WEEK 5 Flashcards

1
Q

Embryology of respiratory tract

A
  1. EMBRYONIC (3-8 WEEKS)
    • Respiratory diverticulum (lung bud) develops from fetal foregut
    • By ~5 weeks, two primary lung buds are visible, which later divide into lobar buds
    • Cells lining lung buds derive from endoderm, blood vessels and connective tissue surrounding lungs are derived from mesoderm
  2. PSEUDOGLANDULAR (5-17 WEEKS)
    • Rapid branching of airways
    • 16-25 primitive segemental bronchi are formed
    • Development of specialised cells such as cilia and mucous glands
  3. CANALICULAR (16-26 WEEKS)
    • GAS EXCHANGE UNIT FORMATION
    • FETUS CAN BE BORN PREMATURELY AT THE END STAGES OF CANALICULAR PHASE DUE TO FORMATION OF GAS EXCHANGE UNITS
    • Lung develop from distal architecture
      • includes terminal bronchioles, alveolar sacs and capillary blood vessels
    • Type 1 and Type 2 pneumocytes (alveolar cells) form
  4. SACCULAR (24-38 WEEKS)
    • Growth and formation of alveolar sacs which become alveoli after birth
    • More surfactant produced
    • Bronchioles continue to elongate, interstitial tissue between the sacs reduce and the alveolar walls become thinner which improves gas exchange
  5. ALVEOLAR (36 WEEKS - 2/3 YEARS)
    • Cells at this stage are well differentiated and the pulmonary vasculature more developed forming the final alveolar
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2
Q

Define the term remodelling

A

Alteration of airway structure following external influence
- Environmental exposures
- Chronic diseases of childhood
- Infection

Leads to abnormalities due to interference of inter-cellular signalling

Seen in asthma and chronic lung disease of prematurity

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3
Q

What are the 3 upper congenital abnormalities affecting the respiratory tract

A
  1. Laryngomalacia
    • Dynamic abnormal collapse of larynx
  2. Tracheomalacia
    • Dynamic abnormal collapse of tracheal wall
  3. Traecho-oesphageal fistula
    • Abnormal connection between trachea and oesophagus
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4
Q

What are the 2 lower congenital abnormalities affecting the respiratory tract

A
  1. Congenital pulmonary airway malformation (CPAM)
    • Abnormal non-functioning lung tissue
  2. Congenital diaphragmatic hernia
    • Diaphragm develops from multiple tissues around 7 weeks and closes by 18 weeks
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5
Q

Most common type of lung cancer

A

Adenocarcinoma

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6
Q

Sleep apnoea management

A
  • Identify exacerbating factors
    • Weight reduction
    • Avoidance of alcohol
    • Diagnose and treat endocrine disorders eg hypothyroidism
  • Continuous positive airway pressure (CPAP)
  • Mandibular repositioning splint
  • Positional therapy devices
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7
Q

Presenting symptoms and signs of respiratory muscle weakness.

A
  • SOB
  • Orthopnoea (SOB while lying down)
  • Ankle swelling (hypoxic cor pulmonale)
  • Morning headache
  • Recurrent chest infection
  • Disturbed sleep
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8
Q

Investigations and management of respiratory muscle weakness.

A

INVESTIGATIONS:
- Lung function
- Lying and standing VC
- Mouth pressures/SNIP
- Assessment of hypoventilation
- Overnight oximetry
- Transcutaneous CO2 monitoring

MANAGEMENT:
- Treat underlying condition
- Oxygen therapy
- Tracheostomy ventilation
- Supportive treatment (not curative)

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9
Q

Other conditions causing excessive daytime sleepiness.

A

NARCOLEPSY

Chronic neurological disorder that affects the brains ability to control sleep-wake cycles

CLINCIAL FEATURES:
- Cataplexy
- Excessive daytime sleepiness
- Hypnagogic/hypnopompic hallucinations
- Sleep paralysis

INVESTIGATIONS:
- PSG
- MSLT
- LOW CSF orexin

TREATMENT:
- Modafinil
- Dexamphetamine
- Venlafaxine (for cataplexy)
- Sodium Oxybate (Xyrem)

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10
Q

Neurological conditions associated with respiratory muscle weakness.

A

CHRONIC VENTILATORY FAILURE

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11
Q

What is used to determine sleep apnoae

A

EPWORTH SLEEPINESS SCALE

It is scored out of 24, with a score of 11 or more regarded as abnormal

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12
Q

Define the principles of management of pulmonary vascular diseases.

A
  • Oxygen
  • Warfarin
  • Direct Oral Anticoagulants
    • RivaroXABAN
    • ApiXABAN
  • Thrombolysis
  • Pulmonary embolectomy
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13
Q

Pulmonary hypertension causes, signs and treatment

A

CAUSES:
- Can be idiopathic
- Congenital heart disease (L to R shunt)
- HIV infection
- Secondary to left heart disease
- Secondary to chronic respiratory disease
- Chronic thromboembolic PH
- Miscellaneous
- Sarcoidosis

SIGNS:
- Elevated JVP
- Right ventricular heave
- Loud pulmonary second heart sound
- Hepatomegaly
- Ankle oedema

TREATMENT:
- Treat underlying condition
- Calcium channel antagonists
- Oxygen
- Anticoagulation
- Diuretics

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14
Q

Outline the management of pancreatic insufficiency and chest infection.

A

BOOSTING NUTRITION

  1. REPLACE ENZYMES: (CREON)
  2. DIET: High energy plus high calorie supplement drinks*Not low fat
  3. NUTRITIONAL SUPPLEMENTS: Fat-soluble vitamin and mineral supplements
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15
Q

Describe the genetics and presenting features of cystic fibrosis.

A

Occur when you inherit TWO MUTATED GENES, (one from each parent)
MOST COMMON AUTOSOMAL RECESSIVE DISORDER
Due to a mutation on the CFTR which is coded on chromosome 7

TWO COMMON PRESENTATIONS OF CF THROUGHOUT LIFE:

  1. PANCREATIC INFUFFICIENCY
    a. The CFTR mutation also affects the pancreas.
    b. The pancreas produces enzymes that digest food. Lack of enzymes causes:
    - Malabsorption
    - Abnormal stools - pale, offensive, float
    - Failure to thrive
    c. Importance of records, growth charts
  2. RECURRENT CHEST INFECTIONS
    • Pneumonia
    • Bronchiectasis
    • Scarring
    • Abscesses
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16
Q

Explain the options available for antenatal and post natal screening for cystic fibrosis

A

ANTENATAL:
- Pre-implantation genetic diagnosis - where a cell is removed from a developing embryo and analysed.
- Chorionic villous sampling - involves removing and testing a small sample of cells from the placenta.
- Amniocentesis - a procedure in which amniotic fluid is removed from the uterus for testing or treatment.

NEONATAL:
Newborn bloodspot day 5 (Guthrie test)
- Screen positive - referred for clinical assessment and sweat test
- Proportion of diagnoses missed
- Measures the concentration of chloride excreted in sweat.
- Elevated in CF.
- IN CHILDREN:
- Tests usually repeated to confirm.
- Less reliable in adults and infants < 6 months old.

17
Q

Define the possible complications of cystic fibrosis.

A
  • Abnormal electrolyte transport across cell membrane
  • Dehydration of airway surface layer
  • Decrease mucociliary clearance
  • Mucous sticks to mucosal surface and causes shearing and inflammation
  • Increase access to bacteria
  • Decrease bacteria killing
18
Q

What 6 respiratory conditions cause clubbing?

A
  1. Lung cancer
  2. Idiopathic pulmonary fibrosis
  3. Bronchiectasis
  4. Cystic fibrosis
  5. Lung abscess
  6. Empyema