PAEDS RENAL/NEONATAL/INFECTION/ALLERGY/DERM/ONCOLOGY/HAEMATOLOGY/MISC Flashcards

Question 1

Q

PROTEINURIA
What is proteinuria?

Answer

A

Persistent proteinuria is significant + should be quantified by measuring the urine protein/creatinine ratio in an early morning sample
Protein should not exceed <20mg/mmol of creatinine

Question 2

Q

PROTEINURIA
What are some causes of proteinuria?

Answer

A

Transient (febrile illness, after exercise = no investigation)
Nephrotic syndrome
HTN
Tubular proteinuria
Increased glomerular perfusion pressure
Reduced renal mass

Question 3

Q

NEPHROTIC SYNDROME
What is nephrotic syndrome?
Who is it most common in?

Answer

A

Basement membrane in the glomerulus becomes highly permeable to protein, allowing proteins to leak from blood into the urine
2–5y

Question 4

Q

NEPHROTIC SYNDROME
What are the signs needed in order to make a diagnosis of nephrotic syndrome?

Answer

A

Heavy proteinuria (>1g/m^2/24h)
Hypoalbuminaemia (<25g/L)
hypercholesterolaemia

Question 5

Q

NEPHROTIC SYNDROME
What are the 3 main types of nephrotic syndrome?

Answer

A

minimal change disease
membranous glomerulonephritis
focal segmental glomerulosclerosis

Question 6

Q

NEPHROTIC SYNDROME
What is minimal change disease?

Answer

A

Most common cause in children with no underlying pathology

Question 7

Q

NEPHROTIC SYNDROME
What are the features of minimal change disease?

Answer

A

1–10y
No macroscopic haematuria
Normal BP, complement levels, renal function
Often precipitated by resp infections
1/3 resolve, 1/3 infrequent relapses, 1/3 frequent relapses

Question 8

Q

NEPHROTIC SYNDROME
what is the epidemiology of congenital nephrotic syndrome?

Answer

A

First 3m of life, rare, high mortality

Question 9

Q

NEPHROTIC SYNDROME
What is the clinical presentation of nephrotic syndrome?

Answer

A

Frothy urine (significant proteinuria)
Generalised oedema (pitting + gravitational), can be periorbital (esp on waking)
May have scrotal, vulval, leg + ankle oedema too
Pallor, breathlessness (pleural effusions) + abdo distension (ascites)

Question 10

Q

NEPHROTIC SYNDROME
What are some investigations for nephrotic syndrome?

Answer

A

Urinalysis (proteinuria + microscopic haematuria)
Urine MC&S (infection)
Renal function (U+Es, creatinine, albumin, urinary Na+ concentration)
Lipid profile
Systemic disease screen
Antistreptolysin O or anti-DNAse B titres + throat swab
Renal biopsy for histology if no steroid response

Question 11

Q

NEPHROTIC SYNDROME
In nephrotic syndrome, what are you looking for with the lipid profile?

Answer

A

Deranged (hyperlipidaemia, hypercholesterolaemia)

Question 12

Q

NEPHROTIC SYNDROME
What are some complications of nephrotic syndrome?

Answer

A

Hypovolaemia as fluid leaks from intravascular to interstitial space
Thrombosis due to loss of antithrombin III
Infection due to leakage of immunoglobulins, weakening the immune system + exacerbated by Tx with steroids

Question 13

Q

NEPHROTIC SYNDROME
What is the general management of nephrotic syndrome?

Answer

A

Strict fluid balance with restriction, no added salt
Tx hypovolaemia if present but albumin infusion is not routine
Diuretics if very oedematous + no evidence of hypovolaemia
Prophylactic PO penicillin V until oedema-free
PCV vaccine

Question 14

Q

NEPHRITIC SYNDROME
What is nephritic syndrome?

Answer

A

Acute nephritis is inflammation within the nephrons of the kidneys
This leads to reduction in kidney function, macroscopic haematuria + proteinuria

Question 15

Q

NEPHRITIC SYNDROME
What are some causes of nephritic syndrome?

Answer

A

Post-streptococcal glomerulonephritis
IgA nephropathy (Berger’s disease)
Vasculitis (HSP, SLE, Wegener’s, polyarteritis nodosa)
Goodpasture’s syndrome
Familial nephritis (Alport’s syndrome)

Question 16

Q

NEPHRITIC SYNDROME
What is post-streptococcal glomerulonephritis?

Answer

A

Nephritis after group A beta-haemolytic strep pyogenes illness (tonsillitis)

Question 17

Q

NEPHRITIC SYNDROME
How does familial nephritis (Alport’s syndrome) present?

Answer

A

X-linked recessive
ESRF by early adult
Associated with nerve deafness + ocular defects
Mother may have haematuria

Question 18

Q

NEPHRITIC SYNDROME
What is the clinical presentation of nephritic syndrome?

Answer

A

Haematuria (often macroscopic) + proteinuria of varying degree
Impaired GFR (rising creatinine), decreased urine output + volume overload
Salt + water retention > HTN (?seizures) + oedema (eyes)

Question 19

Q

NEPHRITIC SYNDROME
What are some investigations for nephritic syndrome?

Answer

A

Urinalysis = haematuria, raised protein, (PCR, RBC casts on microscopy)
FBC, U+Es = raised urea) raised creatinine + hyperkalaemic acidosis
C3/4 may be low (post-strep, SLE)
Antistreptolysin O titre (may be raised), throat/skin swabs for strep
Renal biopsy

Question 20

Q

NEPHRITIC SYNDROME
What is the general management of nephritic syndrome?

Answer

A

Fluid + electrolyte balance, monitor UO + creatinine
Treat HTN + oedema with antihypertensives ±diuretics (ACEi/ARB, furosemide or prednisolone)
Nephritis usually settles alone, may need steroids

Question 21

Q

NEPHRITIC SYNDROME
What is the management of post-strep glomerulonephritis?

Answer

A

Supportive, mostly full recovery (some have worsening renal function), penicillin if active infection

Question 22

Q

HSP
What is Henoch-Schönlein purpura (HSP)?

Answer

A

IgA mediated small vessel vasculitis leading to inflammation affecting the skin, joints, GI tract + kidneys

Question 23

Q

HSP
What is the clinical presentation of HSP?

Answer

A

Palpable purpuric rash affecting extensor surfaces of lower limbs + buttocks
Joint pain (knees + ankles, may be swollen + painful, reduced ROM)
Colicky abdo pain (GI haemorrhage > haematemesis + melaena, intussusception)
Renal involvement (IgA nephritis > haematuria + proteinuria)

Question 24

Q

HSP
What are some investigations for HSP?

Answer

A

Exclude DDx of non-blanching rash
– FBC + blood film (thrombocytopenia, sepsis + leukaemia), CRP, cultures, HSP = afebrile
Urinalysis for proteinuria + haematuria
PCR to quantify proteinuria
Renal biopsy if severe renal issues to determine if Tx

Question 25

Q

HSP
What might happen if proteinuria becomes severe in HSP?
What would you monitor?

Answer

A

Nephrotic syndrome
BP + serum albumin

Question 26

Q

HSP
What is the management of HSP?

Answer

A

Supportive = analgesia for arthralgia, inconsistent evidence for steroid use
Often good prognosis, self-limiting but 1/3 recur

Question 27

Q

HAEMOLYTIC URAEMIC SYNDROME
What is haemolytic uraemic syndrome (HUS)?

Answer

A

Thrombosis within small blood vessels throughout the body, usually triggered by a bacterial toxin (shiga)

Question 28

Q

HAEMOLYTIC URAEMIC SYNDROME
What is the classic HUS triad?

Answer

A

Microangiopathic haemolytic anaemia (due to RBC destruction)
AKI (kidneys fail to excrete waste products like urea)
Thrombocytopenia

Question 29

Q

HAEMOLYTIC URAEMIC SYNDROME
What are some causes of HUS?

Answer

A

Mostly E. Coli 0157 producing Shiga toxin, can be Shigella (?Petting zoo)
Use of Abx + antimotility agents to treat gastroenteritis caused by these pathogens can increase risk of HUS

Question 30

Q

HAEMOLYTIC URAEMIC SYNDROME
What is the clinical presentation of HUS?

Answer

A

Prodrome of bloody diarrhoea
Urine > reduced output, haematuria or dark brown
Abdo pain, lethargy
Oedema, HTN, bruising

Question 31

Q

HAEMOLYTIC URAEMIC SYNDROME
What are some investigations for HUS?

Answer

A

FBC (anaemia, thrombocytopenia), fragmented blood film
U+Es reveal AKI
Stool culture

Question 32

Q

HAEMOLYTIC URAEMIC SYNDROME
What is the management of HUS?

Answer

A

ABCDE as emergency
Often self-limiting so supportive > refer to paeds renal unit for ?dialysis
Anti-hypertensives, careful fluid balance, blood transfusions
Plasma exchange if severe + not associated with diarrhoea

Question 33

Q

HAEMATURIA
How can you differentiate the source of haematuria based on its presentation?

Answer

A

Glomerular = brown urine, deformed red cells, presence of casts, often with proteinuria
Lower urinary tract = red urine, occurs at beginning or end of stream, not accompanied by proteinuria

Question 34

Q

HAEMATURIA
What is the most common cause of haematuria?
What are the other 2 broad causes?

Answer

A

UTI
Glomerular or non-glomerular

Question 35

Q

HAEMATURIA
What are some glomerular causes of haematuria?

Answer

A

Acute/chronic glomerulonephritis,
IgA nephropathy,
familial nephritis,
post-strep glomerulonephritis,
HSP,
goodpasture’s

Question 36

Q

HAEMATURIA
What investigations for haematuria should all patients get?

Answer

A

Urinalysis + urine MC&S
FBC, platelets, clotting + sickle cell screen
U+Es, creatinine, albumin, Ca2+, phosphate
USS kidneys + urinary tract

Question 37

Q

HAEMATURIA
What investigations would you do if you suspected glomerular haematuria?

Answer

A

ESR, C3/4 + anti-DNA antibodies
Throat swab + antistreptolysin O/anti-DNAse B titres
Hepatitis B/C screen
Renal biopsy if recurrent haematuria, abnormal renal function/complement levels or significant persistent proteinuria

Question 38

Q

HYPOSPADIAS
What is hypospadias?

Answer

A

Urethral meatus is abnormally displaced posteriorly on the penis

Question 39

Q

HYPOSPADIAS
What is the clinical presentation of hypospadias?

Answer

A

Ventral urethral meatus
Hooded prepuce
Chordee (ventral or downwards curvature of the penis in more severe forms)
Usually identified during NIPE

Question 40

Q

HYPOSPADIAS
What is the management of hypospadias?

Answer

A

Do NOT circumcise as foreskin often needed for later reconstructive surgery
Refer to paediatric specialist urologist
Mild cases may not require any treatment
Surgery done <2y to correct position of meatus + straighten penis

Question 41

Q

UTI
What is a urinary tract infection (UTI)?

Answer

A

Growth of bacteria within the urinary tract (>10^5 single organism/ml)

Question 42

Q

UTI
When is a UTI classified as atypical?

Answer

A

Septicaemia
Poor urine flow
Non-E. Coli
Failure to respond

Question 43

Q

UTI
What are some causes of UTI?

Answer

A

# 1 = E. coli, often from bowel contamination
Proteus (M>F, predisposes to formation of phosphate stones in urine)
Pseudomonas (may indicates structural abnormality)

Question 44

Q

UTI
What is a complication of E. Coli which might make it difficult to treat?

Answer

A

Can become resistant to penicillin by producing beta-lactamase (ESBL E. Coli) which breakdown the beta lactam part of Abx making it ineffective

Question 45

Q

UTI
What are some risk factors for UTI?

Answer

A

Incomplete bladder emptying
Vesico-ureteric reflux
Structural abnormality (horseshoe kidney, ureteric strictures)
Inadequate toilet hygiene

Question 46

Q

UTI
What is the clinical presentation of UTI in infants?

Answer

A

Non-specific =
- fever,
- irritable,
- D+V,
- prolonged neonatal jaundice,
- failure to thrive,
- septicaemia,
- febrile convulsions (>6m)

Question 47

Q

UTI
What are some investigations for UTI?

Answer

A

Urinalysis for nitrites, leukocytes esterase
Urine sample MC&S = collection pads (babies), MSU clean catch, suprapubic aspiration from bladder under USS worse case

Question 48

Q

UTI
In terms of performing ultrasounds scans in UTI, what are the guidelines?

Answer

A

USS within 6w if 1st UTI + <6m but responds well to Tx within 48h or during illness if recurrent or atypical bacteria

Question 49

Q

UTI
What are some complications of UTI?

Answer

A

Recurrent kidney infections can cause renal scarring predisposing to HTN, chronic renal failure + even pregnancy complications later in life

Question 50

Q

UTI
What is the supportive management of UTI?

Answer

A

Good fluid intake, analgesia
Wipe front>back
Regular voiding + ensure complete bladder emptying

Question 51

Q

UTI
Admission criteria for UTI?

Answer

A

Admission if <3m, systemically unwell or significant risk factors

Question 52

Q

UTI
What is the management of UTI for >3m with upper UTI?

Answer

A

?Admission for IV, if not PO co-amoxiclav for 7–10d

Question 53

Q

UTI
What is a recurrent UTI?

Answer

A

≥2 UTIs with ≥1 with systemic Sx (or ≥3 without)

Question 54

Q

UT ABNORMALITIES
Name 6 urinary tract abnormalities

Answer

A

Renal agenesis
Multicystic dysplastic kidney
Polycystic kidney disease
Pelvic/horseshoe kidney
Posterior urethral valves
Prune-belly syndrome

Question 55

Q

UT ABNORMALITIES
What is renal agenesis?

Answer

A

Absence of both kidneys

Question 56

Q

UT ABNORMALITIES
What are some other consequences of oligohydramnios in renal agenesis?

Answer

A

Pulmonary hypoplasia > respiratory failure
Limb deformities such as severe talipes

Question 57

Q

UT ABNORMALITIES
What is multicystic dysplastic kidney?

Answer

A

Non-functioning structure with large fluid-filled cysts with no renal tissue or connection with bladder

Question 58

Q

UT ABNORMALITIES
What is the management of multicystic dysplastic kidney?
What is a complication?

Answer

A

Half involuted by 2y + nephrectomy only indicated if very large or HTN
No urine production so if bilateral > Potter syndrome

Question 59

Q

UT ABNORMALITIES
How does polycystic kidney disease differ from MDK?

Answer

A

Some renal function remained in polycystic kidney disease

Question 60

Q

UT ABNORMALITIES
How might autosomal recessive polycystic kidney disease present?

Answer

A

Antenatal USS or with abdo masses or renal failure
Neonates may develop Potter syndrome secondary to oligohydramnios

Question 61

Q

UT ABNORMALITIES
What is pelvic/horseshoe kidney?

Answer

A

Abnormal caudal migration when the lower poles are fused in the midline

Question 62

Q

UT ABNORMALITIES
What is posterior urethral valves?
What is a consequence?

Answer

A

Tissue at proximal end of urethra causes obstruction to urinary outflow, M>F
Back pressure into bladder, ureters + up to kidneys > hydronephrosis
Also prevents complete bladder emptying > risk of UTI

Question 63

Q

UT ABNORMALITIES
How can posterior urethral valves present in utero?
What is a complication of posterior urethral valves?

Answer

A

Oligohydramnios + potentially pulmonary hypoplasia
Risk of dysplastic kidneys, at its worse if bilateral could lead to potter syndrome

Question 64

Q

UT ABNORMALITIES
What is Prune-belly syndrome?

Answer

A

Absent musculature leading to large bladder + Dilated ureters (megacystis-megaureters) + cryptorchidism

Answer 65

A

Antenatal Dx + start prophylactic Abx to prevent UTI
Bilateral hydronephrosis and/or dilated lower urinary tract in a male
Unilateral hydronephrosis in male or any anomaly in female

Answer 66

A

Bilateral seen in bladder neck obstruction or posterior urethral valves
USS within 48h of birth to exclude posterior urethral valves
– Abnormal = MCUG + surgery if required (ablation during cystoscopy)
– Normal = stop Abx, repeat USS after 2-3m

Answer 67

A

Unilateral seen in pelviureteric or vesicoureteric junction obstruction
Abnormal = further investigations
Normal = stop Abx, repeat USS after 2-3m

Answer 68

A

Spectrum of potentially reversible, reduction in renal function
Rapid rise in creatinine + development of oliguria (<0.5ml/kg/h)

Answer 69

A

Pre-renal (most common cause in children)
Renal
Post-renal

Answer 70

A

Hypovolaemia = nephrotic syndrome, haemorrhage, sepsis, burns
Circulatory failure

Answer 71

A

Vascular = HUS, vasculitis, embolus)
Glomerular = glomerulonephritis
Interstitial = interstitial nephritis, pyelonephritis
Tubular = acute tubular necrosis

Answer 72

A

Obstruction = congenital like posterior urethral valve or acquired like blocked urinary catheter

Answer 73

A

FBC, U+Es (high urea), high creatinine, USS to identify if obstruction
Can have hyperkalaemia, hyperphosphataemia + metabolic acidosis

Answer 74

A

Maintain strict fluid balance (IV fluids if hypovolaemic, restrict if overload)
If failure of conservative Mx, severe electrolyte disturbances or acidosis then ?dialysis

Answer 75

A

Structural malformations (congenital dysplastic kidney)
Glomerulonephritis
Hereditary nephropathies
Systemic diseases

Answer 76

A

Failure to thrive, anorexia + vomiting
HTN, acute-on-chronic renal failure, anaemia
Bony deformities from renal osteodystrophy
Incidental proteinuria, polydipsia + polyuria

Answer 77

A

Monitor growth
FBC = anaemia due to reduced EPO
U+Es + electrolytes (Ca2+ low, phosphate high)

Answer 78

A

Diet + NG or gastrostomy feeding may be needed for normal growth
Phosphate restriction + activated vitamin D to prevent renal osteodystrophy
May need recombinant growth hormone
Recombinant erythropoietin to prevent anaemia
Dialysis + transplantation if in ESRF (GFR <15ml/min/1.73m^2)

Answer 79

A

retrograde flow of urine from the bladder into the upper urinary tract
it is usually congenital

Answer 80

A

graded using International Reflux Study grading system

Answer 81

A

micturating cystourethrogram: radiocontrast medium introduced to catheterised bladder, reflux is detected on voiding
indirect cystogram

Answer 82

A

aim is to prevent renal scarring

antibiotic prophylaxis
surgery - not commonly recommended

Answer 83

A

NSAIDs,
Hodgkin’s lymphoma,
infectious mononucleosis

Answer 84

A

M>F,
commoner in Asian children than Caucasians,
do not progress to renal failure

Answer 85

A

Immune complexes made up of streptococcal antigens, antibodies + complement proteins get stuck in glomeruli > inflammation

Answer 86

A

3-10y, M>F + peaks during winter, preceded by URTI or gastroenteritis

Answer 87

A

Meatus displayed anteriorly on top of the penis

Answer 88

A

Albuminuria so severe may need unilateral nephrectomy then dialysis for renal failure until renal transplant

Answer 89

A

Recessive inheritance with increased incidence in Finnish (UK = consanguinity)

Answer 90

A

FBC, CRP/ESR, complement (C3/4) levels, autoimmune screen, hep B/C screen, malaria if abroad

Answer 91

A

Minimal change disease shows normal glomeruli on light microscopy but fusion of podocytes + effacement of foot processes on electron

Answer 92

A

Wilm’s tumour,
trauma,
stones (esp if FHx),
sickle cell disease
other bleeding disorders

Answer 93

A

Upper tract infection involves the kidneys (pyelonephritis) + associated with fever, loin pain/tenderness
Lower tract infection involves bladder (cystitis) + low-grade fever with urinary Sx

Answer 94

A

USS within 6w in all children with recurrent UTIs
DMSA (dimercaptosuccinic acid) scan (renal scarring 4-6m)
Micturating cystourethrogram (<6m) if FHx of vesico-ureteric reflux, dilatation of ureter on USS, poor urinary flow (catheterise + inject contrast into bladder)

Answer 95

A

Loin/abdo (suprapubic) pain,
fever (± rigors),
febrile convulsions,
increased frequency,
dysuria,
haematuria,
recurrent enuresis,
offensive urine

Answer 96

A

ALL children <3m + fever get immediate IV cefuroxime + full septic screen (blood cultures, FBC, CRP lactate, LP etc)

Answer 97

A

Meropenem

Answer 98

A

3d PO trimethoprim, nitrofurantoin, amoxicillin or cephalosporin with follow-up if still unwell after 24-48h

Answer 99

A

Absence of both kidneys causes severe oligohydramnios + hence foetal compression from reduced foetal urine excretion - this can cause Potter syndrome (fatal)
- Low-set ears, beaked nose, prominent epicanthic folds + downward slant to eyes

Answer 100

A

Failure of union of ureteric bud with nephrogenic mesenchyme

Answer 101

A

AD = HTN, haematuria in childhood with renal failure in adulthood
AR = defect on chromosome 6 that encodes fibrocystin, protein for normal renal tubule development

Answer 102

A

Often liver involvement with portal + interlobular fibrosis

Answer 103

A

Abnormal position can predispose to infection or obstruction to urinary outflow

Answer 104

A

Inflammation – from immune cells (Ab’s, Ig’s - IgG), complement proteins, HTN, atherosclerosis, medications/immunisations, infection
Damage to podocytes – protein leakage (albumin, Ab’s)
Increased liver activity – to increase albumin, - Consequential increase in cholesterol + coagulation factors
Reduced oncotic pressure – oedema - Consequential blood volume decrease, RAAS stimulation, exacerbation

Answer 105

A

Pitting oedema - periorbital, ascites, peripheral

Answer 106

A

vesicoureteral reflux (VUR) = most common + most important
previous history of UTI
siblings with a history of UTI
female sex
indwelling urinary catheter
intact prepuce in boys
structural abnormalities of the kidneys and lower urinary tract

Answer 107

A

pyelonephritis occurs after faecal flora colonize the urethra and ascend into the bladder and kidney

Answer 108

A

E.coli = 80% of cases

Answer 109

A

25% have no clinical signs
50% present with only flank pain

toddlers = fever and irritability, poor feeding, lethargy, abdominal pain

older children = fever, vomiting, flank pain, dysuria, urgency, increased frequency

Answer 110

A

urine microscopy and culture
CT KUB with contrast

Answer 111

A

empirical antibiotics then targeted based on cultures
severe = hospitalisation and IV antibiotics

Answer 112

A

children <2yrs diagnosed with a UTI should have a renal USS

Answer 113

A

recurrence
renal scarring
hypertension

Answer 114

A

bedwetting during sleep

Answer 115

A

Primary nocturnal enuresis = never been consistently dry at night
Secondary nocturnal enuresis = previously been dry for >6 months
Monosymptomatic = only has symptoms at night
Non-monosymptomatic = daytime wetting symptoms as well as night time wetting

Answer 116

A

not waking to bladder signals
inadequate levels of vasopressin (ADH)
overactive bladder
constipation
UTIs
Family history
Anxiety/stress
poor bedtime routines

Answer 117

A

large volumes of urine passed at night
wet in the early part of the night
wet more than once per night

Answer 118

A

damp patches that occur at night also occur during the day
the volume of urine passed is variable
children often wake after wetting at night

Answer 119

A

physical examination (back, genitalia + lower limbs)
urinalysis + MS&C
bladder scan
uroflowmetry
ultrasound

Answer 120

A

motivation, support and patience
drinking enough
drinking regularly
stopping drinks before bed
avoid drinks that avoid the bladder
regular timed toileting
establish a bedtime routine
refrain from using nappies
avoid lifting child out of bed before they are awake
treat and prevent constipation

Answer 121

A

antibiotics for infection
laxatives for constipation
alarms
desmopressin
anticholinergic medications (oxybutynin + tolterodine) for detrusor relaxation

Answer 122

A

A genetic disorder which damages glomeruli resulting in gradual loss of kidney function and CKD

Answer 123

A

X-linked Alport syndrome (XLAS)
Autosomal recessive Alport syndrome (ARAS)
Autosomal dominant Alport syndrome (ADAS)

Answer 124

A

haematuria
oedema
hypertension
loss of kidney function
progressive hearing loss
proteinuria
vision problems

Answer 125

A

ACE inhibitors
dialysis
kidney transplant

Answer 126

A

genetic testing
tissue biopsy
urinalysis
hearing tests

Answer 127

A

Warm + dry baby ASAP by vigorous drying (may stimulate breathing)
Heat lamp
Babies <28w in plastic bag while still wet + manage under heat lamp

Answer 128

A

APGAR at 1, 5 + 10m
Stimulate breathing with vigorous drying
Place baby’s head in neutral position to keep airway open (towel under shoulder can help)

Answer 129

A

Inflation breaths if gasping or not breathing –

2 cycles of 5 inflation breaths
No response + HR low = 30s of ventilation breaths
No response, HR <60bpm = chest compressions (3:1 with ventilation breaths)

Answer 130

A

Danger = ensure safety
Unresponsive = shout for help
Open airway = head tilt + chin lift or jaw thrust
Look, listen + feel for breathing (noisy gasps do not count)

Answer 131

A

5 rescue breaths
Infants = neutral position, cover mouth + nose with whole mouth
> 1y = head tilt chin lift, pinch soft part of nose + seal mouths
Ensure chest rise/fall for effectiveness (if not ?obstruction or try jaw thrust)
Note any gag or cough response to actions as sign of life

Answer 132

A

Check circulation –

Infant = brachial or femoral
Child = femoral or carotid
If pulse felt = continue rescue breathing until child takes over

Answer 133

A

Chest compressions 15:2 rescue breaths
Depress sternum by one-third depth of chest
Rate of 100-120bpm

Answer 134

A

Infant = tips of two fingertips or encircle with thumbs
> 1y = heel of 1 hand on lower sternum
Larger = 2 hands interlocked as for adults

Answer 135

A

Loud, responsive, able to breathe, verbal
Encourage cough + continue to observe for deterioration or until obstruction relieved

Answer 136

A

Unable to vocalise/breathe, cyanosis, silent/quiet cough
Conscious = 5 back blows, 5 thrusts
Unconscious = open airway, 5 breaths, CPR

Answer 137

A

Chest thrusts for infant, abdominal if >1y
Infants head down prone for back blows, supine for thrusts
Back blows more effective if child’s head down

Answer 138

A

Apnoea,
RDS,
bronchopulmonary dysplasia,
infections

Answer 139

A

Unable to suck + swallow until 33–34w so will need NG
- Build feeds up slowly to reduce risk of NEC

Answer 140

A

Lack of glycogen stores

Answer 141

A

Inadequate surfactant > high surface tension within alveoli
Leads to atelectasis (lung collapse) as more difficult for alveoli + lungs to expand so there’s inadequate gas exchange > hypoxia, hypercapnia + respiratory distress

Answer 142

A

Prematurity #1
Maternal DM
2nd premature twin
C-section

Answer 143

A

Tachypnoea >60bpm
Increasing oxygen need
Laboured breathing = sternal + subcostal indrawing, nasal flaring, grunting
Cyanosis if severe

Answer 144

A

CXR –

Reticular “ground-glass” changes
Heart borders indistinct
Air bronchograms

Answer 145

A

Short = pneumothorax, infection, apnoea, necrotising enterocolitis
Long = bronchopulmonary dysplasia, retinopathy of prematurity

Answer 146

A

Antenatal dexamethasone
Increases surfactant production

Answer 147

A

Assisted ventilation by CPAP keeping lungs inflated or intubation if severe
Endotracheal surfactant via endotracheal tube
Supplementary oxygen for SpO2 91–95%
Breathing support gradually stepped down as baby develops

Answer 148

A

Disorder affecting premature neonates where part of bowel becomes necrotic
Associated with bacterial invasion of ischaemic bowel wall

Answer 149

A

Very LBW + premature
Formula feeds (breast milk protective)
RDS + assisted ventilation
Sepsis
PDA + other CHD

Answer 150

A

Bilious vomiting
Intolerance to feeds
Distended, tender abdo with absent bowel sounds
Bloody stools

Answer 151

A

Blood culture (sepsis)
CRP
Capillary blood gas = metabolic acidosis
AXR is diagnostic

Answer 152

A

Dilated loops of bowel
Bowel wall oedema (thickened bowel walls)
Pneumatosis intestinalis (intramural gas)
Pneumoperitoneum (free gas in peritoneum = perf)
Football sign = air outlining falciform ligament
Rigler’s sign = air both inside/outside bowel wall
Gas in portal veins

Answer 153

A

Dead bowel > perforation + peritonitis > sepsis + shock
Stricture formation
Short bowel syndrome (malabsorption) if extensive resection required

Answer 154

A

A–E if shocked, ?artificial ventilation, ?circulatory support
Broad spec Abx 1st, NBM with IV fluids + total parenteral nutrition (NG to drain gas + fluid from stomach + intestines)
Surgical emergency > laparotomy for perforation

Answer 155

A

Abnormally high levels of bilirubin in the blood

Answer 156

A

LBW
Breastfeeding
Prematurity
FHx
Maternal diabetes

Answer 157

A

<24h = always pathological, usually haemolytic disease
24h–2w = common
> 2w = also bad

Answer 158

A

Haemolytic diseases #1 = rhesus or ABO incompatibility, G6PD, spherocytosis
Congenital infection (TORCH), sepsis

Answer 159

A

Physiological + breast milk jaundice (common)
Infection (UTI, sepsis)
Haemolysis, polycythaemia, bruising
Crigler-Najjar syndrome (rare inherited disorder with no UGT enzyme)

Answer 160

A

Unconjugated = physiological or breast milk, UTI, hypothyroid, high GI obstruction (pyloric stenosis), Gilbert syndrome
Conjugated (>25umol/L) = bile duct obstruction (biliary atresia), neonatal hepatitis

Answer 161

A

Yellow skin/sclera (may be more visible when outside in sunlight)
Persistent or prolonged jaundice worrying (>2w full term, >3w preterm)

Answer 162

A

High concentration of RBCs in neonate which are more fragile with shorter life
Less developed liver
Foetal RBCs breakdown more rapidly releasing lots of bilirubin > normal rise in bilirubin = mild jaundice from 2–7d

Answer 163

A

Only when all other causes excluded
Usually completely resolves by 10d, most babies otherwise healthy

Answer 164

A

Components of breast milk inhibiting liver to process bilirubin
Increased bilirubin absorption
Inadequate feeds > slow passage of stools

Answer 165

A

AR deficiency of UDP-glucuronyltransferase = defective bilirubin conjugation
Unconjugated hyperbilirubinaemia (not in urine), jaundice may only be present if ill, exercising or fasting

Answer 166

A

FBC + blood film (polycythaemia, G6PD, spherocytosis)
Bilirubin levels
Blood type testing of mother + baby for ABO/Rh incompatibility
Direct Coombs (antiglobulin) test for haemolysis
TFTs, LFTs + urine MC&S

Answer 167

A

Split bilirubin = unconjugated (extra-hepatic) or conjugated (hepatobiliary)
> 24h old = transcutaneous bilirubin meter if high, serum to confirm within 6h
<24h old = serum bilirubin within 2h

Answer 168

A

Kernicterus
Bilirubin-induced encephalopathy caused by unconjugated bilirubin deposition in brain (basal ganglia + brainstem nuclei) as baby’s BBB are not well developed

Answer 169

A

Prematurity as immature liver

Answer 170

A

Bilirubin Tx threshold charts, plot age of baby against total bilirubin level + treat once at threshold
Phototherapy (450mm wavelength blue-green band)
Exchange transfusion if severe

Answer 171

A

Converts unconjugated bilirubin > water-soluble pigment that can be excreted in urine, cover infant’s eyes

Answer 172

A

In perinatal asphyxia, gas exchange, either placental or pulmonary is compromised or ceases resulting in cardiorespiratory depression

Answer 173

A

Hypoxia, hypercarbia + metabolic acidosis
Compromised cardiac output reduces tissue perfusion > hypoxic ischaemic injury to brain

Answer 174

A

Anything leading to asphyxia =
- maternal shock,
- intrapartum haemorrhage,
- prolapsed or nuchal cord,
- placental abruption

Answer 175

A

Sarnat staging –

Mild = poor feeding, generally irritable + hyperalert, resolves in 24h
Moderate = poor feeding, lethargic, hypotonic, seizures, can take weeks to resolve
Severe = reduced GCS, apnoeas, flaccid + reduced/absent reflexes, half die

Answer 176

A

Permanent brain damage > cerebral palsy
Moderate = 40%,
severe = 90%

Answer 177

A

MDT resus –

Dry baby, APGAR, resp support
Treat seizures, EEG
Treat hypotension by volume + inotropes
Monitor + treat electrolytes

Answer 178

A

Therapeutic hypothermia to protect brain from hypoxic injury
Cooled to PR temp 33–34 for 72h to reduce brain damage

Answer 179

A

No agreed definition but <2.6mmol/L often used

Answer 180

A

Jitteriness, irritability, apnoea
Lethargy, drowsiness + Seizures
Long-term may cause permanent neuro disability

Answer 181

A

Regular bedside BM
Prevent by early + frequent feeding
IVI 10% dextrose (central venous catheter if higher concentration of dextrose to prevent skin necrosis) to maintain glucose >2.6mmol/L

Answer 182

A

Main congenital conditions
- Toxoplasmosis,
- Other (HIV),
- Rubella,
- CMV,
- Herpes + Syphilis

Answer 183

A

Cerebral calcification, chorioretinitis + hydrocephalus

Answer 184

A

Most common congenital infection
Herpes simplex virus via personal contact

Answer 185

A

90% normal at birth
5% = hepatosplenomegaly, petechiae at birth, growth issues, neurodevelopmental disabilities (cerebral palsy, epilepsy, microcephaly)
5% = problems later in life, mainly sensorineural hearing loss

Answer 186

A

Herpetic lesions on skin or eye, encephalitis or disseminated disease

Answer 187

A

Rash on soles of feet + hands
Hutchinson’s triad = keratitis, deafness, small + pointed teeth

Answer 188

A

Meconium may be passed due to foetal hypoxia + at birth these infants may inhale it
Lung irritant resulting in mechanical obstruction + chemical pneumonitis + predisposing to infection

Answer 189

A

Post-term deliveries at 42w
Maternal HTN or pre-eclampsia
Smoking or substance abuse
Chorioamnionitis

Answer 190

A

Presence of meconium or dark green staining of amniotic fluid
Respiratory distress

Answer 191

A

CXR = hyperinflation, accompanied by patches of collapse + consolidation
High incidence of air leak > pneumothorax

Answer 192

A

Persistent pulmonary HTN of the newborn due to high pulmonary vascular resistance
RDS, sepsis, congenital diaphragmatic hernia, maternal SSRI use, maternal NSAID use in 3rd trimester (early closure of DA)

Answer 193

A

Artificial (positive pressure) ventilation with oxygenation
Suction if no breathing

Answer 194

A

Split or open section in upper lip, can go up to the nose

Answer 195

A

Defect in hard or soft palate at roof of mouth which leaves an opening between the mouth + nasal cavity

Answer 196

A

Chromosomal disorder or maternal AED therapy

Answer 197

A

MDT = plastic + ENT surgeons, paeds, orthodontist, SALT
Cleft lip repair ≤3m
Cleft palate repair 6-12m

Answer 198

A

Upper + lower oesophagus in 2 sections + does not connect

Answer 199

A

Persistent salivation + drooling from mouth after birth
May cough + choke when fed + have cyanotic aspiration
Some have other congenital malformations (VACTERL association)

Answer 200

A

Wide calibre feeding tube passed + checked by XR if reaches stomach
Continuous suction applied to tube passed into oesophageal pouch to reduce aspiration of saliva + secretions > neonatal surgical unit

Answer 201

A

Bowel protrudes through congenital defect in anterior abdominal wall, adjacent to umbilicus but with no covering sac

Answer 202

A

Socioeconomic deprivation (smoking, mum <20y)

Answer 203

A

Higher risk of dehydration + protein loss –

Wrap infants in several layers of clingfilm to minimise fluid + heat loss
NG tube passed + aspirated frequently
IVI dextrose + colloid support for protein loss

Answer 204

A

May attempt vaginal delivery
Urgent repair (theatre within 4h)

Answer 205

A

Premature babies often <28w diagnosed when infant requires oxygen therapy after they reach 36w gestation

Answer 206

A

Reduced lung volume + reduced alveolar surface area > diffusion defect

Answer 207

A

Increased work of breathing (tachypnoea, nasal flaring, recessions, low SpO2)
Crackles + wheezes on auscultation
Poor feeding + weight gain
Increased susceptibility to infection

Answer 208

A

CXR = widespread areas of opacification, cystic changes, fibrosis
Formal sleep study to assess SpO2 during sleep supports Dx + guides Mx

Answer 209

A

Corticosteroids to mothers in premature labour <34w
CPAP rather than intubation where possible
Use caffeine to stimulate resp effort
Do not over oxygenate

Answer 210

A

Some babies go home with low dose oxygen, weaned over first year
Monthly IM palivizumab for RSV (+ bronchiolitis) protection

Answer 211

A

Congenital absence or complete closure of duodenum This causes intestinal obstruction

Answer 212

A

Abdominal contents protrude through umbilical ring, covered with a transparent sac formed by the amniotic membrane + peritoneum

Answer 213

A

Other major congenital abnormalities, antenatal Dx

Answer 214

A

AXR shows ‘double bubble’ from distension of stomach + duodenal cap

Answer 215

A

Third have Down’s

Answer 216

A

Correct fluid + electrolyte depletion
surgical management is required to remove the narrowed part of bowel and reattach the ends.

Answer 217

A

most appear well at birth
when they atart to feed they are sick (vomit is green)
jaundice
not pass meconium in first day

Answer 218

A

it can be passed on from the mother during pregnancy
it can be passed from the mother’s genital tract during birth

Answer 219

A

preterm labour
premature rupture of membranes
a long time between rupture of membranes and birth
internal foetal monitor
fever
past pregnancy with baby who had strep B
african-american/hispanic
group B strep in urine during pregnancy

Answer 220

A

being fussy, sleepy + having breathing problems (signs of sepsis)
breathing fast + making grunting noises (signs of pneumonia)
breathing problems + periods not breathing
change in BP
convulsions

Answer 221

A

decreased movement in arm or leg
pain with movement of arm or leg
breathing problems
fever
red area on face or other part of the body

Answer 222

A

having to urinate often
having a sudden urge to urinate
pain when urinating
fever
nausea and vomiting
pain in side or back
uterus or belly is sore
fast heart rate

Answer 223

A

blood cultures
lumbar puncture
sputum culture

Answer 224

A

IV antibiotics
NICU admission

Answer 225

A

chorioamnionitis - infection of the amniotic fluid, sac and placenta
endometritis - postpartum infection
preterm labour

Answer 226

A

meningitis
pneumonia
sepsis

Answer 227

A

test for group B strep at 35-37 weeks of pregnancy (vaginal + rectal swab, urine sample)
if test is positive, have IV antibiotics during labour
may be given antibiotics for certain risk factors
- previous pregnancy with strep B infection
- premature rupture of membranes/premature labour
- fever during labour
- rupture of membranes >18hrs before delivery

Answer 228

A

It can be acquired in the womb or during/after delivery
pregnant women can become infected by eating contaminated food - soft cheese, seafood, unpasteurised dairy etc

Answer 229

A

Necrotising enterocolitis,
neonatal jaundice,
feeding issues

Answer 230

A

Cerebral palsy,
hearing/visual impairment,
intraventricular haemorrhage

Answer 231

A

Hypoglycaemia,
hypocalcaemia,
electrolyte imbalance,
fluid imbalance
hypothermia

Answer 232

A

Kidneys + parathyroid not fully developed

Answer 233

A

Excess losses through skin

Answer 234

A

RBCs contain unconjugated bilirubin, they breakdown + release it into blood, conjugated in liver + excreted via biliary system (GI tract) or urine

Answer 235

A

Lethargy, poor feeding > hypertonia, seizures + coma
Permanent damage = dyskinetic cerebral palsy, LD + deafness

Answer 236

A

Temp instability,
macular rash,
bronze discolouration

Answer 237

A

Preterm + intrauterine growth restriction (IUGR) = lack of glycogen stores
Maternal DM = infantile hyperinsulinaemia
LGA, polycythaemia or ill
Transient hypoglycaemia common in first hours after birth

Answer 238

A

Aciclovir, high mortality in disseminated

Answer 239

A

No therapy so no screening

Answer 240

A

If fully treated ≥1m before delivery = no treatment
Any doubts = benzylpenicillin

Answer 241

A

Failure of fusion of the frontonasal + maxillary processes

Answer 242

A

Failure of the palatine processes + nasal septum to fuse

Answer 243

A

Issues feeding, milk aspiration, speech delay + conductive hearing loss, recurrent otitis media (cleft palate)

Answer 244

A

Tracheo-oesophageal fistula + polyhydramnios

Answer 245

A

USS shows free loops of bowel in amniotic fluid antenatally

Answer 246

A

Suffer with RDS, need oxygen therapy or ventilation + intubation at birth

Answer 247

A

C-section at 37w, staged repair as primary closure difficult

Answer 248

A

symptoms are similar to sepsis - listlessness, irritable, poor feeding
- Early onset = low birth weight, obstetric complications, evidence of sepsis soon after birth
- late onset = usually full-term, previously healthy neonates, present with meningitis/sepsis

Answer 249

A

10-50% of newborns with listeria infection die
the death rate is higher in those with early onset listeriosis

Answer 250

A

pregnant women should avoid eating unpasteurised dairy, soft cheeses, raw veg, deli meats, meat spreads and smoked seafood

Answer 251

A

ampicillin + aminoglycoside (gentamycin)

Answer 252

A

NICE traffic light system for <5
Colour (skin, lips, tongue)
Activity
Respiratory
Circulation + hydration
Other

Answer 253

A

i) Pallor
ii) No smile, decreased activity, not responding to social cues, wakes when roused
iii) Nasal flaring, SpO2 ≤95%, crackles in chest RR>50 (6-12m) or >40 (>12m)
iv) Tachy (>160 if <1y, >150 if 1–2y, >140 if 2–5y), CRT ≥3s, dry mucous membranes, reduced urine output
v) 3-6m temp ≥39, fever ≥5d, rigors, joint swelling, non-weight bearing

Answer 254

A

i) Mottled skin
ii) No response to cues, doesn’t wake if roused, weak, high-pitched or constant cry
iii) Grunting, RR>60, mod-severe chest indrawing
iv) Reduced skin turgor, no urine output
v) <3m temp ≥38, non-blanching rash, bulging fontanelle, neck stiffness, status, focal seizures/neuro

Answer 255

A

Common = URTI, tonsillitis, otitis media, UTI
Uncommon = Meningitis, epiglottitis, kawasaki disease, TB

Answer 256

A

Manage at home with safety netting
Regular fluids, monitor child, contact if concerned

Answer 257

A

Clear verbal ± written advice about warning signs with plan of action
Follow up if required
Liaise with other HCPs so direct access if child needs

Answer 258

A

F2F assessment with paeds or specialist for further investigation
?Home with safety net

Answer 259

A

Urgent referral to hospital for specialist assessment (?999)

Answer 260

A

Primary infection by Varicella zoster virus (human herpes virus 3)

Answer 261

A

Immunocompromised
Older age
Steroids
Malignancy
Neonates

Answer 262

A

Prodromal high fever 38-39 often ceases when rash appears, malaise
Very itchy, vesicular rash starts on head + trunk > peripheries
Not infective once vesicles have crusted over (5d usually)

Answer 263

A

Secondary bacterial infection
Shingles (older children)
Ramsay Hunt syndrome (older children)
Risk to immunocompromised, neonates + pregnant women
Rarer = pneumonia, encephalitis

Answer 264

A

Small area of cellulitis or erythema, persistent fever
Small risk staph/group A strep infection > necrotising fasciitis
NSAIDs may increase risk, Rx with Abx (IV if severe or dehydrated)

Answer 265

A

Reactivation of dormant virus > herpes zoster virus (shingles) in dorsal root ganglia

Answer 266

A

Herpes zoster oticus > reactivation of varicella zoster virus in geniculate ganglion of CN7

Answer 267

A

i) Disseminated disease, DIC, pneumonitis (VZIG if exposed to case)
ii) Mother develops shortly before/after delivery infant > VZIG + aciclovir
iii) Risk of foetal varicella syndrome if <20w

Answer 268

A

Camomile lotion to stop itching
Avoid high risk groups
Trim nails
School exclusion until all lesions crusted over (usually 5d after rash)

Answer 269

A

Inflammation of the meninges which line the brain + spinal cord

Answer 270

A

Neonates = GBS or listeria monocytogenes
1m–6y = N. meningitidis (gram -ve diplococci), S. pneumoniae (gram + ve cocci chain), H. influenzae
> 6y = meningococcus + pneumococcus, rarely TB

Answer 271

A

Herpes simplex virus (HSV), enteroviruses, EBV + varicella zoster virus
Aseptic/sterile by malignancy or autoimmune diseases

Answer 272

A

Fever, headache, vomiting, drowsiness, poor feeding, irritable/lethargic
Later may have seizures, focal neurology, decreased GCS/coma
Neonates may have hypothermia, lethargy + hypotonia

Answer 273

A

Meningism = neck stiffness (not always present), photophobia
Bulging fontanelle, opisthotonos, signs of shock
+ve Kernig’s + Brudzinski
Non-blanching petechial/purpuric rash = later sign in meningococcal septicaemia (endotoxin causes DIC + subcut haemorrhages)

Answer 274

A

Kernig = pain/unable to extend leg at knee when it’s bent
Brudzinski = involuntary flexion of hips/knees when neck flexed

Answer 275

A

Blood cultures + serology (before LP + Abx unless undesirable delay)
FBC, U+E, LFTs, CRP, blood glucose
LP for MC&S with protein, cell count, glucose + viral PCR
?CT head if other signs like papilloedema

Answer 276

A

Signs of increased ICP, focal neurology, local infection, unduly delay starting Abx or coagulopathies
Coning of cerebellar tonsils via foramen magnum

Answer 277

A

i) Cloudy/turbid
ii) ++ (make protein)
iii) –– (eat glucose)
iv) ++ neutrophil polymorphs
v) Gram stain

Answer 278

A

i) Clear
ii) Normal/+
iii) Normal/-
iv) + lymphocytes
v) PCR

Answer 279

A

i) Turbid/viscous
ii) +++
iii) –––
iv) + lymphocytes
v) Acid fast bacilli

Answer 280

A

Hearing (sensorineural) loss is key complication
Seizures + epilepsy, cerebral abscess, encephalitis + hydrocephalus
Cognitive impairment, cerebral palsy + LD

Answer 281

A

Supportive = correct shock with fluids, oxygen if needed
<3m = IV cefotaxime + amoxicillin (cover listeria from ?pregnancy)
> 3m = IV ceftriaxone + IV dexamethasone to reduce frequency + severity of hearing loss + neuro damage (NOT before 3m)

Answer 282

A

Milder so supportive + aciclovir if HSV or VSZ

Answer 283

A

IM benzylpenicillin

Answer 284

A

Single dose ciprofloxacin or rifampicin
Ciprofloxacin is prefered as can use for any age, pregnant ladies + does not interfere with OCP

Answer 285

A

Do not give in myasthenia gravis or previous sensitivity,
can cause tendinitis
can trigger seizures

Answer 286

A

Meningitis B vaccine at 8w, 16w + 1y (men C at 1y too) and ACWY offered to teenagers + uni students
Bacterial meningitis + meningococcal = notifiable diseases

Answer 287

A

Inflammation of the brain parenchyma

Answer 288

A

Similar to meningitis = fever, headache, photophobia, neck stiffness
KEY difference = altered mental state (behavioural change, confusion)
Acute onset focal neurology (hemiparesis, dysphasia, focal seizures)

Answer 289

A

FBC, U+Es, blood cultures + serology for viral PCR
LP for MC&S with protein, cell count, glucose + viral PCR
CT/MRI head to visualise brain as ?focal changes, particularly temporal lobes

Answer 290

A

i) Clear
ii) Normal/+
iii) Normal/–
iv) + lymphocytes

Answer 291

A

IV aciclovir to cover HSV, Abx in case bacterial meningitis
Supportive therapy in HDU/ICU if needed

Answer 292

A

Bacteria proliferates into bloodstream as host response includes release of inflammatory cytokines + activation of endothelial cells which can lead to septic shock

Answer 293

A

Most common = N. meningitidis
Neonates = GBS or gram -ve organisms from birth canal

Answer 294

A

Fever, poor feeding, irritable/lethargic, Hx of focal infection
Fever, purpuric non-blanching rash, multi-organ failure

Answer 295

A

Tachycardia + tachypnoea
Cold peripheries
Capillary refill >2s
Hypotensive
Oliguria

Answer 296

A

Septic screen (FBC, U+Es, blood cultures, urine MC&S, LP/CSF, CXR, acute phase reactant like CRP)
Aggressive fluid resus, ?ICU
Broad-spec Abx until cultures back

Answer 297

A

Idiopathic medium-sized vessel systemic vasculitis, mainly affects 6m–5y
More common in children of Japanese or Afro-Caribbean ethnicity

Answer 298

A

Fever + 4 (MyHEART) –

Mucosal involvement (red/dry cracked lips, strawberry tongue)
Hands + feet (erythema then desquamation)
Eyes (bilateral conjunctival injection, non-purulent)
lymphAdenopathy (unilateral cervical >1.5cm)
Rash (polymorphic involving extremities, trunk + perineal regions
Temp >39 for >5d

Answer 299

A

Acute (1–2w) = child most unwell, fever, rash, lymphadenopathy
Subacute (2–4w) = acute Sx settle, desquamation + Risk of coronary artery aneurysms
Convalescent (2–4w) = remaining Sx settle, blood markers normalise slowly

Answer 300

A

Coronary artery aneurysm + sudden death

Answer 301

A

Prompt IVIg to reduce risk of aneurysm + aspirin to reduce risk of thrombosis
If fever persists = infliximab, steroids or ciclosporin

Answer 302

A

Aspirin normally contraindicated in children due to risk of Reye’s syndrome (swelling of the liver + brain)
50% evidence of cardiac impairment + mild MR, long-term follow up

Answer 303

A

Infection with measles virus (Morbillivirus) via droplets (highly contagious)

Answer 304

A

Prodromal Sx for 3–5d (CCCK) – Cough, Coryza, Conjunctivitis, Koplik spots
Maculopapular rash starts on forehead, neck + behind ears > down to limb, trunk
Fever, marked malaise

Answer 305

A

White spots on buccal mucosa = pathognomonic

Answer 306

A

Otitis media (commonest complication)
Pneumonia (commonest cause of death)
Diarrhoea
Febrile convulsions, encephalitis
Subacute sclerosing panencephalitis rare where 5-10y after primary measles > loss of neuro function, dementia + death

Answer 307

A

Notifiable disease
Best treatment is prevention with MMR vaccine
Viral illness so supportive (fluids, isolate if in hospital)
Antivirals in immunocompromised
School exclusion for 4d from rash onset

Answer 308

A

Mild notifiable disease occurring in winter + spring
Spreads via respiratory route, often from known contact, prevention via vaccine

Answer 309

A

Mild prodrome (low-grade fever, sore throat, coryza)
Pink maculopapular rash starts on face then spreads down to cover whole body
Rash not itchy in children but is in adults
Suboccipital + postauricular lymphadenopathy

Answer 310

A

Clinical Dx
Serological confirmation if any risk of exposure of a non-immune pregnant woman

Answer 311

A

Rare but > encephalitis, arthritis, myocarditis + thrombocytopenia
Congenital rubella syndrome > cataracts, CHD + sensorineural deafness
Avoid pregnant women, school exclusion 4d from rash, ensure vaccinated

Answer 312

A

RNA paramyxovirus, occurs in winter + spring, spreads via resp droplets where virus replicates in epithelial cells
Virus accesses parotid glands before further dissemination

Answer 313

A

Fever, malaise + parotitis
Parotitis often unilateral initially then bilateral > uncomfortable + may have earache or pain when eating/drinking
May have hearing loss but often unilateral + transient

Answer 314

A

Viral meningitis + encephalitis
Orchitis (usually unilateral, may reduce sperm count + lead to infertility)
Pancreatitis

Answer 315

A

Notifiable disease
Prophylaxis via vaccine
Clinical Dx, manage Sx as viral
School exclusion for 5d of onset of parotid swelling

Answer 316

A

Caused by coxsackie A16 virus

Answer 317

A

Subsides within few days, supportive with fluids, analgesia
Very contagious, avoid sharing towels + bedding, good handwashing
Only exclude from school if unwell

Answer 318

A

Epstein-Barr virus (EBV), particular tropism for B lymphocytes + epithelial cells of pharynx

Answer 319

A

Triad of severe sore throat (tonsillopharyngitis can limit oral intake), lymphadenopathy (cervical) + pyrexia
May have petechiae on soft palate, splenomegaly + headache

Answer 320

A

FBC (lymphocytosis)
positive Monospot test with heterophile antibodies

Answer 321

A

Conservative (fluids, analgesia)
Avoid alcohol + contact sports for 8w after to reduce risk of splenic rupture
Avoid amoxicillin as can cause florid, pruritic maculopapular rash

Answer 322

A

Reaction to strep pyogenes (group A beta haemolytic) toxin - strep A

Answer 323

A

Prodrome = sore throat, fever, vomiting + abdo pain
Red-pink diffuse rash that is ‘rough sandpaper-like’ + ‘pinhead’, starts on trunk + spreads outwards
May have exudative tonsils + strawberry tongue
Tender cervical lymphadenopathy

Answer 324

A

Throat swab (but start Abx)

Answer 325

A

Notifiable disease
Phenoxymethylpenicillin for 10d to prevent rheumatic fever
Supportive (fluids, pain relief)
School exclusion until 24h after Abx

Answer 326

A

Caused by parvovirus B19, outbreaks common during spring months

Answer 327

A

Prodromal Sx = fever, malaise, headache, myalgia
Followed by classic rose-red rash on face week later (slapped-cheek)
Progresses to maculopapular, ‘lace-like’ rash on trunk + limbs

Answer 328

A

Aplastic crisis (most serious) more common in chronic haemolytic anaemias like sickle cell, thalassaemia + in immunocompromised
Vertical transmission can lead to foetal hydrops + death due to severe anaemia

Answer 329

A

Direct invasion of bacteria = abscess, cellulitis, impetigo
Toxin-mediated (indirect) = toxic shock, food poisoning
Toxin-mediated (direct) = SSS

Answer 330

A

Infections of hair follicles or sweat glands by s. aureus
Systemic Abx + occasionally surgery

Answer 331

A

Localised, highly contagious infection by S. aureus or Strep pyogenes

Answer 332

A

Non-bullous + bullous

Answer 333

A

Pustules or vesicles typically around nose or mouth
Exudate dries > golden crust, itchy but usually not unwell

Answer 334

A

Epidermolytic toxins breakdown proteins that hold skin cells together > fluid-filled vesicles (bullae)
Rupture + fluid exudation > classic golden/honey crusted lesions
More common in neonates or <2y, commonly systemically unwell

Answer 335

A

Risk of SSSS
Post-strep glomerulonephritis

Answer 336

A

Swab vesicles, avoid sharing towels, cutlery, try not to scratch
Hydrogen peroxide 1% cream (or mupirocin)
PO flucloxacillin if severe + systemically unwell
School exclusion until lesions crusted + healed or 48h after Abx

Answer 337

A

Caused by type of S. aureus that produces epidermolytic toxins that breakdown proteins that hold skin together

Answer 338

A

Starts as generalised patches of erythema on the skin, skin looks thin + wrinkled
Bullae formation which burst + leave very sore, erythematous skin below (like a burn/scald)
Nikolsky sign = gentle rubbing causes peeling
Systemic Sx = fever, lethargy, dehydration > sepsis

Answer 339

A

Most need admission for IV flucloxacillin, fluid balance + analgesia

Answer 340

A

Toxin producing S. aureus + group A strep released from infection

Answer 341

A

Fever ≥39
Hypotension (shock)
Diffuse erythematous rash
Desquamation of rash (esp. palms + soles) 1-2w after
Multi-organ dysfunction

Answer 342

A

GI = D+V
CNS = confusion
Thrombocytopenia
Renal failure
Hepatitis
Clotting abnormalities

Answer 343

A

Severe subcutaneous infection with severe pain + systemic illness

Answer 344

A

Acute onset, painful erythematous lesion
Necrotic skin affecting all skin layers down to fascia + muscle

Answer 345

A

IV Abx (flucloxacillin) PLUS surgical debridement
?ICU, ?IVIg

Answer 346

A

HSV1 = lip + skin lesion,
HSV2 = genital lesions

Answer 347

A

Gingivostomatitis
Cold sores on lip
Eczema herpeticum
Herpetic whitlows
Eyes = blepharitis or conjunctivitis
CNS = aseptic meningitis, encephalitis

Answer 348

A

Vesicular lesions on lips, gums, tongue which can lead to painful ulceration + bleeding
High fever, miserable child, oral intake may hurt

Answer 349

A

Widespread vesicular lesions with pus developing on eczematous skin
Fever, lethargy, lymphadenopathy

Answer 350

A

Painful pustules on site of broken skin on fingers
Infected adult kissing a child’s finger

Answer 351

A

Mainly vertical
Rarely = sexual abuse, needles

Answer 352

A

Mild = lymphadenopathy or parotitis
Mod = recurrent bacterial infections, candidiasis, recurrent diarrhoea
Severe = pneumocystis jiroveci, severe failure to thrive, encephalopathy

Answer 353

A

Persistent lymphadenopathy
Hepatosplenomegaly
Recurrent fever
Parotitis
Serious, persistent, unusual, recurrent (SPUR) infections

Answer 354

A

<18m cannot use antibody (transplacental HIV IgG if exposed anyway)
2x HIV DNA PCR blood test (double negative to exclude) for viral load
– Within first 3m + at least 2w after completion of postnatal antiretroviral

Answer 355

A

Antiretrovirals based on viral load + CD4 count
Co-trimoxazole prophylaxis (PCP)
?Additional vaccines but not BCG as live
Regular follow up, check development, psychological support
Safe sex education when older

Answer 356

A

Lung lesion + (mediastinal) lymph nodes = Ghon or primary complex
Primary infection > caseating granulomas followed by period of dormancy with ?reactivation (secondary TB)
If immune system unable to cope it disseminates > miliary TB

Answer 357

A

Pleura,
CNS,
pericardium,
lymph nodes,
GI/GU tract

Answer 358

A

Prolonged fever
Haemoptysis
Cough
Malaise
Anorexia
Weight loss

Answer 359

A

Mantoux ‘tuberculin’ test
Interferon gamma release assays
3x samples of sputum MC&S = gold standard
CXR

Answer 360

A

> 15mm suggests active TB,
6-15mm ?previous exposure (may be BCG)

Answer 361

A

Pleural + pericardial effusions
Lung collapse
Lung consolidation

Answer 362

A

BCG for high risk neonates (FHx, relatives from countries with high TB rate)
Contact tracing
Notifiable to PHE

Answer 363

A

RIPE
- Rifampicin (6m)
- Isoniazid (6m)
- Pyrazinamide (2m)
- Ethambutol (2m)

Answer 364

A

Red urine

Answer 365

A

Isoniazid (+ vit B6) for 6m
Isoniazid (+vit B6) + rifampicin for 3m

Answer 366

A

Induce T + B cell (antibody) immunity
Induce immunological memory
Herd immunity to protect those who haven’t been immunised

Answer 367

A

Not adjusted for prematurity, give chronologically
Babies born <28w should receive first set in hospital due to risk of apnoea

Answer 368

A

Active = give part of pathogen either non-living or attenuated (live but weak)
Passive = give them antibodies to pathogen (natural = cross-placental transfer, artificial = treated with human IgG)

Answer 369

A

MMR, BCG, nasal flu, rotavirus + Men B

Answer 370

A

i) 6-in-one, rotavirus + men B
ii) 6-in-one, rotavirus + PCV
iii) 6-in-one, men B

Answer 371

A

i) Men B, PCV, Hib/Men C + MMR
ii) MMR, 4-in-one preschool booster = DTaP + IPV
iii) HPV
iv) 3-in-1 teenage booster = tetanus, diphtheria + IPV, men ACWY

Answer 372

A

Babies born to mothers with hepatitis B = hep B
Neonates at TB risk = BCG
Children 6m-17y with chronic health conditions get yearly flu vaccine (nasal yearly flu normally 2–10y)

Answer 373

A

i) Neonate, 1m and 1y (as well as 2m, 3m, 4m as normal schedule)
ii) Neonate

Answer 374

A

Hypersensitivity reaction initiated by specific immunoglobulins
Food allergy, eczema, allergic rhinitis, asthma, urticaria, insect sting, drugs, latex + anaphylaxis

Answer 375

A

Objectively reproducible symptoms/signs following a defined stimulus at a dose tolerated by a normal person

Answer 376

A

Hygiene hypothesis = high microbial exposure means less allergy
Skin sensitisation theory = regular exposure via food + preventing exposure via breaks in skin before food means less allergies

Answer 377

A

Inhalant = house-dust mite, plant pollens, moulds in asthma
Ingestant = nuts, cow’s milk, eggs, seafood

Answer 378

A

IgE mediated (some food allergies, allergic asthma)
Non-IgE mediated (coeliac disease, some food allergies)

Answer 379

A

Early phase within minutes where release of histamines from mast cells > urticaria, angioedema, sneezing + bronchospasm with a late response 4-6h later with nasal congestion, cough + bronchospasm of lower airway
Urticaria can be trigger if in sun or if child gets hot/cold

Answer 380

A

Typically, delayed onset of Sx + a more varied clinical course

Answer 381

A

Type 1 = IgE trigger mast cells + basophils to release histamines + cytokines
Type 2 = IgG/M bind to cell-surface antigens which is a host cell but activates immune system as considers foreign > cytotoxic
Type 3 = immune complex mediated with activation of complement/IgG
Type 4 = T-cell mediated delayed type hypersensitivity

Answer 382

A

acute anaphylaxis,
hayfever

Answer 383

A

Skin prick test = test a patch + compare the size of wheals with controls
Patch test = useful in allergic contact dermatitis where place a patch of allergens + assess the skins’ reaction
RAST test = measures total + allergen specific IgE in blood
Food challenge test = slowly increase exposure to allergen + measure response

Answer 384

A

T1 hypersensitivity reaction that is IgE mediated, can be non-atopic where environmental allergens cause allergic inflammatory response in nasal mucosa

Answer 385

A

Seasonal (hayfever)
Perennial (year-round) e.g. house dust mite allergy
Occupational

Answer 386

A

Runny, blocked + itchy nose, sneezing
Red + swollen eyes
Post-nasal drip may cause cough
Associated with personal or FHx of atopy

Answer 387

A

Skin prick testing for pollen, animals + house dust mite

Answer 388

A

Avoid trigger, hoover + Change pillows regularly, good ventilation
PO antihistamines taken prior to exposure to reduce allergic Sx
Nasal corticosteroid sprays like mometasone to suppress local Sx
PO Montelukast or specific immunotherapy if poor control

Answer 389

A

Non-sedating = cetirizine, loratadine
Sedating = chlorphenamine (Piriton) + promethazine
Nasal may be good option for rapid onset Sx in response to trigger

Answer 390

A

Severe T1 hypersensitivity reaction where IgE stimulates mast cells to rapidly release histamine + other pro-inflammatory chemicals (mast cell degranulation)

Answer 391

A

Compromise in ABC = life-threatening medical emergency
Foods (peanuts), insect stings, drugs, latex

Answer 392

A

Rapid onset allergic Sx = urticaria, itching, angioedema with swelling around lips + eyes
Anaphylaxis Sx = SOB, wheeze, stridor (larynx swelling), tachycardia, light-headed + collapse

Answer 393

A

Serum mast cell tryptase within 6h of event = mast cell degranulation

Answer 394

A

Airway = secure
Breathing = oxygen, salbutamol to help wheeze, monitor SpO2, RR
Circulation = IV fluid bolus with collapse, monitor BP, ECG
Disability = lie pt flat to improve cerebral perfusion
Exposure = look for flushing, urticaria + angioedema

Answer 395

A

IM adrenaline (EpiPen if community), repeat after 5m if necessary
Antihistamines like chlorphenamine or cetirizine
Steroids like IV hydrocortisone

Answer 396

A

Primary (uncommon) = intrinsic defect in the immune system
Secondary = due to another disease or treatment (HIV, nephrotic syndrome)

Answer 397

A

T-cell defects
B-cell defects
Combined B- + T-cell defects
Neutrophil defect
Leucocyte function defect
Complement defects

Answer 398

A

Severe/unusual viral + fungal infections + failure to thrive in first 2m

Answer 399

A

Present beyond infancy as passively acquired maternal antibodies, severe bacterial infections, esp. (lower) RTIs.
Selective IgA deficiency (#1)
X-linked (Bruton) agammaglobulinaemia
Common variable immune deficiency

Answer 400

A

Severe combined immunodeficiency = group of inherited disorders of profound defective cellular + humoral immunity
Hyper IgM syndrome = B cells produce IgM but prevented from IgG/A

Answer 401

A

Recurrent bacterial infections
Chronic granulomatous disease = X-linked recessive, defect in phagocytosis as fail to produce superoxide after ingestion

Answer 402

A

Delayed separation of umbilical cord, wound healing, chronic skin ulcers
Leucocyte adhesion deficiency = deficiency of neutrophil surface adhesion molecules so inability to migrate to sites of infection

Answer 403

A

Recurrent bacterial infections (meningococcal, HiB, pneumococcus), SLE-like illness
Hereditary angioedema (measure C4 levels)
Mannose-binding lectin deficiency

Answer 404

A

FBC (WCC, lymphocytes, neutrophils)
Blood film
Complement
Immunoglobulins

Answer 405

A

T-cell + neutrophil = co-trimoxazole for PCP, fluconazole for fungal
B-cell = azithromycin for recurrent bacterial infections

Answer 406

A

Prompt, appropriate + longer Abx courses
Screen for end-organ disease (CT scan)
Ig replacement therapy if antibody deficient
Bone marrow transplantation for SCID, chronic granulomatous disease

Answer 407

A

Highly contagious form of bronchitis caused by Bordetella pertussis > gram -ve aerobic coccobacillus

Answer 408

A

Week of coryza (catarrhal phase)
Paroxysmal spasmodic coughing bouts (attacks)
Followed by classic inspiratory whoop (infants may have apneoa not whoop, paroxysmal phase)
Spasms of cough > vomiting, epistaxis, subconjunctival haemorrhage

Answer 409

A

Can last for months = ‘100 day cough’

Answer 410

A

Nasopharyngeal swab with bacterial culture or PCR
Marked lymphocytosis on blood film (predom high lymphocytes)
Test for anti-pertussis toxin IgG if cough >2w

Answer 411

A

Pneumonia
Convulsions
Bronchiectasis

Answer 412

A

Notify PHE
Prophylaxis = vaccine (esp. infants + pregnant women) or if close contact macrolide (erythromycin)
PO macrolides (azithromycin, clarithromycin) 1st line if onset <21d
School exclusion for 48h following Abx or 21d from onset if no Abx

Answer 413

A

Suffering from cyanotic attacks
<6m

Answer 414

A

poliovirus type 1

Answer 415

A

transmitted via faecal-oral route
incubation period is 3-30 days + can be excreted for up to 6 weeks
replicates in nasopharynx + GI tract and can spread to CNS where it can affect anterior horn cells, motor neurons and the brainstem

Answer 416

A

90-95% of cases are asymptomatic
fatigue
fever
nausea and vomiting
diarrhoea
sore throat
headache
photophobia

Answer 417

A

acute flaccid paralysis (AFP)
- initially fatigue, fever N+V
- asymmetrical lower limb weakness and flaccidity

can progress to life-threatening bulbar paralysis and respiratory compromise

Answer 418

A

virus culture from stool, CSF or pharynx
CSF analysis
serum antibodies to poliovirus
MRI of spinal cord
EMG of affected limb(s)

Answer 419

A

supportive care with rehydration and neurological monitoring
physiotherapy
intubation and ventilation for respiratory paralysis

Answer 420

A

post-poliomyelitis syndrome (PPS) - this usually occurs years after the initial infection
- demonstrates the same features as polio infection
- treated in the same way as polio

Answer 421

A

Corynebacterium diphtheriae

Answer 422

A

it infects the epithelium of the skin and the mucosa of the upper resp tract
it forms a grey pseudomembrane

Answer 423

A

sore throat
low grade fever
dysphagia, dysphonia, dyspnoea and croupy cough can occur in serious illness

Answer 424

A

throat + nose swabs, microscopy and culture
diphtheria antibodies
PCR

Answer 425

A

hospitalisation, isolation
diphtheria anti-toxin
antibiotic (procaine benzylpenicillin)

Answer 426

A

prophylactic antibiotics - erythromycin

diphtheria toxoid immunisation

Answer 427

A

candida albicans fungus

Answer 428

A

moist body folds
treatment with broad spectrum antibiotics
immunosuppression
diabetes mellitus

Answer 429

A

skin folds
vagina
penis
mouth
corners of mouth
nail beds (paronychia)

Answer 430

A

skin scrapings for microscopy and culture

Answer 431

A

oral or topical anti-candidial drugs e.g. nystatin, fluconazole

Answer 432

A

rash/scaling
white/yellow vaginal discharge
white patches on tongue
cracks at the corner of mouth
white/yellow nail that separates from the nail bed

Answer 433

A

Droplet via resp route

Answer 434

A

Contagious 4d before rash + until lesions crusted (often 5d)

Answer 435

A

PO aciclovir

Answer 436

A

Characteristic rash in dermatomal distribution, acute, unilateral, blistering painful rash

Answer 437

A

PO aciclovir + corticosteroids

Answer 438

A

Auricular pain,
facial nerve palsy,
vesicular rash around ear,
?vertigo + tinnitus

Answer 439

A

Microorganisms reach meninges by direct extension from ears, nasopharynx or bloodstream spread

Answer 440

A

Affect hormonal contraception,
not advised in pregnancy
have to monitor LFTs + renal function

Answer 441

A

Mostly viral – herpes viruses (HSV 1 if child or 2 if neonate from birth, VZV), enteroviruses, EBV, resp viruses
Non viral = any bacterial meningitis, TB, lyme disease
Non-infective = autoimmune antibodies against brain

Answer 442

A

Sickle cell disease
immunodeficiency

Answer 443

A

FBC (raised WCC), raised ESR + CRP, raised platelets (week 2)
Echocardiogram with close follow up (6w) to rule out aneurysm

Answer 444

A

anaphylaxis,
aseptic meningitis,
organ dysfunction

Answer 445

A

Avoidance of MMR vaccine

Answer 446

A

Clinical Dx with serological (blood or saliva) testing for epidemiology

Answer 447

A

Raised amylase

Answer 448

A

Mild viral URTI (sore throat, cough, fever)
Painful red vesicular lesions on hands, feet, mouth + tongue (often buttocks too)

Answer 449

A

Oral contact ‘kissing disease’

Answer 450

A

Splenic rupture,
haemolytic anaemia,
chronic fatigue,
EBV associated with Burkitt’s lymphoma

Answer 451

A

Via respiratory droplets

Answer 452

A

Otitis media (#1),
quinsy,
post-strep glomerulonephritis,
rheumatic fever

Answer 453

A

Infects red cell precursors in bone marrow which can cause complications

Answer 454

A

Respiratory secretions,
vertical transmission
transfusions

Answer 455

A

Rapidly by autoinoculation by infected exudate

Answer 456

A

Children with pre-existing skin disease (atopic eczema)

Answer 457

A

Children <5y as when older they develop immunity to toxins

Answer 458

A

Steven-Johnson’s syndrome

Answer 459

A

Toxin producing S. aureus + group A strep released from infection acts as a superantigen to cause multi-organ dysfunction

Answer 460

A

Tampons being left in,
female barrier contraceptive

Answer 461

A

Staph aureus or strep pyogenes ± another synergistic anaerobic organism
May be complication from cellulitis infection in soft tissues

Answer 462

A

Enters via mucous membranes or skin (kissing, genital contact, vertical transmission at birth)

Answer 463

A

Supportive but PO aciclovir if severe, chlorhexidine mouthwash

Answer 464

A

Secondary bacterial infection + septicaemia

Answer 465

A

IV aciclovir as life-threatening, bacterial infection will need Abx

Answer 466

A

Immunocompromised,
overseas contact,
homeless,
IVDU,
alcoholics

Answer 467

A

Majority cases are in Africa + Asia (India, China)

Answer 468

A

Confirms latent TB + differentiates from BCG

Answer 469

A

Acid fast bacilli stain red with Ziehl-Neelson stain on Lowenstein-Jenson culture medium

Answer 470

A

Patchy consolidation,
pleural effusions,
hilar lymphadenopathy

Answer 471

A

Peripheral neuropathy (I’m-so-numb-azid)
co-prescribe pyridoxine (vit B6) after puberty as prophylaxis

Answer 472

A

Gout due to hyperuricaemia,
rash

Answer 473

A

Optic neuritis, reduced acuity + colour (eye-thambutol)

Answer 474

A

Personal/familial tendency to produce IgE in response to ordinary exposures to allergens (triad = eczema, asthma + rhinitis)

Answer 475

A

autoimmune disease,
haemolytic disease of newborn,
transfusion reaction

Answer 476

A

SLE,
RA,
HSP,
post-strep glomerulonephritis

Answer 477

A

TB,
contact dermatitis

Answer 478

A

IgE trigger mast cells + basophils to release histamines + cytokines

Answer 479

A

IgG/M bind to cell-surface antigens which is a host cell but activates immune system as considers foreign > cytotoxic

Answer 480

A

immune complex mediated with activation of complement/IgG

Answer 481

A

T-cell mediated delayed type hypersensitivity

Answer 482

A

Tree pollen or grass (hayfever), house dust mites + pets, mould

Answer 483

A

Severe, prolonged, unusual or recurrent (SPUR) infections

Answer 484

A

DiGeorge syndrome
HIV
Duncan syndrome (X-linked lymphoproliferative disease)
Ataxic telangiectasia
Wiskott-Aldrich

Answer 485

A

Can give fever, advise normal + administer paracetamol

Answer 486

A

diphtheria
tetanus
pertussis DTaP (whooping cough)
polio IPV
Haemophilus influenza B (HiB)
Hepatitis B

Answer 487

A

It is a red itchy bumpy rash which can occur anywhere on the body.
It is common - affects at least 20% of people at some stage in their lives
The swelling and redness often improves in one area and then appears in another
It can tingle and itch but is not usually painful.
It is also called nettle rash, welts or wheals.

Answer 488

A

It is a swelling deep in the skin which occurs in a minority of people with urticaria or sometimes on its own.
It frequently involves the eyelids, lips and sometimes the mouth.
Sometimes the swelling can be very obvious making it difficult to open the eyes for example.

Answer 489

A

Mast cells release histamine in response to a perceived irritant

Answer 490

A

viral infection
idiopathic
cold
heat
exercise
stress
medications - NSAIDS, antihypertensives
thyroid function

Answer 491

A

single episode could be allergy but there needs to be an obvious link and occur within one hour of food consumption
most of the time it is not an allergy

Answer 492

A

single episode can last a few days
can get several episodes over a 4-6 week period = viral
recurrent angioedema + urticaria can last for 16-18 months

Answer 493

A

antihistamines - cetirizine, loratadine and fexofenadine
leukotriene receptor antagonists - montelukast, ranitidine, tranexamic acid
prednisolone for severe attacks

Answer 494

A

Hip, then leg > knee > thigh > foot (least likely)

Answer 495

A

Trauma like # (accidental or NAI)
Infections (septic arthritis, osteomyelitis)
DDH (chronic)
Malignancy (Ewing’s, osteogenic sarcoma)
Neuromuscular disease (CP, Duchenne’s)
ANY CHILD <3Y WITH LIMP NEEDS URGENT ASSESSMENT*

Answer 496

A

Trauma, infection, malignancy
Transient synovitis (acute)
Perthe’s disease (P for primary school, chronic)
Juvenile idiopathic arthritis (chronic)

Answer 497

A

Trauma, infection, malignancy
Slipped upper femoral epiphysis (S for secondary school, acute/chronic)
JIA
Reactive arthritis

Answer 498

A

Full Hx + exam (top>toe)
General obs (HR, BP, temp)
FBC (WCC), CRP/ESR, blood cultures if septic
XR both AP + lateral for joint (+ joints above/below)
USS joint to look for thickening of capsule or effusion

Answer 499

A

Abnormal relationship of femoral head to the acetabulum leading to aberrant development of hip causing instability
Spectrum of dysplasia (underdevelopment), subluxation (partial dislocation) or frank dislocation of the hip

Answer 500

A

First degree FHx, breech at ≥36w or breech delivery ≥28w, multiple pregnancy
– USS hip by 6w even if normal NIPE exam
Other = F>M 6:1, oligohydramnios

Answer 501

A

Painless limp
Limited abduction (reduced ROM)
Leg length discrepancy
May have waddling or abnormal gait but otherwise well

Answer 502

A

NIPE at 72h + 6–8w

Leg length discrepancy
Restricted hip abduction of affected side
Barlow + ortolani tests
Clunking of hips on tests

Answer 503

A

Galeazzi/Allis sign = difference in knee length when hips flexed + feet flat on bed

Answer 504

A

USS by 2w of age
XR may be useful in older infants >3m

Answer 505

A

If <6m = Pavlik harness to hold femoral head in position (flexed + abducted) to allow the hip socket (acetabulum) to develop normal shape (remove after 6-8w)
Surgical reduction if harness fails or Dx >6m = hip spica cast to immobilise hip for prolonged period after surgery (risk of avascular necrosis + re-dislocation)

Answer 506

A

Serious infection of the joint space as it can lead to bone destruction

Answer 507

A

Staphylococcus aureus

Answer 508

A

Usually single joint (knee or hip) + acute onset
Hot, red, swollen + painful joint (including at rest)
Refusal to weight bear
Stiffness + reduced ROM with pain if moved (hip may be held flexed)
Systemic = fever, lethargy, sepsis

Answer 509

A

FBC,
blood cultures,
CRP + ESR,
USS guided joint aspiration for MC&S

Answer 510

A

IV empirical Abx (flucloxacillin) until sensitivities back
Arthroscopic lavage or surgical drainage if resolution does not occur rapidly or deep-seated joint (hip)
Immobilise joint initially but then mobilise to prevent deformity
Rest + analgesia

Answer 511

A

Infection in the bone + bone marrow, often in the metaphysis of long bones

Answer 512

A

S. Aureus #1 or H. influenzae (directly via bone or haematogenous spreading)

Answer 513

A

Acutely unwell child
Refusing to weight bear
Severe pain, swelling + tenderness
May have high fever (low grade if chronic)

Answer 514

A

FBC (Raised WCC), raised ESR/CRP, blood cultures, bone marrow aspiration MC&S
XR can be normal
MRI is best imaging to establish Dx

Answer 515

A

IV empirical Abx (flucloxacillin or clindamycin if allergy) until sensitivities back
Amoxicillin, cefotaxime or ceftriaxone if <4y + suspect H. influenzae
?Surgical drainage or debridement of infected bone

Answer 516

A

Disruption of blood flow to femoral head causing avascular necrosis of the bone
Affects the epiphysis of femur, which is bone distal to growth plate (physis)
Over time, revascularisation or neovascularisation + healing of the femoral head with remodelling of bone

Answer 517

A

Social deprivation
LBW
Passive smoking

Answer 518

A

4-8y, mostly male, limp (no Hx of trauma)
Pain (often unilateral) in hip or groin (?knee referral) with restricted hip movements (internal + external rotation)
+ve Trendelenburg test (abductor dysfunction) = ‘sound side sags’

Answer 519

A

Blood tests all normal
XR of both hips (with frog views) is initial investigation + assesses healing
– Flattening of femoral head
Technetium bone scan or MRI may be needed to confirm Dx if normal XR

Answer 520

A

Premature fusion of the growth plates
Soft + deformed femoral head can lead to early hip OA

Answer 521

A

Keep femoral head within acetabulum (cast, braces)
Physio to retain ROM in muscles + joints without excess stress on the bone

Answer 522

A

i) Conservative + observe, bed rest, traction, crutches, analgesia (good prognosis)
ii) Surgery to improve alignment + function of the femoral head + hip

Answer 523

A

Autoimmune inflammation in joints > joint pain, swelling + stiffness

Answer 524

A

Onset before 16y with no underlying cause
Joint swelling/stiffness
> 6w in duration to exclude other causes (i.e. reactive)

Answer 525

A

Joint pain, swelling + stiffness (particularly morning) = hallmarks
Limping/functional disability
Decreased ROM
Warmth + colour change

Answer 526

A

Systemic JIA (Still’s disease)
Polyarticular JIA
Oligoarticular JIA
Enthesitis-related arthritis

Answer 527

A

Subtle salmon-pink rash
High swinging fevers
Lymphadenopathy, weight loss, muscle pain, splenomegaly
Pleuritis, pericarditis + uveitis

Answer 528

A

Antinuclear antibodies (ANA) + rheumatoid factor = NEGATIVE
Raised inflammatory markers = CRP/ESR, platelets + serum ferritin

Answer 529

A

Macrophage activation syndrome = severe activation of immune system with massive inflammatory response

Answer 530

A

≥5 joints affected, equivalent of RA in adults

Answer 531

A

≤4 joints affected, often just monoarthritis

Answer 532

A

Anterior uveitis = ophthalmologist referral

Answer 533

A

Paeds version of seronegative spondyloarthropathies (ankylosing spondylitis, psoriatic/reactive arthritis, IBD-related arthritis)

Answer 534

A

Sx of psoriasis (psoriatic plaques, nail pitting, dactylitis) or IBD

Answer 535

A

Arthritis that develops following an infection where the organism cannot be recovered from the joint

Answer 536

A

Reiter’s = can’t see (conjunctivitis), can’t pee (urethritis) and can’t climb a tree (arthritis)

Answer 537

A

Same as RA (LESS) –

Loss of joint space
Erosions (causing joint deformity)
Soft tissue swelling
Soft bones (osteopenia)

Answer 538

A

Chronic anterior uveitis > severe visual impairment
Flexion contractures of joints
Growth failure + constitutional problems like delayed puberty
Osteoporosis

Answer 539

A

MDT approach = education, psychologist, physio, pain team, rheumatology

Answer 540

A

NSAIDs for Sx relief during flares
Intra-articular steroids for oligoarthritis
Avoid systemic steroids if possible (osteoporosis, growth suppression)
– IV methylprednisolone can be used if severe arthritis
DMARDs like methotrexate, rarely sulfasalazine for polyarthritis
Biologics if poor control like tocilizumab, adalimumab, etanercept

Answer 541

A

‘Irritable hip’ caused by transient inflammation of the joint synovial membrane

Answer 542

A

Limp + refusal to weight bear, 2–10y
Groin or hip pain (may be referred to knee)
Mild low-grade fever but otherwise systemically well
Limited internal rotation + pain on movement but comfortable at rest

Answer 543

A

# 1 = exclude septic arthritis so if suspect = cultures + joint aspiration
Normal WCC, slight increase in CRP
USS may show effusion but XR normal

Answer 544

A

Self-limiting with only simple analgesia + rest = rapid Sx improvement
Manage at home with safety netting + review to ensure no fever + Sx resolving

Answer 545

A

Displacement of femoral head epiphysis postero-inferiorly along the growth plate (through zone of hypertrophy)

Answer 546

A

Boys, >10y, obese + undergoing growth spurt
Metabolic endocrine abnormalities (hypothyroid)

Answer 547

A

May be Hx of minor trauma that triggers onset
Hip, groin, thigh or knee pain (disproportionate to severity of trauma)
Painful limp + may have antalgic gait
Very restricted internal rotation (+ abduction)

Answer 548

A

XR initial Ix of choice (AP + frog-leg views)
Bloods (incl. inflammatory markers) normal
?Technetium bone scan + MRI scan

Answer 549

A

Surgery = internal fixation (pinning femoral head into position for prevention)

Answer 550

A

Inflammation at the tibial tuberosity where the patellar ligament inserts
10–15y sporty males (football, basketball)

Answer 551

A

Visible or palpable hard + tender lump/swelling at tibial tuberosity
Pain in anterior aspect of knee, often unilateral
Exacerbated by physical activity, kneeling + extension of knee

Answer 552

A

Reduce pain + inflammation = NSAIDs, ice, reduce exercise)
Stretching + physio once settled to strengthen joint + improve function

Answer 553

A

Diagnosis by exclusion –

Pain never present at start of day after waking but can awaken from sleep
Physical activities are not limited, no limp, settles with massage
Bilateral pain in lower limbs (shins/ankles) + not limited to joints
Can be worse after a day of vigorous activity + intermittent

Answer 554

A

≥1 vertebral crush #
≥2 long bone fractures by age 10 (≥3 by age 19)
Bone mineral density less than 2.5 standard deviations below the mean

Answer 555

A

Inherited = osteogenesis imperfecta, haematological issues
Acquired:
– Drug induced (Steroids)
– Endocrinopathies (hypoparathyroidism)
– Malabsorption
– Immobilisation (disabilities)
– Inflammatory disorders

Answer 556

A

Autosomal dominant condition leading to brittle bones + prone to fractures

Answer 557

A

Bone fragility = recurrent + inappropriate #, joint + bone pain
Bone deformity (bowed legs, bent bones, scoliosis)
Impaired mobility due to poor muscle mass
Poor growth > short stature
Hypermobility as ligamentous laxity

Answer 558

A

Conductive hearing loss (otosclerosis)
Blue/grey tinted sclera due to scleral thinness
Valvular prolapse, aortic dissection > aortic incompetence
Hernias
‘Wormian bones’ = skull feels like bubble wrap (wiggly black lines on skull XR)

Answer 559

A

Clinical Dx with XR to diagnose fractures + bone deformities
DEXA scan to look at bone mineral density (osteoporosis)
7 types under the sillence classification

Answer 560

A

i) Mildest form, common with blue sclera
ii) Lethal form, chest too small to allow breathing, lots of rib # + lungs do not function
iii) Normal sclera

Answer 561

A

Physio + OT to maximise strength + function
Paeds for medical treatment + follow up
Orthopaedic surgeons to manage #
Pain team, specialist nurses for advice + support

Answer 562

A

Vitamin D supplementation to prevent deficiency
Bisphosphonates (IV pamidronate) to increase bone density + reduce #

Answer 563

A

Defective bone mineralisation > “soft” + deformed bones (paeds osteomalacia)
Vitamin D deficiency (recommended paeds intake 400IU = 10mg)

Answer 564

A

Darker skin (need more sunlight)
Lack of exposure to sun
Poor diet or malabsorption
CKD as kidneys metabolise vitamin D to active form

Answer 565

A

Increases Ca2+ absorption at gut + reabsorption at kidneys + role in immunity

Answer 566

A

Lack of Ca2+ + phosphate in blood which are required for bone construction > defective bone mineralisation
Low Ca2+ causes secondary hypoparathyroidism as parathyroid gland tries to raise Ca2+ level by secreting parathyroid hormone
This leads to increased resorption of Ca2+ from the bones, worsening the issue

Answer 567

A

Bone pain, swelling + deformities
Muscle weakness + poor growth (gross motor delay)
Pathological or abnormal #
May have hypocalcaemic convulsions or carpopedal spasm

Answer 568

A

Bowing of legs, knock knees
Harrison sulcus = indentation of softened lower ribcage at site of attachment of diaphragm
Rachitic rosary = ends of ribs expand at costochondral junctions causing lumps along chest
Craniotabes = soft skull with delayed closure of sutures + frontal bossing
Expansion of metaphyses (esp. wrist)

Answer 569

A

Serum biochemistry
FBC + ferritin (Fe anaemia), inflammatory markers
Kidney, liver + TFTs, malabsorption screen (anti-TTG)
Autoimmune + rheumatoid tests
XR required to diagnose

Answer 570

A

Low = calcium + phosphate
High = ALP + PTH
25-hydroxyvitamin D levels deficient (<25nmol/L)

Answer 571

A

Osteopenia (radiolucent bones)
Cupping
Fraying of metaphyses
Widened epiphyseal plate

Answer 572

A

Prevention #1 –
- Breastfeeding women should take vitamin D supplement
- Dietary advice to increase calcium + vitamin D
- Children vitamin D deficient > ergocalciferol
Vitamin D + calcium supplementation for children with rickets + specialist input

Answer 573

A

‘Stork mark’
Most common vascular birthmark
Flat red or pink patches on baby’s eyelids, neck or forehead at birth
Fade completely in few months

Answer 574

A

‘Strawberry mark’
Raised marks on skin often red, F>M
Not present at birth, appear in first month, increase in size then shrink + disappear
Normally self-limiting, beware over eye + airway

Answer 575

A

‘Port wine stain’ = permanent, often unilateral
Present at birth + grows with infant, treated with laser therapy
Seen in Sturge-Weber syndrome (neuro Sx)

Answer 576

A

Mole, can be multiple present in Turner’s

Answer 577

A

Flat, light brown patches on skin
> 5 = neurofibromatosis

Answer 578

A

Milk spots (sebaceous plugs where sweat glands plugged by sebum), normal

Answer 579

A

Chronic atopic condition caused by defects in normal continuity of skin barrier leaving tiny gaps for irritants, microbes + allergens to enter + trigger immune response
Leads to inflammation of the skin

Answer 580

A

Infants = dry, red, itchy + sore patches of skin over face + trunk
Young children = extensor surfaces
Older children = mostly on flexor surfaces (creases), face + neck creases

Answer 581

A

Opportunistic bacterial infection as skin breakdown leaves entry, particularly S. aureus
– Presents as increased erythema, yellow crust, pustules
– PO flucloxacillin or admit for IV if severe
Eczema herpeticum

Answer 582

A

Avoid triggers (weather, stress, wash/cleaning products)
Maintenance = artificial skin barrier with emollients (E45), special soap substitutes

Answer 583

A

Thicker emollients (hydromol or epaderm)
Topical steroids (start with hydrocortisone > betnovate > dermovate)
Wet wraps = cover affected areas in thick emollient with wrap to keep moisture overnight
Severe = PO ciclosporin, corticosteroids

Answer 584

A

Thinning of skin
Telangiectasia
Bruising

Answer 585

A

Chronic autoimmune condition where abnormal T-cell activation > hyperproliferation of keratinocytes + so psoriatic skin lesions

Answer 586

A

Plaque
Guttate ‘rain-drop’
Erythrodermic + pustular

Answer 587

A

Well-demarcated, raised, silvery scaling lesions on extensor surfaces (elbows + knees)
Scalp involvement, most often at hair margin

Answer 588

A

Explosive eruption of very small circular plaques on trunk, often 2w after strep throat
Common in paeds, resolves in 3-4m

Answer 589

A

Most severe + life-threatening
Widespread inflammation of skin > extensive erythematous areas or pustules under areas of erythema
Systemic = pyrexia, malaise
Admit

Answer 590

A

Koebner phenomenon = new plaques of psoriasis at sites of skin trauma
Residual pigmentation of skin after lesions resolve
Auspitz sign = small points of bleeding when plaques scraped off
Nail changes (pitting + onycholysis)

Answer 591

A

10–20% develop psoriatic arthritis
Increased risk of CVD, metabolic syndrome, VTE

Answer 592

A

1st line = topical steroids, topical vitamin d analogues (calcipotriol)
2nd line = UV phototherapy
3rd line = immunosuppression with methotrexate or biologics

Answer 593

A

Irritant dermatitis = friction between skin/nappy + contact with urine + faeces
Candida dermatitis = due to breakdown in skin + the warm, moist environment

Answer 594

A

Irritant = sore, red, inflamed skin but spares the skin creases
Candida = involves skin creases, satellite lesions (small similar lesions near edges of principle lesion) + may have oral thrush

Answer 595

A

Delayed changing of nappies
Diarrhoea
Irritant soap products + vigorous cleaning
PO Abx predispose to Candida

Answer 596

A

Highly absorbent nappies
Maximise time not wearing + ensure dry before replacing nappy
Change nappy + clean skin ASAP
Water or gentle alcohol-free products to clean
Topical imidazole if candida

Answer 597

A

Disproportional immune response causes epidermal necrosis > blistering + shedding of top layer of skin
SJS = <10% body surface, toxic epidermal necrolysis affects >10%

Answer 598

A

Meds = AEDs, Abx, allopurinol, NSAIDs
Infections = herpes simplex, mycoplasma pneumonia, CMV, HIV

Answer 599

A

Non-specific Sx of fever, cough, sore throat + itchy skin
Develop purple/red rash that spreads across skin, starts to blister
Few days later skin sheds leaving raw tissue underneath = PAINFUL

Answer 600

A

Secondary infection
Permanent skin damage due to skin involvement + scarring
Visual complications such as severe scarring or even blindness

Answer 601

A

Admission as medical emergency, cease causative meds
Nutritional care, Abx, analgesia, ophthalmology input
Steroids, immunoglobulins + immunosuppressants by specialists

Answer 602

A

it is an abnormally shaped meniscus (usually lateral)
it usually presents in younger people
it is more prone to injury as it is more likely to get stuck/tear

Answer 603

A

a visible or audible snap on terminal leg extension along with pain and swelling in the absence of trauma

Answer 604

A

MRI - can show abnormal shape and any tears
clinical examination

Answer 605

A

symptomatic = arthroscopic partial meniscectomy
asymptomatic = physiotherapy

Answer 606

A

it is lateral curvature in the frontal plane of the spine
- measured >10 degrees on x-ray

Answer 607

A

idiopathic = most common
congenital = usually from congenital structural defect of the spine e.g. spina bifida
secondary = neuromuscular imbalance (cerebral palsy, muscular dystrophy), disorders of bone or connective tissues

Answer 608

A

visible curve in spine
shoulders, waist or hips look uneven
one shoulder blade appears bigger
ribs stick out further on one side
low back pain
back stiffness
pain + numbness in legs
fatigue (due to muscle strain)

Answer 609

A

cerebral palsy
muscular dystrophy
birth defects
infections
tumours
marfan syndrome
down syndrome

Answer 610

A

braces
spinal fusion surgery
spine and rib-based growing operation

Answer 611

A

tilting of the head to one side caused by contraction of the neck muscles

Answer 612

A

congenital
acquired

Answer 613

A

congenital muscular torticollis (CMT) = usually noticed in 1st month after birth. It causes shortening + fibrosis of sternocleidomastoid (can have palpable mass)
malformed cervical spine
spina bifida

Answer 614

A

MSK = muscle spasm
infection = URTI, otitis media, dental infection, pharyngeal infection
atlantoaxial rotatory fixation
inflammation = juvenile idiopathic arthritis
neoplasm = CNS tumours

Answer 615

A

awkward head posture for a prolonged time

Answer 616

A

most of the time it is a clinical diagnosis
- cervical spine x-ray can be useful

Answer 617

A

treat the cause
- muscle spasm = self resolve
- infection = antibiotics
- Congenital = physiotherapy

Answer 618

A

it is an overuse injury that is common in skeletally immature athletic young people

it occurs secondary to repetitive activities such as jumping, sprinting etc.

Answer 619

A

during the stage of bone maturation
10-12 in girls
12-14 in boys

Answer 620

A

repeated traction over the tibial tubercle which results in microvascular tears, fractures and inflammation

Answer 621

A

male gender
age - 12-15 in boys, 8-12 in girls
sudden skeletal growth
repetitive activities such as jumping and sprinting

Answer 622

A

anterior knee pain with or without swelling
pain aggravated by physical activity

Answer 623

A

clinical diagnosis

Answer 624

A

conservative treatment - RICE
surgical treatment in severe cases once the child has fully grown
physiotherapy

Answer 625

A

Intra-abdominal pathology like hernia, testicular torsion

Answer 626

A

Posterior hip dislocation (adduct hips + press down on knees)

Answer 627

A

Relocate a dislocated femoral head (abduct + push thigh anteriorly)

Answer 628

A

Most common <2y,
usually from haematogenous + soft tissue swelling

Answer 629

A

i) GBS, S. aureus, coliforms
ii) S. aureus, pneumococcus, haemophilus
iii) S. aureus, gonococcus (adolescents)

Answer 630

A

Kocher’s modified criteria /5, ≥3 is likely
–Temp>38.5
– Raised CRP/ESR/WCC
– Non-weight bearing

Answer 631

A

Acute = rapid presentation with acutely unwell child
Chronic = deep seated, slow growing infection + Sx

Answer 632

A

M>F,
<10y

Answer 633

A

Open #,
orthopaedic surgery,
sickle cell anaemia (Salmonella predominates),
immunocompromised (HIV),

Answer 634

A

Symmetrical, affects small joints (of hand + feet) as well as large joints (hips + knees)

Answer 635

A

Mild systemic Sx = mild fever, anaemia + reduced growth

Answer 636

A

If rheumatoid factor +ve = seropositive (tend to be older children)

Answer 637

A

ANA +ve but RF -ve

Answer 638

A

Enthesitis = inflammation at the point a tendon or muscle inserts to bone

Answer 639

A

HLA-B27 gene
Prone to anterior uveitis = ophthalmology referral

Answer 640

A

Post STI (chlamydia) in older children or Salmonella, Campylobacter

Answer 641

A

Sx (analgesia, NSAIDs, sometimes intra-articular steroids)

Answer 642

A

Recent viral URTI,
small % will develop Perthe’s

Answer 643

A

Bone mineral density less than 2.5 standard deviations below the mean
DEXA scan

Answer 644

A

Defects in type 1 collagen protein which is essential for the structure + function of bone, as well as skin, tendons + other connective tissues

Answer 645

A

Sunlight, fortified cereals, eggs, oily fish

Answer 646

A

Mole, can be multiple present in Turner’s
‘Mongolian blue spot’, disappear by 4, commonly lower back/buttocks, more common in non-Caucasian
Bruising + NAI so important to document

Answer 647

A

Erythema toxicum neonatorum by histamine reaction = self-limiting red blotches with eosinophils on biopsy

Answer 648

A

HLA-B13,
environmental triggers (alcohol, stress, group A strep)

Answer 649

A

Non-specific Sx of fever, cough, sore throat + itchy skin
Develop purple/red rash that spreads across skin, starts to blister
Few days later skin sheds leaving raw tissue underneath = PAINFUL
Can involve lips + mucous membranes, urinary tract, lungs
Eyes can become inflamed + ulcerated

Answer 650

A

Acutely unwell with DIC,
febrile,
anaemia,
thrombocytopenia,
bleeding,
non-blanching rash,
low ESR

Answer 651

A

Life-threatening = supportive + steroids

Answer 652

A

Affects precursors to B + T cells
It leads to uncontrolled proliferation of immature blast cells affecting both the blood + bone marrow (lymphoid progenitor cells and lymphoblasts)

Answer 653

A

General = anorexia, fever, weight loss, night sweats
Bone marrow infiltration = pancytopenia
Reticuloendothelial infiltration = hepatmospenolmegaly, lymphadenopathy
Other organ infiltration (more common at relapse) = headache, testicular enlargement, bone pain

Answer 654

A

Anaemia (pallor, lethargy),
neutropenia (frequent or severe infections),
thrombocytopenia (bruising, petechiae, epistaxis)

Answer 655

A

FBC + blood film = pancytopenia, WCC up or down, circulating blast cells
Bone marrow aspiration to Dx - blast cells
CXR + CT to identify mediastinal mass and abdominal lymphadenopathy
LP to identify CNS involvement

Answer 656

A

Age 2-10
Female
WCC <20
No CNS disease
Caucasian

Answer 657

A

Psychological impact of childhood cancer
Fertility = offer to freeze eggs or sperm before Tx
CNS development, growth impact, delayed puberty, cardiac + renal toxicity

Answer 658

A

Correct abnormalities with blood/platelet transfusion
Fluids for hydration
Allopurinol to protect kidneys from tumour lysis syndrome

Answer 659

A

Blood and platelet transfusion
Chemotherapy
Steroids
Allopurinol to prevent tumour lysis syndrome
Intrathecal drugs, e.g. methotrexate
Acute control of infections with IV antibiotics
Neutropenia makes this high risk
Stem cell transplant

Answer 660

A

i) Combo chemo often IV vincristine + dexamethasone
ii) IT chemo (methotrexate)
iii) Mod intensity chemo, co-trimoxazole prophylaxis for PCP
iv) Intensive chemo to consolidate remission
v) 2y for girls, 3y for boys as higher recurrence

Answer 661

A

Bone marrow transplantation

Answer 662

A

Malignancies of lymphocytes which accumulate in lymph nodes, may also infiltrate organs + divided histologically

Answer 663

A

Mostly self-limiting like viral URTI (cold, tonsillitis)
Can be HIV, autoimmune or malignancies
Enlarging node without infective cause, persistently enlarged, unusual site (supraclavicular), presence of B Sx or abnormal CXR

Answer 664

A

Fever and sweating
Enlarged rubbery non-tender nodes
Systemic ‘B’ symptoms, e.g. fever
Painful nodes on drinking alcohol
some patients (commonly young women) have disease localised to the mediastinum

Answer 665

A

Presence of Reed-Sternberg cells in lymph node biopsy

Answer 666

A

Using the Ann Arbor system
- I = confined to single LN region
- II = ≥2 nodal areas on same side of diaphragm
- III = nodal areas on both sides of diaphragm
- IV = spread beyond LNs e.g. liver

Each staged subdivided to A if no systemic Sx or B if ‘B’ Sx

Answer 667

A

Combination chemo ± radiotherapy (overall 80% cured)
ABVD
- Adriamycin
- Bleomycin
- Vinblastine
- Decarbazine
autologous marrow transplant

Answer 668

A

T-cell malignancies
B-cell malignancies
Extra-nodal disease

Answer 669

A

May present as ALL or non-Hodgkin lymphoma both being characterised by a mediastinal mass with bone marrow infiltration
Mediastinal mass may cause SVC obstruction

Answer 670

A

Present as non-Hodgkin lymphoma with localised lymph node disease, usually in head + neck or abdomen

Answer 671

A

Often GI > pain from obstruction, a palpable mass or even intussusception

Answer 672

A

Steroids

R-CHOP
- Monoclonal antibodies to CD20 -> Rituximab
- CHOP regimen:
- Cyclophosphamide
- Hydroxy-daunorubicin
- Vincristine
- Prednisolone

Answer 673

A

Almost always primary (unlike adults)
60% are infratentorial

Answer 674

A

Astrocytoma (#1) varies from benign to glioblastoma multiforme
Medulloblastoma arises in the midline of posterior fossa, may have spinal mets
Ependymoma mostly in posterior fossa where it behaves as medulloblastoma
Brainstem glioma
Craniopharyngioma

Answer 675

A

Developmental tumour arising from squamous remnant of Rathke pouch
Not truly malignant but locally invasive (bitemporal hemianopia often lower quadrant as superior chiasmal compression)

Answer 676

A

Evidence of raised ICP
Focal neurology dependant on where the lesion is

Answer 677

A

Headache worse in morning
Papilloedema
Vomiting, esp. in the morning
Behaviour or personality change
Visual disturbance (squint secondary to 6th nerve palsy, nystagmus)

Answer 678

A

Spinal tumours = back pain, peripheral weakness of arms/legs or bladder + bowel dysfunction depending on level of lesion
Ataxia, seizures

Answer 679

A

MRI for visualisation
Avoid LP if raised ICP

Answer 680

A

Outcome depends on location, how much is cleared + how much healthy brain tissue removed
Survivors face neuro disability, growth + endocrine problems, neuropsychological issues

Answer 681

A

1st line = surgical resection + ventriculoperitoneal shunt to reduce risk of coning + treat hydrocephalus
Chemo (fewer options as less drugs cross BBB) or radiotherapy

Answer 682

A

Arise from neural crest tissue in the adrenal medulla + sympathetic nervous system,
most common <5y,
NOT brain tumour

Answer 683

A

Spontaneous regression sometimes occur in v young infants
Spectrum from benign (ganglioneuroma) to highly malignant neuroblastoma

Answer 684

A

Abdominal mass
Sx of metastatic = weight loss, hepatomegaly, pallor, bone pain + limp
Uncommon = paraplegia, cervical lymphadenopathy, proptosis, periorbital bruising, skin nodules

Answer 685

A

Often crosses midline + envelopes major vessels + lymph nodes
Can grow very large
Classically abdo primary is of adrenal origin

Answer 686

A

Raised urinary catecholamine levels
CT/MRI + confirmatory biopsy
Evidence of metastatic disease = bone marrow sampling, MIBG scan ±bone scan

Answer 687

A

Localised primaries + no mets can often be cured with surgery
Metastatic = chemo, autologous stem cell rescue, surgery + radio
Immunotherapy may be used for long-term maintenance

Answer 688

A

Nephroblastoma that originates from embryonal renal tissue, cure rate 80%
> 80% present before age 5, rare after 10y
FHx = Wilm’s tumour susceptibility gene

Answer 689

A

Large abdominal mass found incidentally in an otherwise well child
May have flank pain, anorexia, anaemia, painless haematuria + HTN

Answer 690

A

USS ± CT/MRI showing intrinsic renal mass distorting the normal structure
Mass with characteristic mixed tissue densities (cystic + solid)
Staging for distant mets (often lung), biopsy for histology Dx

Answer 691

A

Initial chemo followed by delayed nephrectomy (full if 1 kidney, partial if bilateral but RARE)
Radiotherapy for more advanced disease

Answer 692

A

Osteogenic sarcoma more common than Ewing sarcoma but Ewing seen more in younger children
Both have male predominance
Uncommon before puberty

Answer 693

A

Limbs most common site (particularly femur, tibia + humerus)
Persistent localised bone pain often precedes mass, otherwise well
May be worse at night + cause disrupted sleep

Answer 694

A

Raised ALP on bloods
Plain XR followed by MRI + bone scan, ?PET scan + bone biopsy
CT chest for lung mets + bone marrow sampling to exclude involvement

Answer 695

A

XR = destruction + variable periosteal new bone formation
Periosteal reaction leads to classic “sunburst” appearance
Ewing sarcoma often shows substantial soft tissue mass

Answer 696

A

Combo chemo before surgery (amputation avoided if possible)
Radio used in Ewing sarcoma for local disease, esp. if surgical resection is impossible or incomplete (e.g. pelvis or axial skeleton)

Answer 697

A

Malignant tumour of retinal cells which can lead to severe visual impairment

Answer 698

A

Retinoblastoma susceptibility gene on chromosome 13 = AD but incomplete penetrance > offer genetic screening
All bilateral tumours are hereditary, 20% of unilateral are

Answer 699

A

White pupillary reflex noted to replace the normal red reflex or a squint
May have decreased visual acuity + nystagmus
Most present within 3y

Answer 700

A

Screened for in NIPE
MRI + Examination under anaesthetic
Biopsy is avoided + treatment based on ophthalmological findings

Answer 701

A

Significant risk of second malignancy (especially sarcoma) amongst survivors of hereditary retinoblastoma

Answer 702

A

Cure cancer + preserve vision
Chemo, particularly if bilateral to shrink tumours > local laser treatment to retina
Radiotherapy or enucleation of eye (removal) if advanced
Most cured but many visually impaired

Answer 703

A

Mostly hepatoblastoma or hepatocellular carcinoma
Primary liver tumours in neonates = haemangioma

Answer 704

A

Abdominal distension or mass are common
Pain + jaundice rare

Answer 705

A

Elevated serum alpha fetoprotein in nearly all cases
USS/CT/MRI to visualise the tumour + extent of disease

Answer 706

A

Chemo, surgery or liver transplant if inoperable
Majority of hepatoblastoma can be cured but prognosis worse for hepatocellular

Answer 707

A

Generalised reabsorptive disorder of renal tubular transport in the PCT resulting in…
– Type 2 (proximal) renal tubular acidosis
– Polydipsia, polyuria, aminoaciduria + glycosuria
– Osteomalacia/rickets

Answer 708

A

Usually secondary to inborn errors of metabolism
– Cystinosis (AR > intracellular accumulation of cysteine, most common)
– Wilson’s disease, galactosaemia, glycogen storage disorders

Answer 709

A

Responsible for transporting oxygen around the body in RBCs
HbF = 2 alpha + 2 gamma subunits
HbA = 2 alpha + 2 beta subunits

Answer 710

A

HbF has greater affinity to oxygen than adult so oxygen binds more easily + is more reluctant to let go = crucial for oxygen to transport from maternal to foetal Hb

Answer 711

A

At birth to compensate for low oxygen concentration in the foetus
By 6m of age very little HbF produced so HbA predominates

Answer 712

A

Hb level below the normal range
Neonate = <14g/dL
1–12m = <10g/dL
1–12y = <11g/dL

Answer 713

A

Increased red cell production
Increased red cell destruction (haemolysis)
Blood loss (uncommon in paeds like Meckel’s, vWD, foetomaternal bleeding)

Answer 714

A

Ineffective erythropoiesis (Fe, folate deficiency, CKD)
Red cell aplasia
Normal reticulocytes, abnormal MCV in nutrient deficiencies

Answer 715

A

G6PD deficiency, haemoglobinopathies, hereditary spherocytosis
Raised reticulocytes, abnormal appearance on blood films, +ve direct antiglobulin test if immune cause

Answer 716

A

Characterised by reduced red cell lifespan due to increased red cell destruction in the circulation (intravascular haemolysis) or liver/spleen (extravascular)
120d

Answer 717

A

Red cell survival reduced significantly but bone marrow production increases too, anaemia = bone marrow cannot compensate
Neonates = immune haemolytic anaemias, children = instrinsic abnormalities (G6PD)

Answer 718

A

Anaemia
Hepatosplenomegaly
Unconjugated bilirubinaemia
Excess urinary urobilinogen

Answer 719

A

Reduced RBC production = congenital red cell aplasia + congenital parvovirus infection > red cell aplasia
Haemolytic anaemia = immune (haemolytic disease of newborn) or hereditary (G6PD etc)

Answer 720

A

Inadequate erythropoietin production
Reduced red cell lifespan
Frequent blood sampling whilst in hospital
Iron + folic acid deficiency after 2-3m.

Answer 721

A

# 1 cause of childhood anaemia
Bone marrow requires iron to produce Hb
Iron mainly absorbed in the duodenum + jejunum + requires acid from the stomach to keep iron in soluble ferrous (Fe2+) form, if acid drops it changes to insoluble ferric (Fe3+) form

Answer 722

A

Inadequate intake = common as infants require additional iron for increasing blood volume
Malabsorption = Crohn’s + coeliac
Blood loss = common in menstruating females

Answer 723

A

Breast milk, formula, cow’s milk or weaning (cereals)
Markedly increased when eaten with food rich in vitamin C + inhibited by tannin in tea

Answer 724

A

Generic = fatigue, SOB, headaches, dizziness, palpitations
Young infants feed more slowly + children tire easily

Answer 725

A

Generic = pallor (inc. conjunctival), tachycardia, tachypnoea
Pica = consumption of non-food materials
Koilonychia, angular cheilitis, brittle hair + nails

Answer 726

A

FBC = low Hb, microcytic (low MCV + MCH), normal reticulocytes
Blood film = hypochromic microcytic red cells
Iron studies:
– Low = serum ferritin, iron + transferrin saturation
– High = total iron binding capacity

Answer 727

A

i) Proportion of transferrin bound to iron
ii) Total space on transferrin for Fe to bind

Answer 728

A

Diet = eat red meat (beef, lamb), oily fish, green veg (broccoli, spinach), dried fruit (raisins)
Children may be given polysaccharide iron complex (Niferex)
Ferrous sulfate or fumarate often used

Answer 729

A

Constipation
Black coloured stools
Nausea

Answer 730

A

Abnormal variant (haemoglobin S) which polymerises to be an abnormal sickle (crescent) shape + so more fragile + easily destroyed > haemolytic anaemia
Amino acid substitution (glutamine > valine)

Answer 731

A

Reduced lifespan so can get trapped in small vessels leading to vaso-occlusion > ischaemia
Can be exacerbated by dehydration, cold, stress, infections + hypoxia, associated with raised haematocrit

Answer 732

A

Autosomal recessive
Abnormal gene for beta-globin on C11
Heterozygous = sickle-cell trait
Homozygous = sickle cell disease (HbSS)

Answer 733

A

More common in Africa, India + the Middle East (areas traditionally affected by malaria)
Sickle-cell trait reduces the severity of malaria making them more likely to survive + pass the genes on

Answer 734

A

6m as HbF unaffected so manifests as HbF reduces
All have moderate anaemia with detectable jaundice from chronic haemolysis
All have marked increase in infection susceptibility, esp. pneumococci + H. influenzae due to hyposplenism secondary to chronic sickling + microinfarction of the spleen

Answer 735

A

Vaso-occlusive (painful) crises
Bones of limbs + spine common (may lead to avascular necrosis e.g. femoral heads)
Pain, fever + often those of triggering infection
Acute chest syndrome

Answer 736

A

Fever or resp Sx (CP, tachypnoea) with new infiltrates on CXR
Can be due to infection (pneumonia, bronchiolitis) or non-infective (pulmonary vaso-occlusion or fat emboli)
Emergency > Abx or antivirals, blood transfusions for anaemia, may need NIV or intubation

Answer 737

A

‘Hand-foot syndrome’ common leading to dactylitis
Priapism in men > urological emergency, aspiration

Answer 738

A

Haemolytic crises (sometimes with associated infection)
Aplastic crises (parvovirus causes cessation of RBC production)
Sequestration crises

Answer 739

A

Sudden hepatic or splenic enlargement, abdo pain + circulatory collapse from accumulation of sickled cells blocking blood flow
Supportive = blood transfusions, fluid resus, splenectomy can prevent this + used in recurrent crises as can lead to splenic infarction > increased infection susceptibility

Answer 740

A

Prenatal Dx via CVS
Detection via Guthrie test
FBC = low Hb, high reticulocytes
Blood film = sickled RBCs
Dx with Hb electrophoresis showing high amounts of HbSS + absent HbA

Answer 741

A

Short stature + delayed puberty
Stroke + cognitive issues
Pulmonary HTN
Chronic renal failure
Psychosocial issues

Answer 742

A

Fully immunised (PCV, HiB, meningococcus)
Avoid vaso-occlusive crisis triggers
PO phenoxymethylpenicillin prophylaxis
PO folic acid as increased demands due to haemolysis
Hydroxycarbamide + hydroxyurea can stimulate HbF production to prevent painful crises
Bone marrow transplant curative + offered if failed response

Answer 743

A

Cold, dehydration, excessive exercise, stress + hypoxia
Dress warmly, plenty of drinks

Answer 744

A

PO or IV analgesia according to need (?opiates)
IV fluids, oxygen
Infection treated with Abx, blood transfusion for severe anaemia
Exchange transfusion if severe (e.g. neuro complications)

Answer 745

A

AR disorder arising from ≥1 gene defects, resulting in a reduced rate of production of ≥1 globin chains
RBCs more fragile + breakdown easily
Alpha = defect in alpha globin chains
Beta = defect in beta globin chains

Answer 746

A

Alpha thalassaemia trait
Often asymptomatic with mild or absent anaemia
Red cells hypochromic + microcytic

Answer 747

A

Mild-moderate hypochromic microcytic anaemia + splenomegaly
Few patients are transfusion dependent

Answer 748

A

Alpha thalassaemia major
Death in utero with foetal hydrops from foetal anaemia
Occurs in families of South-East Asian origin, homozygotes

Answer 749

A

Indian subcontinent, Mediterranean + Middle East
Beta thalassaemia minor (1 abnormal + 1 normal gene)
Beta thalassaemia intermedia (2 defective or 1 defective + 1 deletion genes)
Beta thalassaemia major (homozygous for deletion genes)

Answer 750

A

Carriers of abnormally functioning beta-globin gene
Mild microcytic + hypochromic anaemia (monitor)
Differentiate from Fe deficiency by measuring serum ferritin (normal)

Answer 751

A

More severe microcytic anaemia, beta-globin mutation allow a small amount of HbA and/or a large amount of HbF to be produced
Monitor + occasional blood transfusion

Answer 752

A

Most severe form with no HbA as abnormal beta globin gene
- Severe transfusion-dependent anaemia from 3-6m, jaundice, failure to thrive

Answer 753

A

Extramedullary haematopoiesis can occur if no regular blood transfusions
Leads to hepatosplenomegaly + bone marrow expansion leading to maxillary overgrowth + skull bossing

Answer 754

A

FBC + blood film = hypochromic microcytic anaemia
HbA2 raised in beta-thalassaemia trait, HbA2 + HbF raised in major
Serum ferritin to differ between Fe anaemia + check iron overload
Hb electrophoresis for Dx
DNA testing via CVS before birth

Answer 755

A

Repeated + Regular blood transfusions can cause chronic iron overload
Heart (cardiomyopathy, heart failure)
Liver (cirrhosis)
Pancreas (diabetes)
Pituitary (delayed growth + sexual maturation)
Skin (hyperpigmentation)
Arthritis + joint pain

Answer 756

A

Lifelong monthly blood transfusions for the most severe cases
Desferrioxamine for iron chelation to prevent overload
Bone marrow transplant can be curative, reserved for beta thalassaemia major

Answer 757

A

Haemophilia A = factor VIII deficiency
Haemophilia B = factor IX deficiency
X-linked recessive (M>F), A>B, girls with Turner’s increased risk as 1 X

Answer 758

A

Neonates = intracranial haemorrhage, haematomas + cord bleeding
Recurrent spontaneous bleeds, often large, into joints (haemoarthrosis) + muscles (cause arthropathy if not prevented)
Easy bruising, haematomas, mouth/gum bleeding, haematuria

Answer 759

A

Around 1y as children become more mobile
NAI

Answer 760

A

FBC + blood film
Prothrombin time (factors 2, 5, 7, 10, extrinsic) normal
Activated partial thromboplastin time (intrinsic) = greatly increased
Severity dependent on amount of FVIII:C or FIX:C levels
Prenatal Dx with CVS

Answer 761

A

IV infusion of recombinant FVIII or FIX concentrate if active bleeding (or prophylactic to reduce arthropathy risk)
Desmopressin stimulates vWF release for bleeding/prevention, TXA
AVOID aspirin, NSAIDs + IM injections (can worsen bleeding)

Answer 762

A

Formation of antibodies against the clotting factor can render it ineffective

Answer 763

A

Facilitates platelet adhesion to damaged endothelium
Acts as carrier protein for FVIII:C, protecting it from inactivation + clearance

Answer 764

A

Deficiency of vWF leading to defective platelet plug formation + deficient FVIII:C > most common inherited bleeding disorder
AD, type 1 most common + mildest
Severity increases with type 2, type 3 has very low or no vWF (AR)

Answer 765

A

Bruising, excessive + prolonged bleeding after surgery, mucosal bleeding (epistaxis, menorrhagia, bleeding gums)
In contrast to haemophilia = spontaneous soft tissue bleeding like large haematomas uncommon

Answer 766

A

FBC (normal platelets) + blood film, biochemical screen including renal + liver function
Prolonged bleeding time
Prothrombin time normal
APTT = elevated or normal
vWF antigen decreased, vWF multimers variable

Answer 767

A

Pressure applied if active bleeding
Minimise bleeding with desmopressin or TXA
Severe = plasma derived FVIII concentrate or vWF infusion
AVOID aspirin, NSAIDs + IM injections as can worsen bleeding

Answer 768

A

Nasal or s/c
Release of vWF + FVIII concentrate

Answer 769

A

Secondary to

Haemorrhagic disease of the newborn due to vitamin K deficiency
Liver disease as location of clotting factor production
ITP + DIC

Answer 770

A

Essential for factors 2, 7, 9 + 10 (1972) production + naturally occurring anticoagulants like protein C + S
Prolonged prothrombin time so increased bleeding
All neonates get vitamin K IM to facilitate coagulation

Answer 771

A

Inadequate intake = neonates, long-term chronic illness
Malabsorption = coeliac, cystic fibrosis
Vitamin K antagonists = warfarin

Answer 772

A

Commonest cause of thrombocytopenia in childhood
T2 hypersensitivity reaction with destruction of circulating platelets by anti-platelet IgGs

Answer 773

A

1-2w post viral
Petechiae or purpuric rash (petechiae are pin-prick, purpura are larger)
Can cause epistaxis, other mucosal bleeding + bruising

Answer 774

A

FBC shows marked thrombocytopenia
May have compensatory megakaryocyte increase in bone marrow

Answer 775

A

Often acute + self-limiting
Severe bleeding may need prednisolone, IVIg, blood/platelet transfusions

Answer 776

A

rhesus positive father and negative mother have a rhesus positive baby
mother + baby have incompatible antigens which induces primary immune response
problems tend to occur in subsequent pregnancies + results in destruction of foetal haemoglobin

Answer 777

A

anti-D antibodies in mother detected by Coombe’s test that all women have at 1st antenatal appointment
routine USS may detect hydrops fetalis or polyhydramnios
mild cases = jaundice, pallor + hepatosplenomegaly, hypoglycaemia
severe cases = oedema, petechiae + ascites

Answer 778

A

indirect coombe’s test show antibodies
antenatal USS shows hydrops fetalis
fetal blood sample

Answer 779

A

transfusion of O negative packed cells cross-matched with maternal blood at 16-18 weeks

Answer 780

A

50% = normal haemoglobin + bilirubin but should be monitored for anaemia for 6-8 weeks
25% = require transfusion + may require phototherapy to avoid kernicterus
25% = stillborn or have hydrops fetalis

Answer 781

A

kernicterus which can cause extrapyramidal, auditory and visual abnormalities and cognitive deficit
late-onset anaemia
graft-versus-host disease
portal vein thrombosis + portal hypertension

Answer 782

A

identify all women who have been sensitised by coombe’s testing at first antenatal visit

anti-D immunoglobulin should be given to all rhesus negative women at 28 + 34 weeks

Answer 783

A

Trisomy 21,
immunocompromised (HIV, immunosuppressants)

Answer 784

A

80% of leukaemias in children,
peaks at 2–5y
associated with ionising radiation

Answer 785

A

Hepatosplenomegaly,
lymphadenopathy

Answer 786

A

CNS = headaches, vomiting + nerve palsies,
testicular enlargement,
bone pain

Answer 787

A

Mass anywhere along sympathetic chain so could lead to spinal cord compression

Answer 788

A

HbF production decreases from 32w with HbA + HbA2 production increasing

Answer 789

A

FBC and blood film = WCC usually high
Blast cells on film and in bone marrow

Answer 790

A

Hodgkin’s lymphoma (more common in adolescence)
Non-Hodgkin’s (more common in childhood)

Answer 791

A

high ESR
FBC = anaemia (normochromic normocytic)
reed sternberg cells
low Hb
high serum lactase dehydrogenase

Answer 792

A

Any lymphoma not involving Reed-Sternberg cells

Answer 793

A

Fever and sweating
Enlarged rubbery non-tender nodes
Systemic ‘B’ symptoms, e.g. fever
GI and skin involvement

Answer 794

A

A malignant proliferation of haemopoietic stem cells (immature blood cells)

Answer 795

A

AML - Acute Myeloid Leukaemia
CML - Chronic Myeloid Leukaemia
ALL - Acute Lymphoblastic Leukaemia
CLL - Chronic Lymphoblastic Leukaemia

Answer 796

A

Neoplastic proliferation of blast cells (immature blood cells)
affects myeloid progenitor cells and myeloblasts

Answer 797

A

Preceding haematological disorders
Prior chemotherapy
Exposure to ionising radiation
Down’s syndrome

Answer 798

A

Anaemia -> breathlessness, fatigue, pallor
Infection
Hepatosplenomegaly
Peripheral lymphadenopathy
Gum hypertrophy
Bone marrow failure and bone pain

Answer 799

A

Because of bone marrow failure

Answer 800

A

Because of tissue infiltration

Answer 801

A

FBC - anaemia, thrombocytopaenia, neutropoenia
Blood film - leukaemic blast cells
Bone marrow biopsy - Auer rods
Cytogenetic analysis and immuno-phenotyping

Answer 802

A

FBC = anaemia and thrombocytopenia and neutropenia

BM biopsy = leukaemic blast cells (with Auer rods)

Answer 803

A

Blood and platelet transfusions
IV fluids
Allopurinol to prevent tumour lysis
Infection control with IV antibiotics
Chemotherapy
Steroids
Sibling matched allogenic bone marrow transplant

Answer 804

A

Uncontrolled clonal proliferation of myeloid cells (basophils, eosinophils and neutrophils)

Answer 805

A

Philadelphia chromosome

forms a fusion gene BCR/ABL on chromosome 22 – has tyrosine kinase activity – simulate cell division

Answer 806

A

Insidious onset

Symptomatic anaemia
Abdominal pain - splenomegaly
Weight loss, tiredness, palor
Gout - due to purine breakdown
Bleeding - due to platelet dysfunction

Answer 807

A

FBC - anaemia, raised myeloid cells, high WCC (eosinophilia, basophilia, neutrophilia)
Increased B12
Blood film - left shirt, basophilia
Bone marrow biopsy - increased cellularity
Philadelphia chromosome seen in 80+% of cases  t(9;2) - Stimulates cell division

Answer 808

A

Chemotherapy
Tyrosine kinase inhibitors, e.g. Imatinib - Given orally
Stem cell transplant

Answer 809

A

FORMS fusion gene BCR/ABL on chromosome 22 –> tyrosine kinase activity –> stimulates cell division

Answer 810

A

Proliferation of mature B lymphocytes leads to accumulation of mature B cells that have escaped apoptosis
Chronic malignant transformation of mature lymphoid cells

Answer 811

A

● Normal or low Hb
● Raised WCC with very high lymphocytes
● Blood film – smudge cells may be seen in vitro

Answer 812

A

Watch and wait
Chemotherapy
Monoclonal antibodies, e.g. rituximab
Targeted therapy, e.g. bruton kinase inhibitors (ibrutinib)

Answer 813

A

Radiation exposure
Chemicals (benzene compounds)
Drugs

Answer 814

A

Child = failure to meet expectations (disabled, wrong sex), born after forced or commercial sex work
Parent = MH issues, substance abuse, LD, young
Family = stepparents, domestic abuse, multiple or closely spaced births
Environment = low socioeconomic status

Answer 815

A

Emotional = persistent emotional mistreatment of a child resulting in adverse effects of a child’s emotional development
Sexual = forcing a child to take part in sexual activities
Neglect = persistent failure to meet a child’s basic physical + psychological needs

Answer 816

A

PV/PR bleed or itching
PV discharge
STIs
Bruising
Oversexualised child
Dilated anus

Answer 817

A

Inadequate food, drink, emotional support, clothing, shelter
Inadequate supervision or access to medical care = severe + persistent infections (scabies, lice), failure to engage with child health promotion, failure to attend follow-ups

Answer 818

A

Too many injuries, wrong site, unusual shape or pattern
Delay in presenting (old injuries), multiple A&E visits
No Hx, Hx inconsistent with injuries or that changes

Answer 819

A

Shins, knees, elbows, toddlers can bump their heads
Abdo, genitalia, insides of arms/legs, behind neck or other soft bits, young babies that cannot roll

Answer 820

A

# = metaphyseal, multiple # at different healing stages, posterior rib # in babies v. specific, radial, humeral, femoral
Bruising, burns, scalds, failure to thrive
Torn frenulum (forcing bottle into mouth)

Answer 821

A

FBC, clotting screen, bone profile, radiology
Developmental + social services assessment
If suspected > hospital admission + can break confidentiality
Fundoscopy for retinal haemorrhages

Answer 822

A

Shaken baby syndrome = retinal haemorrhages, subdural haematoma + encephalopathy

Answer 823

A

Child act 2004 allows to speak to child without parents’ consent, safeguards children

Answer 824

A

None
Microcephaly
Short palpebral fissures, hypoplastic upper lip, small eyes, smooth philtrum
LDs, poor growth + cardiac malformations
Can have alcohol withdrawal Sx a birth = irritable, hypotonic, tremors

Answer 825

A

Med emergency as can cause meningitis, sinus thrombosis
External ear may protrude forwards, severe otalgia (classically behind), fever
Swelling, erythema + tenderness over mastoid process
Abx ±mastoidectomy

Answer 826

A

Persistence of thyroglossal duct
Midline, smooth + moves when they stick their tongue out
USS shows thin walled + anechoic (echoic suggests cyst infection)

Answer 827

A

Not in midline, tend to appear along border of sternocleidomastoid
75% originate from second branchial cleft, often unilateral + smooth

Answer 828

A

Soft lesion in posterior triangle that transilluminates (seen in Turner’s)

Answer 829

A

Found on lateral aspect of eye + produces sebaceous material
Can communicate intracranially causing meningitis
USS shows heterogeneous + have variable amounts of calcium + fat

Answer 830

A

Fluid constituents + bag size = NaCl 0.9% + dextrose 5% + KCl 10mmol (500ml)
Rate of administration in ml/hour
Signature

Answer 831

A

Weight ([Age + 4] x 2), including weight change
Fluid input/output in past 24h
Fluid status (dehydrated)
Recent bloods (electrolytes)

Answer 832

A

0.9% NaCl + 5% dextrose + KCl 10mmol
100ml/kg/day for first 10kg
50ml/kg/day for next 10kg
20ml/kg/day for every kg after 20kg
Divide by 24 = ml/hour

Answer 833

A

<5% = slight thirst, dry lips
5-10% = sunken eyes, reduced skin turgor, decreased urine output, dry lips + mucous membranes (no shock)
>10% = reduced GCS, cold, mottled peripheries, anuria, sunken fontanelle, CRT >2s, hypotension (late)

Answer 834

A

(Well weight [kg] – current weight [kg]) ÷ well weight

- % dehydration x 10 x weight (kg)

Answer 835

A

Given in shock
0.9% NaCl at 20ml/kg over <10m
After >3 boluses call for paeds intensive care support as risk > pulmonary oedema

Answer 836

A

Trauma, primary cardiac pathology (heart failure), DKA (after first 20ml/kg)
10ml/kg boluses to prevent pulmonary oedema

Answer 837

A

Day 1 = just 10% dextrose
From day 2 = Na (3mmol/kg/day) + K (2mmol/kg/day)
Day 1 = 60ml/kg/day
Day 2 = 90ml/kg/day
Day 3 = 120ml/kg/day
Day 4 + beyond = 150ml/kg/day

Answer 838

A

i) When half a standard population of children reach that level of development
ii) Age a child is expected to have reached a milestone (often 2 standard deviations from the mean)

Answer 839

A

Age correct up to 2 years
9m born 2 months early should only be expected to be at developmental stage of 7m

Answer 840

A

Ongoing process of following child over time
Can be incorporated into well-child checks, general physical exam or routine vaccine visits

Answer 841

A

Gross motor
Fine motor + vision
Speech, hearing + language
Social, emotional + behavioural

Answer 842

A

New born = Limbs flexed, symmetrical posture, head lag on pulling up

Answer 843

A

8 months = Crawl (some may bottom shuffle or commando crawl)

Answer 844

A

2 years = Runs, kick ball (2.5y)

Answer 845

A

Turns head to follow object (fix + follow)

Limit age = 3 months

Answer 846

A

transfers toys from one hand to the other
- limit age = 9 months

Answer 847

A

Crayon scribbles
3 brick tower

Answer 848

A

draws a cross

Answer 849

A

Startles at loud sounds, quietens to parent’s voice

Answer 850

A

Responds to own name, imitates adult sounds “dada, mama”

Answer 851

A

joins two or more words together to make simple phrases, “Give me teddy”

Answer 852

A

Smiles responsively
– Limit age 8w

Answer 853

A

Drinks from cup with 2 hands

Answer 854

A

i) Seek out other children + play with them, turn-taking, follows simple rules, bowel control, fork + spoon
ii) Has best friend, bladder control, dresses self, imaginative play
iii) Knife + fork

Answer 855

A

Moro (startle)
Grasp (palmar/plantar)
Sucking/rooting
Stepping
Asymmetrical tonic neck reflex

Answer 856

A

i) Sudden extension of head causes symmetrical extension then flexion of limbs. Stops 3–4m
ii) Touch palm (palmar) or sole (plantar) + baby will grasp or curl toes. Stops 4–5m
iii) Head turns to stimulus when touched near mouth, assists in breastfeeding. Stops at 4m

Answer 857

A

i) Stepping movements when held vertically + dorsum of feet touch surface. Stops at 2m
ii) Baby supine + head turned to one side > arm on that side stretches out + opposite flexes at elbow

Answer 858

A

Parachute
Postural support
Labyrinthine righting
Lateral propping

Answer 859

A

i) Suspend baby prone + slowly lower head towards a surface > arms + leg extend in protective fashion
i) When held upright if feet touch a surface legs take weight + may push up like a bounce

Answer 860

A

i) Head moves in opposite direction to which body is tilted
ii) When sitting, arms extends on the side to which child falls as saving mechanism

Answer 861

A

Persistence of primitive reflexes + lack of development of postural reflexes is the hallmark of UMN abnormality in the infant (cerebral palsy)

Answer 862

A

i) Implies slow acquisition of all skills or of one particular field
ii) Cognitive, physical, both or relate to specific functional skills
iii) Maldevelopment of a skill

Answer 863

A

i) Loss/abnormality of physiological function or anatomical structure
ii) Any restriction or lack of ability due to the impairment
iii) Results from disability + limits fulfilment of a normal role

Answer 864

A

Prenatal
Perinatal
Postnatal

Answer 865

A

Genetics (Down’s, fragile X)
Congenital hypothyroidism
Teratogens (alcohol + drug abuse)
Congenital infection (TORCH)
Neurocutaneous syndromes (tuberous sclerosis, neurofibromatosis)

Answer 866

A

Extreme prematurity (intraventricular haemorrhage)
Birth asphyxia (HIE)
Hyperbilirubinaemia
Hypoglycaemia

Answer 867

A

Infection (meningitis, encephalitis)
Anoxia (suffocation, near-drowning, seizures)
Head trauma (accidental or NAI)
Hypoglycaemia

Answer 868

A

Bio = prems, LBW, birth asphyxia, hearing/vision impairment
Environment = poverty, poor parental education, maternal substance abuse

Answer 869

A

Slow development in all developmental domains
Presents in first 2y of life
Down’s, fragile X, foetal alcohol syndrome, Rett syndrome + metabolic disorders

Answer 870

A

Slow development in gross motor domain
- Cerebral palsy, ataxia, myopathy, spina bifida + visual impairment

Answer 871

A

Slow development in fine motor domain
- Dyspraxia, cerebral palsy, muscular dystrophy, visual impairment, congenital ataxia (rare)

Answer 872

A

Slow development in speech + language domain
- Specific social circumstances, hearing impairment, LD, neglect, autism + cerebral palsy, cleft lip/palate

Answer 873

A

Exposure to multiple languages, sibling that do all the talking
Referral to SALT, audiology + health visitor with safeguarding if ?neglect

Answer 874

A

Slow development in personal + social domain
- Emotional + social neglect, parenting issues + autism

Answer 875

A

Thorough Hx + exam (hearing + vision)
Cytogenic (chromosome karyotype)
Metabolic (TFTs, LFTs, U+Es, CK, lactate)
Infection (congenital infection screen)
Focal neuro (CT/MRI head, EEGs)

Answer 876

A

Parental phenotype + genotype
Nutrition
Pregnancy factors
Psychosocial deprivation
Endocrine function

Answer 877

A

Weight = naked infant or child only in underclothing
Height = >2y standing height, <2y horizontal
Head circumference = occipitofrontal circumference is a measure of head + accurate representation of brain size + development

Answer 878

A

Microcephaly = ?brain not formed properly, ?LDs
Macrocephaly = ?hydrocephalus
Note = small babies likely to have small heads, compare ALL values

Answer 879

A

Faulty technique (inexperienced staff), faulty equipment (wrongly calibrated), uncooperative child
Boys = [(Dad + mum height in cm) ÷ 2] + 7
Girls = [(Dad + mum height in cm) ÷ 2] – 7

Answer 880

A

Assess if a child’s overall height is abnormal (<2nd or >98th centile)
– GP review if <2nd, Paeds review if <0.4th
Assess if a child is failing to follow their growth potential (drop centile line)
– More concerning than consistently 9th centile
Assess if a child is losing or gaining weight quickly
– ?Pathology

Answer 881

A

First 2y = growth velocity fastest in utero + infancy, driven by nutritional factors
2y-puberty = steady slow growth (genes, thyroid + growth hormones, health)
Puberty = rapid growth spurt driven by sex hormones
When the epiphyses fuse

Answer 882

A

i) Understands comparatives “which one is BIGGER”
ii) “Why”, “when”, “how” questions, understands complex instructions “before you put x in y give z to mummy”

Answer 883

A

First within 72h of birth + second by GP at 6–8w
- General observation, eyes, heart, hips + genitalia

Answer 884

A

Weight, height, head circumference (HC = measure of brain size)
Palpate sutures + fontanelle
Dysmorphic features
Reflexes (grasp, sucking, rooting, moro)

Answer 885

A

Red reflex (congenital cataracts, retinoblastoma)
- Movement (visual loss)

Answer 886

A

HR 110–160bpm
Murmur (CHD)
Femoral pulse (coarctation)
Central cyanosis (cyanotic CHD)

Answer 887

A

Barlow + Ortolani test (DDH)

Answer 888

A

Testes (cryptorchidism)
Ambiguous genitalia (CAH)
Genitalia (hypospadias)
Imperforate anus (bladder/vaginal fistula)

Answer 889

A

Detect congenital abnormalities that were not identified at birth
Check for potential problems that could arise due to FHx
Provide opportunity for parents to ask questions about baby

Answer 890

A

9 months old

Answer 891

A

18 months

Answer 892

A

6 weeks = Lifts head when lying prone + moves it side-side

Answer 893

A

3 months = Holds head upright when held sitting

Answer 894

A

6 months = Rolls, sits without support (6m = rounded back, 8m = straight back)

Limit age = 9 months old

Answer 895

A

10 months = Stand independently, cruise around furniture

Answer 896

A

12 months = Walk unsteadily, broad gait hands apart

Answer 897

A

15 months = Walks steadily
– Limit age 18m: ?Duchenne’s, ?hip issues, ?cerebral palsy

Answer 898

A

3 years = Jump, stand on 1 leg briefly, pedal tricycle, stairs (1 foot up 2 down)

Answer 899

A

4 years = Hops, balance on one leg for few seconds, stairs like adult

Answer 900

A

5 years = Rides bike, skip on both feet

Answer 901

A

Reaches for toys
– Limit age = 6 months

Answer 902

A

Palmar grasp of objects, transfers toys
– Limit age (toys) 9m

Answer 903

A

mature pincer grip
- limit age = 12 months

Answer 904

A

Index finger to point, casting bricks (disappear by 18m) + builds 2 brick tower

Answer 905

A

Vertical line
6 brick tower

Answer 906

A

Copies circle
8 brick tower or train with 4 carriages

Answer 907

A

draw a circle
copies or makes bridge

Answer 908

A

draws square
makes steps (after demonstration)

Answer 909

A

draw a triangle

Answer 910

A

Cooing noises, vocalises alone or when spoken to “aa, aa”

Answer 911

A

Turns head to sounds, understands “bye bye” + “no” (7m), monosyllabic babbles (consonants) “bababa”

Answer 912

A

Understands names “drink”,
3 words other than “mama” and “dada”

Answer 913

A

6-10 words,
understands nouns “show me the SPOON”
is able to show two body parts, “where is your nose?” - baby will point

Answer 914

A

talks constantly in 3-4 word sentences
understands 2 joined commands “push me fast Daddy”

Answer 915

A

Puts food in mouth,
shakes rattle

Answer 916

A

Separation anxiety from parent,
stranger fear (until 2y)

Answer 917

A

Waves bye-bye, plays peek-a-boo, claps

Answer 918

A

Uses spoon to feed self

Answer 919

A

Extends interest beyond parents (waves at strangers),
parallel play (next to but not with children),
symbolic play (copies actions like feeding a doll),
dry by day,
removes some clothes

Answer 920

A

Retinal haemorrhages
Encephalopathy
Subdural haemotoma

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PAEDS RENAL/NEONATAL/INFECTION/ALLERGY/DERM/ONCOLOGY/HAEMATOLOGY/MISC Flashcards

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