Vascular: this would be an unusual presentation for cardiovascular disease to present with cough and breathlessness although congenital heart disease has to be considered. Acute problems such as pulmonary embolism found in adults would be very unusual in childhood.

Infective: (inflammation, immune,); infection and inflammatory disorders such as asthma would be the leading cause of an acute presentation with breathing difficulty in childhood with such symptoms representing the leading cause of hospital admission in the UK; underlying risk factors within the child must be considered in addition within this differential

Traumatic: in childhood a history of trauma is not always present particularly in the area of child protection. In addition spontaneous pneumothorax may occur most commonly in older adolescents and young adults

Autoimmune: as autoimmune chest conditions usually are manifest with fibrosis these symptoms are rare although very rarely conditions such as sarcoidosis may occur

Metabolic: (this includes inborn errors of metabolism) Consider whether a problem in another system for example metabolic acidosis in Diabetic Ketoacidosis may be driving apparent breathlessness (Kussmaul’s breathing)

Iatrogenic/idiopathic: consider ingestion / inhalation of foreign body

Neoplastic: respiratory tumours are very rare in childhood

Congenital/genetic: congenital lung abnormalities are well recognised although more commonly present in younger children; other genetic factors include conditions predisposing to infection and chronic chest disease such as Cystic Fibrosis or immunodeficiencies.

Degenerative/developmental: consider the effect of profound disability on the predisposition to both respiratory disease perhaps due to recurrent aspiration and structural chest wall abnormalities (such as scoliosis)

Endocrine/environmental: parental cigarette smoking is a very important factor in childhood respiratory disease

Functional: tics and functional coughs occur, with symptoms of acute anxiety sometime being difficult to distinguish from asthma like symptoms

Information regarding how unwell Harry is currently:

· Have any red flags for serious illness been noticed, for example has he been able to talk? Has he changed colour; been cyanosed?

Information regarding the diagnosis:

· Could you describe the pattern of the cough, was this productive and if so what colour was the sputum?

· Has he had a fever?

· Has he had any episodes like this before?

Yes as he has a history of marked breathlessness and difficulty in talking. This is a marker of severity of breathlessness. The duration of symptoms of significant breathlessness is also concerning.

The background history needs to be explored further.

Harry's early warning score is as follows:

Respiratory rate: 46/min = 4
SaO2: 89% in Air = 4
Heart rate: 120/min = 1
Blood pressure: 98/66 = 0
Capillary refill time: <2sec = 0
Conscious level: Alert = 0

TOTAL SCORE = 9

Yes - The score above 4 indicates that immediate intervention may be needed followed by close observation.

The observations suggest a high risk of respiratory failure.

A targeted assessment/examination of Harry’s cardiorespiratory system is needed to be able to initiate specific treatment, in addition to initiating high flow oxygen treatment. If any history of wheeze is present, a trial of nebulised salbutamol is indicated. Continuous monitoring of his observations, to be vigilant in case of deterioration, is then needed.

1.    Acute severe asthma

2.  Acute Pneumonia (Lower Respiratory Tract Infection/ LRTI)

1.    Cardiac Failure

2.    Upper airway obstruction including foreign body

                 3.Pneumothorax 

1.    Past medical history; previous chest symptoms

2.    Birth history; or example prematurity

3.    Immunisation history; risk for specific infections

               4. Growth and wellbeing; 

Yes:

1.    The structure of the examination is very similar but with a more flexible approach

2.    An increased emphasis is placed on looking for signs of increased respiratory effort and respiratory distress.

3.    Normal ranges for respiratory rate vary according to age.

4. Aspects such as a ‘carbon dioxide flap’ would be very unusual

VASCULAR – no symptoms or signs to suggest a cardiovascular cause for his symptoms

INFECTIVE,(INFLAMMATORY,IMMUNE)

Infection is likely given the history of fever and focal signs noted on respiratory examination. More information will be necessary to identify any underlying cause for infection however. There is no particular history to suggest unusual infection or TB but this may need to be explored further.

He has had asthma treatment, compliance and technique has been checked in the community and this treatment has not been felt to be effective for symptomatic chestiness or to reduce the frequency of his symptoms. The diagnosis of asthma is not certain.

No indication from the history or examination is present to indicate TRAUMA, AUTOIMMUNE, METABOLIC, IATROGENIC, IDIOPATHIC, NEOPLASTIC cause.

A Congenital/genetic cause for his recurrent infections and respiratory symptoms remains a possibility and further investigations will be needed.

No additional factors have been so far identified to indicate a Degenerative/developmental, Endocrine/environmental or Functional disorder 

1.    Sputum M,C&S; (or if he is not able to produce sputum (this is typical in children under approximately 7 years of age) a cough swab would be appropriate.

2.    Blood investigations;

· FBC looking for the white cell response to infection and anaemia which contribute to breathlessness

· CRP looking for elevation consistent with infection

· Blood culture and sputum culture; to try to identify a pathogen

· Urea and electrolytes to look for indications of dehydration.

· Blood gas; at this stage this may not add to the treatment although should he not improve this may give an indication of whether his impending respiratory failure at presentation is progressing.

· Serum lactate can contribute as it reflects the peripheral circulation in unwell children and can assist in the assessment of acutely ill children

· Blood Gas; this (Mainly capillary blood gas) is usually done in the most severely ill children, it is important to remember that this will remain normal until decompensation occurs late in the illness. Please review your previous information regarding interpretation of blood gases.

       3.Chest X-ray; looking for signs of acute infection and any persistent changes which may indicate on-going recurrent or atypical infection I the chest.

Lower respiratory tract infection

You would refer to your local antibiotic guidelines for choice of antibiotic treatment; as he is unwell & has not been drinking well you opt for Intravenous antibiotics using Amoxicillin as treatment for community acquired pneumonia, this is to be reviewed after reviewing the chest xray with senior colleagues and after 48 hours to ensure he is responding.

1.    Review of his CXR and comparison to previous films to look for signs indicating a chronic picture.

2.    Tests of immunity; immunoglobulins and subtypes (considering specific antibodies to immunisations), because there is a history of multiple previous infections and this may put him at risk for chronic lung damage or bronchiectasis.

3.    Specific tests for Cystic Fibrosis; (Harry was born in the Ukraine and therefore may not have had the routine neonatal screen for CF).

4. Discussions with the GP / asthma nursing team regarding appointments and possible issues of compliance with medication should be considered, as in children with asthma, chronic non compliance with preventer treatment can lead to recurrent presentations and chronic symptoms. Potential pre-existing lack of compliance may have an effect on the parental engagement in Harry’s future care.

Please revise the ABCDE system:

The most obvious changes are areas of shadowing in the lower zones, in particular obscuring the heart border on the right and left indicating consolidation.

It is reported to show: ‘bilateral streaky changes in both lower zones consistent with infection, evidence of bronchial wall thickening coexists in the lower and upper zones with possible evidence of chronic disease warranting further evaluation’.

          Sweat chloride test

.

Correct answer.

Children with evidence of chronic lung changes should be tested for cystic fibrosis with the sweat test continuing to be the part of the gold standard diagnosis. Genetic tests are likely to then be required.

·          Measurement of serum immunoglobulin levels

.

Correct answer.

Primary immune deficiencies may present with recurrent respiratory infections leading to supporative lung disease; immunoglobulin subtype levels measurements of responses to vaccinations and lymphocyte numbers / HIV test can also be helpful.

·          CT scan of the chest

.

Correct answer.

This can give further information regarding disruption of the architecture of the lung although this does not give definitive information regarding the cause of this in most instances unless a specific structural change is present for example congential abnormalities of the lung.

·          Bronchoscopy

.

This is not a diagnostic test for bronchiectasis but may be useful with lavage in giving microbiological information. (The rare occasion where this is diagnostic involves specific investigations looking for a foreign body usually in localised disease or at function of cilia, for Primary Ciliary Dyskinesia, a very rare condition, done under specific indications only where other causes have been excluded).

·          Culture of aspirate from the trachea

.

It does identify pathogens, but does not identify the underlying condition. However, the finding of specific organisms may be important for example staphylococci or pseudomonas would indicate underlying lung pathology rather than straightforward community acquired pneumonia. 

    ·          Histamine causes constriction of the airways due to release of secretions.

.Correct answer.

Due to degranulation of mast cells releases histamine and other inflammatory mediators leading to localised swelling and release of secretions narrowing airways.

·          IgE is important in the initial priming of the surface cells within the lungs and upper airways.

.Correct answer.

In the Sensitisation phase the allergen cross links with IgE; this phase is asymptomatic.

·          Children do not experience bronchoconstriction due to lack of smooth muscles surrounding the airways.

.

Younger children are less likely to respond to bronchodilators due to a lack of beta 2 receptors, it is partially true that in addition there is proportionally less musculature in comparison to adults.

·          Recurrent symptoms in childhood asthma are due to airway hyper responsiveness.

.Correct answer.

In established asthma inflammatory cell infiltrates including lymphocytes, basophils and eosinophils lead to relates of inflammatory mediators with subsequent exposure to both physical and allergenic factors   

    There is a high prevalence of cystic fibrosis in individuals of Irish descent.

.Correct answer.

The gene frequency is highest amongst those of northern European descent with a gene frequency in the UK of 1/25.

·          The CFTR Gene codes for the sodium channel across epithelial cell membranes.

The chloride transport is affected across epithelial cell membranes causing secretions to be thickened, sticky and predisposing to the blockage of pancreatic ducts, vas deferens and recurrent respiratory and sinus infections.

·          It arises due to abnormalities in the CFTR Gene

.Correct answer.

Multiple malformations have been identified in the structure and function of this gene leading to differences in the quantity, structure and function of the CFTR protein lead to the considerable variation in expression of the disease.

·          CF is inherited in an autosomal recessive fashion.

.Correct answer.

It is the most common serious autosomal recessive condition in the European Caucasian population (approximately 1/2500 live births).

·          Children in the UK with cystic fibrosis are diagnosed via the neonatal screen.

.Correct answer.

The majority (but not all) children with CF have a positive neonatal heel-prick screen for Immunoreactive Trypsinogen (IRT), this is released from the pancreas, usually in low levels but is usually high in CF. it can also be elevated in premature or unwell infants and is unreliable if taken after the second week of life. A negative screen indicates a low risk. Infants with a positive screen will warrant genetic testing for the common genetic changes noted in CF.

  Reliever inhalers are usually brown

·          Infants should always be prescribed nebulisers as they are too small to use a spacer.

.

Spacers for infants have a mask attachment for parents to place over their mouth & nose.

·          Children who present with wheeze who have been given a blue and brown inhaler by their GP should be assumed to have asthma.

This alone does not equate to diagnosis.

·          Teenagers should use a spacer with their metered dose inhaler

.Correct answer.

Technique without a spacer can be poor in all age groups - Technique should be assessed. Many teenagers will require a spacer.

 Weight gain

.Correct answer.

Typically weight loss is seen