Paeds Flashcards

Question

Simple febrile convulsion vs complex

Answer 1

Complex - Focal, >15 minutes, Repeat seizure within 24 hours

Answer 2

A chronic disorder of movement and posture due to non progressive brain abnormalities occurring before the brain is fully developed

Answer 3

Delayed milestones, failure to thrive, epilepsy, urinary incontinence, constipation, drooling, sleep disturbance, contractures

Answer 4

Right lower quadrant opacity, dilated gas-filled proximal bowel with absence of gas distally, multiple fluid levels, perforation

Answer 5

- Peritonitis - Failed enema - Perforation - Prolonged history

Answer 6

ASD - ejection systolic VSD - pansystolic at left sternal border

Answer 7

XO karotype

Answer 8

Bicuspid aortic valve

Answer 9

Dyskinetic CP

Answer 10

- 5 rescue breaths - 15:2 (3:1 for newborn) chest compressions on lower half of sternum with depth of 1/3 of chest - Use 2 thumb encircling technique for chest compression for infants

Answer 11

Basal ganglia and substantia nigra

Answer 12

PA - bitemporal superior quadrantinopia CP - bitemporal inferior quadrantinopia

Answer 13

Aplastic - reduced reticulocytes Sequestration - increased reticulocytes

Answer 14

Dexamethasone

Answer 15

FAT RN Fever Anaemia Thrombocytopenia Renal dysfunction Neuro abnormalities

Answer 16

- Short stature - DM - Delayed puberty - Nasal polyps - Rectal prolapse - Male infertility

Answer 17

Testicular torsion

Answer 18

Haemoglobin electrophoresis

Answer 19

Myoclonic -> treat with sodium valproate/leviteracetam

Answer 20

Refer to Paeds

Answer 21

- Blood pressure and urinalysis

Answer 22

Hydroxyurea -> increases levels of HbF

Answer 23

- Loud systolic murmur in pulmnary area - Ejection systolic murmur

Answer 24

Right axis deviation

Answer 25

- Congenital cataracts - Congenital HD - Undescended testes - DHH - Newborn hearing

Answer 26

Reduced alpha and beta angles Reduced bony coverage of femoral epiphysis

Answer 27

- Premature joint degeneration - Arthritis - Chronic lower back pain - Delay in walking - Avascular necrosis

Answer 28

- Diazepam - Baclofen - Botox

Answer 29

Vomiting - forceful and active, Reflux - sphincter incompetence

Answer 30

Coxsackie - HFM Slapped Cheek - Parvovirus Roseola - HH6

Answer 31

Reversal of left-right shunt to a right-left shunt due to pulmonary HTN

Answer 32

Frontal lobe

Answer 33

Henoch-Schonlein Purpura

Answer 34

Waterhouse-Friderichsen syndrome

Answer 35

Roseola - HH6 Slapped Cheek - Parvovirus Hand, foot mouth - Coxsackie virus Chickenpox - Varicella

Answer 36

Think Wilms tumour

Answer 37

- Soft - Systolic - Sensitive - Short - Symptomless

Answer 38

- Intubation - IV Cefuroxime

Answer 39

Keep open - Prostaglandins Promote closure - Ibuprofen/Indomethacin

Answer 40

the patient cannot be persuaded to inform their parents or carers the patient understands the advice or treatment the patient's physical and/or mental health will suffer if they do not receive the treatment/advice the treatment/advice is in the patient's best interest the patient is likely to continue having sex with or without treatment

Answer 41

Bronchiectasis

Answer 42

Patau - 13 Edward - 18 Down - 21

Answer 43

Think congenital syphilis

Answer 44

Widening of the joints

Answer 45

Newborn - otoacoustic emission test If abnormal - auditory brainstem response test 3 years - Pure tone audiometry

Answer 46

Nasal flaring

Answer 47

Koplik spots -> measles

Answer 48

- Short stature - Lymphoedema - Spoon shaped nails - Webbed neck - CHD - Delayed puberty - Wide spaced nipples

Answer 49

Telescoping of a segment of proximal bowel into a distal one

Answer 50

- Rectal air insufflation/air enema if stable - If unstable: laparoscopic reduction

Answer 51

Where the femoral head and acetabulum do not articulate correctly

Answer 52

Barlow - attempt to dislocate the femoral head Ortolani - attempt to relocate the dislocated femoral head

Answer 53

If <4.5 months: US If >4.5 months: X-ray

Answer 54

Until 2 years

Answer 55

- Microcephaly - Clef lip and palate - Polydactyly

Answer 56

- Micrognathia - Low set ears - Rocker-bottom feet - Overlapping fingers

Answer 57

- Prednisolone - Immunosuppressives such as ciclosporin - Fluid restrict and low salt

Answer 58

Slapped cheek syndrome

Answer 59

- Impulsivity - Inattention - Hyperactivity

Answer 60

ECG -> can be cardiotoxic

Answer 61

- Microcephaly - Short palpebral fissures - Absent philtrum - Reduced IQ - Cardiac abnormalities

Answer 62

Reversal of the shunt across the VSD leading to a right to left shunt and exacerbated cyanosis

Answer 63

seven or more episodes in a single year, five or more episodes/year in two years, or three or more episodes/year in three years

Answer 64

Think mesenteric adenitis

Answer 65

Family therapy

Answer 66

Upto 6 months

Answer 67

Think Congenital adrenal hyperplasia

Answer 68

Think congenital diaphragmatic hernia -> tinkling bowels sounds

Answer 69

Opioid withdrawal

Answer 70

Osteosarcoma

Answer 71

Bordetella pertussis -> whooping cough

Answer 72

X-linked recessive

Answer 73

Think Turners

Answer 74

Think congenital rubella

Answer 75

ASD - Ejection systolic VSD - pan systolic at lower left sternal edge Pulmonary stenosis - Ejection systolic Tricuspid regurg - pan systolic at lower left sternal edge

Answer 76

VP shunt insertion

Answer 77

Toxic build up of unconjugated bilirubin

Answer 78

MMR Influenza BCG Oral polio Oral rota Typhoid Yellow fever

Answer 79

Haemophilus influenzae type B

Answer 80

airway obstruction may develop

Answer 81

previously children but now more common in adults due to immunisation programme (Hib vaccine)

Answer 82

- tripod position= easier to breathe leaning forward and extending neck in seated position - drooling - rapid onset - high temp but generally well - stridor

Answer 83

direct visualisation ONLY by senior/airway trained staff may do x-ray if concern about foreign body

Answer 84

lateral view= thumb sign (swelling of epiglottis)

Answer 85

acute epiglottitis

Answer 86

DO NOT EXAMINE THE THROAT due to risk of acute airway obstruction immediate senior invl eg. anaesthetics or ENT= direct visualisation & endotracheal intubation may be needed - O2 and IV Abx

Answer 87

? acute interstitial nephritis= drug induced AKI eg. penicillin, NSAIDs, rifampicin; SLE; infection marked interstitial oedema and interstitial infiltrate in the connective tissue between renal tubules

Answer 88

young females. Symptoms include fever, weight loss and painful, red eyes. Urinalysis is positive for leukocytes and protein.

Answer 89

peritonsillar abscess (quinsy) otitis media rare= RF, glomerulonephritis

Answer 90

deviation of uvula to unaffected side, trismus (difficulty open mouth); severe throat pain (lateralises to one side); reduced neck mobility

Answer 91

urgent ENT review needle aspiration or incision & drainage + IV Abx consider tonsillectomy to prevent recurrence

Answer 92

100 - 150 micrograms (0.1 - 0.15 ml 1 in 1,000)

Answer 93

150 micrograms (0.15 ml 1 in 1,000)

Answer 94

300 micrograms (0.3ml 1 in 1,000)

Answer 95

500 micrograms (0.5ml 1 in 1,000)

Answer 96

SpO2 < 92% (unlike in adults) PEF 33-50% best or predicted Too breathless to talk or feed Heart rate >125 (>5 years) >140 (1-5 years) Respiratory rate >30 breaths/min (>5 years) >40 (1-5 years) Use of accessory neck muscles

Answer 97

SpO2 <92% PEF <33% best or predicted Silent chest Poor respiratory effort Agitation Altered consciousness Cyanosis

Answer 98

SpO2 > 92% PEF > 50% best or predicted No clinical features of severe asthma

Answer 99

give a beta-2 agonist via a spacer (for a child < 3 years use a close-fitting mask) give 1 puff every 30-60 seconds up to a maximum of 10 puffs if symptoms are not controlled repeat beta-2 agonist and refer to hospital steroid therapy for all children for 3-5d

Answer 100

2 - 5 years= 20 mg od OR 1-2 mg/kg od (max 40mg) > 5 years= 30 - 40 mg od OR 1-2 mg/kg od (max 40mg)

Answer 101

1st line= FeNO, diagnosis if ≥ 35 ppb - if normal or not available= BDR with spirometry: diagnose if FEV1 increase ≥ 12% or ≥ 10% of predicted normal - if not available= PEF bd for 2w: diagnose if PEF variability ≥ 20% - if still not confirmed= skin prick to house dust mite OR total IgE level and blood eosinophil count: diagnose if sensitisation or raised total IgE level and the eosinophil count is > 0.5 x 109/L - still doubt then refer to paeds and ?bronchial challenge test

Answer 102

difficulty performing tests so: - treat with ICS - if still symptoms at age 5 then attempt Ix - refer to specialist resp paeds any child with admission to hospital or 2+ admissions to A&E with wheeze in 12m period

Answer 103

1) SABS + low dose ICS bd then consider MART pathway (new) or conventional pathway 2) SABA + low dose MART 3) SABA + moderate MART or 2) SABA + low dose ICS + LTRA (trial 8-12w, stop if ineffective) 3) SABA + low dose ICS + LABA (with or without LTRA depending on trial) 4) SABA + moderate dose ICS + LABA (+/- LTRA) 4/5) specialist referral

Answer 104

1) 8 to 12 week trial of twice-daily paediatric low-dose ICS as maintenance therapy + SABA as required consider stopping ICS and SABA treatment after 8 to 12 weeks if symptoms are resolved. Review the symptoms after a further 3 months 2) if symptoms restart then SABA + low dose ICS 3) SABA + moderate dose ICS 4) SABA + moderate dose ICS + LTRA trial 5) refer

Answer 105

25-50% (only the ICS nothing else)

Answer 106

involves either obliteration or discontinuity with the extrahepatic biliary system resulting in an obstruction

Answer 107

biliary atresia

Answer 108

cholestasis in the 1st few weeks of life= jaundice beyond physiological 2w; dark urine and pale stools; appetite and growth disturbances (may be normal)

Answer 109

unclear but infectious agents, congenital malformations and retained toxins within bile all contribute

Answer 110

unique to neonatal children: - perinatal form= within 1st 2w of life - postnatal form= within 2-8w of life

Answer 111

proximal ducts are patent however the common duct is obliterated

Answer 112

atresia of the cystic duct and cystic structures are found in the porta hepatisw

Answer 113

atresia of the left and right ducts to the level of the porta hepatis, this occurs in >90% of cases of biliary atresia

Answer 114

- jaundice - hepatomegaly with splenomegaly - abnormal growth - cardiac murmurs if associated with cardiac abnormalities

Answer 115

- Serum bilirubin= total bilirubin may be normal but conjugated bilirubin high - LFTs= serum bile acids and aminotransferases raised (but can't differentiate between other causes of neonatal cholestasis) - USS of biliary tree and liver= may be distension and tract abnormalities - Percutaneous liver biopsy and intraoperative cholangioscopy - Rule out= serum alpha 1-antitripsin def and sweat chloride test (CF invl biliary tract and a 1-a def may be a cause of neonatal cholestasis

Answer 116

Surgery is definitive= dissection of abnormalities into distinct ducts and anastomosis creation - then after surgery= Abx and bile acid enhancers

Answer 117

- Unsuccessful anastomosis formation - Progressive liver disease - Cirrhosis with eventual hepatocellular carcinoma

Answer 118

- good if surgery successful - surgery fails= liver transplant in 1st 2yrs of life

Answer 119

permanent dilation of airways (bronchi) secondary to chronic infection or inflam causing irreversible damage to elastic and muscular components of bronchial wall

Answer 120

- post infective= TB, measles, pertussis, pneumonia - CF - bronchial obstruction= lung ca, foreign body - immune def= selective IgA, hypogammaglobulinaemia - ABPA - ciliary dyskinetic syndrome= Kartagener's syndrome, Young's syndrome - yellow nail syndrome

Answer 121

- persistent cough with large volumes of sputum - dyspnoea - haemoptysis

Answer 122

- coarse crackles and wheeze - clubbing

Answer 123

CXR= tramlines CT= widespread tram-track and signet ring signs

Answer 124

- physical training eg. inspiratory muscle training good for non-CF - postural drainage - Abx for exacerbations + long term rotating Abx in severe - bronchodilators in severe - immunisations - surgery in selected (eg. localised disease)

Answer 125

- H.influenzae (most common) - Pseudomonas aeruginosa - Klebsiella spp. - Strep pneumoniae

Answer 126

use previous microbio cultures if available, if not then: - local guidelines or amox 500mg 3xd 7-14d ( if 1-4yrs then 250mg, 1-11m then 125mg) or clarithromycin 250- 500mg bd 7-14d (if 1m-11yrs= if 30-40kg then 250mg; 20-29kg 187.5mg; 12-19kg 125mg; 8-11kg 62.5mg; <8kg then 7.5mg/kg)

Answer 127

cough, symptoms & signs of infection and clear chest on exam

Answer 128

pneumonia, COVID, viral-induced wheeze, or an infective exacerbation of asthma

Answer 129

children 6m-5yrs who present with wheeze with resp tract infection

Answer 130

typically <12m high RR, wheeze may be present, hyperinflation, fine crackles throughout lung fields

Answer 131

respiratory syncytial virus (RSV)

Answer 132

feverish child, RR 60+, signs of increased work of breathing and/or crackles in chest and/or cyanosis

Answer 133

- ?agitation and consciousness= agitation and behavioural change may be sign of hypoxia - signs of exhaustion, cyanosis, invl of accessory muscles at rest - examine chest, RR, pulse and BP - O2 sats on air - peak flow if possible in viral-induced wheeze or infective asthma exacerbation - assess hydration status

Answer 134

- RR 60-70+ - apnoea, grunting, moderate or severe chest indrawing - cyanosis - no response to social cues, unable to be roused or cannot stay awake - look ill - clinical dehydration - <3m + temp at least 38°C - >3m with temp >39°C - PEF rate <50% best or predicted - O2 sats 90% on air or less; or 92% or less in <6w old or underlying health issue

Answer 135

- 6-12m + RR 50-60 or >12m with RR 40-60, nasal flaring, crackles - O2 sats 92% or less on air - pallor reported by parent/carer - no response to normal social cues, awakes only with prolonged stimulation, decreased activity - Poor feeding in infants (less than 50% of normal fluid intake in preceding 24 hours), dry mucous membranes, reduced urine output. - CRT equal to or greater than 3 seconds.

Answer 136

may be Tx at home but how to seek advice if any deterioration

Answer 137

bronchiolitis

Answer 138

<2yrs, peak incidence 3-6m

Answer 139

- coryzal prodome 1-3d followed by persistent cough - tachypnoea, chest recessions - wheeze/crackles - fever, usually less than 39 - poor feeding (typically after 3-5d of illness) - apnoea without other clinical signs in young infants eg. <6w

Answer 140

think bronchiolitis

Answer 141

agitation and behavioural changes

Answer 142

usually self-limiting, symptoms tend to peak between 3-5d of onset

Answer 143

- immediate admission if indicated - If O2 sats <92% give supp O2 whilst awaiting hospital admission - usually self-limiting= paracetamol if child is distressed; regular fluids; don't try to reduce fever by undressing - check on child through night

Answer 144

- apnoea - looks very unwell - severe resp distress= grunting, marked chest recession, RR >70 - central cyanosis

Answer 145

listlessness or decreased respiratory effort, recurrent apnoea, and/or failure to maintain adequate oxygen saturation despite oxygen supplementation.

Answer 146

- RR >60 - difficulty breastfeeding/ oral intake 50-75% of usual - clinical dehydration - <92% sats on air

Answer 147

Chronic lung disease (including bronchopulmonary dysplasia). Haemodynamically significant congenital heart disease. Neuromuscular disorders. Immunodeficiency. Age under three months. The infant having been born prematurely, particularly before 32 gestational weeks. Factors that might affect a carer's ability to look after a child Longer distance to healthcare in case of deterioration.

Answer 148

educed skin turgor and/or a capillary refill time of more than three seconds, and/or dry mucous membranes, and/or reduced urine output

Answer 149

typically... - <5yrs - RR normal or increased - hyperinflation may be present - wheeze - not usually any crackles present

Answer 150

life-threatening features. severe attack persisting after initial bronchodilator treatment. moderate attack with worsening symptoms despite initial bronchodilator treatment and/or who have had a previous near-fatal asthma attack. infant/child having been born prematurely, any significant medical history, such as congenital heart disease, chronic lung disease of prematurity, cystic fibrosis, bronchiectasis, and immune deficiency, and the ability of the child's carers to cope with the ill child (in particular, assess the carer's experience, their level of anxiety, and the time they have available to care for the child).

Answer 151

- supp O2 if life-threatening or sats <94% - SABA= nebulised salbutamol (5 mg >5 yrs, and 2.5 mg to children 2–5 yrs). Should be O2 driven (flow rate 6L/min) - if mild/moderate= pressurised metered dose inhaler with large volume spacer; one puff with 5 tidal breaths every 30-60secs up to 10 puffs; can repeat every 10-20mins if responsive, if not successful- nebuliser + admission - monitor peak flow for response to Tx - prednisolone if diagnosed with asthma - Abx if bacterial infection= amoxicillin or doxy (if >12yrs)

Answer 152

48hrs later= peak flow, may need maintenance Tx if mild intermittent wheeze and other resp symptoms only with viral URTI

Answer 153

acute bronchiolar inflam

Answer 154

maternal IgG

Answer 155

- RSV (most common) - adenovirus - mycoplasma - may be secondary bacterial infection - more serious if bronchopulmonary dysplasia (eg. premature); congenital heart disease or CF

Answer 156

- coryzal symptoms (including mild fever) precede: - dry cough - increasing breathlessness - wheezing, fine inspiratory crackles (not always present) - feeding difficulties associated with increasing dyspnoea are often the reason for hospital admission

Answer 157

nasal swab= immunofluorescence of nasopharyngeal secretions may show RSV

Answer 158

humidified oxygen is given via a head box and is typically recommended if the oxygen saturations are persistently < 92% NG feeding may be needed if children cannot take enough fluid/feed by mouth suction is sometimes used for excessive upper airway secretions

Answer 159

child <1yr

Answer 160

1yr-puberty

Answer 161

- look, feel, listen for breathing - 5 rescue breaths - circulation= brachial or femoral pulse in infants, children use femoral - chest compressions 15:2 (100-120/min) for both infants & children

Answer 162

15:2 100-120/min depth: depress the lower half of the sternum by at least one-third of the anterior-posterior dimension of the chest (which is approximately 4 cm for an infant and 5 cm for a child) in children: compress the lower half of the sternum in infants: use a two-thumb encircling technique for chest compression

Answer 163

minimal change disease (75%)

Answer 164

most idiopathic - drugs= NSAIDs, rifampicin - Hodgkin's lymphoma, thymoma - infectious mononucleosis

Answer 165

T-cell and cytokine-mediated damage to the glomerular basement membrane → polyanion loss the resultant reduction of electrostatic charge → increased glomerular permeability to serum albumin

Answer 166

nephrotic syndrome normotension - hypertension is rare highly selective proteinuria only intermediate-sized proteins such as albumin and transferrin leak through the glomerulus signs on renal biopsy

Answer 167

normal glomeruli on light microscopy electron microscopy shows fusion of podocytes and effacement of foot processes

Answer 168

oral corticosteroids (80% steroid responsive) if resistant= cyclophosphamide

Answer 169

1/3 have just one episode 1/3 have infrequent relapses 1/3 have frequent relapses which stop before adulthood

Answer 170

Minimal change disease causing nephrotic syndrome

Answer 171

Measles is a highly contagious, airborne infection caused by a morbillivirus of the paramyxovirus family. Infects via the resp tract.

Answer 172

lifelong immunity

Answer 173

- otitis media (most common) - pneumonia (most common cause of death) - encephalitis= 1-2w following onset of illness - subacute sclerosing panencephalitis= very rare, 5-10yrs following illness - febrile convulsions - keratoconjunctivitis, corneal ulceration - diarrhoea - increased incidence appendicitis - myocarditis

Answer 174

adults, infants, immunocompromised people, during pregnancy

Answer 175

usually self-limiting, symptoms resolve over about a week

Answer 176

- prodromal symptoms= fever (39C+), cough, coryza, conjunctivitis - maculopapular rash (with or without Koplik's spots)

Answer 177

Parvovirus B19, or fifth disease (also known as slapped cheek syndrome). Streptococcal infection (for example, scarlet fever). Herpes virus type 6 (roseola infantum). Rubella. Kawasaki disease. Early meningococcal disease.

Answer 178

- notify local Health Protection Team (HPT) to confirm clinical diagnosis - Rest, drink fluids, paracetamol/ibuprofen - susceptible pt who are unwell= specialist advcie or admission - advice from HPT for Mx if immuocomp, pregnant or infants who have been in contact with measles

Answer 179

exclusion for 4d after initial development of rash and avoid susceptible people

Answer 180

serious Cx eg. pneumonia, neuro problems (febrile convulsions, encephalitis)

Answer 181

occurs 10–12 days after contracting the infection and lasts for 2–4 days before the rash becomes apparent. increasing fever (typically 39C without antipyretics), malaise, cough, rhinorrhoea, and conjunctivitis. This latter symptom may help differentiate measles from other flu-like illnesses.

Answer 182

increases during the prodromal phase to around 39ºC at about the time the rash appears, and then gradually decreases

Answer 183

may appear on the buccal mucosa at the end of the prodromal phase, a day or so before, or around the same time as the rash, and disappear over the next 2-3 days. 2–3 mm red spots with white or blue-white centres. These are pathognomonic for measles but can easily be confused with other mouth lesions.

Answer 184

erythematous and maculopapular and may become confluent as it progresses. It appears on the face and behind the ears first (when other symptoms tend to be at their most severe), before descending down the body to the trunk and limbs, and forming on the hands and feet last, over the course of about 3–4 days. The rash fades after it has been present on an area for about 5 days, with the total duration of rash being up to 1 week, after which time the person should feel better.

Answer 185

immunocompromised

Answer 186

mild and have a shorter duration, and there may not be the typical rash. rare

Answer 187

- notify public health if suspect clinically - they will send pt oral fluid testing kit for IgM/IgG and/or viral RNA testing to confirm diagnosis

Answer 188

contact public health if= immunocomp, <1yr, preg, MMR vaccine contraindicated: - immunoglobin in infants <6m within 72hrs; MMR in others within 3d (repeat after at least 1m; if <15m when had 2 doses then give 3rd dose at 18m; if <12m with 2 doses give a further 2 doses

Answer 189

RNA paramyxovirus one of most infectious viruses

Answer 190

aerosol transmission

Answer 191

from prodrome until 4d after rash starts

Answer 192

- prodrome= fever, irritable, conjunctivitis - Koplik spots before the rash= white spots on buccal mucosa - rash= behind ears then to whole body; discrete maculopapular becoming blotchy and confluent; desquamation typically spares the palms and soles may occur after a week - diarrhoea in 10%

Answer 193

desquamation that typically spares the palms and soles may occur

Answer 194

starts behind ears then to the whole body discrete maculopapular rash becoming blotchy & confluent

Answer 195

IgM antibodies can be detected within a few days of rash onset

Answer 196

mainly supportive admission may be considered in immunosuppressed or pregnant patients notifiable disease → inform public health

Answer 197

otitis media

Answer 198

subacute sclerosing panencephalitis

Answer 199

if a child not immunized against measles comes into contact with measles then MMR should be offered (vaccine-induced measles antibody develops more rapidly than that following natural infection) this should be given within 72 hours

Answer 200

decrease in the frequency of bowel movements, characterized by the passing of hardened stools that may be large and associated with straining and pain.

Answer 201

4 per day in 1st week of life to 2 per day at 1yrs Between 3 per day and 3 per week is usual by 4yrs

Answer 202

constipation that cannot be explained by any anatomical or physiological abnormality

Answer 203

pain, fever, inadequate fluid intake, reduced fibre intake, toilet training issues, effects of drugs, psychosocial issues, FHx

Answer 204

2 or more of: - <3 complete stools per week (unless exclusively breastfed-stools may be infrequent) - hard large stool - rabbit droppings stool - overflow soiling in children >1yrs (loose, smelly, passed without sensation/awareness)

Answer 205

- Hx of severe constipation - overflow soiling - faecal mass palpable on abdo exam

Answer 206

- no Ix for idiopathic/functional - if diagnosed then exclude underlying cause: if red flags= urgent referral without Tx in primary care; amber flags= referral and Tx in primary care

Answer 207

- maintenance laxtative Tx - impaction= disimpaction regimen - Behavioural interventions= scheduled toileting, bowel habit diary, rewards system - fluid and fibre intake

Answer 208

high fibre= fruit, veg, high fibre bread, baked beans, wholegrain cereals

Answer 209

water in drinks

Answer 210

physically active, exposed to hot environments, or obese.

Answer 211

700mL (from milk)

Answer 212

800 mL from milk and complementary foods and beverages, of which 600 mL is assumed to be water from drinks.

Answer 213

1300mL (900mL from drinks)

Answer 214

1700mL (1200mL from drinks)

Answer 215

boys= 2400mL (1800mL from drinks) girls= 2100mL (1600mL from drinks)

Answer 216

boys= 3300mL (2600mL from drinks) girls= 2300mL (1800mL from drinks)

Answer 217

- Movicol Paediatric Plain/Movicol - If fails after 2w then + a stimulant (if stool hard add lactulose/docusate)

Answer 218

may initially increase symptoms of soiling and abdo pain 1) oral laxative regimen then review within 1w= Movicol Paediatric Plan 2) Once disimpacted start maintenance laxative Tx (half of the disimpaction dose)

Answer 219

Maintenance Tx (even if only constipated for few days) aim for regular soft stools 1) Movicol Paediatric Plain 2) Persists then add stimulant; if hard consider + lactulose 3) Continue dose for several weeks after regular bowel movements established 4) Specialist advice if fails after 3m if >1yr or 4w if <1yr 5) follow up

Answer 220

- manual evacuation of bowel under anaesthesia (oral and rectal meds failed) - Psychological/behavioural interventions eg. toilet training - polyethylene glycol solutions for whole-gut lavage (often via nasogastric tube). - antegrade colonic enema (a surgical procedure).

Answer 221

- meconium passed within 48hrs of birth (full-term baby) - constipation after a few weeks after birth at least - precipitating factors= poor diet/fluids; anal fissure; infection; opitates/sedating antihistimines; timing of toilet training; psychosocial factors

Answer 222

There is normal appearance of the anus and surrounding area. Digital rectal examination is not routinely required to make the diagnosis. The abdomen is soft and flat, or distended only to a degree consistent with age or excess weight. They are generally well with normal development, and height and weight are within normal limits. Motor and neurodevelopment are within normal limits (including normal gait, tone, and power in lower limbs).

Answer 223

bright red erythema and local oedema

Answer 224

- present from birth or 1st few weeks of like ?Hirschsprungs - >48hrs to pass meconium ?Hirschspungs or CF - abdo distension with vomiting ?H - FHx H - Ribbon stool pattern <1yr ?anal stenosis - leg weakness or motor delay - fistuale, fissure, bruising, tight anus - lumbosacral or gluteal abnormalities: asymmetry ect

Answer 225

- faltering growth, developmental delay, systemic features - started on intro of cows milk - possible maltreatment

Answer 226

Polyethylene glycol 3350 plus electrolytes (macrogol)= can add fruit squash: 1 sachet in 62.5mL water Movicol if 12yrs+

Answer 227

polyethylene 3350 plus electrolytes (MPP): 1-11m= half sachet daily 1-6yrs= 1 sachet daily 7-11yrs= 2 sachets daily Movicol: 12-18hrs= 1 sachet daily adjust to produce regular soft stools eg. 1 sachet every 2-3d

Answer 228

Disimpaction MPP: 1-11m= half sachet daily 1-5yrs= 2 sachets day 1, then 4 for 2d, then 6 for 2 days then 8 daily until resolves 5-12yrs= 4 sachets day 1, then increase by steps of 2 daily to max of 12 daily until impaction resolves Movicol: 12-18yrs= 4 on day 1, then increase by steps of 2 until max 12 daily until resolves then for maintenance use half the disimpaction dose

Answer 229

2+ of: stool pattern= Fewer than 3 complete stools per week (type 3 or 4 on Bristol Stool Form Scale) (this does not apply to exclusively breastfed babies after 6 weeks of age); Hard large stool 'Rabbit droppings' (type 1) Symptoms associated with defecation= Distress on passing stool; Bleeding associated with hard stool; Straining History= Previous episode(s) of constipation; Previous or current anal fissure

Answer 230

2+ of: stool pattern= Fewer than 3 complete stools per week (type 3 or 4); Overflow soiling (commonly very loose, very smelly, stool passed without sensation); 'Rabbit droppings' (type 1); Large, infrequent stools that can block the toilet Symptoms associated with defecation= Poor appetite that improves with passage of large stool; Waxing and waning of abdominal pain with passage of stool; Evidence of retentive posturing: typical straight-legged, tiptoed, back arching posture; straining; anal pain History= Previous episode(s) of constipation; Previous or current anal fissure; Painful bowel movements and bleeding associated with hard stools

Answer 231

idiopathic dehydration low-fibre diet medications: e.g. Opiates anal fissure over-enthusiastic potty training hypothyroidism Hirschsprung's disease hypercalcaemia learning disabilities

Answer 232

? Hirschsprungs disease or CF

Answer 233

red flag in child constipation

Answer 234

only by specialist

Answer 235

polyethylene glycol 3350 + electrolytes (Movicol Paediatric Plain) using an escalating dose regimen as the first-line treatment + a stimulant laxative if Movicol Paediatric Plain does not lead to disimpaction after 2 weeks substitute a stimulant laxative singly or in combination with an osmotic laxative such as lactulose if Movicol Paediatric Plain is not tolerated inform families that disimpaction treatment can initially increase symptoms of soiling and abdominal pain

Answer 236

first-line: Movicol Paediatric Plain add a stimulant laxative if no response substitute a stimulant laxative if Movicol Paediatric Plain is not tolerated. Add another laxative such as lactulose or docusate if stools are hard continue medication at maintenance dose for several weeks after regular bowel habit is established, then reduce the dose gradually

Answer 237

do not use dietary interventions alone as first-line treatment although ensure the child is having adequate fluid and fibre intake consider regular toileting and non-punitive behavioural interventions for all children consider asking the Health Visitor or Paediatric Continence Advisor to help support the parents.

Answer 238

bottle-fed infants: give extra water in between feeds. Try gentle abdominal massage and bicycling the infant's legs breast-fed infants: constipation is unusual and organic causes should be considered

Answer 239

offer extra water, diluted fruit juice and fruits if not effective consider adding lactulose

Answer 240

- cough= barking seal-like, worse at night - stridor= DO NOT examine throat - fever - coryzal symptoms - increased work of breathing eg. retraction

Answer 241

-Occasional barking cough - No audible stridor at rest - No or mild suprasternal and/or intercostal recession - The child is happy and is prepared to eat, drink, and play

Answer 242

- Frequent barking cough - Easily audible stridor at rest - Suprasternal and sternal wall retraction at rest - No or little distress or agitation - The child can be placated and is interested in its surroundings

Answer 243

- Frequent barking cough - Prominent inspiratory (and occasionally, expiratory) stridor at rest - Marked sternal wall retractions - Significant distress and agitation, or lethargy or restlessness (a sign of hypoxaemia) - Tachycardia occurs with more severe obstructive symptoms and hypoxaemia

Answer 244

risk of precipitating airway obstruction

Answer 245

- moderate or severe croup - <3m - known upper airway abnormality eg. laryngomalacia, Down's - uncertain about diagnosis

Answer 246

acute epiglottitis, bacterial tracheitis, peritonsillar abscess and foreign body inhalation

Answer 247

- most clinically - CXR= steeple sign (PA view shows subglottic narrowing)

Answer 248

single dose oral dexamethasone (0.15mg/kg) regardless of severity (alternative is pred) Emergency Tx= nebulised adrenaline and high flow O2

Answer 249

laryngotracheobronchitis

Answer 250

parainfluenza virus type 1 or 3

Answer 251

Symptoms are typically worse at night and increase with agitation. Prodromal, non-specific upper respiratory tract symptoms (cough, rhinorrhoea, coryza, and fever) may have been present for between 12 and 72 hours. The clinical features of croup result from inflammation, swelling of upper airway structures (larynx, vocal cords and trachea), and oedema, leading to narrowing of the subglottic region.

Answer 252

increasing upper airway obstruction, sternal/intercostal recession, asynchronous chest wall and abdominal movement, fatigue, pallor or cyanosis, decreased level of consciousness or tachycardia. The degree of chest wall recession may diminish with the onset of respiratory failure as the child tires.

Answer 253

48hrs but can be up to 1w

Answer 254

inhaled budesonide (2 mg nebulised as a single dose) or intramuscular dexamethasone (0.6 mg/kg as a single dose) are possible alternatives.

Answer 255

if child is >10% dehydrated or 5-10% and oral/enteral rehydration not tolerated/possible

Answer 256

oral rehydration adequate

Answer 257

100 mL/kg for the first 10 kg 50 mL/kg for each 1 kg body weight over 10 kg

Answer 258

100 mL/kg for the first 10 kg 50 mL/kg for each 1 kg body weight between 10-20 kg 20 mL/kg for each 1 kg body weight over 20 kg (max. 2 litres in females, 2.5 litres in males)

Answer 259

0.9% sodium chloride + 5% dextrose potassium is added as required

Answer 260

significant lag in a child's physical, cognitive, behavioural, emotional, or social development, relative to established growth milestones. It is crucial for clinicians to identify and address these delays promptly to improve long-term outcomes.

Answer 261

Gross motor Fine motor and Vision Hearing, speech and language Social, emotional and behavioural

Answer 262

genetic disorders; prenatal exposure to toxins/drugs/alcohol; premature; nutritional deficiencies; environmental factors

Answer 263

doesn't smile at 10w can't sit unsupported at 12m can't walk at 18m

Answer 264

hand preference before 12m is abnormal and may indicate cerebral palsy

Answer 265

variant of normal, cerebral palsy and neuromuscular disorders eg. Duchenne muscular dystrophy

Answer 266

always check hearing environmental deprivation and general developmental delay

Answer 267

1) ASD 2) Cerebral palsy 3) Fragile X syndrome 4) Down syndrome 5) Fetal Alcohol Spectrum Disorders (FASDs)

Answer 268

A range of effects that can occur in an individual exposed to alcohol before birth.

Answer 269

Characterised by impairments in social interaction and communication alongside restricted interests and repetitive behaviours.

Answer 270

1) examination= physical and neuro for developmental milestones 2) Ix= genetic testing, metabolic screening, neuroimaging (MRI/CT), hearing/vision assessment 3) Refer for specialist assessment 4) Early intervention Services= regardless of cause; eg. occupational therapy, SALT, physio and educational support

Answer 271

Reaches for object Holds rattle briefly if given to hand Visually alert, particularly human faces Fixes and follows to 180 degrees

Answer 272

Holds in palmar grasp Pass objects from one hand to another Visually insatiable, looking around in every direction

Answer 273

Points with finger Early pincer

Answer 274

Good pincer grip Bangs toys together

Answer 275

12m cerebral palsy

Answer 276

Little or no head lag on being pulled to sit Lying on abdomen, good head control Held sitting, lumbar curve

Answer 277

Lying on abdomen, arms extended Lying on back, lifts and grasps feet Pulls self to sitting Held sitting, back straight Rolls front to back

Answer 278

Sits without support (Refer at 12 months)

Answer 279

Pulls to standins Crawls

Answer 280

Cruises Walks with one hand held

Answer 281

Walks unsupported (Refer at 18 months)

Answer 282

squats to pick up a toy

Answer 283

Runs Walks upstairs and downstairs holding on to rail

Answer 284

Rides a tricycle using pedals Walks up stairs without holding on to rail

Answer 285

Hops on one leg

Answer 286

normal variant and runs in families

Answer 287

7-8m (refer at 12m)

Answer 288

13-15m (refer at 18m)

Answer 289

smiles (refer at 10w)

Answer 290

laughs, enjoys friendly handling

Answer 291

not shy may put hand on bottle when being fed

Answer 292

shy; takes everything to mouth plays peek-a-boo

Answer 293

waves bye-bye plays pat-a-cake

Answer 294

drinks from cup + uses spoon, develops over 3m period helps getting dressed/undressed

Answer 295

takes off shoes, hat but unable to replace plays contentedly alone

Answer 296

competent with spoon, doesn't spill with cup puts hat and shoes on plays near others but not with them (parallel play)

Answer 297

uses spoon and fork

Answer 298

can dress and undress independently except for laces and buttons plays with other children

Answer 299

uses knife a fork

Answer 300

6w (refer at 10w)

Answer 301

Quietens to parents voice Turns towards sound Squeals

Answer 302

Double syllables 'adah', 'erleh'

Answer 303

says mama and dada understands no

Answer 304

knows and responds to own name

Answer 305

knows about 2-6 words (refer at 18m) understands simple commands- 'give it to mummy'

Answer 306

combines 2 words points to parts of the body

Answer 307

vocab of 200 words

Answer 308

Talks in short sentences (e.g. 3-5 words) Asks 'what' and 'who' questions Identifies colours Counts to 10 (little appreciation of numbers though)

Answer 309

Asks 'why', 'when' and 'how' questions

Answer 310

12-15m (refer at 18m if doesn't know 2-6 words)

Answer 311

7m (refer at 9m)

Answer 312

18-24w (refer 2-2.5yrs)

Answer 313

combined test

Answer 314

between 11-13+6 weeks

Answer 315

nuchal translucency measurement + serum B-HCG + pregnancy-associated plasma protein A (PAPP-A)

Answer 316

↑ HCG, ↓ PAPP-A, thickened nuchal translucency

Answer 317

combined test= similar to Downs but hCG LOWER so lower HCG, ↓ PAPP-A, thickened nuchal translucency

Answer 318

Quadruple test offered if women book later in pregnancy, offered betweeen 15-20w

Answer 319

between 15-20w

Answer 320

alpha-fetoprotein, unconjugated oestriol, human chorionic gonadotrophin and inhibin A

Answer 321

alpha-fetoprotein= ↓ unconjugated oestriol= ↓ human chorionic gonadotrophin= ↑

Answer 322

alpha-fetoprotein= ↓ unconjugated oestriol= ↓ human chorionic gonadotrophin= ↓

Answer 323

alpha-fetoprotein= ↑ unconjugated oestriol= ↔ human chorionic gonadotrophin= ↔

Answer 324

either a 'lower chance' or 'higher chance' result lower chance= 1 in 150 chance or more eg. 1 in 300 higher= 1 in 150 chance or less eg. 1 in 100

Answer 325

offered a second screening test (NIPT) or a diagnostic test (e.g. amniocentesis or chorionic villus sampling (CVS) NIPT preferred as non-invasive and very high sensitivity and specificity

Answer 326

analyses small DNA fragments that circulate in the blood of a pregnant woman (cell free fetal DNA, cffDNA) cffDNA derives from placental cells and is usually identical to fetal DNA analysis of cffDNA allows for the early detection of certain chromosomal abnormalities

Answer 327

Non-invasive prenatal screening test

Answer 328

sensitivity and specificity are very high for trisomy 21 (>99%) and similarly high for other chromosomal abnormalities

Answer 329

10w gestation

Answer 330

1) Combined test= 11-13+6w or quadruple test 15-20w (only if miss the combined) 2) If result comes back as 'higher chance', women is offered NIPT (preferred) or diagnostic test (eg. amniocentesis or chorionic villus sampling)

Answer 331

increased in age... 20yrs 1 in 1,500 30 1 in 800 35 1 in 270 40 1 in 100 45 1 in 50 or greater remember this is by starting at 1/1,000 at 30 years and then dividing the denominator by 3 (i.e. 3 times more common) for every extra 5 years of age

Answer 332

mode: - nondisjunction (94% of cases)= risk of recurrence is 1 in 100 if mother <35yrs - Robertsonian translocation (usually onto 14) (5%)= risk of recurrence is 10-15% if mother is translocation carrier or 2.5% if father is translocation carrier - Mosaicism (1%)

Answer 333

the presence of two genetically different populations of cells in the body

Answer 334

caused by a random event shortly after the egg and sperm join together. When cells are dividing, some cells receive an extra copy of chromosome 21. Others do not. People with mosaic Down syndrome have some cells with two and some cells with three copies of chromosome 21 diagnosed when there is a mixture of two types of cells. Some have the usual 46 chromosomes, and some have 47. Those cells with 47 chromosomes have an extra chromosome 21

Answer 335

approx 1 in 100 if the mother is less than 35 years old. If the trisomy 21 is a result of a translocation the risk is much higher

Answer 336

face= upslanting palpebral fissures, epicanthic folds, Brushfield spots in iris, protruding tongue, small low-set ears, round/flat face flat occiput single palmar crease, pronounced 'sandal gap' between big and first toe hypotonia congenital heart defects (40-50%) duodenal atresia Hirschsprung's disease

Answer 337

congenital heart defects (40-50%) duodenal atresia Hirschsprung's disease

Answer 338

upslanting palpebral fissures epicanthic folds Brushfield spots in iris protruding tongue small low-set ears round/flat face

Answer 339

flat occiput single palmar crease, hypotonia

Answer 340

multiple may be present endocardial cushion defect (most common, 40%, also known as atrioventricular septal canal defects) ventricular septal defect (c. 30%) secundum atrial septal defect (c. 10%) tetralogy of Fallot (c. 5%) isolated patent ductus arteriosus (c. 5%)

Answer 341

- sub fertility - learning difficulties - short stature - repeated resp infections (+hearing impairment from glue ear) - ALL - hypothyroidism - Alzheimer's disease - atlantoaxial instability

Answer 342

Males are almost always infertile due to impaired spermatogenesis. Females are usually subfertile, and have an increased incidence of problems with pregnancy and labour.

Answer 343

Features= duodenal atresia, Hirschsprungs and congenital heart defects Later Cx= ALL, alzheimer's, hypothyroidism, atlantoaxial instability

Answer 344

Down's syndrome

Answer 345

refractive errors are more common strabismus: seen in around 20-40% cataracts: congenital and acquired are both more common recurrent blepharitis glaucoma

Answer 346

otitis media and glue ear very common= hearing problems

Answer 347

describes the passage of gastric contents into the oesophagus with or without regurgitation and/or vomiting. It is considered a normal physiological process after feeds and meals in healthy infants, and it is often asymptomatic.

Answer 348

GOR that causes troublesome symptoms or complications that may need treatment. It can be clinically difficult to differentiate between GOR and GORD.

Answer 349

result of transient lower oesophageal sphincter relaxation and other anatomical and physiological features normal process after feeds in infants

Answer 350

preterm birth, parental history, obesity, hiatus hernia, neurodevelopmental disorders or genetic conditions, asthma, and cystic fibrosis.

Answer 351

most don't get any Cx reflux oesophagitis, oesophageal stricture, faltering growth, aspiration pneumonia, and dental erosion.

Answer 352

generally self-limiting, and symptoms usually begin before the age of 8 weeks and resolve before one year of age in 90% of infants.

Answer 353

visible regurgitation and one or more of the following: - Distressed behaviour such as excessive crying, crying when feeding, back arching. - Hoarseness and/or chronic cough. - A single episode of pneumonia. - Unexplained feeding difficulties such as refusing to feed, gagging, choking. - Faltering growth. - Heartburn, retrosternal pain, or epigastric pain (in children over one year of age).

Answer 354

- red flags - RFs and previous Tx - abdo exam - developmental history - weight and growth - feeding assessment eg. by health visitor

Answer 355

uncertainty; faltering growth; unexplained distress; GORD not responding to or needing ongoing treatment(s); feeding aversion; unexplained iron deficiency anaemia; onset after six months of age or persisting after one year of age.

Answer 356

- advise about breastfeeding technique, positioning and attachment - 1-2w trial of reducing feed volumes if formula fed - 1-2w trial of thicken formula if formula fed - 1-2w trial alginate theraoy - consider trial of Mx for possible cow's milk allergy if appropriate - consider 4w trial of omeprazole suspension if appropriate - consider referral if don't resolve or reoccur

Answer 357

before 8w and 90% resolve before 1yr if regurg +/or vomiting after 6m of age or >1yrs then ?different diagnosis

Answer 358

nocturnal, immediately post-prandial, long after meals, digested or undigested). Regurgitation may be frequent, with 5% of affected infants having six or more episodes each day.

Answer 359

breast-, formula-, or mixed feeding, and any feeding problems, including resistance or refusal to feed or dietary restrictions. If bottle-fed, ask about the type of formula used; how it is prepared; the size, timing, frequency, and duration of feeds; and the volume consumed over 24 hours. If breastfed, ask about the timing, frequency, and duration of feeds; quality of milk supply; and any feeding problems. Wet and dirty nappy frequency and any change.

Answer 360

?hypertrophic pyloric stenosis

Answer 361

? intestinal obstruction, for example, due to Hirschsprung disease, intestinal atresia, mid-gut volvulus, or intussusception.

Answer 362

? intestinal obstruction or another acute surgical condition such as strangulated hernia or anatomic abnormality.

Answer 363

? raised intracranial pressure (for example, caused by meningitis, hydrocephalus, or brain tumour)

Answer 364

? cow's milk allergy, gastroenteritis, inflammatory bowel disease, or an acute surgical condition such as intussusception or mid-gut volvulus.

Answer 365

? cow's milk allergy or food allergy

Answer 366

? suggests another cause for symptoms (not GORD/GOR), such as UTI.

Answer 367

Blood-stained vomit (haematemesis) not caused by blood ingested from a nosebleed or cracked maternal nipple and/or melaena — suggesting an upper gastrointestinal bleed. Bile-stained (green or yellow-green) vomiting, abdominal tenderness, mass, and/or distension — suggesting intestinal obstruction or another acute surgical condition needing paediatric surgery assessment. Frequent, forceful (projectile) vomiting in infants up to two months of age — suggesting hypertrophic pyloric stenosis needing paediatric surgery assessment. Fever and systemically unwell or dehydrated. Dysphagia — for example, due to an oesophageal motility disorder or other obstruction.

Answer 368

If an infant is well, thriving, and presents with effortless regurgitation of feeds and suspected gastro-oesophageal reflux (GOR)= It is normal and usually becomes less frequent with time and resolves in 90% of affected infants before one year of age. It does not usually need further investigation or treatment.

Answer 369

- breastfeeding technique - if persists= 1-2w trial alginate therapy (Gaviscon infant) - If improves= continue and stop Tx at regular intervals eg. every 2w to see if symptoms are improving and ?stop Tx - No improvement= 4w trial omeprazole suspension - Still no improvement= refer

Answer 370

Do NOT recommend the use of positional management (head elevation or left lateral positioning)

Answer 371

- reduce volume of feeds if XS for child's weight 1) 1-2w trial smaller more frequent feeds 2) 1-2w trial thickened formula 3) unsuccessful= stop thickened formula and 1-2w trial Gaviscon infant (alginate therapy) added to infant formula 4) If works then continue and stop Tx at regular intervals eg. every 2w to see if symptoms are improving and ?stop Tx

Answer 372

GOR (40% of infants regurg their feeds to certain extent so degree or normal physiology)

Answer 373

typically develops before 8 weeks vomiting/regurgitation= milky vomits after feeds, may occur after being laid flat excessive crying, especially while feeding

Answer 374

GOR= A normal physiological process where stomach contents occasionally flow back into the oesophagus; Typically occurs after meals and resolves without intervention, especially in infants and young children. Does not cause significant symptoms or complications in most cases GORD= pathological condition where reflux causes troublesome symptoms or complications (e.g., oesophagitis, stricture, or Barrett's oesophagus).; Symptoms include heartburn, regurgitation, chest pain, or difficulty swallowing, often requiring treatment.; Distinguished from GOR by frequency, severity, and impact on quality of life. GOR is benign and transient, while GORD is a chronic condition requiring medical attention due to associated symptoms and risks.

Answer 375

GORD distinguished from GOR by frequency, severity, and impact on quality of life. GOR= no signif symptoms or Cx, resolves without intervention and normal GORD= troublesome symptoms eg. pain, regurg; Cx eg. failure to thrive; impact on QOL, frequent and severe

Answer 376

distress failure to thrive aspiration frequent otitis media in older children dental erosion may occur

Answer 377

if medical treatment is ineffective then fundoplication may be considered

Answer 378

Henoch-Schonlein purpura

Answer 379

palpable purpuric rash (with localized oedema) over buttocks and extensor surfaces of arms and legs abdominal pain polyarthritis features of IgA nephropathy may occur e.g. haematuria, renal failure

Answer 380

Henoch-Schonlein purpura

Answer 381

analgesia for arthralgia Tx of nephropathy supportive

Answer 382

usually excellent, HSP is a self-limiting condition, especially in children without renal involvement BP and urinanalysis should be monitored to detect progressive renal involvement around 1/3rd of patients have a relapse

Answer 383

BP and urinalysis

Answer 384

Congenital inguinal hernia and Infantile umbilical hernia

Answer 385

Indirect hernias resulting from a patent processus vaginalis Occur in around 1% of term babies. More common in premature babies and boys 60% are right sided, 10% are bilaterally Should be surgically repaired soon after diagnosis as at risk of incarceration

Answer 386

Should be surgically repaired soon after diagnosis as at risk of incarceration

Answer 387

Symmetrical bulge under the umbilicus More common in premature and Afro-Caribbean babies The vast majority resolve without intervention before the age of 4-5 years Complications are rare

Answer 388

The vast majority resolve without intervention before the age of 4-5 years

Answer 389

elective caesarean section at term is advised

Answer 390

suppressive therapy and be advised that the risk of transmission to their baby is low

Answer 391

- insulinoma - self-administration of insulin/sulphonylureas - liver failure - Addison's - alcohol - nesidoblastosis

Answer 392

beta cell hyperplasia

Answer 393

increased ratio of proinsulin to insulin it is a tumour in your pancreas (makes extra insulin)

Answer 394

causes exaggerated insulin secretion mechanism is thought to be due to the effect of alcohol on the pancreatic microcirculation → redistribution of pancreatic blood flow from the exocrine into the endocrine parts → increased insulin secretion

Answer 395

hormonal response and sympathoadrenal response

Answer 396

the first response of the body is decreased insulin secretion. This is followed by increased glucagon secretion. Growth hormone and cortisol are also released but later

Answer 397

increased catecholamine-mediated (adrenergic) and acetylcholine-mediated (cholinergic) neurotransmission in the peripheral autonomic nervous system and in the central nervous system

Answer 398

Blood glucose levels and severity of symptoms are not always correlated, esp in pts with diabetes

Answer 399

cause autonomic symptoms due to the release of glucagon and adrenaline: - Sweating - Shaking - Hunger - Anxiety - Nausea

Answer 400

cause neuroglycopenic symptoms due to inadequate glucose supply to the brain: - Weakness - Vision changes - Confusion - Dizziness

Answer 401

convulsion and coma

Answer 402

BM <3.3: - sweating, shaking, hunger, anxiety, nausea BM <2.8: - weakness, vision changes, confusion, dizziness Severe and rare: - coma and convulsion

Answer 403

is cause is not clear then= serum insulin and c-peptide levels

Answer 404

insulin and C-peptide are released in equimolar amounts from the pancreas, making C-peptide a marker of endogenous insulin production.

Answer 405

Interpretation= endogenous insulin production Causes= Insulinoma, Sulfonylurea use/abuse

Answer 406

Interpretation= Exogenous insulin administration Causes= Exogenous insulin overdose, Factitious disorder

Answer 407

Interpretation= Non-insulin-related cause Causes= Alcohol-induced hypoglycaemia, Critical illness (e.g., sepsis), Adrenal insufficiency, Growth hormone deficiency, Fasting/starvation

Answer 408

Initially, oral glucose 10-20g should be given in liquid, gel or tablet form Alternatively, a propriety quick-acting carbohydrate may be given: GlucoGel or Dextrogel. A 'HypoKit' may be prescribed which contains a syringe and vial of glucagon for IM or SC injection at home

Answer 409

If the patient is alert, a quick-acting carbohydrate may be given (like in the community) If the patient is unconscious or unable to swallow, subcutaneous or intramuscular injection glucagon may be given. Alternatively, intravenous 20% glucose solution may be given through a large vein

Answer 410

in Mexico in early 2009. In June 2009, the WHO declared the outbreak to be a pandemic. pandemic caused by new strain of H1N1 virus

Answer 411

subtype of influenzae A virus and most common cause of flu in humans

Answer 412

patients with chronic illnesses and those on immunosuppressants pregnant women young children under 5 years old

Answer 413

fever greater than 38ºC myalgia lethargy headache rhinitis sore throat cough diarrhoea and vomiting A minority of patients may go on to develop an acute respiratory distress syndrome which may require ventilatory support.

Answer 414

2 main Tx available: 1) Oseltamivir (Tamiflu)= oral 2) Zanamivir (Relenza)= inhaled (or IV if acutely unwell)

Answer 415

a neuraminidase inhibitor which prevents new viral particles from being released by infected cells nausea, vomiting, diarrhoea and headaches

Answer 416

a neuraminidase inhibitor may induce bronchospasm in asthmatics

Answer 417

Small bowel obstruction secondary to adhesions

Answer 418

Maximum normal diameter = 35 mm Valvulae conniventes extend all the way across

Answer 419

Maximum normal diameter = 55 mm Haustra extend about a third of the way across

Answer 420

Invagination of one portion of the bowel into the lumen of the adjacent bowel, most commonly around the ileo-caecal region

Answer 421

6-18m old boys>girls

Answer 422

- intermittent, severe, crampy, progressive abdo pain - inconsolable crying - during paroxysm (attack) infant will draw knees up and turn pale - vomiting - bloodstained stool= 'red-current jelly' late sign - sausage-shaped mass in RUQ

Answer 423

intussusception

Answer 424

red-current jelly like stool (bloodstained)

Answer 425

intussusception

Answer 426

USS= target like mass

Answer 427

1) reduction by air insufflation under radiological control 2) fails or signs of peritonitis= surgery

Answer 428

type of vasculitis predominately seen in children rare but important to diagnose as serious Cx

Answer 429

coronary artery aneurysm

Answer 430

- high grade fever >5d resistant to antipyretics - conjunctival injection - bright red, cracked lips - strawberry tongue - cervical lymphadenopathy - red palms of hands and soles of feet which later peel

Answer 431

clinical- no specific diagnostic test

Answer 432

high-dose aspirin + IV Ig and ECHO to screen for coronary artery aneurysm

Answer 433

high-grade, lasts for >5d and resistant to antipyretics

Answer 434

History= Sputum, wheeze, breathlessness may be absent in acute bronchitis whereas at least one tends to be present in pneumonia. Examination= No other focal chest signs (dullness to percussion, crepitations, bronchial breathing) in acute bronchitis other than wheeze. Moreover, systemic features (malaise, myalgia, and fever) may be absent in acute bronchitis, whereas they tend to be present in pneumonia.

Answer 435

BMI of less than 18.5; or unintentional weight loss greater than 10% within the last 3-6 months; or a BMI of less than 20 and unintentional weight loss greater than 5% within the last 3-6 months

Answer 436

MUST (Malnutrition Universal Screen Tool).

Answer 437

dietician support if the patient is at high-risk a 'food-first' approach with clear instructions (e.g. 'add full-fat cream to mashed potato'), rather than just prescribing oral nutritional supplements (ONS) such as Ensure if ONS are used they should be taken between meals, rather than instead of meals

Answer 438

RNA paramyxovirus

Answer 439

in winter and spring

Answer 440

by droplets respiratory tract epithelial cells → parotid glands → other tissues

Answer 441

infective 7 days before and 9 days after parotid swelling starts

Answer 442

- fever - malaise, muscular pain - parotitis ('earache', 'pain on eating')= unilaterally 1st then becomes bilateral in 70%

Answer 443

MMR vaccine= 80% efficacy

Answer 444

- rest - paracetamol for high fever/discomfort - notifiable disease

Answer 445

- orchitis - hearing loss= unilateral and transient - meningoencephalitis - pancreatitis

Answer 446

uncommon in pre-pubertal males but occurs in around 25-35% of post-pubertal males. Typically occurs four or five days after the start of parotitis. Unilateral= only transiently, diminish sperm count, mobility, and morphology. Bilateral= 15-30% men; causes infertility in 30-87% of them.

Answer 447

acute infectious disease caused by a paramyxovirus, characterised by bilateral parotid swelling. It is spread by respiratory droplets, fomites or saliva.

Answer 448

from around 1–2 days before onset of symptoms to about 9 days afterwards, although it may be asymptomatic in 15–20% of people.

Answer 449

most have life-long immunity after 1 episode of infection

Answer 450

oophoritis but rarely causes infertility or premature menopause

Answer 451

cerebellar ataxia, facial palsy, transverse myelitis, and Guillain–Barre syndrome, thyroiditis, mastitis, prostatitis, hepatitis, and thrombocytopenia.

Answer 452

inform local Health Protection Team

Answer 453

usually self resolves ever 1-2w with no long term Cx

Answer 454

stay off for 5d after the initial development of parotitis

Answer 455

There are signs of mumps encephalitis (for example, an altered level or loss of consciousness, focal neurological signs, or seizures). The person develops mumps meningitis (characterized by severe headache, neck ache, high fever, lethargy, and vomiting). Following epididymo-orchitis (particularly if it was bilateral), a man has an abnormal semen analysis or is experiencing infertility.

Answer 456

offered immunization with the combined measles, mumps, and rubella vaccine if they are not already fully immunized, unless they are pregnant or severely immunocompromised.

Answer 457

no as it is a live vaccine

Answer 458

same way as otherwise healthy people but can't give vaccine if a contact so if they develop symptoms seek medical advice

Answer 459

Bed rest. Scrotal support. Application of warm or cold packs. Paracetamol or ibuprofen. Inform the man that, in most cases, the symptoms will completely resolve within 2 weeks, and there are unlikely to be long-term problems with fertility. If the man is concerned about fertility, offer semen analysis at least 3 months after the mumps has resolved

Answer 460

physical, emotional, sexual abuse, neglect, fabricated or induced illness

Answer 461

Severe and persistent infestations (e.g. Scabies or head lice) Parents who do not administer essential prescribed treatment Parents who persistently fail to obtain treatment for tooth decay Parents who repeatedly fail to attend essential follow-up appointments Parents who persistently fail to engage with child health promotion Failure to dress the child in suitable clothing Animal bite on an inadequately supervised child

Answer 462

Failure to seek medical advice which compromises the child's health Child who is persistently smelly and dirty Repeat observations that: - poor standards of hygiene that affects the child's health - inadequate provision of food - living environment that is unsafe for the child's development stage

Answer 463

Persistent dysuria or anogenital discomfort without a medical explanation Gaping anus in a child during examination without a medical explanation Pregnancy in a young women aged 13-15 years Hepatitis B or anogenital warts in a child 13-15 years

Answer 464

Persistent or recurrent genital or anal symptoms associated with a behavioural or emotional change Anal fissure when constipation and Crohn's disease have been excluded as the cause STI in a child younger than 12 years (where there is no evidence of vertical or blood transmission) Sexualised behaviour in a prepubertal child

Answer 465

Any serious or unusual injury with an absent or unsuitable explanation Cold injuries in a child with no medical explanation Hypothermia in a child without a suitable explanation Oral injury in a child with an absent or suitable explanation

Answer 466

Bruising, lacerations or burns in a child who is not independently mobile or where there is an absent or unsuitable explanation Human bite mark not by a young child One or more fractures if there is an unsuitable explanation, including: - fractures of different ages - X-ray evidence of occult fractures Retinal haemorrhages with no adequate explanation

Answer 467

- story inconsistent with injuries - repeated attendances at A&E (see different doctors) - delayed presentation - usual changes in behaviour/emotion/developmental stage not explained by anything else - child with a frightened, withdrawn appearance = 'frozen watchfulness' - child discloses abuse themselves - refusing to let child to speak to healthcare professional on their own - poor school attendance - failure to attend appointments

Answer 468

bruising fractures: particularly metaphyseal, posterior rib fractures or multiple fractures at different stages of healing torn frenulum: e.g. from forcing a bottle into a child's mouth burns or scalds failure to thrive STIs e.g. Chlamydia, Gonorrhoea, Trichomonas

Answer 469

metaphyseal, posterior rib fractures or multiple fractures at different stages of healing

Answer 470

causing physical harm to a child such as shaking, hitting, throwing, burning, or suffocating.

Answer 471

forcing or tempting a child to take part in sexual activities.

Answer 472

conveying to children/young people that they are worthless, unloved, or a burden.

Answer 473

persistent failure to meet the child’s basic physical and/or psychological needs.

Answer 474

misrepresentation of the child as ill by the caregiver by fabricating or inducing symptoms.

Answer 475

advice should be sought from a named professional for child safeguarding or a senior colleague. Consent should be obtained before sharing confidential information unless this will increase the risk of harm to the child or young person.

Answer 476

Children's social care should be contacted to discuss the need for a referral. If the child or young person is thought to be in immediate danger, they should be referred immediately to children's social care and/or the police. If hospital admission is needed, the admitting paediatrician should be made aware of any safeguarding concerns.

Answer 477

Other alerting features should be sought, then: Information should be obtained from other agencies and colleagues. If these investigations lead to a suspicion of maltreatment, children's social care should be contacted to discuss the need for a referral. If it is thought that referral is not justified, the child should be reviewed regularly. A written record needs to be made of the outcome in cases where maltreatment has been considered.

Answer 478

Erb-Ducenne paralysis and Klumpke's paralysis

Answer 479

damage to C5,6 roots winged scapula may be caused by a breech presentation

Answer 480

damage to T1 loss of intrinsic hand muscles due to traction

Answer 481

Do not perform an intimate examination unless there is an urgent health need to do so. A forensic intimate examination should only be undertaken by professionals specifically trained in forensic aspects of sexual assaults (such as the police if the person wishes to report the assault, or to a Sexual Assault Referral Centre (SARC) if locally available). DNA can be gathered for up to 7 days after vaginal penetration, up to 2 days in oral penetration and for up to 3 days in anal/penile penetration irrespective of washing or bathing. Assess the need for emergency contraception (EC) Assess the need for prophylaxis against sexually transmitted infection (STI

Answer 482

1 working day

Answer 483

child over the age of 12 years has sufficient understanding, and it is presumed by law that a young person aged 16 and older has the capacity to consent to medical treatment.

Answer 484

Document all actions in the child or young person's clinical record, including: - What was observed and/or heard, from whom, and when. - Any concerns, including those considered minor. - Decisions or actions relating to those concerns. - Any outcomes.

Answer 485

Arrange hospital admission where required. Ensure that the receiving paediatrician is aware of your concerns. If the child or young person is thought to be in immediate danger, refer immediately to children's social care and/or the police. Consider the safety of other children living with or in contact with the suspected perpetrator. If the child or young person is not thought to be in immediate danger, contact children's social care to discuss the need for a referral to them, using local multi-agency safeguarding procedures. A referral may trigger a child protection investigation, a family assessment to determine whether supportive services need to be offered, or alternative explanations may be identified.

Answer 486

caused by hypertrophy of the circular muscles of the pylorus

Answer 487

2-4w of life with vomiting, although rarely may present later at up to 4m

Answer 488

boys 4x > girls first borns most commonly affected 10-15% +ve FHx

Answer 489

'projectile' vomiting, typically 30 minutes after a feed constipation and dehydration may also be present a palpable mass may be present in the upper abdomen hypochloraemic, hypokalaemic alkalosis due to persistent vomiting

Answer 490

hypochloraemic, hypokalaemic alkalosis

Answer 491

upper abdo

Answer 492

'projectile' vomiting, typically 30 minutes after a feed

Answer 493

Ramstedt pyloromyotomy.

Answer 494

pyloric stenosis

Answer 495

prompt Tx can reduce risk of permanent damage to joint and systemic infection

Answer 496

hip, knee and ankle

Answer 497

joint pain limp fever systemically unwell: lethargy

Answer 498

swollen, red joint typically, only minimal movement of the affected joint is possible

Answer 499

joint aspiration: for culture. Will show a raised WBC raised inflammatory markers blood cultures

Answer 500

Kocher criteria: - fever >38.5 degrees C - non-weight bearing - raised ESR - raised WCC

Answer 501

severe systemic reaction to staphylococcal exotoxins, the TSST-1 superantigen toxin. Commonly known be related to infected tampons.

Answer 502

1) fever: temperature > 38.9ºC 2) hypotension:SBP < 90 3) diffuse erythematous rash 4) desquamation of rash, especially of the palms and soles 5) involvement of three or more organ systems: e.g. gastrointestinal (diarrhoea and vomiting), mucous membrane erythema, renal failure, hepatitis, thrombocytopenia, CNS involvement (e.g. confusion)

Answer 503

removal of infection focus (e.g. retained tampon) IV fluids IV antibiotics

Answer 504

Ix for possible underlying cause and damage to kidneys (unlike in adults)

Answer 505

E. coli (responsible for around 80% of cases) Proteus Pseudomonas

Answer 506

- incomplete bladder emptying= infrequent voiding, hurried micturition, obstruction by full rectum due to constipation, neuropathic bladder - Vesicoureteric reflux= developmental anomaly found in 35% children with UTI - Poor hygiene eg. not wiping front to back in girls

Answer 507

boys until 3m (due to more congenital abnormalities) then it is a lot higher in girls

Answer 508

8% girls and 2% boys

Answer 509

poor feeding, vomiting, irritability

Answer 510

abdo pain, fever, dysuria

Answer 511

dysuria, frequency, haematuria

Answer 512

T >38C, loin pain/tenderness

Answer 513

- any signs or symptoms - unexplained fever 38C or higher (test urine after 24hrs at latest) - have an alternative site of infection but who remain unwell (consider urine test after 24hrs at latest)

Answer 514

clean catch if not possible= urine collection pads (cotton wool balls, gauze & sanitary towels NOT suitable) if non-invasive methods not possible= invasive methods eg. suprapubic aspiration

Answer 515

<3m= immediate referral to paeds >3m with upper UTI= admit to hospital or oral Abx cephalosporin or co-amox 7-10d >3m with lower UTI= oral Abx 3d depending on local guidelines eg. trimeth, nitro, cephalo or amox; bring back if unwell still after 24-48hrs Recurrent UTI= Abx prophylaxis

Answer 516

urine collection for microscopy and culture (not just microscopy or dip- inadequate for diagnosis) may need imaging of urinary tract: - static radioisotope scan eg. DMSA= 4-6m after initial infection if recurrent UTI - MCUG= only if <6m and present with atypical or recurrent infections

Answer 517

- <6m with 1st UTI which responds to Tx should have USS within 6w - >6m with 1st UTI which responds to Tx don't need imaging unless recurrent or atypical infection

Answer 518

need prompt consideration of possible underlying causes and damage to kidneys (renal scarring)

Answer 519

seriously ill poor urine flow abdominal or bladder mass raised creatinine septicaemia failure to respond to treatment with suitable antibiotics within 48 hours infection with non-E. coli organisms

Answer 520

static radioisotope scan (e.g. DMSA): identifies renal scars.

Answer 521

if <6m and present with atypical or recurrent infections identifies vesicoureteric reflux

Answer 522

2+ episodes of UTI with acute pyelonephritis/upper urinary tract infection or 1 episode of UTI with acute pyelonephritis/upper urinary tract infection plus one or more episode of UTI with cystitis/lower urinary tract infection or 3+ episodes of UTI with cystitis/lower urinary tract infection.

Answer 523

referred to paeds specialist for assessment and Ix

Answer 524

Tx, specialist advice and consider Abx prophylaxis if behavioural and hygiene measures not effective If Abx prophy review within 6m

Answer 525

During the acute infection for children aged under 6 months with recurrent UTI. Within 6 weeks for children aged 6 months and over with recurrent UTI.

Answer 526

primary infection with varicella zoster virus

Answer 527

reactivation of dormant varicella zoster virus in dorsal root ganglion

Answer 528

very infectious spread via resp route can be caught by someone with shingles

Answer 529

4d before rash until 5d after rash first appeared

Answer 530

older children/adults

Answer 531

- fever initially - itchy rash starting on head/trunk before spreading - rash initially macular then papular then vesicular - systemic upset mild

Answer 532

supportive= trim nails, keep cool, calamine lotion

Answer 533

most infectious period is 1-2 days before the rash appears, but infectivity continues until all the lesions are dry and have crusted over (usually about 5 days after the onset of the rash).

Answer 534

varicella zoster immunoglobulin (VZIG) if chickenpox develops consider IV aciclovir

Answer 535

secondary bacterial infection of the lesions= usually single infected lesion/small area of cellulitis but in small no can cause= invasive group A strep soft tissue infections resulting in necrotising fasciitis

Answer 536

secondary bacterial infection of the lesions causing invasive group A strep soft tissue infections resulting in necrotising fasciitis

Answer 537

- pneumonia - encephalitis (cerebellar invl may be seen) - disseminated haemorrhagic chickenpox - arthritis, nephritis, pancreatitis v rare

Answer 538

miliary opacities secondary to healed varicella pneumonia. Multiple tiny calcific miliary opacities noted throughout both lungs. These are of uniform size and dense suggesting calcification. There is no focal lung parenchymal mass or cavitating lesion seen.

Answer 539

chickenpox

Answer 540

virus persists in sensory nerve ganglia of dorsal root, yrs later it can reactivate causing herpes zoster (shingles)

Answer 541

young children= bacterial skin infection adults= lung invl pregnancy= severe materal chickenpox and fetal varicella syndrome; later preg, varicella can cause neonatal chickenpox immunocompromised= evere disseminated chickenpox with varicella pneumonia, encephalitis, hepatitis, and haemorrhagic complications.

Answer 542

Small, erythematous macules which appear on the scalp, face, trunk, and proximal limbs, and progress over 12–14 hours to papules, clear vesicles (which are intensely itchy), and pustules. Vesicles can also occur on the palms and soles, and mucous membranes, with painful and shallow oral or genital ulcers. Vesicles appear in crops. Crusting occurs usually within 5 days, and crusts fall off after 1–2 weeks.

Answer 543

urgent specialist advice on whether to continue to breastfeed & whether baby needs Tx to minimise risk of Cx

Answer 544

immunocompetent adult or adolescent 14yrs+ who presents within 24hrs of rash, esp if severe or at risk of Cx

Answer 545

chlorphenamine (avoid in preg, breastfeeding and <1yrs)

Answer 546

immediate specialist advice from obs: - may advise antivirals - close monitoring daily and low threshold for admission (or if can't monitor admit) - deteriorat= admission admit if= resp or neuro symptoms, haemorrhagic rash, bleeding, severe disease, immunosuppression eg. recent use of systemic corticosteorids

Answer 547

admit if suspect serious Cx specialist advice ?admission for IV aciclovir

Answer 548

- contact in same room 15mins+ - face-to-face (conversation) - chickenpox contact from 24hrs before onset of rash to crusting of lesions - disseminated zoster contact from 48hrs before rash onset to crusting of lesions - localised zoster contact eg. opthalmic from day of rash onset until crusting of lesions

Answer 549

definitie history of chickenpox or shingles or 2 doses of varicella containing vaccine and not immunocompromised

Answer 550

urgent specialist advice - varicella zoster IgG within 24-48hrs of exposure= if shows V-Z IgG then evidence of immunity so can just reassure; if negative then prophylaxis needed (antivirals or VZ immunoglobulin)

Answer 551

Fever initially Itchy, rash starting on head/trunk before spreading. Initially macular then papular then vesicular Systemic upset is usually mild

Answer 552

Prodrome: irritable, conjunctivitis, fever Koplik spots: white spots ('grain of salt') on buccal mucosa Rash: starts behind ears then to whole body, discrete maculopapular rash becoming blotchy & confluent

Answer 553

Fever, malaise, muscular pain Parotitis ('earache', 'pain on eating'): unilateral initially then becomes bilateral in 70%

Answer 554

Rash: pink maculopapular, initially on face before spreading to whole body, usually fades by the 3-5 day Lymphadenopathy: suboccipital and postauricular

Answer 555

Also known as fifth disease or 'slapped-cheek syndrome' Caused by parvovirus B19 Lethargy, fever, headache 'Slapped-cheek' rash spreading to proximal arms and extensor surfaces

Answer 556

Reaction to erythrogenic toxins produced by Group A haemolytic streptococci Fever, malaise, tonsillitis 'Strawberry' tongue Rash - fine punctate erythema sparing the area around the mouth (circumoral pallor), 'sand paper like'

Answer 557

Caused by the coxsackie A16 virus Mild systemic upset: sore throat, fever Vesicles in the mouth and on the palms and soles of the feet

Answer 558

Erythema infectiosum or fifth disease

Answer 559

slapped cheek

Answer 560

parovirus B19

Answer 561

- mild feverish illness hardly noticeable - rash appears after a few days= rose-red rash on cheeks, may spread to rest of body but rarely involves palms and soles - child starts to feel better when rash appears - rash peaks after a week - for months after warm bath, sunlight, heat or fever will trigger bright red cheeks and the rash again

Answer 562

recurrence of slapped cheeked for months after initial illness

Answer 563

slapped cheek

Answer 564

no Tx needed adults= virus may cause acute arthritis

Answer 565

if exposed before 20w then prompt advice as can affect unborn baby in 1st 20w of preg

Answer 566

resp route

Answer 567

3-5d before the appearance of the rash, no longer infectious once rash appears

Answer 568

no school exclusion as no longer infectious by time rash occurs

Answer 569

self-limiting condition affecting children

Answer 570

Coxsackie A16 (most common) Enterovirus 71 (more serious) (intestinal viruses of the Picornaviridae family)

Answer 571

very contagious and typically occurs in outbreaks at nursery

Answer 572

- mild systemic upset= sore throat, fever - oral ulcers - followed later by vesicles on palms and soles of the feet

Answer 573

Hand, foot and mouth disease

Answer 574

symptomatic= hydration and analgesia, soft diet, measures to reduce risk of transmission reassurance no link to disease in cattle

Answer 575

do not need school exclusion keep child off if they are unwell until feel better; contact school if you suspect may be large outbreak

Answer 576

vesicular eruptions in the mouth and papulovesicular lesions of the distal limbs mild and self-limiting

Answer 577

Coxsackie A16 virus

Answer 578

caused by the Coxsackie virus A6 and presents with a more widespread and extensive skin disease, nail shedding, and a higher risk for adult infection.

Answer 579

normally immune following previous exposure but adult cases can occur

Answer 580

rare dehydration most common due to oral pain secondary bacterial infection of lesions rare= encephalitis, aseptic meningitis, acute flaccid paralysis

Answer 581

clinical= fever (38-39), malaise, loss of apeitie, cough, abdo pain, sore mouth; scattered ulcerative lesions of oral cavity within 1-2d; macules and papules of hands and feet after oral lesions; sides of fingers, dorsum of hands and margins of heels most common

Answer 582

avoid close contact, esp around time of delivery if contact within 3w of delivery then seek specialist advice

Answer 583

educed urine output, lethargy, cold peripheries, and reduced skin turgor

Answer 584

may last 12–36 hours. Early symptoms are fever (typically 38–39°C), malaise, loss of appetite, cough, abdominal pain, and sore mouth. Rarely, vomiting occurs if HFMD is caused by enterovirus 71.

Answer 585

mild illness= sore mouth/throat, low grade fever (38C typically), abdo pain, cough within 1-2 days get scattered ulcerative lesions of oral cavity then soon after get macules and papules of the hands and feet

Answer 586

Scattered ulcerative lesions of the oral cavity occur within 1–2 days. Typically, they begin as 2–8 mm erythematous macules and papules, and appear most commonly on the hard palate, tongue, and buccal mucosa, but also on the lips and pharynx. Lesions progress rapidly to vesicles, which readily erode leaving shallow yellow-grey ulcers surrounded by an erythematous halo. In severe cases, the lesions may coalesce leaving the tongue red and oedematous.

Answer 587

Macules and papules of the hands and feet usually soon follow the oral lesions. Typically, they are 2–5 mm sparse erythematous macules and papules, often with a central greyish vesicle. The sides of the fingers, dorsum of the hands, and margins of the heels are more frequently affected than the palms or the soles. Lesions are frequently elliptical with the long axis running parallel to the skin lines. The buttocks and groin area may also be affected. Lesions may be asymptomatic or painful.

Answer 588

more widespread rash that extends beyond the palms and soles and may preferentially occur in areas prone to atopic dermatitis (the antecubital and popliteal fossae). Petechiae and severe blistering may be observed. There may be subsequent skin peeling and/or nail shedding.

Answer 589

exanthem subitum or sixth disease

Answer 590

roseola infantum

Answer 591

Roseola infantum

Answer 592

human herpes virus 6 (HHV6)

Answer 593

children 6m-2yrs

Answer 594

- high fever lasting a few days - followed by maculopapular rash - Nagayama spots - febrile convulsions in 10-15% - diarrhoea and cough

Answer 595

papular enanthem on the uvula and soft palate

Answer 596

aseptic meningitis hepatitis

Answer 597

not needed

Answer 598

supportive maintain fluids

Answer 599

German measles

Answer 600

viral infection caused by togavirus

Answer 601

risk of congenital rubella syndrome - if 20w gestation or less need to refer urgently to obs and arrange serology testing and contact HPT

Answer 602

MMR vaccine

Answer 603

winter and spring

Answer 604

from 7d before symptoms appear to 4d after the onset of the rash

Answer 605

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

Answer 606

maculopapular, initially on the face before spreading to the whole body, usually fades by the 3-5 day

Answer 607

arthritis thrombocytopaenia encephalitis myocarditis

Answer 608

miscarriage, stillbirth, congenital rubella syndrome (CRS)

Answer 609

ary according to the stage of pregnancy when infection occurs — before 8–10 weeks gestation there is a high likelihood of multiple defects.

Answer 610

not specific to clinical features, needs to be confirmed with lab Ix 1) notify HPT 2) pregnant= serology testing 3) others= HPT will provide advice on testing eg. may request an oral fluid sample

Answer 611

suboccipital, postauricular, and cervical) — may precede rash and last for 2 weeks after the rash resolves.

Answer 612

pregnant women

Answer 613

- notify local Health Protection Team immediately if suspect - drinks fluids, rest, paracetamol/ibuprofen - school exclusion for 5d after rash starts

Answer 614

at least 5d after the rash starts and avoid contact with pregnant women

Answer 615

no documented risk of congenital rubella syndrome

Answer 616

no Tx to prevent Do not give MMR in preg if haven't already have it, wait till after delivery

Answer 617

rubella IgM not detected and IgG detected= likely rubella infection is remote and advise to return if rash develops IgM and IgG not detected= susceptible to rubella, retest after 4w IgM detected (regardless of IgG)= second confirmatory test

Answer 618

Reaction to erythrogenic toxins produced by Group A haemolytic strep (usually strep pyogenes)

Answer 619

2-8yrs, peak at 4yrs

Answer 620

Group A haemolytic strep (usually strep pyogenes)

Answer 621

via the respiratory route by inhaling or ingesting respiratory droplets or by direct contact with nose and throat discharges, (especially during sneezing and coughing)

Answer 622

- fever 24-48hrs - malaise, headache, N/V - sore throat - strawberry tongue - rash= fine punctate erythema 1st on torso and spares palms and soles; more obvious in flexures; rough sandpaper texture; flushed appearance with cicumoral pallor - desquamination later around fingers and toes

Answer 623

fine punctate erythema ('pinhead') which generally appears first on the torso and spares the palms and soles children often have a flushed appearance with circumoral pallor. The rash is often more obvious in the flexures it is often described as having a rough 'sandpaper' texture desquamination occurs later in the course of the illness, particularly around the fingers and toes

Answer 624

scarlet fever

Answer 625

throat swab (not routinely indicated) but start Abx immediately don't wait for results

Answer 626

- notifiable disease - phenoxymethylpenicillin (penicillin V) 10 days - azithromycin if allergy

Answer 627

can return to school 24hrs after starting Abx

Answer 628

- otitis media (most common) - rheumatic fever= 20d after infection - acute glomerulonephritis= 10d after infection - invasive Cx= bacteraemia, meningitis, necrotizing fasciitis; rare but may present acutely with life-threatening illness

Answer 629

otitis media

Answer 630

incubation period is usually 2–3 days. People can be infectious for 2–3 weeks after the onset of symptoms, unless they are treated.

Answer 631

'a credible report of two or more probable or confirmed scarlet fever cases attending the same school or nursery or other childcare setting, notified within 10 days of each other (two maximum incubation periods), with an epidemiological link between cases, for example they are in the same class or year group'.

Answer 632

- pregnant - postpartym (within 28d giving birth) - neonates (up to 28d) - 75yrs+ -immunocompromised/suppressed - DM - CVD - malignancy - chickenpox with active lesions within 7d before iGAS infection - within 48hrs starting Abx if exposure ongoing - IVDU - alcohol dependent - cormorbidities eg. skin breakdown

Answer 633

Invasive GAS infection, such as pneumonia, meningitis, streptococcal toxic shock syndrome, or necrotizing fasciitis.

Answer 634

Initial sore throat, fever, headache, fatigue, nausea, and vomiting. A pinpoint, sandpaper-like blanching rash that develops on the trunk within 48 hours after initial symptoms, before spreading to the rest of the body and flexures. Possible strawberry tongue, cervical lymphadenopathy, circumoral pallor.

Answer 635

children <5yrs= NICE fever traffic light system FeverPAIN score= assess likelihood of Strep infection in adult with sore throat and guide decision making re antibiotic usage

Answer 636

otitis media Viral wheeze, bronchiolitis and croup in infants and young children sinusitis

Answer 637

torsion of colon around its mesenteric axis resulting in compromised blood flow and closed loop obstruction

Answer 638

Sigmoid volvulus (80%)= large bowel obstruction caused by the sigmoid colon twisting on the sigmoid mesocolon. A similar problem may also occur at the caecum (20% of cases).

Answer 639

In most people (around 80%) the caecum is a retroperitoneal structure so not at risk of twisting. In the remaining minority there is however developmental failure of peritoneal fixation of the proximal bowel putting these patients at risk of caecal volvulus.

Answer 640

Sigmoid and caecal

Answer 641

older patients chronic constipation Chagas disease neurological conditions e.g. Parkinson's disease, Duchenne muscular dystrophy psychiatric conditions e.g. schizophrenia

Answer 642

all ages adhesions pregnancy

Answer 643

constipation abdominal bloating abdominal pain nausea/vomiting

Answer 644

large bowel obstruction (large, dilated loop of colon, often with air-fluid levels) + coffee bean sign

Answer 645

small bowel obstruction may be seen

Answer 646

sigmoid volvulus: rigid sigmoidoscopy with rectal tube insertion caecal volvulus: management is usually operative. Right hemicolectomy is often needed

Answer 647

congenital diverticulum of the small intestine. It is a remnant of the omphalomesenteric duct (also called the vitellointestinal duct) and contains ectopic ileal, gastric or pancreatic mucosa.

Answer 648

irregular, bulging pouch in the colon wall

Answer 649

occurs in 2% of the population is 2 feet from the ileocaecal valve is 2 inches long

Answer 650

Meckel's diverticulum

Answer 651

- usually asymptomatic - abdo pain mimicking appendicitis - rectal bleeding= painless massive GI bleeding - intestinal obstruction= secondary to omphalomesenteric band (most common), volvulus and intussusception

Answer 652

Meckel's diverticulum

Answer 653

Meckel's diverticulum

Answer 654

- haemodynamically stable with less severe or intermittent bleeding= Meckel's scan - more severe eg. transfusion required= mesenteric arteriography

Answer 655

uses 99m technetium pertechnetate, which has an affinity for gastric mucosa haemodynamically stable with less severe or intermittent bleeding in Meckel's diverticulum

Answer 656

removal if narrow neck or symptomatic options are between wedge excision or formal small bowel resection and anastomosis

Answer 657

normally, in the foetus, there is an attachment between the vitellointestinal duct and the yolk sac. This disappears at 6 weeks gestation the tip is free in the majority of cases associated with enterocystomas, umbilical sinuses, and omphaloileal fistulas. arterial supply: omphalomesenteric artery. typically lined by ileal mucosa but ectopic gastric mucosa can occur, with the risk of peptic ulceration. Pancreatic and jejunal mucosa can also occur.

Answer 658

overdoses (e.g. paracetamol) self-mutilation (e.g. cutting, burning)

Answer 659

mental health or behavioural problems, e.g. depression, severe anxiety and impulsivity a history of self-harm living in care or a secure institution abusive home life poor communication with parents

Answer 660

some feel that has positive purpose= way to relieve unbearable pressure or pain some view it as suicide prevention strategy, means of protecting themselves coping strategy over which they have control may be means of communicating pain and distress to others

Answer 661

normal behaviour for a 2-year-old. However, if this behaviour persists beyond 3 years it could be a sign of autism.

Answer 662

could be sign of autism

Answer 663

if excessive or usual patterns

Answer 664

haematological cause or non-accidental injury

Answer 665

Coagulation disorders= - haemorrhagic disease of the newborn - haemophilia Thrombocytopaenia= maternal alloimmune thrombocytopaenia birth trauma: cephalohaematoma congenital infections e.g. rubella

Answer 666

Accidental injury Non-accidental injury Coagulation disorders= haemophilia Thrombocytopaenia= - ITP - Thrombocytopaenia with Absent Radius (TAR) - congenital infection

Answer 667

Accidental injury Non-accidental injury Coagulation disorders= - haemophilia - von Willebrand's disease - liver disease Thrombocytopaenia= - ITP - ALL - meningococcal septicaemia - Thrombocytopaenia with Absent Radius (TAR) - congenital infection

Answer 668

bony prominences - shins - elbows - forehead

Answer 669

excessive multiple bruises of different ages bruise patterns which may indicate slapping, being gripped tightly (fingertip marks) or the use of inflicting instruments (e.g. belt)

Answer 670

sites which may raise concern include the face, ears, neck, buttocks, trunk or proximal parts of limbs

Answer 671

initially red then changes to purple, blue or black (over 1-3 days) later fades to yellow or green light bruises typically fade within 2 weeks, more severe bruising may take longer

Answer 672

immune-mediated reduced in platelet count

Answer 673

antibodies are directed against the glycoprotein IIb/IIa or Ib-V-IX complex example of type II hypersensitivity reaction

Answer 674

more acute in children and may follow an infection or vaccination

Answer 675

bruising petechial or purpuric rash bleeding is less common and typically presents as epistaxis or gingival bleeding

Answer 676

Immune (or idiopathic) thrombocytopenic purpura (ITP)

Answer 677

- FBC= isolated thrombocytopenia - blood film - if atypical features= bone marrow examination

Answer 678

isolated thrombocytopenia

Answer 679

only if atypical features eg: - lymph node enlargement/splenomegaly, high/low white cells - failure to resolve/respond to Tx

Answer 680

atypical so do bone marrow exam

Answer 681

- usually no Tx needed - avoid activities that may result in trauma eg. team sports - other options if platelet count very low (<10 *10^9) or significant bleeding

Answer 682

it resolves in around 80% of children within 6m with or without Tx

Answer 683

if the platelet count is very low (e.g. < 10 * 109/L) or there is significant bleeding.

Answer 684

oral/IV corticosteroid IV immunoglobulins platelet transfusions can be used in an emergency (e.g. active bleeding) but are only a temporary measure as they are soon destroyed by the circulating antibodies

Answer 685

they are soon destroyed by the circulating antibodies

Answer 686

Children's Act of 1989 and 2004

Answer 687

prematurity low birth weight disability chronic illness

Answer 688

personal history of child abuse teenage parents single parent substance abuse psychiatric disorder isolation large family size

Answer 689

cradle cap

Answer 690

quite common skin disorder seen in children typically affects the scalp (cradle cap), nappy area, face and limb flexures

Answer 691

cradle cap

Answer 692

cradle cap in 1st few weeks of life- early sign erythematous rash with coarse yellow scales

Answer 693

reassure it doesn't affect the baby and resolves within few weeks massage topical emollient onto scalp to loosen scales, brush gently with soft brush and wash off with shampoo if severe/persistent= topical imidazole cream

Answer 694

tends to resolve spontaneously by around 8 months of age.

Answer 695

1 in every 1000 babies

Answer 696

cleft lip and palate

Answer 697

a component of more than 200 birth defects

Answer 698

isolated cleft lip (15%) isolated cleft palate (40%) combined cleft lip and palate (45%)

Answer 699

maternal antiepileptic use

Answer 700

polygenic inheritance

Answer 701

cleft lip results from failure of the fronto-nasal and maxillary processes to fuse cleft palate results from failure of the palatine processes and the nasal septum to fuse

Answer 702

feeding: orthodontic devices may be helpful speech: with speech therapy 75% of children develop normal speech increased risk of otitis media for cleft palate babies

Answer 703

cleft lip is repaired earlier than cleft palate, with practices varying from repair in the first week of life to three months cleft palates are typically repaired between 6-12 months of age

Answer 704

typically affects 1-3% newborns the socket of the hip is too shallow and the femoral head is not held tightly in place, so the hip joint is loose. In severe cases, the femur can come out of the socket (dislocate).

Answer 705

- female (6x > boys) - breech presentation - FHx - 1st born - oligohydramnios - birth weight >5kg - congenital calcaneovalgus foot deformity

Answer 706

left 20% bilateral

Answer 707

- 1st degree FHx of hip problems in early life - breech at or after 36w no matter the presentation at birth or mode of devlivery - multiple pregnancy

Answer 708

all infants at newborn and 6w baby check using Barlow and Ortolani test certain babies need USS too

Answer 709

Barlow and Ortolani test at newborn and 6w check

Answer 710

attempts to dislocate an articulated femoral head screens for developmental dysplasia of the hip

Answer 711

attempts to relocate a dislocated femoral head screens for developmental dysplasia of the hip

Answer 712

- Barlow and Ortolani test - symmetry of leg length - level of knees when hips and knees are bilaterally flexed - restricted abduction of hip flexion

Answer 713

USS >4.5m= x-ray first line

Answer 714

- most unstable hips will spontaneously stabilise by 3-6w - Pavlik harness (dynamic flexion-abduction orthosis) in children <4-5m - older children may need surgery

Answer 715

failure of the neural plate to fuse normally during early development of the fetus

Answer 716

- spina bifida occulta - meningocele - myelomeningocele - anencephaly

Answer 717

Often an incidental finding May be overlying skin lesion such as tuft of hair, birth mark or sinus Involvement of the spinal cord may result in neurological defects

Answer 718

Spina bifida occulta (neural tube defect)

Answer 719

Cyst of meninges and cerebrospinal fluid No neurological involvement Good prognosis following surgical correction

Answer 720

Meningocele (neural tube defect)

Answer 721

Cyst of meninges and cerebrospinal fluid but also involving the spinal cord nerves Often results in bladder and bowel incontinence, paraparesis of the legs and hydrocephalus (associated Arnold-Chiari malformation)

Answer 722

Myelomeningocele (neural tube defect)

Answer 723

Absence of cranium and brain. Affected babies are stillborn.

Answer 724

Anencephaly (neural tube defect)

Answer 725

Intrauterine diagnosis= serum alpha-feto protein (AFP) at 15-17w may also be detected by routine USS at 18-20w

Answer 726

folic acid 0.4 mg per day from before conception and until the end of the first trimester if either partner has a neural tube defect, or if they have had a previous pregnancy with a neural tube defect then folic acid 5 mg per day should be used. The risk of recurrence in future pregnancies is around 1 in 50.

Answer 727

- either parent has neural tube defect - previous preg with neural tube defect - FHx neural tube defects - pts taking anti-epileptics - DM - coeliac disease (or other malabsorption states) - sickle cell anaemia

Answer 728

infectious disease typically presents in children 'cough of 100 days'

Answer 729

Gram-negative bacterium Bordetella pertussis

Answer 730

2, 3, 4 m and 3-5yrs pregnant women as newborns vulnerable

Answer 731

infection or immunisation does not result in lifelong protection so adolescents and adults may develop whooping cough even if they had routine vaccines

Answer 732

1) catarrrhal phase 2) paroxysmal phase 3) convalescent phase

Answer 733

whooping cough

Answer 734

symptoms similar to viral URTI, lasts around 1-2w

Answer 735

- cough increases in severity - coughing bouts worse at night and after feeding - cough may be ended by vomiting and central cyanosis - inspiratory whoop (not always present; caused by forced inspiration against closed glottis) - infants= may have spells of apnoea - persistent coughing -> subconjunctival haemorrhages or anoxia leading to syncope and seizures - lasts 2-8w

Answer 736

cough subsides over weeks to months

Answer 737

pt has acute cough 14d or more without another apparent cause, and one or more of the following features: - Paroxysmal cough. - Inspiratory whoop. - Post-tussive vomiting. - Undiagnosed apnoeic attacks in young infants.

Answer 738

- nasal swab for Bordetella pertussis= may take days-weeks to come back - PCR - serology= can be used if cough 21d+ but the others need to be within 21d

Answer 739

<6m= admit Notifiable disease - Oral macrolide eg. calrithromycin, azithro or erythro= if onset of cough within 21d (erraditate and reduce spread) - household contacts= Abx prophy - Abx does not seem to alter course of the illness

Answer 740

until 48hrs after starting Abx or 21d from onset of symptoms if no Abx

Answer 741

subconjunctival haemorrhage pneumonia bronchiectasis seizures

Answer 742

16-32w pregnant

Answer 743

resp secretions

Answer 744

if untreated then infectious 21d from onset of coughing

Answer 745

uncommon and low grade

Answer 746

whooping cough

Answer 747

adults and young infants (who may present with apnoea)

Answer 748

may be atypical, and is associated with high rates of hospitalization, severe illness, and death.

Answer 749

local Public Health England centre within 3 days

Answer 750

if the onset of the cough is within the previous 14 days or 21 days in the case of close contact of a group1 priority individual.

Answer 751

quite common and benign set of symptoms seen in young infants

Answer 752

bouts of XS crying and pulling-up of the legs, often worse in the evening

Answer 753

20% infants cause unknown

Answer 754

1) <5m when symptoms start and stop 2) recurrent & prolonged crying, fusing or irritability without obvious cause that can't be prevented or resolved 3) no evidence of faltering growth, fever or illness - most common in late afternoon/evening - drawing up knees to abdo or arching back when crying - clenching of fists

Answer 755

infantile colic

Answer 756

unknown may be due to a neurodevelopmental stage causing an exacerbation of normal infant crying due to abnormal gastrointestinal motility and gas production; reduced gut microbiome diversity; central nervous system deregulation; and psychosocial factors.

Answer 757

1st 6w of life equally in breastfed and bottle fed infants

Answer 758

parent or carer stress and sleep deprivation, fatigue, loss of confidence in parenting skills, anxiety or depression, premature cessation of breastfeeding, and increased risk of child maltreatment.

Answer 759

serial weight measurements; orofacial conditions that may affect feeding; muscle tone and neurological signs suggesting an underlying cause; and parent/carer interaction with the infant.

Answer 760

- advice about info and support - reassure- holding baby, gentle motion, white noise, winding techniques - encourage parent/carer to look after own wellbeing by resting when able, taking 'time out' if needed - continue breastfeeding where possible - use of simeticone or lactase drops NOT RECOMMENDED

Answer 761

Parents/carers feel unable to cope despite reassurance and advice in primary care. There is suspected faltering growth, or symptoms are severe, worsening, or persist beyond 5 months of age. There is a suspected underlying cause for symptoms which cannot be managed in primary care.

Answer 762

- ventricular septal defect (VSD)= most common, 30% - atrial septal defect (ASD) - patent ductus arteriosus (PDA) - coarctation of the aorta - aortic valve stenosis

Answer 763

VSD more common but in adults ASD more common new diagnosis as generally present later

Answer 764

- tetralogy of Fallot - transposition of the great arteries (TGA) - tricuspid atresia

Answer 765

Fallot more common

Answer 766

tetralogy of Fallot (presents 1-2m) but TGA most common lesion at birth

Answer 767

Fallot presents at 1-2m old TGA presents at birth

Answer 768

depends very much on the severity and any other coexistent defects.

Answer 769

common in hands and feet in 1st 24hrs of life, may occur when baby is crying or unwell from any cause

Answer 770

can be recognised clinically when the concentration of reduced haemoglobin in the blood exceeds 5g/dl

Answer 771

Nitrogen washout test (also known as the hyperoxia test) The infant is given 100% oxygen for ten minutes after which arterial blood gases are taken. A pO2 of less than 15 kPa indicates cyanotic congenital heart disease

Answer 772

supportive care prostaglandin E1 e.g. alprostadil

Answer 773

used to maintain a patent ductus arteriosus in ductal-dependent congenital heart defect this can act as a holding measure until a definite diagnosis is made and surgical correction performed

Answer 774

often seen in healthy newborns and refers to cyanosis around the mouth and the extremities such as the hands and feet. It is differentiated from other causes of peripheral cyanosis with significant pathology as it occurs immediately after birth in healthy infants. It is a common finding and may persist for 24 to 48 hours.

Answer 775

around 1-2m but may not be picked up until 6m

Answer 776

TOF is the result of anterior malalignment of the aorticopulmonary septum

Answer 777

- VSD - RV hypertrophy - RV outflow tract obstruction, pulmonary stenosis - overriding aorta

Answer 778

the severity of right ventricular outflow tract obstruction (pulmonary stenosis)

Answer 779

- the 4 characteristics - cyanosis= episodic hypercyanotic tet spells when infant upset, in pain or has fever - causes right-to-left shunt - ejection systolic murmur due to pulmonary stenosis (VSD doesn't usually casue a murmur) - right-sided arotic arch in 25%

Answer 780

CXR= 'boot-shaped' heart ECG= right ventricular hypertrophy

Answer 781

in unrepaired TOF due to near occlusion of the RV outflow tract (pulmonary stenosis) features= tachypnoea, severe cyanosis that may result in loss of consciousness typically when infant is upset, in pain or has a fever

Answer 782

?TOF tet spells

Answer 783

ejection systolic murmur due to pulmonary stenosis (the VSD doesn't usually cause a murmur)

Answer 784

surgical repair is often undertaken in two parts cyanotic episodes may be helped by beta-blockers to reduce infundibular spasm

Answer 785

form of cyanotic congenital heart disease

Answer 786

failure of the aorticopulmonary septum to spiral during septation

Answer 787

1) aorta leaves the right ventricle 2) pulmonary trunk leaves the left ventricle

Answer 788

- cyanosis - tachypnoea - loud single S2 - prominent right ventricular impulse

Answer 789

loud single S2 prominent right ventricular impulse

Answer 790

'egg-on-side' appearance

Answer 791

maintenance of the ductus arteriosus with prostaglandins surgical correction is the definite treatment.

Answer 792

'GCS = 13, M5 V4 E4 at 21:30'.

Answer 793

M6 V5 E4 (for both adults and children)

Answer 794

6. Infant moves spontaneously or purposefully 5. Infant withdraws from touch 4. Infant withdraws from pain 3. Abnormal flexion to pain for an infant (decorticate response) 2. Extension to pain (decerebrate response) 1. No motor response

Answer 795

5. Smiles, oriented to sounds, follows objects, interacts. 4. Cries but consolable, inappropriate interactions. 3. Inconsistently inconsolable, moaning. 2. Inconsolable, agitated. 1. No verbal response.

Answer 796

4. Eyes opening spontaneously 3. Eye opening to speech 2. Eye opening to pain 1. No eye opening or response

Answer 797

Patau (trisomy 13) Edward's (trisomy 18) Fragile X Noonan Pierre-Robin Prader-Willi William's Cri du chat (chromosome 5p deletion syndrome) Down's (trisomy 21)

Answer 798

Patau syndrome

Answer 799

Cri du chat syndrome

Answer 800

chromosome 5p deletion syndrome

Answer 801

Microcephalic, small eyes Cleft lip/palate Polydactyly Scalp lesions

Answer 802

Micrognathia Low-set ears Rocker bottom feet Overlapping of fingers

Answer 803

Learning difficulties Macrocephaly Long face Large ears Macro-orchidism

Answer 804

Webbed neck Pectus excavatum Short stature Pulmonary stenosis

Answer 805

Micrognathia Posterior displacement of the tongue (may result in upper airway obstruction) Cleft palate

Answer 806

Hypotonia Hypogonadism Obesity

Answer 807

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

Answer 808

Characteristic cry (hence the name) due to larynx and neurological problems Feeding difficulties and poor weight gain Learning difficulties Microcephaly and micrognathism Hypertelorism

Answer 809

Patau syndrome (trisomy 13)

Answer 810

Edward's (trisomy 18)

Answer 811

Noonan syndrome

Answer 812

Pierre-Robin syndrome

Answer 813

Prader-Willi syndrome

Answer 814

William's syndrome

Answer 815

Cri du chat syndrome (chromosome 5p deletion syndrome)

Answer 816

high pitch cry that sounds like a cat

Answer 817

Treacher-Collins syndrome. One of the key differences is that Treacher-Collins syndrome is autosomal dominant so there is usually a family history of similar problems

Answer 818

presence of only 1 sex chromosome (X) or deletion of the short arm of one of the X chromosomes

Answer 819

- short stature - shield chest, widely spaced nipples - webbed neck - primary amenorrhoea - bicuspid aortic valve (15%), coarctation of aorta (5-10%) - cystic hygroma (diagnosed prenatally) - high-arched palate - short fourth metacarpal - multiple pigmented naevi - lymphoedema in neonates (especially feet) - gonadotrophin levels will be elevated - hypothyroidism horseshoe kidney

Answer 820

autoimmune disease (esp autoimmune thyroiditis) and Crohns

Answer 821

bicuspid aortic valve (15%), coarctation of the aorta (5-10%) an increased risk of aortic dilatation and dissection are the most serious long-term health problems for women with Turner's syndrome regular monitoring in adult life for these complications is an important component of care

Answer 822

increased risk of aortic dilatation and dissection

Answer 823

cystic hygroma

Answer 824

lymphoedema in neonates (especially feet)

Answer 825

gonadotrophin levels will be elevated

Answer 826

Persistent oxygen saturation of less than 92% when breathing air. Grunting, marked chest recession, or a RR >60 Cyanosis (indicated by pale/mottled/ashen/blue skin, lips or tongue). Note: auscultation revealing absent breath sounds with a dull percussion note should raise the possibility of a pneumonia complicated by effusion and should trigger a referral to hospital. Child looks seriously unwell, does not wake, or if roused does not stay awake, or does not respond to normal social cues. A temperature of 38°C or higher in a child aged three months or less.

Answer 827

do not usually have pneumonia, and need not be treated with antibiotics, but should be reviewed if symptoms persist. A history of conjugate pneumococcal vaccination gives greater confidence to this decision.

Answer 828

amoxicillin 1st line 5 days or clarithromycin co-amox if penumonia associated with influenzae

Answer 829

3-6% all children usually in 1st 3m of life in formula fed infants and rarely seen in breastfed

Answer 830

both immediate (IgE mediated) and delayed (non-IgE mediated) seen CMP allergy= immediate reactions CMP intolerance= mild-moderate delayed reactions

Answer 831

- regurg and vomiting - diarrhoea - urticaria, atopic eczema - colic symptoms= irritability, crying - wheeze, chronic cough - rare= angioedema and anaphylaxis

Answer 832

often clinical= improvement with cow's milk protein elimination Ix= consider refer to specialist allergy clinic for skin prick/patch testing; total IgE and specific IgE (RAST) for cow's milk protein (if suspect IgE mediated allergy or faltering growth or significant atopic eczema with multiple or cross-reactive food allergies)

Answer 833

severe (failure to thrive)= refer to paeds Formula fed= extensive hydrolysed formula (eHF) milk 1st line; if severe then use amino acid-based formula (AAF) Breastfed= continue breastfeeding; eliminate cow's milk protein from maternal diet; consider Ca supplements to prevent deficiency; when breastfeeding stops use eHF until 12m and at least for 6m

Answer 834

CMPI usually resolves in most children in children with IgE mediated intolerance around 55% will be milk tolerant by the age of 5 years in children with non-IgE mediated intolerance most children will be milk tolerant by the age of 3 years a challenge is often performed in the hospital setting as anaphylaxis can occur.

Answer 835

L= inability to digest lactose (sugar in milk) due to low/absent lactase enzyme; non-immune mediated (enzyme def); bloating, diarrhoea, abdo pain; older children/adults; lifelong; Dx= lactose hydrogen breath test/lactose elimination; Mx= lactose free products CMPI/A= immune reaction to proteins in cows milk eg. whey, casein; immune mediated (IgE or non-IgE); vomiting, diarrhoea, skin rash, wheeze or anaphlyaxis; infants; Mx= specialised hypoallergenic formulas and avoid all cows milk & dairy; often resolves by early childhood

Answer 836

non-IgE mediated= by 3yrs IgE mediated= usually by 5yrs

Answer 837

reproducible immune-mediated allergic response to one or more proteins in cow's milk.

Answer 838

IgE-mediated food allergy produces immediate symptoms, which may affect multiple organ systems, typically up to 2 hours after cow's milk ingestion. Non-IgE-mediated food allergy reactions usually manifest between 2 and 72 hours after cow's milk ingestion. Mixed IgE and non-IgE allergic reactions involve both IgE and non-IgE mediated responses.

Answer 839

Parental/carer stress and reduced quality of life. Restricted diet and malnutrition. Development of atopic comorbidities. Severe and life-threatening reactions.

Answer 840

urticaria, angio-oedema, itch, cough, hoarseness, wheeze, or breathlessness after cow's milk ingestion.

Answer 841

one or more symptoms such as gastro-oesophageal reflux disease, abdominal discomfort, constipation, diarrhoea, or atopic eczema, particularly if symptoms are treatment-resistant.

Answer 842

2-4w then reintroduction of cows milk to confirm diagnosis if clear improvement

Answer 843

until child is 9-12m old and for at least 6m; then reintroduction using milk ladder if non-IgE mediated

Answer 844

oral food challenge under medical supervision is gold standard

Answer 845

planned home reintroduction or supervised challenge of cow's milk is necessary to determine if tolerance has been acquired. if current atopic eczema/immediate onset symptoms then refer to specialist for allergy testing and ongoing Mx if not then reintroduction using 'milk ladder'

Answer 846

reintroduces baked milk products first as heating reduces allergenicity. Once tolerance is established, greater exposure to less processed milk should be gradually encouraged, ending in the reintroduction of fresh cow's milk. The iMAP Milk Allergy Guideline Milk ladder and Milk ladder recipes leaflets may be helpful for parents/carers. have oral antihistamines available at home, in case there is a return of symptoms on reintroduction. If symptoms return on reintroduction of cow's milk, a cow's milk-free diet should be continued, and the child should be re-evaluated after a further 6 to 12 months.

Answer 847

IgE= rapid onset (within mins, up to 2hrs); acute angioeodema, acute urticaria, atopic eczema (acute flare), erythema, pruritus; colickly abdo pain, diarrhoea, nausea, vomiting; wheezing, SOB, cough, nasal itching, cogestion; signs of anaphylaxis non-IgE= delayed onset (2-72hrs); atopic eczema, erythema, pruritus; abdo pain; blood +/or mucus in stool; faltering growth; GORD, vomiting, colic, loose/frequent stools; pallor & tiredness, perianal redness; wheeze, SOB, cough

Answer 848

- trauma= renal tract, ureter, bladder - infection= TB - malignancy= RCC, urothelial (TCC), SCC, prostate ca, penile ca (SCC) - renal disease= golmerulonephritis - stones= microscopic haematuria - structural abnormalities= BPH, cystic renal lesions, vascular malformations, renal vein thrombosis due to RCC - coagulppathy - drugs - benign= exercise - gynae= endometriosis - iatrogenic= catheterisation, radiotherapy - pseudohamaturia

Answer 849

cause tubular necrosis or interstitial nephritis= aminoglycosides, chemotherapy interstitial nephiritis= penicillin, sulphonamides, NSAIDs anticoags

Answer 850

faltering growth

Answer 851

impairment of long term growth cognitive, immune, cardiac and GI impairment

Answer 852

weight loss in 1st few days of life and weight loss following the 1st few days of life

Answer 853

normal, usually stops by day 3-4 and by 3w should have regained their birthweight due to body fluid adjustment

Answer 854

by 3w of age

Answer 855

if lose >10% of birth weight in early days of life or fail to regain birth weight by 3w refer to paeds eg. illness, marked weight loss, failure to respons to feeding support

Answer 856

assessment of childs weight +/- height/length plotted on growth charts in red book

Answer 857

growth standards and UK birth and preterm growth statistics lines on the chart called centiles, which mark the weight or height below which a specified percentage of age and gender-matched children will fall. Eg 50% of children are below the 50% centile. Head circumference, weight, and height/length are plotted over time to visually represent growth. A healthy child will normally approximately follow the centile they are born on as they grow, but if a child fails to gain weight in this way they will begin to drop through the centiles.

Answer 858

A fall across 1 or more weight centile spaces, if birthweight was below the 9th centile. A fall across 2 or more weight centile spaces, if birthweight was between the 9th and 91st centiles. A fall across 3 or more weight centile spaces, if birthweight was above the 91st centile When current weight is below the 2nd centile for age, whatever the birthweight.

Answer 859

<2yrs= growth chart >2yrs= BMI

Answer 860

BMI is below the 2nd centile (can be due to undernutrition or a small build). BMI is below the 0.4th centile (suggests probable malnutrition).

Answer 861

their length (if <2 years old) or height (if >2 years old).

Answer 862

often multifactorial 1) inadequate intake of nutrients 2) inadequate absorption of nutrients 3) XS energy output due to an underlying condition

Answer 863

Decreased availability of nutritious food. Lack of knowledge of appropriate healthy food. Feeding issues, eg breastfeeding problems, cleft lip/palate, poor appetite and weaning struggles. Child maltreatment. Developmental delay. Eating disorders.

Answer 864

Troublesome vomiting, eg due to reflux, obstruction, side effects to medication, food sensitivities/allergy, underlying metabolic disease or central nervous system disease. Malabsorption due to bowel disease, eg chronic diarrhoea, food allergy/sensitivity, coeliac disease, inflammatory bowel disease, or protein-losing enteropathy.

Answer 865

Immunodeficiency such as severe combined immune deficiency (SCID). Genetic conditions such as Down's syndrome. Malignancies. Cardiac disease such as congenital heart disease. Respiratory disease such as cystic fibrosis or more commonly laryngomalacia. Endocrine disease such as diabetes or hyperthyroidism. Metabolic disease such as inborn errors in metabolism. Renal disease such as renal tubular acidosis.

Answer 866

prompt diagnosis and establishing underlying cause and initiating correct Tx quickly

Answer 867

assess the baby for signs or symptoms of an underlying disease causing faltering growth. Discuss or refer to paediatrics (urgency dependent on the individual situation) all babies with: - symptoms or signs suggestive of an acute or chronic underlying condition. - rapid weight loss or severe under-nutrition. - safeguarding concerns. - slow linear growth or unexplained short stature.

Answer 868

- feeding support eg. by midwife - direct observation and training eg. breastfeeding support workers - support to continue breastfeeding - advise to express milk to promote supply and give breast milk first before any formula top ups - follow up to review growth and weight (no more than once daily)

Answer 869

Weight should be measured again at appropriate intervals (no more than once daily if less than 1 month old) ' record all measurements in the parent or carer-held Personal Child Health Record. If an infant with faltering growth develops new clinical symptoms or signs after the initial assessment, develops marked weight loss or fails to respond to feeding support consider referral to paediatrics.

Answer 870

- refer to paeds if needed - eating solids= do they have interest in food, mealtime arrangements and practices eg. do family all eat together; what food is being offered and is it appropriate; any food aversion or avoidance; does caregiver respond to childs mealtime cues - encourage relaxed mealtimes not too short or long eg. 20-30mins - nutrient rich healthy food appropriate for developmental age, size of portion, texture - avoid too many high energy drinks eg. milk as can reduce appetitie - routine eg. 3 meals a day 2 snakcs - encourage child to explore food, feed themselves, be messy - refer depending on the individual situation eg a dietician, psychologist, occupational therapist, school nurse or social services.

Answer 871

monitor the child's growth but the frequency of this will be dependent on the individual clinical situation. Normally, no more than: Monthly from 1 year of age. Fortnightly if between 6-12 months. Weekly if between 1-6 months. Daily if less than 1 month old.

Answer 872

at least 4-8w

Answer 873

genetic congenital infections birth metabolic disorders?

Answer 874

fragile X Downs

Answer 875

cytomegalovirus toxoplasmosis rubella

Answer 876

hypoxia rhesus haemolytic disease intraventricular haemorrhage

Answer 877

PKU maple syrup urine disease homocystinuria

Answer 878

- transient synovitis - septic arthritis/osteomyelitis - juvenile idiopathic arthritis - trauma - developmental dysplasia of hip - perthes disease -slipped upper femoral epiphysis (SUFE)

Answer 879

transient synovitis

Answer 880

septic arthritis/osteomyelitis

Answer 881

juvenile idiopathic arthritis

Answer 882

developmental dysplasia of the hip

Answer 883

perthes disease

Answer 884

slipped upper femoral epiphysis

Answer 885

slipped upper femoral epiphysis

Answer 886

asymmetric gait, and is a deviation from a normal age-appropriate gait pattern. eg. antalgic gait

Answer 887

- pain waking child up at night= ?malignancy - red, swelling, stiffness= infection or inflam joint disease - weight loss, anorexia, fever, night sweats, fatigue= malignancy, infection, inflam - unexplained rash or bruising= haem or inflam joint disease; child maltreatment - limp and stiffness worse in the morning= inflam joint disease - unable to bear weight or painful limitation of range of motion= trauma or infection - severe pain, anxiety, agitation after traumatic injury= neurovasc compromise or impending compartment syndrome - palpable mass= malignancy or infection

Answer 888

- has fever/red flags - maltreatment - <3yrs - >9yrs with painful or restricted hip movements (in particular internal rotation)= exclude SUFE

Answer 889

transient synovitis is rare in this age group, septic arthritis more common

Answer 890

to exclude SUFE

Answer 891

rest and simple analgesia A&E if unable to weight bear, become unwell, develop fever if improving within 48hrs then review 1w from symptom onset to confirm complete symptom resolution

Answer 892

an X-ray should be arranged. A child with persistent limp and normal initial X-ray should be referred to paediatric orthopaedics or paediatric rheumatology department (the urgency depending on clinical judgement) for further investigation.

Answer 893

- fracture or soft tissue injury - DDH

Answer 894

- transient synovitis - fracture or soft tissue injury - Perthes disease (Legg-Calve-Perthes disease)

Answer 895

self-limiting inflammatory disorder of the hip. It is more common in boys than in girls and is rare in children aged younger than 3 years. It presents acutely with mild to moderate hip pain and limp, and there is no (or mild) restriction of hip movements, especially abduction and internal rotation. Children are otherwise well and afebrile. There is usually a history of viral illness, but the absence of a viral illness does not rule out the possibility of synovitis. The diagnosis of transient synovitis is one of exclusion, after other causes of hip pain and limp have been ruled out.

Answer 896

An idiopathic avascular necrosis of the developing femoral head. It is more common in boys than in girls. Onset is usually over weeks, and the child will typically present with limitation of hip rotation and a subacute limp sometimes with referred pain to the groin, thigh, or knee. It is typically unilateral, though bilateral involvement is present in 10% of cases. The child is systemically well with no other joint involvement and no evidence of joint inflammation. Most children with Perthes' disease have good outcomes, but long-term complications may include chronic pain and osteoarthritis.

Answer 897

perthes disease

Answer 898

- fracture or soft tissue injury - SUFE - perthes - Osgood-Schlatter disease - Sever's disease - Osteochondritis dissecans - Chondromalacia patellae

Answer 899

subtle undisplaced spiral fracture of the tibia typically seen in pre-school children. It is usually caused by a sudden twist, often after an unwitnessed fall. Affected toddlers (often new walkers) present with difficulty or refusal to bear weight.

Answer 900

A displacement of the proximal femoral epiphysis from the metaphysis. It is slightly more common in boys than girls and in children who are overweight. It can present with an acute/insiduous onset of pain (hip, thigh, or knee), and the child may walk with an antalgic gait out-toeing, with shortening of the affected limb. It is sometimes associated with endocrine abnormalities, such as hypothyroidism, and in children being treated for growth hormone deficiency or with a history of radiotherapy treatment. Prompt diagnosis is crucial to avoiding further displacement and the development of avascular necrosis.

Answer 901

An overuse injury caused by multiple small avulsion fractures within the ossification centre (apophysis) of the tibial tuberosity at the inferior attachment of the patellar ligament. It is a usually self-limiting disorder causing anterior knee pain during adolescence.

Answer 902

An overuse injury thought to be caused by repetitive microtrauma from the pull of the Achilles tendon on the unossified apophysis. It is most common in boys aged 10–12 years who are active in sports, such as running and football, and produces heel pain as a result of inflammation of the calcaneal apophysis. It often resolves within 2 weeks to 2 months, but a child may have recurrent symptoms until skeletal maturity.

Answer 903

Occurs when a small piece of subchondral bone begins to separate from its surrounding area due to a disturbance of the local blood supply. This bone and the cartilage covering it may break loose, causing pain and possibly hindering joint motion. It is the most common cause of a loose body in the joint space, typically affecting the knee between the ages of 10–19 years. The aetiology is uncertain but trauma, vascular abnormalities, defects in ossification, and genetics have all been suggested as possible causes. Clinical findings are subtle and a small effusion or limited range of joint movement may be the only sign. Locking or instability suggest a loose body in the joint.

Answer 904

Describes anterior knee pain typically felt when walking up or down stairs. Affects children between the ages of 10–19 years in a ratio of three girls to two boys.

Answer 905

- septic arthritis and osteomyelitis - other infections eg. discitis - malignancy= primary bone tumours, soft tissue sarcoma, leukaemia and lymphoma - Non-malignant haematological disease eg. sickle cell and haemophilia - metabolic disease eg. rickets - inflam muscle or joint disease eg. JIA and Lyme arthritis - neuromusc disease eg. cerebral palsy or spina bifida - muscular dystrophies - primary anatomical abnormality eg. limb length discrepancy - intra-abdo pathology eg. inguinal hernia and appendicitis; inguinoscrotal disorders eg. testicular torsion

Answer 906

Infection of the synovium and joint space. It can present in any joint but most commonly affects the lower limbs, and can lead to joint destruction, permanent loss of joint function, and sepsis. Clinical features include refusal to bear weight and fever. There may be evidence of joint inflammation.

Answer 907

infection of the bone. It usually involves a single bone but may rarely affect multiple sites, and can lead to inflammation and bone destruction. The most common presenting signs are pain with palpation and decreased limb use.

Answer 908

transient synovitis. However, transient synovitis is rare in children younger than 3 years of age.

Answer 909

1) fever 2) non-weight bearing on affected side 3) ESR >40 4) WCC >12,000

Answer 910

inflammation of the disc space and adjacent vertebral endplates that typically affects the lumbar spine. It is more common in neonates and young children. Presentation can be very non-specific, and a high index of suspicion is required for diagnosis. Clinical presentation ranges from refusal to walk or bear weight to irritability on spine immobility. Point tenderness over the spine can aid in the diagnosis. It may also present with unusual symptoms, such as abdominal pain, limp, hip or leg pain. Limited spinal flexion, stiff posture, and local tenderness on spine examination are also suggestive of discitis.

Answer 911

osteosarcoma, peaks after 10yrs

Answer 912

leukaemia and neuroblastoma

Answer 913

night pain, hepatosplenomegaly, lymphadenopathy, or pathological fractures.

Answer 914

Sickle cell disease encompasses a group of inherited conditions that have the inheritance of sickle haemoglobin in common. It can cause a limp through avascular necrosis of the hip, increased susceptibility to infections (including osteomyelitis), and stroke (about 10% of children with sickle cell disease are at risk of stroke, which may present as sudden onset of a limp)

Answer 915

Haemophilia may result in easy bruising, bleeding after dental procedures, haemarthrosis, soft-tissue/muscle haematomas in toddlers, and swollen joints in active toddlers (usually boys). May be related to family history of bleeding disorders.

Answer 916

Osteomalacia (called rickets in children) is due to a lack of vitamin D or problems with its metabolism. When osteomalacia affects the growing skeleton, it is referred to as rickets. Clinical features of rickets include bone pain and tenderness, skeletal deformity (such as bowing of the legs) and kyphoscoliosis, and proximal muscle weakness.

Answer 917

JIA encompasses a group of disorders presenting with inflammatory joint disease in children. It is characterized by joint pain, swelling without a large effusion, and morning stiffness that persists for longer than 6 weeks, and has no known cause. It can affect single or multiple joints. Systemic symptoms of lethargy and anorexia may be present. Eye involvement in the form of uveitis is also commonly noted. Often children with JIA are otherwise well and blood tests and X-rays may be normal.

Answer 918

second most common symptom to the classic rash associated with Lyme disease. In cases where the erythema migrans rash (commonly called the bull’s eye rash) does not appear or goes unnoticed, Lyme arthritis may be the first clinical sign. It typically presents without a fever. The child will bear partial weight and present with a limp.

Answer 919

degenerative condition affecting the hip joints of children, typically between the ages of 4-8 years.

Answer 920

due to avascular necrosis of the femoral head, specifically the femoral epiphysis. Impaired blood supply to the femoral head causes bone infarction

Answer 921

5x more in boys 10% cases are bilateral

Answer 922

hip pain: develops progressively over a few weeks limp stiffness and reduced range of hip movement

Answer 923

early changes include widening of joint space, later changes include decreased femoral head size/flattening

Answer 924

plain x-ray technetium bone scan or magnetic resonance imaging if normal x-ray and symptoms persist

Answer 925

osteoarthritis premature fusion of the growth plates

Answer 926

Catterall staging 1= Clinical and histological features only 2= Sclerosis with or without cystic changes and preservation of the articular surface 3= Loss of structural integrity of the femoral head 4= Loss of acetabular integrity

Answer 927

To keep the femoral head within the acetabulum: cast, braces If less than 6 years: observation Older: surgical management with moderate results Operate on severe deformities

Answer 928

Most cases will resolve with conservative management. Early diagnosis improves outcomes.

Answer 929

irritable hip. It generally presents as acute hip pain following a recent viral infection. It is the commonest cause of hip pain in children.

Answer 930

- acute hip pain following recent viral infection - limp/refusal to weight bear - groin or hip pain - low grade fever in minority; if high grade then ?septic arthritis

Answer 931

fever is a red flag, indicating the need for urgent specialist assessment children may be monitored in primary care (with a presumptive diagnosis of transient synovitis) 'If the child is aged 3-9 years, well, afebrile, mobile but limping, and has had the symptoms for less than 72 hours

Answer 932

self-limiting, rest and analgesia

Answer 933

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

Answer 934

common ALL (75%), CD10 present, pre-B phenotype T-cell ALL (20%) B-cell ALL (5%)

Answer 935

condition of enlarged lymph nodes, typically indicating an immune response to an infection or malignancy

Answer 936

The lymphatic system, comprising lymph vessels, nodes, and other tissues, plays a critical role in body's immunity. Lymph nodes are small, bean-shaped structures that produce and store cells that fight infection and disease. They filter the lymph fluid as it flows through them, trapping bacteria, viruses, and other foreign substances to be destroyed by white blood cells. Lymphadenopathy usually occurs when these nodes produce more cells or become filled with debris from a destroyed cell.

Answer 937

infective neoplastic= leukaemia and lymphoma autoimmune= SLE, RA graft versus host disease sarcoidosis drugs

Answer 938

infectious mononucleosis HIV, including seroconversion illness eczema with secondary infection rubella toxoplasmosis CMV tuberculosis roseola infantum

Answer 939

phenytoin and to a lesser extent allopurinol, isoniazid

Answer 940

leukaemia and lymphoma

Answer 941

the sudden, unexpected and unexplained death of an apparently healthy baby commonest cause of death in 1st yr of life

Answer 942

putting the baby to sleep prone parental smoking: increases the risk up to 5 fold prematurity: 4-fold increased risk bed sharing: odds ratio 5.1 hyperthermia (e.g. over-wrapping) or head covering (e.g. blanket accidentally moves) other= male; multiple births; social classes IV and V; maternal drug use; winter

Answer 943

- breastfeeding - room sharing (NOT bed sharing) - use of dummies

Answer 944

siblings should be screened for potential sepsis and inborn errors of metabolism.

Answer 945

genetic disorders caused by enzyme defects in metabolic pathways, leading to toxic metabolite accumulation or deficiency. eg... 1. Disorders of Carbohydrate Metabolism= Hereditary Fructose Intolerance: (Deficiency of aldolase B; triggers hypoglycemia and vomiting after fructose ingestion.) 2. Disorders of Amino Acid Metabolism= Maple Syrup Urine Disease (Defective metabolism of branched-chain amino acids; presents with lethargy and a sweet-smelling urine) and Phenylketonuria (PKU): (Deficiency of phenylalanine hydroxylase; causes intellectual disability if untreated.) many more Management involves dietary restrictions, supplements, and sometimes enzyme replacement. Early diagnosis via newborn screening is critical.

Answer 946

iron def anaemia 10% of children in the UK. The prevalence is higher in Asian, Afro-Caribbean and Chinese children

Answer 947

socioeconomic - iron supplemented milk formulas may be more expensive unmodified cow's milk - a poor source of iron due to it being in a form that is not absorbed well, therefore should be introduced after 1 year of age (breast milk is relatively low in iron but it is present in a form that is easily absorbed) ethnic origin - e.g. Asian mothers may introduce solids later

Answer 948

supplementary iron in milk dietary education free formulas for at risk infants

Answer 949

necrotizing enterocolitis increased mortality depends on the gestation respiratory distress syndrome intraventricular haemorrhage chronic lung disease, hypothermia, feeding problems, infection, jaundice retinopathy of prematurity hearing problems

Answer 950

important cause of visual impairment in babies born before 32 weeks gestation the cause is not fully understood and multivariate. One of the contributing factors is thought to be over oxygenation (e.g. during ventilation) resulting in a proliferation of retinal blood vessels (neovascularization) screening is done in at-risk groups

Answer 951

testicular growth at around 12 years of age (range = 10-15 years)

Answer 952

testicular volume >4ml

Answer 953

max height spurt at 14

Answer 954

12yrs range 10-15yrs

Answer 955

11.5yrs range= 9-13yrs

Answer 956

breast development at around 11.5 years of age (range = 9-13 years)

Answer 957

height spurt reaches its maximum early in puberty (at 12) , before menarche increase of only about 4% of height following menarche

Answer 958

13yrs (11-15yrs)

Answer 959

gynaecomastia may develop in boys asymmetrical breast growth may occur in girls diffuse enlargement of the thyroid gland may be seen

Answer 960

This condition is related, but distinct from classic testicular torsion. It classically occurs in boys aged 8-11 years.

Answer 961

small vestigial remnants: - appendix testis: a small structure on the anterosuperior aspect of the testis (an embryologic remnant of the Mullerian duct system) - appendix epididymis: a small remnant of the Wolffian duct located at the head of the epididymis

Answer 962

similar, but often less severe symptoms to testicular torsion, i.e.: sudden-onset testicular pain the testicular body itself is usually non-tender but there is a tender mass palpable a 'blue-dot' sign may be seen through the scrotum

Answer 963

Torsion of the appendix testis or appendix epididymis

Answer 964

usually clinical although Doppler ultrasound scans may be helpful

Answer 965

conservative treatment is possible, but many children will have an exploratory operation to exclude classic testicular torsion, given the consequences of a missed diagnosis.

Answer 966

1) testicular torsion= most common around puberty 2) irreducible inguinal hernia= most common in children <2yrs 3) epididymitis= rare in prepubescent children

Answer 967

38°C or higher

Answer 968

traffic light system

Answer 969

emergency ambulance to A&E

Answer 970

urgent face-toface assessment within 2hrs

Answer 971

face to face assessment (urgency depending on clinical judgement

Answer 972

child can be managed at home

Answer 973

- look for signs of dehydration - regular fluids - continue breastfeeding - do not underdress or over wrap to prevent overcooling or overheating - avoid using cool water sponging to lower temp - check child regularly incl during the night - keep away from nursery/school until recovered

Answer 974

paracetamol or ibuprofen initially and consider to switching to ibuprofen is paracetamol alone is ineffective and vise vera

Answer 975

- non-blanching rash/signs of CNS infection - seizure - dehydrated - fever >5d - becomes more unwell - distressed that unable to look after child at home

Answer 976

- traffic light system - appearance, temp, HR, RR, CRT, fluid status - BP if HR or CRT abnormal - height and duration of fever, method of measurement, associated symptoms eg. cough, pain, SOB, itch, rash, confusion, swelling, N&V - any perinatal Cx= maternal fever, prematurity (these are RFs for serious bacterial infection in <3m) - ?medical conditions eg. immunosuprression/def - recent med use - foreign travel

Answer 977

<4w= electronic thermometer in axilla 4w-5yrs= electronic thermometer or chemical dot thermometer in the axilla, or an infra-red tympanic thermometer.

Answer 978

if they have ANY of the relevant clinical features (only need 1 to be in that category)

Answer 979

1) Colour (skin, lips, tongue) 2) Activity 3) Respiratory 4) Circulation and hydration 50 Other

Answer 980

1) Colour= normal 2) Activity= responds normally to social cues; content or smiles; stays awake or awakens quickly; strong normal cry or not crying 3) Resp= / 4) Circulation and hydration= normal skin and eyes; moist mucus membranes 5) Other= none of amber or red signs or symptoms

Answer 981

1) Colour= pallor reported by parent/carer 2) Activity= does not respond normally to social cues; no smile; wakes only with prolonged stimulation; decreased activity 3) Resp= nasal flaring; tachypnoea: 6-12m with RR >50; >12m with RR >40; O2 sats ≤ 95% on air; crackles on auscultation 4) C&H= tachycardia; <12m and >160bpm; 12-24m and >150bpm; 2-5yrs and >140 bpm; poor feeding in infants; dry mucous membranes; CRT 3secs or more; reduced urine output (wet nappies in infants) 5) T ≥ 39°C if 3-6m; fever 5d+; rigors; swelling of limb or joint; non-weight bearing limb or not using an extremity

Answer 982

1) Colour= pale, mottled, ashen or blue 2) Activity= no response to social cues; appears ill to healthcare professional; does not wake or if roused does not stay awake; weak, high-pitched or continuous crying 3) Resp= grunting; tachypnoea: RR 60 or more; moderate or severe chest indrawing 4) C&H= reduced skin turgor 5) T ≥ 38°C if <3m; non-blanching rash; bulging fontanelle; neck stiffness; focal neuro signs; focal seizures; status epilepticus

Answer 983

- meningococcal disease - bacterial meningitis - herpes simplex encephalitis - pneumonia - UTI - septic arthritis or osteomyelitis - Kawasaki disease

Answer 984

dose every 4-6hrs if needed (max 4 doses in 24hrs) 1-2m= 30-60mg (every 8hrs, max 60mg/kg per day) 2-5m= 60mg 6-23m= 120mg 2-3yrs= 180mg 4-5yrs= 240mg 6-7= 240-250mg 8-9= 360-375mg 10-11= 480-500mg 12-15= 480-750mg 16yrs+= 500mg-1000mg (1g) (oral)

Answer 985

1-2m= 5 mg/kg 3–4 times a day (off-label indication under 3 months of age or body weight less than 5 kg) 3-5m= 50mg 3xd 6-11m= 50mg 3-4x a day 1-3yrs= 100mg 3xd 4-6yrs= 150mg 3xd 7-9= 200mg 3xd 10-11yrs= 300mg 3xd 12-17yrs= 300-400 3-4x d

Answer 986

1) T 2) HR 3) RR 4) CRT Also look for signs of dehydration eg. reduced skin turgor and cool extremities

Answer 987

Child can be managed at home with appropriate care advice, including when to seek further help

Answer 988

safety net or refer to a paediatric specialist for further assessment a safety net includes verbal or written information on warning symptoms and how further healthcare can be accessed, a follow-up appointment, liaison with other healthcare professionals, e.g. out-of-hours providers, for further follow-up

Answer 989

refer child urgently to a paediatric specialist

Answer 990

1) oral Abx should not be prescribed to children with fever without apparent source (unless ?sepsis for example) 2) if suspect pneumonia but child not going to hospital then do not routinely need CXR

Answer 991

bedwetting

Answer 992

'involuntary discharge of urine by day or night or both, in a child aged 5 years or older, in the absence of congenital or acquired defects of the nervous system or urinary tract'

Answer 993

either primary (the child has never achieved continence) or secondary (the child has been dry for at least 6 months before)

Answer 994

constipation, DM, UTI if recent onset

Answer 995

1) look for underlying cause/trigger 2) general advice= fluid intake; toilet patterns (encourage empty bladder regularly during day and before sleep); lifting and waking 3) Reward system eg. star charts= give for agreed behaviour rather than dry nights eg. using toilet to pass urine before sleep 4) Enuresis alarm (have sensor pads that sense wetness; high success rate) 5) Desmopressin= short term eg. sleepovers or if alarm ineffective

Answer 996

desmopressin

Answer 997

'should be given for agreed behaviour rather than dry nights' e.g. Using the toilet to pass urine before sleep

Answer 998

primary bedwetting without daytime symptoms primary bedwetting with daytime symptoms secondary bedwetting

Answer 999

never achieved sustained continence at night and does not have daytime symptoms. This is thought to be caused by sleep arousal difficulties, polyuria, and/or bladder dysfunction.

Answer 1000

ever achieved sustained continence at night and has daytime symptoms, such as wetting, urinary frequency, or urgency. This may be caused by an overactive bladder, congenital malformations, neurological disorders, urinary tract infection (UTI), or chronic constipation.

Answer 1001

bedwetting occurs after the child or young person has been previously dry at night for more than 6 months. This is usually due to an underlying cause, such as diabetes, UTI, constipation, psychological problems (for example behavioural or emotional problems), or family problems (vulnerable child or family).

Answer 1002

- FHx - m>f (2:1) - delay in attaining bladder control - obesity - psycho or behavioural disorders eg. ADHD, autism, anxiety, depressive and conduct disorders

Answer 1003

There are any daytime symptoms. The child has previously been dry at night without assistance for 6 months.

Answer 1004

1) advice= reward system, toilet patterns, fluid intake 2) <5yrs= reassure that many children that age wet bed and normally resolves without Tx 3) 5yrs+= if <2x a week then reassure and wait-and-see; if Tx needed then positive reward system + enuresis alarm; may need desmopressin

Answer 1005

- refer to enuresis clinic or secondary care for Ix - >2yr and despite awareness of toileting needs and showing appropriate toileting behaviour, are struggling to not wet themselves during the day as well as the night= assessment and Ix for specific medical problem

Answer 1006

manage underlying cause or arrange referral to paeds/enuresis clinic if can't be identified or managed in primary care

Answer 1007

all infants (aged 0 to 12 months) living in areas of the UK where the annual incidence of TB is 40/100,000 or greater all infants (aged 0 to 12 months) with a parent or grandparent who was born in a country where the annual incidence of TB is 40/100,000 or greater. The same applies to older children but if they are 6 years old or older they require a tuberculin skin test first previously unvaccinated tuberculin-negative contacts of cases of respiratory TB previously unvaccinated, tuberculin-negative new entrants under 16 years of age who were born in or who have lived for a prolonged period (at least three months) in a country with an annual TB incidence of 40/100,000 or greater healthcare workers prison staff staff of care home for the elderly those who work with homeless people

Answer 1008

Bacille Calmette-Guerin (BCG) to protect against TB

Answer 1009

to high-risk infants

Answer 1010

live attenuated Mycobacterium bovis. Protects against TB. It also offers limited protection against leprosy.

Answer 1011

any person being considered for the BCG vaccine must first be given a tuberculin skin test. The only exceptions are children < 6 years old who have had no contact with tuberculosis given intradermally, normally to the lateral aspect of the left upper arm

Answer 1012

yes but not administered simultaneously there should be a 4 week interval

Answer 1013

previous BCG vaccination a past history of tuberculosis HIV pregnancy positive tuberculin test (Heaf or Mantoux)

Answer 1014

over the age of 35, as there is no evidence that it works for people of this age group.

Answer 1015

keratinocytes of the skin and mucous membranes and it is carcinogenic.

Answer 1016

hyman papullomavirus

Answer 1017

6 & 11= genital warts 16 & 18= linked to cervical ca and other ca

Answer 1018

>99.7% cervical ca (HPV testing integral to cervical ca screening; samples first tested for HPV and only if they are +ve then cytology is performed) 85% anal ca 50% vulval and vaginal ca 20-30% mouth and throat ca

Answer 1019

HPV smoking COCP high parity

Answer 1020

16 & 18 (oncogenic) and the strains that cause genital warts (6 & 11)

Answer 1021

12-13 years olds, both girls and boys also offered to gay, bisexual, and other men who have sex with men (GBMSM) to protect against anal, throat and penile cancers.

Answer 1022

All 12 and 13 yr old GIRLS AND BOYS in year 8 - given at school - one dose

Answer 1023

eligible GBMSM under the age of 25 also receive 1-dose, offered through sexual health clinics eligible GBMSM aged 25 to 45 years receive a 2-dose schedule, offered through sexual health clinics eligible individuals who are immunosuppressed or those known to be HIV-positive receive a 3-dose schedule

Answer 1024

- confirmed anaphylactic reaction to previous dose of vaccine containing same antigens - confirmed anaphylactic reaction to another component contained in the relevant vaccine eg. egg protein

Answer 1025

febrile illness/intercurrent infection

Answer 1026

pregnancy immunosuppression

Answer 1027

an evolving or unstable neuro condition

Answer 1028

asthma or eczema history of seizures (if associated with fever then advice should be given regarding antipyretics) breastfed child previous history of natural pertussis, measles, mumps or rubella infection history of neonatal jaundice family history of autism neurological conditions such as Down's or cerebral palsy low birth weight or prematurity patients on replacement steroids e.g. (CAH)

Answer 1029

At birth= BCG if RFs 2m= 6 in 1; oral rotavirus; Men B 3m= 6 in 1; oral rotavirus; PCV 4m= 6 in 1; Men B 12-13m= Hib/MenC; MMR; PCV; Men B 2-8yrs= annual flu 3-4yrs= 4 in 1 pre-school booster; MMR 12-13yrs= HPV 14yrs (Year 9)= 3 in 1 teenage booster; Men ACWY New uni students (up to 25yrs)= Men ACWY

Answer 1030

if baby deemed at risk of TB eg. TB in family in past 6m

Answer 1031

Diphtheria Tetanus Whooping cough Polio Hib Hep B

Answer 1032

measles mumps rubella

Answer 1033

diphtheria tetanus whooping cough polio

Answer 1034

Tetanus Diphtheria Polio

Answer 1035

2m, 3m, 4m

Answer 1036

at birth if RFs for TB

Answer 1037

12-13m (Hib also in 6 in 1)

Answer 1038

12-13m 2-4yrs

Answer 1039

2-8yrs annual

Answer 1040

13-18yrs ( 14yrs in Year 9 normally)

Answer 1041

13-18yrs New uni students (up to 25yrs)

Answer 1042

GP practices will automatically send letters inviting 17-and 18-year-olds in school year 13 to have the Men ACWY vaccine. Students going to university or college for the first time as freshers, including overseas and mature students up to the age of 25, should contact their GP to have the Men ACWY vaccine, ideally before the start of the academic year'

Answer 1043

Haemophilus influenzae B vaccine

Answer 1044

Pneumococcal Conjugate Vaccine

Answer 1045

Meningococcal B vaccine

Answer 1046

Meningococcal C vaccine

Answer 1047

Meningococcal vaccine covering A, C, W and Y serotypes

Answer 1048

Human Papilloma Vaccine

Answer 1049

H. influenzae type b and meningococcal group C (Hib/MenC) booster.

Answer 1050

At 2–11 years of age = each year from September: Live attenuated influenza vaccine (LAIV — Fluenz Tetra®). If LAIV is unsuitable and the child is in a clinical risk group, use inactivated flu vaccine.

Answer 1051

Born to a mother or living with parents or close household contacts known to have hepatitis B. Whose parents are people who inject drugs. Who receive regular blood transfusions (for example for haemophilia). In residential or day care for people with severe learning disability (decisions on immunization should be made on the basis of a local risk assessment). Who are at high risk of requiring medical or dental procedures when travelling to visit relatives in countries where there is a moderate or high prevalence of hepatitis B.

Answer 1052

vaccination is recommended for non-immune children who are close contacts of people who are immunocompromised (for example they have a sibling or parent with leukaemia or undergoing chemotherapy). For children aged 1 year or older, give two doses of varicella vaccine (Varivax® or Varilrix®) spaced 4–8 weeks apart. The varicella vaccine and the measles, mumps, and rubella (MMR) vaccine should be spaced 4 weeks apart if they are not administered on the same day.

Answer 1053

MenACWY, MenB, PCV13 or PPV23, annual flu vaccine

Answer 1054

PCV13 or PPV23

Answer 1055

PCV13 or PPV23 Annual flu

Answer 1056

PCV13 or PPV23 Annual flu

Answer 1057

PCV13 or PPV23 Annual flu

Answer 1058

PCV13 or PPV23 Annual flu Hep B

Answer 1059

PCV13 or PPV23 Annual flu Hep A & B

Answer 1060

Hep A and B

Answer 1061

PCV13 or PPV23 Annual flu

Answer 1062

PCV13 or PPV23 Annual flu MenACWY Men B

Answer 1063

Pneumococcal conjugate vaccine (PCV13) can be administered to children aged up to 10 years. Pneumococcal polysaccharide vaccine (PPV23) can be administered to children aged 2 years and over.

Answer 1064

severe immunosuppression allergy to neomycin children who have received another live vaccine by injection within 4 weeks pregnancy should be avoided for at least 1 month following vaccination immunoglobulin therapy within the past 3 months (there may be no immune response to the measles vaccine if antibodies are present)

Answer 1065

malaise, fever and rash may occur after the first dose of MMR. This typically occurs after 5-10 days and lasts around 2-3 days

Answer 1066

oral, live attenuated vaccine

Answer 1067

2 doses are required, the first at 2 months, the second at 3 months the first dose should not be given after 14 weeks + 6 days and the second dose cannot be given after 23 weeks + 6 days due to a theoretical risk of intussusception

Answer 1068

around 85-90% effective and is predicted to decrease hospitalisation by 70% offers long-term protection against rotavirus

Answer 1069

a cell-free purified toxin that is normally given as part of a combined vaccine.

Answer 1070

5 doses in total: 2m 3m 4m 3-5yrs 13-18yrs

Answer 1071

- clean wound - tetanus prone wound - high-risk tetanus prone wound

Answer 1072

1) classify wound eg. clean or tetanus prone 2) ? tetanus vaccine +/- tetanus immunoglobulin

Answer 1073

Wounds less than 6 hours old, non-penetrating with negligible tissue damage

Answer 1074

puncture-type injuries acquired in a contaminated environment e.g. gardening injuries wounds containing foreign bodies compound fractures wounds or burns with systemic sepsis certain animal bites and scratches

Answer 1075

heavy contamination with material likely to contain tetanus spores e.g. soil, manure wounds or burns that show extensive devitalised tissue wounds or burns that require surgical intervention

Answer 1076

no vaccine nor tetanus immunoglobulin is required, regardless of the wound severity

Answer 1077

if tetanus prone wound: reinforcing dose of vaccine high-risk wounds (e.g. compound fractures, delayed surgical intervention, significant degree of devitalised tissue): reinforcing dose of vaccine + tetanus immunoglobulin

Answer 1078

reinforcing dose of vaccine, regardless of the wound severity for tetanus prone and high-risk wounds: reinforcing dose of vaccine + tetanus immunoglobulin

Answer 1079

utilise a weakened form of the pathogen to stimulate an immune response akin to a natural infection without causing the disease

Answer 1080

immunocompromised due to live nature of the vaccines

Answer 1081

BCG measles, mumps, rubella (MMR) influenza (intranasal) oral rotavirus oral polio yellow fever oral typhoid

Answer 1082

- live attenuated - Inactivated preparations - Toxoid (inactivated toxin) - Subunit and Conjugate vaccines - Messenger RNA (mRNA) vaccines - Viral vector vaccines - can also be monovalent (specific to one antigen/strain/subtype of pathogen) or multivalent/polyvalent (various)

Answer 1083

as may pose a risk to immunocompromised pts

Answer 1084

employ pathogens that have been killed, often by heat or chemicals, to elicit an immune response might require booster doses to maintain immunity as the induced immune response is generally weaker than live attenuated vaccines

Answer 1085

inactivated preparations= to maintain immunity as the induced immune response is generally weaker than live attenuated vaccines also toxiod

Answer 1086

rabies hep A influenza (IM)

Answer 1087

these vaccines are designed around the toxins produced by certain bacteria. The toxins are rendered harmless (detoxified) and used as antigens in the vaccine. When the immune system encounters these non-toxic toxoids, it learns to combat the natural toxins the bacteria produce, providing immunity against the toxic effects of infections toxoid vaccines often require booster doses to maintain immunity since, like inactivated vaccines, the immune response they induce can wane over time

Answer 1088

tetanus diphtheria pertussis (whooping cough)

Answer 1089

subunit and conjugate vaccines are often grouped together. Subunit means that only part of the pathogen is used to generate an immunogenic response. A conjugate vaccine is a particular type that links the poorly immunogenic bacterial polysaccharide outer coats to proteins to make them more immunogenic

Answer 1090

pneumococcus (conjugate) haemophilus (conjugate) meningococcus (conjugate) hepatitis B human papillomavirus

Answer 1091

introduce a piece of mRNA into cells which then produce a protein to trigger an immune response.

Answer 1092

some COVID-19 vaccines

Answer 1093

use a harmless virus (different from the target pathogen) to deliver critical parts of the target pathogen to stimulate an immune response can be produced more rapidly in response to emerging threats

Answer 1094

some Ebola and COVID-19 vaccines

Answer 1095

different types are available, including whole inactivated virus, split virion (virus particles disrupted by detergent treatment) and sub-unit (mainly haemagglutinin and neuraminidase)

Answer 1096

contains inactivated Inaba and Ogawa strains of Vibrio cholerae together with recombinant B-subunit of the cholera toxin

Answer 1097

contains HBsAg adsorbed onto aluminium hydroxide adjuvant and is prepared from yeast cells using recombinant DNA technology

Answer 1098

'valency' in vaccinology denotes the number of distinct antigenic components or serotypes a vaccine can protect against. Essentially, it specifies the extent of a vaccine's antigenic reach.

Answer 1099

contain a singular antigenic component, conferring immunity against one strain or subtype of a pathogen

Answer 1100

measles vaccine

Answer 1101

these vaccines comprise multiple antigenic components, safeguarding against various strains or subtypes of a pathogen or, occasionally, multiple pathogens

Answer 1102

quadrivalent influenza vaccine, protective against four distinct virus strains

Answer 1103

- >65yrs - chronic respiratory disease (including asthmatics who use inhaled steroids) - chronic heart disease (HF, IHD, including HTN if associated with cardiac Cx) - CKD (at stages 3, 4 or 5, chronic kidney failure, nephrotic syndrome, kidney transplantation) - chronic liver disease: cirrhosis, biliary atresia, chronic hepatitis - chronic neurological disease: (e.g. Stroke/TIAs) - DM (including diet controlled) - immunosuppression due to disease or treatment (e.g. HIV) - asplenia or splenic dysfunction - pregnant women - health and social care staff directly involved in patient care (e.g. NHS staff) - those living in long-stay residential care homes - carers of the elderly or disabled person whose welfare may be at risk if the carer becomes ill (at the GP's discretion)

Answer 1104

- >65yrs - asplenia or splenic dysfunction - chronic respiratory disease: COPD, bronchiectasis, CF, interstitial lung disease. Asthma is only included if 'it requires the use of oral steroids at a dose sufficient to act as a significant immunosuppressant' - chronic heart disease: IHD if requiring medication or follow-up, HF, congenital heart disease. Controlled HTN is not an indication for vaccination - CKD (at stages 4 and 5, nephrotic syndrome, kidney transplantation) - chronic liver disease: including cirrhosis and chronic hepatitis - DM if requiring medication - immunosuppression (either due to disease or treatment). This includes patients with any stage of HIV infection - cochlear implants - patients with CSF leaks

Answer 1105

autosomal dominant disorder associated with short stature. Results in abnormal cartilage

Answer 1106

autosomal dominant Caused by a mutation in the fibroblast growth factor receptor 3 (FGFR-3) gene

Answer 1107

abnormal cartilage giving rise to: - short limbs (rhizomelia) with shortened fingers (brachydactyly) - large head with frontal bossing and narrow foramen magnum - midface hypoplasia with a flattened nasal bridge - 'trident' hands - lumbar lordosis

Answer 1108

- 70% sporadic mutation - advancing parental age at time of conception - once present it is inherited in autosomal dominant fashion

Answer 1109

- no specific - limb lengthening procedures= application of Ilizarov frames and targeted bone fracture

Answer 1110

initially gonads in fetus are undifferentiated on the Y chromosome there is a sex-determining gene (SRY gene) which causes differentiation of the gonad into a testis if absent (i.e. in a female) then the gonads differentiate to become ovaries

Answer 1111

congenital adrenal hyperplasia true hermaphroditism maternal ingestion of androgens

Answer 1112

congenital adrenal hyperplasia

Answer 1113

assesses health of newborn baby

Answer 1114

1 and 5mins of age if score is low then repeated at 10mins

Answer 1115

Appearance Pulse Grimace (reflex irritability) Activity (muscle tone) Respiratory effort

Answer 1116

>100= 2 <100= 1 absent= 0

Answer 1117

strong crying= 2 weak, irregular= 1 nil= 0

Answer 1118

pink= 2 body pink, extremities blue= 1 blue all over= 0

Answer 1119

active movement= 2 limb flexion= 1 flaccid= 0

Answer 1120

cries on stimulation/sneezes, coughs= 2 grimace= 1 nil= 0

Answer 1121

0-3 is very low 4-6 moderate low 7-10 means baby is in good state

Answer 1122

both homozygotes and heterozygotes manifest disease (there is no carrier state) both males and females affected

Answer 1123

passed on to 50% of children only affected individuals can pass on disease normally appears in every generation risk remains the same for each successive pregnancy

Answer 1124

non-penetrance: lack of clinical signs and symptoms (normal phenotype) despite abnormal gene. E.g. 40% otosclerosis spontaneous mutation: new mutation in one of gametes e.g. 80% of individuals with achondroplasia have unaffected parents

Answer 1125

Recessive= metabolic Dominant= structural some exceptions

Answer 1126

some 'metabolic' conditions such as Hunter's and G6PD are X-linked recessive whilst others such as hyperlipidaemia type II and hypokalaemic periodic paralysis are autosomal dominant some 'structural' conditions such as ataxia telangiectasia and Friedreich's ataxia are autosomal recessive

Answer 1127

Achondroplasia Acute intermittent porphyria Adult polycystic disease Antithrombin III deficiency Ehlers-Danlos syndrome Familial adenomatous polyposis Hereditary haemorrhagic telangiectasia Hereditary spherocytosis Hereditary non-polyposis colorectal carcinoma Huntington's disease Hyperlipidaemia type II Hypokalaemic periodic paralysis Malignant hyperthermia Marfan's syndromes Myotonic dystrophy Neurofibromatosis Noonan syndrome Osteogenesis imperfecta Peutz-Jeghers syndrome Retinoblastoma Romano-Ward syndrome tuberous sclerosis Von Hippel-Lindau syndrome Von Willebrand's disease (type 3-most severe is recessive)

Answer 1128

dominant (80% type 1) type 3 most severe form is recessive

Answer 1129

only homozygotes are affected males and females are equally likely to be affected not manifest in every generation - may 'skip a generation'

Answer 1130

25% chance of having an affected (homozygote) child 50% chance of having a carrier (heterozygote) child 25% chance of having an unaffected (i.e. genotypical) child

Answer 1131

all children will be carriers

Answer 1132

Autosomal recessive disorders are often metabolic in nature and are generally more life-threatening compared to autosomal dominant conditions

Answer 1133

Albinism Ataxic telangiectasia Congenital adrenal hyperplasia Cystic fibrosis Cystinuria Familial Mediterranean Fever Fanconi anaemia Friedreich's ataxia Gilbert's syndrome Glycogen storage disease Haemochromatosis Homocystinuria Lipid storage disease: Tay-Sach's, Gaucher, Niemann-Pick Mucopolysaccharidoses: Hurler's PKU Sickle cell anaemia Thalassaemias Wilson's disease

Answer 1134

recessive (some say dominant)

Answer 1135

90% dominant

Answer 1136

form of childhood epilepsy that typically occurs between the age of 4 and 12 years.

Answer 1137

seizures characteristically occur at night seizures are typically partial (e.g. paraesthesia affecting the face) but secondary generalisation may occur (i.e. parents may only report tonic-clonic movements) the child is otherwise normal

Answer 1138

centrotemporal spikes

Answer 1139

?benign rolandic epilepsy

Answer 1140

excellent, stops by adolescence

Answer 1141

benign rolandic epilepsy

Answer 1142

oedema of scalp at presenting part of the head, typically vertex

Answer 1143

may be due to mechanical trauma of the initial portion of the scalp pushing through the cervix in a prolonged delivery or secondary to the use of ventouse (vacuum) delivery.

Answer 1144

soft puffy swelling due to localised oedema crosses suture lines

Answer 1145

none needed usually resolves in few days

Answer 1146

Caput succedaneum (C.S= crosses suture lines)

Answer 1147

CS= present at birth; typically forms over vertex and crosses suture lines; resolves within days CH= typically develops several hrs after birth; most commonly in parietal region; doesn't cross suture lines; may take months to resolve Both= swelling on head of newborn, more common following prolonged difficult deliveries; Mx conservative

Answer 1148

swelling on newborns head

Answer 1149

develops several hrs after delivery and is due to bleeding between the periosteum and skull

Answer 1150

parietal region

Answer 1151

none may take 3m to resolve

Answer 1152

1) antenatal 2) newborn 3) 1st month 4) following months 5) pre school 6) ongoing

Answer 1153

Ensure intrauterine growth Check for maternal infections e.g. HIV Ultrasound scan for fetal abnormalities Blood tests for Neural Tube Defects

Answer 1154

Clinical examination of newborn Newborn Hearing Screening Programme e.g. oto-acoustic emissions test Give mother Personal Child Health Record

Answer 1155

Heel-prick test day 5-9 - hypothyroidism, PKU, metabolic diseases, cystic fibrosis, medium-chain acyl Co-A dehydrogenase deficiency (MCADD) Midwife visit up to 4 weeks (usually health visitor takes over at 2w)

Answer 1156

Health visitor input GP examination at 6-8 weeks Routine immunisations

Answer 1157

National orthoptist-led programme for pre-school vision screening to be introduced

Answer 1158

Monitoring of growth, vision, hearing Health professionals advice on immunisations, diet, accident prevention

Answer 1159

<3yrs following intro of cereals (i.e gluten) into diet

Answer 1160

failure to thrive diarrhoea abdominal distension older children may present with anaemia many cases are not diagnosed to adulthood

Answer 1161

jejunal biopsy showing subtotal villous atrophy IgA Tissue Transglutaminase antibodies (tTG)

Answer 1162

herniation of abdominal viscera into the chest cavity due to incomplete formation of the diaphragm. This can result in pulmonary hypoplasia and hypertension which causes respiratory distress shortly after birth.

Answer 1163

usually represents a failure of pleuroperitoneal canal to close completely

Answer 1164

left-sided posterolateral Bochdalek hernia which accounts for around 85% of cases.

Answer 1165

Only around 50% of newborns with CDH survive despite modern medical intervention.

Answer 1166

rubella, toxoplasmosis and cytomegalovirus

Answer 1167

cytomegalovirus maternal infection is usually asymptomatic

Answer 1168

1) Sensorineural deafness 2) Congenital cataracts 3) Congenital heart disease (e.g. patent ductus arteriosus) 4) Glaucoma Growth retardation Hepatosplenomegaly Purpuric skin lesions 'Salt and pepper' chorioretinitis Microphthalmia Cerebral palsy

Answer 1169

1) Cerebral calcification 2) Chorioretinitis 3) Hydrocephalus Anaemia Hepatosplenomegaly Cerebral palsy

Answer 1170

1) Low birth weight 2) Purpuric skin lesions 3) Sensorineural deafness 4) Microcephaly Visual impairment Learning disability Encephalitis/seizures Pneumonitis Hepatosplenomegaly Anaemia Jaundice Cerebral palsy

Answer 1171

patients less than 16 years old may consent to treatment if they are deemed to be competent (an example is the Fraser guidelines, previously termed Gillick competence), but cannot refuse treatment which may be deemed in their best interest between the ages of 16-18 years it is presumed patients are competent to give consent to treatment patients 18 years or older may consent to treatment or refuse treatment

Answer 1172

the young person understands the professional's advice the young person cannot be persuaded to inform their parents the young person is likely to begin, or to continue having, sexual intercourse with or without contraceptive treatment unless the young person receives contraceptive treatment, their physical or mental health, or both, are likely to suffer the young person's best interests require them to receive contraceptive advice or treatment with or without parental consent

Answer 1173

Fraser Guidelines

Answer 1174

at 16 years or older a young person can be treated as an adult and can be presumed to have capacity to decide under the age of 16 years children may have capacity to decide, depending on their ability to understand what is involved where a competent child refuses treatment, a person with parental responsibility or the court may authorise investigation or treatment which is in the child's best interests* *in Scotland those with parental responsibility cannot authorise procedures a competent child has refused

Answer 1175

Some doctors use the term Fraser competency when referring to contraception and Gillick competency when referring to general issues of consent in children

Answer 1176

peripheral cyanosis eg. hands and feet very common in 1st 24hrs of life, may occur when child is crying or unwell from any cause

Answer 1177

when the concentration of reduced haemoglobin in the blood exceeds 5g/dl

Answer 1178

The nitrogen washout test (also known as the hyperoxia test) the infant is given 100% oxygen for ten minutes after which arterial blood gases are taken. A pO2 of less than 15 kPa indicates cyanotic congenital heart disease

Answer 1179

supportive care prostaglandin E1 e.g. alprostadil - used to maintain a patent ductus arteriosus in ductal-dependent congenital heart defect - this can act as a holding measure until a definite diagnosis is made and surgical correction performed

Answer 1180

often seen in healthy newborns and refers to cyanosis around the mouth and the extremities such as the hands and feet. It is differentiated from other causes of peripheral cyanosis with significant pathology as it occurs immediately after birth in healthy infants. It is a common finding and may persist for 24 to 48 hours.

Answer 1181

sites of embryonic developmental fusion

Answer 1182

midline of the neck, external angle of the eye and posterior to the pinna of the ear. They typically have multiple inclusions such as hair follicles that bud out from its walls. They may develop at other sites such as the ovary and in these sites are synonymous with teratomas.

Answer 1183

may be classified either as low grade fibrosarcomas or non aggressive fibrous tumours. They commonly present as large infiltrative masses. They may be divided into abdominal, extra abdominal and intra abdominal. All types share the same biological features. Extra abdominal desmoids have an equal sex distribution and primarily arise in the musculature of the shoulder, chest wall, back and thigh. Abdominal desmoids usually arise in the musculoaponeurotic structures of the abdominal wall. Intra abdominal desmoids tend to occur in the mesentery or pelvic side walls and occur most frequently in patients with familial adenomatous polyposis coli syndrome.

Answer 1184

diarrhoea usually lasts for 5-7 days and stops within 2 weeks vomiting usually lasts for 1-2 days and stops within 3 days

Answer 1185

normal, dehydrated or shocked

Answer 1186

Appears to be unwell or deteriorating Decreased urine output Skin colour unchanged Warm extremities Altered responsiveness (for example, irritable, lethargic) Sunken eyes Dry mucous membranes Tachycardia Tachypnoea Normal peripheral pulses Normal capillary refill time Reduced skin turgor Normal blood pressure

Answer 1187

Decreased level of consciousness Cold extremities Pale or mottled skin Tachycardia Tachypnoea Weak peripheral pulses Prolonged capillary refill time Hypotension

Answer 1188

children younger than 1 year, especially those younger than 6 months infants who were of low birth weight children who have passed six or more diarrhoeal stools in the past 24 hours children who have vomited three times or more in the past 24 hours children who have not been offered or have not been able to tolerate supplementary fluids before presentation infants who have stopped breastfeeding during the illness children with signs of malnutrition

Answer 1189

jittery movements increased muscle tone hyperreflexia convulsions drowsiness or coma

Answer 1190

- you suspect septicaemia or - there is blood and/or mucus in the stool or - the child is immunocompromised consider if: - the child has recently been abroad or - the diarrhoea has not improved by day 7 or - you are uncertain about the diagnosis of gastroenteritis

Answer 1191

Admit for IV rehydration

Answer 1192

continue breastfeeding and other milk feeds encourage fluid intake discourage fruit juices and carbonated drinks

Answer 1193

give 50 ml/kg low osmolarity oral rehydration solution (ORS) solution over 4 hours, plus ORS solution for maintenance, often and in small amounts continue breastfeeding consider supplementing with usual fluids (including milk feeds or water, but not fruit juices or carbonated drinks)

Answer 1194

child needs 100 mL/kg fluids per day. For example, if a child weighs 5 kg, the maintenance fluid volume is 500 mL/day.

Answer 1195

child needs 1000 mL plus 50 mL/kg for each kg over 10 kg per day. For example, if a child weighs 15 kg, the maintenance fluid volume is 1250 mL/day.

Answer 1196

child needs 1500 mL plus 20 mL/kg for each kg over 20 kg per day. For example, if a child weighs 25 kg, the maintenance fluid volume is 1600 mL/day.

Answer 1197

(240–250 mOsm/L) Age 5 years or younger= give 50 mL/kg body weight for fluid deficit replacement, as well as maintenance volume of ORS solution, over 4 hours. Note: breastfeeding can continue, but do not routinely give oral fluids other than ORS solution. Age 5–11 years: give 200 mL ORS solution after each loose stool, in addition to the child's normal fluid intake. Age 12–16 years: give 200–400 mL ORS solution after every loose stool, dose according to fluid loss After rehydration, for children at increased risk of dehydration: give 5 mL/kg body weight after each large watery stool, to prevent recurrence of dehydration.

Answer 1198

- advice from local health protection team - esp if <6m or malnourished or immunocompromised

Answer 1199

- do not give Abx - specialist monitoring for Cx= haemolytic uraemic syndrome

Answer 1200

- specialist advice for metronidazole Tx - don't go swimming for 2w after last diarrhoea episode

Answer 1201

Abx not needed unless severe or immunocompromised

Answer 1202

drug Tx needed specialist advice for microbio clearance to confirm Tx success 1w after finishing Tx

Answer 1203

Abx not needed as self-limiting UNLESS severe, immunocompromised or worsening

Answer 1204

dont go swimming 2w after last episode of D specialist advice if immunocompromised

Answer 1205

child is systemically unwell and/or there are clinical features suggesting severe dehydration and/or progression to shock. There is intractable or bilious vomiting. There is acute-onset painful, bloody diarrhoea in previously healthy children, or confirmed Shiga toxin-producing Escherichia coli (STEC) infection There is a suspected serious complication, such as haemolytic uraemic syndrome or sepsis.

Answer 1206

most common cause of gastroenteritis diarrhoea, fever and vomiting for the first 2 days. The diarrhoea may last up to a week main risk is severe dehydration Tx is rehydration

Answer 1207

most common cause in the developed world is cows' milk intolerance toddler diarrhoea: stools vary in consistency, often contain undigested food coeliac disease post-gastroenteritis lactose intolerance

Answer 1208

LH= high Testosterone= low

Answer 1209

?Primary hypogonadism (Klinefelter's syndrome)

Answer 1210

LH= low Testosterone= low

Answer 1211

?Hypogonadotrophic hypogonadism (Kallman's syndrome)

Answer 1212

LH= high testosterone= normal/high

Answer 1213

Androgen insensitivity syndrome

Answer 1214

LH= low Testosterone= high

Answer 1215

?Testosterone-secreting tumour

Answer 1216

Klinefelter's syndrome

Answer 1217

chromosomal analysis

Answer 1218

often taller than average lack of secondary sexual characteristics small, firm testes infertile gynaecomastia - increased incidence of breast cancer elevated gonadotrophin levels

Answer 1219

?Klinefeleters

Answer 1220

cause of delayed puberty secondary to hypogonadotrophic hypogonadism

Answer 1221

X-linked recessive trait inheritance failure of GnRH-secreting neurons to migrate to hypothalamus

Answer 1222

Kallman's syndrome

Answer 1223

'delayed puberty' hypogonadism, cryptorchidism anosmia sex hormone levels are low LH, FSH levels are inappropriately low/normal patients are typically of normal or above average height Cleft lip/palate and visual/hearing defects are also seen in some patients

Answer 1224

Kallman's syndrome

Answer 1225

X-linked recessive condition due to end-organ resistance to testosterone causing genotypically male children (46XY) to have a female phenotype (complete androgen insensitivity syndrome= testicular feminisation syndrome)

Answer 1226

'primary amenorrhoea' undescended testes causing groin swellings breast development may occur as result of conversion of testosterone to oestradiol

Answer 1227

buccal smear or chromosomal analysis to reveal 46XY genotype

Answer 1228

counselling- raise child as female bilateral orchidectomy (as increased risk of testicular ca due to undescended testes) oestrogen therapy

Answer 1229

46XY (male)

Answer 1230

Androgen insensitivity syndrome

Answer 1231

congenital heart defect characterised by low insertion of the tricuspid valve resulting in a large atrium and small ventricle. It is sometimes referred to as 'atrialisation' of the right ventricle.

Answer 1232

exposure to lithium in utero

Answer 1233

patent foramen ovale (PFO) or atrial septal defect (ASD) is seen in at least 80% of patients, resulting in a shunt between the right and left atria Wolff-Parkinson White syndrome

Answer 1234

cyanosis prominent 'a' wave in the distended jugular venous pulse, hepatomegaly tricuspid regurgitation= pansystolic murmur, worse on inspiration right bundle branch block → widely split S1 and S2

Answer 1235

tricuspid regurgitation= pansystolic murmur, worse on inspiration right bundle branch block → widely split S1 and S2

Answer 1236

congenital cyst found in the mouth. They are common on the hard palate, but may also be seen on the gums where the parents may mistake it for an erupting tooth. No treatment is generally required as they tend to spontaneously resolve over the course of a few weeks.

Answer 1237

before 2 years but clears in around 50% of children by 5 years of age and in 75% of children by 10 years of age

Answer 1238

itchy, erythematous rash repeated scratching may exacerbate affected areas in infants the face and trunk are often affected in younger children, eczema often occurs on the extensor surfaces in older children, a more typical distribution is seen, with flexor surfaces affected and the creases of the face and neck

Answer 1239

- avoid irritants - simple emollients - topical steroids - wet wrapping - severe= oral ciclosporin

Answer 1240

large quantities should be prescribed (e.g. 250g / week), roughly in a ratio of with topical steroids of 10:1 if a topical steroid is also being used the emollient should be applied first followed by waiting at least 30 minutes before applying the topical steroid creams soak into the skin faster than ointments emollients can become contaminated with bacteria - fingers should not be inserted into pots (many brands have pump dispensers)

Answer 1241

large amounts of emollient (and sometimes topical steroids) applied under wet bandages

Answer 1242

baby may show symptoms of alcohol withdrawl at birth eg. irritable, hypotonic, tremors

Answer 1243

short palpebral fissure thin vermillion border/hypoplastic upper lip smooth/absent filtrum learning difficulties microcephaly growth retardation epicanthic folds cardiac malformations

Answer 1244

trinucleotide repeat X linked dominant

Answer 1245

learning difficulties large low set ears, long thin face, high arched palate macroorchidism hypotonia autism is more common mitral valve prolapse

Answer 1246

range from normal to mild

Answer 1247

can be made antenatally by chorionic villus sampling or amniocentesis analysis of the number of CGG repeats using restriction endonuclease digestion and Southern blot analysis

Answer 1248

Gastroschisis and exomphalos

Answer 1249

congenital defect in the anterior abdominal wall just lateral to the umbilical cord.

Answer 1250

vaginal delivery may be attempted newborns should go to theatre as soon as possible after delivery, e.g. within 4 hours

Answer 1251

the abdominal contents protrude through the anterior abdominal wall but are covered in an amniotic sac formed by amniotic membrane and peritoneum.

Answer 1252

Beckwith-Wiedemann syndrome Down's syndrome cardiac and kidney malformations

Answer 1253

c-section is indicated to reduce the risk of sac rupture a staged repair may be undertaken as primary closure may be difficult due to lack of space/high intra-abdominal pressure - if this occurs the sacs is allowed to granulate and epithelialise over the coming weeks/months - this forms a 'shell' - as the infant grows a point will be reached when the sac contents can fit within the abdominal cavity. At this point the shell will be removed and the abdomen closed

Answer 1254

growing pains

Answer 1255

often not related to growth so current term is benign idiopathic nocturnal limb pains of childhood

Answer 1256

boys and girls 3-12yrs

Answer 1257

- nocturnal limb pain never present at the start of the day after the child has woken no limp no limitation of physical activity systemically well normal physical examination motor milestones normal symptoms are often intermittent and worse after a day of vigorous activity

Answer 1258

Infancy (birth to 2-years-old) Childhood (3 to 11-years-old) Puberty (12 to 18-years-old)

Answer 1259

environmental: this is the most important factor affecting fetal growth e.g. maternal nutrition and uterine capacity placental hormonal genetic: predominately maternal

Answer 1260

nutrition and insulin not dependent on growth hormone as they have a low amount of receptors and finally growth isn't affected by hypopituitarism and there is no effect of the thyroid

Answer 1261

In childhood growth is driven by growth hormone and thyroxine and in puberty growth is driven by growth hormone and sex steroid, high amount of growth hormone is important in growth spurts. Genetic factors are the most important determinant of final adult height.

Answer 1262

infants aged 0-1 years should have at least 5 recordings of weight children aged 1-2 years should have at least 3 recordings of weight children older than 2 years should have annual recording of weight children below 2nd centile for height should be reviewed by their GP children below 0.4th centile for height should be reviewed by a paediatrician

Answer 1263

migraine without aura

Answer 1264

A= >= 5 attacks fulfilling features B to D B= Headache attack lasting 4-72 hours C= Headache has at least two of the following four features: - bilateral or unilateral (frontal/temporal) location - pulsating quality - moderate to severe intensity - aggravated by routine physical activity D= At least one of the following accompanies headache: - nausea and/or vomiting - photophobia and phonophobia (may be inferred from behaviour)

Answer 1265

ibuprofen triptans if >=12yrs but follow up needed (sumatriptan nasal spray but poorly tolerated due to bad taste) oral triptans not licensed <18yrs

Answer 1266

tingling, heat and heaviness/pressure sensations

Answer 1267

tension type headache

Answer 1268

A= At least 10 previous headache episodes fulfilling features B to D B= Headache lasting from 30 minutes to 7 days C= At least two of the following pain characteristics: - pressing/tightening (non/pulsating) quality - mild or moderate intensity (may inhibit but does not prohibit activity) - bilateral location - no aggravation by routine physical activity D= Both of the following: - no nausea or vomiting - photophobia and phonophobia, or one, but not the other is present

Answer 1269

Otoacoustic emission test A computer-generated click is played through a small earpiece. The presence of a soft echo indicates a healthy cochlea

Answer 1270

Auditory brainstem response test (in newborns and infants)

Answer 1271

Distraction test performed by health visitor, requires 2 trained staff

Answer 1272

recognition of familiar objects Uses familiar objects e.g. teddy, cup. Ask child simple questions - e.g. 'where is the teddy?'

Answer 1273

Performance testing or Speech discrimination tests - Uses similar-sounding objects e.g. Kendall Toy test, McCormick Toy Test

Answer 1274

Pure tone audiometry

Answer 1275

Pure tone audiometry

Answer 1276

questionnaire in the Personal Child Health Records- 'Can your baby hear you?'

Answer 1277

rare autosomal recessive disease caused by a deficiency of cystathionine beta synthase. This results in severe elevations in plasma and urine homocysteine concentrations.

Answer 1278

often patients have fine, fair hair MSK= Marfanoid body habitus: arachnodactyly etc; osteoporosis; kyphosis neuro: may have learning difficulties, seizures ocular= downwards (inferonasal) dislocation of lens; severe myopia increased risk of arterial and venous thromboembolism also malar flush, livedo reticularis

Answer 1279

increased homocysteine levels in serum and urine cyanide-nitroprusside test: also positive in cystinuria

Answer 1280

vitamin B6 (pyridoxine) supplements.

Answer 1281

congenital abnormality of the penis genetic element usually identified on newborn baby check; if missed parents may notice abnormal urine stream

Answer 1282

a ventral urethral meatus a hooded prepuce chordee (ventral curvature of the penis) in more severe forms the urethral meatus may open more proximally in the more severe variants. However, 75% of the openings are distally located.

Answer 1283

cryptorchidism (present in 10%) and inguinal hernia.

Answer 1284

refer to specialist services corrective surgery is typically performed when the child is around 12m it is essential that the child is NOT circumcised prior to the surgery as the foreskin may be used in the corrective procedure in boys with very distal disease, no treatment may be needed.

Answer 1285

autoimmune thryoiditis

Answer 1286

autoimmune thyroiditis post total-body irradiation (e.g. in a child previous treated for acute lymphoblastic leukaemia) iodine deficiency (the most common cause in the developing world)

Answer 1287

hypoxic ischaemic encephalopathy

Answer 1288

Down's syndrome Prader-Willi syndrome hypothyroidism cerebral palsy (hypotonia may precede the development of spasticity)

Answer 1289

spinal muscular atrophy spina bifida Guillain-Barre syndrome myasthenia gravis muscular dystrophy myotonic dystrophy

Answer 1290

- acutely ill child eg. septicaemic - central causes - neuro and muscular causes

Answer 1291

West syndrome

Answer 1292

type of childhood epilepsy which typically presents in the first 4 to 8 months of life and is more common in male infants. They are often associated with a serious underlying condition and carry a poor prognosis

Answer 1293

characteristic 'salaam' attacks: flexion of the head, trunk and arms followed by extension of the arms this lasts only 1-2 seconds but may be repeated up to 50 times progressive mental handicap

Answer 1294

the EEG shows hypsarrhythmia in two-thirds of infants CT demonstrates diffuse or localised brain disease in 70% (e.g. tuberous sclerosis)

Answer 1295

poor prognosis vigabatrin 1st line ACTH also used

Answer 1296

- ejection murmurs - venous hums - Still's murmur

Answer 1297

due to turbulent blood flow at the outflow tract of the heart

Answer 1298

due to the turbulent blood flow in the great veins returning to the heart heard as continuous blowing noise heard just below the clavicles

Answer 1299

low-pitched sound heard at the lower left sternal edge

Answer 1300

soft-blowing murmur in the pulmonary area or short buzzing murmur in the aortic area may vary with posture localised with no radiation no diastolic component no thrill no added sounds (e.g. clicks) asymptomatic child no other abnormality

Answer 1301

haemorrhage that occurs into the ventricular system of the brain. It is relatively rare in adult surgical practice and when it does occur, it is typically associated with severe head injuries. In premature neonates it may occur spontaneously. The blood may clot and occlude CSF flow, hydrocephalus may result. In neonatal practice the vast majority of IVH occur in the first 72 hours after birth, the aetiology is not well understood and it is suggested to occur as a result of birth trauma combined with cellular hypoxia, together the with the delicate neonatal CNS.

Answer 1302

largely supportive, therapies such as intraventricular thrombolysis and prophylactic CSF drainage have been trialled and not demonstrated to show benefit. Hydrocephalus and rising ICP is an indication for shunting.

Answer 1303

in 1st 24hrs or after 14d

Answer 1304

jaundice from 2-14d

Answer 1305

rhesus haemolytic disease ABO haemolytic disease hereditary spherocytosis glucose-6-phosphodehydrogenase def

Answer 1306

due to a combination of factors, including more red blood cells, more fragile red blood cells and less developed liver function. It is more commonly seen in breastfed babies

Answer 1307

after 14d (or 21d if premature)

Answer 1308

conjugated and unconjugated bilirubin: the most important test as a raised conjugated bilirubin could indicate biliary atresia which requires urgent surgical intervention direct antiglobulin test (Coombs' test) TFTs FBC and blood film urine for MC&S and reducing sugars U&Es and LFTs

Answer 1309

biliary atresia hypothyroidism galactosaemia urinary tract infection breast milk jaundice prematurity (due to immature liver function; increased risk of kernicterus) congenital infections e.g. CMV, toxoplasmosis

Answer 1310

Can be physiological or be a cause for prolonged jaundice (>14d) jaundice is more common in breastfed babies mechanism is not fully understood but thought to be due to high concentrations of beta-glucuronidase → increase in intestinal absorption of unconjugated bilirubin

Answer 1311

not inherited; it is due to random, somatic mutation in GNAS gene

Answer 1312

precocious puberty cafe-au-lait spots polyostotic fibrous dysplasia short stature

Answer 1313

McCune-Albright syndrome

Answer 1314

resp distress in newborn as a result of meconium in the trachea occurs in the immediate neonatal period

Answer 1315

in immediate neonatal period

Answer 1316

?meconium aspiration syndrome

Answer 1317

- post term delivery= 44% in babies born >42w - Hx of maternal HTN, pre-eclampsia, chorioamnionitis, smoking or substance abuse

Answer 1318

inflamed lymph nodes within the mesentery. It can cause similar symptoms to appendicitis and can be difficult to distinguish between the two. It often follows a recent viral infection and needs no treatment

Answer 1319

occipital-frontal circumference < 2nd centile

Answer 1320

normal variation e.g. small child with small head familial e.g. parents with small head congenital infection perinatal brain injury e.g. hypoxic ischaemic encephalopathy fetal alcohol syndrome syndromes: Patau craniosynostosis

Answer 1321

most DNA found in nucleus but small amount in mitochondria It encodes protein components of the respiratory chain and some special types of RNA

Answer 1322

inheritance is only via the maternal line as the sperm contributes no cytoplasm to the zygote none of the children of an affected male will inherit the disease all of the children of an affected female will inherit the disease generally, encode rare neurological diseases poor genotype:phenotype correlation - within a tissue or cell there can be different mitochondrial populations - this is known as heteroplasmy

Answer 1323

all children will get the disease

Answer 1324

affected female can only pass on disease not male

Answer 1325

rare neuro disease

Answer 1326

muscle biopsy classically shows 'red, ragged fibres' due to increased number of mitochondria

Answer 1327

Leber's optic atrophy= symptoms typically develop at around the age of 30 years; central scotoma → loss of colour vision → rapid onset of significant visual impairment MELAS syndrome= mitochondrial encephalomyopathy lactic acidosis and stroke-like episodes MERRF syndrome= myoclonus epilepsy with ragged-red fibres Kearns-Sayre syndrome= onset in patients < 20 years old, external ophthalmoplegia, retinitis pigmentosa. Ptosis may be seen sensorineural hearing loss

Answer 1328

nappy rash

Answer 1329

irritant dermatitis candida dermatitis seborrhoeic dermatitis psoriasis= less common atopic eczema= other areas also affected

Answer 1330

irritant dermatitis

Answer 1331

most common cause due to irritant effect of urinary ammonia and faeces creases are spared

Answer 1332

erythematous rash which involve the flexures and has characteristic satellite lesions

Answer 1333

erythematous rash with flakes; may coexist with scalp rash

Answer 1334

- disposable nappies not towel - expose napkin area to air when possible - apply barrier cream eg. Zinc and castor oil - mild steroid cream eg. 1% hydrocortisone if severe - Suspected candidal= topical imidazole; stop barrier cream until candida settled

Answer 1335

- thyroglossal cyst - brachial cyst - dermoids - thyroid gland= rare; cysts or lymphoma - lymphatic malformations - infantile haemangioma - lymphadenopathy

Answer 1336

Located in the anterior triangle, usually in the midline and below the hyoid (65% cases) Derived from remnants of the thyroglossal duct Thin walled and anechoic on USS (echogenicity suggests infection of cyst)

Answer 1337

Six branchial arches separated by branchial clefts Incomplete obliteration of the branchial apparatus may result in cysts, sinuses or fistulae 75% of branchial cysts originate from the second branchial cleft Usually located anterior to the sternocleidomastoid near the angle of the mandible Unless infected the fluid of the cyst has a similar consistency to water and is anechoic on USS

Answer 1338

Derived from pleuripotent stem cells and are located in the midline Most commonly in a suprahyoid location They have heterogeneous appearances on imaging and contain variable amounts of calcium and fat

Answer 1339

Usually located posterior to the sternocleidomastoid Cystic hygroma result from occlusion of lymphatic channels The painless, fluid filled, lesions usually present prior to the age of 2 They are often closely linked to surrounding structures and surgical removal is difficult They are typically hypoechoic on USS

Answer 1340

May present in either triangle of the neck Grow rapidly initially and then will often spontaneously regress Plain x-rays will show a mass lesion, usually containing calcified phleboliths As involution occurs the fat content of the lesions increases

Answer 1341

Located in either triangle of the neck May be reactive or neoplastic Generalised lymphadenopathy usually secondary to infection in children (very common)

Answer 1342

one of the leading causes of death among premature infants

Answer 1343

Initial symptoms can include feeding intolerance, abdominal distension and bloody stools, which can quickly progress to abdominal discolouration, perforation and peritonitis.

Answer 1344

abdo x-ray

Answer 1345

dilated bowel loops (often asymmetrical in distribution) bowel wall oedema pneumatosis intestinalis (intramural gas) portal venous gas pneumoperitoneum resulting from perforation air both inside and outside of the bowel wall (Rigler sign) air outlining the falciform ligament (football sign)

Answer 1346

1. Nil per os (NPO): The patient is kept without food or drink to rest the bowel. 2. Nasogastric decompression: A tube is inserted through the nose into the stomach to relieve gas and fluid. 3. Parenteral nutrition: Nutritional needs are met intravenously since oral feeding is stopped. 4. Broad-spectrum antibiotics: To treat potential bacterial infections that might be causing or complicating NEC. 5. Fluid resuscitation and electrolyte correction: As NEC can lead to dehydration and electrolyte imbalance. 6. Close monitoring SURGERY IF SEVERE

Answer 1347

First-Line Antibiotics: Amoxicillin: To cover Gram-positive bacteria. Gentamicin: To cover Gram-negative bacteria. Metronidazole: To cover anaerobic bacteria.

Answer 1348

5-9 days of life

Answer 1349

congenital hypothyroidism cystic fibrosis sickle cell disease phenylketonuria medium chain acyl-CoA dehydrogenase deficiency (MCADD) maple syrup urine disease (MSUD) isovaleric acidaemia (IVA) glutaric aciduria type 1 (GA1) homocystinuria (pyridoxine unresponsive) (HCU)

Answer 1350

yes in the 1st 24hrs of life but without any consequence as they can use alternative fuels like ketones and lactate

Answer 1351

< 2.6 mmol/L

Answer 1352

in the 1st few hours after birth

Answer 1353

preterm birth (< 37 weeks) maternal diabetes mellitus IUGR hypothermia neonatal sepsis inborn errors of metabolism nesidioblastosis Beckwith-Wiedemann syndrome

Answer 1354

- may be asymptomatic - autonomic (hypoglycaemia → changes in neural sympathetic discharge) - neuroglycopenic - apnoea - hypothermia

Answer 1355

'jitteriness' irritable tachypnoea pallor

Answer 1356

poor feeding/sucking weak cry drowsy hypotonia seizures

Answer 1357

severity of hypoglycaemia and if newborn is symptomatic

Answer 1358

asymptomatic= encourage normal feeding (breast or bottle); monitor blood glucose symptomatic or very low blood glucose= admit to neonatal unit; IV infusion of 10% dextrose

Answer 1359

Pre-Feed Glucose <1.0mmol/l OR Clinical Signs Consistent with Hypoglycaemia at a higher blood glucose concentration

Answer 1360

neonatal sepsis Werdnig-Hoffman disease (spinal muscular atrophy type 1) hypothyroidism Prader-Willi

Answer 1361

maternal drugs e.g. benzodiazepines maternal myasthenia gravis

Answer 1362

'male Turner's' autosomal dominant condition associated with a normal karyotype. It is thought to be caused by a defect in a gene on chromosome 12

Answer 1363

Turner's syndrome (webbed neck, widely-spaced nipples, short stature, pectus carinatum and excavatum) cardiac: pulmonary valve stenosis ptosis triangular-shaped face low-set ears coagulation problems: factor XI deficiency

Answer 1364

pulmonary valve stenosis

Answer 1365

- Flat feet (pes planus)= all ages - In toeing= 1st yr - Out toeing= all ages - Bow legs (genu varum)= 1-2nd yr - Knock knees (genu valgum)= 3-4th yr

Answer 1366

All ages Absent medial arch on standing Typically resolves between the ages of 4-8 years Orthotics are not recommended Parental reassurance appropriate

Answer 1367

1st year Possible causes: - metatarsus adductus: abnormal heel bisector line. 90% of cases resolve spontaneously, severe/persistent cases may require serial casting - internal tibial torsion: difference the thigh and foot ankle: resolves in the vast majority - femoral anteversion: 'W' sign resolves in around 80% by adolescence, surgical intervention in the remaining not usually advised

Answer 1368

All ages Common in early infancy and usually resolves by the age of 2 years Usually due to external tibial torsion Intervention may be appropriate if doesn't resolve as increases risk of patellofemoral pain

Answer 1369

1st-2nd year Increased intercondylar distance Typically resolves by the age of 4-5 years

Answer 1370

3rd-4th year Increased intermalleolar distance Typically resolves spontaneously

Answer 1371

infection of newborn eye

Answer 1372

refer for same-day opthalmology/paeds assessment

Answer 1373

Chlamydia trachomatis Neisseria gonorrhoeae

Answer 1374

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

Answer 1375

Central abdominal pain and URTI Conservative management

Answer 1376

High caecum at the midline Feature in exomphalos, congenital diaphragmatic hernia, intrinsic duodenal atresia May be complicated by the development of volvulus, an infant with volvulus may have bile stained vomiting

Answer 1377

upper GI contrast study and USS

Answer 1378

laparotomy, if volvulus is present (or at high risk of occurring then a Ladd's procedure is performed (includes division of Ladd bands and widening of the base of the mesentery)

Answer 1379

Absence of ganglion cells from myenteric and submucosal plexuses Occurs in 1/5000 births Full-thickness rectal biopsy for diagnosis Delayed passage of meconium and abdominal distension Treatment is with rectal washouts initially, after that an anorectal pull through procedure

Answer 1380

Associated with tracheo-oesophageal fistula and polyhydramnios May present with choking and cyanotic spells following aspiration VACTERL associations

Answer 1381

Usually delayed passage of meconium and abdominal distension The majority have cystic fibrosis X-Rays will not show a fluid level as the meconium is viscid, PR contrast studies may dislodge meconium plugs and be therapeutic Infants who do not respond to PR contrast and NG N-acetyl cysteine will require surgery to remove the plugs

Answer 1382

Jaundice > 14 days Increased conjugated bilirubin Urgent Kasai procedure

Answer 1383

Telescoping bowel Proximal to or at the level of, ileocaecal valve 6-9 months of age Colicky pain, diarrhoea and vomiting, sausage-shaped mass, red jelly stool. Treatment: reduction with air insufflation

Answer 1384

- pyloric stenosis - acute appendicitis - mesenteric adenitis - intussusception - intestinal malrotation - Hirschsprungs disease - Oesophageal atresia - Meconium ileus - Biliary atresia - Necrotising enterocolitis

Answer 1385

Splints and harnesses or traction. In later years osteotomy and hip realignment procedures may be needed. In arthritis a joint replacement may be needed. However, this is best deferred if possible as it will almost certainly require revision

Answer 1386

Usually diagnosed in infancy by screening tests. May be bilateral, when disease is unilateral there may be leg length inequality. As disease progresses child may limp and then early onset arthritis. More common in extended breech babies.

Answer 1387

Initially no obvious change on plain films and USS gives best resolution until 3 months of age. On plain films Shentons line should form a smooth arc

Answer 1388

Hip pain (may be referred to the knee) usually occurring between 5 and 12 years of age. Bilateral disease in 20%.

Answer 1389

Remove pressure from joint to allow normal development. Physiotherapy. Usually self-limiting if diagnosed and treated promptly.

Answer 1390

X-rays will show flattened femoral head. Eventually in untreated cases the femoral head will fragment.

Answer 1391

Slipped upper femoral epiphysis

Answer 1392

Typically seen in obese male adolescents. Pain is often referred to the knee. Limitation to internal rotation is usually seen. Knee pain is usually present 2 months prior to hip slipping. Bilateral in 20%.

Answer 1393

Bed rest and non-weight bearing. Aim to avoid avascular necrosis. If severe slippage or risk of it occurring then percutaneous pinning of the hip may be required.

Answer 1394

X-rays will show the femoral head displaced and falling inferolaterally (like a melting ice cream cone) The Southwick angle gives indication of disease severity

Answer 1395

During development the umbilicus has two umbilical arteries and one umbilical vein. The arteries are continuous with the internal iliac arteries and the vein is continuous with the falciform ligament (ductus venosus). After birth, the cord dessicates and separates and the umbilical ring closes.

Answer 1396

Up to 20% of neonates may have an umbilical hernia, it is more common in premature infants. The majority of these hernias will close spontaneously (may take between 12 months and three years). Strangulation is rare.

Answer 1397

due to defects in the linea alba that are in close proximity to the umbilicus. The edges of a paraumbilical hernia are more clearly defined than those of an umbilical hernia. They are less likely to resolve spontaneously than an umbilical hernia.

Answer 1398

infection of the umbilicus. Infection with Staphylococcus aureus is the commonest cause. The condition is potentially serious as infection may spread rapidly through the umbilical vessels in neonates with a risk of portal pyaemia, and portal vein thrombosis. Treatment is usually with a combination of topical and systemic antibiotics.

Answer 1399

These consist of cherry red lesions surrounding the umbilicus, they may bleed on contact and be a site of seropurulent discharge. Infection is unusual and they will often respond favourably to chemical cautery with topically applied silver nitrate.

Answer 1400

characterised by urinary discharge from the umbilicus. It is caused by persistence of the urachus which attaches to the bladder. They are associated with other urogenital abnormalities.

Answer 1401

typically present as an umbilical discharge that discharges small bowel content. Complete persistence of the duct is a rare condition. Much more common is the persistence of part of the duct (Meckel's diverticulum). Persistent vitello-intestinal ducts are best imaged using a contrast study to delineate the anatomy and are managed by laparotomy and surgical closure.

Answer 1402

<1= 110-160 1-2= 100-150 2-5= 90-140 5-12= 80-120 >12= 60-100

Answer 1403

<1= 30-40 1-2= 25-35 2-5= 25-30 5-12= 20-25 >12= 15-20

Answer 1404

- congenital heart defect - classed as acyanotic but intreated can be cyanotic as eventually result in late cyanosis in lower extremities - connection between pulmonary trunk and descending aorta

Answer 1405

patent ductus arteriosus

Answer 1406

with the first breaths due to increased pulmonary flow which enhances prostaglandins clearance

Answer 1407

premature born at high altitude maternal rubella infection in 1st trimester

Answer 1408

left subclavicular thrill continuous 'machinery' murmur large volume, bounding, collapsing pulse wide pulse pressure heaving apex beat

Answer 1409

- expectant supportive care as often spontaneous closure - is haemodynamically unstable or ventilator dependent after 1w of age= ibuprofen, indomethacin or paracetamol

Answer 1410

inhibits prostaglandin synthesis given to the infant, not to the mother in the antenatal period closes the connection in the majority of cases indomethacin use is declining due to increased side-effect profile compared to other drugs

Answer 1411

- closure if= moderate/large; prior episode of endocarditis or small audible PDA - technique= transcatheter ΡDA closure (pharmacological not effective in term infants)

Answer 1412

Prostaglandin E1 to keep PDA open until after surgical repair

Answer 1413

autosomal recessive condition caused by a disorder of phenylalanine metabolism

Answer 1414

usually due to defect in phenylalanine hydroxylase, an enzyme which converts phenylalanine to tyrosine. In a small number of cases the underlying defect is a deficiency of the tetrahydrobiopterin-deficient cofactor, e.g. secondary to defective dihydrobiopterin reductase. High levels of phenylalanine lead to problems such as learning difficulties and seizures. The gene for phenylalanine hydroxylase is located on chromosome 12.

Answer 1415

usually presents by 6 months e.g. with developmental delay child classically has fair hair and blue eyes learning difficulties seizures, typically infantile spasms eczema 'musty' odour to urine and sweat (secondary to phenylacetate, a phenylketone)

Answer 1416

Guthrie test: the 'heel-prick' test done at 5-9 days of life - also looks for other biochemical disorders such as hypothyroidism hyperphenylalaninaemia phenylpyruvic acid in urine

Answer 1417

poor evidence base to suggest strict diet prevents learning disabilities dietary restrictions are however important during pregnancy as genetically normal fetuses may be affected by high maternal phenylalanine levels

Answer 1418

non-retractable foreskin

Answer 1419

if non-retractile foreskin and/or ballooning during micturition in <2yrs= expectant approach as will resolve over time AVOID forcible retraction as can cause scar formation personal hygiene important if >2yrs and recurrent balanoposthitis or UTI then consider Tx

Answer 1420

s.pnuemoniae

Answer 1421

1st line= amoxicillin no respone= + macrolide suspected mycoplasma or chlamydia= use macrolide associated with influenzae= co-amoxiclav

Answer 1422

episodic viral wheeze: only wheezes when has a viral upper respiratory tract infection (URTI) and is symptom free inbetween episodes multiple trigger wheeze: as well as viral URTIs, other factors appear to trigger the wheeze such as exercise, allergens and cigarette smoke epsiodic wheeze= no risk of asthma trigeer wheeze= risk of asthma

Answer 1423

- symptomatic - 1st line= SABA via spacer - + montelukast or ICS or both

Answer 1424

ICS or montelukast trial for 4-8w

Answer 1425

'development of secondary sexual characteristics before 8 years in females and 9 years in males' more common in females

Answer 1426

first stage of breast development

Answer 1427

first stage of pubic hair development

Answer 1428

1) Gonadotrophin dependent ('central, true') 2) Gonadotrophin independent ('pseudo, false')

Answer 1429

due to premature activation of hypothalamic-pituitary-gonadal axis FSH and LH raised

Answer 1430

due to XS sex hormones LH and FSH low

Answer 1431

gonadotrophin dependent

Answer 1432

gonadotrophin independent XS sex hormones

Answer 1433

uncommon and usually has organic cause

Answer 1434

gonadotrophin release from intracranial lesion

Answer 1435

gonadal tumour

Answer 1436

adrenal cause (tumour or adrenal hyperplasia)

Answer 1437

usually idiopathic or familial and follows normal sequence of puberty

Answer 1438

usually idiopathic or familial organic causes rare= associated with rapid onset, neuro symptoms and dissonance eg. McCune Albright syndrome

Answer 1439

newborn infants with underdeveloped lungs eg. due to oligohydramnios or congenital diaphragmatic hernia

Answer 1440

syncopal episode (or presyncope) that occurs in response to pain or emotional stimuli. typically in 6m-3yrs

Answer 1441

caused by neurally-mediated transient asystole in children with very sensitive vagal cardiac reflexes

Answer 1442

child goes very pale, falls to floor, secondary anoxic seizures common, rapid recovery

Answer 1443

none and prognosis excellent

Answer 1444

most common ocular malignancy found in children average age of diagnosis 18m

Answer 1445

autosomal dominant 10% hereditary caused by loss of function of the retinoblastoma tumour suppressor gene on chromosome 13

Answer 1446

absence of red reflex, replaced by white pupil (leukocoria)- most common strabismus visual problems

Answer 1447

- enucleation - or chemo, external beam radiation therapy or photocoagulation (depending on how advanced) >90% survive into adulthood

Answer 1448

inadequately mineralised bone in developing and growing bones= results in soft and easily deformed bones usually due to vit D def called osteomalacia in adults

Answer 1449

dietary deficiency of calcium, for example in developing countries prolonged breastfeeding unsupplemented cow's milk formula lack of sunlight

Answer 1450

aching bones and joints lower limb abnormalities= in toddlers genu varum (bow legs); in older children - genu valgum (knock knees) 'rickety rosary' - swelling at the costochondral junction kyphoscoliosis craniotabes - soft skull bones in early life Harrison's sulcus

Answer 1451

- low vit D - reduced seru calcium (may get symptoms from hypocalcaemia) - raised ALP

Answer 1452

oral vit D

Answer 1453

until treated

Answer 1454

until recovered

Answer 1455

Conjunctivitis Fifth disease (slapped cheek) Roseola Infectious mononucleosis Head lice Threadworms Hand, foot and mouth

Answer 1456

until symptoms have settled for 48hrs

Answer 1457

retinal haemorrhages, subdural haematoma and encephalopathy

Answer 1458

shaken baby syndrome

Answer 1459

intentional shaking of a child (0-5yrs) sometimes hard to determine whether mechanism of injury is definitely intentional shaking so hard for courts to convict suspects

Answer 1460

plagiocephaly craniosynostosis

Answer 1461

parallelogram shaped head the incidence of plagiocephaly has increased over the past decade. This may be due to the success of the 'Back to Sleep' campaign

Answer 1462

premature fusion of skull bones

Answer 1463

slipped upper femoral epiphysis or slipped capital femoral epiphysis

Answer 1464

rare hip condition, classically seen in obese boys displacement of the femoral head epiphysis postero-inferiorly

Answer 1465

obese boys, 10-15yrs

Answer 1466

acute following trauma or more common chronic with persistent symptoms hip, groin, medial thigh or knee pain loss of internal rotation of leg in flexion bilateral slip in 20%

Answer 1467

AP and lateral (typically frog-leg) views x-ray diagnostic

Answer 1468

internal fixation= single cannulated screw placed in centre of epiphysis

Answer 1469

osteoarthritis avascular necrosis of femoral head chondrolysis leg length discrepancy

Answer 1470

obesity nasal problems= polyps, deviated septum, hypertrophic nasal turbinates recurrent tonsillitis Downs hypothyroidism

Answer 1471

- croup= 6m-3yrs - acute epiglottitis= 2-6yrs - inhaled foreign body - laryngomalacia= 4w; congenital abnormality of larynx

Answer 1472

respiratory distress syndrome or hyaline membrane disease

Answer 1473

condition seen in premature infants caused by insufficient surfactant production and structural immaturity of lungs

Answer 1474

prematurity (50% born 26-28w; 25% born at 30-31w) male diabetic mothers c-section 2nd born of premature twins

Answer 1475

common to resp distress in newborn eg. tachypnoea, intercostal recession, expiratory grunting and cyanosis

Answer 1476

CXR= ground glass appearance with indistinct heart border

Answer 1477

- prevention during preg= maternal corticosteroids to induce fetal lung maturation - oxygen - assisted ventilation - exogenous surfactant given via endotracheal tube

Answer 1478

pts who have had cardiac arrest, traumatic brain injury or neonatal hypoxic-ischaemic encephalopathy

Answer 1479

slows down metabolic rates of cells so reduces demand for O2 and production of harmful by-produccts that can lead to cell death

Answer 1480

Reduction of Metabolic Rate: Hypothermia decreases the metabolic rate by approximately 5-7% per degree Celsius reduction in body temperature, thereby conserving ATP and limiting the accumulation of lactic acid. Inhibition of Apoptosis: Cooling can reduce the activity of enzymes involved in programmed cell death (apoptosis), thus preserving cellular integrity. Reduction of Inflammatory Response: Hypothermia suppresses the inflammatory response, including the reduction of cytokine release, which can mitigate further tissue damage. Decreased Free Radical Production: By slowing metabolic processes, hypothermia reduces the production of free radicals, which can cause oxidative stress and damage cellular components.

Answer 1481

therapeutic cooling

Answer 1482

Surface Cooling: Utilises cooling blankets, ice packs, or cooling helmets. These devices are applied externally and provide a non-invasive method to lower body temperature. Endovascular Cooling: Involves the insertion of a catheter into a large vein (e.g., femoral vein), through which cold saline or other cooling fluids circulate, directly cooling the blood. Cold Fluid Infusion: Rapid infusion of cold intravenous saline can be used to quickly lower the body temperature.

Answer 1483

32-26C 24-72hrs the gradual rewarming period to avoid rapid T fluctuations that can cause rebound hyperthermia or Cx

Answer 1484

Temperature Monitoring: Core temperature should be continuously monitored using an oesophageal, bladder, or intravascular thermometer. Haemodynamic Stability: Patients undergoing therapeutic cooling require close monitoring of blood pressure, heart rate, and cardiac output, as hypothermia can induce bradycardia and hypotension. Electrolyte Management: Regular monitoring and correction of electrolytes, especially potassium, magnesium, and phosphate, is crucial due to shifts that occur during cooling and rewarming. Coagulation: Hypothermia can impair coagulation pathways, so coagulation profiles should be regularly assessed. Infection Control: The immune response is suppressed at lower temperatures, increasing the risk of infection. Prophylactic antibiotics may be considered in some cases.

Answer 1485

Cardiovascular: Bradycardia, arrhythmias, hypotension. Coagulopathy: Increased risk of bleeding due to impaired platelet function and clotting enzyme activity. Infection: Increased susceptibility to infections due to immunosuppression. Electrolyte Imbalance: Hypokalaemia during cooling and hyperkalaemia during rewarming. Shivering: Can increase metabolic rate and counteract the cooling process. sedation, neuromuscular blockade, or specific anti-shivering protocols.

Answer 1486

conducted slowly, typically at a rate of 0.25-0.5°C per hour, to minimise the risk of complications such as electrolyte imbalances, hypotension, and rebound hyperthermia. Continuous monitoring and supportive care are essential during this phase.

Answer 1487

(Enterobius vermicularis, sometimes called pinworms) Infestation occurs after swallowing eggs that are present in the environment.

Answer 1488

90% asymptomatic - perianal itching, esp at nigjt - girls may have vulval symptoms

Answer 1489

just treat empirically can apply sellotape to perianal area and send to lab for micro to see eggs

Answer 1490

hygiene measure and single dose mebendazole for all members of household (>6m; may need more if infestation persists)

Answer 1491

cause of resp distress caused by delayed resoption of fluid in the lungs

Answer 1492

following c-section, may be due to the lung fluid not being 'squeezed out' during passage through birth canal

Answer 1493

hyperinflation of lungs and fluid in horizontal fissure

Answer 1494

?Transient tachypnoea of the newborn (TTN)

Answer 1495

observation and supportive care may need supp O2 to maintain sats usually settles within 1-2d

Answer 1496

genes which normally control the cell cycle loss of function results in an increased risk of cancer both alleles must be mutated before cancer occurs

Answer 1497

p53= Common to many cancers, Li-Fraumeni syndrome APC= Colorectal cancer BRCA1 & BRCA2= Breast and ovarian cancer In men, there is an increased risk of breast cancer and prostate cancer NF1= Neurofibromatosis Rb= Retinoblastoma WT1= Wilm's tumour Multiple tumor suppressor 1 (MTS-1, p16)= Melanoma

Answer 1498

Tumour suppressor genes - loss of function results in an increased risk of cancer Oncogenes - gain of function results in an increased risk of cancer

Answer 1499

relatively common in children and may be found during the newborn exam. Usually no treatment is required as they typically resolve by 3 years of age Associations: - Afro-Caribbean infants - Down's syndrome - mucopolysaccharide storage diseases

Answer 1500

2-3% term male infants but more common in preterm 25% bilateral

Answer 1501

- infertility - torsion - testicular ca - psychological

Answer 1502

Unilateral undescended testis: - referral should be considered from around 3 months of age, with the baby ideally seeing a urological surgeon before 6 months of age - orchidopexy: Surgical practices vary although the majority of procedures are performed at around 1 year of age Bilateral undescended testes: - Should be reviewed by a senior paediatrician within 24hours as the child may need urgent endocrine or genetic investigation

Answer 1503

abnormal backflow of urine from the bladder into the ureter and kidney. It is a relatively common abnormality of the urinary tract in children and predisposes to urinary tract infection (UTI), being found in around 30% of children who present with a UTI. As around 35% of children develop renal scarring it is important to investigate for VUR in children following a UTI

Answer 1504

ureters are displaced laterally, entering the bladder in a more perpendicular fashion than at an angle therefore shortened intramural course of the ureter vesicoureteric junction cannot, therefore, function adequately

Answer 1505

antenatal period: hydronephrosis on ultrasound recurrent childhood urinary tract infections reflux nephropathy= - term used to describe chronic pyelonephritis secondary to VUR - commonest cause of chronic pyelonephritis - renal scar may produce increased quantities of renin causing hypertension

Answer 1506

Ix (USS renal tract) if <6m after 1st episode or >6m after 3 lower UTI diagnosed following a micturating cystourethrogram a DMSA scan may also be performed to look for renal scarring - this is typically located at the upper and lower pole and is associated with cortical thinning

Answer 1507

I= Reflux into the ureter only, no dilatation II= Reflux into the renal pelvis on micturition, no dilatation III= Mild/moderate dilatation of the ureter, renal pelvis and calyces IV= Dilation of the renal pelvis and calyces with moderate ureteral tortuosity V= Gross dilatation of the ureter, pelvis and calyces with ureteral tortuosity

Answer 1508

Conservative management is often the first line of treatment, especially in low-grade VUR. This involves long-term, low-dose antibiotic prophylaxis to prevent urinary tract infections (UTIs), which can lead to renal scarring. Regular monitoring with renal ultrasound and micturating cystourethrogram (MCUG) or direct radionuclide cystogram (DRNC) is also recommended to assess progress. Surgical intervention may be considered if there's persistent high-grade reflux, breakthrough UTIs despite prophylaxis, or evidence of renal damage. Several surgical options exist including endoscopic injection of bulking agents (Deflux), open ureteric reimplantation or laparoscopic robot-assisted ureteric reimplantation.

Answer 1509

inherited neurodevelopmental disorder caused by microdeletion on chromosome 7

Answer 1510

William's syndrome

Answer 1511

FISH studies

Answer 1512

elfin-like facies characteristic like affect - very friendly and social learning difficulties short stature transient neonatal hypercalcaemia supravalvular aortic stenosis

Answer 1513

one of most common childhood malignancies <5yrs, meadian age 3yrs

Answer 1514

Beckwith-Wiedemann syndrome as part of WAGR syndrome with Aniridia, Genitourinary malformations, mental Retardation hemihypertrophy around one-third of cases are associated with a loss-of-function mutation in the WT1 gene on chromosome 11

Answer 1515

abdominal mass (most common presenting feature) painless haematuria flank pain other features: anorexia, fever unilateral in 95% of cases metastases are found in 20% of patients (most commonly lung)

Answer 1516

children with an unexplained enlarged abdominal mass in children - possible Wilm's tumour - arrange paediatric review with 48 hours

Answer 1517

nephrectomy chemotherapy radiotherapy if advanced disease prognosis: good, 80% cure rate

Answer 1518

epithelial tubules, areas of necrosis, immature glomerular structures, stroma with spindle cells and small cell blastomatous tissues resembling the metanephric blastema

Answer 1519

?Wilms tumour so paeds review within 48hrs

Answer 1520

Alport's syndrome (in around 85% of cases - 10-15% of cases are inherited in an autosomal recessive fashion with rare autosomal dominant variants existing) Rett syndrome Vitamin D resistant rickets pseudohypoparathyroidism was previously classified as an X-linked dominant condition but has now been shown to be inherited in an autosomal dominant fashion in the majority of cases

Answer 1521

males, females can only be carriers EXCEPT in turners syndrome= affected due to only having 1 X chromosome

Answer 1522

by heterozygote females (carriers) and male-to-male transmission is not seen. Affected males can only have unaffected sons and carrier daughters.

Answer 1523

Each male child of a heterozygous female carrier has a 50% chance of being affected whilst each female child of a heterozygous female carrier has a 50% chance of being a carrier. The possibility of an affected father having children with a heterozygous female carrier is generally speaking extremely rare. However, in certain Afro-Caribbean communities G6PD deficiency is relatively common and homozygous females with clinical manifestations of the enzyme defect are seen.

Answer 1524

Androgen insensitivity syndrome Becker muscular dystrophy Colour blindness Duchenne muscular dystrophy Fabry's disease G6PD deficiency Haemophilia A,B Hunter's disease Lesch-Nyhan syndrome Nephrogenic diabetes insipidus Ocular albinism Retinitis pigmentosa Wiskott-Aldrich syndrome

Answer 1525

X-linked recessive

Answer 1526

X-linked recessive

Answer 1527

X-linked recessive

Answer 1528

X-linked recessive

Answer 1529

X-linked recessive

Answer 1530

Chronic granulomatous disease (in > 70%)

Paeds Flashcards

FLUIDS (in notebook); osteogenesis imperfecta; go to resp FC and update asthma new Mx; JIA?