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Respiratory

The following are recognised causes of recurrent cough in the pre-school child: A Parainfluenzae infection B Exercise C Retropharyngeal abscess D Congenital ciliary dysmotility E Postnasal drip Comments: Cough indicates irritation to nerve receptors in the pharynx, larynx, trachea or bronchi. Recurrent causes include: (False) (False) (False) (True) (True)

Asthma, sometimes exacerbated by allergic rhinitis and postnasal drip. Infections: pertussis, RSV, mycoplasma. Bronchiectasis: cystic fibrosis, Kartagener Syndrome. Recurrent aspiration, especially in children with abnormal swallowing. Habit cough or smoking. Other: inhaled foreign body, TB.

Copyright 2002 Dr Colin Melville

Causes of lung cysts on chest x-ray include: A Staphylococcal pneumonia B McLeod's Syndrome C Tuberculosis D Aspergillus infection E Congenital lobar emphysema Comments: Causes can be divided into: (True) (True) (True) (False) (True)

Congenital: Lobar emphysema, cystic adenomatoid malformation, bronchogenic cyst, sequestration (intralobar, extralobar). Acquired: Bronchiectasis, associated with any long-standing infection such as cystic fibrosis, post-measles, post-pertussis, TB.

Staphylococcal and Klebsiella pneumonia are characteristically associated with cavitation. Aspergillus usually, is happy to colonise cavities that are already present. Invasive pulmonary Aspergillosis may cause nodular infiltrates on chest x-ray. Copyright 2002 Dr Colin Melville

An 18 month old boy presents with recurrent cough. The following make a diagnosis of recurrent aspiration more likely: A Cerebral palsy B History of coughing with feeds C Presence of dextracardia D Presence of failure to thrive E Coughing spasms ending in vomiting Comments: Gastro-oesophageal reflux is usually mild, and resolves during the first year of life as the child is weaned on to more solid foods and adopts an upright posture. In children with brain abnormalities, the control to the upper GI tract is abnormal, and symptoms can persist. Dextracardia may be associated with Kartagener Syndrome (ciliary dysmotility), while failure to thrive suggests cystic fibrosis. Coughing spasms ending in vomiting suggest pertussis. Copyright 2002 Dr Colin Melville (True) (True) (False) (False) (False)

Acute laryngotracheal bronchitis (croup): A Is usually caused by RSV infection. B Is usually preceded by 2-3 days of coryza. C Responds best to risinic adrenaline. D If agitated, the patient should receive mild sedation. E May require intravenous antibiotics. Comments: Acute laryngotracheal bronchitis is usually caused by viruses, particularly parainfluenza viruses. They are spread by droplets or secretions, and replicate specifically in the respiratory epithelium, without evidence of systemic spread. Upper respiratory symptoms have usually been present for a few days before a cough becomes apparent. This is brassy with intermittent inspiratory stridor. As the child becomes upset, nasal flaring and recession and cough worsen. The temperature is rarely high. In children with a high fever and severe respiratory compromise, the possibility of bacterial tracheitis should be considered. Although aerosolised racenic adrenaline may temporarily improve aeration, oral or inhaled steroids have a longer duration effect for less severe cases, usually allowing the patient to be discharged home. Sedation is never indicated in a child with respiratory compromise unless the airway is to be protected mechanically. Copyright 2002 Dr Colin Melville (False) (True) (False) (False) (True)

In a 6 month old child with recurrent chest infections, the following factors may suggest an underlying diagnosis:

A Two year old sibling at nursery. B Persistent lymphadenopathy >0.5cm. C A history of cradle cap. D Full blood count showing a haemoglobin of 9.5, white cell count of 4.9x109/L (N 65%, L - 21%). E Maternal smoking Comments:

(True) (True) (True) (True) (True)

Recurrent chest symptoms can be caused by infection or atopy, and in the atopic child, symptoms are often precipitated by viruses anyway. The most common source of organisms is from an older sibling, and parental smoking may contribute to recurrent chest symptoms. Bronchiolitis is commoner in babies whose mothers smoke, particularly if this has occurred during pregnancy. Children with atopy often have cradle cap in the neonatal period. Persistent gross lymphadenopathy may be found in HIV infection, and is also a feature of Omenn's Syndrome. Lymphopenia associated with recurrent chestiness, particularly if there is failure to thrive should suggest severe combined immune deficiency. Copyright 2002 Dr Colin Melville

Characteristic features of obstructive sleep apnoea/hypoventilation include: A Apnoea occurring during REM sleep. B Symptoms developing after tonsillectomy. C Desaturations detected by pulse oximeter. D Chronic hypercapnia. E Association with Down's Syndrome. Comments: The incidence is 1:1000 or 1% of the children who habitually snore. The peak incidence is 2-5 years, coinciding with normal lymphoid hyperplasia and frequent upper respiratory infections. Prepubertal sex incidence is equal, but there is a male predominance in adults. There is failure to maintain upper airway patency during sleep, which affects blood gas homeostasis. During sleep PAO2 decreases and PACO2 increases only slightly in normal children. When adenotonsillar hypertrophy, nasal obstruction, obesity, or craniofacial abnormalities are present, or neurological conditions (hypotonia, cranial nerve weakness) occur there may be partial or complete airway obstruction with hypoxaemia and hypercapnia. This may result in increased respiratory effort and airway muscle tone may lead to the resumption of airway patency, which also occurs during arousal from sleep. The cycle can recur. Chronic hypoxaemia can lead to polycythemia, growth failure, pulmonary hypertension and cor pulmonale, arrhythmias or death. Daytime hypersomnulance is associated with behavioural problems and impaired school performance. Diagnosis may be delayed since the symptoms are most severe during REM sleep in the last third of the night when the parents are asleep, and children do not generate the loud disruptive snoring noises of an adult. Copyright 2002 Dr Colin Melville (True) (False) (True) (True) (True)

Recognised complications of pertussis include:

A Encephalopathy B Hypoxic fits C Frenular tears D Retinal haemorrhage E Bronchiectasis Comments:

(True) (True) (True) (True) (True)

Complications of pertussis occur particularly in children under the age of 6 months. Hospitalisation under the age of 2 months exceeds 80%, with pneumonia occurring in 25%, seizures in 4%, encephalopathy in 1% and death in 1%. Other complications include:

Apnoea. Secondary infections (otitis media, pneumonia). Adult respiratory distress syndrome. Bronchiectasis. Haemorrhages: pulmonary, sub-conjunctival, scleral, epistaxis, CNS, pneumothorax, subcutaneous emphysema, umbilical and inguinal herniae, frenular tears, rectal prolapse. Dehydration and malnutrition, tetany has been associated with profound post-pertussis alkalosis. Apnoea, hypoxia, CNS bleeds.

Copyright 2002 Dr Colin Melville

Regarding central cyanosis: A It can be reliably diagnosed when >5g/l of desaturated haemoglobin are present. B If the haemoglobin is 6g/dl, the child will be pre-terminal before cyanosis appears. C Central cyanosis is indicated by circum-oral blueness. D It can be associated with a normal arterial PO2 in methaemoglobinaemia. E May be caused by right to left shunts. Comments: Cyanosis is a bluish or purplish tinge of the skin or mucous membranes resulting from the presence of >5g/dl of reduced haemoglobin in the blood perfusing the skin. It is, therefore, not detectable in patients with severe anaemia, even if they are gravely hypoxaemic. Central cyanosis is usually detectable once the oxygen saturation drops below about 80-85%. It may be respiratory or cardiac in origin. Occasionally, cyanosis can be because of abnormal pigments such as sulphaemoglobin or methaemoglobin. In this cases, arterial oxygen tension is normal. Methaemoglobin: Fe types:
3+

(True) (True) (False) (True) (True)

instead of normal Fe

2+

in haemoglobin in some haemoglobin molecules. Two

Genetic (asymptomatic) Acquired: ingestion of oxidant drugs such as analine dyes, GTN, dapsone. These give symptoms of

anaemia haemolysis. Sulphaemoglobin: Acquired by ingestion of sulphonamides or phenaciten. Copyright 2002 Dr Colin Melville

Wheeze in infancy: A Is strongly associated with bronchial hyper-reactivity. B Is indicative of chronic respiratory symptoms needing inhaled corticosteroids therapy. C As responsive to bronchodilator therapy as with older children. D Is not correlated with family smoking habits. E Has a good prognosis. Comments: Wheeze is a musical added sound that is caused by air flow through narrowed airways, which act like organ pipes. It is very common in infancy, particularly in relation to viral infections and to smoking, particularly of the mother. Its relationship to asthma has not been totally clarified, but it responds disappointingly to bronchodilator and corticosteroid therapy. Copyright 2002 Dr Colin Melville (True) (False) (False) (False) (True)

The following are characteristic presentations of cystic fibrosis: A A 26 week gestation infant with x-ray appearance of meconium ileus. B An 8 month old girl admitted the second time with lower respiratory tract changes on chest x- ray. C A 9 month old Indian girl with failure to thrive. D A 3 week old term female with prolonged unconjugated jaundice. E A 4 year old boy with mouth breathing due to nasal polyps. Comments: Typical clinical features at presentation are GI or respiratory. (True) (True) (False) (False) (True)

RESPIRATORY: Recurrent chest infections, bronchiectasis, pneumothorax, sinusitis or nasal polyps, haemoptysis, aspergillosis. GASTRO-INTESTINAL: Meconium ileus (neonate), steatorrhoea, malabsorption, failure to thrive or growth failure, rectal prolapse, prolonged conjugated jaundice.

Copyright 2002 Dr Colin Melville

Bronchiolitis:

A Is rare outside infancy. B Is usually caused by parainfluenza viruses. C May be caused by influenza viruses A or B. D Commonly presents with cough and wheeze. E Paroxysms of coughing can be mistaken for pertussis. Comments:

(True) (False) (True) (True) (True)

Acute bronchiolitis is a common lower respiratory tract disease of infants, occurring during the first 2 years of life with a peak incidence at 3-6 months of age and wintertime epidemics. In the UK, RSV accounts for about 70% of cases, with occasional parainfluenza, mycoplasma, adenovirus cases. Risk factors include overcrowding, prematurity, maternal smoking. The disease begins with upper respiratory symptoms and a moderate fever. Feeding diminishes, and there is progressive respiratory distress with wheezing and cough. Symptoms usually worsen over a few days before gradually improving. In severe cases, the respiratory rate may exceed 80 breaths per minute with marked increased work of breathing and cyanosis. There may be a palpable liver from hyperinflated lungs pushing down the diaphragm. Widespread fine crackles and wheezes are often heard. X-ray shows hyperinflation some areas of consolidation. Risk factors for severe disease include congenital heart disease, bronchopulmonary dysplasia, immune deficiency, gastro-oesophageal reflux. Treatment is supportive. Nasogastric or intravenous fluids may be required with humidified oxygen. The use of ribarvirin is controversial, steroids are not beneficial, and antibiotics have no therapeutic value unless there is secondary bacterial pneumonia. The low incidence of bacterial complications is not reduced further by antibiotic therapy. Copyright 2002 Dr Colin Melville

Regarding total lung capacity: A It is a specific measure of lung size. B It depends on the thickness of the alveolar wall. C It is reduced in severe cerebral palsy. D It is increased in infants with cystic fibrosis. E It can be measured by the helium dilution technique. Comments: Total lung capacity equals the vital capacity plus the residual volume. It is, therefore decreased in most lung abnormalities. In cystic fibrosis there are areas of overinflation, but also areas of atelectasis, so the overall lung volume is reduced. In the helium dilution technique, the change in concentration of a known volume of helium is used to estimate the total lung capacity following equilibration. Copyright 2002 Dr Colin Melville (True) (False) (True) (False) (True)

The following are recognised associations with pulmonary hypertension:

A An apgar of 3 at 5 minutes B Meconium aspiration C Hyaline membrane disease D Hypoglycaemia E Oligohydraminos Comments:

(True) (True) (True) (True) (True)

Persistent fetal circulation occurs with all of these. It is usually treated using nitric oxide, a specific pulmonary vasodilator. Copyright 2002 Dr Colin Melville

The following are recognised causes of pulmonary eosinophilia: A Asthma B Loeffler's Syndrome C Hookworm infestation D Aspergillus fumigatus E Schistosomiasis Comments: Loeffler's Syndrome is characterised by widespread transitory pulmonary infiltrations which may resemble miliary TB, and by a blood eosinophil level as high as 70%. There is usually paroxysmal cough, breathlessness, pleurisy, and little or no fever. There may be hepatosplenomegaly especially in infants, and local pneumonic consolidation may occur. Possible underlying causes include: (True) (True) (True) (True) (False)

Drugs (antibiotics, crack cocaine). Helminthic infections: toxocara, ascaris, and strongyloides.

The differential diagnosis includes:

Vasculidities (eosinophilic pneumonia plus polyarteritis). Asthma, including allergic bronchopulmonary aspergillosis. Filaraisis. Chronic eosinophilic pneumonia.

Copyright 2002 Dr Colin Melville

The following are recognised treatments for complications of cystic fibrosis: A DNAase to assist in reinflating collapsed lung segments. B Rectal pull-through and anastamosis for rectal prolapse. C Pancreatic transplant for diabetes mellitus. (False) (False) (False)

D Nebulised tobramycin for pseudomonas colonisation of the lower respiratory tract. E Hypotonic saline drinks for hypernatraemic dehydration. Comments:

(True) (False)

Human recombinant DNAase given as a single daily aerosol seems to improve pulmonary function, decrease the frequency of chest exacerbations, and promotes a sense of well-being in patients with mild to moderate disease with purulent secretions. This may be because, in the inflamed airway, the nuclei from dead cells accounts for much of the viscidity of secretions. Rectal prolapse is usually idiopathic, occurring between 1 and 5 years. Intestinal parasites, malnutrition, acute diarrhoea, ulcerative colitis, pertussis, Ehler's Danlos Syndrome, meningocele, cystic fibrosis, and chronic constipation can also predispose to it. Following defecation the prolapse usually resolves spontaneously, or through manual reinsertion by the patient or parent. Nebulised tobramycin or gentamicin may be given when airway pathogens are resistant to oral antibiotics, or where infection is difficult to control at home. Hypernatramic dehydration should be treated in the usual way. Copyright 2002 Dr Colin Melville

Regarding the sweat test: A Sweating is enhanced by application of atropine. B The filter paper is left on for a total of about 4 hours. C At least 25mg of sweat is necessary for a reliable result. D More than 60mmol/L of chloride in sweat is diagnostic of cystic fibrosis. E False/positive results may be encountered in children with nephrotic syndrome. Comments: The sweat test is conducted using pilocarpine iontophoresis. A 3mA current carries pilocarpine into the skin of the forearm stimulating local sweating. The arm is washed with distilled water and sweat collected on a filter paper or gauze. The duration of collection is usually 30-60 minutes. The filter paper is removed, weighed and eluted in distilled water. At least 50mg and preferably 100mg of sweat should be collected for reliable results. It may not be possible to collect this amount in young infants. More than 60mmol/L of chloride is diagnostic of CF when one or more other criteria are present. In healthy adults, the sweat chloride values increase slightly, but 60mmol/L still differentiates CF from other conditions. False/negative results may be encountered in nephrotic syndromes. Copyright 2002 Dr Colin Melville (False) (False) (False) (True) (False)

Diffusion capacity of carbon monoxide: A Is a specific measure of lung perfusion. B Depends on the thickness of the alveolar wall. C Depends on the surface area available for gas exchange. D Is increased in cigarette smokers. E Is increased in emphysema. (False) (True) (True) (False) (False)

Comments: By Fick's law, the volume of gas diffusing across a membrane equals A/T x D x difference in partial pressure. In life it is impossible to measure accurately the area (A) or the thickness (T), and these are subsumed into a single constant, the diffusion capacity for carbon monoxide. DL=volume of transferred carbon dioxide divided by partial pressure difference between the alveoli and the capillary blood. Since the capillary blood normally does not contain carbon dioxide this term disappears. Diffusion will be increased in healthy compared with unhealthy lungs, where the thickness is likely to increase and the surface area available for gas exchange to decrease. VQ imbalances can indirectly interfere with carbon dioxide diffusion capacity by decreasing the available area of lung for gas exchange, but it is not a specific measure of lung perfusion. Copyright 2002 Dr Colin Melville

The following respiratory symptoms may be exacerbated by gastro-oesophageal reflux: A Asthma B Central apnoea C Obstructive apnoea D Stridor E Wheeze Comments: Gastro-oesophageal reflux may cause or worsen a large number of respiratory disorders including: (True) (True) (True) (True) (True)

Aspiration pneumonia. Asthma. BPD. CF. Trachea-oesophageal fistula. Obstructive and central apnoea. Stridor. Hoarseness. Cough. Hiccups.

Copyright 2002 Dr Colin Melville

In lung perfusion scanning: A Emphysema and pulmonary embolism give similar appearances. B Iodine sensitivity is a contraindication. C Is always abnormal in Scimitar Syndrome. D May show decreased upper lobe perfusion in mitral stenosis. (False) (False) (True) (False)

E Shows decreased perfusion in McLeod's Syndrome. Comments:

(True)

In radionucleotide lung scans, macroaggregated human serum albumin is injected intravenously, and becomes trapped in the pulmonary capillary bed. The distribution of radioactivity is proportional to pulmonary capillary blood flow. It will, therefore, be abnormal in pulmonary embolism, and congenital cardiovascular and pulmonary defects. Acute changes in distribution or perfusion may reflect alterations 133 in pulmonary ventilation. Using inhaled radioactive xenon ( Xe), the distribution of pulmonary ventilation may be estimated. After intravenous injection of the xenon dissolved in saline, both pulmonary perfusion and ventilation can be evaluated by continuous recording of the rate of appearance and disappearance of the xenon over the lung. The appearance of xenon early after injection is a measure of perfusion, whereas the rate of washout during breathing is a measure of ventilation. Scimitar Syndrome consists of an anomalous vein draining into the inferior vena cava that is visible on x-ray as a crescentic shadow of vascular density along the right border of the cardiac silhouette. McCleod's Syndrome consists of a congenital lung cyst. Copyright 2002 Dr Colin Melville

In cystic fibrosis: A The sweat chloride is higher than the sodium. B The secretions are viscid because water cannot be actively transported form the respiratory epithelial cell. C The amino acid at position 508 of the CTRE gene acts as a regulator of the chloride channel. D The DeltaF508 mutation explains most of the inter-racial differences in the incidence of cystic fibrosis. E The CFTR traverses the cell membrane 7 times, and is arranged in ring formation. Comments: The CF gene codes for a protein of 1480 amino acids called the CF transmembrane regulator (CFTR). This is expressed in epithelial cells of airways, the GI tract (including pancreas and billiary system), the sweat glands and the genitourinary system. In 70% of affected whites, the deletion is in a phenylalanine residue at amino acid position 508 (?F508). Over 400 gene mutations have now been described, and these show particular racial distributions. Cultured CF intestinal epithelial cells homozygous ?F508 are unresponsive to the secretory effects of cholera toxin. The postulated epithelial pathophysiology in airways involves and inability to secrete salt and secondly to secrete water in the face of excessive reabsorption of salt and water. Desiccated secretions become viscous and harder to clear. The defective protein is unable to secrete chloride irons in response to cyclic AMP mediated signals. Copyright 2002 Dr Colin Melville (True) (False) (True) (True) (True)

Pneumocystis carinii: A Predisposes to pneumothorax. B Can cause pneumonia with very few signs on chest x-ray. (True) (False)

C Is an obligate intracellular organism. D May cause extrapulmonary infection. E Is usually diagnosed by finding a rising titre of neutralising antibodies. Comments:

(False) (True) (False)

Pneumocystis carinii pneumoniae is a life-threatening infection occurring in immunosuppressed hosts. With rare exceptions, the organism is localised to the lungs. It is a common extracellular parasite, that has attributes of both fungi and protozoa. It affects 40% of infants and children with AIDS and 12% of those with leukaemia if not prophylaxis is given. Cell mediated immunity is the major defence mechanism, so PCP pneumonia is common in those with severe combined immune deficiency, while it is rarely found in X-linked agammaglobulinaemia. There are 2 clinical forms:

Epidermic infantile: 3-6 month old infants, with subtle onset of tachypnoea but no fever. Progressive recession and increased work of breathing with cyanosis. Sporadic: Children and adults with immune deficiency. Abrupt fever, tachypnoea, dyspnoea and cough with cyanosis.

Added sounds are usually minimal in both forms. The chest x-ray reveals bilateral diffuse alveolitis, initially perihilar, progressing peripherally, and tended to spare the apical areas. Definitive diagnosis requires demonstration of the organism in the lung by bronchio-alveolar lavage, tracheal aspirates, or bronchial brushings, transbronchial lung biopsy, needle aspiration or open lung biopsy. Treatment is high dose septrin plus steroids. Copyright 2002 Dr Colin Melville

Recognised complications of bronchoscopy include: A Haemorrhage B Pneumothorax C Segmental collapse D Hypoxic ischaemic encephalopathy E Empyema Comments: Complications of bronchoscopy depend on the instrument used, the procedure performed and the indication for the procedure. Transient hypoxia, cardiac arrhythmias, layrngospasm and bronchospasm, iatrogenic infection, bleeding, pneumothorax, and pneumomediastinum may occur. Subglottic oedema is much commoner with rigid bronchoscopy than with flexible bronchoscopy. Post-bronchoscopy croup is treated with oxygen and humidification, risimic adrenaline inhaled steroids. Copyright 2002 Dr Colin Melville (True) (True) (True) (False) (True)

Regarding community acquired pneumonia in infancy: A Streptococcus pneumoniae is the most common pathogen. B It may be caused by Staphylococcus aureus. (False) (True)

C Ciprofloxacin is an appropriate treatment if blood cultures are negative. D Bordatella pertussis infection is usually mild. E It may be caused by mycoplasma urealiticum. Comments:

(False) (False) (True)

Respiratory viruses account for the majority of community acquired pneumonia in infancy. These tend to be seasonal, with RSV giving wintertime epidemics. A large number of bacteria may account for pneumonia in the first few weeks of life including chlamydia, mycoplasma (especially urealiticum) and occasionally haemophilus, streptococci and bordatella pertussis. Staphylococcus may be a pathogen at any age. Bordatella usually causes a very severe illness in the first few weeks of life, which is the reason for the immunisation programme. Copyright 2002 Dr Colin Melville

The following are true of cystic fibrosis: A Infertility in men is a result of testicular atrophy. B In children under one year of age the commonest cause of pneumonia is Staphylococcus. C 10% of patients will not require pancreatic enzyme supplementation. D In neonates 10 - 10% present with meconium ileus. E In the school age child, it usually presents with hepatic fibrosis. Comments: In more than 95% of males, the body and tail of the epididimus, the vas deferens, and the seminal vesicles are obliterated or atratic. In females, the glands of the uterine cervix are distended with mucus, and copious amounts of mucus collect in the cervical canal. More than 95% of males are azoospermic, but sexual function is generally unimpaired. Female fertility rate is reduced. Pregnancy may cause a progression in those with severe pulmonary disease. In infancy, the commonest cause of pneumonia is probably viruses with staphylococcal and pseudomonal infections occurring in older children. Colonisation with pseudomonas surpatia may be associated with particularly rapid pulmonary deterioration and death. More than 85% of children show evidence of mild digestion due to exocrine pancreatic insufficiency. Stools are frequent, bulky and greasy, and the children fail to gain weight even when intake is massive. There may be visible droplets of fat, with abdominal protuberance and decreased muscle mass with growth failure. Sexual maturation may be delayed by an average of 2 years. Individuals homozygous for F508 virtually all have pancreatic insufficiency. Prolonged jaundice may be a neonatal presentation of CF. Chronic active hepatitis may also occur, with cirrhosis as a late consequence. Copyright 2002 Dr Colin Melville (False) (False) (True) (True) (False)

The following are recognised complications of foreign body inhalation: A Pulmonary abscess B Asthma C Angioneurotic oedema (True) (False) (False)

D Hyperinflation of the affected lung E Hyperinflation of the opposite lung Comments:

(True) (True)

The symptoms, physical findings and complications of foreign body depend on their nature, location and the degree of obstruction. Sharp objects cause local oedema, infection, while obstructed objects can produce distal atelectasis with later bronchiectasis, pulmonary abscess or empyema. A large foreign body in the larynx may produce a cough that becomes croupy with profound obstruction, aphonia, haemoptysis, dyspnoea, wheeze and cyanosis. X-rays may reveal a radio-opaque foreign body. Obstruction may cause distal collapse with compensatory hyperexpansion of the opposite lung, or may cause a ball valve effect with hyperinflation of the affected lung. Foreign bodies are most likely to enter the right main bronchus because of its more vertical path. Most can be safely removed by bronchoscopy, but the risk of complication is significantly elevated if diagnosis is delayed more than 24 hours. Copyright 2002 Dr Colin Melville

Which of the following statements are true of childhood asthma. A over 90% of patients show exercise-induced bronchoconstriction B hypercapnia is the first physiological disturbance in status asthmaticus C infants are unresponsive to bronchodilators D spontaneous cure occurs before adolescence E cough may be the only symptom (True) (False) (True) (False) (True)

Comments: Exercise induced symptoms occur in approximately 90% of cases of childhood asthma. Hypoxia is the first typical feature of severe asthma with hypercapnia occurring late and is a particularly worrying sign indicating the requirement for ventilation. As a consequence of immature bronchiole musculature, infants are unresponsive to bronchodilators.

Regarding inhaler devices: A Metered dose inhalers can usually be used from the age of about 7 years. B The Spinhaler requires co-ordination of device actuation with inhalation. C The Turbohaler can usually be used from about 3 years of age. D Salbutamol can be used with the Nebuhaler. E A face mask can be attached to a spacer, so that it can be used in infants. Comments: Metered dose inhalers require great co-ordination, and start to be used effectively in children only from about the age of 12 or 13. Most other devices are specific to individual manufacturers, and the drug chosen is more on the basis of the patient's ability to use the inhalation device than on the characteristics of the drug itself. This is because in a chronic disease such as asthma, compliance and ability to use the drug dispensing device makes by far the greatest difference in achieving adequate control of symptoms. A number of inhaler devices have been developed which do not require co-ordination of actuation with (False) (False) (True) (False) (True)

inhalation. These include:

Turbohaler: Terbutaline, Budesonide. From 3 years. Spinhaler: Cromoglycate. From 3 years. Diskhaler: Salbutamol, Beclomethasone. From 3 years.

Spacer devices include:

Nebuhaler: Terbutaline, Budesonide. From infancy (with mask). Volumatic: Salbutamol, Beclomethasone. From infancy (with mask).

Nebulisers can be used at all ages, but recent research suggest they are no more effective than a spacer device with a similar dose of drug given, even in acute asthma. Copyright 2002 Dr Colin Melville

Concerning Cystic Fibrosis: A There is a carrier frequency of 1/220 in the general population. B A sibling of an affected individual has a 2/3 chance of being a carrier. C It can usually be diagnosed antenatally in a family with a surviving affected member. D Linkage disequilibrium probes may be useful in epidemiological studies. E In suspected cases, the sweat test is the most appropriate first investigation. Comments: The carrier frequency is 1:22 in Caucasian populations, though considerably less in other ethnic groups. This can usually picked up antenatally using appropriate probes. Previously linkage disequilibrium probes were useful epidemiologically, though more often now more specific probes are available, e.g. for the delta F508 mutation. If there is an affected sibling, then this implies that both parents are carriers. Neither is likely to be homozygote, because of the reduced fertility, particularly in males. Siblings therefore have a 25% chance of being affected, a 50+% chance of being carriers, and a 25% chance of being unaffected. The sweat test is still the most appropriate first investigation. Copyright 2002 Dr Colin Melville (False) (False) (True) (True) (True)

Hypoxaemic respiratory failure (Type I): A Can be caused by respiratory muscle weakness and fatigue. B Is found in mountain sickness. C Can lead to pulmonary hypertension. D Can lead to CO2 retention if treated with 100% oxygen. E Can lead to ventricular failure. Comments: (False) (True) (True) (False) (True)

Hypoxaemia can occur because of 3 mechanisms:

Hypoventilation: muscular dystrophy, e.g. Werdig-Hoffman, Guillain-Barre, drugs, myasthenia gravis. Diffusion impairment: pneumonia (bacterial, viral, TB). VQ inequality: obstructive airways disease, restrictive airways disease.

ARDS and hyaline membrane disease in addition to lung infections cause both diffusion impairments and VQ inequality. In the case of VQ imbalance and hypoventilation, CO2 usually rises (type II respiratory failure). In these cases, the patient relies more and more on hypoxia to maintain respiratory drive, an excess of oxygen can be counterproductive. Hypoxia is a potent pulmonary vasoconstrictor, and can lead to pulmonary hypertension. The brain and myocardium are also particularly sensitive to hypoxia. Copyright 2002 Dr Colin Melville

Regarding idiopathic primary pulmonary haemosiderosis: A It is inherited as an autosomal recessive. B The absence of digital clubbing is usual. C Fever is generally absent. D Patients usually have associated polycythaemia. E There is often immunoglobulin of complement deposition in the histology of lung biopsies. Comments: The cause of this condition is unknown. Most manifestations are due to loss of blood into the alveoli, and the affects of chronic blood loss. There is cough, haemoptysis, dyspnoea, wheezing and occasional cyanosis associated with fatigue and pallor. There may be bloody sputum, or vomiting of large quantities of blood. The child is usually febrile during acute attacks, and digital clubbing is often present. The condition is initially, therefore, difficult to differentiate from recurrent bacterial pneumonia. Initially, the only early manifestation may be chronic iron deficiency anaemia, with typical haematological indices. The stool usually contains occult blood from swallowed sputum. Open lung biopsy demonstrates haemosiderin laden macrophages, and the absence of immunoglobulin or complement deposition on the alveolar basement membrane virtually excluded Goodpasture Syndrome. Copyright 2002 Dr Colin Melville (False) (False) (False) (False) (False)

Which of the following may cause pulmonary hypertension? A coarctation of the aorta B pulmonary stenosis C patent ductus arteriosus D kyphoscoliosis E schistosomiasis (False) (False) (True) (True) (True)

Comments: Pulmonary hypertension may occur as a primary disorder or secondary to cardiac diseases such as PDA,

VSD, ASD etc. It is also recognised with intrinsic lung diseases such as fibrosis and with structural abnormalities - kyphoscoliosis.

Frequent episodic asthma: A Is suffered by 42% of all children with asthma. B Is defined as an attack rate of ever 2-4 months. C Should be treated with inhaled regular prophylactic therapy, such as inhaled steroids. D Is characterised by normal growth rate. E Usually requires a burst of oral steroids to bring under control. Comments: Types of chronic asthma include: (False) (False) (True) (True) (False)

Infrequent episodic asthma: affects 75% of asthmatic children, with fewer than 4 episodes per year. Intermittent bronchodilators are given. Frequent episodic asthma: 20%, symptoms 2-4 weekly. Low dose inhaled prophylactic therapy plus intermittent bronchodilator. Persistent asthma: 5%, high dose inhaled prophylaxis plus intermittent bronchodilators longacting bronchodilators such as salmeterol. These children need regular monitoring in an asthma clinic and recording of growth and asthma diary. Exercise-induced: pre-exercise bronchodilator. The British Asthma Society Guidelines have recently been updated (1997), and you are strongly advised to familiarise yourself with the step up and step down approach.

Copyright 2002 Dr Colin Melville

A 9 month old child presents with respiratory distress, worsening over 2 days. Blood gases show a pH of 7.25, a PCO2 of 7.5kPa, a PO2 of 8.5kPa, and a base excess of -4. A Results are consistent with bronchopulmonary dysplasia. B Blood gases suggest type 1 respiratory failure. C Immediate intubation is required. D Results are consistent with late severe asthma. E Bicarbonate may be necessary to correct the acidosis. Comments: In interpreting blood gas results, the following sequence may be useful: (False) (False) (False) (True) (False)

Inspect the pH: Is it low, normal or high? Inspect the CO2: Is it low, normal or high? Inspect the PO2: Is it low, normal or high?

If the pH is low then an acidosis is present, and inspecting the CO2 will enable you to determine whether this is due to respiratory or metabolic causes. Inspecting the PO2 will tell you whether the child is hypoxic or not. In this case, the pH is reduced, and the CO2 is high, with a base deficit of only -4, insufficient to explain the acidosis from metabolic causes. This is, therefore, a respiratory acidosis, and the PO2 is also a little low suggesting type 2 respiratory failure. Possible causes would include pneumonia, early hyaline membrane disease, ARDS. In asthma, the initial stages show a low CO2, with this climbing only as a preterminal event. The results would therefore be consistent with late severe asthma. In bronchopulmonary dysplasia, there is usually long-term CO2 retention with compensatory increase in bicarbonate leading to a positive base excess and normal pH. Bicarbonate is usually only considered if the base deficit exceeds about -8 or 10. Copyright 2002 Dr Colin Melville

Long-acting 2 agonists: A Can be used to prevent activity-induced symptoms without anti-inflammatory therapy. B Become less effective over time (tolerance). C Are beneficial in acute viral croup. D Protect against allergen challenge for up to 48 hours. E Should not be used in association with erythromycin. Comments: Long-acting 2 agonists, e.g. salmeterol, can be used twice daily to assist in prophylaxis in chronic asthma as Step 3 of the British Thoracic Society Asthma Guidelines. There is no evidence that the bronchodilator effect wanes with time, though there is debate that it may become less effective in protecting against exercise or methocoline induced bronchospasm. Its duration of action is around 12 hours, and has gone completely by 36 hours. Aminophylline interacts with erythromycin, giving an increased risk of toxicity. There is no evidence that salmeterol works in viral croup, though oral steroids are highly effective. Copyright 2002 Dr Colin Melville

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The oxygen dissociation curve is shifted to the right by: A Decreased haemoglobin concentration B Reduced temperature C Reduced pH D Increased partial pressure of carbon dioxide E Increased DPG Comments: The curved shape of the oxygen dissociation curve means that the loading of oxygen to the tissues is little affected by significant drops in alveoli PO2 concentration. The steep lower part of the dissociation curve means that peripheral tissues can take off large amounts of oxygen for only a small drop in capillary PO2, assisting the diffusion of oxygen into the tissues. The position of the oxygen dissociation curve is shifted to the right by acidosis, hypercapnia, raising the temperature, and increasing the amount of 23DPG (23 diphosphoglycerate, and end product of red cell metabolism, whose concentration (False) (False) (True) (True) (True)

increases in chronic hypoxia, at altitude, or in chronic lung disease). A reduced haemoglobin reduces the total oxygen carrying capacity of the blood, but does not change the shape of the curve. Copyright 2002 Dr Colin Melville

In lung empyema: A Strep. pneumoniae is usually isolated from the pleural cavity. B Installation of urokinase may be helpful. C Anti-TB triple therapy is indicated if the fever does not settle within 14 days. D An underlying malignancy should be excluded. E Initial treatment of the pneumonia has been inadequate. Comments: Empyema is purulent pleurisy, an accumulation of pus in the pleural spaces. In the majority of cases, no organism can be grown, but organisms most commonly associated include Staphylococci, pneumococci, and haemophilus influenzae. It occurs most frequently in infants and pre-school children. It can also occur by extension from a lung or intra-abdominal abscesses, or following trauma or thoracic surgery. There are often multiple locculations. The initial signs are of a pneumonia, and most patients have a fever. X-ray suggests fluid in the pleural space. A chest drain is often required and urokinase is being trialed to assist in breaking down the locculations. Complications of Staph. infection include bronchopleural fistulae, pyopneumothorax. Other complications include purulent pericarditis, pulmonary abscess, peritonitis, osteomyelitis of the ribs. Disseminated infection including meningitis, arthritis, osteomyelitis. Response to antibiotics is generally slow and may be required for 3-4 weeks. Copyright 2002 Dr Colin Melville (False) (True) (False) (True) (False)

Regarding lung development: A The pseudoglandular phase lasts between 16 and 26 weeks. B Alveolar capillaries first appear about 20 weeks of gestation. C The primitive airways appear as a dorsal outpouching of the foregut epithelium. D The pulmonary vascularture is derived from endoderm. E The peribronchial mesenchyme (spalnchnopleura) plays an essential role in shaping the lungs during embryogenesis. Comments: Lung morphogenesis is divided into 5 periods: 1. 2. Embryonic: 4-7 weeks, primitive airways appear as ventral outpatching of the endoderm of the foregut. The bronchial buds branch, with the peribronchial mesenchyme having an essential role in shaping the lungs during embryogenesis. Pseudoglandular: 7-16 Weeks , lungs resemble an exocrine gland, with major airways present, and close association between pulmonary arteries and veins. Incomplete separation of trachea and foregut result in tracheaoesophageal fistula. The diaphragm forms during this period, and failure to form results in diaphragmatic herniae. (False) (False) (False) (False) (True)

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Canalicular: 16th - 26th week gestation. Epithelial growth predmoninants over mesenchymal growth. The bronchial tree becomes tubular with distal subdivision, and epithelial cells develop into type 2 pneumocytes (surfactant producing). Saccular: 26th - 28th week. Terminal airways widen, forming saccules. Alveolar: No clear division between this and the saccular period, but probably around 32 weeks onwards. Production of alveoli is stimulated by physical breathing movements and thyroid hormones. It is impaired by glucocorticoids. Their failure to develop results in pulmonary hypoplasia, for example, in spinal cord lesions.

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Recognised causes of acute upper airway obstruction include: A Angio-oedema B Asthma C Mumps D Retro-pharyngeal abscess E Laryngomalacia Comments: The differential diagnosis includes: Croup: Viral (very common), recurrent or spasmodic (common), and bacterial tracheitis (rare), and epiglottitis (uncommon, but very important). Rarer Causes: Quinsy, laryngeal foreign body, angio-oedema, smoke inhalation, trauma, diphtheria, measles, EBV infection. Laryngomalacia causes chronic stridor, though this may present acutely e.g. during superadded viral infection. Copyright 2002 Dr Colin Melville (True) (False) (False) (True) (False)

The following lung function tests are compatible with severe scoliosis: A An FEV1 of 65% of normal. B An FEV1/2 of 65% of normal. C Total lung capacity of 95% of normal. D Tidal volume of 105% of normal. E Peak flow of 50% of normal. Comments: Restrictive lung disease occurs when the lungs or the structures that surround them (pleura, rib cage or abdomen) limit the expansion of the lungs in a volume dependent fashion. Examples are interstitial abnormalities (pulmonary oedema, inflammation), collapse or consolidation of alveoli, or external (True) (False) (False) (True) (True)

compression of the lungs (ascites, pneumothorax). The consequences are: Increased muscle effort required to maintain tidal volume. Alveolar volume decrease, so there is decreased functional residual capacity, arterial PO2 decreases, and there is arterial hypoxaemia causing respiratory drive to increase. There is a decreased residual breathing capacity. Because there is no obstructive defect, the slope of the forced expiratory curve is initially normal, until limited by the restrictive nature of the defect. The FEV1 may, therefore, be reduced, but the FEV1/2 is unlikely to be significantly down. Total lung capacity is usually severely reduced. Tidal volume may be normal, but there will be decreased respiratory reserve. Peak flow is usually reduced. Copyright 2002 Dr Colin Melville

Concerning Tuberculosis: A The infection rate is increased in Crohn's Disease. B Overcrowded living conditions do not significantly affect prevalence. C The treatment of lymph node infection is of a greater duration than pulmonary infection. D The tuberculin skin test is a good indicator of disease activity. E In pregnant women treatment should be delayed until after birth. Comments: Clinical features of TB depend on whether the infection is primary or following reactivation. Primary infection: Asymptomatic in 50% of infants and 90% of older children. There is local 'walling off' of the bacteria by inflammatory cells, but the Mantoux test may be become positive, and the disease may reactivate later. Symptomatic: the local host response fails to restrict the tubercle bacilli. In the lung, the infective focus is the Ghon focus and together with the local spread to regional lymph nodes this constitutes the Ghon complex. After a few weeks, fever, weight loss, cough and chest x-ray changes occur. There may be healing of the primary complex with calcification, and enlargement of peribronchial lymph nodes. Pleural effusions may also occur, and there may be local dissemination. Primary infection rarely affects other organs such as the gut, skin and superficial lymph nodes. Reactivation: Both asymptomatic and symptomatic infections may become dormant, subsequently reactivating and spreading via lymphatic and haematogenous spread. Post-primary TB may be local or disseminated (milliary), or may affect the bones, joints, kidney, pericardium or CNS. TB meningitis is particularly common in children under the age of 2, and early recognition is necessary to prevent serious morbidity or mortality. The incidence of TB has greatly increased on the back of HIV. Infection is prevalent in areas of overcrowding and poor living conditions. The tuberculin skin test may become positive even in asymptomatic disease, and is not a good indicator of disease activity. Pregnant women should be treated immediately to minimise the chance of them passing on the disease to their offspring. Copyright 2002 Dr Colin Melville (False) (False) (False) (False) (False)

The following are signs of severe asthma: A A silent chest in a 7 year old girl. B Inability to feed in a 10 month old child. (True) (True)

C A heart rate of >90 in a 5 year old child. D Decreased right-sided breath sounds in a 10 year old girl. E A respiratory rate of 60 in a 2 year old boy. Comments: Features of severe and life-threatening acute asthma include:

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Severe: Too breathless to talk of feed, respiratory rate >50/min, pulse rate >140/min, peak flow <50% predicted or best value. Life-threatening: Peak flow <33% predicted or best value, fatigue, agitation, drowsiness, cyanosis, silent chest, or a poor respiratory effort.

Copyright 2002 Dr Colin Melville

Lung biopsy may be useful in the following cases: A A 6 month old boy ventilated for adenovirus infection. B A 2 year old with leukaemia and possible adreamycin toxicity. C A 4 year old child with dense lower zone opacities on chest x-ray. D A 3 month old Afro-Caribbean boy with "ground glass" chest x-ray. E A 4 month old with severe confirmed RSV positive bronchiolitis. Comments: In case A, adenovirus can be complicated by an obliterative bronchiolitis, and a lung biopsy may be helpful in diagnosing this. Adriamycin causes a cardio-toxicity, with acute myocarditis and chronic cardiomyopathy both occurring. This is dose dependent, occurring in 30% of patients with a total 3 cumulative dose >550mg/m . Part D describes a typical presentation of HIV infection, with pneumocystis as the likely organism. Lung biopsy is a recognised means of confirming this diagnosis. Lung biopsy may be the only way to establish a diagnosis, especially in protractive non-infectious disease. In infants and small children, an open surgical biopsy is the procedure of choice. In older patients, transbronchial biopsies using flexible forceps and fluoroscopic guidance can be used. This is most appropriate where there is diffuse lung disease such as pneumocystis. Lung biopsy can also be performed with a thoracoscope depending of the location and nature of the lesion requiring biopsy. Copyright 2002 Dr Colin Melville (True) (False) (False) (True) (False)

Regarding respiratory surfactant: A It is produced by alveolar type 2 epithelial cells. B It is composed primarily of phospolipids such as dipalmitoyilelecithin. C It reduces lung compliance by changing surface tension. D Production may be enhanced by the administration of antenatal steroids to mother. E At 24 weeks gestation, production is 75% of that at term. (True) (True) (False) (True) (False)

Comments: Surfactant is produced by type 2 (Clara) cells, and consists principally of phospholipids such as dipalmitoyilelecithin, which is synthesised from fatty acids. Surfactant is formed relatively late in fetal life, and deficiency leads to respiratory distress syndrome. By reducing surface tension, the compliance of the lung is increased, and the work of breathing is reduced with each breath. Each alveoli is inherently unstable and liable to atelectasis (collapse).

The following syndromes are associated with respiratory tract abnormalities: A CHARGE Syndrome B Ciliary dyskinesia C Hurler's Syndrome D Tay Sachs Disease E Cri-du-Chat Syndrome Comments: CHARGE Syndrome consists of Coloboma, Heart disease, Atresia co-ani, Retarded growth and development and or CNS abnormalities, Genital anomalies and/or hypogonadism, Ear abnormalities and/or deafness. 50% of infants with co-anal atresia have other anomalies consistent with this syndrome. Ciliary dyskinesia includes syndromes such as Kartagener's which is due to ciliary dysmotility, and is associated with chronic sinusitis, situs inversus. In Cri-du-Chat Syndrome, the abnormal larynx is responsible for the strange cry in infancy. Copyright 2002 Dr Colin Melville (True) (True) (False) (False) (True)

Characteristic features of idiopathic diffuse interstitial fibrosis of the lung (HAMMAN-RICH) include: A Cyanosis on exercise B Inspiratory crackles on auscultation C Hypercapnia D Decreased FEV1/FVC ratio E Decreased gas transfer factor Comments: This is a rare, chronic and often fatal disorder only occasionally seen in infants and children. There is an uncontrolled inflammatory process leading to progressive fibrosis. Clinically, there is progressive pulmonary insufficiency resulting from interstitial fibrosis and alveolar-capillary block. The onset is insidious with dyspnoea on exercise, later at rest. Gradually, anorexia, weight loss, fatigue ability followed by cyanosis, clubbing, cor pulmonae and right sided heart failure occur. Inspiratory crackles may be heard. There is no increase in airway resistance, but vital capacity, compliance and diffusion capacity are decreased. The spirometry shows a restrictive defect, with an increased FEV1:FVC ratio. Copyright 2002 Dr Colin Melville (True) (True) (False) (False) (True)

The following conditions are associated with sleep apnoea: A Anorexia nervosa B Large uninflammed tonsils C Guillain-Barre Syndrome D Ondine's Curse E Diencephalic Syndrome Comments: Several medical conditions are major risk factors for obstructive sleep apnoea and hypoventilation. Anatomical Factors: Adenotonsillar hypertrophy, micrognathia, retrognathia, macroglossia, morbid obesity, congenitally narrow nasopharynx, swollen nasal turbulence, co-nasal stenosis. Diminished ventilatory responses to hypoxaemia and hypercapnia. Diminished arousal responses. Sedation or general anaesthesia. Guillain-Barre Syndrome can occasionally cause bulbar palsy, which is associated with upper airway obstruction. Copyright 2002 Dr Colin Melville (False) (True) (True) (True) (False)

A 7 year old girl presents with acute cough and wheeze, and is given nebulised salbutamol. The following are indications for admission to hospital: A A peak flow rate of 80% of that predicted for height. B Continuing cough. C Respiratory rate of 40/min. D Saturation of 90% in air. E She looks tired. Comments: Patients should be given nebulised bronchodilator therapy, and admitted if: 1. 2. 3. 4. They have not responded clinically. They are exhausted. Their peak flow is markedly reduced (severe is defined as <50% of predicted or best value, and life-threatening as <33% of predicted or best value). The oxygen saturation is reduced below 92%. (False) (False) (False) (True) (True)

Copyright 2002 Dr Colin Melville

Chest signs that are reliable diagnostically are found in: A A neonate with meconium aspiration. B A 3 month old child following aspiration pneumonia. (False) (False)

C A 4 month old child with bronchiolitis. D A 6 month old with lobar pneumonia. E A 2 year old with inhaled foreign body. Comments:

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Unfortunately, clinical chest signs are remarkably unhelpful, and in general are less helpful the younger the child. Although in meconium aspiration chest signs may be found such as coarse crepitations, the diagnosis is usually on the basis of the general appearance of the child (meconium stained, meconium in the oro-phayrnx or recovered from the lungs) associated with an appropriate chest x-ray. A 3 month old with aspiration pneumonia normally presents with a sudden onset of respiratory distress, but focal signs are often difficult to locate at this age. A child with bronchiolitis, however, has more generalised signs, and a fairly typical clinical picture. Lobar pneumonia can easily be missed in a 6 month old child, and a chest x-ray is, therefore, usually included as part of a septic screen. The diagnosis of inhaled foreign body may be difficult even on x-ray in a 2 year old child. Copyright 2002 Dr Colin Melville

In the normal adolescent lung: A There is an intrapleural pressure of 30cmH2O (3kPa) at the end of normal expiration. B There is a resting pulmonary blood flow of 10L/min. C The V:Q ratio is greater in apical than basal segments of the lung when upright and at rest. D The majority of airway resistance is from large airways. E Cartilage is present in all respiratory bronchioles. Comments: Because of surfactant, the pressure difference across the pleura required to inflate the lungs, is usually no more than about 4cmH2O. Resting pulmonary blood flow in an adult is around 5L/min. Gas rises, so the V:Q ratio is higher in the apical than the basal segments. The majority of airway resistance is from small airways, which is why asthmatics have a vastly increased airway resistance. Cartilage disappears in the terminal bronchioles. Copyright 2002 Dr Colin Melville

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