Sickle cell disease, and others

Question 1

What are the four specific anesthetic concerns for patients with glycogen storage disease

Answer 1

Hypoglycemia
Acidosis
Cardiac dysfunction
Hepatic dysfunction

Question 2

What is the normal hemoglobin found in adults, its aa and chromosome location

Answer 2

hemoglobin A
Glutamic acid
6th position of chromosome 11

Question 3

What is the sickle cell hemoglobin, and its aa

Answer 3

hemoglobin S

Valine

Question 4

Thalassemia is assoc. with which hemoglobin abnormality

Answer 4

hemoglobin C

Lysine replaced for glutamic acid

Question 5

Name the four crisis associated with sickle cell disease

Answer 5

vaso-occlusive crisis
aplastic crisis
sequestration crisis
hemoltyic crisis

Question 6

Preop preparation for sickle cell patients include

Answer 6

Correct infections
Achieve adequate hydration- crystalloids
Stabilize hematologic state
-hematologist clearance
-ensure HbA concentration at least 40%
-ensure blood available, central line placed
-ensure hematocrit between 30-40%

Question 7

Intraop management of sickle cell patients include

Answer 7

Avoid Acidosis due to 
-hypoventilation
-preop medication
Maintain optimal oxygenation
-Fi02 > 0.5
Prevent circulatory stasis
-avoid hypotension
-tourniquet use
-positioning hazards
Maintain normal body temp
Spinal/Epidural safe if
-hydration, perfusion, and oxygentation OK
Regional anesthesia-hazard d/t vasoconstr.

Question 8

Postop management of sickle cell patients include

Answer 8

Maintain oxygentation
Maintain good pain control
Maintain normothermia
Maintain intravascular volume 
Ambulate ASAP

Question 9

Osteogenesis Imperfecta is characterized by

Answer 9

collagen production defects causing osteoporosis, joint laxity, tendon weakness, valve disease

Question 10

Anesthetic considerations of osteogenesis imperfecta include

Answer 10

Handle patient carefully-prevent fx
gentle airway management- prevent fx
evaluate CV staus-valve dx
evaluate pulmonary sys- kyphoscoliosis
obtain platelets
prevent hyperthermia

*increased incidence of MH

Question 11

Neurofibromatosis is characterized by

Answer 11

Nervous system supportive tissue disease

• cafe-au-lait spots
• menigiomas, gliomas
• airway obstruction-laryngeal fibromas
• pheochromocytoma
• peripheral nerve sarcomas

Question 12

Anesthetic considerations for Neurofibromatosis include

Answer 12

Airway evaluation prior to induction
fibrosis alveolitis
-v/q mismatch
-dec. pulmonary complicance
--pulmonary HTN
Hypertension/Cardiac dysrhythmias
Cervical spine compromise
Sensitivity to muscle relaxants

Question 13

Ehlers-Danlos Syndrome is characterized by

Answer 13

Connective tissue disorder

• hypermobility of joints
• BLEEDING tendancy
• Dilation of GI/Respiratory tract
• PNEUMOTHORAX
• CONDUCTION abnormalities

Question 14

Anesthetic considerations for Ehlers-Danlos Syndrome include

Answer 14

Avoid invasive monitoring-bleeding tendency
Avoid IM injections
Avoid instrumentation of nose/esophagus
Maintain low airway pressures
REGIONAL anesthesia-CONTRAINDICATED

Question 15

Riley-Day Syndrome (Dysautonomia) is caused by

Answer 15

a deficency of dopamine b-hydroxlyase leading to decreased in NOrepi

Question 16

Riley-Day syndrome is characterized by

Answer 16

Excessive secretions
Vomiting crisis
Vasomotor instability
Gag reflex/esophageal motility impairment
Seizure disorders
Mental retardation
Pain insensitivity

Question 17

Anesthetic management of Riley-Day syndrome include

Answer 17

Thorough pulmonary evaluation
Avoidance of Opioids
Avoidance of Hypotension
Avoid corneal abrasion