Bronchiectasis And Cystic Fibrosis Flashcards
What is bronchiectasis
• DEFINITION - Chronic dilatation of one or more bronchi. The bronchi exhibit poor mucus clearance and there is predisposition to recurrent or chronic bacterial infection. Radiological :
• bronchial dilatation bigger than the adjacent blood vessel, bronchial wall thickening
• Therefore gold standard diagnostic investigation
= CT (specifically High Resolution CT)
Wha is signet right sign
Ss
What are the symptoms of bronchiectasis
Very Common:
• Chronic cough
• Daily sputum production – can vary in quantity, colour
and consistency
Common: • Breathlessness on exertion • Intermittent haemoptysis • Nasal symptoms • Chest Pain • Fatigue
Less Common:
• Wheeze
What are the causes of bronchiectasis
• Post infective – whooping cough, TB
• Immune deficiency – Hypogammaglobulinaemia
• Mucociliary clearance defects – Cystic fibrosis, primary ciliary dyskinesia, Young’s syndrome (triad of bronchiectasis, sinusitis, and reduced fertility), Kartagener syndrome (triad of bronchiectasis, sinusitits, and situs inversus)
• Obstruction – foreign body, tumour, extrinsic lymph node
• Toxic insult – gastric aspiration (particularly post lung transplant),
inhalation of toxic chemicals/gases
• Allergic bronchopulmonary aspergillosis
• Secondary immune deficiency – HIV, malignancy
• Rheumatoid arthritis
• Associations – inflammatory bowel disease; yellow nail syndrome
What are some organisms tat can cause bronchiectasis
- Haemophilus influenzae • Pseudomonas aeruginosa
- Moraxella catarrhalis
- Stenotrophomonas maltophilia
- Streptococcus pneumoniae
- Fungi – aspergillus, candida
- Nontuberculous mycobacteria (NTM)
- Less common - Staphylococcus aureus
What are indications that a patient might have bronchiectasis
- Patient labelled as “asthma” without any great objective evidence, particularly lifelong
- History of severe chest infection earlier in life (bacterial, viral or atypical)
- Lifelong chest infections ?genetic cause
- Nasal and ear symptoms or admitted to SCBU at birth
- Recurrent chest infections ?immunodeficiency
- Sputum culture positive for common organisms such as haemophilus or pseudomonas
- Inflammatory bowel disease, rheumatoid arthritis - Systemic cytokines release - can change teh architecture of an airway
Descrbe the management for bronchiectasis
- PHYSIO / AIRWAYS CLEARANCE
- Sputum sampling – routine culture and NTM
- Exclude immunodeficiency / treat identifiable causes
- Management plan for infective exacerbations
- Consider long-term therapies at future visits
- Flu vaccine
- Pulmonary Rehabilitation if MRC dyspnoea score > 3
- An established MDT is key
How is an exacerbation/flare up definite
• How do we define “exacerbation” in bronchiectasis? • Recent international expert consensus………..
A person with bronchiectasis with a deterioration in 3 or more key symptoms for at least 48 hours:
• Cough
• Sputum volume and / or consistency
• Sputum purulence
• Breathlessness and / or exercise tolerance
• Fatigue
• Haemoptysis
Should start a course of antibiotics
Describe teh pathophysiology of cf
• Autosomal recessive
• 1 in 2,500 in the UK are born with CF
Long arm Chromosome 7 defect
Leads to Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) mutation
Leads to ineffective cell surface chloride transport
Leads to thick, dehydrated body fluids in organs which have CFTR
- Cftr not present of functioning - does not conduct chloride into lumen, not proper ENaC - not enough resporbtion of sodium, dehydration of the liquid layer
Describe cf diagnosis
One or more of the characteristic phenotypic features -
• Or a history of CF in a sibling -
• Or a positive newborn screening test result -
And
• An increased sweat chloride concentration
(> 60 mmol/l) – SWEAT TEST
• Or identification of two CF mutations – genotyping – may need EXTENDED GENOTYPING
Decsribe the presentation of cf
- Meconium ileus
• In 15-20% of newborn CF infants the bowel is blocked by the sticky secretions. There are signs of intestinal obstruction soon after birth with bilious vomiting, abdominal distension and delay in passing
meconium. - Intestinal malabsorption
• Over 90% of CF individuals have intestinal malabsorption. In most this is evident in infancy. The main cause is a severe deficiency of pancreatic enzymes. - Chest infections
- Newborn screening
What are teh complications of cf
Ss
What are lifestyle advice for cf
- No smoking
- Avoid other CF patients
- Avoid friends / relatives with colds / infections
- Avoid jacuzzis (pseudomonas)
- Clean and dry nebulisers thoroughly
- Avoid stables, compost or rotting vegetation – risk of aspergillus fumigatus inhalation
- Annual influenza immunisation
- Sodium chloride tablets in hot weather / vigorous exercise
How is cf managed
- Complex - led by CF Specialist Centres / MDT’s
- Holistic care / Multisystem (organ) focus
- Key is maintaining lung health (chest physio, infection management) and nutritional state – BMI, pancreatic status, vitamin status: FEV1 and weight / BMI
- Targeted therapies (Genotype specific)
- Managing other co-morbidities e.g. diabetes, liver disease, etc.
- Concordance (physio and drugs) and other Psychosocial factors
