Musculoskeletal diseases 2 Flashcards
Which agents are safe to administer to the patient with hypokalemic periodic paralysis?
a. rocuronium
b. terbutaline
c. D5LR
d. acetazolamide
a. rocuronium
d. acetazolamide
Familial periodic paralysis is characterized by
acute episodes of skeletal muscle weakness accompanied by serum potassium concentration changes
two variants of the disease- hypokalemic & hyperkalemic
Hypokalemic periodic paralysis is presnt if
skeletal muscle weakness follows a glucose-insulin infusion
With hypokalemic periodic paralysis, the patient becomes weak when the serum K+
decreases
Hyperkalemic periodic paralysis is present if skeletal muscle weakness follows
oral potassium administration
The patient with hyperkalemic periodic paralysis becomes weak when
serum K+ increases
_______ is the treatment for both forms of familial periodic paralysis
Acetazolamide
For patients with familial periodic paralysis, _______ should be avoided at all costs.
Hypothermia
For patients with hyperkalemic familial periodic paralysis, __________ is contraindicated.
succinylcholine
For patients with hypokalemic familial periodic paralysis ________________ should be avoided because _______
succinylcholine because this condition might be associated with MH
_______ should be monitored for patients with familial periodic paralysis.
Serum potassium levels
Familial periodic paralysis is a disorder of ____________ and not a disease of ____________
the skeletal muscle membrane (reduced excitability) and not a disease of the neuromuscular junction
Hypokalemic periodic paralysis is associated with a _______ channelopathy
calcium
Hyperkalemic periodic paralysis is associated with a _________ channelopathy
sodium
Why is acetazolamide the treatment of choice for both forms of familial periodic paralysis?
it creates a non-anion acidosis which protects against hypokalemia and it also facilitates renal potassium excretion which guards against hyperkalemia
For patients with hypokalemic periodic paralysis, we should avoid these drugs:
glucose containing solutions
potassium wasting diuretics
beta-2 agonists
succinylcholine
For patients with hyperkalemic periodic paralysis, we should avoid these drugs:
succinylcholine
potassium containing solutions (LR)
Malignant hyperthermia is primarily associated with the genetic mutation of the:
a. SERCA2 pump
b. ryanodine receptor
c. dihydropyridine receptor
d. actin myofilament
b. ryanodine receptor
List three co-existing diseases that are definitely associated with MH:
King-Denborough syndrome
Central core disease
multiminicore disease
What two classes of drugs are known to trigger MH?
halogenated anesthetics
depolarizing neuromuscular blockers
MH is an
inherited disease of skeletal muscle that is characterized by disorder calcium homeostasis
Describe the pathophysiology of MH.
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