Paediatrics

Question 1

Give 7 signs of respiratory distress

Answer 1

Increased work of breathing
Tachypnea (>60/min)
Nasal flaring
Intercostal recessions
Tracheal tug
Cyanosis (Central>peripheral)
Grunting

Question 2

Name 4 causative pathogens for viral induced wheeze

Answer 2

Respiratory syncytial virus (RSV)
Rhinovirus (HRV)
Human metapneumovirus (hMPV)
Influenza

Question 3

What virus is responsible for the common cold?

Answer 3

Rhinovirus (HRV)
(Small-sized positive strand RNA virus)

Question 4

Atopy is related to a deficiency in what cytokine?

Answer 4

Interferon B

Question 5

What is the treatment for an acute episode of viral induced wheeze (mild-moderate)

Answer 5

Oxygen
Bronchodilator; Salbutamol +/- Spacer

Question 6

What treatment should be considered in an asthmatic experiencing an episode of viral induced wheeze

Answer 6

Oral Prednisolone (inhaled steroid)

Question 7

What treatment is considered for patients experiencing severe, recurrent attacks of wheezing (viral induced wheeze) (2 options)

Answer 7

Low dose inhaled corticosteroid
or
Leukotriene receptor antagonist-montelukast

Question 8

What is the 1st and 2nd line treatment for bacterial infection causing wheeze?

Answer 8

1st line - Amoxicillin

2nd Line - Doxycycline (if amoxicillin isn’t tolerated/is contraindicated)

Question 9

List indications for seeking medical attention in an episode of viral induced wheeze (8 options)

Answer 9

If child requires salbutamol inhaler >2 times/week

Night time coughing

Increased breathing rate

Episodes of apnea

Signs of increased breathing effort

Reduced feeding (<50% normal feeds) /features of dehydration (dry mouth/infrequent passage of urine)

Becomes less responsive

Persistent/worsening fever

Question 10

Give 3 pieces of self care advice for patients with viral induced wheeze

Answer 10

Paracetamol +/- Ibuprofen for fever

Regular fluids

If wheeze returns, start regular salbutamol via spacer and refer to viral wheeze action plan

Question 11

List 5 DDx for viral induced wheeze

Answer 11

Asthma
Bronchiolitis
Inhaled foreign body
Cystic fibrosis
Gastro-oesophageal reflux

Question 12

What treatment should be given for the acute episode of viral induced wheeze? (severe-life threatening)

Answer 12

Oxygen
Bronchodilator; Salbutamol +/- spacer
Ipratropium bromide
Oral Steroids; Prednisolone (1mg/kg)

Question 13

Define episodic viral wheeze

Answer 13

Wheezing that occurs during discrete time periods, often in association with clinical evidence of a viral upper respiratory infection, with the absence of wheeze between episodes.

Question 14

Define multi-trigger wheeze

Answer 14

Describes discrete exacerbations of wheezing but there are also symptoms between episodes, e.g in response to allergens, emotions or activity.

Question 15

List 5 risk factors for viral induced wheeze

Answer 15

Prematurity
Exposure to smoking
Bronchiolitis
Increased exposure to viruses (when younger)
Smaller airway anatomy (Poiseuille’s law)

Question 16

What tool is used to predict asthma in children? (List the major and minor predictive factors)

Answer 16

Asthma Predictive Index (API)

One major decisive factors;
- Parents with asthma
- Physician diagnosis of eczema
- Sensitivities to air allergens (positive RASTS or Skin-prick tests)

Or

Two minor decisive factors;
- Food allergies
- More than 4% blood eosinophils
- Wheezing apart from colds

Question 17

What pattern of inheritance is seen in Cystic Fibrosis?

Answer 17

Autosomal Recessive Disorder

Question 18

Cystic fibrosis occurs secondary to a defect in which gene and protein channel?

Answer 18

CFTR gene and cAMP regulated chloride channel

Question 19

Describe the pathophysiology of cystic fibrosis

Answer 19

CFTR Gene Defect > Defective ion transport > Airway surface liquid depletion > Defective mucocilary clearance > mucus obstruction.

Mucus obstruction increases propensity to infection and inflammation in positive feedback loop.

Question 20

Where is the genetic defect in cystic fibrosis?

Answer 20

Delta F508 on the long arm of chromosome 7

Question 21

Name 3 organisms that commonly colonise cystic fibrosis patients

Answer 21

Staphylococcus aureus
Pseudomonas aeruginosa
Aspergillus

Question 22

What is a feature is suggestive of cystic fibrosis in neonates (1)

Answer 22

Failure to pass meconium (meconium ileus)

Occurs due to thickening of meconium.

Question 23

List features of cystic fibrosis in children (6)

Answer 23

Failure to thrive, short stature, delayed puberty
Big appetite
Malabrospiton +/- steatorrhoea
Chronic Wet/Productive Cough
Recurrent lower airway infections
Chronic sinusitis +/- nasal polyps

Question 24

List some complications of cystic fibrosis (5)

Answer 24

Nasal polyps
Male infertility
Diabetes mellitus
Rectal prolapse (in children)
Pseudo Bartter syndrome

Question 25

What is pseudo-Bartter syndrome? (cystic fibrosis complication)

Answer 25

Hypochoremic metabolic alkalosis with hypokalaema, in the absence of renal tubular pathology, mostly occuring in neonatal period. Patients have high levels of choride and potassium in their sweat.

Question 26

What 5 conditions does the heel prick test test for?

Answer 26

Cystic Fibrosis Sickle Cell SCID (Severe Combined Immunodeficiency) Congenital hypothyroidism Inherited metabolic diseases

Question 27

Which organs are most affected in cystic fibrosis? (3)

Answer 27

Respiratory system Pancreas Intestine (bulky stools > intestinal blockage)

Question 28

What investigation (and results) which may be suggestive of cystic fibrosis in children?

Answer 28

Sweat test - Sweat Chloride >60mmol/L

Question 29

What additional tests would you order in Cystic Fibrosis? (3)

Answer 29

Lung Function test (FEV1, FVC, FEF) Sputum sample (resp infections) Oxygen saturations

Question 30

What is acute epiglottitis and what anatomical location does it effect?

Answer 30

A cellulitis (deep skin infection) of the supraglottis

Question 31

What pathogen commonly causes acute epiglottitis?

Answer 31

Haemophillus Influenzae Type B (Hib)

Question 32

What is the median age of presentation for acute epiglottitis?

Answer 32

Between 6-12 years old

Question 33

Name 4 red flag symptoms of acute epiglottitis (4 Ds)

Answer 33

Drooling (due to inability and pain on swallowing) Dyspnea Dysphonia (hoarsness of voice) Dysphagia (difficulty swallowing)

Question 34

What investigation is diagnostic for acute epiglottitis?

Answer 34

Direct visualisation via fibre optic laryngoscopy (showing inflamed epiglottis)

Question 35

List diagnostic features of acute epiglottitis (7)

Answer 35

Rapid onset High fever Sore throat +/- anterior neck tenderness Drooling Classic Tripod Positioning Cervical lymphadenopathy Stridor (emergency)

Question 36

What clinical feature of acute epiglottitis would be seen as an emergency?

Answer 36

Stridor. Suggests upper airway obstruction

Question 37

What clinical feature is more commonly seen in croup than acute epiglottitis?

Answer 37

Cough

Question 38

Describe the acute management of acute epiglotitis.

Answer 38

Secure airway - Lie patient un upright position > avoids aggrevating any airway obstruction. IV antibiotics (Cefotaxime/Ceftriaxone) Adjuncts; Oxygen + Dexamethasone

Question 39

Once stable, what oral antibiotic is given to patients with acute epiglotitis?

Answer 39

Amoxicillin

Question 40

What IV antibiotics are given to a patient with acute epiglottitis?

Answer 40

Cefotaxime/Ceftriaxone

Question 41

Name 4 complications of acute epiglottitis

Answer 41

Abscess formation Meningitis Sepsis Pneumothorax

Question 42

Name 2 risk factors for acute epiglottitis

Answer 42

Non vaccination with Hib vaccine. Immunocompromise

Question 43

What virus is associated with Ramsey Hunt Syndrome? (Shingles rash affecting facial nerve) (results in facial paralysis, hearing loss and rash)

Answer 43

Varicella Zoster Virus

Question 44

What virus causes glandular fever?

Answer 44

Epstein Barr Virus (EBV)

Question 45

What virus causes hand, foot and mouth disease in Children?

Answer 45

Coxsackie A virus

Question 46

What immunoglobulin and cytokine (IL) are involved in atopy (rhinitis/eczema ect)?

Answer 46

IgE and IL-4

Question 47

When should a child receive their MMR Jabs? (2 doses)

Answer 47

1st dose at 1 years old 2nd dose at 3 years and 4 months old

Question 48

What kind of virus is measles?

Answer 48

Single stranded RNA virus

Question 49

What is the incubation period for Measles?

Answer 49

2 weeks

Question 50

What are the 2 phases of Measles and what are their symptoms?

Answer 50

Catarrhal phase - Fever, Coryza, Conjunctivitis, Cough - Koplik spots (white spots on buccal mucosa) Exanthem phase (rash) - Erythematous maculopapular blanching rash (starts @ head and progresses to trunk/legs over a few days) - Generalised lymphadenopathy

Question 51

What are the 3Cs and 1K for Measles symptoms?

Answer 51

Coryza, Cough, Conjunctivitis. Koplik Spots

Question 52

What diagnostic test is used to diagnose Measles?

Answer 52

Measles specific IgM and IgG serology (ELISA)

Question 53

What does the presence of IgM and IgG antibodies indicate in Measles?

Answer 53

IgM indicates acute infection and tends to appear at onset of the exanthem stage. IgG indicates past infection or prior vaccination. IgG is not diagnostic but does support a +ve IgM result

Question 54

How is measles managed in children? (2)

Answer 54

Supportive treatment - Paracetamol and Ibuprofen Vitamin A supplementation

Question 55

Give 4 possible complications of measles. Which is the most common and what is the most common cause of death?

Answer 55

Pneumonia (most common cause of death) Otitis media (most common) Encephalitis Subacute sclerosing panencephalitis (SSPE)

Question 56

How long after measles infection does Subacute Sclerosing Panencephalitis tend to present?

Answer 56

7 - 10 years after

Question 57

What is Subacute Sclerosing Panencephalitis?

Answer 57

A generalised, demyelinating inflammation of the brain caused by persistent measles virus infection.

Question 58

Describe the 4 stages of Subacute Sclerosing Panencephalitis

Answer 58

Stage 1 - Dementia and Personality Changes Stage 2 - Epilepsy and Myoclonus Stage 3 - Spasticity and Extrapyramidal symptoms Stage 4 - Vegetative state and autonomic failure

Question 59

What diagnostic investigation would suggest Subacute Sclerosing Panencphalitis?

Answer 59

CSF fluid showing elevated anti-measles IgG

Question 60

If a child is not immunized against measles and they come into contact with someone with measles, how should this be managed?

Answer 60

Offer MMR vaccine. Vaccine induced measles antibodies develop more rapidly than the natural infection.

Question 61

Who should be notified if a patient has measles?

Answer 61

Public Health England

Question 62

What kind of virus is mumps?

Answer 62

RNA paramyxovirus

Question 63

During which seasons does mumps tend to present?

Answer 63

Winter and Spring

Question 64

How does Mumps typically manifest?

Answer 64

Parotitis (swelling and inflammation of the parotid glands)

Question 65

What is the incubation period for mumps?

Answer 65

14-21 days

Question 66

Describe the pattern of infectivity for mumps

Answer 66

Patients are infective 7 days before and 9 days after parotid swelling starts

Question 67

Give 3 complications of Mumps. Which is the most common?

Answer 67

Orchiditis (most common) Aseptic meningitis Hearing loss (less common since MMR vaccine)

Question 68

How is mumps managed?

Answer 68

Self limiting disease Supportive treatment - Paracetamol for high fever Notifiable Disease - Inform public health England

Question 69

What kind of virus is Rubella?

Answer 69

Positive sense, single stranded RNA virus

Question 70

What classic triad of symptoms is seen in congenital rubella syndrome?

Answer 70

Sensorineural hearing loss Cataracts Cardiac defects (i.e patent ductus arteriosus)

Question 71

Describe 4 clinical features of rubella

Answer 71

Maculopapular rash (begins in head and progresses to trunk/legs) Arthralgia General malaise, conjunctivitis and coryza Lymphadenopathy

Question 72

Rubella is generally a clinical diagnosis but what group of patients require a diagnostic investigation? and why?

Answer 72

Pregnant women require serology to confirm diagnosis (detection of IgM antibodies) Due to potential to develop congenital rubella syndrome

Question 73

Describe the management of rubella in pregnant and non-pregnant patients.

Answer 73

Pregnant - Specialist referral - IM immunoglobulin Non-pregnant - Supportive care - NSAIDs for arthralgia - Antihistamines for pruritis

Question 74

Why shouldn't you give NSAIDs in chickenpox/shingles?

Answer 74

Can cause pneumonitis

Question 75

During which trimester is it most dangerous for a pregnant woman to contract rubella? And why?

Answer 75

1st trimester as this is the period of organogenesis

Question 76

What is a classic triad of symptoms for Congenital Rubella Syndrome? (CCC)

Answer 76

Cardiac defects (most common - PDA, Pulmonary artery stenosis) Cataracts Cochlear defects (bilateral sensorineural hearing loss)

Question 77

What diseases does the 6 in 1 vaccine protect against?

Answer 77

Diphtheria Tetanus Pertussis (whooping cough) Polio Haemophalus influenzae b (Hib) Hepatitis B

Question 78

At what ages do children receive the 6 in 1 vaccine? (3 doses)

Answer 78

2 months, 3 months and 4 months

Question 79

At what age to boys and girls receive their HPV vaccine?

Answer 79

12-13 years old

Question 80

What vaccines do children aged 3.5 years receive? (5)

Answer 80

MMR Diphtheria, tetanus, pertussis, hepatitis B

Question 81

What pathogen does the fever pain score screen for?

Answer 81

Likely streptococcus infection

Question 82

What 5 parameters are measured in the fever pain score? (F-PAIN)

Answer 82

Fever in past 24 hours Pus on tonsils (purulent tonsils) Attend clinic rapidly (<3 days) Inflamed tonsils (severely) No cough or coryza (i.e pharyngeal illness)

Question 83

What pathogen typically causes Scarlet Fever?

Answer 83

Group A Streptococcus (Streptococcus pyogenes, GAS)

Question 84

What is the incubation period for Scarlet Fever?

Answer 84

2-4 days

Question 85

List symptoms for the Initial phase of Scarlet Fever (3)

Answer 85

Acute tonsilitis - Fever (lasting 24-28 hours) - Malaise, headache, myalgia - Sore throat

Question 86

List symptoms for the Exanthem phase of Scarlet fever (2)

Answer 86

Strawberry Tongue Scarlet coloured maculopapular rash - Blanches with pressure - Has a sandpaper texture

Question 87

When does the rash tend to appear in Scarlet Fever? And how long does it last?

Answer 87

Typically 12-48 hours after onset of fever. Lasts for 7 days

Question 88

List symptoms of the Desquamation phase of Scarlet fever and describe when it occurs.

Answer 88

Occurs 7-10 days after resolution of rash. Skin desquamation occurs on face, trunk, hands, fingers and toes

Question 89

What are the 1st, 2nd and 3rd line treatments for Scarlet Fever?

Answer 89

1st line - Phenoxymethylpenicillin (penicillin V) 2nd line - Amoxicillin (if penicillin v cannot be tolerated) 3rd line - Azithromycin (if penicillin allergy)

Question 90

What antibiotic is given in Scarlet Fever if the patient has a true penicillin allergy?

Answer 90

Azithromycin

Question 91

Give 4 possible complications of penicillin use.

Answer 91

Hypersensitivity reactions Haemolytic anaemia (positive Coombs test) Drug induced interstitial nephritis Seizures

Question 92

What kind of antibiotic is Penicillin?

Answer 92

Beta Lactam Antibiotic

Question 93

What kind of antibiotic is Azithromycin?

Answer 93

Macrolides

Question 94

In which patients is Azithromycin and Clarithromycin contraindicated?

Answer 94

Hepatic failure

Question 95

Give 3 possible side effects of azithromycin

Answer 95

GI Upset QT Prolongation Hypertrophic Pyloric Stenosis (in infants up to 6 weeks)

Question 96

Give 1 complication of Scarlet fever and describe it's how and when it may present.

Answer 96

Poststreptococcal glomerulonephritis May present 1-6 weeks following streptococcal infection Presents with; Haematuria Hypertension Oedema (facial)

Question 97

Chicken pox is a risk factor for invasive group A streptococcal soft tissue infections, such as necrotising fasciitis, how may this present?

Answer 97

Presence of a skin wound May be warm, oedematous and purple Skin may be peeling with white discharge

Question 98

Give 3 risk factors that would make a person high risk of invasive Group A Streptococcal infection and complications (i.e necrotising fasciitis).

Answer 98

Concurrent chickenpox or influenza Immunocompromised Comorbidities such as skin breakdown, diabetes or underlying malignancy

Question 99

What virus is responsible for Erythema infectiosum (slapped cheek syndrome)

Answer 99

Parvovirus B19

Question 100

What complication can Parvovirus B19 cause in adults?

Answer 100

Acute arthritis

Question 101

What complication can Parvovirus B19 cause in Pregnant women?

Answer 101

Hydrops fetalis

Question 102

What is PEFR

Answer 102

Peak Expiratory Flow Rate

Question 103

What is the PEFR in moderate Viral Induced Wheeze?

Answer 103

At least 50%

Question 104

What is the PEFR in acute severe Viral Induced Wheeze?

Answer 104

<50%

Question 105

What is the PEFR in severe Viral Induced Wheeze?

Answer 105

<33%

Question 106

What pathogen most commonly causes bronchiolitis?

Answer 106

RSV (respiratory syncytial virus)

Question 107

What is the most common lower respiratory infection in children <1 years old?

Answer 107

Bronchiolitis

Question 108

Give 4 risk factors for bronchiolitis

Answer 108

Social deprivation Winter months Prematurity or bronchopulmonary dysplasia Smoking and air pollution

Question 109

Give 4 symptoms of bronchiolitis

Answer 109

Symptoms are generally mild Corysal symptoms (mild fever, runny nose, sneezing) Cough Tachypnea Wheeze`

Question 110

Give 6 red flag symptoms of bronchiolitis that require hospital admission

Answer 110

Poor feeding Breathing difficulties (RR>70) Features of respiratory dirsress Apnea Central cyanosis Persistent O2 sats <92% when breathing air

Question 111

Give 3 symptoms that would make you 'consider' referring a child with bronchiolitis to hospital

Answer 111

Respiratory rate >60 Difficulty breast feeding or inadequate oral fluid intake (50-75% of usual volume) Clinical dehydration

Question 112

How is bronchiolitis diagnosed?

Answer 112

Patient must have coryzal symptoms lasting 1-3 days followed by; Persistent cough AND either, tachypnea or chest recession and Either wheeze or crackles (heard on auscultation)

Question 113

Give 1 common complicatin of bronchiolitis. How may it present?

Answer 113

Hyponatremia May present with; Tachypnoea, tachycardia and seizures

Question 114

Give 4 differentials for bronchiolitis

Answer 114

Pneumonia (consider if high fever + focal crackles) Viral induced wheeze or early onset asthma (consider in older infants) Cystic fibrosis Croup (inspiratory stridor, hoarse cry, barking cough)

Question 115

How is bronchiolitis managed?

Answer 115

Largely supportive; Chest physiotherapy Oxygen supplementation CPAP Upper airway suction (if apnea or respiratory distress_

Question 116

What is the most common cause of Croup?

Answer 116

Parainfluenza virus type 1 or type 3

Question 117

Give 5 classic symptoms of Croup

Answer 117

Sudden onset Seal-like barking cough Inspiratory stridor Voice hoarseness Respiratory distress (more severe cases)

Question 118

What can cause symptoms of Croup to increase?

Answer 118

Agitation

Question 119

Describe mild croup (3)

Answer 119

Seal like braking No stridor or sternal/intercostal recessions at rest Symptoms resolve in 48 hours

Question 120

Describe moderate croup (4)

Answer 120

Seal like braking Stridor AND sternal/intercostal recessions at rest. Little or no agitation or lethargy Requires hospital admission

Question 121

Describe severe croup (4)

Answer 121

Seal like braking Stridor AND sternal/intercostal recessions Associated with agitation/lethargy Requires hospital asmission

Question 122

What ages does croup typically present?

Answer 122

Between 6 months and 3 years old

Question 123

What season is croup most common?

Answer 123

Autumn (September to December)

Question 124

Give 3 differentials for croup

Answer 124

Bacterial tracheitis Epiglottitis Foreign body in upper airway

Question 125

What is the 1st line treatment for Croup? What is the 2nd line if the child is too unwell for the 1st? (non-emergency)

Answer 125

1st line - Oral Dexamethasone 2nd line - Inhaled budenoside or IM dexamethasone

Question 126

What is the emergency management of croup? (3)

Answer 126

Nebulised adrenaline (epinephrine) Corticosteroids (dexamethasone oral or IM/budenoside) Supplemental oxygen

Question 127

What is the 1st line antibiotic for pneumonia in a child >1 with non-severe symptoms? (plus penicillin allergy?)

Answer 127

Amoxicillin Penicillin allergy - Clarithromycin

Question 128

What is the 1st line antibiotic for pneumonia in a child >1 with severe symptoms?

Answer 128

Co-amoxiclav

Question 129

Describe 4 severe symptoms of pneumonia in children

Answer 129

Difficulty breathing O2 sats <90% Signs of respiratory distress Poor feeding

Question 130

What type of hypersensitivity reaction is Asthma and what antibody is it mediated by?

Answer 130

Type 1 hypersensitivity reaction. IgE mediated

Question 131

What are the 2 types of asthma?

Answer 131

Allergic/Eosinophilic - Atopy (IgE mediated) Non-allergic/Non-eosinophilic (associated with environmental factors., i.e smoking, cold, pollution ect)

Question 132

What is the primary diagnostic test for asthma and what result would you expect?

Answer 132

Spirometry FEV1/FVC <80% predicted

Question 133

What test should be performed on all patients presenting with an acute asthma attack and why?

Answer 133

Peak Expiratory Flow Rate (PEFR) Measures airflow obstruction so can indicate severity.

Question 134

Describe the drug ladder for Asthma (7)

Answer 134

1. SABA 2. Low Dose ICS (beclometasone) + SABA 3. LTRA (montelukast) + SABA 4. LABA (salmeterol) +SABA 5. Combined ICS and LABA in single inhaler (MART) 6. Combined ICS and LABA in single inhaler (increase dose of ICS) 7. Consider adding oral theophyline or oral beta agonist

Question 135

What is the criteria for moderate asthma? (1)

Answer 135

PEFR 50-75%

Question 136

What is the criteria for severe asthma? (4)

Answer 136

PEFR 33-50% RR >25 HR >110 Can't complete sentences in one breath

Question 137

What is the criteria for life threatening asthma? (7)

Answer 137

Any one of CHEST. PEFR <33% Sp02 <92% Cyanosed Hypotension Exhaustion, confusion Silent chest Tachy/brady/arrhythmas

Question 138

What is the 1st line treatment for acute severe asthma?

Answer 138

Oxygen + SABA + Prednisolone (or IV hydrocortisone) Switch SABA to ipratropium bromide if poor response. Quadruple prednisolone dose and give for 14 days

Question 139

What is the 1st line for acute life threatening asthma?

Answer 139

Oxygen + SABA/Ipratropium bromide + Prednisolone (or IV hydrocortisone)

Question 140

Once stable, what should patients whom have experienced acute asthma be prescribed to prevent future relapses?

Answer 140

Oral prednisolone

Question 141

Define Kawasaki Disease

Answer 141

An acute, febrile (feverish), systemic vasculitis of unknown origin

Question 142

What complication is common in Kawasaki disease?

Answer 142

Coronary artery aneurysms

Question 143

Give 5 symptoms of Kawasaki Disease (CRASH)

Answer 143

C - Conjunctivitis R - Rash (blanching maculopapular) A - Adenopathy S - Strawberry tongue H - Hand and Feet changes (desquamation)

Question 144

What is the main difference in symptoms between Scarlet fever and Kawasaki Disease?

Answer 144

Scarlet fever - Fever <5 days Kawasaki disease - Fever >5 days

Question 145

What is an important diagnostic test for patients with Kawasaki Disease?

Answer 145

Echocardiogram - To screen for coronary artery aneurysms

Question 146

What are the 1st and 2nd line treatments for Kawasaki Disease?

Answer 146

1st line - IV immunoglobulins (IVIG) + High dose aspirin 2nd line - Corticosteroids - Methylprednisolone/prednisolone

Question 147

What risk does aspirin use bring in children?

Answer 147

Developing Reye's syndrome

Question 148

What is Reye Syndrome?

Answer 148

Describes a rare type of hepatic encephalopathy associated with aspirin use in children

Question 149

Give 2 clinical features of Reye syndrome

Answer 149

Preceeding viral infection - Symptoms begin 3-5 days after viral illness Acute encephalopathy - Severe vomiting - Altered mental status - Neuro symptoms - Seizures, fixed pupils -

Question 150

What investigation results would you see in Reye's syndrome? (4)

Answer 150

LFTs - Raised AST/ALT Hyperammonemia Hypoglycemia Metabolic acidosis

Question 151

Define Juvenile Idiopathic Arthritis

Answer 151

A collection of chronic paediatric inflammatory diseases characterised by onset before 16 years old and the presence of arthritis for at least 6 weeks.

Question 152

Name 6 types of JIA. Which is the most common?

Answer 152

Oligoarticular JIA (Most common) Seronegative polyarticular Seropositive polyarticular Systemic JIA Psoriatic JIA Enthesitis-related JIA

Question 153

Define oligoarticular JIA

Answer 153

Describes paediatric arthritis involving up to 4 joints within 6 months of disease onset.

Question 154

Give 2 clinical features of oligoarticular JIA

Answer 154

Asymmetrical pattern (most commonly affecting weight bearing joints - Knee and Ankle) Extra-articular manifestations - Chronic anterior uveitis (bilateral is common)

Question 155

Give 3 investigation results you'd expect to see in a patient with oligoarticular JIA

Answer 155

Positive ANA (70%) Negative RF Raised ESR

Question 156

Give 4 risk factors for JIA

Answer 156

Female sex HLA polymorphism Age under 6 Family history of autoimmunity

Question 157

What is the 1st and 2nd line (and adjuncts) treatments for oligoarticular JIA

Answer 157

1st line - Intra-articular corticosteroid (Triamcinolone or Methylprednisolone acetate) 2nd line - TNF alpha inhibitors (Adalimumab or Etanercept) Adjuncts; NSAIDs, Methotrexate

Question 158

When should TNF-alpha (2nd line) inhibitors be considered in patients wit oligoarticular JIA?

Answer 158

When disease activity is moderate/high and there are poor prognostic features after 3 months of treatment with methotrexate and intra-articular corticosteroids

Question 159

Give 2 contraindications for Adalimumab (TNF-a inhibitor)

Answer 159

Demyelinating diseases Hepatitis B

Question 160

What factor is positively present in seropositive polyarticular arthritis and not in seronegative?

Answer 160

RF factor

Question 161

Give 2 clinical features of seronegative polyarticular arthritis. (mention which joints are commonly affected)

Answer 161

Symmetrical or asymmetrical pattern of joint involvement. (Most commonly affects with cervical spine and temporomandibular joints) Chronic anterior uveitis

Question 162

What investigation results would you expect in seronegative polyarticular arthritis?

Answer 162

Positive ANA (30%) Negative RF Raised ESR

Question 163

Define polyarticular arthritis

Answer 163

Describes arthritis involving >5 joints within 6 months of disease onset

Question 164

What pattern of incidence is displayed in seronegative polyarticular arthritis?

Answer 164

Bimodal incidence. Presents between 1-4 years old and 6-12 years old.

Question 165

Give 2 clinical features of seropositive polyarticular arthritis

Answer 165

Symmetrical pattern of joint involvement Rheumatoid nodules (present on extensor surface of elbows and Achilles tendon (30% of cases))

Question 166

What are the 1st and 2nd line treatments for polyarticular JIA?

Answer 166

1st line - DMARD - Methotrexate 2nd line - Lefunomide/Sulfasalazine

Question 167

What are the 1st and 2nd line adjuncts for polyarticular JIA?

Answer 167

1st line adjunct - TNF-a inhibitor (adalimumab) 2nd line adjunct - IL-6 inhibitor (tocilizumab)

Question 168

What kind of drug is tocilizumab?

Answer 168

IL-6 inhibitor

Question 169

What kind of drug is adalimumab?

Answer 169

TNF-a inhibitor

Question 170

What should be given in conjunction with methotrexate? and why?

Answer 170

Folic acid. To decrease side effects (Anaemia)

Question 171

What type of anaemia occurs with methotrexate treatment?

Answer 171

Megaloblastic anaemia (due to folic acid deficiency)

Question 172

What 3 tests should be conducted before starting methotrexate? And when should the be repeated?

Answer 172

FBC, Creatinine and LFTs Repeat every 3-4 months during treatment

Question 173

What should patients be screened for before starting methotrexate?

Answer 173

Hepatitis B and C

Question 174

Give 1 serious side effect of tocilizumab (IL-6 inhibitor) and suggest 2 tests to monitor this.

Answer 174

Hepatotoxicity Monitor ALT and AST every 4-8 weeks during 1st month of treatment.

Question 175

Define Systemic JIA (Still Disease(

Answer 175

Describes arthritis involving >1 joint AND intermittent fever that lasts for at least 2 weeks with fever spikes occulting on at least 3 consecutive days AND >1 extra-articular manifestation.

Question 176

Give 4 extra-articular manifestations seen in Systemic JIA (Still Disease)

Answer 176

Salmon pint macular rash (non-pruritic) Generalised lymphadenopathy Spleno/Hepatomegaly Serositis (peritonitis, pleuritis and/or pericarditis)

Question 177

What is Koebner's phenomenon? (systemic JIA)

Answer 177

Phenomenon whereby salmon pink rash can be elicited by scratching the skin

Question 178

Give 3 laboratory findings you'd expect to see in systemic JIA

Answer 178

Raised ESR (chronic) and CRP (acute) Anaemia, Leukocytosis and Thrombocytosis RF negative

Question 179

What is the 1st and 2nd line treatment for Systemic JIA?

Answer 179

1st line Oral/IV corticosteroid - Methylprednisolone or Prednisolone 2nd line - Tocilizumab (IL-6 inhibitor), Canakinumab or Anakinra (IL-1 inhibitor)

Question 180

What kind of drug is Anakinra?

Answer 180

IL-1 inhibitor

Question 181

What kind of drug is Canakinumab?

Answer 181

IL-1b monoclonal antibody

Question 182

Define psoriatic arhritis

Answer 182

Describes either; Arthritis + Psoriasis OR Arthritis and >=2 of the following; - Dactylitis - Nail changes (pitted nails, onycholysis) - 1st degree relative with psoriasis

Question 183

Describe 2 clinical features of psoriatic JIA

Answer 183

Asymmetrical pattern of joint involvement Extra-articular manifestations; - Chronic anterior uveitis - Nail changes - Psoriatic skin lesions

Question 184

Give 3 investigation findings you'd expect in a patient with psoriatic JIA

Answer 184

ANA positive (50%) HLA-B27 may be positive RF negative

Question 185

Describe the management of psoriatic JIA

Answer 185

NSAIDs and Intra-articular steroid injections (Triamcinolone or Methylprednisolone acetate)

Question 186

What is enthesitis?

Answer 186

Inflammation of the enthesis (the site where Ligaments and Tendons attach to Bone)

Question 187

Define Enthesitis related JIA

Answer 187

Arthritis with Enthesitis

Question 188

Give 2 clinical features of Enthesitis related JIA

Answer 188

Asymmetrical pattern of join involvement. (may have sacroiliac tenderness and/or inflammatory lumbosacral pain) Extra-articular mainfestations; - Acute anterior uveitis - IBS - May progress to Ankylosing Spondylitis

Question 189

Give 2 investigation findings you may see in a patient with Enthesitis related JIA

Answer 189

HLA-B27 positive (80%) RF negative

Question 190

Give 5 differentials for JIA

Answer 190

Septic arthritis (fever + malaise) Perthes disease (hip w/ limp) Malignancy (leukaemia/lymphomas - may present with swelling) Non-accidental injury Reactive arthritis (2nd to viral throat infection - 2 weeks after infection)

Question 191

What is Macrophage Activation Syndrome (MAS)? How is it characterised?

Answer 191

Rare complication of Systemic JIA Characterised by; Thrombocytopenia Elevated transaminases Low fibrinogen Increased ferritin

Question 192

What is the treatment for macrophage activation syndrome?

Answer 192

IV prednisolone +/- cyclosporine

Question 193

Define Perthes' disease. Between what ages does it typically manifest?

Answer 193

Describes an idiopathic avascular necrosis of the femoral head. Manifests between the ages of 4-10.

Question 194

What type of gait is seen in Perthes disease?

Answer 194

Antalgic gait (on weight bearing leg)

Question 195

Give 3 clinical features of Perthes' disease

Answer 195

Antalgic gait (on weight bearing leg) Hip pain projecting to knee (exacerbated by internal rotation) Restricted range of movement (especially regarding internal rotation and abdunctio)

Question 196

What may an X-ray show in a patient with Perthes' disease? (2)

Answer 196

Widening joint space (early) Decreased femoral head size/flattening (later)

Question 197

Give 2 complications of Perthes' disease

Answer 197

Osteoarthritis Premature fusion of growth plates

Question 198

What is the management of Perthes' disease in a child <6 years old

Answer 198

Conservative management (monitor)

Question 199

What is the management of Perthes' disease in a child >6 years old

Answer 199

Femoral osteotomy

Question 200

Give 3 risk factors for Perthes' disease

Answer 200

Male sex Hyperactivity Hyper-coagulable states (Excess factor VII, Factor V Leiden, Protein S deficiency)

Question 201

What does developmental dysplasia of the hip refer to?

Answer 201

DDH refers to; Hip instability Subluxation/dislocation of the femoral head and/or Acetabular dysplasia in the developing hip joint.

Question 202

Give 3 examination findings you'd likely see in a patient with DDH

Answer 202

Positive Barlow test (hip adduction) Positive Ortolani test (Hip Abduction) Limited hip abduction

Question 203

What are the 4 main risk factors for DDH?

Answer 203

Female sex Breech presentation at birth Positive family history Oligohydramnios (too little amniotic fluid)

Question 204

What does a positive Barlow test show?

Answer 204

Positive shows reduced hip is subluxatable or dislocatable

Question 205

What does a positive Ortolani test show?

Answer 205

Positive shows that the dislocated hip is reducible

Question 206

What clinical features would an infant <6 months with DDH have? (2)

Answer 206

Asymptomatic Positive Barlow and Ortolani Signs

Question 207

What clinical features would an infant 6-18 months with DDH have? (3)

Answer 207

Inability to abduct hip Prominent Galeazzi sign (unequal knee height when lying supine) Asymmetrical gluteal folds

Question 208

What clinical features would an infant >18 months with DDH have? (4)

Answer 208

Hip pain Hip deformity (coxa vara - decreased femoral angle) Waddling or Trendelenburg gait Leg Length Discrepency

Question 209

Give 3 criteria that warrant screening for DDH

Answer 209

1st degree relative with DDH/early life hip problems Breech presentation at or after 36 weeks Multiple pregnancy

Question 210

What is the 1st line investigation for DDH in infants <4.5 months

Answer 210

Ultrasound at 6 weeks

Question 211

What is the 1st line investigation for DDH in infants >4.5 months?

Answer 211

X-ray

Question 212

What are the 1st and 2nd line treatments for an infant <6 months with DDH?

Answer 212

1st line - Observation 2nd line - Hip abduction orthosis (splint) and further evaluation at 6 months

Question 213

What are the 1st and 2nd line treatments for infants with DDH aged between 6-18 months?

Answer 213

1st line - Closed reduction with spica casting 2nd line - Open reduction with spica casting

Question 214

Give 6 red flag features of clinical dehydration in a child <5

Answer 214

Appears to be unwell or deteriorating Altered responsiveness (irritable, lethargic) Sunken eyes Tachycardia Tachypnoea Reduced skin tugor

Question 215

Give 5 symptoms that suggest hypernatraemic dehydration in a child <5

Answer 215

Jittery movements Increased muscle tone Hyperreflexia Convulsions Drowsiness or Coma

Question 216

When is screening for Down's, Pagets and Edwards syndrome conducted?

Answer 216

Between 10-14 weeks pregnancy

Question 217

Which chromosome is tripled in Down's Syndrome?

Answer 217

Chromosome 21 (Trisomy 21)

Question 218

Give 6 appearance characteristics for an individual with Down's syndrome

Answer 218

Upward slanting eyelids (palpebral fissures) Epicanthal folds (eyes) Low set ears Protruding tongue Short stature Transverse palmar crease

Question 219

What is the most common heart defect in Down's Syndrome? Describe its pathophysiology and what can result from it.

Answer 219

Atrioventricular Septal Defect (left to right shunt) Results in excessive pulmonary blood flow and biventricular volume overload leading to pulmonary hypertension and heart failure

Question 220

What type of shunt occurs in a Atrioventricular Septal Defect?

Answer 220

Left to Right Shunt

Question 221

What GI defects may occur in Down's Syndrome? (2)

Answer 221

Duodenal atresia/stenosis Hirschprung disease

Question 222

What urogenital defects may occur in Down's Syndrome? (2)

Answer 222

Hypogonadism Decreased fertility (men)

Question 223

What cancer are people with Down's syndrome more susceptible to?

Answer 223

Acute Lymphoblastic Leukaemia

Question 224

Give 6 complications of Down's Syndrome

Answer 224

Hypothyroidism Type 1 diabetes Obstructive sleep apnea (snoring) Hearing loss (due to otitis media) Early onset Alzheimer's Disease Learning Difficulties

Question 225

Why does Down's syndrome increase susceptibility to Alzheimer's disease?

Answer 225

The gene encoding amyloid precursor protein (generates amyloid beta) is located on Chromosome 21. (overexpressed)

Question 226

When does Hirschprung's Disease typically present?

Answer 226

1st year of life

Question 227

Give 4 features of Hirschprung's disease

Answer 227

Vomiting Abdominal Distension Enterocolitis Constipation

Question 228

How is Hirschprung's disease diagnosed? What will the test show?

Answer 228

Rectal biopsy showing absence of ganglion cells

Question 229

Describe the pathophysiology of Hirschprung's disease

Answer 229

Absence of ganglion cells (aganglionosis) results in the lumen of the distal colon being tonically contracted, causing a functional obstruction.

Question 230

How does duodenal atrasia/stenosis (down's syndrome) present intrauterine (1) and post-partum (3)?

Answer 230

Intrauterine - Polyhydramnios Post-partum - Vomiting, Upper abdomen distension and delayed meconium passage

Question 231

What diagnostic test is used to diagnose duodenal atrasia/stenosis prenatally? What will it show?

Answer 231

Ultrasound. Shows Double Bubble Sign

Question 232

What diagnostic test is used to diagnose duodenal atrasia/stenosis postnatally? What will it show?

Answer 232

Abdominal X-ray. Shows Gasless distal bowel and Double Bubble Sign

Question 233

Give 2 drugs that are contraindicated in Down's Syndrome. Suggest why.

Answer 233

Carbamazepine and Phenytoin Carbamazepine can exacerbate hypothyroidism

Question 234

How may otitis media present in Down's Syndrome? (3)

Answer 234

Otalgia (ear pain) Bulging tympanic membrane Myringitis (erythema of tympanic membrane)

Question 235

When is the quadruple test conducted to screen for Down's Syndrome?

Answer 235

15-18 weeks

Question 236

What quadruple test findings would suggest Down's Syndrome? (4)

Answer 236

Decreased free Estriol Decreased Alpha Fetoprotein (AFP) Increased Inhibin A Increased B-hCG (B-hCG and Inhibin A are HIgh up while Estriol and alpha- fEtoprotein are dEficient)

Question 237

Patau's Syndrome describes an abnormality in which chromosome?

Answer 237

Chromosome 13 (Trisomy 13)

Question 238

Give 4 clinical features of Patau's Syndrome

Answer 238

Microcephaly/Holoprosencephaly Small eyes Polydactyly (presence of >5 fingers/toes) Rocker bottom feet (Convex deformity of plantar aspect of foot - vertically positioned talus and prominent heel)

Question 239

Give 2 complications of Patau's syndrome

Answer 239

Cardiac defects - Ventricular Septal Defect and Patent Ductus Arteriosus Polycystic Kidney Disease

Question 240

Edward's Syndrome describes an abnormality in which chromosome?

Answer 240

Chromosome 18 (trisomy 18)

Question 241

Give 4 clinical characteristics of Edward's Syndrome

Answer 241

Micrognathia (congenital mandibular hypoplasia - undersized jaw) Low-set ears Overlapping fingers/clenched fists Rocker-bottom feet

Question 242

What results are seen in a Quadruple test positive for Edward's Syndrome?

Answer 242

Decreased Estriol Decreased AFP Decreased B-HCG Normal/Decreased Inhibin A

Question 243

What is the most significant risk factor for Downs, Patau's and Edwards syndrome?

Answer 243

Increased maternal age

Question 244

What is the pattern of inheritance in Fragile X syndrome?

Answer 244

X-linked dominant

Question 245

What mutation is seen in what gene in Fragile X syndrome?

Answer 245

CGG trinucleotide repeat in FMR1 gene (during oogenesis)

Question 246

Fragile X syndrome affects which sex more? And why?

Answer 246

Males. Because it is X-linked dominant (males only inherit 1 x chromosome)

Question 247

The severity of Fragile X syndrome features depends on what variable?

Answer 247

The number of CGG trinucleotide repeats

Question 248

Give 6 clinical features of Fragile X Syndrome

Answer 248

Male Intellectual disability (+/- autism/hyperactivity) Microcephaly Large ears, testes (macro-orchidism) and face Mitral valve prolapse > mitral regurgitation Hypermobile joints

Question 249

Fragile X syndrome patients are susceptible to mitral valve prolapse, leading to mitral regurgitation. How may this sound on auscultation?

Answer 249

Soft, Decrescendo Pan Systolic murmur (radiates to axilla)

Question 250

Prader Willi And Angelman Syndrome occur due to what genetic abnormalities?

Answer 250

“Prader misses his Papa” “Angel misses her Mother” Prader Willi Syndrome occurs due to mutation/deletion of Paternal gene copy and maternal gene methylation. Angelman syndrome occurs due to mutation/deletion in Maternal gene copy with paternal gene methylation.

Question 251

Give 5 clinical features of of Prader Willi Syndrome

Answer 251

Muscular Hypotonia and poor feeding during infancy Hyperphagia + Childhood obesity Developmental delay/short stature Hypogonadism and infertility Behavioural problems - OCD, temper ect

Question 252

Give 3 complications of Prader Willi Syndrome

Answer 252

Sleep apnea (most common) Type 2 diabetes Obesity

Question 253

Give 5 clinical features of Angelman Syndrome

Answer 253

Epileptic Seizures Easily excitable (may show inappropriate laughter) Ataxia Intellectual disability Fascination with Water

Question 254

Describe William's Syndrome

Answer 254

William's Syndrome is a multisystem developmental disorder caused by a deletion in chromosome 7

Question 255

William's Syndrome is caused by a deletion in which chromosome?

Answer 255

Chromosome 7

Question 256

Give 5 clinical features of William's Syndrome

Answer 256

Hypersociability Learning Difficulties Cardiac Disease (supravalvular aortic stenosis and renal artery stenosis) Hypercalcaemia (due to increased sensitivity to vitamin D) Elfin Facies (elven faces)

Question 257

How are Prader Willi, Angelman and William's Syndrome diagnosed?

Answer 257

FISH (fluorescence in-situ hybridisation)

Question 258

What chromosome is deleted in Prader Willi and Angelman Syndrome

Answer 258

Chromosome 15

Question 259

What hormone is elevated in Prader Willi Syndrome? What symptom does this lead to?

Answer 259

Ghrelin Hyperphagia (extreme hunger)

Question 260

What is nocturnal enuresis?

Answer 260

Bed wetting

Question 261

What is the 1st line treatment and 2nd line for nocturnal enuresis in children >5?

Answer 261

1st line - Enuresis alarm (sensor pads that sense wetness) 2nd line - Desmopressin (short term control, can be used for sleep overs or if enuresis alarm is ineffective)

Question 262

What is the 1st line management for nocturnal enuresis in children <5?

Answer 262

Reassurance and lifestyle advice (regular toileting and DONT restrict fluids)

Question 263

At what age is hearing first formally assessed? What test is used?

Answer 263

Newborn - Otoacoustic emission

Question 264

What ratio of chest compressions:ventilation is given to children?

Answer 264

15:2

Question 265

What should you do before chest compressions in paediatric basic life support?

Answer 265

5 rescue breaths

Question 266

What is the gold standard investigation for stable children with suspected Meckel's diverticulum?

Answer 266

Technetium scan

Question 267

What is the 1st line management for Hirschprungs disease? and why?

Answer 267

Rectal washouts/bowel irrigation To prevent entrocolitis

Question 268

When is Meningitis B vaccine given (3)?

Answer 268

2 months, 4 months and 12 months

Question 269

What is the pattern of inheritance for Von Willebrand disease?

Answer 269

Autosomal dominant

Question 270

Describe the pathophysiology of Von Willebrand disease (3)

Answer 270

Reduction or malfunction in Von Willebrand Factor Factor usually promotes platelet adhesion to damaged endothelium. VWF also stabilizes and carries factor VIII

Question 271

What factor does Von Willebrand Factor stabilize and carry?

Answer 271

Factor VIII

Question 272

Give 5 symptoms of Von Willebrand Disease

Answer 272

Bleeding from minor wounds Mucosal bleeding Excessive post-operative bleeding Easy/excessive bruising Epistaxis (nose bleeds)

Question 273

Give 2 diagnostic tests and results for Von Willebrand Disease

Answer 273

Prolonged Activated Partial Thromboplastin Time (APTT) Reduced Factor VIII activity

Question 274

What type of medication should be avoided in Von Willebrand disease?

Answer 274

NSAIDs

Question 275

Give 2 medications used in the management of Von Willebrand disease

Answer 275

Desmopressin (increases clotting factor VWF and Factor VIII) Von Willebrand Factor/Factor VIII concentrate (used in surgery for major bleeds)

Question 276

Von Willebrand Disease is a disease of what process?

Answer 276

Platelet adhesion

Question 277

Give 1 medication which may exacerbate symptoms of Von Willebrand Disease. Suggest why

Answer 277

Aspirin As it inhibits platelet function

Question 278

What clotting factor is deficient in haemophila A

Answer 278

Factor VIII

Question 279

What clotting factor is deficient in haemophila B

Answer 279

Factor IX

Question 280

What clotting factor is deficient in haemophila C

Answer 280

Factor XI

Question 281

In what population is haemophila C more common?

Answer 281

Jewish

Question 282

What is the pattern of inheritance for Haemophilia A?

Answer 282

X-linked recessive

Question 283

Give 5 clinical features of Haemophilia A

Answer 283

Male Spontaneous bleeding or delayed onset bleeding in response to trauma Bleeding into soft tissues (knee, illiopsoas and neck) Recurrent bruising or haematoma formaiton Oral mucosa bleeding, epistaxis or excessive bleeding following minor trauma

Question 284

Give 3 diagnostic test results that would be seen in Haemophilia A diagnosis (2 normal, 1 prolonged)

Answer 284

Prolonged Activated Partial Thromboplastin Time (aPTT) Normal platelet count Normal prothrombin time

Question 285

Give 3 treatments for haemophilia

Answer 285

Substitution of clotting factors (VII - A, IX - B, XI - C) Desmopressin (used in mild hemophilia A) Tranexamic acid (inhibits break down of clots to reduce clotting risk)

Question 286

What chromosomal abnormality is commonly seen in chronic myeloid leukaemia? (And occasionally Acute Lymphoblastic Leukaemia)

Answer 286

Philadelphia chromosome (t9:22) BCR-ABL gene fusion

Question 287

In what chromosome is the Philadelphia Chromosome an abnormality?

Answer 287

Chromosome 22

Question 288

What is the most common childhood cancer?

Answer 288

Acute Lymphoblastic Leukaemia (ALL)

Question 289

Describe the pathophysiology of acute lymphoblastic leukaemia (3)

Answer 289

Malignancy of lymphoid cells, affecting B or T lymphocyte lineages. Describes the arrested maturation and uncontrolled proliferation of immature lymphocyte precursor cells (lymphoblasts/blast cells) Results in bone marrow failure and tissue infiltration.

Question 290

Describe the prognosis of ALL in children (relate to chromosomal abnormality) (2)

Answer 290

Philadelphia chromosome (T9:22) - Bad prognosis - Hypodiploidy T(12:21) - Good prognosis - Hyperdiploidy

Question 291

Give 2 risk factors for ALL

Answer 291

Ionizing radiation (x-rays) exposure during pregnancy Downs syndrome (trisomy 21)

Question 292

Give 7 clinical features of ALL

Answer 292

Sudden onset and rapid progression (days/weeks) Fever, nightsweats and unexplained weight loss Marrow failure (anaemia, Infection, Bruising/bleeding) Painless lymphadenopathy Bone pain Hepatosplenomegaly Meningeal leukemia (headache and cranial nerve palsies)

Question 293

Give 3 indications of bone marrow failure

Answer 293

Anaemia (low Hb) Infection (low functioning WC) Bleeding and bruising (low platelets)

Question 294

What is tumour lysis syndrome?

Answer 294

Condition triggered by combination chemotherapy. Occurs when a large number of cancer cells die within a short period, releasing their contents into the blood.

Question 295

Biochemically, how does tumour lysis syndrome present? (4)

Answer 295

High uric acid (hyperuricemia) High potassium (hyperkalaemia) High phosphate (hyperphosphatemia) Low calcium (hypocalcaemia)

Question 296

What is used in the treatment of tumour lysis syndorme?

Answer 296

Allopurinol (xanthine oxidate inhibitor - reduced uric acid production) Rasburicase (recombinant version of urate oxidase - enzyme that metabolise uric acid into allantoin)

Question 297

What are the 3 types of beta thalassemia?

Answer 297

Minor (trait- carrier) Intermedia Major

Question 298

Give 5 clinical features of beta thalassaeia majora

Answer 298

Children <1 Failure to thrive Recurrent bacterial infections Severe anaemia from 3-6 months) Extramedullary haematopoiesis > hepatosplenomegaly + bone expansion

Question 299

What sign is seen on skull x-ray of patients with beta thalassemia majora?

Answer 299

Hair on end sign

Question 300

What type of blood cells would be seen on a blood film in Beta Thalassemia majora?

Answer 300

Large and small irregular hypochromic red blood cell

Question 301

What is the pattern of inheritance on Sickle Cell anaemia?

Answer 301

Autosomal recessive

Question 302

What does being heterozygous for sickle cell anaemia protect against?

Answer 302

Falciparum malaria

Question 303

Describe 1 complication of sickle cell anaemia (3)

Answer 303

Vaso-occlusive crisis Deformed sickle cells have a strong adherence to the endothelium, leading to vaso-occlusive crisis. The obstruction of small vessels can lead to tissue infarction and intense pain.

Question 304

Give 5 triggers for vaso-occlusive crisis in sickle cell anaemia

Answer 304

Hypoxia Cold weather Infection Dehydration Acidosis

Question 305

How may vaso-occlusive crisis present in children with sickle cell anaemia?

Answer 305

Dactylitis (inflammation of digits)

Question 306

What test is used to confirm diagnosis of sickle cell anaemia? What does it help distiunguish?

Answer 306

Hb Electrophoresis Distinguishes HbSS (homozygotes) and HbAS (heterozygotes) states

Question 307

What is the 1st line long term management of sickle cell anaemia? How does it work?

Answer 307

Hydroxyurea Increases HbF levels

Question 308

What vaccine should sickle cell patients receive every 5 years?

Answer 308

Pneumococcal polysaccharide vaccine

Question 309

What is the MOA for tranexamic acid

Answer 309

Inhibits the lysing binding sites of plasminogen thus inhibiting the conversion to plasmin

Question 310

How are chest compressions performed in children >1?

Answer 310

Single hand compressing the lower half of the sternum. 30:2

Question 311

How are chest compressions performed in children <1?

Answer 311

Two thumb encircling technique

Question 312

What is the most common cause of gastroenteritis in children?

Answer 312

Rotavirus

Question 313

What type of virus is rotavirus?

Answer 313

RNA retrovirus

Question 314

How is rotavirus transmitted?

Answer 314

Faecal-oral route

Question 315

When is the rotavirus vaccine given? (2)

Answer 315

8 weeks old and 12 weeks old

Question 316

3 risk factors for viral gastroenteritis

Answer 316

Lack of immunisation against rotavirus Age <5 years Exposure to people with gastroenteritis

Question 317

Give 4 clinical features of gastroenteritis

Answer 317

Vomiting (NICE say usually lasts 1-2 days and stops within 3 days) Non-bloody diarrhoea (NICE say usually lasts 5-7 days and stops within 2 weeks) Abdominal pain Evidence of dehydration

Question 318

How is gastroenteritis diagnosed

Answer 318

Clinical diagnosis

Question 319

If a dehydrated child weighs 5kg how is their fluid replacement calculated?

Answer 319

Weight 0-10kg = 100ml/Kg fluids 5kg child receives 500ml/day fluid (maintenance fluid volume)

Question 320

If a dehydrated child weighs 15kg how is their fluid replacement calculated?

Answer 320

Weight 10-20kg = 1000ml/kg + 50ml/kg for each kg above 10kg per day 15kg child receives 1250ml/day fluid (maintenance fluid volume)

Question 321

If a dehydrated child weighs 25kg how is their fluid replacement calculated?

Answer 321

Weight >20kg = 1500ml/Kg + 20ml/Kg for each Kg above 20kg per day 25kg child receives 1600ml/day fluid (maintenance fluid volume

Question 322

How is Low Osmolarity Oral Rehydration Salt given in children based on age? (3)

Answer 322

<5 years old - 50ml/kg over 4 hours + ORS solution for maintenance Age 5-11 - 200mL ORS after each loose stool Age 12-16 - 200-400mL ORS solution after loose stool

Question 323

Give 2 complications of gastroenteritis

Answer 323

Clinical dehydration Hypernatremia dehydration

Question 324

Give 6 risk factors making a child increased risk of dehydration

Answer 324

Infants <1 (particularly <6 months) Low birthweight Passed >6 stools in last 24 hours Vomited >3 times in last 24 hours Stopped breastfeeding Signs of malnutrition

Question 325

Give 4 signs of clinical shock in children

Answer 325

Decreased level of consciousness Pale/mottled skin Cold extremities Tachycardia/tachypnea/hypotension

Question 326

How is appendicitis pain often described?

Answer 326

Presents as umbilical pain that moves to the lower right quadrant

Question 327

What organ is the appendix connected to?

Answer 327

Caecum (below the ileocecal valve)

Question 328

What is the main cause of appendicitis?

Answer 328

Luminal obstruction caused by faecolith > infection

Question 329

Give 5 complications of appendicitis

Answer 329

Abscess formation Peritonitis Sepsis Intra-abdominal adhesions Bowel obstruction

Question 330

Give 5 clinical features of appendicitis

Answer 330

Umbilical pain that moves to the lower right quadrant (McBurney's Point) Abdominal guarding and rebound tenderness (peritonitis) Low grade fever, pyrexia and malaise Nausea and vomiting Constipations

Question 331

Name and describe 3 signs seen in appendicitis. Describe

Answer 331

Rovsing's sign - Pain is greater in RIF when LIF is pressed Psoas sign - Pain on extending the hip (suggests retrocaecal appendix) Cope's sign - Pain on flexion and internal rotation of right hip (indicates pelvic appendicitis)

Question 332

Coeliac disease is mediated by which cell type?

Answer 332

T cells

Question 333

Describe the pathophysiology of coeliac disease

Answer 333

Commonly manifests in the duodenum > t-cell mediated response to ingested gluten > villous atrophy and malabsorption

Question 334

Give 3 risk factors for coeliac disease

Answer 334

HLA-DQ2 and HLA-DQ8 Autoimmune disorders (T1DM, Autoimmune hepatitis, Hashimotos thyroiditis, IgA deficiency) Herpetiformis (vesicular, pruritic skin eruption)

Question 335

What are 2 classifications of coeliac disease?

Answer 335

Non responsive coeliac disease Refractory coeliac disease

Question 336

Describe non responsive coeliac disease

Answer 336

Persistent symptoms and enteropathy that do not respond after 6-12 months of a gluten free diet

Question 337

Describe refractor coeliac disease

Answer 337

Persistent or recurrent symptoms and villous atrophy on duodenal biopsy, despite adherence to gluten free diet for at least 12 months

Question 338

Give 4 clinical features of coeliac disease

Answer 338

Diarrhoea Steatorrhoea + foul smelling stools Abdominal pain + distension Weight loss/Failure to thrive

Question 339

What test is used to confirm diagnosis of coeliac disease? what will it show? (3)

Answer 339

Small bowel endoscopy and histology Villous atrophy Crypt Hyperplasia Increased Intraepithelial Lymphocytes

Question 340

Describe coeliac serology (2)

Answer 340

1st line - IgA tissue transglutaminase (IgA-tTG) raised 2nd line - IgA Anti Endomysial antibody (IgA-EMA) raised

Question 341

In coeliac serology, if both IgA-tTG and IgA-EMA are negative, what test should be performed?

Answer 341

Total IgA (Investigate IgA deficiency)

Question 342

If IgA deficiency test is positive (coeliac serology) what test should be performed?

Answer 342

IgG DGP (Deamidated gliadin peptide)

Question 343

What is the 1st line management of coeliac disease?

Answer 343

Long term adherence to gluten free diet Avoid; Wheat, Barley and Rye.

Question 344

What 4 functional areas are assessed in a Developmental Examination

Answer 344

Gross Motor Vision and Fine Motor Hearing, Speech and Language Social, Emotional and Behavioural

Question 345

At what age should a child be able to crawl?

Answer 345

8-9 months

Question 346

At what age should a child be able to stand independently?

Answer 346

10 months

Question 347

At what age should a child be able to walk (unsteadily)? (give age limit)

Answer 347

12 months Age limit:18 months

Question 348

At what age should a child be able to run and jump?

Answer 348

2.5 years

Question 349

At what age should a child show a palmar grasp of a pencil?

Answer 349

4-6 months

Question 350

At what age should a child be able to build a tower of 3, 6, 8 bricks?

Answer 350

3 - 18 months 6 - 2 years 8 - 2.5 years

Question 351

At what age should a child be able to build a bridge with blocks?

Answer 351

3 years

Question 352

At what age should a child be able to build a bridge with blocks?

Answer 352

3 years

Question 353

At what age should a child be able to draw a line, circle, cross, square and triangle?

Answer 353

Line - 2 years Circle - 3 years Cross - 3.5 years Square - 4 years Triangle - 5 years

Question 354

At what age should a child have a polysyllabic babble? (babababa,lalalalala)

Answer 354

7 months

Question 355

At what age should a child be using sounds discriminantly to parents (dada/mama)

Answer 355

10 months

Question 356

What age should a child be using 2/3 words other than mama/dada?

Answer 356

12 months

Question 357

At what age should a child be using 6-19 words and be able to show you two body parts?

Answer 357

18 months

Question 358

At what age should a child be able to play 'peek a boo' or wave bye bye

Answer 358

10-12 months

Question 359

At what age should a child be able to hold a spoon and safely get food to their mouth?

Answer 359

18 months

Question 360

At what age should a child display symbolic play? (e.g turning a banana into a phone)

Answer 360

18-24 months

Question 361

At what age should a child be toilet trained (dry by day)?

Answer 361

2 years

Question 362

What age should a child be able to hop on one leg?

Answer 362

3-4 years

Question 363

At what age should a child display features of parallel play?

Answer 363

2.5-3 years

Question 364

What is the most common cause of chronic diarrhoea in children?

Answer 364

Cows milk intolerance

Question 365

Give 4 classifications of diarrhoea

Answer 365

Secretory (cholera, c.difficile, E.coli) Osmotic Motility disorders (increased - thyrotoxicosis, decreased - Intussusception/obstruction) Inflammatory (bloody diarrhoea - Salmonella, campylobacter, Crohn's, Coeliac)

Question 366

Define malnutrition

Answer 366

Malnutrition describes an imbalance in macronutrients (carbohydrates, fats and protein) and/or micronutrients (vitamins and minerals)

Question 367

How is malnutrition defined by NICE? (3)

Answer 367

BMI <18.5 OR Unintentional weight loss >10% in last 3-6 months OR BMI <20 AND unintentional weight loss >5% in last 3-6 months

Question 368

Name 2 forms of malnutrition

Answer 368

Kwashiorkor Marasmus

Question 369

How is Kwashiorkor malnutrition defined?

Answer 369

Chraracterised by decreased intake of protein and essential aminoacids

Question 370

Give 3 risk factors for Kwashiorkor malnutrition

Answer 370

Premature cessation of breast feeding Chronic GI infection Protein-deficient diet

Question 371

Give 6 clinical features of Kwashiorkor malnutrition

Answer 371

Muscle atrophy/poor growth Diarrhoea Bilateral pitting oedema Distended bowel Hepatosplenomegaly Skin/hair depigmentation

Question 372

Give 3 investigation results seen in Kwashiorkor malnutrition

Answer 372

Decreased albumin Decreased glucose Decreased K, Mg and Hb

Question 373

How is Kwashiorkor malnutrition managed?

Answer 373

Gradually increasing, high protein diet.

Question 374

Give 1 possible complication of malnutrition management

Answer 374

Refeeding syndrome

Question 375

Describe the pathophysiology of refeeding syndrome

Answer 375

Rapid increase in food intake > glycaemia > massive insulin release > increased displacement of Mg, K and PO3 from extracellular to intracellular > reduced serum levels

Question 376

Give 5 clinical features of refeeding syndrome

Answer 376

Oedema Tachycardia (torsades de points – ventricular tachycardia) Seizures Ataxia Rhabdomyolysis

Question 377

How is refeeding syndrome managed?

Answer 377

Electrolyte and vitamin supplementation continued for at least 10 days

Question 378

Define Marasmus malnutrition

Answer 378

Describes lack of calories and a discrepancy between height and weight

Question 379

Give 4 clinical features of Marasmus

Answer 379

Profound muscle wasting (atrophy) Loss of subcutaneous fat Thin dry skin No oedema

Question 380

What disease is associated with marasmus malnutrition?

Answer 380

HIV

Question 381

What 4 defects are present in tetralogy of Fallot?

Answer 381

Pulmonary stenosis (right ventricular outflow tract obstruction) Right ventricular hypertrophy Ventricular Septal Defect Overriding aorta (aorta is displaced over the VSD(r

Question 382

Give 4 risk factors for tetralogy of fallot

Answer 382

DiGeorge syndrome (deletion in chromosome 22) Down's syndrome (trisomy 21) Maternal alcohol consumption during pregnancy Maternal diabetes

Question 383

Give 4 clinical features of tetralogy of fallot

Answer 383

Cyanosis (due to right > left shunt via VSD) Intermittent hypercyaniotic, hypoxic episodes (Tet spells - after crying) Squatting Harsh ejection systolic murmur (At left upper sternal border)

Question 384

Why do untreated children with tetralogy of fallot tend to squat?

Answer 384

Squatting increases systemic vascular resistance > decreases right to left shunt > Increases blood flow to pulmonary circulation > decreases hypoxemia and relieves symptoms

Question 385

What test is used to confirm tetralogy of fallot?

Answer 385

Echocardiography

Question 386

What are the 1st, 2nd and 3rd line medical therapies for treating acute hypercyanotic episodes in tetralogy of fallot?

Answer 386

1st line - Manoeuvres to increase systemic venous return (arms and knees to chest) 2nd line - Propranolol (beta blocker) 3rd line - Phenylephrine (alpha receptor agonist)

Question 387

Define cyanotic congenital heart disease

Answer 387

Describes any heard defect that reduces the amount of oxygen delivered to the body. Typically characterised by a right ot left shunt

Question 388

Give 5 examples of cyanotic congenital heart diseases

Answer 388

Tetralogy of Fallot Transposition of great vessels Tricuspic valve atresia Ebstein anomaly Persistent truncus arteriosus (DiGeorge syndrome)

Question 389

Define acyanotic congenital heart disease

Answer 389

Describes congenital cardiac malformations that affect the atrial or ventricular walls, heart valves or large blood vessels. Characterised by a left to right shunt

Question 390

Give 2 clinical features caused by a left to right shunt

Answer 390

Pulmonary hypertension Right ventricular hypertrophy

Question 391

Give 5 examples of acyanotic congenital heart defects

Answer 391

Ventricular Septal Defect (VSD) Atrial Septal Defect (ASD) Patent foramen ovale (PFO) Patent ductus arteriosus (PDA) Coarctation of the aorta

Question 392

Define transposition of the great vessels

Answer 392

Anatomical reversal of the aorta and pulmonary artery

Question 393

Give 2 risk factors for Transposition of the Great Arteries

Answer 393

Maternal diabetes (most common) Di George Syndrome

Question 394

What can make transposition of the great vessles less fatal?

Answer 394

Mixing of blood occurring via either; Intracardiac shunt (PFO, VSD, ASD) or Extracardiac connection (PDA)

Question 395

Give 3 clinical features of Transposition of the Great Arteries

Answer 395

Postnatal cyanosis Tachypnoea 'Egg-on side' appearance on chest x-ray

Question 396

What sign is seen on an x-ray in a patient with Transposition of the Great Arteries

Answer 396

Egg-on-side appearance (Enlarged, globular heart resembles an egg lying on its side)

Question 397

How might you distinguish between Transposition of Great Arteries and Tetralogy of Fallot?

Answer 397

TOGA - Early onset (first few days) ToF - Later onset (1-2 months)

Question 398

What medication may be used in the initial management of Transposition of the Great Arteries? And why?

Answer 398

Alprostadil (prostaglandin E1) Used to prevent closure of the Ductus Arteriosus

Question 399

Define tricuspid valve atresia

Answer 399

Describes absent or rudimentary tricuspid valve resulting in no blood flow between the Right atrium and ventricle.

Question 400

What 2 echocardiogram findings are present in Tricuspid Atresia?

Answer 400

Right Ventricular hypoplasia Right Atrial dilation (due to volume overload)

Question 401

Give 3 clinical features of Tricuspid Atresia

Answer 401

Central cyanosis (within days of birth( Tachypnea Holosystolic murmur (at lower left sternal border)

Question 402

What are more common VSDs or ASDs?

Answer 402

VSDs

Question 403

Define Ebstein anomaly

Answer 403

Describes a congenital heart defect characterised by low insertion of the tricuspid valve, resulting in a large atrium and small ventricle

Question 404

What is the main risk factor for Ebstein's anomaly?

Answer 404

In-utero Lithium exposure

Question 405

Give 2 other cardiac defects associted with Ebstein Anomaly

Answer 405

Interatrial communication (ASD) (90%) Conduction disorders (WPW, supraventricular tachycardia)

Question 406

Give 3 clinical features of Ebstein Anomaly

Answer 406

Cyanosis Right bundle branch block Tricuspid regurgitation (Pansystolic murmur -worse on inspiration)

Question 407

Define Persistent Truncus Arteriosus

Answer 407

Describes underdevelopment of the aorticopulmonary septum. Truncus arteriosus doesn't divide resulting in a single trunk that receives output from both ventricles.

Question 408

What is the truncus arteriosus?

Answer 408

Part of the developing heart that divides into the pulmonary trunk and aorta

Question 409

What syndrome is associated with Persistent Truncus Arteriosus?

Answer 409

DiGeorge Syndrome

Question 410

What determines the severity of Persistent Truncus arteriosus?

Answer 410

Pulmonary Vascular Resistance (PVR)

Question 411

Define pulmonary vascular resistance (PVR)

Answer 411

The resistance that must be overcome in order to move blood through the pulmonary vasculature

Question 412

How is Pulmonary Vascular Resistance Calculated?

Answer 412

PVR = (mPAP-PAWP)/Cardiac output mPAP - Mean pulmonary artery pressure PAWP - Pulmonary Artery Wedge Pressure

Question 413

What units are used to express PVR? And what is considered abnormal?

Answer 413

Woods units >2 is considered abnormal

Question 414

DiGeorge syndrome occurs due to a deletion in which chromosome?

Answer 414

Chromosome 22

Question 415

Define DiGeorge Syndrome

Answer 415

Syndrome characerised by defective development of the 3rd and 4th pharyngreal pouches, leading to hypoplastic thymus and parathyroids

Question 416

Give 5 clinical features of DiGeorge Syndrome

Answer 416

Cardiac anomalies (tetralogy of fallot, persistent truncus arteriosus, VSD, ASD, systolic murmur) Facial defects (prominent nasal bridge, prominent ears, cleft lip and palate, micrognathia) Thymus aplasia/hypoplasia (Recurrent infections due to T cell deficiency) Recurrent infections (parainfluenza, thrush) Hypoparathyroidism (hypocalcemia)

Question 417

Give 3 features of hypocalcemia

Answer 417

Tetany; Cramps Spasms Hyporeflexia

Question 418

Give 3 investigation results seen in DiGeorge Syndrome

Answer 418

Decreased Parathyroid Hormone Decreased Calcium Decreased T-lymphocyte count

Question 419

What 2 signs are positive in hypocalcemia?

Answer 419

Trousseau sign (spasm of hand) Chvostek sign (contraction of ispilateral facial muscles after percussion over facial nerve)

Question 420

What x-ray feature may be present in a patient with DiGeorge Syndrome?

Answer 420

No thymic shadow

Question 421

What is the most common congenital heart defect?

Answer 421

Ventricular Septal Defect

Question 422

Give 3 risk factors for VSD

Answer 422

Downs, Pataus and Edwards Syndrome Intrauterine infections (TORCH) Maternal diabetes, obesity and smoking

Question 423

Define TORCH (maternal intrauterine infections)

Answer 423

Toxoplasmosis Other agents (Varecilla zoster, Parvovirus B19) Rubella Cytomegalovirus Herpes Simplex/HIV

Question 424

Give 6 clinical features of VSD (post-natal)

Answer 424

Failure to thrive Features of heart failure; Hepatomegaly Tachypnoea Tachycardia Pallor Pan-systolic murmur

Question 425

Give 6 clinical features of VSD

Answer 425

Failure to thrive Features of heart failure; Hepatomegaly Tachypnea Tachycardia Pallor Pan-systolic murmur

Question 426

What shunt is seen in VSD?

Answer 426

Left to right shunt

Question 427

Give 5 complications of VSD

Answer 427

Aortic regurgitation Infective endocarditis Eisenmengers complex Right sided heart failure Pulmonary hypertension

Question 428

What is Eisenmenger's syndrome?

Answer 428

Occurs due to prolonged pulmonary hypertension from left > right shunt Results in right ventricular hypertrophy and increased right ventricular pressure. Eventually leads to reversal of blood flow (right>left shunt)

Question 429

What is the most common cause of infective endocarditis?

Answer 429

Staphylococcus aureus

Question 430

Give 5 clinical features of infective endocarditis

Answer 430

Janeway lesions Oslers nodes Splinter haemorrhages Fever, nightsweats, weightloss Cardiac murmur

Question 431

What valve is most commonly affected by infective endocarditis?

Answer 431

Mitral valve

Question 432

Give 3 risk factors for ASD

Answer 432

Down Syndrome Fetal alcohol syndrome Holt-Oram syndrome (skeletal abnormalities in hands and arms)

Question 433

Name 2 types of ASD

Answer 433

Ostium Secundum (70%) (Holt-Oram syndrome) Ostium Primum (abnormal AV valves)

Question 434

Give 3 clinical features of ASD

Answer 434

Asymptomatic (small defect) Ejection systolic murmur Widely split second heart sound (S2)

Question 435

What causes the 2nd heart sound (S2)?

Answer 435

Closure of pulmonary and aortic valves

Question 436

What causes the 1st heart sound (S1)?

Answer 436

Closure of mitral and tricuspid valves

Question 437

Give 2 complications of ASD

Answer 437

Paradoxical embolism (increases risk of ischemic stroke) Heart failure

Question 438

The Ductus Arteriosus is a connection between which vessels?

Answer 438

Pulmonary trunk and descending aorta

Question 439

When and why does the ductus ateriosus close?

Answer 439

Closes with first breath due to increased pulmonary blood flow which enhances prostaglandin clearance

Question 440

Give 6 risk factors for PDA

Answer 440

Prematurity Maternal Rubella (during 1st trimester) Maternal Alcohol consumption Maternal phenytoin use Maternal prostaglandin use Downs syndrome

Question 441

What shunt is seen in PDA?

Answer 441

Left to right shunt (acyanotic)

Question 442

Give 5 clinical examination findings of PDA

Answer 442

Left subclavicular thrill Continuous machinery murmur (left sternal border) Large volume, bounding collapsing pulse Wide pulse pressure Heaving apex beat

Question 443

How is PDA managed in neonates?

Answer 443

Indomethacin or Ibuprofen (inhibit prostaglandin synthesis)

Question 444

What causes the Ductus Arteriosus to remain patent?

Answer 444

Prostaglandin E2 production by the placenta

Question 445

What is the MOA of indomethacin?

Answer 445

COX1/2 inhibitor

Question 446

Give 1 contraindication for indomethacin use

Answer 446

GI disease (haemorrhage, ulceration, perforation ect)

Question 447

What diuretic should be used in management of PDA? and why?

Answer 447

Chlorothiazide As furosemide simulates renal synthesis of PGE2 and is associated with nephro and ototoxicity.

Question 448

What should be given in PDA if there is a contraindication to ibuprofen?

Answer 448

Paracetamol

Question 449

Where is the foramen ovale?

Answer 449

Hole between left and right atria

Question 450

What condition is associated with PAtent Foramen Ovale?

Answer 450

Loeys-Dietz syndrome (connective tissue disorder)

Question 451

Define coarctation of the aorta

Answer 451

Congenital narrowing of the descending aorta

Question 452

Give 4 conditions associated with coarctation of the aorta

Answer 452

Turners syndrome Bicuspid aortic valve Berry aneurysms Neurofibromatosis

Question 453

Give 4 clinical features of coarctation of the aorta

Answer 453

Shock (neonates) Hypertension at young age (resistant to treatment) Delayed brachia -femoral pulse Ejection sytolic murmur

Question 454

Give 3 risk factors for coarctation of the aorta

Answer 454

Male Turners syndrome DiGeorge syndrome

Question 455

How is coarctation of the aorta managed in neonates? (1)

Answer 455

Alprostadil (Prostaglandin E1 - maintain patency of ductus arteriosus in neonates)

Question 456

What condition is associated with dextrocardia?

Answer 456

Kartagner's syndrome

Question 457

What is Kartagener's syndrome? What is it caused by?

Answer 457

Primary ciliary dyskinesia (absent or dysmotile cilia) Defect in dynein arm of microtubules

Question 458

Give 4 clinical features of Kartagner syndrome

Answer 458

Recurrent otitis media, sinusitis and nasal polyps Bronchiectasis Dextrocardia/situ iversus Subfertility (deminished sperm motility and defective ciliary action in fallopian tubes)

Question 459

Define febrile convulsion

Answer 459

Seizure provoked by fever in otherwise normal children

Question 460

Between what ages do febrile convulsions tend to occur?

Answer 460

6 months and 5 years old

Question 461

Give 3 clinical features of febrile convusions

Answer 461

Usually occur early in a viral infection (as temperature rises) i.e otitis media Brief seizures (<5 mins) Tonic clonic seizures

Question 462

Define simple febrile convulsion (4)

Answer 462

<15 minutes Generalised seizure No recurrence in 24 hours Should recover in an hour

Question 463

Recurrent febrile convulsions be managed? (2)

Answer 463

Rectal diazepam or buccal midazolam

Question 464

Define complex febrile seizure

Answer 464

15-30 minutes Focal seizure May have repeat seizures within 24 hours

Question 465

Define biliary atresia. When does it present?

Answer 465

Describes complete obliteration or discontinuity of the extrahepatic biliary system (commonly bowel duct), resulting in obstruction to flow of bile (cholestasis) Typically presents in first weeks of life

Question 466

Name and define 3 types of biliary atresia. Which is most common?

Answer 466

Type 1 - Proximal ducts are patent, but common duct is obliterated Type 2 - Atresia of the cystic duct and cystic duct structures, found in the porta hepatis Type 3 - Atresia of the left and right ducts to the level of the porta hepatis (most common)

Question 467

What 3 structures are found in the porta hepatis?

Answer 467

Hepatic portal vein Hepatic artery proper Common bile duct

Question 468

Describe the pathophysiology of biliary atresia

Answer 468

Obliteration of bile system > Obstruction of bile flow (cholestasis) > Secondary biliary cirrhosis and portal hypertension

Question 469

Give 5 clinical features of biliary atresia

Answer 469

Presents in first few weeks of life Prolonged neonatal jaundice (extending beyond physiological 2 weeks) Dark urine and pale stools Appetite and growth disturbance Hepato-splenomegaly

Question 470

How is biliary atresisa diagnosed?

Answer 470

Cholangiography (fails to show biliary tree)

Question 471

What 3 laboratory findings may be seen in biliary atresia?

Answer 471

Elevated Conjugated bilirubin Elevated AST/ALP Elevated GGT

Question 472

What ultrasound findings may be seen in biliary atresia? (2)

Answer 472

Absence of gall bladder No dilatation of biliary tree

Question 473

Define volvulus

Answer 473

Describes twisting of a loop of bowel on it's mesentery (common cause of intestinal obstruction) Most commonly presents as midgut volvulus

Question 474

What is the mesentery?

Answer 474

A double layered fold of peritoneum that anchors the colon to the posterior abdominal wall

Question 475

Describe normal intestinal rotation

Answer 475

Midgut starts to elongate in utero (4th week) > Midgut herniates out of the umbilicus (6th week) > 90 degree counter-clockwise rotation of the midgut > re-entry of the midgut into the abdominal cavity (10th week) > 180 degree rotation inside the abdominal cavity > fixation of the duodenojejunal flexure and cecum to the posterior abdominal wall.

Question 476

In total, how many degrees rotation occurs in normal intestinal rotation?

Answer 476

270 degrees rotation

Question 477

What are the 2 types of intestinal malrotation?

Answer 477

Non rotation - The entire colon is left sided and the entire small bowel is right sided Incomplete rotation - Cecum remains fixed in the right upper quadrant by peritoneal bands (Ladd bands)

Question 478

Give 3 clinical signs of intestinal malrotation (bowel obstruction) in neontates

Answer 478

Bilious vomiting (thick, green vomit) Abdominal distension Absent bowel sounds

Question 479

Give 4 conditions associated with intestinal malrotation

Answer 479

Exomphalos (organs herniate through umbilicus into hernial sac) Congenital diaphragmatic hernia Intrinsic duodenal atresia Congenital heart defects

Question 480

What diagnostic tests are used to diagnose intestinal malrotation? Which is gold standard?

Answer 480

Upper GI contrast study (gold standard) Ultrasound study

Question 481

Describe the initial resuscitation management of intestinal malrotation (4)

Answer 481

NG tube IV fluids Correction of electrolyte imbalance Broad Spectrum Antibiotics

Question 482

How is intestinal malrotation managed?

Answer 482

Laparotomy (if volvulus present) Ladd's procedure (division of Ladd bands)

Question 483

Define intussusception

Answer 483

Describes when the proximal part of the bowel invaginates into the distal part, resulting in mechanical obstruction and bowel ischemia

Question 484

Where in the bowel does intussusception commonly occur?

Answer 484

Ileo-caecal region

Question 485

Give 5 clinical features of intussusception

Answer 485

Acute cyclical colicky abdominal pain (sudden onset screaming or crying spells) Sausage shaped mass in right upper quadrant Red currant jelly stool Legs drawn up + Abdominal tenderness Vomiting (initially nonbilious)

Question 486

What investigation is used to confirm intussusception?

Answer 486

Abdominal Ultrasound

Question 487

If an infant with intussusception presents with features of bowel obstruction/perforation, what investigtion should be performed?

Answer 487

Abdominal X-ray

Question 488

What would an ultrasound show in a patient with intussusception? (2)

Answer 488

Target like mass Pseudokidney sign (longitudinal view shows distal loop of bowel resembling kidney)

Question 489

How is intussusception managed? (3)

Answer 489

Hypovolemia - IV fluid resuscitation (saline) Bowel obstruction - Abdominal X-ray, NG tube (drainage), IV antibiotics (Gentamycin, Amoxicillin and Metronidazole) Curative - Air enema (Reduction with air insufflation)

Question 490

Describe features of small bowel obstruction (4)

Answer 490

Abdominal pain – Colicky Vomiting and/or nausea – Early onset, Large volume of bilious vomit Constipation – late onset Abdominal distension – Less severe than LBO

Question 491

Describe features of large bowel obstruction (4)

Answer 491

Abdominal pain – colicky or constant Vomiting and/or nausea – Late onset, initially bilious but progresses to fecal vomiting Constipation – Early onset Abdominal distention – Early and significant abdominal distension

Question 492

Define pyloric stenosis. When does it typically present?

Answer 492

Typically presents in 1st 4 weeks of life Describes idiopathic hypertophy and hyperplasia of pyloric sphincter muscles, making it difficult to pass food through

Question 493

Give 3 risk factors for pyloric stenosis

Answer 493

Exposure to nicotine during pregnancy Macrolide antibiotics - Erythromycin/Azithromycin (esp during 1st 2 weeks of life) Bottle feeding

Question 494

Give 3 clinical features of pyloric stenosis

Answer 494

Projectile vomiting occurring after feeding (non-bilious - as obstruction is proximal to ampulla of Vater) Palpable olive shaped structure in epigastrium (sign of pyloric hypertrophy) Visible gastric peristalsis proximal to site of obstruction

Question 495

How is pyloric stenosis diagnosed?

Answer 495

Clinical - if palpable signs are present If not - Ultrasound confirms diagnosis

Question 496

How is pyloric stenosis managed?

Answer 496

Ramstedt pyloromyotomy (muscle splitting incision of pyloric sphincter)

Question 497

Define oesophageal atresia

Answer 497

Describes abnormal development of tracheoesophageal septum. Congenital defect in which upper oesophagus does not connect to lower oesophagus. Manifests immediately after birth

Question 498

What is the most common type of oesophageal atresia?

Answer 498

Gross type C - Oesophageal atresia with fistula connected distally to trachea

Question 499

Give 3 clinical features of oesophageal atresia

Answer 499

Manifests immediately after birth Prenatal - polyhydraminos - as fetus is unable to swallow amniotic fluid Post-natal - Excessive secretions/foaming from the mouth. Vomitng, choking, drooling, inability to feed

Question 500

Give 1 complication of oesophageal atresia. How may it present? (3)

Answer 500

Aspiration pneumonia 2nd to tracheoesophageal fistula. Presents with; Coughing Rales (discontinuous rattling on auscultation) Cyanotic attacks

Question 501

How is oesophageal atresia diagnosed?

Answer 501

NG tube placement - Tube cannot pass through oesophagus Abdominal/Thorax x-ray - Shows oesophageal pouch

Question 502

What should also be investigated in oesophageal atresia? How does it occur?

Answer 502

VACTERL anomalies Vertebral Anal Cardiac Tracheoesophageal fistula Renal Limb Occurs due to defect in embryonic mesoderm

Question 503

How is oesophageal atresia managed? (2)

Answer 503

Surgery within 1st 24 hours to reconnect upper and lower oesophagus Gastrostomy tube - to allow enteral feeding

Question 504

What is the most common nephrotic syndrome in children?

Answer 504

Minimal change disease

Question 505

How is nephrotic syndrome defined? How is it typically characterised?

Answer 505

Defined as proteinuria due to podocyte pathology Characterised by triad of; proteinuria, hypoalbuminaemia and oedema Not associated with haematuria but is associated with blood clotting due to protein loss

Question 506

How is nephritic syndrome described and what is it characterised by? (4)

Answer 506

Defined by haematuria due to inflammatory damage Characterised by; Haematuria + red cast cells Proteinuria leading to oedema Hypertension Oliguria and progressive renal impairment

Question 507

What triad is classically seen in nephrotic syndrome?

Answer 507

Proteinuria Hypoalbuminaemia Oedema

Question 508

Name 3 types of nephrotic syndrome. Which is most common? (

Answer 508

Minimal change disease (most common) Focal segmental glomerulosclerosis Membranous nephropathy

Question 509

Minimal change disease is typically idiopathic, but can occur secondary to what? (4)

Answer 509

Hodgkin's lymphoma Leukaemia Drugs - NSAIDs/Lithium Hepatitis B/C

Question 510

What diagnostic test is used to diagnose minimal chage disease? What will it show?

Answer 510

Renal biopsy + Electron microscopy Shows effacement (shortening/thinning) of podocyte foot processes

Question 511

Describe the management of minimal change disease

Answer 511

Initial treatment - Prednisolone Infrequent relapses; - Increase dose of prednisolone or - Calcineurin inhibitor (Ciclosporin or Tacrolimius) Frequent relapses - DMARD - Mycophenolate mofetil

Question 512

How does Nephritic syndrome typically present? (4)

Answer 512

Haematuria + red cast cells Proteinuria leading to oedema Hypertension Oliguria and progressive renal impairment

Question 513

Name 4 types of nephritic syndrome. Which is most common?

Answer 513

IgA nephropathy (most common) Good pastures (Anti-GBM) Post streptococcal (cresenteric) SLE

Question 514

How does IgA nephropathy typically present?

Answer 514

Presents with macroscopic haematuria following a respiratory infection

Question 515

How is IgA nephropathy distinguished from post-streptococcal glomerulonephritis? (2)

Answer 515

Post streptococcal has low complement levels and proteinuria (IgA does not) IgA nephropathy develops 1-2 days after Upper RTI, post streptococcal occurs 1-2 weeks after.

Question 516

Define goodpastures syndrome

Answer 516

Now called Anti-glomerular basement membrane disease (GBM) Describes a rare type of small vessel vasculitis associated with both pulmonary haemorrhage and progressive glomerulonephritis

Question 517

What causes goodpastures syndrome?

Answer 517

Caused by anti-glomerular basement membrane antibodies against Type IV collagen (type found in basement membranes of alveoli and glomeruli)

Question 518

How is goodpastures diagnosed? (2)

Answer 518

Renal biopsy - Showing IgG deposits along basement membrane Antiglomerular basement membrane antibody (Anti-GMB confirms diagnosis)

Question 519

How is goodpastures syndrome managed? (3)

Answer 519

Prednisolone Plasmapheresis Cyclophosphamide

Question 520

How may goodpastures syndrome present? (3)

Answer 520

Haemoptysis (due to pulmonary haemorrhage) Reduced urine output (AKI) Oedema

Question 521

What bacterium causes Whooping cough?

Answer 521

Bordetella pertussis (gram negative)

Question 522

When are infants given vaccinations for whooping cough?

Answer 522

2 months, 3 months, 4 months and 3.5 years

Question 523

How long is the incubation period for whooping cough?

Answer 523

5-10 days (maximum 21)

Question 524

What criteria would make you suspect whooping cough?

Answer 524

Suspect in person with acute cough lasting >14 days + 1 or more of the following; Paroxysmal cough (recurring intense episodes of cough) Inspiratory whoop Post-tussive (after coughing) vomiting Undiagnosed apnea

Question 525

Give 4 clinical features of whooping cough

Answer 525

Coughing bouts (worse at night) Post cough vomiting Inspiratory whoop Apnea

Question 526

How is whooping cough diagnosed? (2)

Answer 526

Nasal sawb for Bordetella pertussis PCR and serology (increasingly used now due to availability)

Question 527

How is whooping cough managed? (3)

Answer 527

Oral macrolide - Clarithromycin, azithromycin, erythromycin Household contacts offered antibiotic prophylaxis Notify pyblic health england

Question 528

How long should a child with whooping cough be excluded from school? (2)

Answer 528

48 hours after commencing antibiotics Or 21 days after onset of symptoms (if no antibiotics)

Question 529

What is the number 1 cause of painless massive GI bleeding in children aged 1-2?

Answer 529

Meckel's diverticulum

Question 530

Define Meckel's diverticulum

Answer 530

Describes a true diverticulum (involving all layers of the gut wall) at the terminal ileum

Question 531

Meckel's diverticulum occurs due to a reminant of what?

Answer 531

Vitelline duct (omphalomesenteric duct)

Question 532

What mucosa can be present in Meckel's diverticulum?

Answer 532

Ectopic ileal, gastric or pancreatic mucosa

Question 533

What are the rule of 2s for Meckel's diverticulum? (3)

Answer 533

Occurs in 2% of the population Is 2 feet from the ileocaecal valve Is 2 inches long

Question 534

How does Meckel's diverticulum typically present? (3)

Answer 534

Right Lower Quadrant Pain (mimicing appendicitis) Rectal bleeding Intestinal obstruction 2nd to omphalomesenteric band, volvulus and intussusception)

Question 535

Define androgen insensitivty syndrome

Answer 535

Describes an X-linked recessive condution due to end organ resistance to testosterone causing genotypically male children (46XY) to have a female phenotype

Question 536

What is the pattern of inheritance in androgen insensitivity syndrome?

Answer 536

X-linked recessive (46XY)

Question 537

Give 3 clinical features of androgen insensitivity syndrome

Answer 537

Primary amenorrhoea Undescended testes causing groin swellings Delayed puberty (no pubic hair, small breasts, slim build)

Question 538

How is androgen insensitivity syndrome diagnosed? (2)

Answer 538

Buccal smear or chromosomal analysis (46XY genotype)

Question 539

Define Kallman's syndrome

Answer 539

Recognised cause of delayed puberty secondary to hypogonadotrophic hypogonadism. Occurs due to failure of GnRH secreting neurones to migrate to the hypothalamus

Question 540

Give 4 clinical features of Kallman's syndrome

Answer 540

Delayed puberty (male) Ansomia (lack of smell) Hypogonadism, cryptorchidism Cleft lip/palate and hearing/visual defects (rare)

Question 541

What is the pattern of inheritance for Kallman Syndrome?

Answer 541

X-linked recessive

Question 542

Describe the expected LH and Testosterone levels in Primary hypogonadism, (klinefelters), Hypogonadotrophic hypogonadism (Kallman's) and Androgen insensitivity syndrome

Answer 542

Klinefelters - High LH, Low Testosterone Kallman's - Low LH, Low Testosterone Androgen insensitivity - High LH, Normal/High testosterone

Question 543

What karyotype is Klinefelter's syndrome associated with?

Answer 543

47, XXY

Question 544

Give 5 clinical features of Klinegelter's syndrome (primary hypogonadism)

Answer 544

Taller than average Lack secondary sexual characteristics Small, firm testes Gynecomastia (increased incidence of breast cancer) Infertility

Question 545

What immunisation is offered to boys and girls at 12-13 years old (year 8)?

Answer 545

HPV (Human papilloma virus)

Question 546

What is the most common cardiac pathology associated with Duchenne muscular dystrophy?

Answer 546

Dilated cardiomyopathy

Question 547

Give 5 clinical features of otitis media

Answer 547

Acute onset of; Fever Irritability Vomiting Anorexia Previous viral upper respiratory infection

Question 548

When are antibiotics indicated in a child with otitis media? (4)

Answer 548

If; Tympanic membrane is perforated Child is <3 months Child is <2 years and infection is bilateral If symptoms present for 4 or more days

Question 549

How is otitis media managed? (1st, 2nd, 3rd and 4th line)

Answer 549

1st line - Oral analgesics (NSAIDs/Paracetamol) 2nd line - Amoxicillin (Offered if symptoms don't improve after 4 days) 3rd line - Clarithromycin/erythromycin (if penicillin allergy) 4th line - Co-amoxiclav (if symptoms don't subside after 2-3 days on previous AB)

Question 550

Give 3 intracranial complications of otitis media

Answer 550

Meningitis (headache, vomiting, photophobia, phonophobia) Sigmoid sinus thrombosis (sepsis, swinging pyrexia, meningitis) Brain abscess (sepsis and neuro signs - CN compression)

Question 551

Give 4 extra-cranial complications of otitis media

Answer 551

Facial nerve palsy (LMN lesion in CN VII) Mastoiditis Petrositis (Gradenigo syndrome- otorrhoea, pain inside ear and eye, ispilateral VI nerve palsy) Labyrinthitis (vertigo, nausea, voming and imbalance)

Question 552

What is Gradenigo syndrome? What it is a feature of? How does it present?

Answer 552

Feature of petrositis (infection of otitis media spreading to pertrous temporal bone) Presents with; otorrhoea, pain deep inside the ear and eye and ispilateral N VI palsy

Question 553

How may labyrinthitis present in otitis media? (4)

Answer 553

Vertigo Nausea Vomiting Imbalance

Question 554

What is the 1st line treatment for neonatal sepsis?

Answer 554

Benzylpenicillin + Gentamycin

Question 555

What vaccination should pregnant women be offered between 16-32 weeks?

Answer 555

Pertussis (whooping cough) + Influenza

Question 556

What virus commonly causes Hand, Foot and Mouth Disease?

Answer 556

Coxsackie A16

Question 557

What is the most common cause of vomiting in infancy?

Answer 557

GORD (Gastro-oesophageal Reflux Disease)

Question 558

Give 2 clinical features of GORD in children

Answer 558

Typically develops before 8 weeks Vomiting/regurgitation following feeds

Question 559

How is GORD managed in children?

Answer 559

Trials of; Smaller more frequent feeds Thickened formula Alginate therapy (should not bee used at the same time as thickening) PPI (omeprazole - 4 week trial)

Question 560

When should use of a PPI be considered in infants with GORD? (3)

Answer 560

If presenting with 1 or more of; Unexplained feeding difficties (refusing feeds, gagging or choking) Distressed behaviour Faltering growth

Question 561

What is the commonest cause of respiratory distress in a newborn?

Answer 561

Trnsient Tachypnoea of the newborn

Question 562

What is a common risk factor for TTN?

Answer 562

Caesarean sections (occurs due to delayed resorption of fluid in the lungs)

Question 563

How it TTN diagnosed? What may the investigation show?

Answer 563

Chest X-ray May show; Hyperinflation of the lungs Fluid filled horizontal fissure

Question 564

How is TTN managed?

Answer 564

Observation and supportive care (symptoms usually resolve in 1-2 days) Supplementary O2 may be required to maintain sats

Question 565

How is neonatal sepsis categorised?

Answer 565

Early onset sepsis (within first 72 hours of birth) Late onset sepsis (7-28 days of life)

Question 566

What are the 2 most common causes of neonatal sepsis?

Answer 566

Group B streptococcus E.coli

Question 567

What is the most common cause of early onset sepsis?

Answer 567

Group B Streptococcus

Question 568

What is the most common cause of late onset sepsis?

Answer 568

Environmental pathogens; Staphylococcus epidermidis (coagulase negative)

Question 569

Give 3 risk factors for neonatal sepsis

Answer 569

Prematurity ( <37weeks) Low birth weight (<2.5kg) Maternal GBS infection

Question 570

Give 6 clinical features of neonatal sepsis

Answer 570

Respiratory distress (grunting, nasal flaring, tachypnoea) Tachycardia Apnoea Jaundice Seizures Poor-feeding, abdominal distension and/or vomiting Features tend to be non-specific

Question 571

What clinical feature is the most common presentation of neonatal sepsis?

Answer 571

Respiratory distress (grunting ect)

Question 572

What investigations should be ordered in ?neonatal sepsis? (3)

Answer 572

Blood culture (establish diagnosis) FBC (neutrophilia and neutropenia) ABG (metabolic acidosis)

Question 573

How is neontal sepsis managed?

Answer 573

1st line - IV Benzylpenicillin + Gentamicin Remeasure CRP 18-24 hours after presentation

Question 574

When can antibiotics be stopped in neonatal sepsis?

Answer 574

Stop at 48 hours if CRP <10mg/L and negative blood culture at presentation and at 48 hours

Question 575

Give 5 features of cows milk intolerance

Answer 575

Regurgitation and vomiting Diarrhoea Urticaria (rash) and atopic eczema Colic symptoms (irritability/crying) Wheeze, chronic cough

Question 576

How is cows milk intolerance diagnosed? What additional investigations would be performed?

Answer 576

Clinical diagnosis (improvement with cow's milk elimination) Skin prick/patch test Total IgE and specific IgE (RAST) for cows milk protein

Question 577

How is cows milk intolerance managed (if formula fed)?

Answer 577

If failing to thrive - refer to paediatrician 1st line - Extensive hydrolysed formula 2nd line - Amino acid based formula (severe cases/no response to eHF)

Question 578

How is cows milk intolerance managed if breast fed?

Answer 578

Continue breastfeeding Eliminate cows milk from maternal diet

Question 579

What is the prognosis for cows milk intolerance

Answer 579

In children with IgE mediated intolerance - 55% will be milk tolerant by 5 In children with non-IgE mediated intolerance, most children will be milk tolerant by 3.

Question 580

Is cows milk intolerance IgE mediated or non-mediated?

Answer 580

Both

Question 581

Give 3 characteristics of hypospadias

Answer 581

Ventral urethral meatus Hooded prepuce Chordee (ventral curvature of penis)

Question 582

What condition is commonly associated with hypospadias?

Answer 582

Cryptorchidism (undescended testes)

Question 583

What is it important not to do before surgery of hypospadias?

Answer 583

Circumcision (as foreskin may be used in corrective procedure)

Question 584

What is the most appropriate next step for a child presenting with limp/hip pain and a fever?

Answer 584

Refer urgently for same-day assessment

Question 585

What type of vaccine is the MMR jab?

Answer 585

Live attenuated

Question 586

How is biliary atresisa diagnosed?

Answer 586

Cholangiography (fails to show biliary tree)

Question 587

What vaccine should be offered to all asthmatic children on corticosteroids?

Answer 587

Influenza vaccine

Question 588

Define Potter's Syndrome

Answer 588

Describes the typical physical appearance caused by pressure in utero caused by oligohydraminos. Occurs due to bilateral renal agenesis

Question 589

Why does bilateral renal agenesis result in Potter syndrome?

Answer 589

Renal tract complications lead to; Lack of amniotic fluid resulting in foetal compression (causing facial and limb characteristics) Lack of amniotic fluid affects lung development, leading to pulmonary hypoplasia

Question 590

How may necrotising enterocolitis present? (4)

Answer 590

Feeding intolerance Abdominal distension Bloody stools Bilious vomiting

Question 591

How is necrotising enterocolitis diagnosed? What will the test show? (4)

Answer 591

Abdominal x-ray; Shows dilated bowel loops (asymmetrical) Bowel wall oedema Pneumatosis intestinalis (intramural gas) Air both inside and outside of bowel wall (Rigler sign)

Question 592

How is necrotising enterocolits managed? (4)

Answer 592

Laparotomy (initially) Stop oral feeds Barrier nurse Give antibiotics - Cefotaxime and vancomycin

Question 593

What antibiotics are given in the management of necrotising enteroclolitis?

Answer 593

Cefotaxime and vancomycin

Question 594

How are mitochondrial diseases inherited?

Answer 594

Maternal inheritance Non of children in an affected male will inherit disease All of children of an affected female will inherit disease

Question 595

Define Immune Thrombocytopenia (ITP)

Answer 595

Describes an immune mediated reduction in platelet count (thrombocytopenia) Typically follows infection or vaccination

Question 596

Give 3 clinical features of ITP

Answer 596

Bruising Petechial or purpuric rash Epistaxis or gingival bleeding (less common)

Question 597

How is achondroplasia (dwarfism) inherited?

Answer 597

Autosomal dominant

Question 598

If two parents have achondroplasia, what are the chances of their child having the same condition?

Answer 598

1/4 - unaffected 1/2 - affected heterozygous 1/4 - affected homozygous

Question 599

Define Duchene muscular dystrophy

Answer 599

Describes an X-linked recessive inherited disorder in dystrophin genes required for normal muscular function

Question 600

Give 4 clinical features of Duchenne muscular dystrophy

Answer 600

Progressive proximal muscle weakness form 5 years Disproportionally large calves Grower's sign - Child uses arms to stand from a squatted position Intellectual impairment (30%)

Question 601

What investigation is used to diagnosed Duchenne muscular dystrophy?

Answer 601

Genetic testing

Question 602

What biochemical marker is likely to be raised in Duchenne muscular dystrophy?

Answer 602

Creatinine Kinase

Question 603

When can children with scarlet fever return to school?

Answer 603

24 hours after commencing antibiotics

Question 604

When can children with measles return to school?

Answer 604

4 days from onset of rash

Question 605

When can children with rubella return to school?

Answer 605

5 days from onset of rash

Question 606

Give 3 fractures that are commonly seen in non-accidental injury

Answer 606

Radial Humeral Femoral

Question 607

Give 4 causes of neonatsal jaundice (in firt 24 hours)

Answer 607

Rhesus haemolytic disease ABO haemolytic disease Hereditary spherocytosis Glucose-6-dehydrogenase

Question 608

What is the most common cause of childhood hypothyroidism?

Answer 608

Autoimmune thyroiditis

Question 609

How is constipation diagnosed in a child <1/

Answer 609

2 or more of the following are required for diagnosis Stool pattern; - < 3 complete stools per week - type 3/4 on Bristol stool chart - Hard large stool - Rabbit droppings (type 1) Symptoms associated with defication - Distress on passing stool - Bleeding due to hard stool - Straining History - Previous episode of constipation - Previous or current anal fissure or more

Question 610

How is constipation diagnosed in a child >1?

Answer 610

2 or more of the following are required for diagnosis; Stool pattern; - Fewer than 3 complete stools per week (type 3 or type 4) - Overflow soiling (commonly loose, smelly stools passed without sensation) - Rabbit droppings (type 1) - Large infrequent stools that block the toilet Symptoms associated with defication; - Poor appetite that improves with passage of large stools - Evidence of retentive posture (straight legged, tiptoe) -Anal pain/straining History; - Previous episodes of constipation

Question 611

Give 5 red flag symptoms suggesting underlying disorder causing constipation in a child

Answer 611

Timing - Reported from birth or first few weeks of life Passage of meconium >48 hours Ribbon stools Abdominal distension Undiagnosed/known weakness in legs/locomotor delay

Question 612

Before starting treatment for constipation, what does a child need to be assessed for?

Answer 612

Faecal impaction

Question 613

Give 3 factors that suggest faecal impaction

Answer 613

Symptoms of severe constipation Overflow soiling Faecal mass palpable in the abdomen

Question 614

What is encopresis and how does it occur?

Answer 614

Term used for faecal incontinence Sign of chronic constipation where rectum becomes stretched and looses sensation. Hard stools remain in the rectum while loos ones are able to bypass, resulting in soiling

Question 615

How is faecal impactation managed? (2)

Answer 615

1st line - Macrogol (using escalating dose regimen 2nd line - Senna (Stimulant laxative)

Question 616

Describe the escalating dose regiment for a child aged 1-5 presenting with constipation. (disimpactation doses)

Answer 616

Two sachets on day 1 > 4 sachets for 2 days > 6 sachets for 2 days > 8 sachets daily Until one or two formed stools (soft) are produced each day)

Question 617

Describe the maintenance regimen for children with constipation (after disimpactation).

Answer 617

1st line - Macrogol (escalating dose regimen) (if child required disimpactation, dose is HALF disimpactation dose) 2nd line - Senna (stimulant laxative)

Question 618

What should be done if diarrhoea occurs 2nd to laxative use?

Answer 618

Reduce dose of laxative (as can lead to hypokalemia)

Question 619

Name 4 types of laxatives

Answer 619

Bulk-forming laxatives (ispaghula husk and methylcellulose) Osmotic laxatives (lactulose and macrogol) Stimulant laxatives (senna and sodium picosulfate) Stool softener laxatives (docusate sodium)

Question 620

Name 2 bulk forming laxatives and describe how they work

Answer 620

Ispaghula husk and methylcellulose Work similar to dietary fibre, increasing the bulk of stools, helping them retain fluid and encouraging bowels to push stools out

Question 621

Name 2 osmotic laxatives and describe how they work

Answer 621

Lactulose and Macrogol Act to soften stools, making them easier to pass by increasing the amount of water in the bowel

Question 622

Name 2 stimulant laxatives and describe how they work

Answer 622

Senna and sodium picosulfate Work by speeding up movement of bowels by stimulating contraction of the muscle lining of digestive tract

Question 623

Name 1 stool softener laxative and describe how it works

Answer 623

Docusate sodium Increases fluid content of hard, dry stools, making them easier to pass

Question 624

What is the management of bacterial meningitis? (2)

Answer 624

<3 months - IV amoxicillin (or ampicillin) ++ IV cefotaxime (to cover listeria) >3 months - IV cefotaxime (or ceftriaxone)

Question 625

What age should a child be able to sit without support?

Answer 625

6-8 months

Question 626

Define impetigo

Answer 626

Describes a superficial bacterial infection of the skin, commonly caused by Staphylococcus aureus and/or streptococcus pyogenes

Question 627

Name 3 types of impetigo

Answer 627

Non-bullous impetigo - Caused by Staphylococcus Aureus and/or Streptococcus pyogenes (majority of cases) Bullous impetigo - Staphylococcus aureus MRSA caused impetigo

Question 628

How does Non bullous Impetigo tend to present? (3)

Answer 628

Thin walled vesicles/pustules which release exudate forming a golden/brown crust May be pruritis (itchy) Negative Nikolsky sign

Question 629

What is Nikolsky sign?

Answer 629

Sign when top layers of skin slip away from bottom layers when rubbed. Skin rubs off leaving a red raw base

Question 630

How is impetigo spread?

Answer 630

Via direct contact with discharges from scabs o an infected person

Question 631

What is the incubation period of impetigo?

Answer 631

4-10 days

Question 632

Where on the body does impetigo tend to manifest?

Answer 632

Manifests on flexures, face, trunk and limbs (can be widespread on infants)

Question 633

How is impetigo diagnosed? (2)

Answer 633

Usually clinical Swabs (of exudate from moist lesion) for culture

Question 634

Name 4 complications of impetigo that would require referral to secondary care

Answer 634

Acute glomerulonephritis (following streptococcal impetigo) Cellulitis Sepsis Scarlet fever, urticaria and erythema multiforme (following streptococcal infection)

Question 635

How long should a child with impetigo be excluded from school?

Answer 635

Until lesions have healed, dried or crusted over Or 48 hours after starting antibiotics

Question 636

How is localised non-bullous impetigo managed? (1st and 2nd line)

Answer 636

1st line Hydrogen peroxide 1% cream (for patients not systemically unwell) 2nd line - Short course of topical antibiotic (Fusidic acid or Mupirocin)

Question 637

How is widespread non-bullous impetigo managed in children? (1st, 2nd and 3rd line)

Answer 637

1st line - Topical fusidic acid or Mupirocin (if fusidic acid resistant) (if not sytemically unwell) OR 1st line - Oral flucloxacillin (do not combine with topical, one or the other) 2rd line - Clarithromycin/Erythromycin (if penicillin allergy)

Question 638

What is the 1st and 2nd line for bullous impetigo in children?

Answer 638

Oral antibiotics; 1st line - Oral flucloxacillin 2nd line - Clarithromycin/Erythromycin (if penicillin allergy)

Question 639

What is the difference between bullous and non bullous impetigo? (4)

Answer 639

Size of the vesicles. Non-bullous tend to be pustules, while bullous tend to be vesicles >5mm in diameter) Bullous tends to present on trunkand upper extremities, whils non-bullous is on face, nose and mouth Bullous may present with systemic signs (fever, malaise, weakness)

Question 640

What reflex may occur in a young child suddenly immersed in cold water? How is it characterised?

Answer 640

Mammalian diving reflex Characterised as bradycardia, apnea and vasoconstriction of nonessential vascular beds with shunting of blood to the coronary and cerebral circulation

Question 641

What nervous system mediates laryngospasm in drowning?

Answer 641

Parasympathetic

Question 642

Describe infectious mononucleosis. Mention triad of symptoms

Answer 642

AKA glandular fever, infectious mononucleosis is an acute condition caused by Epstein-Barr Virus. Present with a triad of; Sore throat Lymphadenopathy Pyrexia/fever

Question 643

What pathogen causes infectious mononucleosis?

Answer 643

Epstein Barr Virus

Question 644

How is the presentation of lymphadenopathy different in infectious mononucliosis vs tonsilitis?

Answer 644

IM - Lymphadenopathy occurs in the anterior and posterior triangles of the neck Tonsillitis - Typically only occurs in the upper anterior cervical chain

Question 645

What medication can trigger a maculopular, pruritic rash in a patient with infectious mononucleosis?

Answer 645

Ampicillin/amoxicillin

Question 646

What 3 blood findings may be presewnt in a patient with infectious mononucleosis?

Answer 646

Lymphocytosis Haemolytic anaemia (2nd to cold agglutins IgM) Hepatitis (transient rise in ALT)

Question 647

What tests are used to diagnose infectious mononucleosis?

Answer 647

Monospot test + FBC in 2nd week of illnss

Question 648

What does the monospot test diagnose and what does it detect?

Answer 648

Diagnoses - Infectious mononucleosis Detects - Heterophile antibodies produced in response to EBV infection.

Question 649

Give one alternative name for EBV

Answer 649

Human herpesvirus 4 - HHV-4

Question 650

Define haemolytic uraemic syndrome

Answer 650

HUS describes a thrombotic microangiopathy in whihc microthrombi, consisting mainly of platelets, form and occlude arterioles and capillaries

Question 651

What triad is typically seen in Haemolytic Uraemic syndrome?

Answer 651

Acute kidney injury Microangiopathic haemolytic anaemia (normocytic anaemia) Thrombocytopenia

Question 652

What type of aneamia is seen in microangiopathic haemolytic anaemia?

Answer 652

Normocytic anaemia

Question 653

Give 4 clinical features of haemolytic uraemic syndrome

Answer 653

Bloody diarrhoea (for past 5-10 days) Thrombocytopenia (rash, mucosal bleeding, prolonged bleeding) Normocytic anaemia ; Fatigue, dyspnea, pallor, jaundice) Impaired renal function; Hematuria, proteinuria, oliguria

Question 654

Give 3 secondary causes of haemolytic uraemic syndrome. Which is most common?

Answer 654

Shiga toxin producing E.coli (most common) Pneumococcal infection HIV

Question 655

What is the main cause of primary haemolytic uraemic syndrome?

Answer 655

Compliment dysregulation

Question 656

What invesitgations would you order for haemolytic uraemic syndrome? (5)

Answer 656

FBC - (Anaemia, Increased reticulocytes and schistocytes) Coagulation profile (decreased platelets) WCC - Increased WBCs U&Es - Increased creatinine Urinalysis (haematuria and proteinuria - AKI)

Question 657

What medication should be offered to close contacts of a patient within 7 days of a patient with bacterial meningitis?

Answer 657

Oral Ciprofloxacin

Question 658

What immunisations are given at 12-13 months? (4)

Answer 658

Hib/MenC + MMR + PCV + Men B

Question 659

What vaccines are given at 2 months?

Answer 659

6-1 vaccine Oral rotavirus vaccine Men B

Question 660

What vaccines are given at 3 months?

Answer 660

6-1 vaccine Oral rotavirus vaccine PCV (pneumococcal)

Question 661

What vaccines are given at 4 months?

Answer 661

6-1 vaccine Men B

Question 662

What vaccines are given at 3-4 years?

Answer 662

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

Question 663

What diseases does the 4-1 pre-school booster protect against?

Answer 663

Diphtheria Tetanus Whooping cough Polio

Question 664

Describe the management of chicken pox (3)

Answer 664

School exclusion (until vesicles have crusted over - usually 5 days after onset of rash) Calamine lotion Immunocompromised - Varricella zoster immunoglobulin

Question 665

Describe 2 symptoms of chicken pox

Answer 665

Fever Itchy, rash starting on head/trunk before spreading. (initially macular then papular then vesicular)

Question 666

Decribe NICE guidence on school exclusion for a child with chicken pox

Answer 666

Most infectious period is 1-2 days before rash appears. Infectivity continues until all lesions are dry and have crusted over (usually 5 days after onset of rash)

Question 667

Name 4 drugs that can cause Steven Johnson Syndrome

Answer 667

Allopurinol Penicillin Lamotrigine Phenytoin

Question 668

Give 4 complications of chickenpox

Answer 668

Bacterial superinfection Cerebellitis DIC Progressive disseminated disease

Question 669

How may bacterial meningitis appear on LP?

Answer 669

Turbid appearance, raised polymorphs, raised protein, low glucose

Question 670

How may viral meningitis appear on LP?

Answer 670

Clear appearance, raised lymphocytes, normal/raised protein, normal/low glucose

Question 671

How may TB meningitis appear on LP?

Answer 671

Turbid/clear appearance, raised lymphocytes, raised protein, low glucose

Question 672

How may Encephalitis appear on LP?

Answer 672

Clear appearance,normal/raised lymphocytes, normal/raised protein, normal/low glucose

Question 673

How is estimated weight calculated for children

Answer 673

Estimated weight = (age+4)x2

Question 674

What ml of bolus administered to a child in shock?

Answer 674

20m/kg Caveat - In DKA or HF give 10ml/kg

Question 675

How is hourly rate of fluid resuscitation calculated in children?

Answer 675

((Deficit-initial bolus)/48hr) + Maintenance per hour

Question 676

Define neonatal hypoglycaemia

Answer 676

Blood glucose <2.6mmol/L

Question 677

Give 2 features of neonatal hypoglycaemia

Answer 677

Autonomic features; Jitteriness, irritability, pallor, tachypnoea Neuroglycopenic symptoms; poor feeding/sucking, weak cry, drowsy, seizures

Question 678

How is asymptomatic hypoglycaemia managed? (2)

Answer 678

Encourage normal feeding (breast or bottle) Monitor blood glucose

Question 679

How is symptomatic/very low blood glucose neonatal hypoglycaemia managed? (2)

Answer 679

Admit to the neonatal unit IV infusion of 10% dextrose

Question 680

Define ophthalmia neonatorum

Answer 680

Infection of the newborn eye

Question 681

What 2 organisms commonly cause Ophthalmia neonatorum

Answer 681

Chlamydia trachomatis Neisseria gonorrhoeae

Question 682

How may ophthalmia neonatorum present? (3)

Answer 682

Neonate presenting with history of 'sticky eyes' Bilateral purulent discharge Conjunctival injection

Question 683

How should ophthalmia neonatorum be managed?

Answer 683

Same day referral to ophthalmology/paediatric assessment

Question 684

Give 4 neonatal characteristics of maternal Rubella Infection

Answer 684

Sensorineural deafness Congenital Cataracts Congenital heart disease (PDA) Glaucoma

Question 685

Give 3 neonatal characteristics of maternal Toxoplasmosis infection

Answer 685

Cerebral calcification Chorioretinitis (inflammation of choroid in eye) Hydrocephalus

Question 686

Give 4 neonatal characteristics of maternal cytomegalovirus infection

Answer 686

Low birth weight Purpuric skin lesions Sensorineural deafness Microcephaly

Question 687

Roseola infantum is caused by what pathogen?

Answer 687

Human Herpes Virus 6 (HHV6)

Question 688

What is the incubation period of Roseola infantum? Who does it typically affect?

Answer 688

5-15 days Affects children aged 6 months to 2 years

Question 689

Give 5 features of Roseola infantum

Answer 689

High fever: Lasting a few days, followed by; Maculopapular rash Nagayama spots (papular enanthem on the uvula and soft palate) Febrile convulsions Diarrhoea and cough

Question 690

What lice causes headlice?

Answer 690

Pediculosis capitis

Question 691

How long does it take head lice eggs to hatch?

Answer 691

7-10 days

Question 692

How are headlice spread?

Answer 692

Direct head to head contact

Question 693

How are headlice diagnosed?

Answer 693

Fine-toothed combing of wet or dry hair

Question 694

Give 4 treatments of headlice

Answer 694

Malathion Wet combing Dimeticone Isopropyl myristate

Question 695

What organism commonly causes meningitis in neonate - 3 months

Answer 695

Group B Streptococcus

Question 696

What organisms commonly causes meningitis between 1 month and 6 years? (3)

Answer 696

Neisseria Meningitidis Streptococcus pneumoniae Haemophilus influenzae

Question 697

What organisms commonly cause meningitis in children >6 years? (2)

Answer 697

Neisseria meningitidis Streptococcus pneumoniae

Question 698

Define precocious puberty. In which sex is it more common?

Answer 698

Development of secondary sexual characteristics before 8 years in females and 9 years in males. More common in females.

Question 699

What term is used to describe the first stage of breast development?

Answer 699

Thelarche

Question 700

What term is used to describe the first stage of pubic hair development?

Answer 700

Adrenarche

Question 701

Name and describe 2 classifications for precocious puberty

Answer 701

Gonadotrophin dependent (due to premature activation of Hypothalamic-pituitary-gonadal axis) (FSH and LH is raised) Gonadotrophin independent (pseudo) (due to excess sex hormones_ (FHS and LH is low)

Question 702

What may bilateral enlargement of testes and precocious puberty suggest ?

Answer 702

Gonadotrophin release from an intracranial tumour

Question 703

What may unilateral enlargement of a testicle and precocious puberty suggest?

Answer 703

Gonadal tumour

Question 704

What may small testes and precocious puberty suggest?

Answer 704

Adrenal cause (tumour or adrenal hyperplasia)