Paeds new Flashcards
Pneumonia defintion?
- inflammation of the lung parenchyma (the part of lungs involved in gas transfer e.g. alveoli + resp bronchioles)
- congestion caused by viruses or bacteria or irritants
Causes of pneumonia in children?
Viral more common
Viral: RSV, adenovirus, rhinovirus, influenza
Bacterial: Strepmpneumoniae, Hib
Presentation of pneumonia in children
- temperature (over 38.5)
- rapid breathing/difficulty breathing
cough - chest pain
- vomiting
- decreased activity
- loss of appetite/poor feeding
Investigations for paediatric pneumonia?
- Sputum sample (can be difficult)
- Blood cultures.
- CXR: look for consolidation.
Describe the treatment of pneumonia.
PO amoxicillin.
Co-amoxiclav if complicated or unresponsive.
O2, analgesia, IV fluids if indicated.
Bronchiolitis:
- causative agent?
- age?
- RSV
- Babies 0-2
Investigations for bronchiolitis?
Nasopharyngeal aspirate or throat swab
RSV rapid testing and viral cultures
FBC
Home or hospital for bronchiolitis?
Hospital if severe apnoea/resp distress: grunting, RR>70, central cyanosis, sats<92%,
Consider hospital if rr>70, inadequate fluids, clinical dehydration
No role for antibiotics, steroids or bronchodilators
What is asthma?
Chronic obstructive resp disease characterised by episodic exacerbations of bronchoconstriction
Presentation of asthma?
- Cough - nocturnal
- SOB
- wheeze/whistling
- chest congestion/tightness
Diagnosis of asthma in children?
- If under 3: may use wait and see approach
- If under 5: need to go off the history
- If over 5: spirometry, PEF
Acute management of asthma?
- Oxygen if needed
- SABA
- Prendisolone 1mg/kg IV
If no improvement:
- IV salbutamol bolus
- Aminophylline/MgSO4/salbutamol infusion
Long-term asthma management in under 5s?
- SABA for wheeze episodes (salbutamol prn)
- ICS (beclametasone)
- Leukotrine-receptor agonist (montelukast)
- stop LTRA and refer to asthma specialist
List three possible side effects of inhaled corticosteroids
- Adrenal suppression
- Growth suppression
- Osteoporosis (although this has not been shown to affect bone fractures)
Wheeze vs stridor?
Wheeze = expiratory, polyphonic
Inhalers: name 2 ‘preventers’.
ICS act as ‘preventers’ e.g. beclamethasone, budenoside.
Inhalers: name 2 ‘relievers’.
Beta agonists e.g. salbutamol.
Muscarinic antagonists e.g. ipratropium bromide.
Why might asthma treatment fail in children?
- Adherence.
- Wrong diagnosis.
- Environmental factors.
- Choice of drug.
- Bad disease.
Non-resp causes of wheeze?
- GORD
- bronchomalacia
- cystic fibrosis
What constitutes the upper airway?
Nose, pharynx, larynx
Name 3 URTI.
- Rhinitis.
- Otitis media.
- Pharyngitis.
- Tonsillitis.
- Laryngitis.
Name 3 LRTI.
- Bronchitis.
- Croup.
- Epiglottitis (bacterial).
- Tracheitis.
- Bronchiolitis.
- Pneumonia.
Would you expect a patient with bronchitis or with bronchiolitis to be hypoxic and tachypnoeic? Explain why.
Bronchiolitis.
Bronchiolitis affects the respiratory portion of the airway, where gas exchange takes place therefore you may see hypoxia and tachypnoea.
Bronchitis affects the conducting portion of the airway and so is unlikely to have these effects.
Name 4 LRTI that could be caused by RSV.
- Acute bronchiolitis.
- Wheezy bronchitis.
- Asthma exacerbation.
- Pneumonia.
- Croup.
Why are infants more susceptible to descending infection?
Infants have a poor innate immune response and so are more susceptible to descending infections.
What is croup?
Acute larngotracheobronchitis.
Describe the cough that is associated with croup.
A barking seal like cough - often worse at night.
How do you treat croup?
usually self-limiting
can give steroids e.g. dexamethosone, beclamethasone.
Describe the aetiology of recurrent wheeze.
- Persistent infantile wheeze.
- Viral episodic wheeze.
- Asthma.
What is persistent infantile wheeze normally associated with/exacerbated by?
Persistent infantile wheeze tends to affect the small airways. It is associated with parental smoking or post-viral infection.
Are inhalers likely to help a child with persistent infantile wheeze?
No. Inhalers are unlikely to help; symptoms will improve as the child gets older.
Are inhalers likely to help a child with viral episodic wheeze?
Bronchodilators may help but there is no benefit from inhaled steroids.
Symptoms are likely to improve with age.
Viral induced wheeze:
- common causes?
- affects which age?
- RSV, rhino virus
- children aged 2-5
VIW vs bronchiolitis?
Bronchiolitis: babies, 3-5 days gradual build up
VIW: 2-5 yrs, viral prodrome for 1-2 days then suddenly worsens
How to manage a viral induced wheeze?
Reliever inhaler for the minority=SABA=salbutamol
If SABA 2-10 puffs PRN to max 4 hourly via a spacer
Otherwise, supportive/comfort care
How to identify viral induced wheeze (how does it differ from asthma)?
- No wheeze with exercise
- No interval symptoms
- No excess of atopy
- No benefit from regular inhaled steroids
- No wheeze except with viral infections
Pathaphysiology of cystic fibrosis
Autosomal recessive
Reduced airway surface liquid impedes mucus clearance allowing excessive bacterial growth
Pancreas: duct occluded in utero leading to pancreatic insufficiency (can lead to CF-related DM)
GI: increased mucus can cause meconium ileus
Biliary tree: can have cholestasis leading to neonatal jaundice
Neonatal test to identify CF
Heel prick- Guthrie test
Investigations for CF:
Sweat chloride over 60mmol/L
CXR, sweat test, glucose tolerance test, microbiology, LFT, coag, bone profile, PFTs- all generally involved in annual assessments
Name 2 respiratory illnesses that can present with stridor.
- Croup - often a louder stridor.
- Acute epiglottitis - often a quieter stridor due to inflamed epiglottis blocking oesophagus and trachea.
Why are children more at risk of epiglottitis than adults?
Epiglottis is floppier, broader, longer and angled more obliquely to trachea and have a larger tongue
Therefore higher risk of acute airway obstruction
Aetiology of epiglottitis?
Reduced since the Hib vaccination
Normally Haemophilus infleunzae and Streptococcus pneumoniae which locally invade
V rarely due to trauma or non-infectious causes
Presentation of epiglottitis
- S.O.B
- Drooling
- Difficulty swallowing (dysphagia)
- Muffled voice (dysphonia)
- Typically no cough
- Stridor is a late sign
Describe the immediate management for acute epiglottitis.
Secure the airway - anaesthetist, ENT surgeon.
Management of epiglottitis
Oxygen, nebulised adrenaline, IV antibiotics (3rd gen cephalosporins eg ceftriaxone), IV steroids, nil by mouth until airway improved
Name a bacteria that causes acute epiglottitis.
H.influenzae B.
Acute epiglottitis is a severe acute illness.
You do a lumbar puncture and find raised proteins and low glucose. Is this likely to be due to a bacterial or a viral infection?
Bacterial.
Give 3 potential consequences of hearing loss.
- Speech and language delay.
- Social problems e.g. behavioural issues.
- Academic underachievement.
How does hearing loss in children often present?
- Parental concern.
- Speech, behavioural or educational problems.
- Incidentally on screening.
What are the 3 types of hearing loss?
- Conductive hearing loss.
- Sensori-neural hearing loss.
- Mixed.
Give 3 causes of conductive hearing loss.
- Glue ear.
- Ear wax.
- Otitis media.
- Perforated ear drum.
Describe the management of conductive hearing loss.
Conductive hearing loss is usually ENT managed:
- Wait and wait - most will resolve on their own.
- Grommet insertion.
- Temporary hearing aid.
Give 3 risk factors for sensori-neural hearing loss.
- Family history.
- SCBU.
- Consanguinity.
Describe the management of sensori-neural hearing loss.
Sensori-neural hearing loss is often managed by a paediatrician. Treatments involve hearing aids or cochlea implants.
How would you manage mixed hearing loss?
You would address the conductive problem first and then offer a hearing aid.
When is hearing tested in children?
- New-born hearing screen.
- School entry hearing test.
- Long term monitoring is done in high risk groups.
Is the new-born hearing screen an objective or subjective test?
It is an objective test - response or no response.
If there are concerns, the patient is followed up with evoked response audiometry.
What are the 3 aims of hearing testing in children?
- Measure hearing threshold (dB).
- To be frequency specific (Hz).
- Obtain single ear information if possible.
Name 4 types of subjective hearing testing.
- Behavioural observational audiometry.
- Distraction testing.
- Visual reinforcement audiometry.
- Performance testing and play audiometry.
Name 2 bacterial and 2 viral organisms that can cause acute otitis media.
Bacterial: S.pneumoniae., H.influenzae.
Viral: RSV, rhinovirus
come from nasopharyngeal area through eustachian tube
Give 3 symptoms of acute otitis media.
- Severe pain lasting weeks
- Coryzal symptoms
- Generally unwell.
- Otorrhoea.
Otitis media investigations?
- ALWAYS test function of facial nerve on examination
- Otoscopy
- Discharge sent for microscopy and culture
Describe the treatment for acute otitis media.
Watch and wait - most spontaneously resolve in 24 hrs
Analgesia.
If recurrent, offer antibiotics and consider a grommet.
Give 2 potential complications of acute otitis media.
- Extra-cranial: mastoiditis, TM perforation.
- Intra-cranial: meningitis, abscess.
What is the function of a grommet?
A grommet keeps the middle ear aerated and prevents the accumulation of fluid in the middle ear.
When might a grommet be indicated?
- Recurrent AOM.
- Chronic otitis media + effusion (glue ear)
- ET dysfunction.
What is the common name for otitis media + effusion?
Glue ear.
What causes glue ear?
Infection!
45% follow AOM.
Presentation of glue ear? Examination findings?
Difficulty hearing and pressure sensation
Examination: TM dull and light reflex is lost
Pathophysiology of glue ear?
inflammatory fluid build up leading to conductive hearing impairment via chronic inflammatory changes and eustachian tube dysfunction
Give 3 risk factors for glue ear.
- Bottle fed
- paternal smoking
- atopy
- genetic disorders egCF and Downs
Causes of deafness in children? (infections)
- Temporary=OME
- Meningitis, mumps and measles
Aetiology of tonsillitis
Viral mostly
Bacterial-group A strep most common (S.pyogenes)
Most common in age 5-15
Tonsillitis- bacterial vs viral assessment
Centor score:
Age, exudate, tender/swollen anterior cervical LNs, temp over 38, absent cough
Management of tonsillitis
Viral is self limiting
Bacterial- should swab for culture and commence antibiotics (usually benzylpencillin)
What are the criteria for considering a tonsillectomy?
- >7 episodes of acute tonsilitis in a year.
- OSA or sleep-deprived breathing.
What is the epidemiology of periorbital cellulitis?
Peak in under 10s
Males twice as commonly affected
Peak occurrence in later winter and early spring
What are the causes of periorbital cellulitis?
S. pneumoniae and S. aureus
How to manage periorbital cellulitis?
If mild preseptal can do from home with broad spectrum empirical abx
If more extensive- hospital, IV abx, supportive therapy
If large abscesses, intracranial complications at px and in frontal sinusitis, need urgent drainage
Complications of periorbital cellulitis?
- Visual related: (vision loss is 11%) start to lose red colour vision (sign of optic nerve compromise)
- Neurological complications: sepsis, intracranial abscess, cavernous sinus thrombosis
Describe the types of manifest and latent strabismus
Manifest:
Esotropia=eye turns in
Exotropia=eye turns out
Hypertropia=eye goes up
Hypotropia=eye goes down
Latent:
Esophoria=inwards under occluder
Exophoria=outwards under occluder
Hyperphoria=up under occluder
Hypophoria= down under occluder
what tests to identify strabismus
Cover test (manifest squint) Cover/uncover test (latent squint)
What shape lens for myopia? For hypermetropia?
Myopia/short sighted= concave
Hypermetropia/long sighted=convex
Causes of microcytic anaemia?
- Iron deficiency
- thalassaemia
- chronic inflamm
Two microcytic anaemias and how you could tell them apart?
Iron-deficiency anaemia (high TIBC on iron profile)
Thalasaemia (low TIBC on iron profile)
What is the most common cause of anaemia in children?
Iron deficiency.
Why are kids more susceptible to iron deficiency anaemia than adults?
30% of their iron comes from diet and 70% from recycled rbcs (whereas adults have 5% from diet and 95% from recycling)
Have higher expenditure
Give 3 signs of anaemia in children.
- Pallor.
- Irritable.
- Lethargy.
- SOB.
- Tachycardic
- Murmur
- Poor growth
Risk factors for iron-deficiency anaemia in infants and children?
Infants = maternal iron deficiency, premature, low birth weight, multiple pregnancy, exclusively breastfed after 6 months
Children=veggie/vegan, GI disorders, chronic blood loss
What is the treatment for iron deficiency anaemia?
Side effect?
- Diet advice, ferrous sulphate tablets
- Constipation
Why does the treatment for iron deficiency anaemia sometimes fail?
Non-compliance.
Give 3 signs of beta thalassaemia major.
- Severe anaemia.
- Jaundice.
- Splenomegaly.
- Failure to thrive.
- Present at 6months+ (when adult Hb takes over)
Describe the management of beta thalassaemia major.
- Genetic counselling.
- Blood transfusions with iron chelation to prevent overload
Long term sequelae of untreated thalassaemia major?
Progressive severe anaemia
Try to compensate with bone marrow expansion and extramedullary haematopoiesis
What may carriers of thalassaemia have?
Mild microcytic hypochromic anaemia
Which type of thalassaemia is generally incompatible with life?
alpha major (4/4 genes missing to make alpha chain)
What does a low reticulocyte count indicate?
A production problem e.g. non-haemolytic.
Infection
Chronic inflamm
Bone marrow malignancy
Bone marrow failure (aplastic)
What does a high reticulocyte count indicate?
A degradation problem e.g. bleeding or haemolysis.
What are some types of haemolytic anaemia?
Intrinsic to RBC:
- sickle cell
- enzyme deficiencies: G6PD, pyruvate kinase
- spherocytosis
Extrinsic:
- auto and alloimmune
- DIC
- TTP
- malaria
How do haemolytic anaemias present?
- Splenomegaly
- Cholecystitis/gall stones
- Jaundice (hyperbilirubinaemia)
- Increased lactate dehydrogenase
- Leg ulcers
Describe the molecular structure of haemoglobin.
Four protein chains (globin) each surrounding a non-protein group (heme).
What is the difference between adult and fetal haemoglobin?
Adult Hb: the four protein chains are made up 2 alpha and 2 beta chains.
Feta Hb: the four protein chains are made up of 2 alpha and 2 gamma chains.
Describe the inheritance pattern seen in sickle cell disease.
Autosomal Recessive.
When do symptoms of sickle cell disease start to present? WHy?
Between 3 and 6 months as HbF levels fall
Pathophysiology of sickle cell disease?
Sickling causes deformed and easily destroyed rbcs= occlusion of microcirculation and chronic haemolytic anaemia
Give 3 consequences of sickle cell disease.
- Life expectancy 20 years younger
- Anaemia.
- Infection.
- Painful crises.
- Stroke.
- Acute chest infection/infarction.
- Aplastic crises
- Splenic sequestration.
What is the affect of sickle cell disease on Hb and reticulocyte count?
Low Hb.
Raised reticulocyte count.
Describe the management for sickle cell disease.
- Hydroxycarbamide.
- Transfusions.
- Stem cell transplants.
-prophylactic penicillin + pneumococcal, influenza and meningococcal vaccines to prevent infections
How does hydroxycarbamide help in sickle cell disease?
Increases foetal haemoglobin production
What is the advantage of being a sickle-cell carrier?
Protected against malaria.
Why are children with sickle cell more predisposed to infection?
How is this prevented?
Microvascular occlusion leads to splenic infarction leads to increased susceptibility to infection, specifically bacterial sepsis.
Prevented by:
-prophylactic penicillin
-pneumococcal, influenza and meningococcal vaccines.
What are some triggers for a painful crisis in sickle cell?
cold
dehydration
infection
hypoxia
What is acute chest syndrome in sickle cell?
Occurs after painful crises.
Presents with pain, resp distress, hypoxia, chest X ray signs.
How can you spot aplastic crises in a child with sickle cell?
- very anaemic
- low reticulocyte count (normally high in sickle cell)
Cause of aplastic crises in sickle cell?
Parvovirus B19 (slapped cheek)
Most common hereditary/intrinsic haemolytic anaemia in Europeans?
Spherocytosis.
How can spherocytosis be treated?
Splenectomy - can increase RBC survival.
Describe the inheritance pattern seen in hereditary spherocytosis.
Autosomal Dominant.
Describe the inheritance pattern for G6PD?
X-linked recessive
What can trigger haemolysis in G6PD?
- eating broad beans
- infection
- antimalarials
What is haemolytic disease of the newborn?
transplacental maternal antibodies causing alloimmune haemolysis of foetal rbcs
Most commonly due to rhesus alloimmunisation (Rh + rbcs from foetus enter rh - maternal blood circulation)
Why does haemolytic disease of newborn occur then and not in utero?
In utero, bilirubin cleared by placenta
In neonate, liver does clearing and can’t handle high bilirubin load
Presentation of haemolytic disease of the newborn?
Jaundice
Pallor
Hepatosplenomegaly
Severe= oedema, ascites, petechiae
What anaemia will haemolytic disease of newborn present as?
Normocytic. Increased reticulocyte count
Management of haemolytic disease of newborn?
50%= normal Hb and bilirubin and need monitoring for late onset anaemia at 6-8 weeks 25%= mod disease and may require transfusion 25%= severe disease= stillborn or have hydrops fetalis- require immediate resus, temp stabilisation, exchange transfusion.
What is fanconi anaemia?
X-linked/autosomal recessive condition
Bone marrow failure, solid tumours, leukaemia
How to manage fanconi anaemia?
Treat specific symptoms in each patient
Only curative= stem cell transplant
Cancer treatment
Surgery for skeletal malformations
What are the liver dependent clotting factors?
10, 9, 7, 2 = “1972”
Name 2 coagulopathies.
- Haemophilia.
- Von Willebrand disease
what factor is deficient in haemophilia a
clotting factor 8 (classic haemophilia, most common)
Describe the inheritance pattern seen in haemophilia.
X linked recessive.
what factor is deficient in haemophilia b
clotting factor 9 (also known as christmas disease)
how does severe haemophilia present?
- Easy bruising
- Mouth bleeds
- Haematomas
- Joint bleeds
- Increased APTT
how does mild/mod haemophilia present?
Delayed presentation until following trauma or bleeds with surgery/dental extractions
Management of haemohpilia?
Factor VIII for haemophilia a
Factor IX for haemophilia b
How does von willebrand factor work?
Assists in platelet plug formation by attracting circulating platelets and binds to CF VIII, preventing its clearance from the plasma
Presentation of Von Willebrand disease?
Bleeding tendency from mucosa eg epistaxis/menorrhagia, spontaneous bleeding
How to manage von willebrand disease?
tranexamic acid and desmopressin
or
concetrates with vwf or factor VIII-vwf
Testing for 1st degree relatives
When does immune thrombocytopenia occur?
Commonly after viral infection or sometimes after immunisation
What diagnosis would you suspect in a child with a single figure platelet count but is otherwise well?
ITP.
They would have a normal blood film and clotting, just very low platelets.
Management of ITP?
- Most children will not need treatment and recover spontaneously in weeks-months.
- Avoid NSAIDs and aspirin
Give 3 signs of thrombocytopenia.
- Petechial rash.
- Bruising.
- Bleeding.
Give 2 causes of thrombocytopenia in children.
- ITP.
- Marrow failure.
What can trigger acute ITP?
Viral infection.
How to manage fanconi anaemia?
Treat specific symptoms in each patient
Only curative= stem cell transplant
Cancer treatment
Surgery for skeletal malformations
Describe rickets presentation
Rachitic rosary, limb deformity, weakness, misery
Metaphyseal swellings, bowing deformities, slowing of linear growth, motor delay, hypotonia, fractures, resp distress
Causes of low vit d/rickets?
Maternal vitamin D deficiency causes low stores in newborn, exclusive breastfeeding will exacerbate
Lack of dietary intake ie prolonged unsupplemented breastfeeding
What is rickets?
Severe vitamin D deficiency (child version of osteomalacia)
Pathophysiology of rickets?
lack of vit D; inadequate mineralisation of bone matrix
Decreased vit D > decreased calcium and phosphate > secondary hyperparathyroidism
In children, this occurs before the growth plates have closed.
Presentation of rickets?
Hypocalcaemic seizures or tetany, bony deformity (eg genu varum and valgum), irritable and reluctant to weight bear, severe can result in cardiomyopathy
Delayed walking, waddling gait, impaired growth, fractures, dental deformities
Investigating rickets?
Bloods, wrist X ray (long bone showing cupping, splaying and fraying of metaphysis eg champagne glass wrist) required for diagnosis
Management of rickets?
Diet, sunlight, vit D supplementation=oral calciferol and calcium supplement
Maintenance dose of calciferol is recommended for family members
What are the 3 main differentials for a limping child?
- Infection e.g. sepsis/osteomyelitis.
- Trauma e.g. NAI, fracture.
- Tumour.
What is the likely cause of a limp in a child aged 0-3?
- Trauma - toddlers fracture, NAI
- Infection - osteomyelitis, septic arthritis
- Displasia - DDH
What is the likely cause of a limp in a child aged 3-10?
- Trauma
- Infection - transient synovitis, osteomyelitis
- Perthe’s disease.
What is the likely cause of a limp in a child aged 10-15?
- Trauma.
- Infection - osteomyelitis
- SUFE
- Perthe’s disease
What must you remember to consider as a differential in a limping child?
Intra-abdominal pathology e.g. hernia, testicular torsion.
What investigations might you want to do on a child presenting with a limp?
- General observations e.g. HR, BP, T, RR, O2 sats.
- FBC, BM, ESR and CRP.
- XR - AP and lateral views of the the joint and the joints above and below.
- USS - effusion in joints?
- CT/MRI.
Give 3 signs of septic arthritis.
- Children under 2
- Systemically very unwell
- Pain at rest
- Raised WCC and CRP
- Hip = flexed, abducted, externally rotated
Most common cause of septic arthritis?
S. aureus
Septic arthritis investigations and management?
FBC, ESR/CRP, synovial fluid examination and culture, blood cultures. Xray will show later changes (14-21 days)
Surgical emergency: surgical drainage and IV antibiotics- cefuroxime
Describe Kocher’s criteria. What is it used for?
For differentiating transient synovitis from septic arthritis.
- non-weight bearing
- temp> 38.5
- ESR>40 mm/hr
- WCC> 12000 cells/mm
3/4 = septic joint.
What is DDH?
DDH - developmental dysplasia of the hip.
Abnormal relationship of the femoral head to the acetabulum -> aberrant development of the hip.
What tests can be done on clinical examination in the neonatal period to pick up DDH?
- Ortolani test.
- Barlow manoeuvre.
Can be confirmed with USS.
Give 3 risk factors for DDH.
- Female (M:F - 1:8).
- First born.
- Breech birth.
- Family history.
age cut off for uss or pelvic x ray for DDH?
Under 4.5 months = USS
Over 4.5 months= pelvic XR
Describe the management of DDH.
- Pavlik harness.
- Surgical reduction.
Prevention = safe swaddling
What are the 2 main risks associated with the surgical management of DDH.
- Avascular necrosis.
- Re-dislocation.
What is the most common cause of hip pain/limp in children aged 3-10?
Transient synovitis
What is transient synovitis?
Acute onset joint inflammation following illness, often respiratory.
How does transient synovitis differ from septic arthritis?
Transient synovitis:
- no pain at rest
- systemically well
- rest, physiotherapy and NSAIDs often help
- no findings on esr/crp, xray, uss
Transient synovitis management?
- no long term sequelae and self limiting
- Simple analgesia, rest and physio
- Usually resolves within 2 weeks
What is Perthe’s disease?
A self-limiting idiopathic disease characterised by avascular necrosis of the femoral head.
Key points in history for Perthe’s disease?
- 5-10 yr olds
- Developed over weeks
- No history of trauma
- All hip movements limited
- May have referred pain to groin/thigh/knee
- Systemically well
Describe the management of Perthe’s disease.
- If bone age under 6 years: activity restriction, physio, nsaids
- If bone age over 6 years: surgery
Give 3 risk factors for Perthe’s disease.
- ADHD.
- Deprivation.
- Passive smoking.
- LBW.
- Short stature.
What is SUFE?
Slipped upper femoral epiphysis - slippage of the femoral head
Who is likely to be affected by SUFE?
A pre-pubescent obese male.
How does SUFE present?
- several week history of vague groin/thigh discomfort
- Drehmann’s sign (passively flex hip, falls back into external rotation and abduction)
What is the treatment for SUFE?
Surgical pinning of the hip.
Give 3 signs of osteomyelitis in children.
- Joint pain.
- Lethargy.
- Fever.
Name an organism that commonly causes osteomyelitis in children.
Staphylococcus aureus.
Most common site of osteomyelitis in children?
Distal femur and proximal tibia
Pathophysiology of osteomyelitis?
Infection of bone marrow, commonly S. aureus
Inflammatory destruction of bone.
If periosteum involved: necrosis and detachment forming a sequestrum
Bony remodelling causes deformity
NB/ involucrum= viable periosteum separated from underlying bone and forms new bone around it
What is the likely mechanism of osteomyelitis in children?
Haematogenous spread- tends to occur in rapidly growing and highly vascular metaphysis of growing bones (long bones more common)
Investigating osteomyelitis?
FBC, ESR/CRP, blood cultures, bone cultures (gold standard), MRI
Management of osteomyelitis?
Local bone and soft tissue debridement, stabilisation of bone, local antibiotic therapy, reconstruction of soft tissue, reconstruction of osseous defect zone
Antibiotic therapy 4-6 weeks if acute, at least 12 weeks if chronic
IV cefuroxime or IV flucloxacillin, switch to PO if improving.
What is Kohler’s disease?
Osteochondrosis of tarsal navicular bone
A non inflammatory, none infectious derangement of bony growth, affecting the epiphyses
Presentation of kohler’s disease?
Unilateral antalgic gait, local tenderness of medial aspect of foot over navicular bone
Management of kohler’s disease?
Rest, analgesia, avoid excessive weight bearing, short leg cast for immobilisation, treat for at least 6 weeks
Chronic course but rarely over 2 years
3 types of discoid meniscus?
Incomplete (bit thicker and wider than normal), complete (tibia completely covered by meniscus) and hypermobile wrisberg (normal shape but no posterior attachment to tibia)
How will a discoid meniscus present?
More prone to injury than normal shaped meniscus
Some may never experience problems
Most cases= knee problems, vague pain, audible snap on terminal extension, swelling, locking
Management of discoid meniscus?
If asx, do nothing
otherwise, arthroscopic partial meniscectomy and rehab
What is osgood schlatter disease?
Self limiting disorder of the knee
generally in active adolescents before tibial tuberosity has finished ossification, during adolescent growth spurt
Presentation of osgood schlatter disease?
Gradual onset pain and swelling below knee, relieved by rest, provoked by knee extension or hyperflexing while prone
Management of osgood schlatter disease?
Conservative: rest, ice, physio and exercise advice, simple analgesia
Most patients able to return to activity after 2-3 weeks
What is the criteria for making a clinical diagnosis of juvenile idiopathic arthritis?
Joint swelling/stiffness >6 weeks in children <16 and no other cause is identified.
What symptoms are associated with JIA?
- Fever.
- Salmon-pink rash.
- Uveitis.
- Pain.
- Morning stiffness.
- Swelling.
Give 5 potential consequences that can occur if you fail to treat JIA.
- Damage.
- Deformity.
- Disability.
- Pain.
- Bony overgrowth.
- Uveitis.
Subtypes of juvenile idiopathic arthritis?
Oligoarticular (1-4 joints)
Polyarticular (at least 5 joints)- RF positive or negative
Systemic
Psoriatic
Enthesitis-related
Undifferentiated
Qualifying factors for it to be systemic JIA?
at least 2 weeks of fever with at least one of: rash, ln enlargement, hepato/splenomegaly, serositis= pericarditis/pleuritis/peritonitis
Qualifying factors for it to be psoriatic arthritis (JIA)?
Arthritis and psoriasis
or
arthritis and at least 2 of: dactylitis, nail pitting, onycholysis, psoriasis in first degree relative
Qualifying factors for it to be enthesitis related arthritis?
Arthritis or enthesitis and 2 of:
sacroiliac/lumbosacral pain, HLA b27 positive, family history HLA b27 related disease, anterior uveitis
Describe the non-medical treatment for JIA.
- Education, support, liaison with school
- Physiotherapy
Describe the medical treatment for JIA.
- Steroid joint injections.
- NSAIDS.
- Methotrexate.
- Systemic steroids.
5.biologics- etanacerpt.
What extra-articular features might you see in someone with JIA?
- Psoriasis.
- Dactylitis.
- Nail pitting.
- Rash.
- Fluctuating fever.
- Uveitis.
Name a severe, potentially life-threatening complication of systemic onset JIA.
Macrophage-activation syndrome (MAS).
What signs might you see in someone with macrophage-activation syndrome (MAS)?
- High fever.
- Hepatosplenomegaly.
- CNS dysfunction.
- Purpuric rash.
- Cytopaenia.
What is the treatment for macrophage-activation syndrome (MAS)?
- Supportive treatment.
- Steroids.
Give 5 differentials for a painful joint.
Life-threatening differentials:
- Leukaemia.
- Septic arthritis.
- NAI.
Pain and swelling:
- Trauma.
- Infection.
- Reactive arthritis.
- JIA.
Pain and no swelling:
- Hypermobile joint syndrome.
- Perthe’s disease.
Define child development.
The biological, psychological and emotional changes that occur between birth and adolescence as the individual progresses from dependency to increasing autonomy. It is a continuous process with a predictable sequence however each child’s development is unique.
Give 5 influences on a child’s development.
- Genetic factors.
- Stimulating environment.
- Pregnancy factors e.g. premature? Mums health?
- Healthy attachment.
- Medical conditions.
- Abuse/neglect/domestic violence.
- Healthy peer relationships.
- Education.
- Nutrition.
- Parenting style.
What are the 4 domains of child development?
- Gross motor.
- Fine motor and vision.
- Speech, language and hearing.
- Social interaction and self care skills.
What are the developmental milestones for gross motor function?
- Newborn: flexed arms and legs, equal movement in all 4 limbs
- 3m: lifts head on tummy.
- 6m: chest up with arm support, can sit unsupported.
- 9m: craws, pulls to stand
- 12m: walking
- 2 years: walks up stairs, runs
- 3 years: jumps
With regards to gross motor development, at what age would you expect a child to do the following:
a) walking.
b) jumping.
c) crawling.
d) walking up stairs.
a) Walking - 12 months.
b) Jumping - 3 years.
c) Crawling - 8 months.
d) Walking up stairs - 2 years.
What are the developmental milestones for fine motor and visual function?
- 6 weeks: fix and follow
- 6 mo: palmar grasp (picks up cube)
- 1 yr: pincer grip
- 1.5 yrs mo: tower of 3 cubes
- 2 yrs: tower of 6 cubes, draw straight line
- 3 yrs: bridge of 3 cubes, draw circle
With regards to fine motor and visual development, at what age would you expect a child to do the following:
a) drawing with crayons.
b) building a tower of 8 cubes.
c) takes an object in each hand.
d) builds a tower of 2 cubes.
a) Drawing with crayons - 12m.
b) Building a tower of 6 cubes - 2 years.
c) Takes an object in each hand - 6m.
d) Builds a tower of 3 cubes - 18m.
What are the developmental milestones for speech, language and hearing?
- newborn: startles to loud noises
- 6 mo: babbles, turn to sound
- 9 mo: responds to own name, ‘dada’, ‘mama’
- 12 mo: 1 proper word
- 18 mo: nouns e.g. body parts, teddy
- 2 years: verbs e.g. eat, 50+ words, simple sentences (give teddy)
- 3 years: adjectvies e.g. colours, speech mainly understandable
- 4 years: can count 5 objects
With regards to speech, language and hearing, at what age would you expect a child to do the following:
a) form short sentences and name body parts.
b) knows colours and can count.
c) laughs and squeals.
d) has mainly understandable speech.
a) Forms short sentences and name body parts - 2 years.
b) Knows colours and can count - 4 years.
c) Laughs and squeals - 3 months.
d) Has mainly understandable speech - 3 years.
What are the developmental milestones for social interaction and self-care skills?
6 weeks: smile spontaneously
6 mo: finger feeds
9/10 mo: wave bye bye, stranger danger
12 mo: drink from cup, uses spoon/fork
2 years: undresses, dry by day
3 years: parallel play, interactive play evolving, put on t shirt
4 years: play simple board game, gets dressed themself
With regards to social interaction and self-care skills, at what age would you expect a child to do the following:
a) uses cutlery.
b) plays with others, names a friend.
c) smiles.
d) waves bye-bye.
a) Uses cutlery - 12 months.
b) Plays with others, names a friend - 3 years.
c) Smiles - 6 weeks.
d) Waves bye-bye - 9/10 months.
What does ‘The healthy child programme’ encourage?
- Encourages care to keep children healthy and safe.
- Promotes healthy eating and activity.
- Identifies problems in children’s development.
- Identifies ‘at risk’ families for more support.
- Ensures children are prepared for school.
Gross motor developmental red flags?
- Not sitting by 12 months.
- Not walking by 18 months.
Fine motor developmental red flag?
Hand preference before 18 months (cerebral palsy)
Speech and language developmental red flag?
No clear words by 18 months - ASD? Language problems?
3 social developmental red flags?
- No smiling by 3 months
- No response to carers interactions by 8 weeks.
- No interest in playing by 3 years.
Give 5 causes of developmental delay.
- Genetics.
- Pregnancy.
- Factors around birth.
- Factors in childhood.
- Environmental.
Causes of developmental delay: give examples of genetic causes.
- Chromosomal disorders e.g. Down’s syndrome.
- Single gene disorders e.g. Duchenne.
- Polygenic e.g. ASD, ADHD.
- Micro-deletions or micro-duplications.
Causes of developmental delay: give examples of pregnancy related causes.
- Congenital infections e.g. CMV, HIV.
- Exposure to drugs/alcohol e.g. FAS.
- MCA infarct.
Causes of developmental delay: give examples of birth related causes.
- Prematurity.
- Birth asphyxia (due to hypoxia).
Causes of developmental delay: give examples of medical causes that may occur during childhood.
- Infections e.g. meningitis.
- Chronic illness.
- Hearing or visual impairment.
- Acquired brain injury.
How might you investigate developmental delay?
Thorough history and examination. Tailor any investigations to the child e.g.
- Boys not walking by 18m check creatinine kinase for Duchenne.
- Focal neurological signs -> MRI brain.
- Genetic testing.
- Unwell, failure to thrive -> metabolic investigations.
There is no ‘developmental screen’, investigations need to be tailored towards to the child.
Causes of developmental delay: give examples of environmental causes.
- Abuse and neglect.
- Low stimulation.
What is global developmental delay?
Significant delays in at least two of the four areas of development.
Types of epilepsy in childhood?
Absence seizures
Childhood epilepsy syndrome (‘benign’ if can predict from EEG that will stop by certain age)
Infantile spasms=West syndrome
Bects: Rolandic epilepsy (benign epilepsy with centro-temporal spikes)
Juvenile myoclonic epilepsy
Define seizure.
A paroxysmal abnormality of motor, sensory, autonomic and/or cognitive function due to transient brain dysfunction.
-may be epileptic or non-epileptic
Types of seizures? (epileptic and non-epileptic)
Epileptic
- Epilepsies
- Acute asymptomatic seizures
- Febrile seizures
Non epileptic
- convulsive syncope e.g. cardiac syncope, neurally-mediated, hypovolaemic, expiratory-apnoea syncope (blue breath holding spells)
Define convulsion.
A convulsion is a seizure, either epileptic or non-epileptic, with motor components e.g.
- stiffening=tonic
- a massive jerk=myoclonic
- jerking=clonic
- thrashing about=hypermotor
as opposed to a non-convulsive seizure with motor arrest e.g. unresponsive stare, drop attack
Define epileptic seizure.
What makes a seizure eplieptic is the nature of the underlying electival activity in the brain: excessive, hypersynchronous neuronal discharges in all or part of the cerebral cortex.
Define epilepsy.
An umbrella term brain disorders that predispose the patient to having epileptic seizures.
How long do epileptic seizures tend to last for?
30 - 120 seconds.
Give 3 signs of epileptic seizures.
- Movement.
- Tongue biting.
- Head turning.
- Muscle pain.
Causes of epilepsies?
-genetic (70%): caused by allelles at several lociso inhertiace is complex
-structural, metabolic: cerebral dysgenesis, damage (vascular occlusion, infection)
What are febrile convulsions?
Febrile convulsions are epileptic seizures accompanied by fever. They usually occur early in viral infection and tend to be brief generalised tonic-clonic seizures.
Px of febrile convulsion?
Last less than 5 mins, child becomes stiff, then limbs twitch, lose consciousness and may wet/soil themselves
May be sick, foam at mouth and eyes may roll back
Sleepy for up to an hour after event
What is a complex febrile seizure?
Lasting longer than 15 mins
How long do non-epileptic seizures tend to last for?
1 - 20 minutes.
Give 3 signs of non-epileptic seizures.
- Eyes closed.
- Talking/crying.
- Pelvic thrusting.
What 2 broad categories can epileptic seizures be divided into?
Generalised and focal.
Give 3 examples of generalised seizures.
- Absence.
- Myoclonic.
- Tonic.
- Atonic.
- Generalised tonic-clonic.
What are absence seizures?
Seizures where there is a transient loss of consciousness with an abrupt onset and termination. Momentary unresponsive stare with motor arrest, lasts <30s. Developmentally normal but can interfere with school.
Give some possible side effects of the treatment for absence seizures in children?
- Sodium Valporate - weight gain, hair loss, teratogenic.
- Ethosuxamide N+V
How might you investigate suspected absence seizures?
. Observe an episode - hyperventilation, ask the child to blow on a windmill.
2. EEG - would show 3-second spike and wave discharges.
Absence seizure meds?
- Sodium valproate/ ethusoxemide for girls
- Clobazam
What would you expect to see in a tonic seizure?
Generalised increase in tone.
What can absence seizures evolve into?
Juvenile myoclonic epilepsy (JME).
What are the signs of juvenile myoclonic epilepsy?
Clumsiness and GTCS that occur shortly after waking and are often provoked by sleep deprivation.
What would you expect to see in a generalised tonic-clonic seizure?
Sudden onset rigid phase followed by a convulsion in which the muscles jerk rhythmically.
Treatment for tonic-clonic seizures in children?
- Sodium valproate, carbamazepine for girls
- Clobazam
- Side effects of first line treatment for tonic-clonic seizures?
- Contraindication?
- Sodium valproate - weight gain, hair loss, teratogenic
- Carbmazepine - rash, hyponatraemia, can interfere with contraception. Contraindicated in absence and myoclonic seizures.
What would you expect to see in a myoclonic seizure?
Isolated muscle jerking.
First and second line AEDs for focal seizures?
- Lamotrigine/carbmazepine, sodium valproate (not for girls)
- Clobazam
What investigations might you want to do in someone presenting with seizures.
- Eye witness account/video is invaluable!
- ECG.
- EEG.
- MRI or CT.
Why must you do an ECG in those suffering from seizures?
To check for arrhythmia as the cause e.g. long-QT syndrome.
Give 3 potential side effects of AED’s.
- Cognitive disturbances
- Heart disease.
- Drug interactions.
- Teratogenic.
Name common epilepsy syndromes in order of increasing age.
- 3-12 months: Infantile spasms (west syndrome)
- 1-3 yrs: Lennox-Gaustat
- 4-10 years: Benign rolandic epilepsy (15%), childhood absence epilepsy (2%)
- 10-20 yrs: Juvenile absence, juvenile myoclonic
-name does not necesarily correspond to seizures e.g. can have absence and tonic-clonics in “juvenile myoclonic epilepsy”
Name 3 conditions hat are commonly misdiagnosed as epilepsy.
- Sandifer syndrome.
- Benign neonatal sleep myoclonus.
- Syncope.
What is syncope?
Insufficient blood or O2 supply to the brain causes paroxysmal changes in behaviour, sensation and cognitive processes.
What non-neurological disease is sandifer syndrome associated with?
GORD.
Patients present with GORD and a characteristic neck movement disorder.
Common causes of ‘funny turns’? (paroxysmal disorders)
- blue breath holding spells (toddlers)
- reflex anoxic syncope (triggered by pain, fright, discomfort)
- syncope
- migraine
- vertigo
Describe a floppy infant.
Hypotonia, weakness, ligamentous laxity, increased range of joint mobility
What features suggest central hypotonia/UMN in children?
Normal strength, dysmorphic features, normal/brisk tendon reflexes, irritability +/- loud cry, history suggestive of HIE (hypoxic-ischaemic encephalopathy)/birth trauma/sxs hypoglycaemia,seizures
Central=2/3 cases, commonly HIE
What are indicators of peripheral hypotonia/LMN in children?
Decreased strength, reduced/absent reflexes, fasciculation, myopathic face, weak cry
Causes of floppy infant?
Central hypotonia=acute encephalopathies (HIE, hypoglycaemia, intracranial haemorrhage), chronic encephalopathies (cerebral malformations, metabolism errors, chromosomal disorders, endocrine disorders, metabolic disorders), connective tissue disorders (Ehler-Dahlos, OI)
Peripheral hypotonia= spinal cord (syringomyelia), anterior horn cell (spinal muscular atrophy), NMJ (MG), muscular disorders (dystrophies, myopathies), peripheral nerves, metabolic myopathies
What is cerebral palsy?
Non progressive cerebral pathology that leads to a disorder of movement and posture in children.
Damage to immature brain (most between 24 weeks and term)
Why might the clinical signs of cerebral palsy change over time?
The clinical signs may change over time as the brain matures but the underlying aetiology is not progressive.
Classification of cerebral palsy?
- Spastic
- athetoid (hyperkinesia)
- ataxic
- mixed
Give 3 causes of cerebral palsy.
80% antenatal - hypoxia, infection, haemorrhage, ischaemia.
10% peri-natal - hypoxia, infection, haemorrhage.
10% postnatal - hypoxia, infection e.g. meningitis, haemorrhage, encephalopathy, trauma.
Presentation of cerebral palsy?
Motor features- mono/hemi/para/quadriplegia
Don’t meet developmental milestones eg not sitting by 8 months, not walking by 18 months, early hand preference before 1 year
GORD, epilepsy, vomiting, constipation, bladder issues, drooling, orthopaedic problems all common
How to diagnose cerebral palsy?
Definitive dx may not come until 12-18 months
Clinical observation and parental observation
Describe the support that is offered to someone with cerebral palsy.
- Physiotherapists for mobility and hand function.
- SALT for communication.
- Feeding support.
- Sleeping support.
Support for children with disabilities needs to be holistic, child focused and with an MDT approach.
What drug can be used to treat hypertonia in children with cerebral palsy?
Botox
Fits, faints and funny turns causes?
In sleep: benign neonatal sleep myoclonus and parasomnias
On feeding: GORD and sandifer syndrome
Fever: febrile seziures, vaso-vagal syncope
Pain/shock/startle: reflex asystolic syncope, cyanotic breath holding, hyperekplexia
Tired/bored/stress: self gratification behaviour, tics, daydreaming
Excitement: shuddering spells, cataplexy
Potential childhood motor disease?
Tourette syndrome and tics, tremor, dystonia, ataxia, restless legs syndrome, myoclonus, juvenile huntington disease
DSM-V criteria for ADHD
Symptoms must be present before age 12, and present in at least 2 settings, affect functioning.
At least 6 symptoms of inattention for at least 6 months
or
At least 6 symptoms of hyperactivity for at least 6 months
Non drug management of ADHD
parent education and training
CBT can be offered
Meds not advised for pre-school children
Advice: plan day, clear boundaries, brief and specific instructions, incentive schemes, kep social situations short and sweet, exercise, healthy diet, bedtime routine, help at school
Medical management of ADHD
Stimulant drugs (increase dopamine):
Short acting methylphenidate eg ritalin
Long acting methylphenidate eg delmosart
Lisdexamfetamine
Non stimulant drugs (reduced breakdown of noradrenaline):
Atomoxetine (SNRI), guanfacine
Describe autism spectrum disorders
Abnormal development from under 3 years old in:
- social interaction
- communication
- restricted, stereotyped and repetitive behaviour
Diagnosis requires at least 6 symptoms across 3 core areas
Management of autism spectrum disorders
Non-pharm management
behavioural therapies, social skills groups, OT, communication interventions, input from dietician if needed, specialised educational programs and structured support
ICD-10 for anorexia nervosa
Deliberately keeping weight below 85% of expected via restricted diet choice, excessive exercise, induced vomiting, use of appetite suppressants and diuretics Dread of fatness-intrusive overvalued idea Endocrine effects (menstruation stops/puberty delayed if menarche not yet achieved, loss of sexual interest in men)
How to screen for eating disorders?
SCOFF
Do you make yourself sick because you’re uncomfortably full?
Do you worry that you’ve lost control over how much you eat?
Have you recently lost more than one stone (about 6kg) in 3 months?
Do you believe you are fat when others say you are thin?
Would you say that food dominates your life?
Potential clinical signs of anorexia nervosa?
Dry skin, lanugo hair, orange skin and palms, cold hands and feet, bradycardia, drop in BP on standing, oedema, weak proximal muscles (squat test)
Management of anorexia nervosa?
- *1. Family therapy.**
2. IPT.
3. CBT.
Weight restoration at 0.5kg/week - monitor for re-feeding syndrome.
Define bulimia nervosa
Binges followed by compensatory weight loss behaviours eg self induced vomiting, fasting, intensive exercise, abuse of medication
BMI maintained at over 17.5
Describe the aetiology of ADHD.
- Genetic and environmental.
- Neuroanatomical and neurochemical factors too.
- CNS insults e.g. FAS or premature.
ASD signs: communcation, social interaction and social imagination
- Communication: non-verbal communication challenging, only communicate needs, no understanding of jokes
- Interaction: poor eye contact, struggles to understand social roles
- Imagination: no imaginative play, obsessions/rituals struggles with change
RF’s for developing anorexia?
- Social pressure.
- Perfectionist traits.
- Family attitudes to food.
- Low self esteem.
- Occupation/interests.
- Family history.
Give 5 symptoms of depression.
- Loss of interest.
- Fatigue.
- Poor sleep.
- Reduced appetite.
- Low concentration.
- Feelings of guilt and self blame.
- Low confidence.
- Agitated.
- Hopeless.
Describe the non-medical and medical treatment of depression.
Non-medical: Education, CBT, IPT and family therapy.
Medical: fluoxetine, sertraline, citalopram.
Give 3 predisposing factors for a child developing depression.
- Family history.
- Stress in pregnancy.
- Poor attachment.
- Poverty.
- Isolation.
Give 3 precipitating factors for a child developing depression.
- Trauma.
- Drugs.
- Infections.
- Puberty.
- Exam stress.
- Sexual abuse.
- Bullying.
Give 3 perpetuating factors for a child developing depression.
- Chronic illness.
- Malnutrition.
- Ongoing neglect.
- Ongoing poverty.
Describe the foetal circulation.
Placenta -> umbilical vein -> IVC -> RV -> foramen ovale -> LA -> aorta -> umbilical arteries -> placenta.
OR: … RV -> pulmonary artery -> ductus arteriosus -> aorta …
What is the function of the foramen ovale and the ductus arteriosus in the foetal circulation?
They are used to bypass the non-functiong lungs.
Name 4 congenital heart problems that can cause a L -> R shunt.
- VSD.
- ASD.
- AVSD.
- PDA.
Most common septal defect?
VSD
Give 5 signs of a VSD.
- Poor feeding and FTT.
- Tachypnoea.
- Thrill.
- Pan-systolic murmur.
- Increased work of breathing.
You request a CXR for a patient with a VSD. What would you expect to see?
- Cardiomegaly.
- Pulmonary oedema.
- Enlarged pulmonary arteries.
Gold standard diagnosis of septal defect?
Echo
Murmur for VSD and where to listen?
- VSD = very systolic = pansystolic.
- Best heard over tricuspid area (lower left sternal border)
Management of VSD?
Qp/Qs (pulmonary to systemic blood flow ratio) of at least 2.0 requires surgical repair
Aim to close large VSDs within first 2 years of life
75% of small VSDs close spontaneously by age 10
Why are ASD’s often asymptomatic?
ASD’s are often asymptomatic because the blood flow in the atria is low pressure and so breathlessness etc is uncommon.
Most common ASD?
Patent foramen ovale.
Type of murmur in ASD?
- a split double s2
- best heard in pulmonary area (upper left sternal edge)
RFs for ASD
- maternal smoking in first trimester
- maternal diabetes
- maternal rubella
- maternal drug use eg cocaine/alcohol
Give 5 signs of a PDA.
- Poor feeding, FTT.
- Tachypnoea.
- Active precordium.
- Thrill.
- Continuous machinery murmur.
Management of ASD?
If less than 5mm, spontaneous closure within 12 months
Surgical closure required if bigger than 1cm
Describe the management for congenital health defects that cause a L->R shunt.
- Stabilise the patient.
- Increase calorie intake.
- NG tube.
- Diuretics and ACEi to prevent HF symptoms.
- Surgical repair.
AVSD is a common defect in people with which chromosomal abnormality?
Trisomy 21 (Down’s syndrome).
Describe the pathaphysiology of an AVSD?
2 types
Failure of endocardial cushions to fuse properly
Complete AVSD: large left to right shunt causes excessive pulmonary blood flow leading to HF and pulmonary vascular resistance
Partial AVSD: left to right shunt and volume overload of RA and RV but the pulmonary artery pressure is only mildly elevated
Presentation of AVSD?
Tachypnoea, tachycardic, poor feeding, sweating, failure to thrive, all with a complete AVSD are symptomatic by 1 year
Prominent sternal heave, murmur
Murmur in AVSD and where to auscultate?
Ejection systolic murmur in pulmonary area (left upper sternal border)
Mid-diastolic murmur over tricuspid area (left lower sternal border)
Holosystolic murmur might be heard at apex
Management of AVSD?
Surgical management as patients would die by age 2-3 without
Name 2 congenital heart problems that can cause a R -> L shunt.
- Tetralogy of fallot.
- Transposition of the great arteries.
What 4 components make up Tetralogy of fallot?
- Ventricular septal defect
- Pulmonary stenosis
- Right ventricular hypertrophy
- Overriding aorta
Most common cyanotic congenital heart disease
Give 3 signs of Tetralogy of fallot.
- Cyanosis.
- Collapse.
- Acidosis.
If tetralogy of fallot isn’t managed, what can occur?
polycythaemia, cerebral abscess, stroke, infective endocarditis, HF, death
Murmur in tetralogy of fallot and where to listen?
Pansystolic murmur best heard over mid or upper left sternal edge
Smaller the VSD=louder the murmur
What are tet spells?
Hypoxic spells in tetralogy of fallot with peak incidence between 2-4 months of life:
Paroxysm of hyperpnoea, irritability, increasing cyanosis
Management of tetralogy of fallot?
Medical= squatting, prostaglandin infusion, beta blockers, morphine, saline bolus
Surgical management for definitive repair or palliative management
Which defects have a higher risk of HF?
Significant left to right shunt= VSD, ASD, PDA
Transposition of the great arteries
Describe the two types of transposition of the great arteries?
dextro-transposition=aorta is anterior and to the right of the pulmonary artery
levo-TGA= aorta anterior and to the left of the pulmonary artery
What is coarctation of the aorta?
Arterial duct tissue encircles the aorta at the point of insertion of the duct. When the duct closes, the aorta constricts, this causes severe obstruction to LV outflow.
When do you get cyanotic heart disease?
Absent/not wide enough tricuspid/pulmonary/aortic valve
Coarctation of aorta
Ebstein anomaly
Tetralogy of fallot
TGA
Truncus arteriosus
Anywhere with a right to left shunt
What is rheumatic fever?
Systemic illness 2-4 weeks after pharyngitis due to cross reactivity to group A beta-haemolytic streptococcus (S. pyogenes)
What are the diagnostic criteria for rheumatic fever? Describe
Revised Jones diagnostic criteria= positive throat culture or anti-streptolysin O/anti-DNA B titre and 2 major or 1 major+2 minor criteria
What are the major criteria in the revised jones criteria?
SPECS
Sydenham’s chorea, polyarthritis, erythema marginatum, carditis, subcut nodules
What are the minor criteria in the revised jones criteria?
CAPE
CRP/ESR raised
Arthralgia
Pyrexia
ECG-prolonged PR
Management of acute rheumatic fever?
Antibiotics=benzathine benzylpenicillin
Aspirin/NSAIDs
Assess for emergency valve replacement
Secondary prevention with abx every 3-4 weeks
What factors lead to subacute bacterial endocariditis?
Endothelial damage, platelet adhesion, microbial adherence
What organisms cause IE?
S. aureus, S. viridans, S. pneumoniae, HACEK organisms
Presentation of IE?
persistent low grade fever, heart murmur, splenomegaly, petechiae/oslers nodes/janeway lesions/splinter haemorrhages
Criteria for diagnosis of IE? describe
Modified Dukes criteria
2 major
1 major and 3 minor
5 minor
Major criteria in modified dukes?
positive blood cultures, evidence of endocardial involvement (echo)
Minor criteria in modified dukes?
predisposition, fever, vascular phenomena, immunologic phenomena, microbiology evidence, other echo findings
Management of IE?
IV antibiotics
May require surgical intervention
What is coarctation of the aorta?
Arterial duct tissue encircles the aorta at the point of insertion of the duct. When the duct closes, the aorta constricts, this causes severe obstruction to LV outflow.
Give 3 signs of coarctation of the aorta.
- Radio-femoral delay.
- Weak femoral pulses.
- Difference in pre and post-ductal saturations.
Give 3 signs of aortic stenosis.
- Palpable thrill.
- Ejection systolic murmur.
- LVH.
What is a possible consequence of persistent pulmonary hypertension, like that seen in CHD associated with a L->R shunt?
Eisenmenger syndrome: high pressure pulm. blood flow damages pulmonary vasculature -> increased resistance (pulm. hypertension) -> RV pressure increase -> shunt direction reverses -> CYANOSIS!
What is a possible consequence of persistent pulmonary hypertension, like that seen in CHD associated with a L->R shunt?
Eisenmenger syndrome: high pressure pulm. blood flow damages pulmonary vasculature -> increased resistance (pulm. hypertension) -> RV pressure increase -> shunt direction reverses -> CYANOSIS!
What factors aid reflux in GORD?
Low tone of muscular portion of lower oesophagus, short and narrow oesophagus, delayed gastric emptying, shorter lower oesophageal sphincter, liquid diet and high calorie requirement (distends stomach), significant periods recumbent
Presentation of GORD?
Distressed, unexplained feeding difficulties, hoarseness +/- chronic cough, single episode pneumonia, faltering growth, if able may report pain
When does GORD or regurgitation (=posseting) present normally
Within 2 weeks of life
How to manage a breast fed baby with gord
Use alginate eg gaviscon mixed with water after feeds
How to manage a formula fed baby with gord
1= do not over feed (less than 150ml/kg/day) 2= decreased feed volume by increasing frequency (2-3 hourly) 3= use feed thickener 4= stop thickener and use alginate added to formula
What to use after alginate if no success in GORD?
histamine antagonists or ppi
Name 5 potential causes of poor feeding
Infection, metabolic disorders, genetic disorders, structural abnormalities, neurological disorders, mum and feeding technique
Presentation of pyloric stenosis
Baby starts bringing up small amounts of milk after feeding, which gets worse over a few days
Then projectile yellow vomit (milk curdles in stomach acid)
Reduced faeces
If untreated: dehydration and not gaining weight
When does pyloric stenosis present
About 6 weeks after birth
You do a blood gas on a neonate with pyloric stenosis. What would it show?
Metabolic Alkalosis - low K+ and Cl-.
The baby has vomited up all the HCl and the kidneys go into overdrive - increased K+ secretion.
How to manage pyloric stenosis
- IV fluids.
- Repeat gases to monitor alkalosis.
- Stop feeding to stop vomiting.
- Pyloromyotomy once stable
You suspect pyloric stenosis in a neonate. What investigations might you do?
- U+E.
- Blood gas.
- USS - hypertrophy of pyloric sphincter
Potential presentation of IBS
Bloating, cramping, chronic or intermittent diarrhoea/constipation, urgency with defecation, incomplete sensation of defecation, passage of mucus in stool
May have dizziness, nausea, loss of appetite
How to manage IBS
Diet and lifestyle management eg small meals, more often, low fat and high carbs
How common is gastroenteritis?
Very!
At least one episode per year for most children
Common causes of gastroenteritis
Rotavirus is most common infantile (NB rotarix oral vaccine at 8 and 12 weeks)
Norovirus commonest acorss all age groups
Campylobacter is most common bacterial cause
Also E.coli and adenovirus
Presentation of gastroenteritis
Sudden onset diarrhoea +/- vomiting, abdo pain, mild fever
(bloody diarrhoea if campylobacter- generally due to undercooked meat and unpasteurised milk)
Management of gastroenteritis
Self limiting
Send a stool sample if ?septicaemia/blood or mucus in stool/immunocompromised
Encourage fluids (not carbonated drinks)
IV or oral therapy if dehydrated
Give full strength milk asap
Leave it 48 hours before returning to school
How long do sxs of gastroenteritis last?
Diarrhoea around 5-7 days
Vomiting around 1-2 days
Potential complications of gastroenteritis?
Haemolytic uraemic syndrome, reactive complications eg Reiter’s syndrome, toxic megacolon, acquired/secondary lactose intolerance
What counts as constipation in children?
Less than 3 complete stools/week
Hard/large stool
Rabbit droppings
Overflow soiling in children over 1
Management of simple constipation in children?
Diet and lifestyle advice- high fibre, increase fluids
Then add Macrogol eg Movicol
Then add stimulant laxative eg Senna
Consider adding in lactulose
In secondary care can do manual evacuation, antegrade colonic enema, polyethylene glycol solutions
Describe the pathophysiology of appendicitis
Luminal obstruction (faecolith or lymphoid hyperplasia, impacted stool) Decreased venous drainage and localised inflammation by commensal bacteria causes and increased pressure. This causes ischaemia which can lead to necrosis and perforation
Presentation of appendicitis
Dull peri-umbilical pain transfers to a sharp right iliac fossa pain
May have vomiting, anorexia, nausea, diarrhoea or constipation
MAGNET: migration of pain to RIF, anorexia, guarding, nausea, elevated temp, tenderness in RIF
What can be found on examination of appendicitis
Rebound tenderness and percussion pain over McBurneys point and guarding Psoas sign (pain when patient lies on left side while right thigh is flexed backward) Rovsing's sign (pain in RLQ upon palpation of LLQ)
Management of acute appendicitis
Antibiotics and laparoscopic appendicectomy
Why are children at higher risk of appendix perforation?
Tend to have delayed presentation
Two types of childhood hernia?
Umbilical and epigastric
How to manage hernias in children?
Umbilical- surgery if still present after 3 years
Epigastric- surgery only if uncomfortable or a nuisance
Describe how Crohn’s disease affects the body (microbiology and location)
Remitting and relapsing disease, affects anywhere between the mouth and the anus
Transmural inflammation, deep ulcers and fissures with cobblestone mucosa, skip lesions
Non-caseating granulomatous inflammation
Presentation of Crohns disease
Abdominal pain and bloody/mucus containing diarrhoea
Oral aphthous ulcers, perianal disease (tags, fistulae)
Extra-intestinal features=MSK, eyes, skin, renal, hepatobiliary
Investigations for IBD?
Faecal calprotectin, routine bloods, stool microscopy and culture, colonoscopy with biopsy (=definitive diagnosis and can distinguish between UC and CD)
How to manage Crohn’s disease? Acute attack
In acute attack: avoid anti-motility drugs eg loperamide as it can cause toxic megacolon
In acute attack: fluid resus, nutritional support, prophylactic heparin, corticosteroid and immunosuppressant eg mesalazine or azathioprine
How to manage Crohn’s disease? Maintenance
Azathioprine, smoking cessation
Surgery can be used- ileocaecal resection, stricturoplasty, small/large bowel resections
Course and pathophysiology of ulcerative colitis?
Relapsing/remitting
Diffuse continual mucosal inflammation of large bowel only. Begins in the rectum and spreads proximally.
Mucosal and submucosal inflammation only, crypt abscesses and goblet cell hypoplasia
What is a protective factor against UC?
Smoking!
Presentation of UC?
Bloody diarrhoea, PR bleeding and mucus discharge, increased faecal frequency, urgency and tenesmus
Extra-intestinal: MSK, skin, eyes, hepatobiliary
How to induce remission in UC?
Corticosteroid and immunosuppressant eg mesalazine or azathioprine
Maintenance management of UC?
Immunomodulators- mesalazine or sulfasalazine
Next line=infliximab
Surgery= ileostomy and complete resection= curative, but many have sub-total colectomy to begin with
Pathophysiology of Coeliac disease
T cell mediated autoimmune response.
Response to gliadin and genetic (HLA-DQ2/DQ8)
Anti-gluten CD4 t cell response, with anti-gluten antibodies, anti-tissue transglutaminase, endomysin antibodies and intraepithelial lymphocytes activated
Causes epithelial cell destruction and villous atrophy
which HLA molecules is Coeliac Disease often associated with?
DQ2 and DQ8.
What are the forms of coeliac disease?
Classical, atypical, latent, silent, potential
What are potential extra-intestinal features of coeliac disease?
Dermatitis herpetiformis, osteoporosis, anemia, short, delayed puberty, arthritis
How to investigate for coeliac disease?
Serology
Must have had gluten in diet for at least 6 weeks prior to testing
IgA and IgAtTG, IgAEMA
Duodenal biopsy
Common causes of failure to thrive
Down syndrome, cerebral palsy, heart disease, infections, milk allergy, CF, coeliac disease, GORD
What is marasmus
Protein and energy malnutrition.
Loss of fat mass especially buttocks
Chronic diarrhoea and emaciated body type
There is NO oedema
What is marasmus usually a sign of?
Poor socioeconomic status, child abuse+neglect, lack of food resources
How to manage marasmus?
Similarly to kwashiorkor and reintroduce food slowly and correct any electrolyte imbalances
What is kwashiorkor
Inadequate protein with a reasonable calorie intake
Presentation of kwashiorkor
Fatigue, irritability, lethargy, failure to thrive, loss of muscle mass, generalised oedema, pot belly, fatty liver, prone to infections, hair and skin changes (erythematous, exfoliation, pigment, dry hair, thin nails)
What is seen in bloods for kwashiorkor?
low glucose, low protein, high cortisol and GH, low salt, iron deficiency anaemia, metabolic acidosis
Management of kwashiorkor?
Correct fluids/electrolytes- needs to be done in about 48 hours
Reintroduce foods slowly afterwards using RUTF (ready to use therapeutic foods eg peanut butter, milk powder, sugar, vegetable oil, minerals and vitamins)
Treatment takes around 2-6 weeks
Pathophysiology of Hirschprung’s disease
Congenital aganglionic megacolon disease Aganglionic segment (short/long/total) remains in tonic state so there is a failure in peristalsis Functional obstruction due to accumulated faeces
Presentation of Hirschprung’s disease
Abdominal distension, bilious vomiting, failure to pass meconium within 48 hours after birth
Males 4 times more likely
What is a big complication of Hirschprung’s disease
Hirschprung’s enterocolitis: stasis allows bacterial proliferation, which can cause sepsis and death
How to investigate for Hirschprungs
Initially a plain X ray
Gold standard=rectal suction biopsy
Management of Hirschprungs
IV antibiotics, NG tube, bowel decompression initially
Definitive= surgery to resect aganglionic section and connect unaffected bowel to dentate line
What is intussusception?
Who does it happen to?
- Movement/telescoping of one part of bowel into another > obstruction, inflamm, bloody stools
- Peak at 5-7 months, rare after 2 years, males twice as likely
Presentation of intussusception
- Colicky pain.
- Vomiting.
- Abdominal mass.
- Redcurrant jelly stool.
How to diagnose intussusception?
abdominal uss
How to manage intussusception?
- Gas in anal canal to reduce intussusception.
- Analgesia e.g. morphine.
- IV fluids if shocked.
What is meckel diverticulum?
Outpouching of lower part of small intestine- a remnant of umbilical cord
Presentation of meckel diverticulum?
Most asx
- Severe rectal bleeding.
- Intussusception.
- Volvulus.
What is malrotation with volvulus?
Occurs during embryonic development at about the 10th week
Bowel twists so blood supply is cut off
Presentation of malrotation with volvulus?
- Obstruction with bilious vomiting
- Bouts of crying
- pull legs into body
- not passing faeces
Give a potential consequence of malrotation.
SMA blood supply to the small intestine can be compromised -> infarction.
How to manage malrotation with volvulus
Dx by Xray
Tx= operation- stop necrosis of bowel and may need bowel resection
What is toddler diarrhoea
Chronic nonspecific diarrhoea, at least 3 loose watery stools/day
Some alternate with constipation
Self limiting at about age 5-6
NOT due to malabsorption or serious bowel problem
What are the 4Fs for diet consideration in toddler diarrhoea
Fat, fluid, fruit juices and fibre
What is colic
Self-limiting repeated episodes of excessive and inconsolable crying for at least 3hrs a day, at least 3 days a week for at least 1 week
Occurs in up to 4 months of age
Presentation of colic
Often crying in late afternoon or evening, draws knees up and arches back
Management of colic?
REASSURANCE
Soothing strategies, parental wellbeing aids
Discourage simeticone (infracol) or lactase drops, probiotics, herbal supplements and maternal diet modification
Exclude other possible causes of distress
Describe the three types of biliary atresia
Type I= common bile duct atresia with patent proximal ducts
Type II= common hepatic duct atresia with cystic structures in porta hepatis
Type III= R and L hepatic duct atresia to level of porta hepatis (most common)
What is biliary atresia?
Extrahepatic bile ducts obliterated by inflammation and subsequent fibrosis
Presentation of biliary atresia?
Presentation shortly after birth
Persistent jaundice
Pale stools and dark urine in term infants with normal birth weights
What investigations are required in biliary atresia and what results shown?
LFTs: increased GGT, TGs normal
USS
Liver histology from percutaneous biopsy
Management of biliary atresia?
Surgery, liver transplant needed before 8 weeks
Prognosis without treatment is 18 months
Complications of biliary atresia?
Ascending cholangitis, cirrhosis, portal hypertension, liver failure, hepatocellular carcinoma
Pathophysiology of cow’s milk protein allergy
Immune-mediated allergic response to casein and whey
IgE mediated type 1 hypersensitivity or non-IgE(T cell activation)
Presentation of cow’s milk protein allergy
Acute and rapid onset (less than 2 hrs after ingestion)
- pruritus
- erythema
- Urticaria and lip swelling
- acute colicky abdo pain
- vomiting
- diarrhoea
- may have resp signs
Management of cow’s milk protein allergy
Specialised formula feeds for at least 6 months/ 9-12 months old, then reevaluate
A 6-month old breast fed infant developed widespread urticaria immediately after the first formula feed.
a) What investigation might you do?
b) What is the most likely diagnosis?
a) Skin-prick test for cow’s milk.
b) IgE mediated cow’s milk allergy.
A 4-month-old infant, formula fed since birth, has loose stools and faltering growth. Skin prick test to cow’s milk is negative but elimination of cow’s milk results in resolution of symptoms which return on trial re-introduction. What is the most likely diagnosis?
Non-IgE mediated cow’s milk allergy.
Choledochal cysts pathophysiology?
Usually in extrahepatic ducts (CBD and HDs), sometimes in intrahepatic ducts
Type I cysts are most common-lower end of CBD joins up with pancreatic duct: pancreatic juice and bile mix leading to a weakening of bile duct wall and ballooning- can lead to pancreatitis and cholangitis
Management of choledochal cyst
USS to identify
Commonly incidental finding
Surgery for removal of cyst
Presentation of choledochal cyst
May have jaundice, intermittent abdo pain, cholangitis, peritonitis, lump, pancreatitis
Aetiology of neonatal hepatitis syndrome
in 20%- viral (cytomegalovirus, rubella, hep a/b/c)
80%- nonspecific virus
Presentation of neonatal hepatitis syndrome
Usually at 1-2 months
Jaundice, not gaining wt/ht, hepatosplenomegaly
Management of neonatal hepatitis syndrome
Ix: liver biopsy and bloods
Mx: none specific, may need vitamins, potentially a liver transplant
Presentation of liver failure in children
Increased AST and ALT with prolonged clotting +/- jaundice +/- encephalopathy
Causes of liver failure
Viruses eg HSV, EBV, CMV, Hep A/B/E
Inherited metabolic disorders eg Wilsons, mitochondrial
Toxins
Medication eg erythromycin
Autoimmune
Low blood flow eg HF
Chronic: hep C, autoimmune, haemochromatitis, alpha 1 antitrypsin deficiency, CF, biliary atresia, sclerosing cholangitis
Give 3 signs of UTI in children.
- Fever.
- Miserable.
- Vomiting.
- Dysuria.
What investigations might you do on a child who you suspect has a UTI?
- Urine dip.
- MCS on clean catch urine. A sample can also be obtained using an in-out catheter.
- USS KUB.
- DMSA - renal scarring.
- MCUG - reflux.
What is the treatment for UTI in children?
IV cefuroxime.
Switch to PO trimethoprim if stable.
How would you treat a UTI that has been caused by ESBL e.coli?
You would give meropenem.
ESBL bacteria are resistant to all penicillins and cephalosporins.
Presentation of pyelonephritis
At least 38 degrees and bacteriuria
or
Less than 38 degrees and loin pain/tenderness and bacteriuria
When would you expect children to be dry overnight
Age 5
Factors contributing to nocturnal enuresis
Small bladder, inability to recognise full bladder, hormones, UTI, sleep apnoea, diabetes, chronic constipation, structural problem in urinary tract or nervous system
Management of nocturnal enuresis
<5= reassurance and advice
Non drug treatment: fluid intake, diet, toilet behaviour, rewards system then enuresis alarm if not responding (still more than 1-2 bed wets in a week)
Drug= desmopressin if over 5 +/- enuresis alarm
Imipramine hydrochloride is last line
Causes of acute renal failure
Hypoperfusion (blood loss, surgery, shock)
Blockage of urinary tract
Nephrotoxic drugs
Haemolytic uraemic syndrome (e.coli usually)
Glomerulonephritis
Symptoms of acute renal failure
Haemorrhage, fever, rash, bloody diarrhoea, vomiting, anuria or polyuria, pallor, oedema, eye inflammation, stomach mass
Treatment of acute renal failure?
Treat underlying cause
U+Es
Dietary changes
Antihypertensives
Iv fluids
Diuretics
How long for it to be classed as chronic renal failure?
Potential causes?
Over 3 months
Causes: long term blockage, alport syndrome, nephrotic syndrome, PKD, cystinosis, chronic conditions eg diabetes, untreated acute kidney disease
What is alport syndrome
Inherited condition- X-linked most common, but can be autosomal recessive/dominant
Deafness, renal damage and eye defects
Treatment of chronic renal failure
Diuretics
Dialysis and transplant
Diet changes (limit protein, potassium, phosphorus, sodium)
Growth promoters/bisphosphonates/iron tablets
What defines nephrotic syndrome
Generalised oedema, heavy proteinuria (>200mg/mmol) and hypoalbuminaemia (<25g/L)
(Flattened podocytes allow protein leakage)
(Normal protein less than 20mg/mmol, normal albumin 35-45g/L)
Causes of nephrotic syndrome
Which is most common?
Minimal change disease=most common
Others= congenital nephrotic syndromes, focal segmental glomerulosclerosis, mesangiocapillary glomerulonephritis
Management of nephrotic syndrome
High dose steroids- most respond well but some will have relapse (=steroid-resistant nephrotic syndrome)- in which case may need maintenance low dose steroids or immunomodulatory drugs eg cyclophosphamide
Low salt diet, prophylactic abx (as they leak Igs and steroids!), immunisations
Prednisolone for first episode 60mg/m2 for 4-6 weeks then wean down
What is minimal change disease
Microscopically damage not evident. Idiopathic
Develop nephrotic syndrome more quickly but much more responsive to treatment
What is congenital nephrotic syndrome
What is needed in management
Genetic condition (parents need 1 faulty and 1 healthy gene each to pass on)
Requires frequent albumin infusions to aid growth and development
Typically leads to renal failure
Causes of nephritic syndrome
Post-streptococcal glomerulonephritis, henoch schnolein purpura, alport syndrome, sle/lupus nephritis
Describe post-strep glomerulonephritis
10-14 days after skin/throat infection with group A strep
Haematuria, oedema, decreased urine output, htn, proteinuria, tired
Normally self limiting so needs supportive tx only
If strep infection persisting= penicillin
What is henoch schnolein purpura
IgA vasculitis
Rash of raised red or purple spots usually on legs and bottom (extensor surfaces)
Px of henoch schonlein purpura
Rash, arthritis, abdo pain, kidney impairment (self limiting and mild usually), may get PR bleeding and intussusception
Usually all preceded by 2-3 weeks of fever, headache, arthralgia/myalgia, abdo pain
Mx of Alport syndrome
ACEi/ARB, diuretics, limit sodium
Monitor for potential renal failure
Mx of Lupus nephritis
Immunosuppressants eg cyclophosphamide or hydroxychloroquine, antihypertensives, may need steroids
Types of hypospadias
When does it form
Subcoronal (near head of penis)
Midshaft
Penoscrotal (where penis and scrotum meet)
Weeks 8-14 of pregnancy
Management of hypospadias
Surgery between 3 and 18 months, may need to be done in stages
Types of renal malformation?
Autosomal recessive polycystic kidney disease
Duplication (single renal unit with more than one collecting system)
Fusion (kidneys joined, but ureters enter bladder on each side)- most common= horseshoe
Malrotation (little clinical significance)
Multicystic dysplastic kidney (nonfunctioning renal unit but contralateral kidney normally fine)
Renal agenesis (bilat=fatal, unilateral)
Renal ectopia=kidney fails to ascend from true pelvis
Renal hypoplasia (underdeveloped)
Pathophysiology of vescioureteral reflux
Urine flows from bladder back into ureters, potentially causing infection
Shorter than normal attachment between ureter and bladder and incompetent valve
Describe the different grades of vesicoureteral reflux
I=into ureter
II=into ureter and renal pelvis without distension
III=into ureter and renal pelvis with mild swelling
IV= mod swelling
V=severe swelling and twisting or ureter
Management of vesicoureteral reflux
Can go away by itself (junction gets longer with age)
Encourage frequent voiding
May need surgery to stop reflux
Triad of haemolytic uraemic syndrome
Coombs test negative, thrombocytopenia, AKI
Typically a/w e.coli
What is haemolytic uraemic syndrome
Toxins bind from e.coli causing thrombin and fibrin to be deposited in microvasculature
RBCs damaged while passing through occluded small vessels causing haemolysis
Px haemolytic uraemic syndrome
Profuse diarrhoea turning bloody 1-3 days later, fever, abdo pain and vomiting
Mx of HUS
Supportive- fluid and electrolyte mx, dialysis PRN, antihypertensive, keep circulating volume up
General management of eczema?
Avoid triggers, keep nails short, emollients
Rest of treatment depends on severity: may include corticosteroids, abx, antihistamines, calcineurin inhibitors, phototherapy, immunomodulators or biologic medications
What steroid would you give for mild eczema?
Hydrocortisone 1%
What steroid for moderate eczema?
Betamethasone valerate 0.025% or clobetasone butyrate 0.05%
What steroid for severe eczema?
Betamethasone valerate 0.1%
May need maintenance steroids or topical calcineurin inhibitors eg tacrolimus
What is Stevens-Johnson syndrome?
Toxic epidermal necrolysis. Rare, acute and potentially fatal skin reaction with sheet-like skin and mucosal loss
Nearly always caused by medications
What medications commonly cause Stevens-Johnson syndrome
Cotrimoxazole, penicillin, cephalosporins, anti-convulsants, allopurinol, paracetamol, NSAIDs
Usually within the first week of abx therapy
Presentation of Stevens-Johnson syndrome
Initially a flu-like illness
Then, tender/painful red skin rash on trunk. extending to face and limbs over a course of hours-days
(Skin lesions: macules/diffuse erythema/targetoid/blisters)
The blisters merge to form sheets of skin detachment
At least 2 mucosal surfaces affected eg conjunctivitis/mouth ulcers/cough/diarrhoea
Acute phase lasts 8-12 days
Management of Stevens Johnson syndrome
Skin biopsy shows keratinocyte necrosis
SCORTEN illness severity score to predict mortality
Cessation of suspected causative drug, admission, analgesia, temp maintenance, nutrition and fluids
General care of affected mucosal surfaces
Classification of allergic rhinitis?
Seasonal (if due to grass and tree pollens=hay fever)
Perennial (throughout year, due to dust mites and animal dander)
Intermittent (less than 4 days/week or less than 4 consecutive weeks)
Persistent (more than 4 days/week or more than 4 consecutive weeks)
Occupational
Management allergic rhinitis? Mild-mod
Nasal irrigation with saline
Allergen avoidance
PRN antihistamine- intranasal as first line eg azelastine
Can use 2nd generation non sedating oral eg loratidine or cetirizine for mild-mod symptoms
What can be used in mod-severe allergic rhinitis?
Intranasal corticosteroid during periods of allergen exposure eg mometasone fluroate or fluticasone propionate
For hayfever, what pollens affect you when?
Tree=early-late spring
Grass=late spring-early summer
Weed=early spring-late autumn
What causes urticaria?
Histamine release
Due to: allergens, bee and wasp stings, autoimmune/idiopathic (chronic), physical triggers eg cold/friction, medications
Types of antihistamine
Non sedating, sedating, short and long acting=H1 antihistamine
H2 antihistamines
What is angio-oedema?
Swelling deep to the skin, commonly soft areas of skin eg eyelids and lips, more painful than weals/hives and takes longer to clear.
Presentation of anaphylaxis
Skin features-urticaria, erythema, flushing, angio-oedema
Involves at least 1 of Resp/CV/GI system
How to manage anaphylaxis
ABCDE, high flow oxygen, IM adrenaline, repeat 3-5 mins if required, treat a drop in BP with fluid bolus, consider salbutamol if wheeze, antihistamines for itch(cetirizine)
Prescribe an epipen
What are the most common vascular birth marks?
Macular stains or salmon patches: Angel’s kisses (forehead/nose/upper lip/eyelids- disappear with age) or Stork Bites (back of neck, disappear with age)
Hemangioma (grow rapidly 6-9 months then shrink and lose red colour)
Port-wine stain/nervus flammeus (grows with the child, often face/arms/legs, flat pink/red/purple mark)
What are the most common pigmented birth marks?
Moles/congenital naevi, cafe au lait spots, mongolian spots (blue-grey spots on lower back or buttocks, common on darker skin)
Causes of infectious rash in children
Meningococcal, steven-johnsons syndrome, impetigo, kawasaki, staphylococcal scalded skin syndrome, eczema herpeticum, erythema nodosum, erythema multiforme, measles, glandular fever, hand foot and mouth disease, erythema infectiosum, chicken pox, nappy rash, scabies, tinea corporis, tinea capitis, mollusucm contagiosum
What are the three phases in Kawasaki disease?
Acute phase=weeks 1-2
Subacute phase= weeks 2-4
Convalescent phase=weeks 4-6
Pathophysiology of kawasaki disease?
Idiopathic. Not contagious.
systemic vasculitis
Mainly affects under 5s.
What are NICE criteria for diagnosis of kawasaki?
Over 38 degrees for at least 5 days and at least 4 key symptoms:
Bilateral conjunctival infection, mouth/throat changes, hand and feet changes, rash, swollen glands
How to manage kawasaki disease?
IV immunoglobulin and high dose PO aspirin (to prevent thrombosis)
What should you be aware of when giving children aspirin?
Reyes syndrome: persistent vomiting, lethargy, liver and brain damage
Presentation of measles
Initially coryzal sxs, fever, small grey-white spots in mouth (Koplik spots) lasting a few days
2-4 days later= maculopapular rash on head/neck and spreads to rest of body
Management of measles
Self limiting viral illness and usually clears in about 7-10 days
Mx: stay at home for at least 4 days from when rash first appears, antipyretic, fluids, gently clear crust from eyes, hot steam
PREVENTION: MMR vaccination at 12-13 months and 3 years 4 months
What are some complications of measles?
dehydration, otitis media, conjunctivitis, laryngitis, airway infections, febrile seizures
What causes chicken pox?
Varicella zoster virus
Presentation of chicken pox?
Fever, aches and headache for 1 day preceding
rash: small itchy blisters that scab over
Management of chickenpox?
AVOID aspirin (reye's syndrome) Antipyretic, emollient, sedating antihistamine at night, keep nails short, dress comfortably, fluids Stay off school until all blisters crusted over
If an at risk group eg under 1 month, immunocompromised, severe heart/lung disease=aciclovir or vaccination
When are you infectious with chickenpox?
7-21 day incubation
Infectious from 2 days before spots appear until all blisters crusted over
What type of infection is rubella? When is peak incidence?
Viral. Mainly in spring and early summer
Presentation of rubella?
Prodromal phase: low grade fever, headache, mild conjunctivitis, anorexia, rhinorrhoea
Rash occurs 14-17 days after exposure: pink discrete macules that coalesce behind ears and on face, spreads to trunk then extremities
Lymphadenopathy-cervical, suboccipital, post auricular
Management of rubella?
PCR is gold standard diagnosis
Keep out of school for 4 days after rash appears, antipyretics, ask re contact with pregnant women
PREVENTION: MMR vaccination at 12-13 months and 3 years 4 months
What causes diphtheria?
Gram positive aerobic bacillus Corynebacterium diphtheriae causing acute upper resp tract infection
Presentation of diphtheria?
Early sxs=coryzal
Nasal discharge- watery then purulent and bloody
Membranous pharyngitis with fever, enlarged cervical LNs, oedema of soft tissues= bull neck appearance
Cutaneous infection: pustules rupture causing punched out ulcers often on lower legs, feet and hands, with surrounding oedematous pink/purple skin. Takes 2-3 months to heal leaving a depressed scar
May cause difficulty swallowing, paralysis and HF
Management of diphtheria?
PREVENTION: 6-in-1 vaccination at 2, 3 and 4 months
Antibiotics (eyrtho/azithro/clarithro/penicillin), reinforcing vaccine dose, manage contacts with abx prophylaxis
Booster vaccinations: first between 3.5 and 5 years, second between 13-18
Pathophysiology of scalded skin syndrome?
Staphylococcal disease. Also known as Ritter disease and Lyell disease
Affects under 5s, especially neonates normally
Exotoxins from S. aureus bind to desmosomes, causing skin cells to become unstuck- causes red blistering skin
Presentation of scalded skin syndrome?
Fever irritability and widespread erythema, then 24-48 hours later=fluid filled blisters form and rupture easily
Nikolsky sign positive= gentle strokes causes exfoliation
Investigations for scalded skin syndrome?
Tzanck smear, skin biopsy, bacterial culture
Management of scalded skin syndrome?
Admit to hospital
IV antibiotics eg flucloxacillin
Supportive -antipyretic, fluids, petroleum jelly
Healing usually complete within 5-7 days of starting treatment
Pathophysiology of whooping cough?
Bordetella pertussis bacterial infection
Gram negative bacillus. Toxins paralyse cilia and promote inflammation
Describe the first phase of whooping cough?
1st phase=catarrhal phase for 1-2 weeks
Coryzal sxs: rhinitis, conjunctivitis, irritability, sore throat, low grade fever, dry cough
Describe the second phase of whooping cough?
2nd phase=paroxysmal for 2-8 weeks
Severe paroxysms of coughing followed by inspiratory gasp (whoop)
NB/ in infants under 3 months the whoop is less common and apnoea is more common. Paroxysms more common at night and often followed by vomiting
Describe the third phase of whooping cough?
Convalescent phase=up to 3 months
Investigations for whooping cough?
If cough for less than 2 weeks: nasopharyngeal aspiratr
If cough for more than 2 weeks: anti pertussis toxin IgG serology in under 5s or toxin detection in oral fluid for 5-17year olds
FBC=lymphocytosis
Management of whooping cough?
Hospital if under 6 months and acutely unwell, sig breathing difficulties, feeding difficulties, complications eg pneumonia
Macrolide antibiotic if cough for less than 21 days= clarithromycin (under 1 month), azithromycin otherwise
Supportive
NB/ abx don’t alter course once disease established
PREVENTION= vaccination at 2,3,4 months and booster at 3 years 4 months
Presentation of polio?
Fever, tiredness, headache, vomiting, neck stiffness and arm/leg pain
In 1 in 200 cases, affects NS leading to temporary or permanent paralysis
Management of polio?
No treatment; need physio and OT
PREVENTION= vaccination at 2,3,4 months, 3years 4 months and 13-18 years
How to diagnose TB in children?
Mantoux test= positive tuberculin skin test
IGRA
CXR
History of contact
Lab cultures very difficult to do in kids
TB drugs?
RIPE
Rifampicin, isoniazid, pyrazinamide, ethambutol
Who gets BCG vaccination?
If born in areas of UK where rates are high
If a parent/grandparent born in a country with high TB rates
Causes of meningitis in children?
Bacterial: Mainly N. meningitidis and S. pneumoniae
In neonates= GBS (group B strep from mum’s vagina)
Viral: HSV, enterovirus and VZV
What is the presentation of meningitis in neonates and babies?
Non-specific!
Eg Hypotonia, poor feeding, lethargy, hypothermia, bulging fontanelle
How to investigate for meningitis?
Kernigs and brudzinski’s signs
LP if under 1 month and fever, 1-3 months fever and unwell, under 1 year with unexplained fever and other features of serious illness
Management of meningitis?
Primary care: benzylpenicillin
Secondary care bacterial:
If under 3 months: cefotaxime and amoxicillin (to cover listeria)
Over 3 months=ceftriaxone
Dexamethasone QDS for 4/7 if LP suggestive bacterial meningitis
PEP for contacts: ciprofloxacin
Viral=aciclovir
What CSF findings are suggestive of bacterial meningitis?
Cloudy, high protein, low glucose, high neutrophils, cultured for bacteria
What CSF findings are suggestive of viral menigitis?
Clear, normal protein, normal glucose, lymphocytosis, negative culture
Causes of encephalitis?
Most common=HSV 1(children) and HSV 2(neonates- from genital warts)
Others= VZV, CMV, EBV, enterovirus, adenovirus, influenza virus
Presentation of encephalitis?
Altered consciousness, altered cognition, unusual behaviour, acute onset focal seizures and focal neurological symptoms, fever
Management of suspected encephalitis?
Ix: LP (CSF and PCR) or CT if LP CI. MRI after LP, swabs, HIV testing
Mx: aciclovir for HSV/VZV or ganciclovir (CMV)
Repeat LP to ensure successful tx
How does HSV1 affect children?
Infectious for 7-12 days
Commonly gingivostomatitis: fever, restless, dribbling, swollen gums which bleed easily, foul breath, ulcers on tongue/throat/palate/inside cheeks, enlarged LNs
Management of HSV?
Can use antivirals eg aciclovir/valaciclovir but for mild/uncomplicated eruptions require no treatment
What is slapped cheek syndrome caused by?
Also known as fifth disease/erythema infectiosum
Parvovirus B19
When are the peak incidences for slapped cheek syndrome?
april and may
Presentation of slapped cheek syndrome?
Commonly 3-15 years old
Rash=bright red scald-like on 1 or both cheeks, painless
May have mild coryzal symptoms
1 in 4 will be asx
By time rash develops, no longer infectious
Management of slapped cheek syndrome?
None needed. Caution around pregnant women
Types of impetigo and what caused by?
Non-bullous (about 70% cases)= S.aureus, S.pyogenes or both
Bullous= S.aureus
Presentation of impetigo?
Non-bullous= golden/brown crusts from vesicles/pustules, commonly on face, limbs and flexures, may be mildly itchy
Bullous-more common in infants= blisters rupture leaving flat yellow/brown crust, may have systemic features
Management of impetigo?
Good hygiene, stay away from school until healed or 48 hours after abx
Localised non-bullous=hydrogen peroxide 1% cream (2nd line=fusidic acid 2%)
Widespread non-bullous or bullous=oral flucloxacillin
How to manage candida?
topical antifungal eg miconazole gel
If vaginal (v. uncommon before puberty)= fluconazole
Causes of toxic shock syndrome?
Staph or strep- from female barrier contraceptives, tampons, injury on skin, childbirth, nasal packing, ongoing staph or strep infection
Presentation of toxic shock syndrome?
Fever, flu-like sxs, N+V, diarrhoea, widespread sunburn like rash. lips/tongue/whites of eye turning bright red, dizziness and syncope, dyspnoea, confusion
Management of toxic shock syndrome?
Antibiotics and fluids
What causes scarlet fever?
Group A strep- affects people with recent strep throat or impetigo. Children over 3 at risk
Symptoms of scarlet fever?
Sudden fever a/w sore throat, lymphadenopathy, headache, N+V, loss of appetite, swollen and red strawberry tongue, abdo pain, malaise
12-48 hours after fever=rash: starts below ears/neck/chest/armpit/groin)- scarlet blotches (boiled lobster appearance). Then looks like sunburn with goose pimples, pastia lines (red streaks from ruptured capillaries)
Management of scarlet fever?
Penicillin
Mostly follows benign course
What is Coxsackie’s disease?
Hand foot and mouth disease, generally affects under 10s.
Group A coxsackie virus- faecal-oral transmission
Presentation of coxsackie’s disease?
Prodromal phase then tender oral ulcerative lesions and maculopapular lesions on hands and feet
Management of coxsackie’s disease?
Supportive mx-fluids, soft diet, antipyretic analgesics, don’t need to isolate the child
Symptoms improve within 3-6 days with full resolution within 7-10 days
Red flags for primary immunodeficiency in kids?
faltering growth, need IV abx for infections, FH, at least 4 infections a year, at least 2 sinus infections a year, at least 2 pneumonias in past 3 years, frequent deep tissue abscesses, persistent fungal infections (over 6 months), at least 2 deep seated infections over 3 years, at least 2 months on at least 2 abx with little effect
Diagnosis of diabetes in children?
Random plasma glucose > 11.1 mmol/L if symptomatic
Normal fasting blood glucose? post prandial glucose? ketones?
Fasting: 3.5-5.6mmol/L
Post prandial: <7.8
Ketones: <0.6
DKA management?
Fluid, insulin, monitor glucose hourly, monitor electrolytes especially potassium and ketones 2-4 hourly, hourly neuro obs
Fluids in DKA?
0.9% NaCl 20ml/kg bolus if shocked, 10ml/kg if not shocked
Maintenance and deficit fluids
5% fluid deficit in mild DKA
7% deficit in moderate
10% deficit in severe
Do no subtract boluses given for shock
Initial management of type I diabetes mellitus in kids?
Aim for glucose 4-7: need to be able to check this
Give insulin (pump or injection): basal bolus regime most common
Carb counting
Advice for hypos
Lifestyle advice
Carb counting in DMT1?
Insulin:carb ratio= 1 unit for every 15 grams of carb
Correction factor= 1 unit insulin brings blood glucose down by 8
Aim for glucose of 6
Presentation of hypoglycaemia?
Autonomic fxs: irritable, hungry, nauseous, shaky, anxious, sweaty
Neuroglycopenic fxs: confused, drowsy, headache, coma, seizures
Management of hypoglycaemia?
Check levels to confirm
Glucose tablets/gel/containing food or drink first
Check glucose in 15 mins
Follow up with longer acting carb (bread or biscuit)
Severe hypo (requiring additional assistance from another person)= IM glucagon
Long term management of diabetes?
Aim for HbA1c less than 48mmol/mol
Monitoring at least annually: eyes, urine, feet, BP, injection sites, bloods
Types of cryptorchidism?
True undescended= lies along normal path of descent but never present in scrotum
Ectopic testis= lies outside normal path and outside of scrotum
Ascending testis=previously in scrotum but higher now
Absent/atrophic testis=non palpable testis was in scrotum at birth but later disappears
How to manage ?cryptorchidism?
6-8 week baby check exam
If not descended, re examine at 4-5 months to see if spontaneous descent
If not surgical treatment (orchodexy) before 12 months of age
Pathophysiology of testicular torsion?
Testis twists around spermatic cord and cuts off the blood supply to the testicle
Presentation of testicular torsion?
Sudden severe pain on one side of the scrotum, scrotal skin swells and turns red
Nausea and vomiting potential
May have just abdominal pain
Management of testicular torsion?
Surgical emergency: blood supply must be restored within 6 hours or the testicle will infarct
If surgery delayed past 12 hours, 75% will need orchidectomy
Newborns with torsion- testes almost always infarcted so need orchidectomy
What is a bell clapper deformity?
No tissue holding the testes to the scrotum so testes swing inside the scrotum= higher risk of torsion
Cut offs for precocious puberty?
Girls under 8
Boys under 9
2 types of precocious puberty?
Gonadotrophin dependent/ central precocious puberty= true precocious puberty with premature activation of the HPG axis
Gonadotrophin independent/precocious pseudopuberty= due to increased production of sex hormones
Causes of central precocious puberty?
Mainly idiopathic or abnormalities of CNS
Causes of precocious pseudopuberty?
CAH, tumours (HCG secreting), McCune- Albright syndrome, silver-russell syndrome, testotoxicosis, exogenous androgen exposure, severe hypothyroidism
What can you give for true precocious puberty?
GnRH agonists
Define delayed puberty
Girls= absence of breast development by 13 or primary amenorrhoea with normal breast development by 15 Boys= absence of testicular development by age 14
Causes of delayed puberty
Most common= constitutional delay in growth and puberty (CDGP)
Central causes= CDGP, chronic illness, malnutrition, excessive exercise, psychosocial deprivation, steroids, hypothyroid, tumours, congential anomalies, irradiation treatment, trauma, idiopathic hypogonadotrophic hypogonadism
Peripheral causes= bilat testicular damage, syndromes eg Prader-Willi, Klinefelter, rugs. irradation, Turner syndrome, intersex disorders, PCOS
How to manage delayed puberty?
Most are CDGP so need reassurance and monitoring
Can start course of sex hormones (low dose testosterone or gradual increase of oestrogen then cyclical progestogen)
Causes of congenital hypothyroidism
Thyroid dysgenesis, thyroid hormone metabolism disorders, hypothalamic/pituitary dysfunction,
What happens in congenital hypothyroidism isn’t treated?
cretinism: long term irreversible neurological problems and poor growth with intellectual disability
spasticity, gait problems, dysarthria, profound mental disability
How to treat congenital hypothyroidism?
levothyroxine titrated to TFTs
Define gonadotrophin deficiency/hypogonadotrophinism
Low serum concentrations of LH and FSH in presence of low circulating concentrations of sex steroids
How to manage gonadotrophin deficiency?
Induce and maintain secondary sexual characteristics- testosterone and hCG or oestrogen and progestogen
What is Kallmann syndrome?
Hypothalamic gonadotrophin releasing hormone deficiency (GnRH) and deficient olfactory sense (hyposmia or anosmia)
X linked recessive or autosomal recessive
Greater penetrance in males
Presentation of Kallmann syndrome?
Females= primary amenorrhoea Males= cryptorchidism, micropenis
Most are normal stature
Lots have associated stigmata- nerve deafness, colour blind, cleft palate, renal abnormalities
How to manage Kallmann syndrome?
Exogenous sex steroids to induce and maintain secondary sexual characteristics
What is congenital adrenal hyperplasia?
Group of autosomal recessive disorders of cortisol biosynthesis+/- aldosterone +androgen excess
21-hydroxylase deficiency
What is the classic form of CAH?
Ambiguous external genitalia in girls
Boys have no signs at birth
What is the salt-losing form of CAH?
Boys present at 7-14 days with vomiting weight loss, lethargy, hyponatraemia, hyperkalaemia and dehydration
Girls will be identified before a crisis due to genitalia
How to manage classic CAH?
Glucocorticoid replacement= hydrocortisone
+/- Mineralcorticoids= fludrocortisone
Salt losing form need NaCl
Consider surgery for genitalia
Types of androgen insensitivity syndrome? describe
Complete=testosterone has no effect so genitals are entirely female
Partial= testosterone has some effect so genitals not as expected eg penis underdeveloped, fully/partial cryptorchidism, some female genitalia.
How will females with complete androgen insensitivity syndrome present? and partial?
Complete=Develop breasts but amenorrhoea, no pubic or axillary hair, no womb or ovaries
Partial=enlarged clitoris, undescended testicles, hypospadias, often raised as boys
Causes of childhood obesity?
Diet, exercise lack, sleep deprivation (low leptin and high ghrelin), genetics, socio-economics, medication, endocrine eg PCOS/hypothyroid/Cushings/Prader-Willi
Name 2 drugs that can be given as meningitis prophylaxis.
- Rifampicin.
- Ciprofloxacin.
2-years-old, 2m history of malaise, pallor and reduced appetite. Occasional febrile episodes associated with a pink rash and soreness in her left thigh. Now reluctant to weight bear despite walking at 13 months. All immunisations are up to date and developmental history shows no concerns.
O/E: low grade fever and cervical lymphadenopathy.
ESR is significantly raised.
Give 3 differentials.
- JIA.
- ALL.
- Transient synovitis.
- Septic arthritis.
3-year-old, 7-day history of high fevers. Now developed red eyes, a rash, sore mouth and throat. Miserable and unwell with a diffuse maculopapular rash on his torso. He has bilateral injected conjunctiva, red cracked lips and a strawberry tongue. 3x2cm cervical swelling and swollen reddened palms.
Give 3 differentials.
- Kawasaki disease.
- Scarlet fever.
- Shingles/chickenpox.
- Measles - ask about immunisation history.
Commonest cancer in children?
Leukaemia- ALL and then AML
Pathophysiology of acute lymphoblastic leukaemia?
Disruption of lymphoid precursor cells in the bone marrow leading to excessive production of immature blast cells: causes drop in functional RBCs/WBCs/platelets
Presentation of leukaemia in children?
Anaemia (lethargy, pallor)
Thrombocytopenia (easy bruising/bleeding)
Leukopenia (fevers, infections)
May complain of bone pain
Non specific sxs (weight loss, malaise)
Investigations for leukaemia?
FBC (pancytopenia, thrombocytopenia with significant lymphocytosis), serum biochemistry
Blood films (blast cells)
CXR (exclude mediastinal mass)
Bone marrow aspirate confirms diagnosis
LP
Management of leukaemia in children?
Chemo, supportive care with blood products, prophylactic anti-fungals
Chemo in 5 phases (induction, consolidation, interim maintenance, delayed intensification, maintenance)
Maintenance= 2 years for girls and 3 years for boys
Stem cell transplants in high risk patients in 1st remission or relapsed patients
Name 5 different types of primary brain tumour?
Astrocytoma, medulloblastoma, diffuse midline glioma, ependymoma, craniopharyngioma, embryonal tumours, pineoblastoma, brainstem glioma, choroid plexus carcinoma, germ cell tumour
What is the most common brain and spinal cord tumour in children (apart from mets)?
Astrocytoma
Presentation of brain tumour?
morning headaches, nausea/vomiting, diplopia, seizure, increased head circumference, bulging fontanelle, signs of raised intracranial pressure
4 main types of astrocytoma?
Grade 1=pilocytic astrocytoma
Grade 2=diffuse (fibrillary) astrocytoma
Grade 3=anaplastic astrocytoma
Grade 4=glioblastoma multiforme
80% are low grade (1 or 2)
What is the most common high grade brain tumour in children?
Medulloblastoma (2nd most common overall)
Most commonly found in cerebellum
What is Wilm’s tumour? How presents?
Most common paediatric renal mass= nephroblastoma
Painless upper quadrant abdominal mass, haematuria in 20%, hypertension in 25%, ,may have acquired von willebrand disease
how to manage wilm’s tumour?
Nephrectomy and chemo
Cure in about 90% cases
How does neuroblastoma present?
Presents late- majority of symptoms due to mass effect or mets.
Systemic fxs, bruising, weakness/paralysis/BB dysfunction, bone pain, abdominal distension, htn, horner’s syndrome
Almost all are stage 3 or 4 at diagnosis
Where does neuroblastoma arise from?
Adrenal or paraspinal sites most common
arises from developing sympathetic nervous system
Investigating neuroblastoma?
FBC, esr, pt, catecholamine by products in urine, CT, MRI for paraspinal lesions, biopsy
Presentation of retinoblastoma?
Leukocoria (abnormal appearance of pupil), visual deterioration, red and irritated eye, faltering growth, squint, absent red reflex
Management of retinoblastoma?
Eye conserving treatments eg cryo/thermotherapy, photocoagulation, chemo
Enucleation for all unilateral tumours filling over half of the eye or invading eye structures and subsequent fitting of prosthesis
V sensitive to radiotherapy
Which childhood cancer has the best overall survival?
Retinoblastoma (about 99%)
What are the 2 main types of primary bone tumour in in children?
Osteosarcoma (more common) or ewing’s sarcoma (v rare)
Presentation of hepatoblastoma?
Abdominal mass +/- poor appetite, weight loss, lethargy, fever, vomiting, jaundice
Investigating hepatoblastoma?
Raised alpha fetoprotein
What immunisations at 8 weeks?
6 in 1
rotavirus
men B
What is contained in the 6 in 1 vaccination?
diphtheria, hepatitis b, Hib, polio, tetanus, whooping cough
What immunisations at 12 weeks?
6 in 1, PCV (pneumococcal), rotavirus
What immunisations at 16 weeks?
6 in 1, Men B
What immunisations at 1 year?
Hib/Men C, MMR, PCV, MenB
What immunisations at 3 years 4 months?
MMR, 4 in 1 pre school booster (diphtheria, tetanus, whooping cough, polio)
What immunisations at 12-13 years?
HPV
What immunisations at 14 years?
3 in 1 teenage booster (tetanus, diphtheria, polio), MenACWY
What is klinefelter’s syndrome?
Extra X chromosome (commonly 47XXY)
How does klinefelter’s syndrome present in kids?
Delayed speech or learning difficulties, rapid growth in mid-childhood or truncal obesity, hypogonadism
Small firm testes, decreased facial and pubic hair, loss of libido, impotence, gynaecomastia, history undescened testes
Infertility
Diagnosis of klinefelter’s? Management?
Low/low-normal testosterone, elevated FSH and LH, chromosomal analysis
Testosterone replacement
What is Turner’s syndrome?
Loss or abnormality of 2nd X chromosome in phenotypic girls
45X
Different age presentations of Turners syndrome?
Newborns: borderline small, lymphoedema of hands/feet, excessive skin at nape of neck, cardiac abnormalities
Infants: close to third centile, feeding difficulties, poor weight gain, poor sleeping
Preschool: short stature, recurrent middle ear infections, behavioural difficulties, high activity levels
Adolescence: ovarian failure, impaired pubertal growth spurt, obesity, htn, higher prevalence immune disorders, foot problems, learning disabilities
Can have dysmorphic features eg webbed neck, short fingers, lymphoedema, high palate
Can have renal and heart problems
how to diagnose turner’s syndrome?
Via amniocentesis
Chromosomal analysis
Measure gondatrophins, TFTs, renal and heart tests, bone age
Management of turner’s syndrome?
MDT
Short stature- GH therapy
May supplement with oestrogen
Gonadal failure-combined oestrogen and progestogen
Neonatal features of down’s syndrome?
Hyperflexibility, muscular hypotonia, transient myelodysplasia, bradycephaly, oblique palpebral fissures, epicanthic folds, ring or iris speckles (brushfield spots), low set/folded ears, stenotic meatus, flat nasal bridge, protruding tongue, high arched palate, loose skin on nape of neck, single palmar crease, short little finger, short broad hands, gap between hallux and second toes, congenital heart defects, duodenal atresia
What is down’s syndrome? Biggest risk factor?
trisomy 21
maternal age
Management of down’s syndrome?
Annual health checks-hearing, resp (OSA), eyes, heart, neurological, GI, hip, thyroid. Extra support in academia and help with living.
What is edward’s syndrome?
Trisomy 18
Presentation of edward’s syndrome?
More likely to be female
Low birth weight, craniofacial abnormalities (low set and malformed ears, micrognathia, prominent occiput, small facial features, microcephaly, cleft lip), skeletal abnormalities, congenital heart defects, GI abnormalities, GU abnormalities (horse shoe kidney), neurological problems, pulmonary hypoplasia
Management of edward’s syndrome? What to take into account when making decisions?
Feeding difficulties common, 38.5% foetuses die during labour
Chance of survival to 3 months is 20% and to 1 year=8%
May decide to not give life sustaining treatment.
Otherwise manage cardiac failure and resp insufficiency
What is patau syndrome?
Trisomy 13
Presentation of patau syndrome?
Many never survive until term and are stillborn or spontaneously abort
Low birth weight, congenital heart defects, holoprosencephaly causing midline facial defects, brain and cns abnormalities, GI and GU malformations
Life expectancy of patau syndrome?
median is 2.5 days. About 50% live over a week, 5-10% live over a year
What is fragile x syndrome?
Most common cause of sex linked general learning disability. Delayed milestones and typical features eg high forehead, large testicles, facial asymmetry, large jaw and long ears.
Potential diagnoses coming under fragile x syndrome?
ADHD, ASD, obsessive compulsive behaviours, emotional lability, aggressive behaviours, specific speech disorders eg echolalia and perseveration
Management of fragile x patients?
MDT approach and manage each syptom
No cure
Most diagnosed by age 3 usually
Name 4 types of muscular dystrophy?
duchenne muscular dystrophy ( most common and one of most severe) , myotonic dystrophy, facioscapulohumeral muscular dystrophy, becker muscular dystrophy, limb girdle muscular dystrophy, emery-dreifuss muscular dystrophy
Male infant not walking by 18 months- concern? what test?
Concerned about duchenne muscular dystrophy
measure creatine kinase
Presentation of ?muscular dystrophy?
Gower’s maneouvre (stands up by facing floor, wide feet, lift bottom first then hands walked up legs)
Waddling gait, lordosis/scoliosis, large calves and leg muscles compared to other muscles
Management of muscular dystrophy?
Depends on type and severity eg mobility and breathing assistance
Steroids
Ataluren if has DMD, is over 5 and can still walk
Creatine supplements
Treat swallow and heart complications
What features would make you suspect Angleman’s syndrome?
Developmental delay apparent by 6 months, but forward progression with no loss of skills once acquired
Sitting late at 12 months, walking late at 3-4 years, 10% can’t walk, flat and turned out feet, lean forward when run, speech impairment, laughter and smiling/happy demeanour, excitable personality. hand-flapping, short attention span, microcephaly, epilepsy, sleep disorders, excess chewing, drooling, fascination with water, hypopigmentation, prominent mandible
What is the lifespan of angleman’s syndrome?
normal lifespan
Presentation prader willi syndrome?
Hypotonia, developmental delay, obesity, learning disability, behavioural problems
Genetics of angleman’s syndrome?
Maternal deletion of chromosome 15 or paternal uniparental disomy
Genetics of prader willi syndrome?
Paternal deletion of chromosome 15 or maternal uniparental disomy
Diagnostic criteria for prader willi?
Between birth and 3 years: 5 points including 4 major
3 years-adult=8 points including 5 major
Major (1 point); hypotonia with poor suck, feeding problems, excessive weight gain 1-6 years, characteristic facies, hypogonadism, cryptorchidism, global developmental delay, hyperphagia, chromosomal abnormality
Minor (0.5 points); infantile lethargy, behavioural problems, sleep disturbance, short and failed pubertal growth spurt, hypopigmentation, small hands and feet, narrow hands, eye abnormalities, thick saliva, speech articulation defects, skin picking
Management of prader willi?
MDT
GH treatment to maintain normal growth
SSRIs trial
No role for appetite suppressants
What is noonan’s syndrome (formerly leopard syndrome)?
Autosomal dominant condition characterised by: congenital heart disease, short stature, broad and webbed neck, sternal deformity, developmental delay, facial features, cryptorchidism, increased bleeding tendency
What is william’s syndrome?
Rare autosomal dominant disease characterised by CV disease, distinctive facies, connective tissue abnormalties, intellectual disability, growth and endocrine abnormalities
Specific cognitive profile: good on verbal short term memory and language, but poor visuospatial construction
Overly friendly
Infantile hypercalcaemia
Management of william’s syndrome?
MDT management- manage infantile hypercalcaemia with diet and corticosteroids, yearly surveillance, monitoring CV complications
What is newborn respiratory distress syndrome?
Also known as hyaline membrane disease and surfactant deficiency lung disease
Usually affects premature babies who don’t have the necessary surfactant (enough produced by week 34 normally)
Presentation of newborn resp distress syndrome?
Peripheral and central cyanosis, rapid and shallow breathing, flaring nostrils, grunting
Management of newborn resp distress syndrome?
Treatment during prem labour= steroid injection before delivery and then a 2nd dose 24 hours later
Treatment after birth= extra oxygen if mild symptoms. More severe= ventilator, dose of artificial surfactant,IV fluids and nutrition
What warrants mild chronic lung disease of prematurity (bronchopulmonary dysplasia)?
Breathing room air at 36 weeks for babies born before 32 weeks
or
air by 56 days of postnatal age for babies born after 32 weeks
What warrants moderate chronic lung disease of prematurity?
Needing less than 30% oxygen at 36 weeks if born before 32 weeks
or
needing less than 30% oxygen at 56 days of postnatal age if born after 32 weeks
What warrants severe chronic lung disease of prematurity?
Need more than 30% oxygen to breathe +/- CPAP at 36weeks/56 days postnatal
What can be seen on CXR of a baby with chronic lung disease of prematurity?
Diffuse haziness, coarse interstitial pattern=atelectasis, inflammation, +/-pulmonary oedema
Management of chronic lung disease of prematurity?
Minimise ventilation associated lung injury, continuous oxygen monitoring, nasal CPAP, maintain o2 between 91-95%, send them home on oxygen
Potential: Dexamethasone short term, diuretics, methylxanthines eg caffeine (increases resp drive and decreases apnoea)
What is meconium?
Dark green faecal material produced during pregnancy, which is passed by the baby
Why is meconium aspiration bad?
Vasoactive and cytokine substances activate inflammatory pathways and inhibit the effect of surfactant
Causes varying degrees of respiratory distress in the newborn
Potential consequences: airway obstruction, foetal hypoxia, pulmonary inflammation, infection (chemical pneumonia), surfactant inactivation, persistent pulmonary htn
Management of meconium aspiration?
Depends on severity of resp distress eg obs, routine care, ventilation/oxygen, antibiotics, surfactant, inhaled nitric oxide if pulmonary htn, steroids
Prognosis of meconium aspiration?
80% have 3-4 day illness then discharged home
Complications= air leak, cerebral palsy, chronic lung disease
What is hypoxic ischaemic encephalopathy?
also known as intrapartum asphyxia
Brain injury caused by oxygen deprivation leading to death or severe impairment eg epilepsy, developmental delay, motor impairment, neurodevelopmental delay and cognitive impairment, cerebral palsy
Damage is permanent
What events could lead to asphyxia?
Acute maternal hypotension, cardiac complications, injury from umbilical cord, impaired blood flow to brain during birth, intrapartum haemorrhage, placental abruption, labour and delivery stress, trauma etc
What comes under TORCH infection?
Toxoplasmosis
Others= syphilis, parvovirus b19, vzv, enteroviruses, lymphocytic choriomeningitis virus, HIV, zika
Rubella
Cytomegalovirus
HSV
What do torch infections generally do?
Infections during pregnancy that cause congenital defects
Generally greater risk of harm if mum infected in early pregnancy
Can cause miscarriage or spontaneous abortion
Congenital toxoplasmosis issues?
Chorioretinitis with blindness, anaemia, hepatic and neurological symptoms
What can the ‘others’ of Torch infection cause?
Syphilis= bowed sabre shins, hutchinson teeth, typical facial appearance
Parvovirus b19= anaemia, hydrops fetalis, myocarditis
VZV= chorioretinitis, cataracts, limb atrophy, cerebral cortical atrophy, neurodisability
Zika= microcephaly, positional/hearing/ocular abnormalities
What can rubella do to the neonate?
Intrauterine growth restriction, intracranial calcifications, microcephaly, cataracts, cardiac defects, neuro disease
Blueberry muffin appearance
Most occur if exposure during first 16 weeks of pregnancy
What can cytomegalovirus do to the neonate?
Growth restriction, sensorineural hearing loss, intracranial calcification, microcephaly, hydrocephalus, hepatosplenomegaly, optic atrophy, delayed psychomotor development
More severe in 1st trimester, but more common in 3rd trimester
What drug can be used to stop the progression of hearing loss due to CMV?
Ganciclovir
Describe physiological jaundice in the neonate?
Increased rbc breakdown and immature liver function
Present at 2-3 days of age
Jaundice begins to disappear towards end of first week and resolved by 10 days
Bilirubin under 200 micromol/L and baby remains well
Causes of early neonatal jaundice aka onset in under 24 hours
Haemolytic disease (rhesus, ABO incompatibility, G6PD deficiency, spherocytosis)
Infection (toxoplasmosis/TORCH)
Haematoma
Maternal autoimmune haemolytic anaemia (SLE)
Gilbert’s syndrome
Dubin-Johnson syndrome
Crigler-Najjar syndrome
Causes of prolonged neonatal jaundice aka lasting over 14 days (term) or over 21 days (preterm)
Infection, hypothyroid, hypopituitarism, galactosaemia, breast milk jaundice, GI eg biliary atresia
How common is jaundice in the 1st week of life?
60% term and 80% preterm babies develop it
15% of healthy infants get physiological breastmilk jaundice
What is kernicterus?
A complication of neonatal jaundice- bilirubin encephalopathy (unconjugated)
Occurs mainly in premature babies.
Features of kernicterus?
Early features= jaundice, hypotonia, poor feeding, absent startle reflex
May then develop: high pitched cry, hypertonia of extensor muscles with arched back and hyperextended neck, bulging fontanelle, seizures
Potential complications= chronic bilirubin encephalopathy, extrapyramidal signs, visual problems, hearing problems, cognitive defects, dental enamel
Management of kernicterus?
Manage neurological complications, phototherapy and exchange transfusions
What is the most common GI emergency in neonates?
Necrotising enterocolitis
What can be found on examination of a baby with necrotising enterocolitis?
Distension, visible intestinal loops, altered stools, bloody mucoid stool and bilious vomiting, decreased bowel sounds, erythema, associated systemic features
Management of ?necrotising enterocolitis?
Confirm diagnosis by AXR and get baseline biochemistry
Nil by mouth, bowel decompression, Iv fluids and TPN (total parenteral nutrition), IV abx for 10-14 days (gentamicin and metronidazole), surgery if perforated/necrotic bowel suspected
NB/ stages i= suspected, iia=mild, iib=moderate, iiia=advanced, iiib=deteriorating
What is gastroschisis?
Congenital defect of the abdominal wall where the abdominal contents herniate into the amniotic sack (usually the small intestine, but sometimes stomach, colon and ovaries). There is no covering membrane.
The opening is less than 5cm in length
Management of gastroschisis?
Scheduled preterm delivery may improve the post op outcome.
Primary closure of the defect
Simple v complex gastroschisis?
Simple= intact, uncompromised and continuous bowel Complex= bowel perforation, atresia or necrosis
Difference between gastroschisis and exomphalos?
Exomphalos has bowel contents in a sac, is more variable in size, and has a more central location
Gastroschisis has no covering membrane, is under 5 cm in length and tends to be to the right of umbilical cord insertion
What is oesophageal atresia?
Congenital abnormality with a blind ending oesophagus in isolation or with at least one fistula(e) communicating between normal oesophagus and trachea (tracheo-oesophageal fistula0
Associated anomalies with oesophageal atresia?
VACTERL syndrome (vertebral, anorectal, cardiovascular, tracheo-oesophageal, oseophageal atreisa, renal abnormalities, limb) CHARGE association (coloboma, heart, atresia, retarded development, genital hypoplasia, ear) Chromosomal abnormalities eg trisomies
Postnatal presentation of oesophageal atresia?
Respiratory distress, choking, feeding difficulties, frothing in first few hours after birth
Management of oesophageal atresia?
Surgery
Presentation of bowel atresia?
Persistent vomiting-often bilious and within hours of birth
on Xray= double bubble sign of duodenal atresia, colonic atresia has normal air levels
Most common metabolic problem in neonates?
Gestational diabetes and hypoglycaemia
(glucose passes through placenta and elevates glucose levels in foetus, causing increased insulin secretion, this leads to hypoglycaemia)
Plasma glucose under 30 mg/dL
Pathophysiology of neonatal hyperthyroidism?
Usually caused by maternal graves disease- antibodies cross the placenta and cause the foetus to develop hyperthyroidism
Usually temporary, but can be life threatening.
Untreated congenital hyperthyroidism?
Risk of craniosynostosis (early skull closing), intellectual disability, growth failure, hyperactivity in later childhood
Management of congenital hyperthyroidism?
Antithyroids and beta blockers- stopped when antibodies have disappeared (usually fully resolved by 6 months)
NB/ if mum took thyroid drugs during pregnancy, the child may not show symptoms until 3-7 days
How many women carry group b strep in genitals?
1 in 4
Presentation of neonate with GBS infection?
Sepsis, pneumonia, meningitis signs, BP changes, seizures
Management of GBS infection?
IV antibiotics- penicillin G
What can listeria do to a neonate?
Low birth weight, prematurity, circulatory and/or respiratory insufficiency, meningitis, sepsis
Management of neonatal listeria infection?
Ampicillin and aminoglycoside eg gentamicin
Cause of encephalitis in neonates?
HSV2 from vertical transmission
Presentation of HSV encephalitis?
Non specific: decreased levels of consciousness, seizures, lethargy, fever
Management of HSV encephalitis? Prognosis?
IV aciclovir Highly lethal (around 50%) Complications= deaf, vision loss, cerebral palsy, seizure
Management of cleft life/palate?
Surgery
Cleft lip at 3-6 months
Palate at 6-12 months
What is ophthalmia neonatorum?
Conjunctivitis in newborn due to vertical transmission (contact with mum’s birth canal infected by STI)
Commonly chlamydia
Also: haemophilus, strep, staph, pseudomonas, HSV, adenovirus, enterovirus
Chance of ophthalmia neonatorum in babies born to untreated chlamydia?
30-50%
Presentation of ophthalmia neonatorum?
Red, discharging, swollen lids, usually bilateral
Lid oedema, conjunctival oedema, mucopurulent conjunctivitis, cornea can be involved
Management of ophtalmia neonatorum?
Culture conjunctiva and PCR to establish causative organism
Bacterial= systemic penicillin G or cephalosporin for gonorrhoea Chlamydia= systemic erythromycin or topical azithromycin Herpetic= topical and systemic aciclovir
Premature baby still needing breathing support by 36 weeks corrected age = what?
Chronic lung disease/ bronchopulmonary dysplasia
Caused by underdeveloped lungs
Management of bronchopulmonary dysplasia?
Oxygen (can further damage lungs), breathing support (CPAP or mechanical ventilation), may need steroids
Pathophysiology of retinopathy of premature baby?
Abnormal blood vessel growth are fragile and weak, causing bleeding and leakage
Usually bilateral
Ranges from abnormal blood vessel growth to full retinal detachment (stage i-v)
Can cause blindness
Management of retinopathy of premature baby?
Laser therapy or cryotherapy: destroys peripheral retina and slows/reverses abnormal vessel growth, but loses some side vision
Why are premature babies at risk of osteopenia?
May not receive the full amount of calcium and phosphorus needed to form strong bones
Haven’t been as active in the womb and thus have weak bones
Very premature babies lose more phosphorus in urine than term babies
Can be due to low vit D
How to treat osteopenia of premature baby?
Supplements- calcium and phosphorus added to IV fluids or breast milk
Special formulas if no breast milk
Vit D supplements for babies with liver problems
What is periventricular leukomalacia?
White matter surrounding ventricles deprived of blood and thus oxygen. This causes softening of the white matter
Consequences of periventricular leukomalacia?
Most commonly cerebral palsy
May have visual problems and learning disability
Not progressive
Presentation depends on amount of brain tissue damaged
What is apnoea in a prem baby?
Prem baby pauses breathing for over 15-20 seconds
or
pauses breathing for under 15 seconds but has bradycardia or low sats
Usually self limiting
What is transient tachypnoea of the newborn?
Caused by leftover fluid in lungs making it harder for the alveoli to stay open
RR over 60
Most improve over 24-48 hours, and most cases self limit
Who is more likely to have transient tachypnoea of the newborn?
Born before 38 weeks, c-section delivery, born to diabetic or asthmatic mother, twins, male
Forms of child abuse
Physical, neglect, emotional, sexual
What may come under child neglect?
Medical eg unimmunised, non attendance
Nutritional eg faltering growth, obesity
Emotional
Educational eg poor school attendance
Physical eg inadequate hygiene, persistent infestation, inappropriate clothing
Failure to supervise eg frequent A+E trips, injury suggesting lack of care such as sun burn
Normal presentation/history of toddler’s fracture?
Subtle undisplaced spiral fracture of tibia
Usually pre school, sudden twist after an unwitnessed fall
Local tenderness over tibial shaft or on general strain on tibia
What counts as mild dehydration and features?
Less than 5%
Thirst, dry lips, restlessness, irritability
What counts as moderate dehydration and features?
5-10%
Sunken eyes, decrease skin turgor, decreased urine output
What counts as severe dehydration and features?
Over 10%
Cold, mottled, hypotensive, anuria, decreased consciousness
What fluids needed for not dehydrated/dehydrated/shocked?
Not dehydrated=maintenance
Dehydrated=maintenance+deficit
Shocked=bolus+maintenance+deficit
What maintenance fluids do neonates need?
10% glucose with increasing rate every day
Monitor U+Es every day
From day 2 if needed= sodium 2-3mmol/kg/day and potassium 1-2mmol/kg/day
How to estimate child’s weight?
(age + 4) x 2
What fluids for maintenance in children? How much?
0.9% NaCl (normal saline)
First 10kg=100ml/kg
Next 10kg=50ml/kg
Every other kg=20ml/kg
Calculates how much in 24 hours
How to calculate the deficit fluids in children?
Deficit x 10 x wt(kg)
Where estimated deficit=
mild= negligible, mod=5%, severe=10%
What bolus do you use and which volume?
0.9% NaCl, volume=20ml/kg
May use 10ml/kg in some cases eg trauma, DKA
Tends to be 20ml/kg if shocked in DKA, 10ml/kg if not shocked
What constitutes an atypical UTI in children and what do you need to do if this is the case?
Atypical= septicaemia/IV abx needed, non e.coli, poor urine flow, abdo/bladder mass, increase creatinine, failure to respond to treatment within 48 hours Recurrent= at least 2 episodes with at least 1 with systemic features, or at least 3 episodes without systemic features
Need to investigate for a structural abnormality
How to investigate ?structural abnormality in the case of UTI?
- USS renal tract (looks at size and drainage of kidneys and bladder)
- Micturating cystourethrogram/MCUG (looks for vesicoureteric reflux, looks at bladder and posterior urethral valve)
- DMSA scan/radionucleide imaging (looks at renal function and any renal scarring)
Features of nephritic syndrome?
Haematuria, proteinuria, impaired GFR, salt and water retention (htn and oedema)
Management of nephritic syndrome?
Fluid balance management (input/output, fluid restriction, diuretics, salt restriction)
Correct other imbalances (K, acidosis)
Dialysis may be needed (uncommon)
Penicillin for treatment of an ongoing strep infection
Why is sinusitis uncommon in children?
Maxillary sinus exists at birth but only grows to full size after 2nd dentition
Ethmomid sinuses only 2-3 cells at birth
No/rudimental frontal sinuses at birth (develop by 7-8 years)
Narrowest point in child’s airway vs adult’s?
Children=subglottis
Adults=vocal cords
What is the most common airway abnormality in children and it’s presentation?
Laryngomalacia
Normal voice, stridor worse on feeding/exertion/supine, failure to thrive in 10%, increased work of breathing
When do you expect primitive reflexes to disappear?
By 4-6 months, lots of babies lose them within 2 months
What options for formula for children with cow’s milk protein allergy?
Hydrolysed eg althera
Amino acid based eg alfamino
Goat milk formula from birth
Soy formula from 6 months
What constitutes faltering growth?
Failure to gain adequate weight or height/length during infancy or early childhood
Birth weight <9th centile with a fall across at least 1 wt centiles
Birth weight between 9-91st centile with a fall across at least 2 wt centiles
Birth weight over the 91st centile with a fall across at least 3 wt centiles
Current weight below 2nd centile whatever the birth weight
When would you expect a baby to get back to his birth weight?
3 weeks
Causes of osteoporosis in children?
Inherited/congenital eg OI, inborn errors of metabolism, haem problems, idiopathic
Acquired eg drug induced eg steroids, endocrinopathies, malabsorption
What is osteogenesis imperfecta?
AD inherited condition, v rare
Mostly due to defects in type i collaegen
Characteristed by bone fragility, fractures, deformity, bone pain, poor growth, impaired mobility
How can you manage osteogenesis imperfecta?
MDT management esp ortho team and metabolic bone specialist
Bisphophonates
Describe the types of osteogenesis imperfecta?
type 1- most common, mild
2- lethal
3- progressively deforming, severe
4- moderate
What may indicate a shaken baby?
Hypoxia, subdural haematoma, rib fractures, retinal haemorrhages, may have other fractures, torn frenulum
How should a premature baby be fed?
IV fluids/parenteral nutrition as suckle and swallow only starts from 32-34 weeks
Start small volumes of expressed breast milk and build up to full feeds slowly (risk of NEC if too quick)
Causes of acute scrotum in children?
Testicular torsion!!!
Also torsion of appendix testis (self limiting, but if any diagnostic doubt treat as testicular torsion)
Vomits and presentation age in pyloric stenosis, malrotation and intussusception
Malrotation: bilious green vomit at 24 hours
Pyloric stenosis: milky vomit at 4 weeks
Intussusception: milky then green vomit at 6 months
