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Phase 3a🩺 > Paeds 2 🦠🩸♋️ > Flashcards

Paeds 2 🦠🩸♋️ Flashcards

Infectious disease, Skin and allergy, Haematology, Oncology

1
Q

what is Kawasaki disease?

A

a type of vasculitis - predominately seen in children

leads to inflammation of the coronary arteries and medium/large vessels

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2
Q

What is the cause of Kawasaki disease?

A

largely unknown
infectious cause
autoimmune reaction
genetic predisposition

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3
Q

What are the features of Kawasaki disease?

A

CRASH and burn
C - conjunctivitis with limbus sparing
R - rash - all body parts polymorphous - then flakes (desquamation)
A - adenopathy - enlarged lymph nodes (cervical)
S -strawberry tongue + red and cracked lips
H - hands and feet swollen with rash.

high grade fever for more than 5 days which is characteristically resistant to antipyretic.

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4
Q

How is Kawasaki managed?

A

IVIG intravenous immunoglobulin
high dose aspirin (one of the few indications for the use of aspirin in children, due to the risk of Reyes syndrome aspirin is usually contraindicated in children)
Echo - to screen for coronary artery aneurysms.

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5
Q

What are the complications of Kawasaki? (3)

A

coronary artery aneurysm
ischaemia of the heart muscle
MI

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6
Q

What would you see from blood tests of Kawasaki? (4)

A 
anaemia 
increased WBC
more immature WBC
increased ESR and CRP 
increased liver enzymes
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7
Q

What is the incubation period of measles?

A

10-14 days

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8
Q

What symptoms do you get during the prodromal period of measles and how long does it last?

What are the symptoms following the prodromal period?

A

it lasts for usually 3 days
cough
conjunctivitis
coryza

Koplik spots - spots on mucus membrane

following the prodromal period a rash occurs: starts on the face and behinds ears and then transfers to the whole body.
A desecrate maculopapular rash becoming blotchy and confluent.

in the immunocompromised - a rash is often not present

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9
Q

what are the investigations of measles?

A

IgM antibodies can be detected within a few days of rash onset

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10
Q

what is the management of measles?

A

mainly supportive
admission may be considered in immunosuppressed or pregnant patients
notifiable disease –> inform public health

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11
Q

What are the complications of measles (5)

A 
pneumonia 
diarrhoea 
encephalitis (typically occurs 1-2 weeks following the onset of the illness 
otitis media (MC)
febrile convulsions 
corneal ulceration 
myocarditis 
increased incidence of appendicitis

subacute sclerosing pan-encephalitis (very rare, may occur 5-10 years following infection of infant under 2)

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12
Q

How is it managed if an unvaccinated child comes into contact with measles ?

A

if child is not vaccinated and comes into contact with measles then the MMR vaccine should be given within 72 hours.

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13
Q

what virus is chicken pox caused by?

A

varicella zoster

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14
Q

How is chicken pox transmitted? When is someone with chickenpox infective?

A

respiratory route
or skin to skin contact

infectivity = 24 hours before rash until 5 days after the rash first appeared (when blisters have crusted over)

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15
Q

what is the incubation period of chicken pox?

A

10-21 days

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16
Q

What are the clinical features of chicken pox?

A

fever initially
itchy rash starting on head trunk before spreading
initially macular then papular then vesicular then scab over
systemic upset is usually mild

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17
Q

what is the management of chicken pox?

A

keep cool, trim nails
calamine lotion
school exclusion (infective until lesions are dry and crusted over - usually around 5 days after onset of the rash

in immunocompromised patients and new born consider varicella zoster immunoglobulin, if chicken pox develops then acyclovir should be considered

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18
Q

What are the complications of chicken pox? (3)

A

common complication is secondary bacterial infection of the lesions - NSAIDs may increase this risk

rare complications include pneumonia, encephalitis, disseminated haemorrhage chickenpox, arthritis, nephritis and pancreatitis

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19
Q

what is rubella caused by?

A

toga virus - spread by respiratory route

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20
Q

What are the clinical features of rubella?

A

prodrome - low grade fever
rash - maculopapular, initially on the face/behind ears before spreading to the whole body usually fades by the 3-5 day
lymphadenopathy: suboccipital and post auricular

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21
Q

What are the complications of rubella? (3)

A

arthritis
thrombocytopenia
encephalitis
myocarditis

can cause severe damage to the foetus (congenital rubella syndrome)

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22
Q

what is diphtheria caused by?

A

gram positive bacterium Corynebacterium diptheriae

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23
Q

What does diphtheria infection cause? Why is it rare in the UK?

A

local disease with membrane formation affecting the nose, pharynx or larynx or systemic disease with myocarditis and neurological manifestations

immunisations have eradicated disease in the UK

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24
Q

What is Staphylococcal scalded skin syndrome?

A

Staphylococcal scalded skin syndrome (SSSS) is a rare, severe, superficial blistering skin disorder which is characterised by the detachment of the outermost skin layer (epidermis). This is triggered by exotoxin release from specific strains of Staphylococcus aureus bacteria.
The blistering of large areas of skin gives the appearance of a burn or scalding, hence the name staphylococcal scalded skin syndrome. SSSS is often used interchangeably with the eponymous name Ritter von Ritterschein disease (Ritter disease), particularly when it presents in newborn children.

nikolsky sign - The sign is present when slight rubbing of the skin results in exfoliation of the outermost layer.

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25
Q

how is scalded skin syndrome managed?

A

IV anti-staphylococcal antibiotic, analgesia and monitoring fluid balance

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26
Q

what is whooping cough caused by?

A

gram negative bacterium bordetella pertussis

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27
Q

what are the clinical features of whooping cough?

A

2-3 days of coryza precede the onset of:
coughing bouts - usually worse at night and after feeding, may be ended by vomiting and associated central cyanosis
insipiratory whoop - not always present - caused by force inspiration against a closed glottis
infants may have spells of apnoea
persistent coughing can cause subjconjunctival haemorrhages or even anoxia leading to syncope and seizure
symptoms may last 10-14 weeks and is more severe in infants
there is marked lymphocytosis

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28
Q

what is the diagnostic criteria for whooping cough?

A

if a person has an acute cough for more than 14 days or more without another apparent cause and has one or more of the following features
paroxysmal cough
inspiratory whoop
post-tussive vomiting
undiagnosed apnoeic attacks in young infants

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29
Q

how is whooping cough diagnosed?

A

nasal swab culture to look for bordetella pertussis

PCR and serology are now increasingly used

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30
Q

how is whooping cough managed?

A

under 6 months if it suspected then admit them
it is notifiable disease
an oral macrolide - e.g. clarithromycin or azithromycin

clarithromycin for infants less than 1 month
azithromycin or clarithromycin for children aged 1 month or older and non-pregnant

household contacts should be offered prophylaxis

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31
Q

What are the complications of whooping cough? (3)

A

subconjunctival haemorrhage
pneumonia
bronchiectasis
seizures

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32
Q

what are the stages of whooping cough?

A

catarrhal phase is the first stage (runny nose stage - typically lasts one to two week

paroxysmal stage - duration is highly variable lasting from one to ten weeks, intense drawn out bouts of coughing during this phase. Attacks tend to be more frequent at night

convalescent phase - the third stage - this can last for week to months and a chronic cough that becomes less paroxysmal (fewer sudden outbursts of coughing)

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33
Q

what is tuberculosis caused by?

A

mycobacterium tuberculosis

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34
Q

What is primary TB? Explain the pathophysiology behind primary TB.

A

a non-immune host is exposed to tuberculosis
A small lung lesion known as a Ghon focus develops 3 weeks following initial infection
The Ghon focus is composed of tubercle-laden macrophages.
The combination of a Ghon focus and a hilar lymph node is known as a Ghon complex.

In immunocompetent people this initially heals by fibrosis and calcification. Those who are immunocompromised may develop disseminated disease (miliary tuberculosis)

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35
Q

what is secondary TB?

A

if the host becomes immunocompromised the initial infection may become reactivated
Reactivation usually occurs in the apex of the lungs and may spread locally or to more distant sites

causes of immunocompromise - HIV, immunosupressants, malnutrition

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36
Q

Where might extra pulmonary infection of TB occur? (3)

A 
CNS - tuberculosis meningitis - the most serious complication 
vertebral bodies (potts disease)
cervical lymph nodes - scrofuloderma
renal - WBC in urines
addisons disease
GI tract
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37
Q

How do you test for TB?

A

Mantoux tuberculin skin test

this test just shows that they have been exposed to TB not whether it is latent or active

interferon gamma release assay

positive results - do xray

Acid-fast smear and mycobacterial culture to differentiate active from latent tb)

labs of sputum

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38
Q

how do you treat latent TB?

A

isoniazid for 9 months

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39
Q

How do you treat active TB? Give a side effect of each medication.

A 
RIPE 
Rifampicin
isoniazid
pyrazinamide
ethambutol
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40
Q

what are the common causes of meningitis in neonates up to 3 months?

A

group B streptococcus (usually acquired from the mother at birth)
E.coli and other gram negative organisms
listeria monocytogenes

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41
Q

What are the common causes of meningitis in infants? (3)

A 
Neisseria meningitidis (meningococcus)
Streptococcus pneumoniae (pneumococcus)
Haemophilus influenzae
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42
Q

what are the common causes of meningitis in children older than 6?

A 
Neisseria Meningitidis (meningococcus)
Streptococcus pneumoniae (pneumococcus)
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43
Q

What are some viral causes of meningitis? (3)

A

enterovirus
EBV
adenovirus
mumps

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44
Q

What are the early signs of meningitis? (5)

A 
headache
temp
cold hands and feet 
discolored skin (blotchy)
poor feeding 
irritability 
hypotonia
drowsiness
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45
Q

What are late signs of meningitis? (5)

A 
neck stiffness
kernigs sign 
Brudzinskis sign
photophobia 
bulging fontanelle
arched back 
seizure 
shock: increased HR, RR, cap refill and BP decrease

non blanching purpuric rash

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46
Q

How is meningitis diagnosed? (4)

A 
Septic screen 
blood - coagulation screen, LFT, PCR, rapid antigen test, blood gas 
CT head
Culture - throat swab, urine and stool
TB - mantoux test, CXR
LP
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47
Q

What are the contraindications to lumbar puncture? (3)

A 
focal neurological signs 
Papillodema 
significant bulging of the fontanelle 
DIC
signs of cerebral herniation
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48
Q

How is meningitis managed in children?

A

antibiotics
if less than 3 months - IV amoxacillin and cefotaxime
if older than 3 months - IV ceftriaxone/cefotaxime

Dexamethasone in children 3 months or older with suspected or confined bacterial meningitis and LP indicates (should be given before or with abx)

Fluids

Cerebral monitoring

Public health notification
prophylaxis of contacts with ciprofloxacin

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49
Q

What would the characteristics of the CSF be if it was bacterial meningitis?

A

cloudy/turbid
Elevated opening pressure
low glucose
high protein
white cells 10-500 polymorphs

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50
Q

What would the CSF show if there is viral meningitis?

A

clear CSF
Normal/elevated opening pressure
60-80% of plasma glucose
protein is normal/raised
white cells 15-1000 lymphocytes

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51
Q

What is erythema infectiosum and what is it caused by?

A

slapped cheek syndrome

Parvovirus B19

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52
Q

What are the clinical features of slapped cheek syndrome?

A

mild fever
rose-red rash makes the cheeks appear bright red
the rash may spread to the rest of the body but will spare the palms and soles.
rash peaks after a week and then fades

other presentations:
- asymptomatic
- pancytopaenia in immunocompromised patients
aplastic crisis e.g sickle cell disease

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53
Q

what is the treatment for slapped cheek syndrome?

A

most children recover and need no specific treatment

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54
Q

What is impetigo?

A

a superficial bacterial skin infection

it may be a primary infection or a complication of an existing skin condition such as eczema, scabies or insect bites.

It is common in children in warm weather

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55
Q

what is impetigo caused by?

A

staphylcoccus aureus or streptococcus pyogenes

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56
Q

what are the common locations that impetigo occur?

A

anywhere on body but tend to occur on face, flexures and limbs not covered by clothing

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57
Q

What are the features of impetigo?

A

golden, crusted skin lesions typically found around the mouth

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58
Q

What are the three types of impetigo?

A

Non-bullous/ cruted impetigo
tiny blisters that eventually burst and leave small wet patches of red skin that may weep fluid, gradually they crust over

Bullous impetigo causes larger fluid containing blisters that look clear, then cloudy. they are more likely to stay longer without bursting

Ecthyma - punched out ulcers with yellow crust and red edges

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59
Q

What is the management for limited, localised impetigo?

A

first line - hydrogen peroxide 1% cream

second line - topical fusidic acid or mupirocin (MRSA)

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60
Q

what is the management for extensive impetigo?

A

oral flucloxacillin
or if allergic to penacillin - erythromycin

children should be excluded from school until lesions are crusted and healed or 48 hours after commencing antibiotic treatment.

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61
Q

what is scarlet fever caused by?

A

group A haemolytic streptococci

usually streptococcus pyogenes

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62
Q

what is the peak incidence of scarlet fever?

A

2-6 years

peak incidence at 4 years

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63
Q

What is the classical presentation of scarlet fever?

A

fever that typically lasts 24 to 48 hours
malaise, headache, nausea/vomiting
sore throat
strawberry tongue
rash - fine punctate erythema (pinhead) which generally appears first on the torso and spares the palms and soles
the rash is often described as having a rough sandpaper texture
desquamation occurs later in the course of the illness

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64
Q

how is scarlet fever diagnosed?

A

throat swab

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65
Q

How is scarlet fever treated?

A

oral penicillin V for 10 days

patients with penicillin allergy should be given azithromycin

scarlet fever is a notifiable disease
children can return to school 24 hours after starting antibiotics

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66
Q

What are the complications of scarlet fever? (3)

A

otitis media
rheumatic fever (usually 20 days after infection )
acute glomerularnephritis (typically 10 days after infections)
invasive complications rare but are dangerous (bacteraemia , meningitis, necrotising fasciitis)

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67
Q

What is mumps caused by?

A

RNA paramyxovirus

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68
Q

How is mumps spread and what is the infective period?

A

spread by droplets
it is infective for 1-2 days before the onset of symptoms and 9 days after parotid swelling
it has an incubation period of 14-18 days

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69
Q

What are the clinical features of mumps?

A

fever
malaise
muscular pain
parotitis (earache, pain on eating) unilateral initially then becomes bilateral in 70%

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70
Q

how is mumps managed?

A

rest
paracetamol for high fever and discomfort
it is a notifiable disease

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71
Q

What are the complications of mumps? (3)

A

orchitis - uncommon in pre-pubertal males but occurs in 25-35% of post-pubertal males - typically occurs 4-5 days after parotitis
hearing loss - usually unilateral and transient
meningoencephalitis
pancreatitis

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72
Q

what causes toxic shock syndrome?

A

toxin-producing staphylococcus aureus (TSST-1 superantigen toxin) and group A streptococci

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73
Q

What is toxic shock syndrome characterised by? (5)

A

fever (>38.9)
hypotension (sys <90)
diffuse erythematous, macular rash

can also cause organ dysfunction including:
mucositis
GI - vomiting/diarrhoea
renal impairment
liver impairment
clotting abnormalities and thrombocytopenia
CNS - altered consciousness

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74
Q

How is toxic shock syndrome treated?

A

areas of infection should be surgically debrided
antibiotics - clindamycin + benzylpenicillin/vancomycin
IVIG to neutralise circulating toxin

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75
Q

What causes encephalitis ?

A

Inflammation if the brain parenchyma (MC HSV)

delayed brain swelling following a disordered neuroimmunological response to an antigen, usually a virus (post-infectious encephalopathy e.g. following chicken pox)
a slow virus infection such as HIV infection or subacute sclerosing pan encephalitis following measles

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76
Q

How do you treat encephalitis caused by HSV?

A

high dose acyclovir

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77
Q

what are the routes HIV can be passed to children?

A

mother-to-child transmission - during pregnancy, and delivery or through breast feeding.

children may also be infected by infected blood products, contaminated needle or through sexual abuse.

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78
Q

how is HIV diagnosed in children?

A

in children over 18 month, HIV is diagnosed by detecting antibodies to the virus.

prior to 18 months - they will have the mothers antibodies which suggests exposure but not deffo infection
before 18months diagnosis is by HIV DNA PCR

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79
Q

what are the clinical features of HIV in infants?

A

a proportion of HIV infected infants progress rapidly to symptomatic disease and onset of AIDs in the first year of life
some children remain asymptomatic for months or years

clinical presentation depends on degree of immunosuppression

mild immunosupresion: lymphadenopathy or parotitis

moderate - recurrent bacterial infection

severe - pneumocystis jiroveci pneumonia (PCP), severe FTT, encephalopathy and malignancy, although this is rare in children

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80
Q

How is HIV in children treated? What drug is given prophylactically and why?

A

antiretroviral therapy (ART)

prophylaxis against PCP with co-trimoxazole

immunisation

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81
Q

what is hand foot and mouth disease caused by?

A

most commonly coxsackie A16 and enterovirus 71

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82
Q

What are the clinical features of hand foot and mouth disease?

A

mild systemic upset - fever, sore throat
oral ulcers
followed later by vesicles on the palms and soles of feet

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83
Q

how is hand foot and mouth disease managed?

A

symptomatic treatment only

do not need to be excluded from school

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84
Q

what is eczema?

A

inflammatory skin condition characterized by dry, pruritic skin with chronic relapsing course.

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85
Q

What are the risk factors for eczema? (4)

A 
age <5 years 
fam history of eczema 
allergic rhinitis
asthma 
active or passive exposure to smoke
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86
Q

What are the clinical features of eczema? How may infants and children present?

A

pruritus
xerosis (dry skin)

infants usually show involvement of the cheeks, forehead, scalp and extensor surfaces.
Children typically have involvement of flexures, particularly the wrists, ankles, antecubital and popliteal fossa.

Erythema is often noted in the acute flares
scaling in acute flares
papules

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87
Q

how is eczema treated?

A

avoid irritants
1st line - emollients
if not controlled with emollients intermittent topical corticosteroids can be added

if infection suspected add oral antibiotic

if persistent pruritus - antihistamine or doxepin

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88
Q

what is stevens-johnsons syndrome?

A

Stevens-Johnson syndrome (SJS) is a severe skin detachment with mucocutaneous complications. It is an immune reaction to foreign antigens. SJS is a more severe form of erythema multiforme major and a less severe manifestation of toxic epidermal necrolysis (TEN)

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89
Q

what can cause stevens-johnsons syndrome?

A

Infection (URTI, OM, pharyngitis, mycoplasma pneumoniae, herpes, EBV, CMV

vaccination can precipitate

Medicines - many -e.g. anticonvulsants, antibiotics

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90
Q

What is the difference between stevens-johnsons syndrome and toxic epidermal necrolysis

A

SJS - <10% of total body surface involvement - any of the causes

TEN - >30% of total body surface involvement - drug related

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91
Q

what are the symptoms of stevens-johnsons syndrome?

A

sudden onset of rash
erosions or ulcerations of the eyes, lips, mouth, pharynx, oesophagus, GI tract, kidneys, liver, anus, genital area or urethra.
eye involvement may include conjunctivitis, corneal ulceration and uveitis.

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92
Q

What investigations should you order for stevens-johnson syndrome? (5)

A 
skin biopsy - will show keratinocyte apoptosis with detachment of the epidermal layer of the skin from the dermal layer 
blood cultures - will be negative 
FBC
glucose 
magnesium 
phosphate 
urea 
LFTs
ABG
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93
Q

How is stevens-johnsons syndrome managed? (4)

A

withdraw causative agent
prophylactic anticoagulation (enoxaparin)
PPI (omeprazole)
Dressing, topical antibacterials and emollients
ophthalmological examination
IV fluids
pain relief

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94
Q

what is allergic rhinitis?

A

inflammatory disorder of the nose where the nose becomes sensitized to allergens such as house dust mites and grass, tree and weed pollens

95
Q

what are the different classifications of allergic rhinitis ?

A
  • seasonal: symptoms occur around the same time every year. Seasonal rhinitis which occurs secondary to pollens is known as hay fever
  • perennial: symptoms occur throughout the year
  • occupational: symptoms follow exposure to particular allergens within the work place
96
Q

What are the clinical features of allergic rhinitis? (4)

A 
sneezing 
nasal pruritus 
eye redness 
nasal congestions 
rhinorrhoea
97
Q

how is allergic rhinitis managed?

A

oral antihistamine plus allergen avoidance

intranasal antihistamine plus allergen avoidance

2nd line - montelukast

if severe an intranasal corticosteroid can be added

98
Q

what is urticaria?

A

it is also known as hives
a skin condition characterised by erythematous, blanching, oedematous, non painful, pruritic lesions that typically resolves within 24 hours and leave no residual symptoms

chronic - episodes lasting for more than 6 weeks.

99
Q

what is angioedema?

A

swelling involving the deeper layers of the sub-dermis and occurs in association with urticaria. It can also occur in the absence of urticaria

100
Q

what causes urticaria and/or angio-oedema?

A

usually caused by IgE-mediated reaction (T1 hypersensitivity reaction). most common agents involved are drugs (NSAIDs, penicillins, muscle relaxants, diuretics) and foods (milk, eggs, peanuts, tree nuts, shellfish)

101
Q

What are the symptoms of urticaria and angioedema? (3)

A 
erythematous oedematous lesions 
pruritus 
resolved within 24 hours 
swelling of face, tongue or lips
blanching lesions
102
Q

What investigations would you consider for urticaria? (5)

A 
FBC
complete metabolic panal 
urinalysis 
ESR
CRP
anti-IgE receptor antibodies 
TSH 
skin biopsy
103
Q

what is the definition of anaemia in neonates, in children from 1 to 12 months and children 1 year to 12 years?

A

neonates - Hb less than 140g/L
1 month to 12 months - HB less than 100g/L
1 year to 12 years - Hb less tha 110g/L

104
Q

what can anaemia result from?

A

reduced red cell production, increased red cell destruction (haemolysis)
blood loss

105
Q

what are the clinical features of iron deficiency anaemia in children?

A

usually asymptomatic until the HB drops below 60-70g/L
as it gets lower: children tire easily, young infants feed more slowly
they may appear pale

106
Q

how is iron deficiency anemia diagnosed?

A

a low haemoglobin and haematocrit level
MCV - low
MCH - the mean Hb mass per cell - is low
low MCV and low MCH = hypochromic anemia
there will be a decrease serum iron
the total iron-binding capacity will be increased
transferrin saturation will be less than 16%
there will be a low serum ferritin

107
Q

what can cause iron deficiency anemia?

A

inadequate dietary intake
poor intestinal absorption - coeliac disease
increased iron requirement - children have increase iron demands during rapid periods of growth

108
Q

How is iron deficiency anaemia managed? How long is treatment give for?

A

dietary advice and oral iron supplement

ferrous sulfate

give until Hb back to normal + 3 months

109
Q

what is red cell aplasia?

A

complete absence of red cell production

110
Q

what can cause red cell aplasia?

A
  • congenital
  • transient erythroblastopenia of childhood
  • parvovirus B19 infection - only causes red cell aplasia in children with inherited haemolytic anaemia
111
Q

What are the diagnostic cues of red cell aplasia? (3)

A

low reticulocyte count despite low Hb
normal bilirubin
negative direct antiglobulin test (coombs test)
absent red cell precursors on bone marrow examination

112
Q

What is diamond-blackfan anaemia and how is it treated?

A

rare congenital type of bone marrow failure that causes anaemia and is characterised by pure red cell aplastic and associated with congenital bone abnormalities

treated with oral steroids and monthly red blood cell transfusions are given to children if they are steroid unresponsive

113
Q

What is transient erythroblastopenia of childhood?

A

it is triggered by viral infections and has the same haematological features as diamond-blackfan anemia

usually recovers in weeks

114
Q

What are some common causes of haemolytic anaemia in children?

A
  • red cell membrane disorders (hereditary spherocytosis)
  • red cell enzyme disorders (G6PD deficiency)
  • haemoglobinopathies (abnormal haemoglobulins e.g. beta thalassaemia and sickle cell disease)
115
Q

What does haemolysis lead to? (3)

A

anemia
hepatomegaly and splenomegaly
increased blood levels of unconjugated bilirubin
excess urinary urobilinogen

116
Q

What are some diagnostic cues to haemolysis? (3)

A

raised reticulocyte count on blood film
unconjugated bilirubinemia and raised urinary urobilinogen
abnormal appearance of red cells on blood film
if there is an immune cause the there would be a positive direct antiglobulin
increased red blood cell precursors in bone morrow?
LDH

117
Q

what is hereditary spherocytosis?

A

it is an inherited abnormality of the RBC and causes defects in the structural membrane proteins
It is inherited dominantly in 75% of people

The abnormal cells are spherical and are removed by the spleen, resulting in reduced red-cell life span

118
Q

what is the presentation of hereditary spherocytosis?

A

pallor
FTT
jaundice (due to increased levels of unconjugated bilirubin
splenomegaly

*aplastic crisis may be precipitated by parvovirus infection.

119
Q

What are the investigations for hereditary spherocytosis? (3)

A

FBC - Hb may be normal or reduced, MCHC (average conc of Hb in blood cell) may be elevated

Reticulocyte count - will be elevated

blood smear - will show spherocytes and may also show pincer cells (due to protein band-3 deficiency)

there will be elevated unconjugated bilirubin

120
Q

how is hereditary spherocytosis managed?

A

less than 28 days - supportive care +/- a RBC transfusion, sometimes folic acid may be added

If the neonate has jaundice use phototherapy or exchange transfusion

In children older than 28 days with severe HS :
supportive care and RBC transfusion for symptomatic anaemia until a time when a splenectomy is deemed appropriate (wait until at least 6 years of age)
also give folic acid supplementation

Mild/moderate HS - generally just supportive care
folic acid can be added in patients with severe haemolysis

121
Q

Why does glucose 6 phosphate dehydrogenase deficiency cause anaemia?

A

G6PD is essential for preventing oxidative damage to red blood cells.
Red cells lacking G6PD are susceptible to oxidant induced haemolysis.

So when there is oxidative stress haemolysis patients are usually asymptomatic until oxidative stress

122
Q

What is the inheritance pattern of G6PD deficiency? What populations is G6PD seen in?

A

x linked recessive

  • it is common among populations originating from parts of the world where malaria is or was common
    (africa, asia, mediterranean and the Middle East)
123
Q

What are the things than can cause oxidative stress and should be avoided in patients with G6PD deficiency? (4)

A

metabolic acidosis
Henna
foods and drinks (fava beans, red wine, soy products)
painkillers - aspirin and ibuprofen
infections - viral hepatitis, pneumonia
certain medication - quinidine, primaquine, chloroquine, ciprofloxacin

124
Q

What is the presentation of G6PD deficiency? (3)

A 
neonatal jaundice is often seen 
intravascular haemolysis 
gall stones
splenomegaly may be present 
pallor 
dark urine
125
Q

What investigations would you perform for G6PD deficiency and what would they show? (3)

A

FBC - anaemia and normochromic red cell index
reticulocyte count - typically elevated
Urinalysis - haemoglobinuria
unconjugated bilirubin will be elevated
lactate dehydrogenase will be high
blood smear will show anisocytosis, abnormal forms and bite cells
blood film will show heinz bodies

126
Q

how is G6PD deficiency treated?

A

in acute haemolysis:
supportive care plus folic acid
If there is severe anaemia - blood transfusion

In neonates with prolonged jaundice - phototherapy

127
Q

what is the inheritance pattern of sickle cell anaemia?

A

autosomal recessive

128
Q

what is sickle cell anaemia and what causes it?

A

it is caused by a single gene defect in the beta chain of haemoglobin which results in production of sickle haemoglobin (HbS)

polymerisation of sickle Hb in RBCs can be triggered by hypoxia and acidosis which causes cells to become rigid and deform into a sickle shape. These deformed cells may cause vaso-occlusion in the small vessels or adhere to vascular endothelium - this slows blood flow. These deformed cells are also prone to haemolysis, which contributes to anaemia.

129
Q

What are the clinical features of sickle cell anaemia? (5)

A

anaemia - all have moderate - - anaemia with clinically detectable jaundice from chronic haemolysis

  • increased susceptibility to infection from encapsulated organisms such as pneumococci and haemophilus influenzae. Also increased risk of osteomyelitis caused by salmonella and other organisms
  • Painful Crisis - vaso-occlusive crisis - pain may affect many organs of the body with varying frequency and severity - pain can affect bones in limbs and spine
  • Dactylitis - swollen hands and feet
  • acute anaemia (sudden drop in Hb), haemolytic crisis, aplastic crisis (parvo virus b19 infection), splenic sequestration crisis (sudden splenic enlargement - abdo pain and circulatory collapse from accumulation of sickled cells in the spleen)
  • jaundice
  • pallor
  • tachycardia
  • lethargy
  • failure to thrive
  • Priapism (prolonged erection)
  • splenomegaly
  • acute chest syndrome
130
Q

What are the long term problems of sickle cell anaemia? (3)

A 
short and delayed puberty 
stoke and cognitive problems
cardiac enlargement 
renal dysfunction 
gall-stones 
leg ulcers
131
Q

What investigations would you perform for sickle cell? (4)

A 
heel prick test 
Hb electrophoresis 
blood smear - presence of nucleated red blood cells, sickle-shaped cells and howell jolly bodies 
increase reticulocytes 
FBC will show anaemia
132
Q

How do you manage sickle cell anaemia? (5)

A

avoid precipitants of crises (cold weather, hypoxia, stress, dehydration)
Pneumococcal immunisation, antibiotic prophylaxis with phenoxymetylpenicillin
pain management

Bld transfusions

For Crisis
- pain relief, oxygen, hydration

Prevention of recurrent crisis:
hydroxycarbamide (increases fetal Hb production and protects against further crisis)

Crizanlizumab (targets P-selectin. P-selectin is an adhesion molecule found on endothelial cells on the inside walls of blood vessels and platelets. It prevents red blood cells from sticking to the blood vessel wall and reduces the frequency of vaso-occlusive crises.)

Bone marrow transplant if severe

133
Q

What are some causes of normocytic anemia? (3)

A 
anaemia of chronic disease 
chronic kidney disease
aplastic anaemia 
haemolytic anaemia 
acute blood loss
134
Q

what is beta-thalassaemia?

A

it is an inherited microcytic anaemia caused by mutations of the beta-globin gene leading to decreased or absent synthesis of beta-globin, resulting in ineffective erythropoiesis
it is an autosomal recessive condition

135
Q

What is the presentation of beta thalassaemia? (4)

A

Will not present until 3-6 months of age when fetal Hb is replaced by adult Hb

jaundice 
severe anaemia 
failure to thrive 
hepatosplenomegaly 
delayed puberty 
facial - maxillary overgrowth, skull bossing

if they have beta-thalassaemia trait - asymptomatic

136
Q

What investigations would you perform for beta thalassaemia? (5)

A 
FBC
Blood smear
reticulocyte count - raised 
Abdo uss
Hb analysis 
LFTs
Skull x-ray (widening of diploeic space)
- microcytic anemia

there would be no HbA but increased HbA2

137
Q

how would you treat beta-thalassaemia?

A

Transfusions
iron monitoring and chelation (desferrioxamine) - this is because people having regular transfusions can have iron overload

they may need a splenectomy
they may also need a stem cell transplant if severe

138
Q

What is fanconi syndrome and what are the symptoms? (4)

A

generalised proximal tubular dysfunction

polydipsia and polyuria
salt depletion and dehydration 
hyperchloraemic metabolic acidosis 
rickets 
FTT/poor growth
139
Q

what kind of anemia is fanconi anaemia?

how is it inherited?

A

aplastic

AR

140
Q

What are the features of fanconi anaemia? (5) what are these patients at higher risk of?

A

short stature
abnormal radii and thumbs
renal malformation
pigmented skin lesions (cafe au lait spots)
bone marrow failure which becomes apparent at 5-6 years.
Neonates with Fanconi anaemia nearly always have abnormal blood count.
they have an increased risk of acute myeloid leukaemia

141
Q

what is the treatment for fanconi anaemia?

A

bone marrow transplant

142
Q

What are the main causes of haemolytic anaemia in the new born? (4)

A

immune (haemolytic disease of the newborn)
red cell structural disorders (hereditary spherocytosis)
red cell enzyme disorders (G6PD deficiency )
Haemoglobinopathies

143
Q

what is haemolytic disease of the new born?

A

it is immune haemolytic anaemia
it is due to antibodies against blood group antigens

The mother makes antibodies against the baby’s blood group and these antibodies cross the placenta into the baby’s circulation causing fetal or neonatal anaemia

144
Q

how should rhesus D negative women be treated?

A

anti D immunoglobulin at 28 weeks and following the delivery of a rhesus positive infant or at 40 weeks

145
Q

What is the basic pathophysiology of rhesus negative mothers and why does it only affect second pregnancy?

A

along with the ABO system the Rhesus system is the most important antigen found on red blood cells. The D antigen is the most important antigen of the rhesus system
around 15% of mothers are rhesus negative (Rh -ve)
if a Rh -ve mother delivers a Rh +ve child a leak of fetal red blood cells may occur
this causes anti-D IgG antibodies to form in mother
in later pregnancies these can cross placenta and cause haemolysis in fetus
this can also occur in the first pregnancy due to leaks

146
Q

what tests should be performed for all babies born to rhesus negative mother?

A

all babies born to Rh -ve mother should have cord blood taken at delivery for FBC, blood group & direct Coombs test
Coombs test: direct antiglobulin, will demonstrate antibodies on RBCs of baby
Kleihauer test: add acid to maternal blood, fetal cells are resistant

147
Q

What can the affected fetus from a rhesus negative mother present with? (3)

A 
oedematous (hydrops fetalis, as liver devoted to RBC production albumin falls)
jaundice, anaemia, hepatosplenomegaly
heart failure
kernicterus (bilirubin-induced neurological damage)
treatment: transfusions, UV phototherapy
148
Q

What is haemophilia? What are the different types and the inheritance pattern?

A

it is a bleeding disorder
usually inherited - X-linked recessive disorder of coagulation

Haemophilia A is due to a deficiency of clotting factor VIII 
Haemophilia B (christmas disease) due to a lack of clotting factor IX

there is also acquired haemophilia - may have autoimmune-related aetiology with no inheritance pattern

149
Q

What are the clinical features of haemophilia? (4)

A

recurrent or severe bleeding - spontaneous or trauma-induced bleeding in joints and muscles; excessive bleeding after surgery, dental procedures or trauma, recurrent nasal/oral mucosa bleeding; easy bruising, GI bleeding, haematuria

bleeding into muscles - MSK bleeding is the hallmark of haemophilia
hemarthrosis - bleeding in joint cavity
haematomas

150
Q

What investigations would you perform for haemophilia? (4)

A

FBC
APTT - activated partial thromboplastin time will usually prolonged
plasma factor VIII and IX assay - levels of factor VIII and IX are used to establish diagnosis and severity
prothrombin time (PT) normal
bleeding time - normal
LFTs

151
Q

what are common causes of bruising in neonates?

A

coagulation disorders (hemorrhagic disease of the new born, haemophilia)
Thrombocytopenia (maternal alloimmune thrombocytopenia)
birth trauma and congenital infections e.g. rubella

152
Q

what are common causes of bruising in infants?

A

accidental injury
non-accidental injury
coagulation disorders
thrombocytopenia (ITP, congenital infection)

153
Q

what are common causes of bruising in older children?

A

accidental injury
non-accidental injury
coagulation disorders (haemophilia, VWD, liver disease)
thrombocytopenia - ITP, acute lymphoblastic leukaemia, meningococcal septicaemia, congenital infection

154
Q

What is von willebrand’s disease? What is its inheritance?

A

most common inherited bleeding disorder
there is variable penetrance and phenotypic expression

it characteristically behaves like a platelet disorder

it is due to either quantitative or qualitative abnormality of von willebrand’s factor

Autosomal dominant (apart from type 3)

155
Q

What is the role of vWF?

A

it promotes platelet adhesion to damaged endothelium and platelet aggregation

156
Q

what are the different types of von willebrand’s disease?

A

type 1: partial reduction in vWF
type 2: abnormal form of vWF
type 3: total lack of vWF (AR)

157
Q

What are the symptoms of von willebrand’s disease? (4)

A 
bleeding from minor wounds 
post operative bleeding 
Epistaxis
Prolonged bleeding from mucosal surfaces (dental)
fam history of bleeding 
easy or excessive bruising 
menorrhagia
158
Q

What investigations would you perform for von willebrand’s disease?

A

prothrombin time will be normal
activated partial thromboplastin time (APTT) may be prolonged but not always
von willebrand factor antigen
von Willebrand factor function assay (ristocetin cofactor and collagen binding assays)
Factor VIII activity value may be decreased (in the absence of vwf, factor 8 has a decreased half life)

159
Q

How is VWD managed?

A

Antifibrinolytic therapy - tranexamic acid

desmopressin (induces release of Factor 8 and vWF from endothelial cells)

2nd line vWB containing concentrate

*if acute severe bleeding give vWB factor concentrate and platelet transfusion as first line

160
Q

what is thrombocytopenia?

A

low platelets

161
Q

how is haemophilia A and B managed?

A

if there is bleeding
A - recombinant factor VIII concentrate
B - recombinant factor IX concentrate

162
Q

What is ITP? What is the compensatory finding in this condition?

A

immune thrombocytopenia is the commonest cause of thrombocytopenia in childhood.
It is usually caused by the destruction of circulating platelets by antiplatelet IgG autoantibodies.
the reduced platelet count may be accompanied by a compensatory increase of megakaryocytes in the bone marrow

163
Q

what are the clinical features of ITP?

A

most children present between the ages of 2 and 10 often with the onset 1-2 weeks after a viral infection

petechiae, purpura and/or superficial bruising
it can causes epistaxis and other mucosal bleeding

absent splenomegaly or hepatomegaly

164
Q

how is ITP diagnosed?

A

ITP is a diagnosis of exclusion
low platelet count less on blood smear

*in younger children, a congenital cause (such as Wiskott-Aldrich or Bernard-Soulier syndromes) should be considered.

bone marrow examination should be performed if the child is going to be treated with steroids, since the treatment may temporarily mask the diagnosis of acute lymphoblastic leukaemia (ALL)

165
Q

How is ITP managed?

A

usually the disease is acute, benign and self-limiting
most children can be managed at home and do not require hospital admission.

most children do not need therapy
the treatment options include oral prednisolone or IVIG

platelet transfusions are reserved for life-threatening haemorrhage.

166
Q

what is acute lymphoblastic leukaemia?

A

it is the most common malignancy in childhood.

It arises from malignant proliferation of pre-B (common ALL) or T-cell lymphoid precursors.

167
Q

what age group is ALL common in?

A

the peak incidence is at around 2-5 years of age and boys are more commonly affected than girls

168
Q

How does acute lymphoblastic leukemia present? (5)

A

typically a short history (days or weeks) and with signs and symptoms reflecting pancytopenia, bone marrow expansion and lymphadenopathy

purpura, easy bruising, epistaxis due to thrombocytopenia

hepatosplenomegaly
pallor, ecchymoses or petechiae
fever
fatigue, dizziness, palpitations and dyspnoea
epistaxis, menorrhagia
bone pain - secondary to bone marrow infiltration

169
Q

What investigations would you perform for ALL and what would you see? (4)

A
  • bone marrow: hyper cellularity and infiltration by lymphoblast’s
  • FBC - anaemia, leucocytosis, neutropenia and/or thrombocytopenia
  • peripheral blood smear - leukaemic lymphoblast’s
  • serum electrolytes
  • chest XR may show evidence of a mediastinal mass, pleural effusion or LRTI
  • RFT - urea may be normal or elevated
  • LFTs - may be normal or elevated
170
Q

What are the factors that lead to poor prognosis in ALL? (3)

A 
age < 2 years or > 10 years
WBC > 50* 109/l at diagnosis
CNS disease present
non-Caucasian
male sex
171
Q

what is acute myeloid leukaemia?

A

it is more common in adults than children but accounts for 5% of all childhood malignancies
it results from malignant proliferation of myeloid cell precursors in the bone marrow, peripheral blood or extramedullary tissues.

172
Q

What are the features of acute myeloid leukaemia? (4)

A 
anaemia - pallor, lethargy, weakness 
neutropenia - WCC may be very high however functioning neutrophil levels may be low leading to frequent infection 
thrombocytopenia - bleeding 
splenomegaly 
bone pain

*lymphadenopathy and intrathoracic extramedullary disease is less prominent than in ALL

173
Q

what is the classification used for AML?

A 
MO - undifferentiated
M1 - without maturation
M2 - with granulocytic maturation
M3 - acute promyelocytic
M4 - granulocytic and monocytic maturation
M5 - monocytic
M6 - erythroleukaemia
M7 - megakaryoblastic
174
Q

What conditions predispose to leukaemia? (4)

A

down’s syndrome increase risk of AML and ALL

Fanconi syndrome, bloom syndrome, ataxia telangiectasia, Kostmann’s syndrome, diamond blackfan syndrome, kleinfelters, turners, neurofibromatosis, incontinentia pigmenti all increase the risk of AML.

175
Q

what is Hodgkin’s lymphoma?

A

Hodgkin’s lymphoma (HL), also referred to as Hodgkin’s disease, is an uncommon haematological malignancy arising from mature B cells. It is characterised by the presence of Hodgkin’s cells and Reed-Sternberg cells.

176
Q

What is Hodgkin’s lymphoma characterised by? (3)

A

the presence of Reed-Sternberg cells
it is characterised by lymphadenopathy - painless, non tender, asymmetrical
Systemic - B symptoms - weight loss greater than 10%, night sweats, fever (Pel-Ebstein)

177
Q

What are the investigations for AML? (3)

A

FBC - anaemia, microcytosis, leucocytosis, neutropenia and thrombocytopenia

peripheral blood smear - blasts on blood film, presence of Auer rods

coagulation panel
serum electrolytes  
renal function 
LFTs
bone marrow biopsy - will show bone marrow hypercellularity and infiltration by blasts, blasts >20%, Auer rods
178
Q

what are the risk factors for Hodgkin’s lymphoma?

A

history of EBV infection

fam history of Hodgkin’s lymphoma

179
Q

what are the different types of Hodgkin’s lymphoma ?

A

Nodular sclerosing Hodgkin lymphoma (NSHL) - most common in children and adolescents
Mixed-cellularity Hodgkin lymphoma (MCHL)
Lymphocyte-depleted Hodgkin lymphoma (LDHL)
Lymphocyte-rich classical Hodgkin lymphoma (LRHL)

180
Q

what are the common sites for hodgkins lymphoma?

A

most common site is cervical and mediastinal

dissemination to extra-nodal sites is uncommon

181
Q

What is the staging for hodgkins lymphoma?

A

Ann Arbor system
I -Single lymph node region
II- Two or more regions on the same side of the diaphragm
III- Involvement of lymph node regions on both sides of the diaphragm
IV - Involvement of extra nodal sites

A- no B symptoms
B- B symptoms

182
Q

What investigations would you perform for hodgkins lymphoma? (3)

A 
FBC 
LN biopsy
metabolic panel 
ESR 
CT neck chest and abdomen/pelvis
PET scan 
CXR
Bone marrow biopsy - if radiological evidence of at least stage 3)  - the presence of Hodgkin's cells

EBV serology

183
Q

what is a lymphoma?

A

Lymphoma is the malignant proliferation of lymphocytes which accumulate in lymph nodes or other organs. Lymphoma may be classified as either Hodgkin’s lymphoma (a specific type of lymphoma characterized by the presence of Reed-Sternberg cells) or non-Hodgkin’s lymphoma (every other type of lymphoma that is not Hodgkin’s lymphoma).

184
Q

What are the clinical features of non-Hodgkin’s lymphoma? (5)

A 
peripheral lymphadenopathy 
splenomegaly 
night sweats 
weight loss 
fatigue 
malaise 
fever 
extra nodal disease - gastric (dyspepsia, dysphagia, weight loss, abdominal pain), bone marrow (pancytopenia, bone pain), lungs, skin, central nervous system (nerve palsies)
185
Q

how can you differentiate hodgkins from non-Hodgkin’s?

A

Lymphadenopathy in Hodgkin’s lymphoma can experience alcohol-induced pain in the node
‘B’ symptoms typically occur earlier in Hodgkin’s lymphoma and later in non-Hodgkin’s lymphoma
Extra-nodal disease is much more common in non-Hodgkin’s lymphoma than in Hodgkin’s lymphoma

186
Q

What investigations would you perform for non-Hodgkin’s lymphoma? (4)

A

excisional node biopsy
CT chest abdomen and pelvis - to assess staging
HIV test
FBC - thrombocytopenia, pancytopenia, lymphocytosis
blood smear - nucleated red blood cells, left shift
LDH - an indirect indication of the proliferative rate of the lymphoma - an important diagnostic and prognostic factor

187
Q

What are the risk factors for non-Hodgkin’s lymphoma? (3)

A 
males 
>50
immunocompromised 
EBV infection 
human T-lymphocytotrophic virus 1 (HYLV-1)
human herpesvirus-8
H.pylori 
coeliac disease
HIV
Hep C
188
Q

what is the staging used for non-hodgkins lymphoma

A

same as HL - ann arbor system

189
Q

how is non-Hodgkin’s lymphoma managed?

A

Management is dependent on the specific sub-type of non-Hodgkin’s lymphoma and will typically take the form of watchful waiting, chemotherapy or radiotherapy.
All patients will receive flu/pneumococcal vaccines
Patients with neutropenia may require antibiotic prophylaxis

190
Q

What are the complications of Hodgkin’s lymphoma? (3)

A

Bone marrow infiltration causing anaemia, neutropenia or thrombocytopenia
Superior vena cava obstruction
Metastasis
Spinal cord compression
Complications related to treatment e.g. Side effects of chemotherapy

191
Q

What are the different types of ALL?

A

T cell
B cell

192
Q

What are the complications of ALL? (3)

A

neutropenic sepsis - give tazocin
hyperuricaemia - give allopurinol
secondary cancer
stunted growth

193
Q

what are the different types of brain tumours?

A

Astrocytoma - the most common - varies from benign to highly malignant
Medulloblastoma - arises in the midline of the posterior fossa
ependymoma
brainstem glioma
craniopharyngioma

194
Q

what are the classifications of brain tumours?

A

supratentorial - cortex - astrocytoma

Infratentorial - cerebellar - medulloblastoma, astrocytoma, ependymoma. - brainstem, - brainstem glioma

spinal cord - astrocytoma, ependymoma

Midline - craniopharyngioma

195
Q

what are the clinical features of a supratentorial tumour?

A

seizures
hemiplegia
focal neurological signs

196
Q

what are the symptoms of a midline tumour?

A

visual field loss - bitemporal hemianopia

pituitary failure - growth failure, diabetes insipidus, weight gain

197
Q

what are the symptoms of cerebellar and IVth ventricle tumour?

A

truncal ataxia
coordination difficulties
abnormal eye movements

198
Q

What are the symptoms of a brainstem tumour?

A

cranial nerve defects
pyramidal tract signs (hyperreflexia, weakness, spasticity, and a Babinski sign)
cerebellar signs - ataxia

*prognosis is poor
diffuse brainstem gliomas are inoperable

199
Q

what are the most common sites for a low grade glioma?

A

cerebellum and optic pathway

200
Q

What is the most common malignant brain tumour in children?

A

primitive neuroectodermal tumours (PNET) - majority occur in the cerebellum (similar histologically to medulloblastoma however worse prognosis) they usually arise in the midline and invade the fourth ventricle and cerebral hemispheres

201
Q

How do children with an ependymoma present?

A

they usually present with obstructive hydrocephalus

Ependymomas develop from ependymal cell. These cells line the fluid filled areas of the brain (ventricles) and the spinal cord and produce CSF.

202
Q

how do brainstem gliomas present?

A

cranial nerve palsies, ataxia and pyramidal tract signs

203
Q

how do primitive neuroectodermal tumours present?

A

headache, vomiting and ataxia

204
Q

what does a craniopharyngioma present with?

A

visual field loss due to compression of the optic chiasm

pituitary dysfunction - growth failure and diabetes insipidus

205
Q

what is a craniopharyngioma?

A

slow growing midline epithelial tumours arising from the remnant Rathke’s pouch

206
Q

what are the symptoms of a retinoblastoma?

A

absent or abnormal light reflex, squint or visual deterioration

207
Q

what is a Wilm’s tumour?

A

it is a nephroblastoma - the most common form of renal malignancy in childhood.
it arises from embryonic renal tissue

208
Q

what age group does a Wilm’s tumour present in?

A

2-5 years

209
Q

What are the clinical features of a Wilms’ tumour? (3)

A

abdominal mass (most common presenting feature)
painless haematuria
flank pain
other features: anorexia, fever
unilateral in 95% of cases
metastases are found in 20% of patients (most commonly lung)

210
Q

What are Wilms’ tumours associated with? (3)

A

genitourinary abnormalities e.g. horseshoe kidney, hypospadias
hemihypertrophy syndrome

beckwith-wiedemann syndrome

around one-third of cases are associated with a loss-of-function mutation in the WT1 gene on chromosome 11

associated with aniridia (absent iris)

WAGR (wilms tumour, anridia, genitourinary abs, range of developmental delays)

211
Q

What investigations would you perform for a wilms’ tumour? (5)

A

FBC
Renal function - decreased clearance creatinine/raised serum creatinine
LFT
urinalysis
abdominal US
CT scan of abdomen - claw sign in involved kidney
biopsy
Genetic testing

212
Q

how do you treat a wilm’s tumour?

A

chemotherapy
nephrectomy
radiotherapy if advanced disease

213
Q

what is a neuroblastoma?

A

Neuroblastoma is one of the top five causes of cancer in children, accounting for around 7-8% of childhood malignancies. The tumour arises from neural crest tissue of the adrenal medulla (the most common site) and sympathetic nervous system.

214
Q

what are the common sites for neuroblastoma involvement?

A

they can occur anywhere in the sympathetic nervous system

commonly occur in the adrenals
the sympathetic chain - abdomen, thorax, pelvis, neck

215
Q

What are the clinical features of a neuroblastoma? (5)

A

abdominal mass - a firm, non tender abdominal mass is the most common mode of presentation
abdominal distension
abdominal pain
bone pain is common in patients with metastatic disease
systemic signs: pallor, weight loss, bone pain from disseminated disease
hepatomegaly or lymph nodes enlargement
compression of nerves - Horner’s syndrome
decreased appetite
panda eyes - periorbital ecchymosis - in patients with orbital metastases

216
Q

What investigations would you perform for a neuroblastoma? (5)

A

urine catecholamine - catecholamine degradation products homovanillic acid (HVA) and vanilylmandelic acid (VMA) are secreted by the majority of tumours

FBC, serum electrolytes, creatinine/urea, LFTs

USS abdomen - heterogenous mass with internal vascularity; may show calcifications or areas of necrosis

CT/MRI

MIBG scan (identifies sites of mets)

217
Q

how do you treat a neuroblastoma?

A

surgical resection
chemo
radio

218
Q

What are poor prognostic factors in neuroblastoma? (3)

A 
age >18 months 
stage 3 and 4 disease 
raised serum ferritin 
raised LDH 
raised neurone- specific enolase (NSE)
unfavourable histology 
MYCN oncogene amplification
219
Q

What is a retinoblastoma? What is the inheritance pattern?

A

Most common intraocular malignancy in children.
In 30% to 40% of cases, the disease is associated with a germline mutation in the RB1 gene, which carries an associated increased risk of secondary non-ocular tumours.

Autosomal dominant

220
Q

How does a retinoblastoma present?

A

Absent red reflex, replaced by leukocoria - white pupillary reflex (most common)

Visual problems

Strabismus

221
Q

How do you diagnose and treat a retinoblastoma?

A

fundoscopy and examination under anaesthesia
wide-field fundus photography and spectral domain optical coherence tomography ophthalmic A and B scan USS

treat with surgery, laser eblation, cryotherapy, enucleation, chemo

222
Q

what are the two most common forms of bone tumours in childhood?

A

osteosarcoma - older children, most common

Ewing’s sarcoma: younger children, less common

223
Q

what is an osteosarcoma?

A

malignant tumour of the bone-producing mesenchymal cells

224
Q

How does an osteosarcoma present? (4)

A

10-20 years of age
Persistent bone pain (worse at night)
Bone swelling
Palpable mass
Restricted joint movement

225
Q

What is the most common site for an osteosarcoma?

A

The femur
Other common sites involve the tibia and humerus

226
Q

How would you diagnose an osteosarcoma according to nice guidelines? What would you see?

A

~~~
Very urgent direct access x ray within 48 hrs, if suggestive of sarcoma then very urgent specialist assessment within 48hrs

Poorly defined lesion, fluffy appareance, periosteal reaction (irritation to lining of the bone),“sun-burst” appearance

Bone biopsy
LDH and alkaline phosphatase - will usually be raised
MRI
CT thorax
isotope bone scan

227
Q

How do you treat osteosarcomas? What are the main complications?

A

Surgical resection (often with limb amputation)
Adjuvant chemotherapy

Metastasis and pathological fractures

228
Q

How does an Ewing’s sarcoma present?

A

usually occurs in bone but can also occur in soft tissue
localised intermittent pain (worse at night) and swelling, sometimes a pathological fracture.
Commonly affects the diaphysis of long bones
the most common site is the femoral diaphysis
metastasis to lungs and bone common

229
Q

How do you diagnose and Ewing’s tumour? (4)

A 
plain x-ray of bony lesions 
biopsy 
LDH and alkaline phospatatse 
MRI 
CT chest 
isotope bone scans 
bone marrow aspirates and trephines
230
Q

what is a rhabdomyosarcoma?

A

it is the most common soft tissue sarcoma in childhood - commonly in aged <10 years
most are either embryonic or alveolar subtypes

231
Q

what are the clinical features of rhabdomyosarcoma?

A

mass, pain and obstruction of:

  • bladder
  • pelvis
  • nasopharynx
  • parameningeal
  • orbit
  • intrathoracic

lymph involvement in common, distant metastases are rare

232
Q

What investigations would you perform for rhabdomyosarcoma? (3)

A 
CT or MRI of primary site 
biopsy 
CT if chest 
bone marrow aspirates 
isotope bone scan 
LP
233
Q

what is the most common primary paediatric hepatic tumour? and what is seen in this type of tumour?

A

hepatoblastoma
usually seen in the first year of life
serum AFP levels are raised by >80%

Phase 3a🩺 (12 decks)

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