Paediatrics

Question 1

What is the the inheritence and epidemiology of cystic fibrosis

Answer 1

autosomal recessive
1/25 have the CFTR protein mutation in white Europeans, 1/2500 have CF

Question 2

What is the pathology of CF

Answer 2

trinucleotide deletion on chromosome 7= misfolded protein= CFTR mutation
this mutations the chloride channel which leads to a change in chloride transport across cell membranes and causes mucous secretions in different systems to be very thick

Question 3

What is a characteristic sputum presentation of CF

Answer 3

thick sputum with pus ‘cupfuls’
can be brown (chronic)/ yellow/ green (if infection)

Question 4

If both parents have the faulty gene, what is the inheritance patterns of their offspring

Answer 4

1/4 child with CF
1/2 carrier
1/4 not CF and not carrier

Question 5

If both parents are healthy, one sibling has cystic fibrosis and a second child does not have the disease and is not a carrier, what is the likelihood of the second child being a carrier?

Answer 5

The child in question cannot have CF because his sibling (1/4) does. This means the child has 2/4 chance of being a carrier but because there is already a child with CF, it goes down to 2/3

Question 6

Presentation of CF (7 Cs) and explain them

3 resp, 2 GI, 1 reproductive, 1 development

Answer 6

Chronic cough
reCurrent lower resp infections (due to reduced clearance of mucus from airways)
Crackles on auscultation (due to thick mucus in lungs)
low weight/ height on growth Charts (can lead to failure to thrive due to reduced ADEK absorption)
meConium ileus (thick mucus causes delay in meconium- baby’s first poop- for over 24 hours and with abso distention and steatorrhea
Congenital bilateral absence of vas deference in males (healthy sperm but no way of sperm to reach testes for ejaculation= infertility in males)
panCreatitis/ laCk of digestive enzymes (thick secretions in GI causes blockages of ducts= lack of digestive enzymes eg pancreatic lipase= reduced fat absorption= reduced ADEK absorption and steatorrhea)

Question 7

Diagnostic tests for CF, explain each one and which is GS

Answer 7

1. heel prick test in first few days (positive result= raised blood immunoreactive trypsinogen) SCREENING
2. sweat test GS (sweat sample induced by electrodes on a aptch of skin sent to lab, diagnostic result= chloride concentration above 60mmol/L)
3. genetic testing for CFTR gene during pregnancy via amniocentesis

Question 8

What are 2 common colonisers in CF children and management for both

Answer 8

1. staph aureus- long term prophylactic flucoxacillin
2. pseudomonas- hard to treat and worsens CF prognosis- treated long term with nebulised antibiotics tobramycin and oral ciprofloxacin

Question 9

What is the medical management of cystic fibrosis 5

Answer 9

1. prophylactic antibiotics
2. bronchodilators eg salbutamol
3. medicines to thin secretions (dornase alfa)
4. creon (pancreatic anzyme replacements)
5. ADEK vitamin supplements

Question 10

What are the medical condition complications of cystic fibrosis 3 and how are these mitigated

Answer 10

1. cystic fibrosis associated liver disease (blocks ducts in liver)
2. CF related diabetes (pancreas does not make enough insulin due to pancreatic scarring/ blockages)
3. malabsorption causing deficiencies and ostseoporosis (due to low vit D and calcium absorption)
   ->regular reviews for intestinal absorption, diabetes and other complications twice a year as adults and every few weeks as a child/ at the beginning of diagnosis

Question 11

What is pneumonia

Answer 11

Infection of the lung tissue which can cause inflammation of tissue and sputum build up in airways and alveoli

Question 12

What is the presentation of pneumonia in children 4

Answer 12

wet and productive cough
high fever over 38.5
tachypnoea
lethargy

cOugh, Over, tachypnOea, Overly tired (4 Os)

Question 13

What are clinical signs of pneumonia that can indicate sepsis 4

Answer 13

tachycardia
hypoxia
hypotension
confusion/ delerium

Question 14

What are the 3 characteristic chest signs of pneumonia on auscultation

Answer 14

1. bronchial breath sounds (harsh sounds equally loud on inspiration and expiration due to consolidation of lung tissue)
2. focal course crackles (due to air passing through sputum)
3. dullness to percussion (due to consolidation/ lung tissue collapse)

Question 15

Causes of pneumonia

Answer 15

Bacterial:
streptococcus pneumonia (MC in adults)
Haemophilus influenza type b (MC in under 5s)
Group A strep (pyogenes)
Group B strep (MC in neonates)
mycoplasma pnuemonia (MC in over 5s)

Viral:
Respiratory syncytial virus (RSV)

Question 16

Ix of child pneumonia 4

Answer 16

1. chest xray for diagnostic doubt/ severe cases (not routinely required)
2. sputum cultures and throat swabs for bacterial cultures to identifying causative organism
3. viral PCR to identifying causative organism
4. capillary blood gas to monitor respiratory function

Question 17

How can pneumonia be identified on a chest xray

Answer 17

consolidation (patchy usually)

Question 18

management of child pneumonia 3

Answer 18

1. antibiotics
   -> amoxicillin first line with added macrolide (erythromycin/ azithromycin)
   -> in penicillin allergy, use macrolide as monotherapy
2. IV antibiotics only when sepsis/ intestinal absorption issues
3. O2 to maintain sats over 92%

Question 19

Common Ix for recurrent lower resp tract infections 4

Answer 19

bloods- FBC for wbc and capillary blood gas
chest xray (scarring/ structural abnormality)
sweat test (for CF)
HIV test

Question 20

What is bronchiolitis and HOW DOES IT HAPPEN, main cause and what are the 3 signs of a bronchiolitics baby’s chest on auscultation

Answer 20

inflammation/ infection of bronchioles (lower resp)
due to viral infection (usually respiratory synctial virus) which causes mucus production in infants which can reduce air that can get to and leave the alveoli= bronchial breath sounds, wheeze and crackles

Question 21

Epidemiology of Bronchiolitis

Answer 21

common in winter
affects under 1 year olds
affects up to 2 year olds if they have chronic lung disease

Question 22

Presentation of bronchiolitis 3

Answer 22

1. coryzal symptoms (runny nose, sneezing, mucus in throat, watery eyes)
2. dyspnoea and tachypnoea (heavy and fast breathing)
3. fever

Question 23

What can bronchiolitis lead to 2

Answer 23

1. apnoeas (episodes where child stops breathing)
2. respiratory distress

Question 24

Signs of respiratory distress 4

Answer 24

1. use of accessory muscles (sternocleidomastoid, abdominal and intercostal muscles)
2. . cyanosis
3. . abnormal airway noises
4. tracheal tugging

BANT (blue, accessory, noises, tugging)

Question 25

typical progression of bronchiolitis

Answer 25

starts with upper resp infection with coryzal symptoms
peaks at 3/5 days
recover between 2 weeks
often have a long-term bronchiolitic cough afterwards

Question 26

crou

When can bronchiolitis patients be admitted to hospital 4

Answer 26

apnoeas
half of their milk intake
resp rate above 70 or sats below 92
under 3 months with pre existing condition eg CF

Question 27

Management of bronchiolitis 2

Answer 27

1. ensure adequate intake (oral/ NG/ IV) but avoid overfeeding because a full stomach can restrict breathing so small frequent feeds and gradually increase as tolerated
2. O2 if under 92

Question 28

Ventilatory support step up 4

Answer 28

1. high flow humidified O2 via tight nasal cannula (oxygenates and prevents lung collapse at end of expiration)
2. continuous positive airway pressure (CPAP) via sealed nasal cannula with higher and more controlled pressures
3. non re-breath mask
4. intubation and ventilation via endotracheal tube to fully control ventilation

CHECK ORDER

Question 29

How to assess ventilation 1

Answer 29

capillary blood gas

Question 30

CLINICAL signs of poor ventilation 2

Answer 30

high pCO2= airways collapsed and cannot clear CO2
low pH= respiratory acidosis where CO2 is building up (if with hypoxia then type 2 resp failure)

Question 31

Prophylaxis against RSV and who is this given to only?

Answer 31

palivizumab monoclocal antibody for RSV
given as a monthly injection
to high risk babies only eg premature

Question 32

Diagnosis of bronchiolitis 2

Answer 32

viral PCr to confirm causative organism eg RSV
clinical diagnosis

Question 33

What is Asthma

Answer 33

Reversible constriction of the airways with followed by airway remodelling

Question 34

What are the risk factors for asthma 4

Answer 34

genetic
premature
parental smoking
allergen exposure eg dust/ mould

Question 35

Clinical presentation of asthma 3

Answer 35

EXPIRATORY wheeze
dry cough worse at night (diurnal variation)
progressively worsening SOB

Question 36

Ix and results for asthma 4

Answer 36

1. peak flow: FVC normal, FEV1 reduced, FEV1:FVC ratio under 70% if poorly controlled
2. spirometry: reversible (only for over 5s)
3. fractional exhaled nitric oxide (correlate to inflammation)
4. do a baseline xray

Question 37

Signs of moderate asthma 2

Answer 37

peak flow over 50% predicted and normal speech

Question 38

signs of severe asthma 4

Answer 38

peak flow under 50% predicted
cannot complete a sentence in one breath
sat under 92
signs of resp distress

Question 39

signs of life threatening asthma 4

Answer 39

peak flow under 33% predicted
silent chest (means no air entry due to airway constriction)
cyanosis
altered consciousness/ confusion

Question 40

management for asthma for non paediatrics (non emergency) 8

Answer 40

1. SABA
2. add ICS beclomethasone low dose
3. add LTRA
4. add LABA and cont LTRA if good response
5. switch ICS/LABA to low dose MART
6. titrate up to med dose MART OR change to mod dose ICS and separate LABA
7. increase ICS to high dose as a separate inhaler, or trial theophylline/ LAMA and refer to specialist who can add oral steroids at the lowest dose possible to achieve good control under specialist guidance.

-> if asthma well controlled for 3 months then consider decreasing maintenance therapy (maintenance therapy= drugs that aim for long term mx, not symptomatic relief)

Question 41

asthma management for under 5s

Answer 41

1. SABA PRN
2. SABA plus 8 week trial ICS (restart if symptoms reoccur within 4 weeks of finishing trial)
3. refer to specialist

Question 42

acute (emergency) mod- severe asthma management

Answer 42

OSHIIE
Oxygen
Salbutamol
Hydrocortisone
Ipratropium bromide (nebuliser)
IV magnesium
to escalate this further: call anaesthetics and ICU for intubation and ventilation

Question 43

What is the mx for acute asthma exacerbation that can be handled in community?

Answer 43

prednisolone for 3-5 days (steroids should be given to all children with an asthma exacerbation)

Question 44

What is croup

Answer 44

upper resp tract infection which causes oedema in the larynx

Question 45

causes of croup

Answer 45

MC parainfluenza or RSV
used to be caused by diptheria which lead to epiglottis and has high mortality but is not common due to vaccination

Question 46

age of croup

Answer 46

6 months to 2 years

Question 47

presentation of croup 4

Answer 47

SOB
stridor
barking cough in clusters of coughing episodes
hoarse voice

Question 48

Management of croup 1, 3

Answer 48

1. supportive treatment at home with fluids and rest, not going to school, until 3 days of being ill or if fever has gone for 24 hours
   IF SEVERE
   1. oral dexamethasone 0.15mg/kg
2. oxygen if needed
3. nebulised adrenaline for relief of severe symptoms

Question 49

complications of croup 3

Answer 49

otitis media
secondary infection eg pneumonia
dehydration

Question 50

What is acute epiglottitis and main cause and why is it life threatening

Answer 50

inflammation of epiglottis due to infection
MC= haemophilus influenza type B
life threatening emergency as it can swell and obscure the airway within hours of first symptoms

Question 51

Clinical presentation of epiglottitis 5

5 Ds

Answer 51

dysphagia
dysphonia
drooling
distress
tripoD position (sat forward, hand on each knee)

Question 52

investigations for epiglottitis 2 and what should you not examine 1

Answer 52

clinical
thumb sign on lateral chest x ray with acute epiglottis swelling
DO NOT examine throat

Question 53

management of epiglottitis 5

Answer 53

1. alert senior paediatrician and aneasthetist
2. secure airway if patient’s condition is severe (intubation and transfer for ICU at any time just incase it closes)- not usually required
3. oxygen
4. nebulised adrenaline
5. IV antibiotic 3rd gen cephalosporin eg ceftriaxone

Question 54

progression/ complication of epiglottitis 1

Answer 54

epiglottic abscess with pus build up around epiglottis

Question 55

What is a virally induced wheeze and why does it occur only in small children

Answer 55

wheeze due to a viral illness, in children under 3 where inflammation and oedema can restrict their small airways significantly

Question 56

3 differences between asthma vs virally induced wheeze

Answer 56

virally induced wheeze triggered by virus alone , no other allergens
virally induced wheeze usually under 3 years
virally induced wheeze has no atopic history

Question 57

what does a focal wheeze indicate and next step

Answer 57

hecfocal airway obstruction eg tumour or foreign object
urgent senior review

Question 58

typical presentation of virally induced wheeze 4

Answer 58

1-2 days coryzal symptoms with fever and cough
then SOB, respiratory distress and expiratory wheeze throughout chest

Question 59

Mangement of virally induced wheeze 2

Answer 59

only if symptoms:
1. SABA inhaler via spacer maximum of 4 hourly up to 10 puffs
2. LTRA and ICS via spacer
3. passmed says second line is montelukast

Question 60

What is Otitis media

Answer 60

infection of middle ear (space between tympanic membrane and inner ear)

Question 61

2 main causes of otitis media and how does this occur

Answer 61

strep pneumoniae MC, haemophilus influenzae
bacteria often enters from mouth via eustachian tube

Question 62

Presentation of otitis media 4

Answer 62

ear pain in affected ear
reduced hearing in affected ear
discharge
fever

Question 63

investigation for otitis media 1 and results

Answer 63

otoscope: bulging red inflammed tympanic membrane (normal appearance is shiny grey)

Question 64

what is the management for otitis media 2

Answer 64

1. self resolves, take paracetomol for pain/ fevers
2. if more serious/ under 2 years old (NICE doesn’t like giving antibiotics for otitis media) then amoxiciliin for 5 days (erythromycin if penicillin allergy)

Abx indications:
tympanic membrane is perforated
the child is under 3-months old
the child is under 2 years AND the infection is bilateral
if symptoms are present for 4 or more days

Question 65

What is glue ear and main symptom

Answer 65

otitis media with effusion (middle ear becomes full of fluid= hearing loss in the ear)

Question 66

main complication of glue ear

Answer 66

recurrent infection (otitis media)

Question 67

management for glue ear 3

Answer 67

1. usually resolves within 3 months by itself so conservative treatment
   if continuing past 3 months/ co-morbidities then requires audiometry referral which will lead to hearing aids or grommets

Question 68

What are grommets and who typically gets given them

Answer 68

tubes inserted into the tympanic membrane via day case GA surgery
this allows for fluid to drain from middle ear through tympanic membrane to external ear
these fall out within a year

chronic glue ear

Question 69

What are the two categories of hearing loss

Answer 69

congenital and acquired due to childhood illness

Question 70

what are causes of hearing loss 3, 2, 3

Answer 70

congenital:
rubella or cytomegalovirus infection during pregnancy
genetics: autosomal recessive MC
Downs syndrome

labour:
premature
hypoxia at birth

after birth:
jaundice
meningitis/ encephalitis
otitis media/ glue ear

Question 71

How is hearing loss screened for in the UK?

Answer 71

newborn hearing screening programmes (NHSP) in all neonates (around 5 weeks old)
automated otoacoustic emissions test, if issues automated auditory brainstem response test’

Question 72

Why does Gastro-oesophageal reflux occur in paeds

Answer 72

immaturity of the lower oesophageal sphincter which causes stomach contents to reflux into oesophagus

Question 73

When are children most likely to have a G-O reflux and when does this typically stop

Answer 73

usually after a large feed
usually stops after 1 year

Question 74

Presentation of gastro oesophageal reflux in children 5

Answer 74

chronic cough
hoarse cry
distress after feeding
poor weight gain
-> in over 1 year old, can experience heartburn and acid regurgitation (adult like symptoms)

Question 75

Management of GORD 6

Answer 75

1. conservative: smaller volume more frequent meals, regular burping, keep baby upright after feed 2 weeks
2. 2 weeks trial thickened formula if formula fed- milk mixed with starch
3. 2 week trial gaviscon (alginate therapy)
4. 4 weeks PPI eg omeprazole
5. referaal to paeds if not responding to tx/ issues with faltering growth
6. fundoplication surgery (folds fundus around lower oesophagus to reinforce the LOS)

Question 76

Investigation for GO reflux 2 and when is this done

Answer 76

1. barium meal with xray
2. endoscopy
   rarely and only when further Ix is required

Question 77

What is sandifer’s syndrome, outcome

Answer 77

abnormal muscle contractions of back/ neck which are due to GOR
this presents as muscle contractions for a few minutes after a feed (pain response to GOR)
sandifers resolves as reflux improves

Question 78

What is pyloric stenosis- explain physiology

Answer 78

narrowing of the pyloric sphincter which is between the stomach and duodenum
this means the stomach has to perform more powerful peristalsis to push food into duodenum
the power of the peristalsis can eject the food into the oesophagus, out of the mouth and across the room= projective vomiting

Question 79

Presentation of pyloric stenosis 3

Answer 79

non billous projectile vomiting after every feed
weight loss
palpable olive sized mass over upper abdomen where pyloric sphincter is hypertrophied

Question 80

Investigation for pyloric stenosis 2

Answer 80

1. blood gas shows hypochloric (low Cl-) and hypokalaemic (low K+) metabolic alkalosis (as baby is vomiting out the acid from the stomach)
2. diagnosed by abdo US which shows thickened pylorus muscle

Question 81

Management for pyloric stenosis 2

Answer 81

1. Ramstedt’s pyloromyotomy which widens the hole between the stomach and duodenum (quick and good prognosis)
2. can give fluids for hypovolaemia

Question 82

What is biliary atresia and what does this cause

Answer 82

congenital condition with an sclerotic section of the bile duct, reducing bile flow
prevents normal excretion of conjugated bilirubin (which is the normal function of bile duct)

Question 83

Presentation of biliary atresia 3

Answer 83

jaundice > 3 weeks
dark urine
pale stool

Question 84

Ix for biliary atresia 4

Answer 84

1. blood test: conjugated and unconjugated bilirubin (positive test= high conjugated bilirubin)
2. LFT (raised)
3. US for structural abnormalities
4. definitive mx= cholangiography (x-ray plus contrast dye)

Question 85

Management for biliary atresia 2

Answer 85

1. surgical resolvement- Kasai procedure
2. complete resolution often requires a full liver transplant

Question 86

Complications of biliary atresia 2

Answer 86

1. cirrhosis of liver
2. hepatocellular carcinoma

Question 87

What is intussusception and what does this do

Answer 87

Section of bowel that ‘telescopes’ (folds inwards) into another section of bowel
this narrows the lumen and obstructs the passage of faeces through the bowel

Question 88

Where is the most common location of intussusception and who does it affect

Answer 88

ileocecal junction in distal ileum
6 months- 2 year old boys mostly

Question 89

Associated conditions that increase risk of intussusception 4

Answer 89

meckel’s diverticulum
henoch-schonlein purpura
cystic fibrosis
viral illness

Question 90

What is the most common cause of obstruction in neonates

Answer 90

intussusception

Question 91

Presentation of intussusception 4

Answer 91

severe colicky abdo pain
stool: red current jelly stool (like jam)- late in presentation
sausage shaped palpable mass in RUQ
signs of intestinal obstruction (vomiting, constipation, abdo distention)

4 Ss (like lots of Ss in intussusception)

Question 92

Investigation of Intussusception 1

Answer 92

1. Diagnosed by US abdo with a mass that looks like an arrow target

Question 93

Management of Intussusception

Answer 93

1. IV fluids
2. Air enema (air is pumped into colon to force the bowel to straighten into its normal position
3. If enema unsuccessful/ perforation/ gangrenous, surgical restoration required
4. if perforation and peritonitis then broad spec gentamycin

Question 94

complications of intussusception 3

Answer 94

obstruction
perforation
peritonitis

Question 95

What is gastroenteritis and its presentation 3

Answer 95

inflammation from stomach to intestines
nausea, vomiting, diarrhoea

Question 96

Causes of gastroenteritis (MC)

Answer 96

cause: most likely viral
viral: rotavirus/ norovirus
bacterial: E.coli, bacillus cereus (from left out rice)

Question 97

Ix and mx for gastroenteritis 3

Answer 97

-> stool microscopy, culture and sensitivities to identify causative organism
1. isolate patient because it is highly contagious
2. oral/ IV fluids (dehydration is a major concern)

Question 98

Why are antibiotics typically not required for gastroenteritis

Answer 98

antibiotics not required usually unless high risk of complications because it increases risk of haemolytic uraemic syndrome

Question 99

why are antimobility medications not recommended for bloody stool or bacterial gastroenteritis

Answer 99

increases risk of toxic megacolon

Question 100

complications of gastroenteritis 4

Answer 100

1. lactose intolerance
2. Irritable bowel syndrome
3. Reactive arthritis
4. Guillain–Barré syndrome

Question 101

What is appendicitis and explain the progression

Answer 101

inflammation of appendix
infection and obstruction of appendix leads to gangrene and perforation
this results in release of faeces into abdominal cavity
this leads to peritonitis (inflammation of peritoneal cavity)

Question 102

Presentation of appendicitis- symptoms 4

Answer 102

1. central abdo pain which localises in RIF
2. nausea
3. vomiting
4. loss of appetite

Question 103

Clinical signs of appendicitis 5

Answer 103

1. tenderness at McBurney’s point (2/3 distance from umbilicus to ASIS)
2. Rosving’s sign (palpation of left IF causes pain in RIF)
3. guarding on abdo palpation
4. rebound tenderness (pain when quickly releasing pressure on RIF)- this suggests peritonitis caused by a ruptured appendix
5. Obturator sign (pain on passive internal rotation of the hip when the right knee is flexed)

G RORT

Question 104

Investigations of appendicitis 5

Answer 104

-bloods: FBC (high neutrophils), CRP
-urine dip (exclude UTI, appendicitis can have high leukocytes without UTI)
-pregnancy test to exclude ectopics
-US (first line imaging) if not definitive from other ix, otherwise clinical dx
-if everything else negative then do exploratory laparoscopy to visualise appendix

Question 105

Management of appendicitis 1

Answer 105

emergency appendicectomy (laproscopic)

Question 106

Differentials of appendicitis 3

Answer 106

ectopic pregnancy (take pregnancy test!)
meckel’s diverticulum
ovarian cysts

Question 107

Complications of appendicitis 2

Answer 107

1. peritonitis
2. sepsis

Question 108

What are the two types of constipation

Answer 108

1. idiopathic/ functional- no underlying cause
2. secondary causes

Question 109

What are the secondary causes of constipation 6

Answer 109

hirschprungs
CF
hypothyroidism
intestinal obstruction
cows milk intolerance
abuse (eg sexual abuse)

Question 110

Presentation of constipation 4

Answer 110

less than 3 stools a week
difficulty stools to pass
rabbit dropping stools
abdominal pain

Question 111

Red flags of constipation 5

Answer 111

1. not passing meconium within 48 hours of birth (CF/ hirschprungs)
2. neurological signs/ abnormal back pain (can suggest spinal cord lesion)
3. vomiting (intestinal obstruction/ Hirschprungs)
4. failure to thrive ie poor growth (hypothyroidism/ coeliac)
5. blood in stools

Question 112

how is idiopathic constipation diagnosed

Answer 112

clinical, without investigations

Question 113

Management of constipation for children 3

Answer 113

1. ensure good hydration, high fibre diet
2. laxatives: macrogol osmotic laxative first line long term then weaned off when a regular bowel habit is established
3. encourage/ praise going to toilet eg star charts to prevent withholding
   in children, first line involves a combination of medical, dietary and behavioral managment as above

Question 114

What is the mx ladder for constipation in normal adults 5

Answer 114

1. 4 weeks of increased fibre, fluids and exercise
2. 2 months of bulk forming laxative eg isphagula husk
3. 2 months of osmotic laxartive eg lactulose/ macrogol
4. 2 months of stimulant laxative eg senna/ sodium picosulfate
5. if still constipated, stop all laxatives and take prucalopride 1mg

Question 115

What are the risks of stimulant and osmotic laxatives

Answer 115

stimulant: if used long term, can make the bowel lazy and dependant on this medication
osmotic: can cause electrolyte imbalances and dehydration

Question 116

What is Meckel’s diverticulum and where is this located? Is it always something to be treated?

Answer 116

Congenital outpouching (diverticulum) of the distal ileum- 2cm from the ileocaecal valve
asymptomatic and doesn’t require treatment unless complications occur

Question 117

What are the complications of meckel’s diverticulum 4?

Answer 117

1. risk of peptic ulceration of omphalomesenteric artery= rupture and bleed
2. volvulus/ intussusception

Question 118

Presentation of meckel’s diverticulum 3

Answer 118

abdominal pain
rectal bleeding in kids 1-2 years
obstruction due to intussusception/ volvulus as a complication

Question 119

ix and management of meckel’s diverticulum 1, 1

Answer 119

ix- 99 technetium scan
surgical resection of diverticulum if symptomatic

Question 120

What is a common differential diagnosis to failure to thrive

Answer 120

UTI

Question 121

Compare marasmus and kwashiokor

Answer 121

Marasmus is the complete deficiency of all macronutrients- proteins, carbs and fats
Kwashiokor is a predominantly protein deficiency

Question 122

What is infantile colic and what is its epidemiology and main characteristic and cause

Answer 122

episodes of excessive crying and pulling up of legs
worse in evening
no known cause

Question 123

What is the diagnostic criteria of infantile colic 3

Answer 123

1. infant less than 5 months when symptoms start/ stop
2. recurrent and prolonged episodes of crying without an obvious cause
3. no illness signs eg fever or signs of poor growth

Question 124

What is the management for infantile colic 3

Answer 124

1. reassure parents- encourage them to look after their own wellbeing
2. advice for calming child- holding baby, white noise
3. specialist paediatric referral if parents unable to cope or faltering growth or continuing over 5 months

Question 125

What is hirschsprungs and the pathophysiolocy. How does it present? 2

Answer 125

a section of bowel has missing nerve cells due to failure of parasympathetic auerbach and meissners plexus to develop= functional obstruction
presents as delayed meconium passing in neonates and constipation in older children

loss of ganglionic cells in myenteric plexus

Question 126

What is the investigation for hirschsprungs 2

Answer 126

Abdo x-ray with contrast (contrast enema)
GS diagnosis- rectal suction biopsy (shows absence of nerve cells)

Question 127

What is the management for hirschsprungs 2

Answer 127

bowel irrigation
definitive management: surgery of affected segment of colon

Question 128

What is cows milk protein allergy and who is affected

Answer 128

allergic reaction to protein in cow’s milk, seen mostly in formula fed children in first 3 months of life

Question 129

What are the two types of CMPA and how does each change the presentation. Which is more common

Answer 129

IgE mediated- symptoms shown within 2 hours of feed
non IgE mediated- symptoms shown between 2 hours and 1 week of feed
non IgE is most common

Question 130

What are the two main proteins involved in CMPA

Answer 130

casein and whey protein

Question 131

What are the clinical features of CMPA 4

Answer 131

GI: vomiting + diarrhoea
SKIN: skin rash- hives
RESP: chronic cough

Question 132

What are the investigations 2 for CMPA

Answer 132

Ix:
1. (sensitive but not specific) skin prick/ patch testing or IgE testing for cow’s milk protein
2. GS: oral food challenge

Question 133

What is the management for CMPA for formula fed and breastfed children 3

Answer 133

first line replacement for formula: extensive hydrolysed formula
if severe: give amino acid-based formula
if breastfeeding: mum to eliminate cows milk protein from diet

Question 134

What is a choledochal cyst and symptoms 3 and prognosis 1

Answer 134

dilation of the biliary ducts which causes poor bile flow
abdo pain, jaundice, abdominal mass
higher rate of cancer of bile duct in adulthood

Question 135

What is neonatal hepatitis syndrome and symptoms 4

Answer 135

liver inflammation that affects neonates
jaundice, enlarged liver and spleen, malabsorption, failure to thrive

Question 136

What are the main causes of neonatal hepatitis 3

Answer 136

1. cholestasis (impaired bile flow)
2. genetics (alpha 1 antitrypsin deficiency)
3. viruses (hep a/b/c, rubella, cytomegalovirus)

Question 137

What are the causes of liver failure 3 in children and what type are each

Answer 137

biliary atresia- chronic
virus- acute
hepatitis- chronic

Question 138

What is the most common hernia in neonates and children. What two risk factors increase your chance?

Answer 138

Inguinal
male and premature

Question 139

What do each of the LFTs mean? 5

Answer 139

ALT>AST= NAFLD, AST>ALT alchol related liver disease
raised GGT alone= alcohol
raised GGT + ALP= liver obstruction
raised ALP alone= increased bone turnover
very high ALT is an indicator of acute hepatitis

Question 140

What is the management for an inguinal hernia 1 and how does this change depending on age

Answer 140

herniotomy
children of a few months- highest risk of strangulations so urgent herniotomy
children of 1+ age are of lower risk= elective surgery

Question 141

What is coeliacs disease

Answer 141

autoimmune reaction to gluten

Question 142

What is the clinical presentation of coeliacs 5

Answer 142

1. failure to thrive in young children
2. anaemia, secondary to iron/B12/folate deficiency
3. steatorrhoea
4. dermatitis herpetiformis (elbows, knees, buttocks, back, or scalp)
5. mouth ulcers

Question 143

What is coeliacs genetically associated with 2 and what condition is closely linked to this

Answer 143

HLA-DQ 2/8
type 1 diabetes

Question 144

What are the investigations for coeliacs and results if applicable 5

Answer 144

1. anti-TTG (sensitive) and anti-EMA (specific) and total IgA (low IgA can make anti TTG and EMA appear low)
2. endoscopy and intestinal biopsy showing crypt hypertrophy and villous atrophy

Question 145

What is the management for coeliacs

Answer 145

gluten free diet (stay away from BROW- barley, rye, oats and wheat)

Question 146

Name the features of Crohns 4

Answer 146

N – No blood or mucus (these are less common in Crohns.)
E – Entire GI tract
S – “Skip lesions” on endoscopy
T – Terminal ileum most affected and Transmural (full thickness) inflammation
Crohn’s (crows NEST)

Question 147

Name the features of ulcerative colitis 5

Answer 147

Ulcerative Colitis (remember U – C – CLOSEUP)

C – Continuous inflammation
L – Limited to colon and rectum
O – Only superficial mucosa affected
S – Smoking is protective
E – Excrete blood and mucus
U – Use aminosalicylates
P – Primary sclerosing cholangitis

Question 148

What are the general clinical features of IBD 4

Answer 148

abdo pain
diarrhoea
weight loss
anaemia

Question 149

What are the Extra-Intestinal Manifestations of IBD 8

Answer 149

A PIE SAC
Aphthous Ulcers
Pyoderma Gangrenosum
Iritis
Erythema Nodosum
Sclerosing Cholangitis (for UC only)
Arthritis
Clubbing of Fingertips

Question 150

Investigations for IBD 6

Answer 150

1. FBC + CRP (indicates active inflammation)
2. coeliac screen to exclude coeliacs
3. stool microscopy and culture to exclude c difficile
4. TFT to exclude hyperthyroidism
5. faecal calprotectin
6. endoscopy with biopsy GS

Question 151

What is the management for crohns (2 stages) 3

Answer 151

inducing remission:
1st line steroids
then mesalazine (aminosalicylate)
maintaining remission:
azathioprine (DMARD to immunosuppress)
surgically resect areas (if it only affects distal ileum)

SMAS

Question 152

What is failure to thrive

Answer 152

poor physical growth and development

Question 153

What is the management of UC (2 stages) 6

Answer 153

inducing remission:
1st line mesalazine (aminosalicylate), 2nd line pred
IF severe: IV hydrocortisone then IV ciclosporin (DMARD)
maintaining remission:
mesalazine (aminosalicylate)
surgery can remove the disease fully- patient left with an ileostomy or J pouch

Question 154

What are the initial Ix for failure to thrive 2

Answer 154

1. urine dipstick for UTI
2. coeliac screen

Question 155

What is the genetic abnormality in turner’s syndrome

Answer 155

in females, one x chromosome in the sex chromosome pair is fully or partially missing
45X

Question 156

Clinical features of Turners 4

Answer 156

• short stature
• webbed neck
• widely spaced nipples
• primary ammenorrhoea (not having period by age 15)
• BICUSPID AORTIC VALVE

Question 157

What does turners put you at increased risk of 5

Answer 157

1. coarctation of aorta + ejection systolic murmur
2. horshoe kidney
3. autoimmune conditions: hypothyroisism, T2DM, coeliacs
4. infertility due to streaky ovaries

Question 158

How is turners diagnosed 3

Answer 158

Before birth: amniocentesis and chorionic villous sampling
After birth: karyotype analysis

Question 159

Management of turners 2

Answer 159

basically symptoms management:
-human growth hormone therapy somatropin (to increase height)
–oestrogen replacement therapy (for development of female sexual characteristics)

Question 160

What is the genetic abnormality in Downs syndrome

Answer 160

trisomy 21

Question 161

What is a risk factor that increases Downs syndrome risk

Answer 161

greater maternal age

Question 162

What are the clinical features of Downs syndrome 5

Answer 162

-> flat face and nose
->prominant epicanthic folds (Aegyosal)
-> single palmar crease
-> brachycephaly (small head with back of head being flat)
-> hypotonia

Question 163

What does downs put you at increased risk of 4

Answer 163

-> learning disabilities
-> vision problems (refractive errors)
-> hearing issues due to reccurent otitis media + glue ear
-> septal defects (atrioventricular septal defect)

travel down from brains

Question 164

How is Down’s, Pataus and Edwards syndrome screened for and when is this done?

Answer 164

combined test at 10-14 weeks (measures nuchal translucency measurement in an US & beta HCG and pregnancy associated plasma protein in the blood test)

after 14 weeks: quadruple blood test (beta HCG, alpha fetoprotein, inhibin A and unconjugated estriol)
-> downs is high hcg and inhibin A and low estriol and alpha fetoprotein
-> edwards is low for everything

if quadruple test indicated high risk then women can have non invasive prenatal testing (NIPT) which is very sensitive/ specific and no risk of miscarriage OR diagnostic test of amniocentesis and chorionic vilus sampling

These women will be confirmed to have Downs with genetic testing postnatally:
chromosomal karyotype will show trisomy 21

Question 165

What is the genetics behind Kleinfelters and what are the clinical features 3

Answer 165

47 XXY (males have an extra x chromosome= female characteristics)
infertility, gynaecomastia, testicular atrophy

Question 166

What is the genetic abnormality in Edwards. Clinical features of Edwards, ix for edwards

Answer 166

Trisomy 18
EDWARDs mneumonic
Elongated head (like alien)
Digits overlap when holding fist clenched
W (V) VSD
Apnoea= leading cause of death
Rocker Bottom feet
Dyplasia of kidney- horseshoe kidney

in quadriple test, everything is low or normal

Question 167

What is the genetic abnormality in Pataus syndrome and clinical features

Answer 167

Trisomy 13
cleft lip, very small eyes, rocker bottom feet
PDA

PPATaus
Polydactyly
PDA
Abnormally small eyes
clefT lip

Question 168

What is the abnormality in Fragile X syndrome and clinical features 6. Inheritence, mutation.

Answer 168

where the x chromosome is abnormally susceptible to damaged, especially by folic acid deficiency
mutation: fragile messenger ribonuecloprotein 1 (FMR1) which causes 200+ CGG repeats- this condition has anticipation so there are increaseing repeats per generation
inheritence: x linked dominant

in males, FXTAS (fragile x assocaited tremo and ataxia syndrome)= tremor + ataxia seen in males after 50
in females, FXPOI (fragile x primary ovarian insufficiency)= irregualar/ absent periods and early menopause

1. prominent facial features: long face, low set ears
2. learning difficulities
3. after puberty, large testes
4. ADHD
5. anxiety
6. mitral valve prolapse

Question 169

How is fragile x syndrome inheritered and how does this affect expression

Answer 169

x linked dominant pattern
-> if a dad is affected, daughters will be affected and sons will not because dads only give their affected x to daughters
-> if mum is affected, 50% of all children will inherit condition
-> however, males express this fully and females have variable expression so they can have less severe clinical features

Question 170

What is the abnormality in prader willi syndrome.

Answer 170

genetic mutation on chromosome 15- loss of paternal contribution= only one copy is active

Question 171

What is the abnormality in williams syndrome- what is a clinical features

Answer 171

Deletion of genes on chromosome 7 (partially deletion)
genes that are deleted are involved with elastin production
overly friendly personality

Question 172

Can noonans be inherited?

Answer 172

Yes- autosomal dominant

Question 173

Clinical features of UTI

Answer 173

unexplained fever, vomiting, poor feeding, abdominal pain
in older children: LUTS- dysuria, frequency, urgency

Question 174

What are the two types of UTI and names

Answer 174

Lower- cystitis
Upper- pyelonephritis

Question 175

Mx for UTI 2

Answer 175

-under 3 months urgent referral to paediatrician and send off MSU
- over 3 months do urine dipstick and if leukocytes and nitrates positive then 3 days oral antibiotics, most likely cefalexin

Question 176

What is pyelonephritis

Answer 176

infection of kidney
where bacteria has travelled from urinary tract to kidneys via urethra

Question 177

What are the causative organisms of UTI/ pyelonephritis and what increases risk of UTI/ pyelonephritis

Answer 177

uropathogenic E.Coli MC
other causes: klebsiella, proteus, enterobacter, pseudomonas (hospital acquired)
female

Question 178

Clinical features of pyelonephritis 5

Answer 178

fever
nausea and vomiting
unilateral flank pain
urine change: foul smelling/ haematuria

Question 179

How is pyelonephritis diagnosed 2 and managed 2

Answer 179

diagnosed in children with fever 38 or higher plus MSU bacteria in urine
OR
loin pain without fever plus MSU bacteria in urine

over 3 monthg can consider referral to paeds- if being managed in community, start abx cefalexin for 7-10 days
under 3 months urgent referral to paeds

Question 180

What is noctural enuresis and the two types, explain each

Answer 180

bed wetting
primary: children that have never consistently been dry at night
secondary: children that have been dry for 6 months previously

Question 181

What is the management for nocturnal enuresis 4

Answer 181

1. conservative: go toilet before bed, reduce fluids before bed
2. 1st line- set an alarm to go to the toilet at night
3. investigate if underlying conditions eg diabetes/ diabetes insipidus (low ADH)/UTI
4. desmopressin

Question 182

What happens in nephrotic syndrome and what age is it most common
Compare this to nephritic syndrome

Answer 182

the basement membrane in the glomerulus becomes highly permeable to protein, allowing proteins to leak from the blood into the urine
2-5 years

basement membrane in nephritic syndrome= permeable to RBCs

Question 183

What is are clinical features of nephrotic syndrome 5

Answer 183

Low serum albumin (less than 30g/L)
High urine protein content (>3.5g protein in urine dipstick)= FROTHY URINE
Oedema
deranged lipid profile (high cholesterol)
hypercoagulability (increased risk of blood clots)

Question 184

What are the main causes of nephrotic syndrome in children and adults

Answer 184

minimal change disease- children
membranous nephropathy- adults

Question 185

How is nephrotic syndrome managed 5

Answer 185

1. high dose steroids for 12 weeks
2. low salt diet (potentially fluid restriction and diuretics for persistant oedema
3. albumin infusions if albumin is low
4. biopsy- minimal change has no change in light microscopy but thickened GBM in membranous microscopy , electron microscopy shows podocyte effacement in minimal change but subpodocyte immune complex deposition in membranous nephropathy
5. in steroid resistant children, ACE inhibitors can be used and immunosuppressants eg tacrolimus

Question 186

What are the complications of nephrotic syndrome 3

Answer 186

low blood pressure
thrombosis
relapse

Question 187

What is are clinical features of nephritic syndrome 4

Answer 187

haematuria
hypertension
mild proteinuria (<3.5g/day)
mild oedema

Question 188

What are the main causes of nephritic syndrome and how can you determine between the two adn what antibodies are produced

Answer 188

IgA nephropathy- onset within days of an URTI/ gastroenteritis, IgM
post strep glomerulonephritis- onset within weeks of strep infection (skin/ resp), IgG

Question 189

What do investigations show for each type of nephritic syndrome

Answer 189

IgA= biopsy + immunofluorescence microscopy shows IgA complex deposition in kidneys
post strep= starry sky in immunofluorescence microscopy

Question 190

management for nephritic syndrome 1

Answer 190

steroids shown not to be useful so manage BP with ACE inhibitors

Question 191

What is Hypospadias

Answer 191

congenital abnormality of penis where urethral opening is on the underside of the penis

Question 192

What is the management for Hypospadias and when is this done

Answer 192

routine referral once the problem is identified (usually identified in the NIPE) surgery at roughly 1 year old
no circumcision- foreskin used in the surgery

Question 193

What is Phimosis

Answer 193

foreskin on the penis that cannot be retracted

Question 194

What is a Vesicoureteric reflux

Answer 194

abnormal backflow of urine from the bladder into the ureter and kidney

Question 195

What are the possible presentations of a Vesicoureteric reflux 2

Answer 195

recurrent childhood UTI
chronic pyelonephritis

Question 196

What is the ix and mx for Vesicoureteric reflux 1, 2

Answer 196

diagnostic ix: micturating cystourethrogram
mx:
1. grade degree of reflux
2. if high grade (3+) consider continous abx prophylaxis with trimethoprim and if still getting recurrent UTIs, surgery

Question 197

How is Vesicoureteric reflux graded 5

Answer 197

1. reflux into ureter onyl
2. reflux into ureter and renal pelvis
3. reflux into ureter + renal pelvis with mild swelling
4. reflux into ureter + renal pelvis with moderate swelling
5. reflux into ureter + renal pelvis with severe swelling + tortuosity of ureter

Question 198

What is haemoytic uraemic syndrome, causes 2 and what is it usually predisposed by 1

Answer 198

thrombosis in small blood vessels throughout the body
usually triggered by Shiga toxins from either E. coli O157 or Shigella
gastroenteritis treated with antiobiotics

Question 199

What is the triad of Haemolytic Uraemic Syndrome and explain the pathophysiology of each

Answer 199

Haemolytic anaemia (damage to RBC from thrombi in blood vessels)
Acute kidney injury (thrombi affect the blood flow to kidney)
Thrombocytopenia (low platelets)

Question 200

What is the presentation of Haemolytic Uraemic Syndrome 1 and when does it present 6

Answer 200

week after diarrhoea from gastroenteritis;
Fever
Abdominal pain
Reduced urine output (oliguria)
Haematuria
Bruising
Jaundice (due to haemolysis)

Question 201

What is the management of Haemolytic Uraemic Syndrome 3

Answer 201

1. hospital emergency (it is an emergency)
2. supportive management- IV fluids, transfusions
3. stool culture for causative organism

Question 202

What are the 5 congenital kidney abnormalities and explain each:

Answer 202

Horseshoe kidney: The kidneys may be fused together, forming a single arched kidney
Polycystic or multicystic kidney disease: One or both kidneys have fluid-filled cysts
Renal agenesis: Baby is born with one kidney, or baby is born without kidneys
Renal hypoplasia: Baby is born with one or two abnormally small kidneys
Renal dysplasia: One or both kidneys have not formed as they should

Question 203

What is AKI and what is the classification for it

Answer 203

abrupt decline in kidney function (hours-days)
1. serum creatinine above 26 micromol/L within 48 hours - needs a baseline
OR 1.5 times the baseline in 7 days (increase of 50%)
2. urine output <0.5ml/kg/hour for 6 or more hours (consecutive)

Question 204

What is the staging method of acute kidney injury

Answer 204

KDIGO:
Stage 1
Increase in creatinine to 1.5-1.9 times baseline, or
Increase in creatinine by ≥26.5 µmol/L, or
Reduction in urine output to <0.5 mL/kg/hour for ≥ 6 hours
Stage 2
Increase in creatinine to 2.0 to 2.9 times baseline, or
Reduction in urine output to <0.5 mL/kg/hour for ≥12 hours
Stage 3 Increase in creatinine to ≥ 3.0 times baseline, or
Reduction in urine output to <0.3 mL/kg/hour for ≥24 hours
or The initiation of kidney replacement therapy, or,
In patients <18 years, decrease in eGFR to <35 mL/min/1.73 m2

Question 205

What does the urea: creatinine ratio mean in AKI

Answer 205

> 100:1= pre renal
<40:1= renal
40-100= post renal

Question 206

What are the causes of AKI 3 categories and examples of each

Answer 206

pre renal: hypoperfusion eg cardiovascular shock
renal: nephron damage eg acute tubular necrosis and sepsis
post renal: obstruction eg stones, BPH

Question 207

What is the presentation of a person with AKI 3

Answer 207

fluid overload signs eg oedema, oliguria
metabolic acidosis (due to high urea in blood)
arrythmias/ cardiac symptoms (due to high K+)

Question 208

What are the ECG signs of high K+ 3

Answer 208

tall tented T wave
P wave flattening
wide QRS

Question 209

What are ix for AKI 3

Answer 209

1. bloods: FBC, UE, CRP, bone profile, ABG (For ph)
2. urine dipstick (blood/ protein suggest renal cause)
3. US for any new onset AKI

Question 210

What is the management of AKI 4

Answer 210

1. treat complications of AKI:
   -hyperkalemia- calcium gluconate
   -metabolic acidosis- sodium bicarbonate
   -fluid overload- diuretics
2. haemo- dialysis as a last resort (indicated by AFUKE)
   ```
   -Acidosis (pH <7.1)
   -Fluid overload (odema)
   -Uremia (symptomatic)
   -K+ >6.4
   -ECG changes due to K+
   ~~~

Question 211

What is CKD

Answer 211

eGFR of <60mL/min/1.73m2 for 3 or more months (normal=120)

Question 212

What are the stages of CKD

Answer 212

1 90+ with renal signs (if no signs then no CKD)
2 60-89 with renal signs (if no signs then no CKD)
3a 45-59
3b 30-44
4 15-29
5 less than 15

Question 213

compare 2 investigations of AKI and CKD

Answer 213

AKI: no anaemia
CKD: anaemia of chronic kidney disease

AKD USS is normal
CKI USS shows bilateral small atrophied kidneys

Question 214

What condition is eczema and what is the pathophysiology behind it

Answer 214

chronic atopic condition
defects in the skin barrier allow for allergens and irritants to enter and cause an immune response with inflammation

Question 215

What is the typical presentation of eczema

Answer 215

presents in early childhood with dry, red, itch patches of flexor surfaces (insides of elbows and knees, on face and neck

Question 216

How is eczema managed during maintenance periods

Answer 216

1. create artificial barriers over skin to compensate for defective skin barrier- emollients and soap substitutes provide a thick and greasy layer
2. avoid activities that break down the skin barrier eg scratching and hot water baths

Question 217

What is the rule of emollient use in eczema and give examples (2 categories)

Answer 217

use emollients that are as thick as tolerated and required to maintain the eczema

thin creams: E45, cetraben cream
thick, greasy emollients: cetraben ointment, 50:50 liquid paraffin ointment

Question 218

How are eczema flares managed 3

Answer 218

1. thicker emollients
2. topical steroids
3. wet wraps (covering affected areas in a thick emollient and applying a wrap to keep moisture locked in overnight)

Question 219

What is the steroid ladder from weakest to most potent

Answer 219

Mild: Hydrocortisone 0.5%, 1% and 2.5%
Moderate: Eumovate (clobetasone butyrate 0.05%)
Potent: Betnovate (betamethasone 0.1%)
Very potent: Dermovate (clobetasol propionate 0.05%)

Question 220

Where are steroids avoided in children and why

Answer 220

areas of thin skin such as the face, around the eyes and in the genital region
steroids can cause skin thinning which can make skin more prone to flares

Question 221

What is the most common opportunistic bacterial infection for eczema patients and what is the treatment for this

Answer 221

staph aureus
oral flucloxacillin

Question 222

What is eczema herpeticum and causes 2

Answer 222

viral skin infection in patients with eczema caused by the herpes simplex virus (MC) or varicella zoster virus (VZV)

Question 223

How does eczema herpeticum present 3

Answer 223

1. widespread, painful, vesicular rash with pus
2. systemic symptoms such as fever and reduced oral intake
3. lymphadenopathy

Question 224

What is the management for eczema herpeticum 2

Answer 224

oral aciclovir and urgent referral

Question 225

What is stevens johnson syndrome and sx 3, how much of the body does it affect

Answer 225

syndrome where the immune system overreacts to a threat
sx: erythematous MP rash with target shaped lesions and nikolsky sign (rubbing skin= rubs off top layers of skin)
less than 10%

Question 226

What are the causes of SJ syndrome (2 cat: 4,4)

Answer 226

adverse drug reaction: to allopurinol, anti-epiletics, antibiotics, NSAIDS
infections: HSV, cytomegalovirus, mycoplasma pneumonia, HIV

Question 227

Management for SJ syndrome 4

Answer 227

hospital admission with steroids and immunosuppressants according to a dermatology specialist

Question 228

What are the complication of SJ syndrome 2

Answer 228

1. secondary infection eg cellulitis
2. skin scarring

Question 229

What is allergic rhinitis and presentation 3

Answer 229

nose become sensitized to allergens such as house dust mites and pollen
1. sneezing
2. clear nasal dischatge
3. bilateral nasal obstruction

Question 230

How is allergic rhinitis managed 3

Answer 230

1. avoid allergen
2. mild= antihistamines- fexofenadine
3. moderate/ severe/ ineffective antihistamines= intranasal corticosteroids eg mometasone/ fluticasone

Question 231

What are the two types of antihistamine, how they were and examples of each?

Answer 231

sedating (blocks central and peripheral H1 receptors)
e.g promethazine/ cyclizine

non-sedating (only blocks peripheral H1 receptors)
e.g fexofenadine/ cetirizine

Question 232

What is urticaria

Answer 232

itchy, pale pink raised skin (hives)

Question 233

Management for urticaria 2

Answer 233

1st line: non sedating antihistamines eg fexofenadine for 6 weeks after acute event
2. prednisolone for severe/ resistant episodes

Question 234

What is Kawasaki 1 and 2 serious complication of this

Answer 234

medium vessel vasculitis
1. coronary artery aneurysm
2. Reyes syndrome from high dose aspirin treatment

Question 235

What is the presentation of Kawasaki 6

Answer 235

CRASH + BURN

Conjunctivitis
Rash/Red cracked lips
Adenopathy (same as lymphadenopathy)
Strawberry tongue
Hands and feet redness and peeling
+ BURN= 5+ days of high fever which is resistant to antipyretics

Question 236

How is Kawasaki managed 3

Answer 236

1. high-dose aspirin
2. intravenous immunoglobulin
3. echocardiogram to screen for coronary artery aneurysms

Question 237

What is the cause of chicken pox 1

Answer 237

varicella zoster virus

Question 238

What is the presentation of chicken pox 3

Answer 238

1. widespread, erythematous, raised, vesicular (fluid filled), blistering lesions and lasts several days
2. fever
3. Eventually the lesions scab over, at which point they stop being contagious

in question: 2 days fever then clusters of vesicles on face and torso

Question 239

How does chicken pox spread 2 and how long until an exposed child develops it?

Answer 239

direct contact or resp droplets
1-3 weeks

Question 240

What is a complication of chicken pox

Answer 240

After the infection the virus can lie dormant in the sensory dorsal root ganglion cells and cranial nerves reactivate later in life as shingles or Ramsay Hunt syndrome

Question 241

how is chicken pox managed 4

Answer 241

1. conservatively at home (kids shouldn’t go to school until lesions crusted over!!)
2. calamine lotion and chlorphenamine for itching
3. aciclovir if severe
4. varicella zoster immune globulin vaccine for prophylaxis of close contacts with high risk

Question 242

What is the pathophysiology of scalded skin syndrome and what is the cause

Answer 242

cause: staph aureus
staph aureus produces epidermolytic toxins which are protease enzymes and break down proteins in skin= damaged skin

Question 243

Presentation of scalded skin syndrome 3

Answer 243

1. starts with patches of erythema on skin with thin and wrinkled skin
2. formation of fluid filled blisters (bullae) which burst
3. Nikolsky sign which gentle rubbing of the blisters causes the epidermis of skin to peel away easily

Question 244

What is the management for scalded skin syndrome 2

Answer 244

IV flucloxacillin and IV
fluids to prevent dehydration

Question 245

What are hte two conditions that have postive nikolsky sign

Answer 245

steven johnson syndrome and scalded skin syndrome

Question 246

What is measles caused by 1 and symptoms 3

Answer 246

Morbillivirus
1. rash starting behind ears/forehead then spreads to whole body, looking blotchy and so dense that they are confluent
2. koplik spots- white spots on inside of mouth that appear a few days before the rash
3. high fever

Question 247

What is the management of measles 4

Answer 247

1. supportive
2. notify public health
3. ensure close contacts all are given MMR vaccine within 72 hours
4. school exclusion under 5 days after rash onset (no longer infectious after day 4)

Question 248

What are two common complication of measles

Answer 248

otitis media and pneumonia

Question 249

What is rubella, cause and presentation 3

Answer 249

german measles, togavirus
1. rash starting on face then downwards and clears on face
2. palatal petechiae
3. lymphadenopathy

Question 250

What is diphtheria caused by and typical presentation 4

Answer 250

C. diptheriae

fever + sore throat + white membrane over tonsils + no vaccinations= diptheria

Question 251

What is the cause 1 and symptoms of mumps 3

Answer 251

kalmumps virus

1. swelling of parotid glands (one or both)
2. pain whilst swallowing/ chewing
3. systemic symptoms eg fever, loss of appetite

Question 252

What type of reaction is anaphylaxis? Explain the pathophysiology in anaphylaxis

Answer 252

severe type 1 hypersensitivity reaction

IgE) stimulates mast cells to rapidly release histamine and other pro-inflammatory chemicals. This is called mast cell degranulation. This causes a rapid onset of symptoms, with airway, breathing and/or circulation compromise (ABC compromise is what differentiates anaphylaxis from a non-anaphylactic allergic reaction)

Question 253

What is the immediate management of anaphylaxis 2

Answer 253

1. ABCDE
2. IM adrenaline (500mc child over 12, 300 for 6-12 and 150 younger than that)

Question 254

What is the presentation of anaphylaxis 5

Answer 254

sudden and rapid onset of systems as defined by resus council UK
1. airway: stridor due to swelling of throat
2. breathing: wheeze, SOB
3. circulation: tachycardia, hypotension
4. skin: itching, widespread erythematous rash

Question 255

How often can adrenaline be repeated and what is the limit to this and where can it be given

Answer 255

every 5 minutes-
anterolateral aspect of middle third of thigh
if ABC problems still after 2 doses of adrenaline, is is called refractory anaphylaxis

Question 256

What is refractory anaphylaxis and the treatment of it 2

Answer 256

ABC problems after 2 doses IM adrenaline
1. IV fluids given for shock
2. IV adrenaline infusion

Question 257

What is the non immediate management after anaphylaxis 4

Answer 257

1. non sedating antihistamine eg chlorphenamine given for itching
2. referral to specialist allergy clinic if first presentation
3. patient given two adrenaline auto-injectors and trained on how to use it
4. discharge strategy decided

Question 258

Explain discharge strategy for anaphylaxis 3 and why do we do this 1

Answer 258

1. fast track discharge- 2 hours after symptom resolution if good response to adrenaline and complete resolution of symptoms
2. minimum 6 hours after symptom resolution if 2 doses IM given or previous biphasic reaction
3. minimum 12 hours after symptom resolution if refractory anaphylaxis
   -> some people have biphasic anaphylactic reactions

Question 259

How is anaphylaxis confirmed 1 and why?

Answer 259

serum mast cell tryptase within 6 hours of the event.
Tryptase is released during mast cell degranulation and stays in the blood for 6 hours before gradually disappearing

Question 260

What is the cause of whooping cough and what type of organism is this

Answer 260

Bordetella pertussis
gram neg bacteria

Question 261

What is the presentation of whooping cough 5

Answer 261

intractable cough lasting more than 14 days without any apparent cause and one or more of the following features:
1. Paroxysmal cough.
2. Inspiratory whoop.
3. Post-tussive vomiting. (vomiting after coughing burst)
4. Undiagnosed apnoeic attacks in young infants.

Question 262

eWhat are investigations for whooping cough 1

Answer 262

1. nasal swab culture (0-2 weeks of cough) or PCR (0-4 weeks) for Bordetella pertussis

passmed: per nasal swab is best as bordatella is more likely to be picked up on swab in the nose

Question 263

What is the management for whooping cough 4 and when can they go back to school

Answer 263

1. notify public health
2. supportive care
3. azithromycin within first 3 weeks of cough
4. household contacts given prophylaxis of azithromycin
5. school exclusion until 48 hours after abx or 2 weeks after start of sx if left untreated

Question 264

What is polio caused by 1 and how does it affect the body 1 what is a complication of polio 1

Answer 264

poliovirus
affects nerve in CNS
can cause paralysis

Question 265

What causes slapped cheek syndrome and presentation 2

Answer 265

parovirus B19
fever, bright red rash on cheeks that can reappear after feeling hot/ being in sun

Question 266

What is impetigo caused by and what are the two classifications

Answer 266

staph aureus/ strep pyogenes
non bullous and bullous (if widespread bullous= stacph scalded skin syndrome)

Question 267

What is the presentation of impetigo 1

Answer 267

golden crusted skin lesions around the mouth

Question 268

What is the management for impetigo 3

Answer 268

1. hydrogen peroxide 1% cream (antiseptic cream) or fuscidic acid if it is near eyes
2. oral flucloxacillin in severe impetigo
3. exclusion from school 48hrs after starting abx, or until all of the lesions have crusted over.
4. NOT reportable

Question 269

What is Rheumatic fever?

Answer 269

fever 2-6 weeks after a step pyogenes infection (complication of scarlet fever not being treated)

Question 270

What is the pathophysiology of rheumatic fever? 3

Answer 270

1. strep pyogenes infection- cell wall of strep pyogenes has an M protein
2. body produces antibodies to M protein
3. these antibodies cross react with myosin and smooth muscles of arteries

Question 271

What is involved in the diagnostic criteria for rheumatic fever

Answer 271

Diagnosis:
-> evidence of recent strep infection and 2 major criteria
-> evidence of recent strep infection and 1 major criteria and 2 minor criteria

Major criteria: JONES
Joint involvement (polyarthritis)
O looks like a heart- myocarditis
Nodules- subcutaneous
Erythema marginatum
Sydenham Chorea

Minor criteria: FECAP
Arthralgia (pain in 1 joint)
Fever
Elevated ESR
CRP high
Arthralgia (pain in 1 joint)
Prolonged PR interval

Question 272

How is rheumatic fever managed? 5

Answer 272

1. stat dose of IM benzylpenicillin then oral phenoxymethylpenicillin for at least 10 day
2. NSAIDs first line- high dose aspirin for anti-inflammatory
3. pred if not responsive to NSAIDs/ myocarditis
   treat complications:
4. heart failure meds eg diuretics/ ACEi for carditis and heart failure
5. notify PHE

Question 273

What are the nodules found in rheumatic heart fever called and what type are these

Answer 273

Aschoff bodies
granulomatous

Question 274

Pathophysiology of toddler’s diarrhoea? 3

Answer 274

1. toddler’s with a high fibre (fruit)/ low fibre diet, sugar (snacks) and fluid diet (juice) upsets colon
2. causing more fluid to be kept in colon and not absorbed into body
3. causes frequent and runny stools

Question 275

Management of toddler’s diarrhoea? 2

Answer 275

1. 4Fs for diet changes
   -> high Fat, enough Fibre, Fluids should be mainly from water/ milk, restrict Fruit juice
2. no other management- colon gets better at dealing with stools as they grow older

Question 276

What is toxic shock syndrome cause and pathophys

Answer 276

staph aureus/ strep pyogenes infection- produces toxins that can trigger an over the top T cell led immune response

Question 277

What is the diagnostic criteria for toxic shock syndrome 6

Answer 277

1. fever over 38.9
2. hypotension, systolic under 90
3. diffuse erythematous rash
4. desquamation of rash, esp on palms and soles
5. involvement of 3+ organ systems with symptom presentation
6. nausea and vomiting

put 3 tamp high heat on the stove (3+ organ systems)
tamps start getting hot (gets a temp of 38.9 plus)
turns red because its so hot (diffuse red rash)
so hot it starts to peel (desquamation of palms/ soles)
turn the temp of the stove down to under 90 (systolic under 90)
tamps all burst (nausea/ vomiting)

Question 278

Management for toxic shock syndrome 3

Answer 278

1. IV fluids
2. IV Abx: clindamycin (works against toxins) and penicillin (works against bacteria)
3. report (strep A infections are a notifiable disease)

Question 279

What is scarlet fever caused by, its age and method of spread

Answer 279

strep pyogenes infection
toddlers 2-6 years
respiratory droplets

Question 280

What is the clinical presentation of scarlet fever 4

Answer 280

fever
sore throat
strawberry tongue
fine rash that appears on torso (not hands and soles) with a rough sandpaper texture then later desquamination of fingers and toes

can present with tonsillitis too!!

Question 281

How is scarlet fever managed 4

Answer 281

1. immediate oral penicillin 10 days-> if allergic, give azithromycin
2. throat swab (do not wait on this to start antibiotics)
3. notifiable disease
4. schoole exclusion until 24 hours after first dose of abx

Question 282

What causes hand, foot and mouth disease 2

Answer 282

coxsackie A16 or enterovirus 71

Question 283

When does hand foot and mouth disease outbreaks happen and why

Answer 283

outbreaks in nursery because it is very contagious

Question 284

Clinical features of hand foot and mouth disease 2

Answer 284

oral ulcers later followed by vesicles on the hands and soles of the feet

Question 285

What is the management of hand, foot and mouth disease 2

Answer 285

symptomatic
keep then off school until they feel better but no exclusion necessary

Question 286

What are febrile convulsions and what age do they occur

Answer 286

seizures provoked by fever
6 months to 5 years

Question 287

What are the general clinical features of febrile convulsions 3

Answer 287

less than 5 minute convulsions
typically tonic-clonic
after viral infection (this causes temp to rise rapidly)

Question 288

What are the types of febrile convulsion and explain characteristics of each 3

Answer 288

simple: no reoccurrence within 24 hours and complete recovery within an hour, generalised seizure
complex: 15-30 mins, focal seizure, can have repeat seizures within 24 hours
febrile status epilepticus: longer than 30 mins

Question 289

What is the management of febrile convulsions 2

Answer 289

1. if first seizure/ complex seizure then admission to paediatrics
2. if repeated then benzodiazepine rescue medication (rectal diazepam/ buccal midazolam)

Question 290

What are the clinical features of juvenile myoclonic epilepsy 2 and who does it affect 1

Answer 290

1. infrequent seizures, often in the morning/ after sleep deprivation
2. daytime absences
3. sudden myoclonic seizures
   -> onset in teenage girls

Question 291

What is the management of juvenile myoclonic epilepsy 2

Answer 291

sodium valproate, lamotrigine for females

Question 292

What is benign rolandic epilepsy main feature 1

Answer 292

paraesthesis (unilateral face) usually on waking uo

Question 293

What are absence seizures features and age affected, 2 features

Answer 293

4-8 years old
few-30 seconds where you lose awareness of surroundings
no warning, quick recovery

Question 294

What is the investigation and result and treatment of absence seizures 2

Answer 294

EEG: generalized spike at 3Hz
sodium valproate/ ethosuximide

Question 295

What is the prognosis of absence seizures 1

Answer 295

large majority become seizure free in adolescence

Question 296

What are infantile spasms and features

Answer 296

brief spasms in first few months of life

features: flexion of head, trunk, limbs and extension of arms (Salaam attack) which lasts for 1/2 seconds then repeats up to 50 times

Question 297

What is the mx of infantile spasms and why does this need to be managed?

Answer 297

vigabatrin
can lead to poor developmental growth if not controlled

Question 298

What are the complications of undescended testes 3

Answer 298

infertility
torsion
testicular cancer

Question 299

what is the management for undescended testes, unilateral and bilateral 3, 1

Answer 299

for unilateral:
1. referrals at 3 months
2. see urologist before 6 months of age
3. orchidopexy surgery often around 1 year

for bilateral
1. reviewed by senior paediatrician within 24 hours as child may need urgent endocrine/ genetic investigations

Question 300

What is testicular torsion and what age can it usually occur and peak incidence age

Answer 300

twist of the spermatic cord= testicular ischaemia and necrosis
10-30 (peak is 13-15)

Question 301

What are the clinical features of testicular torsion 4

Answer 301

1. sudden onset pain
2. loss of cremasteric reflex
3. elevation of testes does not ease pain (prehn’s sign)
4. tender, swollen testes that retracts upwards

Question 302

management of testicular torsion 1

Answer 302

urgent surgical exploration and detorsion within 6 hours
orchidectomy if the testicle is necrotic

Question 303

What is bell clapper testes 1 and what are people with this at increased risk of 1

Answer 303

testes that sit horizontally and not vertically in scrotum
bilateral testicular torsion

Question 304

What is the cause of tuberculosis 1, characteristic of this organism 2 and how does it spread 1

Answer 304

Mycobacterium tuberculosis
aerobic, acid-fast bacilli
respiratory droplets

Question 305

What is the pathophysiology of primary disease and latent and reactivation tuberculosis 6

Answer 305

1. respiratory droplets deposited in alveoli
2. Mycobacterium tuberculosis is engulfed by alveolar macrophages
3. Mycobacterium tuberculosis proliferates in macrophages and causes active disease if the immune response is inadequate
4. latent infection: Th1 cell response causes caseating granuloma formation which contains the Mycobacterium (caseous centre with necrotic material surrounded by lymphocytes and macrophages)- these are found in ghon focus which is the primary site on inflammation
5. miliary reaction= when TB disseminates all over body in the form ghon complexes
6. reactivation= when immunity is low, TB can reactivate

Question 306

what are the non specific clinical features of TB 3, features of pulmonary TB 1 and features of extrapulmonary TB 1

Answer 306

1. fever
2. night sweats
3. weight loss
4. long term cough +- haemoptysis
5. enlarged lymph nodes, symptoms based on organ involved eg skeletal pain

Question 307

What are investigations 3 and results for active TB

Answer 307

1. chest xray= bilateral hilar lymphadenopathy, reactivation= upper lobe cavitary lesion
2. sputum MSC- 3 samples needed= (microscopy= Ziehl Neelsen stain on sputum sample stains acid red, culture is GS to identify bacteria as it is most sensitive and specific but takes long- needs Lowenstein Jenson agar)
3. NAATbro

Question 308

Investigation for latent TB (no signs of active infection)

Answer 308

Mantoux (Tuberculin) Skin test- after 72hrs induration of >5mm is a positive resulthypoxic

Question 309

What is the management of active TB and duration

Answer 309

1. RIPE 2 months (rifampicin, isoniazid, pyrazinamide, ethambutol)
2. continuation phase 4 months (rifampicin, isoniazid)

Question 310

What is the treatment for latent TB

Answer 310

3 months isoniazid and rifampicin

Question 311

What are the side effects of RIPE treatment and any solutions where relevant

Answer 311

rifampicin: red urine, flu like symptoms
isoniazid: peripheral neuropathy (prevent with vit B6), hepatitis, agranulocytosis
pyrazinamde: hyperuricaemia causing gout, athralgia, myalgia
ethambutol: (eyes) optic neuritis (check visual acuity before and during treatment)

Question 312

What is osteogenesis imperfecta and explain type 1’s inheritance and what the abnormality is

Answer 312

brittle bone disease due to improper bone formation
autosomal dominant- abnormality in type 1 collagen due to decreased synthesis of collagen

Question 313

What are the symptoms of osteogenesis imperfecta 3

Answer 313

blue sclera
fractures following minor trauma
deafness secondary to otosclerosis

Question 314

What are the investigations for osteogenesis imperfecta 2

Answer 314

1. skeletal survery for NAI concerns (x-rays entire body)
2. bloods: calcium, phosphate, PTH, ALP usually all normal

Question 315

What is the management for osteogenssis imperfecta 3

Answer 315

1. bisphosphates to increase bone density eg risedronate
2. vit D supplements to prevent deficiency
3. physiotherapy

Question 316

What is rickets 1 and adult equivalent

Answer 316

poorly mineralised bones in children whilst growing, causing soft bones
osteomalacia

Question 317

What are the causes of rickets 3

Answer 317

calcium deficiency
vitamin D deficiency (due to lack of sunlight)
prolonged breastfeeding

Question 318

what are the features of rickets 5

Answer 318

1. acheing bones (child may be reluctant to walk)
2. in toddlers- genu varum (bow legs)
3. older children- genu valgum (knock knees)
4. kyphoscoliosis
5. widening of wrist/ ankle/ knee joints

Question 319

Investigations and results for rickets 3

Answer 319

low vitamin D levels
reduced serum calcium
raised alkaline phosphatase

Question 320

management of rickets 1

Answer 320

oral vitamin D

Question 321

What is transient synovitis and who does it affect

Answer 321

acute hip pain in 3-9 year olds after a recent viral infection

Question 322

presentation of transient synovitis

Answer 322

1. limp/ refusal to weight bear BUT is mobile (if immobile + unable to weight bear, it is likely sepsis
2. hip pain
3. low grade fever (high grade= think sepsis)

Question 323

management of transient synovitis 2

Answer 323

rest and analgesia- it is self limiting

Question 324

compare transient synovitis to perthe’s disease 4

Answer 324

transient synovitis: after infection/fever AND shorter presentation
perthes= gradual over weeks (due to reduction in blood supply) AND worse after exercise of hip

Question 325

What is the criteria for diagnosis of septic arthritis in children called 1 and its criteria 4

Answer 325

Kocher criteria
fever >38.5 degrees C
non-weight bearing
raised ESR >40
raised WCC >12k
0 points= low risk
1 point= 3% risk
2 points= 40% risk
3 points=93% risk
4 points= 99% risk of septic arthritis

Question 326

symptoms of septic arthritis in children 3 and what are the most commonly affected joints in septic arthritis in children

Answer 326

1. hot, red, swollen, painful joint
2. minimal movement of joint possible= no weight bearing
3. high fever

hip, knee and ankle

Question 327

What is Sepsis 6 and time frame

Answer 327

FABULOS
Fluids (give)
Antibiotics (give)
Blood cultures (take)
Urine output (take)
Lactate (take)
Oxygen (give)
within Sixty minutes

Question 328

investigations for septic arthritis 3

Answer 328

1. joint aspiration culture which will show high WBC
2. raised inflammatory markers
3. blood cultures

Question 329

What is osteomyeltitis

Answer 329

bone infection

Question 330

what are the two types of osteomyelitis and compare the two (cause, age group affected)

Answer 330

haematogenous:
-from bacteriamia (bacteria in blood)
-children
non-haematogenous:
-from contagious spread of infection from adjacent tissue to bone/ direct trauma to bone
-adults

Question 331

what is the most common cause of osteomyelitis and the axception

Answer 331

Staph. aureus is the most common cause except in patients with sickle-cell anaemia where Salmonella species predominate

Question 332

What is the investigation for osteomyelitis 1

Answer 332

MRI

Question 333

what is the management of osteomyelitis

Answer 333

flucloxacillin for 6 weeks
clindamycin if penicillin-allergic

Question 334

What causes Perthe’s disease and where does it affect

Answer 334

avascular necrosis of femoral head= affects hip

Question 335

what are the symptoms of Perthe’s disease 3

Answer 335

1. progressive hip pain (over weeks)
2. worse after exercise
3. limp

Question 336

What are the investigations for Perthe’s disease

Answer 336

1. x ray
2. MRI if normal x-ray and symptoms persist

Question 337

What is the main complication of perthes disease

Answer 337

osteoarthritis

Question 338

What is discoid meniscus and how is it fixed

Answer 338

-> congenital abnormal development of meniscus leading to discoid shaped meniscus
-> surgery

Question 339

What is slipped femoral epiphysis and who is at risk of this

Answer 339

displacement of femoral head postero-inferiorly, typically in obese boys

Question 340

What are the features of slipped femoral epiphysis 2

Answer 340

1. hip pain
2. loss of internal rotation of leg in flexion

Question 341

What is the investigation for slipped femoral epiphysis 1

Answer 341

AP and frog leg x ray

Question 342

what is the management for slipped femoral epiphysis 1

Answer 342

internal fixation with a screw in the centre of the epiphysis

Question 343

What is Osgood Schlatter 1and what typically causes it 2

Answer 343

inflammation of area below the knee (where patellar tendon attaches to tibiax)
sports eg football, underlying cause= tendons and bones grow at different rates)

Question 344

What is developmental dysplasia of the hip

Answer 344

Where the ball and socket hip joint do not form correctly, causing the ball to come out of the socket

Question 345

What are the risk factors of developmental dysplasia of the hip 3

Answer 345

female
breech birth
birth weight over 5kg

Question 346

Which hip is more commonly affected in developmental dysplasia of the hip

Answer 346

left (due to most common position in utero means the left hip is against mums lumbosacral spine)

Question 347

What is the screening for developmental dysplasia of the hip 2

Answer 347

1. all infants screened at newborn check and 6-8 baby check using Barlow and Ortolani test (if dislocatable/ dislocates then urgent 2 week referral)
2. 6 week routine US screening required for all babies from
   -multiple pregnancy
   -breech position at 36 weeks or after (regardless of presentation at birth or mode of delivery)
   -first degree family hx of hip problems early in life

Question 348

What are the clinical examinations of developmental dysplasia of the hip 3

Answer 348

1. Barlow (dislocated the femoral head- both legs in and down)
2. Ortolani (repositions the femoral head after it has been dislocated- opposite to Barlows movements)
3. symmetry of leg length

Question 349

How is developmental dysplasia of the hip diagnosed

Answer 349

ultrasound

Question 350

What is the management for developmental dysplasia 3

Answer 350

1. clicky hip usually spontaneously stabilise by 2 months
2. Pavlik harness in children under 5 months
3. older children require surgery

Question 351

What is the definition of juvenile idiopathic arthritis

Answer 351

arthritis in a child under 16 years, lasting for more than 6 weeks

Question 352

What is Stills disease

Answer 352

systemic onsent juvenile idiopathic arthritis (a type of juvenile idiopathic arthritis)

Question 353

What are the features of Stills disease 6

Answer 353

non specific: fever + weight loss + lymphadenopathy
specific: salmon- pink rash, arthritis, uveitis (pink rash, pink joints, pink eyes)

Question 354

What are the investigations for stills disease 2

Answer 354

1. anti nuclear antibodies positive, particularly in oligoarticular
2. RF negative

Question 355

What is the difference between oligoarticular juvenile idiopathic arthritis and polyarticular

Answer 355

oligoarticular- arthritis in four joints or less
polyarticular in five joints or more

Question 356

What is the management of juvenile idiopathic arthritis 2

Answer 356

NSAIDs
DMARD eg methotrexate

Question 357

Cause of roseola infantum? Sx? Mx? Comp?

Answer 357

-> viral illness caused by human herpesvirus 6
-> 3 day high fever then MP
-> rash on 4th day that starts on the trunk and spread to extermities and rash disappears after 24 hours
-> supportive, self limiting
-> febrile convulsions

Question 358

What is scoliosis

Answer 358

curvature of the spine in the coronal plane

Question 359

What are the subdivisions of scoliosis and explain about each one

Answer 359

structural: curvature with rotation of the spine. this affects 1+ vertebral body, cannot be corrected with posture changes.
non-structural: only curvature of the spine

Question 360

What type of scoliosis is treated and how

Answer 360

severe/ structual
surgery with bilateral rod stabilisation of the spine
non structural= lifestyle: good posture with brace, exercise

Question 361

What is torticollis

Answer 361

stiff neck , making it hard/ painful to turn head

Question 362

What is the main cause of torticollis 1

Answer 362

muscle spasms in the neck

Question 363

What is the management of torticollis 3 and why is this the management that is given

Answer 363

usually only lasts for a few days so supportive management:
1. analgesia
2. warm compress over area
3. avoid activities that strain the neck eg trampolines/ sports

Question 364

What is the procedure for newborn resuscitation 7

Answer 364

1. Dry baby and cover
2. In 30 secs: assess tone, respiratory rate, heart rate
3. If gasping or not breathing, ensure airway open and give 5 inflation breaths
4. Reassess- if not increase in heart rate, check chest movements
5. If no improvement, another 5 inflation breaths
6. If HR <60bpm and chest moving, start compressions and ventilation breaths at a rate of 3 compressions to 1 breath
7. continue reassessing HR every 30 seconds

inflation breaths replace fluid in alveoli with air and sustain pressure to open the lungs, ventilation breaths are to deliver oxygen and remove co2

Question 365

What is neonatal respiratory distress syndrome, rfx and symptoms

Answer 365

NEONATAL: babies with lack of lung surfactant leads to alveolar collapse and SOB hours after birth

rfx: prematurity before 34 weeks (before this there is not enough surfactant and before 24 weeks there is NO surfactant)

sx: showing signs of respiratory distress and cyanosis minutes- hours after birth

Question 366

what is the mx for neonatal respiratory distress? 2

Answer 366

intratracheal instillation of artificial surfactant, if still struggling to keep sats up, use CPAP

Question 367

What is Bronchopulmonary dysplasia and risk factors 2

Answer 367

chronic lung disease that affects premature babies that requires them to be on prolonged oxygenation/ ventilation support

1. having neonatal respiratory distress syndrome
2. babies whos mothers smoke

Question 368

What is the management of bronchopulmonary dysplasia 3

Answer 368

1. if required, oxygenation or ventilation which is slowly weaned off- CPAP preferred over invasive ventilation
2. steroid/ bronchodilators inhaler in acute setting
3. if born before 32 weeks then IV caffeine

Question 369

What is meconium aspiration syndrome and what are the risk factors for this 3

Answer 369

when neonate breathes in meconium and amniotic fluid into lungs at time of delivery, which leads to neonatal respiratory distress syndrome

post-term babies, maternal HTN, smoking/ substance abuse

Question 370

What is hypoxic ischaemic encephalopathy and what can it lead to

Answer 370

brain damage caused by lack of oxygen around the time of birth, leading to developmental/ neurological problems

Question 371

What are the common causes of hypoxic ischaemic encephalopathy 4

Answer 371

placental abruption
uterine rupture
prolonged labour
shoulder dystocia

Question 372

What are the investigations for hypoxic ischaemic encephalopathy 2

Answer 372

1. EEG to monitor brain function
2. MRI

Question 373

What is the treatment for hypoxic ischaemic encephalopathy 2

Answer 373

therapeutic hypothermia (special cooling mattress to keep babys temp between 33-34 degree for 72 hours then rewarming over a period of 12 hours). MRI done after this to check for damage

oxygen if needed

Question 374

What does TORCH infections mean

Answer 374

group of diseases that cause congenital conditions if fetus exposed to them in utero up to after birth:
toxoplasma gondii, other (HIV, parovirus, varicella zoster), rubella, cytomegalovirus, herpes simplex virus

Question 375

How are TORCH infections transmitted 3

Answer 375

1. via placenta
2. via birth canal
3. via breast milk

Question 376

What are the symptoms of TORCH infections 4

Answer 376

non specific symptoms at or after birth:
-learning disabilities
-failure to thrive
-fetal intrauterine growth retardation/ limb hypoplasia
-congenital deafeness

Question 377

How are TORCH infections diagnosed antenatally and postnatally?

Answer 377

after baby is born:
PCR
viral cultures
antibody testing

in utero:
-> amniotic can be tested via PCR for T and O and C and H
-> R is tested for via IgM testing
-> IgM and IgG together suggest a current infection with TORCH

Question 378

What is cleft lip and palate

Answer 378

congenital condition that results in upper lip or palate failing to join in the womb

Question 379

causes of cleft lip/ palate

Answer 379

1. maternal use of antiepileptics during pregnancy
2. pataus
3. folic acid deficiency during pregnancy

Question 380

how can babies with cleft lip/ palate be managed

Answer 380

orthodontic devices to help with feeding
definitive surgery (lip at 3 months, palate at 6 months)

Question 381

what two developmental conditions that can be a result of using epileptic/ teratogenic medication during pregnancy

Answer 381

cleft lip and palate
spina bifida

Question 382

What is transposition of the great arteries and symptoms

Answer 382

aorta and pulmonary artery are switched (R ventricule pumps deoxygenated blood into aorta)

sx: cyanosis in first 24 hours of life but can be asymptomatic because PDA and VSD can compensate- in this case then it will present as respiratory distress, poor feeding and poor weight gain

Question 383

What is the ix 2 and mx 2 for transposition of the great arteries

Answer 383

ix:
echo
cxr: eggs on a string appearance

mx:
prostaglandin infusion- alprostadil to maintain PDA and mixing of blood
surgical correction is definitive

Question 384

What happens if PDA is not closed after birth, how do you close it and what is the exception?

Answer 384

causes pulmonary oedema
to close it give indithomecin or ibuprofen
exception: alongside other congenital heart defects in which case keep it open until surgery completed- give prostaglandin E1 to do this ie alprostadil

Question 385

What part of the brain does HSV encephalitis

Answer 385

temporal and inferior frontal lobes

Question 386

What are the features of HSV encephalitis 6

Answer 386

fever, headache, vomiting, seizures, changes in personality/ mood
focal features: wernickes aphasia

Question 387

What are the Ix for HSV encephalitis 4

Answer 387

PCR for HSV
CT/ MRI: medial temporal and inferior frontal changes (petechial haemorrhages )
CSF: high lymphocytes, high protein
EEG: lateralised periodic discharges at 2 Hz

Question 388

What is treatment for HSV encephalitis

Answer 388

intravenous aciclovir

Question 389

Where is listeria monocytogenes bacteria found?

Answer 389

poorly processed meat and unpasteurised milk

Question 390

when is neonatal hypoglycaemia most common and what are the risk factors 3

Answer 390

transition between
first few hours after birth

maternal diabetes mellitus
preterm + mAcrosomia
neonatal sepsis

Question 391

What is the management of neonatal hypoglycaemia

Answer 391

asymptomatic 2-2.5 glucose= additional frequent feeds (3 hourly), observe and support breastfeeding

asymptomatic 1-1.9 glucose= inform paediatrician, buccal glucose gel and if needs more than 2 doses in 24 hours admit to specialist care baby unit

if under 1 glucose/ symptomatic admit to neonatal unit and give IV glucose, check if sepsis and continue breastfeeding (if IV access difficult, give glucose gel whilst trying to secure access)

always recheck after 30 minutes after an intervention occurs

Question 392

What is gastroschisis and its management

Answer 392

congenital defect= hole in baby’s anterior abdominal wall just lateral to the umbilical cord (causes baby’s bowel to develop outside of baby’s body in womb)

cover bowels with cling film to protect it and newborn to theatre asap after delivery (within 4 hours)

Question 393

What is oesophageal atresia and what are the symptoms

Answer 393

congenital condition where upper and lower part of oesophagus do not connect, instead upper oesophagus forms a pouch

symptoms: choking and cyanotic spells after aspiration

Question 394

What is necrotising enterocolitis and what are the two main risk factors of this

Answer 394

bacterial invasion of colon eg E.coli
prematurity and empirical antibiotic use for over 5 days

Question 395

What are the features of necrotising enterocolitis 2

Answer 395

abdominal distension and passage of bloody stools in neonates (2-3rd week of life)

Question 396

What are investigations and results for necrotising enterocolitis

Answer 396

X-Rays may show pneumatosis intestinalis and evidence of free air

Question 397

What is the management of necrotising enterocolitis

Answer 397

Treatment is with total gut rest and TPN, babies with perforations will require laparotomy

Question 398

What is the pathophysiology of bowel atresia and symptoms 2

Answer 398

gap or narrowing in the bowel that occurs during development of foetus= doesn’t develop properly and causes obstruction= bile vomit and no defecation

Question 399

Where does bowel atresia most commonly occured 1 and what is the investigation for this

Answer 399

small intestine
supine abdominal x-ray (will see dilated loops)

Question 400

What is the management of bowel atresia 5

Answer 400

stop oral milk intake
tube into stomach to drain fluid and air
IV fluids
surgery to cut out affected bowels or make a temporary stoma which will be operated on again to close to redirect bowels to rectum

STIS

Question 401

What is the reasoning for jaundice in the first 24 hours after birth and explain the 4 main causes and appropriate investigations for this

Answer 401

pathological (after 24 hours= physiological)

1. rhesus haemolytic disease of the newborn (mums blood gets into babies circulation and her antibodies start attacking the babys RBCs)
2. ABO incompatability disease (mums blood gets into babies circulation and her antibodies start attacking the babys RBCs)
3. hereditary spherocytosis (sphere shaped RBC)
4. Glucose 6 Phosphate dehydrogenase deficiency (RBC too fragile due to deficiency)

ix: direct antiglobulin test Coombs test (for haemolytic disease), FBC and blood film

Question 402

What causes jaundice in the neonate from 24 hours to 14 days and whats the pathophysiology behind this

Answer 402

breastfed babies- breast milk jaundice

combination of factors: liver not fully developed, prematurity, increased RBC turnover, insufficient calorie intake

Question 403

What is the timeline for prolonged jaundice 1 and what are the causes of this 6

Answer 403

over 14 days

biliary atresia
hypothyroidism
hepatitis
infections: sepsis OR urinary tract infection
breast milk jaundice (cant continue for 3 months but rule out other underlying causes)

Question 404

What are the investigations for prolonged neonatal jaundice 4

Answer 404

1. LFTS: serum bilirubin for conjugated and unconjugated bilirubin (raised conjugated BR indicates biliary atresia)
2. urine dipstick + MSU
3. TFTs
4. FBC

Question 405

What are signs of neonatal jaundice 4

Answer 405

1. yellowness of sclera
2. blanching test for children with darker skin tones
3. signs of bilirubin encephalopathy: poor feeding, arching of body
4. pale stools, dark urine

Question 406

What is kernicterus and what value is classed as this and what is the managment for this?

Answer 406

where bilirubin crosses BBB and can cause brain damage eg CP/ learning disabilities
unconjugated blood >25mg/dL in blood

mx: exchange transfusion

Question 407

When should we treat neonatal jaundice and how

Answer 407

after doing bloods to check bilirubin levels and plotting it on a bilirubin graph. If it is higher than the lower blue line, requires phototherapy, if higher than upper red line then exchange transfusion is needed

Question 408

What is a hepatoblastoma

Answer 408

cancer of liver in children

Question 409

What is Wilms tumour, sx, ix and mx

Answer 409

cancer of kidneys in children (nephroblastoma)
sx: abdo mass+ distention, HTN, haematuria
ix: VERY urgent appt within 48 hours if unexplained abdominal mass, CT to confirm
mx: chemo then surgery

Question 410

What is neuroblastoma

Answer 410

cancer from immature nerve cells (neuroblasts) typically starting in adrenal medulla

Question 411

Features of neuroblastoma 4

Answer 411

bone pain
paraplegia (unable to move legs)
racoon eyes
horners syndrome (anhydrosis, ptosis, miosis- constricted pupils)

N for not able to move legs
T for triad of horners

NeuRoBlasToma

Question 412

investigation for neuroblastoma 3

Answer 412

1. raised urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels
2. MIBG scan
3. biopsy

Question 413

What is retinoblastoma and its pathophysiology (geneology) 2

Answer 413

ocular malignancy

1. autosomal dom
2. due to loss of function of retinoblastoma tumour suppressor gene on chromosome 13

Question 414

What are the features of retinoblastoma 3

Answer 414

1. absence of red reflex, replaced by leukocoria
2. strabismus

Question 415

What is the management for retinoblastoma 3

Answer 415

-> 2 week wait for ophthalmological assessment when absent red reflex

1. laser photocoagulation
2. chemotherapy

Question 416

What is pica and what does it indicate

Answer 416

compulsive eating of inedible food eg dirt, paper, chalk
iron/zinc deficiency

Question 417

What is gonadatrophin deficiency and what are the symptoms and the three causes

Answer 417

little or no sex hormone production (estrogen/ progesterone/ testosterone)

lack of pubic hair, lack of growth spurt, no deepening of males voices, less breast develoment in females

1. issue with hypothalamus
2. issue with pituitary gland
3. issue with testes/ ovaries

Question 418

explain the hypothalamic pituitary gonadal axis

Answer 418

hypothalamus produces gonadatrophin releasing hormone which acts on anterior pituitary gland

the anterior pituitary gland releases FSH and LH

FSH acts on nurse cells: granulosa cells in females and sertoli cells in males

LH acts on secretory cells: leydig cells in males and theca cells in females

these bindings result in oestrogen and testosterone (gonad) production

gonads have a negative feedback effect on the anterior pituitary and hypothalamus

Question 419

What is Kallman syndrome, its pathophysiology and inheritence

Answer 419

a cause of delayed puberty secondary to hypogonadotropic hypogonadism

failure of GnRH-secreting neurons to migrate to the hypothalamus = decreased GnRH production= hypogonadotropic hypogonadism

x linked recessive

Question 420

What are the features of kallmans syndrome 4

Answer 420

anosmia
hypogonadism
delayed puberty
testes fail to descend

typcially expressed by males (due to nature of inheritence being x-linked recessive)

THAD

Question 421

What is the management for kallmans syndrome 2

Answer 421

1. testosterone/ oestrogen supplements
2. LH/FSH for if fertility is desired later in life as it can result in egg/ sperm production

Question 422

What is a squint and types 2

Answer 422

squint= misalignment of visual axes
type 1= concomitant: due to imbalance in extraocular muscles, MC is convergent than divergent
type 2= paralytic (rare): Due to paralysis of extraocular muscles

Question 423

What are the ix for squint? 2

Answer 423

1. corneal light reflection test: hold light 30cm from face to see if the light reflects symmetrically on pupils (identify if there is squint)
2. cover test: focus on an object and cover an eye, observe movement of uncovered eye, repeat for other eye
   (identify type of squint)

Question 424

What is the mx for squint? 2

Answer 424

1. routine referral to opthamology (urgent if red flags: diplopia, headaches, nystagmus, limited abduction)
2. eye patches to prevent amblyopia

Question 425

What is periorbital cellulitis and risk of progression

Answer 425

infection anterior to orbital septum from a superficial tissue injury eg chalazion/ insect bite

can progress to orbital cellulitis= medical emmergency (affects fat and munscles posterior to orbital septum)

Question 426

What are the features of periorbital cellulitis?

Answer 426

1. redness/ swelling of eye + eyelid
2. occular pain
3. exopthalmus

Question 427

What are the ix and mx for periorbital cellulitis?

Answer 427

1. bloods: FBC, WBC, inflamm markers
2. CT with contrast to image orbital tissues
3. blood cultures and micro swab to determine causative organism eg step/ staph aureus, haemophillus B

-> emergency admission and IV abx

Question 428

What is the most common Arrhythmias in paediatrics 1

Answer 428

supraventicular tachycardias (SVT) including Wolff-Parkinson-White (WPW) syndrome

Question 429

what is infective endocarditis an infection of

Answer 429

infection of endocardium (inner lining of the heart) and valves

Question 430

what are the two causes of IE

Answer 430

1. causative bacteria
2. vegetations (infective thrombotic masses- masses that are a mix of the causative bacteria, platelets/ fibrin and antibodies)

Question 431

what valve is affected in IE and what is the exception

Answer 431

mitral valve typically
tricuspid in IV drug users

Question 432

risk factors for IE (6)

Answer 432

poor dental hygeine
prosthetic valve
intravenous drug use
rheumatic heart disease
male
elderly
2 lifestyle, 2 to do with heart, 2 non-modifiable risk factors

Question 433

3 bacterial causes of IE, where from and which are the most common

Answer 433

1. most common= staphylococcus aureus (from intravenous drug use)
2. strep viridian’s due to mouth surgery/ dentists (common in developing countries)
3. staph epidermis due to prosthetic valve surgery

Question 434

pathophysiology of IE vegetation formation

Answer 434

damaged endocardium has increased platelet deposition and antibodies and bacteria adheres to this, causing vegetation to be formed

Question 435

clinical signs of IE 4

Answer 435

splinter haemorrhages (nails)
Janesway lesions (non painful on palm of hands)
Osler nodes (painful on underside of fingers/ toes)
Roth spots (eyes- retinal haemorrhages with pale centres)

Question 436

symptoms of IE (4)

Answer 436

fever
confusion
night sweats
finger clubbing

iNFeC (symptoms for each constanent- no vowels)

Question 437

what are the specific clinical features of IE caused by

Answer 437

vasculitis (inflammation of small blood vessels)

Question 438

when should you suspect IE

Answer 438

if someone comes in with a fever and a new murmur, suspect IE

Question 439

how is IE diagnosed (criteria)

Answer 439

duke’s criteria= both of these:
1. 2 separate blood cultures taken 12 hours apart are positive with typical pathogens that cause IE
2. new valvular regurgitation OR echocardiogram positive for IE (shows vegetations)

Question 440

investigations for IE (3 plus diagnostic)

Answer 440

1. blood cultures (3 separate from different sites over 24 hours, before antibiotics)
2. TOE for diagnosis
3. FBC- high CRP, ESR and neutrophillia
4. ECG if long PR interval= aortic root abscess

Question 441

what is the advantage of TOE over TTE and what they stand for

Answer 441

trans-oesophageal echocardiogram
Transthoracic echocardiogram
TOE is more sensitive and diagnostic

Question 442

treatment for IE 3

Answer 442

prolonged course of antibiotics (6 weeks)
2 weeks IV then switch to oral
if valve is incompetent, replace valve with a different prosthetic

Question 443

what antibiotics should be prescribed for IE (Staph, non staph, MRSA, prosthetic valve, unknown)

Answer 443

staph= flucloxacillin and rifampicin (replace flucloxacillin with vancomycin if MRSA)
strep= benzylpenicillin and gentamicin
MRSA= vancomycin and rifampicin
gentamicin for prosthetic valve
1st line when the organism is unknown= ampicillin and gentamicin

Question 444

differentials for IE 5

Answer 444

SLE
antiphsopholipid syndrome
rheumatic heart disease
PE
sepsis + secondary cardiac involvement

Question 445

What are the paediatric developmental milestones and explain them in depth

Answer 445

Gross motor:
6 weeks: can hold head prone
6 months: sits unsupported
9 months: crawling
12 months: unsteady walk
15 months: steady walk

Fine motor and vision:
6 weeks: fixes and follows objects
6 months: palmar grasp
9 months: object permanence (baby understanding object still exists even if its not visible eg toy being hidden/ parent when leaving the room)
12 months: mature princer grip

Hearing, speech and language:
6 weeks: startle to loud sounds
6 months: coos alone
9 months: mama, dada
12 months: few non mama dada 2 syllable words

Social and behavioral:
6 weeks: social smile
6 months: shakes rattle
9 months: stranger danger
12 months: waves bye

Question 446

What are some causes of feailure to reach each of the developmental milestone categories

Answer 446

autism= failure to reach language milestones
cerebral palsy= issues with fine motor, hand preference shown before 1 year, normally is at 2 years
gross motor: downs, fetal alcohol, cerebral palsy

Question 447

What is the DM 5 criteria for diagnosing anorexia nervosa 3

Answer 447

1. restriction of energy intake leading to low BMI in context of their age, sex and developmental trajectory
2. intense fear of gaining weight
3. disturbance of self perception of body weight/ shape

Question 448

What is the mx for anorexia nervosa

Answer 448

in adults:
CBT ED
in younger:
1st line anorexia focused family therapy
2nd line CBT

Question 449

What is Bulimia nervosa

Answer 449

episodes of binge eating followed by intentional vomiting or other purgative behaviours eg laxatives or intense exercise

Question 450

What is the DSM 5 criteria for diagnosing bulimia nervosa

Answer 450

1. recurrent episodes of binge eating
2. lack of control over eating during the binge episodes
3. recurrent inappropriate compensatory behaviour to prevent weight gain
4. episodes occur on average once a week for 3 months

Question 451

What are signs of bulimia nervosa 2

Answer 451

-> erosion of teeth (due to recurrent vomiting)
-> Russell’s sign (calluses on knuckles/ back of hand due to repeated self-induced vomiting

Question 452

What is the mx for bulimia nervosa 5

Answer 452

1. referral to specialist
2. bulimia-nervosa-focused guided self-help (adults)
3. if above doesn’t work after 4 weeks then CBT ED
4. high dose fluoxetine
5. children: 1st line: bulimia-nervosa-focused family therapy then CBT

Question 453

what are the two categorisations of hypothyroidism and how do they each cause hypothyroidism

Answer 453

primary- issue with thryoid gland, lack of T3/4 (one i in thyroid= 1)
secondary- issue with pituitary, lack of TSH (two i in pituitary= 2)

Question 454

what are the causes of primary hypothyroidism (4) what is the cause of secondary hypothyroidism (1)

Answer 454

Hashimotos thyroiditis, De Quervain’s thyroiditis, dietary iodine deficiency, carbimazole

pituitary adenoma

Question 455

what three diseases are associated with hypothyrodiism

Answer 455

downs and turners syndrome and coeliac disease (DOWN the stairs, TURN the corner and you’ll SEE it)

Question 456

6 symptoms of hypothyroidism

Answer 456

weight gain, lethargy, cold intolernace, loss of lateral aspect of eyebrow, constipation, fluid retention

Question 457

what are the investigations for hypothyroidism (3)

Answer 457

1st line: thyroid function test
2. antithyroid peroxidase antibody levels for autoimmune causes
3. fasting blood glucose in patients with non-specific fatigue and weight gain due to association with T1DM

Question 458

treatment for hypothyroidism and how does this work

Answer 458

levothyroxine
synthetic T4

Question 459

what are the thyroid function test results for primary and secondary hypothyroidism

Answer 459

primary: low T3, high TSH
secondary: low T3, low TSH

Question 460

what is hashimotos thyroiditis, what does it lead to and what type of hypothyroidism does it cause

Answer 460

thyroid gland is attacked by immune system via antithyroid antibodies and leads to loss of function
primary hypothyroidism

Question 461

what two diseases can hashimotos be associated with

Answer 461

T1DM
addisons

Question 462

what is the presentation of hashimotos like 2 and what does this progress to

Answer 462

same as hypothyroidism plus goitre of thyroid gland which progresses to atrophy

Question 463

what is the gold standard investigation for hashimotos

Answer 463

anti TPO antibodies

Question 464

What is congenital adrenal hyperplasia? Pathophys?

Answer 464

a group of autosomal recessive disorders that impair adrenal steroid production

faulty gene= adrenal glands unable to secrete corticosteroids= brain detects this in the blood which leads to overproduction of ACTH from anterior pituitary

this causes the hyperplasia and stimulates increased production of adrenal androgens, but because of the faulty gene, corticosteroids cannot be produced. However, other androgens are made because of this stimulation, including sex hormones.

Question 465

What are the main causes of congenital adrenal hyperplasia 2

Answer 465

1. MC 21-hydroxylase deficiency
2. 11-beta hydroxylase deficiency

Question 466

What are the features of congenital adrenal hyperplasia 4

Answer 466

1. virilization (female infants present with ambiguous genitalia due to excessive androgen exposure in utero, males are normal at birth= delays diagnosis)
2. early puberty
3. salt wasting crisis (dehydration, hypotension and electrolyte imbalances)
4. infertility (due to hormonal imbalances)

Question 467

How is congenital adrenal hyperplasia diagnosed?

Answer 467

ACTH stimulation testing
postiive test result= increase in levels of serum concentration of 17-hydroxyprogesterone

Question 468

What is the mx for congenital adrenal hyperplasia 2

Answer 468

1. hydrocortisone to replace glucocorticoid to reduce ACTH levels and minimize adrenal androgen production
2. fludrocortisone if there is mineralcorticoid deficiency (aldosterone)

glucocorticoid and mineralcorticoid are both corticosteroids but one has more of an immunity and metabolic role (gluco) and mineral has a role with BP and fluid volume

Question 469

What are atrial septal defects and ventricular septal defects?

Answer 469

a hole between the left and right atria causing blood to shunt from left to right

a hole between the left and right ventricles causing blood to shunt from left to right

Question 470

What are the sx of atrial and ventricular septal defects?

Answer 470

SOB
poor weight gain
difficulty feeding
lower resp tract infections
can be asymptomatic all throughout childhood

Question 471

What are the Ix of atrial septal defects and results? 3

Answer 471

-> stethoscope exam
ejection systolic murmur
fixed splitting of S2
-> ECG: RBBB with RAD (right axis deviation)
-> echo= diagnostic

Question 472

What is the mx for atrial + ventricular septal defect? 5

Answer 472

1. referral to paediatric cardiologist
2. if small + asymptomatic, watch and wait to see if they spontaneously close
3. surgical closure with transvenous catheter closure/ open heart surgery
4. anticoagulants eg aspirin/ DOACs for atrial and prophylactic abx for IE for VSD
5. furosemide (anti-congestive HF meds) for teens/ adults

Question 473

What are the risks of atrial septal defects? 2

Answer 473

risks:
-> stroke (DVT embolus travels via SVC to right atrium then to left atrium and up to brain)
-> pulmonary HTN/ R sided HF due to right side overload/ eisenmenger syndrome

Question 474

What are the associated conditions with ventricular septal defects 2

Answer 474

downs/ turners syndrome (chromosomal abnormalities)
congenital infections

Question 475

What are the ix of ventricular septal defects and results? 2

Answer 475

-> stethoscope exam
pan systolic murmur
-> echo= diagnostic

Question 476

What are the risks of ventricular septal defects? 2

Answer 476

-> pulmonary HTN/ R sided HF due to right side overload/ eisenmenger syndrome
-> infective endocarditis

Question 477

What are the causes of pan systolic murmur? 3

Answer 477

mitral + tricuspid regurgitation and ventricular septal defect

Question 478

what is anaemia and what is its value in men and women

Answer 478

low concentration of haemoglobin
Hb <130 in men, <120 in women

Question 479

what is microcytic anaemia 2

Answer 479

MCV of less than 80
small hypochromic (lighter coloured due to reduced haemoglobin) RBCs

Question 480

what r the causes of microcytic anaemia (4)

Answer 480

iron deficiency, anaemia of chronic disease, thalassemia, sideroblastic anaemia
(TICS: Thalassaemia, Iron deficiency, anaemia of Chronic disease, Sideroblastic anaemia)

Question 481

What are the ix and results for iron deficiency anaemia?

Answer 481

FBC: low ferratin, low MCV
blood film:
1. target cells (non specific bullseye pattern)
2. howell jolly bodies (nucleated RBCs)
small, hypochromic cells

Question 482

what are the iron studies results for iron deficiency anaemia (5)

Answer 482

low ferritin (unless active inflammation)
low serum iron
low transferrin saturation
high transferrin
high TIBC

Question 483

What does new onset microcytic anaemia in elderly pts indicate?

Answer 483

potential underlying malignancy

Question 484

what is thalassaemia 1, its inheritence 1 and what does this cause 1

Answer 484

inherited autosomal recessive alpha or beta globin mutations
which causes haemoglobin chain abnormalities

Question 485

iron studies results for thalassemia (anaemia) (5)

Answer 485

normal/ raised ferritin
normal/ raised serum iron
normal/ low transferrin
normal/ raised transferring saturation
normal/ low TIBC

transferrin and TIBC is normal/ low
everything else is normal/ raised

Question 486

investigations for thalassaemia 3 with results

Answer 486

blood film: generally microcytic, hypochromic cells
Hb electrophoresis: high HbA2 and HbF and low HbA
X ray- increase bone marrow activity looks like a hair on end appearance (increase in activity due to poor erythropoiesis)

Question 487

treatment for thalassemia causing anaemia (3)

Answer 487

1. repeated transfusions (but this can lead to iron overload which has the complication of organ failure so..)
2. iron chelation therapy eg desferrioxamine
3. splenectomy
4. definitive treatment: bone marrow stem cell transplant (but this is not used often due to high risks)

Question 488

compare blood transfusion and splenectomy for treating thalassemia (1, 2)

Answer 488

blood transfusions (can have complications- risk of side effects from too much iron)
splenectomy (reduces mass RBCs destruction and reduces transfusion complications)

Question 489

complication of thalassemia (1) and why 1

Answer 489

organ failure (heart and liver)

not enough oxygen carried to these organs

Question 490

what is alpha and what is beta thalassaemia

Answer 490

alpha= genetic disorder with a deficiency in alpha globin chains of Hb
beta= genetic disorder with a partial/ complete deficiency in beta globin chains of Hb

Question 491

what r the types of alpha thalassaemia based on and what chromosome

Answer 491

based on deletions of the 4 alleles on chromosome 16 responsible for alpha globin

Question 492

symptoms for alpha thalassaemia 3

Answer 492

usually asymptomatic
jaundice
fatigue

Question 493

what r the 4 types of alpha thalassaemia

Answer 493

silent carrier
mild anaemia
marked anaemia
HbH Barts

Question 494

what is the silent carrier type 1 alpha thalassaemia’s gene mutation and what symptoms r there

Answer 494

one gene deletion
asymptomatic

Question 495

what is the mild anaemia type 2 alpha thalassaemia’s gene mutation

Answer 495

2 gene deletions

Question 496

what is the marked anaemia type 3 alpha thalassaemia’s gene mutation and how is damage caused in this type (1)

Answer 496

3 gene deletions
beta chains form tetramers known as HbH which cause damage due to their high affinity to oxygen and by causing haemolysis

Question 497

what is the HbH Barts type 4 alpha thalassaemia’s gene mutation and what damage caused in this type (3)

Answer 497

4 gene deletions (gamma chains form tetramers called Hb Barts which causes sever hypoxia and high carbon monoxide levels, leading to heart failure and hepatosplenomegaly and oedema)

Question 498

what is hydrops fetalis and what causes this

Answer 498

heart failure in utero due to HbH Barts alpha thalassaemia mutation

Question 499

what mutation determine the types of beta thalassaemia 3

Answer 499

based on point mutations at 2 gene points on chromosome 11

Question 500

what r the types of beta thalassaemia 3 and explain their gene mutations (reduced/ absent… what?)

Answer 500

thalassaemia minor= 1 normal alleles and reduces/ absent beta chain allele
thalassaemia intermedia= 2 reduces beta chain alleles
thalassaemia major= 2 absent beta chain alleles

Question 501

what is the pathophysiology of beta thalassaemia 3
what can it potentially lead to 1

Answer 501

beta deficiency causes alpha chain accumulation in RBCs which forms inclusions which damages RBCs, leading to haemolysis and potentially hypoxia

Question 502

signs of beta thalassaemia 4 and when do they develop

Answer 502

chipmunk facies (enlarged forehead and cheekbones due to ineffective erythropoiesis causing bone changes )
failure to thrive
hepatospenomegaly
gall stones (presenting feature may have right upper quadrant pain)

develop within 5 months of life

Question 503

What is fanconi anaemia and pathophys and inheritence

Answer 503

autosomal recessive condition which causes impaired response to DNA damage

Question 504

What are the features of fanconi anaemia 4

Answer 504

-> cafe au lait spots
-> short stature
-> abnormal thumbs/ absent
-> aplastic anaemia

Fingers-> thumb abnormality
Aplastic aneamia
(N) Migit-> short stature
Cafe au Lait spots
o
n
i

Question 505

What is aplastic anaemia? Unique features? 2

Answer 505

pancytopenia + hypoplastic bone marrow (eventually leads to bone marrow failure) so the body does not produce enough new RBCs
-> form of non haemolytic normocytic anaemia

-> acute lymphoblastic/ myeloid leukaemia presenting feature
-> paroxysmal nocturnal haemoglobinuri

Question 506

What type of anaemia is sickle cell?

Answer 506

haemolytic normocytic

Question 507

Compare haemolytic and non haemolytic normocytic anaemia

Answer 507

HAEMOLYTIC:
-high reticulocyte to make up for RBC death which shows the BM has a good response-

NON HAEMOLYTIC:
-reduced reticulocyte count due to failing BM-

Question 508

what is the inheritance type of sickle cell disease and what is the expression in hetero/homozygotes and what is the genotype for homozygous

Answer 508

autosomal recessive (heterozytes get the traits, homocytes get the disease)
genotype for homozygous= HbS HbS

Question 509

what is the gene mutation for sickle cell disease, what chromosome and what is the effect of this mutation (3)

Answer 509

single nucleotide mutation on gene on Cr11: glutamic acid substitution with valine which causes B-globin polymerisation

Question 510

what r the 4 consequences of sickle shaped cells

Answer 510

reduced oxygen carrying capacity
cause endothelial damage
RBC sequestration (build up of RBC in spleen)
reduced lifespan

Question 511

exacerbators of sickle cell (4)

Answer 511

hypoxia
cold weather
parvovirus B19
physical exertion

Question 512

acute presentation of sickle cell (6- broken up into 3 organ systems)

Answer 512

GI: sequestration crisis
RESP: dyspnoea, cough, hypoxia
MSK: bone pain, joint pain

Question 513

what is sequestration crisis

Answer 513

blood outflow from the spleen is blocked which causes accumulation of blood in the spleen= splenomegaly

Question 514

complications of chronic sickle cell disease (5)

Answer 514

avascular necrosis of joints
silent CNS infarcts
retinopathy
nephropathy
osteomyelitis
CROANS (c= chronic complications)

Question 515

4 investigations for sickle cell disease and results

Answer 515

FBC: low MCV, low haemoglobin levels
blood film: shows sickled erythrocytes and howell jolly bodies
sickle solubility test: HbS present
haemoglobin electrophoresis: band of HbS

Question 516

what is a limitation of sickle solubility test

Answer 516

only detects HbS presence- doesn’t differentiate between heterozygous and homozygous

Question 517

acute management for sickle cell (4)

Answer 517

morphine, oxygen, IV fluids, transfusion exchange
MOIT

Question 518

chronic management for sickle cell (2)

Answer 518

hydroxycarbamide and folic acid supplements in combination with each other

hydroxy- car - baa mide
hydroxy in a car with a sheep that goes baa whos name is mide

Question 519

what does hydroxycarbamide do (3)

Answer 519

inhibits ribonucleotide reductase, decreases DNA synthesis and raised HbF (foetal) levels in the sickle cell

Question 520

what is the last resort treatment for sickle cell disease 1

Answer 520

bone marrow stem cell transplant

Question 521

what r the bleeding dysfunctions 4

Answer 521

1. over anticoagulation eg overdose heparin
2. DIC
3. haemophillia A and B
4. von willebrands disease

Question 522

what is purpuric rash and petechiae

Answer 522

purpuric rash: rash of purple spots on skin due to small bleeding from small BVs under the skin

petechiae: red spots on skin due to small bleeding from small BVs under the skin

Question 523

what is ITP 1 and why is this an issue with primary haemostasis 1

Answer 523

1. autoimmune IgG destruction of GP2b/3a platelets
2. platelets cant be activated= issue with primary haemostasis

Question 524

What is the presentation of ITP 3

Answer 524

petechiae
purpuric rash
bleeding
in children, having a fever/ viral illness previous to this but currently well

Question 525

What is primary and secondary haemostasis

Answer 525

1: platelet activation= initiation and formation of platelet plug
2: intrinsic and extrinsic coagulation cascade activation= formation of a fibrin clot

Question 526

2 types of people/ presentations that can have ITP

Answer 526

-> children post viral infection
-> adult females with autoimmune condition eg HIV

Question 527

investigations (3) and results for ITP (3,2,1)

Answer 527

FBC: raised WCC, low Hb, low platelets (mainly isolated thrombocytopenia)
bloods: normal PT and APTT
blood smear: normal RBC appearance
normal blood investigations vs DIC which are deranged

Question 528

Mx of ITP 2

Answer 528

1. mostly reassure
2. if severe, then oral prednisolone and IV IgG if active bleeding (as it works faster to increase platelet count)

Question 529

What is the presentation of DIC and blood test results

Answer 529

bleeding from lots of different areas eg eyes, mouth, ears ets

High PT and APTT
low platelets

Question 530

what r the two types of haemophilia, which is more common and what part of the coagulation do they affect, how r they inherited

Answer 530

A and B
A (b is rare)
intrinsic pathway
x-linked recessive

Question 531

what is haemophilia A and what type of disorder is it and how is it inherited

what is haemophilia B and what type of disorder is it

Answer 531

factor 8 deficiency
secondary haemostasis disorder (exposure of the factor in the bloodstream initiates fibrin to stabilise clot)
factor 9 deficiency
secondary haemostasis disorder

Question 532

presentation of haemophilia (4)

Answer 532

spontaneous bleeds
easy bruising
epistaxis (nose bleeds)
haemarthrosis (bleeding into joint spaces)

SEEH he’s bleeding!

Question 533

investigation and results for haemophilia (1-> 2, 1-> 1)

Answer 533

APTT is abnormal but PT is normal

A= IV F 8 plus desmopressin (releases F8 stored in vessel walls)
B= IV F9

Question 534

what type of disorder is von willibrands disease and what is the inheritance and location of the mutation

Answer 534

a primary haemostasis disorder
auto dom mutation of VWF gene on chromosome 12= less functional VWF in blood

Question 535

what is a consequence of VW disease and explain how this comes about

Answer 535

vwf protects factor 8 from liver protein C destruction so lack of vwf can caused factor 8 deficiency

Question 536

presentation of VW disease (4)

Answer 536

epistaxis
GI bleeding
menorrhagia
easy bruising

Question 537

investigation and results for VW disease (1:2)

Answer 537

bloods: low plasma vWF measurement, prolonged PT and prolonged APTT if factor 8 is low

Question 538

treatment for VW disease (2)

Answer 538

1. desmopressin (increases VWF release from endothelial Weibel Palade bodies)
2. tranexamic acid can reduce acute bleeding

Question 539

explain the pathophysiology of leukaemia 3

Answer 539

leukaemia= cancer of WBCs

1. immature blast cells uncontrollable proliferate
2. this take up space in bone marrow
3. the lack of space in the bone marrow means fewer healthy cells can mature and be released into the blood

Question 540

what cells in the haematopoeitic pathways does each type of leukaemia affect (4)
what is the key characteristic for each leukaemia? (4) And what age does it affect? 4

Answer 540

CML: myeloid stem cell
AML: myeloblast
ALL: lymphoblast
CLL: B lymphocytes

(ALL): children younger than 6 (<-)
(AML): auer rods (70+)
(CLL): smudge cells (adults 60+)
(CML): philadelphia chromosome (adults 40+)

Question 541

what is ALL, what is the mutation, what condition is it associated with

Answer 541

rapid proliferation of immature lymphoblasts
t(12:21)
Downs

Question 542

presentation for ALL (5) and typical ALL presentation

Answer 542

B SYMPTOMS (fever, night sweats, weight loss)
swollen testicles
hepatosplenomegaly
lymphadenopathy
HEADACHES/CN PALSIES (if infiltration of CNS)

ALL symptoms in question: Hepatosplenomegaly and the presence of bruising together with the symptoms of anaemia (soft systolic murmur and shortness of breath on exertion)

ALL ix in question: anaemia, neutropenia + thrombocytopaenia

Question 543

investigations for ALL 2

Answer 543

1. FBC: anaemia, thrombocytopenia, neutropenia
2. > 20% lymphoblasts on bone marrow biopsy (diagnosis)

Question 544

What indicates poor prognosis in ALL 5

Answer 544

male
under 2 years/ over 10 years
not white
when diagnosed WBC count higher than 20
having T/B cell receptors

Question 545

what is treatment for ALL (4)

Answer 545

1. chemotherapy + allopurinol
2. blood/ platelet transfusion
3. antibiotics
4. bone marrow transplant
   CABAB (like kebab)

Question 546

what is AML and what is the mutation

Answer 546

rapid proliferation of immature myoleblasts
t(15:17)

Question 547

presentation for AML (4)

Answer 547

anaemia
infections
hepatosplenomegaly
gum hypertrophy

Question 548

2 investigations and results for AML

Answer 548

FBC= anaemia, thrombocytopenia
bone marrow biopsy-> auer rods in cytology (diagnosis) and >20% myeloblasts (auer rods are MPO aggregrates in neutrophils cytoplasm)

Question 549

treatment for AML (4)

Answer 549

1. chemotherapy + allopurinol
2. blood/ platelet transfusion
3. antibiotics
4. bone marrow transplant

Question 550

why is allopurinol always given with chemo in AML and ALL

Answer 550

prevent tumour lysis syndrome-
where chemo releases uric acid form cells which accumulates in the kidneys

Question 551

what is CLL and what happens to the cells

Answer 551

proliferation of B lymphocytes
accumulation of incompetent lymphocytes due to failure of cell apoptosis

Question 552

presentation of CLL (4)

Answer 552

often asymptomatic
lymphadenopathy
might have night sweats and weight loss (B symptoms- due to bone marrow failure)

Question 553

what is the most common leukaemia in adults (not kids)

Answer 553

CLL

Question 554

investigations and results for CLL (2)

Answer 554

FBC= anaemia, thrombocytopenia, leukocytosis (high WCC)
blood film= smudge cells

Question 555

treatment for CLL (3)

Answer 555

1. watch and wait in early stages
2. chemotherapy (rituximab)
3. stem cell/ bone marrow transplant

Question 556

complication for CLL 1

Answer 556

Richter transformation- cancer becomes more aggressive

Question 557

what is the origin of CML and what gene mutation is it associated with

Answer 557

uncontrollable proliferation of myeloblasts from the myeloid stem cell

philadelphia chromosome translocation gene mutation (BCR-ABL gene)

Question 558

presentation of CML (8)

Answer 558

hepatosplenomegaly
hyper viscosity: headaches, thrombotic events
bone marrow failure: thrombocytopenia, neutropenia, anaemia
B symptoms
gout (tumour overproduces uric acid)

Question 559

treatment for CML (4)

Answer 559

1. tyrosin kinase inhibitors (imantinib)
2. allopurinol to prevent gout
3. chemotherapy
4. stem cell/ bone marrow transplant

Question 560

investigations for CML (2)- what is diagnostic

Answer 560

FBC= anaemia, thrombocytopenia, leukocytosis
genetic testing for BCR ABL (diagnostic)

Question 561

What causes haemolytic disease of the newborn (HDN)

Answer 561

incompatability of mother and newborn regarding antigens and antibodies:

-> mum is rhesus D- (no rhesus D antigen) but baby is rhesus D + (has RhD antigens)
-> fetuses blood makes its way into mum’s bloodstream
-> mum has immune response to baby’s RhD antigens and produces anti-D antibodies which sensitises her to these antigens
-> this does not normally affect the first pregnancy as IgM anti D antibodies are formed which cannot cross the placenta
-> in next pregnancy when baby is RhD+, mums immune response causes IgG anti D antibodies which can cross via placenta to fetus
-> these antibodies attach to RBC of fetus= immune system of fetus to attack its own RBCs

-> first baby that sensitizes mum can be due to antepartum bleeding/ miscarriage/ amniocentesis

Question 562

What is the presentation of Haemolytic Disease of the Newborn? 3

Answer 562

-yellowing of skin/ eyes (jaundice- due to high bilirubin levels from RBC destruction)
-pale skin and weakness (anaemia)
-oedema leading to hydrops fetalis

Question 563

What is the mx and prophylaxis for haemolytic disease of hte newborn

Answer 563

mum: give anti D immunoglobulin for birth, amniocentesis, miscarriage and antenatal haemorrhages

baby: treat any symptoms appropriately

anti-D is only prophylaxis and only has a role when mum hasn’t been sensitized already- so anti D antibody testing is done at the first antenatal visit to find out if mum is Rhesus +/-

anti-D immunoglobulin at 28 and 34 weeks to prevent sensitization for Rh - mums

Question 564

What are the ix for haemolytic disease of the newborn 2

Answer 564

ix: direct coombs test= positive (agglutination) of RBCs with coombs agent (take bloods from cord after delivery)
PLUS Kleihauer test: add acid to maternal blood, fetal cells are resistant

Question 565

What does the UK vaccination schedule involve

Answer 565

1 month: (after newborn bloodspot) offeref BCG if at increased risk, this is a live vaccine
2 months: 6 in 1, oral rotavirus and Men B
3 months: 6 in 1, oral rotavirus and PCV (pneumococcal)
4 months: 6 in 1, Men B
12 months: HiB/Men C + MMR + PCV + Men B
3 years, 4 months (pre school boosters): 4 in 1 + MMR
12/13: HPV
13/18: 3 in 1, Men ACWY

6 in 1= Diphtheria, tetanus, pertussis (whooping cough), polio, Haemophilus influenzae type b (Hib) and hepatitis B

4 in 1= Diphtheria, tetanus, pertussis (whooping cough) and polio

3 in 1= Tetanus, diphtheria and polio

live vaccines= MMR, polio, BCG and rotavirus

Question 566

What are the uk vaccines offered to over 65s

Answer 566

annual influenza
PCV (pneumococcal)
shingles

Question 567

What is the appropriate ix for coeliacs when there is an Iga deficiency

Answer 567

endomyseal antibodies

Question 568

How does aplastic crisis in Sickle Cell present?

Answer 568

combination of tachypnoea, tachycardia in the absence of splenomegaly.

Question 569

how does pityriasis rosea present

Answer 569

initial herald patch, then
xmas tree distribution appears roughly a week after
herald patch and rest of rash look different to each other

Question 570

how do you calculate IV fluid requirements in paediatrics

Answer 570

first 10kg 100ml per kilo
next 10kg 50ml per kilo
next 10kg 20ml per kilo
over 24 hours then divide to get per hour

Question 571

Explain the red traffic light system and mx for each

Answer 571

categories: colour, activity, resp rate, circulation/ hydration, other

(green) Low: normal colour, moist membranes, alert/ responds normally to social cues

(orange) Intermediate: pallor, abnormal activity, RR above 50 and sats 95 or below, tachycardic (over 140 in 2-5 year old) and CRP 3+ and dry mucous membranes/ reduced urine output, other: 5 days of fever, non weight bearing joint and 3-6 months old temp 39+

(red) Severe: blue, absent activity, RR>60 or O2 90- and grunting, reduced skin turgor, other: under 3 month temp 38+, non blanching rash, bulging fontanelle

Low risk: safety net
Inter risk: F2F assessment to judge if admission required
High risk: urgent admission

Question 572

What is Molluscum contagiosum presentation and management

Answer 572

shiny papules that have a dimple in the middle
mx: no tx necessary but avoid spread by not sharing towles, no school exclusion

Question 573

explain the feverpain score for sore throat explain mx and ddx

Answer 573

determines likelihood of strep (bacterial) cause for sore throat to prevent prescribing abx for all sore throats

each of these gives a point /5
fever in past 24 hours
pus on tonsils
absence of cough/ coryza
severe tonsil inflammation
not longer than 3 days ago (sx no longer than 3 days ago)
FeverPAIN

in 2 or 3 consider abx
in 4, give antibiotics

ddx of sore throat (pharyngitis)= EBV viral cause so infectious mononucleosis

Question 574

what are normal examination findings in paediatrics 4

Answer 574

respiratory rate between 30-60 breaths per minute,
pulse between 100-160 beats per minute in a newborn, temperature of around 37 Celsius
pass urine and stool regularly.

Question 575

What are the four paediatric cardiac defects and describe the findings on a stethoscope examination

Answer 575

Ventricular septal defect Pansystolic murmur in lower left sternal border
Coarctation of the aorta Crescendo-decrescendo murmur in the upper left sternal border
Patent ductus arteriosus Diastolic machinery murmur in the upper left sternal border
Pulmonary stenosis Ejection systolic murmur in the upper left sternal border

Question 576

mx of recurrent UTIs in children 2

Answer 576

in 6 weeks: US scan and DMSA

Question 577

What is the mx for resuscitation in kids

Answer 577

5 rescue breaths first (as kids are more likely to suffer a respiratory arrest)
15:2 compressions

Question 578

CMPA vs GORD presentation

Answer 578

both have non-billous, non-projectile vomiting
CMPA: diarrhoea and can have blood in stools

Question 579

What is the most common cause of febrile convulsions

Answer 579

roseola infantum- HH6

Question 580

what is the step up for ventilation?

Answer 580

1. high flow oxygen
2. CPAP
3. intubation

Question 581

What conditions have the nikolski sign 2

Answer 581

SJ syndrome
scalded skin syndrome

Question 582

what is koebner phenomenon

Answer 582

psoriasis develops in areas of trauma/ friction

Question 583

explain GCS

Answer 583

out of 15 (M6V5E4)

eye opening:
4 spontaneous
3 to voice
2 to pain
1 none

motor:
6 normal spontaneous movement
5 withdraws to touch
4 withdraws to pain
3 abnormal flexion
2 abnormal extension
1 none

verbal:
5 coos/ babbles
4 irritable cries
3 cries to pain
2 moans to pain
1 none

mild= 13-15
mod= 9-12
severe=3-8

Question 584

explain APGAR out of 10

Answer 584

pulse >100 2
pulse <100 1
absent 0

strong cry 2
weak cry 1
no cry 0

pink 2
extremities blue 1
all blue 0

actively moving 2
limb flexion 1
floppy 0

cries on stimulation/ sneezes/ coughs 2
grimaces 1
non 0

Question 585

Explain prader willi syndrome mutation and characteristics and mx

Answer 585

ch15: total expression of mum due to loss of paternal chromosome- this is called genomic printing
15q11-13 deletion (aww ur 15 cute, boy is like no i want to be 11-13)

sx:
insatiable hunger
pale
hypotonia
almond eyes
behavioral problems during adolescent years

mx
give growth hormones (isn’t allowed to be 11-13, needs to grow)
dietician (due to hunger + obesity issues)

cm

Question 586

Explain angelman syndrome mutation and characteristics and mx

Answer 586

caused by UBE3A gene (you should be in 3a)- total expression of dad Chromosome 15

1. hydrophillic- loves water activities + shiny things (sensory hits the spot)
2. spaced out teeth
3. LD
4. neuro sx- seizures
5. happy demeaner- happy

Question 587

explain noonans syndrome, inheritence, mutation, sx, increased rfx

Answer 587

autosomal dominant
mutatation: PTPN11
Male version of turners PLUS
sx:
factor 11 deficiency
pulmonary valve stenosis
triangle face
low set ears

CHD
leukaemia
neuroblastoma

Question 588

Henoch-Schonlein purpura

Answer 588

It commonly presents with a triad of purpura/petechiae on the buttocks and lower limbs, abdominal pain and arthralgia
give NSAIDS

Question 589

explain williams cause, sx and dx

Answer 589

microdeletion on chromosome 7
Symptoms:
elfin facies
very friendly and social
learning difficulties
aortic stenosis

cute friendly little elfin facies elf boy with LD that everyone loves and loves talking about fishes but everyones sad bc he’s going to die of his aortic stenosis)

Question 590

What is the action for suboptimal o2 sats in resuscitation

Answer 590

if well perfused and no other issues, then in first 10 minutes of life, suboptimal O2 readings can be expected form a healthy neonate= NO action needed

Question 591

define cyanosis medically

Answer 591

hb of more than 5g/dl