Paediatrics 1 Flashcards
Shunt direction for Ventricular Septal Defect
Left to Right
Effects of a moderate VSD
Enlarged atria and ventricles leads to pulmonary HTN and Congestive heart failure
Effects of Severe VSD
Severe pulmonary HTN and early onset heart failure
RF for VSD
Prematurity
Genetic Conditions - Downs, Edwards, Patau
Family Hx
Upper vs Lower RTI’s
Upper - rhintis, otitis media, pharyngitis, tonsilitis, Laryngitis
Lower - Bronchitis, croup, epiglottitis, Tracheitis, bronchiolitis, pneumonia
Clinical presentation of VSD
Often asymptomatic
Pansystolic murmur at lower left sternal border
Poor feeding
Tachypnoea
Dyspnoea
Failure to thrive
Diagnosing VSD
ECHO, ECG, XR
XR finding for VSD
Cardiomegaly
Tx for VSD
Diuretics for pulmonary congestion
ACE-i
Surgical repair
Complications of VSD
Eisenmengers, Endocarditis, Heart Failure
Shunt in ASD
Left to right
RF for ASD
Maternal smoking, FHx of CHD, Maternal diabetes, Maternal Rubella
Clinical Presentation of ASD
Tachypnoea, Poor weight gain, Recurrent Chest infections, Soft systolic ejection murmur in 2nd intercostal space, Wide flexed split S2 sound
Ix for ASD
ECG and Echo
Tx for ASD
Small - conservative, wait and weight it may close
Surgical closure if larger than 1cm
Complications of ASD
Stroke from DVT, AF, Pul Htn, Eisenmengers
Croup summarise
Viral - para flu
Spring/autumn
Self limiting
Worse at night
Barking sound
Stridor
Recession
Steroids help
Most common cyanotic congenital heart disease
Overriding aorta
Large VSD
Pulmonary stenosis
RVH
Epidemiology of ToF
More common in males
Rubella
Increased maternal age
Pathophysiology of ToF
Decreased right ventricular outflow. Dilated and displaced aorta.
Mild - asymptomatic but as heart grows they become cyanotic at 1-3 years
Moderate - Cyanosis and resp distress in first few months
Extreme - seen on antenatal scan, cyanosis quickly
Clinical Presentation of ToF
Irritability, Cyanosis, Clubbing, Poor feeding, Poor weight gain, Ejection systolic murmur in pul region and tet spells
What is a tet spell
Baby suddenly turns bluish and faints
Ix for ToF
CXR - boot shaped heart
MRI,Cardiac catheter
Echo
Tx for ToF
Prostaglandin infusion PGE1 to maintain ductus arteriosus
Beta blockers
Morphine to reduce resp drive
Surgical repair under bypass 3mo-4y but needs ICU
Why do you maintain the ductus arteriosus in ToF
To provide additional pulmonary blood flow to improve oxygen sats
Complications of ToF
Pulmonary regurg, Lifelong follow up
RF for ToF
Downs, Digeorge
What is Transposition of the Great Arteries (TGA)
Aorta rises from the right ventricle and pulmonary artery from the left ventricle
Epidemiology of TGA
More common in males
Maternal age over 40
Rubella
Maternal Diabetes
Alcohol consumption
Pathophysiology of TGA
Deox blood is delivered systemically
Mixing needs to be present to sustain life so a patent foramen ovale, VSD or patent ductus arteriosus must be present
Clinical presentation of TGA
Cyanosis in first 24 hours, Right ventricular heave, Loud S2 heart sound, Systolic murmur if VSD present,
Dx of TGA
Low SATS, ECHO, CXR shows egg on a string due to narrowed mediastinum and cardiomegaly, metabolic acidosis
Tx for TGA
PGE1 to ensure PDA
Surgical repair
What is a PDA
Persistent connection between the aorta and pulmonary artery
Shunt in PDA
Left to right
RF for PDA
Female and premature
Clinical presentation of PDA
Resp distress, Apnoea, Tachypnoea, Tachycardia, Continuous machinery murmur at left sternal edge
Dx of PDA
Echo, ECG/CXR
Tx for PDA
Cardiac catheterisation to close around 1 years old or sooner in more severe cases
Premature - Indomethacin or ibuprofen will inhibit prostaglandin and stimulate closure
What is croup
Acute laryngotracheobronchitis
Epidemiology of croup
Children ages 6mo-3yo
Common in autumn and spring
More common in boys
Pathophysiology of croup
Mucosal inflammation anywhere between nose and trachea
Caused by parainfluenza mainly but also Adenovirus, Rhinovirus, Enterovirus
Clinical features of corup
Mild: Barking cough with no stridor, no recession, child happy to eat or drink
Moderate: Frequent barking cough with audible stridor at rest, suprasternal recession, no agitated
Severe: Frequent barking cough, Prominent stridor, Sternal recession, agitated and distressed potentially tachy
Exam and Hx for Croup
1-4 days of non specific rhinorrhea, fever and barking cough
Worse at night
Stridor
Decreased bilateral air entry
Tachypnoea
Costal recession
Resp failure red flags
Drowsiness, Lethargy, Cyanosis, Tachycardia, Laboured breathing
Dx of Croup
FBC, CRP, U+E, CXR to exclude a foreign body
Tx of Croup
Symptoms from 48 hours-week
Paracetamol or Ibuprofen for sore throat
Admission if moderate/severe and if dehydrated
Single dose of dexamethasone 0.15mg/kg or prednisolone
Nebulised adrenaline for relief of severe symptoms
Oxygen if needed
Monitor for needed ENT intervention if suspected airway blockage
Complications of Croup
Otitis Media, Dehydration due to reduced fluids, Superinfection such as pneumonia
Define Bronchiolitis
Viral infection of the bronchioles
Epidemiology of Bronchiolitis
under 2’s
Common
Winter and spring peak
Caused by RSV
RF for Bronchiolitis
Breastfeeding for less than 2 mo
Smoke
Older siblings who could spread
Chronic lung disease of prematurity
Clinical features of Bronchiolitis
Symptoms onset 2-5 days
Low grade fever
Rhinorrhoea
Cough
Reduced feeding
Signs of resp distress: nasal flaring, tracheal tug, head bobbing, grunting, recession
Inspiratory crackles
Ix for Bronchiolitis
Nasopharyngeal aspirate for RSV culture
FBC, Urine, Blood gas
Home Tx for Bronchiolitis and prevention
Supportive at home
Palvizumab vaccine considered if child under 9 mo with chronic lung or child under 2 with severe immunodeficiency require long term vent
When to admit urgently in bronchiolitis
Apnoea
- Resp Rate > 70
- Central cyanosis
- SpO2 < 92%
When to do a non urgent admission in bronchiolitis
- Resp Rate > 60
- Clinical dehydration
Inpatient management for bronchiolitis
O2
Fluids
CPAP if in resp failure
Suctioning of secretions
Ribavirin for severe
What treatment has no evidence for use in bronchiolitis
Bronchodilation, Abx and steroids
What is Pneumonia
Infection of the LRT and lung parenchyma leading to consolidation
Epidemiology of Pneumonia
Highest incidence in infants
Viral cause more common in younger infants
Bacterial more common in older infants
Viral disease more common in winter
Aetiology of Pneumonia
Neonates: Group B strep, E.coli, Klebsiella, S.Aureus
Infants: Strep pnuem, Chlamydia
School age: Strep pneum, S.Aureus, Group a strep, Mycoplasma pneum
Clinical presentation of Pneumonia
Usually precede a URTI, Fever, SOB, Lethargy, Signs of resp distress, wheeze and hyperinflation when viral
Auscultation: dullness to percuss, crackles, Decreased breathing sounds, Bronchial breathing
Ix for Pneumonia
Clinical mainly
CXR - fluids in lungs
Tx for Pneumonia
Manage at home with analgesia
Admitted: O2 and Iv fluids
Abx:
Neonates: Broad spectrum
Infants: amox/co-amox
Over 5: Amox/Erythromycin
Complications of Pneumonia
Risk of parapneumonic collapse and empyema
Is whooping cough a URTI or LRTI
URTI
Whooping cough causative organsism
Bordatella Pertussis - Gram -ve bacillus
Epidemiology of Whooping Cough
Less common now due to vax
Impacts infants worse
Clinical presentation of Whooping cough
Catarrhal phase: 1-2w with coryzal Sx
Paroxysmal phase: week 3-6, inspiratory whoop
Cough worse at night
Spasmodic coughing episodes
Low grade fever
Sore throat
Convalescent phase - downgrade to cough and can last 3 mo
Dx for Whooping cough
Naso-pharyngeal swab with pertussis
FBC
Antibody test
Tx for Whooping cough
Macrolide
Prophylactic Abx for contacts
Isolate for 21 days after symptom onset or for 5 days after Abx
Complications of Whooping cough
Seizures, Pneumonia, Bronchiectasis, Encephalopathy, Otitis Media
What is Asthma
Reversible paroxysmal contriction of the airways with inflammatory exudate and followed by airway remodelling
Most common chronic condition in children
Aetiology of Asthma
Genetic, Premature, Low birth weight, Smoking, Early viral bronciolitis, Cold air, allergen exposure
Clinical presentation of asthma
Episodic wheeze, dry cough, SOB, Reduced peak flow
Ix for Asthma
- FEV1 significantly reduced
- FVC normal
- FEV1:FVC may be <70% if poorly controlled
- Reversible spirometry is highly suggestive of asthma
- ENO levels of nitric oxide correlate to inflammation
- Baseline chest x ray
Management of Asthma
Step 1: SABA PRN - Salbutamol
Step 2: ICS Preventer therapy - Beclomethasone
Step 3: LTRA Montelukast
Step 4: Strop LTRA if hasn’t helped and add LABA - Salmeterol
Step 5: Switch ICS/LABA for ICS MART: Formoterol and ICS
Step 6: Add a separate LABA
Step 7: High dose ICS (>400mcg), referral
Management of Asthma in under 5
Step 1: SABA PRN - Salbutamol
Step 2: SABA + 8 week trial of ICS if symptoms reoccur within 4 weeks, restart ICS
Step 3: Refer to specialist
What is viral indued wheeze
Episodic wheeze - symptom of viral URTI and symptom free between events
Multiple trigger wheeze - URTI and other factors trigger wheeze
Management of Viral induced wheeze
Symptomatic - Saba with spacer max of 4 hourly up to ten puffs
LTRA and ICS via spacer
Multiple trigger: trial ICS or LTRA for 4-8 weeks
What is Respiratory Distress syndrome
Affects premature neonates, before the lungs start producing adequate surfactant, common in below 32 w babies
Pathophysiology of Resp distress syndrome
Inadequate surfactant leads to high surface tension in alveoli leading to atelectasis as it more difficult for the alveoli to expand so inadequate gas exchange and hypoxia, hypercapnia and resp distress
Management of Resp distress syndrome
Dexamethasone to mothers with suspected preterm labour to increase surfactant production, Intubation and ventilation may be needed to fully assist breathing if distress is severe, Endotracheal surfactant via tube, CPAP, O2
Complications of resp distress syndrome
Short term - Pneumothorax, Infection, Apnoea, Intraventricular haemorrhage, Pul Haemorrhage, Necrotising Enterocolitis
Long term complications of resp distress syndrome
Chronic lung disease of prematurity, Retinopathy of prematurity, Nuerological, hearing, visual impairment
What is Bronchopulmonary dysplasia
Infants who still require oxygen at postnatal age of 36 weeks are described as having BPD
Bronchopulmonary Dysplasia aetiology
lung samage from pressure and vilume trauma of artificial ventilation, oxygen toxicity and infection
CXR - widespread opacification and sometimes cystic changes, fibrosis and even lung collpase
Tx for Bronchopulmonary dysplasia
Weaned onto CPAP followed by additional oxygen where needed and sometimes corticosteroids to facilitate weaning however there is a risk of neurodevelopmental issues with these
What is epiglottitis
inflammation and swelling of the epiglottis caused by infection. Life threatening emergency
Epiglottitis causative organism
Haemophilus influenzae B
Typical presentation of epiglottitis
Drooling, Tripod position, unvaxed, fever, sore throat, difficulty swallowing, muffled voice, scared and quiet child, septic and unwell
Ix for epiglottitis
If acutely unwell and obvious then dont investigate just treat them. Dont scare the child.
Can do a Xr of neck - thumb sign due to swollen epiglottis, can exclude a foreign body
Management of epiglottitis
Alert the most senior paediatrician available and anaesthetics.
Ensure airway is secure, prep for intubation and prep ICU
Tx once airway is secure: IV AbX (Ceftriaxone) and steroids (dexamethasone)
Prognosis for Epiglottitis
Most recover without intubation
Epiglottic abscess - pus around the epiglottis - similar tx to epiglottitis
Briefly summarise Laryngomalacia
Where part of the larynx above the vocals cords is structured in a way that partially blocks the airway
Chronic stridor
Omega shaped epiglottis
In infants peaking at 6 mo. intermittent stridor that is worse during feeding, upset, lying on back and during infection
Usually self resolving. Rarely surgery or a tracheostomy is needed
Define Cystic Fibrosis
Autosomal reccessive condition affecting mucus glands
Genetic mutation in CF
Mutation of the cystic fibrosis transmembrane conductance regulatory gene on chromosome 7 - lots of variants with the most common being delta-F508 which codes for a chloride channel
CF incidence
1/2500
Key consequences on physiology of CF
Thick pancreatic and biliary secretions that blocks ducts resulting in lack of digestive enzymes
Low volume thick airway secretions - reduce airway clearance resulting in bacterial colonisation and increased infection
Congenital bilateral absence of vas deferens - male infertility
Presentation of CF
Screened for in bloodspot test
Meconium ileus is the first sign - not passing meconium in 24 hours with abdo distension and vomiting
Recurrent LRTI, Failure to thrive and pancreatitis
Symptoms of CF
Chronic cough, Thick sputum production, Recurrent RTI, Loose greasy stools, Salty child, Poor weight and height gain
Signs of CF
Low weight or height on chart, Nasal polyps, Clubbing, Crackles and wheeze, Abdo distension
Causes of clubbing in children
Hereditary clubbing, Cyanotic heart disease, IE, CF, Tuberculosis, IBD, Liver Cirrhosis
Dx of CF
Newborn blood spot, sweat test, Genetic testing
What is the sweat test
Gold standard for CF
Patch of skin chosen, Pilocarpine is applied, Electrodes next to patch, current causes sweating, lab is sent sweat to look for a chloride concentration of over 60 mmol/L
Microbial colonisers in CF
S. Aurues, Hib, Klebsiella, E.coli, Burkhoderia cepacia, Psedomonas aeruginosa
Pseudomonas Aeruginosa in CF
Hard to clear, resistant, reccomend isolating those infected. Keep away from other children with CF.
Tx - Tobramycin or ciprofloxacin
Management of CF
Chest Physio - several times a day to clear mucus and reduce infection and colonisation risk
Exercise - clears mucus and improves resp reserve
High calorie diet - due to malabsorption, increased resp effort and coughing
CREON - digests fats
Prophylactic fluloxicillin
Treat infections
Bronchodilators like salbutamol
Nebulised DNase
Nebulised hypertonic saline
Vax - pneumococcal, influenza and varicella
Monitoring for CF
Specialist clinics typically every six months, regular sputum monitoring, screening for diabetes, osteoporosis, vit d def, liver failure
Prognosis for CF
Median life expectancy of 47
- 90% develop pancreatic insufficiency
- 50% develop diabetes
- 30% develop liver disease
- Most males are infertile
What is Primary Ciliary Dyskinesia
Aka Kartagners syndrome. Autosomal recessive affecting the cilia. More common when there is consanguinity
PCD pathophysiology
dysfunction of motile cilia most notably in resp tract. build up of mucus in lungs which increases risk of infection
Sx for PCD
Similar to CF, lots of infection, poor growth and bronchiectasis, also affects fallopian tubes and flagella of sperm so reduced or absent fertility
What is PCD strongly linked with
Situs Inversus
What is Kartagners Triad
Three features of PCD:
- Paranasal Sinusitis
- Bronchiectasis
- Situs Inversus
What is Situs Inversus
All internal organs are mirrored in the body
25% of those with situs inversus have PCD
Dx of PCD
Recurrent infection. Family history looking for consanguinity. Imaging to look for situs inversus. Semen analysis, key test is to take a sample of ciliated epithelium
Management of PCD
Similar to CF and bronchiectasis
What is Pyloric Stenosis
Progressive hypertrophy of the pyloric sphincter causing gastric outlet obstruction
Epidemiology of Pyloric Stenosis
More common in boys, 1/500, Family Hx, First borns
Clinical presentation of Pyloric Stenosis
Presents at 4-6 weeks
Non bilious, forceful, projectile vomiting after feed
Will continue to feed despite vomiting
Weight loss
Dehydration
Constipation
Visible Peristalsis
Palpable olive sized pyloric mass felt
Ix for Pyloric Stenosis
Test feed with NG tube and empty stomach and feel for visible peristalsis and mass
USS - Hypertrophy of the muscle
Blood gas - Hypochloremic, Hypokalaemic Metabolic Alkalosis
Management of Pyloric Stenosis
Correct mentabolic imbalances - NaCl
Fluid bolus for hypovolemia
NG tube feed
Ramsteds Pyloromyotomy - feeding can commence after 6 hours
What is Hirschsprungs disease
Nerve cells of the myenteric plexus are absent in the distal bowel and rectum, specifically the parasympathetic ganglionic cells resulting in a lack of peristalsis
Epidemiology of Hirschprungs
90% present in neonates. Average age of presentation is 2 days. Males. Downs