Paeds Flashcards
How do you estimate the weight of a child?
Weight (kg) = (Age in years + 4) x 2
What are the two causes of dehydration?
- Reduced intake
2. Increased losses
Give 4 causes of reduced intake (leading to dehydration):
- Dysphagia e.g. cerebral palsy, developmental delay
- Vomiting e.g. gastroenteritis, GORD
- Behavioural/Psych e.g. anorexia
- Social e.g. child neglect
Give 4 causes of increased losses (leading to dehydration):
- Gut losses e.g. IBD, stoma
- Kidney losses e.g. Nephrogenic diabetes insipidus
- Skin e.g. burns, cystic fibrosis
- Lungs e.g. tracheostomy, cardioresp diseases
Give 3 signs of moderate dehydration:
- Sunken eyes
- Reduced skin turgor
- Decreased urine output
Give 3 signs of severe dehydration:
- Reduced consciousness
- Cold mottled peripheries
- Anuria
Give 3 signs of mild dehydration:
- Thirst
- Dry lips
- Restless/irritable
Give 2 consequences of chronic deprivation from fluids or feeds:
- Failure to thrive/malnutrition
- Developmental delay
- Constipation
- UTIs
How do you work out maintenance fluids in children?
First 10kg = 100ml/kg daily
Next 10kg = 50ml/kg daily
Every other kg = 20ml/kg daily
A 7 year old girl needs maintenance fluids, she weighs 26kg - calculate the volume and rate:
1st 10kg: 10 x 100 = 1000ml/day
2nd 10kg: 10 x 50 = 500ml/day
Last 6kg: 6 x 20 = 120ml/day
1000 + 500 + 120 = 1620ml/day
= 67.5 ml/hr
What type of fluids do you give for maintenance in kids?
0.9% sodium chloride + 5% glucose
What type of fluids do you give for deficit in kids?
0.9% sodium chloride + 5% glucose
How do you work out the volume and rate needed for deficit fluids in kids?
Deficit (%) x 10 x weight (kg)
What type of fluid do you give for a bolus in kids?
0.9% sodium chloride
What volume of fluid do you give as a bolus? Are there any exceptions?
20 mls/kg UNLESS: - Trauma (too much fluid dislodges the clots the body is trying to form in response to blood loss) - DKA (risk of cerebral oedema) - Any other risk of cerebral oedema
Give 4 things you must monitor in a child receiving IV fluids:
- Normal obs (RR, O2, HR, BP, Temp)
- Neurological status (signs of cerebral oedema)
- Fluid balance (input and output)
- U&E - at least every 24hrs
What is the aim in giving a fluid bolus?
Restore bp and perfusion
How do you give maintenance fluids to neonates? At what rate?
Increase fluids over four days: Day 1 = 60mls/kg Day 2 = 90mls/kg Day 3 = 120mls/kg Day 4 = 150mls/kg
How much Na and K do neonates require per day?
Na: 2-3 mmol/kg/day
K: 1-2 mmol/kg/day
What type of fluid is given to neonates for maintenance?
10% dextrose with personalised amounts of Na and K added
Definition of a UTI:
10^5 organisms/ml grown on a culture of an appropriate sample
What is acute cystitis?
A lower urinary tract infection
What is acute pyelonephritis?
An upper urinary tract infection
Give 3 long term complications of UTI in children:
- Kidney scarring
- HTN
- CKD
Give 3 long term complications of UTI in children:
- Kidney scarring
- HTN
- CKD
What is the most common bug responsible for UTIs in kids?
E.coli
Which bug is more common in boys with UTI?
Proteus
Give a situation in which you would need to investigate a child with UTI further:
Atypical bug grown e.g. pseudomonas, klebsiella
Give 4 situations in which you would need to investigate a child with UTI further:
(i.e. for structural abnormalities, scarring, underlying pathology)
- Atypical bug grown e.g. pseudomonas, klebsiella, anything not E.coli!
- Failure to respond to suitable abx within 48hrs
- Required IV abx
- Raised creatinine
- Poor urine flow
- Recurrent UTI (2 or more with at least one including systemic signs, OR; three or more without systemic signs)
- Abdo mass/bladder mass
How do you treat UTI in a child <3 months old?
Minimum 2 to 4 days IV abx
Followed by oral abx
A child older than 3 months presents with a fever >38 degrees and loin pain. A diagnosis of UTI is reached - how long should you give abx for and by what route?
Systemically unwell +/- loin pain = likely UPPER UTI
Therefore, give: 7 to 10 days oral abx
OR: IV abx for 2-3 days, followed by oral abx for a total duration of 10 days
Abx = cefalexin or co-amox
Consider IV depending on clinical judgement, use a lower threshold for IV abx in younger children and those with significant risk factors or severely ill
How do the symptoms of an upper UTI differ from a lower UTI?
Upper: fever, vomiting, loin pain
Lower: dysuria, freq., mild abdo pain, enuresis
How should you treat a lower UTI in a child >3 months? (route and duration of abx)
Oral abx for 3 days.
Reassess in 24-48hrs.
Abx = trimethoprim or nitrofurantoin
Give 3 examples of abx you might use to treat UTI:
- Trimethoprim
- Cefalexin
- Co-amoxiclav
- Nitrofurantoin
Abx choice, route & duration for:
- UTI in <3 months old
- Upper UTI in >3 months old
- Lower UTI in >3 months old
- IV min. 2 to 4 days, followed by oral
- Oral 7 to 10 days, OR IV 2 to 3 days followed by oral (cefalexin or co-amox)
- Oral 3 days, reassess in 24-48hrs (trimethorpim or nitro or amox or cefalexin)
Name 3 further investigations you might do in a child with atypical UTI:
- Micturating Cystourethrogram (MCUG)
- DMSA scan
- USS of urinary tract
What is an MCUG? What does it look for?
Micturating Cystourethrogram: Looks for vesicoureteral reflux using a dye inserted into the bladder to observe the direction and flow of urine
What is posterior urethral valve (PUV)?
A congenital malformation in which the urethra is obstructed by flaps of tissue.
Only in boys.
1 in every 8000 male births.
Why does PUV cause hydronephrosis?
The bladder pushes hard to get urine past the blockage, increasing the pressure and pushing urine back into the ureters and kidneys. This causes the kidneys and bladder to swell and leads to damage.
How is PUV diagnosed?
Routine USS during pregnancy or picked up after birth when investigating urinary symptoms
How is vesicoureteral reflux graded?
1 to 5, 5 being the worst, likely requiring surgical correction
Give 3 features of nephrotic syndrome:
- Heavy proteinuria (frothy urine)
- Hypoalbuminemia
- Oedema
What is a common cause of nephrotic syndrome?
Minimal change disease
What is minimal change disease? How does it present?
Podocytes are the basic unit of the glomerulus. They have processes which act as a filter. In minimal change disease the podocyte processes are damaged, making the filter leaky, allowing through large protein molecules. This manifests as: nephrOtic syndrome.
Give 6 investigations needed in a child with suspected nephrotic syndrome and explain your choices:
- Urine dipstick & analysis to check for protein and haematuria
- FBC to check albumin level
- MSU to rule out UTI
- LFTs to exclude liver pathology
- Bone profile (calcium, phosphate, alkaline phosphates)
- U&Es
- ESR, CRP, glucose, Igs, Hep B, Hep C, HIV
How do you measure proteinuria? What is normal?
Use a urine sample from first thing in the morning.
Normal = creatinine < 20mg/mmol
There is no level that = definitely nephrotic
What is a normal range for albumin levels?
Approx. 35-45 g/L
Why does the risk of thrombosis and infection increase in nephrotic syndrome?
Along with albumin loss, you also lose other proteins e.g. antibodies and clotting factors
How do you treat the first episode of steroid sensitive nephrotic syndrome?
- 60 mg/m² for four weeks
- 40 mg/m² on alternate days for four more weeks
What further treatment can you offer a child who is having frequent relapses of steroid sensitive nephrotic syndrome?
Prophylactic steroids.
Alternative immunosuppressants e.g. Levamisole
What are 3 key questions to ask the mum in a labour history to determine the risk of neonatal sepsis?
- When did your water’s break? How long was this before delivery? (prolonged rupture of membranes)
- Did you have any fevers before, during or after labour?
- Were you told you had/have group B strep? If yes, were you given abx? How long for?
What is primary urinary incontinence?
A child who has never been dry for more than 3 months
What is secondary urinary incontinence?
A child who was dry for a period of more than 3 months, but is now incontinent
How are day symptoms of urinary incontinence managed in children?
- Behavioural advice
2. Oxybutynin & laxatives
How are night time symptoms of urinary incontinence managed in children?
- Behavioural advice:
- Drink >6 cups of water/day
- Drink majority of fluids before 4pm
- Stop drinking an hour before bed
- Aim for 4-7 voids/day - Behaviour change devices e.g. ‘wobble watch’ alarm
- Desmopressin:
- Last resort
- Taken an hour before bed
- Cannot drink at all for 8 hours following (risk of hyponatremia if they do drink)
Why might you do non-invasive bladder studies? What do they involve?
To investigate urinary incontinence.
The child is asked to drink a lot of fluid and then wait until they really need a wee. The volume of the bladder is then measured on USS before and after voiding. Pressure during voiding is also measured.
Why might you do non-invasive bladder studies? What do they involve?
To investigate urinary incontinence.
The child is asked to drink a lot of fluid and then wait until they really need a wee. The volume of the bladder is then measured on USS before and after voiding. Pressure during voiding is also measured.
Epidemiology of nephrotic syndrome:
- More common in Asian families
2. Boys > Girls
3 key features of nephritic syndrome:
- Coca cola coloured urine (haematuria)
- Reduced urine output
- Hypertension
(4. Oedema, less than in nephrotic)
Name a common cause of nephritic syndrome:
Acute Post-streptococcal Glomerulonephritis (PSGN)
Explain the pathophysiology of Acute PSGN:
Group A Beta-Haemolytic strep has a specific surface antigen and produces an enzyme (streptolysin) that can completely lyse red blood cells.
It initiates a type III hypersensitivity reaction in which immune complexes of antigens and antibodies are formed (IgM and IgG).
These are deposited in the glomerular basement membrane.
This initiates an inflammatory reaction in the glomerulus.
This damages the podocytes, causing them to allow through large molecules like RBCs and protein, as well as producing less urine.
Leads to: haematuria, (some) proteinuria and oliguria.
What results might you see in acute PSGN for the following tests:
- FBC
- U&Es
- Immunoglobulins/Immunology
- Urinalysis `
- Mild normochromic, normocytic anaemia
- Increased urea and creatinine
- Anti-DNase B, low C3/C4
- Haematuria (macroscopic), proteinuria
How do you manage acute PSGN? (3)
- Supportive fluids:
- Measure input/output
- Restrict salt
- Diuretics?
- Correct electrolyte imbalances - Penicillin for strep (2 weeks)
- Dialysis if needed (uncommon)
There are 3 types of AKI: pre-renal, renal, post-renal. Which type is acute PSGN?
Renal
What is wheeze?
An expiratory polyphonic sound due to turbulence through narrowed airways
What might a monophonic expiratory wheeze indicate?
A mass
What is stridor?
Turbulent airflow through a partially obstructed upper airway
Heard more on inspiration
Seen in croup and epiglottitis
What age group is croup typically seen in?
6 months to 2 years
How might croup present? (3)
- Stridor
- Worse at night
- +/- Low grade fever
What is the most common cause of croup?
Parainfluenza virus accounts for 75% of all cases
How might acute epiglottitis present? (4)
- High fever
- Drooling
- Tripod stance (neck strained upwards)
- Soft stridor
- Muffled voice
What is the most common cause of acute epiglottitis?
H.influenzae type B
Give 6 signs of respiratory distress:
- Tachypnoea
- Intercostal and sternal recession
- Use of accessory muscles (head bobbing in infants)
- Tripodding or anchoring
- Nasal flaring
- Inspiratory/expiratory noises e.g. stridor, wheeze, grunting
What is the first line treatment for croup?
Oral dexamethasone single dose 150 micrograms/kg
A child seen in A&E with croup begins to rapidly desaturate - what emergency medication should you give and what dose?
Nebulised adrenaline with an oxygen face mask 500 micrograms/kg
What risks should you be aware of when adrenaline begins to wear off in a child with respiratory distress?
Risk of: 1. Bronchospasm 2. Worsening resp distress 3. Tachycardia Be prepared to intubate
What is bronchiolitis?
An acute viral infection of the lower respiratory tract with inflammation of the bronchioles
What is the most common cause of bronchiolitis?
Respiratory syncytial virus (RSV)
NICE criteria for suspecting bronchiolitis in children under 2 years old:
A 1 to 3 day history of coryzal symptoms followed by:
- Persistent cough and
- Tachypnoea and chest recession or
- Wheeze/crackles on chest auscultation
How do very young babies with bronchiolitis present?
With apnoea and no other signs
Typical features of bronchiolitis:
- Persistent cough
- Tachyponea
- Increased WOB
- Low grade fever (less than 39)
- Coryza
In a child with suspected bronchiolitis, when might you worry about pneumonia?
If temperature is above 39 and there are persistent focal crackles on auscultation
What are the 3 main conditions to consider in a child presenting with stridor?
- Croup
- Epiglottitis
- Tracheitis
How does bacterial tracheitis present?
- Rapid onset
- Fever >39
- Cough
- Hoarse voice
NB: Seen in 6 months to 14 years old, rare condition
How do you manage bronchiolitis? (primary and secondary care)
Primary care: - Anti-pyretic - Careful safety netting advice (e.g. virus is self-limiting, peaks at days 3-5, come back if needed) Secondary care: - Admit if hypoxic - Viral throat swabs/NPA - IV fluids - NGT feeding - Humidified oxygen
NB: Normally self-limiting condition
3 Biological risk factors for bronchiolitis in children:
- Chronic lung disease (CF, chronic lung disease of prematurity)
- Congenital heart disease
- Immunocompromised
3 Environmental risk factors for bronchiolitis in children:
- Older siblings
- Nursery attendance
- Passive smoking
What is an NPA? Why is it done in suspected bronchiolitis?
Nasopharyngeal aspiration is a rapid test of the causative virus
Determines whether to barrier nurse as RSV is very infective
How can bronchiolitis be prevented and in what group of patients would this be done?
Immunoprophylaxis with palivizumab
A monthly IM injection during winter months (Nov to March)
Given to those at high risk of severe RSV bronchiolitis:
- Very premature babies
- SCIS
- Heart disease
- Lung conditions e.g. cystic fibrosis
What is the Guthrie card?
Newborn heel-prick test done at five days old
What does the Guthrie card screen for?
- Cystic fibrosis
- Congenital hypothyroidism
- Sickle-cell disease
- 6 rare inherited metabolic diseases including:
- Maple syrup urine disease
- Phenylketonuria (PKU)
If a child has a positive result for cystic fibrosis on their Guthrie card, how do you confirm the diagnosis?
- Test for the CFTR gene, then;
- A positive sweat test >60mmol/L and one of:
- Typical COPD
- Exocrine pancreatic insufficiency
- Positive family hx (often a sibling)
What is the pattern of inheritance for cystic fibrosis?
Autosomal recessive
What is the most common mutation seen in cystic fibrosis?
CF is caused by a mutation in the CFTR gene on chromosome 7
The most common mutation is DF508.
This is a class II mutation which results in incorrect folding.
What do CFTR proteins do?
They are ion channels that regulate the movement of chloride ions across epithelial membranes
What does CFTR stand for?
Cystic fibrosis transmembrane conductance regulators
How do faulty CFTR proteins result in the symptoms of cystic fibrosis?
Decreased secretion of Cl- and increased resorption of Na+
Results in reduced epithelial lining fluid and reduced hydration of mucus
Mucus is stickier to bacter promoting infection
Increased viscosity of secretions from the resp tract, pancreas, GI tract and sweat glands
How does CF present perinatally?
- Picked up on screening
- Bowel obstruction with meconium ileus
- Rectal prolapse
- Haemorrhagic disease of the newborn
- Prolonged jaundice
What is haemorrhagic disease of the newborn? Why does it occur in babies with CF?
A rare form of bleeding disorder due to low stored of vitamin K at birth. Typically presented with intracranial haemorrhage.
Babies with CF are at risk of developing deficiencies in fat soluble vitamins due to pancreatic insufficiency.
Give 5 ways in which CF presents during infancy/childhood:
- Acute pancreatitis
- Failure to thrive
- Portal hypertension and variceal haemorrhage
- Sinusitis and nasal polyps
- Pseudo-bartter’s syndrome
Give 2 ways in which CF might present during adolescence/adulthood:
- Male infertility with congenital bilateral absence of the vas deferens
- Osteoporosis/malacia (due to malabsorption of calcium)
Describe the journey of blood from the placenta in fetal circulation:
- Oxygenated blood leaves the placenta
- Enters the umbilical vein
- a) 70-80% goes into the portal vein, then through the liver, then into the hepatic vein, then into the IVC
b) 20-30% into the ductus venosus and then into the IVC - From the IVC to the right atrium
6.
a) Some blood goes from the RA through the foramen ovale to left atrium, then from the LA to the aorta
OR;
b) Some blood goes into the right ventricle then to the pulmonary artery via the ductus arteriosus to the aorta - From the aorta blood is carried around the body and to the internal iliac arteries
- From the internal iliac arteries blood goes to the umbilical arteries
- Then back again to the placenta
What triggers the closing of the ductus arteriosus after birth?
A reduction in circulating prostaglandins
What is an ostium secundum?
An atrial septal defect in the middle of the septum
Allows blood to shunt from the left atrium into the right
What is an ostium primum?
An atrial septal defect at the bottom of the septum, close to the mitral and aortic valve
Also associated with interventricular septal defects
What is a sinus venosus ASD?
A very rare and difficult to detect ASD at the very top of the atrial septum
What might an ASD sound like on auscultation?
A systolic murmur in the pulmonary area
Fixed and widely split second heart sound
What might an ASD sound like on auscultation?
An ejection systolic murmur in the pulmonary area (caused by increased flow across the pulmonary valve)
Fixed and widely split second heart sound (caused by a delay in the pulmonary valve closing)
May radiate to the back
What is a ‘split s2’ heart sound?
A normal S2 heart sound is the aortic and pulmonary heart valves closing simultaneously
Any pathology that causes these closures to be out of sync will cause a “split” S2 sound
For example, in an ASD the closure of the pulmonary valve is delayed, splitting S2
What is a ‘split s2’ heart sound?
A normal S2 heart sound is the aortic and pulmonary heart valves closing simultaneously
Any pathology that causes these closures to be out of sync will cause a “split” S2 sound
For example, in an ASD the closure of the pulmonary valve is delayed, splitting S2
How does an ASD present? (3)
Asymptomatic when younger
Older children and adults get signs/symptoms due to chronic volume overflow dilating the atria e.g. murmur & palpitations
Stretching of the RA can lead to RBBB
What is a restrictive VSD?
A small ventricular septal defect
Causes few signs and symptoms but carries a risk of infection (infective endocarditis)
What murmur does a VSD cause?
Harsh loud pansystolic murmur, loudest at the lower left sternal edge
Transmits to the upper sternal edge and axillae
How might a child with a VSD present? (2 symptoms & 5 signs)
- Poor feeding
- Failure to thrive
- Tachypnoea
- Hepatomegaly
- Oedema
- Harsh pansystolic murmur at lower left sternal edge
What type of heart murmur is often associated with trisomy 21?
AVSD
What murmur will you hear with a PDA?
Continuous machinary murmur in the pulmonary area
What vessels does the ductus arteriosus connect?
Pulmonary artery and aorta
2 symptoms and 3 signs seen in a baby with a PDA:
- Poor feeding
- Failure to thrive
- Tachyponea
- Continuous machinery murmur in pulmonary area
- Hepatomegaly
- Oedema
- Easily palpable femoral pulses
NB: Often preterm babies
How do you manage a left to right shunt in a newborn? (5)
- Increase calorie intake to compensate for catabolic state, probably through an NG feed
- Diuretics for oedema/HF (furosemide)
- ACE inhibitor (captopril - reduces afterload)
- Surgical or catheter device occulusion
What is coarctation of the aorta?
Narrowing of the aorta, causing reduced/no blood flow to the lower limbs
Baby depends on keeping their ductus arteriosus open to survive
3 signs of coarctation of the aorta in a newborn:
- Weak femoral pulses (always compare to brachial)
- Discrepancy between upper and lower limb BP
- Pre and post ductual difference in saturations (if ductus arteriosus is open)
NB: There will be a murmur over the back once colaterals have developed in older children
NB: If the ductus arteriosus has closed/is closing before collaterals have developed, the baby will present collapsed and acidotic
What type of murmur does aortic stenosis cause?
Ejection systolic murmur in the aortic region
What type of mumur does pulmonary stenosis cause?
Eejction systolic murmur in the left upper sternal edge
Radiates to the back, especially if pulmonary branches are also stenosed
What is transposition of the great arteries?
Pulmonary artery and aorta are attached in the wrong places
Circulation is in parallel, oxygenated blood in the lungs, de-oxygenated blood in the body
Survival is aided by the foramen ovale
How do you manage transposition of the great arteries? (2)
Initial temporary management = atrial septostomy at birth to make the foramen ovale even larger and promote mixing of blood
Surgical repair within a week to prevent RV failure
What are the four features of tetralogy of fallot?
- Infundibular thickening - narrowing of the pulmonary artery
- Ventricular septal defect
- Overriding aorta
- Right ventricular hypertrophy
What causes a hypercyanotic spell in tetralogy of fallot?
As the pulmonary artery narrows blood flow to the lungs decreases and deoxygenated blood goes via the septal defect into the body via the aorta
What syndrome should you rule out in a child with tetraology of fallot? How?
Check for 2q deletion, associated with digeorge syndrome
When does pyloric stenosis typically present?
In the second to fourth weeks after birth
How does pyloric stenosis typically present in newborns? (4)
- Projectile vomiting, typically 30 minutes after a feed
- Constipation, dehydration, weight loss, persistent hunger
- Palpable ‘olive’ mass in the right upper quadrant or epigastrum
- Hypercholraemic, hypokalaemic alkalosis due to persistent vomiting
What electrolyte abnormality is commonly associated with pyloric stenosis in newborns?
Hypochloremic hypokalaemic metabolic alkalosis
Explanation: Persistent vomiting causes progressive loss of fluids which contain hydrochloric acid, in turn this causes the kidneys to retain hydrogen ions in favour of potassium. There may be no electrolyte abnormalities if the duration of the illness is short.
Risk factors for pyloric stenosis in newborns: (3)
- M>F (4 times more common in M)
- Positive family Hx
- First born
Give 4 differential diagnoses to consider when thinking of pyloric stenosis:
- Feeding problem or milk intolerance
- GORD
- Gastroenteritis
- Other bowel obstruction e.g. duodenal atresia, osophageal atresia
- Intestinal malrotation/acute midgut volvulus
What surgery is done to manage pyloric stenosis?
Ramstedt’s pyloromyotomy
What is pyloric stenosis?
Thickening of the muscle of the pylorus makes the passage between the stomach and small bowel (duodenum) narrower.
What is a congenital diaphragmatic hernia?
Severity ranges from thinning of an area of the diaphragm to complete absence of the diaphragm.
CDH may allow the stomach and intestines to move through an opening (hernia) into the chest cavity, crowding the lungs.
How does a congenital diaphragmatic hernia present in a newborn? (4 signs)
- Scaphoid abdomen (caved in)
- Respiratory distress (dysponea & tachyponea)
- Heart sounds louder on the right side
- Tinkling bowel sounds
What organism is responsible for causing scarlet fever?
Group A haemolytic streptococci
What would you see on an ECG of a child with a large VSD?
Right ventricular hypertrophy
What investigations would you order when diagnosing a large VSD?
- ECG = RVH
- CXR = signs of HF
- Echo (gold standard diagnostic test, with doppler to show direction of flow, exact size and location)
- Septic screen
- Pre and post-ductal stats
What is hypoplastic left heart syndrome?
A congenital heart condition where the left ventricle does not develop properly and so is much smaller than usual. The mitral valve is often closed or very small. The aorta is also small than usual.
What is Hirshsprung disease?
A condition in which nerve cells are missing at the end of a child’s bowel. Stool moves normally through the bowel until it reaches the area lacking nerve cells, at this point it moves slowly or stops and can lead to severe constipation or intestinal obstruction.
Neonatal signs/symptoms of Hirschsprung’s: (3)
- Abdominal distension
- Failure to pass meconium in first 48 hours of life
- Repeated vomiting
3 signs/symptoms of Hirschsprung’s in older infants & children:
- Chronic constipation resistant to usual treatments
- Early satiety
- Abdo discomfort and distension
- Poor nutrition and poor weight gain
Give 3 investigations necessary when diagnosing Hirschsprung’s disease:
- Plain abdo XR - may show obstruction and dilated lower bowel
- Anorectal manometry (measures pressures of anal sphincter muscles, sensation and neural reflexes)
- Rectal biopsy - histology for definitive diagnosis
How do you manage Hirschsprung’s disease acutely and surgically? (4)
- Treat any obstruction/constipation - rehydration, NG feeding, enemas
- Treat any enterocolitis - broad spectrum abx
- Surgical Swenson’s procedure: removes the aganglionic segment of bowel and creates an end-to-end anastomosis
- Other colo-anal anastomosis procedure
What is the first line treatment for constipation in children?
Movicol
Give 4 differential diagnoses for vomiting in a newborn:
- Gastroenteritis
- Pyloric stenosis
- Malrotation +/- volvulus
- TEF (types B/D/H)
- Necrotizing enterocolitis
- Milk protein intolerance
What is a TOF?
Tracheoesophageal fistula - a birth defect that can cause liquid to pass through an abnormal connection between the oesophagus and trachea
How might TEF present postnatally? (4 signs/symptoms)
- Choking/Feeding difficulties
- Respiratory distress
- Vomiting
- Unable to pass an NG tube
- Overflow of saliva and aspiration
What is an H-type TEF?
A connection between the oesophagus and trachea with NO oesophageal atresia (i.e. the oesophagus is completely functional and normal other than the extra connection)
What is NEC?
Necrotising Enterocolitis - a GI emergency in neonates that involves a combination of vascular, mucosal, toxic and other insults to the intestine, aetiology unknown
Croup is most often diagnosed clinically, however, if a CXR is done - what classic sign might be seen?
Steeple sign - tapering of the upper trachea
Give a syndrome that ADHD is commonly associated:
Fragile X syndrome
Give 4 conditions commonly associated with Down’s Syndrome:
- Hypothyroidism (also hyperthyroidism but hypo is much more common)
- Type 1 diabetes
- ToF
- Hirschsprungs disease
A health visitor finds abnormal hearing in a baby’s ‘Newborn Hearing Screening Programme’ check - what test should they be offered next?
Auditory Brainstem Response test as a newborn/infant
In suspected meningitis in an <3 month old, what abx should you give as well as IV cefotaxime? And why?
IV amoxicillin to cover for listeria
What is transient tachypnoea of the newborn (TTN)?
A delay in the resorption of fluid in the lungs after birth.
More common following c-section and is the most common cause of resp distress in newborns.
How do you treat transient tachypnoea of the newborn (TTN)?
Observe and give supportive care if necessary
Oxygen may be needed to maintain saturations
Should self-resolve in 48 hours following delivery
What immunisations are given at 2 months? (3)
- 6 in 1 vaccine (diptheria, tetanus, whooping cough, polio, Hib, hep B)
- Oral rotavirus vaccine
- Men B
What immunisations are given at 3 months? (3)
- 6 in 1 vaccine (diptheria, tetanus, whooping cough, polio, Hib, hep B)
- Oral rotavirus vaccine
- PCV
What immunisations are given at 4 months? (2)
- 6 in 1 vaccine (diptheria, tetanus, whooping cough, polio, Hib, hep B)
- Men B
What immunisations are given at 12-13 months? (4)
- Hib/Men C
- MMR
- PCV
- Men B
What immunisations are given at 3 to 4 years just before school starts? (2)
- 4 in 1 pre-school booster (diptheria, tetanus, whooping cough, polio)
- MMR
What immunisation is given at 12-13 years? (1)
- HPV
What immunisations are given at 13-18 years? (2)
- 3 in 1 teenage booster (tetanus, diptheria, polio)
2. Men ACWY
What triad is associated with shaken baby syndrome?
- Retinal haemorrhages
- Subdural haematoma
- Encephalopathy
If jaundice develops within the first 24 hours of life, is this physiological or pathological?
Always pathological
Give 4 possible causes of jaundice in the first 24 hours after birth:
- rhesus haemolytic disease
- ABO haemolytic disease
- hereditary spherocytosis
- glucose-6-phosphodehydrogenase
During what time period might physiological jaundice develop?
2 to 14 days after birth
NB: more commonly seen in breastfed babies
What does a prolonged jaundice screen include? (6)
- Conjugated and unconjugated bilirubin
- Direct antiglobin test (Coomb’s test)
- TFTs
- FBC and blood film
- Urine MC&S
- U&Es & LFTs
Give 4 possible causes of prolonged jaundice:
- Biliary atresia
- Hypothyroidism
- Galactosaemia
- UTI
- Breast milk jaundice
- Prematurity
- Congenital infections (CMV, toxoplasmosis)
What is William’s syndrome?
An inherited neurodevelopmental disorder caused by a microdeletion on chromosome 7
Give 4 features associated with William’s syndrome:
- Elfin-like face
- Sociable and friendly affect
- Short stature
- Learning difficulties
- transient neonatal hypercalcaemia
- Supravalvular aortic stenosis
Give 4 investigations needed in diagnosing leukaemia and what results you might see:
- FBC = anaemia, thrombocytopenia, neutropenia
- Blood film = blast cells
- Bone marrow aspiration and biopsy
- CXR = medial stinum widening
- Lumbar puncture
- Uric acid
What is neutropenia?
Abnormally low levels of neutrophils
What is thrombocytopenia?
Abnormally low platelets
What might medial stinum widening on a CXR indicate in a child presenting with bruising and frequent infections?
A mediastinal mass indicative of acute lymphoblastic leukaemia
A mediastinal mass is found at the time of diagnosis in 10% to 15% of children with ALL
Give 5 signs/symptoms of ALL:
- Bruising/petechiae
- Fever
- Fatigue
- Frequent infections
- Anaemia
- Organomegaly (liver or spleen)
- Lymphadenopathy
- Bone or joint pain
How are boys and girls with ALL treated differently?
Boys receive treatment for longer due to a higher chance of relapse
Give two groups of children who have an increased incidence of leukaemia:
- Down’s syndrome
- Faconi’s anaemia
- Ataxia-telangiectasia
- Bloom’s syndrome
Management of ALL: (2)
- High intensity chemotherapy via a central venous catheter (e.g. Hickman line) for 2 to 3 years
- Haemopoietic stem cell transplant in relapsed patients or high risk patients in first remission
What is the ‘double hit’ theory of cancer?
Cancer results from an interaction between environment and genetic susceptibility
