CHAPTER 3

Fluid and Electrolyte

Management of the Surgical Patient

BASIC SCIENCE QUESTIONS

1. What percentage of body weight is made up of water?

A. 10-20%
B. 30-40%
C. 50-60%
D. 70-80%

Answer: C
Water constitutes approximately 50 to 60% of total body weight. In an average young adult male 60%
of total body weight is TBW, whereas in an average young adult female it is 50%. The lower
percentage of TBW in females correlates with a higher percentage of adipose tissue and lower
percentage of muscle mass. Estimates of percentage of TBW should be adjusted downward
approximately 10 to 20% for obese individuals and upward by 10% for malnourished individuals.
(See Schwartz 9th ed., pp 51 & 52.)

2. Which of the following is the largest fluid compartment in the body?

A. Plasma
B. Central spinal fluid
C. Interstitial fluid
D. Intracellular fluid

Answer: D
Intracellular fluid is the largest fluid compartment in the body and makes up approximately 40% of
total body weight (Fig. 3.1). Extracellular fluid, which is composed of plasma and interstitial fluid,
makes up 20% of body weight. Central spinal fluid is a very small fluid compartment, composed
mostly of plasma. (See Schwartz 9th ed., p 52; See Fig. 3-1.)
FIG. 3-1. Functional body fluid compartments. TBW = total body water.

3. Which of the following is the cation present in largest amounts in intracellular fluid?
A. Sodium
B. Chloride
C. Potassium
D. Calcium

Answer: C
Potassium is the most common cation present in intracellular fluid (Fig. 3-2). Sodium is the most
common cation present in extracellular fluid (plasma and interstitial fluid). Calcium is virtually
absent in intracellular fluid, and is present only in small amounts in extracellular fluid. Chloride is an
anion. (See Schwartz 9th ed., p 52; See Fig. 3-2.)
FIG. 3-2. Chemical composition of body fluid compartments.

CLINICAL QUESTIONS

1. If 1 liter of 0.9% NaCl solution is given intravenously, how much will be distributed to the
interstitial space?
A. 100 cc
B. 250 cc
C. 400 cc
D. 750 cc

Answer: D
Sodium is confined to the extracellular fluid (ECF) compartment, and because of its osmotic and
electrical properties, it remains associated with water. Therefore, sodium-containing fluids are
distributed throughout the ECF and add to the volume of both the intravascular and interstitial spaces.
Although the administration of sodium-containing fluids expands the intravascular volume, it also
expands the interstitial space by approximately three times as much as the plasma.
One liter of normal saline will be distributed 3:1 to the interstitial space. Therefore, 750 ml will
be distributed to the interstitial space and 250 ml will remain in the intravascular volume. (See
Schwartz 9th ed., p 53.)

2. What is the approximate serum osmolality for a patient with the following laboratory findings?
Na 130 Cl 94 K 5.2 CO2 14 Glucose 360 BUN 84 Creatinine 3.2
A. 270
B. 290
C. 310
D. 330

Answer: C
The principal determinants of osmolality are the concentrations of sodium, glucose, and urea (blood
urea nitrogen, or BUN): Calculated serum osmolality = 2 sodium + (glucose/18) + (BUN/2.8)
For this patient: (130 2) + (360/18) + (84/2.8) = 264 + 20 + 30 = 310 (See Schwartz 9th ed., p
53.)

3. A patient develops a high output fistula following abdominal surgery. The fluid is sent for
evaluation with the following results: Na 135 K 5 Cl 70. Which of the following is the most likely
source of the fistula?
A. Stomach
B. Small bowel
C. Pancreas
D. Biliary tract

Answer: C
The composition of pancreatic secretions is marked by a high level of bicarbonate (HCO3). (Table
3-1) In this example, the patient has a total of 140 mEq of cation (Na + K) and only 70 mEq of anion
(Cl). The remaining 70 mEq (to balance the 140 mEq of cation) must be bicarbonate. (See Schwartz
9th ed., p 54, and Table 3-1.)

TABLE 3-1 Composition of GI secretions

4. Which of the following diagnoses would be most likely in a patient who presents with
normovolemic hyponatremia?
A. Syndrome of inappropriate anti-diuretic hormone secretion (SIADH)
 B. High output renal failure
C. Water toxicity
D. GI losses

Answer: A
Water toxicity would be associated with hypervolemia. Primary renal disease and GI losses would
be expected to result in hypovolemia (Fig. 3-3). A normal volume status in the setting of
hyponatremia should prompt an evaluation for a syndrome of inappropriate secretion of ADH. (See
Schwartz 9th ed., p 56; See Fig. 3-3A.)

FIG. 3-3A. ADH = anti-diuretic hormone; SIADH = syndrome of inappropriate secretion of anti-
diuretic hormone.

5. A patient is admitted with a glucose of 500 and a sodium of 151. Which of the following is the best
approximation of the patients actual serum sodium level?
A. 158
B. 151
C. 145
D. 138

Answer: C
Hyponatremia also can be seen with an excess of solute relative to free water, such as with untreated
hyperglycemia or mannitol administration. Glucose exerts an osmotic force in the extracellular
compartment, causing a shift of water from the intracellular to the extracellular space. Hyponatremia
therefore can be seen when the effective osmotic pressure of the extracellular compartment is normal
or even high. When hyponatremia in the presence of hyperglycemia is being evaluated, the corrected
sodium concentration should be calculated as follows: For every 100-mg/dL increment in plasma
glucose above normal, the plasma sodium should decrease by 1.6 mEq/L.
For this patient, a serum glucose of 500 is roughly 400 mg above normal. To correct for the
elevated serum glucose, multiply 4 1.6 = 6.4. This value can be subtracted from 151 to obtain a
corrected serum sodium of 144.6. (See Schwartz 9th ed., p 55.)
6. Which of the following is the most likely diagnosis in a patient with a serum sodium of 152 mEq/L,
a urine sodium concentration of >20 mEq/L, and a urine osmolality of >300 mOsm/L?
A. Syndrome of inappropriate anti-diuretic hormone (SIADH)
B. Diabetes insipidus
C. Renal tubular disease
D. Cushings syndrome

Answer: D
Hypernatremia results from either a loss of free water or a gain of sodium in excess of water. Like
hyponatremia, it can be associated with an increased, normal, or decreased extracellular volume (see
Fig. 3-3B). Hypervolemic hypernatremia usually is caused either by iatrogenic administration of
sodium-containing fluids, including sodium bicarbonate, or mineralocorticoid excess as seen in
hyperaldosteronism, Cushings syndrome, and congenital adrenal hyperplasia. Urine sodium
concentration is typically >20 mEq/L and urine osmolarity is >300 mOsm/L. (See Schwartz 9 th ed., p
56.)
This patient has hypernatremia, urinary sodium excretion >20mEq/L and elevated urinary
osmolality, which are all suggestive of sodium retention.

FIG. 3-3B. Differential diagnosis of hypernatremia

7. Which of the following can contribute to hyperkalemia in patients with renal insufficiency?
A. Loop diuretics
B. Aspirin
C. Calcium channel blockers
D. Nonsteroidal anti-inflammatory drugs (NSAIDs)
Answer: D
A number of medications can contribute to hyperkalemia, particularly in the presence of renal
insufficiency, including potassium-sparing diuretics, angiotensinconverting enzyme inhibitors, and
NSAIDs. (See Schwartz 9th ed., p 56.) Loop diuretics would tend to contribute to hypokalemia.
Aspirin and calcium channel blockers have no significant effect on potassium levels.

8. Which of the following would cause decreased deep tendon reflexes?

A. Hypokalemia
B. Hypomagnesemia
C. Hypocalcemia
D. Hypoglycemia

Answer: A
Hypokalemia causes decreased deep tendon reflexes. Hypomagnesemia and hypocalcemia cause
increased deep tendon reflexes. Hypoglycemia has no effect on deep tendon reflexes. (See Schwartz
9th ed., p 57.)

9. Which of the following is an early ECG change seen in hyperkalemia?

A. Prolonged PR interval
B. Sine wave formation
C. Peaked T waves
D. Flattened P wave

Answer: C
Although all of the listed findings are associated with hyperkalemia, peaked T waves are the first
ECG change seen in most patients.
ECG changes that may be seen with hyperkalemia include high peaked T waves (early), widened
QRS complex, flattened P wave, prolonged PR interval (first-degree block), sine wave formation,
and ventricular fibrillation. (See Schwartz 9th ed., p 57.)

10. A postoperative patient with a potassium of 2.9 is given 1 mEq/kg replacement with KCl
(potassium chloride). Repeat tests after the replacement show the serum K to be 3.0. The most
likely diagnosis is
A. Hypomagnesemia
B. Hypocalcemia
C. Metabolic acidosis
D. Metabolic alkalosis

Answer: A
In cases in which potassium deficiency is due to magnesium depletion, potassium repletion is difficult
unless hypomagnesemia is first corrected. (See Schwartz 9th ed., p 57.)
Alkalosis will change serum potassium (a decrease in 0.3 mEq/L for every 0.1 increase in pH
above normal). This is not enough to explain the lack of response to repletion in the patient.
Metabolic acidosis would not decrease potassium. Calcium does not play a role in potassium
metabolism.

11. What is the actual serum calcium level in a patient with an albumin of 2.0 and a serum calcium
level of 6.6?
A. 6.6
B. 7.4
C. 8.2
D. 9.9

Answer: C
When total serum calcium levels are measured, the albumin concentration must be taken into
consideration: Adjust total serum calcium down by 0.8 mg/dL for every 1-g/dL decrease in albumin.
(See Schwartz 9th ed., p 57.)
0.8 2 = 1.6 + 6.6 = 8.2

12. Which of the following is a cause of acute hypophosphatemia?

A. Chronic ingestion of magnesium containing laxatives
B. Insulin coma
C. Refeeding syndrome
D. Rhabdomyolosis

Answer: C
Acute hypophosphatemia is usually caused by an intracellular shift of phosphate in association with
respiratory alkalosis, insulin therapy, refeeding syndrome, and hungry bone syndrome. Clinical
manifestations of hypophosphatemia usually are absent until levels fall significantly. In general,
symptoms are related to adverse effects on the oxygen availability of tissue and to a decrease in high-
energy phosphates and can be manifested as cardiac dysfunction or muscle weakness.
Refeeding syndrome occurs when excess calories are given to a starved person (anorexia).
Refeeding syndrome is a potentially lethal condition that can occur with rapid and excessive feeding
of patients with severe underlying malnutrition due to starvation, alcoholism, delayed nutritional
support, anorexia nervosa, or massive weight loss in obese patients. With refeeding, a shift in
metabolism from fat to carbohydrate substrate stimulates insulin release, which results in the cellular
uptake of electrolytes, particularly phosphate, magnesium, potassium, and calcium. (See Schwartz 9th
ed., p 64.)
Magnesium containing laxatives can cause hypermagnesemia in patients with renal failure but
does not affect phosphorous levels.
Patients with insulin coma (hypoglycemia) are not at risk for hypophosphatemia. However,
hypophosphatemia is common in diabetic ketoacidosis.
Rhabdomyolosis is associated with hyperkalemia and hyperphosphatemia.

13. Hypomagnesemia clinically resembles which of the following?

A. Hypoglycemia
 B. Hypokalemia
C. Hypophosphatemia
D. Hypocalcemia

Answer: D
The magnesium ion is essential for proper function of many enzyme systems. Depletion is
characterized by neuromuscular and central nervous system hyperactivity. Symptoms are similar to
those of calcium deficiency, including hyperactive reflexes, muscle tremors, tetany, and positive
Chvosteks and Trousseaus signs (see Table 3-2). Severe deficiencies can lead to delirium and
seizures. A number of ECG changes also can occur and include prolonged QT and PR intervals, ST-
segment depression, flattening or inversion of P waves, torsades de pointes, and arrhythmias.
Hypomagnesemia is important not only because of its direct effects on the nervous system but also
because it can produce hypocalcemia and lead to persistent hypokalemia. When hypokalemia or
hypocalcemia coexists with hypomagnesemia, magnesium should be aggressively replaced to assist in
restoring potassium or calcium homeostasis. (See Schwartz 9th ed., p 58.)

TABLE 3-2 Clinical manifestations of abnormalities in potassium, magnesium, and calcium levels

14. A patient presents obtunded to the ER with the following labs:

Na 130 Cl 105 K 3.2 HCO3 15
Which of the following is the most likely diagnosis?
A. GI losses
B. Lactic acidosis
C. Methanol ingestion
D. Renal failure

Answer: A
This is a normal anion gap acidosis. Lactic acidosis, methanol ingestion, and renal failure are all
associated with an increased anion gap. (See Schwartz 9th ed., p 58; See Table 3-3.)
Evaluation of a patient with a low serum bicarbonate level and metabolic acidosis includes
determination of the anion gap (AG), an index of unmeasured anions.
AG = (Na) (Cl + HCO3)
The normal AG is 12 mmol/L and is due primarily to the albumin effect, so that the estimated
AG must be adjusted for albumin (hypoalbuminemia reduces the AG). Corrected AG = actual AG
[2.5(4.5 albumin)].
Metabolic acidosis with an increased AG occurs either from ingestion of exogenous acid such as
from ethylene glycol, salicylates, or methanol, or from increased endogenous acid production of the
following:
Hydroxybutyrate and acetoacetate in ketoacidosis
Lactate in lactic acidosis
Organic acids in renal insufficiency

TABLE 3-3 Etiology of metabolic acidosis

Increased Anion Gap Metabolic acidosis

Exogenous acid ingestion
Ethylene glycol
Salicylate
Methanol
Endogenous acid production
Ketoacidosis
Lactic acidosis
Renal insufficiency
Normal Anion Gap
Acid administration (HCl)
Loss of bicarbonate
GI losses (diarrhea, fistulas)
Ureterosigmoidoscopy
Renal tubular acidosis
Carbonic anhydrase inhibitor

15. Which of the following is the best choice to replace isotonic (serum) fluid loss?
A. D5 NS with 20 mEq KCl/liter
B. D5 NS with 20 mEq KCl/liter
C. 3% saline solution
D. Lactated Ringers

Answer: D
Lactated Ringers best approximates serum electrolytes and would be the fluid of choice to replace
isotonic serum fluid loss. (See Schwartz 9th ed., p 60; See Table 3-4.)

TABLE 3-4 Electrolyte solutions for parenteral administration

16. Which of the following should be the first treatment administered to a patient with a potassium
level of 6.3 and flattened P waves on their ECG?
A. Kayexalate
B. Insulin and glucose
C. Calcium gluconate
D. Inhaled albuterol

Answer: B
Treatment options for symptomatic hyperkalemia are listed in Table 3-5. The goals of therapy include
reducing the total body potassium, shifting potassium from the extracellular to the intracellular space,
and protecting the cells from the effects of increased potassium. For all patients exogenous sources of
potassium should be removed, including potassium supplementation in IV fluids and enteral and
parenteral solutions. Potassium can be removed from the body using a cation-exchange resin such as
Kayexalate that binds potassium in exchange for sodium. It can be administered either orally, in alert
patients, or rectally. Immediate measures also should include attempts to shift potassium
intracellularly with glucose and bicarbonate infusion. Nebulized albuterol (10 to 20 mg) may also be
used. Use of glucose alone will cause a rise in insulin secretion, but in the acutely ill this response
may be blunted, and therefore both glucose and insulin may be necessary. Circulatory overload and
hypernatremia may result from the administration of Kayexalate and bicarbonate, so care should be
exercised when administering these agents to patients with fragile cardiac function. When ECG
changes are present, calcium chloride or calcium gluconate (5 to 10 mL of 10% solution) should be
administered immediately to counteract the myocardial effects of hyperkalemia. Calcium infusion
should be used cautiously in patients receiving digitalis, because digitalis toxicity may be
precipitated. All of the aforementioned measures are temporary, lasting from 1 to approximately 4
hours. Dialysis should be considered in severe hyperkalemia when conservative measures fail. (See
Schwartz 9th ed., p 60, and Table 3-5.)

TABLE 3-5 Treatment of symptomatic hyperkalemia

Potassium removal
Kayexalate
Oral administration is 1530 g in 50100 mL of 20% sorbitol
Rectal administration is 50 g in 200 mL of 20% sorbitol
Dialysis
Shift potassium
Glucose 1 ampule of D50 and regular insulin 510 units IV
Bicarbonate 1 ampule IV
Counteract cardiac effects
Calcium gluconate 510 mL of 10% solution

D50 = 50% dextrose.

17. The approximate IV rate for maintenance fluids for a 50-kg patient would be
A. 75 ml/hr
B. 90 ml/hr
C. 105 ml/hr
D. 120 ml/hr

Answer: B
Once the daily total is established, dividing by 24 will give an approximate hourly rate.
Alternatively, dividing by 25 (instead of 24) gives a rapid approximate rate. In other words, the
hourly IV rate will be
4 ml/kg/hour for the 1st 10 kg
2 ml/kg/hour for the 2nd 10 kg
1 ml/kg/hour for each kg >20 kg
In this example, 4 10 = 40 (for the 1st 10 kg), 2 10 = 20 (for the 2nd 10 kg), and 1 30 = 30
(for the remaining kg). 40 + 20 + 30 = 90 ml/hr. (The number if one divides by 24 instead of 25 is
87.5 ml/hr.) (See Schwartz 9th ed., p 63.)
 CHAPTER 4
Hemostasis, Surgical Bleeding,
and Transfusion

BASIC SCIENCE QUESTIONS

1. What percentage of platelets can be sequestered in the spleen?

A. 15%
B. 30%
C. 45%
D. 60%

Answer: B
Platelets are anucleate fragments of megakaryocytes. The normal circulating number of platelets
ranges between 150,000 and 400,000/L. Up to 30% of circulating platelets may be sequestered in
the spleen. (See Schwartz 9th ed., p 68.)

2. Which of the following is required for platelet adherence to exposed areas of an injured vessel?
A. Prothrombin
B. von Willebrand factor
C. Glycoprotein IX
D. Prostaglandin GI2

Answer: B
Platelets do not normally adhere to each other or to the vessel wall but can form a plug that aids in
cessation of bleeding when vascular disruption occurs. Injury to the intimal layer in the vascular wall
exposes subendothelial collagen to which platelets adhere. This process requires von Willebrands
factor (vWF), a protein in the subendothelium that is lacking in patients with von Willebrands
disease. The vWF binds to glycoprotein I/IX/V on the platelet membrane. After adhesion, platelets
initiate a release reaction that recruits other platelets from the circulating blood to seal the disrupted
vessel. Up to this point, this process is known as primary hemostasis.
Prothrombin initiates the common phase of the coagulation cascade, which occurs after primary
hemostasis.
Prostaglandin GI is a vasodilator and inhibits platelet aggregation. (See Schwartz 9th ed., p 68.)

3. Which of the following drugs irreversibly inhibits platelet COX (cyclo-oxygenase)?

 A. Ibuprofen
B. Clopidogrel
C. Aspirin
D. Celebrex

Answer: C
Arachidonic acid released from the platelet membranes is converted by COX to prostaglandin
G2(PGG2) and then to prostaglandin H2(PGH2), which, in turn, is converted to TXA2. TXA2 has
potent vasoconstriction and platelet aggregation effects. Arachidonic acid may also be shuttled to
adjacent endothelial cells and converted to prostacyclin (PGI2), which is a vasodilator and acts to
inhibit platelet aggregation. Platelet COX is irreversibly inhibited by aspirin and reversibly blocked
by NSAIDs but is not affected by COX-2 inhibitors.
Ibuprofen is a nonsteroidal anti-inflammatory drug (NSAID) and reversibly affects platelet
COX.
Both aspirin and clopidogrel irreversibly inhibit platelet function, clopidogrel through selective
irreversible inhibition of ADP-induced platelet aggregation and aspirin through irreversible
acetylation of platelet prostaglandin synthase.
Celebrex is a COX-2 inhibitor and therefore does not affect platelet COX. (See Schwartz 9th
ed., p 68.)

4. An abnormal aPTT (partial thromboplastin time) is associated with an abnormality in which

portion of the clotting mechanism?
A. Platelet aggregation
B. Intrinsic pathway
C. Extrinsic pathway
D. Coagulation (clot formation)

Answer: B
One convenient feature of depicting the coagulation cascade with two merging arms is that commonly
used laboratory tests segregate abnormalities of clotting to one of the two arms (Table 4-1). An
elevated activated partial thromboplastin time (aPTT) is associated with abnormal function of the
intrinsic arm of the cascade, whereas an elevated prothrombin time (PT) is associated with the
extrinsic arm. (See Schwartz 9th ed., p 69.)

TABLE 4-1 Coagulation factors tested by the PT and the aPTT

5. Patients with factor V Leiden are predisposed to thrombosis because they have a genetic mutation
in factor V which
A. Leads to inadequate production of factor V
B. Leads to overproduction of factor V
C. Leads to an inability to inactivate factor V
D. Leads to an inability to activate factor V

Answer: C
A third major mechanism of inhibition of thrombin formation is the protein C system. On its
formation, thrombin binds to thrombomodulin and activates protein C to activated protein C (APC),
which then forms a complex with its cofactor, protein S, on a phospholipid surface. The APCprotein
S complex cleaves factors Va and VIIIa so they are no longer able to participate in the formation of
tissue factorVIIa or prothrombinase complexes. Of interest is an inherited form of factor V that
carries a genetic mutation, called factor V Leiden, that is resistant to cleavage by APC and thus
remains active (procoagulant). Patients with factor V Leiden are predisposed to venous
thromboembolic events. (See Schwartz 9th ed., p 70.)

CLINICAL QUESTIONS

1. A patient with hemophilia has a factor level of 8%. This is considered to be

A. Mild hemophilia
B. Moderately severe hemophilia
C. Severe hemophilia
D. Extremely severe hemophilia

Answer: A
Hemophilia A and hemophilia B are inherited as sex-linked recessive disorders with males being
affected almost exclusively. The clinical severity of hemophilia A and hemophilia B depends on the
measurable level of factor VIII or factor IX in the patients plasma. Plasma factor levels 1% of
normal are considered severe disease, factor levels between 1 and 5% moderately severe, and levels
of 5 to 30% mild disease. Patients with severe hemophilia have severe spontaneous bleeds,
frequently into joints, which leads to crippling arthropathies. Intramuscular hematomas,
retroperitoneal hematomas, and GI, genitourinary, and retropharyngeal bleeding are added clinical
sequelae seen with severe disease. Intracranial bleeding and bleeding from the tongue or lingual
frenulum may be life-threatening with severe disease. Patients with moderately severe hemophilia
have less spontaneous bleeding but are likely to bleed severely after trauma or surgery. Those with
mild disease do not bleed spontaneously and frequently have only minor bleeding after major trauma
or surgery. Because platelet function is normal in individuals with hemophilia, patients may not bleed
immediately after an injury or minor surgery because they have a normal response with platelet
activation and formation of a platelet plug. At times, the diagnosis of hemophilia is not made in these
patients until after their first minor procedure (e.g., tooth extraction or tonsillectomy). (See Schwartz
9th ed., p 71.)

2. Which of the following is the best choice to prepare a patient with type 1 von Willebrands disease
for surgery?
A. Recombinant (pure) factor XIII
B. von Willebrand factor
C. Factor XIII
D. Desmopressin

Answer: D
von Willebrands disease (vWD), the most common congenital bleeding disorder, is characterized by
low levels of factor VIII. It is an autosomal dominant disorder, and the primary defect is a low level
of vWF, a large glycoprotein responsible for carrying factor VIII and platelet adhesion. The latter is
important for normal platelet adhesion to exposed subendothelium and for aggregation under high-
shear conditions. Patients with vWD have bleeding that is characteristic of platelet disorders (i.e.,
easy bruising and mucosal bleeding). Menorrhagia is common in women. vWD is classified into three
types. Type I is a partial quantitative deficiency, type II is a qualitative defect, and type III is total
deficiency. One treatment for vWD is an intermediate-purity factor VIII concentrate such as Humate-P
that contains vWF as well as factor VIII. The second treatment strategy is desmopressin acetate,
which raises endogenous vWF levels by triggering release of the factor from endothelial cells.
Desmopressin acetate is used once a day because time is needed for synthesis of new stores of vWF
within the endothelial cells. Historically, patients with type I disease have been found to respond
well to desmopressin acetate. Type II patients may respond, depending on the particular defect. Type
III patients are usually unresponsive. (See Schwartz 9th ed., p 71.)

3. Hemophilia C is caused by a deficiency of

A. Factor VIII
B. Factor IX
C. Factor X
D. Factor XI
Answer: D
Factor XI deficiency, an autosomal recessive inherited condition sometimes referred to as
hemophilia C, is more prevalent in the Ashkenazi Jewish population. Spontaneous bleeding is rare,
but bleeding may occur after surgery, trauma, or invasive procedures. Patients with factor XI
deficiency who present with bleeding or for whom surgery is planned and who are known to have
bled previously are treated with fresh-frozen plasma (FFP). Each milliliter of plasma contains 1 unit
of factor XI activity, so the volume needed depends on the patients baseline level, the desired level,
and the plasma volume. Recombinant factor VIIa treatment has been used successfully in children
with severe factor XI deficiency who require major operations such as open heart surgery.
Desmopressin acetate also may be useful in the prevention of surgical bleeding in these patients. (See
Schwartz 9th ed., pp 71-72.)

4. Factor XIII deficiency most commonly presents as

A. Severe intraoperative bleeding
B. Delayed bleeding after injury or surgery
C. Spontaneous hemarthrosis
D. Spontaneous gastrointestinal bleeding

Answer: B
Congenital factor XIII deficiency, originally recognized by Franois Duckert in 1960, is a rare
autosomal recessive disease usually associated with a severe bleeding diathesis. The male:female
ratio is 1:1. Although acquired factor XIII deficiency has been described in association with hepatic
failure, inflammatory bowel disease, and myeloid leukemia, the only significant association with
bleeding in children is the inherited deficiency. Bleeding typically is delayed, because clots form
normally but are susceptible to fibrinolysis. Umbilical stump bleeding is characteristic, and there is a
high risk of intracranial bleeding. Spontaneous abortion is usual in women with factor XIII deficiency
unless they receive replacement therapy. Replacement can be accomplished with FFP,
cryoprecipitate, or a factor XIII concentrate. Levels of 1 to 2% are usually adequate for hemostasis.
(See Schwartz 9th ed., p 72.)

5. Bleeding in patients with thrombasthenia is treated with

A. Factor V
B. Factor VII
C. Fresh frozen plasma transfusion
D. Platelet transfusion

Answer: D
Thrombasthenia or Glanzmann thrombasthenia is a rare genetic platelet disorder, inherited in an
autosomal recessive pattern, in which the platelet glycoprotein IIb/IIIa complex is either lacking or
present but dysfunctional. This defect leads to faulty platelet aggregation and subsequent bleeding.
The disorder was first described by Dr. Eduard Glanzmann in 1918. Bleeding in thrombasthenic
patients must be treated with platelet transfusions. (See Schwartz 9th ed., p 72.)
6. Bleeding in patients with the Bernard-Soulier syndrome is treated with
A. Factor V
B. Factor VII
C. Fresh frozen plasma transfusion
D. Platelet transfusion

Answer: D
The Bernard-Soulier syndrome, caused by a defect in the glycoprotein Ib/IX/V receptor for vWF, is
necessary for platelet adhesion to the subendothelium. Transfusion of normal platelets is required to
treat bleeding in these patients. (See Schwartz 9th ed., p 72.)

7. A patient with partial albinism and a bleeding disorder most likely has
A. von Willebrands disease
B. Hemophilia C
C. Dense granule deficiency
D. Factor XIII deficiency

Answer: C
The most common intrinsic platelet defect is storage pool disease. It involves loss of dense granules
[storage sites for ADP, adenosine triphosphate (ATP), Ca 2+, and inorganic phosphate] and -
granules. Dense granule deficiency is the most prevalent of these. It may be an isolated defect or
occur with partial albinism in the Hermansky-Pudlak syndrome. Bleeding is variable, depending on
the severity of the granule defect. Bleeding is caused by the decreased release of ADP from these
platelets. Patients with mild bleeding as a consequence of a form of storage pool disease can be
treated with desmopressin acetate. It is likely that the high levels of vWF in the plasma after
desmopressin acetate administration somehow compensate for the intrinsic platelet defect. With more
severe bleeding, platelet transfusion is required. (See Schwartz 9th ed., p 72.)

8. First line therapy in an adult with idiopathic thrombocytopenia purpura includes

A. Retuximab
B. Splenectomy
C. IV immunoglobulin
D. Desmopressin

Answer: C
First line therapy for ITP in adults is corticosteroids and IV immunoglobulin. Splenectomy is second
line therapy. Desmopressin is not used in the treatment of ITP. (See Schwartz 9 th ed., pp 72-73, and
Table 4-2.)

TABLE 4-2 Management of idiopathic thrombocytopenic purpura (ITP) in adults

First Line
a. Corticosteroids: The majority of patients respond, but only a few long term.

b. IV immunoglobulin: Indicated with clinical bleeding, along with platelet transfusion, and when
condition is steroid unresponsive. Response is rapid but transient.

c. Anti-D immunoglobulin: Active only in Rh-positive patients before splenectomy. Response is

transient.

Second Line

a. Splenectomy: Open or laparoscopic. Criteria include severe thrombocytopenia, high risk of

bleeding, and continued need for steroids. Treatment failure may be due to retained accessory
splenic tissue.

Third Line
a. Patients for whom first- and second-line therapies fail are considered to have chronic ITP. The
objective in this subset of patients is to maintain the platelet count >2030109/L and to minimize
side effects of medications.

b. Rituximab, an anti-CD20 monoclonal antibody: Acts by eliminating B cells.

c. Alternative medications producing mixed results and a limited response: Danazol, cyclosporine A,
dapsone, azathioprine, and vinca alkaloids.

d. Thrombopoietic agents: A new class of drugs for patients with impaired production of platelets
rather than accelerated destruction of platelets. Second-generation drugs still in clinical trials
include AMG531 and eltrombopag.

9. The diagnosis of heparin-induced thrombocytopenia is made by

A. >20% fall in platelet count
B. Positive serotonin release assay
C. Platelets 25,000 with clinical bleeding
D. Prolonged aPTT

Answer: B
Heparin-induced thrombocytopenia (HIT) is a form of drug induced immune thrombocytopenia. It is
an immunologic disorder in which antibodies against PF4 formed during exposure to heparin affect
platelet activation and endothelial function with resultant thrombocytopenia and intravascular
thrombosis. The platelet count typically begins to fall 5 to 7 days after heparin has been started, but if
it is a re-exposure, the decrease in count may occur within 1 to 2 days. HIT should be suspected if the
platelet count falls to 100,000/L or if it drops by 50% from baseline in a patient receiving heparin.
Although HIT is more common with full-dose unfractionated heparin (1 to 3%), it also can occur with
prophylactic doses or with low molecular weight heparins. Interestingly, approximately 17% of
patients receiving unfractionated heparin and 8% of those receiving low molecular weight heparin
develop antibodies against PF4, yet a much smaller percentage develop thrombocytopenia and even
fewer clinical HIT. In addition to the mild to moderate thrombocytopenia, this disorder is
characterized by a high incidence of thrombosis, which may be arterial or venous. Importantly, the
absence of thrombocytopenia in these patients does not preclude the diagnosis of HIT.
The diagnosis of HIT may be made by using either a serotonin release assay or an enzyme-linked
immunosorbent assay (ELISA). The serotonin release assay is highly specific but not sensitive, so that
a positive test result supports the diagnosis but a negative result does not exclude HIT. On the other
hand, the ELISA has a low specificity, so although a positive ELISA result confirms the presence of
antiheparin-PF4, it does not help in the diagnosis of clinical HIT. A negative ELISA result,
however, essentially rules out HIT. (See Schwartz 9th ed., p 73.)

10. In addition to stopping the heparin, a patient with heparin-induced thrombocytopenia (HIT) should
be treated with
A. Lepirudin
B. Low molecular weight heparin
C. Warfarin
D. Aspirin

Answer: A
The initial treatment of suspected HIT is to stop heparin and begin an alternative anticoagulant.
Stopping heparin without adding another anticoagulant is not adequate to prevent thrombosis in this
setting. Alternative anticoagulants are primarily thrombin inhibitors. Those available in the United
States are lepirudin, argatroban, and bivalirudin. In Canada and Europe, danaparoid also is available.
Danaparoid is a heparinoid that has approximately 20% cross reactivity with HIT antibodies in vitro
but a much lower cross reactivity in vivo. Because of warfarins early induction of a hypercoagulable
state, only once full anticoagulation with an alternative agent has been accomplished and the platelet
count has begun to recover should warfarin be instituted. (See Schwartz 9th ed., p 73.)

11. The most effective treatment for bleeding secondary to thrombotic thrombocytopenic purpura is
A. Platelet transfusion
B. Desmopressin
C. Emergency splenectomy
D. Plasmapheresis

Answer: D
In thrombotic thrombocytopenic purpura (TTP), large vWF molecules interact with platelets, which
leads to activation. These large molecules result from inhibition of a metalloproteinase enzyme,
ADAMTS13, which cleaves the large vWF molecules. TTP is classically characterized by
thrombocytopenia, microangiopathic hemolytic anemia, fever, and renal and neurologic signs or
symptoms. The finding of schistocytes on a peripheral blood smear aids in the diagnosis. The most
effective treatment for TTP is plasmapheresis, although plasma infusion also has been attempted. A
recent study comparing these two modalities reported a higher relapse rate and a higher mortality
with plasma infusions. Platelet transfusions are contraindicated. Additionally, rituximab, a
monoclonal antibody against the CD20 protein on B lymphocytes, has shown promise as an
immunomodulatory therapy directed against acquired TTP, which in the majority of cases is
autoimmune mediated. (See Schwartz 9th ed., p 73.)

12. In a 70-kg patient, transfusion of 1 unit of platelets should raise the circulating platelet count by
approximately
A. 10,000
B. 20,000
C. 30,000
D. 40,000

Answer: A
One unit of platelet concentrate contains approximately 5.5 1010 platelets and would be expected to
increase the circulating platelet count by approximately 10,000/L in the average 70-kg person. (See
Schwartz 9th ed., p 74.)

13. Which of the following is a common initiating event for disseminated intravascular coagulation
(DIC)?
A. Spider bite
B. Depressed skull fracture
C. Type A influenza
D. Amniotic fluid embolization

Answer: D
The presence of an underlying condition that predisposes a patient to DIC is required for the
diagnosis. Specific injuries include central nervous system injuries with embolization of brain matter,
fractures with embolization of bone marrow, and amniotic fluid embolization. Embolized materials
are potent thromboplastins that activate the DIC cascade. Additional causes include malignancy,
organ injury (such as severe pancreatitis), liver failure, certain vascular abnormalities (such as large
aneurysms), snakebites, illicit drugs, transfusion reactions, transplant rejection, and sepsis. DIC
frequently accompanies sepsis and may be associated with multiple organ failure. (See Schwartz 9th
ed., p 74.)

14. A patient with a prolonged aPTT and deep venous thrombosis should be evaluated for which of
the following conditions?
A. Heparin-induced thrombocytopenia
B. Thrombotic thrombocytopenic purpura
C. Antiphospholipid syndrome
D. Protein C deficiency
Answer: C
Among the most common acquired disorder of coagulation inhibition is the antiphospholipid
syndrome (APLS), in which the lupus anticoagulant and anticardiolipin antibodies are present. These
antibodies may be associated with either venous or arterial thrombosis, or both. In fact, patients who
show recurrent thrombosis should be evaluated for APLS. The presence of antiphospholipid
antibodies is very common in patients with systemic lupus erythematosus but also may be seen in
association with rheumatoid arthritis and Sjgrens syndrome. There are also individuals who have
no autoimmune disorders but develop transient antibodies in response to infections or who develop
drug-induced APLS. The hallmark of APLS is a prolonged aPTT in vitro but an increased risk of
thrombosis in vivo. (See Schwartz 9th ed., p 75.)

15. Which of the following would increase the effect of warfarin and require a decrease in the dose
given for anticoagulation?
A. Barbiturates
B. Corticosteroids
C. Cephalosporins
D. Oral contraceptive pills

Answer: C
Cephalosporins are among the agents that can increase the effect of warfarin. (See Schwartz 9th ed., p
76, and Table 4-3.)

TABLE 4-3 Medications that can alter warfarin dosing

16. A patient on chronic warfarin therapy presents with acute appendicitis. INR is 1.4. Which of the
following is the most appropriate management?
A. Proceed immediately with surgery without stopping the warfarin
B. Stop the warfarin, give fresh frozen plasma, and proceed with surgery
C. Stop the warfarin and proceed with surgery in 8-12 hours
D. Stop the warfarin and proceed with surgery in 24-36 hours

Answer: A
Surgical intervention may prove necessary in patients receiving anticoagulation therapy. Increasing
experience suggests that surgical treatment can be undertaken without discontinuing the anticoagulant
program, depending on the procedure being performed. Furthermore, the risk of thrombotic
complications may be increased when anticoagulation therapy is discontinued abruptly. When the
aPTT is 1.3 times the control value in a patient receiving heparin or when the INR is 1.5 in a patient
taking warfarin, reversal of anticoagulation therapy may not be necessary. However, meticulous
surgical technique is mandatory, and the patient must be observed closely throughout the
postoperative period. (See Schwartz 9th ed., p 76.)

17. Which of the following devices is most advantageous for hemostasis during a thyroidectomy?
A. Monopolar electrocautery
B. Bipolar electrocautery
C. Harmonic scalpel
D. Argon coagulator

Answer: C
The Harmonic scalpel is an instrument that cuts and coagulates tissue via vibration at 55 kHz. The
device converts electrical energy into mechanical motion. The motion of the blade causes collagen
molecules within the tissue to become denatured, forming a coagulum. No significant electrical
current flows through the patient. The instrument has proved advantageous in performing
thyroidectomy, hemorrhoidectomy, and transsection of the short gastric veins during splenectomy, and
in transecting hepatic parenchyma.
Heat achieves hemostasis by denaturation of protein that results in coagulation of large areas of
tissue. With cautery, heat is transmitted from the instrument by conduction directly to the tissue. When
electrocautery is used, heating occurs by induction from an alternating current source. (See Schwartz
9th ed., p 77.)

18. Which topical anticoagulating agent is best for use in patients with a coagulopathy?
A. Gelfoam
B. Fibrin sealant
C. Thrombostat (topical thrombin)
D. Surgiceal

Answer: B
Thrombin-derivative products direct the conversion of fibrinogen to fibrin, aiding in clot formation.
Thrombin takes advantage of natural physiologic processes, thereby avoiding foreign body or
inflammatory reactions, and the wound bed is not disturbed.
Fibrin sealants are prepared from cryoprecipitate (homologous or synthetic) and have the
advantage of not promoting inflammation or tissue necrosis. The sealant is administered using a dual
syringe compartment system. In one compartment is fibrinogen, factor XIII, fibronectin, and
fibrinolysis inhibitors. The second compartment contains thrombin and calcium chloride. The use of
fibrin glue is particularly helpful in patients who have received heparin or who have deficiencies in
coagulation (e.g., hemophilia or von Willebrands disease).
Purified gelatin solution can be prepared into several vehicles, including powders, sponges or
foams, and sheets or films. Gelatin is hygroscopic, absorbing many times its weight in water or
liquid. It is effectively metabolized and degraded by proteinases in the wound bed over a period of 4
to 6 weeks. Gelfoam provides effective hemostasis for operative fields with diffuse small-vessel
oozing. Thrombin may be applied to this vehicle to boost hemostasis. Gelatin is relatively
inexpensive, readily available, pliable, and easy to handle. Although relatively inert, the implanted
gelatin can serve as a nidus for infection. (See Schwartz 9th ed., p 77, and Table 4-4.)

TABLE 4-4 Common hemostatic agents

19. What percent of the population is Rh negative?

A. 5%
B. 15%
C. 25%
D. 35%

Answer: B
Rh-negative recipients should receive transfusions only of Rh-negative blood. However, this group
represents only 15% of the population. Therefore, the administration of Rh-positive blood is
acceptable if Rh-negative blood is not available. However, Rh-positive blood should not be
transfused to Rh-negative females who are of childbearing age. (See Schwartz 9th ed., p 78.)

20. What is the maximum number of units of blood that can be autologously donated for elective
surgery as long as the patients hemoglobin is >11 gm?
A. A single donation can be made 2-3 weeks before surgery
B. 2 donations can be made 2 and 4 weeks before surgery
C. 3 donations can be made, 1 week apart, starting 4 weeks before surgery
D. 5 donations can be made, 3-4 days apart, starting 6 weeks before surgery

Answer: D
The use of autologous transfusion is growing. Up to 5 units can be collected for subsequent use during
elective procedures. Patients can donate blood if their hemoglobin concentration exceeds 11 g/dL or
if the hematocrit is >34%. The first procurement is performed 40 days before the planned operation
and the last one is performed 3 days before the operation. Donations can be scheduled at intervals of
3 to 4 days. Administration of recombinant human erythropoietin accelerates generation of red blood
cells and allows for more frequent harvesting of blood. (See Schwartz 9th ed., p 78.)

21. When should cryoprecipitate be given to a patient needing a massive transfusion of packed
 RBCs?
A. 1 unit of cryoprecipitate should be given for each unit of PRBCs
B. 10 units of cryoprecipitate should be given for each unit of PRBCs
C. After 6 units of PRBCs, cryoprecipitate should be given if the serum fibrinogen level is 100
mg/dl
D. Never, fresh frozen plasma will provide the necessary factors

Answer: C
(See Schwartz 9th ed., p 80, and Table 4-5.)

TABLE 4-5 Component therapy administration during massive transfusion

22. Which of the following best assesses clot strength?

A. Clinical history
B. Thrombin levels
C. Ivy bleeding time
D. Thromboelastogram (TEG)

Answer: D
TEG measures the viscoelastic properties of blood as it is induced to clot in a low-shear environment
(resembling sluggish venous flow). The patterns of change in shear elasticity allow the kinetics of
clot formation and growth as well as the strength and stability of the formed clot to be determined.
The strength and stability data provide information about the ability of the clot to perform the work of
hemostasis, whereas the kinetic data determine the adequacy of quantitative factors available for clot
formation.
The usefulness of TEG has been sufficiently documented in general surgery, cardiac surgery,
urologic surgery, obstetrics, pediatrics, and liver transplantation. It is the only test measuring all
dynamic steps of clot formation until eventual clot lysis or retraction. Its role in evaluating
coagulopathic patients is still being investigated. (See Schwartz 9th ed., p 84.)