CHILD'S HEALTH 1 - Resp, ENT, Cardio, Gastro, Renal and GU Flashcards
What are the most common causes of Pneumonia (an acute lower respiratory tract infection) in
a) Neonates
b) Children <5
c) Children >5
Neonates –> Organisms from maternal genital tract (Group B Strep, E.Coli bacilli, gram -ve enterococci)
– Infants (<5 years) –> Respiratory viruses are most common, but bacterial infection also occurs
– Children (>5yrs) –> Strep pneumoniae (most common), mycoplasma and chlamydia
name some things seen in the presentation of children with pneumonia
Fever, cough and rapid breathing are the most common presenting symptoms.
Cough is typically wet, but not often that productive in children.
Viral LRTI:
Usually lower fever (less than 39 °C)
Chest signs usually bilateral
These are usually preceded by a URTI
In contrast to asthma, the most sensitive clinical sign of pneumonia in children is increased
respiratory rate
What are some signs seen in pneumonia? Why doesn’t pneumonia present with a wheeze?
What actually is affected/going on in a pneumonia?
Hear Crepitation’s in the lungs - (a later sign)
Stony dull percussion (an earilier sign)
Reduced air entry on auscultation
They will often look unwell
Because Pneumonia primarily affects the alveoli, cuasing inflammation and pus build up there.
Wheeze is caused by narrowing of the airways - pneumonia doesnt effect the tubes!! Hence why you’ll hear wheeze in resp conditions that do cause bronchoconstriction such as asthma and bronchitis
Outline the 4 phases of a lobar pneumonia
4 phases
- Congestion - blood vessels become filled with blood, making lung tiusse appear red and heavy
- Red hepatization - Alveoli fill with RBCs, and a small amount of fibrin, and other inflammatory cells
- Gray Hepatization - RBC decrease, fibrin and inflammatory cells increase - as tissue consolidates
Resolution - The inflammation exudate get reabsorbed and lung tissues goes back to normal
How do you investigations would you do for pneumonia
Clinical diagnosis!!!
– CXR –> Can be used to confirm diagnosis but cannot differentiate between viral and bacterial infection
– Sputum sample sent for culture to help determine antibiotic sensitivity
What can pneumonias look like on x-ray?
Consolidation -
Can lead to mucous plugs/gunk that stop air entry - leading to lobar collapse
Describe the treatment of pneumonia.
PO amoxicillin. Erythromycin second line
Co-amoxiclav if complicated or unresponsive.
O2, analgesia, IV fluids if indicated.
What antibiotics might you use in a child with pneumonia caused by mycoplasma pneumoniae?
Mycoplasma pneumoniae is intracellular and so amoxicillin won’t work therefore give macrolides e.g. clindamycin, erythromyocin.
along with Co amoxiclav
What may be happening if child doesn’t respond to penicillin sensitive bacteria eg strep pneumoniae or strep a pneumonia?
Source control - bacteria has moved and lead to an empyema - need chest drain
Name the infections that affect the Upper respiratory tract.
- rhinitis (viral)
- otitis media (bact and viral)
- pharyngitis (viral)
- tonsillitis (bact and viral)
- laryngitis (viral)
Name the infections that affect the lower respiratory tract.
bronchitis’ (b and v)
- croup (viral)
- epiglottitis (bacterial only )
- tracheitis(b and v)
- bronchiolitis (viral)
- pneumonia (b and v)
What is Croup? Who is most commonly affected by it?
Croup, also known as laryngotracheobronchitis, is a viral upper respiratory tract infection.
Children between 2 and 5 years old are most commonly affected
What are the causes of Croup?
Main cause: Parainfluenza virus
Influenza
Adenovirus
Respiratory Syncytial Virus (RSV)
What is the presentation of Croup?
Usually improves in <48 hours
Mild croup:
Occasional barking cough with no stridor at rest
No or mild recessions
Well looking child
Stridor, hoarse voice
Moderate croup:
Frequent barking cough and stridor at rest
Recessions at rest
No distress
Severe croup:
Prominent inspiratory stridor at rest
Marked recessions
Distress, agitation or lethargy
Tachycardia
“Barking” Seal like cough, occurring in clusters of coughing episodes - worse at night
Low grade fever
WHEN EXAMINING THEM, DO NOT AGITATE THEM, AND DO NOT LOOK IN THE BACK OF THEIR THROAT AS IT CAN CLOSE OFF THEIR AIRWAY
Rule out epiglottitis!!
always think of forgein bodies in these kind of presentations!!
What is the treatment of Croup?
A single dose of oral dexamethasone (0.15 mg/kg) is to be taken immediately regardless of severity - peaks at about 6 hours, and wears off after 12
In severe upper airways obstruction, nebulized
epinephrine (adrenaline) with oxygen by face mask - for bronchodilation - but wears off v quickly aka 5 mins - but can give as many as you need but if your giving adrenaline nebs then you need to call the anaestetist
Budesonide nebuliser (a steroid)
Remember cause is viral!
What is Acute Epiglotitis?
Epiglottitis refers to inflammation and localised oedema of the epiglottis, which can result in potentially life-threatening airway obstruction.
What is the most common cause of Epiglottitis? What is its classificiation?
Haemophilus Influenza B - (gram-negative coccobacillus)
Other organisms have become more common in the developed world, such asStreptococcus pneumoniaeandStreptococcus pyogenes.
What are the risk factors for Epiglottitis?
Peak age 6-12 (can occur at any)
Male gender
Unvaccinated
Immunocompromised
now far less common due to the introduction of HiB vaccine
What are the symptoms and signs of Epiglottitis?
Rapid Onset:
Dysphagia
Dysphonia (stridor)
Drooling
Distress
Stridor
Tripod Position: A sign of respiratory Distress
Lean forward, mouth open, tongue out = max air in)
Pyrexia
What is the primary investigation of Epiglottitis?
If acutely unwell then NO Ix but immediate Tx
Laryngoscopy:diagnostic and will demonstrate swelling and inflammation of the epiglottis
Lateral neck radiograph:securing the airway is the priority but, once done, an x-ray can be performed looking for thethumb sign (soft tissue shadow that looks like a thumb pressed into the trachea);
What is the management for epiglottitis?
EMERGENCY
First Line:
- Secure airway, Endotracheal intubation
- Nebulised adrenaline
- IV antibiotics - Amoxicillin, Co-Amoxiclav, Erythromycin, Doxycycline
Second Line:
- Dexamethasone
Outline some differences in clinical presentation of epiglottitis and croup
Croup has a slower onset
Severe barking cough in croup, slight cough in epiglottis
Drooling in seen in epiglotitis
Low grade fever in croup, higher in epiglottitis
What is the difference between wheeze and stridor?
Wheeze: polyphonic noise heard on expiration.
Stridor: monophonic high pitched noise heard on inspiration.
Stridor - upper RT,
Wheeze - Lower RT
Outline the Tucson models for childhood wheezes - what are the 3 categories and what are the causes for each
framework that categorizes wheezing patterns in young children into three groups:
Transient Early Wheezing (TEW): 0-3 years Peak
Causes:
Small airways
Mother smoking
Early viruses
Preterm
Normally will resolve by 3
Viral episodic wheeze: Peaks 3-6 years
RSV/ Other LRI
Airway hyper-reactivity
Associated with atopy (genetic tendency to develop allergic conditions).
Ig-E associated asthma - rises in incidence 3 years onwards and peaks at 6+
Associated with Atopy
Airway hyper-reactivity
What is asthma?
This is a disease which is due to reversible airway bronchoconstriction, most often due to allergic stimuli.
What are the symptoms of asthma?
Chest tightness
Episodic Dyspnoea/SOB
Wheeze
Dry Cough (typically but can be wet)
How would you treat a viral epidosic wheeze?
No benefit from regular inhaled steroids
Use bronchodilators
May use oral steroids
No interval symptoms and no excess of atopy
What may be seen on examination and spirometry for asthma?
History
* Cough after exercise or sometimes in the early morning, disturbing
sleep.
* Shortness of breath
Spirometry
* Peak expiratory flow rate (PEFR) <80% predicted for height.
- Peak flow rate (PEFR):measured multiple times a day over 2-4 weeks. Variability of >20% is diagnostic
* FEV1/FVC <80% predicted.
An improvement in FEV1 of 12% or more in response to either β2 agonists or corticosteroid treatment trials, together with an increase in volume of 200 ml or more, is considered diagnostic for asthma.
What is the stepwise managements of asthma in children Under 5?
Under 5 yrs old
Short-acting beta agonist – salbutamol
Add a low dose corticosteroid inhaler
Add leukotriene receptor antagonist (LTRA) - oral montelukast
What is the stepwise managements of asthma in children aged 5 - 12?
Start a SABA – salbutamol as required
Add a regular low dose corticosteroid inhaler - beclomethasone
Add a LABA inhaler - salmeterol.
Titrate up the corticosteroid inhaler to a medium dose.
Oral leukotriene receptor antagonist - montelukast
Increase the dose of the inhaled corticosteroid to a high dose.
Give some possible risk of long term ICS? What should doctors do to help manage this?
Adrenal suppression! -
Growth suppression - (in the short term, but they should catch up)
Osteoporosis? - no evidence for an increased risk of fractures - but maybe slightly less bone density
Main factor for BMD in adult life is weight bearing exercise - which may be imparied in uncontrolled asthma
So on balance, pros often outweigh the cons in terms of risk - just measure growth for kids on ICS
What are some signs of an acute asthma attack in children?
Mild: SaO2 >92% in air, vocalising without difficulty, mild chest wall recession and moderate tachypnoea.
Moderate: SaO2 <92%, breathless, moderate chest wall recession.
Severe: SaO2 <92%, PEFR 33-50% best or predicted, cannot complete sentences in one breath or too breathless to talk/feed, heart rate >125 (over 5 years old) or >140 (2-5 years old), respiratory rate >30 (over 5 years) or >40 (2-5 years).
Life-threatening: SaO2 <92%, PEFR <33% predicted, silent chest, poor respiratory effort, cyanosis, hypotension, exhaustion, confusion, A normal pCO2 When the patient has a high respiratory rate, one would expect a low CO2. Therefore, a high CO2 indicates exhaustion as they can no longer blow it off effectively.
What is the immendaite management of an asthma attack?
Oxygen: SaO2 <94% should receive high flow oxygen to maintain saturations between 94-98%.
Bronchodilators: Inhaled SABA (salbutamol) – via nebuliser if severe. Inhaler and spacer device is as effective as nebuliser in children with mild/moderate asthma attack.
Ipatropium bromide (anti-muscuranic) added in if no or poor response to inhaled SABA
Corticosteroids: A short course (3 days) or steroids should be commenced. Oral prednisolone is first-line
Steroid therapy should be given to all children who have an asthma attack, so for moderate asthma attacks give oral pred and salbutamol
What is the main cause of a virally induced wheeze? Who does it most commonly effect?
Kids under the age of 5.
Commonly RSV or rhinovirus.
What would a child with a Virally induced wheeze present with?
How is it different to asthma in its presentation?
cold like symptoms, fever, runny nose and an expiratory bilateral wheeze.
Generally not asthma if: Presenting < 3 years of age
No atopic history
Only occurs during viral infections
What is the treatment for a Virally induced wheeze?
Airway:
Oxygen
Breathing
Salbutamol inhalers or nebulisers
Dexamethasone if mod-severe
Circulation
If severe/life-threatening for:
IV access
IV hydrocortisone
IV Magnesium +/- IV Aminophylline +/- IV salbutamol
What is persistent infantile wheeze normally associated with/exacerbated by?
What ages is it most common in?
Persistent infantile wheeze tends to affect the small airways. It is associated with parental smoking or post-viral infection.
ages 3-6
but also cold air, dust, animal dander and exercise. This has been called multiple-trigger wheeze. In the preschool
age group, where a formal diagnosis of asthma may be
unjustified, this distinction is helpful as many children in
this group benefit from asthma preventer therapy and a
significant proportion go on to have asthma.
Are inhalers likely to help a child with persistent infantile wheeze?
No. Inhalers are unlikely to help; symptoms will improve as the child gets older.
What is bronchiolitis? Who is it most common in?
This is a lower respiratory tract infection which leads to the blockage of small airway in the lungs
– It is most seen in children younger than 2 and cases spike in autumn and winter
Commonest respiratory infection in infants
can make v young children v ill
What are the most common causes of bronchiolitis, and what things can make it more severe?
Most common – Respiratory Syncytial Virus (RSV)
Can be caused by Influenza, ADV
prematurity,
Being under the age of 2
Exposure to second hand smoke
Downs syndrome or cystic fibrosis, Congenital heart disease or immunodeficiency.
What are some investigations to do for bronchiolitis ?
Its a clinical diagnosis , dont need investigations
Can do viral swab and MPA - not to diagnose, but so we can isolate kids or put kids w the same viruses in together
What are some things you would see on Xray for bronchiolitis, and why?
you can count too many ribs anteriorly . 7 here ! the chest is hyperinflated, as the kids are maxing out their lung spaces to try and get enough air in
What are some symptoms of bronchiolitis?
General –> coryza, fever, irritability, poor feeding
– Dry cough
– Coryzal symptoms precede
– Wheeze and crackles on auscultation
– Respiratory distress –> chest wall recession, nasal flaring
– May experience pauses in breathing
Typical pattern of worsening symptoms on day 3-4 days of illness, but will get better within 3 weeks of supportive management
grunting is a severe symptom
A low-grade fever is typical in bronchiolitis
Would you expect a patient with bronchitis or with bronchiolitis to be hypoxic and tachypnoeic? Explain why.
Bronchiolitis.
Bronchiolitis affects the respiratory portion of the airway, where gas exchange takes place therefore you may see hypoxia and tachypnoea.
Bronchitis affects the conducting portion of the airway and so is unlikely to have these effects.
Typical pattern of worsening symptoms on day 3-4 days of illness
Bronchiolitis is incredibly common in infants. What are some presenting features of it that would prompt towards hospital admission?
decreased sats, or needing oxygen enviroment or needing respiratory support
Grunting
Decreased feeding, as are in such difficulty feeding - hence that lots of babies w severe bronchiolitis will need NG tube, - and so smaller, more frequent feeds
How do we assess oxygenation and ventilation?
Oxygenation - look at blood O2 sats levels
Ventilation - Look at work of breathing, resp rate, most importantly BLOOD GAS - LOOK AT CO2
if you have poor ventilation, CO2 will be high
What is the management of Bronchiolitis? What can be given to help prevent it in high risk children?
Management:
– This is largely supportive and based on symptomatic management and most cases self-resolve
– Nutritional support, fluids, and oxygen therapy if low oxygen saturations
SpO2 <92% –> admit to hospital
if high flow o2 - needs to be humidified
CPAP - delivers high pressure to keep you airways open
BIPAP - used in neonates as they wont fight against the machine - gives alternating pressures,
Mechanical ventilation
Prevention:
– Palivizumab (monoclonal antibody) (as immunisations) , can be given to infants who are at high risk of severe infection. This includes children with underlying heart/lung disease and premature babies.
What is cystic fibrosis?
Cystic fibrosis (CF) is an inherited, autosomal recessive, multi-system disease affecting mucus glands.
Respiratory problems most prominent, as well as pancreatic insufficiency.
A mutation on what gene causes CF?
What does this mutation go on to cause?
cystic fibrosis transmembrane conductance regulatory gene on chromosome 7
Δ-F508 is the most common mutation, where the codon for phenylalanine (F) in the CFTR gene is deleted, resulting in proteolytic degradation.
How does a mutation to the CFTR gene cause disease?
Misfolded CFTR protein can’t migrate from the endoplasmic reticulum to the cell membrane, meaning there’s a lack of CFTR protein on the epithelial surface
This means that it can’t pump chloride ions out, which means water doesn’t get drawn in, and the secretions are left overly thick.
What are the main pathogens that can cause lung infections in people with cystic fibrosis? What anbtx would you give for these
- Low volume thick airway secretions that reduce airway clearance, resulting in bacterial colonisation and susceptibility to airway infections, especially with:
S. Aureus – flucloxacillin
H. influenzae – amoxicillin
Pseudomonas aeruginosa - ciprofloxacin
What is the pathophysiology of a CFTR dysfunction in relation to the Hepatobiliary system?
How can CF lead to male infertility?
Thicker biliary secretions leads to an increased risk of biliary obstruction.
Could lead to liver cirrhosis
CFTR mutation can cause atrophy of the vas deferens leading to infertility
What are the symptoms of CF?
Chronic cough w/Thick sputum production
Recurrent respiratory tract infections
Loose, greasy stools (steatorrhoea) due to a lack of fat digesting lipase enzymes
Abdominal pain and bloating
Most common cause of Meconium Ileus in infants
Parents may report the child tastes particularly salty when they kiss them, due to the concentrated salt in the sweat
Poor weight and height gain (failure to thrive)
What are some signs of CF?
- Low weight
- Nasal polyps
- Finger clubbing
- Crackles and wheezes
- Abdominal distension
What other investigations can you do for CF? What is gold standard?
Other than heel-prick/Guthrie test (looks for serum Serum Immunoreactive Trypsinogen.)
Sweat test:gold standard test; induce sweating (by placing electrodes on skin) followed by analysis of sweat to check Cl- concentration
A result of> 60 mmol/L (sweat chloride) is positive and requires referral to a cystic fibrosis specialist (normal value < 40 mmol/Ll)
Genetic testing:Genetic testing for CFTR gene mutation can be performed during pregnancy, via amniocentesis
- Lung function tests:obstructive pattern seen; and allows monitoring of treatment
-
Sputum sample:microbiological investigation during exacerbations
Faecal elastase:test for pancreatic insufficiency
What are some first line treatments to help manage CF?
Exercise, no smoking!
- Airway clearance techniques:minimum 2 times per day. Chest physiotherapy and postural drainage.
- Bronchodilator: inhaledsalbutamolfor exacerbations
rhDNasee.g. dornase alfa - an enzyme that breaks down material in secretions, making them less viscous
- Nebulised hypertonic saline
what is the new miracle cure for CF?
personalised genetic treatments - targets specific mutation - F508 del,
Lumacaftor is a CFTR corrector, acts as a Pharmalogical chaperone to bring the F508 del CFTR protein to the cell membrane
Ivacaftor, a CFTR potentiator binds to channel directly, increases the chances of it opening. primarily the targets G551D mutation.
Can be used in combination with one another
What is otitis media? How does it happen?
The bacteria enter from the back of the throat through the eustachian tube. Bacterial infection of the inner ear is often preceded by a viral upper respiratory tract infection.
The anatomy of the eustachian tube in younger children is immature, typically being short, straight, wide (only becoming more oblique as the child grows), meaning infection is more likely.
What are the causes of otitis media?
Bacteria- Streptococcus pneumoniae (most common), haemophilus influenzae, and staphylococcus aureus
Viral- Respiratory syncytial virus, rhinovirus, adenovirus
What is the diagnostic finding for otitis media? What are some signs and symptoms of it
Otoscopy examination will reveal a bulging, red/cloudy tympanic membrane
- Ear pain often associated with holding, tugging or rubbing of the ear in children
- Reduced hearing
- Otorrhea - discharge from ear
- Recent upper respiratory tract infection
- Balance issues and vertigo: if infection affects the vestibular system
-
Non-specific symptoms
- Fever
- Irritability and poor feeding
- Vomiting
- Sore throat
What is the treatment for otitis media? When is it an emergency?
Will usually resolve within 3-7 days
Can give amoxicillin for 5 days.
2nd line would be Co-amoxiclav
Criteria for antibiotics
- symptoms for 4 or more days,
- systemically unwell,
- immunocompromised,
- < 2 years old with bilateral otitis media,
- acute otitis media with perforation/discharge
-Emergency symptoms of mastoiditis - *tenderness behind the ear and the ear sticking out more prominently,
What can recurrent otitis media lead to?
What are the symptoms of this?
Recurrent ear infections can lead to otitis media
with effusion (also called glue ear, Fig. 17.3c). Children are usually asymptomatic apart from possible
decreased hearing. The eardrum is seen to be dull and
retracted, often with a fluid level visible.
What is the function of a grommet?
A grommet keeps the middle ear aerated and prevents the accumulation of fluid in the middle ear.
When might a grommet be indicated?
- Recurrent AOM.
- Chronic otitis media + effusion.
- ET dysfunction.
Give 3 causes of conductive hearing loss.
- Glue ear.
- Ear wax.
- Otitis media.
- Perforated ear drum.
Describe the management of conductive hearing loss.
Conductive hearing loss is usually ENT managed:
- Wait and wait - most will resolve on their own.
- Grommet insertion.
- Temporary hearing aid.
Give 3 risk factors for sensori-neural hearing loss.
Congenital infection, eg rubella, CMV
Meningitis
Genetics
Consanguinity.
Usher Syndrome
hypoxic-ischemic injury to the brainstem, hemorrhage into the inner ear, toxic effects of bilirubin or aminoglycoside,
Describe the management of sensori-neural hearing loss.
Sensori-neural hearing loss is often managed by a paediatrician. Treatments involve hearing aids or cochlea implants.
How would you manage mixed hearing loss?
You would address the conductive problem first and then offer a hearing aid.
When is hearing tested in children?
- New-born hearing screen.
- School entry hearing test.
- Long term monitoring is done in high risk groups.
What is seen in peri orbital cellulitis?
In periorbital cellulitis there is fever with erythema,
tenderness, and oedema of the eyelid or other skin
adjacent to the eye
How should you treat periorbital cellulitis, and what can it lead to?
Periorbital cellulitis should
be treated promptly with intravenous antibiotics such
as high-dose ceftriaxone to prevent posterior spread
of the infection causing orbital cellulitis.
Simply put, what is a squint?
Squint - a misalingment of the eyes, strabismus
Eyes not aligned - images in retina don’t match —> get double vision.
In kids, as eye-brain connection isnt fully developed, brain copes with this by reducing the signal from the less dominant eye
–> As this eye becomes ignored, this becomes a lazy eye
–> If untreated, becomes more and more disconnected from the brain == Called amblyopia
What are the two kinds of squints, and what causes them?
Concomitant (non paralytic) squints are due to differences in the control of the extra ocular muscles. The severity of the squint can vary.
Paralytic squints are rare. They are due to paralysis in one or more of the extra ocular muscles.
Outline the two tests used to detect squints.
Corneal light reflex test
A Light in shone from a distance at both corneas simultaneously. If the light
reflection does not appear in the same position in the
two pupils, a squint is present
Cover test
The child is encouraged to look at a toy/light. If the
fixing (dominant) eye is covered, the *squinting eye will move to take up fixation.** (Fig. 4.9).
Cover test should be performed at different distances
name some causes of paralytic squints
Causes of paralytic squints
- Infections like meningitis or encephalitis can damage the nerves
- Birth trauma to the nerve
- Nero disorders like cerebral palsy - hinder muscle control
Tumours or growth
Name the common left to right shunts in the heart.
Atrail septal defect
Ventricular septal defect
Patent Ductus arteriosus
Outline what happens in patent ductus arteriosus, and what it is assosciated with
DA allows for blood to flow from pulmonary artery to aorata, bypassing lungs
– In term infants, The Ductus arteriosis normally closes shortly after birth due to increased pulmonary flow (this enhances clearance of maternal prostaglandins which maintain duct patency)
– But a failure of the ductus arteriosus to close results in left-to-right shunt between the aorta and the pulmonary artery, as the pressure is higher in the aorta than pulmonary artery
– It is associated with prematurity and maternal rubella infection, downs syndrome
What is the murmur that you would often find in PDA, and where is it best heard?
Continuous machinery like murmur, on left sternal edge
large volume, bounding, collapsing pulse
by a forceful upstroke followed by a rapid drop in systolic pressure, giving it a ‘water-hammer’ feel.
What are some symptoms of PDA?
What signs would you find on examination?
Symptoms:
– Asymptomatic at birth with no cyanosis
– However, if left untreated can lead to Eisenmenger syndrome and heart failure later in life
– Continuous ‘machine-like’ murmur described as “rolling-thunder” with a left sub-clavicular thrill
– Wide pulse pressure
– Heaving apex beat
– Poor growth
large volume, bounding, collapsing pulse
by a forceful upstroke followed by a rapid drop in systolic pressure, giving it a ‘water-hammer’ feel.
What is the management of PDA?
– Indomethacin –> inhibits prostaglandin E synthesis causing closure of the ductus arteriosus
– If very large or persistent, requires surgical repair
indomethacin is given to the neonate in the postnatal period, not to the mother in the antenatal period, around 1 week after delivery
Normal physiology - how does the septum primum Initally develop in the heart of a foetus?
During development, a tissue called the septum primum between the left and right atria grows downward, slowly creating two separate chambers by closing the gap (ostium primum) (know as the first opening)
The septum primum then fuses with the endocardial cushion and closes the ostium primum completely.
A hole appears in the upper area of the septum primum, called the ostium secundum. (known as the second opening)
Normal physiology - how does the septum Secundum Initally develop in the heart of a foetus?
There is also the septum secundum (an adjacent part of the septum) which grows downward, just to the right of the septum primum. It covers the ostium secundum and leaves a small gap called the foramen ovale, thus creating a makeshift valve that allows blood to go from right atrium to left atrium, but not the other way
What are the 3 types of Atrail septal defect?
The types of atrial septal defect from most to least common are:
- Ostium secondum defect. (most common)
- Patent foramen ovale, where theforamen ovalefails to close (although this not strictly classified as anASD).
- Ostium primum,
What is the most common form of atrial septal defect? Outline what happens in it
Ostium secondum, where theseptum secondumfails to fully close, leaving a hole in the wall.
most common congenital heart condition ,in adults, (15% of all congenital heart defects) and 90% of ASDs
Outline what happens in Ostium primum defect. Where is it commonly seen
- Ostium primum, where theseptum primum (first, left sided septum) fails to fully close, leaving a hole in the wall.
Ostium primum = hole in septum primum, (ON THE LEFT - SO AFFECTS LEFT VENTRICLE )
This tends to lead toatrioventricular valve defectsmaking it anatrioventricular septal defect.
Found in 25% of down syndrome cases.
(Less common then ostium secundum defect)
What are the clinical manifestations of a ASDs?
Describe the presentation of them
Can often be asymptomatic in childhood, and first presentation in adulthood/late childhood can sometimes be stroke or heart failure or eisenmenger syndrome
- Signs
- Mid-systolic, crescendo-decrescendo murmur loudest at the upper left sternal border
- Splitting S2 sound: due to pulmonary valve closing after the aortic valve, as extra blood is flowing through it
- Cyanosis: if reversal of shunt
- Symptoms
- Dyspnoea
- Difficulty feeding
- Poor weight gain
- Lower respiratory tract infections
What is the specific murmur that you would most likely hear in ASDs?
Ejection systolic, with Splitting of the S2 sound
What are the management of ASD?
- Watch and wait: for small ASD as it may spontaneously resolve
-
Surgical closure of ASD
- Trans-catheter closure
- Open heart surgery
- Anticoagulants: used to reduce the risk of clots and stroke in adults
What is the most common congenital heart defect? Outline what it is.
Ventricular Septal Defect
When the ventricular septum has a gap in it.
This can vary in size from tiny to the entire septum, forming one large ventricle.
Outline the pathophysiology behind Ventricular septal defect, initally
Oxygenated blood then enters the left atrium and then the left ventricle. Here, blood travels from the high pressure LV to the low pressure RV (left to right shunt). The blood flowing through the VSD causes a holosystolic murmur
At this point, patient’s are acyanotic as there is no deoxygenated blood entering the systemic circulation.
What can happen overtime in a patient with VSD? What condition can they go on to develop?
Overtime, this causes increase of blood in Right sided circulation = causing an increase in pressure.
Now shunt is reversed = deoxygenated blood enters the systemic circulation via the aorta and cause cyanosis - this is known as Eisenmenger syndrome.
Causes Cyanosis at this point
What are some signs and symptoms of VSD?
- Signs
- Pansystolic murmur at the lower left sternal border
- May be a systolic thrill on palpation
- Cyanosis: due to deoxygenated blood in systemic circulation
- Tachypnoea
- Symptoms
- Dyspnoea
- Poor feeding
- Failure to thrive
. There are fine crepitations audible in both lung fields - as VSD can cause pulmonary hypertension
What is the specific murmur that you would most likely hear in VSDs?
A pansystolic murmur, on the lower left sternal boarder, louder in smaller defects
A pansystolic murmur is one which last from the beginning S1 to the end of S2, and therefore obscures these heart sounds
What are some medical interventions fo VSD
Duiretics, like spironlactone
Cardiac catherisation
What are the main causes of heart failure in neonates?
- Hypoplastic left heart syndrome -left side of the heart severely underdeveloped
- Critical aortic valve stenosis
- Severe coarctation of the aorta
- Interruption of the aortic arch
- transposition of the great arteries -
Think - all lead to obstructed systemic circulation, so heart is fucked from the off - causes heart failure straight away
What are some characteristics of innocent murmurs head in children?
Characteristics of an innocent ejection murmur include:
soft-blowing murmur in the pulmonary area or short buzzing murmur in the aortic area
may vary with posture
localised with no radiation
no diastolic component
no thrill
no added sounds (e.g. clicks)
asymptomatic child
no other abnormality
What is the main causes of heart failure in infants? (1st year of life)
- Large PDA, VSD or ASD
- Anything that causes mixes of circulation
What is the main causes of heart failure in older children/adolescents?
More due to acquired conditions:
Rheumatic fever, cardiomyopathies, Eisenmenger syndrome
What are some symptoms of heart failure in children?
Shortness of breath
– Inability to gain weight/poor growth
– Predisposition to recurrent chest infections
– Hepatomegaly
– Signs of specific cause –> e.g., murmur, cardiomegaly, cyanosis
What is some general management for heart failure in children?
The underlying cause of heart failure must be treated.
- Bed rest and nurse in semi-upright position: infants in chair/seat.
- Supplemental oxygen (not in left to right shunt).
- Diet: sufficient calorie intake.
- Diuretics.
- Angiotensin converting enzyme inhibitors
name 3 cyanatoic congenital heart diseases. (Right to left shunts)
Tetralogy of Fallot
transposition of the great arteries
Eisenmenger Syndrome
What are the four things that characterise tetralogy of Fallot?
Pulmonary stenosis
Right Ventricular hypertrophy
Ventricular septal defect
Overriding aorta
It is the most common form of congenital cyanotic heart disease
the severity of the right ventricular outflow tract obstruction (pulmonary stenosis) determines the degree of cyanosis and clinical severity#
PROVE
Pulmonary stenosis
Right Ventricular hypertrophy
Overriding aorta
Ventricular septal defect
E = Ejection systolic murmur
Pathophysiology of tetralogy of Fallot - what does
a) Pulmonary stenosis
b) Ventricular septal defect
c) Overriding aorta
all lead to/cause?
What of these features would most determine the degree of cyanosis and clinical severity?
TETROLOGY OF FALLOT -
Pulmonary stenosis - leads to RV hypertrophy as it has to push harder to get blood through
VSD - blood shunts from right to left due to RV hypertrophy - Deoxygenated blood enters circulation , get cyanosis
Overriding Aorta - Entrance to aorta is further right then normal, above VSD - so more deoxygenated blood enters aorta when the RV contracts upwards
Tetralogy of Fallot: the severity of the right ventricular outflow tract obstruction (pulmonary stenosis) determines the degree of cyanosis and clinical severity
Would a baby born with tetralogy of fallot be cyanotic? Why?
YES! There is a greater pressure in the RV than the LV and so blood is shunted into the LV -> CYANOSIS!
the severity of the right ventricular outflow tract obstruction (pulmonary stenosis) determines the degree of cyanosis and clinical severity
What are some risk factors for tetralogy of fallot?
- Neonates and babies: typically manifests at around 1-2 months of life
- Family history of congenital heart disease
- Rubellainfection
- Increased age of the mother (over 40 years)
- Alcohol consumption in pregnancy
- Diabetic mother
- Down syndrome: trisomy 21
What murmur would you hear in tetrology of fallot, and where would it be best heard? What is the signature x-ray finding in ToF?
Ejection systolic murmur in the pulmonary region (think due to pulmonary stenosis)
Boot shaped heart, due to right ventricular hypertrophy
What are soem clinical manifestations of tetralogy of fallot?
- Signs
- Ejection systolic murmur: due to pulmonary stenosis
- Reduced SpO2, particularly when distressed
- Respiratory distress
- Cyanosis
- Clubbing
- Symptoms
- Poor weight gain or ‘failure to thrive’
- Difficulty feeding
- Hypercyanotic or ‘tet’ spells: cyanosis, breathlessness and syncope, particularly when crying or feeding
- Squatting posture
What happens in transpostions of the great arteries?
Transposition of the great arteries is a condition where the attachments of the aorta and the pulmonary trunk to the heart are swapped (“transposed”). This means the right ventricle pumps blood into the aorta and the left ventricle pumps blood into the pulmonary vessels. In this scenario are two separate circulations that don’t mix: one travelling through the systemic system and right side of the heart and the other traveling through the pulmonary system and left side of the heart.
What murmur would you hear in Transpostion of the great arteries? What is the signature x-ray finding in it?
trick question - no murmur dickhead! – butGives a loud single S2==> pulmonary valve is flowing high pressure blood from the left ventricle
Loud S2!
Egg on string appearance
What are some symptoms and signs of transpositions of the great arteries?
Symptoms:
– Cyanosis shortly after birth e.g., on day 2 when ductus arteriosus closes
– The more mixing of blood there is, the less severe the presentation
– Gives a loud single S2 but no murmur ==> pulmonary valve is flowing high pressure blood from the left ventricle
– If left untreated, leads to heart failure and mortality
What investigations would you do, and what would you see for transpostions of the great arteries?
Diagnosis:
– Echocardiogram is the investigation of choice
– Pulse oximetry –> shows reverse differential cyanosis (SpO2 in legs > SpO2 in arms), as the legs can receive some oxygenated blood through the PDA. In
contrast, the branches to the arms occur before the PDA so they only get deoxygenated blood.
– CXR –> shows narrow upper mediastinum with an ‘egg on side’ appearance of the cardiac shadow
What is the management in transpostion of the great arteries?
– Prostaglandin infusion (5mg/kg/min) and oxygen to manage acute cyanosis
e administration of prostaglandins (such as alprostadil) to promote duct patency.
– Surgical correction –> arterial switch procedure is the definitive treatment
What are some risk factors for coarction of the aorta?
Turners syndrome, bicuspid aortic valve, male sex
What are some things seen in the presentation of coarction of the aorta? What murmur? Acyanotic or Cyanotic?
What genetic condtion is it often seen in?
Radiofemoral delay
Differential in blood pressure in upper and lower limbs (high in arms, low in Legs)
Mid Ejection systolic murmur (similar to aortic stenosis)
It’s acyanotic
Can often be seen with Turners syndrome
What is ebsteins anomoly? What is it associated with?
Low insertion of the tricuspid valve, meaning that the right atrium is too big and the right ventricle is too small - leads to poor flow to the pulmonary vessels
Associated w Lithium use in pregnancy, ASD, PFO. WPW syndrome
What are some signs of ebstein’s anomaly (murmur)? Is it cyanotic or not?
very prominent murmur during systole and diastole, loudest over the left sternal edge.
A chest X-Ray shows massive cardiomegaly, particularly prominent in the right atrium.
Evidence of heart failure (e.g. oedema)
Gallop rhythm heard on auscultation characterised by the addition of the third and fourth heart sounds:
Severe tricuspid regurgitation and an enlarged right atrium raise right atrial pressure.
When right atrial pressure exceeds left atrial pressure, deoxygenated blood flows from the right atrium to the left atrium through the ASD/PFO.
Cyanosis
Shortness of breath and tachypnoea
Poor feeding
Collapse or cardiac arrest
What can rheumatic fever do to the skin and nervous system?
Skin Subcutaneous nodules =
Firm painless nodules occur over extensor surfaces of joints, such as the elbows.
Erythema marginatum ==
rash pink rings of varying sizes affecting the torso and proximal limbs
Chorea is the key nervous system symptom. This involves irregular, uncontrolled and rapid movements of the limbs. This is also known as Sydenham chorea and historically as St Vitus’ Dance.
Outline what is the major and minor criteria for rheumatic fever, in the Jones Criteria.
Two major, or one major and two minor, criteria plus supportive evidence of preceding group A streptococcal infection
(markedly raised or rising ASO titre or positive rapid streptococcal antigen test or positive group A streptococcus
on throat culture)
Major Criteria:
J – Joint arthritis
O – Organ inflammation, such as carditis
N – Nodules
E – Erythema marginatum rash
S – Sydenham chorea
Minor Criteria:
F Fever
E ECG Changes (prolonged PR interval) without carditis
A Arthralgia without arthritis
R Raised inflammatory markers (CRP and ESR)
JONES FEAR
What is Pyloric Stenosis?
Hypertrophy (thickening) and therefore narrowing of the pylorus, leading to gastric outlet obstruction.
You suspect pyloric stenosis in a neonate. What investigations might you do?
- U+E.
- Blood gas.
- Ultrasound scan - hypertrophy of pyloric sphincter. - the key invesitgatiom
How would you treat a neonate with pyloric stenosis?
- IV fluids.
- Repeat gases to monitor alkalosis.
- Stop feeding to stop vomiting.
- Ramstedt pyloromyotomy is used to manage pyloric stenosis.
- (This involves division of the hypertrophied muscle down to,
but not including, the mucosa)
What are some signs of severe dehydration vs shock seen in gastroenteriris?
Signs of dehydration:
– Reduced urine output, lethargic, sunken eyes
– However, they are haemodynamically stable (normal BP, normal capillary refill time, warm extremities)
Signs of shock:
– Reduced consciousness, pale skin, appears unwell
– They will start to get haemodynamically compromised –> low BP, high HR, long CRT, cold extremities
Outline the pathophysiology behind appendicitis
Normally occurs due to luminal obstruction
As the appendix continues to secrete mucus the fluid and mucus build up = increasing pressure = makes appendix get bigger
= and push on the visceral nerve fibres causing pain
Bacteria now trapped and able to multiply resulting in invasion of gut organisms into the appendix wall
This leads to oedema, ischaemia, necrosis, perforation and inflammation
What signs and symptoms would you see in acute appendicitis?
Describe the pain seen
- Periumbilical pain (referred pain) which migrates to the right iliac fossa (McBurney’s point)
- Low-grade fever: > 38°C suggests alternative pathology e.g. mesenteric adenitis
- Reduced appetite and anorexia
- Nausea and vomiting: persistent vomiting is unusual
- Diarrhoea: rare but may occur in pelvic appendicitis or due to a pelvic abscess
What signs would you see in someone with acute appendicits? What examinations would you do?
- Right iliac fossa tenderness: rebound tenderness (pain when pressure is taken off) or percussion tenderness (pain during percussion) suggests localised peritonism
- Guarding
- Rovsing’s sign: pain in the right iliac fossa is worsened by pressing on left iliac fossa
- Psoas sign: pain is worsened by extending the hip
- Obturator sign: pain is worsened by flexing and internally rotating the hip
Tachycardia, hypotension and generalised peritonism: suggests perforation
What is the definitive management for appendicitis?
Prompt appendicectomy:laparoscopic
Postoperative antibiotics
Outline what an umbilical hernia is. What is it assossicated with?
This is a herniation of the bowel content through the umbilicus. It is a common finding in neonates and usually asymptomatic
Outline the two types of inguinal hernia
Direct hernia: hernia protrudes through the posterior wall of the inguinal canal.
- Less common (20%)
Indirect hernia: hernia protrudes through the deep inguinal ring and into the inguinal canal
- More common (80%)
What are the clinical features of an inguinal hernia ?
groin swelling and likely, the presence of risk factors.
nausea, vomiting, constipation, abdominal pain/discomfort could indicate obstruction or strangulation
an inguinal/inguino-scrotal mass that you cannot ‘get above’,
is reducible when lying flat (goes back into abdomnal wall
does not transilluminate,
has a positive cough reflex.- If the swelling expands upon coughing
How do you manage inguinal hernias in children?
Most hernias can be successfully reduced by ‘taxis’
(gentle compression in the line of the inguinal canal)
with good analgesia. Surgery can then be planned
for a suitable time when any oedema has settled and the child is well. If reduction is impossible, emergency surgery is required because of the risk of compromise of the bowel or testis. In girls, sometimes the ovary can become incarcerated within a hernia.
Outline the pathophysiology behind Crohn’s
A foreign pathogen triggers the immune system, which are able to get through the gut wall due a defect in the epithelium
This triggers a large and uncontrolled immune response - These immune cells invade deep into the mucosa and organise themselves into granulomas
This leads to ulcers forming throughout all layers, hence Crohn’s being said to be transmural, and cobblestone in appearence
the ileum is the most commonly affected site of crohns
What are the signs of Crohn’s?
Abdominal tenderness
Fever
Mouth ulcers
Rectal examination will show blood, skin tags, fissures and fistulas
eye problems - uveitis
Lethargy and general ill health without
gastrointestinal symptoms can be the presenting
features, particularly in older children. There may be
considerable delay in diagnosis as it may be mistaken
for psychological problems
What are the symptoms of Crohn’s?
Weight loss
Diarrhoea
Abdominal pain (most common in RLQ where the ileum is as this is the site most affected by Crohns)
Lethargy and general ill health without
gastrointestinal symptoms can be the presenting
features, particularly in older children. There may be
considerable delay in diagnosis as it may be mistaken
for psychological problems
What are some cutaneous, muscoskeletal and eye features you may see in a patient with Ulcerative collitis?
Erythema Nodosum - inflamed subcutaneous fat (see picture)
Pyoderma gangrenosum - rapidly enlarging, very painful ulcer.
- Osteoporosis and arthritis
- Clubbing
- Uveitis - eye inflammation
- Conjunctivitis
taken from netters illustrated human pathology
What is the gold standard investigation for UC? What would you see on it?
Colonoscopy -
- Red and raw mucosa with widespreadshallow ulceration
- No inflammation beyond the submucosa, unless fulminant disease
- Pseudopolyps: mucosa adjacent to ulcers is preserved, which has the appearance of polyps - They are caused by mucosal repair after chronic inflammation.
- Crypt abscessesdue to neutrophil migration through gland walls
- Goblet cell depletion, withinfrequentgranulomas
picture taken from netters illustrated human pathology
What are the surgical options for UC?
PANPROCTOCOLECTOMY = Removal of the colon and rectum
Left with ileostomy or or J-pouch this is where small intestine is used to make rectum
severe UC can lead to toxic megacolon
Surgery will be curative
State some histological features that will be seen in ulcerative colitis
Pseudopolyps
Crypt abscess
Goblet cell depletion,
Infrequent granulomas
Increased plasma cells in lamina propria
What are some classical clinical presentations of Coeliac disease?
What are some more subtle signs?
Suspect in all with diarrhoea, weight loss & anaemia
Steatorrhoea (fat in stools) & stinking stools - due to Inability to properly absorb fat from diet
Anaemia - Due to inability to absorb B12, folate, iron
Weight loss
Fatigue & weakness
Diarrhoea
Bloating
Nausea + vomiting
Other, more subtle, modes of presentation – e.g.
short stature, anaemia, abdominal pain and
screening, e.g. children with diabetes mellitus,
are now much more common.
What is the gold standard test for diagnosing coeliac disease? What would you see?
what about on Histology?
Endoscopically
Required for definite diagnosis
+ve Findings:
Villous atrophy
Crypt hyperplasia
Increased epithelial WBCs
Target cells and Howell Jolly Bodies
Define faltering growth. How do centiles in a growth chart work?
The failure to gain adequate weight or achieve adequate growth during infancy and childhood.
The modern British growth charts consist of nine centile
lines (0.4th, 2nd, 9th, 25th, 50th, 75th, 91st, 98th and 99.6th). The distance between each centile line (2/3 of a standard deviation) is known as a centile space
NICE defines faltering growth as weight that has fallen down 2 centile lines.
What are the thresholds for concern about faltering growth in infants and children?
a fall across 1 or more weight centile spaces, if birth weight was below the 9th centile.
a fall across 2 or more weight centile spaces, if birth weight was between the 9th and 91st centiles.
a fall across 3 or more weight centile spaces, if birth weight was above the 91st centile.
when current weight is below the 2nd centile for age, whatever the birth weight.
Causes of faltering growth: give examples of inadequate calorie intake.
- Impaired suck/swallow. - eg Cleft palate, cerebral palsy
- Inadequate availability of food.
- Psychosocial deprivation.
- Exclusion diets e.g. veganism
Infant difficult to feed – resists feeding or disinterested
* Conflict over feeding, intolerance of normal feeding behaviour, e.g.
messiness, throwing food around, leading to an early cessation of meals
Causes of faltering growth: give examples of malabsorption.
- Coeliac disease.
- Pancreatic disease e.g. CF.
- Liver disease.
- Enteropathy e.g. infective causes - giardia.
- Cows milk protein intolerance.
Causes of faltering growth: give an example of inadequate retention.
Vomiting e.g. severe GORD, pyloric stenosis.
Causes of faltering growth: give example increased calorie requirements.
- Chronic illness e.g. CHD, CKD, CF.
- Thyrotoxicosis.
- Malignancy.
Define Marasmus.
Marasmus is a deficiency of all macronutrients, and in it, the child has a wasted, wizened
appearance (Fig. 13.15). Oedema is not present.
Affected children are often withdrawn and apathetic
Define kwashiorkor - when is it commonly seen?
kwashiorkor is a type of malnutrition caused by protein deficiency. It mainly occurs in children who are weaning off breast milk, while marasmus can develop in infants.]
If your diet has a lot of carbohydrates and very little proteins, you may develop kwashiorkor
What is Hirschsprung’s disease?
Congential condition where there is an Absence of parasympathetic ganglion cells in the myenteric plexus
It’s complex web of neurones, ganglion cells, receptors, synapses and neurotransmitters. It is responsible for stimulating peristalsis of the large bowel.
Outline the pathophysiology of Hirschsprung’s disease
Hirschsprung’s occurs when the parasympathetic ganglion cells do not travel all the way down the colon, and a section of colon at the end is left without these parasympathetic ganglion cells.
The aganglionic section of colon does not relax, causing it to becomes constricted. This leads to loss of movement of faeces and obstruction in the bowel
Outline the presentation of hirschprungs.
diagnosis varies significantly depending on the individual and the extent of the bowel that is affected.
Delay in passing meconium (more than 24 hours)
Chronic constipation since birth
Abdominal pain and distention
Vomiting (Billious)
Poor weight gain and failure to thrive
What is a key complication that can occur in Hirschprungs disease? How do you manage it?
Hirschsprung-associated enterocolitis (HAEC)
it’s inflammation and obstruction of the intestine occurring in around 20% of neonates with Hirschsprung’s disease.
It typically presents within 2-4 weeks of birth with fever, abdominal distention, diarrhoea (often with blood) and features of sepsis. It is life threatening and can lead to toxic megacolon and perforation of the bowel.
It requires urgent antibiotics, fluid resuscitation and decompression of the obstructed bowel.
What is the treatment for Hirschprungs?
If Hirschsprung;s disease is suspected, a full thickness rectal biopsy should be performed as investigation. However, while this is being organised etc, bowel washouts should be performed to allow the baby to pass meconium, so this is the initial treatment
Management is surgical and
usually involves an initial colostomy of aganglionic section of the bowel, followed by anastomozing normally innervated bowel to the anus.
What is Intussusception?
Intussusception describes the invagination of proximal bowel into a distal segment.
It most commonly involves ileum passing into the caecum through the ileocaecal valve
It’s the most common cause of intestinal obstruction in infants after
the neonatal period. Although it may occur at any age,
the peak age of presentation is 3 months – 2 years
of age.
What are the key signs and symptoms for intussception?
– Abdominal pain during which infant characteristically will pull their legs up to the chest area, and abdo distension
– Vomiting (which can be green in colour due to bile)
– Blood-stained stool (red-currant jelly stool)
– Sausage-shaped mass in RUQ
– Can cause venous obstruction giving bowel necrosis and perforation
What are some investigations for Intussception?
Investigation
ultrasound is now the investigation of choice and may show a target-like mass
An X-ray of the abdomen may show distended small bowel and absence of gas in the distal colon or rectum
The abdominal film demonstrates a large soft tissue opacity (‘sausage-shaped’) in the left upper quadrant. .
What is Meckel diverticulum a remnant of?
Meckel’s diverticulum is an outpouching or bulge in the lower part of the small intestine.
Which contains ectopic gastric mucosa or pancreatic tissue
What can Meckels Diverticulum lead to?
Tthe pouch releases acid like the stomach which over time can cause ulcers to develop in the small intestine. These can worsen and bleed which shows as blood in the poo.
It can also leak the liquid inside the small intestine into the abdomen, leading to a serious infection called peritonitis.
The pouch can also twist leading to a condition called volvulus.
Twisting can cause the blood supply to the intestine to be cut off, which can be a life threatening condition.
It can also fold inwards leading to a condition called intussusception.
Describe what ‘Colic’ is
The term ‘colic’ is used to describe a common symptom complex that occurs during the first few months of life.
Paroxysmal, inconsolable crying or screaming often
accompanied by drawing up of the knees and passage
of excessive flatus takes place several times a day.
Regarding Bilirubin levels, what would be seen in Biliary artresia?
A high levels of conjugated bilirubin suggests the liver is processing the bilirubin for excretion (by conjugating it), but it is not able to excrete the conjugated bilirubin because it cannot flow through the biliary duct into the bowel.
Biliary atresia presents shortly after birth with significant jaundice due to high conjugated bilirubin levels.
What is a choledochal cyst? How may they present?
They are cystic dilatations of the extrahepatic biliary
system
They present with neonatal jaundice or, in
older children, presentation is with abdominal pain, a
palpable mass, jaundice, or cholangitis
What things would you typically see in Neonatal hepatitis syndrome?
Baby has intrauterine growth restriction, Jaundice, failure to thrive, itchy rash, dark urine and hepatomegaly.
What would a liver biopsy show in neonatal hepatitis syndrome? What blood tests would you do?
Liver biopsy will show: Multinucleated giant cells & Rosette formation.
This will both ↑unconjugated + ↑ conjugated bilrubin.
With associated deranged LFT’s
What is Vesicoureteric reflux?
developmental anomaly of the vesicoureteric
junctions. The ureters are displaced laterally and enter directly into the bladder rather than at an angle, where urine has a tendency to flow from the bladder back into the ureters.
Can lead to frequent UTIs and renal scarring - which in turn can lead to CKD and hypertension
Atypical and recurrent UTIs - what are some special investiagtions used?
DMSA (Dimercaptosuccinic Acid) Scan - Inject radioactive material (DMSA) and use a gamma camera to assess how well the material is taken up by the kidneys.
looks for renal scarring in a child with vesicoureteric reflux
Micturating Cystourethrogram (MCUG) - catheterising the child, injecting contrast into bladder and see whether the contrast is refluxing into the ureters. **This is necesscary to diagnose VER
Name some congenital abnormaliles of the kidney that you may see on investigation
Horseshoe kidney
Duplex kidney (two ureters from one kidney)
Solitary kidney
What is the treatment for UTI in children?
Infants younger tahn 3 months - 2-4 IV cefuroxime (1st gen Ceph, cant get through BBB so need to rule out meningitis). followed by oral - full septic screen, bloods, cultures, lactate
Systemically well children - Treat with 3 days oral antibiotics. Advised to return if no better at 24-48hrs for reassessment - Trimethoprim
Systemically unwell - (Fever > 38 +/- loin pain / tenderness) Treat with 7-10 days oral antibiotics
(Consider IV antibiotics according to clinical judgement. Use lower threshold for IV antibiotics in younger children, those with significant risk factors and severely ill.)
What antibiotic should you give for a UTI caused by ESBL E Coli?
What does ESBL stand for?
E coli
Extended spectrum ß-lactamase producer (ESBL)
Resistant to all penicillins and cephalosporins
Change to meropenem for serious infections
Fosfomycin
For uncomplicated UTIs: Fosfomycin is often a good choice for oral therapy in cases of cystitis caused by ESBL-producing E. coli. It is typically given as a single dose.
3. Nitrofurantoin
For uncomplicated cystitis only: Effective for lower urinary tract infections, but it should not be used for pyelonephritis or systemic infections.
Causes of AKI - give examples of Pre Renal causes of an AKI
MOST COMMON IN CHILDREN
Reduced blood flow to kidney leading to a falling GFR
Shock, + Hypovolaemia (fluid loss) - this could be due to burns, sepsis, acute haemorrhage trauma or bleeding, anaphylaxis -
Decompensated Liver disease- Hepatorenal syndrome
GI losses – diarrhoea and vomiting, Gastroenteritis
Causes of AKI - give examples of intrarenal causes of an AKI
Vascular:
– haemolytic uraemic syndrome - most common type of renal
– vasculitis
– embolus
- Tubular:
– acute tubular necrosis
– ischaemic
– toxic - drugs - Glomerular:
– glomerulonephritis - Interstitial:
– pyelonephritis
What drugs can be nephrotoxic and are known to cause intra renal disease?
NSAIDs – inhibits COX which causes excess vasoconstriction of afferent arteriole
Aminoglycosides - eg gentamicin – 10-15% incidence of ATN
ACE-I/ARBs – results in dilated efferent arterioles decreasing GFR
Methotrexate
Gentamicin
DAMN = (diuretics, ACEi/ ARBs, metformin, NSAIDs)
Loop diuretics are considered to be more harmful for kidneys than Thiazides and K+ sparing ones
Causes of AKI - give examples of postrenal causes of an AKI in children
Obstruction:
– congenital, e.g. posterior
urethral valves in boys, urethra is blocked
– acquired, e.g. blocked urinary
catheter
Hepatorenal syndrome - how can liver cirrhosis lead to renal failure?
Liver cirrhosis leads to portal hypertension
In response to this, portal blood vessels dilate, stretched by large amounts of blood pooling there.
This leads to a loss of blood volume elsewhere, including kidneys.
Leads to hypotension in the kidney and activation of the RAAS. This causes renal vasoconstriction, which combined with low circulation volume leads to starvation of blood to the kidney
Hepatorenal syndrome is fatal within a week or so unless liver transplant is performed
Hyperkalaemia is a complication of AKI, and can lead to a cardiac arrest. How can you manage hyperkalaemia?
3 THINGS -
- IV stat of Calcium Gluconate - Stabilisation of the myocardium
- Variable rate insulin with dextrose infusion - to increase cellular uptake of potassium = Reduction of serum potassium
- oral calcium resonium - Reduction of total body potassium - treat underlying cause
What is the first line management for an AKI?
Treat underlying cause
- Regular monitoring: fluids, urine output, daily weights, baseline creatinine, serial U&Es
- Cease nephrotoxic drugs
- Hypovolaemia: IV fluids
- Hypervolaemic: fluid restriction and diuretics e.g. furosemide
- Obstruction: catheter insertion; surgery to relieve obstruction
TREAT COMPLICATIONS - Hyperkalaemia, pulmonary oedema, uraemia, acidaemia - Bicarbonate
What would you also consider for management in severe AKI?
Dialysis – removal of toxic products in body for patients with failed kidneys.
refer to critical care, get specialist involved!!
Use of a vasopressor, ie Noradrenaline
Consider the need for an inotrope (e.g., dobutamine) to optimise cardiac output if kidney hypoperfusion is caused by impaired cardiac function due to poor left ventricular systolic function
CKD classification - outline the stages GFR 1-5 entail?
1 >90 Normal or increased GFR with other evidence of renal damage*
2 60–89 Slight decrease GFR with other evidence of renal damage*
3 A - 45–59, 3 B - 30–44 = Moderate decreased GFR with or without evidence of other renal damage
4 15–29 Severe decreased GFR with or without evidence of renal damage
5 <15 Established renal failure
What are some of the management options for CKD in kids?
Improve nutritional intake with calorie supplements
Salt and bicarbonate supplements
phosphate restriction and
activated vitamin D to prevent renal
osteodystrophy
recombinant human EPO, and Growth hormone
Diaylsis and transplantation
How can Glomerulonephritis be classified?
Nephrotic syndrome – protein leaks due to inflammation of podocytes
Acute GN (nephritic syndrome) – blood vessels inflamed 🡪 blood leaks out
Rapidly progressive GN – features of acute nephritis, focal necrosis and rapidly progressing renal failure
How can Nephritic syndrome be differentiated from Nephrotic syndrome?
Nephritic syndrome can be differentiated from nephrotic syndrome because the proteinuria is generally under 3.5 grams per day, or within the “sub-nephrotic range”.
What is Post-streptococcal glomerulonephritis?
Post-streptococcal glomerulonephritis (PSGN) is usually an immunologically-mediated delayed consequence of pharyngitis or skin infections caused by streptococcus pyogenes
- What are the initial investigations for PSGN?
-
Bloods: low levels of C3 and CH50
as C3 has been deposited in kidney
FBC - mild normchronmic and normocytic anaemia
raised antiDNAse B
Incresased Urea, hyperkalaemia
- Positive streptozyme test confirms recent group A streptococcal infection
Macroscopic haematuria
How does SLE cause glomerulonephritis? Where are is the most common site of deposition?
It is a type III hypersensitivity reaction where immune complexes are deposited in various places including kidney
Most common site of deposition is in the subendothelial space of the glomerular basement membrane
Can also be in the Bowerman’s space, Basement membrane, and in Mesangial cells.
Briefly outline the pathophysiology of Lupus
Cells have faulty DNA, and when these faulty cells undergo apoptosis, these are released and become nuclear antigens
immune cells recognise them as forgein and create antinuclear antibodies that bind to them.
These can travel in the blood and get stuck in lungs, joint, skin, CNS, Heart Kidneys etc
What is Henoch-Schonlein purpura? What is it similar to?
Similar to IgA Nephropathy.
IgA vasculitis, also known as Henoch-Schonlein purpura: the difference is IgA nephropathy only affects the kidneys, while IgA vasculitis can cause nephritic or nephrotic syndrome, and also presents with colicky abdominal pain, bloody stool, arthritis, and palpable skin lesions, hyperteniosn and haematuria
What would you see on imaging for Henoch-Schonlein purpura?
On electron microscopy: immune complexes are seen in the mesangium.
- On immunofluorescence: IgA immune complexes in the mesangium
Outline the course of congenital nephrotic syndrome. When does it present and where is it most common?
presents in first 3 months of life, very rare
Recessively inherited, more common in consanguineois families
leads to severe hypoalbuminaemia
unilateral nephrectomy may be necessary for its control, followed by dialysis for stage 5 (most severe) chronic kidney disease, which is continued until the child is no longer nephrotic and old enough for renal transplantation.
outline the pathophysiology behind minimal change disease. What is affected?
caused byT-cells in the blood, that release cytokines-glomerular-permeability factor, that specifically damages the foot processes of the podocytes, making them flatten out - a process called effacement.
Damaged foot processes lose their negatively charged coat, eventually allowing negatively charged molecules,
MOST COMMONLY SEEN IN CHILDREN
What do you see in the urine of someone with minimal change disease?
- allowing negatively charged molecules, like albumin, to slip into the nephron.
- Even though albumin goes through, other larger proteins like immunoglobulins don’t.
- So there’s selective proteinuria; in contrast to other causes of nephrotic syndrome which are characterised by non-selective proteinuria.
What is Hypospadias? What condition can it be associated with?
Hypospadias is a condition affecting males, where the urethral meatus (the opening of the urethra) is abnormally displaced to the ventral side (underside) of the penis, towards the scrotum. This might be further towards the bottom of the glans (in 90% of cases), halfway down the shaft or even at the base of the shaft.
There can also be an associated condition called chordee, where the head of the penis bends downwards.
Cryptorchidism (undescended testes) is present in around 10% of patients with hypospadias
What is the bacteria that mainly causes HUS? What does it release?
E. coli O157 or Shigella release Shiga toxins, that can pass out of the gut and enter the bloodstream.
most common is E. coli O157
What does HUS do the red blood cells?
And what would you see on a blood film in HUS?
Microangiopathic haemolytic anaemia (MAHA) involves the destruction of red blood cells (haemolysis) due to pathology in the small vessels (microangiopathy). Tiny blood clots (thrombi) partially obstruct the small blood vessels and churn the red blood cells as they pass through, causing them to rupture.
would see Schistocytes (1 mark) or helmet cells (1 mark)
Outline the presentation of haemoyltic uraemic syndrome
E. coli O157 and Shigella cause gastroenteritis. Diarrhoea is the first symptom, which turns bloody within 3 days. Around a week after the onset of diarrhoea, the features of HUS develop:
Fever
Abdominal pain
Lethargy
Pallor
Reduced urine output (oliguria)
Haematuria
Hypertension
Bruising
Jaundice (due to haemolysis)
Confusion
