Resp and cardio Flashcards
1
Q
Effects of scoliosis on respiration
Management options
A
Respiratory effects = restrictive lung disease
- Rigid thoracic cage not allowing full expansion
- Decr insp muscle strength
- Reduced lung growth
Mx
- Bracing
- Spinal implants/distraction devices (rods allowing for growth)
- Spinal fusion (for severe scoliosis in adolescents)
2
Q
Cyanosis + scar if <5 or >5
A
<5 - hypoplastic L heart post BCPC repair
>5 - hypoplastic L heart post Fontan
3
Q
Cyanosis + no scar
A
Unrepaired TOF
4
Q
CF complications
A
- Lungs
- recurrent infection, inflammation
- reduced lung function
- pulmonary HTN
- OSA
- Cardiac
- R and L heart dysfunctino
- Cor pulmonale
- ENT and sleep
- Nasal polyps
- OSA
- Dental staining
- Liver
- CF related liver disease
- Liver transplant
- Pancreas
- Hyperglycaemia and CF related diabetes
- GI
- Malabsorption, malnutrition
- Mec ileus
- Constipation, DIOS, rectal prolapse
- Infertility
- No vas deferens in 99% of boys
- Female infertility impacted by malnutrition
- Mental Health
- Depression and Anxiety
- Medications
- Chronic steroids
- Chronic/recurrent abx
5
Q
CF diagnosis
A
- Antenatal diagnosis - family history
- Newborn screening - top % of IRT + genetics panel +/- sweat test (if additional evidence needed)
- Clinical presentation
- Mec ileus (presents before IRT result back generally)
- FTT
- Chronic resp infections/bronchiectasis
- Infertility in adults
6
Q
Benefits of modulators in CF
A
Reduce exacerbations, daily symptoms and improve QOL
Improve FEV1
Reduce treatment burden
- Ivacaftor - stabilises BSLs → reduction of insulin requirement
Side effect - monitor LFTs
7
Q
A
Cough
- Characteristics of cough (dry vs wet, how much sputum per day in tsp/TB/cup etc)
- Any haemoptysis?
- What type of airway clearance PT do they do? (chest wall percussion in younger kids to loosen mucus, PEP devices, oscillatory vests)
- Medications for airway clearance?
- Pulmozyme (mucolytics)
- Hypertonic saline and mannitol (mucus thinner)
- Bronchodilators (Ventolin)
- Compliance (BD) and technique
- Exercise
8
Q
Investigations to ask about in patients with CF
A
Lungs
- Lung function (FEV1) - know their baseline; big drop from baseline necessitates admission for tune up
- Chest CT (?present of bronchiectasis)
- Sputum micro (?presence of pseudomonas or MRSA)
- If pseudomonas: age at colonisation, previous attempts at eradication (methods), antibiotics for this (inhaled tobramycin, colistin, amikacin)
- NTM (non tb mycobacterium)
- ABPA (treat with steroids)
9
Q
Antibiotic questions to ask about in CF
A
What antibiotics do they take, route, when did they start
Any drug allergies and SEs (?resistance ?hearing loss with ahminoglycosides)
Can they take tablets?
10
Q
Next steps for CF kids if dropping FEV1 despite optimisation of meds/chest PT etc
A
Screen for causes outside the lung
- CF related diabetes
- Malnutrition
Ventilatory support (O2, bipap etc)
Lung transplant when FEV1 <30%, or life threatening haemoptysis, or resp failure
Palliative care
11
Q
Signs and mx of pancreatic insufficiency in CF
A
Diarrhoea, foul smelling
Faecal elastase screening
OGTT screening and HBA1C
Mgmt
- pancreatic enzyme replacement (Creon)
- supplementation of fat soluble vitamins
- endocrinology involvement +/- insulin
12
Q
Mgmt of malnutrition in CF
A
- Dietician and food diary
- Salt replacement
- Pancreatic enzyme supplements (creon)
- Fat soluble vitamin supplements
- Appetite stimulants
- High energy shakes
- PEG
- TPN
13
Q
Screening for CF related liver disease in children
A
Yearly liver USS and LFTs
14
Q
psychosocial things to ask about in CF
A
Children
- Education, CF knowledge
- Compliance and tablet taking
- Developmental concerns (ASD, ADHD)
Teens
- Compliance and treatment burden
- Nutrition and body image
- Sexuality, safe sex, fertility
- Smoking and vaping
- Mental health - depression, anxiety, mortality and life goals
- Transition and the future
Family
- CF knowledge, treatment and OPC burden
- Smoking
- Sibling impacts
- Family planning
- Financials
- Location (rural vs metro, access to healthcare resources)
Environment
15
Q
Other systems to ask about in CF
A
- Venous access, bloods (GAs, long lines vs ports)
- Needle phobia
- Immunisations - flu vax, covid >16yo, consider hep A and men B
- Endocrine
- DEXA scans for vit D deficiency
- Delayed puberty
16
Q
Support groups for CF
A
CF apps
CHIPS
CF australia
CF organisations can provide funding for CF equipment
17
Q
Respiratory consequences of NM disease (duchesses, SMA2) and screening/mgmt of these
A
- Inability to ventilate (hypoventilation +/- apnoeas): first nocturnal, then progression to daytime, then 24/24
- Screen with spirometry and PSG and daytime cap gas
- Mx: BiPAP
- Reduced lung capacity due to scoliosis and also collapsed lung from dependent positioning
- Screen: clinical exam and xrays
- Mx: scoliosis surgery and spinal fusion and aim to maintain ambulation as long as possible
- Inability to cough
- Screen: peak cough flow
- Mx: PT for airway clearance techniques and breathing techniques, BiPAP, cough assist
- Aspiration risk due to upper airway hypotonia
- Screen: clinical history
- Mx: formal swallow assessment from SP, change diet (thickened fluids/puree), PEJ/PEG +/- fundo
18
Q
What are important considerations when starting a child on Bipap?
A
- Will the child die if the resp support is ceased?
- If so: contact power companies for power backup, appropriate alarms, home monitoring (spO2)
- TRAINING
- Child - mask removal and communication
- Guardian - first aid (bag mask ventilation), trache, ventilation and suction, trouble shooting for leaks and disconnection
19
Q
Criteria for obstructive vs restrictive vs mixed picture spirometry
A
Obstructive - FEV1 (low)/FVC (low/normal) is LOW.
- Bronchodilator response is positive if >20% or >200ml change with BD
- eg: asthma, bronchiectasis (CF etc)
Restrictive
- FEV1/FVC preserved but both FEV1 and FVC are LOW
- TLC low
- eg: NMD
Mixed picture
- Low FEV1/FVC AND low TLC
20
Q
- Right thoracotomy scar - ddx
A
CV
- PA banding
- ‘shunt’
Resp
- Pneumothorax
- Pleurectomy
- Pulmonary resections (wedge resection, pneumonectomy, lobectomy)
21
Q
Left thoracotomy - ddx
A
CV
- CoA
- PDA ligation
- PA banding
- ‘Shunt’
Resp
- Pneumothorax
- Pleurectomy
- Pulmonary resections (wedge resection, pneumonectomy, lobectomy)
22
Q
Median sternotomy ddx
A
Cardiac
- Cardiac transplant
- Valvular surgery/repair
- VSD/septal defects
- Fontan (hypoplastic L heart)
23
Q
Left parasternal heave = ?
A
Right heart dilatation or hypertrophy
24
Q
Palpable P2 (thrill over pulm valve) = ?
A
Pulmonary HTN
25
If there is a thrill associated with a murmur, what grade is that murmur?
Grade 4-6
* 4: easily heard murmur + thrill
* 5: thrill + murmur heard with stethoscope partly off chest wall (tilted)
* 6: thrill + murmur heard with stethoscope entirely off chest wall (5-10mm)
26
Grade 1 vs 2 murmur - define
Soft murmur heard
1: only in quiet surrounds
2: in noisy surrounds
27
Grade 3 vs 4 murmur - define
Loud/easily heard murmur heard
3: without thrill
4: with a thrill
28
Grade 4 murmur (murmur + thrill) heard in Suprasternal region = ?
AS
29
Grade 4 murmur (murmur + thrill) heard at the LUSE = ?
Pulmonary stenosis
30
Grade 4 murmur (murmur + thrill) heard at LLSE = ?
VSD
31
What effect does inspiration have on murmurs?
Increases r**_I_**ght sided murmurs
32
What effect does expiration have on murmurs?
Increases l**_E_**ft sided murmurs
33
Murmur loudest at RUSE - ddx?
Aortic stenosis (Eject systolic)
Pulmonary Stenosis
34
Murmur loudest at LUSE - ddx?
* ASD
* PS
* AS
* CoA
\*all Ejection systolic
35
Murmur loudest at LLSE - ddx?
* VSD (pan systolic)
* AVSD
* TOF (VSD)
* TR (pan systolic)
* HOCM
36
Murmur loudest at apex - ddx?
* MR
* MVP
37
Cardiac tick box spiel / summary
In summary, today I examined XX, a XX year old boy/girl with respect to the cardiovascular system. Of note… XX appeared well/unwell today, with (ab)normal vital signs including XXX
* Growth and development
* Cyanotic/acyanotic
* Heart surgery (any scars as evidence) vs no heart surgery
* Any features of heart failure? Or chronicity ?
* Specific cardiac features
* murmur: grade, ES or pan systolic, loudest at, radiating to
* Dysmorphism
* There are/are no distinctive facial features to suggest an underlying genetic condition (list if present)
* Putting these findings together, this could be consistent with (R or L VOTO)
* In the presence of previous surgery, this murmur is consistent with
* If cyanotic: ‘palliative surgery for complex congenital heart disease’
* If acyanotic: ‘congenital heart lesion with partial correction’
* I would like to confirm my diagnosis with an ECG, CXR and echocardiogram
38
Steps of cardiovascular exam
IHUGVIDEP
Inspection
* Growth - Short ?Noonan or turners or T21. Tall marfans
- Scars (thoracotomy or sternotomy, drain scars/pacing wires), groin incisions for catheter
* Cyanosis?
Hands
* Clubbing?
* Peripheral cyanosis
* Peripheral cap refill
* Any stigmata of endocarditis (Janeway lesions, oslers nodes or splinter haemmhorages)
Wrist
* Radial pulse, radial-radial delay
* Offer to check for radial-femoral delay (to maintain report I will come back to this)
Arms
* Blood pressure
* ?Water-hammer pulse
Neck
* Check JVP at 45 degrees if \>5yo
* Carotid pulse
* Scars ?central line
Eyes
* Pallor
* Scleral icterus
Mouth
* Cyanosis? (They appear cyanotic but I would like to confirm this with oxygen saturation’s)
* Dentition comment (relevant for IE)
Chest
* Inspection
* Scars
* Symmetry
* Pectus excavatum (Scooped in) or carinatum (pigeon)
* Palpation
* Heaves (palpate over sternum; palms on chest, indicates RV dilation or hypertrophy)
* Thrills (supracalvicularly, suprasternally then over each valve) - if thrill palpable then murmur is at LEAST grade 4
* Apex beat (both sides)
* Auscultation
* Listen in all 4 valve spots with Bell and diaphragm
* Listen for radiation in axilla and carotid and on the back between the scapula (coarctation or peripheral pulmonary stenosis) and supraclavicular
* Maneuvers
* Inspiration - increases R sided murmurs
* Expiration - increases L sided murmurs
* Sitting up
* Rolling to left
* Valvsalva manoeuvres (blow on your thumb)
Lungs
* Listen for creps
Abdomen
* Hepatomegaly and/or ?pulsatile liver (tricuspid regurgitation)
* Sacral oedema and peripheral oedema
39
Describe the features of the aortic stenosis murmur
* RUSE
* Ejection systolic
* Radiates to carotids (mostly R side)
* Carotid thrill
40
Describe the features of pulmonary stenosis murmur
* Ejection systolic
* LUSE
* Radiates to the back
* Thrill at LUSE
41
Describe the features of ASD murmur
* Ejection systolic
* Loudest at LUSE
* Widely split, fixed S2
* Diastolic murmur at apex (from incr flow across mitral valve)
42
Describe the features of PDA murmur
* Continuous/pansystolic murmur
* Loudest at LEFT infraclavicular area (LUSE)
* Thrill
* Bounding pulses
43
Describe the features of VSD murmur
* Pansystolic murmur loudest at LLSE
* Note - larger defect, softer murmur
* Mid diastolic rumble at apex (functional stenosis across mitral valve)
44
Describe the features of TR murmur
* Pansystolic murmur
* Loudest at LLSE
* Pulsatile liver
* Neck vein distension
45
Describe the features of Still's murmur (innocent)
Systolic murmur loudest at LLSE
Grade 2-3/6
46
What murmur is loudest at the apex?
Mitral valve problems
* MR - pansystolic
* Radiates to axilla
* Mitral valve prolapse - mid to late systolic
* Valsalva strain increases murmur
* MS - diastolic
47
What cardiac defects are most commonly associated with T21?
* AVSD
* VSD
* ASD
48
What cardiac defects are associated with Noonans?
* PS
* ASD
* HOCM
49
What cardiac defects are associated with DiGeorge?
* Truncus arteriosus
* TOF
* VSD
50
What cardiac defects are associated with Williams?
* Supravalvular AS
* Peripheral pulmonary stenosis
51
What cardiac defects are associated with Turner syndrome?
* CoA
* Bicuspid AV
52
What cardiac defects are associated with Alagille syndrome?
* TOF
* Peripheral Pulmonary Stenosis
53
What cardiac defects are associated with CHARGE syndrome?
Conotruncal defects
* Trunks arteriosus
* TOF
* CoA or other arch issues
54
What cardiac defects are associated with VACTERL?
VSD, ASD, TOF
55
Risk factors for congenital cardiac disease
* Maternal infections (TORCH etc)
* Maternal medications (anticonvulsants, ACEi, Li, retinici acid, valproate, warfarin)
* Maternal drugs, alch, smoking
* Maternal medical conditions
* CHD: DM, HTN, obesity, PKU, thyroid, connective tissue disorders, epilepsy
* CCHB: SLE, sjogrens
* Fertility treatment: incr risk of septal defects and cyanotic CHD
56
Complications of cardiac conditions to ask about in long case
* Brain injury
* Infective endocarditis (?needing prophylaxis)
* Arrhythmias and heart blocks
* Neurodevelopment - ADHD
* Poor growth, nutrition
* Intercurrent illnesses (flu and RSV immunisations)
* Missed school from recurrent admissions
* Financial and emotional consequences of signifiant disease burden
57
Cardiology transition management points
* Genetic counselling /recurrence risk (arrhythmia and cardiomyopathy, sudden cardiac death etc)
* Pregnancy CI in Eisenmengers or severe pulmonary HTN (relative in severe CCF and severe cyanosis spO2 \<80%)
* Contraception - theoretical risk of thrombosis
* Prenatal, genetic screening
* For complex CHD, transfer to a dedicated adult congenital cardiac clinic
* Assess …
* How well the patient knows their anatomy
* How well the patient knows their limitations (exercise)
* How well the patient knows their prognosis
58
Cyanotic heart conditions
6 Ts
* TGA
* TOF
* TA
* TE
* Truncus
* TAPVD
1P
* PA
1H
* HLHS (and other single ventricle physiology, see 2 Ds below:)
2Ds
* DORV
* DILV
59
Post renal transplant complications
Short term
* Acute rejection
* Immunosuppressants (steroids, tacrolimus etc)
- Medication compliance
- Side effects - education and monitoring for side effects
- Infection susceptibility (CMV, EBV, BK virus) and prophylaxis
* Coordination of ix
- Drug levels
- Infection screens
* Mental health of patient and family (burden of blood tests and outpatient reviews)
* Expectation vs reality
* Coping?
Long term
* Chronic rejection/graft failure
* Review cx if CKD - anaemia, growth failure, bone health (renal osteodystrophy)
* CV health - long term effects of HTN and steroids (incr risk of IHD and stroke)
* Mental health of patient and family
* Emergency plans - contact numbers
60
What cardiac defect(s) am I?
* Usually well grown
* RV heave/ sternal deformity
* PV ESM, wide and fixed split S2
* IRBBB on ECG in 95%+, RVH
* CXR: heart big, lungs wet
ASD
AVSD (although would expect L superior axis)
61
What cardiac defect am I?
* LLSE pan systolic murmur
* +/- thrill
* May have normal ECG and CXR in older child
VSD
62
What cardiac defect am I?
* Pansystolic murmur
* Subclav
* +/- wide PP
* May have normal CXR and ECG (but also may have large heart and wet lungs and left sided ECG changes)
PDA
63
What cardiac defect am I?
* Pansystolic murmur
* Mitral region
* CXR - cardiomegaly with dry lungs
* ECG - Left sided ECG changes
Mitral regurgitation
64
What cardiac defect am I?
* Diastolic murmur +/- ESM
* Aortic region
* Wide PP
* LV ECG
* CXR - Cardiomegaly
Aortic regurgitation
(screen for RHD risk factors)
65
Progression of surgical events for the palliated cyanotic patient
1. Initial operation within first few days of life (Norwood = BT shunt to PA band)
- Have 'shunt' murmur which is continuous (can't distinguish S1 or S2)
2. BCPC at 3-12months of life (spO2 around 80-85 after)
- Should NOT have a murmur
3. Fontan operation around 4-5yo (spO2 low 90%s after this)
66
CXR appearance of cyanotic patient
* Small heart
* Lungs NOT wet (less flow to lungs, cyanotic)
* \*check abdominal organs for isomerism as clue for complex avsd/pa/tapvd)
67
Features of tetralogy of fallow (TOF)
1. VSD (shunt R to L)
2. PS (obstruction of blood to lungs → CYANOSIS)
3. RVH (from PS)
4. Overriding aorta
68
When is TOF fixed surgically and what would you expect clinically for them to have after (oe and ix)?
Fixed surgically at 6-10 months of age
Post op expected to have residual degree of pulmonary stenosis/regurg (25-30% req PVR before 18)
* Pulmonary murmur
* Sternotomy scar
* ECG - RVH, RBBB, wide QRS (correlates w degree of RV dilatation)
69
When is AVSD fixed surgically and what would you expect clinically for them to have after (oe and ix)?
Fixed 2-4 months if VSD big
T21 in 80%!
Post op expected to have residual mitral regurgitation
* Apical pan systolic murmur
* ECG: Left superior axis +/- L sided changes (LA or Lv big)
70
Post operative coarctaction short case - what would you expect clinically?
* LAteral thoracotomy scar
* Aortic ESM (strong association with bicuspid aortic valve)
* HTN
* 4 limb BPs ?residual deficits with discrepancy between upper and lower limbs (UL BP > LL BP)
71
When is TGA fixed surgically and what would you expect clinically for them to have after (oe and ix)?
* Fixed first 1-2 weeks of life (arterial switch)
* Old sternotomy scar
* May have pulmonary ESM (residual branch pulmonary stenosis common)
* May have aortic root dilatation and AR (PSM at aortic region), AS/AR, PS/PR
* ECG - RVH, BVH, RAH, no RBBB (unlike TOF)
* CXR: egg on a string
72
Sitting forwards increases what murmur?
Aortic incompetece/regurg
73
Rolling to the side increases what murmur?
Mitral stenosis
74
Valsalva increases what murmur?
HOCM
AR
75
Questions to ask on CF long case
RESP
* How was it diagnosed?
* Age
* A/N diagnosis, NST? Family history, med ileum, FTT, recurrent chest infections?
* Mutation and class
* Are they on a CFTR modulator? Ivacaflor or Orkambi?
* What is their baseline - FEV1, cough (always, spontaneous, nocturnal, only with PT, only when unwell), sputum volume production, haemopytsis
* Previous micro: bacteria (staph, haemoph, burkholderia, achromobacter, PsA), aspergillus, scedosporium, candida
* PsA and burkholderia - linked with worsening lung function so try to eradicate as early as possible. If unable to eradicate, patient becomes colonised with it.
* Only care about candida if they are immunosuppressed
* NTM types (MAC/abscesses)
* ABPA - steroids?
* Are they on any abx? (PRN/regular/rotating/oral or inhaled tobi/tips, amik or colistan)
* Side effects of medications (abx, steroids)
* Reactive airways? (preventer/reliever; trial off recently?)
* When was last CT? Bronchiectasis?
* Last admission and why? frequency of hospital admissions?
* Adherence to medications and chest PT
* Chest PT
* How often
* Form: mucolytics (pulmozyme, mannitol, hypertonic saline), Ventolin, manual chest PT (oscillatory PEP or exercise)
* Ventilatory requirements
* Bipap/O2 - all day, just overnight?
* Lung translant - bilateral
* Occurs when FEV1 \<30% and life expectancy \<2yrs
GIT
* CFR Liver disease? LFTs, liver USS 2 yrly
* Constipation/DIOS/mec ileus/rectal prolapse
Nutrition
* Pancreatic enzyme replacement (Creon)
* Oily stool? (if poorly adherent)
* Check growth
* Annual nutritional bloods - iron, zinc, vitamin AED
* Salt supps
* Dietician, are they on extra nutritional shakes? PEG?
Endocrine
* CFRDM (presents with LOW and worsening lung function): are they on insulin? short or long acting?
* Pubertal delay
* DEXA
ENT
* nasal polyps, sinus
* OSA
Venous access
* Port?
Psychosocial issues
* Mental health
* Treatment adherence
* Transition
* Smoking
* School attendance
* Any CF associations or knowledge/education
* Burden of care
* Parents - Financial, relationship, work
Other
* CFR arthritis
* Immunisation (extra vaccines - flu/pneumococcal/hep A)
* smoking exposure
76
Cx of cardiac transplant
Rejection - acute/chronic
Infx
Coronary Artery Disease
HTN
Neoplasia
Abnormal renal function
Osteoporosis
77
Causes and risks of aortic root dilatation
* Bicuspid valve (isolated, turners, conotruncal abnormalities)
* Connective tissue disease (Marfan, Ehlers dances, loeys-dietz, osteogenesis imperfecta)
* Risk of ascending aortic dilatation → dissection → high mortality
78
Pre op assessment prior to scoliosis surgery
Lung function (FVC)
Sleep study
Multidisciplinary clinic to optimise other issues (aspiration, constipation, medication rationalisation)
Establishment of pre-operative resp support (Bipap)
79
OSA
- Definition
- Causes
- Diagnosis
- Mx
Recurrent oropharyngeal upper airway collapse during sleep leading to multiple cycles of hypoxic episodes followed by blood reoxygenation. Often worse in REM sleep (reduced muscle tone)
**Causes**
* *Adenotonsillar hypertrophy*
* *Chronic rhinitis/hayfever*
* Laryngomalacia (\<1)
* *Obesity*
* Anatomical
* Syndromes (T21, PWS, BWS, Pierre-Robin)
* Abnormal muscular tone (CP, hypotonia, muscular dystrophy)
* Mucopolysaccharoidosis, Achondroplasia
* *Diagnosis via**
- Overnight oximetry (good PPV, but poor NPV) -\> look for clusters of desaturations assoc w rise in HR
- Polysomnography (sleep study)
* *Mx in children**
* *Mild**
- IN steroids (nasonex)
- Weight/allergy mx
- Positional
- Watchful waiting (CHAT study)
- Adenotonsillectomy if medical tx not successful
* *Severe**
- Adenotonsillectomy
- Turbinectomy, septoplasty, other jaw distracting surgeries
- CPAP
- Rarely tracheostomy
80
1. What is protracted bacterial bronchitis
2. What are the most common bacteria found in these cases?
3. What should you be suspicious of if the cough doesn't respond to 4 weeks abx?
1. Cough lasting \>4 weeks that is WET in nature with response to 2 weeks abx tx (ADF) with no other features to indicate another cause
2. H. influenzae, Moraxella catarralis, Strep pneumonia
3. Bronchiectasis
81
Definition of chronic cough
Ddx
persistence of cough \>4 weeks
Most common 3:
1. Protracted bacterial bronchitis
2. Bronchiectasis
3. AsthmaDefinition of chronic cough
82
ASTHMA SEVERITY LEELS
1. Infrequent intermittent asthma (Symptom-free for at least 6 weeks at a time (flare-ups up to once every 6 weeks on average but no symptoms between flare-ups)
2. Frequent intermittent asthma (Flare-ups more than once every 6 weeks on average but no symptoms between flare-ups, normal exam and PFT between flares)
3. Persistent asthma
* Daytime symptoms \>2 days/week
* Nocturnal symptoms \>1 night/week
* Attacks \<6 weeks apart
* May have abnormal lung function
* Multiple presentations to ED
83
Montelukast
Mechanism of action
Age cut off
Indication
Mechanism of action = LT receptor antagonist
Can be used in children \>= 2 years of age
Main indication = alternative to ICS in children with Frequent intermittent asthma or Mild persistent asthma
Trialed first IF:
1. The child is unable to use inhaled therapy
2. The child also has significant allergic rhinitis
3. The parents have strong concerns about adverse effects of ICS
84
humanized monoclonal antibody TREATMENTS FOR SEVERE EOSINOPHILIC ASTHMA
Mepolizumab
Omalizumab
Mepo
i. Humanised anti-IgE Monoclonal Ab
ii. Prevents binding of free IgE to high affinity receptors on basophils and mast cells
iii. Approved for moderate to severe allergic asthma in children 12 years or older
iv. Delivered by SC every 2-4 weeks
Omal
i. An add-on maintenance therapy for severe asthma with an eosinophilic phenotype in patients age 12 years and older
ii. Anti-IL-5 Mab injected SC every 4 weeks
iii. Decreases the production an survival of eosinophils, a major inflammatory cell involved in asthma pathogenesis
85
Ivacaftor
- What is it used for
- What does it do
- For class 3 (G551D) and 4 mutations in CF pts over age of 2
- \*Potentiator\*
- Disease modifying agent: partially corrects the channel defect allowing Cl transport
- Initial FEV1 increase then stability
- Decr in sweat chloride to almost non CF levels
- Improvement in nutrition
86
What disease modifying agents can you use in delta 508 homozygous (class 2) CF patients
What effects do these have on CF
**Orkambi** in \>2yo (ivacaftor and lumicaftor)
- _\*corrector\*_
- For Class 2 mutations (delta 508 homozygous) in CF in pts \>12yo
- -\> improves protein folding and incr trafficking to cell surface
- 3-4% incr in LFT and 34% decr in pulmonary exacerbations
- No incr in nutrition or improvement in QOL
**Symdeko** (Tezacaftor + ivacaftor) in \>5yo
- _\*corrector\*_
- Incr FEV1, reduce pulm exacerbations
**Trikafta** (elexacaftor, texacantor and ivacaftor)
- _\*amplifier\*_
- improved/partially corrected protein folding and Cl passage through channel
- reduces exacerbations, sweat Cl and QOL
- improves FEV1 and BMI (incr)
87
Spirometry measures
what is considered abnormal in kids?
1. FVC = vital capacity (max amt of expired air after maximum inspiraotyr capacity)
- \> abnormal if \<80% predicted
2. FEV1 (or FEV 0.75)
- \> vol of air expired in 1st (or first 0.75) seconds of expiration
- \> abnormal if \<80% predicted
2b. ratio of FEV1/VC values
- \> abnormal if \<78 ~80 (actual value, not % predicted)
3. FEF 25-75% - average RATE of flow during middle half of an FVC manoeuvre
- \> marker of disease affecting MEDIUM sized airways (CF, asthma, bronchiolitis)
- \> anything below 67% is abnormal
Anything BELOW 1.64 z- scores of reference (we don't care about values that are 'too high')
88
What findings rule out
obstruction vs restriction on spirometry?
Normal FEF 25-75% rules out obstruction
Normal FVC rules out restriction
89
Diagnosis of primary ciliary dyskinesia
Screening - nasal NO levels (_low,_ need to be done on 2 separate occasions as can be transiently low w URTIs)
Electron microscopy for cilia cross-sectional structure can be normal in some patients w PCD
Gene panel
90
Inheritance pattern, Sx and Cx of PCD
**Autosomal-recessive**
Disorder of motile cilia characterised by **chronic lung disease (bronchiectasis)**, **chronic nasal congestion and sinusitis** from first few months of life, **recurrent OM** -\> **hearing impairment** and **subfertility** (all men infertile; women variable, incr risk ectopic preg).
Nasal symptoms and respiratory distress usually start soon after birth, and by adulthood bronchiectasis is invariable.
91
Types of behavioural insomnia
\*problem falling asleep and staying asleep\*
Type 1 - sleep association type
Type 2 - limit setting disorder (naughty children)
Type 3 - Mixed
Mx
- Exclude physiological cause (red flag - if child falls asleep ok but wakes up during night)
- daytime behaviour/limit problems
- Sleep hygiene
If behavioural
- sudden or graduated extinction (controlled crying)
- fading (move bedtime 15min every 3 nights) with positive bedtime routines (reading etc)
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Physiologic causes for night waking
GORD
Eczema
Asthma
OSA
PLMD
\*ADHD\*
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Mx of sleep disturbance in ADHD
Behavioural therapy
Melatonin
Atomoxetine instead of stimulants (norepinephrine reuptake inhibitor and is believed to work by increasing norepinephrine and dopamine levels in the brain)
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What is delayed sleep phase
Mx
Common in adolescents
'Permanent jet lag' - going to bed LATE (1, 2am) and getting up late
Promoted by Late homework, TV, texting, internet
Mx - sleep hygiene, advance bedtime by 15mins each 3 night, melatonin as adjuvant
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Causes of bronchiectasis
Post-infectious (Severe pneumonia, whooping cough, measles)
Inhaled FB
CF
IgA deficiency IgG subclass deficiency
Primary ciliary dyskinesia
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Asthma treatment step up approach \< 5years
Step 1: SABA PRN
Step 2: Regular preventer (Low dose ICS or montelukast) + reliever PRN
Step 3: Low dose ICS + Montelukast + reliever PRN
Step 4: Refer to paed/resp specialise
97
Asthma treatment step up approach \> 5years
Step 1: SABA PRN
Step 2: Regular preventer (Low dose ICS OR montelukast) + reliever PRN
Step 3:
High dose ICS
OR Low dose ICS + Montelukast
OR ICS/LABA combination (low dose)
+ reliever PRN
Step 4: Refer to paed/resp specialise
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SMART therapy
Symbicort (low dose budesonide-formoterol) combination can be used for both regular BD maintenance use AND as a PRN reliever (instead of salbutamol)
Good for teenagers
\*Note
- Not suitable for Seretide (fluticasone/salmeterol) as not as quick onset
- Not suitable for young children
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Coarctation of aorta murmur (pre op)
A grade 2 to 3/6 ejection systolic murmur ULSB
Radiates to left interscapular area (see table Heart Murmur Intensity) > axilla
An apical systolic ejection sound (click) may be present if a bicuspid aortic valve is also present
100
Tall p wave on ECG
RA enlargement
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Bifid/long p wave on ECG
LA enlargement
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Causes of single ventricle physiology
Hypoplastic Left Heart Syndrome (HLHS)
Pulmonary Atresia/Intact Ventricular Septum
Tricuspid Atresia
Ebstein anomaly
Double inlet left ventricle
Double outlet right ventricle
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CXR interpretation
Assess image quality: PIER
- Position: is this a supine AP file? PA? Lateral?
- Inspiration: count the posterior ribs. You should see 10 to 11 ribs with a good inspiratory effect
- Exposure: well-exposed films have good lung detail and an outline of the spinal column
- Rotation: the space between the medial clavicle and the margin of the adjacent vertebrae should be roughly equal to each other; look for indwelling lines or objects
ABCDEFG
A. Airway
B. Bones and soft tissues
- Scan the bones for symmetry, fractures, osteoporosis, and lesions. Evaluate the soft tissues for foreign bodies, swelling, and subcutaneous air.
C. Cardiac
- Evaluate the heart size: the heart should be <50% of the chest diameter on PA films and <60% on AP films.
D. Diaphragm
- Check the hemidiaphragms for position (the right is commonly slightly higher than the left due to the liver) and shape (may be flattened bilaterally in chronic asthma or emphysema, or unilaterally in case of tension pneumothorax or foreign body aspiration).
- Look below the diaphragm for free gas.
E. Effusions
F. Fields
Check lungs for infiltrates (interstitial vs. alveolar), masses, consolidation (+/- air bronchograms), pneumothoraces, and vascular markings.
G. Great vessels / gastric bubble
- Check aortic size and shape and the outlines of pulmonary vessels. The aortic knob should be clearly seen. The gastric bubble should be seen clearly and not displaced.
H. Hila and mediastinum
- Evaluate the hila for lymphadenopathy, calcifications, and masses. The left hilum is normally higher than the right. Check for widening of the mediastinum (which may indicate aortic dissection in the appropriate clinical setting) and tracheal deviation (which may indicate a mass effect, e.g. from large goitre, or tension pneumothorax). In children, be careful not to mistake the thymus for a mass!
