Paediatrics

Question 1

Causes of pneumonia in children

Answer 1

Commonly viral: adenovirus, rhinovirus, influenza, RSV, parainfluenza
2nd most common= Streptococcus pneumoniae

Question 2

Presentation of pneumonia in children

Answer 2

Temperature (over 38.5), rapid breathing/difficulty breathing
May have cough, vomiting, chest/abdo pain, decreased activity, loss of appetite/poor feeding

Question 3

Management of paediatric pneumonia

Answer 3

Antibiotics if indicated. CXR and bloods, sputum culture. Supportive therapy.

Question 4

What is croup and its presentation

Answer 4

Infection of upper airway (often parainfluenza virus) leads to obstruction of breathing and a barking cough

Px: originally begins with typical cold then 3-5 days of loud barking cough, fever, hoarse voice, noisy/laboured breathing. Symptoms worse at night

Question 5

How to manage croup?

Who most commonly affected?

Answer 5

Manage at home with comfort measures

Common between 6 months and 3 years

Question 6

Presentation of asthma?

Answer 6

Cough (nocturnal and worsens with virus), SOB, wheeze/whistling, chest congestion or tightness

Question 7

Diagnosis of asthma in children?

Answer 7

If over 5: spirometry, PEF, allergy testing
If under 5: need to go off the history
If under 3: may use wait and see approach

Question 8

How to identify a viral induced wheeze?

Answer 8

No wheeze with exercise, no wheeze except when a/w viral infections

Question 9

How to manage a viral induced wheeze?

Answer 9

Reliever inhaler for the minority=SABA=salbutamol
If SABA 2-10 puffs PRN to max 4 hourly via a spacer
Otherwise, supportive/comfort care

Question 10

Natural history of bronchiolitis?

Answer 10

Viral infection of bronchioles, commonly by RSV
Usually affects under 2s
Mainly in the winter and spring months
There is excess mucus, inflammation, constriction and oedema

Question 11

Presentation of bronchiolitis?

Answer 11

Symptoms increase over 2-5 days: low grade fever, nasal congestion, rhinorrhoea, cough, feeding difficulty

Usually lasts 7-10 days

Question 12

Investigations for bronchiolitis?

Answer 12

Nasopharyngeal aspirate or throat swab
RSV rapid testing and viral cultures
FBC

Question 13

Home or hospital for bronchiolitis?

Answer 13

Hospital if severe apnoea/resp distress: grunting, RR>70, central cyanosis, sats<92%

Consider hospital if rr>70, inadequate fluids, clinical dehydration

No role for antibiotics, steroids or bronchodilators

Question 14

Pathaphysiology of cystic fibrosis

Answer 14

Autosomal recessive mutation in CFTR (CF transmembrane conductane regulator gene)
DeltaF508 is commonest mutation

MUCUS

Reduced airway surface liquid impedes mucus clearance allowing excessive bacterial growth
Pancreas: duct occluded in utero leading to pancreatic insufficiency (can lead to CF-related DM)
GI: increased mucus can cause meconium ileus
Biliary tree: can have cholestasis leading to neonatal jaundice

Question 15

Neonatal test to identify CF

Answer 15

Heel prick- Guthrie test

Question 16

Investigations for CF:

Answer 16

Diagnosis= history and positive chloride sweat test

Sweat chloride over 60mmol/L suggestive, 40-60 is borderline

CXR, sweat test, glucose tolerance test, microbiology, LFT, coag, bone profile, PFTs- all generally involved in annual assessments

Heel prick, then genetic testing, then sweat test

Question 17

Why are children more at risk of epiglottitis than adults?

Answer 17

Epiglottis is floppier, broader, longer and angled more obliquely to trachea and have a larger tongue
Therefore higher risk of acute airway obstruction

Question 18

Aetiology of epiglottitis?

Answer 18

Reduced since the Hib vaccination
Normally Haemophilus infleunzae and Streptococcus pneumoniae which locally invade

V rarely due to trauma or non-infectious causes

Question 19

Presentation of epiglottitis

Answer 19

4Ds
Dyspnoea, dysphagia, drooling, dysphonia (muffled hot potato voice)

Typically no cough
Symptoms in less than 12 hours
Stridor is a late sign
Tripod position- leans forward on outstretched arms with neck extended and tongue out

Question 20

Management of epiglottitis

Answer 20

Secure airway before further investigation
Throat swabs, bloods, lateral neck Xray (thumb print sign, thickened aryepiglottic folds, increased opacity of larynx and vocal cords)

Oxygen, nebulised adrenaline, IV antibiotics (3rd gen cephalosporins eg ceftriaxone), IV steroids, nil by mouth until airway improved

Question 21

What is acute otitis media

Symptoms

Answer 21

Severe pain and visible inflammation of tympanic membrane lasting days-weeks
Sxs: pain, malaise, fever, coryzal symptoms

Question 22

What causes otitis media

Answer 22

Infection from nasopharyngeal organisms migrating via eustachian tube (which is shorter wider and straighter in children)

Bacterial= S.pneumoniae
Viral=RSV, rhinovirus

Question 23

Investigations for otitis media

Answer 23

ALWAYS test function of facial nerve on examination
Otoscopy
Discharge sent for microscopy and culture

Question 24

How to manage acute otitis media

Answer 24

Watch and wait- most spontaneously resolve within 24 hours
Simple analgesia
Complication monitoring= mastoiditis which will need IV abx

Question 25

What proportion of children will have had at least 1 episode of otitis media by age 3

Answer 25

more than 2/3

Question 26

What is otitis media with effusion

Answer 26

glue ear Viscous inflammatory fluid build up leading to conductive hearing impairment via chronic inflammatory changes and eustachian tube dysfunction

Question 27

What are risk factors for otitis media with effusion?

Answer 27

Bottle fed, paternal smoking, atopy, genetic disorders egCF and Downs

Question 28

Presentation of otitis media with effusion?

Answer 28

Difficulty hearing and pressure sensation | O/E: TM will be dull and light reflex is lost, may see a bubble behind the TM

Question 29

How to manage otitis media with effusion?

Answer 29

Active surveillance- around 50% resolve within 3 months | If no resolution may need hearing aids or myringotomy and grommet insertion

Question 30

Indications for grommets?

Answer 30

Small plastic tubes inserted in opening in eardrum | Relieve OME if hearing loss is 25-30dB on 2 occasions 3 months apart

Question 31

Causes of deafness in children (infections)

Answer 31

Temporary=OME | Meningitis, mumps and measles can cause it

Question 32

Aetiology of tonsillitis

Answer 32

Viral mostly Bacterial-group A strep most common (S.pyogenes) Most common in age 5-15

Question 33

Tonsillitis- bacterial vs viral assessment

Answer 33

Centor score: Age, exudate, tender/swollen anterior cervical LNs, temp over 38, absent cough

Question 34

Management of tonsillitis

Answer 34

Viral is self limiting | Bacterial- should swab for culture and commence antibiotics (usually benzylpencillin)

Question 35

What is the epidemiology of periorbital cellulitis?

Answer 35

Peak in under 10s Males twice as commonly affected Peak occurrence in later winter and early spring

Question 36

What are the causes of periorbital cellulitis?

Answer 36

Commonly from contiguous spread from sinuses with ethmoidal sinus being most common Others= dental infection, trauma, foreign bodies, impetigo S. pneumoniae and S. aureus

Question 37

What is the classification used for periorbital cellulitis?

Answer 37

Chandler classification, types i-v

Question 38

What is type I of chandler classification?

Answer 38

Pre-septal cellulitis- inflammation and oedema anterior to orbital septum

Question 39

type II of chandler classification?

Answer 39

post-septal, orbital cellulitis: inflammation into orbital tissue but no abscess formation Eyelids may be swollen and may have conjunctival chemosis with variable degree of proptosis and visual loss

Question 40

type III chandler classification?

Answer 40

subperiosteal abscess Pronounced eyelid oedema, conjunctival chemosis and tenderness along orbital rim with variable degree of motility/proptosis/visual acuity changes

Question 41

type iv chandler classification?

Answer 41

intra-orbital abscess-pus inside or outside muscle cone Proptosis, conjunctival chemosis, decreased motility and visual loss can be severe

Question 42

type v chandler classification?

Answer 42

Cavernous sinus thrombosis Bilateral marked eyelid oedema and involvement of CNs III, V, VI May have sepsis signs

Question 43

How to manage periorbital cellulitis?

Answer 43

If mild preseptal can do from home with broad spectrum empirical abx If more extensive- hospital, IV abx, supportive therapy If large abscesses, intracranial complications at px and in frontal sinusitis, need urgent drainage

Question 44

Complications of periorbital cellulitis?

Answer 44

``` Visual related (vision loss is 11%)- start to lose red colour vision (sign of optic nerve compromise) Neurological complications eg sepsis, intracranial abscess, cavernous sinus thrombosis ```

Question 45

Describe the types of manifest and latent strabismus

Answer 45

``` Manifest: Esotropia=eye turns in Exotropia=eye turns out Hypertropia=eye goes up Hypotropia=eye goes down ``` ``` Latent: Esophoria=inwards under occluder Exophoria=outwards under occluder Hyperphoria=up under occluder Hypophoria= down under occluder ```

Question 46

what tests to identify strabismus

Answer 46

``` Cover test (manifest squint) Cover/uncover test (latent squint) ```

Question 47

What are potential septal defects?

Answer 47

Ventricular septal defect Atrial septal defect Atrioventricular septal defect Tetralogy of fallot

Question 48

What is the most common septal defect?

Answer 48

VSD

Question 49

What is eisenmenger's syndrome?

Answer 49

A left to right shunt switches direction due to an increase in pressure of the low oxygen-containing side CYANOTIC

Question 50

Sizes of VSD and what it may cause

Answer 50

Small=restrictive VSD-generally asx Moderate=dilatation of left atrium and ventricle, at risk of congestive heart failure and arrhythmias Large=early HF and severe pulmonary hypertension, may develop eisenmenger's syndrome

Question 51

What presentation with VSD?

Answer 51

tachypnoea SOB, feeding problems, developmental issues undernourished, forehead sweat, increased breathing work, cyanotic, may have down's syndrome, clubbing, hyperactive precordium, thrill, systolic murmur

Question 52

Murmur for VSD and where to listen?

Answer 52

``` Systolic murmur (holosystolic, pansystolic or early systolic) Best heard over tricuspid area (lower left sternal border) with radiation to right lower sternal border ```

Question 53

Gold standard diagnosis of septal defect?

Answer 53

Echo

Question 54

Management of VSD?

Answer 54

Qp/Qs (pulmonary to systemic blood flow ratio) of at least 2.0 requires surgical repair Aim to close large VSDs within first 2 years of life 75% of small VSDs close spontaneously by age 10

Question 55

What is the second most common septal defect?

Answer 55

ASD

Question 56

What are the 5 types of atrial septal defect?

Answer 56

``` patent foramen ovale (most common) ostium secundum defet ostium primum defect sinus venosus defect coronary sinus defect (least common) ```

Question 57

Presentation of ASD?

Answer 57

Vast majority are asymptomatic | Large= tachypnoea, poor weight gain, recurrent chest infections

Question 58

Type of murmur in ASD, and where to auscultate?

Answer 58

Soft systolic ejection murmur | Best heard in pulmonary area (left upper sternal edge)

Question 59

RFs for ASD

Answer 59

maternal smoking in first trimester, maternal diabetes, maternal rubella, maternal drug use eg cocaine/alcohol

Question 60

Management of ASD?

Answer 60

If less than 5mm, spontaneous closure within 12 months | Surgical closure required if bigger than 1cm

Question 61

What group of people is more likely to have an atrioventricular septal defect?

Answer 61

People with Down's syndrome

Question 62

Describe the pathaphysiology of an AVSD? | 2 types

Answer 62

Failure of endocardial cushions to fuse properly Complete AVSD: large left to right shunt causes excessive pulmonary blood flow leading to HF and pulmonary vascular resistance Partial AVSD: left to right shunt and volume overload of RA and RV but the pulmonary artery pressure is only mildly elevated

Question 63

Presentation of AVSD?

Answer 63

Tachypnoea, tachycardic, poor feeding, sweating, failure to thrive, all with a complete AVSD are symptomatic by 1 year Prominent sternal heave, murmur

Question 64

Murmur in AVSD and where to auscultate?

Answer 64

Ejection systolic murmur in pulmonary area (left upper sternal border) Mid-diastolic murmur over tricuspid area (left lower sternal border) Holosystolic murmur might be heard at apex

Question 65

Management of AVSD?

Answer 65

Surgical management as patients would die by age 2-3 without

Question 66

Describe the tetralogy of fallot?

Answer 66

Ventricular septal defect Pulmonary stenosis Right ventricular hypertrophy Overriding aorta Most common cyanotic congenital heart disease

Question 67

Presentation of tetralogy of fallot?

Answer 67

Mild=usually asx, but as disease progresses, develops cyanosis Mod-severe=cyanotic and resp distress Extreme= pulmonary atresia or absent pulmonary valves Murmur

Question 68

If tetralogy of fallot isn't managed, what can occur?

Answer 68

polycythaemia, cerebral abscess, stroke, infective endocarditis, HF, death

Question 69

Murmur in tetralogy of fallot and where to listen?

Answer 69

Pansystolic murmur best heard over mid or upper left sternal edge Smaller the VSD=louder the murmur

Question 70

What are tet spells?

Answer 70

Hypoxic spells in tetralogy of fallot with peak incidence between 2-4 months of life: Paroxysm of hyperpnoea, irritability, increasing cyanosis

Question 71

Management of tetralogy of fallot?

Answer 71

Medical= squatting, prostaglandin infusion, beta blockers, morphine, saline bolus Surgical management for definitive repair or palliative management

Question 72

Which defects have a higher risk of HF?

Answer 72

Significant left to right shunt= VSD, ASD, PDA | Transposition of the great arteries

Question 73

Describe the two types of transposition of the great arteries?

Answer 73

dextro-transposition=aorta is anterior and to the right of the pulmonary artery levo-TGA= aorta anterior and to the left of the pulmonary artery

Question 74

When do you get cyanotic heart disease?

Answer 74

``` Absent/not wide enough tricuspid/pulmonary/aortic valve Coarctation of aorta Ebstein anomaly Tetralogy of fallot TGA Truncus arteriosus ``` Anywhere with a right to left shunt

Question 75

What is rheumatic fever?

Answer 75

Systemic illness 2-4 weeks after pharyngitis due to cross reactivity to group A beta-haemolytic streptococcus (S. pyogenes)

Question 76

What are the diagnostic criteria for rheumatic fever? Describe

Answer 76

Revised Jones diagnostic criteria= positive throat culture or anti-streptolysin O/anti-DNA B titre and 2 major or 1 major+2 minor criteria

Question 77

What are the major criteria in the revised jones criteria?

Answer 77

SPECS | Sydenham's chorea, polyarthritis, erythema marginatum, carditis, subcut nodules

Question 78

What are the minor criteria in the revised jones criteria?

Answer 78

``` CAPE CRP/ESR raised Arthralgia Pyrexia ECG-prolonged PR ```

Question 79

Management of acute rheumatic fever?

Answer 79

Antibiotics=benzathine benzylpenicillin Aspirin/NSAIDs Assess for emergency valve replacement Secondary prevention with abx every 3-4 weeks

Question 80

What factors lead to subacute bacterial endocariditis?

Answer 80

Endothelial damage, platelet adhesion, microbial adherence

Question 81

What organisms cause IE?

Answer 81

S. aureus, S. viridans, S. pneumoniae, HACEK organisms

Question 82

Presentation of IE?

Answer 82

persistent low grade fever, heart murmur, splenomegaly, petechiae/oslers nodes/janeway lesions/splinter haemorrhages

Question 83

Criteria for diagnosis of IE? describe

Answer 83

Modified Dukes criteria 2 major 1 major and 3 minor 5 minor

Question 84

Major criteria in modified dukes?

Answer 84

positive blood cultures, evidence of endocardial involvement (echo)

Question 85

Minor criteria in modified dukes?

Answer 85

predisposition, fever, vascular phenomena, immunologic phenomena, microbiology evidence, other echo findings

Question 86

Management of IE?

Answer 86

IV antibiotics | May require surgical intervention

Question 87

What factors aid reflux in GORD?

Answer 87

Low tone of muscular portion of lower oesophagus, short and narrow oesophagus, delayed gastric emptying, shorter lower oesophageal sphincter, liquid diet and high calorie requirement (distends stomach), significant periods recumbent

Question 88

Presentation of GORD?

Answer 88

Distressed, unexplained feeding difficulties, hoarseness +/- chronic cough, single episode pneumonia, faltering growth, if able may report pain

Question 89

When does GORD or regurgitation (=posseting) present normally

Answer 89

Within 2 weeks of life

Question 90

How to manage a breast fed baby with gord

Answer 90

Use alginate eg gaviscon mixed with water after feeds

Question 91

How to manage a formula fed baby with gord

Answer 91

``` 1= do not over feed (less than 150ml/kg/day) 2= decreased feed volume by increasing frequency (2-3 hourly) 3= use feed thickener 4= stop thickener and use alginate added to formula ```

Question 92

What to use after alginate if no success in GORD?

Answer 92

histamine antagonists or ppi

Question 93

Name 5 potential causes of poor feeding

Answer 93

Infection, metabolic disorders, genetic disorders, structural abnormalities, neurological disorders, mum and feeding technique

Question 94

Presentation of pyloric stenosis

Answer 94

Baby starts bringing up small amounts of milk after feeding, which gets worse over a few days Then projectile yellow vomit (milk curdles in stomach acid) Reduced faeces If untreated: dehydration and not gaining weight

Question 95

When does pyloric stenosis present

Answer 95

About 6 weeks after birth

Question 96

How to manage pyloric stenosis

Answer 96

Pyloromyotomy (laparoscopically)

Question 97

Potential presentation of IBS

Answer 97

Bloating, cramping, chronic or intermittent diarrhoea/constipation, urgency with defecation, incomplete sensation of defecation, passage of mucus in stool May have dizziness, nausea, loss of appetite

Question 98

How to manage IBS

Answer 98

Diet and lifestyle management eg small meals, more often, low fat and high carbs

Question 99

How common is gastroenteritis?

Answer 99

Very! | At least one episode per year for most children

Question 100

Common causes of gastroenteritis

Answer 100

Rotavirus is most common infantile (NB rotarix oral vaccine at 8 and 12 weeks) Norovirus commonest acorss all age groups Campylobacter is most common bacterial cause Also E.coli and adenovirus

Question 101

Presentation of gastroenteritis

Answer 101

Sudden onset diarrhoea +/- vomiting, abdo pain, mild fever | bloody diarrhoea if campylobacter- generally due to undercooked meat and unpasteurised milk

Question 102

Management of gastroenteritis

Answer 102

Self limiting Send a stool sample if ?septicaemia/blood or mucus in stool/immunocompromised Encourage fluids (not carbonated drinks) IV or oral therapy if dehydrated Give full strength milk asap Leave it 48 hours before returning to school

Question 103

How long do sxs of gastroenteritis last?

Answer 103

Diarrhoea around 5-7 days | Vomiting around 1-2 days

Question 104

Potential complications of gastroenteritis?

Answer 104

Haemolytic uraemic syndrome, reactive complications eg Reiter's syndrome, toxic megacolon, acquired/secondary lactose intolerance

Question 105

What counts as constipation in children?

Answer 105

Less than 3 complete stools/week Hard/large stool Rabbit droppings Overflow soiling in children over 1

Question 106

Management of simple constipation in children?

Answer 106

Diet and lifestyle advice- high fibre, increase fluids Then add Macrogol eg Movicol Then add stimulant laxative eg Senna Consider adding in lactulose In secondary care can do manual evacuation, antegrade colonic enema, polyethylene glycol solutions

Question 107

Describe the pathophysiology of appendicitis

Answer 107

``` Luminal obstruction (faecolith or lymphoid hyperplasia, impacted stool) Decreased venous drainage and localised inflammation by commensal bacteria causes and increased pressure. This causes ischaemia which can lead to necrosis and perforation ```

Question 108

Presentation of appendicitis

Answer 108

Dull peri-umbilical pain transfers to a sharp right iliac fossa pain May have vomiting, anorexia, nausea, diarrhoea or constipation MAGNET: migration of pain to RIF, anorexia, guarding, nausea, elevated temp, tenderness in RIF

Question 109

What can be found on examination of appendicitis

Answer 109

``` Rebound tenderness and percussion pain over McBurneys point and guarding Psoas sign (pain when patient lies on left side while right thigh is flexed backward) Rovsing's sign (pain in RLQ upon palpation of LLQ) ```

Question 110

Management of acute appendicitis

Answer 110

Antibiotics and laparoscopic appendicectomy

Question 111

Why are children at higher risk of appendix perforation?

Answer 111

Tend to have delayed presentation

Question 112

Two types of childhood hernia?

Answer 112

Umbilical and epigastric

Question 113

How to manage hernias in children?

Answer 113

Umbilical- surgery if still present after 3 years | Epigastric- surgery only if uncomfortable or a nuisance

Question 114

Describe how Crohn's disease affects the body (microbiology and location)

Answer 114

Remitting and relapsing disease, affects anywhere between the mouth and the anus Transmural inflammation, deep ulcers and fissures with cobblestone mucosa, skip lesions Non-caseating granulomatous inflammation

Question 115

Presentation of Crohns disease

Answer 115

Abdominal pain and bloody/mucus containing diarrhoea Oral aphthous ulcers, perianal disease (tags, fistulae) Extra-intestinal features=MSK, eyes, skin, renal, hepatobiliary

Question 116

Investigations for IBD?

Answer 116

Faecal calprotectin, routine bloods, stool microscopy and culture, colonoscopy with biopsy (=definitive diagnosis and can distinguish between UC and CD)

Question 117

How to manage Crohn's disease? Acute attack

Answer 117

In acute attack: avoid anti-motility drugs eg loperamide as it can cause toxic megacolon In acute attack: fluid resus, nutritional support, prophylactic heparin, corticosteroid and immunosuppressant eg mesalazine or azathioprine

Question 118

How to manage Crohn's disease? Maintenance

Answer 118

Azathioprine, smoking cessation | Surgery can be used- ileocaecal resection, stricturoplasty, small/large bowel resections

Question 119

Course and pathophysiology of ulcerative colitis?

Answer 119

Relapsing/remitting Diffuse continual mucosal inflammation of large bowel only. Begins in the rectum and spreads proximally. Mucosal and submucosal inflammation only, crypt abscesses and goblet cell hypoplasia

Question 120

What is a protective factor against UC?

Answer 120

Smoking!

Question 121

Presentation of UC?

Answer 121

Bloody diarrhoea, PR bleeding and mucus discharge, increased faecal frequency, urgency and tenesmus Extra-intestinal: MSK, skin, eyes, hepatobiliary

Question 122

How to induce remission in UC?

Answer 122

Corticosteroid and immunosuppressant eg mesalazine or azathioprine

Question 123

Maintenance management of UC?

Answer 123

Immunomodulators- mesalazine or sulfasalazine Next line=infliximab Surgery= ileostomy and complete resection= curative, but many have sub-total colectomy to begin with

Question 124

Pathophysiology of Coeliac disease

Answer 124

T cell mediated autoimmune response. Response to gliadin and genetic (HLA-DQ2/DQ8) Anti-gluten CD4 t cell response, with anti-gluten antibodies, anti-tissue transglutaminase, endomysin antibodies and intraepithelial lymphocytes activated Causes epithelial cell destruction and villous atrophy

Question 125

What are the forms of coeliac disease?

Answer 125

Classical, atypical, latent, silent, potential

Question 126

Describe the classical form of coeliac disease

Answer 126

Arises between 9 and 24 months Crypt hyperplasia and villous atrophy Features of malabsorption eg steatorrhoea, failure to thrive, anorexia

Question 127

Describe the atypical form of coeliac disease?

Answer 127

No intestinal symptoms | Extra-intestinal symptoms eg osteoporosis, anaemia, peripheral neuropathy

Question 128

Describe the latent form of coeliac disease

Answer 128

HLA DQ2/8 positive but normal mucosa present

Question 129

Describe the silent form of coeliac disease

Answer 129

Damaged mucosa, positive serology, no symptoms

Question 130

What is potential coeliac disease

Answer 130

Genetically predisposed people

Question 131

What are potential extra-intestinal features of coeliac disease?

Answer 131

Dermatitis herpetiformis, osteoporosis, short, delayed puberty, arthritis

Question 132

How to investigate for coeliac disease?

Answer 132

Serology Must have had gluten in diet for at least 6 weeks prior to testing IgA and IgAtTG, IgAEMA Duodenal biopsy

Question 133

Common causes of failure to thrive

Answer 133

Down syndrome, cerebral palsy, heart disease, infections, milk allergy, CF, coeliac disease, GORD

Question 134

What is marasmus

Answer 134

Protein and energy malnutrition. Loss of fat mass especially buttocks Chronic diarrhoea and emaciated body type There is NO oedema

Question 135

What is marasmus usually a sign of?

Answer 135

Poor socioeconomic status, child abuse+neglect, lack of food resources

Question 136

How to manage marasmus?

Answer 136

Similarly to kwashiorkor and reintroduce food slowly and correct any electrolyte imbalances

Question 137

What is kwashiorkor

Answer 137

Inadequate protein with a reasonable calorie intake

Question 138

Presentation of kwashiorkor

Answer 138

Fatigue, irritability, lethargy, failure to thrive, loss of muscle mass, generalised oedema, pot belly, fatty liver, prone to infections, hair and skin changes (erythematous, exfoliation, pigment, dry hair, thin nails)

Question 139

What is seen in bloods for kwashiorkor?

Answer 139

low glucose, low protein, high cortisol and GH, low salt, iron deficiency anaemia, metabolic acidosis

Question 140

Management of kwashiorkor?

Answer 140

Correct fluids/electrolytes- needs to be done in about 48 hours Reintroduce foods slowly afterwards using RUTF (ready to use therapeutic foods eg peanut butter, milk powder, sugar, vegetable oil, minerals and vitamins) Treatment takes around 2-6 weeks

Question 141

Pathophysiology of Hirschprung's disease

Answer 141

``` Congenital aganglionic megacolon disease Aganglionic segment (short/long/total) remains in tonic state so there is a failure in peristalsis Functional obstruction due to accumulated faeces ```

Question 142

Presentation of Hirschprung's disease

Answer 142

Abdominal distension, bilious vomiting, failure to pass meconium within 48 hours after birth Males 4 times more likely

Question 143

What is a big complication of Hirschprung's disease

Answer 143

Hirschprung's enterocolitis: stasis allows bacterial proliferation, which can cause sepsis and death

Question 144

How to investigate for Hirschprungs

Answer 144

Initially a plain X ray | Gold standard=rectal suction biopsy

Question 145

Management of Hirschprungs

Answer 145

IV antibiotics, NG tube, bowel decompression initially Definitive= surgery to resect aganglionic section and connect unaffected bowel to dentate line

Question 146

What is intussusception? | Who does it happen to?

Answer 146

Movement/telescoping of one part of bowel into another Peak at 5-7 months, rare after 2 years, males twice as likely Most cases are ileo-colic type (distal ileum passes into caecum through ileo-caecal valve)

Question 147

Presentation of intussusception

Answer 147

Sudden onset, inconsolable crying episodes, pallor, child may draw knees up to chest Later stages= red-currant consistency stools (blood and mucus), sausage shaped mass in RUQ

Question 148

How to diagnose intussusception?

Answer 148

abdominal uss

Question 149

How to manage intussusception?

Answer 149

Nonoperative=air or contrast enema | Surgical reduction if contraindication to enema

Question 150

What is meckel diverticulum?

Answer 150

Outpouching of lower part of small intestine- a remnant of umbilical cord

Question 151

Presentation of meckel diverticulum?

Answer 151

Most asx In minority: pouch releases acid causing bleeding ulcers In serious complications, can lead to peritonitis Pouch can twist causing volvulus or intussusception

Question 152

What is malrotation with volvulus?

Answer 152

Occurs during embryonic development at about the 10th week | Bowel twists so blood supply is cut off

Question 153

Presentation of malrotation with volvulus?

Answer 153

Bouts of crying, pull legs into body, not passing faeces, cramps, green vomit, may become dehydrated Most diagnosed by age of 1

Question 154

How to manage malrotation with volvulus

Answer 154

Dx by Xray | Tx= operation- stop necrosis of bowel and may need bowel resection

Question 155

What is toddler diarrhoea

Answer 155

Chronic nonspecific diarrhoea, at least 3 loose watery stools/day Some alternate with constipation Self limiting at about age 5-6 NOT due to malabsorption or serious bowel problem

Question 156

What are the 4Fs for diet consideration in toddler diarrhoea

Answer 156

Fat, fluid, fruit juices and fibre

Question 157

What is colic

Answer 157

Self-limiting repeated episodes of excessive and inconsolable crying for at least 3hrs a day, at least 3 days a week for at least 1 week Occurs in up to 4 months of age

Question 158

Presentation of colic

Answer 158

Often crying in late afternoon or evening, draws knees up and arches back

Question 159

Management of colic?

Answer 159

REASSURANCE Soothing strategies, parental wellbeing aids Discourage simeticone (infracol) or lactase drops, probiotics, herbal supplements and maternal diet modification Exclude other possible causes of distress

Question 160

Describe the three types of biliary atresia

Answer 160

Type I= common bile duct atresia with patent proximal ducts Type II= common hepatic duct atresia with cystic structures in porta hepatis Type III= R and L hepatic duct atresia to level of porta hepatis (most common)

Question 161

What is biliary atresia?

Answer 161

Extrahepatic bile ducts obliterated by inflammation and subsequent fibrosis

Question 162

Presentation of biliary atresia?

Answer 162

Presentation shortly after birth Persistent jaundice Pale stools and dark urine in term infants with normal birth weights

Question 163

What investigations are required in biliary atresia and what results shown?

Answer 163

LFTs: increased GGT, TGs normal USS Liver histology from percutaneous biopsy

Question 164

Management of biliary atresia?

Answer 164

Surgery, liver transplant needed before 8 weeks | Prognosis without treatment is 18 months

Question 165

Complications of biliary atresia?

Answer 165

Ascending cholangitis, cirrhosis, portal hypertension, liver failure, hepatocellular carcinoma

Question 166

Pathophysiology of cow's milk protein allergy

Answer 166

Immune-mediated allergic response to casein and whey | IgE mediated type 1 hypersensitivity or non-IgE(T cell activation)

Question 167

Presentation of cow's milk protein allergy

Answer 167

Acute and rapid onset (less than 2 hrs after ingestion), pruritus, erythema, acute urticaria, angio-oedema, oral pruritus, colicky abdo pain, vomiting, diarrhoea, may have resp signs

Question 168

Management of cow's milk protein allergy

Answer 168

Elimination diet for at least 6 months or until 9-12 months old, then reevaluate every 6-12 months Replace formula with hypoallergenic: extensively hydrolysed or amino acid formula

Question 169

What is wilson's disease?

Answer 169

Autosomal recessive disorder of hepatic copper deposition Mutations in gene ATP7B Typical onset in 2nd-3rd decade

Question 170

Presentation of wilson's disease

Answer 170

HEPATIC features: acute liver failure, chronic hepatitis and cirrhosis, fulminant hepatic failure+/- haemolytic anaemia PSYCH features: severe depression, neuroses NEURO features: asymmetrical tremor, speaking difficulty, hypersalivation, clumsiness with hands Kayser-fleischer rings, sunflower cataracts May be a/w rheumatoid-osteopenia, cardiac arrhythmias, hypoparathyroidism, pamcreatitis, infertility

Question 171

What investigations for wilson's disease

Answer 171

Low serum caeruloplasmin Increased urinary copper Liver biopsy Assay

Question 172

Management of wilson's disease

Answer 172

Avoid high copper food, penicillamine or zinc/trientine (chelating agents), may need liver transplant

Question 173

Choledochal cysts pathophysiology?

Answer 173

Usually in extrahepatic ducts (CBD and HDs), sometimes in intrahepatic ducts Type I cysts are most common-lower end of CBD joins up with pancreatic duct: pancreatic juice and bile mix leading to a weakening of bile duct wall and ballooning- can lead to pancreatitis and cholangitis

Question 174

Management of choledochal cyst

Answer 174

USS to identify Commonly incidental finding Surgery for removal of cyst

Question 175

Presentation of choledochal cyst

Answer 175

May have jaundice, intermittent abdo pain, cholangitis, peritonitis, lump, pancreatitis

Question 176

Aetiology of neonatal hepatitis syndrome

Answer 176

in 20%- viral (cytomegalovirus, rubella, hep a/b/c) | 80%- nonspecific virus

Question 177

Presentation of neonatal hepatitis syndrome

Answer 177

Usually at 1-2 months | Jaundice, not gaining wt/ht, hepatosplenomegaly

Question 178

Management of neonatal hepatitis syndrome

Answer 178

Ix: liver biopsy and bloods Mx: none specific, may need vitamins, potentially a liver transplant

Question 179

Presentation of liver failure in children

Answer 179

Increased AST and ALT with prolonged clotting +/- jaundice +/- encephalopathy

Question 180

Causes of liver failure

Answer 180

Viruses eg HSV, EBV, CMV, Hep A/B/E Inherited metabolic disorders eg Wilsons, mitochondrial Toxins Medication eg erythromycin Autoimmune Low blood flow eg HF Chronic: hep C, autoimmune, haemochromatitis, alpha 1 antitrypsin deficiency, CF, biliary atresia, sclerosing cholangitis

Question 181

Common causes of UTI in children

Answer 181

E coli, Klebsiella, S. saprolyticus | Nb/ pseudomonas suggests structural abnormality

Question 182

Management of UTI in children

Answer 182

In under 3 months: IV cefuroxime for 7 days and USS renal tract In over 3 months: oral abx for 3/7 (trimethoprim, nitrofurantoin, cephalosporin, amoxicillin) If pyelonephritis: 7-10 days abx, ciprofloxacin or co-amoxiclav

Question 183

Presentation of pyelonephritis

Answer 183

At least 38 degrees and bacteriuria or Less than 38 degrees and loin pain/tenderness and bacteriuria

Question 184

When would you expect children to be dry overnight

Answer 184

Age 5

Question 185

Factors contributing to nocturnal enuresis

Answer 185

Small bladder, inability to recognise full bladder, hormones, UTI, sleep apnoea, diabetes, chronic constipation, structural problem in urinary tract or nervous system

Question 186

Management of nocturnal enuresis

Answer 186

<5= reassurance and advice Non drug treatment: fluid intake, diet, toilet behaviour, rewards system then enuresis alarm if not responding (still more than 1-2 bed wets in a week) Drug= desmopressin if over 5 +/- enuresis alarm Imipramine hydrochloride is last line

Question 187

Causes of acute renal failure

Answer 187

Hypoperfusion (blood loss, surgery, shock) Blockage of urinary tract Nephrotoxic drugs Haemolytic uraemic syndrome (e.coli usually) Glomerulonephritis

Question 188

Symptoms of acute renal failure

Answer 188

Haemorrhage, fever, rash, bloody diarrhoea, vomiting, anuria or polyuria, pallor, oedema, eye inflammation, stomach mass

Question 189

Treatment of acute renal failure?

Answer 189

``` Treat underlying cause U+Es Dietary changes Antihypertensives Iv fluids Diuretics ```

Question 190

How long for it to be classed as chronic renal failure? | Potential causes?

Answer 190

Over 3 months Causes: long term blockage, alport syndrome, nephrotic syndrome, PKD, cystinosis, chronic conditions eg diabetes, untreated acute kidney disease

Question 191

What is alport syndrome

Answer 191

Inherited condition- X-linked most common, but can be autosomal recessive/dominant Deafness, renal damage and eye defects

Question 192

Treatment of chronic renal failure

Answer 192

Diuretics Dialysis and transplant Diet changes (limit protein, potassium, phosphorus, sodium) Growth promoters/bisphosphonates/iron tablets

Question 193

What defines nephrotic syndrome

Answer 193

Generalised oedema, heavy proteinuria (>200mg/mmol) and hypoalbuminaemia (<25g/L) (Flattened podocytes allow protein leakage) (Normal protein less than 20mg/mmol, normal albumin 35-45g/L)

Question 194

Causes of nephrotic syndrome | Which is most common?

Answer 194

Minimal change disease=most common | Others= congenital nephrotic syndromes, focal segmental glomerulosclerosis, mesangiocapillary glomerulonephritis

Question 195

Management of nephrotic syndrome

Answer 195

High dose steroids- most respond well but some will have relapse (=steroid-resistant nephrotic syndrome)- in which case may need maintenance low dose steroids or immunomodulatory drugs eg cyclophosphamide Low salt diet, prophylactic abx (as they leak Igs and steroids!), immunisations Prednisolone for first episode 60mg/m2 for 4-6 weeks then wean down

Question 196

What is minimal change disease

Answer 196

Microscopically damage not evident. Idiopathic | Develop nephrotic syndrome more quickly but much more responsive to treatment

Question 197

What is congenital nephrotic syndrome | What is needed in management

Answer 197

Genetic condition (parents need 1 faulty and 1 healthy gene each to pass on) Requires frequent albumin infusions to aid growth and development Typically leads to renal failure

Question 198

Causes of nephritic syndrome

Answer 198

Post-streptococcal glomerulonephritis, henoch schnolein purpura, alport syndrome, sle/lupus nephritis

Question 199

Describe post-strep glomerulonephritis

Answer 199

10-14 days after skin/throat infection with group A strep Haematuria, oedema, decreased urine output, htn, proteinuria, tired Normally self limiting so needs supportive tx only If strep infection persisting= penicillin

Question 200

What is henoch schnolein purpura

Answer 200

IgA vasculitis | Rash of raised red or purple spots usually on legs and bottom (extensor surfaces)

Question 201

Px of henoch schonlein purpura

Answer 201

Rash, arthritis, abdo pain, kidney impairment (self limiting and mild usually), may get PR bleeding and intussusception Usually all preceded by 2-3 weeks of fever, headache, arthralgia/myalgia, abdo pain

Question 202

Mx of Alport syndrome

Answer 202

ACEi/ARB, diuretics, limit sodium | Monitor for potential renal failure

Question 203

Mx of Lupus nephritis

Answer 203

Immunosuppressants eg cyclophosphamide or hydroxychloroquine, antihypertensives, may need steroids

Question 204

Types of hypospadias | When does it form

Answer 204

Subcoronal (near head of penis) Midshaft Penoscrotal (where penis and scrotum meet) Weeks 8-14 of pregnancy

Question 205

Management of hypospadias

Answer 205

Surgery between 3 and 18 months, may need to be done in stages

Question 206

Types of renal malformation?

Answer 206

Autosomal recessive polycystic kidney disease Duplication (single renal unit with more than one collecting system) Fusion (kidneys joined, but ureters enter bladder on each side)- most common= horseshoe Malrotation (little clinical significance) Multicystic dysplastic kidney (nonfunctioning renal unit but contralateral kidney normally fine) Renal agenesis (bilat=fatal, unilateral) Renal ectopia=kidney fails to ascend from true pelvis Renal hypoplasia (underdeveloped)

Question 207

Pathophysiology of vescioureteral reflux

Answer 207

Urine flows from bladder back into ureters, potentially causing infection Shorter than normal attachment between ureter and bladder and incompetent valve

Question 208

Describe the different grades of vesicoureteral reflux

Answer 208

I=into ureter II=into ureter and renal pelvis without distension III=into ureter and renal pelvis with mild swelling IV= mod swelling V=severe swelling and twisting or ureter

Question 209

Management of vesicoureteral reflux

Answer 209

Can go away by itself (junction gets longer with age) Encourage frequent voiding May need surgery to stop reflux

Question 210

Triad of haemolytic uraemic syndrome

Answer 210

Coombs test negative, thrombocytopenia, AKI | Typically a/w e.coli

Question 211

What is haemolytic uraemic syndrome

Answer 211

Toxins bind from e.coli causing thrombin and fibrin to be deposited in microvasculature RBCs damaged while passing through occluded small vessels causing haemolysis

Question 212

Px haemolytic uraemic syndrome

Answer 212

Profuse diarrhoea turning bloody 1-3 days later, fever, abdo pain and vomiting

Question 213

Mx of HUS

Answer 213

Supportive- fluid and electrolyte mx, dialysis PRN, antihypertensive, keep circulating volume up

Question 214

Where abouts are babies usually affected with atopic dermatitis?

Answer 214

Face, neck, scalp, elbow and knees

Question 215

Where abouts are children usually affected with atopic dermatitis?

Answer 215

Inside elbows, back of knees, sides of neck, around mouth, wrists, ankles and hands

Question 216

General management of eczema?

Answer 216

Avoid triggers, keep nails short, emollients Rest of treatment depends on severity: may include corticosteroids, abx, antihistamines, calcineurin inhibitors, phototherapy, immunomodulators or biologic medications

Question 217

What steroid would you give for mild eczema?

Answer 217

Hydrocortisone 1%

Question 218

What steroid for moderate eczema?

Answer 218

Betamethasone valerate 0.025% or clobetasone butyrate 0.05%

Question 219

What steroid for severe eczema?

Answer 219

Betamethasone valerate 0.1% | May need maintenance steroids or topical calcineurin inhibitors eg tacrolimus

Question 220

What is Stevens-Johnson syndrome?

Answer 220

Toxic epidermal necrolysis. Rare, acute and potentially fatal skin reaction with sheet-like skin and mucosal loss Nearly always caused by medications

Question 221

What medications commonly cause Stevens-Johnson syndrome

Answer 221

Cotrimoxazole, penicillin, cephalosporins, anti-convulsants, allopurinol, paracetamol, NSAIDs Usually within the first week of abx therapy

Question 222

Presentation of Stevens-Johnson syndrome

Answer 222

Initially a flu-like illness Then, tender/painful red skin rash on trunk. extending to face and limbs over a course of hours-days (Skin lesions: macules/diffuse erythema/targetoid/blisters) The blisters merge to form sheets of skin detachment At least 2 mucosal surfaces affected eg conjunctivitis/mouth ulcers/cough/diarrhoea Acute phase lasts 8-12 days

Question 223

Management of Stevens Johnson syndrome

Answer 223

Skin biopsy shows keratinocyte necrosis SCORTEN illness severity score to predict mortality Cessation of suspected causative drug, admission, analgesia, temp maintenance, nutrition and fluids General care of affected mucosal surfaces

Question 224

Classification of allergic rhinitis?

Answer 224

Seasonal (if due to grass and tree pollens=hay fever) Perennial (throughout year, due to dust mites and animal dander) Intermittent (less than 4 days/week or less than 4 consecutive weeks) Persistent (more than 4 days/week or more than 4 consecutive weeks) Occupational

Question 225

Management allergic rhinitis? Mild-mod

Answer 225

Nasal irrigation with saline Allergen avoidance PRN antihistamine- intranasal as first line eg azelastine Can use 2nd generation non sedating oral eg loratidine or cetirizine for mild-mod symptoms

Question 226

What can be used in mod-severe allergic rhinitis?

Answer 226

Intranasal corticosteroid during periods of allergen exposure eg mometasone fluroate or fluticasone propionate

Question 227

For hayfever, what pollens affect you when?

Answer 227

Tree=early-late spring Grass=late spring-early summer Weed=early spring-late autumn

Question 228

What causes urticaria?

Answer 228

Histamine release | Due to: allergens, bee and wasp stings, autoimmune/idiopathic (chronic), physical triggers eg cold/friction, medications

Question 229

Types of antihistamine

Answer 229

Non sedating, sedating, short and long acting=H1 antihistamine H2 antihistamines

Question 230

What is angio-oedema?

Answer 230

Swelling deep to the skin, commonly soft areas of skin eg eyelids and lips, more painful than weals/hives and takes longer to clear.

Question 231

Presentation of anaphylaxis

Answer 231

Skin features-urticaria, erythema, flushing, angio-oedema | Involves at least 1 of Resp/CV/GI system

Question 232

How to manage anaphylaxis

Answer 232

ABCDE, high flow oxygen, IM adrenaline, repeat 3-5 mins if required, treat a drop in BP with fluid bolus, consider salbutamol if wheeze, antihistamines for itch(cetirizine) Prescribe an epipen

Question 233

What are the most common vascular birth marks?

Answer 233

Macular stains or salmon patches: Angel's kisses (forehead/nose/upper lip/eyelids- disappear with age) or Stork Bites (back of neck, disappear with age) Hemangioma (grow rapidly 6-9 months then shrink and lose red colour) Port-wine stain/nervus flammeus (grows with the child, often face/arms/legs, flat pink/red/purple mark)

Question 234

What are the most common pigmented birth marks?

Answer 234

Moles/congenital naevi, cafe au lait spots, mongolian spots (blue-grey spots on lower back or buttocks, common on darker skin)

Question 235

Causes of infectious rash in children

Answer 235

Meningococcal, steven-johnsons syndrome, impetigo, kawasaki, staphylococcal scalded skin syndrome, eczema herpeticum, erythema nodosum, erythema multiforme, measles, glandular fever, hand foot and mouth disease, erythema infectiosum, chicken pox, nappy rash, scabies, tinea corporis, tinea capitis, mollusucm contagiosum

Question 236

What are the three phases in Kawasaki disease?

Answer 236

Acute phase=weeks 1-2 Subacute phase= weeks 2-4 Convalescent phase=weeks 4-6

Question 237

Presentation of kawaski disease in acute phase?

Answer 237

Fever for at least 5 days (over 38), rash, red or hard fingers or toes, hand/foot swelling, conjunctival infection, dry/cracked lips, strawberry tongue, swollen lymph nodes

Question 238

What happens in subacute phase of kawasaki disease?

Answer 238

Symptoms less severe. Fever subsides, may have diarrhoea, vomiting, abdo pain, pyuria, lethargy, headache, malaise, jaundice and peeling skin

Question 239

What happens in the convalescent phase of kawasaki disease?

Answer 239

Begin to recover, symptoms start to go away, residual lethargy.

Question 240

Pathophysiology of kawasaki disease?

Answer 240

Idiopathic. Not contagious. Also known as mucocutaneous lymph node syndrome. Mainly affects under 5s.

Question 241

What are NICE criteria for diagnosis of kawasaki?

Answer 241

Over 38 degrees for at least 5 days and at least 4 key symptoms: Bilateral conjunctival infection, mouth/throat changes, hand and feet changes, rash, swollen glands

Question 242

How to manage kawasaki disease?

Answer 242

High dose aspirin initially then move onto low dose IVIg-Gamma globulin (after which symptoms will improve after 36 hours) If IVIg failure, consider steroids

Question 243

What should you be aware of when giving children aspirin?

Answer 243

Reyes syndrome: persistent vomiting, lethargy, liver and brain damage

Question 244

Presentation of measles

Answer 244

Initially coryzal sxs, fever, small grey-white spots in mouth (Koplik spots) lasting a few days 2-4 days later= maculopapular rash on head/neck and spreads to rest of body

Question 245

Management of measles

Answer 245

Self limiting viral illness and usually clears in about 7-10 days Mx: stay at home for at least 4 days from when rash first appears, antipyretic, fluids, gently clear crust from eyes, hot steam PREVENTION: MMR vaccination at 12-13 months and 3 years 4 months

Question 246

What are some complications of measles?

Answer 246

``` Common= dehydration, otitis media, conjunctivitis, laryngitis, airway infections, febrile seizures Uncommon= hepatitis, squint, meningitis encephalitis Rare= optic neuritis, heart and nerve problems, subacute sclerosing panencephalitis several years after ```

Question 247

What causes chicken pox?

Answer 247

Varicella zoster virus- commonly before age 10

Question 248

Presentation of chicken pox?

Answer 248

Fever aches and headache for about a day before the rash: spots in crops become small itchy blisters and then scab over May lose appetite and have feeding problems

Question 249

Management of chickenpox?

Answer 249

``` AVOID aspirin (reye's syndrome) Antipyretic, emollient, sedating antihistamine at night, keep nails short, dress comfortably, fluids Stay off school until all blisters crusted over ``` If an at risk group eg under 1 month, immunocompromised, severe heart/lung disease=aciclovir or vaccination

Question 250

When are you infectious with chickenpox?

Answer 250

7-21 day incubation | Infectious from 2 days before spots appear until all blisters crusted over

Question 251

What type of infection is rubella? When is peak incidence?

Answer 251

Viral. Mainly in spring and early summer

Question 252

Presentation of rubella?

Answer 252

Prodromal phase: low grade fever, headache, mild conjunctivitis, anorexia, rhinorrhoea Rash occurs 14-17 days after exposure: pink discrete macules that coalesce behind ears and on face, spreads to trunk then extremities Lymphadenopathy-cervical, suboccipital, post auricular

Question 253

Management of rubella?

Answer 253

PCR is gold standard diagnosis Keep out of school for 4 days after rash appears, antipyretics, ask re contact with pregnant women PREVENTION: MMR vaccination at 12-13 months and 3 years 4 months

Question 254

What causes diphtheria?

Answer 254

Gram positive aerobic bacillus Corynebacterium diphtheriae causing acute upper resp tract infection

Question 255

Presentation of diphtheria?

Answer 255

Early sxs=coryzal Nasal discharge- watery then purulent and bloody Membranous pharyngitis with fever, enlarged cervical LNs, oedema of soft tissues= bull neck appearance Cutaneous infection: pustules rupture causing punched out ulcers often on lower legs, feet and hands, with surrounding oedematous pink/purple skin. Takes 2-3 months to heal leaving a depressed scar May cause difficulty swallowing, paralysis and HF

Question 256

Management of diphtheria?

Answer 256

PREVENTION: 6-in-1 vaccination at 2, 3 and 4 months Antibiotics (eyrtho/azithro/clarithro/penicillin), reinforcing vaccine dose, manage contacts with abx prophylaxis Booster vaccinations: first between 3.5 and 5 years, second between 13-18

Question 257

Pathophysiology of scalded skin syndrome?

Answer 257

Staphylococcal disease. Also known as Ritter disease and Lyell disease Affects under 5s, especially neonates normally Exotoxins from S. aureus bind to desmosomes, causing skin cells to become unstuck- causes red blistering skin

Question 258

Presentation of scalded skin syndrome?

Answer 258

Fever irritability and widespread erythema, then 24-48 hours later=fluid filled blisters form and rupture easily Nikolsky sign positive= gentle strokes causes exfoliation

Question 259

Investigations for scalded skin syndrome?

Answer 259

Tzanck smear, skin biopsy, bacterial culture

Question 260

Management of scalded skin syndrome?

Answer 260

Admit to hospital IV antibiotics eg flucloxacillin Supportive -antipyretic, fluids, petroleum jelly Healing usually complete within 5-7 days of starting treatment

Question 261

Pathophysiology of whooping cough?

Answer 261

Bordetella pertussis bacterial infection | Gram negative bacillus. Toxins paralyse cilia and promote inflammation

Question 262

Describe the first phase of whooping cough?

Answer 262

1st phase=catarrhal phase for 1-2 weeks | Coryzal sxs: rhinitis, conjunctivitis, irritability, sore throat, low grade fever, dry cough

Question 263

Describe the second phase of whooping cough?

Answer 263

2nd phase=paroxysmal for 2-8 weeks Severe paroxysms of coughing followed by inspiratory gasp (whoop) NB/ in infants under 3 months the whoop is less common and apnoea is more common. Paroxysms more common at night and often followed by vomiting

Question 264

Describe the third phase of whooping cough?

Answer 264

Convalescent phase=up to 3 months

Question 265

Investigations for whooping cough?

Answer 265

If cough for less than 2 weeks: nasopharyngeal aspiratr If cough for more than 2 weeks: anti pertussis toxin IgG serology in under 5s or toxin detection in oral fluid for 5-17year olds FBC=lymphocytosis

Question 266

Management of whooping cough?

Answer 266

Hospital if under 6 months and acutely unwell, sig breathing difficulties, feeding difficulties, complications eg pneumonia Macrolide antibiotic if cough for less than 21 days= clarithromycin (under 1 month), azithromycin otherwise Supportive NB/ abx don't alter course once disease established PREVENTION= vaccination at 2,3,4 months and booster at 3 years 4 months

Question 267

Presentation of polio?

Answer 267

Fever, tiredness, headache, vomiting, neck stiffness and arm/leg pain In 1 in 200 cases, affects NS leading to temporary or permanent paralysis

Question 268

Management of polio?

Answer 268

No treatment; need physio and OT PREVENTION= vaccination at 2,3,4 months, 3years 4 months and 13-18 years

Question 269

How to diagnose TB in children?

Answer 269

Mantoux test= positive tuberculin skin test IGRA CXR History of contact Lab cultures very difficult to do in kids

Question 270

TB drugs?

Answer 270

RIPE | Rifampicin, isoniazid, pyrazinamide, ethambutol

Question 271

Who gets BCG vaccination?

Answer 271

If born in areas of UK where rates are high | If a parent/grandparent born in a country with high TB rates

Question 272

Causes of meningitis in children?

Answer 272

Bacterial: Mainly N. meningitidis and S. pneumoniae In neonates= GBS (group B strep from mum's vagina) Viral: HSV, enterovirus and VZV

Question 273

What is the presentation of meningitis in neonates and babies?

Answer 273

Non-specific! | Eg Hypotonia, poor feeding, lethargy, hypothermia, bulging fontanelle

Question 274

How to investigate for meningitis?

Answer 274

Kernigs and brudzinski's signs LP if under 1 month and fever, 1-3 months fever and unwell, under 1 year with unexplained fever and other features of serious illness

Question 275

Management of meningitis?

Answer 275

Primary care: benzylpenicillin Secondary care bacterial: If under 3 months: cefotaxime and amoxicillin (to cover listeria) Over 3 months=ceftriaxone Dexamethasone QDS for 4/7 if LP suggestive bacterial meningitis PEP for contacts: ciprofloxacin Viral=aciclovir

Question 276

What CSF findings are suggestive of bacterial meningitis?

Answer 276

Cloudy, high protein, low glucose, high neutrophils, cultured for bacteria

Question 277

What CSF findings are suggestive of viral menigitis?

Answer 277

Clear, normal protein, normal glucose, lymphocytosis, negative culture

Question 278

Causes of encephalitis?

Answer 278

Most common=HSV 1(children) and HSV 2(neonates- from genital warts) Others= VZV, CMV, EBV, enterovirus, adenovirus, influenza virus

Question 279

Presentation of encephalitis?

Answer 279

Altered consciousness, altered cognition, unusual behaviour, acute onset focal seizures and focal neurological symptoms, fever

Question 280

Management of suspected encephalitis?

Answer 280

Ix: LP (CSF and PCR) or CT if LP CI. MRI after LP, swabs, HIV testing Mx: aciclovir for HSV/VZV or ganciclovir (CMV) Repeat LP to ensure successful tx

Question 281

How does HSV1 affect children?

Answer 281

Infectious for 7-12 days Commonly gingivostomatitis: fever, restless, dribbling, swollen gums which bleed easily, foul breath, ulcers on tongue/throat/palate/inside cheeks, enlarged LNs

Question 282

Management of HSV?

Answer 282

Can use antivirals eg aciclovir/valaciclovir but for mild/uncomplicated eruptions require no treatment

Question 283

What is slapped cheek syndrome caused by?

Answer 283

Also known as fifth disease/erythema infectiosum Parvovirus B19

Question 284

When are the peak incidences for slapped cheek syndrome?

Answer 284

april and may

Question 285

Presentation of slapped cheek syndrome?

Answer 285

Commonly 3-15 years old Rash=bright red scald-like on 1 or both cheeks, painless May have mild coryzal symptoms 1 in 4 will be asx By time rash develops, no longer infectious

Question 286

Management of slapped cheek syndrome?

Answer 286

None needed. Caution around pregnant women

Question 287

Types of impetigo and what caused by?

Answer 287

Non-bullous (about 70% cases)= S.aureus, S.pyogenes or both | Bullous= S.aureus

Question 288

Presentation of impetigo?

Answer 288

Non-bullous= golden/brown crusts from vesicles/pustules, commonly on face, limbs and flexures, may be mildly itchy Bullous-more common in infants= blisters rupture leaving flat yellow/brown crust, may have systemic features

Question 289

Management of impetigo?

Answer 289

Good hygiene, stay away from school until healed or 48 hours after abx Localised non-bullous=hydrogen peroxide 1% cream (2nd line=fusidic acid 2%) Widespread non-bullous or bullous=oral flucloxacillin

Question 290

How to manage candida?

Answer 290

topical antifungal eg miconazole gel If vaginal (v. uncommon before puberty)= fluconazole

Question 291

Causes of toxic shock syndrome?

Answer 291

Staph or strep- from female barrier contraceptives, tampons, injury on skin, childbirth, nasal packing, ongoing staph or strep infection

Question 292

Presentation of toxic shock syndrome?

Answer 292

Fever, flu-like sxs, N+V, diarrhoea, widespread sunburn like rash. lips/tongue/whites of eye turning bright red, dizziness and syncope, dyspnoea, confusion

Question 293

Management of toxic shock syndrome?

Answer 293

Antibiotics and fluids

Question 294

What causes scarlet fever?

Answer 294

Group A strep- affects people with recent strep throat or impetigo. Children over 3 at risk

Question 295

Symptoms of scarlet fever?

Answer 295

Sudden fever a/w sore throat, lymphadenopathy, headache, N+V, loss of appetite, swollen and red strawberry tongue, abdo pain, malaise 12-48 hours after fever=rash: starts below ears/neck/chest/armpit/groin)- scarlet blotches (boiled lobster appearance). Then looks like sunburn with goose pimples, pastia lines (red streaks from ruptured capillaries)

Question 296

Management of scarlet fever?

Answer 296

Penicillin | Mostly follows benign course

Question 297

What is Coxsackie's disease?

Answer 297

Hand foot and mouth disease, generally affects under 10s. | Group A coxsackie virus- faecal-oral transmission

Question 298

Presentation of coxsackie's disease?

Answer 298

Prodromal phase then tender oral ulcerative lesions and maculopapular lesions on hands and feet

Question 299

Management of coxsackie's disease?

Answer 299

Supportive mx-fluids, soft diet, antipyretic analgesics, don't need to isolate the child Symptoms improve within 3-6 days with full resolution within 7-10 days

Question 300

Red flags for primary immunodeficiency in kids?

Answer 300

faltering growth, need IV abx for infections, FH, at least 4 infections a year, at least 2 sinus infections a year, at least 2 pneumonias in past 3 years, frequent deep tissue abscesses, persistent fungal infections (over 6 months), at least 2 deep seated infections over 3 years, at least 2 months on at least 2 abx with little effect

Question 301

What makes you think about developmental delay in children?

Answer 301

Global delay= sig delays in at least 2 areas: motor function, speech, language, cognitive, play, social skills Generally only noticeable once at school age

Question 302

Potential causes of developmental delay?

Answer 302

``` Genetic/hereditary eg Down syndrome Metabolic eg PKU Trauma eg shaken baby syndrome Psychosocial eg PTSD Toxic eg prenatal alcohol exposure or lead poisoning Deprivation of food or environment Infections May be idiopathic ```

Question 303

What are febrile convulsions?

Answer 303

Commonly in 6months-3 years, when a child has a fever. Usually benign NB/ 1 in 3 will have another seizure during subsequent infection

Question 304

Px of febrile convulsion?

Answer 304

Last less than 5 mins, child becomes stiff, then limbs twitch, lose consciousness and may wet/soil themselves May be sick, foam at mouth and eyes may roll back Sleepy for up to an hour after event

Question 305

What warrants a complex febrile seizure?

Answer 305

Lasting over 15 mins, may only affect one side of body

Question 306

Types of epilepsy in childhood?

Answer 306

Absence seizures Childhood epilepsy syndrome ('benign' if can predict from EEG that will stop by certain age) Infantile spasms=West syndrome Bects: Rolandic epilepsy (benign epilepsy with centro-temporal spikes) Juvenile myoclonic epilepsy

Question 307

What warrants a floppy infant?

Answer 307

Hypotonia, weakness, ligamentous laxity, increased range of joint mobility

Question 308

What features suggest central hypotonia/UMN in children?

Answer 308

Normal strength, dysmorphic features, normal/brisk tendon reflexes, irritability +/- loud cry, history suggestive of HIE (hypoxic-ischaemic encephalopathy)/birth trauma/sxs hypoglycaemia,seizures Central=2/3 cases, commonly HIE

Question 309

What are indicators of peripheral hypotonia/LMN in children?

Answer 309

Decreased strength, reduced/absent reflexes, fasciculation, myopathic face, weak cry

Question 310

Causes of floppy infant?

Answer 310

Central hypotonia=acute encephalopathies (HIE, hypoglycaemia, intracranial haemorrhage), chronic encephalopathies (cerebral malformations, metabolism errors, chromosomal disorders, endocrine disorders, metabolic disorders), connective tissue disorders (Ehler-Dahlos, OI) Peripheral hypotonia= spinal cord (syringomyelia), anterior horn cell (spinal muscular atrophy), NMJ (MG), muscular disorders (dystrophies, myopathies), peripheral nerves, metabolic myopathies

Question 311

What is cerebral palsy?

Answer 311

Non progressive interference/lesion/abnormality leading to a disorder of movement and/or posture and of motor function Damage to immature brain (most between 24 weeks and term)

Question 312

Classification of cerebral palsy?

Answer 312

Spastic, athetoid (hyperkinesia, 'stormy movement'), ataxic, mixed

Question 313

Presentation of cerebral palsy?

Answer 313

Motor features- mono/hemi/para/quadriplegia Don't meet developmental milestones eg not sitting by 8 months, not walking by 18 months, early hand preference before 1 year GORD, epilepsy, vomiting, constipation, bladder issues, drooling, orthopaedic problems all common

Question 314

How to diagnose cerebral palsy?

Answer 314

Definitive dx may not come until 12-18 months | Clinical observation and parental observation

Question 315

Fits, faints and funny turns causes?

Answer 315

In sleep: benign neonatal sleep myoclonus and parasomnias On feeding: GORD and sandifer syndrome Fever: febrile seziures, vaso-vagal syncope Pain/shock/startle: reflex asystolic syncope, cyanotic breath holding, hyperekplexia Tired/bored/stress: self gratification behaviour, tics, daydreaming Excitement: shuddering spells, cataplexy

Question 316

Potential childhood motor disease?

Answer 316

Tourette syndrome and tics, tremor, dystonia, ataxia, restless legs syndrome, myoclonus, juvenile huntington disease

Question 317

DSM-V criteria for ADHD

Answer 317

Symptoms must be present before age 12, and present in at least 2 settings, affect functioning. At least 6 symptoms of inattention for at least 6 months or At least 6 symptoms of hyperactivity for at least 6 months

Question 318

Non drug management of ADHD

Answer 318

parent education and training CBT can be offered Meds not advised for pre-school children Advice: plan day, clear boundaries, brief and specific instructions, incentive schemes, kep social situations short and sweet, exercise, healthy diet, bedtime routine, help at school

Question 319

Medical management of ADHD

Answer 319

Stimulant drugs (increase dopamine): Short acting methylphenidate eg ritalin Long acting methylphenidate eg delmosart Lisdexamfetamine Non stimulant drugs (reduced breakdown of noradrenaline): Atomoxetine (SNRI), guanfacine

Question 320

Describe autism spectrum disorders

Answer 320

Abnormal development from under 3 years old Abnormal functioning in reciprocal social interaction, communication and restricted, stereotyped and repetitive behaviour Diagnosis requires at least 6 symptoms across 3 core areas

Question 321

Management of autism spectrum disorders

Answer 321

Non-pharm management behavioural therapies, social skills groups, OT, communication interventions, input from dietician if needed, specialised educational programs and structured support

Question 322

ICD-10 for anorexia nervosa

Answer 322

``` Deliberately keeping weight below 85% of expected via restricted diet choice, excessive exercise, induced vomiting, use of appetite suppressants and diuretics Dread of fatness-intrusive overvalued idea Endocrine effects (menstruation stops/puberty delayed if menarche not yet achieved, loss of sexual interest in men) ```

Question 323

How to screen for eating disorders?

Answer 323

SCOFF Do you make yourself sick because you're uncomfortably full? Do you worry that you've lost control over how much you eat? Have you recently lost more than one stone (about 6kg) in 3 months? Do you believe you are fat when others say you are thin? Would you say that food dominates your life?

Question 324

Potential clinical signs of anorexia nervosa?

Answer 324

Dry skin, lanugo hair, orange skin and palms, cold hands and feet, bradycardia, drop in BP on standing, oedema, weak proximal muscles (squat test)

Question 325

Management of anorexia nervosa?

Answer 325

Anorexia nervosa focused family therapy

Question 326

Define bulimia nervosa

Answer 326

Binges followed by compensatory weight loss behaviours eg self induced vomiting, fasting, intensive exercise, abuse of medication BMI maintained at over 17.5

Question 327

Presentation of diabetes in children?

Answer 327

Tiredness, toilet, thirsty, thinner

Question 328

Diagnosis of diabetes in children?

Answer 328

Random plasma glucose > 11.1 mmol/L if symptomatic

Question 329

Normal fasting blood glucose? post prandial glucose? ketones?

Answer 329

Fasting: 3.5-5.6mmol/L Post prandial: <7.8 Ketones: <0.6

Question 330

DKA management?

Answer 330

Fluid, insulin, monitor glucose hourly, monitor electrolytes especially potassium and ketones 2-4 hourly, hourly neuro obs

Question 331

Fluids in DKA?

Answer 331

0.9% NaCl 20ml/kg bolus if shocked, 10ml/kg if not shocked Maintenance and deficit fluids 5% fluid deficit in mild DKA 7% deficit in moderate 10% deficit in severe Do no subtract boluses given for shock

Question 332

Initial management of type I diabetes mellitus in kids?

Answer 332

Aim for glucose 4-7: need to be able to check this Give insulin (pump or injection): basal bolus regime most common Carb counting Advice for hypos Lifestyle advice

Question 333

Carb counting in DMT1?

Answer 333

Insulin:carb ratio= 1 unit for every 15 grams of carb Correction factor= 1 unit insulin brings blood glucose down by 8 Aim for glucose of 6

Question 334

Presentation of hypoglycaemia?

Answer 334

Autonomic fxs: irritable, hungry, nauseous, shaky, anxious, sweaty Neuroglycopenic fxs: confused, drowsy, headache, coma, seizures

Question 335

Management of hypoglycaemia?

Answer 335

Check levels to confirm Glucose tablets/gel/containing food or drink first Check glucose in 15 mins Follow up with longer acting carb (bread or biscuit) Severe hypo (requiring additional assistance from another person)= IM glucagon

Question 336

Long term management of diabetes?

Answer 336

Aim for HbA1c less than 48mmol/mol | Monitoring at least annually: eyes, urine, feet, BP, injection sites, bloods

Question 337

Types of cryptorchidism?

Answer 337

True undescended= lies along normal path of descent but never present in scrotum Ectopic testis= lies outside normal path and outside of scrotum Ascending testis=previously in scrotum but higher now Absent/atrophic testis=non palpable testis was in scrotum at birth but later disappears

Question 338

How to manage ?cryptorchidism?

Answer 338

6-8 week baby check exam If not descended, re examine at 4-5 months to see if spontaneous descent If not surgical treatment (orchodexy) before 12 months of age

Question 339

Pathophysiology of testicular torsion?

Answer 339

Testis twists around spermatic cord and cuts off the blood supply to the testicle

Question 340

Presentation of testicular torsion?

Answer 340

Sudden severe pain on one side of the scrotum, scrotal skin swells and turns red Nausea and vomiting potential May have just abdominal pain

Question 341

Management of testicular torsion?

Answer 341

Surgical emergency: blood supply must be restored within 6 hours or the testicle will infarct If surgery delayed past 12 hours, 75% will need orchidectomy Newborns with torsion- testes almost always infarcted so need orchidectomy

Question 342

What is a bell clapper deformity?

Answer 342

No tissue holding the testes to the scrotum so testes swing inside the scrotum= higher risk of torsion

Question 343

Cut offs for precocious puberty?

Answer 343

Girls under 8 | Boys under 9

Question 344

2 types of precocious puberty?

Answer 344

Gonadotrophin dependent/ central precocious puberty= true precocious puberty with premature activation of the HPG axis Gonadotrophin independent/precocious pseudopuberty= due to increased production of sex hormones

Question 345

Causes of central precocious puberty?

Answer 345

Mainly idiopathic or abnormalities of CNS

Question 346

Causes of precocious pseudopuberty?

Answer 346

CAH, tumours (HCG secreting), McCune- Albright syndrome, silver-russell syndrome, testotoxicosis, exogenous androgen exposure, severe hypothyroidism

Question 347

What can you give for true precocious puberty?

Answer 347

GnRH agonists

Question 348

Define delayed puberty

Answer 348

``` Girls= absence of breast development by 13 or primary amenorrhoea with normal breast development by 15 Boys= absence of testicular development by age 14 ```

Question 349

Causes of delayed puberty

Answer 349

Most common= constitutional delay in growth and puberty (CDGP) Central causes= CDGP, chronic illness, malnutrition, excessive exercise, psychosocial deprivation, steroids, hypothyroid, tumours, congential anomalies, irradiation treatment, trauma, idiopathic hypogonadotrophic hypogonadism Peripheral causes= bilat testicular damage, syndromes eg Prader-Willi, Klinefelter, rugs. irradation, Turner syndrome, intersex disorders, PCOS

Question 350

How to manage delayed puberty?

Answer 350

Most are CDGP so need reassurance and monitoring | Can start course of sex hormones (low dose testosterone or gradual increase of oestrogen then cyclical progestogen)

Question 351

Causes of congenital hypothyroidism

Answer 351

Thyroid dysgenesis, thyroid hormone metabolism disorders, hypothalamic/pituitary dysfunction,

Question 352

What happens in congenital hypothyroidism isn't treated?

Answer 352

cretinism: long term irreversible neurological problems and poor growth with intellectual disability spasticity, gait problems, dysarthria, profound mental disability

Question 353

How to treat congenital hypothyroidism?

Answer 353

levothyroxine titrated to TFTs

Question 354

Define gonadotrophin deficiency/hypogonadotrophinism

Answer 354

Low serum concentrations of LH and FSH in presence of low circulating concentrations of sex steroids

Question 355

How to manage gonadotrophin deficiency?

Answer 355

Induce and maintain secondary sexual characteristics- testosterone and hCG or oestrogen and progestogen

Question 356

What is Kallmann syndrome?

Answer 356

Hypothalamic gonadotrophin releasing hormone deficiency (GnRH) and deficient olfactory sense (hyposmia or anosmia) X linked recessive or autosomal recessive Greater penetrance in males

Question 357

Presentation of Kallmann syndrome?

Answer 357

``` Females= primary amenorrhoea Males= cryptorchidism, micropenis ``` Most are normal stature Lots have associated stigmata- nerve deafness, colour blind, cleft palate, renal abnormalities

Question 358

How to manage Kallmann syndrome?

Answer 358

Exogenous sex steroids to induce and maintain secondary sexual characteristics

Question 359

What is congenital adrenal hyperplasia?

Answer 359

Group of autosomal recessive disorders of cortisol biosynthesis+/- aldosterone +androgen excess 21-hydroxylase deficiency

Question 360

What is the classic form of CAH?

Answer 360

Ambiguous external genitalia in girls | Boys have no signs at birth

Question 361

What is the salt-losing form of CAH?

Answer 361

Boys present at 7-14 days with vomiting weight loss, lethargy, hyponatraemia, hyperkalaemia and dehydration Girls will be identified before a crisis due to genitalia

Question 362

How to manage classic CAH?

Answer 362

Glucocorticoid replacement= hydrocortisone +/- Mineralcorticoids= fludrocortisone Salt losing form need NaCl Consider surgery for genitalia

Question 363

Types of androgen insensitivity syndrome? describe

Answer 363

Complete=testosterone has no effect so genitals are entirely female Partial= testosterone has some effect so genitals not as expected eg penis underdeveloped, fully/partial cryptorchidism, some female genitalia.

Question 364

How will females with complete androgen insensitivity syndrome present? and partial?

Answer 364

Complete=Develop breasts but amenorrhoea, no pubic or axillary hair, no womb or ovaries Partial=enlarged clitoris, undescended testicles, hypospadias, often raised as boys

Question 365

Causes of childhood obesity?

Answer 365

Diet, exercise lack, sleep deprivation (low leptin and high ghrelin), genetics, socio-economics, medication, endocrine eg PCOS/hypothyroid/Cushings/Prader-Willi

Question 366

Causes of microcytic anaemia?

Answer 366

Low MCV: iron deficiency, thalassaemia, sideroblastic anaemia, lead toxicity, inflammation, haemoglobinopathy

Question 367

Presentation of anaemia?

Answer 367

Pallor, pale conjuctivae, tachycardia, murmur, irritability, lethargy, poor growth, weakness, sob, signs of haemolysis eg splenomegaly

Question 368

Risk factors for iron deficiency anaemia in infants and children?

Answer 368

Infants= maternal iron deficiency, premature, low birth weight, multiple pregnancy, exclusively breastfed after 6 months, excess cow's milk consumption, aboriginal Children=veggie/vegan, GI disorders, chronic blood loss, heavy menstrual bleeding, extreme athletes

Question 369

Why are kids more susceptible to iron deficiency anaemia than adults?

Answer 369

30% of their iron comes from diet and 70% from recycled rbcs (whereas adults have 5% from diet and 95% from recycling) Have higher expenditure

Question 370

Management of iron deficiency anaemia?

Answer 370

Diet advice and iron supplementation (generally oral)

Question 371

Which type of thalassaemia is generally incompatible with life?

Answer 371

alpha major (4/4 genes missing to make alpha chain)

Question 372

What may carriers of thalassaemia have?

Answer 372

Mild microcytic hypochromic anaemia

Question 373

Presentation of beta thalassaemia major?

Answer 373

Progressive severe anaemia if left untreated | Try to compensate with bone marrow expansion (deformity) and extramedullary haematopoiesis

Question 374

How to manage beta thalassaemia major?

Answer 374

Genetic counselling and antenatal diagnosis Regular transfusion with iron chelation therapy to prevent iron overload May need bone marrow transplantation

Question 375

What is haemolytic disease of the newborn?

Answer 375

transplacental maternal antibodies causing alloimmune haemolysis of foetal rbcs Most commonly due to rhesus alloimmunisation (Rh + rbcs from foetus enter rh - maternal blood circulation)

Question 376

Why does haemolytic disease of newborn occur then and not in utero?

Answer 376

In utero, bilirubin cleared by placenta | In neonate, liver does clearing and can't handle high bilirubin load

Question 377

Presentation of haemolytic disease of the newborn?

Answer 377

Jaundice Pallor Hepatosplenomegaly Severe= oedema, ascites, petechiae

Question 378

What anaemia will haemolytic disease of newborn present as?

Answer 378

Normocytic. Increased reticulocyte count

Question 379

Management of haemolytic disease of newborn?

Answer 379

``` 50%= normal Hb and bilirubin and need monitoring for late onset anaemia at 6-8 weeks 25%= mod disease and may require transfusion 25%= severe disease= stillborn or have hydrops fetalis- require immediate resus, temp stabilisation, exchange transfusion. ```

Question 380

Pathophysiology of sickle cell disease?

Answer 380

Autosomal recessive condition Adenine to thymine base change causes glutamic acid to become valine HbS Sickling causes deformed and easily destroyed rbcs= occlusion of microcirculation and chronic haemolytic anaemia

Question 381

When do symptoms of sickle cell disease start to present? WHy?

Answer 381

Between 3 and 6 months as HbF levels fall

Question 382

Complications of sickle cell disease?

Answer 382

Life expectancy 20 years younger | Stroke, aplastic crisis, painful crises, splenic sequestration, acute chest syndrome

Question 383

How to manage sickle cell disease?

Answer 383

Most complications require urgent blood transfusions Long term: hydroxycarbamide, prevent infection (influenza, pneumococcal and meningococcal vaccines, penicillin), transfusion programmes, stem cell transplants, gene therapies, prevent crises (eg dehydration precipitates sickling)

Question 384

How does hydroxycarbamide help in sickle cell disease?

Answer 384

Increases foetal haemoglobin production

Question 385

What is fanconi anaemia?

Answer 385

X-linked/autosomal recessive condition | Bone marrow failure, solid tumours, leukaemia

Question 386

How to manage fanconi anaemia?

Answer 386

Treat specific symptoms in each patient Only curative= stem cell transplant Cancer treatment Surgery for skeletal malformations

Question 387

what factor is deficient in haemophilia a

Answer 387

clotting factor VIII (classic haemophilia, most common)

Question 388

what factor is deficient in haemophilia b

Answer 388

clotting factor IX (also known as christmas disease)

Question 389

how does severe haemophilia present?

Answer 389

Increased APTT | Prolonged bleeding, muscle and joint bleeds

Question 390

how does mild/mod haemophilia present?

Answer 390

Delayed presentation until following trauma or bleeds with surgery/dental extractions

Question 391

Management of haemohpilia?

Answer 391

Factor VIII for haemophilia a | Factor IX for haemophilia b

Question 392

How does von willebrand factor work?

Answer 392

Assists in platelet plug formation by attracting circulating platelets and binds to CF VIII, preventing its clearance from the plasma

Question 393

Presentation of von willebrand disease?

Answer 393

NB more severe in patients with blood group O Bleeding tendency from mucosa eg epistaxis/menorrhagia, spontaneous bleeding

Question 394

Describe the types of von willebrand disease?

Answer 394

Type 1=quantitative- mild Type 2= qualitative- variable Type 3=quantitative- very low undetectable levels, most severe form, has low factor VIII

Question 395

How to manage von willebrand disease?

Answer 395

tranexamic acid and desmopressin or concetrates with vwf or factor VIII-vwf Testing for 1st degree relatives

Question 396

When does immune thrombocytopenia occur?

Answer 396

Commonly after viral infection or sometimes after immunisation

Question 397

Presentation of ITP?

Answer 397

Usually self limiting and recovers in 6-8 weeks Some asx Most- petechiae or bruising, epistaxis Less common= GI bleeds, haematuria, menorrhagia

Question 398

Management of ITP?

Answer 398

For most- not treatment, avoids NSAIDs and aspirin, advice and support If bleeding actively may need prednisolone, IVIg and Iv anti-D Ig in rhesus positive babies

Question 399

Commonest cancer in children?

Answer 399

Leukaemia- ALL and then AML

Question 400

Pathophysiology of acute lymphoblastic leukaemia?

Answer 400

Disruption of lymphoid precursor cells in the bone marrow leading to excessive production of immature blast cells: causes drop in functional RBCs/WBCs/platelets

Question 401

Presentation of leukaemia in children?

Answer 401

``` Anaemia (lethargy, pallor) Thrombocytopenia (easy bruising/bleeding) Leukopenia (fevers, infections) May complain of bone pain Non specific sxs (weight loss, malaise) ```

Question 402

Investigations for leukaemia?

Answer 402

``` FBC (pancytopenia, thrombocytopenia with significant lymphocytosis), serum biochemistry Blood films (blast cells) CXR (exclude mediastinal mass) Bone marrow aspirate confirms diagnosis LP ```

Question 403

Management of leukaemia in children?

Answer 403

Chemo, supportive care with blood products, prophylactic anti-fungals Chemo in 5 phases (induction, consolidation, interim maintenance, delayed intensification, maintenance) Maintenance= 2 years for girls and 3 years for boys Stem cell transplants in high risk patients in 1st remission or relapsed patients

Question 404

Name 5 different types of primary brain tumour?

Answer 404

Astrocytoma, medulloblastoma, diffuse midline glioma, ependymoma, craniopharyngioma, embryonal tumours, pineoblastoma, brainstem glioma, choroid plexus carcinoma, germ cell tumour

Question 405

What is the most common brain and spinal cord tumour in children (apart from mets)?

Answer 405

Astrocytoma

Question 406

Presentation of brain tumour?

Answer 406

morning headaches, nausea/vomiting, diplopia, seizure, increased head circumference, bulging fontanelle, signs of raised intracranial pressure

Question 407

4 main types of astrocytoma?

Answer 407

Grade 1=pilocytic astrocytoma Grade 2=diffuse (fibrillary) astrocytoma Grade 3=anaplastic astrocytoma Grade 4=glioblastoma multiforme 80% are low grade (1 or 2)

Question 408

What is the most common high grade brain tumour in children?

Answer 408

Medulloblastoma (2nd most common overall) | Most commonly found in cerebellum

Question 409

What is Wilm's tumour? How presents?

Answer 409

Most common paediatric renal mass= nephroblastoma Painless upper quadrant abdominal mass, haematuria in 20%, hypertension in 25%, ,may have acquired von willebrand disease

Question 410

how to manage wilm's tumour?

Answer 410

Nephrectomy and chemo | Cure in about 90% cases

Question 411

How does neuroblastoma present?

Answer 411

Presents late- majority of symptoms due to mass effect or mets. Systemic fxs, bruising, weakness/paralysis/BB dysfunction, bone pain, abdominal distension, htn, horner's syndrome Almost all are stage 3 or 4 at diagnosis

Question 412

Where does neuroblastoma arise from?

Answer 412

Adrenal or paraspinal sites most common | arises from developing sympathetic nervous system

Question 413

Investigating neuroblastoma?

Answer 413

FBC, esr, pt, catecholamine by products in urine, CT, MRI for paraspinal lesions, biopsy

Question 414

Presentation of retinoblastoma?

Answer 414

Leukocoria (abnormal appearance of pupil), visual deterioration, red and irritated eye, faltering growth, squint, absent red reflex

Question 415

Management of retinoblastoma?

Answer 415

Eye conserving treatments eg cryo/thermotherapy, photocoagulation, chemo Enucleation for all unilateral tumours filling over half of the eye or invading eye structures and subsequent fitting of prosthesis V sensitive to radiotherapy

Question 416

Which childhood cancer has the best overall survival?

Answer 416

Retinoblastoma (about 99%)

Question 417

What are the 2 main types of primary bone tumour in in children?

Answer 417

Osteosarcoma (more common) or ewing's sarcoma (v rare)

Question 418

Presentation of hepatoblastoma?

Answer 418

Abdominal mass +/- poor appetite, weight loss, lethargy, fever, vomiting, jaundice

Question 419

Investigating hepatoblastoma?

Answer 419

Raised alpha fetoprotein

Question 420

What immunisations at 8 weeks?

Answer 420

6 in 1 rotavirus men B

Question 421

What is contained in the 6 in 1 vaccination?

Answer 421

diphtheria, hepatitis b, Hib, polio, tetanus, whooping cough

Question 422

What immunisations at 12 weeks?

Answer 422

6 in 1, PCV (pneumococcal), rotavirus

Question 423

What immunisations at 16 weeks?

Answer 423

6 in 1, Men B

Question 424

What immunisations at 1 year?

Answer 424

Hib/Men C, MMR, PCV, MenB

Question 425

What immunisations at 3 years 4 months?

Answer 425

MMR, 4 in 1 pre school booster (diphtheria, tetanus, whooping cough, polio)

Question 426

What immunisations at 12-13 years?

Answer 426

HPV

Question 427

What immunisations at 14 years?

Answer 427

3 in 1 teenage booster (tetanus, diphtheria, polio), MenACWY

Question 428

What is klinefelter's syndrome?

Answer 428

Extra X chromosome (commonly 47XXY)

Question 429

How does klinefelter's syndrome present in kids?

Answer 429

Delayed speech or learning difficulties, rapid growth in mid-childhood or truncal obesity, hypogonadism Small firm testes, decreased facial and pubic hair, loss of libido, impotence, gynaecomastia, history undescened testes Infertility

Question 430

Diagnosis of klinefelter's? Management?

Answer 430

Low/low-normal testosterone, elevated FSH and LH, chromosomal analysis Testosterone replacement

Question 431

What is Turner's syndrome?

Answer 431

Loss or abnormality of 2nd X chromosome in phenotypic girls | 45X

Question 432

Different age presentations of Turners syndrome?

Answer 432

Newborns: borderline small, lymphoedema of hands/feet, excessive skin at nape of neck, cardiac abnormalities Infants: close to third centile, feeding difficulties, poor weight gain, poor sleeping Preschool: short stature, recurrent middle ear infections, behavioural difficulties, high activity levels Adolescence: ovarian failure, impaired pubertal growth spurt, obesity, htn, higher prevalence immune disorders, foot problems, learning disabilities Can have dysmorphic features eg webbed neck, short fingers, lymphoedema, high palate Can have renal and heart problems

Question 433

how to diagnose turner's syndrome?

Answer 433

Via amniocentesis Chromosomal analysis Measure gondatrophins, TFTs, renal and heart tests, bone age

Question 434

Management of turner's syndrome?

Answer 434

MDT Short stature- GH therapy May supplement with oestrogen Gonadal failure-combined oestrogen and progestogen

Question 435

Neonatal features of down's syndrome?

Answer 435

Hyperflexibility, muscular hypotonia, transient myelodysplasia, bradycephaly, oblique palpebral fissures, epicanthic folds, ring or iris speckles (brushfield spots), low set/folded ears, stenotic meatus, flat nasal bridge, protruding tongue, high arched palate, loose skin on nape of neck, single palmar crease, short little finger, short broad hands, gap between hallux and second toes, congenital heart defects, duodenal atresia

Question 436

What is down's syndrome? Biggest risk factor?

Answer 436

trisomy 21 | maternal age

Question 437

Management of down's syndrome?

Answer 437

Annual health checks-hearing, resp (OSA), eyes, heart, neurological, GI, hip, thyroid. Extra support in academia and help with living.

Question 438

What is edward's syndrome?

Answer 438

Trisomy 18

Question 439

Presentation of edward's syndrome?

Answer 439

More likely to be female Low birth weight, craniofacial abnormalities (low set and malformed ears, micrognathia, prominent occiput, small facial features, microcephaly, cleft lip), skeletal abnormalities, congenital heart defects, GI abnormalities, GU abnormalities (horse shoe kidney), neurological problems, pulmonary hypoplasia

Question 440

Management of edward's syndrome? What to take into account when making decisions?

Answer 440

Feeding difficulties common, 38.5% foetuses die during labour Chance of survival to 3 months is 20% and to 1 year=8% May decide to not give life sustaining treatment. Otherwise manage cardiac failure and resp insufficiency

Question 441

What is patau syndrome?

Answer 441

Trisomy 13

Question 442

Presentation of patau syndrome?

Answer 442

Many never survive until term and are stillborn or spontaneously abort Low birth weight, congenital heart defects, holoprosencephaly causing midline facial defects, brain and cns abnormalities, GI and GU malformations

Question 443

Life expectancy of patau syndrome?

Answer 443

median is 2.5 days. About 50% live over a week, 5-10% live over a year

Question 444

What is fragile x syndrome?

Answer 444

Most common cause of sex linked general learning disability. Delayed milestones and typical features eg high forehead, large testicles, facial asymmetry, large jaw and long ears.

Question 445

Potential diagnoses coming under fragile x syndrome?

Answer 445

ADHD, ASD, obsessive compulsive behaviours, emotional lability, aggressive behaviours, specific speech disorders eg echolalia and perseveration

Question 446

Management of fragile x patients?

Answer 446

MDT approach and manage each syptom No cure Most diagnosed by age 3 usually

Question 447

Name 4 types of muscular dystrophy?

Answer 447

duchenne muscular dystrophy ( most common and one of most severe) , myotonic dystrophy, facioscapulohumeral muscular dystrophy, becker muscular dystrophy, limb girdle muscular dystrophy, emery-dreifuss muscular dystrophy

Question 448

Male infant not walking by 18 months- concern? what test?

Answer 448

Concerned about duchenne muscular dystrophy | measure creatine kinase

Question 449

Presentation of ?muscular dystrophy?

Answer 449

Gower's maneouvre (stands up by facing floor, wide feet, lift bottom first then hands walked up legs) Waddling gait, lordosis/scoliosis, large calves and leg muscles compared to other muscles

Question 450

Management of muscular dystrophy?

Answer 450

Depends on type and severity eg mobility and breathing assistance Steroids Ataluren if has DMD, is over 5 and can still walk Creatine supplements Treat swallow and heart complications

Question 451

What features would make you suspect Angleman's syndrome?

Answer 451

Developmental delay apparent by 6 months, but forward progression with no loss of skills once acquired Sitting late at 12 months, walking late at 3-4 years, 10% can't walk, flat and turned out feet, lean forward when run, speech impairment, laughter and smiling/happy demeanour, excitable personality. hand-flapping, short attention span, microcephaly, epilepsy, sleep disorders, excess chewing, drooling, fascination with water, hypopigmentation, prominent mandible

Question 452

What is the lifespan of angleman's syndrome?

Answer 452

normal lifespan

Question 453

Presentation prader willi syndrome?

Answer 453

Hypotonia, developmental delay, obesity, learning disability, behavioural problems

Question 454

Genetics of angleman's syndrome?

Answer 454

Maternal deletion of chromosome 15 or paternal uniparental disomy

Question 455

Genetics of prader willi syndrome?

Answer 455

Paternal deletion of chromosome 15 or maternal uniparental disomy

Question 456

Diagnostic criteria for prader willi?

Answer 456

Between birth and 3 years: 5 points including 4 major 3 years-adult=8 points including 5 major Major (1 point); hypotonia with poor suck, feeding problems, excessive weight gain 1-6 years, characteristic facies, hypogonadism, cryptorchidism, global developmental delay, hyperphagia, chromosomal abnormality Minor (0.5 points); infantile lethargy, behavioural problems, sleep disturbance, short and failed pubertal growth spurt, hypopigmentation, small hands and feet, narrow hands, eye abnormalities, thick saliva, speech articulation defects, skin picking

Question 457

Management of prader willi?

Answer 457

MDT GH treatment to maintain normal growth SSRIs trial No role for appetite suppressants

Question 458

What is noonan's syndrome (formerly leopard syndrome)?

Answer 458

Autosomal dominant condition characterised by: congenital heart disease, short stature, broad and webbed neck, sternal deformity, developmental delay, facial features, cryptorchidism, increased bleeding tendency

Question 459

What is william's syndrome?

Answer 459

Rare autosomal dominant disease characterised by CV disease, distinctive facies, connective tissue abnormalties, intellectual disability, growth and endocrine abnormalities Specific cognitive profile: good on verbal short term memory and language, but poor visuospatial construction Overly friendly Infantile hypercalcaemia

Question 460

Management of william's syndrome?

Answer 460

MDT management- manage infantile hypercalcaemia with diet and corticosteroids, yearly surveillance, monitoring CV complications

Question 461

4 domains of developmental milestones?

Answer 461

Gross motor, fine motor and vision, speech and language and hearing, social interaction and self care skills

Question 462

Development of gross motor skills?

Answer 462

``` Newborn: flexed arms and legs, equal movement in all 4 limbs 3 months: lifts head when on tummy 6 months: can lift chest up with support from arms, rolls, can sit unsupported 9 months: pulls to stand 1 year: walking 2 years: walks up steps 3 years: jumps 4 years: hops 5years: rides a bike ```

Question 463

Development of fine motor and vision skills?

Answer 463

``` 4 mo: palmar grasp 8 mo: cube in each hand 12 mo: scribble with crayon, pincer grip 18 mo: tower of 2 cubes 3 years: tower of 8 cubes ```

Question 464

Development of speech, language and hearing?

Answer 464

3 mo: laugh and squeal 9 mo: 'dada', 'mama' 12 mo: 1 proper word 2 years: 2 word sentences, names body parts 3 years: speech mainly understandable 4 years: knows colours, can count 5 objects 5 years: knows meaning of words eg lake

Question 465

Development of social and self care?

Answer 465

6 weeks: smile spontaneously 6 mo: finger feeds 9 mo: wave bye bye 12 mo: uses spoon/fork 2 years: take some clothes off, feed a doll 3 years: play with others, name a friend, put on t shirt 4 years: dress with no help, play simple board game

Question 466

Gross motor developmental red flags?

Answer 466

Not sitting by 1 year, not walking by 18 months

Question 467

Fine motor developmental red flags?

Answer 467

hand preference before 18 months

Question 468

Speech and language developmental red flags?

Answer 468

no clear words by 18 months

Question 469

Social developmental red flags?

Answer 469

No smiling by 3 months, no response to carers interactions by 8 weeks, not interested in playing with peers at 3 years

Question 470

What counts as a global developmental delay?

Answer 470

Over 2 or more domains

Question 471

What is the mildest form of OI? Lethal form? Commonests?

Answer 471

Type I is mildest Type II is lethal Type I is commonest= 60%

Question 472

What is rickets?

Answer 472

Severe vitamin D deficiency (child version of osteomalacia)

Question 473

Pathophysiology of rickets?

Answer 473

lack of vit D; inadequate mineralisation of bone matrix Decreased vit D > decreased calcium and phosphate > secondary hyperparathyroidism In children, this occurs before the growth plates have closed.

Question 474

Presentation of rickets?

Answer 474

Hypocalcaemic seizures or tetany, bony deformity (eg genu varum and valgum), irritable and reluctant to weight bear, severe can result in cardiomyopathy Delayed walking, waddling gait, impaired growth, fractures, dental deformities

Question 475

Investigating rickets?

Answer 475

Bloods, wrist X ray (long bone showing cupping, splaying and fraying of metaphysis eg champagne glass wrist) required for diagnosis

Question 476

Management of rickets?

Answer 476

Diet, sunlight, vit D supplementation=oral calciferol and calcium supplement Maintenance dose of calciferol is recommended for family members

Question 477

Presentation of transient synovitis?

Answer 477

Progressive pain (hip, groin, thigh), limp (inability to weight bear), fever and instability, acute onset Usually no pain at rest, systemically well Often preceded by a viral infection More common in boys Most common cause of hip pain in children aged 3-10 Usually no findings on esr/crp, xray, uss

Question 478

management of transient synovitis?

Answer 478

NO long term sequelae and self limiting Simple analgesia, rest and physio Usually resolves within 2 weeks

Question 479

Presentation of septic arthritis?

Answer 479

Most are under 2 years Fever, pain at rest, inability to weight bear, irritability, if hip=flexed and abducted and externally rotated Increased ESR Mainly hip/knee/ankle/shoulder/elbow

Question 480

Most common cause of septic arthritis?

Answer 480

S. aureus

Question 481

Management of suspected septic arthritis?

Answer 481

FBC, ESR/CRP, synovial fluid examination and culture, blood cultures. Xray will show later changes (14-21 days) Surgical emergency: surgical drainage and IV antibiotics- cefuroxime

Question 482

Most common site of osteomyelitis in children?

Answer 482

Distal femur and proximal tibia

Question 483

Pathophysiology of osteomyelitis?

Answer 483

Infection of bone marrow, commonly S. aureus Inflammatory destruction of bone. If periosteum involved: necrosis and detachment forming a sequestrum Bony remodelling causes deformity NB/ involucrum= viable periosteum separated from underlying bone and forms new bone around it

Question 484

What is the likely mechanism of osteomyelitis in children?

Answer 484

Haematogenous spread- tends to occur in rapidly growing and highly vascular metaphysis of growing bones (long bones more common)

Question 485

Investigating osteomyelitis?

Answer 485

FBC, ESR/CRP, blood cultures, bone cultures (gold standard), MRI

Question 486

Management of osteomyelitis?

Answer 486

Local bone and soft tissue debridement, stabilisation of bone, local antibiotic therapy, reconstruction of soft tissue, reconstruction of osseous defect zone Antibiotic therapy 4-6 weeks if acute, at least 12 weeks if chronic

Question 487

What is perthe's disease?

Answer 487

Self limiting hip disorder caused by varying degrees of ischaemia and subsequent necrosis of the femoral head There is abnormal growth of epiphysis and eventual remodelling of regenerated bone Symptoms occur with subchondral collapse and fracture

Question 488

Key points in history for perthe's disease?

Answer 488

Commonly 5-10 year olds and caucasian. Boys are 80% of cases Developed over weeks No history of trauma All hip movements limited Subacute limp May have referred pain to groin/thigh/knee Unilateral normally Systemically well Internal rotation of hip causes guarding or spasm (roll test)

Question 489

Management of perthe's disease?

Answer 489

If bone age under 6 years: activity restriction, physio, nsaids If bone age over 6 years: surgery

Question 490

What is Kohler's disease?

Answer 490

Osteochondrosis of tarsal navicular bone A non inflammatory, none infectious derangement of bony growth, affecting the epiphyses

Question 491

Presentation of kohler's disease?

Answer 491

Unilateral antalgic gait, local tenderness of medial aspect of foot over navicular bone

Question 492

Management of kohler's disease?

Answer 492

Rest, analgesia, avoid excessive weight bearing, short leg cast for immobilisation, treat for at least 6 weeks Chronic course but rarely over 2 years

Question 493

3 types of discoid meniscus?

Answer 493

Incomplete (bit thicker and wider than normal), complete (tibia completely covered by meniscus) and hypermobile wrisberg (normal shape but no posterior attachment to tibia)

Question 494

How will a discoid meniscus present?

Answer 494

More prone to injury than normal shaped meniscus Some may never experience problems Most cases= knee problems, vague pain, audible snap on terminal extension, swelling, locking

Question 495

Management of discoid meniscus?

Answer 495

If asx, do nothing | otherwise, arthroscopic partial meniscectomy and rehab

Question 496

4 forms of slipped femoral epiphysis?

Answer 496

``` Pre slip (wide epiphyseal line, no slippage) Acute form (spontaneous, sudden slippage) Acute on chronic (slippage acutely on already existing chronic slip) Chronic (steadily progressive slip, 85% cases) ``` each either stable (can walk) or unstable (can't walk)

Question 497

Common age for slipped femoral epiphysis?

Answer 497

Boys at 13 Girl at 11.5 Ie at onset of puberty Tends to be in children v tall and thin or v short and obese

Question 498

Presentation of slipped femoral epiphysis?

Answer 498

Discomfort in hip/groin/medial thigh/knee during walking and a limp Drehmann's sign (passively flex hip, falls back into external rotation and abduction) Acute= within 3 weeks of onset Chronic= more than 3 weeks after onset

Question 499

Management of slipped femoral epiphysis?

Answer 499

Avoid moving/walking, analgesia and surgery (screw fixation, surgical pinning)

Question 500

What is osgood schlatter disease?

Answer 500

Self limiting disorder of the knee | generally in active adolescents before tibial tuberosity has finished ossification, during adolescent growth spurt

Question 501

Presentation of osgood schlatter disease?

Answer 501

Gradual onset pain and swelling below knee, relieved by rest, provoked by knee extension or hyperflexing while prone

Question 502

Management of osgood schlatter disease?

Answer 502

Conservative: rest, ice, physio and exercise advice, simple analgesia Most patients able to return to activity after 2-3 weeks

Question 503

Bedside tests for developmental dysplasia of hip?

Answer 503

``` 6-8 week baby check: Observe for symmetry (folds and legs) Ortolani test (forward pressure to each femoral head- positive=joint laxity) Barlow test (gentle backward pressure to femoral head- see if dislocatable) ``` Galeazzi sign (hip and knee at 90 degrees and compared heights of knees)

Question 504

age cut off for uss or pelvic x ray for DDH?

Answer 504

Under 4.5 months = USS | Over 4.5 months= pelvic XR

Question 505

Management of DDH?

Answer 505

Bracing (<6 months) Dynamic flexion-abduction orthosis (<4.5-6 months) Surgery for non response to harness or splint Prevention= safe swaddling

Question 506

Subtypes of juvenile idiopathic arthritis?

Answer 506

``` Oligoarticular (1-4 joints) Polyarticular (at least 5 joints)- RF positive or negative Systemic Psoriatic Enthesitis-related Undifferentiated ```

Question 507

Qualifying factors for it to be systemic JIA?

Answer 507

at least 2 weeks of fever with at least one of: rash, ln enlargement, hepato/splenomegaly, serositis= pericarditis/pleuritis/peritonitis

Question 508

Qualifying factors for it to be psoriatic arthritis (JIA)?

Answer 508

Arthritis and psoriasis or arthritis and at least 2 of: dactylitis, nail pitting, onycholysis, psoriasis in first degree relative

Question 509

Qualifying factors for it to be enthesitis related arthritis?

Answer 509

Arthritis or enthesitis and 2 of: | sacroiliac/lumbosacral pain, HLA b27 positive, family history HLA b27 related disease, anterior uveitis

Question 510

Management of JIA?

Answer 510

Physio, OT, physical activity encouraged, NSAIDs, steroids, methotrexate May need to use more novel agents eg biologics- etanacerpt. Or sulfasalazine

Question 511

BLS algorithm key points for children

Answer 511

3s's initially: safety, stimulate, shout If not breathing normally 5 rescue breaths 15:2 compressions and breaths Attempt bls for 1 minute before going to get help if by yourself compressions on lower half of sternum: 2 fingers or thumbs for infants heel of hand for children rate= 100-120 bpm

Question 512

Head positions in child bls?

Answer 512

Infants under 1= neutral position Older children= sniffing morning air Suspected neck injury= jaw thrust otherwise=head tilt and chin lift

Question 513

What is newborn respiratory distress syndrome?

Answer 513

Also known as hyaline membrane disease and surfactant deficiency lung disease Usually affects premature babies who don't have the necessary surfactant (enough produced by week 34 normally)

Question 514

Presentation of newborn resp distress syndrome?

Answer 514

Peripheral and central cyanosis, rapid and shallow breathing, flaring nostrils, grunting

Question 515

Management of newborn resp distress syndrome?

Answer 515

Treatment during prem labour= steroid injection before delivery and then a 2nd dose 24 hours later Treatment after birth= extra oxygen if mild symptoms. More severe= ventilator, dose of artificial surfactant,IV fluids and nutrition

Question 516

What warrants mild chronic lung disease of prematurity (bronchopulmonary dysplasia)?

Answer 516

Breathing room air at 36 weeks for babies born before 32 weeks or air by 56 days of postnatal age for babies born after 32 weeks

Question 517

What warrants moderate chronic lung disease of prematurity?

Answer 517

Needing less than 30% oxygen at 36 weeks if born before 32 weeks or needing less than 30% oxygen at 56 days of postnatal age if born after 32 weeks

Question 518

What warrants severe chronic lung disease of prematurity?

Answer 518

Need more than 30% oxygen to breathe +/- CPAP at 36weeks/56 days postnatal

Question 519

What can be seen on CXR of a baby with chronic lung disease of prematurity?

Answer 519

Diffuse haziness, coarse interstitial pattern=atelectasis, inflammation, +/-pulmonary oedema

Question 520

Management of chronic lung disease of prematurity?

Answer 520

Minimise ventilation associated lung injury, continuous oxygen monitoring, nasal CPAP, maintain o2 between 91-95%, send them home on oxygen Potential: Dexamethasone short term, diuretics, methylxanthines eg caffeine (increases resp drive and decreases apnoea)

Question 521

What is meconium?

Answer 521

Dark green faecal material produced during pregnancy, which is passed by the baby

Question 522

Why is meconium aspiration bad?

Answer 522

Vasoactive and cytokine substances activate inflammatory pathways and inhibit the effect of surfactant Causes varying degrees of respiratory distress in the newborn Potential consequences: airway obstruction, foetal hypoxia, pulmonary inflammation, infection (chemical pneumonia), surfactant inactivation, persistent pulmonary htn

Question 523

Management of meconium aspiration?

Answer 523

Depends on severity of resp distress eg obs, routine care, ventilation/oxygen, antibiotics, surfactant, inhaled nitric oxide if pulmonary htn, steroids

Question 524

Prognosis of meconium aspiration?

Answer 524

80% have 3-4 day illness then discharged home | Complications= air leak, cerebral palsy, chronic lung disease

Question 525

What is hypoxic ischaemic encephalopathy?

Answer 525

also known as intrapartum asphyxia Brain injury caused by oxygen deprivation leading to death or severe impairment eg epilepsy, developmental delay, motor impairment, neurodevelopmental delay and cognitive impairment, cerebral palsy Damage is permanent

Question 526

What events could lead to asphyxia?

Answer 526

Acute maternal hypotension, cardiac complications, injury from umbilical cord, impaired blood flow to brain during birth, intrapartum haemorrhage, placental abruption, labour and delivery stress, trauma etc

Question 527

What comes under TORCH infection?

Answer 527

``` Toxoplasmosis Others= syphilis, parvovirus b19, vzv, enteroviruses, lymphocytic choriomeningitis virus, HIV, zika Rubella Cytomegalovirus HSV ```

Question 528

What do torch infections generally do?

Answer 528

Infections during pregnancy that cause congenital defects Generally greater risk of harm if mum infected in early pregnancy Can cause miscarriage or spontaneous abortion

Question 529

Congenital toxoplasmosis issues?

Answer 529

Chorioretinitis with blindness, anaemia, hepatic and neurological symptoms

Question 530

What can the 'others' of Torch infection cause?

Answer 530

Syphilis= bowed sabre shins, hutchinson teeth, typical facial appearance Parvovirus b19= anaemia, hydrops fetalis, myocarditis VZV= chorioretinitis, cataracts, limb atrophy, cerebral cortical atrophy, neurodisability Zika= microcephaly, positional/hearing/ocular abnormalities

Question 531

What can rubella do to the neonate?

Answer 531

Intrauterine growth restriction, intracranial calcifications, microcephaly, cataracts, cardiac defects, neuro disease Blueberry muffin appearance Most occur if exposure during first 16 weeks of pregnancy

Question 532

What can cytomegalovirus do to the neonate?

Answer 532

Growth restriction, sensorineural hearing loss, intracranial calcification, microcephaly, hydrocephalus, hepatosplenomegaly, optic atrophy, delayed psychomotor development More severe in 1st trimester, but more common in 3rd trimester

Question 533

What drug can be used to stop the progression of hearing loss due to CMV?

Answer 533

Ganciclovir

Question 534

Describe physiological jaundice in the neonate?

Answer 534

Increased rbc breakdown and immature liver function Present at 2-3 days of age Jaundice begins to disappear towards end of first week and resolved by 10 days Bilirubin under 200 micromol/L and baby remains well

Question 535

Causes of early neonatal jaundice aka onset in under 24 hours

Answer 535

Haemolytic disease (rhesus, ABO incompatibility, G6PD deficiency, spherocytosis) Infection (toxoplasmosis/TORCH) Haematoma Maternal autoimmune haemolytic anaemia (SLE) Gilbert's syndrome Dubin-Johnson syndrome Crigler-Najjar syndrome

Question 536

Causes of prolonged neonatal jaundice aka lasting over 14 days (term) or over 21 days (preterm)

Answer 536

Infection, hypothyroid, hypopituitarism, galactosaemia, breast milk jaundice, GI eg biliary atresia

Question 537

How common is jaundice in the 1st week of life?

Answer 537

60% term and 80% preterm babies develop it | 15% of healthy infants get physiological breastmilk jaundice

Question 538

What is kernicterus?

Answer 538

A complication of neonatal jaundice- bilirubin encephalopathy (unconjugated) Occurs mainly in premature babies.

Question 539

Features of kernicterus?

Answer 539

Early features= jaundice, hypotonia, poor feeding, absent startle reflex May then develop: high pitched cry, hypertonia of extensor muscles with arched back and hyperextended neck, bulging fontanelle, seizures Potential complications= chronic bilirubin encephalopathy, extrapyramidal signs, visual problems, hearing problems, cognitive defects, dental enamel

Question 540

Management of kernicterus?

Answer 540

Manage neurological complications, phototherapy and exchange transfusions

Question 541

What is the most common GI emergency in neonates?

Answer 541

Necrotising enterocolitis

Question 542

What can be found on examination of a baby with necrotising enterocolitis?

Answer 542

Distension, visible intestinal loops, altered stools, bloody mucoid stool and bilious vomiting, decreased bowel sounds, erythema, associated systemic features

Question 543

Management of ?necrotising enterocolitis?

Answer 543

Confirm diagnosis by AXR and get baseline biochemistry Nil by mouth, bowel decompression, Iv fluids and TPN (total parenteral nutrition), IV abx for 10-14 days (gentamicin and metronidazole), surgery if perforated/necrotic bowel suspected NB/ stages i= suspected, iia=mild, iib=moderate, iiia=advanced, iiib=deteriorating

Question 544

What is gastroschisis?

Answer 544

Congenital defect of the abdominal wall where the abdominal contents herniate into the amniotic sack (usually the small intestine, but sometimes stomach, colon and ovaries). There is no covering membrane. The opening is less than 5cm in length

Question 545

Management of gastroschisis?

Answer 545

Scheduled preterm delivery may improve the post op outcome. | Primary closure of the defect

Question 546

Simple v complex gastroschisis?

Answer 546

``` Simple= intact, uncompromised and continuous bowel Complex= bowel perforation, atresia or necrosis ```

Question 547

Difference between gastroschisis and exomphalos?

Answer 547

Exomphalos has bowel contents in a sac, is more variable in size, and has a more central location Gastroschisis has no covering membrane, is under 5 cm in length and tends to be to the right of umbilical cord insertion

Question 548

What is oesophageal atresia?

Answer 548

Congenital abnormality with a blind ending oesophagus in isolation or with at least one fistula(e) communicating between normal oesophagus and trachea (tracheo-oesophageal fistula0

Question 549

Associated anomalies with oesophageal atresia?

Answer 549

``` VACTERL syndrome (vertebral, anorectal, cardiovascular, tracheo-oesophageal, oseophageal atreisa, renal abnormalities, limb) CHARGE association (coloboma, heart, atresia, retarded development, genital hypoplasia, ear) Chromosomal abnormalities eg trisomies ```

Question 550

Postnatal presentation of oesophageal atresia?

Answer 550

Respiratory distress, choking, feeding difficulties, frothing in first few hours after birth

Question 551

Management of oesophageal atresia?

Answer 551

Surgery

Question 552

Presentation of bowel atresia?

Answer 552

Persistent vomiting-often bilious and within hours of birth | on Xray= double bubble sign of duodenal atresia, colonic atresia has normal air levels

Question 553

Most common metabolic problem in neonates?

Answer 553

Gestational diabetes and hypoglycaemia (glucose passes through placenta and elevates glucose levels in foetus, causing increased insulin secretion, this leads to hypoglycaemia) Plasma glucose under 30 mg/dL

Question 554

Pathophysiology of neonatal hyperthyroidism?

Answer 554

Usually caused by maternal graves disease- antibodies cross the placenta and cause the foetus to develop hyperthyroidism Usually temporary, but can be life threatening.

Question 555

Untreated congenital hyperthyroidism?

Answer 555

Risk of craniosynostosis (early skull closing), intellectual disability, growth failure, hyperactivity in later childhood

Question 556

Management of congenital hyperthyroidism?

Answer 556

Antithyroids and beta blockers- stopped when antibodies have disappeared (usually fully resolved by 6 months) NB/ if mum took thyroid drugs during pregnancy, the child may not show symptoms until 3-7 days

Question 557

How many women carry group b strep in genitals?

Answer 557

1 in 4

Question 558

Presentation of neonate with GBS infection?

Answer 558

Sepsis, pneumonia, meningitis signs, BP changes, seizures

Question 559

Management of GBS infection?

Answer 559

IV antibiotics- penicillin G

Question 560

What can listeria do to a neonate?

Answer 560

Low birth weight, prematurity, circulatory and/or respiratory insufficiency, meningitis, sepsis

Question 561

Management of neonatal listeria infection?

Answer 561

Ampicillin and aminoglycoside eg gentamicin

Question 562

Cause of encephalitis in neonates?

Answer 562

HSV2 from vertical transmission

Question 563

Presentation of HSV encephalitis?

Answer 563

Non specific: decreased levels of consciousness, seizures, lethargy, fever

Question 564

Management of HSV encephalitis? Prognosis?

Answer 564

``` IV aciclovir Highly lethal (around 50%) Complications= deaf, vision loss, cerebral palsy, seizure ```

Question 565

Management of cleft lip/palate?

Answer 565

Surgery Cleft lip at 3-6 months Palate at 6-12 months

Question 566

What is ophthalmia neonatorum?

Answer 566

Conjunctivitis in newborn due to vertical transmission (contact with mum's birth canal infected by STI) Commonly chlamydia Also: haemophilus, strep, staph, pseudomonas, HSV, adenovirus, enterovirus

Question 567

Chance of ophthalmia neonatorum in babies born to untreated chlamydia?

Answer 567

30-50%

Question 568

Presentation of ophthalmia neonatorum?

Answer 568

Red, discharging, swollen lids, usually bilateral | Lid oedema, conjunctival oedema, mucopurulent conjunctivitis, cornea can be involved

Question 569

Management of ophtalmia neonatorum?

Answer 569

Culture conjunctiva and PCR to establish causative organism ``` Bacterial= systemic penicillin G or cephalosporin for gonorrhoea Chlamydia= systemic erythromycin or topical azithromycin Herpetic= topical and systemic aciclovir ```

Question 570

Premature baby still needing breathing support by 36 weeks corrected age = what?

Answer 570

Chronic lung disease/ bronchopulmonary dysplasia | Caused by underdeveloped lungs

Question 571

Management of bronchopulmonary dysplasia?

Answer 571

Oxygen (can further damage lungs), breathing support (CPAP or mechanical ventilation), may need steroids

Question 572

Pathophysiology of retinopathy of premature baby?

Answer 572

Abnormal blood vessel growth are fragile and weak, causing bleeding and leakage Usually bilateral Ranges from abnormal blood vessel growth to full retinal detachment (stage i-v) Can cause blindness

Question 573

Management of retinopathy of premature baby?

Answer 573

Laser therapy or cryotherapy: destroys peripheral retina and slows/reverses abnormal vessel growth, but loses some side vision

Question 574

Why are premature babies at risk of osteopenia?

Answer 574

May not receive the full amount of calcium and phosphorus needed to form strong bones Haven't been as active in the womb and thus have weak bones Very premature babies lose more phosphorus in urine than term babies Can be due to low vit D

Question 575

How to treat osteopenia of premature baby?

Answer 575

Supplements- calcium and phosphorus added to IV fluids or breast milk Special formulas if no breast milk Vit D supplements for babies with liver problems

Question 576

What is periventricular leukomalacia?

Answer 576

White matter surrounding ventricles deprived of blood and thus oxygen. This causes softening of the white matter

Question 577

Consequences of periventricular leukomalacia?

Answer 577

Most commonly cerebral palsy May have visual problems and learning disability Not progressive Presentation depends on amount of brain tissue damaged

Question 578

What is apnoea in a prem baby?

Answer 578

Prem baby pauses breathing for over 15-20 seconds or pauses breathing for under 15 seconds but has bradycardia or low sats Usually self limiting

Question 579

What is transient tachypnoea of the newborn?

Answer 579

Caused by leftover fluid in lungs making it harder for the alveoli to stay open RR over 60 Most improve over 24-48 hours, and most cases self limit

Question 580

Who is more likely to have transient tachypnoea of the newborn?

Answer 580

Born before 38 weeks, c-section delivery, born to diabetic or asthmatic mother, twins, male

Question 581

Forms of child abuse

Answer 581

Physical, neglect, emotional, sexual

Question 582

What may come under child neglect?

Answer 582

Medical eg unimmunised, non attendance Nutritional eg faltering growth, obesity Emotional Educational eg poor school attendance Physical eg inadequate hygiene, persistent infestation, inappropriate clothing Failure to supervise eg frequent A+E trips, injury suggesting lack of care such as sun burn

Question 583

Normal presentation/history of toddler's fracture?

Answer 583

Subtle undisplaced spiral fracture of tibia Usually pre school, sudden twist after an unwitnessed fall Local tenderness over tibial shaft or on general strain on tibia

Question 584

Criteria for septic arthritis rather than just irritable hip?

Answer 584

Kocher's criteria | Fever over 38.5, cannot weight bear, ESR>40 in 1st hour, WCC>12

Question 585

RFs for DDH?

Answer 585

Breech, female, C-section, 1st child, premature, oligohydramnios, FH, club feet, spina bifida, infantile scoliosis

Question 586

What constitutes an atypical UTI in children and what do you need to do if this is the case?

Answer 586

``` Atypical= septicaemia/IV abx needed, non e.coli, poor urine flow, abdo/bladder mass, increase creatinine, failure to respond to treatment within 48 hours Recurrent= at least 2 episodes with at least 1 with systemic features, or at least 3 episodes without systemic features ``` Need to investigate for a structural abnormality

Question 587

How to investigate ?structural abnormality in the case of UTI?

Answer 587

1. USS renal tract (looks at size and drainage of kidneys and bladder) 2. Micturating cystourethrogram/MCUG (looks for vesicoureteric reflux, looks at bladder and posterior urethral valve) 3. DMSA scan/radionucleide imaging (looks at renal function and any renal scarring)

Question 588

Features of nephritic syndrome?

Answer 588

Haematuria, proteinuria, impaired GFR, salt and water retention (htn and oedema)

Question 589

Management of nephritic syndrome?

Answer 589

Fluid balance management (input/output, fluid restriction, diuretics, salt restriction) Correct other imbalances (K, acidosis) Dialysis may be needed (uncommon) Penicillin for treatment of an ongoing strep infection

Question 590

What is choanal atresia? Presentation? Management?

Answer 590

Failure of the nose to canalise (bony or membranous) Px: Cyclical cyanosis: cries and goes pink, when stops goes blue and repeat. No misting on a cold spatula Mx: secure airway and send to tertiary centre for dilatation +/- stent insertion

Question 591

Management of epistaxis?

Answer 591

ABC Med tx= topical naseptin, silver nitrate cautery Surgical tx= electrocautery NB/ teenage boy with persistent nosebleeds and nasal obstruction- ?juvenile nasopharyngeal angiofibroma

Question 592

Why is sinusitis uncommon in children?

Answer 592

Maxillary sinus exists at birth but only grows to full size after 2nd dentition Ethmomid sinuses only 2-3 cells at birth No/rudimental frontal sinuses at birth (develop by 7-8 years)

Question 593

Narrowest point in child's airway vs adult's?

Answer 593

Children=subglottis | Adults=vocal cords

Question 594

What is the most common airway abnormality in children and it's presentation?

Answer 594

Laryngomalacia | Normal voice, stridor worse on feeding/exertion/supine, failure to thrive in 10%, increased work of breathing

Question 595

What tool to produce standardised disability levels and what domains assessed?

Answer 595

WHO-DAS (disability assessment schedule) | Cognition, mobility, self care, social, life activities, participation

Question 596

What to do if ESBL for UTI?

Answer 596

Resistant to all penicillins and cephalosporins | Therefore switch to IV meropenem (or gentamicin)

Question 597

empirical Abx for pneumonia

Answer 597

Amoxicillin

Question 598

Medical words for short sighted and long sighted? | Difference of at least 1 unit in refractive error of each eye?

Answer 598

Short=myopia Long=hypermetropia 1 unit difference= asinometropia

Question 599

Treatment of amblyopia?

Answer 599

1. Refractive adaptation (glasses for 16-18 weeks) 2. Occlusion of better seeing eye (full or part time) 3. Atropine drops/ointment into better seeing eye (blurs vision due to dilation of pupil and paralysis of accommodation)

Question 600

Ocular movements name for the right movement and left and midline movements?

Answer 600

Right= dextro elevation/version/depression Left=laevo elevation/version/depression Midline= direct elevation, primary position, direct depression

Question 601

Management of strabismus?

Answer 601

Conservative= glasses/contact lenses, prisms (good for diplopia), orthoptic exercises (latent strabismus) Surgery: For esotropia; bilateral medial rectus recession or weaken medial rectus+ strengthen lateral rectus in the same eye For exotropia: bilateral lateral rectus recession or weaken lateral rectus+strengthen medial rectus in same eye Also: botulinum toxin into muscle that needs weakening, done under ketamine anaesthesia

Question 602

What shape lens for myopia? For hypermetropia?

Answer 602

Myopia/short sighted= concave | Hypermetropia/long sighted=convex

Question 603

When do you expect primitive reflexes to disappear?

Answer 603

By 4-6 months, lots of babies lose them within 2 months

Question 604

What is sturge weber syndrome?

Answer 604

Port wine birthmark on upper half of face, abnormal blood vessels in brain and eye abnormalities (eg glaucoma)

Question 605

What counts as mild dehydration and features?

Answer 605

Less than 5% | Thirst, dry lips, restlessness, irritability

Question 606

What counts as moderate dehydration and features?

Answer 606

5-10% | Sunken eyes, decrease skin turgor, decreased urine output

Question 607

What counts as severe dehydration and features?

Answer 607

Over 10% | Cold, mottled, hypotensive, anuria, decreased consciousness

Question 608

What options for formula for children with cow's milk protein allergy?

Answer 608

Hydrolysed eg althera Amino acid based eg alfamino Goat milk formula from birth Soy formula from 6 months

Question 609

What fluids needed for not dehydrated/dehydrated/shocked?

Answer 609

Not dehydrated=maintenance Dehydrated=maintenance+deficit Shocked=bolus+maintenance+deficit

Question 610

What maintenance fluids do neonates need?

Answer 610

10% glucose with increasing rate every day Monitor U+Es every day From day 2 if needed= sodium 2-3mmol/kg/day and potassium 1-2mmol/kg/day

Question 611

How to estimate child's weight?

Answer 611

Weight (kg) = (age (yrs) + 4) x 2

Question 612

What fluids for maintenance in children? How much?

Answer 612

0.9% NaCl (normal saline) First 10kg=100ml/kg Next 10kg=50ml/kg Every other kg=20ml/kg Calculates how much in 24 hours

Question 613

How do you calculate the fluid rate in children?

Answer 613

Rate ml/h= total (ml) / 24

Question 614

How to calculate the deficit fluids in children?

Answer 614

Deficit x 10 x wt(kg) Where estimated deficit= mild= negligible, mod=5%, severe=10%

Question 615

What bolus do you use and which volume?

Answer 615

0.9% NaCl, volume=20ml/kg May use 10ml/kg in some cases eg trauma, DKA Tends to be 20ml/kg if shocked in DKA, 10ml/kg if not shocked

Question 616

What constitutes faltering growth?

Answer 616

Failure to gain adequate weight or height/length during infancy or early childhood Birth weight <9th centile with a fall across at least 1 wt centiles Birth weight between 9-91st centile with a fall across at least 2 wt centiles Birth weight over the 91st centile with a fall across at least 3 wt centiles Current weight below 2nd centile whatever the birth weight

Question 617

When would you expect a baby to get back to his birth weight?

Answer 617

3 weeks

Question 618

What is ARFID?

Answer 618

Avoidant or restrictive food intake disorder Significant weight loss and nutritional deficiencies with a reliance on supplements but not underlying condition causing it

Question 619

Measure of severity of dka and corresponding fluids?

Answer 619

Use pH of blood to rate severity 5% fluid deficit in milk DKA= 7.2-7.29 +/or bicarb <15 7% deficit in mod DKA= 7.1-7.19 +/or bicarb <10 10% deficit in severe DKA= <7.1 +/or bicarb <5 Do not subtract boluses given for shock

Question 620

What are the 3 shunts in the foetal circulation?

Answer 620

Foramen ovale: right atrium to left atrium (bypass lungs) Ductus arteriosus: pulmonary artery to aorta Ductus venosus: bypass liver

Question 621

How will coarctation of the aorta present?

Answer 621

Weak femoral pulses Pre and post ductal difference in saturations if DA still patent Discrepancy between upper and lower limbs In older children= may develop murmur over back after collaterals develop If ductus arteriosus closes straight away: babies present collapsed and acidotic

Question 622

How will aortic stenosis present?

Answer 622

Weak pulses all over. Thrill palpable in suprasternal region and carotid area. Ejection systolic murmur in aortic area

Question 623

How does pulmonary stenosis present?

Answer 623

Ejection systolic murmur in the left upper sternal edge. Murmur often radiates to back. Right ventricular heave if significant stenosis

Question 624

Simple pathophysiology of transposition of great arteries?

Answer 624

Pulmonary artery and aorta switched | Deoxygenated blood travels to body and oxygenated blood travels through the lungs

Question 625

What are the causes of hearing loss in children?

Answer 625

70% non syndromic eg congenital infections esp CMV, general infections 30% syndromic eg ushers syndrome, wardenbergs syndrome, treacher collins, alport syndrome

Question 626

With conductive hearing loss, where is the problem likely to be?

Answer 626

Middle ear eg glue ear, ear wax, perforated ear drum, eustachian tube dysfunction

Question 627

With sensironeural hearing loss, where is the problem likely to be?

Answer 627

Inner ear- most cases affecting the cochlea

Question 628

What are the two broad types of hearing test?

Answer 628

Objective- otoacoustic emissions, auditory brainstem response Subjective- distraction test, visual reinforcement audiometry, performance testing, pure tone audiometry

Question 629

Causes of osteoporosis in children?

Answer 629

Inherited/congenital eg OI, inborn errors of metabolism, haem problems, idiopathic Acquired eg drug induced eg steroids, endocrinopathies, malabsorption

Question 630

What is osteogenesis imperfecta?

Answer 630

AD inherited condition, v rare Mostly due to defects in type i collaegen Characteristed by bone fragility, fractures, deformity, bone pain, poor growth, impaired mobility

Question 631

How can you manage osteogenesis imperfecta?

Answer 631

MDT management esp ortho team and metabolic bone specialist | Bisphophonates

Question 632

Describe the types of osteogenesis imperfecta?

Answer 632

type 1- most common, mild 2- lethal 3- progressively deforming, severe 4- moderate

Question 633

Describe rickets presentation

Answer 633

Rachitic rosary, limb deformity, weakness, misery Metaphyseal swellings, bowing deformities, slowing of linear growth, motor delay, hypotonia, fractures, resp distress

Question 634

Causes of low vit d/rickets?

Answer 634

Maternal vitamin D deficiency causes low stores in newborn, exclusive breastfeeding will exacerbate Lack of dietary intake ie prolonged unsupplemented breastfeeding

Question 635

What may indicate a shaken baby?

Answer 635

Hypoxia, subdural haematoma, rib fractures, retinal haemorrhages, may have other fractures, torn frenulum

Question 636

Normal HR, RR and systolic BP for under 1s

Answer 636

110-160 bpm RR: 30-40 BP: 80-90

Question 637

Normal HR, RR and systolic BP for 1-2 yos?

Answer 637

100-150 bpm RR: 25-35 BP: 85-95

Question 638

Normal HR, RR and systolic BP for 2-5 yos?

Answer 638

95-140bpm RR: 25-30 BP: 85-100

Question 639

Normal HR, RR and systolic BP for 5-12 yos?

Answer 639

90-110bpm RR: 20-25 BP: 90-110

Question 640

How should a premature baby be fed?

Answer 640

IV fluids/parenteral nutrition as suckle and swallow only starts from 32-34 weeks Start small volumes of expressed breast milk and build up to full feeds slowly (risk of NEC if too quick)

Question 641

How to manage jaundice?

Answer 641

Treat underlying | Phototherapy or exchange transfusions

Question 642

Causes of acute scrotum in children?

Answer 642

Testicular torsion!!! | Also torsion of appendix testis (self limiting, but if any diagnostic doubt treat as testicular torsion)

Question 643

Vomits and presentation age in pyloric stenosis, malrotation and intussusception

Answer 643

Malrotation: bilious green vomit at 24 hours Pyloric stenosis: milky vomit at 4 weeks Intussusception: milky then green vomit at 6 months

Question 644

Acute asthma management?

Answer 644

Oxygen if needed, SABA, prendisolone 1mg/kg, IV salbutamol bolus, aminophylline/MgSO4/salbutamol infusion options