Paeds Peer Teaching 1 Flashcards
A baby has congenital heart disease and is breathless. Which direction is the shunt in? Give 3 examples of conditions which give this picture.
L to R shunt
- VSD
- PDA
- ASD
A baby has congenital heart disease and is cyanotic. Which direction is the shunt in? Give 2 examples of conditions which give this clinical picture.
R to L shunt
- Tetralogy of Fallot
- Transposition of the Great Arteries
A baby has a VSD (Ventricular Septal Defect).
Which direction is the shunt?
List 4 signs / symptoms.
L - R shunt
- Tachycardia
- Tachypnoea
- FTT (Failure to thrive)
- Heart failure
What kind of murmur would you hear if a baby has a VSD? Where would you hear it?
Which direction is the shunt?
- Pansystolic murmur
- L lower sternal edge
- Shunt is L to R
What is the management of a VSD?
Small - will close spontaneously.
Large - surgical closure + diuretics
What signs and symptoms would you see in a child with ASD (Atrial Septal Defect)?
Which direction is the shunt?
- May be asymptomatic
- Tachypnoea
- FTT
- Wheeze
Shunt direction: L to R
What murmur will you hear (and where) if a child has an Atrial Septal Defect?
Which direction is the shunt?
Ejection systolic murmur (L upper sternal edge)
Shunt direction: L to R
What signs and symptoms would you see in a child with a PDA?
Which murmur would you hear and where?
- Tachypnoea
- FTT
- Bounding pulse
Murmur: Continuous machinery murmur (below L clavicle)
What is your management of a patient with ASD (atrial septal defect)?
Small - close spontaneously
Large - Surgical closer
What is your management of a patient with a PDA?
- NSAIDs (Indomethacin)
or - Surgical ligation
What are the 4 components of Tetralogy of Fallot?
- Pulmonary Stenosis
- VSD
- Overriding aorta
- RVH
What signs and symptoms would you see in a child with Tetralogy of Fallot?
- Severe cyanosis
- Hypercyanotic spells on: exercise, crying, defecating
- Ejection systolic murmur
What is your management of a child with Tetralogy of Fallot?
Which direction is the shunt?
Is this cyanotic or acyanotic?
Surgery at 6 months to close VSD, relieve pulmonary out tract obstruction
R to L
Describe the pathophysiology of Transposition of the Great Arteries.
Pulmonary artery and aorta ‘swap’.
RV > Aorta > Body > RA
LV > Pul artery > Lungs > LA
What are the signs and symptoms of Transposition of the Great Arteries?
- Often present on day 2 of life (after Ductus arteriosus closes) with severe life threatening cyanosis.
What is your management of Transposition of the great arteries going to be?
- Maintain PDA (prostaglandin infusion)
- Surgical: atrial sepstostomy and correction
A ‘well’ child has Pulmonary Stenosis.
What is the pathophysiology of this?
What signs and symptoms might you see?
- Pulmonary valve leaflets partially fused together > obstructs RV outflow
- Asymptomatic
- Ejection systolic murmur (L upper sternal edge) and palpable thrill
A ‘well’ child has Aortic stenosis.
What is the pathophysiology of this?
What signs + symptoms might you see?
What murmur will you hear?
- Aortic valve leaflets partially fused together > obstructs LV outflow
- Reduced exercise tolerance, chest pain / syncope on exertion
- Ejection systolic murmur (R. upper sternal edge) AND Carotid thrill
A ‘sick’ child has Coarctation of the Aorta. What is the pathophysiology behind this? Describe the symptoms.
Narrowing of the aorta - commonly at ductus arteriosus.
- Symptoms become more severe with age.
- Asymptomatic, then SOB, arterial hypertension, intermittent claudication.
What murmur will you hear if a patient has coarctation of the aorta?
Describe the patient’s pulse.
- Ejection systolic murmur (L upper Sternal edge).
- Radial:radial / radial:femoral delay.
What is the management of a patient with Coarctation of the Aorta?
- Stent
- Surgical repair
What are the 4 S’s of harmless murmurs?
- Soft
- Systolic
- aSymptomatic
- L Sternal edge
What investigations should you do if you detect a murmur?
- Antenatal ECHO
- Neonatal ECHO, ECG, CXR
What syndrome arises if R to L shunt is not treated?
Eisenmenger’s syndrome.
- Long standing R to L shunt increases pulmonary pressure over time, leading to thickening of the pulmonary arteries. This causes RVH and increases pressure in RV, reversing the shunt to L to R.
What is another name for ‘Croup’?
Laryngotracheobronchitis
What is ‘croup’?
Upper airway obstruction caused by the Parainfluenza virus.
At what age are children most likely to get croup?
-6m - 6years.
Peak incidence = 2 years
What are the symptoms of Croup?
- Seal-like, barking cough
- Hoarseness
- Breathlessness
- Poor feeding
- Preceded by a fever; worse at night.
What is the management of Croup?
- Single dose Oral Dexamethasone 0.15mg/kg or Nebulised Budesonide
- If severe:
> High flow oxygen
> Nebulised adrenaline
Describe Acute Epiglottitis.
LIFE THREATENING MEDICAL EMERGENCY
- upper airway obstruction
- intense swelling of epiglottis and surrounding tissue
What is the causative organism of Acute Epiglottitis?
Haemophilus Influenzae B
Describe the presentation of a child with acute epiglottitis.
- sore throat in a septic-looking child
- Child unable to speak or swallow (drooling)
- Sitting upright, immobile with open mouth to optimise airway
- Soft inspiratory stridor
- Increased respiratory distress.
- Little / no cough
Why has the incidence of acute epiglottis decreased in recent years?
Introduction of Hib vaccine
How should you manage a child with Acute Epiglottitis?
- DO NOT EXAMINE THROAT IF SUSPECTED
- call anaesthetics to intubate
- IV cefuroxime
What is the causative organism of Whooping cough?
Bordatella Pertussis
- highly infectious and contagious
- epidemic every 3 - 4 years
- intubation up to 10 - 14 days
What are the symptoms of Whooping cough?
- Inspiratory whoop (forced inhalation against a closed glottis)
- Spasms of cough -> worse at night, cause vomiting, epistaxis and subconjunctival haemorrhages.
How would you investigate Whooping cough?
Per Nasal Swab culture.
How would you manage a child with Whooping Cough?
- <1 month: Azithromycin (5 days)
- > 1 month: Azithromycin / Erythromycin (7 days)
- School exclusion
What is the typical age range for children with Bronchiolitis?
1 - 9 months
Which pathogen causes Bronchiolitis?
Respiratory Syncytial Virus
also:
- Parainfluenza virus, human metapneumovirus
What are the symptoms of Bronchiolitis?
- Coryzal
- Breathlessness
- Poor feeding
List 6 signs of respiratory distress seen in Bronchiolitis.
- Nasal flaring
- Head bobbing
- Subcostal recessions
- Intercostal recessions
- Tracheal tug
- Grunting
List some ‘other’ signs of Bronchiolitis.
- Fine end inspiratory crackles
- High pitched wheeze
- Cyanosis on feeding
Investigations for Bronchiolitis?
- PCR analysis of nasal secretions
- CXR: hyperinflation
Describe the management of a baby with Bronchiolitis
- Supportive:
> humidified oxygen
> NG feeds
> Fluids
When would Palivizumab be used in Bronchiolitis?
- CF, Immunocompromised, Congenital Heart Disease, Down’s
- > a monoclonal antibody
- > IM once per month through autumn and winter
Describe the pathophysiology of Asthma.
- Chronic inflammatory disorder of lower airways secondary to hypersensitivity
- Reversible airway obstruction
What are the 3 cardinal features of Reversible airway obstruction in Asthma?
- Bronchospasm
- Mucosal swelling and inflammation
- Increased mucous production -> mucous plug
List 7 clinical features of Asthma.
- Intermittent dyspnoea
- Sputum production
- Wheeze
- Cough (nocturnal)
- Diurnal variation
- Exercise tolerance
- Disturbed sleep
How is asthma diagnosed?
- Clinical symptoms
- FEV1:FVC ratio < 70%
- Bronchodilator reversibility: FEV1 improvement by 12% or more
- FeNO >= 35ppb
A child under 5 years is having a ‘moderate’ asthma attack. What signs might you see?
- Sats > 92%
- No clinical features
A child under 5 is having a ‘severe’ asthma attack. What signs might you see?
- Sats <92%
- Unable to talk
- HR > 140
- RR > 40
- Use of accessory neck muscles
A child under 5 is having a ‘life threatening’ asthma attack. What signs might you see?
- Sats <92%
- Silent Chest
- Bradycardia
- Poor resp effort
- Altered consciousness
- Cyanosed
A child over 5 is having a ‘moderate’ asthma attack. What signs might you see?
- Sats > 92%
- PEF > 50% of best predicted
- No clinical features
A child over 5 is having a ‘severe’ asthma attack. What signs might you see?
- Sats < 92%
- PEF <50%
- Unable to complete sentences
- HR > 125
- RR > 30
- Use of accessory neck muscles
A child over 5 is having a ‘life threatening’ asthma attack. What signs might you see?
- Sats < 92%
- PEF < 33%
- Silent chest
- Poor resp effort
- Altered consciousness
- Cyanosed
Describe the Acute management of an Asthma attack.
- A to E assessment
- High flow oxygen
- Salbutamol news
- IV hydrocortisone
- Ipratropium Bromide news
- Magnesium Sulphate IV + call ICU
- Salbutamol IV
What should you look out for if you give Salbutamol IV?
What changes on an ECG would you see with this?
- Hypokalaemia
- ST segment sagging
- T wave depression
- U wave elevation
Describe the pathophysiology of Cystic Fibrosis.
- Autosomal Recessive Defect in CFTR
- codes cAMP regulated Chloride channels in cell membranes (Chromosome 7).
- Commonest Autosomal Recessive disorder in caucasians -> 1 in 25 carriers
- Causes increased viscosity of secretions and blockage of narrow passageways.
List 2 clinical features of Cystic Fibrosis.
- Reduction in air surface liquid layer and impaired ciliary function. Retention of secretions
- Pancreatic ducts blocked by thick secretions -> maldigestion, malabsorption, steatorrhoea
Which 2 pathogens are responsible for ‘Chronic Endobronchial infection’ seen in CF?
- Pseudomonas
- Staph aureus
How do neonates with CF present?
- Meconium ileus
> pancreatic ducts are blocked by thick secretions -> maldigestion, malabsorption, steatorrhoea
How might CF affect kids when they reach puberty?
- Diabetes mellitus
- Delayed puberty
- Male infertility
- Female subfertility
How is CF diagnosed?
- Guthrie heel prick screening test (at 6-9 days of life)
- Sweat test (Chloride ions)
- Faecal elastase (low levels)
- Gene abnormalities in CFTR protein
Describe the management of a patient with CF.
- Aim to prevent progression of lung disease
- Maintain adequate nutrition
- High calorie, high fat diet
- Chest physio and postural draining
- Pancreatic enzyme replacement therapy
- Prophylactic Abx
Describe the presentation of a child with Pyloric Stenosis.
2 - 8 weeks of life
- projectile vomiting (post feeds)
- NOT bile stained (above ampulla of Vater).
What is the pathophysiology behind pyloric stenosis?
Narrowing of the pylorus, impairing gastric emptying
Describe the signs and symptoms seen in a baby with pyloric stenosis.
- Weight loss, FTT, hungry after feeds
- Visible gastric peristalsis
- Palpable abdominal mass on feeding
What investigations would you do if you suspected Pyloric stenosis?
- USS abdo = diagnostic
- Hypokalaemic, hypochloraemic, metabolic alkalosis
How would you manage a baby with pyloric stenosis?
- Rehydration + correct electrolyte imbalance
- Surgical: Ramstedt’s pyloromyotomy
What is ‘intussusception’?
Invagination of proximal bowel into a distal segment.
- commonly: ileum moves into caecum via ileo-caecal valve
At what age does Intussusception tend to present?
3 months - 2 years
Boys > Girls
List 5 clinical features of Intussusception.
- Severe paroxysmal abdominal colic pain
- Child draws knees up to chest, becomes pale, screaming in pain
- Vomiting (may become bilious)
- Blood and mucous in the stool -> REDCURRANT JELLY STOOL
- RLQ abdo mass - sausage shaped
What investigations should you do if you suspect intussusception?
- USS abdo (diagnostic)
> doughnut / target sign - Xray abdo: distended small bowel, absence of gas in the large bowel
What is the management of Intussusception?
Rectal air insufflation
Describe the pathophysiology of Intestinal Malrotation.
- Surgical emergency
- Obstruction of small bowel -> congenital anomaly of rotation of the midgut
- > can lead to volvulus and infarction of entire midgut
Describe the presentation of Intestinal Malrotation.
- Bilious vomiting (below ampulla of Vater)
- Abdo pain
- Tenderness (peritonitis / ischaemic bowel)
What investigations should you do if you suspect Intestinal Malrotation?
- Upper GI contrast study (diagnostic)
- USS abdo
What is the management of Intestinal Malrotation?
- Surgical correction
- Ladd’s procedure -> rotates bowel anti-clockwise
What is the pathophysiology behind Necrotising Enterocolitis?
- Bacterial invasion of ischaemic bowel wall
- More common in babies fed with cow’s milk
How does NEC present?
- Bilious vomiting (below ampulla of Vater)
- Abdo pain and distension
- Fresh blood in stool
- Infant may rapidly become shocked
What investigations should you do if you suspect NEC?
- If shocked: septic work up
- Abdo xray:
> distended loops of bowel
> thickening of bowel wall with intramural gas
> Rigler and Football sign
What’s your management of NEC?
If shocked: A - E assessment
- Stop oral feeds
- Broad spectrum Abx
- Surgery for any bowel perforation
List 2 complications of NEC.
- Bowel perforation
- Malabsorption if extensive ischaemia
When is the football sign seen in children with NEC?
- Seen with massive pneumoperitoneum
- in supine position, air collects anterior to abdominal viscera
What is Hirschsprung’s disease?
- Large bowel obstruction
- Absence of ganglionic cells from myenteric plexus of large bowel.
- results in a narrow, contracted segment of bowel.
- Commonly: ileum moves into caecum via ileocaecal valve
How does Hirschsprung’s disease present?
- Failure to pass meconium within 48 hrs of life
- Boys > > girls
- Associated with Down’s syndrome
List 3 clinical features of Hirschsprung’s disease
- Abdo distension
- Later: bile stained vomit
- Can lead to Enterocolitis from C. diff infection
What investigations should you order for Hirschsprung’s Disease?
- Rectal examination: narrow segment. withdrawal causes flow of liquid stool and flatus
- Suction rectal biopsy (diagnostic)
What is the management for Hirschsprung’s disease?
- Enema’s
- Surgical resection of affected colon.
What causes jaundice?
Raised serum bilirubin -> leads to yellow skin and yellow sclera
-> due to increased RBC breakdown -> release of Haemoglobin
Why is high unconjugated bilirubin bad?
- Can be deposited in basal ganglia
- Kernicterus
-> encephalopathy with seizures and coma.
Can cause choreoathetoid cerebral palsy.
What are the causes of jaundice in the first 24 hours of life?
- Haemolytic disorders -> Rhesus incompatibility, ABO incompatibility, G6PD deficiency, spherocytosis, Pyruvate kinase deficiency
- Congenital infection -> Toxoplasmosis, CMV, Syphilis, Rubella, Herpes, Hepatitis
What are the causes of jaundice from 24hrs - 2 weeks of life?
- Physiological jaundice
- Breast milk jaundice
- Infection, haemolytic disorders, bruising, Crigler-Najjar syndrome.
What are the causes of jaundice beyond 2 weeks of life?
SERIOUS
- Hypothyroidism, pyloric stenosis
- Conjugated: biliary atresia, neonatal hepatitis
What investigations should you do for ?jaundice?
- TORCH screening
- Coomb’s test
Mx of jaundice?
- Phototherapy (450nm, converts unconjugated bilirubin into harmless soluble pigment)
- Exchange transfusion
A child has bile stained vomit. Differentials?
- Intestinal obstruction (distal to Ampulla of Vater)
- malrotation
- duodenal atresia
- meconium ileus
- NEC
A child has haematemesis. Differentials?
- Oesophagitis
- Peptic ulderation
- Oral / nasal bleeding
A child has projectile vomiting in the first few weeks of life. Diagnosis?
Pyloric stenosis
A child vomits at the end of paroxysmal coughing. Diagnosis?
Whooping cough.
A child has tenderness/abdo pain on movement. Diagnosis?
Surgical abdomen
A child has abdo distension. Differentials?
- Intestinal obstruction (incl. strangulated inguinal hernia)
A child has hepatosplenomegaly. Differentials?
- Chronic liver disease
- Neonatal hepatitis
- Biliary atresia
- Primary sclerosing cholangitis
A child has blood in their stool. Differentials?
- Intussusception
- Gastroenteritis (Salmonella / Campylobacter)
A child is severely dehydrated and shocked. Differentials?
- Severe gastroenteritis
- Systemic infection (UTI, Meningitis)
- DKA
A child is classed as ‘Failure to Thrive’ (drops 2 deciles). Differentials?
- GORD
- Coeliac disease
- Cow’s Milk Protein Allergy
- Other chronic GI conditions.
What triad of symptoms is seen in Nephrotic syndrome?
- Hypoalbuminaemia <25g/l
- Proteinuria: urine protein >1mg/m2/24hrs
- Oedema: peripheral, scrotal/vulval, periorbital, ascites
What are the common causes of Nephrotic syndrome?
- Minimal change disease
- Focal-segmental glomerulosclerosis
- post streptococcal nephritis
Treatment for steroid-sensitive Nephrotic syndrome.
Prednisolone oral 60mg/m2/day
Treatment for steroid resistant nephrotic syndrome.
- Diuretics, salt restriction, ACEi, NSAIDs
- Cyclophosphamide +/- cyclosporin
3 things for Haemolytic uraemia syndrome?
- Acute renal failure
- Thrombocytopenia
- Microangiopathic haemolytic anaemia
Describe the course of Haemolytic Uraemia Syndrome.
- follows prodrome of bloody diarrhoea (E. coli)
Describe the signs & symptoms of HUS.
- Abdo pain
- Decrease urine output
- Normocytic anaemia
Investigations for HUS?
- Fragmented blood film
- Stool culture
- FBC
Treatment for HUS?
- Supportive
- Plasma exchange if severe
Complications of HUS?
- HTN
- Chronic renal failure
3 things for Henoch-Schonlein Purpura (HSP)?
- Purpura (raised like sandpaper)
- Arthritis
- Abdo pain
Describe the course of HSP?
Follows URTI - Strep pyogenes
Signs and symptoms of HSP?
- Rash on buttocks, extensor surfaces of limbs
- Haematuria
- Proteinuria
Treatment for HSP?
Prednisolone (oral)
What is HSP associated with?
- Buerger’s / IgA nephropathy
episodes of macroscopic haematuria post URTI
Characteristic features of a tonic clonic seizure?
Stiffen -> jerk + Loss of consciousness
Characteristic features of an absence seizure?
Brief pause, eyes roll up, unaware
Characteristic features of West Syndrome?
- Head nodding
- Arm jerk
- EEG shows hypsarrhythmia
Define status epilepticus.
Defined as >30mins tonic clonic, but treat at 5 mins.
Buccal midazolam OR
IV Lorazepam ->IV phenytoin -> Rapid Sequence induction
What is a febrile convulsion?
Single, tonic clonic episode
- Lasts less than 20mins
- High fever
- Treat if lasts longer than 5 mins.
What organisms cause Meningitis?
- Neiserria meningitidis (meningococcal disease)
- H influenzae
- S. pneumonia
- E. coli (neonates)
- Group B strep
- Listeria monocytogenes
- TB
How might a child with meningitis present?
- Signs of sepsis
- Generally unwell
- Poor feeding
- Stiff neck
- Seizures
- Apnoea
- Lethargy
- Meningeal signs
- Non-purpuric rash
Investigations for meningitis?
- Septic screen
- Cultures
+/- Lumbar Puncture
When is a Lumbar Puncture contraindicated?
- focal neurology
- DIC
- Purpura
- Coning risk
How is Meningitis treated?
- Get urgent senior help
- Admit child
- Community: give IM BenPen
- Hospital: IV Ceftriaxone
- Prophylaxis: Rifampicin PO / Single dose IM Ceftriaxone
What is the triad for Screening Criteria?
- Identifiable when latent / early stage disease
- Condition is treatable
- Early identification = better prognosis
What are the aims of screening?
- Aims to identify unrecognised disease in apparently well people
- Allocation of funding on cost-benefit basis
What investigation is carried out to identify Sickle Cell Anaemia or Thalassaemia? (and when is it carried out?)
Maternal blood test
0 - 10 weeks
The triple test screens for Trisomy. What does the Triple Test use as serum markers and when is this test carried out?
- hCG
- PAPP-A
- USS nuchal translucency
Triple test: 11 - 13 weeks
Which trisomy disorders is the triple test looking to identify?
- Down’s (T21)
- Patau’s (T13)
- Edward’s (T18)
The quadruple test screens for trisomy. What does the quadruple test use as serum markers and when is this carried out?
- AFP
- Estriol
- hCG
- Inhibin-A
Quadruple test: 15 - 20 weeks
If the triple or quadruple test identifies a person with a higher risk of trisomy, what tests can be offered?
CVS
Amniocentesis
What and when is the congenital anomaly screen?
- A detailed US scan
- At 18 - 20 weeks
What does the Congenital Anomaly Screen look for?
- Neural tube defects
- Major heart defects
- Renal genesis
- Skeletal / CNS abnormality.
What 6 conditions does Guthrie’s test (heel prick) screen for?
- Sickle cell disease
- CF
- Congenital Hypothyroidism
- Phenylketonuria
- MCAD
- MSUD (maple syrup urine disease
Tell me about CVS.
- 11 - 13 weeks gestation
- Placental biopsy of foetal cells
- 2% risk of miscarriage
- Detects chromosome disorders and inherited disorders
Tell me about Amniocentesis.
- 15 - 20 weeks gestation
- Amniotic biopsy of foetal cells
- 1% risk of miscarriage
- Detects chromosome and sex determination
2 conditions of the newborn identified by karyotyping.
- Turner’s
- CAH
Name a condition which is identified by specific gene testing.
- Cystic Fibrosis
- > CFTR gene
How might a child with Down’s syndrome present?
- Intellectual disability
- Stunted growth
- Dysmorphia
Signs of Down’s Syndrome?
- Umbilical hernia
- Hypotonia
- Macroglossia
- Epicanthic fold
- Single palmar crease
- Abnormal outer ears
- Flattened nose
Conditions which Down’s syndrome is associated with?
- CHD
- AVSD > VSD
- Visual + hearing problems
- Duodenal atresia
- Haematological cancers
- Early onset dementia
- Thyroid disease
Describe your management plan for a child with Down’s syndrome.
- ECHO at birth
- Regular hearing, visual, dental check ups
- Educational and social support
- Thyroid and Coeliac screening
How does Patau’s syndrome arise?
- Nondisjunction of chromosomes during meiosis
List some signs + symptoms of Patau’s syndrome
- Polydactyly
- Midline defects (cleft lip / palate)
- Heart defects (VSD, PDA)
- Global developmental delay
What is the prognosis for someone with Patau’s syndrome?
- Many die in utero
- 80% of live births die within 1 year.
What is Edward’s syndrome associated with?
- IUGR
- Polyhydramnios
Signs and symptoms of Edward’s syndrome?
- Prominent occiput
- Kidney malformation
- Developmental delay
- Midline defects (cleft lip / palate)
- Heart defects (VSD / PDA)
Prognosis for a kid with Edward’s syndrome?
- Only 3% affected make it to live birth.
Median survival is 15 days.
Tell me about Turner’s syndrome.
45 XO
- Female with single deletion / partial copy of X chromosome in some or all cells.
- > excellent prognosis for live births
Signs / symptoms of Turner’s syndrome?
- Short stature
- Shield chest
- Low set ears
- Webbed neck
- Micrognathia
- Wide spaced nipples
What is Turner’s syndrome associated with?
- Amenorrhoea
- Delayed puberty
- Sterility
- Coarctation of the Aorta
- Bicuspid aortic valve
- Obesity
- Horse shoe kidney
- Thyroid disorder
Treatment for Turner’s syndrome?
- Growth hormone
- Oestrogen replacement (COCP)
- Fertility
