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67 Cards in this Set
- Front
- Back
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What is a cough?
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An airway defence mechanism to prevent inhalation of foreign particles and to assist the removal of foreign particles and secretions from the airways. It is initiated voluntarily or by reflex.
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What are some respiratory defence mechanisms?
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Nose filtration of air by hair, turbines and conchae; laryngeal receptors which induce reflex glottis closure; mucociliary escalator comprised of ciliated cells, airway surface liquid, mucous and cough reflex removing particulate matter including bacteria laden alveolar macrophages, from the lower airways.
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What are the 3 phases of a cough simulated in the lower respiratory tract?
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Inspiratory phase, glottis closure with thorax compression by diaphragmatic relaxation and ab muscle contraction, expulsive phase with rapid airflow through trachea. Usually associated with bronchoconstriction to improve clearance.
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What nerve innervates structures associated with cough?
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CNX, Vagus nerve
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What is different about a cough activated from larynx receptors compared to one activated from senses further down the respiratory tract?
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The initial phase is expiratory, there is no inspiratory phase as this would move the particle deeper down into the lungs.
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What is the furthest division of the airway which can stimulate a cough reflex?
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Segmental bronchi, any stimulation distal to this point will not elicit a cough.
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What stimuli types can cause cough receptors be stimulated?
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chemical, mechanical or inflammatory stimuli
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Where is Inflammation the most potent stimuli for cough?
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larger airways, trachea, larynx
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Where are cough receptors most numerous?
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Around carina/bifurcation points in the bronchial tree.
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What type of receptors are the main mediators of cough?
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Rapidly adapting receptors (RARs) or Irritant receptors, that respond to mechanical and chemical stimuli. They have myelinated fibres along th vagus to the cough centre in the medulla oblongata.
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What do unmylinated C fibres do with regards to coughing?
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Modulate the cough response. Respond to the same chemical and mechanical stimuli as RARs, but directly inhibit cough, while bronchial types are also able to induce neurogenic inflammation through stimulation of nerves’ release of bradykinin and tachykinins which can stimulate RARs.
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What are the different ways that a cough are classified?
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Dry or productive, acute or chronic (>3wks).
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What are some causes of chronic cough?
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chronic bronchitis, asthma, post-nasal drip, gasto-oesophageal reflux, bronchiectasis (cancer??).
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What is the carrier rate of cystic fibrosis in Europeans?
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1 in 22.
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How was the gene involved in cystic fibrosis discovered?
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Positional cloning, allowing cloning of a likely region (ie chromosome 7 as its genes are expressed in CF effected tissues) and characterisation of the specific protein produced by the genes until a candidate gene was found. The gene was named CFTR.
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What is the most common mutation of the CFTR gene which interferes with its protein product’s function?
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DeltaF08, a 3 pair deletion found in 75% of CF patient’s chromosomes.
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What conditions were able to be identified as forms of CF through molecular analysis?
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Congenital bilateral absence of the vas deferens, some bronchiectasis and pancreatitis’.
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What is bronchiectasis?
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Lung condition involving the irreversible dilatation of one or more bronchi, generally by destruction of elastic and muscular components of the wall. It is often the end result of an underlying disease process.
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What can cause bronchiectasis?
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Any cause of chronic bronchial infection - childhood lower respiratory infections (whooping cough or measles), tb, congenital disorders (hypogammaglobulinaemia, Primary ciliary dysfunction), recurrent gastric aspiration, Young’s syndrome (viscous mucous), Yellow nail syndrome (titanium poisoning?).
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How is bronchiestasis recognised clinically?
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Presence of suppuratives bronchopulmonary disease, history of frequent cough productive of copious purulent secretions. CXR with thickened airway walls and dilated airways. Course crackles on auscultation.
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What are the changes in the type of appearance of bronchiectasis from mild to severe?
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Cylindrical bronchiectasis, varicose bronchiectasis, saccular/cystic bronchiectasis.
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What are complications of bronchiectasis?
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Chronic suppuratives infection leading to lung destruction/respiratory failure, haemoptysis, massive pulmonary haemorrhage, cor pulmonale at late stage, clubbing.
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What causes the break down of tissure in bronchiectasis?
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Proteolytic enzymes from inflammatory cells (elastase/collagenase), oxygen free radicals.
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What more serious outcome might follow a localised bronchiectasis?
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Obstruction from foreign bodies, mucoid impaction, hilar lymphadenopathy and neoplasm.
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What type of pattern of inheritance is CF?
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Autosomal recessive.
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What is PCD?
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Primary Ciliary dyskinesia, structural abnormalities (dynein arm mutation, cilary disorientation) of reparatory epithelial cilia leading to inadequate removal of secretions.
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What is Kartagener’s syndrome?
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A triad of situs inversus (organs on the opposite side to usual), sinusitis and bronchiectasis (the latter two due to Primary Ciliary Dystfunction).
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What is the prototype immunodeficiency condition that can cause chronic sinopulmonary infection?
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Agammaglobulinaemia, where cilia and airway liquid is normal but lack of Ig allows growth of bacteria which damages the airways.
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How is bronchiectasis treated?
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Specific where possible (removal of obstruction, replacement of gammaglobulin), general infection control and removal of purulent secretions by antibiotics, physiotherapy and other drugs, and the use of exercise to maintain airway clearance.
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What was the median survival for Cystic fibrosis patients in 2010?
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39 years.
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When was new-born screening for Cystic Fibrosis commenced in NSW?
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1981
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What is the most important indicator for establishing the prognosis of a CF patient?
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Lung function, as 90% of mortality is due to progressive respiratory failure.
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What are non-genetic factors known to be involved in the prognosis of a patient with CF?
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sputum pathogen (p.aeruginosa, b.cepacia); nutritional status; presence of diabetes; care within a specialised CF clinic; overall compliance; family support; passive and active smoking; pancreatic status (related to lung sufficiency).
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Do men or women survive better in CF?
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Men, maybe related to hormones or exercise factors.
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What pulmonary complications are associated with adverse prognosis in CF?
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Pneumothorax, massive haemoptysis, cor pulmonale.
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What are the 6 Classes of CFTR mutations?
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Class I mutations = stop/nonsense protein that is breaken down; Class II mutations = altered processing/folding of protein leading to it being broken down; Class III mutations = altered regulation of the channel/unable to open normally; Class IV mutations = altered conductance of channel/unable to fit ion through; Class V mutations = normal channel with reduced production; Class VI mutations – decreased stability of protein/abnormal regulation of other channels so that it is degraded more quickly.
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What class does the mutation deltaF508 belong to?
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Class II – Incorrect processing/folding leading to the proteins destruction.
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What genetically directed treatments are available to patients with CF?
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CFTR correctors (read through stop in Class I, blocking of breakdown pathway in Class II), CFTR potentiators (make the channel work better in Class III, and possibly in IV, V and VI too).
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What treatments are used to manage chest disease in CF?
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Treatment of broncho pulmonary infection with antibiotics, daily removal of viscid secretions by physioltherapy, bronchodilators to increase airway activity, regular exercise/fitness, at later stages nocturnal oxygen for improved QOL, mucolytics to reduce viscosity, lung transplantation.
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Do you want a CF patient to have high or low energy diets?
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High energy with pancreatic enzyme supplements as they otherwise have issue digesting fats and ADEK vitamins.
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What organisms usually colonise the airways of a CF patient?
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Staphylococcus aureaus early on and 90% get pseudomonas aeruginosa later, associated with a decline in pulmonary status.
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Is the CFTR gene a large gene?
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Yes, it is 27 exons, `250 kb of DNA
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What is the value of DNA genetic diagnoses?
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A genetic diagnosis can be made at any age or stage of development, including efore signs and symptoms of that disorder appear. Any tissue provides a satisfactory source of DNA, unlike protein based assays. It can detect carriers as well as the at-risk.
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What does the deltaF508 mutation involve?
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pheynylalaline (F) at position 508 in the protein is deleted (delta), it is in 70% CF chromosomes.
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Rate is the overall detection rate of CF from DNA testing in Australia?
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80%
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What other disease mutation, besides CFTR, is also especially ethinic specific?
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Thalassaemia
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Why might the process of a medical practitioner explaining the genetic testing for CF be difficult?
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The technology is complex, the result obtained may have wider implications for the whole family.
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What are the common pathogens which cause Bronchitis, and what do you use as treatment?
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Streptococcus pneumonia, Haemophilus influenza, moraxella catarrhalis, treat with amoxycillin OR doxycillin.
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What is the pathogen commonly responsibly for bronchiolitis, and how do you treat it?
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Bordatella pertusis, treat with Azithromycin OR clarithromycin OR Erythromycin.
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What are the common pathogens that cause bronchiectasis and what guides treatment?
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Bordatella pertusus, staphylococcus aureus, pseudomonas aeroginosa, therapy is often guided by findings on sputum examination.
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What are the common and the atypical pathogens that cause pneumonia, and how do you treat for them?
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Strep pneumonia, mycoplasma pneumonia, Chlamydia pneumonia, and treat with Beta lactam PLUS macrolide. Atypical pneumonia is caused by Legionelal pheumophila, Chlamydia psittaci, staphylococcus aureus, mycobacterium tuberculosis, anaerobes and burkholderia pseudomallei, look at specific guidlines for individual treatment.
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Should you use antibiotics in acute bacterial bronchitis?
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No, unless the patient has a chronic lung disease.
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What are intrapersonal issues which can develop in a recurrently or chronically ill child?
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Regressive behaviour, low self esteem/social withdrawal/oversensitivity, moodiness/depression, denial/rebellion, secondary gain.
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How does regressive behaviour manifest itself in a child?
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It is turning back to a more immature pattern of feeling and thinking during stress, serving a defensive purpose. In children,m it may be shown as clinging or demanding behaviour.
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Explain how a chronically ill child might become socially withdrawn?
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If a child feels less competent in their life, they are likely to evaluate themselves in negative terms, and perhaps withdraw from their social group, which may potentiate depression.
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What is the estimated prevalence of children with a moderate to severe chronic handicapping illness?
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5-10%
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Describe how moodiness or depression may develop in a chronically ill child?
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Frequent separation from parents, hospital environment, anxiety over not understanding illness.
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How might a child present with depression?
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somatic complaints such as crying, enuresis (bed-wetting), encopresis (incontinence), sleep disturbance and anorexia.
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How does denial or rebellion develop in a chronically ill youth?
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Normal exploratory behaviour may be limited by hospital rules, the medical condition, parental concerns. The child may act our or may become withdrawn and passive.
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Why are 3-5 year olds especially at risk of denial or rebellion during hospitilisations?
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1) The parent is the primary attachment object, yet they are unable to control much of what happens to the child in hospital. 2) The child is at a stage wehre they wish to establish dependence and autonomy, but they are restricted due to the passive role required within a hospital. 3) Preoperational cognitive abilities exist, making the ordeal difficult for the child to comprehend.
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Explain secondary gain in terms of chronic illness.
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This is when a child sees the benefits of being ill such as increased attention and privileges, and accentuates behaviours and allows themself to become more ill by being non-compliant in order to receive these benefits.
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What are the factors which can hinder/help a parents’ compliance for their child’s treatment?
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parent’s perception of the child’s vulnerability, parents’ own health beliefs, their perceptions of the severity of the illness, their child’s actual vulnerability, potential effectiveness of the medical treatment.
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What is QOL?
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Quality of life, the measurement of physical psychological and social functioning of the patient.
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Which sex has worse QOL as self-assessed in CF patients?
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Women.
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Do males or females rely more on denial to cope with the stress of chronic illness?
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Females
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What, in detail, is the cause of the pathology that occurs in CF?
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A mutation in CFTR that transports Cl. In most exocrine gland duct cells (GIT, lungs, reproductive) CFTR transports Cl out of the cell, which decreases the functionality of ENaC Na transport into the cell. So in CF, Cl stays in the cell, Na is transport into the cell isn't decreased, so water osmotically follows it into the cell, leading to dehydrated mucous, meaning that cilia are unable to effectly clear it, leading to mucous plugs, and an increased risk of infection and inflammation and fibrosis, destroying tissue function. The exception to this process is in sweat gland duct cells, where CFTR transports Cl into the cell from fluid, and enhances ENaC Na transport in, so in CF, reduced/absent functioning leads to saltier sweat.
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How is the CFTR channel activated?
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Agonist induced cAMP increase activates pKA that phosphylates the R domain of the CFTR, allowing ATP to bind to the nucleotide binding domain in order to open/close the channel pore.
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