Week 5: CF, Cardiothoracic Surgery, Pulmonary Vascular Disease Flashcards

1
Q

Genetics of CF and resulting pathophysiology

A

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

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2
Q

Presenting features of CF

A
  • infection & bronchiecstasis
  • chronic sputum
  • clubbing
  • haemoptysis
  • pneumothorax
  • *pancreatic insufficiency = growth, stool
  • diabetes
  • osteoporosis
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3
Q

Pancreatic insufficiency

A

Pancreatic insufficiency, enzyme deficiency => abn stools, malabsorption

=> CREON enzyme replacement + nutrition planning and supplement

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4
Q

Chest Infection in CF

A

decreased mucociliary clearance

shearing = mucosal damage = bacterial adherance = chronic inflamm => bronchiecstasis

=> Azithromycin (inflamm and abx)

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5
Q

Screening options in CF

A

antenatal: pre-implant genetic diagn, CVS (placental cells), amnioscentesis
neonatal: guthrie test day 5 universal

post-natal: sweat test Cl- 60mh/l , faecal elastase

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6
Q

** CF Implications **

A

STERILITY AND INFECTION CONTROL PARAMOUNT DUE TO RISK OF INFECTION

  • polypharmacy
  • social and mental impacts
  • occupational and hobby limitations
  • lung transplants
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7
Q

Lung Transplantation

A

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!
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8
Q

New Modulator Drugs

A

CFTR protein targetting therefore mutation specific

= ↑QoL but small improvement to lung funct.

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9
Q

Difference between 1º and 2º Pneumothorax

A

1º = no hx/path., developmental nature. APEX.

2º = path. DIFFUSE

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10
Q

Other types of Pneumothorax

A

Recurrent: 1+ episodes

Persistent: Air leak maintains

Open pneumothorax: hole in chest wall

Tension pneumothorax
=> tx. talc-pleurodesis

SPontaneous pneumothorax

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11
Q

Lung Abcesses

A

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

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12
Q

Thymic Tumours

A

good prognosis, mortality in multiple endocrine neoplasia

*myasthemia graves (THYOMA)

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13
Q

Indication for lung surgery for pneumothoraces

A

RECURRENT ipsi or contralateral

Only after 2nd episode (1st episode special circumstances)

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14
Q

Bronchogenic cysts

A

developmental: mucus lakes dont form, presses on trachea

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15
Q

Singificant contraindications to surgery for lung cancer

A
  • overweight: post-pred causes Wt. gain
  • 65y/o+
  • existing renal failure: imm suppr.
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16
Q

Pathophys. and signs and symptoms of a PE

w/ background of pleuritic pain, cough, haemoptsis

A

necrosis d/t infarctinon

  • pyrexia
  • pleural rub
  • dull percussion (effusion)
17
Q

Findings and pathophys of a PE w/ background of isolated acute dyspnoea

A

V/Q mismatch

  • tachycard., tachyopnea
  • hypoxia
18
Q

Pathophys and findings of a PE w/ background of syncope or cardiac arrest

A

large thrombus to cardiac output,

  • tachy,
  • hypotension
  • SHOCK
  • hypoxia
19
Q

PE Investigations

A

Pre-Tests: WELLS score; REVISED GENEVA SCORE

Gold: CTPA: CT pulmonary angiogram

  • Trop1: cardiac;
  • d-dimer: clotting
  • V/Q scan
  • ECHO
20
Q

Nature of PE Tx

A

known cause: 3mos of Tx
unknown/irreversible: LT

comorb SIGNIFICANT
PESI score: prognosis SIGNIFICANT

21
Q

Pulmonary arterial tree pressure

A

> 25mmHg = HYPERTENS (measured via doppler ECHO)

22
Q

Pathogenesis of Cor Pulmonale

A

RV hypertrophy d/t chronic hypertension d/t chronic hypoxia

=> RHS cardiac failure d/t inability sufficiently fill and pump blood
=> oedema in extremities

23
Q

Pulm. Hypertens. presentation and symptoms

A

exertional dyspnoea and chest tightness; pre-syncope; haemoptysis

  • ↑JVP
  • hepatomegaly
  • oedema
24
Q

Classification for P. Hypertension Aetiology & Pathophys

A
G1. idiopathic
G2. 2º heart disease
G3. 2º resp disease
G4. chronic thromembolic PH remodelling
G5 sarcoidosis, collagen vasc. disease
25
Q

P. Hypertension Diagnosis

A

Gold: ECHO tricuspid regurgitation, dilated RV

  • V/Q
  • CTPA
  • R. heart catherisation: direct pressure measurement
  • CXR
  • ECG changes
26
Q

Tx P.Hypertens

A

symptomatic control and prophylaxis

=> ACoag (warfarin for clots)
=> diuretics = HF
=> O2 rx
=> Digoxin = improve cardiac contractions and ↓↓HR