3- Paediatric respiratory - breath sounds (2/3) Flashcards
Lower respiratory tract infections
- Pneumonia
- Acute bronchitis
- Bronchiectasis exacerbation
Pneumonia
Background
- Infection of lung tissue
- Can be seen as consolidation on chest x-ray
causes of pneumonia
Bacteria
- Streptococcus pneumonia (most common)
- Group A strep (e.g. streptococcus pyogenes)
- Group B strep (in pre-vaccinated infants, often contracted during birth)
- Staphylococcus aureus (x-ray): round air filled cavities
- Haemophilus influenza
- Mycoplasma pneumonia (atypical)
o Extra-pulmonary manifestation such as Erythema multiforme
Virus
- Respiratory syncytial virus (RSV)
- Influenza and parainfluenza virus
pathophysiology of pneumonia
- Infection causes inflammation of lung tissue and sputum filling the airways and alveoli
presentation of pneumonia
- Cough (typically wet and productive)
- High fever (> 38.5ºC)
- Tachypnoea
- Tachycardia
- Increased work of breathing
- Lethargy
- Delirium (acute confusion associated with infection)
signs of pneumonia
There may be a derangement in basic observations. These can indicate sepsis secondary to the pneumonia:
* Tachypnoea (raised respiratory rate)
* Tachycardia (raised heart rate)
* Hypoxia (low oxygen)
* Hypotension (shock)
* Fever
* Confusion
Characteristics of chest signs of pneumonia:
- Bronchial breath sounds these are harsh breath sounds that are equally loud on inspiration and expiration. These are caused by consolidation of the lung tissue around the airway.
- Focal coarse crackles caused by air passing through sputum similar to using a straw to blow into a drink.
- Dullness to percussion due to lung tissue collapse and/or consolidation.
Investigations for CXR
- Chest xray (not routinely required) – but the gold standard
- Sputum cultures
- Throat swabs for culture and viral PCR
Sepsis
- Blood cultures
- Capillary blood gas analysis and blood lactate
management of pneumonia
Antibiotics according to local guidelines
- E.g. PO Amoxicillin +- Macrolide (erythromycin/ clarithromycin) (will cover atypical pneumonia)
- If penicillin allergic can treat with just macrolide monotherapy
- IV Abx if sepsis or problems with intestinal absorption
- Oxygen if sats <92%
Complicated pneumonia
Background
- Parapneumonic effusion e.g.transudate/ exudate
- Empyema e.g. presence of pus in the pleural space
Investigation
- CXR
Management
- Long course of antibiotics
- Chest drain and intrapleural fibrinolytic agent (urokinase)
- VATS (video assisted thoracoscopic surgery)
Bronchiectasis
Background
- Abnormal dilatation of the airways with associated destruction of bronchial tissue
- Potentially reversible in children
Pathophysiology of bronchiectasis
- Usually caused by severe infection which leads to structural damage within bronchial walls, which causes dilation.
- Scarring which arises as a consequence of immune response, reduces the number of cilia within the bronchi-> further predisposing the individual to further infections
causes of bronchiectasis
- Most associated with cystic fibrosis
- Non-CF causes
NON-CF causes of bronchiectasis
Post-infections
- Streptococcus pneumonia
- Staphylococcus aureus
- Adenovirus
- Measles
- Influenza
- Mycobacterium tuberculosis
Immunodeficiency
- Antibody deficiency e.g. agammaglobulinemia, common variable immune deficiency of IgA/IgG deficiency
- HIV infection
- Ataxia telangiectasia
Primary ciliary dyskinesia e.g. Kartagener’s
- Autosomal recessive genetic defect -> causes reduced efficacy of bronchial cilia
- Reduces Mucociliary clearance – susceptible to infection
Post obstructive
- Foregin. Body aspiration
Congenital syndromes
- Youngs
o Bronchiectasis
o Reduced fertility
o Rhinosinusitis
- Yellow -nail syndrome
o Pleural effusions
o Lymphoedema
o Dystrophic nails
o Bronchiectasis’s (40%)
bronchiectasis presentation
- Chronic , productive cough
- Chest pain
- Wheeze
- Breathlessness on exertion
- Haemoptysis
- Recurrent or persistent infections of lower tract
examination findings bronchiectasis
Examination
- Finger clubbing
- Inspiratory crackles
- Wheezing
investigations for bronchiectasis
Two-fold investigations: diagnosis and cause
- Imaging
- bronchoscopy
imaging for bronchiectasis
Chest X-ray
- Bronchial wall thickening
- Airway dilation
- Can appear normal
High res CT (HRCT)- gold standard
- Bronchial wall thickening, diameter of bronchus larger than accompanying bronchial artery – signet ring sign
- Cystic fibrosis - bilateral upper lobe bronchiectasis
- Post TB infection- unilateral upper lobe bronchiectasis
- Foreign body inhalation- focal bronchiectasis (lower lobe)
bronchoscopy
o Not routine
o For patients with focal bronchiectasis evident on HRCT
Investigating the underlying cause of bronchiectasis
- Chloride sweat test – CF
- CFTR gene mutation
- FBC with leucocyte differential
- Immunoglobulin panel for immunoglobulin deficiency
- Ciliary biopsy during bronchoscopy
- HIV test
- Microbiological assessment
o E.g. pseudomonas spp colonisation- think CF - Lung function- obstructive pattern or mixed obstructive and restrictive pattern- scarring compromises lung compliance
Management of bronchiectasis
Aim: relieve symptoms, prevent progression of disease and ensure normal growth and development
- Chest physiotherapy
o Mucus clearing techniques - Bronchodilators for those with a wheeze
- Exacerbations and abxs
o Non encapsulated haemophilus, streptococcus pneumonia and Moraxella catarrhalis
o Some children need short courses and others more continuous treatment - Follow up regularly
Complications of bronchiectasis
- Recurrent infection
- Life-threatening haemoptysis
- Lung abscess
- Pneumothorax
- Poor growth and development
Prognosis of bronchiectasis
- Depends on the cause
- E.g. if post -infective- treatment should halt disease
CF background
Cystic fibrosis is an autosomal recessive disease caused by a mutation in the CF transmembrane conductance regulator gene (CFTR) resulting in multisystem dysfunction. In 2000 the life expectancy of a child born in 2000 was 50 years
pathophysiology of CF
CTFR is a protein chloride channel which is found in many epithelial tissues.
- Cl- moves down its conc gradient using ATP
- This fault also affects sodium reabsorption (ENaC) which reduces the amount of water in secretions
- This reduces airway surfactant liquid and impedes mucus clearance and immunological functions
- Different organs have different sensitivity to loss of CFTR protein function e.g. vas deferens very sensitive
which organs are specifically affected by CF
respiratory system
pancreas
GI
reproductive
respiratory system and CF
- Reduced mucus clearance and reduction in effectiveness of Mucociliary escalator impedes immunological function
- Provides a niche for bacterial growth with biofilm mode of growth providing ideal conditions to protect bacteria from host immune system and antibiotics
- Pro-inflammatory cascade -> tissue damage
Pancrease and CF
- Pancreatic duct occluded in-utero -> permanent damage to exocrine pancreas -> pancreatic insufficiency
- CF-related diabetes mellitus
GI and CF
- CTFR defect causes small intestine to secrete viscous mucus which can cause bowel obstruction in utero -> meconium ileus
- Cholestasis in-utero can cause neonatal jaundice
- Can cause CF-related liver disease later in life
reproductive systema and CF
o 98% of men are infertile due to congenital absence of vas deferens
o Nutrition important predictor of successful pregnancy. Must be carefully planned -> can be associated in deterioration of lung health
Risk factors for CF
Genetic
- Autosomal recessive
- More than 2000 identified mutations in CTFR gene
- Categorised into 5
- E.g. Class 1 – nonsense mutation and no complete CFTR protein formed
- E.g. Class 5 completes CTFR protein but deficiency in number
presentation of CF in neonates
Meconium ileus
- Abdominal distension and delayed passage of meconium and bilious vomiting in first day of life
- Failure to thrive
- Prolonged neonatal jaundice
presentation of CF in infancy
o Failure to thrive
o Recurrent chest infractions
o Pancreatic insufficiency: steatorrhea
presentation of CF in adults
o Rectal prolapse
o Nasal polyps
o sinusitis
presentation of CF in adolescents
o DM secondary to pancreatic insufficiency
o Chronic lung disease
o DIOS, gallstones, liver cirrhosis
examination of CF patient
- Hands: finger clubbing
- Face: nasal polyps
- Chest: hyperinflated, crepitiations, portacath (indwelling vascular access device)
- Abdomen: faecal mass (if constipated/DIOS), may have
Investigations (diagnosis and monitoring) for CF
- Chest radiograph: to assess for hyperinflation, there may be evidence of bronchial thickening (undertaken annually as part of annual assessment)
- Chloride sweat test (at diagnosis and annually) - >60mmol/L
- Microbiological assessment e.g. cough swab/sputum sample (at every clinical encounter)
- Glucose tolerance test(at annual assessment at teenage and beyond)
- Liver function test and coagulation (at annual assessment)
- Bone profile(at annual assessment)
- Lung function testing– spirometry / lung clearance index
the chloride sweat test
- This test measures the electrolyte concentration in sweat
- Sweat sample is collected by pilocarpine iontophoresis. Careful technique must be employed to ensure and adequate sample is collected to avoid inaccurate results. A common reason for failure of the test is an insufficient sample in a small baby (limit 3kg).
interpreting a chloride sweat test
- A sweat chloride >60mmol/L is suggestive of CF. A sweat chloride of 40-60mmol/L is borderline and should be repeated
- A single sweat test is not sufficient to diagnose CF, a second test or identification of genetic mutation should confirm the diagnosis.
- There are a number of reasons for a false positives/negatives.
complications of CF: resp
o Allergic bronchopulmonary aspergillosis (ABPA
o Bronchiectasis (do CT)
o Haemoptysis
o Pulmonary hypertension
o Pneumothorax
o Nasal polyps
complications of CF: GI
- Rectal prolapse (frequent passage of bulky stools)
- Distal intestinal obstruction syndrome (DIOS)
–>Obstruction of distal ileum and affects up to 10% of children with CF
–>Due to slower intestinal transit
–>Colicky abdominal pain - CF related liver disease
–>Cholestasis
–> Gallstones
–> Liver cirrhosis
complications of CF: endocrine
- CF related diabetes (CFRD)- older children
o DKA rarer due to not an absolute lack of insulin - Delayed puberty
- Osteoporosis
Sub or infertility
managment of CF
MDT approach e.g. GP, resp paediatrician, CF nurse, dietician, physiotherapist, psychologist and SW
- education
- resp
- GI
- preventing chest infections
CF managment: education
- Patient and family
- Segregation to reduce cross-infection
- When admitted in a side room
- Patiens who are pseudomonas naïve attened diff clinics to those with chronic infection
CF management: respiratory
- Physiotherapy
o Mucus clearance via twice-daily - Mucolytics and DNase
o DNase (dornase alpha) is inhaled and reduces viscosity of mucus by digesting DNA which is abundant in the sputum of patients with CF
o Hypertonic saline can aid airway clearance (and can be used at time of physiotherapy to further aid clearance)(limited evidence to support use in under 12 yrs). - Small molecule therapy- Ivacaftor/ lumicaftor
- Physical exercise
CF management: GI
- Creon
o Pancreatic enzyme supplementation for meals which contain fat - Fat soluble vitamins (ADEK)
- Monitor growth
o Metabolic demand may eb higher and so nutrition followed carefully for optimal growth
o May have poor weight gain – supplement meals. May need gastrotomy in extreme malnutrition
CF management: common chest infections
e.g. S.aureus, H. influenzae and Pseudomonas aeruginosa
- Sputum culture
- 2 weeks of antibiotics even if child asymptomatic
- High doses
- IV may be required if oral not responsive
- Prophylactic antibiotics recommended until age of 3
- Regular azithromycin recommended to improve lung function
Chronic infections in CF
e.g. pseudomonas aeruginosa
Associated with poor lung function
Biofilms aid colonisation and protection from host immunity and antibiotics
Long term antibiotics
Inhaled antibiotics
Annual review CF:
microbiological assessment, blood tests, lung functions tests, CXR, oral glucose tolerance test (OGTT)
Tracheobronchomalacia
Background
- “Floppy” airways
- Flaccidity of supporting cartilage
Types 3 groups
- Congenital
- Extrinsic compression
- Acquired
Management
- Nothing
- Prophylactic Antibiotics/physio
- CPAP
- Surgery
Prognosis very good (usually)!
Obstructive sleep apnoea background
- Apnoea is defined as a lack of breathing.
- Obstructive apnoea refers to a lack of airflow in the face of respiratory effort.
- It is most often associated with sleep.
- The obstructive sleep apnoea syndrome (OSAS) may be due to tonsillar/adenoidal hypertrophy, macroglossia, or micrognathia.
obstructive sleep apnoea causes
Causes
- Upper airway obstruction e.g. Adenotonsillar enlargement
- Genetic conditions e.g. Pierre Robin syndrome, trisomy- 21
OSA presentation
Presentation
- Snoring and sleep disturbance.
- Daytime sleepiness or inattention.
- Enuresis.
- Only about 15% of snoring children have significant airway obstruction.
investigations for OSA
- Sleep study
o Overnight pulse oximetry - Chest x-ray and ECG
o To examine for secondary heart cardiac consequences of airway obstruction
Management
- Depends on aetiology
- Surgery
o Tonsillectomy or adenoidectomy - CPAP (positive airway pressure support)
Paediatric long term ventilatory support
Who is eligible?
Any child below the age of 17 who is
- Medically stable
- Requires a mechanical aid for breathing either invasively by tracheostomy or by non-invasive mask interface for all or part of the 24 h day (after a failure to wean)
- For longer than 3 months
what may cause a child to require long term ventilatory support
- Neuromuscular
- Upper airway obstruction
- Obesity syndrome
- Hypoventilation
o Neuromuscular weakness
o Mechanical e.g. scoliosis - Central
o Brainstem lesions
o Neurological disorders - Trauma
goal/ types of long term ventilation
Goal
- Maintain adequate ventilation/ oxygenation
Types:
- Maintain open airways (pressure only i.e. CPAP)
o Upper airway obstruction
o Tracheobronchomalacia
- Provide artificial ventilation (pressue and rate i.e. BiPAP)
o Neuromuscular conditions
o Central hypoventilation
considerations for LTV
Interface
- Non-invasive e.g. facemask/ nasal
- Invasive e.g. tracheostomy
Duration
- Overnight only
- 24/7
- Somewhere in between
Modality
o CPAP
o BiPAP
considerations for LTV
Interface
- Non-invasive e.g. facemask/ nasal
- Invasive e.g. tracheostomy
Duration
- Overnight only
- 24/7
- Somewhere in between
Modality
o CPAP
o BiPAP
