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menses despite developing breasts 3 years ago. Her previous medical history is unremarkable,
and she has never been sexually active. On examination, her height is at the 65th percentile,
weight is at the 50th percentile, and blood pressure is 110/70 mm Hg. Breasts exhibit Tanner
stage IV development. Vaginal examination demonstrates a short, blind vaginal pouch and pelvic
ultrasound fails to locate a uterus. What is the next best step in the diagnosis of this patient?
D. Serum progesterone
QID: 33090
Patients with primary amenorrhea, breast development, and Müllerian anomalies all
fail to demonstrate a visible or palpable uterine cervix on physical examination. They
fall into two categories: those with complete androgen insensitivity syndrome (46 XY)
and those with a karyotype of 46 XX. The distinction can be made by the serum
testosterone level. Patients with complete androgen insensitivity syndrome have male
levels of testosterone.
The karyotype in patients with androgen insensitivity syndrome is 46 XY, and they have
testes that are often intraabdominal. Breast development (with smaller nipples and areolae
than normal) is caused by an enzymatic conversion of male levels of androgen to estrogen.
The testes in these patients secrete normal male amounts of Müllerian-inhibiting
substance; hence, patients have only a vaginal dimple and no uterus or tubes.
Treatment should consist of gonadal resection to avoid malignant neoplasia once puberty is
complete and the creation of a neovagina when the patient is prepared to be sexually active.
Psychological counseling is also an important component in the care of these patients.
Alternatively, women may present with similar symptoms but no lower vaginal bulge. When
ultrasonography confirms a normal uterus and ovaries, one should suspect the possibility of
a transverse, obstructing vaginal septum or cervical agenesis. MRI is the diagnostic
method of choice in these patients. If the MRI scan confirms a transverse septum, surgical
correction is indicated. These procedures can be extremely difficult, and the surgeon must
be prepared to use tissue expanders, split-thickness skin grafts, or other techniques to effect
a functional vagina. Surgical construction of a functional cervix is extremely unlikely. In
general, it is recommended that these women undergo hysterectomy.
Creation of a neovagina can be accomplished by using one of two general approaches. The
Frank method of vaginal dilatation uses dilatation of the vaginal pouch with vaginal forms
(usually thermoplastic acrylic resin [Lucite] dilators) over the course of weeks to months.
Alternatively, a McIndoe vaginoplasty, which involves the surgical creation of a neovaginal
space using a split-thickness skin graft, may be performed. Both of these methods should be
initiated in proximity to the time when the patient anticipates having vaginal intercourse.
A 29-year-old G2P1001 at 28 weeks’ gestation presents for a prenatal office visit. Her pregnancy
has been complicated by red cell sensitization with an anti-D titer of 1:128. Ultrasound reveals an
appropriately grown fetus with evidence of fetal hydrops, including scalp edema and ascites.
Which of the following is the most appropriate next step in patient care?
A. Amniocentesis
B. Cesarean delivery
D. Induction of labor
QID: 26716
Option C (Fetal blood sampling) is correct. This fetus is demonstrating sonographic signs
of hydrops due to anemia resulting from red cell sensitization. At this gestational age, the
plan of management would be fetal blood sampling to confirm anemia and intrauterine blood
transfusion.
Option A (Amniocentesis) is incorrect. This fetus already demonstrates sonographic
signs of hydrops; an amniocentesis would not add any valuable information.
Option B (Cesarean delivery) is incorrect. Due to the early gestational age, the fetus
should be treated in utero, rather than delivered.
Option D (Induction of labor) is incorrect. Due to the early gestational age, the fetus
should be treated in utero, rather than delivered.
Option E (Repeat anti-D titer) is incorrect. This fetus already demonstrates sonographic
signs of hydrops; a repeat anti-D titer would not add any valuable information.
Serial ultrasonic examinations of a woman with a fetus at risk for hemolytic disease can be a
useful adjunct to amniocentesis in confirming fetal well-being and determining the advent of
fetal hydrops. The examination should include a routine fetal assessment plus a
determination of placental size and thickness and hepatic size. Both the placenta and the
fetal liver are enlarged with hydrops. Fetal hydrops is easily diagnosed by the
characteristic appearance of one or more of the following: ascites, pleural effusion,
pericardial effusion, or skin edema. Appearance of any of these factors during an
ultrasonic examination eliminates the need for diagnostic amniocentesis and necessitates
therapeutic intervention based on fetal gestational age.
Doppler assessment of peak velocity in the fetal middle cerebral artery (MCA) may
prove to be the most valuable ultrasonic tool for detecting fetal anemia. A value above
1.5 multiples of the median for gestational age is considered predictive. For accurate
evaluation, the Doppler gate is placed over the fetal MCA just as it bifurcates from the
carotid siphon. Color Doppler is clearly advantageous for this examination. After 35 weeks'
gestation, this test may produce a higher false-positive rate (Figure 16-3).
Figure 16-1 Modified Liley chart used to determine the appropriate management of the patient with isoimmunization. The ΔOD
450 nm level in the amniotic fluid at a given weeks' gestation determines whether fetal transfusion or delivery is advisable.
Figure 16-2 Queenan curve for ΔOD 450 values for the management of the patient with isoimmunization. OD, optical density;
Rh, rhesus. Adapted from Queenan JT, Tomai TP, Ural SH, et al: Deviations in amniotic fluid optical density at a wavelength of
450 nm in Rh-immunized pregnancies from 14 to 40 weeks' gestation: A proposal for clinical management. Am J Obstet
Gynecol 168:1370-1376, 1993.
Figure 16-3 Middle cerebral artery (MCA) Doppler peak velocities based on gestational age. MoM, multiples of the median.
Data from Moise KJ, Jr: Management of Rhesus alloisoimmunization. Obstet Gynecol 100(3):600-611, 2002.
*Values are for normal fetuses from 15 to 30 weeks' estimated gestational age.
Data from American College of Obstetricians and Gynecologists: Management of isoimmunization in pregnancy. Technical Bulletin
No. 148. Washington, DC, ACOG, 1990.
Hgb, hemoglobin; RBCs, red blood cells; MCV, mean corpuscular volume.
QID: 27605
Option C (Chronic bronchitis from enlargement of the airspaces distal to the terminal
bronchioles) is incorrect. This is a description of emphysema, such as that that would
occur in a child with alpha1-antitrypsin deficiency.
Option D (Immotile sperm and infertility) is incorrect. Respiratory disorders that are
associated with decreased fertility include cystic fibrosis (CF), due to failure of the normal
formation of mesonephric duct structures, and Kartagener’s syndrome, due to immotile
sperm.
240 Adenoviruses
Kenneth McIntosh
Adenoviruses cause 5-8% of acute respiratory disease in infants, plus a wide array of other
syndromes, including pharyngoconjunctival fever, follicular conjunctivitis, epidemic
keratoconjunctivitis, myocarditis, hemorrhagic cystitis, acute diarrhea, intussusception, and
encephalomyelitis. Adenoviral pneumonia may have serious long-term sequelae, including
bronchiolitis obliterans. Only one third of the 49 serotypes have been associated with
disease.
Etiology.
The Adenoviridae are DNA viruses of intermediate size, which are classified into subgenera
A to F. The virion has an icosahedral coat (capsid) made up of 252 subunits (capsomers) of
which 240 are "hexons" and 12 are "pentons." The hexons have a cross reacting antigen
common to all mammalian adenoviruses. The penton confers type specificity, and antibody
to it is protective. Adenoviruses can also be classified by their characteristic DNA
"fingerprints" on gels after being digested with restriction endonucleases, and this
classification generally conforms to their antigenic types.
All adenovirus types, except types 40 and 41, grow in primary human embryonic kidney
cells, and most grow in HEp-2 or HeLa cells, producing a typical destructive cytopathic
effect. Types 40 and 41 (and other serotypes as well) grow in 293 cells, a line of human
embryonic kidney cells into which certain "early" adenovirus genes have been introduced.
Many adenovirus types, but particularly the common childhood types (1, 2, and 5), are shed
for prolonged periods from both the respiratory and gastrointestinal tracts. These types also
establish low-level and chronic infection of the tonsils and adenoids.
Epidemiology.
Adenoviral infections are distributed worldwide. They occur year-round but are most
prevalent in spring or early summer and again in midwinter in temperate climates. Certain
types tend to occur in epidemics, notably types 4 and 7 in outbreaks of febrile respiratory
disease, types 3, 7, and 21 in severe pneumonia; type 3 in pharyngoconjunctival fever; type
11 in hemorrhagic cystitis; and types 8, 19, and 37 in epidemic keratoconjunctivitis. For
unexplained reasons, adenovirus types 3 and 7 cause severe epidemics of pneumonia in the
children of northern China and Korea, with mortality rates in hospitalized cases of 5-15%.
A 45-year-old man presents to the physician because he has been feeling tired and lethargic for
the last 6 months. He states that he has experienced difficulty concentrating and has become
increasingly indecisive during this time. He reports that his wife has told him on numerous
occasions that he snores loudly. He does not take any regular medications and is allergic to
acetylsalicylic acid. He typically drinks 50 to 70 g of ethanol weekly and smokes a half a pack of
tobacco daily. His vital signs are as follows: blood pressure (BP), 140/100 mm Hg; pulse, 59
beats/minute; temperature, 37.5°C (99.5°F); respirations, 12 breaths/minute; and body mass
index (BMI), 35 kg/m2 (35 lb/in2). Physical examination is within normal limits. What is the most
appropriate next step in the management of this patient?
A. Amitriptyline
B. Electroencephalography (EEG)
C. Lorazepam
D. Polysomnography
E. Sertraline
QID: 33526
1. Intermittent upper airway obstruction that causes snoring and apneic episodes
throughout the night
2. Patients experience decreased rapid eye movement (REM) sleep and wake up tired
3. Evaluation: Overnight sleep study using polysomnography
4. Treatment: Continuous positive airway pressure mask, weight reduction in obese
persons, surgery
A young patient is transported from the scene of an automobile accident to the ER. The patient
complains of pelvic pain. Radiography of the pelvis is ordered and reveals the image shown (see
figure). Which of the following is the most likely diagnosis?
A. Fracture of pubic symphysis
C. Rupture bladder
D. Ruptured urethra
QID: 27344
Option D (Ruptured urethra) is correct. The figure shows abnormal widening of the pubic
symphysis with an associated ruptured urethra and a high-riding bladder.
Option C (Rupture bladder) is incorrect. The bladder is high riding, but there is no
evidence of a rupture. Rupture of the urethra is present.
Option E (Transverse fracture of left femoral head) is incorrect. The left femoral head
has no evidence of fracture. Abnormal widening of the pubic symphysis with an associated
ruptured urethra and a high-riding bladder are seen in this figure.
Urethral Injuries
Urethral injuries are associated with 4% to 14% of all pelvic fractures17,18 and are more
common in cases of bilateral pelvic injuries.19,20 Diagnosis of urethral injuries is made by a
high index of suspicion in the presence of blood at the urethral meatus, inability to urinate,
and/or a palpable full bladder on abdominal examination. When blood is present at the
meatus, retrograde urethrography aids in diagnosis of any urethral injury. In the presence of
minor urethral injury, a catheter can be placed by an experienced urologist with or without
the aid of a cystoscope.21
Urethral injuries are classified as those confined to the posterior urethra (above the
urogenital diaphragm) and to the anterior urethra (below the urogenital diaphragm). Posterior
urethral injuries are further subclassified as type I (urethral stretch), type II (urethral
disruption proximal to the urogenital diaphragm), and type III (proximal and distal disruption
of the urogenital diaphragm).
For treatment of posterior urethral injuries, early endoscopic realignment has become more
accepted as an excellent initial treatment option.22 Realignment of the damaged urethra with
a stented Foley catheter can lead to complete healing of the urethral injury or need for future
endoscopic treatment of developed urethral strictures. If realignment of the damaged urethra
cannot be achieved, then suprapubic catheterization, followed by delayed combined
antegrade and retrograde endoscopic repair or open surgical repair are the potential
treatment options.
page 2296
page 2297
Figure 76-17 Extraperitoneal bladder injury. Contrast agent is extravasated to the space of Retzius
(asterisk) after retrograde filling of the bladder through the indwelling Foley catheter.
In contrast to posterior urethral injuries that are often associated with many other pelvic
injuries, anterior urethral injuries are often isolated and often associated with straddle
injuries. The bulbar urethra is often the site of injury. The best initial treatment modality for
anterior urethral injuries is not well defined; however, most would agree that primary
realignment with Foley catheter, if possible, is the best initial treatment. In cases of severe
anterior urethral injury, a suprapubic catheter may be required, followed by delayed open
surgical repair.23
A 63-year-old man presents to the physician, because he has been experiencing difficulty
obtaining and sustaining an erection while being sexually intimate with his wife. He was
diagnosed with type II diabetes 5 years ago and has been poorly compliant with therapy. He also
has a history of benign prostatic hypertrophy and stable angina. His current medications include
metformin, doxazosin, and isosorbide dinitrate. On examination, he has reduced pinprick
sensation bilaterally in the lower extremities. At the end of the consultation, the patient requests
therapy with sildenafil for his erectile dysfunction. What is the most appropriate advice for this
patient with regard to his current therapeutic regimen?
A. Use of sildenafil and doxazosin is contraindicated
QID: 33239
Sildenafil, the first selective phosphodiesterase type V inhibitor (see also Chs 14, 18), was
being developed for another possible indication and was found incidentally to influence
erectile function. In contrast to intracavernosal vasodilators, it is not sufficient of itself to
cause erection independent of sexual desire, but it enhances the erectile response to sexual
stimulation. It has transformed the treatment of erectile dysfunction.
Mechanism of action
Phosphodiesterase V is the isoform that inactivates cGMP. Nitrergic nerves release nitric
oxide (or a related nitrosothiol), which diffuses into smooth muscle cells where it activates
guanylate cyclase. The resulting increase in cytoplasmic cGMP mediates vasodilation via
activation of protein kinase G (Ch. 14). Consequently, inhibition of phosphodiesterase V
potentiates the effect on penile vascular smooth muscle of endothelium-derived nitric oxide
and of nitrergic nerves that are activated by sexual stimulation. Other vascular beds are also
affected, suggesting other possible uses.**
Peak plasma concentrations occur approximately 30-120 minutes after an oral dose and are
delayed by eating, so it is taken an hour or more before sexual activity. It is given as a single
dose as needed. (For possible long-term indications requiring 24 hour enzyme inhibition, it
needs to be given three times daily.) It is metabolised by the 3A4 isoenzyme of cytochrome
P450, which is induced by carbamazepine, rifampicin and barbiturates and inhibited by
cimetidine, macrolide antibiotics, antifungal imidazolines, some antiviral drugs (such as
ritonavir) and also by grapefruit juice (Ch. 8). These drugs can potentially interact with
sildenafil in consequence. A dramatic pharmacodynamic interaction occurs with organic
nitrates, which work through increasing cGMP (Ch. 17) and are, therefore, markedly
potentiated by sildenafil. Consequenty, concurrent nitrate use contraindicates sildenafil.
A 51-year-old man is brought into the emergency room after three episodes of vomiting bright red
blood. He has a known history of cirrhosis secondary to alcoholic liver disease. On arrival, his
vital signs are as follows: blood pressure, 90/60 mm Hg; pulse, 115 beats/minute; temperature
37.6°C (99.6°F); and respirations, 18 breaths/minute. His abdomen is dull to percussion
throughout. A nasogastric tube is inserted, and bright red blood is found when stomach contents
are aspirated. What is the most appropriate next step in the management of this patient?
A. Balloon tamponade
E. Propranolol
QID: 33275
Option C (Intravenous [IV] normal saline) is correct. This patient has an upper
gastrointestinal bleed, most likely the result of bleeding esophageal varices. However, the
exact cause is unimportant in this case, because the patient is hemodynamically unstable.
He is hypotensive and tachycardic and thus requires hemodynamic resuscitation. The option
of IV normal saline meets this requirement.
Option B (Endoscopic band ligation) is incorrect. If medical therapy with octreotide fails,
endoscopic band ligation is the next step in the management of bleeding esophageal
varices.
Option D (Octreotide) is incorrect. Were this patient hemodynamically stable, the use of
octreotide is considered the first step in the management of bleeding esophageal varices.
Option E (Propranolol) is incorrect. Propranolol and other nonselective beta blockers are
used as prophylaxis against bleeding esophageal varices. There is no role acutely,
especially not in hypotensive patients.
The first step in the evaluation and therapy for the patient with acute GI hemorrhage is to
determine the severity of blood loss. Vital signs should be recorded immediately. If the
systolic blood pressure drops more than 10 mm Hg and/or the pulse increases more than 10
beats per minute as the patient changes positions from supine to standing, it is likely the
patient has lost at least 800 mL (15%) of circulating blood volume. Hypotension, tachycardia,
tachypnea, and mental status changes in the setting of acute GI hemorrhage suggest at
least a 1500-mL (30%) loss of circulating blood volume.
The goal of resuscitation is to restore the normal circulatory volume. Initially, at least two
large-bore intravenous catheters are used to administer isotonic solutions (e.g., lactated
Ringer's solution or 0.9% NaCl), and blood products if indicated. If the patient is in shock, a
central venous access should be established. The amount of blood products to be
transfused must be individualized. Transfusions of packed red blood cells are provided to
prevent complications (e.g., angina, congestive heart failure, stroke) of acute blood loss.
Therefore, the need for blood transfusion depends on multiple factors, including the patient's
age, overall health, and response of vital signs to initial resuscitation. In view of the risks of
blood transfusion, it is not appropriate simply to transfuse until an arbitrary hematocrit is
achieved. If coagulation studies are abnormal, as commonly observed in cirrhotic patients,
fresh-frozen plasma and/or platelets may be required to control ongoing hemorrhage.
Opinions differ regarding the use of nasogastric lavage in preparation for endoscopic
examination, although aspiration of gastric blood may be particularly important in patients
with liver cirrhosis, because blood in the GI tract may precipitate hepatic encephalopathy.
Commonly involves stomach and small bowel Surgery for localized disease
as well
Colon cancer Change in bowel habit, anemia, weight loss Surgery
Colon polyp Usually asymptomatic Endoscopic or surgical removal
Ischemic colitis Typically elderly patients Supportive (self-limited)
History of vascular disease
May present with abdominal pain
Meckel's diverticulum Painless hematochezia in young patient Surgery
Located at distal ileum
Hemorrhoids Rectal bleeding associated with bowel Supportive
movement
From Cecil Essentials of Medicine 6E by Andreoli et al
Figure 32-2 Approach to the patient with acute gastrointestinal bleeding. EGD = esophagogastroduodenoscopy.
The medical intern on call is summoned to the bedside of a 70-year-old woman who is
complaining of chest pain. She is postoperative day 2 after a hysterectomy for endometrial
cancer. A 12-lead electrocardiogram (ECG) is ordered, and the tracing is shown (see figure).
Based only on the evidence presented, which of the following is the most likely diagnosis?
A. Acute pericarditis
Option A (Acute pericarditis) is incorrect. The ECG in acute pericarditis generally shows
diffuse ST-segment elevation. This is the classic ECG for an acute pulmonary embolism,
showing an S wave in lead I, a Q wave in lead III, and an inverted T wave in lead III (S1, Q3,
T3 pattern).
Option D (Left bundle branch block) is incorrect. There is an incomplete right bundle
branch block pattern (an R wave in aVR and V1 and an S wave in V6). The ECG showing left
bundle branch block would typically show QRS duration greater than 0.12 s; broad notched
R waves with ST depression in leads I, aVL, and V6; and broad QS waves in V1 to V3. This is
the classic ECG for an acute pulmonary embolism, showing an S wave in lead I, a Q wave in
lead III and an inverted T wave in lead III (S1, Q3, T3 pattern).
ELECTROCARDIOGRAPHY.
page 562
page 563
VARIABLE POINTS
DVT symptoms/signs* 3.0
PE as or more likely
†
3.0
HR >100 beats/min 1.5
Immobilization/surgery‡ 1.5
Previous DVT or PE 1.5
Hemoptysis 1.0
Malignancy 1.0
TOTAL SCORE PRETEST PROBABILITY§
<2.0 Low
2.0 to 6.0 Moderate
>6.0 High
*Including objectively measured leg swelling and pain with palpation in the deep vein region.
†
PE as likely or more likely than an alternative diagnosis. Physicians were told to use clinical information,
along with chest radiography, electrocardiography, and laboratory tests.
‡
If in previous 4 weeks.
§
Of the 437 patients with a negative D-dimer result (by the SimpliRED assay) and low clinical probability,
only one developed PE during follow-up; thus, the negative predictive value for the combined strategy of
using the clinical model with D-dimer testing in these patients was 99.5%.
DVT =deep venous thrombosis, PE =pulmonary embolism, HR =heart rate.
From Wells PS, Anderson DR, Rodger M, et al: Excluding pulmonary embolism at the bedside without
diagnostic imaging: Management of patients with suspected pulmonary embolism presenting to the
emergency department by using a simple clinical model and D-dimer. Ann Intern Med 2001;135:98.
Electrocardiographic findings, which are present in the majority of patients with acute PE,
include ST-segment abnormalities, T-wave changes, and left or right axis deviation. Only
one third of patients with massive or submassive emboli have manifestations of acute cor
pulmonale such as the S1-Q3-T3 pattern, right bundle branch block, P-wave pulmonale, or
right axis deviation. All of these findings are also nonspecific. The utility of
electrocardiography in suspected acute PE is derived more from its ability to establish or
exclude alternative diagnoses, such as acute myocardial infarction, rather than diagnosing or
excluding PE.
CHEST RADIOGRAPHY.
The chest radiograph is often abnormal in patients with acute PE, but it is nearly always
nonspecific. Common radiographic findings include pleural effusion, atelectasis, pulmonary
infiltrates, and mild elevation of a hemidiaphragm. Classic findings of pulmonary infarction,
such as Hampton's hump or decreased vascularity (Westermark's sign), are suggestive of
the diagnosis, but they are infrequent. A normal chest radiograph in the setting of dyspnea
and hypoxemia without evidence of bronchospasm or anatomic cardiac shunt is strongly
suggestive of PE. Under most circumstances, however, the chest radiograph cannot be used
for conclusive diagnosis or exclusion. Although the radiograph may exclude other processes,
such as pneumonia, pneumothorax, or rib fracture, which may cause symptoms similar to
acute PE, PE may frequently coexist with other underlying heart or lung diseases.
Symptoms, signs, radiographic findings, electrocardiography, and the plasma D-dimer
measurement cannot be considered diagnostic of PE or DVT. When these entities are
suspected, further evaluation with noninvasive or invasive testing is necessary.
A 27-year-old woman develops the pictured lesion 2 days after wearing a new pair of earrings.
What is the most likely diagnosis?
B. Atopic dermatitis
D. Nummular dermatitis
E. Seborrheic dermatitis
QID: 33610
Option A (Allergic contact dermatitis) is correct. This patient has allergic contact
dermatitis, most likely the result of nickel in her new earrings. Allergic contact dermatitis
should be suspected when there is exposure to an allergen and a reaction develops at least
48 hours later. As pictured, there is an erythematous base and a slightly white scale with a
defined border.
Option C (Irritant contact dermatitis) is incorrect. Irritant contact dermatitis is the result of
direct toxic injury to the skin and will occur in any individual given sufficient exposure. This is
in contrast to allergic contact dermatitis, where individuals with atopy are more likely to
develop it. Irritant contact dermatitis is differentiated from allergic contact dermatitis by the
acute speed of the reaction (less than 12 hours usually), a very sharp border without spread
and an absence of papules in the acute phase.
BASIC INFORMATION
DEFINITION
ETIOLOGY
DIAGNOSIS
DIFFERENTIAL DIAGNOSIS
• Impetigo
• Lichen simplex chronicus
• Atopic dermatitis
• Nummular eczema
• Seborrheic dermatitis
• Psoriasis
• Scabies
WORKUP
• Medical history: gradual onset vs. rapid onset, number of exposures, clinical
presentation, occupational history
• Physical examination: contact dermatitis in the neck may be caused by necklaces,
perfumes, after-shave lotion; involvement of the axillae is often secondary to
deodorants, clothing; face involvement can occur with cosmetics, airborne allergens,
aftershave lotion
LABORATORY TESTS
A. All-trans-retinoic acid
C. Imatinib
E. Splenectomy
QID: 27241
Option C (Imatinib) is correct. The cellular appearance of the peripheral smear and bone
marrow aspirate is characteristic of chronic myelogenous leukemia. The presence of the
Philadelphia chromosome (t9;22) make this diagnosis virtually certain. Protein-tyrosine
kinase inhibitors such as STI571 or imatinib mesylate that inhibit the bcr-abl tyrosine kinase
have revolutionized the treatment of chronic myelogenous leukemia. Recent studies show
imatinib is superior to interferon alpha plus low-dose cytarabine as first-line therapy in newly
diagnosed, chronic-phase CML.
Option A (All-trans-retinoic acid) is incorrect. Transretinoic acid can effectively induce
most newly diagnosed acute promyelocytic leukemia patients into remission, without the
myelosuppressive effects of chemotherapy. The cellular appearance of the peripheral smear
and bone marrow aspire are characteristic for chronic myelogenous leukemia. The presence
of the Philadelphia chromosome (t9;22) make this diagnosis virtually certain. This is not the
treatment for CML.
Imatinib
TREATMENT
Taken from Practical Guide to the Care of the Medical Patient 6E by Ferri
A 22-year-old G1P0 at 34 weeks’ gestation is involved in a motor vehicle accident. She was not
wearing her seat belt and as she jolted forward, her abdomen hit the steering wheel. She is
brought to labor and delivery for assessment. She is observed for 2 hours. The maternal status is
stable. Fetal heart trace is reassuring. No uterine contractions are noted on external uterine
monitoring. There is no evidence of vaginal bleeding. The ultrasound examination does not
detect a placental abruption. Which of the following is the most appropriate next step in patient
care?
A. Administer betamethasone
D. Induce labor
Option E (Obtain a blood type and rhesus [Rh] status) is correct. A pregnant woman
involved in a motor vehicle accident in which abdominal trauma has occurred should have
blood type and Rh status checked. Those women who are Rh-negative should be given
RhoGAM.
Option C (Discharge home on bed rest) is incorrect. Before being discharged home, the
patient should have her blood type and Rh status checked because those women who are
Rh-negative should receive RhoGAM.
Option D (Induce labor) is incorrect. There is no indication to deliver this patient in that
both she and the fetus are stable.
Because the greatest risk for fetal-to-maternal hemorrhage occurs during labor and
delivery, Rh immune globulin was initially administered only during the immediate
postpartum period. This resulted in a 1% to 2% failure rate, thought to be due to exposure
of the mother to fetal red blood cells during the antepartum period. The indications for the
use of Rh immune globulin have therefore been broadened to include any antepartum
event (such as amniocentesis) that may increase the risk of transplacental
hemorrhage. The routine prophylactic administration of Rh immune globulin at 28
weeks' gestation is now the standard of care. Despite adherence to this suggested Rh
immune globulin protocol, 0.27% of primiparous Rh-negative patients still become
sensitized.
Taken from Essentials of Obstetrics & Gynecology 4E by Hacker et al
Fetal erythrocytes have been demonstrated in the maternal circulation following rupture of a
tubal pregnancy. Consequently, Rh immune globulin should be given to an Rh-negative
woman with an ectopic pregnancy. Because chorionic villi in gestational trophoblastic
disease are avascular and are devoid of fetal erythrocytes, Rh immune globulin is
probably not necessary following molar pregnancy. At least one case of sensitization
following a molar pregnancy, however, has been reported.
A 34-year-old gravida IV para III female at 37 weeks of gestation is brought to the emergency
room following the sudden onset of abdominal pain and vaginal bleeding 2 hours ago. The pain
has been constant from onset. She has also experienced frequent, strong contractions. She has
been otherwise well, abstinent from intercourse for the past 2 months, and her previous medical
and obstetric history are unremarkable. Her vital signs are blood pressure (BP), 100/65 mm Hg;
pulse, 110 beats/minute; temperature, 37.7°C (99.8°F); and respirations, 14 breaths/minute. The
uterus is painful and rigid. Speculum examination demonstrates mild vaginal bleeding. Fetal
heart rate tracing is not reassuring. What is the most likely diagnosis?
A. Abruptio placenta
B. Placenta previa
C. Spontaneous abortion
D. Uterine rupture
E. Vasa previa
QID: 33070
Option A (Abruptio placenta) is correct. This patient is demonstrating the classic signs of
placental abruption: painful, abrupt vaginal bleeding associated with uterine contractions and
nonreassuring fetal heart tracing. The pain is constant, and the uterus is firm because of
tetanic contractions. Vaginal bleeding can be highly variable and does not correlate well with
the severity of abruption.
Option B (Placenta previa) is incorrect. Placenta previa is classically bright red painless
bleeding that is not as rapid in onset as abruption. The uterus is usually soft.
Option D (Uterine rupture) is incorrect. Uterine rupture is painful third trimester bleeding,
but is very rare. Unlike abruptio placenta, it is associated with constant heavy vaginal
bleeding. A classic presentation on the examination is a fetus that is at a +2 station that
suddenly retracts into the -1 position.
Option E (Vasa previa) is incorrect. Vasa previa has a classic triad of membrane rupture
followed by vaginal bleeding and then fetal bradycardia. Vasa previa is bleeding from fetal
vessels and is diagnosed using the Apt test.
ABRUPTIO PLACENTAE
• Maternal hypertension
• Placental abruption in a prior pregnancy
• Trauma
• Polyhydramnios with rapid decompression
• Premature rupture of membranes
• Short umbilical cord
• Tobacco use
• Folate deficiency
A 55-year-old female is brought to the emergency room with a sudden onset of severe chest,
back, and abdominal pain that began 20 minutes ago. The pain is described as being very sharp
with a tearing-like character. Her previous medical history is notable for hypertension treated with
hydrochlorothiazide. She does not smoke tobacco or drink alcohol.
On examination, her blood pressure is 145/95 mm Hg in the right arm and 119/75 mm Hg in the
left arm; pulse, 105 beats/minute; temperature, 37.3°C (99.1°F); and respirations, 17
breaths/minute. A chest X-ray (CXR) demonstrates a widened mediastinum, and
electrocardiography (ECG) reveals nonspecific ST and T wave changes. What is the most
appropriate next step in the management of this patient?
A. Aortography
B. Emergent surgery
C. Intravenous labetalol
QID: 33208
Option C (Intravenous labetalol) is correct. Ripping chest pain that radiates into the back
and stomach, combined with a pulse deficit, previous history of hypertension and widened
mediastinum on chest X-ray (CXR) should be considered an aortic dissection until proven
otherwise. As soon as aortic dissection is suspected, immediate intravenous (IV) beta-
blocker therapy should be started to reduce the pulse and blood pressure of the patient in an
attempt to limit the severity of the dissection.
• Chest x-ray may show widened mediastinum (62%) and displacement of aortic
intimal calcium.
• Transesophageal echocardiography (TEE) is study of choice in unstable patients, but
operator dependent.
• MRI has the highest sensitivity and specificity but limited availability; not suitable for
unstable patients; contraindicated with pacemakers, metal devices.
• Helical CT is least operator dependent, involves intravenous contrast.
• TEE, MRI, helical CT are imaging modalities of choice. Sensitivities (98% to 100%)
and specificities (95% to 98%) nearly equal in skilled hands. Test of choice depends
on clinical circumstances and availability.
• With medium or high pretest probability, a second diagnostic test should be done if
the first is negative.
• Transthoracic echocardiography has poor sensitivity
• Aortography rarely done now.
TREATMENT
ACUTE GENERAL Rx
A 3260-g (7 lb 2 oz) male infant is born at 38 weeks’ gestation to a healthy, 26-year-old woman.
The only abnormality noted during the course of the pregnancy was that the woman’s fundal
height measured consistently “large for date” of gestation. The infant is delivered via
spontaneous, vaginal delivery and has Apgar scores of 9 and 10 at one and five minutes,
respectively. On the first day postpartum, the mother calls for help as she is feeding the infant;
the infant ejects formula from the nose and mouth and then begins to cough, choke, and turn
blue as soon as the mother begins to feed him. Which of the following is the most likely reason
for the mother’s abnormal uterine fundal height during the pregnancy?
QID: 27597
Option D (Fetal macrosomia, which accounted for large uterine fundal size) is
incorrect. At 3260 g (7 lb 2 oz), this infant is not considered macrosomic and would not
have been so as a fetus.
Option E (Hyperglycemia, causing the fetus to produce more urine than normal) is
incorrect. Maternal hyperglycemia does cause polyuria in the fetus, much as it does in
diabetic individuals. However, the question stem states that the mother is healthy. Also, a
fetus continually exposed to high blood glucose levels is likely to develop either macrosomia
or intrauterine growth restriction.
Examination of the pregnant uterus is vital. The fundal height is measured in centimeters
from the symphysis pubis to the top of the uterus. Serial measurements over the course of
pregnancy provide an excellent assessment of fetal growth with a rough approximation
between centimeters and weeks, gestation from 18-34 weeks in a patient with a normal body
habitus. Fetal heart tones should be auscultated, and fetal position and estimated weight
determined during the last trimester. Lastly, uterine contractions can be easily palpated
during a routine exam and, if frequent and many weeks before term, may point to the
possibility of preterm labor.
Diagnosis.
Management.
Figure 300-1 Diagrams of the five most commonly encountered forms of esophageal atresia and
tracheoesophageal fistula, shown in order of frequency.
A 72-year-old man with a history of smoking 60 packs per year comes to the emergency room
with increased shortness of breath. He usually can increase his oxygen and take more frequent
puffs on his metered-dose inhalers, but this has not helped him on this occasion. His oxygen
saturation is 90% on 3 L. He has decreased breath sounds, which are more obvious on the left
side. The remainder of his examination is only notable for tachycardia. A radiograph of the chest
is shown (see figure). Which of the following is the most appropriate next step in management?
D. Sputum cultures
QID: 27435
Option A (100% O2 via face mask) is correct. The first treatment of pneumothorax is 100%
oxygen via face mask.
Option B (CT scan of the thorax) is incorrect. A CT scan of the thorax may be useful to
determine the degree of lung collapse, but the first treatment strategy would be
administration of oxygen.
Option D (Sputum cultures) is incorrect. Sputum cultures are not indicated in this case;
the initial treatment of pneumothorax is administration of oxygen.
A 32-year-old man presents to the physician, because he noticed a large amount of blood in the
toilet bowl whist defecating this morning. The blood was bright red in color and located primarily
in the bowl. The stool was brown-colored and did not have an unusual smell. He denies any
abdominal pain, nausea, vomiting, or passage of mucus. Two years ago, he experienced an
isolated episode of vomiting bright red blood. He is currently employed as an aircraft mechanic,
smokes 1 pack of cigarettes a day for the last 10 years, and drinks 30 to 40 g of ethanol weekly.
His father and brother have also had several episodes of bright red blood per rectum, but he
denies any knowledge of a family history of colon cancer. On examination, his vital signs are as
follows: blood pressure, 118/75 mm Hg; pulse, 77 beats/minute; temperature, 37.6°C (99.7°F);
and respirations, 13 breaths/minute. There are multiple telangiectasias on the face, inside the
nose, on the oral mucosa, and upper thorax. The abdomen is tympanic to percussion, and
normal bowel sounds are auscultated. It is soft and nontender to palpation, and there is no
organomegaly or masses. Rectal examination reveals a normal anal reflex and bright red blood
on the glove. There is no mucus or palpable hemorrhoids. What is the most likely diagnosis?
A. Familial colonic polyposis syndrome
B. Gardner syndrome
C. Meckel diverticulum
D. Osler-Rendu-Weber syndrome
E. Peutz-Jeghers syndrome
QID: 33268
The skin examination can be helpful for suggesting a potential source if certain stigmata are
present. Lymphadenopathy or abdominal masses may suggest sources for intra-abdominal
pathology (Table 5-1).
A 47-year-old man presents to the emergency room 30 minutes after the sudden onset of chest
pain is located more prominently over the left hemithorax and does not radiate elsewhere. It is
sharp in quality, notably more severe on inspiration, and does not change with position. He has
been previously well and is not on any current medications. His blood pressure is 130/85 mm Hg;
pulse, 107 beats/minute; temperature, 38.6°C (101.4°F); and respirations, 24 breaths/minute.
Auscultation reveals a fourth heart sound and rales over the left posterior hemithorax.
Electrocardiography (ECG) demonstrates a right bundle branch block that was not present on his
previous electrocardiogram performed 1 month ago. What is the most appropriate next step in
the management of this patient?
A. Echocardiography
B. Leg ultrasonography
C. Nebulized salbutamol
D. Pulmonary arteriography
Option E (Ventilation-perfusion [V/Q] scan) is correct. This patient has a likely pulmonary
embolism (PE). The diagnosis is suspected because of the finding of pleuritic chest pain
(pain that changes with respiration), dyspnea, tachypnea, tachycardia, and new onset right
bundle branch block. Patients can also be febrile with a PE. At present, ventilation-perfusion
scan (V/Q scan) or spiral computed tomography (CT) scan are considered the first
diagnostic steps in patients with an intermediate to high probability of having a pulmonary
embolism. If the options had included anticoagulant therapy, that would have been the first
step in the management of this patient, because of the reasonably high probability of this
patient having a PE.
Clinical features
Dyspnea is the most frequent symptom, and tachypnea is the most frequent sign in
pulmonary embolism (PE). Dyspnea, syncope, hypotension, or cyanosis indicates a massive
PE, while pleuritic pain, cough, or hemoptysis often suggests a small embolism located near
the pleura. On examination, young or previously healthy individuals may simply appear
anxious but otherwise seem well, even with a large PE.
Many drugs interact with warfarin to enhance or diminish its effect. Ask
the patient to report any changes in treatment.
Investigations
Figure 5.19 Ventilation:perfusion scan following pulmonary embolus. (Courtesy of Ivor Jones and the Nuclear Medicine staff,
Derriford Hospital, Plymouth.)
A 75-year-old female presents to the emergency room with a 5-day history of intermittent nausea
and vomiting. She has also noticed increasing abdominal distension during this period. She has
been otherwise well and does not take any medications. Her previous medical and surgical
history is unremarkable. On examination, her vital signs are blood pressure (BP), 110/65 mm Hg;
pulse, 98 beats/minute; temperature, 38.4°C (101.1°F); and respirations, 14 breaths/minute.
Overall, she appears significantly dehydrated and is not jaundiced. Her abdomen is soft, but
distended and diffusely tender to deep palpation. Increased, high-pitched bowel sounds are
noted throughout the abdomen. Rectal examination is normal. Upright abdominal radiograph
demonstrates distended loops of small bowel with multiple air-fluid levels and air in the biliary
tree. What is the most likely diagnosis?
B. Cholelithiasis
C. Gallstone ileus
D. Hepatic adenoma
E. Intussusception
QID: 33074
Option C (Gallstone ileus) is correct. This patient has a gallstone ileus, where a large
gallstone has travelled into the ileum via a biliary-enteric fistula causing obstruction.
Gallstone ileus accounts for 25% of nonstrangulated small bowel obstruction in
patients older than 65 years of age. Classically, the symptoms of gallstone ileus are
subacute over a period of 3 to 10 days while the gallstone travels through the small bowel
until becoming permanently lodged in the ileum. Abdominal radiograph is the first and best
investigation for gallstone ileus and demonstrates the signs of small bowel obstruction
combined with pneumobilia (air in the biliary tree).
Option A (Adhesive small-bowel obstruction) is incorrect. This patient has never had
abdominal surgery and would therefore be unlikely to have an adhesion resulting in small-
bowel obstruction.
Option B (Cholelithiasis) is incorrect. As gallstones and cholecystitis are likely the inciting
event for creating the biliary-enteric fistula, this patient likely has cholelithiasis. However, the
cholelithiasis is a likely incidental diagnosis that is not responsible for her present illness.
A biotechnology company is seeking to develop a new test for the detection of diabetes using an
optical finger probe similar to a pulse oximeter. They screen 1000 random individuals using the
current diagnostic criteria of having two fasting blood glucose readings more than126 mg/dL.
They compare the results obtained with their new test to those of a standard blood test. Their
results are as follows:
Healthy Diabetes
Positive optical test 50 90
A. 10%
B. 64%
C. 90%
D. 94%
E. 99%
QID: 33417
Option D (94%) is correct. Accuracy is defined as the proportion of individuals who are
correctly diagnosed by the screening test. Using the equation (true positive [TP] + true
negative [TN])/1000, we get (850 + 90)/1000 = 94%.
Option A (10%) is incorrect. The prevalence of diabetes found in this random population of
individuals can be calculated as the TPs plus the false negatives (FNs) divided by 1000
individuals. This calculation yields a 10% prevalence of diabetes.
Option B (64%) is incorrect. Positive predictive value is the proportion of people with a
positive test who actually have the condition. Using the formula TP/(TP+ false positive [FP]),
we get 90/(90 + 50) = 64%.
Option C (90%) is incorrect. Sensitivity is defined as the proportion of individuals who have
the condition that are correctly identified by the screening test. Using the formula TP/(TP+
FN), we get 90/(90+10) = 90%.
Option E (99%) is incorrect. The negative predictive value is how likely a negative test is
truly negative. Using the formula TN/(TN+ FN), we get 850/(850 + 10) = 99%.
The validity of a test is defined as its ability to distinguish between who has a disease and
who does not. Validity has two components: sensitivity and specificity. The sensitivity of the
test is defined as the ability of the test to identify correctly those who have the disease. The
specificity of the test is defined as the ability of the test to identify correctly those who do not
have the disease.
Suppose we have a hypothetical population of 1,000 people, of whom 100 have a certain
disease and 900 do not. A test is available that can yield either positive or negative results.
We want to use this test to try to distinguish persons who have the disease from those who
do not. The results obtained by applying the test to this population of 1,000 people are
shown in Table 5-1.
Example: Assume a population of 1,000 people, of whom 100 have a disease and 900 do not have the disease
Screening Test to Identify the 100 People with the Disease
True Characteristics in the Population
Results of Screening Disease No Disease Total
Positive 80 100 180
Negative 20 800 820
Total 100 900 1,000
How good was the test? First, how good was the test in correctly identifying those who had
the disease? Table 5-1 indicates that of the 100 people with the disease, 80 were correctly
identified as "positive" by the test, and a positive identification was missed in 20. Thus, the
sensitivity of the test, which is defined as the proportion of diseased people who were
correctly identified as "positive" by the test, is 80/100 or 80%.
Second, how good was the test in correctly identifying those who did not have the disease?
Looking again at Table 5-1, of the 900 people who did not have the disease, the test
correctly identified 800 as "negative." The specificity of the test, which is defined as the
proportion of nondiseased people who are correctly identified as negative by the test, is
therefore 800/900 or 89%.
Note that to calculate the sensitivity and specificity of a test, we must know who "really" has
the disease and who does not from another source than the test we are using. We are in fact
comparing our test results with some "gold standard"-an external source of "truth" regarding
the disease status of each individual in the population. Sometimes this truth may be the
result of another test that has been in use, and sometimes it is the result of a more definitive,
and often more invasive, test (e.g., cardiac catheterization or tissue biopsy). However, in real
life, when we use a test to identify diseased and nondiseased persons in a population, we
clearly do not know who has the disease and who does not. (If this were already established,
testing would be pointless.) But to quantitatively assess the sensitivity and specificity of a
test, we must have another source of truth with which to compare the test results.
A 32-year-old gravida I, para 0 female presents to the labor and delivery ward following a large
discharge of clear fluid from her vagina 1 hour ago. Her pregnancy thus far as been
unremarkable except for positive third-trimester vaginal group B Streptococcus cultures. On route
to the hospital, she begins to have regular contractions and progresses to full cervical dilation
after 10 hours. Intrapartum penicillin is administered. A compound foot presentation is discovered
and immediate cesarean section is undertaken. A 3.0-kg (6.6 lb) male neonate is delivered with 1
and 5 minute APGAR scores of 9 and 9, respectively. At 1 hour of age, the infant is heard
grunting on expiration. He is tachypneic and has mild nasal flaring. He is tachycardic, but S1 and
S2 heart sounds are auscultated and there is no murmur. The abdomen is soft, without
organomegaly and there are no masses palpated. There are no petechiae or purpura and the
skin appears pale pink. A chest X-ray (CXR) reveals prominent perihilar streaking and fluid within
the horizontal fissure. During the physical examination, he appears to become cyanosed and
supplemental oxygen is commenced, resulting in a dramatic improvement in his symptoms. What
is the most likely diagnosis?
A. Neonatal sepsis
C. Pneumothorax
QID: 33092
7. In addition to RDS, what are other neonatal diffuse lung diseases? How might they
be differentiated clinically?
Other neonatal diffuse lung diseases include transient tachypnea of the newborn (TTN),
congestive heart failure (CHF), neonatal pneumonia, and meconium aspiration. Neonatal
pneumonia may occur in the preterm or term infant. Meconium aspiration and TTN are
generally diseases of term infants, and CHF and pneumonia may occur in either.
Figure 57-1 Frontal chest radiograph of a premature infant with diffuse ground-glass lung disease and low lung volumes, which
is indicative of RDS. (Courtesy Richard Markowitz, Children's Hospital of Philadelphia.)
A 10-year-old boy is brought to the emergency department by his parents following the
appearance of a rash 6 hours earlier. The parents report that the boy has been sick with a sore
throat and non-productive cough for the last three days, but did not seek treatment as he was
eating well and otherwise active. However this morning the boy began to complain of malaise,
general muscle aches, and a headache, and his parents measured an oral temperature of 39°C
(102.2°F). Several hours later the boy suddenly developed a rash, which is described as red
spots. The lesions appeared at the same time all over his body and are progressively worsening.
There is no history of sick contacts or recent travel. The boy has no other medical problems and
no known allergies. Vital signs are significant for hypotension, tachycardia, and a temperature of
40°C (104°F). The patient appears anxious and restless but is alert. Physical examination is
significant for a diffuse, erythematous papular rash, shown. The rash is present on the trunk and
extremities, but not the face, palms, and soles. There is no blanching of the lesions when
pressure is applied with a glass slide. No nuchal rigidity is appreciated, and Kernig’s and
Brudzinski’s signs are negative. Blood tests shown. What is the most appropriate treatment at
this time?
WBC (white blood cell count) Hb (hemoglobin) Hct (hematocrit) Platelets
14 15 45 100
B. Penicillin G
C. Plasma exchange
D. Prednisone
E. Supportive therapy
QID: 47967
Option B (Penicillin G) is correct. This is the 1st line treatment for meningococcemia, which
is a systemic infection by the bacteria Neisseria meningitides. It is contracted by inhalation of
airborne droplets of infected nasopharyngeal secretions, which then spread hematogenously
and can result in meningitis and fulminant meningococcemia. Patients usually present with
an antecedent upper respiratory infection, which is followed by high fever, chills, myalgias,
and other constitutional symptoms. Changes in mental status may also occur. The hallmark
rash reflects systemic endothelial damage and diffuse intravascular coagulation (DIC), and is
characterized by the sudden appearance of widespread purpuric lesions with necrotic
centers that do not blanche under pressure. This patient’s history, appearance of the rash,
and lab values are strongly consistent with this diagnosis and empirical antibiotic treatment
should be immediately initiated.
Option A (Doxycycline) is incorrect. This is the treatment of choice for Rocky Mountain
spotted fever, which is a tick-borne disease caused by the organism Rickettsia rickettsii.
Patients classically present with a history of tick exposure, fever, and a petechial rash that
begins on the palms and soles and spreads centripetally. This is an unlikely diagnosis in this
patient given the lack of history of tick exposure and absence of lesions on the palms and
soles. Furthermore, the lesions illustrated are purpuric, which coupled with an antecedent
upper respiratory infection and evidence of consumptive coagulopathy is strongly suggestive
of fulminant meningococcemia.
Neisseria meningitidis
1. Classification
a. Serogroups based on the polysaccharide capsule; most common = A, B, C,
Y, and W135
b. Serotypes based on the outer membrane protein
c. Immunotypes based on LPS
2. Pathogenesis
a. Neisseria meningitidis enters the respiratory tract, invades mucous
membranes, and spreads via the bloodstream.
b. Antiphagocytic capsule is important for virulence.
c. Released endotoxin induces fever and increases vascular permeability,
potentially leading to shock and petechiae (capillary leakage in skin).
3. Meningococcal diseases
○ Meningococcal infection is most common in children younger than 5 years
of age and those with deficiency of terminal complement components
(C5-C9).
a. Meningitis: acute onset of fever, headache, and stiff neck
b. Acute meningococcemia: septicemia with or without meningitis
Characterized by fever, shock, and generalized hemorrhage ranging
from petechiae to purpura
Can be rapidly fatal (mortality rate of 25% or higher) if not treated
promptly
c. Waterhouse-Friderichsen syndrome: complication of meningococcemia
Marked by overwhelming disseminated intravascular coagulation
and bilateral hemorrhagic adrenal necrosis with septic shock, acute
hypotension, tachycardia, and petechiae
d. Chronic meningococcemia: milder disease characterized by persistent (for
weeks) bacteremia
Low-grade fever, arthritis, and petechial skin lesions
e. Mild febrile disease with pharyngitis, pneumonia, arthritis, or urethritis
Transmission of N. meningitidis
a. Inhalation of aerosol droplets (often from asymptomatic nasopharyngeal
carriers)
b. Person-to-person spread from infected persons
Prevention
a. Breast-feeding infants for the first 6 months of life
b. Active immunization of children older than 2 years of age with a polyvalent
conjugate, anticapsular vaccine (not effective against serogroup B)
c. Postexposure prophylaxis with rifampin, quinolones, or sulfonamides (only
if the organism is proven susceptible)
Treatment
a. Penicillin is the drug of choice. Alternatives include broad-spectrum
cephalosporins (ceftriaxone), chloramphenicol, and sulfonamides (if
susceptible).
b. Antibiotics must be secreted from mucosa to eliminate carrier state.
A 46-year-old man has left lower abdominal pain, and a CT scan demonstrates sigmoid colon
thickening and mesenteric “stranding.” After three days of IV antibiotics and inpatient
hospitalization for this episode of presumed acute diverticulitis, he is sent home on oral
antibiotics. He develops profuse watery diarrhea and crampy abdominal pain over the
subsequent two to three days. Which of the following is the most likely diagnosis?
A. Antibiotic-associated colitis
B. Ischemic colitis
C. Missed appendicitis
E. Recurrent diverticulitis
QID: 27701
Option A (Antibiotic-associated colitis) is correct. Clostridium difficile colitis is common
after antibiotic therapy. It can be treated by oral antibiotics in most cases.
Option C (Missed appendicitis) is incorrect. The initial CT findings and location of the
pain are consistent with the diagnosis of acute diverticulitis. The watery diarrhea and
abdominal symptoms do not call the original diagnosis into doubt but rather suggest
evolution of antibiotic-associated enterocolitis.
Option D (Partial colonic obstruction) is incorrect. Large bowel obstruction may occur
chronically if a stricture develops as a result of repeated attacks of inflammation or in the
setting of untreated acute inflammation. However, in this case, the resolution of the toxic
symptoms and the evolution of the watery diarrhea during antibiotic therapy make antibiotic-
associated enterocolitis more likely.
Option E (Recurrent diverticulitis) is incorrect. This is not likely if fevers and focal
tenderness have not recurred.
C. difficile is part of the normal intestinal flora in a small number of healthy people and
hospitalized patients. The disease develops in people taking antibiotics, because the drugs
alter the normal enteric flora, either permitting the overgrowth of these relatively resistant
organisms or making the patient more susceptible to the exogenous acquisition of C. difficile.
The disease occurs if the organisms proliferate in the colon and produce their toxins.
C. difficile produces two toxins (Table 40-5), an enterotoxin (toxin A) and a cytotoxin (toxin
B). The enterotoxin is chemotactic for neutrophils, stimulating the infiltration of
polymorphonuclear neutrophils into the ileum with release of cytokines. Toxin A also
produces a cytopathic effect, resulting in disruption of the tight cell-cell junction, increased
permeability of the intestinal wall, and subsequent diarrhea. The cytotoxin causes actin to
depolymerize, with the resultant destruction of the cellular cytoskeleton both in vivo and in
vitro. Although both toxins appear to interact synergistically in the pathogenesis of disease,
enterotoxin A-negative isolates can still produce disease. Additionally, production of one or
both toxins does not appear to be sufficient alone for disease (e.g., carriage of C. difficile
and high levels of toxins are common in young children while disease is rare). Bacterial
"surface layer proteins" (SLPs) are important for the binding of C. difficile to the intestinal
epithelium, leading to localized production of toxins and subsequent tissue damage. Other
C. difficile virulence factors are summarized in Table 40-5.
A 51-year-old man is brought into the emergency room after three episodes of vomiting bright red
blood. He has a known history of cirrhosis secondary to alcoholic liver disease. On arrival, his
vital signs are as follows: blood pressure, 90/60 mm Hg; pulse, 115 beats/minute; temperature
37.6°C (99.6°F); and respirations, 18 breaths/minute. His abdomen is dull to percussion
throughout. A nasogastric tube is inserted, and bright red blood is found when stomach contents
are aspirated. What is the most appropriate next step in the management of this patient?
A. Balloon tamponade
D. Octreotide
E. Propranolol
QID: 33275
Option C (Intravenous [IV] normal saline) is correct. This patient has an upper
gastrointestinal bleed, most likely the result of bleeding esophageal varices. However, the
exact cause is unimportant in this case, because the patient is hemodynamically unstable.
He is hypotensive and tachycardic and thus requires hemodynamic resuscitation. The option
of IV normal saline meets this requirement.
Option B (Endoscopic band ligation) is incorrect. If medical therapy with octreotide fails,
endoscopic band ligation is the next step in the management of bleeding esophageal
varices.
Option D (Octreotide) is incorrect. Were this patient hemodynamically stable, the use of
octreotide is considered the first step in the management of bleeding esophageal varices.
Option E (Propranolol) is incorrect. Propranolol and other nonselective beta blockers are
used as prophylaxis against bleeding esophageal varices. There is no role acutely,
especially not in hypotensive patients.
The first step in the evaluation and therapy for the patient with acute GI hemorrhage is to
determine the severity of blood loss. Vital signs should be recorded immediately. If the
systolic blood pressure drops more than 10 mm Hg and/or the pulse increases more than 10
beats per minute as the patient changes positions from supine to standing, it is likely the
patient has lost at least 800 mL (15%) of circulating blood volume. Hypotension, tachycardia,
tachypnea, and mental status changes in the setting of acute GI hemorrhage suggest at
least a 1500-mL (30%) loss of circulating blood volume.
The goal of resuscitation is to restore the normal circulatory volume. Initially, at least two
large-bore intravenous catheters are used to administer isotonic solutions (e.g., lactated
Ringer's solution or 0.9% NaCl), and blood products if indicated. If the patient is in shock, a
central venous access should be established. The amount of blood products to be
transfused must be individualized. Transfusions of packed red blood cells are provided to
prevent complications (e.g., angina, congestive heart failure, stroke) of acute blood loss.
Therefore, the need for blood transfusion depends on multiple factors, including the patient's
age, overall health, and response of vital signs to initial resuscitation. In view of the risks of
blood transfusion, it is not appropriate simply to transfuse until an arbitrary hematocrit is
achieved. If coagulation studies are abnormal, as commonly observed in cirrhotic patients,
fresh-frozen plasma and/or platelets may be required to control ongoing hemorrhage.
Opinions differ regarding the use of nasogastric lavage in preparation for endoscopic
examination, although aspiration of gastric blood may be particularly important in patients
with liver cirrhosis, because blood in the GI tract may precipitate hepatic encephalopathy.
Figure 32-2 Approach to the patient with acute gastrointestinal bleeding. EGD = esophagogastroduodenoscopy.
A 73-year-old woman is brought to the emergency room because this morning she was found by
her daughter to be acutely confused. She has otherwise been in excellent health and does not
take any regular medication. The daughter reports that her mother's mental status was normal
yesterday. On arrival in the emergency room, she is disorientated to person, place, and time.
She is diaphoretic and tremulous. Her blood pressure is 125/85 mm Hg; pulse, 88 beats/minute;
temperature, 37.4°C (99.3°F); and respirations, 14 breaths/minute. Occasional ectopic heart
beats are detected during auscultation of the heart. There is bilaterally symmetrical 4/5 muscle
strength in the upper and lower extremities. Laboratory evaluation following an overnight fast
demonstrates the following:
Serum
Sodium 136 mEq/L
Chloride 97 mEq/L
Potassium 4.1 mEq/L
Bicarbonate 22 mEq/L
Magnesium 1.6 mEq/L
Calcium 8.4 mg/dL
Urea nitrogen (BUN) 11 mg/dL
Creatinine 0.6 mg/dL
Glucose 40 mg/dL
Insulin 15 micro-U/mL (Normal <9 micro-U/L)
Pro-insulin 12 micro-U/mL (Normal <20% total insulin)
C-peptide 3.01 ng/mL (Normal 0.78 – 1.89 ng/L)
D. Exogenous insulin
E. Sulfonylurea use
QID: 33220
Hypoglycemic symptoms are classified according to the their type and their timing in relation
to meals. Symptoms, such as confusion, slurred speech, blurred vision, seizures, and coma,
result from inadequate delivery of glucose to the brain (neuroglycopenia). Symptoms, such
as tremors, sweating, palpitations, and nausea, result from a counter-regulatory discharge of
catecholamines (adrenergic). When symptoms occur within 5 hours of the previous meal,
they are considered to be "postprandial"; if they occur more than 5 hours after a meal they
are considered to be "fasting." Insulinomas most commonly cause fasting neuroglycopenic
symptoms, although postprandial and adrenergic symptoms may also occur.
Surreptitious Sulfonylurea or
Test Insulinoma Insulin Use Meglitinide Use
Insulin ↑ ↑ ↑
C-peptide ↑ ↓ ↑
Proinsulin ↑ ↓ Nl
Drug screen Neg Neg Pos
A 19-year-old woman is brought to the physician by her parents because they have noticed that
she is increasingly losing weight. She states that there is nothing wrong with her behavior and
that she is purposefully trying to lose weight simply because she is overweight. Her parents state
that her sole hobby is to photograph food and that she has forsaken all other activities to devote
herself to food photography. Further questioning reveals that she is intensely fearful of being
overweight. A review of systems finds that she has not had menses in the last 4 months despite
no sexual activity. Physical examination reveals a body mass index (BMI) of 15 kg/m2 (15 lb/in2),
lanugo, and dry skin. What is the most likely diagnosis?
A. Anorexia nervosa
B. Binge-eating disorder
D. Bulimia nervosa
E. Schizophrenia
QID: 33512
Option A (Anorexia nervosa) is correct. The diagnosis of anorexia requires four elements:
a refusal to maintain appropriate body weight, an intense fear of gaining weight, undue
influence of body shape/weight on self-image, and amenorrhea (in postmenarchal females).
Some anorexics can purge, but the behavior is not primary and not as frequent as in bulimia.
Also, note for the diagnosis that the patient must be below 85% of minimally recommended
weight. In this case, all four criteria are met.
Option D (Bulimia nervosa) is incorrect. The diagnosis of bulimia requires that there be
episodes of binge eating followed by inappropriate compensatory behavior. The patient must
be unduly influenced by body size/weight, and the behavior must exist twice a week for 3
months. Note that the compensatory behavior does not need to be purging in nature.
Excessive exercise, abuse of laxatives, enemas, and diuretics are also considered
inappropriate compensatory behaviors.
Comorbid
Disorder Features Conditions Complications*
Anorexia Maintenance of weight <85% normal for Depressive disorders Hypotension
nervosa age and height Obsessive-compulsive Bradycardia
Distorted perceptions of own body size disorder Hypothermia
Intense fear of gaining weight or Personality disorders Leukopenia, anemia
becoming fat; self-esteem largely Osteoporosis
determined by weight Dry skin, lanugo (fine, downy
Amenorrhea: absence of at least three hair)
consecutive menstrual cycles Cardiac arrhythmias
Denial of serious health effects of very
low body weight
Restricting type: obsessive-compulsive
behavior, social withdrawal, restrained
emotional expression, strong need for
control
Bulimia Repeated binge eating Depressive disorders Loss of dental enamel
nervosa Use of inappropriate methods to prevent Anxiety disorders Fluid and electrolyte
weight gain Substance use disorders disturbances
Sense of lack of control during binge- Personality disorders Cardiac arrhythmias
eating episodes (frequently borderline Swollen parotid glands
Typically normal weight personality disorder) Calluses on back of hand
Self-esteem largely determined by from self-induced vomiting
weight Esophageal tears or ruptures
Restricted intake between binges Dehydration
Hiding binge eating and purging from
others
Impulsive behaviors (e.g., stealing,
promiscuity, overspending)
B. Emergent surgery
C. Intravenous labetalol
QID: 33208
Option C (Intravenous labetalol) is correct. Ripping chest pain that radiates into the back
and stomach, combined with a pulse deficit, previous history of hypertension and widened
mediastinum on chest X-ray (CXR) should be considered an aortic dissection until proven
otherwise. As soon as aortic dissection is suspected, immediate intravenous (IV) beta-
blocker therapy should be started to reduce the pulse and blood pressure of the patient in an
attempt to limit the severity of the dissection.
• Chest x-ray may show widened mediastinum (62%) and displacement of aortic
intimal calcium.
• Transesophageal echocardiography (TEE) is study of choice in unstable patients, but
operator dependent.
• MRI has the highest sensitivity and specificity but limited availability; not suitable for
unstable patients; contraindicated with pacemakers, metal devices.
• Helical CT is least operator dependent, involves intravenous contrast.
• TEE, MRI, helical CT are imaging modalities of choice. Sensitivities (98% to 100%)
and specificities (95% to 98%) nearly equal in skilled hands. Test of choice depends
on clinical circumstances and availability.
• With medium or high pretest probability, a second diagnostic test should be done if
the first is negative.
• Transthoracic echocardiography has poor sensitivity
• Aortography rarely done now.
TREATMENT
ACUTE GENERAL Rx
A 34-year-old gravida IV para III female at 37 weeks of gestation is brought to the emergency
room following the sudden onset of abdominal pain and vaginal bleeding 2 hours ago. The pain
has been constant from onset. She has also experienced frequent, strong contractions. She has
been otherwise well, abstinent from intercourse for the past 2 months, and her previous medical
and obstetric history are unremarkable. Her vital signs are blood pressure (BP), 100/65 mm Hg;
pulse, 110 beats/minute; temperature, 37.7°C (99.8°F); and respirations, 14 breaths/minute. The
uterus is painful and rigid. Speculum examination demonstrates mild vaginal bleeding. Fetal
heart rate tracing is not reassuring. What is the most likely diagnosis?
A. Abruptio placenta
B. Placenta previa
C. Spontaneous abortion
D. Uterine rupture
E. Vasa previa
QID: 33070
Option A (Abruptio placenta) is correct. This patient is demonstrating the classic signs of
placental abruption: painful, abrupt vaginal bleeding associated with uterine contractions and
nonreassuring fetal heart tracing. The pain is constant, and the uterus is firm because of
tetanic contractions. Vaginal bleeding can be highly variable and does not correlate well with
the severity of abruption.
Option B (Placenta previa) is incorrect. Placenta previa is classically bright red painless
bleeding that is not as rapid in onset as abruption. The uterus is usually soft.
Option D (Uterine rupture) is incorrect. Uterine rupture is painful third trimester bleeding,
but is very rare. Unlike abruptio placenta, it is associated with constant heavy vaginal
bleeding. A classic presentation on the examination is a fetus that is at a +2 station that
suddenly retracts into the -1 position.
Option E (Vasa previa) is incorrect. Vasa previa has a classic triad of membrane rupture
followed by vaginal bleeding and then fetal bradycardia. Vasa previa is bleeding from fetal
vessels and is diagnosed using the Apt test.
ABRUPTIO PLACENTAE
• Maternal hypertension
• Placental abruption in a prior pregnancy
• Trauma
• Polyhydramnios with rapid decompression
• Premature rupture of membranes
• Short umbilical cord
• Tobacco use
• Folate deficiency
When adenocarcinoma of the pancreas arises in older adults, molecular analysis of the
carcinoma cells reveals multiple genetic alterations from normal pancreatic ductal cells. A point
mutation involving which of the following genes, which leads to formation of a protein product that
is constitutively active, is most likely to be present in these carcinoma cells?
A. APC
B. HER2
C. K-RAS
D. p53 (TP53)
E. SPINK1
QID: 95445
Option C (K-RAS) is correct. RAS mutations are common in epithelial cancers, particularly
adenocarcinomas, and are the most common gene mutations in pancreatic
adenocarcinomas. The abnormally activated RAS protein drives intracellular signal
transduction pathways that promote production of transcription factors driving cellular
proliferation.
Option A (APC) is incorrect. APC gene mutations are most likely to be associated with
adenocarcinomas of the colon.
Option D (p53 (TP53)) is incorrect.Although p53 (TP53) mutations are common in many
forms of carcinoma, the mechanism is that of a tumor suppressor gone awry. p53 protein
acts in cell cycle checkpoint pathways and in control of cellular apoptosis.
Option E (SPINK1) is incorrect. The SPINK1 and PRSS1 gene mutations are associated
with hereditary pancreatitis.
The symptomatic course of pancreatic carcinoma is typically brief and progressive. Despite
the tendency of lesions of the head of the pancreas to obstruct the biliary system, fewer than
20% of pancreatic cancers overall are resectable at the time of diagnosis. There has long
been a search for biochemical tests that could be useful in the early detection of pancreatic
cancer. The K-RAS oncogene is mutated in 90% of pancreatic cancers; however, the utility
of screening tests for K-RAS mutations remains unproven. Serum levels of many enzymes
and antigens (e.g., carcinoembryonic antigen and CA19-9 antigen) have been found to be
elevated, but these markers are not specific nor are they sensitive enough to be used as
screening tests. Several imaging techniques, such as endoscopic ultrasonography and CT,
have proved of great value in diagnosis and the performance of percutaneous needle
biopsy. Both of these techniques, while useful in establishing a diagnosis, are not useful as
screening tests.
A 44-year-old woman has experienced pain in her neck over the past 2 weeks. On physical
examination her thyroid is enlarged, firm, and tender to palpation. Her serum thyroxine is
increased. A fine needle aspiration biopsy shows multinucleated giant cells along with acute and
chronic inflammatory cells and destruction of thyroid follicles. At follow-up 2 months later she is
euthyroid. Which of the following is the most likely risk factor for development of her disease?
A. HLA-DR3 allele
C. Iodine deficiency
F. Viral pneumonia
QID: 95548
Option A (HLA-DR3 allele) is incorrect. This HLA antigenic type is associated with
development of Graves disease.
Option B (Idiosyncratic drug reaction) is incorrect. Drugs are not likely to produce
inflammation of the thyroid gland.
Option C (Iodine deficiency) is incorrect. Iodine deficiency would lead to a goiter with
enlarged colloid-filled follicles lined by inactive flattened epithelium.
Option D (Prior radiation exposure) is incorrect. Radiation to the region of the neck
encompassing the thyroid gland is a risk for thyroid carcinoma.
Option E (RET gene mutation) is incorrect. RET gene mutations are associated with
multiple endocrine neoplasia (MEN) IIa or IIb. Medullary carcinoma of the thyroid gland may
occur in this setting.
A 31-year-old man has had a feeling of heaviness in his scrotum for over 6 months. The
examining physician notes an enlarged right testis. An ultrasound reveals a solid 5-cm mass in
the body of the right testis. Laboratory studies show a serum α-fetoprotein (AFP) of 81 ng/mL
and human chorionic gonadotropin (HCG) of 15 IU/L. A right orchiectomy is performed, and on
gross examination the testicular mass is soft and reddish brown. Microscopic examination shows
cords and sheets of primitive cells with large nuclei. Which of the following is the most likely
diagnosis?
A. Choriocarcinoma
B. Embryonal carcinoma
E. Teratoma
QID: 95485
Option C (Leydig cell tumor) is incorrect. Leydig cell tumors are not common. Most are
small brown masses.
Option D (Squamous cell carcinoma) is incorrect. Squamous cell carcinomas do not
arise in the testis.
Option E (Teratoma) is incorrect. A pure teratoma in the testis is very uncommon. Usually
a malignant component such as embryonal carcinoma is present as well as part of a mixed
germ cell tumor.
Option F (Yolk sac tumo) is incorrect. Yolk sac tumor is typically present in the pediatric
age group.
A 10-year-old girl has developed increasing weakness over the past year. She has no muscle
pain or atrophy. She has epilepsy. Other family members are affected as shown by the pedigree
in the image. On physical examination she exhibits ataxia. A gastrocnemius biopsy is performed
and on microscopic examination shows increased numbers of ragged red fibers. Later in life, she
suffers a “stroke” at age 20. Which of the following conditions is she most likely to have?
B. Channelopathy
C. Lambert-Eaton syndrome
D. Mitochondrial myopathy
E. Myotonic dystrophy
QID: 95609
Clinical Course and Genetics. The relationship between clinical course in the
mitochondrial disorders and the genetic alterations is not entirely clear; however, three
general categories have been defined. One set of mutations consists of point mutations in
mtDNA. These disorders tend to show a maternal pattern of inheritance, and some
examples include myoclonic epilepsy with ragged red fibers (MERRF), Leber hereditary optic
neuropathy (LHON), and mitochondrial encephalomyopathy with lactic acidosis and
strokelike episodes (MELAS). A second set of mutations involves genes encoded by nuclear
DNA and shows autosomal-dominant or autosomal-recessive inheritance. Some cases of
subacute necrotizing encephalopathy (Leigh syndrome), exertional myoglobinuria, and
infantile X-linked cardioskeletal myopathy (Barth syndrome) are due to mutations in nuclear
DNA. The final subset of mitochondrial myopathies is caused by deletions or duplications of
mtDNA. Examples include chronic progressive external ophthalmoplegia, characterized by a
myopathy with prominent weakness of external ocular movements. Kearns-Sayre syndrome,
another myopathy in this group, is also characterized by ophthalmoplegia but, in addition,
includes pigmentary degeneration of the retina and complete heart block.
A 45-year-old woman feels a “lump” in her left breast. Her physician palpates a 2-cm irregular
area in the upper outer quadrant. A biopsy is performed and microscopic examination shows no
evidence for carcinoma. Which of the following microscopic findings in this biopsy is most likely
to suggest an increased risk for subsequent development of breast carcinoma?
A. Fat necrosis
B. Galactocele
D. Multiple cysts
E. Sclerosing adenosis
QID: 95524
Option C (Lobular epithelial hyperplasia) is correct. Particularly when atypical
microscopic features are present, lobular or ductal hyperplasia is the component of
fibrocystic changes that is associated with subsequent increased risk for development of
carcinoma.
Option A (Fat necrosis) is incorrect. This lesion may sometimes be difficult to distinguish
grossly from carcinoma, but it does not predispose to carcinoma.
Option D (Multiple cysts) is incorrect.Simple cysts with fibrocystic disease are not
associated with an increased risk for carcinoma.
A 25-year-old woman has the sudden onset of fever, malaise, and nausea. On physical
examination she is afebrile. Her blood pressure is 160/90 mmHg. She has presacral edema. A
routine urinalysis shows 1+ protein, no ketones, no glucose, and 4+ blood, with RBC
casts seen on urine microscopic examination. A renal biopsy is performed; the light microscopic
findings are shown in the image. By immunofluorescence there is granular deposition of IgG and
C3 in glomerular capillary basement membranes. By electron microscopy there are electron-
dense subepithelial “humps.” Infection with which of the following organisms most likely preceded
development of her renal disease?
Courtesy of Dr. John A. Blackmon, Florida State University College of Medicine.
A. Candida albicans
B. Group A streptococus
C. Hepatitis B
D. Mycobacterium tuberclosis
E. Schistosoma hematobium
QID: 95456
Option A (Candida albicans) is incorrect. Candida urinary tract infections can occur when
patients are immunocompromised or very ill.
A 50-year-old man has a past medical history including paresthesias, difficulty moving one or
more extremities, loss of sensation, and ataxia over the past 22 years. These problems have
waxed and waned. He now has developed paraplegia and incontinence. His brain T2-weighted
MR image is shown. Which of the following conditions involving his CNS is most likely to explain
the course of his disease?
A. Meningioma
B. Multiple sclerosis
D. Traumatic injury
E. Wernicke disease
QID: 95622
Option B (Multiple sclerosis) is correct. The history is most consistent with multiple
sclerosis, a demyelinating disease that has exacerbations and remissions, but in most
affected persons is eventually progressive. Note the bright plaques of demyelination
involving the white matter of centrum semiovale.
Option D (Traumatic injury) is incorrect.A traumatic injury is not consistent with the range
and variability of his findings. His neurologic problems are not service connected.
A 60-year-old woman develops severe chest pain over the past 6 hours. On physical
examination she has tachycardia with hypotension. Laboratory studies show an elevated serum
creatine kinase MB fraction. A coronary angiogram is performed emergently and reveals greater
than 90% occlusion of the left circumflex artery. Which of the following cellular changes is most
indicative of an irreversible injury to her myocardial fibers?
A. Glycogen depletion
C. Nuclear karyorrhexis
D. Diminished intracellular pH
QID: 95048
Option E (Cell membrane blebbing) is incorrect. Such blebs are reversible and do not
constitute an irreversible injury.
Nuclear changes appear in the form of one of three patterns, all due to
nonspecific breakdown of DNA. The basophilia of the chromatin may
fade (karyolysis), a change that presumably reflects DNase activity. A
second pattern (also seen in apoptotic cell death) is pyknosis,
characterized by nuclear shrinkage and increased basophilia. Here the
DNA apparently condenses into a solid, shrunken basophilic mass. In
the third pattern, known as karyorrhexis, the pyknotic or partially
pyknotic nucleus undergoes fragmentation. With the passage of time (a
day or two), the nucleus in the necrotic cell totally disappears.
Once the necrotic cells have undergone the early alterations described,
the mass of necrotic cells may have several morphologic patterns.
Although the terms are somewhat outmoded, they are routinely used
and their meanings are understood by both pathologists and clinicians.
When denaturation is the primary pattern, coagulative necrosis
develops. In the instance of dominant enzyme digestion, the result is
liquefactive necrosis; in special circumstances, caseous necrosis
and fat necrosis may occur.
A 41-year-old man has epigastric burning pain along with nausea following meals for the past 6
months. He has lost 2 kg (4.4 lb) during that time. He has this pain when eating, but not often in
between meals or at night. On physical examination there are no abnormal findings. Upper GI
endoscopy is performed and there are two sharply demarcated ulcerations, 0.8 cm and 1.6 cm in
diameter, located in the antrum. Which of the following drugs used by this man is most likely to
have caused his illness?
B. Cimetidine F. Ibuprofen
C. Cefotaxime G. Isoniazid
D. Cocaine H. Omeprazole
QID: 95412
Option D (Cocaine) is incorrect. Cocaine is a powerful vasoconstrictor, but its effect on the
GI tract is minimal.
Option E (Ferrous sulfate) is incorrect. Ferrous sulfate does not produce gastric
ulceration.
Chronic use of NSAIDs suppresses mucosal prostaglandin synthesis; aspirin also is a direct
irritant. Cigarette smoking impairs mucosal blood flow and healing. Alcohol has not been
proved to directly cause peptic ulceration, but alcoholic cirrhosis is associated with an
increased incidence of peptic ulcers. Corticosteroids in high dose and with repeated use
promote ulcer formation. In some patients with duodenal ulcers, there is too-rapid gastric
emptying, exposing the duodenal mucosa to an excessive acid load. Duodenal ulcer also is
more frequent in patients with alcoholic cirrhosis, chronic obstructive pulmonary disease,
chronic renal failure, and hyperparathyroidism. In the latter two conditions, hypercalcemia
stimulates gastrin production and therefore acid secretion. Genetic influences appear to play
no major role in peptic ulceration. Finally, there are compelling arguments that personality
and psychological stress are important contributing factors, even though hard data on cause
and effect are lacking. Indeed, we might develop ulcers by trying to fathom their cause(s).
A. Acanthamoeba
B. Candida albicans
C. Chlamydia trachomatis
D. Cytomegalovirus (CMV)
F. Hemophilus aegypticus
G. Pseudomonas aeruginosa
QID: 95641
Option E (Herpes simplex virus) is correct. These lesions can ulcerate acutely and lead
to scarring with chronic blindness.
Option A (Acanthamoeba) is incorrect. This organism does not produce dendritic ulcers.
Option B (Candida albicans) is incorrect. The eye is an uncommon site for fungal
infections.
A 47-year-old woman has had episodes of right upper quadrant pain during the past 2 weeks.
Her stools have become pale in color over the past 3 days. Laboratory studies show her serum
total bilirubin is 9.7 mg/dL. A cholangiogram shows that a gallstone has passed into the common
bile duct. Which of the following cellular alterations is most likely to be visualized on her skin
surfaces?
A. Calcification
B. Hemosiderosis
C. Icterus
D. Lipofuscin deposition
E. Steatosis
QID: 95436
Option C (Icterus) is correct. She probably has a “jaundiced” appearance to her sclerae
and skin due to the increased amount of bilirubin. The bile pigments impart a yellow color to
the tissues. She has biliary tract obstruction from cholelithiasis and choledocholithiasis.
Option E (Steatosis) is incorrect. Fatty change (steatosis) is a process that occurs in the
liver, but biliary tract obstruction does not typically cause it. Steatosis can occur in the setting
of alcohol abuse. Nonalcoholic fatty liver is seen in patients with obesity and diabetes
mellitus. An element of steatosis can often be present with chronic hepatitis.
These conditions are considered together, since they frequently go hand in hand.
Choledocholithiasis is defined as the presence of stones within the bile ducts of the biliary
tree, as opposed to cholelithiasis (stones in the gallbladder). In Western nations, almost all
biliary tract stones are derived from the gallbladder, although both cholesterol and
pigmented stones can form de novo anywhere in the biliary tree. In Asia, there is a much
higher incidence of primary stone formation within the biliary tree, usually pigmented as a
result of the biliary tract infections noted earlier in the discussion of gallstones.
Choledocholithiasis may be asymptomatic or may cause symptoms from (1) obstruction, (2)
pancreatitis, (3) cholangitis, (4) hepatic abscess, (5) secondary biliary cirrhosis, and (6)
acute calculous cholecystitis.
A 10-year-old girl has complained of joint pain for the past 2 years. The pain is localized to her
knees and ankles; it occurs either with or without movement. The arthralgias are accompanied by
fever. On physical examination she has a temperature of 37.8° C (100° F). There is no deformity
of her knees. She has generalized lymphadenopathy. Which of the following is the most likely
diagnosis?
A. Gonococcal arthritis
B. Ankylosing spondylitis
D. Tuberculous arthritis
E. Gout
QID: 95596
Option C (Juvenile rheumatoid arthritis) is correct. Also known as Still disease, juvenile
rheumatoid arthritis (JRA) can involve just a single joint or a few joints (oligoarticular) or
more than five joints (polyarticular). About 5% of rheumatoid arthritis cases occur in persons
less than 16 years of age. Large joints are more often involved than small joints with JRA.
Option E (Gout) is incorrect. Gouty arthritis usually involves the big toe of males with
hyperuricemia who are past the third decade of life.
JRA differs from RA in adults in the following ways: (1) oligoarthritis is more common, (2)
systemic onset is more frequent, (3) large joints are affected more often than small joints, (4)
rheumatoid nodules and rheumatoid factor are usually absent, and (5) antinuclear antibody
seropositivity is common. Pathogenetic factors, similar to those in RA, include genetic
association with particular HLA haplotypes (DRB1); mycobacterial, bacterial, or viral
infection; abnormal immunoregulation with the prevalence of activated CD4+ T cells within
involved joints; and cytokine production. The morphologic changes in joint pathology are
similar to those seen in adult RA.
Commonly targeted joints are the knees, wrists, elbows, and ankles. They become warm
and swollen and are often involved symmetrically. Pericarditis, myocarditis, pulmonary
fibrosis, glomerulonephritis, uveitis, and growth retardation are potential extra-articular
manifestations. A systemic onset may begin rather abruptly, associated with high spiking
fevers, migratory and transient skin rash, hepatosplenomegaly, and serositis. Long-term
studies of patients with jra show that one third to one half of patients have active disease
when followed for at least 10 years.
A 21-year-old woman has a routine Pap smear performed for a health screening examination.
The pathology report indicates that some cells are found cytologically to have larger, more
irregular nuclei. A follow-up cervical biopsy is shown in the image. No inflammatory cells are
present. Which of the following descriptive terms is best applied to these Pap smear and biopsy
findings?
Courtesy of Dr. David Cohen, Tel Aviv University
A. Anaplasia
B. Aplasia
C. Dysplasia
D. Hyperplasia
E. Metaplasia
QID: 95133
Before we leave the subject of differentiation and anaplasia, we should discuss dysplasia, a
term that literally means disordered growth. Dysplasia is encountered principally in epithelia,
and it is characterized by a constellation of changes that include a loss in the uniformity of
the individual cells as well as a loss in their architectural orientation. Dysplastic cells also
exhibit considerable pleomorphism and often contain hyperchromatic nuclei that are
abnormally large for the size of the cell. Mitotic figures are more abundant than usual,
although almost invariably they conform to normal patterns. Frequently the mitoses appear
in abnormal locations within the epithelium. Thus, in dysplastic stratified squamous
epithelium, mitoses are not confined to the basal layers and may appear at all levels and
even in surface cells. The architecture of the tissue may be disorderly. For example, the
usual progressive maturation of tall cells in the basal layer to flattened squames on the
surface may be lost and replaced by a scrambling of dark basal-appearing cells throughout
the epithelium. When dysplastic changes are marked and involve the entire thickness of the
epithelium, but the lesion remains confined to the normal tissue, it is considered a
preinvasive neoplasm and is referred to as carcinoma in situ. Once the tumor cells move
beyond the normal confines, the tumor is said to be invasive. Dysplastic changes are often
found adjacent to foci of invasive carcinoma, and in some situations, such as in long-term
cigarette smokers and Barrett esophagus, severe epithelial dysplasia frequently antedates
the appearance of cancer. However, dysplasia does not necessarily progress to cancer. Mild
to moderate changes that do not involve the entire thickness of epithelium may be
reversible, and with removal of the inciting causes, the epithelium may revert to normal.
An autopsy study reveals that evidence for atheroma formation can begin even in children. The
gross appearances of the aortas are recorded and compared with microscopic findings of
atheroma formation. Which of the following lesions shown in the image is most likely to be the
first visible gross evidence for the formation of an atheroma?
A. Calcification
B. Exudate
C. Fatty streak
D. Hemorrhage
E. Thrombus
F. Ulceration
QID: 95293
Option C (Fatty streak) is correct. This is the first sign of more to come. The fatty streak is
benign and reversible, but it may be the precursor to more severe plaques if risk factors are
present and continue.
Fatty streaks are the earliest lesion of atherosclerosis. They are composed of lipid-filled
foam cells. They are not significantly raised and thus do not cause any disturbance in blood
flow. Fatty streaks begin as multiple yellow, flat spots less than 1 mm in diameter that
coalesce into elongated streaks, 1 cm long or longer. They contain T lymphocytes and
extracellular lipid in smaller amounts than in plaques.
A 38-year-old man has had scaling patches on his elbows, knees, and scalp for the past 6
months. On physical examination the lesions have the appearance shown. In addition he has
yellow-brown discoloration with thickening and pitting of his fingernails. A punch biopsy of one
lesion is performed and on microscopic examination shows thinning of the stratum granulosum,
downward projection of the rete pegs, small neutrophilic aggregates in the superficial epidermis,
and marked overlying parakeratosis. Therapy with methotrexate and UVA light reduces the size
of the lesions. Which of the following is the most likely diagnosis?
From Klatt E: Robbins and Cotran Atlas of Pathology, 2nd ed. Philadelphia, Elsevier, 2010.
A. Bullous pemphigoid
B. Dermatitis herpetiformis
D. Lichen planus
E. Pemphigus vulgaris
F. Psoriasis
QID: 95572
Option D (Lichen planus) is incorrect. Lichen planus is a self-limited disease that usually
disappears in a year or two. There are pruritic, flattened, purplish papules on the skin, and
white to reticulated areas on mucous membranes. Microscopically, there is a dense,
bandlike upper dermal infiltrate of lymphocytes.
Clinically, psoriasis most frequently affects the skin of the elbows, knees, scalp, lumbosacral
areas, intergluteal cleft, and glans penis. The most typical lesion is a well-demarcated, pink
to salmon-colored plaque covered by loosely adherent scales that are characteristically
silver-white in color. Variations exist, with some lesions occurring in annular, linear, gyrate,
or serpiginous configurations. Psoriasis can be one cause of total body erythema and
scaling known as erythroderma. Nail changes occur in 30% of cases of psoriasis and consist
of yellow-brown discoloration (often likened to an oil slick), with pitting, dimpling, separation
of the nail plate from the underlying bed (onycholysis), thickening, and crumbling. In the rare
variant called pustular psoriasis, multiple small pustules form on erythematous plaques. This
type of psoriasis is either benign and localized (hands and feet) or generalized and life-
threatening, with associated fever, leukocytosis, arthralgia, diffuse cutaneous and mucosal
pustules, secondary infection, and electrolyte disturbances.