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Paediatrics ILA 1-3 Flashcards

1
Q

how is measles spread?

A

droplet spread

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2
Q

what are the ‘4 C’s’ symptoms in the prodrome phase of measles?

A
  1. cough
  2. coryza- catarrhal inflammation of nasal membranes
  3. conjunctivitis
  4. cranky
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3
Q

what is the incubation period for measles?

A

7-12 days

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4
Q

what are 4 signs of measles?

A
  1. koplik spots on the palate

2. raised temperature

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5
Q

what are 4 complications of a measles infection?

A
  1. otitis media- most common
  2. croup
  3. tracheitis
  4. pneumonia
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6
Q

what is a chronic complication of measles that develops 7-13 years after the primary infection?

A

subacute sclerosing parencephalitis

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7
Q

what are 5 symptoms of subacute sclerosing parencephalitis?

A
  1. changes in behaviour
  2. myoclonus
  3. dystonia
  4. dementia
  5. choreoatheotosis- involuntary twitching or writhing
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8
Q

how do you treat measles?

A
  1. supportive care
  2. ensure isolation
  3. ribovarin if immunocompromised
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9
Q

how is mumps spread?

A

droplet spread/ salivary spread

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10
Q

what is the incubation period of mumps?

A

14-21 days

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11
Q

when is the infective period of mumps?

A

7 days before and 9 days after swelling of the parotid glands

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12
Q

what are 3 signs and symptoms of mumps?

A
  1. prodromal malaise
  2. raised temperature
  3. painful parotid swelling becoming bilateral in 70%
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13
Q

what is a complication from mumps that can reduce sperm count?

A

orchitis

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14
Q

how do you treat mumps?

A
  1. rest
  2. analgesia
  3. fluids
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15
Q

how is rubella spread?

A

droplet spread

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16
Q

what is the incubation period of rubella?

A

2-3 weeks

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17
Q

what is the infective period of rubella?

A

5 days before and 5 days after the onset of the rash

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18
Q

what are 3 signs of rubella?

A
  1. macular rash that first occurs on the face and fades in 3-5 days
  2. suboccipital lymphadenopathy
  3. mild, low grade fever
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19
Q

what are 2 complications of rubella?

A
  1. small joint arthritis

2. malformations in utero

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20
Q

what are the three most common in utero malformations associated with rubella and when would the infection have to occur to produce each malformation?

A
  1. first 4 weeks- eye anomaly
  2. 4-8 weeks- cardiac abnormalities
  3. 8-12 weeks- deafness
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21
Q

how do you diagnose measles?

A

clinical examination and serological test

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22
Q

how do you diagnose mumps?

A

clinical examination and buccal swab and culture

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23
Q

how do you diagnose rubella?

A

clinical examination and serological testing

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24
Q

what are 4 signs of erythrovirus (AKA parvovirus)?

A
  1. malar erythema/ slapped cheek appearance
  2. macular rash mainly on the limbs
  3. mild constitutional upset
  4. fever
  5. malaise
  6. headache
  7. myalgia
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25
Q

how is erythrovirus spread?

A

droplet spread

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26
Q

what is a dangerous haematological complication of erythrovirus and who is at risk?

A

it can cause aplastic crisis in people with chronic haemolytic anaemia and conditions like sickle cell disease, thalassaemia, spherocytosis

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27
Q

how do you diagnose erythrovirus and which antibody is involved?

A

serology, igM

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28
Q

how do you treat erythrovirus?

A
  1. supportive care
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29
Q

what is the % risk of fetal death with erythrovirus and what is the cause?

A

10%, mostly hydrops fetalis because of inhibited multiplication of erythroid progenitor cells

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30
Q

what 5 fetal problems can occur due to erythrovirus?

A
  1. hydrops fetalis
  2. growth restriction
  3. placentomegaly
  4. oedema
  5. pancytopenia
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31
Q

what viruses can cause hand, foot and mouth disease?

A

coxsackie virus and enterovirus

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32
Q

what are some signs and symptoms of hand, foot and mouth disease?

A
  1. low grade fever
  2. rash/vesicles on the hands and feet
  3. oral ulcers and vesicles
  4. malaise
  5. sore mouth
  6. loss of appetite
  7. sore throat
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33
Q

what is the incubation time for hand, foot and mouth disease?

A

5-7 days

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34
Q

how do you treat hand, foot and mouth disease?

A
  1. symptomatic treatment
  2. analgesics and antipyretics
  3. fluid and nutritional support
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35
Q

how do you diagnose hand, foot and mouth disease?

A

clinical examination and serological testing

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36
Q

what is the cause of roseola infatum?

A

herpes virus 6

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37
Q

what are some signs and symptoms of roseola infantum?

A
  1. increased temperature
  2. maculopapular rash following 3-5 days of fever, commonly beginning on the neck and trunk
  3. uvulopalatoglossal ulcers
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38
Q

what is a dangerous complication of roseola infantum?

A

febrile seizures, can cause encephalitis

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39
Q

what are 4 less common causes of rashes in children?

A
  1. a few maculopapular rashes- meningococcaemia
  2. purpuric rashes- ITP, henon-schonlein purpura
  3. drug maculopapular rashes- amoxicillin
  4. scabies
  5. eczema
  6. still’s disease
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40
Q

how is chickenpox spread?

A

droplets, can be caught from someone with shingles

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41
Q

what is the incubation period for chickenpox?

A

11-21 days

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42
Q

when is the infective period of chickenpox?

A

4 days before the rash until all lesions have scabbed (roughly 5 days)

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43
Q

what are 4 signs of chickenpox?

A
  1. crops of skin vesicles of different ages starting on the face scalp or trunk and spreading
  2. pruritis
  3. malaise
  4. temperature
  5. headache
  6. sore throat
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44
Q

where are chickenpox vesicles usually more concentrated?

A

the torso

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45
Q

how do you diagnose chickenpox?

A
  1. clinical examination
  2. pcr
  3. tzanck smear
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46
Q

what are 3 differentials for chickenpox?

A
  1. hand, foot and mouth disease
  2. insect bites
  3. scabies
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47
Q

what is the course of chickenpox?

A

rash starts 2 days after onset of fever, lesions cluster around areas of pressure or hyperaemia

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48
Q

what are 4 complications of chickenpox?

A
  1. secondary bacterial staph infection
  2. encephalitis
  3. pupura fulminans
  4. pneumonia
  5. meningitis
  6. myelitis
  7. thrombosis
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49
Q

how do you identify purpura fulminans?

A

blackish clusters of lesions

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50
Q

how do you identify necrotising fasciitis?

A

coalescing, bluish clusters of lesions

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51
Q

in which conditions is chickenpox dangerous?

A
  1. immunosuppression
  2. cystic fibrosis
  3. severe eczema
  4. neonates
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52
Q

how do you treat chickenpox

A
  1. keep the patient cool
  2. calamine lotion soothes
  3. chlorhexidine daily antiseptic
  4. flucloxacillin if bacterial superinfection
  5. if immunosupressed or on steroids- anti varicella zoster immunoglobulin and aciclovir
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53
Q

what virus causes shingles?

A

varicella zoster virus

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54
Q

how do you treat shingles?

A

oral analgesia and aciclovir

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55
Q

what are the risks of contracting varicella during pregnancy?

A

fetal varicella syndrome in 2% in the last 20 weeks- can cause cerebral cortical atrophy and cerebellar hypoplasia

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56
Q

how do you prevent contracting varicella during pregnancy?

A

vaccination

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57
Q

how do you treat babies when they are born after the mother had varicella during pregnancy?

A

zoster immunoglobulin, if infected give then acyclovir and isolate

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58
Q

what is an infection that can be vertical transmitted from mother to baby?

A

HIV

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59
Q

how do you diagnose vertically acquired HIV infection?

A
  1. HIV viral pcr
  2. p24 antigen
  3. specific IgA
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60
Q

what are 4 signs symptoms in a child that would make you consider a HIV infection?

A
  1. lymphadenopathy
  2. hepatosplenomegaly
  3. persistent diarrhoea
  4. recurrent infections
  5. recurrent fever
  6. thrombocytopenia
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61
Q

how do you treat vertically transmitted HIV?

A
  1. HAART- highly active anti retroviral therapy

2. teach children about safe sex and other issues before puberty

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62
Q

what are 4 causative organisms of infective encephalitis?

A
  1. herpes simplex virus
  2. varicella zoster virus
  3. rabies virus
  4. parvovirus
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63
Q

how do you diagnose encephalitis?

A
  1. clinical examination
  2. CSF PCR
  3. test stool, urine and blood
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64
Q

what are 4 non-infective differentials of encephalitis?

A
  1. hypoglycaemia
  2. diabetic ketoacidosis
  3. hepatic failure
  4. subarachnoid haemorrhage
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65
Q

what are 5 signs and symptoms of infective encephalitis?

A
  1. flu-like prodrome
  2. decreased consciousness
  3. odd behaviour
  4. vomiting
  5. fits
  6. increased temperature
  7. meningism
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66
Q

how do you treat infective encephalitis?

A
  1. give acyclovir if HSV suspected
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67
Q

what bacteria cause toxic shock syndrome?

A
  1. staph aureus

2. group A strep

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68
Q

what are 5 signs and symptoms of toxic shock syndrome?

A
  1. fever >39
  2. hypotension
  3. diffuse erythematous rash
  4. impaired consciousness
  5. gi/renal/liver impairment
  6. clotting abnormalities
  7. PVL released by s aureus can cause necrotising fasciitis
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69
Q

how do you treat toxic shock syndrome?

A
  1. intensive care
  2. surgical debridement of infected areas
  3. IV ceftriaxone/ clindamycin with IV Ig
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70
Q

what % of patients with bacterial meningitis are <16 years old?

A

80%

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71
Q

what % of survivors of bacterial meningitis have neurological impairment?

A

10%

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72
Q

what are 7 signs and symptoms suspicious of bacterial meningitis in infants?

A
  1. irritability
  2. abnormal cry
  3. lethargy
  4. difficulty feeding
  5. fever
  6. seizures
  7. apnoea
  8. bulging fontanelles
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73
Q

what are 5 septic signs of bacterial meningitis?

A
  1. increased temperature
  2. cold hands/ feet
  3. arthralgia and limb pain
  4. abnormal skin colour
  5. odd behaviour
  6. rash
  7. DIC
  8. raised pulse
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74
Q

what are 3 meningeal signs of bacterial meningitis?

A
  1. neck stiffness
  2. kernig’s sign- resistance to knee extension with hip flexion
  3. brudzinski’s sign- hips flexed on bending head forward
  4. photophobia
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75
Q

when is lumbar puncture contraindicated in bacterial meningitis?

A

if there is cardioresp instability, focal neurological signs or raised icp

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76
Q

how are the white cells, protein and glucose affected in the CSF of someone with bacterial meningitis?

A
  1. neutrophils
  2. increased protein
  3. decreased glucose
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77
Q

how are the white cells, protein and glucose affected in the CSF of someone with viral meningitis?

A
  1. lymphocytes
  2. normal protein
  3. normal glucose
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78
Q

what are 4 cerebral complications of bacterial meningitis?

A
  1. hearing impairment
  2. local vasculitis
  3. subdural effusion
  4. hydrocephalus
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79
Q

how do you diagnose meningitis?

A
  1. lumbar puncture
  2. clinical presentation- kernig’s and brudzinski’s
  3. lumbar puncture
  4. bloods- fbc, coagulation, calcium, magnesium, phosphate, crp
  5. blood culture
  6. ct/mri
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80
Q

how do you treat bacterial meningitis?

A
  1. immediate IV broad-spectrum antibiotics- EG cephalosporin and vancomycin
  2. ampicillin if l.monocytogenes is suspected (older, younger, immunocompromised)
  3. adjuvant dexamethasone (not recommended in neonates)
  4. supportive therapy (EG ABCDE, 2 large bore cannulas)
  5. rifampicin or ciprofloxacin for pphx of contacts
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81
Q

name 4 different bacteria that can cause meningitis?

A
  1. neisseria meningitiis
  2. haemophilus influenzae
  3. strep pneumoniae
  4. escherichia coli
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82
Q

what is a noticeable dermatological sign of septicaemia?

A

purpuric rash

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83
Q

what are 6 infections that can be caused by streptococcus pneumoniae?

A
  1. pharyngitis
  2. otitis media
  3. conjunctivitis
  4. sinusitis with pneumonia
  5. sepsis
  6. meningitis
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84
Q

what is the clinical presentation of impetigo?

A

honey-coloured crusted lesions that appear on the face, neck or hands initially and can spread, very infectious

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85
Q

what bacteria can cause impetigo?

A

staph and strep

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86
Q

what skin condition makes you more likely to develop impetigo?

A

atopic eczema

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87
Q

how do you treat impetigo?

A
  1. topical antibiotic cream
  2. flucloxacillin in severe infections
  3. children should not attend school until lesions are dry
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88
Q

what is a boil and what is the most common causative bacteria?

A

an infection of a hair follicle or sweat gland usually caused by staph aureus

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89
Q

how do you treat boils?

A
  1. systemic antibiotics

2. surgical excision

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90
Q

what should you suspect if a child has repeated cases of boils?

A

nasal carriage of staph aureus

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91
Q

what are 2 signs and symptoms of periorbital cellulitis?

A
  1. fever
  2. erythema, tenderness and oedema of the eyelid
  3. often unilateral and following trauma
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92
Q

what 2 anatomical locations/ pathologies can periorbital cellulitis commonly spread from?

A
  1. paranasal sinus

2. dental abscess

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93
Q

how do you treat periorbital cellulitis?

A

intravenous antibiotics like ceftriaxone to prevent posterior spread

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94
Q

what are three signs and symptoms of orbital cellulitis?

A
  1. proptosis
  2. reduced visual acuity
  3. painful ocular movement
  4. fever
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95
Q

how do you diagnose orbital cellulitis?

A
  1. clinical presentation

2. ct/mri

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96
Q

how do you treat orbital cellulitis?

A

antibiotics and drainage of any abscesses

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97
Q

what are 4 signs and symptoms of staphylococcal scalded skin syndrome?

A
  1. fever
  2. malaise
  3. localised infection around eyes, nose and mouth
  4. peeling and blistering skin
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98
Q

how do you treat staphylococcal scalded skin syndrome?

A
  1. IV flucloxacillin
  2. analgesia
  3. monitoring of fluid balance
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99
Q

what 3 vaccines does a baby get at 8 weeks old?

A
  1. 6 in 1 vaccine
  2. rotavirus vaccine
  3. meningitis B vaccine
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100
Q

what 3 vaccines does a baby get at 12 weeks old?

A
  1. 6 in 1 vaccine second dose
  2. pneumococcal PCV vaccine
  3. rotavirus vaccine second dose
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101
Q

what 2 vaccines does a baby get at 16 weeks old?

A
  1. 6 in 1 vaccine third dose

2. meningitis B vaccine second dose

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102
Q

what 6 diseases does the 6 in 1 vaccine protect against?

A
  1. diphtheria
  2. hepatitis B
  3. haemophilus influenza type b
  4. polio
  5. tetanus
  6. whooping cough
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103
Q

what 4 vaccines does a child get at 1 year old?

A
  1. HIB/ MenC
  2. MMR
  3. pneumococcal PCV vaccine second dose
  4. meningitis B vaccine third dose
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104
Q

what vaccine does a child get yearly between the ages of 2 and 10?

A

flu vaccine

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105
Q

what 2 vaccines does a child get at the age of 3 years and 4 months?

A
  1. MMR second dose

2. 4 in 1 pre-school booster

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106
Q

what vaccine does a female child get at the age of 12 to 13 years?

A
  1. HPV vaccine
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107
Q

what 2 vaccines does a child get at the age of 14 years?

A
  1. 3 in 1 teenage booster

2. MenACWY

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108
Q

what % of children have allergic rhinitis, asthma or eczema?

A

40%

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109
Q

what % of children have any food allergy?

A

6%

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110
Q

what is the cause of an allergy?

A

polymorphisms/mutations in genes leading to IgE or non-IgE mediated immune responses

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111
Q

describe an IgE mediated allergic response

A
  1. early phase within minutes caused by histamine release
  2. late phase response after several hours
  3. generally more severe and usually caused by inhaled allergens
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112
Q

describe a non-IgE mediated allergic response

A
  1. delayed onset of response
  2. varying clinical course
  3. often less severe than IgE mediated
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113
Q

what are 6 signs and symptoms of allergic disease?

A
  1. mouth breathing
  2. allergic salute- rubbing from itchy nose
  3. pale, swollen inferior nasal turbinates
  4. hyperinflated chest
  5. atopic eczema
  6. allergic conjunctivitis
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114
Q

what are 2 important things to do in a child with allergies?

A
  1. check their growth

2. identify triggers and manage if they have severe disease

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115
Q

is food allergy usually IgE mediated or non IgE mediated?

A

IgE mediated

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116
Q

what are 3 common food allergies in infants?

A
  1. milk
  2. peanuts
  3. eggs
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117
Q

what are 2 common food allergies in older children?

A
  1. nuts

2. fish

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118
Q

what is the clinical picture of a non-IgE mediated food allergy?

A

the allergy occurs hours after ingestion and involves the GI tract

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119
Q

what are 4 signs and symptoms of non-IgE mediated food allergy?

A
  1. diarrhoea
  2. vomiting
  3. abdominal pain
  4. faltering growth
  5. in first few weeks of life, can present with bloody stools
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120
Q

what are 3 signs and symptoms of IgE mediated food allergy?

A
  1. urticaria
  2. facial swelling
  3. anaphylaxis
  4. occurs 10-15 minutes after ingestion of food
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121
Q

how do you diagnose an allergy?

A
  1. skin prick test

2. IgE levels in blood

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122
Q

how do you diagnose a non-IgE allergy?

A
  1. endoscopy
  2. intestinal biopsy
  3. double blind placebo controlled food challenge
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123
Q

how do you treat a food allergy?

A
  1. avoidance of the food trigger
  2. non-sedating antihistamines for mild reactions
  3. epinephrine IM for severe reactions
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124
Q

what are the two types of eczema?

A
  1. atopic

2. non-atopic

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125
Q

what % of children with atopic eczema have another allergy?

A

50%

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126
Q

what % of young infants with severe eczema have IgE mediated food allergy?

A

40%

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127
Q

what are the two types of allergic rhinitis?

A
  1. atopic

2. non-atopic

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128
Q

what % of children are affected by allergic rhinitis?

A

20%

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129
Q

what is a symptom of allergic rhinitis that can cause a sore throat?

A

post-nasal drip

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130
Q

what are 3 conditions that are associated with allergic rhinitis?

A
  1. eczema
  2. sinusitis
  3. adenoidal hypertrophy
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131
Q

name 3 ways that you can treat rhinoconjuncitivitis?

A
  1. non-sedating antihistamines
  2. topical corticosteroid nasal/eye preparations
  3. cromogylcate eye drops
  4. leukotriene receptor antagonists
  5. nasal decongestants
  6. allergy immunotherapy
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132
Q

what is the physiology of urticaria?

A

local vasodilation and increased permeability of capillaries and venules

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133
Q

what happens if urticaria involved deeper tissues?

A

angioedema

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134
Q

if the allergy cause is urticarial, what life threatening condition is the patient at risk of?

A

anaphylaxis

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135
Q

how do you treat allergic urticaria?

A
  1. non-sedating antihistamines

2. omalizumab

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136
Q

what are 2 useful tumour markers in neuroblastoma?

A
  1. VMA

2. HVA

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137
Q

what is a useful tumour marker in germ cell tumours?

A
  1. high alpha fetoprotein
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138
Q

what is the primary curative treatment in all childhood cancers?

A

chemotherapy (surgery may follow in some diagnoses)

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139
Q

what are 4 common opportunistic infections in children with immunodeficiency?

A
  1. PCP
  2. aspergillosis
  3. candidiasis
  4. coagulase negative staph
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140
Q

what is a side effects of doxorubicin?

A

cardiotoxicity

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141
Q

what are 2 side effects of cisplatin?

A
  1. renal failure

2. deafness

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142
Q

what is a side effect of cyclophosphamide?

A

haemorrhagic cystitis

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143
Q

what is a side effect of vincristine?

A

neuropathy

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144
Q

what type of leukaemia accounts for 80% of cases in children?

A

acute lymphoblastic leukaemia, peaking at ages 2-5

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145
Q

what are two rare forms of blood cancer in children?

A
  1. myeloproliferative disorder

2. chronic myeloid leukaemia

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146
Q

what causes symptoms in leukaemia and what is the usual course of development?

A

disseminated disease from infiltration of bone marrow and other organs by leukaemic blast cells over a course of weeks

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147
Q

how do you diagnose leukaemia?

A
  1. FBC- low Hb, thrombocytopenia, evidence of circulating leukaemic blast cells
  2. bone marrow examination to confirm diagnosis
  3. clotting screen needed as 10% have DIC on diagnosis
  4. LP and CXR are done
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148
Q

what are 4 prognostic factors in leukaemia?

A
  1. age
  2. WCC at presentation
  3. cytogenetics
  4. response to treatment
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149
Q

how do you protect renal function in leukaemia treatment?

A

hydration and allopurinol

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150
Q

a combination of chemo and steroids gives what % of remission in leukaemia?

A

95%

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151
Q

what are 7 signs and symptoms of acute leukaemia?

A
  1. malaise
  2. anorexia
  3. nausea and vomiting
  4. lethargy
  5. easy bruising
  6. infection
  7. bone pain
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152
Q

how do you treat leukaemia?

A
  1. blocks of intense chemotherapy
  2. intrathecal chemo prevents CNS relapse
  3. cotrimoxazole pphx for pcp pneumonia
  4. relapse is high dose chemo and bone marrow transplantation
  5. supplemental steroid treatment
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153
Q

what are the most common paediatric brain tumours in order and % of cases?

A
  1. 60% infratentorial
  2. 40% astrocytoma
  3. 20% medulloblastoma
  4. 8% ependymoma
  5. 6% brainstem glioma
  6. 4% craniopharyngioma
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154
Q

what are 3 signs and symptoms of a brain tumour?

A
  1. raised ICP- irritable, drowsy, headache, vomiting, diplopia, decreased responsiveness
  2. focal neurological deficits
  3. ALSO spinal invasion can cause pack pain, incontinence and weakness in peripheries
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155
Q

how are brain tumours diagnosed?

A
  1. MRI

2. LP can stage since some tumours metastasise within CSF

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156
Q

how do you treat brain tumours?

A
  1. 1st line is surgery and is aimed at treating hydrocephalus
  2. chemotherapy and radiotherapy
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157
Q

which lymphoma is more common in childhood?

A

non-hodgkins lymphoma

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158
Q

which lymphoma is more common in adolescense?

A

hodgkins lymphoma

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159
Q

what are 2 symptoms of lymphoma?

A
  1. painless lymphadenopathy (often in the neck)

2. B symptoms- fever, night sweats, weight loss

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160
Q

how do you diagnose lymphoma?

A
  1. lymph node biopsy
  2. radiological assesment of nodal sites
  3. bone marrow biopsy
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161
Q

how do you treat lymphoma?

A

combination chemo with radio, and monitor treatment response with PET scans

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162
Q

what is the % cure rate with and without disseminated disease?

A

80% without, 60% with

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163
Q

what is burkitt lymphoma and what are the three primary types?

A

a B-cell variant of non-hodgkins lymphoma consisting of endemic variant (chronic malaria due to ebv), sporadic (ebv) and associated with immunodeficiency (HIV)

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164
Q

how do you treat burkitt lymphoma?

A

multiagent chemotherapy

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165
Q

what lymphocyte is most commonly involved in lymphoma?

A

B-cells

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166
Q

what tissue does neuroblastoma form from?

A

neural crest tissue in the adrenal medulla and sympathetic nervous system

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167
Q

what are 5 signs and symptoms of neuroblastoma?

A
  1. abdominal mass
  2. nerve compression
  3. bone pain
  4. weight loss
  5. malaise
  6. neck lump
  7. bruising, particularly around the eye
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168
Q

what is the usual organ of origin in abdominal primary neuroblastoma?

A

adrenal glands

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169
Q

how do you diagnose neuroblastoma?

A
  1. raised HVA/VMA

2. biopsy can be useful

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170
Q

how do you treat neuroblastoma?

A
  1. local primaries without mets by surgery
  2. mets by chemo, autologous stem cell rescue, surgery and radiotherapy
  3. there is a high relapse rate
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171
Q

what are 2 clinical syndromes that can present with neuroblastoma near the thoracic outlet?

A
  1. horner’s syndrome- ptosis, miosis, anhydrosis

2. superior vena cava syndrome- dyspnea, facial swelling, cough, distended neck veins, edema of the upper extremeties

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172
Q

what tissue does wilm’s nephroblastoma originate from?

A

embryonal renal tissue- undifferentiated mesodermal tumour of intermediate cell mass

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173
Q

what % of children with wilm’s nephroblastoma present before 5 years old?

A

80%

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174
Q

what are the four conditions in the acronym ‘wagr’ that are associated with wilm’s nephroblastoma?

A
  1. beckwith- (W)iedemann syndrome- overgrowth syndrome
  2. (A)niridia- iris is not present
  3. (G)U malformations
  4. (R)etardation
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175
Q

what tissue does rhabdomyosarcoma form from?

A

primitive mesenchymal tissue (cells that form MSK system amongst others) as it is a skeletal muscle cancer

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176
Q

what are 4 signs and symptoms of rhabdomyosarcoma that is retroperitoneal/abdominal?

A
  1. obstruction
  2. perforation
  3. bloating
  4. discomfort
  5. increased abdominal girth
  6. abdominal mass
  7. tenderness
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177
Q

what are 4 signs and symptoms of rhabdomyosarcoma that is a gastrointestinal stromal (occurs in the wall of intestine) tumour?

A
  1. anaemia symptoms- dizziness, fatigue, pallor
  2. weight loss
  3. GI bleeding
  4. may only present following perforation or obstruction
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178
Q

what are 4 signs and symptoms of rhabdomyosarcoma that occurs in the soft tissue of the extremities?

A
  1. painless mass
  2. weight loss
  3. fatigue
  4. anorexia
  5. painless mass is deep to the deep fascia
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179
Q

how do you diagnose rhabdomyosarcoma?

A
  1. CT/MRI
  2. endoscopy
  3. CT chest
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180
Q

how do you treat rhabdomyosarcoma?

A

multimodality treatment- chemotherapy and surgery

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181
Q

what is the primary symptom of ewing sarcoma/ osteosarcoma?

A

persistent localised bone pain with a mass

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182
Q

how do you diagnose ewing sarcoma/ osteosarcoma?

A
  1. X-ray, MRI and bone scan
  2. chest CT for lung mets
  3. bone marrow sampling
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183
Q

how do you treat ewing sarcoma/ osteosarcoma?

A

surgery and chemotherapy

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184
Q

which tumour accounts for 5% of severe visual impairments in children?

A

retinoblastoma

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185
Q

what is the cause of all bilateral retinoblastoma tumours?

A

hereditary/ genetics

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186
Q

which chromosome is the gene mutation for retinoblastoma located on?

A

chromosome 13

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187
Q

how do you diagnose retinoblastoma?

A
  1. clinical examination
  2. fundoscopy
  3. MRI scan
  4. examination under general anaesthesia
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188
Q

how do you treat retinoblastoma?

A
  1. chemotherapy
  2. laser treatment
  3. radiotherapy in advanced disease
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189
Q

what are the 2 most common primary malignant liver tumours?

A
  1. hepatoblastoma

2. hepatocellular carcinoma

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190
Q

what are 4 symptoms of primary malignant liver tumours?

A
  1. abdominal distension
  2. abdominal mass
  3. weight loss
  4. jaundice
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191
Q

what is elevated in the blood in primary malignant liver tumours?

A

serum alpha fetoprotein

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192
Q

how do you diagnose primary malignant liver tumours?

A
  1. serum alpha fetoprotein
  2. clinical examination
  3. ultrasound
  4. MRI
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193
Q

how do you treat primary malignant liver tumours?

A

chemotherapy or surgery

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194
Q

what treatment method are germ cell tumours highly sensitive to?

A

chemotherapy

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195
Q

what is langerhan’s cell histiocytosis?

A

abnormal proliferation of histiocytes and cytokine over production, leading t widespread inflammation

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196
Q

what are some signs and symptoms of langerhan’s cell histiocytosis?

A
  1. bone pain and/or swelling
  2. skin rash
  3. polydipsia and polyuria
  4. failure of growth or sexual maturation
  5. vertebra plana (a vertebra has lost almost all of its height)
  6. hepatosplenoegaly
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197
Q

what are langerhans cells?

A

dendritic cells of the skin

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198
Q

how do you diagnose langerhan’s cell histiocytosis?

A
  1. FBC
  2. LFTs
  3. serum albumin
  4. tissue biopsy
  5. coagulation screen
  6. renal function
  7. X-ray

very disseminated disease so loads of tests are fair game

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199
Q

how do you treat langerhan’s cell histiocytosis?

A
  1. chemotherapy
  2. surgery
  3. radiotherapy
  4. corticosteroid common
  5. desmopressin if diabetes insipidus
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200
Q

what is the anaemic Hb count in neonates?

A

<140g/L

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201
Q

what is the anaemic Hb count in 1-12 month old babies?

A

<100g/L

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202
Q

what is the anaemic Hb count in 1-12 year old children?

A

<110g/L

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203
Q

what are the three main pathological reasons a child will become anaemic?

A
  1. reduced red cell production
  2. haemolysis
  3. blood loss
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204
Q

what are 2 causes of reduced red cell production in anaemia and what conditions do they indicate?

A
  1. ineffective erythropoiesis (iron deficiency anaemia)

2. absence of production (red cell aplasia)

205
Q

what are 3 causes of iron deficiency anaemia?

A
  1. inadequate uptake
  2. malabsorption
  3. blood loss
206
Q

what are 2 symptoms of iron deficiency anaemia in babies?

A
  1. fatigue

2. slow feeding

207
Q

what is the colour and size of red blood cells and amount of reticulocytes in iron deficiency anaemia and what else is lowered?

A
  1. microcytic
  2. hypochromic
  3. normal reticulocytes
  4. low ferritin
208
Q

what are 3 causes of red cell aplasia?

A
  1. congenital
  2. transient erythroblastopenia of childhood
  3. parvovirus B19
209
Q

how do you diagnose red cell aplasia?

A

on bloods-

  1. low reticulocyte count
  2. normal bilirubin
  3. negative coomb’s test
  4. absent red cell precursors on bone marrow exam
210
Q

how do you treat red cell aplasia?

A
  1. steroids
  2. monthly RBC transfusions
  3. stem cell transplant
211
Q

what triggers transient erythroblastopenia of childhood?

A

viral infection, recovers in several weeks

212
Q

how does haemolytic anaemia cause anaemia?

A

reduced RBC lifespan, circulatory destruction in lover and spleen leads to anaemia when bone marrow can no longer compensate

213
Q

what are the three main paediatric causes of haemolytic anaemia?

A
  1. red cell membrane disorders (spherocytosis)
  2. red cell enzyme disorders
  3. haemoglobinopathies
214
Q

what are 4 signs of haemolytic anaemia?

A
  1. hepato/splenomegaly
  2. anaemia
  3. increased blood levels of unconjugated bilirubin
  4. increased urinary urobilinogen
215
Q

what are 5 diagnostic clues in haemolytic anaemia?

A
  1. raised reticulocyte count
  2. unconjugated bilirubinaemia
  3. abnormal rbc appearance
  4. positive coomb’s test
  5. increased RBC precursors
216
Q

what is coomb’s test?

A

a test that checks for antibodies against red blood cells

217
Q

what are 5 signs and symptoms of spherocytosis?

A
  1. jaundice
  2. anaemia
  3. splenomegaly
  4. aplastic crisis
  5. gallstones
218
Q

what is diagnostic in haemolytic anaemia?

A

blood film

219
Q

how do you treat haemolytic anaemia?

A
  1. oral folic acid
  2. splenectomy if poor growth with penicillin pphx after
  3. up to date vaccinations
  4. aplastic crisis is 3 blood transfusions
220
Q

how is G6PD deficiency inherited?

221
Q

what are 2 signs and 3 precipitating factors in G6PD deficiency?

A
  1. neonatal jaundice
  2. acute haemolysis
  3. precipitated by infection
  4. precipitated by fava beans
  5. precipitated by naphthalene
222
Q

what are 4 signs/symptoms of haemolysis in G6PD deficiency?

A
  1. fever
  2. abdo pain
  3. malaise
  4. passage of dark urine
223
Q

how do you diagnose G6PD deficiency?

A

measure G6PD in RBC, blood picture between episodes looks normal

224
Q

how do you treat G6PD deficiency?

A

educate patient about acute haemolysis and specific foods to avoid. treat any precipitating infection

225
Q

how do haemoglobinopathies cause anaemia?

A
  1. reduced HbA

2. production of abnormal Hb

226
Q

what is the inheritance of sickle cell anaemia?

A

autosomal recessive

227
Q

what is the most severe form of sickle cell anaemia?

228
Q

what are 6 signs and symptoms of sickle cell anaemia?

A
  1. anaemia
  2. infection
  3. painful crisis
  4. priapism
  5. splenomegaly
  6. stroke
  7. cognitive problems
  8. renal dysfunction
229
Q

what medication should sickle cell anaemia patients take once daily?

A

folic acid

230
Q

what 4 things should sickle cell anaemia patients avoid?

A
  1. cold
  2. dehydration
  3. excessive exercise
  4. stress
231
Q

how do you treat sickle cell anaemia painful crises?

A
  1. oral/ IV analgesia
  2. good hydration
  3. exchange transfusion
232
Q

what medication might children who have recurrent visits with sickle cell anaemia benefit from and what do you have to monitor with this medication?

A

hydroxycarbamide, but monitor for white cell suppression

233
Q

what % of patients with serious sickle cell anaemia die before the age of 40?

234
Q

what eye condition do children with sickle cell disease often develop in their adolescense?

A

proliferative retinopathy

235
Q

what 2 regions does beta thalassaemia most often occur?

A
  1. indian subcontinent

2. middle east

236
Q

what is the physiology of beta thalassaemia?

A

there is a reduction in beta globulin and so a reduction in HbA (adult haemoglobin)

237
Q

what are 3 signs and symptoms of beta thalassaemia?

A
  1. severe anaemia
  2. faltering growth
  3. extra-medullary haematopoiesis (formation and maturation of blood cells outside of the bone marrow)
  4. heterozygotes are usually asymptomatic
238
Q

how do you treat beta thalassaemia?

A
  1. blood transfusions at the time of symptomatic anaemia

2. ensure that there is iron chelation with desferrioxamine to prevent iron overload

239
Q

how many alpha deletions can severe alpha thalassaemia have?

A

4 alpha deletions

240
Q

how do you diagnose alpha thalassaemia?

A
  1. liquid chromatography

2. Hb electrophoresis

241
Q

how do you treat alpha thalassaemia?

A
  1. blood transfusions

2. folic acid supplementation

242
Q

why does haemolytic disease of the newborn occur?

A

antibodies against the rhesus blood group antigens of the baby if the baby is the second rhesus +ve baby of a rhesus -ve mother

243
Q

what are four reasons that anaemia of prematurity occurs?

A
  1. inadequate production of erythropoietin
  2. reduced RBC lifespan
  3. frequent blood sampling
  4. iron/folic acid deficiency
244
Q

what is bone marrow failure syndrome more commonly known as?

A

aplastic anaemia

245
Q

what are 4 causes of aplastic anaemia?

A 
1. inherited
acquired-
2. viruses
3. drugs- sulphonamides and chemo
4. toxins- benzene gluie
246
Q

what are 4 signs and symptoms of aplastic anaemia?

A
  1. anaemia
  2. peripheral pancytopenia
  3. infection
  4. easy bruising/ bleeding
247
Q

what is the most common form of inherited aplastic anaemia?

A

fanconi anaemia

248
Q

what are 3 specific signs of fanconi anaemia?

A
  1. short stature
  2. abnormal thumbs
  3. renal malformations
249
Q

what age does bone marrow failure become apparent in children with fanconi anaemia?

A

around age 5-6

250
Q

what condition is fanconi anaemia at risk of transforming into?

A

acute leukaemia

251
Q

how do you treat fanconi anaemia?

A

bone marrow transplant

252
Q

what is a rare cause of bone marrow failure and what are 3 signs and symptoms?

A

shwachman-diamond syndrome

  1. bone marrow failure
  2. pancreatic failure
  3. skeletal abnormalities
253
Q

what 8 screening tests in bloods are necessary to screen for bleeding disorders?

A
  1. FBC
  2. blood film
  3. PT 2,5,7,10
  4. activated partial thromboplastin time/ aptt
  5. thrombin time
  6. quantitative fibrinogen assay
  7. D-dimers
  8. biochemical screen
254
Q

what type of inheritance is haemophilia A and B?

A

X-linked inheritance

255
Q

what factor is deficient in haemophilia A?

256
Q

what factor is deficient in haemophilia B?

257
Q

what is a rheumatological risk in severe haemophilia?

A

spontaneous bleeding into muscles and joints that can cause arthritis

258
Q

at what age do most children present with haemophilia?

259
Q

what are 5 signs and symptoms of haemophilia?

A
  1. excessive bruising and bleeding
  2. mucocutaneous bleeding
  3. fatigue
  4. extensive cutaneous purpura
  5. distended and painful abdomen
260
Q

how do you treat haemophilia?

A
  1. recombinant factor 8/9 IV in bleeding
261
Q

what medications/methods of taking medications should be avoided in haemophilia?

A
  1. IM injections
  2. NSAIDs
  3. aspirin
262
Q

if peripheral access is difficult in haemophilia, what access is required?

A

central venous catheter

263
Q

what medications can help haemophilia management without blood products?

A
  1. desmopressin

2. von willebrand factor- helps platelet adhesion and carries factor 8

264
Q

what is von willebrand disease and what is the inheritance patterns?

A

a qualitative/ quantitative deficiency in von willebrand factor which is autosomal dominant in inheritance

265
Q

what is the most common type of von willebrand disease and when is it usually diagnosed?

A

type 1, it is mild and usually diagnosed in puberty

266
Q

what blood reading is given by vitamin K deficiency?

A

prolonged prothrombin time

267
Q

what platelet count is necessary for thrombocytopenia

268
Q

what are 4 symptoms and signs of thrombocytopenia?

A
  1. bruising
  2. petechiae
  3. purpura
  4. mucosal bleeding
269
Q

what is immune thrombocytopenic purpura and what age does the condition normally present?

A

IgG autoantibody destruction of platelets. usually presents between 2-10y.

270
Q

if immune thrombocytopenic purpura presents with atypical features, what investigation is necessary, and what are 3 features?

A
  1. anaemia
  2. infection
  3. lymphadenopathy

bone marrow exam!

271
Q

how do you treat immune thrombocytopenic purpura?

A
  1. oral prednisolone
  2. IV anti-D or Ig
  3. rituximab is an option
  4. splenectomy for drug therapy failure
272
Q

what is disseminated intravascular coagulation?

A

activation of a coagulation pathway leading to diffuse fibrin deposition in microvasculature

273
Q

what are 2 common caused of DIC and what are 3 symptoms?

A

causes- severe sepsis or shock

  1. bruising
  2. purpura
  3. haemorrhage
274
Q

what are 5 signs of DIC where it should be suspected?

A
  1. thrombocytopenia
  2. prolonged PT
  3. prolonged APTT (partial thromboplastin time)
  4. low fibrinogen
  5. haemolytic anaemia
275
Q

what % of VTE events are secondary to underlying disorders?

276
Q

what are 3 congenital thrombophilia disorders?

A
  1. protein C/S deficiency
  2. antithrombin deficiency
  3. factor 5 leiden
277
Q

when should you screen for congenital thrombophilia disorders?

A

in an unanticipated venous thrombosis or child with FH of neonatal purpura fulminans

278
Q

what are 3 common hormonal causes that can lead to secondary disorders of sexual development?

A
  1. excessive androgens in XX
  2. inadequate androgen action
  3. gonadotropin insufficiency
279
Q

what is an inborn error of metabolism?

A

a disorder of enzymatic reactions that degrade, synthesise or interconvert molecules within cells

280
Q

what 6 features/signs/symptoms should trigger investigations for inborn errors of metabolism?

A
  1. dysmorphic features
  2. severe presentations of common illnesses
  3. significant metabolic acidosis
  4. unexplained respiratory alkalosis
  5. early onset seizures
  6. developmental regression
281
Q

what 5 things should be covered in the history when you suspect inborn errors of metabolism?

A
  1. family history of IEoM
  2. learning difficulty
  3. consanguinity
  4. sudden death
  5. epilepsy
282
Q

what is a common mode of inheritance for inborn errors of metabolism?

A

autosomal recessive

283
Q

what are two broad ways of managing inborn errors of metabolism, and what are 2 specific treatments for each?

A
  1. medication- symptomatic therapy, enzyme replacement therapy
  2. dietary manipulation- supplying deficient product, prevent accumulation of toxic substrate
284
Q

what is a common trigger of inborn error of metabolism?

285
Q

what should be measured if there is unexplained encephalopathy, respiratory alkalosis, recurrent vomiting or seizures?

A

ammonia levels (urea cycle error)

286
Q

how do you treat an inborn error of metabolism that cause a high ammonia?

A
  1. stop feeds
  2. give 10% dextrose
  3. IV ammonia scavengers
  4. arginine
287
Q

what are the three types of glycogen storage disorders?

A
  1. hepatic- associated with hypoglycaemia
  2. muscular
  3. cardiac
288
Q

what are 4 features of glycogen storage disorders?

A
  1. poor feeding
  2. hepatomegaly
  3. raised lactate
  4. neutropenia
289
Q

how do you diagnose lysosomal storage disorders?

A
  1. urinary glycosaminoglycan screen
  2. oligosaccharide screen
  3. white cell enzyme testing
290
Q

what are mucopolysaccharidoses?

A

progressive disorder that affects neurological, ocular, cardiac and skeletal function

291
Q

what do most patients with mucopolysaccharidoses present with and what is the treatment?

A

developmental delay, supportive treatment

292
Q

what 2 signs should make you consider mitochondrial disease?

A
  1. multi-system elevated lactate

2. MRI showing characteristic features

293
Q

what is the most common lipid storage disorder?

A

gaucher disease

294
Q

what is the most common inherited disorder of lipid metabolism?

A

familial hypercholesterolaemia

295
Q

how do you treat familial hypecholesterolaemia?

A
  1. low fat diet
  2. statin
  3. ezetimibe
296
Q

what is a pathological cause of musculoskeletal disorder?

297
Q

what is a common initial symptom of musculoskeletal disorders?

A

bowing of the tibiae

298
Q

what 2 things are indicated if a child has a painful flat foot?

A

tendo-achilles contracture or juvenile idiopathic arthritis

299
Q

what are 3 causes of in-toeing?

A
  1. metatarsus varus
  2. medial tibial torsion
  3. persistent anteversion of femoral neck
300
Q

what degenerative condition should be excluded in older boys with in-toeing?

A

duchenne muscular dystrophy

301
Q

what is positional talipes and what causes it?

A

also known as ‘clubfoot’ where the foot rests downwards and inwards due to intrauterine compression, it can be gently manipulated back into position with passive exercises

302
Q

what is talipes equinovarus?

A

a complex abnormality where the entire foot is inverted and supinated, affected foot is shorter and the calf is thinner.

303
Q

what are 2 causes of talipes equinovarus?

A
  1. familial

2. oligohydramnios

304
Q

how do you treat talipes equinovarus?

A

plaster casting and bracing

305
Q

what is vertical talus?

A

the foot is stiff and rocker-bottom in shape (the bottom of the foot is convex rather than concave)

306
Q

how do you treat vertical talus?

A

corrective surgery

307
Q

what is talipes calcaneovalgus?

A

dorsiflexed and everted foot

308
Q

what is tarsal coalition and how is it treated?

A

an abnormal connection between tarsal bones in the foot, it must be treated with surgery as it gets progressively more rigid

309
Q

what are 3 problems with the position of the hip in developmental dysplasia of the hip?

A
  1. dysplasia
  2. subluxation
  3. dislocation
310
Q

what is the usual presentation of developmental dysplasia of the hip?

A

limp or abnormal gait

311
Q

what is scoliosis?

A

lateral curvature in the frontal plane of the spine

312
Q

what is abnormal in the structural form of scoliosis?

A

rotation of the vertebral bodies causing prominence in the back from rip asymmetry

313
Q

what are 4 causes of scoliosis?

A
  1. idiopathic
  2. congenital
  3. neuromuscular imbalance
  4. connective tissue disorder
314
Q

what is torticollis?

A

an abnormal, asymmetrical head or neck position

315
Q

what is a common cause of torticollis?

A

sternocleidomastoid tumour, there will be a mobile, non-tender nodule

316
Q

what are 3 causes of torticollis presenting later in life due to muscular spasm?

A
  1. ENT infection
  2. spinal tumour
  3. C-spine arthritis
317
Q

what are 3 common characteristics of growing pains?

A
  1. the pain often wakes the child
  2. the pain is never present at the start of the day
  3. the pain is symmetrical in the lower limbs
318
Q

what 2 broad categories of disorders should be suspected in a child with hypermobile joints?

A
  1. chromosomal abnormalities

2. collagen disorders

319
Q

what are 4 signs and symptoms of localised complex regional pain syndrome in the lower limbs?

A
  1. ankle and foot involvement
  2. hyperaesthesia
  3. allodynia
  4. bizarre posturing
320
Q

what are 3 signs and symptoms of diffuse complex regional pain syndrome?

A
  1. widespread pain
  2. disturbed sleep patterns
  3. feeling exhausted during the day
321
Q

what is osteomyelitis and what are 2 commonly affected locations?

A

infection of the metaphysis of the long bones usually, often affecting the distal femur and the proximal tibia

322
Q

what infectious cause of osteomyelitis should be considered in the immunocomprimised?

A

tuberculosis

323
Q

what is the clinical presentation of osteomyelitis?

A

a painful immobile limb in a child with a febrile illness. infection sites are often erythematous and warm

324
Q

what 2 levels will be raised in bloods of a child with osteomyelitis?

A

CRP and WCC

325
Q

what blood cancer can present with bone pain in children?

A

acute lymphoblastic leukaemia

326
Q

what are 3 signs and symptoms of bone tumours?

A
  1. pathological fracture
  2. bone pain
  3. regional swelling
327
Q

which bones does the benign tumour ‘osteoid osteoma’ commonly affect children?

A
  1. femur
  2. tibia
  3. spine
328
Q

what are 2 characteristics of the pain caused by an osteoid osteoma?

A
  1. the pain is more severe at night

2. it improves with nsaids

329
Q

what is osgood-schlatter disease?

A

osteochondritis of a patellar tendon in adolescent physically active males

330
Q

what is the clinical presentation of osgood-schlatter disease?

A

knee pain following exercise with localised tenderness and swelling. can be bilateral.

331
Q

how do you treat osgood schlatter disease?

A
  1. reduced exercise
  2. physiotherapy
  3. knee immobiliser splint
332
Q

what is chondromalacia patellae, and who does it usually affect?

A

a condition where there is softening of the articular cartilage behind the patellae, mostly occurring in adolescent females

333
Q

how do you treat chondromalacia patellae?

A

physiotherapy

334
Q

what is osteochondritis dissecans?

A

avascular necrosis of a small segment of bone, usually around the knee, that can cause cracks in the bone and articular cartilage

335
Q

what are 2 signs and symptoms of osteochondritis dissecans?

A
  1. persistent knee pain

2. localised tenderness over femoral epicondyles

336
Q

how do you treat osteochondritis dissecans?

A
  1. rest

2. quadriceps exercises

337
Q

what are 6 causes of back pain in younger children?

A
  1. mechanical causes
  2. tumours
  3. vertebral osteomyelitis/ discitis
  4. spinal cord/ nerve root entrapment
  5. sheuermann disease
  6. spondylolysis
338
Q

what condition causes a feeling on instability in the knee and how do you treat it?

A

subluxation of the patella, quadriceps exercise

339
Q

what is a common cause of acute hip pain in children with a limp?

A

transient synovitis

340
Q

what is the clinical presentation of transient synovitis causing a limp?

A
  1. acute hip pain following an infection
  2. decreased movement
  3. referred knee pain
341
Q

what condition might transient synovitis precede in the hip?

A

perthes disease- avascular necrosis of the capital femoral epiphyses of the femoral head

342
Q

how do you treat transient synovitis?

A

bed rest and skin traction

343
Q

what is the more common age and gender of children affected by perthes disease?

A

5:1 boys age 5-10

344
Q

how can you diagnose perthes disease?

A

bone scan and MRI

345
Q

how do you treat perthes disease?

A
  1. rest
  2. physiotherapy
  3. plaster casts
  4. surgery
346
Q

what can a slipped capital femoral epiphyses lead to if it is not treated with surgery and who is it more common in?

A

avascular necrosis, most common in obese boys

347
Q

what observations of the movements of the hip can you make in a slipped capital femoral epiphyses and how is it diagnosed?

A

restricted abduction and internal rotation of the hip, diagnosed by x-ray

348
Q

what are 5 signs and symptoms of acute arthritis in children?

A
  1. pain
  2. swelling
  3. heat
  4. redness
  5. restricted movement
349
Q

what is the clinical presentation of reactive arthritis?

A

transient joint swelling, usually of ankle and knees, following extra-articular infection

350
Q

how do you treat reactive arthritis?

351
Q

what are 5 signs and symptoms of septic arthritis?

A
  1. erythhema
  2. acutely tender joint
  3. warm joint
  4. reduced range of movement
  5. detectable joint effusion
352
Q

what is the most common cause and age in septic arthritis?

A

staph aureus, <2 years old

353
Q

how do you diagnose septic arthritis?

A
  1. blood cultures
  2. fbc with raised WCC and CRP
  3. ultrasound showing joint effusion
  4. joint aspirate is DIAGNOSTIC since xray is usually normal
354
Q

how do you treat septic arthritis?

A

prolonged IV abx with joint washout and surgical drainage

355
Q

what is the most common inflammatory joint disease in children?

A

juvenile idiopathic arthritis

356
Q

what is the clinical presentation of juvenile idiopathic arthritis?

A

joint swelling >6w in <16yo with no infection. there is stiffness at rest, morning stiffness and pain.

357
Q

what factor may be present in the blood of a patient with juvenile idiopathic arthritis?

A

antinuclear factor

358
Q

what are 5 complications of juvenile idiopathic arthritis

A
  1. chronic anterior uveitis
  2. flexion contracture
  3. growth failure
  4. anaemia of chronic disease
  5. delayed puberty
  6. osteoporosis
  7. amyloidosis
359
Q

how do you treat juvenile idiopathic arthritis?

A
  1. NSAIDS and analgesics
  2. joint injections
  3. methotrexate
  4. systemic corticosteroids
  5. cytokine modulators
360
Q

what is the commonest form of vasculitis in children?

A

henoch schonlein purpura

361
Q

what is the clinical presentation of henoch schonlein purpura?

A

purpuric rash on the legs and buttocks, often with abdo pain, haematuria and proteinuria

362
Q

what are 4 signs and symptoms of juvenile dermatomyositis?

A
  1. malaise
  2. progressive weakness
  3. progressive facial rash
  4. muscle pain
363
Q

what are 2 potential complications of juvenile dermatomyositis?

A
  1. respiratory failure

2. aspiration pneumonia

364
Q

what is the inheritance pattern of achondroplasia?

A

autosomal dominant

365
Q

what are 6 features of achondroplasia?

A
  1. short stature
  2. large head
  3. frontal bossing
  4. depression of nasal bridge
  5. lumbar lordosis
  6. hydrocephalus
366
Q

what are 2 features of thanatophoric dysplasia and what does it commonly result in?

A
  1. large head
  2. short limbs with folds of skin

commonly results in stillbirth

367
Q

what is arthrogryphosis?

A

stiffness and contracture of joints, with muscle atrophy around suspected joints

368
Q

what intrauterine condition is associated with arthrogryphosis?

A

oligohydramnios

369
Q

how do you manage arthrogryphosis?

A
  1. physiotherapy

2. correction of deformities

370
Q

what is osteogenesis imperfecta?

A

disorder of collagen metabolism causing bone fragility, bowing and frequent fractures

371
Q

what is the inheritance pattern of type 1 osteogenesis imperfecta?

A

autosomal dominant

372
Q

what are 2 features in childhood that might show a child has type 1 osteogenesis imperfecta?

A
  1. frequent fractures

2. blue sclera

373
Q

how do you treat osteogenesis imperfecta type 1?

A

bisphosphonates

374
Q

which type of osteogenesis imperfecta is the severe lethal form?

375
Q

what is osteopetresis?

A

a condition where the bones are dense and brittle

376
Q

what are 5 signs and symptoms of osteopetresis?

A
  1. faltering growth
  2. recurrent infection
  3. hypocalcaemia
  4. anaemia
  5. thrombocytopaenia
377
Q

how do you treat osteopetresis?

A

bone marrow transplant

378
Q

what are 4 signs and symptoms of marfans syndrome

A
  1. tall stature
  2. long thin digits
  3. hyperextensive joint
  4. hyperextensible lenses of the eyes
379
Q

what are the two main cardiovascular problems with marfans syndrome?

A

aneurysms and valvular incompetence

380
Q

what are 5 causes of disorders of sexual development in women?

A
  1. excessive androgens
  2. inadequate androgen action
  3. gonadotropin insufficiency
  4. ovotesticular DSD
381
Q

what ages must puberty occur in boys and girls to be considered precocious?

A
  1. before 9 years old in boys

2. before 8 years old in girls

382
Q

what are 2 central causes of precocious puberty?

A
  1. craniopharyngioma
  2. pituitary tumour
    (gonadotrophin dependent)
383
Q

what are 2 peripheral causes of precocious puberty?

A
  1. testis or adrenal problems

2. raised HCG from a tumour

384
Q

describe the 4 stages of puberty in boys

A
  1. scrotal and testicular enlargement. downy hair. textured scrotum
  2. penile growth. darker and curlier hair
  3. penile growth in length and breadth, larger glans, adult type hair
  4. adult sized testes and scrotum. adult quantity and pattern of pubic hair presenting on the inside of the thighs
385
Q

describe the 4 stages of breast development during puberty

A
  1. areolar enlargement and breast bud development
  2. enlargement of entire breast
  3. enlargement of the areola and papilla
  4. adult breast configuration with protrusion of the nipple
386
Q

describe the 4 stages of pubic hair development during puberty in girls

A
  1. straight hair extending along labia
  2. more, darker hair in a triangle formation
  3. more dense, curled and adult distribution of hair
  4. abundant adult type hair that may extend onto the medial aspect of the thighs
387
Q

what investigations can be done to diagnose precocious puberty?

A
  1. history- onset of secondary sexual characteristics
  2. tanner staging of puberty
  3. skeletal x-ray for bone age
  4. ct/mri
  5. tsh, lh, fsh, hcg, afp, gh, pituitary testing
388
Q

how do you manage precocious puberty?

A
  1. synthetic GnRH analogue
  2. endogenous oestrogen to accelerate growth
  3. reassurance that the child will develop normally
389
Q

how do you define delayed puberty?

A

lack of any pubertal signs by the age of 13 in girls and 14 in boys

390
Q

what are 3 functional causes of delayed puberty?

A
  1. constitutional delay
  2. underlying chronic disease
  3. malnutrition
391
Q

what are 2 organic causes of delayed puberty?

A
  1. hypogonadotropic hypogonadism

2. hypergonadotropic hypogonadism

392
Q

what is hypogonadotropic hypogonadism?

A

lack of gonadotropin production or action

393
Q

what is hypergonadotropic hypogonadism?

A

gonadal insufficiency with elevated gonadotropins

394
Q

what are some investigations that can be done for delayed puberty?

A
  1. height and tanner staging
  2. non-dominant wrist x-ray
  3. basal fsh and lh testing
  4. brain mri
  5. thyroid function test
395
Q

how do you manage delayed puberty?

A
  1. support and observation if not psychologically affected
  2. short course of testosterone in boys and oestrogen in girls to stimulate puberty
  3. treat chronic illness
  4. permanent cause in girls add cyclic progesterone after breakthrough bleeding
396
Q

what are 5 useful observations with ambiguous genitalia?

A
  1. exposure to testosterone or progesterone?
  2. penis size and urethral position?
  3. fused labia?
  4. vaginal opening?
  5. descended gonads?
397
Q

what are 2 chromosomal disorders of sexual development and what are the corresponding sex chromosomes?

A
  1. turner’s syndrome- X
  2. klinefelter’s syndrome-
    XXY
398
Q

what is the clinical presentation of turner’s syndrome?

A
  1. short stature
  2. premature ovarian failure
  3. phenotypically female
  4. 20-30% neck webbing
  5. dysmorphic features
  6. congenital heart defects
399
Q

how do you diagnose turner’s syndrome?

A
  1. karyotype testing
  2. bone age
  3. echocardiogram
  4. serum fsh and amh
400
Q

how do you manage turner’s syndrome?

A
  1. surveillance and preventive care
  2. GH supplementation with poor growth
  3. low-dose oestrogen with pubertal delay with cyclic progesterone
  4. cardiovascular assessment if cardiac abnormality
  5. ovarian HRT, breast implants and monitoring after cyclical bleeding
401
Q

what is the clinical presentation of klinefelter’s syndrome?

A
  1. infertility
  2. hypogonadism
  3. gynaecomastia
  4. normal puberty
  5. tall stature
402
Q

how do you diagnose klinefelter’s syndrome?

A
  1. karyotype test

2. blood or urine sampling for hormone levels

403
Q

how do you manage klinefelter’s syndrome?

A
  1. testosterone replacement therapy

2. speech and language therapy during childhood

404
Q

what % of undescended testis are palpable in the upper portion of the scrotum or the inguinal canal?

405
Q

what are 2 consequences for delayed/ lack of treatment of undescended testis?

A
  1. higher incidence of testicular cancer

2. reduced fertility

406
Q

what malformation alongside undescended testis might suggest a disorder of sexual development?

A

hypospadias

407
Q

how do you treat undescended testis?

A
  1. if palpable- orchiopexy
  2. if non-palpable-
    exam under anaesthesia and orchiopexy, or surgical exploration
  3. if bilateral non-palpable-
    endocrinology and urology referral
  4. post-pubertal-
    biopsy (as well as orchiopexy or orchiectomy)
408
Q

what are the three types of congenital hypothyroidism?

A
  1. athyreosis
  2. thyroid dysgenesis
  3. dyshormonogenesis
409
Q

what are 3 acquired causes of hypothyroidism?

A
  1. prematurity
  2. hashimoto’s thyroiditis
  3. hypopituitarism
410
Q

what are 5 signs of hypothyroidism at birth?

A
  1. prolonged neonatal jaundice
  2. poor feeding
  3. hypotonia
  4. widely opened posterior fontanelle
  5. inactivity
411
Q

what are 4 later signs of hypothyroidism in children?

A
  1. delayed puberty
  2. short stature
  3. low IQ
  4. delayed dentition
412
Q

how do you diagnose cogenital/acquired hypothyroidism?

A
  1. universal neonatal screening - heel prick
  2. low T4, high TSH, low Hb
  3. bone age less than physical age
413
Q

how do you manage hypothyroidism?

A

levothyroxine replacement adjusted according to growth and clinical state

414
Q

what is the typical clinical presentation of hyperthyroidism?

A

pubertal girl with palpitations, tremor, anxiety and tachycardia

415
Q

how do you diagnose hyperthyroidism?

A
  1. low TSH and high T4

2. fine needle cytology of goitres may show juvenile autoimmune thyroiditis

416
Q

how do you manage hyperthyroidism?

A
  1. carbimazole

2. propylthiouracil until euthyroid then lower maintenance dose

417
Q

what is the clinical presentation of croup?

A

Usually a child under the age of 6, with stridor, a barking cough, and hoarseness from laryngeal obstruction

418
Q

what time of year is croup the most common?

419
Q

what are 3 viral causes of croup?

A
  1. parainfluenza virus
  2. respiratory syncytial virus
  3. measles
420
Q

what is the pathophysiology of croup?

A

subglottic oedema, inflammation and exudate

421
Q

how do you treat croup?

A

mild disease (minimal recession, stridor, no cyanosis) can be sent home with oral dexamethasone, children with more severe cases must be treated with a steroid, given oxygen if they have low sats or decompensation and nebulised adrenaline if there is a poor response

422
Q

if croup fails to improve with steroids, what condition should be considered?

A

bacterial tracheitis

423
Q

what are the three upper respiratory tract pathologies that cause stridor in children?

A
  1. croup
  2. epiglottitis
  3. bacterial tracheitis
424
Q

what is the commonest lung infection in children?

A

acute bronchiolitis

425
Q

what are 5 signs and symptoms of acute bronchiolitis?

A
  1. cough
  2. tachypnoea
  3. wheeze
  4. inspiratory crackles
  5. coryza (preceding cough)
426
Q

what virus typically causes acute bronchiolitis?

A

respiratory syncytial virus

427
Q

what investigations should be done in acute bronchiolitis?

A

if severe, CXR to exclude pneumothroax or lobar collapse, blood gases and FBC

428
Q

how do you treat acute bronchiolitis?

A
  1. oxygen until sp02 is around 92%
  2. nasogastric feeding
  3. steroids and nebulised adrenaline can reduce changes of admission
429
Q

what should be given to immune compromised patients in order to prevent them developing acute bronchiolitis?

A

ribavirin prophylaxis

430
Q

what are 6 signs and symptoms of pneumonia?

A
  1. malaise
  2. poor feeding
  3. respiratory distress
  4. tachypnoea
  5. cyanosis
  6. typical lobar signs in older children (pleural pain, crackles, bronchial breathing
431
Q

what is the oxygen saturation threshold for admittance in children with pneumonia?

A

92% with signs of respiratory distress

432
Q

what tests should be done for pneumonia?

A
  1. CXR
  2. FBC
  3. sputum cultures
  4. blood cultures

these are considered in severe pneumonia and are not regularly required in community-acquired pneumonia

433
Q

is viral or bacterial lower respiratory tract infection more common in children?

434
Q

how do you treat pneumonia?

A
  1. mild cases can be discharged with no antibiotics

2. amoxicillin 1st line, alternatives are co-amoxiclav, azithromycin

435
Q

what are four bacterial causes of pneumonia?

A
  1. pneumococcus
  2. mycoplasma
  3. haemophilus
  4. staphylococcus
436
Q

what lower respiratory tract condition should be considered in children if they have overseas contacts, are HIV positive or have a strange CXR?

A

tuberculosis

437
Q

what are 3 signs of whooping cough?

A
  1. apnoea
  2. bouts of coughing that end with vomiting and are worse at nights or with feeds
  3. whooping after coughing
438
Q

how do you diagnose whooping cough?

A

PCR via nasal swab

439
Q

how do you treat whooping cough?

A
  1. azithromycin if infants are under 1 month of age

2. children greater than 1 month of age are given a macrolide or trimethoprim

440
Q

is diphtheria an URTI or a LRTI?

441
Q

what are 5 signs and symptoms of diphtheria?

A
  1. tonsilitis
  2. dysphagia
  3. muffled voice
  4. pronchopneumonia
  5. brassy cough and then airway obstruction
442
Q

how do you diagnose diphtheria?

A

PCR of swab below pseudomembrane in the oropharynx

443
Q

how do you treat diphtheria?

A

diphtheria antitoxin and erythromycin

444
Q

what % of the paediatric population is asthmatic?

445
Q

what are 4 factors that increase the prevalence of asthma?

A
  1. low birthweight
  2. family history
  3. atopy
  4. pollution
446
Q

what are 5 triggers for asthma?

A
  1. pollen
  2. house dust mite
  3. feathers
  4. fur
  5. exercise
447
Q

what are 4 differentials for an asthma attack?

A
  1. foreign body
  2. pertussis
  3. croup
  4. pneumonia
448
Q

what are 5 aspects of the general management of asthma?

A
  1. annual symptom review
  2. oral steroid use
  3. check inhaler technique
  4. check medication adherence
  5. personalised self-management action plan
449
Q

how do you treat a standard exacerbation of asthma?

A

rescue prednisolone

450
Q

what is the stepwise treatment algorithm for asthma?

A
  1. begin with an inhaled beta agonist like salbutamol
  2. add an inhaled steroid like beclometasone
  3. review diagnosis, check concordance, eliminate triggers
  4. add a dose of montelukast
  5. refer to specialist and increase inhaled steroid dose
  6. add prednisolone at the lowest functional dose
451
Q

what are 6 important points in the treatment of severe asthma in an emergency?

A
  1. sit the patient up and put them on 100% oxygen
  2. nebulised salbutamol with ipatropium bromide
  3. hydrocortisone or prednisolone
  4. consider a dose of magnesium sulfate
  5. IV aminophylline
  6. nebulise continuously until improvement and then in less frequent intervals
452
Q

what 4 things should be ensured before discharge follow a severe exacerbation of asthma?

A
  1. the peak flow is greater than 75% of the predicted peak flow
  2. there is good inhaler technique
  3. the patient is stable on the discharge regimen
  4. there is a written management plan
453
Q

what are 3 symptoms of a life threatening asthma exacerbation?

A
  1. PEFR <33% of expected
  2. sats <92%
  3. silent chest
454
Q

what are 3 symptoms of an acute severe asthma exacerbation?

A
  1. PEFR 33-50% of expected
  2. inability to complete sentences
  3. use of accessory muscles
455
Q

what are 3 symptoms of a moderate asthma exacerbation?

A
  1. PEFR 50-70% of expected
  2. no features of severe asthma
  3. worsening symptoms
456
Q

what are the two types of brittle asthma?

A
  1. type 1- wide variability in PEFR despite intensive therapy
  2. type 2- sudden severe attacks despite apparently well controlled asthma
457
Q

what pathological changes does a dry cough with a prolonged expiratory phase suggest?

A

narrowing of small-moderate sized airways

458
Q

what pathological changes does a barking cough suggest?

A

tracheal inflammation

459
Q

what pathological changes does a moist cough suggest?

A

lower airway infection

460
Q

what is the current life expectancy for newborns with cystic fibrosis?

461
Q

which chromosome is the CFTR gene on?

A

chromosome 7

462
Q

what substance is raised in blood taken from the heel in infants who have cystic fibrosis?

A

immunoreactive trypsinogen

463
Q

what substance is low in the blood that demonstrates pancreatic insufficiency due to cystic fibrosis?

A

faecal elastase

464
Q

how do you diagnose cystic fibrosis?

A
  1. sweat test detecting chloride

2. older children children can have spirometry

465
Q

how do you treat cystic fibrosis?

A
  1. physiotherapy 2x a day to clear mucus
  2. physical exercise
  3. prophylactic abx with fluclox
  4. azithromycin to reduce infections
  5. lung transplantation
466
Q

what is primary ciliary dyskinesia?

A

congenital abnormality in structure and function of cilia

467
Q

what are 5 signs and symptoms of primary ciliary dyskinesia?

A
  1. recurrent urti/lrti
  2. recurrent productive cough
  3. purulent nasal discharge
  4. chronic ear infections
  5. 50% dextrocardia (heart is flipped)
468
Q

how do you treat primary ciliary dyskinesia?

A

daily physiotherapy and antibiotics

469
Q

what are 4 causes of heart failure in neonates?

A
  1. hypoplastic left heart syndrome
  2. aortic stenosis
  3. coarctation of the aorta
  4. interruption of the aortic arch
470
Q

what are 3 causes of heart failure in infants?

A
  1. VSD
  2. ASD
  3. patent ductus arteriosus
471
Q

what are 3 causes of heart failure in older children?

A
  1. cardiomyopathy
  2. eisenmengers
  3. rheumatic heart disease
472
Q

if heart failure is progressive in the first week of life, what kind of shunt is it?

A

left to right shunt

473
Q

what are 3 causes of left to right shunt?

A
  1. ASD
  2. VSD
  3. patent ductus arteriosus
474
Q

what are signs and symptoms of left to right shunts?

A
  1. chronic chest infections
  2. heart failure
  3. arrhythmias
  4. ejection systolic murmur
  5. cardiomegaly
475
Q

what are 5 signs of large ventricular septal defects?

A
  1. tachycardia
  2. tachypnoea
  3. hepatomegaly
  4. soft pansystolic murmur
  5. cardiomegaly
476
Q

how do you treat paediatric heart failure due to large ventricular septal defects?

A
  1. diuretics- captopril
  2. increased calories
  3. surgery to repair the defect
477
Q

what murmur is heard with a patent ductus arteriosus?

A

continuous murmur below the left clavicle

478
Q

what type of pulse is there with patent ductus arteriosus?

A

collapsing pulse

479
Q

what complications can arise with a large patent ductus arteriosus?

A
  1. heart failure

2. pulmonary hypertension

480
Q

how do you treat patent ductus arteriosus?

A

surgical closure with coil or occlusion device

481
Q

what infection does treatment of patent ductus arteriosus help to prevent?

A

bacterial endocarditis

482
Q

what are 2 causes of right to left shunts?

A
  1. tetralogy of fallot

2. translocation of the great arteries

483
Q

how do right to left shunts usually present?

A

cyanosis in the first week of life

484
Q

what is the nitrogen washout test and what is it used for?

A

it is used to determine if a cyanosed neonate has heart disease. the neonate is placed in 100% O2 for 10 minutes and if the right radial artery PaO2 remains low, they have heart disease.

485
Q

how do you manage a cyanosed neonate?

A
  1. ABC

2. prostaglandin infusion

486
Q

what are the 4 features of tetralogy of fallot?

A
  1. overriding aorta
  2. subpulmonary stenosis
  3. right ventricular hypertrophy
  4. large ventricular septal defect
487
Q

what murmur and nail pathology are present in children with tetralogy of fallot?

A
  1. loud ejection systolic murmur at left sternal edge

2. clubbing

488
Q

what does the CXR look like with tetralogy of fallot?

A

small, boot shaped heart with an untilted apex and decreased pulmonary vascular markings

489
Q

how do you manage hypercyanotic spells in children with tetralogy of fallot?

A
  1. pain relief
  2. sedation
  3. iv propranolol
  4. bicarbonate
  5. muscle paralysis/ ventilation
490
Q

what is translocation of the great arteries?

A

the aorta is connected to the right ventricle and the pulmonary artery is connected to the left ventricle

491
Q

is translocation of the great arteries a cyanotic condition?

492
Q

what can be heard on auscultation in translocation of the great arteries?

A

the 2nd heart sound is loud and single

493
Q

how do you treat translocation of the great arteries?

A
  1. maintain ductus arteriosus with prostaglandins
  2. ballon atrial septostomy to enlarge the foramen ovale
  3. arterial switching surgery
494
Q

what syndrome can develop with untreated translocation of the great arteries?

A

eisenmenger syndrome, where the shunt reverses to a left to right shunt

495
Q

what genetic condition are atrioventricular septal defects commonly seen in?

A

Down Syndrome

496
Q

what are the three lesions associated with outflow obstruction in a well child?

A
  1. aortic stenosis
  2. pulmonary stenosis
  3. adult-type coarctation of the aorta
497
Q

what are three lesions associated with outflow obstruction in a sick child?

A
  1. coarctation of the aorta
  2. interruption of the aortic arch
  3. hypoplastic left heart syndrome
498
Q

what is the most common childhood arrhythmia?

A

SVT (supraventricular tachycardia)

499
Q

how does supraventricular tachycardia present?

A
  1. pulmonary oedema
  2. heart failure
  3. hydrops foetalis
500
Q

how do you treat supraventricular tachycardia?

A
  1. circulatory/ respiratory support
  2. vagal stimulating manoeuvre with IV adenosine
  3. electrical cardioversion
501
Q

what are 5 signs and symptoms of infective endocarditis?

A
  1. sustained fever
  2. malaise
  3. raised ESR
  4. unexplained anaemia
  5. haematuria
502
Q

how do you diagnose infective endocarditis?

A
  1. blood culture

2. ECHO is diagnostic

503
Q

how do you treat infective endocarditis?

A
  1. high dose penicillin and aminoglycoside IV
  2. surgical removal of prosthetic valves
  3. good dental hygiene for prophylaxis
504
Q

what is the clinical presentation of dilated cardiomyopathy?

A

previously well child presenting with HF and cardiomegaly

505
Q

how do you diagnose dilated cardiomyopathy?

506
Q

how do you treat dilated cardiomyopathy?

A
  1. diuretics
  2. ace inhibitors
    carvedilol
507
Q

what are 5 signs and symptoms of myocarditis?

A
  1. fatigue
  2. dyspnoea
  3. chest pain
  4. fever
  5. palpitations
508
Q

how do you treat myocarditis?

A

most cases resolve by themselves but some patients will get intractable heart failure and require a heart transplant

Phase 3A Flashcards (6 decks)

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