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103707142 Respiratory Medicine 1 50

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    Respiratory Medicine Pastest

    1. A 49-year-old woman has been admitted with haemoptysis and epistaxis, the chest X-rayshows multiple rounded lesions with alveolar shadowing. Laboratory parameters show a

    leucocytosis without eosinophilia but with microhaematuria, proteinuria as well asantineutrophil cytoplasmic antibodies (c-ANCA). Which drug treatment is the most

    appropriate?

    Erythromycin

    Ampicillin

    Cyclophosphamide in combination with corticosteroids Your answer

    Ciclosporin

    Aciclovir

    The combination of prednisolone and cyclophosphamide is now established as the standardinduction therapy for patients with generalised Wegeners granulomatosis or microscopic

    polyangiitis. There is consensus on how corticosteroids should be used, but less so for

    cyclophosphamide. Prednisolone is given in doses of around 1 mg/kg per day initially, afterwhich the dose is reduced rapidly, typically at weekly intervals. Controlled trials show that

    the addition of pulses of methylprednisolone is unlikely to confer additional benefit.

    Traditionally, patients received daily oral cyclophosphamide (2 mg/kg per day), but latterly

    intravenous boluses have proved increasingly popular, given in doses of 0.50.75 g/m2

    body surface area at intervals of 2 weeks (at least for short periods) to 2 months.

    2. A 30-year-old man from Somalia attends your clinic with a productive cough.Sputum is smear-positive for tuberculosis. What does this mean?

    He needs treatment for tuberculosis and his close contacts needscreening, but he is not infectious to casual contacts

    He needs treatment for tuberculosis, his close contacts need

    screening and he needs to be isolated from casual contacts

    Your

    answer

    He needs treatment for tuberculosis, but he is not infectious toclose or casual contacts

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    He has multidrug-resistant tuberculosis

    He has HIV-associated tuberculosis

    Smear-positive tuberculosis means the patient is highly infectious to both closecontacts (more than 8 hours together per day) and casual contacts, such as otherpatients on the ward and healthcare workers. He therefore needs isolating in anegative-pressure room and contacts should wear particulate masks until he hasreceived antituberculous therapy for 2 weeks. The sputum may remain positiveafter this time, but the organisms will be dead. Culture-positive tuberculosismeans the immediate smear is negative, but prolonged culture has showntuberculosis. Smear-positive TB does not necessarily indicate drug resistance orassociated HIV infection.

    3. A 69-year-old former coal-miner is referred to you by the on-call team. There is asmoking history and he has been managed by his GP for COPD. He has been admitted with

    dyspnoea that is now so bad that he is unable to manage at home and cannot walk from the

    chair to the bathroom. There is a cough productive of black sputum. Lung function testsshow a mixed restrictive and obstructive picture. A chest X-ray shows marked changes

    with massive fibrotic masses predominantly in the upper lobes. There are also changes

    consistent with lung destruction and emphysema. His rheumatoid factor is positive. Which

    diagnosis fits best with this clinical picture?

    Progressive Massive Fibrosis (PMF) Your answer

    Chronic obstructive pulmonary disease

    Tuberculosis

    Asthma

    Category 1 pneumoconiosis

    PMF is associated with fibrotic masses in the apices, sometimes up to 10 cm in diameter.

    There are also emphysematous changes. Usually there is a history of dust inhalation such

    as coal dust, and rheumatoid factor and antinuclear antibody are often positive. There is a

    mixed obstructive and restrictive lung defect with reduced transfer factor. PMF mayrapidly progress, even in the absence of further dust exposure, leading to respiratory failure

    and eventually death. Category 2 pneumoconiosis progresses to PMF in around 7% of

    cases, the rate of progression of category 3 pneumoconiosis is much higher, at around 30%.

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    4. A 56-year-old woman, who is known to suffer from rheumatoid arthritis,

    complains she has had recurrent haemoptysis for over 5 years. She has neversmoked and only takes a non-steroidal anti-inflammatory agent. According toher, she coughs up phlegm every day and at times this contains streaks of freshblood. She has no known respiratory disease, but tends to get frequent chestinfections that are relieved by a course of antibiotics. What is the most likelydiagnosis?

    Lung cancer

    Bronchiectasis

    Your answer

    Tuberculosis

    Pulmonary embolism

    Atypical pneumonia

    Some 34% of patients with rheumatoid arthritis develop bronchiectasis. This ischaracterised by recurrent haemoptysis. The history of expectorating phlegm onmost days and frequent chest infections are suggestive of the diagnosis. A high-resolution computed tomography (CT) scan of her lungs will establish thediagnosis.

    5. You review a 56-year-old man with chronic obstructive pulmonary disease, who issuffering an acute exacerbation. Which of the following features would suggest suitability

    for non-invasive ventilation?

    Profound hypoxia

    Hypercapnia without profound hypoxia Your answer

    Confusion

    Cryptogenic fibrosing alveolitis

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    Decreased conscious level

    The answer is (b). Non-invasive ventilation (NIV) is suitable for patients who are

    conscious, cooperative and able to protect their airway. Continuous positive airwaypressure (CPAP) or bilevel positive airway pressure (BIPAP) is applied via a tight-fitting

    nose- or face-mask. NIV may be particularly useful in a patient suffering acute hypercapnicrespiratory failure associated with chronic obstructive pulmonary disease, if the patient is

    not profoundly hypoxic. NIV may also be an option in a patient who has mild hypoxiaassociated with community-acquired pneumonia. There is a reduced risk of ventilator-

    associated pneumonia, and improved patient comfort and preservation of airway protection

    mechanisms.

    6. A 30-year-old asylum seeker has been complaining of cough, fever and weight loss. Thechest X-ray shows a large, upper lobe lesion, the sputum shows acid-fast bacilli that are

    confirmed asMycobacterium tuberculosis by polymerase chain reaction (PCR). Drug

    therapy with isoniazid, rifampicin, ethambutol and pyrazinamide has been started under

    directly observed therapy (DOT). During the next 4 weeks the disease is still progressing.What is the most likely reason?

    Infection with multi-resistant tuberculosis Your answer

    Infection with atypical mycobacteriae

    Underlying bacterial pneumonia

    Carcinoma of the lung

    Aspiration pneumonia

    Multidrug-resistant tuberculosis is defined as resistance to rifampicin and isoniazid with or

    without resistance to other anti-TB drugs. The treatment of patients with drug-resistanttuberculosis should only be carried out by specialist physicians with appropriate experience

    in the management of such cases. Initial drug resistance is uncommon (< 2%) in previously

    untreated White patients born in the UK. Higher levels of resistance occur in ethnic

    minority groups, particularly those of the Indian subcontinent and Black-African ethnicorigin, with isoniazid resistance occurring in 46% of such patients. HIV-positivity,

    independent of ethnic group, is also a marker for increased drug resistance: a positive HIV

    result increases at least fourfold the chances of single- or multiple-drug resistance

    compared with an HIV-negative individual.

    Treatment is complex, time-consuming and demanding for both the patient and the

    physician. Such treatment should only be carried out by physicians with substantial

    experience in managing complex resistant cases, and only in hospitals with appropriateisolation facilities. This may require transfer of the patient to an appropriate unit.

    Treatment of such patients has to be planned on an individual basis and needs to include

    reserve drugs. Such treatment must be closely monitored because of increased toxicity, but,

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    more importantly, full compliance is essential to prevent the emergence of further drug

    resistance. Therefore all such treatment must be directly observed throughout, both on an

    in-patient and an out-patient basis. Treatment should start with five or more drugs to whichthe organism is, or is likely to be, susceptible and continue until sputum cultures become

    negative. Drug treatment then has to be continued with at least three drugs to which the

    organism is susceptible on in vitro testing for a minimum of a further 9 months and perhapsup to or beyond 24 months, depending on the in vitro drug-resistance profile, the available

    drugs and the patients HIV status. Consideration may also have to be given to resection of

    pulmonary lesions under drug cover.

    7. Which of the following is the best agent for treating chlamydia pneumonia?

    Clarithromycin Your answer

    Piperacillin

    Clindamycin

    Ampicillin

    Imipenem

    Macrolide antibiotics (eg clarithromycin or erythromycin) are the treatment of choice for

    chlamydia and other atypical pneumonias. The most frequent side-effects are nausea,

    vomiting and diarrhoea. It should be kept in mind that treatment is likely to be mosteffective when given over a long rather than a short time, suboptimal doses are avoided and

    compliance is strict.

    8. You are asked to see a 32-year-old immigrant who complains of chronic cough and

    weight loss over the past few months. Examination of sputum reveals acid and alcohol fastbacilli (AAFBs) and tuberculosis is confirmed. You elect to begin treatment with isoniazid,

    rifampicin, ethambutol and pyrazinamide as he is from an area where high levels of drug

    resistance are present. Which of the following blood tests is most desirable before startingtherapy?

    Liver function testing Your answerSerum calcium

    Renal function testing

    Clotting

    Full blood count

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    Both isoniazid and rifampicin may be associated with significant hepatic dysfunction. In

    particular, severe and sometimes fatal hepatitis has been seen with use of isoniazid.

    Particular problems occur in slow acetylators who have markedly elevated serum isoniazidlevels. In patients with existing liver dysfunction, rifampicin and isoniazid should only be

    used in cases of absolute clinical necessity. Even then, dose reduction of rifampicin is

    recommended and initial weekly monitoring of liver function tests should be carried out.

    9. A 67-year-old woman has been diagnosed as suffering from bronchiectasis on a high-

    resolution computed tomography (HRCT) scan of the lung. Which one of the followingstatements is NOT true?

    She is at risk of developing a pneumothorax

    She is at risk of developing a brain abscess

    Massive haemoptysis is the commonest cause of death in her age group Your answerRecurrent chest infections are likely at her age

    Her immunoglobulin levels should be checked

    Congenital and acquired immunodeficiencies are well-known causes of bronchiectasis. To

    exclude them, the serum immunoglobulins should be checked. In particular, the IgG

    subclass (IgG1, -2, -3 and -4). Patients with an immunoglobulin deficiency should betreated with regular immunoglobulin infusions. Pneumothorax, recurrent chest infections

    and brain abscesses are well-known complications of bronchiectasis. Haemoptysis is a

    common symptom but is rarely a cause of death.

    10.Which one of the following conditions is MOST likely to be associated with lowFEV1 and normal TLCO?

    Asthma

    Your answer

    Emphysema

    Sarcoidosis

    Pulmonary hypertension

    Fibrosing alveolitis

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    The diffusion of CO from the alveoli to the pulmonary blood is governed by theintegrity of the alveolar membrane, the capillary blood volume, or both (air-blood barrier). A reduction in the diffusion capacity of CO is encountered inconditions affecting the capillary bed size such as pulmonary emboli andpulmonary vasculitis, or conditions that cause changes in the characteristics of

    the alveolar membrane which include diseases in which some form of intra-alveolar filling process has occurred and the air to blood diffusion pathway isactually lengthened (pneumonia, pulmonary oedema, alveolar proteinosis).Similarly, TLCO is reduced in patients with infiltrative disorders of the lung thataffects both the capillary bed size and the alveolar membrane integrity such assarcoidosis, interstitial lung diseases, or collagen vascular diseases. Removal ordestruction of lung tissue, such as surgery or emphysema decreases bothmembrane and blood volume components and produces low TLCO. An increase inTLCO results occasionally from an increase in capillary blood volume secondaryto haemodynamic changes in pulmonary circulation; an increase in pulmonaryarterial or left atrial pressures, as in congestive heart failure, or an increase in

    pulmonary blood flow, as in arterial septal defect. The TLCO is sometimesincreased in patients with bronchial asthma during an attack, but the cause ofthis change is not known. Alveolar haemorrhage from any cause can result in afalse increase of TLCO despite the presence of an underlying diffusion defect.

    11. Which of the following does not increase the risk of death in patients with severepneumonia?

    Diastolic blood pressure < 60 mmHg

    Age 49 years Your answer

    Urea > 7 mmol/l

    WBC count < 4 109/l

    Atrial fibrillation

    Patients over 60 years of age with severe pneumonia have an increased of risk of dying.

    The other factors that increase the risk of death are tachypnoea (respiratory rate > 30/min),

    underlying disease, confusion, multi-lobular involvement, albumin < 35 g/l, hypoxia pa(O2)< 8 kPa, leucocytosis > 20 109/l and bacteraemia.Leukopaenia is also associated with a

    higher rate of mortality in pneumonia.

    12. Which one of the following statements with regard to sarcoidosis is true?

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    Parenchymal lung disease is often accompanied with pleural effusion

    Clubbing of the fingers is an early feature

    Jaundice and portal hypertension are the predominant features of hepaticsarcoidosis

    A positive tuberculin test in a patient with chronic sarcoidosis is

    suggestive of concomitant tuberculosis

    Your

    answerHypercalcaemia when it manifests is usually resistant to steroid therapy

    Sarcoidosis is a systemic disorder of unknown cause that is characterised by its

    pathological hallmark, the noncaseating granuloma primarily affecting the respiratory tract,

    skin, eye, heart, kidneys and liver. Pleural disease is relatively infrequent, with effusions

    occurring in fewer than 5% of patients. Clubbing of the fingers is not a recognised featureof sarcoidosis. Although liver biopsy reveals granulomatous involvement in 4070% of

    patients, clinically significant hepatic disease is rare. A tuberculin test is usually negative in

    chronic sarcoidosis, however, most sarcoidosis patients who develop tuberculosis becometuberculin-positive. Hypercalcaemia, a potentially important complication of sarcoidosis,

    occurs in fewer than 10% of patients and is thought to be due to elevated levels of 1,25-dihydroxyvitamin D (calcitriol), which is produced by macrophages within the granulomas.High-dose glucocorticoids are very helpful in vitamin D intoxication, granulomatous

    diseases such as sarcoidosis, and haematologic malignancies known or likely to be

    glucocorticoid-responsive.

    13. A 37-year-old man presents with increasing breathlessness and is diagnosed afterinvestigation with emphysema.

    Which of the following conditions is most likely to predispose to the development of

    emphysema?

    Allergic bronchopulmonary aspergillosis

    Hypogammaglobulinaemia

    Childhood bronchiolitis Your answer

    Bronchial adenoma

    Crohns disease

    McLeods syndrome is unilateral emphysema following childhood bronchiolitis.Emphysema is most commonly related to smoking, although it may also be associated to

    1-antitrypsin deficiency. The other stems are associated with bronchiectasis.

    Bronchiectasis may occur as a consequence of bronchial obstruction either by intrinsic orextrinsic pathology. Infections such as tuberculosis and previous pneumonia may lead to

    bronchiectasis. Congenital syndrome such as cystic fibrosis, immotile cilia syndrome and

    Kartageners syndrome may also be implicated in the development of bronchiectasis.Inflammatory bowel disease has also been associated with development of bronchiectasis.

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    14. The nurses on the ward ask you to look at a chest drain that has beeninserted in a patient for a pneumothorax. They are concerned about whether itis still working. When the patient coughs, nothing happens. When he breathes in

    and out, the fluid in the tube moves up and down. What does this mean?

    Air and fluid are draining from the pleural space

    Fluid alone is draining from the pleural space

    Air is no longer draining from the pleural space as the drain isblocked

    Air is no longer draining from the pleural space, but the drain isstill working

    Your

    answer

    He needs to commence suction to the drain

    The fluid level rising and falling in the drain swinging shows it is still in contactwith the pleural space and the fluid level is moving with respiration. Air is notbubbling out of the drain when the patient coughs, as the air has stopped

    draining from the pleural space and the lung has re-inflated. If a drain does notbubble or swing, then it is blocked or kinked and is not working. With a simplepneumothorax, there would be minimal fluid drainage from the chest. Suction isnecessary if the drain is still bubbling, but the lung has not fully re-inflated onthe chest X-ray.

    15. A patient presents with symptoms suggesting bronchiectasis and abdominal distension,bloating and foul-smelling faeces. What is the most likely diagnosis?

    Carcinoma of the lung

    Wegeners granulomatosis

    Pneumococcus pneumonia

    Goodpastures syndrome

    Cystic fibrosis Your answer

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    The United States Cystic Fibrosis Foundation Registry data show that as many as 10% of

    cystic fibrosis patients are not diagnosed until adult life. The main presentation is with

    respiratory problems, usually recurrent lower respiratory infections with chronic sputumproduction. Some patients are given a prior diagnosis of bronchiectasis, atypical asthma,

    nasal polyposis or allergic bronchopulmonary aspergillosis. A new diagnosis of cystic

    fibrosis has been described even in adults in their seventh decade. Depletion of sodium,chloride and potassium due to excessive sweating, and secondary renal chloride retention,

    may result in presentation with dehydration and heat exhaustion in an otherwise apparently

    completely fit adult. Pancreatic insufficiency can lead to steatorrhoea. The vast majority ofpatients with cystic fibrosis can be diagnosed by a sweat test.

    16. A 38-year-old man presents with emphysema. He is a non-smoker. He also has

    abnormal liver function tests, and his liver biopsy reveals evidence of cirrhosis. You

    suspect alpha-1 antitrypsin deficiency. What is the genotype that fits best with this clinicalpicture?

    PiMM

    PiMZ

    PiSZ

    PiZZ Your answer

    PiSS

    This man has severe disease, which is most commonly associated with the PiZZ genotype.Such a patient may even present with liver disease in childhood and require eventual

    transplantation. Around 1015% of patients with PiZZ genotype develop cirrhosis by theage of 50 years, and 75% develop respiratory problems. Heterozygotes are less likely to

    develop liver disease, and tend to develop less severe lung disease, although the PiMZ orPiSZ genotypes (heterozygotic state) may accelerate emphysema associated with smoking.

    Levels of1-antitrypsin correlate with genotype; in homozygotes they run at around 10%of the normal level, while heterozygotes have levels at around 60% of normal. Best

    prognosis from1-antitrypsin deficiency is seen in non-smoking patients; for this reason,advise patients to refrain from smoking at all costs.

    17. A 72-year-old man is admitted via the on-call team with an acute respiratory tractinfection. He has a 40 pack-year smoking history and continues to smoke 15 cigarettes per

    day. After recovery from his initial infection, lung function testing indicates that his FEV 1(forced expiratory volume in 1 second) is less than 50% of predicted, and chronicobstructive pulmonary disease (COPD) is diagnosed. This is his third exacerbation this

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    year so far, which have necessitated hospital admission. What is the best pharmacological

    intervention for him?

    Salbutamol prn for symptom relief

    Low-dose beclometasone inhaled-steroid therapy as prophylaxis

    High-dose inhaled steroid therapy as prophylaxisInhaled anticholinergics for symptom relief

    Combination therapy with a high-dose inhaled steroid and a long-acting

    2-agonistYour

    answer

    Recent evidence has shown that, for patients who suffer recurrent severe exacerbations of

    COPD and who have an FEV1 less than 50% predicted, combination therapy with high-

    dose inhaled steroids and a long-acting b2-agonist may reduce severe exacerbations. Along-term study (TORCH) is continuing to ascertain if this combination may be disease-

    modifying. Of course, this combination should be used in conjunction with smoking

    cessation where possible, and long-term home oxygen therapy if the criteria for its

    prescription are met. COPD carries a worse long-term survival than some cancers. Oncerespiratory failure criteria have been met, the 5-year survival rate is only around 25%.

    There is now value in using low-dose inhaled steroid therapy in the management of COPD.

    18. Which lung disease is associated with the clinical observations pink puffer and blue

    bloater?

    Cystic fibrosis

    Pulmonary fibrosis

    COPD Your answer

    Small-cell lung cancer

    Tuberculosis

    Pink puffers have a good respiratory drive.

    Features include: purse-lip breathing with intense dyspnoea, patient is often thin and elderly,little sputum produced, oedema and overt heart failure are rare complications.

    Investigations: blood gases are near normal until pre-terminally there is very severe airwaysobstruction, total lung capacity is increased, there is a reduction in transfer factor.

    Blue bloaters have a poor respiratory drive.

    Features include: quite mild dyspnoea, patient often obese, large volumes of sputum

    produced, infective exacerbations, patient often oedematous, may develop cor pulmonale.

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    Investigations: blood gases hypercapnia, hypoxaemia, elevated plasma bicarbonate, severe

    nocturnal hypoxaemia, airways obstruction may only be moderate, transfer factor

    approximately normal.

    19. An 18-year-old student presents to A&E suffering from severe pharyngitis and earache,

    there is also lethargy and muscle ache. He also complains of wheezing, but there is noprevious history of asthma. On examination he has a non-exudative pharyngitis and bullous

    myringitis, wheeze on auscultation but no evidence of consolidation. There is erythema

    nodosum. The white blood cell count is 14 109/l , but there is no specific indication ofbacterial infection. Cold agglutinins are detected. A chest X-ray reveals dramatic upper-

    lobe consolidation quite out of character with the clinical examination. What diagnosis best

    fits this clinical picture?

    Streptococcus pneumoniae pneumoniaHaemophilus influenzae pneumonia

    Tuberculosis

    Mycoplasma pneumonia Your answer

    Chlamydia pneumonia

    Mycoplasma occurs in epidemics occurring every 4 to 5 years, more commonly amongstclose-knit populations like those in schools and colleges. Peak age of presentation is 5-20

    years, and mycoplasma is said to account for 7% of community-acquired pneumonia. Cold

    agglutinins are associated with up to 50% of cases, muscle tenderness is said to occur in up

    to 50% of cases and, often, X-ray appearances are much worse than is suggested by theclinical examination of the respiratory system. Treatment is a 2-week course of

    erythromycin or clarithromycin. X-ray resolution is complete by week 8 in around 90% ofpatients.

    20. The anatomical dead space may be used to calculate alveolar ventilation by subtracting

    it from the tidal volume and multiplying the result by the respiratory rate. What would youexpect the normal anatomical dead space to be in a healthy adult male?

    50 ml

    150 ml Your answer

    250 ml

    350 ml

    450 ml

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    The normal anatomical dead space is approximately 150 ml. If we take tidal volume to be

    about 500 ml and respiratory rate to be about 15/minute, this gives a normal alveolar

    ventilation of (500 150) 15 = 5250 ml/min. Diseases that cause an additionalphysiological dead space where parts of lung do not take part in gas exchange, eg

    pneumonia, may increase this dead space.

    21. A 74-year-old man, who is a retired builder of railway carriages, presents to his GP.Over the past year or so he has noticed gradually increasing shortness of breath on exertion.

    He has a frequent dry and non-productive cough. On examination the GP notices that he

    has digital clubbing, peripheral oedema and a raised JVP, with fine end-inspiratory crackles

    heard at the bases on auscultation. Chest X-ray reveals irregular shadows in the lower lung

    zones and thickened pleural plaques affecting the lower zones. What diagnosis would fitbest with this clinical history and these findings on examination and investigation?

    Asthma

    Bronchial carcinoma

    Cryptogenic fibrosing alveolitis

    Asbestosis Your answer

    Silicosis

    The clue lies in this mans occupation since asbestos fibres were often used in the buildingof railway carriages. There is a long gap between exposure to the fibres and asbestosis of

    some 2030 years, therefore many cases are still coming to light, and quite some time afterthe exposure to asbestos was curtailed. Currently, around 510 new cases per 100,000 of

    the population occur per year. Without the exposure to asbestos option C would have beena reasonable alternative answer. Diagnosis is confirmed on exposure history, typical

    imaging studies and isolation of fibres. Arterial blood gas measurement in these patients

    usually demonstrates hypoxia, while pulmonary function tests demonstrate decreased vitalcapacity, decreased total lung capacity and decreased gas transfer. Death may occur due to

    cor pulmonale or asbestos exposure-related cancer. such as mesothelioma or bronchial

    carcinoma. Therapy is with oxygen by prescription and effective management of acuteinfections, but decline is usually inexorable.

    22. A 50-year-old woman patient presents with increasing dyspnoea. She isobese, smokes and takes oestrogens for menopausal symptoms. On examination

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    you find clinical, electrocardial and radiological findings of a right-sided heartfailure without signs of left ventricular failure. What is the most likely cause forthe cor pulmonale?

    Asthma

    Recurrent pneumonias

    Recurrent small pulmonary embolisms

    Your answer

    Bronchiectasis

    Carcinoma of the lung

    Pulmonary hypertension develops as the result of a critical reduction of cross-sectional area for blood flow. The relatively high flow through non-occludedvessels may cause secondary hypertensive changes in the resistive or precapillaryvessels. Patients with mean pulmonary artery pressures over 30 mmHg have a 5-year survival rate of 30%, but this rate falls to 10% if the mean pulmonary arterypressure is over 50 mmHg.

    Most patients do not have an obvious history of venous thrombosis or pulmonary

    embolism. Dyspnoea is present in virtually all, but in the early stages may occuronly on exertion. Compensatory right ventricular hypertrophy develops, andthere may be a period of months to years when symptoms remain stable, butultimately right ventricular function deteriorates. Symptoms then includeworsening dyspnoea, fatigue, presyncope, syncope (rarely), pleuritic pain,angina-like pain, abdominal swelling and peripheral oedema. Signs of respiratoryand right ventricular failure develop, with cyanosis, grossly elevated jugularvenous pulse, palpable right ventricular heave, right ventricular gallop, loud P2,hepatomegaly, ascites and peripheral oedema. Bruits may be heard over thepulmonary arteries.

    Ventilationperfusion lung scans typically show one or more mismatchedsegmental or larger perfusion defects, with most patients having several bilateralmismatched perfusion defects. Pulmonary angiography is the most definitivediagnostic test. It shows narrowed segmental pulmonary arteries, sometimesaccompanied by post-stenotic dilatation, irregularity of the intima, luminalnarrowing of the central arteries and oddly shaped vessels. Pulmonary fibre-opticangioscopy is useful to define surgical accessibility.

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    23. A 61-year-old man with a 40 pack-year smoking history presents with chronic cough,

    haemoptysis and weight loss. Unfortunately his chest X-ray reveals a large mass at the lefthilum, suggestive of a bronchial carcinoma. A raised calcium is noted on routine blood

    work-up. What type of carcinoma would best fit this clinical picture?

    Adenocarcinoma of the bronchus Correct answer

    Squamous cell-carcinoma of the bronchus Your answer

    Small-cell bronchial carcinoma

    Large-cell bronchial carcinoma

    Bronchioalveolar-cell carcinoma

    Squamous-cell and small-cell bronchial carcinomas tend to occupy a central location.

    Squanmous-cell carcinomas are more frequently associated with hypercalcaemia, so thecorrect answer in this case is option B.

    The other options tend to occur more frequently in the periphery. In terms of frequency,adenocarcinomas are the commonest, making up around 35% of the total, squamous-cell

    carcinomas make up around 20-30%, small-cell carcinomas around 20%, large cell

    carcinomas 15-20% and bronchioalveolar-cell carcinomas around 5% of the total.Bronchioalveolar-cell carcinoma has no correlation with cigarette smoking.

    24. A 26-year-old man presents with fever, headache and a non-productivecough. The chest X-ray shows increased interstitial markings. The laboratoryexamination shows an elevated LDH and anaemia with the presence of coldagglutinins. What is the most appropriate treatment?

    Erythromycin

    Your answer

    Piperacillin

    Clindamycin

    Ampicillin

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    Imipenem

    This patient presents with mycoplasma pneumonia. With regard to treatment,the pathogen lacks a cell wall and hence is not susceptible to penicillin,cephalosporins or other cell-wall active antibiotics. Macrolide antibiotics (egerythromycin or clarithromycin) are the treatment of choice for mycoplasma andother atypical pneumonias. The most frequent side-effects are nausea, vomitingand diarrhoea. It should be kept in mind that treatment is likely to be mosteffective when given over a long rather than short time, suboptimal doses areavoided and compliance is strict.

    Acute cold autoimmune haemolytic anaemia is commonly seen in adolescents andyoung adults following infection with mycoplasma. Haemolysis occurs

    approximately 12 weeks following infection and is most commonly associatedwith a rise in polyclonal anti-IgM antibodies with mycoplasma pneumonia. Thetypical patient is usually a young adult who experiences a respiratory tractinfection accompanied by headache, myalgia, cough and fever, and with a chestX-ray that shows bronchopneumonia. The cough is often non-productive, butwhen sputum is obtained it is mucoid, shows predominantly mononuclear cellsand no dominant organism. A characteristic feature is the relatively highfrequency of extrapulmonary complications such as rash, neurological syndromes(aseptic meningitis, encephalitis, neuropathies), myocarditis, pericarditis andhaemolytic anaemia. The diagnosis should be suspected in those patients with arelatively mild form of pneumonia, particularly in previously healthy young

    adults.

    25. A patient with small-cell lung cancer has a serum sodium concentration of 121 mmol/l.

    Which of the following is the most likely cause?

    Sodium-restricted diet

    Sodium-reduced water drinking

    SIADH Your answer

    Liver metastases

    Bone metastases

    The continued secretion of vasopressin (antidiuretic hormone, ADH) in an amount inexcess of the bodys needs, leads to overhydration in both the intracellular and extracellular

    compartments the so-called syndrome of inappropriate antidiuretic hormone secretion

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    (SIADH). The cerebral oedema resulting from water intoxication causes drowsiness,

    lethargy, irritability, mental confusion and disorientation, with seizures and coma being the

    most profound features. Peripheral oedema is remarkably rare. The patient is usuallyasymptomatic until the sodium concentration falls below 120 mmol/l and the

    hyponatraemia is dilutional in type with a low serum osmolality. Urine osmolality usually

    exceeds 300 mOsmol/kg. The commonest cancer causing this syndrome is small-cellcancer, where it is clinically obvious in 10% of cases, with subclinical involvement

    detectable by a water-loading test in more than 50%. Restriction of fluid to a daily intake of

    7001000 ml may redress the hyponatraemia, but demethylchlortetracycline(demeclocycline) 6001200 mg daily is often highly effective, making water restriction

    unnecessary.Infusion of hypertonic saline is hazardous, often precipitating cardiac failure

    or cerebral oedema.

    26. A 29-year-old office secretary has been suffering from intermittent pain and tenderness

    affecting her elbows, wrist and ankles for last 2 years. Symptomatic relief had beingobtained from NSAIDs. For last 3 months she has been increasingly unwell, and with night

    sweats, fever and a weight loss of about 6.4 kg (14 lbs). She also developed a non-

    productive cough and left-sided pleuritic chest pain. She smoked 30 cigarettes per day. Onexamination she had temperature of 37.8 C. The syno vium was palpable over her wrist

    joints. On respiratory examination her left lower zone was dull to percussion with

    decreased breath sounds. A chest X-ray confirmed a left-sided pleural effusion in addition

    to some fibrotic patches on both the upper zones. Aspiration showed a straw-coloured fluidwith a protein concentration of 46 g/l and a glucose concentration of 1.6 mmol/l. The fluid

    contained many lymphocytes but no malignant cells. A culture was sterile on the fifth day.

    What is the probable cause of the pleural effusion?

    Sarcoidosis

    Rheumatoid pleural effusion

    Tuberculosis Your answer

    Pleural effusion secondary to lung malignancy

    Lymphoma

    Two main points for the diagnosis are the unilateral pleural effusion and upper zone

    shadowing. Rheumatoid effusions are unusual in the young and are small andasymptomatic. This woman may have rheumatoid disease but this is not the cause of her

    effusion. It is now well recognised that tuberculosis may be associated with aninflammatory polyarthritis which may follow a similar pattern to rheumatoid arthritisseen

    here. Lymphocyte predominance suggests lymphoma, carcinoma or tuberculosis. A low

    glucose concentration favours an infection, malignancy or a rheumatoid condition. But thefibrotic shadow of the upper zone clinches the diagnosis in this case and is typical of post-

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    primary tuberculosis. Sarcoidosis shows upper zone fibrosis with pleural effusion at end-

    stage only, and it is rare. No other feature favours the diagnosis .

    27. A 24-year-old medical student (height 165 cm, weight 78 kg) has beencomplaining of a few months history of shortness of breath on exertion and ofcoughing up blood once. She is a few days away from her final examination andsmokes 20 cigarettes per day. She takes no medication except for the oralcontraceptive pill.

    What is the most likely diagnosis?

    Hyperventilation syndrome due to stress

    Tuberculosis

    Recurrent pulmonary embolism

    Your answer

    Sarcoidosis

    Goodpastures syndrome

    Pulmonary embolism can present in diverse ways. The syndrome of pleuritic painor haemoptysis, in the absence of circulatory collapse, is the most frequentmode of presentation of acute pulmonary embolism. It occurred in 60% ofpatients recruited in a collaborative investigation, the Prospective Investigationof Pulmonary Embolism Diagnosis (PIOPED). A syndrome of dyspnoea in theabsence of haemoptysis or pleuritic pain or circulatory collapse occurred in 25%.Circulatory collapse (systolic blood pressure less than 80 mmHg or loss ofconsciousness) was an uncommon mode of presentation, occurring in 15%.Obesity and a high oestrogen content in oral contraceptives have been linked tothromboembolic events. Most patients with pulmonary embolism had smoked atone time or continued to smoke at the time of their pulmonary embolism.

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    28. A 23-year-old army cadet is admitted to hospital with cough, headaches andmalaise. He has a temperature of 38 C. His blood count, renal and liverfunctions are normal. Cold agglutinins are positive. A chest X-ray shows bi-basalshadowing. What is the most likely diagnosis?

    Legionella pneumonia

    Viral pneumonia

    Q fever

    Klebsiella pneumonia

    Mycoplasma pneumonia

    Your answer

    Mycoplasma pneumonia is a common cause of pneumonia often occurring inyoung people. It is characterised by headaches, malaise and cough. Chest X-rayfindings may not correlate with the patients condition. The white blood countmay be normal and cold agglutinins occur in half of the patients. Extrapulmonarycomplications are pericarditis, myocarditis, erythema multiforme, vomiting,diarrhoea and meningoencephalitis.

    29. You have a Caucasian patient with cystic fibrosis on the ward. His 20-year-oldsister does not have cystic fibrosis, but comes to ask you about the chances ofher having children with cystic fibrosis. They have the same parents. Which of

    the following statements is correct?

    She has a 1 in 4 chance of being a carrier of the cystic fibrosisgene

    She is not a carrier of the cystic fibrosis gene

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    If she does not have the F508 deletion she is not a cysticfibrosis carrier

    Genetic testing is not necessary to assign carrier status

    Her Caucasian partner has a 1 in 25 chance of being a cysticfibrosis carrier

    Youranswer

    The carrier frequency for cystic fibrosis (CF) in Caucasians is 1 in 25. The CF geneis inherited autosomal recessively. Therefore a sibling of an affected person has

    a 1 in 2 chance of being a CF carrier. In the case of this patient's sister, sheknows she doesn't have CF, so her chance of being a carrier for the CF gene is2/3. CF accounts for 1 in 2500 live births. CF is much rarer in patients of AfroCaribbean and Asian origin. There are more than 1000 different mutationsrecognised in the gene for the cystic fibrosis transmembrane conductance

    regulator. The most common is F508, which accounts for around 67% of CFalleles in the UK and northern Europe: 85% of CF alleles can be screened forgenetically. Genetic testing would be necessary to assign carrier status, althoughthe diagnosis of cystic fibrosis could be made clinically with sweat testing onsomeone who is homozygous.

    30. A 58-year-old man with a heavy previous asbestos-exposure history smokes 20

    cigarettes per day and has done since he was 17 years old.

    What is his increased risk of developing bronchial carcinoma compared to a lifelong non-smoker, never exposed to asbestos?

    100 times

    50 times Your answer

    20 times

    10 times

    2 times

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    Asbestos exposure increases the risk of developing bronchial carcinoma 5 times, whereas

    smoking increases the risk approximately 1011 times. However, if a patient has both risk

    factors then the risk is multiplicative and is approximately 50 times greater than a lifelongnon-smoker never exposed to asbestos.

    31. A 42-year-old has had Raynauds phenomenon for many years, but now presents to herGP with non-specific symptoms of shortness of breath and a dry non-productive cough, as

    well as heartburn and a flitting arthralgia affecting a number of small joints. On

    examination the GP notices that her face appears a little waxy and expressionless and thather hands are cold with nodular thickening of the skin over the fingers. There is

    telangiectasia. The most striking abnormality on respiratory examination are fine end-

    expiratory crackles on chest auscultation. Chest X-ray reveals a fibrotic lung picture.

    Antinuclear antibodies are positive. What diagnosis fits best with this clinical picture?

    Amyloidosis

    Cryptogenic fibrosing alveolitis

    Systemic lupus erythematosus

    Progressive systemic sclerosis Your answer

    Primary pulmonary hypertension

    The picture of Raynauds disease, peripheral calcinosis, oesophageal symptoms,

    sclerodactyly, telangiectasia and evidence of pulmonary fibrosis is highly suggestive of

    progressive systemic sclerosis, indeed of the CREST syndrome. Positive antinuclearantibodies support the diagnosis. Some 30% of patients are also rheumatoid factor-positive.

    At present, there are no proven disease-modifying agents and patients suffer fromprogressive symptoms including worsening pulmonary hypertension: however,

    penicillamine does show at least some promise in this area. Calcium antagonists may proveuseful in the treatment of Raynauds, and there are a number of therapies that can be

    employed for symptomatic reflux disease.

    32. A 72-year-old man with ischaemic heart disease is on a variety of medicationfollowing a myocardial infarction 12 months ago, including aspirin, atenolol,

    lisinopril, amiodarone and furosemide (frusemide). He is becoming progressivelybreathless and his cardiologist sends him for pulmonary function tests, whichshow a restrictive ventilatory defect with decreased gas transfer.

    Which one of his medications is most likely to be the cause of theseabnormalities?

    Aspirin

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    Atenolol

    Lisinopril

    Amiodarone

    Your answer

    Furosemide

    Aspirin is associated with nasal polyps and asthma, atenolol withbronchoconstriction, lisinopril with cough and furosemide very rarely with

    bronchoconstriction. Amiodarone is associated with pulmonary fibrosis.

    33. A 35-year-old woman visits her GP complaining of a dry non-productive cough andlethargy. There has been slow weight loss over the past few months and there is a nodular

    rash on her shins. Blood testing reveals mild hypercalcaemia, a non-specific increase in

    immunoglobulins, normochromic normocytic anaemia and a raised serum ACE level. Achest X-ray reveals bilateral hilar lymphadenopathy. Tuberculin skin testing is negative.

    What diagnosis fits best with this clinical picture?

    Sarcoidosis Your answer

    Tuberculosis

    Lymphoma

    Lymphangitis carcinomatosa

    Cryptogenic fibrosing alveolitis

    Sarcoid is most common in young women and the UK prevalence is approximately 19 per

    100,000 of the population. It is a multisystem granulomatous disorder: 50% of sufferers

    present with respiratory symptoms, or abnormalities on chest X-ray; 10% of cases areassociated with erythema nodosum; 25% with uveitis; and 10% of established cases are

    associated with hypercalcaemia. A definitive diagnosis is usually obtained viatransbronchial biopsy in over 90% of cases. The disease severity varies amongst racial

    groups, and is more severe in Black populations. Treatment with corticosteroids is usual for

    severe disease or persistent changes on chest X-ray, but mortality in the UK fromsarcoidosis is probably less than 5%.

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    34. A 65-year-old man complains of lethargy, fever, dry cough, headache, chest pain andincreasing shortness of breath. He returned from a cruise 2 days ago. His chest X-ray

    shows bilateral infiltrates, the p(O2) is 8.35 kPa. What is the most likely diagnosis?

    Tuberculosis

    Pulmonary embolism

    Small-cell carcinoma of the lung

    Sarcoidosis

    Legionella pneumonia Your answer

    Legionella infection is the cause of around 25% of cases of community-acquired

    pneumonia admitted to hospital, although there is wider geographical and seasonalvariation. Infection tends to lead to moderate or severe infection rather than mild illness,

    and most patients require hospital admission within 57 days of the start of symptoms. Theincubation period is usually 210 days, with a mean of 7 days; males are two to three timesmore frequently affected than females. Infection at the extremes of age is rare and the

    highest incidence is in 40- to 70-year-old people, with a mean age of 53 years. People

    particularly at risk include cigarette smokers, alcoholics, diabetics and those with a chronicillness or who are receiving corticosteroids or immunosuppressive therapy. Consequently,

    the type of patient who requires admission to hospital is particularly at risk from a

    nosocomial source. Typically, the illness starts fairly abruptly with high fever, shivers, bad

    headache and muscle pains. Upper respiratory tract symptoms, herpes labialis and skinrashes are uncommon. The cough is usually dry initially, but dyspnoea is common and the

    illness often progresses quickly. Sometimes there may be a history of a recent hotel holiday

    abroad or a stay in hospital, which can alert the clinician to the possible diagnosis. Thepatient commonly looks toxic and ill, with a high fever over 39 C. Confusion and delirium

    or diarrhoea can dominate the clinical picture, masking the true diagnosis of pneumonia.

    Focal neurological signs, particularly of a cerebellar type, are well described. Amnesia onrecovery is common.

    35. A patient presents with shortness of breath. His transfer coefficient (KCO) is 160% of

    predicted. What is the most likely cause?

    Fibrosing alveolitis

    Asthma

    Pulmonary haemorrhage Your answer

    Pulmonary embolus

    Pneumonia

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    The transfer coefficient (Kco), which is obtained along with the TLco, represents the

    uptake of carbon monoxide per litre of effective alveolar volume (VA), that is, Kco = TLco

    /VA. To a large extent, Kco allows correction for any real or effective reduction of alveolarvolume, tending to be normal after lung resection, when both TLco and VA are reduced

    approximately to the same degree.In some conditionsKco may increase. The latter usually

    results from an increase in red blood cells in the lungs due to greater blood flow,haemorrhage, or polycythaemia.Kco is similarly increased in these conditions, as it is if, at

    full inflation, the density of pulmonary capillaries per unit alveolar volume is greater than

    normal. This occurs most commonly in patients with extrapulmonary volume restriction,when the density of pulmonary capillaries is unusually high in relation to the (restricted)

    lung volume at which the measurement is made.

    36. A 65-year-old woman has been diagnosed as having lung cancer. Which of the

    following statements is most appropriate?

    Hypertrophic pulmonary osteoarthropathy (HPOA) is commonlyseen patients with small-cell carcinoma

    Inappropriate secretion of antidiuretic hormone (ISADH) iscommonly seen in patients with squamous-cell carcinoma

    Hypercalcaemia may occur without bone metastasis

    Your

    answer

    Paraneoplastic syndromes occur more commonly with squamous-cell carcinomas

    Hypercalcaemia associated with bone metastasis is best treatedwith intravenous steroids

    Paraneoplastic syndromes are a variety of non-metastatic metabolic orneuromuscular manifestations of lung cancer. They are commonly associatedwith small-cell carcinoma. HPOA is characterised by painful symmetricalarthropathy involving the wrist, ankle and knee joints. It is most commonly seenin patients with squamous-cell carcinoma and is virtually unknown in those withsmall-cell carcinoma.

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    Inappropriate secretion of ADH is most common in patients with small-cellcarcinoma. Hypercalcaemia may occur as a paraneoplastic syndrome withsquamous-cell carcinoma. This is due to the production of a parathyroidhormone-related peptide by the tumour, which increases bone resorption, andrenal tubular reabsorption of calcium. This is best treated with intravenous fluids

    and bisphosphonates. Steroids are mainly used for treating hypercalcaemiaassociated with sarcoidosis.

    37. A 45-year-old man who races pigeons becomes breathless. Which of the following

    features is suggestive of extrinsic allergic alveolitis?

    Almost immediate onset after exposure

    Eosinophilia of sputumPositive skinprick testing

    Type 1 hypersensitivity reaction

    Circulating IgG precipitins Your answer

    Extrinsic allergic alveolitis (EAA) is characterised by type 3 (immune-complex) and type 4(cell-mediated) hypersensitivity reactions to inhaled antigen(s). Despite its name, EAA is

    not allergic and therefore items associated with allergy tend to be false in EAA eg wheeze,

    immediate symptoms (not a type 1 reaction), raised IgE, positive skinprick test,eosinophilia of blood or sputum. In the acute form fever, cough and marked shortness of

    breath (SOB) occurs between 46 h after exposure. Subacutely there is weight loss and

    fatigue and in the chronic form exertional SOB and pulmonary fibrosis (typically upperlobe). The CXR shows fine reticular/nodular shadowing progressing eventually to a fibrotic

    pattern with shrunken lungs.

    38. A 55-year-old man presents with increasing shortness of breath. He has beenworking in the sandblasting industry and exposed to quartz particles. What is themost likely diagnosis?

    Asbestosis

    Sarcoidosis

    Silicosis Your answer

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    Eosinophilic pneumonitis

    Asthma

    Silicosis is a fibrotic disease of the lungs due to inhalation of crystalline silicondioxide, usually in the form of quartz. Silicosis may affect anyone involved inquarrying, carving, mining, tunnelling, grinding or sandblasting, if the dustgenerated contains quartz. Between 50 and 60 cases are diagnosed in the UKeach year, generally in people involved in the production of slate or granite,among miners cutting through rock and in fettlers in foundries. Crystalline silicais present in the earths crust usually as quartz, although other forms such as

    crystobalite and tridymite occur occasionally. All are extremely toxic tomacrophages. Quartz seems to be most toxic when freshly fractured, suggestingthat its surface properties are important in toxicity.

    Silicosis presents a spectrum of clinical appearances depending on thecircumstances in which it is contracted. The most severe, acute silicosis, may beacquired after very heavy exposure over just months, such as sandblastingwithout respiratory protection. These patients become intensely breathless anddie within months. The X-ray shows appearances resembling pulmonary oedema.Less heavy exposure causes progressively less dramatic symptoms, ranging from aprogressive upper lobe fibrosis with slowly increasing exertional dyspnoea over

    several years (accelerated silicosis) to radiographic nodular change similar tocoal-workers pneumoconiosis unassociated with any symptoms or physical signs.The latter type of silicosis is the most common, and is usually associated withexposure to dust containing 1030% silica over a prolonged period. Simple nodularsilicosis differs from coal-workers pneumoconiosis in that the lesions tend to belarger (35 mm) and that it is progressive even after dust exposure ceases.Lesions increase in size and become more profuse. Moreover, extensive simplesilicosis may be associated with some restriction of lung volumes. Acceleratedsilicosis and progressive massive fibrosis cause lung restriction and lead to corpulmonale and cardiorespiratory failure.

    39. A patient with lung cancer underwent radiotherapy 2 weeks ago, he nowcomplains of a severe dry cough, increasing dyspnoea and tachypnoea. Onexamination there are local crepitations, his chest X-ray shows hazyconsolidation that correspond to the irradiation field. What is the most likelydiagnosis?

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    Pulmonary embolism

    Mycoplasma pneumonia

    Radiation pneumonitis

    Your answer

    Legionella pneumonia

    Cancer progression

    Symptoms begin within a few weeks of radiotherapy and may persist for weeks ormonths. They occur in 1030% of patients following radiotherapy for lung cancer.A cough (which can be severe and may produce thick sputum) and breathlessnessare the principal symptoms, but may be accompanied by fever of variabledegree.

    On examination there may be tachypnoea, cyanosis in severe disease, and localcrepitations. Telangiectases, the result of cutaneous radiation damage, are oftenobserved in the overlying skin. The most characteristic X-ray feature is an area ofhazy consolidation demarcated by a sharp margin (crossing anatomical pulmonaryplanes) that corresponds to the limits of the irradiation field, though additionaleffects are usually detectable beyond these boundaries. Computed tomography

    provides the best means of early identification, ground-glass attenuation andinteralveolar septal thickening being the early characteristic features. Denselocal fibrosis may develop up to a year or two after radiation, and magneticresonance imaging may be required to allow differentiation from tumourrecurrence.

    40. A 60-year-old smoker is being evaluated for a coronary bypass graft. Which is the bestpreoperative screen of pulmonary function for this patient?

    Arterial pH

    Arterialp(CO2)

    Arterialp(O2)

    Forced expiratory volume in 1 second/forced vital capacity (FEV1/FVC)ratio

    Your answer

    Oxygen saturation

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    Pulmonary function tests provide the best preoperative screen, because they reflect

    dynamic measurements of pulmonary function in the patient. The arterial blood gas

    measurements reflect acidbase disorders, but they do not predict pulmonary complicationsin the postoperative state.

    41. A 40-year-old male complains of increasing shortness of breath, his chest X-ray showsan elevated hemidiaphragm on the left side. Which feature shows the sniff test?

    Decreased vital capacity in a supine position

    Decreased vital capacity while standing

    Paradoxical hemidiaphragm movement Your answer

    Decreased transfer coefficient (Kco)

    Increased transfer coefficient (Kco)

    The diagnosis of unilateral paralysis, suggested by asymmetric elevation of the affected

    hemidiaphragm on X-ray, can be confirmed by fluoroscopy. During a forced inspiratory

    manoeuvre (the sniff test), the unaffected hemidiaphragm descends forcefully, increasingintra-abdominal pressure and pushing the paralysed hemidiaphragm cephalad (paradoxical

    motion).

    42. A 40-year-old man presents with a 2-month history of cough and breathlessness. He has

    also noted haemoptysis, which he says has gradually worsened. On examination he hasbilateral basal crepitations. His chest X-ray shows diffuse shadowing. He has moderaterenal failure. What is the most likely diagnosis?

    Tuberculosis

    Goodpastures disease Your answer

    Bronchial carcinoma

    Legionella pneumonia

    Pulmonary embolism

    Goodpasture described a man with renal failure, glomerulonephritis and pulmonary

    haemorrhage. Goodpastures disease consists of diffuse pulmonary haemorrhage andglomerulonephritis with linear deposition of antibody (90% of which are directed against thea-3 chain of type-IV collagen) along the glomerular basement membrane. In practice,

    glomerulonephritis proves to be a much commoner threat to survival than lung haemorrhage,

    and the diagnosis of Goodpastures disease is reached more conveniently by serologicaltesting (for anti-GBM antibodies) and from kidney rather than lung biopsy.

    In some cases, however, lung disease dominates the clinical picture, where the majority of

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    patients are male smokers and some report a recent exposure to volatile hydrocarbons; case

    reports have additionally identified recent exposure to chlorine and smoked cocaine. This

    suggests that when there is susceptibility, inhaled toxic agents enhance pulmonaryendothelial damage and thus allows the initiation of autoimmunity or the ready access of

    existing autoantibody to basement membrane.

    Respiratory presentation is with cough, breathlessness and haemoptysis, which isintermittent and ranges from occasional streaks to massive fatal bleeding. Systemicsymptoms of fever, joint pains or weight loss are unusual. The chest X-ray shows patchy or

    diffuse shadowing due to intra-alveolar blood, usually resolving over the course of 2 weeks

    unless there is further bleeding. At the time of bleeding there may be arterial hypoxaemiaand reduced lung volumes. Serial measurement ofTLco can be used to monitor progression,

    and prolonged bleeding may lead to iron-deficiency anaemia. The carbon monoxide (CO)

    diffusing capacity or transfer factor (TLco) is widely used as a simple test of the integrity ofthe alveolar capillary membrane and of the overall gas-exchanging function of the lungs. It

    has good sensitivity but poor specificity, as impairment can result from a variety of

    pathological processes. Renal function may be normal initially but then deteriorates over

    days to weeks.

    Steroids, other immunosuppressant drugs (cyclophosphamide in particular) and

    plasmapheresis are all used (in some circumstances) to control renal disease, and are

    additionally helpful in treating pulmonary haemorrhage. Patients should not smoke and

    should avoid hydrocarbon exposure.

    43. A 38-year-old woman presents with recurrent chest infections. For some time she has

    noticed that her nails are yellow and misshapen and that she often has oedematous legsafter standing all day in the shop where she works. Examination reveals evidence of lower

    lobe consolidation and possible pleural effusion. This is confirmed on chest X-ray. Her

    nails are very abnormal, thickened and yellow and she has bilateral lymphoedema affectingher legs. Which diagnosis fits best with this clinical picture?

    Bronchiectasis

    Asthma

    Underlying bronchial carcinoma

    COPD

    Yellow-nail Syndrome Your answer

    Yellow-nail syndrome is an abnormality of lymphatic drainage associated with recurrent

    bronchiectasis, small bilateral pleural effusions, lymphoedema and grossly thickened,

    yellow nails. Her X-ray changes are chronic and associated with bronchiectasis.Management is with antibiotics to treat episodes of acute infection and regular follow-up.

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    44. A 46-year-old meat-factory worker is found to have Q fever pneumonia. Which of the

    following statements is correct?

    He requires high-dose penicillin for his treatment

    His occupation is not important for the diagnosis

    There is no long-term sequel of the diseaseThe organism responsible is Coxiella pneumoniae

    The organism is usually inhaled from infected dust Your answer

    Q fever is due to Coxiella burnetii and is acquired through contact with animals. Theorganism is very resistant to drying and is inhaled from infected dust. It is not notifiable,

    but can occur in outbreaks in farming communities and in abattoirs. A chest X-ray may

    show multi-lobar consolidation. Treatment is with prolonged courses of tetracyclines.Rarely, infection can be persistent leading to chronic symptoms including fatigue, malaise

    and sweats. In cases of chronic disease, culture-negative endocarditis should be suspected.

    Lengthy antibiotic courses can be curative.

    45. A 65-year-old man came to clinic with a history of proximal muscle weakness. He has

    had a cough for 8 weeks. There is associated pain in the small joints of the hands, and has

    small haemorrhages in the nail folds. He is apyrexic and on examination there is nolymphadenopathy or clubbing. Bibasal crackles can be heard and his chest X-ray reveals

    diffuse reticular infiltrates. Lung function tests show a restrictive pattern. What is the

    underlying cause of his interstitial lung disease?

    Cryptogenic fibrosing alveolitisSLE

    Ankylosing spondylitis

    Polymyositis Your answer

    Rheumatoid arthritis

    Polymyositis and dermatomyositis are inflammatory conditions involving the muscle andskin, respectively. Patients often complain of proximal muscle weakness. Pain in the small

    joints of the fingers is associated with the condition. The patients may present with ragged

    cuticles and haemorrhages at the finger-nail folds. Interstitial lung disease can occur in

    these patients. Underlying malignancy of the lungs, ovaries, breasts or stomach is presentin 58% of cases.

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    46. A 45 year old man, presenting with weight loss and cough, is found to have small cell

    lung cancer. Which of the following best describes the additional biocemical and clinicalfeatures that may occur in this condition?

    Cushing's syndrome, if present, is characterised by buffalo hump, striae

    and central obesity

    Hypercalcaemia is commonly seen

    Hypertrophic pulmonary osteoarthropathy is a very rare featureYour

    answer

    Acidois is commonly seen

    Syndrome of inappropriate antidiuretic hormone secretion (SIADH)occurs in >60% of cases

    Small-cell carcinoma is uncommonly associated with hypercalcaemia and the syndrome of

    inappropriate antidiuretic hormone secretion (SIADH). Due to the short natural history,Cushings syndrome in small-cell carcinoma does not manifest classically by buffalohump, striae or central obesity. Its presence is suspected by arterial hypertension,

    hyperglycaemia, hypokalaemia, alkalosis and muscle weakness. Chemotherapy is the

    treatment of choice. Hypertrophic pulmonary osteoarthropathy is very rare in small-cellcarcinoma of the lung.

    47. A 67-year-old patient with lung cancer complains of difficulty breathing, coughing and

    swelling of his face, neck, upper body and arms. Superior vena cava syndrome isdiagnosed. Which of the following treatments is most likely to be successful in giving early

    relief of symptoms?

    Corticosteroids

    Radiotherapy Your answer

    Surgery

    Chemotherapy

    Antihypertensive drugs

    Superior vena cava syndrome (SVCS) is a collection of symptoms caused by the partialblockage of the vein that carries blood from the head, neck, chest and arms to the heart.

    Symptoms that may indicate this syndrome include difficulty breathing, coughing and

    swelling of the face, neck, upper body and arms. In rare instances, patients may complain

    of hoarseness, chest pain, difficulty swallowing and coughing up blood. Physical signs ofSVCS include swelling of the neck or chest veins, collection of fluid in the face or arms

    and rapid breathing. The syndrome of superior vena cava obstruction is relieved in about

    80% of sufferers, but usually requires a more conventional course of 510 fractions of

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    radiotherapy. Pain from bone secondaries can be relieved in more than 50% of sufferers by

    a single fraction of 8 Gy, often given at the same time as a clinic visit. Brain metastases

    generally respond poorly to radiotherapy. A 48-hour trial of dexamethasone, 4 mg orallyfour times daily, is recommended as initial management in the case of brain metastases.

    48. A 72-year-old woman who smokes 510 cigarettes per day and has a past history of

    whooping cough presents with chronic cough and recurrent chest infections for review. Sheadmits to producing frequent amounts of purulent sputum and of intermittently suffering

    night sweats for a number of months. There have also been occasional episodes of

    haemoptysis. Chest X-ray reveals hyperinflation, crowded lung markings and small cyst-like spaces at the lung bases. What is the most likely underlying pathology?

    Tuberculosis

    Asthma

    Bronchiectasis Your answerChronic lung abscess

    Bronchial carcinoma

    The past history of whooping cough and smoking, coupled with the X-ray changes are very

    suggestive of bronchiectasis. Non-pharmacological management involves self-

    physiotherapy and adequate hydration. Chronic-intermittent, oral antibiotic therapy is usedby some physicians, although it may encourage multi-drug resistance and its use is not

    endorsed by all. Influenza and pneumococcal vaccinations are strongly recommended.

    Surgical referral may be recommended for patients with chronic severe localised infectionthat fails to resolve after iv antibiotic therapy.

    49. A 46-year-old woman is admitted to hospital with a left basal, community-acquired pneumonia. She is on the appropriate antibiotics. She is still pyrexialfour days after admission and a chest X-ray confirms a left pleural effusion. Thehouse officer has performed a diagnostic tap. Which of the following is anindication to insert a chest drain?

    Pleural fluid protein level more than 50% of serum protein level

    Pleural fluid LDH more than 60% of serum LDH

    Haemorrhagic pleural fluid

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    Pleural fluid pH < 7.2

    Your answer

    Pleural fluid glucose < 1.6 mmol/l

    This woman has a parapneumonic effusion. The indications for chest tubeinsertion in patients with infected pleural effusions are: presence of organismson a Gram stain of the pleural fluid, a frankly purulent pleural fluid, pleural pH


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