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Phase 3a > Paediatrics > Flashcards

Paediatrics Flashcards

1
Q

Give 7 signs of respiratory distress

A

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

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2
Q

Name 4 causative pathogens for viral induced wheeze

A

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

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3
Q

What virus is responsible for the common cold?

A

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

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4
Q

Atopy is related to a deficiency in what cytokine?

A

Interferon B

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5
Q

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

A

Oxygen
Bronchodilator; Salbutamol +/- Spacer

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6
Q

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

A

Oral Prednisolone (inhaled steroid)

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7
Q

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

A

Low dose inhaled corticosteroid
or
Leukotriene receptor antagonist-montelukast

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8
Q

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

A

1st line - Amoxicillin

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

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9
Q

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

A

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

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10
Q

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

A

Paracetamol +/- Ibuprofen for fever

Regular fluids

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

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11
Q

List 5 DDx for viral induced wheeze

A

Asthma
Bronchiolitis
Inhaled foreign body
Cystic fibrosis
Gastro-oesophageal reflux

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12
Q

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

A

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

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13
Q

Define episodic viral wheeze

A

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.

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14
Q

Define multi-trigger wheeze

A

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

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15
Q

List 5 risk factors for viral induced wheeze

A

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

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16
Q

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

A

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

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17
Q

What pattern of inheritance is seen in Cystic Fibrosis?

A

Autosomal Recessive Disorder

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18
Q

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

A

CFTR gene and cAMP regulated chloride channel

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19
Q

Describe the pathophysiology of cystic fibrosis

A

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.

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20
Q

Where is the genetic defect in cystic fibrosis?

A

Delta F508 on the long arm of chromosome 7

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21
Q

Name 3 organisms that commonly colonise cystic fibrosis patients

A

Staphylococcus aureus
Pseudomonas aeruginosa
Aspergillus

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22
Q

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

A

Failure to pass meconium (meconium ileus)

Occurs due to thickening of meconium.

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23
Q

List features of cystic fibrosis in children (6)

A

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

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24
Q

List some complications of cystic fibrosis (5)

A

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

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25
Q

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

A

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.

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26
Q

What 5 conditions does the heel prick test test for?

A

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

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27
Q

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

A

Respiratory system
Pancreas
Intestine (bulky stools > intestinal blockage)

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28
Q

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

A

Sweat test - Sweat Chloride >60mmol/L

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29
Q

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

A

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

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30
Q

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

A

A cellulitis (deep skin infection) of the supraglottis

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31
Q

What pathogen commonly causes acute epiglottitis?

A

Haemophillus Influenzae Type B (Hib)

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32
Q

What is the median age of presentation for acute epiglottitis?

A

Between 6-12 years old

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33
Q

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

A

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

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34
Q

What investigation is diagnostic for acute epiglottitis?

A

Direct visualisation via fibre optic laryngoscopy (showing inflamed epiglottis)

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35
Q

List diagnostic features of acute epiglottitis (7)

A

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

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36
Q

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

A

Stridor.

Suggests upper airway obstruction

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37
Q

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

A

Cough

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38
Q

Describe the acute management of acute epiglotitis.

A

Secure airway - Lie patient un upright position > avoids aggrevating any airway obstruction.

IV antibiotics (Cefotaxime/Ceftriaxone)

Adjuncts; Oxygen + Dexamethasone

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39
Q

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

A

Amoxicillin

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40
Q

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

A

Cefotaxime/Ceftriaxone

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41
Q

Name 4 complications of acute epiglottitis

A

Abscess formation
Meningitis
Sepsis
Pneumothorax

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42
Q

Name 2 risk factors for acute epiglottitis

A

Non vaccination with Hib vaccine.

Immunocompromise

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43
Q

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

A

Varicella Zoster Virus

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44
Q

What virus causes glandular fever?

A

Epstein Barr Virus (EBV)

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45
Q

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

A

Coxsackie A virus

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46
Q

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

A

IgE and IL-4

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47
Q

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

A

1st dose at 1 years old

2nd dose at 3 years and 4 months old

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48
Q

What kind of virus is measles?

A

Single stranded RNA virus

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49
Q

What is the incubation period for Measles?

A

2 weeks

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50
Q

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

A

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

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51
Q

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

A

Coryza, Cough, Conjunctivitis.

Koplik Spots

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52
Q

What diagnostic test is used to diagnose Measles?

A

Measles specific IgM and IgG serology (ELISA)

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53
Q

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

A

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

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54
Q

How is measles managed in children? (2)

A

Supportive treatment - Paracetamol and Ibuprofen

Vitamin A supplementation

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55
Q

Give 4 possible complications of measles.

Which is the most common and what is the most common cause of death?

A

Pneumonia (most common cause of death)

Otitis media (most common)

Encephalitis

Subacute sclerosing panencephalitis (SSPE)

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56
Q

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

A

7 - 10 years after

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57
Q

What is Subacute Sclerosing Panencephalitis?

A

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

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58
Q

Describe the 4 stages of Subacute Sclerosing Panencephalitis

A

Stage 1 - Dementia and Personality Changes

Stage 2 - Epilepsy and Myoclonus

Stage 3 - Spasticity and Extrapyramidal symptoms

Stage 4 - Vegetative state and autonomic failure

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59
Q

What diagnostic investigation would suggest Subacute Sclerosing Panencphalitis?

A

CSF fluid showing elevated anti-measles IgG

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60
Q

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

A

Offer MMR vaccine.

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

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61
Q

Who should be notified if a patient has measles?

A

Public Health England

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62
Q

What kind of virus is mumps?

A

RNA paramyxovirus

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63
Q

During which seasons does mumps tend to present?

A

Winter and Spring

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64
Q

How does Mumps typically manifest?

A

Parotitis (swelling and inflammation of the parotid glands)

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65
Q

What is the incubation period for mumps?

A

14-21 days

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66
Q

Describe the pattern of infectivity for mumps

A

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

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67
Q

Give 3 complications of Mumps.

Which is the most common?

A

Orchiditis (most common)

Aseptic meningitis

Hearing loss (less common since MMR vaccine)

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68
Q

How is mumps managed?

A

Self limiting disease

Supportive treatment - Paracetamol for high fever

Notifiable Disease - Inform public health England

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69
Q

What kind of virus is Rubella?

A

Positive sense, single stranded RNA virus

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70
Q

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

A

Sensorineural hearing loss

Cataracts

Cardiac defects (i.e patent ductus arteriosus)

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71
Q

Describe 4 clinical features of rubella

A

Maculopapular rash (begins in head and progresses to trunk/legs)

Arthralgia

General malaise, conjunctivitis and coryza

Lymphadenopathy

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72
Q

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

A

Pregnant women require serology to confirm diagnosis (detection of IgM antibodies)

Due to potential to develop congenital rubella syndrome

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73
Q

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

A

Pregnant
- Specialist referral
- IM immunoglobulin

Non-pregnant
- Supportive care
- NSAIDs for arthralgia
- Antihistamines for pruritis

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74
Q

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

A

Can cause pneumonitis

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75
Q

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

A

1st trimester as this is the period of organogenesis

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76
Q

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

A

Cardiac defects (most common - PDA, Pulmonary artery stenosis)

Cataracts

Cochlear defects (bilateral sensorineural hearing loss)

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77
Q

What diseases does the 6 in 1 vaccine protect against?

A

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

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78
Q

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

A

2 months, 3 months and 4 months

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79
Q

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

A

12-13 years old

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80
Q

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

A

MMR

Diphtheria, tetanus, pertussis, hepatitis B

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81
Q

What pathogen does the fever pain score screen for?

A

Likely streptococcus infection

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82
Q

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

A

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)

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83
Q

What pathogen typically causes Scarlet Fever?

A

Group A Streptococcus (Streptococcus pyogenes, GAS)

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84
Q

What is the incubation period for Scarlet Fever?

A

2-4 days

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85
Q

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

A

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

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86
Q

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

A

Strawberry Tongue

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

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87
Q

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

A

Typically 12-48 hours after onset of fever.

Lasts for 7 days

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88
Q

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

A

Occurs 7-10 days after resolution of rash.

Skin desquamation occurs on face, trunk, hands, fingers and toes

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89
Q

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

A

1st line - Phenoxymethylpenicillin (penicillin V)

2nd line - Amoxicillin (if penicillin v cannot be tolerated)

3rd line - Azithromycin (if penicillin allergy)

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90
Q

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

A

Azithromycin

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91
Q

Give 4 possible complications of penicillin use.

A

Hypersensitivity reactions

Haemolytic anaemia (positive Coombs test)

Drug induced interstitial nephritis

Seizures

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92
Q

What kind of antibiotic is Penicillin?

A

Beta Lactam Antibiotic

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93
Q

What kind of antibiotic is Azithromycin?

A

Macrolides

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94
Q

In which patients is Azithromycin and Clarithromycin contraindicated?

A

Hepatic failure

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95
Q

Give 3 possible side effects of azithromycin

A

GI Upset

QT Prolongation

Hypertrophic Pyloric Stenosis (in infants up to 6 weeks)

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96
Q

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

A

Poststreptococcal glomerulonephritis

May present 1-6 weeks following streptococcal infection

Presents with;
Haematuria
Hypertension
Oedema (facial)

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97
Q

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

A

Presence of a skin wound

May be warm, oedematous and purple

Skin may be peeling with white discharge

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98
Q

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

A

Concurrent chickenpox or influenza

Immunocompromised

Comorbidities such as skin breakdown, diabetes or underlying malignancy

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99
Q

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

A

Parvovirus B19

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100
Q

What complication can Parvovirus B19 cause in adults?

A

Acute arthritis

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101
Q

What complication can Parvovirus B19 cause in Pregnant women?

A

Hydrops fetalis

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102
Q

What is PEFR

A

Peak Expiratory Flow Rate

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103
Q

What is the PEFR in moderate Viral Induced Wheeze?

A

At least 50%

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104
Q

What is the PEFR in acute severe Viral Induced Wheeze?

A

<50%

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105
Q

What is the PEFR in severe Viral Induced Wheeze?

A

<33%

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106
Q

What pathogen most commonly causes bronchiolitis?

A

RSV (respiratory syncytial virus)

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107
Q

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

A

Bronchiolitis

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108
Q

Give 4 risk factors for bronchiolitis

A

Social deprivation

Winter months

Prematurity or bronchopulmonary dysplasia

Smoking and air pollution

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109
Q

Give 4 symptoms of bronchiolitis

A

Symptoms are generally mild

Corysal symptoms (mild fever, runny nose, sneezing)

Cough

Tachypnea

Wheeze`

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110
Q

Give 6 red flag symptoms of bronchiolitis that require hospital admission

A

Poor feeding

Breathing difficulties (RR>70)

Features of respiratory dirsress

Apnea

Central cyanosis

Persistent O2 sats <92% when breathing air

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111
Q

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

A

Respiratory rate >60

Difficulty breast feeding or inadequate oral fluid intake (50-75% of usual volume)

Clinical dehydration

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112
Q

How is bronchiolitis diagnosed?

A

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)

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113
Q

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

A

Hyponatremia

May present with;
Tachypnoea, tachycardia and seizures

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114
Q

Give 4 differentials for bronchiolitis

A

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)

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115
Q

How is bronchiolitis managed?

A

Largely supportive;

Chest physiotherapy

Oxygen supplementation

CPAP

Upper airway suction (if apnea or respiratory distress_

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116
Q

What is the most common cause of Croup?

A

Parainfluenza virus type 1 or type 3

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117
Q

Give 5 classic symptoms of Croup

A

Sudden onset

Seal-like barking cough

Inspiratory stridor

Voice hoarseness

Respiratory distress (more severe cases)

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118
Q

What can cause symptoms of Croup to increase?

A

Agitation

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119
Q

Describe mild croup (3)

A

Seal like braking

No stridor or sternal/intercostal recessions at rest

Symptoms resolve in 48 hours

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120
Q

Describe moderate croup (4)

A

Seal like braking

Stridor AND sternal/intercostal recessions at rest.

Little or no agitation or lethargy

Requires hospital admission

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121
Q

Describe severe croup (4)

A

Seal like braking

Stridor AND sternal/intercostal recessions

Associated with agitation/lethargy

Requires hospital asmission

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122
Q

What ages does croup typically present?

A

Between 6 months and 3 years old

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123
Q

What season is croup most common?

A

Autumn (September to December)

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124
Q

Give 3 differentials for croup

A

Bacterial tracheitis

Epiglottitis

Foreign body in upper airway

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125
Q

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

A

1st line - Oral Dexamethasone

2nd line - Inhaled budenoside or IM dexamethasone

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126
Q

What is the emergency management of croup? (3)

A

Nebulised adrenaline (epinephrine)

Corticosteroids (dexamethasone oral or IM/budenoside)

Supplemental oxygen

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127
Q

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

A

Amoxicillin

Penicillin allergy - Clarithromycin

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128
Q

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

A

Co-amoxiclav

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129
Q

Describe 4 severe symptoms of pneumonia in children

A

Difficulty breathing

O2 sats <90%

Signs of respiratory distress

Poor feeding

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130
Q

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

A

Type 1 hypersensitivity reaction.

IgE mediated

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131
Q

What are the 2 types of asthma?

A

Allergic/Eosinophilic - Atopy (IgE mediated)

Non-allergic/Non-eosinophilic (associated with environmental factors., i.e smoking, cold, pollution ect)

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132
Q

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

A

Spirometry

FEV1/FVC <80% predicted

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133
Q

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

A

Peak Expiratory Flow Rate (PEFR)

Measures airflow obstruction so can indicate severity.

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134
Q

Describe the drug ladder for Asthma (7)

A
  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
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135
Q

What is the criteria for moderate asthma? (1)

A

PEFR 50-75%

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136
Q

What is the criteria for severe asthma? (4)

A

PEFR 33-50%

RR >25

HR >110

Can’t complete sentences in one breath

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137
Q

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

A

Any one of CHEST.

PEFR <33%

Sp02 <92%

Cyanosed

Hypotension

Exhaustion, confusion

Silent chest

Tachy/brady/arrhythmas

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138
Q

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

A

Oxygen + SABA + Prednisolone (or IV hydrocortisone)

Switch SABA to ipratropium bromide if poor response.

Quadruple prednisolone dose and give for 14 days

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139
Q

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

A

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

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140
Q

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

A

Oral prednisolone

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141
Q

Define Kawasaki Disease

A

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

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142
Q

What complication is common in Kawasaki disease?

A

Coronary artery aneurysms

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143
Q

Give 5 symptoms of Kawasaki Disease (CRASH)

A

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

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144
Q

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

A

Scarlet fever - Fever <5 days

Kawasaki disease - Fever >5 days

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145
Q

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

A

Echocardiogram - To screen for coronary artery aneurysms

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146
Q

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

A

1st line - IV immunoglobulins (IVIG) + High dose aspirin

2nd line - Corticosteroids - Methylprednisolone/prednisolone

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147
Q

What risk does aspirin use bring in children?

A

Developing Reye’s syndrome

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148
Q

What is Reye Syndrome?

A

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

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149
Q

Give 2 clinical features of Reye syndrome

A

Preceeding viral infection
- Symptoms begin 3-5 days after viral illness

Acute encephalopathy
- Severe vomiting
- Altered mental status
- Neuro symptoms - Seizures, fixed pupils
-

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150
Q

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

A

LFTs - Raised AST/ALT

Hyperammonemia

Hypoglycemia

Metabolic acidosis

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151
Q

Define Juvenile Idiopathic Arthritis

A

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

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152
Q

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

A

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

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153
Q

Define oligoarticular JIA

A

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

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154
Q

Give 2 clinical features of oligoarticular JIA

A

Asymmetrical pattern (most commonly affecting weight bearing joints - Knee and Ankle)

Extra-articular manifestations - Chronic anterior uveitis (bilateral is common)

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155
Q

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

A

Positive ANA (70%)

Negative RF

Raised ESR

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156
Q

Give 4 risk factors for JIA

A

Female sex

HLA polymorphism

Age under 6

Family history of autoimmunity

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157
Q

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

A

1st line - Intra-articular corticosteroid (Triamcinolone or Methylprednisolone acetate)

2nd line - TNF alpha inhibitors (Adalimumab or Etanercept)

Adjuncts; NSAIDs, Methotrexate

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158
Q

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

A

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

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159
Q

Give 2 contraindications for Adalimumab (TNF-a inhibitor)

A

Demyelinating diseases

Hepatitis B

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160
Q

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

A

RF factor

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161
Q

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

A

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

Chronic anterior uveitis

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162
Q

What investigation results would you expect in seronegative polyarticular arthritis?

A

Positive ANA (30%)

Negative RF

Raised ESR

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163
Q

Define polyarticular arthritis

A

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

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164
Q

What pattern of incidence is displayed in seronegative polyarticular arthritis?

A

Bimodal incidence.

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

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165
Q

Give 2 clinical features of seropositive polyarticular arthritis

A

Symmetrical pattern of joint involvement

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

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166
Q

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

A

1st line - DMARD - Methotrexate

2nd line - Lefunomide/Sulfasalazine

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167
Q

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

A

1st line adjunct - TNF-a inhibitor (adalimumab)

2nd line adjunct - IL-6 inhibitor (tocilizumab)

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168
Q

What kind of drug is tocilizumab?

A

IL-6 inhibitor

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169
Q

What kind of drug is adalimumab?

A

TNF-a inhibitor

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170
Q

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

A

Folic acid. To decrease side effects (Anaemia)

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171
Q

What type of anaemia occurs with methotrexate treatment?

A

Megaloblastic anaemia (due to folic acid deficiency)

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172
Q

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

A

FBC, Creatinine and LFTs

Repeat every 3-4 months during treatment

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173
Q

What should patients be screened for before starting methotrexate?

A

Hepatitis B and C

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174
Q

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

A

Hepatotoxicity

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

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175
Q

Define Systemic JIA (Still Disease(

A

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.

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176
Q

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

A

Salmon pint macular rash (non-pruritic)

Generalised lymphadenopathy

Spleno/Hepatomegaly

Serositis (peritonitis, pleuritis and/or pericarditis)

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177
Q

What is Koebner’s phenomenon? (systemic JIA)

A

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

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178
Q

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

A

Raised ESR (chronic) and CRP (acute)

Anaemia, Leukocytosis and Thrombocytosis

RF negative

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179
Q

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

A

1st line Oral/IV corticosteroid - Methylprednisolone or Prednisolone

2nd line - Tocilizumab (IL-6 inhibitor), Canakinumab or Anakinra (IL-1 inhibitor)

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180
Q

What kind of drug is Anakinra?

A

IL-1 inhibitor

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181
Q

What kind of drug is Canakinumab?

A

IL-1b monoclonal antibody

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182
Q

Define psoriatic arhritis

A

Describes either;

Arthritis + Psoriasis

OR

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

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183
Q

Describe 2 clinical features of psoriatic JIA

A

Asymmetrical pattern of joint involvement

Extra-articular manifestations;
- Chronic anterior uveitis
- Nail changes
- Psoriatic skin lesions

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184
Q

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

A

ANA positive (50%)

HLA-B27 may be positive

RF negative

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185
Q

Describe the management of psoriatic JIA

A

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

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186
Q

What is enthesitis?

A

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

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187
Q

Define Enthesitis related JIA

A

Arthritis with Enthesitis

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188
Q

Give 2 clinical features of Enthesitis related JIA

A

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

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189
Q

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

A

HLA-B27 positive (80%)

RF negative

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190
Q

Give 5 differentials for JIA

A

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)

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191
Q

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

A

Rare complication of Systemic JIA

Characterised by;
Thrombocytopenia
Elevated transaminases
Low fibrinogen
Increased ferritin

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192
Q

What is the treatment for macrophage activation syndrome?

A

IV prednisolone +/- cyclosporine

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193
Q

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

A

Describes an idiopathic avascular necrosis of the femoral head.

Manifests between the ages of 4-10.

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194
Q

What type of gait is seen in Perthes disease?

A

Antalgic gait (on weight bearing leg)

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195
Q

Give 3 clinical features of Perthes’ disease

A

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)

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196
Q

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

A

Widening joint space (early)

Decreased femoral head size/flattening (later)

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197
Q

Give 2 complications of Perthes’ disease

A

Osteoarthritis

Premature fusion of growth plates

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198
Q

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

A

Conservative management (monitor)

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199
Q

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

A

Femoral osteotomy

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200
Q

Give 3 risk factors for Perthes’ disease

A

Male sex

Hyperactivity

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

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201
Q

What does developmental dysplasia of the hip refer to?

A

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

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202
Q

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

A

Positive Barlow test (hip adduction)

Positive Ortolani test (Hip Abduction)

Limited hip abduction

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203
Q

What are the 4 main risk factors for DDH?

A

Female sex

Breech presentation at birth

Positive family history

Oligohydramnios (too little amniotic fluid)

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204
Q

What does a positive Barlow test show?

A

Positive shows reduced hip is subluxatable or dislocatable

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205
Q

What does a positive Ortolani test show?

A

Positive shows that the dislocated hip is reducible

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206
Q

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

A

Asymptomatic

Positive Barlow and Ortolani Signs

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207
Q

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

A

Inability to abduct hip

Prominent Galeazzi sign (unequal knee height when lying supine)

Asymmetrical gluteal folds

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208
Q

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

A

Hip pain

Hip deformity (coxa vara - decreased femoral angle)

Waddling or Trendelenburg gait

Leg Length Discrepency

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209
Q

Give 3 criteria that warrant screening for DDH

A

1st degree relative with DDH/early life hip problems

Breech presentation at or after 36 weeks

Multiple pregnancy

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210
Q

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

A

Ultrasound at 6 weeks

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211
Q

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

A

X-ray

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212
Q

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

A

1st line - Observation

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

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213
Q

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

A

1st line - Closed reduction with spica casting

2nd line - Open reduction with spica casting

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214
Q

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

A

Appears to be unwell or deteriorating

Altered responsiveness (irritable, lethargic)

Sunken eyes

Tachycardia

Tachypnoea

Reduced skin tugor

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215
Q

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

A

Jittery movements

Increased muscle tone

Hyperreflexia

Convulsions

Drowsiness or Coma

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216
Q

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

A

Between 10-14 weeks pregnancy

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217
Q

Which chromosome is tripled in Down’s Syndrome?

A

Chromosome 21 (Trisomy 21)

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218
Q

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

A

Upward slanting eyelids (palpebral fissures)

Epicanthal folds (eyes)

Low set ears

Protruding tongue

Short stature

Transverse palmar crease

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219
Q

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

A

Atrioventricular Septal Defect (left to right shunt)

Results in excessive pulmonary blood flow and biventricular volume overload leading to pulmonary hypertension and heart failure

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220
Q

What type of shunt occurs in a Atrioventricular Septal Defect?

A

Left to Right Shunt

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221
Q

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

A

Duodenal atresia/stenosis

Hirschprung disease

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222
Q

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

A

Hypogonadism

Decreased fertility (men)

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223
Q

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

A

Acute Lymphoblastic Leukaemia

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224
Q

Give 6 complications of Down’s Syndrome

A

Hypothyroidism

Type 1 diabetes

Obstructive sleep apnea (snoring)

Hearing loss (due to otitis media)

Early onset Alzheimer’s Disease

Learning Difficulties

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225
Q

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

A

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

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226
Q

When does Hirschprung’s Disease typically present?

A

1st year of life

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227
Q

Give 4 features of Hirschprung’s disease

A

Vomiting

Abdominal Distension

Enterocolitis

Constipation

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228
Q

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

A

Rectal biopsy showing absence of ganglion cells

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229
Q

Describe the pathophysiology of Hirschprung’s disease

A

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

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230
Q

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

A

Intrauterine - Polyhydramnios

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

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231
Q

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

A

Ultrasound. Shows Double Bubble Sign

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232
Q

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

A

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

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233
Q

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

A

Carbamazepine and Phenytoin

Carbamazepine can exacerbate hypothyroidism

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234
Q

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

A

Otalgia (ear pain)

Bulging tympanic membrane

Myringitis (erythema of tympanic membrane)

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235
Q

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

A

15-18 weeks

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236
Q

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

A

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)

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237
Q

Patau’s Syndrome describes an abnormality in which chromosome?

A

Chromosome 13 (Trisomy 13)

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238
Q

Give 4 clinical features of Patau’s Syndrome

A

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)

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239
Q

Give 2 complications of Patau’s syndrome

A

Cardiac defects - Ventricular Septal Defect and Patent Ductus Arteriosus

Polycystic Kidney Disease

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240
Q

Edward’s Syndrome describes an abnormality in which chromosome?

A

Chromosome 18 (trisomy 18)

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241
Q

Give 4 clinical characteristics of Edward’s Syndrome

A

Micrognathia (congenital mandibular hypoplasia - undersized jaw)

Low-set ears

Overlapping fingers/clenched fists

Rocker-bottom feet

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242
Q

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

A

Decreased Estriol

Decreased AFP

Decreased B-HCG

Normal/Decreased Inhibin A

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243
Q

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

A

Increased maternal age

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244
Q

What is the pattern of inheritance in Fragile X syndrome?

A

X-linked dominant

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245
Q

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

A

CGG trinucleotide repeat in FMR1 gene (during oogenesis)

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246
Q

Fragile X syndrome affects which sex more? And why?

A

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

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247
Q

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

A

The number of CGG trinucleotide repeats

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248
Q

Give 6 clinical features of Fragile X Syndrome

A

Male

Intellectual disability (+/- autism/hyperactivity)

Microcephaly

Large ears, testes (macro-orchidism) and face

Mitral valve prolapse > mitral regurgitation

Hypermobile joints

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249
Q

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

A

Soft, Decrescendo Pan Systolic murmur (radiates to axilla)

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250
Q

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

A

“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.

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251
Q

Give 5 clinical features of of Prader Willi Syndrome

A

Muscular Hypotonia and poor feeding during infancy

Hyperphagia + Childhood obesity

Developmental delay/short stature

Hypogonadism and infertility

Behavioural problems - OCD, temper ect

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252
Q

Give 3 complications of Prader Willi Syndrome

A

Sleep apnea (most common)

Type 2 diabetes

Obesity

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253
Q

Give 5 clinical features of Angelman Syndrome

A

Epileptic Seizures

Easily excitable (may show inappropriate laughter)

Ataxia

Intellectual disability

Fascination with Water

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254
Q

Describe William’s Syndrome

A

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

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255
Q

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

A

Chromosome 7

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256
Q

Give 5 clinical features of William’s Syndrome

A

Hypersociability

Learning Difficulties

Cardiac Disease (supravalvular aortic stenosis and renal artery stenosis)

Hypercalcaemia (due to increased sensitivity to vitamin D)

Elfin Facies (elven faces)

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257
Q

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

A

FISH (fluorescence in-situ hybridisation)

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258
Q

What chromosome is deleted in Prader Willi and Angelman Syndrome

A

Chromosome 15

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259
Q

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

A

Ghrelin

Hyperphagia (extreme hunger)

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260
Q

What is nocturnal enuresis?

A

Bed wetting

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261
Q

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

A

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)

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262
Q

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

A

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

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263
Q

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

A

Newborn - Otoacoustic emission

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264
Q

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

A

15:2

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265
Q

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

A

5 rescue breaths

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266
Q

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

A

Technetium scan

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267
Q

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

A

Rectal washouts/bowel irrigation

To prevent entrocolitis

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268
Q

When is Meningitis B vaccine given (3)?

A

2 months, 4 months and 12 months

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269
Q

What is the pattern of inheritance for Von Willebrand disease?

A

Autosomal dominant

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270
Q

Describe the pathophysiology of Von Willebrand disease (3)

A

Reduction or malfunction in Von Willebrand Factor

Factor usually promotes platelet adhesion to damaged endothelium.

VWF also stabilizes and carries factor VIII

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271
Q

What factor does Von Willebrand Factor stabilize and carry?

A

Factor VIII

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272
Q

Give 5 symptoms of Von Willebrand Disease

A

Bleeding from minor wounds

Mucosal bleeding

Excessive post-operative bleeding

Easy/excessive bruising

Epistaxis (nose bleeds)

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273
Q

Give 2 diagnostic tests and results for Von Willebrand Disease

A

Prolonged Activated Partial Thromboplastin Time (APTT)

Reduced Factor VIII activity

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274
Q

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

A

NSAIDs

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275
Q

Give 2 medications used in the management of Von Willebrand disease

A

Desmopressin (increases clotting factor VWF and Factor VIII)

Von Willebrand Factor/Factor VIII concentrate (used in surgery for major bleeds)

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276
Q

Von Willebrand Disease is a disease of what process?

A

Platelet adhesion

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277
Q

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

A

Aspirin

As it inhibits platelet function

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278
Q

What clotting factor is deficient in haemophila A

A

Factor VIII

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279
Q

What clotting factor is deficient in haemophila B

A

Factor IX

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280
Q

What clotting factor is deficient in haemophila C

A

Factor XI

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281
Q

In what population is haemophila C more common?

A

Jewish

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282
Q

What is the pattern of inheritance for Haemophilia A?

A

X-linked recessive

283
Q

Give 5 clinical features of Haemophilia A

A

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

284
Q

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

A

Prolonged Activated Partial Thromboplastin Time (aPTT)

Normal platelet count

Normal prothrombin time

285
Q

Give 3 treatments for haemophilia

A

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)

286
Q

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

A

Philadelphia chromosome (t9:22)

BCR-ABL gene fusion

287
Q

In what chromosome is the Philadelphia Chromosome an abnormality?

A

Chromosome 22

288
Q

What is the most common childhood cancer?

A

Acute Lymphoblastic Leukaemia (ALL)

289
Q

Describe the pathophysiology of acute lymphoblastic leukaemia (3)

A

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.

290
Q

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

A

Philadelphia chromosome (T9:22) - Bad prognosis - Hypodiploidy

T(12:21) - Good prognosis - Hyperdiploidy

291
Q

Give 2 risk factors for ALL

A

Ionizing radiation (x-rays) exposure during pregnancy

Downs syndrome (trisomy 21)

292
Q

Give 7 clinical features of ALL

A

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)

293
Q

Give 3 indications of bone marrow failure

A

Anaemia (low Hb)

Infection (low functioning WC)

Bleeding and bruising (low platelets)

294
Q

What is tumour lysis syndrome?

A

Condition triggered by combination chemotherapy.

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

295
Q

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

A

High uric acid (hyperuricemia)

High potassium (hyperkalaemia)

High phosphate (hyperphosphatemia)

Low calcium (hypocalcaemia)

296
Q

What is used in the treatment of tumour lysis syndorme?

A

Allopurinol (xanthine oxidate inhibitor - reduced uric acid production)

Rasburicase (recombinant version of urate oxidase - enzyme that metabolise uric acid into allantoin)

297
Q

What are the 3 types of beta thalassemia?

A

Minor (trait- carrier)

Intermedia

Major

298
Q

Give 5 clinical features of beta thalassaeia majora

A

Children <1

Failure to thrive

Recurrent bacterial infections

Severe anaemia from 3-6 months)

Extramedullary haematopoiesis > hepatosplenomegaly + bone expansion

299
Q

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

A

Hair on end sign

300
Q

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

A

Large and small irregular hypochromic red blood cell

301
Q

What is the pattern of inheritance on Sickle Cell anaemia?

A

Autosomal recessive

302
Q

What does being heterozygous for sickle cell anaemia protect against?

A

Falciparum malaria

303
Q

Describe 1 complication of sickle cell anaemia (3)

A

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.

304
Q

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

A

Hypoxia

Cold weather

Infection

Dehydration

Acidosis

305
Q

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

A

Dactylitis (inflammation of digits)

306
Q

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

A

Hb Electrophoresis

Distinguishes HbSS (homozygotes) and HbAS (heterozygotes) states

307
Q

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

A

Hydroxyurea

Increases HbF levels

308
Q

What vaccine should sickle cell patients receive every 5 years?

A

Pneumococcal polysaccharide vaccine

309
Q

What is the MOA for tranexamic acid

A

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

310
Q

How are chest compressions performed in children >1?

A

Single hand compressing the lower half of the sternum.

30:2

311
Q

How are chest compressions performed in children <1?

A

Two thumb encircling technique

312
Q

What is the most common cause of gastroenteritis in children?

A

Rotavirus

313
Q

What type of virus is rotavirus?

A

RNA retrovirus

314
Q

How is rotavirus transmitted?

A

Faecal-oral route

315
Q

When is the rotavirus vaccine given? (2)

A

8 weeks old and 12 weeks old

316
Q

3 risk factors for viral gastroenteritis

A

Lack of immunisation against rotavirus

Age <5 years

Exposure to people with gastroenteritis

317
Q

Give 4 clinical features of gastroenteritis

A

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

318
Q

How is gastroenteritis diagnosed

A

Clinical diagnosis

319
Q

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

A

Weight 0-10kg = 100ml/Kg fluids

5kg child receives 500ml/day fluid (maintenance fluid volume)

320
Q

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

A

Weight 10-20kg = 1000ml/kg + 50ml/kg for each kg above 10kg per day

15kg child receives 1250ml/day fluid (maintenance fluid volume)

321
Q

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

A

Weight >20kg = 1500ml/Kg + 20ml/Kg for each Kg above 20kg per day

25kg child receives 1600ml/day fluid (maintenance fluid volume

322
Q

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

A

<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

323
Q

Give 2 complications of gastroenteritis

A

Clinical dehydration

Hypernatremia dehydration

324
Q

Give 6 risk factors making a child increased risk of dehydration

A

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

325
Q

Give 4 signs of clinical shock in children

A

Decreased level of consciousness

Pale/mottled skin

Cold extremities

Tachycardia/tachypnea/hypotension

326
Q

How is appendicitis pain often described?

A

Presents as umbilical pain that moves to the lower right quadrant

327
Q

What organ is the appendix connected to?

A

Caecum (below the ileocecal valve)

328
Q

What is the main cause of appendicitis?

A

Luminal obstruction caused by faecolith > infection

329
Q

Give 5 complications of appendicitis

A

Abscess formation

Peritonitis

Sepsis

Intra-abdominal adhesions

Bowel obstruction

330
Q

Give 5 clinical features of appendicitis

A

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

331
Q

Name and describe 3 signs seen in appendicitis. Describe

A

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)

332
Q

Coeliac disease is mediated by which cell type?

A

T cells

333
Q

Describe the pathophysiology of coeliac disease

A

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

334
Q

Give 3 risk factors for coeliac disease

A

HLA-DQ2 and HLA-DQ8

Autoimmune disorders (T1DM, Autoimmune hepatitis, Hashimotos thyroiditis, IgA deficiency)

Herpetiformis (vesicular, pruritic skin eruption)

335
Q

What are 2 classifications of coeliac disease?

A

Non responsive coeliac disease

Refractory coeliac disease

336
Q

Describe non responsive coeliac disease

A

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

337
Q

Describe refractor coeliac disease

A

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

338
Q

Give 4 clinical features of coeliac disease

A

Diarrhoea

Steatorrhoea + foul smelling stools

Abdominal pain + distension

Weight loss/Failure to thrive

339
Q

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

A

Small bowel endoscopy and histology

Villous atrophy
Crypt Hyperplasia
Increased Intraepithelial Lymphocytes

340
Q

Describe coeliac serology (2)

A

1st line - IgA tissue transglutaminase (IgA-tTG) raised

2nd line - IgA Anti Endomysial antibody (IgA-EMA) raised

341
Q

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

A

Total IgA (Investigate IgA deficiency)

342
Q

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

A

IgG DGP (Deamidated gliadin peptide)

343
Q

What is the 1st line management of coeliac disease?

A

Long term adherence to gluten free diet

Avoid; Wheat, Barley and Rye.

344
Q

What 4 functional areas are assessed in a Developmental Examination

A

Gross Motor

Vision and Fine Motor

Hearing, Speech and Language

Social, Emotional and Behavioural

345
Q

At what age should a child be able to crawl?

A

8-9 months

346
Q

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

A

10 months

347
Q

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

A

12 months

Age limit:18 months

348
Q

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

A

2.5 years

349
Q

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

A

4-6 months

350
Q

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

A

3 - 18 months

6 - 2 years

8 - 2.5 years

351
Q

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

A

3 years

352
Q

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

A

3 years

353
Q

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

A

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

354
Q

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

A

7 months

355
Q

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

A

10 months

356
Q

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

A

12 months

357
Q

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

A

18 months

358
Q

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

A

10-12 months

359
Q

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

A

18 months

360
Q

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

A

18-24 months

361
Q

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

A

2 years

362
Q

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

A

3-4 years

363
Q

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

A

2.5-3 years

364
Q

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

A

Cows milk intolerance

365
Q

Give 4 classifications of diarrhoea

A

Secretory (cholera, c.difficile, E.coli)

Osmotic

Motility disorders (increased - thyrotoxicosis, decreased - Intussusception/obstruction)

Inflammatory (bloody diarrhoea - Salmonella, campylobacter, Crohn’s, Coeliac)

366
Q

Define malnutrition

A

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

367
Q

How is malnutrition defined by NICE? (3)

A

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

368
Q

Name 2 forms of malnutrition

A

Kwashiorkor

Marasmus

369
Q

How is Kwashiorkor malnutrition defined?

A

Chraracterised by decreased intake of protein and essential aminoacids

370
Q

Give 3 risk factors for Kwashiorkor malnutrition

A

Premature cessation of breast feeding

Chronic GI infection

Protein-deficient diet

371
Q

Give 6 clinical features of Kwashiorkor malnutrition

A

Muscle atrophy/poor growth

Diarrhoea

Bilateral pitting oedema

Distended bowel

Hepatosplenomegaly

Skin/hair depigmentation

372
Q

Give 3 investigation results seen in Kwashiorkor malnutrition

A

Decreased albumin

Decreased glucose

Decreased K, Mg and Hb

373
Q

How is Kwashiorkor malnutrition managed?

A

Gradually increasing, high protein diet.

374
Q

Give 1 possible complication of malnutrition management

A

Refeeding syndrome

375
Q

Describe the pathophysiology of refeeding syndrome

A

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

376
Q

Give 5 clinical features of refeeding syndrome

A

Oedema

Tachycardia (torsades de points – ventricular tachycardia)

Seizures

Ataxia

Rhabdomyolysis

377
Q

How is refeeding syndrome managed?

A

Electrolyte and vitamin supplementation continued for at least 10 days

378
Q

Define Marasmus malnutrition

A

Describes lack of calories and a discrepancy between height and weight

379
Q

Give 4 clinical features of Marasmus

A

Profound muscle wasting (atrophy)

Loss of subcutaneous fat

Thin dry skin

No oedema

380
Q

What disease is associated with marasmus malnutrition?

A

HIV

381
Q

What 4 defects are present in tetralogy of Fallot?

A

Pulmonary stenosis (right ventricular outflow tract obstruction)

Right ventricular hypertrophy

Ventricular Septal Defect

Overriding aorta (aorta is displaced over the VSD(r

382
Q

Give 4 risk factors for tetralogy of fallot

A

DiGeorge syndrome (deletion in chromosome 22)

Down’s syndrome (trisomy 21)

Maternal alcohol consumption during pregnancy

Maternal diabetes

383
Q

Give 4 clinical features of tetralogy of fallot

A

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)

384
Q

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

A

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

385
Q

What test is used to confirm tetralogy of fallot?

A

Echocardiography

386
Q

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

A

1st line - Manoeuvres to increase systemic venous return (arms and knees to chest)

2nd line - Propranolol (beta blocker)

3rd line - Phenylephrine (alpha receptor agonist)

387
Q

Define cyanotic congenital heart disease

A

Describes any heard defect that reduces the amount of oxygen delivered to the body.

Typically characterised by a right ot left shunt

388
Q

Give 5 examples of cyanotic congenital heart diseases

A

Tetralogy of Fallot

Transposition of great vessels

Tricuspic valve atresia

Ebstein anomaly

Persistent truncus arteriosus (DiGeorge syndrome)

389
Q

Define acyanotic congenital heart disease

A

Describes congenital cardiac malformations that affect the atrial or ventricular walls, heart valves or large blood vessels.

Characterised by a left to right shunt

390
Q

Give 2 clinical features caused by a left to right shunt

A

Pulmonary hypertension

Right ventricular hypertrophy

391
Q

Give 5 examples of acyanotic congenital heart defects

A

Ventricular Septal Defect (VSD)

Atrial Septal Defect (ASD)

Patent foramen ovale (PFO)

Patent ductus arteriosus (PDA)

Coarctation of the aorta

392
Q

Define transposition of the great vessels

A

Anatomical reversal of the aorta and pulmonary artery

393
Q

Give 2 risk factors for Transposition of the Great Arteries

A

Maternal diabetes (most common)

Di George Syndrome

394
Q

What can make transposition of the great vessles less fatal?

A

Mixing of blood occurring via either;

Intracardiac shunt (PFO, VSD, ASD) or

Extracardiac connection (PDA)

395
Q

Give 3 clinical features of Transposition of the Great Arteries

A

Postnatal cyanosis

Tachypnoea

‘Egg-on side’ appearance on chest x-ray

396
Q

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

A

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

397
Q

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

A

TOGA - Early onset (first few days)

ToF - Later onset (1-2 months)

398
Q

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

A

Alprostadil (prostaglandin E1)

Used to prevent closure of the Ductus Arteriosus

399
Q

Define tricuspid valve atresia

A

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

400
Q

What 2 echocardiogram findings are present in Tricuspid Atresia?

A

Right Ventricular hypoplasia

Right Atrial dilation (due to volume overload)

401
Q

Give 3 clinical features of Tricuspid Atresia

A

Central cyanosis (within days of birth(

Tachypnea

Holosystolic murmur (at lower left sternal border)

402
Q

What are more common VSDs or ASDs?

A

VSDs

403
Q

Define Ebstein anomaly

A

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

404
Q

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

A

In-utero Lithium exposure

405
Q

Give 2 other cardiac defects associted with Ebstein Anomaly

A

Interatrial communication (ASD) (90%)

Conduction disorders (WPW, supraventricular tachycardia)

406
Q

Give 3 clinical features of Ebstein Anomaly

A

Cyanosis

Right bundle branch block

Tricuspid regurgitation (Pansystolic murmur -worse on inspiration)

407
Q

Define Persistent Truncus Arteriosus

A

Describes underdevelopment of the aorticopulmonary septum.

Truncus arteriosus doesn’t divide resulting in a single trunk that receives output from both ventricles.

408
Q

What is the truncus arteriosus?

A

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

409
Q

What syndrome is associated with Persistent Truncus Arteriosus?

A

DiGeorge Syndrome

410
Q

What determines the severity of Persistent Truncus arteriosus?

A

Pulmonary Vascular Resistance (PVR)

411
Q

Define pulmonary vascular resistance (PVR)

A

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

412
Q

How is Pulmonary Vascular Resistance Calculated?

A

PVR = (mPAP-PAWP)/Cardiac output

mPAP - Mean pulmonary artery pressure
PAWP - Pulmonary Artery Wedge Pressure

413
Q

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

A

Woods units

> 2 is considered abnormal

414
Q

DiGeorge syndrome occurs due to a deletion in which chromosome?

A

Chromosome 22

415
Q

Define DiGeorge Syndrome

A

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

416
Q

Give 5 clinical features of DiGeorge Syndrome

A

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)

417
Q

Give 3 features of hypocalcemia

A

Tetany;
Cramps
Spasms
Hyporeflexia

418
Q

Give 3 investigation results seen in DiGeorge Syndrome

A

Decreased Parathyroid Hormone

Decreased Calcium

Decreased T-lymphocyte count

419
Q

What 2 signs are positive in hypocalcemia?

A

Trousseau sign (spasm of hand)

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

420
Q

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

A

No thymic shadow

421
Q

What is the most common congenital heart defect?

A

Ventricular Septal Defect

422
Q

Give 3 risk factors for VSD

A

Downs, Pataus and Edwards Syndrome

Intrauterine infections (TORCH)

Maternal diabetes, obesity and smoking

423
Q

Define TORCH (maternal intrauterine infections)

A

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

424
Q

Give 6 clinical features of VSD (post-natal)

A

Failure to thrive

Features of heart failure;

Hepatomegaly

Tachypnoea

Tachycardia

Pallor

Pan-systolic murmur

425
Q

Give 6 clinical features of VSD

A

Failure to thrive

Features of heart failure;

Hepatomegaly

Tachypnea

Tachycardia

Pallor

Pan-systolic murmur

426
Q

What shunt is seen in VSD?

A

Left to right shunt

427
Q

Give 5 complications of VSD

A

Aortic regurgitation

Infective endocarditis

Eisenmengers complex

Right sided heart failure

Pulmonary hypertension

428
Q

What is Eisenmenger’s syndrome?

A

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)

429
Q

What is the most common cause of infective endocarditis?

A

Staphylococcus aureus

430
Q

Give 5 clinical features of infective endocarditis

A

Janeway lesions

Oslers nodes

Splinter haemorrhages

Fever, nightsweats, weightloss

Cardiac murmur

431
Q

What valve is most commonly affected by infective endocarditis?

A

Mitral valve

432
Q

Give 3 risk factors for ASD

A

Down Syndrome

Fetal alcohol syndrome

Holt-Oram syndrome (skeletal abnormalities in hands and arms)

433
Q

Name 2 types of ASD

A

Ostium Secundum (70%) (Holt-Oram syndrome)

Ostium Primum (abnormal AV valves)

434
Q

Give 3 clinical features of ASD

A

Asymptomatic (small defect)

Ejection systolic murmur

Widely split second heart sound (S2)

435
Q

What causes the 2nd heart sound (S2)?

A

Closure of pulmonary and aortic valves

436
Q

What causes the 1st heart sound (S1)?

A

Closure of mitral and tricuspid valves

437
Q

Give 2 complications of ASD

A

Paradoxical embolism (increases risk of ischemic stroke)

Heart failure

438
Q

The Ductus Arteriosus is a connection between which vessels?

A

Pulmonary trunk and descending aorta

439
Q

When and why does the ductus ateriosus close?

A

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

440
Q

Give 6 risk factors for PDA

A

Prematurity

Maternal Rubella (during 1st trimester)

Maternal Alcohol consumption

Maternal phenytoin use

Maternal prostaglandin use

Downs syndrome

441
Q

What shunt is seen in PDA?

A

Left to right shunt (acyanotic)

442
Q

Give 5 clinical examination findings of PDA

A

Left subclavicular thrill

Continuous machinery murmur (left sternal border)

Large volume, bounding collapsing pulse

Wide pulse pressure

Heaving apex beat

443
Q

How is PDA managed in neonates?

A

Indomethacin or Ibuprofen (inhibit prostaglandin synthesis)

444
Q

What causes the Ductus Arteriosus to remain patent?

A

Prostaglandin E2 production by the placenta

445
Q

What is the MOA of indomethacin?

A

COX1/2 inhibitor

446
Q

Give 1 contraindication for indomethacin use

A

GI disease (haemorrhage, ulceration, perforation ect)

447
Q

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

A

Chlorothiazide

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

448
Q

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

A

Paracetamol

449
Q

Where is the foramen ovale?

A

Hole between left and right atria

450
Q

What condition is associated with PAtent Foramen Ovale?

A

Loeys-Dietz syndrome (connective tissue disorder)

451
Q

Define coarctation of the aorta

A

Congenital narrowing of the descending aorta

452
Q

Give 4 conditions associated with coarctation of the aorta

A

Turners syndrome

Bicuspid aortic valve

Berry aneurysms

Neurofibromatosis

453
Q

Give 4 clinical features of coarctation of the aorta

A

Shock (neonates)

Hypertension at young age (resistant to treatment)

Delayed brachia -femoral pulse

Ejection sytolic murmur

454
Q

Give 3 risk factors for coarctation of the aorta

A

Male

Turners syndrome

DiGeorge syndrome

455
Q

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

A

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

456
Q

What condition is associated with dextrocardia?

A

Kartagner’s syndrome

457
Q

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

A

Primary ciliary dyskinesia (absent or dysmotile cilia)

Defect in dynein arm of microtubules

458
Q

Give 4 clinical features of Kartagner syndrome

A

Recurrent otitis media, sinusitis and nasal polyps

Bronchiectasis

Dextrocardia/situ iversus

Subfertility (deminished sperm motility and defective ciliary action in fallopian tubes)

459
Q

Define febrile convulsion

A

Seizure provoked by fever in otherwise normal children

460
Q

Between what ages do febrile convulsions tend to occur?

A

6 months and 5 years old

461
Q

Give 3 clinical features of febrile convusions

A

Usually occur early in a viral infection (as temperature rises) i.e otitis media

Brief seizures (<5 mins)

Tonic clonic seizures

462
Q

Define simple febrile convulsion (4)

A

<15 minutes

Generalised seizure

No recurrence in 24 hours

Should recover in an hour

463
Q

Recurrent febrile convulsions be managed? (2)

A

Rectal diazepam or buccal midazolam

464
Q

Define complex febrile seizure

A

15-30 minutes

Focal seizure

May have repeat seizures within 24 hours

465
Q

Define biliary atresia. When does it present?

A

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

466
Q

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

A

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)

467
Q

What 3 structures are found in the porta hepatis?

A

Hepatic portal vein

Hepatic artery proper

Common bile duct

468
Q

Describe the pathophysiology of biliary atresia

A

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

469
Q

Give 5 clinical features of biliary atresia

A

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

470
Q

How is biliary atresisa diagnosed?

A

Cholangiography (fails to show biliary tree)

471
Q

What 3 laboratory findings may be seen in biliary atresia?

A

Elevated Conjugated bilirubin

Elevated AST/ALP

Elevated GGT

472
Q

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

A

Absence of gall bladder

No dilatation of biliary tree

473
Q

Define volvulus

A

Describes twisting of a loop of bowel on it’s mesentery (common cause of intestinal obstruction)

Most commonly presents as midgut volvulus

474
Q

What is the mesentery?

A

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

475
Q

Describe normal intestinal rotation

A

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.

476
Q

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

A

270 degrees rotation

477
Q

What are the 2 types of intestinal malrotation?

A

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)

478
Q

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

A

Bilious vomiting (thick, green vomit)

Abdominal distension

Absent bowel sounds

479
Q

Give 4 conditions associated with intestinal malrotation

A

Exomphalos (organs herniate through umbilicus into hernial sac)

Congenital diaphragmatic hernia

Intrinsic duodenal atresia

Congenital heart defects

480
Q

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

A

Upper GI contrast study (gold standard)

Ultrasound study

481
Q

Describe the initial resuscitation management of intestinal malrotation (4)

A

NG tube

IV fluids

Correction of electrolyte imbalance

Broad Spectrum Antibiotics

482
Q

How is intestinal malrotation managed?

A

Laparotomy (if volvulus present)

Ladd’s procedure (division of Ladd bands)

483
Q

Define intussusception

A

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

484
Q

Where in the bowel does intussusception commonly occur?

A

Ileo-caecal region

485
Q

Give 5 clinical features of intussusception

A

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)

486
Q

What investigation is used to confirm intussusception?

A

Abdominal Ultrasound

487
Q

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

A

Abdominal X-ray

488
Q

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

A

Target like mass

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

489
Q

How is intussusception managed? (3)

A

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)

490
Q

Describe features of small bowel obstruction (4)

A

Abdominal pain – Colicky

Vomiting and/or nausea – Early onset, Large volume of bilious vomit

Constipation – late onset

Abdominal distension – Less severe than LBO

491
Q

Describe features of large bowel obstruction (4)

A

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

492
Q

Define pyloric stenosis. When does it typically present?

A

Typically presents in 1st 4 weeks of life

Describes idiopathic hypertophy and hyperplasia of pyloric sphincter muscles, making it difficult to pass food through

493
Q

Give 3 risk factors for pyloric stenosis

A

Exposure to nicotine during pregnancy

Macrolide antibiotics - Erythromycin/Azithromycin (esp during 1st 2 weeks of life)

Bottle feeding

494
Q

Give 3 clinical features of pyloric stenosis

A

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

495
Q

How is pyloric stenosis diagnosed?

A

Clinical - if palpable signs are present

If not - Ultrasound confirms diagnosis

496
Q

How is pyloric stenosis managed?

A

Ramstedt pyloromyotomy (muscle splitting incision of pyloric sphincter)

497
Q

Define oesophageal atresia

A

Describes abnormal development of tracheoesophageal septum.

Congenital defect in which upper oesophagus does not connect to lower oesophagus.

Manifests immediately after birth

498
Q

What is the most common type of oesophageal atresia?

A

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

499
Q

Give 3 clinical features of oesophageal atresia

A

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

500
Q

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

A

Aspiration pneumonia 2nd to tracheoesophageal fistula.

Presents with;

Coughing
Rales (discontinuous rattling on auscultation)
Cyanotic attacks

501
Q

How is oesophageal atresia diagnosed?

A

NG tube placement - Tube cannot pass through oesophagus

Abdominal/Thorax x-ray - Shows oesophageal pouch

502
Q

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

A

VACTERL anomalies

Vertebral
Anal
Cardiac
Tracheoesophageal fistula
Renal
Limb

Occurs due to defect in embryonic mesoderm

503
Q

How is oesophageal atresia managed? (2)

A

Surgery within 1st 24 hours to reconnect upper and lower oesophagus

Gastrostomy tube - to allow enteral feeding

504
Q

What is the most common nephrotic syndrome in children?

A

Minimal change disease

505
Q

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

A

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

506
Q

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

A

Defined by haematuria due to inflammatory damage

Characterised by;

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

507
Q

What triad is classically seen in nephrotic syndrome?

A

Proteinuria
Hypoalbuminaemia
Oedema

508
Q

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

A

Minimal change disease (most common)

Focal segmental glomerulosclerosis

Membranous nephropathy

509
Q

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

A

Hodgkin’s lymphoma

Leukaemia

Drugs - NSAIDs/Lithium

Hepatitis B/C

510
Q

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

A

Renal biopsy + Electron microscopy

Shows effacement (shortening/thinning) of podocyte foot processes

511
Q

Describe the management of minimal change disease

A

Initial treatment - Prednisolone

Infrequent relapses;
- Increase dose of prednisolone or
- Calcineurin inhibitor (Ciclosporin or Tacrolimius)

Frequent relapses
- DMARD - Mycophenolate mofetil

512
Q

How does Nephritic syndrome typically present? (4)

A

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

513
Q

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

A

IgA nephropathy (most common)

Good pastures (Anti-GBM)

Post streptococcal (cresenteric)

SLE

514
Q

How does IgA nephropathy typically present?

A

Presents with macroscopic haematuria following a respiratory infection

515
Q

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

A

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.

516
Q

Define goodpastures syndrome

A

Now called Anti-glomerular basement membrane disease (GBM)

Describes a rare type of small vessel vasculitis associated with both pulmonary haemorrhage and progressive glomerulonephritis

517
Q

What causes goodpastures syndrome?

A

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

518
Q

How is goodpastures diagnosed? (2)

A

Renal biopsy - Showing IgG deposits along basement membrane

Antiglomerular basement membrane antibody (Anti-GMB confirms diagnosis)

519
Q

How is goodpastures syndrome managed? (3)

A

Prednisolone
Plasmapheresis
Cyclophosphamide

520
Q

How may goodpastures syndrome present? (3)

A

Haemoptysis (due to pulmonary haemorrhage)

Reduced urine output (AKI)

Oedema

521
Q

What bacterium causes Whooping cough?

A

Bordetella pertussis (gram negative)

522
Q

When are infants given vaccinations for whooping cough?

A

2 months, 3 months, 4 months and 3.5 years

523
Q

How long is the incubation period for whooping cough?

A

5-10 days (maximum 21)

524
Q

What criteria would make you suspect whooping cough?

A

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

525
Q

Give 4 clinical features of whooping cough

A

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

526
Q

How is whooping cough diagnosed? (2)

A

Nasal sawb for Bordetella pertussis

PCR and serology (increasingly used now due to availability)

527
Q

How is whooping cough managed? (3)

A

Oral macrolide - Clarithromycin, azithromycin, erythromycin

Household contacts offered antibiotic prophylaxis

Notify pyblic health england

528
Q

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

A

48 hours after commencing antibiotics

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

529
Q

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

A

Meckel’s diverticulum

530
Q

Define Meckel’s diverticulum

A

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

531
Q

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

A

Vitelline duct (omphalomesenteric duct)

532
Q

What mucosa can be present in Meckel’s diverticulum?

A

Ectopic ileal, gastric or pancreatic mucosa

533
Q

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

A

Occurs in 2% of the population

Is 2 feet from the ileocaecal valve

Is 2 inches long

534
Q

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

A

Right Lower Quadrant Pain (mimicing appendicitis)

Rectal bleeding

Intestinal obstruction 2nd to omphalomesenteric band, volvulus and intussusception)

535
Q

Define androgen insensitivty syndrome

A

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

536
Q

What is the pattern of inheritance in androgen insensitivity syndrome?

A

X-linked recessive (46XY)

537
Q

Give 3 clinical features of androgen insensitivity syndrome

A

Primary amenorrhoea

Undescended testes causing groin swellings

Delayed puberty (no pubic hair, small breasts, slim build)

538
Q

How is androgen insensitivity syndrome diagnosed? (2)

A

Buccal smear or chromosomal analysis (46XY genotype)

539
Q

Define Kallman’s syndrome

A

Recognised cause of delayed puberty secondary to hypogonadotrophic hypogonadism.

Occurs due to failure of GnRH secreting neurones to migrate to the hypothalamus

540
Q

Give 4 clinical features of Kallman’s syndrome

A

Delayed puberty (male)

Ansomia (lack of smell)

Hypogonadism, cryptorchidism

Cleft lip/palate and hearing/visual defects (rare)

541
Q

What is the pattern of inheritance for Kallman Syndrome?

A

X-linked recessive

542
Q

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

A

Klinefelters - High LH, Low Testosterone

Kallman’s - Low LH, Low Testosterone

Androgen insensitivity - High LH, Normal/High testosterone

543
Q

What karyotype is Klinefelter’s syndrome associated with?

A

47, XXY

544
Q

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

A

Taller than average

Lack secondary sexual characteristics

Small, firm testes

Gynecomastia (increased incidence of breast cancer)

Infertility

545
Q

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

A

HPV (Human papilloma virus)

546
Q

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

A

Dilated cardiomyopathy

547
Q

Give 5 clinical features of otitis media

A

Acute onset of;

Fever
Irritability
Vomiting
Anorexia
Previous viral upper respiratory infection

548
Q

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

A

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

549
Q

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

A

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)

550
Q

Give 3 intracranial complications of otitis media

A

Meningitis (headache, vomiting, photophobia, phonophobia)

Sigmoid sinus thrombosis (sepsis, swinging pyrexia, meningitis)

Brain abscess (sepsis and neuro signs - CN compression)

551
Q

Give 4 extra-cranial complications of otitis media

A

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)

552
Q

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

A

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

553
Q

How may labyrinthitis present in otitis media? (4)

A

Vertigo

Nausea

Vomiting

Imbalance

554
Q

What is the 1st line treatment for neonatal sepsis?

A

Benzylpenicillin + Gentamycin

555
Q

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

A

Pertussis (whooping cough) + Influenza

556
Q

What virus commonly causes Hand, Foot and Mouth Disease?

A

Coxsackie A16

557
Q

What is the most common cause of vomiting in infancy?

A

GORD (Gastro-oesophageal Reflux Disease)

558
Q

Give 2 clinical features of GORD in children

A

Typically develops before 8 weeks

Vomiting/regurgitation following feeds

559
Q

How is GORD managed in children?

A

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)

560
Q

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

A

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

561
Q

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

A

Trnsient Tachypnoea of the newborn

562
Q

What is a common risk factor for TTN?

A

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

563
Q

How it TTN diagnosed? What may the investigation show?

A

Chest X-ray

May show;
Hyperinflation of the lungs
Fluid filled horizontal fissure

564
Q

How is TTN managed?

A

Observation and supportive care (symptoms usually resolve in 1-2 days)

Supplementary O2 may be required to maintain sats

565
Q

How is neonatal sepsis categorised?

A

Early onset sepsis (within first 72 hours of birth)

Late onset sepsis (7-28 days of life)

566
Q

What are the 2 most common causes of neonatal sepsis?

A

Group B streptococcus

E.coli

567
Q

What is the most common cause of early onset sepsis?

A

Group B Streptococcus

568
Q

What is the most common cause of late onset sepsis?

A

Environmental pathogens;

Staphylococcus epidermidis (coagulase negative)

569
Q

Give 3 risk factors for neonatal sepsis

A

Prematurity ( <37weeks)

Low birth weight (<2.5kg)

Maternal GBS infection

570
Q

Give 6 clinical features of neonatal sepsis

A

Respiratory distress (grunting, nasal flaring, tachypnoea)

Tachycardia

Apnoea

Jaundice

Seizures

Poor-feeding, abdominal distension and/or vomiting

Features tend to be non-specific

571
Q

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

A

Respiratory distress (grunting ect)

572
Q

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

A

Blood culture (establish diagnosis)

FBC (neutrophilia and neutropenia)

ABG (metabolic acidosis)

573
Q

How is neontal sepsis managed?

A

1st line - IV Benzylpenicillin + Gentamicin

Remeasure CRP 18-24 hours after presentation

574
Q

When can antibiotics be stopped in neonatal sepsis?

A

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

575
Q

Give 5 features of cows milk intolerance

A

Regurgitation and vomiting

Diarrhoea

Urticaria (rash) and atopic eczema

Colic symptoms (irritability/crying)

Wheeze, chronic cough

576
Q

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

A

Clinical diagnosis (improvement with cow’s milk elimination)

Skin prick/patch test
Total IgE and specific IgE (RAST) for cows milk protein

577
Q

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

A

If failing to thrive - refer to paediatrician

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

578
Q

How is cows milk intolerance managed if breast fed?

A

Continue breastfeeding
Eliminate cows milk from maternal diet

579
Q

What is the prognosis for cows milk intolerance

A

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.

580
Q

Is cows milk intolerance IgE mediated or non-mediated?

A

Both

581
Q

Give 3 characteristics of hypospadias

A

Ventral urethral meatus

Hooded prepuce

Chordee (ventral curvature of penis)

582
Q

What condition is commonly associated with hypospadias?

A

Cryptorchidism (undescended testes)

583
Q

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

A

Circumcision (as foreskin may be used in corrective procedure)

584
Q

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

A

Refer urgently for same-day assessment

585
Q

What type of vaccine is the MMR jab?

A

Live attenuated

586
Q

How is biliary atresisa diagnosed?

A

Cholangiography (fails to show biliary tree)

587
Q

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

A

Influenza vaccine

588
Q

Define Potter’s Syndrome

A

Describes the typical physical appearance caused by pressure in utero caused by oligohydraminos.

Occurs due to bilateral renal agenesis

589
Q

Why does bilateral renal agenesis result in Potter syndrome?

A

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

590
Q

How may necrotising enterocolitis present? (4)

A

Feeding intolerance

Abdominal distension

Bloody stools

Bilious vomiting

591
Q

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

A

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)

592
Q

How is necrotising enterocolits managed? (4)

A

Laparotomy (initially)

Stop oral feeds

Barrier nurse

Give antibiotics - Cefotaxime and vancomycin

593
Q

What antibiotics are given in the management of necrotising enteroclolitis?

A

Cefotaxime and vancomycin

594
Q

How are mitochondrial diseases inherited?

A

Maternal inheritance

Non of children in an affected male will inherit disease

All of children of an affected female will inherit disease

595
Q

Define Immune Thrombocytopenia (ITP)

A

Describes an immune mediated reduction in platelet count (thrombocytopenia)

Typically follows infection or vaccination

596
Q

Give 3 clinical features of ITP

A

Bruising

Petechial or purpuric rash

Epistaxis or gingival bleeding (less common)

597
Q

How is achondroplasia (dwarfism) inherited?

A

Autosomal dominant

598
Q

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

A

1/4 - unaffected

1/2 - affected heterozygous

1/4 - affected homozygous

599
Q

Define Duchene muscular dystrophy

A

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

600
Q

Give 4 clinical features of Duchenne muscular dystrophy

A

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%)

601
Q

What investigation is used to diagnosed Duchenne muscular dystrophy?

A

Genetic testing

602
Q

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

A

Creatinine Kinase

603
Q

When can children with scarlet fever return to school?

A

24 hours after commencing antibiotics

604
Q

When can children with measles return to school?

A

4 days from onset of rash

605
Q

When can children with rubella return to school?

A

5 days from onset of rash

606
Q

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

A

Radial

Humeral

Femoral

607
Q

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

A

Rhesus haemolytic disease

ABO haemolytic disease

Hereditary spherocytosis

Glucose-6-dehydrogenase

608
Q

What is the most common cause of childhood hypothyroidism?

A

Autoimmune thyroiditis

609
Q

How is constipation diagnosed in a child <1/

A

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

610
Q

How is constipation diagnosed in a child >1?

A

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

611
Q

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

A

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

612
Q

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

A

Faecal impaction

613
Q

Give 3 factors that suggest faecal impaction

A

Symptoms of severe constipation

Overflow soiling

Faecal mass palpable in the abdomen

614
Q

What is encopresis and how does it occur?

A

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

615
Q

How is faecal impactation managed? (2)

A

1st line - Macrogol (using escalating dose regimen

2nd line - Senna (Stimulant laxative)

616
Q

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

A

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)

617
Q

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

A

1st line - Macrogol (escalating dose regimen)
(if child required disimpactation, dose is HALF disimpactation dose)

2nd line - Senna (stimulant laxative)

618
Q

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

A

Reduce dose of laxative (as can lead to hypokalemia)

619
Q

Name 4 types of laxatives

A

Bulk-forming laxatives (ispaghula husk and methylcellulose)

Osmotic laxatives (lactulose and macrogol)

Stimulant laxatives (senna and sodium picosulfate)

Stool softener laxatives (docusate sodium)

620
Q

Name 2 bulk forming laxatives and describe how they work

A

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

621
Q

Name 2 osmotic laxatives and describe how they work

A

Lactulose and Macrogol

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

622
Q

Name 2 stimulant laxatives and describe how they work

A

Senna and sodium picosulfate

Work by speeding up movement of bowels by stimulating contraction of the muscle lining of digestive tract

623
Q

Name 1 stool softener laxative and describe how it works

A

Docusate sodium

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

624
Q

What is the management of bacterial meningitis? (2)

A

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

> 3 months - IV cefotaxime (or ceftriaxone)

625
Q

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

A

6-8 months

626
Q

Define impetigo

A

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

627
Q

Name 3 types of impetigo

A

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

Bullous impetigo - Staphylococcus aureus

MRSA caused impetigo

628
Q

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

A

Thin walled vesicles/pustules which release exudate forming a golden/brown crust

May be pruritis (itchy)

Negative Nikolsky sign

629
Q

What is Nikolsky sign?

A

Sign when top layers of skin slip away from bottom layers when rubbed.

Skin rubs off leaving a red raw base

630
Q

How is impetigo spread?

A

Via direct contact with discharges from scabs o an infected person

631
Q

What is the incubation period of impetigo?

A

4-10 days

632
Q

Where on the body does impetigo tend to manifest?

A

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

633
Q

How is impetigo diagnosed? (2)

A

Usually clinical

Swabs (of exudate from moist lesion) for culture

634
Q

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

A

Acute glomerulonephritis (following streptococcal impetigo)

Cellulitis

Sepsis

Scarlet fever, urticaria and erythema multiforme (following streptococcal infection)

635
Q

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

A

Until lesions have healed, dried or crusted over

Or 48 hours after starting antibiotics

636
Q

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

A

1st line Hydrogen peroxide 1% cream (for patients not systemically unwell)

2nd line - Short course of topical antibiotic (Fusidic acid or Mupirocin)

637
Q

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

A

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)

638
Q

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

A

Oral antibiotics;

1st line - Oral flucloxacillin

2nd line - Clarithromycin/Erythromycin (if penicillin allergy)

639
Q

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

A

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)

640
Q

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

A

Mammalian diving reflex

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

641
Q

What nervous system mediates laryngospasm in drowning?

A

Parasympathetic

642
Q

Describe infectious mononucleosis. Mention triad of symptoms

A

AKA glandular fever, infectious mononucleosis is an acute condition caused by Epstein-Barr Virus.

Present with a triad of;

Sore throat

Lymphadenopathy

Pyrexia/fever

643
Q

What pathogen causes infectious mononucleosis?

A

Epstein Barr Virus

644
Q

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

A

IM - Lymphadenopathy occurs in the anterior and posterior triangles of the neck

Tonsillitis - Typically only occurs in the upper anterior cervical chain

645
Q

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

A

Ampicillin/amoxicillin

646
Q

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

A

Lymphocytosis

Haemolytic anaemia (2nd to cold agglutins IgM)

Hepatitis (transient rise in ALT)

647
Q

What tests are used to diagnose infectious mononucleosis?

A

Monospot test + FBC in 2nd week of illnss

648
Q

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

A

Diagnoses - Infectious mononucleosis

Detects - Heterophile antibodies produced in response to EBV infection.

649
Q

Give one alternative name for EBV

A

Human herpesvirus 4 - HHV-4

650
Q

Define haemolytic uraemic syndrome

A

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

651
Q

What triad is typically seen in Haemolytic Uraemic syndrome?

A

Acute kidney injury

Microangiopathic haemolytic anaemia (normocytic anaemia)

Thrombocytopenia

652
Q

What type of aneamia is seen in microangiopathic haemolytic anaemia?

A

Normocytic anaemia

653
Q

Give 4 clinical features of haemolytic uraemic syndrome

A

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

654
Q

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

A

Shiga toxin producing E.coli (most common)

Pneumococcal infection

HIV

655
Q

What is the main cause of primary haemolytic uraemic syndrome?

A

Compliment dysregulation

656
Q

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

A

FBC - (Anaemia, Increased reticulocytes and schistocytes)

Coagulation profile (decreased platelets)

WCC - Increased WBCs

U&Es - Increased creatinine

Urinalysis (haematuria and proteinuria - AKI)

657
Q

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

A

Oral Ciprofloxacin

658
Q

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

A

Hib/MenC + MMR + PCV + Men B

659
Q

What vaccines are given at 2 months?

A

6-1 vaccine

Oral rotavirus vaccine

Men B

660
Q

What vaccines are given at 3 months?

A

6-1 vaccine

Oral rotavirus vaccine

PCV (pneumococcal)

661
Q

What vaccines are given at 4 months?

A

6-1 vaccine

Men B

662
Q

What vaccines are given at 3-4 years?

A

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

MMR

663
Q

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

A

Diphtheria

Tetanus

Whooping cough

Polio

664
Q

Describe the management of chicken pox (3)

A

School exclusion (until vesicles have crusted over - usually 5 days after onset of rash)

Calamine lotion

Immunocompromised - Varricella zoster immunoglobulin

665
Q

Describe 2 symptoms of chicken pox

A

Fever

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

666
Q

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

A

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)

667
Q

Name 4 drugs that can cause Steven Johnson Syndrome

A

Allopurinol

Penicillin

Lamotrigine

Phenytoin

668
Q

Give 4 complications of chickenpox

A

Bacterial superinfection

Cerebellitis

DIC

Progressive disseminated disease

669
Q

How may bacterial meningitis appear on LP?

A

Turbid appearance, raised polymorphs, raised protein, low glucose

670
Q

How may viral meningitis appear on LP?

A

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

671
Q

How may TB meningitis appear on LP?

A

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

672
Q

How may Encephalitis appear on LP?

A

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

673
Q

How is estimated weight calculated for children

A

Estimated weight = (age+4)x2

674
Q

What ml of bolus administered to a child in shock?

A

20m/kg

Caveat - In DKA or HF give 10ml/kg

675
Q

How is hourly rate of fluid resuscitation calculated in children?

A

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

676
Q

Define neonatal hypoglycaemia

A

Blood glucose <2.6mmol/L

677
Q

Give 2 features of neonatal hypoglycaemia

A

Autonomic features; Jitteriness, irritability, pallor, tachypnoea

Neuroglycopenic symptoms; poor feeding/sucking, weak cry, drowsy, seizures

678
Q

How is asymptomatic hypoglycaemia managed? (2)

A

Encourage normal feeding (breast or bottle)

Monitor blood glucose

679
Q

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

A

Admit to the neonatal unit

IV infusion of 10% dextrose

680
Q

Define ophthalmia neonatorum

A

Infection of the newborn eye

681
Q

What 2 organisms commonly cause Ophthalmia neonatorum

A

Chlamydia trachomatis

Neisseria gonorrhoeae

682
Q

How may ophthalmia neonatorum present? (3)

A

Neonate presenting with history of ‘sticky eyes’

Bilateral purulent discharge

Conjunctival injection

683
Q

How should ophthalmia neonatorum be managed?

A

Same day referral to ophthalmology/paediatric assessment

684
Q

Give 4 neonatal characteristics of maternal Rubella Infection

A

Sensorineural deafness

Congenital Cataracts

Congenital heart disease (PDA)

Glaucoma

685
Q

Give 3 neonatal characteristics of maternal Toxoplasmosis infection

A

Cerebral calcification

Chorioretinitis (inflammation of choroid in eye)

Hydrocephalus

686
Q

Give 4 neonatal characteristics of maternal cytomegalovirus infection

A

Low birth weight

Purpuric skin lesions

Sensorineural deafness

Microcephaly

687
Q

Roseola infantum is caused by what pathogen?

A

Human Herpes Virus 6 (HHV6)

688
Q

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

A

5-15 days

Affects children aged 6 months to 2 years

689
Q

Give 5 features of Roseola infantum

A

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

690
Q

What lice causes headlice?

A

Pediculosis capitis

691
Q

How long does it take head lice eggs to hatch?

A

7-10 days

692
Q

How are headlice spread?

A

Direct head to head contact

693
Q

How are headlice diagnosed?

A

Fine-toothed combing of wet or dry hair

694
Q

Give 4 treatments of headlice

A

Malathion

Wet combing

Dimeticone

Isopropyl myristate

695
Q

What organism commonly causes meningitis in neonate - 3 months

A

Group B Streptococcus

696
Q

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

A

Neisseria Meningitidis

Streptococcus pneumoniae

Haemophilus influenzae

697
Q

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

A

Neisseria meningitidis

Streptococcus pneumoniae

698
Q

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

A

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

More common in females.

699
Q

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

A

Thelarche

700
Q

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

A

Adrenarche

701
Q

Name and describe 2 classifications for precocious puberty

A

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)

702
Q

What may bilateral enlargement of testes and precocious puberty suggest ?

A

Gonadotrophin release from an intracranial tumour

703
Q

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

A

Gonadal tumour

704
Q

What may small testes and precocious puberty suggest?

A

Adrenal cause (tumour or adrenal hyperplasia)

Phase 3a (6 decks)

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