Respiratory Flashcards

Question

ARDS (def, causes -inc most common cause - management)

Answer 1

resp distress, new bilat patchy or homogeneous pulm infiltrates, no cardiogenic cause for this oedema (pulm art pressure <18) common causes inc pneumonia, aspiration, sepsis, shock; rarer inc fat or amniotic fluid embolism, reperfusion after lung transplant or pulm embolectomy, inhalational injury inc near drowning, acute panc, TRALI, eclampsia, burns, vasculitis, altitude, heroin/barbiturate overdose sepsis is commonst predisposing factor, 20-40% severe sepsis pts will dev ARDS mechanical ventilation cornerstone of management, limit oedema with diuretics and fluid restriction, pt adopt prone position; surfactant replacement in neonatal resp distress

Answer 2

ARDS: Berlin Definition, which broadly consists of 4 key points: Acute onset within 7 days PaO2:FiO2 ratio <300 (with PEEP or CPAP >5cmH2O) Bilateral infiltrates on CXR Alveolar oedema not explained by fluid overload or cardiogenic causes The degree of ARDS severity can be further defined, based on degree of hypoxemia via the PaO2:FiO2 ratio direct causes: pneumonia, smoke inhalation, fat embolus, aspiration (not just asp pneum); indirect inc polytrauma, sepsis, major burns, acute panc dyspnoea, usually in the presence of a related risk factor or underlying cause. This then rapidly leads to hypoxia and tachypneoa, often with inspiratory crackles on auscultation Multiple conditions present in a similar manner to ARDS, therefore ensuring the Berlin criteria are met for the diagnosis to be made is key. Other differentials to consider include Congestive Heart Failure, Interstitial Lung Disease, Diffuse Alveolar Haemorrhage, and Drug-Induced Lung Injury. ABG, routine bloods (and inc amylase/CRP), CXR, echo to exclude cardio cause (i) supportive treatment with ventilation (ii) focused treatment of the underlying cause. It is highly likely that patients with ARDS will require early intubation and ITU admission for respiratory and circulatory support. Patients who remain severely hypoxic despite conventional therapy, Extra-Corporeal Membrane Oxygenation (ECMO) can be considered *Proning patients has also been shown to improve oxygenation and CO2 clearance, therefore can be used in conjunction to the other ventilation measures

Answer 3

SIRS if 2 or more of temp >38/<36, HR >90, RR >20, WBC >12,000 or <4000 sepsis if SIRS + source of infection severe sepsis if lactic acidosis or SBP either <90 or dropped 40 or more from normal septic shock if severe sepsis w hypotension despite adequate fluid resus

Answer 4

t1rf can be split based on A-a gradient (alveolar pO2 - arterial pO2), and all causes of t1rf can progress to t2rf if sufficiently severe normal gradient if FiO2 down gradient decreased if: Diffusion defect: Structural changes to the alveolar component of the alveolar-capillary interface, such as decreased surface area or increased thickness, may result in diffusion defects across the membrane. Hypercapnia does not occur, as carbon dioxide more soluble than oxygen; seen in emphysema, ILD, ARDS, CHF V/Q mismatch: PE, COPD r->l shunt: physiological when V/Q ratio reaches zero (no vent, still perfusion); AVM, complete atelectasis, severe pneumonia or oedema t2rf either manifested by "won't breathe" due to a central drive issue or "can't breathe" as a result of a peripheral neuromuscular defect, resistive loading (narrow airway) or restrictive defect or dead space volume >50% that leads to hypoventilation and hypercapnia note CO2 more soluble than O2 so less lung needed for its effective removal, hence why the t1rf aetiologies don't cause t2rf initally alcohol, benzos, opioids, encephalitis, stroke, tumour, head/cord injury GBS, myasthenia gravis, organophosphate, SCI/transverse myselitis, ALS, tetanus/botulism flail chest, kyphoscoliosis, OHS, effusions muscular dystrophy, diaphragm paralysis/rupture hypovent with dead space >50% eg PE, bronchitis/COPD, bronchiectasis

Answer 5

Obesity hypoventilation syndrome is a respiratory consequence of morbid obesity that is characterized by alveolar hypoventilation during sleep and wakefulness In morbid obesity, central fat accumulation imposes a significant load on the respiratory system, with overall effect of marked decrease in lung volumes, in lung and chest wall compliance and increased airway resistance, in all contributing to a higher work of breathing Individuals with OHS have greater degree of central obesity reflected by larger neck circumferences and higher waist: hip ratios than those with eucapnic obesity or OSA Eucapnic obese individuals have a higher rate of oxygen consumption and CO2 production at baseline. This is generally compensated by increased respiratory drive to increase minute ventilation and maintain alveolar ventilation individuals with OHS fail to compensate their respiratory drive in response to the added load created by excess weight, thus permitting a gradual increase in PCO2 OHS is a diagnosis of exclusion that requires evaluation for other potential causes of hypoventilation and hypercapnia such as obstructive or restrictive lung diseases, neuromuscular disease, severe restrictive chest wall disorders, metabolic causes like hypothyroidism and congenital hypoventilation syndromes treatments inc weight loss and CPAP/BIPAP

Answer 6

V/Q mismatch. If we assume that there’s no gas exchange with an area of pneumonia, there is still blood flowing round it (possibly more due to inflammation). Whether that blood comes from the pulmonary artery/ right heart, or the bronchials, it all drains into the left atrium. There it mixes with, and dilutes all the blood that’s passed through the lungs and is oxygenated. So, if the blood passing through the right lung has sats of 100%, and the blood passing through the pneumonia enters the LA with sats of 75% (as if venous), the blood pumped out of the left heart will have sats somewhere between the two, probably in the 80’s if it’s a big pneumonia (interestingly supplemental oxygen might be limited in potential to improve this) On the other hand a pneumonectomy has no blood flowing through it because the PA stump has been tied off. Therefore there’s no blood draining from it into the LA and no shunt

Answer 7

bound to Hb, 2a/2b subunits with porphyrin moity that binds 4O2, adult is type A and foetal type F; pH, pCO2, temp and diphosphoglycerate influence Hb affinity for O2; 1g binds 1.39 ml, 15g per 100ml; PaO2 is amount not bound, but total amount is what keeps us alive and is (1.39x[Hb]x%sat)+(0.003xpO2) 97.5% saturated in arterial blood, 75% in mixed venous blood; sigmoidal shape so Hb largely saturated at pp >8kPa (loading plateau), so fluctuations in barometric pressure have little effect on blood O2 content, and remains same over large range of ventilation rates so can regulate Paco2 without effecting O2 supply; marked fall in saturation <8kPa facilitates unloading modest right shift of curve will result in release of O2, this is how temp, pCO2, pH and DPG work; Bohr effect is exercising muscle is warm with high pCO2 and low pH (lactic acid, extra pCO2 to bicarb/proton) so facilitates greater O2 release; RBCs have high levels of 2,3DPG due to DPG mutase acting on glycolysis intermediate 1,3BPG, 2,3DPG has higher affinity for type A Hb, displacing bound O2 to shift curve to right (RBCs make more 2,3-DPG in chronic aneamia, altitude acclimatisation, heart failure) type F less sensitive and curve shifts to left - important as pO2 in blood to foetus is 4kPa which type a would be 55% saturated at and type f is 75% %saturation measured by pulse oximetry based on changing red/IR light absorption, allowing pulsatile component (arterial blood) to be measured

Answer 8

3 forms: dissolved, bicarbonate, carbamino compounds; 25x more soluble than O2 so dissolved CO2 represents 5-10% of total; most is bicarbonate (90%) due to carbonic acid dissociation, fast in RBCs where CA is and slow in plasma; proteins reversibly bind to amino groups so 5% bound to plasma proteins and those in RBCs, however the R-NHCOOH will dissociate and cause a pH change unless buffered Haldane effect is amount CO2 carried depends on pO2 and Hb%sat as deoxy Hb weaker acid so binds more protons at physiological pH (to His residues) helping maintain gradient for bicarbonate production, also forms more carbamino compounds, so deoxy blood carries more CO2 - and in lungs O2 binds so protons released and less CO2 made into bicarb, meaning more free to be breathed out CO2 down conc gradient from tissue into plasma, some forms carbamino compounds, some converted to bicarbonate, both release protons which are buffered by proteins, thus non bicarbonate bases important in minimising pH change driven by CO2 flux; most dissolved CO2 enters RBCs where rapidly hydrated and degraded by CA, buffered by imidazole groups on Hb which have pKa near 7 making them good buffers; bicarbonate leaves RBC down conc grad with charge movement compensated by Cl (Cl-HCO3 exchanger) so Hamburger's phenomenon/chloride shift where RBCs accumulate Cl and this increased anion content causes RBCs to swell, their membranes are permeable so exchange rapid in capillary beds; pH fall or pCO2 generate proton) inc shifts Hb curve to right so oxy to deoxy so Haldane effect; reverse Haldane in lungs with high pO2 favouring CO2 exit

Answer 9

matching airflow to bloodflow: Va 4l per min and Q 5l per min (whole CO) so overall ratio 0.8 but there is regional variation; Va higher at base due to effect of gravity on Pip/compliance and perfusion better at base due to effect of gravity on Pa, so both increase down lung but bloodflow proportionately greater at base and Va at apex: Q shows 5-fold difference from apex to base, Va 2-fold so Va/Q varies from 0.7 at botom to 3 at top; ratio doesn't change much in lower 2/3 of lung, dramatic change in upper 1/3 due to change in Q ratio important as can have marked effect on gas exchange; increased ratio means inc Pao2 and dec Pco2, actual pulmonary venous concs mix of all ratios based on relative contribution; regional ratios can influence disease eg TB at apex as overventilation relative to Q provides high O2 environment; hypoxic vasoconstriction causes diversion of blood to better ventilated parts of the lung. However, in most physiological states the haemoglobin in these well ventilated alveolar capillaries will already be saturated. This means that red cells will be unable to bind additional oxygen to increase the pO2. As a result, the pO2 level of the blood remains low, which acts as a stimulus to cause hyperventilation. However, as the rest of the lung can still remove CO2, hypercapnia does not occur. In cases of severely limited ventilation, hypercapnia may develop

Answer 10

right to left as in septal defect or in bronchial circulation with admixture as some bronchial venous blood enters pulmonary veins; all individuals thus have some right to left shunt 0f ~1-2% CO; alveolar shunt where blood passes alveoli without gas exchange due to poor diffusion eg oedema, pneumonia or atelectasis, or underventilation (at base of lung); effect of venous admixture is to lower Pao2 to 12kPa, 20% of this is from anatomic shunting and 80% due to low vent/perf ratios at base of lung

Answer 11

often said it's bc hypoxia relied on by these pts to drive ventilation but this not the case in acute decompensations - experis show high drive even when oxer-oxygenated (until slip into coma driven by acidosis) instead it is mix of increased VQ mismatch as higher alveolar pO2 relieves hypoxic vasoconstriction in the less well ventilated parts of the lungs, effectively increasing dead space + the haldane effect with more oxyhaemoglobin so less CO2 bound to Hb

Answer 12

poorly defined collections of neurons with numerous components; dorsal respiratory group associated with inspiration (in medulla) projects to motor neurons innervating inspiratory muscles; ventral respiratory group has inspiratory and expiratory functions; inspiratory cells make repeating bursts of APs that propagate to diaphragm in absence of afferent input, give basic periodicity, pneumotaxic centre can terminate through inhibitory impulses; thus central pattern generator is neural network in DRG/VRG that are bilaterally paired with cross communication to ensure symmetric movements; we know pneumotaxic inhibits as stimulating it activates inspiration and normal rhythm can exist without it; apneustic centre excites medulla groups to prolong inspiration giving regular inspiratory phase; voluntary control possible (singing, talking) but involuntary can take over

Answer 13

swallow a bead and occlude a bronchus: no ventilation, so ventilation perfusion ratio zero and hypoxic constriction redirects bloodflow; airplane loses cabin pressure so lower Pio2 so lower PAo2 giving hypoxia, so breathing mask; restrictive disease minimises elastic work at cost of higher flows/more work against resistance through small, fast breaths; obstructive have long slow breaths to minimise flow/work against resistance, purses lips to hold airways open and breathes at higher lung volumes; bradycardia may accompany hypercapnia/hypoxia

Answer 14

loss of compliance stiffens lungs so more work for normal Va, due to fibrosis or scarring of alveoli (eg filtering mechanisms overwhelmed by carbon (black lung), silicon (silicosis - glass workers), asbestos (asbestosis), cellulose (brown lung - textile workers) - a restrictive lung disease with FEV1 and FVC reduced but FEV1 may reduce less so FEV1/FVC ratio (prop of vital capacity person can expire in one second) normal or even increased in contrast with obstructive pulmonary diseases; increased compliance may occur in eg emphysema (an obstructive pulmonary disease) due to damage to alveoli causing difficulty exhaling as elastic recoil decreased and more work done to exhale; changing compliance may change FRC; chest wall has own pressure-volume curve which interacts with that of the lungs to give total effect; at 0 transmural pressure lungs at less than residual volume and chest wall at 75% of vital capacity, at FRC transmural pressure across chest wall is negative to resist outwards recoil, oposing pressures equal at FRC, total pressure thus zero as total always sum of transmural pressure across each of lungs and chest wall, altering pressure volume curve for lungs or chest wall thus alters system and thus influence FRC, hence altered compliance affecting FRC (decrease together/increase together)

Answer 15

70dynes per cm for air/water interface at 37 degrees c; arises due to non-compensated pull between liquid molecules whose interactions are stronger than the gas can provide; inflation/deflation of lungs in air and saline show saline far more compliant and less hysteresis, and surface tension deduced to account for 2/3 to 3/4 of elastic recoil of lungs; surface tension discovered as oedema foam has air bubbles which are very stable; type ii pneumocytes secrete phospholipid rich surfactant, (90% lipids, 50% of which are DPPC, proteins 10%, half plasma proteins and half contribute to inate immunity by promoting phagocytosis, help improve surfactant rate of disribution with congenital absence leading to acute respiratory distress syndrome) principle component dipalmitoyl phosphatidyl choline (DPPC) with production dependent on precursors (glucose, palmitate, choline) supplied by pulmonary circulation; turnover rate high has each lung expansion has surfactant renewal; surface balance used to study effect of surfactant, test material in saline with adjustable surface area, adding detergent reduces surface tension independent of surface area, lung washings reduce it dependent on surface area and at low surface area the surface tension falls to very low values

Answer 16

surfactant reduces surface tension to increase compliance and decrease the work of breathing, also allows alveoli of different sizes to co-exist as LaPlace's law p = 2D(surface tension)/R so smaller alveoli should collapse into larger ones (atelectasis) as larger pressure developed in smaller ones, but surfactant reduces surface tension more in smaller alveoli (also they're tethered together to keep each other open) DPPC is amphipathic and intermolecular forces oppose attractive forces between surface water dependent on surfactant per unit area (reduction greatest when film compressed); at low lung volumes some DPPC squeezed out of surface layer so upon expansion amount per area is less requiring new surfactant or redistribution of old film, thus hysteresis; in quiet breathing surface area can remain ~constant which impairs surfactant distribution, deep sighs or yawns increase the volume to help spread new surfactant, abdo/thoracic surgery patients may find it painful to breathe deeply leading to poor surfactant distribution and possibly atelectasis foetal lung surfactant production matures at 85-90% gestation period, premature babies often have insufficient surfactant production leading to infant respiratory distress syndrome (laboured breathing as a result of increased compliance), developing atelectasis and pulmonary oedema; surfactant helps keep alveoli dry, inwards collapsing force from surface tension would lower interstitial pressure to draw fluid from capillaries giving oedema, surfactant reduces this; rapidly expanding alveoli expand faster than surfactant reaches surface so surface tension increases, may double in inspiration, to help slow expansion of some alveoli to match slower ones (and reverse for expiration with faster than surfactant can leave surface, tension halving and collapse slowing)

Answer 17

turbulent in large airways at high flow rates as in exercise, laminar (and silent) in small airways, though changing calibre and irregular surfaces means most flow is transitional; poiseuille's law, halving radius causes 16-fold increase in resistance and doubling length only doubles resistance, and viscosity not density influences resistance; however largest resistance/pressure drop is up to 7th generation as large number of small airways arranged in parallel and thus hard to diagnose obstructive pulmonary disease early as change in R tends to start in small airways which make little contribution to total R; reynolds number , flow laminar if <2000, turbulent if >3000 but only really for long straight tubes, with bifurcations in bronchial tree establishing eddies leading to transitional flow; laminar flow prop to pressure and turbulent to sqrt pressure so less laminar flow, more work to generate the same flow

Answer 18

lung volume - as lungs expand radial traction causes airway radius to increase so R drops, and alveoli are dilated helping to pull open the bronchioles bronchial smooth muscle can contract or relax to change airway diameter, symp makes it relax and decreases mucous secretion and parasymp contract and increase mucous secretion, beta2 agonists thus used as asthma treatment to dilate airways; increased Pco2 can induce airway dilatation and decreased airway constriction dynamic - flow-volume curves show flow quickly rises to peak then declines over most of expiration with expiratory flow rate limited by increased resistance due to airway compression by intrathoracic pressure during forced expiration Pip can reach +30cmH2O and PA even greater (due to elastic recoil as well as Pip), PA drops towards mouth but Pip remains same and at equal pressure point they are equal, beyond this point airway could collapse hence cartilage increasing Pip also increases PA so doesn't affect max expiratory flow rate, the difference which can affect it is thus elastic recoil and decreased elastic recoil is main reason why smaller lungs have smaller max flow rate; less elastic recoil in emphysema so EPP shifts distally which can cause airway collapse, stopping flow until pressure rises and airway opens giving wheezing, patients compensate by breathing at higher lung volume where compliance less (so more elastic recoil) and pursing lips so greater pressure drop there

Answer 19

blood is 1/2 weight of lungs, heart in middle so pulmonary arterial pressure at top 11mmHg less than in middle which is llmmHg less than at base; lung has 3 zones based on PA, Pa, Pv: zone 1 PA>Pa>Pv, capillaries collapsed with no bloodflow, doesnt exist in healthy people as Pa should overcome PA unless Pa falls in haemorrhage or PA increased in forced ventilation; zone 2 Pa>PA>Pv, PA supercedes Pv and bloodflow is difference between Pa and PA, initially venous end of capillary occluded by PA, Pa forces capillary open and flow resumes, cycle repeats; zone 3 Pa>Pv>PA so flow determined by normal arteriovenous pressure difference; zone 4 may exist in low lung volumes where Pip can partially collapse vessels due to reduced radial tethering, only at base; exercise increases pulmonary arterial pressure so moves boundaries of each zone upwards, improving perfusion

Answer 20

pressure difference on alveoli/extra-alveolar vessels mediates resistance: pulmonary arts/veins influenced by Pip and capillaries by alveolar volume with tissue network around vessels stopping Pip having an effect; high volume means big alveolus, small capillary, large arteries/veins and vice versa around low volumes; resistance smallest around FRC and increases in reductions to RV and increases to TLC; regional hypoxia causes localised vasoconstriction to divert blood away from area with little change in pulmonary arterial pressure, general hypoxia causes general vasoconstriction (in CF or emphysema, or at altitude) increasing vascular resistance and pressure leading to pulmonary hypertension and oedema; signalling for this unclear with low Po2 possibly acting on smooth muscle of pulmonary vasculature

Answer 21

pH and Pa(gas) are stimuli, and overventilating a patient can stop breathing due to fall in Paco2; bilateral chemoreceptors in medulla at level of cranial nerve roots 8-11 and very superficial, with additional areas in root of cranial nerve XII; integrator of all these signals central but distinct from DRG-VRG, direct local application of acidic/high pCO2 saline increases ventilation, anesthetics and cold solutions decrease; sensors bathed in ECF of brain, composition influenced by CSF and metabolism, with CSF most important, and blood brain barrier regulating entry into CSF as shown by radioactive dye injection BBB impermeable to proton/bicarbonate but CO2 gets in, if bicarbonate supplied and pCO2 increased to maintain pH then no change in ventilation so sensors respond to pH; CO2 lowers the CSF pH and its delivery is facilitated by vasodilation of cerebral vessels in respone to inc Paco2, CSF also has greater change as no protein so reduced buffering capacity; Paco2 may be elevated without inc ventilation in patients with chronic lung disease as bicarbonate actively transported into CSF if pH displaced for long period to buffer and restore to normal levels in CSF

Answer 22

aortic/carotid bodies with type 1 glomus cells , decreased pH/Pao2 inhibits a K channel to depolarise the cell, calcium enters and NT released signalling to medulla to increase ventilation; 20-40% of response to hypercapnia is due to peripheral receptors so central more dominant, peripheral sole response to hypoxaemia; Pao2 below 8kPa gives marked ventilation increase, any increase before this would do little as Hb still 90% saturated; in absence of carotid bodies severe hypoxia can depress CNS activity and reduce ventilation; gases interact with Pao2 below 13kPa stimulating response if Paco2 raised, combined effects greater than sum of individuals stretch receptors in airway smooth muscle discharge in dilation to prolong expiration time, hering-breuer inflation reflex from these receptors along vagus to inhibit DRG-VRG and apneustic centre to prevent overinflation of lungs - animals may use it to control rate/depth of breathing, not humans unless large TV, also projects to cardiac vagal motor neurons to give tachycardia sinus arrhythmia; irritant receptors between airway epithelial cells detect irritants and inflammation to initiate coughing, gasping, prolonged inspiration; unmyelinated C fibre endings (pulmonary if near alveoli, affected by pulmonary circulation, bronchial if affected by bronchial circulation), pulmonary also called j (juxtapulmonary) receptors (though usually call them pulmonary c fibres) stimulated by large inflation, injury (PE/pneumonia), chemical agents, oedema, and cause rapid shallow breathing in eg left heart failure -> responsible in part for tachypnoea and the feeling of dyspnoea

Answer 23

to distinguish between cardiac and resp causes of cyanosis in baby abg taken, breath 100% O2 for 10-15 mins, another ABG taken; PaO2 less than 20kPa suggests r->l shunt ie cardiac cause like tof, if >25-50kPa suggests this unlikely, consider lung pathology, l->r shunt causing pulmonary oedema, or mixing of pulm and systemic circulations danger if dependent on l->r mixing through pda if duct dependent (eg pulm stenosis) as O2 may stimulate duct to close, so PGE1 should be available during the procedure finally dont forget non cardioresp causes of cyanosis inc central resp depression (inc fits), polycythaemia, and methaemoglobinaemia (well baby who is cyanosed, normal hyperoxic test, blood looks brownish even after breathing O2) cyanosis appears when when the level of deoxygenated hemoglobin in the arteries is above 5 g/dL (typically sats of 85%) - > thus cyanosis is rare in anemia and common in polycythemia, but tissue may still be hypoxic; peripheral cyanosis without central may be due to reduced skin bloodflow sec to vasoconstriction (if eg cold)

Answer 24

obstructive: air trapping giving increased residual volume, FRC and TLC; vital capacity is decreased restrictive: vital capacity, FRC, residual volume, TLC, and ERV all decreased so FEV1/FVC, if >0.7 look at FVC and TLC, if these low its restrictive, then TLCO low means fibrosis and normal means neuro/obesity/bone etc; if FVC and TLC normal then normal lung mechanics, if TLCO low means vasc problem like HTN or oedema if FEV1/FVC <0.7 look at FVC, if small then mixed picture, if normal look at TLCO which is low in emphysema and normal in asthma/chronic bronchitis/bronchiectasis

Answer 25

inc pO2 by inc'g fiO2 then PIP, then inspiratory time, then PEEP (risk of ptx and reduced CV function so careful with PIP and PEEP) dec pCO2 by inc rate, inc PIP to raise both inc PEEP; another way to raise pCO2 is to extubate, or if ventilation still necessary increase dead space by using longer endotrach tube

Answer 26

dislodged endotrach tube (listen to lungs, look for chest movement) blocked tube (ditto) ventilator failure (inspect it) ptx (transilluminate +/- CXR) intraventricular h+ (cranial uss)

Answer 27

hypercap: eg OSA, central resp depression, resp neuromusc disease eg GBS, thoracic wall disorders (eg scoliosis), obesity alkalosis: hypervent syndrome, often anxiety but also consider PE, resp stimulant ingestion, and early stages of salicylate overdose

Answer 28

decrease: infiltration/fibrosis, PE, pulm HTN, VQ mismatch (pneumonia, pulm oedema), anaemia increase: polycythaemia, exercise (higher in athletic individuals), pulmonary haemorrhage, may be inc'd in asthma but oft normal

Answer 29

right shift, ie makes Hb release O2 better but take it up worse: increased pCO2/dec'd pH (Bohr effect), inc'd temp, chronic hypoxia, anaemia, inc'd RBC 2,3DPG (production of this increased in chronic hypoxia eg altitude or chronic lung disease), HbS, polycythemia left shift: dec'd pCO2, inc'd pH, dec'd temp, metHb, carboxy Hb, HbF

Answer 30

derivatives of gut tube with signals from mesoderm dictating characteristics: liver mesenchyme makes lung buds develop into hepatic cells, bronchial mesenchyme into bronchial buds, stomach mesenchyme into gastric glands etc; 2 tracheo-oesophageal ridges start to separate oesophagus from lung bud as respiratory divericulum, lung bud then bifurcates into two branches to form paired bronchi and lungs; the ridges fuse to divide oesophagus from trachea/lungs and laryngo-tracheal endoderm lines airways barium swallow can diagnose tracheoesophageal fistula or oesophageal atresia affects 1 in 5000 births due to failure of two componanets to separate; causes severe choking in neonatal baby as fluids eg milk may be aspirated into lungs; gastric contents may enter respiratory system; polyhydramnios (excess amniotic fluid and distension of uterus) frequent complaint as foetus cannot swallow amniotic fluid; 90% of cases proximal oesophagus blind ending, distal part comes off trachea, 4% proximal and distal blind ending, 4% both parts connect into oesophagus and fused, 1% each with proximal part connected and distal blind ending or both parts connecting separately; repaired surgically by closing off fistula and connecting parts of oesophagus FGF10 secreted by mesenchyme guides bronchial branch outgrowth and induces new gene expression in cells at end of bronchial branches; negative feedback with Shh inhibiting Fgf10 expression locally, arresting outgrowth to promote next round of branching; replacing piece of tracheal mesoderm with bronchial causes ectopic lobe of lung to arise directly from trachea lungs mature progressively with glandular period (week 5-16) with brnaching to form bronchi, no respiration, foetus can't survive; some respiration possible towards end of canalicular period where respiratory bronchioles developing, foetuses born towards end of this period (weeks 22-25) may survive if given intensive care; terminal sac week 26-birth with capillaries and primary alveoli developing; alveolar priod from week 36 until 8-12 years old where new alveoli continue to mature postnatally, ~1/6th of your adult alveoli number when born

Answer 31

Exposure is most commonly from suicide attempts using car exhaust, and accidental exposures from incomplete combustion in charcoal burners, faulty heaters, fires, and industrial accidents Carbon monoxide has ~210 times the affinity for haemoglobin than oxygen, giving tissue hypoxia and also triggers endothelial oxidative injury, lipid peroxidation and an inflammatory cascade Normal COHb 0.5% <10% (nil, commonly found in smokers) 10 – 20% (nil or vague nondescript symptoms) 30 – 40% (headache, tachycardia, confusion, weakness, nausea, vomiting, collapse) 50 – 60% (coma, convulsions, Cheyne-Stokes breathing, arrhythmias, ECG changes) 70 – 80% (circulatory and ventilatory failure, cardiac arrest, death) chronic exposure often lower dose for longer time which incs risk of neurological sequelae: can include headache, personality changes, poor concentration, dementia, psychosis, Parkinsonism, ataxia, peripheral neuropathy and hearing loss get ABG (HbCO and lactate up, paO2 normal), ECG give NRBM O2, hyperbaric O2 if acidotic or significant sx; treat coexistent cyanide toxicity if suspected (e.g. house fire) If you are a one-pack-a-day smoker, your carboxyhemoglobin is about 6%, but could be as high as 10%

Answer 32

anastomotic leaks, strictures at site of anastomosis (may need balloon dilatation), GORD, rec cough, bronchitis, pneumonia note in most cases surgical strictures are due to scarring

Answer 33

2 shunts, foramen ovale and ductus arteriosus, achieve pulmonary bypass right and left heart beat in parallel instead of in series, as they do in the adult; right ventricular output higher than left due to the shunts total CO is 4x higher than it is in adult, ductus venosus bypasses liver from umbilical vein to IVC lungs: entirely fluid filled but ready to carry out gas exchange at birth with alveolar dev in last few weeks of birth and continuing postnatally + late in dev surfactant production can insert artificial surfactant into lungs, or give mum high dose of synthetic glucocorticoids 24hrs before premature delivery to stimulate lung maturation from around 10 weeks gestation can detect foetal breathing movements and this provides important mechanostimulation for alevoli dev/surfactant release

Answer 34

fetal breathing movements start at week 10, peak 2-3 weeks before delivery to strengthen resp muscles with absent movements stopping proper lung growth/maturation, happens during REM for 1-4hrs a day fetal lungs secrete Cl rich lung liquid at 330-450ml per day and is 1/3 to 1/2 daily amniotic fluid turn over; at birth LL secretion stops (shortly before delivery catecholamines inhib Cl pump), circulating adr to beta receptors opens lung epithelium Na channels and LL absorbed, mechanisms for absorption also under control of TH and cortisol; latter inc betar and Na pump expression, also makes PMNT up so more NA to adr, and upreg of enzymes doing T4 to T3 30% ll reabsorbed in labour via mouth (ie pressure pushes out of mouth) due to raised intrathoracic pressure during vaginal delivery and over next 12 hours rest lymph to blood to kidneys, diuresis for ~12 hours; c-section prevents some hormone changes and squeezing out of fluid, too much fluid causes transient tachypnea of newborn surfactant mainly DPPC, appears in amniotic fluid after 28 weeks (moved ut by breathing movement with cortisol inducing its production and betaR required for its release; reduce surface tension to increase compliance lungs filled with fluid, pO2/pH fall immediately after birth and pCO2 rises then O2 quickly rises as fetus breathes; for first breath baby grunts against closed glottis to establish FRC by creating high trans-pulmonary pressure with light sound not needed to trigger (blind + death babies still breathe) but generalised arousal due to inc sensory input, cold (c fibres), tying/cutting umbilical cord cause hypoxia and hypercapnia in newborn, activating reticular formation where nuclei controlling resp are

Answer 35

the commonest cause of respiratory distress in the newborn period. It is caused by delayed resorption of fluid in the lungs It is more common following caesarean section; thought due to the lung fluid not being 'squeezed out' during the passage through the birth canal but actually more about lack of stress hormones causing lung liquid resorption Chest x-ray may show hyperinflation of the lungs and fluid in the horizontal fissure. Management observation, supportive care supplementary oxygen may be required to maintain oxygen saturations Transient tachypnoea of the newborn usually settles within 1-2 days

Answer 36

a risk of oxygen therapy, generally paO2 raised above 11-13kPA, 16kPa as a maximum; may make infarcts in MI or stroke worse, injure lungs, or cause retinopathy, or suppress breathing in chronically hypercapnic pts so titrate the O2 being received to avoid hyperoxia; reserve 15L/min non-rebreathe mask for emergency situations

Answer 37

ARDS is pulm oedema not explained by cardiac or fluid overload reasons but inc'd perm of pulm caps; causes inc sepsis, pancreatitis, trauma, pneumonia, aspiration toxic shock syndrome: fever, sunburn like rash, skin peeling, and low blood pressure/shock; due to staph aureus/strep pyogenes; may also see some of d&v, myalgia, AKI, liver inflam, confusion, thrombocytopenia

Answer 38

rr >60/min, nasal flaring, intercostal recession, expiratory grunt, cyanosis, poor feeding if mum is diabetic: transient tachypnoea of newborn due to caesarean sec to macrosomia, resp distress syndrome also more common, as is persistent fetal circulation and polcythemia induced stiffnes of lungs congenital diaphragmatic hernia, esp if mum had no antenatal care; caused by failure to close of foramen of Bochdalek and leads to hypoplastic lungs, worsens when air enters intestine esp if eg bag and mask ventilation; may see scaphoid abdo, reduced breath sounds/chest movement, displaced heart sounds; delayed presentation can give epsodes of breathlessness after feeding and intermittent obstruction (pain/vomiting) intubate, NG tube to deflate intestines, pulm vasodilators for HTN, resus with fluids etc, then refer for surgical correction general diffs: resp distress syndrome (within 4 hrs of birth, esp if premie, asphyxia, hypotherm, acidosis), sepsis/pneumonia (more likely if PROM, maternal pyrexia, culture pos vaginal swabs, neonate may be floppy and acidotic), meconium aspiration syndrome (esp postmature), PTX (vent history, traumatic delivery, spont in 1% of deliveries) phrenic nerve palsy after traumatic birth, congenital heart disease, pulmonary h+ baby developes resp distress +/- cyanosis shortly after birth, intubated and ventilated but doesnt seem to need it, extubated and resp distress again; sx may improve when crying; consider choanal atresia (problem as babies nasal breathers); intubate or magill airway until surgery can be done; sometimes clearing the nares with eg saline can work wonders to calm resp distress if problem is more mucous blocking the choana

Answer 39

consider GORD and tracheo-oesophageal fistula (contrast barium swallow)

Answer 40

DVTs are almost always unilateral. Bilateral DVT is rare and bilateral symptoms are more likely due to an alternative diagnosis such as chronic venous insufficiency or heart failure. DVTs can present with: Calf or leg swelling Dilated superficial veins Tenderness to the calf (particularly over the site of the deep veins) Oedema Colour changes to the leg To examine for leg swelling, measure the circumference of the calf 10cm below the tibial tuberosity. More than 3cm difference between calves is significant. consider that they may have a PE well's score: 2+ do uss, <2 do d dimer if pos then uss; repeat negative ultrasound scans after 6-8 days if a positive D-dimer and the Wells score suggest a DVT is likely treatment dose apixaban or rivaroxaban then doac, warf, or lmwh (pregnancy); note you need to do age adjusted d-dimer as d dimer levels are higher in older people, so conventional threshold for negativity too low ie more false positives 3 months if there is a reversible cause (then review) Beyond 3 months if the cause is unclear, there is recurrent VTE, or there is an irreversible underlying cause such as thrombophilia (often 6 months in practice) 3-6 months in active cancer (then review) When patients have their first VTE without a clear cause, the NICE guidelines from 2020 recommend reviewing the medical history, baseline blood results and physical examination for evidence of cancer. In patients with an unprovoked DVT or PE that are not going to continue anticoagulation (they have finished 3-6 months of treatment and are due to stop), NICE recommends considering testing for: Antiphospholipid syndrome (check antiphospholipid antibodies) Hereditary thrombophilias (if first deg rel has also had dvt or PE)

Answer 41

often from DVT of calf, femoral, or iliac veins (more likely the more proximal the vein); also sometimes from IVC, right side of heart, catheters in subclavian or jugular veins; may be silent, but be aware of oedema or tenderness in leg with erythema and pain on dorsiflexion; compression US to confirm/exclude PE presents in various ways dependent on size: acute massive usually in patient recovering from surgery who suddenly collapses with hypotension, cyanosis, tachypnoea, engorged neck veins major and minor PE will have dyspnoea, hyperventilation and after infarction occurs also pleuritic pain and haemoptysis plus effusion and segmental collapse or consolidation on CXR; chronic small emboli may give pulmonary hypertension and progressive dyspnoea also a single PE can present with a chronic cough, breathless, haemop episodes (1+) etc; additive calf oedema (ie one leg swells then the other) points to this rather than HF do ecg to exclude MI but often normal or signs of right heart strain if PE CXR often normal but may have some signs d dimer good to exclude PE but dont confirm it as clot could be elsewhere CTPA (CT pulmonary angiography) is definitive investigation, can also do V/Q scan with multiple areas of perfusion defects not matched on scans of ventilation defects as would be for eg carcinoma or effusion first suspect: dyspnoea, resp rate >20/min, pleuritic pain, haemoptysis (sudden collapse); score +1 if COPD, pneumonia, pneumothorax unlikely; score +1 if major risk factor for venous thrombosis present (pregnant, travel, surgery, major illness, previous thrombosis, immobility); if 2 then start heparin anticoagulation immediately and do CTPA; if +1 then do d dimer, if positive move to LMWH and CTPA; if zero can still do d dimer but remember it is to exclude and not confirm PE, CTPA is gold standard; note you need to do age adjusted d-dimer as d dimer levels are higher in older people, so conventional threshold for negativity too low ie more false positives note you start LMWH before definitive results come in, can reevaluate later; once confirmed can move to oral warfarin or rivaroxaban thrombolytic therapy with alteplase reserved for patients with massive PE; give if signs point to that and exclude other things like pneumothorax etc, not if active H+, recent surgery, or trauma high flow O2 for acute PE may also be good note other things may embolise to lungs eg fat (fratcure of long bones), amniotic fluid (post partum), air (disconnected central line), tumour (if invades veins), infected vegetation (tricuspid endocarditis), foreign materials (from contaminated drugs by IVDU) be aware that pregnancy plus CTPA has risk of later beast cancer, maybe first do US for DVT and a CXR, if that normal and US -ve then half dose VQ scan (gamma emiting albumin injected, impacts in caps so gamma camera can pick up perfusion; ventilation from radiolabelled xenon) pathophys: Obstruction of the pulmonary arteries creates dead space ventilation as alveolar ventilation exceeds pulmonary capillary blood flow. This contributes to ventilation-perfusion mismatch, with vascular occlusion of the arteries increasing pulmonary vascular resistance. In addition, humoral mediators, such as serotonin and thromboxane, are released from activated platelets and may trigger vasoconstriction in unaffected areas of lung. As the pulmonary artery systolic pressure increases, right ventricular after load increases, leading to right ventricular failure. As the right ventricular failure progresses, impairment in left ventricular filling may develop once diagnosed you can calculate a PESI score which is a risk assessment tool sued to decide whether to ambulate the pt or not looking at obs and past medical history; troponin is also a useful marker as it can help tell you the degree of heart strain

Answer 42

well's score: signs/symptoms of DVT 3 pts, PE most likely diagnosis 3 pts, HR >100 1.5pts immobilisation 3 days or surgery in last 4 weeks 1.5 pts, previous PE 1.5 pts, haemoptysis 1, malignancy 1; 4+ CTPA <4 d dimer note you need to do age adjusted d-dimer as d dimer levels are higher in older people, so conventional threshold for negativity too low ie more false positives causes of pulmonary hypertension can split into 5 groups: Group 1 – Primary pulmonary hypertension (idiopathic or heritable) or connective tissue disease such as systemic lupus erythematous (SLE) Group 2 – Left heart failure usually due to myocardial infarction or systemic hypertension Group 3 – Chronic lung disease such as COPD, ILD Group 4 – Pulmonary vascular disease such as (rec or chronic) pulmonary embolism, arteritis Group 5 – Miscellaneous causes such as sarcoidosis, glycogen storage disease, sickle cell pt may have sob, chest pain, tiredness and palpitations, signs of right heart failure PH has right sided heart strain causing ECG changes such as: Right ventricular hypertrophy seen as larger R waves on the right sided chest leads (V1-3) and S waves on the left sided chest leads (V4-6) Right axis deviation Right bundle branch block CXR: Dilated pulmonary arteries Right ventricular hypertrophy raised NT-proBNP blood test result indicates right ventricular failure Echo can be used to estimate pulmonary artery pressure, and pulm hypertension likely if >20mmHg; after this CT scan can look for more evidence but the echo is the diagnostic test

Answer 43

defined by a mean pulmonary arterial pressure (mPAP) >20 mmHg at rest Radiographic signs of PH include a characteristic configuration of the cardiac silhouette due to right heart (right atrium [RA]/RV) and PA enlargement, sometimes with pruning of the peripheral vessels. In addition, signs of the underlying cause of PH, such as LHD or lung disease, may be found ECG will show right heart strain to subgroup need forced spirometry, body plethysmography, lung diffusion capacity for carbon monoxide (DLCO), and ABG need an echo for heart and valve function, and to estimate PAP, and a CT scan may eb done to look for cause and further evaluate likelihood of PH, VQ scan can be used for chronic multiple PEs if suspect that FBCs, U&Es, LFTs, iron studies, NT-pro BNP, HIV and hepatitis serology, autoimmune screen, look for antiphos syndrome if multiple PEs; liver US in case that is cause or portocaval shunt etc gold standard ix is right heart catheterisation exercise, diuretics/O2 as needed, vaccination against flu/pneumococcus, if vasoreactivity shown during right heart catheterisation start CCB then re-assess after 3-6mo and inc therapy if needed/start if no CCB with eg endothelin R antag + phosphodiesterase inhibitor In patients who are at intermediate–low risk despite receiving ERA/PDE5i therapy, adding selexipag should be considered In patients who are at intermediate–high or high risk while receiving oral therapies, the addition of i.v. epoprostenol or i.v./s.c. treprostinil and referral for LTx evaluation should be considered Lung transplantation remains an important treatment option for patients with PAH refractory to optimized medical therapy

Answer 44

one of the leading causes of pulm hypertension exact pathophys unclear but hyperdynamic state -> raised CO -> inc shear stress on pulmonary vessels giving vasoconstriction and remodelling; portosystemic shunts allow not only iNOS activation but also vasoactive substances like VIP and secretin from the splanchnic circulation into systemic initial echo then right heart cathetrisation will diagnose as part of pulm hypertension workup CCB no as inc hepatic venous gradient and shunting, anticoag no as bleeding risk supplemental O2 yes to prevent pulm vasoconstriction ERAs, prostanoids, and PDE5i yes if mild can have liver transplant, if severe then no due to risks -> can try to control medically then transplant

Answer 45

rare, congenital diffuse lung disease characterized by abnormal blood vessels in the lungs that cause highly elevated pulmonary blood pressure and an inability to effectively oxygenate and remove carbon dioxide from the blood typically presents in newborn babies within hours of birth as rapid and labored breathing, blue-colored lips or skin, quickly leading to respiratory failure and death Most cases of ACD are caused by mutations affecting the gene FOXF1 or its nearby enhancer region; abnormal lung development is characterized by thickened alveolar interstitium, misplacement of pulmonary capillaries away from the alveolar surface, and fewer capillaries overall. This results in poor gas exchange and pulmonary hypertension If an echocardiogram is performed, marked thickening of the right ventricle will be seen, resulting from highly elevated pulmonary blood pressure. ACD is generally resistant to treatment. Babies who have persistent symptoms that are poorly relieved by standard therapies for neonatal pulmonary hypertension is commonly observed in ACD gold standard for ACD diagnosis is by examination of lung tissue under a microscope. The diagnosis is made if the pathologist sees the characteristic findings of ACD: misplaced pulmonary veins adjacent to pulmonary arteries, abnormal alveoli with thickened interstitia and abnormal capillary development. Due to the rapidly progressive course of ACD, this diagnosis is frequently made during autopsy. If ACD is suspected early, examination of tissue from lung biopsy results in the quickest diagnosis Initial treatments attempt to improve low blood oxygenation and high pulmonary blood pressures. Because blood oxygen content is usually very low, babies with ACD are often intubated, sedated, and mechanically ventilated with pure oxygen. Pulmonary vasodilators like sildenafil or inhaled nitric oxide can be used to reduce pulmonary blood pressures; As symptoms worsen, ECMO can be used, but it also offers only brief improvement. There are no effective treatments for severe ACD For infants with atypical ACD who initially had milder symptoms and present at months of life, there can be better response to therapy. There have been reports of infants with ACD surviving to 20 or 36 months without lung transplantation. Bilateral lung transplantation may be the definitive treatment

Answer 46

All criteria must be negative to rule out PE, done to reassure if you doubt it’s PE (but still <2% risk it might be); if you are suspicious of PE move straight to Well’s score Crit: >/=50yo, HR >/=100, O2 sats

Answer 47

cap pressure in parietal pleura plus negative intrapleural pressure and oncotic forces result in fluid entering the pleural cavity, lower pressure of the pulmonary system supplying the visceral pleura favours movement of fluid from pleural space into veins and lymphatics so parietal filtrates and visceral absorbs thus effusion from: raised cap pressure, dec'd plasma oncotic pressure, inc'd cap permeability, obstructed lymphatic drainage patients typically present with dyspnoea, sometimes pleuritic pain, and features of associated diseases like heart failure or carcinoma signs: dec'd expansion on side of effusion, stony dullness, diminished breath sounds, reducec vocal resonance/tactile fremitus, sometimes bronchial breathing inquire about asbestos/TB exposure, smoking, signs of other disease (look for lymphadenopathy, heart failure, breast lumps), certain drugs eg dantrolene

Answer 48

CXR: dense white opacity with concave upper edge (usually, although can be less smooth due to consolidation of tissue); small effusions may just blunt costophrenic angle, so look to see if you can see it if patient supine may only see the fluid as haziness US can be used to guide test tube placement and CT to detect tumours hiding within the fluid and the underlying condition of the lungs and mediastinum thoracocentesis is key: protein >30g/L and LDH >200 units/L suggest fluid is an exudate so investigate for pleural disease; bloodstained suggests malignancy, severe inflam, or pulmonary infarction; pus indicates empyema, milky suggests chylothorax, blood suggests haemothorax; low glucose content suggests infection or connective tissue disorder; high amylase content may mean associated pancreatitis or adenocarcinoma; neutrophils in acute infection and lymphocytes in chronic (esp TB, malignancy); can send to microbiology to identify pathogen CT guided biopsy for areas of pleural thickening, to check for malignancy and TB further investigations based on suspected cause

Answer 49

transudates/exudates; if borderline can compare to serum, exudates have pleural:serum protein ratio >0.5 and LDH >0.6 transudates: cardiac/renal failure, hepatic cirrhosis, hypoproteinaemia (malnutrition or nephrotic syndrome) usually bilateral fluid can sometimes pass from peritoneum into pleural cavity by minor connections, more common on right side; includes fluid from ascites and peritoneal dialysis myxoedema and Meig's syndrome are rarer causes treating heart failure with diuretics incs protein content so may appear to be exudate; malig may obstruct SVC or lymph drainage giving a transudate treat the underlying causes to resolve exudate: often unilateral; malignancy: mets from lung, breast, ovary, GI, lymphoma, also mesothelioma; fluid will be bloodstained with high lymphocyte count, cytology may show malignant cells; milky chylous effusion from malignancy of thoracic duct; infection: parapneumonic effusion from pneumonia, secondary infection of this fluid then gives empyema: high risk of this if pH <7.2 so should drain rapidly, always drain an empyema once one occurs and alteplase plus DNase may help some patients; TB too other disorders: rheumatoid arthritis, SLE, asbestos (can recur, giving diffuse pleural thickening), may complicate a PE, dressler's syndrome subdiaphragmatic disease: pancreatitis, probably by inflam of diaphragm, usually left sided with high amylase content; ascites; spread of infection from subphrenic or intrahepatic abscess

Answer 50

heart failure > pneumonia > malignancy > PE most dont need drain, can be managed as outpatients; thoracocentesis can be done on a day unit admit and chest drain: malignant effusion, complicated parapneumonic effusion, unwell with massive effusion no need for thoracocentesis if patient has obvious cause for transudative effusion unless atypical eg unilat, chest pain, fever, failure to respond to therapy thoracocentesis send to: cytology, microbiology, biochem (for glucose, protein, LDH), process in ABG analyser for pH; consider measuring haematocrit (haemothorax), chylomicrons, amylase, adenosine deaminase (TB) so history, exam, CXR, pleural USS, treat transudative cause if likely, if this fails or exudative cause likely then thoracocentesis; if cause apparent then treat; if not then CT thorax and pleural biopsy; still not sure then reconsider TB and PE but 10-15% effusions will have unknown aetiology so monitor and therapeutic relief

Answer 51

transudative: LVF, atelectasis (often in ITU or after surgery, effusion will be small), cirrhosis, hypoalbuminaemia, PE, nephrotic syndrome, peritoneal dialysis; less common: constrictive pericarditis, hypothyroidism, meig's syndrome (in women with ovarian or other pelvic tumours, resolves once tumour out, comes alongside ascites), malignancy, mitral stenosis, urinothorax (ipsi to obstructed kidney with retroperitoneal urine leak, resolves once obstruction removed, smells like urine with low pH and creatinine > serum creatinine) exudative: malignancy, parapneumonic, TB; less common: complex parapneumonic, PE, RA, SLE, rarely sarcoidosis, other autoimmune dieases, pancreatitis, abscesses, oesophageal rupture, dresslers syndrome, post-CABG surgery, radiotherapy (small, unilat, up to 6 months after surgery), benign asbestos, chylothorax, drug induced rare causes like yellow nail syndrome, familial Mediterranean fever, amyloidosis

Answer 52

spontaneous from bullae rupture, often exist in apex of lung where more -ve pleural space pressure may aggravate minor lesions so most common in tall, thin men; many bulla in COPD so inc'd risk of pneumothorax there; also sec to acute asthma, CF, ILD, TB, nec pneumonia, lung cancer may be iatrogenic or traumatic too, and from +ve pressure endotracheal ventilation on ITU acute pleuritic pain and breathlessness, reduced breath sounds and hyperresonance on side of pneumothorax pleural line and absent lung markings may resolve spontaneously is <2cm so leave alone unless resp distress; high flow O2 in hospital can help as pO2 regulated and pN2 reduced meaning total gas pressure in pleural caps reduced and air from the pneumothorax absorbed more quickly due to higher pressure gradient (except in COPD patients due to risk of high O2 sats) prim ptx: if the rim of air is < 2cm and the patient is not short of breath then discharge should be considered otherwise, aspiration should be attempted if this fails (defined as > 2 cm or still short of breath) then a chest drain should be inserted sec ptx: if the patient is > 50 years old and the rim of air is > 2cm and/or the patient is short of breath then a chest drain should be inserted. otherwise aspiration should be attempted if the rim of air is between 1-2cm. If aspiration fails (i.e. pneumothorax is still greater then 1cm) a chest drain should be inserted. All patients should be admitted for at least 24 hours if the pneumothorax is less the 1cm then the BTS guidelines suggest giving oxygen and admitting for 24 hours

Answer 53

all causes of reg PTX can cause tension If the patient is hemodynamically unstable and clinical suspicion is high for pneumothorax, immediate needle decompression must be performed without delay. Needle decompression is done at the second intercostal space in the midclavicular line above the rib - can also go in triangle of safet if first attempt didn't work (which it won't in 50% of cases), can even be good first attempt if obese or large pec muscles Following needle decompression, a CXR is done, and a chest tube is usually placed tamponade can mimic tension PTX

Answer 54

acute chest pain, retrosternal, radiating to the neck or the back; may see dyspnoea, emesis, neck pain, cough - though these may be related to sx of trigger; subcut emphysema v common may be iatrogenic (eg intubation, endoscopy, central vasc access or procedures), trauma, asthma, COPD, bronchiectasis, ILD, malignancy, sports, child birth, toxic fumes, may be spontaneous (smoking and drugs predispose); tends to happen more in younger ppl as pleura less fibrosed CXR almost always shows, can do CT if still suspicious; USS may pick up in emergency generally benign, admit for 24hrs to observe, give analgesia, consider high flow O2 to speed resolution; treat any underlying cause if severe (usually leak from lung or oesophagus) may get tamponade or airway obstruction - VATS/thoracotomy to relieve in these cases

Answer 55

Air extravasation in other body cavities and spaces can cause pneumomediastinum, pneumoperitoneum, pneumoretroperitoneum, and pneumothorax. The air travels from these areas along pressure gradients between intra-alveolar and perivascular interstitium, spreading to the head, neck, chest, and abdomen by connecting fascial and anatomic planes. Air will preferentially accumulate in subcutaneous areas with the least amount of tension until the pressure increases enough to dissect along other planes, causing extensive subcutaneous spread which can result in respiratory and cardiovascular collapse Iatrogenic causes may occur due to malfunction of the ventilator circuit, inappropriate closure of the pop-off valve, Valsalva maneuvers that increase thoracic pressure, and trauma to the airway inc injury in the trachea or pharynx during traumatic intubation, overinflation of endotracheal tube (ET) cuffs, or increased airway pressure against a closed glottis. Injury to the esophagus during NG placement. Via the cervical soft tissues during tracheotomy, via the chest wall during arthroscopic shoulder surgery, via the extremities as a result of industrial accidents, via bowel or esophageal perforation, or via a tube thoracostomy track or in the course of central venous access procedures, or percutaneous or transbronchial lung biopsy. Also gas generation by local infections. Treat cause and should resolve in <10 days. For mechanically ventilated patients, reducing tidal volume, reducing positive end-expiratory pressure, and minimizing bronchospasm and air trapping can halt the progression and promote reabsorption. In emergency you can put in a subcut drain and later confirm resolution with CT. Extension of air into the neck can cause dysphagia and compression or closure of the airway. Also facial swelling and closing of eyes. On ventilatory support, if unable to reach appropriate tidal volumes, this may lead to high peak pressures and precipitate barotrauma or expanding pneumothorax. If subcutaneous emphysema obstructs the thoracic outlet, it can reduce cardiac preload, and result in poor cerebral perfusion.

Answer 56

contains triglycerides from the lacteals giving it a milky colour (unless not eaten for some time), as well as lymphocytes usually from damage to thoracic duct in root of neck from trauma (inc traumatic delivery), surgery (most often cardiac), and neoplastic disease purulent effusion may look similar drain the chylous effusion continuously (beware fat soluble vit deficiency and lymphocyte deficiency); give medium chain triglycerides (these bypass lacteals and go straight to portal vein); effusion often settles but if persists then surgery necessary

Answer 57

growth spurt (may pop up seemingly overnight), congenital, vit D def/rickets, chronic resp disease eg obstructive causes, turner, noonan, EDS, marfans, morquio syndrome, asphyxiating thoracic dystrophy, ts18, ts21, homocytinuria, osteogenesis imperfecta, scoliosis

Answer 58

thick yellow nails w onycholysis, often accompanied by lymphoedema, also associated with rec pleural effusions, bronchiectasis, chronic bronchitis, and sinus infections

Answer 59

svco (may be raised too high to see), tension ptx, pulm HTN, large PE

Answer 60

small cell carcinoma is 15%, SCC is 40%, ACC 30%, large cell carcinoma another 15% those from the bronchial tree tend to present earlier with chest symptoms (cough, haemoptysis, dyspnoea, wheeze) whereas peripheral ones may grow undetected for some time until general symptoms (weight loss, weakness, fever, clubbing) or mets get noticed; sometimes SVC obstruction or dysphagia CXR is useful diagnostic tool; SCC often a cavitating mass; central tumours may obstruct a bronchus giving lobar collapse; also may see effusion and hilar involvement; CT scan after CXR for more detail and then bronchoscopy for biopsy of central tumours, sputum cytology diagnostic in 40% of cases; radiological guidance with percutaneous biopsy of peripheral tumours may be good; biopsy or sputum used to identify type of cancer based on histology which then determines the treatment route SCC usually central, ACC usually peripheral, LCC can be both SCC most strongly linked to smoking, ACC most common type seen in non-smokers SCC from epithelial cells, ACC from bronchial mucosa or alveoli surface, mucous making cells Small cell grows and spreads quickly so treated with chemo small cell also usually in larger airways and has neurosecretory/endocrine symptoms

Answer 61

Only use sputum cytology for investigation in people with suspected lung cancer who have centrally placed nodules or masses and who decline or cannot tolerate bronchoscopy Offer people with known or suspected lung cancer a contrast-enhanced chest CT scan to further the diagnosis and stage the disease. Include the liver, adrenals and lower neck in the scan Ensure that all people with lung cancer who could potentially have treatment with curative intent are offered positron-emission tomography CT (PET‑CT) before treatment Offer endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) for biopsy of paratracheal and peri-bronchial intra-parenchymal lung lesions 80% of lung cancers occur in smokers or those who stopped recently cigar and pipe smoke incs risk but not as much as cigarettes; second hand smoke is not safe asbestos latency for lung cancer is 10-30 years cancers in non-smokers more likely to have EGFR mutations and be adenocarcinomas cough, chest pain, weight loss, dyspnoea are most common presenting symptoms pain as tumour extends into mediatinum, pleura, or chest wall; haempotysis from H+; pnuemonia, abscess, or airway collapse from obstruction; effusion as spreads into pleura; hoarseness if effects rec laryngeal nerve; dysphagia, diaphragm paralysis, SVC syndrome, horner syndrome, pericarditis/tamponade small cell carcinomas may produce ACTH or ADH (cushings from former, hyponatraemia from latter), SCCs may produce PTH giving hypercalcaemia known for lung cancers to make calcitonin (hypocalcaemia), gonadotropins (gynaecomastia) lung also most common site for tumour mets often secondary involvement from lung and breast tumour mets; ovarian carcinoma another that likes to spread here chest pain, dyspnoea, and recurrent pleural effusions may be due to mesothelioma; concurrent pulmonary asbestosis only in 20% of those with mesothelioma

Answer 62

send pleural fluid for biochem, microbiol, cytology, flow cytometry lateral view on CXR can pick up smaller effusions empyema contained in septations, may look like chest wall mass rather than smooth effusion; split pleura sign on CT pleural thickening: non-neoplastic: fibrinous pleuritis due to inflam from infection, infarction, CTD neoplastic - mets most common and can be from any organ but most often lungs or breast (histology will show pseudoglandular appearance eg adenocarcinoma origin often) prim neoplasm - mesothelioma, often asbestos with 20-40yr latency but can be from eg radiotherapy; will have bloody pleural effusion, chest pain, dyspnoea; histology needed to show its this (will look epitheloid or sarcomatoid) rather than a met in lung cancer, lobectomy has best outcomes, but if poor lung function something that spares more lung eg wedge resection or segmentectomy may be preferred; adjuvant chemo, follow up for at least 5 years; if not fit for operation radical radiotherapy another option molecular testing on biopsies allows some immunomodulatory adjuvants to chemo talc pleurodesis for malig pleural effusion

Answer 63

Bronchoscopy if the tumour is central - this can be endobronchial ultrasound guided scan (EBUS) Thoracoscopy or percutaneous fine needle aspiration (FNA) for peripheral tumours Effusion aspiration and cytology; this after CT scan, CXR; Staging whole body FDG-PET and other integrated PET scans brain CT or MRI on biopsy: hyperchromatic nuclei, pleomorphism, mitotic figures, high N:C ratio, disorganised architecture SCC: maliganant squamous cells with keratinization; adenocarcinoma: large glandular structures filled with mucin LCC: large, poorly differentiated cells without squamous or glandular features

Answer 64

malignant infiltration and inflammation of lymphatic vessels secondary to the metastatic spread of malignancy from a primary site Unexplained progressive dyspnea, which evolves over days to weeks, is the commonest presentation in almost 60 % of patients. Although a history of underlying malignancy is noted in most cases, lymphangitic carcinomatosis could rarely be the initial presentation of an occult malignancy. Pleuritic chest pain will be present if the neoplasm obstructs the subpleural lymphatics. Cough, hemoptysis, weight loss, and lethargy are usually uncommon attempts at definitive therapy involve consideration for surgical resection/chemotherapy /radiotherapy after a definite diagnosis & staging of the underlying tumor is made; some tumours will respond to chemotherapy nearest differential diagnosis to pulmonary lymphangitic carcinomatosis is pulmonary tumor embolism. Signs and symptoms of pulmonary hypertension and cor pulmonale are much more common in the latter. Although clinical and radiological overlaps are common, both conditions represent end-stage malignancy also consider PE, CAP - esp atypical, PCP, heart failure, ILD prognosis usually <6mo but occasionally longer if mx options available

Answer 65

malignancy Lymphoma - NHL/Hodgkins Leukemia Lung cancer Mediastinal Germ cell tumour Thymus: Thymoma, Thymic carcinoma Neuroblastoma Thyroid cancer Vascular Thoracic aortic aneurysm Aortic dissection Sarcoidosis Infection Pericardial/bronchogenic/oesophageal cyst malignancies here can give SVCO

Answer 66

large mediastinal mass -> HL, NHL, ALL/LBL, lung cancer, thymoma, germ cell tumour CXR!! if rec laryngeal nerve could maybe possibly slightly be involved lymphocytosis - young pt most oft viral (EBV etc), old pt (more likely haem onc) coeliac disease -> EALT

Answer 67

flushing (often earliest symptom) diarrhoea bronchospasm hypotension right heart valvular stenosis (left heart can be affected in bronchial carcinoid) urinary 5-HIAA plasma chromogranin give octreotide, manage tumour

Answer 68

airway inflam and inc'd responsiveness resulting in obstruction dynamic and heterogeneous asthma has a genetic component and may run in families but families share environments too; atopy is heritable tendency to make lots of IgE in response to small amounts of antigen moving from poorer to richer area (eg tokelau to new zealand, east to west germany) seems to be associated with inc incidence of asthma (so environmental cause) occupational asthma due to many different compounds in the workplace - will get better at weekends or when go home in evening inhaling antigen often gives early astmatic reaction peaking after 20mins and a late one dev'ing 6-12hrs later

Answer 69

wheeze, dyspnoea, cough, chest tightness may occur first at any age, be episodic or persistent may only get symptoms during infection or exposure to certain allergens symptoms may also be chronic usually diurnal with PEF worse in morning (morning dips) and cough/wheeze sometimes waking patient in early hours: in asthmatics seek info on nocturnal wakening; also ask about in treatment reviews as shows poor control and patients dont always think to mention it sometimes cough can be the only symptom (little or no wheeze or dyspnoea) and this can delay getting the diagnosis from home environment (passive) smoking and allergens (esp dust and cat dander) may perpetuate symptoms occupational if symptoms improve at weekends or on holiday; are symptoms worse on returning to work, esp on evening afterwards any trigger factors: exercise, cold air (outdoors?), feather pillows, allergens, drugs (b blockers, aspirin), seasonal factors, resp infections; reversibility can be assessed by asking about response to bronchodilators and steroids (and help in old people erroneously diagnosed with COPD) general signs: diffuse bilat wheeze, prolonged expiratory phase, lower costal margin paradox; sometimes no signs between episodes ask about allergies and eczema (inc in past but not now) chronic severe childhood asthma can cause ribcage deformity (harrison's sulcii) when diagnosing ask first could this patient's symptoms be caused by asthma (so eg child with recurrent cough may not have recurrent chest infections but actually asthma) but use term like possible asthma until confirmed through investigations etc ie ask does this patient really have asthma? then take it further: could it be occupational? is there additional lung disease like bronchiectasis or allergic bronchopulmonary aspergillosis?

Answer 70

recognise symptoms, establish evidence of airway obstruction, assess variability/reversibility and provocability of obstruction, monitor progress and review diagnosis, consider additional diagnoses and exclude others (CF, carcinoma, inhaled foreign body, COPD) reversibility: spirometry, then salbutamol, then spirometry again 15-20mins later or trial course of steroids and see if FEV1 improves AND/OR variability: diary of peak expiratory flow rate looking for 20%+ variability between highest and lowest recordings, and possibly morning dips TLC and RV often up but TLco often normal and Kco elevated post exercise fall in FEV1 or peak flow (by 20%+) suggests asthma (most useful if normal peak flow or spirometry in clinic limits use of the other tests) skin prick test to identify atopy CXR essential in older patients who have smoked to exclude carcinoma or in children if features to suggest CF or bronchiectasis bronchoscopy sometimes to investigate vocal cord dysfunction, inhaled foreign bodies, rarer causes of obstruction like some tumours exhaled NO levels inc'd in asthma patients (marker of airway inflam tho so not specific) Patients >= 17 years patients should be asked if their symptoms are better on days away from work/during holidays. If so, patients should be referred to a specialist as possible occupational asthma all patients should have spirometry with a bronchodilator reversibility (BDR) test all patients should have a FeNO test Patients 5-16 years all patients should have spirometry with a bronchodilator reversibility (BDR) test a FeNO test should be requested if there is normal spirometry or obstructive spirometry with a negative bronchodilator reversibility (BDR) test Patients < 5 years - diagnosis should be made on clinical judgement Reversibility testing in adults, a positive test is indicated by an improvement in FEV1 of 12% or more and increase in volume of 200 ml or more in children, a positive test is indicated by an improvement in FEV1 of 12% or more

Answer 71

any patient with asthma may dev, some more prone than others patients must know how to recognise and respond to acute attack, and ideally so should their family/friends signs are any one of: PEF 33-50%, resp rate >25 per min, heart rate >110bpm, inability to complete sentences in one breath is then life threatening if: altered consciousness, hypotension, exhaustion, arrhythmia, cyanosis, poor resp effort, silent chest, PEF <33%, O2 sats <92%, paO2 <8kPa near fatal if: paCO2 raised or need for mechanical ventilation immediate management: highest O2 conc available should be given, aim for sats >94% high dose nebulised beta 2 eg 5mg salbutamol (2.5 if <5yo), repeat after 20-30mins if condition not improving or back to back (5x6min nebs) if life threatening also if mod/severe then nebulised ipratropium 250mcg every 20-30 mins for first 2 hours then every 4-6 hours if no nebuliser then multiple does from inhaler with spacer device high dose systemic steroid prednisolone oral 40mg (20 if <5yo) or hydrocortisone iv 100mg (in adults, in kids do 4mg/kg with 100mv max) 6-hourly if no or limited response then IV Magnesium Sulphate 10% 40 mg/kg (max 2 gram) over 20 minutes, risk of hypotension, aim for serum Mg 1.5-2.5mmol/L; can be first line if known not to respond to first line mx if severe and not responding to initiall nebs then consider iv salbutamol 250microg (or in kids 15mcg/kg up to 250 max); repeated salbutamol may give tachycardia, lactic acidosis (causing tachypnoea) Consider a continuous intravenous infusion of salbutamol for severe refractory asthma. 1-2micrograms/kg/min with doses up to 5microgram/kg/min on PICU. Requires ECG monitoring IV Aminophylline loading dose 5 mg/kg over 20 minutes; followed by infusion: 2 years – 12 years (1 mg/kg/hr); > 12 years (500 – 700 micrograms/kg/hr) - no need to load if already taking orally intubation/ventilation: Persistent hypoxia +/- rising hypercapnia despite maximal medical treatment, depressed consciousness, resp arrest; risk of increasing bronchospasm, hypotension (have fluid boluses pre-prepared) and barotrauma; Use pressure controlled mode: high pressures may be needed – limit PIP < 35; start with PEEP 5 – 7 (higher PEEP may be required)  Consider slow rate 5 - 15 breaths per minute. Adjust to ensure adequate emptying  Inspiratory:Expiratory ratio of at least 1:2. Adjust to ensure adequate emptying  May need to allow permissive hypercapnia order ABG, U&Es, CXR, ecg (monitoring if IV salbutamol infusion) afterwards check patient is monitoring PEF, check their inhaler technique, identify any precipitating causes; follow up with GP within 2 days of discharge and a specialist within 1mo; can discharge when patient stable on therapy they could do at home

Answer 72

status asthmaticus and near-fatal asthma basically mean same thing, not responding to good first line medical therapy with T2RF developing asthma involves inflammation and edema of the bronchial mucosa, increased mucus production with airway plugging, and bronchospasm; as airway obstruction progresses, expiration becomes active and inspiration starts before termination of the previous expiration, resulting in air trapping and hyperinflation. Areas of obstruction and premature airway closure cause ventilation/perfusion mismatch -> hypoxemia Dynamic hyperinflation with progressive increased lung volumes stretches the pulmonary vasculature, increasing pulmonary vascular resistance and right ventricular after-load. Pulmonary vasoconstriction, secondary to hypoxia and acidosis, further contributes to the increase in right ventricular afterload, high negative pulmonary pressures generated during inspiration in spontaneously breathing patients causes an increased left ventricular afterload; CO decreases, with exagerrated fall in systole which may causes pulsus paradoxus (sys drop >10mmHg), negative intrapleural pressure also favours dev of pulmonary oedema if life no or poor response to 15lpm O2 and 3x salbut + 3x ipra nebs (20 min between each of the same type ie diff neb every 10 mins) and IV/PO steroids, then bolus salbutamol IV + MgSO4 IV while nebs continue every 20 mins, if IV bolus of salbutamol has effect then stop nebs and start IV salbutamol infusion (look for tachycardia, hyperglycemia, hypokalemia, lactic acidosis -> actually increasing tachypnoea by itself; also needs continuous cardiac monitoring so might be saved for PICU); if no infusion following salbutamol bolus then aminophylline bolus followed by infusion (some trusts will prefer to do aminophylline first line and salbutamol second, they have equal efficacy so will just be what ppl are more familiar using; also if pt on regular aminophylline or theophylline then no bolus, go straight for infusion -> but do check serum level before starting) if worsening despite above, or pH <7.2, hypotensive, sats <90% on 15lpm despite max medical therapy or tiring/dropping GCS or becoming more agitated then intubate and ventilate -> high risk, esp of provoking hypotension, bronchospasm, or barotrauma; ketamine preferred over propofol due to hypotension risk; high pressures may be needed -> better for higher PEEP than PIP past 35, slow rate and adjust I:E ratio to ensure adequate emptying, permissive hypercapnia is okay note no role for CXR normally, do only if suspecting PTX or a foreign body or mediastinal mass; decreased air entry is common and due to mucous plugging, not consolidation from pneumonia

Answer 73

chronic bronchitis: hypersecretory disorder (productive cough most days for 3+ months of 2+ years) with no other cause for chronic cough (TB, bronchiectasis) emphysema: dilation of airspaces distal to terminal bronchioles inc destruction of their walls, reducing gas transfer; may be centriacinar or panacinar (former copd/coal, latter a1at) obstruction results from destruction of alveoli (so loss of elastic recoil and outward traction on small airways so they collapse on expiration), airway inflam, and accumulation of secretions from chronic bronchitis smoke from indoor cooking in poor countries and smoking in rich countries is big causative factor dyspnoea which is only partially responsive and is progressive gradually over years due to sedentary lives many people dont notice progressive breathlessness until much of the lung function has been lost wheeze is not as prominent as asthma cough and sputum from bronchitis, with infective exacerbations giving purulent sputum and inc'd cough, and possibly bronchopneumonia: mucociliary defence mechanism is lessened spectrum from pink puffers (resp drive preserved and pCO2 maintained), blue bloaters (resp drive poor and easily drift into hypoxia, hypercapnia, right heart failure) spirometry is key for diagnosing by looking for obstruction (FEV1/FVC <0.7) reversibility testing can be good to identify that condition is actually undiagnosed asthma (and the two conditions can coexist) TLC and RV often inc'd due to hyperinflation and tranfer factor for CO and transfer coefficient down CXR can show hyperinflated lungs, exclude diagnoses like cancer, and show complications like pneumothorax and bronchopneumonia bullae can be seen on CXR or in high resolution CT scans hypoxia within the lungs can lead to pulmonary hypertension giving right ventricular hypertrophy and tricuspid regurg, ultimately cor pulmonale severe COPD gives cachexia due to reduced appetite, incd resp effort, and circulating inflam mediators such inflam overspill may also inc risk of osteoporosis, diabetes, heart failure depression and anxiety in up to 50% of COPD patients

Answer 74

grade breathlessness with MMRC score: (0 if SOB with strenuous exercise only, 1 if with hurrying or up gentle hill; 2 if walks slower than ppl of same age due to SOB; 3 if have to stop after 100 yards or a few mins; 4 if too SOB to leave house or eg SOB while dressing) smoking cessation is most important thing, and if done soon enough then can even get rate of FEV1 decline returning to that of a normal ageing person; pharmacotherapy exists to help with this SABA are good first line treatments for short term relief of episodes of breathlessness LAMAs (tiotropium/spiriva) and LABAs (salmeterol) are reasonable for maintenance therapy if chronic and stable COPD; see if asthma/atopy history, eosinophilia, variation of 400ml+ in FEV1 or diurnal variation 20%+ in peak flow, then LABA + ICS (later add LAMA -> all 3 together in trelegy), otherwise LABA + LAMA (called elipta) mucolytics may improve cough symptoms, and CBT should be considered for mental health rehabilitation therapy to stop progressive decline: exercise training, breathing control techniques, dietary advice (lose weight if obese, maintain weight if not) COPD and chronic hypoxia have mortality of 50% within 3 years due to cor pulmonale, survival improved by O2 therapy for 15hrs a day+: doesnt affect progression of COPD but relieves pulmonary vasoconstriction to alleviate afterload on the right heart preventing premature death from cor pulmonale; indicated if pO2 persistently <7.3kPa, measured at 2 diff times 3 weeks apart at stable phases (so not measuring hypoxia during an exacerbation)

Answer 75

CXR, oximetry, ABG, FBC, U&Es, ecg, sputum culture (as infection often causes the exacerbation) nebulised salbutamol 5mg and ipratropium 250mcg - no ipratropium nebs if on inhaler with LAMA in -> continue these inhalers instead, have better efficacy (you should always continue the maintenance inhalers); oral prednisolone for 5 days starting with 40mg -> no need to slow wean if only on for 5 days; antibiotics (eg co-amox +/- doxy), carbocisteine +/- saline nebs, oxygen (sats 88-92% if retainer or mod-severe COPD otherwise if mild and normally no exacerbations >94%) ventilation if t2rf meeting criteria

Answer 76

antigen presented to Th2 ultimately leads to IgE production, reexposure activates mast cells which release histamine, cytokines, PGs, leukotrienes, tryptases (these plus inflam damage the lungs); immediate phase within 30 mins is histamine mediated and by direct vagal affs (ie not t cell mediated) and get obstruction and wheeze; 6 hrs later get neutrophils, eosinophils, Th2 and remodelling giving smooth muscle and epithelial proliferation lasting for up to 24 hrs mucus plugs in bronchi, eosinophil rich sputum, oedema, smooth muscle and goblet cell hyperplasia, raised mast cell numbers, and thickened BM Moderate – PEFR more than 50–75% best or predicted (at least 50% best or predicted in children) and normal speech, with no features of acute severe or life-threatening asthma. Acute severe – PEFR 33–50% best or predicted, (less than 50% best or predicted in children) or respiratory rate of at least 25/min in people over the age of 12 years, 30/min in children between the ages of 5 and 12 years, and 40/min in children between 2 and 5 years old, or pulse rate of at least 110/min in people over the age of 12 years, 125/min in children between the ages of 5 and 12 years, and 140/min in children between 2 and 5 years old, or inability to complete sentences in one breath, or accessory muscle use, or inability to feed (infants), with oxygen saturation of at least 92%. Life-threatening – PEFR less than 33% best or predicted, or oxygen saturation of less than 92%, or altered consciousness, or exhaustion, or cardiac arrhythmia, or hypotension, or cyanosis, or poor respiratory effort, or silent chest, or confusion. not all asthma is allergic: pollutants, microbes, smoking etc activates macros and NKTs and signal innate lymphoid ILC2 cells which recruit eosinophils and trigger remodelling

Answer 77

Newly-diagnosed asthma Short-acting beta agonist (SABA) 2 Not controlled on previous step OR Newly-diagnosed asthma with symptoms >= 3 / week or night-time waking SABA + low-dose inhaled corticosteroid (ICS) 3 SABA + low-dose ICS + leukotriene receptor antagonist (LTRA) 4 SABA + low-dose ICS + long-acting beta agonist (LABA) Continue LTRA depending on patient's response to LTRA 5 SABA +/- LTRA Switch ICS/LABA for a maintenance and reliever therapy (MART), that includes a low-dose ICS 6 SABA +/- LTRA + medium-dose ICS MART OR consider changing back to a fixed-dose of a moderate-dose ICS and a separate LABA 7 SABA +/- LTRA + one of the following options: increase ICS to high-dose (only as part of a fixed-dose regime, not as a MART) a trial of an additional drug (for example, a long-acting muscarinic receptor antagonist or theophylline) seeking advice from a healthcare professional with expertise in asthma But if pt asthma well controlled dont need to change just to fit latest guidelines! Children managed similarly to adult except paeds steroid doses, and if <5yo and LTRA doesnt work refer to paeds asthma specialist, also at stage 2 do moderate dose of ics and trial for 8 weeks and if sx resolved go on to low dose and follow as above up to LTRA

Answer 78

may be unrelated but consider: primary pulmonary haemosiderosis (and a similar condition associated with cow's milk intolerance) pph may be associated with goodpasture syndrome and have same antibodies), the pt may also often have haemoptysis and patchy shadowing on CXR due to alveolar infiltrates; BAL may show haemosiderin filled macros; treat with cows milk protein free diet if cow's milk intolerance linked (and note this version, heiner syndrome, may have rec bronchopneumonia in history), and if not then various specialist treatments with eg steroids (pph can be idiopathic or linked to a couple of kinds of autoantibodies inc antiGBM)

Answer 79

asthma (esp dustmite related), GORD, postnasal drip, sinusitis, inhaled foreign body, CF, immunodef

Answer 80

Presenting before 3 years of age No atopic history Only occurs during viral infections coryzal symptoms 1-2 days before note: asthma tests are usually not accurate before 5 years old, so before that tend to go for one form of VIW

Answer 81

Wheeze is extremely common in pre-school children, with an estimated 25% of children having an episode of wheeze before 18 months. Viral-induced wheeze is now one of the most common diagnoses made on paediatric wards. There is however ongoing debate regarding the classification of wheeze in this age group and the most appropriate management. Over recent years, led by the European Respiratory Society Task Force, the favoured classification for pre-school wheeze is to divide children into one of two groups; episodic viral wheeze: only wheezes when has a viral upper respiratory tract infection (URTI) and is symptom free inbetween episodes multiple trigger wheeze: as well as viral URTIs, other factors appear to trigger the wheeze such as exercise, allergens and cigarette smoke Episodic viral wheeze is not associated with an increased risk of asthma in later life although a proportion of children with multiple trigger wheeze will develop asthma. Management Parents who are smokers should be strongly encouraged to stop. Episodic viral wheeze treatment is symptomatic only first-line is treatment with short acting beta 2 agonists (e.g. salbutamol) or anticholinergic via a spacer next step is intermittent leukotriene receptor antagonist (montelukast), intermittent inhaled corticosteroids, or both there is now thought to be little role for oral prednisolone in children who do not require hospital treatment in hospital can be managed as acute asthma inc mag sulf, aminophylline etc; mx by aiming to get from nebulised salb to inhalers (when no longer needing supplemental O2) and then stretching until not needing more than 4-hourly when can discharge can send them home with salb inhaler to use prn and seek med attention if need to use more often than 4 hourly Multiple trigger wheeze trial of either inhaled corticosteroids, typically for 4-8 weeks

Answer 82

Mild if: Able to talk and feed normally SpO2 ≥94% Respiratory rate <40/min Mod/severe if: Too breathless to talk or feed Hypoxia Respiratory rate >40/min if mild: Trial of bronchodilators via spacer if mod/sev: Oxygen (aim SpO2 94-98%) Trial of bronchodilators via spacer with nasal cannula O2 or nebulised with O2 Salbutamol 2.5mg and Ipratropium Bromide 250mcg if good response: stretch to 4 hourly then home with spacer, training, smoking cessation advice to parents if poor response: Consider VT 1-2L/kg (can start at 0.5L/kg and titrate up in older children) Trial of back-to-back bronchodilators Consider IV MgSO4 40mg/kg given over 30mins - can repeat after 1-2hrs but check serum Ca/Mg first; while giving any dose need ECG and BP monitoring Systemic corticosteroids (PO Prednisolone or IV Hydrocortisone) for: Hypoxia Severe symptoms Atopy or ‘suspected asthma’ Antibiotics for bacterial infection Escalate to Consultant, Anaesthetics, ICU/retrieval service CXR for alternative cause or signs of infection Bloods & blood gas (lactate, glucose, K+) Fluid balance, IV fluids if episodic wheeze long term mx is home with inhaler + spacer to use during episodes, come in if >4hrly use needed if multiple trigger wheeze (or had severe episode or eosinophilia) then trial of ICS (Beclomethasone 100-200mcg BD) for 6-12weeks, continuing at lowest effective dose and avoid/treat triggers

Answer 83

note - acute is quite often viral urti think asthma (night, wheeze, atopy), copd (smoking history, sputum), airway reactivity after acute bronchitis (recent acute cough w sputum), bronchiectasis (daily purulent sputum, pneumonia/whooping cough in childhood, rec haemoptysis) lung cancer (pneumonia failing to resolve in 4-6 weeks, persistent, haemoptysis) rhinitis + PND (chronic sneezing, nasal blockage/discharge, feel it dripping down back of throat) gord (heartburn, acid reflux after eating or when lying down, nocturnal element) drugs (ACEi) ild (fine insp crackles, persistent dry cough) idiopathic (long history with no signs and neg ix - dx of exclusion)

Answer 84

progressive dyspnoea, dry cough, lung crackles, diffuse infiltrates and small lungs on CXR, may get clubbing LFTs will show reduced TLC and VC (restrictive disease) with normal FEV1/VC ratio, impaired gas diffusion (transfer factor) if possible, remove exposure to provoking antigen or at least reduce it eg mask, loft coat and hat; oral steroids to suppress any inflam component esp in acute episodes, stop taking the drug causing it

Answer 85

reticular opacities, honeycombing, ground-glass opacification, consolidation, traction bronchiectasis, and cysts can all show and the pattern of these can be assigned to a particular kind of appearance/cause upper lobe CHARTS coal workers’ pneumoconiosis histiocytosis ankylosing spondylitis allergic bronchopulmonary aspergillosis radiation tuberculosis silicosis/sarcoidosis lower lobe DRASC drugs rheumatoid arthritis asbestosis scleroderma cryptogenic fibrosing alveolitis spirometry, high res CT urine dipstick, full differential blood cell count, serum urea, electrolytes and creatinine, and liver function tests BAL should be considered in all patients with suspected infection, malignancy and some rare ILDs - not for IPF biopsy may be performed IPF Prednisolone (tapering from 0.5 mg/kg/day to 10–20 mg/day) with azathioprine (2 mg/kg, maximum150 mg/day) and N-acetylcysteine (NAC, 600 mg three times a day) Referral to a transplant centre should be made if the disease is advanced (TLCO <40% predicted) or progressive (⩾10% decline in FVC or ⩾15% decline in FVC during 6 months of follow-up), if <65yo and no sig co-morbs; often will need palliative care COP usually responds to corticosteroid therapy but the optimum dose and length of treatment is not known. Initial doses of 0.75–1 mg/kg, weaning over 6–12 months, are reasonable CTD-ILD initial treatment for ILD is oral prednisolone at an initial dose of 0.5–1 mg/kg with the aim of tapering to a maintenance dose of 10 mg/day or less, often in association with an immunosuppressive agent (usually oral or intravenous cyclophosphamide or oral azathioprine); in systemic sclerosis avoid high dose steroid as risk of renal crisis Sarcoidosis Oral corticosteroids are the first line of therapy in patients with progressive disease determined by radiology or on lung function, significant symptoms or extrapulmonary disease; methotrexate if sterodis not working; transplant if end stage

Answer 86

CT thorax vital for this as well described patterns eg UIP, OP, NSIP etc; can also let you spot coexistant problems in emphysema youd see: hypertransradiancy and hyperinflation, bulla (which can lead to rec PTX) a reticular pattern (small opacities resembling a net when seen together) represents ild usually; these opacities can inc septal thickening cyst walls from honeycombing etc; will also get tractional airway dilation and ground-glass opacity if consensus not reached then can do BAL or (more useful) lung biopsy with video assisted thoracoscopic surgery UIP is histo counterpart of ipf (clinical diagnosis), with NSIP often associated with connective tissue disease but sometimes idiopathic if asbestos related will see needle like asbestos bodies on BAL

Answer 87

natural history of pulmonary fibrosis is that it is a chronic, irreversible condition with a poor prognosis (most dead <5yrs). It is primarily an issue of diffusion (thickened alveolar capillary barrier due to fibrosis). CO2 is 20x more soluble than O2 (the entire reason Hb exists is that O2 doesn’t dissolve very well in blood). If your lungs are so cooked that you can’t effectively diffuse out your CO2 anymore, you probably need morphine and a side room, not a tight fitting mask making you feel like you’re suffocating/have your head out the car window. They would lose muscle mass, lungs will be damaged by the high pressures. You would never get them off NIV or a ventilator, they’d die on it. An “acute exacerbation” of IPF isn’t an acute exacerbation, it’s actually accelerated progression, usually caused by infection or aspiration Exception is generally only as bridge to transplant, but you can always discuss anyway

Answer 88

type 3 and 4 hypersens prev sensitisation then re-exposure gives fever, cough, dyspnoea usually 8-12 hours after contact; may have crackles but wheeze unusual, resolves after a few days chronic form after several acute attacks giving pulm fibrosis avoid exposure, steroids may help in early disease

Answer 89

a heterogeneous group of very rare lung diseases - most general practitioners and paediatricians will face none or one of these patients in their whole career and even paediatric pulmonologists may manage only a few cases of chILD role of the general paediatrician facing a chILD suspicion is to question about the family and medical history and to initiate investigations (routine laboratory tests and chest radiography) before referring the patient rapidly to a specialised centre diagnosis of chILD has been defined as the presence of at least three of the following criteria: 1) respiratory symptoms, 2) clinical signs of respiratory insufficiency, 3) hypoxaemia or low pulsed oxygen saturation and 4) diffuse parenchymal lung disease on chest radiography or thoracic computed tomography (CT) scan sx include tachypnoea, dyspnoea on exertion (such as feeding in infants/neonates) or at rest, persistent dry cough, failure to thrive, retractions, crackles, digital clubbing, cyanosis and, less frequently, chest wall deformity (pectus excavatum and others) paeds resp will do combo of pulm function tests, BAL, lung biopsy, genetics

Answer 90

primary infection often asymp, sometimes leaves calcification on CXR (healed primary focus) post primary from reactivation: persistent cough, sputum production, haemoptysis; systemic symptoms: fever, night sweats, anorexia, weight loss cavitating apical lesions on CXR, may also see pleural effusion, hilar node enlargement, calcified previous granuloma consider TB in patient with pyrexia of unknown origin as can affect any organ not just lung mantoux test suggests exposure to previous infection or BCG vaccine; if +ve and no BCG then a primary infection is highly likely; latent tuberculosis infection diagnosis and treatment should be considered for any BCG-vaccinated person whose skin test is 10 mm or greater 6mo rifampicin and isoniazid supplemented for first 2 mo by pyrazinamide and ethambutol consider infectious for at least first 2 weeks, keep in single room with full isolation gear if in hospital; -ve pressure ventilation room if they have drug resistant TB

Answer 91

much commoner in asian population primary infection via inhalation gives ghon focus spread to mediastinal and hilar lymph nodes forming primary complex, then rapid immune response confining the primary lesion which may calcify in 25% cases prim lesion extrrapulmonary secondary when reactivates/reinfected w type 4 hypersens giving caseating granulomatous process -> may get haemat spread giving miliary TB, or CNS/bone/lymph/renal involvement; also tuberculus empyema ZN stain, culture -> early morning sputum/urine sample radiology, esr/anaemia of chronic disease, histo RIPE for 2mo and RI for further 4

Answer 92

latent confirm with mantoux test if contact has TB, if pos assess for active, if not active can do ifn gamma release assay and if that also pos then treat latent infection; so IGRA test is used in patients that do not have features of active TB but do have a positive Mantoux test to confirm a diagnosis of latent TB; besides those with contacts, can also do ix in any immunosupp'd ppl based on risk factors; in children (exposed to ill ppl) w pos mantoux test can go straight to managing, if neg then ifn gra and rpt mantoux after 6 weeks 4 weeks-2yo and exposed then assess for active, start latent treatment and do mantoux test; if pos complete treatment, if neg rpt at 6 weeks; if also neg then ifn GRA, stop treatment if this neg and if rpt mantoux or ifn GRA pos then complete latent TB mx if neonate exposed to someone with <2 weeks treatment then isoniazid with pyridoxine, mantoux test -> if pos complete 6mo treatment, if neg then ifn GRA (is pos then 6mo isoniazid + pyrid if neg stop treatment) latent mx get isoniazid + pyridoxine for 6mo (or these + rifamp for 3mo) - MDT or seek advice if severe liver disease; HIV and HBV/HCV testing before starting treatment note ifn gra = quantiferon test

Answer 93

pulmonary TB CXR, 3 deep cough sputum samples (at least one in morning) for micro/culture if can't produce these then induced sputum in kids, that or BAL in adults if HIV+, rapid info needed, or <16yo then NAAT on sputum extrapulm TB still CXR + culture a sputum sample, then site specific tests: eg pleural fluid/biopsy, CT/MRI head + CSF, biopsy of tuberculoma, lymph node or pericardial biopsy, ascitic fluid, biopsy of liver/omentum, early morning urine culture, biopsy of urogen site, bone biopsy or joint fluid aspiration for culture mx w/o CNS involvement isoniazid (with pyridoxine), rifampicin, pyrazinamide and ethambutol for 2 months then isoniazid (with pyridoxine) and rifampicin for a further 4 months. mx w CNS involvement isoniazid (with pyridoxine), rifampicin, pyrazinamide and ethambutol for 2 months then isoniazid (with pyridoxine) and rifampicin for a further 10 months dexamethasone or prednisolone, initially at a high dose with gradual withdrawal over 4 to 8 weeks (including for kids)

Answer 94

Rifampicin can cause red/orange discolouration of secretions like urine and tears. It is a potent inducer of cytochrome P450 enzymes therefore reduces the effect of drugs metabolised by this system. Isoniazid can cause peripheral neuropathy. Pyridoxine (vitamin B6) is usually co-prescribed prophylactically to reduce the risk of peripheral neuropathy. Pyrazinamide can cause hyperuricaemia (high uric acid levels) resulting in gout. Ethambutol can cause colour blindness and reduced visual acuity. Rifampicin, isoniazid and pyrazinamide are all associated with hepatotoxicity

Answer 95

Chronic granulomatous condition - commonly involves lungs, skin, eyes Features: * Erythema Nodosa (painful red skin lesions on anterior surface of legs) lupus pernio (vilaceous nodules on nose/cheeks/ears)* Short of breath, cough, wheeze * Fatigue * Arthralgia * Lymphadenopathy * Anterior uveitis * Young and non-smoker Investigations: * Chest X-Ray: Bilateral Hilar Lymphadenopathy -> progressing to fibrosis * ACE raised * FBC (may have leukopenia and anaemia) * ESR raised * Serum Calcium raised (due to vit D production by macros) * CD4+>CD8+ on biopsy 4 stages: stage I: hilar or mediastinal nodal enlargement only stage II: nodal enlargement and parenchymal disease stage III: parenchymal disease only stage IV: end-stage lung disease (pulmonary fibrosis) Management: Corticosteroids for acute or chronic deterioration Pathology: Non caseating granulomas with multinucleate giant cells in the centre

Answer 96

Bronchitis Usually self limiting in 3 weeks; viral infection is leading cause, and usually in autumn/winter typically present with an acute onset of: cough: may or may not be productive sore throat rhinorrhoea wheeze The majority of patients with have a normal chest examination, however, some patients may present with: Low-grade fever Wheeze Differentiating acute bronchitis from pneumonia History: Sputum, wheeze, breathlessness may be absent in acute bronchitis whereas at least one tends to be present in pneumonia. Examination: No other focal chest signs (dullness to percussion, crepitations, bronchial breathing) in acute bronchitis other than wheeze. Moreover, systemic features (malaise, myalgia, and fever) may be absent in acute bronchitis Management analgesia good fluid intake consider antibiotic therapy if patients: are systemically very unwell have pre-existing co-morbidities Have raised CRP>20; doxy first line unless child or pregnant

Answer 97

prodrome (pre-illness phase) lasts between 2–10 days. The commonest symptoms are fever, a new and continuous cough, shortness of breath, fatigue, loss of appetite, anosmia (loss of smell) and ageusia (loss of taste). Other symptoms include headache, muscle pain, sore throat, nasal congestion, chest pain, nausea and vomiting, diarrhoea, skin rashes 3 most common: Headache. Runny nose. Sore throat. 81% develop only mild to moderate symptoms (up to mild pneumonia), while 14% develop severe symptoms (dyspnea, hypoxia, or more than 50% lung involvement on imaging) and 5% of patients suffer critical symptoms (respiratory failure, shock, or multiorgan dysfunction); at least ⅓ may show no symptoms some people – over half of a cohort of home-isolated young adults– continue to experience a range of effects, such as fatigue, for months after recovery, a condition called long COVID; long-term damage to organs has been observed Mild sx: supportive care includes medication such as paracetamol or NSAIDs to relieve symptoms; if high risk (eg immunosupp) + in hospital then start remdesivir (other antivirals exist for a similar point eg nirmatrelvir and ritonavir (Paxlovid) or molnupiravir (Lagevrio)) Mod sx: In those with low oxygen levels, use of dexamethasone + remdesivir is strongly recommended; if rapidly inc'g O2 needs or sys inflam then tocilizumab Severe sx: NIV or ventilation and if needed can escalate to ecmo

Answer 98

Post-bronchodilator FEV1/FVC FEV1 (of predicted) Severity < 0.7 > 80% Stage 1 - Mild - symptoms should be present to diagnose COPD in these patients < 0.7 50-79% Stage 2 - Moderate < 0.7 30-49% Stage 3 - Severe < 0.7 < 30% Stage 4 - Very severe following investigations are recommended in patients with suspected COPD: post-bronchodilator spirometry to demonstrate airflow obstruction: FEV1/FVC ratio less than 70% chest x-ray: hyperinflation, bullae, flat hemidiaphragm. Also important to exclude lung cancer full blood count: exclude secondary polycythaemia body mass index (BMI) calculation remember FEV1 reduced as less elastic recoil means less alveolar pressure so less flow (and get obstruction as less PA vs Pip meaning EPP shifts distally) thus FEv1 relates to degree of loss of elastic recoil and more severe gives less FEV1

Answer 99

acute laryngotracheobronchitis (croup): commonest in 1-3yo, parainfluenza virus, coryza over a couple of days then sx dev; cool steam mist vaporiser, maintain fluids, steroid nebs (dexamathasone), adr nebs as rescue if needs intensive care transfer acute epiglottitis: commonest in 2-6yo, HiB; high fever, looks toxic, drooling with weak and muffled voice, coughing rare, neck extended and leaning forwards; lat neck x-ray shows enlarged epiglottis resembling a thumb; do not examine throat or agitate child (no cannulae etc), experienced ent surgeon and anaesthetist ASAP, adr nebs in meantime, intubation ideally in icu, and then laryngoscopy can also confirm diagnosis by visualising inflamed epiglottis; then iv abx eg cefotaxime bacterial tracheitis: staph aureus follow on from croup like illness - croupy child fails to improve, becomes toxic w high fever, productive chesty cough, and stridor; can't exclude epiglottitis so manage similarly, intubation oft needed, then trach swabs and iv abx diptheria: coryza then pseudomembrane causing airway obstruction, corynebacterium diphtheriae; generally sore throat, pseudomembrane, problems swallowing/breathing, cervical lymphadenopathy; if toxin gets in blood can cause myocarditis in week 2 (tachycard, heart failure), as well as neuritis: resp failure (from diaphragm paralysis) and in weeks 3-8 palatal and limb paralysis; vaccination, if have disease then antitoxin and abx, manage airway inhaled foreign body: always suspect if acute episode of choking/coughing, usually <4yo, after several days pneumonia devs; suspect also if unresolving pneumonia or bronchiectasis; stridor, wheeze, aphonia, collapse of lobe may make it obvious Neck and chest X-rays are the most important investigations in every patient suspected of having a foreign body aspiration; An expiratory chest X-ray should be requested when the standard inspired film is normal, since this strategy allows visualisation of air trapped by a valve-like effect due to partial obstruction of the bronchial lumen; only 10% of FBs are radio-opaque therefore an initial normal chest X-ray does not rule out the possibility of foreign body aspiration If a child is unable to speak or cough, dislodgement using five back blows followed by chest compressions in infants less than one year of age is recommended. Abdominal thrusts are not advisable because they may cause damage to the liver, which is relatively large and unprotected in this age group. For children >1 year of age five abdominal thrusts should be performed If this is not successful and the child is unresponsive continue with Basic Life Support and get senior anaesthetic help. If the child is alert, encourage coughing. Consider direct laryngoscopy, needle cricothyrotomy or intubation, ventilation and stabilisation (+/- transfer) for a definitive intervention as next steps. Bronchoscopy is the only procedure that can be both diagnostic and therapeutic. Removal of the foreign body from the airway must be undertaken using a rigid instrument; if stable refer to ENT or resp for bronch depending on local protocol retropharyngeal abscess (toxic with neck extension and cervical lymphad) treated with incision under GA angioedema, burns to neck/larynx, in neonate hypocalc also severe upper airway obstruction -> negative intrathoracic pressure -> pulm oedema (aka postobstructvie pulm edema 'POPE': Type I follows a sudden, severe episode of upper airway obstruction such as postextubation laryngospasm, epiglottitis, croup, and choking, and is seen in strangulation and hanging. Type I POPE may be associated with any cause of acute airway obstruction. Type II POPE develops after surgical relief of chronic upper airway obstruction. Reported causes include tonsillectomy and removal of upper airway tumors.

Answer 100

PA and lat CXR, neck x ray, barium swallow, consider CT/MRI scan, flexible laryng/bronchoscopy laryngomalacia: in first month of life, may be some exp stridor as well, worse when supine/agitated/urti, improves by 1-3yo subglottic stenosis: may be congen or acquired by repeated/prolonged intubation, inhaled noxious fumes/burns; improves as ages vocal cord palsy: central or periph eg rec laryngeal nerve palsy from intrathoracic path compression: subglottic haemangioma, cysts, vascular rings, laryngeal web, laryngeal papillomatosis (HPV, poss vertical transmission), retrosternal goitre, neoplasia, cystic hygroma

Answer 101

refers to an excessive increase in compliance of the trachea, such that the airway is more susceptible to dynamic and/or static collapse; this is distinguishable from intrinsic airway stenosis caused by mural problems such as complete tracheal rings. TM may be localized or generalized. The mainstem bronchi may also be affected, which is referred to as tracheobronchomalacia (TBM) often congenital and linked to TOF, BPD, CF, laryngomalacia; sx generally emerge around 2-3mo barking cough with expiratory rhonchi or inspiratory stridor may be present in most patients with TM/TBM. The extensive airway collapse can lead to ineffective cough and reduced clearance of secretions; airway obstruction often results in insufficient ventilation; therefore, patients may experience exercise intolerance, hypoxic episodes, or apneic events; symptoms can be worsened by any activities or conditions that increase the intrathoracic pressure and the patient's respiratory efforts, including activities such as coughing, crying, Valsalva maneuvers, feeding, forced expiration, or lying supine for diagnosis direct visualization is achieved through flexible and rigid endoscopy, including laryngoscopy, tracheoscopy, and bronchoscopy - dynamic bronchoscopy is considered the gold standard often resolves over time so patients affected by mild to severe TM/TBM may benefit from medical management: pratropium bromide (Atrovent) is administered to minimize the secretions without thickening the secretions as may occur with glycopyrronium bromide, hypertonic saline is also nebulized to thin the secretions as much as possible, chest physiotherapy to help with mucociliary clearance, as well as control of gastroesophageal reflux to minimize aspiration of inflammatory gastric contents, are also encouraged Surgical treatment is reserved for the most severe cases and must be specific to the type and location of the TM/TBM - ENT will evaluate and decide on this

Answer 102

inhaled - coughing, choking, may present as unconscious w/ cardiorespiratory arrest; open airway, visualise, remove with forceps; alternating backblows and chest thrusts and try to remove; if however obstruction only partial then seat child however they are most comfortable, and arrange urgent surgical removal if in lower bronchus may have wheeze, cough, unexplained fever/dyspnoea, and maybe but not always a history of choking, coughing, or wheezing while eating or playing; CXR, surgery swallowed - oft witnessed, sometimes present with gagging, vomiting, distension, obstruction, rectal bleeding (dep on site where lodges); perf of oesophagus or gut is possible; vitals, observe oropharynx, resp and gastro exams; if radioopqaue then plain AXR; CT another option, and can do endoscopy w/ removal - normally is large, magnetic, certain batteries`

Answer 103

Laryngomalacia occurs in infants, peaking at 6 months. It presents with inspiratory stridor, a harsh whistling sound when breathing in. Usually this is intermittent and become more prominent when feeding, upset, lying on their back or during upper respiratory tract infections Infants with laryngomalacia do not usually have associated respiratory distress sore throat + stridor -> ?epiglottitis secure airway (tracheostomy may be needed) then IV antibiotics (e.g. ceftriaxone) Steroids (i.e. dexamethasone)(all should be done by seniors w anaesthetist available); most pts wont need intubation though

Answer 104

bronchoconstriction: b blockers, opioids, NSAIDs cough: ACEi bronchiolitis obliterans: penicillamine diffuse parencyhmal lung disease: bleomycin, methotrexate, sulfasalazine, penicillamine, amiodarone, hydralazine, nitrofurantoin PE: oestrogens Pulm HTN: oestrogens pleural effusion: amiodarone, nitrofurantoin, phenytoin, methotrexate resp depression: opioids, benzos

Answer 105

CAP usually strep pneumoniae (50-60%), other causes inc myco or chlamidya pneumoniae (10% each) and more rarely H influenzeae, viral; HAP if pnuemonia devs 2+ days after admission to hospital, may be similar to other pathogens if mild on normal wards; in context of ITU, endotracheal intubation or already on antibiotics, often is g-ve bacteria like pseudomonas or e coli in 50% cases (MR)staph aureus in 20% cases, these from broad spectrum antibiotics promoting colonisation of resp tract and intubation etc allowing aspiration of g-ve bacti from GI tract, also impaired cough from surgery etc COPD and immunosuppression allow low virulence H influenzeae to spread and cause bronchopnuemonia assess severity with CURB-65: confusion, urea >7mmol/L, resp rate >30/min, blood pressure sys <90 or dias <60, age over 65; 1 treat at home unless clinical picture suggests worse than the CURB65 score; score 2 and negotiate with patient; score 3+ treat in hospital; be aware of need for ITU, esp if score 4+ cough, purulent sputum, fever; often pleuritic pain and dyspnoea; may have history of recent URTI localized crackles, dullness, bronchial breathing; elderly patients may present without resp signs, just confusion be on lookout for resp failure (cyanosis, tachypnoea) or septicaemia (rigors, high fever) CXR see consolidation and complications like effusion or empyema; bloods for infection (FBC, U&Es, CRP), oximetry; blood culture, sputum culture, pleural fluid aspiration if effusion beware also is more likely in patients with immunosuppresion (eg autoimmune disease, post transplant, HIV) and those on antineoplastic chemo also assess oxygenation, social circumstances, comorbid illnesses urgent CXR key in confirming the diagnosis mild in fit patient treat at home if in hospital: monitor resp rate, O2 sats, pulse, blood pressure, temperature, CRP and give antibiotics urgently O2 given to maintain sats >94% and pO2 >kPa, paracetamol or NSAIDs to control pleuritic pain antibiotics: generally amoxicillin 500mg tds orally, clarithromycin if penicillin allergy or if atypical eg mycoplasma pneumoniae pneumocystis pneumonia due to pnuemocystis jirovecii in immunocomp and HIV patients with subacute cough, dyspnoea, fever, perihilar infiltrates on CXR, desaturate on exercise, treat with high dose co-trimoxazole

Answer 106

type of pneumonia usually only seen in paediatric patients. They are well defined, rounded opacities that represent regions of infected consolidation; note you can still get in adults but it is rarer no specific bacterium that causes round pneumonia - common causes of regular pneumonia apply here proposed theory as to why children develop round pneumonia and adults do not relates to the development of inter-alveolar communications and collateral airways. These are called pores of Kohn and canals of Lambert, and when they develop, they allow air-drift between the parenchymal subsegments. In adults, these allow lateral dissemination of infection throughout a lobe, leading to lobar pneumonia. In children, where these have not developed, the limited spread of infection results in round pneumonia

Answer 107

Organisms include viruses / Streptococcus pneumococcus / Haemophilus influenzae / Group A Streptococcus / Staphylococcus aureus / Mycoplasma pneumoniae * A diagnosis of CAP should be made up to 48 hours of a hospital admission e.g. if treating secondary infection on NIV. * Consider the possibility of infection or co-infection with viruses such as Covid or flu Symptoms and signs suggestive of bacterial pneumonia: * Cough, repeated / persistent fever > 38 C (usually at least 48 hours) + chest wall recession + tachypnoea + crackles on auscultation * In older children more common symptoms are breathlessness, pleuritic chest pain, abdominal pain (pneumonia is the commonest extra-abdominal cause of abdominal pain) and headache Generalised wheeze with low-grade temperature is NOT typical for bacterial pneumonia The absence of focal crackles on auscultation do not exclude pneumonia in children – occult pneumonia (pneumonia without chest signs) is not uncommon in children features of severe CAP: Significant tachypnoea * Significant tachycardia * Severe respiratory distress (significant recession (age < 12 months), nasal flaring, grunting) * Apnoeas (ages < 12 months) * Hypoxia (sustained O2 sats ≤ 92% in air) * Capillary refill time > 2 seconds * Signs of severe dehydration * Cyanosis Unless CAP is severe, investigations are NOT routinely required if investigating, then do: CXR (AP) 2. Bloods: FBC, CRP, U&E, blood culture, blood gas 3. Consider microbiological tests e.g. Throat swab for viral respiratory screen, sputum sample for MC&S indications for CXR: - hypoxia or - significant respiratory distress, or - considering pleural effusion as a diagnosis (no improvement in symptoms despite >48 hours of adequate antibiotic therapy, focal reduced breath sounds, prolonged fever), or - possibility of TB/atypical pneumonia Start antibiotic treatment as soon as possible after establishing the diagnosis. Aim for < 4 hours or < 1 hour if sepsis suspected home mx: Well child. No severe signs or symptoms of CAP:  SpO2 >92% in air &,  Eating & Drinking &,  Obs within normal limits, &  No co-morbidities or patient risk factors for severe infection e.g. CLD, neuromuscular disease, immunosuppression Give oral abx Reassess if there is:  Increased work of breathing, distress or agitation  Persistence of fever 48 hours after initiation of treatment Signs or symptoms of severe CAP:  Requires O2 to keep O2 sats > 92%  Requires respiratory support for increased WOB  Requires IV fluids or NG feeds e.g. persistent vomiting, unable to feed  High risk for severe infection e.g. CLD, neuromuscular disease, immunosuppression then admit: Oral or IV antibiotics with daily review - 2/3rds maintenance IV / NG fluids if dehydrated or not drinking - Oxygen via mask or high flow nasal cannula +/- NIV as required Consider IV antibiotics if:  Unable to take oral antibiotics or severity of disease requires IVs e.g. very severe work of breathing, reduced level of consciousness  Complicated CAP e.g. pleural effusion or necrotising  Oral antibiotics not tolerated or absorbed e.g. persistent vomiting, severe diarrhoea  No improvement despite ≥48 hours of adequate oral antibiotics (check dose and adherence)  Co-morbidities that predispose to severe pneumonia (e.g. significant immunosuppression) Follow-up chest x-ray is NOT required in those who were previously healthy and recovering uneventfully * Children with round pneumonia, pleural effusion, collapse or persisting symptoms should be followed up in a Respiratory team clinic 6 - 8 weeks after discharge with a repeat CXR

Answer 108

CAP with parapneumonic effusion abx (generally most likely organism is strep pneumonia, but also eg staph aureus or GAS can lead to empyema too), can add clindamycin, esp if thinking staph or GAS chest drain will be needed if there is a significant fluid collection (>1-2 cm depth) and lack of resolution (spiking fever, no clinical improvement) after 24-48 hours of appropriate antibiotics, or if pH <7.2 or LDH >900 with glucose <4 or septations on USS. More immediate drainage will be needed for a large collection with mediastinal shift, or respiratory distress with oxygen requirement if you want a chest drain putting in will need up to date FBC and coags Generally liaise with surgeons who will insert the drain, or resp team sometimes will after return from theatre should be started on intrapleural urokinase for 3 days, after each admin drain locked off for 4 hours and then 8 hours with gentle suction on drain, regular analgesics important abx continue IV until apyrexial for 24 hours then switch to oral if not improving (in inflam markers or pleural shadowing) then thoracic CT, and if surgical candidate then referral for VATS, if not surgical candidate can consider tPA + DNase or intrapleural irrigation if high bleeding risk, also consider abx switch and other types or surgery

Answer 109

infection and/or inflam damages bronchi so permanently dilated: after injury comes repair, with fibronectin and integrin receptors used for cell migration and adhesion during this process; bacti like p aeruginosa also adhere here stimulating inflam, and persistent cycle of chronic infection and inflam occurs if body cant eradicate the infection which further damages the bronchi may be local (if eg obstruction or bronchus) or diffuse (eg Ig deficiency) pertussis and measles in childhood are important causes, in adults pneumonia and TB; other childhood lung infections may also manifest later in life with bronchiectasis also after obstruction (foreign body, tumour, enlarged lymph nodes from TB or cancer); also from mucous plugs in allergic bronchopulmonary aspergillosis in asthmatics (high serum IgE) and +ve skin prick test; also primary ciliary dyskinesia also causes this (alongside sinusitis, otitis, subfertility in men, and dextrocardia or even situs invertus in up to 50% of people with condition), it is autosomal recessive; higher incidence of ectopic pregnancy due to fallopian tubes not working right CF may also cause bronchial damage can also damage cilia and continue the vicious cycle chronic cough with copious purulent sputum, mild cases may be misdiagnosed as chronic bronchitis fever, pleuritic pain and inc cough and sputum from exacerbation haemoptysis can occur and may be severe may get malaise, fatigue, weight loss, halitosis, coarse crackles, clubbing CXR may show peribronchial thickening but may also be normal, CT is the key investigation to confirm diagnosis and extent of the disease then investigate the cause with ciliary function test, test for CF, serum Ig test, skin prick for ABA; bronchoscopy for obstruction; sputum microbiology can treat cause eg remove obstruction, iv Ig replacement, suppress immune response with oral or inhaled steroids for ABA antibiotics for chronic infection mucolytic agents may also be helpful inhaled bronchodilators if a reversible obstruction, and physiotherapy to help clear mucous

Answer 110

chronic productive cough w purulent sputum, halitosis, rec chest infections, episodes of haemoptysis, and clubbing; may hear coarse crackles and wheeze CXR may show tram lines CT: diameter of a bronchus should measure approximately 0.65-1.0 times that of the adjacent pulmonary artery branch between 1 and 1.5 may be seen in normal individuals, especially those living at high altitude greater than 1.5 indicates bronchiectasis may see signet ring sign, tram track sign, string of pearls sign etc ix if persistent purulent sputum, rheum arth, IBD, or immunosupp'd and dev rec cough, COPD with 2+ exacerbations in prev year initial ix: FBC, IgE levels, aspergillus testing, serum IgG/M/A, test for CF/PCD, sputum culture, baseline Ig against strep pneumoniae; consider autoimune screen if other sx and A1AT testing if emphysema, bronchocoscopy if localised, HIV/immunodef testing if suspicious resection can be considered for localised disease if p aeruginosa colonised and 3+ exacerbations a year then inhaled colistin (gent as second choice) or oral azithro/erythro; latter is first line if no p aeru step 1: teach chest physio, treat underlying cause, annual flu vaccine, get pneumococcal vaccine, treat exacerbations step 2: 3+ exacerbations a year despite step 1 consider mucolytics eg carbocisteine trial for 6mo step 3: long term colistin, gent, or macrolide depending on colonisation (macrolide even if no pathogen) step 4: if 3+ a year despite above then inhaled abx and macrolide step 5: if 5+ exacerbations a year despite above then Iv abx every 2-3mo throughout ensure optimal control of asthma, COPD etc Consider transplant referral in bronchiectasis patients aged 65 years or less if the FEV1 is <30% with significant clinical instability or if there is a rapid progressive respiratory deterioration despite optimal medical management if haemoptysis: IV abx, TXA, embolisation by IR is often first step for large haemorrhage; oral abx would suffice if haemorrhage <10ml

Answer 111

some diseases of the respiratory tract are characterized by unusually thick forms of mucus that accumulate within the airways, known as mucous plugs asthma, ABPA, lipoid pneumonia, COPD, CF, and sometimes pneumonia esp atypical (and of them esp mycoplasma) may present like a foreign body: cough, wheeze +/- reduced air entry, dyspnoea inc desaturation (may be significant), lobe collapse, T2RF, recurrent infections and bronchiectasis; suspect if not responding to initial mx of whatever resp condition you initially suspected may appear as a branching tubular opacity that is distinct from the normal vascular shadows. This classic feature is the finger-in-glove sign, however is hard to see on XR; CT shows better; mucoid impaction is more common on right side, and in upper lobe hydration, humidification of inhaled O2, nebs or meds to loosen sputum and do bronchodilation, chest physio; if fails may need bronch to remove plug _. ig you havent got a CT prior to this point (ie if pt well you may treat empirically after CXR) then do get the CT

Answer 112

mycoplasma pneumoniae: prolonged and gradual onset flu-like symptoms classically precede a dry cough bilateral consolidation on x-ray cold agglutins (IgM): may cause an haemolytic anaemia erythema multiforme, erythema nodosum meningoencephalitis, Guillain-Barre syndrome and other immune-mediated neurological diseases deranged LFTs, peri/myocarditis, serology to diagnose; macrolides legionella: also flu like, dry cough, deranged LFTs, treated with macrolide but lymphopenia, hyponat, bradycard, urinary antigens diagnose psittacosis from cleaning bird cage/pet birds; Flu-like symptoms (90%): fever, headache and myalgia; organomeg, fails to respond to penicils Respiratory symptoms (82%): dyspnoea, dry cough and chest pain serology confirms (usually part of atypc pneumonia screen) coxiella: prodrome: fever, malaise causes pyrexia of unknown origin transaminitis atypical pneumonia endocarditis, manage with doxy as for psittacosis CURB65 =2 oral or iv amoxi + clarithro, doxy and clarithro if atypc; 3+ iv co-amox and clarith, specialist if atypc metro to cover for anaerobes if aspiration CXR 4-6 weeks later to check recovery, rule out any cancers etc

Answer 113

eosinophils in blood plus in lung infiltrates which give CXR shadows and confirmed by BAL or biopsy include aspergillus/other fungi, drugs (sulphonamides/NSAIDs/tetracyclines/nitrofurantoin), parasites (ascariasis, schistosomiasis), vasculitides (eGPA), idiopathic

Answer 114

bronchoconstriction: wheeze, cough, dyspnoea. Patients may have a previous label of asthma; think this in pt with chronic asthma + bronchiectasis + rec pulmonary infiltrates eosinophilia, elevated serum IgE; positive radioallergosorbent (RAST) test to Aspergillus steroids, maybe itraconazole

Answer 115

in longstanding asthma/CF patients, usually in second decade type 1 and 4 hypersens after sensitisation get rec episodes of fever, wheezing, crepitations, dyspnoea and migratory pulm infiltrates on xray; then bronchiectasis devs, then fibrosis FBC will show eosinophilia, will also be in sputum, will see raised IgE and positive aspergillus serology or skin test; dd are other pulm eosinophilias (see other card) but this one commonest impossible to avoid antigen so steroids and bronchodilators mainstay of treatment

Answer 116

large haemoptysis is 150-400ml a day, massive is 400ml+; these are life threatening and need admission and urgent investigation after initial investigations (autoimmune screen, CXR, clotting screen, fbc, crp, G&S) a CT thorax is useful ANCA activate the neutrophils causing acute inflam, and in time macros and T cells mediate chronic inflam and scarring GPA gives necrotic/cavitating lung masses and palisaded granulomas, EGPA asthma and eosinophilia plus pulmonary infiltrates, MPA leucocytoclastic vasculitis; all may cause haemoptysis may see consolidation, ground glass, cavitating lesion, thickened vessel walls; bronchial wall thickening giving narrowed bronchial lumen after CT may want to do BAL (microbiology, cytology), biopsy, and EBUS guided lymph node biopsy - all via bronchoscopy

Answer 117

TB, malignancy w post obstructive pneumonia (lung cancer, lymphoma, mets), vasculitis, right up lobe pneumonia klebsiella give necrotising pneumonia - see abscess in lung which looks a bit like tumour TB 3 samples for microscopy for acid-fast bacilli, Mycobacteria culture, and specialist molecular tests/drug sensitivity testing

Answer 118

rare in kids CF, pneumonia, TB, foreign body inhalation, bronchiectasis, bleeding diathesis, idiopathic haemosiderosis, vasculitis (GPA), AV fistula, ruptured hydatid cyst, aspergillus, and varices that can ooze or rupture (latter giving massive H+) secondary to pulm HTN (eg valvular heart disease)

Answer 119

Was the blood definitely coughed up from the chest? Blood in the mouth may be vomited, may have come from the nose in epistaxis, or may appear on chewing or tooth brushing in patients with gum disease. * A short history of streaks of blood with purulent sputum suggests acute bronchitis. * A sudden episode of a small volume of blood with pleuritic pain and breathlessness suggests pulmonary embolism. * Recurrent streaks of blood in clear sputum should prompt a search for lung cancer. * Recurrent blood streaks in purulent sputum over weeks suggest possible tuberculosis or cancer with infection; over years, they suggest bronchiectasis. * Larger volumes of haemoptysis (>20 mL, for example) suggest specific causes: * lung cancer eroding a pulmonary vessel * bronchiectasis (such as in cystic fibrosis) * cavitatory disease (such as bleeding into an aspergilloma) * pulmonary vasculitis * pulmonary arteriovenous malformation

Answer 120

a life-threatening and medical emergency that can be caused by numerous disorders and presents with hemoptysis, anemia, and diffuse alveolar infiltrates. Early bronchoscopy with bronchoalveolar lavage is usually required to confirm the diagnosis and rule out infection; Up to 1/3 pts dont have haemoptysis, and infiltrates can be unilat; therefore AH must be considered in patients with otherwise unexplained alveolar infiltrates Broadly 3 types: 1. DAH associated with vasculitis or capillaritis Most cases of DAH are caused by pulmonary capillaritis and are closely associated with systemic vasculitis and findings such as anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, anti-GBM disease, systemic lupus erythematosus (SLE), and collagen vascular diseases (CVDs). It is also seen with several other conditions, including the use of certain drugs and transplantation. 2. Bland pulmonary hemorrhage (without capillaritis or vasculitis) In this pattern, RBCs leak into the alveoli without any evidence of inflammation or destruction of the alveolar capillaries, venules, and arterioles. The epithelial lesions are usually microscopic and are scattered geographically. Anti-GBM diseases and SLE can induce both pulmonary capillaritis and bland pulmonary hemorrhage. 3. DAH associated with another process or condition In this third category of DAH, alveolar hemorrhage is caused by other processes, such as diffuse alveolar damage, inhalation, or cytotoxic drug therapy. Clinical scenarios suggestive of vasculitis are: 1) DAH, 2) acute glomerulonephritis (GN), 3) pulmonary-renal syndrome, 4) deforming or ulcerating upper airway disease, 5) cavitary or nodular disease on chest imaging, 6) palpable purpura, 7) mononeuritis multiplex, or 8) multisystem disease If sinus disease, skin manifestations, pulmonary parenchymal nodules, and cavitary lesions coexist with positivity for anti-proteinase 3 (PR3) C-ANCA and biopsy-proven granuloma, then Wegener's granulomatosis (WG) should be considered. DAH with GN and skin manifestations, positivity for P-ANCA, and necrotizing non-granulomatous lesions on end-organ biopsy may lead to a diagnosis of microscopic polyangiitis (MPA). Churg-Strauss syndrome (CSS) should be considered if asthma, eosinophilia, pulmonary infiltrates, and DAH coexist. In young smokers with GN and DAH presenting as either bland alveolar hemorrhage or pulmonary capillaritis, Goodpasture's syndrome or anti-GBM disease are possible diagnoses When the above conditions have been considered but no suggestive findings are found, the following four conditions should be considered: 1) anti-GBM disease in limited pulmonary form or onset, for which positivity to the antibody with linear deposits in the lungs would be diagnostic, 2) pulmonary-limited MPA, which would be positive for positive anti-myeloperoxidase (MPO) P-ANCA, 3) pauci-immune isolated pulmonary capillaritis, which would show evidence of neutrophilic pulmonary capillaritis upon biopsy, or 4) idiopathic pulmonary hemosiderosis, a diagnosis of exclusion, when the biopsy shows evidence of acute, subacute, and chronic bland DAH and no evidence of vasculitis

Answer 121

antenatal dx through CVS or amniocent sweat test: pilocarpine to induce sweating on small area of skin, collect sample, needs 2+ samples to have [Cl] >60mmol/L DNA analysis newborn screening looks for elevated serum immunoreactive trypsin, then confirm with tests above false positive sweat test: adrenal insuff, congenital adrenal hyperplasia, diabetes insipdus, G6PD, ectodermal dysplasia, hypothyroidism, mucopolysaccharidoses

Answer 122

resp: rec chest infections is commonest way to present, bronchiectasis develops and leads to patchy areas of collapse and consolidation; later haemoptysis, PTX, cor pulmonale; linked to ABPA GI: meconium ileus is early presentation in 15% of cases, or else pancreatic insuff + associated malabsoprtion (second commonest way to present is deficiency of fat soluble vitamins); failure to thrive despite big appetite; may get pancreatitis, DM, biliary cirrhosis, gallstones, rectal prolapse, distal intestinal obstruction in older children ENT: sinusitis occurs in most, nasal polyps in 10-20% of pts repro: delayed puberty as for all chronic illness, reduced female fertility due to abnormal cervical mucous, males almost always infertile due to atresia of vas deferens skeletal: short stature as for all chronic illness in children, clubbing -> HPOA CV: due to salt loss in sweating, higher risk of heat stroke or CV collapse in hot weather; cor pulmonale after pulm HTN metabolic: in infants can cause hyponatraemic hypochloraemic metabolic alkalosis haemat: anaemia of chronic disease

Answer 123

autosomal recessive, can present at any age due to biological reserve antenatal diagnosis possible if parents thought to have high risk of having child with it most cases picked up using newborn heel prick test, then suspicious cases confirmed using sweat test and genetic test (of blood or saliva) heel prick test: 5 days old, collect 4 drops of blood from heel: sickle cell, CF, congen hypothyroid,6 inheritable metabolic diseases during childhood: recurrent LRTIs, failure to thrive, nasal polyps (in children nearly always due to cf), diarrhoea, acute/chronic panc, portal hypertension, chronic sinus problems, bronchiectasis, male infertility - obstructive azoospermia; meconium ileus, malabsorption as child; cough with purulent sputum, obstructive picture, crackles, wheezes (mostly upper lobe), clubbing (signs and symptoms not usually babies or very young kids) bronchiectasis, prog airway obstruction, cor pulmonale, then death; also problems with resistant bacteria eg p aeruginosa, mrsa chest physio 2x daily to clear mucous, rhDNase mucolytic; reg sputum samples for culture, proph antibiotics; chronic p aeru get nebulised colistimethate sodium or tobramycin; if panc insuff confirmed with stool elastase or faecal fat then prescribe panc enzymes; hepatosplenomegaly, poor bile flow, prog liver failure, death so transplant may be needed screen for diabetes, give insulin replacement if picked up may need ca, vit d, bisphosphonates, regular dexa regular monitoring incs: PFTs, CXR, DEXA, blood glucose, resp cultures, LFTs and liver uss if abnorm - ursodeoxycholic acid to correct LFTs, stop if corrected, blood vit d; flu and pneumococcal vaccines constipation, intussusception, appendicitis, cholecysitis

Answer 124

Provide regular routine reviews for people with cystic fibrosis, and do these more frequently immediately after diagnosis and in early life. For example: weekly in their first month of life every 4 weeks when they are between 1 and 12 months old every 6 to 8 weeks when they are between 1 and 5 years old every 8 to 12 weeks when they are over 5 years old every 3 to 6 months as adults at each review include: a clinical assessment, including a review of clinical history and medicines adherence, and a physical examination with measurement of weight and length or height measurement of oxygen saturation taking respiratory secretion samples for microbiological investigations, using sputum samples if possible, or a cough swab or nasal pharyngeal aspirate (NPA) lung function testing with spirometry (including forced expiratory volume in 1 second [FEV1], forced vital capacity [FVC], and forced expiratory flow [FEF] 25–75%) in adults, and in children and young people who can do this Discuss the use of airway clearance techniques with people with cystic fibrosis who do not have clinical evidence of lung disease and their parents or carers (as appropriate). Provide them with training in airway clearance techniques and explain when to use them. Do not offer high-frequency chest wall oscillation as an airway clearance technique for people with cystic fibrosis except in exceptional clinical circumstances. The specialist cystic fibrosis team will decide whether these circumstances apply Offer a mucoactive agent to people with cystic fibrosis who have clinical evidence of lung disease Offer rhDNase (dornase alfa; recombinant human deoxyribonuclease) as the first choice of mucoactive agent - if inadequate response then both rhDNase and hypertonic sodium chloride or hypertonic sodium chloride alone; Consider mannitol dry powder for inhalation for children and young people who cannot use rhDNase and hypertonic sodium chloride because of ineligibility, intolerance or inadequate response Offer flucloxacillin as antibiotic prophylaxis against respiratory Staphylococcus aureus infection for children with cystic fibrosis from the point of diagnosis up to age 3, and consider continuing up to 6 years of age. If a person with cystic fibrosis develops a new Pseudomonas aeruginosa infection (that is, recent respiratory secretion sample cultures showed no infection): commence eradication therapy with a course of oral or intravenous antibiotics, together with an inhaled antibiotic and follow this with an extended course of oral and inhaled antibiotics; Nebulised tobramycin or colistin are often used in initial eradication and chronic suppression regimens; If eradication treatment is not successful, offer sustained treatment with an inhaled antibiotic: consider nebulised colistimethate sodium as first-line treatment; For people with chronic Pseudomonas aeruginosa infection who are clinically deteriorating despite regular inhaled colistimethate sodium, consider nebulised aztreonam, nebulised tobramycin, or tobramycin DPI For people with cystic fibrosis and deteriorating lung function or repeated pulmonary exacerbations, offer long-term treatment with azithromycin at an immunomodulatory dose. Perform a clinical assessment and liver function blood tests at the annual review for people with cystic fibrosis; If liver function blood tests are abnormal, perform a liver ultrasound scan and consider ursodeoxycholic acid treatment Think about stopping ursodeoxycholic acid if liver function blood tests return to normal and clinical assessment and liver ultrasound scan show no liver disease. If ursodeoxycholic acid is stopped, monitor for re-emergence of liver disease using clinical assessment and liver function blood tests. Think about referring people with cystic fibrosis to a liver specialist if the liver function blood test results are persistently abnormal despite treatment with ursodeoxycholic acid. Refer people with cystic fibrosis to a liver specialist if they have any of the following: chronic progressive liver disease, based on clinical assessment, liver function blood tests or the findings on a liver ultrasound scan liver failure, based on clinical assessment and liver function tests test annually for DM from age 10yo; annual review should also have a psych assessment and look for signs of malabsorption, checking faecal elastase if present

Answer 125

periorbital petechiae, bronchopneumonia, bronchiectasis, otitis media, convulsions (esp in infants), cerebral h+ (long term mental retardation), rectal prolapse or umbilical hernia will see lymphocytosis very commonly, can culture pertussis from nasal swab or IgG/M serology

Answer 126

AR inheritance rec chest infections, rec sinus infections (may appear as rec headaches/face pain), nasal polyps, rec otitis media, bronchiectasis, clubbing, male infertility similar to CF but kartageners has no GI features situs inversus in 50% of pts or may see eg dextrocardia; absent frontal sinuses on skull x-ray EM of cilia from nasal brushing demonstrates abnorms in dynein arm manage the bronchiectasis (rate of progression of this determines overall prognosis)

Answer 127

CF, kartagener, GORD, bronchiectasis, immunodef, tumour, foreign body, structural airway defect, bulbar palsy/unsafe swallow, abx resistance

Answer 128

pneumococcus - from 2mo age, commonest bacti cause, patchy in younger kis and lobar in older, effusions poss, rusty sputum/haemop HiB - from 1mo, most common <5yo but less prev; usually lobar, less acute than pneumococcus mycoplasma - 3-15yo, most common if 10yo+; gradual onset, cough, fever, headache, chest pain, sore throat; usually unilat; bronchopneumonia picture; associated with erythema multiforme, arthropathy, meningoencephalitis, otitis media, bronchiolitis, croup; positive coombs test w cold agglutinins in 50%, some may have hamolytic anaemia; WCC oft normal staph aureus - usually infants or immunocomp (chronic granulomatous disease) or tracheostomy/prolonged intubation, CF, post-flu; patchy consolidation may form cyst then abscess, which if ruptures may cause PTX or empyema chlamydia - infants up to 3mo, acquired during birth, conjunctivitis commonest presentation, pertussis like cough, interstitial infiltrates on CXR, eosinophilia; swab from eye, giemsa stain shows chlam inclusion bodies, plus do culture and serology (para)influenza - malaise, coryzal, peribronchial thickening, interstitial pneumonia, oft also bronchitis/bronchiolitis RSV - bronchiolitis PCP - immunocomp'd children, chronic progressing afebrile pneumonia, oft broncho; cough, dyspnoea, tachypnoea, intercost recession, maybe hypoxia/cyanosis; HIVpos infant may present in first few months with bronchiolitis like syndrome but RSV neg - check for PCP

Answer 129

can be any cause of pneumonia but esp think of PCP, atypical pneumonia, measles giant-cell pneumonitis

Answer 130

pertussis - 1-2wks nasal discharge, conjunctivitis, malaise, dry cough, fever, sore throat; then for 1-6wks og paroxysms of gasping coughs, more common at night and maybe triggered by eg cold; sometimes mucous plugs or watery secretions, rarely other chest signs; can get cyanosis during attacks, post cough vomiting; sweating attacks and cough syncope in adults; suspect if acute cough 14 days or more with one plus of paroxysms, inspiratory whoop, post cough vomiting, undiagnosed apnoeic attacks in children (may be only symptom in young children); 3mo gradual return to normal but can be retriggered by other resp infections; notify phe if suspect; admit if 6mo or less and unwell, significant breathing difficulties or complications otherwise if onset in last 21 days give clarithro, erythro if pregnant woman; no work or school for 48hrs; proph antibiotics for close contacts that are unimmunised infants, women at 32weeks+ gestation, people who work or live with infant too young to be vaccinated croup - sudden onset barking cough, stridor, recession, usually worse at night; may have prodrome of urti for up to 48hrs before; hoarse voice; resp distress or failure sometimes; admit if these two or sternal recession at rest, high fever, resp rate >60, CHD, no wet nappy for 12 hours, <3mo O2 and oral dexamethasone while waiting for ambulance; otherwise single dose oral dexameth, should resolve within 48hrs, tell parents take to hosp if admission criteria inc continuous stridor or recession); paracetamol/ibuprofen; westley criteria are used to stratify croup severity and this can help guide mx

Answer 131

pneumonia - coarse crackles, tachypnoea infective exacerbation of asthma - wheeze and cough viral induced wheeze - under 5yo, wheeze and no history of asthmatic wheeze bronchiolitis - up to 2yrs, peak incidence 3-6mo; tachypnoeic, cough, may wheeze, maybe fine crackles; urti prodrome of 1-3 days; cough and tachyp (or chest recession, or both) and wheeze (or crackles, or both); may have fever (though if >39 consider pneumonia), poor feeding, periods of apnoea; snotty/coryza often, reduced feeds often; admit if high work of breathing, cyanosis, sats <92, looks unwell, apnoeas, RR>60, feeding <50% of normal, reduced wet nappies; support with O2/vapotherm/NG feeds if admitted, otherwise home +/- nasal saline drops to clear nose, feed little and often; tell parents 3-5 days peak (so will get worse before gets better), ibuprofen/paracetamol to relieve fever, dont smoke indoors, keep eye on kid for more serious things note transmitted upper airway sounds can sound like wheeze cough, signs of infection, clear chest consider: otitis media, URTI, pharyngitis, rhinitis, bronchitis, croup

Answer 132

Viral infection. 75% due to Respiratory syncytial virus (RSV).  Affects children < 2 years but more common (1 in 3 infants) in < 12 months. 2-3% of all infants require hospitalization with bronchiolitis.  Common for history of contact with older sibling or adult with cough and cold.  Results in small airway oedema and increased mucous and debris in airways, leading to plugging and atelectasis.  Admission is for o Oxygen therapy and/or supplementary feeding via NG tube and/or respiratory support Risk of more severe disease / requirement for respiratory support Infants < 3 months, particularly neonates (< 4 weeks) Prematurely born infants Infants with significant co-morbidities (chornic lung, heart, immunoded, or neruomusc disease) Typical history of coryzal prodrome lasting 1 – 3 days, followed by: - Persistent cough, and - Either tachypnoea or chest recession, or both, and - Wheeze or crackles on chest auscultation, or both  Young infants may present with apnoea without other clinical signs.  May cause a fever (usually < 39C).  Consider alternative diagnoses – Fever > 39C and/or persistent focal crackles – ?pneumonia – Persistent wheeze without crackles or recurrent episodic wheeze or personal or family history of atopy, especially in > 1 years - ?VIW  Symptoms often deteriorate over the first 72 hours, peak between 3 – 5 days. The cough resolves in most infants by 3 – 4 weeks. If O2 sats persistently > 90% in > 6 week old, or 92% < 6 weeks old, feeds > 50% normal, if any mild work of breathing improves in 4 hour observation, and no risk of severe disease, may go home if worse than these criteria then  Admit  Humidified O2 via Head box or heated humidified high flow nasal cannula oxygen (HFNC or “Optiflow”) to keep saturations >94 - 97%.  Consider CPAP or BLPAP if O2 requirement >40%  Nasal suction  Stop feeds, IV fluids.  Apnoea monitor if <8 weeks of age Maximal accessory muscle use. Poor respiratory effort. Apnoea. Unable to maintain oxygen saturations despite oxygen supplementation Listless, Exhausted, cyanosis Then urgent senior review, may need intubation and ventilation Do not routinely perform chest x-ray in children with bronchiolitis  Changes on x-ray may mimic a pneumonia and should not be used to determine the need for antibiotic -> and secondary bacti infection is rare All admitted patients should have both a SARS-CoV-2 swab and a full respiratory viral panel throat swab Consider capillary blood gases in those with impending respiratory failure or severe worsening respiratory distress e.g. persistent oxygen requirement > 50% Clear Nasal passages:  Saline nose drops, especially before feeding.  Suction can be used if signs of respiratory distress from nasal blockage or presenting with apnoea Nasogastric feeds should be considered in infants with moderate disease, those who cannot maintain oral intake due to increase RR up to 70/min, vomiting or increased work of breathing.  In infants with severe disease, RR > 70/min, or persistent vomiting, IV fluids are required – use 0.9% sodium chloride + 5% glucose at 2/3rds maintenance Discharge criteria for children recovering from bronchiolitis:  Maintaining saturations in air AND minimal recession for > 4 hours (including during sleep) o > 90% for children aged 6 weeks and over o > 92% for babies under 6 weeks or for any age child with underlying health conditions  Feeding orally >70% of their daily fluid requirements  Parent / carer advice leaflet, with verbal highlighting of red flags / key safety information

Answer 133

palivizumab (synagis) provides passive immunisation, given im into thigh once a month between oct and feb for high risk babies esp ex-prem

Answer 134

due to fat-containing products accruing in the distal airways and alveoli, leading to an inflammatory reaction that hinders gas exchange. It can be organized into two categories, exogenous and endogenous, based on the source of lipids In the past, the most common exogenous agents included mineral oil-based laxatives and nasal decongestants. Other causes include inhalation of petroleum jelly used as a lubricant, i.e., tracheostomy care; incidence growing due to vaping which often contains oils, and inhaling THC containing oils endogenous due to release of fat/cholesterol into lung due to dyslipidemia, lung inflam, lung infection, or malignancy can be acute or chronic, and presents with standard resp sx like SOB, cough, hypoxia, haemoptysis; b/l infiltrates on imaging and diffuse crackles; be highly suspicious in pt with acute lung disease and no obvious comorbidities; characteristic appearance on CT, will need standard resp workup including sputum and blood culture, bloods, lung function tests etc to rule out ARDS, pneumonia, fibrosis, lung cancer etc; bronchoscopy to diagnose you can see this in kids and babies - think if pneumonia/whatever and not getting better; one reason is aspiration of oil based foods when on ketogenic diet

Answer 135

Heart failure Valvular disease Angina Pulmonary embolism Pulmonary hypertension Pneumonia, PTX, asthma, COPD Anaemia Obesity Anxiety Pleural effusion, aspiration Phrenic nerve palsy Respiratory muscle weakness SVCO Fever

Answer 136

most important clinical point with regard to tracheal tugging is detecting upper airway obstruction, which frequently is seen in patients with laryngomalacia, the most common laryngeal disease of infancy. However, tracheal tugging also can appear in respiratory distress due to other causes, even if the primary problem is not associated with the upper airway. In such cases, tracheal tugging signifies a marked increase in respiratory work, leading to more dynamic diaphragmatic contractions that create a downward movement as seen in cases of upper airway obstruction. For this reason, whenever tracheal tugging is observed, clinicians should be aware of an emergency condition, such as upper airway obstruction or respiratory distress also olivers sign, tracheal tug in systole, due to thoracic aortic aneurysm pushing down the left main bronchus

Answer 137

Airways Start at the top in the midline and review the airways. trace down the trachea to the carina is it straight and midline? is there any narrowing? trace down both main bronchi is the carina wide (more than 100 degrees)? is there bronchial narrowing or cut-off? is there any inhaled foreign body? Breathing Look for lung and pleural pathology. both lungs should be well expanded and similar in volume can you count 10 posterior ribs bilaterally? is one lung larger than the other? compare the apical, upper, middle and lower zones in turn are they symmetrical? are there areas of increased density? trace the lung vessels do they branch out progressively and uniformly? can you see the retrocardiac and retrodiaphragmatic lung vessels? are there extra lines in the periphery that aren't vessels? trace the lateral margins of the lung to the costophrenic angles are the costophrenic angles crisp? trace the hemidiaphragms in to the vertebra can you see the whole of the hemidiaphragm? trace the cardiac borders can you clearly see the left and right heart border? can you see the descending aorta? Circulation Look at the heart and vessels (systemic and pulmonary). check the cardiac position is 1/3 to the right and 2/3 to the left? assess cardiac size is the cardiothoracic ratio < 50%? check the position and size of the aortic arch and pulmonary trunk check the width of the upper mediastinum look at the hilar vessels can you see them clearly on both sides? are they at a similar height? can you see a preserved hilar point bilaterally? Disability Check for any bony pathology (fracture or metastasis). trace along each posterior (horizontal) rib on one side of the chest is there a fracture or abnormal area? repeat with the other side of the chest now trace lateral and anterior ribs on the first side repeat on the other side now, check the clavicles and shoulders can you trace around the cortex of the bones? finally the check the vertebral bodies are they all rectangular and of a similar height? can you see 2 pedicles per vertebral body? are there disc spaces? Everything else Review the upper abdomen, soft tissues and take a look at some final check areas. is there free gas under the diaphragms? is there subcutaneous emphysema? is the gastric bubble in the correct place? is there a hiatus hernia? is there an absent breast shadow? are there any surgical clips? check again... are the lung apices clear? is there any retrocardiac or retrodiaphragmatic pathology?

Answer 138

airway, bones (and surgical emphysema), cardiac (<50% if PA and <60% if AP aka can be slightly bigger in AP films), diaphragm, effusions inc costophrenic angle check, fields of lungs, fissures of lungs, and foreign bodies (surgical clips, pacemakers etc), great vessel shape/size and gastric bubble, hila and mediastinum, then give impression

Answer 139

A - alveolar oedema (bat wing opacities) B - Kerley B lines C - cardiomegaly D - dilated upper lobe vessels E - pleural effusion

Answer 140

can't normally say where an opacity is on CXR, but can if borders lost between structures as this means they're touching (so technically is more of a loss of silhouette sign) left heart border - lingula right heart border - right middle lobe left hemidiaphragm - left lower lobe right hemidiaphragm - right lower lobe aortic knuckle - left upper lobe/middle mediastinum right paratracheal stripe - right upper lobe/anterior mediastinum

Answer 141

right border is RA, SVC and maybe IVC can contribute left border is formed by left ventricle and left atrial appendage, then above that aortopulmonary window and then aortic knuckle

Answer 142

breast, colorectal, renal*, uterine leiomyosarcoma, head and neck SCC in kids - rhabdomyosarcoma, osteosarcoma/ewings sarcoma, wilms tumour, neuroblastoma others inc choriocarcinoma, melanoma, testicular, thyroid, pancreatic

Answer 143

left hilum is normally slightly higher than right; if either is lower than normal could be pulled by lobar collapse/atelectasis, equally could be pulled upwards by upper lobe collapse; also mass effect bilat symmetrical hilar enlargement - maybe sarcoidosis, TB, mycoplasma, heart failure, lymphoma, pulmonary hypertension unilat - suspicious for lung/breast cancer asymmetric hilar enlargement

Answer 144

luminal - aspirated foreign material, mucus plug (eg asthma, ABPA, CF), endobronch mass, misplaced endotrach tube mural - lung cancer extrinsic - compression by adjacent mass (neoplasm or lymphadenopathy) pt will be breathless, may have pleuritic pain, cough, haemoptysis, wheeze

Answer 145

mediastinum can be partitioned into three compartments from anterior to posterior, which is best viewed on the lateral film. The anterior compartment contains the heart, thyroid, and thymus. The middle mediastinal compartment includes the esophagus, trachea, and many lymph nodes. The posterior compartment is the paravertebral area and is primarily composed of the vertebral column measured mediastinal width greater than 6 to 8 cm Anterior Retrosternal goiter Thymoma Germ-cell tumor Lymphadenopathy (lymphoma) Middle Aortic arch aneurysm Dilated pulmonary artery Tracheal lesion Posterior Esophageal lesions Hiatal hernia Descending aortic aneurysm Paraspinal abscess

Answer 146

most likely viral URTI but could be asthma (over 1yo, nocturnal), croup (barking, stirdor), pneumonia (difficulty breathing), bronchiolitis (diff breathing and <6mo), gord (after food), whooping cough (in bouts, no whoop if v young or old) dont miss: inhaled foreign body (abrupt onset, unilat signs), epiglottitis or tracheitis (sick app, stridor)

Answer 147

most likely asthma (over 5yo) but could be bronchiolitis (<6mo), viral pneumonia, virus induced wheeze dont miss inhaled foreign body (unilat) or mucous plugging

Answer 148

most likely: viral URTI (coryza, red throat or TM) could also be: UTI (oft no urinary sx), other viral infections, bacterial URTI, chest infection, non-infectious (kawasaki, antibiotic fever, VTE) dont miss: sepsis, meningitis

Answer 149

most likely viral urti (any time), gord (soon after feed), any cause of fever, any cause of cough (vomit after coughing) could also be gastroenteritis dont miss obstruction or vomiting due to v sick child (inc look for dehydration signs)

Answer 150

most like asthma if >5yo w wheeze and prolonged expiration or bronchiolitis if <6mo w wheeze and crackles but could also be pneumonia, pulm oedema (hepatomeg, sweaty, tachycard) dont miss inhaled foreign body (abrupt onset and unilat), croup or epiglottitis (stridor), or DKA (tachypnoea main sign) acoustics of stridor may be explained by Bernoulli's principle, which states that as the speed of a moving fluid increases, the pressure within the fluid decreases. When airflow is forced through a narrowed tube, a local area of low pressure creates a vacuum effect distal to the narrowing and the airway collapses

Answer 151

resp causes - congen or acquired lung problems, congen or acquired obstruction congen cyanotic heart disease lack of resp drive - seizure, CNS malform or infection, drugs neuromusc problems age of baby, when/how came on, how is child breathing, what was baby doing when colour change, any other sx, any prev breathing problems? poor feeding, fever, sleepiness? any FH of heart problems? how was pregnancy and labour? rapid A-E assessment including check femoral pulses ix - ABG, CXR, nitrogen washout test, ECG, echo if cardiac cause likely and timeframe fits PDA closing then give PGE2 infusion, control temp, acidosis, blood glucose; is if is cardiac go further - is it VATER? is it some syndrome like noonan/down also note resp causes of cyanosis cause more florid resp sx as acidosis and hypercap stimulate ventilation more than hypoxia alone does (latter what you'd get with cardiac cause, though may also get an acidosis over time due to lactate)

Answer 152

the “received wisdom” taught in clinic and on the wards is that infants with bronchiolitis do not respond to salbutamol because they do not have β2-adrenoceptors bronchiolitis is a clinical diagnosis, beginning with an upper respiratory tract infection followed by signs of respiratory distress, a harsh cough, bilateral crackles, air trapping and wheeze, and is caused by infection and inflammation of the bronchioles. In contrast, children over 1 year to 18 months who have wheeze are more likely to have acute bronchospasm. We recognise several subtypes of this episodic pre-school wheeze, including viral-induced wheeze, multi-trigger wheeze and post bronchiolitis wheeze infants <12 months of age with wheeze predominantly have bronchiolitis, and do not respond clinically to salbutamol It is a myth that there are no β2-adrenoceptors in the developing lung. In fact, radio-pharmacological studies have demonstrated pharmacologically functional adrenoceptors in mammals on day one of life: β2-adrenoceptors are clearly found in bronchial smooth muscle, in addition to pulmonary vasculature and type II alveolar cells Henderson et al. administered histamine to 40 infants, and randomly allocated infants to receive either salbutamol or saline. They found that salbutamol induced a more rapid recovery of maximal flow rates, in contrast with saline controls. These studies therefore demonstrate that β2-adrenoceptors are functional in the infant lung infants <1 year of age have functional β2-adrenoceptors within the lung. The reason for the lack of improvement with β-agonists in wheeze associated with acute bronchiolitis, is that bronchiolitic wheeze is caused by mucous obstruction and airway oedema at the level of the bronchioles, rather than due to muscular constriction (bronchospasm) at the level of the bronchi. It is therefore unsurprising that wheeze in bronchiolitis does not resolve with β-agonists

Answer 153

sleep-related disorder in which the effort to breathe is diminished or absent, typically for 10 to 30 seconds either intermittently or in cycles, and is usually associated with a reduction in blood oxygen saturation; it can result from chiari malform resp control centre fails to give signal to inhale, neurological feedback mechanism that monitors blood levels of carbon dioxide and in turn stimulates respiration fails to react quickly enough to maintain an even respiratory rate, allowing the entire respiratory system to cycle between apnea and hyperpnea Drops in blood oxygen levels that are severe but not severe enough to trigger brain-cell or overall death may trigger seizures even in the absence of epilepsy, and can trigger seizures in those with epilepsy that is well controlled; looks different from OSA in that no effort is made to breathe also Congenital central hypoventilation syndrome (CCHS), often referred to by its older name "Ondine's curse," is a rare and very severe inborn form of abnormal interruption and reduction in breathing during sleep. This condition involves a specific homeobox gene, PHOX2B, which guides maturation of the autonomic nervous system; Children who have it must have tracheotomies and access to mechanical ventilation on respirators while sleeping, but most do not need to use a respirator while awake; can survive with it if use this technology

Answer 154

Some babies can take a pause in their breathing for up to 10 seconds or a few seconds longer. Their next few breaths may be fast and shallow. Then they breathe steadily again tends to occur during sleep, but can occur while awake, and is a form of central sleep apnoea normally stops by 6mo

Answer 155

trauma to phrenic nerve/diaphragm from surgery or injury, compression of phrenic nerve (spondylosis, tumour, chest tube, sclerosing mediastinitis), neuropathy (DM, MS), inflam (herpes, zika, lyme disease, sarcoidosis, amyloidosis), spread of LA from nerve block may be asympt or dyspnoea eg on exertion or even at rest; hypovent and hypercarb possible, and may be fatigued CXR often shows, and can do eg PFTs; may be bilat but usually unilat, lots of other ix like EMGs poss but usually not needed treat cause, watch and wait; can ventilate if needed; surgical plication if doesn't improve with time (eg 6mo) to prevent paradoxical movement on inspiration (improves exercise tolerance, dyspnoea etc), can do phrenic nerve pacing

Answer 156

the factors which lead to P-Hyp and the clinical conditions associated with these factors are: *Inadequate intra-thoracic space: Congenital diaphragmatic hernia (CDH); intrathoracic tumors; pleural effusions *Prolonged oligohydramnios: Renal agenesis; prolonged rupture of fetal membranes *Decreased or absent fetal breathing movements: CNS lesions; phrenic agenesis With P-Hyp, there is inadequate pulmonary parenchymal tissue and pulmonary blood flow for normal gas exchange. CLINICAL FEATURES of P-Hyp include: *Immediate respiratory distress with tachypnea, cyanosis, retractions, hypercarbia, acidosis *With specific conditions, there are typical clinical signs, for example: -CDH: scaphoid abdomen -Oligohydramnios: “Potter’s facies,” arthrogryposis -CNS lesions: other signs of abnormal CNS function If there is concern that P-Hyp is present, follow the guidelines below in addition to basic resuscitation technique: intubate and ventilate Use low ventilatory pressures (PIP <25 cmH20) and high rates (60-90/min) with very short Ti Obtain CXR Place pre- (right hand) and post-ductal (lower extremity) saturation monitors to help evaluate for pulmonary hypertension complicating P-Hyp. *Consider early use of inhaled nitric oxide therapy if known/suspected CDH: Do not use bag and mask ventilation. This will cause distension of stomach and small bowel (which are in the chest) and worsen respiratory status. Insert NGT to decompress stomach Notify surgeons immediately

Respiratory Flashcards

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