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NUNA 684 Advanced Pathophysiology > Exam IV: Pheo, Spleen, Syndromes > Flashcards

Exam IV: Pheo, Spleen, Syndromes Flashcards

1
Q

what is a pheochromocytoma

A

Catecholamine secreting tumor from the chromaffin cells of the adrenal system

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2
Q

Chromaffin cells produce ______ and ______

A

epinephrine and norepinephrine

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3
Q

Pheo can be _______

A

lethal

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4
Q

Pheo resection in the OR can ______ _______

A

cure HTN

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5
Q

Pheos found equally in _____ & ______

A

males and females

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6
Q

usually in adults ___-___ years but ___% in children

A

30-50 years
10%

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7
Q

Familial pheo can be part of ______ _____ ______ (_____) Syndrome

A

Multiple Endocrine Neoplastic (MEN)

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8
Q

Almost _____% of MEN II pts have or will have a pheo

A

100

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9
Q

pheo - ____% in the adrenal medulla

A

80%

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10
Q

pheo can be _____ or _____

A

malignant or benign

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11
Q

pheo can secrete _____ and/or _____

A

NE and/or epi

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12
Q

Most common pheos secrete norepi:epi at a ____:____ ratio. Normal (non-pheo) numbers are _______.

A

85:15
reversed

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13
Q

most pheos secrete without ______ control

A

neurogenic

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14
Q

pheo symptoms occur _____ to ______ times a day - a few minutes to several hours

A

infrequently to several

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15
Q

pheo hallmark symptom

A

HTN (test question)

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16
Q

other pheo symptoms:

A
  • HA
  • sweating
  • pallor
  • palpitations
  • orthostatic HoTN
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17
Q

pheo norepi effects: increased ______ and ______ with reflex _______ (alpha agonist)

A

SBP and DBP
bradycardia

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18
Q

pheo epi effects: increased _____ and decreased ______, tachycardia (____ agonist)

A

SBP
DBP
beta

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19
Q

pheo - increased _____, normal CO, slightly decreased ____ _____

A

SVR
plasma volume

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20
Q

pheo - cardiomyopathy usually _____

A

LV

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21
Q

pheo EKG changes - _____ changes, flat/inverted T waves, prolonged _____, peaked _____ waves, L axis deviation, dysrhythmias

A

ST
QT
P

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22
Q

pheo - increased _____ d/t _____ insulin release

A

BG
inhibited

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23
Q

Pheo - Diagnosis

24 hr _____ ______ and catecholamines

A

urine metanephrines

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24
Q

Pheo - Diagnosis

most sensitive test

A

plasma free metanephrines

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25
Q

Pheo - Diagnosis

Normetanephrine (norepinephrine metabolite) >_____ pg/mL and/or

A

400

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26
Q

Pheo - Diagnosis

Metanephrine (epinephrine metabolite) >_____ pg/mL

A

220

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27
Q

Pheo - Diagnosis

If questionable results: ______ ______ test or _____ ______ test (if DBP <100)

A

Clonidine suppression
glucagon stimulation

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28
Q

Pheo - Diagnosis

location found by

A
  • CT
  • MRI
  • PET
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29
Q

Pheo - Diagnosis

functionality by ______ (concentrates in catecholamine-secreting tumors)

A

MIBG

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30
Q

Pheo - Diagnosis

catecholamine samples from _____ _____ (cath lab procedure)

A

adrenal vein

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31
Q

Pheo - Diagnosis

differentials - must differentiate from 3 other pathologies

A
  • MH
  • thyroid storm
  • carcinoid crisis
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32
Q

Pheo - Diagnosis

  • Catecholamines produced by pheos are metabolized within ______ cells. So plasma free metanephrine levels are ____ _____ for diagnosing a pheo than plasma epi and norepi levels.
A

chromaffin
more accurate

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33
Q

Reminder: Most pheos secrete ↑ _____ ( ___ _____ ).

A

norepi (α-agonist)

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34
Q

____-blockade to ↓ BP, ↑ volume, prevent HTN episodes, re-sensitize receptors, ↓ myocardial dysfunction

A

α

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35
Q

Most common: ______ (____) – non-competitive, non-selective (α1 and α2 ) irreversible α-blocker

A

Phenoxybenzamine (Dibenzyline®

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36
Q

overtreatment of a pheo can lead to _____ ______

A

orthostatic hypotension

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37
Q

D/C alpha blockade ____-____ hours pre-op to avoid refractory hypotension

A

24-48

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38
Q

Prazosin (Minipress®) & doxazosin (Cardura®) - ____ ____ blocker, shorter acting, less tachycardia, easier to titrate

A

pure α1

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39
Q

Treat phenoxybenzamine-induced tachycardia with:

A

non-selective β-blockade (usually propranolol; also atenolol, metoprolol, labetalol).

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40
Q

EXTREME CAUTION with ___-blockade before ___-blockade

A

β
α
(β2 blockade leads to unopposed α effects which causes vasoconstriction and HTN crisis)

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41
Q

Other adjuncts: Metyrosine (tyrosine inhibitor causes blocked catecholamine synthesis), ____ channel blockers, ____ inhibitors

A

Ca++
ACE

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42
Q

primary intra-op goals of pheo:

A

Avoid catecholamine release (drugs, stimulation*)
Maintain CV stability with short-acting drugs

*Think about laryngoscopy, incision, light anesthesia, emergence…

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43
Q

Ligation of tumor-related vessels leads to _____ ______

A

significant HoTN

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44
Q

Standard monitoring + ____ ____ (consider CVP, PA cath, TEE)

A

arterial line

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45
Q

pheo - intra op you must avoid ______

A

hypovolemia

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46
Q

avoid drugs with known ____ effects:

A

CV
MSO4, atracurium, atropine, sux, pancuronium, ephedrine, ketamine

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47
Q

______ ______ most common approach but insufflation and tumor manipulation causes HTN.

A

Laparoscopic adrenalectomy

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48
Q

_____ ______ required for large tumors

A

open excision

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49
Q

Common with pheo: ____ ____ intra-op regardless of pre-op α-blockade

A

SBP >200

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50
Q

Have _____, ____-____ drugs prepared and ready for pheo intra-op

A

potent, rapid-onset

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51
Q

_____ is drug of choice; phentolamine, NTG, labetolol, MgSO4, esmolol, diltiazem.

A

Nipride

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52
Q

↑ anesthetic depth leads to ↑ risk of hypotension with ____ _____

A

venous ligation

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53
Q

Lidocaine and/or β-blockers (propranolol, esmolol) for _____ _____

A

ventricular dysrhythmias

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54
Q

Common: SBP ____ after venous ligation
Pre-treat with crystalloids; pressors and inotropes if needed.

A

< 80

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55
Q

avoid ____ ____ blood because it’s high in ______

A

cell saver
catecholamines

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56
Q

after pheo resection, increased _____ and decreased _____. Begin _____ solution after resection

A

insulin
glucose
dextrose

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57
Q

With complete resection, most pts eventually become _____tensive.

A

normo

58
Q

Plasma catecholamines levels do not normalize for __-__ days.

A

7-10

59
Q

Approx 50% will be hypertensive for ____ _____; approx. 25% remain hypertensive for ____.

A

several days
life

60
Q

Most frequent immediate post-op cause of death:

A

Hypotension d/t sudden ↓ catecholamines with refractory vasodilation. (treat with fluids and pressors if necessary)

61
Q

steroids for ______

A

hypoadrenalism

62
Q

Functions of the Spleen

A

Blood filtering, erythrocyte morphology, immunity related to blood-borne antigens

63
Q

Hematopoiesis until the ___ _____ month

A

5th gestational

64
Q

Macrophages engulf abnormal (SC dz, thalassemia, spherocytosis) and older (>120 days) blood cells which can lead to ____, _____, ______ ______.

A

anemia, splenomegaly, splenic infarction

65
Q

Splenic macrophages, histiocytes and IgM play important roles in ______.

A

immunity

66
Q

spleen - Minor role as red blood cell and platelet _____

A

reservoir

67
Q

______ ______ done for hypersplenism, ITP, TTP, hereditary spherocytosis, hereditary hemolytic anemia, hemoglobinopathies, malignancies, abscesses, cysts.

A

Elective splenectomies

68
Q

Emergent splenectomies done for splenic _____.

A

trauma

69
Q

The spleen receives ___% of cardiac output.

A

5

70
Q

splenic bleeding is usually _____

A

arterial

71
Q

Blunt abdominal trauma + ____ ____ _____ pain = high risk of splenic trauma (commonly from fractured ____)

A

left upper quadrant
rib

72
Q

splenectomy - Usually done ______ but with hemodynamic instability, ______ may be approach of choice.

A

laparoscopically
laparotomy

73
Q

for splenectomy, have _____ available

A

blood

74
Q

Asplenic pts have increased risk for _____ processes (bacteremia, pneumonia, meningitis) and _____ for life.

A

infectious
thromboembolism

75
Q

Most carcinoid tumors occur in the

A

bronchus, jejunum/ileum or colon/rectum.

76
Q

carcinoid tumors secrete ___ ____ and ____ substances that may or may not cause symptoms.

A

GI peptides
vasoactive

77
Q

large amounts cause

A

carcinoid syndrome

78
Q

carcinoid syndrome symptoms

A
  • flushing
  • diarrhea
    (can also have pruritus, tearing, and facial edema)
79
Q

carcinoid syndrome precipitated by:

A

stress, ETOH, exercise, diet, drugs (catecholamines, SSRIs)

80
Q

Serotonin antagonists used for ______; H1/H2 blockers for ______ and _______.

A

diarrhea
flushing and pruritus

81
Q

carcinoid syndrome can cause life threatening ______ ______

A

carcinoid crisis

82
Q

CC symptoms:

A

flushing, diarrhea, abd pain, and CV instability

83
Q

CC can be precipitated by:

A

sux, benzylisoquinolines (except cisatracurium), catecholamines, dopamine, isuprel, thiopental

84
Q

Preventing/Treating Carcinoid Syndrome

Avoid _____ conditions.
may need ___/_____

A

precipitating
diet/supplements

85
Q

Preventing/Treating Carcinoid Syndrome

_____ antagonists, ______ analogues, ___/___ blockers
S-statin analogues (octreotide, lanreotide) for 24-48 hrs pre-op and continued throughout procedure.

A

5-HT3
somatostatin
H1/H2

86
Q

Preventing/Treating Carcinoid Syndrome

Only cure: _____ _____ of the carcinoid tumor

A

Surgical excision

87
Q

Preventing/Treating Carcinoid Syndrome

GA with art line, epidural with monitoring if treated pre-op with ______.

A

octreotide

88
Q

Preventing/Treating Carcinoid Syndrome

May have delayed awakening d/t:

A

↑ serotonin levels.

89
Q

Fibromyalgia

Widespread MSK pain with _____, _____, ______ and mood issues

A

fatigue, sleep, memory

90
Q

Fibromyalgia

Current theory: Repeated _____ _____ causes increased neurotransmitters which leads to a hyperactive response to _____

A

painful stimuli
pain

91
Q

Fibromyalgia frequently associated with:

A

H/A, TMJ disorders, irritable bowel syndrome, anxiety, depression

92
Q

Fibromyalgia

_____ more than _____, no ______. Treat with exercise, stress reduction, alternative medicine (acupuncture, massage therapy, yoga), meds (NSAIDS, anti-depressants, gabapentinoids).

A

females
males
cure

93
Q

Fibromyalgia

______ (____): 1st FDA approved drug for fibromyalgia

A

Pregabalin (Lyrica®)

94
Q

Sarcoidosis

Autoimmune, interstitial, granulomatous, usually in ____, ____, ____, ____, ______

A

lungs, lymphatics, bone, liver, CNS

95
Q

Sarcoidosis

____ _____ lung dz causing dyspnea, cough, pulmonary HTN and cor pulmonale.

A

Chronic restrictive

96
Q

Sarcoidosis

Usually diagnosed by ______; many asymptomatic at time of diagnosis.

A

radiography

97
Q

Sarcoidosis

Common manifestations: _______ (uncontrolled synthesis of a vitamin D3 enzyme by macrophages) and ____ ____ _____ (neurosarcoidosis, parotid gland)

A

Hypercalcemia
facial nerve paralysis

98
Q

Sarcoidosis

biopsy done with _______

A

mediastinoscopy

99
Q

Sarcoidosis

Treatment: ______ for symptoms and hypercalcemia

A

Corticosteroids

100
Q

Sarcoidosis AIs: Pts have poor tolerance to apnea d/t ____ _____. Keep PIPs as low as possible to minimize ______ risks.

A

small FRC
barotrauma

101
Q

Systemic Lupus Erythematosus

Multisystem, chronic inflammatory, caused by _____ _____

A

antibody production

102
Q

Systemic Lupus Erythematosus

Usually _____ _____, exacerbated by ______ (surgery, pregnancy, infection)

A

young females
stressors

103
Q

Systemic Lupus Erythematosus

Can be drug induced (usually… 5)

A

procainamide, hydralazine, isoniazid, penicillamine, methyldopa

104
Q

Systemic Lupus Erythematosus

symptoms

A

Arthralgias, maculopapular (butterfly) rash, fever, anemia, leukopenia

105
Q

Systemic Lupus Erythematosus

_____ and/or hypertension worsens prognosis.

A

Nephritis

106
Q

Systemic Lupus Erythematosus

Systemic involvement: CNS, _____, _____, ______, liver, hematologic, MSK and skin

A

heart, lungs, kidneys

107
Q

Systemic Lupus Erythematosus

Treatment based on systems affected and symptoms with:

A

ASA, NSAIDs, chemo, steroids

108
Q

Systemic Lupus Erythematosus

AIs: Consider organ dysfunction, _____ neck, ____ _____ nerve palsy, drugs

A

arthritic
recurrent laryngeal

109
Q

Scleroderma (Systemic Sclerosis)

_____/______ dz causes inflammation, vascular sclerosis, fibrosis of skin and organs

A

Collagen/autoimmune

110
Q

Scleroderma (Systemic Sclerosis)

Vascular injury leads to leaking proteins causing _____ and ______ in skin, MSK, CNS, CV, lungs, kidneys, GI tract

A

edema and fibrosis

111
Q

Scleroderma (Systemic Sclerosis)

Can become _____ _____ (Calcinoses, Raynaud’s phenomenon, Esophageal hypomotility, Sclerodactyly, Telangiectasis) with poor prognosis.

A

CREST syndrome

112
Q

Scleroderma (Systemic Sclerosis)

CV: Conduction, contractility, ____ ____ HTN, cor pulmonale (Consider eval pre-op.)

A

pulm artery

113
Q

Scleroderma (Systemic Sclerosis)

Lungs: May need ↑ _____, acidosis/hypoxia leading to increased pulm VR, sensitive to _____

A

PIPs
opioids

114
Q

Scleroderma (Systemic Sclerosis)

Renal: HTN, irreversible renal failure (Tx: ____ _____)

A

ACE inhibitors

115
Q

Scleroderma (Systemic Sclerosis)

GI: ↓ motility, dysphagia, GERD, Vit K malabsorption causing coag problems (Tx: ______. ______ ineffective.)

A

Octreotide
Prokinetics

116
Q

Scleroderma (Systemic Sclerosis)

_______: Oral/nasal bleeding with intubation

A

Telangiectasis

117
Q

Scleroderma (Systemic Sclerosis)

Skin: Difficult ____ _____, peripheral vasoconstriction causes ______ in OR

A

IV access
hypothermia

118
Q

Scleroderma (Systemic Sclerosis)

_______ may be difficult but can be advantageous (peripheral vasodilation, opioid sparing).

A

Regional

119
Q

What is Porphyria?

A

A group of inborn errors of metabolism resulting from a specific enzyme deficiency in the heme synthetic pathway causing overproduction of porphyrins

120
Q

Heme is a ______

A

porphyrin

121
Q

Binds with proteins to make _______ and ______ ______ isoenzymes

A

hemoglobin and cytochrome P-450

122
Q

Heme production is regulated by _____ _____ (___) ______

A

aminolevulinic acid (ALA) synthetase

123
Q

ALA is easily ______ by drugs using P-450 system

A

induced

124
Q

With porphyria, ↑ _____ requirements causes _____ stimulation which leads to accumulated ______ (compounds preceding the site of the deficiency)

A

heme
ALA
intermediates

125
Q

porphyria classified as ______ or ______; acute and non-acute

A

hepatic or erythropoietic

126
Q

only forms of porphyria relevant for anesthesia

A

acute

127
Q

certain drugs cause _____-_____ _____

A

life-threatening reaction

128
Q

acute porphyrias are inherited, _____ dominant

A

autosomal

129
Q

acute porphyrias more frequent in women in there ____s or ____s, and is rare after ______

A

30s or 40s
menopause

130
Q

acute porphyrias are also triggered by ____

A

stress (childbirth, fasting, dehydration, illness, infection)

131
Q

acute porphyrias (4)

A
  • plumboporphyria
  • acute intermittent porphyria
  • hereditary coproporphyria
  • variegate porphyria
132
Q

“Silent” or “latent” porphyria pts may show ____ _______after perioperative triggering drugs.

A

1st symptoms

133
Q

porphyria - ______ “inducers” more high risk than _____ exposure

A

Multiple
single

134
Q

porphyria avoid list

A

Barbiturates (thiopental, methohexital), sevoflurane (?), etomidate (?), pentazocine (Talwin), ephedrine (?), ketamine (?), traditional seizure meds

135
Q

porphyria - acute exacerbation symptoms

A

Skeletal muscle weakness, HTN, tachycardia, abdominal pain, seizures, electrolyte imbalance (especially hyponatremia)

136
Q

porphyria - NPO status can trigger. Consider _____ _____

A

glucose IVF

137
Q

porphyria - Rule out acute crisis with _____ _______ (____) level.

A

urine porphobilinogen (PBG)

138
Q

Short acting drugs tolerated well; avoid _____ _____. (Total dose and length of exposure ↑ risk of triggering.)

A

propofol infusion

139
Q

porphyria - Regional OK after careful _____ assessment.

A

neuro

140
Q

drugs that are OK for porphyria

A

N2O, isoflurane, desflurane, opioids, benzos, MRs OK.

141
Q

treating acute porphyria crisis:

A
  • Stop any possible triggers
  • Fluid/glucose loading, monitor electrolytes
  • Consider analgesics, anti-emetics, β-blockers, benzodiazapines or propofol for seizures
  • Heme (arginate) infusion, somatostatin (Octreotide®) to ↓ ALA synthetase, plasmapheresis

NUNA 684 Advanced Pathophysiology (16 decks)

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