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

Pituitary Gigantism (rare) GH secreting pituitary adenoma/tumor

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?

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.

Pituitary 2 Flashcards by Shea Green

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

Constitutional Tall Stature

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

Klinefelter’s Syndrome
Marfan’s Syndrome

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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

Klinefelter’s Syndrome

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Which genetic syndrome causing Tall Stature?

◦- inherited autosomal dominant disorder - connective tissue disease

Tall stature

Long, thin fingers and hyperextensibility of joints

Marfan’s Syndrome

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3 endocrine causes of Tall Stature

Pituitary Gigantism (rare)
Precocious Puberty
Hyperthyroidism

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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

Pituitary Gigantism (rare)

GH secreting pituitary adenoma/tumor

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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

Precocious Puberty

Girls

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What are 4 causes of precocious puberty?

Congenital adrenal hyperplasia,
virilizing adrenal tumors,
testicular and ovarian tumors,
ovarian cysts

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Which cause of tall stature?

◦Overproduction or over-treatment with exogenous thyroxine

◦Increased growth and advanced bone age

Hyperthyroidism

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Is excess GH or PRL more common?

PRL (prolactin)
GH

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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?

Chromosomal cause: Klinefelter’s Syndrome

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

bone age,
thyroid function tests,
sex steroid hormone concentrations,
karyotype,
GH-related studies

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3 diagnostic components to dx pituitary gigantism

IGF-1 levels
GH suppression tests (OGTT)
Radiographic imaging (MRI)

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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?

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

What type of imaging?

MRI of hypothalamus & pituitary gland

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Definition of what?

◦Excessive growth hormone (GH) after puberty

◦Mean age - 40-45 years

Diagnosis delayed due to slow progression of signs and symptoms

Acromegaly

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What is the MC cause of Acromegaly?

GH secreting adenoma of pituitary

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What are the somatic effects of Acromegaly?

Stimulation of growth of tissues

Skin
Connective tissue
Cartilage
Bone
Viscera

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What are the 2 metabolic effects of Acromegaly?

Insulin antagonism
Lipogenesis

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

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HA
Visual field defects (bitemporal hemianopsia)
CN palsies

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?

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12 year interval
75%: macroadenomas (10mm or greater)
60%: HA
10%: visual sxs

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

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Acral Overgrowth

What type of growth is described? (Acromegaly)

◦Synovial tissue/cartilage enlarge

◦Hypertrophic arthropathy of knees, ankles, hips, spine

◦Back pain and kyphosis

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Articular Overgrowth

Acromegaly

Bone density increases in what 2 structures?
3 results of CVD?
Height changed how?

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Spine & hips
HTN, LVH, cardiomyopathy
Height NOT necessarily taller (depends on the onset of GH excess)

**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

**Decreased secretion of gonadotropins** due to a _Macroadenoma_ (Pituitary Function messed up)

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

◦Skin thickening and skin tags ◦Hyperhidrosis is common (~50%), often malodorous ◦Increased hair growth and hirsutism ***(THICK, hairy, sweaty, smelly skin)***

3 Soft Tissue clinical manifestations of acromegly

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

**Acromegaly** * _Macroglossia_ puts pts at risk for what?? * 50% develop this...

OSA

Acromegaly pts are at increased risk of what 3 tumors?

* Benign uterine tumors * Colonic adenomatous polyps * Adenocarcinoma of colon/stomach/esophagus/melanoma (10% develop malignant tumors)

Acromegaly pts have enlargment of what organs? (5)

**_Visceral organs_** * Thyroid * Heart * Liver * Kidneys * Prostate "**Thy** **heart** **liv**es **ki**ndly and **pro**udly"

What are the 2 common laboratory abnormalities of Acromegaly??

* Hyperglycemia (25% w/ overt DM) * Hyperprolactinemia (\>200)

**Acromegaly** * Mortality rate is 2-3X the expected rate, mostly due to what 2 conditions? * Reduced overall survival by how many yrs on avg?

* CVD & Cancer * 10 yrs

**T/F** * Treatment of Acromegaly which lowers GH to normal levels DOES NOT return mortality rate to normal

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

What is the SINGLE BEST test for acromegaly??

Serum IGF-1 Concentration

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)

**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 \>

What test would you do if _IGF-1 was elevated and OGTT had inadequate suppression_ and you're still suspicious for **Acromegaly?**

MRI of pituitary * **If normal:** get Chest/Abd CT * **If + =** GH secreting pituitary adenoma

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

* 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

**T/F** * Treatment of Pituitary Gigantism & Acromegaly often does NOT restore normal GH secretion

True

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

* **Surgery** (Transphenoidal) (TX OF CHOICE!!) * **Radiation** (cranial) * **_Pharm:_** * ****Bromocriptine, * Octreotide, * GH receptor antagonists, * Combo therapy (Bromo+Octreotide in children)

**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??

* **CHILDREN** * **Microadenomas** (\<10mm) = better success compared to macroadenomas (low success rate) * **GH:** 1-2 hours * **IGF-1:** 7-10 days

What can result from _Cranial Radiation_ as an adjunctive therapy in treatment of PG & Acromegaly?

* **Children:** * delayed efficacy * **P**anhypopituitarism * _**D**eficiencies of:_ GH, gonadotropin, thyrotropin * **L**earning disabilities * **E**motional changes * **O**besity ## Footnote ***(LEPOD)***

**Which pharm tx for PG & Acromegaly?** ◦a dopamine analog, was the agent used most commonly until recently

Bromocriptine

**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

Octreotide

Long Term Management of PG & Acromegaly consists of _what 3 things & how often_??

1. Clinical exams 2. Serum IGF-1 3. OGTT (Every 3-4 months)

**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?

* Annually * Thyroxine & Cortisol

**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?

* Repeat yearly for the first "several years" after surgery * Visual Field Assessment (Semi-annually)

**Systemic Evaluation of Long Term Management of pts/ w PG or Acromegaly:** * Colonoscopy how often/age? * CV exam?

* 3-4 yr intervals in pts 50 y/o+ * Annual cardiovascular eval

Summary of PG and Acromegaly: 8 steps

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

Pituitary 2 Flashcards

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