Conditions Flashcards
What is laryngomalacia and what are the structural changes seen?
Laryngomalacia is a condition affecting infants, where the part of the larynx above the vocal cords (the supraglottic larynx) is structured in a way that allows it to cause partial airway obstruction. This leads to a chronic stridor on inhalation, when the larynx flops across the airway as the infant breathes in. Stridor is a harsh whistling sound caused by air being forced through an obstruction of the upper airway.
Structural Changes
There are two aryepiglottic folds at the entrance of the larynx. They run between the epiglottis and the arytenoid cartilages. They are either side of the airway and their role is to constrict the opening of the airway to prevent food or fluids entering the larynx and trachea. In laryngomalacia the aryepiglottic folds are shortened, which pulls on the epiglottis and changes it shape to a characteristic “omega” shape.
The tissue surrounding the supraglottic larynx is softer and has less tone in laryngomalacia, meaning it can flop across the airway. This happens particularly during inspiration, as the air moving through the larynx to the lungs pulls the floppy tissue across the airway to partially occlude it. This partial obstruction of the airway generates the whistling sound.
What are the presenting features of laryngomalacia?
Laryngomalacia occurs in infants, peaking at 6 months. It presents with inspiratory stridor, a harsh whistling sound when breathing in. Usually this is intermittent and become more prominent when feeding, upset, lying on their back or during upper respiratory tract infections. Infants with laryngomalacia do not usually have associated respiratory distress.
It can cause difficulties with feeding, but rarely causes complete airway obstruction or other complications.
How is laryngomalacia managed?
The problem resolves as the larynx matures and grows and is better able to support itself, preventing it from flopping over the airway. Usually, no interventions are required and the child is left to grow out of the condition.
Rarely tracheostomy may be necessary. This involves inserting a tube through the front of the neck into the trachea, bypassing the larynx. Surgery is also an option to alter the tissue in the larynx and improve the symptoms.
How is whooping cough diagnosed and managed?
Bordetella pertussis is a gram negative bacteria. A nasopharyngeal or nasal swab with PCR testing or bacterial culture can confirm the diagnosis within 2 to 3 weeks of the onset of symptoms.
Where the cough has been present for more than 2 weeks patients can be tested for the anti-pertussis toxin immunoglobulin G. This is tested for in the oral fluid of children aged 5 to 16 and in the blood of those aged over 17.
Management
Pertussis is a notifiable disease. Therefore Public Health need to be notified of each case.
Management typically involves simple supportive care. Vulnerable or acutely unwell patients, those under 6 months and patients with apnoeas, cyanosis or patients with severe coughing fits may need to be admitted. Measures to prevent spread are important, such as avoiding contact with vulnerable people, disposing of tissues and careful hand hygiene.
Macrolide antibiotics such as azithromycin, erythromycin and clarithromycin can be beneficial in the early stages (within the first 21 days) or vulnerable patients. Co-trimoxazole is an alternative to macrolides.
Close contacts with an infected patient are given prophylactic antibiotics if they are in a vulnerable group, for example pregnant women, unvaccinated infants or healthcare workers that have contact with children or pregnant women.
The symptoms typically resolve within 8 weeks, however they can last several months. It is also known as the “100-day cough” due to the potential long duration of the cough. A key complication of whooping cough is bronchiectasis.
What is bronchopulmonary dysplasia and what are the features?
Chronic lung disease of prematurity (CLDP) is also known as bronchopulmonary dysplasia. It occurs in premature babies, typically those born before 28 weeks gestation. In BPD the lungs and the airways (bronchi) are damaged, causing tissue destruction (dysplasia) in the tiny air sacs of the lung (alveoli)
These babies suffer with respiratory distress syndrome and require oxygen therapy or intubation and ventilation at birth. Diagnosis is made based on chest xray changes and when the infant requires oxygen therapy after they reach 36 weeks gestational age.
Features
Low oxygen saturations
Increased work of breathing
Poor feeding and weight gain
Crackles and wheezes on chest auscultation
Increased susceptibility to infection
How can bronchopulmonary dysplasia be prevented?
Prevention
There are several measure that can be taken to minimise the risk of CLDP.
- Giving corticosteroids (e.g. betamethasone) to mothers that show signs of premature labour at less than 36 weeks gestation can help speed up the development of the fetal lungs before birth and reduce the risk of CLDP.
Once the neonate is born the risk of CLDP can be reduced by:
- Using CPAP rather than intubation and ventilation when possible
- Using caffeine to stimulate the respiratory effort
- Not over-oxygenating with supplementary oxygen
How is bronchopulmonary dysplasia managed?
A formal sleep study to assess their oxygen saturations during sleep supports the diagnosis and guides management. Babies may be discharged from the neonatal unit on a low dose of oxygen to continue at home, for example 0.01 litres per minute via nasal cannula. They are followed up to wean the oxygen level over the first year of life.
Babies with CLDP require protection against respiratory syncytial virus (RSV) to reduce the risk and severity of bronchiolitis. This involves monthly injections of a monoclonal antibody against the virus called palivizumab. This is very expensive (around £500 per injection) so is reserved for babies meeting certain criteria.
What are the key consequences of the cystic fibrosis mutation?
The key consequences of the cystic fibrosis mutation are:
- Thick pancreatic and biliary secretions that cause blockage of the ducts, resulting in a lack of digestive enzymes such as pancreatic lipase in the digestive tract
- Low volume thick airway secretions that reduce airway clearance, resulting in bacterial colonisation and susceptibility to airway infections
- Congenital bilateral absence of the vas deferens in males. Patients generally have healthy sperm, but the sperm have no way of getting from the testes to the ejaculate, resulting in male infertility
What is primary ciliary dyskinesia?
Primary ciliary dyskinesia (PCD) is also known as Kartagner’s syndrome. It is an autosomal recessive condition affecting the cilia of various cells in the body. It is more common in populations where there is consanguinity, meaning the parents are related to each-other. Consanguinity increases the risk of a child having two copies of the same recessive genetic mutation.
PCD causes dysfunction of the motile cilia around the body, most notably in the respiratory tract. This leads to a buildup of mucus in the lungs, providing a great site for infection that is not easily cleared. This leads to a similar respiratory presentation to cystic fibrosis, with frequent and chronic chest infections, poor growth and bronchiectasis.
It also affects the cilia in the fallopian tubes of women and the tails (flagella) of the sperm in men, leading to reduced or absent fertility.
There is a strong link between primary ciliary dyskinesia and situs inversus.
What is Kartagner’s triad?
Kartagner’s triad describes the three key features of Primary Ciliary Dyskinesia. Not all patients will have all three features. These are:
- Paranasal sinusitis
- Bronchiectasis
- Situs Inversus: where all the internal (visceral) organs are mirrored inside the body. Therefore the heart is on the right, the stomach is on the right and the liver is on the left. Dextrocardia is when only the heart is reversed. Situs inversus on its own does not cause any problems, and patients can expect to live a normal life. A small number have associated congenital heart disease, such as transposition of the great arteries.
How is primary ciliary dyskinesia diagnosed and managed?
Diagnosis
Patients typically present with recurrent respiratory tract infections. Take a careful family history and a history of consanguinity in the parents. Examination and imaging (e.g. chest xray) can be used to diagnose situs inversus. Semen analysis can be used to investigate for male infertility.
The key investigation for establishing the diagnosis is to take a sample of the ciliated epithelium of the upper airway and examine the action of the cilia. A sample can be obtained through nasal brushing or bronchoscopy. Often several samples are required.
Management
Management is similar to cystic fibrosis and bronchiectasis with daily physiotherapy, a high calorie diet and antibiotics.
What are the causes of tonsillitis in paeds?
Tonsillitis refers to inflammation in the tonsils.
The most common cause of tonsillitis is a viral infection. Viral infections do not require or respond to antibiotics.
The most common cause of bacterial tonsillitis is group A streptococcus (Streptococcus pyogenes). This can be effectively treated with penicillin V (phenoxymethylpenicillin). The most common cause of otitis media, rhinosinusitis and the most common alternative bacterial cause of tonsillitis is Streptococcus pneumoniae.
Other causes:
Haemophilus influenzae
Morazella catarrhalis
Staphylococcus aureus
What are the features of tonsillitis?
A typical presentation is a child with a fever, sore throat and painful swallowing. Children aged 5 to 10 are most often affected, with another peak between ages 15 and 20.
Tonsillitis can present with non-specific symptoms, particularly in younger children. They may present with only a fever, poor oral intake, headache, vomiting or even abdominal pain.
Examination of the throat will reveal red, inflamed and enlarged tonsils, with or without exudates. Exudates are small white patches of pus on the tonsils.
Always examine the ears (otoscopy) to visualising the tympanic membranes and palpate for any cervical lymphadenopathy when assessing a child with suspected tonsillitis.
What are the centor criteria?
The Centor criteria can be used to estimate the probability that tonsillitis is due to a bacteria infection, and will benefit from antibiotics.
A score of 3 or more gives a 40 – 60 % probability of bacterial tonsillitis, and it is appropriate to offer antibiotics. A point is given if each of the following features are present:
- Fever over 38ºC
- Tonsillar exudates
- Absence of cough
- Tender anterior cervical lymph nodes (lymphadenopathy)
What is the FeverPAIN score?
The FeverPAIN score is an alternative to the Centor criteria. A score of 2 – 3 gives a 34 – 40% probability and 4 – 5 gives a 62 – 65% probability of bacterial tonsillitis:
Fever during previous 24 hours
P – Purulence (pus on tonsils)
A – Attended within 3 days of the onset of symptoms
I – Inflamed tonsils (severely inflamed)
N – No cough or coryza
How is tonsillitis managed?
It is important to exclude other serious pathology, such as meningitis, epiglottitis and peritonsillar abscess. When tonsillitis is the most likely diagnosis, calculate the Centor criteria or FeverPAIN score.
Educate patients or parents with likely viral tonsillitis, and give safety net advice about when to seek medical advice. Advise simple analgesia with paracetamol and ibuprofen to control pain and fever. NICE clinical knowledge summaries suggest advising patients to return if the pain has not settled after 3 days or the fever rises above 38.3ºC. If this occurs you can start antibiotics or consider an alternative diagnosis.
Consider prescribing antibiotics if the Centor score is ≥ 3 or the FeverPAIN score is ≥ 4. Also consider antibiotics if they are at risk of more serious infections, for example young infants, immunocompromised patients or those with significant co-morbidity, or there is a history of rheumatic fever.
Delayed prescriptions can be considered. This involves educating patients or parents about the likely viral nature of the sore throat, and providing a prescription that is to be collected only in the event that the symptoms do not improve or worsen in the next 2 – 3 days.
Consider admission if the patient is immunocompromised, systemically unwell, dehydrated, has stridor, respiratory distress or evidence of a peritonsillar abscess or cellulitis.
Choice of Antibiotic
Penicillin V (also called phenoxymethylpenicillin) for a 10 day course is typically first line. The trouble with penicillin V is it tastes bad, so young children requiring syrups are often reluctant to take it. Amoxicillin has a better taste but is not part of the guidelines.
Clarithromycin is the first line choice in true penicillin allergy.
What are the complications of tonsillitis?
- Chronic tonsillitis
- Peritonsillar abscess, also known as quinsy
- Otitis media if the infection spreads to the inner ear
- Scarlet fever
- Rheumatic fever
- Post-streptococcal glomerulonephritis
- Post-streptococcal reactive arthritis
What is quinsy?
Quinsy is the common name for a peritonsillar abscess. Peritonsillar abscess arises when there is a bacterial infection with trapped pus, forming an abscess in the region of the tonsils.
Peritonsillar abscesses are usually a complication of untreated or partially treated tonsillitis, although it can arise without tonsillitis.
Quinsy can occur just as frequently in teenagers and young adults as it does in children, unlike tonsillitis which is much more common in children.
What are the presenting features of quinsy (peritonsillar abscess)?
Patients present with similar symptoms to tonsillitis:
Sore throat
Painful swallowing
Fever
Neck pain
Referred ear pain
Swollen tender lymph nodes
Additional symptoms that can indicate a peritonsillar abscess include:
Trismus, which refers to when the patient is unable to open their mouth
Change in voice due to the pharyngeal swelling, described in textbooks as a “hot potato voice”
Swelling and erythema in the area beside the tonsils on examination
Bacteria
Quinsy is usually due to a bacterial infection. The most common organism is streptococcus pyogenes (group A strep), but it is also commonly caused by staphylococcus aureus and haemophilus influenzae.
How is quinsy (peritonsillar abscess) managed?
Patients should be referred into hospital under the care of the ENT team for incision and drainage of the abscess under general anaesthetic.
Quinsy typically has an underlying bacterial cause, therefore antibiotics are appropriate before and after surgery. A broad spectrum antibiotic such as co-amoxiclav would be an appropriate choice to cover the common causes, but local guidelines will guide antibiotic choice according to local bacterial resistance.
Some ENT surgeons give steroids (i.e. dexamethasone) to settle inflammation and help recovery, although this is not universal.
What are the indications for tonsillectomy?
A common question you will get from patients and parents is whether a child needs a tonsillectomy for recurrent tonsillitis. The NICE clinical knowledge summaries give the number of episodes required for a tonsillectomy:
7 or more in 1 year
5 per year for 2 years
3 per year for 3 years
Other indications are:
Recurrent tonsillar abscesses (2 episodes)
Enlarged tonsils causing difficulty breathing, swallowing or snoring
What is the main significant complication of tonsillectomy and how is it managed?
Post tonsillectomy bleeding is the main significant complication after a tonsillectomy. Significant bleeding can occur in up to 5% of patients who have had a tonsillectomy and it requires urgent management. This can happen up to 2 weeks after the operation. Bleeding can be severe and in rare cases life threatening, usually due to aspiration of blood.
Management:
- Call the ENT registrar and get them involved early
- Get IV access and send bloods including a FBC, clotting screen, group and save and crossmatch
Keep the child calm and give adequate analgesia
- Sit them up and encourage them to spit the blood rather than swallowing
- Make the child nil by mouth incase an anaesthetic and operation is required
- IV fluids for maintenance and resuscitation as required
- If there is severe bleeding or airway compromise, call an anaesthetist as intubation may be required.
Prior to going back to theatre there are two options for stopping less severe bleeds:
- Hydrogen peroxide gargle
- Adrenalin soaked swab applied topically
What is otitis media and what are the common causes?
Otitis media is the name given to an infection in the middle ear. The middle ear is the space that sits between the tympanic membrane (ear drum) and the inner ear. This is where the cochlea, vestibular apparatus and nerves are found. It is a very common site of infection in children. The bacteria enter from the back of the throat through the eustachian tube. A bacterial infection of the middle ear is often preceded by a viral upper respiratory tract infection.
Bacteria
The most common bacterial causes of otitis media, as well as other ENT infections such as rhino-sinusitis and tonsillitis is:
Streptococcus pneumoniae.
Other common causes include:
- Haemophilus influenzae
- Moraxella catarrhalis
- Staphylococcus aureus
What are the presenting features of otitis media?
Otitis media typically presents with ear pain, reduced hearing in the affected ear and general symptoms of upper airway infection such as fever, cough, coryzal symptoms, sore throat and feeling generally unwell.
When the infection affects the vestibular system it can cause balance issues and vertigo. When the tympanic membrane has perforated there may be discharge from the ear.
It is worth noting that symptoms can be very non-specific, particularly in young children and infants. They may present with symptoms of fever, vomiting, irritability, lethargy or poor feeding. It is always worth examining the ears in unwell children.
How does the tympanic membrane look in otitis media?
In a normal child the tympanic membrane should be “pearly-grey”, translucent and slightly shiny. You should be able to visualise the malleus through the membrane and a cone of light reflecting the light of the otoscope.
Otitis media will give a bulging, red, inflamed looking membrane. When there is a perforation, you may see discharge in the ear canal and a hole in the tympanic membrane.
How is otitis media managed?
Consider referral to paediatrics for assessment or admission if symptoms are severe or there is diagnostic doubt. Always refer for specialist assessment and to consider admission in infants younger than 3 months with a temperature above 38ºC or 3 – 6 months with a temperature higher than 39ºC.
Most cases of otitis media will resolve without antibiotics, and NICE guidelines from 2018 highlight the importance of not providing antibiotics for otitis media. They state that most cases of otitis media will resolve within 3 days without antibiotics, but it can last for up to a week. Complications (mainly mastoiditis) are rare. Give simple analgesia to help with pain and fever.
There are three options regarding prescribing antibiotics to patients with otitis media:
- Immediate antibiotics
- Delayed prescription
- No antibiotics
Consider prescribing antibiotics at the initial presentation in patients who have significant co-morbidities, are systemically unwell or are immunocompromised. Children less than 2 years with bilateral otitis media and children with otorrhoea (discharge) are more likely to benefit from antibiotics.
Consider a delayed prescription that can be collected and used after 3 days if symptoms have not improved or have worsened at any time. This can be useful with patients that are very keen on antibiotics or where you suspect they might get worse.
The first line choice of antibiotic is amoxicillin for 5 days. Alternatives are erythromycin and clarithromycin.
Always safety-net, offering education and advice to patients and parents on when to seek further medical attention.
What are the complications of otitis media?
Otitis medial with effusion
Hearing loss (usually temporary)
Perforated eardrum
Recurrent infection
Mastoiditis (rare)
Abscess (rare)
What is glue ear?
Glue ear is also known as otitis media with effusion. The middle ear becomes full of fluid, causing a loss of hearing in that ear.
The Eustachian tube connects the middle ear to the back of the throat. It helps drain secretions from the middle ear. When it becomes blocked, this causes middle ear secretions (fluid) to build up in the middle ear space.
The main symptom of glue ear is a reduction in hearing in that ear. The main complication of glue ear is infection (otitis media).
Otoscopy can show a dull tympanic membrane with air bubbles or a visible fluid level, although it can look normal.
How is glue ear managed?
Management
Referral for audiometry to help establish the diagnosis and extent of hearing loss. Glue ear is usually treated conservatively, and resolves without treatment within 3 months. Children with co-morbidities affecting the structure of the ear, such as Down’s syndrome or cleft palate may require hearing aids or grommets.
Grommets
Grommets are tiny tubes inserted into the tympanic membrane by an ENT surgeon. This allows fluid from the middle ear to drain through the tympanic membrane to the ear canal. Usually grommets are inserted under general anaesthetic as a day case procedure. The procedure is relatively safe with few complications. Grommets usually fall out within a year, and only 1 in 3 patients require further grommets to be inserted for persistent glue ear.
What are the common causes of hearing loss?
Congenital
- Maternal rubella or cytomegalovirus infection during pregnancy
- Genetic deafness can be autosomal recessive or autosomal dominant
- Associated syndromes, for example Down’s syndrome
Perinatal
- Prematurity
- Hypoxia during or after birth
After birth
- Jaundice
- Meningitis and encephalitis
- Otitis media or glue ear
- Chemotherapy
What is audiometry?
Younger children (under 3 years) are tested by looking for a basic response to sound (i.e. turning towards a sound). Older children can be tested properly with headphones and specific tones and volumes. The results of audiometry testing are recorded on an audiogram, which can help identify and differentiate conductive and sensorineural hearing loss.
Audiogram
Audiograms are charts that document the volume at which patients can hear different tones. The frequency in hertz (Hz) is plotted on the x-axis, from low to high pitched. The volume in decibels (dB) is plotted on the y-axis, from loud at the bottom to quiet at the top. It is worth noting that the lower down the chart, the higher the decibels and the louder the volume.
Hearing is tested to establish the minimum volume required for the patient to hear each frequency, and this level is plotted on the chart. The louder the sound required for the patient to hear, the worse their hearing is and the lower on the chart they will plot. For example, a 1000 Hz sound will be played at various volumes until the patient can just about hear the sound. If this sound is heard at 15 dB, a mark is made on the chart where 1000 Hz meets 15 dB. If this sound can only be heard at 80 dB, a mark is made where 1000 Hz meets 80 dB.
Hearing is tested in both ears separately. Both air and bone conduction are tested separately. The following symbols are used to mark each of these separate measurements:
X – Left sided air conduction
] – Left sided bone conduction
O – Right sided air conduction
[ – Right sided bone conduction
When a patient has normal hearing, all readings will be between 0 and 20 dB, at the top of the chart.
In patients with sensorineural hearing loss, both air and bone conduction readings will be more than 20 dB, plotted below the 20 dB line on the chart. This may affect only one side, one side more than the other or both sides equally.
In patients with conductive hearing loss, bone conduction readings will be normal (between 0 and 20 dB), however air conduction readings will be greater than 20 dB, plotted below the 20 dB line on the chart. In conductive hearing loss, sound can travel through bones but is not conducted through air due to pathology along the route into the ear.
In patients with mixed hearing loss, both air and bone conduction readings will be more than 20 dB, however there will be a difference of more than 15 dB between the two (bone conduction > air conduction).
How is hearing loss managed?
Establishing the diagnosis is the first step. After the diagnosis is established, input from the multidisciplinary team is required for support with hearing, speech, language and learning:
Speech and language therapy
Educational psychology
ENT specialist
Hearing aids for children who retain some hearing
Sign language
How are cleft lip and palate managed?
Patients should be referred to the local cleft lip services. This involves the specialist multi-disciplinary team:
Specialist nurses to support and coordinate care
Plastic, maxillofacial and ENT surgeons
Dentists
Speech and language therapists
Psychologists
General practitioners
The first priority is to ensure the baby can eat and drink. This may involve specially shaped bottles and teats. The specialist nurse will follow the child up through surgery and beyond to ensure good development.
The definitive treatment is to surgically correct the cleft lip or palate. This leaves a subtle scar, but is generally very successful, giving full functionality to the child. Cleft lip surgery is usually performed at 3 months, whilst cleft palate surgery done at 6 – 12 months.
What is ankyloglossia and how is it managed?
Tongue tie is also known as ankyloglossia. This is when a baby is born with a short and tight lingual frenulum, the attachment of the tongue to the floor of the mouth. This prevents them properly extending their tongue out of the mouth and makes it difficult for them to latch onto the breast. It usually presents as poor feeding or when noticed by the mother, midwife or doctor on newborn checks.
Management
Mild tongue tie can be monitored and would not be expected to cause any issues.
When it affect feeding they may benefit from treatment. Tongue tie can be cured with a frenotomy. This involves a trained person cutting the tongue tie. This can usually be done on the ward or in the clinic without any anaesthetic. Complications are very rare, and include excessive bleeding, scar formation and infection.
What is a cystic hygroma and what are the features?
A cystic hygroma is a malformation of the lymphatic system that results in a cyst filled with lymphatic fluid. It is most commonly a congenital abnormality and is typically located in the posterior triangle of the neck on the left side.
It may be seen on antenatal scans, picked up on routine baby checks or discovered later when noticed incidentally.
Key Features
Cystic hygromas most commonly present in the neck or armpit. They:
- Can be very large
- Are soft
- Are non-tender
- Transilluminate
To transilluminate the cystic hygroma, hold a pen torch flat against the skin and watch as the whole thing lights up like a bulb.
What are the complications of cystic hygromas and how are they managed?
Complications
Depending on the location and size, cystic hygromas can interfere with feeding, swallowing or breathing. It can become infected, in which case it will turn red, hot and tender. There can be haemorrhage into the cyst.
Management
Treatment varies based on the size, location and complications. Watching and waiting can be appropriate as it is a benign condition. They do not resolve spontaneously, but can show some regression.
Aspiration (giving temporary improvement), surgical removal and sclerotherapy are treatment options.
What are thyroglossal cysts and what are the features?
During fetal development, the thyroid gland starts at the base of the tongue. From here it gradually travels down the neck to its final position in front of the trachea, beneath the larynx. It leaves a track behind called the thyroglossal duct, which then disappears. When part of the thyroglossal duct persists it can give rise to a fluid filled cyst. This is called a thyroglossal cyst.
Ectopic thyroid tissue is a key differential diagnosis, as this commonly occurs at a similar location.
The main complication is infection of the cyst, causing a hot, tender and painful lump.
Features
Thyroglossal cysts usually occur in the midline of the neck. They are:
- Mobile
- Non-tender
- Soft
- Fluctuant
Thyroglossal cysts move up and down with movement of the tongue. This is a key feature that demonstrates a midline neck lump is a thyroglossal cyst. This occurs due to the connection between the thyroglossal duct and the base of the tongue.
How are thyroglossal cysts managed?
Ultrasound or CT scan can confirm the diagnosis.
Thyroglossal cysts are usually surgically removed to provide confirmation of the diagnosis on histology and prevent infections. The cyst can reoccur after surgery unless the full thyroglossal duct is removed.
What is splitting of the second heart sound?
During inspiration the chest wall and diaphragm pull the lungs open. This also pulls the heart open. This is called negative intra-thoracic pressure. This causes the right side of the heart to fill faster as it pulls in blood from the venous system. The increased volume in the right ventricle causes it to take longer for the right ventricle to empty during systole, causing a delay in the pulmonary valve closing. When the pulmonary valve closes slightly later than the aortic valve, this causes the second heart sound to be “split”.
How does E coli cause gastroenteritis and what is the complication?
Escherichia coli (E. coli) is a normal intestinal bacteria. Only certain strains cause gastroenteritis. It is spread through contact with infected faeces, unwashed salads or contaminated water.
E. coli 0157 produces the Shiga toxin. This causes abdominal cramps, bloody diarrhoea and vomiting. The Shiga toxin destroys blood cells and leads to haemolytic uraemic syndrome (HUS).
The use of antibiotics increases the risk of haemolytic uraemic syndrome, therefore antibiotics should be avoided if E. coli gastroenteritis is considered.
How is campylobacter jejuni spread?
Campylobacter is a common cause of travellers diarrhoea. It is the most common bacterial cause of gastroenteritis worldwide. Campylobacter means “curved bacteria”. It is a gram negative bacteria that has a curved or spiral shape. It is spread by:
Raw or improperly cooked poultry
Untreated water
Unpasteurised milk
Incubation is usually 2 to 5 days. Symptoms resolve after 3 to 6 days. Symptoms are:
Abdominal cramps
Diarrhoea often with blood
Vomiting
Fever
Antibiotics can be considered after isolating the organism where patients have severe symptoms or other risk factors such as HIV or heart failure. Popular antibiotic choices are azithromycin or ciprofloxacin
How is shigella spread and what is the complication?
Shigella is spread by faeces contaminating drinking water, swimming pools and food. The incubation period is 1 to 2 days and symptoms usually resolve within 1 week without treatment. It causes bloody diarrhoea, abdominal cramps and fever. Shigella can produce the Shiga toxin and cause haemolytic uraemic syndrome. Treatment of severe cases is with azithromycin or ciprofloxacin.
How is salmonella spread?
Salmonella is spread by eating raw eggs or poultry, or food contaminated with the infected faeces of small animals. Incubation is 12 hours to 3 days and symptoms usually resolve within 1 week. Symptoms are watery diarrhoea that can be associated with mucus or blood, abdominal pain and vomiting. Antibiotics are only necessary in severe cases and should be guided by stool culture and sensitivities.
What is bacillus cereus and how is it spread?
Bacillus cereus is a gram positive rod spread through inadequately cooked food. It grows well on food not immediately refrigerated after cooking. The typical food is fried rice left out at room temperature.
Whilst growing on food it produces a toxin called cereulide. This toxin causes abdominal cramping and vomiting within 5 hours of ingestion. When it arrives in the intestines it produces different toxins that cause a watery diarrhoea. This occurs more than 8 hours after ingestion. All of the symptoms usually resolves within 24 hours.
The typical course is vomiting within 5 hours, then diarrhoea after 8 hours, then resolution within 24 hours.
What are the viral causes of gastroenteritis?
Viral gastroenteritis is common. It is highly contagious. Common causes are:
- Rotavirus
- Norovirus
What are the causes of vulvovaginitis in children?
Vulvovaginitis refers to inflammation and irritation of the vulva and vagina. It is a common condition often affecting girls between the ages of 3 and 10 years.
This irritation is caused by sensitive and thin skin and mucosa around the vulva and vagina in young girls. The vagina is more prone to colonisation and infection with bacteria spread from faeces. It can be exacerbated by:
- Wet nappies
- Use of chemicals or soaps in cleaning the area
- Tight clothing that traps moisture or sweat in the area
- Poor toilet hygiene
- Constipation
- Threadworms
- Pressure on the area, for example horse riding
- Heavily chlorinated pools
Vulvovaginitis improves and is much less common after puberty, as oestrogen helps keep the skin and vaginal mucosa healthy and resistant to infection.
What are the presenting features of vulvovaginitis?
Vulvovaginitis is a common presentation in young girls before puberty. It presents with:
Soreness
Itching
Erythema around the labia
Vaginal discharge
Dysuria (burning or stinging on urination)
Constipation
A urine dipstick may show leukocytes but no nitrites. This will often result in misdiagnosis as a urinary tract infection.
How is vulvovaginitis managed?
Often patients have already been treated for urinary tract infections and thrush, usually with little improvement in symptoms. It is unusual for girls to develop thrush before puberty.
Generally no medical treatment is required and management focuses on simple measures to improve symptoms:
Avoid washing with soap and chemicals
Avoid perfumed or antiseptic products
Good toilet hygiene, wipe from front to back
Keeping the area dry
Emollients, such as sudacrem can sooth the area
Loose cotton clothing
Treating constipation and worms where applicable
Avoiding activities that exacerbate the problem
In severe cases an experienced paediatrician may recommend oestrogen cream to improve symptoms.
What is nephrotic syndrome and what are the features?
Nephrotic syndrome occurs when the basement membrane in the glomerulus becomes highly permeable to protein, allowing proteins to leak from the blood into the urine. It is most common between the ages of 2 and 5 years. It presents with frothy urine, generalised oedema and pallor.
Nephrotic syndrome features a classic triad of:
- Low serum albumin
- High urine protein content (>3+ protein on urine dipstick)
- Oedema
There are three other features that occur in patients with nephrotic syndrome:
- Deranged lipid profile, with high levels of cholesterol, triglycerides and low density lipoproteins
- High blood pressure
- Hyper-coagulability, with an increased tendency to form blood clots
What are the causes of nephrotic syndrome?
The most common cause in children is minimal change disease, causing over 90% of cases in children under 10. In minimal change disease, nephrotic syndrome occurs in isolation, without any clear underlying condition or pathology. There are a number of secondary causes of nephrotic syndrome, where it occurs due to an underlying condition.
It can be secondary to intrinsic kidney disease:
- Focal segmental glomerulosclerosis
- Membranoproliferative glomerulonephritis
It can also be secondary to an underlying systemic illness:
- Henoch schonlein purpura (HSP)
- Diabetes
- Infection, such as HIV, hepatitis and malaria
How is minimal change disease diagnosed and managed?
Minimal change disease is the most common cause of nephrotic syndrome in children. It can occur in otherwise healthy children, without any clear risk factors or reason for developing the condition. It is not clear why it occurs in most cases.
A renal biopsy and standard microscopy in minimal change disease is usually NOT able to detect any abnormality. Urinalysis (analysis of the urine) will show small molecular weight proteins and hyaline casts.
Management of minimal change disease is with corticosteroids (i.e. prednisolone). The prognosis is good and most children make a full recovery, however it may reoccur.
How is nephrotic syndrome managed?
Nephrotic syndrome should be managed by experienced paediatricians with input from renal specialists. General management is with:
High dose steroids (i.e. prednisolone)
Low salt diet
Diuretics may be used to treat oedema
Albumin infusions may be required in severe hypoalbuminaemia
Antibiotic prophylaxis may be given in severe cases
High dose steroids are given for 4 weeks and then gradually weaned over the next 8 weeks:
80% of children will respond to steroids, and are referred to as steroid sensitive
80% of steroid sensitive patients will relapse at some point and need further steroids
Patients that struggle to wean steroids due to relapses are referred to as steroid dependant
Patients that do not respond to steroids are referred to as steroid resistant
In steroid resistant children, ACE inhibitors and immunosuppressants such as cyclosporine, tacrolimus or rituximab may be used.
What are the complications of nephrotic syndrome?
- Hypovolaemia occurs as fluid leaks from the intravascular space into the interstitial space causing oedema and low blood pressure.
- Thrombosis can occur because proteins that normally prevent blood clotting are lost in the kidneys, and because the liver responds to the low albumin by producing pro-thrombotic proteins.
- Infection occurs as the kidneys leak immunoglobulins, weakening the capacity of the immune system to respond. This is exacerbated by treatment with medications that suppress the immune system, such as steroids.
- Acute or chronic renal failure
- Relapse
What is nephritis and what are the causes?
Nephritis refers to inflammation within the nephrons of the kidneys. It causes:
- Reduction in kidney function
- Haematuria: invisible or visible amounts of blood in the urine
- Proteinuria: although less than in nephrotic syndrome
The two most common causes of nephritis in children are post-streptococcal glomerulonephritis and IgA nephropathy (Berger’s disease).
What is post-streptococcal gomerulonephritis and how is it managed?
Post-streptococcal glomerulonephritis occurs 1 – 3 weeks after a β-haemolytic streptococcus infection, such as tonsillitis caused by Streptococcus pyogenes. Immune complexes made up of streptococcal antigens, antibodies and complement proteins get stuck in the glomeruli of the kidney and cause inflammation. This inflammation leads to an acute deterioration in renal function, causing an acute kidney injury.
Consider a diagnosis of post-streptococcal glomerulonephritis where there is evidence of recent tonsillitis caused by streptococcus. This could be a history of tonsillitis, positive throat swab results and anti-streptolysin antibody titres found on a blood test.
Management is supportive and around 80% of patients will make a full recovery. In some cases patients can develop a progressive worsening of their renal function. They may need treatment with antihypertensive medications and diuretics if they develop complications such as hypertension and oedema.
What is IgA nephropathy and how is it managed?
IgA nephropathy is also known as Berger’s disease. This condition is related to Henoch-Schonlein Purpura, which is an IgA vasculitis. IgA deposits in the nephrons of the kidney causes inflammation (nephritis). When a renal biopsy is taken the histology will show “IgA deposits and glomerular mesangial proliferation”.
It usually presents in teenagers or young adults.
Management involves supportive treatment of the renal failure and immunosuppressant medications such as steroids and cyclophosphamide to slow the progression of the disease.
