Pituitary Pathologies Simplified

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“Simple can be harder than complex: You have to work hard to get your thinking clean to make it simple. But it’s worth it in the end because once you get there, you can move mountains.” - Steve Jobs Friday, March 21, 14

description

Presentation at the 2014 Philippine Society of Endocrinology & Metabolism annual convention

Transcript of Pituitary Pathologies Simplified

Page 1: Pituitary Pathologies Simplified

“Simple can be harder than complex:You have to work hard to get your thinking clean to make it simple. But it’s worth it in the end because once you get there, you can move mountains.”

- Steve Jobs

Friday, March 21, 14

Page 2: Pituitary Pathologies Simplified

Iris Thiele Isip Tan MD, MScAssoc. Prof. 4, UP College of Medicine

Chief, Medical Informatics Unit

GPs asPituitaryStalkers

Pituitary Pathologies

Simplified

David and Goliath by Robert Temple Ayres

Friday, March 21, 14

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to be aPituitaryStalker

How

optical_illusion_tshirt by Kevinhttps://flic.kr/p/a573df

Remember by clsawyerhttp://www.sxc.hu/photo/659706

Ask Remember Refer

Friday, March 21, 14

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to be aPituitaryStalker

How

“The world is full of obvious things which nobody by any

chance ever observes.”- Sherlock Holmes

optical_illusion_tshirt by Kevinhttps://flic.kr/p/a573df

Have a questioning mind!

Ask

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David and Goliath

Who was

the real

underdog?

David and Goliath by Robert Temple Ayres

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DidGoliathacromegaly

have?

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David and GoliathPainting by Giovan Francesco Nagli

Height “6 cubits and a span” (2.5-3 meters)Slow-moving and ponderousGoliath saw “sticks”Stone sunk into foreheadGoliath fell forward

DidGoliathacromegaly

have

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38/F previously healthy

3-month history of

blurring of vision

headache by beermughttp://www.sxc.hu/photo/1413980

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Optometrist or ophthalmologist?

Prescribed eye glasses

Progressed to dimness of

vision

frame on eyechart by Brybshttp://www.sxc.hu/photo/1152188

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Diffuse throbbing headaches

followed by progressive blurring of visionFamily physician?

Internist?Neurologist?

headache by beermughttp://www.sxc.hu/photo/1413980

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Menses stopped 6 mos ago

followed by the

headaches

Gynecologist?

No galactorrhea

Endocrinologist?

headache by beermughttp://www.sxc.hu/photo/1413980

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Who makes the initial diagnosis of a pituitary lesion?

Diagnostic Source

Total percentage (n=404)Total percentage (n=404)Total percentage (n=404)Diagnostic Source Prolactinoma Non-functioning Acromegaly

Internist/Family Med 16 23 39Endocrinologist 34 14 25

Gynecologist 34 10 <1

Ophthalmologist/ Optometrist

6 25 6

Relatives/ self 2 1 6

Neurologist 0 3 5

Dentist 0 0 5

Other 8 24 14

Drange et al JCEM 85:168–174, 2000

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Pituitary adenomas come to clinical attention

mass effects

hormone excess

hormone insufficiency

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to be aPituitaryStalker

How

optical_illusion_tshirt by Kevinhttps://flic.kr/p/a573df

Remember by clsawyerhttp://www.sxc.hu/photo/659706

Ask Remember

Illness script

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Key Elements of the Clinical Diagnostic Reasoning Process Bowen J. NEJM

2006;355:2217-2225

Patient’s story

Data acquisition

Accurate “problem representation”

Generation of hypothesis

Search for and selection of illness script

Diagnosis

Knowledge

Context

Experience

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Expert clinicians store and recall knowledge as diseases, conditions, or syndromes - “illness scripts” - that are connected to problem representations.These representations trigger clinical memory, permitting the related knowledge to become accessible for reasoning.

A room lamp without lampshade by Mattoxhttp://www.sxc.hu/photo/1120345

Bowen J. NEJM 2006;355:2217-2225

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Illness Script - Structure Bowen J. NEJM 2006;355:2217-2225

Predisposing conditions

Pathophysiological insult

Clinical consequences

Rare 17% of populationEqual sex incidenceIncidence increases with age

Tumor growthCompression of optic nerveExtension to cavernous or sphenoid sinusCompression of normal pituitary

Non-functioningpituitary adenoma

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By OpenStax College [CC-BY-3.0 (http://creativecommons.org/licenses/by/3.0)], via Wikimedia Commons

and The Pituitary Optic Chiasm

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Image from http://www.medicinemcq.com/index.php/journals/sub_details/138/39/FMGE-MARCH-2012-ENT-AND-OPHTHALMOLOGY

and The Pituitary Cavernous Sinus

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Illness Script - Structure Bowen J. NEJM 2006;355:2217-2225

Predisposing conditions

Pathophysiological insult

Clinical consequences

Rare 17% of populationEqual sex incidenceIncidence increases with age

Tumor growthCompression of optic nerveExtension to cavernous or sphenoid sinusCompression of normal pituitary

Nonspecific headacheVisual disturbances (bitemporal hemianopsia)Ophthalmoplegia, diplopia and/or ptosisCSF rhinorrheaAnterior pituitary failure (hypopituitarism)

Non-functioningpituitary adenoma

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Image from http://www.medicinemcq.com/index.php/journals/sub_details/138/39/FMGE-MARCH-2012-ENT-AND-OPHTHALMOLOGY

Bitemporal hemianopsia

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Illness Script - Structure Bowen J. NEJM 2006;355:2217-2225

Predisposing conditions

Pathophysiological insult

Clinical consequences

Most common pituitary tumorF:M ratio 10:1

Neoplastic transformation of anterior pituitary lactrotrophs resulting in excess synthesis and secretion of prolactin

Prolactinoma

Oligo- or amenorrheaGalactorrheaErectile dysfunction or decreased libidoHypopituitarism (compression from large tumor)

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Hypergonadotropic hypogonadismOvarian failure

Hyper-prolactinemia

galactorrhea

headache

amenorrhea

Pituitaryadenoma

visual disturbances

Discriminating feature

Defining feature

Discriminating feature

Discriminating feature

Defining feature

Hypo-gonadotropic

hypogonadism

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Patient presenting with secondary amenorrhea;negative pregnancy test

Check TSH and prolactin levels

Both normal

Normal TSHAbnormal prolactin

Progestogen challenge test

No withdrawal bleed

Estrogen/progestogen challenge test

Withdrawal bleed

Normogonadotropichypogonadism

No withdrawal bleed

Outflow obstruction

Withdrawal bleed

Check FSH and LH

FSH >20 IU/LLH >40 IU/L

Hypergonadotropic hypogonadism

FSH & LH <5 IU/L

Perform MRI to evaluate for pituitary tumor

Normal MRI: hypogonadotropic hypogonadism

Normal prolactinAbnormal TSH

Thyroid disease

Prolactin <100 ng/mL(100 mcg/L)

Consider other causes

Prolactin >100 ng/mL

Perform MRI to evaluate for prolactinoma

Negative MRI

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What happens when you can’t match an illness script?

59/F with dizziness, vomiting and weakness ...

Rounadabout by ColinBroughttp://www.sxc.hu/photo/1265027

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Third time admitted for vomiting, dizziness and general weakness

Previous admissions also with hypoglycemia and pneumonia or UTI

Normal endoscopy end to end ...

What to do?!

Photo by Ashley Webbhttps://flic.kr/p/5u36oV

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Adrenal insufficiency?!

31 years ago Postpartum bleeding needing BT Delivered twins at homeRetained placenta

Screaming by ralaenin http://www.sxc.hu/photo/579286

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Illness Script - Structure Bowen J. NEJM 2006;355:2217-2225

Predisposing conditions

Pathophysiological insult

Clinical consequences

5.1 per 100,000 womenSevere postpartum vaginal bleedingSevere hypotension or shock requiring fluid replacement or blood transfusion

Sheehan’s syndrome

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Illness Script - Structure Bowen J. NEJM 2006;355:2217-2225

Predisposing conditions

Pathophysiological insult

Clinical consequences

5.1 per 100,000 women (Iceland)Severe postpartum vaginal bleedingSevere hypotension or shock requiring fluid replacement or blood transfusion

Pituitary enlargementLactotroph hyperplasiaPituitary gland susceptible to ischemiaPostpartum pituitary necrosis

Failure of postpartum lactationFailure to resume regular mensesVarying degrees of hypopituitarismEmpty sella on CT scan or MRI

Sheehan’s syndrome

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Sheehan’s SyndromePresentation

At ER following a stressful event

Coma of hypothyroidismAdrenal insufficiencyHypoglycemiaHyponatremia

http://www.sxc.hu/photo/65898Ozkan& Kolac, Neuroendocrinol Lett 2005;26(3):257-60

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GH deficiency

Reduced energy and vitalityReduced muscle mass and strengthDecreased sweating and impaired thermogenesisIncreased central adiposityIncreased CV riskDecreased BMD

Prabhakar & Shalet, Postgrad Med J 2006;82:259–66

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ACTH deficiency

Fatigue, weakness, anorexia, weight lossNausea, vomiting, abdominal painHypoglycemiaCirculatory collapse if acute onsetLoss of axillary and pubic hair in women

Prabhakar & Shalet, Postgrad Med J 2006;82:259–66

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Gonadotrophin deficiencyMen: erectile dysfunction, soft testes, reduced muscle mass and energyWomen: oligo/amenorrhea, dyspareunia, breast atrophyBoth: loss of libido, flushes, infertility, regression of sexual characteristics, reduced BMD

Prabhakar & Shalet, Postgrad Med J 2006;82:259–66

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TSH deficiencyFatigue, apathy, psychomotor retardationCold intolerance, dry skinConstipationWeight gain

Prabhakar & Shalet, Postgrad Med J 2006;82:259–66

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Illness Script - Structure Bowen J. NEJM 2006;355:2217-2225

Predisposing conditions

Pathophysiological insult

Clinical consequences

40-70 cases per million populationMean age of onset 30 yOnset to diagnosis 5-15 yMean age at diagnosis: 40 y in men and 45 y in women

GH excessGH-secreting pituitary adenomas

Inc incidence of colonic polyps & colon adenoCA, diabetes, hypertension and CVDSoft tissue swelling & enlargement of extremitiesIncrease in ring and/or shoe sizeHyperhidrosisCoarsening of facial featuresPrognathism, macroglossia

Acromegaly

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Rondo HattonAmerican Actor

Acromegaly attributed to exposure to German mustard gas attack in World War I

http://i1139.photobucket.com/albums/n545/travislouie37/Rondoisgoodwiththeladies.jpg

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Sheehan’s syndrome

Non-functioning adenoma

hypopituitarism

Functioning Pituitaryadenoma

Discriminating feature

Defining feature

Discriminating feature

Discriminating feature

mass effects

ACTH

TSH

Excess secretion

PRL

GH

Hormone deficiency

Postpartum hemorrhage

Defining feature

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At rounds more than a decade ago with MALA ...How much does a

human pituitary gland weigh?

Seagull in front of sea by cobrasoft http://www.sxc.hu/photo/1437851

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“But what physician has not had patients who don’t make any sense at all? To tell the truth, they’re our stock-in-trade. We talk and write about the ones we can make sense of.”

- Walker Percy

Dr. House MD Caricature Hugh Laurie by Nelson Santoshttps://flic.kr/p/46tXvx

Friday, March 21, 14

Page 42: Pituitary Pathologies Simplified

to be aPituitaryStalker

How

optical_illusion_tshirt by Kevinhttps://flic.kr/p/a573df

Remember by clsawyerhttp://www.sxc.hu/photo/659706

Ask Remember Refer

Friday, March 21, 14

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Thank You@endocrine_witch

Friday, March 21, 14