Paediatrics (Joe) Flashcards
Define Pneumonia?
Lower Respiratory Tract Infection/ Pneumonia is caused by infection and subsequent inflammation of the alveoli and terminal bronchioles.
This leads to an entire bronchopulmonary segment or lobe becoming consolidated, which means that tissue is filled with inflammatory cells and oedema.
What are the main different causes of Pneumonia?
Bacteria
Atypical Bacteria
Viral
Fungal
What are the main bacterial causes of Pneumonia?
Streptococcus pneumonia
Staphylococcus aureus
Haemophilus influenzae
Moraxella catarrhalis
What are the main Atypical bacterial causes of Pneumonia?
Legions of Psittaci MCQ:
- Legionella pneumophilia
- Chlamydia psittaci
- Mycoplasma pneumonia
- Chlamydia pneumonia
- Coxiella burnettii (Q fever)
What are the main viral causes of pneumonia?
Respiratory Syncytial virus (RSV)
Influenza
Parainfluenza
What are the main causes of Pneumonia in Neonates, Infants and School age children?
Neonates: Group B Strep (Streptococcus Pyogenes)
Infants: Strep pneumoniae
School age: Strep pneumoniae, staph aureus, mycoplasma
What is a common cause of pneumonia in closed populations such as schools?
Mycoplasma pneumonia: Has extra respiratory symptoms of:
- Erythema multiforme, erythema nodosum
- Guillain-Barre Syndrome (and rarely other neurological complications e.g. aseptic meningitis, cerebellar disease, transverse myelitis).
- Cold agglutinin production with haemolytic anaemia
- Chlamydia pneumoniae
What are the clinical symptoms of pneumonia?
- Cough (typically wet and productive)
- SOB
- High fever (> 38.5ºC)
- Increased work of breathing
- Lethargy
- Delirium (acute confusion associated with infection)
What are some clinical signs of pneumonia?
- Tachypnoea (raised respiratory rate)
- Tachycardia (raised heart rate)
- Hypoxia (low oxygen)
- Hypotension (shock)
- Fever
*Confusion
What are the characteristic 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.
Reduced breath sounds
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.
What are the investigations for pneumonia?
1st Line:
- Sputum culture and throat swabs
- Bloods and blood cultures
- Capillary blood gas/ABG for acidosis and lactate
Gold Standard: Chest X-Ray
What scoring system is used to assess severity of pneumonia and further management?
CURB-65:
Confusion +/-
Urea >7
Respiratory Rate >30
Blood pressure: systolic < 90 or diastolic <60
More than 65 years old
CURB-65 mortality by score
- 0 or 1 - 1.5%
- 2 - about 10%
- 3 or more - 10% or more
CURB-65 interpretation and management
Management based on score:
- 0/1: home-based care, give oral amoxicillin for 5 days (macrolide e.g. clarithromycin, doxycycline or tetracycline if penicillin allergic).
- 2: hospital-based care, 7-10 day course of dual antibiotic therapy with amoxicillin (IV or oral) and a macrolide
- 3: Hospital/ITU-based care, 7-10 day course of dual antibiotic therapy with IV co-amoxiclav/ceftriaxone/tazocin and a macrolide.
What are the different types of pneumonia?
Community Acquired: Pneumonia that develops out in the community or less that 48 hours following hospital admission
Hospital Acquired:Pneumonia that develops more than 48 hours after hospital admission.
Most common organisms are: P. Aeruginosa, S aureus, Enterobacteria
Aspiration pneumonia: Occurs in patients with an unsafe swallow. On CXR the right main bronchus is wider and more vertical so it is more likely affected
What is the management for Pneumonia in Children?
- Manage at home with Analgesia
- If Admitted: Oxygen therapy and IV fluids
Antibiotics:
- Neonates: Broad Spectrum IV Antibiotics
- Infants: Amoxicillin/Co-amoxicalv
- Over 5s: Amoxicillin/Erythromycin
What is the management for CAP?
1st Line: Amoxicillin 5 days (macrolide used in pen allergy)
2nd Line: Amoxicillin +/- Macrolide (clarithromycin) 7-10 days
3rd Line IV Co-amoxiclav + Macrolide 7-10 days
What is the Management for HAP?
HAP within 5 days of admission: Co-amoxiclav or cephalosporin (e.g cefuroxime)
HAP more than 5 days after admission: Tazocin or cephalosporin (e.g. ceftazidime) or quinolone.
What are some complications of Pneumonia?
- Pleural effusion
- Parapneumonic collapse and Empyema (suspect if persistent, swinging fever with leucocytosis found after antibiotic therapy)
- Abscess (can be caused by S. pneumoniae, Klebsiella, staph aureus). Can develop pyopneumothorax.
- Pneumothorax
- Septicaemia
- Atrial fibrillation
- Post-infective bronchiectasis
What vaccines are available for prevention of Pneumonia?
Pneumococcal Vaccine: Routinely offered as 3 injections at 2 months, 4 months and 12-13 months
Define Pertussis?
Whooping Cough is a severe upper respiratory tract infection characterised by intense bouts of spasmodic coughing that may lead to apnoea in infants.
What is the Aetiology of Whooping Cough?
Pertussis is primarily caused by the Gram-negative bacterium, Bordetella pertussis.
What is the Epidemiology of Whooping Cough?
- Much less incidence now due to vaccination programme
- Vaccinations: 2,3,4 months, booster at 3 years 4 months
- Impacts infants more dramatically
What is the Clinical progression of Whooping Cough?
Catarrhal Phase:
- Lasts 1-2 weeks: coryzal symptoms
Paroxysmal Phase:
- Occurs week 3-6: characteristic ‘inspiratory whoop’
- Cough worse at night
- Spasmodic coughing episodes - can lead to vomiting
- Low grade fever
- Sore throat
Convalescent phase
- Downgrade of cough, may last up to 3 months
What are the Investigations for Whooping Cough?
- Nasal-pharyngeal swab with pertussis
- FBC
- Antibody test
What is the management of Whooping Cough?
Macrolide Antibiotic: Clarithromycin
- Prophylactic Abx give to close contacts who ae in higher risk health groups
- Isolation for 21 days after symptom onset or 5 days after antibiotics
Define Croup?
Croup, also referred to as acute laryngotracheobronchitis, is an acute respiratory syndrome that affects the larynx, trachea, and bronchi.
It is characterized by inflammation and oedema and swelling that results in partial obstruction of the upper airway at the larynx.
What is the epidemiology of Croup?
Typically affects children aged 6 months to 6 years with the highest incidence in Under 3s
More common in autumn and winter (spring?)
More common in boys
What is the Aetiology of Croup?
- Parainfluenza Virus
- Influenza
- Adenovirus
- Respiratory Syncytial Virus (RSV)
What are the clinical features of Croup?
-
Mild: Occasional barking cough with no audible stridor, no recession, child happy to
eat and drink as normal -
Moderate: Frequent barking cough with audible stridor at rest, suprasternal
recession, child not agitated -
Severe: Frequent barking cough, prominent stridor (high pitched breathing
indicating an upper airway obstruction), marked sternal recession, agitated and
distressed child potentially with tachycardia
What is the typical Presentation of Croup on examination and History?
- 1-4 days history of non-specific rhinorrhoea (thin, nasal discharge), fever and barking
cough - Worse at night
- Stridor
- Decreased bilateral air entry
- Tachypnoea
- Costal recession
What are some Respiratory Failure Red Flags?
- Drowsiness
- Lethargy
- Cyanosis
- Tachycardia
- Laboured breathing
What are some differential diagnoses for Croup?
- Epiglottitis: This presents with sudden onset high fever, drooling, dysphagia, and a muffled voice but lacks the classic barking cough.
- Foreign body aspiration: This usually involves a sudden onset of choking, coughing, or wheezing after an episode of eating or playing with small objects.
- Bacterial tracheitis: Patients with this condition often have a high fever and a severe, rapidly progressing respiratory distress. They might have a history of preceding viral infection.
- Asthma: Characterized by recurrent episodes of wheezing, shortness of breath, chest tightness, and coughing.
What are the investigations for Croup?
- While the diagnosis of croup is primarily clinical, throat swabs for viral PCR can help identify the causative virus.
- X-ray of the neck may show the classic “steeple sign” indicative of subglottic narrowing in severe or atypical cases.
What is the Management for Croup?
Mild Cases:
- Oral Dexamethasone 150mcg/kg repeated after 12 hours if required
More severe cases:
- Oral Dexamethasone
- Oxygen
- Nebulised Budesonide
- Nebulised adrenaline may be indicated in situations where there are significant concerns about airway patency.
- Intubation and ventilation (It is important to minimize distress in children with croup as crying can exacerbate upper airway obstruction.)
What are some possible complications of Croup?
- Otitis Media
- Dehydration due to reduced fluid Intake
- Superinfection: Pneumonia
Define Asthma?
Asthma is a chronic inflammatory airway disease leading to variable airway obstruction.
Characterised by airway hypersensitivity reversible airway obstruction and Bronchospasm
This bronchoconstriction is reversible with bronchodilators such as inhaled salbutamol.
What is the epidemiology of Asthma?
Affects nearly 10% of children in the UK
What is the Aetiology of Asthma?
Unclear but likely multifactorial
Family History of Asthma
History of Atopy (allergy/Eczema)
What are the risk factors for asthma?
- Genetic
- Prematurity
- Low birth weight
- Parental smoking
- Viral bronchiolitis in early life
- Cold air
- Allergen exposure
What are the clinical features of asthma?
Cough
Breathlessness
Bilateral Polyphonic Wheeze
Chest tightness
What drugs can trigger asthma?
Aspirin
Beta blockers
What may be found in sputum from an asthmatic?
Curschmann spirals:
Mucus plugs that look like casts of the small bronchi
Charcot-Leyden crystals:
From break down of eosiophils.
What is Samter’s Triad
Nasal Polyps
Asthma
Aspirin sensitivity
What is the Atopic Triad?
Atopic Rhinitis (Hayfever)
Allergic Asthma (Asthma)
Atopic Dermatitis (Eczema)
What are the classifications of asthma?
Intermittent
Mild Persistent
Moderate Persistent
Severe Persistent
What features of the presentation are suggestive of Asthma?
- Episodic symptoms with intermittent exacerbations
- Diurnal variability, typically worse at night and early morning
- Dry cough with wheeze and shortness of breath
- Typical triggers
- A history of other atopic conditions such as eczema, hayfever and food allergies
- Family history of asthma or atopy
- Bilateral widespread “polyphonic” wheeze heard by a healthcare professional
- Symptoms improve with bronchodilators
What features of the presentation are suggestive of different diagnosis to Asthma?
- Wheeze only related to coughs and colds, more suggestive of viral induced wheeze
- Isolated or productive cough
- Normal investigations
- No response to treatment
- Unilateral wheeze suggesting a focal lesion, inhaled foreign body or infection
What are some typical triggers for Asthma?
Dust (house dust mites)
Animals
Cold air
Exercise
Smoke
Food allergens (e.g. peanuts, shellfish or eggs)
What are some differential diagnoses for Asthma?
- Respiratory tract infections: Symptoms include fever, malaise, cough, and dyspnoea.
- Viral wheeze: Characterized by recurrent wheezing episodes associated with viral infections.
- Foreign body inhalation: Sudden onset of coughing, choking, and unilateral decreased breath sounds.
- Bronchiolitis: Predominantly in infants; symptoms include cough, wheeze, and feeding difficulties.
- Allergic reactions or anaphylaxis: Acute onset of urticarial rash, swelling, difficulty breathing, and potentially, shock.
How is Asthma Diagnosed?
No gold standard test or diagnostic criteria.
Children generally not diagnosed until they are at least 2-3 years old.
Clinical diagnosis based on typical history and examination
- Low probability of asthma: If child is symptomatic then referred to a specialist.
- Intermediate or high probability of asthma: A trial of treatment implemented and if it improves symptoms then a diagnosis can be made.
What are some investigations used to aid diagnosis of Asthma?
- Detailed history highlighting the episodic nature of wheeze, breathlessness, cough, and chest tightness
- Serial peak flow readings, both symptomatic and asymptomatic done several times a day over 2-4 weeks to indicate reversible airflow obstruction
- Spirometry with reversibility testing (In children aged >5)
- Trial of a short-acting beta agonist (SABA) inhaler in those suspected of having asthma
- Direct Bronchial Challenge Test with histamine or methacholine
- FeNO testing where cases are unclear
What are the parameters for spirometry with reversibility testing?
FEV1 Significantly Reduced
FVC Normal
FEV1:FVC <70% if poorly controlled
Use of bronchodilators should improve FEV1 by >12% AND increase FEV1 by 200ml
What are the principles for Asthma management?
- Start at the most appropriate step for the severity of the symptoms
- Review at regular intervals based on the severity
- Step up and down the ladder based on symptoms
- Aim to achieve no symptoms or exacerbations on the lowest dose and number of treatments
- Always check inhaler technique and adherence at each review
What is the medical management for Asthma in a patient Under 5 years old?
- Start a short-acting beta-2 agonist inhaler (e.g. salbutamol) as required
- Add an 8 week trial of low dose corticosteroid inhaler If symptoms reoccur within 4 weeks of trial ending then restart ICS
- Add an LRTA
- Refer to a specialist.
What is the medical management for Asthma in a patient 5-16 years old?
- Start a short-acting beta-2 agonist inhaler (e.g. salbutamol) as required
- Add a regular paediatric low dose corticosteroid inhaler
Assess Inhaler Technique
- Consider offering LRTA (monteleukast) and reviewing response in 4-8 weeks
- Consider stopping LRTA and add a long-acting beta-2 agonist inhaler (e.g. salmeterol). Continue salmeterol only if the patient has a good response.
- If asthma is uncontrolled then consider Changing ICS and LABA to MART Regime
- If asthma is uncontrolled on MART Regime then consider increasing ICS dose to paediatric moderate dose.
- Increase the dose of the inhaled corticosteroid to a high dose.
Referral to a specialist. They may require daily oral steroids.
What is the medical management for Asthma in a patient over 17 (adult)?
- Start a short-acting beta 2 agonist inhaler (e.g. salbutamol) as required
- Add a regular low dose corticosteroid inhaler
Assess Inhaler Technique
- Consider offering a leukotriene receptor antagonist (LTRA) in addition to the low dose ICS. Review the response to treatment in 4 to 8 weeks.
- Add a long-acting beta-2 agonist inhaler (e.g. salmeterol). Continue salmeterol only if the patient has a good response. (Consider stopping LRTA)
- If asthma uncontrolled, offer to change the person’s ICS and LABA maintenance therapy to a maintenance and reliever therapy (MART) regimen with a low maintenance ICS dose.
- Titrate the inhaled corticosteroid up to a moderate dose.
- If asthma is uncontrolled on a moderate maintenance ICS dose with a LABA (either as MART or a fixed-dose regimen), with or without an LTRA, consider a trial of an additional drug (Theophylline). Alternatively change ICS to high dose
Do ICS slow growth?
There is evidence that inhaled steroids can slightly reduce growth velocity and can cause a small reduction in final adult height of up to 1cm when used long term (for more than 12 months). This effect was dose-dependent, meaning it was less of a problem with smaller doses.
Explain that these are effective medications that work to prevent poorly controlled asthma and asthma attacks that could lead to higher doses of oral steroids being given. Poorly controlled asthma can lead to a more significant impact on growth and development. The child will also have regular asthma reviews to ensure they are growing well and on the minimal dose required to effectively control symptoms.
What are some side effects of ICS?
Stunted Growth
Oral thrush
Good inhaler technique can reduce this risk by ensuring that the medication reaches the lungs and not stays in the back of the mouth or throat
What are indications for good asthma control?
No Night time symptoms
Inhaler no more than 3 times a week
No breathing difficulties, cough or wheeze most days
Able to exercise without symptoms
Normal Lung Function Tests
What are some side effects of Salbutamol?
Fine Tremor
HYPOKALAEMIA
Headache
Palpitations
Muscle cramps
What is an Acute Exacerbation of Asthma?
A rapid deterioration in the symptoms of asthma. This could be triggered by any of the typical asthma triggers, such as infection, exercise or cold weather.
What is the presentation of an Acute asthma exacerbation?
- Progressively worsening shortness of breath
- Signs of respiratory distress
- Fast respiratory rate (tachypnoea)
- Expiratory wheeze on auscultation heard throughout the chest
- The chest can sound “tight” on auscultation, with reduced air entry
- Silent Chest: Airways are so tight it is not possible for the child to move enough air through the airways to create a wheeze. This might be associated with reduce respiratory effort due to fatigue.
(A less experienced practitioner may think because there is no respiratory distress and no wheeze the child is not as unwell, however in reality this a silent chest is life threatening.)
What are the different severities of an acute asthma exacerbation?
Moderate: PEF > 50% predicted
Severe: PEF < 50% predicted
Life threatening: PEF < 33% predicted
What are the features of a Moderate acute asthma exacerbation?
PEF > 50% predicted
Normal speech
What are the features of a Severe acute asthma exacerbation?
- PEF < 50% predicted
- O2 saturations < 92%
- Incomplete sentences
- Signs of respiratory distress
-
Respiratory rate:
- > 40 in 1-5 years
- > 30 in >5 years
-
Heart rate:
- > 140 in 1-5 years
- > 125 in >5 years
What are the features of a Life Threatening acute asthma exacerbation?
33, 92, CCHEST:
- PEF < 33% predicted
- <92% - Oxygen Stats
Cyanosis
Confusion/Consciousness/AMS
Hypotension
Exhaustion and poor respiratory effort
Silent Chest
Tachycardia
What are the staples of management in acute virally induced wheeze or asthma?
- Supplementary oxygen if required (i.e. oxygen saturations less than 94% or working hard)
- Bronchodilators (e.g. salbutamol, ipratropium and magnesium sulphate)
- Steroids to reduce airway inflammation: prednisone (orally) or hydrocortisone (intravenous)
- Antibiotics only if a bacterial cause is suspected (e.g. amoxicillin or erythromycin)
What is the stepwise progression of Bronchodilators in acute asthma exacerbations?
- Inhaled or nebulised salbutamol (a beta-2 agonist)
- Inhaled or nebulised ipratropium bromide (an anti-muscarinic)
- IV magnesium sulphate
- IV aminophylline
What is the Management for an acute asthma exacerbation for Moderate to Severe?
Maintain oxygen saturations between 94-98% with high flow oxygen if necessary.
- Administer inhaled salbutamol via spacer: 10 puffs every 2 hours
- Proceed to nebulised salbutamol if necessary
- Add nebulised ipratropium bromide
- If O2 saturations remain <92%, add magnesium sulphate
- Add intravenous salbutamol if no response to inhaled therapy
- If severe or life-threatening acute asthma is not responsive to inhaled therapy, add aminophylline
All patients should receive steroids (Oral Prednisolone or IV hydrocortisone) given IV only if the patient is unable to take the dose orally
If the patient is not responding to salbutamol or ipratropium, consult with a senior clinician
What is a typical step down regime for salbutamol?
10 puffs 2 hourly then 10 puffs 4 hourly then 6 puffs 4 hourly then 4 puffs 6 hourly.
What must be monitored when giving high doses of salbutamol?
Serum potassium as salbutamol drives potassium into cells
What are some differential diagnoses for acute asthma exacerbations?
- Pneumothorax: Very sudden onset, chest pain, possible deviation of the trachea
- Anaphylaxis: Very sudden onset, associated with antigen exposure
- Inhalation of a foreign body: Unilateral chest signs
- Cardiac arrhythmia: Chest pain or palpitations, tachycardia or changes in blood pressure
What should be considered before discharging a patient following an acute asthma exacerbation?
- When the child is well on 6 puffs 4 hours of Salbutamol
- Finish the course of steroids if started (typically 3 day course)
- Provide Safety net information about when to return to hospital or seek help
- Provide an individualised asthma action plan
Define Virally Induced Wheeze?
An acute wheezy illness caused by a viral infection.
Small children (typically under 3 years) have small airways.
When these small airways encounter a virus (commonly RSV or rhinovirus) they develop a small amount of inflammation and oedema, swelling the walls of the airways and restricting the space for air to flow.
This inflammation also triggers the smooth muscles of the airways to constrict, further narrowing the space in the airway.
What are some distinguishing features between a virally induced wheeze and Asthma?
- Presenting before 3 years of age
- No atopic history
- Only occurs during viral infections
- Asthma has other triggers such as exercise, cold weather, dust and strong emotions
What viruses are the common causes of a virally induced wheeze?
RSV
Rhinovirus
What are the clinically features of a virally induced wheeze?
Evidence of a viral illness (fever, cough and coryzal symptoms) for 1-2 days preceding the onset of:
- Shortness of breath
- Signs of respiratory distress
- Expiratory wheeze throughout the chest
What is the management for a virally induced wheeze?
- Supplementary oxygen if required (i.e. oxygen saturations less than 94% or working hard)
- Bronchodilators (e.g. salbutamol, ipratropium and magnesium sulphate) Max of 4 hourly up to 10 puffs
- Steroids to reduce airway inflammation: prednisone (orally) or hydrocortisone (intravenous)
What should you think if you hear a focal wheeze on auscultation?
Neither viral-induced wheeze or asthma cause a focal wheeze.
If you hear a focal wheeze be very cautious and investigate further for a focal airway obstruction such as an inhaled foreign body or tumour.
These patients will require an urgent senior review.
Define Bronchiolitis?
Bronchiolitis is a widespread chest infection, predominantly affecting infants aged 1-12 months.
Bronchiolitis describes inflammation and infection in the bronchioles, the small airways of the lungs.
What is the Epidemiology of Bronchiolitis?
Bronchiolitis epidemics are typically observed during winter months, with approximately 90% of affected children aged between 1-9 months.
The disease is rarely diagnosed in children older than 12 months.
Peaks in winter and spring months
What is the Aetiology of Bronchiolitis?
Respiratory Syncytial Virus (RSV) is the most common cause (80% of cases)
What are some risk factors for Bronchiolitis?
- Breastfeeding for < 2 months
- Smoke Exposure
- Older siblings who attend nursery/schools
- Chronic Lung disease of prematurity
Why are adults and children older than 2 years less affected by Bronchiolitis?
When a virus affects the airways of adults, the swelling and mucus are proportionally so small that it has little noticeable effect on breathing.
This swelling and constriction of the airway caused by a virus has little noticeable effect on the larger airways of an older child or adult, however due to the small diameter of a child’s airway, the slight narrowing leads to a proportionally larger restriction in airflow. This is described by Poiseuille’s law, which states that flow rate is proportional to the radius of the tube to the power of four. Therefore, halving the diameter of the tube decreases flow rate by 16 fold.
This causes the harsh breath sounds, wheeze and crackles heard on auscultation when listening to a bronchiolitic baby’s chest.
What are the clinical features of Bronchiolitis?
- Coryzal symptoms
- Signs of respiratory distress
- Dyspnoea (heavy laboured breathing)
- Tachypnoea (fast breathing)
- Poor feeding
- Mild fever (under 39ºC)
- Apnoeas: Episodes where the child stops breathing
- Wheeze and crackles on auscultation
What are the signs of respiratory distress in paediatrics?
- Raised respiratory rate
- Use of accessory muscles of breathing, such as the sternocleidomastoid, abdominal and intercostal muscles
- Intercostal and subcostal recessions
- Nasal flaring
- Head bobbing
- Tracheal tugging
- Cyanosis (due to low oxygen saturation)
- Abnormal airway noises
What is the typical RSV course in Bronchiolitis?
- Bronchiolitis usually starts as an upper respiratory tract infection (URTI) with coryzal symptoms.
- From this point around half get better spontaneously. The other half develop chest symptoms over the first 1-2 days following the onset of coryzal symptoms.
- Symptoms are generally at their worst on day 3 or 4.
- Symptoms usually last 7 to 10 days total and most patients fully recover within 2 – 3 weeks.
- Children who have had bronchiolitis as infants are more likely to have viral induced wheeze during childhood.
What are some differential diagnoses for Bronchiolitis?
- Asthma: Characterised by recurrent episodes of wheezing, breathlessness, chest tightness, and coughing, particularly at night or in the early morning.
- Pneumonia: Symptoms include cough with phlegm or pus, fever, chills, and difficulty breathing.
- Foreign body aspiration: This condition may present with sudden onset of respiratory distress, choking, gagging, wheezing, or coughing.
What are the investigations for Bronchiolitis?
Clinical Diagnosis
- Nasopharyngeal aspirate for RSV culture
- Chest X-rays may be considered in severe cases
What are some reasons to admit a patient with Bronchiolitis?
- Aged under 3 months or any pre-existing condition such as prematurity, Downs syndrome or cystic fibrosis
- 50 – 75% or less of their normal intake of milk
- Clinical dehydration
- Respiratory rate above 70
- Oxygen saturations below 92%
- Moderate to severe respiratory distress, such as deep recessions or head bobbing
- Apnoea’s
- Parents not confident in their ability to manage at home or difficulty accessing medical help from home
What is the management of Bronchiolitis?
Prophylaxis: Administration of Palivizumab in high-risk patients.
Supportive Care: Including adequate hydration and nutrition, and fever management with NG tube or IV fluids
Saline nasal drops and nasal suctioning: Can help clear secretions particularly prior to feeding
Oxygen Therapy: Supplementary oxygen if sats < 92%. This may be escalated to mechanical ventilation in severe cases.
Antiviral Therapy: Ribavirin may be used in severe cases.
What is Palivizumab?
An RSV Monoclonal antibody that targets the RSV.
A monthly injection is given as prevention against RSV.
Considered in:
- Children <9 months with chronic lung disease of prematurity
- Children < 2 years with severe immunodeficiency require long term ventilation
What is the main complication of Bronchiolitis?
Bronchiolitis Obliterans
Rare, chronic complication of bronchiolitis, colloquially known as popcorn lung
What is the pathophysiology of Bronchiolitis Obliterans?
- The bronchioles are injured due to infection or inhalation of a harmful substance, leading to an overactive cellular repair process and subsequent build-up of scar tissue.
- The scar tissue obstructs the bronchioles, impairing oxygen absorption in the body.
- The scarring and narrowing of the bronchioles may continue to worsen over time, potentially leading to respiratory failure
What is the aetiology of Bronchiolitis Obliterans?
- Viral infections, with Adenovirus being the most frequent.
- It may also develop as a complication following bone marrow or lung transplants.
What are the clinical features of Bronchiolitis Obliterans?
Symptoms of bronchiolitis obliterans are progressive and usually encompass:
Dry cough
Shortness of breath
Hypoxia
Wheezing
Lethargy
What are some investigations to diagnose Bronchiolitis Obliterans?
Chest X-rays: These may often appear normal in patients with bronchiolitis obliterans.
CT scan: Used to detect early lung changes and allows for an earlier diagnosis.
Lung biopsy: This is sometimes performed to confirm the diagnosis.
Pulmonary function tests: A significantly reduced FEV1 (16-21%) is often observed in bronchiolitis obliterans. Serial FEV1 measurements may be useful for monitoring lung transplant patients and detecting the condition early.
What is the management for Bronchiolitis Obliterans?
Supportive Management as there is no definitive cure
- Immunosuppressive agents: Tacrolimus, cyclosporin, mycophenolate mofetil, and prednisone have been used to treat bronchiolitis obliterans after transplant.
Define Cystic Fibrosis?
A progressive, autosomal recessive disorder that causes persistent lung infections and limits the ability to breathe over time.
What are the genetics of Cystic Fibrosis?
- Autosomal Recessive
- Cystic Fibrosis Transmembrane Conductance Regulatory Gene (CFTR)
- Chromosome 7
- Delta-F508
What is the Epidemiology of Cystic fibrosis?
Approximately 1 in 25 have the CFTR protein mutation
Probability of having a child with CF is 1 in 2500
Exam Question: Both parents are healthy, one sibling has cystic fibrosis and a second child does not have the disease, what is the likelihood of the second child being a carrier?
2 in 3
What is the pathophysiology of Cystic Fibrosis?
Mutation in CFTR causes it to become dysfunctional.
CFTR has reduced function meaning that less Cl-, Na+ and water are released into ductal secretions leading to the thickening of the mucus secretion.
What are some key consequences of the cystic fibrosis mutation?
- Thick pancreatic and biliary secretions that cause blockage of the ducts, resulting in a lack of digestive enzymes such as pancreatic lipase in the digestive tract
- Low volume thick airway secretions that reduce airway clearance, resulting in bacterial colonisation and susceptibility to airway infections
- Congenital bilateral absence of the vas deferens in males. Patients generally have healthy sperm, but the sperm have no way of getting from the testes to the ejaculate, resulting in male infertility
- Meconium ileus often the first sign of CF where the child does not pass meconium within 24 hours, abdominal distention and vomiting.
How does Cystic Fibrosis typically present?
- Meconium ileus is often the first sign
- recurrent respiratory tract infections
- Failure to thrive (faltering growth)
- Malabsorption syndromes
- Pancreatitis
What are the Symptoms of Cystic Fibrosis?
- Chronic cough
- Thick sputum production
- Recurrent respiratory tract infections
- Loose, greasy stools (steatorrhoea) due to a lack of fat digesting lipase enzymes
- Abdominal pain and bloating
- Parents may report the child tastes particularly salty when they kiss them, due to the concentrated salt in the sweat
- Poor weight and height gain (failure to thrive)
What are the clinical signs of Cystic Fibrosis?
- Low weight or height on growth charts
- Nasal polyps
- Finger clubbing
*Crackles and wheezes on auscultation
- Abdominal distention
What are the causes of clubbing in children?
- Hereditary clubbing
- Cyanotic heart disease
- Infective endocarditis
- Cystic fibrosis
- Tuberculosis
- Inflammatory bowel disease
- Liver cirrhosis
What are some differential diagnoses for Cystic Fibrosis?
- Bronchiectasis: Chronic cough, recurrent chest infections, and production of large amounts of sputum
- Asthma: Chronic cough, wheezing, shortness of breath, chest tightness
- Chronic Obstructive Pulmonary Disease (COPD): Chronic cough, recurrent chest infections, shortness of breath, wheezing
- Gastroesophageal Reflux Disease (GORD): Heartburn, regurgitation, chest pain, cough, and dysphagia
- Coeliac Disease: Diarrhoea, bloating, weight loss, fatigue, anaemia
How is Cystic Fibrosis Diagnosed?
Newborn blood spot testing is performed on all children shortly after birth and picks up most cases
The sweat test is the gold standard for diagnosis
Genetic testing for CFTR gene can be performed during pregnancy by amniocentesis or chorionic villous sampling, or as a blood test after birth
How does the Cystic fibrosis Sweat Test work?
Gold standard investigation for CF
- A patch of skin is chosen for the test, typically on the arm or leg.
- Pilocarpine is applied to the skin on this patch.
- Electrodes are placed either side of the patch and a small current is passed between the electrodes.
- This causes the skin to sweat.
- The sweat is absorbed with lab issued gauze or filter paper and sent to the lab for testing for the chloride concentration.
- The diagnostic chloride concentration for cystic fibrosis is more than 60mmol/l.
Why does CF lead to recurrent chest infections?
Patients with cystic fibrosis struggle to clear the secretions in their airways. This creates a perfect environment with plenty of moisture and oxygen for colonies of bacteria to live and replicate.
What are the key colonisers of the lungs in CF?
Staph aureus
Pseudomonas Aeruginosa
Haemophilus influenzae
Klebsiella pneumonia
E.coli
What is the management of CF?
MDT Input
- Chest physiotherapy several times a day is essential to clear mucus and reduce the risk of infection and colonisation
- Exercise improves respiratory function and reserve, and helps clear sputum
- High calorie diet is required for malabsorption, increased respiratory effort, coughing, infections and physiotherapy
- CREON tablets to digest fats in patients with pancreatic insufficiency (these replace the missing lipase enzymes)
- Prophylactic flucloxacillin to reduce the risk of bacterial infections (particularly staph aureus)
- Bronchodilators such as salbutamol inhalers can help treat bronchoconstriction
- Nebulised DNase (dornase alfa) is an enzyme that can break down DNA material in respiratory secretions, making secretions less viscous and easier to clear
- Nebulised hypertonic saline
- Vaccinations including pneumococcal, influenza and varicella
What are some more long term treatment options for CF?
- Lung transplantation is an option in end stage respiratory failure
- Liver transplant in liver failure
- Fertility treatment involving testicular sperm extraction for infertile males
- Genetic counselling
Define Acute Epiglottitis?
A rapidly progressive infection (typically with Haemophilus influenzae Type B) that leads to inflammation of the epiglottis and adjacent tissues.
This inflammation can swiftly progress to blockage of the upper airway within hours, posing a risk of death.
Acute Epiglottitis is a life threatening emergency
What is the Epidemiology of Acute Epiglottitis?
Most common in children aged 1-6 years
Can occur at any age
Rare condition due to the routine vaccination program against haemophilus
What may be a Typical exam presentation of Acute Epiglottitis?
In you exams keep a lookout for an unvaccinated child presenting with a fever, sore throat, difficulty swallowing that is sitting forward and drooling and suspect epiglottitis.
What is the Aetiology of Acute Epiglottitis?
Primarily Haemophilus influenzae Type B
What are the clinical features of Acute Epiglottitis?
- Patient presenting with a sore throat and stridor
- Drooling
- Tripod position: sat forward with a hand on each knee
- High fever
- Difficulty or painful swallowing
- Muffled voice
- Scared and quiet child
- Septic and unwell appearance
What are some differential diagnoses for Acute Epiglottitis?
- Croup: Characterized by a barking cough, inspiratory stridor, and hoarseness.
- Peritonsillar abscess: Presents with severe throat pain, muffled “hot potato” voice, drooling, and trismus (difficulty opening the mouth).
- Bacterial tracheitis: Severe respiratory distress, high fever, and purulent tracheal secretions can be noted.
What are the investigations for Acute Epiglottitis?
If the patient is well:
- Laryngoscope to visualise inflamed epiglottis
If the patient is acutely unwell and epiglottitis is suspected:
- Investigations should not be performed: ensure patient is stable
- Performing a lateral X-ray of the neck shows a characteristic “thumb sign” or “thumbprint sign”. This is caused by the oedematous and swollen epiglottis.
What is the management of Acute Epiglottitis?
- Do not examine or upset the child without senior support to prevent prompt closing of the airway due to distress
- Securing the airway, possibly through endotracheal intubation, as a first priority (potential for Tracheostomy)
- Transfer patient to ICU
- Culturing and examination of the throat once the airway is secure
- Administration of IV antibiotics, typically cefuroxime and steroids (dexamethasone)
What is the prognosis for Acute Epiglottitis?
Most patients recover without requiring intubation.
Death can occur in severe cases or if it is not diagnosed and managed in time.
Common complication of an epiglottic abscess forming which is a collection of pus around the epiglottis. This is also life threatening and is managed similarly to epiglottitis
What is Respiratory Distress Syndrome
Affects premature neonates, before the lungs start producing adequate surfactant,
common in below 32 week babies.
What is the Pathophysiology of Respiratory Distress Syndrome?
Inadequate surfactant leads to high surface tension within alveoli leading to
atelectasis (lung collapse) as it is more difficult for the alveoli and the lungs to expand
leading to inadequate gaseous exchange and hypoxia, hypercapnia and respiratory
distress.
What is the Management of Respiratory Distress Syndrome?
- Dexamethasone is given to mothers with suspected or confirmed preterm labour to increase production of surfactant and reduce the incidence and severity of respiratory distress syndrome in the baby
- Intubation and ventilation may be needed to fully assist breathing if the distress is severe
- Endotracheal surfactant, which is artificial surfactant delivered into the lungs via an endotracheal tube
- CPAP via a nasal mask to keep the lungs inflated during breathing
- Supplementary oxygen to maintain sats between 91 and 95%
What are some short term complications of Respiratory Distress Syndrome?
- Pneumothorax
- Infection
- Apnoea
- Intraventricular Haemorrhage
- Pulmonary Haemorrhage
- Necrotising Enterocolitis
What are some Long term complications of Respiratory Distress Syndrome?
- Chronic lung disease of prematurity
- Retinopathy of Prematurity
- Neurological, hearing and visual impairment
What is Bronchopulmonary Dysplasia?
Infants who still require oxygen at a postmenstrual age of 36 weeks are described as having BPD
Define Otitis Media?
Otitis media is an infection-induced inflammation of the middle ear, frequently occurring after a viral upper respiratory tract infection.
What is the pathophysiology of Otitis Media?
- The middle ear is the space that sits between the tympanic membrane (ear drum) and the inner ear.
- This is where the cochlea, vestibular apparatus and nerves are found.
- It is a very common site of infection in children.
- The bacteria enter from the back of the throat through the eustachian tube.
- A bacterial infection of the middle ear is often preceded by a viral upper respiratory tract infection.
What is the Epidemiology of Otitis media?
Otitis media is a common condition, predominantly affecting young children.
What is the aetiology of Otitis Media?
The primary cause of otitis media is bacterial infection, particularly common in young children, often following a viral upper respiratory tract infection.
What bacteria are common causes of Otitis Mdia?
Streptococcus pneumoniae
Other:
- Haemophilus influenzae
- Moraxella catarrhalis
- Staphylococcus aureus
What are the different Types of Otitis Media?
- Acute Otitis Media: Deep-seated pain, impaired hearing, systemic illness, and fever. The tympanic membrane may show blood vessel injection and diffuse erythema.
- Chronic Benign Otitis Media: Characterized by a dry tympanic membrane perforation without chronic infection.
- Chronic Secretory Otitis Media (Glue Ear): Persistent pain lasting several weeks after the initial episode with an abnormal-looking drum and reduced mobility of the membrane.
- Chronic Suppurative Otitis Media: Persistent purulent drainage through the perforated tympanic membrane.
What is the clinical presentation of Otitis media?
- Ear pain with reduced hearing
- Fever
- Coryzal symptoms: sore throat and general malaise
- Irritability
- feeling of fullness in the ear
- Discharge if tympanic membrane is perforated
- If the infected affects the vestibular system then balance issues and vertigo
What are the investigations for Otitis Media?
Clinical Diagnosis
- Good history
- Physical examination using an Otoscope to visualise the Tympanic membrane
What is seen on Otoscopy in Otitis Media?
Otitis media will give a bulging, red, inflamed looking membrane.
When there is a perforation, you may see discharge in the ear canal and a hole in the tympanic membrane.
What are some differential diagnoses for Otitis Media?
- Upper respiratory tract infection: Symptoms include a runny nose, cough, and sore throat.
- Otitis externa: characterized by pain exacerbated by tugging of the auricle, accompanied by otorrhea and possible hearing loss
- Mastoiditis: presenting with postauricular pain, erythema, and swelling, as well as protrusion of the ear
- Temporomandibular joint disorder: characterized by jaw pain, difficulty in opening the mouth, and clicking or popping sounds during jaw movement
What is the management of Otitis Media?
Admit:
- Any child under 3 months with a temp of 38 degrees
- Any child of 3-6 months with a temp of >39 degrees
Pain and Fever:
- Treat with simple analgesia such as paracetamol or ibuprofen
Antibiotics:
- Most cases will resolve without need for Abx
- Delayed Prescription: ask the parents to collect Abx prescription in 3-4 days if symptoms have not improved by then
- Immediate Prescription: Given to those who are systemically unwell or at high risk of complications (immunocompromised)
- Amoxicillin for 5 days is first line. Alternatives are erythromycin and clarithromycin
Safety net: offer education to parents and patients on when to seek further medical attention
What are some complications of Otitis Media?
- Otitis medial with effusion
- Hearing loss (usually temporary)
- Perforated eardrum
- Facial nerve palsy
- Recurrent infection
- Mastoiditis (rare)
- Abscess (rare)
What are some Life threatening Complications of Otitis Media?
Meningitis: An important and life-threatening complication presenting with sepsis, headache, vomiting, photophobia, and phonophobia.
Sigmoid sinus thrombosis: Patients present with sepsis, swinging pyrexia, and meningitis.
Brain abscess: A patient will present with sepsis and neurological signs due to compression of cranial nerves.
Define Glue Ear
Otitis Media with effusion
The middle ear becomes full of fluid, causing a loss of hearing in that ear.
The Eustachian tube connects the middle ear to the back of the throat. It helps drain secretions from the middle ear. When it becomes blocked, this causes middle ear secretions (fluid) to build up in the middle ear space.
What is the Presentation of Glue Ear?
- Reduction of hearing in that ear
- Infection of the middle ear (Otitis media)
- Persistent pain lasting several after the initial episode
What is seen on Otoscopy in Glue ear?
Otoscopy can show a dull tympanic membrane with air bubbles or a visible fluid level although it can look normal.
What is the management of Glue Ear?
- Referral for audiometry to help establish the diagnosis and extent of hearing loss.
- Glue ear is usually treated conservatively, and resolves without treatment within 3 months
- Children with co-morbidities affecting the structure of the ear, such as Down’s syndrome or cleft palate may require hearing aids or grommets.
What are Grommets?
- Tympanostomy Tubes (Grommets are tiny tubes inserted into the Tympanic membrane by and ENT surgeon.
- They allow for fluid to drain from the middle ear through the tympanic membrane and into the ear canal.
- Grommets usually fall out within a year and only 1/3 of patients require further grommets to be inserted for persistent glue ear.
What are some Congenital causes of hearing loss?
- Genetic Deafness: autosomal recessive or autosomal dominant conditions. (eg. Pendred’s Syndrome)
- Maternal rubella or CMV infection during pregnancy
- Associated syndromes: Downs
What are some Perinatal causes of hearing loss?
- Prematurity
- Hypoxia during or after birth
What are some Post natal causes of Hearing Loss?
- Jaundice
- Meningitis and Encephalitis
- Otitis media and Glue Ear
- Chemotherapy
What is the Newborn hearing screening Programme (NHSP)?
- Tests hearing in all neonates.
- Uses equipment that delivers sound to each eardrum individually to check for a response.
- Can identify congenital hearing problems early.
How may children with hearing problems present?
- Picked up via NHSP
Parental Concerns about hearing or behavioural changes associated with not being able to hear:
- Ignoring calls or sounds
- Frustration or bad behaviour
- Poor speech and language development
- Poor school performance
What investigations are used to assess hearing loss?
History and Physical Examination
Otoscope
Audiometry Testing
How do you interpret and audiogram?
X-axis: Frequency in Hertz (Hz)
Y-axis: Volume in decibles (dB)
Hearing is tested to establish the minimum volume required for a patient to hear each frequency
Normal Hearing: all readings between 0 and 20 dB
Sensorineural hearing loss: both air and bone conduction readings will be more than 20 dB, plotted below the 20 dB line on the chart. This may affect only one side, one side more than the other or both sides equally.
Conductive hearing loss: bone conduction readings will be normal (between 0 and 20 dB), however air conduction readings will be greater than 20 dB, plotted below the 20 dB line on the chart. In conductive hearing loss, sound can travel through bones but is not conducted through air due to pathology along the route into the ear.
Mixed hearing loss: both air and bone conduction readings will be more than 20 dB, however there will be a difference of more than 15 dB between the two (bone conduction > air conduction).
What is the management for hearing loss?
Establishing diagnosis is the first step
MDT Team input for support with hearing, speech, language and learning
- Speech and language therapy
- Educational Psychology
- ENT Specialist
- Hearing aids for children who retain some hearing
- Sign language
Define Orbital Cellulitis?
Orbital cellulitis is a sight- and life-threatening emergency. It describes infection of the structures behind the orbital septum around the eyeball.
Define Periorbital/Preseptal Cellulitis?
An eyelid and skin infection in front of the orbital septum (in front of the eye). It presents with swollen, red, hot skin around the eyelid and eye.
What is the Epidemiology of Orbital cellulitis and Periorbital Cellulitis?
Orbital cellulitis is less common than preseptal cellulitis, with the latter accounting for 80% of cases, mostly occurring in children under the age of 10.
What are the risk factors for Orbital/periorbital cellulitis?
- Trauma
- Surgical – ocular, adnexal or sinus
- Sinus disease – ethmoidal sinusitis is the most common site of infection that spreads to the orbit
- Other facial infections – preseptal, dental abscess or dacryocystitis
What are the clinical features of Periorbital Cellulitis?
The typical patient with preseptal cellulitis is a child with an erythematous swollen eyelid, mild fever and erythema surrounding the orbit.
What are the clinical features of Orbital Cellulitis?
- Periocular pain and swelling
- Fever
- Malaise
- Erythematous, swollen and tender eyelid
- Chemosis
- Proptosis
- Restricted eye movements +/– diplopia
What are the important findings that suggest Periorbital cellulitis and not Orbital?
- No proptosis
- Normal eye movements
- No chemosis
- Normal optic nerve function
What are the investigations for Orbital cellulitis?
Blood tests: FBC, CRP to screen for raised inflammatory markers
Swabs sent for microscopy, culture and sensitivity
CT Sinus and Orbit with contrast is gold standard: investigation to distinguish orbital cellulitis from preseptal cellulitis
What is the management of Orbital Cellulitis?
Patients with orbital cellulitis require admission for IV antibiotics and close monitoring with input from the ophthalmology, ENT and Medical teams
May require surgical drainage
What is the management of Periorbital cellulitis?
Young or systemically unwell children should be admitted for IV antibiotics.
Otherwise, treatment is with oral antibiotics and daily outpatient review.
Vulnerable patients may require admission for monitoring in case of progression to Orbital cellulitis
What is Strabismus?
A “Squint” refers to the misalignment of the eyes.
When they are not aligned the images on the retina do not match and the person experiences double vision.
What is the possible progression of Strabismus?
- When this occurs in childhood, before the eyes have fully established their connections with the brain, the brain will cope with this misalignment by reducing the signal from the less dominant eye.
- This results in one eye they use to see (the dominant eye) and one eye they ignore (the “lazy eye”).
- If this is not treated, this “lazy eye” becomes progressively more disconnected from the brain and over time the problem becomes worse.
- This is called amblyopia.
What are Concomitant Squints?
Squints due to differences in the control of the extra ocular muscles
What are Paralytic Squints?
Squints due to paralysis in one or more of the extra ocular muscles
These are rare
Define Strabismus
The eyes are misaligned
Define Amblyopia
The affected eye becomes passive and has reduced function compared to the other dominant eye
Define Esotropia
inward positioned squint (affected eye towards the nose)
Define Exotropia
outward positioned squint (affected eye towards the ear)
Define Hypertropia
upward moving affected eye
Define Hypotropia
downward moving affected eye
What are the causes of Squints?
Usually idiopathic
Other causes of squint include:
- Hydrocephalus
- Cerebral palsy
- Space occupying lesions, for example retinoblastoma
- Trauma
What are the investigations for a Squint?
- General inspection
- Eye movements
- Fundoscopy (or red reflex) to rule out retinoblastoma, cataracts and other retinal pathology
- Visual acuity
Examination Tests:
- Hirschberg’s Test
- Cover Test
What is Hirschberg’s Test?
Shine a pen-torch at the patient from 1 meter away. When they look at it, observe the reflection of the light source on their cornea.
The reflection should be central and symmetrical.
Deviation from the centre will indicate a squint. Make a note of the affected eye and the direction the eye deviates.
What is the Cover Test for Squints?
Cover one eye and ask the patient to focus on an object in front of them. Move the cover across to the opposite eye and watch the movement of the previously covered eye.
If this eye moves inwards, it had drifted outwards when covered (exotropia) and if it moves outwards it means it had drifted inwards when covered (esotropia).
What is the Management of a Squint?
Occlusive Patch: Used to Cover the good eye and force the weaker eye to develop.
Atropine Drops: In the good eye causing vision in that eye to become blurred and force the patient to use the other.
Management is coordinated by an Ophthalmologist
Why should Squints be treated as soon as possible?
Up until the age of 8 years the visual fields are still developing, therefore treatment needs to start before 8 years. The earlier the better. Delayed treatment increases the risk of the squint becoming permanent.
Define Febrile Convulsions?
Febrile seizures are a type of seizure that occur in association with a fever, without evidence of intracranial infection or defined cause.
These seizures are typically short-lived, lasting less than 15 minutes, and are tonic-clonic in nature.
What is the Epidemiology of Febrile Convulsions?
- Febrile seizures are relatively common, affecting approximately 3% of children.
- The seizures predominantly occur in children aged between 6 months and 5 years.
What is the aetiology of Febrile Convulsions?
Febrile seizures occur due to an abrupt rise in body temperature, often related to an infection.
Both viral and bacterial infections can trigger febrile seizures, with the most common infections including upper respiratory tract infections, ear infections, and the common childhood exanthems
What are the types of Febrile Convulsion?
Simple Convulsion:
- Simple febrile convulsions are generalised, tonic clonic seizures. They last less than 15 minutes and only occur once during a single febrile illness.
Complex Convulsion
- Consist of partial or focal seizures, last more than 15 minutes or occur multiple times during the same febrile illness.
What are the clinical features of a Febrile Convulsion?
- High fever, often over 38°C
- Tonic-clonic seizures, which might involve rhythmic jerking of arms and legs and loss of consciousness
- Postictal drowsiness or confusion following the seizure
What are some differential diagnoses for Febrile Convulsions?
- Meningitis: presents with fever, headache, neck stiffness, and altered mental status
- Encephalitis: symptoms include fever, headache, altered mental status, seizures, and neurological deficits
- Seizures due to electrolyte imbalances: typically associated with altered mental status, muscle twitching or cramping, and fatigue
- Epilepsy: recurrent seizures without a fever, can be accompanied by postictal confusion and fatigue
- Trauma: Always consider non-accidental injury
- Syncopal Episode
- Intracranial Space occupying lesions: Brain tumours or haemorrhage
What are the investigations for Febrile Convulsions?
Clinical Diagnoses
Investigations to find underlying cause of Fever:
Bloods
Lumbar Puncture
Electroencephalogram (EEG)
What is the management for Febrile Convulsions?
- Identify source and manage fever with simple analgesia: Paracetamol/Ibuprofen
- Instructions on appropriate use of antipyretics
- Caution against prophylactic use of antipyretics
- Advice against sponging the child to cool them down
- Safety net advice should another seizure occur
What advice should parents be given if another convulsion occurs?
- Stay with the child
- Put the child in a safe place, for example on a carpeted floor with a pillow under their head
- Place them in the recovery position and away from potential sources of injury
- Don’t put anything in their mouth
- Call an ambulance if the seizure lasts more than 5 minutes
What is the prognosis for Febrile Convulsions?
They do not typically cause lasting damage.
2-7.5% risk of developing Epilepsy after a simple febrile convulsion
10-20% risk of developing Epilepsy after a complex febrile convulsion
What is West Syndrome?
Infantile spasms
A rare disorder starting in infancy at around 6 months of age which is characterised by clusters of full body spasms.
What are the first line treatments for West Syndrome?
Prednisolone
Vigabatrin
What is the prognosis of West Syndrome?
Poor Prognosis.
1/3 die by age 25 however 1/3 are seizure free
What is Global Developmental Delay?
Refers to a child displaying slow development in all developmental domains.
Must remember that children develop at different rates and so there is a good amount of flexibility in the milestones
What are some potential causes of Global Developmental Delay?
- Down’s syndrome
- Fragile X syndrome
- Foetal alcohol syndrome
- Rett syndrome
- Metabolic disorders
What is Gross motor delay?
A developmental delay that is specific to the gross motor domain
What are some potential causes of Gross Motor Delay?
- Cerebral palsy
- Ataxia
- Myopathy
- Spina bifida
- Visual impairment
What is Fine Motor Delay?
A developmental delay that is Specific to the Fine Motor Domain
What are some potential causes of Fine Motor Delay?
- Dyspraxia
- Cerebral palsy
- Muscular dystrophy
- Visual impairment
- Congenital ataxia (rare)
What is Language Delay?
A developmental delay that is specific to the speech and language domain
What are some potential causes of Speech and Language Delay?
- Specific social circumstances, for example exposure to multiple languages or siblings that do all the talking
- Hearing impairment
- Learning disability
- Neglect
- Autism
- Cerebral palsy
What is Personal and Social Delay?
A developmental delay that is specific to the personal and social domain
What are some potential causes of Personal and Social Delay?
- Emotional and social neglect
- Parenting issues
- Autism
Define Gastro-oesophageal Reflux?
Gastro-oesophageal reflux is where contents from the stomach reflux through the lower oesophageal sphincter into the oesophagus, throat and mouth.
What is the Pathophysiology of Gastro-Oesophageal Reflux in infants?
In babies there is immaturity of the lower oesophageal sphincter, allowing stomach contents to easily reflux into the oesophagus.
It is normal for a baby to reflux feeds, and provided there is normal growth and the baby is otherwise well this is not a problem, however it can be upsetting for parents.
This usually improves as they grow and 90% of infants stop having reflux by 1 year.
What is the Presentation of Gastro-oesophageal reflux in infants?
Most babies will have some Reflux
Sometimes the reflux may increase and cause the baby distress leading to:
- Chronic cough
- Hoarse cry
- Distress, crying or unsettled after feeding
- Reluctance to feed
- Pneumonia
- Poor weight gain
- Vomiting
Children over 1 year may experience similar symptoms to adults with GORD such as heart burn, acid regurgitation, chest pain, nocturnal cough and bloating
What are some potential causes of Vomiting in infants?
- Overfeeding
- Gastro-oesophageal reflux
- Pyloric stenosis (projective vomiting)
- Gastritis or gastroenteritis
- Appendicitis
- Infections such as UTI, tonsillitis or meningitis
- Intestinal obstruction
- Bulimia
What are the red flag signs for reflux?
- Not keeping down any feed: pyloric stenosis or intestinal obstruction
- Projectile or forceful vomiting: pyloric stenosis or intestinal obstruction
- Bile stained vomit: intestinal obstruction
- Haematemesis or melaena: peptic ulcer, oesophagitis or varices
- Abdominal distention: intestinal obstruction
- Reduced consciousness, bulging fontanelle or neurological signs: meningitis or raised intracranial pressure
- Respiratory symptoms: aspiration and infection
- Blood in the stools: gastroenteritis or cows milk protein allergy
- Signs of infection: pneumonia, UTI, tonsillitis, otitis or meningitis
- Rash, angioedema and other signs of allergy: cows milk protein allergy
- Apnoea’s are a concerning feature: May indicate serious underlying pathology and need urgent assessment
What are some possible investigations used in gastro-oesophageal reflux in infants where there may be underlying pathology?
Usually a clinical diagnosis and no Ix needed
May use:
- Barium Meal
- Endoscopy
- Fundoplication
What is the management for infants who have gastro-oesophageal reflux?
First-line management includes: reassurance and practical advice, such as:
- Small, frequent meals
- Burping regularly to help milk settle
- Not over-feeding
- Keep the baby upright after feeding (i.e. not lying flat)
Further management, if still bothersome, include:
- Gaviscon mixed with feeds
- Thickened milk or formula (specific anti-reflux formulas are available)
- Proton pump inhibitors (e.g., omeprazole) where other methods are inadequate
What is Sandifer’s Syndrome?
This is a rare condition causing brief episodes of abnormal movements associated with gastro-oesophageal reflux in infants. The infants are usually neurologically normal.
The key features are:
- Torticollis: forceful contraction of the neck muscles causing twisting of the neck
- Dystonia: abnormal muscle contractions causing twisting movements, arching of the back or unusual postures
What is the Prognosis for Sandifer’s Syndrome?
Condition tends to resolve as the reflux is treated or improves. Generally a good outcome
Differentials include more serious conditions such as infantile spasms (West Syndrome) and Seizures
Define Pyloric Stenosis?
Hypertrophy of the Pyloric Sphincter causing narrowing of the gastric outlet leading to gastric outlet obstruction.
What is the Pathophysiology of Pyloric Stenosis?
- The pyloric sphincter is a ring of smooth muscle the forms the canal between the stomach and the duodenum.
- Hypertrophy (thickening) and therefore narrowing of the pylorus is called pyloric stenosis. This prevents food traveling from the stomach to the duodenum as normal.
- After feeding, there is increasingly powerful peristalsis in the stomach as it tries to push food into the duodenum.
- Eventually it becomes so powerful that it ejects the food into the oesophagus, out of the mouth and across the room. This is called “projectile vomiting”.
What is the Epidemiology of Pyloric Stenosis?
Incidence: Affects 1-3 per 1000 live births.
Age: Predominantly presents in infants aged 6-8 weeks old.
Gender: It is more prevalent in males compared to females.
What is the Aetiology of Pyloric Stenosis?
Multifactorial
- Genetics: Pyloric stenosis can run in families and is more common in children of parents who had the condition.
- Gender: Males are more frequently affected.
- Prematurity: Infants born prematurely have a higher risk of pyloric stenosis.
What are the clinical features of Pyloric Stenosis?
- Post Prandial Vomiting: Projectile in nature that occurs after feeds. Intensity of vomiting may increase as obstruction worsens
- Palpable mass: During or after feeding, a firm round mass can be felt in the upper abdomen that feels like a large smooth olive. This is the Hypertrophied Pyloric Sphincter
- Hypochloric Metabolic Acidosis: Baby is vomiting hydrochloric acid from the stomach which is causing a metabolic acidosis.
Other:
- Weight loss and failure to thrive
- Constipation
- Visible Peristalsis
What are some differential diagnoses for Pyloric Stenosis?
- Gastroenteritis: Presents with diarrhoea, vomiting, and fever, but lacks a palpable abdominal mass.
- GORD (Gastroesophageal reflux disease): Characterised by vomiting, poor weight gain, and irritability but lacks projectile vomiting and palpable mass.
- Infantile colic: Presents with crying and fussiness, usually without vomiting.
What are some complications of Pyloric Stenosis?
- Metabolic Alkalosis: Persistent vomiting may result in loss of gastric acid, leading to a hypochloremic, hypokalemic metabolic alkalosis.
- Dehydration: Persistent vomiting can also lead to severe dehydration.
What is is the diagnostic investigation for Pyloric stenosis?
Abdominal Ultrasound: Visualise the hypertrophic pyloric sphincter. A hypertrophied muscle with a length of >16-18mm and a muscle wall thickness of >3-4mm are indicative of pyloric stenosis.
Other:
ABG: May show hypochloric metabolic acidosis
What is the Management for Pyloric Stenosis?
Immediate supportive care and definitive surgical intervention:
Supportive Care: The infant should be kept nil-by-mouth and administered IV fluids. Infants with severe dehydration may require acute fluid resuscitation.
Definitive Surgical Intervention: A pyloromyotomy (Ramstedt’s procedure) is performed to cut the hypertrophic pyloric sphincter, thereby widening the gastric outlet.
Define Inflammatory Bowel Syndrome (IBS)?
A common, chronic gastrointestinal disorder characterized by abdominal pain or discomfort associated with altered bowel habits, without any identifiable structural or biochemical abnormalities.
What is the Epidemiology of IBS?
IBS is a commonly occurring condition, affecting approximately 10-20% of adults worldwide.
What is the Aetiology of IBS?
It is considered a multifactorial condition, potentially involving genetic predisposition, altered gut microbiota, low-grade inflammation, and abnormalities in the gut-brain axis.
What are the clinical features of IBS?
- Abdominal discomfort or pain relieved by defaecation
- Altered bowel frequency or stool
- Altered stool passage
- Abdominal bloating
- Passage of mucus
- Symptoms worsened by eating
- Lethargy
- Nausea
- Backache
What criteria is used to diagnose IBS?
The Manning criteria for diagnosis of IBS includes:
Abdominal discomfort or pain relieved by defecation OR associated with altered bowel frequency or stool form
** At least two of the following:**
- Altered stool passage (e.g., straining or urgency)
- Abdominal bloating
- Symptoms worsened by eating
- Passage of mucus
Physical Examination and other investigations will reveal No abnormalities
What are some differential diagnoses for IBS?
- Inflammatory Bowel Disease (IBD): Symptoms may include bloody diarrhea, weight loss, and fever.
- Coeliac Disease: Symptoms may include diarrohea, weight loss, and anemia.
- Colorectal Cancer: Symptoms may include rectal bleeding, weight loss, and changes in bowel habits.
What are the investigations for IBS?
Investigations to rule out other causes of symptoms
- Faecal calprotectin (raised in IBD, not IBS)
- Full Blood Count, ESR and CRP: also raised in IBD, not IBS
- Coeliac serology
What is the management for IBS?
- Dietary and lifestyle modifications: Including regular exercise, stress management, and dietary changes (such as low-FODMAP diet).
- Psychotherapy: Cognitive-behavioral therapy, hypnotherapy, and mindfulness-based therapy can be beneficial.
- Pharmacotherapy: Medications such as antispasmodics, laxatives, or anti-diarrheal agents may be used depending on the predominant symptoms.
Define Acute Gastritis?
Inflammation of the stomach that presents with nausea and vomiting
Define Enteritis?
Inflammation of the Intestines that presents with Diarrhoea
Define Gastroenteritis?
Inflammation of the stomach and intestines that presents with nausea, vomiting and diarrhoea.
What is the epidemiology of Gastroenteritis?
Common world wide and can affect individuals of all age groups.
Very common in children
What is the most common cause of Gastroenteritis?
Viral causes:
- Rotavirus: Most common cause of infantile gastroenteritis
- Norovirus: Most common cause of gastroenteritis across all ages
- Adenovirus: Often causes respiratory infections but can cause gastroenteritis particularly in children
What are the Bacterial causes of Gastroenteritis?
- Campylobacter Most common bacterial cause of Gastroenteritis world wide
- Staphylococcus aureus: Found in cooked meats and cream products
- Bacillus cereus: Primarily associated with reheated rice
- Clostridium perfringens: Commonly found in reheated meat dishes or cooked meats
- E.coli, including E.coli 0157 (which can cause haemolytic uraemic syndrome)
- Salmonella
- Shigella
What are some parasitic causes of Gastroenteritis?
Cryptosporidium
Entamoeba histolytica
Giardia intestinalis
Schistosoma
What are the clinical features of Gastroenteritis?
Nausea and Vomiting and Diarrhoea
Systemic Symptoms:
- Malaise
- Fever
What are some differential diagnoses for gastroenteritis?
- Food poisoning: Rapid onset of symptoms, often in a group who have shared a meal
- Irritable Bowel Syndrome (IBS): Chronic condition with alternating periods of diarrhoea and constipation, often associated with stress or certain foods
- Inflammatory Bowel Disease (IBD): Chronic conditions such as Crohn’s disease or ulcerative colitis. Symptoms include diarrhoea, abdominal pain, weight loss, and blood in the stool
- Peptic Ulcer Disease: Symptoms include burning upper abdominal pain, nausea, and vomiting
What are some investigations for Gastroenteritis?
Clinical Diagnosis
- Stool cultures and Microscopy to identify causative pathogen
What is the Management for Gastroenteritis?
- Good Hygeine
- Isolation and barrier nursing
- Children to stay off school until 48 hours after symptoms have completely resolved
- Fluid replacement/challenge
- Antibiotics: Only in certain patients or if causative organism identified
What are the indications for antibiotic use in gastroenteritis?
- Systemically unwell patients
- Immunosuppressed individuals
- The elderly
What antibiotics are used to treat salmonella and shigella?
Ciprofloxacin
What antibiotics are used to treat campylobacter?
Macrolides: Erythromycin
Clarithromycin may lead to C.diff infection
What antibiotics are used to treat Cholera?
Tetracycline
What are the features of Norovirus gastroenteritis?
- Abrupt onset
- Occurs 24-48 hours post inoculation
- Condition is typically self limiting
- May lead to pre-renal AKI in frail patients
What is the main concern in gastroenteritis?
Dehydration
What are some post gastroenteritis complications?
- Lactose intolerance
- Irritable bowel syndrome
- Reactive arthritis
- Guillain–Barré syndrome
Define Constipation in Children
A clinical condition wherein the child defaecates fewer than three times per week, or experiences significant difficulty in passing stool.
Chronic constipation in this population is often characterised by hard, pellet-like stool that is difficult to pass.
What is the Epidemiology of Constipation in Children?
Constipation in children is a common occurrence. The frequency varies with the child’s diet and lifestyle.
What are some potential primary causes of constipation in children?
Idiopathic or Functional Constipation: No significant underlying cause other than lifestyle factors:
- Low Fibre Diet
- Avoidance of using the toilet
- Poor fluid intake/dehydration
- Sedentary lifestyle
- Habitually not opening bowels
- Psychosocial problems: Difficult home or school environment.
What are some secondary causes of constipation in children?
- Hirschsprung’s disease
- Cystic fibrosis (particularly meconium ileus)
- Hypothyroidism
- Spinal cord lesions
- Sexual abuse
- Intestinal obstruction
- Anal stenosis
- Cows milk intolerance
What are the clinical features that suggest constipation in children?
- Less than 3 stools a week
- Hard stools that are difficult to pass: Rabbit dropping stools
- Faecal impaction causing overflow soiling, with incontinence of particularly loose smelly stools
- Straining and painful passages of stools
- Abdominal pain
- Holding an abnormal posture, referred to as retentive posturing
- Rectal bleeding associated with hard stools
- Hard stools may be palpable in abdomen
- Loss of the sensation of the need to open the bowels
Define Encopresis?
Faecal Incontinence
- Not considered pathological until >4 years of age.
- Sign of chronic constipation where the rectum becomes stretched and looses sensation.
- Causes overflow diarrhoea as hard stools cannot pass but loose stools bypass the blockage and leak out causing soiling.
What are some rarer causes of Encopresis?
- Spina bifida
- Hirschprung’s disease
- Cerebral palsy
- Learning disability
- Psychosocial stress
- Abuse
What are some differential diagnoses for constipation in children?
- Hirschsprung’s disease: Presents with a delay in passing meconium (>48 hours), a distended abdomen, forceful evacuation of meconium after digital rectal examination, and a history of chronic constipation with poor response to Movicol disimpaction regimens and poor weight gain.
- Irritable Bowel Syndrome (IBS): May cause chronic constipation and is associated with abdominal pain, bloating, and altered bowel habit. Pain is typically relieved by defecation.
- Hypothyroidism: Can lead to constipation, along with other symptoms such as weight gain, fatigue, cold intolerance, and slow growth in children.
- Celiac Disease: While more commonly associated with diarrhoea, it can sometimes cause constipation. Other symptoms include failure to thrive, abdominal pain, and bloating.
- Lead poisoning: Constipation is one of the symptoms along with learning difficulties, irritability, loss of developmental skills in children, and possibly anaemia.
- Anal fissure: Pain during and after bowel movements can lead to constipation due to the child’s fear of experiencing pain again.
- Functional constipation: Characterized by normal anorectal and colonic physiology but passage of hard stools, infrequent stools, or painful defecation.
- Neurological disorders: like Spina Bifida and Cerebral Palsy. These conditions may impact the nerves that control bowel function, leading to constipation.
What is the pathophysiology of Desensitisation of the Rectum?
- Often patients develop a habit of not opening their bowels when they need to and ignoring the sensation of a full rectum.
- Over time they loose the sensation of needing to open their bowels, and they open their bowels even less frequently.
- They retain faeces in their rectum which leads to faecal Impaction
- Over time the rectum stretches as it fills with more and more faeces. This leads to further desensitisation of the rectum
- The longer this goes on the harder it is to reverse and treat
What are some Red Flags in the history or examination that should alert you to serious underlying conditions causing constipation in children?
- Not passing meconium within 48 hours of birth: cystic fibrosis or Hirschsprung’s disease
- Neurological signs or symptoms: particularly in the lower limbs (cerebral palsy or spinal cord lesion)
- Vomiting: intestinal obstruction or Hirschsprung’s disease
- Ribbon stool: anal stenosis
- Abnormal anus: anal stenosis, inflammatory bowel disease or sexual abuse
- Abnormal lower back or buttocks: spina bifida, spinal cord lesion or sacral agenesis
- Failure to thrive: coeliac disease, hypothyroidism or safeguarding
- Acute severe abdominal pain and bloating: obstruction or intussusception
What are the investigations for constipation in children?
Clinical diagnosis through history and examination
- May palpate impacted faeces on abdominal exam
What is the management for Constipation in Children?
- Correct any reversible contributing factors, recommend a high fibre diet and good hydration
- Start laxatives: Movicol is first line
- Faecal impaction may require a disimpaction regimen with high doses of laxatives at first
- Encourage and praise visiting the toilet. This could involve scheduling visits, a bowel diary and star charts.
- Laxatives should be continued long term and slowly weaned off as the child develops a normal, regular bowel habit.
Define Appendicitis
inflammation of the appendix
What is the epidemiology of Appendicitis?
Appendicitis is a common condition, particularly in populations with a Western diet.
** Peak incidence is 10-20 years**
It is estimated to affect approximately one-sixth of individuals in the United Kingdom during their lifetime.
What is the aetiology of appendicitis?
An obstruction within the appendix from various factors:
Fibrous tissue, foreign body, faecolith
What may be the progression of appendicitis?
- The inflammation can quickly proceed to gangrene and rupture.
- The appendix can rupture and release faecal content and infective material into the abdomen.
- This leads to peritonitis, which is inflammation of the peritoneal contents
- May lead to Sepsis
What are the clinical features of Appendicitis?
Symptoms:
- Pain: Acute appendicitis manifests as progressively worsening periumbilical pain, which typically migrates to the right iliac fossa.
- Gastrointestinal symptoms: These include nausea, vomiting, anorexia, and changes in bowel habits, such as constipation or diarrhoea.
- Systemic features: Patients may exhibit signs of infection, such as fever and tachycardia.
Signs:
- McBurney’s Point Tenderness: located one-third of the way from the anterior superior iliac spine to the umbilicus, may be particularly tender.
- Rovsing’s sign: eliciting right iliac fossa pain with palpation of the left iliac fossa, may also be present.
- Guarding on abdominal palpation
- Psoas sign: pain with passive extension of the right thigh
- Obturator sign:pain with passive internal rotation of the right hip
What are some signs suggestive of a ruptured appendix?
- Rebound tenderness is increased pain when quickly releasing pressure on the right iliac fossa
- Percussion tenderness is pain and tenderness when percussing the abdomen
What are some differential diagnoses for appendicitis?
- Renal calculi: Signs and symptoms include severe pain in the back or side below the ribs, pain that radiates to the lower abdomen and groin, haematuria, nausea, vomiting, and frequent urination.
- Biliary disease: Symptoms may encompass severe abdominal pain, jaundice, nausea, vomiting, and fever.
- Bowel obstruction: Persistent vomiting, severe abdominal pain, bloating, inability to pass gas or stool, and constipation are key indicators.
- Gastroenteritis: This condition can present with nausea, vomiting, diarrhoea, abdominal cramping, and sometimes fever.
- Ectopic pregnancy: Symptoms include lower abdominal pain, often unilateral, vaginal bleeding, and symptoms of pregnancy.
- Pelvic Inflammatory Disease: Lower abdominal pain, increased vaginal discharge, irregular menstrual bleeding, pain during intercourse, fever, and pain during pelvic examination are commonly seen.
- Meckel’s diverticulum: Pain may mimic appendicitis. It can also cause gastrointestinal bleeding or intestinal obstruction.
- Urinary tract infection: Common symptoms are dysuria, frequency, urgency, suprapubic pain, and haematuria.
- Mesenteric adenitis: This condition can mimic appendicitis and typically presents with right-sided abdominal pain, fever, and sometimes diarrhoea.
- Diverticulitis: Symptoms can be similar to appendicitis but the pain is usually on the left side. It can also present with change in bowel habits, fever, nausea, and vomiting.
- Ovarian torsion: This presents with sudden onset lower abdominal pain, often associated with nausea and vomiting. There may be a history of previous similar episodes. Pain can be intermittent if the ovary detorts spontaneously.
- Ovarian cyst : An ovarian cyst may present with lower abdominal pain, which can be sharp or dull. If the cyst ruptures or causes ovarian torsion, the pain can be severe. There may be associated bloating, feeling full quickly when eating, or difficulty eating.
- Cholecystitis: This condition typically presents with right upper quadrant abdominal pain, which may radiate to the right shoulder. Pain is often associated with meals (especially fatty foods), and there may be associated nausea, vomiting, and fever.
- Perforated peptic ulcer: This condition typically presents with sudden onset severe abdominal pain, which is generalized rather than localized. The abdomen is usually rigid (‘board-like’) on examination.
What are the investigations for Appendicitis?
Bedside:
- VBG to check lactate levels
- Pregnancy test (urine hCG) should be done in all females of reproductive age presenting with an acute abdomen
- A urine dip may show the presence of leukocytes, indicative of appendicitis
Laboratory:
- FBC for white cell count to identify signs of infection
- CRP to detect inflammation
- U&Es to assess renal function if dehydration is suspected
- LFTs and amylase to rule out biliary differentials
- Clotting, G&S for theatre
- Blood cultures if sepsis is suspected
Imaging:
- Abdominal Ultrasound: In children or pregnant women
- Erect chest x-ray to rule out perforation
- CT of the abdomen and pelvis or ultrasound of the right iliac fossa (RIF) for further evaluation
It is important to note that imaging is generally only utilised when there is doubt about the diagnosis or to rule out differentials. As acute appendicitis is primarily a clinical diagnosis, if suspected, the patient should be sent to the operating theatre without unnecessary delay for imaging.
What is the Gold Standard investigation for diagnosis of appendicitis?
CT Abdomen and Pelvis
Use ultrasound for children and pregnant women due to radiation
What is the management for Appendicitis?
- Administer prophylactic antibiotics; initiate full septis 6 if appropriate
- Laparoscopic appendicectomy is 1st line management
- If there is evidence of perforation: open appendicectomy is preferred, with copius lavage in theatre*
As per NICE guidelines, if there is negative imaging, a non-operative management strategy with IV fluids and antibiotics can be a safe and effective approach in selected patients with uncomplicated acute appendicitis
What are some potential complications of Appendicitis?
- Local abscess formation
- Perforation
- Gangrene
- Postoperative wound infection
- Peritonitis
What are some potential complications of an Appendectomy?
- Bleeding, infection, pain and scars
- Damage to bowel, bladder or other organs
- Removal of a normal appendix
- Anaesthetic risks
- Venous thromboembolism (deep vein thrombosis or pulmonary embolism)
What are the 2 conditions that make up Inflammatory Bowel Disease?
Crohn’s Disease
Ulcerative Colitis
Define Crohn’s Disease?
chronic relapsing inflammatory bowel disease (IBD). It is characterised by a transmural granulomatous inflammation which can affect any part of the gastrointestinal tract
What part of the GI tract is most commonly affected by Crohn’s disease?
Ileum, Colon or both
What is the Epidemiology of Crohn’s disease?
- More common in northern climates and developed countries
- Bimodal Age of Onset: 15-40 years and 69-80 years
- Common in Caucasian and Ashkenazi Jews
What are some risk factors for Crohn’s Disease?
- Family History: NOD2 mutation
- Caucasian, Ashkenazi Jews
- Female
- NSAIDS
- Depression
- HLA-B27
- Smoking
- Chronic Stress
crows (crohn’s) Nests
What are the General features of Crohn’s disease that are distinct from Ulcerative Colitis?
No blood or mucus (these are less common in Crohns.)
Entire GI tract
Skip lesions” on endoscopy
Terminal ileum most affected and Transmural (full thickness) inflammation
Smoking is a risk factor (don’t set the nest on fire)
Crohn’s is also associated with weight loss, strictures and fistulas.
What are the clinical features of Crohn’s Disease?
Symptoms:
- Crampy Abdominal Pain often in RLQ (ileum area)
- Nausea and Vomiting
- Diarrhoea
- Weight Loss and Malabsorption
- Fever and fatigue
Signs:
- Cachectic and Pale: due to anaemia
- Aphthous mouth ulcers
- Perianal skin tags, fistulae, abscess
A PIE SAC
What are some Extraintestinal Manifestations of Crohn’s Disease?
Arthritis (often asymmetrical and non-deforming)
Pyoderma Gangrenosum
Iris: Anterior uveitis, Episcleritis
Erythema Nodosum
Sacroileitis (Anklylosing Spondylosis) and Sclerosing Cholangitis
Apthous Mouth Ulcers
Clubbing
What are the investigations for Inflammatory Bowel Disease?
** Blood Tests:**
- FBC: raised WCC
- CRP/ESR Raised
- Thrombocytosis
- Anaemia
- Low albumin, iron, B12 and folate (secondary to Malabsorption)
Stool Sample:
- Faecal Calprotectin: 90% sensitive and specific for IBD
Imaging:
- Endoscopy (OGD and Colonoscopy): with Biopsy is the gold standard investigation for diagnosis of IBD
- Abdominal X-ray: rule out Toxic Megacolon in UC
What is the Gold standard investigation for diagnosing IBD?
Endoscopy AND biopsy: OGD or Colonoscopy
What would be seen on Colonoscopy and Biopsy in Crohn’s Disease?
- Intermittent inflammation (‘skip lesions’)
- Cobblestone mucosa (due to ulceration and mural oedema)
- Rose-thorn ulcers (due to transmural inflammation), ± fistulae or abscesses.
- Non-caseating granulomas
What would be seen on Colonoscopy and Biopsy in Ulcerative Colitis?
- Colonoscopy will reveal continuous inflammation with an erythematous mucosa, loss of haustral markings, and pseudopolyps.
- Biopsy will reveal loss of goblet cells, crypt abscess, and inflammatory cells (predominantly lymphocytes)
What is the management of Crohn’s disease to induce remission?
1st Line: Steroid Monotherapy (Oral prednisolone, IV hydrocortisone)
2nd Line: Addition of Immunosuppressant if there are 2 or more exacerbations in a 12 month period.
- Azathiprine
- Mercaptopurine
- Methotrexate (may be used if patients do not tolerate the above or are TMPT deficient)
3rd Line: Biological agents ae recommended in patients with severe disease who fail to respond to the above
- Infliximab
- Adalimumab
What is the management of Crohn’s disease to maintain remission?
1st Line: Immunosuppressants (Azathioprine or Mercaptopurine)
Alternatives:
- Methotrexate
- Infliximab
- Adalimumab
What are the surgical options for Crohn’s Disease?
Surgery is rarely curative in Crohn’s disease and so should be maximally conservative
Surgical options only really used when the disease only affects the distal ileum or to treat complications (strictures and fistulas)
What are some complications of Crohn’s Disease?
- Peri-anal Abscess
- Anal Fissure
- Anal Fistula
- Strictures and obstruction
- Perforation and Sepsis
- Anaemia and Malabsorption
- Osteoporosis
Define Ulcerative Colitis
Ulcerative colitis (UC) is a chronic relapsing-remitting inflammatory disease that primarily affects the large bowel.
What is the Epidemiology of Ulcerative Colitis?
- Most commonly diagnosed IBD.
- Bimodal age of Onset: 15-25 years and 55-65 years
- Can develop at any age
What is the aetiology of Ulcerative colitis?
Unknown Cause: Multifactorial
- Genetic predisposition (HLA-B27)
- Environmental factors
- Dysregulation of immune system (autoimmune conditions))
- Non/Ex smoker
UC goes with Close Up
What are the General features of Ulcerative Colitis that are distinct from Crohn’s disease?
Continuous inflammation
Limited to colon and rectum
Only superficial mucosa affected
Smoking is protective
Excrete blood and mucus
Use aminosalicylates
Primary sclerosing cholangitis
What are the clinical features of Ulcerative Colitis?
The main symptoms of UC are gastrointestinal and systemic.
Gastrointestinal symptoms include:
- Diarrhoea often containing blood and/or mucus
- Tenesmus or urgency
- Pain in the left iliac fossa
Systemic symptoms include:
- Weight loss
- Fever
- Pallor and fatigue: due to anaemia
- Clubbing
What is a condition is highly associated with Ulcerative Colitis?
Primary Sclerosing Cholangitis
What is the criteria to assess severity of Ulcerative Colitis?
Truelove and Witt’s Severity Index:
What are some differential diagnoses for Ulcerative Colitis?
- Crohn’s disease: Abdominal pain, weight loss, diarrhoea, oral ulcers, anal fissures, and perianal fistulas.
- Infectious colitis: Acute onset of diarrhoea, fever, and abdominal pain. May be associated with recent antibiotic use, travel, or consumption of contaminated food or water.
- Ischemic colitis: Sudden onset of abdominal pain, blood in stools, and a history of vascular disease or risk factors.
What is the Management for to induce remission Ulcerative Colitis?
Mild-Moderate Disease: Proctitis/Protosigmoiditis.
- 1st Line: Topical Aminosalicylate (Mesalazine)
- If after 4 weeks, symptoms worsen then consider adding Oral ASA
If remission is not achieved within 4 weeks move to 2nd Line:
- 2nd Line: Corticosteroids (oral Predisolone)
- If after 2-4 weeks, symptoms worsen consider adding Oral Tacrolimus
In left sided or extensive disease then 1st line is High dose oral ASA
Acute Severe Disease:
- 1st Line: IV Corticosteroids (IV Hydrocortisone)
If no improvement within 72 hours or worsening symptoms move to second line:
* 2nd Line: Add IV Ciclosporin or Consider surgery
What is the management of Ulcerative Colitis in maintaining Remission?
1st Line: Aminosalicylate (Mesalazine oral or rectal)
Alternatives:
- Azathioprine
- Mercaptopurine
What are the surgical options for Ulcerative Colitis?
Ulcerative colitis usually only affects the colon and rectum.
-
Panproctocolectomy: removing the colon and rectum
- Patient is left with either a permanent ileostomy
- Colectomy: with temporary End ileostomy (ileo-anal anastomosis (J pouch))
What are the indications for Surgery in Ulcerative Colitis?
- Failure to induce remission via medical means
What are some complications of Ulcerative Colitis?
Short-term/acute complications
- Toxic megacolon: this describes a severe form of colitis, and is seen in around 15% of ulcerative colitis patients.
- Massive lower gastrointestinal haemorrhage: this occurs in up to 3% of patients.
Long-term complications:
-
Colorectal cancer: this occurs in 3-5% of patients. There is a higher risk with disease duration, severity and extent of colitis, and concomitant primary sclerosing cholangitis (PSC).
NICE guidance suggests offering colonoscopy surveillance to high risk patients. - Cholangiocarcinoma: ulcerative colitis approximately doubles the risk of cholangiocarcinoma.
- Colonic strictures: these can cause large bowel obstruction.
Variable-term complications
- Primary Sclerosing Cholangitis: this is characterised by inflammation and fibrosis of the extra- and intra-hepatic biliary tree and affects 3-7% of patients with ulcerative colitis. LFTs should be monitored yearly to check for the presence of PSC.
- Inflammatory pseudopolyps: these are areas of normal mucosa between areas of ulceration and regeneration.
Define Coeliac Disease?
A T cell-mediated inflammatory autoimmune disease that impacts the small bowel.
It occurs when sensitivity to prolamin (Gluten), a group of plant storage proteins, results in villous atrophy in the lining of the small intestine and malabsorption.
What is the Epidemiology of Coeliac Disease?
- Affects approximately 1% of the Global Population
- Affects Females more commonly (Female:Male 2:1)
- Bimodal Age of Onset: Infancy or 50-60 years old
What is the Aetiology of Coeliac Disease?
- Family History of the HLA-DQ2 allele
- Co-existing autoimmune conditions: such as T1DM.
What are the auto-antibodies in Coeliac Disease?
- Anti-tissue Transglutaminase (anti-TTG)
- Anti-Endomysial (anti-EMA)
What is the most commonly affected part of the GI tract in Coeliac Disease?
Small Bowel particularly the Jejunum
What are the clinical features of Coeliac Disease?
Often Asymptomatic
Gastrointestinal Symptoms:
- Abdominal pain
- Distension
- Nausea and vomiting
- Diarrhoea
- Steatorrhoea (bolded to signify severe disease)
Systemic Symptoms:
- Fatigue
- Signs of vitamin deficiency: Easy bruising (Vit K), Neurological signs (Vit B12)
- Weight loss or failure to thrive in children (bolded to signify severe disease)
- Dermatitis herpetiformis
- Anaemia: secondary to Iron, B12 or Folate Deficiency
What are some differential diagnoses for Coeliac Disease?
- Irritable bowel syndrome: Characterised by recurrent abdominal pain, bloating, and altered bowel habits.
- Inflammatory bowel disease: Characterised by abdominal pain, diarrhoea, rectal bleeding, weight loss, and fever.
- Lactose intolerance: Symptoms include bloating, diarrhoea, and abdominal cramps after consumption of lactose-containing foods.
What are the investigations for Coeliac Disease?
Stool cultures: Rule out infection
Basic Blood Tests: FBC, U&E, LFT, Vitamin D, B12, Folate and Iron
Put patient on a Gluten challenge diet for 6 weeks:
-
Serological Blood Tests:
- Total IgA antibody levels: if Total IgA is low then coeliac Ab test will be negative even if it is positive.
- Anti-TTG IgA antibody
- Anti-EMA antibody: if IgA TTG is weakly positive
- Anti-TTG IgG antibody: if the patient is IgA deficient
- Endoscopy (OGD) + Biopsy: of duodenum/Jejunum
What is the Gold Standard investigation for Coeliac Disease and what are the results?
Oesophagealgastroduodenoscopy + Biopsy
- Raised intraepithelial lymphocytes
- Crypt Hypertrophy
- Villous Atrophy
How is the Biopsy assessed for Coeliacs disease?
Marsh Classification:
0: normal
1: raised intra epithelial lymphocytes (IEL)
2: raised ILE + crypt hyperplasia
3a: partial villous atrophy (PVA)
3b: subtotal villous atrophy (SVA)
3c: total villous atrophy (TVA)
What are some conditions associated with Coeliacs Disease?
- Type 1 Diabetes Mellitus
- Thyroid Disease
- Autoimmune Hepatitis
- PBC & PSC
- Down’s Syndrome
What is the management for Coeliac Disease?
Lifelong Gluten Free Diet
What are some complications of Coeliac Disease if left untreated?
- Anaemia (due to deficiencies in iron, B12, or folate)
- Vitamin deficiency
- Hyposplenism (and increased susceptibility to encapsulated organisms)
- Osteoporosis (A DEXA scan may be necessary)
- Enteropathy-associated T cell lymphoma (EATL), a rare type of non-Hodgkin lymphoma, with risk proportional to adherence to a gluten-free diet.
Define Failure to Thrive?
Poor physical growth and development in a child.
What is the NICE guidelines on faltering growth in children?
A fall in weight across:
- One or more centile spaces if their birthweight was below the 9th centile
- Two or more centile spaces if their birthweight was between the 9th and 91st centile
- Three or more centile spaces if their birthweight was above the 91st centile
What are the categories of causes for Failure to thrive?
Anything that leads to inadequate energy and nutrition:
- Inadequate Nutritional Intake
- Difficulty Feeding
- Malabsorption
- Increased Energy Requirements
- Inability to Process Nutrition
What are some causes of Inadequate nutritional intake that may lead to failure to thrive in children??
- Maternal malabsorption if breastfeeding
- Iron deficiency anaemia
- Family or parental problems
- Neglect
- Availability of food (i.e. poverty)
What are some causes of Difficulty feeding that may lead to failure to thrive in children??
- Poor suck, for example due to cerebral palsy
- Cleft lip or palate
- Genetic conditions with an abnormal facial structure
- Pyloric stenosis
What are some causes of Malabsorption that may lead to failure to thrive in children??
- Cystic fibrosis
- Coeliac disease
- Cows milk intolerance
- Chronic diarrhoea
- Inflammatory bowel disease
What does this show?
Atopic dermatitis seen on the ankle joint, upper leg and buttocks of an infant.
What condition does this show?
Stevens-Johnson Syndrome
What are the clinical features of Slapped Cheek Syndrome rash?
Slapped cheek syndrome: Rash on both cheeks, fever, upper respiratory tract infection symptoms.
What are the clinical features of Hand foot and Mouth Disease rash?
Blisters on hands and feet, grey ulcerations in the buccal cavity, fever, lethargy.
What are the clinical features of Scarlet fever Rash?
Coarse red rash on the cheeks, sore throat, headache, fever, ‘sandpaper’ texture rash, bright red tongue.
What are the clinical features of the Measles rash?
Erythematous, blanching maculopapular rash, fever, cough, runny nose, conjunctivitis, Koplik spots.
What are the clinical features of the Urticaria rash?
Raised, itchy red rashes, usually not accompanied by fever.
What are the clinical features of the Chicken Pox rash?
Maculopapular vesicular rash that crusts over and forms blisters.
What are the clinical features of the Roseola rash?
Lace-like red rash across the whole body, high fever.
What are the clinical features of the Rubella rash?
Rash that starts on the head and spreads to the trunk, postauricular lymphadenopathy.
CREAM
What are the clinical features of Kawasaki disease?
Persistent High Fever above 39 degrees for more than 5 days
- Conjunctivitis: Bilateral and non-exudative
- Rash: Widespread Erythematous maculopapular rash and desquamation (skin peeling) on palms and soles
- Erythema/oedema of hands and feed
- Adenopathy: cervical, commonly unilateral and non-tender
- Mucosal involvement: Strawberry tongue and oral fissues.
What are the clinical features of Measles?
- High fever above 40 degrees Celsius
- Coryzal symptoms
- Conjunctivitis
- A rash appearing 2-5 days after onset of symptoms
- Koplik spots: small grey discolourations of the mucosal membranes in the mouth, appearing 1-3 days after symptoms begin during the prodrome phase of infection. These are pathognomonic for measles.
What are the clinical features of Chickenpox?
Distinctive Rash
- Starts as raised red, itchy spots, primarily on the face or chest, before spreading to the rest of the body.
- Progresses into small, fluid-filled blisters over a span of a few days.
- Eventually crusts over and heals, typically leaving no scars unless the blisters have been scratched and infected.
- Peak infectivity is 1-2 days before the rash appears, until 5 days after the rash first appeared.
Other accompanying symptoms:
- Mild fever
- Fatigue
- Loss of appetite
- General discomfort.
What are the clinical features of Rubella?
Incubation period is 15-20 days
- Fever (low grade)
- Coryza
- Arthralgia
- A rash that typically begins on the face and moves down to the trunk, sparing the limbs
- Lymphadenopathy, classically post-auricular
What would the results of CSF analysis be in Bacterial, Viral and Fungal Meningitis?
Fungal:
- Lymphocytosis
- Increased Protein Concentration
- Decreased Glucose Concentration
What are the clinical features of Impetigo>
- Erythematous macule that vesiculates or pustulates
- Superficial erosion with a characteristic golden crust
- Impetigo may be bullous (causing large blisters) or non-bullous (causing sores)
- bullous impetigo tends to cause systemic symptoms
- These features are typically very infectious, prompting caution regarding close contact and shared items.
