Musculoskeletal diseases 2 Flashcards

1
Q

Which agents are safe to administer to the patient with hypokalemic periodic paralysis?
a. rocuronium
b. terbutaline
c. D5LR
d. acetazolamide

A

a. rocuronium
d. acetazolamide

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2
Q

Familial periodic paralysis is characterized by

A

acute episodes of skeletal muscle weakness accompanied by serum potassium concentration changes
two variants of the disease- hypokalemic & hyperkalemic

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3
Q

Hypokalemic periodic paralysis is presnt if

A

skeletal muscle weakness follows a glucose-insulin infusion

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4
Q

With hypokalemic periodic paralysis, the patient becomes weak when the serum K+

A

decreases

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5
Q

Hyperkalemic periodic paralysis is present if skeletal muscle weakness follows

A

oral potassium administration

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6
Q

The patient with hyperkalemic periodic paralysis becomes weak when

A

serum K+ increases

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7
Q

_______ is the treatment for both forms of familial periodic paralysis

A

Acetazolamide

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8
Q

For patients with familial periodic paralysis, _______ should be avoided at all costs.

A

Hypothermia

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9
Q

For patients with hyperkalemic familial periodic paralysis, __________ is contraindicated.

A

succinylcholine

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10
Q

For patients with hypokalemic familial periodic paralysis ________________ should be avoided because _______

A

succinylcholine because this condition might be associated with MH

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11
Q

_______ should be monitored for patients with familial periodic paralysis.

A

Serum potassium levels

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12
Q

Familial periodic paralysis is a disorder of ____________ and not a disease of ____________

A

the skeletal muscle membrane (reduced excitability) and not a disease of the neuromuscular junction

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13
Q

Hypokalemic periodic paralysis is associated with a _______ channelopathy

A

calcium

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14
Q

Hyperkalemic periodic paralysis is associated with a _________ channelopathy

A

sodium

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15
Q

Why is acetazolamide the treatment of choice for both forms of familial periodic paralysis?

A

it creates a non-anion acidosis which protects against hypokalemia and it also facilitates renal potassium excretion which guards against hyperkalemia

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16
Q

For patients with hypokalemic periodic paralysis, we should avoid these drugs:

A

glucose containing solutions
potassium wasting diuretics
beta-2 agonists
succinylcholine

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17
Q

For patients with hyperkalemic periodic paralysis, we should avoid these drugs:

A

succinylcholine
potassium containing solutions (LR)

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18
Q

Malignant hyperthermia is primarily associated with the genetic mutation of the:
a. SERCA2 pump
b. ryanodine receptor
c. dihydropyridine receptor
d. actin myofilament

A

b. ryanodine receptor

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19
Q

List three co-existing diseases that are definitely associated with MH:

A

King-Denborough syndrome
Central core disease
multiminicore disease

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20
Q

What two classes of drugs are known to trigger MH?

A

halogenated anesthetics
depolarizing neuromuscular blockers

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21
Q

MH is an

A

inherited disease of skeletal muscle that is characterized by disorder calcium homeostasis

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22
Q

Describe the pathophysiology of MH.

A

a defective ryanodine receptor contributes to excessive Ca2+ release from the endoplasmic reticulum and this leads to sustained skeletal muscle contraction, a hypermetabolic state, and increased oxygen consumption

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23
Q

MH is not associated with the following conditions:

A

Duchenne muscular dystrophy
Becker muscular dystrophy
neuroleptic malignant syndrome
myotonia congenita
myotonic dystrophy
osteogenesis imperfecta

24
Q

Consequences of increased intracellular calcium in the myocyte include

A

rigidity from sustained contraction
accelerated metabolic rate and rapid depletion of ATP
increased oxygen consumption
increased CO2 and heat production
mixed respiratory and lactic acidosis
sarcolemma breaks down
potassium and myoglobin leak into the systemic circulation

25
Q

Factors that increase the risk of MH include

A

Male sex
youth
geography- families from Wisconsin, Nebraska, West Virginia, and Michigan appear to be at higher risk

26
Q

The earliest signs of malignant hyperthermia include (choose 3):
a. tachycardia
b. hyperthermia
c. cola-colored urine
d. increased EtCo2
e. masseter spasm
f. DIC

A

a. tachycardia
d. increased EtCo2
e. masseter spasm

27
Q

The most sensitive indicator of MH is

A

EtCO2 that rises out of proportion to minute ventilation

28
Q

MH can occur as late as ______ hours after exposure to a triggering agent

A

6 hours

29
Q

____________ is typically a late sign of MH.

A

hyperthermia

30
Q

Conditions to consider in your differential diagnosis of MH includes

A

thyroid storm
pheochromocytoma
sepsis
heatstroke

31
Q

_____ & _______ are two entities that exist on a continuum with MH.

A

Trismus and masseter muscle rigidity

32
Q

_____ describes a tight jaw that can still be opened and is a normal response to __________

A

trismus; succinylcholine

33
Q

_____________ muscle rigidity describes a tight jaw that cannot be opened. ____________ will not relax the jaw.

A

Masseter muscle rigidity; a neuromuscular blocker

34
Q

Anyone who has experienced MH or masseter spasm should be referred for

A

a halothane contracture test for diagnosis

35
Q

Early signs of MH include

A

tachycardia
tachypnea
masseter spasm
warm soda lime
irregular heart rhythm

36
Q

Intermediate signs of MH include

A

cyanosis
irregular heart rhythm
patient warm to touch

37
Q

Late signs of MH include

A

muscle rigidity
cola-colored urine
coagulopathy
irregular heart rhythm
overt hyperthermia

38
Q

What drug is contraindicated in the management of malignant hyperthermia?
a. verapamil
b. mannitol
c. calcium chloride
d. insulin

A

a. verapamil
CCB can result in life-threatening hyperkalemia

39
Q

Considerations for preventing MH include:

A

flushing the anesthesia machine with high flow oxygen for up to 100 minutes
the vaporizers must be physically removed from the machine
external parts should be removed and replaced (CO2 absorbent, circuit, and breathing bag)
dantrolene prophylaxis is unwarranted

40
Q

The four steps to treat MH include.

A

Discontinue the triggering agents. Convert to an IV technique.
call for help and notify the surgeon to terminate the procedure
hyperventilate with 100% O2 at a minimum FGF of 10L/min
apply charcoal filters to the inspiratory and expiratory port and then apply a new breathing circuit and bag

41
Q

The last 4 steps of treating MH include

A

administer dantrolene or Ryanodex 2.5 mg/kg IV q5-10 mint
cool the patient
treat acidosis and electrolyte disturbances
maintain UO above 2mL/kg/hr

42
Q

If the patient with MH doesn’t present with s/sx of MH within the first _________ of the procedure, then it is very unlikely that it will occur later.

A

hour

43
Q

As an alternative to the purging guidelines (10L FGF at 100% O2 for min 20-100 min.), you can use

A

a charcoal filter such as the Vapor-clean
flush with high FGF for 90 seconds prior to using the machine on the patient

44
Q

After the patient is stabilized, they should be

A

observed in the ICU as MH can reoccur up to 36 hours

45
Q

What are the MOAs of dantrolene?

A

it reduces Ca2+ release from the RyR1 receptor in the skeletal myocyte
it prevents Ca2+ entry into the myocyte which reduces the stimulus for calcium-induced calcium release

46
Q

What should dantrolene be reconstituted with?

A

60 mL of preservative free water
NS introduces additional solute and prolongs the time required for dantrolene to dissolve

47
Q

Most common side effects of dantrolene are

A

weakness
venous irritation

48
Q

What is the continuous infusion rate of dantrolene?

A

1 mg/kg q6 hr. or 0.1-0.3 mg/kg/hr for 48-72 hours

49
Q

If the patient requires more than ___________, reconsider the diagnosis of MH

A

20 mg/kg

50
Q

Patients with MH should be cooled until

A

their temperature drops below 38 degrees C

51
Q

Ways to cool the patient include

A

Cold IVF
cold fluid lavage of stomach and bladder
ice packs

52
Q

What can be given to correct lactic acidosis?

A

sodium bicarbonate 1-2 mEq/kg IV titrated to ABG and base deficit

53
Q

What can be done to treat hyperkalemia?

A

CaCl 5-10 mg/kg IV
insulin 0.15 units/kg + D50 1 mL/kg
Hyperventilation

54
Q

What can be given to protect against dysrhythmias for the patient with MH?

A

procainamide 15 mg/kg IV
lidocaine 2 mg/kg IV
co-administration of a CCB with dantrolene can precipitate life-threatening hyperkalemia

55
Q

For patients with MH, urine output can be maintained with

A

IV hydration
mannitol 0.25 g/kg IV
furosemide 1 mg/kg IV