Rx Oncology/MSK Flashcards

1
Q

A 60-year-old man comes to the clinic because a 6-week history of vague epigastric pain. The patient also reports fatigue and mentions that the epigastric pain is mostly postprandial. Fecal occult blood test is positive. Gastroduodenoscopy shows a mass in the pyloric region. The tumor is centered near the pyloric sphincter on the lesser curvature. Biopsies from the margin of the mass are consistent with adenocarcinoma.

Which of the following physical examination findings is most likely to be seen in this patient due to the mass effect of the tumor?

options;

A. Decreased bowel sounds

B.Jaundice

C.Koilonychia

D.Palpable cord in the calf

E.Periumbilical nodule

A

Jaundice

The hepatoduodenal ligament contains the common bile duct, the hepatic artery, and the hepatic portal vein. Neoplasia in this area may disrupt the hepatobiliary system, resulting in jaundice.

others

Koilonychia (related to iron deficiency anemia potentially from bleeding), palpable calf cordfrom underlying deep vein thrombosis, and metastasis to the periumbilical region are potential complications of the patient’s gastrointestinal cancer; however, these are not related to mass effect. Cancer of the pyloric region would not be likely to cause mass effect that leads to decreased bowel sounds. This symptom is more likely to be associated with obstruction of the left colon.

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2
Q

A 40-year-old man comes to the physician because of a 2-week history of gradually worsening fatigue, nausea, vomiting, and abdominal distention. Physical examination is remarkable for scleral icterus; sublingual jaundice; a nodular, palpable liver; and a flapping tremor with wrist extension. Abdominal imaging reveals a large, irregular lesion in the caudate lobe of the liver.

Which of the following is most representative of the severity of organ involvement?

A. Decreased estrogen level

B.Decreased level of circulating von Willebrand factor

C.Elevated transaminases

D.Increased β-human chorionic gonadotropin level

E.Increased prothrombin time

F.Increased serum albumin level

A

Increased prothrombin time

Chronic liver disease can cause a deficiency in vitamin K-dependent clotting factors, leading to an increased prothrombin time.

others

Although liver enzymes (eg, AST and ALT) are often elevated in chronic liver disease, they are not indicative of liver function and therefore not best representative of severity of organ involvement. Liver failure would not cause decreased estrogen levels, but rather would result in increased estrogen levels. Likewise, serum albumin levels would decrease, not increase, in chronic liver disease. Levels of von Willebrand factor and β-human chorionic gonadotropin levels are not generally affected by hepatocellular carcinoma or liver disease.

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3
Q

A 50-year-old woman comes to her physician’s office complaining of headaches that are localized on the right side of her head. The pain increases when she gets out of bed in the morning. Her symptoms have gradually worsened over the past 6 months. MRI demonstrates a tumor attached to the dura mater. She is referred to a neurosurgeon who resects the mass, confirming that the tumor arises from the arachnoid.

Which of the following histologic features of this tumor is most likely to be observed?

A

Psammoma bodies

Psammoma bodies can be found in meningiomas, which arise from meningothelial cells of the meninges. They are typically found in middle-aged women, and cause symptoms of headache and focal neurologic defects.

other options

Oligodendrogliomas are characterized by cells with a “fried egg” appearance, due to the presence of dark nuclei within a small amount of cytoplasm. Ependymomas demonstrate perivascular pseudorosettes that represent round-to-oval nuclei and long, delicate processes around vessels. Glioblastoma multiforme cells have a pseudopalisading appearance. Medulloblastomas are indicated by hyperchromatic nuclei with scant cytoplasm, appearing as small blue cells.

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4
Q

A 2-year-old boy is brought to a clinic because of a painless abdominal mass that his mother noticed while bathing him. She explains that the patient has been eating well and producing normal amounts of urine and stool. Development is normal and the patient has met all of his milestones. On evaluation today, vital signs are within normal limits. Physical examination is normal without focal deficits, but a right abdominal pass is palpated. While performing an ultrasound, the technician notes that the kidney calyces on the right are highly distorted by a mass. A CT scan of the child’s abdomen is shown below.

Which of the following is the cause of this patient´s most likely diagnosis?

A

Abnormal proliferation of the metanephric blastema

Wilms tumor originates from embryonic renal cells, metanephros, and manifests as a unilateral palpable mass in a young child. It is the most common renal malignancy in children age 2–4 years.

There are many mutations associated with Wilms tumor such as WT1, FWT1m FWT2 and p53.
In the case of WT1 mutation, Wilms tumor can also be associated with WAGR syndrome and Denys-Drash syndrome. It is associated with the deletion of tumor suppressor gene WT1 on chromosome 11.
Hemihypertrophy is seen with Beckwith-Wiedemann syndrome, a syndrome associated with Wilms tumor. Yet hemihypertrophy is not a feature of sporadic Wilms tumor.

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5
Q

A 69-year-old man complains that he has been experiencing dizziness, tinnitus, and poor balance for the past 3 months. On CT scan he is found to have a 2-cm, sharply circumscribed mass at the cerebellopontine angle. The mass is surgically removed, and the specimen appears as multiple irregular fragments of tan-pink soft tissue. A photomicrograph of the specimen is shown below.

Which of the following tumors would most likely result in these findings?

A

Schwannoma

Schwann cell tumors often affect cranial nerve VIII (and are called acoustic neuromas), and thus are commonly seen at the cerebellopontine angle. The most common signs and symptoms of schwannomas include hearing loss, tinnitus, vertigo, hydrocephalus, and increased intracranial pressure.

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6
Q

A 46-year-old woman comes to her primary care physician after visiting the optometrist. While being fitted for new glasses, the patient was found to have persistent constriction of the right pupil. The patient also complains of a chronic nonproductive cough for the past 4 months.

She has a temperature of 37.3° C (99° F), her heart rate is 78/min, and her respiratory rate is 18/min. Her blood pressure is 135/78 mm Hg, and pulse oximetry is 97%. There is marked drooping of the right eyelid. An X-ray of the chest is ordered, and results are shown.

A

Second-order (preganglionic) sympathetic neuron

The sympathetic nerve fibers of the inferior cervical or stellate ganglion may be compressed by Pancoast tumors, leading to Horner syndrome (unilateral ptosis, miosis, and anhidrosis - “PAM is horny”). Pancoast tumors may cause hoarseness (compress recrrent laryngeal nerve in neck). In addition, a right-sided Pancoast tumor may cause SVC syndrome (distended neck veins and right arm swelling).

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7
Q

A 57-year-old man presents to his physician with skin lesions on his ear. These lesions are removed and sent for pathologic examination, but results indicate they are not cancerous. Within 6 months of removal, one of the lesions reappears, and is shown in the image below. The physician explains to the patient that he may be at risk for developing a type of skin neoplasm that carries a risk of metastasis.

Which of the following is a feature of the malignancy this patient is at risk for developing?

A

Keratin formation in well-differentiated cases

Actinic keratosis is the most common precancerous dermatosis and can progress to squamous cell carcinoma. Histologically, well-differentiated SCCs can have keratinized pearls or bridges.

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8
Q

A 16-year-old girl comes to her dermatologist with a skin lesion on her forearm (shown in the image). She has had numerous skin lesions similar to this one removed since childhood. While in the office, her dermatologist performs a brief physical examination that, in addition to the skin finding, reveals an unsteady gait and signs of numbness in the extremities.

Which of the following is most likely to be found in this patient?

A

Defective nucleotide excision repair

Xeroderma pigmentosum (familial dyspllastic melanocytic nevi) is caused by a defect of nucleotide excision repair proteins. It is associated with a 2000-fold increased risk of skin cancer.

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9
Q

An otherwise healthy 8-year-old boy is brought to the pediatrician because of headaches during the past 7-10 days. Physical examination shows normal vital signs. However, the patient is in the 5th percentile of the growth curve, even though he was in the 60th percentile last year. Neurologic examination reveals the patient is not able to notice the movement of the physician’s fingers in the temporal visual field of both sides. The rest of the physical examination is unremarkable. A non-contrast CT scan of the head is shown.

Which of the following laboratory findings is most likely in this patient?

A

Decreased growth hormone level

Craniopharyngioma is the most common childhood supratentorial tumor and presents with headaches and bitemporal hemianopia. It is derived from remnants of Rathke’s pouch and can appear as a calcified cyst on imaging. In normal development, Rathke’s pouch gives rise to the anterior pituitary gland - deficiencies of the anterior pituitary hormones, such as growth hormone, are responsible for the symptoms of growth failure, hypothyroidism, and diabetes insipidus often seen in these patients.

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10
Q

A 35-year-old man comes to his primary care provider because of a 2-month history of fever, a 7-kg (15-lb) weight loss, and night sweats. Physical examination shows painless generalized lymphadenopathy. Cytotoxic chemotherapy is initiated; however, 4 days into his treatment the patient develops severe pain in his left great toe, which is accompanied by nausea. He also has developed a rash and now has multiple excoriations on his extremities. Results of laboratory studies are shown.

Na+: 140 mEq/L
K+: 6.5 mEq/L
Ca2+: 7.5 mEq/L
Phosphorus: 5.1mg/dL (normal: 3.0 - 4.5mg/dL)
Uric acid: 23 mg/dL (normal: 3.5 - 8.0 mg/dL)
Lactate dehydrogenase: 5000 U/L (normal: 118 - 273 U/L)

Given this patient’s current presentation, he is at greatest risk for which of the following complications?

A

Cardiac arrhythmia

Tumor lysis syndrome (TLS) is a common result of cytotoxic chemotherapy for lymphoma/leukemia and bulky cancers. It can cause excess potassium and low calcium (leading to weakness and arrhythmias) and excess uric acid (leading to gout and renal failure). TLS is treated with allopurinol, a xanthine oxidase inhibitor, or rasburicase, an exogenous enzyme that converts uric acid to allantoin which is then excreted in urine.

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11
Q

A 57-year-old woman who was diagnosed with lung cancer 4 months ago presents with weight gain. She reports that her weight gain has caused her clothes to fit tighter around the waist. Additionally, she has noticed purple streaks on the skin of her abdomen, and her face has recently “ballooned.” Physical examination shows facial hair above the upper lip, fatty tissue deposits between the shoulders, and muscular atrophy of her arms and legs.

Which of the following additional symptoms is this patient most likely experiencing?

A

Poor wound healing and facial plethora

Cushing syndrome is characterized by weight gain, abdominal striae, moon facies, poor wound healing, and facial plethora. Small cell lung carcinoma can cause paraneoplastic syndromes including Cushing syndrome, SIADH, and Lambert-Eaton myasthenic syndrome.

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12
Q

A 42-year-old woman (gravida 1, para 1) presents to her physician with an unintentional weight loss of 15 lb over the past 3 months. She reports that her appetite has increased, and she has had to change shirts more often because of frequent perspiration. Physical examination reveals a pelvic mass that is tender to palpation. On further workup, the mass is resected and sent for pathologic examination. The specimen is shown in the image.

Which of the following is the most likely diagnosis?

A

Struma ovarii

A biopsy specimen of a struma ovarii, a monodermal teratoma, shows mature thyroid follicles. A minority of patients present with symptoms of hyperthyroidism and are at risk for malignant thyroid carcinoma.

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13
Q

A 26-year-old man visits a physician because of progressively worsening vision in his right eye and slightly decreased balance over the past few months. Ophthalmoscopic examination reveals the image shown. MRI of the brain shows a small, vascular mass in the cerebellum. The physician also finds a cavernous hemangioma on the patient’s right arm. The patient notes that his father suffered from a similar decline in vision and was treated surgically.

Which of the following symptoms is the patient most likely to develop in the future?

A

Hematuria

Von Hippel-Lindau disease, an autosomal dominant disorder caused by a deletion on chromosome 3, is associated with hemangioblastomas (in the retina, brainstem, and cerebellum). It causes renal cell carcinoma in 50%–60% of cases.

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14
Q

A 50-year-old man who has a 50 pack-year smoking history comes to his physician because of flank tenderness. He has no history of signifcant illness. Family history is significant for myocardial infarction in his paternal grandfather at age 76 years, and breast cancer in his sister at age 58 years. On physical examination, the patient has flank tenderness and a palpable abdominal mass. A CT scan is shown.

Which of the following is the most likely diagnosis?

A

Renal cell carcinoma

Renal cell renal carcinoma is the most common renal malignancy overall, and usually presents with flank pain and hematuria. Its highest incidence is in men between 50 and 70 years of age. Be aware that Wilms’ tumor is the most common cause of renal malignancy in children.

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15
Q

A 59-year-old man comes for evaluation because of intermittent dysphagia during the past year. He reports a 30-year history of postprandial heartburn and regurgitation, and he states his symptoms are worsened when lying down or leaning forward. He denies smoking or alcohol use. His family history is negative for malignancy. He undergoes an endoscopic study of the esophagus. A biopsy specimen is obtained from a red, irregular lesion in the distal esophagus.

Which of the following pathologic findings on examination of the lesion is most likely?

A

Mucin-positive cells

Barrett esophagus describes intestinal metaplasia of the distal esophagus. In Barrett esophagus, the normal squamous epithelium changes to columnar epithelium with mucin-positive goblet cells.

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16
Q

A 15-year-old high school student visits an outpatient clinic with a 3-day history of sore throat and fever. He says he has pain with swallowing but denies any fatigue, nasal congestion, or cough. He is the quarterback on his high school football team and is eager to recover so he can play in an upcoming game.

His blood pressure is 118/80 mm Hg, temperature is 38.5°C (101.3°F), pulse is 56, and respiratory rate is 13. A physical examination reveals creamy, white discharge on both tonsils and a bright red oropharynx. There is no cervical lymphadenopathy or splenomegaly. The lungs are clear to auscultation bilaterally.

Treatment of this infection will decrease the likelihood of development of which of the following complications?

A

Mitral stenosis

Rheumatic heart disease is a potential complication of S. pyogenes pharyngitis. It can cause valve leaflet thickening, commissural fusion, and mitral valve stenosis, which can be avoided with appropriate treatment.

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17
Q

A 78-year-old woman presents to her primary care provider with a 6-week history of headaches and blurred vision in her right eye. She first noticed blurring of her vision while reading a book at night in low light. Since then, she has noticed worsening of both symptoms while reading the daily newspaper or any fine print. The patient also reports symptoms of general fatigue and stiffness and pain in her neck and shoulders on awakening, which she has had for the past month. On physical examination, she appears fatigued and is found to have a low-grade fever. Muscle strength is 5/5 in all areas.

What is the most appropriate treatment for this patient’s condition?

A

Corticosteroids

Giant cell (temporal) arteritis (GCA) is a granulomatous inflammatory disease characterized by headaches and blindness secondary to inflammation of the posterior ciliary and ophthalmic arteries (branches of the internal carotid). Generally, 50% of patients presenting with GCA also have polymyalgia rheumatica. Corticosteroid treatment should be started when either condition is first suspected, especially in the case of GCA because of the high risk of blindness associated with this condition.

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18
Q

A 52-year-old assistant who spends much of her time typing presents to the clinic complaining of chronic and sharp pain in her right finger joints over the past year. Her finger joints are stiff for approximately 30 minutes in the morning. The stiffness generally improves with rest and worsens with activity. She denies fevers, weight loss, fatigue, and weakness. Physical examination reveals bony growth at her distal and proximal interphalangeal joints. A grating sound is heard with active and passive movements of her fingers. The rest of her physical examination is unremarkable.

Which laboratory values in the chart are most consistent with this patient’s condition?

A

Hb 13.4, MCV 95

Osteoarthritis is a chronic noninflammatory joint disease. Risk factors include female gender, obesity, age >50 years, and chronic mechanical trauma. Osteoarthritis presents asymmetrically with joint pain that worsens with activity and improves with rest; distal interphalangeal joint involvement is characteristic. It does not cause abnormalities in hemoglobin level or corpuscular volume.

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19
Q

A mother brings her 5-year-old son to his pediatrician’s office. The boy has been experiencing frequent falls and has demonstrated progressive difficulty walking, jumping, and hopping. Muscle biopsy reveals variation in fiber diameter, an increased number of internalized nuclei, and muscle fiber degeneration and regeneration. A Western blot analysis confirms the diagnosis.

Which of the following diseases demonstrates the same inheritance pattern as the condition described above?

A

Glucose-6-phosphate dehydrogenase deficiency

Both Duchenne muscular dystrophy (DMD) and glucose-6-phosphate dehydrogenase (G6PD) deficiency are inherited in an X-linked recessive pattern.

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20
Q

An 81-year-old man presents to the emergency department with acute back pain after losing his balance and falling while getting out of his car. He also reports a 2-month history of dull, constant lower back pain and difficulty maintaining a stream while urinating. The patient also notes that his clothes have been looser and that he has lost some weight over the past few months. X-ray of the spine is shown.

Which of the following is the most likely diagnosis?

A

Metastatic adenocarcinoma

In older men with back pain and symptoms of prostate cancer, metastasis to the bone should be considered and is far more common than primary cancers of bone.

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21
Q

An 8-year-old boy is brought to the pediatrician by his parents because of swelling and pain over his right femur, which has been present for the past 2–3 weeks. The child and parents deny any history of trauma to the region. The patient reports the pain often is worse at night, and his mother states he has been having low-grade fevers of 37.8°C–38.1°C (100.0°F–100.6°F) during this period. There is no erythema over the region, but examination reveals a firm and immobile mass that is tender to palpation over the proximal right lower extremity near the greater trochanter. Results of a complete blood count show:

Red blood cells (x 10^6/microL): 4.5

Hemoglobin (g/dL): 12.9

Hematocrit (%): 38.2

MCV (fL): 78

MCHC (%): 34

White blood cells (x 10^3/microL): 9.5

Neutrophils (%): 53

Lymphocytes (%): 39.5

Cytologic study of a specimen obtained on fine-needle aspiration reveals sheets of small, round, uniform cells with scant clear cytoplasm, divided into irregular lobules by fibrous strands.

Genetic evaluation of this patient is most likely to reveal which of the following?

A

t(11;22) chromosomal translocation

Ewing sarcoma is diagnosed histologically with blue anaplastic cells and is associated with t(11;22) translocations.

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22
Q

A mother brings her 3-year-old son to the pediatrician because she is concerned about his increasing weakness and clumsiness. Although he met all of his early motor milestones as a baby, the mother recalls that he was not able to walk on his own until 18 months of age. He is now having increasing difficulty keeping up physically with his peers during playtime. On examination, the child’s cognitive development is appropriate for age.

Examination of the patient’s lower extremities reveals normal patellar and Achilles reflexes, normal sensation, and 3/5 muscle strength. His calf muscles are enlarged (see image for example). Biopsy of the muscle reveals wide variation in the diameter of individual muscle fibers, with degenerative necrosis of some and interspersed regenerating fibers.

Although the mother has not experienced the symptoms seen in her son, she states that she had a “sickly” brother who died at a young age after being confined to a wheelchair for some time. Her husband does not have any history of muscle weakness in his family. The mother is now 30 weeks pregnant with a baby girl and is concerned that this child may have the same health issues as her brother.

Which of the following is the probability that the same condition causing this patient’s weakness will develop in his sister?

A

Negligible

Duchenne muscular dystrophy is inherited in an X-linked recessive pattern characterized by bulging, fatty calf muscles manifesting in early childhood

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23
Q

A 28-year-old African American woman comes to her physician for evaluation because of a 1-month history of fevers and painful joints. The patient takes no medications. Temperature is 37.8°C (100°F). Physical examination shows an erythematous rash bilaterally on her cheeks. Basic metabolic panel reveals the laboratory values shown.

Additional laboratory studies show a positive Venereal Disease Research Laboratory test and an antinuclear antibody test titer of 1:2560.

This patient is most likely to have which of the following additional physical examination findings on follow-up evaluation?

A

Painless erythematous oropharyngeal ulcers

Systemic lupus erythematosus (SLE) often manifests with mucosal ulcerations that are usually painless. Patients with SLE often have a false-positive VDRL.

24
Q

A 12-year-old boy is brought to the pediatrician’s office by his mother because he is experiencing leg pain. The pain is located 2 inches from the tibial tuberosity and has persisted during the past 2 weeks. The mother also notes that the child has had intermittent fevers during this time. On examination, the site of the pain is erythematous and swollen. A biopsy specimen shows sheets of many uniform cells with scant, clear cytoplasm and no evidence of normal bony matrix.

Which of the following is the most likely diagnosis?

A

Ewing sarcoma

Ewing sarcoma is one of the small round blue cell, or primitive neuroectodermal, tumors. It occurs most commonly in white males younger than 15 years. Ewing sarcoma is associated with a t(11;22) translocation and is classically characterized by “onion skinning” of the periosteum.

25
Q

A 52-year-old woman comes to the primary care physician because of blisters on her skin and in her mouth that have become progressively more uncomfortable during the past 3 weeks. She is most frustrated about the painful “raw spots” these blisters leave when they rupture. The patient has a 23-pack-year history of smoking, and currently smokes one pack per day. Her BMI is 21 and she does her best to follow a healthy diet. She has a history of Epstein-Barr virus mononucleosis as a 22-year-old, and recently had a minor viral upper respiratory infection.

Her vital signs include: a heart rate of 87 beats/min; blood pressure of 125/83 mmHg; respiratory rate of 17 breaths/min, and an oxygen saturation of 97%. On examination, tender erosions are found on her back and trunk. One of the lesions is biopsied with direct immunofluorescence against IgG. A light micrograph of the specimen is shown.

Which aspect of epithelial cell junctions is most likely targeted by autoantibodies in this condition?

A

Desmosomes

Pemphigus vulgaris is an autoimmune blistering disease of the skin and mucosa, caused by antibodies directed against desmosomes (macula adherens). Severe cases may involve life-threatening infection and thermoregulatory disruption.

26
Q

A 25-year-old woman of short stature sees her physician for genetic counseling prior to conception. She has been told in the past that she has an inherited disorder passed down by one of her parents. She is in good physical health and has no cognitive impairment.

On physical examination, the patient measures 124 cm (4 ft 1 in) tall. Her limbs are shortened relative to the length of her torso and head and her facial features are symmetric and proportionate with mild frontal bossing. Her spouse does not have a family history of this disease. The geneticist explains the options for genetic testing.

Dysfunctional proliferation of which cell type is most likely responsible for this patient’s disorder?

A

Chondrocytes

Achondroplasia usually manifests with short stature, decreased limb length, and normal intelligence. It is caused by abnormal endochondral ossification of long bones due to the impaired activity of chondrocytes. Intramembranous ossification is unaffected.

27
Q

A 32-year-old woman comes to the physician reporting that lately she has felt “more fatigued, especially at the end of the day.” In the office, she has difficulty rising from her chair to move to the examination table. On physical examination, the patient has asymmetric drooping of her left eyelid, a subjective feeling of “blurred vision” when reading the eye chart on the wall, and a facial droop bilaterally. A plain x-ray of her chest is shown.

Which of the following tests is most likely to confirm a diagnosis in this patient?

A

Electromyography

Myasthenia gravis (MG) is an autoimmune disorder characterized by the presence of acetylcholine receptor antibodies. Thymomas occur in approximately 10% of patients with MG. Electromyography can be used to confirm the diagnosis

28
Q

An 87-year-old woman visits her primary care physician for a routine physical examination. She has a history of compression fractures secondary to minor trauma and most recently sustained a wrist fracture when she fell while bathing. She has a 35–pack-year history of smoking but quit 25 years ago. The patient has no new symptoms but reports increasing stiffness and joint pain. After noting reduced mobility in her previously affected joints, her physician orders x-ray studies that reveal cortical thinning and increased radiolucency.

What changes in calcium, phosphorus, alkaline phosphatase, and magnesium levels, as listed in the table, would be expected?

A

Calcium nomal, phosphorus normal, alkaline phosphatase normal, magnesium normal. All normal

Osteoporosis is characterized by loss of bone mass despite normal bone mineralization. It is most common in elderly women and does not produce gross metabolic abnormalities.

29
Q

A 12-year-old boy is brought to the pediatrician with the complaint of high spiking fevers for the past several weeks. The fevers are periodic, with his temperature returning to normal between episodes. He has had a flat rash with small, salmon-pink colored spots present on his axillae and around his waist, which seems to get worse as his fevers worsen. He does not have a history of travel outside the United States, and does not report weight loss, respiratory symptoms, diarrhea, or constipation. Vital signs are: temperature 38.0°C (100.4°F), pulse 89/min, respiratory rate 16/min, blood pressure 104/60 mm Hg, and oxygen saturation 98%.

Upon abdominal examination, percussion of the liver reveals a span of 14 cm, and the spleen is palpable 10 cm below the bottom of the rib cage. The cervical, axillary, and inguinal nodes are enlarged. Peripheral blood smear shows normal RBCs and RBC-laden macrophages. Laboratory tests show:

RBC count: 1,700,000/mm³
Hematocrit: 18%
Hemoglobin: 5.2 g/dL
WBC count: 30,000/mm³

Which of the following is another typical finding in this boy’s illness?

A

Polyarticular joint pain and swelling

Systemic juvenile idiopathic arthritis (JIA), the most common arthritis in the pediatric population, often presents with joint pain/limping, spiking fevers, and swelling and rash. Treatment includes nonsteroidal anti-inflammatory drugs, steroids, methotrexate, and intravenous immune globulin, as well as some newer, biologic drugs.

30
Q

A previously healthy 23-year-old woman is brought to the emergency department after she was involved in a motor vehicle crash that left her with multiple lower extremity fractures. About 48 hours after being admitted to the hospital, she begins to have some trouble breathing and seems confused. Her blood pressure is 92/45 mm Hg, pulse is 128, respiratory rate is 28, and oxygen saturation is 92%. On physical examination, no penetrating injury to the chest is detected, but some petechiae are found on her neck. An embolus in the pulmonary circulation is suspected.

Which of the following is the most likely type of embolus in this patient?

A

Embolus that stains positively with Sudan black

The presence of a fat embolus should be considered highly likely in patients with evident or suspected long bone fractures and respiratory distress after trauma

31
Q

A 62-year-old white woman comes to her family doctor because of of lower back pain located over the spine. When asked about the onset of pain, she does not recall any recent trauma. She has had no significant weakness, sensory changes, numbness, or tingling. She additionally denies any recent fevers, night sweats, or weight loss. Her past medical history is significant for severe osteoarthritis of the knees for the past 10 years, which has significantly limited her physical activity. She otherwise has no chronic medical problems and takes only ibuprofen to help with her knee pain.

Which of the following sets of serum laboratory findings is most likely in this patient? (calcium, phosphorus and alkaline phosphatase levels)

A

Clacium, phosphorus and alkaline phosphatase all normal

Osteoporosis describes loss of bone mass with a risk of vertebral compression fractures. It may present with acute back pain, loss of height, and increased kyphosis. Osteoporosis is associated with normal calcium, phosphorus, and alkaline phosphatase levels.

32
Q

A 57-year-old man presents to his primary care physician with a report of sudden-onset, severe pain in his knee, which has been present for the past day. He denies any history of trauma. However, he does mention that he has been binge drinking alcohol over the past 2 days. On physical examination, the physician observes edema with erythema. Severe pain is elicited on movement of the left knee joint, but there is no overlying skin ulceration. An x-ray of the knee shows subcortical bone cysts.

Which additional joint aspirate finding would be most characteristic of the underlying disease process?

A

Negatively birefringent needle crystals

Gout is caused by urate crystals and results in painful swollen joints (often the first metatarsophalangeal joint). The presence of negatively birefringent needle crystals in the joint aspirate is diagnostic.

33
Q

A 68-year-old woman with a medical history notable for obesity and osteoporosis presents to her primary care physician with ongoing pain in her knees. She describes the pain as a deep ache that worsens throughout the day, especially on days when she works in her garden. Rest and nonsteroidal anti-inflammatory drugs (NSAIDs) relieve her pain.

The patient denies recent trauma, prolonged morning stiffness, joint erythema, or systemic symptoms. She currently takes supplements to treat her osteoporosis. Physical examination is remarkable for some crepitus in the right knee and mild tenderness with effusions bilaterally. Active and passive range of motion are restricted in both knees.

What structural changes might one expect on histologic inspection of the joint space of this patient’s knees?

A

Dislodged pieces of cartilage and subchondral bone

Osteoarthritis is a chronic disease of degenerating joints in which pieces of cartilage and bone are broken off into the joint space, causing pain and restriction of joint movement. Unlike other pathologic joint processes, no typically frank histologic evidence of inflammation is associated with osteoarthritis.

34
Q

A 17-year-old boy with an uncomplicated past medical history presents to his pediatrician with pain in his right leg of several months duration. The pain is localized above the right knee and waxes and wanes, sometimes worsening at night. He denies any trauma, fatigue, malaise, or generalized weakness. Anti-inflammatory medications have failed to provide long-term relief. Of note, his family history is relevant for an unspecified ocular cancer in his younger sister.

On physical examination the patient appears well and has a firm soft-tissue mass superior to the right knee that is tender to palpation. The mass is biopsied, revealing a malignant bone tumor of the distal femur.

Which of the following is the most likely diagnosis?

A

Osteosarcoma

Genetic mutations of the Rb gene are associated with both osteosarcoma and retinoblastoma.

The diagnosis of ocular cancer in the patient’s sister should raise suspicion for a genetic predisposition to malignancy involving the retinoblastoma (Rb) gene. In this mutation, the two-hit hypothesis explains that each patient has a defective “retinoblastoma” (Rb) tumor-suppressor gene and thus only requires one somatic mutation to become fully nonfunctional and cancerous.

35
Q

A 68-year-old man presents to his physician’s office with diffuse pelvic pain of 2 weeks’ duration. He reports that it started gradually and that the aching quality of the pain has been worsening. The pain does not seem to be related to activity but is greater at night. The patient has no history of arthritis; he has tried taking ibuprofen and acetaminophen, but they do not relieve his pain.

On further questioning, he admits to an unintentional weight loss of 10 lb in the past 2 months and straining and a weak stream during urination; he has also noticed some blood in his urine. He denies any recent trauma or shooting radiculopathy. On physical examination, the area over the lumbar spine and several pelvic areas areas are tender to palpation. X-ray of the lumbar spine and pelvis is shown in the image.

Which of the following is the best next step to determine the source of this patient’s symptoms?

A

Digital rectal examination

Prostate cancer commonly produces osteoblastic lesions on metastasis. A digital rectal examination is used to detect the presence of prostate cancer.

A helpful mnemonic for remembering the cancers that commonly metastasize to bone is: lead (Pb) Kettle. Prostate; breast; Kidney, thyroid, lung.

36
Q
A
37
Q

A 15-year-old boy is brought by his parents to a neurologist because he is having increased difficulty maintaining his balance and normal gait. The patient states that he has been “feeling weaker” than normal, especially when trying to climb stairs, rise from a chair, or brush his teeth. His mother reports that one of her brothers also had similar symptoms that developed during his teenage years. Physical examination reveals that the patient has difficulty rising from a chair to a standing position.

Laboratory studies reveal a serum creatine kinase level of 13,021 IU/L. The aspartate aminotransferase (AST) level is 43 U/L, and the alanine aminotransferase (ALT) level is 44 U/L. Lactate dehydrogenase and aldolase levels are also elevated. The neurologist suspects this boy has an inherited condition.

What type of genetic mutation is most likely responsible for this patient’s symptoms?

A

In-frame deletion

BMD (Becker muscular dystrophy) manifests with muscle weakness and/or cardiac disease beginning in adolescence. It is caused by DMD mutations, such as in-frame deletions (eliminate codons without altering the reading frame), and the resulting dystrophin protein retains some residual function.

Patients with BMD usually have increased lactate dehydrogenase and aldolase levels, as this patient does. AST and ALT levels are also elevated, signifying muscle disease. BMD is a milder form of Duchenne muscular dystrophy (DMD).

38
Q

A 52-year-old man presents to his primary care physician for an annual well-patient examination. Fasting blood work completed the previous week is notable for an LDL-cholesterol level of 125 mg/dL. The patient says that his father died from a heart attack at 55 years of age. His vital signs are within normal limits except for a blood pressure of 142/82 mm Hg. The physician prescribes a medication to the patient to reduce his risk of having a myocardial infarction. Three months later, the patient presents to the emergency department with complaints of severe muscle aches and a darkening of his urine. Laboratory studies consequently show that the patient has a blood urea nitrogen level of 20 mg/dL and a creatinine level of 1.4 mg/dL. A urine dipstick test is also positive for blood, but microscopy reveals no RBCs in the high power field.

Which of the following is the most likely cause of the patient’s increased creatinine level?

A

Acute tubular necrosis

Statin use can lead to rhabdomyolysis and myoglobinuria (myoglobin in blood acts as nephrotoxin) , resulting in acute tubular necrosis.

39
Q

A 55-year-old woman comes to the physician because of pain and tenderness in the joints of her hands that is at its worse in the morning and lasts until lunchtime. She also says her hands feel stiff for approximately 30 minutes after she wakes up. On examination, these joints appear swollen and erythematous and seem to have an ulnar deviation. Her temperature is 98°F (36.7°C). Laboratory studies show an erythrocyte sedimentation rate (ESR) of 60 mm/hr.

Which of the following HLA subtypes has been associated with this condition?

A

HLA-DR4

HLA-DR4 is strongly associated with rheumatoid arthritis as well as type 1 diabetes mellitus. Classic RA symptoms include include morning stiffness, joint pain, an elevated ESR, and possible ulnar deviation when the hand joints are affected.

40
Q

A mother brings her 2-month-old daughter to the emergency department because of lethargy and a fever of 39.2°C (102.6°F). The mother is also concerned because the infant is not gaining weight.

Physical examination reveals that the infant has an increased head circumference and prominent hepatosplenomegaly. Mastoid and paranasal sinus malformations are also noted during the course of the workup. Results of laboratory tests are as follows:

HGB: 7.9 g/dL,
Hct: 28%,
WBC: 373/μL,
MCV: 78.2
Reticulocyte count: 6.5%

Despite fluid resuscitation and initiation of antibiotic therapy, the infant dies. On autopsy, histologic analysis of the bone marrow reveals a lack of a medullary canal, with persistence of the primary spongiosa, and deposition of dense, sclerotic bone.

Which malfunctioning cells are the cause of this patient’s disease process?

A

Osteoclasts (malfunctioning)

Osteopetrosis is due to a failure of bone resorption and remodeling secondary to malfunctioning osteoclasts and the consequently unbuffered action of osteoblasts. This disorder presents with pancytopenia, anemia, and compression of cranial nerves from bony overgrowth.

41
Q

A 42-year-old woman presents to her primary care physician reporting that she has had increasing pain and swelling of the joints in her hands and feet for several months. Her pain is worse for the first hour in the morning, which makes getting ready for work nearly impossible. However, at the end of the day, she is able to continue to type on the computer and go to the gym without the same pain and stiffness. She also states that it is becoming very difficult for her to perform common household tasks. Her vital signs are within normal limits. On physical examination, she has symmetric swelling of the joints of her hands and feet. An x-ray of both wrists obtained from a patient with a similar presentation is shown in the image.

Which of the following laboratory serologic findings would most likely be positive in this patient?

A

IgM antibody against Fc portion of IgG

Most cases of RA (Rheumatoid arthritis) involve accumulation of rheumatoid factor (IgM anti-IgG), which prompts an influx of inflammatory cells into the synovium.

42
Q

A 66-year-old man comes to his primary care physician because of a 2-month history of back pain. He has no history of trauma and cannot recall straining his back at any point. He denies recent weight loss, night sweats, and fevers, as well as increased urinary frequency; has no hesitancy or pain on urination.

Physical examination reveals a negative straight leg raise test and age-appropriate range of motion. The patient does not smoke and drinks alcohol only on rare occasions. He reports recently having some difficulty with his hearing. An x-ray of his pelvis shows enlarged, cancellous bone with thick coarsened cortices.

Which of the following sets of laboratory values would be expected in this patient? (calcium, phosphate and alk phos levls)

A

Normal calcium, normal phosphate, elevated alkaline phosphatase

Paget disease of the bone has both osteolytic (osteoclasts) and osteosclerotic (osteoblasts) stages, resulting in increased bone mass and a mosaic pattern of lamellar bone. Paradoxically, this heavy bone is more prone to fracture, and alkaline phosphatase is commonly elevated. Patients with Paget disease will have a normal phosphate level and, in most cases, a normal calcium level (elevations occur rarely).

43
Q

A 35-year-old man presents to the emergency department with multiple rib fractures after a low-speed motor vehicle collision. No one else involved in the crash was injured. Laboratory findings are as follows.

Calcium: 15.3 mg/dL
Phosphate: 1.8 mg/dL
Alkaline phosphatase: 172 IU/L.

A radiograph of the patient’s femur is shown below.

A

Osteitis fibrosa cystica

Osteitis fibrosa cystica may be diagnosed with hypercalcemia, elevated alkaline phosphatase, hypophosphatemia, and cystic lesions on x-ray.

Patient presenting with multiple fractures from a minor accident, in the setting of high serum calcium and low phosphate, is most likely suffering from osteitis fibrosa cystica, or von Recklinghausen’s disease of the bone.

44
Q

A 22-year-old man presents to the neurologist’s office because he is experiencing persistent muscle weakness. Over the past 3 months, he has had difficulty walking and diffuse weakness that has been getting worse. He has also noticed that his vision is blurry in both eyes. His father, who is 55, currently has similar symptoms, which began about 5 years ago. Vital signs include a blood pressure of 119/65 mm Hg and a heart rate of 76. Physical examination reveals considerable muscle wasting, myotonia, and bilateral cataracts. The neurologist also has the patient walk and notices a wide-stepping gait.

Which of the following is the inheritance pattern of the disease this patient most likely has?

A

Autosomal dominant

Myotonic dystrophy is an autosomal-dominant disorder characterized by muscle wasting, a wide-stepping gait, and bilateral cataracts. It is caused by a CTG trinucleotide repeat and exhibits anticipation, whereby the number of CTG repeats can increase across generations and cause symptoms at younger ages.

45
Q

A 32-year-old homeless man presents to the emergency department after falling down a flight of stairs. He has a newly fractured right wrist. However, additional x-rays indicate that he has other fractures, which actually occurred before the fall. He denies any other falls in the past. Laboratory studies indicate:

Calcium: 9.8 mg/dL
Phosphorus: 2.1 mg/dL
Alkaline phosphatase: 98 U/L
Vitamin D: 10 ng/mL

Which of the following histologic findings would corroborate the most likely diagnosis for this patient?

A

Areas of increased unmineralized osteoid adjacent to normal trabeculae

Osteomalacia is found in malnourished adults with vitamin D and calcium deficiencies. The lack of calcium and phosphorus causes osteocytes to produce less mineralized bone surrounding the cells. It may present with pathologic fractures or bone pain and a waddling gait.

46
Q

A 24-year-old man has sensorineural hearing loss, heart block, cerebellar ataxia, easy fatigability, and proximal muscle weakness. He was diagnosed with a myopathy in childhood. His mother, sister, and a maternal aunt were known to have a similar syndrome. No one on his father’s side is known to be affected. A muscle biopsy is performed (see image).

What is the most likely mode of inheritance for this man’s disorder?

A

Mitochondrial

The ragged red fiber myopathies (deletion of mitochondria DNA) are inherited through the mother, because all of the embryo’s mitochondria are provided via the ovum.

47
Q

A 7-year-old boy has been experiencing progressive muscle weakness since he was 3 years old. When rising from a seated or lying position, the boy requires assistance from his upper extremities. His serum creatine phosphokinase levels are markedly elevated. The boy’s uncle had the same symptoms and died when he was 23 years old.

What type of mutation is most likely responsible for this patient’s condition?

A

Deletion

DMD (Duchenne muscular dystrophy), characterized by progressive muscular weakness starting before the age of 5 years, is usually due to a frameshift mutation in the dystrophin gene.

48
Q

A 65-year-old man comes to the physician with a 6-month history of intermittent headaches. In the past few weeks, the patient has had two brief episodes of vision loss in his right eye lasting approximately 10–15 seconds per episode. The patient had an appendectomy when he was in college and takes vitamin D supplements in the winter. On examination today, his temperature is 98.6°F (37°C), blood pressure is 130/80 mm Hg, pulse is 70/min, and respiratory rate is 16/min. A complete blood cell count shows:

WBC: 19,300/mm3
Hemoglobin: 15.5 g/dL
Platelet count: 1,020,000/mm3

A blood smear is shown.

Item: 1 of 10

QID: 2665.22

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A 65-year-old man comes to the physician with a 6-month history of intermittent headaches. In the past few weeks, the patient has had two brief episodes of vision loss in his right eye lasting approximately 10–15 seconds per episode. The patient had an appendectomy when he was in college and takes vitamin D supplements in the winter. On examination today, his temperature is 98.6°F (37°C), blood pressure is 130/80 mm Hg, pulse is 70/min, and respiratory rate is 16/min. A complete blood cell count shows:

WBC: 19,300/mm3
Hemoglobin: 15.5 g/dL
Platelet count: 1,020,000/mm3

A blood smear is shown.

This patient most likely has which of the following disorders?

A

Essential thrombocythemia

Essential thrombocythemia is a myeloproliferative disorder characterized by increased platelets in the peripheral blood and increased megakaryocytes in the bone marrow. Patients may be asymptomatic, or they may present with signs and symptoms of increased bleeding and/or thrombosis.

49
Q

A 64-year-old man comes to the emergency department because of a 2-day history of difficulty walking and extreme pain around his right ankle. He denies any recent falls or trauma to his leg and states that the pain began “all of a sudden.” Medical history is relevant for a recent renal transplantation due to kidney failure. He was recently seen by his physician because he had watery diarrhea after a trip to Mexico and was “given a pill.” On examination, his temperature is 37.7°C (99.9°F), blood pressure is 127/80 mm Hg, pulse is 78, and respiratory rate is 16. His mentation is normal, and he denies any chest pain or shortness of breath. The patient is unable to plantar flex his right foot. There is also a large, soft, muscular mass on the proximal portion of his right calf, and he reports exquisite tenderness around the right ankle when reflexes are tested.

Which of the following medications did this patient most likely receive to treat his infection?

A

Ciprofloxacin

Fluoroquinolones cause tendinitis, particularly Achilles tendinitis. People at increased risk for fluoroquinolone-induced tendinitis are those ≥60 years and those with a history of renal disease, hemodialysis, renal transplantation, or long-term glucocorticoid use.

50
Q

A 19-year-old man, employed as a construction worker in Southern California, comes to his primary care physician with a 6-month history of acne vulgaris that has only mildly improved with a strict face-washing regimen and topical antibiotics. Physical examination reveals numerous papules and pustules with surrounding erythema on the face, back, and chest. The doctor prescribes an oral antibiotic that is commonly used for treatment of acne in similar patients. The patient agrees to take the medication as prescribed and continues his routine of working long shifts on the construction site. Four days after he starts taking the prescribed medication, severe blistering develops over his face, the back of his neck, and the backs of his hands. He returns to the physician in a panic.

What is the mechanism of action of the antibiotic that was most likely prescribed for this patient?

A

Reversibly binds to the 30S subunit of bacterial ribosome

Blistering, desquamating rashes are symptoms of photosensitivity reactions, an adverse effect of tetracycline use. Tetracyclines reversibly bind to the 30S subunit of ribosomes, inhibiting protein synthesis. They are used to treat acne.

51
Q

A 63-year-old white man comes to his physician complaining of weakness in his legs. He states that he has had difficulty standing up after sitting down in a chair and when climbing stairs for the past 2 months. On physical examination, his extraocular movements and the muscles of facial expression are intact. There is bilateral weakness of his hip flexors and extensors. His deep tendon reflexes are mildly reduced, and his sensation is normal. The doctor finds no rash, and no family members have had similar symptoms. A biopsy of the rectus femoris is taken and confirms the diagnosis of polymyositis.

What is the most common type of inflammatory marker in this man’s muscle biopsy?

A

Lymphocyte

Polymyositis is a subacute inflammatory myopathy that upon muscle biopsy shows infiltrates of CD8+ T-lymphocytes and macrophages.

52
Q

A 68-year-old male presents to his primary care doctor with several months of progressive muscle weakness, fatigue, eyelid drooping, double vision, and difficulty swallowing. The symptoms are intermittent, and he notices he is usually symptom free after waking up in the morning. He denies recent illness, changes in diet, or travel. The doctor confirms his diagnosis with a serologic assay and prescribes him a daily medication. Two months later he presents to the emergency department with acute-onset difficulty breathing, diarrhea, and tremor. He reports accidentally taking three times his normal dose of medication. He appears diaphoretic and in distress. Blood pressure is 110/80 mm Hg, heart rate 55/min, and respiratory rate 19/min. Pupils are 1 mm and poorly reactive to light. The oropharynx is nonobstructed, and nonerythematous, but excessive salivation is noted. Soft wheezing is heard in all lung fields. Bowel sounds are increased. Cardiovascular and extremity exams are normal.

After management of the patient’s airway, which of the following is most likely to be administered?

A

Atropine

Acetylcholinesterase inhibitor toxicity manifests with Diarrhea, Urination, Miosis, Bronchospasm, Bradycardia, Excitation (muscle twitches), Lacrimation, Sweating, and Salivation (remember the DUMBBELSS mnemonic). It is treated with atropine, a muscarinic antagonist.

53
Q

A 40-year-old man presents to the physician with trouble breathing. He reports that recently he has developed bruising after minor injuries and that he has been feeling weak more often than usual. The patient has a long history of medically managed Crohn disease. Further questions reveal that the patient had an acute episode of gout 3 months ago and was prescribed a medicine to prevent future attacks. Physical examination is notable for multiple ecchymoses. Respiratory rate is 26/min. Laboratory tests show:

Hemoglobin: 5.5 g/dL
Hematocrit: 13%
WBC count: 2200/mm³
Platelet count: 39,000/mm³

Results from a bone-marrow biopsy are revealing (see image).

A

Azathioprine and allopurinol

When used concomitantly, allopurinol (and febuxostat) interferes with the metabolism of azathioprine’s toxic metabolite, 6-mercaptopurine, which can lead to fatal hematologic disorders, such as aplastic anemia.​

54
Q

A 42-year-old woman presents to her physician because of worsening fatigue. The patient also recently noticed occasional difficulty opening her left eye and reports that, although she feels fine when she wakes up, her head “feels heavy” toward the end of the day.

Which of the following is the most appropriate pharmacologic therapy?

A

Pyridostigmine

Myasthenia gravis is an autoimmune condition in which antibodies against the postsynaptic nicotinic acetylcholine receptors lead to fatigable weakness, especially of the facial muscles. It can be treated with acetylcholinesterase inhibitors, such as pyridostigmine or neostigmine.

55
Q

A 60-year-old woman with a history of peptic ulcer disease visits her primary care physician and reports that she has had intermittent joint pain and morning stiffness for the past couple of years. Her hands have been affected the most, and she has recently had difficulty gardening because of the pain. She has no known history of coronary or cerebrovascular disease. On physical examination, both of her knees and hands are tender to palpation, warm, and swollen. An x-ray of her hands is shown in the image. Her physician prescribes a medication for immediate pain relief.

A

Decreased prostaglandin production

Selective COX-2 inhibitors, such as celecoxib, are a type of anti-inflammatory medication, and they block the conversion of arachidonic acid to endoperoxides, leading to decreased levels of prostaglandins and prostacyclin. They are the anti-inflammatory agents of choice for patients who are at high risk for gastrointestinal disturbances but at low risk for cardiovascular disease.

56
Q

A 3-year-old girl is brought to the office by her mother due to discomfort and swelling under her right arm. The patient has no known medical problems. The mother reports that she attends daycare and has a healthy, 5-year-old sister. There is a pet dog and a pet cat in the home. Her temperature is 38°C (100.4°F). Physical examination reveals an enlarged, tender, erythematous lymph node in the right axilla. Close inspection of the skin reveals a series of small linear scratches on the right forearm.

Which of the following pharmacotherapies is most likely to be prescribed for this patient?

A

Azithromycin

The clinical picture of a break in the skin, regional lymphadenopathy, and low-grade fever are highly suggestive of cat scratch disease (CSD), caused by the organism Bartonella henselae. Azithromycin is the antibiotic of choice for CSD.

57
Q

A previously healthy, 27-year-old pregnant woman at 10 weeks’ gestation visited her doctor for a routine physical check-up and was found to have a temperature of 37.2°C (98.9°F) and a blood pressure of 147/90 mm Hg. Physical examination and laboratory studies were unremarkable, and her doctor prescribed an antihypertensive medication. One week later the patient returns to the office complaining of fever, fatigue, and diffuse muscle aches and joint pain. Her blood pressure is 145/86 mm Hg and temperature is 38°C (100.4°F). Physical examination is notable for a scaling, erythematous rash on the patient’s cheeks and the bridge of her nose.

Which of the following best describes the mechanism of action of the drug the patient was mostly likely prescribed?

A

Causes vasodilation of arterioles

Patients with drug-induced lupus often present with generalized myalgias, arthralgias, fever, rash, and serositis. Hydralazine, which is known to cause drug-induced lupus, is an antihypertensive agent used in pregnant patients that acts by directly dilating arterioles by means of vascular smooth muscle relaxation.