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DT4-Exam 1 > Pituitary Adrenal & Pancreas- Leah : ) > Flashcards

Pituitary Adrenal & Pancreas- Leah : ) Flashcards

1
Q

List the 6 anterior pituitary hormones

A
  • GH
  • LH
  • FSH
  • ACTH
  • TSH
  • PRL
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2
Q

List the 2 posterior pituitary hormones.

Where are they synthesized?

A
  • Oxytocin, ADH
  • synthesized by hypothalamic neurons
  • sent to pituitary via neuronal axons
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3
Q

Embryologic source of the anterior pituitary

A

oral ectoderm –> rathke’s pouch

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4
Q

Location of pituitary gland in relation to brain and skull:

In what structure does it sit?

A
  • below hypothalamus
  • Sella Turcica of the sphenoid bone
  • Surrounded by diaphragma sella
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5
Q

How is the pituitary connected to the hypothalamus?

A

-Infundibulum/ stalk: neuronal connection to POSTERIOR pituitary
(But note compression of the stalk affects anterior + posterior pituitary)

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6
Q

Why is the anterior pituitary subject to ischemia?

A
  • low pressure venous system (portal system) supplies the tissue
  • no direct arterial supply
    (same system delivers trophic hormones from the hypothalamus)
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7
Q

What are the three main cells of the anterior pituitary?

What hormones do each secrete?

A
  • acidophils (GH, PRL)
  • basophils (ACTH, FSH, LH)
  • chromophobe (TSH)
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8
Q

The release of what two anterior pituitary hormones can be INHIBITED?
What are the inhibitory signals?

A

-DA inhibits the release of prolactin
-SOMATOSTATIN inhibits the release of GH
(DA and somatostatin come from the hypothalamus)

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9
Q

What are the stimulatory hormones of the hypothalamus?

A

Stimulatory hormones from the hypothalamus are “releasing” hormones: TRH, GnRH, etc.

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10
Q

Hyperpituitarism:
most common cause
three other causes?

A
  • Adenomas = #1 (10% intracranial neoplasms)

- Also: Hyperplasia, carcinoma, non-pituitary/hypothalamic causes

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11
Q

What is a macro vs microadenoma of the pituitary?
What syndrome might these tumors be assc with?
Age group

A
  • MICRO is LESS than 1 cm
  • MACRO GREATER than 1 cm
  • 3% related to MEN1
  • Affects ADULTS of most any age (craniopharyngioma = childhood pituitary lesion)
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12
Q

Mass effects of adenoma (all types, 4):

A
  • Erosion/ expansion of sella on CT
  • Bitemporal hemianopia “MOST IMPORTANT THING”
  • ^^ ICP
  • Compression –> HYPOpituitarism
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13
Q

Where do larger pituitary tumors expand?

A

-expand SUPERIORLY–> compress chiasm

hard to go posterior due to enclosure of pituitary in the sella

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14
Q

Pituitary adenoma:
general gross appearance
malignant or benign?
histo appearance?

A

-round, well-circumscribed
-benign
-monomorphic cells + loss of reticulin fibrin network
(may see compressed normal fibrin network)

*Monomorphic: all cells one type, basophils vs. acidophils etc.

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15
Q

Pituitary adenomas are aggressive when assc with what genetic mutation?

A

–Rb
we always learn about this gene for eye tumors but it can be in most any tumor!!

(for example, I had an Rx question about the Rb gene in osteosarcoma– another aggressive tumor)

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16
Q

Atypical adenoma: describe it (3 features); what is the prognosis?

A
  • ^^ mitoses
  • ^^ KI67 stain (just means lots of dividing cells)
  • p53 reactivity

(worse prognosis than normal adenomas)

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17
Q

Only criteria for pituitary adenocarcinoma?

Assc mutation?

A
  • evidence of distal mets

- ACTIVATING mutations in HRAS

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18
Q

Most common TYPE of hyperfunctional pituitary adenoma?

Two histo findings?

A
  • prolactinoma
  • eosinophilic (acidophils secrete PRL)
  • psammoma bodies
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19
Q

Signs/ Sx specific to prolactinoma (3):

A
  • amennorhea, infertility
  • loss of libido
  • galactorrhea
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20
Q

Who is diagnosed late in the case of prolactinoma?

A

-post-menopausal women (don’t notice infertility/ amennorhea)

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21
Q

Causes of ^^ PRL that are not caused by prolactinomas (4)

A
  • physiologic stress (preggos, nursing, etc)
  • hypothalamic injury/ loss of DA
  • Drugs that decrease DA (phenothiazines, haloperidol)
  • renal failure, thyroid failure, ^^estrogen
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22
Q

Treatment for prolactinoma (2- 1 drug, 1 surgery)

A
  • Bromocriptine (DOPA agonist)

- transphenoidal surgery

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23
Q

Second most common typical hyperfunctional adenoma:

  • assc cell type?
  • 2 classic symptoms (adults vs kiddos)
A
  • GH adenoma
  • acidophils
  • acromegaly (adults) or gigantism (kids)
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24
Q

5 possible diseases assc with GH secreting adenomas:

aside from the 2 classic symptoms

A
  • glucose intolerance/ DM
  • HTN
  • osteoporosis + arthritis
  • CHF
  • muscle weakness

(I think of this as basically all of the disease that fat old people get. Growth hormone causes the diseases you see in people that grow old and grow fat.)

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25
Q

Those with pituitary adenomas (GH secreting) are at risk of developing what other cancers?

A

GI cancers

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26
Q

Corticotroph adenomas:
usual size
usual cell type
name of the assc disease?

A
  • MICROadenomas, less than 1 cm
  • basophils
  • Cushings DISEASE
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27
Q

Cushings Disease vs Syndrome: whats the difference?

A

-Cushings DISEASE = PITUITARY disease causing ^^^ ACTH.
-Cushings Syndrome = IATROGENIC (#1!!) or caused by OTHER endocrine organs
(adrenals ^ cortisol, ectopic release etc.)

(It should be a SYN for doctors to give patients lots of steroids.)

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28
Q

What is Nelson’s Syndrome?

A
  • Removal of adrenals –> to loss of negative feedback on pituitary/ACTH.
  • ^^^ ACTH release –> hyperpigmentation or pituitary apoplexy
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29
Q

What is pituitary apoplexy?

A

Acute hemorrhage/ Infarction of a big fat pituitary

  • Asstd. with rapidly growing adenoma
  • Caused by Nelson’s syndrome, post-partum etc.
  • Medical EMERGENCY (Panhypopituitarism: give replacement steroids)
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30
Q

Pitiuitary apoplexy:
symptoms
treatment

A
  • Panhypopituitarism–> HA, blurred vision, cardiac collapse, LOC, death
  • need mineralocorticoids and glucocorticoids
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31
Q

What’s a “Null Cell” Tumor?

A

-Produces no hormones, get only mass effect (possible Panhypopituitarism)

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32
Q

Symptoms of FSH/ LH secreting tumors?

A
  • Similar to Null Cell, may be asx –> could get loss of libido.
  • Will still cause mass effects.
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33
Q

How common are TSH secreting tumors?

A

-Very rare, less than one percent. Don’t see these.

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34
Q

Hypopituitarism causes loss of function of what other three organs?

A

-gonads
-adrenals
-thyroid
(symptoms observed accordingly– gonadal atrophy, pallor, loss of secondary sex features, hypotension: intuitive)

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35
Q

In the case of hypothalamic causes of hypopituitarism, what additional finding is assc?
(Finding that would not be seen in primary hypopituitarism)

A

-Lack of ADH, Diabetes Insipidus

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36
Q

Causes of hypopituitarism: (5)

A
  • “Null adenomas”: tumors that secrete NOTHING take over the gland
  • Sheehan syndrome (post partum hemorrhage)
  • Ischemic necrosis: SCA, shock, DIC, ^^ ICP
  • POU1FI mutation
  • Empty sella syndrome
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37
Q

What is empty sella syndrome?
What does it cause?
Classic patient?

A
  • Enlarged empty area of sella gets filled with CSF
  • Compresses the pituitary
  • Common in obese multiparous women (chronic hernia toon of subarachnoid space)
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38
Q

Symptoms of low oxytocin?
Symptoms of low ADH?
high ADH?

A
  • Oxy: NO clinical changes (Agreed, UNLESS PREGGOS/ POSTPARTUM)
  • low ADH: Diabetes Insipidus
  • high ADH: SiADH
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39
Q

Labs assc with DI (urine, blood)?

Life threatening complication?

A
  • decreased urine osmolality
  • HYPOvolemic HYPERnatremia
  • Life threatening dehydration–> polydypsia
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40
Q

Most common cause of SiADH?

-assc findings? (lab findings, urine findings)

A
  • neuroendocrine cancers; #1 = lung small cell carcinoma
  • HYPERvolemic hyponatremia
  • concentrated urine
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41
Q

What is a Rathke’s Cleft Cyst?
Histo findings?
Related disease?

A
  • vestigial remnant of the pars intermedia
  • ciliated cuboidal/ columnar epi–> squamous epi (metaplasia)
  • Causes DI by compression of posterior pit.
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42
Q

Lymphocytic Hypophysitis:

  • Who gets it?
  • Two assc findings + one histo finding?
A
  • post-partum/ preggos autoimmune reaction
  • ^^PRL, low ACTH
  • Lymphocytic infiltrate
  • not really clinically relevant
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43
Q

Granulomatis Hypophysitis:

  • Symptoms
  • Classic histo
A
  • classic MASS effects of pituitary lesion (visual Sx. N/V, HA)
  • variable hypopituitarism
  • lymph and histiocytes infiltrate

(Both sexes get this, lymphocytic was in post-partum females)

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44
Q 
Craniopharyngioma:
prevalence of this tumor?
benign or malignant?
age group
Assc symptoms based on age group?
Source?
A
  • 1-5% intracranial tumors, BENIGN
  • bimodal distribution, kids + adults
  • Kids: hypopituitarism
  • Adults: mass effect (i.e. hemianopia)
  • comes from Rathke’s Pouch epithelium
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45
Q

Craniopharyngioma:
assc genetics-
histo findings-
Finding in surgery?

A

Genetics: WNT/ B-catenin mutations

Histo: basaloid epi/ adamintomatous/ keratin/cholesterol clefts/ calcification etc.

Surgery: “MOTOR OIL” texture, keratin in the tumor produces oily substance.

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46
Q

Pineal gland:

  • Location
  • two cell types
  • function (wha does it secrete?)
A
  • next to superior colliculi of midbrain
  • neuroglial and epithelial cells
  • photosensory organ –> secretes melatonin
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47
Q

Pineal gland:

what kinds of tumors can it get?

A
  • any tumor occuring in gonads, because germ cells are present
  • aka the third gonad
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48
Q

Three layers of the adrenal cortex and what does it secrete?

A

-GFR like the kidney
zona: glomerulosa –> fasiculata –> reticularis
-the layers respectively secrete salt –> sugar –> sex; the deeper you go, the sweeter it gets
(salt= mineralocorticoids, sugar = glucocorticoids, sex= androgens)

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49
Q

What type of cells are found in the adrenal medulla and what do they secrete?

A
  • chromaffin cells

- secrete catecholamines

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50
Q

Three types of hyperadrenalism:

A
  • Hypercortisol (Cushings)
  • Hyperaldo (Conn’s)
  • Hyperadrenogenital syndromes
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51
Q

4 causes for excess cortisol + which is most common?

Which is second most common?

A

-#1 iatrogenic
-Hypersecretion of ACTH by HPA axis
(#1 ENDOGENOUS cause)
-Adrenocortical neoplasm or hyperplasia
-Neuroendocrine tumors–> ectopic ACTH

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52
Q

What causes adrenal hyperplasia?

A

-AR inherited enzyme deficincies

Yes, enzyme disorders USUALLY = AR!

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53
Q

How is dexamethasone used in diagnosing potential causes of increased ACTH?

A

Dexa = exogenous glucocorticoid–> Suppression testing

  • HIGH DOSE Dexa–> DECREASE ACTH–> PITUITARY lesion
  • NO RESPONSE to Dexa–> ADRENAL or ECTOPIC lesion
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54
Q

Classic signs of Cushings (5)?

A
  • fast twitch muscle fiber atrophy
  • moon facies
  • Buffalo hump + Belly fat w striae
  • hyperlycemia/ glucosuria/ polydypsia/ DM
  • behavioral changes, poor wound healing
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55
Q

How does EXOGENOUS Cushings effect the adrenal glands? ENDOGENOUS?

A
  • exogenous/ iatrogenic: SMALL adrenals
  • endogenous: adrenal HYPERPLASIA
  • *unilateral hyperplasia may be adrenal neoplasm
  • *bilateral nodular hyperplasia = ^^^ ACTH
56
Q

Adrenal adenoma vs carcinoma:
gross appearance?
benign or malignant?
unilateral or bilateral?

A

-adenoma: yellow (lipid filled), encapsulated, benign

  • carcinoma: larger, hemorrhagic/necrotic, +/- capsule, malignant
  • both usually unilateral*
57
Q

Adrenal adenomas vs. carcinomas:

  • Which is “functional”?
  • Which is more common?
A
  • adenomas more common
  • carcinomas larger, invasive, and functional

*adenomas often hormonally silent

58
Q

Adrenal tumor typical histo (adenoma vs. carcinoma):

A
  • benign adenomas: round little nests of pink cells (monomorphic)
  • malignant carcinomas: hemorrhage, necrosis; ^ mitosis, ^N/C ratio
59
Q

Changes seen in pituitary in response to adrenal neoplasms secreting cortisol?

A
  • Crooke’s Hyaline

- deposition of pink material (keratin) around PALE cells

60
Q

Response of the following lesions of dexamethasone: pituitary, ectopic, and adrenal Cushings?

A

-pituitary: responds to only very high doses of dexa.
(normal low doses= no response)

  • ectopic: no response to dexa
  • adrenal cause: no response to dexa but ACTH will already be LOW.
61
Q

Symptoms of hyperaldo:

A

-HYPERnatremia, HYPERtension, HYPOkalemia

Think of this as the OPPOSITE of spiro.

62
Q

Aldo secreting adrenal tumor:

  • Does it cause primary or secondary hyperaldo?
  • what population gets it?
  • effect on other adrenal gland
A

Conn’s Tumor (adenoma)

  • primary hyperaldo
  • middle aged women
  • NO effect on ACTH–> NO atrophy of adjacent adrenal gland
  • *In contrast to CORTISOL secreting tumors (although those are usually bialteral)
63
Q

Secondary hyperaldo:

causes (4)

A
  • CHF
  • decreased renal perfusion
  • hypoalbuminemia
  • pregnancy

(anything that will cause ^^ renin)

64
Q

What is glucocorticoid-supressible hyperaldo?

How is this possible?

A
  • aldo levels respond to administering exogenous dexa.
  • Familial, CYP 11B1/ 11B2 mutation–> HYBRID cells in ZG/ZF

(CPY11B1 + CYP11B2 = 11B Hydroxylase/ Aldo synthase hybrid)

65
Q

How do you treat adrenal neoplasms? hyperplasia?

A
  • hyperplasia: treat medically, symptomatically.

- neoplasm: surgical removal

66
Q

Two adrenal diseases assc with excess androgens?

A
  • Congenital hyperplasisa

- adrenocortical neoplasms (viruilizing carcinomas most common)

67
Q

How does congential adrenal hyperplasia work?
Whats the most common cause?
What is the inheritance pattern?

A

1 Deficient Enzyme = 21 B hydroxylase, AR

Enzyme deficiency–> LOW cortisol synthesis–> pituitary ^^ ACTH–> ^^ adrenal stimualtion + hypertrophy

68
Q

Populations predisposed to congenital adrenal hyperplasias?

Whats the rule for determining the effects of various adrenal enzyme deficiencies?

A

Ashekanzi Jews, Hispanics

  • (1) in the first position = HYPERtension
  • (1) in the second position = HYPERandrogens

(Note, no 1 in first position = salt wasting, HYPOtension)

69
Q

Adrenal Myelolipoma:

What is it made up of?

A

-Fat/ hematopoietic cells

70
Q

Chronic Adrenal Insufficiency:

  • Disease name:
  • Cause:
  • Lab Findings, Pituitary effect?
A
  • Addison’s
  • Autoimmune cause
  • low glucocorticoids
  • ^^^ACTH output + Crooke’s Hyaline
71
Q

Autoimmune Polyendocrine Syndrome Type 1 (APS1):
Assc findings (4):
How common is it?
Whats the assc gene?

A

Clinical findings:

  • candidiasis
  • hypogonadism/ adrenal insufficiency
  • pernicious anemia
  • skin findings(ectodermal dystrophy)
  • RARE cause of adrenal insufficiency
  • Mutation in AIRE gene (autoimmune regulator)
  • Failure of self tolerance
72
Q

What’s APS2 in comparison to APS1?

A
  • Similar autoimmunity causing adrenal insufficiency

- Lack the other findings

73
Q

Other causes of primary adrenal insufficiency (3):

Classic finding assc with most all types of adrenal insufficiency?

A
  • TB, fungi, infection, metastatic disease (usually lung)
  • Historically, TB a very common cause of adrenal insufficiency

-most cases have “hyperpigmentation”

74
Q

Secondary adrenal insufficiency causes

A

-lesions of the pituitary or hypothalamus

75
Q

Morphologic changes assc with autoimmune or TB induced chronic adrenal insufficiency (3): (i.e. Addisons)

A
  • lymphoid infiltrate of CORTEX
  • shrunken adrenals
  • Cavitary lesions in TB assc adrenal insufficiency
76
Q

What is the most common cause of ACUTE adrenal insufficiency?
What are the symptoms/ outcome?
What is the treatment?

A
  • rapid withdraw of cortisol/steroid treatment
  • no normal ACTH response in these patients due to ATROPHIC ADRENALS

Clinical presentation:

  • vomiting, ab pain
  • vascular collapse, coma, death

Tx: Replace cortisol IV !!

77
Q

What is a less common cause of adrenal insufficiency?

A

MASSIVE HEMORRHAGE

  • DIC
  • pregnancy/ birth trauma
  • Waterhouse Friedrichson Syndrome following neisseria (see blood and fibrin thrombi on histo)
78
Q

Why do newborns get acute adrenal insufficiency?

A

coagulopathy/ prothrombin deficiency –> adrenal basically becomes a ball of blood

*Same concept applies to other etiologies: warfarin, DIC etc. cause ^^ bleeding, adrenals susceptible to bleeding

79
Q 
Adrenal Medulla: 
embryologic source- 
cell type- 
products- 
types of cancers/ histo?
A

neural crest cells
chromaffin cells
ne/epi
All are neuroendocrine tumors (small blue cells, salt and pepper, ribbons and cords)

80
Q

Neuroendocrine stains?

A
  • silver stain

- synapto/ chromogranin

81
Q

What is the “rule of 10’s” for pheochromocytoma?

A

10% of pheos are:

  • bilateral
  • familial
  • malignant
  • NOT HTN assc (90% are)
82
Q

Symptoms and risks assc with pheo?

A
  • Mass/ pain
  • sympathetic symptoms (releases NE/Epi)
  • transitory severe HTN
  • can cause all diseases assc with HTN (CVA, MI, heart failure, sudden death etc.)
83
Q

What may precipitate pheo symptoms?

A
  • stress/ exercise (logical, you’re ramping up SNS)

- BUT ALSO: posture change and palpation of the mass

84
Q

How can pheo cause sudden death?

A

-^^ catechols –> ventricular arrythmia

85
Q

Pheo:

special cell type, stain, and pattern?

A
  • sustentacular cells
  • S100 positive
  • “Nodules” – Zell Ballen Pattern; whorls

(also note its a neuroendocrine tumor so it will have other stains like synapto/chromo etc.)

86
Q

How is pheo diagnosed

A
  • urinary catechols, VMA, Dopa ^^

- second step: CT

87
Q

Treatment for pheo

A

-remove adrenal if unilateral, must medically manage HTN if bilateral (10% cases)

88
Q

Neuroblastoma:

  • who gets this?
  • where is it located in the body?
  • If inherited, whats the inheritance pattern?
A
  • kids usually under 2 yoa (older = bad prog.)
  • can be anywhere in sympathetic chain (50% adrenal)
  • AD if inherited
89
Q

Neuroblastoma:

symptoms?

A
  • often non specific cancer sx
  • may have protuberant abdomen/ palpable mass at adrenals
  • may produce catechols (symptathetic sx), but not always.
90
Q

Neuroblastoma:

-two histo findings you should think of?

A
  • Homer Wright Rosettes

- Small round blue cells

91
Q

How do you diagnose neuroblastoma? What is the most specific marker?

A

Urine:

  • ^^ catechols/ VMA
  • BUT HVA ^^ is most specific
92
Q

Two genetic changes assc with AGRESSIVE neuroblastomas?

A
  • short arm chromosome 1 deleted

- myc amplification

93
Q

Genes assc with MEN I, IIa, and IIb/III

A
  • I: LOF tumor supressor “Menin”
  • IIa: RET GOF on chromosome 10
  • IIb/III: RET GOF, but different RET than IIa
94
Q

MEN assc tumors are generally preceded by…?

A

hyperplasias

95
Q

Tumors assc with MEN 1?

Whats the most common lesion?

A

3 P’s + duodenal gastrinomas
(pituitary, pancreas, parathyoid)

*most common lesion = hyperparathyroidism hyperplasia/adenoma

96
Q

MEN IIa: what tumors are assc?

A

2 medullary tumors + parathyroid hyperplasia

thyroid medullary carcinoma + pheo

97
Q

Thyroid medullary carcinoma:
benign or malignant?
preceded by?

A

-VERY AGRESSIVE
(so much that those with MEN II get prophylactic thyroidectomies)
-preceded by C cell hyperplasia

98
Q

What signs and symptoms are assc with hyperparathyroidism?

A

-^^ Ca = stones, groans, psychic overtones

groans = GI upset, psychic = anxiety/ depression etc.

99
Q

What is the difference in MEN IIa/IIb clinically?

A

-IIa = 2 Medullary + parathyroid hyperplasia
-IIb = 2 Medullary + Neuromas
(Remember, Medullary = Thyroid + Pheo)

-Both “RET” mutations.

100
Q

Populations susceptible to diabetes? (3)

A
  • Native americans
  • hispanics
  • african americans

(and of course obese people)

101
Q

DM is a leading cause of what other three diseases?

A
  • End stage renal disease (most common cause dialysis)
  • Blindness (PDR/NPDR)
  • Amputation (lower limb gangrene)
102
Q

In the islets of the endocrine pancreas, what do each of the following cells secrete?

  • a
  • b
  • delta

Also what is the function of pancreatic polypeptide?

A
  • a glucagon
  • b insulin
  • delta somatostatin

*pancreatic polypeptide stimualtes GI enzyme secretion

103
Q

What two endocrine pancreatic cells are NOT found in the islets? What do they secrete?

A
  • D1: VIP

- Enterochromaffin: serotonin

104
Q

Whats the function of VIP?
Remind me, where does it come from?
What is classically assc w/ excess VIP?

A
  • Iduces gylcogenolysis + gastric fluid secretion
  • Comes from D1 cells!! not in islets!! (exocrine cells)
  • EXCESS CAUSES WATERY DIARRHEA
105
Q

DM is chronic disorder of metabolism of what substances?

A

DONT GET TRICKED!!!
-carbs, AND FAT/ PROTEIN!!
All three.

106
Q

Type 1 DM is aka? Type II?

A

-I insulin dependent, juvenile
-II non-insulin dependent, adult onset
**But note that because kiddos are getting fat, they can also get type 2!
(Unless melissa makes them healthy in lets get moving)

107
Q

Long term, glucose effects what basic body parts? (4)

A
  • vessels
  • nerves
  • eyes
  • kidney
108
Q

Symptoms of DM1 and how do they come about? (5)

A
  • POLYURIA (osmotic diuresis)–> ^^ blood concentration
  • ^^ blood concentration–> POLYDYPSIA
  • Failure of glucose to enter cells–> POLYPHAGIA
  • Chronic fat breakdown–> DKA
  • Failure of glucose to enter cells + fat/ protein breakdown–> WEIGHT LOSS
109
Q

How does fat breakdown cause DKA?

Where are free fatty acids metabolized, what enzyme breaks them down, what do they form?

A
  • free fatty acids oxydized in liver by acetyl coenzyme A

- form ketoacids = acetoacetic acid, hydroxybuteric acid

110
Q

Two signs of DKA?

A
  • nausea/ vomiting
  • hyperventilation (to blow off the acid)
  • Kussmal breathing!!
111
Q

DM2 symptoms

A
  • polyuria/dypsia possible
  • usually Asx: incidental lab finding
  • hyperosmolar coma possible in severe untreated cases

(Hyperglycemia–> Severe dehydration–> Hyperosmolar non-ketotic coma)

112
Q

Pathogenesis DM1:

Pathogenesis DM2:

A

DM1: autoimmune attack on B cells –> loss of insulin

DM2: Obesity/chronically ^^ glucose –> peripheral insulin resistance –> chronic high glucose causes insulin decrease (B cel exhaustion) in late disease

113
Q

DM1:

name two factors that increase genetic susceptibility

A

HLADR3/4; Northern European Descent

(Note that individuals with DR3/4 usually DO NOT get DM1, but that the prevalence of these genes in DM1 patients is higher than in the general population.)

HLA-DQA1*301 + HLA=DQB1 *0302 have higher linkage disequilibrium w/ DR3/4

114
Q

What is early “insulitis” in DM1:
What cells mediate this?
What How are cells activated?
What does it look like on histo?

A
  • T cell (mostly CD8) mediated
    -^^ MHC Class 1/2 abberant expression
    -Lymphocytic infiltration on histo
    (obvious, but we don’t generally biopsy the pancreas– diagnosed clinically!)
115
Q

What antibodies are present in DM1?

Are these disease causing?

A
  • Islet cell AutoBb’s; commonly against glutamic acid decarboxylase (GAD)
  • THESE DON’T CAUSE DISEASE, they are a result of T cell attack (insulitis).
116
Q

Environmental triggers that may ^^ likelihood of a patient developing DM1?
(3)

A
  • coxsackie (release of Ag/ molecular mimicry– GAD shares coxsackie sequence)
  • drugs
  • toxins
117
Q

What do the islet cells of the pancreas look like in late type 1 DM and late type 2?

A
  • type 1: atrophy and depleted B cells

- type 2: atrophy, amyloid, mild B cell depletion

118
Q

What is glycosylated Hb? What is it used to measure?

A
  • ^^ levels of glucose allow the sugar to non-enzymatically attach to proteins, including Hb.
  • Levels of glycosylated Hb (Hb A1C) correlate to average blood glucose over 3 month period.
119
Q

What are advanced glycosylation end products?

What effects are they assc with?

A
  • glycosylated long-lived proteins
  • deposit in kidney (BM thickens)
  • allow LDL to deposit in vessels
120
Q

Three vascular problems assc with diabetes and which is the #1 killer in diabetics?

A
  • atherosclerosis
  • gangrene
  • MI (#1 killer)
121
Q

What is the second leading cause of death in diabetics?

What does it look like histologically? (3)

A

second cause of death= chronic kidney disease

  • mesangial thickening/ proliferation
  • Kimmelsteil Wilson nodules made of mucopolysaccharides (pink balls)
  • hyaline arteriolosclerosis
122
Q

Diabetics can develop pyelonephritis in addition to chronic kidney disease. What complication can result from the pyelonephritis?

A

-necrotizing papillitis

123
Q

Common fungal infection in diabetics?

How does it appear on culture?

A
  • mucor, right angle branching

* emergent URI*; mucor is INVASIVE

124
Q

3 effects of DM on the eyes

A
  • retinopathy (proliferative v non proliferative)
  • cataracts
  • glaucoma
125
Q

How is the nervous system effected in DM?

A

1: bilateral symmetric peripheral neuropathy

  • most often lower extremities lose sensation, but may also lose motor.
  • can see autonomic dysfxn, most commonly gastroparesis, but also diarrhea/ impotence etc
126
Q

How does diabetes effect the immune system?

A
  • ^^ risk infection (immunosuppressive state!)

- delayed healing

127
Q

What fasting blood sugar is diagnostic of DM?

What random glucose is diagnostic?

A
  • fasting above 126 is diagnostic, 100-125 is borderline

- random glucose above 200 is diagnostic.

128
Q

Most common types of pancreatic tumors:
functional or nonfunctional?
specific type that is most common?

A

1= B cell insulinoma

-functional, can secrete insulin or gastrin (decrease blood glucose; ^ HCL respectively)

129
Q

Location and gross appearance of neuroendocrine pancreatic tumors?
Histo appearance?

A

-multicystic, hemorrhagic, vascular tumor in TAIL of the pancreas
(tumors in the head are usually exocrine)

-Neuroendocrine tumor= salt + pepper cells; cords + ribbons

130
Q

Insulinoma:
Triad of symptoms
benign or malignant?
How do we determine malignancy?

A

WHIPPLE TRIAD:

  • Hypoglycemia
  • Confusion
  • Fasting glucose UNDER 50
  • *I think Jeff has this. He gets so stupid when he hasn’t eaten.

Usually benign (90%), only mets can determine tumor is malignant

131
Q

Define nesidioblastosis:
who gets it?
is it permanent?

A

transient congenital hyperinsulinism caused by islet hyperplasia
usually seen in maternal diabetes

132
Q 
Zollinger Ellison Syndrome: 
Cause? 
Clinical presentation (2)
Benign or malignant?
With what genetic disorder might this syndrome be associated?
A

-Gastrin secreting tumor in the pancreas or duodenum

Clinical presentation:

  • Ulcers in unusual places (jejunum instead of stomach/ duodenum)
  • DIARRHEA

-Over 50% malignant; 25% MEN1 assc

133
Q

Glucagonoma:
symptoms? (3)
Population?

A

Symptoms:

  • mild DM
  • rash (necrotic migratory erythema)
  • anemia of chronic disease

Population:
-adult females

134
Q

Describe the rash assc with glucagonoma

A

necrolytic migratory erythema

  • usually at groin
  • erythema –> blisters –> scarring/ hyperpigmentation
135
Q

VIP-omas cause what syndrome?

A

WDHA syndrome

Watery Diarrhea + Hypokalemia + Anchlorhydria

DT4-Exam 1 (17 decks)

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