Paediatrics Pt. 5

Question 1

What is BMD and DMD?

Answer 1

• X-linked recessive degenerative muscle disorders, characterised by progressive muscle weakness and wasting of variable distribution and severity.
• DMD: Rapidly progressive form.
• BMD: Slowly progressive form.

Question 2

What is the aetiology of BMD and DMD?

Answer 2

• DMD: Gene mutations on Xp21 result in the absence of dystrophin (<5% of normal). Two-thirds are inherited, one-third are de novo mutations. Dystrophin protein forms part of a membrane-spanning protein complex of the muscle sarcolemma. This connects the cytoskeleton to the basal lamina.
• BMD: Exon deletions exist in the dystrophin gene Xp21 in 70% of cases. Dystrophin levels are 30–80% of normal. Abnormal translation of the dystrophin gene produces abnormal but partially functional dystrophin.

Question 3

What is the epidemiology of BMD and DMD?

Answer 3

• DMD: 1/3000 live male births.

- BMD: 3–6/100,000 live male births.

Question 4

What are symptoms of BMD and DMD?

Answer 4

• DMD: Child appears healthy at birth. Onset of symptoms from 1–6 years with a waddling gait, toe-walking, difficulty running, climbing stairs or getting up from a seated or lying position. By 10 years braces are required for walking, by 12 years most children are
  wheelchair bound. In 20% there is associated learning disability.
• BMD: Symptoms appear around 10 years and are a milder version of those in DMD.

Question 5

What are signs of BMD and DMD?

Answer 5

• Distribution of muscle weakness: Symmetrical pelvic and shoulder girdle weakness.
• Calf muscle pseudohypertrophy: Excess adipose replacement of muscle fibres.
• Gower’s sign: Child pushes hands down against thighs to overcome proximal muscle and pelvic girdle weakness to stand up from seated position on floor.

Question 6

What are investigations of BMD and DMD?

Answer 6

• Bloods: increased CK present from birth.
• Genetic testing.
• EMG: Establishes myopathy excludes neurogenic causes of muscle weakness.
• Muscle biopsy: Immunostaining for dystrophin.
• Lung function: decreased vital capacity (VC) secondary to decreased muscle strength leads to hypoventilation and atelectasis.

Question 7

What is the management of BMD/DMD?

Answer 7

• Physiotherapy helps prevent contractures
• Exercise and psychological support
• Tendoachilles lengthening and scoliosis surgery
• Weakness of intercostal muscles may lead to nocturnal hypoxia
  
  • This presents with daytime headache, irritability and loss of appetite
• Overnight CPAP may help
• Glucocorticoids (e.g. prednisolone) may help delay wheelchair dependence
• If the left ventricular ejection fraction drops, cardioprotective drugs (e.g. carvedilol) and left ventricular assist devices may be considered

Question 8

What are complications of BMD/DMD?

Answer 8

• Loss of mobility
• Limb contractures
• Scoliosis
• Osteoporosis
• Respiratory insufficiency and infection
• Dilated cardiomyopathy at >15 years.

Question 9

What is the prognosis of BMD/DMD?

Answer 9

• DMD: Respiratory and cardiac failure is the main cause of death. Few live beyond their late twenties.
• BMD: Disease develops later and less rapidly. Most patients walk beyond 16 years of age and may maintain this into adulthood.

Question 10

What is dystrophia myotonia type i?

Answer 10

• Myotonia is delayed relaxation after sustained muscle contraction
• Can be identified clinically and by EMG
• Relatively COMMON
• Autosomal dominant
• Caused by a nucleotide triplet repeat expansion - CTG in the DMPK gene
• It is useful to check the mother for myotonia
  
  • This may manifest as slow release of handshake or difficulty releasing the tightly clasped fist
• Older children can present with a myotonic facial appearance, learning difficulties and myotonia

Question 11

What is the presentation of dystrophia myotonia type I in newborns?

Answer 11

• Hypotonia
• Feeding difficulties
• Respiratory difficulties
• Thin ribs
• Talipes
• Oligohydramnios
• Reduced foetal movements

Question 12

What are causes of the floppy (hypotonic) infant?

Answer 12

• Central
  
  • Cortical
    
    • HIE
    • Cortical malformations
  • Genetic
    
    • Downs
    • Prader-Wili
  • Metabolic
    
    • Hypothyroidism
    • Hypocalcaemia
• Peripheral
  
  • Neuromuscular
    
    • Spinal muscular atrophy
    • Myopathy
    • Myotonia
    • Congenital myasthenia

Question 13

What are neurocutaneous syndromes?

Answer 13

• Embryological disruption causes syndromes involving abnormalities in both systems
• The nervous system and the skin have a common ectodermal origin

Question 14

What is neurofibromatosis type 1?

What is the criteria for NF1?

Answer 14

• Type 1 is autosomal dominant
• It is caused by a mutation in the neurofibromin-1 (NF1) gene
• Arises in about 50% as a de novo mutation
• To make a the diagnosis of NF type 1, two or more of the following criteria need to be present:
  
  • 6+ café-au-lait spots > 5 mm in size before puberty or > 15 mm after puberty
  • 1+ neurofibroma (unsightly, firm nodular overgrowth of any nerve)
  • Axillary freckling
  • Optic glioma (may cause visual impairment)
  • 1 Lisch nodule (hamartoma of the iris seen on slit-lamp)
  • First-degree relative with NF1
• Cutaneous featured tend to become more obvious after puberty
• Neurofibromata appear in the course of a any peripheral nerve
• Visual or auditory impairment may result if there is compression of the 2nd or 8th nerves

Question 15

What is neurofibromatosis type 2?
What is its genetic inheritance?
What are its features?

Answer 15

• Less common
• Autosomal dominant
• Characterised by:
  
  • Multiple inherited schwannomas
  • Meningiomas
  • Ependymomas
• Mutation in the NF2 gene
• Usually presents in adolescence
• Bilateral acoustic neuromata are the predominant feature
• This usually presents with deafness
• It sometimes presents with a cerbellopontine angle syndrome with facial nerve paresis and cerebellar ataxia
• NF1 and NF2 are associated with MEN syndromes

Question 16

What is Tuberous Sclerosis?

Answer 16

• Autosomal dominant
• Caused by mutations in the TSC1 and TSC2 genes
• Cutaneous features:
  
  • Depigmented ash leaf shaped patches or amelanotic naevi which fluoresce under UV light (Wood’s light)
  • Roughened patches of skin (shagreen patches) usually over the lumbar spine
  • Angiofibromata (adenoma sebaceum) - in a butterfly distribution over the bridge of the nose and cheeks
• Neurological features:
  
  • Infantile spasms and developmental delay
  • Epilepsy (often focal)
  • Intellectual disability (often with autism)
• Other features:
  
  • Subungual fibromata
  • Phakomata (dense white areas on the retina) from local degeneration
  • Rhabdomyomata of the heart
  • Angiomyolipomas
  • Polycystic kidneys
  • Cysts in the lungs
• In the brain, there are subependymal nodules and cortical tubers
• Subependymal nodules may enlarge over time and form subependymal giant cell astrocytomas, which can block CSF causing hydrocephalus

Question 17

What is Sturge-Weber syndrome?

What may it present with in its SEVERE form?

Answer 17

• Sporadic disorder
• Characterised by a haemangiomatous facial lesion (port wine stain) in the distribution of the trigeminal nerve
• This is associated with a similar lesion intracranially (ipsilateral leptomeningeal angioma)
• In the MOST SEVERE form, it may present with:
  
  • Epilepsy
  • Intellectual disability
  • Contralateral hemiplegia
• The ophthalmic division of the trigeminal nerve is ALWAYS involved

Question 18

What is the management of Sturge-Weber syndrome?

Answer 18

• Intractable epilepsy –> hemispherectomy

- Laser treatment for port wine stain

Question 19

What is Osgood-Schlatter syndrome?

Answer 19

• Extra-articular disease consisting of a tibial tubercle apophyseal traction injury causing pain and inflammation.

Question 20

What is the aetiology of Osgood-Schlatter syndrome?

Answer 20

• General: Traumatic mechanism. Coincides with the year following a rapid growth spurt. Bilateral in 25%.
• Mechanism: Stress from quadriceps contraction is transmitted through the patellar tendon onto a small portion of the partially developed tibial tubercle apophysis, possibly resulting in a partial avulsion fracture through the ossification centre. 2 heterotopic bone formation occurs in the tendon near its insertion point, forming the visible lump

Question 21

What is the epidemiology of Osgood-Schlatter syndrome?

Answer 21

• Most common knee disorder in adolescence; post-rapid growth spurt
• Girls 10–11 years, boys 13–14 years.
• M>F (increased sporting activities in boys).

Question 22

What are symptoms of Osgood-Schlatter syndrome?

Answer 22

• Knee pain after exercise
• Localised tenderness
• Swelling over the tibial tuberosity
• Often hamstring tightness

Question 23

What are the signs of Osgood-Schlatter syndrome?

Answer 23

• Look: Soft tissue swelling over the proximal tibial tuberosity.
• Feel: Tenderness over the proximal tibial tuberosity at the site of patellar insertion.
• Move: Pain is reproducible on extending the knee against resistance, stressing the quadriceps or squatting with the knee in full flexion. Hamstrings and quadriceps are usually tight and frequently weak. Knee joint examination is normal as this is an extra-articular condition.

Question 24

What are investigations of Osgood-Schlatter syndrome?

Answer 24

• X-ray knee: Shows fractures of the tibial tubercle, possibly a separate ossicle.

Question 25

What is the management of Osgood-Schlatter syndrome?

Answer 25

• Rest: Avoidance of offending activity and other sports with strain on quadriceps. Aim is the achievement of pain-free state.
• Medical: NSAIDs (pain relief/reduce inflammation).
• Orthopaedic devices: Infrapatellar strap and in severe cases a knee immobiliser splint may be used.
• Surgical: Excision of the mobile ossicle is rarely required (skeletally mature adults).
• Rehabilitation: Quadriceps-stretching and hip extension exercises should be advised after the acute symptoms have resolved so as to reduce tension on the tibial tubercle.

Question 26

What are complications of Osgood-Schlatter syndrome?

Answer 26

• Non-union of the tibial tubercle
• Patellar tendon avulsion
• Continuing pain
• Bony prominence.

Question 27

What is the prognosis of Osgood-Schlatter syndrome?

Answer 27

• Benign self-limitng disease

Question 28

What is Perthes disease?

Answer 28

• Avascular necrosis of the capital femoral epiphysis of the femoral head due to interruption of the blood supply
• This is followed by revascularisation and reossification over 18-36 months

Question 29

What are clinical features of Perthes disease?

Answer 29

• Insidious onset
• Limp or hip or knee pain
• May initially be mistaken for transient synovitis

Question 30

What are investigations for Perthes disease?

Answer 30

• If suspected, X-rays of both hips should be performed
• Early signs include increased density in the femoral head, which subsequently becomes fragmented and irregular
• Bone scan and MRI may be helpful

Question 31

What is the management of Perthes disease?

Answer 31

• Acute Pain - supporting care with simple analgesia
• < 5 years
  
  • Mobilisation and monitoring (healing potential is good at this age)
  • Non-surgical containment using splints
• 5-7 years
  
  • Mobilisation and monitoring
  • Surgical containment
• 7-12 years
  
  • Surgical containment
  • Salvage procedure (remodel the acetabulum)
• 12+ years
  
  • Salvage procedure
  • Replacement arthroplasty
• Older children have a poorer prognosis

Question 32

What is osteomyelitis?

Answer 32

• Infection of the metaphysis of long bones
• MOST COMMON sites:
  
  • Distal femur
  • Proximal tibia
  • However, any bone can be affected
• Usually caused by haematogenous spread of a pathogen
• It can arise from direct spread from an infected wound
• Skin is swollen over the affected site
• Sometimes osteomyelitis can spread to cause septic arthritis
• Sickle cell disease is associated with an increased risk of staphylococcal and salmonella osteomyelitis
• TB can cause osteomyelitis, especially in immunodeficient children

Question 33

What are causes of osteomyelitis?

Answer 33

• Most infections are caused by Staphylococcus aureus
• Other causes include:
  • Streptococcus
  • Haemophilus influenzae

Question 34

What are clinical features of osteomyelitis?

Answer 34

• Markedly painful, immobile limb (pseudoparesis)
• Acute febrile illness
• Swelling and exquisite tenderness over the infected site
• Movement of the limb causes severe pain
• May be a sterile effusion of an adjacent joint
• Back pain (vertebral infection)
• Limp or groin pain (pelvis infection)
• Occasionally there will be multiple foci
• NOTE: osteomyelitis can also present insidiously or non-specifically in pre-verbal children

Question 35

What are investigations for osteomyelitis?

Answer 35

• Blood cultures (usually positive)
• WCC and CRP are raised
• X-rays are initially normal
  
  • After 7-10 days, subperiosteal new bone formation and localised bone rarefaction become visible
• Ultrasound may show periosteal elevation at presentation
• MRI allows identification of infection in the bone (subperiosteal pus and purulent debris in the bone)
• Radionuclide bone scan may be helpful if the site of infection is unclear

Question 36

What is the management of acute osteomyelitis?

Answer 36

• High-dose IV empirical antibiotics (usually for 2-4 weeks)
• Once the patient has demonstrated clinical recovery and acute-phase reactants have returned to normal, patients can be switched to oral antibiotics
• IMPORTANT: take blood cultures before starting antibiotics
• The regimen should be altered once results of MC&S arrive
• NOTE: in children who respond well, early transition to oral antibiotics (after 3 days to 1 week) may be considered
• Affected limbs should be immobilised, analgesia should be given and associated comorbidities should be addressed
• Surgical debridement may be necessary if there is dead bone or a biofilm

Question 37

What is the management of chronic osteomyelitis?

Answer 37

• Clinical assessment, disease staging (Cierny-Mader classification) and optimisation of comorbidities
• Surgical debridement
• IV antibiotics
• Functional rehabilitation

Question 38

What is phimosis and foreskin disorders?

Answer 38

• Unretractile foreskin secondary to either a physiological or pathological process.

Question 39

What is the aetiology of phimosis and foreskin disorders?

Answer 39

• Physiological phimosis: The foreskin is not fully developed at birth. Unretractile foreskin may be normal until adolescence.
  Ballooning of the foreskin is a normal process that aids the breakdown of adhesions.
  Foreskin protects the glans whilst the neonate is incontinent of urine (ammoniacal).
• Pathological phimosis: Most likely to be secondary to balanitis xerotica obliterans (BXO) which is a progressive fibrotic condition of unknown aetiology (may also affect the urethral meatus).

Question 40

What is the epidemiology of phimosis and foreskin disorders?

Answer 40

• Physiological: 50% of cases at 1 year of age, 90% by 3 years of age, and 99% by age 17.
• Pathological: BXO = 0.6% (<15 years).

Question 41

What are signs and symptoms of phimosis and foreskin disorders?

Answer 41

• General: Forceful retraction should not be attempted. Often the child will self-retract, allowing inspection.
• Physiological: May have a history of ballooning and spraying of urine. Distal erythema (secondary to urine ammonia irritation). Should have a spout of mucosa as the foreskin is retracted.
• Pathological: There is a white fibrotic ring at the distal foreskin. Absence of normal mucosal spout. Associated with pain +/- haemorrhage.
• Balanitis: Often misdiagnosed. True balanitis involves oedema, erythema and generation of purulent material from the distal phimotic foreskin.
• Investigations aren’t normally required

Question 42

What is the management of phimosis and foreskin disorders?

Answer 42

• Conservative: Don’t retract a foreskin. Variable results for the use of topical steroids for physiological phimosis. Gentle retraction with tissue drying in older boys may aid retraction and prevent ammonia irritation.
• Preputial plasty: Small non-traumatic dorsal slit procedure to widen the meatus.
• Circumcision: Only treatment for BXO. Usually performed under a GA with the sleeve dissection method. In neonatal ritual procedures, devices such as the PlastibellTM may be used.

Question 43

What are complications of phimosis and foreskin disorders?

Answer 43

• Pathological: Progressive phimosis and possible urinary retention.
• Circumcision: Haemorrhage, infection, meatal stenosis, glans injury, urethrocutaneous fistula, anaesthetic risks.

Question 44

What is the prognosis of phimosis and foreskin disorders?

Answer 44

• Physiological: Majority will retract with time.
• Pathological: Advanced BXO may affect the urethral meatus and extend proximally which may require extensive reconstructive surgery

Question 45

What is pnuemonia?

Answer 45

• Infection of the lung parenchyma.

Question 46

What is the aetiology of pnuemonia?

Answer 46

• Neonates: Organisms from the female genital tract: group B haemolytic streptococcus, Escherichia coli and gram-negative bacilli, Chlamydia trachomatis
• Infants–preschool children:
  
  • Viral (most common): parainfluenza, influenza, adenovirus and RSV. RSV can be particularly dangerous to ex-preterm infants and infants with underlying CLD of prematurity.
  • Bacterial: Streptococcus pneumoniae (90% of bacterial pneumonia). Staphylococcus aureus is uncommon but causes severe infection.
• Older children–adolescents: As above, but also atypical organisms such as Mycoplasma pneumoniae and Chlamydia pneumoniae. TB should be considered at any age.
• Aspiration pneumonia: Enteric gram-negative bacteria +/- Strep. pneumoniae, Staph. aureus
• Non-immunised: Haemophilus influenzae, Bordetella pertussis, measles.
• Immunocompromised (inherited or acquired):
  
  • Viral: CMV, VZV, HSV, measles and adenoviruses.
  • Bacterial: Pneumocystis carinii, TB.

Question 47

What is the epidemiology of pneumonia?

Answer 47

• 29/10,000 children <5 years of age. 12/10,000 children <14 years of age.
• Decreasing since the introduction of the conjugate pneumococcal vaccine in all children

Question 48

What are signs and symptoms of pneumonia?

Answer 48

• General: Fever, tachycardia, tachypnoea, cough, sputum (yellow, green or rusty in Strep. pneumoniae), vomiting particularly post-coughing, poor feeding, diarrhoea, preceding URTI (especially viral infections).
• Signs of consolidation: decreased Breath sounds, dullness to percussion, increased tactile/vocal fremitus, bronchial breathing, coarse crepitations.

Question 49

What are investigations for pneumonia?

Answer 49

• CXR: Focal consolidation suggests a bacterial cause; diffuse consolidation bronchopneumonia suggests a viral cause.
• Bloods: increased WCC, increased ESR/CRP, U&Es (SIADH), mycoplasma serology.
• Urine: Pneumococcal antigen.
• Microbiology: Blood and sputum MC&S.
• Blood film: RBC agglutination by Mycoplasma (cold agglutinins; see Haemolytic anaemia).
• Immunofluorescence/PCR: Can detect RSV on nasopharyngeal aspirate.

Question 50

What is the management of pneunomia?

Answer 50

• Determine severity
  
  • Measure temperature
  • Examine chest
  • Record BP, HR and RR
  • Note the degree of agitation and consciousness
  • Note signs of exhaustion, cyanosis and accessory muscle use
  • Assess hydration status (capillary refill, examining skin turgor, dry mucous membranes and urine output)
• Immediately refer to hospital if:
  
  • Persistent SpO2 < 92% on air
  • Grunting, marked chest recession, RR > 60/min
  • Cyanosis
  • Child looks seriously unwell, does not wake, or does not stay awake if roused or does not respond to normal social cues
  • Temperature > 38 degrees in a child < 3 months
  • Consider admission if: dehydration, decreased activity, nasal flaring, predisposing diseases (e.g. chronic lung disease)
  • Whilst awaiting hospital admission
    
    • Give controlled supplemental oxygen if SpO2 < 92%
• If hospital admission is not needed
  
  • Most children can be managed at home
  • Prescribe antibiotics
    
    • IMPORTANT: all children with a clinical diagnosis of pneumonia should receive antibiotics because bacterial and viral pneumonia cannot be reliably distinguished from each other
    • NOTE: children < 2 yrs presenting with mild respiratory tract symptoms do NOT usually have pneumonia and do not usually require antibiotics
    • AMOXICILLIN is first-line for 7-14 days
    • Alternatives: co-amoxiclav, cefaclor or macrolides (e.g. erythromycin)
    • Macrolides can be added at any stage if there is no response to first-line treatment
• Advice
  
  • Paracetamol or ibuprofen if the child is distressed by the fever (should NOT be given simultaneously but may be interchanged)
  • Advise adequate fluid intake
  • Advise parents not to smoke at home
  • Check on the child regularly through day and night
  • Seek medical advice if they are unable to cope or the child’s condition deteriorates (e.g. signs of increased respiratory effort/distress, reduced fluid intake, reduced responsiveness, worsening or unresolving fever)

Question 51

What are complications of pnuemonia?

Answer 51

• Pleural effusion, empyema, lung abscess, septic shock, ARDS, ARF.

Question 52

What is the prognosis of pnueomonia?

Answer 52

• Most resolve within 1–3 weeks; however, children with an underlying respiratory, cardiac, immune or neurological abnormality may respond more slowly to treatment, and have a higher mortality.

Question 53

What is nephrotic syndrome?

Answer 53

• Characterised by hypoalbuminaemia, proteinuria and oedema.

Question 54

What is the aetiology of nephrotic syndrome?

Answer 54

• All causes of glomerulonephritis (GN) can cause nephrotic syndrome.
• Primary: Minimal change disease (MCD), focal segmental glomerulosclerosis (FSGS) and membranous nephropathy (MN).
• Secondary: SLE, post-infectious (group A b-hemolytic streptococcus, syphilis, malaria, TB, varicella, hepatitis B, HIV, EBV), collagen vascular disease, HSP, hereditary nephritis (Alport syndrome), sickle cell disease.

Question 55

What is the epidemiology of nephrotic syndrome?

Answer 55

• Developed countries: 2–7/100,000/year (UK). MCD is the cause of nephrotic syndrome in 90% of children; most common in boys <5 years. Peak age: 2–4 years.
• Developing countries: Infectious causes of GN: malaria (40%), HBV infection (6%) and group A b-haemolytic streptococcal infection (rare).

Question 56

What are signs and symptoms of nephrotic syndrome?

Answer 56

• General: Anorexia, lethargy, oliguria, hypertension.
• GI: Diarrhoea, poor feeding, abdominal pain.
• Oedema: Swelling of face, ascites, oedema of legs/scrotum.
• Symptoms of complications: Infections, renal vein thrombosis, loin pain, haematuria.

Question 57

What are investigations of nephrotic syndrome?

Answer 57

• Bloods: U&E, decreased albumin, “ESR/CRP, lipid profile (secondary hyperlipidaemia).
• Post-infectious nephropathy: Plasmodium falciparum (thick and thin blood films), ASOT, HBV/EBV/HIV serology, HIV PCR.
• Urine dipstick: 3/4 + protein, microscopic haematuria.
• MSU: MC&S.
• 24-h urine collection: Creatinine clearance and 24-h protein excretion
• Renal USS: Other renal diseases may cause proteinuria, e.g. polycystic kidney disease.
• Renal biopsy: Ror older children with haematuria, “BP, renal impairment, steroid-resistant patients.
• Doppler USS, renal angiogram, CT, MRI: If renal thrombosis is suspected.

Question 58

What is the management of nephrotic syndrome?

Answer 58

• Symptomatic treatment: Limit oedema with low-sodium diet and diuretics.
• Monitor: BP, U&E, Ca2+, weight, fluid balance.
• Treatment of initial presentation: Longer duration (6 months) of initial prednisolone treatment is associated with fewer relapses and lower total prednisolone dose over the first 2 years.
• Treatment of relapse: Prednisolone daily until in remission, then a slow gradual reduction of dosage.
• Treatment of steroid-resistant patients: Alternate-day prednisolone with long-term cyclosporin or cyclophosphamide. Steroid-sensitive patients (85–90% cases) respond after 4 weeks, steroid-resistant (10–15% cases) have no remission after 4 weeks.
• Treatment of hypertension: ACE inhibitors are the drug of choice.
• Prevention of complications: Penicillin prophylaxis to prevent pneumococcal peritonitis and septicaemia, mobilisation/TED stockings to prevent thrombosis.

Question 59

What are complications of nephrotic syndrome?

Answer 59

• Renal failure: secondary to hypovolaemia, diuretics or renal vein thrombosis.
• Increased Infection risk: Peritonitis, pneumococcal infections due to urinary loss of Ig
• Hypercoagulability: Renal vein thrombosis and DVT secondary to hypovolaemia, urinary loss of antithrombin III, protein C and S, increased synthesis of fibrinogen in the liver, immobility secondary to leg oedema and steroid therapy.
• Hyperlipidaemia: Increased Synthesis of triglycerides and cholesterol with albumin in the liver.

Question 60

What is the prognosis of nephrotic syndrome?

Answer 60

• Steroids have improved prognosis but still is affected by complications and S/E of treatment.

Question 61

What is acute renal failure?

Answer 61

• A significant deterioration in renal function occurring over hours or days, resulting in increased plasma urea, creatinine and oliguria. Complete recovery of renal function usually occurs within days/weeks.

Question 62

What is the aetiology of acute renal failure?

Answer 62

• Pre-renal
  
  1. Hypovolaemia (haemorrhage, GI losses, DKA, burns, diarrhoea, septic shock)
  2. Cardiac failure (severe coarctation, hypoplastic left heart, myocarditis)
  3. Hypoxia (pneumonia, RDS).
• Intrinsic renal
  
  1. Acute tubular necrosis (ATN) (80% of instrinsic renal causes) due to circulatory compromise or nephrotoxic drugs (paracetamol, aminoglycosides)
  2. Acute GN (see chapter)
  3. Acute interstitial nephritis (infection, drugs: NSAIDs, frusemide, penicillin)
  4. Small/large vessel obstruction (renal artery/vein thrombosis, vasculitis, HUS, TTP).
• Post-renal (obstructive)
  
  1. Neuropathic bladder: may be acute in transverse myelitis, spinal trauma
  2. Stones (bilateral pelviureteric junction or ureteral)
  3. Urethral prolapse of bladder ureterocoele
  4. Iatrogenic: catheters, stents, nephrostomy or surgery.

Question 63

What is the epidemiology of acute renal failure?

Answer 63

• 0.8/100,000 children

Question 64

What are symptoms of acute renal failure?

Answer 64

• Vomiting, anorexia, oliguria, convulsions, previous sore throat and fever (post-streptococcal GN), bloody diarrhoea and progressive pallor (HUS), drug history.

Question 65

What are signs of acute renal failure?

Answer 65

• Assess intravascular volume status: volume depleted (cool peripheries, tachycardia, postural hypotension) or overloaded (oedema, weight gain, pulmonary
  oedema) ? Is patient septic? Is patient obstructed? Examine abdomen for palpable bladder.

Question 66

What are investigations for acute renal failure?

Answer 66

• Bloods: decreased Hb (hypovolaemia/haemorrhage), increased WCC, increased CRP, blood cultures (sepsis), increased urea, increased creatinine, increased K+ , increased phosphate, increased Ca2+ , decreased Mg2+, LFTs, venous capillary blood gas, clotting
  (DIC), ASOT (post-streptococcal GN).
• Blood film: HUS/TTP (RBC fragmentation).
• Urine: Urinalysis for blood, protein (GN), glucose (interstitial nephritis), microscopy for casts (GN), urine Na + , urea, creatinine, osmolality to differentiate between pre-renal and intrinsic renal failure.
• ECG: Signs of hyperkalaemia; tall tented T waves –> small or absent P waves –> increased P–R interval –> widened QRS complex –> sine wave pattern –> asystole.
• CXR: Signs of pulmonary oedema.
• Renal USS: In ARF, kidneys appear normal or increased in size and echogenicity, may detect stones or clot in renal vein thrombosis (RVT).
• Renal biopsy: If diagnosis has not been determined.
• Monitor: Daily U&E, temperature, PR, RR, BP, O2 saturation, strict input/output (need to catheterise), daily weights.

Question 67

What is the management of acute renal failure?

Answer 67

• Ultrasound may be useful to identify obstruction, small kidneys in CKD or large, bright kidneys with loss of cortical medullary differentiation (typical of an acute process)
• Use diuretics when necessary (i.e. to treat fluid overload or oedema whilst the patient is awaiting renal
• Fluid restriction will also be helpful
• Pre-renal failure: Hypovolaemia should be urgently addressed with fluid replacement and circulatory support
• Renal failure: A high-calorie, normal protein feed will decrease catabolism, uraemia and hyperkalaemia
• Manage metabolic abnormalities
• Refer immediately to urology if any of the following are present:
  
  • Pyelonephrosis
  • Obstructed solitary kidney
  • Bilateral upper urinary tract obstruction
  • Complications of AKI caused by urological obstruction
• Relief can be achieved by nephrostomy or bladder catheterisation (Post-renal failure)
• Dialysis

Question 68

What are indications for dialysis?

Answer 68

• Failure of conservative management
• Hyperkalaemia
• Severe hyponatraemia or hypernatraemia
• Pulmonary oedema or severe hypertension due to volume overload
• Severe metabolic acidosis
• Multisystem failure

Question 69

What are complications of acute renal failure?

Answer 69

• Heart failure and pulmonary oedema (volume overload)
• GI bleeding (gastric ulceration and platelet dysfunction)
• Muscle wasting due to hypercatabolic state
• Uraemic encephalopathy.

Question 70

What is the prognosis of acute renal failure?

Answer 70

• Depends on the causative factor.
• Recovery of renal function following ARF is most likely following pre-renal causes, HUS, ATN, acute interstitial nephritis or uric acid nephropathy

Question 71

What is chronic renal failure?

Answer 71

• Characterised by decreased GFR, persistently increased urea and decreased creatinine concentration.

Question 72

What is the aetiology of chronic renal failure?

Answer 72

• Age <5 years: Congenital abnormalities: hypoplasia, dysplasia, obstruction (posterior urethral valve), malformations.
• Age >5 years:
  1. Hereditary disorders: Alport syndrome (thickened glomerular basement membrane),
     autosomal recessive polycystic disease
  2. All causes of GN and tubulointerstitial nephritis may lead to CRF (see GN chapter)
  3. VUR
  4. Systemic disease (HSP, SLE).

Question 73

What is the epidemiology of chronic renal failure?

Answer 73

• CRF prevalence not known but prevalence of end-stage renal failure (ESRF) 15–40/million (UK)
• More common in Asian children.

Question 74

What are symptoms of chronic renal failure?

Answer 74

• Antenatal diagnosis
• Failure to thrive
• Delayed puberty,
• Malaise, anorexia, anaemia, incidental (blood test/urinalysis).

Question 75

What are signs of chronic renal failure?

Answer 75

• Palpable kidneys (polycystic disease)

- Pallor, oedema, pigmentation, scratch marks, hypertension, growth retardation and rickets.

Question 76

What are investigations for chronic renal failure?

Answer 76

• Bloods: decreased Hb, MCV (usually normocytic) decreased Na+ , increased K+ , increased urea, increased creatinine, decreased Ca2+, increased phosphate, increased ALP, increased PTH (secondary hyperparathyroidism).
• Urine: 24-h collection for protein and creatinine clearance.
• X-rays: For signs of osteomalacia and hyperparathyroidism.
• Renal USS: For anatomical/hereditary abnormalities, measure size (small shrunken kidneys consistent with CRF), exclude obstruction/stones.
• Renal biopsy: For changes specific to the underlying disease, contraindicated in shrunken kidneys.

Question 77

What is the management of chronic renal failure?

Answer 77

• Aim to prevent symptoms and metabolic abnormalities, allow normal growth and development and preserve residual renal function
• Diet
  
  • Anorexia and vomiting are common
  • Calorie supplements or NG/gastrostomy feeding is often necessary to optimise growth
  • Protein intake should be sufficient to maintain growth and normal albumin (but preventing the accumulation of toxic metabolic by-products)
• Prevention of renal osteodystrophy
  
  • Decreased activation of vitamin D leads to phosphate retention and hypocalcaemia, which, in turn, leads to secondary hyperparathyroidism and eventually osteitis fibrosa cystica and osteomalacia
  • Phosphate restriction (by reducing milk), using calcium carbonate as a phosphate binder and activated vitamin D supplements can help
• Control of salt and water balance and acidosis
  
  • Many children will also have obligatory loss of salt and water
  • They need salt supplements and a lot of water
  • Treatment with bicarbonate supplements is needed to prevent acidosis
• Acidosis
  
  • Reduced EPO production and circulation of marrow-toxic metabolites leads to anaemia
  • Responds well to the administration of SC recombinant human erythropoietin
• Hormonal abnormalities
  
  • Growth hormone resistance, characterised by a high GH level but poor growth, is a feature of CKD
  • Recombinant human GH is effective in improving growth for up to 5 years of us
  • Many children with stage 4/5 CKD will have delayed puberty or subnormal pubertal growth spurt

Question 78

What are complications of chronic renal failure?

Answer 78

• Haematological: Anaemia, abnormal platelet activity (bruising, epistaxis).
• Cardiovascular: Accelerated atherosclerosis, increased BP and pericarditis.
• Neurological: Peripheral and autonomic neuropathy, proximal myopathy.
• Renal osteodystrophy: Osteoporosis, osteomalacia, secondary/tertiary hyperparathyroidism.
• Endocrine: Amenorrhoea.
• Peritoneal dialysis: Peritonitis (e.g. Staphylococcus epidermidis).
• Haemodialysis:
  
  1. Acute: hypotension due to excessive removal of extracellular fluid
  2. Long-term: atherosclerosis, sepsis (2 peritonitis with Staph. aureus infection)
  3. Amyloidosis: leads to periarticular deposition, arthralgia (e.g. shoulder) and carpal tunnel syndrome
• Transplantation/immunosuppression: Opportunistic infections (e.g. Pneumocystis carinii), malignancies (lymphomas and skin), and side effects of immunosuppressant drugs.

Question 79

What is the prognosis of chronic renal failure?

Answer 79

• Depends on complications

- Timely dialysis/transplantation improves survival.

Question 80

What is acute glomerulonephritis?

Answer 80

• Bilateral inflammation of the renal glomeruli with proliferation of cellular elements secondary to an immunological mechanism.

Question 81

What is the aetiology of acute glomerulonephritis?

Answer 81

• Post-infectious: secondary to group A b-haemolytic streptococcus; Other causative organisms: diplococcal, staphylococcal, mycobacterial, CMV, coxsackie virus, EBV.
• Haemolytic uraemic syndrome: Haemolytic anaemia, thrombocytopenia and endothelial damage to glomerular capillaries due to E.coli 0157, Shigella or Salmonella.
• Henoch–Schonlein purpura (HSP): Hypersensitivity vasculitis and inflammatory response within the glomerular capillaries
• Berger disease: Diffuse mesangial deposition of IgA and IgG.
• Systemic lupus erythematosus (SLE) nephritis: The immune system produces antinuclear antibodies. Antibodies and complement complexes accumulate in the kidneys resulting in an inflammatory response.

Question 82

What is the epidemiology of acute glomerulonephritis?

Answer 82

• Typically 2–12 years old, although may be any age
• M : F = 2 : 1.
• Incidence depends on underlying cause but generally rare. SLE is uncommon in children.

Question 83

What are the signs and symptoms of acute glomerulonephritis?

Answer 83

• General: Characterised by the sudden onset of haematuria, proteinuria and red cell casts.
  Often associated with hypertension, oedema (face, ankles and ascites) and impaired renal function. Flank pain may be present secondary to renal capsule swelling.
• HSP: Characteristic petechial/purpuric rash on buttock and extensor surfaces, arthralgia commonly at the ankles, symptoms of nephritis, abdominal pain

Question 84

What are investigations for acute glomerulonephritis?

Answer 84

• Urine: Microscopy; RBC +/- casts and culture, 24-hour collection; protein and creatinine clearance.
• Bloods: decreased Hb, increased WCC, decreased Plt (HUS), increased urea, increased ESR/CRP, metabolic acidosis, GFR
• Specific: increased Antistreptolysin titre, (increasedd in 1–3/52 and peaks at 3–5/52), RBC fragmentation, stool culture (HUS), IgA antibodies, antinuclear antibodies (SLE).
• Imaging: Renal USS
• Surgical: Renal biopsy may be necessary.

Question 85

What is the management of acute glomerulonephritis?

Answer 85

• General: Correct electrolyte abnormalities/acid–base imbalances. Fluid restriction if severe oedema present.
• Medical: PO antibiotics for post-infective causes (penicillin for streptococcus), antihypertensive therapy, treatment of pulmonary oedema, possible use of steroids +/- cytotoxic/immunosuppressive agents (HSP, vasculitis).

Question 86

What are complications of acute glomerulonephritis?

Answer 86

• Hypertension: Encephalopathy, convulsions, end-organ damage, cerebral haemorrhage.
• Renal failure: Uraemia, metabolic acidosis, electrolyte abnormalities, fluid overload.

Question 87

What is the prognosis of acute glomerulonephritis?

Answer 87

• General: 10% may develop chronic glomerulonephritis. Mortality depends on cause.
• Post-infection: Good for >95%; acute phase lasts 1–2/52, hypertension for 3–4/52 and microscopic haematuria for <12/12 but rapidly progressive to CRF in 1%.
• HUS: Life-threatening condition: mortality 5–30%; some may relapse.
• IgA nephropathy: 10–30% progress to CRF.
• HSP: Usually resolves spontaneously without treatment. 1–2% develop CRF.

Question 88

What is a nephroblastoma? (Wilms tumour)

Answer 88

• Originates from embryonal renal tissue
• MOST COMMON renal tumour of childhood
• Over 80% of patients present < 5 years of age

Question 89

What are the clinical features of a Wilms tumour?

Answer 89

• Large abdominal mass
• Usually found incidentally in an otherwise well child
• Haematuria
• Rare symptoms: abdominal pain, anorexia, anaemia, hypertension

Question 90

What are investigations for Wilms tumours?

Answer 90

• Ultrasound
• CT/MRI
• Shows an intrinsic renal mass

Question 91

What is the management of Wilms tumours?

Answer 91

• Surgery and chemotherapy (may be post-operative or pre-operative)
• Subsequent management is dependent on histological findings
• 5% have bilateral disease
• More than 80% of patients are cured

Question 92

What is chronic lung disease of prematurity?

Answer 92

• Oxygen requirement at corrected age of term with characteristic radiological changes (also known as bronchopulmonary dysplasia).

Question 93

What is the aetiology of CLD of prematurity?

Answer 93

1. Volutrauma and barotraumas 2 to positive pressure ventilation (PPV)
2. High inspired oxygen concentration (>40%); toxic to the immature lung
3. Activation of inflammatory mediators (2 to free radicals, barotraumas and infection)
4. Inadequate nutritional supplementation.

Question 94

What is the epidemiology of CLD of prematurity?

Answer 94

• Inversely related to gestation and birthweight.

- Increased incidence secondary to increased survival of very low-birthweight infants.

Question 95

What are signs and symptoms and CLD of prematurity?

Answer 95

• Most properly managed neonates are asymptomatic as they are given ventilatory support, but some CLD babies may have some chronic recession.
• Poor weight gain with increased energy intake.

Question 96

What are investigations for CLD of prematurity?

Answer 96

• ABG: Compensated respiratory acidosis reflecting chronic high pCO2.
• CXR: Characteristic hyperinflation and cystic changes. Used to determine severity, and distinguishes CLD from atelectasis, pneumonia and air-leak syndrome.

Question 97

What is the management of CLD of prematurity?

Answer 97

• General: PPV and oxygen therapy are still necessary for preterm survival; therefore must reduce tisk
• Ventilation: Oxygen toxicity, barotraumas and volutrauma can be minimised by strict monitoring and maintenance of pH, pCO2 and pO2 within small ranges dependent on age of preterm infant. There is no evidence that high-frequency ventilation is superior to
  conventional in prevention (Cochrane database).
• Nutritional support: Early parenteral nutrition. Prevents further lung injury and aids tissue repair.
• Prevention:
  
  1. Surfactant within 2 hours with early extubation to nasal CPAP for the treatment of RDS decreases
     incidence of CLD.
  2. Use of steroids to prevent CLD is highly controversial as it has been shown to have an adverse affect on neurodevelopmental outcome. Steroids are generally only used in ventilator-dependent neonates who are ‘stuck’ on the ventilator.
• Long-term: Home O2 supported by community children’s nurses; ‘hospital at home’ team.

Question 98

What are complications of CLD of prematurity?

Answer 98

• Pulmonary hypertension —> cor pulmonale, increased risk of respiratory infections especially RSV pneumonia (paliviazumab, monoclonal antibody prophylaxis for RSV, is currently being considered by NICE).

Question 99

What is the prognosis of CLD of prematurity?

Answer 99

• Severely affected may need long-term home O2 therapy

Question 100

How does arthritis present?

Answer 100

• Pain, swelling, heat, redness and restricted movement in a joint
• In acute monoarthritis, the child may be systemically unwell with a fever
• If septic arthritis or osteoarthritis is suspected, urgent diagnosis and treatment is required

Question 101

What is reactive arthritis?

Answer 101

• MOST COMMON form of arthritis in childhood
• Characterised by transient joint swelling (< 6 weeks)
• Often affects the ankles and knees
• Usually follows evidence of extra-articular infection
• Possible low grade fever

Question 102

What are the causes of reactive arthritis?

Answer 102

• Enteric bacteria (Salmonella, Shigella, Campylobacter and Yersinia) are often the cause in children
• Sexually transmitted infections (Chlamydia, Gonococcus) is more likely in adolescents
• More rare causes include Mycoplasma and Borrelia burgdorferi (Lyme disease)
• Rheumatic fever and post-streptococcal reactive arthritis are more common in low-income countries

Question 103

What is the management of reactive arthritis?

Answer 103

• NO treatment required - it is self-resolving
• NSAIDs for pain-relief
• Others: steroids, DMARDs

Question 104

What is septic arthritis?

Answer 104

• Serious infection because it can lead to bone destruction
• Most common in children < 2 yrs
• Usually results from haematogenous spread
• However, it can occur following a puncture wound or an infected skin lesion (e.g. chickenpox)
• Can occur from the spread of adjacent osteomyelitis to the joint capsule
• Usually only ONE joint is affected
• The hip is of particular concern in infants and young children
• After the neonatal period, the most common organism is Staphylococcus aureus
• Consider predisposing illnesses such as immunodeficiency and sickle cell disease

Question 105

What are clinical features of septic arthritis?

Answer 105

• Erythematous, warm, acutely tender joint
• Reduced range of movement
• Acutely unwell, febrile child
• Infants may hold the limb still (pseudoparesis, pseudoparalysis)
• Infants will cry when the affected limb is moved
• Joint effusion
• Co-existent osteomyelitis (15%)
• Pain may be referred to the knee

Question 106

What are investigations for septic arthritis?

Answer 106

• High WCC
• Raised acute-phase proteins
• Ultrasound of deep joints (reveal an effusion)
• X-rays to exclude trauma (X-rays are likely to be normal in septic arthritis)
• Aspiration under ultrasound guidance is the definitive investigation
• This should be performed IMMEDIATELY

Question 107

What is the management of septic arthritis?

Answer 107

• Prolonged course of antibiotics (initially IV for 2 weeks, followed by 4 weeks of oral antibiotics)
  
  • Suspected Gram-positive
    
    • Vancomycin + joint aspiration
    • 2nd line = clindamycin or cephalosporin + joint aspiration
  • Suspected Gram-negative
    
    • 3rd generation cephalosporin (e.g. ceftriaxone) + joint aspiration
    • 2nd line = IV ciprofloxacin + joint aspiration
• Affected joints should be aspirated to dryness as often as required (through closed needle aspiration or arthroscopically)
• Washing out of the joint or surgical drainage may be required

Question 108

What is juvenile idiopathic arthritis?

Answer 108

• MOST COMMON chronic inflammatory joint disease in children
• Defined as persistent joint swelling (> 6 weeks duration) presenting before 16 years of age in the absence of infection or any other defined cause
• There at least 7 subtypes of JIA

Question 109

What are the 7 subtypes of JIA?

Answer 109

• Oligoarthritis (persistent)
• Oligoarthritis (extended)
• Polyarthritis (RF negative)
• Polyarthritis (RF positive)
• Systemic arthritis
• Psoriatic arthritis
• Enthesitis-related arthritis
• Undifferentiated arthritis

Question 110

How is JIA classified?

Answer 110

• Polyarthritis = > 4 joints
• Oligoarthritis = < 4 joints
• Systemic = with fever and rash
• Other subtypes: psoriatic arthritis, enthesitis
• Subtyping can be further classified by the presence of rheumatoid factor and HLA-B27 tissue type

Question 111

What are clinical features of JIA?

Answer 111

• Gelling (stiffness after rest)
• Morning stiffness
• Joint pain
• Young children: may present with intermittent limp or deterioration in behaviour or mood or avoidance of previously enjoyed activities
• Joint swelling (may be minimal)

Question 112

What investigations are done for JIA?

Answer 112

• Baseline investigations may be normal at first
• ANA may be present
• IMPORTANT: in any child with widespread joint pain, fatigue or multisystem involvement, possibly consider connective tissue disorder
• If there are systemic features, consider sepsis or malignancy

Question 113

What are complications of JIA?

Answer 113

• Bone expansion (leading to leg lengthening or valgus deformity, or discrepancy in digit length)
• Chronic anterior uveitis
  
  • If not detected and treated, it could lead to severe visual impairment
• Flexion contractures of the joints
  
  • Occurs when the joint is held in the most comfortable position for a long time
  • Can lead to joint destruction
• Growth failure
  
  • May be generalised from anorexia, chronic disease and systemic corticosteroid therapy
  • Localised overgrowth can occur due to prolonged active knee synovitis
• Constitutional problems
• Osteoporosis
• Amyloidosis

Question 114

What is the management of JIA?

Answer 114

• Managed by a specialist paediatric rheumatology MDT
• Physical and occupational therapy
• Inactivity leads to deconditioning, disability and decreased bone mass
• NSAIDs for pain and stiffness
• Corticosteroids are useful adjunctive agents
• DMARDs (oral or SC methotrexate is the first-line DMARD) - used when the disease fails to respond to conventional treatments
  
  • 2nd line: sulfasalazine
• Other treatments: TNF-alpha inhibitors, interleukin receptor antagonists, anti-emetics

Question 115

What is the prognosis of JIA?

Answer 115

• Most children can expect good disease control and quality of life
• With poor disease control, patients can experience significant morbidity from joint damage and visual impairment leading to uveitis and fractures from osteoporosis

Question 116

What is septicaemia?

Answer 116

• Bacteraemia: Proliferation of bacteria in the circulation.
• Septicaemia: Systemic response to infection; tachypnoea, tachycardia and fever or hypothermia.
• Sepsis syndrome/systemic inflammatory response syndrome (SIRS): Evidence of reduced end-organ perfusion (oliguria/altered GCS) with elevated lactate levels.
• Septic shock: Sepsis syndrome plus hypotension that does not respond to fluid therapy.

Question 117

What is the aetiology of septicaemia?

Answer 117

• Early-onset neonatal sepsis: due to GBS or E. coli.
• Late-onset neonatal sepsis: Usually due to Neisseria meningitidis, Streptococcus pneumoniae, Hib, HSV, CMV or enterovirus.
• Hospital acquired: Due to S. aureus, S. epidermidis or gram-negative organisms.
• Immunocompromised septicaemia: Infected by broader spectrum of pathogens including fungi.
• Older children: Usually caused by Neisseria meningitides or Strep. pneumoniae.

Question 118

What are signs and symptoms of septicaemia?

Answer 118

Presentation depends on the system affected:

• CNS: infant: bulging fontanelle (neonates), lethargy, irritability, poor feeding. Child: headache, photophobia, neck stiffness, seizures, decreased GCS.
• Respiratory: tachypneoa, apnoea, grunting, cyanosis.
• Cardiovascular: tachycardia, hypotension.
• GI: poor feeding, abdominal pain, vomiting, diarrhoea.
• General: lethargy, fever, hypothermia, purpuric rash.

Question 119

What are investigations for septicaemia?

Answer 119

• Bloods: increased/decreased WCC (neutropenia/neutrophilia), increased CRP, U&E, blood glucose, clotting, ABG (hypoxia, metabolic acidosis).
• Radiology: CXR, USS abdomen if intra-abdominal sepsis is suspected.
• MC&S: MSU, blood culture CSF (LP if vital signs are stable enough to tolerate procedure). LP is contraindicated if there are signs of raised ICP, purpura or deranged clotting (DIC).

Question 120

What is the management of septicaemia?

Answer 120

• Assess HIGH RISK of severe illness or death from sepsis:
  
  • Behaviour:
    
    • No response to social cues
    • Appears ill
    • Does not wake, or if roused does not stay awake
    • Weak, high-pitched and continuous cry
  • Heart Rate:
    
    • Tachycardia (different at different ages)
    • < 60 bpm at any age
  • Respiratory Rate
    
    • Tachypnoea (different at different ages)
    • Grunting
    • Apnoea
    • SpO2 < 90% on air
  • Mottled or ashen appearance
  • Cyanosis of the skin, lips or tongue
  • Non-blanching rash
  • Aged < 3 months with temperature > 38 degrees
  • Temperature < 36 degrees
• Transfer IMMEDIATELY to an acute hospital setting if there are signs of severe illness or if immunity is impaired
• If in the community and meningococcal disease is suspected, give IM benzylpenicillin
• Identify the source of infection
  
  • Investigations include: FBC, blood culture, CRP, urinalysis, LP, CXR
  • Contraindications for LP: signs of raised ICP, focal neurological signs, shock, purpura
  • Perform LP in the following children with suspected sepsis:
    
    • < 1 month
    • 1-3 months who appear unwell
    • 1-3 months with WCC < 5 or > 15 x 109/L

Question 121

What is the management of septicaemia in children with moderate to high risk?

Answer 121

• Carry out VBG for
  
  • Blood gas (including glucose and lactate)
  • Blood culture
  • FBC
  • CRP
  • Urea and electrolytes
  • Creatinine
• Review venous lactate within 1 hour
• Lactate > 2 mmol/L or evidence of AKI
  
  • Treat as HIGH RISK
• Lactate < 2 mmol/L
  
  • Repeat structured assessment at least hourly
  • Ensure review by senior clinician within 3 hours of meeting 2 or more of the moderate to high risk criteria
  • Once the cause is identified, manage it

Question 122

What is the management of septicaemia in children with high risk?

Answer 122

• Arrange immediate review by senior clinician
• Carry out VBG for:
  
  • Blood gas (including glucose and lactate)
  • Blood culture
  • FBC
  • CRP
  • Urea and electrolytes
  • Creatinine
  • Clotting screen
• Give broad spectrum antibiotics at the MAXIMUM dose without delay
• Monitor children continuously
• Monitor mental state of the child using GCS or AVPU
• Lactate > 4 mmol/L
  
  • Give IV fluid bolus without delay
  • Refer to critical care for review of central access and initiation of inotropes or vasopressors
• Lactate 2-4 mmol/L
  
  • Give IV fluid bolus without delay
• Lactate < 2 mmol/L
  
  • Consider IV fluids
• Antibiotics
  
  • If meningococcal sepsis
    
    • IM benzylpenicillin (in the community)
    • IV ceftriaxone (in hospital)
  • Follow local guidelines for other infections
  • Neonates presenting within the first 72 hours of life should receive IV benzylpenicillin + gentamicin
• IV fluid

Question 123

What is the paediatric sepsis 6?

Answer 123

• Give high flow oxygen
• Obtain IV/IO access and take bloods
  
  • Blood gas and lactate (FBC, U&E, CRP)
  • Blood glucose (treat hypoglycaemia)
  • Blood cultures
• Give IV/IO antibiotics
• Consider fluid resuscitation
  
  • Aim to restore normal physiological parameters (urine output > 0.5 ml/kg/hr)
  • Give 10-20 ml/kg isotonic fluid over 5-10 mins
  • Repeat as necessary, monitor urine output
• Involve senior clinicians early
• Consider inotropic support early
  
  • If normal physiological parameters are not restored after > 40 ml/kg fluids, consider ICU admission

Question 124

What are causes of nappy rash?

Answer 124

• Common
  
  • Irritant (contact) dermatitis
  • Infantile seborrhoeic dermatitis
  • Candida infection
  • Atopic eczema
• Rare
  
  • Acrodermatitis enteropathica
  • Langerhans cell histiocytosis
  • Wiskott-Aldrich syndrome

Question 125

What is the most common type of nappy rash?

Answer 125

• Irritant dermatitis is the MOST COMMON type of nappy rash
  
  • This can occur if the nappy is not changed frequently but it can also happen even with regular cleaning of the nappy area
  • It tends to be due to the irritant effects of urine on the skin of susceptible infants
  • The flexures tend to be spared in irritant dermatitis causing nappy rash (this distinguishes it from other causes of nappy rash)
  • The rash is erythematous and may have a scalded appearance
• MANAGEMENT: mild cases respond to the use of protective emollient
  
  • More severe cases may require topical corticosteroids

Question 126

What is the management of nappy rash?

Answer 126

• Advise the parents/carers about self-management strategies
  
  • Consider using a nappy with high absorbency and ensure that it fits properly
  • Leave nappy off as much as possible to help skin drying of the nappy area
  • Clean the skin and change the nappy every 3-4 hours or as soon as possible after wetting/soiling, to reduce skin exposure to urine and faeces
    
    • Use water, or fragrance-free or alcohol-free baby wipes
    • Dry gently after cleaning
    • Bath the child daily
    • Do NOT use soap, bubble bath, lotions or talcum powder
  • ADVICE: NHS choices leaflet on nappy rash
• If mild erythema and child is asymptomatic
  
  • Advise on the use of barrier preparation to protect the skin (available OTC)
  • Apply thinly at each nappy change
  • Options: Zinc and Castor oil ointment BP, Metanium ointment, white soft paraffin BP ointment
• If the rash appears inflamed and is causing discomfort
  
  • If > 1 month = hydrocortisone 1% cream OD (max 7 days)
• If the rash persists and CANDIDAL INFECTION is suspected or confirmed on swab
  
  • Advise against the use of barrier protection
  • Prescribe topical imidazole cream (e.g. clotrimazole, econazole, miconazole)
  • Frequency depends on preparation used
• If the rash persists or BACTERIAL INFECTION is suspected or confirmed on swab
  
  • Prescribe oral flucloxacillin for 7 days
  • If penicillin allergy: clarithromycin (7 days)
• Arrange to review the child

Question 127

What is infantile seborrhoeic dermatitis?

Answer 127

• Tends to present in the first 3 months of life
• It tends to start on the scalp as an erythematous scaly eruption
• The scales form a thick yellow adherent layer, commonly called cradle cap
• The rash may spread to the face and then extend to the flexures and napkin area
• Unlike atopic eczema, this rash is NOT itchy and the child does not seem to be bothered by it
• It is associated with a subsequent risk of developing atopic eczema

Question 128

What is the management of infantile seborrhoeic dermatitis?

Answer 128

• Reassure the parents (it is NOT a serious condition)
• Resolves over a few weeks/months
• If scalp is affected, advise:
  
  • Wash scalp with baby shampoo, followed by gentle brushing with a soft brush to loosen scales and improve the condition of the skin
  • Softening the scales with baby oil first, followed by gentle brushing, then washing off with baby shampoo
  • Soaking the crusts overnight with white petroleum jelly or slightly warmed vegetable/olive oil, and shampooing in the morning
  • Emulsifying ointment can be used if these measures don’t work
• If conservative measures are ineffective, prescribe topical imidazole cream (e.g. clotrimazol, econazole, meconazole)
• Use 2-3 times per day (depending on preparation) until symptoms disappear
• Consider specialist advice if it lasts > 4 weeks
• If other areas of skin are affected, advise bathing the infant at least once per day using an emollient as a soap substitute

Question 129

What is atopic eczema?

Answer 129

• 20% prevalence amongst children in the UK
• Onset is usually within the first year of life
• Most cases will resolve in adolescence

Question 130

How is atopic eczema diagnosed?

Answer 130

• Clinical
• NOTE: if the disease is unusually severe, atypical or associated with unusual infections or faltering growth, an immune deficiency should be excluded

Question 131

What are clinical features of atopic eczema?

Answer 131

• Itching (pruritus)
  
  • This leads to scratching and exacerbation of the rash
  • Excoriated areas become erythematous, weeping and crusted
• Distribution of the eruption changes with age
• Atopic skin is usually dry
• Prolonged scratching and rubbing can lead to lichenification (accentuation of normal skin markings)

Question 132

What are complications of atopic eczema?

Answer 132

• Causes of exacerbation of eczema:
  
  • Bacterial infection
  • Viral infection (e.g. HSV)
  • Ingestion of an allergen (e.g. egg)
  • Contact with an irritant or allergen
  • Environment (heat, humidity)
  • Change or reduction in medication
  • Psychological stress
  • Unexplained
• Skin infection is usually caused by Staphylococcus or Streptococcus
• Staphylococcus aureus, in particular, thrives on atopic skin and releases superantigens, which seem to worsen eczema
• Herpes simplex virus infection, although rare, is potentially very serious because it can spread rapidly on atopic skin causing an extensive vesicular reaction called eczema herpeticum
• Regional lymphadenopathy is common in active eczema and resolves when the skin improves

Question 133

How is eczema managed?

Answer 133

• Assessment of Eczema (Severity)
  
  • CLEAR - normal skin with no evidence of active eczema
  • MILD - areas of dry skin and infrequent itching
  • MODERATE - areas of dry skin, frequent itching and redness (with/without excoriation and localised skin thickening)
  • SEVERE - widespread areas of dry skin, incessant itching and redness (with/without excoriation and localised skin thickening)
  • INFECTED - eczema is weeping, crusting or there are pustules with fever and malaise
  • IMPORTANT: remember to assess the psychological impact of eczema on the child
  • Consider using questionnaires such as the Children’s Dermatology Life Quality Index (CDLQI)
• Separate management depending on severity
• Emollients used in all eczema
• Eczema herpeticum treated with systemic aciclovir
• Dietary elimination
  
  • Food allergy should be suspected in young children with moderate/severe, particularly if associated with gut dysmotility (colic, vomiting, altered bowel habit) or faltering growth
  • MOST COMMON allergens resulting in eczema:
    
    • Egg
    • Cow’s milk
  • Allergen specific IgE antibodies in the blood and skin-prick testing may be helpful
  • Dietary elimination for 4-6 weeks may be necessary to detect a response

Question 134

How is MILD eczema managed?

Answer 134

• Emollient
• Consider prescribing a mild corticosteroid (e.g. hydrocortisone 1%) for areas of red skin
  
  • This should be continued for 48 hours after the flare has been controlled
• Routine follow-up is not normally necessary
• Refer for a routine dermatology appointment if the diagnosis is uncertain, current management has failed to control eczema, there is facial eczema or there is a recurrent secondary infection

Question 135

How is MODERATE eczema managed?

Answer 135

• Emollients used
• Prescribe a moderately potent topical steroid: betamethasone valerate 0.025% or clobetasone butyrate 0.05%
• Treatment should be continued for 48 hours after the flare has been controlled
• If there is severe itching or urticaria, consider prescribing a 1-month trial of a non-sedating antihistamine (e.g. cetirizine, loratidine, fexofenadine)
• Consider prescribing a maintenance regimen of topical corticosteroids to control areas of skin prone to frequent flares (not face, genitals or axillae)
• 2nd line: topical calcineurin inhibitors (e.g. tacrolimus) - only prescribed by a specialist
• Review regularly if the child is young and using a large amount of topical corticosteroids

Question 136

How is SEVERE eczema managed?

Answer 136

• Prescribe a potent corticosteroid: betamethasone valerate 0.1% to be used on inflamed areas
  
  • For delicate areas of inflamed skin (e.g. face and flexures) use a moderate potency steroid
• Occlusive dressings or dry bandages may be useful
• Consider non-sedating antihistamine if there is severe itching
• If the itching is affecting sleep, consider using a sedating antihistamine (e.g. chlorphenamine)
• If there is SEVERE, extensive eczema causing psychological distress, consider a course of oral corticosteroids
• Prescribe a maintenance regimen of topical corticosteroids

Question 137

How is infected eczema managed?

Answer 137

• Swab the infected skin
• 1st line: flucloxacillin
  
  • If penicillin allergy: erythromycin (or clarithromycin)
• If the area of infection is localised, topical antibiotics should be effective
• NOTE: these should NOT be used for longer than 2 weeks

Question 138

What is sudden infant death syndrome?

Answer 138

• The sudden death of an infant <1 year that remains unexplained after a thorough case investigation, including performance of a complete postmortem, examination of the death scene and a review of the clinical history (National Institute of Child Health and
  Development).

Question 139

What is the aetiology of SIDS?

Answer 139

• Many factors have been implicated but none has been proven.
• Prolonged QT interval: Is a marker of reduced cardiac electrical stability and is strongly associated with SIDS. “ Sympathetic activity in these infants may be sufficient to cause fatal arrhythmias such as torsades de pointes.
• Upper Airway Obstruction
  
  1. Infants have sites of anatomical and physiological vulnerability such as a shallow
     hypopharynx and position of the tongue and epiglottis
  2. Infants are obligate nasal breathers for the first few months of life and so prone positioning
     may compress their only airway.
• Central apnoea: Infants can have reflex-like apnoeic responses to a number of conditions such as hypoxia, hypoglycaemia, infection and stimulation of the upper larynx (e.g. GOR). Such apnoeic responses are probably due to incomplete development of the CNS, increased vagal tone and decreased respiratory muscle reserve.
• Thermoregulatory dysfunction: Minor changes in temperature (hot or cold) can induce autonomic dysfunction in infants.
• Brainstem dysfunction: Cardiorespiratory function, autonomic mechanisms, chemoreceptor sensitivity and thermoregulation are all controlled by the medullary
  and related structures of the brain. Autopsy examinations of the brainstems of infants
  with a diagnosis of SIDS have demonstrated hypoplasia or decreased neurotransmitter binding of the arcuate nucleus (medulla).

Question 140

What is the epidemiology of SIDS?

Answer 140

• SIDS is the most common cause of death in infants aged 1 month to 1 year.
• Peak incidence: 1–4 months.
• UK incidence: 300 cases/year. M > F.
• Prevalence: 1.7 cases/1000 live births.
• Seasonal variation: More common during winter.

Question 141

What are features in a history of SIDS?

Answer 141

• Diagnosis of exclusion
• Classic presentation:
  
  1. Usually occurs during hours of extended sleep (10pm–10am)
  2. Child is found dead usually in the position the child was put to bed
  3. Checks whilst the child was asleep usually revealed no problems
  4. Parent may report that the child ‘was not himself or herself’ before going to sleep
  5. May report GI or respiratory infection in the weeks preceding death.
• Alerts for child abuse: Unclear, inconsistent history, unwanted child, poor antenatal/postnatal care, age >6 months.

Question 142

What are signs of SIDS?

Answer 142

• Fully undress the child and note:
  
  1. General condition of child (hygiene, nutrition, growth parameters)
  2. Signs of illness (vomit, hydration, nasal discharge, rash)
  3. Signs of trauma, abuse (see Safeguarding Children chapter) or evidence of bleeding
  4. Clothing (do not discard – should stay with the child).

Question 143

What are investigations for SIDS?

Answer 143

• Blood culture should be obtained in all cases.
• Depending on history and examination, further microbiological samples may be taken (swabs, SPA, LP) or metabolic samples (blood, urine, CSF) may be required.
• Postmortem examination.

Question 144

What advice can be given to prevent SIDS?

Answer 144

• Infants should sleep on their back
• Overheating by heavy wrapping and high room temperature should be avoided (head should be uncovered and the blanked tucked in no higher than the shoulders)
• Infants should be placed in the feet to foot position (feet a the foot of the cot)
• No one should smoke near the infant
• Parents should seek medical advice promptly if their infant becomes unwell
• Parents should have the baby in their bedroom for the first 6 months of life
• Parents should avoid bringing the baby into their bed when they are tired or have had alcohol or drugs
• Parents should avoid sleeping with their infant on the sofa, settee or armchair
• If possible, the infant should be breastfed

Question 145

What are causes of congenital hypothyroidism?

Answer 145

• Maldescent of the thyroid and athyrosis
  
  • MOST COMMON cause of sporadic congenital hypothyroidism
  • In early foetal life, the thyroid migrates from the base of the tongue to its normal site
  • In maldescent, the thyroid will halt at a point along this migration
• Dyshormonogenesis
  
  • Inborn error of thyroid hormone synthesis
• Iodine deficiency
  
  • MOST COMMON cause worldwide
• TSH deficiency
  
  • Usually associated with pituitary dysfunction
  • Manifest with GH, gonadotrophin and ACTH deficiency

Question 146

What are clinical features of congenital hypothyroidism?

Answer 146

• Faltering growth
• Feeding problems
• Prolonged jaundice
• Constipation
• Pale, cold, mottled dry skin
• Coarse facies
• Hoarse cry
• Goitre
• Umbilical hernia
• Delayed development

Question 147

What are clinical features of acquired hypothyroidism?

Answer 147

• Females > males
• Short stature/poor growth
• Cold intolerance
• Dry skin
• Cold peripheries
• Bradycardia
• Thin, dry hair
• Pale, puffy eyes with loss of eyebrows
• Goitre
• Slow-relaxing reflexes
• Constipation
• Delayed puberty/amenorrhoea
• Obesity
• Slipped upper femoral epiphysis
• Poor concentration
• Deterioration in school work
• Learning difficulties

Question 148

What is the management of children with hypothyroidism?

Answer 148

• Thyroxine treatment should be started within 2-3 weeks of age to reduce the risk of impaired neurodevelopment
• Treatment is life-long with oral replacement of thyroxine
• With adequate and early intervention, intelligence and development should be normal

Question 149

What causes acquired hypothyroidism?

Which children have increased risk of acquired hypothyroidism?

Answer 149

• Usually results from autoimmune thyroiditis
• Increased risk in children with:
  • Down syndrome
  • Turner syndrome

Question 150

What causes hyperthyroidism in children?

Answer 150

• Usually results from autoimmune thyroiditis (Graves disease) secondary to the production of thyroid-stimulating antibodies

Question 151

What are clinical features of hyperthyroidism?

Answer 151

• Anxiety, restlessness
• Increased appetite
• Sweating
• Diarrhoea
• Weight loss

Question 152

What is the management of hyperthyroidism?

Answer 152

• Carbimazole or propylthiouracil
• IMPORTANT: carbimazole is associated with a risk of neutropaenia
  
  • Families should be seek urgent medical attention and a blood count if a sore throat or fever occur whilst on treatment
• Beta-blockers for symptomatic relief
• Treatment is usually given for around 2 years
• Other options: radioiodine treatment, surgery
• NOTE: neonatal hyperthyroidism may occur due to the transplacental transfer of TSIs

Question 153

What is the common cold? (Coryza)

Answer 153

• MOST COMMON infection of childhood
• Features include clear or mucopurulent nasal discharge and nasal blockage
• Educate the parents that colds are self-limiting and have no specific curative treatment
• Pain may be treated with paracetamol or ibuprofen
• Cough may persist for up to 4 weeks after a common cold

Question 154

What are the most common pathogens for the common cold?

Answer 154

• Rhinoviruses
• Coronaviruses
• Respiratory syncytial virus (RSV)

Question 155

What is the management of common cold?

Answer 155

• Reassure that the common cold is self-limiting
• Symptoms may peak after 2-3 days and they will typically resolve within 2 weeks
• Encourage rest, adequate fluid intake and a healthy diet
• Encourage the use of paracetamol or ibuprofen for symptomatic relief

Question 156

What is the pathophysiology of pharyngitis?

Answer 156

• In pharyngitis, the pharynx and the soft palate become inflamed and local lymph nodes are enlarged and tender
• Usually due to viral infection (mainly adenoviruses, enteroviruses and rhinoviruses)
• In older children, group A b-haemolytic streptococcus is common

Question 157

What is tonsilitis and its common causes?

Answer 157

• Tonsillitis is a form of pharyngitis characterised by intense inflammation of the tonsils, often with a purulent exudate
• Common causes of tonsillitis:
  • Group A b-haemolytic streptococci
  • EBV

Question 158

What is the difference between viral and bacterial tonsilitis?

Answer 158

• Headache, apathy, abdominal pain, white tonsillar exudate and cervical lymphadenopathy are more common with bacterial infection

Question 159

What is the management of tonsilitis/pharyngitis?

Answer 159

• Arrange hospital admission if:
  
  • Difficulty breathing
  • Clinical dehydration
  • Peri-tonsillar abscess or cellulitis
  • Signs of marked systemic illness or sepsis
  • A suspected rare cause (e.g. Kawasaki disease, diphtheria)
• Specific cases to watch out for:
  
  • DMARDs - could cause immunodeficiency
  • Carbimazole - can cause idiosynchratic neuropaenia
  • NOTE: take urgent FBC in both cases
• Medical Management (if bacterial tonsillitis is confirmed using rapid antigen testing)
  
  • Phenoxymethylpenicillin
  • If penicillin allergy: clarithromycin
• To eradicate the organism completely, 10 days of antibiotic treatment is required for pharyngitis and tonsillitis
• RARELY, children may be admitted to hospital for IV fluids and analgesia if they are unable to swallow solids or liquids
• WARNING: amoxicillin should be avoided because it may cause a widespread maculopapular rash if the tonsillitis is due to infectious mononucleosis

Question 160

What advice should be given to those pharyngitis/tonsilitis?

Answer 160

• Adequate fluid intake
• Paracetamol or ibuprofen when necessary
• Salt water gargling, lozenges or anaesthetic sprays (e.g. Difflam) may provide temporary relief of throat pain
• Children can return to school after fever has resolved and they are no longer feeling unwell and/or after taking antibiotics for 24 hours
• Patients with recurrent tonsillitis may require referral to ENT

Question 161

What is Scarlet fever?

Answer 161

• Group A streptococcal infection can result in Scarlet Fever
• Fever usually precedes the presence of headache and tonsillitis by 2-3 days
• Appearance of the rash is variable
• The typical rash will be a ‘sandpaper-like’ maculopapular rash with flushed cheeks and perioral sparing
• The tongue is often white and coated and may be sore or swollen

Question 162

What is the management of Scarlet fever?

Answer 162

• Antibiotics
  
  • Phenoxymethylpenicillin (penicillin V) - 4/day for 10 days
  • Azithromycin (if penicillin allergy)
• Paracetamol or ibuprofen can be given for symptomatic relief
• NOTIFIABLE disease so inform local PHE centre)
• Symptoms should resolve after 1 week
• Stay away from nursery/school for 24 hours after starting antibiotics
• This is needed to prevent complications such as acute glomerulonephritis and rheumatic fever

Question 163

What are complications of Scarlett fever?

Answer 163

• Suppurative: otitis media, throat infection/abscess, acute sinusitis/mastoiditis, streptococcal pneumonia, meningitis, endocarditis, osteomyelitis, necrotising fasciitis, toxic shock syndrome
• Non-suppurative (autoimmune): rheumatic fever (arthritis, carditis, erythema marginatum), streptococcal glomerulonephritis

Question 164

What is acute otitis media?

Answer 164

• Most children have at least one episode of acute otitis media
• Most common: 6-12 months
• Infants and young children are susceptible to otitis media because their Eustachian tubes are short, horizontal and function poorly

Question 165

What is the presentation of acute otitis media?

Answer 165

• Ear pain and fever
  
  • IMPORTANT: every child with a fever MUST have their tympanic membrane examined
• In acute otitis media, the tympanic membrane is bright red and bulging with loss of the normal light reflection
• Occasionally ,the tympanic membrane can perforate and pus can become visible in the external canal

Question 166

What are causative pathogens of acute otitis media?

Answer 166

• RSV
• Rhinovirus
• Pneumococcus
• Haemophilus influenzae
• Moraxella catarrhalis

Question 167

What are complications of acute otitis media?

Answer 167

• Mastoiditis

- Meningitis

Question 168

What is the management of acute otitis media?

Answer 168

• Admit if:
  
  • Severe systemic infection
  • Complications (e.g. meningitis, mastoiditis, facial nerve palsy)
  • Children < 3 months with a temperature > 38 degrees
• ADVICE
  
  • Advise that the usual course of acute otitis media is about 3 days but can last up to 1 week
  • Advise regular doses of paracetamol or ibuprofen for pain
  • There is no evidence to support the use of decongestants or antihistamines
• Medical Management
  
  • No antibiotic prescription - most cases will resolve spontaneously. Advise to seek help if the symptoms haven’t improved after 3 days or if the child deteriorates clinically
  • Back-up antibiotic prescription - advise that the antibiotic is NOT needed immediately but should be used if the symptoms have not improved after 3 days or if they have worsened significantly at any time
  • Immediate antibiotic prescription - seek medical help if the symptoms worsen rapidly or the patient becomes systemically unwell
    
    • Amoxicillin - 5-7 days is first-line
    • Clarithromycin or erythromycin can be used if penicillin allergic
  • NOTE: antibiotics marginally reduce the duration of the pain but have no effect on risk of hearing loss

Question 169

What can recurrent ear infections lead to?

Answer 169

• Can lead to otitis media with effusion (aka glue ear)
  
  • Children are usually asymptomatic except for possible reduced hearing
  • The eardrum is dull and retracted, often with a fluid level visible
  • This is common in 2-7 year olds
  • Usually resolves spontaneously
  • It may cause conductive hearing loss (most common cause in children)
    
    • This can interfere with normal speech development and result in learning difficulties
• In these children, ventilation tubes (grommets) are often inserted
  
  • The benefits do NOT last longer than 12 months
  • If problems recur after the extrusion of the grommet, reinsertion of grommets with adjuvant adenoidectomy is often advocated

Question 170

What is Sinusitis?

Answer 170

• Infection of the paranasal sinuses may occur with viral URTIs
• Occasionally, you might get a secondary bacterial infection
• The frontal sinuses are rarely affected because the frontal sinuses do not develop until late childhood
• Antibiotics and analgesia may be used for acute sinusitis

Question 171

What is the management of sinusitis if symptoms last >10 days?

Answer 171

• Refer to hospital if there are symptoms and signs of:
  
  • Severe systemic infection
  • Intraorbital or periorbital problems (e.g. periorbital cellulitis, displaced eyeball, double vision)
  • Intracranial complications (e.g. features of meningitis)
• Symptoms lasting < 10 days
  
  • Do NOT offer an antibiotic
  • ADVICE
    
    • Acute sinusitis is usually caused by a virus and takes 2-3 weeks to resolve
    • It can be complicated by bacterial infection (2% risk)
      
      • Symptoms, such as fever, can be managed using paracetamol or ibuprofen
  • Some people may find some relief using nasal saline or nasal decongestants
  • Medical advise should be sought if symptoms worsen rapidly, if they do not improve in 3 weeks or become systemically unwell

Question 172

What is the management of sinusitis if symptom lasts >10 days?

Answer 172

• Refer to hospital if there are symptoms and signs of:
  
  • Severe systemic infection
  • Intraorbital or periorbital problems (e.g. periorbital cellulitis, displaced eyeball, double vision)
  • Intracranial complications (e.g. features of meningitis)
• Symptoms lasting > 10 days
  
  • Consider high-dose nasal corticosteroid for 14 days for adults and children > 12 years old (e.g. mometasone)
    
    • May improve symptoms but unlikely to affect duration of illness
    • Could cause systemic side-effects
  • Consider NO antibiotic prescription or back-up prescription
    
    • Antibiotics are unlikely to change the course of the illness
    • The back-up prescription should be used if symptoms get considerably worse of it is still has not improved by 7 days
    • 1st line: phenoxymethylpenicillin
      
      • NOTE: clarithromycin if penicillin allergy
    • 2nd line: co-amoxiclav
  • Advise patients to seek medical advice if they develop complications or their symptoms don’t improve/worsen
  • Consider alternative diagnoses such as dental infection
  • ADVICE: NHS Choices information on Sinusitis

Question 173

What is a UTI?

Answer 173

• Symptomatic bacteriuria: Presence of urine bacteria which is not a contaminant of urethral flora with concomitant pyuria. Broad clinical categories: upper UTI (acute pyelonephritis) and lower UTI (cystitis, urethritis).
• Asymptomatic bacteriuria: Detection of incidental bacteriuria in an asymptomatic child.

Question 174

What is the aetiology of a UTI?

Answer 174

• General: Proliferation of bacteria in the urinary tract, ascending infections secondary to bacteria in the periurethral flora and distal urethra. F > M as short urethra and closer proximity of perianal colonic organisms
• Neonates: 70% ascending infection, 30% are of haematogenous origin.
• Infants, children, adolescents: Majority are ascending infections.

Question 175

What organisms are responsible for UTIs?

Answer 175

• Gram-negative bacteria: Escherichia coli (90%), Streptococcus faecalis and Klebsiella species from the child’s faecal flora
• Proteus (males; present under the prepuce)
• Staphylococcus saprophyticus (common in adolescent girls)
• Pseudomonas (usually in children with congenital urinary tract anomalies (see chapter) or acquired renal problems, e.g. stones)
• Adenovirus 11 and 12 in haemorrhagic cystitis.

Question 176

What is an atypical UTI?

Answer 176

• Seriously ill child, poor urine flow, abdominal/bladder mass, increased creatinine, septicaemia, failure to respond to treatment <48, non-E. coli infections.

Question 177

What are recurrent UTIs?

Answer 177

• 2 + acute pyelonephritis/upper UTIs, 1 upper with 1 + lower UTI episodes, 3 + lower UTI episodes

Question 178

What are clinical features of UTIs in infants?

Answer 178

• Fever
• Vomiting
• Lethargy or irritability
• Poor feeding/faltering growth
• Jaundice
• Septicaemia
• Offensive urine
• Febrile seizure (> 6 months)

Question 179

What are clinical features of UTIs in children?

Answer 179

• Dysuria, frequency and urgency
• Abdominal pain or loin tenderness
• Fever with or without rigors
• Lethargy and anorexia
• Vomiting, diarrhoea
• Haematuria
• Offensive/cloudy urine
• Febrile seizure
• Recurrence of enuresis (bed wetting)

Question 180

What are investigations for UTIs?

Answer 180

• Urine collection: All infants and children presenting with an unexplained pyrexia >38 should have a urine sample tested <24 hours. ‘Clean-catch’ sample/urine collection pads/suprapubic aspiration/mid-stream urine sample (MSU) dependent on age. Adhesive plastic bags/cotton wool balls may be contaminated with faecal +/- genital flora and are therefore not used.
• Urine dipstick: May be diagnostic; Leucocyte esterase + ve/nitrite + ve (UTI). Leucocyte esterase - ve/nitrite + ve (suspected – treat as UTI). Leucocyte esterase + ve/nitrite -ve (suspected – await MC&S unless clinically apparent). Leucocyte esterase - ve/nitrite + ve (no UTI).
• Urine MC&S: Pyuria + ve/bacteriuria - ve (UTI). Pyuria + ve/bacteriuria - ve and pyuria - ve/
  bacteriuria + ve (suspected – treat as UTI). Pyuria -ve/bacteriuria - ve (no UTI).
• Bloods: Possible increased WCC, increased CRP, U&Es.
• Radiological investigation

Question 181

What is the management of UTI?

Answer 181

• ALL infants < 3 months of age with suspicion or a UTI or if seriously ill should be referred IMMEDIATELY to hospital
  
  • IV antibiotics (e.g. co-amoxiclav) for at least 5-7 days
  • This should be followed by oral prophylaxis
• Infants aged > 3 months and children with acute pyelonephritis/upper UTI
  
  • If urine dipstick is positive for either leucocyte esterase OR nitrites, send a urine sample for culture and start antibiotic therapy
  • Features of pyelonephritis:
    
    • Bacteriuria + fever > 38 degrees
    • Bacteriuria + loin pain/tenderness
  • Oral antibiotics (e.g. trimethoprim for 7 days)
    
    • NOTE: choice of antibiotic should be based on resistance patterns
  • If oral cannot be used, give IV antibiotics (e.g. co-amoxiclav for 2-4 days, followed by oral antibiotics for 7-10 days)
  • NOTE: the choice of antibiotic will vary depending on sensitivities
• Children with cystitis/lower UTI
  
  • Features of cystitis/lower UTI:
    
    • Dysuria but NO systemic symptoms
  • Oral antibiotics (e.g. trimethoprim or nitrofurantoin) for 3 days
  • ADVISE
    
    • Seek medical attention if the child is still unwell after 24-48 hours of antibiotic treatment
    • Encourage adequate fluid intake
• Infants and children with atypical UTI should have an ultrasound of the urinary tract to identify structural abnormalities
• DMSA and MCUG may also be performed in children < 6 months presenting with atypical or recurrent UTI

Question 182

What are medical measures to prevent UTIs?

Answer 182

• AIM: ensure washout of organisms that ascend into the bladder from the perineum and reduce the presence of aggressive organisms in the stool, perineum and under the foreskin
• High fluid intake to produce high urine output
• Regular voiding
• Ensure complete bladder emptying
• Treatment and/or prevention of constipation
• Good perineal hygiene
• Lactobacillus acidophilus - probiotic to encourage colonisation of the gut by this organism and reduce the number of pathogenic organisms
• Antibiotic prophylaxis (controversial)

Question 183

How should children with recurrent UTIs, renal scarring or reflux be followed up?

Answer 183

• Urine should be dipsticked with any non-specific illness in case it is caused by a UTI and urine should be sent for MC&S
• Long-term low-dose antibiotic prophylaxis can be used
• Circumcision in boys may be considered
• Anti-VUR surgery may be considered if there is progression of scarring
• Regular blood pressure checks if renal defects are present
• Urinalysis to check for proteinuria (suggestive of progressive CKD)
• Regular assessment of renal growth and function

Question 184

What are complications of UTIs?

Answer 184

• Chronic pyelonephritis, chronic renal failure (CRF) and hypertension (3%).
• VUR accounts for CRF in 20% of children and 5–10% in adults.

Question 185

What is the prognosis of UTIs?

Answer 185

• Infants who present with first symptomatic UTI aged <1 year are significantly more at risk of having recurrence (30% risk). Preschool presentation of a UTI has a recurrence of 12%