear female or have ambiguous external genitalia at birth

69
Q

What about the sex organs with 5-a-reductase?

A

The internal sex organs develop normally

They have cryptorchid testes

70
Q

Hormone levels in 5-a-reductase?

A

Testosterone/LH/FSH are all normal until puberty

Then testosterone skyrockets

71
Q

What happens with 5-a-reductase when the Testosterone goes up?

A

They suddenly become male

Secondary sex characteristics develops

External genitalia become masculinized

Penis develops

Body hair, adams apple and voice change

72
Q

What is guevedoce?

A

It means penis at 12

73
Q

Why didnt the sun ever shine on the castle?

A

It was full of knights

Junior - endo II (7 decks)

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