Paeds Notes

Question 1

Cystic fibrosis: AR/AD?

Answer 1

AR

Question 2

Cystic fibrosis: 1 in ? affected?

Answer 2

1 in 2000

Question 3

Cystic fibrosis: 1 in ? carriers?

Answer 3

1 in 22

Question 4

Life expectancy of cystic fibrosis

Answer 4

Mid 30s

Question 5

Mutation in cystic fibrosis and which chromosome

Answer 5

CFTR on Chr7. most commonly delta f508

Question 6

Organism that specifically affects cystic fibrosis

Answer 6

pseudomonas auriginosa

Question 7

If not through newborn screening, how might CF present?

Answer 7

meconium ileum

Question 8

2 diagnostic tests for CF

Answer 8

Heel prick
Sweat test (>60mmol/l of sweat Cl- supports diagnosis)

Question 9

Treatment for CF

Answer 9

MDT approach
• Education
• Respiratory:
o Monitor FEV1 and symptoms
o Physiotherapy
o Nebulisers
o Prophylactic and rescue antibiotics
• Nutritional/ GI
o Monitor: weight/ regularly asses diet, OGTT (Over 12) o Replacement enzymes
o Multivitamin
o High calorie/ high protein diet
• Psychological

Question 10

When do you give Abx to tonsillitis/pharyngitis? And what antibiotic for how long?

Answer 10

10 days Pen V if FeverPAIN score:
0-1 = nothing
2-3 = delayed
4-5 = give Abx

Question 11

Indication for tonsilectomy

Answer 11

7 in one year or 10 in 2 years

Question 12

scarlet fever cause

Answer 12

Group A strep (think of strep throat)

Question 13

cause of croup

Answer 13

parainfluenza 3

sometimes RSV

Question 14

management of croup

Answer 14

oral dex and support

IV adrenaline is emergency

Question 15

presentation of bacterial tracheitis

Answer 15

viral prodrome 2-5 days then rapid deterioration with continuous biphasic stridor

Question 16

cause of bacterial tracheitis

Answer 16

S.aureus

Question 17

management of bacterial trachetiis

Answer 17

cefotaxime + fluclox

?intubation

Question 18

key feature of diptheria

Answer 18

tonsilitis + grey false membrane over them

Question 19

management of diptheria

Answer 19

Diptheria antitoxin

erythromycin to patient and contacts

Question 20

epiglottitis cause

Answer 20

Hib

Question 21

presentation of epiglottitis

Answer 21

VERY RAPID ONSET

Very high fever, won’t swallow and will drool instead. continuous stridor.

Question 22

management of epiglotittis

Answer 22

Call anaesthesitst, paediatrician and ENT surgeon for controlled intubation.
Cefotaxime as Abx

Question 23

age at which viral wheeze tends to stop

Answer 23

5 (most is 2-3)

Question 24

brittle asthma types

Answer 24

type 1 - variable PEFR

type 2 - sudden attack on normal background

Question 25

when to discharge after acute asthma

Answer 25

when stable on >4hrly salbutamol

Question 26

what to discharge acute asthma attack with

Answer 26

- 3-7days pred - follow up in 1 months in clinic - inhaler technique

Question 27

when is acute bronchiolitis worst?

Answer 27

5 days

Question 28

bronchiolitis cause?

Answer 28

RSV

Question 29

bronchiolitis prevention?

Answer 29

Monthly pavalizumab

Question 30

whooping cough timeline

Answer 30

1 week of coryza 3 months of paroxysmal phase

Question 31

treatment of whooping cough

Answer 31

macrolide

Question 32

latent TB worldwide number?

Answer 32

2 billion

Question 33

death from TB / year

Answer 33

2.5 million

Question 34

investigations for TB (3)

Answer 34

tuberculin interferon gamma release assay Zn stain for acid fast bacilli

Question 35

TB management: latent and active

Answer 35

latent = 3m RI Active = 2mRIPE and 4mRI rifampicin, isoniazid, pyrazinamide, ethambutol

Question 36

pulmonary blood flow before vs after first breath

Answer 36

6x increase

Question 37

why does the foramen ovale close

Answer 37

blood goes to lung and so left atrial pressure increases blood excluded from placenta to right atrial pressure decreases

Question 38

Why are innocent murmurs SASSy?

Answer 38

soft asymptomatic (incl. sats) systolic left sternal edge

Question 39

flow murmur scenario

Answer 39

fever anaemia day 1 murmur (due to physiologic tricuspid regurgitate)

Question 40

type of murmur: | aortic stenosis

Answer 40

ejection systolic upper right

Question 41

type of murmur: | ASD

Answer 41

soft systolic and S2 splitting

Question 42

type of murmur: | pul stenosis

Answer 42

ejection systolic upper left

Question 43

type of murmur: | VSD

Answer 43

harsh pan systolic left chest

Question 44

type of murmur: | coarc of aorta

Answer 44

systolic on left chest

Question 45

R to L presentation L to R presentation R to L or L to R: - tetralogy of fallot - ASD - transposition of great arteries - VSD - PDA - Eisenmenger

Answer 45

``` RL = blue LR = breathless (acyanotic) ``` ``` tetra RL asd LR tga RL VSD LR PDA LR Eisen RL ```

Question 46

tetralogy of fallot

Answer 46

1. large VSD 2. over-riding aorta 3. pulmonary stenosis 4. RV hypertrophy

Question 47

most common congenital heart disease?

Answer 47

VSD (30% of total), and 9 most common account for 80%

Question 48

how do the following present? L to R shunt (VSD, PDA, ASD) R to L shunt (ToF, TGA) Mixing shunts (ASD+VSD) Outflow obstruction in well child (Pul or aortic stenosis) Left heart outflow obstruction (coarctation of aorta, hypoplastic left heart, interruption of aortic arch)

Answer 48

L to R shunt = breathless R to L shunt = blue Mixing shunts = blue and breathless Outflow obstruction in well child = asymptomatic murmur Left heart outflow obstruction = sudden circulatory collapse with some absent pulses

Question 49

eisenmenger syndrome

Answer 49

longstanding L2R shunt (e.g. VSD/ASD/PDA) goes unnoticed. This eventually causes pulmonary hypertension and reversal of the shunt, causing cyanosis in adults.

Question 50

types of ASD and which is more common

Answer 50

primum (Downs) | secumdum (85%)

Question 51

presentation of ASD

Answer 51

asymptomatic with murmur (fixed S2 splitting and soft systolic left sternal edge)

Question 52

CXR for ASD

Answer 52

cardiomegaly (RV dilatation - remember its L2R), enlarged pul. arteries, increased pul. vascular markings.

Question 53

presentation of VSD

Answer 53

asymptomatic with murmur (pansystolic): - small VSD = loud murmur - large VSD = quiet murmur

Question 54

CXR and ECG with VSD

Answer 54

- small VSD = normal | - large VSD = cardiomegaly, enlarged pul arteries, increase pul vast marking, biventricular hypertrophy on ECG

Question 55

management of VSD

Answer 55

- small = most close spontaneously | - large = heart failure meds (diuretics and ACEi) + surgery at 6m

Question 56

when is a ductus arteriosus considered failed to have closed?

Answer 56

1 months

Question 57

presentation of PDA

Answer 57

machinery murmur collapsing/bounding pulse breathless

Question 58

management of PDA

Answer 58

closure by coil

Question 59

what is the nitrogen washout test

Answer 59

aims: "does this blue kid have congenital heart problem?" method: 100% oxygen for 10 mins results: if right radial artery ppO2 is still low, congenital heart disease can be diagnosed

Question 60

management of cyanosed neonate

Answer 60

ABC IV prostaglandin Do ECG, Echo and assess cause and need for surgery

Question 61

CXR with tetralogy of fallot

Answer 61

Small heart with up tilted apex (boot shaped RV hypertrophy)

Question 62

when is surgery for ToF

Answer 62

6 months

Question 63

When does TGA present

Answer 63

day 2 of life with rapid deterioration as duct closes (since it's two parallel circuits at this point)

Question 64

management of TGA

Answer 64

as with any cyanosed baby (ABC, IV prost, investigate) | balloon atrial septostomy in 20% of cases, arterial switch surgery on day 3-5

Question 65

atrioventricular septal defect common in?

Answer 65

Downs

Question 66

How do AVSDs present

Answer 66

antenatal USS cyanosis at birth Heart failure 2-3 weeks no murmur

Question 67

how does aortic stenosis/pul stenosis occur in babies?

Answer 67

leaflets fused abnormally (as opposed to calcification in elderly

Question 68

when does coarctation of the aorta present?

Answer 68

normal at first check, circ. collapse at 2-3 days when duct closes

Question 69

what is coarctation associated with?

Answer 69

Turners

Question 70

management of hypoplastic left heart syndrome

Answer 70

Norwood operation

Question 71

WPW Syndrome ECG change

Answer 71

delta wave

Question 72

presentation of SVT

Answer 72

narrow complex rapid tachycardia 250-300 +/- pulmonary oedema (breathlessness)

Question 73

management of SVT

Answer 73

RESTORE SINUS 1. vagal stimulating manaenovres (carotid massage) 2. IV adenosine (in adults its 6,12,12) 3. DC cardio version LONG TERM - flecainide or sotalol 90% don't have further attack so stop treatment at 1 yr If WPW present, need ablation

Question 74

long QT syndrome presentation

Answer 74

sudden LOC during exercise, stress, emotion in late childhood (confused with epilepsy) can cause sudden death from VT

Question 75

long QT inheritance and associations

Answer 75

AD inheritance (its structural) association: - erythromycin - electrolyte disorders, - head injury

Question 76

mortality of Coxsackie virus myocarditis in children

Answer 76

80%

Question 77

management of bacterial endocarditis

Answer 77

Benzyl pen + gent +/- surgery

Question 78

criteria for kawasaki

Answer 78

Fever for >5days and 4/5 of: - non purulent conjunctivitis - lymphadenopathy - maculpapular rash - extremity involvement (Red finger tips) - mucositis (strawberry tongue, cracked lips)

Question 79

management of kawasaki

Answer 79

High dose aspirin Immuniglobulin need angiogram

Question 80

jones diagnostic criteria

Answer 80

for rheumatic fever. Need 2 major or 1 major /2minor AND evidence of previous strep infection (scarlett fever or positive throat swab) ``` Major: carditis (murmur, CCF, cardiomegaly, friction rub, echo) poly arthritis -erythema marginatum subcutaneous nodule sydenham's chorea ``` ``` minor: fever arthralgia prolonged PR interval high ESR/CRP ```

Question 81

treatment for rheumatic fever

Answer 81

rest aspirin benzylpenicillin followed by Pen V (prevents secondary rheumatic fever)

Question 82

what is posseting vs regurg vs vomit

Answer 82

small amounts of milk that's burped up alongside swallowed air. normal in babies. regurg is larger more frequent losses. vomiting is forceful ejection

Question 83

when does GORD in baby resolve usually

Answer 83

12 months, resolved in almost everyone

Question 84

does GORD affect weight of baby?

Answer 84

Not usually

Question 85

complications of GORD (4)

Answer 85

``` faltering growth oesophagitis (haematemesis, pain, iron def anaemia) pul aspiration (pneumonia, apnoea) Sandifer syndrome (dystonic neck posture to relieve pain) ```

Question 86

GORD management

Answer 86

Conservative: - feed little and often - upright after feeds - food thickener Medical: - antacid - ranitidine or omeprazole Surgical - Nissen fundoplication if underlying disorder (CP) to wrap fundus around sphincter

Question 87

incidence of pyloric stenosis

Answer 87

1 in 400

Question 88

M:F in pyloric stenosis

Answer 88

M>F 4:1

Question 89

management of pyloric stenosis

Answer 89

Randmstedt pyloromyotomy - can then be fed at 6hrs and discharged at 2 days

Question 90

what is duodenal atresia associated with (2)

Answer 90

Downs | Polyhydramnios

Question 91

Investigation for duodenal atresia

Answer 91

Abdo plain film = double bubble sign

Question 92

management of duodenal atresia

Answer 92

ABC Drip and suck Surgery

Question 93

when does appendicitis typically present

Answer 93

>5 (under 3 it is vanishingly rare)

Question 94

how does mesenteric adeninitis present

Answer 94

like appendicitis but without peritonism, guarding, rebound tenderness.

Question 95

peak age for intussuception

Answer 95

6m-2y

Question 96

primary vs secondary intusssuception

Answer 96

``` primary = lymphoid hyperplasia of peters patch secondary = pathological point (meckels, polyp) usually older children ```

Question 97

presentation of intussuception

Answer 97

PAROXYSMAL colicky pain where child become pale and draws up legs bile vomit redcurrant jelly stools (late sign) sausage shapes mass in upper quadrant

Question 98

CI of air insufflation in intusseoction

Answer 98

peritonitis shock perforation known lead pathological point

Question 99

diagnosis of mecekls diverticulum

Answer 99

technetium scan showing increased uptake by ectopic gastric mucosa

Question 100

when does the in utero gut return to the abdominal cavity

Answer 100

10w

Question 101

when does volvulus due to malrotation present

Answer 101

first 1-3 days (90% in first year)

Question 102

diagnosis of volvulus due to malrotation

Answer 102

urgent upper GI contrast shows corkscrew appearance

Question 103

what 3 features of chronic abdo pain suggest non-organic cause (which is the case with >90%)

Answer 103

1. periodic pain with intervening good health 2. periumbilical 3. related to school hours (not there in holidays/evenings)

Question 104

recurrent idiopathic abdo pain definition

Answer 104

pain enough to interrupt ADLs for at least 3 months

Question 105

what is an abdominal migraine

Answer 105

abdo pain in addition to headaches that comes in attacks (long periods ok and then 12-48hrs of pain)

Question 106

treatment of abdominal migraine

Answer 106

NSAIDs and triptans

Question 107

investigation results for eosinophilic oesophagitis

Answer 107

endoscopy with biopsy: - linear furrows and trachealisation of oesophagus - eosiniphilic infiltration on biopsy

Question 108

management of eosinophilic oesopahgitis

Answer 108

swallowed corticosteroids (fluticasone, viscous budesonide)

Question 109

what is the only abnormal thing if dehydration <5%?

Answer 109

dry mucous membranes

Question 110

what suggests >10% dehydration as opposed to just 5-10% dehydration?

Answer 110

anuria coma low BP (late sign) Mottled/pale skin --> tachypnoea occurs in moderate 5-10% dehydration, don't get caught out

Question 111

calculating fluid requirement

Answer 111

Fluids required = maintenance fluid PLUS fluid deficit maintenance fluid: first 10kg = 100ml/kg second 10kg = 50ml/kg thereafter = 20ml/kg fluid deficit = estimated % dehydration as a % of body weight (e.g. 10% dehydrated if 7.5kg = 750ml extra) THIS IS ALL NORMAL 0.9% SALINE

Question 112

how to rehydrate

Answer 112

try oral then NG persistent vomiting = IV rehydration

Question 113

deaths from gastroenteritis per year

Answer 113

1.5 million

Question 114

most common virus causing gastroenteritis

Answer 114

rotavirus (60%)

Question 115

dysentry causes

Answer 115

CASES: ``` campylobacter amoeba shigella E.coli salmonella ```

Question 116

Revise table of gastroenteritis

Answer 116

PDF or desktop

Question 117

why avoid juice in gastroenetritis

Answer 117

osmotically draws even more water into gut

Question 118

when are Abx required in gastroenteritis

Answer 118

- sepsis - salmonella under 6m - specific infection (cdiff, cholera, shigella, giardia)

Question 119

advice for parents at home with gastroenteritis in child

Answer 119

``` diarrhoea lasts 5-7d vomiting 1-2d safety net dehydration symptoms lactose intolerance can follow GE exclude from school until 48h last D or V ```

Question 120

key question with chronic diarhoea

Answer 120

are they failing to thrive? yes = CF, coeliac, lactose intolerance No = overflow constipation, toddler diarrhoea

Question 121

how to manage toddler diarhoea

Answer 121

fibre down sugar down fat up

Question 122

gastroenteritis with eosinophilia and Tx

Answer 122

giardiasis metronidazole

Question 123

Cows milk protein intolerance vs lactose intolerance

Answer 123

milk protein intolerance is an ALLERGY, so comes with atopic individuals. these people can't have it anymore and symptoms subside after cessation. lactose intolerance is common after gastroentertisis (when superficial lactase enzymes are stripped from bowel). these take a break from lactose but can return after Sx subside.

Question 124

prevalence and incidence of coeliac

Answer 124

1% | 1 in 3000

Question 125

histopathology of coeliac

Answer 125

<3:1 villus height:crypt depth ratio with lymphocyte invasion

Question 126

how and when does coeliac usually present

Answer 126

CLASSICALLY 8-24 months faltering growth, abdo distension, buttock wasting, abnormal stools, irritable ATYPICALLY later in life with mild non specific GI Sx and low growth or anaemia.

Question 127

screening for coeliac

Answer 127

anti TTG | EMA (endomysial antibodies)

Question 128

does screening diagnose coeliacs?

Answer 128

no, you need small intestine biopsy

Question 129

treatment of coealic

Answer 129

remove wheat, rye, barley

Question 130

complications of coealic (3)

Answer 130

Enteropathy associated T-cell lymphoma Small bowel lymphoma ulcerative jejunitis

Question 131

management of crohns and UC

Answer 131

o Crohn’s disease: ♣ First line: - Immunosuppression (steroids, azathioprine) - Anti-inflammatory (5-ASA a.k.a. mesalazine) ♣ Second line: - Add other immunosuppressants (mercaptopurine, methotrexate + folate) ♣ Third line: - Add anti-TNF biologic (infliximab, adalimumab) ♣ Surgery may be needed at any stage if there is perforation, obstruction or toxic megacolon. o Ulcerative colitis: ♣ First line: - Topical 5-ASA (suppository/enema) - Oral 5-ASA or steroids ♣ Second line: - Add anti-TNF biologic (infliximab, adalimumab) ♣ Colectomy can be needed at any stage in fulminant or very refractory disease

Question 132

management of longstanding simple constipation

Answer 132

1st line = stool softners (macrogol laxative) 2nd line = stimulant (Senna)

Question 133

diagnosis of Hirschsprungs

Answer 133

rectal biopsy shows absence of ganglion cells with presence of large acetylcholinesterase positive nerve trunks

Question 134

management of Hirschspurng

Answer 134

in the 75% of cases it is localised to dial bowel: - cut it out and form colostomy - anatomise innervated bowel to anus later on

Question 135

does hepatitis cause conj or unconj hyperbilirubinaemia

Answer 135

conj

Question 136

choledocal cyst

Answer 136

dilation og bile duct from gall bladder to duodenum causing a pseudo-obstruction and jaundice

Question 137

what does jaundice <24hrs mean

Answer 137

probs haemolysis and serious cause (rhesus, ABO, G6PD, HS)

Question 138

what does jaundice 2d-2w mean

Answer 138

physiological or breast milk jaundice probs

Question 139

complication of kernicterus

Answer 139

cerebral palsy learning difficulties cerebral deafness

Question 140

complications of chronic liver disease

Answer 140

Nutrition (fat malabsorption, loss of fat soluble vitamins ADEK) Pruritus Encephalopathy due to ammonia cirrhosis/portal hypertension (ascites, varices, spelnomeglay)

Question 141

what is biliary atresia

Answer 141

progressive fibrosis and obliteration of biliary tree. unknown aetiology but causes chronic liver failure within 2y untreated.

Question 142

presentation of biliary atresia

Answer 142

mild jaundice pale stools faltering growth hepatosplenomegaly (portal hypertension)

Question 143

investigations of biliary atresia

Answer 143

LFT - raised conj bili US - contracted or absent gallbladder ERCP - diagnostic

Question 144

management of biliary atresia

Answer 144

surgery (palliative or liver transplant) and fat soluble vitamins supplementation

Question 145

investigations of A1ATd

Answer 145

plasma anti trypsin is low

Question 146

presentation of A1ATd

Answer 146

prolonged neonatal jaundice bleeding (vitKdef) hepatomegaly

Question 147

what % of febrile children will have a UTI?

Answer 147

7%

Question 148

what is the % recurrence of a UTI

Answer 148

12-30%

Question 149

which bug for UTI is more common in boys

Answer 149

Proteus (can cause phosphate stones)

Question 150

mild vs severe vesicoureteric reflux

Answer 150

``` mild = into ureter only severe = dilating renal pelvis and calyces as well ```

Question 151

how does VUR lead to hypertension

Answer 151

reflux --> hydronephrosis --> infection will track up easily --> renal scarring --> hypertension

Question 152

atypical UTI infection: refer or don't refer

Answer 152

refer urgently for USS

Question 153

when do you refer for a 'within 6 weeks' USS after a UTI

Answer 153

<3m | recurrent

Question 154

glomerular vs non-glomerular haematuria

Answer 154

glomerular: | brown urine, deformed RBCs, casts +/- proteinuria

Question 155

most common cause of haematuria

Answer 155

UTI

Question 156

indications for renal biopsy

Answer 156

persistent proteinuria recurrent haematuria abnormal renal function persistent abnormal complement

Question 157

investigation of haematuria

Answer 157

``` Urine MC&S Blood pressure USS kidney and UT Bloods full-panel protein and calcium excretion/ACR ```

Question 158

investigation of glomerular haematuria

Answer 158

``` Urine MC&S Blood pressure USS kidney and UT Bloods full-panel protein and calcium excretion/ACR ``` PLUS ESR complement levels throat swab hepB/C screen

Question 159

presentation of nephritic syndrome

Answer 159

``` hypertension oliguria (AKI) periorbital oedema/pulmonary oedema haematuria proteinuria ```

Question 160

most common cause of nephritic syndrome

Answer 160

80% are post-infectious (group A beta haemolytic strep) other causes are IgA, HSP, SLE

Question 161

special investigations of post-strep Glomerulonephritis

Answer 161

C3 (low) ASOT (high) Anti-DNAse B (high)

Question 162

management of post-strep Glomerulonephritis

Answer 162

penicillin 10 day | + management of AKI

Question 163

commonest presentations of HSP

Answer 163

rash on legs and extensor surface (urticarial then maculopapular then purpuric) = 100% GI Sx (colicky pain, bloody diarrhoea) = 75% Joint pain and oedema = 50% Haematuria, oedema are less common

Question 164

haematuria following URTI?

Answer 164

IgA nephropathy (1-2days after) in young adult males OR Post-streptococcal GN (1-2 weeks after) with low C3 complement

Question 165

SLE main autoantibody

Answer 165

dsDNA

Question 166

HUS age group

Answer 166

<5

Question 167

Causes of HUS

Answer 167

E.coli O157 | shigella

Question 168

presentation of HUS

Answer 168

microangiopathic haemolytic anaemia acute renal failure thrombocytopenia

Question 169

treatment of HUS

Answer 169

Mx is supportive fluid blood transfusion dialysis

Question 170

how do you best measure proteinuria

Answer 170

urine albumin to creatinine ratio

Question 171

causes of nephrotic syndrome

Answer 171

MNCS (90%) | FSGS

Question 172

complication of nephrotic syndrome

Answer 172

thrombosis (haemoconcentration and loss of antithrombin III), infection (loss of Ig in urine), hypvolaemia, hyperlipidaemia

Question 173

management of nephrotic syndrome

Answer 173

90% respond to steroids 70% will relapse whether frequently or infrequently penicillin prophylaxis for pneumococcus gastric protection immune modulators (tacrolimus, rituximab, levamisole)

Question 174

investigation to diagnose diabetes insipidus and another to distinguish between types

Answer 174

ADH insensitivity or lack of production - cannot concentrate urine therefore investigation is serum (high) and urine osmolality (low) desmopressin test distinguishes

Question 175

management of DI

Answer 175

central - give desmopressin | nephrogenic - difficult, encourage hydration

Question 176

management of nocturnal enuresis

Answer 176

advice (reassurance, Stop punishments, use reward charts for agreed behaviour not dry nights e.g. toilet before bed) <7 = enuresis alarm >7 = desmopressin if short term control or alarm hasn't worked or alarm

Question 177

treatment of the hyperkalaemia in an AKI

Answer 177

cardiac stabiliser: calcium gluconate push K+ into cells: bicarb insulin/dextrose salbutamol get rid of K+: calcium resonium dialysis

Question 178

treatment of the acidosis in an AKI

Answer 178

bicarb | +/- dialysis

Question 179

stages of CKD

Answer 179

``` eGFR 1 = >90 2 = 60-90 3a = 45-60 3b = 30-45 4 = 15-30 5 = <15 ```

Question 180

top 5 causes for CKD in children

Answer 180

``` renal dysplasia +/- reflux (34) obstructive uropathy (18) glomerular disease (10) congenital nephrotic syndrome (10) PCKD (4) ```

Question 181

presentation of CKD

Answer 181

mostly asymptomatic from stages 1-3 ``` Sx include: bone deformity (vit D) hypertension (RAAS) proteinuria anaemia (EPO) ```

Question 182

management of CKD

Answer 182

high calorie, low protein diet (but enough to grow well), avoiding milk as phosphate leads to 2' hyperpara EPO, vit D and growth hormone dialysis/transplant in stage 5

Question 183

definition of hypertension in children

Answer 183

>95 centile for height age and sex | 100 when born, goes up by 2mmHg every year.

Question 184

4 renal causes of hypertension

Answer 184

scarring vasculitis renal failure coarctation/renal artery stenosis --> low blood to kidneys

Question 185

what happens if you lower BP in malignant hypertension too quickly

Answer 185

watershed infarcts

Question 186

where does the fluid in a hydrocele come from

Answer 186

a small gap in the processus vaginalis allows peritoneal fluid in (small version of a hernia defect)

Question 187

blue spot on testes in acute pain

Answer 187

torsion of hydatid of Morgani

Question 188

inguinal hernias more common on right or left

Answer 188

right 3x

Question 189

when should inguinal hernias be surgically reduced (herniotomy) if manual reduction is not possible: prem baby infant child

Answer 189

prior to discharge within a month elective

Question 190

when should you operate on a hydrocele in an infant/child

Answer 190

if persisting beyond 2y

Question 191

what % of boys have an undescended testes: newborn by 3 months

Answer 191

5% | 1%

Question 192

what do you do if the testes are impalpable bilaterally

Answer 192

karyotype for gender | pelvic ultrasound

Question 193

management of cryptorchidism

Answer 193

orchidopexy at 1y old

Question 194

time window for testicular torsion

Answer 194

6hrs

Question 195

RF for testicular torsion

Answer 195

Bell clapper deformity (no posterior attachment of the gubernaculum). It usually happens bilaterally. This is why you attach both tests to the scrotum when untorting one side.

Question 196

``` match the following girl boy wolfian mullerina mesonephric paramesonephric ```

Answer 196

girl, mullerian, mesonephric

Question 197

at what age are the genitlia fully formed

Answer 197

12 weeks

Question 198

management of balanatis

Answer 198

warm water cleaning hydrocortisone cream imidazole cream if purulent (bacterial), use fluclox PO

Question 199

management of balanitis xerotica obliterate (true phimosis secondary to balanitis)

Answer 199

topical steroid | circumcision

Question 200

behcets

Answer 200

oral ulcer genital ulcer arthritis (anetrior) uveitis amongst other things

Question 201

most common cause of cerebral palsy

Answer 201

80% is antenatal stuff: - cerebrovascular accident - cortical migration disorder - maldevelopment - congenital infection

Question 202

what % of CP is due to hypoxic ischaemic injuries around birth

Answer 202

only 10%

Question 203

3 types of CP and where the lesion is

Answer 203

``` spastic = UMN (pyramidal or corticospinal tract) dystonic = basal ganglia or extrapyramidal tract ataxic = cerebellum ```

Question 204

Sx of spastic CP

Answer 204

velocity dependent increased tone

Question 205

Sx of dystonic CP

Answer 205

chorea athetosis (Writhing movement) dystonia (antagonistic contractions of trunk leading to twisting)

Question 206

Sx of ataxic CP

Answer 206

poor coordination limb hypotonia ataxic gate

Question 207

most common cause of dystonic CP

Answer 207

hyperbilirubinaemia from rhesus disease

Question 208

which is the 'worst' type of CP in terms of GMFCS

Answer 208

dystonic CP, which is usually GMFCS 4 or 5

Question 209

What are the GMFCS scores

Answer 209

``` 1 - no limitation to walking 2 - some limitation to walking 3 - walks with handheld aid 4 - self transported in wheelchair 5 - transported in manual wheelchair ```

Question 210

what complications do you monitor for in CP

Answer 210

``` learning difficulty epilepsy visual impairment squint hearing loss speech disorder behaviour disorder undernutrition respiratory problems ```

Question 211

medication for spasticity in CP

Answer 211

baclofen

Question 212

medication for dystonia in CP

Answer 212

trihexyphenidyl

Question 213

2nd line for dystonia and spasticity in CP

Answer 213

botulinum toxin

Question 214

surgery option for CP

Answer 214

rhizotomy - cutting part of the tracts in the spine

Question 215

which members of the MDT are used in managing CP and why

Answer 215

physio - improve family handling of child, advise activities to prevent complications OT - equipment dietician SALT - swallow in early days and language therapy later Psychologist

Question 216

age range for febrile convulsions to occur

Answer 216

6m-6y (peak is 18m)

Question 217

key question to ask in a seizure history in a child

Answer 217

fever

Question 218

``` If the following area has a seizure, what Sx will there be? frontal temporal occipital parietal ```

Answer 218

``` frontal = motor temporal = aura, dejavu, lip smacking, plucking occipital = vision parietal = sensory ```

Question 219

only motor Sx in absence seizure?

Answer 219

eyelid flicking

Question 220

remission rate of absence epilepsy

Answer 220

80%

Question 221

Mx of absence epilepsy

Answer 221

sodium valproate/ethosuximide

Question 222

how does CECTS present

Answer 222

childhood epilepsy with centre-temporal spikes a.k.a benign rolandic epilepsy: - focal onset seizure - 1/2min, often nocturnal and wake child with gurgling sound - speech arrest and hypersalivation - hemifacial motor seizure - 50% progress to gen ton clon

Question 223

Mx of CECTS/benign rolandic epilepsy

Answer 223

very well managed by making sure child has enough sleep. sleep deprivation is a big trigger. unlikely to ever go away though.

Question 224

typical age for: absence epilepsy CECTS Juvenile myoclonic epilepsy

Answer 224

2-10 7-10 10-20

Question 225

presentation of juvenile myoclonic epilepsy

Answer 225

myoclonic jerks on awakening (often in morning) in 10-20yo

Question 226

investigations in epilepsy

Answer 226

ECG (don't miss arrhythmia like long QT) EEG structural imagine: MRI, CT functional imaging: PET, SPECT

Question 227

1st line absence epilepsy

Answer 227

sodium valproate

Question 228

1st line tonic clonic epilepsy

Answer 228

sodium valproate

Question 229

1st line myoclonic epilepsy

Answer 229

sodium valproate

Question 230

1st line focal epilepsy

Answer 230

carbamazepine or lamotrigine

Question 231

2nd line epilepsy

Answer 231

ethosuximide, lamotrigine, clobazam, levetiracetam, topiramate, gabapentin

Question 232

can children drive if they have epilepsy?

Answer 232

1 year seizure free

Question 233

when can you stop antiepileptics

Answer 233

2 year seizure free

Question 234

management of status epilepticus

Answer 234

``` 0min = ABCDE, O2, DEFG 5min = IV lorazepam, buccal midazolam, rectal diazepam 15min = IV lorazepam 25min = phenytoin (or phenobarbitone) 45min = thiopental rapid sequence induction ```

Question 235

what makes a febrile convulsion 'complex'?

Answer 235

if it is focal as opposed to generalised tonic clonic >15m twice+ in same illness

Question 236

do febrile convulsions increase chance of epilepsy?

Answer 236

simple --> no | complex --> slightly

Question 237

advice for parents after febrile convulsion

Answer 237

1) can't prevent with antipyretics 2) place in recovery position 3) try to film it 4) call 999 after 5 mins

Question 238

parixymasal disorder

Answer 238

page 216

Question 239

what is a breath holding attack and when do they resolve by

Answer 239

resolves by 18 months precipitated by crying/temper tantrum, child takes deep breath and stops breathing, becoming deeply acyanotic, floppy and can have clonic jerks. it lasts no more than a few minutes and there is no post-ictal phase

Question 240

management of breath holding attack

Answer 240

reassurance

Question 241

cause of vasovagal syncope

Answer 241

hypotension and decreased cerebral perfusion

Question 242

how do vasovagal syncope episodes recover

Answer 242

by lying flat

Question 243

what is a reflex anoxic syncope/seizure

Answer 243

sudden unexpected pain or fear causes a vaguely mediated bradycardia --> limp and falls to ground, pale, clonic jerks. lasts 1 minute with rapid recovery

Question 244

Mx of reflex anoxic seizure

Answer 244

reassurance

Question 245

what are parasomnias with examples

Answer 245

Parasomnias are a category of sleep disorders that involve abnormal movements, behaviors, emotions, perceptions, and dreams that occur while falling asleep, sleeping, between sleep stages, or during arousal from sleep. ``` night terrors sleepwalking sleedtalking confusional arounsals narcolepsy ```

Question 246

night terror vs night mare

Answer 246

terror = early in night in stage 4 sleep, children confused, disorientated but still asleep, no recollection, more distressing to watch nightmare = late in night in REM, children then wake up and are aware of having a bad dream, distressing for child

Question 247

by what age should there no longer be a head lag when pulling up a child to sitting

Answer 247

1 months

Question 248

what question do you ask yourself when looking at a child with a motor disorder

Answer 248

Central or peripheral

Question 249

once you've figured out a child has a central motor disorder, what's the next question you ask yourself?

Answer 249

``` motor cortex (weakness) or basal ganglia (chorea, athetosis=writhing, movement initiation) or cerebellum (DANISHP) ```

Question 250

once you've figured out a child has a peripheral motor disorder, what's the next question you ask yourself?

Answer 250

anterior horn cell (signs of denervation:weakness, loss of reflexes, fasciculations, wasting) or neuromuscular transmission problem (diurnal worsening as ACh depletes) or primary muscle disease (proximal muscle wasting) or peripheral neuropathy (specific pattern)

Question 251

Gower's sign

Answer 251

using hands and knees to stand up >3yo = duchenne sign

Question 252

investigation for peripheral motor disorder

Answer 252

``` EMG nerve biopsy muscle biopsy nerve conduction study plasma CK DNA testing ```

Question 253

mutation in spinal muscular atrophy

Answer 253

AR mutation of SMN1 gene for anterior horn cells

Question 254

SMA aka?

Answer 254

spinal muscle atrophy aka Werdnig-Hoffman disease

Question 255

prevalence of spinal muscle atrophy

Answer 255

1 in 6000 (2nd most common neuromuscular disease in UK)

Question 256

What is SMA

Answer 256

progressive weakness and wasting of skeletal muscle cells due to degeneration of LMN in anterior horn of spinal cord

Question 257

types of SMA

Answer 257

0 - survive a few months 1 - flaccid paralysis, absent reflexes, weak cry, poor suck, never sit. death from respiratory failure by 1y 2 - present 3-15m. can sit but never walk 3 - present after 1 y. can walk

Question 258

in what is the mutation for charcot marie tooth disease

Answer 258

myelin gene

Question 259

inheritance of most common mutation of charcot maize tooth disease

Answer 259

CMTA. 2/3 AD inheritance 1/3 de novo

Question 260

how does charcot marie tooth disease present

Answer 260

symmetrical slowly progressive distal muscle wasting (e.g. foot drop). lower limbs more affected than upper limbs

Question 261

how do you diagnose charcot marie tooth disease

Answer 261

nerve conduction study | nerve biopsy = onion bulb (due to attempts at remyelination)

Question 262

how does Guillain barre syndrome present

Answer 262

ascending symmetrical weakness 2-3 weeks post-infection (URTI or campylobacter gastroenteritis) that gets worse over 2-3 weeks. if left long enough, will stop diaphragm and need to ventilate

Question 263

management of GBS

Answer 263

supportive | immunoglobulin can help

Question 264

prognosis of GBS

Answer 264

90% recover, but can take up to 2 years.

Question 265

LMN vs UMN facial palsy

Answer 265

upper spares upper

Question 266

management of bells palsy

Answer 266

investigate for lyme disease and HSV if HSV-->acicilovir lubricate eye and wear patch if can't close eye

Question 267

cause of myaesthenia gravis

Answer 267

antibodies to nACh receptors in NMJ

Question 268

presentation of myasthenia gravis

Answer 268

``` after 10yo eye movement problem ptosis loss of facial expression proximal muscle weakness ```

Question 269

diagnosis of myasthenia gravis

Answer 269

improvement after neostigmine and identification of antibodies in blood

Question 270

management of myasthenia gravis

Answer 270

neostigmine immunosuppressive therapy plasma exchange in crisis

Question 271

inheritance of duchenne

Answer 271

X-linked recessive | but 1/3 are de novo

Question 272

gene that is mutated in duchenne

Answer 272

dystrophin - connects muscle fibre to extracellular matrix - when mutated there is myofibre necrosis

Question 273

incidence of duchenne

Answer 273

1 in 3000-6000 males

Question 274

presentation of duchenne

Answer 274

``` waddling gait language delay towers sign psudoheypertrophy of calves leaning difficulties clumsiness ```

Question 275

usual onset of duchenne

Answer 275

3-5

Question 276

Becker's muscular dystrophy

Answer 276

like duchenne, but not as bad a mutation so onset is later (11) and not as severe

Question 277

life expectancy with duchenne

Answer 277

20s - because as breathing muscles become affected you get infections. also cardiomyopathy. no longer ambulant by 10-14

Question 278

presentation and age for dermatomyositis

Answer 278

``` 5-10y symmetrical proximal muscle weakness (CK up) heliotrop rash gottron papules fever raised CRP/ESR ```

Question 279

management of dermatomyositis

Answer 279

2yrs corticosteroids immunosuppression physio

Question 280

how does friedrich ataxia present

Answer 280

``` similar to charcot marie tooth: worsening ataxia dysarthria distal wasting of limbs absent reflexes death by 40-50 ```

Question 281

what is friedrich ataxia caused by

Answer 281

AR mutation of FXN gene --> lack of frataxin protein

Question 282

headache red flags

Answer 282

``` early morning headaches waking up at night worse headache ever had rapid onset neck stiffness TMJ pain rash focal neurological signs increasing head circumference altered conscious level personality change worse lying down ```

Question 283

migraine more common in boys or girls

Answer 283

boys>girls | after puberty its women>men

Question 284

how do cluster headaches present

Answer 284

non pulsatile severe behind the eye pain. can cause unilateral redness/swelling of eye. attacks occur in clusters a few times a day for a period of weeks

Question 285

how do you manage cluster headaches

Answer 285

triptans for acute attack | Ca blockers for prevention

Question 286

headache type in raised ICP

Answer 286

worse lying down worse in morning wakes you up morning vomiting

Question 287

Mx of raised ICP

Answer 287

ABCDE elevate to 25 degrees to help drainage intubate, mannitol, refer to neuro centre if severe

Question 288

what brain bleed is classic in NAI (non-accidental injury)

Answer 288

subdural

Question 289

most common cause of meningitis

Answer 289

viral (enterovirus, EBVm adenosine, mumps)

Question 290

bacterial causes of meningitis: | 0-3m

Answer 290

GBS Ecoli Listeria

Question 291

bacterial causes of meningitis: | 3m+

Answer 291

Neisseria meningitides strep pneumonia (Haemophilis as well if <6)

Question 292

kernig vs brudninski

Answer 292

``` kernig = hamstring stretch brud = neck flexion causes knee flexion ```

Question 293

CI to LP

Answer 293

- focal neurology - coagulopathy - infection at LP site - signs of raised ICP - thrombocytopenia - cardiorespiratory instability

Question 294

what is the fluid challenge amount given to children

Answer 294

20ml/kg

Question 295

what antibiotics are used

Answer 295

``` <3m = cefotaxime >3m = ceftriaxone ``` give cipro to rifamp to household contacts

Question 296

what is X-linked adrenoleukodystrophy a disease of

Answer 296

peroxisome enzyme defect, leading to an accumulation of long chain fatty acids. it causes a neurodegenrative disorder

Question 297

what is wilsons disease

Answer 297

- AR mutation of Chr 13 - Leads to abhorrent storage of copper in many organs --> liver failure, psychosis, extrapyramidal signs - Presents in childhood around 12yo

Question 298

how is wilsons disease diagnosed

Answer 298

Diagnosed by reduced copper/caeruloplasmin in blood and urine (as it’s all in the liver) or by liver biopsy

Question 299

management of wilsons disease

Answer 299

low copper diet and penicillamine (copper chelation)

Question 300

diagnosis of type 1

Answer 300

>1 neurofibroma >6 cafe au lait spots other things like axillary freckling, optic glioma, lisch nodule, relatives

Question 301

mutation in neurofibromatosis

Answer 301

AD mutation of NF1 gene, but 50% have a de novo mutation

Question 302

what is neurofibromatosis associated with

Answer 302

MEN syndromes

Question 303

features of tuberous sclerosis

Answer 303

depigmented ash leaf shaped patches | angiofibrimata in butterfly distribution on bridge of nsoe

Question 304

what is sturge weber syndrome

Answer 304

a neurological condition where you get: - mental retardation - seizures - port wine stain in trigeminal distribution - glaucoma effectively, it is a birthmark (capillary malformation) that affects the skin and brain

Question 305

4 developmental domains

Answer 305

fine motor gross motor hearing and language social

Question 306

investigations for developmental delay

Answer 306

``` urine tests for metabolic problems TFTs chromosome analysis CGH array exome sequencing imaging (MRI) ```

Question 307

fever + change in personality

Answer 307

encephalitis

Question 308

catergories for causes of anaemia

Answer 308

Don't make enough - iron def, folic acid def, chronic renal failure RBCs get destroyed - hereditary spherocytosis, G6PD, thalassaemia, sickle, immune things like haemolytic disease Blood loss - fetomaternal bleeding, meckel diverticulum, vWF disease

Question 309

what does low reticulocyte mean

Answer 309

red cell production issue

Question 310

why os bilirubin helpful when looking at cause of anaemia

Answer 310

tells you if haemolysis is the problem

Question 311

investigation for sickle cell and thalssaemia?

Answer 311

Hb electrophoresis

Question 312

microcytic normocytic macrocytic definitions

Answer 312

micro <83 fL normal macro >96 fL

Question 313

``` Micro, normo or macro? iron def B12/folate haemolysis thalaseamia chronic disease hypothyroidism alcohol excess myelodysplasia ```

Answer 313

``` micro macro normo/macro micro micro/normo macro macro macro ```

Question 314

iron def anaemia: FBC blood film ferritin

Answer 314

microcytic, hypochromic anaemia with low ferritin

Question 315

if no improvement in iron def anaemia, what should you investigate for

Answer 315

``` coeliac mockers diverticulum (chronic bleeding) ```

Question 316

causes of red cell aplasia (anaemia)

Answer 316

parvovirus B19 | diamond-blackfan congenital anaemia

Question 317

``` red cell aplasia: reticulocyte count bilirubin Coombs test red cell precursors in bone marrow ```

Answer 317

Low Normal Negative for Abs Absent

Question 318

inheritance of hereditary spherocytosis and G6PDd

Answer 318

AD (northern european children) | G6PD = X-linked (African&Mediterranean boys)

Question 319

How can HS and G6PD present

Answer 319

neonatal jaundice gallstones haemolysis after infection

Question 320

Ix for HS and G6PD

Answer 320

``` HS = spherocytes on blood film G6PD = G6PD activity ```

Question 321

incidence of sickle cell

Answer 321

1 in 2000

Question 322

inheritance of sickle cell

Answer 322

AR

Question 323

mutation in sickle cell

Answer 323

glu to val point mutation to form HbS chain

Question 324

presentation of sickle cell

Answer 324

i. Acute – splenic crisis, stroke, venous thrombus, priapism, acute chest syndrome, PE. ii. Chronic – gallstones, blindness, kidney failure, leg ulcers, bony pain iii. Hyposplenism causing infection

Question 325

Sickle: Hb reticulocytes LDH

Answer 325

Low High High

Question 326

emergencies in sickle cell?

Answer 326

Acute chest syndrome A vaso-occlusive crisis of pulmonary vasculature pulmonary infiltrate, visible on a chest X-ray. Requires close monitoring and potentially a transfusion to the % of HbS Neurological deficits Probably a stroke. Difficult to be picked up as these patients don’t present like usual stroke victims who are old + atheromatous. Needs usual stroke care and a transfusion to the % of HbS Infection Encapsulated bacteria pose a big risk to sickle patients who have badly functioning spleens, especially in children. Prophylactic penicillin is used to prevent this Acute splenic sequestration Spleen rapidly expands and Hb drops rapidly

Question 327

when to transfuse in sickle cell

Answer 327

in the 4 emergencies

Question 328

long term medication for sickle cell

Answer 328

hydroxycarbamide

Question 329

types of beta thalassaemia

Answer 329

``` carrier = one normal, one faulty beta haemoglobin gene. can still make HbA (=alpha+beta) intermedia = both faulty but still working. variable Sx. major = no functioning HbA, will die if untreated ```

Question 330

clinical features of beta thalasaemia

Answer 330

1. Normal foetal life (as and genes are ok so HbF can be made) 2. Anaemia in first few months of life (as HbA take sover) 3. Jaundice 4. Medullary hyperplasia (as bone marrow tries desperately to make RBCs) leading to: i. Facial changes due to meduallry hyperplasia and frontal bossing ii. ‘Hair on end’ appearance of cranium due to medullary expansion 5. Splenomegaly/hepatomegaly (due to the extra-medullary haematopoiesis)

Question 331

Mx of B thal

Answer 331

1. Life long regular blood transfusions 2. Iron chelation (to prevent iron overload from transfusions) = desferrioxamine 3. Folic acid 4. Bone marrow transplantation in early life

Question 332

incidence of leukaemia

Answer 332

3 per 100,000

Question 333

types of leukaemia in children

Answer 333

80% ALL | 20% AML

Question 334

types of ALL

Answer 334

75% common | 15% T-cell

Question 335

peak incidence of ALL

Answer 335

2-5

Question 336

Sx and duration of onset of Sx in ALL

Answer 336

over several weeks: - general malaise and anorexia - bone marrow infiltration (anaemia so pale and tired, neutropenia so infections, thrombocytopaenia so bruising petechiae and nose bleeds) - hepatosplenomegaly and lymphadenopathy as RES tries to take over blood production. acutely as DIC

Question 337

prevention of tumour lysis syndrome?

Answer 337

allopurinol: prevents uric acid production as all the cells break down

Question 338

investigations for leukaemia

Answer 338

``` FBC blood film bone marrow examination clotting screen (?DIC) CXR (?mediastinal disease) LP (?CSF disease) Cytogenetic testing ```

Question 339

management for leukaemia

Answer 339

correct abnormalities: - RBC transfusion - platelets Chemotherapy: 1. Multidrug chemotherapy in 3 phases: i. Induction phase – 3 months’ intense chemotherapy to induce remission ii. Consolidation phase – 4 months’ intense chemotherapy to consolidate the remission iii. Maintenance phase – 2 years less intense chemotherapy to maintain remission 2. Some patients get a transplant

Question 340

bad prognostic indicators for leukaemia

Answer 340

``` age < 2 years or > 10 years WBC > 20 * 109/l at diagnosis T or B cell surface markers non-Caucasian male sex ```

Question 341

prognosis in ALL at 5 years

Answer 341

85%

Question 342

prognosis of AML at 5 years

Answer 342

60-70%

Question 343

bleeding: muscles and joints vs mucocutaneous

Answer 343

haemophilia (coagulation cascade problem) platelet disorders and vWF (primary haemostats and platelet plug formation)

Question 344

inheritance of haemophilia

Answer 344

X-linked recessive

Question 345

types of haemophilia

Answer 345

``` A = factor 8 deficiency B = factor 9 deficiency ```

Question 346

when does haemophilia present

Answer 346

end of first year of life when they start crawling/walking/falling

Question 347

what is the severity spectrum with haemophilia

Answer 347

severity depends on how bad the mutation to the gene is: severe = sponteneous bleed moderate = bleed after minor trauma mild = bleed after surgery

Question 348

what will the following show for haemophilia: - aPTT - PT - F8&9 - FBC

Answer 348

- long - normal - low depending on type a or b - normal

Question 349

which factors are involved with: - intrinsic pathway - extrinsic pathway - common pathway

Answer 349

- extrinsic = F7 - intrinsic = F8, 9, 11 - common = F10 and others

Question 350

what does aPTT and PT test

Answer 350

activated PTT = intrinsic (8, 9, 11) PT = extrinsic (7) both = common

Question 351

treatment for haemophilia in general special treatment for haemophilia A

Answer 351

recombinant factor when bleeding avoid aspirin, NSAIDs and IM injections Hameophilia A can also have desmopressin, which releases endogenous stores of F8

Question 352

what does vWF do

Answer 352

it lives in the serum and when there is an injury it binds to the exposed collagen. it then also binds to platelets in the plasma and activates them forming the primary platelet plug it also stabilises F8 in the plasma

Question 353

presentation of vWD

Answer 353

bruising excessive bleeding after surgery epistaxis or menorrhagia

Question 354

treatment of vWD

Answer 354

1. Tranexamic acid – reduces clot breakdown (a.k.a. an anti-fibrinolytic) 2. DDAVP/Desmopressin – this releases endogenous FVIII and vWF. This is good for if you need a short term boost. 3. vWF/FVIII concentrate – very expensive so is a last line therapy, but has to be used before things like surgery.

Question 355

HSP more common in boys or girl

Answer 355

boys 2x

Question 356

what is ITP

Answer 356

Immune (or idiopathic) thrombocytopenic purpura (ITP) is an immune-mediated reduction in the platelet count. IgG Antibodies are directed against the glycoprotein IIb/IIIa (like abciximab) or Ib-V-IX complex. Immune thrombocytopenia (ITP) in children it is typically more acute than in adults equal sex incidence may follow an infection or vaccination presents as petechiae, purpura, bruising, usually runs a self-limiting course over 1-2 weeks Chronic ITP more common in young/middle-aged women tends to run a relapsing-remitting course

Question 357

what do protein C and S do?

Answer 357

'put brakes' on the clotting cascade. so a deficiency means there's no brake on the cascade and it is a pro-thrombotic state

Question 358

investigations for a child with a VTE

Answer 358

95% it is due to an underlying disorder, so must go looking for it: - protein C and S levels - antithrombin assay - PCR for factor V leiden and prothrombin mutation

Question 359

presentation of lymphoma

Answer 359

``` painless lymphadenopathy over several months B symptoms: - night sweats - fever - weight loss ```

Question 360

hodgkin vs non-hodgkin: histology age

Answer 360

reed sternberg cells in hodgkin NHL = childhood HL = adolescence

Question 361

investigations for lymphoma

Answer 361

lymph node biopsy bone marrow biopsy for staging radiological assessment bloods (LDH, FBC, urate, Ca)

Question 362

cure rate for hodgkins

Answer 362

80%

Question 363

long term complication from hodgkin

Answer 363

secondary malignancy or heart failure from radiotherapy at young age

Question 364

what is burkitt's lymphoma

Answer 364

a high grade B cell NHL: - endemic (african) form is associated with EBV = in face and jaw - sporadic form = ileocaecal tumours

Question 365

Li-Fraumeni syndrome mutation

Answer 365

p53 germ line mutation causing early cancer (breast, leukaemia, sarcoma)

Question 366

mutation causing WIlms tumour

Answer 366

WT1

Question 367

most common solid tumour in children

Answer 367

brain

Question 368

what does vomiting with no diarrhoea raise alarm bells of

Answer 368

brain tumour

Question 369

key feature of medulloblastoma

Answer 369

cerebellar signs

Question 370

key feature of astrocytoma

Answer 370

supratentorial signs: seizure, hemiplegia, focal signs

Question 371

from what do neuroblastoma arise?

Answer 371

neural crest tissue in adrenal medulla and sympathetic nervous system (NOT BRAIN)

Question 372

features of neuroblastoma

Answer 372

``` abdominal mass pallor, weight loss bone pain, limp hepatomegaly paraplegia proptosis ```

Question 373

key investigation in neuroblastoma

Answer 373

urine catecholamines are raised: | raised urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels

Question 374

what is Wilms tumour

Answer 374

a nephroblastoma = cancer arising from embryonal renal tissue

Question 375

commonest presentation of wilms tumour

Answer 375

abdominal mass | +/- haematuria

Question 376

Tx of Wilms

Answer 376

chemo + nephrectomy | 80% cure

Question 377

age group for bone tumours

Answer 377

after puberty

Question 378

M:F in bone tumours

Answer 378

M > F

Question 379

presentation of bone tumours

Answer 379

persistent localised bone pain

Question 380

2 types of bone tumours and most likely place to arise

Answer 380

Osteosarcoma (knee or shoulder) Ewing's (femoral diaphysis or hip)

Question 381

prevalence in UK of T1DM

Answer 381

1 in 500

Question 382

name a: rapid short intermediate acting insulin

Answer 382

novorapid humulin-S humulin-I

Question 383

what type of insulin regime do children start on?

Answer 383

basal bolus

Question 384

``` how do the following affect blood glucose? illness alcohol anxiety/excitement stress menstruation hot weather ```

Answer 384

``` up down down up up down down ```

Question 385

long term complications of diabetes

Answer 385

``` macrovascular = HTN, CVD, CHD microvascular = retinopathy, nephropathy, peripheral neuropathy ```

Question 386

catergories for osce station looking at 'assessing a child with diabetes'

Answer 386

``` assess diabetes - symptoms - complications assess impact on life - school - growth and development - psychological ```

Question 387

biochemical cutoffs for DKA

Answer 387

sugar >11.1 blood ketones >3 VBG <7.3 +/- bicarb <15

Question 388

treatment of DKA

Answer 388

1) ABCDE 2) rehydrate with 0.9% saline 3) insulin infusion (+ add 5% glucose when <14mmol/L) 4) correct potassium 5) later, establish PO fluids and SC insulin. identify cause of DKA

Question 389

causes of hypoglycaemia in infancy

Answer 389

transient neotatal hypoglycaemia persistent hypoglycaemic hypoerinsulinism of infancy

Question 390

causes of hypoglycaemia after neonatal period

Answer 390

fasting hypoglycaemia: - with insulin excess (overtreatment, insulinoma) - without insulin excess (hormone def, liver disease, inborn errors of metabolism/ GSDV) Non-fasting hypoglycaemia: - maternal diabetes, galactosaemia, fructose intolerance

Question 391

Tx of hypoglycaemia

Answer 391

``` PO glucose Glucagon injection (if LOC) IV glucose (if glucagon doesn't work) ```

Question 392

what is McArdle disease

Answer 392

most common type of glycogen storage disorder (i.e. inborn error of metabolism that causes fasting hypoglycaemia without insulin excess). GSD V. characterized by exercise intolerance relieved by rest

Question 393

what does RAAS do to: - BP - Na - K

Answer 393

increases BP increases Na decreases K

Question 394

CAH cause

Answer 394

21-a-hydroxylase deficiency in 90% of cases. This leads to: - no aldosterone - no cortisol - buildup of 17OHP substrate that shunts towards making too much testosterone

Question 395

CAH presentation

Answer 395

- virilisation of external genitalia - salt loss adrenal crisis in 1-3 weeks - tall stature, muscular build, acne

Question 396

diagnosis of CAH

Answer 396

high 17OHP in blood

Question 397

management of CAH

Answer 397

- lifelong glucocorticoids - lifelong mineralocorticoids - ?surgery to correct genitalia

Question 398

What is addisons disease

Answer 398

primary adrenal insufficiency, often caused by AI destruction of adrenals

Question 399

presentation of addisons: - infants - older

Answer 399

infants: salt loosing crisis (lowNa, highK, met acidosis) older: lethargy and pigmentation

Question 400

diagnosis of addisons - plasma cortisol - ACTH - synacthen

Answer 400

- low cortisol - high ACTH - cortisol stays low on synacthen test

Question 401

Mx of adrenal crisis

Answer 401

IV saline glucose hydrocortisone

Question 402

Ix of cushings syndrome: - dex suppression - 24hr urine cortisol

Answer 402

- dex at night doesn't suppress cortisol at 9am next morning | - high urinary cortisol

Question 403

what type of T3 do fetuses make

Answer 403

reverse T3 = inactive derivative

Question 404

incidence of congenital hypithyroidism

Answer 404

1 in 4000

Question 405

causes of congenital hypothyrpidism

Answer 405

1) iodine deficiency (most common worldwide but not in UK) 2) maldescent of thyroid from tongue to larynx 3) pituitary dysfunction 4) inborn error of thyroid syntheiss

Question 406

what is a Guthrie test what it looks for in cong hypothy

Answer 406

the neonatal heelprick test done when babies are 5d old raised TSH

Question 407

Mx of cong hypothy

Answer 407

lifelong thyroxine from 2-3 weeks of age (otherwishe neurodevelopment will be very slow)

Question 408

Mx of hyperthyroidism

Answer 408

beta blocker for Sx carbimazole = pro-drug that blocks thyroid peroxidase enzyme that puts idone on thyroglobulin to make T3 and T4 propiothyiouriaciol = blocks T4 to T3 conversion block (radiotherapy thyroidectomy) and replace (thyroxine)

Question 409

most common inherited metabolic disorder

Answer 409

phenylketonuria and MCADD (1 in 10,000)

Question 410

Presentation and management of: | phenylketonuria

Answer 410

learning difficulties, seizures, microcephaly phenylalanine restricted diet

Question 411

Presentation and management of: | MCADD

Answer 411

hypoglycaemia if fasting don't fast

Question 412

Presentation and management of: | glutaric aciduria type 1

Answer 412

macrocephaly with encephalopathic crisis 6-18m resulting in dystonic-dyskinetic movements specialist diet and dialy carnitine

Question 413

Presentation and management of: | isovaleric acidaemia

Answer 413

metabolic acidosis low [protein diet and carnitine/glycine

Question 414

Presentation and management of: | homocystinuria

Answer 414

marfanoid appearance, lens dislocation, osteoporosis, VTE low protein diet and pyridoxine/folate

Question 415

maple syrup urine disease

Answer 415

maple syrup smelling urine and encephalopathy in first week of life low protein diet

Question 416

causes of ambiguous genitalia: - if female - if male

Answer 416

- CAH, prenatal exposure to androgens | - 5areductase deficiency (no DHT from testosterone so can't virilise), androgen insensitivity, impaired testes

Question 417

how long is the growth phase for an infantile haemangioma

Answer 417

9m

Question 418

how long do haemangiomas take to involute

Answer 418

50% by 5y | 70% by 7y

Question 419

Mx of haemangioma

Answer 419

Conservative | propranolol if complications such as obstructed vision, ulceration, pain, bleeding or disfigurement

Question 420

What is PHACES

Answer 420

``` variation of infantile haemangiomas including: Posterior fossa defect Haemangioma Arterial abnormalities Cardiac abnormalities Eye abnormalities Sternal clefting ```

Question 421

what is sturge weber syndrome

Answer 421

port wine stain (a flat haemangioma) in trigeminal distribution + neurological problems (seizure, developmental delay) + eye problems (blindness, retinal detachment, glaucoma)

Question 422

incidence of port wine stain

Answer 422

1 in 300

Question 423

natural progression of port wine stain

Answer 423

darken and thicken with time. permanent.

Question 424

what are port wine stains caused by

Answer 424

slow flow capillary vascular malformations

Question 425

what is an exanthem

Answer 425

``` a rash that appears abruptly affecting several areas of the skin simultaneously. 6 classic ones are: measles scarlett fever rubella staph scalded skin erythema infectiosum/slapped cheek roseola ```

Question 426

caused of measles

Answer 426

paramyxovirus/morbillivirus

Question 427

presentation of measles

Answer 427

``` conjunctivitis koplik spots cough THEN widespread maculopapular/morbilliform rash ```

Question 428

complications from measles

Answer 428

encephalitis pneumonia otitis media subacute sclerosing panencephalitus 7 years later (like dementia)

Question 429

scarlett fever cause

Answer 429

group A strep

Question 430

scarlett fever presentation

Answer 430

strawberry tongue sandpaper rash circumoral pallor

Question 431

management of scarlett fever

Answer 431

penicillin

Question 432

cause of rubella

Answer 432

rubella virus

Question 433

presentation of rubella

Answer 433

prodrome, e.g. low-grade fever rash: maculopapular, initially on the face before spreading to the whole body, usually fades by the 3-5 day lymphadenopathy: suboccipital and postauricular Forschemer's spots (petechiae on hard palate)

Question 434

complications of scarlett fever

Answer 434

otitis media pneumonia glomerulonephritis rheumatic fever

Question 435

complications of rubella

Answer 435

congenital rubella syndrome in 1st trimester

Question 436

diagnosis of rubella

Answer 436

unlike the other exanthems, this needs serology

Question 437

SSSS cause

Answer 437

S. aureus toxins

Question 438

SSSS presentation

Answer 438

Erythema following by blistering with Nikolsky's sign. No mucosal involvement Pain, fever, shock (from fluid loss)

Question 439

Mx of SSSS

Answer 439

``` IV Abx emolients analgesia dressings fluid and temperature balance ```

Question 440

erythema infectiosum AKA and cause

Answer 440

a.k.a 5th disease a.k.a slapped cheek syndrome caused by parvovirus B19

Question 441

presentation of erythema infectiosum

Answer 441

slapped cheek for 4 days with circumoral sparing | mesh like net red appearance across buttocks and trunk after a week

Question 442

roseola infantum presentation and cause

Answer 442

6m old sudden onset fever which then GOES away AND THEN maculopapular rash appears HHV6 and HHV7 (think that it is 6th disease)

Question 443

causes of bullous and non bullous impetigo

Answer 443

non-bullous - S. aureus and Strep pyogenes (group A) bullous - S. aureus

Question 444

Mx of periorbital or orbital cellulitis

Answer 444

High dose ceftriaxone

Question 445

what is a herpetic whitlow

Answer 445

painful erythematous oedematous white pustules on site of broken skin. typically fingers. caused by autoinnoculation

Question 446

chicken pox cause

Answer 446

VZV (HHV-3)

Question 447

how many herpes viruses are there and what are they

Answer 447

``` 8: HSV1 - lip and skin lesions HSV2 - genital lesions VZV - chicken pox CMV EBV HHV6 - roseola HHV7 - roseola HHV8 - kaposi ```

Question 448

after how long should new lions stop arising gin chicken pox

Answer 448

10 days, after which you worry of immunocompromise

Question 449

cause of warts

Answer 449

human papillomavirus

Question 450

how long do molluscum take to go away

Answer 450

up to a year

Question 451

what is a kerion

Answer 451

a severe pustular ringworm (dermatophyte) infection

Question 452

what is seborrhoeic dermatitis and aka

Answer 452

aka cradle cap | scales form thick yellow adherent layer on scalp of <3m baby

Question 453

Mx of cradle cap

Answer 453

emolients clears scales using ointments containing sulphur and salicylic acid ?topical steroid

Question 454

What is nappy rash

Answer 454

an irritant contact dermatitis from urine/diarhoea on skin

Question 455

where does nappy rash occur

Answer 455

Nappy area, but spares the flexures/skin creases

Question 456

Mx of nappy rash

Answer 456

emollient + ?steroid

Question 457

genetics in eczema

Answer 457

fillagrin null mutation

Question 458

Mx of eczema (brief)

Answer 458

soap/irritant avoidance emollients steroids

Question 459

what is erythema nodosum

Answer 459

submit fat inflammation causing tender red nodules on skins

Question 460

erythema multiforme appearance

Answer 460

target lesions WITH A CENTRAL RED PAPULE

Question 461

area of body most likely to be injured in a fatal childhood accident

Answer 461

head injury

Question 462

presentation of brain bleed in children: neuro or shock

Answer 462

compared to adults, relatively more shock because the sutures aren't fused so there is less mass effect and fewer neuro signs

Question 463

management of choking

Answer 463

unconscious = CPR conscious: child = 5 abdo thrusts infant = 5 back blows, 5 chest thrusts

Question 464

when to refer to burns unit

Answer 464

- partial thickness burn >5% of - body surface area deep partial thickness/full thickness - face, ears, hands, feet, genitalia, perineum, major joint affected

Question 465

grade of burns which grades appear dry

Answer 465

superficial = epidermis only partial = into dermis. can be superficial or deep type depending on depth full thickness = into subcutaneous fat and tissue superficial and full are dry, other is moist

Question 466

when is activated charcoal useful in a poisoning

Answer 466

within 1 hour of poisoning to prevent some of it being absorbed

Question 467

Sx of paracetamol overdose

Answer 467

Early = abdo pain + vomiting | Late (12-24hr) = liver failure

Question 468

Mx of paracetamol overdose

Answer 468

Serum paracetamol concentration, INR and LFT IV NAC if required

Question 469

Sx of salicylate overdose

Answer 469

``` Early = tinnitus + vomiting + hyperventilation (respects alkalosis) Late = metabolic acidosis ```

Question 470

Mx of salicylate overdose

Answer 470

Serum salicylate Alkalinisation of urine Haemodialysis

Question 471

Sx of alcohol overdose

Answer 471

Hypoglycaemia Coma Respiratory failure

Question 472

Mx of alcohol overdose

Answer 472

Serum ETOH Treat hypoglycaemia Support ventilation

Question 473

what is blount disease

Answer 473

severe progression of unilateral leg bowing (genu varum)

Question 474

what measurement can be used to see whether someone with knock knees (genu valgum) is normal

Answer 474

Intermalleolar distance <8cm when standing with knees together

Question 475

pathological cause of genu valgum

Answer 475

JIA

Question 476

examination for flat feet

Answer 476

Ask to go on tip toes and observe arch | Aldo passively extend big toe

Question 477

do you refer flat feet?

Answer 477

if there is evidence of a pathological cause: | hyper mobility, contracture, tarsal coalition, JIA

Question 478

causes of in-toeing: - infant - toddler - child

Answer 478

metatarsus adductus medial tibial torsion persistent anteversion of femoral neck

Question 479

clubfoot name and deformity

Answer 479

talipes equinovarus: - inversion and supination of foot with plantarflexion - shorter foot and thin calf muscles - foot is fixed

Question 480

incidence and M:F of clubfoot

Answer 480

1 in 1000 | male x2

Question 481

Mx of clubfoot

Answer 481

Ponsetti method = plaster casting and bracing | Severe = corrective surgery

Question 482

Deformity in talipes calcaneovalgus

Answer 482

dorsiflexion and turned out/everted

Question 483

definitions: talipes: - varus - valgus - equinus - calcaneus

Answer 483

``` turned in (like inversion) turned out (like eversion) like plantarflexion like dorsiflexion ```

Question 484

Mx of calcaneovalgus

Answer 484

self corrects with exercises

Question 485

how does tarsal coalition present

Answer 485

progressively more rigid foot, presenting in pre-adolescent years

Question 486

Pes cavus deformity

Answer 486

high arched foot.

Question 487

Pes cavus in older children: association?

Answer 487

Neuromuscular disorder (e.g friedrich ataxia, hereditary motor sensory disorder)

Question 488

how might DDH present

Answer 488

neonatal screening | Limp/abnoaml gait

Question 489

type of pain in growing pain

Answer 489

``` 3-12y at night, symmetrical NEVER at start of day doesn't limit activities pGALS normal ```

Question 490

score for hypermobility

Answer 490

beighton score | >6/9 is hypermobile

Question 491

Mx of hypermobility

Answer 491

Investigate for Marfan or Ehlers-Danlos MDT treatment: PT/OT, orthotics, simple pain relief

Question 492

what is complex regional pain syndrome and Mx

Answer 492

non-organic dramatic MSK pain with hyperaesthesia and allodynia. Mx is PT based

Question 493

presentation of transient synovitis

Answer 493

<5yr hip pain referred to knee with a preceeding URTI mild fever (not really ill like septic arthritis) improves in a few days

Question 494

Mx of transient synovitis

Answer 494

Worried about differential of septic arthritis, so do Xray, FBC, CRP, USS If unsure joint aspirate and cultures

Question 495

Presentation of perthes disease

Answer 495

young boy 5-10 | insidious onset of limp, hip/knee pain.

Question 496

Imaging in perthes disease or SUFE

Answer 496

frog leg lateral view Xray of both hips to show AVN MRI can be helpful

Question 497

does the hip reossify in perthes disease? Any long term consequences?

Answer 497

Yes, over 18-36 months. Risk of degenrative arthritis in adult life

Question 498

Mx of perthes

Answer 498

Rest and PT to optimise hip movement | Surgery sometimes used

Question 499

Presentation of SUFE

Answer 499

hip pain and limp can be insidious or acute. In obese 10-15yr old boy.

Question 500

Mx of SUFE

Answer 500

urgent referral and surgical pin fixation

Question 501

cause of chondromalacia patellae

Answer 501

soft cartilage of patella causes pain when tightly opposed to femoral condyles (pain walking up stairs/getting up from chair)

Question 502

presentation of reactive arthritis

Answer 502

pain, swelling, redness, restricted ROM oligoarthritis FOLLOWING an infection. Usually not ill like in septic arthritis, and usually affects more than one joint.

Question 503

Mx of reactive arthritis

Answer 503

NSAIDs and reassurance

Question 504

usually place for septic arthritis

Answer 504

knee

Question 505

common age for septic arthritis

Answer 505

<2

Question 506

pathogen for septic artheitis

Answer 506

S. aureus

Question 507

Mx of septic arthritis

Answer 507

Get joint aspirate and send it off IV Abx Surgical washout if no improvement or if in hip immobilise and then subsequent mobilisation

Question 508

what is JIA

Answer 508

umbrella term for different types of juvenile arthritides: - systemic - oligoarthritis (most common) - polyarhtirits - psoriatic - enthesitis related

Question 509

What is Still's disease

Answer 509

systemic arthritis type of JIA where there is arthritis AND fever, rash, hepatosplenomegaly, lymphadenppathy

Question 510

in polyarthritis type JIA, who is RF+

Answer 510

10-16yo. | the 1-6yo tend to be RF-

Question 511

which JIA subtype is more common in boys

Answer 511

enthesitis related arthritis

Question 512

incidence of JIA

Answer 512

1 in 1000

Question 513

complications of uveitis

Answer 513

posterior synechiae, cataract, glaucoma, band keratopathy

Question 514

management of uveitis

Answer 514

Steroid (drops, oral, IV) Methotrexate Humira

Question 515

common complication of JIA

Answer 515

chronic anterior uveitis 33% | Need ophthalmological review every 3m

Question 516

management of JIA

Answer 516

Paediatric rheumatologist! PT Analgesia (ibuprofens/naproxen NOT opiod or COX2) Immunosuppression (MTX - no alcohol, folate and LFT monitoring monthly - steroids or biologics) 3/12 opthalm review encourage to do everything even during flare

Question 517

common spread type in chilren with osteomyelitis

Answer 517

haematogenous | compared to adults where its usually contiguous (e.g. trauma)

Question 518

common area for haematogenous spread in osteomyelitis

Answer 518

metaphyseal area of long bone

Question 519

presentation of osteomyelitis

Answer 519

``` PAINPAINPAIN pseudo paresis (can't move limb) ```

Question 520

Mx of osteomyelitis

Answer 520

several weeks of IV Abx and oral therapy afterwards. main need surgery to debride or drain

Question 521

common site of osteoid osteoma

Answer 521

spine

Question 522

what is Scheuermann disease

Answer 522

osteochrontisi of vertebral body

Question 523

what is adams forward bend test

Answer 523

ask someone with scoliosis to bend over and touch toes - scoliosis resolves = functional scoliosis - scoliosis doesn't resolve = structural scoliosis

Question 524

what is torticollis

Answer 524

dystonic condition causing abnormal neck position

Question 525

most common cause of torticollis

Answer 525

sternomastoid tumour, which occurs in first few months of life. presents as a mobile non tender nodule in body of sternocleidomastoid muscle

Question 526

mutation in achondroplasia

Answer 526

FGFR3 AD | but 50% are new mutations

Question 527

what is cleidocranial dysostosis

Answer 527

AD disorder. absence of part or all fo clavicles and delayed closure of anterior fontanelle. can bring shoulder in from of chest and have short stature. STRANGER THINGS BOY ACTOR

Question 528

what is arthrogryposis

Answer 528

defined as >2 congenital contractures, associated with oligohydramnios or chromosomal dosorders

Question 529

most common type of osteogenesis imperfecta and presentation

Answer 529

type 1 = AD = fractures in childhood, blue sclera, hearing loss

Question 530

Mx of OI type 1

Answer 530

bisphosphonates and fracture splinting

Question 531

presentation of type 2 OI

Answer 531

still born

Question 532

what is osteopetrosis

Answer 532

MARBLE BONE DISEASE! carbonic anhydrase and proton pump are not working, meaning osteoclasts cannot properly resorb bone. This means you get extremely dense bones with high mineral content that are brittle. Also, patients get problems with anaemia/thrombocytopaenia because there’s no space for the haematopoietic cells!

Question 533

marfans syndrome inheritance

Answer 533

AD

Question 534

risks with marfans syndrome

Answer 534

cardiovascular degeneration of media of vessel walls resulting in dilates aortic root. need echo monitoring

Question 535

what do you physically give in active vs passive vaccination? which is longer lasting

Answer 535

``` Passive = antibody. immediate effect but short lasting Active = antigen (because then the body has to actively make an antibody) ```

Question 536

CI of giving vaccine

Answer 536

Don't give to someone in acute illness | Don't give live vaccine to immunocompromised

Question 537

how are most primary immunodeficiencys inherites

Answer 537

X linked

Question 538

SPUR for infections in immunodeficiency

Answer 538

severe prolonged unusual recurrent

Question 539

what broad spectrum antibiotic should you use in children with sepsis of unknown origin

Answer 539

``` <1m = cefotaxime + amox >1m = ceftriaxone ```

Question 540

presentation of mumps

Answer 540

parotitis (initially unilateral and then bilateral) with fever and malaise --> otalgia + pain on eating

Question 541

Mx of mumps

Answer 541

supportive

Question 542

complicaitons of mumps

Answer 542

hearing loss meningitis/encephalitis orchitis (unilateral and in post-pubertal children)

Question 543

presentation of glandular fever

Answer 543

``` lymphadenpathy tonsilitis + soft palate petechiae fever/malaise splenomegaly 50% rash/jaundice sometimes ```

Question 544

specific lab investigations for glandular fever (3)

Answer 544

1. abnormal lymphocytes 2. +ve mono spot test 3. seroconversion with 3 antibodies (viral capsid, IgG and IgM EB nuclear antigen)

Question 545

what antibiotic do you avoid in EBV infection and why

Answer 545

amoxicillin --> rash

Question 546

how do enteroviruses present

Answer 546

90% nonspecific febril eillness D&V Rash (can be non-blanching petechial)

Question 547

is hand foot and mouth disease painful

Answer 547

yes, that's why children present unable to eat or drink. it is a painful vesicular rash

Question 548

what is herpangina

Answer 548

vesicular and ulcerated lesion son soft palate and uvula caused by enterovirus.

Question 549

what is hand foot and mouth disease caused by

Answer 549

Picornaviridae family, most commonly: coxsackie A16 enterovirus 71

Question 550

Most common cause of meningitis overall

Answer 550

enterovirus

Question 551

what is bornhol disease

Answer 551

pleural disease: an acute illness caused by enterovirus causing fever, pleuritic chest pain and muscle tenderness. self limiting

Question 552

MSSA Abx

Answer 552

fluclox | cipro for broader cover

Question 553

MRSA Abx

Answer 553

Vancomycin | Clindamycin

Question 554

Most common type of group A strep

Answer 554

Strep pyogenes | +ve coccus

Question 555

How does toxic shock syndrome occur?

Answer 555

S. aureus or Group A strep produces an exotoxin that acts as a superantigen. can arise uncommonly from any site of infection

Question 556

presentation of toxic shock syndrome

Answer 556

fever >39 degrees hypotension (hence the shock) diffuse macular rash multi-organ dysfunction: - mucositis, D/v, renal liver impairment, cltoiting abnormalities, low GCS - 1-2 weeks later you get desqamation of palms, soles, fingers

Question 557

management of toxic shock syndrome

Answer 557

ABCDE in ICU surgical debridment of obvious areas of infection Ceftriaxone and clindamycin IVIG

Question 558

types of nec fash

Answer 558

type 1 = mixed aerobe and anaerobe | type 2 = strep pyogenes

Question 559

risk groups for types of nec fash

Answer 559

type 1 = diabetics post op | type 2 = IVDU

Question 560

presentation of nec fash

Answer 560

intensely tender red area that is acute and rapidly progressing. in 3-5 days there will be skin breakdown and bullae, following by gangrene and anaesthesia in the area

Question 561

Mx of nec fash

Answer 561

Benzyl pen + fluclocacillin + metronidazole (to cover anaerobes) extensive surgical debridement

Question 562

What do T lymphocytes do

Answer 562

recognize antigen using the t cell receptor in the context of the major histocompatibility complex. alongside B lymphocytes that produce antibodies, they make up the adaptive immune system

Question 563

types of T lymphocyte and what they do

Answer 563

CD4 helper - uses MHCII and when with antigen releases cytokines with two types of response: 1) Th1 = macrophage activation 2) humoral immunity CD8 cytotoxic - uses MHCI and kills virally infected cells using perforins and granzymes and interferons

Question 564

how does HIV infect the CD4 cell

Answer 564

interacts with CD4 TCR alongside CCR5 coreceptor and virus can get into the cell. reverse transciptase enzyme lets virus replicate

Question 565

4 stages of HIV infection

Answer 565

Stage 1 = acute seroconversion where viral load goes up and causes a non specific viral picture stage 2&3 = continuous turnover of viruses and cells. is asymptmatic and lasts up to 10 years but CD4 count slowly drops. some lymphadenopathy maybe. stage 4 = AIDS. weird infections kill you due to lack of CD4 T cells

Question 566

what is hairy leukoplakia and what does it mean

Answer 566

white plaque son side of tongue. it is an AIDS defining EBV infection of the tongue

Question 567

how do you diagnose children with HIV

Answer 567

``` >18m = serology to detect IgG against HIV <18m = serology can only confirm exposure due to maternal antibodies ```

Question 568

how can you confirm a child is not infected if mum had HIV

Answer 568

Once postnatal antiretroviral therapy is completed --> 2x -ve HIV PCR within first 3m of life

Question 569

What levels of CD4 indicate: - normal - asymptomatic HIV - AIDS - about to die

Answer 569

>500 200-500 <200 <50

Question 570

what is the transmission rate from mother to baby completely untreated when breastfeeding

Answer 570

25-40%

Question 571

here in the UK, what advice is given to prevent vertical transmission of HIV

Answer 571

ART during and after pregnancy post exposure prophylaxis after birth for baby avoid breast feeding pre-labour caesarean if viral load is high

Question 572

cause of lyme disease

Answer 572

spirochete: | Borrelia burgdorferi

Question 573

presentation of lyme disease

Answer 573

50% have tick bite in past couple weeks erythematous macule at site of tick bite that spreads with a bright leading edge = erythema migrans fever, malaise, myalgia, arthralgia, lymphadenopathy If disseminates: - CN palsy - meningitis - arthritis - myocarditis/heart block

Question 574

Mx for lyme disease

Answer 574

``` <12y = amox >12y = doxycycline ``` complicated = IV ceftriaxone

Question 575

what does cyclical fever every few days raise alarm bells of in someone who has recently been travelling

Answer 575

malaria

Question 576

typhoid/paratyphoid cause

Answer 576

caused by salmonella group (e.g. salmonella typhi)

Question 577

presentation of typhoid

Answer 577

* initially systemic (fever, malaise, arthralgia) * relative bradycardia * abdominal pain, distension * constipation: although Salmonella is a recognised cause of diarrhoea, constipation is more common in typhoid * rose spots: present on the trunk in 40% of patients, and are more common in paratyphoid

Question 578

Mx of typhoid

Answer 578

Co-trimoxazole, chloramphenicol, ampicillin

Question 579

ENT wise, what are gromits used for

Answer 579

to resolve conductive hearing loss Sx in otitis media with effusion (glue ear)

Question 580

describe limitation of newborn hearing screening

Answer 580

otoacoustic emission test cochlea audotry brainstem response tests brainstem neither tests auditory nerve

Question 581

most common age for acute otitis mediA

Answer 581

6-12 months

Question 582

what can you do if grommets fail in children

Answer 582

adenoidectomy (remove adenoids which opens Eustachian tube on the mouth end more to allow gunk out)

Question 583

when do you give antibiotics in otitis media, and what course do you prescribe

Answer 583

5 days amox if: * Symptoms lasting more than 4 days or not improving * Systemically unwell but not requiring admission * Immunocompromise or high risk of complications secondary to significant heart, lung, kidney, liver, or neuromuscular disease * Younger than 2 years with bilateral otitis media * Otitis media with perforation and/or discharge in the canal

Question 584

cause of childhood obstructive sleep apnoea

Answer 584

adenotonsillar hypertrophy

Question 585

incidence of trisomy 21

Answer 585

1 in 650

Question 586

most common cause of the reason trisomies occur

Answer 586

94% is... meiotic nondisjunction = fail to separate during anaphase of either meiosis 1 or 2. this leads to a gamete with 2x chromosome 21 for eg, which wen feritlised becomes trisomy 5% is translocation 1% is a mosaic of trisomy

Question 587

appearance of trisomy 21

Answer 587

``` round face with flat nasal bridge epicanthic folds small mouth and protruding tongue hypotonic flat occipur single palmar crease incurved 5th finger wide sandal gap ```

Question 588

% of downs with congebnital heart disease`?

Answer 588

40% AVSD

Question 589

appearance of Edwards syndrome

Answer 589

``` small mouth and chin prominent occiput clenched fist with overlapping fingers malformed ears rocker bottom feet and flexed big toe ```

Question 590

prognosis with Edwards/pataus syndrome

Answer 590

most die in infancy

Question 591

appearance of pataus syndrome

Answer 591

small or absent eyes cleft lip and palate polydactyl

Question 592

presentation of turners syndrome

Answer 592

``` lymphedema of hands and feet in neonate webbed neck spoon shaped nails small stature widely spaced nipples ovarian dysgenesis (infertility) coarctation of aorta hypothyroidism renal abnormalities pigmented moles ```

Question 593

what can you give around puberty for a girl with turners

Answer 593

oestrogen replacement therapy to help her go through puberty

Question 594

what is wrong genetically in fragile X syndrome

Answer 594

trinucleotide repeat in X chromosome

Question 595

appearance of fragile X

Answer 595

long face large everted ears learning difficulty macro-orchidism

Question 596

complications of fragile X

Answer 596

mitral valve prolapse | scoliosis

Question 597

noonan syndrome inheritance

Answer 597

AD

Question 598

presentation of noonan syndrome

Answer 598

``` very similar to turners: broad forehead drooping eyelids wide eye distance short webbed neck trident har line pectus excavatum short pulmonary stenosis ```

Question 599

Williams syndrome presentation

Answer 599

``` Short stature Learning difficulties Friendly, extrovert personality Transient neonatal hypercalcaemia Supravalvular aortic stenosis ```

Question 600

presentation of Prader-willi

Answer 600

hypotonia hypogonadism obesity

Question 601

genetic investgations options from most crude to most precise

Answer 601

karyotyping DNA PCR (analysis of small samples) CGH array (compares sample to known array to see if chinks are added or missing) Mutation analysis (point mutations) Next gen sequencing (whole exome sequencing) whole genome sequencing is reserved for research tool as it looks at introns and exons (100,000 genome project).

Question 602

% child mortality

Answer 602

4.5% (6 million per year)

Question 603

top 2 causes of child mortality <5

Answer 603

50% is infectious diseases, of which pneumonia is most common 50% is neonatal death (if you include 1-15yo, then malignancy accounts for 24%)

Question 604

at what income level is poverty defined as

Answer 604

60% less than median income

Question 605

when does a child attain 6/6 vision

Answer 605

5 yo

Question 606

what is corneal light reflex used for

Answer 606

Detection of a squint may be made by the corneal light reflection test - holding a light source 30cm from the child's face to see if the light reflects symmetrically on the pupils

Question 607

amblyopia?

Answer 607

lazy eye | brain and eye not moving in sync

Question 608

myopia hypermetropa long sighted short sighted

Answer 608

Short = myopia (its a shorter word)

Question 609

what is astigmatism

Answer 609

refractive error of the lens meaning there is a constant, slight blur at any distance

Question 610

what is the UK healthy child programme

Answer 610

from 0-19 years, that is the overarching name for the plan for: - screening - immunization - developmental reviews - heatlh promotion/education

Question 611

4 basic human rights of child

Answer 611

from 1989 act: 1. survival 2. development 3. protection 4. participation

Question 612

4 types of abuse

Answer 612

physical (80% mother!) sexual emotional neglect

Question 613

most common symptom in CFS other Sx

Answer 613

post-exertional malaise and fatigue ``` cognitive dysfunction sleep disturbance muscle pain joint pain headaches sore throat ```

Question 614

is there a trigger in CFS

Answer 614

yes, usually some sort of viral infection, which because of a genetic predisposition causes CFS to start

Question 615

TOTT investigation screen

Answer 615

``` FBC ESR/CRP U/E LFTs CK TTG TFTs ferritin urine dip BP ```

Question 616

Management of CFS

Answer 616

making diagnosis helps advice is two fold: SLEEP - anchor wake up time for cortisol hit ACTIVITY - avoid boom bust. must pace yourself. the coloured diary charts help. CBT/graded exercise therapy is the next step (with a specialit)

Question 617

is the pain real in chronic pain syndrome

Answer 617

YES, remember pain is an emotion not a physical thing and chronic pain syndrome is a wiring problem leading to allogynia/hyperalgesia

Question 618

definition of ADHD

Answer 618

1. inattention 2. hyperactivity 3. impulsivity must be >6m and in at least 2 settings

Question 619

screening questionnaire for ADHD

Answer 619

connors

Question 620

ways to gather information when making ADHD diagnosis

Answer 620

parental interview conors questionnaire school observation school reports

Question 621

Mx of ADHD

Answer 621

non-medical parent education school education/special assistance/strategies CBT methyphenidate CNS stimulant (SE appetite down). atomoxetine can also be used.

Question 622

triad in autism spectrum disorder

Answer 622

impaired social interaction SAL delayed development routines/ritualistic behaviour

Question 623

diagnostic tools for autism

Answer 623

ADOS (done by SALT) = Autism Diagnostic Observation Schedule | 3DI (parent questionnaire)

Question 624

is autism associated with epilepsy/seizures?

Answer 624

yes

Question 625

what is a normal IQ

Answer 625

>80 | <35 is severe learning difficulties

Question 626

are eating disorders associated with social class

Answer 626

no, but it is a problem of western society

Question 627

SCOFF questions for anorexia

Answer 627

sick - do you make yourself sick because youre too full control - do you worry you've lost control of eating one stone - have you ever lost one stone in a short space of time fat - you you believe you are fat when others say you are thin food - does food dominate your life

Question 628

what is lanugo hair

Answer 628

a condition characterized by excessive hair growth in certain areas of the body. Lanugo is actually a sign that the body is trying to protect itself during the starvation process by trapping in heat that muscles and fat can no longer provide.

Question 629

can you have bulimia without being underweight?

Answer 629

yes. in fact, you must have a BMI >17.5 to have bulimia nervosa. if you have <17.5, then you have BN and AN

Question 630

red flags for anorexia nervisa

Answer 630

``` BMI<13 weight loss >1kg/week T <34.5 hypotensive <80/50 HR <40 sats <92% or limbs blue purpura electrolyte abnormality ```

Question 631

what is refeeding syndrome

Answer 631

Refeeding syndrome is a syndrome consisting of metabolic disturbances that occur as a result of reinstitution of nutrition to patients who are starved, severely malnourished or metabolically stressed due to severe illness. When too much food and/or liquid nutrition supplement is consumed during the initial four to seven days of refeeding, this triggers synthesis of glycogen, fat and protein in cells, to the detriment of serum (blood) concentrations of potassium, magnesium, and phosphorus.[1][2] Cardiac, pulmonary and neurological symptoms can be signs of refeeding syndrome. The low serum minerals, if severe enough, can be fatal. 'caused by low phosphate?'`

Question 632

mortality of anorexia nervisa

Answer 632

5-10%

Question 633

Mx of refeeding syndrome

Answer 633

vitamin B | stop refeeding and reintroduce slowly

Question 634

screen for DDH in breech baby with a normal newborn exam?

Answer 634

Yes

Question 635

cradle cap (Seb dermatitis) treatment

Answer 635

``` mild = baby oil/shampoo severe = topical hydrocortisone ```

Question 636

diagnosis of pertussis

Answer 636

per nasal swab

Question 637

conservative treatment for chickenpox

Answer 637

topical calamine lotion

Question 638

what syndrome is neonatal hypotonia most associated with

Answer 638

prader-willi

Question 639

average age for GOOD pincer grip cut off age for early pincer grip

Answer 639

12 months 12 months

Question 640

elfin facies = ?condition

Answer 640

williams syndrome

Question 641

flattened philtrum = ?condition

Answer 641

foetal alcohol syndrome

Question 642

asthma, SABA not working. what's the next step: <5 >5

Answer 642

<5 = moderate dose ICS >5 = low dose ICS

Question 643

until what age is a phimosis okay?

Answer 643

2y

Question 644

how much of a fever do children with bronchiolitis have?

Answer 644

low grade (<39). If it's >39, suspect pneumonia (esp. if focal as opposed to generalised crackles)

Question 645

Main sequelae of respiratory distress syndrome in premature baby

Answer 645

retinopathy of prematurity | renal failure

Question 646

paediatric basic life support

Answer 646

``` hello hello? can i get some help open airway check for breathing 5 rescue breaths (omitted in adults) check for BRACHIAL/FEMORAL (not carotid) pulse 15:2 CPR ```

Question 647

when do majority of children achieve day and night urinary continence

Answer 647

3-4

Question 648

initial management of Hirschsprung's disease

Answer 648

bowel irrigation (whilst biopsy comes back)

Question 649

Roseola infant cause, presentation, complications and ?exclusion

Answer 649

``` HHV6 high fever for few days THEN rash nagayama spots on uvula/palate aseptic meningitis/hepatitis exclusion not required ```

Question 650

management of exompahlos vs gastroschisis

Answer 650

exompahlos = bowel out of abdomen with peritoneal/amniotic covering --> staged closure with completion at 6-12m gastroschisis = defect in abdominal wall just lateral to umbilical cord. gut out of body with no covering --> immediate correction

Question 651

do you grow out of a cows milk allergy

Answer 651

yes, by age 5, but usually by age 1-2

Question 652

management of cows milk protein allergy

Answer 652

formula fed --> use extensive hydrolysed formula (eHF), or even amino acid formula if severe and eHF doesn't work breast fed --> carry on breast feeding but mum to stop milk products. then use eHF from 6-12 months after breast stops

Question 653

risk factors for surfactant lung disease

Answer 653

``` (prematurity) male sex diabetic mothers caesarean second born of premature twins ```

Question 654

turners syndrome heart auscultation sign and why

Answer 654

ejection systolic due to bicuspid aortic valve

Question 655

dietary advice for CF

Answer 655

high calorie high fat pancreatic enzymes every meal

Question 656

M:F in DDH

Answer 656

female 6x other RFs are breech, FH, firstborn, oligohydramnios, macrosomia

Question 657

painless massive GI bleed in 1-2 year old? | rule of 2s?

Answer 657

Meckel's diverticulum 2% of population 2 feet from ileocaecal valve 2 inches long

Question 658

viral wheeze age group and management

Answer 658

pre-school children 1st line = SABA + spacer 2nd line = montelukast and inhaled ICS

Question 659

contraindications for MMR (5)

Answer 659

severe immunosuppresion allergy to neomycin other live vaccine <4wks ago IVIG therapy in past 3m

Question 660

what is choanal atresia? presentation? management?

Answer 660

- posterior nasal airway occluded by soft tissue or bone - presents with episodes of cyanosis worst when feeding that disappears when crying. unilateral subtype may go unnoticed. (Remember all newborn babies are obligate nasal breathers so having a blocked nose is bad) - penetration procedures.

Question 661

ebsteins anomaly? presentations? cause?

Answer 661

tricuspid valve is lower and floppy so RA is massive and there is tricuspid regurg (pan-syslotic) and tricuspid stenosis (mid-diastolic) caused by lithium in pregnancy

Question 662

haemophilia in a girl???

Answer 662

think about turners syndrome (only one X so susceptible to X-linked recessive)

Question 663

competent use of knife and fork age?

Answer 663

5 years | 4 prongs of a fork and one knife

Question 664

distribution of atopic eczema in an infant?

Answer 664

face and trunk

Question 665

maternal cause of orofacial clefts?

Answer 665

maternal anti-epileptic use

Question 666

can you go to school with head lice

Answer 666

yes

Question 667

full name for mongolian blue spot

Answer 667

congenital dermal melanocytosis

Question 668

drawing circle

Answer 668

3 years | because you need 3 things: pen, paper and hand

Question 669

cystic hygroma vs branchial cyst

Answer 669

cystic hygroma = posterior triangle, soft, transilluminate branchial cyst = lateral in anterior triangle. anechoic on USS.

Question 670

multiple miliary opacities in both lungs (not TB)?

Answer 670

pneumonia from chickenpox. one of the 3 main complications of varicella: - pneumonia - encephalitis - disseminated haemorahgic chickenpox

Question 671

what happens if you miss DDH

Answer 671

arthritis in your 30s

Question 672

if a mum misses MMR vaccine but now wants it, what can you do

Answer 672

give one now and then in 3m | if >10 or in an outbreak&<10, 1m is ok

Question 673

fragile X heart problem

Answer 673

mitral valve prolapse

Question 674

when do you worry about undescended testes

Answer 674

review at GP @3m and make referral to surgeon surgery by 6m