Paediatrics

Question 1

What is mottled skin a sign of?

Answer 1

Poor perfusion

Question 2

Uptill what age is brachial pulse easier to assess than radial pulse?

Answer 2

6 months

Question 3

When is pulse rate best measured?

Answer 3

When child is calm

Question 4

What does CRT show in children?

Answer 4

Compromised circulation due to peripheral circulation shutdown

Question 5

What vaccinations are given at 8 weeks?

Answer 5

6 in 1
Meningococcal group B
Rotavirus

Question 6

What vaccinations are given at 12 weeks?

Answer 6

6 in 1
Pneumococcal
Rotavirus

Question 7

What vaccinations are given at 16 weeks?

Answer 7

6 in 1
Meningococcal group B

Question 8

What vaccinations are in the 6 in 1 vaccine?

Answer 8

Diphtheria
Tetanus
Pertussis
Polio
Haemophilus influenzae type b
Hepatitis B

Question 9

When is the 6 in 1 vaccine administered?

Answer 9

8, 12, 16 weeks

Question 10

When is the rotavirus vaccine administered?

Answer 10

8 and 12 weeks

Question 11

When is the pneumococcal vaccine administered?

Answer 11

12 weeks and 1 year

Question 12

When is the meningococcal group B vaccine adminstered?

Answer 12

8 and 16 weeks

Question 13

What vaccines are given at 1 year?

Answer 13

Haemophilus influenzae B
Meningococcal B and C
Pneumococcal
MMR

Question 14

What vaccines are given at 3 years and 4 months?

Answer 14

Diphtheria
Tetanus
Polio
Pertussis
MMR

Question 15

What vaccines are given at 14 years (year 9)?

Answer 15

Diphtheria, tetanus, polio
Meningococcal groups ACWY

Question 16

What are the major functional areas of developmental milestones?

Answer 16

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

Question 17

What defines the limit for a child to meet a certain developmental milestone?

Answer 17

Two standard deviations from mean age of acquisition

Question 18

What is otitis media?

Answer 18

General term to describe disorders of middle ear inflammation including acute otitis media, otitis media with effusion, chronic otitis media

Question 19

Epidemiology of otitis media?

Answer 19

Peak incidence between 6-18 months
Common complication of viral respiratory infection
More common in boys

Question 20

Aetiology of otitis media?

Answer 20

Respiratory virus/ bacteria

Question 21

Pathophysiology of otitis media?

Answer 21

Colonisation of nasal passage, eustachian tube and middle ear cause inflammation
Can lead to suppuration, effusion which can increase pressure against tympanic membrane

Question 22

Risk factors for otitis media?

Answer 22

Exposure to tobacco smoke
Lack of breastfeeding
Daycare attendance
Having siblings
Supine feeding
Low socioeconomic status
Craniofacial anomaly
Immunological deficiency

Question 23

Classification of otitis media?

Answer 23

Acute otitis media
Otitis media with effusion
Chronic otitis media

Question 24

What is the clinical presentation of otitis media?

Answer 24

Otalgia
Preceeding upper respiratory symptoms
Bulging tympanic membrane
Myringitis
Fever
Irritability and sleep disturbance

Question 25

Investigations to diagnose otitis media?

Answer 25

Otoscopy Clinical diagnosis

Question 26

Differentials for for otitis media?

Answer 26

Otitis media with effusion Mastoiditis Myringitis Cholesteatoma

Question 27

Management of otitis media?

Answer 27

Analgesia; paracetamol, ibuprofen Antibiotics if symptoms last longer than 72 hours and over 6 months; Amoxicillin, co-amoxiclav, Cefuroxime Tympanocentesis

Question 28

What could be cause of recurrent otitis media?

Answer 28

Anatomical anomaly

Question 29

Complications of otitis media?

Answer 29

Bullous myringitis Acutely perforated tympanic membrane Mastoiditis Otitis media with effusion

Question 30

Prognosis of otitis media?

Answer 30

Patient makes recovery in 2-3 days Long term complications are rare

Question 31

Gold standard investigation your congenital heart disease in children?

Answer 31

Echocardiogram

Question 32

What are the shunts present in foetal circulation?

Answer 32

Ductus venosus; connects umbilical vein to inferior vena cava to bypass liver Foramen ovale; connects RA to LA to bypass pulmonary circulation Ductus arteriosus; connects pulmonary artery to aorta to bypass pulmonary circulation

Question 33

What happens during the first breath?

Answer 33

Air inflates alveoli which decreases pulmonary vascular resistance Pressure in right atrium falls below left atrium squashing atrial septum and functions . as a functional closure of the foramen ovale Increased oxygenation causes a fall in prostaglandins and hence closure of ductus arteriosus

Question 34

What is needed for patency of ductus arteriosus?

Answer 34

Prostaglandin

Question 35

Features of an innocent murmur?

Answer 35

Soft Short Systolic Symptomless Situational; quieter with standing and typically presents during illness

Question 36

Features of a murmur prompting referral?

Answer 36

Murmur louder than 2/6 Diastolic murmur Louder on standing Failure to thrive, feeling difficulty, ,cyanosis, shortmen of breath

Question 37

Investigation to diagnose congenital heart disease?

Answer 37

ECG Chest X-ray Echocardiogram

Question 38

Causes of a pan-systolic murmur?

Answer 38

Mitral regurgitation Tricuspid regurgitation Ventricular septal defect

Question 39

Causes of ejection systolic murmur?

Answer 39

Aortic stenosis Pulmonary stenosis Hypertrophic obstructive cardiomyopathy

Question 40

Murmur heard in ASD?

Answer 40

Mid-systolic Crescendo-decresendo Heard loudest at upper left sternal boarder Fixed split second heart sound

Question 41

Murmur heard in patent ductus arteriosus?

Answer 41

Continuous crescendo- decresendo machinery murmur during second heart sound

Question 42

Murmur heard in tetralogy of fallot?

Answer 42

Arises from pulmonary stenosis Ejection systolic murmur heard loudest at the pulmonary area

Question 43

Acyanotic heart defects?

Answer 43

VSD ASD PDA However if Eisenmengers syndrome occurs can become cyanotic

Question 44

What is eisenmenger syndrome?

Answer 44

Reversal of a left to right shunt due to pulmonary pressure exceeding systemic pressure and converting shunt to right to left resulting in cyanosis

Question 45

When does ductus arteriosus close?

Answer 45

Functional closure achieved in first 3 days of life Complete closure in just 2-3 weeks

Question 46

Risk factors for PDA?

Answer 46

Genetic Maternal rubella infection Prematurity

Question 47

Pathophysiology of PDA?

Answer 47

Pressure is higher in aorta and hence systemic circulation so shunt is left to right This increases pulmonary pressure resulting in right heart strain leading to right ventricular hypertrophy Increased bloodflow through pulmonary vessels leads to increased left heart pressure and compensatory left ventricular hypertrophy

Question 48

Presentation of PDA?

Answer 48

Murmur; continuous crescendo-decrescendo machinery murmur during second heart sound Shortness of breath Difficulty feeding Poor weight gain Lower respiratory tract infection

Question 49

Management of PDA?

Answer 49

Typically mointerned till 12 months of age via echocardiogram, unlikely to close after 1 year of age Give ibuprofen or indomethacin Trans-catheter or surgical closure

Question 50

What is an ASD?

Answer 50

Hole in the septum between two atria connecting right and left atria allow blood to flow between

Question 51

Pathophysiology of ASD?

Answer 51

During normal development two walls, the septum primum and septum secondum fuse with endocardial cushion in middle of the heart Defects in this process result in ASD

Question 52

Types of ASD?

Answer 52

Ostium secondum Patent foramen ovale (not technically an ASD) Ostium primum Presented in order of how common these defects are

Question 53

Complication of ASD?

Answer 53

Stroke Atrial fibrillation /atrial flutter Pulmonary hypertension Eisenmenger syndrome

Question 54

Presentation of ASD?

Answer 54

Murmur; mid systolic crescendo decrescendo murmur heard best at upper left sternal border with fixed split second heart sound Shortness of breath Difficulty feeding Poor weight gain Lower respiratory tract infection

Question 55

Management of ASD?

Answer 55

Referral to paediatric cardiologist Correction of defect; Transvenous catheter closure, Open heart surgery Anticoagulation can be used to reduce risk of clots and stroke

Question 56

Associations with VSD?

Answer 56

Down's syndrome Turner's syndrome

Question 57

What is a VSD?

Answer 57

Congenital hole in the septum between the ventricles

Question 58

Pathophysiology of VSD?

Answer 58

Left to right shunt due to higher pressure in LV than RV, can ihad to right heart overload, right ventricular hypertrophy and pulmonary hypertension Can eventually lead to Eisenmengers syndrome

Question 59

Presentation of VSD?

Answer 59

Shortness of breath Tachypnoea Poor feeding Failure to thrive Murmur; Pan systolic murmur heard at left sternal border in third and fourth intercostal space Systolic thrill on palpation

Question 60

Management of VSD?

Answer 60

Often close spontaneously Transvenous catheter closure or open heart surgery

Question 61

What congenital defects can lead to eisenmangers syndrome?

Answer 61

Atrial septal defect Ventricular septal defect Patent ductus arteriosus

Question 62

Pathophysiology of eisenmenger's syndrome?

Answer 62

Left to right shunt shifts blood to right side of heart increasing pressure in pulmonary vessels leading to pulmonary hypertension, right ventricular hypertrophy Eventually pulmonary pressure exceeds systemic pressure shunt is reversed to a right to left shunt resulting in cyanosis

Question 63

Presentation of eisenmenger syndrome?

Answer 63

Cyanosis Clubbing Dyspnoea Plethoric complexion Examination findings; Right ventricular heave Loud P2 Raised JVP Peripheral oedema

Question 64

Prognosis of eisenmanger's syndrome?

Answer 64

Reduced life expectancy around 20 years compared to healthy individuals Main causes of death include heart failure, infection, thromboembolism and haemorrhage Pregnancy is higher risk

Question 65

Management of eisenmanger's syndrome?

Answer 65

Timely detection and correction of defect to prevent this complication Only definitive treatment is heart and lung transplantation Medical management; Pulmonary hypertension; slidenafil Treat arrhythmia Treat polycythaemia Prevent thrombosis Infective endocarditis prophylaxis

Question 66

What is coarctation of aorta?

Answer 66

Narrowing of aortic arch usually around the ductus arteriosus

Question 67

What genetic condition is associated with coarctation of aorta?

Answer 67

Turners syndrome

Question 68

Pathophysiology of coarctation of aorta?

Answer 68

Reduces pressure of blood distal to narrowing and increases pressure proximal to narrowing such as heart and just three branches of aorta

Question 69

Presentation of coarctation of aorta?

Answer 69

Weak femoral pulse Systolic murmur heard best below left clavicle or left scapula Tachypnoea Underdeveloped left arm and legs

Question 70

Management of coarctation of aorta?

Answer 70

In severe cases prostaglandin E2 can be used to maintain patency of ductus Surgical correction and ligate ductus

Question 71

What is aortic valve stenosis ?

Answer 71

When babies are born with a narrowed aortic valve orifice which restricts blood flow from the left ventricle into the aorta

Question 72

Symptoms of aortic valve stenosis?

Answer 72

Mild cases can remain asymptomatic Fatigue Shortness of breath Dizziness and fainting Symptoms are worse on exertion

Question 73

Signs of aortic valve stenosis?

Answer 73

Murmur; Ejection systolic crescendo- decrescendo which radiates over carotids Ejection click just before murmur Palpable thrill during systole Slow rising pulse and narrow pulse pressure

Question 74

Management of aortic valve stenosis?

Answer 74

Percutaneous balloon aortic valvoplasty Surgical aortic valvotomy Valve replacement

Question 75

Complications of aortic valve stenosis?

Answer 75

Left ventricular outflow tract obstruction Heart failure Ventricular arrhythmia Bacterial endocarditis Sudden death, Often on onexertion

Question 76

What is pulmonary valve stenosis?

Answer 76

Narrowing in right ventricular outflow tract

Question 77

Associations of congenital pulmonary valve stenosis?

Answer 77

Tetralogy of fallot William syndrome Noonan syndrome Congenital rubella syndrome

Question 78

Symptoms of pulmonary valve stenosis?

Answer 78

Fatigue on exertion Shortness of breath Dizziness and fainting

Question 79

Signs of pulmonary stenosis?

Answer 79

Murmur; Ejection systolic murmur .heard loudest at pulmonary area Palpable thrill in pulmonary area Right ventricular heave due to RVH Raised JVP with giant a waves

Question 80

Management of pulmonary stenosis?

Answer 80

Asymptomatic; watch and wait Balloon valvuloplasty Open heart surgery

Question 81

Defects that make up tetralogy of fallot?

Answer 81

Ventricular septal defect Overriding aorta Pulmonary valve stenosis Right ventricular hypertrophy

Question 82

Pathophysiology of tetralogy of fallot?

Answer 82

VSD provides left to right shunt Overriding aorta means aorta is connected more to the right so some blood from right ventricle empties into aorta creating a right to left shunt Pulmonary stenosis encourages passage of blood from right to left and causes RVH All these defects produce an overall right to left shunt and a cyanotic baby

Question 83

Risk factors for developing tetralogy of fallot?

Answer 83

Rubella infection Maternal age over 40 years Alcohol consumption in pregnancy Diabetic mother

Question 84

What is seen on chest x-ray in tetralogy of fallot?

Answer 84

Boot shaped heart

Question 85

Signs and symptoms of tetralogy of fallot?

Answer 85

Cyanosis Clubbing Poor feeding and poor weight gain Tet spells Murmur; ejection systolic due to pulmonary stenosis

Question 86

What is a tet spell?

Answer 86

Intermittent symptomatic periods where right to left shunt becomes temporarily worsened precipitating a cyanotic episode

Question 87

Pathophysiology of tet spell?

Answer 87

Pulmonary vascular resistance increases or systemic resistance decreases Such as ef child is physically exerting themself, a lot of CO2 is generated causing systemic vasodilation and hence reduces pressure Blood is pumped through path of least resistance so blood from RV into aorta Often precipitated by waking, physical exertion, crying

Question 88

Treatment for tet spells?

Answer 88

Squat, or in younger children pull knees to chest in younger children to increase systemic vascular resistance Supplementary oxygen Beta-blockers, morphine and fluids to help increase systemic pressure Sodium bicarbonate; buffer metabolic acidosis Phenylephrine infusion to increase systemic vascular resistance

Question 89

Management of tetralogy of fallot?

Answer 89

In neonates prostaglandin infusion to keep ductus open Total surgical repair by open heart surgery -> 5% mortality from surgery

Question 90

What is ebstein's anomaly?

Answer 90

Tricuspid valve is set lower in the right side of the heart producing a larger RA and smaller RV resulting in Right to left shunt through an ASD Blood bypasses lungs leading to cyanosis

Question 91

What condition is ebstein's anomaly associated with?

Answer 91

Wolff- Parkinson White syndrome Torsades de pointes

Question 92

Presentation of ebstein's anomaly?

Answer 92

Heart failure Gallop rhythm Cyanosis Shortness of breath Tachypnoea Poor feeding Collapse or cardiac arrest

Question 93

What is seen on ECG in ebstein's anomaly?

Answer 93

Arrhythmias Right atrial enlargement Right bundle branch block Left axis deviation

Question 94

Management of ebstein's anomaly?

Answer 94

Treat arrhythmia and heart failure Surgical correction

Question 95

What is transposition of the great arteries?

Answer 95

Attachments of the aorta and pulmonary trunk to the heart are swapped meaning the right ventricle pumps blood into aorta and left ventricle into pulmonary trunk

Question 96

What other conditions are associated with transposition of the great arteries?

Answer 96

Ventricular septal defect Coarctation of the aorta Pulmonary stenosis

Question 97

What is prognosis of transposition of great arteries?

Answer 97

Unless other co-existing shunt is present, baby will be born cyanosed and require immediate correction

Question 98

Presentation of transposition of great arteries?

Answer 98

Usually diagnosed during antenatal ultrasound scan, ensure woman delivers in a hospital capable of managing the baby VSD or PDA can initially compensate but will quickly develop respiratory distress, tachycardia, poor feeling, poor weight gain

Question 99

Management of transposition of great arteries?

Answer 99

Prostaglandin infusion to maintain patency of ductus, Balloon septostomy to create larger ASD to allow mixing of blood Arterial switch operation

Question 100

What is bronchiolitis?

Answer 100

Inflammation and infection of the bronchioles usually caused by virus

Question 101

Epidemiology of bronchiolitis?

Answer 101

Common upto the age of 2 years Most commonly diagnosed in infants under 6 months

Question 102

Pathophysiology of bronchiolitis?

Answer 102

Infection and inflammation of airways leads to hypersecretion of mucus which can block narrower airways Oedematous inflammation can also cause airways to become narrowing further limiting gas exchange

Question 103

Presentation of bronchiolitis?

Answer 103

Coryzal symptoms Dyspnoea Tachypnoea Poor feeding Mild fever Apnoea Wheeze and crackles on auscultation

Question 104

Signs of respiratory distress?

Answer 104

Increased respiratory rate Use of accessory muscles Intercostal and subcostal recessions Nasal flaring Head bobbing Tracheal tugging Cyanosis Abnormal airway noises

Question 105

Epidemiology of congenital heart defects?

Answer 105

1% of live births

Question 106

Risk factors for congenital heart disease?

Answer 106

Infection; Maternal rubella infection Exposure to teratogenic drugs; thalidomide, isotretinoin, lithium Maternal alcohol consumption Poorly controlled type 1 and type 2 diabetes

Question 107

What is a wheeze?

Answer 107

Whistling sound caused by narrowed airways, typically heard during expiration

Question 108

What is grunting?

Answer 108

Exhalation with the glottis partially closed to increase positive end expiratory pressure

Question 109

What is stridor?

Answer 109

High pitched inspiratory noise caused by obstruction of upper airway

Question 110

Typical course of RSV?

Answer 110

Starts as URTI with coryzal symptoms Chest symptoms develop in 1-2 days following coryzal symptoms Symptoms worsen on days 3 or 4 Symptoms last 7 to 10 days and patients fully recover in 2-3 weeks

Question 111

Prognosis of bronchiolitis?

Answer 111

Usually makes full recovery More likely to develop viral induced wheeze

Question 112

Indications to admit child with bronchiolitis?

Answer 112

Under three months Pre-existing condition; prematurity, cystic fibrosis, downs syndrome Clinical dehydration Respiratory rate over 70 Oxygen sats under 92% Moderate to severe respiratory distress Apnoea Concerns around managing child at home

Question 113

Management of bronchiolitis?

Answer 113

Ensure adequet fluid and oral intake Saline nasal drops and nasal suctioning Supplementary oxygen Ventilatory support if needed

Question 114

Signs of poor ventilation?

Answer 114

Measured on capillary blood gas Raising pCO2 Falling pH

Question 115

What is palivizumab?

Answer 115

Monoclonal antibody that targets RSV and is given as a monthly injection to high risk babies to protect them against RSV bronchiolitis

Question 116

Indications for palivizumab injection?

Answer 116

Ex premature Congenital heart disease

Question 117

What is viral induced wheeze?

Answer 117

Acute wheezy illness caused by viral infection usually presenting before age of 3 years with no atopic history

Question 118

Pathophysiology of viral induced wheeze?

Answer 118

Inflammation, oedema and swelling in walls of airways in response to virus results in airway constriction which can be made worse by smooth muscle constriction Reduced airflow through narrower airways resulting in symptoms of wheeze

Question 119

Presentation of viral induced wheeze?

Answer 119

Shortness of breath Signs of respiratory distress Expiratory wheeze throughout chest

Question 120

What is acute asthma?

Answer 120

Rapid deterioration of asthma usually triggered by allergy, infection, cold weather, exercise

Question 121

Presentation of acute asthma?

Answer 121

Shortness of breath Signs of respiratory distress Tachypnoea Expiratory wheeze Establish severity of attack

Question 122

Features of a moderate acute asthma attack?

Answer 122

Peak flow >50% predicted Normal speech

Question 123

Features of a severe acute asthma attack?

Answer 123

Peak flow <50% predicted 02 sats <92% Unable to complete sentences in one breath Signs of Respiratory distress Respiratory rate >40 in age 1-5 years, >30 in age over 5 years Heart rate >140 in 1-5 years, >125 in age over 5 years

Question 124

Signs of a life threatening acute asthma attack?

Answer 124

Peak flow <33% predicted Saturations < 92% Exhaustion. and poor respiratory effort Hypotension Silent chest Cyanosis Altered conciousness/confusion

Question 125

How is acute asthma managed?

Answer 125

Mild; regular salbutamol such as 4-6 puffs every 4 hours Severe 1) Salbutamol, 10 puffs every 2 hours 2) Nebulised salbutamol/ ipratropium bromide 3) Oral prednisolone 4) IV hydrocortisone 5) IV magnesium sulphate 6) IV salbutamol 7) IV aminophylline It still not controlled consider ICU admission, intubation and ventilation

Question 126

What is a typical step down regime for inhaled salbutamol?

Answer 126

10 puffs every 2 hours then 10 puffs every 4 hours then 6 puffs every 4 hours then 4 puffs every 6 hours

Question 127

What electrolyte should be monitored when taking large doses of salbutamol?

Answer 127

Potassium Salbutamol causes potassium to shift from blood into cells

Question 128

What is chronic asthma?

Answer 128

Chronic inflammatory airway disease leading to variable airway obstruction as a result of hypersensitive airways

Question 129

Presentation of asthma?

Answer 129

Dry cough with wheeze, shortness of breath Episodic symptoms with intermittent exacerbations Diurnal variability; typically worse at night and early morning History of other atopic conditions Family history of asthma /atopy Bilateral widespread polyphonic wheeze Symptoms improve with bronchodilators

Question 130

Typical triggers for asthma?

Answer 130

Dust Animals Cold air Exercise Smoke Food allergens

Question 131

Investigations to diagnose asthma?

Answer 131

Clinical Spirometry and peak flow variability Fractional exhaled nitric oxide Direct bronchial challenge test

Question 132

Medical management of asthma under 5 years of age?

Answer 132

1) SABA 2) Add low close corticosteroid inhaler or oral montelukast 3) Add other option from step 2 4) Refer to specialist

Question 133

Medical management of asthma in children aged 5 to 12 years?

Answer 133

1) SABA 2) Low dose corticosteroid inhaler 3) LABA 4) Titrate up corticosteroid and consider adding either montelukast or theophylline 5) Increase dose of inhaled corticosteroid 6) Referral to specialist

Question 134

Medical management of asthma in children over the age of 12 years?

Answer 134

1) SABA 2) Regular low dose corticosteroid inhaler 3) LABA, continue only if good response 4) Titrate up corticosteroid inhaler to medium dose, consider trial of montelukast, theophylline or LAMA (tiotropium) 5) Titrate up corticosteroid inhaler to high dose 6) Referall to specialist and consider oral steroids

Question 135

Issues surrounding corticosteroids and growth?

Answer 135

Slows down growth in children Dose dependant effect

Question 136

Advice for cleaning spacer device?

Answer 136

Cleaned once a month Avoid scrubbing Allow to airdry to prevent static as this can prevent medication being inhaled

Question 137

What is pneumonia?

Answer 137

Infection of lung tissue

Question 138

Symptoms of pneumonia?

Answer 138

Wet and productive cough High fever Tachypnoea Tachycardia Increased work of breathing Lethargy Delerium

Question 139

Characteristic signs of pneumonia?

Answer 139

Bronchial breath sounds; harsh breath sounds equally lond on inspiration and expiration caused by consolidation of the lung tissue around airway Focal coarse crackles; air passing through sputum Dullness to percussion; lung tissue collapse/ consolidation

Question 140

Causes of pneumonia?

Answer 140

Most common; streptococcus pneumonia Bacterial; group A strep, group B strep, staphylococcus aureus, haemophilus influenzae, mycoplasma pneumonia Viral; RSV, parainfluenza virus, influenza virus

Question 141

Atypical cause of pneumonia?

Answer 141

Mycoplasma pneumonia

Question 142

Investigations to diagnose pneumonia?

Answer 142

Sputum culture, throat swab, bacterial culture, viral PCR

Question 143

Management of pneumonia?

Answer 143

Amoxicillin is first line antibiotic Macrolide ( erythromycin, clarythromycin, azithromycin) can be used to cover atypical causes or when allergic to penicillin

Question 144

What is croup?

Answer 144

Acute infective respiratory disease of upper airway causing oedema in larynx

Question 145

What age group does croup commonly affect?

Answer 145

6 months to 2 years

Question 146

Cause of croup?

Answer 146

Most common; parainfluenza virus Influenza Adenovirus RSV

Question 147

Presentation of croup?

Answer 147

Increased work of breathing Barking cough Hoarse voice Stridor Low grade fever

Question 148

What causative agent of croup used to be associated with high mortality?

Answer 148

Diptheria Leads to epiglottistis, vaccination has reduced incidence

Question 149

Management of croup?

Answer 149

Oral dexamethasone; 150 mcg/kg, can be repeated in 12 hours if needed Oxygen Nebulised adrenaline, budesonide

Question 150

What is epiglottitis?

Answer 150

Inflammation and swelling of the epiglottis which can swell and completely occlude airway resulting in life threatening complication

Question 151

Cause of epiglottistis?

Answer 151

Haemophilus influenzae type B

Question 152

Presentation of epiglottitis?

Answer 152

Sore throat Stridor Difficulty/ Painful swallowing Drooling Tripod position Scared and quiet child High fever

Question 153

Investigations to diagnose Acute epiglottitis?

Answer 153

Should not perform investigations as this can startle child and cause airway obstruction A lateral view neck X-ray would show thumb sign, and useful in ruling out foreign body

Question 154

Management of epiglottitis?

Answer 154

Not to distress child Ensure airway is secure; may require intubation or tracheostomy IV antibiotics; ceftriaxone and steroids

Question 155

Prognosis of epiglottitis?

Answer 155

Most children recover not requiring intubation Common complication is epiglottic abscess

Question 156

What is laryngomalacia?

Answer 156

The supraglottic larynx (above the vocal cords) is structured in a way to cause partial airway obstruction

Question 157

Pathophysiology of laryngomalacia?

Answer 157

Tissues surrounding supraglottic larynx is softer and has less tone meaning it can flop across the airway, particularly during inspiration across the airway to partially occlude it, which generates characteristic whistling sound

Question 158

Peak age of laryngomalacia?

Answer 158

6 months

Question 159

Presentation of laryngomalacia?

Answer 159

Inspiratory stridor; harsh whistling sound when breathing in Usually intermittent and more prominent when feeding, upset, lying on back or during URTI

Question 160

Management of laryngomalacia?

Answer 160

Usually resolves as larynx matures and is better adapted to support itself In severe cases surgery can be performed to support larynx

Question 161

What is whooping cough?

Answer 161

URTI caused by bordetella pertussis, a gram negative bacteria

Question 162

Presentation of whooping cough?

Answer 162

Coryzal symptoms with low grade fever More severe coughing fits develop Large, loud inspiratory whoop Following coughing can lead to fainting, vomiting

Question 163

Investigations to diagnose whooping cough?

Answer 163

Nasal swabs with PCR/ bacterial culture within 2-3 weeks of the onset of symptoms Anti-pertussis toxin immunoglobulin G if symptoms present for more than 2 weeks

Question 164

Management of whooping cough?

Answer 164

Notify public health Supportive care Macrolides can be useful in first 21 days Prophylactic antibiotics to vunerable close contacts

Question 165

Complications of whooping cough?

Answer 165

Bronchiectasis

Question 166

What is chronic lung disease of prematurity?

Answer 166

Bronchopulmonary dysplasia Occurs in premature babies born before 28 weeks suffering with respiratory distress syndrome requiring oxygen therapy

Question 167

Features of chronic lung disease of prematurity?

Answer 167

Low oxygen saturations Increased work of breathing Poor feeding and weight gain Crackles and wheeze Increased susceptibility to infection

Question 168

How can chronic ling disease of prematurity be prevented?

Answer 168

Corticosteroids to mothers showing signs of pre-term labour Use CPAP rather than intubation and ventilation Use caffeine to stimulate respiratory effort Not to over oxygenate with supplemental oxygen

Question 169

Management of chronic lung disease of prematurity?

Answer 169

Sleep study to assess oxygen sats during sleep to guide treatment Palivizumab injection for RSV protection

Question 170

What is cystic fibrosis?

Answer 170

Autosomal recessive condition affecting CFTR gene on chromosome 7 affecting mucus glands and secretion of thick mucus

Question 171

What isthe most common mutation in cystic fibrosis?

Answer 171

delta- F508

Question 172

Consequences of cystic fibrosis mutation?

Answer 172

Thick pancreatic and biliary secretions; lack of enzyme secretions in digestive tract Low volume thick airway secretions; reduced airway secretion resulting in bacterial colonisation Congenital bilateral absence of the vas deferens

Question 173

What is often the first sign of cystic fibrosis?

Answer 173

Meconium ileus

Question 174

Symptoms of cystic fibrosis?

Answer 174

Chronic cough Thick sputum production Recurrent respiratory tract infection Lose greasy stools Abdominal pain and bloating Poor weight and height gain

Question 175

Signs of cystic fibrosis?

Answer 175

Low weight or height on growth charts Nasal polyps Finger clubbing Crackles / wheeze Abdominal distention

Question 176

Causes of clubbing in children?

Answer 176

Hereditary clubbing Cyanotic heart disease Infective endocarditis Cystic fibrosis Tuberculosis Inflammatory bowel disease Liver cirrhosis

Question 177

How is cystic fibrosis diagnosed?

Answer 177

Newborn blood spot test Sweat test Genetic tost

Question 178

Gold standard test to diagnose Cystic fibrosis?

Answer 178

Sweat test; chloride concentration more than 60 mmol/ L

Question 179

Examples of microbial colonisers in CF patients?

Answer 179

Staphylococcus aureus Haemophilus influenzae Klebsiella pneumoniae Escherichia coli Burkhodheria cepacia Pseudomonas aeruginosa

Question 180

How is colonisation with pseudomonas aeruginosa managed?

Answer 180

Nebulised antibiotic long term such as tobramycin Oral ciprofloxacin

Question 181

Management of cystic fibrosis ?

Answer 181

Chest physiotherapy; To clear mucus Exercise to improve lung function High calorie diet CREON tablets; replace pancreatic secretions Prophylactic flucloxacillin Treat chest infections Nebulised hypertonic saline and DNAase: reduce viscosity Vaccinations

Question 182

What vaccinations are given to CF patients?

Answer 182

pneumococcal, influenza, varicella

Question 183

Monitoring requirements for patients with CF?

Answer 183

Every 6 weeks Sputum cultures for colonisation Screening for diabetes, osteoporosis, vitamin D deficiency, liver failure

Question 184

Prognosis of CF?

Answer 184

median life expectancy 47 years 90% of patients develop pancreatic insufficiency 50% of adults develop CF related diabetes 30% of adults develop CF related liver disease

Question 185

Why does cystic fibrosis cause male infertility?

Answer 185

Congenital bilateral absence of the vas deferens

Question 186

What is primary ciliary dyskinesia ( Kartagner syndrome ) ?

Answer 186

Autosomal recessive condition causing dysfunctional motility of the cilia, mostly affecting those in respiratory tract

Question 187

Pathophysiology of Kartagner's syndrome?

Answer 187

Dysfunctional motility of cilia leads to build up of mucus in the lungs creating a suitable environment for chest infections with frequent and chronic chest infections Also affects motility of cillia in fallopian tubes and sperm tails leading to fertility issues Similar presentation to CF

Question 188

What is Kartagner's traid?

Answer 188

Paranasal sinusitis Bronchiectasis Situs inversus

Question 189

What is situs inversus?

Answer 189

all internal organs are located in mirrored locations inside the body

Question 190

Association of primary ciliary dyskinesia and situs inversus?

Answer 190

25% of patients with situs inversus have PCD 50% of patients with PCD have situs inversus

Question 191

Investigations to diagnose Kartagner's syndrome?

Answer 191

Family history; consanguinity Clinical presentation Biopsy of ciliated epithelium

Question 192

Management of Kartagner's syndrome?

Answer 192

Similar to CF with physiotherapy, high calorie diet, antibiotics

Question 193

Causes of abdominal pain in children?

Answer 193

Constipation UTI Coeliac disease IBD Mesenteric adenitis Abdominal migraine Pyelonephritis Henoch-Schonlein purpura Tonsilitis Diabetic ketoacidosis Infantile colic

Question 194

Surgical causes of abdominal pain in children?

Answer 194

Appendicitis Intussuscepticn Bowel obstruction Testicular torsion Ectopic pregnancy

Question 195

Red flags for abdominal pain in children?

Answer 195

Persistent/ bilious vomit Severe chronic diarrhoea Fever Rectal bleeding Weightloss / faltering growth Dysphagia Nighttime pain Abdominal pain

Question 196

What is abdominal migraine?

Answer 196

Episodes of central abdominal pain lasting more than an hour Associated symptoms; nausea, vomiting, anorexia, pallor, headache, photophobia, aura

Question 197

What is pizotifen used for and what are the concerns?

Answer 197

Prevention of abdominal migraine Slow stopping as abrupt discontinuation can lead to withdrawal symptoms

Question 198

Causes of constipation in children?

Answer 198

Idiopathic Hirschprung's disease cystic fibrosis Hypothyroidism

Question 199

Presentation of constipation in children?

Answer 199

Less than 3 stools per week Hard stools that are difficult to pass Straining and painful passage of stool Abdominal pain Rectal bleeding

Question 200

What is Encopresis?

Answer 200

Faecal incontinence Considered pathological if after age of 4 years Usually caused by overstretching of rectum

Question 201

Causes of encopresis?

Answer 201

Spina bifida Hirschprung Cerebral palsy Learning disability Psychosocial stress Abuse

Question 202

Causes of desensitisation of rectum?

Answer 202

Hirschprung Cystic fibrosis Hypothyroidism Spinal cord lesions Sexual abuse Intestinal Obstraction Anal stenosis Cow milk protein

Question 203

Complications of constipation?

Answer 203

Pain Reduced sensation Anal fissures Haemorrhoids Overflow and soiling Psychosocial issues

Question 204

What laxative is used first line for constipation?

Answer 204

Movicol

Question 205

Red flags for constipation?

Answer 205

Not passing meconium; cystic fibrosis, hirschsprung disease Neurological signs especially in lower limbs; cerebral palsy, spinal cord lesion Vomiting; intestinal obstruction, hirschsprungs disease Ribbon stool; anal stenosis Abnormal anus; anal stenosis, IBD, sexual abuse Abnormal lower back/ buttocks; spina bifida, spinal cord lesion Failure to thrive; coeliac disease, hypothyroidism, safeguarding

Question 206

Presentation of GORD?

Answer 206

Chronic cough Hoarse cry Distress, crying or unsettled after feeding Reluctance to feed Pneumonia Poor weight gain

Question 207

Causes of vomiting?

Answer 207

Overfeeding Appendicitis GORD UTI, tonsilitis, meningitis Pyloric stenosis Gastritis/ gastroenteritis Intestinal obstruction Bulimia

Question 208

Management of GORD?

Answer 208

Small frequent meals Burping Not to over-feed Keep baby upright Gaviscon mixed with feeds Thickened milk/ formula Ranitidine or omeprazole Surgical fundoplication can be considered in very severe cases

Question 209

What is Sandifer's syndrome?

Answer 209

Brief episodes of abnormal movements associated with GORD in infants torticollis; foreceful contractions of the neck muscles causing twitching of the neck Dystonia; abnormal muscle contractions causing twisting movements, arching of the back or unusual postures

Question 210

What is pyloric stenosis

Answer 210

Hypertrophy of the pylorus of the stomach resulting in powerful peristalsis that ultimately pushes food up the oesophagus

Question 211

Features of pyloric stenosis?

Answer 211

Projectile vomit Olive shaped mass in abdomen Failing to thrive

Question 212

What does blood gas show in pyloric stenosis?

Answer 212

Hypochloric Metabolic alkalosis Due to vomiting of stomach acid

Question 213

Management of pyloric stenosis?

Answer 213

Laparoscopic pyloromyotomy - Ramstedt's operation

Question 214

Red flags for vomiting?

Answer 214

Not keeping any feed down; pyloric stenosis, intestinal obstruction Projectile vomit; pyloric stenosis, intestinal obstruction Bile stained vomit Haematemesis, melena Abdominal distention Reduced conciousness, bulding fontanelle

Question 215

What is the most common cause of gastroenteritis in children?

Answer 215

Viral

Question 216

Differentials in children with diarrhoea?

Answer 216

Infection IBD Lactose intolerance Coeliac disease Cystic fibrosis Toddler's diarrhoea IBS Medications

Question 217

Causes of viral gastroenteritis?

Answer 217

Rotavirus Norovirus Adenovirus - more associated with subacute illness

Question 218

What does E.coli 0157 produce?

Answer 218

Shiga toxin which causes abdominal cramps, bloody diarrhoea and vomiting

Question 219

What is associated with E.coli 0157 infection?

Answer 219

Shiga toxin destroys red blood cells and can lead to haemolytic uraemic syndrome

Question 220

Why should antibiotics be avoided if E. coli gastroenteritis is suspected?

Answer 220

Antibiotics increase risk of haemolytic uraemic syndrome

Question 221

How is E.coli transmitted?

Answer 221

Infected faeces Unwashed salads Contaminated water

Question 222

How is campylobacter jejuni transmitted?

Answer 222

Raw or improperly cooked poultry Untreated water Unpasteurised milk

Question 223

Antibiotic of choice when treating campylobacter jejuni gastroenteritis?

Answer 223

Azithromycin Ciprofloxacin

Question 224

Symptoms of campylobacter jejuni gastroenteritis and incubation period?

Answer 224

Incubation; 2-5 days Symptoms; abdominal pain, diarrhoea, vomiting, fever

Question 225

Principles of managing gastroenteritis?

Answer 225

Barrier nursing and rigorous infection control Faecal sample tested with microscopy, culture and sensitivities Ensure well hydrated status and consider electrolyte replacement with dioralyte Antidiarrhoeal and antiemetic is not generally recommended

Question 226

What is coeliac disease?

Answer 226

Autoimmune reaction triggered by exposure to gluten resulting in inflammation in the small bowel

Question 227

Pathophysiology of coeliac disease?

Answer 227

Exposure to gluten results in autoantibodies created against gluten Auto antibodies target epithelial cells leading to inflammation Inflammation leads to malabsorption of nutrients

Question 228

Presentation of coeliac disease?

Answer 228

Failure to thrive Diarrhoea Fatigue Weightloss Mouth ulcers Anaemia secondary to iron, B12, folate deficiency Dermitis herpetiformis

Question 229

Genetic associations with coeliac disease?

Answer 229

HLA - DQ2 HLA- DQ8

Question 230

Auto- antibodies present in coeliac disease?

Answer 230

Tissue transglutaminase- anti- TTG Endomysial antibodies - anti endomysial Deaminated gliadin peptide antibodies

Question 231

Investigations to diagnose coeliac disease?

Answer 231

Bloods; check antibodies when patient is eating gluten Endoscopy and biopsy

Question 232

What is seen on biopsy in coeliac disease?

Answer 232

Crypt hypertrophy Villous atrophy

Question 233

Diseases associated with coeliac disease?

Answer 233

Type I diabetes mellitus Thyroid disease Autoimmune hepatitis Primary biliary cirrhosis Primary sclerosing cholangitis Down's syndrome

Question 234

Complications of untreated coeliac disease?

Answer 234

Vitamin deficiency /anaemia Osteoporosis Ulcerative jejunitis Enteropathy- associated T-cell lymphoma of the intestine Non- Hodgkin lymphoma Small bowel adenocarcinoma

Question 235

Extra intestinal manifestations of IBD?

Answer 235

Finger clubbing Erythema nodorsum Pyoderma gangrenousum Episcleritis/ Iritis Inflammatory arthritis Primary sclerosing cholangitis (UC)

Question 236

Investigations performed in IBD?

Answer 236

Bloods Faecal calprotectin Endoscopy+ biopsy Imaging to look for complications

Question 237

Management of Crohn's disease?

Answer 237

Induce/ maintain remission; Steroids; Monitor nutrition, growth If inadequet response add immunosuppression; first line is azathioprine/ mercaptopurine alternatives are methotrexate, infliximab, adalimumab When combination of steroids and immunosepressants induce remission continue maintaince therapy Surgical resection, if disease only affects distal ileum resection can reduce disease flares

Question 238

Management of ulcerative collitis?

Answer 238

Induce remission for mild to moderate disease; First line; aminosalicylate Second line; corticosteroid Induce remission for severe disease; First line; IV corticosteroid Second line; IV ciclosporin Maintaining remission; Aminosalicylate, Azathioprine, Mercaptopurine Surgery Only affects colon, total pan proctocolectomy with ileostomy or J-pouch can cure disease

Question 239

What is biliary atresia?

Answer 239

Congenital narrowing of the bile duct resulting in cholestasis

Question 240

What is the key finding in biliary atresia?

Answer 240

High conjugated bilirubin'

Question 241

Management of biliary atresia?

Answer 241

Surgery; Kasai portoenterostomy Sometimes patients require a full liver transplant

Question 242

Causes of intestinal obstruction?

Answer 242

Meconium ileus Hirschsprung's disease Oesophageal atresia Duodenal atresia Intussusception Malrotation with volvulus Strangulated hernia

Question 243

Presentation of intestinal obstruction?

Answer 243

Persistent vomiting, may be bilious Abdominal pain with distention Failure to pass stool or wind Abnormal bowel sounds; high pitched tinkling sounds, may also be absent

Question 244

Investigations to diagnose intestinal obstruction?

Answer 244

Abdominal Xray

Question 245

Management of bowel obstruction?

Answer 245

Refferal to surgery Nil by mouth Insert NG tube IV fluids

Question 246

What is hirschsprung's disease?

Answer 246

Congenital absence of the nerves of the myenteric plexus in the distal bowel and rectum which affects bowel peristalsis

Question 247

Pathophysiology of hirschsprung's disease?

Answer 247

Absence of parasympathetic ganglion cells due to incomplete migration from proximal bowel during embryonic development Aganglionic section does not under go peristalsis leading to obstruction

Question 248

Genetic associations of hirschsprung's disease?

Answer 248

Down's syndrome Neurofibromatosis Waardenburg syndrome MEN II

Question 249

Presentation of hirschsprung disease?

Answer 249

Delay in passing meconium Chronic constipation since birth Abdominal pain and distention Vomiting Poor weight gain and failure to thrive

Question 250

What is Hirschsprung associated enterocollitis?

Answer 250

Inflammation and obstruction of intesting occuring around 20% of neonates affected with hirschsprung

Question 251

Presentation of Hirschsprung associated enterocollitis?

Answer 251

2-4 weeks after birth Fever Abdominal distention Diarrhoea often bloody Can lead to toxic mogacolon

Question 252

Management of Hirschsprung?

Answer 252

surgical removal of aganglionic bowel

Question 253

Investigations to diagnose hirschspring's?

Answer 253

Abdominal x-ray Rectal biopsy

Question 254

Associations of Intussusception?

Answer 254

Concurrent viral Illness Henoch- Schoniein purpura. cystic fibrosis Intestinal polyps Meckel's diverticulum

Question 255

Presentation of Intussusception?

Answer 255

Severe colicy abdominal.pain Pale, lethargic, unwell child Redcurrent jelly stool RUQ mass ; Sausage shaped Vomit

Question 256

Complications of bowel obstruction?

Answer 256

Obstruction Gangrenous bowel Perforation Death

Question 257

How is insulin administered in type 1 diabetes?

Answer 257

combination of long acting, given once a day and short acting injected 30 minutes before intake of carbohydrates

Question 258

What is the name of the Insulin regime?

Answer 258

Basal bolus regime Basal- long acting, usually taken at night Bolus - short acting taken around times of meals

Question 259

Types of insulin pumps?

Answer 259

Tethered pump Patch pump

Question 260

What is an insulin pump?

Answer 260

Device that continuously infuses insulin at different rates

Question 261

Inclusion criteria for use of insulin pump?

Answer 261

Over 12 years Difficulty controlling HbA1c

Question 262

What is a tethered insulin pump?

Answer 262

device with replaceable infusion sets and insulin

Question 263

What is a patch pump?

Answer 263

Sits directly on skin and needs full replacement when pump runs out of insulin

Question 264

Symptoms of a hypoglycaemic event?

Answer 264

Hunger Tremor Sweating Irritability Dizziness Pallor Reduced conciousness

Question 265

Management of hypoglycaemia?

Answer 265

Rapid acting glucose such as lucozade and slower acting Severe cuses can be given IV 10% dextrose or IM glucagon

Question 266

How is Type I diabetes monitored in children?

Answer 266

HbAlc every 3-6 months in a red top EDTA bottle Capillary blood glucose or flash glucose monitoring

Question 267

What is diabetic ketoacidosis?

Answer 267

Life threatening emergency experienced by type 1 diabetics due to absolute lack of insulin Is a common way for undiagnosed T1DM to present in children

Question 268

When does ketogenesis occur?

Answer 268

Glucose stores have been exhausted so fatty acids are converted to ketones to be used as fuel

Question 269

Pathophysiology of diabetic ketoacidosis?

Answer 269

Lack of insulin leads to ketogenesis making blood more acidic, leading to dehydration and potassium imbalance

Question 270

What happens to the potassium balance in DKA?

Answer 270

Insulin normally drives potassium into cells so in DKA potassium is high

Question 271

Why are children with DKA at risk of cerebral oedema?

Answer 271

In DKA hyperglycaemia and hyperkalaemia lead to water leaving cells and entering extracellular space, rapid correction of dehydration can cause cerebral oedema

Question 272

Presentation of DKA?

Answer 272

Polyuria Polydipsia Nausea and vomiting Weight loss Acetone smell in breath Dehydration Altered consciousness Symptoms of an underlying trigger

Question 273

What is required for diagnosis of DKA?

Answer 273

Hyperglycaemia; 11mmol/l Ketosis; >3mmol/l Acidosis; ph <7.3

Question 274

Management of DKA in children?

Answer 274

Correct dehydration over 48 hours Give fixed rate insulin infusion Avoid fluid bolus, correct potassium, monitor for signs of cerebral oedema

Question 275

What is adrenal insufficiency?

Answer 275

Lack of steroid hormones produced by the adrenal glands, particularly cortisol and aldosterone

Question 276

Types of adrenal insufficiency?

Answer 276

Addisons disease; specific type of primary insufficiency as a result of autoimmune attack Secondary insufficiency; inadequate ACTH secretion from pituitary gland due to hypoplasia of pitutiary, surgery, infection, radiotherapy Tertiary insufficiency; inadequate CRH release from hypothalamus

Question 277

Features of adrenal insufficiency in babies?

Answer 277

Lethargy Vomiting Poor feeding Hypoglycaemia Jaundice Failure to thrive

Question 278

Features of adrenal insufficiency in children?

Answer 278

Nausea and vomiting Poor weight gain/ weight loss Reduced appetite Abdominal pain Muscle weakness/ cramps Developmental delay/ poor academic performance Bronze hyperpigmentation

Question 279

Investigations performed to diagnose adrenal insufficiency?

Answer 279

U+E; hyponatraemia, hyperkalaemia, hypoglycaemia Synacthen test

Question 280

Management of adrenal insufficiency?

Answer 280

Replace cortisol; hydrocortisol Replace mineralocorticoid; Fludrocortisone Patient is given steroid card and emergency ID tag

Question 281

Monitoring requirement for children diagnosed with adrenal insufficiency?

Answer 281

Regular review by paediatric specialist to monitor for; Growth and development Blood pressure U+E Glucose Bone profile Vitamin D

Question 282

How should steroids be adjusted during a time of acute illness?

Answer 282

Increase steroid dose Has risk of hypoglycaemia so ensure high carbohydrate diet

Question 283

Presentation of addisonian crisis?

Answer 283

Reduced conciousness Hypotension Hypoglycaemia

Question 284

Management of addisonian crisis?

Answer 284

Parenteral steroids IV fluids Correct hypoglycaemia, hyponatraemia, hyperkalaemia

Question 285

What is congenital adrenal hyperplasia?

Answer 285

Congenital deficiency of 21-hydroxylase enzyme causing an underproduction of cortisol and aldosterone and overproduction of androgens

Question 286

What is the mode of inheritance of congenital adrenal hyperplasia?

Answer 286

Autosomal recessive

Question 287

Pathophysiology of congenital adrenal hyperplasia?

Answer 287

21-hydroxylase enzyme is responsible for converting progesterone to aldosterone and cortisol. Hence progesterone is converted into excess amounts of testosterone resulting in low aldosterone and cortisol with high testosterone

Question 288

Presentation of CAH?

Answer 288

Poor feeding, vomiting, dehydration, arrhythmia, skin hyperpigmentation Female patients; tall for their age, facial hair, absent periods, deep voice, early periods Male patients; Tall for age, deep voice, large penis, small testicles, early puberty

Question 289

Management of CAH?

Answer 289

Hydrocortisone, Fludrocortisone replacement Females with virilised genitals may require corrective surgery

Question 290

What is growth hormone deficiency?

Answer 290

Disruption of growth hormone axis

Question 291

Causes of growth hormone deficiency?

Answer 291

Congenital; GH1 mutation, GHRHR mutation Empty sella syndrome Acquired deficiency; infection, trauma, iatrogenic

Question 291

Presentation of growth hormone deficiency in neonates?

Answer 291

Micropenis Hypoglycaemia Severenjaundice

Question 292

Presentation of growth hormone deficiency in children?

Answer 292

Poor growth, severely slowing from age 2-3 Short stature Slow development of movement and strength Delayed puberty

Question 293

Investigations to diagnose growth hormone deficiency?

Answer 293

Growth hormone stimulation test MRI Xray Genetic test

Question 294

Management of growth hormone deficiency?

Answer 294

Daily SC growth hormone (somatropin) Close monitoring of height and development

Question 295

Symptoms of a UTI in babies?

Answer 295

Fever Lethargy Irritability Vomiting Poor feeding Urinary frequency

Question 296

Symptoms of UTI in children?

Answer 296

Fever Abdominal pain/ suprapubic pain Vomiting Dysuria Urinary frequency Incontinence

Question 297

What is the criteria for diagnosing acute pyelonephritis?

Answer 297

Temperature greater than 38 Loin pain/ tenderness

Question 298

Investigations to diagnose UTI?

Answer 298

Urine dip; nitrates, leukocytes Mid stream sample for cultures and sensitivity

Question 299

Management of UTI in children?

Answer 299

Children under 3 months; IV antibiotics; ceftriaxone Children over 3 months; oral antibiotics; Trimethoprim, Nitrofurantoin, Cefalexin, Amoxicillin

Question 300

Investigations for recurrent UTI?

Answer 300

Children under 6 months with first UTI should have USS within 6 weeks If infected with atypical antibiotics should have USS during time of illness DMSA scan; 4-6 months from illness to assess damage from recurrent/ atypical UTI

Question 301

What is vesico-ureteric reflux?

Answer 301

Tendency of urine to flow back from bladder into ureter which increases risk of upper urinary tract infection and subsequent renal scarring

Question 302

How is vesico-ureteric reflux diagnosed?

Answer 302

Micturating cystourethrogram (MCUG)

Question 303

Management of vesico-ureteric reflux?

Answer 303

Avoid constipation Avoid an excessively full bladder Prophylactic antibiotics Surgical input from paediatric urology

Question 304

What is an MCUG?

Answer 304

Used to investigate atypical/ recurrent UTI in children under 6 months

Question 305

What is vulvovaginitis?

Answer 305

Inflammation and irritation of the vulva and vagina affecting girls between ages of 3 and 10 years

Question 306

Risk factors for vulvovaginitis?

Answer 306

Wet nappies Use of chemicals or soaps in cleaning the area Tight clothing that traps moisture or sweat in the area Constipation Threadworm Pressure on the area, e.g horse riding Heavy chlorinated pools

Question 307

How does puberty affect incidence of vulvovaginitis?

Answer 307

Oestrogen keeps vaginal mucosa healthy and restraint to infection

Question 308

Presentation of vulvovaginitis?

Answer 308

Soreness Itching Erythema around the labia Vaginal discharge Dysuria Constipation

Question 309

How to differentiate UTI and vulvovaginitis?

Answer 309

Urine dipstick will show leukocytes but no nitrates

Question 310

Management of vulvovaginitis?

Answer 310

No medical management just conservative measures to improve symptoms Avoid washing with soap and chemicals Avoid perfumed or antiseptic products Good toilet hygiene Keep the area dry Emollients Loose cotton clothing Treat constipation and worms Avoid activities that worsen problem

Question 311

What is the most common age for nephrotic syndrome?

Answer 311

2-5 years old

Question 312

Classic triad for nephrotic syndrome? and other signs and symptoms

Answer 312

Hypoalbuminaemia Proteinuria Oedema De-ranged lipid profile Hypertension Hyper-coaguability

Question 313

Most common cause of nephrotic syndrome in children?

Answer 313

Minimal change disease

Question 314

Causes of nephrotic syndrome?

Answer 314

Intrinsic kidney disease; Focal segmental glomerulosclerosis, membranoproliferative glomerulonephritis Secondary causes; Henoch- Schonlein purpura, diabetes, Infections

Question 315

Pathophysiology of nephrotic syndrome?

Answer 315

Increased permeability of the glomerular basement membrane allowing proteins to leave blood and enter urine

Question 316

Management of minimal change disease?

Answer 316

High dose steroids (In steroid resistant cases ACE-i and immunosuppressants are used) Low salt diets Diuretics Albumin infusions Antibiotic prophylaxis in severe cases

Question 317

What is the regimen of steroids in nephrotic syndromes?

Answer 317

High dose steroids given for 4 weeks and slowly weened over the next 8 weeks

Question 318

Complications of nephrotic syndrome?

Answer 318

Hypovolaemia Thrombosis Infection Acute/ chronic renal failure Relapse

Question 319

What is nephritis?

Answer 319

Inflammation of the nephrons of the kidney

Question 320

Triad of nephritic syndrome?

Answer 320

Haematuria Hypertension Protienuria

Question 321

Common cause of nephritic syndrome in children?

Answer 321

Post-streptococcal glomerulonephritis IgA nephropathy (Berger disease)

Question 322

Pathophysiology of post-streptococcal glomerulonephritis?

Answer 322

1-3 weeks following infection with beta haemolytic streptococcus Streptococcal antigen immune complexes, antibodies and complement proteins attach to kidney glomeruli and cause inflammation leading to acute kidney injury

Question 323

What is IgA nephropathy?

Answer 323

AKA Berger's disease where IgA deposits in nephrons of the kidney leading to inflammation

Question 324

What is seen on biopsy in IgA nephropathy?

Answer 324

IgA deposits Glomerular mesangial proliferation

Question 325

Management of nephritic syndrome?

Answer 325

Supportive treatment Antihypertensive Immunosuppression; steroids and immunosuppresants

Question 326

What is haemolytic uraemic syndrome?

Answer 326

Thrombosis within small vessels throughout the body triggered by the shiga toxin

Question 327

Triad of Haemolytic uraemic syndrome?

Answer 327

Haemolytic anaemia AKI Thrombocytopenia

Question 328

Aetiology of haemolytic uraemic syndrome?

Answer 328

Shiga toxin Most commonly produced by E.coli 0157, shigella

Question 329

Signs and symptoms of haemolytic uraemic syndrome?

Answer 329

Reduced urine output Haematuria Abdominal pain Lethargy and irritability Confusion Oedema Hypertension Bruising

Question 330

Prognosis of haemolytic uraemic syndrome?

Answer 330

Medical emergency and 10% mortality

Question 331

Management of HUS?

Answer 331

Antihypertensives Maintain fluid balance Blood transfusion and dialysis if required

Question 332

By what age do children have control over urination?

Answer 332

Daytime; 2 years Night time; 3-4 years

Question 333

What is primary nocturnal enuresis?

Answer 333

When a child has never managed to be consistently dry at night

Question 334

Causes of primary nocturnal enuresis?

Answer 334

Variation on normal development Overactive bladder Fluid intake prior to bedtime Failure to wake due to deep sleep or underdeveloped bladder signals Psychological distress Secondary causes

Question 335

Investigations to manage enuresis?

Answer 335

2 week diary of toileting, fluid intake, bedwetting episodes

Question 336

Management of primary nocturnal enuresis?

Answer 336

Reassurance in children under 5 years Lifestyle changes; reduce fluid intake before bed, go to toilet before sleeping Encouragement and positive re-inforcement Treat underlying cause and exacerbating factors Enuresis alarms Pharmacological treatment

Question 337

What is secondary nocturnal enuresis?

Answer 337

A child who has previously been dry at night for atleast 6 months begins bed wetting

Question 338

Causes of secondary nocturnal enuresis?

Answer 338

UTI Constipation T1DM New psychosocial problems Maltreatment

Question 339

What is diurnal enuresis?

Answer 339

Daytime incontinence

Question 340

Causes of diurnal enuresis?

Answer 340

Urge incontinence Stress incontinence Recurrent UTI Psychosocial problems Constipation

Question 341

Medical management of enuresis?

Answer 341

Desmopressin Oxybutynin Imipramine

Question 342

Which type of polycystic kidney disease presents in children?

Answer 342

Autosomal recessive

Question 343

Cause of ARPKD?

Answer 343

Mutation in polycystic kidney and hepatic disease 1 (PKHD 1) gene on chromosome 6 which codes for fibrocystin/ polyductin protein complex which creates tubules and maintains healthy epithelium in kidney, liver and pancreas

Question 344

Features of ARPKD?

Answer 344

Cystic enlargement of renal collecting duct Oligohydramnios Pulmonary hypoplasia Potter syndrome Congenital liver fibrosis Usually can can polycystic kidneys on antenatal scans

Question 345

What causes potter syndrome?

Answer 345

Oligohydramnios

Question 346

Characteristics of Potter syndrome?

Answer 346

Underdeveloped ear cartilage Low set ears Flat nasal bridge Skeletal abnormalities

Question 347

Prognosis of ARPKD?

Answer 347

Poor; around 1/3 will die as a neonate and 1/3 will not make it to adulthood Oligohydramnios leads to poor development of lungs Large cystic kidneys take up too much space and make breathing difficult Many require dialysis in first few days of life and then are in end stage renal failure before reaching adulthood

Question 348

Co-morbidities associated with ARPKD?

Answer 348

Liver failure Portal hypertension Progressive renal failure Hypertension Chronic lung disease

Question 349

What is Wilms tumour?

Answer 349

Tumour of kidneys affecting children usually under the age of 5

Question 350

Presentation of Wilms tumour?

Answer 350

Abdominal pain Haematuria Lethargy Fever Hypertension Weight loss

Question 351

Investigations for Wilms tumour?

Answer 351

Ultrasound scan CT/ MRI; staging Biopsy; histology and make definitive diagnosis

Question 352

Management of Wilms tumour?

Answer 352

Surgical excision and adjuvant therapy

Question 353

What is a posterior urethral valve?

Answer 353

Tissue at the proximal end of the urethra causes obstruction to urine output creating back pressure into the bladder, ureters and up to kidney causing hydronephrosis

Question 354

Presentation of posterior urethral valve?

Answer 354

Difficulty urinating Weak urinary stream Chronic urinary retention Palpable bladder Recurrent UTI Impaired kidney function In severe cases can cause bilateral hydronephrosis in-utero and is seen on USS

Question 355

Investigations to diagnose posterior urethral valve?

Answer 355

Abdominal USS; enlarged thickened bladder, bilateral hydronephrosis MCUG; shows location of extra-urethral tissue Cystoscopy

Question 356

Management of posterior urethral valve?

Answer 356

Ablation/ removal of excess tissue

Question 357

Risk factors for undescended testes?

Answer 357

Family history Low birth weight, SGA, Premature Maternal smoking during pregnancy

Question 358

At what age is referral needed for undescended testes?

Answer 358

6 months

Question 359

Management of undescended testes?

Answer 359

Orchidopexy

Question 360

What is hypospadias?

Answer 360

Urethral meatus is abnormally displaced posteriorly on the penis

Question 361

Management of hypospadias?

Answer 361

Do not circumsise infant until urologist has seen Surgical correction

Question 362

Complications of hypospadias?

Answer 362

Difficulty directing urination Cosmetic/ psychological concerns Sexual dysfunction

Question 363

Differentials for scrotal swelling?

Answer 363

Hydrocele Partially descended testes Inguinal hernia Testicular torsion Haematoma Tumour

Question 364

What is a hydrocele?

Answer 364

Collection of fluid in tunica vaginalis

Question 365

Types of hydrocele?

Answer 365

Simple hydrocele fluid collection is reabsorbed over time Communicating hydrocele; connection between peritoneum and scrotum called processus vaginalis allows fluid to collect in testes

Question 366

Management of hydrocele?

Answer 366

Most resolve spontaneously For large; surgical ablation of processus vaginalis

Question 367

Issues surrounding neonatal resuscitation?

Answer 367

Large surface area to weight ratio so get cold quickly Babies are born wet so lose heat rapidly Babies born through meconium may have it in their mouth/ airway

Question 368

Principles of neonatal resuscitation?

Answer 368

Warm the baby Calculate APGAR score Stimulate breathing Inflation breaths

Question 369

How can the baby be kept as warm as possible after delivery?

Answer 369

Dry baby as quickly as possible, vigorous drying can help stimulate Place under heat lamps Babies under 28 weeks are placed in plastic bag

Question 370

How can breathing be stimulated in the neonate?

Answer 370

Keep head in neutral position to keep airway open Place towel under shoulders

Question 371

How are inflation breaths provided to neonate?

Answer 371

When neonate is gasping or not breathing despite stimulation Two cycles of 5 inflation breaths lasting 5 seconds, if no response then 30 seconds of ventilation breaths, if still no response then start chest compressions coordinated with ventilation breaths

Question 372

What is the APGAR score?

Answer 372

Score ranging from 0 to 10 rating 5 features of newborn Appearance Pulse Grimmace Activity Respiration

Question 373

What are the benefits of delayed umbilical cord clamping?

Answer 373

Placental transfusion, can help improve iron stores, improve blood pressure, reduction in intraventricular haemorrhage and reduction in necrotising enterocollitis

Question 374

What are the negatives of delayed cord clamping?

Answer 374

Risk of neonatal jaundice requiring phototherapy

Question 375

What happens to the neonates straight after birth?

Answer 375

Skin to skin Clamp umbilical cord Dry the baby Keep baby warm with hat and blanket Vitamin K injection Label baby Measure height and weight

Question 376

Why is vitamin K given to newborn?

Answer 376

Vitamin K is needed for clotting and babies are born deficient Helps reduce haemorrhage, especially through umbilical stump

Question 377

Benefits of skin to skin contact?

Answer 377

Keeps baby warm Improves mother and baby interaction Calms baby Improves breast feeding

Question 378

When is the NIPE completed?

Answer 378

Within 72 hours from birth

Question 379

When is the blood spot screening test completed?

Answer 379

day 5, maximum day 8

Question 380

What conditions are screened for in the heel prick test?

Answer 380

Sickle cell disease Cystic fibrosis Congenital hypothyroidism Phenylketonuria Medium chain acyl- CoA dehydrogenase deficiency Maple syrup urine disease Isovaleric acidaemis Glutaric aciduria type 1 Homocystin

Question 381

How long do results of heel prick test take?

Answer 381

6-8 weeks

Question 382

When is the NIPE examination performed?

Answer 382

72 hours post birth 6-8 weeks by GP

Question 383

What is the purpose of the NIPE examination?

Answer 383

Pick up abnormalities in the newborn

Question 384

What is caput succedaneum?

Answer 384

Fluid collection in the scalp outside the periosteum due to pressure on a specific area on the scalp during traumatic, prolonged or instrumental delivery

Question 385

Management of caput succedaneum?

Answer 385

Nothing, usually resolves in a few days

Question 386

What is a cephalohaematoma?

Answer 386

Collection of blood between skull and periosteum caused by damage to blood vessels during traumatic, prolonged or instrumental delivery

Question 387

How is caput succedaneum differentiated from cephalohaematoma?

Answer 387

In cephalohaematoma does not cross suture lines and causes discolouration of affected skin

Question 388

Management of cephalohaematoma?

Answer 388

Usually resolves over a couple of months Monitor for anaemia and jaundice

Question 389

What is facial paralysis?

Answer 389

Birth injury usually associated with forceps delivery due to facial nerve injury

Question 390

Prognosis of facial paralysis as a result of birth injury?

Answer 390

Function returns to normal in a couple of weeks If function does not return, then referral to neurologist is needed

Question 391

What is Erb's palsy?

Answer 391

Injury to C5/C6 nerves of the brachial plexus during birth

Question 392

Risk factors for Erb's palsy?

Answer 392

Shoulder dystocia Traumatic birth Instrumental delivery Large birth weight

Question 393

Presentation of Erb's palsy?

Answer 393

Weakness of shoulder abduction and external rotation, arm flexion and finger extension leading to waiter tip appearance

Question 394

Prognosis of Erb's palsy?

Answer 394

Function returns spontaneously within a few months If not then specialist input may be required

Question 395

Risk factors for clavicle fracture?

Answer 395

Shoulder dystocia Traumatic delivery Instrumental delivery Large birth weight

Question 396

Signs on examination indicating fractured clavicle?

Answer 396

Noticeable lack of movement/ asymmetry of movement in the affected arm Asymmetry of the shoulders with affected shoulder lower than normal Pain and distress on movement of the arm

Question 397

What is neonatal sepsis?

Answer 397

Infection in neonatal period

Question 398

Causative agents of neonatal sepsis?

Answer 398

Group B streptococcus E.coli Listeria Klebsiella Staphylococcus aureus

Question 399

Risk factors for neonatal sepsis?

Answer 399

Vaginal GBS colonisation GBS sepsis in previous baby Maternal sepsis, chorioamnionitis, fever over 38 Prematurity Premature rupture of membranes Prolonged rupture of membranes

Question 400

Features of neonatal sepsis?

Answer 400

Fever Reduced tone and activity Poor feeding Respiratory distress Vomiting Tachycardia/ Bradycardia Hypoxia Jaundice within 24 hours Seizures Hypoglycaemia

Question 401

Red flags for neonatal sepsis?

Answer 401

Confirmed/ suspected sepsis in mother Signs of shock Seizures Term baby needing mechanical ventilation Respiratory distress staring more than 4 hours after birth Presumed sepsis in another baby in a multiple pregnancy

Question 402

Management of neonatal sepsis?

Answer 402

one RF for sepsis; monitor for 12 hours two or more RF or red flag start antibiotics Take blood cultures before antibiotics Baseline FBC and CRP

Question 403

Antibiotic of choice for neonatal sepsis?

Answer 403

Benzylpenicillin, gentamycin Cefotaxime can be used in lower risk

Question 404

Monitoring requirements for neonatal sepsis?

Answer 404

CRP in 24 hours Blood cultures in 36 hours Repeat CRP at 5 days

Question 405

What is hypoxic ishcaemic encephalopathy?

Answer 405

Neurological malfunction as a result of hypoxia during child birth resulting in ischaemic damage to the brain tissue which can lead to permanent neurological damage

Question 406

When should you suspect hypoxic ishcaemic encephalopathy?

Answer 406

Events during perinatal/ intrapartum period which could lead to hypoxia Acidosis in umbilical artery blood gas Poor APGAR score

Question 407

Causes of hypoxic ishcaemic encephalopathy?

Answer 407

Maternal shock Intrapartum haemorrhage Prolapsed cord Nuchal cord

Question 408

What staging system is used to grade hypoxic ishcaemic encephalopathy?

Answer 408

Sarnat staging

Question 409

Grading of hypoxic ishcaemic encephalopathy?

Answer 409

Mild; Poor feeding, generally irritable, hyper-alert, resolves within 24 hours, normal prognosis Moderate; Poor feeding, lethargic, hypotonic, seizures, can take weeks to resolve, up to 40% develop cerebral palsy Severe; Reduced consciousness, apnoea, flaccid, reduced/ absent reflexes, up to 50% mortality, upto 90% develop cerebral palsy

Question 410

Management of hypoxic ishcaemic encephalopathy?

Answer 410

Coordinated by neonatology Supportive care with ventillation, circulatory support, nutrition, acid base balance Therapeutic hypothermia

Question 411

What is the benefit of therapeutic hypothermia?

Answer 411

Reduce inflammation and neurone loss after acute hypoxic injury and helps reduce risk of cerebral palsy, developmental delay, learning disability, blindness, death

Question 412

Pathophysiology of jaundice?

Answer 412

Rapid foetal RBC haemolysis leads to build up of bilirubin

Question 413

Causes of neonatal jaundice?

Answer 413

Increased production of bilirubin; Haemolytic disease of newborn, ABO incompatibility, Haemorrhage, Intraventricular haemorrhage, Cephalo-haematoma, Polycythaemia, Sepsis and DIC, G6PD deficiency Reduced bilirubin clearnace; Prematurity, breast milk jaundice, Neonatal cholestasis, Extrahepatic biliary atresia, Endocrine disorders, Gilbert syndrome

Question 414

At which point in the neonatal period is jaundice always pathological?

Answer 414

In the first 24 hours

Question 415

What is defined as prolonged jaundice?

Answer 415

More than 14 days in full term babies More than 21 days in pre-term babies

Question 416

Causes of prolonged jaundice?

Answer 416

Biliary atresia Hypothyroidism G6PD deficiency

Question 417

Investigations performed in neonatal jaundice?

Answer 417

FBC and blood film Conjugated bilirubin Blood type test Direct coombes test TFT Blood and urine culture G6PD levels

Question 418

Management of neonatal jaundice?

Answer 418

Supportive management Treat cause Phototherapy

Question 419

What is Kernicterus?

Answer 419

Type of brain damage as a result of excessive bilirubin levels

Question 420

Pathophysiology of Kernicterus?

Answer 420

Bilirubin crosses BBB and directly damages CNS presenting with less responsive, floppy, drowsy baby This damage is permanent

Question 421

What can Kernicterus lead to?

Answer 421

Cerebral palsy Learning difficulty Deafness

Question 422

Risk factors for prematurity?

Answer 422

Social deprivation Smoking, alcohol, drugs Overweight/ underweight mother Maternal co-morbidities Twins Personal/ family history of prematurity

Question 423

How can prematurity be prevented if symptoms show before 24 weeks?

Answer 423

Prophylactic vaginal progesterone Prophylactic cervical cerclage

Question 424

When pre-term birth is confirmed what can be done to improve outcomes?

Answer 424

Tocolysis with nifedipine Maternal corticosteroids before 35 weeks IV MgSO4 before 34 weeks Delayed cord clamping

Question 425

Issues in early life of neonates with prematurity?

Answer 425

Respiratory distress syndrome Hypothermia Hypoglycaemia Poor feeding Apnoea and bradycardia Neonatal jaundice Intraventricular haemorrhage Retinopathy of prematurity Necrotising enterocolitis Immature immune system and infection

Question 426

Long term effects of prematurity?

Answer 426

Chronic lung disease of prematurity Learning and behavioral difficulties Susceptible to infections Hearing and visual impairment Cerebral palsy

Question 427

What is apnoea?

Answer 427

Periods of spontaneous cessation of breathing for more than 20 seconds with oxygen desaturation, bradycardia

Question 428

Causes of apnoea?

Answer 428

Immaturity of autonomic nervous system and can be an indicator of developing illness; Infection Anaemia Airway obstruction CNS pathology GORD Neonatal abstinence syndrome

Question 429

Management of apnoea?

Answer 429

Attach neonate to apnoea monitor Tactile stimulation IV caffeine

Question 430

What is retinopathy of prematurity?

Answer 430

Abnormal development of blood vessels in the retina leading to scarring, retinal detachment and blindness

Question 431

Who is at risk of developing retinopathy of prematurity?

Answer 431

Those born before 32 weeks

Question 432

Pathophysiology of retinopathy of prematurity?

Answer 432

Retinal vessel formation is stimulated by hypoxia, so higher concentrations of oxygen after delivery means stimulus for vessel development is removed When hypoxic environment returns abnormal vessel formation along with scar tissue formation Abnormal vessels may regress and leave retina with no blood supply

Question 433

What is plus disease?

Answer 433

additional findings of retinopathy such as tortuous vessels, hazy vitreous humour

Question 434

Treatment for retinopathy of prematurity?

Answer 434

Transpupillary laser photocoagulation Cryotherapy Intravitreal VEGF inhibitors Surgery

Question 435

What is seen on chest X-ray in respiratory distress syndrome?

Answer 435

Ground glass appearance

Question 436

Pathophysiology of respiratory distress syndrome?

Answer 436

Inadequate surfactant release leads to high surface tension in alveoli leading to atelectasis making gas exchange difficult leading to respiratory distress and type 2 respiratory failure

Question 437

Management of respiratory distress syndrome?

Answer 437

Antenatal steroids for women in pre-term labour Neonates may require intubation and ventilation, endotracheal surfactant, CPAP and supplemental oxygen

Question 438

At what gestation does respiratory distress syndrome commonly occur?

Answer 438

before 32 weeks

Question 439

What is necrotising enterocolitis?

Answer 439

necrosis of premature bowel which is a life threatening emergency leading to bowel perforation and death

Question 440

Risk factors for developing necrotising enterocolitis?

Answer 440

Very low birth weight/ very premature Formula fed Respiratory distress and assisted ventilation Sepsis PDA/ other congenital heart defect

Question 441

Presentation of necrotising enterocolitis?

Answer 441

Intolerance to feed Vomiting, green bilious Generally unwell Distended tender abdomen Absent bowel sounds Blood in stool If perforated presents with peritonitis, shock

Question 442

Investigations to diagnose necrotising enterocolitis?

Answer 442

FBC; thrombocytopenia, neutropenia CRP; inflammation Capillary blood gas Blood culture Abdominal X-ray; dilated loops of bowel, bowel wall oedema, pneumatosis intestinalis, pneumoperitoneum

Question 443

Management of neonatal necrotising enterocollitis?

Answer 443

Nil by mouth, IV fluids and TPN Antibiotics Surgery to remove necrotic bowel

Question 444

Complications of neonatal enterocollitis?

Answer 444

Perforation, peritonitis Sepsis Death Strictures Abscess formation Recurrence Long term stoma Short bowel syndrome

Question 445

What is neonatal abstinence syndrome?

Answer 445

Withdrawal symptoms experienced by neonates born to mothers who used substances during pregnancy

Question 446

Substances that cause neonatal abstinence syndrome?

Answer 446

Opiates Methadone Benzodiazepines Cocaine Amphetamines Nicotine Cannabis Alcohol SSRI antidepressants

Question 447

When do withdrawal symptoms present?

Answer 447

Opiates, diazepam, SSRI, alcohol; 3-72 hours after birth Methadone, benzodiazepines; 24 hours to 21 days

Question 448

Presentation of neonatal abstinence syndrome?

Answer 448

CNS; irritability, increased tone, high pitched cry, not settling, tremors, seizures Vasomotor and respiratory; Yawning, sweating, unstable temperature, tachypnoea Metabolic and gastrointestinal; poor feeding, regurgitation, vomiting, hypoglycaemia, loose stools and sore nappy area

Question 449

Management of neonatal abstinence syndrome?

Answer 449

Keep babies for at least 3 days, test urine sample Consider weaning treatment Medical management options; phenobarbitone

Question 450

Additional considerations for neonates with abstinence syndrome?

Answer 450

Testing for hepatitis B, C and HIV Safeguarding and social services Follow-up with relevant professionals Maternal support to stop using substance Check suitability for breastfeeding

Question 451

Alcohol in early pregnancy can lead to?

Answer 451

Miscarriage Small for dates baby Preterm delivery

Question 452

Features of fetal alcohol syndrome?

Answer 452

Microcephaly Thin upper lip Smooth flat philtrum Short palpable fissure Learning disability Behavioral difficulty Hearing and vision problems Cerebral palsy

Question 453

What is sudden infant death syndrome?

Answer 453

Sudden, unexplained death of infant

Question 454

Risk factors for SIDS?

Answer 454

Prematurity Low birth weight Smoking during pregnancy Male baby

Question 455

What is juvenile idiopathic arthritis?

Answer 455

Autoimmune inflammation of the joints affecting children and adolescents

Question 456

What is the key presentation of inflammatory arthritis?

Answer 456

Joint pain Swelling Stiffness

Question 457

Subtypes of juvenile idiopathic arthritis?

Answer 457

Systemic JIA Polyarticular JIA Oligoarticular JIA Enthesitis related arthritis Juvenile psoriatic arthritis

Question 458

Presentation of Still's disease (Systemic JIA)?

Answer 458

Salmon pink rash High swinging fever Enlarged lymph nodes Weight loss Joint inflammation and pain Splenomegaly Muscle pain Pleuritis/ pericarditis

Question 459

Blood results in systemic JIA?

Answer 459

anti-ANA, RF; negative Raised CRP, ESR, platelets and serum ferritin

Question 460

Complication of systematic JIA?

Answer 460

Macrophage activation syndrome severe activation of the immune system with a massive inflammatory response

Question 461

Presentation of macrophage activation syndrome?

Answer 461

Acutely unwell child DIC Anaemia Thrombocytopenia Bleeding Non blanching rash

Question 462

What is a key finding in macrophage activation syndrome?

Answer 462

Low ESR

Question 463

What is polyarticular JIA?

Answer 463

Seronegative Idiopathic arthritis affecting 5 or more joints in the body Tends to be symmetrical

Question 464

Presentation of polyarticular JIA?

Answer 464

Symmetrical joint involvement Mild fever Anaemia Reduced growth

Question 465

What is the paediatric equivalent of rheumatoid arthritis?

Answer 465

Polyarticular JIA

Question 466

What is oligoarticular JIA?

Answer 466

Inflammation of 4 or less joints

Question 467

Epidemiology of oligoarticular JIA?

Answer 467

Affects girls more than boys Tends to affect large joints

Question 468

Associations of oligoarticular JIA?

Answer 468

Anterior uveitis

Question 469

Blood results in oligoarticular JIA?

Answer 469

anti ANA positive RF negative

Question 470

What is enthesitis related arthritis?

Answer 470

Inflammatory joint arthritis with enthesitis

Question 471

Epidemiology of enthesitis related arthritis?

Answer 471

More common in male children over 6 years

Question 472

Genetic association of enthesitis related arthritis?

Answer 472

HLA- B27

Question 473

Associations of enthesitis related arthritis?

Answer 473

Psoriasis IBD Anterior uveitis?

Question 474

Presentation of enthesitis related arthritis

Answer 474

Localised tenderness of entheses; Interphalengeal joint of hands Wrist Greater trochanter Quadricep insertion on ASIS Around patella Base of achilles at the calcaneous Metatarsal head

Question 475

What is juvenile psoriatic arthritis?

Answer 475

Seronegative inflammatory arthritis associated with psoriasis presenting with symmetrical small joint polyarthritis and asymmetrical arthritis affecting large joints

Question 476

Signs of juvenile psoriatic arthritis?

Answer 476

Plaques of psoriasis on skin Nail pitting Oncholysis Dactylitis Enthesitis

Question 477

Management of JIA?

Answer 477

NSAIDs; ibuprofen Steroids DMARD; methotrexate, sulfasalazine, leflunomide Biological therapy; TNF-inhibitors (etanercept, infliximab, adalimumab)

Question 478

What is Ehlers- Danlos syndrome?

Answer 478

Group of genetic conditions causing defects in collagen resulting in hypermobility of joints and connective tissue defects

Question 479

Types of Ehlers- Danlos syndrome?

Answer 479

Hypermobile; joint hypermobility, soft stretchy skin Classical ; smooth, velvety stretchy skin, severe joint hypermobility and abnormal wound healing Vascular; most dangerous, fragile blood vessels, thin translucent skin Kyphoscoliotic; hypotonia, as child grows kyphoscoliosis

Question 480

Mode of inheritance of Ehlers- Danlos syndrome?

Answer 480

Autosomal dominant for all other types apart from hypermobile Hypermobile has no single mode of inheritance

Question 481

Most common presentation of Ehlers- Danlos syndrome?

Answer 481

Hypermobile Ehlers- Danlos syndrome

Question 482

Presentation of Ehlers- Danlos syndrome?

Answer 482

Joint pain Hypermobility in joints Joint dislocation Soft stretchy skin Easy bruising and poor wound healing Bleeding Headache Autonomic dysfunction GORD Abdominal pain, IBS Menorrhagia, dysmenorrhoea Premature rupture of membranes in pregnancy Urinary incontinence Pelvic organ prolapse TMJ dysfunction Myopia

Question 483

What is the use of the Beighton score?

Answer 483

Assess extent of hypermobility

Question 484

Components of the Beighton score?

Answer 484

Palms flat on the floor with straight legs (1 point) Elbows hyperextended Knees hyperextended Thumbs can bend and touch forearm Little finger hyperextends past 90 degres

Question 485

Management of Ehlers- Danlos syndrome?

Answer 485

No cure Physiotherapy to strengthen and stabilise joints Occupational therapy to maximise function Maintain good posture in joints Moderate intensity of exercise

Question 486

Complications of hypermobility?

Answer 486

Increased wear and tear of joints so generalised osteoarthritis at an earlier age

Question 487

Association of hypermobile Ehlers- Danlos syndrome?

Answer 487

POTS Due to autonomic dysfunction

Question 488

What is Henoch- Schonlein Purpura?

Answer 488

IgA vasculitis presenting with purpuric rash usually triggered by URTI or gastroenteritis

Question 489

Epidemiology of HSP?

Answer 489

Often previous URTI or gastroenteritis Commonly affects children under 10 years

Question 490

Features of HSP?

Answer 490

Purpura; purple in colour typically on legs and buttock Joint pain; affects 3/4 of patients typically affecting knee and ankle joint Abdominal pain; 50% of patients and can lead to haemorrhage, intussusception and infarction Renal involvement; IgA nephritis

Question 491

Investigations to diagnose HSP?

Answer 491

FBC; thrombocytopenia, leukopenia U+E- kidney function Serum albumin CRP, Blood cultures Urine dipstick and PCR Blood pressure

Question 492

Criteria to diagnose HSP?

Answer 492

Palpable purpura and one more of the following; Diffuse abdominal pain Arthritis/ arthralgia IgA deposits on histology Proteinuria or haematuria

Question 493

Management of HSP?

Answer 493

Supportive; analgesia Steroids can be considered in severe cases of GI or renal involvement Close monitoring of urine dipstick and BP

Question 494

Prognosis of HSP?

Answer 494

Abdominal pain settles in a few days and those with no renal involvement will make full recovery 1/3 of patients have recurrence in 6 months and some develop ESRF

Question 495

What is Kawasaki disease?

Answer 495

Systemic vasculitis of medium sized vessels

Question 496

Epidemiology of kawasaki disease?

Answer 496

Under 5 years More common in Asian chidlren; Japanese and Korean More common in boys

Question 497

Presentation of Kawasaki disease?

Answer 497

Persistent high fever for more than 5 days Widespread erythematous maculopapular rash Cervical lymphadenopathy Bilateral conjunctivitis Strawberry tongue Cracked lips

Question 498

Investigations to diagnose Kawasaki disease?

Answer 498

FBC; anaemia, leukocytosis, thrombocytosis LFT; hypoalbuminaemia, elevated liver enzymes ESR; raised Urinalysis; leukocytes Echo; coronary artery aneurysm

Question 499

Phases of Kawasaki disease?

Answer 499

Acute; unwell, fever, rash, lymphadenopathy, lasting 1-2 weeks Subacute; desquamation, arthralgia, risk of coronary artery aneurysm forming, 2-4 weeks Convalescent; symptoms disappear, coronary artery aneurysm regress

Question 500

Management of Kawasaki disease?

Answer 500

High dose aspirin IVIG- reduce risk of coronary artery aneurysm

Question 501

Complication of Kawasaki disease?

Answer 501

Coronary artery aneurysm

Question 502

What is rheumatic fever?

Answer 502

Autoimmune condition triggered by streptococcus bacteria affecting joints, heart, skin and nervous system

Question 503

Pathophysiology of rheumatic fever?

Answer 503

Infection with group A beta haemolytic streptococcus triggers production of antibodies against bacteria, Immune cross reaction results in these antibodies attacking cells of immune system

Question 504

Presentation of rheumatic fever?

Answer 504

Joint involvement; arthritis affecting large joints Heart involvement; tachycardia, bradycardia, mitral valve disease, pericardial rub, heart failure Skin; subcutaneous nodules, erythema marginatum

Question 505

Investigations to diagnose rheumatic fever

Answer 505

Throat swab Anti streptococcal titres Echo, ECG, CXR

Question 506

What is the criteria used to diagnose rheumatic fever?

Answer 506

Jones criteria

Question 507

What is the course of anti streptococcal antibody titres?

Answer 507

Rise over 2-4 weeks Peaks around 3-6 weeks Gradually fall over 3-12 months

Question 508

What are the components of the Jones criteria?

Answer 508

Requires two major or one major and 2 minor criteria Major criteria; Joint arthritis Organ inflammation Nodules Erythema marginatum Sydenham chorea Minor; Fever ECG changes Arthralgia without arthritis Raised inflammatory markers

Question 509

Management of rheumatic fever?

Answer 509

NSAIDs Aspirin and steroids Prophylactic antibiotics Monitor for complications

Question 510

Complications of rheumatic fever?

Answer 510

Recurrence of rheumatic fever Mitral stenosis Chronic heart failure

Question 511

What is eczema?

Answer 511

Chronic atopic condition caused by defects in skin barrier leading to inflammation of the skin

Question 512

Pathophysiology of eczema?

Answer 512

Defects in skin barrier provides gaps for irritants, microbes and allergens to enter and stimulate an immune response resulting in inflammation

Question 513

Presentation of eczema?

Answer 513

Dry, red, itchy, sore patches on flexor surfaces of limbs , face and neck Periods to flares

Question 514

Management of eczema?

Answer 514

Maintenance with emollients Topical steroids

Question 515

Steroid ladder used to treat eczema?

Answer 515

Hydrocortisone; 0.5, 1%, 2.5% Eumovate; clobetasone butyrate 0.05% Betnovate; betamethasone 0.1% Dermovate; clobetasol propionate 0.05%

Question 516

What is eczema herpeticum?

Answer 516

Viral skin infection caused by HSV, VZV

Question 517

Causes of eczema herpeticum?

Answer 517

Herpes simplex virus Varicella zoster virus

Question 518

Risk factors for eczema herpeticum?

Answer 518

Dermatitis Eczema

Question 519

How is rash is in eczema herpeticum described?

Answer 519

Erythematous, painful, itchy rash with vesicles and pus After they burst the leave small punched out ulcers

Question 519

Presentation of eczema herpeticum?

Answer 519

Widespread pain vesicular rash Fever, lethargy, irritability Reduced oral intake Lymphadenopathy

Question 520

Management of eczema herpeticum?

Answer 520

Viral swab of vesicle Acyclovir

Question 521

Complication of eczema herpeticum?

Answer 521

Bacterial superinfection requiring antibiotics

Question 522

What is psoriasis?

Answer 522

Chronic autoimmune condition producing psoriatic skin lesions

Question 523

Description of psoriatic plaques?

Answer 523

Dry, flaky, scaly, rough, faintly erythematous lesions that appear in raised plaques Found on extensor surfaces

Question 524

Types of psoriasis?

Answer 524

Plaque; Thickened plaques with silver scales Guttate; Small raised papules across trunk and limbs, spontaneously resolves within 3-4 months, triggered by streptococcal throat infection Pustular; pustular lesions under skin, most severe form with systemic symptoms requiring hospital admission Erythodermic; extensive erythematous inflammation covering most surface area of skin resulting in ulceration requiring admission

Question 525

Most common type of psoriasis in children?

Answer 525

Guttate

Question 526

Signs specific to psoriasis?

Answer 526

Auspitz sign; small points of bleeding when plaques are scraped off Koebner phenomenon; development of psoriasis in areas of trauma Residual pigmentation; after lesion resolves

Question 527

Management of psoriasis?

Answer 527

Topical steroids Topical vitamin D Topical dithranol Phototherapy If all topical treatments failed unlicensed medications can be trialed such as oral methotrexate, cyclosporine, retinoids or biologic medications

Question 528

What additional treatment options are available for psoriasis in adults?

Answer 528

Topical tarcolimus Vit D and steroid combo; Dovobet, Enstilar

Question 529

Associations of psoriasis?

Answer 529

Nail psoriasis Psoriatic arthritis Psychological implications

Question 530

Pathophysiology of acne?

Answer 530

Localised inflammation in pilosebaceous unit due to blockage and stagnant sebum providing nutrients for bacterial growth

Question 531

What bacteria contributes to acne?

Answer 531

propionibacterium acnes

Question 532

Step wise treatment for acne?

Answer 532

Topical benzoyl peroxide Topical retinoid Topical antibiotic such as clindamycin Oral antibiotic such as lymecycline Oral COCP; dianette

Question 533

Side effects of isotretinoin?

Answer 533

Dry skin and lips Photosensitivity Depression, anxiety, aggression, suicidal ideation Stevens Johnson syndrome/ Toxic epidermal necrolysis

Question 534

What is viral exanthem?

Answer 534

Widespread eruptive rash caused by 6 infections known as first, second, third, fourth, fifth and sixth disease

Question 535

Causes of viral exanthems?

Answer 535

First disease; Measels Second disease; Scarlet fever Third disease; Rubella Fourth disease; Duke's disease Fifth disease; Parovirus B19 Sixth disease; Roseola infantum

Question 536

Causes of measels?

Answer 536

Measels virus

Question 537

Presentation of measels?

Answer 537

Symptoms start 10-12 days after exposure Fever Coryzal symptoms Conjunctivitis Koplik spots; greyish white sports on buccal mucosa Rash starting on face, behind ear, and then spreads across body Symptoms resolve in 7 to 1- days

Question 538

Isolation requirements following measels?

Answer 538

4 days after symptoms resolve

Question 539

Complications of measels?

Answer 539

Pneumonia Diarrhoea Dehydration Encephalitis/ meningitis Hearing, vision loss Death

Question 540

Cause of scarlet fever?

Answer 540

Group A streptococcus exotoxin

Question 541

Presentation of scarlet fever?

Answer 541

Red-pink blotchy rough sandpaper like rash that starts on trunk and spreads Fever Lethargy Flushes face Sore throat Strawberry tongue Cervical lymphadenopathy

Question 542

Management of scarlet fever?

Answer 542

Phenoxymethylpenicillin for 10 days

Question 543

Exclusion period for scarlet fever?

Answer 543

24 hours after starting antibiotics

Question 544

Conditions associated with group A streptococcus?

Answer 544

Post streptococcal glomerulonephritis Acute rheumatic fever

Question 545

Presentation of rubella?

Answer 545

Erythematous macular rash starting on face and spreads to rest of the body lasting 3 days Mild fever, joint pain, sore throat Lymphadenopathy

Question 546

Exclusion period for rubella?

Answer 546

atleast 5 days after rash appears

Question 547

Complications of rubella?

Answer 547

Thrombocytopenia Encephalitis Congenital rubella syndrome

Question 548

What is Duke's disease?

Answer 548

AKA fourth disease No specific organism has been found to cause it.... may not even exist

Question 549

Presentation of Duke's disease?

Answer 549

Non specific viral rashes

Question 550

Other names for parovirus B19 infection?

Answer 550

Fifth disease Slapped cheek syndrome Erythemia infectiosum

Question 551

Presentation of Parovirus B19 infection?

Answer 551

Mild fever, coryza and non specific viral symptoms After 2-5 days rapidly occuring bright red rash appears on both cheeks Erythematous reticular rash appears on trunk and limbs Gradually fades over 1-2 weeks

Question 552

Exclusion period for parovirus B19?

Answer 552

None, once rash has appeared non infectious

Question 553

Complications of parovirus B19 infection?

Answer 553

Aplastic anaemia Encephalitis/ meningitis Pregnancy complications Rarely hepatitis, myocarditis, nephritis

Question 554

What is the cause of roseola infantum?

Answer 554

Human Herpes Virus-6 and HHV-7

Question 555

Course of roseola infantum?

Answer 555

Presents 1-2 weeks after infection with high fever that comes on suddenly and lasts 3-5 days and disappears Rash appears 1-2 days after fever consisting of mild erythematous macular rash across arms, legs, trunk and face which is not itchy

Question 556

Complication of roseola infantum?

Answer 556

Febrile convulsion Immunocompramised patients; Myocarditis, thrombocytopenia, GBS

Question 557

What is erythema multiforme?

Answer 557

Erythematous rash caused by hypersensitivity reaction

Question 558

Causes of erythema multiforme?

Answer 558

Viral infection Medication Herpes simplex virus Mycoplasma pneumonia

Question 559

Presentation of erythema multiforme?

Answer 559

Widespread erythematous itchy rash producing characteristic target lesions Sore mouth Mild fever, muscle ache, headache, general flu like symptoms

Question 560

Management of erythema multiforme?

Answer 560

Treat the cause IV fluid, analgesia, steroids

Question 561

What is utricaria?

Answer 561

Hives Small itchy lumps that appear on skin Classified as acute or chronic

Question 562

Pathophysiology of utricaria?

Answer 562

Release of histamine from mast cells resulting in skin changes

Question 563

Causes of acute utricaria?

Answer 563

Allergy to food, medication, animals Contact with chemicals, latex or stinging nettles Medications Viral infections Insect bites Dermatographism

Question 564

Causes of chronic utricaria?

Answer 564

Chronic idiopathic utricaria Chronic inducible utricaria; Sunlight Temperature change Exercise Strong emotion Hot/ cold weather Pressure Autoimmune

Question 565

Management of Utricaria?

Answer 565

Antihistamine; Fexofenadine Anti-leukotrienes; montelukast Omalizumab; targets IgE Cyclosporin

Question 566

What is the cause of chickenpox?

Answer 566

Varicella zoster virus

Question 567

Presentation of chickenpox?

Answer 567

Widespread erythematous, raised vesicular blistering lesions Rash starts on trunk and spreads outward over 2-5 days Fever Itchy General fatigue and malaise

Question 568

When is chickenpox no longer contagious?

Answer 568

When all lesions have scabbed over

Question 569

Complications of chickenpox?

Answer 569

Bacterial superinfection Dehydration Conjunctival lesions Pneumonia Encephalitis Lie dormant in sensory dorsal root ganglion cells and cranial nerve and reactivate as shingles or Ramsay Hunt syndrome

Question 570

Management of chickenpox?

Answer 570

Usually self limiting condition not requiring treatment Calamine lotion, chlorphenamine Aciclovir can be considered in immunocompromised or over 14 years

Question 571

School exclusion for chickenpox?

Answer 571

Until all lesions have crusted over, usually 5 days since rash appeared

Question 572

Cause of Hand, foot and mouth disease?

Answer 572

Coxsackie A virus

Question 573

Incubation period for Coxsackie A virus?

Answer 573

3-5 days

Question 574

Presentation of Hand, foot and mouth disease?

Answer 574

Starts as URTI with malaise, sore throat, dry cough and raised temperature After 1-2 days small mouth ulcers appear followed by blistering red spots across the body mostly on hand, foot Painful mouth ulcers, especially on tongue

Question 575

Management of Hand, foot and mouth disease?

Answer 575

Supportive, no treatment Monitor fluid intake, paracetamol

Question 576

Precautions with Hand, foot and mouth disease?

Answer 576

Highly contagious Avoid sharing sharing towels, bedding, Careful handling of nappies

Question 577

Complications of Hand, foot and mouth disease?

Answer 577

Dehydration Bacterial superinfection Encephalitis

Question 578

What is molluscum contagiosum?

Answer 578

Viral skin infection caused by molluscum contagiosum virus

Question 579

What type of virus is molluscum contagiosum?

Answer 579

Poxvirus

Question 580

Features of molluscum contagiosum?

Answer 580

Small flesh coloured papules with a central dimple Appear in crops of multiple lesions in a local area

Question 581

Prognosis of molluscum contagiosum?

Answer 581

Resolve on their own, but can take upto 18 months Scratching lesions can leave scars and spread infection

Question 582

Management of molluscum contagiosum in immunocompramised patients?

Answer 582

Topical potassium hydroxide, benzoyl peroxide, podophyllotoxin, imiquimod, tretinoin Surgical removal, cryotherapy

Question 583

What is pityriasis rosea?

Answer 583

Generalised, self limiting rash which may be caused by either HHV-6, HHV-7 but no definitive cause is found

Question 584

Presentation of pityriasis rosea?

Answer 584

Prodromal symptoms; headache, tiredness, loss of appetite and flu like symptoms Starts as herald patch; scaly pink/red oval rash >2cm usually somewhere on torso Then widespread scaly oval lesions appear Other symptoms; generalised itch, low grade pyrexia, headache, lethargy

Question 585

Course of disease in pityriasis rosea?

Answer 585

Rash resolves without treatment within 3 months

Question 586

Management of pityriasis rosea?

Answer 586

No treatment, patient education and reassurance Antihistamine to help with itching

Question 587

What is seborrhoeic dermatitis?

Answer 587

Inflammatory skin condition affecting sebaceous glands caused by malassezia yeast colonisation

Question 588

What is infantile seborrhoeic dermatitis?

Answer 588

Crusted flaky scalp which usually resolves by 4 months of age but can last upto 1 year

Question 589

Management of infantile seborrhoeic dermatitis?

Answer 589

Baby oil, olive oil to gently brush of flakes White petroleum jelly Topical antifungal; clotrimazole, miconazole

Question 590

What is seborrhoeic dermatitis of the scalp?

Answer 590

Dandruff

Question 591

Management of seborrhoeic dermatitis of the scalp?

Answer 591

Ketoconazole shampoo

Question 592

What is seborrhoeic dermatitis of the face and body?

Answer 592

Red, flaky, crusted, itchy skin commonly affecting eyelids, nasolabial folds, ears, upper chest and back

Question 593

What is ringworm?

Answer 593

Fungal infection of the skin with specific names based on which part of the body is affected Tinea capitis; ringworm of scalp Tinea pedis; feet, AKA athletes foot Tinea cruris; groin Tinea corporis; body Onychomycosis; fungal nail infection

Question 594

Most common fungus responsible for ringworm infection?

Answer 594

Trichophyton

Question 594

Presentation of ringworm?

Answer 594

Well demarcated itchy erythematous rash Thickened discoloured nails

Question 595

Management of ringworm?

Answer 595

Antifungal cream; cotimazole, miconazole Antifungal shampoo Oral antifungal; fluconazole, griseofluvin, itraconazole Mild topical steroid; Daktacort Fungal nail; amorolfine lacquer +/- oral terbinafine

Question 596

Composition of Daktacort?

Answer 596

2% miconazole 1% hydrocortisone cream

Question 597

What is tinea incognito?

Answer 597

Fungal skin infection as a result of steroid use to treat initial fungal infection

Question 598

Causes of nappy rash?

Answer 598

Bacterial; staphylococcus, streptococcus Fungus; candida

Question 599

What is a nappy rash?

Answer 599

Contact dermatitis in region of nappy

Question 600

Risk factors for nappy rash?

Answer 600

Delayed changing of nappies Irritant soap products and vigorous cleaning Poorly absorbent nappies Diarrhoea Oral Abx that predispose to candida infection Preterm infants

Question 601

Signs that would point to candida infection rather than simple nappy rash?

Answer 601

Rash extending into skin folds Large red macules Well demarcated scaly border Circular pattern to rash Satellite lesions

Question 602

Management of nappy rash?

Answer 602

Switch to highly absorbent nappy Change nappy and clean skin as soon as possible Use water or gentle alcohol free products Ensure nappy area is dry Maximise time not wearing nappy

Question 603

Complications of nappy rash?

Answer 603

Candida infection Cellulitis Jacquet's erosive diaper dermatitis Perianal psuedoverrucous papules and nodules

Question 604

What is scabies?

Answer 604

Tiny mites called sacroptes scabiei which burrow under the skin causing intense itching and infection

Question 605

Cause of scabies?

Answer 605

sacroptes scabiei

Question 606

Presentation of scabies?

Answer 606

Incredibly itchy, small, red spots Track marks from where mites may have burrowed Classically found in the webs of fingers

Question 607

Management of scabies?

Answer 607

Permethrin cream; apply to whole body and then wash 8-12 hours later Repeat treatment in one week Single dose oral ivermectin is last option for difficult to treat scabies Treat close contacts as disease is contagious, all clothes, bedsheets and linen should be washed

Question 608

What is cluster scabies?

Answer 608

Severe scabies infestation in those who are immunocompramised

Question 609

Cause of headlice?

Answer 609

Pediculus humanus capitis parasite

Question 610

Management of head lice?

Answer 610

Dimeticone 4% solution is left overnight then washed, repeat in 1 week

Question 611

What is the cause of a non-blanching rash?

Answer 611

Bleeding under the skin resulting from burst capillaries

Question 612

Causes of a non-blanching rash?

Answer 612

Meningococcal sepsis/ other bacterial sepsis Henoch Schonlein purpura Idipathic thrombocytopenic purpura Acute leukaemia Haemolytic uraemic syndrome Mechanical trauma; usually around neck and face Trauma; non accidental injury Viral illness

Question 613

Investigations to help identify cause of non blanching rash?

Answer 613

FBC U+E CRP ESR Coagulation screen Blood culture Meningococcal PCR Lumbar puncture Blood pressure Urine dipstick

Question 614

What is erythema nodosum?

Answer 614

Red lumps appear across patients shin due to inflammation of subcutaneous fat on patient shin due to hypersensitivity reaction

Question 615

Associations of erythema nodosum?

Answer 615

Streptococcal throat infection Gastroenteritis Mycoplasma pneumonia Tuberculosis Pregnancy Medications; oral contraceptives IBD Sarcoidosis Lymphoma/ leukaemia

Question 616

Presentation of erythema nodosum?

Answer 616

Red inflamed subcutaenous nodules across both shins Raised, painful tender nodules Over time nodules settle and appear as bruises Investigations may be required to diagnose cause

Question 617

Investigations to diagnose erythema nodosum?

Answer 617

Inflammatory markers Throat swab Chest Xray Stool microscopy/ culture Faecal calprotectin

Question 618

Management of erythema nodosum?

Answer 618

Investigations to find underlying cause Analgesia and steroids Usually resolves in 6 weeks

Question 619

What is impetigo?

Answer 619

Superficial skin infection caused by staphylococcus infection

Question 620

Classification of impetigo?

Answer 620

Bullous Non bullous

Question 621

Presentation of non bullous impetigo?

Answer 621

Occurs around nose or mouth Exudate forms golden crust No systemic symptoms

Question 622

Management of non bullous impetigo?

Answer 622

Topical fusidic acid Hydrogen peroxide 1% Oral flucloxicillin for severe cases

Question 623

School exclusion for non bullous impetigo?

Answer 623

Until lesions have crusted Until 48 hours since commencing antibiotics

Question 624

Presentation of bullous impetigo?

Answer 624

Fluid filled vesicles that grow and burst forming a golden crust More common in neonates and those under 2 years Systemic symptoms

Question 625

Management of bullous impetigo?

Answer 625

Oral/ IV flucloxicillin

Question 626

Complications of impetigo?

Answer 626

Cellulitis Sepsis Scarring Post streptococcal glomerulonephritis Staphylococcus scalded skin syndrome Scarlet fever

Question 627

What is staphylococcal scalded skin syndrome?

Answer 627

Epidermolytic toxins secreted by staph aureus are protease enzymes which damage skin

Question 628

Presentation of staphylococcal scalded skin syndrome?

Answer 628

Generalised patches of erythema Thin skin Nikolsky sign; gentle rubbing causes skin to peel away Systemic symptoms

Question 629

What is Nikolsky sign?

Answer 629

Nikolsky sign; gentle rubbing causes skin to peel away

Question 630

Treatment of staphylococcal scalded skin syndrome?

Answer 630

IV antibiotics

Question 631

What is Stevens- Johnson syndrome?

Answer 631

Immune response causes epidermal necrolysis resulting in blistering and shedding of top layer of skin

Question 632

Difference between Stevens- Johnson syndrome and toxic epidermal necrolysis?

Answer 632

Stevens- Johnson syndrome affects < 10% of BSA Toxic epidermal necrolysis affects >10%

Question 633

Causes of Stevens- Johnson syndrome?

Answer 633

Genetics; HLA Medications; anti-epileptics, antibiotics, allopurinol, NSAIDs Infection; Herpes simplex, mycoplasma pneumoniae, CMV, HIV

Question 634

Presentation of Stevens- Johnson syndrome?

Answer 634

Systemic illness symptoms Skin blistering Skin breaks leaving raw tissue exposed Eyes become inflammed and ulcerated

Question 635

Management of Stevens- Johnson syndrome?

Answer 635

Supportive care Steroids Immunoglobulins Immunosuppressant

Question 636

Complications of Stevens- Johnson syndrome?

Answer 636

Secondary infection Permanent skin damage Visual complications

Question 637

Most common cause of anaemia in infancy?

Answer 637

Physiological anaemia of infancy

Question 638

Causes of anaemia in infancy?

Answer 638

Physiological Anaemia of prematurity Blood loss Haemolysis; haemolytic disease of newborn, hereditary spherocytosis, G6PD deficiency Twin-twin transfusion

Question 639

Pathophysiology of physiological anaemia of infancy?

Answer 639

Normal dip in Hb at 6-9 weeks Negative feedback in response to high oxygen delivery suppressing erythropoietin

Question 640

Why are premature infant prone to anaemia compared to term infants?

Answer 640

Less time in-utero receiving iron from mother RBC production cannot keep up with rapid growth in first few weeks Blood tests remove significant volumes of blood

Question 641

Causes of anaemia in older children?

Answer 641

Iron deficiency anaemia Blood loss Sickle cell anaemia Thalassaemia Leukaemia Hereditary spherocytosis/ eliptocytosis Sideroblastic anaemia

Question 642

Causes of microcytic anaemia?

Answer 642

Thalassaemia Anaemia of chronic disease IDA Lead poisoning Sideroblastic anaemia

Question 643

Causes of normocytic anaemia?

Answer 643

Acute blood loss Anaemia of chronic disease Aplastic anaemia Haemolytic anaemia Hypothyroidism

Question 644

Causes of macrocytic anaemia?

Answer 644

Megaloblastic; B12, folate deficiency Normoblastic; Alcohol Reticulocytosis Hypothyroidism Liver disease Drugs such as azathioprine

Question 645

Symptoms of anaemia?

Answer 645

Tiredness Shortness of breath Headache Dizziness Palpitations Worsening of other conditions Pica and hairloss indicate IDA

Question 646

Signs of anaemia?

Answer 646

Pale skin Conjunctival pallor Tachycardia Raised respiratory rate Koilonychia, angular cheilitis, atrophic glossitis, brittle hair and nails, indicate IDA Jaundice; haemolytic anaemia Bone deformities; Thalassaemia

Question 647

Investigations performed for suspected anaemia?

Answer 647

FBC; Hb and MCV Blood film Reticulocyte count Ferritin B12 and folate Bilirubin Direct coombes test Hb electrophoresis

Question 648

Causes of iron deficiency anaemia?

Answer 648

Menorrhagia Low dietary intake Poor absorption; coeliac, IBD, IBS Helminth infection Medication; PPI

Question 649

Iron studies results on iron deficiency anaemia?

Answer 649

Low iron Low ferritin, can be raised if patient has infection Transferrin saturation; low

Question 650

Management of iron deficiency anaemia?

Answer 650

Treat underlying cause Ferrous sulphate/ ferrous fumarate

Question 651

Types of leukaemia which affect children?

Answer 651

ALL AML CML; rare

Question 652

Epidemiology of leukaemia?

Answer 652

ALL; peaks around age 2-3 years AML; peaks under age 2

Question 653

Risk factors for leukaemia?

Answer 653

Genetic; Down's syndrome, Kleinfelter, Noonan, Fanconi's anaemia Radiation exposure,

Question 654

Symptoms of leukaemia?

Answer 654

Persistent fatigue Unexplained fever Failure to thrive Weight loss Pallor Petechiae Unexplained bleeding Abdominal pain Generalised lymphadenopthy Unexplained persistent bone/ joint pain Hepatosplenomegaly

Question 655

Investigations to diagnose leukaemia?

Answer 655

FBC; pancytopenia, but high WCC of proliferating cell line Blood film; blast cells Bone marrow/ lymph node biopsy

Question 656

Management of paediatric leukaemia?

Answer 656

Chemotherapy is primary treatment Radiotherapy, bone marrow transplant or surgery can be considered

Question 657

Complications of chemotherapy in children?

Answer 657

Failure to treat leukaemia Stunted growth and development Immunodeficiency/ infections Neurotoxicity Infertility Secondary malignancy Cardiotoxicity

Question 658

Which paediatic leukaemia has a better prognosis ?

Answer 658

ALL

Question 659

What is idiopathic thrombocytopenic purpura?

Answer 659

Spontaneous low platelet count with a purpuric non blanching rash

Question 660

Pathophysiology of ITP?

Answer 660

Type II hypersensitivity reaction due to antibodies that target platelets

Question 661

Causes of ITP?

Answer 661

Viral infection trigger Idiopathic

Question 662

Presentation of ITP?

Answer 662

Bleeding Bruising Petechial rash - around 1mm Symptom onset 24-48 hours

Question 663

Epidemiology of ITP?

Answer 663

Children under 10 years History of recent viral illness

Question 664

Differentials for ITP?

Answer 664

Heparin induced thrombocytopenia Leukaemia

Question 665

Management of ITP?

Answer 665

Usually monitor until platelet count returns to normal Treatment if actively bleeding or severe thrombocytopenia <10; Prednisolone IVIG Blood transfusion Caution with activities which may cause bleeding, avoid NSAIDs, seek attention if injury

Question 666

Why are platelet infusions avoided in ITP?

Answer 666

Antibodies will destroy platelets

Question 667

Complications of ITP?

Answer 667

Chronic ITP Anaemia Intracranial and subarachnoid haemorrhage Gastrointestinal bleeding

Question 668

What is sickle cell anaemia?

Answer 668

Genetic condition producing crescent shaped red blood cells, making them more fragile leading to haemolytic anaemia

Question 669

Pathophysiology of haemolytic anaemia?

Answer 669

Abnormal Hb variant (HbS) affect beta globin chain causing Hb to polymerise and make red cells less stable

Question 670

Genetic change in sickle cell anaemia?

Answer 670

Single base mutation changing 6th amino acid to valine instead of glutamic acid

Question 671

Inheritance of sickle cell anameia?

Answer 671

Autosomal recessive

Question 672

Advantage of being a sickle cell carrier?

Answer 672

Reduced severity of malaria infection

Question 673

How is sickle cell anameia diagnosed?

Answer 673

Those women at higher risk are offered antenatal screening All newborns are screened during 5 day heel prick test

Question 674

Complications of sickle cell anaemia?

Answer 674

Anaemia Increased risk of infection Stroke Avascular necrosis of large joints Pulmonary hypertension Priaprsm CKD Sickle cell crises Acute chest syndrome

Question 675

Management of those with sickle cell?

Answer 675

Avoid dehydration and triggers for crisis Ensure vaccines are upto date Blood transfusion Bone marrow transplant can be curative

Question 676

Triggers for sickle cell crisis?

Answer 676

Infection Dehydration Cold Significant life events

Question 677

Management of sickle cell crisis?

Answer 677

Treat infection Keep warm Hydration Simple analgesia

Question 678

What is a vaso-occlusive crisis?

Answer 678

Painful crisis caused by blockage of capillaries typically caused by deydration Commonly results in priapism

Question 679

What is splenic sequestration crisis?

Answer 679

RBC block flow to spleen causing acute painful splenomegaly resulting in severe anaemia and hypovolaemic shock

Question 680

Management of splenic sequestration crisis?

Answer 680

Splenectomy

Question 681

What can recurrent splenic crisis lead to?

Answer 681

Splenic infarction

Question 682

What is an aplastic crisis?

Answer 682

Loss of RBC production most commonly triggered by parovirus B19 leading to severe anaemia

Question 683

Management of aplastic crisis?

Answer 683

Blood transfusion and supportive care

Question 684

Criteria to diagnose acute chest syndrome?

Answer 684

Fever/ respiratory symptoms New infiltrates seen on CXR

Question 685

Cause of acute chest syndrome?

Answer 685

Infection; pneumonia, bronchiolitis Pulmonary vaso occlusion

Question 686

Management of acute chest syndrome?

Answer 686

Supportive Antibiotics, antivirals Incentive spirometry Artificial ventilation

Question 687

Inheritance mode for thalassaemia?

Answer 687

Autosomal recessive

Question 688

Why do patients with thalassaemia have pronounced forehead and malar eminence?

Answer 688

Bone marrow expands to compensate for chronic anaemia

Question 689

Features of thalassaemia?

Answer 689

Microcytic anaemia Fatigue Pallor Jaundice Gallstone Splenomegaly Poor growth and development Pronounced forehead and malar eminence

Question 690

Investigations to diagnose thalassaemia?

Answer 690

FBC; microcytic anaemia Hb electrophoresis DNA testing

Question 691

Features of iron overload?

Answer 691

Fatigue Liver cirrhosis Infertility Impotence Heart failure Arthritis Diabetes Osteoporosis/ joint pain

Question 692

Cause of alpha thalassaemia?

Answer 692

Defect in alpha globin chain on chromosome 16

Question 693

Cause of beta thalassaemia?

Answer 693

Defect in beta globin chain on chromosome 11

Question 694

Types of beta thalassaemia?

Answer 694

Minor Intermedia Major

Question 695

Features of beta thalassaemia minor?

Answer 695

Carries one abnormal and one normal functioning gene Microcytic anaemia, typically only need monitoring

Question 696

Features of beta thalassaemia intermedia?

Answer 696

2 defective copies Causes more significant microcytic anaemia Require monitoring and occasional blood transfusions

Question 697

Features of beta thalassaemia major?

Answer 697

Homozygous for deletion, no functioning beta globin gene Results in severe microcytic anaemia, splenomegaly, bone deformities Require regular transfusion, iron chelation, splenectomy and bone marrow transfusion

Question 698

What is hereditary spherocytosis?

Answer 698

Sphere shaped red blood cells which are more fragile and easily destroyed as they pass through spleen

Question 699

Epidemiology of hereditary spherocytosis?

Answer 699

Most common form of haemolytic anaemia in northern europeans

Question 700

Presentation of hereditary spherocytosis?

Answer 700

Jaundice Anaemia Gallstones Splenomegaly Haemolytic crisis

Question 701

Presentation of haemolytic anaemia?

Answer 701

Significant haemolysis, anaemia, jaundice Usually triggered by infection

Question 702

How is hereditary spherocytosis diagnosed?

Answer 702

Family history and clinical features Blood film; spherocytes MCHC raised Raised reticulocytes

Question 703

Management of hereditary spherocytosis?

Answer 703

Folate supplementation Splenectomy Cholecystectomy Transfusions in acute crisis

Question 704

Inheritance patterns of hereditary spherocytosis/ elliptocytosis?

Answer 704

Autosomal dominant

Question 705

Inheritance for G6DP deficiency?

Answer 705

X-linked recessive

Question 706

Epidemiology of G6PD deficiency?

Answer 706

More common in males Triggered by infection, medication and fava beans

Question 707

Pathophysiology of G6PD deficiency?

Answer 707

G6PD enzyme protects from damage by ROS, deficiency of enzyme leads to damage to RBC and increased haemolysis

Question 708

Presentation of G6PD deficiency?

Answer 708

Neonatal jaundice Anaemia Intermittent jaundice Gallstones Splenomegaly

Question 709

Investigations to diagnose G6PD deficiency?

Answer 709

Blood film; Heinz body G6PD enzyme assay

Question 710

Management of G6PD deficiency?

Answer 710

Avoid triggers Splenectomy

Question 711

Medications that trigger G6PD deficiency?

Answer 711

Primaquine Ciprofloxacin Nitrofurantoin Trimethoprim Sulphonylurea Sulfasalazine

Question 712

What HPV strains does the HPV vaccine protect against?

Answer 712

6, 11, 16, 18

Question 713

Signs of sepsis?

Answer 713

Deranged physical observations Prolonged CRT Fever Deranged behavior Poor feeding Inconsolable cry Weak cry Reduced conciousness Reduced body tone Skin colour changes

Question 714

Investigations for paediatric sepsis?

Answer 714

Blood tests; FBC, U+E, CRP, Clotting, lactate Blood gas Blood cultures Urine dipstick, cultures and microscopy

Question 715

Management of paediatric sepsis?

Answer 715

IV access IV fluids; 20ml/kg when lactate is above 2 or signs of shock Antibiotics Supplemental oxygen; below 94% or signs of shock

Question 716

Signs of meningococcal septicaemia?

Answer 716

Non blanching rash DIC

Question 717

Most common cause of meningitis in neonate?

Answer 717

Group B streptococcus

Question 718

When is a lumbar puncture indicated in a child?

Answer 718

Under 1 month presenting with fever 1-3 months with fever and unwell Under 1 year with unexplained fever, and other features of serious illness

Question 719

Most common cause of meningitis in children?

Answer 719

Neisseria meningitidis Streptococcus pneumoniae

Question 720

Presentation of meningitis in children?

Answer 720

Fever Neck stiffness Vomiting Headache Photophobia Altered conciousness Bulging fontanelle

Question 721

Tests to assess meningeal irritation?

Answer 721

Kernig's tests Brudzinski's tests

Question 722

Management of bacterial meningitis?

Answer 722

Community; suspected meningitis, non blanching rash give IM benzylpenicillin Hospital; Under 3 months give cefotaxime and amoxicillin Over 3 months give ceftriaxone Steroids 4 times a day for 4 days in infants over 3 months Single dose of ciprofloxacin to close contacts within 24 hours

Question 723

What is given to close contacts of a confirmed meningitis case?

Answer 723

Single dose ciprofloxacin

Question 724

Causes of viral meningitis?

Answer 724

HSV VZV

Question 725

Complications of meningitis?

Answer 725

Hearing loss Seizures and epilepsy Cognitive impairment Learning disability Memory loss Cerebral palsy

Question 726

Why are steroids given to children over 3 months with meningitis?

Answer 726

Reduce neurological complications, hearing loss

Question 727

Cause of encephalitis in children?

Answer 727

HSV-1, HSV-2 VZV Chickenpox CMV EBV

Question 728

Presentation of encephalitis?

Answer 728

Altered consciousness, cognition Unusual behavior Acute onset/ focal neurological symptoms Acute onset of focal seizures Fever

Question 729

Investigations to diagnose encephalitis?

Answer 729

Lumbar puncture CT/ MRI scan EEG recording Swabs HIV testing

Question 730

Contraindications to LP in children?

Answer 730

GCS under 9 Hemodynamically unstable Active seizure Post ictal

Question 731

Management of encephalitis?

Answer 731

Aciclovir; HSV, VZV Ganciclovir; CMV

Question 732

Complications of encephalitis?

Answer 732

Lasting fatigue Prolonged recovery Changes in personality, mood, cognition, memory and behaviour Learning disability Headache Chronic pain Movement disorders Sensory disturbance Seizure Hormonal balance

Question 733

Causes of infectious mononucleosis?

Answer 733

EBV

Question 734

Where is EBV found?

Answer 734

Saliva of infected person

Question 735

Presentation of infectious mononucleosis?

Answer 735

Fever Sore throat Fatigue Lymphadenopathy Tonsillar enlargement Splenomegaly Itchy maculopapular rash upon receiving amoxicillin

Question 736

Investigations to diagnose infectious mononucleosis?

Answer 736

Heterophile antibody test; monospot test, paul-bunnell test Specific antibody test for IgM and IgG

Question 737

Management of infectious mononucleosis?

Answer 737

Self limiting acute illness that lasts 2-3 weeks

Question 738

Complications of infectious mononucleosis?

Answer 738

Splenic rupture Glomerulonephritis Haemolytic anaemia Thrombocytopenia chronic fatigue Burkitt's lymphoa

Question 739

What is mumps?

Answer 739

Viral infection spread by respiratory droplets with an incubation period of 14-25 days

Question 740

Presentation of mumps?

Answer 740

Prodrome; flu like symptoms of fever, muscle ache, lethargy, reduced appetite, headache and dry mouth Followed by parotid swelling with pain

Question 741

How is mumps diagnosed?

Answer 741

History PCR testing of saliva

Question 742

Management of mumps?

Answer 742

Supportive Notify public health

Question 743

Complications of mumps?

Answer 743

Pancreatitis Orchitis Meningitis Sensorineural hearing loss

Question 744

How is HIV transmission during birth limited?

Answer 744

Depends on maternal viral load if < 50; vaginal delivery If >50 consider C-section if >400 C-section if >10000 give IV zidovudine

Question 745

If breastfeeding recommended in HIV positive mothers?

Answer 745

No- even if viral load is undetectable

Question 746

Indication to test children for HIV?

Answer 746

Parents are HIV positive When immunodeficiency is suspected Young people who are sexually active Needle stick injury Sexual abuse IVDU

Question 747

When are babies born to HIV positive parents tested?

Answer 747

Viral load test at 3 months- if negative have not contracted HIV HIV antibody test at 24 months; checks for exposure after 3 months test, should be negative unless breastfed

Question 748

Management of paediatric HIV?

Answer 748

Antiretroviral therapy Continue with normal childhood vaccination- avoid live vaccines if severely immunocompramised Prophylactic co-trimoxazole; prevent pneumocystis jirovecii pneumonia Treat opportunistic infections

Question 749

What does paediatric HIV MDT involve?

Answer 749

Regular follow up to monitor growth and development Dietician input for nutrition Parental education Disclosing diagnosis is often delayed to the child Psychological support Specific sex education when appropriate

Question 750

Risk of developing chronic hepatitis B infection?

Answer 750

90% in neonates 30% in children under 5 Less than 10% for adolescents

Question 751

Breastfeeding advice in hepatitis B postivie mother?

Answer 751

Safe to breastfeed in appropriately vaccinated children

Question 752

When to test children for hepatitis B?

Answer 752

After 12 months of age in positive mothers After close contact

Question 753

Management of hepatitis B in children?

Answer 753

Regular monitoring of ALT, HbeAg, HBV DNA, physical Consider antiviral medication if signs of hepatitis

Question 754

When are children born to hepatitis C positive mothers tested?

Answer 754

18 months

Question 755

Medical treatment of hepatitis C in children?

Answer 755

pegylated interferon, ribavirin

Question 756

Management of depression in children?

Answer 756

First line is psychological therapy with CBT, non directive supportive therapy, interpersonal therapy, family therapy Medications; fluoxetine second line; sertraline, citalopram....continue for 6 months after remission Mood and feelings questionnaire can be used to assess progress Consider admission in severe cases

Question 757

When should children be refferred to CAMHS for depression?

Answer 757

Moderate to severe depression

Question 758

How is GAD assessed in children?

Answer 758

GAD-7 anxiety questionnaire

Question 759

Management of GAD in children?

Answer 759

Mild; watch and wait, self help strategies Moderate to severe; counselling, CBT, SSRI

Question 760

Associations of OCD?

Answer 760

Anxiety Depression Eating disorder ASD Phobia

Question 761

Management of OCD?

Answer 761

Education and self help Referral to CAMHS Patient and carer education CBT SSRI medication

Question 762

What is autism spectrum disorder?

Answer 762

Full range of people affected by a deficit in social interaction, communication and flexible behaviour

Question 763

Features of ASD?

Answer 763

Social interaction; Lack of eye contact Delay smiling Avoid physical contact Unable to read non verbal cues Difficulty establishing friendships Not displaying desire to share attention Communication; Delay, absence or regression in language development Lack of appropriate non verbal communication Difficulty with imaginative behaviour Repetitive use of words/ phrases Behaviour; Greater interest in objects, numbers or patterns than people Stereotypical repetitive movement Intensive and deep interests that are persistent and rigid Repetitive behaviour and fixed routine Anxiety and distress working outside normal routine Extremely restricted food preference

Question 764

Management of ASD?

Answer 764

MDT Child psychology and CAMHS SALT Dietician Peadiatrician Social worker Specially trained educators

Question 765

What is required for a diagnosis of ADHD?

Answer 765

Display of behavioral disturbance across all settings

Question 766

What is tourettes?

Answer 766

Development of tics which are persistent for 1 year tics are involuntary movements or sounds performed repetitively throughout the day

Question 767

Associations of tourettes?

Answer 767

OCD ADHD

Question 768

Management of tourettes?

Answer 768

Habit reversal training Exposure with response prevention Antipsychotic medications can be trialed

Question 769

What is Down's syndrome?

Answer 769

Trisomy 21

Question 770

Features of Down's syndrome?

Answer 770

Hypotonia Bradycephaly Short neck Short stature Flattened face and nose Prominent epicanthic folds Upward sloping palpable fissures Single palmar crease

Question 771

Complications of Down's syndrome?

Answer 771

Learning disability Recurrent otitis media Deafness Visual problems; myopia, strabismus, cataracts Hypothyroidism Cardiac defects; ASD, VSD, PDA and tetralogy of fallot Atlantoaxial instability Leukaemia Dementia

Question 772

Monitoring of Downs syndrome?

Answer 772

Regular thyroid check - every 2 years Echocardiogram Regular audiometry Regular eye checks

Question 772

Management of Down's syndrome?

Answer 772

MDT Occupational therapy SALT Physiotherapy Dietician Paediatrician GP Health advisor Cardiologist ENT, audiology Optician, ophthalmology Social service Educational support

Question 773

Prognosis of Down's syndrome?

Answer 773

Varies based on severity of complications Average life expectancy is 60 years

Question 774

Management of Kleinfelter syndrome?

Answer 774

Testosterone injections Advanced IVF Breast reduction surgery SALT Occupational therapy Physiotherapy Educational support

Question 774

What is Klinefelter syndrome?

Answer 774

Male has an additional X chromosome so phenotype is 47XXY

Question 775

Features of Kleinfelter syndrome?

Answer 775

Tall height Wide hips Gynaecomastia Weak muscles Small testicles Reduced libido Shyness Infertility Subtle learning difficulties

Question 776

What is turner's syndrome?

Answer 776

Single X chromosome 45XO

Question 776

Prognosis of Kleinfelter syndrome?

Answer 776

Normal life expectancy Increased risk of; Breast cancer, osteoporosis, diabetes, depression

Question 777

Features of Turner's syndrome?

Answer 777

Short stature Webbed neck High arched palate Downward sloping eyes with ptosis Broad chest with widely spaced nipples Cubitus valgus Underdeveloped ovaries and reduced function Late or incomplete puberty Infertile

Question 778

Associations of Turner syndrome?

Answer 778

Recurrent otitis media Recurrent UTI Coarctation of aorta Hypothyroidism Hypertension Obesity Diabetes Osteoporosis Various specific learning disabilities

Question 779

Management of Turners syndrome?

Answer 779

Growth hormone therapy Oestrogen and progesterone Fertility treatment

Question 780

Inheritance pattern for Noonan syndrome?

Answer 780

Autosomal dominant

Question 781

Features of Noonan syndrome?

Answer 781

Short stature Broad forehead Downward sloping eyes with ptosis Hypertelorism Prominent nasolabial folds Low set ears Webbed neck Widely spaced nipples

Question 782

Associations of noonan syndrome?

Answer 782

Congenital heart disease; pulmonary valve stenosis, HOCM, ASD Cryptorchidism Learning disability Bleeding disorder Increased risk of leukaemia or neuroblastoma

Question 783

Management of Noonan syndrome?

Answer 783

No treatment, manage symptoms, and mainly cardiac screening

Question 784

What is Marfans syndrome?

Answer 784

Autosomal dominant connective tissue disease affecting fibrillin

Question 785

Features of marfans syndrome?

Answer 785

Tall stature Long neck Long limbs Long fingers High arch palate Hypermobility Pectus carinatum, pectus excavatum Downward sloping palpable fissures

Question 786

Associations of marfans?

Answer 786

Lens dislocation in eye Joint dislocation and pain due to hypermobility Scoliosis Pneumothorax GORD Mitral valve prolapse Aortic valve prolapse Aortic aneurysm

Question 787

Management of marfans?

Answer 787

Monitor for cardiac complications Keep blood pressure and heart rate low Annual echocardiogram and ophthalmology

Question 788

What is fragile X-syndrome?

Answer 788

Mutation of FMR1 gene on X chromosome which plays role in cognitive development Fragile X Mental Retardation protein 1

Question 789

Inheritance mechanism of Fragile X?

Answer 789

X-linked Hence always affects men but not alway women

Question 790

Features of fragile X?

Answer 790

Intellectual disability Long narrow face Large ears Large testicles after puberty Hypermobile joints ADHD Autism Seizures

Question 791

What is Prada- Willi syndrome?

Answer 791

Deletion of functional gene on chromosome 15

Question 792

Features of Prada- Willi syndrome?

Answer 792

Constant insatiable hunger Hypotonia Mid- moderate learning disability Hypogonadism Fair, soft skin prone to bruising Mental health, especially anxiety Narrowed forehead Almond shaped eyes Strabismus Thin upper lip Downturned mouth

Question 793

Management of Prada- Willi syndrome?

Answer 793

Growth hormone Dieticians Educational support Social worker Psychologist Physiotherapist Occupational therapy

Question 794

Purpose of growth hormone in Prada- Willi syndrome?

Answer 794

Improve muscle development and body composition

Question 795

What is angelman syndrome?

Answer 795

Chromosome 15 deletion resulting in loss of function of UBE3A gene

Question 796

Features of angelman syndrome?

Answer 796

Delayed development and learning disability Severe delay or absence of speech development Ataxia Fascination with water Happy demeanour Inappropriate laughter Hand flapping Abnormal sleep pattern Epilepsy ADHD Dysmorphic feature Microcephaly Fair, skin, light hair, blue eyes Wide mouth, widely spaced teeth

Question 797

Management of angelman syndrome?

Answer 797

Parental education Social services/ support Educational support Physiotherapy Occupational therapy Psychology, CAMHS

Question 798

What is williams syndrome?

Answer 798

Chromosome 7 deletion

Question 799

Features of Williams syndrome?

Answer 799

Broad forehead Starburst eye Flattened nasal bridge Long philtum Wide mouth with widely spaced teeth Small chin Very sociable Mild learning disability

Question 800

Till what age is exclusive breastfeeding recommended?

Answer 800

6 months

Question 801

When is weightloss in infants normal?

Answer 801

Upto 10% by 5 day which is regained by day 10

Question 802

How are babies fed in the first week of life?

Answer 802

Gradual increase in milk volume per day until day 5 where roughly daily intake should be 150mls/ kg

Question 803

Phases of growth in children?

Answer 803

Birth- 2 years; rapid growth driven by nutrition 2 years- puberty; steady slow growth During puberty; rapid growth driven by sex hormones

Question 804

What is the definition of overweight and obesity in children?

Answer 804

BMI over 85th centile is overweight BMI over the 95th centile is obese

Question 805

Definition of faltering growth?

Answer 805

Fall in weight across one centile space if birthweight is below ninth centile Fall in weight across two centile spaces if birthweight is between 9th and 91st centile Fall in weight across three centiles if birthweight is above 91st centile

Question 806

Causes of failure to thrive?

Answer 806

Inadequate nutritional intake; maternal malabsorption, IDA, family/ parental problems, neglect, lack of availability of food Difficulty feeding; poor suck, cleft lip, genetic condition with abnormal facial structure, pyloric stenosis Causes of malabsorption; cystic fibrosis, coeliac disease, cow's milk protein intolerance, chronic diarrhoea, IBD Increased energy requirements; hyperthyroidism, chronic disease, malignancy, chronic infection Metabolic conditions

Question 807

Management of faltering growth?

Answer 807

Manage underlying cause Lactation support Dietician input Supplements

Question 808

What is defined as short stature?

Answer 808

Height more than 2 standard deviations below average for their age and sex

Question 809

Causes of short stature?

Answer 809

Familial Constitutional delay in development Malnutrition Chronic disease; coeliac, IBD, IBS, congenital heart disease Endocrine; hypothyroidism Genetic; Downs Achondroplasia

Question 810

Domains of developmental milestones?

Answer 810

Gross motor Fine motor Language Personal and social

Question 811

Gross motor development milestones?

Answer 811

Newborn Limbs flexed, symmetrical pattern Marker head lag on pulling up 6-8 weeks Raises head to 45 degrees in prone (tummy time) 6-8 months Sits without support (initially with round back then eventually with a straight back by 8 months) Limit; 9 months 8-9 months Crawling  10 months Stands independently Cruises around furniture 12 months Walks unsteadily; broad gait with hands apart Limit; 18 months 15 months Walks steadily 2.5 years Runs and jump

Question 812

Vision and fine motor developmental milestones?

Answer 812

6 weeks Follows moving object or face by turning the head (fixing and following) Limit; 3 months  4 months Reaches out for toys  Limit; 6 months 4-6 months Palmar grasp 7 months  Transfers toys from one hand to another Limit; 9 months 10 months Mature pincer grip Limit; 12 months 16-18 months Makes marks with crayons Brick building Tower of 3; 18 months Tower of 6; 2 years Tower of 8 or a train with 4 bricks; 2.5 years Bridge; 3 years Steps; 4 years Pencil skills  Line; 2 years Circle; 3 years Cross; 3.5 years Square; 4 years Triangle; 5 years

Question 813

Hearing, speech and language developmental milestones?

Answer 813

Newborn  Startles to loud noises 3-4 months Vocalises alone or when spoken to, coos and laughs  7 months Turns to soft sounds out of sight Polysylabic babble 7-10 months Sounds used indiscriminately Sounds used discrimately to parents at 10 months  12 months Two/ three words together other than Mama, Dada 18 months 6-10 words  Able to show two parts of the body 20-24 months Joins words to make simple phrases 2.5-3 years Talks constantly in 3/4 word sentences

Question 814

Social and personal developmental milestones?

Answer 814

6 weeks Smiles responsively Limit; 8 weeks 6-8 months Puts food in their mouth 10-12 months Waves bye-bye Plays peek-a-boo 12 months Drinks from a cup with two hands  18 months Holds spoon and gets food safely to mouth 18-24 months Symbolic play Limit; 2-2.5 years 2 years Toilet training; dry by day Pulls off some clothing 2.5- 3 years Parallel play Interactive play evolving Takes turns

Question 815

Red flags in development?

Answer 815

Loss of developmental milestones Not able to hold object at 5 months Not sitting unsupported at 12 months Not standing independently at 18 months Not walking independently at 2 years Not running at 2.5 years No words at 18 months No interest in others by 18 months

Question 816

Causes for global developmental delay?

Answer 816

Downs syndrome Fragile X Foetal alcohol syndrome Rett syndrome Metabolic disorders

Question 817

Causes of gross motor developmental delay?

Answer 817

Cerebral palsy Ataxia Myopathy Spina bifida Visual problems

Question 818

Causes of fine motor developmental delay?

Answer 818

Dyspdaxia Cerebral palsy Muscular dystrophy Visual impairement Congenital ataxia

Question 819

Causes of language developmental delay?

Answer 819

Specific social circumstances Hearing impairement Learning disability Neglect Autism Cerebral palsy

Question 820

Causes of personal and social developmental delay?

Answer 820

Emotional and social neglect Parenting issues Autism

Question 821

Typesnof learning difficulties?

Answer 821

Dyslexia; specific difficulty in reading, writing and spelling Dysgraphia; specific difficulty in writing Dyspraxia; difficulty in physical coordination Non verbal learning difficulty; difficulty in processing non verbal information

Question 822

Classification of learning difficulty?

Answer 822

Based on IQ; 55-70; mild 40-55; moderate 25-40; Severe Under 25; Profound

Question 823

Causes of learning difficulties?

Answer 823

Family history Genetic disorders such as Downs Antenatal problems; FAS, Maternal chickenpox Birth problems; premature, hypoxic ischaemic encephalopathy Meningitis in early childhood Autism Epilepsy

Question 824

Management of learning disability?

Answer 824

MDT approach Health visitors Social worker School Educational psychologist Paediatrician , GP, nurses Occupational therapy Speech and language therapy

Question 825

What is tanner staging?

Answer 825

Scale used to determine pubertal stage based on examination findings of sex characteristics

Question 826

Investigations to diagnose delayed puberty?

Answer 826

FBC, U&E, coeliac screen FSH, LH thyroid function test Growth hormone Serum prolactin Genetic microarray for kleinfelter or turner's syndrome X-ray of wrist, pelvic ultrasound, MRI of brain

Question 827

What are frazer guidelines?

Answer 827

Guidelines for providing contraception to patients under 16 years without parental input or consent established by House of Lords in 1985 and the child must meet following criteria; Mature and intelligent enough to understand treatment Can't be persuaded to discuss with parent or let health card professional discuss with them Likely to have inet ourselves regardless of treatment Physical or mental health is likely to suffer without treatment Treatment is in their best interest

Question 828

Examples of hypersensitivity reactions in children?

Answer 828

Asthma Atopic eczema Allergic rhinitis Hay-fever Food allergies Animal allergies

Question 829

Coombes ane Gell Classification of hypersensitivity reactions?

Answer 829

Type 1; IgE antibodies trigger mast cell and basophils to release histamines and other cytokines causing an immediate reaction Type 2; IgG and IgM antibodies react to allergen and activate complement system leading to direct damage to local cells Type 3; immune complexes accumulate and damage local tissues resulting in autoimmune disease Type 4; cell mediated hypersensitivity reactions caused by T-cells such as organ rejection

Question 830

Investigations for diagnosing allergy?

Answer 830

Skin prick test, patch test RAST test for specific IgE Food challenge testing- Gold standard

Question 831

Management of allergy?

Answer 831

Establish correct allergen Avoid allergen Avoid foods that trigger reactions Regular hoovering and changing bedsheets for those allergic to house dust mites Prophylactic Antihistamines Patients at risk of anaphylaxis should have epipen

Question 832

Presentation of anaphylaxis?

Answer 832

Rapid onset of allergic symptoms; urticaria, itching, angio-oedema with swelling around the eyes and lips and abdominal pain Shortness of breath Wheeze Swelling of larynx, causing stridor, tachycardia, lightheadedness, collapse

Question 833

Management of anaphylaxis in paediatrics?

Answer 833

ABCDE IM adrenaline Antihistamine; chlorphenamine and cetrizine Steroids; Hydrocortisone Monitor for biphasic reaction

Question 834

How can anaphylaxis be confirmed?

Answer 834

Mast cell tryptase within 6 hours of event

Question 835

What is a biphasic reaction?

Answer 835

Second anaphylaxis reaction after successful treatment of the initial reaction

Question 836

Risk factors for anaphylaxis?

Answer 836

Asthma requiring inhalers Poor access to medical treatment Adolescents Nut/ insect sting allergies Significant comorbidities

Question 837

What is allergic rhinitis?

Answer 837

IgE mediated type 1 hypersensitivity reaction to environmental allergens in the nasal mucosa

Question 838

Presentation of allergic rhinitis?

Answer 838

Runny, itchy, blocked nose Sneezing Itchy, red, swollen eyes

Question 839

Triggers for allergic rhinitis?

Answer 839

Tree pollen/ grass House dust mites Pets Other allergens

Question 840

Management of allergic rhinitis?

Answer 840

Avoid triggers Antihistamines Nasal corticosteroids, Antihistamines

Question 841

What is cows milk protein allergy?

Answer 841

IgE mediated hypersensitivity to Cow's milk protein within 2 hours of ingestion

Question 842

Epidemiology of cows milk protein allergy?

Answer 842

Children under 3 years

Question 843

Presentation of cows milk protein allergy?

Answer 843

Bloating and wind Abdominal pain Diarrhoea Vomiting Urticarial rash Angio-oedema Cough/ wheeze Sneezing Watery eyes Eczema

Question 844

Management of cows milk protein allergy?

Answer 844

Breast feeding mothers should avoid dairy Replace formulas with hydrolysed formulas

Question 845

What is SCID?

Answer 845

Severe combined immunodeficiency where infants are born with absent or dysfunctioning B and T cells Most severe condition to cause immunodeficiency

Question 846

Presentation of SCID?

Answer 846

Persistent severe diarrhoea Failure to thrive Opportunistic infections Unwell after live vaccination Omenn syndrome

Question 847

Causes of SCID?

Answer 847

Mutation in common gamma chain on X chromosome coding for IL receptors on T and B cells JAC3 mutation Adenosine deaminase deficiency Omenn syndrome

Question 848

What is omenn syndrome?

Answer 848

Mutation in RAG-1 or RAG-2 that codes for proteins on T and B cells resulting in abnormally functioning T cells

Question 849

Inheritance of Omenn syndrome?

Answer 849

Autosomal recessive

Question 850

Inheritance of SCID?

Answer 850

X-linked recessive

Question 851

Presentation of omenn syndrome?

Answer 851

Red, scaly, dry rash Alopecia Diarrhoea Failure to thrive Lymphadenopathy Hepatosplenomegaly

Question 852

Management of SCID?

Answer 852

Immunoglobulin therapy Minimise risk of infection Avoid live vaccines Haematopoetic stem cell transplant

Question 853

What is burton's agammaglobulinaemia?

Answer 853

X-linked agammaglobulinaemia resulting in deficiency in all classes of immunoglobulins

Question 854

What is DiGeorge syndrome?

Answer 854

22q 11.2 microdeletion of chromosome 22 leading to defect in development of third pharyngeal pouch and third brachial cleft affecting thymus gland development and creates malfunctioning T cells

Question 855

Features of DiGeorge syndrome?

Answer 855

Congenital heart disease Abnormal facies Thymus gland incompletely developed Cleft palate Hypothyroidism and hypocalcaemia

Question 856

Causes of tonsilitis?

Answer 856

Group A strep; strep pyogenes Haemophillus influenzea Moraxella catarrhalis Staph aureus VIRAL

Question 857

Structure affected in tonsillitis?

Answer 857

Waldeyer's tonsillar ring contains 6 areas of lymphoid tissue containing the adenoid, tubal tonsils, palatine tonsils, lingual tonisil Palatine tonsils typically become inflammed in tonsilitis

Question 858

Presentation of tonsillitis?

Answer 858

Fever Sore throat Painful swallowing Tympanic swelling Cervical lymphadenopathy

Question 859

What is the centor criteria?

Answer 859

estimates probability of tonsilitis being caused by bacterial infection A score of 3 (40-60% probability) more indicates treatment with antibiotics, a point is given for each of the following; Fever over 38 Tonsillar exudates Absence of cough Tender anterior cervical lymph nodes

Question 860

What is the FeverPAIN score?

Answer 860

Alternative to centor criteria, score of 2-3 (34-40%) and 4-5 (62-65%) Fever for 24 hours Purulence Attended within 3 days of onset of symptoms Inflammed tonsils No cough or coryza

Question 861

Management of tonsillitis?

Answer 861

phenoxymethylpenicillin for 10 days if centor score over 3 or FeverPAIN over 4 Clarythromycin if allergic

Question 862

Complications of tonsillitis?

Answer 862

Chronic tonsillitis Quinsy Otitis media Scarlet fever Rheumatic fever Post streptococcal glomerulonephritis Post streptococcal reactive arthritis

Question 863

What is quinsy?

Answer 863

Peritonsillar abscess in region of tonsils as a result of bacterial infection

Question 864

Presentation of quinsy?

Answer 864

Sore throat Painful swallowing Fever Neck pain Reffered ear pain Swollen tender lymph nodes Trismus Change in voice Swelling and erythema

Question 865

Causative organism of quinsy?

Answer 865

Group A strep Staphylococcus aureus Haemophillus influenzae

Question 866

Management of quinsy?

Answer 866

ENT referal for incision and drainage Co-amoxiclav Dexamethasone

Question 867

Indications for tonsillectomy?

Answer 867

Recurrent tonsilitis; 7 or more in a year 5 per year for 2 years 3 per year for 3 years recurrent tonsillar abscess Enlarged tonsils causing difficulty breathing, swallowing, snoring

Question 868

Causes of hearing loss in children?

Answer 868

Congenital; Maternal rubella, CMV Genetic deafness Down's syndrome Perinatal; Prematurity, hypoxia during birth After birth; Jaundice Meningitis, encephalitis Otitis media with glue ear Chemotherapy

Question 869

Management of hearing loss?

Answer 869

SALT Educational psychology ENT specialist Hearing aids Sign language

Question 870

Where do nosebleeds originate from?

Answer 870

Kiesselbach's plexus

Question 871

Management of recurrent nosebleeds?

Answer 871

Nasal packing Nasal cautery Consider naseptin (chlorhexidine, neomycin)

Question 872

What is cleft lip and palate?

Answer 872

Cleft lip is congenital condition with a split in the upper section of the lip

Question 873

Management of cleft palate??

Answer 873

Ensure child can eat and drink Plastic, maxillofacial ENT surgeons Dentist SALT Psychologist GP Cleft lip surgery is usually performed at 3 months, whereas cleft palate is performed at 6-12 months

Question 874

Complications of cleft lip?

Answer 874

Feeding, swallowing difficulties Speech problems Psycho-social implications

Question 875

What is tongue tie?

Answer 875

Poor extension of tongue due to short and tight lingual frenulum

Question 876

Management of tongue tie?

Answer 876

Frenotomy

Question 877

What is cystic hygroma?

Answer 877

malformation of the lymphatic system resulting in a cyst filed with lymphatic fluid located in the posterior triangle on the left side

Question 878

Features of cystic hygroma?

Answer 878

Can be large Soft Non tender Transilluminate

Question 879

Management of cystic hygroma?

Answer 879

Surgical removal

Question 880

Complications of cystic hygroma?

Answer 880

Interfere with feeding, breathing, swallowing Infection Haemorrhage

Question 881

What is a thyroglossal cyst?

Answer 881

persistence of thyroglossal duct which accumulates fluid

Question 882

What is a branchial cyst?

Answer 882

failure of second brachial cleft to close properly leaving space in lateral aspect of neck

Question 883

Symptoms of syncope?

Answer 883

Hot/ clammy Sweaty Heavy Dizzy/ lightheaded Vision going blurry/ dark Headache

Question 884

Causes of syncope?

Answer 884

Dehydration Missed meals Extended standing in warm environment Vasovagal response Hypoglycaemia Anaemia Infection Anaphylaxis Arrhythmia Valvular heart disease HOCM

Question 885

Management of syncope?

Answer 885

Treat causes Avoid triggers

Question 886

Treatment of tonic-clonic seizures?

Answer 886

First line; sodium valproate Second line; lamotrigine, carbamazepine

Question 887

Features of focal seizures?

Answer 887

Starts in temporal lobes affecting hearing, speech, memory, emotions presents as hallucinations, memory flashback, dejavu, doing strange things on autopilot

Question 888

Management of focal seizures?

Answer 888

First line; carbamazepine, lamotrigine Second line; sodium valproate, levetiracetam

Question 889

Management of absence seizures?

Answer 889

Sodium valproate/ ethosuximide

Question 890

Management of atonic seizures?

Answer 890

First line; sodium valproate Second line; lamotrigine

Question 891

Management of myoclonic seizures?

Answer 891

Sudden breif muscle contractions

Question 892

Management of myoclonic seizures??

Answer 892

First line; sodium valproate Options; lamotrigine, levetiracetam, topiramate

Question 893

What are infantile spasms?

Answer 893

West syndrome staring around 6 months of age characterised by clusters of full body spasms Poor prognosis, 33% die by 25 years

Question 894

Management of west syndrome?

Answer 894

Prednisolone Vigabatrin

Question 895

General advice for those with epilepsy?

Answer 895

Take showers Cautious with swimming Cautious with heights, traffic and near heavy, hot or electrical equipment

Question 896

Side effects of carbamazepine?

Answer 896

Agranulocytosis Aplastic anaemia Induces P450 enzymes

Question 897

Side effects of sodium valproate?

Answer 897

Teratogenic Liver damage, hepatitis Hair loss Tremor

Question 898

Side effects of phenytoin?

Answer 898

Folate and Vit D deficiency Megaloblastic anaemia Osteomalacia

Question 899

Side effects of ethosuximide?

Answer 899

Night terrors Rashes

Question 900

Side effects of lamotrigine?

Answer 900

Stevens johnson syndrome Leukopenia

Question 901

Management of acute seizure?

Answer 901

Put patient in safe position Recovery position if possible Put something under head Remove obstacles that could lead to injury Note starting and call ambulance if more than 5 minutes

Question 902

Management of status epilepticus?

Answer 902

ABCDE Gain IV access If no IV then rectal lorazepam If seizures persist then phenobarbital or phenytoin Midazolam or diazepam in community

Question 903

What is status epilepticus?

Answer 903

Seizures lasting more than 5 mins or more than 3 seizures in 1 hour

Question 904

What is a febrile convulsion?

Answer 904

Seizures occuring in children aged 6 months to 5 years caused by high fever?

Question 905

Types of febrile convulsion

Answer 905

Simple febrile; generalised tonic clonic lasting less than 15 minutes and occur only once during febrile illness Complex; partial/ focal lasting more than 15 minutes occurring multiple times in an illness

Question 906

Differentials for febrile convulsion?

Answer 906

Epilepsy Meningitis, encephalitis Space occupying lesion Syncope Electrolyte abnormality Trauma

Question 907

Risk of developing epilepsy?

Answer 907

1.8% in general population 2-7.5% after simple febrile convulsion 10-20% after complex febrile convulsion

Question 908

What is a breath holding spells?

Answer 908

Involuntary episodes during which a child holds their breath triggered by an upsetting stimulus or scary event between 6 and 18 months of age and usually outgrow by 4-5 years of age

Question 909

Classification of breath holding spells?

Answer 909

Cyanotic breath holding spells Pallid breath holding spells

Question 910

Management of breath holding spells?

Answer 910

Reassurance and likely to grow out of it

Question 911

What is a reflex anoxic seizure?

Answer 911

Child startled leads to vagus nerve sending signals to heart leading to reduced contractions lasting 30 seconds

Question 912

Causes of headaches in children?

Answer 912

Tension headache Migraine Ear, nose, throat infection Analgesic headache Problems with vision Raised ICP Brain tumour Meningitis Encephalitis CO poisoning

Question 913

Management of migraine in children?

Answer 913

Rest, fluids, low stimulus environment Paracetamol Ibuprofen Sumatriptan Anti-emetics Prophylactic treatment; Propanolol, pizotifen, topiramate

Question 914

What is abdominal migraine?

Answer 914

Central abdominal pain for more than 1 hour Associated with nausea, vomiting, headache, pallor

Question 915

What is cerebral palsy?

Answer 915

Permanent neurological problems as a result of damage to the brain around the time of birth Non progressive

Question 916

Causes of cerebral palsy?

Answer 916

Antenatal; maternal infection, trauma during pregnancy Perinatal; birth asphyxia, pre-term birth Post natal; meningitis, severe neonatal jaundice, head injury

Question 917

Types of cerebral palsy?

Answer 917

Spastic; hypertonia affecting UMN Dyskinetic; problems controlling muscle tone with hypertonia and hypotonia causing athetoid movement Ataxic; problems with coordinated movement resulting from damage to cerebellum Mixed

Question 918

Patterns of spastic cerebral palsy?

Answer 918

Monoplegia Hemiplegia Diplegia Quadriplegia

Question 919

Presentation of cerebral palsy?

Answer 919

Obstetric trauma Failure to meet milestone Hand preference below 18 months Problems with coordination, speech and walking High stepping gait Feeding and swallowing problems Learning difficulty

Question 920

Gait examinations in cerebral palsy?

Answer 920

Hemiplegic/ diplegic; UMN lesion Broad based gait, ataxic gait; cerebellar lesion High stepping gait; foot drop/ LMN lesion Waddling gait; pelvic muscle weakness due to myopathy

Question 921

Complications of cerebral palsy?

Answer 921

Learning disability Epilepsy Kyphoscoliosis Muscle contractures Hearing and visual impairment GORD

Question 922

Management of cerebral palsy?

Answer 922

Physiotherapy Occupational therapy Speech and language therapy Dietician Orthopaedic surgeons Muscle relaxant; baclofen Anti-epileptic Glycopyrronium bromide; drooling Social worker

Question 923

Causes of squint?

Answer 923

Idiopathic Hydrocephalus Cerebral palsy Space occupying lesion Trauma

Question 924

What is hydrocephalus?

Answer 924

Build up of CSF either due to overproduction or reduced drainage

Question 925

Causes of hydrocephalus?

Answer 925

Aqueduct stenosis Arachnoid cysts Arnold chiari malformation Chromosomal abnormalities

Question 926

Presentation of hydrocephalus?

Answer 926

Bulging anterior fontanelle Poor feeding and vomiting Poor tone Sleepiness

Question 927

Management of hydrocephalus?

Answer 927

Ventriculoperitoneal shunt

Question 928

Complications of ventriculoperitoneal shunt?

Answer 928

Infection Blockage Excessive draining Interventricular haemorrhage Outgrowing them- typically needs changing every 2 years

Question 929

What is craniosynostosis?

Answer 929

Skull sutures close prematurely resulting in abnormal head shapes

Question 930

Presentation of craniosynostosis?

Answer 930

saggistal synostosis; long and narrow from front to back coronal synostosis; bulging on one side of the forehead metopic synostosis; pointy triangular forehead lambdoid synostosis; flattening on side of occiput Anterior fontanelle closure before 1 year of age Small head in proportion to body

Question 931

Investigations to diagnose craniosynostosis?

Answer 931

Skull X-ray CT head

Question 932

Management of craniosynostosis?

Answer 932

Surgical reconstruction

Question 933

Most common types of abnormal head shapes?

Answer 933

Plagiocephaly Brachycephaly

Question 934

Presentation of abnormal head shapes?

Answer 934

3-6 months aged baby Preference to sleep on one side of head

Question 935

Management of abnormal head shape?

Answer 935

Reassurance that it should normalise Congenital muscular torticollis, physiotherapy Plagiocephaly helmet

Question 936

What is muscular dystrophy?

Answer 936

Group of genetic conditions that lead to gradual muscle weakening and muscle wasting

Question 937

What is Gower's sign?

Answer 937

Use of specific technique to rise from lying position due to proximal muscle weakness

Question 938

What is duchennes muscular dystrophy?

Answer 938

X-linked recessive condition providing defective gene for dystrophin, a protein that holds muscles together

Question 939

Presentation of duchennes muscular dystrophy??

Answer 939

Present around 3-5 years Weakness in muscles around pelvis Life expectancy of 25-35 years

Question 940

What medication can improve prognosis of duchennes muscular dystrophy by 2 years?

Answer 940

Oral steroids Creatinine supplementation can also help with muscle strength

Question 941

What is Beckers muscular dystrophy?

Answer 941

Similar to duchenne muscular dystrophy

Question 942

What is myotonic dystrophy?

Answer 942

Presents in adulthood with; Progressive muscle weakness Prolonged muscle contraction Cataract Cardiac arrhythmia

Question 943

Presentation of facioscapulohumeral dystrophy?

Answer 943

Presents in childhood with weakness around face progressing to shoulders and arms Initial sign is eyes slightly open and weakness in pursing lips and unable to blow out cheeks

Question 944

What is Oculopharyngeal muscular dystrophy?

Answer 944

Presents in late adulthood affecting ocular muscles and pharynx

Question 945

What is limb gridle muscular dystrophy?

Answer 945

Teenage years with progressive weakness around limb girdles

Question 946

What is emery- dreifuss muscular dystrophy?

Answer 946

Childhood contractures commonly in elbows and ankles

Question 947

What is spinal muscular atrophy?

Answer 947

Autosomal recessive progressive loss of motor neurones and progressive weakness which has no cure

Question 948

Types of fractures?

Answer 948

Buckle Transverse Oblique Spiral Segmental Salter- Harris Comminuted Greenstick

Question 949

When is Salter- Harris classification of fractures used?

Answer 949

When fracture affects growth plate

Question 950

Types of salter-harris fractures?

Answer 950

Type 1; Straight across Type 2; Above Type 3; Below Type 4; Through Type 5; Crush

Question 951

Principles of fracture management in children?

Answer 951

Consider safeguarding Mechanical alignment; Closed reduction; manipulation of fracture Open reduction; surgery Provide stability to fix bone in position while healing; External cast K- wire Intramedullary wires Intramedullary nails Screws Plates Pain management ladder; Step 1; Paracetamol/ ibuprofen Step 2; Morphine

Question 952

Presentation of hip pain in children?

Answer 952

Limp Refusal to use affected leg Refusal to bear weight Inability to walk Pain Swollen and tender joint

Question 953

Causes of joint pain in children?

Answer 953

0-4 Yrs; Septic arthritis Developmental dysplasia of the hip Transient sinovitis 5-10 Yrs; Septic arthritis Perthes disease Transient sinovitis 10-16 Yrs; Septic arthritis Slipped upper femoral epiphysis Juvenile idiopathic arthritis

Question 954

Red flags for hip pain ?

Answer 954

Under 3 years Fever Waking at night Weight loss Anorexia Night sweats Fatigue Persistent pain Stiffness in morning Swollen/ red joint

Question 955

Criteria for urgent referral for investigation of hip pain?

Answer 955

Child under 3 Child older than 9 with restricted/ painful hip Not able to bear weight Evidence of neurovascular compramise Severe pain/ agitation Red flags for serious pathology Suspicion of abuse

Question 956

Investigations to assess painful hip?

Answer 956

Bloods; CRP, ESR, X ray Ultrasound; effusion Joint aspiration MRI

Question 957

Mortality of septic arthritis?

Answer 957

10%

Question 958

Causative agents of septic arthritis?

Answer 958

Staph aureus; most common Group A streptococcus Haemophilus influenza E.coli

Question 959

Differentials of septic arthritis?

Answer 959

Transient synovitis Perthes disease Slipped upper femoral epiphysis Juvenile idiopathic arthritis

Question 960

Management of septic arthritis?

Answer 960

Antibiotics for 3-6 weeks Surgical drainage and washout

Question 961

What is transient synovitis?

Answer 961

Transient inflammation and irritation of the synovial membrane

Question 962

Presentation of transient synovitis?

Answer 962

Limp Refusal to weight bear Groin/ hip pain Mild low grade fever

Question 963

Management of transient synovitis?

Answer 963

Safety net advise, attend A+E if persistent fever

Question 964

Prognosis of transient synovitis?

Answer 964

Resolves within 2 weeks 20% may have recurrence

Question 965

What is Perthes disease?

Answer 965

Disruption to blood flow in femoral head leaving to avascular necrosis affecting the growth plate

Question 966

Epidemiology of perthes disease?

Answer 966

Occurs in children 4-12, more common in 5-8 years More common in boys

Question 967

Presentation of perthes disease?

Answer 967

Pain in hip/ groin Limp Restricted hip movement Referred knee pain No history of trauma

Question 968

Investigations to diagnose perthes disease?

Answer 968

Blood Technetium bone scan MRI scan

Question 969

Management of Perthes disease?

Answer 969

Bed rest, traction, crutches, analgesia Physiotherapy Regular xrays to assess healing Surgery

Question 970

What is slipped upper femoral epiphysis?

Answer 970

Head of femur is displaced along growth plate

Question 971

Epidemiology of slipped upper femoral epiphysis?

Answer 971

More common in boys Presents 8-15 years More common in obese children Average age is 12 in boys and 11 in girls

Question 972

Presentation of SUFE?

Answer 972

History of minor trauma Hip, groin, knee or thigh pain Restricted range of hip movement Painful limp May be undergoing growth stunt Prefer external rotation and limited internal rotation of hip joint

Question 973

Investigations to diagnose SUFE?

Answer 973

Blood tests; inflammatory markers Technetium bone scan CT/ MRI scan

Question 974

Management of SUFE?

Answer 974

Surgery

Question 975

What is osteomyelitis?

Answer 975

Infection of bone and bone marrow typically along metaphysis of long bones

Question 976

Risk factors for osteomyelitis?

Answer 976

Open bone fracture Orthopaedic surgery Immunocompromised Sickle cell anaemia HIV

Question 977

Presentation of osteomyelitis?

Answer 977

Refusing to use limb or weight bear Pain Swelling Tenderness

Question 978

Management of osteomyelitis?

Answer 978

Antibiotic therapy Drainage and debridement

Question 978

Investigations to diagnose osteomyelitis?

Answer 978

Xray Bone scan MRI scan is gold standard Blood test and cultures

Question 979

What is osteosarcoma?

Answer 979

Type of bone cancer

Question 980

Epidemiology of osteosarcoma?

Answer 980

Typically presents in 10-20 year olds Most common femur, other sites include tibia and humerus

Question 981

Presentation of osteosarcoma?

Answer 981

Persistent bone pain, typically worse at night Bone swelling Palpable mass Restricted joint movements

Question 982

Diagnosis of osteosarcoma?

Answer 982

Urgent Xray within 48 hours- poorly defined lesion Bloods- raised ALP CT, MRI, Bone scan, PET Bone biopsy

Question 983

Management of osteosarcoma?

Answer 983

Surgical resection, limb amputation Adjuvant chemotherapy MDT; oncology, nurse, physio, OT, psychology, dietician, prosthesis, social services

Question 984

Complications of osteosarcoma?

Answer 984

Pathological bone fracture Metastasis

Question 985

What is talipes and its types?

Answer 985

Fixed abnormal ankle position Talipes equinovarus; ankle in plantar flexion and supination Talipes calcaneovalgus; ankle in dorsiflexion and pronation

Question 986

Management of talipes?

Answer 986

Ponseti method Physiotherapy Surgery

Question 987

What is developmental dysplasia of the hip?

Answer 987

Structural abnormalities in hip due to abnormal development of foetal bones during pregnancy leading to instability of hips and potential for subluxation/ dislocation

Question 988

Risk factors for DDH?

Answer 988

First degree family history Breech presentation from 36 weeks Breech presentation at birth Multiple pregnancy

Question 989

Findings suggesting DDH?

Answer 989

Different leg length Restricted hip abduction on one side Significant bilateral restriction in abduction Difference in knee level when hips are flexed

Question 990

Tests to assess DDH?

Answer 990

Ortolani Barlow

Question 991

Diagnosis of DDH?

Answer 991

USS of the hips

Question 992

Management of DDH?

Answer 992

Pavlik harness; adjusted every 6-8 weeks Surgical correction

Question 993

What is rickets?

Answer 993

Vitamin D deficiency resulting in poor bone mineralisation producing soft bones

Question 994

Presentation of rickets?

Answer 994

Lethargy Bone pain Swollen wrist Bone deformity Poor growth Dental problem Muscle weakness Pathological/ abnormal fracture Bone deformities; bowing of knee, knock knee, rachitic rosary, craniotabes Delayed dentition

Question 995

Investigations to diagnose rickets?

Answer 995

Serum 25-hydroxyvitaminD X-ray Low calcium Low phosphate High PTH and ALP

Question 996

Management of rickets?

Answer 996

Prevention Vit D and calcium supplementation

Question 997

What is achondroplasia?

Answer 997

Disproportionate short stature due to skeletal dysplasia

Question 998

Cause of achondroplasia?

Answer 998

Mutation in fibroblast growth factor receptor gene 3 (FGFR3) on chromosome 4 causing dysfunction in epiphyseal plates restricting bone length growth

Question 999

Mechanism of inheritance in achondroplasia?

Answer 999

Autosomal dominant

Question 1000

Features of achondroplasia?

Answer 1000

Disproportionate short stature with unaffected spine length Short digits Bow legs Disproportionate skull; flattened mid face and nasal bridge Foramen magnum stenosis

Question 1001

Associations of achondroplasia?

Answer 1001

Recurrent otitis media Kyphoscoliosis Spinal stenosis OSA Obesity Cervical cord compression Hydrocephalus

Question 1002

Management of anchondroplasia?

Answer 1002

MDT; paeds, nurses, physio, OT, dietician, ortho, ENT, genetics

Question 1003

What is osgood schlatter disease?

Answer 1003

Inflammation at tibial tuberosity where patella ligament inserts

Question 1004

Presentation of osgood schlatter disease?

Answer 1004

Visible/ palpable hard and tender lump at tibial tuberosity Pain in anterior aspect of the knee Pain exacerbated by physical activity

Question 1005

Management of osgood schlatter disease?

Answer 1005

Reduction in physical activity, Ice, NSAIDs Stretching and physiotherapy

Question 1006

Prognosis of osgood schlatter disease?

Answer 1006

Usually left with hard bony lump on knee Anvulsion fracture

Question 1007

What id osteogenesis imperfecta?

Answer 1007

Genetic condition that makes bones brittle as a result of a collagen synthesis defect

Question 1008

Features of osteogenesis imperfecta?

Answer 1008

Recurrent and inappropriate fractures Hypermobility Blue/ grey sclera Triangular face Short stature Deafness from early adulthood Dental problems Bone deformities Joint and bone pain

Question 1009

Management of osteogenesis imperfecta?

Answer 1009

Bisphosphonates and vitamin D supplementation Physiotherapy, occupational therapy Orthopaedic surgeon Specialist nurse, social worker

Question 1010

Triad of ASD?

Answer 1010

Impaired communication Impaired social interaction Ritualistic behaviour

Question 1011

Risk factors for DDH?

Answer 1011

female sex: 6 times greater risk breech presentation positive family history firstborn children oligohydramnios birth weight > 5 kg congenital calcaneovalgus foot deformity