Paediatrics (Joe) Flashcards

1
Q

Define Pneumonia?

A

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.

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2
Q

What are the main different causes of Pneumonia?

A

Bacteria
Atypical Bacteria
Viral
Fungal

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3
Q

What are the main bacterial causes of Pneumonia?

A

Streptococcus pneumonia
Staphylococcus aureus
Haemophilus influenzae
Moraxella catarrhalis

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4
Q

What are the main Atypical bacterial causes of Pneumonia?

A

Legions of Psittaci MCQ:

  • Legionella pneumophilia
  • Chlamydia psittaci
  • Mycoplasma pneumonia
  • Chlamydia pneumonia
  • Coxiella burnettii (Q fever)
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5
Q

What are the main viral causes of pneumonia?

A

Respiratory Syncytial virus (RSV)
Influenza
Parainfluenza

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6
Q

What are the main causes of Pneumonia in Neonates, Infants and School age children?

A

Neonates: Group B Strep (Streptococcus Pyogenes)

Infants: Strep pneumoniae

School age: Strep pneumoniae, staph aureus, mycoplasma

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7
Q

What is a common cause of pneumonia in closed populations such as schools?

A

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
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8
Q

What are the clinical symptoms of pneumonia?

A
  • Cough (typically wet and productive)
  • SOB
  • High fever (> 38.5ºC)
  • Increased work of breathing
  • Lethargy
  • Delirium (acute confusion associated with infection)
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9
Q

What are some clinical signs of pneumonia?

A
  • Tachypnoea (raised respiratory rate)
  • Tachycardia (raised heart rate)
  • Hypoxia (low oxygen)
  • Hypotension (shock)
  • Fever

*Confusion

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10
Q

What are the characteristic chest signs of pneumonia?

A

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.

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11
Q

What are the investigations for pneumonia?

A

1st Line:

  • Sputum culture and throat swabs
  • Bloods and blood cultures
  • Capillary blood gas/ABG for acidosis and lactate

Gold Standard: Chest X-Ray

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12
Q

What scoring system is used to assess severity of pneumonia and further management?

A

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.
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13
Q

What are the different types of pneumonia?

A

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

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14
Q

What is the management for Pneumonia in Children?

A
  • 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
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15
Q

What is the management for CAP?

A

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

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16
Q

What is the Management for HAP?

A

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.

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17
Q

What are some complications of Pneumonia?

A
  • 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
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18
Q

What vaccines are available for prevention of Pneumonia?

A

Pneumococcal Vaccine: Routinely offered as 3 injections at 2 months, 4 months and 12-13 months

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19
Q

Define Pertussis?

A

Whooping Cough is a severe upper respiratory tract infection characterised by intense bouts of spasmodic coughing that may lead to apnoea in infants.

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20
Q

What is the Aetiology of Whooping Cough?

A

Pertussis is primarily caused by the Gram-negative bacterium, Bordetella pertussis.

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21
Q

What is the Epidemiology of Whooping Cough?

A
  • Much less incidence now due to vaccination programme
  • Vaccinations: 2,3,4 months, booster at 3 years 4 months
  • Impacts infants more dramatically
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22
Q

What is the Clinical progression of Whooping Cough?

A

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
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23
Q

What are the Investigations for Whooping Cough?

A
  • Nasal-pharyngeal swab with pertussis
  • FBC
  • Antibody test
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24
Q

What is the management of Whooping Cough?

A

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
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25
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.
26
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
27
What is the Aetiology of Croup?
* **Parainfluenza Virus** * Influenza * Adenovirus * Respiratory Syncytial Virus (RSV)
28
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
29
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
30
What are some Respiratory Failure Red Flags?
* Drowsiness * Lethargy * Cyanosis * Tachycardia * Laboured breathing
31
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.
32
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.
33
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.)
34
What are some possible complications of Croup?
* Otitis Media * Dehydration due to reduced fluid Intake * Superinfection: Pneumonia
35
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.
36
What is the epidemiology of Asthma?
Affects nearly 10% of children in the UK
37
What is the Aetiology of Asthma?
Unclear but likely multifactorial Family History of Asthma History of Atopy (allergy/Eczema)
38
What are the risk factors for asthma?
* Genetic * Prematurity * Low birth weight * Parental smoking * Viral bronchiolitis in early life * Cold air * Allergen exposure
39
What are the clinical features of asthma?
Cough Breathlessness Bilateral Polyphonic Wheeze Chest tightness
40
What drugs can trigger asthma?
Aspirin Beta blockers
41
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.
42
What is Samter's Triad
Nasal Polyps Asthma Aspirin sensitivity
43
What is the Atopic Triad?
Atopic Rhinitis (Hayfever) Allergic Asthma (Asthma) Atopic Dermatitis (Eczema)
44
What are the classifications of asthma?
Intermittent Mild Persistent Moderate Persistent Severe Persistent
45
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
46
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
47
What are some typical triggers for Asthma?
Dust (house dust mites) Animals Cold air Exercise Smoke Food allergens (e.g. peanuts, shellfish or eggs)
48
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.
49
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.
50
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
51
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**
52
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
53
What is the medical management for Asthma in a patient **Under 5 years old**?
1. Start a short-acting beta-2 agonist inhaler (e.g. salbutamol) as required 2. Add an **8 week trial of low dose corticosteroid inhaler** If symptoms reoccur within 4 weeks of trial ending then restart ICS 3. Add an LRTA 4. Refer to a specialist.
54
What is the medical management for Asthma in a patient **5-16 years old**?
1. Start a short-acting beta-2 agonist inhaler (e.g. salbutamol) as required 2. Add a regular paediatric low dose corticosteroid inhaler **Assess Inhaler Technique** 3. Consider offering LRTA (monteleukast) and reviewing response in 4-8 weeks 4. 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. 4. If asthma is uncontrolled then consider Changing ICS and LABA to MART Regime 6. If asthma is uncontrolled on MART Regime then consider increasing ICS dose to paediatric moderate dose. 7. Increase the dose of the inhaled corticosteroid to a high dose. Referral to a specialist. They may require daily oral steroids.
55
What is the medical management for Asthma in a patient **over 17 (adult)**?
1. Start a short-acting beta 2 agonist inhaler (e.g. salbutamol) as required 2. Add a regular low dose corticosteroid inhaler **Assess Inhaler Technique** 3. Consider offering a leukotriene receptor antagonist (LTRA) in addition to the low dose ICS. Review the response to treatment in 4 to 8 weeks. 4. Add a long-acting beta-2 agonist inhaler (e.g. salmeterol). Continue salmeterol only if the patient has a good response. (Consider stopping LRTA) 5. 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. 5. Titrate the inhaled corticosteroid up to a moderate dose. 6. 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
56
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.
57
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
58
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
59
What are some side effects of Salbutamol?
Fine Tremor HYPOKALAEMIA Headache Palpitations Muscle cramps
60
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.
61
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.)
62
What are the different severities of an acute asthma exacerbation?
Moderate: PEF > 50% predicted Severe: PEF < 50% predicted Life threatening: PEF < 33% predicted
63
What are the features of a Moderate acute asthma exacerbation?
PEF > 50% predicted Normal speech
64
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
65
What are the features of a Life Threatening acute asthma exacerbation?
**33, 92, CCHEST:** * PEF **< 33%** predicted * **<92%** - Oxygen Stats **C**yanosis **C**onfusion/Consciousness/AMS **H**ypotension **E**xhaustion and poor respiratory effort **S**ilent Chest **T**achycardia
66
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)
67
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
68
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.** 1. Administer **inhaled salbutamol** via spacer: 10 puffs every 2 hours 2. Proceed to **nebulised salbutamol** if necessary 3. Add nebulised **ipratropium bromide** 4. If O2 saturations remain <92%, **add magnesium sulphate** 5. Add **intravenous salbutamol** if no response to inhaled therapy 6. 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
69
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.
70
What must be monitored when giving high doses of salbutamol?
Serum potassium as salbutamol drives potassium into cells
71
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
72
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
73
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.
74
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
75
What viruses are the common causes of a virally induced wheeze?
RSV Rhinovirus
76
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
77
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)
78
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.
79
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.
80
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
81
What is the Aetiology of Bronchiolitis?
Respiratory Syncytial Virus (RSV) is the most common cause (80% of cases)
82
What are some risk factors for Bronchiolitis?
* Breastfeeding for < 2 months * Smoke Exposure * Older siblings who attend nursery/schools * Chronic Lung disease of prematurity
83
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.
84
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
85
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
86
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.
87
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.
88
What are the investigations for Bronchiolitis?
**Clinical Diagnosis** * Nasopharyngeal aspirate for RSV culture * Chest X-rays may be considered in severe cases
89
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
90
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.
91
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
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What is the main complication of Bronchiolitis?
**Bronchiolitis Obliterans** Rare, chronic complication of bronchiolitis, colloquially known as **popcorn lung**
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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
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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.
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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
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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.
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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.
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Define Cystic Fibrosis?
A progressive, autosomal recessive disorder that causes persistent lung infections and limits the ability to breathe over time.
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What are the genetics of Cystic Fibrosis?
* Autosomal Recessive * Cystic Fibrosis Transmembrane Conductance Regulatory Gene (CFTR) * Chromosome 7 * **Delta-F508**
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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
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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**
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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.
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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.
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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
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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)
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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
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What are the causes of clubbing in children?
* Hereditary clubbing * Cyanotic heart disease * Infective endocarditis * Cystic fibrosis * Tuberculosis * Inflammatory bowel disease * Liver cirrhosis
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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
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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
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How does the Cystic fibrosis Sweat Test work?
**Gold standard investigation for CF** 1. A patch of skin is chosen for the test, typically on the arm or leg. 2. Pilocarpine is applied to the skin on this patch. 3. Electrodes are placed either side of the patch and a small current is passed between the electrodes. 4. This causes the skin to sweat. 5. The sweat is absorbed with lab issued gauze or filter paper and sent to the lab for testing for the chloride concentration. 6. The **diagnostic chloride concentration for cystic fibrosis is more than 60mmol/l.**
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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.
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What are the key colonisers of the lungs in CF?
Staph aureus Pseudomonas Aeruginosa Haemophilus influenzae Klebsiella pneumonia E.coli
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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**
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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
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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**
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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**
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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.
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What is the Aetiology of Acute Epiglottitis?
Primarily **Haemophilus influenzae Type B**
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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
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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.
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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.
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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)
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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
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What is Respiratory Distress Syndrome
Affects premature neonates, before the lungs start producing adequate surfactant, common in below 32 week babies.
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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.
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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%
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What are some short term complications of Respiratory Distress Syndrome?
* Pneumothorax * Infection * Apnoea * Intraventricular Haemorrhage * Pulmonary Haemorrhage * Necrotising Enterocolitis
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What are some Long term complications of Respiratory Distress Syndrome?
* Chronic lung disease of prematurity * Retinopathy of Prematurity * Neurological, hearing and visual impairment
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What is Bronchopulmonary Dysplasia?
Infants who still require oxygen at a postmenstrual age of 36 weeks are described as having BPD
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Define Otitis Media?
Otitis media is an infection-induced inflammation of the middle ear, frequently occurring after a viral upper respiratory tract infection.
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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.
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What is the Epidemiology of Otitis media?
Otitis media is a common condition, predominantly affecting young children.
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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.**
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What bacteria are common causes of Otitis Mdia?
**Streptococcus pneumoniae** **Other:** * Haemophilus influenzae * Moraxella catarrhalis * Staphylococcus aureus
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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.
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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
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What are the investigations for Otitis Media?
**Clinical Diagnosis** * Good history * Physical examination using an Otoscope to visualise the Tympanic membrane
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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.
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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
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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**
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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)
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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.
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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.
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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
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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.
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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.**
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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.
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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**
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What are some Perinatal causes of hearing loss?
* Prematurity * Hypoxia during or after birth
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What are some Post natal causes of Hearing Loss?
* Jaundice * Meningitis and Encephalitis * Otitis media and Glue Ear * Chemotherapy
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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.
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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
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What investigations are used to assess hearing loss?
History and Physical Examination Otoscope **Audiometry Testing**
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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).
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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
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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.
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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.
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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.
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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
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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.
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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**
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What are the important findings that suggest Periorbital cellulitis **and not Orbital**?
* No proptosis * Normal eye movements * No chemosis * Normal optic nerve function
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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
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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
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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**
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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.
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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.**
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What are Concomitant Squints?
Squints due to differences in the control of the extra ocular muscles
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What are Paralytic Squints?
Squints due to paralysis in one or more of the extra ocular muscles These are rare
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Define Strabismus
The eyes are misaligned
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Define Amblyopia
The affected eye becomes passive and has reduced function compared to the other dominant eye
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Define Esotropia
inward positioned squint (affected eye towards the nose)
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Define Exotropia
outward positioned squint (affected eye towards the ear)
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Define Hypertropia
upward moving affected eye
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Define Hypotropia
downward moving affected eye
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What are the causes of Squints?
**Usually idiopathic** **Other causes of squint include:** * Hydrocephalus * Cerebral palsy * Space occupying lesions, for example retinoblastoma * Trauma
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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**
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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.
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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).
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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**
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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.
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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.
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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.**
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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**
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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.
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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
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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
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What are the investigations for Febrile Convulsions?
**Clinical Diagnoses** Investigations to find underlying cause of Fever: **Bloods** **Lumbar Puncture** **Electroencephalogram (EEG)**
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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
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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
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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
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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.
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What are the first line treatments for West Syndrome?
Prednisolone Vigabatrin
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What is the prognosis of West Syndrome?
Poor Prognosis. 1/3 die by age 25 however 1/3 are seizure free
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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**
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What are some potential causes of Global Developmental Delay?
* Down’s syndrome * Fragile X syndrome * Foetal alcohol syndrome * Rett syndrome * Metabolic disorders
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What is Gross motor delay?
A developmental delay that is specific to the gross motor domain
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What are some potential causes of Gross Motor Delay?
* Cerebral palsy * Ataxia * Myopathy * Spina bifida * Visual impairment
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What is Fine Motor Delay?
A developmental delay that is Specific to the Fine Motor Domain
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What are some potential causes of Fine Motor Delay?
* Dyspraxia * Cerebral palsy * Muscular dystrophy * Visual impairment * Congenital ataxia (rare)
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What is Language Delay?
A developmental delay that is specific to the speech and language domain
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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
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What is Personal and Social Delay?
A developmental delay that is specific to the personal and social domain
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What are some potential causes of Personal and Social Delay?
* Emotional and social neglect * Parenting issues * Autism
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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.
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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.
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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**
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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
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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
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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
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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
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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
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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
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Define Pyloric Stenosis?
Hypertrophy of the Pyloric Sphincter causing narrowing of the gastric outlet leading to gastric outlet obstruction.
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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”.
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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.
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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.
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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
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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.
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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.
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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
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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.
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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.
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What is the Epidemiology of IBS?
IBS is a commonly occurring condition, affecting approximately 10-20% of adults worldwide.
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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.
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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
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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**
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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.
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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
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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.
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Define Acute Gastritis?
Inflammation of the stomach that presents with nausea and vomiting
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Define Enteritis?
Inflammation of the Intestines that presents with Diarrhoea
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Define Gastroenteritis?
Inflammation of the stomach and intestines that presents with nausea, vomiting and diarrhoea.
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What is the epidemiology of Gastroenteritis?
Common world wide and can affect individuals of all age groups. Very common in children
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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**
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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
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What are some parasitic causes of Gastroenteritis?
Cryptosporidium Entamoeba histolytica Giardia intestinalis Schistosoma
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What are the clinical features of Gastroenteritis?
**Nausea and Vomiting and Diarrhoea** Systemic Symptoms: * Malaise * Fever
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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
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What are some investigations for Gastroenteritis?
**Clinical Diagnosis** * Stool cultures and Microscopy to identify causative pathogen
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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
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What are the indications for antibiotic use in gastroenteritis?
* Systemically unwell patients * Immunosuppressed individuals * The elderly
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What antibiotics are used to treat salmonella and shigella?
Ciprofloxacin
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What antibiotics are used to treat campylobacter?
Macrolides: Erythromycin **Clarithromycin may lead to C.diff infection**
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What antibiotics are used to treat Cholera?
Tetracycline
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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
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What is the main concern in gastroenteritis?
Dehydration
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What are some post gastroenteritis complications?
* Lactose intolerance * Irritable bowel syndrome * Reactive arthritis * Guillain–Barré syndrome
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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.
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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.
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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.
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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
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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
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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.
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What are some rarer causes of Encopresis?
* Spina bifida * Hirschprung’s disease * Cerebral palsy * Learning disability * Psychosocial stress * Abuse
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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.
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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
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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
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What are the investigations for constipation in children?
**Clinical diagnosis through history and examination** * May palpate impacted faeces on abdominal exam
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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.
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Define Appendicitis
inflammation of the appendix
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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.
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What is the aetiology of appendicitis?
An obstruction within the appendix from various factors: Fibrous tissue, foreign body, faecolith
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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
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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
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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
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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.
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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.**
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What is the **Gold Standard** investigation for diagnosis of appendicitis?
**CT Abdomen and Pelvis** **Use ultrasound for children and pregnant women due to radiation**
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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
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What are some potential complications of Appendicitis?
* Local abscess formation * Perforation * Gangrene * Postoperative wound infection * Peritonitis
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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)
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What are the 2 conditions that make up Inflammatory Bowel Disease?
Crohn's Disease Ulcerative Colitis
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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**
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What part of the GI tract is most commonly affected by Crohn's disease?
Ileum, Colon or both
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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**
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What are some risk factors for Crohn's Disease?
* Family History: **NOD2 mutation** * Caucasian, Ashkenazi Jews * Female * NSAIDS * Depression * HLA-B27 * Smoking * Chronic Stress
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# crows (crohn's) **Nests** What are the General features of Crohn's disease that are distinct from Ulcerative Colitis?
**N**o blood or mucus (these are less common in Crohns.) **E**ntire GI tract **S**kip lesions” on endoscopy **T**erminal ileum most affected and Transmural (full thickness) inflammation **S**moking is a risk factor (don’t set the nest on fire) **Crohn’s is also associated with weight loss, strictures and fistulas.**
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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
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# A PIE SAC What are some Extraintestinal Manifestations of Crohn's Disease?
**A**rthritis (often asymmetrical and non-deforming) **P**yoderma Gangrenosum **I**ris: Anterior uveitis, Episcleritis **E**rythema Nodosum **S**acroileitis (Anklylosing Spondylosis) and Sclerosing Cholangitis **A**pthous Mouth Ulcers **C**lubbing
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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
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What is the Gold standard investigation for diagnosing IBD?
Endoscopy **AND** biopsy: OGD or Colonoscopy
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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
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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)
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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
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What is the management of Crohn's disease to maintain remission?
**1st Line:** Immunosuppressants **(Azathioprine or Mercaptopurine)** **Alternatives:** * Methotrexate * Infliximab * Adalimumab
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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**)
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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
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Define Ulcerative Colitis
Ulcerative colitis (UC) is a chronic relapsing-remitting inflammatory disease that **primarily affects the large bowel.**
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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**
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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**
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# UC goes with **Close Up** What are the General features of Ulcerative Colitis that are distinct from Crohn's disease?
**C**ontinuous inflammation **L**imited to colon and rectum **O**nly superficial mucosa affected **S**moking is protective **E**xcrete blood and mucus **U**se aminosalicylates **P**rimary sclerosing cholangitis
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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
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What is a condition is highly associated with Ulcerative Colitis?
Primary Sclerosing Cholangitis
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What is the criteria to assess severity of Ulcerative Colitis?
Truelove and Witt's Severity Index:
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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.
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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
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What is the management of Ulcerative Colitis in maintaining Remission?
**1st Line:** **Aminosalicylate** (Mesalazine oral or rectal) **Alternatives:** * Azathioprine * Mercaptopurine
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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)**)
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What are the indications for Surgery in Ulcerative Colitis?
* Failure to induce remission via medical means
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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.
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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.
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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
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What is the Aetiology of Coeliac Disease?
* Family History of the **HLA-DQ2** allele * Co-existing autoimmune conditions: such as T1DM.
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What are the auto-antibodies in Coeliac Disease?
* **Anti-tissue Transglutaminase (anti-TTG)** * Anti-Endomysial (anti-EMA)
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What is the most commonly affected part of the GI tract in Coeliac Disease?
Small Bowel **particularly the Jejunum**
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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
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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.
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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
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What is the Gold Standard investigation for Coeliac Disease and what are the results?
**Oesophagealgastroduodenoscopy + Biopsy** * Raised intraepithelial lymphocytes * Crypt Hypertrophy * Villous Atrophy
311
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)
312
What are some conditions associated with Coeliacs Disease?
* Type 1 Diabetes Mellitus * Thyroid Disease * Autoimmune Hepatitis * PBC & PSC * Down's Syndrome
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What is the management for Coeliac Disease?
**Lifelong Gluten Free Diet**
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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.
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Define Failure to Thrive?
Poor physical growth and development in a child.
316
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**
317
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
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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)
319
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
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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
321
What are some causes of increased energy requirements that may lead to failure to thrive in children?
* Hyperthyroidism * Chronic disease, for example congenital heart disease and cystic fibrosis * Malignancy * Chronic infections, for example HIV or immunodeficiency
322
What are some causes of an inability to process nutrients properly that may lead to failure to thrive in children?
* Inborn errors of metabolism * Type 1 diabetes
323
How is Failure to thrive initially assessed?
Assessment done to establish the cause: * History and Examination * Pregnancy, birth, developmental and social history * Feeding or eating history * Observe feeding * Mums physical and mental health * Parent-child interactions * Height, weight and BMI (if older than 2 years) and plotting these on a growth chart * Calculate the **mid-parental height** centile
324
What is involved in a Feeding and Eating History?
**Feeding History:** * Breast or bottle feeding * Feeding times * Volume and Frequency * Difficulties feeding **Eating History:** * Food choices * Food Aversion * Meal time routines * Appetite
325
What are the outcomes from the initial assessment for failure to thrive that would suggest inadequate nutrition or a growth disorder?
* Height more than 2 centile spaces below the mid-parental height centile * BMI below the 2nd centile
326
What investigations should be carried out following the initial failure to thrive assessment?
* Urine Dipstick for UTI * Coeliac Screen (Anti-TTG/EMA antibodies) **Further investigations are usually not necessary if there are no other clinical concerns**
327
What is the management for Failure to Thrive?
**MDT Input** * Regular reviews to monitor weight gain (too frequent may increase parental anxiety) * Aim to provide options to resolve the underlying problem causing the failure to thrive.
328
What are some potential suggestions to help manage failure to thrive when it is caused by inadequate nutrition?
* Encouraging regular structured mealtimes and snacks * Reduce milk consumption to improve appetite for other foods * Review by a dietician * Additional energy dense foods to boost calories * Nutritional supplements drinks
329
Define Kwashiorkor?
A type of Severe acute **Oedematous malnutrition** specifically due to **protein deficiency** that typically occurs in children around the time of weaning and up to 5 yeas of age in developing countries.
330
Define Marasmus?
A type of Severe acute deficiency of **All nutrients** that **leads to muscle wasting and is without oedema**
331
What is Marasmic Kwashiorkor?
The presence of severe wasting in addition to oedema due to a combination of all nutrient deficiency due to Marasmus and added protein deficiency due to Kwashiorkor
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What is the Epidemiology of Kwashiorkor?
* **almost solely** found in developing countries * Sub-Saharan Africa, South East Asia, Central America * Occurs in children living in areas with endemic food insecurity or famine * Children often **<5 years old**
333
What is the Aetiology of Kwashiorkor?
Unknown but thought to be due to Protein deficiency * Associated with cultures who's diets are based on Corn or Cassava.
334
What are the clinical features of Kwashiorkor?
* **Oedematous Malnutrition** (Often Central oedema) * Anaemia * Skin Lesions: Hyperkeratosis and skin depigmentation * Hepatosteatosis * **Wasting (often muscle) is seen in Marasmus**
335
What are the investigations for Kwashiorkor?
**Clinical Diagnosis** Specific investigations are generally unnecessary for the vast majority of children and are only required to look for underlying co-existing conditions, to exclude other differentials of oedema, and to assess complications. **Other Investigations:** * Bloods: Anaemia and protein profile * TB skin testing * HIV Serology
336
What are some differential diagnoses for Kwashiorkor?
* Marasmus * Chronic Undernutrition * Congestive Heart Failure * Glomerulonephritis
337
What are the different categories for Kwashiorkor?
**Uncomplicated:** Can be treated at home with RUTF **Complicated:** A life threatening condition and requires stabilisation in an inpatient facility to be treated
338
What is the management for Kwashiorkor?
**Uncomplicated:** * Community based therapy with **Ready to use therapeutic food (RUTF)** * Oral Antibiotics: as sepsis is likely a co-morbidity **Complicated:** * Facility based care with **Regular milk based liquid foods** * Empirical antibiotic therapy as sepsis occurs in 15-60% of children with severe malnutrition. * Vitamin supplementation should be considered
339
What is the Criteria for Marasmus?
Malnutrition without Oedema but: * A weight for a **height/length Z-score of <-3** or * Mid-upper arm circumference (MUAC) < 11.5cm
340
What are some acute complications of Kwashiorkor?
* Sepsis * Micronutrient deficiencies * Shock * Dehydration * Hypoglycaemia * Electrolyte imbalance * Hypothermia * Anaemia
341
What are some long term complications of Kwashiorkor?
* Growth Stunting * Loss of Vision due to loss of Vitamin A
342
Define Hirschsprung's disease?
A congenital condition where nerve cells of the myenteric plexus are absent in the distal bowel and rectum leading to an inability for peristalsis to occur and for food to pass along its length of the bowel.
343
What is the Epidemiology of Hirschsprung's Disease?
* 90% present in the neonatal period * Average age of Presentation: **2 Days** * Affects males more commonly than females * Associated with Down's Syndrome
344
What is the pathophysiology of Hirschsprung's disease?
* Congenital absence of parasympathetic ganglion cells in the distal bowel and rectum due to impaired migration of the cells during foetal development. * The **aganglionic** segment of colon cannot relax causing it to become constricted and hence leads to a loss of movement of faeces in the bowel. * This will lead to proximal obstruction and the bowel becomes distended and full. * Bacteria can build up leading to Enterocolitis and sepsis **When the entire colon is affected this is called total colonic aganglionosis**
345
What is the most common type of Hirschsprung's disease?
**Short Segment** where the disease is confined to the rectosigmoid part of the colon
346
What syndromes are associated with Hirschsprung's disease?
* Down's Syndrome * Neurofibromatosis * Waardenburg Syndrome * Multiple Endocrine Neoplasia Type II
347
What are the clinical features of Hirschsprung's Disease?
**Presentation and age of diagnosis varies depending on individual and extent of bowel affected. May present acutely after birth or more gradually developing symptoms.** * Delay in passing Meconium (> 24 hours) * Chronic Constipation since birth * Abdominal pain and distension * Bilious Vomiting * Poor weight gain and failure to thrive
348
What is a complication of Hirschsprung's disease?
**Hirschsprung-Associated Enterocolitis (HAEC)** * Inflammation and obstruction of the intestine occuring in 20% of neonates with Hirschsprung's disease
349
How does HAEC present?
* 2-4 weeks following birth * Fever, Abdominal distension, Diarrhoea (often bloody) and features of sepsis. * Can lead to **toxic Megacolon and perforation**
350
How is HAEC managed?
* Urgent Antibiotics, * Fluid resuscitation * Decompression of the obstructed bowel.
351
What are the investigations for Hirschsprung's Disease?
**Rectal Suction Biopsy:** Bowel histology demonstrates an absence of ganglionic cells. **Abdominal X-ray:** Can diagnose intestinal obstruction and features of HAEC
352
What are the indications for a Rectal Suction Biopsy?
To test for Hirschsprung's disease in anyone who has: * Delayed passage of Meconium * Constipation in first few weeks of life * Chronic Abdominal distension * Positive family history of Hirschsprung's * Faltering Growth
353
What is the management of Hirschsprung's Disease?
* **Definitive management is Surgery:** Surgical removal of the aganglionic section. **Swenson, Soave, Dunhamel pull through surgery** **Patients with HAEC:** * IV Antibiotics * Fluid resuscitation * Decompression of intestinal obstruction
354
Define Intussusception?
A condition where the bowel **Invaginates or telescopes** into itself. This thickens the overall size of the bowel and narrows the lumen at the folding areas leading to a palpable mass in the abdomen and obstruction to the passage of faeces through the bowel.
355
What is the most common scenario in Intussusception?
The ileum passing into the caecum through the ileocaecal valve
356
What is the Epidemiology of Intussusception?
* Typically occurs in Infants **3 months to 2 years old** * More common in boys * **Most common cause of obstruction in neonates**
357
What are some risk factors for Intussusception?
* Cystic Fibrosis * Meckel's Diverticulum * Hirschsprung's Disease * Rotavirus Vaccine > 23 weeks
358
What are some associated conditions with Intussusception?
* Concurrent **Viral Illness** * Henoch-Schonlein purpura * Cystic Fibrosis * Intestinal Polyps * Meckel's Diverticulum
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What are the clinical features of Intussusception?
* **Redcurrant Jelly Stool** * Right upper quadrant **Sausage shaped mass** on palpation * Severe colicky abdominal pain * Pale, Lethargic and unwell child * Vomiting * Intestinal obstruction features: Absolute constipation, abdominal distension etc.
360
What are some differential diagnoses for Intussusception?
* **Gastroenteritis:** Presents with diarrhoea, vomiting, and abdominal pain, but lacks the characteristic 'redcurrant jelly' stool and palpable abdominal mass. * **Appendicitis:** Characterised by lower right abdominal pain, vomiting, and fever, but does not involve the passage of 'redcurrant jelly' stools. * **Volvulus:** Presents with severe abdominal pain, vomiting, and possibly a distended abdomen, but lacks the 'redcurrant jelly' stools and specific mass. * **Meckel's diverticulum:** Can present with painless rectal bleeding and occasionally abdominal pain, but lacks the typical colicky pain and lethargy seen in intussusception.
361
What are the investigations for Intussusception?
**Abdominal Ultrasound** is the initial investigation of choice and see a **Target Shaped Mass** ** Contrast Enema may also be used**
362
What is the management of Intussusception?
**Medical Emergency:** * IV Fluids * **Therapeutic Enemas:** Contrast, water or air are pumped into the colon to force the folded bowel out and into the normal position. * **Surgical Reduction** if enemas do not work, The child is haemodynamically unstable or the child has periotonitis * **Surgical Resection** If the bowel becomes gangrenous or perforated
363
What are some potential complications of Intussusception?
* Intestinal Obstruction * Gangrenous bowel due to a disruption of the blood supply * Perforation * Death
364
Define Meckel's Diverticulum?
* A congenital Diverticulum of the small intestine. * It is a remnant of the Omphalomesenteric (Vitellointestinal duct) * It contains Ectopic ileal, gastric or pancreatic mucosa
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What is the Rule of 2s for Meckel's Diverticulum?
* Occurs in 2% of the population * Is 2 feet from the proximal from the Ileocaecal valve * Is 2 Inches long * 2:1 Male to female ratio
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What is the Epidemiology of Meckel's Diverticulum?
* Most prevalent congenital abnormality of the GI tract * Affects 2% of the population * Affects males 2:1 ratio * Peak incidence is 1-5 years but often **< 2 years**
367
What is the Aetiology of Meckel's Diverticulum?
The incomplete obliteration of the vitello-intestinal duct, an embryonic structure that typically regresses around the sixth week of gestation.
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What artery supplies Meckel's Diverticulum?
Omphalomesenteric Artery
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What are the clinical features of Meckel's Diverticulum?
**Usually Asymptomatic** * Abdominal pain mimicking appendicitis in the RLQ due to inflammation * Rectal Bleeding: **Meckel's Diverticulum is the most common cause of painless massive GI bleeding requiring a transfusion in children between the ages 1-2** * Intestinal obstruction: Secondary to an Omphalomesenteric band, Volvulus and intussusception
370
What are some differential diagnoses for Meckel's Diverticulum?
* **Gastroenteritis:** Presents with abdominal pain, diarrhoea, and possibly fever. * **Appendicitis:** Characterised by right lower quadrant abdominal pain, fever, and nausea or vomiting. * **Peptic ulcer disease:** May cause abdominal pain, heartburn, and bleeding (melena or hematemesis). * **Inflammatory bowel disease:** Symptoms include chronic abdominal pain, diarrhoea, and potentially blood in stool. * **Intestinal obstruction:** Manifests with abdominal pain, vomiting, inability to pass gas or stool, and abdominal distension.
371
What are the investigations for Meckel's Diverticulum?
* **CT scan** * 99mTC scan: To identify ectopic gastric mucosa * Small bowel enema (in some cases)
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What is the Management for Meckel's Diverticulum?
**Surgical removal:** if narrow neck or symptomatic. * Wedge excision * Formal small bowel resection and anastomosis
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What are some complications of Meckel's Diverticulum?
* **Intussusception:** The diverticulum can act as the apex for ileoileal type * **Obstruction:** Can occur if the diverticulum becomes entrapped in a hernia (termed a Littre's hernia) * **Ulceration and perforation:** (eg. by foreign body), can result in peritonitis and massive haemorrhage
374
Define Biliary Atresia?
A congenital condition where a section of the bile duct is either narrowed or absent due to fibrosis and destruction resulting in Cholestasis, Liver failure and potentially death
375
What is the Epidemiology of Biliary Atresia?
* More common in females * Most common cause of Neonatal cholestasis * Neonatal cholestasis 2-8 weeks post birth
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What is the aetiology of Biliary Atresia?
Unknown but thought to be multifactorial. Potentially an aberrant immune response to a viral infection affecting the bile ducts
377
What are the different types of Biliary Atresia?
* **Type I:** Common bile duct is obliterated * **Type II:** Atresia of the cystic duct in the porta hepatis * **Type III:** Most common atresia of the right and left ducts at the level of the porta hepatis
378
What are the clinical features of Biliary Atresia?
**Presents shortly after birth with:** * **Significant Jaundice:** That is persistent for more than **14 days** in term babies and **21 days** in premature babies * **Dark urine and pale stools:** due to the increased conjugated bilirubin and an obstructive jaundice picture * Appetite disturbance * Hepatosplenomegaly * Abnormal Growth
379
What are some differential diagnoses for Biliary Atresia?
* **Alagille syndrome:** Presents with neonatal jaundice, peripheral pulmonary artery stenosis, and characteristic facial features. * **Choledochal cyst:** Presents with the classic triad of abdominal pain, jaundice, and an abdominal mass. * **Neonatal hepatitis:** This condition also presents with jaundice, along with hepatomegaly and elevated liver enzymes. * **Inborn errors of metabolism:** Conditions such as galactosemia and tyrosinemia can present with jaundice, poor feeding, and developmental delay.
380
What are the investigations for Biliary Atresia?
**Blood tests:** * **Serum Conjugated AND unconjugated bilirubin:** Conjugated bilirubin will be raised in biliary atresia as the liver can conjugate the bilirubin but the bile ducts strictures leads to obstruction and therefore it is not excreted * LFTs: Raised **Imaging:** * Hepatic scintigraphy (Technetium-99m scan): The liver will take up the isotope but there will be poor excretion into the bowel, indicating destroyed bile ducts. * **Abdominal ultrasound:** This may reveal echogenic fibrosis. * **Cholangiography:** This is the **definitive diagnostic test**, which will fail to show normal architecture of the biliary tree, confirming biliary atresia.
381
What is the management of Biliary Atresia?
**Surgery via a Kasai Portoenterostomy:** Involves attaching a section of the small intestine to the opening of the liver where the bile duct normally attaches. **Liver Transplant:** Whilst surgery is a successful procedure that prolongs survival, patients often require a full liver transplant to resolve the condition.
382
What are some complications for Biliary Atresia?
* Cirrhosis and HCC * Progressive Liver Disease
383
Define Cow's Milk Protein Allergy?
* A condition typically affecting infants and young children under 3 years. It involves hypersensitivity to the protein in cow’s milk. * This may be IgE mediated, in which case there is a rapid reaction to cow’s milk, occurring within 2 hours of ingestion. * It can also be non-IgE medicated, with reactions occurring slowly over several days.
384
How is Cow's Milk Protein Allergy different to lactose and cow's milk intolerance?
Lactose is a sugar not a protein. Cow's Milk intolerance is not an allergic process and does not involve the immune system
385
What is the Epidemiology of Cow's Milk Protein Allergy?
* Occurs in 3-6% of all children * Typically presents in the first 3 months * More common in formula fed babies and those with a family history of other Atopic conditions * Rarely seen in exclusively breastfed infants
386
What is the presentation of Cow's Milk Protein Allergy?
* **Usually presents prior to 1 yea of age and becomes apparent when child weaned from breast milk to formula** **Gastrointestinal symptoms:** * Bloating and wind * Abdominal pain * Diarrhoea * Vomiting **General allergic symptoms in response to the cow’s milk protein:** * Urticarial rash (hives) * Angio-oedema (facial swelling) * Cough or wheeze * Sneezing * Watery eyes * Eczema * **In rare cases anaphylaxis can occur**
387
How is Cow's Milk Protein Allergy diagnosed?
* Full History and Examination * **Skin prick allergy testing:** can support the diagnosis but is not always necessary
388
What is the Management for Cow's Milk Protein Allergy?
**Avoiding Cows milk should fully resolve symptoms** * Breast feeding mothers should avoid dairy products * Replace formula with special hydrolysed formulas designed for cows milk allergy
389
What is the prognosis of Cow's Milk Protein Allergy?
**Cow's Milk Protein Allergy usually resolves in most children** * In children with IgE mediated intolerance around 55% will be milk tolerant by the age of 5 years * In children with non-IgE mediated intolerance most children will be milk tolerant by the age of 3 years * A challenge is often performed in the hospital setting as anaphylaxis can occur.
390
What are the symptoms of Cow's Milk Intolerance?
Cow's Milk Intolerance will present with similar GI symptoms however it will not give the allergic features seen in Cow's Milk Protein Allergy.
391
Define Infantile Colic?
Infantile colic is characterised by paroxysms of persistent and uncontrollable crying in an otherwise healthy infant.
392
What is the epidemiology of Infantile Colic?
* Extremely Common affecting approximately 15-20% of infants * More common in the first 6 weeks of life.
393
What is the Aetiology of Infantile Colic?
Unknown but likely multifactorial. **Gastrointestinal Aetiology?** * Thought to be a functional GI disorder in infants due to **Gut Microbiome alterations, increased GI inflammatory markers or GI dysmotility**
394
What are the clinical features of Infantile colic?
* **Paroxysms of Uncontrollable crying** **Other Features:** * Facial flushing * Tense abdomen * Drawing up of legs to the abdomen * Clenched fists * Circumoral pallor * Stiffening and tightening of arms * Back arching
395
What are some features of the Cry in Colic that is different to normal infant crying?
* Louder * Higher in frequency * Described as **Screaming** rather than crying * More piercing and grating in nature
396
What is the Criteria to define Infantile Colic?
**Wessell Criteria** * Unexplained crying or fussiness * In an otherwise healthy infant * All red flags and organic causes of crying ruled out (see below in differential diagnosis) * Resolves by 3 months of age * Lasts for greater than 3 hours per day * Occurs on greater than 3 days per week * Persists for greater than 3 weeks
397
How is Infantile Colic Diagnosed?
**Diagnosis of Exclusion** as it occurs in otherwise healthy infants. * Must be absent of **Red Flag Features**
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What **Red Flag** features must be absent for a diagnosis of Infantile Colic?
* Fever * Evidence of diarrhoea, vomiting, abdominal distention * Reduced conscious state e.g. lethargy, drowsiness, floppy * Signs of trauma e.g. bruising, bleeding, fractures * Poor feeding * Poor weight gain and growth * Signs of developmental delay
399
What are some differential diagnoses for Infantile Colic?
* **Normal Crying:** Usually consolable by soothing, feeding or burping * **Intussusception:** will often be present with vomiting and redcurrant jelly stools * **Cow's Milk Protein Allergy:** Presents with other symptoms such as vomiting, diarrhoea and Allergy symptoms * **GORD:** Often occurs after feeding but otherwise and is worse when the infant is lying down. * **UTI:** Fever is likely to be present
400
What is the Management for Infantile Colic?
**Self Limiting and benign condition** that usually resolves by 3-5 months of age. * Caregiver educational support and reassurance * Appropriate feeding techniques * Potential dietary Changes if other first line techniques fail
401
Define Malrotation?
* A congenital anomaly in which the Midgut undergoes abnormal rotation and fixation during embryogenesis. * The misplacement of the gut makes it **Susceptible to Volvulus** a life threatening condition where the bowel loops twist.
402
What is the Epidemiology of Malrotation?
* Rare condition
403
What is the Aetiology of Malrotation?
* Abnormal rotation and fixation of the midgut during embryonic development * Process usually happens between the 4th and 12 weeks of gestation
404
What is the Clinical presentation of Malrotation?
* **Bilious Vomiting:** Often occuring within the first day of life. * Abdominal pain and distension
405
What ae some differential diagnoses for Malrotation?
* **Gastroesophageal reflux disease (GORD):** Typically presents with non-bilious vomiting, failure to thrive, and irritability. * **Pyloric stenosis:** This condition may cause projectile, non-bilious vomiting after feeding, visible peristaltic waves, and a palpable "olive-like" mass in the epigastrium. * **Duodenal atresia:** Presents with bilious vomiting and features "double bubble" sign on abdominal imaging. * **Intestinal obstruction:** Symptoms include bilious vomiting, abdominal pain, distention, and constipation.
406
What are the investigations for Malrotation?
**Upper GI Contrast Study:** Gold standard * Reveals the obstruction point as no contrast can pass distally. * Proximal bowel may have a corkscrew appearance. **Abdominal X-Ray with Contrast** * May show double bubble sign
407
What is the Management for Malrotation?
**Urgent Surgical Laparotomy** to relieve the obstruction and correct the anatomical abnormality
408
What is a major complication of Malrotation?
**Volvulus** leading to bowel obstruction Volvulus is the **twisting of the Bowel Loops** which leads to intestinal obstruction.
409
What is the management of a Volvulus in children?
Ladd's Procedure
410
Define Necrotising Enterocolitis (NEC)?
A Condition affective premature neonates where by a part of the bowel becomes necrotic due to ischaemia leading to infection, inflammation, and potentially, perforation, peritonitis and shock.
411
What is the Epidemiology of NEC?
* Typically presents within the **first 3 weeks** of life in **Premature neonates** * Fatal in 1/5th of cases * Most common surgical emergency in neonates
412
What are some risk factors for NEC?
* Prematurity * Low birth weight (<1500g) * Non-breastfed infants/ Formula feeds * Sepsis * Respiratory distress and acute hypoxia * Poor intestinal perfusion * PDA and other congenital heart defects
413
What is the clinical presentation of Necrotising Enterocolitis?
* Intolerance to feeds * Vomiting: often **Bile streaked** * Fresh Blood in stool * Abdominal distension * Absent Bowel Sounds * Signs of systemic compromise and generally unwell.
414
What are some differential diagnoses for Necrotising Enterocolitis?
* **Sepsis:** May present with systemic signs of illness, vomiting, poor feeding, and lethargy. * **Gastroenteritis:** Presents with diarrhoea and vomiting, but usually without the severe abdominal distension seen in NEC. * **Intestinal malrotation with volvulus:** Typically presents with bilious vomiting, abdominal pain and bloody stools. * **Hirschsprung's disease:** Presents with abdominal distension, constipation, and failure to pass meconium within 48 hours of birth.
415
What are the investigations for Necrotising Enterocolitis?
**Blood Tests:** * **Full blood count** for thrombocytopenia and neutropenia * **CRP** for inflammation * **Capillary/ arterial blood gas** will show a metabolic acidosis * **Blood culture** for sepsis **Abdominal X-ray** Investigation of choice. * Dilated Bowel loops * **Rigler's sign:** Both sides of the bowel are visible due to gas in the peritoneal cavity * Bowel wall oedema (Thickened bowel walls) * Pneumatosis intestinalis (Gas within the bowel wall) * Pneumoperitoneum (Free gas within the peritoneal cavity indicates perforation) * Portal venous gas
416
What is the gold standard investigation for Necrotising Enterocolitis? What may be seen?
**Abdominal X-ray** done in the supine position * Dilated Bowel loops * **Rigler's sign:** Both sides of the bowel are visible due to gas in the peritoneal cavity * Bowel wall oedema (Thickened bowel walls) * Pneumatosis intestinalis (Gas within the bowel wall) * Pneumoperitoneum (Free gas within the peritoneal cavity indicates perforation) * Portal venous gas
417
What is the management of Necrotising Enterocolitis?
* Patient is made **Nil By Mouth** * **IV Fluids** * **NG Tube** for gastric decompression * **Total Parental Nutrition (TPN)** to provide nutrition to the rest of the bowel * **Broad Spectrum Antibiotics:** IV Cefotaxime * **Surgical Intervention:** immediate referral to the neonatal surgical team as resection of necrotic sections may be necessary or in the case of bowel perforation.
418
What are some complications of Necrotising Enterocolitis?
* Perforation and Peritonitis * Sepsis * Death * Strictures * Abscess formation * Recurrence * Long term Stoma * **Short Bowel Syndrome** after surgery
419
What prevention mechanisms can be done to help prevent NEC?
* Encourage breastfeeding in mothers of premature babies * Delayed cord clamping at delivery
420
Define Neonatal Jaundice?
A clinical condition that presents as a yellowing of a newborn's skin and eyes. It results from the accumulation of bilirubin, a by-product of the breakdown of red blood cells, in the body.
421
What is the Epidemiology of Neonatal Jaundice?
* Common condition * Most cases are physiological and self limiting but some may be pathological and represent serious conditions
422
What is the physiology of Neonatal physiological jaundice?
* There is a **high concentration of red blood cells in the foetus and neonate.** * These red blood cells are **more fragile** than normal red blood cells. Foetal red blood cells **break down more rapidly** than normal red blood cells, releasing lots of bilirubin. * The foetus and neonate also have **less developed liver function.** * Normally this bilirubin is excreted via the placenta, however at birth the foetus **no longer has access to a placenta to excrete bilirubin**. * This leads to a **normal rise in bilirubin shortly after birth**, causing a mild yellowing of skin and sclera from 2 – 7 days of age. * This **usually resolves completely by 10 days.** Most babies remain otherwise healthy and well. **In Summary:** * Relative polycythaemia in newborns * Shorter red blood cell lifespan compared to adults * Less efficient hepatic bilirubin metabolism in the first few days of life
423
How can the physiological mechanisms of neonatal jaundice be split?
Increased production of bilirubin Decreased clearance of bilirubin
424
What are some causes of neonatal jaundice due to an increased production of bilirubin?
* Haemolytic disease of the newborn * ABO incompatibility * Haemorrhage * Intraventricular haemorrhage * Cephalo-haematoma * Polycythaemia * Sepsis and disseminated intravascular coagulation * G6PD deficiency
425
What are some causes of neonatal jaundice due to a decreased clearance of bilirubin?
* Prematurity * Breast milk jaundice * Neonatal cholestasis * Extrahepatic biliary atresia * Endocrine disorders (hypothyroid and hypopituitary) * Gilbert syndrome
426
How can the causes of neonatal jaundice be classified?
**Age of Onset:** * Causes **<24 hours:** **Jaundice within the first 24 hours of life is PATHOLOGICAL** * Causes **24 hours - 14 days** * Causes **> 14 days (> 21 days if preterm)**
427
What are some causes of neonatal jaundice that cause jaundice within 24 hours of birth?
* Neonatal Sepsis: Neonates with jaundice within 24 hours of birth and any other clinical features or risk factors for sepsis need sepsis treatment immediately * Haemolytic disorders (Rhesus incompatibility, ABO incompatibility, G6PD deficiency, spherocytosis) * Congenital infections (TORCH screen indicated)
428
What are some causes of neonatal jaundice that cause jaundice within 24 hours and 14 days of birth?
* Physiological jaundice * Breast milk jaundice * Dehydration * Infection, including sepsis * Haemolysis * Bruising * Polycythaemia * Crigler-Najjar Syndrome
429
What are some causes of neonatal jaundice that cause jaundice after 14 days of birth?
* Physiological jaundice but this is longer than would be expected so should prompt further investigation * Breast milk jaundice * Infection * **Hypothyroidism** * **Biliary obstruction (including biliary atresia)** * **G6PD Deficiency** * Neonatal hepatitis
430
What are the clinical features of neonatal jaundice?
* **yellowing of the skin and eyes** * Poor feeding * Lethargy * **Kernicterus** in severe cases where jaundice is left untreated
431
What are some differential diagnoses for Neonatal Jaundice?
* **Haemolytic disorders:** Yellow skin and eyes, pallor, splenomegaly * **Congenital infections:** Fever, lethargy, poor feeding, rash * **Sepsis:** Fever, lethargy, poor feeding, irritability * **Dehydration:** Dry mouth, decreased urination, lethargy * **Bruising:** Discoloration, swelling, tenderness * **Hypothyroidism:** Prolonged jaundice, poor feeding, hypotonia, macroglossia, umbilical hernia * **Biliary obstruction (including biliary atresia):** Prolonged jaundice, clay-coloured stools, dark urine * **Neonatal hepatitis:** Prolonged jaundice, hepatomegaly
432
What initial investigations should be performed in neonatal jaundice?
* Measuring and Plotting **Total Bilirubin Levels** on nomograms (treatment threshold charts). * These take into account the patients gestation, age and bilirubin levels. * If **total bilirubin** reaches threshold then they should be commenced on treatment to lower their bilirubin level. * **Depending on the clinical history, further investigations may also be warranted to elucidate a cause**
433
What further investigations may be used to look for a cause of neonatal jaundice?
* **Full blood count and blood film** for polycythaemia or anaemia ** Conjugated bilirubin:** elevated levels indicate a hepatobiliary cause * **Blood type testing of mother and baby** for ABO or rhesus incompatibility * **Direct Coombs Test (direct antiglobulin test)** for haemolysis * **Thyroid function**, particularly for hypothyroid * **Blood and urine cultures** if infection is suspected. Suspected sepsis needs treatment with antibiotics. * **Glucose-6-phosphate-dehydrogenase (G6PD) levels** for G6PD deficiency
434
What is the management for neonatal jaundice?
**Phototherapy:** Usually adequate to correct neonatal jaundice if bilirubin levels are elevated **Exchange transfusion:** May be required if the bilirubin levels are extremely high.
435
How does phototherapy work to treat neonatal jaundice?
* Converts unconjugated bilirubin into isomers that can be excreted in bile and urine without requiring conjugation in the liver. * **Does not use UV light but uses blue light** * Patients should have a repeat measurement in 24 hours if bilirubin levels near the phototherapy line. * Once complete a rebound bilirubin is measured 12-18 hours later to ensure the bilirubin levels do not rise above threshold again.
436
What is a major complication of neonatal jaundice?
**Kernicterus** * Type of brain damage caused by excessive bilirubin levels * Bilirubin can cross the BBB and directly damage the CNS. * The damage to the CNS is permeant causing cerebral palsy, learning disability and deafness.
437
How does Kernicterus present?
A less responsive, floppy, drowsy baby with poor feeding.
438
Define a Urinary Tract Infection
An infection that can occur in any section of the urinary tract, which includes the kidneys, ureters, bladder, and urethra.
439
What is the Epidemiology of UTIs in children?
* More common in infants and young children * More common in females
440
What is the aetiology of a UTI in children?
Bacterial invasion of the urinary tact * **E. coli** * **Klebsiella** * **Proteus** * **Staph saprophyticus**
441
Define acute Pyelonephritis?
Inflammation and infection of the kidney that can lead to scarring and consequently a reduction in kidney function.
442
Define Cystitis
Inflammation of the bladder
443
What are the clinical features of UTIs in children?
Signs and Symptoms may differ depending on the age of the child: **Infants under 3 months:** * Fever * Vomiting * Lethargy * Irritability * Poor feeding * Failure to thrive * Offensive urine **Infants aged 3-12 months:** * Fever * Poor feeding * Abdominal pain * Vomiting **Children over 1 year old:** * Frequency * Dysuria * Abdominal pain **Fever becomes less common in children over one year old.**
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What are some differential diagnoses for UTIs in children?
* **Appendicitis:** Mainly characterised by severe abdominal pain, vomiting, and sometimes fever. * **Pylonephritis:** Symptoms may include fever, flank pain, nausea, vomiting, and frequent urination. * **Cystitis:** Presents with dysuria, urinary frequency, and lower abdominal pain. * **Vesicoureteral reflux:** Recurrent UTIs, persistent bacteriuria, and unexplained fevers may suggest this condition.
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What are the investigations for a UTI in children?
**Urine Dipstick:** Raised leukocytes and nitrites **Clean Catch Urine Sample and Culture** **Older children can have MSU**
446
What clinical features may point towards a diagnosis of acute pyelonephritis?
* Temperature greater than **38 degrees** * **Loin pain or tenderness** * Nausea and vomiting
447
What is the management for UTIs in children?
**All children under 3 months with a fever should start immediate IV antibiotics (Cefotaxime)** * Oral antibiotics can be considered in children over 3 months * **Nitrofurantoin** often for Lower UTIs * **Cephalosporins (cefalexin)** often for upper UTIs
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How are recurrent UTIs tested for?
**Ultrasound Scans:** * All children under 6 months with their first UTI should have an abdominal ultrasound within 6 weeks, or during the illness if there are recurrent UTIs or atypical bacteria * Children with recurrent UTIs should have an abdominal ultrasound within 6 weeks * Children with atypical UTIs should have an abdominal ultrasound during the illness **Dimercaptosuccinic Acid (DMSA) Scan:** * Used 4-6 months after illness to assess for damage from recurrent or atypical UTIs.
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How can UTIs be prevented?
* High fluid intake to produce a high urine output * Regular Voiding * Ensuring complete bladder emptying * Prevention/treatment of Constipation * Prophylactic Abx can be considered.
450
Define Enuresis?
Involuntary urination
451
Define Nocturnal Enuresis?
Also known as **Bed wetting** A condition where an individual involuntarily urinates during sleep. It is considered a typical part of development until the age of 5.
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How can Nocturnal Enuresis be categorised?
**Primary:** Children who have never achieved urinary continence overnight **Secondary:** Children who have previously achieved night time continence for **at least 6 months** but have subsequently lost it.
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What is the epidemiology of Nocturnal Ensuresis?
* More prevalent in boys * Usually no underlying physiological condition * 2/3 of cases has strong family history of delayed dry nights.
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What is the Aetiology of Primary Nocturnal Enuresis?
* **Variation of Normal Development in < 5s** * Overactive bladder. Frequent small volume urination prevents the development of bladder capacity. * Fluid intake prior to bedtime, particularly fizzy drinks, juice and caffeine, which can have a diuretic effect * Failure to wake due to particularly deep sleep and underdeveloped bladder signals * Psychological distress, for example low self esteem, too much pressure or stress at home or school * Secondary causes such as chronic constipation, urinary tract infection, learning disability or cerebral palsy
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What is the Aetiology of Secondary Nocturnal Enuresis?
* Urinary tract infection * Constipation * Type 1 diabetes * New psychosocial problems (e.g. stress in family or school life) * Maltreatment
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What are the clinical features of Nocturnal Enuresis?
* **Involuntary Urination during sleep** * Signs of other conditions in the cases of Secondary Nocturnal Enuresis
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What are some differential diagnoses for Nocturnal Enuresis?
* **Diabetes mellitus:** Symptoms may include polyuria, polydipsia, unexplained weight loss, and persistent hunger. * **Urinary tract infections:** Symptoms can involve dysuria, urinary frequency, lower abdominal pain, and fever. * **Constipation:** Symptoms can include less frequent bowel movements, hard or dry stools, abdominal pain, and bloating.
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What are the investigations for Nocturnal Enuresis?
**Establish underlying causes** * 2 Week Diary of Toileting, fluid intake and bed wetting episodes. * Detailed history, examination and urine dip **Investigations for Secondary Causes:** * Urine dip * Urine osmolarity * Renal Ultrasound
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What is the management of Primary Nocturnal Enuresis?
* Reassure parents of children under 5 years that it is likely to resolve without any treatment * Lifestyle changes: reduced fluid intake in the evenings, pass urine before bed and ensure easy access to a toilet * Encouragement and positive reinforcement. **Star charts** Avoid blame or shame. Punishment should very much be avoided. * Treat any underlying causes or exacerbating factors, such as constipation * Enuresis alarms * Pharmacological treatment: Trial of DDAVP (Synthetic ADH)
460
What is the management of Secondary Nocturnal Enuresis?
Treat the underlying causes: often UTIs or constipation.
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What is Diurnal Enuresis?
Daytime incontinence. Most children have control of daytime urination by 2 years old. This occurs more frequently in girls
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What are the 2 main causes for Diurnal Enuresis?
* **Urge Incontinence:** Overactive bladder that gives little warning before emptying * **Stress Incontinence:** Leakage of urine during physical exertion, coughing or laughing. **Other Causes:** * Recurrent UTIs * Psychosocial problems * Constipation
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What are Enuresis Alarms?
* An enuresis alarm is a device that makes a noise at the first sign of bed wetting, waking the child and stopping them from urinating. * It requires quite a high level of training and commitment and needs to be used consistently for a prolonged period (i.e. at least 3 months). * Some families may find them very helpful, whereas others may find they add to the burden and frustration and are counter productive.
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What are some pharmacological treatment options for Nocturnal Enuresis?
Desmopressin: ADH analogue Oxybutynin: Anti-cholinergic that reduces bladder contraction Imipramine: TCA
465
Define Acute Kidney Injury (AKI)?
Characterized by a rapid and sustained decrease in renal function, leading to oliguria, disturbed electrolytes, fluid balance, and the accumulation of urea and waste products. This reduction is biochemically manifested by an increase in urea and creatinine levels.
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What is the KDIGO Criteria for an AKI?
* Increase in serum creatinine by **≥25 µmol/l within 48 h**, or * Increase in serum creatinine **≥ 1.5x (50% increase) the baseline** within the last 7 days, or * Urine volume **< 0.5 ml/kg/h** for 6 hours
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What is the Epidemiology of AKI?
AKI affects 10-20% of all hospitalized patients and up to 50% of critically ill patients in intensive care.
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What are some risk factors that may predispose someone to an AKI?
* Older age (e.g., above 65 years) * Sepsis * Chronic kidney disease * Heart failure * Diabetes * Liver disease * Cognitive impairment (leading to reduced fluid intake) * Medications (e.g., NSAIDs, gentamicin, diuretics and ACE inhibitors) * Radiocontrast agents (e.g., used during CT scans)
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What are the Pre-renal causes of an AKI?
The most common. **Insufficient blood supply (hypoperfusion) to kidneys reduces the filtration of blood.** This may be due to: * Dehydration * Shock (e.g., sepsis or acute blood loss) * Heart failure * Renovascular (renal artery stenosis) * Autoregulation due to NSAIDs, ACEis, ARBs
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What are the renal causes of an AKI?
Renal causes are due to **intrinsic disease in the kidney.** This may be due to: * Acute tubular necrosis * Glomerulonephritis * Acute interstitial nephritis * Haemolytic uraemic syndrome * Rhabdomyolysis
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What are the Post-Renal causes of an AKI?
involve **obstruction to the outflow of urine away from the kidney,** causing back-pressure into the kidney and reduced kidney function. This is called an obstructive uropathy. Obstruction may be caused by: * Kidney stones * Posterior urethral valves * Tumours (e.g., retroperitoneal, bladder or prostate) * Strictures of the ureters or urethra * Benign prostatic hyperplasia (benign enlarged prostate) * Neurogenic bladder
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What is the most common cause of AKI in children?
Pre-renal (hypovolaemia) due to infections such as gastroenteritis burns, sepsis, haemorrhage and nephrotic syndrome
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What is Acute Tubular Necrosis?
Damage and death of the epithelial cells of the renal tubules. It is the most common intrinsic cause of an AKI and occurs due to Ischaemia or Nephrotoxins **Muddy Brown Casts** on urinalysis confirms acute tubular necrosis
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What are the clinical features of an AKI?
* Rapid rise in serum creatinine levels and urea * **Oliguria:** Decrease in urine output (<0.5 ml/kg/h for 6 hours) * **Fluid overload signs** (e.g., edema, hypertension, pulmonary edema) * Signs related to underlying cause (e.g., sepsis, rashes in vasculitis) * Signs of uremia in severe cases (e.g., fatigue, anorexia, nausea, pruritus, altered mental status)
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What investigations are used to diagnose AKI?
* **Bloods:** Full blood count (FBC), urea and electrolytes (U&Es), liver functions tests (LFTs), glucose, clotting, bone profile, creatine kinase (CK), C-reactive protein (CRP). * **Venous blood gas (VBG)/arterial blood gas (ABG):** For acidosis, hypoxia, urgent potassium level. * **Urine tests:** Dipstick (looking for blood and protein), microscopy, culture & sensitivity (MC&S; to exclude infection), biochemistry (electrolytes, osmolality), urine protein:creatinine ratio (uPCR; to quantify proteinuria). * **ECG:** To look for hyperkalaemia. * **Chest X-ray (CXR):** To identify pulmonary oedema. * **Renal ultrasound:** To evaluate renal size (normal is 10–13 cm) and echotexture, hydronephrosis, structural kidney disease. **If the cause is unclear then an acute renal screen may be necessary**
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What is the management for an AKI?
**Regular Monitoring of Circulation and Fluid Balance** * IV fluids for dehydration and hypovolaemia * Withhold medications that may worsen the condition (e.g., NSAIDs and ACE inhibitors) * Withhold/adjust medications that may accumulate with reduced renal function (e.g., metformin and opiates) * Relieve the obstruction in a post-renal AKI (e.g., insert a catheter in a patient with prostatic hyperplasia) * Dialysis may be required in severe cases
477
# AEIOU What are the indications for acute dialysis or haemofiltration?
**A**cidosis (severe metabolic acidosis with pH of <7.20) **E**lectrolyte imbalance (resistant hyperkalaemia) **I**ntoxication (drug overdose, poisoning) **O**edema (refractory pulmonary oedema) **U**raemia (encephalopathy or pericarditis)
478
What are some complications of an AKI?
* Fluid overload, heart failure and pulmonary oedema * Hyperkalaemia * Metabolic acidosis * Uraemia (high urea), which can lead to encephalopathy and pericarditis
479
What is defined as Chronic Renal Failure in Children?
eGFR < 15 ml/min
480
What are some causes of Chronic Renal Failure in Children?
* Structural Malformations * Glomerulonephritis * Hereditary Nephropathies (PKD) * Systemic Diseases
481
What is the clinical presentation of Chronic renal failure in children?
**Symptoms generally do not develop until renal function falls to less than 1/3rd of normal. * Often picked up on Antenatal ultrasound * Anorexia and Lethargy * Polydipsia and Polyuria * Faltering Growth * Hypertension * Acute on Chronic Renal failure precipitated by infection or dehydration * Incidental finding of proteinuria
482
What is the management of Chronic Renal Failure in Children?
* Sufficient feeding with good protein intake to maintain growth -> this can be supplemented with NG/gastrostomy feeding if necessary * Phosphate restriction and activated vit D to prevent renal osteodystrophy * Bicarbonate supplements to prevent acidosis * EPO to prevent anaemia * Growth hormone * Dialysis and transplantation if necessary
483
Define Nephrotic Syndrome?
Nephrotic syndrome occurs when the basement membrane in the glomerulus becomes highly permeable to protein, allowing proteins to leak from the blood into the urine. * It is most common between the ages of 2 and 5 years. * It presents with frothy urine, generalised oedema and pallor.
484
What is the Triad for Nephrotic Syndrome?
* Low serum albumin < 25g/dl * High urine protein content (>3+ protein on urine dipstick or a urine Protein:Creatinine ratio of > 200mmg/mol) * Oedema
485
What are the causes of Nephrotic Syndrome?
* **Minimal Change Disease:** causing over 90% of cases in children under 10 ** Secondary to Intrinsic Kidney Disease:** * Focal Segmental Glomerulosclerosis * Membranous Nephropathy * Membranoproliferative Glomerulonephritis **Systemic Illness:** * Henoch Schonlein Purpura * Diabetes * Infection: HIV, Hepatitis, Malaria
486
What is Minimal Change disease?
Most common cause of Nephrotic syndrome in children. Unknown Aetiology Despite the clinical symptoms the condition is marked by minimal or no change visible under light microscopy to nephrological structures. More subtle changes are seen on electron microscopy.
487
What are the clinical features of Nephrotic Syndrome?
* Low serum albumin: causing ankle swelling * **Frothy Urine:** High urine protein content (>3+ protein on urine dipstick) * **Oedema:** Facial and periorbital swelling * Fatigue * Weight gain due to fluid retention * Deranged lipid profile, with high levels of cholesterol, triglycerides and low density lipoproteins * High blood pressure * Hyper-coagulability, with an increased tendency to form blood clots
488
What are some differential Diagnoses for Minimal Change disease?
* **Focal segmental glomerulosclerosis (FSGS):** Presents with proteinuria, hypertension, and oedema. However, FSGS often progresses to kidney failure and responds less well to corticosteroids compared to MCD. * **Membranous nephropathy:** Characterized by heavy proteinuria, hypertension, and oedema. It typically affects adults and is less common in children. * **Secondary causes of nephrotic syndrome:** Conditions such as diabetes, lupus, or certain infections and medications can lead to nephrotic syndrome.
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What are the investigations for Minimal Change disease?
* **Urinalysis:** Identify proteinuria, **Hyaline Casts** * **Blood Tests:** Low albumin and elevated cholesterol * **Renal Biopsy:** * standard microscopy in minimal change disease will not detect any abnormality * Electron Microscopy will show diffuse loss of podocyte foot processes, vacuolation, and the appearance of microvilli.
490
What is the management of Minimal Change disease?
**Corticosteroid Therapy:** Prednisolone 60mg/m2/alternate days for 28 days (Max dose is 80mg) **2nd Line:** Oral immunosuppressive agents such as ciclosporin, Tacrolimus in steroid resistant children **Fluid Restriction and reduced salt intake** to manage oedema and prevent further complications * Diuretics (Furosemide) may be used to treat oedema: 1-2mg/kg/day * Albumin infusions may be required in severe hypoalbuminaemia * Pneumococcal immunisations
491
What are some complications of Nephrotic Syndrome?
* **Hypovolaemia** occurs as fluid leaks from the intravascular space into the interstitial space causing oedema and low blood pressure. * **Thrombosis** can occur because proteins that normally prevent blood clotting are lost in the kidneys, and because the liver responds to the low albumin by producing pro-thrombotic proteins. * **Infection** occurs as the kidneys leak immunoglobulins, weakening the capacity of the immune system to respond. This is exacerbated by treatment with medications that suppress the immune system, such as steroids. * **Acute or chronic renal failure** * **Relapse**
492
What is the Prognosis for Minimal Change disease?
* 1/3 of patients resolve completely and never have another episode. * 1/3 have further relapses requiring additional steroid treatment. * 1/3 are dependent on continued steroid/immunosuppression therapy.
493
Define Nephritic Syndrome?
Group of kidney disorders that result in the presence of red blood cells in urine (haematuria), non-nephrotic range proteinuria, and often hypertension. These conditions are typically characterized by inflammation and damage to the glomeruli, the tiny blood vessels within the kidneys that filter waste and excess water from the bloodstream.
494
What is the Epidemiology of Nephritic Syndrome?
* Can occur at any age, but certain causes such as post-streptococcal glomerulonephritis and IgA nephropathy are more common in children and young adults. * Other causes like rapidly progressive glomerulonephritis and Goodpasture's disease occur more frequently in older adults.
495
What is the Aetiology of Nephritic Syndrome?
**From conditions that cause inflammation and damage to the glomeruli. These include:** * **Autoimmune diseases**, such as systemic lupus erythematosus (SLE) or Henoch-Schönlein purpura * **Infections**, such as post-streptococcal infection * **Genetic disorders**, such as Alport's syndrome * **Other diseases** that damage the kidney, including Goodpasture's disease, rapidly progressive glomerulonephritis, IgA nephropathy, and membranoproliferative glomerulonephritis
496
What are the most common causes of Nephritic Syndrome in Children?
**Post Strep Glomerulonephritis** **IgA Nephropathy (Berger's Disease)**
497
What is the triad of Nephritic syndrome?
* Haematuria, often microscopic but may be macroscopic * Hypertension * Non-nephrotic range proteinuria * and Oedema but less severe than nephrotic syndrome
498
What are the clinical features of Nephritic Syndrome?
* Haematuria, often microscopic but may be macroscopic * Hypertension * Non-nephrotic range proteinuria * Oedema (less severe than in nephrotic syndrome) In severe cases, patients may experience symptoms of acute kidney injury such as oliguria or anuria.
499
# SHARP AIM what are some differential diagnoses for Nephritic Syndrome?
* **SLE:** Presents with a wide range of symptoms including fatigue, joint pain, rash, and fever. Renal involvement can cause nephritic syndrome. * **Henoch-Schönlein purpura:** Symptoms include a purpuric skin rash, joint pain, gastrointestinal symptoms, and nephritic syndrome. * **Anti-GBM disease (Goodpasture's disease):** Presents with a combination of lung and kidney disease, including coughing, shortness of breath, hemoptysis, and nephritic syndrome. * **Rapidly Progressive glomerulonephritis (GN):** Patients may present with rapidly declining kidney function over weeks to months, often with nephritic syndrome. * **Post-streptococcal GN:** Typically presents 3-4 weeks after a streptococcal throat or skin infection, with features of nephritic syndrome. * **Alport's syndrome:** A genetic disorder that often presents with hearing loss, eye abnormalities, and nephritic syndrome. * **IgA nephropathy (Berger's disease):** Typically presents 1-2 days following a respiratory or gastrointestinal infection, often with macroscopic hematuria and nephritic syndrome. * **Membranoproliferative GN:** Presents with nephritic or nephrotic syndrome, or a combination of both. May be associated with chronic infections or autoimmune diseases.
500
What is Post Strep Glomerulonephritis?
Post-streptococcal glomerulonephritis occurs **1 – 3 weeks after a β-haemolytic streptococcus infection**, such as tonsillitis caused by Streptococcus pyogenes. Immune complexes made up of streptococcal antigens, antibodies and complement proteins get stuck in the glomeruli of the kidney and cause inflammation. This inflammation leads to an acute deterioration in renal function, causing an acute kidney injury.
501
How does Post Strep Glomerulonephritis Present?
**PSGN patients typically present with a sudden onset of haematuria, oliguria, hypertension, and/or oedema 1–3 weeks post-infection.** Some patients may remain asymptomatic, revealing only microscopic haematuria upon investigation.
502
What are the investigations for Post Strep Glomerulonephritis?
**The first-line investigation consists of urinalysis and microscopy and culture:** * Urinalysis typically tests positive for blood and sometimes protein. * Urine microscopy usually reveals the presence of dysmorphic red blood cells, indicating bleeding from the glomerulus. * A full blood count may demonstrate raised white cell count, indicative of an infectious process. * Urea and electrolytes often depict an acute kidney injury (AKI). * **Immunoglobulins, complement (with low C3 levels being common), and autoantibodies** such as raised anti-streptolysin titre and raised DNAse B titre can aid in establishing the diagnosis. * Blood cultures are warranted in febrile patients. **The gold-standard method for diagnosis in adults is a renal biopsy** where the classical finding is subepithelial 'humps' on electron microscopy.
503
What is the Management for Post Strep Glomerulonephritis?
**Management is mainly focused on handling the AKI as per standard treatment guidelines for any cause of AKI.** * Antihypertensives and diuretics * As the course of the infection is generally self-limiting, additional specific therapies for PSGN are usually unnecessary
504
What is IgA Nephropathy?
IgA nephropathy (IgAN) is a type of glomerulonephritis (GN) distinguished by the deposition of IgA in the mesangium of the glomerulus. It holds the title as the most common form of primary GN globally.
505
What is the clinical presentation of IgA Nephropathy?
* Recurrent gross or microscopic haematuria, generally occurring **12–72 hours after an upper respiratory tract or gastrointestinal infection.** * Mild proteinuria. * Hypertension. * Less commonly, presentations can include nephrotic syndrome or a rapidly progressive GN, resulting in acute renal failure. * Associations with Henoch-Schönlein purpura (HSP)/IgA vasculitis, chronic liver disease, inflammatory bowel disease (IBD), and skin and joint disorders, such as psoriasis, have been observed.
506
What are the investigations for IgA Nephropathy?
**Initial investigation with a urinalysis and urine MC&S.** * Urinalysis often reveals presence of blood ± protein, with dysmorphic red blood cells on urine microscopy suggesting glomerular bleeding. **The gold-standard diagnostic tool for IgAN is renal biopsy** * which shows diffuse mesangial IgA immune complex deposition. * Serum IgA levels are elevated in approximately 50% of patients.
507
What is the management of IgA Nephropathy?
**The primary approach to management involves supportive care and reduction of cardiovascular risk:** * Dietary salt restriction. * Proteinuria management (>0.5 g/day) with an ACE inhibitor or angiotensin II receptor blocker (ARB). * Hypertension treatment. **Patients are risk stratified into low and high risk of progression to chronic kidney disease (CKD) and end-stage kidney disease (ESKD):** * High-risk individuals are considered for corticosteroid treatment * Immunosuppression is offered for patients presenting with a rapidly progressive GN.
508
Define Henoch-Schonlein Purpura (HSP)
* IgA vasculitis, is an immune-mediated small vessel vasculitis. * It predominantly affects children, especially those aged 3-5 years, * It is characterized by palpable purpura, arthritis or arthralgia, abdominal pain, and renal disease.
509
What is the Epidemiology of HSP?
* Most common Vasculitis in children * Peak incidence in 3-5s * More common in males * More common in autumn and winter following URTI
510
What is the Aetiology of HSP?
Unknown but thought to be immune mediated as it is often preceded by an URTI
511
What are the clinical features of HSP?
* Purpura or petechiae, primarily located on the buttocks and lower limbs * Abdominal pain * Arthralgia or arthritis, predominantly in the knees and ankles * Renal involvement - Nephritis (hematuria and/or proteinuria) * Patients may present with a fever * Often, a history of a recent upper respiratory tract infection
512
What are some differential Diagnoses for HSP?
* **Leukocytoclastic vasculitis:** Presents with palpable purpura, but typically lacks the systemic features of HSP * **Idiopathic thrombocytopenic purpura (ITP):** Presents with petechiae and purpura, but lacks abdominal pain, arthralgia, and renal involvement * **Meningococcemia:** Presents with purpuric rash and fever, but also includes symptoms like rapid disease progression, severe illness, and potential neurological symptoms.
513
What are the investigations for HSP?
**Primarily a clinical diagnosis:** * Urinalysis: To monitor for nephritis (haematuria and proteinuria) * Blood pressure: To assess for hypertension secondary to renal involvement * In severe cases, a kidney biopsy may be necessary
514
What is the management of HSP?
* Analgesia and anti-inflammatory effects are typically achieved with NSAIDs * Antihypertensives may be needed to control blood pressure in cases of significant renal involvement * Following an episode of HSP, regular urine tests should be conducted for 12 months to monitor for potential renal impairment.
515
Define Hypospadias?
A congenital condition affecting males, where the urethral meatus (the opening of the urethra) is abnormally displaced to the ventral side (underside) of the penis, towards the scrotum. This might be further towards the bottom of the glans (in 90% of cases), halfway down the shaft or even at the base of the shaft.
516
What are Epispadias?
Where the urethral meatus is displaced to the dorsal side of the penis
517
How are Hypospadias diagnosed?
These are congenital conditions that ae usually diagnosed on examination of the newborn (NIPE Exam)
518
What is the management of Hypospadias?
**Warn parents not to circumcise the infant until a urologist says it is ok** * **Mild Cases:** May not require any treatment **moderate/severe cases:** * Surgery: performed after 3-4 months of age * Surgery aims to correct the position of the meatus and straighten the penis
519
What are some potential complications of Hypospadias?
* Difficulty directing urination * Cosmetic and psychological concerns * Sexual dysfunction
520
Define Haemolytic Uraemic Syndrome (HUS)?
* A renal-limited form of **thrombotic microangiopathy.** * This syndrome is characterised by the triad of microangiopathic haemolytic anaemia (MAHA), thrombocytopenia, and acute kidney injury (AKI). * Usually this is triggered by Shiga Toxins from either **E.coli 0157 or Shigella**
521
What is the Epidemiology of HUS?
* Most often affects children following an episode of gastroenteritis * Typical cases (80-90%) are associated with Diarrhoea and Food poisoning * Atypical cases (10%) are associated with complement deficiency
522
What is the Aetiology of HUS?
**Typical HUS:** Most often caused by bacterial infections, especially E. coli 0157:H7 subtype, but also Shigella, Salmonella, Yersinia, and Campylobacter. **Atypical HUS:** Predominantly due to inherited or autoimmune complement deficiencies. **Antibiotics and antimotility agents used to treat gastroenteritis caused by E.coli 0157 or shigella can increase the risk of HUS**
523
What is the classical triad of HUS?
Classic Triad of: * Microangiopathic haemolytic anaemia * Acute kidney injury * Thrombocytopenia (low platelets)
524
What is the clinical presentation of HUS?
E. coli O157 and Shigella cause gastroenteritis. **Diarrhoea is the first symptom**, which turns bloody within 3 days. Around a week after the onset of diarrhoea, the features of HUS develop: * Fever * Abdominal pain * Lethargy * Pallor * Reduced urine output (oliguria) * Haematuria * Hypertension * Bruising * Jaundice (due to haemolysis) * Confusion
525
What are some differential diagnoses for HUS?
**Thrombotic Thrombocytopenic Purpura**
526
What is the pathophysiology of HUS?
The formation of blood clots consumes platelets, leading to thrombocytopenia. The blood flow through the kidney is affected by thrombi and damaged red blood cells, leading to acute kidney injury. Microangiopathic haemolytic anaemia (MAHA) involves the destruction of red blood cells (haemolysis) due to pathology in the small vessels (microangiopathy). Tiny blood clots (thrombi) partially obstruct the small blood vessels and churn the red blood cells as they pass through, causing them to rupture.
527
What are the investigations for HUS?
**Urine dipstick:** May show haematuria and non-nephrotic range proteinuria. **Full blood count (FBC):** Shows normocytic anaemia (secondary to haemolysis), thrombocytopenia, and possibly a raised neutrophil count. **Urea and Electrolytes (U&Es):** Show a raised urea and creatinine. **Coagulation studies:** Typically normal. **Blood film:** Will reveal reticulocytes (secondary to haemolysis) and schistocytes (fragmented red cells). **Liver function tests (LFT), lactate dehydrogenase (LDH), D-dimer:** Will be raised, consistent with haemolysis. **Haptoglobin:** Will be low, consistent with haemolysis. **Stool culture:** Particularly important in typical HUS to identify the causative pathogen.
528
What is the management of HUS?
**A medical emergency that requires hospital admission and supportive care:** * Hypovolaemia (e.g., IV fluids) * Hypertension * Severe anaemia (e.g., blood transfusions) * Severe renal failure (e.g., haemodialysis)
529
What is the prognosis of HUS?
HUS is self limiting and most patients fully recover with **Good early supportive care**
530
Define Phimosis?
* The condition where the **foreskin is too tight to be retracted over the glans of the penis.** * This condition is considered normal in infants and young children but is expected to resolve naturally over time. * In adults, the presence of phimosis may be indicative of an underlying pathological condition.
531
Define Paraphimosis?
The inability to **replace the foreskin to its original position after it has been retracted**, leading to venous congestion and potentially causing oedema and ischaemia of the glans penis.
532
What is the Epidemiology of Phimosis?
Phimosis is normal in infants and young children, while paraphimosis is often seen in adults and elderly, particularly in those with catheterization or following improper foreskin care.
533
What is the Aetiology of Phimosis in adults?
**Various conditions that cause scarring of the foreskin and/or glans, leading to adhesions. ** These include: * Sexually Transmitted Infections (STIs) * Eczema * Psoriasis * Lichen planus * Lichen sclerosis * Balanitis
534
What are the clinical features of Phimosis?
The main symptom is the inability to retract the foreskin. In some severe cases, it can interfere with normal urination or sexual function.
535
What are the clinical features of paraphimosis?
The main signs include a swollen and painful glans, and a tight band of foreskin behind the glans. If left untreated, it can lead to signs of ischaemia such as discolouration and severe pain.
536
What are some differential diagnoses for Phimosis?
* **Balanitis Xerotica Obliterans (BXO):** Presents with whitish skin changes, pruritus, painful erections, and difficulty with micturition. * **Balanitis:** Signs and symptoms include redness, swelling, and a discharge with a foul smell.
537
What are some differential diagnoses for Paraphimosis?
* **Penile Fracture:** Characterised by a cracking sound, immediate detumescence, pain, swelling, and a hematoma. * **Penile Carcinoma:** Usually presents as a painless lump or ulcer on the penis.
538
What are the investigations for Phimosis and Paraphimosis?
**Clinical diagnosis based on history and physical examination** * May use ultrasound or uroflowmetry in uncertain cases or if complications are suspected
539
What is the management of Phimosis?
* Topical steroid creams to reduce inflammation and encourage stretching of the foreskin * Circumcision may be considered in recurrent or refractory cases
540
What is the management of Paraphimosis?
* Apply manual pressure over time to reduce oedema to the glans * **Dorsal Slit** may be used to cut the foreskin and relieve constriction.
541
What are some potential complications of Phimosis and Paraphimosis?
Urinary Retention Penile Ischaemia
542
Define Vesicouretric reflux (VUR)?
Developmental abnormality where the ureters are displaced and enter directly into the bladder rather than at an angle causing reflux of urine into the renal pelvis and can cause scarring with UTIs.
543
What is the epidemiology of Vesicoureteric Reflux?
* Most common in infants and young children
544
What is Primary VUR?
**Most common type** Occurs when a child is born with a defect at the vesicoureteric junction. This spot normally works as a valve however if there is a defect then this valve cannot close and thus when the bladder is filled it enables urine to reflux into the ureter.
545
What is Secondary VUR?
Obstruction at some point in the urinary tract that causes increased pressure and back flow into the ureters. Most commonly caused by: * Recurrent UTIs * Posterior Urethral valve disorder * Flaccid neurogenic bladder
546
How is VUR classified?
**Grade I:** Urine backs up into ureters **Grade II:** Urine fills ureters and renal pelvis **Grade III:** Urine fills and stretches the ureter and renal pelvis **Grade IV:** Ureter becomes curvy and the renal pelvis and calices become swollen. **Grade V:** Swelling of the ureter and renal pelvis and calyx causing renal failure
547
What are the clinical features of VUR?
* Milder cases: no symptoms Severe cases: * Recurrent UTIs * Unexplained fevers * Persistent bacteriuria
548
What are the investigations for VUR?
**Abdominal Ultrasound:** Detects blockages and swelling **Voiding Cystourethrogram (VCUG):** Shows how urine moves through the vesicoureteric junction using contrast dye. **Radionuclide cystogram:** Tracer injected into blood vessel which then traces the path.
549
What is the management for VUR?
**Depends on severity and age** * May improve with age and without input * May require surgery: remove a blockage or repair a valve
550
Define Eczema?
Eczema is a **chronic atopic condition** caused by defects in the normal continuity of the skin barrier, leading to **inflammation** in the skin Also known as **Atopic Dermatitis*
551
What is the Epidemiology of Eczema?
* Atopic dermatitis is one of the most common skin disorders globally, affecting people of all ages. * Childhood onset is common, with up to **20% of children affected.** * Prevalence decreases with age, but adult-onset cases can occur. * **A family history of atopy (e.g. asthma, allergic rhinitis) is a significant risk factor.** * Urbanisation and industrialisation are associated with a higher prevalence.
552
What is the Pathophysiology of Eczema?
* The simplified pathophysiology is that eczema is caused by defects in the barrier that the skin provides. * Tiny gaps in the skin barrier provide an entrance for irritants, microbes and allergens that create an immune response, resulting in inflammation and the associated symptoms. * In many cases, atopic dermatitis is associated with an IgE-mediated allergic response to environmental allergens.
553
What may be seen on histology in Atopic Dermatitis?
* **Epidermal acanthosis:** thickening of the epidermis due to hyperplasia. * **Hyperkeratosis:** thickening specifically of the stratum corneum. * **Parakeratosis:** retained nuclei in the stratum corneum indicating problems with the usual differentiation process.
554
How can Dermatitis be classified?
* Atopic eczema * Allergic contact dermatitis * Irritant contact dermatitis * Seborrheic dermatitis * Venous eczema (stasis dermatitis) * Asteatotic dermatitis (eczema craquele) * Erythrodermic eczema * Pompholyx eczema
555
What are the clinical features of Atopic Eczema?
* Childhood predominance: symptoms tend to become less severe with age. * Associated with atopic phenotype: asthma, hayfever, raised eosinophils. * In infants, the face is a common site. In older children/adults, the antecubital fossa and posterior knee **(flexor surfaces)** are affected. * The skin is itchy, erythematous, and oozing. There may be vesicles, which may have crusted over. * **Episodic flares** * Eventually, the skin becomes dry and flaky. Repeated scratching causes lichenification (thick, leathery skin, also called lichen simplex et chronicus.)
556
What is Erythrodermic Eczema?
* This is a dermatological emergency and may complicate atopic eczema. * It is syndrome characterised by widespread redness (>90%) * There is often skin exfoliation too, which leads to exfoliative dermatitis.
557
What are the clinical features of Stasis dermatitis?
* Also known as venous eczema. * This is eczema associated with chronic venous insufficiency (venous hypertension), usually affecting the gaiter area. * There may be associated skin changes therefore, including: venous ulcers, lipodermatosclerosis, and haemasiderosis.
558
What are the clinical features of Pompholyz Eczema?
* This is a subtype of eczema associated with intensely itchy vesicles that erupt in the hands. * It is also referred to as dishydrotic eczema.
559
What are the clinical features of Eczema Craquele?
Eczema associated with dry skin.
560
What does this show?
Atopic dermatitis seen on the ankle joint, upper leg and buttocks of an infant.
561
What are the investigations for Eczema?
**Clinical Diagnosis**
562
What is the management of Eczema?
**Management of Flares** * Conservative management * **Use liberal Emollient + Topical Steroid** **Emollients:** * Use liberally (Thin or thick layers depending on severity) * This creates a barrier for the skin. **Topical Steroids:** * Steroid ladder depending on the severity of Eczema * **Mild:** Hydrocortisone (0.5%, 1% and 2.5%) * **Moderate:** Eumovate (clobetasone butyrate 0.05%) * **Potent:** Betnovate (beclomethasone 0.1%) * **Very potent:** Dermovate (Clobetasol propionate 0.05%) **Specialist Treatments:** * Oral Steroids * DMARDs (Methotrexate, Azathioprine, Tacrolimus, Ciclosporin) * Biologics (Baricitinib)
563
What are some conservative management steps that can be taken for Eczema?
* Avoid triggers: soaps, perfumes, biological detergents, or synthetic fabrics. Replace these where possible (soap substitutes, non biological detergents, natural fabrics e.g. cotton.) * Avoid allergens. * Keep the area cool and dry. * Sedating antihistamine can reduce itching and aid sleep. * Liberal emollients should be applied frequently. * Psychological support may be needed.
564
What are some emollients that can be used to treat Eczema?
**Thin Emollients:** * E45 * Diprobase cream * Oilatum cream * Aveeno cream * Cetraben cream * Epaderm cream **Thick Greasy Emollients:** * 50:50 Ointment * Hydromol ointment * Diprobase ointment * Cetraben ointment * Epaderm ointment
565
What are some side effects to using topical steroids to treat Eczema?
* Thinning of the skin which can make it more prone to flares, bruising and tearing * Cause Telangiectasia (enlarged blood vessels)
566
What are some complications of Eczema?
**Stratching:** * Poor sleep * Poor Mood * Skin breakdown leading to infection **Psycho-social:** * Insecurities surrounding skin appearance * Normal ADLs disrupted due to skin condition such as avoiding swimming **Eczema Herpeticum**
567
How does Eczema cause bacterial infection?
**Bacterial Infection:** * Staph aureus is the most common organism that can enter through breaks in the skins protective barrier * Treatment with oral Abx (flucloxacillin)
568
What is Eczema Herpeticum?
Eczema herpeticum is a viral skin infection caused by the herpes simplex virus (HSV) or varicella zoster virus (VZV). It was previously known as Kaposi varicelliform eruption It usually occurs in a patient with a pre-existing skin condition, such as atopic eczema or dermatitis, where the virus is able to enter the skin and cause an infection.
569
What is the Presentation of Eczema Herpeticum?
A typical presentation is a patient who suffers with eczema that has developed a widespread, painful, vesicular rash with systemic symptoms such as fever, lethargy, irritability and reduced oral intake. There will usually be lymphadenopathy (swollen lymph nodes).
570
What are the characteristics of the Rash seen in Eczema Herpeticum?
* The rash is usually widespread and can affect any area of the body. * It is erythematous, painful and sometimes itchy, with vesicles containing pus. * The vesicles appear as lots of individual spots containing fluid. * After they burst, they leave small punched-out ulcers with a red base.
571
What is the management of Eczema Herpeticum?
* **Viral swabs** of the vesicles can be used to confirm the diagnosis, although treatment is usually started based on the clinical appearance. * **Treatment is with aciclovir.** A mild or moderate case may be treated with oral aciclovir, whereas more severe cases may require IV aciclovir.
572
Define Stevens-Johnson Syndrome (SJS)?
Stevens-Johnson syndrome (SJS) is an immune-complex-mediated hypersensitivity disorder. It ranges from mild to severe forms, part of a spectrum that includes toxic epidermal necrolysis (TEN) at its most severe end where there is blistering and shedding of the top layer of skin.
573
What is the Epidemiology of Stevens-Johnson Syndrome?
* Rare disorder * Affects both genders and all age groups
574
What is the Pathophysiology of SJS?
Hypersensitivity often due to adverse drug reactions. Infectious agents may also place a role particularly viral pathogens such as HSV, EBV and HIV.
575
What are some major causes of Stevens-Johnsen Syndrome?
**Medications** * Anti-epileptics * Antibiotics (particularly **Sulphonamides**) * Allopurinol * NSAIDs **Infections** * Herpes simplex * Mycoplasma pneumonia * Epstein-Barr Virus * Cytomegalovirus * HIV
576
What are the clinical features of SJS?
* Often **presents within a week of medication intake** * **Initially with non-specific symptoms** such as cough, cold, fever, and sore throat. * **Erythematous macules**, later becoming target-shaped, appear after a few days. * A few days later, **flaccid blisters** develop and the Nikolsky sign is positive. * Mucosal ulceration is seen in at least two of the following: conjunctiva, mouth, urethra, pharynx, or gastrointestinal tract. **SJS affects less than 10% of the body surface, in contrast to Toxic Epidermal Necrolysis (TEN), which involves more than 30% of the skin.**
577
What are some differential diagnoses for SJS?
* **Erythema Multiforme:** Characterized by target lesions, typically on the hands and feet, and less severe mucosal involvement. * **Drug Rash with Eosinophilia and Systemic Symptoms (DRESS):** Presents with fever, rash, and internal organ involvement, often with a delay of 2-6 weeks after drug exposure. * **Acute Generalized Exanthematous Pustulosis (AGEP):** Noted for the rapid development of numerous small non-follicular sterile pustules on a background of oedematous erythema.
578
What condition does this show?
Stevens-Johnson Syndrome
579
What are the investigations for Stevens-Johnson Syndrome?
**Clinical Diagnosis** * Can be supported by a **skin biopsy** that shows **necrotic keratinocytes and sparse lymphocytic infiltrate**
580
What is the management of SJS?
**Medical Emergency** so should be admitted to dermatology or burns unit * **Supportive care** is essential focusing on skin care and prevention of ocular complications * Treatment may include steroids, immunoglobulins and immunosuppressant medications * Antiseptics, analgesia and ophthalmology input
581
What are some complications of SJS?
* **Secondary infection:** The breaks in the skin can lead to secondary bacterial infection, cellulitis and sepsis. * **Permanent skin damage:** Skin involvement can lead to scarring and damage to skin, hair, nails, lungs and genitals. * **Visual complications:** Depending on the severity, eye involvement can range from sore eyes to severe scarring and blindness.
582
Define Allergic Rhinitis?
A condition caused by **an IgE-mediated type 1 hypersensitivity reaction.** Environmental allergens cause an allergic inflammatory response in the **nasal mucosa.**
583
What is the epidemiology of Allergic Rhinitis?
* A common condition * Often present in individuals with immune disorders. * Often presents with Atopy and asthma.
584
What are the different classifications of Allergic Rhinits?
* **Seasonal** for example hay fever * **Perennial (year round)** for example house dust mite allergy * **Occupational** associated with the school or work environment
585
What are the clinical features of Allergic Rhinitis?
* Runny, blocked and itchy nose * Sneezing * Itchy, red and swollen eyes * Associated with Personal or family history of **atopy conditions**
586
What are the investigations for allergic rhinitis?
**Clinical diagnosis** * Skin prick tests or Blood tests for IgE specific antibodies to identify the allergen.
587
What are some differential diagnoses for Allergic Rhinitis?
* **Sinusitis:** Facial pain or pressure, nasal stuffiness, nasal discharge, loss of smell, and cough * **Nasal Polyps:** Chronic sinusitis, runny nose, postnasal drip, decreased or absent sense of smell, facial pain or headache, snoring, and frequent nosebleeds * **Deviated Nasal Septum:** Nosebleeds, facial pain, headache, postnasal drip, loud breathing and snoring during sleep * **Common Cold:** Sore throat, cough, congestion, body aches, and fatigue
588
What are some triggers for Allergic Rhinitis?
* **Tree pollen or grass** allergy leads to seasonal symptoms (hay fever) * **House dust mites** and pets can lead to persistent symptoms, often worse in dusty rooms at night. Pillows can be full of house dust mites. * **Pets** can lead to persistent symptoms when the pet or their hair, skin or saliva is present * **Other allergens** lead to symptoms after exposure (e.g. mould)
589
What is the management for Allergic Rhinitis?
* **Avoid Triggers** * **Oral Antihistamines** taken prior to exposure to reduce allergic symptoms * **nasal irrigation** with saline * **Nasal Corticosteroids** if initial measures are ineffective.
590
Give examples of some non-sedating and Sedating antihistamines
**Non-Sedating:** * Cetirizine * Loratadine * Fexofenadine **Sedating:** * Chlorphenamine (Piriton) * Promethazine
591
Define Urticaria?
* Also known as hives. They are small itchy lumps that appear on the skin. * They may be associated with a patchy erythematous rash. This can be localised to a specific area or widespread. * They may be associated with angioedema and flushing of the skin.
592
What is the pathophysiology of Urticaria?
Urticaria are caused the release of histamine and other pro-inflammatory chemicals by mast cells in the skin. This may be part of an allergic reaction in acute urticaria or an autoimmune reaction in chronic idiopathic urticaria.
593
What are the causes of Acute Urticaria?
** typically triggered by something that stimulates the mast cells to release histamine. This may be:** * Allergies to food, medications or animals * Contact with chemicals, latex or stinging nettles * Medications * Viral infections * Insect bites * Dermatographism (rubbing of the skin)
594
What are the causes of Chronic Urticaria?
**Chronic Urticaria i an autoimmune condition and is subclassified depending on the cause:** * Chronic Idiopathic Urticaria * Chronic Inducible Urticaria * Autoimmune Urticaria
595
What is Chronic Idiopathic Urticaria?
Recurrent episodes of chronic urticaria without a clear underlying cause or trigger
596
What are some causes of Chronic Inducible Urticaria?
* Sunlight * Temperature change * Exercise * Strong emotions * Hot or cold weather * Pressure (dermatographism)
597
What is Autoimmune Urticaria?
Chronic Urticaria associated with an underlying autoimmune condition such as SLE
598
What is the management of Urticaria?
**Antihistamines:** Usually Fexofenadine * Oral steroids may be considered short course for severe flares. **Very Problematic cases:** * Anti-leukotrienes (Montelukast) * Omalizumab targets IgE * Ciclosporin
599
Define Anaphylaxis?
An acute and **severe type 1 hypersensitivity reaction. It is a systemic, potentially life-threatening condition** that involves multiple organ systems due to the release of mediators from mast cells and basophils.
600
What are some common triggers of Anaphylaxis?
* **Animals:** Insect stings, animal dander * **Foods:** Nuts, peanuts, shellfish, fish, eggs, milk * **Medications:** Antibiotics, IV contrast media, NSAIDs
601
What is the pathophysiology of Anaphylaxis?
* Type 1 hypersensitivity reaction. * Immunoglobulin E (IgE) stimulates mast cells to rapidly release histamine and other pro-inflammatory chemicals. * This is called mast cell degranulation. * This causes a rapid onset of symptoms, with airway, breathing and/or circulation compromise. * The key feature that differentiates anaphylaxis from a non-anaphylactic allergic reaction is compromise of the airway, breathing or circulation.
602
What is the presentation of Anaphylaxis?
**Rapid Onset Allergic Symptoms:** * Urticaria * Itching * Angio-oedema, with swelling around lips and eyes * Abdominal pain **Additional symptoms that indicate anaphylaxis are:** * Shortness of breath * Wheeze * Swelling of the larynx, causing stridor * Tachycardia * Light headedness * Collapse
603
What are the clinical features of Anaphylaxis based on body system?
* **Airway:** Swollen lips/tongue, sneezing * **Respiratory:** Wheezing, shortness of breath * **Cardiovascular:** Tachycardia, hypotension/shock, angioedema * **Gastrointestinal:** Abdominal pain, diarrhoea, vomiting * **Dermatological:** Urticaria, pruritis, flushed skin
604
What are some differential diagnoses for Anaphylaxis?
* **Vasovagal reaction:** Characterized by hypotension, bradycardia, pallor, diaphoresis, and nausea. * **Panic attack:** Shortness of breath, tachycardia, sweating, tremor, and feeling of impending doom, but lacks skin involvement. * **Asthma exacerbation:** Primarily respiratory symptoms such as wheezing, cough, and breathlessness, without systemic involvement. * **Carcinoid syndrome:** Flushing, diarrhoea, abdominal pain, and wheezing due to serotonin release but generally has a more chronic course.
605
What are the investigations for Anaphylaxis?
**Often Clinical Diagnosis** * Measurement of **serum levels of mast cell tryptase,** which rises within an hour **(max within 6 hours)** of onset and can confirm the diagnosis.
606
What is the management for Anaphylaxis?
**ABCDE Approach:** **A**irway: Secure the airway **B**reathing: Provide oxygen if required. Salbutamol can help with wheezing. **C**irculation: Provide an IV bolus of fluids **D**isability: Lie the patient flat to improve cerebral perfusion **E**xposure: Look for flushing, urticaria and angio-oedema Once a diagnosis of anaphylaxis is established, there are three medications given to treat the reaction: * **Immediate Intramuscular adrenaline 500mcg**, repeated after 5 minutes if required * **Antihistamines**, such as oral chlorphenamine or cetirizine * **Steroids**, usually intravenous hydrocortisone **Post crisis:** Patients should be monitored for 6-12 hours after initial presentation in case of a rebound episode.
607
What may be for the patient after their first anaphylactic episode?
* Counselling on the use of Adrenaline Auto-Injectors (Epipen) * Allergy avoidance * Supply of two auto-injectors * Education for family and child for BLS
608
Define Nappy Rash?
Contact dermatitis in the nappy area. It is caused by friction between the skin and nappy and contact with urine and faeces in a dirty nappy.
609
What is the epidemiology of Nappy Rash?
* Most babies will get nappy rash at some point * Most common between ages of 9-12 months * Breakdown in the skin and the warm moist environment can lead to added infection with Candida or bacteria
610
What are some risk factors for Nappy Rash?
* Delayed changing of nappies * Irritant soap products and vigorous cleaning * Certain types of nappies (poorly absorbent ones) * Diarrhoea * Oral antibiotics predispose to candida infection * Pre-term infants
611
What is the presentation of Nappy Rash?
* Nappy rash present with sore, red, inflamed skin in the nappy area. * The rash appears in individual patches on exposure areas of the skin that come in contact with the nappy. * It tends to **spare the skin creases**, meaning the creases in the groin are healthy. * Nappy rash is uncomfortable, may be itchy and the infant may be distressed. * Long standing rash can lead to erosions and ulceration
612
What are the signs of candidal infection than simple nappy rash?
* Rash extending into the skin folds * Larger red macules * Well demarcated scaly border * Circular pattern to the rash spreading outwards, similar to ringworm * Satellite lesions, which are small similar patches of rash or pustules near the main rash * **Oral thrush**
613
What is the management for Nappy Rash?
* Switching to highly absorbent nappies (disposable gel matrix nappies) * Change the nappy and clean the skin as soon as possible after wetting or soiling * Use water or gentle alcohol free products for cleaning the nappy area * Ensure the nappy area is dry before replacing the nappy * Maximise time not wearing a nappy * Infection with candida or bacteria warrants treatment with anti-fungal cream
614
What are some complications of Nappy Rash?
* Candida infection * Cellulitis * Jacquet’s erosive diaper dermatitis * Perianal pseudoverrucous papules and nodules
615
What is a Non-blanching rash?
Non-blanching rashes are caused by bleeding under the skin when when pressed do not go white. **Petechiae** are small (< 3mm), non blanching, red spots on the skin caused by burst capillaries. **Purpura** are larger (3 – 10mm) non-blanching, red-purple, macules or papules created by leaking of blood from vessels under the skin.
616
What is the most concerning differential diagnosis in an infant with a non-blanching rash?
Meningococcal septicaemia
617
What are some differential diagnoses for Non-blanching rashes in children?
* **Meningococcal Septicaemia** * Henoch Schonlein Puprura * Idiopathic thrombocytopenic purpura * Acute leukaemia * Haemolytic Uraemic Syndrome * Trauma * Viral illness
618
What is the management of a Non-blanching rash?
**Needs immediate investigation** due to the risk of meningococcal septicaemia
619
What are some causes of Fever and rash in children?
* Septicaemia * Slapped cheek syndrome (Fifth Disease or Parvovirus B19) * Hand, foot and mouth disease * Scarlet fever * Measles * Urticaria (hives) * Chickenpox * Roseola * Rubella
620
What are the clinical features of septicaemia?
Rapid development of a non-blanching purpuric skin rash, lethargy, headache, fever, rigors, vomiting.
621
What are the clinical features of Slapped Cheek Syndrome rash?
**Slapped cheek syndrome:** Rash on both cheeks, fever, upper respiratory tract infection symptoms.
622
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.
623
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.
624
What are the clinical features of the Measles rash?
Erythematous, blanching maculopapular rash, fever, cough, runny nose, conjunctivitis, Koplik spots.
625
What are the clinical features of the Urticaria rash?
Raised, itchy red rashes, usually not accompanied by fever.
626
What are the clinical features of the Chicken Pox rash?
Maculopapular vesicular rash that crusts over and forms blisters.
627
What are the clinical features of the Roseola rash?
Lace-like red rash across the whole body, high fever.
628
What are the clinical features of the Rubella rash?
Rash that starts on the head and spreads to the trunk, postauricular lymphadenopathy.
629
What are some investigations for Rashes in children?
* Blood tests (CBC, Coagulation profile) * Blood cultures * Viral serology * Skin biopsy **The specific tests would depend on the child's clinical presentation and suspected diagnosis.**
630
What is the management of these rashes: * Septicaemia * Slapped Cheek Syndrome * Hand Foot and Mouth Disease * Scarlet Fever * Measles * Urticaria * Chicken Pox * Roseola * Rubella
* **Septicaemia:** Immediate broad-spectrum IV antibiotics, notify a senior paediatrician. * **Slapped cheek syndrome:** Generally self-limiting, symptomatic treatment. * **Hand, foot, and mouth disease:** Generally self-limiting, symptomatic treatment. * **Scarlet fever:** Treated with antibiotics, usually phenoxymethylpenicillin. * **Measles:** Supportive management, immunisation is crucial. * **Urticaria:** Antihistamines and/or steroids, identify and avoid trigger. * **Chickenpox:** Supportive treatment, antiviral treatment for high-risk groups. * **Roseola:** Self-limiting, supportive management. * **Rubella:** Self-limiting, supportive management, immunisation for prevention.
631
Define Kawasaki Disease?
Also known as **mucocutaneous lymph node syndrome.** It is a systemic, **medium-sized vessel vasculitis.**
632
What is the Epidemiology of Kawasaki Disease?
* Affects young children **Typically under 5 years** * More common in Asian children particularly **Japanese and Korean** * More common in boys
633
What is the Aetiology of Kawasaki disease?
**Not understood** but thought to be multifactorial. No clear cause or trigger
634
# CREAM What are the clinical features of Kawasaki disease?
**Persistent High Fever above 39 degrees for more than 5 days** * **C**onjunctivitis: Bilateral and non-exudative * **R**ash: Widespread Erythematous maculopapular rash and desquamation (skin peeling) on palms and soles * **E**rythema/oedema of hands and feed * **A**denopathy: cervical, commonly unilateral and non-tender * **M**ucosal involvement: Strawberry tongue and oral fissues.
635
What are some differential diagnoses for Kawasaki disease?
* **Scarlet Fever:** Characterized by a high fever, "strawberry tongue," and a red rash with a sandpaper-like texture. * **Measles:** Presents with fever, cough, coryza, conjunctivitis, and a maculopapular rash. * **Drug Reactions:** These can cause similar skin manifestations and fever, but are often associated with the onset of a new medication. * **Juvenile Rheumatoid Arthritis:** This can cause fever and rash, as well as joint pain and swelling. * **Toxic Shock Syndrome:** This typically includes fever, rash, hypotension, and multisystem involvement.
636
What are the investigations for Kawasaki disease?
* **Full blood count** can show anaemia, leukocytosis and thrombocytosis * **Liver function tests** can show hypoalbuminemia and elevated liver enzymes * **Inflammatory markers** (particularly ESR) are raised * **Urinalysis** can show raised white blood cells without infection * **Echocardiogram** can demonstrate coronary artery pathology
637
What is the disease course of Kawasaki disease?
**Acute phase:** * The child is most unwell with the fever, rash and lymphadenopathy. * This lasts 1 – 2 weeks. **Subacute phase:** * The acute symptoms settle, the desquamation and arthralgia occur and there is a risk of coronary artery aneurysms forming. * This lasts 2 – 4 weeks. **Convalescent stage:** * The remaining symptoms settle, the blood tests slowly return to normal and the coronary aneurysms may regress. * This last 2 – 4 weeks.
638
What is the management of Kawasaki disease?
* **High dose aspirin** to reduce the risk of thrombosis * **IV immunoglobulins** to reduce the risk of coronary artery aneurysms * **Regular Echocardiograms** to monitor for coronary artery aneurysms **Public health should be informed**
639
What is a major complication of Kawasaki disease?
Coronary Artery Aneurysms
640
Why is the fact that aspirin is used in the management of Kawasaki disease odd in children?
Aspirin is rarely used in children due to the risk of **Reye's Syndrome**
641
Define Mealses?
A highly contagious disease caused by the Measles morbillivirus. It is transmitted via droplets from the nose, mouth, or throat of infected persons.
642
What is the epidemiology of Measles?
* Most common in unvaccinated children * Prevalent in areas with low vaccination rates
643
What is the Aetiology of Measles?
* **Measles Morbillivirus** * Single stranded Enveloped RNA virus * Transmitted via respiratory droplets or direct contact with nasal or throat secretions of infected individuals
644
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.**
645
What are some differential diagnoses for Measles?
* **Rubella:** Similar to measles but often milder. The rash typically begins on the face and spreads to the rest of the body. Enlarged lymph nodes, particularly behind the ears and at the back of the skull, are common. * **Roseola:** Characterized by a sudden high fever followed by a rash once the fever subsides. The rash usually starts on the chest, back, and abdomen, spreading to the neck and arms. * **Scarlet Fever:** Presents with a characteristic sandpaper-like rash, a high fever, and a sore throat. The tongue may also become red and bumpy, giving it a 'strawberry' appearance.
646
What are the investigations for Measles?
**1st:** * Measles-specific IgM and IgG serology (ELISA), * Most sensitive 3-14 days after onset of the rash. **2nd:** * Measles RNA detection by PCR, best for swabs taken 1-3 days after rash onset.
647
What is the management of Measles?
* Supportive care, usually involving antipyretics. * **Vitamin A administration for all children under 2 years.** * **Ribavirin** may reduce the duration of symptoms but is not routinely recommended due to the potential side effects. * Avoid school for at least 5 days after initial onset of rash ** Notify Public Health**
648
What are some complications of Measles?
* **Otitis Media** * Pneumonia * Febrile Convulsions * Encephalitis
649
Define Chickenpox?
An acute infectious disease caused by the varicella-zoster virus (VZV), a member of the human herpes virus family. This highly contagious illness, predominantly seen in children, is characterised by a vesicular rash, mild fever, and malaise.
650
Why is Chickenpox dangerous in Pregnancy?
* More severe cases in the mother, such as varicella pneumonitis, hepatitis or encephalitis * Foetal varicella syndrome * Severe neonatal varicella infection (if infected around delivery)
651
What is the Epidemiology of Chickenpox?
* Very common disease globally * Majority of cases occur in children aged 1-9 years * Highly contagious with 90% secondary infection rate in susceptible household contacts * Usually mild in children but can be severe in adults or immunocompromised.
652
What is the Pathophysiology of Chickenpox?
The aetiology of chickenpox is the varicella virus, specifically human herpes virus 3 (HHV3). The virus is airborne and spreads through direct contact with the rash or by breathing in particles from an infected person's sneezes or coughs.
653
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.
654
What are some differential diagnoses for Chickenpox?
* **Herpes simplex:** Characterised by painful, grouped vesicles on an erythematous base, usually around the mouth or genital area. * **Hand, foot, and mouth disease:** Presents with a rash on the hands, feet, and inside the mouth, often alongside fever and malaise. * **Scabies:** Manifests as intense itching, especially at night, and a pimple-like rash.
655
What are the investigations for Chickenpox?
**Clinical Diagnosis**
656
What is the management for Chickenpox?
**Conservative Management:** * Symptom control: Analgesics, antipyretics, wear long clothing and cut nails to prevent scratching * Avoid School until lesions have crusted over * Immunocompromised may be give IV Aciclovir
657
What can be done for mothers who are unsure if they are immune to Chickenpox?
IgG levels for VZV can be tested. If positive this indicates immunity, If negative then women can be offered the varicella vaccine
658
What is the treatment for Chickenpox in pregnancy?
Treated with Oral Aciclovir if they present within 24 hours of rash onset and are more than 20 weeks gestation
659
What are some complications of Chickenpox?
* Secondary bacterial skin infections due to scratching * Pneumonia (more common in adults) * Encephalitis (rare) * Reye's syndrome (a severe complication, primarily in children) * Congenital varicella syndrome (if infection occurs during early pregnancy) * Reactivation of the virus as herpes zoster (shingles) later in life
660
Define Rubella?
Also known as German measles, is a contagious viral illness. It is caused by the **rubella togavirus** and transmitted through respiratory droplets.
661
What is the epidemiology of Rubella?
* Less common due to widespread vaccination * Often occurs in winter and spring * Highest prevalence in the unvaccinated
662
What is the aetiology of Rubella?
**Rubella togavirus**, which is transmitted via respiratory droplets or aerosols. Children are routinely vaccinated for rubella as part of the **MMR Vaccine** starting at 12 months of age
663
What may occur if Rubella infection occurs in the first 20 weeks of pregnancy?
**Congenital Rubella Syndrome** * Congenital Deafness * Congenital Cataracts * Congenital heart disease (PDA and Pulmonary Stenosis) * Learning disability
664
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
665
What are some differential diagnoses for Rubella?
* **Measles:** Fever, cough, conjunctivitis, coryza, Koplik spots, and a rash that typically begins at the hairline and moves downwards to involve the entire body, including the limbs. * **Scarlet fever:** Sudden onset of fever, sore throat, "strawberry" tongue, and a fine, sandpaper-like rash, most often on the neck, underarm, and groin. * **Fifth disease (Erythema infectiosum):** A mild illness that might cause a "slapped cheek" rash on the face and a lacy red rash on the trunk and limbs, often after a few days of mild fever or cold-like symptoms.
666
What are the investigations for Rubella?
**Serological Testing** * Rubella specific IgM or a rise in Rubella specific IgG
667
What is the management of Rubella?
**supportive Treatment:** * Analgesics and Anti-pyretics
668
Why should pregnant women not receive the MMR vaccine if they are not vaccinated against rubella?
It is a live vaccine and therefore may induce Congenital Rubella Syndrome. They should be vaccinated after giving birth
669
What are some complications for Rubella?
**Serious risk to unvaccinated pregnant women** for Congenital Rubella Syndrome causing: * Cataracts * Deafness * Patent ductus arteriosus * Brain damage
670
Define Diphtheria?
A toxin-mediated bacterial disease caused by Corynebacterium diphtheriae.
671
What are some risk factors for Diphtheria?
* Unvaccinated individuals * Inadequately vaccinated individuals * Exposure to an infected individual * Travel from endemic areas
672
What is the Epidemiology of Diphtheria?
Generally been eradicated in the UK due to vaccination
673
What is the pathophysiology of Diphtheria?
Infection which causes local disease with membrane formation affecting the nose, pharynx or larynx or systemic disease with myocarditis and neurological manifestations.
674
What are the clinical features of Diphteria?
* Child with severe sore throat and fever for 2 days * Lymphadenopathy in neck * Rapid breathing may present as Stridor * Thick greyish membrane on tonsils
675
What are the investigations for Diphteria?
**Nasal swabs and Bacterial Culture and Microscopy** * Blood agar containing potassium tellurite (**Hoyle's/Tinsdale Media**) * Shows **irregular Gram positive Rods** * Staining shows **Metachromatic granules** **Elk Test for toxins** **PCR**
676
What is the management for Diphtheria?
* Diphtheria Antitoxin * Erythromycin
677
Define Staphylococcal Scalded Skin Syndrome (SSSS)?
A severe desquamating rash that primarily affects infants caused by a type of Staphylococcal aureus bacteria that produces **epidermolytic toxins**.
678
What is the epidemiology of SSSS?
* Affects infants **particularly under 5** due to their immature renal clearance * Can occur in adults with renal insufficiency or immune compromise
679
What is the pathophysiology of SSSS?
* SSSS occurs due to the production of an **exfoliative exotoxin** by Staphylococcus aureus. * This exotoxin splits the epidermis in the granular cell layer, **specifically targeting desmoglein 1.** * It is on the same spectrum as impetigo, with the layer of skin involved being the same.
680
What is the clinical presentation of SSSS?
SSSS usually starts with generalised patches of erythema on the skin. Then the skin looks thin and wrinkled. This is followed by the formation of fluid filled blisters called bullae, which burst and leave very sore, erythematous skin below. This has a similar appearance to a burn or scald **Nikolsky sign is where very gentle rubbing of the skin causes it to peel away. This is positive in SSSS.** Systemic symptoms include fever, irritability, lethargy and dehydration. If untreated it can lead to sepsis and potentially death.
681
what are some differential diagnoses for SSSS?
* **Toxic Epidermal Necrolysis (TEN):** manifests with widespread erythema and necrosis, leading to detachment of the epidermis. It involves mucous membranes, which differentiates it from SSSS * **Pemphigus vulgaris:** characterised by flaccid blisters and erosions on the skin and mucous membranes; Nikolsky sign is also positive * **Bullous Impetigo:** typically presents with localized bullae filled with pus, often with surrounding erythema and tenderness
682
What are the investigations for SSSS?
**Clinical Diagnosis** * Skin biopsy can help differentiate it from other conditions such as TEN
683
What is the management for SSSS?
**IV Antibiotics** **Fluid and electrolyte balance** to prevent dehyration
684
Define Polio?
Poliomyelitis is caused by poliovirus infection which results in a minor GI illness in 95% of cases. Occasionally it can result in the major illness Acute flaccid Paralysis.
685
What are some risk factors for Polio?
* **Unvaccinated** * Poor sanitation * Poverty * Endemic area
686
What are the clinical features of polio?
* **Asymptomatic in 95% of cases** * Minor GI illness * Major Acute Flaccid Paralysis in less than 5% of cases
687
What is the management of Polio?
Diagnosed with Viral culture from stool or CSF analysis. Antibodies against poliovirus **No cure for polio** so supportive treatment
688
Define Tuberculosis (TB)?
A chronic caseating granulomatous disease caused by Mycobacterium tuberculosis.
689
What is the Epidemiology of TB?
* 1/3 of the global population has latent TB infection * More than 95% of deaths due to TB occur in low- and middle-income countries, where it is a leading cause of mortality * Lifetime risk of reactivating TB is 5–15%; in the setting of HIV, it is 5–15% per year * HIV testing is mandatory in TB as it increases the risks of extrapulmonary TB and the difficulty treating it * The incidence of TB in the UK is high compared with other Western countries (13.9/100,000 in 2012)
690
How is TB Transmitted?
TB is transmitted by inhalation of droplets infected with M. tuberculosis. These droplets are produced by infected patients when they cough – infective organisms can survive for long periods of time in the environment.
691
What are the bacteria that can cause TB?
MTC organisms = TB causing: M. tuberculosis M. africanum M. microtis M. bovis (from unpasteurised milk)
692
What is the morphology of M. TB?
* **Gram Positive Rod Bacilli** * Non motile + non spore forming * **Mycolic acid capsule: Acid fast staining (w/ Zeihl Nieelsen)** Resistant to phagocytic killing. Slow growing (15-20 hrs)
693
What are the risk factors for TB infection?
* Close contact with active tuberculosis (e.g., a household member) * Immigrants from areas with high tuberculosis prevalence * People with relatives or close contacts from countries with a high rate of TB * Immunocompromised (e.g., HIV or immunosuppressant medications) * Malnutrition, homelessness, drug users, smokers and alcoholics
694
What is the disease course of TB?
**Immediate clearance** of the bacteria (in most cases) **Primary active tuberculosis** (active infection after exposure) **Latent tuberculosis** (presence of the bacteria without being symptomatic or contagious) **Secondary tuberculosis** (reactivation of latent tuberculosis to active infection) **Miliary tuberculosis** (When the immune system cannot control the infection, disseminated and severe disease can develop)
695
What is Latent TB?
Latent tuberculosis is present when the immune system encapsulates the bacteria and stops the progression of the disease. Patients with latent tuberculosis have no symptoms and cannot spread the bacteria. Most otherwise healthy patients with latent tuberculosis never develop an active infection. When latent tuberculosis reactivates, and an infection develops, usually due to immunosuppression, this is called secondary tuberculosis.
696
What are some areas of Extrapulmonary TB?
* Lymph nodes * Pleura * Central nervous system * Pericardium * Gastrointestinal system * Genitourinary system * Bones and joints * Skin (cutaneous tuberculosis)
697
What is the common presentation of TB?
**Subacute to chronic in onset** Symptoms depend on the main site of infection, but are usually accompanied by: * **night sweats, Fever, Weight loss** * Chronic cough (>3 weeks) productive of purulent sputum * Haemoptysis (coughing up blood) * Lethargy * Lymphadenopathy * **erythema nodosum** * Spinal Tuberculosis (Spinal pain)
698
What are the investigations for TB?
* **Sputum samples for MC&S x3:** Take 6 weeks to grow. Zeilh Neelsen staining for Acid Fast Bacilli * **Chest X-ray** shows patchy nodular consolidation with cavitation. Pleural effusions and hilar lymphadenopathy. May have millet seeds in miliary TB. * **Cultures:** Sputum culture, Mycobacterium blood cultures and Lymph node aspiration or biopsy for caseating granulomas. * Nucleic Acid Amplification Test (NAAT) * **Immune Response Tests:** Mantoux test and Interferon Gamma Release Assay (IGRA). Cannot tell the difference between latent or active * HIV Test
699
What are some signs of TB?
Auscultation - often normal (may have crackles) Consolidation in lung Lung Collapse Clubbing
700
What is the Mantoux Test?
The Mantoux test involves injecting tuberculin into the intradermal space on the forearm. Tuberculin is a collection of tuberculosis proteins isolated from the bacteria. It does not contain any live bacteria. The infection creates a bleb under the skin. After 72 hours, the test is “read”. This involves measuring the induration of the skin at the injection site. An induration of 5mm or more is considered a positive result. it will have a positive result from the BCG vaccine
701
What is the management for Active TB?
* Rifampicin: **6 months** * Isoniazid: **6 months** (+ Pyridoxine) * Pyrazinamide: **2 months** * Ethambutol: **2 months** **Treatment is for 12 months in CNS, pericarditis, Spinal TB.**
702
what is the management for Latent TB?
Isoniazid and rifampicin for 3 months Isoniazid for 6 months ** Plus Pyridoxine to prevent peripheral neuropathy caused by Isoniazid**
703
What are some additional management steps other than pharmacology for TB?
* Testing for other infectious diseases (e.g., HIV, hepatitis B and hepatitis C) * Testing contacts for tuberculosis (Contact tracing) * Notifying UK Health Security Agency (UKHSA) of suspected cases * Isolating patients with active tuberculosis to prevent spread (usually for at least 2 weeks of treatment) * A specialist MDT guides management and follow-up * Individualised regimes are required for multidrug‑resistant tuberculosis and extrapulmonary disease
704
What are the side effects of Rifampicin?
* Liver toxicity * Hepatic enzyme (p450) inducer – must check interaction with other medications * **Turns bodily fluids a red/orange colour** * Haemolysis
705
What are the side effects of Isoniazid?
* Peripheral neuropathy (pyridoxine is given to prevent this) * Liver toxicity
706
What are the side effects of Pyrazinamide?
* Liver toxicity * Athralgia * Hyperuricaemia causing **Gout**
707
What are the side effects of Ethambutol?
* Visual disturbance (colour blindness, loss of acuity etc.) * Avoid in chronic kidney disease
708
What vaccine is available for TB?
Bacillus Calmette Guerin (BCG) * A live attenuated vaccine of Mycobacterium bovis bacteria. * Before vaccination the Mantoux test is done and the vaccine is only given if the test is negative.
709
Define Meningitis?
Inflammation of the meninges from both infective and non-infective causes. This is a notifiable condition to PHE
710
What are the different infective causes of Meningitis?
Viral: **Enterovirus (coxsackie) HSV2 VZV** Bacterial: **N. Meningitidis S. pneumonia** Fungal: Cryptococcus Neoformans (primary in the immunosuppressed population) Parasitic: Amoeba, Toxoplasma Gondii
711
What is the most common cause of meningitis?
Viral infection (Most commonly **enteroviruses**) More common but less severe than bacterial causes.
712
What are the main risk factors for meningitis?
Extremes of age (Infant/elderly) Immunocompromised Pregnancy Travel Crowded environment - barracks/uni Non-vaccinated
713
What vaccines are available for meningitis coverage?
N. Meningitidis - Men B + Men C + Men ACWY S. pneumoniae - PCV Vaccine
714
What are the symptoms of Meningitis?
Meningism: Headache, Fever, Neck stiffness Non-Blanching Purpuric Rash Nausea + Vomiting Seizures Photophobia Purpuric Rash - Bacterial Meningitis Non-Blanching Purpuric Rash - Meningococcal Septicaemia
715
What are the clinical signs of meningitis?
**Kernig's Sign:** When the hip is flexed and the knee is at 90°, extension of the knee results in pain **Brudzinski Sign:** Severe neck stiffness causes the hips and knees to flex when the neck is flexed
716
What are the primary investigations in meningitis?
**1st Line: Bloods** FBC - raised WCC CRP - raised Blood glucose - compared with CSF Blood culture - to determine viral/bacterial CT Head - Look for Brain Lesions/Abscesses/CIs for LP **Lumbar Puncture (LP) + CSF Analysis (Gold Standard)**
717
What are some contraindications for a lumbar puncture?
Raised ICP GCS <9 Focal Neurological signs
718
Where is a lumbar puncture usually taken from?
Between L3/L4 Since spinal cord ends L1/2
719
What are some non-infective causes of Meningitis?
* Malignancies such as leukemia, lymphoma, and other tumors * Chemical meningitis * Certain drugs, including NSAIDs and trimethoprim * Systemic inflammatory diseases such as sarcoidosis, * Systemic Lupus Erythematosus, Behcet's disease.
720
What is the most common cause of meningitis in neonates?
Group B Streptococcus - **Streptococcus Pyogenes** E.coli Strep. pneumonia Listeria
721
What does the Non-blanching Purpuric Rash indicate in Meningitis?
Bacterial Meningococcal Septicaemia: This rash indicates the infection has caused disseminated intravascular coagulopathy (DIC) and subcutaneous haemorrhages.
722
What is the presentation of Meningitis in Neonates?
Neonates and babies can present with very non-specific signs and symptoms: * Hypotonia * Poor feeding * Lethargy * Hypothermia * **Bulging Fontanelle.**
723
In Paediatric cases of fever and general unwellness what does NICE recommend as an important investigation?
A Lumber Puncture in all children: * Under 1 month presenting with fever * 1 to 3 months with fever and are unwell * Under 1 year with unexplained fever and other features of serious illness
724
What are some differential diagnoses for Meningitis?
* Encephalitis * Subarachnoid Haemorrhage * Brain Abscess * Sinusitis * Migraine
725
What would the results of CSF analysis be in Bacterial, Viral and Fungal Meningitis?
Fungal: * Lymphocytosis * Increased Protein Concentration * Decreased Glucose Concentration
726
What is the management of Bacterial Meningitis in the community?
STAT dose of **IM Benzylpenicillin** and immediate transfer to hospital In True Penicillin allergy then immediate transfer to hospital
727
What is the management of Bacterial Meningitis in the Hospital?
**Ideally perform a blood culture and a lumbar puncture prior to starting Abx unless the patient is acutely unwell.** Broad Spec Abx - Cover All Likely Organisms: 1st Line - **Ceftriaxone or Cefotaxime** (as they get through the BBB) WTIH OR AFTER **IV Dexamethasone** - Prevent neurological sequelae 2nd Line: Chloramphenicol Once Blood cultures have been done then can tailor Abx: Eg. IV Benzylpenicillin for N.Meningitidis
728
What antibiotics should be used to treat bacterial meningitis in children?
Under 3 Months: **Cefotaxime and Amoxicillin** (covers for listeria) Over 3 Months: **Ceftriaxone**
729
What Post Exposure Prophylaxis should be done in Meningitis cases?
Contact Tracing: * This risk is highest for people that have had **close prolonged contact within the 7 days prior to the onset of the illness.** * The risk decreases 7 days after exposure. Therefore, if no symptoms have developed 7 days after exposure they are unlikely to develop the illness. **A single dose of ciprofloxacin. It should be given as soon as possible and ideally within 24 hours of the initial diagnosis.**
730
What is the management of Viral Meningitis?
Usually only requires Supportive treatment **Acyclovir** can be used to treated suspected or confirmed HSV/VZV infections
731
What are some complications of Meningitis?
* Hearing loss is a key complication * Seizures and epilepsy * Cognitive impairment and learning disability * Memory loss * Cerebral palsy, with focal neurological deficits such as limb weakness or spasticity * Septic Shock and DIC * Coma and Death
732
Define Encephalitis?
Encephalitis is a pathological condition characterised by inflammation of the brain parenchyma, also known as the "encephalon".
733
What is the aetiology of Encephalitis?
* Predominantly Viral Infection * Bacterial and fungal pathogens can also lead to encephalitis (Rarely in the UK) * Autoimmune Encephalitis - NMDA receptor antibodies
734
What are the most common Viral causes of Encephalitis?
**Herpes Simplex Virus Type 1 (HSV-1)** Others: * HSV-2 * CMV * EBV * VZV * HIV
735
What are the clinical features of Encephalitis?
* **Altered Mental Status** * Fever * Flu-like prodromal illness * Seizures * Acute onset Focal neurological deficits * Headaches * Behavioural changes
736
What investigations are done in Encephalitis?
**Encephalitis should be suspected in any patient presenting with sudden onset behavioural changes, new seizures, and unexplained acute headache** * A routine panel of blood tests * Blood cultures and viral PCR * **Lumbar Puncture + Cerebrospinal fluid (CSF) analysis with viral PCR** * Consideration for malaria blood films in case of exposure risk **CNS Imaging: CT/MRI**
737
What may be seen on MRI in Encephalitis?
Temporal lobes affected Bilateral Multifocal Haemorrhage
738
What is the management of Encephalitis?
Intravenous antiviral medications are used to treat the suspected or confirmed underlying cause: **Aciclovir** treats herpes simplex virus (HSV) and varicella zoster virus (VZV) **Ganciclovir** treats cytomegalovirus (CMV) Supportive management of complications: Anti-convulsant for seizures
739
What are some side effects of Aciclovir?
**Common** * Generalised fatigue/malaise * Gastrointestinal disturbance * Photosensitivity and urticarial rash Others: * Acute renal failure * Haematological abnormalities * Hepatitis * Neurological reactions
740
What are some complications of Encephalitis?
**Lasting fatigue and prolonged recovery** Change in personality or mood Changes to memory and cognition Learning disability **Headaches** Chronic pain Movement disorders Sensory disturbance **Seizures** Hormonal imbalance
741
What is the most common cause of meningitis in children and adults?
**Neisseria meningitidis Streptococcus pneumonia**
742
What are some specific complications related to meningococcal meningitis?
* Risk of DIC * Risk of **Waterhouse Friedrichsen Syndrome**
743
What is Waterhouse Friedrichsen Syndrome?
Adrenal insufficiency caused by intra-adrenal haemorrhage as a result of meningococcal DIC
744
Define Parvovirus B19 Infection?
**Fifth Disease or Erythema infectiosum or Slapped Cheek Syndrome** A viral illness caused by Parvovirus B19
745
What is the epidemiology of Parvovirus B19?
* Most commonly occurs in Children * widespread and can occur in outbreaks, particularly in school settings. * It is most common in late winter and early spring.
746
What is the Aetiology of Parvovirus B19?
* Viral infection that specifically targets the erythroid progenitor cells and thus has certain haematological complications
747
What are the clinical features of Parvovirus B19 infection?
* Non-specific fever prodrome for a few days * 2-5 days later a diffuse bright red rah on cheeks appears (**Slapped cheeks**) * A few days later a Reticular lace like rash across the body * May also have diarrhoea and coryzal symptoms
748
What are some differential Diagnoses for Parvovirus B19 infection?
* **Rubella:** presents with a similar rash, but also includes lymphadenopathy and conjunctivitis * **Scarlet fever:** presents with a similar rash, but also includes a sore throat and a 'strawberry' tongue * **Roseola:** presents with a high fever followed by a rash, but the rash is typically non-pruritic and pink in colour
749
What are the investigations for Parvovirus B19 infection?
**Clinical diagnosis** * Serological testing may also be performed
750
What is the management for Parvovirus B19 infection?
**Supportive management** * Rest * Hydration * OTC remedies for fever and itching. * Rarely requiring hospital admission
751
What are some complications of Parvovirus B19 infection?
* **Red cell aplasia:** Parvovirus infection reduces erythropoiesis, which can precipitate severe anaemia and aplastic crisis in vulnerable groups like those with conditions like sickle cell anaemia and hereditary spherocytosis. * **Severe foetal anaemia:** Infection in the first half of pregnancy can cause severe foetal anaemia that can precipitate hydrops foetalis and subsequent miscarriage. * **Cardiomyopathy**
752
What are some complications of Parvovirus B19 infection in pregnancy?
Typically occur in 1st and 2nd trimesters. * Miscarriage or fetal death * Severe foetal anaemia * Hydrops fetalis (foetal heart failure) * Maternal pre-eclampsia-like syndrome
753
Define Impetigo?
A highly contagious superficial epidermal infection of the skin primarily caused by Staphylococcal and Streptococcal bacteria. It often causes a Golden crust lesion
754
What are the classifications of Impetigo?
**Non-Bullous** * Occurs around nose or mouth * Exudate from lesions forms a **Golden crust** * Does not cause systemic symptoms. **Bullous** * Always caused by **Staph aureus** * Epidermolytic toxins break down proteins in skin causing 1-2cm fluid vesicles that burst and form a golden crust. * Common to have systemic symptoms * May progress to Staphylococcal scalded skin syndrome
755
What is the epidemiology of Impetigo?
* Commonly occurs in infants and school aged children * Can affect individuals of any age * Bullous impetigo is more common in neonates and children under 2
756
What is the aetiology of Impetigo?
* **Staphylococcal aureus** * **Group A Strep (Strep pyogenes)** * Bacteria invade the skin through minor cutes, insect bites, or abrasions leading to infection.
757
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.
758
What are some differential diagnoses for impetigo?
* **Eczema Herpeticum:** Presents with rapid onset of painful, punched-out erosions with or without vesiculation on a background of atopic dermatitis. It may also exhibit systemic symptoms like fever and malaise. * **Herpes Simplex Virus (HSV) infection:** This may manifest as grouped vesicles on an erythematous base, usually accompanied by pain and itching. It can also cause systemic symptoms. * **Contact Dermatitis:** This involves erythematous, pruritic rash, usually in a pattern suggestive of a contact allergen. * **Tinea Corporis (Ringworm):** Exhibits annular erythematous scaly plaques, often with central clearing.
759
What are the investigations for Impetigo?
**Clinical Diagnosis** * May use a **skin swab** for necessary MC&S
760
What is the management of Impetigo?
**Localised non-bullous impetigo:** * Topical treatment with **hydrogen peroxide 1% cream** (apply two or three times daily for 5 days) is first line * If unsuitable, second line options include fusidic acid or mupirocin (if fusidic acid resistance) **Widespread non-bullous impetigo:** * Topical (fusidic acid/mupirocin) or oral antibiotics for 5 days, such as **flucloxacillin** * Clarithromycin (penicillin-allergic) or erythromycin (pregnancy) are alternatives **Bullous impetigo, or impetigo in those systemically unwell or at high risk of complications:** * Oral antibiotics as above for up to 7 days (**Flucloxacillin**) * Patients should avoid sharing items such as towels and should not attend school or work until they have completed 48 hours of antibiotic treatment to limit the spread of infection. **Refer to secondary care if:** * Suspected complications of impetigo (sepsis, glomerulonephritis, or deeper soft tissue infection) * The patient is immunocompromised and infection is widespread
761
What are some complications of Impetigo?
* Cellulitis if the infection gets deeper in the skin * Sepsis * Scarring * Post streptococcal glomerulonephritis * **Staphylococcus scalded skin syndrome** * Scarlet fever
762
Define Toxic Shock Syndrome (TSS)?
* A severe, life-threatening condition characterized by the sudden onset of shock, multi-organ failure, and rash. * It is an exotoxin-mediated multisystemic illness primarily caused by Streptococcus (usually group A), Staphylococcus aureus and MRSA.
763
What is the Epidemiology of Toxic Shock Syndrome?
* Historically associated with tampon use in menstruating women until manufacturers modified the absorbency levels. * Currently, it is attributed to various infections. * Adults are typically more affected than children.
764
What is the Aetiology of TSS?
* Caused by the exotoxin (Toxic Shock Toxin) produced by certain strains of bacteria, acting as a superantigen. This causes polyclonal T cell activation and massive cytokine release, notably IL-1 and TNF-alpha, leading to shock and multi-organ failure.
765
What are some risk factors for Toxic Shock Syndrome?
* Diabetes Mellitus * Staphylococcal cellulitis * Wounds (especially burns) * Alcoholism and intravenous drug use * HIV * Tampon use or gynaecological infections (though less common now)
766
What are the clinical features of Toxic Shock Syndrome?
* Early non-specific flu-like symptoms, possibly accompanied by nausea, vomiting, and diarrhoea. * Rapid progression to high fever and widespread macular rash, which often becomes erythrodermic, covering >90% of the body surface, including mucosal membranes. * Multiorgan involvement, featuring hypotension due to cardiac depression and confusion from encephalopathy.
767
What are the investigations for Toxic Shock Syndrome?
* Sepsis Six * Throat swabs, wound swabs, or swabs from the suspected initial infection site.
768
What are some differential diagnoses for Toxic Shock Syndrome?
* **Meningococcal septicemia:** Rapid onset, fever, limb pain, cold hands and feet, abnormal skin color. * **Stevens-Johnson Syndrome:** Fever, sore throat, fatigue, eye irritation, skin pain, red or purplish skin rash. * **Kawasaki disease:** Fever lasting more than five days, rash, swollen lymph nodes, swollen hands and feet, red eyes.
769
What is the management of Toxic Shock Syndrome?
**ABCDE Approach** * Immediate attention to the source of infection * **Antibiotics:** Generally clindamycin + Cephalosporin/carbapenem for broad spectrum coverage * **Surgical** debridement or drainage * Corticosteroids may improve survival rates
770
Define Scarlet Fever?
An infectious disease caused by toxin producing strains of Streptococcus Pyogenes (Group A Strep)
771
What is the epidemiology of Scarlet Fever?
* Highly contagious and transmitted through infected saliva or mucus * Common in neonates * common in those of 2-8 years
772
What are some risk factors for Scarlet Fever?
* Neonates * Immunocompromised * Concurrent chickenpox or influenza
773
What are the clinical features of Scarlet Fever?
* Initial sore throat, Fever, Headache, fatigue * **Pinpoint sandpaper like blanching rash** on trunk initially then spreads to the rest of the body * **strawberry tongue** * Cervical Lymphadenopathy
774
What are the investigations for Scarlet Fever?
**Clinical Diagnosis**
775
What is the management of Scarlet Fever?
* **Oral Antibiotics:** Benzylpenicillin for 10 days * Notify Public Health
776
What is Coxsackie's Disease?
**Hand Foot and Mouth** disease is caused by the Coxsackie A virus part of the enterovirus family.
777
What is the presentation of Coxsackie Disease?
* Starts with Upper Respiratory Tract symptoms such as **tirdness, sore throat and dry cough** * Fever * After 1-2 days, small mouth ulcers appear followed by blistering red spots across the body * These are most notable on the **Hands, Feed and mouth**
778
What are the investigations for Coxsackie's disease?
**Clinical diagnosis**
779
What is the management of Coxsackie's Disease?
**No Treatment** * Supportive management with adequate fluids and simple analgesia * Rash and illness will resolve spontaneously after a week to 10 days * It is highly contagious and so measures to avoid transmission should be taken
780
Define HIV
Human Immunodeficiency Virus that causes the infection that makes someone HIV positive. AIDS refers to the acquired immunodeficiency syndrome that occurs at the end stages of a HIV infection, once the infection has affected the immune system enough to make the person susceptible to recurrent and unusual infections. AIDS is usually referred to in the UK as late stage HIV.
781
What are the surface glycoproteins of HIV?
gp41 gp120 **Encoded by env**
782
What are the core glycoproteins of HIV?
p15 p17 p24 (useful in the diagnosis of primary HIV infection) **Ecoded by gag**
783
What are the enzymes of HIV?
Integrase Ribonuclease Reverse transcriptase Protein (These are key mechanisms targeted by anti-retroviral therapies) **Encoded by pol**
784
What kind of Virus is HIV?
Single stranded RNA retrovirus
785
What is the natural history of HIV?
**Primary infection (weeks-months post-exposure):** * Can occur between 2-12 weeks post-exposure * Rapidly rising viral load * Decreasing CD4+ count * HIV seroconversion: acute illness, fever/night sweats, maculopapular rash, lymphadenopathy, ulcers (mouth/genital), neurological involvement (meningoencephalitis/transverse myelitis). **Latent period (up to 10 years):** * Low viral load * Stable CD4+ count (usually not below 350x10^6/L) **Symptomatic period:** * Gradually rising HIV viral load * Gradually decreasing HIV CD4+ count * Characterised by systemic symptoms: * Weight loss * Fever/night sweats * Increased risk of opportunistic infections (varies depending on the CD4+ count) **Acquired immunodeficiency syndrome:** * End-stage HIV * Defined by a CD4+ count <200x10^6/L and/or the development of one or more opportunistic infections
786
What are some AIDs defining illnesses based on the CD4 T cell count?
**CD4 <500 ul (Not AIDs but HIV opportunistic infections)** * Mycobacteria Tuberculosis * Kaposi Sarcoma - HHV8 * Coccidiodomycosis * Cervical Cancer **AIDs defining illnesses:** **CD4 <200 ul (the 3 Ps)** * Pneumocystis pneumonia * Progressive multifocal Leukoencephalopathy * Histo**p**lasmosis **CD4 < 100ul: (4 Cs)** * Candidiasis - eosophageal * Cerebral Toxoplasmosis * Cryptococcus * Cryptosporidiosis **CD4 < 50ul:** * CNS lymphoma * CMV * Mycobacterium Avium Complex (MAC) infection
787
How is HIV Transmitted?
* Unprotected anal, vaginal or oral sexual activity * Mother to child at any stage of pregnancy, birth or breastfeeding. This is referred to as vertical transmission. * Mucous membrane, blood or open wound exposure to infected blood or bodily fluids. (This could be through sharing needles, needle-stick injuries or blood splashed in an eye).
788
What are the clinical features of HIV seroconversion illness?
* Fever * Lymphadenopathy * Maculopapular rash (commonly found on the upper chest) * Mucosal ulcers * Myalgia * Arthralgia * Fatigue * **Rarely will this rapidly progress to AIDs within a year**
789
What are some differential diagnoses for HIV seroconversion illness?
* **Influenza:** Characterised by sudden onset fever, cough, headache, muscle and joint pain, severe malaise, sore throat, and a runny nose. * **Mononucleosis (Epstein-Barr virus or cytomegalovirus):** Typically presents with fever, pharyngitis, lymphadenopathy, and fatigue. * **Acute streptococcal pharyngitis:** Symptoms include sore throat, fever, headache, abdominal pain, nausea, and vomiting. * **Viral hepatitis:** May present with jaundice, fatigue, nausea, fever, and muscle aches.
790
What is the epidemiology of HIV in Children?
* Affects 2 million children per year * Main route of transmission is Mother-child during pregnancy, at delivery or through breast feeding.
791
What are the investigations for HIV in children?
**Babies at 3 months: in HIV positive parents** * HIV viral load test at 3 months: if negative the child has not contracted HIV * HIV antibody test at 24 months: If the 3 month test is negative and they are not breast fed this should also be negative. **Children over 18 months** * Presence of HIV antibodies is diagnostic **Children Under 18 months:** * HIV DNA PCR is needed as antibodies may be present from exposure to maternal antibodies not active infection
792
How is HIV managed in children?
* **Antiretroviral therapy (ART)** to suppress the HIV infection * Normal childhood vaccines, avoiding or delaying live vaccines if severely immunosuppressed. * Prophylactic co-trimoxazole (Septrin) for children with low CD4 counts, to protect against pneumocystis jirovecii pneumonia (PCP) * Treatment of opportunistic infections **Paediatric HIV MDT involvement**
793
What steps can be taken to reduce HIV transmission during birth?
**Mode of delivery will be determined by the mother viral load:** * **Normal vaginal delivery** is recommended for women with a viral load < 50 copies / ml * **Caesarean sections** are considered in patients with > 50 copies copies / ml and in all women with > 400 copies / ml * **IV zidovudine** should be given during the caesarean if the viral load is unknown or there are > 10000 copies / ml **Prophylaxis Treatment:** * May be given to the baby depending on the mothers viral load * **Mums Viral load < 50 copies per ml:** Zidovudine for 4 weeks * **Mums Viral Load > 50 copies per ml:** Zidovudine, lamivudine and nevirapine for 4 weeks
794
What advice should be given to HIV positive mothers when considering breast feeding?
HIV can be transmitted during breast feeding even if the mother's viral load is undetectable. Breastfeeding is **never recommended** for mothers with HIV.
795
When should you test for HIV?
* Babies to HIV positive parents * When immunodeficiency is suspected * Young people who are sexually active can be offered testing if there are concerns * Risk factors such as needle stick injuries, sexual abuse or IV drug use.
796
What are the 3 classes of HIV drugs?
Reverse transcriptase inhibitors - nucleoside/non-nucleoside Protease inhibitors Fusion inhibitors
797
What drugs make up HAART?
* 2 Nucleoside reverse transcriptase inhibitors + 1 non-nucleoside reverse transcriptase inhibitor * 2 Nucleoside reverse transcriptase inhibitors + 1 Protease inhibitor
798
What is an important differential diagnosis of HIV seroconversion illness?
Secondary Syphilis
799
What markers are used for monitoring HIV infection?
CD4+ T cell count/ul HIV Viral load (RNA copies/ml)
800
What are the main cells that are infected via HIV? What other cells also can be infected?
CD4 T cells Dendritic cells, macrophages, astrocytes
801
Explain the life cycle of the HIV virus?
1️⃣ Glycoproteins on HIV molecule (gp160; formed of gp120 and gp41) allow it to adhere to: * CD4 receptors (gp120 and gp41 receptors) * CCR5 receptors on Macrophages * CXCR4 Receptors on CD4 T cells 2️⃣ Once viral capsid enters cell, viral enzymes and nucleic acid are uncoated and released. 3️⃣ Using reverse transcriptase, single stranded RNA is converted into double-stranded DNA. 4️⃣ Viral DNA then is integrated into cell own DNA through the action of integrase enzyme. 5️⃣ When infected cell divides the viral DNA is transcribed, producing long chains of viral proteins. Whilst infected cell is replicated, the viral DNA also replicates alongside. 6️⃣ Viral RNA is spliced and the protein chains are cleaved and reassembled by protease enzyme into individual proteins - these combine to form a functioning virus. 7️⃣ The immature virus exocytosed, taking some cell membrane with it to form a new lipid envelope 8️⃣ Once outside of the cell, the virus undergoes further maturation.