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Paediatrics: Core Medical
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- Fetus: Veins carry oxygenated blood, arteries carry deoxygenated blood
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- From the internal iliac arteries to the placenta
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- The placenta supplies oxygen and nutrients in the fetus, not the lungs
- They allow more blood to reach the systemic circulation & tissues, bypassing the liver & lungs (diagram below)
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- Ductus venosus
- From umbilical vein to IVC - Bypass liver
- Foramen ovale
- From right atrium to left atrium - Bypass lungs
- Ductus arteriosus
- From pulmonary artery to aorta - Bypass lungs
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- Foramen ovale: Baby breathes → Alveoli expansion → ↓ pulmonary vascular resistance → ↓ RA pressure → Closure of the foramen ovale
- Becomes fossa ovalis
- Ductus arteriosus: ↓ circulatory prostaglandins → Closure of ductus arteriosus
- Becomes ligamentum arteriosum
- Ductus venosus: No flow through umbilical veins → Duct closure
- Becomes ligamentum venosum
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Formulating differentials:
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- Not pathological. Caused by fast blood flow through various areas of the heart during systole
- All the S’s:
- Soft
- Short
- Systolic
- Symptomless
- Situation dependent (quieter when standing)
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- S2: Closure of the aortic and pulmonary valves
- On inspiration the right side of the heart fills faster than the left → Takes longer for RV to empty due to increased volume → Pulmonary valve closes after the aortic
- Splitting of the second heart sound is normal in children if it is only on insipration
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Constant volume throughout
- Mitral/tricuspid regurgitation
- VSD (left lower sternal border)
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Crescendo-decrescendo murmur (loud)
- Aortic stenosis
- Pulmonary stenosis (caused by TOF)
- ASD (mid-systolic)
- HOCM (fourth intercostal space LSE)
- Coarctation of the aorta
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- Mid-systolic crescendo-decrescendo murmur in second intercostal space LSE
- Atria are filling during ventricular contraction
- Fixed split second heart sound (on inspiration & expiration)
- Shunt from LA → RA causing overfilling of RV, taking londer to empty
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- Continuous "machinery" murmur
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- Ejection systolic due to pulmonary stenosis
Investigations & management:
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- Wait 24 hours & inform senior then review again:
- If still present refer for for ECHO
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- Louder than 2/6
- Diastolic
- Louder on standing
- Other symptoms: FTT, feeding difficulty, SOB, cyanosis
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- ECG
- CXR
- Echocardiogram
Cyanotic vs Acyanotic:
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A heart defect that allows blood to bypass the lungs and enter the systemic circulation → Recirculation of deoxygenated blood.
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- Right side of the heart pumps into the aorta & left side pumps into pulmonary vein.
- This results in no blood flow from the lungs into the systemic circulation
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- VSD
- Coarctation of aorta
- Pulmonary stenosis
- Maternal T1DM/T2DM but not gestational diabetes
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- No effect on development as aorta supplies placental blood to systemic tissues.
- After birth it is fatal if not corrected, there is no oxygenated blood reaching tissues → Cyanosis
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- Antenatal USS usually
- Cyanotic child at or within a few days of birth
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- VSD or PDA as they allow for oxygenated blood to travel into the systemic circulation
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- Immediate:
- VSD: No medication needed
- No VSD: Prostaglandin infusion (alprostadil) to maintain the ductus arteriosus
- Definitive:
- Open heart surgery to perform an "arterial switch" while the patient is on cardiopulmonary bypass
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- Ventricular septal defect
- Overriding aorta: Aortic valve is further right than normal above the VSD.
- Pulmonary stenosis
- RV hypertrophy: Results from the left-to-right shunting and pulmonary stenosis
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- Pulmonary stenosis results in less pulmonary blood flow (main factor)
- Overriding aorta is positioned further right so receives deoxygenated blood from the contracting RV
- The RVH causes a right-to-left shunt
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- Rubella infection
- Mother > 40 years
- Alcohol consumption in pregnancy
- Diabetic mother
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- Can be picked up on antenatal scans or with an ejection systolic murmur on newborn baby check.
- Typically presents within around 1-2 months with cyanosis and/or an ejection systolic murmur in the 2nd intercostal space RSE & tet spells.
- Others: Clubbing, poor feeding
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- Intermittent symptomatic periods where the right-left shunt becomes worsened causing a cyanotic episode.
- Pathophysiology: Exertion (walking, crying) → ↑ CO2 → Systemic vasodilation → Pulmonary vascular resistance > systemic → More deoxygenated RV blood enters the aorta
- Management:
- Squat: Increases SVR encouraging pulmonary blood flow
- O2, β-blockers, IV fluids, morphine
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- Echocardiogram: Establish diagnosis
- Doppler flow studies: Uses colour to demonstrate the flow through the heart
- CXR: ‘Boot shaped’ heart due to RVH
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- Neonatal: Prostaglandin (alprostadil) infusion to maintain the ductus arteriosus
- Definitive: Open heart surgery (5% mortality)
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- Tricuspid valve is set lower in the right side of the heart causing a bigger RA and smaller RV
- Poor blood flow to pulmonary vessels
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- When they also have an ASD as it leads to a right-left shunt
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- With ASD: A few days after birth they present with cyanosis, gallop rhythm, SOB and oedema
- Without ASD: Present later with a pan-systolic murmur (due to tricuspid incompetence)
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- ECG: Arrhythmias, RBBB, LAD
- CXR: Cardiomegaly, right artial enlargement
- Echocardiogram
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- Wolff-Parkinson white (slurred upstroke)
- More likely to have an accessory pathway due to the malformation of the endocardial cushion
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- Treat arrhythmias & HF.
- Definitive: Surgical correction
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- Patient has an:
- atrial septal defect, and either a;
- ventricular septal defect or a patent ductus arteriosus
- Pulmonary pressure is higher than systemic pressure → Right-to-left shunt → Cyanosis
- Patients with the defects above only get cyanosed if they have this syndrome
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- Anatomical defect (ASD/VSD/PDA) → Left-to-right shunt
- ↑ Pulmonary vascular resistance & ↑ left-sided preload → RVH & LVH → HF
- ↓ cardiac output → Pulmonary pressure higher than systemic pressure
- Right-to-left shunt → Cyanosis
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- Cyanosis
- Plethoric (red) complexion
- Hypoxia → ↑ EPO → Polycythaemia
- Clubbing
- Dyspnoea
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- Medical:
- Sildenafil → Dilates pulmonary vessels
- Venesection for polycythaemia
- Anticoagulation
- Prophylactic antibiotics to prevent IE
- Surgical (definitive):
- Heart-lung transplant
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A heart disease which causes oxygenated blood to be pumped around the body abnormally.
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- Septum primum & septum secondum grow downwards towards endocardial cushion & fuse.
- Each septa form holes within them, allowing blood to travel from RA to left, but not the other way
- Lower septum primum blocks the flow from left to right
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- ↑ RA & RV pressure → ↑ Pulmonary vascular resistance → RV & LV hypertrophy → HF
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- Eisenmenger syndrome
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From most to least common:
- Ostium secondum: Septum secondum doesn't fully close
- PFO (although not strictly classed as an ASD)
- Ostium primum: Septum primum doesn't fully close → AV valve defects → Atrioventricular septal defect
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- Small & asymptomatic: Watch & wait
- Large: Transvenous catheter closure/open heart surgery
- Anticoagulants (DOAC/warfarin) to reduce clot, and potential subsequent stroke, risk.
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- Stroke: DVT thrombus goes to brain instead of lung
- Heart failure
- Eisenmenger syndrome
- AF or atrial flutter
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- Congenital hole in the septum between the two ventricles, varying in size from very small to the entire septum
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- Down's syndrome
- Turner's syndrome
- Tetralogy of Fallot
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- Picked up on antenatal USS
- Pan-systolic murmur in LLSE & loud P2 (↑ flow into pulmonary artery) heard on newborn baby check
- Later with cyanosis due to Eisenmenger syndrome
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- All: Risk of IE → Consider antibiotic prophylaxis
- Mild (No symptoms or evidence of severe disease): Watch & wait, they often close spontaneously
- Severe (Pulmonary hypertension, cyanosis, big defect): Transvenous catheter closure or open heart surgery
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- Prematurity
- Maternal rubella infection
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- From aorta into pulmonary vessels → Pulmonary hypertension
- ↑ pulmonary vascular resistance → Right heart strain → RV hypertrophy
- ↑ blood returning to left side of heart → ↑ preload → LVH
- Heart failure due to RVH & LVH
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- Echocardiogram
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- < 1 year: Monitor using echocardiogram, waiting for spontaneous closure
- > 1 year: Trans-catheter or surgical closure
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- Indomethacin - Inhibits prostaglandin synthesis, closing the duct
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- Narrowing of the aortic arch
- Usually around the ductus arteriosus
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- Turner's syndrome
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This depends on where the narrowing is on the arch:
- Reduced blood pressure distal to the narrowing → Femorals
- Increased pressure proximal to narrowing → Arms & head
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- Neonate: Weak femorals
- 4 limb BP: High in arms, low in legs
- Left infraclavicular systolic murmur
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- Severity varies, some need emergency surgery after birth and some can be asymptomatic until adulthood.
- Critical coarctation: Prostaglandin infusion & open heart surgery
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- Narrowing of the aortic valve caused by thickening & calcification of the leaflets
- Can have 2-4 leaflets on the valve
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- Mild: Incidental murmur on routine examination
- Severe: Fatigue, SOB, dizziness & syncope, worse on exertion
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- Ejection systolic murmur
- Palpable thrill
- Narrow pulse pressure
- Slow rising pulse
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- ECG
- Echocardiogram
- Exercise testing: Monitor disease progression
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- Percutaneous balloon valvuloplasty
- Surgical valvulotomy
- Valve replacement
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- Tetralogy of Fallot
- William syndrome
- Noonan syndrome
- Congenital rubella syndrome
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- Same as aortic stenosis
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- Parainfluenza infection → Upper airway oedema & obstruction
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- 6 months - 2 years
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- Bacterial tracheitis caused by S. aureus
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- Simple supportive treatment: Fluids & rest
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Stepwise approach:
- PO dexamethasone (& O₂)
- Nebulised adrenaline & budesonide
- Intubation & ventilation
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- Haemophilus influenza type B (HiB)
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- HiB vaccination
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- Not needed in acute epiglottitis - Treat immediately
- If unsure: Lateral XR of the neck
- "Thumbprint sign" - Oedematous & swollen epiglottis
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- Secure airway - Don't distress the patient as closes airway. Inform anaesthetist & paediatrician immediately
- Intubation/tracheostomy may be needed but often isn't
- IV antibiotics & PO steroids
- Ceftriaxone
- Dexamethasone
- Prophylactic antibiotics for all in the household
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- Epiglottic abscess: Collection of pus around epiglottis presenting in a similar way
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- Bordatella pertussis (gram negative)
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- Present with apnoea
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- Pneumothorax, fainting or vomiting
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- Nasal swab with PCR testing/culture
- Cough > 2 weeks: IgG against pertussis toxin
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- Supportive care
- Antibiotics: Azithromycin/Co-trimoxazole
- Prophylactic antibiotics to all those in the household
- Notify Public Health England (PHE)
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- Yes - Exclusion until 48 hours after commencing antibiotics
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- Bronchiectasis - Damage, widening & thickening of the bronchi
- Hypoxic brain injury
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- Structural laryngeal abnormality where the supraglottic larynx causes partial airway obstruction → Chronic stridor
- Aryepiglottic folds are shortened pulling the epiglottis into an "omega" shape
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- No interventions required usually, as it grows the larynx is better able to support itself
- If upper airway obstruction then may need tracheostomy
Cases: Name the diagnosis & cause.
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- Whooping cough
- Bordatella pertussis (gram negative)
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- Acute epiglottitis
- Haemophilus influenza type B (HiB)
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- Laryngomalacia
- The exact cause of laryngomalacia unknown. Relaxation or a lack of muscle tone in the upper airway may be a factor.
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- Croup (laryngotracheobronchitis):
- Parainfluenza infection → Upper airway oedema & obstruction
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- Respiratory syncytial virus (RSV)
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- 0-1 year
- RSV cause inflammation & oedema in the airways, adult airways are wide enough for this not to affect their function but it can cause occlusion of children's airways
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- Ex-premature children with chronic lung disease
- CF
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- Cyanosis
- Nasal flaring
- Head bobbing
- Tracheal tug
- Intercostal & subcostal recession
- Use of accessory muscles
- Abnormal airway noises
- Raised RR
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- Grunting - Exhaling against a partially closed glottis to maintain more residual volume
- Wheeze
- Stridor - Less likely here.
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- Viral-induced wheeze
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- Day 5-7 of the infection
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- Hyponatraemia
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Most are managed at home, except those with the following:
- Aged < 3 months or with any pre-existing condition (Down's, CF, prematurity)
- Not feeding (> 50% less than normal) / clinical dehydration
- Fluid challenge - Can they drink water?
- Clinical dehydration - Mucous membranes, skin turgor
- RR > 70, SpO2 < 92%
- Signs of severe respiratory distress
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- Adequate fluid intake & nutrition - PO/IV
- Oxygen therapy
- Ventilatory support
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- High-flow humidified O₂ (Airvo) - Adds positive end-expiratory pressure keeping airways patent
- CPAP
- Intubation & ventilation - Insertion of ET tube
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- ABG/VBG looking for pO₂, pCO₂ & lactate
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- Ex-premature children with chronic lung disease & children with congenital heart disease
- Palivizumab monthly injections
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- Can be bacterial or viral.
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- Bronchial breath sounds - Inspiration & expiration are a similar length
- Focal coarse crackles - Air passing through sputum
- Dullness to percussion - Tissue collapse or consolidation
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- Bacterial
- S. pneumonia
- S. pyogenes (group A strep)
- Group B strep (birth)
- S. aureus - Cavitating
- H. influenzae - Unvaccinated
- Mycoplasma
- Viral
- RSV
- Parainfluenza virus
- Influenza
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- Bedside:
- Sweat test → CF
- Bloods:
- FBC → WCC (leukaemia, other malignancy), Hb, platelets
- Sputum culture → Organism could indicate condition (e.g. P. aeruginosa)
- HIV testing
- Radiology:
- CXR → Structural change & scarring
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- Typical: Amoxicillin
- Atypical: Amoxicillin & clarithromycin
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This is chronic lung disease of prematurity.
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- Hypoxia at birth: Capillary wall damage → Leakage of interstitial fluid & pulmonary oedema → Bronchiolar necrosis & loss of ciliated epithelium
- Hyperexpansion & atelectasis
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- Increased opacification of the lung tissue representing thickened tissue and less air
- Atelectasis
- Cardiomegaly (from pulmonary hypertension)
- Hyperinflated lungs
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- Formal sleep study assessing their SpO2 during sleep supports the diagnosis & guides management
- Nasal cannulae providing O2 while sleeping
- RSV prophylaxis with palivizumab
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Genetics & pathophysiology:
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- Mutation of the CFTR gene on chromosome 7
- Most common is 𝜹-F508 mutation
- Autosomal recessive inheritance
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- Chloride channels
- Pancreas, airways & bowel
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- Pancreatitis: Thick pancreatic secretions block the ducts
- LRTI: Loss of airway surface liquid → Thick secretions → Mucociliary escalator dysfunction → Bacterial colonisation
- Male infertility: Congenital absence of vas deferens in males
Presentation, investigations & management:
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- Newborn bloodspot screening
- Meconium ileus: Meconium is not passed in the first 24 hours due to being thick & sticky → Bowel blockage → Distension & vomiting
- AXR: 'Bubbly' appearance of the intestines with a lack of air-fluid levels
- Recurrent LRTI, pancreatitis & FTT in later childhood
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- Congenital heart disease
- Malignancy
- Tuberculosis
- Liver cirrhosis
- IBD
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- Sweat test (Gold standard): Excessive NaCl in sweat due to low fluid secretion
- Pilocarpine is applied to the skin inducing sweating at the site
- Electrodes run a current through this and also induce sweating
- Sweat is collected on gauze & tested for Cl⁻ concentration
- > 60 mmol/L → CF
- Genetic testing for CFTR gene mutation
- Raised immuno-reactive trypsinogen
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- S. aureus
- Prophylactic flucloxacillin
- P. aeruginosa
- Nebulised tobramycin
- PO ciprofloxacin
- Others: H. influenzae, Klebsiella, E. coli, Burkhodheria
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MDT approach:
- Chest physiotherapy
- High calorie diet due to malabsorption
- Creon tablets (given pancreatic impairment)
- Prophylactic flucloxacillin
- Salbutamol
- Nebulised DNase (makes respiratory secretions thinner)
- Vaccinations
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- Diabetes due to pancreatitis
- Osteoporosis due to calcium malabsorption
- Vitamin D deficiency due to liver dysfunction
- Liver failure
Case:
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- Both parents are carriers so have the Cc genotype
- Child cannot be cc because they would have the disease so the remaining options are CC, Cc or cC.
- Carrier likelihood: 2/3
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- Paranasal sinusitis
- Bronchiectasis
- Situs inversus
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- Autosomal recessive genetic condition → Ciliary dysfunction throughout the body (esp. in respiratory tract) → Mucus buildup
- Associated with consanguinity (incest)
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- All internal organs are switched to the opposite side, hence heart is on the right & liver is on the left.
- Present in 50% of cases of Kartagener's
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- Infertility due to fallopian tube cilia problems in women & sperm flagella (tail) problems in men
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- Sample of ciliated epithelium is taken from the upper airway by nasal brushing or bronchoscopy
- Cilia are examined
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- Similar to CF & bronchiectasis
Name the diagnosis & cause based on the following presentations:
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- Bronchiolitis
- RSV
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- Kartagener's syndrome (Primary ciliary dyskinesia)
- Autosomal recessive genetic condition → Ciliary dysfunction throughout the body (esp. in respiratory tract) → Mucus buildup
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- Chronic lung disease of prematurity (Bronchopulmonary dysplasia)
- See notes above for mechanism.
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- Cystic fibrosis
- Mutation of the CFTR gene on chromosome 7
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- Pneumonia
- Causes: Bacterial or viral
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- Viral-induced wheeze or asthma
- Viral: < 3 years, no atopy, only presents when other viral symptoms are present
- Asthma: Can be worsened by viral infection but signs will be present in its absence as well. Triggered by exercise, cold, dust & emotion. Atopic history
- Virus causes airway inflammation & oedema causing turbulent flow, this is heard more loudly on expiration
- Tumour
- Inhaled foreign body
- Pneumonia
- Same as acute asthma in children
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- Constipation
- UTI
- Coeliac disease
- IBD
- IBS
- Mesenteric adenitis
- DKA
- Abdominal migraine
- Pancreatitis
- Adolescent girls: Dysmenorrhoea, Mittelchmerz, ectopic pregnancy, pregnancy, PID, ovarian torsion
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- Appendicitis
- Meckel's diverticulum
- Pyloric stenosis
- Intusussception
- Bowel obstruction
- Testicular torsion
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- Persistent bilious vomiting → Obstruction
- RIF pain, guarding & rigidity
- Rectal bleeding
- Failure to thrive
- Dysphagia
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Bedside:
- Urinalysis → UTI
- Faecal calprotectin → IBD
Bloods:
- FBC: Anaemia → IBD/Coeliac/Blood loss
- ESR/CRP → IBD
- Anti-TTG/EMA → Coeliac
Upper GI vs Lower GI:
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Immune-mediated allergic response to casein and whey, naturally-occurring milk proteins.
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- IgE mediated: Type-I hypersensitivity reaction, with B-cells producing IgE antibodies agains cow’s milk protein. Rapid reaction over ~ 2 hours (anaphylaxis is rare).
- Non-IgE mediated: T-cell activation against cow’s milk protein. Reactions are less severe and occur over several days.
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- < 1 year of age, often when weaned from breast to formula milk but can be in breastfed if mother is drinking dairy products.
- Gastrointestinal symptoms:
- Bloating/wind
- Abdominal pain
- Diarrhoea & vomiting
- Allergic symptoms:
- Urticaria
- Angioedema
- Cough/wheeze
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- Usually a clinical diagnosis is sufficient.
- IgE antibody blood test to cows milk protein can be useful. This has a high sensitivity but low specificity leading to false positives. They may be sensitised (have the IgE), but not be allergic.
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- Mild: Breast feeding mothers avoid dairy products
- Mild: Extensively hydrolysed formulas (casein and whey have been broken down so won't trigger a response).
- This is the first-line, cheaper, formula made from cow’s milk. They break down the casein and whey into smaller peptides which are less immunogenic.
- Severe: Amino acid formulas (neocate)
- This is the second-line, more expensive, formula for those who still have symptoms with eHF (10% of children).
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- Patients have the same GI symptoms but no symptoms suggestive of allergy.
- They grow out of the intolerance by 2-3 years.
- Managed the same but after 1 year they are started on the milk ladder.
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Gluten is broken down to gliadin in the small intestine → Inflammatory response → Cell destruction → Malabsorption.
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- IgA anti-TTG antibodies
- Total IgA because if there's an IgA deficiency then the test will be negative but the disease may still be present
- IgG anti-TTG/anti-EMA
- Anti-EMA antibodies
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- Villous atrophy
- Crypt hypertrophy
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- Common: T1DM & thyroid - All of these patients are tested for coeliac disease
- Other autoimmune: Autoimmune hepatitis, PBC, PSC
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- Lifelong gluten-free diet
- Dapsone antibiotic for dermatitis herpetiformis
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- Immaturity of the lower oesophageal sphincter → Easily allows reflux into the oesophagus.
- Normal up to 1 year and can be physiological if all other variables are normal.
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- Overfeeding
- Pyloric stenosis
- Gastritis/gastroenteritis
- Appendicitis (pain)
- Obstruction
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- Not keeping down any feed
- Vomiting - Projectile / forceful vomiting / bile stained / blood
- Melaena
- Abdominal distention
- Rash, angioedema & signs of allergy
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- Small, frequent meals. Don't overfeed
- Keep baby upright after feeding
- Gaviscon with feeds, thickened milk
- Ranitidine
- Omeprazole
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- Sandifer's syndrome
- Neck: Torticollis
- Back: Dystonia → Twisting & arching
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Pyloric sphincter hypertrophy → Pylorus narrowing → Powerful stomach peristalsis to try to push stomach contents through → Projectile vomiting.
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- Hypochloric metabolic alkalosis (vomiting HCl from the stomach).
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- USS
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- Laparoscopic pyloromyotomy (Ramstedt's operation).
Cases:
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- Coeliac disease
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- Dermatitis herpetiformis (pruritic vesicles on the extensor surfaces)
- Anaemic due to malabsorption of B12/folate
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- Pyloric stenosis
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- GORD
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- Conjugated & unconjugated bilirubin levels
- Biliary atresia - Narrowing/absence of the bile duct → Cholestasis
- 2-8 weeks old
- Surgery: Kasai portoenterostomy (somewhat successful but they often require a full liver transplant).
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- Cow's milk protein allergy (IgE-mediated)
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- Transient lactose intolerance following infection
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- Absolute constipation
- Bilious vomiting
- Tinkling bowel sounds
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- Meconium ileus (CF)
- Hirshprung's disease
- Pyloric stenosis
- Surgical adhesions
- Intusussception
- Imperforate anus
- Volvulus
- Strangulated hernia
- Abdominal mass
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- Depends on the cause - treatment would be tailored to the cause.
- Refer to paediatric surgical unit
- NBM
- Drip & suck - IV fluids & NG tube insertion
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Congenital condition where the myenteric (Auerbach's) plexus of the bowel is absent → No large bowel peristalsis → No passing of meconium.
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- Parasympathetic ganglion cells are missing as they usually migrate down from higher in the GI tract but they don't here.
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- Dilated loops of bowel with fluid levels (meconium ileus - no fluid levels)
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- RF: Family history
- Associations: Down's, neurofibromatosis, MEN II
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- AXR: Obstruction
- Rectal biopsy → Absence of ganglionic cells
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- Fluid resuscitation
- Bowel irrigation/washouts
- Definitive: Surgical removal of aganglionic section of bowel
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- Hirschprung-associated enterocolitis
- Management: Fluids, antibiotics & decompression of obstructed bowel
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Bowel invaginates (folds) into itself → Decreased bowel lumen size causing obstruction.
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- 6 months - 2 years (more common in boys).
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- Preceding viral illness (Common)
- Henoch-Schönlein purpura (HSP)
- Cystic fibrosis
- Meckel's diverticulum
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- Abdominal USS
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- Therapeutic enemas - Pump contrast/water/air into colon to force the folded bowel out
- Surgical reduction
- Surgical resection of gangrenous/perforated bowel
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- Rabbit dropping stools
- Abnormal posture - Retentive posturing
- Faecal impaction causing overflow soiling
- Tenesmus - Always feeling like you need to go
- Abdominal pain
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- Faecal incontinence - Not pathological until 4 years.
- Commonly a sign of chronic constipation causing stretching and desensitisation of the rectum
- Rarer causes: Spina bifida, Hirschprung's, CP
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- Hirschprung's disease
- Hypothyroidism
- Cystic fibrosis - Meconium ileus
- Spinal cord lesions
- Cow's milk intolerance
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- Not passing meconium in the first 24 hours
- Failure to thrive
- Acute abdominal pain/bloating
- Vomiting - Bilious, projectile
- Neurological signs
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- Plenty of fluids & high fibre diet
- Laxatives
- Movicol
- Bowel diary & encouragement to go to the toilet
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- Abdominal pain, blood diarrhoea, mouth ulcers, other autoimmune conditions, WL, anaemia
- Systemically unwell during flares
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- No/less blood or mucus in stool
- Entire bowel is affected
- Skip lesions
- Terminal ileum most affected & Transmural thickness inflammation
- Smoking makes it worse
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- Continuous inflammation
- Limited to colon & rectum
- Only superficial mucosa affected
- Smoking protective
- Excrete blood & mucus
- Use aminosalicylates
- Primary sclerosing cholangitis
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- Finger clubbing
- Erythema nodosum
- Pyoderma gangrenosum
- Episcleritis/iritis
- PSC in UC
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- Rectum
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- Skin: Erythema nodosum, pyoderma gangrenosum
- Primary sclerosin cholangitis (independent of flares)
- Eyes: Episcleritis, scleritis, anterior uveitis
- Osteoporosis (malabsorption)
- Peripheral neuropathy (B12)
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Disease is limited to the mucosa, showing:
- Crypt abscesses
- Goblet cell depletion
- Mucin depletion
- Mucosal ulcers
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- 'Lead pipe sign' - Loss of haustral markings on the bowel
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- Toxic megacolon: AXR to investigate
- ACR (colon dilation), Temp > 38.6°C, HR > 120bpm, anaemia, ↑ neutrophils
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Age of onset is bi-modal:
- 15-40 years: Majority
- 60-80 years: Small second peak
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- Distal ileum
- Proximal colon
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- Abdominal pain (potentially colicky)
- Diarrhoea (often chronic)
- Oral aphthous ulcers (can be painful)
- Perianal disease
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- Bedside: Faecal calprotectin
- Bloods: FBC (macrocytic anaemia), LFT (PSC).
- Radiology: USS/CT/MRI (fistulae, abscesses, strictures)
- Endoscopy: OGD & colonoscopy
- Take biopsies to look for microscopic features.
- If active disease only do flexible sigmoidoscopy because of risk of perforation in colonoscopy.
- If colonoscopy is negative in Crohn's, use MRI to assess the small bowel.
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- Induce remission
- Steroids - PO prednisolone (mild-moderate) / IV hydrocortisone (severe)
- Add azathioprine/mercaptopurine
- Refractory: Infliximab
- Aminosalicylate - Mesalazine (mild-mod)
- Suppository/enema first
- PO if extensive disease
- Steroids - PO prednisolone (mild-mod) / IV hydrocortisone (severe)
- IV ciclosporin (severe)
- Maintain remission
- Azathioprine/Mercaptopurine
- Methotrexate
- Infliximab
- Adalimumab
- If only affecting one area of bowel severely, surgery will be considered.
- Aminosalicylate - Mesalazine (PO/PR)
- Azathioprine
- Mercaptopurine
- Panproctocolectomy: Surgery to remove the colon & rectum. May have hemicolectomy, depends on case.
- Patient has either a permanent ileostomy or ileo-anal anastomosis (J-pouch).
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Medical:
Surgical:
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Medical:
Surgical:
Cases:
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- Omphalocele
- Gastroschisis - No peritoneal covering
- Omphalocele - Staged closure starting immediately and ending at 9-12M (to prevent respiratory complications)
- Gastroschisis - Immediate closure
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- Hirschprung's disease
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- Meconium ileus
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- Intussusception
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