Paediatrics 7

Question 1

Transient synovitis: NICE guidelines for management

Answer 1

• Rest, analgesia and physiotherapy. Tends to resolve in 7 days
• Children aged 3 – 9 years with symptoms of transient synovitis may be managed in primary care if the limp is <48 hours and they are well, however they need to attend A&E if the symptoms worsen or they develop a fever. (safety net)
• They should also be followed up at 48 hours and 1 week to ensure symptoms are improving and then fully resolve.

Question 2

Kawasaki disease

Answer 2

• Medium vessel vasculitis predominantly affecting children
• Tends to affect % children
• More common in Asian children
• Key complication is coronary artery aneurysm
• Will appear very unwell ‘flat’
• Tend to have widespread erythematous maculopapular rashanddesquamation(skin peeling) on the palms and soles. Like strawberry tongue, cracked lips, cervical lymphadenopathy and bilateral conjunctivitis

Question 3

Diagnosis of Kawasaki disease

Answer 3

the presence of high grade fevers (>39) for more than 5 days accompanied by 4/5 of the ‘CREAM’ features:

• Conjunctivitis (bilateral, non-exudative)
• Rash (any non-bullous rash)
• Edema/Erythema of hands and feet
• Adenopathy (cervical, commonly unilateral and non-tender)
• Mucosal involvement (strawberry tongue, oral fissures etc)

Question 4

Kawasaki disease investigations

Answer 4

• Echocardiogram: risk of coronary aneurysms
• Blood tests: CRP or ESR

Question 5

Kawasaki disease stages

Answer 5

• Acute phase: The child is most unwell with the fever, rash and lymphadenopathy. This lasts 1 – 2 weeks.
• Subacute phase: The acute symptoms settle, the desquamation and arthralgia occur and there is a risk of coronary artery aneurysms forming. This lasts 2 – 4 weeks.
• Convalescent stage: The remaining symptoms settle, the blood tests slowly return to normal and the coronary aneurysms may regress. This last 2 – 4 weeks.

Question 6

Kawasaki disease: management

Answer 6

• Intravenous immunoglobulin (IVIg) and high-dose aspirin to prevent coronary artery aneurysms.
• Regular echocardiograms for surveillance of coronary artery aneurysms.
• Patients should be closely monitored as recovery from the acute episode can take several weeks.

Question 7

Causes of failure to thrive

Answer 7

• Inadequate nutritional intake: Maternal malabsorption if breastfeeding, Iron deficient anaemia, family or parental problems, neglect, availability of food (poverty)
• Difficulty feeding: Poor suck (cerebral palsy), cleft lip or palate, genetic conditions with an abnormal facial structure, pyloric stenosis
• Malabsorption: cystic fibrosis, coeliac disease, cows milk intolerance, chromic diarrhoea, IBD
• Increased energy requirements: Hyperthyroidism, Chronic disease i.e. congenital heart disease and cystic fibrosis, Malignancy, chronic infection i.e. HIV or immunodeficiency
• Inability to process nutrition: inborn errors of metabolism, T1D

Question 8

Failure to thrive: definition and assessment

Answer 8

Poor physical growth and development in a child

Assessing failure to thrive
- Pregnancy, birth, developmental and social history
- Feeding or eating history (food diary)
- Observe feeding
- Mums physical and mental health
- Parent-child interactions
-Height,weightandBMI(if older than 2 years) and plotting these on a growth chart
- Calculate themid-parental heightcentile

Question 9

Criteria for inadequate nutrition or a growth disorder

Answer 9

• Height more than 2 centile spaces below the mid-parental height centile
• BMI below the 2nd centile

Question 10

Complications of malnutrition

Answer 10

• Increased risk of infections due to impaired immune function.
• Delayed wound healing and recovery from illnesses.
• Muscle weakness and decreased functional capacity.
• Developmental delays in children.
• Increased morbidity and mortality.

Question 10

Management for malnutrition

Answer 10

• Encouraging regular structured mealtimes and snacks
• Use MUST screening tool
• Reduce milk consumption to improve appetite for other foods
• Review by adietician
• Additionalenergy dense foodsto boost calories
• Nutritional supplements drinks
• Serious: Enteral tube feeding

Question 11

Measles

Answer 11

• caused by measles morbilivirus. Transmitted via droplets from the nose, mouth or throat
• Complications: acute otitis media, bronchopneumonia, encephalitis

Question 12

Measles signs and symptoms

Answer 12

• Prodrome: irritable, conjunctivitis, fever
• High fever above 40 degrees Celsius
• Coryzal symptoms
• A rash appearing 2-5 days after onset of symptoms. Discrete maculopapular rash becoming blotchy and confluent
• Koplik spots: small grey discolouration’s in the mouth

Question 13

Measles investigations

Answer 13

• 1st: Measles-specific IgM and IgG serology (ELISA), most sensitive 3-14 days after onset of the rash.
• 2nd: Measles RNA detection by PCR, best for swabs taken 1-3 days after rash onset.

Question 14

Management of measles

Answer 14

1. Supportive care, usually involving antipyretics.
2. Vitamin A administration for all children under 2.
3. Ribavirin may reduce the duration of symptoms but is not routinely recommended due to side effects.
4. Admission if immunosuppressed or pregnant
5. Notifiable disease - inform public health
6. Management of contacts: if child is not vaccinated and comes into contract the MMR vaccine should be offered within 72 hours

Question 15

Meconium aspiration

Answer 15

• When a newborn inhales a mixture of meconium and amniotic fluid
• Respiratory distress in the new-born as a result of meconium in the trachea.
• It occurs in the immediate neonatal period. Meconium can stimulate an inflammatory response.
• More common in post term babies. It causes respiratory distress, which can be severe.
• Risk factors: maternal hypertension, pre-eclampsia, chorioamnionitis, smoking or substance abuse. Can cause foetal distress and hypoxia.

Question 16

Meconium ileus

Answer 16

• Where the meconium is thickened and causes obstruction of the bowel in the neonate, its most commonly an early sign of cystic fibrosis
• Meconium ileus usually presents as bilious vomiting, a distended abdomen and failure to pass meconium within the first 12–24 hours of life.
• May lead to bowel perforation, peritonitis, malrotation of the bowel and intestinal atresia.

Question 17

Clinical features of meconium aspiration

Answer 17

• Meconium-stained liquor
• Tachypnoea, cyanosis, decreased breath sounds or rate, barrel shaped chest, prolonged expiratory phase
• Respiratory distress at or shortly following birth
• Chest X-ray: hyperinflation, patchy opacification and consolidation
• Increased oxygen requirements (mechanical ventilation may be required for severe cases)
• When aspirated, meconium can cause obstruction, gas trapping, irritation and inflammation leading to damaged lung surfactant, pneumonitis and hypoxia. It can also create a medium for bacteria to grow in the lung.

Question 18

Investigations for meconium aspiration

Answer 18

• Pre- and post- ductal saturations: to detect respiratory involvement
• ABG, Echocardiogram (to see for pulmonary hypertension)
• FBC, CRP, Blood cultures
• Chest x-ray: hyperinflated lungs due to air trapping, patchy pulmonary changes, may have pneumothorax or pneumomediastinum

Question 19

Meconium aspiration management

Answer 19

• For a non-vigorous infant= routine endotracheal suction, may require oropharyngeal suction if the meconium is obstructing the airway. Then Positive Pressure Ventilation (PPV)
• If infant is vigorous: standard neonatal care
• Administer oxygen or CPAP (can exacerbate air trapping) if required
• Infants with respiratory distress should be admitted to the neonatal unit for 4-6 hours to ensure successful transition
• Surfactant therapy
• Antibiotics are started whilst awaiting blood cultures

Question 20

Persistent pulmonary hypertension of the newborn (PPHN)

Answer 20

• Give inhaled nitrous oxide (iNO) if significant PPHN unresponsive to conventional ventilation and oxygen therapy

Question 21

Contraindications for lumbar puncture (any signs of raised ICP)

Answer 21

• focal neurological signs
• papilloedema
• significant bulging of the fontanelle
• disseminated intravascular coagulation
• signs of cerebral herniation
• Lumbar puncture should be performed within an hour of arriving at the hospital before antibiotic treatment is started but should not delay antibiotics over an hour

Question 22

Investigations for meningococcal septicaemia

Answer 22

A lumbar puncture is contraindicated - blood cultures and PCR for meningococcus should be obtained.

Question 23

Meningitis antibiotics

Answer 23

• <3 months: IV amoxicillin + IV cefotaxime
• > 3 months: IV cefotaxime (or ceftriaxone)
• If suspect herpes simplex add acyclovir
• Give steroids if >3 months
• In primary care= If there is suspected meningococcal sepsis (with a non-blanching rash) then IM or IV benzylpenicillin can be given ONLY if this will not delay transfer (and the patient is not allergic)

Question 24

Meningitis steroids

Answer 24

• NICE advices against giving corticosteroids in children younger than 3 months
• Dexamethasone should be considered if the lumbar puncture reveals any of the following: Frankly purulent CSF, CSF white blood cell count greater than 1000/ microlitre. Raised CSF white blood cell count with protein concentration greater than 1 g/litre. Bacteria on gram stain

Question 25

Other management of meningitis

Answer 25

• Fluids: treat any shock i.e. with colloid
• Cerebral monitoring: mechanical ventilation with respiratory impairment
• Public health notification and antibiotic prophylaxis of contacts: ciprofloxacin

Question 26

Neonatal to 3 months

Answer 26

• Group B Streptococcus: usually acquired from the mother at birth. More common in low birth weight babies and following prolonged rupture of the membranes
• coli and other Gram -ve organisms
• Listeria monocytogenes

Question 27

Meningitis causes: 1 month to 6 years

Answer 27

• Neisseria meningitidis (meningococcus)
• Streptococcus pneumoniae (pneumococcus)
• Haemophilus influenzae

Question 28

Meningococcal infection

Answer 28

• Meningococcaemia (septicaemia)
• Meningitis (non-specific signs such as lethargy, headache, fever, rigors and vomiting).
• A mixture of both, associated with a rapidly developing purpuric skin rash.
• Hypovolaemic shock may present with cold peripheries, poor capillary refill time and tachycardia, with decreased urine output.
• Waterhouse-Friderichsen syndrome: When this is associated with massive adrenal haemorrhage and septic shock. This is rare.
• Diagnosed by PCR of blood or CSF cultures

Question 29

Meningitis complications

Answer 29

• 30-50% have permanent neurological squelae
• Hearing loss, seizures, motor deficit, cognitive impairment, Hydrocephalus, visual disturbance
• Acute complications: sepsis, septic shock, DIC, coma, cerebral oedema

Question 30

Viral meningitis

Answer 30

tends to clear within 10 days, may be long term effects like headaches, cognitive and psychological issues. Non-polio enterovirus is the leading cause with mumps, herpesvirus, measles and influenza.

Question 31

Meningitis symptoms different ages

Answer 31

• Children: fever, severe headache, vomiting, common meningitis symptoms
• Infants= irritability, refusing to feed, bulging fontanelles, hypotonia, lethargy, hypothermia

Question 32

When is a lumbar puncture recommended in paediatrics

Answer 32

• Under 1 month presenting with fever
• 1 to 3 months with fever and are unwell
• Under 1 year with unexplained fever and other features of serious illness

Question 33

Migraine treatment

Answer 33

• simple analgesic like paracetamol or NSAID such as ibuprofen.
• Then oral triptan and an NSAID (consider nasal triptan in 12-17)
• If ineffective give a non-oral metoclopramide or prochlorperazine
• Stop potential triggers like COCP

Question 34

When to consider migraine prophylaxis

Answer 34

Preventive treatment should be considered if migraine attacks interfere with school and social life, particularly for children who:

• suffer at least two attacks a month;
• suffer an increasing frequency of headaches;
• suffer significant disability despite suitable treatment for migraine attacks;
• cannot take suitable treatment for migraine attacks.

Question 35

Migraine: Medication prophylaxis

Answer 35

• Propranolol hydrochloride is contra-indicated in asthma. Side-effects such as depression and postural hypotension can further limit its use.
• Topiramate: avoid in women of childbearing age
• Amitriptyline
• If predictable menstrual migraine give frovatriptan or zolmitriptan as ‘mini-prophylaxis’

Question 36

Mumps

Answer 36

• Paramyxovirus
• A viral infection spread by respiratory droplets
• Typically self limiting - lasts 1 week
• Supportive management treating complications if they occur
• Tends to affect 2-12

Question 37

Mumps symptoms

Answer 37

• Prodrome: flu-like
• Symptoms: fever, muscle aches, lethargy, reduced appetite, headache, dry mouth
• Earache, pain on eating
• Parotid swelling: either unilateral or bilateral with associated pain
• Complications: pancreatitis, orchitis, meningitis, sensorineural hearing loss

Question 38

Mumps management and investigations

Answer 38

• Investigations: confirmed on PCR testing with saliva swab (test for antibodies)
• Notifiable disease: inform public health
• Supportive: rest, fluids and analgesia
• Prevent with MMR vaccine

Question 39

Muscular dystrophies

Answer 39

• spectrum of genetic diseases characterised by progressive proximal muscle wasting and weakness.
• Duchenne muscular dystrophy is the most common then Becker muscular dystrophy.
• Both are X-linked recessive so typically affects males
• Due to mutations in the dystrophin gene causing reduced expression which is involved i muscle contractions and stability
• In Duchenne there is no protein in Becker’s there is less expression or the protein is dysfunctional

Question 40

Duchenne’s muscular dystrophy

Answer 40

• Presents in early childhood with muscle wasting and weakness
• Delayed motor milestones, gait abnormalities (waddling gait) and frequent falls
• Children usually become wheelchair-bound before puberty and succumb to respiratory failure by their early twenties
• Pseudohypertrophic calves, as degenerated muscle is replaced by fat
• Difficulty in lifting the child due to proximal muscle weakness
• Grower’s sign: child uses arms to stand up from squatted position
• Associated with dilated cardiomyopathy

Question 41

Becker’s muscular dystrophy

Answer 41

• Presents later in childhood with muscle wasting and weakness
• Patients commonly become wheelchair-bound in their teens and can survive into their thirties

Question 42

Muscular dystrophy investigations

Answer 42

• Gold standard: genetic testing
• Screening: Creatine kinase
• Muscle biopsy: outdated

Question 43

Muscular dystrophy management

Answer 43

• No effective treamtent
• Corticosteroids (prednisolone): slow muscle degeneration
• Physical therapy: maintain mobility
• Supportive care or respiratory and cardiac complications
• Genetic counselling for affected individuals
• Gastronomy feeding in advanced stages
• Spinal surgery in progressive scoliosis
• Cardiac management: ACEi or BB, annual echocarciogram in Duchennes

Question 44

Necrotising enterocolitis

Answer 44

Necrotising enterocolitis is one of the leading causes of death among premature infants where part of the bowel becomes necrotic. Can cause bowel perforation which leads to peritonitis and shock

Question 45

Necrotising enterocolitis: Abdominal x-rays show

Answer 45

• dilated bowel loops (often asymmetrical in distribution)
• bowel wall oedema
• pneumatosis intestinalis (intramural gas)
• portal venous gas
• pneumoperitoneum (gas in the peritoneal cavity) resulting from perforation
• air both inside and outside of the bowel wall (Rigler sign)
• air outlining the falciform ligament (football sign)
• Staged using Bells classification which is a mixture of clinical signs and radiological findings

Question 46

Necrotising enterocolitis risk factors and presentation

Answer 46

• Risk factors= Prematurity, low birth weight, Non breast milk feeds, Sepsis, acute hypoxia, poor intestinal perfusion, congenital heart disease, respiratory distress
• Usually presents in the first three weeks of life
• Inflammation in the immature gut, high mortality rate
• Presentation= vomiting (bile streaked), bloody stools, abdominal distension, feeding intolerance, absent bowel sounds, signs of systemic compromise including an acidosis on a blood gas
• Can quickly progress to abdominal discolouration, perforation and peritonitis

Question 47

Necrotising enterocolitis management

Answer 47

• Patients should be made nil-by-mouth, have a nasogastric tube passed and be admitted to the neonatal unit
• Broad-spectrum antibiotics
• Total parenteral nutrition to rest the bowel
• Supportive treatment with IV fluids and ventilation
• Surgical emergency needs immediate referral to the neonatal surgical team. Surgery to resect necrotic sections of bowel may be necessary, and is essential in cases of bowel perforation or if the infant is deteriorating

Question 48

Examination findings of NEC

Answer 48

• Shiny distended abdomen
• Periumbilical erythema
• Abdominal tenderness
• Bilious gastric aspirate
• Shock

Question 49

NEC prevention and investigations

Answer 49

Prevention= breast feeding encouraged in premature babies as well as delayed cord clamping.

Investigations: Capillary blood gas, blood culture, abdominal x-ray

Question 50

NEC: prognosis

Answer 50

• NEC is a very serious condition that is fatal in around 1/5 cases and can carry significant long-term implications for infants that survive e.g., having a stoma or short gut syndrome.
• Up to 1 in 3 babies with NEC perforate their bowel
• General complications= bowel perforation, DIC, sepsis, adverse neurodevelopment outcomes
• Post operative complications= short bowel syndrome, formation of intestinal stricture, enterocolic fistulae, abscess formation