Thursday, June 10, 2010

Hemoptysis Case 3

A 53-year-old man consults a physician because he has begun coughing up sputum tinged with fresh blood. He does not initially report any other symptoms to his physician. When his physician comments on his hoarseness and cough, the patient discounts these symptoms and attributes them to his long smoking history. He cannot say when they began or became worse.

Q 1
The chronic hoarseness suggests dysfunction of which of the following?
/ A. Palate
/ B. Pharynx
/ C. Tongue
/ D. Trachea
/ E. Vocal cords

Q 2
Laryngoscopy reveal a fungating tumor of the larynx that is located between the false and true vocal cords. This tumor is in which of the following sites?
/ A. Aryepiglottic fold
/ B. Infraglottic compartment
/ C. Piriform recess
/ D. Supraglottic compartment
/ E. Ventricle

Q 3
Biopsy of the mass demonstrates a malignancy. Which of the following is the most likely diagnosis?
/ A. Adenocarcinoma
/ B. Lymphoma
/ C. Oat cell carcinoma
/ D. Sarcoma
/ E. Squamous cell carcinoma

Q 4
In addition to smoking, which of the following is an accepted risk factor for this patient's tumor?
/ A. Alcohol use
/ B. Cocaine use
/ C. Coffee use
/ D. Marijuana use
/ E. Tea use

Q 5
The patient returns to clinic three weeks after receiving the news that his cancer is inoperable. His wife reports that he has been more withdrawn, eating and sleeping poorly and "just seems to have lost all hope." On examination, the patient moves very little, never makes eye contact, and admits to "some" depressed mood, Ioss of appetite, and sleep disruption. Which of the following symptoms is of most concern regarding his risk of suicide?
/ A. Decreased appetite
/ B. Decreased energy
/ C. Diminished concentration
/ D. Guilty and worthless feelings
/ E. Hopelessness

Q 6
Some patients present with hoarseness due to a tumor at the lung apex that involves a nerve that is a branch of which of the following?
/ A. Accessory nerve
/ B. Glossopharyngeal nerve
/ C. Hypoglossal nerve
/ D. Phrenic nerve
/ E. Vagus nerve

Hemoptysis Case 3 Answers
The correct answer is E. Hoarseness specifically suggests dysfunction of the vocal cords, which produce the sounds that are then articulated to speech with the pharynx (choice B), palate (choice A), tongue (choice C), teeth, and lips. While vocal cord dysfunction is specifically suggested by hoarseness, the anatomic lesion may or may not be present at the level of the vocal cords. Vocal paralysis may be the result of local tumor or trauma, intracranial lesions affecting the nucleus ambiguus or its supranuclear tracts, and lesions at the base of the skull, neck, or upper portion of the thorax that involve either the vagus nerve or the recurrent laryngeal nerves.
Air from the trachea (choice D) passes into the larynx to reach the vocal cords, but tracheal dysfunction does not produce hoarseness.

The correct answer is E. The interior of the larynx is divided into 3 compartments: the supraglottic compartment (choice D) above the false vocal cords, the ventricle between the false and true vocal cords, and the infraglottic compartment (choice B) below the true vocal cords.
The aryepiglottic fold (choice A) is the upper free border of the quadrangular membrane found in the supraglottic compartment.
The piriform recess (choice C) lies behind the thyroid laminae and the lateral wall of the supraglottic compartment.


The correct answer is E. The epithelial lining of the larynx is squamous epithelium, and the vast majority of cancers of the larynx are squamous cell carcinomas. Squamous cell carcinoma can present with hoarseness, cough, hemoptysis, or difficulty swallowing. If the symptoms develop insidiously (as in the case), the patient may not seek medical attention until late in the course. While very small laryngeal cancers can be treated successfully with surgery and/or radiation, larger ones are much more problematic, in large part because complete resection of the cancer and any lymph node metastases can be difficult to impossible to perform without compromising the many vital neck structures. Patients treated with partial laryngectomy may retain some speech ability. Patients treated with total laryngectomy can often learn to speak again using esophageal speech (gradual belching of air through the pharyngoesophageal junction), a tracheoesophageal fistula (one way valve between the trachea and the esophagus which makes a sound when air is forced across it), or an electrolarynx (sound source held against the neck). With all three techniques, the sound produced is then turned into articulation by the patient's pharynx, palate, tongue, teeth, and lips. The other tumors listed in the choices are very uncommon in the larynx.

The correct answer is A. The only two risk factors that you will need to associate with laryngeal carcinoma are smoking and alcohol use. Cocaine (choice B), coffee (choice C), and tea (choice E) have not been linked to laryngeal cancer.
Marijuana (choice D) has been suggested as a risk factor for oral cancer in a few cases, but a causal association has not been established.

The correct answer is E. Hopelessness is an ominous sign and is associated with a higher risk for suicide.
Decreased appetite (choice A), decreased energy (choice B), and diminished concentration (choice C) are symptoms indicating the presence of a major depressive episode, or are the result of the medical illness.
Guilty and worthless feelings (choice D) are symptoms of depression. They do not predict risk for suicide.

The correct answer is E. The larynx has complex innervation. The vocal cords and most of the area of the larynx below them are supplied by the recurrent laryngeal nerve, which is a branch of the vagus nerve that passes beneath the subclavian artery before returning to the neck to innervate the larynx. From above the larynx, the superior laryngeal nerve arises from the vagus and divides into the internal laryngeal nerve and the external laryngeal nerve. The innervation above the vocal cords is by the internal laryngeal branch of the vagus nerve. The cricothyroid muscle (the only muscle of the larynx not supplied by the recurrent laryngeal nerve) is supplied by the external laryngeal branch of the superior laryngeal, which also branches off the vagus, but which contains motor fibers originally derived from the accessory nerve (choice A).
The glossopharyngeal nerve (choice B) supplies the pharynx.
The hypoglossal nerve (choice C) supplies the tongue.
The phrenic nerve (choice D) supplies the diaphragm.

Hemoptysis Case 2

A fifty-year-old man presents to his primary care doctor complaining of chronic cough with hemoptysis, weakness, frequent urination, thirst, and a decreased ability to concentrate. Review of systems reveals fever, chills, night sweats, and a twenty-pound weight loss. He has a
seventy pack-year smoking history. On examination, his lungs are clear to auscultation, but neurologic examination reveals global hyporeflexia.
A chest x-ray film reveals a 4-cm hilar non-cavitary opacity in the left lung. Serum electrolytes show K+ = 2.3 mEq/L. Calcium is within normal limits.

Q 1
Which of the following is the most likely diagnosis?
/ A. Adenocarcinoma
/ B. Bronchioloalveolar carcinoma
/ C. Mesothelioma
/ D. Small cell carcinoma
/ E. Squamous cell carcinoma

Q 2
Which of the following substances secreted from the lesion is the most likely cause of this patient's electrolyte disturbance?
/ B. Aldosterone
/ C. Angiotensin ll
/ D. Epinephrine
/ E. PTH

Q 3
As the patient's condition progresses, the patient grows increasingly hoarse. Which of the following is the most likely reason for the patient's hoarseness?
/ A. Enlargement of the mass has severely decreased inspiratory volume.
/ B. Extension of the mass into the larynx
/ C. Impingement on cranial nerve XII
/ D. Impingement on the recurrent laryngeal nerve
/ E. Metastasis into the speech centers of the brain

Q 4
This patient is given cisplatin as part of his chemotherapeutic regimen. Which of the following is a known adverse effect of cisplatin?
/ A. Cardiotoxicity
/ B. Hemorrhagic cystitis
/ C. Nephrotoxicity
/ D. Profound myelosuppression
/ E. Pulmonary fibrosis

Q 5
In addition, this patient is given etoposide. Which of the following best describes the mechanism of action of etoposide?
/ A. Etoposide cross-Iinks DNA
/ B. Etoposide induces single- and double-stranded breaks in DNA
/ C. Etoposide inhibits dihydrofolate reductase
/ D. Etoposide inhibits microtubule formation
/ E. Etoposide inhibits topoisomerase ll

Q 6
The patient has been aware of his diagnosis and prognosis for several weeks now. He makes the statement "I had successes and failures but I'm pretty sure l got as much living out of the last 50 years as anybody could." This statement suggests that the patient is experiencing which of the following of Erikson's stages?
/ A. Ego integrity vs. despair
/ B. Generativity vs. self absorption
/ C. Identity vs. role confusion
/ D. Industry vs. inferiority
/ E. Intimacy vs. isolation

Hemoptysis Case 2 Answers
The correct answer is D. This patient has classic findings for small cell carcinoma of the lung (also known as oat cell carcinoma). Patients with small cell carcinoma typically present with constitutional symptoms, cough, and hemoptysis. Paraneoplastic syndromes are common with this cancer, and may often be the first symptoms to present. This patient has hypokalemia and many of the symptoms that accompany it: muscular weakness, frequent urination, thirst, and decreased ability to concentrate. This patient's radiographic findings (non-cavitary hilar mass) also support the diagnosis of small cell carcinoma.
Adenocarcinomas (choice A) tend to present as peripheral masses, and paraneoplastic syndromes are rare.
Bronchioloalveolar carcinoma (choice B) is a subtype of adenocarcinoma. It arises from the peripheral airways, and while it can present as a discrete mass, it typically is indistinct radiographically as it grows down the airway surfaces. Again, paraneoplastic syndromes are rare.
Mesothelioma (choice C) is a pleural malignancy associated with asbestos exposure. They do not present as hilar masses. 50% metastasize, but death usually results from local extension. They tend to produce large effusions that may obscure the mass on chest x-ray films.
Squamous cell carcinoma (choice E), like small cell carcinoma, has a strong association with smoking. These tumors may present in a similar fashion, with a more central lung lesion, but paraneoplastic syndromes with squamous cell carcinoma tend to be related to the secretion of a PTH-like substance, resulting in hypercalcemia. Thus the diagnosis of small cell carcinoma is more likely.

The correct answer is A. ACTH is often secreted by small cell lung carcinoma. The clinical results are those seen from uninhibited ACTH secretion: the adrenal gland increases the production of glucocorticoids and mineralocorticoids. Thus, patients experience hypokalemia, hyperglycemia, and hypertension. The chronic sequelae of this process (Cushingoid symptoms) are not usually seen, due to the metabolic demands of the neoplasm and the short survival of affected individuals.
None of the other hormones listed above are commonly secreted by small cell cancers.

The correct answer is D. As seen with many hilar masses, impingement on the recurrent laryngeal nerve produces hoarseness. The recurrent laryngeal nerve courses inferiorly to the aortic arch and then turns superiorly to innervate the intrinsic muscles of the larynx (except the cricothyroid muscle). When this nerve sustains unilateral damage, hoarseness results, as patients have difficulty abducting the vocal cords. Bilateral damage results in acute breathlessness, because both of the vocal cords move to the midline and block the airway.
Enlargement of the mass (choice A) to a size that would affect speech would likely produce pulmonary collapse, not hoarseness.
Extension of the mass from the hilum of the lung to the larynx (choice B) would be unlikely. Many critical structures are in this course, and the patient would likely not survive long enough to become hoarse from direct extension.
Impingement on cranial nerve XII (choice C) would affect movement of the tongue. Not only would metastasis or direct extension to the anatomic site of this nerve be unlikely, this event would not make a patient hoarse.
Metastasis to the speech centers (choice E) would produce more profound speech difficulties. The characteristics of the aphasia would depend upon which speech center is affected ( i.e., fluent vs. expressive aphasia).

The correct answer is C. Cisplatin is a heavy metal compound used in the treatment of small cell carcinoma of the lung. Cisplatin cross-links DNA, though it is not a true alkylating agent. The dominant adverse effect seen with its administration is nephrotoxicity, as it is toxic to both proximal and distal renal tubule epithelium. It produces only modest myelosuppression.
Doxorubicin causes cardiotoxicity (choice A) and congestive heart failure may result.
Cyclophosphamide is a chemotherapeutic agent known to cause hemorrhagic cystitis (choice B).
The myelosuppression with cisplatin is modest. Many other chemotherapeutic agents are known to severely suppress the marrow (choice D).
Bleomycin is known to cause pulmonary fibrosis (choice E).

The correct answer is E. Etoposide acts by inhibiting the enzyme topoisomerase II. Topoisomerases are enzymes that create and repair breaks in DNA during replication. This enzyme is needed to relieve topologic and conformational changes as the DNA is "unzipped" during replication and transcription.
Alkylating agents, such as cyclophosphamide, cross-link DNA (choice A).
The antitumor antibiotics, such as bleomycin, act by inducing breaks in DNA (choice B).
Methotrexate is an antimetabolite chemotherapeutic agent that inhibits dihydrofolate reductase (choice C), an enzyme needed to produce purine nucleotides. Thus the "purine shortage" produced hinders cell replication.
The Vinca alkaloids exert their effects by inhibiting microtubule formation (choice D). Without microtubules, cells cannot mobilize their chromosomes, and thus mitosis is inhibited.

The correct answer is A. In the ego integrity vs. despair stage, individuals reconcile their achievements and failures and face the fact that any human life is limited. Persons completing this task find self-worth in reviewing their life events. Persons stuck at this task cannot admit that time has run out.
Generativity vs. self-absorption (choice B) usually occurs from age 30-65. It involves noting ones contributions as a parent (in the broadest sense) and eventually "passing the torch" to the next generation. Persons stuck in this phase refuse to give up any power to their successors, and maintain ultimate authority without making a place for the next generation.
Identity vs. role confusion (choice C) occurs during adolescence, and is usually defined by emotional and or geographic separation from the actual parents, with an internal identity based on both similarities and differences from parental traits and values.
Industry vs. inferiority (choice D) is a stage in which school age (6-12) children explore peer groups and learn that industry (hard work) generally pays off.
Intimacy vs. isolation (choice E) is the stage, in the early twenties, when young adults connect to their peers and significant others in a meaningful and mature way, putting aside fear and inhibitions.

Hemoptysis Case 1

A 28-year-old man is evacuated by ambulance from his work to the emergency department of a local hospital after he abruptly develops massive hemoptysis. When seen in the emergency department, he is already hypotensive and requires transfusion to maintain blood
pressure. Emergency department personnel perform intubation with assisted ventilation to maintain his respiratory function. A chest x-ray film shows diffuse bilateral alveolar infiltrates. According to the patient's wife, the man had a number of minor episodes of hemoptysis with blood- tinged sputum during the past year, but discounted them and refused to seek medical attention.

Q 1
Which of the following is the most likely diagnosis?
/ A. Bronchogenic carcinoma
/ B. Goodpasture syndrome
/ C. Idiopathic pulmonary fibrosis
/ D. Pneumonia
/ E. Tuberculosis


The man is admitted to the hospitaI, and during his hospitalization, is also found to have hematuria, proteinuria, red cell casts in the urine, and rising serum creatinine. A hematoxylin and eosin stained section of a renal biopsy is shown above. Which of the following is the most likely diagnosis?

/ A. End-stage renal disease
/ B. Glomerulonephritis
/ C. Kimmelstiel-Wilson nodules
/ D. Pyelonephritis
/ E. Tubulointerstitial nephritis


A second section from the renal biopsy was stained with immunofluorescent antibodies to human lgG (and human complement) with the result shown. Which of the following is most likely the antigen against which the lgG antibody is directed?
/ A. Collagen
/ B. EIastin
/ C. Fibronectin
/ D. Laminin
/ E. Vitronectin

Q 4
The antigenic determinant against which the lgG is specifically directed is located in the C-terminal domain of one peptide chain of the
molecule. The nucleotide sequence encoding this region is known, and a small portion of the intron (Iower case) and exon (UPPER CASE)
sequence of this region is shown below:
c a t t a g G A A C T C T T G
How many amino acids of the this antigen are translated from the in-frame sequence shown?
/ A. 1
/ B. 2
/ C. 3
/ D. 4
/ E. 5

Q 5
Which of the following would be the most appropriate pharmacotherapy for this patient?
/ A. Erythropoietin
/ B. Famciclovir
/ C. Foscarnet
/ D. Isoniazid
/ E. Methylprednisolone

Q 6
Over the next six months, the patient is treated with repeated plasmapheresis. Which of the following is the rationale of this therapy for this patient?
/ A. Decrease blood clotting factors
/ B. Increase hematocrit
/ C. Increase platelets
/ D. Protect against infection
/ E. Reduce circulating autoantibodies


Hemoptysis Case 1 Answers

The correct answer is B. Goodpasture syndrome is a hypersensitivity disorder of unknown cause that can cause hemoptysis and dyspnea secondary to bleeding from the alveolar capillary bed. The pulmonary hemorrhage may be mild to massive. Most patients, as in this case, are young men.
Bronchogenic carcinoma (choice A) and tuberculosis (choice E) can cause pulmonary hemorrhage, which is usually not massive, but can be excluded because a mass lesion was not seen on chest x-ray.
Idiopathic pulmonary fibrosis (choice C) and pneumonia (choice D) could both cause diffuse pulmonary changes visible on x-ray, but would be unlikely to cause massive hemoptysis.


The correct answer is B. In addition to lung involvement, Goodpasture syndrome is also characterized by severe renal involvement which may precede, coexist with, or follow lung involvement. Renal biopsy characteristically shows rapidly progressive glomerulonephritis with crescent formation in Bowman's spaces.
End-stage renal disease (choice A) with marked scarring of the renal cortex and loss of glomeruli can be seen after many years of kidney disease of many types.
Kimmelstiel-Wilson nodules (choice C) are characteristic of diabetic nephropathy.
Pyelonephritis (choice D) is an infection of the kidney, and can complicate a urinary tract infection or be due to hematogenous dissemination of bacteria.
Tubulointerstitial nephritis (choice E) can be seen with drug reactions and toxins.

The correct answer is A. The photomicrograph shows the linear immunofluorescence characteristic of Goodpasture syndrome. The antigenic determinant in Goodpasture syndrome is on the alpha-3 chain of type IV (basement membrane type) collagen.
Elastin (choice B), fibronectin (choice C), laminin (choice D) and vitronectin (choice E) are all found in the extracellular matrix. These molecules could conceivably be antigenic, but have not been implicated in Goodpasture syndrome.


The correct answer is C. The intron sequence (lower case) is a non-coding region between exons. Therefore, the intron portion of the sequence above (cattag) does not encode any amino acids in the Goodpasture antigen. The 9 nucleotides of the in-frame exon sequence shown (UPPER CASE) would be translated into 3 amino acids (3 nucleotides/amino acid) of the Goodpasture antigen. None of the 3 codons shown (GAA, CTC, TTG) is a stop codon.
If all the nucleotides shown were part of an exon, the sequence would code for only 1 amino acid (choice A) because the second triplet (tag) is a stop codon.


The correct answer is E. Corticosteroids and cyclophosphamide are used to prevent the formation of new antibodies and control the inflammatory response in Goodpasture syndrome. Intravenous administration of corticosteroids, such as methylprednisolone or prednisone, or immunosuppressive therapy with cyclophosphamide is usually carried out for several days.
Erythropoietin (choice A) is a glycoprotein, normally produced in the kidneys, that stimulates red blood cell production. It is indicated for treatment of anemia associated with chronic renal failure, zidovudine therapy in HIV-infected patients, and for cancer patients on chemotherapy. It is contraindicated in patients with uncontrolled hypertension and hypersensitivity to mammalian cell-derived products or to human albumin.
Famciclovir (choice B) is a synthetic acyclic purine nucleoside analog that interferes with DNA synthesis in herpes simplex virus (types 1 and 2) and varicella zoster virus. It is used in the treatment of genital herpes in patients who are not immunocompromised.
Foscarnet (choice C) is an agent that inhibits the replication of all known herpes viruses, including cytomegalovirus, herpes simplex virus (types 1 and 2), human herpesvirus 6, Epstein-Barr virus and varicella zoster virus. This agent is indicated for the treatment of cytomegalovirus (CMV) retinitis, mucocutaneous herpes simplex virus (HSV), and acyclovir-resistant HSV in immunocompromised patients.
Isoniazid (choice D) is an agent indicated for the treatment of all forms of tuberculosis (TB) as well as the prophylaxis of tuberculosis. This agent is associated with the development of severe and sometimes fatal hepatitis. Peripheral neuropathy is the most common side effect seen with isoniazid therapy and is associated with a symmetrical numbness and tingling of the extremities. Pyridoxine administration can treat/prevent this condition.


The correct answer is E. In plasmapheresis, the patient's blood is removed (in small amounts over time). The blood cells are collected and the plasma is discarded, and then the blood cells are resuspended in someone else's plasma before being returned to the patient's body. Plasmapheresis is a very effective, albeit expensive, way of washing autoantibody out of the patient's body, and together with pharmacologic immunosuppression has markedly changed the formerly dismal prognosis of Goodpasture syndrome. Surviving patients often stop producing clinically significant amounts of autoantibody within 12 to 18 months, although some patients may require longer-term immunosuppressive therapy.