Paediatrics Flashcards
1
Q
Presentation of left to right shunt
A
Dyspnoea
Acyanotic
2
Q
Causes of left to right shunt
A
Ventricular septal defect
Patent ductus arteriosus
Atrial septal defect
3
Q
Presentation of right to left shunts
A
Cyanotic (blue)
4
Q
Causes of right to left shunts
A
6Ts:
Tetralogy of Fallot
Transposition of great arteries
Truncus arteriosus
Total anomalous pulmonary venous connection
Tricuspid valve abnormalities
Ton of others: hypoplastic left heart, double outlet right ventricle, pulmonary atresia
5
Q
Causes of cardiac outflow obstruction in a well child (asymptomatic with murmur)
A
Pulmonary stenosis
Aortic stenosis
6
Q
Cause of outflow obstruction in a sick neonate (collapsed with shock)
A
Coarctation of the aorta
7
Q
Presentation of VSD
A
Poor feeding
Failure to thrive
Dyspnoea/SOB
Tachycardia
Tachypnoea
Heart failure - may have hepatomegaly
Pansystolic murmur at lower left sternal edge
Systolic thrill on palpation
8
Q
Conditions associated with VSD
A
Down’s syndrome
Turner’s syndrome
Foetal alcohol syndrome
9
Q
Circulation in the foetus
A
Low left atrial pressure, as little blood returns from lungs
Right atrium > as receives systemic venous return, including from placenta
Foramen ovale open: blood flows right atrium –> left atrium –> left ventricle –> body
Ductus arteriosus connects pulmonary artery and aorta
10
Q
Changes in circulation at birth
A
Breathing –> resistance to pulmonary blood falls –> increased volume of blood through lungs
–> increased left atrial pressure
Loss of placenta –> decreased venous return to right atrium –> decreased right atrial pressure
–> closure of foramen ovale
Ductus arteriosus closes in first few hours or days (1-2 days usually)
11
Q
What is duct-dependent circulation
A
Where babies with congenital heart lesions rely on blood flow through the ductus arteriosus (connecting pulmonary artery to aorta)
When the duct closes, condition deteriorates rapidly
12
Q
What features of a murmur lead you to believe it is not concerning
A
Innocent –> 5Ss
InnoSent = Soft, Systolic, aSymptomatic, left Sternal edge
13
Q
Management of VSD
A
Small: will close spontaneously
Moderate: diuretic therapy (furosemide and spironolactone), feeding with high caloric feeds
Large: as for moderate lesion, surgery before 12 months
14
Q
Findings on CXR for VSD
A
Severe VSD (heart failure): cardiomegaly, pulmonary oedema (increased pulmonary vascular markings)
Enlarged pulmonary artery
15
Q
Why should a large VSD be corrected before 12 months
A
Prevent persistent pulmonary hypertension of the newborn
16
Q
Why might a murmur be heard during febrile illness or anaemia
A
Increased cardiac output
Flow murmur
17
Q
What causes Eisenmenger syndrome
A
When pulmonary pressure increases so much in a left to right shunt lesion, that the shunt becomes right to left, and the patient becomes cyanotic
18
Q
Lesions that may result in Eisenmenger syndrome
A
Atrial septal defect
Ventricular septal defect
Patent ductus arteriosus
19
Q
Time of presentation of VSD
A
Antenatal diagnosis: 16-18 weeks
Presentation at 6-8 weeks old
Congestive heart failure typically presents after 4-6 weeks
20
Q
Presentation of ASD in childhood
A
Typically asymptomatic
Recurrent chest infections
SOB
Difficulty feeding
Poor weight gain
21
Q
Three types of ASD
A
Ostium secondum
Patent foramen ovale
Ostium primum
22
Q
What is ostium secondum
A
Type of ASD where the septum secondum fails to close fully, leaving a hole
23
Q
What is patent foramen ovale
A
Foramen ovale fails to close (not strictly an ASD)
24
Q
What is ostium primum
A
Septum primum fails to close fully, leaving a hole in the wall
Leads to AV valve defects –> atrioventricular septal defect
25
Causes of pansystolic murmurs
Ventricular septal defect
Mitral regurgitation
Tricuspid regurgitation
26
Complications of ASD
Stroke in venous thromoboembolism (e.g. patient with DVT)
Atrial fibrillation/flutter
Pulmonary hypertension
Right sided heart failure
Eisenmenger syndrome
27
Murmur of ASD
Mid-systolic
Crescendo-decrescendo murmur
Loudest at upper left sternal border
Fixed split second heart sound (does not change with inspiration or expiration)
28
Presentation of ASD in adults
Dyspnoea
Heart failure
Stroke
Arrhythmia
29
Why is there a fixed split of second heart sound in ASD?
Left to right shunt increases right ventricle filling
--> RV ejection time is increased --> pulmonary valve closure is delayed
30
Management of ASD
Small + asymptomatic --> watch and wait
Surgical management: transvenous catheter closure, or open heart surgery
Anticoagulants in adults
31
Causes of PDA
Rubella infection
Maternal warfarin therapy
Prematurity - very common
Born at high altitude
32
Presentation of PDA
SOB
Difficulty feeding
Poor weight gain
Lower respiratory tract infections
Usually presents 3-5 days after birth, when duct begins to close
33
Murmur/cardiac signs of PDA
Continuous crescendo-decrescendo, machinery murmur
Heard at upper-left sternal border - best heard below left clavicle
May have thrill
Second heart sound difficult to hear
Large volume, bounding, collapsing pulse
Heaving apex beat
Wide pulse pressure
34
Main investigations in congential cardiac conditions in neonate
Echocardiogram
ECG
CXR
35
Management of PDA
Typically monitored until 1 year of age
Preterm: likely to close spontaneously
Term: less likely to close spontaneously
Medical - indomethacin/ibuprofen (not effective in term infants) --> stimulates closure of PDA
Surgical - catheter closure or PDA ligation, weight >5kg
36
Components of Tetralogy of Fallot
Pulmonary stenosis
VSD
Overriding aorta
Right ventricular hypertrophy
37
Risk factors/causes of Tetralogy of Fallot
Foetal alcohol syndrome
Chromosome 22q11.2 deletion
Rubella infection
Increased age of mother (>40)
Diabetic mother
38
What is an overriding aorta in ToF?
Aortic valve placed further right than normal, above the VSD
Right ventricle contracts --> blood up into aorta --> deoxygenated blood enters aorta
39
Presentation of ToF
Presenting when patent ductus arteriosus begins to close
Cyanosis
Clubbing
Poor feeding
Sweating during feeds
Poor weight gain
Tet spells
40
What are 'tet spells'
Intermittent symptomatic periods where R to L shunt is worsened --> cyanotic episode
Occurs when pulmonary resistance increases, or systemic resistance decreases
41
What may cause a 'tet spell'
Waking
Physical exertion: CO2 vasodilator --> systemic vasodilation
Crying
42
Presentation of 'tet spell'
Irritable
Cyanotic
SOB
May squat/bring knees to chest --> increase systemic vascular resistance
Severe --> reduced consciousness, seizures, potential death
43
Murmur of ToF
Crescendo-decrescendo
Harsh ejection systolic quality
Loudest over upper-left sternal angle
Posterior radiation
Due to right ventricular outflow obstruction (pulmonary stenosis), not VSD
44
Management of 'tet spell'
Knees to chest position
Oxygen: for hypoxia
Morphine: decrease respiratory drive
Beta-blockers: relax RV
IV fluids: increase pre-load
Sodium bicarbonate: for metabolic acidosis
Phenylephrine infusion: increase SVR
45
Management of ToF
Neonates: prostaglandin infusion to maintain ductus arteriosus
Surgery: Blalock-Taussig shunt
46
What is Transposition of the great arteries?
Attachments of the aorta and the pulmonary trunk to the heart are swapped
RV pumps blood into aorta, LV pumps blood into pulmonary arteries
47
Conditions associated with ToGA
VSD
Coarctation of aorta
Pulmonary stenosis
48
Presentation of ToGA
Present as ductus arteriosus closes
Severe, life-threatening cyanosis
Poor feeding
Sweating during feeds
Tachypnoea
Single loud S2 audible
Prominent right ventricular impulse
49
Management of ToGA
VSD or PDA can allow some time for definitive treatment
Prostaglandin infusion to maintan ductus arteriosus
Balloon septostomy: catheter into foramen ovale, inflate balloon --> create large ASD
Arterial switch surgery
50
Finding of ToGA on CXR
'Egg on a string' appearance
51
Conditions associated with pulmonary valve stenosis
Tetralogy of Fallot
William syndrome
Noonan syndrome
Congenital rubella syndrome
52
Presentation of pulmonary valve stenosis
Often asymptomatic
If significant: symptoms of fatigue on exertion, SOB, dizziness + fainting
53
Signs of pulmonary valve stenosis
Ejection systolic murmur, loudest at pulmonary area
Palpable thrill in pulmonary area
Right ventricular heave (due to RVH)
Raised JVP with giant A waves
54
Management of pulmonary valve stenosis
Mild, asymptomatic --> watch and wait
Symptomatic or more significant --> ballon valvuloplasty, or open heart surgery
55
Presentation of aortic valve obstruction
Often asymptomatic
More significant: fatigue, SOB, dizziness, fainting, symptoms worse on exertion
56
Signs of aortic valve stenosis
Ejection systolic murmur, loudest at aortic area
Crescendo-descrendo, radiating to carotids
Ejection click, just before murmur
Palpable systolic thrill
Slow rising pulse, narrow pulse pressure
57
Management of aortic valve stenosis
Exercise testing, echo + ECG to monitor progression
More significant stenosis, may need to restrict physical activities
Percutaneous balloon aortic valvuloplasty
Surgical aortic valvotomy
Valve replacement
58
Complications of aortic valve stenosis
Left ventricular outflow tract obstruction
Heart failure
Ventricular arrhythmia
Bacterial endocarditis
Sudden death (often on exertion)
59
What genetic condition is Coarctation of the Aorta associated with
Turner syndrome
60
Pathophysiology of Coarctation of aorta
Left ventricular outflow obstruction --> increased LV afterload --> LVH
Narrowing of aorta reduces pressure of blood in arteries distal to narrowing, and increases pressure of blood proximally (e.g. first three branches of aorta)
61
Presentation of coarctation of aorta
Typically presents as ductus arteriosus begins to close
Weak/absent femoral pulses
4 limb BP measurement: high BP in arms, low in legs
Radial:femoral delay/radial:radial delay
Tachypnoea/increased work of breathing
Poor feeding
Grey + floppy baby
Cold extremities
62
Murmur in coarctation of the aorta
Potential systolic murmur in left infraclavicular area/below left scapula
63
Management of coarctation of the aorta
Prostaglandin infusion to keep duct open
Surgery: correct coarctation, ligate ductus arteriosus
64
Investigation of cardiac conditions antenatally
Echocardiogram
65
Causes of heart failure in neonate
Obstructed systemic circulation (duct-dependent)
Hypoplastic left heart syndrome
Critical aortic valve stenosis
Severe coarctation of the aorta
Interruption of the aortic arch
66
Causes of heart failure in infants
High pulmonary blood flow
VSD
Atrioventricular septal defect
Large PDA
67
Causes of heart failure in older children and adolescents
Right or left heart failure:
Eisenmenger syndrome (right only)
Rheumatic heart disease
Cardiomyopathy
68
Symptoms of heart failure
Breathlessness: particularly on feeding, or exertion
Sweating
Poor feeding
Recurrent chest infections
69
Signs of heart failure
Poor weight gain, or faltering growth
Tachypnoea
Tachycardia
Heart murmur, gallop rhythm
Enlarged heart
Hepatomegaly
Cool peripheries
70
What is hypoplastic left heart syndrome
Underdevelopment of entire left side of the heart
Mitral valve is small or atretic
LV small
Aortic valve atresia
Ascending aorta very small
Coarctation of the aorta nearly always
71
Presentation of hypoplastic left heart syndrome
May be detected antenatally
Duct-dependent systemic circulation
No flow through Left side of heart: ductal constriction --> profound acidosis, rapid CVS collapse
Weakness/absence of peripheral pulses
72
Management of hypoplastic left heart syndrome
Surgery: norwood procedure
Followed by Glenn or hemi-Fontant at 6 months
Another Fontan at about 3 years
73
What is rheumatic fever
Autoimmune condition
Triggered by streptococcus bacteria (group A, beta-haemolytic streptococci e.g. strep pyogenes)
Type 2 hypersensitivity reaction
74
Presentation of rheumatic fever
Presents 2-4 weeks following streptococcal infection e.g. tonsilitis or pharyngeal infection
Polyarthritis
Mild fever
Malaise
Rash
SOB
Chorea
Nodules
75
Cardiac involvement in rheumatic fever
Inflammation --> pericarditis, myocarditis, endocarditis -->
Tachycardia/bradycardia
Murmur (from valvular heart disease, mitral valve normally)
Pericardial rub
Heart failure
76
Skin findings with rheumatic fever
Subcutaneous nodules: on extensor surfaces, firm and painless
Erythema marginatum rash: pink rings of varying size, on torso and proximal limbs, pink border with fading centre, borders may unite to give 'map-like' appearance
77
Investigations in rheumatic fever
Throat swab for bacterial culture
Antistreptococcal antibodies titres
Echo, ECG and CXR for heart involvement
78
What is the Jones criteria for rheumatic fever
Diagnostic criteria: evidence of recent streptococcal infection plus two major criteria, or one major + two minor
79
Major criteria for rheumatic fever
JONES
Joint arthritis
Organ inflammation e.g. carditis
Nodules
Erythema marginatum rash
Sydenham chorea
80
Minor criteria for rheumatic fever
FEAR
Fever
ECG changes (prolonged PR interval) without carditis
Arthralgia without arthritis
Raised inflammatory markers (CRP, ESR)
81
Management of rheumatic fever
Antibiotics for streptococcal infection to prevent development: e.g. 10 days phenoxymethylpenicillin for streptococcal tonsilitis
NSAIDs for joint pain
Aspirin + steroids for carditis
Prophylactic abx to prevent further infections + recurrence
Monitoring + management of complications
82
Complications of rheumatic fever
Recurrence
Valvular heart disease (mitral stenosis)
Chronic heart failure
83
Which children are at risk of infective endocarditis?
All children of any age with congenital heart diease, except secundum ASD
Risk particularly high with turbulent jet of blood e.g. VSD, coarctation of aorta, or PDA; or prosthetic material
Previous rheumatic fever
84
Clinical signs of infective endocarditis?
Fever
Anaemia + pallor
Splinter haemorrhages
Tender nodules on fingers/toes (Osler's nodes)
Erythematous palms/soles of feet (Janeway lesions)
Clubbing (late)
Necrotic skin lesions
Splenomegaly
Retinal infarcts (Roth spots)
Arthritis/arthralgia
Haematuria (microscopic)
Heart murmurs (changing)
85
Mnemonic for signs of endocarditis (adults)
FROM JANE
Fever
Roth spots
Osler's nodes
Murmur
Janeway lesions
Anaemi
Nail haemorrhage
Emboilism
86
Investigation of infective endocarditis
Blood cultures before antibiotics
Transthoracic Echocardiogrphy
Blood tests: acute-phase reactants (CRP/ESR), FBC, U+Es
Urine dipstick
87
Causative organisms of infective endocarditis
alpha-haemolytic streptococcus (streptococcus viridans)
Others: strep bovis, staph aureus, HACEK group (haemophilus species etc)
88
Management of infective endocarditis
IV antibioics
High-dose penicillin + an aminoglycoside e.g. gentamicin or vancomycin
Minimum 6 weeks
Surgery to remove infected prosthetic material
89
Prophylaxis of infective endocarditis
Good dental hygiene
90
What is croup
Laryngeotracheobronchitis
Viral infection causing upper airway obstruction
91
Common causative agent of croup
Parainfluenza viruses
Others: rhinovirus, RSV, influenza, adenovirus
92
Age of presentation of croup
6 months - 6 years
Peak in 2nd year of life
93
Presentation of croup
Common in autumn
Hoarseness (inflamed vocal cords)
Barking cough (tracheal oedema and collapse)
Harsh stridor (inspiratory)
Difficulty breathing - with chest retraction
Symptoms start/worse at night
Low grade fever
94
Onset of croup
Over days
With preceding coryza
95
Management of croup
Supportive treatment - get child to sit up when coughing, comfort, fluids and rest
?Inhalation of warm moist air
Oral dexamethasone - single dose given to all children regardless of severity (Oral prednisolone if not available)
Nebulised steroids (budenoside)
Nebulised adrenaline if more severe, with oxygen
Intubation + ventilation if necessary
96
Indications for admission with croup
Moderate or severe croup e.g. frequent cough, easily audible stridor at rest, obvious signs of increased work of breathing, may be agitated/distressed etc
<6 months of age
Known upper airway abnormalities
Uncertainty of diagnosis
97
Causative agent of acute epiglottitis
Haemophilus influenza type b (Hib)
Prevented by vaccination in many children
98
Life-threatening differential diagnosis of croup
Acute epiglottitis - more rapid onset
99
Presentation of acute epiglottitis
Sore throat
Stridor (soft)
Drooling
Tripod position - sat forward with hand on each knee
High fever
Difficulty/painful swallowing
Increasing respiratory difficulty
Muffled voice
Scared + quiet child
Septic and unwell appearance
100
Description of cough in acute epiglottitis
Minimal or absent
101
Most important point in investigation/management of acute epiglottitis
Do not lie the child down or examine the throat with a spatula
Do not perform an X-ray
Any of thse can cause total airway obstruction
102
Lateral X-ray sign for acute epiglottitis
Thumb sign/thumbprint sign: soft tissue shadow looks like a thumb is pressed into the trachea
103
Management of acute epiglottitis
Call for senior paediatrician + anaesthetist + ENT surgeon
Anaesthetist to secure airway
Intubation performed under controlled conditions if required
Urgent tracheostomy may be performed
Once airway is secure --> blood for cultures
IV antibiotics e.g. cefuroxime for 3-5 days (tube removed before this)
Rifampicin to close household contacts
104
Another name for whooping cough
Pertussis
105
Causative agent of whooping cough
Bordatella pertussis
106
Presentation of whooping cough
Preceding week of coryzal symptoms: low grade fever, mild cough (cattarhal phase)
Then a paroxysmal/sporadic cough: severe fits, keep building until out of breath
Child goes red or blue in face, mucus from nose and mouth
Followed by inspiratory whoop (inhalation against closed glottis). May be apnoea in infants
Can cough so hard --> fainting, vomiting, epistaxis, subconjunctival haemorrhage, pneumothorax
107
Duration of whooping cough
Paroxysmal phase (severe coughing fits +/- whoop) lasts up to 3 months
Symptoms then gradually decrease (convalescent phase) but may persist for many more months
108
Complications of whooping cough
Pneumothorax
Seizures
Bronchiectasis
109
Diagnosis of whooping cough
Nasopharyngeal or nasal swab with PCR testing or bacterial culture if cough present for 2-3 weeks
If cough >2 weeks, oral fluid tested for anti-pertussis toxin immunoglobulin G (blood if >17)
Lymphocytosis on blood count
110
Management of whooping cough
Supportive care
Antibiotics can be started in first 21 days of symptoms: macrolide e.g. azithromycin, erythromycin etc. Also used in vulnerable patients
Close contacts receive prophylactic antibiotics
Notifiable disease
111
What is the most common LRTI in children?
Bronchiolitis - inflammation and infection in the bronchioles
112
Commonest age for bronchiolitis
1-9 months
113
When might bronchiolitis be more likely in older infants?
Ex-premature infants with chronic lung disease
114
Causative pathogen(s) of bronchiolitis?
Respiratory syncitial virus (RSV)
Others: parainfluenza virus, rhinovirus, adenovirus, influenza virus, human metapneumovirus
115
Symptoms of bronchiolitis
Preceding coryzal symptoms
Dry cough
Increasing breathlessness
Feeding difficulty (due to increased dyspnoea)
Mild fever
Apnoeas are common
116
Findings on examination in bronchiolitis
Dry, wheezy cough
Tachycardia + tachypnoea
Subcostal + intercostal recession
Hyperinflation of the chest
Find end-inspiratory crackles (crepitations)
High-pitched wheeze (expiratory > inspiratory)
117
Signs of repiratory distress in an infant
Raised resp rate
Use of acessory muscles of breathing e.g. sternocleidomastoid, abdominal + intercostal
Intercostal + subcostal recessions
Nasal flaring
Head bobbing
Tracheal tugging
Cyanosis
Abnormal airway noises
118
What is wheezing?
A whistling sound caused by narrowed airways, typically heard during expiration
119
What is grunting?
Caused by exhaling with the glottis partially closed to increase positive end-expiratory pressure
120
What is stridor?
A high pitched, inspiratory noise cause by obstruction of the upper airway
121
When would a child with bronchiolitis be admitted to hospital?
Reported or observed apnoea
Persistent O2 <90% on air
Inadequate oral fluid intake (50-75% of usual volume)
Severe respiratory distress e.g. grunting, marked chest recession, resp rate >70
122
Management of bronchiolitis
Supportive
Humidified oxygen: nasal cannulae or head box
Monitoring
NG feeds
Fluids
123
Prevention of bronchiolitis
IM Palivizumab monthly (MAB against RSV)
Given to high-risk individuals e.g. CF, congenital heart disease, immunocompromised, Down's syndrome, premature infants
124
What is asthma?
Chronic inflammatory airway disease, leading to variable airway obstruction (reversible)
Smooth muscle of airways is hypersensitive to stimuli --> constriction --> airflow obstruction
Atopic condition
125
Atopic conditions
Asthma
Eczema
Hay fever
Food allergies
126
What is viral-induced/episodic wheeze
Small airway obstruction due to inflammation in response to a viral infection
Diagnosed in children <5 years of age
127
Key features of asthma in a child
Diurnal variation of symptoms: worse at night and in early morning
Symptoms with nonviral triggers
Interval symptoms e.g. symptoms between acute exacerbations
Personal or family history of atopic disease
Positive response to asthma therapy
128
Examination findings in long-standing asthma
Hyperinflation of the chest
Generalised polyphonic expiratory wheeze
Prolonged expiratory phase
Harrison's sulci: depression at base of thorax associated with muscular insertion of the diaphragm
129
Diagnosis of asthma
Clinical symptoms
FEV1:FVC <70%
Bronchodilator reversibility: FEV1 improved by 12% or more
FeNO >= 35ppb
130
Conditions screened for on newborn heel prick test
Congenital hypothyroidism
Sickle cell disorders
CF
Phenylketonuria
Medium-chain acyl-CoA dehyodrogenase deficiency (MCADD)
Maple syrup urine disease
Isolvaleric acidaemia
Glutaric aciduria type 1 (GA1)
Homocystinuria
131
First pubertal sign in females
Breast budding
132
First pubertal sign in males
Testicular enlargement
133
Paediatric sepsis 6 algorithm
1. High flow O2
2. IV or IO access - take blood cultures, blood glucose, blood lactate/gas
3. Give IV or IO antibiotics
4. Fluid resuscitation
5. Consider inotropic support e.g. adrenaline
6. Involve seniors/specialists early
134
What antibiotics should be used in suspected sepsis in children when causative organism is unknown
IV 3rd generation cephalosporins e.g. cefotaxime
IV amoxicillin in infants under 3 months to cover for Listeria
135
Features of glomerulonephritis
Proteinuria + haematuria
Oedema
Weight gain secondary to oedema
136
What is Henoch-Schonlein Purpura
Also known as IgA vasculitis
Produces non-blanching rash, general unwellness, fatigue and abdominal pain
Often triggered by URTI or gastroenteritis
137
Classic features of Henoch-Schonlein Purpura
Purpura - typically starting on legs and spreading to buttocks. Urticarial, then becomes maculopapular + purpuric
Joint pain - esp knees and ankles
Abdominal pain
Renal involvement (IgA nephritis)
(Fever can also occur_
138
Complication of Henoch-Schonlein Purpura
IgA Nephropathy
139
Difference between symptoms in IgE-mediated and non IgE-mediated CMPA?
IgE-mediated symptoms appear within minutes-2 hours of consuming cow's milk
Non IgE-mediated develop 2-72 hours after consumption
140
In which children does CMPA more commonly present?
<1 year age
More common in formula-fed babies, or when weaning, or when switched to formula milk
Can be present in breastfed babies when mother consumes dairy products
Family history of atopic conditions
141
Symptoms of CMPA
Bloating + wind
Abdominal pain + Diarrhoea
Vomiting + regurgitation
Urticarial rash/atopic eczema
Angio-oedema
Cough or wheeze
Sneezing
Watery eyes
142
Management of CMPA
Breast-feeding: avoid dairy products
Hydrloysed formulas designed for CMPA
In severe cases, elemental formulas made of basic amino acids (e.g. neocate)
Mostly outgrow by age 3
Start on milk ladder every 6 months or so
143
Two most common causes of nephritis in children
Post-streptococcal glomerulonephritis
IgA nephropathy (Berger's disease)
144
How does nephritis/nephritic syndrome present
Reduction in kidney function
Haematuria
Proteinuria (less than nephrotic syndrome)
145
Presentation of post-streptococcal glomerulonephritis
Occurs 1-3 weeks after a beta-haemolytic strep infection e.g. tonsilitis caused by strep pyogenes
Causes an acute kidney injury
146
Management of post-strep glomerulonephritis
Throat swab + anti-streptolysin antibody titres to confirm diagnosis
Supportive
May need antihypertensives and diuretics if develop hypertension and oedema
147
Management of IgA nephropathy
Supportive of renal failure
Immunosupressant medications e.g. steroids and cyclophosphamide to slow progression
148
Renal biopsy findings in IgA nephropathy
IgA deposits
Glomerular mesangial proliferation
149
What is slapped cheek syndrome
Erythema infectiosum
AKA fifth disease
150
What causes erythema infectiosum
Parovirus B19
151
Presentation of erythema infectiosum
Children aged 3-15 years
One week following exposure, mild prodromal illness e.g. headache, rhinitis, sore throat, low-grade fever, malaise
Symptom free for 7-10 days --> slapped cheek rash with eythematous cheeks
May also have arthropathy
152
Description of rash in erythema infectiosum
Rash on cheeks, spares nose, perioral and periorbital regions
Develops on extremities after a few days (particularly extensor surfaces)
Not itchy in young children
Gradually fades (up to 3 weeks to resolve)
153
Management of erythema infectiosum
Symptomatic
Avoid contact with pregnant women + immunocompromised individuals
154
What is roseola infantum
Common disease of infanncy caused by human herpes virus 6 (and sometimes HHV-7)
Also known as roseola, or sixth disease
155
Features of roseola infantum
High fever lasting a few days +/- coryzal symptom + swollen lymph nodes
Followed 1-2 weeks later by -->
Maculopapular rash (typically on chest, arms + legs; not itchy)
Nagayama spots: papular enanthem on uvula + soft palate
Febrile convulsions are relatively common
Diarrhoea + cough also commonly seen
156
Presentation of haemophilia A
Joint bleeding
Deep muscular bleeds
157
Geneology of haemophilia A
X-linked recessive disorder
158
Coagulation findings in haemophilia A
Prolonged APTT
Normal PT
159
Appropriate topical treatments for pseudomonas species
Ciprofloxacin and gentamicin
160
What side effect needs to be monitored for with IV salbutamol?
Hypokalaemia
161
Definition of moderate asthma attack <5 years old
Sats >92%, no clinical features
162
First line management of asthma in children >5
Short-acting beta-agonist reliever therapy e.g. salbutamol only in children with infrequent, short-lived wheezze and normal lung function
163
First line maintenance therapy for asthma in children >5
Low dose of an inhaled corticosteroid e.g. budesonide, beclometasone, fluticasone, mometasone
Used if symptoms at presentation clearly indicate maintenance therapy (symptoms 3x week, or waking at night), or if uncotrolled with SABA alone
164
What medication regime should be used for asthma in children >5 where ICS does not control symptoms
Add a LABA e.g. salmeterol - only continue if there is a good response
165
What medication regime should be used for asthma in children 5-12 where ICS + LABA does not control symptoms
Consider starting LTRA alongside ICS e.g. montelukast
Consider starting oral theophylline - side effects are strong
166
If asthma uncontrolled on paediatric low dose ICS + LABA in children 5-12
Consider increasing ICS dose
167
Initial management of suspected asthma in children <5
SABA for reliever therapy
168
Initial prevention therapy for suspected asthma in children <5
Very low dose ICS or LTRA
169
Therapy after initial prevention therapy in children <5 with suspected asthma
Very low dose ICS, plus LTRA
170
Problems associated with long-term steroid use in children
Adverse effect on growth
Adrenal suppression
Altered bone metabolism
171
Signs of moderate acute asthma attack in children
Peak flow >50% predicted
Normal speech
No clinical features
172
What medication regime should be used for asthma in children >12 where ICS + LABA does not control symptoms
Titrate up ICS dosage
Consider adding oral LTRA, oral theophylline, or an inhaled long-acting muscarinic antagonist (LAMA) e.g. tiotropium
173
What medication regime should be used for asthma in children 5-12 where ICS + LTRA/LAMA etc does not control symptoms
Titrate ICS up to high dose
Combine treatments from step 4 e.g. LTRA + LAMA
Consider oral beta-2 agonist e.g. salbutamol
174
Signs of severe asthma attack
Peak flow 33-50% predicted
Saturations <92%
Unable to complete sentences in one breath
Signs of respiratory distress
Resp rate >40 (1-5 years); >30 (5-12 years): >25 (12-18)
Heart rate >140 (1-5 years); >125 (5-12 years); >110 (12-18)
175
Signs of life-threatening asthma attack
Peak flow <33% predicted
Saturations <92%
Exhuastion + poor resp effort
Hypotension
Silent chest
Cyanosis
Altered consciousness/confusion
176
Management of moderate asthma attack
Keep calm + reassure
SABA via spacer (or face mask): 2-4 puffs, increasing by 2 puffs every 2 minutes to 10 puffs
Oral prednisolone
Monitor response for 15-30 minutes
177
Principles of management in acute viral induced wheeze or asthma
Supplementary oxygen if required
Bronchodilator therapy
Steroids (oral prednisolone, or IV hydrocortisone)
Antibiotics if bacterial cause suspected
178
Step-up pathway of bronchodilators in acute asthma
Inh or neb salbutamol
Inh or neb ipratropium bromide (anti-muscarinic)
IV magnesium sulphate
IV aminophylline
179
Management of severe acute asthma attack
High-flow oxygen
SABA via spacer (10 puffs) or nebulised - assess response + repeat as required
Oral prednisolone or IV hydrocortisone
Consider: inhaled iptraopium, IV beta-2 agonist e.g. salbutamol, or aminophylline, or magnesium
180
Management of life-threatening acute asthma attack
High-flow oxygen
SABA nebulised - assess response continuously and repeat a required (back to back)
Orl pred or IV hydrocortisone
Nebulised ipratropium
Consider: IV beta-2 agonist e.g. salbutamol, or aminophylline, or magnesium
181
Management of acute asthma attack if responding to treatment
Continue bronchodilators for 1-4 hours PRN
Discharge when stable on 4h treatment
Continue oral prednisolone for 3-7 days
Arrange follow-up
182
Management of asthma attack not responding to treatment
Transfer to HDU/PICU
Senior medical review
Consider IV therapies not already used
Consider CXR to check for pneumothorax, and blood gases
Consider need for mechanical ventilation
183
Geneology of cystic fibrosis
Autosomal recessive defect in CFTR gene affecting mucus glands
184
GI consequences of CF
Thick pancreatic + biliary secretions --> blockage of ducts --> lack of digestive enzymes in digestive tract e.g. pancreatic lipase
185
Respiratory consequences of CF
Low volume thick airway secretions --> reduce airway clearance --> bacterial colonisation + susceptibility to infections
186
Reproductive consequence of CF
Congenital bilateral absence of vas deferens in males
Healthy sperm, but cannot get to ejaculate --> male infertility
187
First sign of CF (not bloodspot test)
Meconium ileus - mecoium thick + sticky, gets stuck and obstructs bowel
Meconium not passed within 24 hours, abdominal distension + vomiting
188
If CF not diagnosed at birth, how does it present in childhood
Recurrent LTRI
Failure to thrive
Pancreatitis
189
Symptoms of CF
Chronic cough
Thick sputum production
Recurrent resp infections
Steatorrhoea
Abdominal pain + bloating
Failure to thrive (poor weight + height gain)
May taste salty when kissed (concentrated salt in sweat)
190
Signs of CF
Low weight or height on charts
Nasal polyps
Finger clubbing
Crackles + wheezes on auscultation
Abdominal distension
191
Causes of clubbing in children
Hereditary
Cyanotic heart disease
Infective endocarditis
CF
Tuberculosis
IBD
Liver cirrhosis
192
Diagnosis of CF (x3)
Newborn guthrie heel prick bloodspot testing
Sweat test (gold standard)
Genetic testing for CFTR gene (during pregnancy or after birth)
193
Key colonisers seen in CF
Staphylococcus aureus
Pseudomonas aeruginosa
Haemophilus influenza
Klebsiella pneumoniae
Escherichia coli
Burkhodheria cepacia
194
Management of pseudomonas colonisation in CF
Long-term nebulised antibiotics e.g. tobramycin
Oral ciprofloxacin
195
Management of CF
Chest physio
Exercise
High calorie diet
CREON tablets if pancreatic insufficiency
Prophylactic flucloxacillin
Treat chest infection
Bronchodilators
Nebulised dornase alfa (reduce viscosity of secretions)
Nebulised hypertonic saline
Vaccinations
196
Longer-term management of CF
Lung transplantation if end-stage resp failure
Liver transplant in liver failure
Fertility treatment
Genetic counselling
197
What conditions do people with CF need to monitored/screened for?
Diabetes
Osteoporosis
Vit D deficiency
Liver failure
198
Early features of meningococcal septicaemia in children
Leg pain
Diarrhoea
Abnormal skin colour
Breathing difficulty
Cold peripheries
199
Triad of findings in Haemolytic Uraemic Syndrome
AKI
Thrombocytopenia (normal clotting time)
Normocytic anaemia (microangiopathic haemolytic anaemia)
200
What triggers HUS?
Usually shiga toxin (bacterial toxin) typically produced by e.coli 0157, or Shigella
201
Risk factors for HUS
Use of antibiotics and anti-motility medications e.g. loperamide to treate gastroenteritis caused by E.coli or Shigella
202
Presentation of HUS
Gastroenteritis (caused by E.coli) with bloody diarrhoea
Symptoms start 5 days after diarrhoea
Reduced urine output
Haematuria
Abdominal pain
Lethargy and irritability
Confusion
Oedema
Hypertension
Bruising
203
Management of HUS
Supportive
Renal dialysis if required
Antihypertensives if required
Fluid balance
Blood transfusions if required
204
Complications of HUS
Abdominal pain
Myocarditis
Encephalitis-like features
Hepatitis
Pancreatitis
Retinal haemorrhages
Hypertension due to renal damage
205
Causative agent of Infectious mononucleosis
Epstein Barr Virus
206
Complication of amoxicillin in infectious mononucleosis
Maculopapular pruritic rash
Also happens with cephalosporins
207
Features of infectious mononucleosis
Fever
Sore throat
Fatigue
Lymphadenopathy
Tonsillar enlargment
Splenomegaly (or rupture)
208
Investigations in glandular fever
Monospot test - heterophile antibodies react to horse red blood cells
Paul-Bunnell test - similar, but uses blood cells from sheep
209
Complications of glandular fever
Splenic rupture - advised to avoid contact sports
Glomerulonephritis
Haemolytic anaemia
Thrombocytopenia
Chronic fatigue
Avoid alcohol
210
Cancer associated with EBV infection
Burkitt's lymphoma
211
Vaccinations in 6-in-1
Parents Will Immunise Toddlers Because Death
P - Polio
W - Whooping Cough (pertussis)
I - Influenza (haemophilus influenzae B)
T - Tetanus
B - hepatitis B
D - Diptheria
212
Vaccinations at 8 weeks
6 in 1
MenB
Rotavirus (oral)
213
Vaccinations at 12 weeks old
6 in 1 booster
Pneumococcal
Rotavirus booster
214
Vaccinations at 16 weeks
6 in 1 booster
MenB booster
215
1 year old vaccinations
2 in 1: Hib and MenC
Pneumococcal booster
MenB booster
MMR dose 1
216
How is the influenza vaccination given to children
Nasal spray
All children primary school --> year 7
Highr risk: from 6 months
217
Vaccinations at 3 years 4 months
4 in 1: diptheria, tetanus, pertussis, polio
MMR dose 2
218
Vaccination at 12-13 years old
HPV - two doses 6-24 months apart
Given before sexual activity
219
Vaccinations at 14 years old
3 in 1: tetanus, diptheria, polio
Meningococcal groups A, C, W, Y (WACY)
220
Types of leukaemia in children
Acute lymphoblastic leukaemia (ALL) - most common
Acute myeloid leukaemia (AML) - next most common
Chronic myeloid leukaemia (CML) - rare
221
Age peak for ALL
2-3 years
222
Age peak for AML
<2 years
223
Risk factors for leukaemia
Radiation exposure e.g. abdo x-ray in pregnancy
Down's syndrome
Kleinfelter syndrome
Noonan syndrome
Fanconi's anaemia
224
Presentation of leukaemia
Typically symptoms of anaemia e.g. fatigue
Unexplained fever
Weight loss
Night sweats
Pallor
Petechiae and abnormal bruising
Unexplained bleeding
Generalised lymphadenopathy
Bone or joint pain
Hepatosplenomegaly
Abdominal pain
225
Diagnosis of leukaemia
FBC: pancytopenia e.g. anaemia, leukopenia and thrombocytopenia
Blood film: blast cells
Bone marrow biopsy
Lymph node biopsy
226
Congenital adrenal hyperplasia inherited mechanism
Autosomal recessive
Deficiency of 21-hydroxylase enzyme --> underproduction of cortisol and aldosterone, overproduction of androgens
Can also be 11-beta-hydroxylase enzyme
227
Consequences of congenital adrenal hyperplasia
Decreased sodium retention --> hyponatraemia
Decreased potassium excretion --> hyperkalaemia
228
Presentation of CAH in females
Typically present at birth with virilised genitalia e.g. enlarged clitoris due to high testosterone
229
Presentation of severe CAH
Hyponatraemia
Hyperkalaemia
Hypoglycaemia
May have metabolic acidosis
Poor feeding
Vomiting
Dehydration
Arrhythmias
230
Presentation of mild CAH
Symptoms relate to high androgen levels
Females: tall for age, facial hair, absent periods, deep voice, early puberty
Males: tall for age, dep voice, large penis, small testicles, early puberty
231
Hyperpigmentation in CAH
Anterior pituitary gland responds to low cortisol by producing ACTH --> melanocyte stimulating hormone --> melanin
232
Management of CAH
Followed for growth and development
Cortisol replacement - hydrocortisone
Aldosterone replacement - fludrocortisone
Corrective surgery for genitals if required
233
Typical presentation of febrile convulsion
18 months old
2-5 minute tonic-clonic seizure
During high fever usually caused by underlying viral illness or bacterial infection e.g. tonsilitis
234
'Sanctuary sites' from chemotherapy
Central nervous system (BBB)
Testes
235
Complications of sickle cell disease
Anaemia
Infection
Stroke
Avascular necrosis in large joints e.g. hip
Pulmonary hypertension
Priapism
CKD
Sickle cell crises
Acute chest syndrome
236
Management of sickle cell disease
Antibiotic prophylaxis, usually with PenV
Hydroxycarbamide - stimulates production of HbF, prevents vaso-occlusive complications
Blood transfusion
Bone marrow transplant/stem cell transplant
237
What conditions come under sickle cell crisis
Vaso-occlusive crisis (painful)
Splenic sequestration crisis
Aplastic crisis
Acute chest syndrome
238
Vaso-occlusive crisis features
Distal ischaemia
Dehydration + raised haematocrit
Pain, fever, features of infection
Pripaism
239
Features of splenic sequestration crisis
Acutely enlarged and painful spleen
Can lead to severe anaemia and hypovolaemic shock
Supportive management e.g. blood transfusion + fluid resuscitation
Splenectomy often used to prevent
240
Aplastic crisis
Typically triggered by parovirus B19
Significant anaemia
Management is supportive (blood transfusions)
Usually resolves spontaneously
241
Acute chest syndrome
Fevere/respiratory symptom with new infiltrates seen on CXR
Antibiotics/antivirials
Blood transfusion
Incentive spirometry to encourage effective breathing
Artifical ventilation
242
Inheritance of haemophilia A/B
X-linked recessive --> almost exclusively affects males
243
Deficiencies in haemophilia
A - factor VIII
B - factor IX
244
Features of haemophilia
Excessive bleeding in response to minor trauma
Spontaneous haemorrhage
Neonates + children: intracranial haemorrhage, haematomas, cord bleeding
Spontaneous bleeding into joints + muscles (severe)
245
Management of haemophilia
IV infusion of clotting factors - prophylactic or response to bleeding. Antibodies can form against these
In acute episodes: desmopressin (stimulate release of vWF), and antifibronlytics e.g. tranexamic acid
246
What is kawasaki disease
Mucocutaneous lymph node syndrome
Systemic, medium-sized vessel vasculitis
Affects children usally <5, typically Asian
247
Key complication of Kawasaki disease
Coronary artery aneurysm
248
Presentation of Kawasaki disease
CRASH + Burn
Conjunctivitis (bilateral)
Rash (widespread erythematous maculopapular)
Adenopathy (cervical lymph nodes)
Strawberry tongue
Hands (palmar erythema, swelling, desquamation)
Burn = Persistent high fever >5 days (>39 degrees)
249
Investigations in Kawasaki disease
FBC - anaemia, low WCC, low platelet
LFT - low albumin, elevated liver enzymes
Inflammatory markers - raised (especially ESR)
Urinalysis - white cells w/o infection
Echocardiogram - rule out coronary artery pathology
250
Cardiac complications of kawasaki disease
Pericardial effusion
Myocardial disease
Valve damage
Coronary artery disease
251
Common age of Perthes disease presentation
5-10 years
Males
252
What is Perthes disease
Avascular necrosis of the capital femoral epiphysis
253
Complication of Perthes disease
Soft and deformed femoral head --> early hip osteoarthritis
Premature fusion of growth plates
254
Presentation of Perthes disease
Pain in hip or groin
Limp
Restricted hip movements
Potentiall referred knee pain
No history of trauma (if minor trauma, consider slipped upper femoral epiphysis)
255
Management of Perthe's disease
Age <6, no significant collapse of the femoral head or gross structural abnormalities :
- Observation including serial X-rays
- Physiotherapy
In severe cases (especially if >6), surgery may be required to improve alignment and function of femoral head + hip
256
Risk factors for developmental dysplasia of the hip
First degree family history
Breech presentation from 36 weeks
Breech presentation at birth if 28 weeks onwards
Multiple pregnancy
Female
Oligohydramnios
High birth weight
Prematurity
257
Screening for DDH
Barlows and Ortolani tests
258
Which children require USS examination for DDH
First degree family history of hip problems in early life
Breech presentation at, or after, 6 weeks gestation (regardless of presentation at birth, or mode of delivery)
Multiple pregnancy
259
Management of DDH
Most unstable hips will spontaneously stabilise by 3-6 weeks of age
Pavlik harness (dynamic flexion-abduction orthosis) in children <4-5 months
Older children may require surgery
260
Causes of intestinal obstruction
Meconium ileus
Hirschsprung's disease
Oesophageal or duodenal atresia
Intussusception
Imperforate anus
Volvulus
Strangulated hernia
261
Presentation of intestinal obstruction
Persistent vomiting (may be bilious)
Abdominal pain + distension
Failure to pass stools or wind
Abnormal bowel sounds
262
Investigation of intestinal obstruction
Abdominal X-ray: dilatedloops of bowel proximal to obstructions, collapsed loops distal. Absence of air in rectum
263
Facial features of Down's syndrome (x7)
Brushfield spots in the iris
Small ears
Upslanted palpebral features
Round face
Flat occiput
Epicanthic folds
Protruding tongue
264
Non-facial features of Down's syndrome (x4)
Short stature
Learning difficulties
Hypotonia
Delayed motor milestones
265
Medical problems associated with Down's syndrome
Congenital heart defects: Tetralogy of Fallot, AVSD, VSD, ASD
Hearing loss
Visual problems: cataracts, strabismus, keratoconus
GI problems: oesphageal/duodenal atresia, Hirschsprung's disease, coeliac disease
Hypothyroidism
AML/ALL
Alzheimer's disease
266
When should an ambulance be called for a febrile convulsion
If the convulsions last longer than 5 minutes, or if recovery takes longer than 60 minutes
267
Characteristic early signs of measles
High fever
Conjunctivitis
Koplik's spots = white lesions on buccal mucosa (pathognomic), typically develop before rash
Irritable
268
Description of the rash in measles
Starts behind ears --> whole body
Maculopapular rash
Flat lesions
Becomes blotchy and confluent
Desquamation typically sparing palms + soles may occur after a week
269
Complications of measles
Otitis media - most common
Pneumonia - most common cause of death
Encephalitis - typically 1-2 weeks following onset
Subacute sclerosing panencephalitis - very rare, may present 5-10 years following illness
Febrile convulsions
Keratoconjunctivitis, corneal ulceration
Diarrhoea
Increased incidence of appendicitis
Myocarditis
270
Risk factors for necrotising enterocolitis
Very low birth weight, or very premature
Formula feeding
Respiratory distress + assisted ventilation
Sepsis
PDA + other congenital heart disease
271
Initial presentation of NEC
Feeding intolerance
Abdominal distension
Bloody stools
Vomiting (green bile)
Generally unwell
Absent bowel sounds
272
Presentation of perforation in NEC
Peritonitis
Shock
Severely unwell
273
Findings on AXR for NEC
Dilated bowel loops (often asymmetrical)
Bowel wall oedema
Intramural gas (pneumatosis intestinalis)
Pneumoperitoneum (due to perforation)
Gas in portal veins
Air both inside and outside of bowel wall (Rigler sign)
Air outlining falciform ligament (football sign)
274
Management of NEC
Nil by mouth
IV fluids, TPN and antibiotics
NG tube may be used to drain fluid + gas
Surgical emergency (some require with medical treatment, some require removal of dead bowel tissue --> short bowel syndrome)
275
What is Ebstein's anomaly
'Atrialisation' of the right ventricle - low insertion of tricuspid valve --> large right atrium, small right ventricle --> tricuspid incompetence
276
What causes Ebstein's anomaly
Exposure to lithium in utero
277
Features of Ebstein's anomaly
Cyanosis
Prominent a-wave in distended JVP
Hepatomegaly
Tricuspid regurgitation - pansystolic murmur, worse on inspiration
RBBB (widely split S1 and S2)
May also have patent foramen ovale or atrial septal defect
278
Features of pyloric stenosis
Hungry baby: thin, pale, failing to thrive
Projectile vomiting (strong peristalsis of stomach), typically 30 minutes after feed
Firm, round mass in upper abdomen (feels like a large olive)
Dehydration + constipation may also be present
Tends to present in first 2-4 weeks of life
279
Blood gas analysis in pyloric stenosis
Metabolic alkalosis
Hypochloraemia
Elevated bicarbonate
Hypokalaemia
280
Diagnosis of pyloric stenosis
Ultrasound - visualise thickened pylorus
281
Management of pyloric stenosis
Laparoscopic pyloromyotomy = Ramstedt's operation
282
Key points of paediatric BLS
Open airway
Look, listen + feel for breathing: no breathing? --> give 5 rescue breaths (more likely to be resp cause)
Check for signs of circulation (brachial or femoral in infants, femoral in children)
Give chest compressions 15:2 ratio with rescue breaths
100-120/min
283
Commonest causative organisms of neonatal sepsis
Group B streptococcus
Escherichia coli
Listeria
Klebsiella
Staphylococcus aureus
284
Risk factors for neonatal sepsis
Vaginal GBS colonisation
GBS sepsis in previous baby
Maternal sepsis, chorioamnionitis, or fever >38
Prematurity, low birthweight
P-PROM
Prolonged rupture of membranes
285
Presentation of neonatal sepsis
Non-specific (high degree of suspicion required)
Respiratory distress or apnoea
Tachycardia or bradycardia
Fever
Reduced tone + activity
Poor feeding
Vomiting
Hypoxia
Jaundice within 24 hours
Seizures
Hypoglycaemia
286
Red flags concerning for neonatal sepsis
Confirmed or suspected sepsis in the mother
Signs of shock
Seizures
Term baby needing mechanical ventilation
Resp distress >4 hours after birth
Presumed sepsis in another baby (in multiple pregnancy)
287
Management of neonatal sepsis
IV benzylpenicillin with gentamicin = first line
Monitor CRP levels (abx ceased at 48 hours if CRP <10mg/L, and negative BC at presentation and at 48 hours)
Ozygen
Normal fluid + electrolyte status
288
Features of Prader-Willi syndrome
Constant, insatiable hunger --> obesity
Hypotonia as an infant
Learning disability
Hypogonadism
MH problems (anxiety)
Dysmorphic features: narrow forehead, almond shaped eyes, strabismus, thin upper lip, downturned mouth
289
Management of Prader-Willi syndrome
Carefully limiting access to food/interaction with dietitians
Growth hormone - improve muscle development + body composition
MDT approach
290
Causes of neonatal hypotonia
Neonatal sepsis
Werdnig-Hoffman disease (spinal muscular atrophy type 1)
Hypothyroidism
Prader-Willi
Maternal drugs e.g. benzodiazepines
Maternal myasthenia gravis
291
Pathophysiology of Meckel's diverticulum
Failure of vitelline duct to obliterate during fifth week of foetal development
Typically just proximal to ileocaecal valve --> mimics appendicitis pain
Haemodynamically unstable patient - due to substantial haemorrhage (rectal bleeding)
292
Rule of 2s in Meckel's diverticulum
Occurs in 2% of population
2 feet from ileocaecal valve
2 inches long
293
Presentation of Meckel's diverticulum
Often asymptomatic
Abdominal pain mimicking appendicitis
Rectal bleeding - most common cause of painless massive GI bleeding requiring a transfusion in children 1-2 years old
Intestinal obstruction
294
Investigations of Meckel's diverticulum
Investigation of choice for stable children with suspected Meckel's --> technetium scan
Mesenteric arteriography may be used in more severe cases
295
Management of Meckel's diverticulum
Removal if narrow neck or symptomatic
296
Presentation of laryngomalacia
Stridor - most common cause in infants - on inhalation
- Intermittent
- Worse when feeding, upset, lying on back, or during URTI
Peaks at arund 6 months
297
Shaken baby syndrome features
Retinal haemorrhages
Subdural haematoma
Encephalopathy
298
Exanthemas in children
Eruptive widespread rashes:
- Measles
- Scarlet fever
- Rubella
- Dukes' disease
- Parovirus B19
- Roseola infantum
299
Is measles a notifiable disease
Yes
300
Causative organism/s of Scarlet fever
Group A streptococcus (typically associated with tonsillitis) e.g. strep pyogenes
301
Features of scarlet fever
Red-pink, blotchy, macular rash with rough skin
Starts on trunk and spreads outwards
Fever
Red, flushed cheeks
Sore throat
Strawberry tongue
Lethargy
Cervical lymphadenopathy
302
Management of scarlet fever
Phenoxymethylpenicillin for 10 days
Notifiable disease
Kept off school until 24 hours after starting abx
303
Causative organism for hand, foot + mouth disease
Coxsackie A virus
304
Presentation of hand, foot + mouth disease
Starts with URTI symptoms - tiredness, sore throat, dry cough, raised temperature
1-2 days later: small mouth ulcers appear
Red, blistering spots across body (on hands, feet, and around mouth)
Painful mouth ulcers
305
Features of benign rolandic epilepsy
4-12 years old
Seizures characteristically occur at night
Typically partial seizures e.g. paraesthesia affecting face
Secondary generalisation may occur
Child otherwise normal, although commonly sleep-deprived
EEG - shows centro-temporal spikes
Seizures stop by adolescence
306
Complications post-gastroenteritis
Lactose intolerance
Irritable bowel syndrome
Reactive arthritis
Guillain-Barre syndrome
307
Features of slipped upper femoral epiphysis
10-15 years old
More common if obese, and if male
Hip, groin, medial thigh or knee pain
Loss of internal rotation of leg in flexion
May present acutely following trauma, or with chronic, persistent symptoms
Biltateral slip in 20% cases
308
Investigation of SUFE
AP + Lateral (frog-leg) x-rays of hips
Displacement of femoral head epiphysis postero-inferiorly
309
Management of SUFE
Internal fixation - single cannulated screw placed in centre of epiphysis
310
Pathophysiology of Hirschsprung's disease
Parasympathetic neuroblasts fail to migrate from neural crest to distal colon
--> developmental failure of parasympathetic plexuses
--> uncoordinated peristalsis
--> functional obstruction
Aganglinic section does not relax --> becomes constricted
311
Features of Hirschsprung's disease
3x more common in males
Associated with Down's syndrome
Neonatal period: failure or delay to pass meconium
Older children: constipation, abdominal distension
Bilious vomiting
Lethargy
Dehydration
312
Investigations for Hirschsprung's disease
Abdominal X-ray
Rectal biopsy = gold standard
313
Management of Hirschsprung's disease
Initial: rectal washouts/bowel irrigation
Definitive: surgery to affected segment (swenson procedure --> remove section of affected bowel and anastamose remaining bowel together)
314
Management of nocturnal enuresis
Look for possible underlying causes
General advice: fluid intake, toileting patterns
Reward systems e.g. reward using toilet to pass urine before sleep
1st line = enuresis alarm
Desmospressin - used for short-term control, or if enuresis alarm not appropriate or working
315
What is Osgood-Schlatters disease
Osteochondrosis characterised by inflammation at the tibial tuberosity where the patella ligament inserts
316
Presentation of Osgood-Schlatters diseas
More common in males
10-15 years
Often unilateral (can be bilateral)
Gradual onset of symptoms
Visible or palpable hard and tender lump at tibial tuberosity
Pain in anterior aspect of the knee
Pain exacerbated by physical activity, kneeling, and on extension of the knee
317
Management of Osgood-Schlatters disease
Reduce physical activity
Ice
NSAIDs for symptomatic relief
Once symptoms settle --> stretching + physiotherapy
Symptoms will fully resolve over time - may be left with a hard, boney lump on knee
318
Rare complication of Osgood-Schlatters disease
Avulsion fracture - tibial tuberosity separated from rest of tibia
Requires surgery
319
Investigations for intussusception
Abdominal ultrasound - target/bull's eye sign
Safer + less invasive than barium enema
320
Congenital diaphragmatic hernia pathophysiology
Usually represents a failure of the pleuroperitoneal canal to close completely
321
Presentation of congenital diaphragmatic hernia
Evidence of bowel sounds in a neonate in respiratory distress
322
Risk factors for GORD
Preterm delivery
Neurological disorders
323
Features of GORD
Typically develops before 8 weeks
Vomiting/regurgitation following feeds
324
Management of GORD
Positioning during feeds
Sleeping on backs
Ensure not being overfed - trial smaller + more frequent feeds
Trial of thickened formula
Trial of alginate therapy (not at same time as thickening agents)
Trial of PPI if: unexplained feeding difficulties, distressed behaviour, faltering growth
Prokinetic agents e.g. metoclopramide, only used with specialist advice
325
Complications of GORD
Distress
Failure to thrive
Aspiration
Frequent otitis media
In older children, dental erosion may occur
326
Presentation of neonatal hypoglycaemia
Autonomic features e.g. irritability, jitteriness, tachypnoea, pallor
Neuroglycopenic features e.g. drowsiness and poor feeding/sucking, weak cry, hypotonia, seizures
Apnoea
Hypothermia
327
Causes of persistent/severe neonatal hypoglycaemia
Preterm birth
Maternal diabetes mellitus
IUGR
Hypothermia
Neonatal sepsis
Inborn errors of metabolism
328
Management of neonatal hypoglycaemia
Asymptomatic: encourage normal feeding, monitor blood glucose
Symptomatic, or very low blood glucose: admit to neonatal unit, IV infusion of 10% dextrose
329
6 weeks personal + social milestone
Social smile - refer at 10 weeks
330
3 months personal + social milestones
Laughs - communicates pleasure
Enjoys friendly handling
331
6 months personal + social milestones
Not shy - curious and engaged with people
332
9 months personal + social milestones
Shy - become cautious and apprehensive with strangers
333
12 months personal + social milestone
Engages with others by pointing + handing objects
Waves bye-bye
Claps hands
334
18 months personal + social milestones
Imitates activities e.g. using a phone
335
2 years personal + social milestones
Extends interest to others beyond parents e.g. waving at strangers
Parallel play with other children
Usually dry during day
336
3 years personal + social milestones
Seek out other children + plays with them
Bowel control
337
4 years personal + social milestones
Has best friend
Dry by night
Dresses self
Imaginative play
338
What is William syndrome
Neurodevelopmental disorder
Caused by a microdeletion on chromosome 7
339
Features of William syndrome
Elfin-like facies:
Broad forehead
Starburst eyes (star-like pattern on iris)
Flattened nasal bridge
Long philtrum
Wide mouth with widely spaced teeth
Small chin
Very sociable, trusting personality
Mild LD
Short stature
340
Conditions associated with William syndrome
Supravavlular aortic stenosis
ADHD
Hypertension
Hypercalcaemia
341
Features of meconium aspiration syndrome
Meconium-stained liquor
Respirtory distrss at or shortly following birth
Typical features on CXR: hyperinflation, patchy opacification, and consolidation
Increased O2 requirements
