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GD 1 > PRECOCIOUS PUBERTY > Flashcards

PRECOCIOUS PUBERTY Flashcards

1
Q

Activates in central precocious puberty

A

HPG axis

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2
Q

Age to diagnose precocious puberty

A

Boy 8

Girls 9

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3
Q

How common central precocious puberty to girls than boys

A

10 folds

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4
Q

LH lvl most reliable findings for CPP

A

0.3 IU/L

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5
Q

What is peripheral PP?

A

Hormonal influence outside HPG

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6
Q

Elevated estradiol lvl in low LH indicates what?

A

Estrogen secreting tumor

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7
Q

Mutiple cafe au lait spots and fibrous dyplasia in bones indicates what?

A

McCune- Albright syndrome or neurofibromatosis

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8
Q

safely prevents premature fusion of growth plates

thereby preserving height potential

A

GNRH analogues (leuprolide )

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9
Q

PE finding(diff diagnosis): abdominal pain

A

gondala malignancy

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10
Q

PE finding(diff diagnosis): assymetric testis

A

gonadal tumor

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11
Q

PE finding(diff diagnosis): cafe au lait spot

A

McCune-albright syndrome and neurofibromatosis

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12
Q

PE finding(diff diagnosis): enlarged thyroid

A

hyper/hypothyroidism

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13
Q

PE finding(diff diagnosis): head trauma

A

Central precocious puberty

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14
Q

PE finding(diff diagnosis): radiation therapy

A

Central precocious puberty

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15
Q

PE finding(diff diagnosis): virilization in girls

A

androgen secreting tumor

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16
Q

absence of breast dev by 13 yo

A

delayed puberty

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17
Q

absence of testicular growth at least 4ml or 2.5cm lenght

A

delayed puberty

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18
Q

most common cause of delayed puberty

A

constitutional delay of growth

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19
Q

gonadal insufficiency with elevated FSH and LH

A

hypergonadotropic HYPOgonadism

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20
Q

gonadal insufficiency with LOW FSH and LH

A

hypergonadotropic HYPERgonadism

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21
Q

delayed puberty treament for boys

A

testosterone cypionate or enanthate

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22
Q

dosage of testosterone cypionate or enanthate

A

50 to 100 mg intramuscularly per month

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23
Q

delayed puberty treament for girls

A

overnight transdermal estradiol

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24
Q

overnight transdermal estradiol dosage

A

6.2mcg

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25
Q

treatment for hypogonadotropic hypogonadism with CELIAC DISEASE

A

gluten free diet

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26
Q

other term for central precocious puberty

A

gonadotropin dependent/ true precocious puberty

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27
Q

CPP(isosexual, contrasexual)?

A

isosexual

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28
Q

other term for peripheral precocious puberty

A

gonadotropin independent/ precocious pseudopuberty

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29
Q

PPP(isosexual, contrasexual)?

A

can be isosexual and contrasexual

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30
Q

90% of girls are idiopathic

A

CPP

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31
Q

result from early activation of HPG axis

A

CPP

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32
Q

serum estradiol are low(PPP or CPP?)

A

CPP

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33
Q

LH undetectable in prebuerts(PPP or CPP?)

A

CPP

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34
Q

obtained during SLEEP has greater diagnostic power

A

LH

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35
Q

particularly helpful in boys with CPP

A

administration of gonadotropinreleasing hormone (GnRH stimulation test) or a GnRH agonist (leuprolide stimulation test)

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36
Q

LH respond to

A

GnRH test

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37
Q

In girls with sexual precocity,

nocturnal LH secretion and LH response to GnRH or GnRH agonist (High/low?)

A

low

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38
Q

proves central nature of sexual precocity

A

estradiol lvl of >50pg/ml

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39
Q

Gonadotropin-independent causes of isosexual precocious puberty:
For girls

A
  • tumors of the ovaries
  • autonomously functioning ovarian cysts
  • feminizing adrenal tumors
  • McCune-Albright syndrome
  • exogenous sources of estrogen
40
Q

Gonadotropin-independent causes of isosexual precocious puberty:
For boys

A
  • congenital adrenal hyperplasia
  • adrenal tumors
  • Leydig cell tumors
  • Human chorionic gonadotropin–producing tumor
41
Q

rationale for using GnRH agonists for treatment of central precocious puberty

A

because GnRH secretes in pulsatile manner

42
Q

GnRH agonist

A

desensitize the gonadotropic cells of the pituitary to the stimulatory effect of endogenous GnRH and effectively halt the progression of central sexual precocity.

43
Q

GnRH antagonist

A

NOT FDA approved

44
Q

what does treatment for precocious puberty does for growth rate and osseous maturation

A

decreases

45
Q

what does treatment for precocious puberty does for menses

A

ceases

46
Q

what does treatment for precocious puberty does for testicular size and freq of erection

A

decreases

47
Q

-If treatment is effective, the serum sex hormone concentrations decrease to WHAT prepubertal levels?

A

testosterone: <10-20 ng/ml
estradiol: <5-10pg/ml

48
Q

if therapy is discontinued?

A

puberty resumes

49
Q

most common brain lesion causing precocious puberty

A

hypothalamic hamartomas

50
Q

glial cells in hamartome produce what?

A

transforming growth factor α

51
Q

transforming growth factor α FUNCTION

A

activate GnRH pulse generator

52
Q

causes gelastic/ psychomotor seizures

A

sessile variant of hypothalamic hamartomas

53
Q

CNS lesions/insults associated with gonadotropin-dependent sexual precocity:

A

tuberculosis meningitis
severe head trauma
hydrocephalus

54
Q

Neoplasms causing precocious puberty include:

A

astrocytomas

optic tract tumors

55
Q

common on children with neurofibromatosis type 1

A

optic tract tumor

56
Q

Approximately 50% of the tumors in the pineal region are

A

germ cell tumors or astrocytomas

57
Q

how does pineal hypothalamic germ cell tumors cause central precocious puberty

A

by secreting HCG

58
Q

how does HCG inc testosterone

A

by stimulating leydig cells

59
Q

unnatural crying/laughter

A

gelastic seizures

60
Q

Symptoms suggesting intracranial lesion:

A

gelastic seizures
cachaxia
diabetes insipidus

61
Q

imbalance of fluids
very thirsty
large amount of urine

A

diabetes insipidus

62
Q

weakness or wasting of body due to chroni illness

A

cachaxia

63
Q

Precocious Puberty Resulting from Organic Brain Lesions(isosexual/contrasexual)

A

ALWAYS isosexual

64
Q

rapidly progressive sexual precocity in children ndicates

A

hypothalamic hamartoma

65
Q

Precocious Puberty Resulting from Organic Brain Lesions TREATMENT

A

gnrh agonist

66
Q

for patients with hypothalmic hamartoma and associated gelastic and psychomotor seizures

A

stereotactic radiation therapy (gamma knife surgery)

67
Q

increases the risk of precocious puberty

A

Radiation therapy, generally for leukemia or intracranial tumors

68
Q

hastens the onset of puberty almost exclusively in girls

A

low dose radiation (18-24 Gy)

69
Q

trigger precocious sexual development in BOTH sexes

A

high dose radiation (25-47 Gy)

70
Q

risk of sexual precocity is (inversely/directly) proportional to the age of the child at the time radiation

A

inversely

71
Q

late effect of high-dose CNS irradiation

A

hypopituitarism with gonadotropin deficiency

72
Q

common cause of hypothyroidism resulting to precocious puberty

A

hashimoto thyroiditis- often undiagnosed(especially in child with trisomy 21)

73
Q

testicular volume of >30ml

A

macroorchidism- persist despite levothyroxine therapy

74
Q 
tumor:
secretes HCG
no spermatogenesis
plasma testosterone is high
FSH and LH low
A

Chorionic Gonadotropin-Secreting Tumors

75
Q

Plasma levels of hCG and α-fetoprotein are usually markedly elevated

A

hepatic tumors

76
Q

usually located in the neurohypophyseal area or the pineal area

A

intracranial tumor

77
Q

Marked elevations of hCG and α-fetoprotein often occur in the cerebrospinal fluid

A

intracranial tumor

78
Q

reported to cause PPP in boys with KLINEFELTER SYNDROME

A

mediastinal tumors

79
Q

associated with patchy cutaneous pigmentation and fibrous dysplasia of the skeletal system

A

McCune-Albright Syndrome

80
Q

what causes McCune-albright syndrome

A

mutation in α-subunit of Gs

81
Q

mutation in α-subunit of Gs

A

resulting in the formation of the putative gsp oncoprotein

82
Q

McCune-albright syndrome has no response to GnRH or leuprolide stimulation. WHY?

A

low levels of LH and FSH

83
Q

aromatase inhibitors

A

letrozole

84
Q

letrozole dosage

A

1.25-2.5mg/day orally

85
Q

letrozole effect

A

limit estrogen in puberty and osseous maturation

86
Q

antiestrogen

A

tamoxifen

87
Q

antiandrogen

A

Spironolactone- -50-100 mg bid
-high dose may cause hyperkalemia
flutamide 125-250 mg bid
bicalutamide 25-50 mg daily

88
Q

can be used to treat hypersomatotropism

A

Depot octreotide (Sandostatin LAR Depot 10-30 mg IM monthly)

Lanreotide (Somatuline Depot, 60-90 mg SC monthly)

89
Q

most common extraglandular manifestation

A

phosphaturia- lead to rickets and osteomalacia

90
Q

transmitted from affected males and unaffected female carriers of the gene to their male offspring

A

Familial Male Gonadotropin Independent Precocious Puberty

91
Q

in Familial Male GonadotropinIndependent Precocious Puberty, TESTOSTERONE lvl(high or low)

A

high

92
Q

cause of high testosterone lvl in Familial Male GonadotropinIndependent Precocious Puberty

A

mutation in LH receptor

93
Q

in Familial Male GonadotropinIndependent Precocious Puberty:

osseous maturation?

A

advanced

94
Q

type IA pseudohypoparathyroidism with single mutation of the Gsα protein results to

A

gonadotropin-independent precocious puberty

95
Q

mutation of the Gsα protein in normal body temp results to

A

type IA pseudohypoparathyroidism

96
Q

mutation of the Gsα protein in cooler temp of testis

A

constitutionally activating–>production of testosterone

97
Q

Familial Male GonadotropinIndependent Precocious Puberty:

treatment

A

ketoconazole

GD 1 (1 decks)

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