Paeds Flashcards
Give 3 signs of respiratory failure.
Hypoxaemia despite high FiO2
Acidosis
Increasing fatigue, or absence of movement with therapy
(OH)
Define pneumonia
An infection of the lower respiratory tract and lung parenchyma that leads to consolidation
(OH)
Give a common infecting agent of pneumonia in neonates, and what is the first line treatment?
group B strep E coli Klebsiella staph aureus (OH) IV Broad spectrum abx
Give a common infecting agent of pneumonia in infants and school age children.
Infants strep pneumoniae chlamydia School age: Strep pneumoniae staph aureus group A strep bordetella pertussis mycoplasma pneumoniae (OH)
What is the first line treatment for pneumonia in children?
Amoxicillin (Co-amox if influenza, erthryomycin 2nd line/m. pneumoniae, Macrolides for atypical pneumonia) PTS
What is croup and how does it present?
Common laryngotracheobronchitis. This is upper airway. Barking cough, harsh stridor and hoarseness, usually preceded by (low-grade) fever and coryza.
Give 6 signs of respiratory distress in an infant.
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 noise
What causes croup?
Usually viral, parainfluenza viruses.
How is croup differentiated from epiglottitis and tracheitis?
Epiglottitis/tracheitis: rarer but more severe, no barking cough, rapid onset, high grade fever >39 degrees, may be drooling. Bacterial.
Croup: fever below 39, barking cough, slower onset, can speak and swallow. Viral
How do you initially manage an upper airway restriction?
Don’t examine the throat or do a swab because it could cause further airway occlusion.
Be calm, confident and well-organised
Observe for hypoxia and deterioration
If severe, administer adrenaline and call anaesthetist.
if resp failure, intubate
What is the most common time of year and age group for croup?
6 months to 3 years, peak at 1 year. Autumn.
How is croup managed?
If upper airway obstruction is mild, and the parents can easily take the child into hospital if they get worse, they can be managed at home with careful observation.
Oral dexamethasone, oral pred and nebulised budesonide reduce severity and duration of croup, and the need for hospitalisation.
(Textbook)
What is pseudomembranous croup?
aka Bacterial tracheitis. Rare but dangerous upper airway condition in which child has high fever, appears toxic and has rapidly progressive airways obstruction with copious secretions. Caused by staph aureus.
How is bacterial tracheitis managed?
IV antibiotics, intubation and ventilation if required.
What is acute epiglottitis? How does it present?
Life-threatening emergency due to risk of respiratory obstruction. Acute onset, fever >39 in a toxic-looking child, very painful throat, cant swallow or speak, soft inspiratory stridor, open-mouthed and sitting upright to optimise airway.
What causes epiglottitis?
H influenza B. This is vaccinated against, so if they havent been vaccinated, have a higher index of suspicion. Swelling of epiglottis and surrounding tissues associated with septicaemia.
What is the most common age group for epiglottitis?
Age 1-6 but can affect all age groups
How is acute epiglottitis managed?
Do not lie the child down or examine the throat.
Admit to ICU/anaesthetic room accompanied by doctor in case of resp obstruction. Call anaesthetist, paediatrician and ENT surgeon.
Intubate, anaesthetise.
Once stable, blood cultures and IV cefuroxime
H influenzae –> rifampicin prophylaxis to close contacts
What is bronchitis? What are the 2 main presenting symptoms?
In children, this refers to acute bronchitis and usually presents with cough and fever. It may be caused by pertussis. (Textbook)
What is pertussis?
Aka whooping cough. Term for the highly contagious respiratory infection caused by bordatella pertussis bacterium.
Describe the presentation and disease course of pertussis.
1 week catarrhal phase: coryza
Then 3-6 weeks paroxysmal phase: coughing fits, inspiratory ‘whoop’. Coughing fits often worse at night and may culminate in vomiting, epistaxis and/or subconjuctival haemorrhage. Child goes red or blue, mucus flows from nose and mouth, apnoea may occur
Convalescent phase: Symptoms decrease.
What are the complications of pertussis?
Pneumonia, convulsions, bronchiectasis. They are rare but there is still a significant mortality, particularly in infants.
Who is most at risk of pertussis?
Not vaccinated
How should you manage a child with cough spasms?
Admit, isolate from other children, do nasal swab and PCR for b. pertussis. Blood film will show lymphocytosis >15x10^9/L
What is the role of antibiotics for pertussis?
Erythromycin eradicates b.pertussis but only decreases symptoms if started in the catarrhal phase so not used for most children. Therefore close contacts should have erythromycin prophylaxis.
What is the commonest serious respiratory infection of childhood, causing admission of 2-3% of infants each year?
Bronchiolitis.
Who is most at risk of bronchiolitis?
1-9 months, rare in >1 year olds
What causes bronchiolitis?
Respiratory syncytial virus (RSV) - 80%
Rest - human metapneumovirus, influenza, parainfluenza viruses, rhinovirus, adenovirus, m. pneumoniae
What are the clinical features of bronchiolitis?
Coryza
Then dry cough and increasing breathlessness
Feeding difficulty associated with increasing dyspnoea is often the reason for hosp admission
Recurrent apnoea in young infants
Who is most at risk of severe bronchiolitis? What is the usual causative organism?
Dual infection with RSV + human metapneumovirus
Affects premature infants, with bronchopulmonary dysplasia, CF, congenital HD
What signs on examination would be suggestive of severe bronchiolitis?
Apnoea in infants <4 months
Sharp, dry cough
Cyanosis/pallor
Hyperinflation of the chest causing downward displacement of the liver and prominent sternum
Subcostal and intercostal recession
Auscultation: fine end-inspiratory crackles, prolonged expiration.
What investigations would you do for bronchiolitis?
Respiratory viruses diagnosed with PCR analysis of nasopharyngeal secretions.
CXR (not always necessary): hyperinflation, air trapping, focal atelectasis.
Pulse oximetry for continuous sats
How would you manage bronchiolitis?
Supportive:
Humidified oxygen through nasal cannulae, concentration required determined by o2 sats
Monitoring for apnoea
Infection control - RSV highly contagious
High-risk infants (preterm, chronic lung disease, immunodeficiency) will receive palivizumab (synergis) which is a mab against RSV, over winter. (£££)
Describe the prognosis/complications of bronchiolitis.
Most recover in 2 weeks, but half will have recurrent episodes of cough and wheeze. Rarer: bronchiolitis obliterans - permanent damage.
What is asthma?
Asthma is a disease of chronic airway inflammation, bronchial hyper-reactivity, and reversible airway obstruction.
What is wheeze and how is childhood wheeze classified?
Wheeze is a high-pitched, expiratory sound from distal airway obstruction. Childhood wheeze is split into recurrent (eg IgE mediated) and episodic (eg viral episodic wheeze aka viral induced wheeze)
(Lissauer)
What are crackles?
Discontinuous ‘moist’ sounds from the opening of the bronchioles (Lissauer)
What is stridor? Give 3 causes.
Harsh, low pitched, mainly inspiratory sound from upper airway obstruction. Laryngomalacia Anaphylaxis Croup Epiglottitis
Which chest disorder is likely in a child with rapid, shallow breathing and reduced chest movement on one side? What would you look for on further examination?
Pneumonia.
Percussion: dullness
Auscultation: bronchial breathing, crackles
Give 5 differentials for a wheezing infant and one sign for each.
Atopic asthma - age >3, triggered by cold/pets, dirunal variation
Transient early wheeze - age <3, winter, preterm, maternal smoking
Bronchiolitis: 1-9 months, poor feeding, dry cough laboured breathing, apnoea, liver displacement
Pneumonia: tachypnoea, nasal flaring, lethargy, cyanosis
Cardiac failure: resp distress, heart murmur, hepatomegaly
[lissauer]
What causes the symptoms of asthma?
Bronchial inflammation occurs as a result of atopy, genetic factors, and environmental factors eg cold, smoking and aerosols. This causes oedema, mucus production, and infiltration with immune cells. This leads to hyper-responsiveness of the bronchi. The airways narrow, causing peak flow variability, wheeze, c cough, breathlessness, and chest tightness.
Which virus triggers most asthma exacerbations?
Rhinovirus
What features of the history would be suggestive of asthma?
Recurrent polyphonic wheeze (more than once)
Triggers - exercise, pets, cold air, laughter
worse at night and early morning
interval symptoms (between exacerbations)
personal or family history of atopy
What investigations would you do to diagnose asthma?
Trial with bronchodilators
examination of chest: there may be Harrison sulci (depressions at base of thorax), hyperinflation, polyphonic expiratory wheeze, prolonged expiration.
Examine nasal mucosa for allergic rhinitis, skin for eczema
Peak flow - diurnal variation, improves after bronchodilators. Variability in PEF in uncontrolled asthma.
What would be first line management for asthma?
SABA such as salbutamol. Effective for 2-4 hours.
Consider ipratropium bromide (anticholinergic) in young infants.
When are long-acting beta agonists indicated? What should always be prescribed with a LABA?
LABAs eg salmeterol/formoterol are used in severe exercise-induced asthma. Effective for 12 hours. (1st line is still a SABA before exercise). Always prescribed with inhaled steroid.
What are 3 side effects of inhaled corticosteroids?
Impaired growth, adrenal suppression, altered bone metabolism. These only really occur in high doses.
What is the first-choice add-on therapy in a child under 5?
Oral leukotriene receptor antagonist such as montelukast.
In a child over 5 it is a LABA eg salmeterol
Why is theophylline rarely used in children?
Side-effects of vomiting, insomnia, headaches, and poor concentration
When would oral prednisolone be indicated in asthma? why is is given on alternate days only?
Alternate days to reduce adverse effect on height
Used for severe persistent asthma where other treatment has failed. Managed by a specialist.
[lissauer]
In what circumstance would you admit a child with asthma to hospital?
If, after high dose bronchodilator therapy, they:
have not responded adequately clinically: persisting breathlessness and tachypnoea
are exhausted
still have marked reduction in PEF predicted or usual
O2 <92% on air
How is moderate acute asthma defined and managed?
O2 >92%, PF >50%:
SABA via spacer start at 2-4 puffs increase by 2 puffs every 2 min up to 10 puffs. Reassess in 1 hr
How is severe acute asthma recognised and managed?
O2 <92, PF <50%, breathless, accessory muscle use, tachypnoea, tachycardia
SABA via spacer, 10 puffs, oral pred/IV hydrocortisone, repeat bronchodilators every 20-30 mins as needed
How is life-threatening acute asthma recognised and managed?
O2 <92%, PF <33%, silent chest, poor resp effort, reduced consciousness, cyanosis
SABA via NEBULISER, ipratropium bromide, IV hydrocortisone, PICU/paediatrician, repeat bronchodilators every 20-30 mins.
[lissauer]
What is cystic fibrosis?
An autosomal recessive disorder affecting the CFTR protein. impaired Cl- transport causes thickened secretions, multi-system effects, mainly pancreatic insufficiency and chronic respiratory infections.
How is CF usually diagnosed?
Part of routine heel prick screening at 5-9 days looking for raised immunoreactive trypsinogen (IRT) and 29 CFTR gene mutations from blood-spot analysis on the Guthrie card.
If this is raised, sweat test is done. High Cl- levels in the sweat due to lack of cl- channel so Cl- can’t leave the sweat.
Less commonly presents clinically with FTT, chest infections, pancreatic insufficiency.
[Ox handbook]
How is CF inherited?
Autosomal recessive.
Which disease is associated with mutation in delta F508?
Cystic fibrosis
Which infectious agents are associated with cystic fibrosis?
S. aureus
H. influenza
P. aeruginosa
Burkholderia species
Which vitamins are given to people with CF and why?
Fat soluble vitamins (A, E, D, K) because loss of CFTR leads to blocked pancreatic ducts, pancreatic enzymes cannot get to the bowel to break down fat and protein, so they cannot absorb fat-soluble vitamins.
What problems can CF cause in infancy? Give 3
Meconium ileus - meconium is thick and impacted, causing small bowel obstruction
Prolonged neonatal jaundice
Hypoproteinaemia, oedema
[ox handbook]
What problems can CF cause in childhood? Give 3
Recurrent LRTIs Bronchiectasis Poor appetite Rectal prolapse Nasal polyps Sinusitis (rare to have symptoms) [ox handbook]
Describe the pulmonary management in CF, outside of exacerbations.
MDT: paediatric pulmonologist, physiotherapist, dietician etc.
Annual multisystem review
Pulmonary care - physiotherapy twice a day, antimicrobial therapy against s. aureus and h. influenzae, oral when well
Mucolytics - recombinant DNAase or inhaled hypertonic saline, 2h before physio
Oral azithromycin (long term anti inflammatory)
How is exacerbation of CF managed?
IV antibiotics against s. aureus and h. influenzae
What GI problems occur in CF and how are they treated?
Distal intestinal obstruction. treated with:
Lactulose
oral acetylcysteine
Gastrographin
What is otitis media and what causes it?
Infection of the middle ear. It is a common cause of conductive hearing loss.
Causes:
viral -RSV, rhinovirus
bacterial - pneumococcus, H influenza, moraxella catarrhalis.
What age group does otitis media usually affect and why?
6-12 months. Most children will have at least one episode of OM.
It affects children more because the eustachian tube is shorter, horizontal and functions poorly.
Why must you examine the tympanic membrane in any febrile child? What are you looking for?
To look for otitis media, a common infection of the middle ear. In acute OM the tympanic membrane would be bright red and bulging with the loss of normal light reflection. Occasionally, there is acute perforation of the eardrum with pus visible in the external canal.
What are two serious but rare complications of otitis media?
Mastoiditis
Meningitis
How is otitis media managed?
Analgesia - paracetamol, ibuprofen, regular until acute inflammation has resolved
most cases resolve spontaneously. Amoxicillin may be prescribed for the parent to use if the child remains unwell after 2-3 days.
recurrent OM can lead to OME.
What is glue ear?
Recurrent OM can lead to glue ear aka otitis media with effusion (OME)/ secretory OM/ serous OM, is the accumulation of fluid in the middle ear without pain and fever sx of OM.
How does OME present?
Asymptomatic apart from possible decreased hearing/inattentiveness.
How does OME appear on examination of the ear?
Eardrum is dull and retracted, often with a fluid level visible.
How is OME diagnosed?
Tympanometry - flat trace +
Pure tone audiometry - conductive loss.
What age groups tend to be affected by OME?
2-7 years, peak between 2.5 and 5 yrs.
How is OME managed?
Like OM, should resolve spontaneously and there is no evidence for abx.
If there is conductive hearing loss, grommets may be beneficial.
Cochrane review suggests adenoidectomy may have better long term benefit - adenoids can harbour organisms with biofilms that contribute to infection spreading to the eustachian tubes. Grossly hypertrophied adenoids may also obstruct the eustachian tubes –> poor ventilation of the middle ear, recurrent infection.
Which type of hearing loss is more common and associated with better prognosis?
Conductive hearing loss.
Which type of hearing loss is associated with profound hearing loss and does not tend to improve?
Sensorineural (rare)
What are the most common causes of sensorineural hearing loss?
Inherited syndromes: Usher syndrome, Waardenburg syndrome. Also Alport, Pendred, Jewel-Lang-Niellsen
What part of the auditory pathway is affected in conductive hearing loss?
Conductive hearing loss is due to an abnormality in the external or middle ear. Usually middle ear, often otitis media with effusion.
What part of the auditory pathway is affected in sensorineural hearing loss?
Inner ear: cochlea/ auditory nerve.
How is sensorineural hearing loss managed?
Amplification with hearing aids
Cochlear implant if insufficient amplification
Usually can be taught in a mainstream school with assistance from peripatetic (visiting) specialist teacher.
Makaton may be useful if also learning disabled.
How is conductive hearing loss managed?
See OME treatment - usually resolves spontaneously but grommets +/- adenoidectomy may be indicated.
What are tympanostomy tubes?
A tympanostomy tube aka grommet/myringotomy tube, is a small tube inserted into the eardrum in order to keep the middle ear aerated for a prolonged period of time, and to prevent the accumulation of fluid in the middle ear.
When are tympanostomy tubes used?
Children with recurrent URTIs and chronic OME that do not resolve with conservative measures.
What is strabismus?
aka squint. Misalignment of the visual axes of the two eyes so they appear to point in different directions.
What causes squint?
Most common type is concomitant, which is usually due to refractive error. The refractive error is usually hypermetropia (short-sightedness). It means the image on the retina is distorted, so the eye tries to compensate.
Also exclude paralytic squint (paralysis of motor nerves;), cataracts, retinoblastoma, other intra-ocular causes.
Pseudosquint can occur when there is a wide epicanthus, and will have normal equal light reflection in both pupils.
How would you investigate a squint in an infant?
Corneal light reflexes:
Pseudosquint can occur when there is a wide epicanthus, and will have normal equal light reflection in both pupils.
Specialist test: cover test, done at 6m and 33cm. The squinting eye moves to become ‘normal’ when the normal (‘fixing’) eye is covered.
Alternating cover test for latent squint: eyes appear normal, but when covered, the squinting eye deviates, and you can see this when the cover is removed.
Describe the management of squint.
Orthoptist + ophthalmic surgeons
Relieve any deprivation eg ptosis/cataract
Correct refractive error: convex lens for hypermetropia
Patch ‘good’ eye to train up the ‘lazy’ eye.
Eye muscle exercises
Refer to opthalmologist if squint is divergent, paralytic of persistent after 2 months of age.
When is squint normal?
Transient neonatal misalignment: <3 months of age, child still learning to see, grows out of it. Only squints occasionally.
What is peri-orbital cellulitis caused by?
Infection of the peri-orbital skin due to s. aureus, or if not immunised, H influenza B.
[Oxford]
What should you suspect in a child with fever, erythema and recent dental abscess?
Peri-orbital cellulitis. May also be systemically unwell, with tenderness and oedema over the orbit.
How is peri-orbital cellulitis managed?
Risk of developing to orbital cellulitis which is an emergency. Consider CT to exclude abscess. IV abx 5-7 days.
How would you identify orbital cellulitis and how would you manage it?
Ocular proptosis, limited ocular movement, decreased visual acuity.
High dose IV 2nd gen cephalosporin (eg cefuroxime) + metronidazole if over 8 yrs.
CT to detect abscess which requires surgical drainage.
Why would you do a CT for peri-orbital cellulitis?
Exclude intracranial abscess, meningitis, cavernous sinus thrombosis, or developing orbital cellulitis.
Describe the normal changes in circulation that occur when a baby is born.
In the fetus, the pressure in the right atrium is higher than that in the left atrium, because the R atrium receives blood from the placenta while the L atrium receives little blood from the fetal lungs. The foramen ovale is open and allows blood to flow from the left atrium into the right atrium. Therefore blood is basically mixed.
As the infant takes its first breaths, there is increased pressure in the L atrium because of increased blood through the lungs, and decreased pressure in the R atrium because there is no longer placental input. This different pressure gradient causes the foramen ovale to close, forming two separate circulations as in the adult.
What is the foramen ovale? When should it close?
Connects the right and left atria in the fetus. Should close immediately at birth. Pathology: PFO/ASD.
What is the ductus arteriosus? When should it close?
Connects the pulmonary artery to the aorta in the fetus. Should close within days of birth. Pathology: PDA.
What is VSD and how does it present?
Ventricular septal defect. Hole in ventricular wall. Pressure in L>R so you get L to R shunt at first, which is acyanotic, but the R heart strain can lead to R to L shunt and cyanosis (Eisenmenger syndrome)
How would you distinguish between a small and large VSD, and which is more common?
Small is more common (80%). Both cause a pansystolic murmur at LLSE but the louder the murmur the smaller the hole, so small VSD = loud murmur.
A large VSD (20%) would be symptomatic, with HF, SOB, FTT, chest infections. Tachypnoeic and tachycardic, hepatomegaly due to HF, active precordium. and apical mid-diastolic murmur due to increased flow across the mitral valve after blood has circulated through the lungs.
What is the management for a small VSD?
It should close spontaneously, which you can identify as the murmur disappears, echocardiogram is normal.
In the meantime keep good dental hygiene to decrease the risk of IE.
What is the management for a large VSD?
Diuretics for heart failure,
captopril for risk of pulmonary hypertension
calories for FTT
surgery at 3-6 months for risk of pulmonary hypertension
What complications are associated with large VSDs?
Pulmonary hypertension due to high pulmonary blood flow. This will ultimately lead to irreversible damage of the pulmonary capillary vascular bed –> risk of Eisenmenger syndrome.
Therefore surgery at 3-6 months of age.
Give 3 signs on X ray for VSD.
Cardiomegaly
Enlarged pulmonary arteries
increased pulmonary vascular markings
pulmonary oedema
What is Eisenmenger syndrome?
High pulmonary blood flow due to a large L to R shunt (eg large VSD, ASD or PDA) or common mixing is not treated, the pulmonary arteries become thick walled, and there is increased resistance to flow (pulmonary hypertension).
Later in life (as quickly as 2 years in large shunts but usually teenagers) the shunt reverses, and the patient becomes blue, which is Eisenmenger syndrome. This situation is progressive and the adult will die in R heart failure in their 30s/40s.
Can develop more quickly in pregnancy.
What are the main types of atrial septal defect and which is most common?
Mainly ostium secondum: septum secundum fails to close
Ostium primum: Septum primum fails to close. This is an AVSD
PFO - foramen ovale fails to close. Not technically an ASD
How does atrial septal defect present?
Asymptomatic.
May have recurrent chest infections/ wheeze. arrhythmias in later life.
Signs - mid-systolic crescendo-decrescendo murmur at upper left sternal edge due to increased blood flow across the pulmonary valve.
Split s2 due to RV stroke volume being equal in inspiration and expiration because blood flowing across ASD. Doesn’t vary with inspiration/expiration.
Give 3 signs of atrial septal defect on CXR.
Cardiomegaly, enlarged pulmonary arteries, pulmonary vascular markings.
Give 4 complications of atrial septal defect.
Paradoxical embolism - normally a clot would go to the lungs, but because the atria connect, the clot could go into the arterial circulation to the brain.
Atrial fibrillation or flutter (adults may require anticoagulation)
Pulmonary hypertension/ right heart failure
Eisenmenger syndrome
Give 3 heart defects that can cause a right to left shunt and therefore cyanotic heart disease.
VSD, ASD, PDA, TGA.
What is patent ductus arteriosus?
Ductus arteriosus fails to close by 1 month after due date.
Blood therefore flows from the descending aorta to the pulmonary artery (L to R), causing right and then left heart strain and hypertrophy.
RFs: Rubella, prematurity, genetics.
Which side of the heart contains oxygenated blood?
The left. Therefore LV is stronger. An L to R shunt means arterial blood gets into the pulmonary circulation.
How does PDA present?
Continous crescendo-decrescendo ‘machinery’ murmur beneath left clavicle. Murmur continues into diastole because the pressure in the pulmonary artery is lower than that in the aorta throughout the cardiac cycle.
Pulse pressure decreased –> collapsing/bounding pulse.
Usually asymptomatic, closes on its own, or may get pulmonary hypertension and HF later in life.
How is PDA usually diagnosed?
Echocardiography. X ray and ECG normal or similar to VSD if duct is large.
What is the management for PDA?
Monitor until 1 yr using echo. At 1 yr it is unlikely to close spontaneously so you close it with transcatheter or surgical closure to abolish risk of IE and pulmonary vascular disease.
Patients who are sympatomatic/ have HF are treated earlier.
Give 2 examples of right to left shunts. Which is the most common?
Tetralogy of fallot (most common)
Transposition of the great arteries
Cyanosis in the first week of life with o2 <94% is likely to be due to which type of cardiac abnormality?
Right to left shunt, eg tetralogy of fallot/TGA. L to R shunt eg septal defect would cause breathlessness, and common mixing would cause breathlessness with cyanosis.
What is the hyperoxia test?
Aka nitrogen washout. Used to determine presence of heart disease in a cyanosed neonate. Infa t put on 100% oxygen for 10 min, if PaO2 remains low, they have cyanotic congenital heart disease, as long as lung disease and persistent pulmonary hypertension of the newborn have been excluded.
What is the management for a duct-dependent lesion?
Duct-dependent means cyanotic eg ToF, TGA.
ABCDE
Prostaglandin infusion (PGE)
Usually duct-dependent so need to maintain ductal patency to survive.
What are the 4 cardinal features of tetralogy of fallot?
- Large VSD
- Overriding aorta
- Subpulmonary stenosis –> right ventricular outflow tract obstruction –>
- Right ventricular hypertrophy.
Give 3 signs of tetralogy of fallot on X ray.
Small heart
Pulmonary artery ‘bay’: L heart border lack of normal pulmonary artery and RV outflow tract.
Uptilted apex –> ‘boot shaped’
Oligaemic lung fields
What is Marfan syndrome and how is it managed?
AD condition --> lack of fibrillin --> abnormal connective tissue.
Features:
Tall stature
Long neck
Long limbs
Long fingers (arachnodactyly)
High arch palate
Hypermobility
Pectus carinatum or pectus excavatum
Downward sloping palpable fissures
Associated Conditions:
Lens dislocation in the eye
Joint dislocations and pain due to hypermobility
Scoliosis of the spine
Pneumothorax
Gastro-oesophageal reflux
Mitral valve prolapse (with regurgitation)
Aortic valve prolapse (with regurgitation)
Aortic aneurysms
Management:
Surgical correction of valve disorders
Minimise the blood pressure and heart rate to minimise the stress on the heart and the risk of complications developing - avoid intense exercise, caffeine and other stimulants, give BB/ARB
Pregnancy - risk of developing aortic aneurysms and associated complications.
Physiotherapy
Genetic counselling
Yearly echocardiograms and review by an ophthalmologist.
How does tetralogy of fallot present?
What would be seen on ECG?
Antenatal diagnosis/murmur (loud harsh ejection systolic murmur at left sternal edge, getting shorted with time as the R ventricular outflow tract gets more obstructed)
If untreated, it can present late with severe cyanosis, hypercyanotic spells and squatting on exercise.
ECG: Right ventricular hypertrophy. Upright T wave in V1 with ‘pure’ R wave (no S wave)
How is tetralogy of fallot managed?
Medical
Neonatal cyanosis - subclavian artery-pulmonary artery shunt to increase pulmonary blood flow.
Surgery at 6 months to close VSD and relieve RV outflow tract obstruction.
An infant with tetralogy of fallot has cyanosis, irritability, hypoxia, pallor, and breathless for 15 minutes. What is the diagnosis and management?
Hypercyanotic spell. Important to identify as can lead to MI/CVA/death.
Usually left limiting and followed by period of sleep but if >15 min, treat: sedation, morphine, IV propranolol, fluids, bicarb to correct acidosis. Muscle paralysis and artificial ventilation in order to reduce metabolic oxygen demand.
What is transposition of the great arteries?
The aorta is connected to the right ventricle and the pulmonary artery is connected to the left ventricle (should be the other way around). Therefore the deoxygenated blood goes into the circulation and the already oxygenated blood goes back to the lungs.
Why is TGA in theory incompatible with life? And why do many infants still survive?
No oxygen going around the body, so should not allow life. There is co-occurring anomaly that allows mixing of the oxygenated and deoxygenated blood eg septal defect.
Also therapeutic interventions to allow mixing.
How does TGA present?
Cyanosis
Day 2 of life as ductal closure leads to marked reduction in mixing of desaturated and saturated blood.
Loud 2nd heart sound
What does TGA look like on X ray?
Narrow upper mediastinum with ‘egg on side’ appearance due to anteroposterior relationship of the great vessels, narrow vascular pedicle and hypertrophied right ventricle.
Increased pulmonary vascular markings due to increased pulmonary blood flow.
How is TGA managed?
Improve mixing to improve cyanosis - maintain patent ductus arteriosus with prostaglandins.
Balloon atrial septostomy - catheter passed into the R atrium –> foramen ovale –> inflated in the L atrium then pulle dback through the atrial septum. This tears the septum to the blood can mix.
Arterial switch operation in first days of life.
Give 3 causes of hepatomegaly.
Infection - hepatitis
Haematological - sickle cell/thalassaemia
Liver disease - polycystic disease
Malignancy - leukaemia
Metabolic - glycogen and lipid storage disorders
Heart failure
Apparent: chest hyperexpansion from bronchiolitis or asthma
[Lissauer]
What is heart failure?
When the heart is unable to pump sufficiently to maintain blood flow to meet the body’s needs.
What is the likely cause of heart failure in a neonate?
Left heart obstruction, such as coarctation of the aorta, aortic valve stenosis, interruption of the aortic arch etc. If the obstructive lesion is severe, arterial perfusion may be achieved by right to left shunting of blood via the arterial duct. This is called ‘duct dependent systemic circulation’.
How might heart failure present?
Symptoms:
Breathlessness (particularly on feeding or exertion), sweating, poor feeding, recurrent chest infections.
What are the signs of heart failure?
Poor weight gain, tachypnoea, tachycardia, murmur, gallop rhythm, enlarged heart, hepatomegaly, cool peripheries
[Lissauer]
What is the most common cause of heart failure in a 3 month old?
Large septal defect (VSD, ASD, PDA) causing high pulmonary blood flow.
As the pulmonary vascular resistance falls over weeks, there is a progressive increase in L to R shunt and increasing pulmonary blood flow. This causes pulmonary oedema and breathlessness.
The pulmonary vascular resistance rises in response to the L to R shunt which may make the symptoms better but will eventually lead to Eisenmenger syndrome (irreversibly raised pulmonary vascular resistance).
What is the most common cause of heart failure in an older child?
L heart failure: Rheumatic cardiac disease, cardiomyopathy.
R heart failure: Eisenmenger syndrome
How is heart failure managed?
Identify cause and quantify function using CXR (cardiac enlargement, lung oedema), echo (congenital heart defect), ABG (metabolic acidosis, low pO2), ECG, U+Es (hyponatraemia due to water retention)
Treat underlying cause
Bed rest, semi-upright, O2 (unless L-R shunt), calories, diuretics, ACE inhibitors.
What are the features of an innocent heart murmur?
5 Ss: systolic short situational (exercise/fever/quiter on standing) symptomless soft
What is rheumatic fever?
The most important cause of heart disease in children worldwide; rare in UK due to abx and reduced virulence. Abnormal immune response to preceding infection with group A beta-haemolytic step (strep pyogenes). Mainly affects children aged 5-15.
How does rheumatic fever present? (hint: Jones)
Pharyngeal infection –> 2-6 weeks LATENT interval –> present with infection, polyarthritis, mild fever and malaise.
JONES:
Joint inflammation
<3 heart damage - new heart murmurs
Nodules on elbows, knees and forearm
Erythema marginatum: rash with thick red margins
Sydenham’s chorea: rapid involuntary movement of face and hands.
What is chronic rheumatic heart disease?
Long term damage from scarring and fibrosis of valves. Usually mitral stenosis. Can occur due to repeated attacks of rheumatic fever with carditis. Present in adult life.
How is acute rheumatic fever managed?
While there is active myocarditis (ECG changes with a raised ESR), bed rest essential.
Anti-inflammatory agents - aspirin high dose, serum levels monitored.
If not resolving, give corticosteroids.
HF - diuretics, ACEIs
Pericardial effusions - pericardiocentesis
Anti-step abx if persisting infection.
How is recurrence of rheumatic fever prevented?
Monthly benzathine penicillin injections
Can be given orally but compliance may be a problem
Oral erythro if penicillin allergy
Until adulthood (but maybe lifelong - controversial.)
Which children are at highest risk of IE?
Congenital heart disease (except secundum ASD)
Turbulent jet of blood (eg VSD), co-arctation of aorta, PDA, prev rheumatic fever, or if prosthetic material inserted at surgery.
Give 5 signs of IE.
Sustained fever Malaise Raised ESR unexplained anaemia or haematuria Pallor Peripheral stigmata - clubbing, splinter haemorrhages in nailbed, necrotic skin lesions Neuro signs from cerebral infarction Retinal infarcts (Roth spots) Arthralgia Murmur (change in character over time)
How is IE diagnosed?
High index of suspicion
Bloods: FBC (raised WCC), ESR (raised)
Repeated blood culture
Echocardiogram - valve vegetations
What is the most common pathogen associated with infective IE? How does it get in to the blood?
Streptococcus viridans
50% of cases. often after dental procedures.
AKA a-haemolytic strep.
Which pathogen can cause IE and is associated with central venous catheters?
Staphylococcus aureus.
How is IE managed?
High dose penicillin/vancomycin + aminoglycoside ASAP
6 weeks IV. Checking serum level of abx will kill the organism.
Surgical removal of infected prosthesis
What is the prophylaxis for IE?
Good dental hygiene
NOT abx in the UK
What is an arrhythmia?
Cardiac arrhythmias are accelerated, slowed, or irregular heart rates caused by abnormalities in the electrical impulses of the myocardium.
[AH youtube]
What type of arrhythmia is normal in children?
Sinus arrhythmia.
How would you initially manage an arrhythmia?
ECG (24hr if intermittent arrhythmia)
Detailed hx + ex
Echocardiogram for underlying congenital heart disease.
What causes supraventricular tachycardia?
SVT is the most common abnormal arrhythmia of childhood. Re-entry within the AVN is the most common mechanism.
[Oxford]
How does SVT present?
Heart failure
Why is SVT concerning?
Can cause poor cardiac output, pulmonary oedema, hydrops fetalis, intrauterine death.
What is re-entry tachycardia?
Mechanism of most SVTs.
Circuit of conduction is set up with premature activation of the atrium via an accessory pathway.
How is SVT managed?
ABCDE
correct tissue acidosis
pos pressure ventilation
vagal stimulating manoeuvres eg carotid sinus massage, cold ice pack to face
IV Adenosine -terminates tachycardia by breaking the re-entry circuit that is set up between the AVN and accessory pathway. Incrementally increasing dose.
if that fails, electrical cardioversion with a synchronised DC shock.
Maintenance therapy until 1 yr of age: fecainide or sotalol
Digoxin, + propranolol if delta waves.
resting ECG will remain abnormal but recurrence is only 10%.
What changes on ECG would be seen with SVT?
Narrow complex tachycardia 250-300bpm
P wave due to retrograde activation of the atrium via the accessory pathway
if HF severe, may have T wave inversion - myocardial ischaemia.
Sinus rhythm: short PR
WPW: short PR interval, delta wave due to antegrade activation of ventricle via the pathway.
What is wolff-parkinson-white syndrome?
Pre-excitation syndrome predisposing to SVT. Abnormal re-entry circuit of the AVN and an accessory conduction pathway connecting atrium to ventricle on L or R lateral cardiac border or within the ventricular septum.
Asso post-surgical repair, cardiomyopathy and Ebstein anomaly.
What is Ebstein anomaly and what syndrome is it associated with?
Congenital heart defect in which the septal and posterior leaflets of the tricuspid valve are displaced towards the apex of the right ventricle of the heart. Basically the RV and RA are one.
Asso with WPW syndrome.
What would be seen on ECG with WPW?
Short PR and delta wave (slow upstroke of QRS complex).
How is WPW managed?
Assessment to ensure they cannot conduct quickly, using atrial pacing. Reduces small chance of sudden death.
If relapse/high risk, percutaneous radiofrequency ablation or cryoablation of the accessory pathway.
Which antibodies are associated with congenital complete heart block?
Anti-Ro or anti-La abs in maternal serum.
Which arrhythmia can rarely occur in babies born to mothers with connective tissue disease?
Congenital complete heart block. Subsequent pregnancies are often affected.
What is congenital complete heart block, and what does it look like on ECG?
Anti-Ro or anti-La abs in maternal serum prevent normal development of the electrical conduction system in the developing heart, with atrophy and fibrosis of the AVN. Needs endocardial pacemaker.
Dissociated P waves and QRS complexes.
Give 2 causes of fetal hydrops.
SVT
rarely - complete congenital heart block
How can complete congenital heart block present in childhood?
Presyncope or syncope
Most remain symptom free for many years
Why must you do an ECG in a child with sudden LOC during exercise/stress? What are you looking for?
Long QT syndrome.
Misdiagnosed as epilepsy.
If unrecognised, sudden death from ventricular tachycardia may occur.
What causes long QT syndrome?
One of a group of channelopathies caused by specific gene mutations with gain or loss of function (others: Brugada, short QT) Autosomal dominant Drugs - erythromycin Electrolytes Head injury
What should you consider in a child with bile stained vomit?
Intestinal obstruction (malrotation with volvulus)
What should you consider in a child with haematemises?
Oesophagitis
Peptic ulceration
Oral/nasal bleeding
What should you consider in a child with projectile vomiting in the first few weeks of life?
Pyloric stenosis - look for olive sign, not keeping anything down. obstruction at the gastroduodenal junction.
What should you consider in a child with vomiting at the end of paroxysmal coughing?
Whooping cough (pertussis)
What should you consider in a child with abdominal tenderness and vomiting?
Surgical abdomen eg appendicitis - pain on movement
What should you consider in a child would abdominal distension and vomiting?
Intestinal obstruction including strangulated inguinal hernia
What should you consider in a child with hepatosplenomegaly and vomiting?
Chronic liver disease
What should you consider in a child with bloody stool?
Intussusception
Gastroenteritis - salmonella or campylobacter
Check kidneys and Hb for HUS.
What could cause severe dehydration and shock in a vomiting child?
Severe gastroenteritis
systemic infection
DKA
What could cause vomiting with seizures?
Raised ICP (fontanelle bulging)
What could cause failure to thrive and vomiting?
Gastro esophageal reflux
Coeliac
Chronic GI conditions
UTI
What is gastro-oesophageal reflux? Why does it happen?
Common in infancy, involuntary passage of gastric contents into the oesophagus, due to inappropriate relaxation of the lower oesophageal sphincter. Functional immaturity in infants.
What is the prognosis of reflux?
Resolves by 1 year due to maturation of lower oesophageal sphincter, upright position and more solids in the diet.
Who is at increased risk of severe GE reflux?
Neuromuscular problems
CP
Surgery to oesophagus or diaphragm
Preterm, bronchopulmonary dysplasia
How is GE reflux managed?
- Upright positioning
- Feed thickening with nestargel, carobel
- omeprazole (PPI)
- ranitidine (H2 receptor antagonist)
- Surgery: Nissen Fundoplication, in which fundus is wrapped around the oesophagus.
Ix:
may need 24hr pH monitoring to investigate degree; endoscopy to identify oesophagitis/exclude other causes
Give 3 complications of GE reflux.
FTT from severe vomiting
oesophagitis - haematemesis, discomfort on feeding or heartburn, iron deficiency anaemia
recurrent pulmonary aspiration - pneumonia, cough/wheeze, apnoea if preterm
Sandifer syndrome: dystonic neck posturing
Apparent Life Threatening Events
What is pyloric stenosis and who is at most risk?
Hypertrophy of pyloric muscle causing gastric outlet obstruction.
2-7 weeks of age, boys (4:1), first-borns, FHx especially on maternal side.
Give 3 clinical features of pyloric stenosis.
Vomiting, increasing in frequency and forcefulness until projectile
Hunger after vomiting until too dehydrated to want to feed
Wt loss if presentation delayed
Hypochloaemic metabolic alkalosis with low plasma sodium and potassium as a result of vomiting stomach contents.
How is the diagnosis of pyloric stenosis confirmed?
TEST FEED: NG tube insertion and aspiration to empty the stomach, small feed of dioralyte, feel for gastric peristalsis from left to right and ‘olive’ pyloric mass in the RUQ.
Ultrasound can confirm dx if in doubt.
How is pyloric stenosis managed?
- Correct fluid and electrolyte disturbance with IV fluids (saline, dextrose, KCl)
- Pyloromyotomy
What is colic?
A term used to describe paroxysmal inconsolable crying or screaming, drawing up of the knees, flatus particularly in the evening.
Occurs in 40% of babies. First few weeks of life and resolves by 4 months.
Benign, but may precipitate NAI as so frustrating and worrying for parents.
How is colic managed?
It is benign but worrying to parents so deal with this. Give support and reassurance.
If severe and persistent consider CMPA or GER - empirical 2 week trial of whey hydrolysate formula followed by trial of anti reflux treatment.
What is the most common surgical cause of abdominal tenderness in children?
Appendicitis. High index of suspicion, dont delay rx as risk of perforation.
Give 3 extra-abdominal causes of abdo pain
URTI Lower lobe pneumonia (referred pain) TESTICULAR TORSION hip and spine therefore always check these things
Give 3 medical causes of abdo pain.
HSP Sickle cell disease Hepatitis IBD Constipation Gynecological Psychological Lead poisoning Nephrotic syndrome/liver disease --> ascites --> peritonitis DKA UTI
What is the typical age for appendicitis?
Children over 3 years old.
How does appendicitis present?
Variable - high index of suspicion eg in children already diagnosed with gastroenteritis/UTI, pre-school children in particular are easy to underestimate for appendicitis, and if the appendix is retrocoecal, localised guarding may be absent.
sx: Anorexia, vomiting, abdo pain starting central then localising to right iliac fossa due to localised peritoneal inflammation
Signs: flushed, halitosis, fever 37.2-38, abdo pain aggravated by movement, McBurney’s point tenderness with guarding (RIF)
How is appendicitis diagnosed?
Progressive so monitor every few hours.
Neutrophilia
USS may support dx - thickened, non-compressible appendix with increased blood flow, or exclude other pathology.
Sometimes diagnostic lap but not routine.
Essentially clinical dx.
How is appendicitis managed?
Appendicectomy
If uncomplicated, straightforward.
If complicated (appendix mass, abscess or perforation), give fluid resus and IV abx first.
If no signs of peritonitis, may give IV abx and do appendicectomy weeks later.
What is the most common cause of failure to thrive?
Inadequate food intake (due to poverty in other countries, poor feeding here)
Give 3 dietary strategies for improving food intake
3 meals and 2 snacks a day
increase number and variety of foods offered
increase energy density of usual foods (eg add cheese, margarine, cream)
Decrease fluid intake, particularly squash
Give 3 behavioural strategies for improving food intake
Meals at regular times with other family members
Praise when food is eaten
Gently encourage child to eat, but avoid conflict and never force feed!
[Lissauer/ Wright 2000]
What is marasmus?
Weight/height less than 70%, wasted appearance (thin but not stunted). Due to severe protein-energy malnutrition. Often withdrawn and apathetic.
What is Kwashiorkor?
Severe wasting with generalised oedema due to severe protein malnutrition. The weight may not be severely reduced due to the oedema.
Give 3 signs of kwashiorkor.
‘Flaky-paint’ rash with hyperkeratosis (thickened skin) and desquamation
Distended abdo, enlarged liver (usually due to fatty infiltration
Angular stomatitis
Sparse, depigmented hair
Diarrhoea
Hypothermia
Bradycardia
Hypotension
Low albumin, potassium, glucose and magnesium
Which condition occurs in protein-energy malnutrition and is more common when infants are not weaned from the breast until 1 year, diet is high in starch, and/or following measles/gastroenteritis?
Kwashiorkor. Hyperkeratosis, oedema, diarrhoea.
How is severe acute malnutrition treated?
Correct hypoglycaemia
Correct hypothermia by wrapping
Correct dehydration with fluids but carefully because risk of HF
Correct electrolyte deficiencies, especially K
Abx as fever may be absent in infection. Treat oral thrush if present
Micronutrients: Vit A and others
Initiate feeding - small volumes, frequently, including through the night.
Which condition involves weight for height more than 3 SDs below the mean, with wasting and apathy?
Marasmus
What causes rickets? How is it different to osteomalacia?
Deficient intake/ Defective metabolism of Vit D –>
–> low serum calcium –> PTH secretion –> normalises serum calcium but GROWING bone/osteoid tissue fails to mineralise –> Rickets
Osteomalacia is failure of MATURE bone to mineralise.
How does vit D deficiency present?
Usually bone deformity, rickets
Hypocalcaemia - Seizures, neuromuscular irritability (tetany), apnoea, stridor. This is more common before 2 years and in adolescence, because high demand for calcium in rapidly growing bone results in hypocalcaemia before rickets.
What causes low phosphate and why is this a problem?
Vit D def –> low Ca –> more PTH.
PTH causes renal losses of phosphate –> high PTH causes low phosphate. Low phosphate reduces bone calcification.
Give 3 causes of vitamin D deficiency.
Malabsorption - IBD, coeliac, CF
Drugs - phenobarbital, phenytoin - vit D metabolism impaired because they deplete renal and hepatic enzymes
Hepatic/renal disease - impaired Vit D metabolism
Nutritional - in the UK this is in Asian/Black infants exclusively breast-fed into late infancy.
Give 3 clinical features of rickets.
Craniotabes: ping-pong ball sensation of the skull when pressing on the occipital or posterior parietal bones.
Palpable constochondral junctions (rachitic rosary)
Widened wrists and ankles
Horizontal depression on lower chest (Harrison sulcus)
Bow-legged
What investigations would you do for ?rickets and what would they show?
Dietary hx for vit and Ca intake
Bloods - serum Ca low/normal, phosphorus low, 25-hydroxyvit D low, plasma alk phos high, PTH high.
X ray: wrist cupping, metaphysial fraying, widened epiphyseal plate.
What are the consequences of vit A deficiency?
Blindness
Eye damage: xerophthalmia, starts with night blindness, then corneal ulceration and scarring
Increased susceptibility to infection especially measles.
Give 3 complications of childhood obesity.
Slipped upper femoral epiphysis (SUFE) Tibia vara (bow legs) Abnormal food structure and function IIH Hypoventilation syndrome Gallbladder disease PCOS T2DM Hypertension Abnormal blood lipids Asthma Malignancy Psychological - depression, low self esteem
Give 3 endogenous causes of obesity. What would indicate these?
Short and obese: Hypothyroidism, Cushing syndrome (Tall –> overnutrition likely)
Prader-Willi syndrome - learning diasbility, dysmorphic, hyperphagia, poor linear growth, hypotonia, undescended testes.
Leptin deficiency - under 3 years
Give 2 drug treatments of obesity and when they would be used.
After dietary, exercise and behavioural approaches (NICE)
Orlistat - lipase inhibitor –> steatorrhea. Reduce fat intake to avoid GI side effects. Consider if fat intake high.
Metformin - consider if insulin insensitivity.
What is intussusception?
Telescoping of proximal bowel into a distal segment, usually ileo-caecal. Commonest cause of intestinal obstruction in infants after neonatal period.
Who is most at risk of intussusception?
3 months- 2 years.
Why is it important to identify intussusception?
Stretching and constriction of the mesentery results in venous obstruction, causing engorgement and bleeding from the bowel mucosa, fluid loss and subsequently bowel perforation, peritonitis and gut necrosis. Needs to be reduced and resuscitated.
What would you see on examination with intussusception?
Sausage shaped mass in the abdomen Redcurrant jelly stool - blood stained mucus. late sign but characteristic. Abdo distension Shock Vomiting Colicky pain and pallor
How is intussusception managed?
Fluid resuscitation, paediatric surgeon involved
Radiological reduction with air.
If unsuccessful or perforation occurs (25%), operative reduction is done.
What is Meckel diverticulum?
An ileal remnant of the vitellointestinal duct which contains ectopic gastric mucosa or pancreatic tissue. Present in 2%.
How does Meckel diverticulum present?
Usually asymptomatic but can present with:
Severe rectal bleeding - may be lifethreatening
intussusception
Volvulus
Diverticulitis - mimics appendicitis.
How is Meckel diverticulum treated?
Surgical resection
What causes malrotation?
The mesentery not being fixed at the duodenogejunal flexure or ileocaecal region, or base is shorter than normal, during rotation of the small bowel in fetal life. Makes it predisposed to volvulus. Ladd bands may cross the duodenum and contribute to bowel obstruction there.
What investigation should you do in a child presenting with dark green vomiting?
Upper GI contrast study to assess intestinal rotation
Vascular compromise? –> urgent laparotomy.
How is malrotation treated?
Operation to untwist the volvulus and broaden the mesentery (doesn’t actually correct the malrotation). Also remove the appendix because they are likely to present in future with appendicitis-like sx so this reduces future diagnostic confusion.
What is the most common age of presentation with malrotation?
1-3 days of life, but can present at any age with obstruction.
Give 3 clinical features of malrotation other than green vomit.
Abdo pain
Tenderness from peritonitis or ischaemic bowel
May have vascular compromise as well- if this is present do urgent laparotomy.
What might be the cause of peri-umbilical pain in a child for at least 3 months?
This is recurrent abdo pain (RAP)
Affects 10% of school age childen
Cause not identified in >90%
Identifiable causes: IBS, abdominal migraine or functional dyspepsia.
What investigations would you do in a child with recurrent abdominal pain?
Try to avoid invasive tests.
Check growth
Urine microscopy and culture
+/- abdo USS for gallstones/PUJ obstruction
What is the likely diagnosis in a child with headaches, midline abdominal pain and vomiting?
Abdominal migraine.
What is irritable bowel syndrome (IBS)?
Disorder of altered gastrointestinal motility (very forceful contractions) and abnormal sensation of intra-abdominal events. Both of these factors are modulated by stress and anxiety.
Give 3 clinical features of IBS.
Abdo pain often worse before or relieved by defecation
Explosive, loose or mucousy stools
bloating
feeling of incomplete defecation
constipation, often alternating with normal or loose stools
Which organism predisposes duodenal ulcers?
H. pylori.
Abdo pain, nausea
How is peptic ulceration treated?
PPI eg omeprazole
If H.pylori on C13 breath test, amoxicillin + metronidazole/clarithromycin.
What is the likely diagnosis in a child with abdo pain, nausea, early satiety, bloating and postprandial vomiting?
Functional dyspepsia
Treat with hypoallergenic diet
What is the most likely cause of gastroenteritis in the UK?
Rotavirus - 60% of cases in children <2 years of age
Particularly winter and early spring
There is a vaccine but it is not given nationally.
What is the most likely causative organism in a child with blood and pus in the stool, pain and tenesmus (cramping rectal pain)?
Shigella - high fever.
Salmonella
What is the likely causative organism in someone with severe abdo pain, diarrhoea and bloody stool?
Bacterial (bloody)
Campylobacter jejuni
What causative organism would you suspect in someone with profuse, rapidly dehydrating diarrhoea?
Cholera
Enterotoxigenic E. Coli (ETEC)
Give 5 differentials for a child with abdo pain, vomiting and diarrhoea.
Gastroenteritis
Systemic infection - septicaemia, meningitis
Local infection - resp, otitis media, hep A, UTI
Surgical - Pyloric stenosis, intussusception, acute appendicitis, necrotising enterocolitis, hirschsprung disease
Metabolic - DKA
Renal - HUS
Coeliac
CMPI
Adrenal insufficiency
How is gastroenteritis treated?
Correct (ideally prevent) dehydration!
ORT
When would you get hyponatraemic dehydration and what can be the result?
When children with diarrhoea drink large quantities of water/hypotonic solutions, so net loss of sodium, leading to hyponatraemic dehydration.
Leads to shift of water from extra- to intracellular compartments –> increase in brain volume –> convulsions.
Greater degree of shock per unit of water loss.
Give 5 red flag signs of shock from dehydration.
Decreased consciousness Sunken eyes Tachycardia Tachypnoea Reduced skin turgor
When would you get hypernatraemic dehydration?
If water loss exceeds sodium loss.
Due to high insensible water losses (fever, hot environment), or profuse, low-sodium diarrhoea.
ECF becomes hypertonic with respect to intracellular fluid –> water going extracellular –> brain shrinkage with rigid skull, jittery movements, increased muscle tone, seizures.
Masks symptoms of dehydration eg sunken eyes.
When would you do a stool culture in a child with symptoms of gastroenteritis?
Required if child appears septic, blood or mucus in stool, or immunocompromised.
Consider if recent foreign travel, diarrhoea not improved by day 7, or dx uncertain.
When would you do blood cultures in a child with gastroenteritis?
If abx are started.
Why must fluids be given slowly in hypernatraemic dehydration?
(0.5mmol/L/hr) because rapid reduction in plasma [Na+] and osmolality –> water into cerebral cells –> cerebral oedema.
Why are anti-diarrhoeal and anti-emetic drugs NOT used for gastroenteritis?
Ineffective, prolong excretion of bacteria in stools, side-effects, cost, attention should be on oral rehydration.
When are abx used in gastroenteritis?
Not all bacterial gastroenteritis - abx are only indicated for sepsis, extra-intestinal spread of bacterial infection, salmonella in <6 month old, malnourished, immunocomp.
Most organisms: ampicillin, co-trimoxazole.
C. diff (asso. pseudomembranous colitis) - vancomycin/metronidazole.
After gastroenteritis, introduction of normal diet may cause watery diarrhoea. What could have caused this?
Temporary lactose intolerance.
‘Clinitest’ will be positive for non-absorbed sugar in stools.
ORT again for 24h and try normal diet again.
What is the pathophysiology of coeliac disease?
The gliadin fraction of gluten provokes a damaging immunological response in the proximal small intestinal mucosa.
Shortening and eventual loss of villi leads to reduced surface area of mucosa and malabsorption.
How does coeliac disease present?
CLASSICAL:
8-24 months after introduction of gluten-containing foods to the diet.
Profound malabsorption with FTT, abdo distension, buttock wasting, abnormal stools, irritability.
NOW:
More likely to present in later childhood with mild GI symptoms, anaemia due to iron/folate deficiency, and growth failure.
Or identified on screening.
Who is screened for coeliac disease?
Increased risk: T1DM, autoimmune thyroid disease, Down syndrome, first degree relatives of individuals with known coeliac disease.
How is coeliac disease diagnosed?
IgA tissue transglutaminase (tTG) abs
Endomysial abs
Must be confirmed by endoscopy –> biopsy shows increased epithelial lymphocytes, villous atrophy and crypt hypertrophy.
Gluten withdrawal should resolve sx, if not then reconsider dx.
When is a gluten challenge indicated?
If initial biopsy or response to gluten withdrawal is doubtful, or when disease presents before age 2, do gluten challenge in later childhood to demonstrate susceptibility of SI mucosa to damage by gluten.
Positive result = serology becomes positive again.
What is the treatment for coeliac disease?
Lifelong gluten free diet.
What is toddler’s diarrhoea?
Chronic non-specific diarrhoea. Commonest cause of persistent loose stools in preschool children.
Due to maturational delay in intestinal motility leading to intestinal hurry.
How does chronic non-specific diarrhoea present?
Diarrhoea with some well formed stools, some explosive and loose, undigested vegetables, children are well, no FTT or dietary factors causing the diarrhea.
How is chronic non specific diarrhoea treated?
Ensure child’s diet contains enough fat, as this slows gut transit, and fibre.
Dont drink excessive fresh fruit juice as this can exacerbate symptoms.
Usually grow out of it by 5 yrs of age but fecal continence may be delayed.
What needs to be considered in chronic diarrhoea following bowel resection, cholestatic liver disease or exocrine pancreatic dysfunction?
Malabsorption.
Which is the more common type of IBD?
Crohn’s disease 2:1 UC
Give 3 differences between Crohn’s and UC.
UC: Colon only, continuous lesions, mucosal inflammation.
Crohn’s: Any part of GI tract, usually terminal ileum and proximal colon. Skip lesions, RIF mass, cobblestone appearance, granulomas, transmural inflammation.
[Oxford]
How does Crohn disease present?
Mainly Lethargy, unwell
Classical GI sx of abdo pain, diarrhoea + wt loss but in reality these are often absent.
May be mistaken for psych problem or anorexia nervosa.
Growth failure, delayed puberty, oral lesions, uveitis, arthralgia, erythema nodosum
What investigations would you do for ?Crohns and what would they show?
Inflamm markers - raised platelets, ESR and CRP
Iron deficiency anaemia
Low serum albumin
Upper GI endoscopy, ileocolonoscopy and small bowel imaging are required.
Histology: Non-caseating epithelioid cell granuloma
Small bowel imaging: Narrowing, fissuring, mucosal irregularities, bowel wall thickening.
How is Crohn’s managed?
- Nutritional therapy - whole protein modular feeds (polymeric diet) 6-8 weeks.
- Systemic steroids
- Immunosuppressants: azathioprine, mercaptopurine, methotrexate
- Anti-TNF agents: infliximab, adalimumab.
Surgery for complications
Long term supplemental enteral nutrition to correct growth failure
[Lissauer]
How does UC present?
Rectal bleeding Diarrhoea Colicky pain \+/- wt loss and growth failure Erythema nodosum Arthritis
What investigations would you do for ?UC and what would they show?
Endoscopy - continues lesion from rectum proximally. 90% of children have pancolitis, whereas in adults colitis is usually confined to distal colon.
Exclude infective colitis
Histology - mucosal inflammation, crypt damage, ulceration
Small bowel imaging to exclude Crohns
