Hypogonadism and Endocrine Neoplasia

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1
FSH and LH are _____ in hypergonadotrophic hypogonadism
elevated
2
LH and FSH is ______ in hypogonadotrophic hypogonadism
low or normal
3
What are the causes of hypergonadotrophic hypogonadism?
autoimmunity
aging
lymphoma
orchitis
viral infections (mumps)
TB
Klinefelter syndrome
XY gonadal dysgenesis
Abiraterone acetate
unilateral orchiectomy
4
What is klinefelter syndrome?
XXY
5
What are the s/s of Klinefelter syndrome in infants?
weak muscles
slow motor development
delay in speech
undescended testicles
6
How do the testicles feel in infants with Klinefelter syndrome?
normal
7
In adolescents how do the testicles feel in patients with Klinefelter syndrome?
firm
fibrotic
small
nontender to palpation
8
What s/s can be seen in an adolescent with Klinefelter syndrome?
gynecomastia
delayed speech, learning disabilities, psychiatric disturbances
mediastinal malignancies
9
What s/s do you see in men with Klinefelter syndrome?
decreased facial and body hair
enlarged breast tissue
infertility
10
How do you diagnose Klinefelter syndrome?
karyotype chromosomes (azoospermia on semen analysis)
serum testosterone (low)
gonodotropins (high)
11
What is the treatment for Klinefelter syndrome?
testosterone at puberty
12
T/F testosterone at puberty for Klinefelter syndrome can treat infertility, gynecomastia and small testes?
false
need IVF
13
What can testosterone help treat with Klinefelter syndrome?
normalize body proportions
promote development of normal secondary sex characteristics
14
What serum testosterone level is associated with primary hypogonadotrophic hypogonadism?
less than 150 ng/dL
15
What are the acquired etiologies for primary hypogonadotrophic hypogonadism?
Kallman syndrome
X linked congenital adrenal hyperplasia
prader willis syndrome
17 ketosteroid reductase deficiency
16
What age do boys not hit puberty by with primary hypogonadotrophic hypogonadism?
14
17
What are the chronic illnesses associated with secondary hypogonadotrophic hypogonadism?
kidney disease
hypo/hyperthyroidism
malnourishment
hemochromotosis
18
What drugs can be causes for secondary hypogonadotrophic hypogonadism?
opiods
marijuana
spironolactone
alcohol
estrogen
ketoconazole
19
What level of serum testosterone is associated with partial male hypogonadism?
150-300 ng/dL
20
T/F spermatognesis is preserved with partial male hypogonadism
true
21
What is the clinical presentation of hypogonadism?
decreased libido
ED
fatigue
depression
reduced exercise endurance
infertility
gynecomastia
headache
fracture
decreased muscle mass and fat gain
testes small or normal
decreased hair
low serum total and free testosterone
22
How to check testosterone levels?
check serum and free in the morning (before 10)
23
How many tests do you need to do to confirm the diagnosis?
two
24
What is a low serum testosterone in the morning?
under 240 ng/dL
25
What are concerning free testosterone levels in ages 18-69? 70 and over?
under 35 pg/mL
under 30 pg/mL
26
What age group is serum testosterone highest?
ages 20-30
27
When should you not screen for testosterone?
during illness, malnutrition and when patient is on certain medications
28
What are the two types of bioavailable testosterone?
free
testerone bound to albumin
29
What are some conditions that decrease SHBG?
moderate obesity
nephrotic syndrome
hypothyroidism
diabetes
glucocorticoids
progestins
androgenic steroids
30
What are some conditions that raise SHBG?
aging
hepatic cirrhosis
hyperthyroidism
anticonvulsants
estrogen
HIV infection
31
Which patients get testosterone replacement therapy
boys who have not entered puberty by 14
primary testicular failure
severe hypogonadotrophic hypogonadism (serum T less than 150)
low to normal testosterone with elevated LH levels
32
What are the CI for testosterone therapy?
active breast or prostate CA
untreated sleep apnea
untreated HF
33
What are the SE of testosterone therapy?
erythrocytosis
hyperlipidemia
gynecomastia
osteoporosis
34
What should you monitor with testosterone?
hematocrit
lipid profile
35
What are the drugs that interact with testosterone?
coumadin
insulin or oral hypoglycemics
36
Where can testosterone topical gels not be applied?
breast
genitals
37
When should you check testosterone after using testerone topical gels?
after 2 weeks
38
Where is testerone cypionate injected and how often?
gluteus medius muscle
every 2-3 weeks
39
How are tesosterone pellets administered?
inject SQ every 3-6 months
40
What is the dosing for testerone undecanoate (aveed)?
second dose at 6 weeks
then every 12 weeks
41
When are testosterone levels measured with aveed?
fourth dose
42
What are some AE associated with testerone undecanoate?
pulmonary oil microembolism
anaphylaxis
43
What is clomiphene citrate used to treat?
hypogonadotrophic hypogonadism
44
How often is clomiphene citrate taken?
by mouth every other day
45
What are gonadotropins used to treat?
hypogonadotropic hypogonadism w/ infertility
46
How are the gonadotropins dosed?
hCG 1,000 units injected SQ 3 x a week for 6 months
47
What is additionally done in addition to gonadotropins?
75 units of FSH are injected SQ after sperm analysis
48
What is a side effect associated with oral testosterone undecanoate?
GI intolerance
49
Why is oral methyltestosterone not recommended?
hepatitis
hepatocellular carcinoma
50
What are the benefits of testosterone replacement therapy?
improve mood, sense of wellbeing, sexual desire
improves exercise endurance
significant weight loss
increased muscle mass
improves longevity
51
What are the risk associated with testosterone replacment?
erythrocytosis
thromboembolic event
aggravates sleep apnea
increases acne
gynecomastia
52
What should you monitor when patients are on testosterone?
HCT
dexa
PSA
DRE
53
When should you consult urology for patients on testosterone replacement?
increase in PSA greater than 1.4 ng/mL in a 12 month period
prostate abnormality on DRE
54
How often do you monitor HCT?
0, 3 and 6 months annually
55
How often do you do a dexa scan in a patient on testosterone replacement?
after 1-2 years of treatment
56
MEN syndrome is inherited ___ ____ traits
autosomal dominant
57
MEN syndrome causes
predisposition to the development of tumors in two or more endocrine glands
58
Which endocrine glands are patients more likely to develop tumors with MEN syndrome?
pituitary
thyroid
parathyroid
adrenal
pancreas
59
A neuroendocrine tumor is a tumor that forms from cells that release ____ into the blood in response to a signal from the ____ system
hormones
nervous
60
Neuroendocrine tumor symptoms are based on
the location of the tumor
61
Treatment for neuroendocrine tumors depends on
the type of tumor
62
What are the three Ps of MEN I?
parathyroid
pancreatic
pituitary
63
What are the common tumors in MEN 2 and 3?
medullary thyroid carcinoma
pheochromocytoma
mucosal and GI ganglioneuromas
64
What is the most common manifestation of MEN 1?
primary hyperparathyroidism caused by hyperplasia of PTH glands
65
What are the clinical manifestions of MEN 1 (w/ primary hyperparathyroidism)?
osteoporosis
hypercalcemia
nepholithiasis
66
What are the common symptoms associated with hypercalcemia?
polydipsia
polyuria
constipation
malaise
67
What do MEN1 associated pituitary tumors commonly secrete?
prolactin (60%)
growth hormone (25%)
68
T/F pituitary tumors associated with MEN1 are larger and more aggressive than pituitary tumors not associated with MEN1?
true
69
Are MEN1-associated pituitary tumors are more/less responsive to medical or surgical treatments?
less
70
When should screening for pituitary tumors associated with MEN1 occur?
age 5
71
When shoudl screening for hyperparathyroidism associated with MEN 1 occur?
age 8
72
What will labs show with MEN1 associated hyperparathyroidism?
increased calcium
increased PTH
73
What is the second most common manifestation of MEN1?
pancreatic islet cell tumors
74
What are insulinomas?
adenoma of the islets of langerhans
usually benign
75
How will insulinomas present?
fasting hypoglycemia in an otherwise healthy well nourished adult
76
What are some s/s of insulinomas?
confusion
blurred vision
anxiety
convulsions
77
What is Zollinger-Ellison Syndrome?
gastrin secreting gut neuroendocrine tumors
result in hypergastrinemia and acid hypersecretion
78
How do patients with Zollinger-Ellison Syndrome present?
PUD
diarrhea
79
Where do primary gastrinomas most commonly arise?
pancreas
duodenal wall
lymph nodes
80
What percentage of gastrinoma are malignant?
2/3
81
When should you start screening for gastrinomas?
20
82
Basal serum gastrin levels of what confirm diagnosis of gastrinoma?
1000 pg/mL
83
When should you screen for insulinoma?
age 5
84
How long should the patient fast for before testing for insulinoma?
72 hour fast
85
When should you start screening for glucagonomas?
less than 10 years old
86
What are the diagnostic requirements for glucagonomas?
elevated serum glucagon levels
hyperglycemia
87
What is the pneumonic to remember the lesions commonly associated with MEN 1?
PAPA PI
parathyroid hyperplasia
pancreatic tumor
pituitary adenoma
88
What is the pneumonic to help remember the lesions associated with MEN2A?
PA ME PHE
parathyroid hyperplasia
medullary thyroid CA
pheochromocytomas
89
What is the pneumonic used to remember the lesions commonly associated with MEN 2B?
MAME PHE
marfanoid body habitus
medullary thyroid CA
pheochromocytomas
mucosal neuromas
90
When should you start screening for medullary thyroid carcinoma?
6 years
91
Which MEN syndrome is associated with incomplete penetrance?
MEN2
92
Which MEN syndrome is associated with hirshsprung disease?
2
93
What should you avoid in patients with MEN 2 syndrome?
GLP-1 agonists since it may increase the risk for medullary thyroid carcinoma
94
What are the symptoms associated with pheochromocytoma?
HTN
HA
sweating
95
What are the symptoms associated with medullary thyroid carcinoma?
diarrhea
96
MEN 3 is associated with which aggressive cancer?
medullary thryoid carcinoma
97
What is suggested for prophylaxis of medullary thyroid carcinoma?
total thyroidectomy at 6 months old
98
What is the least aggressive type of thyroid cancer?
papillary thyroid cancer
99
When do papillary thyroid cancers occur?
head and neck radiation exposure
familial syndroms
100
What are the symptoms of thyroid cancer?
most asymptomatic
palpable, firm, nontender nodule
101
Where can papillary thyroid cancer invade?
trachea
local muscles
102
Where does follicular thyroid cancer spread?
neck nodes
bone
lung
103
Where does medullary thyroid cancer metastasize?
local nodes
adjacent muscle
trachea
mediastinal lymph nodes
104
What are the s/s associated with medullary thyroid cancer?
flushing
diarrhea
105
What is the treatment for thyroid cancers?
surgery
106
How is carcinoma treated?
surgery with 1 cm total thyroidectomy with removal of cervical lymph nodes
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