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Musculoskeletal diseases Study online at https://quizlet.com/_ea86it 1. What is IgG antibodies destroy post-junctional nicotinic, ACh re- the pathophysi- ceptors at the NMJ ’ skeletal muscle weakness ology of Myas- thenia Gravis? 2. When is weak- Muscle we...

Musculoskeletal diseases Study online at https://quizlet.com/_ea86it 1. What is IgG antibodies destroy post-junctional nicotinic, ACh re- the pathophysi- ceptors at the NMJ ’ skeletal muscle weakness ology of Myas- thenia Gravis? 2. When is weak- Muscle weakness becomes worse later in the day or that ness worst in MG develops with exercise. patients? Periods of rest allow for the recovery of skeletal muscle function. 3. What surgery Thymectomy brings symptom relief to many patients. may relieve symptoms in MG? 4. What are S/S of - Diplopia, ptosis (earliest signs) MG? - Bulbar muscle weakness (muscles of the mouth and throat) - dysphagia, dysarthria, and difficulty handling saliva - Dyspnea with exertion - Proximal muscle weakness 5. What situation - Pregnancy exacerbate the - Infection symptoms of - Electrolyte abnormalities MG? - Surgical and psychological stress - Aminoglycoside antibiotics 6. What are con- Pregnancy intensifies the symptoms of myasthenia siderations for gravis neonates of mothers with Anti-AchRIgG antibodies cross the placenta and cause MG? weakness in neonates. 7. How is MG diag- Edrophonium 1 - 2 mg IV (the Tensilon test). nosed? How is it - If muscle weakness worsens, the patient has a cholin- interpreted? ergic crisis. 1 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it - If muscle strength improves, the patient has a myas- thenic crisis. 8. How is MG treat- Anticholinesterases ed? - Oral pyridostigmine (an anticholinesterase medication) is the first-line medical treatment for myasthenia gravis. Immunosuppression - Corticosteroids, cyclosporine, azathioprine, mycophe- nolate Surgery - Thymectomy reduces circulating Anti-AchR IgG in most patients. Plasmapheresis - Provides temporary relief during myasthenic crisis or before thymectomy. 9. How does MG Nondepolarizers: ‘ Sensitivity affect dosing NMBs? Succinylcholine: ‘ Resistance 10. How does Pyri- It impairs the efficacy of pseudocholinesterase. This pro- dostigmine af- longs the duration of succinylcholine. fect the effective- ness of succinyl- choline? 11. How to avoid Volatile anesthetics cause skeletal muscle relaxation by NMBs in patients acting in the ventral horn of the spinal cord. with MG? In many cases, this eliminates the need for neuromuscu- lar blockers. 12. What are Risk of residual neuromuscular blockade: if a neuromus- post-operative cular blocker was used concerns in patients with Risk of pulmonary aspiration: bulbar muscle weakness MG? (mouth and throat) 2 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it 13. What increases - Disease duration > 6 years the risk of the - Daily pyridostigmine > 750 mg/day need for post- - Vital capacity < 2.9 L operative venti- - COPD lation in patients - Surgical approach: median sternotomy > transcervical with MG? thymectomy 14. What is the IgG-mediated destruction of the presynaptic voltage-gat- pathophysiolo- ed calcium channel at the presynaptic nerve terminal. gy of Eaton-Lambert Ca+2 entry into the presynaptic neuron is limited ’ Syndrome? thereby reducing the amount of Ach that is released into the synaptic cleft. The postsynaptic nicotinic receptor is present in normal quantity and functions normally. 15. What is the clin- - Proximal muscles are most affected ical presentation - Weakness is generally worse in the morning and gets of Eaton-Lam- better throughout the day. bert Syndrome? - The respiratory musculature and diaphragm become weak. - Autonomic nervous system dysfunction causes ortho- static hypotension, slowed gastric motility, and urinary retention. 16. What is 3, 4-diaminopyridine (DAP) increases Ach release from the treatment the presynaptic nerve terminal and improves the strength of Eaton-Lam- of contraction. bert Syndrome? Anticholinesterases are not helpful, and the Tension test does not aid in diagnosis. 17. What are Patients are sensitive to succinylcholine AND nondepo- NMBs dosed larizers 3 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it in Eaton-Lam- Volatile anesthetics provide enough muscle relaxation for bert Syndrome? most surgical procedures. 18. How do patients Reversal with anticholinesterases may be inadequate de- with spite proper dosing. Eaton-Lambert Syndrome respond to reversal with anti- cholinesteras- es? 19. What disease Small-cell carcinoma of the lung (oat-cell carcinoma). is associated with Eaton-Lam- Consider the possibility of this disorder in all patients bert Syndrome? with suspected lung cancer undergoing mediastinoscopy, bronchoscopy, or thoracoscopy. 20. Compare MG with Eaton-Lam- bert Syndrome based on: - Region Affected - Defect - Region of NMJ Affected - Common Co-Morbidities - Response to Succinylcholine - Response to Nondepolarizers - Effectiveness of AchE Inhibitors 21. What is the Immunologic assault on myelin in the peripheral nerves. pathophysiolo- gy of The action potential can't be conducted, so the motor Guillain-Barre endplate never receives the incoming signal. 4 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it Syndrome (acute idiopathic polyneuritis)? 22. What precedes A flu-like illness usually precedes paralysis by 1 - 3 weeks. paralysis in - Campylobacter jejuni bacteria GBS? What are - Epstein-Barr virus common etiolo- - CMV gies? What is a - Vaccinations, surgery, and lymphomatous disease long-term conse- quence? 23. What are S/S of Flaccid paralysis GBS? - Begins in the distal extremities and ascends bilaterally towards the proximal extremities, trunk, and face. - Intercostal muscle weakness impairs ventilation. - Facial and pharyngeal weakness causes difficulty swal- lowing. Sensory deficits include paresthesias, numbness, and pain. Autonomic dysfunction: tachycardia or bradycardia, hy- pertension or hypotension, diaphoresis or anhidrosis, and orthostatic hypotension. 24. What is the treat- Plasmapheresis and IV |gG. ment of GBS? What medica- In contrast to multiple sclerosis, steroids and interferon do tions are not use- not improve this condition. ful? 25. How are NMBs - Avoid succinylcholine. There's a risk of hyperkalemia does in GBS? from the proliferation of extrajunctional Ach receptors. - Increased sensitivity to nondepolarizers. 26. What are the Skeletal muscle weakness main anesthet- - Repiratory failure ic considera- - Risk of aspiration tions for GBS? - May require postoperative mechanical ventilation. 5 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it Autonomic dysfunction - Risk for hemodynamic instability from anesthesia, po- sition changes, positive pressure ventilation, and blood loss. - Exaggerated response to indirect-acting sympath- omimetics - Steroids are not useful. 27. What is Hypokalemic periodic paralysis is associated with a cal- the pathophysi- cium channelopathy. ology of Familial Periodic Paraly- Hyperkalemic periodic paralysis is associated with a sodi- sis? um channelopathy. It's a disorder of the skeletal muscle membrane (not a disease of the neuromuscular junction). 28. How is Familial Hypokalemic periodic paralysis Periodic Paraly- - Muscle weakness follows a glucose-insulin infusion. sis diagnosed? Hyperkalemic periodic paralysis - Muscle weakness follows oral potassium administration. 29. What is the treat- Acetazolamide is the treatment for both forms of this ment of Familial disease. Periodic Paraly- - It creates a non-anion gap acidosis, which protects sis? against hypokalemia - Facilitates renal potassium excretion, which guards against hyperkalemia. 30. What condition Hypothermia must be avoided at all costs. must be avoided with both forms Indeed, it's recommended that these patients remain of Familial Peri- normothermic even when they are placed on cardiopul- odic Paralysis? monary bypass! 31. What medica- Do Not Administer tions are safe/un- - Glucose-containing solutions safe to admin- - Potassium wasting diuretics 6 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it ister to Hy- - Beta-2 agonists pokalemic Fa- - Succinylcholine milial Periodic Paralysis? Safe to Administer - Nondepolarizers - Acetazolamide 32. What medica- Do Not Administer tions are safe/un- - Succinylcholine safe to admin- - Potassium-containing solutions (LR) ister to Hyper- kalemic Familial Safe to Administer Periodic Paraly- - Glucose-containing solutions sis? - Potassium wasting diuretics - Beta-2 agonists - Nondepolarizers - Acetazolamide 33. How does Fa- Hypokalemic Periodic Paralysis + Succinylcholine milial Periodic - There's a possible association between MH and hy- Paralysis affect pokalemic periodic paralysis. the use of suc- - Interestingly, muscle rigidity may occur but metabolism cinylcholine? may not increase as it does with MH. Hyperkalemic Periodic Paralysis + Succinylcholine - Patients are at significant risk of succinylcholine-induced hyperkalemia. The safest approach is to avoid succinylcholine in BOTH. 34. How does Fa- Nondepolarizing NMBs are safe in both hypo- and hyper- milial Periodic kalemic periodic paralysis, although patients may be Paralysis affect sensitive to these drugs. Shorter-acting agents are best. the use of NDN- MBs? 35. What are the trig- Halogenated anesthetic agents and depolarizing NMBs gers of malig- (succinylcholine) nant hyperther- mia? 7 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it 36. Describe the Defective ryanodine receptor (RYR1) instructs the sar- pathophysiolo- coplasmic reticulum to release way too much calcium into gy of MH? the cell. - The cell attempts to return excess Ca+2 to the SR via the SERCA2 pump. - This consumes a substantial amount of ATP, increase oxygen consumption, and increase CO2 production. - Breakdown of the sarcolemma allows potassium and myoglobin (toxic to the kidneys) to enter the system circulation. 37. What are the con- - Rigidity from sustained contraction sequences of in- - Accelerated metabolic rate and rapid depletion of ATP creased intracel- - Increased oxygen consumption lular calcium in - Increased CO2 and heat production the myocyte? - Mixed respiratory and lactic acidosis - Sarcolemma breaks down - Potassium and myoglobin leak into the systemic circu- lation 38. What three 1. King-Denborough syndrome co-existing dis- 2. Central core disease eases are defini- 3. Multiminicore disease tively associated with MH? 39. What is the An MH-like syndrome is associated with Duchenne mus- association of cular dystrophy Duchenne Mus- - Due to rhabdomyolysis - not true MH. cular Dystrophy - Normal RyR1 receptor with the delope- - Dantrolene does not treat this condition. ment of MH? - Halogenated agents and succinylcholine can initiate this MH-like syndrome in the patient with DMD, so it's prudent to avoid these agents (use TIVA instead). 8 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it 40. How to treat Assume they have severe hyperkalemia and be immedi- ANY patient with ately treated with calcium chloride. Duchenne mus- cular dystrophy or another mus- cular dystrophy who sustains cardiac arrest on induction? 41. What is the most EtCO2 that rises out of proportion to minute ventilation. sensitive indica- tor of MH? Core temperature can (but does not always) begin to rise within 15 minutes of exposure to a triggering agent, but profound hyperthermia is typically a late sign. 42. What are the ear- Early ly, intermediate, - Tachycardia and late signs of - Tachypnea MH? - Masseter spasm - Warm soda lime - Irregular heart rhythm Intermediate - Cyanosis - Irregular heart rhythm - Patient warm to touch Late - Muscle rigidity - Cola-colored urine - Coagulopathy - Irregular heart rhythm - Overt hyperthermia 43. What are the Trismus describes a tight jaw that can still be opened. differences be- - Normal response to succinylcholine tween Trismus Masseter muscle rigidity describes a tight jaw that cannot 9 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it and Masseter be opened. spasm? - A neuromuscular blocker will not relax the jaw - Assume MH until proven otherwise. 44. What test is used The caffeine-halothane contracture test (requires a live to diagnose MH? muscle biopsy sample) is the gold standard for diagnos- ing malignant hyperthermia. 45. What conditions - Thyroid storm mimic MH? - Serotonergic syndrome - Malignant neuroleptic syndrome - Heatstroke - Sepsis - Metastatic carcinoid - Pheochromocytoma - Cocaine intoxication 46. What are gener- Dantrolene prophylaxis is unwarranted. al recommenda- tions in prepara- Anesthesia machine preparation: tion for the pa- - Must be flushed with high-flow oxygen (20 to over 100 tient at risk for minutes). MH? - CO2 absorbent, the circuit, and the breathing bag should be removed and replaced. - The vaporizers must be physically removed from the machine. 47. What is an al- Using charcoal filter (such as the Vapor-Clean). ternative to the - This filter will maintain a halogenated anesthetic concen- purging guide- tration below 5 ppm for up to 12 hours with a minimum lines? fresh gas flow of 3 L/min. - Place a Vapor-Clean filter on the inspiratory and expira- tory port of the anesthesia machine. - Flush the anesthesia machine with high fresh gas flow (greater than 10 L/min) for 90 seconds prior to using the machine on a patient. 48. What is the MOA 1. It reduces Ca+2 release from the RyR1 receptor in the of Dantrolene? skeletal myocyte. 10 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it 2. It prevents Ca+2 entry into the myocyte, which reduces the stimulus for calcium-induced calcium release. 49. How to prepare - Each vial contains 20 mg of dantrolene + 3 g of mannitol. Dantrolene? - Each vial must be reconstituted with 60 mL of preservative-free water. - Using normal saline introduces additional solute, which prolongs the time required for dantrolene to dissolve into the diluent. 50. How to prepare Each vial contains dantrolene 250 mg (enough for a load- Ryanodex? ing dose for most patients) and only requires 5 mL of sterile water diluent. 51. What are Bolus dose the guidelines - 2.5 mg/kg IV and repeat q 5 - 10 min. for administering - Stop dantrolene when symptoms of hypermetabolism Dantrolene and subside. Ryanodex? Continue in the ICU at 1mg/kg q 6 hr or 0.1-0.3 mg/kg/hr for 48 - 72 hours. - If the patient requires more than 20 mg/kg, reconsider the diagnosis of MH 52. What are side ef- Dantrolene is classified as a muscle relaxant. fects of Dantro- lene? Its most common side effects are muscle weakness and venous irritation. 53. What is the acute 1. Discontinue the triggering agent (volatile agent or suc- treatment of MH? cinylcholine). Continue anesthesia with an IV technique. 2. Call for help, and notify the surgeon to terminate the procedure. 3a. Hyperventilate with 100% O2 at a minimum FGF of 10 L/min (don't waste time changing the soda lime). Hyperventilation provides several benefits: Facilitates CO2 elimination Enhances O2 delivery 11 / 23 Musculoskeletal diseases Study online at https://quizlet.com/_ea86it Drives K+ into cells 3b. If you have a charcoal filter (such as the Vapor-Clean), you can apply a filter to the inspiratory and expiratory port of the anesthesia machine and then apply a new breathing circuit and reservoir bag. The Vapor-Clean will need to be changed every hour. 4. Administer dantrolene or Ryanodex. 5. Cool the patient until his temperature drops below 38 degrees C. Cold IVF Cold fluid lavage of stomach and bladder Ice packs 6. Correct lactic acidosis. Sodium bicarbonate 1 - 2 mEq/kg IV titrated to ABG and base deficit. 7. Treat hyperkalemia. CaCl2 5 - 10 mg/kg IV Insulin 0.15 units/kg + D50 1 mL/kg Hyperventilation 8. Protect against dysrhythmias (Class 1 antiarrhythmics). Procainamide 15 mg/kg IV Lidocaine 2 mg/kg IV Co-administration of a calcium channel blocker with dantrolene can precipitate life-threatening hyperkalemia! 9. Maintain urine output > 2 mL/kg/hr. Protects against renal injury from free myoglobin IV hydration Mannitol 0.25 g/kg IV Furosemide 1 mg/kg IV 10. Check coagulation panels 12 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it Disseminated intravascular coagulation is a late compli- cation and signals impending demise. 54. What is Absence of dystrophin ’ destabilizes the sarcolemma the pathophysi- during muscle contraction and increases membrane ology of DMD? permeability. Breakdown of the sarcolemma ’ myoglobin to enter the systemic circulation Calcium freely enters the cell ’ activates proteases that destroy the contractile elements and cause inflammation, fibrosis, and cell death. 55. What is the clin- Atrophy and painless muscle degeneration. ical presentation - Progressive deterioration of skeletal muscle strength of DMD? culminating in profound weakness. 56. What are respi- Kyphoscoliosis ratory considera- - Restrictive lung disease ’ decreased pulmonary reserve tions for DMD? - Respiratory muscle weakness ’ ‘ risk of aspiration and PNA 57. What are car- Degeneration of cardiac muscle diac considera- - “ contractility tions for DMD? - Mitral regurgitation - Cardiomyopathy - CHF Impaired cardiac conduction - Sinus tachycardia and short PR interval. - Increased R wave amplitude in lead I, and deep Q waves in the limb leads. These patients should receive a cardiac workup before surgery 58. What are GI con- ‘ risk of pulmonary aspiration siderations for - Impaired airway reflexes and gastrointestinal hypomotil- DMD? ity 13 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it 59. What is the Cobb The Cobb Angle describes the magnitude of the spinal angle? What is curvature. an indication for surgery? 40 - 50: Indication for surgery 60. What are pul- Restrictive ventilatory defect monary changes - FEV1 and FRC are decreased in scoliosis? - FEV1/FVC ratio is normal - Decreased VC, TLC, FRC, RV - Decreased chest wall compliance 61. What are RV hypertrophy secondary to ‘ PVR cardiovascular - RV strain and RA enlargement on EKG changes in scoliosis? Valvular disease - Mitral valve prolapse (most common) - Mitral regurgitation - Coarctation of the aorta. 62. What are com- Airway plications of the - Upper airway edema - consider leak test prone position? - ETT kink or inadvertent extubation Neck - Neck rotation vertebral compression cerebral hypoper- fusion Eyes - Ischemic optic neuropathy - Central retinal artery occlusion - Corneal abrasion 14 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it Nerve injuries Abdomen - Increased abdominal pressure - Reduced pulmonary compliance (Jackson frame is bet- ter than Wilson frame) 63. What is an im- Assess respiratory reserve with exercise tolerance, ABG, portant preoper- and vital capacity. ative considera- - VC < 40% predicted correlates with requirement for tion before scol- post-op ventilation. iosis surgery? 64. What are 2 com- Prepare for significant blood loss plications of sco- - IV access, type & crossmatch, autologous donation, cell liosis surgery? saver. Venous air embolism is a risk. - It increases dead space and is observed as a reduction in EtCO2 (increased PaCO2 - EtCO2 gradient). 65. What is a The "wake-up" test is a method of assessing neurologic Wake-Up Test? integrity during complex spine surgery. What are compli- cations? Complications - Air embolism - Pain - Damage to surgical instrumentation - Awareness - Tracheal extubation - Removal of intravenous or arterial lines 66. Compare SSEPs Somatosensory Evoked Potentials (SSEP) vs MEPs - Monitors the posterior spinal cord (dorsal column path- way), which is perfused by the posterior spinal arteries (2). - Monitors sensory function - SEPs do NOT monitor motor function - Neuromuscular blockers don't interfere with the monitor 15 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it Motor Evoked Potentials (MEP) - Monitors the anterior spinal cord, which is perfused by the anterior spinal artery (1). - Monitors motor function - Do NOT use neuromuscular blockers 67. How does Temporomandibular joint: Limited mouth opening rheumatoid arthritis impact Cricoarytenoid joints: Decreased diameter of glottic open- the airway? ing Cervical spine: Atlanto-occipital subluxation with flexion and limited extension 68. What region of thoracolumbar spine the spine is not affected by RA? 69. What is Autoimmune disease that targets the synovial joints. the pathophysi- ology of RA? 70. What are S/S of Hallmark of RA: morning stiffness that generally improves RA? with activity. The joints are painful, swollen, and warm. Other symptoms: weakness, fatigue, and anorexia. Lymph node enlargement in the cervical and axillary ar- eas 71. How does RA dif- RA affects the proximal interphalangeal and metacar- fer from OA? pophalangeal joints in the hands and feet. By contrast, osteoarthritis typically affects the weight-bearing joints. 72. How does RA Eyes: affect the eyes - Sjogren's syndrome ’ risk of corneal abrasion 16 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it and nervous sys- Nervous system: tem? - Peripheral neuropathy due to nerve entrapment 73. How does RA af- Pulmonary: fect the cardiac - Pleural effusion and respiratory - Restrictive ventilatory pattern systems? Diffuse interstitial fibrosis Costochondral involvement limits chest wall expansion Cardiac: - Pericardial effusion or tamponade - Restrictive pericarditis - Aortic regurgitation - Valvular fibrosis - Coronary artery arteritis 74. How does RA Endocrine: affect the en- - Adrenal insufficiency and infections due to chronic docrine, renal, steroid therapy GI, and hemato- logical systems? Renal: - Renal insufficiency due to: Vasculitis NSAIDs GI: - NSAIDs ’ gastric ulcers Hematologic: - Anemia - Platelet dysfunction secondary to NSAIDs 75. What labs help Rheumatoid factor is an anti-immunoglobulin antibody diagnose RA? that is increased in 90% of patients with RA. C-reactive protein is increased. Erythrocyte sedimentation rate is increased. 76. 17 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it What medica- Medical management aims at reducing inflammation with tions are used to antirheumatics, glucocorticoids, and NSAIDs. treat RA? 77. What are the Methotrexate causes liver dysfunction and suppresses side effects of the bone marrow. common antirheumatics Cyclosporine prolongs the duration of succinylcholine. medications used to treat RA? 78. What is Proliferation of antinuclear antibodies ’ vasculitis and tis- the pathophysi- sue destruction ology of Sys- temic Lupus Ery- thematosus? 79. What are the Polyarthritis: can affect any joint, but it generally does not most common involve the spine problems in SLE? Dermatitis: "butterfly" rash 80. What are cardiac Pulmonary: and pulmonary - Restrictive ventilatory defect complications of - Pulmonary hypertension SLE? - Pleural effusion - Recurrent PE Cardiac: - Pericarditis (tamponade is uncommon) - Endocarditis - Hypertension - Conduction defects - Raynaud's phenomenon 81. What are airway, Airway: nervous system, - Cricoarytenoiditis ’ Hoarseness, stridor, and airway ob- renal, and hema- struction tological compli- cations of SLE? Nervous system: 18 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it - Stroke - Psychosis/dementia - Peripheral neuropathy Renal: - Nephritis with proteinuria Hematologic: - Antiphospholipid antibodies - Hypercoagulability - Anemia - Thrombocytopenia - Leukopenia 82. What conditions P = Pregnancy exacerbate lu- | = Infection pus? S = Surgery S = Stress E = Enalapril D = D-penicillamine C = Captopril H = Hydralazine | = Isoniazid M = Methyldopa P = Procainamide 83. What is the treat- Medical treatment aims to suppress the immune system: ment of SLE? - Corticosteroids - NSAIDs - Immunosuppressants (cyclophosphamide, azathio- prine, methotrexate, and mycophenolate mofetil) - Antimalarials (hydroxychloroquine and quinacrine) 84. Why are pa- Cricoarytenoid arthritis may present as hoarseness, stri- tients with SLE at dor, and airway obstruction. There's a risk of post-extu- risk of post-extu- bation laryngeal swelling and airway obstruction, neces- bation laryngeal sitating steroids. Consider a smaller endotracheal tube if edema? signs of cricoarytenoiditis are present. 19 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it 85. Why are patients Antiphospholipid antibodies may develop. Although the with SLE at in- aPTT is prolonged, these patients are prone to hyper- creased risk of coagulability and thrombosis. They are at risk for stroke, stroke, DVT, and DVT, and pulmonary embolism. pulmonary em- bolism? 86. What medication Cyclophosphamide inhibits plasma cholinesterase and used in SLE in- increases the duration of succinylcholine. creases the dura- tion of succinyl- choline? 87. What is Marfan Connective tissue disorder Syndrome? What are 3 physical Physical characteristics characteristics? - Patients are often tall with pectus excavatum (sunken chest) - Kyphoscoliosis - Hyperflexible joints (careful with positioning). 88. What are car- Cardiac diac and pul- - Aortic insufficiency monary compli- - Aortic aneurysm (AAA) cations of Marfan - Aortic dissection ’ tamponade syndrome? Pulmonary - Kyphoscoliosis ’ restrictive disease - High risk of spontaneous pneumothorax (careful with PIP) 89. What is Disorder of procollagen and collagen Ehlers-Danlos Syndrome? Complications: What are the - Arterial aneurysms (AAA) main - Increased bleeding tendency (result of poor vessel in- complications? tegrity, not coagulopathy) 90. What is Osteoge- Connective tissue disorder that leads to weak bones. nesis Imperfec- 20 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it ta? What are the Complications main complica- - Fractures (careful with positioning) tions? - Risk of c-spine fracture and reduced cervical ROM. - Blue sclera ’ susceptible to fracture 91. How does Osteo- Kyphoscoliosis and pectus excavatum ’ restrictive disease genesis Imper- fecta affect the pulmonary sys- tem? 92. What hormone is Thyroxine ’ ‘ BMR and VO2 ’ hyperthermia increased in Os- teogenesis Im- perfecta? 93. What is Multiple Demyelinating disease of the CNS. Sclerosis? What are the S/S? Presentation: - Sensory and motor deficiencies - Autonomic instability - Cranial nerve involvement causes bulbar muscle dys- function (aspiration risk). 94. What is the treat- Corticosteroids, interferon, and azathioprine. ment of MS? 95. What can exacer- S/sx can be exacerbated by stress and increased body bate MS? temperature (as small as 1 degree C). 96. How are NMBs Succinylcholine can cause life-threatening hyperkalemia. dosed in MS? Non-depolarizing agents: increased sensitivity 97. Is neuraxial Classic teaching suggests that, while epidural anesthesia anesthesia safe is safe, spinal anesthesia may exacerbate symptoms. in MS? 98. What is the pathophysi- 21 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it ology of Myoton- Dysfunctional calcium sequestration by the sarcoplasmic ic Dystrophy? reticulum ’ prolonged contracture after a voluntary con- traction 99. What are respira- Respiratory tory and cardiac - Difficult ventilation and intubation due to contractions complications of - Respiratory muscle weakness ’ aspiration Myotonic Dystro- phy? Cardiac: cardiomyopathy and dysrhythmias Sensitivity to anesthetic agents 100. How are NMBs Succinylcholine: avoid because in can exacerbate myoto- dosed in Myoton- nia ic Dystrophy? Non-depolarizers: increased sensitivity 101. What conditions Succinylcholine (use a non-depolarizer instead) increase the risk of contractures NMB reversal with anticholinesterases (theoretical con- in Myotonic Dys- cern - consider sugammadex) trophy? Hypothermia (shivering - sustained contractions) 102. What is Scle- Scleroderma causes excessive fibrosis in the skin and roderma? How organs, particularly in the microvasculature. does it affect dif- ferent body sys- Eyes: - dryness predisposes to corneal abrasion. tems? Airway: skin fibrosis - limits mouth opening and mandibu- lar mobility. Lungs: pulmonary fibrosis and pulmonary hypertension. Heart: dysrhythmias and CHF. Blood vessels: decreased compliance - hypertension. Kidneys: renal failure and renal artery stenosis - HTN. Peripheral and cranial nerves: - nerve entrapment by tight connective tissue - neuropathy. 103. What is CREST CREST syndrome is a type of scleroderma: Calcinosis, syndrome? Raynaud's phenomenon, Esophageal hypomotility, Sclerodactyly, Telangiectasia. 22 / 23  Musculoskeletal diseases Study online at https://quizlet.com/_ea86it 104. What are the Increased risk of mucosal bleeding risks associat- - Avoid nasal intubation ed with Telang- iectasias (spider Limited mouth opening veins)? - May necessitate nasal fiberoptic intubation 105. What is Paget's Excess osteoblastic and osteoclastic activity that causes Disease? What abnormally thick, but weak, bone deposits. are the complica- Caused by excessive PTH or calcitonin deficiency. tions? Complications - Pain and fractures - Peripheral nerve entrapment ’ neuropathies 23 / 23