Week 5: CF, Cardiothoracic Surgery, Pulmonary Vascular Disease Flashcards
Genetics of CF and resulting pathophysiology
CFTR Protein (chromosome 7) abnormality affects Na + Cl excretion therefore osmolarity of mucus + shearing + adherance + neutrophil funct.
Type 1-3 = severe
Type 4-6 = MILD
Presenting features of CF
- infection & bronchiecstasis
- chronic sputum
- clubbing
- haemoptysis
- pneumothorax
- *pancreatic insufficiency = growth, stool
- diabetes
- osteoporosis
Pancreatic insufficiency
Pancreatic insufficiency, enzyme deficiency => abn stools, malabsorption
=> CREON enzyme replacement + nutrition planning and supplement
Chest Infection in CF
decreased mucociliary clearance
shearing = mucosal damage = bacterial adherance = chronic inflamm => bronchiecstasis
=> Azithromycin (inflamm and abx)
Screening options in CF
antenatal: pre-implant genetic diagn, CVS (placental cells), amnioscentesis
neonatal: guthrie test day 5 universal
post-natal: sweat test Cl- 60mh/l , faecal elastase
** CF Implications **
STERILITY AND INFECTION CONTROL PARAMOUNT DUE TO RISK OF INFECTION
- polypharmacy
- social and mental impacts
- occupational and hobby limitations
- lung transplants
Lung Transplantation
BOTH LUNGS, FEV1 30%+ predicted poor funct
- CHOLANGITIS (indication) infection of biliary tree
- increased survival but significant contraindications, attrition d/t rejection, small window of opportunity!
New Modulator Drugs
CFTR protein targetting therefore mutation specific
= ↑QoL but small improvement to lung funct.
Difference between 1º and 2º Pneumothorax
1º = no hx/path., developmental nature. APEX.
2º = path. DIFFUSE
Other types of Pneumothorax
Recurrent: 1+ episodes
Persistent: Air leak maintains
Open pneumothorax: hole in chest wall
Tension pneumothorax
=> tx. talc-pleurodesis
SPontaneous pneumothorax
Lung Abcesses
LUNG ABCESS: involve lung parenchyma
EMPYEMA: pus discharge into pleural space
Empyema thoracis: post-pneumonia/op/trauma, significant malaise, abx inefficiacy?
Often sterile but becomes complicated w/ infection
Thymic Tumours
good prognosis, mortality in multiple endocrine neoplasia
*myasthemia graves (THYOMA)
Indication for lung surgery for pneumothoraces
RECURRENT ipsi or contralateral
Only after 2nd episode (1st episode special circumstances)
Bronchogenic cysts
developmental: mucus lakes dont form, presses on trachea
Singificant contraindications to surgery for lung cancer
- overweight: post-pred causes Wt. gain
- 65y/o+
- existing renal failure: imm suppr.
Pathophys. and signs and symptoms of a PE
w/ background of pleuritic pain, cough, haemoptsis
necrosis d/t infarctinon
- pyrexia
- pleural rub
- dull percussion (effusion)
Findings and pathophys of a PE w/ background of isolated acute dyspnoea
V/Q mismatch
- tachycard., tachyopnea
- hypoxia
Pathophys and findings of a PE w/ background of syncope or cardiac arrest
large thrombus to cardiac output,
- tachy,
- hypotension
- SHOCK
- hypoxia
PE Investigations
Pre-Tests: WELLS score; REVISED GENEVA SCORE
Gold: CTPA: CT pulmonary angiogram
- Trop1: cardiac;
- d-dimer: clotting
- V/Q scan
- ECHO
Nature of PE Tx
known cause: 3mos of Tx
unknown/irreversible: LT
comorb SIGNIFICANT
PESI score: prognosis SIGNIFICANT
Pulmonary arterial tree pressure
> 25mmHg = HYPERTENS (measured via doppler ECHO)
Pathogenesis of Cor Pulmonale
RV hypertrophy d/t chronic hypertension d/t chronic hypoxia
=> RHS cardiac failure d/t inability sufficiently fill and pump blood
=> oedema in extremities
Pulm. Hypertens. presentation and symptoms
exertional dyspnoea and chest tightness; pre-syncope; haemoptysis
- ↑JVP
- hepatomegaly
- oedema
Classification for P. Hypertension Aetiology & Pathophys
G1. idiopathic G2. 2º heart disease G3. 2º resp disease G4. chronic thromembolic PH remodelling G5 sarcoidosis, collagen vasc. disease
