Resp Flashcards by Katty J

How many lobes does the right and left lung have

right = 3 
left = 2

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what are the types of alveolar cells?

type 1 = squamous alveolar cells 95% surface area, perform rapid gas exchange
type 2 = 5% surface area, perform alveolar repair and secrete surfactant

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what is surfactant made of?

90% lipid - DPPC

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what does surfactant do?

decreases surface tension,
stabilises alveoli of different sizes
promotes alveolar dryness

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what is mean arterial pressure in pulmonary circulation?

10 mmHg

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what respiratory muscle accounts for the majority of breathing?

diaphragm

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what chemoreceptors regulate breathing the most?

central chemoreceptors located in medulla (80%, most important) respond to pH changes due to carbon dioxide in CSF.

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what are the surfactant proteins and their actions?

Protein A and D = hydrophillic
Protein B & C = hydrophobic
Protein A = immune defence, protect surfactant, reabsorption and distribution surfactant
B = only found in lungs, distribution of surfactant, essential for lung function
C = enhances rate absorption of surfactant
D = defence

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What increases DPG

hypoxic stress
thyroxine
GH
testosterone
epinephrine

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What are the stages of lung development?

(1) embryonic - weeks 4-5 - lung buds
(2) pseudoglandular - weeks 5-17 - branching, **TOF most likely occurs at this stage
(3) canalicular - 16-25 weeks - bronchial tree, pneumocytes
(4) Saccular - 24/40 - alveolar ducts and sacs
(5) Alveolar - up to 8yrs - further development of alveoli and capillaries

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what is a bronchodilator response defined as

> 12% response in FEV1

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What the criteria for lung function tests?

only 5% variation 
best of 3 attempts taken 
max 8 attempts 
free artefact 
exhalation 3 seconds 
good start (FEV1 1000ml)

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what causes obstructive lung function picture?

asthma, CF, bronchiolitis obliterans

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What causes restrictive lung disease

NMD, obesity, interstitial lung disease

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what causes variable intrathoracic obstruction

bronchomalacia, tracheomalacia, tumour, Moriquio syndrome

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What causes variable extra thoracic obstruction

vocal cord palsy(unilateral), croup, laryngeal web, laryngomalacia

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What happens to FEV1, FVC and FEV1/FVC ratio in obstructive, restrictive and mixed disease?

Obstructive = FEV1 down, FVC down/normal, FEV1/FVC down
Restrictive = FEV1 normal/down, FVC down, FEV1/FVC up/normal
Mixed = all parameters down

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FEV1 values and degree obstruction?

> 70% mild
50% moderate
35% severe
<35% very severe

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what is the 2 yr survival in CF patient with a FEV1 <30%

50% survival in 2 yrs

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what is the most useful markers for disease progression and associated with prognosis?

FEV1

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what does Neuromuscular disease cause

decreased chest wall compliance, decreased total lung capacity

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what is the major determinant of total lung capacity and residual volume

lung elastic recoil

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what are the FVC’s for neuromuscular disease and sleep disorder breathing?

FVC <20% daytime symptoms

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what can cause abnormal diffusion tests?

(1) increased thickness of membrane (fibrosis)
(2) abnormal perfusion (PE, vasculitis, bleeding, exercise)
(3) anaemia

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What are the phases in asthma response?

Early - 15-30 min - IgE and mast cells leading to bronchoconstriction Late (4-12 hrs) - Th2 cells through IL4/5 & IgE leading to inflammation and oedema

what is the most common allergen in asthmatics

dust mite

what is the biggest risk factor for asthma and pre-school wheeze

maternal smoking

what is the FEV1 classification for mild, mod and severe asthma

FEV 1 >80% = mild | FEV1 <60% = severe

how do B2 agonists work?

increase cAMP, decrease Calcium release leading to bronchodilation SE = hypokalaemia, metabolic acidosis

How does aminophylline work?

PDE inhibitor, increase cAMP , decrease Calcium leading to dilatation Also inhibit TNF alpha, leukotrienes SE = Nausea, vomiting, palpitations, arrhythmia

How do steroids work?

inhibit phospholipase A2 and decrease inflammation | SE = growth (max 1.1 cm loss in height), adrenal suppression (>750mcg fluticasone)

How does motelukast work?

CystLT1 antagonist, blocks leukotriene stimulation and inhibits mast cell degranulation

Tacchyphlaxis occurs most commonly with what polymorphism

Arg 16 polymorphism

Describe the cough in trachebronchomalacia

age 1-12 months, barking nature, improves during sleep

what is the pathognomonic sign for bronchial stenosis?

crackles localised to area during forced expiration whilst lying on affected side

What is the initial mechanism in Pierre Robin sequence

hypoplasia of mandible <9 weeks

What is the most syndrome associated with Pierre Robin sequence?

Stickler COL11A/B, COL2A EYE - glaucoma, cataract Joint hyper mobility

A baby has cyanosis whilst asleep but improved with crying? what is the problem?

Coanal atresia

What are the 3 most common causes of stridor in the neonate?

(1) Laryngomalacia - normal cry (2) subglottic stenosis - normal cry (3) vocal cord palsy - weak cry

How is subglottic stenosis defined

<3mm Preterm baby

What should you be worried about in a bilateral vocal cord palsy?

arnold chiari malformation hydrocephalus intra-cranial haemorrhage

what 3 vascular abnormalities can cause stridor

anomalous brachiocephalic artery double aortic arch anomalous Left PA from Right

How would a laryngeal web present

respiratory distress at birth, weak cry, aphonia

which lobe will a foreign body most likely lodge

Right LL

when should you suspect bronchiectasis?

moist cough > 8 weeks reactive airways chest wall deformity systemic signs - clubbing, growth failure

what is the most common bacterial organism in bronchiectasis

Non-typable H. influenza

What mutation in CF does ivacaftor work for?

G551D acts to potentiate CFTR Cl channel

what abnormality does delta F 508 cause in CF

defective protein processing, deletion of codon (phenylalanine)

what organism colonises CF patients first

Staph aureus

What is characteristic about burholderia cepacia infection

sputum is brown, infection is associated with significant decline in lung function

what colour is sputum in ABPA infection

rust coloured

how do you treat ABPA

steroids and intraconozole/voriconozole

what will you find on investigations in ABPA

Serum IgE >1000, Eosinophilia

Indications for a sweat test?

unresponsive asthma rectal prolapse malabsorption

when is a sweat test positive

75mg sweat, >60mmol Cl (<40 = normal)

Reasons for false positive sweat test?

``` adrenal insufficiency hypothyroid hypoparathyroid glycogen storage disease MPS G6PD Diabetes Insipidus Klinefelters ```

what will be low on stool in CF

fecal elastase

What are the treatments for bronchiectasis?

antibiotics anti-mucolytics physio bronchodilators

how does pseudomonas gain resistance in CF

formation of biofilm

how many Cf patients does newborn screening miss?

5-10%

what are the functions of the CFTR receptor

chloride transport regulation sodium bicarb trasnport glutathione

which system is next most affected in childhood by CF

otological

what can cause a false negative sweat test?

malnutrition skin oedema mineralocorticoid use

what are classes of CF defects

``` decreased synthesis defective protein (delta F508) defective regulation (G551D) defective conduction Reduced quantity Increased death ```

how does Ataluren work

allows ribosomal read through in mRNA - works for nonsense mutations in CF

what condition is associated with primary ciliary dyskinesia

Kartageners - situs inversus, chronic sinusitis, chronic Otitis media

what is broadtail pertussis - class

gram negative bacillus

what is a feature seen on FBE in pertussis

lymphocytosis

what infants should be treated with pertussis prophylaxis

exposed <21 days ago

when are children still infectious with whooping cough?

>21 days of cough or >5days of Antibiotics

how much protection do you get with 2 pertussis immunisations and how much with 3?

50% and 95%

what is the best practice to prevent spread of RSV

hand washing

what is palivizumab active against?

RSV R protein

what difference does Palivizumab make

decrease in hospitalisation by 50%. NO effect on need for ventilation or mortality

how does strep pneumonia get resistance? how would you do in first instance?

altered penecillin binding proteins, increase concentration

a baby presents at 4 weeks with staccato cough, afebrile pneumonia. What is the organism? what is the most common mode transmission?

chlamydia trochomanis | intrapartum transmission

what bacteria most likely causes empyema/effusion

step pneumonia

what are the features of an exudate

SG>1.020, high protein, low alb, raised LDH and cholesterol

what percentage of people exposed to TB will contract disease

10-30%

what is a positive mantoux test?

>5mm high risk >10 mm moderate risk >15mm BCG last 5 years

what does quantiferon Gold measure

INF gamma from infected T cells

How should children at risk of TB with negative Mantoux test be managed?

isoniazid for 12 weeks and retest in 3 months

what is the duration of therapy for: (1) latent TB (2) Pulmonary TB (3) Miliary TB

(1) latent - 6 months isoniazid (2) Pulmonary - RIPE 6 months (3) Miliary - RIPE 12 months

what are the side effects of isoniazid

hepatitis and peripheral neuropathy

what are the side effects rifampicin

haemolytic anaemia, thrombocytopenia

what is a side effect Ethambutol

optic neuritis (avoid <7YOA)

what is a side effect pyrazinamide

arthralgia, gout, hyperglycaemia

indications for BCG vaccine

ATSI population 6weeks <5yoa newly arrived immigrant from risk area not yet screened.

PCP is seen in which conditions

``` HIV SCID Hyper IgM Wiscott Aldrich DiGeorge Immunosuppression ```

Differences between REM and Non-REM sleep

REM - low tone, dulled airway reflexes, irregular breathing, decreased TV, dreaming Non-REM - regular breathing, slow RR, better muscle tone, Growth hormone released

when does most tissular growth occur?

age 2-7 years

how many children who snore will have OSA

how many events per hour indicate OSA severity

1-5 events = mild 5-10 events = moderate >10 = severe

how is central hypoventilation defined in NMD PSG testing

pCO2 >50 for 25% of the time

Features of Congenital Central Hypoventilation syndrome?

PHOX2B gene polyalamine repeat expansion >28 = day and night Sx NREM sleep affected more that REM Associated with Hirschsprungs, Neuroblastoma, Ganglioneuroma, arrythmias, sluggish pupillary response

what is the most important investigation in achondroplasia

MRI to look for degree foramen magnum involvement