Paediatrics

Question 1

Resp distress Px

Answer 1

• Increased RR
• Tripod pos
• Stridor / wheeze / grunting
• Tracheal tug
• Accessory muscle use - SCM…
• Supraclavicular / suprasternal / intercostal / substernal / subcostal recession
• Abdominal breathing (see-saw)
• Head bobbing
• Nasal flaring

Question 2

Term neonate maintenance fluids

Answer 2

Use 10% dextrose

Birth-D1 - 50-60ml/kg/d
D2 - 70-80
D3 - 80-100
D4 - 100-120
D5-28 - 120-150

Question 3

Child >28d maintenance fluids

Answer 3

0.9%NaCl + 5% glucose

100ml/kg/d for first 10kg
50ml/kg/d for next 10kg
20ml/kg/d for every kg over 20kg

(4,2,1ml/kg/hr)

Question 4

Paeds replacement fluid

Answer 4

0.9% NaCl + 5% glucose + K replacement if needed

% dehydration = (well weight-current weight)/well weight x 100

deficit (ml) = % dehydration x weight (kg) x 10

Question 5

Paeds resus fluids

Answer 5

Bolus 0.9% NaCl 10ml/kg over 10 mins

Question 6

Developmental milestones

Answer 6

GO OVER THIS AGAIN

Question 7

Milestones concerns

Answer 7

Gross motor
Not sitting by 1yo
Not walking by 18mo

Fine motor
Hand preference before 18mo

Speech + language
Not smiling by 3mo
No clear words by 18mo

Social development
No response to carers by 8wks
Not interested in playing with peers by 3yo

RED FLAGS
regression
poor health / growth
significant FHx
Examination - microcephaly, dysmorphic features
safeguarding indicators

Question 8

Shaken baby syndrome

Answer 8

Intentional shaking of child 0-5yo

retinal haemorrhages
subdural haematoma
encephalopathy

Question 9

Bronchiolitis

Answer 9

Acute infection of bronchioles

RSV

<1yo, mostly <6mo - in winter

RFs
prem, SGA, <12wks, congenital HD, nursery…

Question 10

RSV course

Answer 10

URTI (coryza)
50% get better
50% chest sx in 1-2d
Worse d3-4, lasts 7-10d, max 2-3wks

Question 11

Bronchiolitis Px

Answer 11

Coryza
Cough, SOB, increased RR, reduced feeding, irritable
Mild fever
Apnoeas
Wheeze / crackles bilaterally

Question 12

Bronch Ix

Answer 12

Immunofluorescence of nasopharyngeal secretions may show RSV

Clinical dx
NPA - nasopharyngeal aspirate
Blood gases if severe, CXR

Question 13

Bronch reasons for admission

Answer 13

<3mo, pre-existing condition, reduced feeding, dehydrated, increased RR, resp distress, low sats, apnoeas, parents struggling to manage

Question 14

Bronch Mx

Answer 14

Supportive - calpol, feeds, nasal suctioning, O2

Ventilatory support - high flow O2, CPAP, intubation

Palivizumab - MAb for prevention - for high risk, prem…

Question 15

Viral-induced wheeze

Answer 15

Acute wheeze from viral infection

RSV / rhinovirus commonly

Question 16

Viral induced wheeze Px

Answer 16

SOB
Coryza
Fever
Resp distress
Global wheeze

Question 17

Viral induced wheeze Mx

Answer 17

Salbutamol nebs
Pred
Mg
Monteleukast
Inhaled corticosteroids later on

Question 18

Croup

Answer 18

URTI - inflammation of larynx

Acute laryngotracheobronchitis

6mo-6yo, peak 2yo

Parainfluenza

Question 19

Croup Px

Answer 19

Coryza
Barking cough
Stridor, resp distress
Drowsy, lethargic, cyanosis

Question 20

Croup Ix

Answer 20

Clinical Dx

XR - steeple sign

Question 21

Croup Mx

Answer 21

Oral dexamethasone 0.15mg/kg oral, rpt dose after 12hrs if needed

O2, nebulised budesonide / adrenaline, intubate

Question 22

Epiglottitis

Answer 22

Inflammation of epiglottis

H influenzae B

Vaccine against

Question 23

Epiglottitis Px

Answer 23

Sore throat
Stridor
Drooling
Tripod
High fever
Dysphagia
Muffled voice
Unwell

Question 24

Epiglottitis Ix

Answer 24

lateral neck XR - thumb sign

Laryngoscopy is dx

Throat swab / culture

Question 25

Epiglottitis Mx

Answer 25

Do not distress Anaesthetist / ENT IV ceftriaxone once airway secure Rifampicin to household contacts

Question 26

Whooping cough

Answer 26

URTI from Bordetella pertussis (G-) Resp droplet spread Stay off school for 21d after sx onset, or 48hrs after abx

Question 27

Whooping cough Px

Answer 27

2-3d coryza Coughing fits Loud inspiratory whoop Faint, vomit, PTX, epistaxis... Apnoeas

Question 28

Whooping cough Ix

Answer 28

Nasal swab PCR Anti-pertussis toxin IgG Bloods - WCC raised

Question 29

Whooping cough Mx

Answer 29

Notify PHE (equivalent) Supportive / admit Macrolide within 21d - clarithromycin / azithromycin / erythromycin (ery if pregnant) Co-trimoxazole alternative Prophylactic abx for vulnerable contacts - pregnant, unvaccinated

Question 30

Acute asthma

Answer 30

chronic inflammation + reversible obstruction of airways

Question 31

Acute asthma px

Answer 31

SOB Wheeze cough nocturnal cough tight chest Sx are intermittent, variable, worse at night, triggered

Question 32

Acute asthma severity

Answer 32

Mild/moderate PEF>50% normal speech Severe SOB to talk/feed Deranged obs sats <92% PEF <50% Life-threatening Cyanosis, pallor silent chest poor resp effort reduced GCS sats <92% PEF <33%

Question 33

Acute asthma Mx

Answer 33

- O2 - Salbutamol inhaler 4-6 puffs every 4hrs / 10 puffs every 2hrs - Nebulised salbutamol + ipratropium - 3d oral prednisolone - IV Mg - IV salbutamol - IV aminophylline - Monitor K Discharge when child well on 6 puffs 4hrly

Question 34

Chronic asthma

Answer 34

Chronic inflammatory airway disease, reversible obstruction Atopic Omalizumab - potential tx - anti IgE

Question 35

Chronic asthma px

Answer 35

episodix dx, intermittent exacerbations diurnal variability - worse at night / early morning dry cough wheeze SOB triggers Hx of atopy

Question 36

Chronic asthma Ix

Answer 36

- Clinical dx after 5yo - Spirometry + reversibility testing - Direct bronchial challenge - histamine / methacholine - FeNO - Peak flow variability

Question 37

Chronic asthma Mx <5yo

Answer 37

1. salbutamol 2. low dose ICS / montelukast 3. other step 2 option 4. refer to specialist

Question 38

Chronic asthma Mx 5-12yo

Answer 38

1. Salbutamol 2. Low dose ICS 3. Salmeterol 4. medium dose ICS, ?montelukast / theophylline 5. High dose ICS 6. refer

Question 39

Chronic asthma Mx >12yo

Answer 39

1. salbutamol 2. ICS low dose 3. salmeterol 4. medium dose ICS, ?montelukast / theophylline / tiotropium 5 . high dose ICS, add other option 4 options, refer 6. Oral daily steroids

Question 40

Pneumonia

Answer 40

Lung infection - inflammation of lungs, exudate production Bacterial - Strep pneumonia, GAS, GBS, S aureus, H influenza, M pneumonia Viral - RSV, parainfluenza, influenza

Question 41

Pneumonia Px

Answer 41

cough fever >38 raised RR, HR IWOB Sepsis focal coarse creps Bronchial breath sounds

Question 42

Pneumonia Ix

Answer 42

Bloods CXR - consolidation Sputum culture Throat swabs

Question 43

Pneumonia Mx

Answer 43

Amoxicillin / benpen Add macrolide if atypical / mycoplasma / chlamydia Co-amox O2

Question 44

Laryngomalacia

Answer 44

Floppy larynx -> partial obstruction Tissue around supraglottic larynx is softer -> stridor Px Stridor, intermittent Worse when feeding, upset, lying on back Mx usually grow out of it Rarely - tracheostomy, surgery to alter larynx

Question 45

Chronic lung disease of prematurity (CLDP)

Answer 45

- Bronchopulmonary dysplasia - Occurs in prem babies <28wks, suffer resp distress syndrome, need I+V at birth - CXR changes later, infant may need O2 after 36wks Px Low sats, IWOB Poor feeding/weight gain Crackles / wheeze Increased infections Mx Home O2, wean Pavilizumab for RSV prevention Prevention steroids in prem labour CPAP > intubation after birth Caffeine for apnoeas

Question 46

Cystic fibrosis

Answer 46

Autosomal recessive multi-organ disease Mutation in CFTR gene -> thickened secretions - (low Cl secretion, increased Na resorption, high sodium sweat...) Resp disease, concentrated bile, water deficiency in bowel, pancreatic insufficiency

Question 47

CF Px

Answer 47

Neonates Failure to thrive Meconium ileus Rectal prolapse Resp Cough, thick mucus, wheeze, recurrent infections, bronchiectasis, sinusitis, nasal polyps, spon PTX, haemoptysis, SOB Malabsorption, DM, steatorrhoea, gallstones, infertility, clubbing, osteoporosis

Question 48

CF Ix

Answer 48

- Screen with newborn spot test - Sweat test - high Na + Cl - Genetic testing - Pulmonary function tests

Question 49

CF Mx

Answer 49

Chest physio Exercise High calorie diet Abx Dornase alfa nebs - mucolytic Salbutamol nebs Nebulised hypertonic saline CREON tablets Vit ADEK Ursodeoxycholic acid Fertility Tx Lung / liver transplant

Question 50

Primary ciliary dyskinesia / Kartagner's syndrome

Answer 50

Cilia affected, autosomal recessive - consanguinity Frequent LRTIs, reduced fertility Kartagner's triad Paranasal sinuses Bronchiectasis Situs invertus Chest physio, high calorie diet, abx

Question 51

Bacterial tracheitis

Answer 51

Infection of trachea Pseudomonas IV cefotaxime, fluclox

Question 52

Congenital HD

Answer 52

Acyanotic VSD, ASD, PDA, coarc, AS Cyanotic TOF, TGA, tricuspid atresia Mx at birth of cyanotic Supportive Prostaglandin E1 (alprostadil) - keeps PDA open

Question 53

Innocent murmurs

Answer 53

Venous hums - turbulent blood flow in great veins - continuous blowing noise below clavicles Still's murmur - low-pitched sound heard at L lower sternal edge

Question 54

PDA

Answer 54

- Patent ductus arteriosus - L->R shunt (aorta -> pulm artery) - fetus pulm vascular resistance high, shunt R->L to bypass lungs (also flows from RA->LA through foramen ovale) - resistance decreases at birth, duct closes due to decreased resistance - may stay open - genetics, prem, maternal rubella - Leads to pulm HTN, Eisenmengers, RVH, RHF, eventually LHF Px - continuous machinery murmur - SOB, difficulty feeding Ix - ECHO Mx - Indomethacin - prostaglandin inhibitor - Transcatheter / surgical repair - monitor for 1yr with ECHOs

Question 55

ASDs

Answer 55

Hole in septum between atria L-R shunt Then RHF, pulm HTN, Eisenmengers, R->L, cyanotic Px Mid systolic murmur (from increased flow across pulm valve due to more blood in R heart Fixed split S2 SOB, difficulty feeding, URTIs Ix ECHO Mx Can w+w Transvenous / surgical closure

Question 56

VSDs

Answer 56

Hole in septum between ventricles Downs, Turners association L-R, becomes R-L with Eisenmenger's Px - pan-systolic murmur - SOB, poor feeding, failure to thrive, cyanosis Ix - ECHO Mx - w+w if small, may close spontaneously - Surgical / transvenous closure

Question 57

AVSD

Answer 57

Hole in centre of heart - involves ventricular, atrial septum, mitral, tricuspid valves Down's association Px SOB neonate Poor weight gain / feeding Eisenmenger's over time Mx surgical repair

Question 58

Eisenmenger's syndrome

Answer 58

Reversal of L-R shunt in congenital HD due to pulm HTN In L-R - high pressure pulm flow - damage to pulmonary vasculature, increased resistance through lungs -> pulm HTN, reverse shunt to R-L, cyanotic pt associated with ASD, VSD, PDA Px Original murmur may disappear Cyanosis Clubbing RVF Haemoptysis Mx Heart-lung transplant Tx pulm HTN

Question 59

Coarctation of aorta

Answer 59

- Congenital narrowing of aortic arch, usually around ductus arteriosus - Turner's association - Collateral vessels grow Px - weak femoral pulses - high BP in head, neck, R arm, low BP in lower limbs - systolic murmur - radiofemoral delay - SOB, poor feeding, grey/floppy Ix - USS, CT angio Mx - if severe, prostaglandin E to keep duct open - surgical correction

Question 60

Aortic stenosis

Answer 60

- Congenital narrowing of aortic valve - bicuspid aortic valve Px - fatigue, SOB, dizzy, faint - HF at birth - ejection systolic murmur in aortic area - slow rising pulse, narrow pulse pressure Ix - ECHO Mx - percutaneous balloon aortic valvuloplasty - surgical valvotomy - valve replacement

Question 61

Pulmonary stenosis

Answer 61

- congenital narrowing of pulmonary valve - associations - TOF, William, Noonan, congenital rubella Px - SOBOE, dizzy, faint - RHF - Ejection systolic murmur in pulmonary area Ix - ECHO Mx - Balloon valvuloplasty - valve replacement

Question 62

Tetralogy of Fallot

Answer 62

- VSD, PS, RVH, overriding aorta (allowing blood from both ventricles in) - R->L (stenosis of RV outflow means R pressure >L - cyanotic RFs - rubella, older mother, alcohol in pregnancy, diabetic mother Px - picked up on scans - central cyanosis - clubbing - low birth weight + growth - SOB - ejection systolic murmur Ix - ECHO - CXR - boot shaped heart Mx - prostaglandin infusion - surgery

Question 63

Tet spells

Answer 63

- cyanotic episode, R-L shunt worsened - when pulm vasc resistance increases, or systemic resistance decreases - eg exertion (CO2 is vasodilator), crying Px - low sats Mx - squat - knees to chest - O2 - IV fluids - increase preload, increase vol to pulm vessels - Morphine - reduce resp drive - more effective breathing - sodium bicarb - acidosis - phenylephrine infusion - increase SVR

Question 64

Ebstein's anomaly

Answer 64

- Congenital - tricuspid valve lower, big RA, small RV - poor flow to pulm vessels, poor flow from RA-RV - associated with ASD, WPW Px - HF, oedema - S3,4 - cyanosis, SOB, collapse - worse when duct closes, a few days after birth (blood could flow from aorta -> pulm vessels to get oxygenated) Ix - ECHO Mx - Tx arrhythmias, HF - Surgery

Question 65

Transposition of great arteries

Answer 65

- Attachments of aorta / pulmonary trunk swapped - increased risk in diabetic mothers - RV pumps into aorta, LV into pulmonary vessels - 2 separate circulations - cyanosis - survival depends on PDA (or ASD / VSD if present) Px - detected on antenatal scans - cyanosis at birth, raised RR - shunt compensates to begin - resp distress, tachycardia, poor feeding, sweat Ix - CXR - egg on side appearance Mx - prostaglandin infusion - balloon septostomy - make ASD - open heart surgery

Question 66

HF

Answer 66

Causes - malformation, cardiomyopathy, pericardial effusion, myocarditis, arrhythmias Px - poor feeding, failure to thrive - sweaty - SOB - during feeding - raised RR, HR - Gallop rhythm, rapid weight gain, enlarged liver Ix - CXR, ECG, ECHO, BP (upper/lower limbs), sats (on pre/post ductal limbs) Mx - Sit up, NG feed, O2 - Furosemide + spironolactone - Monitor K

Question 67

Arrhythmias

Answer 67

SVT - SOB, poor feeding, HF, pallor, palpitations - ECG, cardiac monitoring - A-E, DC shock if shocked, vagal manoeuvres (diving reflex, carotid massage, valsalva), adenosine, flecainide / amiodarone VT - much the same - shock, amiodarone, Mg Brady - A-E, o2 - adrenaline bolus + infusion - atropine

Question 68

Constipation causes

Answer 68

Idiopathic, poor fluid/fibre intake Hirschsprung's CF Hypothyroid Spinal cord lesions Sexual abuse, safeguarding Intestinal obstruction Anal stenosis Cow's milk intolerance

Question 69

Encopresis

Answer 69

faecal incontinence >4yo - overflow past impaction - rectum desensitised

Question 70

Constipation red flags

Answer 70

meconium ileus neuro sx vomiting abdo pain abnormal anus failure to thrive

Question 71

Constipation Mx

Answer 71

Hydrate, fibre Disimpaction Movicol After 2wks if no disimpaction, add senna / docusate Can use lactulose if movicol not tolerated Maintenance Movicol

Question 72

Faltering growth causes

Answer 72

Not enough in ineffective feeding, GORD, feed refusal Mx - NG feed, gastrostomy Not absorbed anaemia, biliary atresia, coeliac, infections, IBS, CF, CMPA Too much used up chronic infections (HIV, TB), CLDP, con HD, hyperthyroid, asthma, malignancy, IBD abnormal central control GH, thyroid, psychosocial influence, avoidant/restrictive food intake disorder

Question 73

GORD

Answer 73

reflux of stomach contents via LOS - immature in babies

Question 74

Causes of vomiting

Answer 74

overfeeding GORD pyloric stenosis - projectile gastritis / GE appendicitis infections - UTI, tonsillitis, meningitis intestinal obstruction bulimia

Question 75

GORD Px

Answer 75

Reflux after larger feeds vomiting chronic cough hoarse cry unsettled after feeds reluctant to feed poor weight gain

Question 76

GORD red flags

Answer 76

bile projectile blood reduced GCS blood in stools rash - eg CMPA ?aspiration

Question 77

GORD Mx

Answer 77

- small meals - Gaviscon, thickened milk - omeprazole / ranitidine if nothing else works - severe - barium meal, endoscopy, surgical fundoplication

Question 78

Sandifer's syndrome

Answer 78

torticollis / dystonia with GORD - refer for assessment

Question 79

Pyloric stenosis

Answer 79

hypertrophy + thickening of pyloric sphincter prevents food moving from stomach to duodenum

Question 80

Pyloric stenosis Px

Answer 80

~1mo failure to thrive projectile vomiting - milky see peristalsis in abdo palpate pylorus - olive

Question 81

Pyloric stenosis Ix

Answer 81

Blood gas - high pH, low Cl, low H, low K - metabolic alkalosis Abdo USS

Question 82

Pyloric stenosis Mx

Answer 82

Laparoscopic pyloromyotomy (Ramstedt's operation)

Question 83

Gastroenteritis

Answer 83

infection of intestines Viral - rotavirus, norovirus, adenovirus Bacterial - E coli, campylobacter, shigella, salmonella .... Px N+V+D abdo pain fever blood in stools Ix stool sample bloods assess hydration Mx off school fluids, dioralyte maintain feed no-antiemetic / anti-diarrhoea

Question 84

Coeliacs

Answer 84

Autoimmune reaction to gluten - inflammation in SI - villous atrophy and malabsorption - jejunum mostly Associations T1DM, thyroid, PBC, PSC, Down's, autoimmune hep

Question 85

Coeliacs Px

Answer 85

Asym Failure to thrive diarrhoea fatigue wt loss mouth ulcers anaemia - iron, B12, folate Dermatitis herpetiformis Neuro sx - neuropathy, ataxia, epilepsy

Question 86

Coeliac Ix

Answer 86

- raised anti-TTG - raised anti-EMA - check total IgA - exclude deficiency (would show false negative for ABs) - endoscopy + biopsy - crypt hypertrophy + villous atrophy

Question 87

Coeliac Mx

Answer 87

gluten free diet

Question 88

Biliary atresia

Answer 88

Bile duct narrowed / absent -> cholestasis, build up of conjugated bilirubin

Question 89

Biliary atresia Px

Answer 89

- jaundice after birth >14d (>21 if prem) - dark stools, pale urine - failure to thrive - HSM

Question 90

Biliary atresia Ix

Answer 90

- LFTs, bilirubin, serum A1AT - USS biliary tree

Question 91

Biliary atresia Mx

Answer 91

Surgery - Kasai portoenterostomy May need liver transplant

Question 92

Duodenal atresia

Answer 92

Downs, Vacterl association Blind end to duodenum Bilious vomiting USS - double bubble sign

Question 93

Small bowel atresia

Answer 93

may have multiple .....

Question 94

Intestinal obstruction causes

Answer 94

Meconium ileus Hirschsprung's oesophageal atresia intussusception imperforate anus malrotation + volvulus

Question 95

Obstruction Px

Answer 95

vomiting, bilious abdo pain, distension not passing wind / stools abnormal BS

Question 96

Obstruction Ix

Answer 96

Abdo XR - dilated loops proximal, collapsed loops distal

Question 97

Obstruction Mx

Answer 97

Surgery - laparotomy NBM, NG tube, IV fluids

Question 98

Hirschsprung's disease

Answer 98

congenital - absence of nerve cells in myenteric plexus (Auerbach's) in distal bowel / rectum Aganglionic cells do not relax - bowel remains constricted, obstructs faeces Hirschsprung-associated enterocolitis (HAEC) - infection with hirschsprung's

Question 99

Hirschsprung's Px

Answer 99

depends how much bowel affected acute intestinal obstruction after birth delay passing meconium >24hrs chronic constipation, abdo pain, distension failure to thrive

Question 100

Hirschsprung's Ix

Answer 100

AXR - obstruction Rectal biopsy

Question 101

Hirschsprung's Mx

Answer 101

IV fluids IV abx in HAEC Bowel washouts Surgery to remove affected bowel - pull through op / stoma

Question 102

Intussusception

Answer 102

Bowel telescopes Commonly ileocaecal 6mo-2yo, obstructs faeces

Question 103

Intussusception Px

Answer 103

Colicky abdo pain - draw legs up pale, lethargic, unwell redcurrant jelly stool - late sausage RUQ mass vomiting - milky, then green Obstruction

Question 104

Intussusception Ix

Answer 104

USS - target like mass Contrast enema

Question 105

Intussusception Mx

Answer 105

Enema - air insufflation surgery - laparotomy - reduction +/- resection

Question 106

Meckel's diverticulum

Answer 106

- diverticulum of ASI - remnant of a duct - 2% of pop, 2ft from IC valve, 2in long Px - abdo pain - painless PR bleed (may be massive) - obstruction Ix - Meckel's scan... - CT angio if severe Mx - surgical removal

Question 107

Appendicitis

Answer 107

inflammation of appendix

Question 108

Appendicitis Px

Answer 108

- Abdo pain - central, moving to RIF, tender in McBurney's point (1/3 from ASIS to umbilicus) - Anorexia - N+V - Rovsing's sign - palpation of LIF causes pain in RIF - Guarding - Rebound tenderness, percussion tenderness - Although - children more likely to present atypically

Question 109

Appendicitis Ix

Answer 109

Bloods, inc CRP ?CT USS Diagnostic laparoscopy

Question 110

Appendicitis Mx

Answer 110

Laparoscopic appendicectomy

Question 111

IBD

Answer 111

UC / Crohns - inflammation of walls of GIT - remission / exacerbation

Question 112

Crohn's features

Answer 112

- Entire GI tract - Skip lesions on endoscopy - Terminal ileum most affected - Full thickness of wall affected - transmural - Less commonly blood / mucus - Associated with wt loss, strictures, fistulas - Granuloma, fissures, fistula, abscesses, strictures

Question 113

UC features

Answer 113

- Continuous inflammation, colon and rectum only - Only superficial mucosa - Smoking protects - Blood + mucus - Primary sclerosing cholangitis associated

Question 114

IBD Px

Answer 114

Diarrhoea Abdo pain bleeding wt loss anaemia fever, malaise, dehydration finger clubbing, erythema nodosum, episcleritis, iritis, arthritis, PSC

Question 115

IBD Ix

Answer 115

Bloods Faecal calprotectin OGD + colonoscopy Imaging - USS / CT / MRI

Question 116

Crohn's Mx

Answer 116

Induce remission - oral pred / IV hydrocortisone - immunosuppressants Maintain remission - azathioprine / mercaptopurine Surgery - remove affected bowel, strictures, fistulas

Question 117

UC Mx

Answer 117

Induce remission - mild/mod - mesalazine, then pred - severe - IV hydrocortisone, IV ciclosporin Maintain remission - mesalazine, azathioprine, mercaptopurine Surgery remove bowel

Question 118

Colic

Answer 118

Inconsolable crying, 1wk-3/4mo R/o other causes - eg GI motility.... Mx reassure soothing strategies support - health visitor, friends

Question 119

Toddler's diarrhoea

Answer 119

1-5yo stool - foul smelling, watery, mucus, undigested veg Short mouth->anus transit time No failure to thrive Balanced diet to mx

Question 120

congenital diaphragmic hernia

Answer 120

herniation of abdo viscera into chest - incomplete formation of diaphragm can lead to pulmonary hypoplasia, resp distress after birth Ix USS, fetal MRI, CXR Mx Surgery

Question 121

Gastroschisis

Answer 121

Bowel out of abdo, through defect in abdo wall, no covering Mx May attempt vaginal delivery Surgery ASAP after

Question 122

Exomphalos / omphalocele

Answer 122

Abdo contents protrude through anterior abdo wall, covered by sac Mx C-section birth staged repair - infant needs to grow to fit bowel

Question 123

Hernias

Answer 123

Umbilical common Typically resolve by 3 years of age If large or symptomatic- elective repair age 2-4 If small and asymptomatic perform repair at 5 years old Inguinal indirect in children, urgent in neonates can't get above it, doesn't transilluminate

Question 124

Necrotising enterocolitis (NEC)

Answer 124

prem babies, necrotic bowel, perforation, peritonitis Px not feeding vomit, green bile unwell distended abdo, shock Ix Bloods AXR - dilated bowel loops, bowel wall oedema, pneumatosis intestinalis (gas in bowel wall) Mx NBM, IVF, TPN, abx, surgery to resect

Question 125

Malrotation + volvulus

Answer 125

anomaly of rotation of midgut (occurs in 1st trim) Px 24hrs old Green vomit, bile abdo distention blood / mucus in stools / bowels not opening pale not feeding Ix AXR, USS Mx IVF, laparotomy (Ladd's - division of Ladd bands, widen mesentry base)

Question 126

Hepatoblastoma

Answer 126

Malignant liver ca Px asym abdo mass poor appetite, wt loss, fever, vomit, jaundice Ix raised alpha-fetoprotein CXR, USS, CT / MRI Biopsy Mx Surgical resection chemo transplant

Question 127

Kwashiorkor

Answer 127

malnutrition - reduced protein Px oedema (reduced albumin) Enlarged liver (fatty) hair thinning, teeth loss anorexia, muscle atrophy, distended abdo Ix clinical dx Mx feed

Question 128

Marasmus

Answer 128

Malnutrition of all food groups Px wasting, loss of fat, hypothermia, pyrexia, anaemia, dehydration, dry skin, brittle hair Mx refeed slowly

Question 129

Choledochal cyst

Answer 129

Bile duct cyst -> obstruction + retention of bile Px intermittent abdo pain, jaundice, RUQ mass jaundice, vomiting, enlarged liver pale stools, dark urine Ix bloods, USS, CT, MRCP Mx Surgery

Question 130

Vaccines at birth

Answer 130

BCG if RFs present

Question 131

2mo

Answer 131

6 in 1 (diphtheria, tetanus, pertussis, polio, Haemophilus influenzae type B (Hib), hep B) Oral rotavirus vaccine Meningitis B

Question 132

3mo

Answer 132

6 in 1 vaccine Oral rotavirus vaccine Pneumococcal conjugate vaccine (PCV)

Question 133

4mo

Answer 133

6 in 1 vaccine Meningitis B

Question 134

12-13mo

Answer 134

Hib / Men C - one jab MMR PCV Men B

Question 135

2-10yo

Answer 135

Flu (annual)

Question 136

3yr4mo

Answer 136

4 in 1 (diphtheria, tetanus, pertussis, polio) MMR

Question 137

12-13yo

Answer 137

Human papillomavirus (HPV) - 2 jabs 6-24mo apart

Question 138

14yo

Answer 138

3 in 1 (diphtheria, tetanus, polio) Men ACWY

Question 139

Vaccination schedule

Answer 139

6in1 - 2mo, 3mo, 4mo 4in1 - 3yr4mo 3in1 - 14yo Oral rotavirus - 2mo, 3mo menB - 2mo, 4mo, 12mo PCV - 3mo, 12mo Hib/menC - 12/13mo MMR - 12mo, 3y4mo HPV - 12-13yo menACWY - 14yo

Question 140

NICE Febrile Child Traffic Light Red Flags

Answer 140

- pale, mottled, ashen, blue - no response to social cues - appears ill to healthcare professional - unarousable - weak, high-pitched, continuous cry - grunting - RR>60 - moderate / severe chest indrawing - reduced skin turgor - <3mo T>38 - non-blanching rash - bulging fontanelle - neck stiffness - status epilepticus - focal neurological signs - focal seizures

Question 141

Fever causes

Answer 141

Meningitis Encephalitis Pneumonia UTI Septic arthritis GE Kawasaki

Question 142

Fever Ix

Answer 142

- hydration status, obs, travel abroad - Sepsis bloods, urine dip + culture, CXR, NPA, stool culture - LP - if <1mo, 1-3mo + unwell, >3mo with red flag sx

Question 143

Fever Mx

Answer 143

- A-E - Abx (if <1mo, or 1-3mo unwell) - cefotaxime, ceftriaxone (add amoxicillin for listeria if <3mo) - fluid bolus - 10-20ml/kg

Question 144

Infectious mononucleosis (IM)

Answer 144

EBV infection 2-3wk acute illness, chronic fatigue, saliva spread

Question 145

IM Px

Answer 145

fever, sore throat, fatigue, lymphadenopathy, enlarged tonsils splenomegaly (rupture risk) itchy rash after amoxicillin

Question 146

IM Ix

Answer 146

- Heterophile ABs - not specific to EBV Ags - Monospot test - ABs react with RBCs from horses - Paul-Bunnell test - RBCs from sheep .... Specific AB tests - IgM/IgG

Question 147

IM Mx

Answer 147

Self limiting Avoid alcohol Avoid contact sports

Question 148

Mumps

Answer 148

Viral infection, spread by resp droplets 14-25d incubation, lasts 1wk MMR 80% protection

Question 149

Mumps Px

Answer 149

Flu prodrome Parotid swelling - uni/bilateral Earache, pain on eating fever, myalgia, lethargy, reduced appetite, headache Cx sx abdo pain - pancreatitis testicular pain/swelling - orchitis confusion, neck stiffness, headache - meningoencephalitis sensorineural hearing loss

Question 150

Mumps Mx

Answer 150

Saliva PCR Test blood / saliva for ABs PHE notifiable Supportive - rest, fluids

Question 151

Meningitis

Answer 151

infection of meninges Orgs by age group <3mo - GBS, E coli, listeria 1mo-6yo - N meningitidis, S pneumoniae, H influenzae >6yo - N meningiditis, S pneumoniae Meningococcal septicaemia - bacterial infection in bloodstream

Question 152

Meningitis Px

Answer 152

Fever, neck stiffness, headache, photophobia, reduced GCS, seizures, non-blanching rash, Kernig's/Brudzinski's Hypotonia, poor feeding, lethargy, hypothermia, bulging fontanelle

Question 153

Meningitis Ix

Answer 153

sepsis bloods, meningococcal PCR LP - <1mo + fever, 1-3mo unwell + fever, >1yo unexplained fever - bacteria - high protein, low glucose, high neutrophils - viral - normal protein, normal glucose, raised lymphocytes - CI with raised ICP - eg focal neurology, papilloedema, bulging fontanelles, DIC

Question 154

Meningitis Mx

Answer 154

Community / non-blanching rash - IM benzylpenicillin Abx - <3mo - cefotaxime + amoxicillin - >3mo - ceftriaxone or cefotaxime Dexamethasone >3mo - reduce hearing loss Acyclovir - if viral Ciprofloxacin - single dose for close contacts- PH notification

Question 155

Encephalitis

Answer 155

infection of brain Tends to be viral - HSV, VZV, EBV

Question 156

Encephalitis Px

Answer 156

altered consciousness focal neuro sx focal seizures fever

Question 157

Encephalitis Ix

Answer 157

LP (not if GCS<9, BP low, active seizures) CT / MRI EEG maybe Swabs HIV test

Question 158

Encephalitis Mx

Answer 158

acyclovir

Question 159

Chickenpox

Answer 159

primary VZV infection resp spread / shingles contagious for 4d before -> 5d after rash / pustules crust over 10-21d incubation

Question 160

Chickenpox Px

Answer 160

1-2d prodrome pruritic rash 6d - macular -> papular -> vesicular - on scalp, trunk, face, extremities fever, malaise, headache

Question 161

Chickenpox Ix

Answer 161

Clinical dx Tzanck smear - shows multinucleated giant epithelial cells PCR

Question 162

Chickenpox Mx

Answer 162

Supportive, trim nails Calamine lotion - for itch VZV Ig - for immunocompromised / newborns with peripartum exposure IV acyclovir if needed be aware secondary bacterial infection

Question 163

Impetigo

Answer 163

Superficial bacterial skin infection S aureus, S pyogenes may be bullous - fluid filled lesions direct contact spread, 4-10d incubation No school until all lesions healed / 48hrs after abx

Question 164

Impetigo Px

Answer 164

Usually where not covered by clothing - face, neck, hands, flexures erythematous -> macule -> vesicular/pustular -> rupture causes honey-crusted lesions

Question 165

Impetigo Ix

Answer 165

Clinical dx Swab exudate for MC+S

Question 166

Impetigo Mx

Answer 166

hydrogen peroxide 1st line fusidic acid topical abx oral flucloxacillin if bullous / extensive

Question 167

Hand, foot and mouth (HFM) disease

Answer 167

viral infection Coxsackie A16 commonly V contagious

Question 168

HFM Px

Answer 168

Sore throat fever oral ulcers vesicles on palms /soles of feet

Question 169

HFM Mx

Answer 169

Hydrate, analgesia Keep off school until feeling better

Question 170

Measles

Answer 170

RNA virus, spread by aerosol infective from prodrome until 4d after rash starts 10-14d incubation

Question 171

Measles Px

Answer 171

- Prodrome - irritable, conjunctivitis, fever - Koplik spots - white spots on buccal mucosa - Rash - behind ears -> whole body, maculopapular, blotchy/confluent, desquamation of palms/soles - diarrhoea

Question 172

Measles Ix

Answer 172

serum IgM ABs

Question 173

Measles Mx

Answer 173

Notifiable Sx relief Vaccine for unvaccinated contacts

Question 174

Measles Cx

Answer 174

Otitis media pneumonia encephalitis ...

Question 175

Rubella

Answer 175

Viral infection, german measles Droplet spread / direct contact

Question 176

Rubella Px

Answer 176

- rash - face/neck -> body, pink, maculopapular, itchy, 3-5d - lymphadenopathy - arthritis - fever, headache, malaise, nausea, URTI, conjunctivitis

Question 177

Rubella Ix

Answer 177

Lab ix to confirm....

Question 178

Rubella Mx

Answer 178

- Notifiable - rest, sx control, off school for >5d after rash starts

Question 179

Diphtheria

Answer 179

G+ bacteria, vaccine to prevent releases exotoxin

Question 180

Diphtheria Px

Answer 180

- visit to high risk country - sore throat + diphtheric membrane - grey - cervical lymphadenopathy - bull neck - systemic spread - diaphragm paralysis

Question 181

Diphtheria Ix

Answer 181

Throat swab culture

Question 182

Diphtheria Mx

Answer 182

IM penicillin Diphtheria antitoxin

Question 183

Scarlet fever

Answer 183

Reaction to GAS toxins (often S pygenes) spread by resp droplets risk of invasive GAS (iGAS) - extremes of age, immunocompromised, IVDU...

Question 184

Scarlet fever Px

Answer 184

- 2-4d incubation, infectious for 2-3wks w/o tx - fever (24-48hrs), malaise, headache, N+V - sore throat - strawberry tongue - sandpaper rash - fine, punctate erythema - torso -> everywhere - spares palms, soles - then desquames - cervical lymphadenopathy

Question 185

Scarlet fever Ix

Answer 185

Throat swabs / blood test - not routine

Question 186

Scarlet fever Mx

Answer 186

Pen V 10d (azithromycin if allergy) Off school for 24hrs after starting abx Notifiable

Question 187

Scarlet fever Cx

Answer 187

Otitis media Rheumatic fever Glomerulonephritis iGAS

Question 188

Slapped cheek syndrome

Answer 188

Aka erythema infectiosum / fifth disease Parvovirus B19 resp spread

Question 189

Slapped cheek Px

Answer 189

- asym - prodrome - fever, malaise, headache, myalgia - diffuse rash develops - arthralgia

Question 190

Slapped cheek Ix

Answer 190

If immunocompromsed - bloods to confirm Dx

Question 191

Slapped cheek Mx

Answer 191

Rash can come + go Sx relief not infectious after 1d rash risk of fetal death <20wks gestation

Question 192

Staphylococcal scalded skin syndrome (SSSS)

Answer 192

type of S aureus produces epidermolytic toxins - causes separation of epidermal skin

Question 193

SSSS Px

Answer 193

- erythema patches - bullae - blisters - rubbing skin causes peeling - fever, irritable, lethargy, malaise

Question 194

SSSS Mx

Answer 194

- admit - IV abx - fluids - analgesia

Question 195

Polio

Answer 195

poliovirus, faeces / resp spread Vaccine to prevent Px - sore throat, fever, fatigue, nausea, headache, abdo pain - sx 2-5d - poliomyelitis - paralysis

Question 196

Candida

Answer 196

fungal infection Px - oral thrush - candida oesophagitis - systemic - fever, sepsis, rash, pain, headache, neuro sx Ix - KOH smear test - blood / tissue culture - endoscopy - Blood Ag detection - candida Mx - topical clotrimazole - fluconazole - topical / vaginal - systemic - IV caspofungin

Question 197

Toxic shock syndrome

Answer 197

severe systemic reaction to exotoxins S aureus, strep pyogenes Px - fever >39 - hypotension - diffuse erythematous maculopapular rash - >3 organ systems involved - desquamation after 1-2wks of palms, soles Mx - remove infection focus (eg tampon, surgical debridement) - IV fluids - IV abx - IV Ig maybe

Question 198

Molluscum contagiosum

Answer 198

virus, skin infection direct contact spread resolve alone Px - small, flesh coloured papules, central dimple - appear in crops Mx - dont share towels - if bacterial superinfection - abc, topical fusidic acid / oral fluclox - specialist tx - topical KOH, benzoyl peroxide, surgical removal

Question 199

Head lice

Answer 199

Pediculus capitis direct head-to-head contact Dx seen on fine tooth combing of hair Mx - malathion, wet combing, dimeticone......

Question 200

Hearing loss

Answer 200

Causes - congenital - rubella, CMV, genetic, Down's - perinatal - prem, hypoxia - post-natal - jaundice, meningoencephalitis, OM, chemo Px Not hearing Ix OAE at birth Response to sound Audiometry if older Mx MDT - hearing aids, SALT, ...

Question 201

Cleft lip + palate

Answer 201

Cleft lip - split section of upper lip Cleft palate - defect in hard/soft palate at roof of mouth Cx problems feeding, swallowing, speech... Mx Refer to cleft lip + palate services Teats to eat/drink Surgery - cleft lip 3mo - cleft palate 6-12mo

Question 202

Tongue tie

Answer 202

baby born with short frenulum - cannot latch onto breast Px poor feeding - noticed by mum / midwife Mx frenotomy

Question 203

Cystic hygroma

Answer 203

Cyst filled with lymphatic fluid Px Posterior triangle of left neck (pos. SCM) large, soft, painless transilluminates hypoechoic Mx w+w aspiration surgery

Question 204

Thyroglossal cyst

Answer 204

persistence of thyroglossal duct (where thyroid travelled down in development) - fluid filled Px midline neck, below hyoid painless, soft moves with tongue painful if infected anechoic Ix USS / CT Mx surgical removal

Question 205

Branchial cyst

Answer 205

malformation of 2nd branchial cleft Px Anterior triangle (ant SCM) non-tender, smooth, round no movt on swallowing no transillumination Ix US, aspiration Mx Conservative Surgery if recurrent infections

Question 206

Dermoid cyst

Answer 206

teratoma of cystic nature Px midline of neck, above hyoid Ix heterogenous on imaging

Question 207

Infantile hemangioma

Answer 207

Either triangle of neck grow rapidly. XR - mass lesion, fat.....

Question 208

Periorbital cellulitis

Answer 208

infection of soft tissues anterior to orbital septum - eyelids, skin, subcut tissue Orbit / septum not affected From breaks in skin, sinusitis, RTIs - S aureus, Staph epidermis, Strep, anaerobic bacteria

Question 209

Periorbital cellulitis Px

Answer 209

Red, swollen, painful eye, acute onset fever erythema of eyelid / skin ptosis normal eye movt, vision, reflexes

Question 210

Periorbital cellulitis Ix

Answer 210

Bloods - raised CRP Swab discharge Contrast CT - to see if peri/orbital

Question 211

Periorbital cellulitis Mx

Answer 211

Refer to ophthal Drain abscesses Oral co-amox

Question 212

Orbital cellulitis

Answer 212

infection of fat / muscles posterior to orbital septum, within orbit, not involving globe after URTI of sinuses

Question 213

Orbital cellulitis Px

Answer 213

As periorbital + visual disturbance absent light reflex proptosis ophthalmoplegia drowsy

Question 214

Orbital cellulitis Ix

Answer 214

Orbital cellulitis Ix

Question 215

Orbital cellulitis Mx

Answer 215

Admit IV abx

Question 216

Squint

Answer 216

misalignment of eyes, aka strabismus Pt experiences double vision lazy eye -> becomes passive, reduced function -> amblyopia Concomitant squint - imbalance in extra-ocular muscles, convergent more common than divergent Paralytic squint - paralysis in extra-ocular muscles (rare) Esotropia - inward squint (affected eye in) Exotropia - outward squint (affected eye out) Hypertropia - upward moving affected eye Hypotropia - downward moving affected eye Manifest strabismus - there all the time Latent strabismus - appears when each eye covered

Question 217

Squint causes

Answer 217

Idiopathic Refractive error Hydrocephalus CP SOL - retinoblastoma Trauma

Question 218

Squint examination

Answer 218

Eye movts, fundoscopy, acuity Hirschberg's test / corneal light reflection test - shine pen torch at pt 1m away, look for symmetrical + central reflection of light source - normal Cover test - cover one eye, ask pt to focus on object in front, move cover to opposite eye - if first eye moves inwards, it was exotropia when covered, if it moves outwards, it was esotropia when covered

Question 219

Squint Mx

Answer 219

Start tx <8yo Occlusive patch Atropine drops - blur vision in good eye Glasses

Question 220

Retinoblastoma

Answer 220

ocular malignancy, autosomal dominant Avg age 18mo at dc Px absent red-reflex - white pupil strabismus visual problems Mx enucleation - remove whole eye external beam radiation therapy, chemo, photocoagulation

Question 221

UTIs

Answer 221

infections in urethra, bladder, ureters, kidneys pyelo -> scarring, reduction in function <3mo M>F, >3mo F>M Causes E coli, proteus, pseudomonas

Question 222

Atypical UTIs

Answer 222

Seriously ill Poor urine flow Abdo / bladder mass Raised creatinine Septicaemia Failure to respond to abx in 48hrs Non-E coli organisms

Question 223

Recurrent UTI

Answer 223

- 2+ episodes of UTI with pyelonephritis - 1 episode pyelonephritis + lower UTI - 3+ episodes lower UTI

Question 224

UTI Px

Answer 224

Fever Babies - lethargy, irritable, vomiting, poor feed, urinary frequency Older children - suprapubic pain, vomiting, dysuria, urinary frequency, incontinence, new bedwetting Dark cloudy smelly urine, haematuria, abdo pain, shivers

Question 225

UTI Ix

Answer 225

Septic screen - if unwell Urine dipstick (clean catch) - N + L = UTI - N = UTI - L = maybe UTI, tx if sx - neither = no UTI MC+S if either present Renal USS - look at anatomy For <6mo with UTI, recurrent / atypical DMSA scan - dimercaptosuccinic acid After 4-6mo to assess for scarring MCUG - micturating cystourethrogram Look for VUR

Question 226

UTI Mx

Answer 226

Pyelonephritis <3mo - IV abx >3mo - oral cefalexin, co-amoxiclav - cefuroxime / ceftriaxone / gentamicin if needed Lower UTI <3mo - IV cefuroxime >3mo - trimethoprim / nitrofurantoin, amoxicillin / cefalexin second line

Question 227

Vesico-ureteric reflux (VUR)

Answer 227

abnormal backflow of urine from bladder into ureter / kidney Malfunction of VUJ Px Recurrent UTIs Hydronephrosis on USS antenatal Reflux nephropathy - chronic pyelo, scarring Ix MCUG DMSA Mx Surgery

Question 228

Nephrotic syndrome

Answer 228

Podocyte damage - GBM permeable to proteins 2-5yo Triad - low albumin <25g/L, >3+ protein on dipstick, oedema Steroid sensitive / resistant Also - high cholesterol, increased BP, hypercoagulable, infections

Question 229

Nephrotic syndrome causes

Answer 229

Minimal change disease Most commonly No underlying condition, no abnormality on biopsy intrinsic kidney disease FSGS MPG Systemic illness HSP, DM, HIV, hepatitis, malaria

Question 230

Nephrotic syndrome Px

Answer 230

Frothy urine generalised oedema - face - gravitational pallor

Question 231

Nephrotic syndrome Ix

Answer 231

- Urine dip - 3+ protein - Urine protein:creatinine ratio - Serum albumin <20-25 - U/E, bone profile, FBC, VZV immunity - BP - assess CV status - fluid overload, hypovolaemia

Question 232

Nephrotic syndrome Mx

Answer 232

- Prednisolone - 4ks high dose, 8wks weaning, 80% respond - No added salt diet - Furosemide + spironolactone - for oedema - severe - albumin infusion - Pen V abx prophylaxis - Pneumococcal vaccine - ACEi for HTN - Immunosuppressants if steroid resistant

Question 233

Nephrotic syndrome Cx

Answer 233

Hypovolaemia Thrombosis - loss of factors that prevent clotting Infection - loss of Igs Renal failure RAAS activated High cholesterol - liver in overdrive Relapse

Question 234

Haemolytic uraemic syndrome (HUS)

Answer 234

Thrombosis in small vessels Triggered by shiga toxin - E coli -157, shigella Triad of: - microangiopathic haemolytic anaemia - RBCs churned by clots - AKI - retain urea - thrombocytopenia - low platelets

Question 235

HUS Px

Answer 235

GE, then HUS sx ~5d later Fever Reduced UO Haematuria, dark-brown urine Abdo pain Lethargy, irritability Confusion Oedema HTN Bruising Jaundice (from haemolysis)

Question 236

HUS Ix

Answer 236

FBC - anaemia, negative Coombs Blood film - schistocytes, helmet cells U/E - AKI Stool culture - look for shigella, PCR for toxins

Question 237

HUS Mx

Answer 237

Self-limiting Renal dialysis anti-HTN, fluids, blood if needed Eculizumab maybe

Question 238

Enuresis

Answer 238

Involuntary urination Nocturnal enuresis - bedwetting at night, normally controlled by 7(?)yo Diurnal enuresis - wetting during day, normally controlled by 2yo

Question 239

Primary nocturnal enuresis

Answer 239

child has never been dry at night Causes - normal development - overactive bladder - fluids before bed - stress, pressure at home - chronic constipation, UTI, LD, CP Ix - diary of toileting, fluid intake, bed wetting Mx - +ve reinforcement - Enuresis alarms - desmopressin (ADH analogue) - oxybutynin - anticholinergic - imipramine - TCA, relaxes bladder

Question 240

Secondary nocturnal enuresis

Answer 240

Child wets bed after being dry for >6mo - ?illness Causes UTI, constipation, T1DM, new psychological stress, maltreatment / abuse Mx Tx cause

Question 241

Diurnal enuresis

Answer 241

Urinary incontinence during day, F>M Stress / urge incontinence Recurrent UTIs, psychosocial issues, constipation

Question 242

Wilm's tumour

Answer 242

Nephroblastoma - common childhood malignancy, typically <5yo Px - Abdo mass - painless haematuria - flank pain - anorexia, fever, wt loss - HTN, lethargy - 95% unilateral - mets in 20% (commonly lung) Ix - USS, CT, MRI, biopsy Mx - nephrectomy - chemo - radiotherapy

Question 243

Hypospadias

Answer 243

congenital penis abnormality - ventral urethral meatus - hooded prepuce (foreskin) - chordee (ventral curvature of penis) associated with cryptorchidism Px see on newborn baby check abnormal urine stream Mx surgery at 1yo do not circumcise child - foreskin used in correction

Question 244

Phimosis

Answer 244

Non-retractile foreskin <2yo normal - physiological phimosis Normally can retract by 10yo Pathological phimosis - from recurrent foreskin infection Px Ballooning during micturition painful erections haematuria recurrent UTIs swelling, red, tender Ix Can take swab Mx Reassure, clean Topical steroids Surgery - divide frenulum / circumcision

Question 245

Paraphimosis

Answer 245

Foreskin retracted, unable to be replaced RFs Tight foreskin, scarring, vigorous sexual activity, piercing, catheter insertion Px oedema, pain on erection, irritable, eventually necrotic Mx gentle compression with saline-soaked swab Manual reduction Ice, squeezing, 50% dextrose Refer to urology

Question 246

Balanitis xerotica obliterans (BXO)

Answer 246

Lichen sclerosis of male genitalia - chronic inflammation + scarring Px ballooning during micturition scarring irritation dysuria white, fibrotic, scarred preputial tip Mx circumcision send foreskin to histopathology to confirm dx

Question 247

Cryptorchidism

Answer 247

Undescended testes 5% babies at birth, 25% cases bilateral RFs Prem, low birth weight, hypospadias, FHx, maternal smoking in pregnancy Correct to reduce infertility risk, avoid testicular torsion Px no testis on palpation of scrotum Testes in inguinal canal or elsewhere DDx hormonal - AIS, disorders of sex development retractile testes Mx - w+w - most descend by 3-6mo - review at 6-8wks, then again at 3mo - see surgeon before 6mo - try to move down if in inguinal canal - orchidopexy - surgical correction, 6-12mo - if bilateral undescended - senior paeds review in 24hrs

Question 248

Torsion of appendix of testis

Answer 248

Hydatid of Morgagni Mimics testicular torsion Pain not as severe Blue dot sign NSAIDs to tx

Question 249

Febrile convulsions

Answer 249

- Seizure + high fever - 6mo-5yo - simple - generalised tonic clonic <15mins - complex - partial / focal, >15mins Ix - exclude DDx - epilepsy, meningoencephalitis, SOL, syncope, electrolyte abnormalities, trauma - find infection source - chest, GI, UTI, ears, throat Mx - paracetamol / ibuprofen - tx infection source

Question 250

Cerebral palsy (CP)

Answer 250

disorder of movt + posture due to non-progressive lesion of motor pathways permanent neurological sx resulting from damage to brain around time of birth sx manifest during growth + development

Question 251

CP causes

Answer 251

Antenatal - Maternal infections, eg rubella, toxoplasmosis, CMV - Trauma during pregnancy Perinatal - Birth asphyxia - Pre-term birth Postnatal - Meningitis - Severe neonatal jaundice - Head injury

Question 252

CP Types

Answer 252

Spastic / pyramidal - Damage to UMN, hypertonia, reduced function - Patterns - monoplegia (one limb), hemiplegia (one side), diplegia (4 limbs, mostly legs), quadriplegia (4 limbs severely affected + seizures, speech disturbance etc) Dyskinetic / athetoid / extrapyramidal - Problems controlling movt ...muscle tone - hypertonia + hypotonia - Athetoid movts (slow, involuntary writhing), oro-motor problems - Damage to basal ganglia + substantia nigra Ataxic - Damage to cerebellum - problems with coordinated movt Mixed - Mix of all

Question 253

CP Px

Answer 253

- milestone failure - abnormal tone - hand preference <18mo - problems with coordination, speech, walking, feeding, swallowing - LDs - tiptoeing gait - scissoring gait - Epilepsy, squint, hearing impairment

Question 254

CP Ix

Answer 254

- neuro exam - CT / MRI - EEG, EMG, bloods

Question 255

CP Mx

Answer 255

- MDT - OT/PT, SALT, dieticians, orthopaedics (lengthen tendons, release contractures) - meds - oral diazepam, baclofen, AEDs, glycopyrronium, botox

Question 256

Breath holding spells

Answer 256

Involuntary episodes of holding breath, outgrow by 4-5yo cyanotic breath holding spells - upset, worked up, crying, stop breathing, LOC 1min, then breathe Reflex anoxic seizures - startled - vagus -> drop HR - LOC, seizure like - 30s, regain consciousness - exclude DDx, reassure

Question 257

Childhood epilepsy

Answer 257

Transient episodes of abnormal electrical activity in the brain Ix - EEG, bloods, MRI brain, ECG, genetic / AB testing if needed

Question 258

Generalised tonic clonic

Answer 258

- LOC, tonic + clonic - tongue biting, incontinence, groaning, irregular breathing - post-ictal Mx - sodium valproate - boys - lamotrigine / levetiracetam - girls / 2nd line for boys

Question 259

Focal seizures

Answer 259

- tend to start in temporal lobes - hearing, speech, memory, emotions affected Px - hallucinations, memory flashbacks, deja vu, autopilot Mx - 1st - lamotrigine / levetiracetam - 2nd - carbamazepine

Question 260

Absence seizures

Answer 260

- stare into space, blank, unresponsive 10-20s Ix - EEG - bl synchronous/symmetrical 3Hz spike + wave discharges Mx - ethosuximide - sodium valproate (2nd line for boys) - lamotrigine / levetiracetam (3rd line) If absence seizures + other types - valproate - 1st line boys - lamotrigine / levetiracetam - 1st line girls - then other 1st line drugs not tried - then ethosuximide add on

Question 261

Atonic seizures

Answer 261

- drop attacks - lapse in muscle tone - may indicate Lennox-Gestaut syndrome Mx - valproate - 1st line M - lamotrigine - 1st line F, 2nd line M - clobazam - 2nd line F

Question 262

Myoclonic seizures

Answer 262

- sudden brief muscle contractions, pt awake - may indicate juvenile myoclonic epilepsy - within 1-2hrs waking - clumsy mornings Mx - valproate - 1st M - Levetiracetam - 1st F, 2nd M - clobazam.....2nd F

Question 263

Infantile spasms

Answer 263

- West syndrome - often associated with tuberous sclerosis - serious Px - salaam attack - flexion of head, trunk, arms, extension of arms - lasts 1-2s, repeated <50x - developmental delay Ix - sleep EEG - hypsarrhythmia - CT head mx - vigabatrin - pred if not TS

Question 264

Benign Rolandic epilepsy

Answer 264

- 4-12yo Px - seizures at night - facial paraesthesia, can develop into secondary tonic clonic Ix - EEG - centro-temporal spikes Mx - lamotrigine / levetiracetam if needed - good prognosis

Question 265

Lennox-Gastaut syndrome (LGS)

Answer 265

- an epileptic encephalopathy, onset 3-5yo - different seizure types, intellectual delay, behavioural problems Mx - valproate - for all - lamotrigine 2nd line

Question 266

Dravet syndrome

Answer 266

- frequent myoclonic + myoclonic-atonic seizures Mx - levetiracetam / sodium valproate - ketogenic diet

Question 267

AEDs

Answer 267

- Sodium valproate - teratogenic - only for use in boys, girls <10 who are unlikely to need tx when older, women who cannot have children - Carbamazepine - agranulocytosis - Phenytoin - folate / vit D deficiency - Ethosuximide - night terrors - Lamotrigine - Stevens-Johnson syndrome

Question 268

Tension headache

Answer 268

Triggers - stress, skipping meals, dehydration, infection Px - last 30mins - 7d - symmetrical ache, like band around head - no visual changes Mx - analgesia, hydrate, avoid stress / triggers

Question 269

Migraines

Answer 269

- most common headache in children Px - 4-72hr attack - unilateral, pain, throbbing, aura, photophobia, phonophobia, N+V Mx - paracetamol / ibuprofen - sumatriptan >12yo - domperidone antiemetic - prophylaxis - propranolol (avoid asthmatics), pizotifen (drowsiness), topiramate (teratogenic), valproate

Question 270

Cluster headaches

Answer 270

uncommon in children as for adults

Question 271

Hydrocephalus

Answer 271

Build up of CSF in brain / spinal cord From overproduction / reduced absorption / drainage

Question 272

Hydrocephalus congenital causes

Answer 272

- cerebral aqueduct stenosis - narrow, doesn't drain - arachnoid cysts - block CSF outflow - Arnold-Chiari malformation - chromosomal / congenital malformations

Question 273

Hydrocephalus Px

Answer 273

- increased head circumference - bulging anterior fontanelle - poor feeding, vomiting - poor tone - sleepiness

Question 274

Hydrocephalus Mx

Answer 274

VP shunt

Question 275

Brain tumours

Answer 275

- tend to be primary in paeds - pilocytic astrocytoma, medulloblastoma, ependymoma Px - headache, worse lying down - vomiting - papilloedema - squint, nystagmus, ataxia - personality change Ix - CT / MRI Mx - surgical resection - VP shunt - Chemo (many drugs don't cross BBB) - radio

Question 276

Neuroblastoma

Answer 276

Tumour of neural crest tissue of adrenal medulla and sympathetic NS Px - abdo mass - pallor, wt loss, bone pain, hepatomegaly - paraplegia - proptosis - lung mets - resp sx DDx - hepatoblastoma, Wilms, lymphoma/leukaemia, sarcoma, PKD Ix - raised urinary VMA + HVA levels - AXR (calcification), CT, biopsy Mx - surgery - chemo / radio

Question 277

Craniosynostosis

Answer 277

- skull sutures close early - abnormal head shapes, restricts brain growth - leads to raised ICP Px - abnormal head shape, small - anterior fontanelle closed by 1yo Ix - Skull XR, CT head Mx - mild - monitor - severe - surgical recon

Question 278

Plagiocephaly and brachycephaly

Answer 278

plagio - flattening of one area of head Brachy - flattening at back of head Mx - reassure - place on other side

Question 279

Duchenne muscular dystrophy (DMD)

Answer 279

- x-linked recessive - dystrophin genes affected - weakening + wasting of muscles Px - progressive proximal muscle weakness from 3-5yo - calf pseudohypertrophy - gower's sign - use arms to stand from seated - intellectual impairment Ix - CK raised - genetic testing - definitive (replaced muscle biopsy) Mx - supportive - OT/PT - wheelchairs / braces - ?oral steroids

Question 280

Spinal muscular atrophy (SMA)

Answer 280

- autosomal recessive - progressive loss of motor neurons - muscular weakness - LMNs in spinal cord affected -> LMN signs Mx - MDT - OT/PT - splints, braces, wheelchair - resp support, PEG....

Question 281

Down's syndrome

Answer 281

3 copies of c21 - trisomy 21

Question 282

Down's screening

Answer 282

Offer to all women - to indicate if further invasive tests needed Combined test 11-14wks - first line - Combine USS / maternal blood tests - high nuchal translucency, high bhCG, low PAPPA Triple test - 14-20wks - only maternal bloods - high bhCG, low AFP, low oestriol Quadruple test - as above + high inhibin A

Question 283

Down's antenatal testing

Answer 283

Chorionic villus sampling (CVS) - US-guided biopsy of placental tissue, <15wks - karyotype fetal cells Amniocentesis - US-guided aspiration of amniotic fluid - later in pregnancy - karyotype fetal cells Non-invasive pre-natal testing (NIPT) - blood test from mother - not definitive

Question 284

Down's Px

Answer 284

- Face - upslanting palpebral fissures (gaps between lower and upper eyelid), epicanthic folds (folds of skin covering medial portion of eye and eyelid), Brushfield spots in iris, protruding tongue, small low-set eats, round / flat face - Flat occiput - brachycephaly - Single palmar crease, pronounced 'sandal gap' between big and next toe - Hypotonia - Congenital heart defects (40-50%) - Duodenal atresia - Hirschsprung's disease

Question 285

Down's Ix

Answer 285

- TFTs every 2yrs - ECHO - regular audiometry - regular eye checks

Question 286

Down's Mx

Answer 286

- MDT - eg SALT, OT/PT, dietician, paediatrician, GP.....

Question 287

Down's Cx

Answer 287

- Cardiac - 1/3 - ASD, VSD, TOF, PDA - subfertility - LDs - Myopia, strabismus, cataracts - repeated resp infections - hearing impairment from recurrent OM - ALL - hypothyroid, Alzheimers - atlantoaxial instability

Question 288

Patau syndrome

Answer 288

- trisomy 13 - microcephaly, small eyes, cleft L+P, polydactyly, scalp lesions, LD - 90% die <1yo - neuro/heart defects

Question 289

Edward's syndrome

Answer 289

- trisomy 18 - micrognathia, low-set ears, rocker bottom feet, overlapping fingers, small baby, LD - 95% do not result in live birth - 50% do not survive past 1wk

Question 290

Fragile X syndrome

Answer 290

- trinucleotide repeat disorder - mutation in FMR1 -> lack of cognitive development - x-linked - LD, macrocephaly, long face, large ears, macro-orchidism, ADHD, autism

Question 291

Noonan syndrome

Answer 291

- male Turner's - caused by a number of genes - webbed neck, pectus excavatum, short stature, PS, downward sloping eyes, ptosis, widely spaced nipples

Question 292

Pierre-Robin syndrome

Answer 292

- micrognathia, posterior tongue displacement, cleft palate

Question 293

Prader-Willi syndrome

Answer 293

- loss of genes on arm of c15 - hypotonia, hypogonadism, obesity, dysmorphic, LD, anxiety

Question 294

William's syndrome

Answer 294

- deletion of genes on c7 - random (not inherited) - short, LD, friendly + extroverted, transient neonatal hypercalcaemia, supravalvular AS, starburst eyes,

Question 295

Cri du chat

Answer 295

- cry, feeding difficulties, LD, microcephaly, micrognathia....

Question 296

Klinefelter syndrome

Answer 296

- male has additional X - 47XXY Px - taller, wide hips, gyne, weaker muscles, smaller testicles, lower libido, shy, infertile, subtle LDs Ix - high gonadotrophins, low testosterone - karyotype for dx Mx - testosterone injections - IVF for infertility - breast reduction

Question 297

Turner syndrome

Answer 297

- female with one X - 45XO Px - short, webbed neck, broad chest, wide nipples, cubitus valgus, underdeveloped ovaries, late / incomplete puberty, infertile, downward sloping eyes, ptosis Mx - GH replacement - oestrogen + progesterone replacement - fertility tx

Question 298

Marfan syndrome

Answer 298

- autosomal dominant - fibrillin affected - abnormal connective tissue Px - tall, long neck/limbs/fingers (arachnodactyly), high arch palate, hypermobile, pectus carinatum/excavatum, downward palpable fissures, pes planus Associations - eye issues, joint dislocations, scoliosis, PTX, GORD, aortic aneurysm/dissection, aortic regurg Ix - thumb across palm - fingers around wrist Mx - avoid cardiac cx - exercise, caffeine, give BB/ACEi - physiotherapy - yearly echo / ophthal review

Question 299

Angelmann syndrome

Answer 299

- loss of function of gene (?deletion) - LD, speech dev delay, ataxia, happy, laughing, abnormal sleep, epilepsy, wide mouth, widely-spaced teeth, short broad skull

Question 300

T1DM

Answer 300

pancreas stops producing insulin may be genetic / triggered by virus

Question 301

T1DM Px

Answer 301

- DKA - Polyuria, polydipsia, weight loss - Secondary enuresis (bedwetting in previously dry child) - Recurrent infections - 4 Ts - toilet, thirsty, tired, thinner

Question 302

T1DM Ix

Answer 302

Bloods - FBC, U/E, lab glucose, blood cultures (if fever), HbA1c, - TFTs and thyroid peroxidase antibodies (TPO) for autoimmune thyroid disease - anti-TTG (coeliac) - insulin ABs, anti-GAD ABs and islet cell ABs (check for destruction of pancreas)

Question 303

T1DM Mx

Answer 303

S/C insulin - basal/bolus - Lantus long acting, once nightly - actrapid short acting 30 mins before carbs - insulin pump alternative

Question 304

T1DM monitoring

Answer 304

- HbA1c every 3-6mo - BMs on waking, at each meal, before bed - Flash glucose monitoring, eg FreeStyle Libre

Question 305

T1DM Cx

Answer 305

Lipodystrophy Hypos Hypers, DKA Macrovascular / microvascular Infections etc

Question 306

DKA

Answer 306

high ketones, hyperglycaemia, acidosis First px, or miss insulin dose

Question 307

DKA Px

Answer 307

vomiting, abdo pain, reduced GCS, dehydration, pear drop breath, Kussmauls

Question 308

DKA Ix

Answer 308

Bloods - BM, ketones, gas, FBC, U/E, lab BM, CRP, cultures, LFTs, HbA1c, TFTs, coeliac screen

Question 309

DKA classification

Answer 309

pH 7.2-7.29 +/- bicarb <15 - mild DKA - 5% fluid deficit pH 7.1-7.19 +/- bicarb <10 - moderate DKA - 7% fluid deficit pH <7.1 +/- bicarb <5 - severe DKA - 10% fluid deficit

Question 310

DKA Mx

Answer 310

A-E Fluids - 0.9% NaCl 20ml/kg bolus if shocked (up to 2x further 10ml/kg boluses if needed) - not shocked, but needs fluids - 10ml/kg over 60 mins (subtract from deficit) - Deficit (ml) - %dehydration x weight (kg) x 10 - over 48hrs - maintenance - 100/50/20 rule - over 24hrs - Add deficit and maintenance (x2) - for total requirement over 48hrs Insulin - 1-2hrs after IV fluids - 50 units actrapid in 50ml 0.9%NaCl rate of 0.05-0.1units/kg/hr - change to S/C once ketones <1 - continue long-acting insulin during Monitoring - fluid chart, BMs, ketones, obs, neuro obs - 2x daily weights....

Question 311

DKA Cx

Answer 311

cerebral oedema - hypertonic saline / mannitol

Question 312

Adrenal insufficiency (AI)

Answer 312

adrenal glands do not produce enough steroid hormones - cortisol / aldosterone

Question 313

AI causes

Answer 313

Primary - adrenal glands damaged - TB - Autoimmune - Addisons Secondary - low ACTH, low cortisol - Congenital hypoplasia of pituitary gland - surgery, infection.... Tertiary - lack of CRH from hypothalamus - long term steroids - suppresses axis

Question 314

AI Px

Answer 314

Babies - lethargy, vomiting, poor feeding, hypoglycaemia, jaundice, failure to thrive Older children - N+V, poor weight gain, weight loss, anorexia, abdo pain, muscle weakness/cramps, dev delay, bronze pigmentation (Addison's)

Question 315

AI Ix

Answer 315

- U/E - low Na, high K - BMs - hypoglycaemia - Primary AI - low cortisol, high ACTH, low aldosterone, high renin - Secondary AI - low cortisol, low ACTH, normal aldosterone, normal renin - Short synacthen test - failure of rise in cortisol in response to synacthen - primary AI

Question 316

AI Mx

Answer 316

Hydrocortisone Fludrocortisone Increase dose if sick

Question 317

Addisonian crisis

Answer 317

- acute severe px of addisons - eg triggered by infection, trauma - Px - reduced GCS, hypotension, low BM, low Na, high K - Mx - A-E, IV hydrocortisone, IV fluids, correct BMs

Question 318

Congenital adrenal hyperplasia (CAH)

Answer 318

- deficiency in 21-hydroxylase enzyme -> low aldosterone, low cortisol, high testosterone - enzyme converts progesterone -> aldosterone + cortisol - therefore lack of these - progesterone only converted into testosterone - high

Question 319

CAH Px

Answer 319

Severe - ambiguous genitalia - F have virilised genitalia (large clitoris) - low Na, high K, low BMs - poor feeding, dehydration, vomiting, arrhythmias Mild - px after puberty - F - tall for age, facial hair, absent periods, deep voice, early puberty - M - tall, deep voice, large penis, small testicles, early puberty - hyperpigmentation - high ACTH

Question 320

CAH Ix

Answer 320

High serum 17-hydroxyprogesterone ....

Question 321

CAH Mx

Answer 321

- refer to paeds endo - hydrocortisone, fludrocortisone - surgery for genitalia

Question 322

GH deficiency

Answer 322

due to pathology at hypothalamus / pituitary Causes - congenital - mutation - empty sella syndrome - underdeveloped / damaged pit gland - acquired - trauma, infection.... Patho - may have other pituitary hormone deficiencies

Question 323

GH deficiency Px

Answer 323

- birth - micropenis, hypoglycaemia, severe jaundice - poor growth, short, slow movt/strength dev, delayed puberty

Question 324

GH deficiency Ix

Answer 324

- GH stimulation tests - lack of GH rise in response to meds that stimulate release (eg glucagon, insulin) - ix for thyroid / adrenal issues - MRI brain, genetic testing - XR wrist / DEXA san - bone age

Question 325

GH deficiency Mx

Answer 325

Daily S/C somatropin (GH) monitor H+W

Question 326

Hypothyroidism

Answer 326

underactive thyroid congenital - not formed, doesn't make enough, or from pit/hypo problem - screen for on newborn blood spot acquired - autoimmune

Question 327

Hypothyroidism Px

Answer 327

Congenital - prolonged neonatal jaundice - poor feeding, constipation, sleepy, reduced activity, slow growth - short, hypotonia, puffy face, macroglossia Acquired - fatigue, poor growth, wt gain, poor school performance, constipation, dry skin/hair loss

Question 328

Hypothyroidism Ix

Answer 328

TFTs - TSH, T3/4 Bloods for thyroid ABs Thyroid USS Heel-prick screening

Question 329

Hypothyroidism Mx

Answer 329

Levothyroxine OD

Question 330

Obesity

Answer 330

- use BMI centile charts causes poor diet, lack of exercise, low SES, GH deficiency, hypothyroid, Down's, Cushing's, Prader-Willi Assessment - explore lifestyle / stress - urine dip, protein/glucose, check BP, measure lipids / HbA1c Mx - weight maintenance / reduction of rate of gain - diet + exercise - cognitive approach - no orlistat

Question 331

Pica

Answer 331

- eat non-food Cause - many factors - maybe iron-deficiency anaemia Px - eating non-food - watch for sx of poisoning, cx Ix - bloods, maybe AXR, endoscopy Mx - remove substances, psych review

Question 332

Short stature

Answer 332

- <2nd centile for age/sex Causes - familial - constitutional delay - malnutrition, chronic disease, endo, Down's, achondroplasia, low GH Ix - XR L wrist - bloods - Cushing's ix, GH provocation tests Mx - reassure, tx causes

Question 333

Craniopharyngioma

Answer 333

- pituitary gland brain tumour Px - bitemporal hemianopia, headache, vomit, low GH, low growth, low ACTH, diabetes insipidus (lack of ADH) Ix - CT / MRI Mx - Neurosurgery - chemo / radio

Question 334

Normal puberty

Answer 334

Girls - starts 8-14yo - earlier growth spurt - breast buds, then pubic hair, menarche Boys - 9-15yo - testicle development (>4ml), then penis, scrotum darkening, pubic hair, deeper voice

Question 335

Male genitals Tanner staging

Answer 335

I - testicles <1.5ml, small prepubertal penis II - testicles 1.6-6ml, scrotum skin thins, reddens, enlarges III - testicles 6-12ml, scrotum enlarges, penis lengthens IV - testicles 12-20ml, scrotum enlarges, darkens, penis lengthens V - testicles >20ml, adult scrotum + penis

Question 336

Female breasts Tanner staging

Answer 336

I - no glandular tissue, areola follows skin contours of chest (prepubertal) II - breast bud, areola widens III - breast elevated, extends beyond borders of areola, remains in contour IV - increased breast size and elevation, areola and papilla form secondary mound projecting from contour of breast V - adult size breast, areola returns to contour of surrounding breast with projecting central papilla

Question 337

Pubic hair (M+F) tanner stages

Answer 337

I - no hair (prepubertal) II - small amount of long, downy hair, slight pigmentation at base of penis / scrotum or on labia majora III - hair more coarse + curly, begins to extend laterally IV - adult-like hair, across pubis, spares medial thighs V - hair extends to medial surface of thighs

Question 338

Hypogonadism

Answer 338

Lack of sex hormones (oestrogen, testosterone) -> delay in puberty

Question 339

Hypogonadotrophic hypogonadism

Answer 339

- Low FSH + LH leading to sex hormone deficiency - due to pit/hypo pathology - eg damage, low GH, hypothyroid, high prolactin, Kallman

Question 340

Hypergonadotrophic hypogonadism

Answer 340

- gonads fail to respond to FSH / LH - high FSH / LH - gonads damaged - eg torsion, cancer, congenital (Kleinfelter's, Turner's)

Question 341

Delayed puberty Ix/Mx

Answer 341

- Ix if no puberty changes by 13yo (F), 14yo (M) - H/E, FBC, ferritin, U/E, anti-TTG, early morning FSH/LH, TFTs, IGF-1, prolactin, genetics, XR L wrist, pelvic USS, MRI brain - Tx cause, replace sex hormones if needed

Question 342

Delayed puberty causes by stature

Answer 342

With short stature - Turner's syndrome - Prader-Willi syndrome - Noonan's syndrome Normal stature - Polycystic ovarian syndrome - Androgen insensitivity - Kallman's syndrome - Klinefelter syndrome

Question 343

Precocious puberty

Answer 343

- dev of 2ndary sex characteristics <8yo (F), <9yo (M) - thelarche - first breast development - adrenarche - first pubic hair Gonadotrophin dependent - premature activation of HPG axis - raised FSH + LH - idiopathic, CNS abnormalities, congenital, Turner's, high thyroid, Gonadotrophin independent - due to excess sex hormones - FSH + LH low - adrenal disorders, gonadal tumours, exogenous

Question 344

McCune-Albright syndrome

Answer 344

Due to random, somatic mutation in GNAS gene Px Precocious puberty Café-au-lait spots Polyostotic fibrous dysplasia Short stature

Question 345

Kallman syndrome

Answer 345

- X-linked recessive hypogonadotropic hypogonadism -> delayed puberty - failure of GnRH-secreting neurones to migrate to hypo -> lack of GnRH -> lack of sex hormones Px - delayed puberty, hypogonadism, small testis, micropenis, anosmia Ix - testicular volume - bloods / hormones - sperm, GnRH stimulation test, wrist XR, DEXA scan Mx - HRT - tes/oes/prog - FSH / LH replacement - GnRH pulsatile therapy

Question 346

Androgen insensitivity syndrome (AIS)

Answer 346

- X-linked recessive - end-organ resistance to testosterone - genotypically male (46XY) children grow up phenotypically female Type - depends on degree of genital masculinisation - complete - female genitalia - partial - ambiguous - mild - male Px - "primary amenorrhoea" - undescended testes (groin swellings) - breast dev - testosterone converted into oestradiol Ix - buccal smear - chromosomal analysis Mx - counselling - bl orchidectomy - risk of cancer - oestrogen HRT - sex assignment / genitoplasty

Question 347

Haemolytic disease of the newborn

Answer 347

- Rh negative mother previously sensitised to Rh positive cells - Transplacental passage of ABs - Haemolysis of Rh positive fetal cells Px - Severe anaemia - Neonatal jaundice - Hepatosplenomegaly Mx - Prevent sensitisation with Rh immunoglobulin - Intrauterine transfusion of affected fetuses

Question 348

Osteogenesis imperfecta

Answer 348

- autosomal dominant - brittle bones - collagen affected -> osteoporosis Px - recurrent fractures, hypermobile, blue/grey sclera, triangle face, short, early deafness, bowed legs, scoliosis, joint/bone pain Ix - clinical dx, XR, genetic testing Mx - bisphosphonates, vit D, OT/PT, MDT

Question 349

Rickets

Answer 349

- soft/deformed bones due to low minerals - vit D / Ca Px - bone pain, swollen wrists, poor growth, weak, abnormal fractures, deformities - leg bowing, knock knees, delayed teeth, kyphoscoliosis... Ix - Bloods - low Ca, phosp, vit D, high ALP/PTH.... - XR Mx - ergocalciferol (vit D) + Ca supplements

Question 350

Transient synovitis

Answer 350

3-8yo, recent URTI, inflammation of synovial membrane Recent viral infection Commonest cause of hip pain in children

Question 351

Transient synovitis Px

Answer 351

- acute / gradual - recent viral illness - limp, cannot weight bear - groin / hip pain - mild fever

Question 352

Transient synovitis Mx

Answer 352

- analgesia - self-limiting - needs review to r/o ddx High grade fever = urgent specialist assessment

Question 353

Septic arthritis

Answer 353

- infection in joint - most common <4yo - S aureus, N gonorrhoea, GAS...

Question 354

Septic arthritis Px

Answer 354

- single joint - hip, knee, ankle - rapid onset, hot red swollen, painful - not weight bearing - stiff, reduced ROM - fever, lethargy, sepsis

Question 355

Septic arthritis Ix

Answer 355

- bloods, inc cultures - joint aspiration, for MC+S

Question 356

Kocher Criteria - distinguish septic arthritis from transient synovitis - each point raises % chance

Answer 356

Non-weight bearing Temp >38.5 Raised ESR Raised WCC

Question 357

Septic arthritis Mx

Answer 357

IV abx - IV cefuroxime Surgical drainage / washout

Question 358

Osteomyelitis

Answer 358

- infection of bone / bone marrow - typically metaphysis Px - not weight bearing - pain, swelling, tender - low fever Ix - XR normal - MRI, bone scan - Bloods, inc culture - Bone marrow aspirate / bone biopsy Mx - IV cefuroxime - drainage / debridement

Question 359

Perthes disease

Answer 359

- avascular necrosis of femoral head - 4-12yo, M>F - risk of early OA

Question 360

Perthes Px

Answer 360

- slow onset - pain in hip, groin - limp, may be painless - stiff, reduced ROM - no trauma hx

Question 361

Perthes Ix

Answer 361

- XR (may be normal) - Bloods - MRI / technetium bone scan

Question 362

Perthes Mx

Answer 362

- younger / less severe - conservative, keep in position - bed rest, traction, crutches, analgesia - physio, regular XR - severe / not healing - surgery

Question 363

Slipped upper femoral epiphysis (SUFE)

Answer 363

- head of femur slips along growth plate - M>F, 8-15yo, commonly obese pt, 20% bilateral

Question 364

SUFE Px

Answer 364

- hx of minor trauma - pain out of proportion - hip, groin, thigh, knee pain - restricted ROM, esp int rotation - painful limp - hip externally rotated, shortened

Question 365

SUFE Ix

Answer 365

- XR - AP + lateral (frog leg) - Bloods - technetium bone scan - CT / MRI

Question 366

SUFE Mx

Answer 366

surgery

Question 367

Discoid meniscus

Answer 367

- thicker disc shaped meniscus - congenital Px - anterior / lateral knee pain - clunk at terminal flexion - "snapping knee syndrome" - pain, reduced ROM, effusion - locking / clicking Ix - XR - MRI - bow tie sign Mx - surgical - reshape meniscus - physio

Question 368

Toddler's fracture

Answer 368

undisplaced, spiral fracture of distal tibia in toddlers low-energy rotational trauma

Question 369

Osgood-schlatter's

Answer 369

- inflammation at tibial tuberosity at patella tendon insertion - 10-15yo - multiple small avulsion #'s from running / jumping at same time as growth -> lump -> tender then non-tender Px - gradual onset - lump at tibial tuberosity - pain anterior knee - worsened by physical activity, kneeling Mx - reduce activity - NSAIDs - physio

Question 370

Developmental dysplasia of the hip (DDH)

Answer 370

- abnormal dev -> unstable hips, prone to dislocate Picked up on NIPE, or later when child px with hip asymmetry, reduced ROM, limp

Question 371

DDH RFs

Answer 371

7 Fs - first born, female, fat (macrosomia), fair (caucasian), feet first (breech), fluid (oligohydramnios), FHx Multiple pregnancy

Question 372

DDH screening

Answer 372

- At NIPE, and 6-8wks - look for symmetry in - hips, leg length, skin folds, hip movts Barlow - push knees back, dislocate posteriorly Ortolani - abduct hips, pressure behind legs, reduce hips anteriorly

Question 373

DDH Ix

Answer 373

USS XR - esp if older >4.5mo

Question 374

DDH Mx

Answer 374

- most unstable hips spontaneously stabilise by 3-6wks - Pavlik harness if <6mo - surgery if harness fails

Question 375

Scoliosis

Answer 375

- spine twists causes - idiopathic, congenital, CP, muscular dystrophy, degenerative Mx - babies tend to grow out of it - back brace / surgery

Question 376

Congenital muscular torticollis

Answer 376

- neck deformity - SCM tight + thick - head to one side - r/o acquired - look for infective / neuro sx - USS hips to r/o DDH (RF) - passive stretching / physio to mx

Question 377

Talipes

Answer 377

- aka club foot - inverted / plantarflexed foot - mostly idiopathic Mx - ponseti method - manipulation + progressive casting - achilles tenotomy in 85% - boots + bars - surgery if ponseti method not effective

Question 378

Achondroplasia

Answer 378

- autosomal dominant - short stature - FGFR-3 gene mutation - abnormal cartilage, abnormal function of epiphyseal plates Px - short, proximal limbs short, short fingers, large head, frontal bossing, trident hands, bow legs Associations - obesity, otitis media, OSA Mx - MDT - ?limb lengthening - ?nothing

Question 379

Benign bone tumours

Answer 379

Osteoma - Benign overgrowth of bone Osteochondroma - cartilage capped bony projection on external surface of bone Giant cell tumour - tumour of multinucleated giant cells in fibrous stroma - XR - double bubble appearance

Question 380

Osteosarcoma

Answer 380

- most common primary malignant bone tumour, 10-20yo - tends to be metaphyseal regions of long bones - pagets / radio - RFs Px - bone pain, worse at night, bone swelling, mass Ix - urgent XR <48hrs - fluffy bone, poorly defined lesion, Codman triangle, sunburst appearance - bloods - raised ALP - CT / MRI / PET / bone scan - bone biopsy Mx - surgery - chemo

Question 381

Ewing's sarcoma

Answer 381

- Small round blue cell tumour - pelvis + long bones - severe pain - XR - onion skin appearance

Question 382

Chondrosarcoma

Answer 382

- malignant tumour of cartilage - axial skeleton - more common middle aged

Question 383

Juvenile idiopathic arthritis (JIA)

Answer 383

- autoimmune inflammation in joints -> joint pain, swelling, stiffness Mx - MDT - NSAIDs - Steroids (oligo) - DMARDs (methotrexate, sulfasalazine...) - TNF inhibitor - infliximab, etanercept - ophthal review for anterior uveitis

Question 384

Systemic JIA

Answer 384

- Still's disease - systemic illness Px - salmon pink rash - high fever - large lymph nodes - wt loss, splenomegaly, pleuritis, pericarditis - joint pain, inflammation, muscle pain Ix - Bloods - ANA, RF (normally -ve), raised CRP, ESR, platelets, ferritin Cx - macrophage activation syndrome (MAS) - massive immune system activation, inflammatory response

Question 385

Polyarticular JIA

Answer 385

- 5+ joints, symmetrical, small / large joints - minimal systemic sx - mild fever, anaemia, reduced growth - RA in kids - RF -ve

Question 386

Oligoarticular JIA

Answer 386

- <4 joints, usually 1 - larger joints (knee/ankle) - associated with anterior uveitis, no systemic sx - ANA+, RF-

Question 387

Enthesitis-related arthritis

Answer 387

- paeds version of seronegative sponyloarthropathies (AS, PsA, ReA, IBD-related) - inflammation of joints + entheses - HLA B27 Px - tender entheses - eg IPJ in hand, wrist, greater trochanter, quad at ASIS, base of achilles.... - ?psoriatic plaques, nail pitting, diarrhoea, PR bleed - uveitis - visual blurring, red eye, pain

Question 388

Juvenile psoriatic arthritis

Answer 388

- seronegative arthritis + psoriasis - symmetrical, small joints, or asymmetrical of large joints - psoriatic plaques, nail pitting, onycholysis, dactylitis, enthesitis

Question 389

Ehlers-Danlos syndrome (EDS)

Answer 389

- genetic conditions -> collagen defects -> joint hypermobility Types - hypermobile - classical - vascular - kyphoscoliotic

Question 390

EDS Px

Answer 390

- joint pain, hypermobile - dislocations - bleeding, bruising - soft stretchy skin - headaches, dizziness, syncope - GORD, abdo pan, IBS - menorrhagia, dysmenorrhoea, TMJ dysfunction, myopia, SAH, aortic regurg, mitral prolapse, dissection......

Question 391

EDS Ix - Beighton score for hypermobility

Answer 391

To assess extent of hypermobility - one point for each side of body, max score 9 - Palms flat on floor with straight legs (score 1) - Elbows hyperextend - Knees hyperextend - Thumb can bend to touch forearm - Little finger hyperextends past 90 degrees

Question 392

EDS DDx

Answer 392

Marfan syndrome - hypermobility, high arch palate, arachnodactyly, arm span

Question 393

EDS Mx

Answer 393

- OT/PT - exercise - no cure

Question 394

EDS Cx -postural orthostatic tachycardia syndrome (POTS)

Answer 394

- autonomic dysfunction - no SVR increase when standing - tachycardia, hypotension, syncope

Question 395

Henoch-Schonlein purpura (HSP)

Answer 395

- IgA vasculitis - purpuric rash on lower limbs / buttocks - triggered by URTI / GE - commonly GAS - IgA deposits -> leaking from vessels

Question 396

HSP Px

Answer 396

- purpura (100%) - red/purple, palpable under skin - arthralgia (75%) - knees/ankles - painful, swollen, reduced ROM - abdo pain (50%) - if severe - GI bleed, intussusception, infarction - IgA nephritis (50%) - micro/macroscopic haematuria, proteinuria (if 2+ protein - nephrotic)

Question 397

HSP Dx

Answer 397

EULAR/PRINTO/PRES criteria for dx Need palpable purpura (not petechiae) and at least one of: - Diffuse abdo pain - Arthritis / arthralgia - IgA deposits on biopsy - Proteinuria / haematuria

Question 398

HSP Ix

Answer 398

- bloods - FBC, blood film, renal function, serum albumin, CRP, cultures, urine dip, urine protein:creatinine ratio, BP....

Question 399

HSP Mx

Answer 399

- supportive - ?steroids - monitor urine dip + BP

Question 400

Kawasaki disease

Answer 400

- medium vessel vasculitis - young children Disease course - acute phase - 1-2wks - unwell, fever, rash, lymphadenopathy - subacute phase - 2-4 wks - desquamation, arthralgia, coronary artery aneurysm - convalescent stage - 2-4 wks - everything settles

Question 401

Kawasaki Px

Answer 401

fever >5d and 4/5 of: - Bl non-exudative conjunctivitis - Erythematous polymorphous rash - Strawberry tongue, dry cracked lips - Red swelling + desquamation of hands + feet - Tender cervical lymphadenopathy, unilateral May also have - D+V, fatigue, abdo pain, arthralgia, dysuria

Question 402

Kawasaki Ix

Answer 402

- Bloods - FBC, LFTs, ESR - urinalysis - ECHO

Question 403

Kawasaki Mx

Answer 403

- high dose aspirin - risk of Reye's - IVIg - risk of haemolytic anaemia - corticosteroids in high risk pts

Question 404

Kawasaki Cx

Answer 404

- coronary artery aneurysm - ECHO after 6-8 wks to screen

Question 405

Rheumatic fever

Answer 405

- autoimmune condition triggered by ABs to strep bacteria - caused by GAS - strep pyogenes (tonsillitis) - T2 hypersensitivity - IgM/G - immune system attacks body's cells

Question 406

Rheumatic fever Px

Answer 406

- 2-4wks after strep infection, eg tonslilitis - fever, joint pain, rash, SOB, chorea, nodules - joint pain is migratory - hot, swollen, painful - heart - peri/myo/endocarditis, tachy/brady, murmur, pericardial rub, HF - skin - nodules, pink rash - neuro - chorea

Question 407

Rheumatic fever - Jones criteria for Dx

Answer 407

Dx made when evidence of strep infection plus 2 major criteria OR 1 major and 2 minor Major (JONES) - Joint arthritis - Organ inflammation (eg carditis) - Nodules - Erythema marginatum rash - Sydenham chorea Minor (FEAR) - Fever - ECG changes (PR prolongation) w/o carditis - Arthralgia w/o arthritis - Raised CRP and ESR

Question 408

Rheumatic fever Ix

Answer 408

- throat swab - culture - Anti-streptococcal ABs (ASO) titres - ECHO, ECG, CXR

Question 409

Rheumatic fever Mx

Answer 409

- tx infection - pen V 10d - NSAIDs - joint pain - aspirin + steroids - cardio - prophylactic abv - penicillin - for further infections

Question 410

Allergic rhinitis

Answer 410

- IgE T1 hypersensitivity to environmental allergens -> nasal inflammation - eg hayfever, home dust mites, atopy association Px - runny, blocked nose, sneezing, itchy red swollen eyes Ix - clinical dx on hx - skin prick testing Mx - avoid trigger - antihistamines - non-sedating (cetirizine, loratadine, fexofenadine), sedating (chlorphenamine, promethazine) - nasal corticosteroid sprays - nasal antihistamines

Question 411

Cow's milk protein allergy (CMPA)

Answer 411

- non/IgE mediated hypersensitivity to protein in cow milk - typically <3yo, outgrow after

Question 412

CMPA Px

Answer 412

- usually <1yo, eg when weaned from breast to formula - bloating, wind, abdo pain, D+V - urticaria, angioedema, cough, wheeze, sneezy, watery eyes, eczema, anaphylaxis

Question 413

CMPA DDx

Answer 413

Lactose intolerance Cow's milk protein intolerance (CMPI) no allergic sx - will grow out of it

Question 414

CMPA Ix

Answer 414

- skin prick testing - total IgE, specific IgE (RAST) for cows milk protein

Question 415

CMPA Mx

Answer 415

If formula fed - extensive hydrolysed (eHF) milk - then amino acid formula (AAF) If breastfed - continue - avoid dairy in maternal diet - eHF when breastfeeding stops - try progressing up milk ladder

Question 416

Eczema

Answer 416

-chronic atopic condition - skin inflammation Px - itchy, erythematous rash - face + trunk in infants - extensor surfaces younger children - flexor surfaces, creases of face/neck - older children Mx - emollients - topical steroids - antihistmaine if severe itch - wet wrapping - oral ciclosporin - severe - flucloxacillin for infections

Question 417

Eczema herpeticum

Answer 417

- viral skin infection from HSV / VZV - HSV-1 commonly Px - widespread painful vesicular rash - punched out ulcers - fever, lethargy, irritable, lymphadenopathy Ix - viral swab of vesicles Mx - acyclovir

Question 418

Urticaria

Answer 418

- hives - histamine release from mast cells in skin - allergic / autoimmune Mx - fexofenadine - oral steroids for flares - if problematic - montelukast, omalizumab (IgE MAb), ciclosporin

Question 419

Nappy rash

Answer 419

- usually contact (irritant) dermatitis (urine / faeces contact) - can lead to bacterial / candida infection Types - irritant - creases spared - candida - flexures, macules, scaly border, satellite lesions - seborrhoeic - red, flakes, scalp rash -> baby shampoo / hydrocortisone - psoriasis - red + scaly - atopic - also affects other areas of skin Px - red, inflamed ski in nappy area - check for oral thrush Mx - barrier cream - mild steroid cream - topical imidazole - candida - topical fusidic acid / oral fluclox - bacterial

Question 420

Seborrhoeic dermatitis

Answer 420

- inflammatory skin condition affecting sebaceous glands - Malassezia yeast Px - cradle cap - crusty flaky scalp - red, flaky, crusted, itchy skin - eyelids, nasolabial folds, ears, upper chest, back Mx - cradle cap - baby oil, brush, wash, clotrimazole cream - older pts - ketoconazole shampoo - face + body - antifungal clotrimazole cream, hydrocortisone if inflammed

Question 421

Ringworm

Answer 421

- fungal infection, tinea: - capitis (scalp), pedis (feet), cruris (groin), corporis (body), onychomycosis (fungal nail infection) Px - itchy rash, red, scaly, demarcated, rings, pedis between toes Ix - clinical dx - scrape + send for MC+S Mx - antifungals - clotrimazole cream, ketoconazole shampoo, oral fluconazole.... - steroids for inflammation Tinea incognito - steroids dampen immune response - once stopped fungus returns worse

Question 422

Scabies

Answer 422

- tiny mites burrow + lay eggs under skin Px - itchy, small red spots, track marks - finger webs Mx - permethrin cream - whole body, leave 8-12hrs, then wash off, rpt 1wk later - malathion 2nd line - wash bedding / clothing, tx all household members - oral ivermectin - severe (crusted) scabies - massive infection

Question 423

Roseola infantum

Answer 423

- aka sixth disease - HHV6 - 5-12d incubation, school exclusion not needed Px - high fever for a few days - then maculopapular rash - Nagayama spots - diarrhoea, cough, sore throat Ix - clinical dx - PCR to confirm Mx - self-limiting

Question 424

Birthmarks

Answer 424

see notes

Question 425

Primary immunodeficiency

Answer 425

genetic defects in immune system -> infection risk Types - AB deficiency - combined - eg severe combined immunodeficiency (SCID) - phagocytic cell deficiency - complement deficiency - immune dysregulation - autoinflammatory

Question 426

Primary immunodeficiency Px

Answer 426

- failure to thrive - severe / frequent infections - low lymphocyte count .....

Question 427

Primary immunodeficiency Ix

Answer 427

- FBC - low WCC - total Ig G/A/M +/-E - HIV aBs

Question 428

Primary immunodeficiency Mx

Answer 428

- abx/antiviral prophylaxis - tx infections - Ig replacement - bone marrow transplant

Question 429

Stevens-Johnson Syndrome (SJS) and Toxic Epidermal Necrolysis (TEN)

Answer 429

- severe/disproportionate immune response -> epidermal necrosis - SJS <10% body SA - TEN >10% Causes - penicillin, sulfonamides, NSAIDs, lamotrigine.....infections.... Px - fever, cough, sore throat/mouth/eyes, itchy skin, arthralgia - rash - maculopapular, target lesions, vesicles, bullae - affect urinary tract, lungs Mx - admit to derm / burns - supportive - steroids, Igs, immunosuppression

Question 430

Erythema multiforme

Answer 430

- erythematous rash, from hypersensitivity reaction - eg viral infections, meds, HSV, mycoplasma pneumonia Px - widespread itchy erythematous rash - target lesions - does not affect mucous membranes - sore throat - fever, arthralgia, myalgia Ix - clinical dx - CXR for mycoplasma Mx - resolves alone - severe - admit, IV fluids, analgesia, steroids, abx/antivirals

Question 431

Erythema nodosum

Answer 431

- red lumps on shins - rom hypersensitivity - caused by inflammation of subcut fat - panniculitis Causes - strep throat infections, GE, mycoplasma, TB, pregnancy, meds, IBD.... Px - red, inflamed, subcut nodules across both shins - raised, tender - may settle - bruises Ix - ESR, CRP - throat swab for strep - CXR - mycoplasma, TB, sarcoidosis, lymphoma - stool MC+S - faecal calprotectin - IBD Mx - tx cause - rest, steroids, analgesia - resolve in 6wks

Question 432

Actions after birth

Answer 432

- skin-to-skin, clamp cord (after it stops pulsating), dry baby, keep baby warm, IM vit K, label baby, measure weight + height - feed as soon as alert enough, NIPE <72hrs, blood spot tests d5, OAE test

Question 433

Blood spot test conditions

Answer 433

Sickle cell disease Cystic fibrosis Congenital hypothyroidism Phenylketonuria Medium-chain acyl-CoA dehydrogenase deficiency (MCADD) Maple syrup urine disease (MSUD) Isovaleric acidaemia (IVA) Glutaric acidura type 1 (GA1) Homocystin

Question 434

Caput succedaneum

Answer 434

- fluid on scalp, outside periosteum, instrumental delivery - crosses suture lines, no skin discolouration - resolves in a few days

Question 435

Cephalohaematoma

Answer 435

- blood between skull + periosteum - does not cross suture lines, skin discolouration - anaemia + jaundice risk

Question 436

Facial paralysis

Answer 436

- damage to nerve, forceps delivery - normally resolves in a few months - neurosurg input if not

Question 437

Erb's palsy

Answer 437

- C5/6 injury in brachial plexus after birth - associated with shoulder dystocia, traumatic/instrumental delivery - weakness of shoulder abduction, external rotation, arm flexion, finger extension - waiters tip - resolves in a few months, neurosurg if not

Question 438

Fractured clavicle

Answer 438

- associated with shoulder dystocia, large birth weight... - lack of movt, asymmetry, pain / distress - USS / XR - Immobilise to tx

Question 439

Neonatal life support

Answer 439

- cut cord, dry baby, stimulate - 5 inflation breaths (can use air) - if no chest movt, manage airway, repeat inflation breaths - 15 ventilation breaths (for 30s, every 2s) - if HR <60 -> 3:1 compressions (increase O2 to 100%) - reassess every 30s

Question 440

APGAR

Answer 440

1, 5, 10 mins 0-3 is low, 4-6 moderately low, 7-10 is good state - appearance / colour - pulse - grimace - activity / tone - respiration

Question 441

Hypoxic-ischaemic encephalopathy (HIE)

Answer 441

- brain damage from hypoxia at birth -> CP, death - eg maternal shock, intrapartum haemorrhage, prolapsed cord, nuchal cord (wrapped around neck) Sarnat staging Mild - poor feeding, irritable, resolves <24hrs Moderate - poor feeding, lethargic, hypotonia, seizures, 40% develop CP Severe - reduced consciousness, apnoeas, flaccid, <50% mortality Mx - optimise ventilation, feeds - ICU therapeutic cooling - MDT...

Question 442

Respiratory distress syndrome

Answer 442

- prem neonates -> surfactant deficienct - alveolar collapse -> inadequate gas exchange - can lead to CLDP, ROP Px - resp distress, raised RR - intercostal recession, grunting, cyanosis Ix - XR - ground glass appearance Mx - dex for mother - intubation + ventilation - endotracheal surfactant - CPAP, O2

Question 443

Transient tachypnoea of the newborn (TTN)

Answer 443

- delayed resorption of fluid in the lungs, common during c-sections Px - raised RR, resp distress Ix - CXR - hyperinflation of lungs, fluid in horizontal fissure Mx - observe, support, O2

Question 444

Meconium aspiration

Answer 444

- aspiration of meconium -> resp distress, airway obstruction, hypoxia, infection Dx - meconium-stained amniotic fluids, meconium in airways - resp distress - pneumonia Mx - supportive, O2, ventilate, suction, abx, NICUNeonatal sepsis

Question 445

Neonatal sepsis

Answer 445

- infection in neonatal period - early onset <72hrs, late onset 7-28d Common orgs - GBS, E coli, listeria, klebsiella, S aureus

Question 446

Neonatal sepsis RFs

Answer 446

- vaginal GBS - GBS sepsis in prev baby - maternal sepsis, chorioamnionitis, fever >38 - prematurity (IgG transfer in last 3mo gestation, baby doesn't make own ABs until a few months old) - early ROM - PROM

Question 447

Neonatal sepsis Px

Answer 447

- fever / hypothermia - reduced tone / feeding - resp distress, apnoea - vomiting - tachy/bradycardia - jaundice <24hrs - hypoglycaemia - neuro sx, seizures

Question 448

Neonatal sepsis Ix

Answer 448

- cultures, FBC, CRP, gas, urine dip, MC+S - LP

Question 449

Neonatal sepsis Mx

Answer 449

- benpen + gent - cefotaxime in lower risk - repeat CRP / cultures

Question 450

Neonatal jaundice

Answer 450

- increased bilirubin - <24hrs - pathological - neonatal sepsis, haemolytic disease... - >14d in term / >21d preterm - pathological - - from fetal Hb breakdown, immature liver - breastfed babies more likely to be jaundiced, prem babies worse

Question 451

Neonatal jaundice causes

Answer 451

Increased production - haemolytic disease of newborn, ABO incompatibility, haemorrhage, cephalohaematoma, sepsis, DIC, G6PD deficiency Decreased clearance - prem, breastfed, neonatal cholestasis, biliary atresia, low thyroid, low pituitary, Gilbert

Question 452

Neonatal jaundice Ix

Answer 452

- FBC, blood film - conjugated bilirubin - Blood type for mother + baby - Direct antiglobulin test (DAT) - Coombs - TFTs - blood / urine cultures - G6PD levels

Question 453

Neonatal jaundice Mx

Answer 453

- plot bilirubin levels on chart - phototherapy - exchange transfusion

Question 454

Neonatal jaundice Cx

Answer 454

Kernicterus - brain damage caused by excessive unconjugated bilirubin - crosses BBB, damages CNS Px - floppy, drowsy baby, poor feeding Can lead to CP, LDs, deafness

Question 455

Neonatal hypoglycaemia

Answer 455

- transient <24hrs - normal - <2.6 defined as neonatal hypo Causes of persistent hypo - prem, maternal DM, IUGR, hypothermia, sepsis... Px - jittery baby, irritable, pallor - poor feeding, weak cry, drowsy, low tone - apnoea, hypothermia Mx - asym - encourage feeds - sym - NICU, IV dextrose 10%

Question 456

Neonatal hepatitis syndrome

Answer 456

- early liver inflammation - eg CMV, rubella, hepatitis Px - jaundice, dark urine - failure to thrive - HSM - liver cirrhosis, bruising DDx - biliary atresia - no splenomegaly - neonatal jaundice - no dark urine Ix - bloods - inc LFTs - USS - biopsy Mx - tx cause - vit supplements - liver transplant

Question 457

Prematurity

Answer 457

- born <37wks - associated with - smoking, alcohol, maternal comorbidities.... Mx before birth - vaginal progesterone / cerclage to discourage - tocolysis + nifedipine - corticosteroids <35wks - Mg <34wks - protect brain

Question 458

Apnoeas of prematurity

Answer 458

- immaturity of autonomic NS -> recurrent apnoeas - desaturations, bradycardia Mx - apnoea monitor - tactile stimulation - IV caffeine

Question 459

Retinopathy of prematurity (ROP)

Answer 459

- abnormal dev of blood vessels in retina -> scarring, retinal detachment, blindness - excessive vascularisation from early O2 exposure, abnormal vessels, scar tissue, VEGF - screen evert 2wks if <32wks / <1/5kg Mx - laser therapy - cryotherapy - VEGF inhibitors

Question 460

Neonatal abstinence syndrome (NAS)

Answer 460

- withdrawal in neonate from maternal substance use - opiates, cocaine, meth, nicotine Px - 3-72hrs for opiates, diazepam, SSRI, alcohol - 1-21d for methadone, other benzos - irritable, increased tone, high cry, tremor, seizure - yawning, sweaty, unstable temp, fever, increased RR - poor feeding, vomiting, low BMs, loose stools Mx - NAS chart - urine sample - test for substances - morphine sulfate - for opiate withdrawal - oral phenobarbitone - non-opiate withdrawal

Question 461

Haemorrhagic disease of the newborn (HDN)

Answer 461

- low vit K in newborn babies -> risk of haemorrhage - give IM vit K at birth (can give oral) Intraventricular haemorrhage (IVH) - <72hrs - USS to dx - supportive mx, shunt for raised ICP

Question 462

Cystic periventricular leukomalacia

Answer 462

death of white matter near lateral ventricles -> CP more common in prem babies

Question 463

Fetal alcohol syndrome

Answer 463

microcephaly, thin upper lip, smooth philtrum, short palpebral fissure, LDs, CP, cardiac malformations

Question 464

Congenital rubella syndrome

Answer 464

congenital cataracts, PDA, AS, LD, hearing loss (sensorineural)

Question 465

Congenital varicella syndrome

Answer 465

IUGR, LD, scars, skin changes, limbs not developed, eye damage

Question 466

Congenital CMV

Answer 466

IUGR, hearing / vision loss, LD, seizures

Question 467

Congenital toxoplasmosis

Answer 467

Intracranial calcification + hydrocephalus + chorioretinitis

Question 468

Congenital zika syndrome

Answer 468

microcephaly, IUGR, ventriculomegaly, cerebellar atrophy

Question 469

TORCH

Answer 469

- congenital infection - Toxoplasmosis, Other agents, Rubella, CMV, HSV - also syphilis, zika, parvovirus B19 Px - HSM, fever, lethargy, reduced feeding, anaemia, jaundice Ix - IgM, IgG Mx - tx infection in mother / fetus

Question 470

Sudden infant death syndrome (SIDS)

Answer 470

- unexplained death in infant (cot death) RFs - prematurity, low birth weight, smoking during pregnancy, sleeping prone, bed sharing Minimise risk - baby on back, head uncovered, nothing by head, 16-20 degree room temp, no co-sleeping, no smoking Support - The lullaby trust - Bereavement services CONI - care of next infant

Question 471

VACTERL association

Answer 471

- association of birth defects, tend to co-occur Vertebral defects Anal atresia / malformations - no anal opening - surgery, colostomy first, then 6mo repair Cardiac defects - ASD, VSD, TOF, also truncus arteriosus, TGA Tracheoesophageal fistula - abnormal connection between oesophagus + trachea - Px - choking, coughing, vomiting, cyanosis with feeding - Mx - surgery, NICU, stomach tube feed (O)Esophageal atresia - oesophagus not connected to stomach - unable to feed, surgery Renal abnormalities - defects, kidney failure - transplant / correction Limb abnormalities - eg thumbs, polydactyly, syndactyly, radial aplasia....