1
Q
Heiner Syndrome
A
Cows milk protein induced pulmonary haemosiderosis. Haemosiderosis ( haemoptysis, IDA, lung fibrosis) and chronic sinusitis and OME
2
Q
Bilateral bilateral lymphadenopathy
A
TB LYMPHOMA SARCOID
3
Q
EMBRYOLOGY
A
Embryonic – up to week 5 • Lung bud grows out from the fetal foregut • Single tube branches into two main bronchi Pseudoglandular – weeks 6–16 • Airways grow by branching (out to terminal bronchioles) • Cartilage and lymphatics appear from 10 weeks onwards • Cilia appear •Pulmonary circulation develops, arteries arising from the sixth branchial arches Canalicular – weeks 17–24 • Conventional architecture of the lung appears • Thinning out of distal cells in preparation for gas exchange • Further development of arterial circulation, and appearance of venous system • Surfactant synthesis begins • Lung fills with fluid (lack of fluid at this and later stage, e.g. with renal agenesis, leads to pulmonary hypoplasia) Alveolar sac – weeks 24–40 • Formation of the acinus (respiratory bronchioles, alveolar ducts and alveoli) • Cell differentiation into type I and II pneumocytes • Type I: • >90% of alveolar surface • Major gas-exchanging surface • Type II: • Thought to be the progenitor cell for type I cells • Produces surfactant which maintains surface tension and prevents alveolar collapse during respiration • Surfactant-associated proteins A and D (hydrophilic) involved mainly in innate immunity • Surfactant-associated proteins B and C (hydrophobic) important for surface tension
4
Q
What are causes of false-positive and false-negative sweat tests?
A
- False-positive: adrenal insufficiency, nephrogenic diabetes insipidus, atopic dermatitis, familial cholestasis syndrome, Klinefelter’s, GSD, mucopolysaccharidosis, G6PD - False-negative: oedema, malnutrition, mineralcorticosteroid use
5
Q
Discuss the differences between: - Congenital lobar emphysema - Bronchogenic cyst - CPAM - Pulmonary sequestration
A
- CLE: hyperinflation due to obstruction of developing airway, usually LUL, air-filled, herniates across mediastinum, compression of contralateral lung, usually symptomatic neonate. Usually symptomatic. Associated CHD. Resect only if symptomatic
- Bronchogenic cyst: usually mediastinal but can be anywhere, fluid-filled, sharply marginated. Risk infections. Remnant of primitive foregut
- CPAM: abnormality of branching morphogenesis of the lung, cystic and adenomatous elements, connection with tracheobronchial tree, can arise anywhere along it, blood supply from pulmonary circulation. Often asymptomatic, usually lower lobes. Resection as malignancy risk -
Sequestration: non-functioning mass of lung tissue, lacks communication with tracheobronchial tree, receives arterial blood supply from systemic circulation, usually lower lobes, dull to percuss with decreased BS, predispose to recurrent infections. Usually asymptomatic at birth. May have have continuous or systolic murmur over back. Surg resection or coil embolisation
6
Q
What is the physiological dead space?
A
- The anatomical dead space + alveoli that aren’t involved. - The volume of the lung that does not eliminate CO2
7
Q
Obstructive lung function tests
A
- Asthma, Bronchiectasis, Emphysema, Cystic Fibrosis - FEV1/FVC < 80% - FVC normal or decr - FEF 25-75 decreased - TLC normal or increased
8
Q
Restrictive lung funcion tests
A
- Interstitial Fibrosis, Scoliosis, Obesity, Lung Resection, Neuromuscular diseases, Cystic Fibrosis - FEV1/FVC inc or normal, FVC decr, FEV1 decr - TLC decr - FEF 25-75 normal or decr
9
Q
Fixed upper airway obstruction
A
- Tracheal stenosis, bilateral vocal cord paralysis, goitre ‐ Inspiration and expiration are limited equally - Flattened curve on insp and exp
10
Q
Variable extrathoracic obstruction
A
‐ Limitation of inspiratory flow, flattened inspiratory loop ‐ e.g. Vocal cord paralysis, vocal cord dysfunction ‐ During expiration the vocal cords are passively blown aside ‐ During inspiration vocal cord moves passively with the inhalation and obstructs the glottis
11
Q
Variable intrathoracic obstruction
A
‐ Flattening of expiratory limb ‐ e.g. Tracheomalacia ‐ During expiration there is loss of support resulting in resulting in a narrow trachea and reduced flow ‐ During forced inspiration the negative pleural pressure holds the floppy trachea open
12
Q
Cause of low DLCO in obstructive disease?
A
Bronchiolitis obliterans, CF
13
Q
DLCO in restrictive lung disease - low and normal causes?
A
- DLCO helps in differential diagnosis of restrictive lung disease - Low DLCO with reduced lung volumes suggests ILD, sarcoid, pneumonitis, pulm fibrosis - Normal DLCO associated with low volumes is consistent with extrapulmonary cause of restriction – pleural effusion, obesity, neuromuscular weakness, kyphoscoliosi
14
Q
What is the FeNO, and causes of low and high tests?
A
- Fractional exhaled nitric oxide - Positive test suggests eosinophilic inflammation - Positive >35ppb = asthma, eosinophilic bronchitis, allergic rhinitis, eczema - Lower: smokers, CF/ciliary dyskinesia
15
Q
Discuss the different CFTR genes in CF
A
- Class I (3%) - no protein e.g. G542X. Stop mutation, nonsense, short protein which is deleted, therefore no function. - Class II (90%) - no traffic e.e F508del. Abnormal folding, trafficking defect, therefore no traffic to cell membrane. - Class III (5%) - no function. e.g. G551D. Gating defect, protein can get to cell wall but Cl- cannot get out of cell. - Class IV - less function - Class V - less protein - Class VI - less stable
16
Q
Modulator drugs in CF
A
- Class I (G542X) - nil - Class II (F508) - orkambi (lumacaftor and ivacaftor) >2yo in Aus, tricaftor (3 drugs, very new), symdeco (>12yo) - Class III (G551D) - ivacaftor - allows chloride through. >2yo in Aus. Can be used on homozygotes and heterozygotes. - Best results seen in tricaftor and ivacaftor - improved FEV1 > 10% (but inhaled TOBI also inc by 12%)
17
Q
Newborn screening process for CF
A
- Immunoreactive trypsinogen - picks up to 1% levels (pancreatic duct blocked so enzymes cannot get into GIT therefore absorbed into bloodstream) - CF gene panel -> for 5 positive screens, 1 has true CF. 8% CF is missed (either IRT not in top 1% or has other gene mutations) - Sweat test: normal <30, abnormal >60, indeterminate 30-60
18
Q
Most common CF pathogens in paeds population
A
- Staph > haemophilus > pseudomonas > MRSA > st. maltophilia - Pseudomonas increases through lifespan - MRSA increasing Aus > NZ
19
Q
CF most common chronic infection in a) early childhood and b) later childhood?
A
- A = staph aureus. Not yet routine prophylaxis in NZ. MRSA ~15%, increasing. - B = pseudomonas aeruginosa. - If well then use PO ciprofloxacin + inhaled tobramycin - If unwell then use IV ceftazidime (NZ) or meropenem and tobramycin (Aus). - Causes intense inflammation, develops biofilm, difficult to eradicate. Associated with poorer prognosis and lower survival. - If chronic pseudomonas (>50% samples of 4/year) then use azithromycin Mon/Wed/Fri
20
Q
Discuss bronchiolitis obliterans
A
- After an insult to the lower respiratory tract and leads to chronic obstructive lung disease from fibrosis of the small airways - Post viral: adenovirus, mycoplasma or inflammatory disease (e.g. JIA, SLE) or graft vs. host disease or lung transplant - Chronic cough, SOB, sputum production and wheeze. Hypoxemia and crackles - CXR: variable, hyperlucency and patchy infiltrates. - PFTS: obstructive, variable response to salbutamol -Tx: supportive, treat infections, O2, may require steroid course, immunosuppressants (tacrolimus, cyclosporine, macrolide antibiotics)
21
Q
Discuss CF-related diabetes
A
- Will develop with time - Often deterioration in lung function associated - Decreased insulin production + insulin resistance + normal effect of insulin reduced - Screen with OGTT and HbA1c (unreliable) and confirm with continuous glucose monitoring - Some will require small amount of insulin
22
Q
Airway clearance in CF
A
- Pulmozyme - recombinant human deoxyribonuclease I - an enzyme which cleaves DNA, reduces sputum viscoelasticity, improves lung function and reduces exacerbations - Hypertonic saline nebs 7% - draws water into airways and thins secretions, improves FEV1 and reduces exacerbations - Physio - Exercise
23
Q
Poor prognostic factors in CF?
A
- Malnutrition - Pseudomonas - B. cepacia - CF-related diabetes - Frequent exacerbations - Female, especially teenage
24
Q
Most common chronic infection in bronchiectasis?
A
- Haemophilus influenzae. Note: staph is rare, therefore if present need to check for CF - Moraxella and strep pneumoniae intermittent
25
Discuss the steps of asthma therapy
- 1: SABA (salbutamol) - 2: SABA + low dose ICS (fluticasone, flixotide) - 3: \<5y - leukotrine receptor antagonist (montelukast) \>5y - SABA + ICS + LABA (seretide, salmeterol) \>12y - SMART therapy with symbicort (budesonide/formeterol) - 4: increase ICS dose, add montelukast \>5y - 5: increase ICS dose -\> always check compliance, spacer, lung function tests - LABA monotherapy unsafe(theory reduction in B2 density). Not used \<4y
26
Discuss the emryological lung stages
- Embryonic (26 days to 6 weeks gestation) - Pseudoglandular (6-16 weeks) - major lung elements, except for those required for gas exchange (e.g. alveoli) have appeared. - Canalicular (16-26 weeks) - bronchial lumens enlarge and lung tissue becomes vascularised. Bronchioles and alveolar ducts develop from terminal bronchioles. - Saccular (26 weeks to birth) - specialised cells appear (type 1 and type II alveolar cells) and produces surfactant. - Alveolar (birth to 3 years) - terminal saccules, alveolar ducts, and alveoli increase in number.
27
Diagnostic findings in primary ciliary dyskinesia?
- Abnormal cilia - absent dynein arms, translocation of central tubules - Ciliary beat frequency low - Low exhaled nasal nitric oxide
28
Use of osteltamivir
- Neuraminidase inhibitor - For influenza - Reduces viral spread in airways if given in first 48 hours of symptoms
29
Defects in IF-gamma and IL-12 pathways predisposes to
MYCOBACTERIAL infections (TB)
30
Mantoux cut-off values
- \>15mm positive in everyone - \>10mm if recent immigrant, children \<4y, high risk - \>5mm if HIV, recent TB contact, abnormal CXR - False negatives in young, immune deficiency, HIV/flu/measles - T-cell mediated reaction
31
Treatment of TB
- Chemoprophylaxis in infection - isoniazid 6m +/- rifampicin 3m - Chemotherapy (disease) - isoniazid + rifampicin for 6m + pyrazinamide + ethambutol for first 2m - Total 12m if meningitis. - 6-8 weeks corticosteroids if meningitis/pericarditis/miliary TB - Need sputum culture of gastric aspirates prior to starting therapy - AFB, Ziehl Neelsen stain
32
Side effects of TB meds
Peripheral neuropathy, liver derangements, orange urine/tears (rifampicin) visual disturbance (ethambutol)
33
Discuss pneumocystis jiroveci
- Tachypnoea, resp distress, fever, bilat crackles, hypoxia - CXR: bilat interstitial and alveolar shadowing, ground glass/crazy paving on CT - Yeast-like fungus - Dx: bronchoalveolar lavage, blue/silver stain - Causes: SCID, HIV, DiGeorge, hyper IgM, oncology - Tx: high dose cotrimoxazole (causes pancytopenia, fever, rash), +/- steroids, surfactant if I+V, prophylaxis post treatment - Can get dual infection with CMV as cause of pneumonitis
34
Discuss lymphocytic interstital pneumonitis
- Seen in HIV or immunodeficiency, EBV - Marked lymphadenopathy, parotid hyperplasia, HSM, falling CD40 count, chronic cough, hypoxia, clubbing, ILD - Tx: nil if well, may respond to steroids
35
Discuss pulmonary haemosiderosis
- Triad of iron-deficiency anemia, hemoptysis, and multiple alveolar infiltrates on chest radiographs - Repeated pulm haemorrhage leads to accumulation of hemosiderin in alveoli - Can have haemoptysis, episodic fever, SOB, wheeze - CXR: normal or patchy - Diagnosis via BAL - hemosiderin-laden macrophages - Tx: acute O2, transfusion. Chronic - steroids, hydroxychloroquine, immunosuppression - Can be associated with SLE, GPA, Goodpasture's, cardiac abnormalities, or be idiopathic - Can be associated with Cow's milk hypersensitivity
36
What stage of sleep does OSA worsen in?
REM - irregular breathing, inc upper airways resistance, low tone, decreased tidal volume
37
When do parasomnias occur?
- Partial awakening from N3 deep sleep - Usually 60-90 min after going to sleep - 1 or 2 per night - Usually +ve family history
38
Treatment of periodic limb movement?
- Associated with partial iron deficiency in CNS - treat with iron to keep systemic ferritin \>50-75 to force iron into brain
39
Periodic limb movements vs. restless legs
- PLMD: repetitive (usually every 20 to 40 sec) twitching or kicking of legs or arms during sleep. Interrupted nocturnal sleep or excessive daytime sleepiness. - RLS: irresistible urge to move the legs, arms, or, body, usually accompanied by paresthesias +/- pain. Inc when sedentary, evening time. To relieve symptoms, patients move the affected extremity by stretching, kicking, or walking. Difficulty falling asleep, repeated nocturnal awakenings, or both.
40
Discuss narcolepsy
- Primary hypersomnia
- Due to deficiency of hypocretin (orexin) which normally stimulates the cortex to be awake
- Associated with cataplexy, sleep paralysis, hypnagogic hallucinations
- Ix: decreased hypocretin-1 levels in CSF,
HLA DQB1\*0602 +ve
- Tx: routine scheduled naps and sleeps, stimulants, tricyclics or SSRIs for cataplexy
41
Discuss congenital hypoventilation syndrome
- Ondine's curse - Presents in infancy with apnoea and cyanosis - Hypoventilation, worse in sleep - Absent response to hypercarbia and hypoxia when awake and asleep - Decreased tidal volume - Ventilation better awake and in REM (opp. from usual) - PHOX2B mutation - Usually require trache-ventilation (older can have NIV) - Developmental delay if recurrent hypoxia - Also associated with: failure to develop fever, sudden death (arrhythmia), neural crest tumours, Hirschsprung
42
Describe the different types of sleep and what you might see on a PSG:
N1 - sleep transition N2 - light sleep with spindles (look like fuzzy caterpillars) and K complexes (up then down waves) N3 - slow wave and deep sleep, small EMG REM - lots of eye movement, very small EMG
43
What paO2 corresponds with sats of 50%, 70%, 80%, 90% and 97% on pulse oximetry?
50% = PaO2 27mHg 70% = PaO2 40mmHg 80% = PaO2 45mmHg 90% = PaO2 60mmHg 97% = PaO2 96mmHg
44
Which factors shift the oxygen dissociation curve to the right?
To the right = more free oxygen available - increased temperature - increased PCO2 - decreased pH - increased 2,3-diphosphoglycerate.
45
Which factors shift the oxygen dissociation curve to the left?
To the left = higher affinity for O2, less available in tissues - decreased temperature - decreased PCO2 - increased pH - decreased 2, 3 diphosophoglycerate (seen in Fetal Hb)
46
Definition of pulmonary arterial hypertension:
- Mean PA pressure ≥25 mm Hg WITH - Normal pulmonary capillary wedge pressure ≤15 mmHg (i.e. normal venous pressure) - Increased PVR index ≥3 Wood units/m^2
47
What is Kartagener Triad?
- Situs inversus totalis (50% of patients with primary ciliary dyskinesia have it; 25% of patients with situs inversus have PCD) - Chronic sinusitis - Bronchiectasis
48
What is FVC 25-75% useful for?
- Indicator of mild airway obstruction - Small airways disease
49
What genetic/syndromic anomalies are associated with choanal atresia?
-CHARGE syndrome -Treacher-Collins -Kallmann syndrome -VA(C)TER(L) association -Pfeiffer
50
Poor technique clues on flow-volume loop...
- Abnormal dips in expiratory curve - early starts of inspiratory curve
51
What happens to the FEF50%:FIF50% with - variable extra-thoracic lesions - variable intra-thoracic lesions - fixed obstruction
- variable extrathoracic lesions INCREASE ratio (\>1) - variable intrathoracic lesions DECREASE ratio (\<0.2) - fixed obstructions (intrathoracic or extrathoracic) ratio ~1
52
Ivacaftor is used for which class of CF mutation?
Class 3 G551D
53
Cytokines produced by Th2 in asthma
IL-4/5/13
54
DMD thresholds in FVC
\<60% is SDB \<20% daytime respiratory failure Polysomnography (sleep study) to assess
55
Scimitar syndrome, or pulmonary venolobar syndrome , is...
a rare congenital heart defect characterized by anomalous venous return from the right lung
56
Surfactant def accounts for 10% ILD what are the four genetic def
Protein B (SPB) Protein C ABCA3 Thyroid transcription factor related disease
57
Round pneumonia under 8y most commonly due to
Strep pneumoniae Proposed underdevelopment of pores of kohn and canals of lambert
58
What are the volumes and capacities of the lungs?
- 4 volumes and 4 capacities. - Volumes do not overlap, capacities do. - Capacities are made up of different combinations of lung volumes. - Volumes: --\> tidal volume: amount of air moved in and out of lung during each breath. --\> expiratory reserve volume: amount exhaled with maximal effort after tidal expiration. --\> inspiratory reserve volume: amount inhaled with maximal effort following tidal inspiration. --\> residual volume: volume of gas remaining in the lungs after maximum expiration. -Capacities: --\> inspiratory capacity: amount inspired by maximum effort following tidal expiration (tidal volume + inspiratory reserve volume). --\> functional residual capacity: amount of gas in lungs following tidal expiration (expiratory reserve volume + residual volume). --\> Vital capacity: amount of gas moved with max inspiratory and expiratory effort (inspiratory reserve volume + tidal volume + expiratory reserve volume). --\>Total lung capacity: volume of gas occupying lungs after max inspiration (inspiratory reserve volume + tidal volume + expiratory reserve volume + residual volume).
59
What are the low dose ICS? - low dose achieves 80-90% max efficacy in children
Beclomethasone dipropionate (1/2 the strength of flixotide) = 200mcg/day Beclamethasone dipropionate ultra fine (same strength as flixotide) = 100mcg/day Fluticasone = 100mcg/day Budenoside (1/2 the strength of flixotide) = 200mcg/day
60
What are the STANDARD doses of ICS?
Beclomethasone dipropionate (1/2 the strength of flixotide)= 400-500ncg/day Beclamethasone dipropionate ultra fine (same strength as flixotide) = 200mcg/day Fluticasone= 200-250mcg/day Budenoside (1/2 the strength of flixotide)= 400mcg/day
61
What is the average tidal volume of a child?
approx 6-7ml/kg
62
What happens to the resistance when the radius is halved?
Resistance in respiratory disease is amount of pressure required to generate flow across of gas across the airway resistance is inversely proportional to the radius^4 therefore 1/2 the radius = increase the resistance by 16
63
What are the 2 main cytokines made by the alveolar macrophages that activate the inflammatory cascade?
Tumour necrosis factor- α Interleukin-1β
64
What is choanal atresia?
Most common congenital anomaly of the nose freq 1/1700 live births Can be unilateral or bilateral bony (90%) septum between the nose and pharynx Or can be a unilateral or bilateral membranous (10%) septum between the nose and pharynx Most cases are a mixture of bony and membranous atresia 50-70% of children have other congenital anomalies - particularly CHARGE syndrome 10-20% of patients with CA have CHARGE
65
What is CHARGE syndrome?
C- coloboma H- heart disease A- atresia chonae R- retarded growth and development or CNS anomalies or both G- genital anomalies, hypogonadism or both E- ear anomalies, deafness or both Mutations in the CHD7 gene which is involved in CHROMATIN organization
66
What can nasal polyps be a sign of?
Undiagnosed CF in child \<12 years (most common childhood cause of nasal polyposis) Chronic sinusitis and allergic rhinitis Aspirin sensitivity and asthma (called the Samter Triad. Uncommon)
67
What age group is a retropharyngeal abscess most common and why? Symptoms
3-4 years of age 65% with have had recent URTI sx Decreased as kids get older as retropharyngeal nodes involute after the age of 5 years Presents Fever, misery, decreased oral intake, drooling Sometimes can get stiff neck, sore throat, torticollis Muffled voice, stridor, resp distress, OSA May get bulging of posterior pharyngeal wall (\<50% in infants)
68
What are the DDX of upper airway obstruction?
Bacterial tracheitis Croup Epiglottitis Diptheritic croup (unimms. gray white patches on tonsils) FB Retropharyngeal or peritonsillar abscess Extrinsic compression from laryngeal web, vascular ring) Intraluminal obstruction from laryngeal papilloma, haemangioma (often recurrent sx) Angioedema allergic/anaphylactic/trauma Hyopcalcemic tetany (angioedema) EBV Tumours or malformations of the larynx Ingestion of hot liquids or caustic poisons
69
How to calculate Qp:Qs
Sat Ao - Sat MV/Sat PV- Sat PA Ao= aorta MV= mixed venous PV= pulmonary vein PA= pulmonary artery
70
Inspiratory stridor
Above glottic (VC) Laryngomalacia Supraglottic stenosis FB
71
Expiratory stridor
Below glottic FB Tracheomalacia
72
Biphasic stridor
Fixed obstruction (at glottis) Vocal cord pathology
73
Laryngeal webs
Seen in 22q11 deletion (65% have webs) 75% at glottic level
74
Oxygen dissociation to left ( higher affinity for O2 so less to tissues)
Alkalosis ( decrease H) Decrease PCO2 Hypothermia Decrease 2,3 DPG, fetal Hb
75
Oxygen dissociation curve to right ( less affinity for O2 so more release to tissue)
Acidosis (increased H) Fever Increased PCO2 Increase 2,3DPG, adult Hb
76
Flow volume loops
Flattened inspiratory loop = variable extra thoracic upper airway obstruction- vocal cord dysfunction (unilateral palsy), subglottic stenosis Flattened expiratory loop = variable intrathoracic upper airway obstruction - tracheomalacia Flat insp and exp loop = fixed upper airway obstruction - vascular ring / tracheal stenosis
77
Bilateral bilateral lymphadenopathy
TB LYMPHOMA SARCOID
78
Heiner Syndrome
Cows milk protein induced pulmonary haemosiderosis. Haemosiderosis ( haemoptysis, IDA, lung fibrosis) and chronic sinusitis and OME
79
Chylothorax cause and management
Chyle contains long chain fatty acids. Chylothorax refers to the presence of lymphatic fluid in the pleural space secondary to leakage from the thoracic duct or one of its main tributaries. A tear or leak in the thoracic duct causes chylous fluid to collect in the pleural cavity, which can cause acute or chronic alterations in the pulmonary mechanics. - Most frequently seen as a complication of cardiothoracic surgery in children, other cases may be associated with chest injury or intrathoracic malignancy (particularly lymphoma). In newborns, rapidly increased venous pressure during delivery may lead to thoracic duct rupture. Usually, the patient remains asymptomatic until a large amount of chyle accumulates in the pleural space. Symptoms include dyspnoea and tachypnoea with classical signs of a pleural effusion. Rarely bilateral and usually occurs on the right side. Thoracocentesis demonstrates chyle: a milky fluid containing fat, proteins, lymphocytes and other constituents. Fluid may be yellow or bloody. In new born infants who have not been fed the fluid may be clear. Fluid triglyceride levels are typically \>100mg/dL, with a ratio to serum triglycerides \>1.0. Chyle accumulates quickly and repeated thoracocentesis may cause malnutrition and caloric loss. Hypogammaglobulinaemia and poor cell mediated responses due to loss of T-lymphocytes may also occur. Spontaneous recovery occurs in \> 50%. Initial therapy includes enteral feeds with a low-fat or medium chain TG, high protein diet or parenteral nutrition. If chronic, ligation of the thoracic duct may be considered. The aim of nutrition is to reduce chyle production by instituting total parenteral nutrition or a fat-restricted oral diet supplemented with medium-chain triglycerides (as these are absorbed directly into blood). During digestion, the lymphatic system absorbs the LCTs. The lymphatic system does not, however, absorb MCTs. The MCT diet limits fat consumption to the MCTs, eliminating or greatly reducing the consumption of LCTs. This results in less fluid absorbed into the lymphatic cells and subsequently, less leakage of lymphatic fluid into the thoracic cavity, relieving the chylothorax.
80
81
Restrictive Spirometry
82
Fixed Upper Airway Obstruction
‐ Tracheal stenosis, bilateral vocal cord paralysis, goitre
‐ Inspiration and expiration are limited equally
83
84
85
Flow volume curve shapes
1. Obstructive
2. Restrictive
3. Mixed
4. Intrathoracic obstruction variable
5. Extrathoracic obstruction variable
6. Fixed upper airway obstruction
86
CF mutations and treatment options
87
CF newborn screening pathway ;
Number missed from screening?
IRT, if positive (top 1%)
Genetic screening for common mutations
If 2x mutations- CF diagnosed, have sweat test
1x mutation, either carrier or CFSPID, need sweat test
For 5 positive screen approx. 1 has true CF.About 8% missed
Sweat test
Sweat Chloride:
• \> 60mmol/L – CF
• 40‐59 mmol/L‐ probable/possible (new 30‐59) \< 6/12 of age
• \< 29mmol/L ‐ Normal
88
CF bacteria by ages
89
Treatment of pseudomonas in CF
Pseudomonas aeruginosa‐ most common chronic infection later childhood, teenage and adults
‐ Eradication procedures when first seen
‐ If well – usually oral ciprofloxacin, inhaled tobramycin
‐ If unwell – iv antibiotics (ceftazidime or meropenum & tobramycin) ‐
‐ Causes intense inflammation ‐ Develops biofilm
‐ Recurrent or chronic – add Azithromycin (Mon/Wed/Fri)‐ Associated with poorer prognosis and lower survival
If get sputum that says Pseudomonas resistant to Colisitin - need to outrule that is Burkholderia
90
NTM in CF
on Tuberculosis Mycobactrerium
‐ found in approximately 10 % of cystic fibrosis (CF) patients
* Mycobacterium avium complex (MAC)70% (M avium, M intracellulare and M chimaera)
* Mycobacterium abscessus‐16%
‐ but only a portion will develop NTM disease
‐ Tend to have nodular changes on chest CT scan
‐ Even if meet criteria for NTM lung disease may not necessarily require treatment – monitoring only
‐ Consider if Mycobacterium abscessus complex (MABSC), severe lung disease, and/or anticipated lung transplant
‐ Treatment complex and prolonged
‐ MUST CHECK BEFORE START AZITHROMYCIN FOR PsA and REGULAR MONITORING – if present stop azithromycin (may induce resistance to NTM with monotherapy)
91
ABPA in CF
Prevalence about 10%
Hypersensitivity reaction to spore bearing fungus
(spore size 2‐4 micron)
* Type 1 and 3 immune responses
* Risk factors: Age, Atopy (asthma), Chronic bacterial infection, Male, Pseudomonas, Poor lung function
Treatment:
• Oral steroids & oral antifungals (itraconazole)
92
Airway clearance in CF
Physiotherapy – positive expiratory pressure
Exercise
* rhDNase/Pulmozyme: recombinant human deoxyribonuclease I ‐ an enzyme which selectively cleaves DNA – hydrolysing DNA in sputum reduces sputum viscoelasticity – ↑ lung funcon, ↓ exacerbaon rates ‐ especially children with Pseudomonas
* Hypertonic saline nebs 7% saline – draws water into the airway – thins secretions – ↑ lung funcon, ↓ exacerbation rates
93
Poor prognostic factors in CF
Malnutrition
Pseudomonas aeruginosa
Burkholdaria cepacia
CF related diabetes
Frequent exacerbations
Female gender
Referral for transplantation:
* FEV1 ≤ 30% PREDICTED • PaO 2 \< 7.3 kPa
* Pa C02 \> 6.7 kPa
2 yr survival \< 50%
94
Most common cause chronic infection in bronchiectasis
Haemophilius Influenza
95
CFTR mutations and class
96
PHOX2b mutation associated with
Congenital Central Hypoventilation Syndrome
Neural crest tumours (Phaeo,neuroblastoma)
CCHS is associated with other ANS problems
– Failure to develop fever etc when unwell
– Cardiac arrhythmia and sudden death (holter/pacing)
– Hirschprung’s disease
– Neural crest tumors
– Iris problems (ophthalmol review)
Rx Lifelong ventilation
97
Respiratory condition that gets better with REM sleep
CCHS, worst in stage N3
98
Indicaitons for NIV in NMD
• Symptoms of chronic nocturnal hypoventilation or daytime respiratory failure/hypercapnoea
– Sleepiness, Fatigue/lethargy, Headache, Increased night waking
– Can be subtle
• Preventing recurrent admission
\*\*PSG and lungfunction are a guide not an indication
– Mortality was increased in one RCT that commenced NIV prior to symptoms
– Too early initiation will result in poor compliance/tolerance
99
Lung volumes / capacities
IC ; IRV ; TV ; ERV etc
Paeds
flashcards
Decks in class (25)
# Cards
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Resp99
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Neonate166
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Immunology37
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Cardiac20
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Gen Paed38
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Renal91
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Short cases108
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Endocrine98
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Haem96
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Oncology97
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Infectious Disease122
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Statistics36
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Cardiology187
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Emergency Medicine129
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Rheumatology/dermatology64
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Genetics229
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Metabolic124
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Gastroenterology235
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Cardiology COPY157
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Neurology267
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Derm16
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Nephrology219
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Psych AND Adolescent25
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Pharmacology56
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Pharmacology COPY145
