Paeds Flashcards
Which 3 shunts exist in fetal circulation
- Ductus venous (umbilical vein - IVC, allows liver bypass)
- Foramen vale (R - L atrium, allows bypass of R ventricle + pulmonary circulation)
- Ductus arteriosus (Pulmonary artery - aorta, allows bypass of pulmonary circulation)
What keeps the ductus arteriosus open?
Prostaglandins
When is surfactant produced?
24-28 weeks. Levels are not sufficient until 34weeks gestation.
Neonatal mortality rate
= Deaths which occur within first 28 days of life following live birth
England + Wales = 2.8 per 1000
Globally = 17 per 1000
Japan + Iceland have lowest rates, Afghanistan, Somalia + Africa have highest rates.
Still birth rate
= When a foetus is delivered after 24 weeks gestation without any signs of life
In England + Wales = 4.1 per 1000
Globally = 18
Iceland, Andorra + Denmark have lowest
Pakistan, nigeria + chad have highest
Perinatal mortality rate definition
Deaths occurring within first 6 days of life (including still births)
What gestation is classified as pre-term birth + WHO classification
< 37 weeks gestation
<28 weeks = extreme preterm
28-32 weeks = very preterm
32 - 37 = Moderate preterm
Methods of predicting preterm labour
Transvaginal USS of cervic (if >15mm then preterm labour unlikely)
Fetal fibronectin measurement ( <50 means labour is unlikely)
Neonatal Resuscitation
- Dry + Warm the baby
- Calculate APGAR score - at 1, 5 and 10mins
- Stimulate breathing (Dry baby)
- 2 x cycles of 5 inflation breaths (3s)
- 30s of ventilation breaths
- If no response - Chest compressions if HR <60
- perform these at 3:1 ratio with breaths - IV drugs + Intubation
Causes of neonatal respiratory distress syndrome
- Prematurity (due to lack of surfactant)
- Meconium aspiration
- Congenital cystic lung disease
- Congenital pneumonia
- Pneumothorax
- Milk aspiration
Premature Apnoea
= periods where breathing stops spontaneously for >20s or is associated with desaturation/bradycardia
Caused by immaturity of the autonomic nervous system.
Management of premature apnoea
Monitoring
Tactile stimulation
IV Caffeine
Meconium aspiration risk factors
Post term babies (>42 weeks)
Pre-eclampsia
Chorioamnionitis
Smoking/substance misuse
CXR findings in Meconium aspiration / Respiratory distress syndrome
Ground-glass appearance + hyperinflation
Meconium stained amniotic fluid indicates what
Meconium aspiration
Management of meconium aspiration
Intubate + Ventilate
If Hypertensive give NO
IV Ampicillin + Gentamicin
Management of persistent pulmonary HTN of the newborn
Most will need intubation + ventilation + circulatory support
NOS + Sildenafil
Signs & Symptoms of neonatal abstinence syndrome
CNS: Irritablity + Hypertonia + High pitched cry + seizures
Vasomotor: Yawning + sweating + pyrexia + tachypnoea
GI: Poor feeding + vomitting+ Hypoglycemia
Treatment of opiate induced neonatal abstinence syndrome
Morphine sulfate
Treatment of non-opiate induced neonatal abstinence syndrome
Phenobarbitone
Neonatal hypoglycaemia in first hour of life
Transient + normal
What glucose level is considered neonatal hypoglycemia
< 2.6mmol/L
Causes of persistent neonatal hypoglycaemia
- Sepsis
- Hypoxic-ischemic encephalopathy
- Hypothermia
- IUGR / Preterm birth
- Beckwith Wiedemann syndrome (overgrowth disorder)
- GH deficiency
- Congenital adrenal hyperplasia (remember similar to Addison’s syndrome)
Symptoms of Neonatal hypoglycaemia
Irritability
Tachypnoea
Pallor
Weak cry
Drowsiness / Seizures
Hypothermia
Management of neonatal hypoglycaemia
If asymptomatic - encourage regular feeds
If symptomatic or BM v low = IV 10% dextrose
Necrotising enterocolitis
Bowel necrosis in premature neonates. A life threatening emergency - leading cause of death in preterm infants
Classic Triad in necrotising enterocolitis
Bile stained aspirate
Abdominal distension +/- discolouration
Bloody stools / Bilious vomitting
In what timescale does necrotising enterocolitis typically occur
First 10 days of life
What ABG finding would occur in necrotising enterocolitis
Metabolic acidosis
Gold standard investigation & findings in necrotising enterocolitis
Abdo X ray;
This shows Dilated loops of bowel, bowel wall oedema, gas in the bowel / free gas in peritoneum if perforated.
Football sign - air outlining the falciform ligament
Rigler sign - air inside + outside the bowel
Management of necrotising enterocolitis
Similar to bowel obstruction - NBM + IV fluids + NG drainage + immediate referral to surgeons for removal + stoma
Which organism most commonly causes neonatal sepsis
Group B streptococcus
What are the red flags / Clinical indicators of neonatal sepsis
Maternal sepsis
Seizures
Jaundice
A term baby requiring ventilation after birth
Respiratory distress starting < 4 hours after birth
Which antibiotics should be used for GBS neonatal sepsis
Benzypenecillin
Which antibiotics should be used for neonatal sepsis caused by E.coli
Gentamicin
What screening is there for retinopathy of prematurity
Babies born < 32 weeks or Birth weight < 1.5kg should be screened every 2 weeks
Management for Retinopathy of prematurity
Transpupillary laser photocoagulation
Neonatal jaundice rules of thumb
< 24hrs of life = Pathological, needs urgent invx
First 10 days of life = normal (due to HbF breakdown) - will be unconjugated
After 2 weeks = pathological
Causes of neonatal jaundice
Haemolytic disease of the newborn
Breastmilk jaundice
ABO incompatability
Congenital disease: G6PD deficiency, A1AT def, CF, Gilberts syndrome etc
Sepsis
Neonatal cholestasis
Biliary atresia
Thyroid problems
Investigations for Neonatal jaundice
FBC + Blood film
Conjugated / Unconjugated bilirubin levels
Blood type testing of mother + baby
Direct Coombs test - for haemolysis
TFTs
Blood/urine cultures if sepsis is suspected
Management of neonatal jaundice
Jaundice should be closely monitored + plotted on the chart.
Photobox therapy (UV therapy) commonly used in unconjugated cause.
Kernicterus
Brain damage due to high bilirubin levels.
What are the 3 neonatal screening checks
NIPE Newborn exam - in first 72hrs
Heel prick blood spot - day 5-9
Otoacoustic emission hearing test
Which 9 conditions are screened for in the heel prick blood spot test
- Hypothyroidism
- Haemoglobinopathies
- Cystic Fibrosis
- Phenylketonuria
- Medium chain Acetyl coach dehydrogenase deficiency
- Maple syrup urine disease
- Isoclad acidemia
- GA1
- Homocystinuria
Sweaty foot odour + Seizures in first few days of life
Isovaleric acidemia
A health baby which quickly becomes ill with cerebral oedema and Sweet swelling urine + ear wax
Maple syrup urine disease
Blonde hair, Blue eyed baby with musty smell, LDs and seizures within 6 weeks of life
Phenylketonuria
Acyanotic congenital heart diseases
VSD
ASD
PDA
Pulmonary stenosis
Coarctation of the aorta
Aortic stenosis
Cyanotic congenital heart diseases
The T’s
Transposition of the great arteries
Tetralogy of fallout
Truncus arteriosus
Tricuspid valve anamolies
Tons of others; Ebstein anomoly
ASD murmur
Mid-systolic cresendo-decresendo murmur loudest at the upper left sternal border with fixed split S2
Complications of ASD
AF + Atrial flutter
Stroke following DVT
Pulmonary HTN
Eisenmenger syndrome
VSD murmur
Pan-systolic murmur heard best at left lower sternal border. May feel systolic thrill.
(MR + TR also cause pan-systolic murmur)
PDA murmur
Continuous cresendo-decresendo “machinery” murmur (may not hear S2)
Coarctation of the aorta murmur + Clinical signs
Weak femoral pulse +/- Collapsing pulse!!!
Murmur = systolic murmur heard below left clavicle + left scapula
May have underdeveloped L arm and legs.
Which syndrome is associated with coarctation of the aorta
Turner’s syndrome
Management of coarctation of the aorta
Severe cases require surgery - Give prostaglandin E while waiting for surgery to keep the ductus arteriosus open
Transposition of the great arteries
Aorta + pulmonary trunk are transposed (R ventricle pumps blood into Aorta + L ventricle into pulmonary vessels).
Only survivable if there is a L –> R shunt (e.g VSD)
CXR shows oval/egg shaped heart + increased pulmonary vasculature.
Management = prostaglandins + surgery
Tetralogy of Fallot
Associations = Digeorge syndrome + Chromosome 22 deletions
Causes HF by age 1
Features;
1. VSD
2. Over-riding aorta
3. Pulmonary valve stenosis
4. RVH
Murmur = ejection systolic murmur at pulmonary area
CXR = boot shaped heart
Tet spells
Acute transient worsening of R –> L shunt causing a cyanotic episode. Often happens when the child is exerting themselves (e.g crying or walking).
Treatment;
- some pts may squat or bring knees to chest
- Beta blockers
- IV fluids (to inc pre-load)
- Morphine (decrease respiratory drive)
- Phenylephrine infusion
Ebstein anomaly
Congenital heart condition where tricuspid valve is lower meaning bigger R atrium + smaller R ventricle.
Associated with wolf Parkinson white syndrome
Features = Gallop rhythm + S3 + S4
CXR = box shaped heart
Which drug can cause Ebstein anomaly
Lithium
Common in bipolar mothers
Failure or delay (> 24hrs) to pass meconium + Abdo pain + distension
Hirschprung’s disease
Gold standard investigation for hirschprung’s disease
Rectal biopsy - shows absence of ganglions
Hirschprung’s disease
= colonic aganglionosis
congenital condition where the parasympathetic ganglions of the myenteric/auerbach’s plexus are absent in the terminal bowel/rectum resulting in constricted bowel with distension proximally to this.
Hirschprung’s disease associated conditions
Down’s syndrome
Neurofibromatosis
MEN II
Waardenburg (blue eyes, white spots in hair + hearing loss)
Eisenmenger syndrome
This is when an ASD/VSD/PDA (L –> R shunt) leads to pulmonary HTN and eventually a R –> L shunt causing cyanosis.
Examination findings;
- Right ventricular heave
- Loud S2
- Raised JVP
- Peripheral oedema
- Cyanosis / Clubbing / SOB / plethoric complexion
+ Signs of underlying defect (ASD/VSD/PDA)
Management = Definitive treatment is heart-lung transplant. Medical management involves Sildenafil for pulmonary HTN or venesection for polycythemia + Prophylaxis of thrombosis / IE
Bronchiolitis
–> Inflammation of the bronchioles caused by RSV
Most common in children <1yrs (can’t be diagnosed after 2 yrs) & Premature babies are more at risk.
Symptoms;
- Coryzal symptoms
- Signs of respiratory distress
- Dyspnoea / Tachypnoea
- Mild fever
- Wheeze + Crackles on auscultation
Management = Supportive, ensure adequate intake (may need IV fluids or NG tube) + Nasal drops/suctioning + supplementary oxygen.
Red flags / When to admit a baby with bronchiolitis
Age < 3 months
Pre-existing condition e.g prematurity, CF, downs syndrome
50-75% less milk intake
Dehydration
RR > 70
O2 sats < 92%
Signs of respiratory distress
Apnoeas
What is given as prophylaxis of bronchiolitis in high risk babies?
Palivizumab - monoclonal antibody.
Given to premature babies, those with CHD or existing conditions etc.
Monthly injection
Monitoring of children on ventilatory support
Capillary blood gases.
Signs of poor ventilation;
- Rising pC02
- Falling pH (acidosis) / T2RF
Signs of respiratory distress in infants
Raised RR
Use of accessory muscles
Intercostal recessions
Subcostal recessions
Nasal flaring
Head bobbing
Tracheal tugging
Cyanosis
Abnormal airway noises
What is a focal wheeze indicative of
Foreign body / airways obstruction
(asthma, bronchiolitis and viral wheeze cause a diffuse expiratory wheeze)
Viral induced wheeze
Essentially an acute wheezy illness in a child aged 2 - 5 (not bronchiolitis and not asthma).
Pathophys = inflammation/oedema of the small airways causing smooth muscle constriction.
Typically caused by virus (e.g RSV or rhinovirus)
Symptoms = evidence of viral illness, SOB, Respiratory distress + expiratory wheeze heard throughout the chest.
Management = managed the same as acute asthma: Supplementary oxygen + Nebulised salbutamol / ipratropium + Steroids (oral pred/IV hydrocortisone to reduce inflammation)
If severe/no response can try IV magnesium sulphate or IV aminophylline.
At what age can asthma be diagnosed in a child
> 5yrs
Main differences between viral-induced wheeze + asthma
VIW = Age <5yrs + no history of atopy + occurs only in illness (i.e not triggered by cold weather/exercise etc like asthma)
Croup
Acute infective respiratory disease caused by Parainfluenza virus - typically affecting children 6months - 2 years.
Presentation;
- Respiratory distress & Fever
- seal-like ‘Barking cough’
- Stridor
- Hoarse voice
Management;
- Supportive treatment
- Oral dexamethasone (v effective) - symptoms should resolve in 48hrs
Xray findings in croup
Steeple sign - narrowing below the epiglottis
What scoring system can be used to determine the severity of croup?
Westly scale
3-7 = moderate
8-11 = severe
12+ = respiratory distress
Epiglottitis
Inflammation of the epiglottis typically caused by Haemophilus influenza type B.
(now less common due to vaccinations)
= can be life threatening as may cause total airway obstruction.
Symptoms;
- Stridor
- Drooling
- Tripod position (kids often sat forward with hands on knees)
- Difficulty / pain when swallowing
- Muffled voice
Management = Keep the child calm. Secure airway (consider intubation if obstruction). Give IV Ceftriaxone + Dexamethasone
When is the haemophilia influenza type B vaccination given?
Part of the 6-1 vaccine at 2months, 3 months and 4 months and given as a single vaccine at 12-13months
6 in 1 Vaccine
Given at age 2 months, 3 months + 4 months
1. Diptheria
2. Tetanus
3. Whooping cough (pertussis)
4. Polio
5. Hib
6. Hepatitis B
Investigation + Findings in epiglottitis
Lateral Xray neck
Characteristic thumbprint sign
Complications of epiglottitis
Airways obstruction
Epiglottic abscess
Laryngomalacia
Congenital malformation of the larynx causing a partial obstruction on inspiration.
Typically occurs in infants age 6months.
Signs;
- Chronic Inspiratory stridor (may be more prominent when feeding, crying or lying down)
- Swallowing difficulty + choking
*They rarely have respiratory distress as its more chronic so they adjust to the hypoventilation.
Investigation of choice = laryngoscopy
- this shows omega shape (shortened epiglottic folds)
Management = usually resolves with growth. May need tracheostomy or surgery if severe.
Whooping cough
= URTI caused by Bordetella pertusisss (gram -ve)
Symptoms;
- starts with Coryzal symptoms and then severe coughing fits start after 7 days.
- cough is worse at night + after feeding
- Vomitting
- Cyanosis
- Large inspiratory ‘whoop’ at the end of coughing
Diagnosis = Nasopharyngeal swab (if 2-3 weeks after onset) or Anti-pertussis toxin IgG if < 2 weeks
Management = notify PHE.
Supportive care
Macrolide antibiotics (e.g azithromycin) or Co-trimoxazole if in first 21 days or vulnerable patient.
+ prophylactic Abx for close contacts.
What is the main complication of whooping cough
Bronchiectasis
Pertussis vaccination schedule
Part of the 6 in 1 vaccine given at 8, 12 and 16 weeks.
Also given at age 3-4yrs as the 4in1 booster.
Pregnant women = 16-32 weeks
Cystic Fibrosis
Autosomal recessive condition due to delta-F508 mutation on chromosome 7.
Key consequences;
- Thick pancreatic / biliary secreations (blocks ducts + decreases digestive enzymes)
- Thick airway recreations = inc infection
- Bilateral absence of vas deferens in males
Clinical Presentation of Cystic fibrosis
- Often presents with meconium ileus = failure to pass meconium in first 24hrs + abdo distension + vomitting.
- Recurrent resp infections + thick sputum
- Failure to thrive
- Pancreatitis / Steatorrhoea
- Abdo pain + bloating
- Child may have a ‘salty taste’ due to concentrated sodium in sweat
Investigations for CF
Bloodspot test (day 5) - often picks it up
Gold standard = sweat test - pilocarpine applied to skin (>60mmol/L chloride is diagnostic)
Genetic testing - GFTR gene
Which microorganisms typically colonise in patients with Cystic Fibrosis
Staph aureus
H influenxa
Klebsiella pneumoniae
pseudomonas auerginosa
Management of Cystic fibrosis
Supportive = Chest physiotherapy, High calorie diet, Regular vaccinations, Monitoring every 6 months + avoid other CF patients due to infection risk
Medical = Creon + Prophylactic Flucoxacillin, Bronchodilators, Nebulised DNase + Hypertonic saline
*Fertility treatment + genetic counselling also
What are the main complications of cystic fibrosis
Infection
Diabetes
Osteoporosis / Vit D deficiency
Liver failure
Pancreatic insufficiency
Kartagner’s syndrome
= Primary ciliary dyskinesia.
= autosomal recessive condition causing dysfunction of cilia (similar presentation to CF due to resp infections)
Triad of Features;
1. Paranasal sinusitis
2. Bronchiectasis
3. Situs inverts (mirrored organs) / Dextrocardia
Invx = CXR + Ciliated epithelium sample + Semen analysis
Signs & Symptoms of asthma
Episodic symptoms + Diurnal variability
Dry cough + wheeze + SOB
History of atopy
Bilateral widespread polyphonic wheeze
Reversibility with bronchodilators
Diagnosis / Invx of asthma in children
1st line = Spirometry + reversibility testing (Child >5yrs)
- BDR should improve FEV1 by >12%
If spirometry is normal or reversibility testing is normal despite an obstructive spirometry - 2nd line investigation is FeNO (>35 is diagnostic)
Management of asthma in children aged 5-12yrs
- SABA
- SABA + Low dose ICS
- SABA + Low dose ICS + LTRA
- SABA + Low dose ICS + LABA
- SABA + MART (ics + laba)
- SABA + Med dose ICS + LABA
- Refer - consider high dose ICS or oral theophylline.