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F3 E4 Shea > Pituitary 2 > Flashcards

Pituitary 2 Flashcards

1
Q

Definition of what?

◦Genetic tall stature

–Constitutional tall stature

–Child’s height is similar to his or her parents

taller than their peers

◦Have accelerated growth velocity

◦Moderately advanced bone age

A

Constitutional Tall Stature

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2
Q

What are the 2 genetic syndromes associated w/ tall stature?

A
  • Klinefelter’s Syndrome
  • Marfan’s Syndrome
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3
Q

Which genetic syndrome causing Tall Stature?

–Most common cause of congenital hypogonadism

– Results in “eunuchoidal” proportions (long arms and legs)

– Learning disabilities, mainly in expressive language

– Small testes and gynecomastia

2 or more X chromosomes present in male phenotypes

A

Klinefelter’s Syndrome

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4
Q

Which genetic syndrome causing Tall Stature?

◦- inherited autosomal dominant disorder - connective tissue disease

–Tall stature

–Long, thin fingers and hyperextensibility of joints

A

Marfan’s Syndrome

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5
Q

3 endocrine causes of Tall Stature

A
  • Pituitary Gigantism (rare)
  • Precocious Puberty
  • Hyperthyroidism
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6
Q

Signs of what?

  • GH excess
  • Height exceeds 3-4 SDs from normal
  • HA or visual field involvement
  • Rapid growth rate, body proportional prior to epiphyseal fusion
  • Excessive size for age
A

Pituitary Gigantism (rare)

  • GH secreting pituitary adenoma/tumor
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7
Q

Which cause of Tall Stature?

◦Early tall stature due to early sex steroid secretion

◦Marked advancement of bone age

◦Greater incidence in girls or boys?

◦Early epiphyseal closure

◦Adult short stature

A

Precocious Puberty

  • Girls
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8
Q

What are 4 causes of precocious puberty?

A
  • Congenital adrenal hyperplasia,
  • virilizing adrenal tumors,
  • testicular and ovarian tumors,
  • ovarian cysts
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9
Q

Which cause of tall stature?

◦Overproduction or over-treatment with exogenous thyroxine

◦Increased growth and advanced bone age

A

Hyperthyroidism

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10
Q

Is excess GH or PRL more common?

A
  1. PRL (prolactin)
  2. GH
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11
Q

Dx of pituitary gigantism

  • Most children w/ tall stature DO NOT have pituitary gigantism (rare)
  • r/o genetics cause, precocious puberty, hyperthyroidism
  • Dysmorphic/disproportionate features/neurocog problems suggest what as the cause?
A

Chromosomal cause: Klinefelter’s Syndrome

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12
Q

Initial eval of Tall Stature to r/o Pituitary Gigantism consists of what? (5)

A
  • bone age,
  • thyroid function tests,
  • sex steroid hormone concentrations,
  • karyotype,
  • GH-related studies
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13
Q

3 diagnostic components to dx pituitary gigantism

A
  • IGF-1 levels
  • GH suppression tests (OGTT)
  • Radiographic imaging (MRI)
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14
Q

What is the GOLD STANDARD dx test to dx Pituitary Gigantism?

  • What lab value has to be elevated to move onto this test?
  • How does the test work?
  • What is a “normal” result?
A

GH Suppression Tests (OGTT)

  • Used if IGF-1 suggests GH excess
  • Serum GH response to an oral glucose load
  • Normal: GH concentration falls below tng/dL within 2 hours of glucose load.
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15
Q

What 2 structures are analyzed on radiographic imaging to dx Pituitary Gigantism?

What type of imaging?

A

MRI of hypothalamus & pituitary gland

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16
Q

Definition of what?

◦Excessive growth hormone (GH) after puberty

◦Mean age - 40-45 years

–Diagnosis delayed due to slow progression of signs and symptoms

A

Acromegaly

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17
Q

What is the MC cause of Acromegaly?

A

GH secreting adenoma of pituitary

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18
Q

What are the somatic effects of Acromegaly?

A

Stimulation of growth of tissues

  • Skin
  • Connective tissue
  • Cartilage
  • Bone
  • Viscera
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19
Q

What are the 2 metabolic effects of Acromegaly?

A
  • Insulin antagonism
  • Lipogenesis
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20
Q

Adenoma of pituitary causing Acromegaly may cause what 3 local sxs?

A
  • HA
  • Visual field defects (bitemporal hemianopsia)
  • CN palsies
21
Q

Clinical manifestations of Acromegaly

  • Insidious onset
  • SLOW progression
  • Avg interval from onset of sxs –> until dx is usually how many years?
  • At diagnosis, 75% have what??
  • 60% have what sxs?
  • 10% have what sxs?
A
  • 12 year interval
  • 75%: macroadenomas (10mm or greater)
  • 60%: HA
  • 10%: visual sxs
22
Q

What type of growth associated w/ Acromegaly is described here?

  • Enlarged jaw (macrognathia)
  • Enlarged, swollen hands and feet
    • ↑ shoe and glove size, need to enlarge rings
  • Facial features
    • Enlargement of nose and frontal bones
    • Teeth become spread apart
A

Acral Overgrowth

23
Q

What type of growth is described? (Acromegaly)

◦Synovial tissue/cartilage enlarge

◦Hypertrophic arthropathy of knees, ankles, hips, spine

◦Back pain and kyphosis

A

Articular Overgrowth

24
Q

Acromegaly

  • Bone density increases in what 2 structures?
  • 3 results of CVD?
  • Height changed how?
A
  • Spine & hips
  • HTN, LVH, cardiomyopathy
  • Height NOT necessarily taller (depends on the onset of GH excess)
25
Q

Sxs of acromegaly caused by what??

◦Women menstrual dysfunction, hot flashes and vaginal atrophy

◦Men erectile dysfunction, loss of libido, decreased facial hair growth and testicular atrophy

A

Decreased secretion of gonadotropins due to a Macroadenoma

(Pituitary Function messed up)

26
Q

What skin/hair changes are associated w/ acromegaly??

A

◦Skin thickening and skin tags

◦Hyperhidrosis is common (~50%), often malodorous

◦Increased hair growth and hirsutism

(THICK, hairy, sweaty, smelly skin)

27
Q

3 Soft Tissue clinical manifestations of acromegly

A

◦Macroglossia

◦Deepening of the voice

◦Paresthesias of the hands (CTS ~ 20%) = carpal tunnel syndrome

28
Q

Acromegaly

  • Macroglossia puts pts at risk for what??
  • 50% develop this…
A

OSA

29
Q

Acromegaly pts are at increased risk of what 3 tumors?

A
  • Benign uterine tumors
  • Colonic adenomatous polyps
  • Adenocarcinoma of colon/stomach/esophagus/melanoma

(10% develop malignant tumors)

30
Q

Acromegaly pts have enlargment of what organs? (5)

A

Visceral organs

  • Thyroid
  • Heart
  • Liver
  • Kidneys
  • Prostate

“Thy heart lives kindly and proudly”

31
Q

What are the 2 common laboratory abnormalities of Acromegaly??

A
  • Hyperglycemia (25% w/ overt DM)
  • Hyperprolactinemia (>200)
32
Q

Acromegaly

  • Mortality rate is 2-3X the expected rate, mostly due to what 2 conditions?
  • Reduced overall survival by how many yrs on avg?
A
  • CVD & Cancer
  • 10 yrs
33
Q

T/F

  • Treatment of Acromegaly which lowers GH to normal levels DOES NOT return mortality rate to normal
A

False, tx which lowers GH to normal WILL return mortality rate to normal

34
Q

What is the SINGLE BEST test for acromegaly??

A

Serum IGF-1 Concentration

35
Q

What test has + results in over 85% of acromegaly pts?

(Do this test if IGF-1 is elevated and still have clinical suspicion for Acromegaly)

A

OGTT (Oral Glucose Tolerance Test)

  • Negative/Normal result: GH falls to 2 or less within 2 hours after ingestion of glucose
  • Positive/Abnormal result: GH values are >
36
Q

What test would you do if IGF-1 was elevated and OGTT had inadequate suppression and you’re still suspicious for Acromegaly?

A

MRI of pituitary

  • If normal: get Chest/Abd CT
  • If + = GH secreting pituitary adenoma
37
Q
A
38
Q

What are the 3 goals of therapy for BOTH Pituitary Gigantism & Acromegaly

A
  • Lower serum GH
  • Get IGF-1 within reference range for age/sex of pt
  • Identify/Remove Tumor surgically, destroy w/ raidation, or reduce w/ drug therapy
39
Q

T/F

  • Treatment of Pituitary Gigantism & Acromegaly often does NOT restore normal GH secretion
A

True

40
Q

What are the 3 tx options for both Pituitary Gigantism & Acromegaly?

A
  • Surgery (Transphenoidal) (TX OF CHOICE!!)
  • Radiation (cranial)
  • Pharm:
    • ​Bromocriptine,
    • Octreotide,
    • GH receptor antagonists,
    • Combo therapy (Bromo+Octreotide in children)
41
Q

Transphenoidal Surgery is Treatment of Choice for Pituitary Gigantism AND Acromegaly:

  • Curative for micro/macro adenomas
  • Complication/Recurrence rates are higher in adults or children??
  • GH secretion falls to normal in 80% of pts w/ which type of adenoma? (better success)
  • How soon after surgery do GH levels fall to normal??
  • How soon do serum IGF-1 level fall to normal??
A
  • CHILDREN
  • Microadenomas (<10mm) = better success compared to macroadenomas (low success rate)
  • GH: 1-2 hours
  • IGF-1: 7-10 days
42
Q

What can result from Cranial Radiation as an adjunctive therapy in treatment of PG & Acromegaly?

A
  • Children:
    • delayed efficacy
    • Panhypopituitarism
    • Deficiencies of: GH, gonadotropin, thyrotropin
  • Learning disabilities
  • Emotional changes
  • Obesity

(LEPOD)

43
Q

Which pharm tx for PG & Acromegaly?

◦a dopamine analog, was the agent used most commonly until recently

A

Bromocriptine

44
Q

Which pharm tx for PG & Acromegaly?

  • somatostatin analog****
  • Highly effective - both adults and children with GH excess - most common agent
  • Subcutaneous injection or continuous infusion
A

Octreotide

45
Q

Long Term Management of PG & Acromegaly consists of what 3 things & how often??

A
  1. Clinical exams
  2. Serum IGF-1
  3. OGTT

(Every 3-4 months)

46
Q

Long Term Management of PG & Acromegaly

  • Monitor for S/S of pituitary hormone deficiency how often??
    • Gonadotropin deficiency - amenorrhea in women/androgen deficiency in men
    • Secondary thyroid and adrenal insufficiency
    • Measure serum levels of what 2 things?
A
  • Annually
  • Thyroxine & Cortisol
47
Q

Long Term Management of PG & Acromegaly

  • To monitor size of adenoma, an MRI should be repeated how often?
  • What other assessment needs to be completed and how often?
A
  • Repeat yearly for the first “several years” after surgery
  • Visual Field Assessment (Semi-annually)
48
Q

Systemic Evaluation of Long Term Management of pts/ w PG or Acromegaly:

  • Colonoscopy how often/age?
  • CV exam?
A
  • 3-4 yr intervals in pts 50 y/o+
  • Annual cardiovascular eval
49
Q

Summary of PG and Acromegaly: 8 steps

A
  1. Good H&P
  2. Follow growth curves
  3. If suspicious for pathologic tall stature:
  4. Obtain bone age with wrist radiograph
  5. Rule out precocious puberty, hyperthyroidism, and Klinefelter’s or Marfan’s
  6. IGF-1
  7. Confirm with GH suppression test
  8. Refer to pediatric or adult endocrinology

F3 E4 Shea (12 decks)

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