Question 1

A 54-year-old man is being evaluated for shortness of breath and cough. His medical problems include rheumatoid arthritis, hypertension, and a history of deep vein thrombosis and pulmonary embolism. He takes multiple medications and has smoked for 30 years. BMI is 34 kg/m². Examination shows dullness to percussion over the right lower lobe. Breath sounds are increased, especially during expiration, over the right lung base compared to the left. Cardiac examination shows regular rate and rhythm with normal S1 and S2. There is no murmur. Moderate peripheral edema is present. Which of the following is the most likely cause of his shortness of breath and lung examination findings?

Accepted Answer

11ec25c8_d136_d027_bc81_3333868b3320_MD0003_00 This patient with shortness of breath, cough, and dullness to percussion with increased breath sounds over the right lower lung field likely has lobar pneumonia causing focal lung consolidation. A normal lung is resonant to percussion, and auscultation at the periphery demonstrates vesicular breath sounds that consist of a quiet inspiratory phase and an almost inaudible expiratory phase. When a portion of the lung is consolidated (eg, lobar pneumonia), the density of tissue/fluid increases and dullness to percussion is detected. In addition, sound conducts more rapidly and efficiently through consolidated lung, resulting in increased intensity of breath sounds and a more prominent expiratory component. More rapid and efficient sound conduction also results in increased tactile fremitus (vibration generated by air movement in the lungs) as well as egophony (sounds like the letter "A" when the patient says the letter "E") in areas of lung consolidation. Crackles are also often heard. (Choice B) The lungs are bilaterally hyperresonant to percussion in emphysema, and the intensity of breath sounds is decreased. Wheezing is commonly present as well. (Choice C) Patients with interstitial lung disease have lungs that are resonant to percussion and breath sounds of normal or decreased intensity. Fine crackles are heard at the end of inspiration. (Choice D) Mucus plugging decreases airflow in the affected part of the bronchial tree resulting in collapse of the downstream lung parenchyma (atelectasis). Breath sounds will be absent in the affected area. (Choice E) Unlike lung consolidation, pleural effusions are characterized by decreased breath sounds and decreased tactile fremitus as the fluid in the thoracic cavity acts to insulate sound and vibration originating in the lungs. As with lung consolidation, dullness to percussion is present due to increased tissue/fluid density compared to normal air-filled lung tissue. (Choice F) Air in the thoracic cavity also acts to insulate sounds originating from the lung; therefore, pneumothorax is characterized by decreased breath sounds and decreased tactile fremitus. Hyperresonance to percussion occurs due to a relatively lower density of air alone compared to normal air-filled lung tissue. Educational objective: Lung consolidation (eg, lobar pneumonia) presents with dullness to percussion, increased intensity of breath sounds, and increased tactile fremitus.

Question 2

A 68-year-old woman is brought to the clinic by her daughter due to severe pain in her fingers.  Her daughter says, "Mom has had horrible problems with her joints for a long time, but she has never tried to get help."  The patient adds that her fingers have become more swollen and painful over the last few weeks.  She had similar symptoms in her foot last year.  She was given an unknown pain pill, but it was ineffective.  Medical history is notable for hypertension, hypothyroidism, and early Alzheimer dementia.  Vital signs are normal.  Examination findings of the hands are shown in the image below.  What is the most likely diagnosis in this patient?

Accepted Answer

This patient with multiple white nodules in the hands and a history of painful arthritis in the fingers and feet has tophaceous gout. In most cases, gout is characterized by hyperuricemia and precipitation of uric acid crystals in the joints, leading to episodic monoarticular arthritis (especially in the first metatarsophalangeal joint and knee). The diagnosis is confirmed with arthrocentesis showing an inflammatory effusion with uric acid crystals. Urate crystals may also deposit in the soft tissues, forming tumors known as tophi. Tophi can ulcerate and drain a chalky material. Uric acid tophi are virtually pathognomonic for gout, and even in the absence of microscopic confirmation of crystals, the diagnosis can be made provisionally in patients with visible tophi and a history of episodic monoarthritis. An elevated serum uric acid level is nonspecific but can also contribute to the diagnosis. (Choice A) Calcinosis cutis is characterized by deposition of calcium and phosphorus in the skin. It presents with scattered whitish papules, plaques, or nodules. This patient's history of painful arthritis is more typical for gout. Calcinosis cutis (Choice B) Chondrosarcomas are bone tumors occurring in the 5th or 6th decade of life. They most commonly affect the pelvis, femur, or proximal humerus and would likely not be as multifocal and bilateral as the disease pictured. (Choice D) Severe osteoarthritis can produce Heberden and Bouchard nodes, which are hard, bony nodules over the distal and proximal interphalangeal joints, respectively. This patient's tophi involving several joint spaces and soft tissue structures are more consistent with gout. osteoarthritis (Choice E) Psoriatic arthritis can present with distal interphalangeal joint arthritis, asymmetric oligoarthritis, symmetric polyarthritis, spondyloarthropathy, or aggressively destructive arthritis mutilans. Most patients have established psoriasis, and nail changes (eg, pitting) are common. Psoriatic arthritis (Choice F) Rheumatoid nodules are firm, flesh-colored, and nontender. They typically occur over pressure points such as the elbow and extensor surface of the proximal ulna. Rheumatoid nodules Educational objective: In chronic tophaceous gout, urate crystals can be deposited in the skin, resulting in the formation of tumors with a chalky white appearance.

Question 3

A 52-year-old man comes to the physician with a long history of joint pain.  The patient has pain and stiffness of the small joints of his hands.  The symptoms are worse in the morning and can last several hours.  He also has digit swelling.  The patient's hands are shown in the image below:  Which of the following is the most likely diagnosis?

Accepted Answer

11ec25c8_d13c_eab5_bc81_7b6e97b9a48c_MD0003_00 The clinical history and image provided are most consistent with psoriatic arthritis. Psoriatic arthritis occurs in 5%-30% of patients who have psoriasis. Psoriatic arthritis can present with many different patterns, but the classic presentation involves the distal interphalangeal (DIP) joints. Morning stiffness is present, as it is in all inflammatory arthritides. Deformity of involved joints, dactylitis ("sausage digit," a diffusely swollen finger), and nail involvement are common. This patient shows all of the classic signs: All of the fingers (particularly the left index finger) are swollen, consistent with dactylitis; many of the nails show pitting and onycholysis (separation of nail bed); and the DIP joints are prominently involved. Furthermore, well-demarcated red plaques with silvery scaling-the classic lesions of psoriasis-are seen on the dorsum of each hand. Current treatment options for psoriatic arthritis include nonsteroidal anti-inflammatory agents, methotrexate, and anti-tumor necrosis factor agents. (Choice A) Nail changes do not accompany crystalline arthritis (gout and pseudogout). Cutaneous deposits of monosodium urate occur in chronic gout. These lesions, known as tophi, most commonly occur over joints and the helix of the ear, and they may ulcerate. tophi (Choice B) Dermatomyositis can present with Gottron's papules (violaceous plaques, slightly scaly) overlying the metacarpophalangeal (MCP) joints, which at times can look similar to psoriasis. However, dermatomyositis is not associated with dactylitis or nail changes (onycholysis or nail pitting). Gottron's papules (Choice C) Enteropathic arthritis occurs in 10%-20% of patients with Crohn disease and ulcerative colitis. It most often affects the lower extremities and sacroiliac joints and tends to wax and wane with the symptoms of bowel disease. These patients have prominent gastrointestinal symptoms and other extraintestinal manifestations of inflammatory bowel disease. (Choice D) Neuropathic arthropathy (Charcot joint) most commonly affects the lower limbs. Most cases occur with diabetes, but syphilis and alcoholism are other causes. Peripheral neuropathy is believed to result in decreased proprioception, which leads to frequent trauma and eventual destruction of the joint. (Choice E) Osteoarthritis often affects the DIP joints. However, the pain of osteoarthritis is worse with activity and improves with rest (the opposite of this patient's symptoms). Osteoarthritis is noninflammatory, and dactylitis should not occur. (Choice G) Rheumatoid arthritis also presents with morning stiffness. The MCP and proximal interphalangeal joints are prominently involved, whereas the DIP joints classically are not. Dactylitis and nail involvement are also not expected in rheumatoid arthritis. (Choice H) Polyarthritis affecting the ankles and knees sometimes occurs in sarcoidosis. Sarcoidosis causes protean cutaneous manifestations, the most common of which is erythema nodosum. These patients also often have cough, chest pain, and dyspnea. erythema nodosum Educational objective: Psoriatic arthritis occurs in 5%-30% of patients with psoriasis. The classic presentation involves the distal interphalangeal joints. Morning stiffness, deformity, dactylitis ("sausage digit"), and nail involvement are common. Current treatment options for psoriatic arthritis include nonsteroidal anti-inflammatory agents, methotrexate, and anti-tumor necrosis factor agents.

Question 4

A 44-year-old psychology professor with a history of chronic rheumatoid arthritis comes to the office for a follow-up examination.  She is currently taking prednisone and infliximab, a regimen that has successfully stabilized her condition.  She enjoys swimming 3 or 4 times a week, which helps with her strength and keeps her weight on target.  Temperature is 37.2 C (99 F), blood pressure is 110/70 mm Hg, pulse is 78/min, and respirations are 16/min.  The patient weighs 65 kg (143.3 lb) and is 162.5 cm (5 ft 4 in) tall.  Physical examination reveals no significant changes.  Toward the end of the visit, the patient mentions that a family friend who suffers with chronic pain secondary to a spinal fusion has had a good response to acupuncture.  She would like to try acupuncture instead of the medications she is taking currently.  Which of the following is the most appropriate response to this patient's request?

Accepted Answer

Patients are increasingly interested in complementary and alternative medicine (CAM), and physicians must be prepared to discuss CAM modalities with them and integrate treatments that have demonstrated safety and efficacy. The initial response to a patient who requests an alternative approach is to explore the reasons for requesting a different treatment. This patient may be dissatisfied or have concerns about the quality of current care. She may be experiencing bothersome medication side effects of which the physician is unaware. Once the patient's reasons are clarified, the physician can discuss the risks and benefits of the proposed CAM treatment in addition to suggesting modifications to the current treatment regimen that may address her concerns. (Choice A) This response enthusiastically endorses the patient's request for acupuncture without first understanding what is prompting her request. In addition, it makes a general statement about the benefits of CAM that is inaccurate in her case given the lack of evidence for acupuncture in rheumatoid arthritis and the risks of discontinuing the current treatments. (Choice B) Although studies have not demonstrated acupuncture's efficacy for rheumatoid arthritis, this response rejects the patient's suggestion outright without first exploring the reasons for her request. The risks and benefits of the proposed treatment can be discussed after the physician has gained a better understanding of the patient's perspective. (Choice C) Warning the patient about the risks of stopping her current treatment regimen is dismissive of her request and likely to damage the physician-patient relationship. The first step is to understand why she is requesting a change in her treatment. The risks of stopping her current medications can be addressed at a later point. (Choice D) It would be premature to refer the patient for acupuncture without first understanding the reasons behind her request. Educational objective: The initial response to patients suggesting complementary and alternative medicine is to explore their reasons for requesting such treatment. Physicians must be prepared to discuss the risks and benefits of complementary and alternative approaches to treatment and be open to integrating modalities with proven safety and efficacy. References: A qualitative systematic review of patients' experiences of acupuncture. (http://www.ncbi.nlm.nih.gov/pubmed/25072404) Integrative medicine and patient-centered care. (http://www.ncbi.nlm.nih.gov/pubmed/19733814) Integration of acupuncture into conventional medicine from health professionals' perspective: a thematic synthesis of qualitative studies. (http://www.ncbi.nlm.nih.gov/pubmed/25788323)

Question 5

A 44-year-old man comes to the office due to persistent cough and dyspnea on exertion for approximately the last 3 months. He has had no fever, chills, rhinorrhea, or sputum production. Over the past year, the patient has had progressively worsening shortness of breath and is unable to sleep lying flat. He has no chest pain or diaphoresis but has had palpitations previously. The patient does not use tobacco or alcohol. He moved to the United States from Southeast Asia 10 years ago. On examination, the patient is alert but in mild distress. Temperature is 36.7 C (98 F), blood pressure is 110/70 mm Hg, and pulse is 100/min. BMI is 34 kg/m². Heart sounds are distant due to body habitus. The lungs demonstrate crackles on auscultation. Chest x-ray reveals an enlarged cardiac silhouette with vascular congestion. The left main stem bronchus appears to be elevated. There is no other visible lung pathology. ECG shows an irregularly irregular rhythm. Which of the following is the most likely cause of this patient's presentation?

Accepted Answer

11ec25c8_d137_ba88_bc81_45d400fa39ca_MD0003_00 This patient likely has decompensated heart failure due to mitral stenosis. Mitral stenosis usually occurs in the setting of rheumatic heart disease, which in the United States is most often seen in patients who emigrated from Latin America, Africa, or Asia. Mitral stenosis causes eventual backflow of blood into the left atrium, leading to elevated left atrial and pulmonary vascular pressures. This classically presents as dyspnea on exertion, often the only symptom of mitral stenosis, and can progress to pulmonary edema. Manifestations vary from cough (productive or nonproductive, occasionally with hemoptysis) to orthopnea, paroxysmal nocturnal dyspnea, and other features of decompensated heart failure (eg, vascular congestion, enlarged cardiac silhouette). The increased left atrial pressure also leads to left atrial dilation that causes upward displacement of the left mainstem bronchus on chest x-ray. Left atrial dilation can also disrupt atrial electrical conduction, commonly leading to atrial fibrillation (eg, palpitations, irregularly irregular rhythm on ECG). The loss of organized atrial contraction often precipitates an acute decompensation because not enough pressure can be generated to mitigate the elevated left atrial pressures and provide adequate blood flow across the stenotic valve. (Choice A) Patients with acute pericarditis can go on to develop pericardial effusion and cardiac tamponade, with associated dyspnea, orthopnea, and an enlarged cardiac silhouette on chest x-ray. However, this patient has no history of recent infection or chest pain that would suggest acute pericarditis. (Choice B) Interstitial lung disease can present with progressive dyspnea and persistent cough. However, chest x-ray typically shows increased reticular or nodular interstitial markings without significant cardiac enlargement or left main stem bronchus elevation. chest x-ray (Choice C) Primary lung malignancy can present with symptoms of fatigue, chest pain, hemoptysis, weight loss, progressive dyspnea, and persistent cough. However, it is less likely in the absence of a smoking history and is less likely to explain cardiac enlargement on chest x-ray. (Choice E) Sarcoidosis is a systemic granulomatous disease that may be asymptomatic or present with fatigue, weight loss, cough, dyspnea, and chest pain. Chest x-ray usually shows bilateral hilar adenopathy with or without parenchymal reticular opacities. Educational objective: Mitral stenosis usually occurs in the setting of rheumatic heart disease and presents with exertional dyspnea, cough, and orthopnea. Left atrial dilation is typical and may be recognized by elevation of the left main stem bronchus on chest x-ray. Atrial fibrillation is a common complication and can precipitate acute decompensated heart failure in previously well-compensated patients. References: Rheumatic heart disease. (http://www.ncbi.nlm.nih.gov/pubmed/8736683) Mitral stenosis. (http://www.ncbi.nlm.nih.gov/pubmed/19747723)

Question 6

A 48-year-old man comes to the emergency department due to facial drooping.  The patient says he had mild left ear discomfort in the morning, and he had difficulty closing the left eye to the sunlight while driving to work.  Four hours later, he was unable to sip drinks through a straw, and a colleague remarked that his face appeared droopy.  He has had no upper or lower limb weakness or numbness, headache, fever, dizziness, or hearing loss.  The patient was recently diagnosed with hypertension and hyperlipidemia, which he is managing with lifestyle modification.  He smokes cigarettes and drinks alcohol daily and says he is trying to cut down on both.  Temperature is 37 C (98.6 F), blood pressure is 142/86 mm Hg, and pulse is 84/min.  On physical examination, there is left facial asymmetry with drooping of the left corner of the mouth and flattening of the nasolabial fold.  He is unable to fully close the left eye.  Extraocular movements are intact, and pupils are briskly reactive.  Bilateral hand grips are equal, and there is no pronator drift.  The patient is able to lift his lower extremities against resistance, and gait is normal.  Sensation to light touch and pain is intact on the left side of the face.  Which of the following is the most appropriate pharmacotherapy for this patient?

Accepted Answer

11ec25c8_d143_0539_bc81_79cd5ce44e31_MD0003_00 This patient with acute-onset, unilateral facial drooping involving the upper and lower face likely has Bell palsy, a peripheral neuropathy involving the facial nerve (CN VII). Symptom onset is typically acute (over the course of hours) and progressive, with maximal weakness occurring within 3 weeks. It may be preceded by a prodrome of auricular pain or dysacusis (distortion of sound). Unilateral facial paralysis is characterized by mouth drooping, disappearance of the nasolabial fold, and, most importantly, involvement of the upper face (eg, inability to close the eye, weakness raising the eyebrow), which helps distinguish Bell palsy from acute stroke (in which the upper face is typically spared). Patients with Bell palsy may also have decreased tearing, hyperacusis, and a reduced sense of taste over the anterior two-thirds of the tongue. Sensation is typically intact. upper face The mainstay of therapy is high-dose oral glucocorticoids (eg, prednisone), which should be started within 72 hours of symptom onset. Because many cases are thought to be caused by reactivation of the herpes simplex virus, some studies also suggest the addition of valacyclovir or acyclovir. Patients with incomplete eye closure should also be counseled on corneal protection strategies (eg, moisture chamber, lubricating drops or ointment, taping the eye closed at night). Most patients improve spontaneously within 3-4 months; palsies lasting longer suggest another diagnosis. (Choices A and B) Alteplase is indicated for reperfusion therapy in select patients who arrive within 4.5 hours after an acute ischemic stroke, whereas aspirin and statins are indicated for patients outside this window (and for long-term therapy). Although stroke can cause facial weakness, it typically does not involve the eye or forehead. It may also be associated with facial numbness, dysphagia, or weakness in the limbs. (Choice C) Carbamazepine is used to treat trigeminal neuralgia, which causes paroxysms of severe, unilateral, stabbing pain in a branch of the trigeminal nerve. It is not associated with facial weakness. (Choice D) Ganciclovir is used to treat cytomegalovirus infections, which are not typically associated with the development of Bell palsy. It is not recommended for use in combination with glucocorticoids for Bell palsy. Educational objective: Bell palsy (CN VII peripheral neuropathy) presents with acute, progressive, unilateral facial weakness involving the upper and lower face. A prodrome of auricular pain may be present. Therapy is with glucocorticoids, possibly in combination with acyclovir or valacyclovir.

Question 7

A 43-year-old man is brought to the emergency department due to confusion and left-sided weakness. He has been living in a homeless shelter for the past month, and a staff member says that the patient has had progressive memory impairment, difficulty communicating, and left-sided weakness. The patient was diagnosed with HIV infection 4 years ago and had an episode of Pneumocystis pneumonia 6 months ago. He has not been compliant with antiretroviral therapy. Temperature is 37.1 C (98.8 F), blood pressure is 130/80 mm Hg, pulse is 78/min, and respirations are 16/min. Physical examination shows a disheveled and cachectic man with oral thrush. The patient is not oriented to time, place, or person and has expressive aphasia. Left-sided hyperreflexia, hypertonia, and upgoing plantar reflex are present. CD4 count is 30/mm³, and toxoplasma serology is positive. CT scan of the brain with intravenous contrast reveals several asymmetric, hypodense, nonenhancing white matter lesions with no edema. Which of the following is the most likely cause of this patient's current condition?

Accepted Answer

11ec25c8_d141_a5a8_bc81_1192dce60522_MD0003_00 This patient with untreated AIDS has progressive neurologic symptoms and imaging evidence of nonenhancing, asymmetric white matter lesions, raising strong suspicion for progressive, multifocal leukoencephalopathy (PML), a life-threatening neurologic disease caused by the reactivation of JC virus. JC virus is usually acquired in childhood and lies dormant in the kidneys and lymphoid tissue; most individuals remain asymptomatic. However, those with severe immunocompromise (eg, AIDS with CD4 count <200/mm³) are at risk for reactivation. Reactivated virus spreads to the CNS and lyses oligodendrocytes, causing white matter demyelination. Symptoms include altered mental status, motor deficits, ataxia, and vision abnormalities (eg, diplopia). CT of the brain with contrast usually reveals asymmetric, nonenhancing, hypodense white matter lesions with no surrounding edema. Diagnosis requires lumbar puncture with cerebral spinal fluid evidence of JC virus (by polymerase chain reaction). Brain biopsy is rarely required. There is no specific treatment for PML, but initiation of antiretroviral therapy (in patients with AIDS) can sometimes prevent progression and death. However, most patients have long-term neurologic sequalae. (Choice A) Cerebral toxoplasmosis is the most common CNS infection in patients with untreated AIDS and often causes headache, confusion, fever, and focal deficits. However, imaging typically reveals multiple ring-enhancing lesions with edema (not nonenhancing lesions with no edema). Although this patient's positive toxoplasma serology indicates previous exposure (which is quite common), it does not indicate reactivated/active disease. (Choice B) Herpes simplex encephalitis usually causes acute (not subacute) fever, headache, seizures, focal deficits, and altered mental status. Imaging typically reveals unilateral temporal lobe-enhancing lesions with mass effect. (Choice C) HIV-associated dementia can cause behavioral and personality changes in those not on ART. However, MRI brain usually shows diffuse brain atrophy, ventricular enlargement, and reduced attenuation of white matter structures. The presence of focal, asymmetric white matter lesions in this case make HIV-associated dementia much less likely. (Choice D) Kaposi sarcoma usually causes cutaneous lesions, sometimes with gastrointestinal or mucous membrane involvement. Brain lesions are extremely rare. (Choice E) Multiple sclerosis (MS) is also an inflammatory, demyelinating CNS disease; however, imaging usually reveals well-circumscribed (not irregular) white matter lesions. In addition, MS flares are uncommon in patients with advanced AIDS. (Choice F) Although primary CNS lymphoma is an AIDS-defining illness that occurs much more often in patients with advanced AIDS (compared to the general population), imaging usually reveals a single, well-defined, ring-enhancing lesion with surrounding edema. The presence of multiple asymmetric lesions and lack of enhancement/edema makes primary CNS lymphoma unlikely. (Choice H) Subacute sclerosing panencephalitis is a progressive, fatal disease that occurs years after measles infection. Imaging reveals brain scarring and atrophy. Educational objective: Progressive multifocal leukoencephalopathy is caused by JC virus reactivation and occurs primarily in patients with advanced HIV (CD4 count <200/mm³). Manifestations include subacute neurologic changes (eg, mental status change, motor deficits, ataxia) and imaging evidence of multiple nonenhancing brain lesions with no mass effect (edema).

Question 8

A 74-year-old woman comes to the office due to several days of right ear pain.  This morning she had 2 episodes of dizziness and almost fell.  The patient is currently taking trimethoprim-sulfamethoxazole for an uncomplicated urinary tract infection.  She has had no subjective fever, vision loss, or headaches.  Temperature is 37 C (98.6 F), blood pressure is 116/78 mm Hg, and pulse is 100/min.  Ear examination reveals several erythematous vesicles in the right external auditory canal with no drainage.  The tympanic membrane is visible and intact.  There is mild right-sided facial droop.  The rest of the physical examination is unremarkable.  Which is the most likely cause of this patient's illness?

Accepted Answer

This patient with a vesicular ear rash and facial droop likely has herpes zoster oticus (Ramsay Hunt syndrome). This condition is caused by the reactivation of varicella zoster virus from the geniculate ganglion, disrupting motors fibers of cranial nerve VII (facial nerve) with subsequent spread to cranial nerve VIII (vestibulocochlear nerve). The 2 defining manifestations are a painful, erythematous, vesicular rash on the auditory canal or auricle and ipsilateral facial paralysis. Many patients also develop vestibular (eg, vertigo, nausea/vomiting), hearing, and taste disturbances, but systemic symptoms (eg, fever) are rare (<20%). Treatment with an antiviral medication (eg, valacyclovir) may speed resolution and limit adverse outcomes. However, many patients are left with residual facial muscle weakness or paralysis. (Choice A) Lyme disease is caused by Borrelia burgdorferi, and early disseminated Lyme disease can be associated with facial nerve palsy (~8% of cases). However, the rash of Lyme disease is erythema migrans, a slowly spreading erythematous lesion with central clearing. Vesicular lesions would be atypical. (Choice B) The reactivation of herpes simplex virus (HSV) type 1 is the leading cause of Bell palsy (acute-onset, unilateral facial paralysis). However, HSV usually causes vesicular lesions on the oral mucosa, not the auditory canal. The presence of ear lesions makes varicella zoster reactivation far more likely. (Choice C) Pseudomonas aeruginosa is the leading cause of acute external otitis, which is common in swimmers and usually marked by ear itching and pain. Physical examination typically reveals thick, purulent discharge from the ear canal (unlike this patient). Vesicular lesions are not a common feature of this illness. (Choice D) Although Stevens-Johnson syndrome is frequently associated with the use of antibacterial sulfonamides (eg, trimethoprim-sulfamethoxazole), this illness is usually marked by high fever; a flu-like prodrome; hemorrhagic oral/ocular lesions; and an erythematous, macular rash with a purpuric center. Educational objective: Herpes zoster oticus (Ramsay Hunt syndrome) is caused by the reactivation of varicella zoster virus in the geniculate ganglion, disrupting cranial nerve VII motor fibers, with subsequent spread to cranial nerve VIII. Primary manifestations include a painful, erythematous, vesicular rash on the auditory canal or auricle and ipsilateral facial paralysis. References: Ramsay Hunt syndrome. (http://www.ncbi.nlm.nih.gov/pubmed/11459884)

Question 9

A 44-year-old-man with HIV is being discharged from the hospital.  He was admitted 2 weeks ago for fever and headache and was diagnosed with cryptococcal meningitis.  The patient was treated with amphotericin B and flucytosine, and his symptoms are now resolved.  Vital signs are normal.  Physical examination shows no abnormalities.  Which of the following medications should be prescribed for the patient to take following discharge?

Accepted Answer

Explanation: Cryptococcal meningitis is a common AIDS-defining illness in patients with HIV who have CD4 counts <100/mm³. Patients generally present with subacute fever, headache, and altered mental status. Diagnosis is made when encapsulated yeasts (Cryptococcus neoformans) are identified in cerebrospinal fluid. ³ Patients with cryptococcal meningitis usually require 3 successive phases of treatment, as follows: Induction therapy with liposomal amphotericin B plus flucytosine is given for ≥2 weeks until acute symptoms resolve and/or cerebrospinal fluid becomes sterile. Consolidation therapy with high-dose oral fluconazole is given for ≥8 weeks to prevent disease relapse. Maintenance therapy with low-dose oral fluconazole is given indefinitely or until CD4 counts rise to >100/mm³ for >3 months on antiretroviral therapy (ART). ART should be initiated in all patients with HIV. However, due to the risk of life-threatening CNS complications from immune reconstitution syndrome, ART initiation is generally delayed for approximately 2 weeks after treatment begins for Cryptococcus. (Choice A) Caspofungin, an echinocandin, is used to treat some fungal infections (eg, Aspergillus, Candida), but it is not effective against Cryptococcus species. (Choice C) Itraconazole can be used for consolidation therapy in those unable to take fluconazole. However, it is considered a second-line agent because it is less effective at preventing relapse and has more adverse effects and drug interactions. (Choice D) Cessation of treatment after induction therapy is associated with a high rate of relapse; patients need to have consolidation therapy to prevent recurrence. (Choice E) Although corticosteroids are used as an adjuvant treatment to limit inflammation in some opportunistic infections (eg, severe Pneumocystis pneumonia, tuberculous meningitis), corticosteroids do not improve outcomes in patients with cryptococcal meningitis and may slow clearance of the organism from the CNS. Therefore, corticosteroids are not recommended as part of standard treatment. Educational objective: Cryptococcal meningitis is an AIDS-defining illness that primarily affects patients with CD4 counts <100/mm³. Three phases of treatment are required to ensure eradication and prevent relapse: induction with amphotericin B and flucytosine for ~2 weeks, consolidation with high-dose oral fluconazole for ~8 weeks, and maintenance with low-dose oral fluconazole until CD4 counts recover to >100/mm³ for >3 months on antiretroviral treatment. ³ ³ References: Management of elevated intracranial pressure in patients with cryptococcal meningitis. (http://www.ncbi.nlm.nih.gov/pubmed/9473014)

Question 10

A 70-year-old woman comes to the office due to worsening bilateral shoulder pain for 3 months that is not responsive to analgesics.  The patient also reports tingling in her right hand and forearm for the past 3 weeks.  Medical history is significant for diabetic nephropathy and end-stage renal disease.  The patient has been receiving hemodialysis 3 times a week for 20 years.  Temperature is 37 C (98.6 F), blood pressure is 130/78 mm Hg, pulse is 80/min, and respirations are 16/min.  Examination shows bilateral hypertrophy of the shoulders.  Range of motion of the shoulder joints is restricted in all directions.  Weakness and atrophy of the muscles of the thenar eminence, as well as decreased sensation in the thumb and index, middle, and ring fingers, are noted.  Heart sounds are normal.  There is no hepatosplenomegaly.  X-ray reveals radiolucent bone cysts in both shoulders.  Ultrasound reveals increased rotator cuff thickness and deposits with increased echogenicity between the muscles and tendons of the rotator cuff.  Serum calcium is 8.1 mg/dL.  Which of the following is the most likely diagnosis?

Accepted Answer

Explanation: This patient on long-term dialysis has shoulder pain/hypertrophy and carpal tunnel syndrome due to dialysis-related amyloidosis (DRA). The unifying feature of amyloidoses is misfolded precursor proteins that deposit in extracellular tissues, causing organ dysfunction. When these precursor proteins undergo excessive production, inadequate clearance, or inherited mutation, amyloidosis results. amyloidosis In DRA, beta2-microglobulin (B2-m) is the precursor protein deposited as amyloid due to inadequate elimination despite dialysis. Along with sustained elevation of B2-m, several connective tissue components (eg, glycosaminoglycans, type I collagen) participate in amyloid fibril stabilization. This may explain DRA's affinity for osteoarticular structures, resulting in the following classic triad: Scapulohumeral periarthritis: shoulder pain/hypertrophy with increased rotator cuff thickness and hyperechogenic deposits on imaging Carpal tunnel syndrome: median neuropathy, with thenar eminence atrophy and weakness, tingling and decreased sensation in the first 3 fingers, as well as possible flexor tendosynovitis (finger contractures) Bone cysts: possible pathologic fracture DRA prevalence increases with age and dialysis duration. (Choice A) Acromegaly frequently causes carpal tunnel syndrome, and soft tissue hypertrophy is characteristic. However, enlargement is not isolated to the shoulders. Tissue overgrowth involves the jaw (macrognathia), brow ridge, tongue (macroglossia), and hands and feet. Headaches are a prominent symptom, and acromegaly is not associated with dialysis. Acromegaly (Choice C) Polymyalgia rheumatica prevalence increases with age, and shoulder pain is characteristic; however, the hip girdle is also involved, and there is no hypertrophy or bone cysts. (Choice D) Pseudogout (acute attacks of calcium pyrophosphate crystal deposition in synovial tissues) typically results in a monoarticular, self-limited inflammatory arthritis. Imaging can reveal chondrocalcinosis (calcification of articular cartilage appearing as linear radiodensities on x-ray) and hyperechoic bands within the tendons, not between the muscles and tendons, as in this patient. Bilateral shoulder involvement would be uncommon; it rarely causes bone cysts and is unrelated to dialysis. chondrocalcinosis (Choice E) Tertiary hyperparathyroidism results from inadequately controlled secondary hyperparathyroidism in end-stage kidney disease. Parathyroid hyperplasia results in the autonomous secretion of parathyroid hormone unresponsive to plasma calcium levels, typically resulting in hypercalcemia. Although tertiary hyperparathyroidism can cause bone cysts, this patient's low calcium make it highly unlikely, and soft tissue deposits are not typical. Educational objective: Dialysis-related amyloidosis results from inadequate clearance of beta2-microglobulin despite dialysis, and the prevalence increases with age and dialysis duration. It has a predilection for osteoarticular structures presenting as scapulohumeral periarthritis (shoulder pain/hypertrophy), carpal tunnel syndrome, and bone cysts.

Question 11

A 62-year-old woman comes to the office due to urinary frequency and burning during urination. The patient reports no fever, chills, nausea, back pain, or abdominal pain. Her medical history is significant for long-standing diabetes mellitus and hypertension, for which she takes metformin and lisinopril. The patient does not use tobacco or alcohol. Blood pressure is 149/100 mm Hg and heart rate is 70/min. Hematocrit is 43% and WBC count is 8,500/mm³. Creatinine is 1.1 mg/dL. Urinalysis results are as follows: The patient is given a course of trimethoprim-sulfamethoxazole. Two weeks later, urinalysis reveals 2+ protein but no nitrites, white blood cells, or red blood cells. Her urinary symptoms have resolved. Repeat serum creatinine is 1.1 mg/dL. Which of the following is most likely responsible for her persistent urinalysis abnormality?

Accepted Answer

Explanation: 11ec25c8_d13a_a0bf_bc81_5f1bd25f4bf4_MD0003_00 This patient's presenting symptoms of dysuria and urinary frequency, combined with abnormal urinalysis (eg, hematuria, leukocytes, positive nitrites), suggest urinary tract infection. However, after treatment and resolution of urinary symptoms, she continues to have persistent proteinuria. In the context of her long-standing diabetes and poorly controlled hypertension, persistent proteinuria suggests diabetic nephropathy (DN). Metabolic by-products (eg, advanced glycation end-products, reactive oxygen species) associated with diabetes lead to irreversible changes of DN, primarily affecting the glomerular basement membrane and surrounding structures. These include: Glomerular hyperfiltration Glomerular basement membrane fibrosis and thickening Interstitial fibrosis, mesangial thickening, and nodules (Kimmelstiel-Wilson lesion) A common clinical abnormality in DN is persistently elevated albumin excretion, known as moderately increased albuminuria (ie, albumin-creatinine ratio of 30-300 mg/g). Therefore, screening via random urine albumin-creatinine ratio should be performed at least annually, starting either at diagnosis (type 2) or 5 years after diagnosis (type 1). Risk factor modification (eg, diet optimization, exercise, smoking cessation), glycemic control (A1c ≤7.0%), and medical management of hyperlipidemia and hypertension (eg, ACE inhibitors) also help slow the progression of DN. (Choice A) Acute interstitial nephritis (AIN) can be caused by antibiotics (eg, methicillin), infections (eg, Legionella), or systemic disease (eg, sarcoidosis). Trimethoprim-sulfamethoxazole can be a potential cause of AIN; however, this patient lacks the typical AIN findings of acutely elevated creatinine, pyuria (eg, eosinophiluria), and white cell casts in the urine. (Choice B) Atherosclerotic vascular disease can lead to renal artery stenosis, the most common cause of secondary hypertension in adults. Poorly controlled hypertension is a hallmark of this condition; however, this patient's persistent proteinuria is an uncommon finding. (Choice D) Sulfonamide antibiotics can lead to insoluble crystal precipitation in kidney tubules and subsequent obstruction and acute kidney injury. Other causes include acyclovir, excessive vitamin C, and methotrexate. However, crystal-induced obstruction would not cause persistent proteinuria; moreover, findings of acute creatinine elevation or crystalluria would be expected. (Choice E) In adults, vesicoureteral reflux (ie, retrograde passage of urine into the renal pelvis) is associated with neurogenic bladder and can cause frequent urinary tract infections. Long-standing diabetes may cause autonomic bladder dysfunction; however, this patient does not have associated symptoms of chronic incontinence, and persistent proteinuria would be uncommon. Educational objective: Diabetic nephropathy is characterized by glomerular hyperfiltration, basement membrane thickening, and mesangial nodules. Persistent proteinuria in a patient with long-standing diabetes and poorly controlled hypertension should raise suspicion for this diagnosis. References: Markers of inflammation and oxidative stress in the development and progression of renal disease in diabetic patients. (http://www.ncbi.nlm.nih.gov/pubmed/27344598)

Question 12

A 72-year-old man comes to the emergency department after a syncopal episode.  The patient briefly lost consciousness an hour ago while getting dressed.  He remembers putting on a shirt and tie and the next thing he remembers is finding himself on the floor with his wife at his side.  His wife reports that the patient had no abnormal extremity movements or involuntary micturition during the event.  The patient has had several episodes of lightheadedness, especially in the morning while shaving, but has never lost consciousness or fallen.  He has a history of poorly controlled hypertension and had a recent change in his medications.  Other medical conditions include type 2 diabetes mellitus and hyperlipidemia.  Blood pressure is 142/86 mm Hg while supine and 134/84 mm Hg while upright, and pulse is 72/min while supine and 80/min while upright.  Physical examination shows no carotid bruit and normal jugular venous pressure.  There is a grade 2/6 systolic murmur, best heard at the right second intercostal space, and both heart sounds are normal.  There is loss of vibration sensation in the feet, and ankle reflexes are absent bilaterally.  The remainder of the examination shows no abnormalities.  ECG reveals normal sinus rhythm.  Which of the following is the most likely cause of this patient's syncope?

Accepted Answer

11ec25c8_d13f_d0e7_bc81_b1ff1cc8e5c8_MD0003_00 This patient most likely has carotid sinus hypersensitivity (CSH). In the normal state, carotid sinus baroreceptors detect increases in blood pressure and respond by stimulating a relative increase in parasympathetic (vagal) tone that slows the heart rate and induces vasodilation to reduce and regulate blood pressure. In some individuals, especially elderly men with atherosclerotic disease, the carotid sinus baroreceptors become overly sensitive, triggering an exaggerated vagal response. This can cause a transient reduction in cerebral perfusion that manifests as syncope or presyncope (ie, lightheadedness). carotid sinus baroreceptors The exaggerated vagal response that characterizes CSH is often triggered by minimal tactile stimulus of the carotid sinus (eg, shaving, rubbing of a shirt collar while dressing or turning the head) and causes a >3 second ventricular pause and >50 mm Hg drop in systolic blood pressure. The diagnosis is made by carotid massage producing an exaggerated vagal response; this maneuver is often aided by a tilt table to ensure the patient is secured and does not fall. Treatment can involve placement of a permanent pacemaker. (Choice B) This patient's reduced vibratory sensation and ankle reflexes are consistent with diabetic peripheral neuropathy, which often involves damage to the autonomic nervous system. Although such damage can predispose to orthostasis and syncope, symptoms usually occur immediately after standing. (Choice C) Syncope due to intermittent cardiac arrhythmia (eg, paroxysmal ventricular tachycardia) usually occurs without warning symptoms and is a concern in older patients with cardiomyopathy or ischemic heart disease. The association of tactile stimulus of the carotid sinus with this patient's symptoms makes CSH more likely. (Choice D) Hypovolemia is a potential cause of syncope and presyncope; however, the symptoms are usually orthostatic and occur immediately after standing. (Choice E) Left ventricular outflow obstruction (eg, severe aortic stenosis) is a potential cause of syncope. Although this patient's systolic murmur could represent aortic stenosis, syncope or presyncope due to aortic stenosis is usually exertional. Educational objective: Carotid sinus hypersensitivity involves an exaggerated vagal response that is often triggered by tactile stimulation. The exaggerated response causes a >3 second ventricular pause and >50 mm Hg drop in systolic blood pressure, leading to a transient reduction in cerebral perfusion that manifests as syncope or presyncope. References: Diagnosis of carotid sinus hypersensitivity in older adults: carotid sinus massage in the upright position is essential. (http://www.ncbi.nlm.nih.gov/pubmed/10618329)

Question 13

A 45-year-old woman comes to the emergency department with vomiting and severe right flank pain that radiates to her groin.  The patient has been to the emergency department twice in the past due to similar episodes of renal colic.  Previous imaging showed radio-opaque stones.  The stones passed spontaneously, and she did not seek further medical care.  Imaging now shows another ureteral stone.  A 24-hour urine collection shows urinary calcium excretion of 350 mg (normal is <250 mg in women).  Laboratory results show a serum calcium concentration of 8.9 mg/dL; serum parathyroid hormone is normal.  Further investigations fail to reveal a cause of the hypercalciuria.  Apart from advising increased fluid intake, which of the following interventions will benefit this patient?

Accepted Answer

11ec25c8_d13b_6410_bc81_c1973c0b6855_MD0003_00 Renal stone formation involves the abnormal excretion of stone-forming salts and/or a deficiency of urinary inhibitors of crystal formation due to metabolic disorders or dietary factors. Calcium stones are the most common type of stone seen and usually present in the third or fourth decade of life. Most patients have hypercalciuria (24-hour urinary calcium excretion >4 mg/kg). This patient most likely has recurrent calcium stones due to idiopathic hypercalciuria, which is best treated with increased fluid intake, dietary sodium and protein restriction, and thiazide/amiloride diuretics. The mild volume depletion caused by thiazide diuretics leads to a compensatory rise in reabsorption of sodium and water with resulting increased passive reabsorption of calcium. Thiazides are also thought to modulate calcium channels on the tubular membrane. Lowering the urinary concentration of calcium reduces its precipitation as insoluble calcium salts. thiazide diuretics (Choices A and C) Aldosterone antagonists like spironolactone or eplerenone are potassium-sparing diuretics. Furosemide is a loop diuretic. They all increase urinary calcium excretion, potentially worsening stone formation. (Choice B) Increased sodium load from high dietary sodium reduces reabsorption of sodium and thereby reduces the passive reabsorption of calcium. Typically <100 mEq/day of sodium is recommended. (Choice D) Calcium binds with oxalate in the gut to form unabsorbable calcium oxalate. Reduced dietary calcium leads to an increase in oxalate absorption in the gut, which is then excreted into the urine and binds urinary calcium to form calcium oxalate stones. Excess calcium supplementation (ie, not from food sources) is also associated with increased stone formation. Educational objective: Measures to prevent urinary calcium stone formation include increasing fluid intake, following a low-sodium, low-protein diet, maintaining moderate calcium intake, and taking thiazide diuretics to lower urinary calcium excretion. References: Medical management to prevent recurrent nephrolithiasis in adults: a systematic review for an American College of Physicians Clinical Guideline. (http://www.ncbi.nlm.nih.gov/pubmed/23546565) From crystalluria to kidney stones, some physicochemical aspects of calcium nephrolithiasis. (http://www.ncbi.nlm.nih.gov/pubmed/25374820)

Question 14

A 25-year-old, previously healthy man comes to the office due to 3 months of progressive dyspnea and nonproductive cough.  The patient feels short of breath when cycling 2 miles to his office, which he could do without difficulty in the past.  He does not use tobacco or alcohol.  The patient works at a publishing firm in New York and has not traveled recently.  Family history is significant for asthma in his father.  Temperature is 36.7 C (98.1 F), blood pressure is 130/80 mm Hg, pulse is 78/min, and respirations are 16/min.  Pulse oximetry is 97% on room air.  The lungs are clear on auscultation and heart sounds are normal.  Laboratory results are as follows: 
Chest x-ray shows mediastinal fullness and scattered reticular opacities in the upper lungs.  Which of the following pulmonary function test patterns is most likely to be observed in this patient?

Accepted Answer

11ec25c8_d138_7ddb_bc81_6398fe2bde8c_MD0003_00 This patient's presentation is consistent with sarcoidosis, a systemic, granulomatous disorder of unknown etiology that commonly involves the lungs. Clues to the diagnosis include the patient's hypercalcemia (due to vitamin D conversion by lung macrophages) and chest x-ray findings suggesting hilar lymphadenopathy (described as mediastinal fullness and scattered reticular opacities in the upper lobe). Lung examination is often normal; patients may have only mild symptoms. Sarcoidosis can result in abnormal pulmonary function tests (PFTs) in up to 70% of patients. Granuloma-induced scarring and fibrosis result in the typical restrictive pattern (ie, normal or reduced FEV1, normal or reduced FVC, normal or increased FEV1/FVC ratio, reduced total lung capacity [TLC]). The inflammation and scarring intrinsically impede alveolar gas exchange, leading to decreased diffusion capacity for carbon monoxide (DLCO). Among patients demonstrating restrictive physiology on PFTs, the findings for DLCO help differentiate intrinsic lung disease (eg, fibrosis) from extrinsic causes of hypoventilation (eg, chest wall weakness). (Choice A) Obesity-hypoventilation syndrome, in which excess adipose tissue restricts diaphragmatic excursion, can produce PFT results demonstrating restrictive physiology with chest wall weakness. Findings include decreased FEV1 and TLC with preserved DLCO because the physiology of alveolar gas exchange is unaffected. (Choice B) In diffuse pulmonary or alveolar hemorrhage (eg, pulmonary vasculitis), an abnormal increase in hemoglobin within the alveolar space increases carbon monoxide transfer, resulting in increased DLCO. However, lung volumes and airway mechanics are unaffected; therefore, FEV1 and TLC are normal. (Choices D and E) Obstructive lung conditions (eg, asthma, chronic bronchitis, emphysema) result in lung hyperinflation and airway obstruction during exhalation. Expected PFT findings include increased TLC and decreased FEV1. The results for DLCO allow differentiation between chronic obstructive pulmonary disease (COPD) and severe asthma. COPD is associated with reduced DLCO due to alveolar destruction, whereas severe asthma is associated with increased DLCO due to increased thoracic blood flow caused by increased negative intrathoracic pressures during inspiration. Educational objective: Pulmonary function tests in patients with sarcoidosis-associated lung disease typically follow a restrictive pattern: normal or reduced FEV1, normal or reduced FVC, normal or increased FEV1/FVC ratio, reduced total lung capacity, and decreased diffusion capacity for carbon monoxide. References: Pulmonary sarcoidosis. (http://www.ncbi.nlm.nih.gov/pubmed/26593138) Pulmonary fibrosis in sarcoidosis: clinical features and outcomes. (http://www.ncbi.nlm.nih.gov/pubmed/23952859)

Question 15

A 34-year-old woman comes to the office due to excessive urination at night. For the past 2 weeks, the patient has been getting up at least 3 times at night to urinate. She has a history of hypothyroidism treated with levothyroxine and bipolar disorder treated with lithium carbonate. Blood pressure is 134/78 mm Hg and pulse is 88/min. BMI is 27 kg/m². Physical examination shows decreased skin turgor and dry mucous membranes. Laboratory results after overnight fasting are as follows: This patient's symptoms are most likely caused by a defect in which of the following sites?

Accepted Answer

Explanation: 11ec25c8_d139_dd6d_bc81_ff9e5dada309_MD0003_00 This patient with nocturia and persistently dilute urine (urine osmolality <300 mOsmol/kg) with overnight fasting likely has lithium-induced nephrogenic diabetes insipidus (DI). Normally, antidiuretic hormone (ADH) stimulates renal water reabsorption when serum osmolality is high (eg, following water deprivation). ADH resistance or deficiency causes DI, which presents with dilute urine (low urine osmolality) and polyuria (often worse at night). Nephrogenic DI is caused by renal ADH resistance. One of the most common causes in adults is lithium, which accumulates in renal collecting ducts and blocks responsiveness to ADH. After water deprivation (eg, overnight fasting), serum osmolality rises (eg, >295 mOsmol/kg, often with hypernatremia) and ADH increases appropriately; however, urine osmolality remains low. Central DI can occur following hypothalamic or posterior pituitary damage (eg, trauma, infection) and leads to deficient ADH secretion. After water deprivation, serum osmolality rises but ADH remains low; therefore, urine osmolality remains low. Lithium use is only rarely associated with central DI (Choice C). A desmopressin challenge can differentiate central from nephrogenic DI (as desmopressin causes increased urine osmolality only in central DI). (Choice A) Psychogenic polydipsia is classically associated with psychiatric conditions or disorders affecting thirst. Polyuria and dilute urine are seen; however, in contrast to DI, they represent appropriate responses to excessive water intake, and both ADH sensitivity and production are intact. Therefore, following water deprivation, ADH rises and urine becomes concentrated (ie, urine osmolality increases >300 mOsmol/kg). This patient's dilute urine following water deprivation makes psychogenic polydipsia unlikely. (Choice B) Pancreatic cell destruction causes type 1 diabetes mellitus; severe hyperglycemia (eg, diabetic ketoacidosis) can precipitate glucosuria and associated osmotic diuresis. However, osmotic diuresis is associated with high, rather than low, urine osmolality, and this patient's fasting blood sugar is only mildly elevated. (Choice D) Sodium-glucose cotransporter-2 (SGLT-2) inhibitors are diabetic medications that act on the proximal tubule of the kidney, leading to increased excretion of glucose and associated osmotic diuresis. This patient has no history of SGLT-2 inhibitor use, and her low urine osmolality makes osmotic diuresis unlikely. Sodium-glucose cotransporter-2 (SGLT-2) inhibitors (Choice F) This patient's elevated TSH suggests suboptimally treated hypothyroidism (possibly from chronic lithium therapy). However, hypothyroidism is not associated with nocturia or persistently dilute urine. Educational objective: Chronic lithium therapy is commonly associated with nephrogenic diabetes insipidus (DI), presenting with polyuria and persistently dilute urine. Lithium-induced nephrogenic DI results from lithium accumulation in the renal collecting ducts, which leads to antidiuretic hormone resistance and impaired renal water reabsorption. References: Pathophysiology, diagnosis and management of nephrogenic diabetes insipidus. (http://www.ncbi.nlm.nih.gov/pubmed/26077742)

Question 16

A 26-year-old, previously healthy woman comes to the office due to worsening fatigue, constipation, and nonproductive cough for the past several months.  The patient also reports occasional reddish, painful bumps on her legs and nonpainful, nonpruritic facial eruptions for the past week.  She has had no fever, chest pain, orthopnea, or pedal edema.  The patient does not use tobacco, alcohol, or illicit drugs and is in a monogamous relationship.  Vital signs and pulse oximetry are within normal limits.  Physical examination shows normal oropharyngeal mucosa and jugular venous pressure.  The lungs are clear on auscultation and heart sounds are normal.  The abdomen is soft and nontender.  There are enlarged cervical and axillary lymph nodes.  Examination of the skin reveals 3 violaceous, indurated nodules on the anterior shins, as well as facial eruptions shown in the exhibit.  Chest imaging and laboratory studies are ordered.  Which of the following sets of laboratory findings are expected in this patient?

Accepted Answer

The answer of A 26-year-old, previously healthy woman comes to...

Question 17

A 65-year-old man comes to the office due to tingling, numbness, and burning pain in his left hand for several weeks.  He also has similar symptoms in his right hand but to a lesser extent.  The patient has end-stage renal disease due to hypertension and, for the past 2 years, has been receiving hemodialysis through an arteriovenous fistula of the left arm.  Symptoms worsen during hemodialysis sessions.  The patient has no symptoms in the legs.  He has no muscle weakness, headache, vision changes, speech changes, or swallowing difficulty.  Which of the following is the most likely diagnosis?

Accepted Answer

This patient with end-stage renal disease (ESRD) has tingling, numbness, and burning of the bilateral hands that worsen during hemodialysis, suggesting carpal tunnel syndrome (CTS). CTS occurs due to compression of the median nerve as it passes through the carpal tunnel in the wrist. carpal tunnel CTS is the most common mononeuropathy in patients on hemodialysis, with up to a third reporting symptoms. The etiology in this population is multifactorial. The most common cause is likely dialysis-related amyloidosis, in which inflammation stimulates the formation of beta-2 microglobulin. This is inadequately cleared and deposited as amyloid within the carpal tunnel. Other contributing factors include increased venous pressure during hemodialysis, blood tracking (through fascial planes) from the fistula into the carpal tunnel during vascular access, and deposition of calcium phosphate in the tunnel, all of which worsen extrinsic compression of the nerve. In addition, vascular steal through the fistula may lead to ischemic neuropathy. Manifestations include pain and paresthesia involving the first 3 digits and the radial aspect of the fourth; however, patients may also have referred pain throughout the hand. Symptoms typically worsen during dialysis and are more severe in the arm with vascular access (eg, fistula, graft). In all forms of CTS, including that caused by dialysis-related amyloidosis, symptoms are frequently reproducible with provocative measures. The diagnosis is usually made clinically but can be confirmed with electrodiagnostic studies. provocative measures (Choice B) Cervical myelopathy is characterized by slowly progressive weakness, numbness, and ataxia involving the upper and lower extremities. It would not be expected to cause pain that worsens with hemodialysis. (Choice C) Subclavian steal syndrome causes exercise-induced arm pain, paresthesia, coolness, and color change. It typically affects the entire arm, not just the hands, and bilateral symptoms would be unexpected. (Choice D) Hypocalcemia can result in hand cramping and paresthesia but typically occurs in association with perioral numbness and tetany. It would not usually be more severe unilaterally or worsen during hemodialysis. (Choice E) Uremic polyneuropathy is common in patients with ESRD but causes progressive pain and paresthesia in the feet, not the hands. Because it is due to uremia, the polyneuropathy typically resolves when dialysis is initiated. Educational objective: Carpal tunnel syndrome is the most common mononeuropathy in patients with end-stage renal disease on dialysis. It is characterized by pain and paresthesia in the lateral hand; symptoms typically worsen during dialysis and are more severe in the arm with vascular access.

Question 18

A 45-year-old man comes to the office for the evaluation of excessive wasting of his extremity muscles, which is more apparent on the extensor side.  The weakness began distally and asymmetrically.  He recently started to have difficulties with swallowing, chewing, and speaking.  He feels some movements in his face and tongue.  He also has muscle stiffness.  His bowel, bladder, cognitive, and sensory functions are intact.  The physical examination reveals excessive wasting of his muscles, which is more prominent in the lower extremities.  Fasciculations and hyperreflexia are noted.  His bulbar reflexes are decreased.  What neural pathway is most likely damaged?

Accepted Answer

Explanation: This patient has amyotrophic lateral sclerosis (ALS), a devastating, progressive neurodegenerative disorder. It is characterized by the presence of both upper motor neuron (UMN) and lower motor neuron (LMN) lesions. Spasticity and exaggerated deep tendon reflexes are signs of UMN damage due to degeneration of frontal motor neurons and their axons. Corticobulbar tract damage due to UMN symptoms, sometimes described as pseudobulbar palsy, can cause dysarthria, dysphagia, and pseudobulbar affect (inappropriate laughing, crying). Fasciculations (sometimes manifesting as movements in the face and tongue), weakness, and atrophy indicate LMN damage in the brainstem and spinal cord. Dysarthria and dysphagia can also be due to LMN injury (ie, bulbar palsy) resulting in weakness of the tongue and other muscles. The soft palate reflex may be weak, particularly in bulbar-onset ALS. Asymmetric limb weakness is the most common presentation of ALS; bulbar symptoms (eg, dysarthria, dysphagia) are the second most common. However, the disease may manifest in any body segment (bulbar, cervical, thoracic, lumbosacral). (Choices A and E) Cerebral cortex and pyramidal tract (ie, corticobulbar and corticospinal tract) lesions would not explain the combination of UMN and LMN damage seen in this patient. Educational objective: Amyotrophic lateral sclerosis (ALS) is characterized by the presence of both upper (eg, spasticity, hyperreflexia) and lower (eg, fasciculations, atrophy) motor neuron lesions.

Question 19

A 56-year-old woman comes to the office with her daughter to discuss knee pain, which has been ongoing for several years. The patient has a high tolerance for pain and has previously refused to see a doctor for her condition. Lately, the pain has been affecting the patient's daily activities, and she spends most of the day sitting in a chair. The knee pain and stiffness are more pronounced on the right side and are worse in the morning but gradually improve by the afternoon. She takes no medications. Review of systems is positive for fatigue. Vital signs are within normal limits. BMI is 31 kg/m². Lower extremity examination shows that both knees are swollen and tender but more so on the right side. The patient is unable to fully flex or extend the right knee. There is mild, bilateral atrophy of the quadriceps. Radiographs of the knees reveal osteopenia of the distal femur, multiple periarticular erosions, and soft tissue swelling. Which of the following is the best long-term management for the most likely diagnosis in this patient?

Accepted Answer

11ec25c8_d13d_fc26_bc81_49a74771dcfa_MD0003_00 The 2 most prevalent forms of arthritis are osteoarthritis (OA) and rheumatoid arthritis (RA), and individuals may have both conditions concurrently. OA is much more common and characteristically involves the knees, hips, and distal finger joints. RA, an inflammatory arthritis, typically involves the metacarpophalangeal, proximal interphalangeal, and wrist joints. However, any joint may be affected. This patient has multiple clinical and radiographic features suggestive of RA: Her prolonged morning stiffness, systemic symptoms (ie, fatigue), and soft tissue swelling involving multiple joints are more consistent with RA than OA; she also has X-ray findings that favor a diagnosis of RA over OA, including periarticular erosions (seen here on hand x-rays), distal osteopenia, and soft tissue swelling. periarticular erosions Additional evaluation of patients with suspected RA should include inflammatory markers (eg, erythrocyte sedimentation rate, C-reactive protein), which are typically elevated in RA but normal in OA. Cyclic citrullinated peptide antibodies are present in most patients with RA and can help confirm the diagnosis; rheumatoid factor should also be tested but has lower specificity. Initial management of RA includes nonsteroidal anti-inflammatory drugs and systemic glucocorticoids (eg, prednisone) to acutely reduce pain; however, these interventions do not alter the long-term prognosis and are inadequate for definitive therapy (Choice C). As soon as possible after the diagnosis is made, disease-modifying antirheumatic drugs (DMARDs) should be initiated to slow disease progression; first-line options include methotrexate, a folate antimetabolite that is cost-effective and well tolerated by most patients. Alternate options include sulfasalazine, leflunomide, and hydroxychloroquine. (Choice B) Nonpharmacologic interventions for OA include regular exercise and weight loss; knee braces may reduce pain in some patients but are unlikely to alter the long-term course of the condition. However, none of these interventions are adequate treatment for RA. (Choice D) Total knee arthroplasty is most commonly performed for patients with advanced OA. It is occasionally needed for patients with severe deformity due to RA, but early initiation of DMARD therapy may eliminate the need for later joint replacement. (Choice E) Urate-lowering therapy is indicated for long-term treatment of gout. Gout commonly affects the knee but typically causes episodic symptoms with brief but severe inflammatory changes (eg, redness, warmth, swelling). X-rays in early gout are generally normal, although chronic findings include punched-out erosions with an overhanging rim of cortical bone ("rat bite" lesion). punched-out erosions Educational objective: Clinical findings that favor the diagnosis of rheumatoid arthritis (RA) over osteoarthritis include prolonged morning stiffness, systemic symptoms, and soft tissue swelling. Other characteristic findings in RA include periarticular erosions on x-ray, elevated inflammatory markers, and positive cyclic citrullinated peptide antibodies or rheumatoid factor. Disease-modifying antirheumatic drugs (eg, methotrexate) slow disease progression and should be initiated on diagnosis. References: Early diagnosis and treatment of rheumatoid arthritis. (http://www.ncbi.nlm.nih.gov/pubmed/29759122) Conventional radiology in rheumatoid arthritis. (http://www.ncbi.nlm.nih.gov/pubmed/28774455)

Question 20

A 38-year-old man comes to the office due to progressive shortness of breath and cough with mucoid sputum over the past 6 months. His shortness of breath is worse with exertion. The patient has no chest pain, weight loss, or night sweats. Past medical history is otherwise unremarkable, and he takes no medications. He smoked cigarettes for 5 years but quit 13 years ago. The patient does not use alcohol or illicit drugs. Temperature is 36.7 C (98 F), blood pressure is 128/78 mm Hg, pulse is 78/min, and respirations are 16/min. His pulse oximetry is 96% on room air. BMI is 32 kg/m². Breath sounds are decreased at the bases, and there are no crackles or wheezes. Heart sounds are normal. There is no jugular venous distension or peripheral edema. Chest imaging reveals bilateral basilar hyperlucency. Complete blood count and basic metabolic panel are normal. Which of the following is the most appropriate next step in management?

Accepted Answer

Explanation: This patient with chronic shortness of breath, productive cough, and evidence of destruction of the lower lung lobes likely has alpha-1 antitrypsin (AAT) deficiency. AAT deficiency-related lung disease presents similarly to other forms of chronic obstructive pulmonary disease (COPD) but results in panacinar (panlobular) emphysema. Smoking-induced centriacinar (centrilobular) emphysema most commonly causes disease in the upper lobes of the lungs, whereas the panacinar emphysema of AAT deficiency classically results in greater destruction of the lower lobes. On average, smokers present in their 30s, whereas nonsmokers present in their 40s. AAT deficiency is frequently associated with liver disease, most commonly resulting in neonatal hepatitis, cirrhosis, or hepatocellular carcinoma. AAT deficiency should be considered in a number of situations, including in patients with: COPD at a young age (age ≤45) COPD with minimal or no smoking history basilar-predominant COPD history of unexplained liver disease Diagnosis is confirmed by measuring serum AAT levels, and pulmonary function testing should also be performed. Treatment includes intravenous supplementation with pooled human AAT. (Choice A) Sleep disordered breathing due to obesity such as obstructive sleep apnea (confirmed by polysomnography) more commonly presents with daytime sleepiness, impaired concentration, and morning headaches. (Choice B) Bronchoprovocation testing with methacholine can be used to confirm asthma diagnosis in selected groups of patients such as those with typical asthma symptoms but normal spirometry. Hyperinflation may be seen on chest x-ray, but basilar hyperlucency is unlikely. (Choice C) An echocardiogram is very useful to evaluate cardiac sources of dyspnea. However, this patient's pulmonary findings along with the lack of jugular venous pressure elevation and peripheral edema make a cardiac cause less likely. (Choice E) Sweat chloride testing is used to confirm the diagnosis of cystic fibrosis (CF); however, adult presentations of CF are more commonly characterized by gastrointestinal, endocrine, and fertility problems. The absence of bronchiectasis on lung imaging also makes CF less likely. (Choice F) Lung biopsy is an invasive procedure with associated morbidity. Measurement of the serum AAT level is less invasive, and low values are highly diagnostic for AAT deficiency. Educational objective: Alpha-1 antitrypsin (AAT) deficiency should be considered in patients who lack typical risk factors for chronic obstructive pulmonary disease (eg, age <45) or those with atypical features (eg, basilar-predominant disease). Diagnosis is confirmed by measuring serum AAT levels. < References: Diagnosis and management of patients with a1-antitrypsin (A1AT) deficiency. (http://www.ncbi.nlm.nih.gov/pubmed/22200689) Alpha-1 antitrypsin deficiency. (http://www.ncbi.nlm.nih.gov/pubmed/20303723)

Exam 1: Medicine