paediatric specialites

Question 1

what is eczema

Answer 1

itchy, dry inflammatory skin disease

Question 2

what are the 2 type of causes of eczema

Answer 2

endogenous - internal

exogenous - external

Question 3

give examples of endogenous eczema

Answer 3

• Atopic – ‘genetic barrier dysfunction’
• Seborrheoic – face/scalp – scale associated (babies)
• Discoid – annular/circular patches
• Pomphylx – vesicles affecting palms/soles
• Varicose – oedema/venous insufficiency (rarer in children)

Question 4

give examples of exogenous eczema

Answer 4

-Allergic contact dermatitis (sensitised to allergen)
-Irritant contact dermatitis (friction, cold, chemicals e.g acids, alkalis, detergents, solvents) from repetitive motion e.g. hand washing (history)
Photosensitive/photoaggravated eczema – aggrevated by light, obvious pattern – t-shirt cut line

Question 5

what things may eczema flares in children be associated with

Answer 5

¥	Infections/viral illness
¥	Environment: central heating, cold air
¥	Pets: if sensitised/allergic
¥	Teething – distribution of face and jaw
¥	Stress 
¥	Sometimes no cause for flare found

Question 6

what is the most common type of eczema in kids

Answer 6

atopic eczema

Question 7

what leads to the dry inflamed skin in atopic eczema

Answer 7

Immune mediated defects in the skin barrier function

Question 8

what is atopy

Answer 8

overactive immune response to environmental stimuli

Question 9

what 3 conditions are linked in atopy

Answer 9

asthma, eczema, hay fever

Question 10

where does eczema normally start in infants

Answer 10

face/ neck then spreads

cheek confused with infantile acne

Question 11

what places are most affected by eczema in older children

Answer 11

Flexural pattern predominates -antecubital fossae, popliteal fossae, wrists, hands, ankles

Question 12

what protein is affected in eczema

Answer 12

fliggarin
(Filaggrin proteins bind the keratin filaments together, in the epidermis only. Also play a role in producing a natural moisturising factor)

Question 13

what is the treatment for eczema

Answer 13

dermal 500

Question 14

what areas are mostly affected by seborrheic dermatitis

Answer 14

scalp and face

Question 15

what age range are affected by seborrheic dermatitis

Answer 15

3 -12 months

Question 16

what skin commensal is seborrheic dermatitis associated with

Answer 16

malassezia

Question 17

what is the treatment of seborrheic dermatitis

Answer 17

Emollients, antifungal creams, antifungal shampoos, mild topical steroids (hydrocortisone)

Question 18

describe discoid eczema

Answer 18

Scattered annular/circular patches itchy eczema.

Can occur in this pattern as part of atopic eczema or separate entity/diagnosis.

Question 19

why are high dose steroids needed to treat pomphylx eczema

Answer 19

hand and foot thicker skin

Question 20

what is pomphylx eczema

Answer 20

vesicles of eczema on hands and feet - can be intensely itchy

Question 21

what is varicose eczema associated with

Answer 21

venous insufficiency affecting legs

Question 22

which patients may varicose eczema be seen in

Answer 22

lymphedema
previous chemotherapy
varicose veins

Question 23

what is the treatment of varicose eczema

Answer 23

Emollients, topical steroids, compression stockings (help move fluid)

Question 24

what are some treatments of eczema

Answer 24

emollients

topical steroids

Question 25

how much topical steroid should be used to treat eczema

Answer 25

finger tip unit

Question 26

list some topical steroids for eczema

Answer 26

Mild (hydrocortisone) Moderate (Eumovate) 25x Potent (Betnovate) 100x Very potent (dermovate) 600x

Question 27

what are some side effects of topical steroids

Answer 27

skin thinning in prolonged use (not prescribed 3-4 weeks)
Do not use above eumovate on the skin – too thin
Should not cause thinning if used appropriately and with clear instructions

Question 28

how often should you use topical steroids to treat eczema

Answer 28

once daily for 1-2 weeks - then alternate days for a few more days
maintenance - once a week in some areas

Question 29

what things may give you irritant eczema

Answer 29

repeated contact; water and soaps, touching irritant foods; citrus, tomatoes, chemical irritants.

Question 30

what test is done for contact allergens

Answer 30

patch test

blood test for others

Question 31

what percentage of childhood eczema is mild

Answer 31

80% clear with time

Question 32

what are the immediate symptoms of a food allergy (type 1 hypersensitive)

Answer 32

lip swelling, facial redness/itching, anaphylactoid symptoms

Question 33

what is a type 4 hypersensitivity reaction to food allergy

Answer 33

worsening of eczema 24/48 hours after ingestion

Question 34

what are the 2 ways to test for food allergy

Answer 34

Blood test for specific IgE antibodies to certain foods

Skin prick testing

Question 35

what are the commonest thing for children to be allergic to

Answer 35

milk/dairy, soy, peanuts, eggs, wheat, fish

airborne - house dust mite (most common in eczema), pet dander, pollens

Question 36

how does impetigo manifest

Answer 36

pustules and honey coloured crusted erosions

Question 37

what is the causative organism in impetigo

Answer 37

staph aureus

Question 38

how is impetigo treated

Answer 38

Topical antibacterial (fucidin) or Oral antibiotic (flucloxacillin)

Question 39

what virus causes Molluscum contagoisum

Answer 39

Molluscipox virus

Question 40

is Molluscum contagoisum benign

Answer 40

yes - no treatment

Question 41

how does Molluscum contagoisum manifest

Answer 41

Tiny pale, pearly papules, umbilicated centre

Question 42

how is Molluscum contagoisum treated

Answer 42

reassurance

can use crythrotharpy to activate immune system

Question 43

what cream can be used to treat warts

Answer 43

5% potassium hydroxide

Question 44

what are the most common viral warts in kids

Answer 44

verrucas - on sole of foot

Question 45

what virus causes viral warts

Answer 45

human papilloma virus (HPV)

Question 46

how long do viral warts take to resolve

Answer 46

90% in 24 months

Question 47

what are associated symptoms with viral exanthema

Answer 47

Fever, malaise, headache then rash kicks off (maculopapular over trunk)

Question 48

what virus causes chicken pox

Answer 48

varicella - zoster virus

Question 49

when does chicken pox not give you lifelong immunity

Answer 49

immunocompromised are susceptible at all times

Question 50

how does chicken pox manifest

Answer 50

Red papules (small bumps) progressing to vesicles (blisters) often start on the trunk.
Itchy. Associated with viral symptoms

Question 51

what is the incubation period for chicken pox

Answer 51

10-21 days

Question 52

how long is chicken pox contagious for

Answer 52

1-2 days before rash appears and until all lesions have crusted over

Question 53

what are rare complications of chicken pox

Answer 53

meningitis

encephalitis

Question 54

how is chicken pox able to be reactivated as shingles

Answer 54

lays dormant in dorsal root ganglion

Question 55

how does parvovirus manifest (slapped cheek)

Answer 55

Erythematous rash cheeks initially and then also lace like network rash (trunk and limbs) + viral symptoms

Question 56

how long can slapped cheek take to fade

Answer 56

may be 6 weeks - usually self limiting

Question 57

what people are at risk of severe paravirus and why

Answer 57

haemolytic people - virus targets red cells in bone marrow

Question 58

what virus causes hand, foot and mouth

Answer 58

Coxsackie virus A16

Enterovirus 71

Question 59

how does hand, foot and mouth manifest

Answer 59

clusters of blisters on hands, feet mouth and sometimes bum + viral symptoms

Question 60

how is hand foot and mouth treated

Answer 60

self limiting - supportive treatment

Question 61

what is Orofacial granulomatosis

Answer 61

Lip swelling and fissuring (packing of lips)

Oral mucosal lesions: ulcers and tags, cobblestone appearance

Question 62

what disease is Orofacial granulomatosis associated with

Answer 62

Crohns disease

chronic allergy

Question 63

how does erythema nodosum present

Answer 63

red, Painful, erythematous subcutaneous nodules
Over Shins; sometimes other sites
Slow resolution - like bruises, 6-8 weeks

Question 64

what are causes of erythema nodosum

Answer 64

Infections – Streptococcus (throat), Upper respiratory tract (ASO titre/ blood test)
Inflammatory bowel disease
Sarcoidosis – history of cough, serum ACE level
Drugs – OCP, Sulphonamides, Penicillin
Mycobacterial Infections
Idiopathic

Question 65

how is erythema nodosum treated

Answer 65

dermovate

Question 66

what disease is dermatitis herpetiformis linked to

Answer 66

coeliac disease

Question 67

where is dermatitis herpetiformis found

Answer 67

Extensors - Scalp, shoulders, buttocks, elbows and knees

itchy blisters

Question 68

how is dermatitis herpetiformis investigated

Answer 68

Detailed history, Coeliac screening, Skin biopsy

Question 69

how is dermatitis herpetiformis treated

Answer 69

Emollients
gluten free diet (clear up), topical steroids
dapsone (antibiotic with anti-inflammatory effect on skin)

Question 70

what is associated with urticaria

Answer 70

Associated angioedema (swelling of soft tissue swelling around ribs) (10%)

Question 71

how long may urticaria last for

Answer 71

minutes to 24 hours

Question 72

what is the difference between acute and chronic urticaria

Answer 72

acute <6weeks

chronic >6 weeks

Question 73

what are some causes of urticaria

Answer 73

Viral infection, Bacterial infection, Food or drug allergy, NSAIDS/
OPIATES (make worse), Vaccinations

Question 74

how is urticaria treated

Answer 74

withdraw triggers
Antihistamines -
Ranitidine, Montelukast
Omalizumab, Ciclosporin (immunosuppressants)

Question 75

what is a complication of Kawasaki disease

Answer 75

can get aneurysms of coronary arteries

autoimmune

Question 76

what is the incidence of congenital heart disease

Answer 76

8/1000

Question 77

what are the 8 commonest congenital heart defects

Answer 77

Ventricular Septal Defect (VSD) – most common 
Patent Ductus Arteriosus (PDA)
Atrial Septal Defect              (ASD)
Pulmonary Stenosis
Aortic Stenosis
Coarctation of the Aorta
Transposition of Great Arteries (cyanotic heart condition) 
Tetralogy of Fallot

Question 78

list some drugs that can cause congenital heart disease

Answer 78

Alcohol, Amphetamines, Cocaine, Ecstasy, Phenytoin, Lithium

Question 79

list some infections that can cause congenital heart disease

Answer 79

TORCH (Toxoplasma, others, Rubella, CMV, Herpes)

Question 80

list some maternal causes of congenital heart disease

Answer 80

Diabetes Mellitus, Systemic Lupus Erythematosus

Question 81

when is the most likely period for teratogenic insult

Answer 81

18-60 days

Question 82

what % of congenital heart disease have an underlying chromosomal problem

Answer 82

6-10%

Question 83

what congenital heart defect is associated with trisomy 21 (downs syndrome)

Answer 83

AVSD (atrio-ventricular)

Question 84

what congenital heart defect is associated with trisomy 18 (edwards syndrome)

Answer 84

VSD and PDA

Question 85

what congenital heart defect is associated with trisomy 13 (patau syndrome)

Answer 85

VSD ad ASD

Question 86

what congenital heart defect is associated with turner syndrome

Answer 86

Co-arctation of aorta, bicuspid aortic valve

Question 87

what congenital heart defect is associated with noonan syndrome

Answer 87

Pulmonary Stenosis

Question 88

what congenital heart defect is associated with Williams syndrome

Answer 88

Supravalvular AS

Question 89

what pulse is key to diagnose coarctation of aorta

Answer 89

quiet femoral pulse

Question 90

what investigations may you do for a congenital heart defect

Answer 90

• Blood Pressure
• O2 saturation, arterial BGA (invasive)
• ECG (12 lead, halter monitor, 24hrs, event monitor)
• CXR
• Echocardiogram
• Exercise tests (ECG, sO2)

Question 91

what is dextrocardia

Answer 91

heart on right side

Question 92

what is situs inverses

Answer 92

the major visceral organs are reversed or mirrored from their normal positions

Question 93

BT shunt, balloon valvoplasty, Prostaglandin infusion, pulmonary banding
are all examples of what

Answer 93

operative palliative procedures

Question 94

which L valve open at the start of diastole

Answer 94

mitral

Question 95

which L valve opens at the start of systole

Answer 95

aortic

Question 96

where is best to listen to hear aortic murmurs

Answer 96

upper right and left sternal border

Question 97

where is best to listen to her mitral murmurs

Answer 97

lower left sternal border and apex

Question 98

what percentage of heart murmurs are innocent

Answer 98

70-80%

Question 99

what are the main 4 types of innocent murmurs

Answer 99

Still’s murmur, Pulmonary outflow murmur, Carotid/Brachiocephalic Arterial Bruits

Question 100

in what stage of the cycle are innocent murmurs usually heard

Answer 100

systolic

venous hum continuous

Question 101

do children with innocent murmurs have other signs of heart disease

Answer 101

no - generally well

Question 102

are innocent murmurs localised

Answer 102

yes - don’t radiate far from where loudest

Question 103

how do innocent murmurs change on exercise

Answer 103

louder

Question 104

which is the most common type of innocent murmur

Answer 104

still’s murmur

Question 105

describe stills murmur

Answer 105

LV outflow murmur

Soft systolic; vibratory, musical,”twangy”

Question 106

what age is stills murmur most common in

Answer 106

2-7 years

Question 107

where is stills murmur loudest

Answer 107

apex

left sternal border

Question 108

when is stills murmur loudest

Answer 108

supine position

with exercise

Question 109

describe a pulmonary outflow murmur

Answer 109

right ventricular outflow - Soft systolic, vibratory

Question 110

what age is a pulmonary outflow murmur most common in

Answer 110

8-10 years

Question 111

where is a pulmonary outflow murmur heard loudest

Answer 111

Upper left sternal border, well localised, not radiating to back

Question 112

when is a pulmonary outflow murmur loudest

Answer 112

supine position

with exercise

Question 113

what children is pulmonary outflow murmur most common in

Answer 113

thin / narrow chest

Question 114

which age does Carotid/Brachiocephalic Arterial Bruits affect most

Answer 114

2-10 years

Question 115

where are Carotid/Brachiocephalic Arterial Bruits heard loudest

Answer 115

Supraclavicular, radiates to neck

Question 116

when do Carotid/Brachiocephalic Arterial Bruits get louder

Answer 116

exercise, decreases on turning head or extending neck

Question 117

what age does venous hum occur in

Answer 117

3-8 years

Question 118

describe venous hum

Answer 118

Continuous murmur, sometimes with diastolic accentuation

Soft, indistinct

Question 119

where is venous hum heard loudest

Answer 119

supraclavicular

Question 120

when can you hear a venous hum

Answer 120

Only in upright position, disappears on lying down or when turning head

Question 121

what are the 3 main types of ventricular septal defect

Answer 121

subaortic
perimembranous
muscular

Question 122

what direction is the shunt in VSD

Answer 122

L–> R (acynotic)

Question 123

where is the hole in a VSD

Answer 123

inter ventricular septum

Question 124

describe the murmur of a VSD

Answer 124

Pansystolic murmur lower left sternal edge, sometimes with thrill

Question 125

what does a pan systolic murmur mean

Answer 125

can hear all through systole and equally as loud

Question 126

why do you hear a diastolic rumble in very large VSDs

Answer 126

relative mitral stenosis

- fluid overload form LA to LV

Question 127

what do large VSDs lead to eventually

Answer 127

biventricular hypertrophy and pulmonary hypertension

Question 128

what occurs in a VSD to lead to eisenmenger syndrome

Answer 128

PVR increases leads to increased muscle pumping leading to hypertrophy, which increases the pressure in the RA - pulmonary hypertension and leads to R L shunt

Question 129

what location of a VSD is more troublesome

Answer 129

closer to the apex

Question 130

what are signs of heart failure in a child

Answer 130

tachypnoea
tachycardia
hepatomegaly,
failure to thrive

Question 131

how would a large VSD present at birth

Answer 131

signs of heart failure
tachypnoea (pulmonary congestion from LVSD),
tachycardia (reduced SV),
hepatomegaly
failure to thrive (high calorie expenditure).

Question 132

what is a babies biggest exercise

Answer 132

feeding

Question 133

how would you investigate a VSD

Answer 133

longitudinal echocardiogram with coulured Doppler

Blue and red direction of flow with relation to the ultrasound probe, Yellow and green = turbulence

Question 134

what is the treatment of a VSD

Answer 134

VSD closure - Amplatzer device – plug whole and endocardium grows over
Patch closure – usually pericardium graft

Question 135

what do atrial septal defects usually so

Answer 135

close spontaneously

Question 136

how may an atrial septal defect be diagnosed in adulthood

Answer 136

atrial fibrillation, heart failure or pulmonary hypertension

Question 137

what way is the shunt in atrial septal defects

Answer 137

L > R - acynotic

Question 138

what are signs of a atrial septal defect

Answer 138

Wide fixed splitting of 2nd heart sound, pulmonary flow murmur

Question 139

what congenital heart defect is seen in trisomy 21

Answer 139

low ASD
high VSD
all near valves

Question 140

what are the 4 things of teratology of fallot

Answer 140

1) pulmonary stenosis
2) right ventricular hypertrophy
3) High VSD
4) Overriding aorta – sticks into RV

Question 141

what direction of shunt leads to cyanosis

Answer 141

R > L

Question 142

what shape is the heart on xray in teratology of fallot

Answer 142

boot shaped

Question 143

when can a full correction of teratology of fallot be done

Answer 143

6 months

before palliative - connect subclavian arteries with pulmonary arteries to create shunt

Question 144

what are symptoms of severe aortic stenosis in children

Answer 144

reduced exercise tolerance, exertional chest pain, syncope

Question 145

describe the murmur of aortic stenosis

Answer 145

Ejection (descend and crescendo) systolic murmur upper right sternal border, radiation into carotids. Suprasternal thrill is pathopneumonic aortic stenosis

Question 146

how would you treat aortic stenosis

Answer 146

Balloon aortic valvuloplasty treats

Question 147

what vessel gives oxygenated blood to the foetus

Answer 147

umbilical vein

Question 148

where is foramen ovale

Answer 148

between RA and LA

Question 149

what causes the ductus arteriousus to close within 24-48 hours

Answer 149

prostaglandin release

Question 150

what is the treatment of PDA in pre-term infants

Answer 150

fluid restriction/ diuretics, prostaglandin inhibitors (Indomethacin, Ibuprofen), surgical ligation

Question 151

what may delayed closure of ductus arteriosus

Answer 151

coarctation of aorta

Question 152

what is a coarctation of the aorta

Answer 152

kink in wall - normally descending arch where ductus arteriosus would enter

Question 153

what is the management of co-arctation of the aorta

Answer 153

Re-open PDA with Prostaglandin E1 or E2 - stabilise
Resection co-arctation with end-to-end anastomosis
Subclavian patch repair (fix hyperplasia of aortic arch)
Balloon Aortoplasty – risk of re-stenosis

Question 154

what occurs in transposition of great arteries that makes the baby cyanotic

Answer 154

aorta comes out of RV and PA comes out of LV so there is no systemic oxygenation occurs

Question 155

what will a baby with transposition of the great arteries need to survive

Answer 155

large VSD, PDA or patent foramen ovale

Question 156

what is given at birth when a baby has transposition of the great arteries

Answer 156

Umbilical venous catheter and prostaglandin infusion at birth to keeps PDA open

Question 157

what procedure is used to treat transposition of the great arteries

Answer 157

Raskind’s atrial septostomy procedure

Question 158

what tests give an actual indication of how the liver is functioning

Answer 158

Coagulation - Prothrombin time (PT)/INR, APTT
Albumin
Bilirubin
(Blood glucose) - glycogen store
(Ammonia) – clearance of toxic metabolites

Question 159

what does a split bilirubin tell the difference between

Answer 159

Direct (conjugated) + Indirect (unconjugated)

Question 160

why are ALT/ AST not that specific to liver disease

Answer 160

also elevated in muscular disease

alanine aminotransferase/aspartate aminotransferase

Question 161

what is the most common manifestation of paediatric liver disease

Answer 161

jaundice

Question 162

list symptoms/ signs of paediatric liver disease

Answer 162

Growth failure Ascites
Encephalopathy Varices with portal hypertension
Spider naevi Muscle wasting from malnutrition
Splenomegaly with portal hypertension Hypersplenism
Bruising with petechiae Hepatorenal failure
Clubbing Rickets secondary to vit D deficiency
Loss of fat stores secondary to malnutrition Peripheral neuropathy
Hypertonia

Question 163

what is jaundice

Answer 163

yellow discolouration of skin and tissues due to accumulation of bilirubin
Usually most obvious in sclera of eyes

Question 164

at what bilirubin is jaundice usually most visible

Answer 164

total bilirubin >40-50 umol/l

Question 165

is jaundice at < 24 hours old normal

Answer 165

no - always pathological

Question 166

what are causes of jaundice < 24 hours old

Answer 166

sepsis , haemolysis

Question 167

what are some causes of jaundice from 24hrs - 2 weeks (intermediate)

Answer 167

Physiological, Breast milk, Sepsis, Haemolysis

Question 168

what are some causes of jaundice over 2 weeks old (prolonged)

Answer 168

Extrahepatic obstruction, Neonatal hepatitis, Hypothyroidism, Breast milk

Question 169

does physiological jaundice last > 2 weeks (prolonged)

Answer 169

no

Question 170

describe the process of erythrocytes into unconjugated bilirubin

Answer 170

post mature erythrocytes > haemorrhage (in reticuloendothelial system) > biliverdin > unconjugated bilirubin (biliverdin reductase)

Question 171

is unconjugated bilirubin soluble or insoluble

Answer 171

insoluble

Question 172

what enzyme and where is biliverdin converted into unconjugated bilirubin

Answer 172

by biliverdin reductase

all tissues of body

Question 173

how does unconjugated bilirubin travel to the liver

Answer 173

bound to albumin

Question 174

what enzyme conjugates bilirubin in the liver

Answer 174

UDP glucuranyl transferase

Question 175

is conjugated bilirubin water soluble

Answer 175

yes

Question 176

how ics conjugated bilirubin excreted

Answer 176

by kidneys
bacterial fermantation in gut to be excreted as dark faced
recycled in the entrohepatic circulation

Question 177

is the bilirubin conjugated or not in pre-hepatic jaundice

Answer 177

unconjugated

Question 178

is the bilirubin conjugated or not in post-hepatic jaundice

Answer 178

mostly conjugated

Question 179

is the bilirubin conjugated or not in intra-hepatic jaundice

Answer 179

mixed unconjugates and conjugated

Question 180

is physiological jaundice conjugated or unconjugated

Answer 180

unconjugated

Question 181

why do babies get a physiological jaundice in there first couple of weeks

Answer 181

¥ Shorter RBC life span in infants (80-90 days) – foetal haemoglobin
¥ Relative polycythaemia (Hb 180-200 – breaking down huge amount of red cells and high levels of unconjugated bilirubin)
¥ Relative immaturity of liver function

Question 182

when does a physiological jaundice develop

Answer 182

AFTER the first day of life

Question 183

what kind of jaundice is breast milk jaundice

Answer 183

intrahepatic unconjugated

Question 184

how would you investigate for sepsis in a jaundice baby

Answer 184

urine, blood cultures, torch screen

Question 185

what things may cause haemolysis in a jaundice baby

Answer 185

ABO incompatibility
Rhesus disease
Bruising
Red cell membrane/ enzyme defects

Question 186

how would you investigate for ABO/ rhesus in a jaundice baby

Answer 186

blood group, direct coombs test

Question 187

how would you investigate for red cell membrane defects (spherocytosis) in a jaundice baby

Answer 187

blood film

Question 188

how would you investigate for G6PD red cell enzyme defects in a jaundice baby

Answer 188

G6PD assay

Question 189

name to genetic diseases that may cause jaundice in a baby

Answer 189

Gilberts disease

Crigler najjar

Question 190

what is crigler- najjar syndrome

Answer 190

absence of G6PD - severe

Question 191

how would you investigate for genetic diseases that may cause jaundice in a baby

Answer 191

genotype/ phenotype

Question 192

what is a sever complication of unconjugaed jaundice

Answer 192

kernicterus

Question 193

how does kernicterus occur

Answer 193

unconjugated bilirubin is fat soluble so can cross the blood brain barrier and deposits in brain (basal ganglia)

Question 194

where in the brain does unconjugated bilirubin deposit to cause kernicterus

Answer 194

basal ganglia

Question 195

what are early signs of kernicterus

Answer 195

encephalopathy – poor feeding, lethargy, seizures

Question 196

what are some late consequences of kernicterus

Answer 196

¥ severe choreoathetoid cerebral palsy, learning difficulties, sensorineural deafness (lifelong)

Question 197

how does phototherapy treat unconjugaed jaundice

Answer 197

visible light converts bilirubin to water soluble photo isomers that can be excreted in the urine

Question 198

what is the treatment of unconjugaed jaundice

Answer 198

phototherapy

Question 199

does the treatment of unconjugated jaundice involve UV light

Answer 199

no - visible (450nm)

Question 200

how will a child need to be treated if they are above the threshold on a growth chart

Answer 200

blood transfusion

Question 201

what is prolonged jaundice

Answer 201

Jaundice persisting beyond 2 weeks of life (3 weeks for preterm infants)

Question 202

is conjugated jaundice in infants normal

Answer 202

no - always investigate

Question 203

what is the most important test in prolonged jaundice

Answer 203

split bilirubin

Question 204

what should you always assess in infants with prolonged jaundice

Answer 204

stool colour

Question 205

what should conjugated jaundice be considered until proven otherwise

Answer 205

biliary atresia

Question 206

how should you investigate for biliary atresia

Answer 206

split bilirubin, stool colour, ultrasound, liver biopsy – special liver centre

Question 207

what colour is the stool is biliary atresia

Answer 207

pale (dark urine)

Question 208

list cause of prolonged jaundice

Answer 208

biliary atresia
choledochal cyst
alagille syndrome
neonatal hepatitis

Question 209

list some causes of neonatal hepatitis

Answer 209

Alpha-1 antitrypsin deficiency
Metabolic – galactosaemia, tyrosinaemia
Urea cycle defects
Haemochromatosis
Glycogen storage disorders
Hypothyroidism
Viral hepatitis
Parenteral nutrition – prolonged – after bowel surgery, neo-natal unit

Question 210

what is seen in alagille syndrome

Answer 210

Intrahepatic cholestasis, dysmorphism, congenital cardiac disease

Question 211

what is biliary atresia

Answer 211

Congenital fibro-inflammatory disease of bile ducts leading to destruction of extra-hepatic bile ducts with no flow and obstruction (obstructive jaundice)

Question 212

why does biliary atresia need to be identified immediately

Answer 212

rapid progression to liver failure

Question 213

what is the treatment for biliary atresia

Answer 213

kasai portoenterostomy

Question 214

when is a kasai portoenterostomy work best

Answer 214

performed before 60 days (<9 weeks)

Question 215

what should you ask a child complaining of constipation

Answer 215

how often? how hard? is it painful? has there been a change?

What is normal stool frequency ?

Question 216

what are some signs and symptoms of constipation

Answer 216

• Poor appetite (feel full)
• Irritable
• Lack of energy, look pale
• Abdominal pain or distension
• Withholding or straining – scared of opening their bowels
• Diarrhoea overflow
Only feel batter after they have passed a stool

Question 217

what are some social causes of constipation

Answer 217

poor diet - insufficient fluids 
excessive milk 
not enough plants/ fibre 
poor potty training 
fear of school toilet

Question 218

what medications can cause constipation

Answer 218

opiates, codeine, gaviscon, antispasmodics

Question 219

what are some organic causes of constipation

Answer 219

anal stenosis, anterior anus, Hirschsprung disease, lead poisoning, hypothyroidism

Question 220

describe the viscous cycle of constipation

Answer 220

• Toddler had a hard poo from dehydration/ infection that has caused pain > forms reflex and instinct not to do something painful again ie not touch fire > colon moves stool and sucks out water and salt, arrives in rectum sends message to brain > toddler doesn’t want to so clenches external sphincters to not let out > makes a larger hard stool that is more painful to pass

Question 221

what occurs in mega rectum

Answer 221

back passage gets stretched as they hold internal sphincter open, leading to soiling (overflow diarrhoea)

Question 222

how would you investigate for megarectum

Answer 222

• colonic marker study - take markers Monday, Tuesday, Wednesday and xray on Friday would normally have none but constipated have all in megaractum
• Abdominal examination – sub peritoneal mass, PR

Question 223

what is the dietary treatment of constipation

Answer 223

Increase fibre, fruit, vegetables, fluids

decrease milk

Question 224

list some ways to reduce psychological causes of constipation

Answer 224

Make going to the toilet a pleasant experience
Correct height – stool or lower toilet/ Not cold/ School toilets
Avoid punitive behaviour from parents
Reward ‘good’ behaviour – retrain brain
General praise/ Star charts – after each meal try push out

Question 225

what drugs can be given to soften the stool and stimulate defecation in constipation

Answer 225

osmotic laxatives (lactulose) 
stimulant laxatives (senna, picolax) 
isotonic laxatives (movicol)

Question 226

why do children with mega rectum get a lot of urinary infections and retention

Answer 226

rectum presses on bladder

Question 227

how do you treat impaction

Answer 227

• Empty impacted rectum
Empty colon
Maintain regular stool passage
Slow weaning off treatment

Question 228

when treating constipation what dose of medication and length of treatment os needed

Answer 228

enough to go 3x a day

duration normally linked to duration of treatment

Question 229

does paediatric IBD affect more boys or girls

Answer 229

boys

Question 230

what is the crohns triad

Answer 230

abdominal pain, diarrhoea, bleeding

Question 231

list some presenting features of crohns disease

Answer 231

abdominal pain, diarrhoea, bleeding but symptoms depend where the disease is
Marked effect on growth as it puts you off eating (anorexia)
Erythema nodosum – raised plaques on shins

Question 232

list some presenting features of ulcerative colitis

Answer 232

bloody diarrhoea usually with mucus, relapsing remitting course

Question 233

name a skin manifestation of IBD

Answer 233

erythema nodosum - raised plaques on shins

Question 234

what is IBDU

Answer 234

IBD unclassified - cant differentiate between UC and crohns – pancolytis (full inflammation), more intensive/ severe

Question 235

what test should you do in IBD to exclude infection

Answer 235

stool culture

Question 236

what lab investigations should you do for IBD

Answer 236

Full blood count & ESR – Anaemia, Thrombocytosis, Raised ESR
Biochemistry - Stool calprotectin, Raised CRP, Low Albumin (losing protein)
Microbiology - No stool pathogens

Question 237

what are radiological investigations for IBD

Answer 237

MRI / MRE water (drink lots of water to open up small bowel)
Barium meal and follow-through (younger kids)

Question 238

if a granuloma is found on mucosal biopsy of the colon, what is the diagnosis

Answer 238

Crohns disease

Question 239

where is ulcerative colitis usually worst

Answer 239

left colon - continuous inflammation

Question 240

where should you always check when doing a colonoscopy for potential IBD

Answer 240

terminal ileum

ileo-colonoscopy

Question 241

why do children not like being on steroids for IBD

Answer 241

limit growth, make spotty, gain weight, greasy hair

Question 242

what is the medical treatment of IBD

Answer 242

Anti-inflammatory
Immuno-suppression – steroids
Biologics (infliximab)
Immune modulation – azathioprine

Question 243

what is the first line treatment for crohns in kids

Answer 243

polymeric diet - diet of milkshakes for 8 weeks- changes gut flora, improves nutrition

Question 244

what is the end stage in treatment of UC

Answer 244

colectomy

Question 245

how is crohn’s treated

Answer 245

Polymeric diet > oral prednisolone > steroid sparing agents – azathioprine/ 6MP or methotrexate > biologics infliximab or adalibumab / surgery

Question 246

how is ulcerative colitis treated

Answer 246

5 ASA (anti- inflammatory) or oral corticosteroids to induce remission > maintain with 5 ASA or immune modulation drug like azathioprine > surgery

Question 247

what is the pre-ejection phase of vomititng

Answer 247

Pallor, Nausea, Tachycardia

Question 248

what is the ejection phase of vomititng

Answer 248

Retch, Vomit

Question 249

what is the post ejection phase of vomititng

Answer 249

weakness, lethargy, floppy, shivering

Question 250

what is the most common thing that stimulates the vomiting centre in children

Answer 250

Infection - UTI in children, meningitis/ encephalitis, cellulitis, sepsis (non specific)

Question 251

list things that stimulate the vomiting centre in children

Answer 251

¥	Infection 
¥	Enteric pathogens
¥	Intestinal inflammation
¥	Metabolic derangement
¥	Head injury
¥	Visual stimuli
¥	Middle ear stimuli

Question 252

what age do children present with pyloric stenosis

Answer 252

4-12 weeks

boys> girls

Question 253

how do people with pyloric stenosis present

Answer 253

Projectile non-bilious vomiting, Weight loss, Dehydration +/- shock

Question 254

how would you investigate pyloric stenosis

Answer 254

Test feed
Blood gas
Ultrasound – long thickened muscles at pylorus

Question 255

what confirms the diagnosis of pyloric stenosis

Answer 255

metabolic alkalosis on blood gas

Question 256

what are the characteristic electrolyte disturbances of pyloric stenosis

Answer 256

Metabolic alkalosis (↑pH)
Hypochloraemia (↓Cl)
Hypokalaemia (↓K)

Question 257

how is pyloric stenosis managed

Answer 257

Fluid resuscitation

Surgery – Ramstedts pyloromyotomy – removes pyloris

Question 258

what is seen after a test feed for pyloric stenosis

Answer 258

• Palpation of “olive” tumour - abdomen expands with feed
  Visible gastric peristalsis (pushes milk through tight pylorus)
  Projectile non bilious vomiting

Question 259

why is there a metabolic alkalosis in pyloric stenosis

Answer 259

loss of hydrochloric acid with vomiting

Question 260

what is the name of the surgical procedure that treats pyloric stenosis

Answer 260

ramstedts pyloromyotomy

Question 261

what is bilous vomiting until proven otherwise

Answer 261

intestinal obstruction (Atresia)

Question 262

give causes of billous vomiting

Answer 262

Intestinal atresia (in newborn babies only) Malrotation +/- volvulus
Intussusception – bowel loops
Ileus – infection slows down
Crohn’s disease with strictures – mainly small bowel

Question 263

what is effortless vomiting almost always due to

Answer 263

gastro-oesophageal reflux

Question 264

what are causes of prolonged reflux

Answer 264

o Cerebral palsy
o Progressive neurological problems
o Oesophageal atresia +/- TOF operated (break in connection to oesophagus, fistula)
o Generalised GI motility problem

Question 265

how may gastro-oesophageal reflux present in children

Answer 265

vomiting, haematemesis
feeding problems/ failure to thrive
apnoea, cough, wheeze

Question 266

what is sandifer’s syndrome

Answer 266

association of gastro-oesophageal reflux disease with spastic torticollis and dystonic body movements. Nodding and rotation of the head, neck extension, gurgling sounds, writhing movements of the limbs, and severe hypotonia –these positionings provide relief from discomfort caused by acid reflux

Question 267

what is the usual course of gastro-oesophageal reflux

Answer 267

self limiting - starts at 2 months and gets worse 4-5 months then weans when walking about

Question 268

when would you investigate gastro-oesophageal reflux further

Answer 268

respiratory / feeding problems

Question 269

what is the point of doing a barium swallow

Answer 269

look at anatomy - dysmotility, hiatus hernia, reflux into oesophagus, gastric emptying, strictures

Question 270

how may you investigate gastro-oesophageal reflux

Answer 270

videofluroscopy & barium meal
pH study
Endoscopy
trial of feeding

Question 271

what are disadvantages of a pH study

Answer 271

only detects acid reflux

may be unpleasant for child

Question 272

what does and oesophageal impedance study tell you

Answer 272

how far acid is coming up

can pick up other things that aren’t acid

Question 273

when is an endoscopy done for gastro-oesophageal reflux

Answer 273

if not resolved at 2 years old

Question 274

what is the best test for oesophagitiis

Answer 274

endoscopy

Question 275

what are disadvantage of doing a trial of feeding to investigate gastro-oesophageal reflux

Answer 275

NG tube and hospitalisation required

Question 276

what are disadvantages of videofluoroscopy and barium meal

Answer 276

may miss reflux
radiation
cause aspiration

Question 277

what feeding advice is given to those with GORD

Answer 277

♣ Thickeners for liquids (keeps it down)
♣ Appropriateness of foods – Texture, Amount
♣ Behavioural programme - Oral stimulation, Removal of aversive stimuli
♣ Feeding position – 45 degrees, head end raised

Question 278

what nutritional support is given to those with GORD

Answer 278

♣ Calorie supplements
♣ Exclusion diet (milk free)
♣ Nasogastric tube

Question 279

what is the medical treatment of GORD

Answer 279

♣ Feed thickener
♣ Gaviscon
♣ Prokinetic drugs
♣ Acid suppressing drugs- H2 receptor blockers – rantitidine, Proton pump inhibitors – omeprazole

Question 280

what are indications for surgery to treat GORD

Answer 280

Failure of medical treatment
Persistent: Failure to thrive
Aspiration – recurrent apnoeic episodes
Oesophagitis – mainly neurodescemities

Question 281

what surgical procedure is done to treat GORD

Answer 281

Nissen fundoplication - wraps fundus around LOS

Question 282

what may be complications of a Nissen fundoplication

Answer 282

bloating, dumping, retching

Question 283

how much water is usually lost in the faeces a day

Answer 283

<200ml

9L fluid enters duodenum - 1.5L gets to colon

Question 284

why does the small intestine have such a massive surface area

Answer 284

Mucosal folds + Villi + microvilli

Question 285

what things are secreted by the small intestine

Answer 285

Water for fluidity/enzyme transport/absorption
Ions e.g. duodenal HCO3-
Defence mechanism against pathogens/harmful substances/antigens

Question 286

what is the definition of chronic diarrhoea

Answer 286

4 or more stools per day, For more than 4 weeks

Question 287

how long do acute, persistent and chronic diarrhoea last

Answer 287

acute - <1 weeks
2-4 - persistent
>4 - chronic diarrhoea

Question 288

what motility disturbances may cause chronic diarrhoea

Answer 288

Toddler Diarrhoea
Irritable Bowel Syndrome (functional)
Congenital hyperthyroidism
Chronic intestinal pseudo-obstruction

Question 289

give causes of secretory diarrhoea

Answer 289

Active secretion;
Acute Infective Diarrhoea e.g. cholera
Inflammatory Bowel Disease

Question 290

give causes of osmotic diarrhoea

Answer 290

malabsorption of nutrients ;
Food Allergy
Coeliac Disease
Cystic Fibrosis (fat malabsorption from pancreatic insufficiency)

Question 291

what causes osmotic diarrhoea

Answer 291

Movement of water into the bowel to equilibrate osmotic gradient
Mechanism of action of lactulose/movicol
Generally accompanied by macroscopic and microscopic intestinal injury

Question 292

what predominantly drives the intestinal fluid secretion in secretory diarrhoea

Answer 292

active Cl- secretion via CFTR

Question 293

what is secretory diarrhoea commonly associated with

Answer 293

toxin production from Vibrio cholerae and enterotoxigenic Escherichia coli

Question 294

give causes of inflammatory diarrhoea

Answer 294

• Malabsorption due to intestinal damage
• Secretory effect of cytokines
• Accelerated transit time in response to inflammation
• Protein exudate across inflamed epithelium

Question 295

is stool reducing substance positive in osmotic or secretory diarrhoea

Answer 295

osmotic (digestion of carbs)

Question 296

why does diarrhoea occur in pancreatic disease

Answer 296

lack of lipase and resultant steatorrhoea

Question 297

are electrolytes high in osmotic or secretory diarrhoea

Answer 297

secretory

Question 298

is nocturnal diarrhoea ever normal

Answer 298

no - always pathological

Question 299

how would you determine between secretory and osmotic diarrhoea

Answer 299

stool culture

appearance

Question 300

what happens to osmotic diarrhoea when food is stopped

Answer 300

also stops

Question 301

what are coeliac people allergic to

Answer 301

gluten - wheat, rye, barley

Question 302

what genes are associated with coeliac disease

Answer 302

HLA DQ2, DQ8

Question 303

give symptoms of coeliac disease

Answer 303

Abdominal bloatedness Diarrhoea
Failure to thrive Short stature
Constipation Tiredness
Dermatitis herpatiformis – vesicular skin rash

Question 304

what skin condition is associated with coeliac disease

Answer 304

dermatitis herpatiformis

Question 305

what serological tests can be done for coeliac disease

Answer 305

Anti-tissue transglutaminase
Anti-endomysial – specific
Anti-gliadin
IgA

Question 306

what test should always be done with coeliac screening

Answer 306

IgA - may be naturally deficient

Question 307

what is the gold standard test for coeliac disease if the screening is positive

Answer 307

duodenal biopsy

Question 308

what are the histological findings of a duodenal biopsy for coeliac disease

Answer 308

Lymphocytic infiltration of surface epithelium
(inflammatory cells)
partial /total villous atrophy,
crypt hyperplasia

Question 309

when can young children be diagnosed coeliac without a biopsy

Answer 309

Symptomatic children
Anti TTG >10 times upper limit of normal
Positive anti endomysial antibodies
HLA DQ2, DQ8 positive

Question 310

what is the treatment for coeliac disease

Answer 310

gluten free diet for life

Question 311

what are complications of coeliac disease

Answer 311

osteoporosis, infertility and Increased risk of rare small bowel lymphoma

Question 312

what leads growth in infants

Answer 312

nutrition

Question 313

what leads growth in children

Answer 313

growth hormone

Question 314

what leads growth in puberty

Answer 314

sex steroid

Question 315

how much weight should a baby gain per week in the first 3 months

Answer 315

200g

Question 316

how much weight should a baby gain per week in the first 3-6 months

Answer 316

150g

Question 317

how much weight should a baby gain per week in the first 6-9 months

Answer 317

100g

Question 318

how much weight should a baby gain per week in the first 9-12 months

Answer 318

75-50g

Question 319

how much weight does a child gain from 1- puberty a year

Answer 319

1kg

Question 320

why do babies need so many calories

Answer 320

Growth (35%)
Physical activity
Thermogenesis
Tissue maintenance

Question 321

why can infants rapidly become malnourished

Answer 321

don’t have much reserve

Question 322

how many calories to infants need a day

Answer 322

100kcal and 2g protein/kg/day

Question 323

why is breast feeding better for full term babies

Answer 323

well tolerated
less allergenic 
Low renal solute load
Ca:PO4, LCP Fatty Acids
Improves cognitive development
Reduces infection - Macrophages and lymphocytes, Interferon, lactoferrin (iron), lysozyme, Bifidus factor

Question 324

what are benefits of breast feeding to an infants immune system

Answer 324

develops active immunity
reduces infection
antigen load minimal

Question 325

why is breast feeding not suitable for pre-term babies

Answer 325

need more calories - + fortifiers

Question 326

what are disadvantages of formula feeding

Answer 326

No anti-infection properties
Risk of contamination (food poisoning)
High antigen load
Expensive

Question 327

what are advantages of formula feeding

Answer 327

No transition of BBVs/drugs
Doesn’t need mum (working mum) 
Accurate feed volumes
Provides Vit K
Less jaundice (breast milk jaundice)

Question 328

when should mothers initiate breast feeding (unicef baby friendly 10 steps)

Answer 328

first 30 mins fo birth

Question 329

what is the base of most formula feeds

Answer 329

cows milk

Question 330

what composition of formula milk should be used for 0-6 months

Answer 330

first milk - more like breast

Question 331

why is real cows milk not suitable as main drink for an infant

Answer 331

contains no iron

Question 332

when may a baby receive a specialised formula feed

Answer 332

for cows milk protein allergy
pre term - nutrient – need calories
disease specific

Question 333

what is the most common food allergy in <2s

Answer 333

Cows milk protein allergy

Question 334

what are the majority of cow’s milk protein allergy reactions

Answer 334

delayed, non IgE reactions (develop over days) eg vomiting,diarrhoea,abdominal discomfort/ distension, eczema

Question 335

what is the test for cows milk protein allergy

Answer 335

CMP exclusion - should get better

re challenge after 6 months

Question 336

what are the steps to reintroduce milk into a babies diet to build tolerance

Answer 336

iMAP milk ladder - biscuits/ cookies, muffins, pancakes, cheese, yoghurt, milk

Question 337

what is the first line treatment for cows milk protein allergy

Answer 337

Extensively hydrolysed protein feeds

second line amino acid

Question 338

are lactose free milks suitable for cows milk protein allergy

Answer 338

no - only free of lactase

Question 339

is lactose intolerance an allergy

Answer 339

no - reduced levels of lactase enzyme

Question 340

what is a downside of soya milk

Answer 340

phytoestrogens

Question 341

when is soya milk indicated

Answer 341

Milk allergy when hydrolysed formulae refused
Vegan families, if not breast fed
Consider for children>1 year still on milk free diet

Question 342

why is rice milk not advised for children < 5

Answer 342

arsenic acid

Question 343

what is the problem with organic milks for babies

Answer 343

not calcium fortified

Question 344

what is weaning and when does it start

Answer 344

Transition from milk to a mixed diet - around 6 months

Question 345

what is a benefit of weaning socially

Answer 345

Encourage tongue and jaw movements in preparation for speech and social interaction

Question 346

what children are at risk of vit D deficiency

Answer 346

dark skin
breast feeding mum not on vit D
Scottish in winter

Question 347

how much calcium fortified milk is needed to meet requirements

Answer 347

400-500ml

Question 348

what should be taken <500ml calcium fortified substitute

Answer 348

Alliance calcium liquid or (if >3y) Calcium softies

for breast feeding mums Accrete or Cacit D3

Question 349

list some factors that influence height

Answer 349

Age Sex (M>F by 15cm)
Race Nutrition
Parental heights Puberty (early not as tall)
Skeletal maturity (bone age) General health
Chronic disease –inflammatory Specific growth disorders – affect bones/ hormone regulated
Socio-economic status Emotional well-being

Question 350

when is max height reached in boys and girls

Answer 350

end of puberty
girls- 14/15
boys - 16/18

Question 351

how do you measure a baby under 2

Answer 351

length -lying down facing up

head circumference

Question 352

how do you measure a child over 2

Answer 352

height - stadiometre against wall

Question 353

when may a sitting height be measured

Answer 353

height/ body disproportion e.g achondroplasia, after spinal irradiation

Question 354

how do you measure a childs head circumference

Answer 354

Tape round forehead and occipital prominence (maximal circumference)

Question 355

how is the mid parental height (MPH calculated (potential height for child)

Answer 355

average height of Mum and dad – correct parent of opposite sex i.e. give mum 15 cm if boy)
target centile 10cm +/- MPH

Question 356

what scan do you do to assess a childs bone age

Answer 356

TW 20

wrist

Question 357

what things can confuse TW 20 scan

Answer 357

osteochondrodysplasic bones

sever osteopenia

Question 358

what scan can be done to assess growth potential in a child

Answer 358

TW 20

Question 359

what would you ask in the history of a small stature child

Answer 359

• Birth weight and gestation (born small may remain small)
• PMH – chronic disease, intercurrent illnesses
• Family history (height history) /social history/schooling (developmental delay)
• Systematic enquiry – subtle symptoms of growth disorders
• Dysmorphic features – short stature syndromes
• Systemic examination including pubertal assessment – growth potential, crohns, coeliac etc

Question 360

what are some indications to refer a child to a specialist for growth disorders

Answer 360

Extreme short or tall stature (off centiles) – below 0.4th, above 99.6th
Height below target height – disproportional to parents
Abnormal height velocity (crossing centiles)
History of chronic disease
Obvious dysmorphic syndrome
Early/late puberty

Question 361

give non pathological causes of short stature

Answer 361

familial
constitution delay of growth
SGA/ UGR

Question 362

what may kids with IUGR/ SGA benefit from

Answer 362

growth hormone

Question 363

give pathological causes of short stature

Answer 363

Undernutrition chronic illness (JCA, IBD, Coeliac)
Iatrogenic (steroid) Psychological and social deprivation (anorexia)
Hormonal (GHD, hypothyroid) Syndromes (Tuner, Prader-Willi)

Question 364

give features of turners syndrome

Answer 364

wide carrying angle, infantile , shielded chest, wide nipple, webbing of neck, dystrophic/ small nails

Question 365

what are first line investigations for short stature

Answer 365

FBC/ ferritin
U&E, CRP, LFT
coelaic/ IgA
hormones - IGF-1, TFT, cortisol, prolactin 
karyotype

Question 366

what number of the tanner scale is pre pubertal

Answer 366

1

2 starts puberty

Question 367

describe the tanner staging of puberty

Answer 367

B 1 to 5 (breast development)
G 1 to 5 (genital development)
PH 1 to 5 (pubic hair) – stimulated by LH and FSH
AH 1 to 3 (axillary hair)
T 2ml to 20ml – prader orchidometer -pubertal >4, adult 12-25ml

Question 368

what is the essential tanner stage of puberty in girls

Answer 368

Breast budding (Tanner Stage B 2)

Question 369

what is the essential tanner stage of puberty in boys

Answer 369

Testicular enlargement (Tanner Stage G2 -T 3- 4 ml)

Question 370

when is puberty considered early and delayed in boys

Answer 370

< 9

>14

Question 371

when is puberty considered early and delayed in girls

Answer 371

<8

>13 (rare)

Question 372

which sex are most commonly affected by constitutional delay of growth and puberty

Answer 372

males - FH in dad/ brothers

Question 373

what is the treatment for Constitutional Delay of Growth and Puberty (CDPG)

Answer 373

short course of testosterone

Question 374

what conditions can impair the HPG axis an lead to delayed puberty

Answer 374

septo-optic dysplasia, craniopharyngioma, Kallman’s syndrome

Question 375

what conditions of gonadal dysgenesis may lead to delayed puberty

Answer 375

Turner 45X, Klinefelter 47XXY

Question 376

what causes secondary sexual characteristics to form

Answer 376

sex steroid hormones

Question 377

what imaging must be done in central precocious puberty

Answer 377

MRI - exclude pituitary lesion

Question 378

what is the treatment of central precocious puberty

Answer 378

GnRH agonist

Question 379

what is the cause of Precocious Pseudopuberty

Answer 379

Abnormal sex steroid hormone secretion

Question 380

why must congenital adrenal hyperplasia be excluded in kids

Answer 380

risk of adrenal crisis

Question 381

what are some causes of congenital hypothyroidism

Answer 381

athyreosis/ hypoplastic/ ectopic – no gland or dysfunctional
Dyshormonogenic – dyfucntional thyroxine

Question 382

why is congenital hypothyroidism treated in first 2 weeks

Answer 382

prevent poor development, dysmorphia, severe mental retardation

Question 383

what is the most common cause of acquired hypothyroidism in children

Answer 383

autoimmune hasimotos

Question 384

how may acquired hypothyroidism present in kids

Answer 384

lack of height gain, pubertal delay (or precocity), poor school performance
+ same as adult (constipation, tiredness, skin changes)

Question 385

what centile is an overweight BMI

Answer 385

> 85th

Question 386

what skin condition is associated with obesity

Answer 386

acanthosis nigricans (insulin resistance)

Question 387

how would you examine someone for obesity

Answer 387

Weight, Height, Body mass index (BMI)
Waist circumference
Skin folds

Question 388

what drugs may cause obesity in children

Answer 388

insulin, steroids, anti-thyroid, sodium valproate

Question 389

what is the treatment of childhood obesity

Answer 389

diet and exercise

Question 390

list complications of childhood obesity

Answer 390

• Fatty liver disease (non-alcoholic steatohepatitis)
• Gallstones - Reproductive dysfunction (eg, PCOS)
• Nutritional deficiencies - Thromboembolic disease
• Pancreatitis
• Obstructive sleep apnoea
• Gastroesophageal reflux disease
• Orthopaedic problems (slipped capital femoral epiphysis, tibia vara)
• Stress incontinence
• Psychological -Left ventricular hypertrophy
• Atherosclerotic cardiovascular disease - Right-sided heart failure

Question 391

why must DKA be prevented

Answer 391

can lead to permanent neurological disability

Question 392

what are symptoms of diabetes in children(4Ts)

Answer 392

Thirsty, thinner, tired, using toilet more

Question 393

what is a return to bed wetting/ day wetting a red flag for

Answer 393

diabetes

Question 394

how may diabetes present in under 5’s

Answer 394

4Ts - Thirsty, thinner, tired, using toilet more
heavier than usual nappies
Blurred vision Candidiasis (oral, vulva) Constipation
Recurring skin infections Irritability, behaviour change

Question 395

list some symptoms of DKA

Answer 395

Nausea and vomiting
Abdominal pain 
Sweet smelling/ ketotic breath 		Drowsiness
Rapid, deep sighing respiration 
Coma

Question 396

how should you test for type 1 diabetes in a child

Answer 396

finger prick capillary glucose test

If result >11 mmol/l = diabetes

Question 397

what should you do if you suspect a child has T1DM

Answer 397

refer to local specialist - need insulin ASAP

Question 398

how do you calculate the average weight of a child

Answer 398

weight (kg) = 2 x (age +4)

Question 399

what is the average blood volume of a child (mls)

Answer 399

80mls/ kg

Question 400

when is a childs urine output highest

Answer 400

baby - 1ml/ kg/ hour then falls to normal 0.5ml/kg/ hour

Question 401

what is a childs insensible fluid loss a day (breathing, sweating, metabolism etc.)

Answer 401

20mls/ kg/ day

Question 402

how do you calculate a childs systolic BP

Answer 402

80 + ( 2 x age)

- reaches adult by 20

Question 403

when should you start CPR in a baby

Answer 403

HR <60 - they can’t increase their own CO

Question 404

why is referred pain more common in children

Answer 404

struggle to localise

Question 405

what is the problem of paracetamol having a very narrow therapeutic index in kids

Answer 405

can lead to liver failure very quickly

Question 406

why shouldnt codeine be given to kids

Answer 406

20% can’t metabolise so will have no effect and no other analgesias can be mixed, very rarely a patient can have multiple copies of the metabolic enzyme so have a massive response leading to respiratory depression

Question 407

what fluid should be given to a child for resuscitation

Answer 407

20ml/kg bolus 0.9% NaCl

Question 408

what maintenance fluid should be given to children

Answer 408

0.9% NaCl / 5% Dextrose +/- 0.15% KCl

Question 409

what is the 4,2,1, rule for administrating fluid to a child

Answer 409

4ml/kg 1st 10kg, 2ml/kg 2nd 10 kg, 1ml/kg every kg thereafter
e.g. 10 yrs = 2 x (10+4) = 28kg = 40+20+8 = 68mls/hr

Question 410

list some sentinel signs in babies

Answer 410

feed refusal 
bile vomits
grey colour (tissue perfusion)
Floppy tone
low emperature

Question 411

what is acute constant tummy pain more likely to be

Answer 411

peritonitis (peritoneal irritation)

Question 412

what is the dermatome of the umbilicus

Answer 412

T10 - midgut

Question 413

why should you ask a child if they get tummy pain on speed bumps/ movement

Answer 413

rebound pain = peritonitis

Question 414

what question should you always ask a girl with tummy pain

Answer 414

menstraul cycle and related pain

Question 415

what colour is bile

Answer 415

green

Question 416

what is tenesmus

Answer 416

feeling of incomplete evacuation e.g. due to a pelvis full of pus in appendicitis

Question 417

what investigations may you do on a child with abdominal pain

Answer 417

urine
FBC / electrolytes
Xrays - rarely

Question 418

why are X-rays rarely done in children

Answer 418

radiation is cumulative over lifetime

Question 419

what is the diagnostic test for bowel obstruction

Answer 419

Xray

Question 420

what age range is appendicitis most common in

Answer 420

<4

Question 421

what is murphys triad of appendicitis

Answer 421

pain , vomiting, fever

Question 422

which point is tender in appendicitis

Answer 422

mcBurneys - focal

Question 423

what are signs of appendicitis

Answer 423

guarding/ rebound on examination
murphy’s triad - pain , vomiting, fever
look unwell
tenderness over McBurney’s point

Question 424

what is the management of appendicitis

Answer 424

laparoscopic removal

Question 425

what are complications of appendicitis

Answer 425

abscess
mass - stuck to bowel
peritonitis

Question 426

give feature of non specific abdominal pain

Answer 426

• short duration – 24hrs - central – peri-umbilical
• constant - not made worse by movement – guarding (speedbump)
• no GT disturbance - no temperature
• site & severity of tenderness vary

Question 427

what is the management for non specific abdominal pain

Answer 427

active observation

Question 428

what does mesenteric adenitis normally follow

Answer 428

follows viral illness - URTI cold, sore throat

high temperature

Question 429

which location of pneumonia is most common in children

Answer 429

Right lower lobe

Question 430

what are signs that abdominal pain is actually pneumonia

Answer 430

sick
abdomen soft
high CRP
cough - do chest xray

Question 431

which gender are more affected by pyloric stenosis

Answer 431

males 5:1

Question 432

what kind of vomiting is seen in pyloric stenosis

Answer 432

projectile non billows vomititng

4-16 week history

Question 433

how would you manage pyloric stenosis

Answer 433

0.45 N Saline/ 5% Dextrose + KCl
0.9% Saline for NG loss
periumbilical pyloromyotomy

Question 434

at what age does malrotation +/- volvulus present

Answer 434

very early - first week

Question 435

what happens to the bowel in malrotation +/- volvulus

Answer 435

bowel twists to cause obstruction

Question 436

how would you investigate malrotation +/- volvulus

Answer 436

upper GI contrast study ASAP

Question 437

how is malrotation +/- volvulus managed

Answer 437

ASAP laparotomy to distort

Question 438

what age does itussusception usually present

Answer 438

6-15 months , rare outside

Question 439

what happens when a baby has a dying spell

Answer 439

baby goes white, floppy, stop breathing for 10s then colic stops and start crying

Question 440

how may intussusception present in a child

Answer 440

colic, dying spells, billows vomiting, poor perfusion (long capillary refill)

Question 441

what type of stool is seen in intussusception

Answer 441

bloody mucous PR (redcurrant jelly stool – mucus and blood)

Question 442

what is though to be the cause of intussusception

Answer 442

Thought to be due to enlarged peyers patches in terminal ileum causing self peristalsis

Question 443

what is the characteristic sign on US of intussusception

Answer 443

target sign (oedematous bowel)

Question 444

what is the management of intussusception

Answer 444

pneumostatic reduction  (air enema)
laparotomy

Question 445

what things are umbilical hernias associated with

Answer 445

low birth weight, down syndrome, hypothyroid, mucopolysaccharide

Question 446

what causes an umbilical hernia

Answer 446

muscles around opening to umbilical cord soft close properly

Question 447

how common are umbilical hernias

Answer 447

1 in 6

Question 448

what % of umbilical hernias resolve spontaneously

Answer 448

90%

Question 449

what are complications of umbilical hernias

Answer 449

obstruction

strangulation - blood supply cut off

Question 450

when should an umbilical hernia be repaired

Answer 450

by the age of 4 or if complications

Question 451

how can you distinguish an umbilical hernia from a para-umbilical hernia

Answer 451

points down when crying

Question 452

what things increase the size of an umbilical hernia

Answer 452

when laughing, coughing, crying or going to the toilet

Question 453

what causes an epigastric hernia

Answer 453

Defect in linea albea above umbilicus leads to protrusion of peritoneal fat
present form birth - may only notice when thinner

Question 454

when should an epigastric hernia be repaired

Answer 454

not - cosmetic reasons only

Question 455

what causes gastroschisis

Answer 455

congenital problem when gut forms where it doesn’t go back into amniotic cavity
> gut eviscerated and exposed

Question 456

what percentage of gastroschisis are associated with atresia

Answer 456

10% - fatal

Question 457

what is the management of gastroschisis

Answer 457

primary / delay closure

TPN nutrition

Question 458

what is the survival of gastroschisis

Answer 458

90%

unless associated with atresia - fatal

Question 459

what may be a complication of short gut

Answer 459

defect nips off small bowel

Question 460

what is exophalos

Answer 460

disturbed embryogenesis leads to external organs covered in membrane

Question 461

what anomalies are associated with exophalos

Answer 461

25% cardiac
25% chromosomal - Trisomy13, 18 (fatal), 21
15% renal, neurological
Beckwith-Weideman syndrome

Question 462

what things are associated with Beckwith-Weideman syndrome

Answer 462

hypoglycaemia, macrogloassia (massive tongue), macrosomia

Question 463

what is the management of exophalos

Answer 463

primary / delayed closure

Honey dressings

Question 464

what is the outcome of exophalos

Answer 464

poor due to complications
Antenatal 15% survive to birth
post-natal mortality – 25%

Question 465

what is hypospadias

Answer 465

urethral meatus on the ventral aspect of the penis

Classification – anterior (50%), middle (30%), posterior (20%)

Question 466

what is the management of hypospadias

Answer 466

DO NOT CIRCUMCISE – need skin to reconstruct

one stage or 2 stages procedure

Question 467

how may urology problems present systemically in children

Answer 467

• fever, vomiting
• failure to thrive
• anaemia
• hypertension
• renal failure

Question 468

how may urology problems present locally in children

Answer 468

• pain
• changes in urine
• abnormal voiding
• mass
• visible abnormalities

Question 469

what is the difference between a hernia and a hydrocele

Answer 469

hernia - groin swelling

hydrocele - scrotal swelling

Question 470

are hernias common in girls

Answer 470

no - may contain an ovary

Question 471

what type of swelling are premature babies at risk of

Answer 471

hernias - groin swelling

testis not dropped

Question 472

how common are hernias

Answer 472

affect 2% of boys

Question 473

what type of hernia is more common in children

Answer 473

indirect - through inguinal canal

Question 474

when should hernias be repaired urgently and why

Answer 474

<1 year

risk of incarceration (get stuck)

Question 475

how would you manage a hernia in a child > 1

Answer 475

elective referral and repair

growing proportion of hernia orifice to bowel means less likely to get stuck

Question 476

what is the risk of incarcerated testis

Answer 476

compressed blood supply leads to ischaemia and loss of testis

Question 477

are hydroceles symptomatic

Answer 477

no - painless
increase in size when straining, crying
blue in colour

Question 478

what layer of the testis communicates with the scrotum in hydroceles

Answer 478

tunica vaginalis

Question 479

what is the classic sign of a hydrocele

Answer 479

Chinese lantern sign – glows through torch light

Question 480

what is the management of a hydorcele

Answer 480

conservative until 5 yrs of age

96% go away

Question 481

what is cryptorchidism

Answer 481

undescended testis - Any testis that cannot be manipulated into the bottom half of the scrotum

Question 482

what is meant by ectopic testis

Answer 482

testis in wrong place – superficial femoral pouch, other side (abnormal descent)

Question 483

why are the testis in the scrotum

Answer 483

permatogenesis enzymes work optimally at 34 degrees. (Heat exchange through scrotal skin contributes to drop in temperature)

Question 484

when do testis normally descend

Answer 484

3rd trimester - any premature baby will always have undescended

Question 485

give indications for orchidopexy

Answer 485

• fertility - 1% loss germs cells / month undescent
• increased risk of malignancy
• susceptible to Trauma – fracture, near femoral neck
• Torsion – twists as not fixed
• cosmetic

Question 486

what is cricumcision

Answer 486

removal of the foreskin

Question 487

what is an absolute indication for circumcisison

Answer 487

Balanitis Xerotica Obliterans (BXO) – white scarring of foreskin

Question 488

what are relative indications for circumcision

Answer 488

Balanoprosthitis (inflammation of skin)
Religious - at 6 months
UTI - if grossly dilated urinary tract with kidney damage

Question 489

what are disadvantages of circumcision

Answer 489

painful
bleeding meatal stenosis – urinary meatus narrows
Fistula cosmetic - look different

Question 490

what are suggested advantages of circumcision

Answer 490

prevents UTI
Decreases malignancy
sexual enhancement - ↑nerve endings in prepuce
decrease of AIDS / STD’s

Question 491

describe the grades of vesicle uteric reflux

Answer 491

1 - ureter only
2 – kidneys but not dilated
3+ 4 – dilated kidneys
5- massive dilation of kidneys, poor function, grossly abnormal urinary tract

Question 492

how is vesico uteric reflux managed

Answer 492

conservative - voiding advice, constipation, fluids
antibiotic prophylaxis - Trimethoprim (2mg/kg nocte)
until age 4
STING procedure - mild/moderate with symptoms
ureteric re-implantation

Question 493

what is torsion appendix testis

Answer 493

embryological remnant of girl bits, can twist

Question 494

what is torsion testis

Answer 494

twisting of testis - can lose blood supply

Question 495

what is the differential diagnosis of painful scrotum

Answer 495

torsion testis
torsion appendix testis
epididymitis/ orchitis
rare - trauma, haematocele, incarcerated inguinal hernia

Question 496

how long after testicular torsion till the blood supply is lost

Answer 496

6-8 hours

Question 497

what is the blue dot sign (ischaemia through skin) a sign of

Answer 497

torson appendix

Question 498

what age does torsion testis usually happen in

Answer 498

older kids

torsion appendix in younger

Question 499

what age does torsion appendix testis usually happen in

Answer 499

younger children

Question 500

what should you do if you are in doubt of the cause of an acute painful scrotum

Answer 500

open to explore - can’t miss torsion

Question 501

what are complications of UTI in kids

Answer 501

renal scarring, reflux nephropathy
chronic renal failure
hypertension

Question 502

who should you investigate UTI’s in

Answer 502

• all <6/12 months, atypical infection, odd symptoms, recurrent
pure growth bacteria > 105 – even if low bacteria and symptoms take seriously
Pyuria – pus cells
systemic upset - fever, vomiting

Question 503

how can you investigate UTI’s in kids

Answer 503

US scan - anatomy
renography
o 99mTc MAG3 (drainage, function, reflux)
o 99mTc DMSA (function, scarring)
micturating cystourethrogram (MCUG)- demonstrate reflux

Question 504

when does neurodevelopment stop

Answer 504

2 years

Question 505

what must you work out form the neurological history in a child about the symptoms

Answer 505

if they are static (arrest in development)) or slowly progressive

Question 506

what percentage of children form 10-17 suffer migraines

Answer 506

7.7%

Question 507

what percentage of children doe epilepsy affect

Answer 507

0.7%

Question 508

what is the 2nd most common cancer in children

Answer 508

brain tumours

Question 509

what type of headache duration are most likely to have underlying pathology

Answer 509

isolated acute

chronic progressive - get worse every day

Question 510

what should you ask a child about their headache

Answer 510

Any warning? 	
Location? 	
Severity? 
Duration? 
Frequency?

Question 511

why do you always do fundoscopy in someone with a headache

Answer 511

look for papilloedema

Question 512

where would you listen for a cranial bruit

Answer 512

side of skull - temporal region

AV fistula, aneurysm

Question 513

how are migrainea and tension type headaches diagnosed

Answer 513

clinically on history/ exam

Question 514

what associated symptoms ay be seen in a child with a migraine

Answer 514

abdominal pain, nausea, vomiting

pallor

Question 515

what symptoms may be felt before/ after a migraine

Answer 515

Visual disturbance, paresthesia, weakness
aggravated by bright light (photophobia)
(visual, sensory, motor aura)

Question 516

what things may aggravate a migraine

Answer 516

bright light/ noise

fatigue/ stress

Question 517

what is the mamangement of an acute migraine

Answer 517

effective pain relief
triptans
sleep/ rest. dark room may help

Question 518

if a child has migraines at least once a week what medication could they be put on

Answer 518

Pizotifen, Propranolol, Amitryptyline, Topiramate, Valproate

Question 519

what is the difference in the pain of a migraine and tension type headache

Answer 519

migraine - hemicranial (one side)

tension - diffuse, symmetrical all over (tight band), present all the time

Question 520

is there a strong family history of migraines

Answer 520

yes

Question 521

what is the management of an acute tension type headache

Answer 521

simple analgesia

Question 522

what is the prevention of tension type headaches

Answer 522

amitryptilline - discourage analgesics

Attention to underlying chronic physical, psychological or emotional problems

Question 523

what is waking up from sleep with a headache a sign of

Answer 523

raised ICP

Question 524

what is the treatment of analgesic overuse headaches

Answer 524

stop taking
(Headache is back before allowed to use another dose)

Question 525

what associated features with a headache would require further neuroimaging

Answer 525

Features of cerebellar dysfunction – poor co-ordination, nystagmus,
Features of raised intracranial pressure
New focal neurological deficit eg. new squint
Seizures, esp focal
Personality change

Question 526

what is a convulsion

Answer 526

Seizure where there is prominent motor activity

Question 527

what is an epileptic seizure

Answer 527

an electrical phenomenon coming from brain
An abnormal excessive hyper synchronous discharge from a group of (cortical) neurons
It may have clinical manifestations – depending on where discharge coming from
Paroxysmal change in motor, sensory or cognitive function

Question 528

what is epilepsy

Answer 528

A tendency to recurrent, unprovoked (spontaneous) epileptic seizures – at least 2 for diagnosis

Question 529

what chemical imbalances trigger seizures

Answer 529

Decreased inhibition (gama-amino-butyric acid, GABA)	
Excessive excitation (glutamate and aspartate)
Lead to excessive influx of Na and Ca ions - produces an electrical current

Question 530

what test can be done to measurlthe current of a seizure

Answer 530

EEG - Electroencephalogram

measure summation of a multitude of electrical potentials that result in depolarisation of neurones

Question 531

what is the difference between a focal and generalised seizure

Answer 531

generalsied - affect all brain

focal - restricted to one hemisphere

Question 532

are most epilepsies focal or generalised

Answer 532

generalised

Question 533

what is the commonest cause of an acute symptomatic seizure in childhood

Answer 533

febrile convulsion 
(associated with fever but without evidence of intracranial infection or defined cause for the seizure)

Question 534

what age do febrile convulsions occur at

Answer 534

3 months - 5 years

Question 535

how would you investigate a seizure

Answer 535

history 
video recording if possible
ECG
EEG 
MRI brain 
genetics - familial

Question 536

what single gene disorder is associated with seizures

Answer 536

tuberous sclerosis

Question 537

what is the difference between an interictal and ictal EEG

Answer 537

interictal - not having a seizure - limited value in diagnosing epilepsy

Question 538

what is the role of Anti-epileptic drugs

Answer 538

control seizures, not cure epilepsy

Question 539

what is the fist line anti-epileptic for children with generalised seizures

Answer 539

sodium valproate

not child bearing age

Question 540

what is the fist line anti-epileptic for children with focal seizures

Answer 540

carbamazepine

Question 541

what surgical procedure can be done in the management of epilepsy

Answer 541

vagal nerve stimulator – implantable device that generates electrical impulses, under L clavicle wired to L vagus to break epilepsy cycle, family has magnet to use
Epilepsy surgery - remove focal part

Question 542

what is a reflex anoxic seizure

Answer 542

due to vagally mediated bradycardia
e.g. child hit arm against wall very painful vagally mediated pain causes bradycardia (always triggered by pain, fright, upset)
common in toddlers

Question 543

what is the condition where children have psychogenic seizures

Answer 543

non epileptic attack disorder (NAED)

Question 544

when would you suspect a neuromuscular disorder in a child

Answer 544

floppy 
paucity of limb movements 
weak pelvic girdle 
delayed motor milestones
frequent falls 
myopathic facies 
myotonia 
foot drop 
belly sticks out - poor abdominal s

Question 545

what is GOwers sign

Answer 545

ask child to lie flat and get them to stand up without any aids - will roll onto front

Question 546

what are pes caves an hammer toes a sign of

Answer 546

charcot marie tooth disease

Question 547

what things are seen in myopathic facies

Answer 547

droopy eyelids (ptosis), can’t close mouth, poor head control

Question 548

where do neuromuscular disorders affect

Answer 548

Muscle
Neuromuscular junction-
Nerve -
Anterior Horn Cell

Question 549

what is the commonest neuromuscular disorder in children

Answer 549

duchenne muscular dystrophy

Question 550

what gene is mutated in duchenne muscular dystrophy

Answer 550

Xp21, dystrophin gene

X linked

Question 551

how is duchenne muscular dystrophy inherited

Answer 551

X linked

Question 552

what are signs of duchenne muscular dystrophy

Answer 552

Waddling gait, calf hypertrophy (replaced by fat), Gower’s sign positive

Question 553

why are creatine kinase levels so high in duchenne muscular dystrophy

Answer 553

muscle breakdown

Question 554

what is the main treatment of duchenne muscular dystrophy

Answer 554

steroids

Question 555

what is a complication of duchenne muscular dystrophy

Answer 555

cardiomyopathy

respiratory in teens

Question 556

do myopathies or neuropathies have contracture

Answer 556

myopahty

Question 557

do reflexes last longer in myopathy air neuropathy

Answer 557

myopathy

Question 558

what do anterior horn cell pathologies/ spinal muscular atrophy lad to in adults

Answer 558

motor neuron disease

Question 559

what are the most common cancers in children

Answer 559

leukaemia 33%
brain tumour 25%
lymphoma

Question 560

how are children’s cancer classed

Answer 560

Based on tumour morphology and (primary site)

Question 561

what do children with falcon anaemia have a high change of getting

Answer 561

aplastic leukaemia

get adult cancers in youth

Question 562

what is Li-Fraumeni Familial Cancer Syndrome

Answer 562

AD mutation in p53 – leukaemia, sarcoma, brain tumour, adrenal cortex tumour (50% of cancer by 30, 90% by 70)

Question 563

what canacers are children with beckwith weidmann syndrome screened for

Answer 563

hepatoglastoma

WInn’s tumour

Question 564

name to infections that can drive cancers in kids

Answer 564

HPV - cervical

EBV - lymphoma

Question 565

which presenting symptoms need a same day referral to a paediatric oncologist

Answer 565

unexplained petechiae, hepatosplenomegaly

Question 566

which presenting symptoms need referral to a paediatric oncologist

Answer 566

repeat attendance, same problem, no clear diagnosis 
new neuro symptoms, abdo mass
rest pain (night), back pain and unexplained lump		lymphadenopathy

Question 567

what are signs of brain tumours in pre school kids

Answer 567

persistent/  recurrent vomiting 
abnormal balance/ walking
abnormal eye movements 
behaviour change 
fits/ seizure
abnormal head position

Question 568

what are signs of brain tumours in children 5-11

Answer 568

persistent/  recurrent vomiting 
persisten/ recurrent headache
abnormal balance/ walking
abnormal eye movements 
behaviour change 
fits/ seizure
abnormal head position

Question 569

what are signs of brain tumours in young adults 12-18

Answer 569

persistent/  recurrent vomiting 
persisten/ recurrent headache
abnormal balance/ walking
abnormal eye movements/ blurred/ double vision 
behaviour change 
fits/ seizure
delayed/ arrested puberty

Question 570

list 4 oncological emergencies

Answer 570

• Sepsis / febrile neutropenia
• Spinal cord compression
• Mediastinal mass
• Tumour lysis syndrome

Question 571

how may you inestigate a cancer in a child

Answer 571

MRI - no radiation
biopsy
tumour markers

Question 572

what hormone does testicular cancer secrete

Answer 572

BHCG

Question 573

what are acute SE of chemotherapy

Answer 573

Hair loss, Nausea & vomiting, Mucositis, Diarrhoea / constipation
Bone marrow suppression – anaemia, bleeding, infection

Question 574

what are chronic side effects of radiotherapy

Answer 574

Fibrosis / scarring, Second cancer, Reduced fertility

Question 575

what are chronic side effects of chemotherapy

Answer 575

Organ impairment – kidneys, heart, nerves, ears
Reduced fertility
Second cancer

Question 576

what are acute side effects of radiotherapy

Answer 576

Lethargy, Skin irritation, Swelling

Organ inflammation – bowel, lungs

Question 577

which oncology patients are at risk of sepsis

Answer 577

ANC < 0.5 x 109
Indwelling catheter – route for bacteria
Mucosal inflammation/ dairrhoea
High dose chemo / SCT

Question 578

what bacteria commonly cause sepsis in cancer patients

Answer 578

Pseudomonas aeruginosa
Enterobacteriaciae eg E coli, Klebsiella
Streptococcus pneumoniae
Enterococci
Staphylococcus 
Fungi eg. Candida, Aspergillus

Question 579

how may a child with cancer present with sepsis

Answer 579

Fever (or low temp) –
Rigors
Drowsiness
Shock - Tachycardia, tachypnoea, hypotension, prolonged capillary refill time, reduced UO, metabolic acidosis

Question 580

how may you investigate for sepsis in kids with cancer

Answer 580

Blood culture, FBC, coag, UE, LFTs, CRP, lactacte
CXR (respiratory symptoms) 
Other - Urine microscopy / culture
Throat swab
Sputum culture / BAL
LP
Viral PCRs
CT / USS (pus in liver/ badder)

Question 581

what is the management of sepsis in cancer patients

Answer 581

ABC – oxygen, fluids (IV access)
Broad spectrum antibiotics – IV tazicin
Inotropes
PICU – paediatric intensive care unit

Question 582

what antibiotic is given to kids with cancer who become septic

Answer 582

IV tazicin

Question 583

why is raised ICP not common in babies

Answer 583

fontanelles aren’t fused

Question 584

what is tumour lysis syndrome

Answer 584

Metabolic derangement causing rapid death of Tumour Cells

Release of intracellular contents into plasma – high K , Ph and lead to death from arrhythmia

Question 585

what caner is tumour lysis syndrome common in

Answer 585

burkett’s lymphoma

Question 586

what are clinical features of tumour lysis syndrome

Answer 586

high K, high rate, high phosphate, low calcium
acute renal failure
arrhythmia

Question 587

how is tumour lysis syndrome managed

Answer 587

Avoidance – many fluids 
ECG Monitoring
Hyperhydrate-2.5l/m2 
QDS electrolytes
Diuresis
Never give potassium

Question 588

how may you treat hyperkalaemia in kids

Answer 588

¥ Ca Resonium
¥ Salbutamol
¥ Insulin

Question 589

what are common causes of superior vena cava syndrome

Answer 589

lymphoma

neurobalstoma, germ cell tumour, thrombosis

Question 590

how may Superior Vena Cava syndrome present

Answer 590

facial, neck and upper thoracic plethora, oedema, cyanosis, distended veins, ill, anxious, reduced GCS, watershed mark

Question 591

how may Superior Mediastinal Syndrome present

Answer 591

dyspnoea, tachypnoea, cough, wheeze, stridor, orthopnoea

Question 592

how is SVCS/ SMS managed

Answer 592

keep upright/ calm
urgent biopsy
chemotherapy
steroids

Question 593

what are early signs of raised ICP in children

Answer 593

early morning headache/vomiting
tense fontanelle
increasing head circumference

Question 594

what are late signs of raised ICP in children

Answer 594

constant headache 				papilloedema  
diplopia (VI palsy) – double vision 
Loss of upgaze
neck stiffness					status epilepticus, 
reduced GCS - drowsy				
Cushings triad (low HR, high BP, low RR)

Question 595

which imaging is best for raised ICP

Answer 595

MRI

Question 596

what is the management of raised ICP

Answer 596

dexamethasone

reduces swelling and increases CSF flow

Question 597

what surgical procedures can be done to treat raised ICP

Answer 597

Ventriculostomy – hole in membrane at base of 3rd ventricle with endoscope
EV Drain (temporary) – release oedema
VP shunt – ventricles to tummy

Question 598

what is cushings triad (raised ICP)

Answer 598

low HR, high BP, low RR)

Question 599

what cancers common cause spinal cord compression

Answer 599

Ewing’s (vertebral body)
Medulloblastoma (back of head)
neuroblastoma
germ cell tumour

Question 600

how is spinal cord compression as a complication of cancer managed

Answer 600

Urgent MRI
Start dexamethasone urgently to reduce peri-tumour oedema
Definitive treatment with chemotherapy is appropriate when rapid response is expected

Question 601

how may spinal cord compression as a complication of cancer present

Answer 601

Symptoms vary with level
weakness (90 %) 
pain (55-95 %) 
sensory abnormality (10-55%)  
sphincter disturbance (10-35%

Question 602

what organ separates URTI and LRTI

Answer 602

larynx

Question 603

is the respiratory tract sterile

Answer 603

no

Question 604

are URTI’s self limiting

Answer 604

yes - 99%

Question 605

do you give antibiotics for URTI

Answer 605

no - mostly viral

do more harm than good

Question 606

how many episodes of rhinitis may a child get a year

Answer 606

5-10 - normally winter months

Question 607

how long does rhinitis normally last

Answer 607

11-14 days

self limiting

Question 608

how may a viral illness lead to a more severe virus

Answer 608

breach in epithelium making commensal bacteria infective

pneumonia, bronchiolitis, meningitis, septicaemia

Question 609

how long does otitis media last

Answer 609

3-7 days

Question 610

how may otitis media appear on otoscope

Answer 610

red
dorme not transparent
painful

Question 611

what is the treatment of otitis media

Answer 611

spontaneous rupture of drum

Question 612

what drug should be given in otitis media

Answer 612

analgesia

Question 613

how long does tonsillitis last

Answer 613

2-5 days

Question 614

how do you differentiate between viral and bacterial tonsillitis

Answer 614

throat swab

Question 615

what drug should you not give in tonislitis and why

Answer 615

amoxicillin - rash with EBV

Question 616

how would you treat bacterial tonsillitis

Answer 616

10 days penicillin

Question 617

what organism causes croup

Answer 617

para’flu 1

Question 618

how long does croup last

Answer 618

2-3 days

Question 619

why is epiglottis rare

Answer 619

vaccinated against H. influenzae Type B

Question 620

what is the biggest difference in the presentation of croup and epiglottis

Answer 620

croup the child is well, epiglottis the child is very ill

Question 621

how may a child with group present

Answer 621

Coryza++, stridor, hoarse voice, “barking” cough (airway obstruction)

Question 622

how may a child with epiglottis present

Answer 622

septic, Stridor, drooling

Question 623

what is the treatment of croup

Answer 623

oral dexamethasone

Question 624

what is the treatment of epiglottis

Answer 624

intubation and IV antibiotics

Question 625

what organism causes epiglottis

Answer 625

H. influenzae Type B

Question 626

what is the main management for LRTI

Answer 626

oxygenation, hydration, nutrition

Question 627

what bacterial agents commonly cause LRTI

Answer 627

Strep pneumoniae, Haemophilus influenzae, Moraxella catarrhalis, Mycoplasma pneumoniae, Chlamydia pneumoniae

Question 628

what viral agents commonly cause LRTI

Answer 628

RSV, parainfluenza III, influenza A and B, adenovirus, rhinovirus

Question 629

how may bronchitis present

Answer 629

loose rattly cough. Lasts over 2 weeks.

Post-tussive vomit - “glut” (mucous), but chest free of wheeze/creps

Question 630

what is the most common organism causing bronchitis

Answer 630

haemophillus influenza

pneumococcus

Question 631

does bronchitis need treatment

Answer 631

no -self limiting

Question 632

what is the mechansim of bronchitis

Answer 632

virus disturbs mucociliary escalator clearance, increasing cough and rattle until cleared

Question 633

what are red flags for bronchiectasis not bronchitis

Answer 633

Age <6 mo, >4yr Static weight
Disrupts child’s life Associated SOB (when not coughing)
Acute admission Other co-morbidities (neuro/gastro)

Question 634

what % of infants get bronchiolitis

Answer 634

30-40%

Question 635

how does bronchiolitis present

Answer 635

One off episode - Nasal stuffiness, tachypnoea, poor feeding, Crackles +/- wheeze

Question 636

what is the treatment for bronchiolitis

Answer 636

Maximal observation, minimal intervention (if oxygenation, nutrition and hydration ok)

Question 637

how long may bronchiolitis last for

Answer 637

7-14 days

noticed at 2-5 days at peak

Question 638

what organism commonly cause bronchiolitis

Answer 638

usually RSV

parable III, HMPV

Question 639

is a child has a wheeze is it viral or bacterial

Answer 639

viral

Question 640

what is a LRTI defined as

Answer 640

48 hrs, fever (>38.5oC), SOB, cough, grunting

Question 641

what are symptoms/ signs of LRTI

Answer 641

fever (>38.5oC), SOB, cough, grunting

¥ Reduced or bronchial breath sounds

Question 642

when can you call a LRTI pneumonia

Answer 642

signs are focal (in one area)
Crepitations
High fever

Question 643

what is the first line treatment for pneumonia in kids

Answer 643

oral amoxicillin

macrolide 2nd

Question 644

how would you treat a milk LRTI

Answer 644

not - review if gets worse

Question 645

how does pertussis present

Answer 645

coughing fits, vomiting and colour change.

>2 weeks of cough

Question 646

how would you treat bilateral otitis media in an infant

Answer 646

oral amoxicillin

Question 647

what are the symptoms of asthma

Answer 647

Chronic wheeze
cough - dry, nocturnal, exertional
SOB- at rest

Question 648

what things may trigger asthma in kids

Answer 648

URTI, exercise, allergen, cold weather, etc

Question 649

what is a cardinal feature of asthma that separates it form COPD

Answer 649

reversible/ variable

Question 650

what are the 3 cardinal features of asthma

Answer 650

chronic wheeze
reversible/ variable
responds to treatment

Question 651

what are some potential causes of asthma

Answer 651

Genes (50-80% risk) - Inherently abnormal lungs, poor lung function
Early onset atopy
Late exposures – rhinovirus, exercise, smoking
Occupational – baker, latex etc.

Question 652

can you grow out of asthma

Answer 652

yes

Question 653

if you have asthma how much more likely are you to get COPD

Answer 653

5x

Question 654

what is the differential diagnosis of asthma in <5s

Answer 654

Congenital airway abnormality 
CF 
PCD – primary ciliary dyskinesia
Bronchitis
Foreign body 
(unlikely)

Question 655

what is the differential diagnosis of asthma in >5s

Answer 655

Dysfunctional breathing (psychological)
Vocal cord dysfunction
Habitual cough
Pertissus

Question 656

what is the treatemtn of asthma

Answer 656

Watchful waiting if no QoL implications of symptoms
Low dose ICS for 2 months
Review after 2 months – step up treatment/ give inhaler holiday

Question 657

what can parents do to help their childs asthma

Answer 657

stop tobacco smoke exposure, remove environmental triggers (cat, dog etc.)

Question 658

what drug is given in adult asthma but not kids

Answer 658

LAMA

Question 659

what are the goals of asthma treatment

Answer 659

“minimal” symptoms during day and night 
minimal need for reliever medication 
no attacks (exacerbations) 
no limitation of physical activity 
normal lung function (in practical terms FEV1 and/or PEF >80% predicted or best) 
Increase dose if not effective.

Question 660

what survey can me done to measure control of asthma

Answer 660

SANE questionnaire 
Short acting beta agonist/week
Absence school/nursery
Nocturnal symptoms/week
Excertional symptoms/week

Question 661

what is the goal of well controlled asthma

Answer 661

no more than 1-night wakening to asthma, use blue less than 3 days a week.

Question 662

what classes of medications should be used to treat asthma

Answer 662

¥	Short acting beta agonists
¥	Inhaled corticosteroids (ICS)
¥	Long acting beta agonists*
¥	Leukotriene receptor antagonists*
¥	Theophyllines*
¥	Oral steroids

Question 663

what is the first line treatment of asthma

Answer 663

low dose ICS - review after 2 months for response, may step down to B2 agonist

Question 664

what is the second stage of asthma treatment after diagnosis

Answer 664

B2 agonist

+ regular preventer - ICS

Question 665

what are adverse effects of ICS

Answer 665

height suppression (0.5-1cm), oral candidiasis (brush teeth), adrenocortical suppression (stronger purple inhaler)

Question 666

what is the 3rd stage of asthma treatment in kids

Answer 666

Add on LABA – do not use without ICS. Use as fixed dose inhaler.
Or LTRA - Montelukast only, Rule of thirds for efficiency

Question 667

what is the 4th stage of asthma treatment in kids

Answer 667

refer - high dose ICS

Question 668

what are the 2 types of delivery systems used for inhalers

Answer 668

MDI (metered dose inhaler)/ spacer

dry powder device

Question 669

what is the ling deposition when using a delivery system with an inhaler

Answer 669

20%

5% without

Question 670

what age can use a dry powder device

Answer 670

licensed in over 5s

struggle under 8 - try in girls first

Question 671

does losing weight help treat asthma

Answer 671

no but improves general health

Question 672

what gut disease may a painful joint be assoiciated with

Answer 672

IBD

Question 673

what blood disorder may a painful joint be associated with

Answer 673

haemophilia

Question 674

what are red flag symptoms of joint pain

Answer 674

Fever
Malaise/lethargy
Morning joint stiffness or pain
Night pain refractory to simple analgesia and symptomatic during the daytime

Question 675

what are red flag signs of joint pain

Answer 675

• Joint swelling
• Bony tenderness to palpation
• Muscle weakness
Fall in height or weight growth curve

Question 676

how may you investigate a painful joint

Answer 676

FBC including ESR/ CRP
blood culture 
Xray 
US - effusion of septic arthritis
MRI - inflammation sensitive 
Bone scan - osteomyelitis
CT

Question 677

after inflammation how long does CRP and ESR take to rise

Answer 677

ESR - 48 hours

CRP - 6 hours

Question 678

what is the most common chronic rheumatological disease in children

Answer 678

JIA

Question 679

what age does JIA affect

Answer 679

<16

Question 680

which sex are more affected by JIA

Answer 680

female 3:1

Question 681

what pro inflammatory markers are seen in JIA

Answer 681

TNF, interleukin 1 & 2. (cause presence of antibodies – ANA, RF)

Question 682

give the different classifications of JIA

Answer 682

oligoarthritic < 4 joint
polyarticular > 5 joints
systemic
enthesitis
psoriatic

Question 683

when are children most affected by oligoarthritic JIA

Answer 683

girls 1-5

Question 684

what eye sign is seen in ANA positive oligoarthritis JIA

Answer 684

uveitis

Question 685

how do young girls present with ologiarthritis JIA

Answer 685

large joints affects - knees, ankles, hands, feet and hips
NOT HIP
uveitis
+ve ANA

Question 686

if a child with oligoarthritis JIA presents late are they likely to be ANA -ve or +VE

Answer 686

-ve
(boys > 8)
hip involvement

Question 687

how many joints are affected in oligoarthritic JIA

Answer 687

< 4

Question 688

how many joints are affected in polyarticular JIA

Answer 688

> 5 joints

Question 689

what is the difference between seropositive and seronegative polyarticular arthritis

Answer 689

-ve = RF -ve (20-30%) <5s
\+ve = RG +ve (5-10%) >8s

Question 690

do people with seropositive or seronegative polyarticular JIA have a better or worse prognosis

Answer 690

seropositive

Question 691

what are symptoms of temporomandibular joint involvement in polyarticular JIA

Answer 691

limited bite

micrognathia

Question 692

are systemic manaifestations seen in polyarticular JIA

Answer 692

rare - fever, slight hepatoslenomegaly, lymphadenopathy, pericarditis, chronic uveitis

Question 693

what joints are most commonly affected in polyarticualr JIA

Answer 693

large, fast growing joints
TMJ
onset insidious

Question 694

what is enthesistis JIA

Answer 694

inflammation of the enthesis along with arthritis

Question 695

what is an enthesis

Answer 695

where tendon joins the bone

Question 696

what features are seen in Enthesitis related JIA

Answer 696

Onset of polyarthritis/ oligoarthritis in a boy > 8 years
HLA B27 positivity
Acute anterior uveitis
Inflammatory spinal pain
Sacroilliac joitn tenderness
Family history of enthesitis – related JIA

Question 697

what features are seen is psoriatic JIA

Answer 697

HLA- B27 positive
Family history of psoriasis
Dactylitis (finger or toe inflammation) - mostly affects small joints of hands
Oncholysis, nail pitting

Question 698

what joints does psoriatic JIA most commonly affect

Answer 698

small joints of hand - dactyltiis

Question 699

what are features seen in still’s disease (systemic JIA)

Answer 699

unwell – intermittent fever > 2 weeks (spiking temperature, apyrexial in between), salmon pink erythematous rash, generalised lymphadenopathy, serosisits, hepatomegaly/ splenomegaly
High inflammatory markers

Question 700

what is a key feature of the temperature seen in still’s disease

Answer 700

spiking - child is well in between

Question 701

what are symptoms of JIA

Answer 701

Joint Pain
Swelling
Stiffness: Early morning, relieved by movement Babies / infants: crying or irritability
Limp or abnormal gait Regression of milestones in infants
Delayed puberty/ growth Fatigue (autoimmune conditions)
Poor appetite/ weight loss

Question 702

what are systemic symptoms of JIA

Answer 702

Fever, Rash, Lymphadenopathy, Hepatomegaly/splenomegaly, serositis

Question 703

how long must the arthritis last for it to be JIA

Answer 703

6 weeks

Question 704

in JIA, when is the joint most swollen

Answer 704

morning

relieved by movement

Question 705

what are signs of JIA

Answer 705

Swelling - periarticular soft tissue oedema/intraarticular effusion/ hypertrophy of synovial membrane
Tenosynovitis (swollen tendons)
Joint held in position of maximum comfort (knee fixed flexion)
Range of motion limited at extremes. (Due to inflammation and fluid)

Question 706

what is the differential diagnosis of JIA

Answer 706

Septic arthritis Transient synovitis
Osteomyelitis – infection of bone – Xray/ US will show Rheumatic fever
Connective tissue disorder - Systemic Lupus
Juvenile Dermatomyositis
Associated with IBD
Recurrent haemarthrosis – boys with haemophillia
Growing pain
Leukaemia Neuroblastoma
Primary bone tumour
Perthe’s disease Slipped upper femoral epiphysis
Congenital hip dysplasia Fracture
Trauma Referred pain

Question 707

give factors that may cause a poor prognosis in JIA

Answer 707

Active disease at 6 months Polyarticular onset and course
Extended oligoarticular Female
Rh Factor +ve ANA +ve
Persistent raised inflammatory markers

Question 708

what is the test for JIA

Answer 708

isn’t one
Consider:
Anti-nuclear antibodies (ANA) HLA-B27
inflammatory markers (CRP, ESR, WBC, platelets) Rheumatoid factor
X-ray/ US
MRI with contrast - synovitis
•Exclude differential diagnosis

Question 709

what is the most important investigation in JIA

Answer 709

MSK examination of all joints
pGALS, pREM
movement restriction

Question 710

what is the non pharmacological management of JIA

Answer 710

physiotherapy - strength & condition, range of movement, hydrotherapy
Occupational therapy, Psychology (counselling for patients and parents)
opthalmology screening
Nutrition – address anemia and osteoporosis

Question 711

what is the drug management for JIA

Answer 711

NSIAD
Intra-articular steroids
DMARD - methotrexate
biologics - anti-TNF , in conjunction

Question 712

what DMARD is used to treat JIA

Answer 712

methotrexate - subcutaneous weekly

Question 713

what drug is used to treat oligoarticualr JIA

Answer 713

Intra-Articular steroids

Question 714

what drug is used to treat polyarticualr JIA

Answer 714

systemic steroids - oral/ IV methylprednisolone

Question 715

what are complications of JIA

Answer 715

Altered growth of limbs – localised disturbance such as a leg length discrpency
Scoliosis
Short stature – poor growth with active inflammation
Joint damage / destruction
Micrognathia – TMJ involvement (seen in polyarticular)
Blindness (untreated uveitis)
Contracture – now rare, treated aggressively
Psychosocial effects of chronic disease

Question 716

what screening do all children with JIA undergo

Answer 716

opthalmology every 3-4 months until 12

within 6 weeks of diagnosis

Question 717

what type of JIA is uveitis most common in

Answer 717

ANA positive oligo JIA Common in < 5 years, girls

Question 718

what is the treatment of uveitis

Answer 718

Initially topical steroids to reduce inflammation (More severe need systemic steroids)
tend to have poor response to steroids - DMARD and biologics

Question 719

what is SIRS

Answer 719

systemic inflammatory response syndrome

Question 720

what is seen in SIRS

Answer 720

Fever or hypothermia
Tachycardia
Tachypnoea
Leucocytosis or leucocytopaenia

Question 721

what is meant by bacteraemia

Answer 721

bacteria multiplying in the bloodstream

Question 722

which organs fail in sepsis in children

Answer 722

Respiratory failure Renal failure
Neurologic failure Haematological Failure
Liver failure

Question 723

what pathogens are the most responsible for sepsis in neonates <1 month

Answer 723

group B streptococci, eschericha coli
listeria monocytogenes
(colonisation of female genital tract)

Question 724

what pathogens are the most responsible for sepsis in children

Answer 724

streptococcus pneumoniae, meningococci, group A streptococci, staphylococcus aureus

Question 725

describe the pathophysiology of sepsis

Answer 725

LPS, peptoglycans and bacterial components released in blood stream
stimulate endothelium, neutrophils and monocytes to release cytokines.
This leads to microvascular occlusion, and vascular instability
septic combination of coagulopathy, fever, vasodilation and capillary leak

Question 726

give symptoms of sepsis in children

Answer 726

¥	Fever or hypothermia
¥	Cold hands/feet, mottled, prolonged capillary refill time 
¥	Chills/rigors
¥	Limb pain
¥	Vomiting and/or diarrhoea
¥	Muscles weakness
¥	Muscle/joint aches
¥	Skin rash
¥	Diminished urine output

Question 727

what is the recognition tool for children with sepsis

Answer 727

SEPSIS 6

Question 728

what is the management of sepsis in children (Sepsis 6)

Answer 728

1. Give high flow O2
2. Obtain IV access and obtain bloods (blood culture, blood sugar, lactate)
3. Give IV antibiotics – cefotaxime + amoxicillin if neonate
4. Consider fluid resuscitation - 20mls/ kg crystalloid- aim to reverse shock and normalise HR/ BP
5. Consider early inotropic support – adrenaline
6. Involve senior/ specialist help early

Question 729

what age group has the highest prevelance of sepsis

Answer 729

new borns (preterm > term) 1;300

Question 730

what are the most common organisms that cause meningitis in neonates

Answer 730

group B streprococci, eschericha coli
listeria monocytognes
(female genital tract)

Question 731

what are the most common organism that cause meningitis in children

Answer 731

streptococcus pneumoniae, meningococci, haemophilus influenza

Question 732

give symptoms of meningitis in children

Answer 732

¥	Nuchal rigidity
¥	Headaches, Photophobia
¥	Diminished consciousness
¥	Focal neurological abnormalities
¥	Seizures – late sign 
¥	Meningococcal rash –  large scale purpura and bruising

Question 733

describe a meningococcal rash

Answer 733

large scale purpura

Question 734

how many meningitis present differently in neonates

Answer 734

Lethargy, Irritability
Bulging fontanelle – pressure around brain increases CSF
‘nappy pain’- stretch meninges

Question 735

how may you investigate meningitis in children

Answer 735

FBC
CSF:
Blood and CSF cultures (antigen testing, PCR) – become positive 24 hours later
Urine culture, skin biopsy culture
blood gas; metabolic acidosis
Imaging: CT-cerebrum – make sure you can do a LP

Question 736

what would you expect to see on a FBC in a child with meningitis

Answer 736

leucocytosis, thrombocytopaenia
CRP; elevated – marker of inflammation
coagulation factors; low levels due to DIC, low glucose

Question 737

what finsigs would you expect to see in the CSF of a child with meningitis

Answer 737

pleocytosis (High WCC) , increased protein level, low glucose (bacteria feeds on glucose)

Question 738

who is given chemoprophylaxis if a child has meningitis

Answer 738

close household contacts

Question 739

what is the antibiotic treatment for meneingits in children and neonates

Answer 739

3rd generation cefalosporins

+ amoxicilline if neonate – covers listeria

Question 740

is streptococcus pneumonia gram positive or negative

Answer 740

Gram-positive Duplo-cocci (Diplococcus pneumoniae)

Question 741

where does streptococcus pneumonia colonise

Answer 741

upper airways - 5-10% adults, 20-40% children

Question 742

how is streptococcus pneumonia transmitted

Answer 742

droplets

Question 743

what are complications of pneumococcal meningitis

Answer 743

brain damage, hearing loss, hydrocephalus

Question 744

is Haemophilus influenza type B gram positive or negative

Answer 744

negative

encapsulated

Question 745

what is a common predisposing factor for severe bacterial infections in children

Answer 745

viral illnesses

Question 746

why does meningococcus in the bloodstream have such an early mortality

Answer 746

lipooligosachharide -association endotoxin very potent stimulus of immune system

Question 747

what is the mortality of meningococcal disease

Answer 747

5-15% - 50% of deaths in first 12 hrs, 80% within 48 hrs

Question 748

what are common complications of meningococcal disease

Answer 748

Amputation (14%) – DIC, purpura
skin scarring (48%)
Cognitive impairment/epilepsy/hearing loss

Question 749

where is meningococcus usually found in children

Answer 749

nasopharynx

B and C (X Y becoming more prevalent)

Question 750

what do streptococci and staphylococci have in common

Answer 750

both gram positive cocci

Question 751

what antibiotic is active against streptococci

Answer 751

penicillin

Question 752

what antibiotic is active against staphylococci

Answer 752

flucloxacillin

resistance - need glycopeptide

Question 753

what antibiotic should you use if you are unsure if the infection is staphylococci or streptococci

Answer 753

flucloxacillin

Question 754

what are the main symptoms seen in toxic shock syndrome in children

Answer 754

warm

very red rash

Question 755

describe the sequence of scarlet fever

Answer 755

Contact and incubation 2-4 days 
Malaise, fever, tonsillitis
Couple of days later  Start exanthema
Strawberry tongue (characteristic sign) 
Squamation of hands and feet (after acute phase- 1-2 weeks later)

Question 756

what is a characteristic sign of scarlet fever

Answer 756

strawberry fever

Question 757

when is squamation of the hands and feet seen in a scarlet fever infection

Answer 757

late sign - 1 2 weeks after

Question 758

wat ist he cause of scarlet fever

Answer 758

Group A β-hemolytic streptococci

Question 759

what protects most children < 2 form scarlet fever

Answer 759

maternal IgG antibodies

Question 760

what is the most common ge for a scarlet fever infection

Answer 760

4

rare <2 and >10

Question 761

what are some complications of scarlet fever

Answer 761

Erysipelas, Cellulitis, Impetigo
Streptococcal toxic shock
Rheumatic fever 0.3-3%
Glomerulonephritis

Question 762

what is the treatment of scarlet fever

Answer 762

penicillin 10 days

Question 763

give features of Staphylococcal scalded skin syndrome (SSSS)

Answer 763

fever, widespread redness, fluid-filled blisters, rupture easily, especially in the skin folds. Superficial skin disappears, looks like burns in a patient

Question 764

give symptoms of kawasaki disease

Answer 764

Rash + Fever for at least 5 days and four of the five;
¥	bilateral conjunctival injection
¥	changes of the mucous membranes
¥	cervical lymphadenopathy
¥	polymorphous rash
¥	changes of the extremities
\+peripheral oedema
\+ peripheral erythema
\+ periungual desquamation (under nails)

Question 765

describe the pathophysiology of Kawasaki disease

Answer 765

self-limited vasculitis of medium-sized arteries
increased risk siblings and twins (genetic factor)
aetiology unknown but infectious cause suggested

Question 766

what are complications of Kawasaki disease

Answer 766

vasculitis of coronary arteries and kidney arteries

Question 767

what is the treatment of kawasaki disease

Answer 767

High dose Immunoglobulins
Aspirin – prevent clotting in diseased arteries
Other immunosuppressive agents

Question 768

what is the differential diagnosis for a erythemasotus maculopapulous rash + fever

Answer 768

Measles
Rubella
Enteroviruses
Cytomegalovirus
Human herpes virus 6/7
Parovirus B19
Epstein- Barre virus

Question 769

what is the differential diagnosis for a vesiculobulous rash + fever

Answer 769

Varicella – zoster virus
Herpes simplex virus
Enterovirus

Question 770

what are the different locations of the vesicles in herpes simplex 1, zoster and hand/foot/mouth

Answer 770

simplex - tongue/ mouth
zoster - one dermatome
hand foot mouth

Question 771

what virus cause shingles

Answer 771

herpes zoster

Question 772

how may you differentiate between a vesiculobulbous rash

Answer 772

smear - scrape ulcer base
PCR
immunofluorescence
serology (not acute)

Question 773

is shingles seen in children commonly

Answer 773

no - only immunodeficiency

Question 774

what is the incubation period of varicella zoster virus

Answer 774

14 days (10-21 days)

Question 775

what are complications of varicella zoster virus infections

Answer 775

secondary strep/staph infections skin (10-15%) – cellulitits, strep A leading to toxic shock
meningoencephalitis, cerebellitis,
arthritis

Question 776

what is the treatment for varicella zoster virus infections

Answer 776

aciclovir - normally not needed

Question 777

what percentage of children >5 will have suffered form varicella infection

Answer 777

> 90%

2-% by 1

Question 778

what babies does fatal varicella occur in (very rare)

Answer 778

1st month of life

immunodeficiency - T cell deficiency

Question 779

what are complicatiosn of herpes simplex infection

Answer 779

kerato) conjunctivitis
encephalitis
systemic neonatal infections

Question 780

how is congenital herpes caught

Answer 780

direct contact in the birth canal from mother with herpes type 2

Question 781

when and how - does congenital hereps present

Answer 781

day 4-21
70-80% disseminated/CNS infections – Sepsis, Meningoencephalitis, Hepatitis (jaundice, bleeding)
20-30% skin/eye/mouth (SEM) disease

Question 782

what is the prevalence of HSV 1 in children, young adults and adults

Answer 782

20-30% children 5 yrs of age,
40-50% adolescents,
60-80% adults

Question 783

what are the main causes of hand, foot and mouth disease

Answer 783

coxsackie A16

enterovirus 71

Question 784

what are severe complications of hand, foot and mouth disease

Answer 784

meningitis, encephalitis, acute paralysis, neonatal spies, myocarditis/ pericarditis, hepatitis and chronic infection in immunocompromised patients in neonates

Question 785

what is the difference between a primary and secondary immunodeficiency

Answer 785

primary - body’s immune system is missing or functions improperly
secondary - Components of the immune system itself are all present and functional.
Acquired diseases affecting the immune system and/or treatments negatively influencing the immune system.

Question 786

what mutations cause primary immunodeficiencies

Answer 786

single gene defects

Question 787

what are antibody deficiencies characterised by

Answer 787

deficiency of one of more (sub)classes of antibodies (e.g. IgG, IgA, IgM, IgG2) due to defective B-cell function
Absence of mature B-cells

Question 788

how do antibody deficiencies normally present and by what organism

Answer 788

Presents as recurrent bacterial infections of the upper and/or lower respiratory tract
Mostly caused by S. pneumoniae, H. influenzae

Question 789

what are cellular immunodeficiencies characterised by

Answer 789

impaired T-cell function or the absence of normal T-cells

Question 790

how do cellular immunodeficiencies normally present and by what organism

Answer 790

Presents as Unusual or opportunistic infections often combined with failure to thrive
Pneumocystic jirovecii, CMV (normally asymptomatic infections – not pneumonia)

Question 791

what are innate immune disorders characterised by

Answer 791

defects in phagocyte function
complement deficiency
Absence or polymorphisms in Pathogen Recognition Receptors

Question 792

what organsisms are likely to cause infections in innate immune disorders

Answer 792

defects in phagocyte function - S. aureus (sepsis, skin lesions, abscesses internal organs 
e.g liver)
Aspergillus infections (lung, bones, brain)
Complement deficiencies - N. meningitidis

Question 793

what things may make you suspect a primary immunodeficiency

Answer 793

Severe – requires hospitalisation or IV antibiotics
Persistent – wont completely clear up or clears very slowly
Unusual – caused by an uncommon organism
Recurrent – keeps coming back
Runs in the family – others have had similar susceptibility to infection

Question 794

what are the most common 3 secondary immunodeficiencies

Answer 794

HIV

chemo/radiotherapy - destroys neutrophils and lymphocytes

Question 795

why is HIV in children very rare

Answer 795

mums tested + treated in 1st trimester

Question 796

how may HIC present in children

Answer 796

¥	Recurrent common childhood RTI’s
¥	Persistent oral thrush
¥	Erythematous papular rash
¥	Generalized lymphadenopathy
¥	Recurrent/disseminated VZV/HSV infections (shingles in children) 
¥	Failure to thrive
¥	Developmental delay’s
¥	Opportunistic infections: CMV pneumonia/retinitis, PCP (Pneumocystic jiroveci pneumonia)

Question 797

how is chronic granulomatous disease inheritd

Answer 797

65% X linked

35% ar

Question 798

what is the most common cause of death in chronic granulomatous disease

Answer 798

aspergillosis - severe fungal disease

Question 799

what complex is effected in chronic granulomatous disease

Answer 799

NADPH oxidase complex

Question 800

what test diagnoses chronic granulomatous disease

Answer 800

DHR test - Non-fluorescent rhodamine derivative oxidized by NADPH oxidase to a green fluorescent compound (flow cytometry) – negative test gives diagnosis

Question 801

what is the the curative treatment of chronic granulomatous disease

Answer 801

Stem cell transplant (HSCT)

Question 802

why are fungal infections seen in children in PICU after abdominal surgery/ broad spectrum antibiotics

Answer 802

damage to gut flora favours growth of candida

Question 803

what disease does aspergillus fumigates cause most commonly

Answer 803

pneumonia

Question 804

how may neonatal candidaemia present

Answer 804

2nd/3rd week of life – sepsis syndrome, thrombocytopenia (more than bacterial), hyperglycaemia
attribute to 20-40% mortality

Question 805

what are risk factors for neonatal candidaemia

Answer 805

premature

low birth weight <750g

Question 806

up to how many self limiting viral infections a year is normal in children

Answer 806

8

Question 807

how may you diagnosis an antibody deficiency

Answer 807

1st serum sample to measure IgG, IgA, IgM
2nd serum sample to measure IgG subclasses (prevent recurrent infections)
3rd serum sample to measure immune response to vaccination with S. pneumoniae and H. influenza (should be protected)

Question 808

how may you diagnosis a complement deficiency

Answer 808

measurement of complement activation and/ or individual factors

Question 809

how are most primary immunodeficiencies treated

Answer 809

prophylactic antibiotics

extended vaccination schedule

Question 810

do children with tumour lysis syndrome need more fluids or less fluids

Answer 810

more fluids to wash out

Question 811

what should you never give to a patient with an oncological emergency

Answer 811

potassium

Question 812

what % of births have congenital anomalies

Answer 812

3%

Question 813

what re the main causes of multiple congenital anomaly syndromes

Answer 813

¥ single gene disorders 30%
¥ 10% chromosomal
¥ 5% teratogens/ viruses
¥ 55% unknown – mostly

Question 814

what is an ‘association’ with a syndrome

Answer 814

two features or more features occur together more often than expected by chance
mechanism unclear

Question 815

describe the VATER association

Answer 815

¥ vertebral anomalies (may influence rib anomaly)
Ð ano-rectal atresia
Ð tracheo-oesophageal fistula
Ð radial anomalies

Question 816

what does catch 22 stand for

Answer 816

Cleft palate
Abnormal facies – almond shaped eyes, simple ear 
Thymic hypoplasia / immune deficiency
Calcium problems 
Heart problems 
22 - Caused by 22 deletion

Question 817

give features of turners syndrome

Answer 817

co-arctation of aorta
short stature
neck webbing
Lymphoedema (Puffy feet, nuchal translucency / cystic hygroma)
Increased carrying angle at elbows, low hairline, wide sp nipples, sandal gap
learning difficulties
primary amenorrhoea and infertility

Question 818

give some features of downs syndrome

Answer 818

¥ Learning disability – key feature, need support to lead safe life
¥ Congenital heart disease
¥ Hypothyroidism – monitored annually
¥ Immunity – increased risk of infections
Early onset Alzheimer disease

Question 819

what is a syndrome

Answer 819

A distinct group of symptoms & signs which, associated together, form a characteristic clinical picture or entity – pattern of recognizable features.

Question 820

how would you investigate the genetic cause of a learning disability

Answer 820

Basic - Microarray, Fragile X
Targetted tests driven by phenotype – clinical judgement
Trio based Exome vs. genome trio analysis (Funding remains an issue)
Captured DNA from exome – next data sequencing – compared with parents

Question 821

what is the difference between a deformation and disruption pattern of development

Answer 821

Deformation: Organ parts are there just not functioning properly
Disruption: Parts of organ / body part absent

Question 822

give an example of a deformation and disruption pattern of development

Answer 822

amniotic bands cutting of bloody supply

Question 823

give 2 examples of sequence malformations

Answer 823

Pierre-Robin sequence - small chin to cleft palate (tongue pushes up as has nowhere to go so the palate doesn’t form properly
Fetal akinesia sequence - reduced fetal movement > reduced breathing > lung hypoplasia
contractures – get stuck

Question 824

what is the difference in features between polydactyly, polysyndactylty and acrocephalopolysyndatyly

Answer 824

extra fingers
+ skin in fingers
+ tall forehead

Question 825

what head features may be seen in a malformation syndrome

Answer 825

¥ Head – shape (brachiocephaly, talucephaly), size (macrocephaly, microcephaly, flat occiput)
Measure occipit- frontal circumference – plot on chart

Question 826

what eye features may be seen in a malformation syndrome

Answer 826

Hypertelorism - Inner canthal distance ICD and inter-pupillary distance IPD
Increased (further apart) – midline brain malformation
Telecanthus / epicanthic folds - ICD increased and IPD normal

Question 827

what ear signs may be seen in a malformation syndrome

Answer 827

ear position (low set, posteriorly rotated (reflects immaturity), small, crumpled

Question 828

what changes in the hands may be seen in a malformation syndrome

Answer 828

Finger length, Digital abnormalities (polydactyly extra, oligodactyly less, 
Palmar creases (Single palmar crease in Downs)
Marfans- long finger, measure middle finger length/ total hand length >44%

Question 829

what is the term used for recognising of a pattern of a syndrome

Answer 829

gestalt

Question 830

what is the standard first line test for malformation syndromes

Answer 830

microarray

Question 831

what is the difference between haematuria and proteinuria

Answer 831

blood in urine

protein in urine

Question 832

what is the difference in a neonates GFR compared to an adults

Answer 832

20-30ml/min/1.73m

at 2- 80-120

Question 833

what are the 5 main functions of the kidney

Answer 833

1. Waste handling – urea, creatinine
2. Water handling
3. Salt balance – NA, K, PO4, Cl,
4. Acid base control
5. Endocrine - erythropotien, renin, blood pressure, bone health

Question 834

what does proteinuria signify

Answer 834

glomerular injury

Question 835

what proteins make up the glomerular basement membrane

Answer 835

Type IV collagen and laminin
(Synthesis from podocytes and endothelial cells
Mesangial cells playing a role in turnover)

Question 836

what are the functions of the mesangial cells in the kidney

Answer 836

Glomerular structural support

Regulates blood flow of the glomerular capillaries (form biomechanical unit with capillaries capable of regulating filtration surface area as well as intraglomerular blood volume)

Question 837

what things form the glomerular filtration barrier

Answer 837

fenestrated endothelial cell
glomerular basement membrane (GBM)
podocyte with their “slit diaphragms”.

Question 838

which component of the glomerulus is affected in MCD, FSGS, Lupus

Answer 838

epithelial cell (podocyte) injury

Question 839

which component of the glomerulus is affected in Membranous glomerulopathy, MPGN, PIGN

Answer 839

basement membrane

Question 840

which component of the glomerulus is affected in ¥ Infection associated glomerulonephritis (PIGN), Haemolytic Uraemic Syndrome, Membranoproliferative Glomerulonephritis (MPGN), and Lupus

Answer 840

endothelial cell

Question 841

which component of the glomerulus is affected in HSP, IgA vasculitis/ nephropathy and Lupus

Answer 841

mesangial cell

Question 842

which component of the glomerulus is affected in minimal change disease

Answer 842

podocyte

Question 843

which component of the glomerulus is affected in IgA vasculitis

Answer 843

mesangial cell

Question 844

what are the 3 main features of nephrotic syndrome

Answer 844

proteinuria
hypoalbuminaemia
oedema

Question 845

why do you get oedema in nephrotic syndrome

Answer 845

lose protein from urine drops oncotic pressure which drives water into the 3rd space

Question 846

what are the main 3 tests for proteinuria

Answer 846

urine dipstix
protein: creatine urine
24hr urine collection

Question 847

what urine dipstick marker = proteinuria

Answer 847

> 3+

Question 848

how would a Pr:Cr indicate proteinuria

Answer 848

normal: Pr:CR ratio <20mg/mmol
Nephrotic range: >250mg/mmol (10x )
(best early morning, creatine excreted constantly by kidney)

Question 849

how would a 24 hour urine collection indicate proteinuria

Answer 849

normal <60mg/m²/24hrs

Nephrotic range>1g/m²/24hrs (Adults >3.5g/24hrs)

Question 850

what is urine Na a test of

Answer 850

tells if intravascular space dry

body conserve water for intravascular volume by retaining sodium (low urine sodium

Question 851

what is the most common nephrotic syndrome in children

Answer 851

minimal change disease

80-90% steroid sensitive

Question 852

how is the diagnosis of minimal change disease made

Answer 852

trial of steroids

Question 853

what is the treatment of minimal change disease

Answer 853

8 weeks prednisolone

Question 854

what are typical features of minimal change disease

Answer 854

5 years, normal blood pressure, haematuria (microscopic) but normal renal function.

Question 855

what are atypical features of minimal change disease that will require a biopsy

Answer 855

autoimmune conditions

steroid resistance

Question 856

what are some SE of long term steroids

Answer 856

more susceptible to infections, glucocorticoid toxicity from treatment, GI side effects, glucose intolerance, hypertension, risk of adrenal crisis, altered growth, behaviour (irritable, moody, don’t sleep) Check varicella status/ pneumococcal vaccination

Question 857

what is the outcome of treatment with prednisolone for minimal change disease

Answer 857

95% in 2-4 weeks,

80% relapse, 50% frequent

Question 858

what drugs would a child be put on if the have >4 relapses of MCD / year

Answer 858

immunosuppression - 
Levimazole, 
 Cyclosporin, 
Tacrolimus, , 
Rituximab (CD20 monoclonal antibody)
Alkylating agent (cyclophosphamide oral / nitrogen mustard IV),

Question 859

what is the risk of focal segmental glomerulosclerosis

Answer 859

end stage renal failure - need immunosuppression

Question 860

what congenital mutations will cause an <3 month infant to present with nephrotic syndrome

Answer 860

NPHS1 – nephrin/ NPHS 2 – podocin

Question 861

when would you investigate microscopic haematuria

Answer 861

Investigate > trace on 2 occasions or gross haematuria (persistent)

Question 862

name some things that may cause macroscopic haematuria

Answer 862

UTI (dysuria) – most common cause
Trauma
Stones (pain)
Glomerulonephritis – Post infective GN, IGA/ HSP, Membranoproliferative GN, lupus nephritis 
Benign recurrent haematuria (FH)
Alports syndrome
HUS
Tumour (WIlm’s)
Clotting abnormalities

Question 863

what class of AKI is caused by nephritic syndrome

Answer 863

intrarenal

Question 864

how would you investigate haematuria

Answer 864

Creatinine, FBC, Albumin, Urine
Exclude UTI – urine culture negative
Radiology (check anatomy), ANA/ ANCA antibodies (exclude auto-immune disease), biopsy

Question 865

what is a feature of fluid overload seen in the neck

Answer 865

raised JVP

Question 866

what bacteria usually causes post infectious GN

Answer 866

group A strep- b haemolytic

5-21 days after infection, throat (7-10 days) or skin (2-4 weeks)

Question 867

what is the pathogenisis of post infectious GN

Answer 867

antigen-antibody complex deposition in kidney – activates complement (levels drop)

Question 868

what is the prognosis of post infectious GN

Answer 868

most clear within 6 months and don’t reoccur

Question 869

how would you diagnose post infectious GN

Answer 869

bacterial culture, positive ASOT, low C3

Question 870

what is the treatment of post infectious GN

Answer 870

antibiotics (10 days penicillin), support renal function, volume overload - diuretics

Question 871

what is the most common cause of GN in adults worldwide

Answer 871

IgA nephropathy

Question 872

how many days after a upper respiratory tract infection would IgA nephropathy present

Answer 872

1-2 days

Question 873

what are clinical signs in the urine of IgA nephropathy

Answer 873

Recurrent macroscopic haematuria (frank)
chronic microscopic haematuria
Varying degree of proteinuria

Question 874

how is IgA nephropahty investigated

Answer 874

Clinical picture + Biopsy

Normal complement, negative autoimmine

Question 875

how is IgA nephropahty treated

Answer 875

treat proteinuria – ACEi,

moderate disease – immunosuppression

Question 876

what is henoch schonlein purpura

Answer 876

IgA related vasculitis

Question 877

when is the onset of henoch schonlein purpura

Answer 877

5-15 years

Question 878

what are diagnostic features of henoch schonlein purpura

Answer 878

Mandatory palpable purpura + one of 4

1. Abdominal pain
2. Renal involvement
3. Arthritis or arthralgia (joints)
4. Biopsy - IgA deposition + complement

Question 879

give causes of nephritic syndrome

Answer 879

post infectious GN 
IgA nephropathy
Henoch Schonlein purpura
membranoproliferative GN
lupus nephritis
ANCA positive vasculitis

Question 880

what is the trigger for IgA vasculitis in most children

Answer 880

viral URTI - strep

Question 881

how long does the rash last in IgA vasculitis

Answer 881

4-6 weeks

Question 882

what cell in injured in IgA vasculitis

Answer 882

mesengial

Question 883

what is the treatment of IgA vascultisi

Answer 883

symptomatic - joints, gut
Glucocorticoid therapy - (GI involvement)
Immunosuppression – prednisolone, cyclophosphamide

Question 884

what is an AKI

Answer 884

abrupt loss of kidney function, resulting in the retention of urea and other nitrogenous waste products and in the dysregulation of extracellular volume and electrolytes

Question 885

what are signs of AKI

Answer 885

¥ Anuria/oliguria (<0.5ml/kg/hr) > 8 hours
¥ Rapid rise in plasma creatinine
¥ Hypertension with fluid overload
¥ Serum creatinine: > 1.5x age specific reference creatinine

Question 886

how much does the serum creatinine rise in AKI

Answer 886

> 1.5x

Question 887

what is the difference in the creatine levels in AKI 1-3

Answer 887

1 - >1.5x baseline
2 - 2-3x baseline
3- >3x

Question 888

what are some pre-renal causes of AKI

Answer 888

Volume depletion  hypoperfusion of kidneys 
Oedematous states
Hypotension
hypoperfusion – 	Drugs
renal artery stenosis or occlusion
Hepato-renal syndrome.

Question 889

what are some post -renal causes of AKI

Answer 889

obstructive nephropathy

Question 890

what are some intrinsic causes of AKI

Answer 890

Glomerular disease – HUS, Glomerulonephritis
Tubular injury - acute tubular necrosis (ATN), Consequence of hypoperfusion, Drugs
Interstitial nephritis - NSAID, autoimmune

Question 891

what is the management of AKI (3M’s)

Answer 891

prevention
Monitor – Urine Output, PEWs, BP, weight (water)
Maintain – good hydration
Minimise – drugs (ibuprofen)

Question 892

what is monitored long term after an AKI

Answer 892

blood pressure and proteinuria - risk of CKD

Question 893

what are the treatment principles of treating metabolic bone disease

Answer 893

low phosphate diet
phosphate binders (Ca)
Active vit D to control PTH

Question 894

what is the role of PTH and how is this affected in end stage renal disease

Answer 894

PTH balances Ca in bones
PTH helps kidneys to excrete phosphate
if kidneys can’t excrete phosphate then PTH increases and Ca drops

Question 895

what is the best way to measure blood pressure in under 5s

Answer 895

doppler form wirst

Question 896

what is hypertension defined as in young children

Answer 896

> 95th percentile

borderline >90 but <95th – affected by age, sex, height, weight

Question 897

give factors that affect the progression of CKD

Answer 897

Late referral
Hypertension
Proteinuria
High intake of protein, phosphate and salt (CKD – normal protein, low phosphate)
Bone health - Vitamin D, PTH, Phosphate
Acidosis
Recurrent UTIs

Question 898

what are the grades of vesico- uteric reflux

Answer 898

1-ureter only
2-ureter, pelvis, calyces (no distension)
3-dilatation ureter
4-Moderate dilatation of ureter ± pelvis ±tortuous ureter, obliteration of fornices
5-gross dilatation/tortuosity, no papillary impression in calyces

Question 899

what are the guidelines for investigating UTI in children

Answer 899

Upper tract symptoms (fever, vomiting, systemically unwell), Younger (<6 months high risk), Recurrent

Question 900

what imaging is done to look at the anatomy of the kidneys

Answer 900

ultrasound

Question 901

what scan is done to look for scarring of the kidneys

Answer 901

DMSA (isotope scan)

Question 902

what scans are done to look for urine reflux

Answer 902

Micturating cystourethrogram/ MAG 3 scan

Question 903

what is the treatment of a lower UTI

Answer 903

3 days oral antibiotics -

- Trimethoprim, Co-amoxiclav, cephalosporin

Question 904

how would you treat an upper tract UTI or pyelonephritis

Answer 904

antibiotics for 7-10 days
oral if well - Trimethoprim, Co-amoxiclav, cephalosporin
IV - 3rd generation Cephalosporin or Co-amoxiclav

Question 905

what are complications of UTI

Answer 905

scarring > hypertension

AKI

Question 906

how may you diagnose a UTI

Answer 906

Dipstix – WCC, Leucocyte esterase activity, nitrites (+VE = UTI)
Microscopy - Pyuria >10 WBC per cubic mm
Bacturia
Culture > 10^5 Colony forming units /ml - (gold standard)

Question 907

what is the most common cause of UTI in kids

Answer 907

E.coli

Klebsiella, Proteus (stones), Strep Faecalus

Question 908

what is needed to diagnose a UTI

Answer 908

clinical signs
+ Bacteria culture from midstream urine
Any growth on suprapubic aspiration or catheter

Question 909

what are signs of an upper urinary tract infection

Answer 909

fever, vomiting, lethargy, irritability, abdominal pain/ tenderness, poor feeding

Question 910

what methods can be used to obtain a urine sample in children

Answer 910

• Clean catch urine or midstream urine in adults/ older
• collection pads, urine bags
• catheter samples or suprapubic aspiration (USS)

Question 911

what hereditary conditions can cause CKD in children

Answer 911

Cystic kidney disease
Cystinosis
Syndromic - Turner, Trisomy 21, Branchio-oto-renal, Prune Belly syndrome
bilateral cryptorchidism

Question 912

what does the GFR drop to before symptoms of CKD are seen

Answer 912

<60

Question 913

what is the most common cause of haemolytic uraemia syndrome

Answer 913

post diarrhoea

Entero-Haemorrhagic E.coli (EHEC)

Question 914

what organism commonly causes haemolytic uraemia syndrome

Answer 914

e.coli 0157

Question 915

after onset of bloody diarrhoea, how long is HUS a risk for?

Answer 915

14 days

Question 916

what is the triad of HUS

Answer 916

Microangiopathic haemolytic anaemias
Thrombocytopenia
Acute renal failure

Question 917

how is HUS treated

Answer 917

3Ms:
Monitor: Fluid balance, electrolytes, acidosis
Hypertension
Aware of other organs - Seizures, acute abdomen, diabetes, adrenal crisis
Maintain: IV normal saline and fluid – reduce oliguric HUS by fluid expansion + salt
Renal replacement therapy – 30-40% need RRT
Minimise: No antibiotics

Question 918

how does alport syndrome present

Answer 918

haematuria, proteinuria, hypertensions, early renal failure (20-30)
deafness
eye changes - lenticonus, macular changes in retina

Question 919

why do people with aport syndrome get deafness

Answer 919

collagen in cochlear membrane - SN deafness

Question 920

why do people with aport syndrome get lenticonus

Answer 920

collagen in decements membrane

Question 921

what are some acquired causes of cystic renal diabetes

Answer 921

cancer
Autosomal dominant glomerulocystic kidney disease
Early onset diabetes mellitus (MODY)
Genetic heterogeneity - HNF1β mutations

Question 922

what is seen in developmental cystic renal disease

Answer 922

Dysplasia/ Multicystic dysplastic (MCDK)
Usually sporadic, Non-functioning kidney, usually unilateral.
Ureteric atresia + Hypertrophy of the normal contralateral kidney

Question 923

what are some congenital causes of cystic renal disease

Answer 923

Autosomal Recessive (ARPKD)
Autosomal Dominant (ADPKD)
Juvenile Familial Nephronophthisis (JFN) - normal BP
Potters sequence

Question 924

what are extra renal features of Juvenile Familial Nephronophthisis (JFN)

Answer 924

normal BP
Syndromic:
Retinitis pigmentosa;
Hepatic fibrosis;
Skeletal dysplasia;
CNS abnormalities.

Question 925

what are extra renal features of ARPKD

Answer 925

severe hypertension
Hepatic fibrosis →
Portal hypertension.
Bacterial cholangitis.

Question 926

what are extra renal features of ADPKD

Answer 926

severe hypertension
Extrarenal cysts (liver and/or pancreas)
Cerebral aneurysms in 8% - familial clustering