Paediatrics Flashcards
Stillbirth definition
Foetus born with no signs of life >= 24 weeks of pregnancy
Neonate definition
<= 28 days old
Preterm definition
Gestation <37 weeks of pregnancy
Term definition
37-41 weeks gestation
Low birth weight definition
< 2500g
Very low birth weight definition
< 1500g
Extremely low birth weight definition
< 1000g
Small for gestational age definition
Birthweight < 10th centile for gestational age
Large for gestational age
Birthweight > 90th centile for gestational age
In utero, glucocorticoids can be given to the mother to…
…Help accelerate lung maturation in the foetus
In utero, digoxin or flecainide can be given to the mother to…
…Treat foetal SVT
Rhesus isoimmunisation (aka HDFN due to anti-D antibodies) can be treated in utero via…
…Foetal blood transfusion directly into the umbilical vein
Perinatal isoimmune thrombocytopenia is when…
…Anti-platelet antibodies from the mother cross the placenta and cause thrombocytopenia in the foetus (can be treated with IVIg)
Multiple births are associated with (5 things)…
Preterm labour (median twin gestation is 37 weeks), IUGR, congenital abnormalities, twin-twin transfusion syndrome in mitochondrion twins (share a placenta), complicated deliveries
Foetal problems associated with maternal diabetes
Congenital malformations, IUGR, macrosomia (high birth weight)
Mechanism of macrosomia in maternal diabetes mellitus
Maternal hyperglycaemia causes foetal hyperglycaemia. Insulin does not cross the placenta, so the foetus produces its own, which promotes growth. Macrosomia is associated with increased risk of cephalopelvic disproportion, birth asphyxia, shoulder dystocia and brachial plexus injury
Neonatal problems associated with maternal diabetes
Hypoglycaemia (transient due to hyperinsulinaemia), respiratory distress syndrome, hypertrophic cardiomyopathy, polycythaemia
What is gestational diabetes?
When carbohydrate intolerance occurs only during pregnancy (more common in Asian and Afro-Caribbean women)
Foetal hyperthyroidism is present in 1-2% of babies born to a mother with Graves disease (due to circulating TSH), how is it detected?
Tachycardia on CTG trace and foetal goitre on ultrasound
Maternal SLE with anti-phospholipid syndrome is associated with (5 things)…
…Recurrent miscarriage, IUGR, pre-eclampsia, placental abruption, preterm delivery
What is neonatal lupus syndrome?
Some infants born to a mother with anti-Ro and anti-La antibodies will develop a self-limiting rash and (rarely) heart block
Maternal AITP can cause thrombocytopenia in infants. Describe the mechanism for this and name the treatment
Maternal IgG antibodies cross the placenta and damage foetal platelets. Treated with IVIg
Give the consequences of each of the following drugs/treatments during pregnancy: Cocaine Opioid analgesia Epidural analgesia Sedatives Oxytocin and PGF2 IV fluids
Cocaine - associated with placental abruption and preterm delivery
Opioids - may suppress respiration at birth
Epidural - may cause maternal pyrexia during labour (hard to distinguish from infection)
Sedatives - may cause sedation, hypothermia and hypotension in the newborn
Oxytocin/PGF2 - may cause hyperstimulation of the uterus leading to foetal hypoxia
IV fluids - may cause neonatal hyponatraemia
Triad of Rubella infection in the newborn
Cataracts, deafness, congenital heart disease (PDA)
What is the most common congenital infection? Give its clinical features and treatment
CMV
Clinical features - hepatosplenomegaly, petechiae, sensorineural hearing loss, cerebral palsy, epilepsy, cognitive impairment
Treatment - IV ganciclovir or oral valganciclovir
Newborn toxoplasmosis clinical features and management
Mostly asymptomatic but can present with retinopathy (due to chorioretinitis), cerebral calcification and hydrocephalus
Management - Pyrimethamine + sulfadiazine + calcium folinate (possible adjunctive prednisolone)
Prevention and treatment of maternal varicella zoster infection
Prevention - VZIG
Treatment - Aciclovir
Congenital syphilis features
Rash on soles of feet and on hands
Bone lesions
Describe primary, secondary (terminal) apnoea
In utero, if a foetus is deprived of oxygen they will attempt to breathe (unsuccessfully). This is primary apnoea. If oxygen deprivation continues the foetus will gasp irregularly, followed by another period of apnoea (secondary/terminal) during which HR and BP fall. If an infant is delivered after terminal apnoea they will require help breathing in some form
APGAR score (used to describe a baby’s condition at 1 and 5 minutes after birth, and 5 minute intervals thereafter if the condition remains poor)
Appearance (blue/pale = 0, pink body/blue extremities = 1, pink = 2)
Pulse (absent = 0, below 100bpm = 1, >100bpm = 2)
Grimace (floppy = 0, minimal response to stimulation = 1, prompt response to stimulation = 2)
Activity (absent = 0, flexed arms/legs = 1, active = 2)
Respiration (absent = 0, slow/irregular = 1, vigorous cry = 2)
Define asymmetrical growth restriction and give the mechanism by which it occurs
Weight or abdominal circumference at a lower centile than that of the head. It occurs when the placenta fails to provide adequate nutrition late in pregnancy but brain growth is relatively spared at the expense of liver glycogen and skin fat
Define symmetrical growth restriction
Growth restriction where head circumference and weight/abdominal circumference are equally reduced. Due to poor growth in early pregnancy
Infants with Beckwith-Wiedemann syndrome are often … for gestational age and have difficulty …
Large
Breathing (due to macroglossia)
What is developmental dysplasia of the hip (DDH)?
Aka congenital dislocation of the hip (CDH), it is where the socket portion does not fully cover the ball portion, resulting in increased risk of dislocation. It is often managed with careful observation, but may require hip abduction orthosis (splint) in a Pavlik harness if it persists or worsens. It is associated with Breech deliveries
Vitamin K is given to newborns as an IM injection to avoid what condition?
Haemorrhagic disease of the newborn. This can cause intracranial haemorrhage.
NOTE: Breast milk has much less vitamin K than formula milk, and infants of mothers taking anticonvulsants are at increased risk of HDN
Guthrie Testing via heel prick at day 5-9 of life (after feeding has been established) screens for which conditions?
Phenylketonuria
Hypothyroidism
Haemoglobinopathies (sickle cell and thalassaemia)
CF (given by measuring serum immunoreactive trypsin)
MCAD deficiency
Hypoxic-Ischaemic Encephalopathy (HIE) triad
Hypoxia, hypercapnia, metabolic acidosis
HIE classification
Mild - Infant is irritable and responds excessively to stimulation
Moderate - Infant shows marked abnormalities of tone/movement and may have seizures
Severe - No spontaneous movements or response to pain, seizures are prolonged and multi-organ failure may occur
What is caput succedaneum?
Bruising and oedema of the head extending beyond the margins of the skull bones
What is cephalhaematoma?
Haematoma from bleeding below the periosteum, confined within the margins of the skull sutures (usually involves the parietal bone)
Which nerve roots are affected in Erb’s palsy?
C5 and C6
Give Respiratory Distress Syndrome (RDS) risk factors. Which medications can be given to mothers antenatally to promote surfactant production?
Pre-term babies and babies born to diabetic mothers. Glucocorticoids can be given antenatally
Patent ductus arteriosus (PDA) can cause shunting of blood from the left to the right side of the heart. What are the signs and symptoms of PDA?
Apnoea
Bradycardia
Bounding pulse
Systolic murmur
What is the management for a patent ductus arteriosus (PDA)?
Closure of the duct with IV indomethacin, prostacyclin synthetase inhibitors or ibuprofen. If these are unsuccessful, surgery is required
Why are preterm infants at higher risk of infection?
Because IgG is mainly transferred across the placenta in the final trimester of pregnancy
Periventricular leukomalacia (PVL) is the presence of multiple bilateral cysts in the white matter of the brain. It is associated with high-risk of spastic diplegia. What is spastic diplegia?
A form of cerebral palsy that manifests as especially high tightness or stiffness in the muscles of the lower extremities.
Give the features of Necrotising Enterocolitis (clinical and xray).
NOTE: It is treated with broad spectrum abx and surgery.
Clinical: Difficulty feeding Aspiration of milk from the stomach Vomiting Abdominal distension Rectal bleeding
xray:
Distended loops of bowel
Thickening of the bowel wall with intramural gas
Gas in the portal tract
Retinopathy of prematurity is associated with…
…Use of high concentrations of oxygen, and very low birth weight
Define bronchopulmonary dysplasia.
Ongoing oxygen requirement at the age of 36 weeks.
NOTE: Lung damage occurs due to artificial ventilation, oxygen toxicity and infection (pertussis/RSV). Corticosteroids may facilitate earlier weaning.
CXR changes in bronchopulmonary dysplasia.
Widespread opacification, sometimes with cystic changes
Define kernicterus. Why is its incidence decreasing?
Encephalopathy resulting from the deposition of unconjugated bilirubin in the basal ganglia and brainstem nuclei.
It is less common now because it used to be caused by severe rhesus haemolytic disease, however this has become rarer with the introduction of prophylactic anti-D immunoglobulin for rhesus negative mothers
What is opisthotonos? What conditions is it seen in?
Increased muscle tone causing an infant to lie with an arched back. It is seen in kernicterus and meningitis.
Causes of jaundice at < 24 hours of age.
Rhesus haemolytic disease ABO incompatibility G6PD deficiency Hereditary spherocytosis Congenital infection
Which ABO antibody can cause jaundice in infants? How do we test for ABO incompatibility?
IgG anti-A-haemolysin (IgG can cross the placenta but IgM can’t).
We test for ABO incompatibility with the direct antibody (Coombs’) test. Positive Coombs’ test means ABO incompatibility
Causes of jaundice between 2 days and 2 weeks of age.
Physiological jaundice Breast milk jaundice Dehydration Infection Crigler-Najjar syndrome (deficient or absent UGT)
Causes of jaundice in infants > 2 weeks of age.
Biliary atresia Breast milk jaundice Infection (especially UTI) Congenital hypothyroidism Neonatal hepatitis syndrome
Treatment of neonatal jaundice.
Use threshold table to determine whether bilirubin measurement warrants treatment
Phototherapy (1st line) - converts unconjugated bilirubin into water-soluble pigment
Other options:
Exchange transfusion
IViG
What is the most common cause of respiratory distress in term infants?
Transient tachypnoea of the newborn - caused by delay in the reabsorption of lung liquid, and usually settles within the first day of life
Give 3 complications of meconium aspiration, and give its management.
Pneumothorax
Pneumomediastinum
Persistent pulmonary hypertension
Management:
In normal term infant with no FHx of group B streptococcus, observation is recommended
If there is suspected infection/risk factors for infection, give IV ampicillin AND gentamicin
Consider oxygen therapy/CPAP in severe cases
Give 3 factors that predispose to pneumonia in a neonate.
Prolonged rupture of the membranes
Low birthweight
Chorioamnionitis
Why is an urgent echocardiogram needed in infants with persistent pulmonary hypertension of the newborn? What is the management?
PPHN causes right-to-left shunting of blood, so an echo must be done to check for any congenital heart defects.
Management: Inhaled nitric oxide Sildenafil (viagra) High frequency (oscillatory) ventilation ECMO (in severe but reversible cases)
What is the most common diaphragmatic herniation?
Left-sided herniation of the abdominal contents through the posterolateral foramen of the diaphragm
What is the management of a diaphragmatic hernia?
Large NG tube passed and suction applied to prevent distension of the intrathroacic bowel. Once stabilised, surgery is required to repair the herniation
What is the main consequence of diaphragmatic herniation?
Pulmonary hypoplasia
What is the most common pathogen causing late onset infection in newborns (> 48 hours after birth)?
Coagulase negative Staphylococcus (staph. epidermidis)
Other common organisms include: Gram positives (S. aureus, E. faecalis) Gram negatives (Klebsiella, Pseudomonas, Serratia)
What is the treatment for neonatal meningitis?
Ampicillin or penicillin AND a 3rd generation cephalosporin (e.g. cefotaxime)
Management of Group B Streptococcus infection in neonates.
Benzylpenicillin OR ampicillin
AND
Gentamicin OR cefotaxime OR ceftriaxone
What are the characteristic clinical signs of listeria monocytogenes infection in neonates?
Meconium staining of the amniotic fluid Widespread rash Septicaemia Pneumonia Meningitis
What is the management of listeria monocytogenes infection?
Amoxicillin OR co-trimoxazole (NOT trimethoprim)
If systemic infection: IV ampicillin or benzylpenicillin with gentamicin
What are the 2 organisms to check for in neonates with bacterial conjunctivitis? What are the treatments for each of them?
Gonococcus - treat with 3rd generation cephalosporin
Chlamydia - treat with erythromicin for 2 weeks
Which infants should receive hepatitis B vaccination after birth?
Infants of mothers who are HbsAg positive - vaccination reduces the risk of vertical transmission
What is neonatal stroke?
(AKA cerebral infarction)
It is infarction in the territory of the middle cerebral artery, which may present with seizures
Cleft lip results from failure of fusion of …
Cleft palate results from failure of fusion of …
… the frontonasal and maxillary processes
… the palatine processes and the nasal septum
Infants with Pierre Robin Sequence experience cyanotic episodes which may cause failure to thrive. What 3 craniofacial features are associated with Pierre Robin Sequence?
Micrognathia
Glossoptosis
Midline cleft of the soft palate
Define failure to thrive.
Weight for age that falls below the 5th percentile on multiple occasions or weight deceleration that crosses 2 major percentile lines on a growth chart
How does oesophageal atresia usually present? What occurrence during pregnancy is it associated with?
Persistent salivation and drooling
Polyhydramnios during pregnancy
Give 5 causes of small bowel obstruction in neonates.
Atresia/stenosis of the duodenum Atresia/stenosis of the jejunum/ileum Malrotation with volvulus Meconium ileus Meconium plug
Give 2 causes of large bowel obstruction in neonates.
Hirschsprung disease - absence of the myenteric nerve plexus in the rectum which may extend along the colon
Rectal atresia - absence of the anus at the normal site
What is exomphalos (omphalocele)?
When abdominal contents protrude through the umbilical ring and are covered by a transparent sac (formed by the amniotic membrane and peritoneum)
What is gastroschisis?
When bowel protrudes through a defect in the anterior abdominal wall adjacent to the umbilicus and there is no covering sac
Which, out of exomphalos and gastroschisis, is associated with other major congenital abnormalities?
Exomphalos
What is the difference between posseting and regurgitation?
Both describe the non-forceful return of milk, but posseting refers to small amounts of milk that often accompany the return of swallowed air (a burp), while regurgitation refers to larger, more frequent losses
What is the mechanism of GORD in infants (usually presents early and resolves within 1 year)?
Inappropriate relaxation of the lower oesophageal sphincter as a result of functional immaturity
What condition should you suspect in infants with projectile vomiting? What clinical signs might help you to diagnose it?
Pyloric stenosis
Visible gastric peristalsis
Pyloric mass ('olive shaped') in RUQ
What is the definitive treatment for pyloric stenosis?
Ramstedt pyloromyotomy - this involves dividing the hypertrophied muscle down to but not including the mucosa
What is the typical pattern of symptoms in infant colic?
Paroxysmal inconsolable crying or screaming often accompanied by drawing up of the knees and passage of excessive flatus. Occurs several times per day
Intussusception is the most common cause of intestinal obstruction in infants after the neonatal period. What is the presentation and management of intussusception?
Presentation: Paroxysmal, severe colicky pain with pallor Refusing feeds Vomiting Redcurrant jelly stool Abdominal distension
Management:
Rectal air insufflation (unless there are signs of peritonitis)
Explain Meckel’s diverticulum. What test is used to identify it?
2% of people have an ileal remnant of the omphalomesenteric duct, containing either ectopic gastric mucosa or pancreatic tissue. This can bleed, presenting with severe PR bleeding (neither bright red, nor melaenia). It is identified using a technetium scan, which shows increased uptake by ectopic gastric mucosa in 70% of cases
What predisposes to volvulus in infants?
Malrotation - during rotation of the small bowel in foetal life, if the mesentery is not fixed at the duodenojejunal flexure or in the ileocaecal region, its base is shorter and this predisposes to volvulus
What presents with bilious green vomiting?
Intestinal obstruction
What is a Ladd band?
A fibrous stalk of peritoneal tissue that attaches the caecum to the peritoneum in the right lower quadrant. They may cross the duodenum, leading to obstruction.
What must you always test for before diagnosing someone with IBS?
Coeliac disease
H. pylori produces urease, which forms the basis of which 2 tests for it?
CLO test and C-13 breath test
What is the management of peptic ulceration in children?
PPIs
Eradication of H. pylori if it is present (with amoxicillin AND metronidazole OR clarithromycin)
NOTE: If these treatments fail, an upper GI endoscopy is performed. If this is normal, it is diagnosed as functional dyspepsia
What are the endoscopy findings in a patient with eosinophilic oesophagitis? What is its management?
Linear furrows and trachealisation of the oesophagus
Corticosteroids (fluticasone or viscous budesonide)
What is the most common cause of gastroenteritis in children in developed countries (60% of cases in children < 2 years old)?
Rotavirus
What finding suggests a bacterial cause in patients with gastroenteritis? What is the most common bacterial cause in developed countries?
Blood in the stool
Campylobacter jejuni
What is the most serious complication of gastroenteritis?
Dehydration leading to shock
What is the most accurate measure of dehydration in a child with diarrhoea?
Weight loss
By what mechanism can hyponatraemic dehydration cause seizures?
When children drink a large amount of water to overcome dehydration, they become hyponatraemic. This makes water move from the extracellular compartment to the intracellular compartment, causing an increase in brain volume, which can lead to seizures
Oral rehydration solution is the mainstay of dehydration treatment in children. In what 3 circumstances are IV fluids indicated?
Shock
Deterioration
Persistent vomiting
What is acrodermatitis enteropathica?
Autosomal recessive metabolic disorder characterised by the malabsorption of zinc which results in:
Diarrhoea
Inflammatory rash around the mouth and/or anus
Hair loss
What condition do anti-tTG antibodies indicate?
Coeliac disease
What is the most common cause of persistent diarrhoea in children?
Chronic non-specific diarrhoea
Which portions of the intestines are most commonly affected by Crohns?
Distal ileum or proximal colon
How do we assess severity of ulcerative colitis in children?
Paediatric UC Activity Index (PUCAI):
Severe >65 points
Moderate 10-64 points
Mild <10 points
What is Hirschsprung Disease? How is it diagnosed?
Absence of ganglion cells from the myenteric and submucosal plexuses of the large bowel
Diagnosed via suction full thickness rectal biopsy - will show absence of ganglion cells and presence of large acetylcholinesterase-positive nerve trunks
What should you suspect in a newborn who fails to pass meconium within the first 24 hours of life?
Hirschsprung Disease
How can we measure body temperature in children aged:
< 4 weeks
4+ weeks to 5 years
< 4 weeks old = electronic thermometer in the axilla
4+ weeks to 5 years old = electronic or chemical dot thermometer in the axilla OR infrared tympanic thermometer
NOTE: Axillary measurements tend to underestimate body temperatures by around 0.5 degrees.
During the first few months of life, children are relatively protected from viral infection - why?
Passive immunity from the mother.
In a febrile child, if no clear cause of fever is identified, what should be done?
Urgent investigation with a septic screen and broad-spectrum antibiotics given immediately.
What are the components of a septic screen? (4)
Blood culture
FBC including WCC
Acute phase proteins (e.g. CRP)
Urine sample
What are the red flag features in a febrile child?
Fever > 38 degrees if < 3 months old Fever > 39 degrees if 3-6 months old Colour - pale, mottled or cyanosed Neurological - reduced consciousness, neck stiffness, bulging fontanelle, status epileptics, focal neurological signs, seizures Significant respiratory distress Bile-stained vomiting Severe dehydration or shock
Which parenteral antibiotics should be given to seriously unwell febrile children aged:
< 1 month (who have been discharged from hospital)
1+ months
< 1 month = 3rd generation cephalosporin e.g. cefotaxime (ampicillin is often added to cover listeria)
1+ months = High dose ceftriaxone
NOTE: Aciclovir may be given if herpes simplex encephalitis is suspected.
In children, is meningitis most commonly caused by bacterial infections, viral infections or fungal infections?
Viral infections
What is the pathophysiology of bacterial meningitis?
Bacterial infection of the meninges usually follows bacteraemia
Much of the damage to the meninges is caused by host response to infection rather than the infective organism
Release of inflammatory mediators, recruitment of inflammatory cells and endothelial damage leads to cerebral oedema, raised ICP and decreased cerebral blood flow
Inflammatory responses below the meninges lead to a vasculopathy, resulting in cerebral cortical infarction
Fibrin deposits may block the resorption of CSF by the arachnoid villi leading to hydrocephalus
What organisms should be suspected in bacterial meningitis in children? Break it down by age.
Neonatal - 3 months:
Group B streptococcus
E. coli and other coliforms
Listeria monocytogenes
1 month - 6 years:
N. meningitidis
S. pneumoniae
H. influenzae
> 6 years:
N. meningitidis
S. pneumoniae
What investigations should be ordered for a child with suspected meningitis?
Bloods - CRP, WCC, culture, PCR to check for N. meningitidis
LP - confirm diagnosis, identify causative organism and antibiotic sensitivities
Throat swabs - for culture and PCR
Optional - rapid antigen screens on urine and blood samples
What is the management of meningitis in children?
- Administer single dose of IV/IM benzylpenicillin (consider chloramphenicol or vancomycin if allergic)
- Administer IV ceftriaxone:
Neisseria meningitidis = 7 days
Haemophilus influenzae = 10 days
Strep. pneumoniae = 14 days - Consider dexamethasone if:
- There is frankly purulent CSF
- CSF WBC > 1000/microL
- Raised CSF WBC and protein conc. > 1g/L
- Bacteria on Gram stain
NOTE: Do NOT give steroids in meningococcal septicaemia. - IV 0.9% saline of there are signs of shock/dehydration
What deficiency should we consider testing for in children who have had more than 1 episode of meningococcal disease or an episode caused by a serogroup other than the common ones?
Complement deficiency
What are the cerebral complications of bacterial meningitis? (6)
Hearing impairment
Local vasculitis - can lead to nerve palsies or other focal signs
Local cerebral infarction - may result in seizures/epilepsy
Subdural effusion - particularly with H. influenzae and pneumococcal meningitis
Hydrocephalus
Cerebral abscess
What are the protocols for prophylaxis of bacterial meningitis?
Prophylactic rifampicin or ciprofloxacin can be given to all household contacts for meningococcal meningitis and H. influenzae infection - this will eradicate nasopharyngeal carriage, but should not be given to the patient as the 3rd generation cephalosporin will already achieve this.
Prophylactic meningococcal group C vaccine should be given to all household contacts of patients with group C meningococcal meningitis.
Give 4 common, and 4 uncommon causes (these should be considered in atypical presentations) of viral meningitis.
Common: Enteroviruses EBV Adenoviruses Mumps (now rare in UK due to MMR)
Uncommon: Mycoplasma Borellia burgdorferi (Lyme disease) TB Fungal infections
Give 2 causes of aseptic meningitis.
Malignancy
Autoimmune diseases
How does encephalitis commonly present in children?
Fever
Altered consciousness
Often seizures
What are the most common causes of encephalitis in the UK? (3)
Enteroviruses Respiratory viruses (influenza) Herpes viruses (HSV, VSV, HHV-6)
NOTE: HSV encephalitis is rare in children, but can be devastating.
Until HSV encephalitis is ruled out, what should the initial treatment of children with encephalitis be?
High dose IV aciclovir
What is the management of encephalitis in children? What is the prognosis if untreated?
3 weeks IV aciclovir
If untreated, the mortality rate is 70%
What are the 2 possible causative organisms in toxic shock syndrome?
Toxin-producing S. aureus
Group A streptococci
Toxic shock syndrome is characterised by… (name 3).
Fever > 39 degrees
Hypotension
Diffuse, eryhtematous, macular rash
NOTE: 1-2 weeks after onset of illness, you will see desquamation of the palms, soles, fingers and toes.
In toxic shock syndrome, the toxin acts as a superantigen and can cause organ dysfunction, leading to… (name 6).
Mucositis GI dysnfunction Renal impairment Liver impairment Clotting abnormalities and thrombocytopenia CNS (altered consciousness)
What is the management of toxic shock syndrome?
Intensive care is required
Areas of infection should be surgically debrided
Antibiotics - these often include a 3rd generation cephalosporin (e.g. ceftriaxone), clindamycin (switches off toxin production), IVIG (neutralises circulating toxin)
What is Panton-Valentine Leukocidin (PVL)?
A toxin produced by < 2% of S. aureus strains, which emerged in the UK and causes recurrent skin and soft tissue infections
Can also cause necrotising fasciitis and necrotising haemorrhagic pneumonia following flu-like illness
In children, a procoagulant state induced by the toxin can cause venous thrombosis
What are the 2 common invading organisms in necrotising fasciitis/cellulitis?
Staph. aureus
Group A streptococcus
What is the management of necrotising fasciitis/cellulitis?
Surgical emergency Debridement of all infected and devitalised tissues IV fluids Empirical IV antibiotics (IVIG may also be given)
What does a meningococcal rash look like? What is it called when lesions are extensive?
Purpuric, non-blanching, irregular in size and outline, with necrotic centre.
When the lesions are extensive it is called purport fulminans.
Since the inclusion of the meningococcus C vaccine in the UK, most cases of meningococcal septicaemia are caused by…
…group B meningococci.
What infections can be caused by Strep. pneumoniae? (5)
Pharyngitis Otitis media Conjunctivitis Sinusitis Invasive disease (pneumonia, bacterial sepsis, meningitis) - mainly in young infants with weak immune systems against encapsulated pathogens
How can we avoid Strep. pneumoniae infection in at-risk children (e.g. those with hyposplenism)?
Daily prophylactic penicillin.
Do we vaccinate against Strep. pneumoniae?
Yes, a 13-valent pneumococcal vaccine is included in the UK standard immunisation schedule.
Define impetigo. Where are lesions usually found? (3) What do the lesions look like?
Definition: A localised, highly contagious, staphylococcal or streptococcal skin infection.
Lesions are usually found on the face, neck and hands.
They begin as erythematous macules which become vesicular/pustular or even bulbous. Rupture of the vesicles leads to honey-coloured crusted lesions.
What is the management of impetigo?
Advice - tell parents it won’t scar, inform about importance of hygiene, avoid school until lesions are dry/scabbed over.
Medical:
Localised infection - topical fusidic acid (3-4x/day for 7 days)
Extensive or bullous infection - oral flucloxacillin (4x/day for 7 days)
(Use clarithromycin if allergic to penicillin)
What are boils? How are they treated?
Boils are infections of hair follicles or sweat glands, usually caused by S. aureus.
Treatment = Systemic antibiotics, consider surgical incision.
How is periorbital cellulitis managed?
IV antibiotics (high-dose ceftriaxone)
NOTE: It is almost always unilateral.
What is orbital cellulitis? What are the complications? (3)
It is characterised by proptosis and painful/limited ocular movement with/without reduced visual acuity.
Complications:
Abscess formation
Meningitis
Cavernous sinus thrombosis
What causes scalded skin syndrome?
Exfoliative staphylococcal toxin causes the separation of the epidermis through to the granular cell layers of the skin.
What are the clinical features of scalded skin syndrome?
Fever
Malaise
Purulent, crusting and localised infection around the eyes, nose and mouth
Subsequent widespread erythema and tenderness of the skin
Nikolsky sign - areas of the epidermis will separate on gentle pressure, leaving denuded areas of skin
What is Nikolsky’s sign indicative of?
Scalded skin syndrome
How is scalded skin syndrome managed?
IV antibiotics - flucloxacillin
Analgesia
Monitor hydration/fluid balance
Which human herpes virus is associated with Kaposi’s sarcoma in HIV infected individuals?
HHV-8
What are the 2 ways herpes simplex enters the body?
Mucous membranes
Skin
Which of the herpes simplex viruses is associated with genital lesions and which is associated with lip/skin lesions? How do we manage herpes simplex infection?
NOTE: Both case cause both types of disease.
HSV1 = lip/skin lesions HSV2 = genital lesions
Management = symptomatic (paracetamol/ibuprofen for pain/fever), consider aciclovir (DNA polymerase inhibitor).
What is the most common presentation of HSV in children?
Gingivomastitis - it presents between 10 months and 3 years of age with vesicular lesions on the lips, gums and anterior surfaces of the tongue and hard palate.
It is also accompanied by high fever, and may progress to painful ulceration.
Lasts up to 2 weeks.
Define ‘cold sore’.
Recurrent HSV lesion on the gingival/lip margin.
How does eczema herpeticum present? What complication should we aim to avoid?
Widespread vesicular lesions develop on eczematous skin. This can be complicated by secondary bacterial infection, which can lead to septicaemia.
What are herpetic whitlows? How do they occur?
Painful, erythematous and oedematous white pustules on the site of broken skin (usually on the fingers). They are spread by auto-inoculation from gingivomastitis.
In HSV eye disease (blepharitis or conjunctivitis), what complication should we aim to avoid?
Involvement of the cornea can lead to dendritic ulceration, corneal scaring and loss of vision.
How is chickenpox (primary VZV infection) spread? What is the incubation period? Describe the characteristic rash.
Spread = respiratory droplets
Incubation period = 14 days
Rash = widespread vesicular rash with lesions starting on the head and trunk, and progressing to the peripheries
What are the major complications of chickenpox? (4)
Secondary bacterial infection - mainly with staphylococci and group A streptococci, and can lead to toxic shock syndrome or necrotising fasciitis
Encephalitis - with associated cerebellitis
Purpura fulminans - can lead to loss of large areas of skin by necrosis
Severe progressive disseminated VZV infecition - in immunocompromised patients
What is the management for chickenpox in:
- Immunocompetent adolescents and adults
- Immunocompromised children
What can we do to prevent chickenpox in immunocompromised patients?
- Immunocompetent adolescents/adults = consider oral aciclovir 800mg 5x/day for 7 days
- Immunocompromised children = IV aciclovir
We can give human varicella zoster immunoglobulin in high-risk immunocompromised individuals with deficient T cell function following contact with chickenpox.
What virus causes infectious mononucleosis (glandular fever)? Give 3 other associations of this virus.
EBV
It is also associated with:
Burkitt’s Lymphoma
Lymphoproliferative Disease
Nasopharyngeal Carcinoma
How is EBV transmitted?
Oral contact - glandular fever is referred to as the kissing disease.
What possible feature of glandular fever is most concerning? What advice must be given to avoid any complications?
Hepatosplenomegaly (the splenomegaly in particular) - avoid contact sports.
How can we diagnose glandular fever (although it is largely clinically diagnosed)?
Atypical lymphocytes (numerous large T cells)
Positive monospot test - detects heterophil antibodies
Viral capsid antigen (VCA) IgG and IgM
Anti-EBNA antibodies
What antibiotics cause a florid maculopapular rash in children infected with EBV, and should therefore be avoided in patients with glandular fever?
Ampicillin and amoxicillin.
How is CMV usually transmitted?
NOTE: CMV is only concerning in immunocompromised patients, and can be passed from an immunocompromised mother to their foetus.
Saliva, genital secretion or breastmilk.
In a patient with a CMV mononucleosis-like syndrome, what will the test results show with regards to:
Atypical lymphocytes
Heterophile antibodies
Atypical lymphocytes - positive on blood film
Heterophile antibodies - negative
In an immunocompromised host, what problems can be caused by CMV? (7) How can it be treated if necessary (it’s usually self-limiting)?
Retinitis Pneumonitis Bone marrow failure Encephalitis Hepatitis Oesophagitis Enterocolitis
Treatment if necessary = IV ganciclovir, oral valganciclovir or foscarnet.
Which is more prevalent, HHV-6 or HHV-7?
HHV-6
What condition is classically caused by HHV-6 and HHV-7 infection? How does this present? How is this managed?
HHV-6 and HHV-7 cause exanthema subitem (aka roseola infantum)
Presentation:
High fever and malaise lasting a few days
Followed by generalised macular rash
Management:
Symptomatic
Explain risk of febrile seizures
What condition caused by human parvovirus B19? When do outbreaks commonly occur? How is it transmitted?
NOTE: It infects erythroblastoid red cell precursors.
Erythema infectiosum (aka fifth disease, aka slapped-cheek syndrome)
Most outbreaks occur in Spring, and it is spread by respiratory secretions, vertical transmission or infected blood products.
NOTE: It can also cause aplastic crises in children with chronic haemolytic anaemias (sickle cell/thalassaemia), as well as hydrops fetalis.
Give 3 examples of enteroviruses.
Coxsackie virus
Echoviruses
Polioviruses
How are enteroviruses primarily transmitted?
Faecal-oral and respiratory droplet routes.
What conditions can be caused by enteroviruses? (6) Give details of each.
Hand, foot and mouth disease (largely caused by coxsackie A16) - painful vesicular lesions on the hands, feet, mouth and tongue which resolve within days.
Herpangina (usually coxsackie A) - vesicular and ulcerated lesions on the soft palate and uvula, causing anorexia, painful swallowing and fever.
Meningitis/Encephalitis - enteroviruses are the most common cause of viral meningitis in developed countries.
Pleurodynia (Bornholm Disease) - acute fever, pleuritic chest pain and muscle tenderness which resolves within days.
Myocarditis and pericarditis - rare, but may present with chest pain and/or heart failure associated with febrile illness.
Enteroviral neonatal sepsis syndrome - rare, occurring in first few weeks of life, results from transplacental/intrapartum infection and mimics bacterial sepsis.
Which drugs can be used to treat enteroviruses?
We have no antivirals that are effective against enteroviruses and the use of IVIG is controversial.
What are Koplik’s spots?
White spots on the buccal mucosa seen against bright red background - they are pathognomonic of measles but are difficult to see.
Describe the rash seen in measles.
Maculopapular rash which becomes blotchy and confluent, and may desquamate in the second week. It begins begins the ears and spreads to the whole of the body.
Which 2 rare but serious conditions can measles lead to?
Encephalitis
Subacute Sclerosing Panencephalitis (SSPE) - occurs, on average, 7 years after measles infection and caused by a variant that persists in the CNS. Leads to loss of neurological function, progressing to dementia and death over several years.
Which body must be informed if a patient is diagnosed with measles, mumps or rubella?
The local Health Protection Unit (HPU).
What can be given if necessary to help immunocompromised patients to get over a primary measles infection?
Ribavirin
How is mumps spread? Which cells does the virus replicate in?
respiratory droplets
mumps virus replicates in epithelial cells
What is the incubation period of mumps? What are the clinical features?
Incubation period = 15-24 days
Clinical features:
Parotitis - may cause earache or pain eating/drinking
Fever
Malaise
Plasma amylase will be elevated due to parotid inflammation
Abdominal pain in the event of pancreatic involvement
NOTE: Mumps can be followed by epididymo-orchitis (unilateral, infertility is rare), meningitis or hearing loss (hearing loss is unilateral and transient).
How can mumps diagnosis be confirmed?
Oral swab
How is rubella spread? What is the incubation period?
Respiratory droplets
Incubation period of 15-20 days
How does rubella present? How can it be diagnosed? How is it managed?
Presentation:
Maculopapular rash (usually appearing on the face and spreading centrifugally to cover the whole body) lasting 3-5 days
Lymphadenopathy
Diagnosis:
Clinical, but can be confirmed by serology using oral fluid sample
Management:
Notify local HPU
Symptomatic relief - generally self-limiting
No effective antivirals exist
What are the possible complications of rubella (all are rare in children)? (4)
Arthritis
Encephalitis
Thrombocytopenia
Myocarditis
Kawasaki disease: Who does it most commonly affect? What are the cardinal features? What is the main concerning complication? How is it managed?
Most commonly affects children aged 6 months to 4 years, and is more common in Japanese children.
Cardinal Features = CRASH and Burn: Conjunctivitis Rash Adenopathy (usually cervical) Strawberry tongue Hand - swelling or erythema on hands/feet Burn - fever
NOTE: Patients usually have high inflammatory markers (CRP, ESR, WCC) with a platelet count that rises in the second week of illness.
Complication:
Coronary artery aneurysms can occur and cause narrowing of vessels due to scarring, leading to myocardial ischaemia and death.
Management:
IVIG
High-dose aspirin
Cardiovascular risk assessment
Wafarin long-term in patients with giant coronary artery aneurysms
Corticosteroids, infliximab or ciclosporin in patients with persistent inflammation and fever
How can TB be diagnosed in children?
Sputum samples can be used in children > 8 years old
For children < 8 years old, gastric washings (via NG tube) on 3 consecutive mornings can be used to identify TB using Ziehl-Neelsen or auramine stains and mycobacterial cultures
PCR-based methods can be used but provide limited information about drug-resistance
Tuberculin Skin Test - inject purified protein derivative intradermally into the forearm and observe 48-72 hours later to measure induration in millimetres - this will be positive in patients who have received the BCG vaccination
Interferon-Gamma Release Assays (IGRAs) - blood test which assessed the response of T cells to antigens expressed by M. tuberculosis but not BCG - negative result does not reliably rule out TB infection
In HIV patients, what condition can have a similar appearance to TB on CXR (it occurs in 20% of HIV-infected children)?
Lymphoid interstitial pneumonitis
What is the management of TB in children?
It is a notifiable disease.
Rifampicin + Isoniazid 6 months
Pyrazinamide + Ethambutol for the first 2 months
In adolescents - pyridoxine (vit B6) weekly to prevent peripheral neuropathy caused by isoniazid
NOTE: Treat even if asymptomatic to reduce risk of reactivation later in life.
NOTE 2: Children < 2 years old who have close contact with a sputum smear positive pulmonary TB patient should be started on prophylactic isoniazid.
Non-TB mycobacterial infections:
How are they spread?
What do they sometimes cause in children?
Who is particularly susceptible?
They spread by soil and water (no need to contact trace).
They cause persistent lymphadenitis which can be treated by complete lymph node excision or watchful waiting.
They can cause disseminated disease in immunocompromised individuals, and are also particularly common in patients with cystic fibrosis.
NOTE: Mycobacterium avium intracellulare is particularly common in HIV patients.
HIV transmission in children occurs from mother to child, and can occur via which 3 routes?
Intrauterine (during pregnancy)
Intrapartum (at delivery)
Postpartum (through breastfeeding)
How can HIV be diagnosed in children?
Children > 18 months: Detect antibodies against the virus
Children < 18 months will have transplacental maternal IgG HIV antibodies, so the most sensitive test is HIV RNA PCR
How can HIV be managed in children?
PCP prophylaxis with co-trimoxazole
Immunisations (except BCG)
MDT approach
What is the causative organism in Lyme disease? What are the clinical features? How is is diagnosed? How is it treated?
Causative organism = Borrelia burgdorferi
Clinical features:
Incubation period = 4-20 days
Erythema migrans at site of tick bite
Early features = fever, headache, malaise, myalgia, arthralgia, lymphadenopathy
Late features (weeks/months after infection) = neurological, cardiac and joint conditions)
Diagnosis:
Serology and clinical features
If erythema migrans is not seen, perform ELISA
If ELISA is positive, perform immunoblot test
Treatment: 1st line = doxycycline 2nd line = amoxicillin 3rd line = azithromycin IV ceftriaxone is used for carditis or neurological disease
NOTE: Some people have a Jarish-Herxheimer reaction -antibiotics cause lysis of bacterial membranes leading to the release of toxins causing symptoms to worsen.
What is the newborn vaccination schedule?
Find image
How are most immunodeficiencies inherited?
X-linked recessive or autosomal recessive
How can immunodeficiencies be managed in children? (6)
Antimicrobial prophylaxis:
- For T cell/neutrophil defects, co-trimoxazole can prevent PCP and itraconazole or fluconazole can prevent fungal infections
- For B cell defects antibiotic prophylaxis can prevent bacterial infections
Antibiotic treatment
Screening for end-organ disease
Immunoglobulin replacement therapy - for antibody deficiency
Bone marrow transplantation - e.g. for SCID
Gene therapy
Describe the clinical course of IgE mediated allergic reactions.
Early phase:
Occurs within minutes of exposure to allergen
Caused by histamine release and other mast cell mediators
Cause urticaria, angioedema, sneezing, vomiting, bronchospasm and/or cardiovascular shock
Late phase:
May occur 4-6 hours after exposure to allergen
Especially common with inhalant allergens
Causes nasal congestion in the upper airway, cough and bronchospasm in the lower airway
NOTE: Most severe life-threatening allergic reactions are IgE mediated, and non-IgE allergic responses have a delayed onset of symptoms and varied clinical course.
Define hypersensitivity.
Objectively reproducible symptoms or signs following exposure to a defined stimulus at a dose that is usually tolerated by most people.
Define allergy.
A hypersensitivity reaction initiated by specific immunological mechanisms. This can be IgE mediated or non-IgE mediated (e.g. Coeliac disease).
Define atopy.
A personal and/or familial tendency to produce IgE antibodies in response to ordinary exposures to potential allergens, usually proteins. Strongly associated with asthma, allergic rhinitis and conjunctivitis, eczema and food allergy.
Define anaphylaxis.
A serious allergic reaction with bronchial, laryngeal or cardiovascular involvement that is rapid in onset and may cause death.
What does the hygiene hypothesis of allergy state?
That the increased prevalence of allergy in many countries is due to altered microbial exposure associated with modern living conditions.
In the allergic march, which conditions are children like to get at which stages of life?
Infancy:
Eczema
Food allergy
Primary School years:
Allergic rhinitis
Conjunctivitis
Asthma
What features might you find in a child who is having an allergic reaction (not anaphylaxis)? (3)
Mouth breathing
Allergic salute
Painful or swollen inferior nasal turbinates
In allergic conjunctivitis, you may see blue-grey discolouration below the lower eyelids. You may also see prominent creases - what are these called?
Dennie-Morgan folds.
How do we perform specific allergen immunotherapy?
SC or sublingual injections of solutions of an allergic are given to the patient on a regular basis over 3-5 years, with the aim of developing immune tolerance.
What is a food intolerance?
A non-immunological hypersensitivity reaction to a specific food.
Infants tend to present with different food allergies to older children - which allergies do infants get? (3) Which do older children get? (4)
Infants = Milk, eggs and peanut
Older children = Peanut, tree nut, fish, shellfish
What is pollen food allergy syndrome?
When cross-reactivity of proteins present in fresh fruit/vegetables/nuts occurs with those present in pollens - someone becomes allergic to a pollen that has a very similar protein to that found in a fruit/vegetable/nut, so they also become allergic to that food.
Is an IgE or a non-IgE mediated allergic reaction more likely to present with GI symptoms?
Non-IgE allergic reactions often present with GI symptoms such as diarrhoea, vomiting, abdominal pain and sometimes faltering growth.
What is food protein-induced enterocolitis syndrome?
Where repetitive vomiting due to non-IgE mediated allergies (usually to food protein) results in shock.
How can we diagnose food allergies?
IgE mediated allergies:
Skin prick tests
Patch testing
Measurement of specific IgE antibodies in the blood
Non-IgE:
Harder to diagnose
Endoscopy and intestinal biopsy can be performed - eosinophilic infiltrates
For both:
GOLD STANDARD is exclusion of the relevant food
Cow's milk protein allergy: Definition Classification Presentation Management
Definition = abnormal reaction by the body’s immune system to a protein found in cows’ milk.
Classification = IgE-mediated (immediate) or non-IgE mediated (delayed).
Presentation:
GI symptoms (non-specific)
Skin symptoms - hives and eczema
Babies - wheezing, irritability, facial swelling, poor growth
Management:
Strict cows’ milk elimination from diet for minimum 6 months or until child is 9-12 months old
May require removal of cows’ milk from mother’s diet or change of formula milk
Regularly monitor growth
Re-evaluate tolerance to cows’ milk at later date
How can allergic rhinitis and conjunctivitis be classified?
Based on pattern, severity and seasonality:
Pattern = intermittent or persistent
Severity = mild, moderate or severe
Seasonality = seasonal or perennial
How can we manage allergic rhinitis and conjunctivitis?
For ‘as required’ treatment for occasional symptoms:
If aged 2-5 or preference for oral treatment = oral antihistamine
All others = intranasal azelastine (antihistamine)
For those who want preventative treatment for frequent symptoms:
If main issue is nasal blockage/polyps = intranasal corticosteroid (e.g. beclomethasone)
If main issue is sneezing/nasal discharge = antihistamine or intranasal corticosteroid
For people requiring rapid relief whilst waiting for preventative treatment to take effect:
Intranasal corticosteroids for up to 7 days
Consider adding oral antihistamine
If symptoms are severe, prescribe 5-10 day course of prednisolone
Urticaria and angioedema present with itchy hives or redness, what is the causative mechanism?
Local vasodilation and increased permeability of capillaries and venues, due to activation of skin mast cells and subsequent release of various mediators including histamines.
Chronic urticaria is usually non-allergic. How long does urticaria have to be present to be classed as chronic?
> 6 weeks
How can we assess the severity of urticaria? How can we treat it?
Severity can be assessed with tools such as the Urticaria activity score (UAS7).
Treatment (it’s usually self-limiting):
Offer non-sedating antihistamine (e.g. cetirizine) for up to 6 weeks
If severe, offer oral corticosteroid (short course)
In refractory cases, leukotriene receptor antagonists or anti-IgE antibody (omalizumab) can be used
What are the generic upper respiratory tract symptoms seen in children with respiratory disorders? (5)
Coryza, sore throat, earache, sinusitis, stridor
What are the generic lower respiratory tract symptoms seen in children with respiratory disorders? (3)
Cough, wheeze, respiratory distress
Give 4 groups of children who are at high risk of respiratory failure.
Ex-preterm infants with bronchopulmonary dysplasia
Haemodynamically significant congenital heart disease or disorders causing muscle weakness
Cystic fibrosis
Immunodeficiency
How can you differentiate between stridor and wheeze?
Stridor:
Caused by extrathoracic airway narrowing
Predominantly inspiratory
Wheeze:
Caused by intrathoracic airway narrowing
Predominantly expiratory
Extrathoracic airway obstruction is worse during inspiration, while intrathoracic obstruction is worse during expiration.
Is snoring inspiratory or expiratory? What causes it?
Snoring is inspiratory. It is caused by variable partial upper airway obstruction so it causes a rough inspiratory noise (stertor).
Give 4 conditions that are classed as URTIs.
Common cold (coryza)
Sore throat (pharyngitis, including tonsillitis)
Acute otitis media
Sinusitis
What are the most common causative organisms in the common cold? (3)
Rhinoviruses
Coronaviruses
Respiratory syncytial virus (RSV)
Give 2 common causative organisms in tonsillitis.
Group A beta-haemolytic streptococci
EBV
How is bacterial tonsillitis treated?
Phenoxymethylpenicillin
If penicillin allergic - Clarithromycin
Treatment is required for 10 days, and amoxicillin should be avoided as it can cause a widespread maculopapular rash if the tonsillitis is caused by glandular fever.
NOTE: Watch out for tonsillitis in patients on DMARDs or carbimazole - DMARDs can cause immunodeficiency and carbimazole can cause idiosyncratic neutropenia.
What is the causative organism in scarlet fever? How does it present? How is it treated? What are the complications?
NOTE: Scarlet fever is a notifiable disease.
Causative organism = group A streptococcus
Symptoms = fever, headache, tonsillitis, ‘sandpaper-like’ maculopapular rash with flushed cheeks and perioral sparing, tongue is often white and coated and may be sore
Treatment: (symptoms tend to settle after 1 week)
Antibiotics - phenoxymethylpenicillin 4x/day for 10 days (alternative = azithromycin)
Avoid school for 24 hours after starting antibiotics
Complications:
Suppurative - otitis media, throat infection, sinusitis, strep pneumonia, meningitis, endocarditis, osteomyelitis, necrotising fasciitis, toxic shock syndrome
Non-suppurative (autoimmune) - rheumatic fever, step glomerulonephritis
Acute otitis media: Which age group is it most common in? How would the tympanic membrane appear? List 5 causative organisms. Give 3 complications. How long does it usually last? What is the medical management?
Most common in: 6-12 month olds.
Tympanic membrane: bright red, bulging, loss of normal light reflection.
Causative organisms: RSV, rhinovirus, pneumococcus, H. influenzae, M. catarrhalis.
Complications: tympanic membrane perforation, mastoiditis, meningitis.
How long: usually improved after 3 days but can last 1 week.
Management: Most cases do not require antibiotics but in more severe cases (rapid deterioration or systemic illness) give amoxicillin 5-7 days (alternative clarithromycin/erythromycin)
Otitis media with effusion (aka glue ear):
Symptoms
What age group is it most common in?
How can it be treated?
Symptoms: often asymptomatic but may cause conductive hearing loss (most common cause in children).
Most common in: 2-7 year olds
Treatment: Ventilation tubes (grommets) - the benefits don’t last longer than 1 year. If problems recur after grommets are removed, reinsertion with adjuvant adenoidectomy is advocated.
Is sinusitis usually viral or bacterial?
Viral (though it rarely occurs secondary to bacterial infection)
The paranasal sinuses are most commonly effected in sinusitis - why are the frontal sinuses rarely affected in children?
They do not develop until late childhood.
In patients whose sinusitis symptoms have lasted > 10 days, what treatments should be considered?
High-dose nasal corticosteroid (symptomatic)
Antibiotics (usually not given, as they are unlikely to change the course of the illness) - phenoxymethylpenicillin (alternative - clarithromycin)
At what age do both the tonsils and adenoids reach maximum size? They shrink later in childhood.
8 years old.
Give 2 indications for tonsillectomy alone, and 2 for tonsillectomy with adjuvant adenoidectomy.
Tonsillectomy alone:
Recurrent severe tonsillitis
Peritonsilar abscess (quinsy)
Tonsillectomy with adenoidectomy:
Recurrent otitis media with effusion (glue ear) with hearing loss
Obstructive sleep apnoea
Stridor:
Definition
Most common cause in children
How can severity be assessed?
Definition: A harsh, musical sound caused by partial obstruction of the lower portion of the upper airway.
Most common cause: Croup.
Severity:
Can be assessed by when it occurs - no stridor, only on crying, at rest, biphasic
Can also be assessed by degree of chest retraction - none, only on crying, at rest
Why should you avoid examining the throat of a child with suspected upper airway obstruction?
Examination of the throat using a spatula may precipitate total obstruction of the upper airway.
NOTE: You can attempt examination if full resuscitation equipment and personnel are on hand.
Viral croup account for what proportion of laryngotracheal infections in children?
95%
What is the most common cause of croup? Give 3 other causative organisms.
Most common = parainfluenza virus
Other:
Rhinovirus
RSV
Influenza
Croup affects children of what ages?
6 months to 6 years old (peak incidence 1-2 years old).
What are the clinical features of croup? What is the treatment?
NOTE: Admit all children with croup that is worse than mild.
Coryzal symptoms
Hoarseness due to inflammation of the vocal cords
Barking cough (‘like a sea lion’) due to tracheal oedema and collapse
Harsh stridor
Variable degree of breathing difficulty with chest retraction
Symptoms worse at night
Treatment:
- If admission is not required: single dose of oral dexamethasone (0.15mg/kg)
- Advise that it usually resolves within 48 hours
- Croup causing chest recession at rest: oral dexamethasone OR oral prednisolone OR nebuliser steroids (budesonide)
- Severe: nebuliser adrenaline with oxygen by face mask
Acute epiglottitis is a life-threatening condition due to the risk of respiratory obstruction, and is associated with septicaemia. What is it caused by? What age group is most commonly affected? What are the clinical features?
Causative organism: H. influenzae type B (Hib)
Age group: 1-6 years old.
Clinical features:
High fever in very ill looking child
Intensely painful throat that prevents speaking/swalling -drooling saliva due to this
Soft inspiratory stridor and rapidly increasing respiratory difficulty
Child is sitting immobile, upright, with an open mouth to optimise the airway
What 3 actions should you avoid in a child with suspected acute epiglottitis, as they may precipitate total airway obstruction?
Lying the child down
Examining the throat with a spatula
Performating a lateral neck xray
What is one key difference in the presentation of croup vs acute epiglottitis?
Cough is not a feature of epiglottitis.
What is the management of acute epiglottitis?
Urgent admission and treatment in ITU Secure airway and give supplemental oxygen Take blood culture Start IV antibiotics (e.g. cefuroxime) Consider steroids and adrenaline
What can be given to close contacts of a patient with acute epiglottitis, as prophylaxis?
Rifampicin
How does bacterial tracheitis (pseudomembranous croup) present? What is the causative organism? How is it managed?
Presentation: It is rare but dangerous, and presents similarly to acute epiglottitis - high fever, rapidly progressive airway obstruction with copious thick airway secretions.
Causative organism: S. aureus.
Management: IV antibiotics, consider intubation and ventilation.
What are the 2 possible causative mechanisms in a patient with wheeze?
Mucosal inflammation (e.g. bronchiolitis) Bronchoconstriction (e.g. asthma, mechanical obstruction)
What is the most common serious respiratory infection of infancy?
Bronchiolitis.
Bronchiolitis:
What age group does it affect?
What is the most common causative organism?
Who is at highest risk?
Age group: 1-9 months (90% of cases).
Causative organism: RSV (80% of cases) - others include parainfluenza, rhinovirus, adenovirus, influenza and human metapneumovirus.
High risk: Infants born prematurely who develop bronchopulmonary dysplasia or those with underlying lung or congenital heart disease.
Bronchiolitis:
Examination findings (6)
Management
What can be given to at-risk patients as prophylaxis?
Examination findings: Dry wheezy cough Tachypnoea/tachycardia Subcostal and intercostal recession Hyperinflation Fine end-inspiratory crackles High-pitched wheezes (usually expiratory)
Management: Humidified oxygen if sats are below 92% Consider CPAP Consider upper airway suction if there is evidence of increased secretions Fluids (NG tube)
NOTE: Most patients recover within 2 weeks.
Prophylaxis: Palivizumab (monoclonal antibody against RSV).
What is it called when bronchiolitis causes permanent airway damage?
Bronchiolitis obliterans.
What condition do most wheezy preschool children have?
Viral episodic wheeze.
Give 2 risk factors for viral episodic wheeze.
Maternal smoking during and/or after pregnancy
Prematurity
What is the prognosis for patients with viral episodic wheeze?
Usually resolves after about 5 years (probably due to increased airway size).
What is the management of viral episodic wheeze?
Salbutamol inhaler (with spacer)
Encourage parents to stop smoking (if applicable)
Dosing - when the child is wheezy/breathless, give up to 10 puffs of salbutamol up to every 4 hours
If salbutamol is not effective, intermittent LTRA or intermittent ICS or both may be considered
How do we determine whether someone’s asthma is atopic?
It is classified as atopic asthma if wheezing is experience between viral infections (interval symptoms) and if there is evidence of allergy to one or more inhaled allergens.
What may you hear upon auscultation in a patient with long-standing asthma?
Generalised polyphonic expiratory wheeze.
NOTE: Between attacks, auscultation in patients with asthma is usually normal.
What are Harrison sulci?
Depressions at the base of the thorax associated with the muscular insertion of the diaphragm - they are associated with chronic obstructive airways disease such as asthma in childhood.
How can asthma be investigated in a child?
< 5 years old = clinical diagnosis.
> 5 years old:
Spirometry - FEV1/FVC <70% of expected is a positive result
Bronchodilator reversibility - consider in patients with positive spirometry (improvement in FEV1 of >12% is positive)
Peak expiratory flow variability - monitor for 2-4 weeks
FeNo Test - consider in cases of diagnostic uncertainty (>35ppb is a positive test)
How is asthma managed in patients < 5 years old?
Step 1: SABA as reliever therapy (e.g. salbutamol)
Step 2: Consider 8 week trial of paediatric moderate dose ICS
Step 3: After 8 weeks, stop ICS treatment and continue to monitor symptoms - if symptoms resolved but recurred within 4 weeks, restart ICS at low dose. If recur after > 4 weeks, repeat 8 week ICS trial
Step 4: Consider adding an LTRA (e.g. montelukast)
Step 5: Stop LTRA and refer to child respiratory specialist
How is asthma managed in patients > 5 years old?
Step 1: SABA as reliever (e.g. salbutamol)
Step 2: Paediatric low dose ICS
Step 3: Consider adding LTRA - review in 4-8 weeks
Step 4: Consider stopping LTRA and starting LABA
Step 5: Consider changing ICS and LABA therapy to a MART regimen, with paediatric low maintenance ICS dose
Step 6: Consider increasing ICS to moderate maintenance dose
Step 7: Refer to specialist
Give 2 examples of SABAs.
How long do their effects last?
Salbutamol
Terbutaline
They are effective for 2-4 hours.
Give 2 examples of LABAs. How long do their effects last? You should not prescribe LABAs without which other class of drugs?
Salmeterol
Formoterol
They are effective for 12 hours.
Do not prescribe LABAs within an inhaled corticosteroid (ICS).
What class of drug is ipratropium bromide?
Anticholinergic bronchodilator.
What are the possible side-effects of inhaled corticosteroids? (3)
Mild reduction in height velocity - Impaired growth
Adrenal suppression
Altered bone metabolism
How would you manage a child with severe/life-threatening asthma whilst awaiting admission to hospital?
Supplemental oxygen - use Venturi mask or nasal cannula and maintain 94-98% sats
Treat with SABA - nebulised salbutamol can be used in life-threatening asthma
If SABA was ineffective, consider nebulised tiotropium bromide
What is indicated by:
Dry cough (with prolonged expiratory phase)
Barking cough
Moist cough
Dry cough - narrowing of small-to-moderate sized airways
Barking cough - tracheal inflammation
Moist cough - Either increased mucus secretion or infection in the lower airway
Whooping cough (pertussis): Causative organism Presentation Complications (3) Investigations Management
Causative organism: Bordatella pertussis
Presentation:
1 week of coryza (catarrhal phase)
Followed by development of paroxysmal or spasmodic cough, which is followed by an inspiratory whoop
Cough is worse at night and may cause vomiting
During paroxysm, child may go red/blue in face and mucus may run from nose/mouth
Cough lasts up to 3 months
Symptoms eventually decrease (convalescent phase)
Complications:
Pneumonia
Seizures
Bronchiectasis
Investigations: Culture of perinasal swab PCR is more sensitive Serology There is marked lymphocytosis
Management:
If admission is not needed, and cough started within 21 days, give a macrolide (< 1 month = clarithromycin, 1+ months = azithromycin, pregnant = erythromycin)
(immunisation is used as prevention)
In younger children, viruses are the most common cause of pneumonia. In older children, bacteria are the most common cause of pneumonia.
What are the most common pathogens causing pneumonia in:
Newborns
Infants and young children (< 5 years old)
Children > 5 years old
Newborns: group B streptococcus
Infants and young children: RSV, S. pneumoniae, H. influenzae
(S. aureus is a rare but serious cause)
Children > 5 years old: M. pneumoniae, S. pneumoniae, C. pneumoniae
NOTE: Always consider M. tuberculosis.
What is the treatment of pneumonia in children, where hospital admission is not required?
Amoxicillin 7-14 days
Alternative = co-amoxiclav, cefaclor, macrlolides
What is persistent bacterial bronchitis? Give 2 common causative organisms.
Where there is persistent inflammation of the lower airways due to chronic infection - causes wet cough in children. Thought to be a precursor to bronchiectasis.
Causes:
H. influenzae
M. Catarrhalis
List 4 causes of generalised bronchiectasis, and 3 causes of focal bronchiectasis.
Generalised bronchiectasis: Cystic fibrosis Primary ciliary dyskinesia Immunodeficiency Chronic aspiration
Focal bronchiectasis:
Previous severe pneumonia
Congenital lung abnormality
Obstruction by a foreign body
How can bronchiectasis be managed?
Exercise and nutrition
Airway clearance therapy
Inhaled bronchodilator (e.g. salbutamol)
Inhaled hyperosmolar agent (e.g. hypertonic saline)
Long-term oral macrolide (azithromycin)
Lung transplantation or resection of broncheictatic areas
Cystic fibrosis: Inheritance pattern Average life expectancy Causative mutation Pathophysiology
Inheritance pattern: Autosomal recessive.
Average life-expectancy: 40 years.
Causative mutation: (most commonly) Delta-F508 mutation In CFTR (a cAMP-dependent chloride channel on the membrane of epithelial cells).
Pathophysiology: Abnormal ion transport leads to impaired ciliary function and retention of mucopurulent secretions - this can lead to infection with P. aeruginosa, meconium ileus (10-20% of infants), pancreatic enzyme deficiency (due to duct blockage) and excessive sodium and chloride concentrations in the sweat.
What is measured in the newborn heelprick test to look for cystic fibrosis?
Immunoreactive trypsinogen (IRT) - it will be raised in infants with CF.
How can pancreatic insufficiency be diagnosed in patients with cystic fibrosis?
They will have low faecal elastase.
What range of chloride in sweat would be indicative of cystic fibrosis?
60-125 mmol/L (normal is 10-40 mmol/L in children)
What are the components of respiratory management in patients with cystic fibrosis? (4)
Pulmonary monitoring (every 8 weeks in children)
Airway clearance techniques (to be done twice per day)
Mucoactive agents (first line: rhDNase)
New agents: Lumacaftor and ivacaftor (in patients with F508 mutation)
What are the components of nutritional management in patients with cystic fibrosis? (2)
Oral enteric-coated pancreatic replacement therapy
High-calorie diet
What are the components of infection management in patients with cystic fibrosis? (2)
Continuous prophylactic antibiotics (usually flucloxacillin)
Regular azithromycin decreases respiratory exacerbations
NOTE: Bilateral sequential lung transplantation is the only option for end-stage CF lung disease.
Distal intestinal obstruction syndrome is a complication of what condition?
Cystic fibrosis.
Primary ciliary dyskinesia:
What is it?
How does it present?
NOTE: 50% of patients with PCD will have Kartagener’s syndrome.
Congenital abnormality in the structure or function of cilia lining the respiratory tract, resulting in impaired mucociliary clearance.
Causes recurrent infections and may present with recurrent productive cough, purulent nasal discharge and chronic ear infections. It can progress to severe bronchiectasis.
Which types of immunodeficiency predispose to which lung infections?
IgG deficiency - polysaccharide-capsulated bacteria (e.g. S. pneumoniae)
Cell-mediated immunodeficiencies - opportunistic infections (e.g. PCP)
Neutrophil killing defects - Staphylococcus
Give 5 conditions that are associated with sleep-disordered breathing (OSA).
Neuromuscular disease (e.g. Duchenne)
Craniofacial abnormalities (e.g. Pierre Robin sequence)
Dystonia of upper airway muscles (e.g. cerebral palsy)
Severe obesity
Down’s syndrome
What is the name of the chalky-white greasy coat seen on a baby at birth?
Vernix caseosa.
What is the skin of a preterm infant like, in comparison to a term infant?
Thin, poorly keratinised and transepidermal water loss is markedly increased.
NOTE: Thermoregulation is also impaired as the preterm infant lacks subcutaneous fat and unable to sweat until a few weeks old.
Bullous impetigo:
Most common causative organism
Treatment
S. aureus Systemic antibiotics (e.g. flucloxacillin)
What is the medical term for moles?
Melanocytic naevi.
Give 5 risk factors for melanoma.
Family history
Large numbers of melanocytic naevi (moles)
Fair skin
Repeated episodes of sunburn
Living in a hot climate with repeated sun exposure
Albinism:
What causes it?
How is it categorised?
What ocular impairments is it associated with?
Caused by defect in biosynthesis and distribution of melanin.
Can be categorised as:
Oculocutaneous
Ocular
Partial
Lack of pigment in the iris, retina, eyelids and eyebrows results in failure to develop a fixation reflex. It also causes pendular nystagmus.
What complications can occur in severe case of epidermolysis bullosa? (3)
Fingers and toes can become fused
Contractors of the limbs may develop from repeated blistering and healing
Mucous membrane involvement may result in oral ulceration and stenosis form oesophageal erosions
What is collodion?
What is the main risk?
A rare manifestation of the inherited ichthyoses where infants are born with taut, shiny, parchment-like skin.
There is a risk of dehydration.
List 4 common and 3 uncommon causes of nappy rash.
Common: Irritant (contact) dermatitis - most common cause Infantile seborrheic dermatitis Candida infection Atopic eczema
Rare:
Acrodermatitis enteropathica
Langerhans cell histiocytosis
Wiskott-Aldrich syndrome
NOTE: Irritant dermatitis is usually due to the irritant effects of urine on the skin, and flexures tend to be spared.
How can you differentiate between nappy rash caused by irritant dermatitis and nappy rash caused by satellite lesions?
Nappy rash caused by satellite lesions and does affect the flexures. It is treated with topical antifungals (imidazole).
When does infantile seborrheic dermatitis usually present, and where does it tend to start?
presents in the first 3 months of life and tends to start on the scalp (cradle cap). It is NOT itchy.
NOTE: You may consider using topical imidazole if conservative measures are not effective at treating.
What are the most common causes of skin infection in patients with atopic eczema? (2)
Staphylococcus and streptococcus infections.
NOTE: Herpes simplex virus can cause eczema herpeticum which is potentially serious (treat with aciclovir).
Name a questionnaire that can be used to assess the psychological impact of dermatological conditions on children.
The Children’s Dermatology Life Quality Index (CDLQI).
What medications might you prescribe for mild, moderate, severe and infected eczema?
All: Emollients.
Mild: 1% hydrocortisone.
Moderate:
0.025% betamethasone validate or 0.05% clobetasone butyrate
Consider non-sedating antihistamine (if itchy) e.g. cetirizine
2nd line = topical calcineurin inhibitor e.g. tacrolimus
Severe:
0.1% betamethasone
Consider non-sedating antihistamine (or sedating antihistamine if sleep is affected by itching)
Consider oral corticosteroids in very severe cases
Infected:
Swab infected skin
1st line = flucloxacillin (use erythromycin if allergic) no longer than 2 weeks
What are the most common allergens resulting in eczema?
Egg
Cow’s milk
Warts:
What causes them?
Where are they usually found?
How can we treat them?
Cause: HPV Found on: Fingers and soles (verrucae) Treatments: 1. Daily administration of salicylic acid or lactic acid or glutaraldehyde lotion 2. Cryotherapy with liquid nitrogen
Molluscum contagiosum:
What is it caused by?
What do the lesions look like?
Caused by a poxvirus.
Lesions are small, skin-coloured, pearly papule with central umbilication.
Spontaneously resolves within 18 months.
Ringworm: Cause Appearance How can we make a rapid diagnosis? How can we make a definitive diagnosis?
Cause: Dematophyte fungi invade dead keratinous structures such as the horny layer of the skin, nails and hair.
Appearance: Annular lesion - a severely inflamed pustular patch is called a kerion.
Rapid diagnosis: Examination of skin scrapings for fungal hyphae.
Definitive diagnosis: Culture the fungus.
What is the treatment of:
Tinea capitis
Tinia faciale/corporis/crurus/pedis
Capitis:
Systemic anti fungal therapy with griseofulvin or terbinafine
2nd line = itraconazole or fluconazole
(Consider topical antifungal shampoo)
Other forms of tinea:
Topical antifungals (e.g. terbinafine, naftifine, butenafine)
Topical aluminium acetate
Scabies: Cause At what stage does itching occur? What makes itching worse? Treatment
Cause: Sarcoptes scabiei mite.
Itching: Occurs 2-6 weeks after infestation. Is worse in warm conditions and at night.
Treatment = topical insecticide:
1st line = permethrin 5% cream.
2nd line = malathion aqueous 0.5%.
Apply all over body from chin and ears downwards, and wash off after 8-12 hours (permethrin) or 24 hours (malathion)
Apply for a second time, one week after first application
What can we sue to treat post-scabietic itch?
Crotamiton 10% cream (or topical hydrocortisone).
What is pediculosis capitis?
How is it managed?
Head lice.
Management:
Dimeticone 4% lotion or aqueous solution of malathion 0.5% rubbed into scalp and left overnight before washing hair in the morning.
Repeat one week later.
Wet combing with a fine-tooth comb can remove live lice every 3-4 days for 2 weeks.
Psoriasis: What age does it usually present? Describe the lesions What is the most common form in children? How is it treated?
Age: rarely presents below 2 years old.
Lesions: Small, raindrop-like, round or oval erythematous scaly patches on trunk and upper limbs.
Most common in children: guttate - often follows streptococcal or viral sore throat or ear infection.
Treatment of guttate psoriasis: bland ointments.
NOTE: Plaque psoriasis can be treated with coal-tar preparations, dithranol or calcipotriol.
Pityriasis rosea:
How does it usually begin?
What pattern does it spread in?
Treatment
Begins with herald patch - a single round scaly macule, usually 2-5m in diameter on the trunk, upper arm, neck or thigh.
Pattern of spread - follows the line of the ribs posteriorly (fir tree pattern).
No treatment required - resolves within 4-6 weeks.
What form of alopecia is common in children?
Alopecia areata.
Describe the lesions seen in granuloma annulare.
Annular, with raised flesh-coloured non-scaling edge (usually found over bony prominences, especially on the hands and feet).
What are open comodones and what are closed comedones?
Open comedones = blackheads
Closed comedones = whiteheads
NOTE: These can progress to papule, pustules, nodules and cysts.
Acne vulgaris - medical management:
Mild to moderate acne - topical treatment:
Benzoyl peroxide
Benzoyl peroxide + clindamycin (Duac)
Adapaline (topical retinoid) - contraindicated in breastfeeding and pregnancy
Azelaic acid
Moderate acne not responding to topical treatments:
Consider adding oral antibiotics (lymecycline or doxycycline) for maximum 3 months
Change to alternative antibiotic if not successful
Severe acne or non-responsive acne:
Refer to specialist for treatment with isotretinoin (roaccutane)
NOTE: COCP in combination with topical agents can be used as an alternative to systemic antibiotics in girls.
What are the 2 severe variants of acne?
Acne conglobata
Acne fulminans
What is hereditary angioedema?
A rare autosomal dominant disorder caused by deficiency or dysfunction of C1-esterase inhibitor.
There is no urticaria, but subcutaneous swellings occur, often accompanied with abdominal pain.
Treated with a purified preparation of the inhibitor.
What is the main presenting complaint in patients with left-to-right cardiac shunts? Give 3 defects that cause left-to-right shunting.
PC: Breathlessness
Examples:
Ventricular septal defect
Persistent ductus arteriosus
Atrial septal defect
What is the main presenting complaint in patients with right-to-left cardiac shunts? Give 2 defects that cause right-to-left shunting.
PC: Cyanosis (blue)
Examples:
Tetralogy of Fallot
Transposition of the great arteries
What cardiac condition can present with breathlessness and cyanosis, caused by mixed shunting of blood?
Atrioventricular septal defect (complete)
What 2 conditions can cause outflow obstruction in a well child? How do they present?
Conditions:
Pulmonary stenosis
Aortic stenosis
Presentation:
Asymptomatic with a murmur
What condition causes outflow obstruction in a sick neonate? It presents with collapse caused by shock.
Coarctation of the aorta.
When does the ductus arteriosus close?
Within the first few hours or days of life.
At what stage in pregnancy is a routine foetal anomaly scan performed?
18-20 weeks - it leads to diagnosis of 70% of infants requiring surgery in the first 6 months.
Innocent murmurs:
What percentage of children have them?
What are the 4 hallmarks?
Why are they generally heard best during a febrile illness or anaemia?
Percentage: 30%
Hallmarks: Asymptomatic Soft blowing murmur Systolic murmur only, not diastolic Left sternal edge
They are generally heard best during febrile illness or anaemia due to increased cardiac output.
Why might conditions that cause murmurs not present until several weeks of age?
Because pulmonary vascular resistance is still high - this falls over the first weeks of life, so conditions such as VSD and PDA may present then.
What are the signs (7) and symptoms (4) of heart failure?
Signs: Poor weight gain or faltering growth Tachypnoea Tachycardia Heart murmur, gallop rhythm Cardiomegaly Hepatomegaly Cool peripheries
Symptoms: Breathlessness Sweating Poor feeding Recurrent chest infections
What are the causes of heart failure in:
Neonates (4)
Infants (3)
Older children/adolescents (3)
Neonates - obstructed (duct-dependent) systemic circulation: Hypoplastic left heart syndrome Critical aortic valve stenosis Severe coarctation of the aorta Interruption of the aortic arch
Infants - high pulmonary blood flow:
VSD
Atrioventricular septal defect
Large persistent ductus arteriosus
Older children and adolescents - right or left heart failure:
Eisenmenger syndrome (RHF only)
Rheumatic heart disease
Cardiomyopathy
In the first week of life, heart failure tends to result from ______ _______ _______ (e.g. coarctation of the aorta).
After the first week of life, progressive heart failure is most likely due to ____-__-____ ______.
Left heart obstruction
Left-to-right shunt
What is Eisenmenger syndrome? How is it treated?
A process in neonates in which long-standing left-to-right shunting of blood causes pulmonary hypertension and eventual reversal of the shunt into a cyanotic right-to-left shunt - this causes cyanosis.
The only treatment option is heart-lung transplant.
Heart failure management - what do we do in order to:
- Reduce preload
- Enhance cardiac contractility
- Reduce afterload
- Improve oxygen delivery
NOTE: In duct-dependent cyanotic heart failure caused by a cardiac malformation, we can give prostaglandin infusion to maintain the PDA and buy time for surgical correction.
Reduce preload:
Diuretics (e.g. furosemide)
(More rarely) venous dilators (e.g. nitroglycerin)
Enhance cardiac contractility:
IV agents such as dopamine
Other options: Digoxin, dobutamine, adrenaline, milrinone
Reduce afterload:
Oral ACE inhibitors
IV agents such as hydralazine, nitroprusside and alprostadil
Improve oxygen delivery:
Beta-blockers (e.g. carvedilol)
What is considered a normal oxygen saturation in infants?
> 94%.
What are the causes of cyanosis in a newborn infant with respiratory distress (respiratory rate > 60)?
Congenital cyanotic heart disease
Respiratory disorders (e.g. respiratory distress syndrome meconium aspiration, pulmonary hypoplasia)
Persistent pulmonary hypertension of the newborn (failure of pulmonary vascular resistance to fall after birth)
Infection (e.g. septicaemia)
Inborn errors of metabolism (metabolic acidosis and shock)
What are the 2 main types of atrial septal defect?
Secundum ASD (80%) - defect in the centre of the atrial septum, involving the foramen ovale.
Partial AVSD - defect in the atrioventricular septum:
- Primum ASD - There is an interatrial communication between the bottom end of the atrial septum and the atrioventricular valves
- Regurgitant valve - there are abnormal AV valves, with a left AV valve which has 3 leaflets and tends to lead
What are the signs and symptoms of atrial septal defects?
Symptoms:
None (common)
Recurrent chest infections/wheeze
Arrhythmias (4th decade onwards)
Signs:
Ejection systolic murmur (best heard on upper left sternal edge) - due to increased flow across the pulmonary valve because of left-to-right shunt
Fixed and widely split second heart sound - due to right ventricular stroke volume being equal in both inspiration and expiration
Apical pansytolic murmur (form AV valve regurgitation) - only heard in partial AVSD
What may be seen on a CXR of a child with an ASD?
Cardiomegaly
Enlarged pulmonary arteries
Increased pulmonary vascular markings
What would you find on the ECG of a patient with secundum ASD? (2)
Partial RBBB and right axis deviation (due to right ventricular enlargement).
What would you find on the ECG of a patient with partial AVSD?
Superior QRS - this occurs due to a defect in the middle of the heart where the AV node is found. This displaced node then conducts to the ventricles superiorly, resulting in an abnormal axis.
How do we manage:
Secundum ASD
Partial AVSD
Secundum ASD - percutaneous close/endovascular closure
Partial AVSD - surgical correction
VSDs make up what proportion of cases of congenital heart disease?
30%
How are VSDs categorised?
Based on size:
Small = smaller than the aortic valve (usually < 3mm diameter)
Large = larger than aortic valve
Small VSDs are often asymptomatic - what are the signs of small VSDs?
NOTE: These close spontaneously.
Loud pansystolic murmur at lower left sternal edge (loud murmur implies smaller defect)
Quiet pulmonary second sound (P2)
What are the signs and symptoms of large VSDs?
Signs: Tachypnoea, tachycardia and hepatomegaly from heart failure Active precordium Soft pan systolic murmur or no murmur Apical mid-diastolic murmur Loud pulmonary second sound (P2)
Symptoms:
Heart failure with breathlessness and faltering growth after 1 week old
Recurrent chest infections
In patients with large VSDs, what would you expect to see on:
CXR (4)
ECG (1)
CXR: Cardiomegaly Enlarged pulmonary arteries Increased pulmonary vascular markings Pulmonary oedema
ECG:
Biventricular hypertrophy by 2 months of age
What is the management of large VSDs?
Diuretics (often captopril - for the heart failure)
Additional calorie input
Surgery at 3-6 months
The ductus arteriosus connects the _____ _____ to the _____ _____. It is referred to as patent when it has failed to close by ______ ______ after the expected delivery date.
Pulmonary artery
Descending aorta
1 month
What are the clinical features and management of PDA?
Clinical features:
Continuous murmur below left clavicle - continues into diastole because pulmonary arterial pressure is always lower than aortic pressure
Increased pulse pressure (causing bounding pulse)
Management:
Closure using could or occlusive device introduced through a cardiac catheter at about 1 year of age - this is necessary to abolish lifelong risk of bacterial endocarditis or pulmonary vascular disease.
What test can be used to determine the presence of heart disease in a cyanosed neonate?
The hyperoxia (nitrogen washout) test: The infant is placed in 100% oxygen for 10mins - if the right radial arterial PaO2 remains low (< 15kPa), a diagnosis of cyanotic congenital heart disease can be made.
NOTE: Lung disease and persistent pulmonary hypertension must be excluded.
How do you manage a cyanosed neonate?
ABC - stabilise with artificial ventilation if necessary
Start prostaglandin infusion (5ng/kg/minute)
What are the side-effects to prostaglandins in neonates?
Apnoea Jitteriness Seizures Flushing Vasodilation Hypotension
What is the most common cause of cyanotic congenital heart disease?
Tetralogy of Fallot
What are the 4 clinical features of tetralogy of Fallot?
NOTE: Symptoms develop in late infancy.
Large VSD
Overriding aorta with respect to the ventricular septum
Sub-pulmonary stenosis causing right ventricular outflow tract obstruction
Right ventricular hypertrophy
Why is it important not to miss hypercyanotic spells in tetralogy of Fallot?
They can lead to MI, cerebrovascular accidents and death.
What are the signs of tetralogy of Fallot?
Clubbing will develop in older children
Loud harsh ejection systolic murmur at left sternal edge from day 1 of life
What features are seen on a CXR of a patient with tetralogy of Fallot? (4)
Relatively small heart
May have an up-tilted apex due to right ventricular hypertrophy
Pulmonary artery bay - a concavity on the left heart border where normal pulmonary artery and right ventricular outflow tract should be
Decreased pulmonary vascular markings
What is the treatment of prolonged hypercyanotic spells in a patient with tetralogy of Fallot?
Sedation and pain relief (morphine)
IV propranolol (peripheral vasoconstrictor)
IV fluids
Bicarbonate to correct acidosis
Muscle paralysis and artificial ventilation to reduce metabolic oxygen demand
What is transposition of the great arteries?
A condition in which the main ateriees are connected the wrong way:
Aorta –> Right ventricle
Pulmonary artery –> Left ventricle
This means that deoxygenated blood is returned to the body and oxygenated blood is returned to the lungs - it is not compatible with life.
What are the CXR features of transposition of the great arteries? (2)
Narrow mediastinum with egg on side appearance of the cardiac shadow
Increased pulmonary vascular markings
What is the management of transposition of the great arteries?
Prostaglandin infusion (maintain patent PDA) Balloon atrial septostomy - breaks the flap valve of the foramen ovale and encourages mixing of blood All patients will require surgery - arterial switch procedure in the neonatal period
What is the life expectancy in a patient with Eisenmenger syndrome?
30-40 years - the patients will die of right heart failure, which is why we aim to prevent Eisenmenger syndrome with early intervention for high pulmonary blood flow.
AVSD is most commonly seen in children with _____ _____. What is the pathophysiology of AVSD?
Down syndrome
Defect in the middle of the heart with a single five-leaflet valve between the atria and the ventricles - it stretches across the entire AV junction and tends to leak and can lead to pulmonary hypertension.
What would be seen on the ECG of a patient with AVSD? What is the management?
ECG = superior axis.
Management = treat heart failure medically and surgically repair defect at 3-6 months.
Give 4 examples of complex congenital heart disease
Tricuspid atresia (most common)
Mitral atresia
Double inlet left ventricle
Common arterial trunk (truncus arteriosus)
What is tricuspid atresia?
Complete absence of the tricuspid valve - in this condition, only the left ventricle is effective (the right is small and non-functional). It presents with cyanosis.
How is tricuspid atresia managed?
Early palliation to maintain a secure supply of blood to the lungs at low pressure by:
Black-Taussig shunt insertion (between subclavian and pulmonary arteries)
Pulmonary artery banding operation to reduce pulmonary blood flow if breathless
Operations can be performed to connect the SVC to the pulmonary artery (Glenn) and the IVC to the pulmonary artery (Fontan).
NOTE: Complete corrective surgery is not possible in most cases because there is only one functioning ventricle.
What is aortic stenosis? Give 2 other conditions it is associated with.
Aortic stenosis = aortic valve leaflets are partly fused together, restricting outflow from the left ventricle.
Associations:
Mitral valve stenosis
Coarctation of the aorta
What are the signs and symptoms of aortic stenosis?
Signs: Small volume, slow rising pulse Carotid thrill (ALWAYS) Ejection systolic murmur (maximal at right sternal edge and radiating to carotids) Delayed and soft aortic second sound Apical ejection click
Symptoms:
Reduced exercise tolerance
Chest pain
Syncope
How is aortic stenosis managed?
Regular clinical/echocardiographic monitoring
Balloon valvulotomy (if high resting pressure gradient or symptomatic on exercise)
Aortic valve replacement (eventually)
What is pulmonary stenosis? It is usually asymptomatic but may cause cyanosis. What are the signs?
Pulmonary stenosis = pulmonary valve leaflets are partly fused together and restricting right ventricular outflow.
Signs:
Ejection systolic murmur (best heard at upper left sternal edge)
Ejection click (best heard at upper left sternal edge)
Prominent right ventricular heave - only if severe
How is pulmonary stenosis managed?
If the pressure gradient across the pulmonary valve is markedly increased (> 64mmHg), intervention is needed. Transcatheter balloon dilatation is the treatment of choice.
What are the clinical features of adult-type coarctation of the aorta?
Gradual progression
Asymptomatic
Systemic hypertension in right arm
Ejection systolic murmur at upper sternal edge
Collateral heard with continuous murmur at the back
Radio-femoral delay
What might be seen on the CXR of a patient with adult-type coarctation of the aorta? (2)
NOTE: It can be managed with a stent.
Rub notching (due to development of large collateral intercostal arteries running under the ribs to bypass the coarctation) 3 sign (visible notch in the descending aorta)
Give 3 causes of outflow obstruction in a sick infant.
Coarctation of the aorta
Interruption of the aortic arch
Hypoplastic left heart syndrome
What is coarctation of the aorta? How does it usually present in neonates?
Restriction of the aorta due to arterial duct tissue encircling the aorta just at the point of insertion of the duct. It usually presents with acute circulatory collapse at 2 days when the duct closes.
What is interruption of the aortic arch?
A rare condition in which there is no connection between the proximal aorta and distal to the ductus arteriosus. It usually presents with shock in the neonatal period, and is often associated with other conditions such as Di George syndrome.
What is the pathophysiology of hypoplastic left heart syndrome? How is it treated?
There is no flow through the left side of the heart, so ductal constriction leads to profound acidosis and rapid cardiovascular collapse.
Management = Surgery (Norwood procedure).
Sinus arrhythmias are normal in children - how are they detected?
As a cyclical change in heart rate with respiration - acceleration during inspiration and slowing on expiration.
What is the most common form of childhood arrhythmia?
Superventricular tachycardia
Superventricular tachycardia:
How does it present?
What will an ECG show?
How is it managed?
Presentation:
Symptoms of heart failure as it can cause poor cardiac output and pulmonary oedema.
ECG:
Narrow complex tachycardia of 250-300bpm
Possible P waves
Possible features of myocardial ischaemia
Possible delta waves (in Wolff-Parkinson-White syndrome)
Management:
Correct tissue acidosis
Give positive pressure ventilation
Vagal manoeuvres (carotid massage, cold ice pack to face)
IV adenosine (IMPORTANT) - if this fails, use electrical cardioversion
Once sinus rhythm is restored, maintenance therapy with flecainide or sotalol is required
