PRECOCIOUS PUBERTY Flashcards
(97 cards)
1
Q
Activates in central precocious puberty
A
HPG axis
2
Q
Age to diagnose precocious puberty
A
Boy 8
Girls 9
3
Q
How common central precocious puberty to girls than boys
A
10 folds
4
Q
LH lvl most reliable findings for CPP
A
0.3 IU/L
5
Q
What is peripheral PP?
A
Hormonal influence outside HPG
6
Q
Elevated estradiol lvl in low LH indicates what?
A
Estrogen secreting tumor
7
Q
Mutiple cafe au lait spots and fibrous dyplasia in bones indicates what?
A
McCune- Albright syndrome or neurofibromatosis
8
Q
safely prevents premature fusion of growth plates
thereby preserving height potential
A
GNRH analogues (leuprolide )
9
Q
PE finding(diff diagnosis): abdominal pain
A
gondala malignancy
10
Q
PE finding(diff diagnosis): assymetric testis
A
gonadal tumor
11
Q
PE finding(diff diagnosis): cafe au lait spot
A
McCune-albright syndrome and neurofibromatosis
12
Q
PE finding(diff diagnosis): enlarged thyroid
A
hyper/hypothyroidism
13
Q
PE finding(diff diagnosis): head trauma
A
Central precocious puberty
14
Q
PE finding(diff diagnosis): radiation therapy
A
Central precocious puberty
15
Q
PE finding(diff diagnosis): virilization in girls
A
androgen secreting tumor
16
Q
absence of breast dev by 13 yo
A
delayed puberty
17
Q
absence of testicular growth at least 4ml or 2.5cm lenght
A
delayed puberty
18
Q
most common cause of delayed puberty
A
constitutional delay of growth
19
Q
gonadal insufficiency with elevated FSH and LH
A
hypergonadotropic HYPOgonadism
20
Q
gonadal insufficiency with LOW FSH and LH
A
hypergonadotropic HYPERgonadism
21
Q
delayed puberty treament for boys
A
testosterone cypionate or enanthate
22
Q
dosage of testosterone cypionate or enanthate
A
50 to 100 mg intramuscularly per month
23
Q
delayed puberty treament for girls
A
overnight transdermal estradiol
24
Q
overnight transdermal estradiol dosage
A
6.2mcg
25
treatment for hypogonadotropic hypogonadism with CELIAC DISEASE
gluten free diet
26
other term for central precocious puberty
gonadotropin dependent/ true precocious puberty
27
CPP(isosexual, contrasexual)?
isosexual
28
other term for peripheral precocious puberty
gonadotropin independent/ precocious pseudopuberty
29
PPP(isosexual, contrasexual)?
can be isosexual and contrasexual
30
90% of girls are idiopathic
CPP
31
result from early activation of HPG axis
CPP
32
serum estradiol are low(PPP or CPP?)
CPP
33
LH undetectable in prebuerts(PPP or CPP?)
CPP
34
obtained during SLEEP has greater diagnostic power
LH
35
particularly helpful in boys with CPP
administration of gonadotropinreleasing hormone (GnRH stimulation test) or a GnRH agonist (leuprolide stimulation test)
36
LH respond to
GnRH test
37
In girls with sexual precocity,
| nocturnal LH secretion and LH response to GnRH or GnRH agonist (High/low?)
low
38
proves central nature of sexual precocity
estradiol lvl of >50pg/ml
39
Gonadotropin-independent causes of isosexual precocious puberty:
For girls
- tumors of the ovaries
- autonomously functioning ovarian cysts
- feminizing adrenal tumors
- McCune-Albright syndrome
- exogenous sources of estrogen
40
Gonadotropin-independent causes of isosexual precocious puberty:
For boys
- congenital adrenal hyperplasia
- adrenal tumors
- Leydig cell tumors
- Human chorionic gonadotropin–producing tumor
41
rationale for using GnRH agonists for treatment of central precocious puberty
because GnRH secretes in pulsatile manner
42
GnRH agonist
desensitize the gonadotropic cells of the pituitary to the stimulatory effect of endogenous GnRH and effectively halt the progression of central sexual precocity.
43
GnRH antagonist
NOT FDA approved
44
what does treatment for precocious puberty does for growth rate and osseous maturation
decreases
45
what does treatment for precocious puberty does for menses
ceases
46
what does treatment for precocious puberty does for testicular size and freq of erection
decreases
47
-If treatment is effective, the serum sex hormone concentrations decrease to WHAT prepubertal levels?
testosterone: <10-20 ng/ml
estradiol: <5-10pg/ml
48
if therapy is discontinued?
puberty resumes
49
most common brain lesion causing precocious puberty
hypothalamic hamartomas
50
glial cells in hamartome produce what?
transforming growth factor α
51
transforming growth factor α FUNCTION
activate GnRH pulse generator
52
causes gelastic/ psychomotor seizures
sessile variant of hypothalamic hamartomas
53
CNS lesions/insults associated with gonadotropin-dependent sexual precocity:
tuberculosis meningitis
severe head trauma
hydrocephalus
54
Neoplasms causing precocious puberty include:
astrocytomas
| optic tract tumors
55
common on children with neurofibromatosis type 1
optic tract tumor
56
Approximately 50% of the tumors in the pineal region are
germ cell tumors or astrocytomas
57
how does pineal hypothalamic germ cell tumors cause central precocious puberty
by secreting HCG
58
how does HCG inc testosterone
by stimulating leydig cells
59
unnatural crying/laughter
gelastic seizures
60
Symptoms suggesting intracranial lesion:
gelastic seizures
cachaxia
diabetes insipidus
61
imbalance of fluids
very thirsty
large amount of urine
diabetes insipidus
62
weakness or wasting of body due to chroni illness
cachaxia
63
Precocious Puberty Resulting from Organic Brain Lesions(isosexual/contrasexual)
ALWAYS isosexual
64
rapidly progressive sexual precocity in children ndicates
hypothalamic hamartoma
65
Precocious Puberty Resulting from Organic Brain Lesions TREATMENT
gnrh agonist
66
for patients with hypothalmic hamartoma and associated gelastic and psychomotor seizures
stereotactic radiation therapy (gamma knife surgery)
67
increases the risk of precocious puberty
Radiation therapy, generally for leukemia or intracranial tumors
68
hastens the onset of puberty almost exclusively in girls
low dose radiation (18-24 Gy)
69
trigger precocious sexual development in BOTH sexes
high dose radiation (25-47 Gy)
70
risk of sexual precocity is (inversely/directly) proportional to the age of the child at the time radiation
inversely
71
late effect of high-dose CNS irradiation
hypopituitarism with gonadotropin deficiency
72
common cause of hypothyroidism resulting to precocious puberty
hashimoto thyroiditis- often undiagnosed(especially in child with trisomy 21)
73
testicular volume of >30ml
macroorchidism- persist despite levothyroxine therapy
74
```
tumor:
secretes HCG
no spermatogenesis
plasma testosterone is high
FSH and LH low
```
Chorionic Gonadotropin-Secreting Tumors
75
Plasma levels of hCG and α-fetoprotein are usually markedly elevated
hepatic tumors
76
usually located in the neurohypophyseal area or the pineal area
intracranial tumor
77
Marked elevations of hCG and α-fetoprotein often occur in the cerebrospinal fluid
intracranial tumor
78
reported to cause PPP in boys with KLINEFELTER SYNDROME
mediastinal tumors
79
associated with patchy cutaneous pigmentation and fibrous dysplasia of the skeletal system
McCune-Albright Syndrome
80
what causes McCune-albright syndrome
mutation in α-subunit of Gs
81
mutation in α-subunit of Gs
resulting in the formation of the putative gsp oncoprotein
82
McCune-albright syndrome has no response to GnRH or leuprolide stimulation. WHY?
low levels of LH and FSH
83
aromatase inhibitors
letrozole
84
letrozole dosage
1.25-2.5mg/day orally
85
letrozole effect
limit estrogen in puberty and osseous maturation
86
antiestrogen
tamoxifen
87
antiandrogen
Spironolactone- -50-100 mg bid
-high dose may cause hyperkalemia
flutamide 125-250 mg bid
bicalutamide 25-50 mg daily
88
can be used to treat hypersomatotropism
Depot octreotide (Sandostatin LAR Depot 10-30 mg IM monthly)
Lanreotide (Somatuline Depot, 60-90 mg SC monthly)
89
most common extraglandular manifestation
phosphaturia- lead to rickets and osteomalacia
90
transmitted from affected males and unaffected female carriers of the gene to their male offspring
Familial Male Gonadotropin Independent Precocious Puberty
91
in Familial Male GonadotropinIndependent Precocious Puberty, TESTOSTERONE lvl(high or low)
high
92
cause of high testosterone lvl in Familial Male GonadotropinIndependent Precocious Puberty
mutation in LH receptor
93
in Familial Male GonadotropinIndependent Precocious Puberty:
| osseous maturation?
advanced
94
type IA pseudohypoparathyroidism with single mutation of the Gsα protein results to
gonadotropin-independent precocious puberty
95
mutation of the Gsα protein in normal body temp results to
type IA pseudohypoparathyroidism
96
mutation of the Gsα protein in cooler temp of testis
constitutionally activating-->production of testosterone
97
Familial Male GonadotropinIndependent Precocious Puberty:
| treatment
ketoconazole
