Cardio USMLE 9-27 (3) Flashcards

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1
Q

This 30-year-old woman presents with severe knee pain and a tender effusion on the knee. Based on the patient’s age and gender, as well as the location of the tumor, she most likely has a giant cell tumor. The appearance of the tumor—with a nonsclerotic and sharply defined border—is also a clue that this is a?

A

giant cell tumor.

Giant cell tumors are primary bone tumors that most commonly occur at the epiphyseal end of long bones. They are benign, but locally aggressive tumors occurring most frequently at the distal femur or proximal tibia. Giant cell tumors have a peak incidence in people from age 20 to 40 years old. They tend to occur more often in women.
On radiography, giant cell tumors generally appear as “soap bubble” lytic lesions next to a joint. They are distinguishable from other bony tumors on xX-ray, since they appear to have a nonsclerotic and sharply defined border. Giant cell tumors are typically composed of oval mononuclear cells along with scattered multinucleated giant

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2
Q

Chondrosarcoma more commonly affects the axial skeleton. Ewing sarcoma classically shows onion-skinning on x-ray, and primarily affects younger boys. Osteochondromas affect ?

A

the metaphysis of long bones. Osteosarcoma is more common in younger males and shows periosteal shadowing (Codman triangle) on x-ray.

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3
Q

Giant cell tumors most frequently occur at the epiphyseal end of long bones and most commonly in women age 20–40 years old. The classic radiologic findings are?

A

“soap bubble” lesions at the epiphyses of long bones.

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4
Q

This patient with pearly white/skin-colored papules with central umbilication has the classic dermatological finding associated with molluscum contagiosum. Caused by a poxvirus, an ?

A

enveloped virus with double-stranded, linear DNA, molluscum contagiosum virus is most frequently seen in children. In this population, the virus is often transmitted through skin-to-skin contact or indirect contact with fomites (e.g. gymnasium equipment). Lesions commonly occur on the chest, arms, trunk, legs, face, and intertriginous areas.

Molluscum contagiosum also occurs in adults and may be transmitted sexually. In such cases, if the patient is immunocompetent, lesions tend to be located around the genitalia and surrounding areas, as seen in this patient. Widespread, persistent, and atypical presentations may be seen in immunocompromised patients. The infection often resolves spontaneously, but may require topical therapy or surgery in immunocompromised patients.

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5
Q

The organisms named in the alternate answer choices do not produce the pearly white or skin-colored papules with central umbilication seen in this patient’s skin. Among the non-enveloped RNA viruses, coxsackie virus, which causes hand, foot, and mouth disease, is most closely associated with a rash; however, this is described as red, blister-like lesions. Microsporum, a mold, (and several similar organisms) cause cutaneous infections with a pruritic, flat rash on several parts of the body. Malassezia species, al-shaped, budding yeasts, are responsible for tinea versicolor, an infection in which patches of skin become discolored due to?

A

melanocyte damage by the infecting fungus. Rickettsia are gram-negative, obligate intracellular bacteria capable of causing a rash, which is erythematous; other similar organisms also cause rashes different from those in the illustration. Infections caused by this pathogen often present with systemic symptoms, as well. Dermatological findings of Treponema pallidum, a spiral-shaped bacterium, include maculopapular rashes, characterized by flat, coarse red areas of skin; other spirochetes also cause rashes.

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6
Q

Molluscum contagiosum manifests as white or skin-colored papules or nodules with?

A

central umbilication. It often resolves spontaneously.

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7
Q

The woman has signs of fever, dyspnea, hypotension, and widespread coagulopathy such as oozing IV sites and uncontrollable vaginal bleeding. The inadequate hemostasis should raise suspicion for disseminated intravascular coagulation (DIC), likely secondary to an amniotic fluid embolism that traveled to her pulmonary circulation. Common causes of DIC are gram-negative sepsis, malignancy, pancreatitis, trauma, transfusion reactions, and obstetric complications such as amniotic fluid embolism.

In DIC, there is a massive activation of the coagulation cascade that results in thrombus formation throughout the microvasculature. This results in rapid consumption of both platelets and coagulation factors. Concurrent with this consumption coagulopathy is activation of the fibrinolytic system, which results in elevated fibrin degradation products (FDPs). Ultimately, complications in DIC result from thrombosis and bleeding and include massive blood loss and organ failure. Laboratory findings include?

A

a decreased platelet count, elevated bleeding time, elevated prothrombin time, elevated partial thromboplastin time, and elevated D-dimer.

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8
Q

DIC may be caused by sepsis, malignancy, and obstetric complications. The resultant consumption coagulopathy leads to?

A

laboratory findings of a decreased platelet count, elevated bleeding time, elevated prothrombin time, elevated partial thromboplastin time, and elevated fibrin split products.

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9
Q

This asymptomatic patient presents for a routine physical exam and has a mildly decreased hemoglobin. Because the MCV is decreased, it is considered a microcytic anemia. The two most common causes of microcytic anemia are iron deficiency anemia and thalassemia. Definitive diagnosis of either disease requires iron studies and a hemoglobin electrophoresis. However, a presumptive diagnosis can be made by reviewing the remaining CBC indices.

This patient’s RBC is increased, and his red cell distribution width (RDW) is normal, both of which are consistent with?

A

thalassemia and inconsistent with iron-deficiency anemia. In mild cases of thalassemia, the RBC is actually increased, which is unusual for an anemia (in iron-deficiency anemia, for example, the RBC is decreased). The RDW is a measure of how much variation in size there is in the patient’s red cells. In mild thalassemia, the red cells are all similar in size, meaning the RDW is normal. In iron-deficiency anemia, the red cells get smaller and smaller as the iron deficiency progresses, resulting in an RDW which is increased.
This patient’s Tunisian ethnicity is also consistent with ß-thalassemia, which is more common in individuals from countries around the Mediterranean, North Africa, the Indian subcontinent, and Eastern Europe.
A common morphologic finding in thalassemia is target cells (red cells with a condensation of hemoglobin in the center, giving them a target appearance). Several target cells can be seen in the image above.

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10
Q

Helmet-shaped red cells are seen in disseminated intravascular coagulation (DIC). The blood in DIC shows a microangiopathic hemolytic anemia, characterized by schistocytes (fragmented red cells, such as helmet cells).
Neutrophils with an increased number of nuclear lobes (or hypersegmented neutrophils) are indicative of megaloblastic anemia, a macrocytic anemia due to decreased B12 and/or folate.
Crescent-shaped red cells are seen in ?

A

sickle cell anemia, a genetic disorder in which a point mutation in the beta globin chain causes hemoglobin polymerization (and sickle cell formation) at low oxygen tension. Sickle cell anemia is typically normocytic (the MCV is usually within the normal range), and the RBC is normal to decreased. The RDW is increased during sickle cell crises due to the variation in cell size.
RBCs lacking central pallor (spherocytes) are seen in hemolytic anemias and in hereditary spherocytosis, which are normocytic anemias.

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11
Q

Mild thalassemia typically presents as a microcytic anemia, with two characteristic CBC findings: an increased RBC and a normal RDW. Target cells are frequently present in?

A

the blood smear, although they are seen in other disorders as well.

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12
Q

Certain changes in a pregnant woman’s thyroid hormone levels can be anticipated. Due in part to rising estrogen levels, there is an increase in hepatic synthesis of thyroxine-binding globulin (TBG). The total amount of thyroid hormone in the body (represented by total T4 ) subsequently increases with the rise in TBG. During a normal pregnancy, the level of unbound, active thyroid hormone (represented as free T4 ) typically remains unchanged due to normal negative feedback processes. As such, this patient will have to take increased levels of her thyroid hormone to counteract the elevated TBG. However, a pregnant woman with no history of thyroid disease will be clinically ?

A

euthyroid despite her elevated levels of TBG and total T4.

In a healthy pregnancy neither thyroid-binding globulin nor total T4 decreases; this patient shows no signs of the hypothyroidism that would be associated with these changes, such as constipation and cold intolerance. In addition, she does not present with levels that suggest anorexia or chronic liver disease, low TBG, decreased total T4, and unchanged free T4. Increased estrogen causes TBG to increase. An increase in Free T4 would indicate hyperthyroidism, a complication that occurs in 1 out of 1000–2000 pregnant women and is commonly caused by Graves disease.
PregnantEstrogenGlobulinRisePregnancyEuthyroidHypothyroidismConstipation

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13
Q

During a normal pregnancy, there is an increase in?

A

total thyroxine and thyroxine-binding globulin, but the amount of metabolically active free or unbound thyroxine (T4) remains unchanged due to normal negative feedback processes. Consequently, pregnant women typically remain euthyroid.

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14
Q

This patient with painful ulcerated skin lesions and a history of abdominal pain and intermittent diarrhea most likely has Crohn disease with pyoderma gangrenosum. The diagnosis of Crohn disease is confirmed by skip lesions with edematous (cobblestone) mucosa on colonoscopy. It is not uncommon for a dermatologic manifestation of an underlying disorder to be the chief complaint of a patient with inflammatory bowel disease (IBD). Other extraintestinal manifestations include migratory arthritis, sacroiliitis, ankylosing spondylitis, erythema nodosum, uveitis, and primary sclerosing cholangitis (most common with ulcerative colitis). This patient’s pyoderma gangrenosum would be best addressed by ?

A

administration of oral corticosteroids to treat the underlying Crohn disease.

The other medication choices would not be effective against pyoderma gangrenosum, because they would not address the patient’s underlying IBD. Ampicillin, bacitracin, and tetracycline would be better suited to treating bacterial infections such as cellulitis or necrotizing fasciitis. Glipizide would be useful in controlling blood sugar in a patient with diabetes and a diabetic foot ulcer.

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15
Q

Extraintestinal manifestations of inflammatory bowel disease (IBD) include skin lesions such as pyoderma gangrenosum. Treatment of such manifestations consists of treating the underlying IBD with?

A

oral corticosteroids.

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16
Q

The patient’s debilitating symptoms of unilateral pulsating pain, photophobia, and phonophobia are all consistent with migraine headaches. Patients with migraines often also speak of an aura preceding their headaches, as well as nausea and or vomiting during the episodes.

Migraines are thought to occur due to a variety of causes. Decreased serotonin release from the brainstem is considered one of the initiating factors for migraines. In addition, release of substance P, calcitonin gene-related peptide (CGRP), and vasoactive peptides can lead to?

A

vasodilation, which is believed to contribute to migraine symptoms.
Sumatriptan is a serotonin agonist at 5-HT1B/1D receptors and is a first-line agent for the acute treatment of migraine headaches. Action on these receptors causes vasoconstriction of cerebral and meningeal vessels, which provides symptomatic relief. Sumatriptan and other triptans also inhibit trigeminal nerve activation and prevent vasoactive peptide release. The latter helps to reduce inflammation and irritation, part of the pathophysiology of headaches. Triptans are the first-line treatment for aborting and reducing the severity of migraine headaches. These drugs can also be used to treat cluster headaches.

17
Q

Decreasing reuptake of serotonin and norepinephrine (amitriptyline), calcium channel blockers (verapamil), and nonselective β-blockers (propranolol) can be used for prophylactic treatment of?

A

migraine headaches, but they are not used in the acute setting to abort a migraine. Administration of 100% oxygen can be used to abort cluster headaches but not migraines.

18
Q

A migraine headache is characterized by a unilateral pulsating pain and is associated with photophobia, phonophobia, nausea, vomiting, and aura. Sumatriptan, a serotonin agonist at 5-HT1B/1D receptors, is useful for?

A

treating migraine headaches in the acute setting. Certain β-blockers, calcium channel blockers, and tricyclic antidepressants are useful in the prophylaxis of migraine headaches.

19
Q

This patient presents with signs of bone marrow suppression such as anemia, thrombocytopenia, leukopenia, and a dry bone marrow tap. These findings are consistent with primary myelofibrosis, previously known as chronic idiopathic myelofibrosis, a hematopoietic stem cell disorder. This disease usually manifests after the age of 50.

In primary myelofibrosis, fibrotic replacement of the marrow displaces the blood cell precursors, leading to impaired hematopoiesis, severe anemia, and thrombocytopenia. The presenting signs and symptoms can be vague, including nonspecific systemic symptoms such as severe fatigue (most common), weight loss, night sweats, and fever. Other clinical features include splenomegaly (seen in almost 90% of cases), hepatomegaly, and bone pain. Bone pain and joint tenderness are a result of skeletal changes from the marrow fibrosis and are most common in the lower extremities. The splenomegaly and/or hepatomegaly in patients with myelofibrosis are the result of extramedullary hematopoiesis (as opposed to red blood cell [RBC] and platelet sequestration, which occurs in other processes, such as sickle cell anemia and advanced cirrhosis).
The classic abnormal findings on peripheral blood smear are?

A

nucleated RBCs, left-shifted white blood cells (WBCs), large platelets, and teardrop cells, like those shown in this image

One way to think of these RBCs is to imagine them “crying” because they are being forced to relocate. Definitive diagnosis requires a bone marrow biopsy that demonstrates replacement fibrosis. However, aspiration of the marrow can be difficult, often leading to a “dry tap.” When making the diagnosis, primary myelofibrosis must be distinguished from other chronic myeloproliferative disorders, such as chronic myeloid leukemia, polycythemia vera, and myelodysplastic syndrome, as well as metastatic cancer to the bone marrow.

20
Q

Acanthocytes, or spiny RBCs, are seen in liver disease, abetalipoproteinemia, anorexia nervosa, alcoholism, and hypothyroidism. They form because of abnormal membrane lipids or structural proteins.
Macro-ovalocytes are seen in megaloblastic anemia, commonly associated with vitamin B12 or folate deficiency.
Schistocytes are mechanically sheared RBCs that are seen in a variety of hematologic disorders, including ?

A

disseminated intravascular coagulation, thrombotic thrombocytopenic purpura, and HELLP syndrome (Hemolysis, Elevated Liver enzymes, and Low Platelet count).
Spherocytes are consistent with autoimmune hemolytic anemia (positive Coombs test result) or hereditary spherocytosis (negative Coombs test result).
Target cells are seen in a variety of disorders, including liver disease, thalassemia, and hemoglobin C.
The classic abnormal findings on peripheral blood smear are nucleated RBCs, left-shifted WBCs with large platelets, and teardrop cells, such as those shown in this image (think of the RBCs “crying” because they are being forced to relocate). Definitive diagnosis requires a bone marrow biopsy that demonstrates replacement fibrosis, but aspiration of the marrow is often difficult, leading to a “dry tap.”

21
Q

Classic findings of myelofibrosis on peripheral smear include?

A

teardrop cells; nucleated red blood cells; left-shifted neutrophils; and large, abnormal platelets. Physical examination usually reveals massive splenomegaly.

22
Q

This baby is genotypically female (karyotype 46,XX) but presents with virilization of her external genitalia (resulting in an ambiguous appearance) and hypotension. Both are due to congenital adrenal hyperplasia (CAH). CAH is caused by deficiencies in enzymes required for adrenocortical steroid synthesis, such as 21-hydroxylase and 11β-hydroxylase.

This infant’s symptoms are most consistent with?

A

21-hydroxylase deficiency, which results in an inability to synthesize aldosterone and cortisol. Intermediates in aldosterone/cortisol synthesis are diverted to the synthesis of androgens, and elevated androgen levels lead to masculinization/virilization in genotypically female neonates and precocious puberty in both sexes. In addition, the lack of aldosterone leads to inefficient reabsorption of sodium ions in the kidneys, resulting in hypovolemia and hypotension. Treatment includes intravenous administration of saline solution and steroid hormone replacement.

23
Q

11β-Hydroxylase deficiency also presents with masculinization and prevents synthesis of cortisol and aldosterone, but at a later step in the pathway. This results in accumulation of 11-deoxycorticosterone, a very powerful mineralocorticoid, which causes increased salt retention, hypertension, and hypervolemia. The finding of hypotension in this patient rules out 11β-hydroxylase deficiency.
17α-Hydroxylase deficiency results in ?

A

a phenotypically female newborn and is associated with production of excess aldosterone, resulting in hypertension and hypokalemia. The findings of hypotension and ambiguous genitalia in this patient are inconsistent with 7α-hydroxylase deficiency.
5α-Reductase deficiency only occurs in 46,XY individuals, who are born with male internal reproductive organs and female external genitalia. This patient has a 46,XX karyotype and hypotension, findings that are not consistent with 5α-reductase deficiency.

24
Q

A possible memory aid for enzyme deficiencies in CAH is the mnemonic phrase “One, Up!”: if the CAH deficiency starts with a 1 (eg, 11 or 17), then it causes HYPERtension) and if it ends with a 1 (eg, 11 or 21), then it causes virilization in females.
Complete androgen insensitivity syndrome (AIS) is a result of ?

A

a mutation in the androgen receptor gene. Individuals with AIS have a 46,XY karyotype and testes that are undescended or located in the labia majora. However, they have external female genitalia including a vagina. This patient has a 46,XX karyotype and ambiguous external genitalia.

25
Q

21-Hydroxylase deficiency typically manifests with ambiguous genitalia, hypotension, hypovolemia, and hyperkalemia. It is due to failure to produce ?

A

aldosterone and cortisol and diversion of the intermediates in aldosterone/cortisol synthesis to increased androgen production.

26
Q

This patient presents with fever, as well as coughing, wheezing, and hemoptysis. Given the finding of a cavitary lung mass and his history of sarcoidosis, Aspergillus fumigatus has likely infected the pre-existing lung cavities to form fungal balls, also called aspergillomas.

Under the microscope, A. fumigatus appears as ?

A

a nondimorphic mold with septate hyphae that branch at a V-shaped 45-degree angle (as seen in the image). In patients with significant hemoptysis, treatment is surgical removal of the aspergilloma. Otherwise, itraconazole may be prescribed. A. fumigatus also produces aflatoxins that, when ingested in sufficient quantities, can cause hepatocellular carcinoma.

27
Q

Squamous cell carcinoma of the lung is strongly associated with tobacco smoking and is unlikely to present with fever and air crescents on imaging.
Although the symptoms of aspergillosis can resemble infection with Mycobacterium tuberculosis, which is cultured on Lowenstein-Jensen agar, this patient does not appear to have been exposed to tuberculosis.
Mucor species are molds with ?

A

irregular nonseptate hyphae that branch at irregular angles, typically >90 degrees. Mucor infection is almost always associated with an immunocompromised state, such as diabetic ketoacidosis in patients with diabetes.
Sporothrix is an elliptical budding yeast that is visible in pus and can cause an ascending lymphadenitis with cutaneous manifestations. It does not typically cause respiratory or systemic symptoms.

28
Q

Aspergillus fumagatus is a mold with septate hyphae that branch at V-shaped 45-degree angles. A. fumigatus infection commonly causes aspergillomas (“fungus balls”). Clinical features of invasive aspergillosis include?

A

fever, chills, hemoptysis, shortness of breath, headaches, and occasionally, chest pain.